Paediatrics

Question 1

Describe foetal circulation

Answer 1

Placenta –> umbilical vein –> IVC –> RV –> foramen ovale –> LA –> aorta –> umbilical arteries –> placenta
OR:… RV –> pulmonary artery –> ductus arteriosus –> aorta

Question 2

What are the 3 shunts foetal circulation has?

Answer 2

1. Ductus venosus
2. Foramen oval
3. Ductus arteriosus

Question 3

What is the function of the Forman oval and ductus arterioles in the foetal circulation?

Answer 3

Used to bypass the non-functioning lungs

Question 4

Give 4 ways congenital heart disease may be identified/present?

Answer 4

1. Antenatal US screening
2. Detection of a heart murmur
3. Cyanosis
4. Heart failure
5. Shock

Question 5

What are the hallmarks of an innocent ejection murmur?

Answer 5

All have S in 
- aSymptomatic patient 
- Soft blowing murmur 
- Systolic murmur only, not diastolic 
- left Sternal edge 
AND normal HS with no added sounds, no parasternal thrill and no radiation 
30% children = innocent murmur

Question 6

Give 3 possible causes of cyanosis in newborn with respiratory distress (RR >60bpm)

Answer 6

1. Cardiac disorders –> transposition of great arteries, teratology of Fallot
2. Respiratory disorders –> surfactant deficient, meconium aspiration, pulmonary hypoplasia
3. Persistent pulmonary HTN of the newborn = filature of pulmonary vascular resistance to fall after birth
4. Infection –> septicaemia from group B strep
5. Metabolic disease –> metabolic acidosis and shock

Question 7

Give 2 possible causes of heart failure in neonates

Answer 7

Often obstructed (duct dependent) systemic circulation problems

1. Hypoplastic left heart syndrome
2. Critical aortic valve stenosis
3. Severe coarctation of the aorta
4. Interruption of the aortic arch

Question 8

Give 2 possible causes of heart failure in infants

Answer 8

Due to High pulmonary blood flow

1. Ventricular septal defect
2. Atrioventricular septal defect
3. Large persistent ductus arteriosus

Question 9

Give 2 possible causes of heart failure in older children and adolescents

Answer 9

Often right or left heart failure

1. Eisenmengers syndrome (right heart failure only)
2. Rheumatic heart disease
3. Cardiomyopathy

Question 10

Give 2 possible causes of heart failure due to circulation failure

Answer 10

1. Reduced oxygen carrying capacity –> anaemia
2. Increased tissue demands –> sepsis
3. Iatrogenic –> too much fluid

Question 11

Give 3 symptoms of heart failure

Answer 11

1. Breathlessness –> especially on feeding and exertion
2. Sweating
3. Poor feeding
4. Recurrent chest infections
5. Tachycardia and tachypnoea
6. Heart murmur, gallop rhythm
7. Cardiomegaly and hepatomegaly
8. Sever acidosis, collapse, death –> if severe obstructive lesion causing duct dependent systemic circulation

Question 12

Name 4 congenital heart problems that can cause a left to right shunt

Answer 12

1. VSD
2. ASD
3. AVSD
4. PDA

Question 13

What is a VSD?

Answer 13

Defect in septum between right and left ventricles

Question 14

Give 5 signs of a VSD

Answer 14

1. Poor feeding and failure to thrive
2. tachypnoea
3. Thrill
4. Pansystolic murmur (LLSE)
5. Sweating
6. Gallop rhythm

Question 15

Would you expect to see on a CXR of a patient with a VSD?

Answer 15

1. Cardiomegaly
2. Pulmonary oedema
3. Enlarged pulmonary arteries

Question 16

What is the treatment for a VSD?

Answer 16

Monitor
Large and HF present = diuretics, captopril, calories
Symptomatic after a year = surgery –> cardiac catheterisation or open

Question 17

Name the classifications of ASD

Answer 17

1. Ostium secundum = middle of atrial septum with margins on both sides (standard = 80%) involving foreman ovale
   
   • Blood from left to right atrium
2. Ostium primum = bottom of atrial septum, close to the mitral and aortic valves
   - Often associated with AVSD
3. Sinus venosus = at the top of atrial septum, anatomically and echocardiographically hard to pick up

Question 18

Why are ASD’s often asymptomatic?

Answer 18

Because blood flow in the atria is low pressure and so breathlessness etc is uncommon

Question 19

Give 3 signs of ASD

Answer 19

1. Fixed and widely split S2 sound
2. Ejection systolic murmur in pulmonary region (LUSE)
3. Palpitations
4. Recurrent chest infections
5. Difficulty breathing

Question 20

AVSD is a common defect in people with what chromosomal abnormality?

Answer 20

Trisomy 21 (Downs syndrome)

Question 21

What is the treatment for an AVSD?

Answer 21

Surgery to repair at 2-5 years of age (to avoid long term complications)

Question 22

What is a PDA?

Answer 22

Vessel connecting aorta and pulmonary artery is still patent
- Blood from aorta –> pulmonary artery due to pressure gradient so increased high pressure blood going into lungs

Question 23

Give 3 signs of a PDA

Answer 23

1. Poor feeding, failure to thrive
2. Tachypnoea
3. Active pericardium
4. Thrill
5. Continuous machinery murmur in pulmonary area
6. Hepatomegaly
7. Oedema

Question 24

Describe the management of congenital health defects that cause a L to R shunt

Answer 24

1. Stabilise patient
2. Increase calorie intake
3. NG tube
4. Dietetics and ACEi to prevent HF Symptoms
5. Surgical repair

Question 25

Would a patient with a L --> R shunt or a R --> L shunt appear cyanotic?

Answer 25

A patient with a R --> L shunt = cyanotic

Question 26

Name 2 congenital heart problems that can cause a R --> L shunt

Answer 26

1. Teratology of fallout | 2. Transposition of the great arteries

Question 27

What are the 4 components that make up Teratology of Fallot?

Answer 27

1. Pulmonary stenosis 2. VSD 3. Overriding aorta 4. Right ventricular hypertrophy (RVH)

Question 28

Give 3 signs of Teratology of Fallot

Answer 28

1. Cyanosis 2. Acidosis 3. Collapse 4. Harsh ejection systolic murmur (LSS) 5. Hypercyanotic spells = irritability, inconsolable crying

Question 29

What is the management of Teratology of Fallot?

Answer 29

- Manage hyper cyanotic spells if >15 mins = sedation, pain relief, IV propranolol, bicarb - Very cyanosed = Black Taussig shunt - Surgical repair = at 6-9 months of age

Question 30

What is the Transposition of the Great Arteries?

Answer 30

Great arteries come of f the wrong ventricle - Dexoygenated blood goes into aorta = central cyanosis - Oxygenated blood from lung goes into pulmonary artery

Question 31

What would you see on a CXR of a patient with Transposition of the Great Arteries?

Answer 31

- Narrow upper mediastinum with an ‘egg on side’ appearance | - Increased pulmonary vascular markings

Question 32

How do you manage Transposition of the Great Arteries?

Answer 32

1. Atrial septostomy at birth = make foramen oval bigger to increase mixing of blood 2. Prostaglandin induction = maintain patent ductus arteriosus 3. Surgical repair within 1 week = arterial switch operation

Question 33

What is coarctation of the aorta?

Answer 33

= Narrowing of aorta Arterial duct tissue encircles the aorta at the point of insertion fo the duct When the duct closes, the aorta constricts, causing severe obstruction to LV outflow

Question 34

Give 3 signs of coarctation of the aorta

Answer 34

1. Radio-femoral delay 2. Weak femoral pulses 3. Difference in pre and post ductal saturations 4. 4 Limb BP = discrepancy between upper limb (higher and bounding pulses) and lower limb BP 5. if PDA closed/closing babies = acidotic, collapse, HF

Question 35

Give 3 signs of aortic stenosis

Answer 35

Can be asymptomatic 1. Palpable thrill (in supersternal region and carotid area) 2. Ejection systolic murmur (Aortic area = RUSE) 3. Delayed and soft aortic 2nd sound 4. LVH

Question 36

How is aortic stenosis managed?

Answer 36

- Regular clinics and ECHOs - Symptoms on exercise or high resting pressure gradient (>64mmHg) across aortic valve = Balloon valvotomy - Aortic valve replacement eventually

Question 37

Give 3 signs of Pulmonary stenosis

Answer 37

1. Ejection systolic murmur (LUSE) - often radiates to back 2. Ejection click 3. RV heave (if severe) 4. RVH

Question 38

How is pulmonary stenosis managed?

Answer 38

Transcatheter balloon dilation --> when pressure gradient across pulmonary valve >64 mmHg

Question 39

What is a possible consequence of persistent pulmonary hypertension, like that seen in CHD associated with a L --> R shunt?

Answer 39

Eisenmenger Syndrome

Question 40

Describe the pathophysiology beging Eisenmenger Syndrome

Answer 40

L to R shunt --> persistent plenary HTN --> high pressure pulmonary blood flow --> damage blood vessels --> pulmonary HTN --> back up blood into right heart, RV pressure increase --> shunt direction reverses (R --> L) --> cyanotic

Question 41

Name 3 congenital heart problems that are often associated with Down's syndrome

Answer 41

1. AVSD 2. Teratology of Fallot 3. VSD

Question 42

Name 3 congenital heart problems that are often associated with Turner syndrome

Answer 42

1. Coarctation of the aorta 2. Aortic stenosis 3. Aortic dissection 4. Biscuspid aortic valve

Question 43

Name 3 URTI

Answer 43

1. Rhinitis 2. Otitis media 3. Pharyngitis 4. Tonsillitis 5. Laryngitis

Question 44

Name 3 LRTI

Answer 44

1. Bronchitis 2. Croup 3. Epiglottitis (bacterial) 4. Trachetitis 5. Bronchiolitis 6. Pneumonia

Question 45

Name 3 viruses that can cause childhood respiratory infections

Answer 45

80-90% of childhood resp infections = viral 1. Respiratory syncytial virus (RSV) 2. Rhinoviruses 3. Parainfluenza 4. Metapneumonovirus 5. Adneoviruses

Question 46

Name 3 important bacterial pathogens that can cause childhood respiratory infections

Answer 46

1. Strep pneumoniae (pneumococcus) 2. Haemophilus influenzae 3. Moraxella catarrhalis 4. Bordetella pertussis (= Whooping cough) 5. Mycoplasma pneumoniae

Question 47

Give 3 risk factors for respiratory infections

Answer 47

1. Parental smoking 2. Poor socioeconomic status --> large family size, overcrowded, damp housing 3. Poor nutrition 4. Underlying lung disease --> bronchopulmonary dysplasia (preterm), CF, asthma 5. Immunodeficiency

Question 48

Name 4 LRTI that could be caused by RSV | What investigation is done if RSV is suspected?

Answer 48

1. Acute bronchiolitis 2. Wheezy bronchitis 3. Asthma exacerbation 4. Pneumonia 5. Croup Nasopharyngeal aspirate rapid testing --> PCR

Question 49

Why are infants more susceptible to descending infection?

Answer 49

Poor innate immune response

Question 50

What is stridor and give 3 causes of stridor

Answer 50

High pitched audible wheezing/whistling sounds when a person inhales, exhales or both due to narrowed or partially blocked upper airway (monophonic) disrupting the airflow 1. Croup 2. Epiglottis 3. Bacterial tracheitis 4. Anaphylaxis 5. Foreign body 6. Laryngomalacia

Question 51

What is croup?

Answer 51

Acute larygnotracheobronchitis --> trachea, bronchi and larynx are all affected

Question 52

Who most commonly gets croup, and at what time of year?

Answer 52

6 months to 6 years old --> peak incidence = 2nd year of life Autumn = most common time fo year

Question 53

What virus commonly causes croup?

Answer 53

Parainfluenza virus | = Metapneumovirus, RSV, Influenza

Question 54

Give 3 signs of croup

Answer 54

1. Barking seal like cough --> often worse at night 2. Preceded by low grade fever and coryza 3. Stridor 4. Hoarseness 5. Recession

Question 55

How do you treat croup?

Answer 55

- Do NOT examine throat, force to lie down or agitate child - Can be self limiting - Steroids --> oral dexamethasone, oral prednisone, nebuliser steroids (budesonide) - Severe upper airway obstruction = nebulised epinephrine (adrenaline) with O2 by facemark

Question 56

Name a bacteria that causes epiglottitis?

Answer 56

H. influenza B (HiB) Acute epiglottis = severe life threatening acute illness Presents at 1-6 years old

Question 57

Give 4 signs of acute epiglottitis

Answer 57

1. Pyrexial (>39) 2. Septic 3. Stridor - soft inspiratory 4. Drooling 5. Huge inflamed epiglottis that blocks oesophagus --> muffled voice/don't speak, sit forward, neck extended with open mouth, minimal/absent cough

Question 58

What is the management of acute epiglottitis?

Answer 58

- Intubation (for 24 hours) - Blood cultures and IV Abx (cefuroxime) --> 2-5 days - Prophylaxis with rifampicin for close contacts

Question 59

Give 2 possible causes of bronchitis

Answer 59

= bacterial 1. Pneumococcus 2. Haemophilus influenza

Question 60

Give 2 signs of bronchitis in children

Answer 60

1. Chronic cough --> worse at night 2. Sore throat 3. Fever

Question 61

Would you expect a patient with bronchitis or with bronchiolitis to be hypoxic and tachypnoeic? Explain why

Answer 61

Bronchiolitis --> affects the respiratory portion of the airway, where gas exchange takes place therefore you may see hypoxia and tachypnoea Bronchitis affects the conducting portion of the airway and so is unlikely to have these effects

Question 62

When would a child usually present with bronchiolitis?

Answer 62

1-9 months old | - Rare after 1 year

Question 63

Give 3 risk factors of bronchiolitis

Answer 63

1. Prematurity 2. Chronic lung disease 3. CF 4. Immunocompromised 5. Parental smoking

Question 64

Give 2 possible causes of bronchiolitis

Answer 64

1. RSV = 80% 2. Rhinovirus 3. Influenza 4. Adenovirus 5. Parainfluenza

Question 65

Give 3 signs of bronchiolitis

Answer 65

3-5 days of symptoms, coryza symptoms too 1. Recurrent cough = sharp, dry 2. Widespread wheeze 3. Fine end inspiratory crackles 4. Increasing breathlessness --> feeding difficulties 5. Subcostal and intercostal recession

Question 66

How is bronchiolitis managed?

Answer 66

- Conservative - Oxygen or ventilation - NG feeds --> frequent but small - Ribavirin = antiviral - CF patients = broad spec Abx for 2-3 weeks

Question 67

How is bronchiolitis prevented and who is offered prevention?

Answer 67

Monthly IM injection over winter of Palivizumab (monoclonal Abs) High risk = Chronic lung disease, congenital heart disease, SCID, CF

Question 68

What is pneumonia?

Answer 68

Inflammation of the lung parenchyma with congestion

Question 69

Give 3 possible viral causes of pneumonia

Answer 69

More common in young children 1. RSV 2. Influenza A and B 3. Parainfluenza 4. Adenovirus 5. Metapneumovirus

Question 70

Give 3 possible bacterial causes of pneumonia

Answer 70

More common in older children 1. Strep pneumococcus 2. HiB 3. Staph aureus 4. Klebsiella 5. Mycobacteria penumoniae

Question 71

Give 5 signs of pneumonia in children

Answer 71

1. Fever 2. Miserable 3. Tachypnoea 4. Cough 5. Poor feeding 6. Lethargy

Question 72

Describe the diagnostic criteria for pneumonia in children

Answer 72

History of cough and/or difficulty breathing (<14 days duration) with increased respiratory rate - <2 months = >60/min - 2 to 11 months = >50/min - >11 months = >40/min Bacterial pneumonia in children <3 y/o with fever, chest recession and RR >50/min

Question 73

What investigations might you do in a child who you suspect has pneumonia?

Answer 73

- CXR --> look for consolidation - Blood cultures It is often difficult to get a sputum sample

Question 74

How do you treat pneumonia?

Answer 74

- Oxygen, analgesia, IV fluids - Abx --> based on age, severity and CXR - Newborn = broad spec - Older infants = oral amoxicillin - >5 y/o = amoxicillin or oral macrolide (erythromycin)

Question 75

What antibiotics might you use in a child with pneumonia caused by mycoplasma pneumoniae?

Answer 75

Mycoplasma pneumoniae is intracellular and so amoxicillin won't work therefore give macrolides e.g. clindamycin, erythromyocin

Question 76

Give 2 possible complications of pneumonia

Answer 76

1. Pleural effusions 2. Empyema --> drain 3. Fibrin strands

Question 77

What is a unilateral pleural effusion suggestive of?

Answer 77

Infection --> parapneumonia or empyema

Question 78

What is a bilateral pleural effusion suggestive od?

Answer 78

Fluid overload --> HF, nephrotic syndorme

Question 79

What is whooping cough caused by?

Answer 79

Bordetella pertussis

Question 80

How does whooping cough present?

Answer 80

- Week of coryza (Catarrhal phase) - Paroxysmal cough followed by inspiratory whoop and vomiting (paroxysmal phase) - Symptoms gradually decrease over 3-6 weeks (convalescent phase)

Question 81

How do you treat whooping cough?

Answer 81

Erythromycin --> decreases symptoms only if started during catarrhal phase Close contacts = erythromycin prophylaxis, unvaccinated should be vaccinated

Question 82

What is the difference between wheeze and stridor?

Answer 82

``` Wheeze = Expiratory polyphonic noise which indicates narrowing in the medium to small sized airways Stridor = Monophonic high pitched noise heard on inspiration ```

Question 83

Describe the aetiology of recurrent wheeze

Answer 83

1. Persistent infantile wheeze 2. Viral episodic wheeze 3. Asthma

Question 84

What is persistent infantile wheeze normally associated with/exacerbated by?

Answer 84

Affects small airways | Associated with parental smoking or post viral infection

Question 85

Are inhalers likely to help a child with persistent infantile wheeze?

Answer 85

No - unlikely to help, symptoms improve as child gets older, usually resolved by 3 y/o

Question 86

What is viral episodic wheee normally associated with/exacerbated by?

Answer 86

Normally follow URTI/viral infection | RFs = maternal smoking during and/or after pregnancy, prematurity

Question 87

How does viral episodic wheeze differ from asthma?

Answer 87

- NO interval symptoms - NO excess of atopy - NO benefit from regular inhaled steroids

Question 88

Are inhalers likely to help a child with viral episodic wheeze?

Answer 88

Bronchodilators may help in acute episodes No benefit from inhaled steroids Symptoms usually improve with age, resolve by 5 y/o mostly

Question 89

What is asthma?

Answer 89

Recurrent episode of SOB, wheeze and cough caused by reversible airway obstruction - Most common chronic respiratory disorder in childhood, 15-20% of children

Question 90

What are the 3 main pathophysiological characteristics of asthma?

Answer 90

1. Ariflow limitation 2. Airway hyper-responsiveness 3. Bronchial inflammation

Question 91

Give 3 risk factors for asthma

Answer 91

1. Eczema 2. Hayfever 3. Family history of atopic conditions 4. Low birth weight 5. Bottle fed

Question 92

Name 3 possible precipitants of asthma

Answer 92

1. Cold weather 2. Exercise 3. Strong emotions 4. Allergens (dust/pollen) 5. Pets 6. Drugs --> NSAIDs, BB

Question 93

Give 3 signs of asthma

Answer 93

1. Wheeze 2. Episodic cough 3. SOB 4. Diurnal variation --> worse at night and in early morning 5. Interval symptoms 6. Tachypnoea 7. Barrel chest

Question 94

What are the features of moderate acute asthma?

Answer 94

- Oxygen saturation >92% - Peak flow >50% predicted - No clinical features of severe asthma

Question 95

What are the features of Severe acute asthma?

Answer 95

- Too breathless to talk or feed - Use of accessory neck muscle - Oxygen saturation <92% - RR > 50 bpm in children 2-5, >30 in children >5 - Pulse >130/bpm in children aged 2-5, >120 bpm in children >5 - Peak flow <50% predicted

Question 96

What are the features of Life threatening acute asthma?

Answer 96

- Oxygen saturation <92% - Poor respiratory effort - Cyanosis - Fatigue - Drowsiness/altered consciousness - Silent chest - Peak flow <33% predicted

Question 97

What investigations might you do for someone with suspected asthma?

Answer 97

- Spirometry - PEFR <80% predicted - FEV1:FVC <80% - Bronchodilator response to B agonist therapy  15% increase in FEV1 or PEFR - Skin prick testing for common allergens - CXR --> hyperinflation, flattened hemi-diaphragms, peribronchial cuffing, atelectasis

Question 98

How would you manage an acute exacerbation of asthma in a child?

Answer 98

- O2 - Beta agonist nebulised - Prednisolone 1mg/kg If still not improving - Nebulised ipratropium or salbutamol - Aminophylline/MgSO4/salbutamol infusion

Question 99

Name 2 preventer inhalers

Answer 99

ICS act as preventers - Beclomethasone - Budesonide

Question 100

Name 2 relievers inhalers

Answer 100

Beta agonists --> salbutamol | Muscarinic antagonist --> Ipratropium bromide

Question 101

Describe the step-wise approach to asthma management

Answer 101

SABA -> SABA + ICS -> LABA + ICS -> LABA + increased dose of ICS -> LABA + daily PO steroids

Question 102

Give 3 long term risk of systemic steroids

Answer 102

1. Adrenal suppression 2. Growth suppression 3. Osteoporosis

Question 103

Why might asthma treatment fail in children?

Answer 103

1. Adherence (compliance) 2. Wrong diagnosis 3. Environmental factors --> pets, smokers 4. Choice of drug/device 5. bad disease

Question 104

Give 5 differentials for a painful joint

Answer 104

``` Life threatening differentials 1. Leukaemia 2. Septic arthritis 3. NAI Pain and Swelling 4. Trauma 5. Infection 6. Reactive swelling 7. JIA Pain and no swelling 8. Hypermobile joint syndrome 9. Perthe's disease ```

Question 105

What are the 3 main differentials for a limping child?

Answer 105

1. Infection --> sepsis/osteomyelitis 2. Trauma --> NAI, fracture 3. Tumour

Question 106

What is the likely cause of a limp in a child aged 0-3?

Answer 106

1. Truama --> NAI, fracture 2. Osteomyelitis/septic arthritis 3. Developmental dysplasia of the hip (DDH)

Question 107

What is the likely cause of a limp in a child aged 3-10?

Answer 107

1. Trauma 2. Transient synovitis/irritable hip 3. Osteomyelitis or septic arthritis 4. Perthe's disease 5. JIA

Question 108

What is the likely cause of a limp in a child aged 10-15?

Answer 108

1. Trauma 2. Osteomyelitis/septic arthritis 3. Slipped upper femoral epiphysis (SUFE) 4. JIA 5. Perthes disease

Question 109

What must you remember to consider as a differential in a limping child?

Answer 109

Intra-abdominal pathology --> hernia, testicular torsion

Question 110

Why might you want to ask about socioeconomic class and smoking status in child presenting with a limp?

Answer 110

Social deprivation and passive smoking are RFs for Perthe's disease

Question 111

What investigations might you want to do on a child presenting with a limp?

Answer 111

1. General observations --> HR, BP, temp, RR, O2 sats 2. FBC, BM, ESR, CRP 3. XR --> AP and lateral views of the joint and joints above and below 4. USS --> effusion in joints 5. CT/MRI

Question 112

What is a Toddlers Fracture?

Answer 112

Subtle displaced spiral fracture of the tibia - History of small fall or twisting injury - Immobilise

Question 113

What is Transient synovitis?

Answer 113

Acute onset joint inflammation following illness (often respiratory)

Question 114

How does transient synovitis present?

Answer 114

- Sudden onset limp --> usually unilateral - Hip and/or knee pain - Limited ROM --> particularly internal rotation - Afebrile or mild fever, doesn't appear ill

Question 115

How do you manage transient synovitis?

Answer 115

Self limiting usually within 2 weeks | Rest, physio, NSAIDs

Question 116

What is the most common organism that causes septic arthritis?

Answer 116

Staph aureus | - Most commonly in children <2

Question 117

Give 3 signs of septic arthritis

Answer 117

1. Systemically very unwell = acute febrile illness 2. Pain at rest 3. Hip kept flexed, abducted and externally rotated 4. Raised WCC and CRP

Question 118

Describe Kocher's criteria

Answer 118

``` Diagnosis of septic joint = 3/4 - Temp >38.5 - Cannot weight bare - ESR >40 - WCC >12 (CRP >20 is independent RF) ```

Question 119

How is septic arthritis managed?

Answer 119

``` IV ABx (after aspirate) for up to 3 weeks - Surgical emergency and need joint washout ```

Question 120

What is Perthe's disease?

Answer 120

A self limiting idiopathic disease characterised by vascular necrosis of the femoral head

Question 121

Who is most commonly affected by Perthes disease?

Answer 121

Boys (80%) aged 5-10 years old

Question 122

Give 3 risk factors for Perthe's disease

Answer 122

1. Low birth weight 2. Low socioeconomic status 3. Short stature 4. Passive smoking 5. FHx

Question 123

Give 3 signs of Perthe's disease

Answer 123

1. Pain in hip or knee causing limp 2. Trendelenburg sign 3. Proximal thigh atrophy 4. Decreased ROM --> especially abduction and internal rotation) 5. Effusion

Question 124

What investigations might you do for someone with suspected Perthes disease?

Answer 124

- Roll test --> should invoke guarding or spasm - Assess ROM - AP and lateral XR - MRI

Question 125

Describe the management of Perthe's disease

Answer 125

Contain the hip, either on their own or with the aid of plasters, brace, physio or surgery - >8y/o = surgical --> femoral/pelvic osteotomies

Question 126

What is SUFE?

Answer 126

Slipped upper femoral epiphysis = displacement of the upper femoral epiphysis on the metaphysis through the hypertrophic zone of the growth plate

Question 127

Who is most likely to be affected by SUFE?

Answer 127

A pre pubescent obese male (12-15 years)

Question 128

What can SUFE be associated with?

Answer 128

- Hypothyroidism - Downs - Radio/chemotherapy - Obese, hypogonadal - Tall, thin, often boys, post growth spurt

Question 129

How does SUFE present?

Answer 129

- Several week history of vague groin or thigh discomfort --> 40% bilateral - Antalgic/waddling gait - Decreased ROM - May be leg shortening

Question 130

What is the treatment of SUFE?

Answer 130

Analgesia | Surgical pinning of hip with or without reduction

Question 131

What is DDH?

Answer 131

Developmental dysplasia of the hip | - Abnormal development resulting in instability, dysplasia, subluxation and possible dislocation of the hip

Question 132

Give 3 risk factors of DDH

Answer 132

1. Female 2. First born 3. Breech birth 4. Family history 5. C section

Question 133

What tests can be done on clinical examination in the neonatal period to pick up DDH?

Answer 133

1. Barlow test = whether hip is dislocatable --> adduct and depress 2. Ortolani test = if hip is dislocated --> anterior and abduct

Question 134

Give 3 symptoms of DDH

Answer 134

1. Painless limp 2. Delayed walking 3. Prone to falls

Question 135

Give 3 signs fo DDH

Answer 135

1. Asymmetrical skin creases in thigh or buttock 2. Unequal leg length 3. Galeazzi sign (flex knees with feet together) --> if +ve affected femur appears short due to dislocated hip joint (-ve if bilateral DDH) 4. Older children = limp, +ve trendelenburg

Question 136

What investigations are done on someone who has suspected DDH?

Answer 136

USS = at 6-8 weeks | AP posterior XR = examination of choice after 6 months

Question 137

Describe the management of DDH

Answer 137

1. Pavlik harness --> <6 months | 2. Surgical reduction

Question 138

What are the 2 main risks associated with the surgical management of DDH?

Answer 138

1. Avascular necrosis | 2. Re-dislocation

Question 139

What is chondrosarcoma?

Answer 139

A malignancy neoplasm of cartilage

Question 140

What is the criteria for making a clinical diagnosis of juvenile idiopathic arthritis (JIA)?

Answer 140

Persistent joint swelling/stiffness >6 weeks in children <16 and no other cause is identified

Question 141

What investigations are done for someone with suspected JIA?

Answer 141

Diagnosis of exclusion - FBC, ESR, CRP - Infection --> throat swab, urinalysis, viral serology, cultures - RF, ANA - XR, US, MRI - Synovial fluid aspirate - Ophthalmology review

Question 142

What symptoms are associated with JIA?

Answer 142

1. Swelling --> persistent 2. Joint stiffness --> morning 3. Loss of ROM 4. Limp 5. Pain 6. Uveitis 7. Fever 8. Salmon pink rash

Question 143

What extra articular features might you see in someone with JIA?

Answer 143

1. Psoriasis 2. Dactylitis 3. Nail pitting 4. Rash 5. Fluctuating fever 6. Uveitis

Question 144

Name 5 types fo JIA

Answer 144

1. Oligoarticular (commonest) 2. Polyarticular 3. Psoriatic 4. Enthesitis related 5. Systemic onset JIA

Question 145

Define oligoarticular JIA

Answer 145

<4 joint involved, often asymmetrical Normal ANA+ and associated with a high risk of developing uveitis Most commonly affects knee --> ankle --> wrist --> elbow

Question 146

Define polyarticular JIA

Answer 146

>4 joint involved, often symmetrical and more destructive Commonly affects the small joints of hands/feet Can be RF negative or RF positive

Question 147

How is RF negative polyarticular JIA diagnosed?

Answer 147

Diagnosis of exclusion = IgM RF negative 3 months apart | - Systemic features, asymmetrical joints, chronic hyperaemia, tenosynovitis, bursitis

Question 148

How is RF positive polyarticular JIA diagnosed?

Answer 148

``` Chronic symmetrical inflammatory polyarthritis with positive RF on 2 occasions at least 3 months apart (worst prognosis) 4 or more criteria for at least 6 weeks - Morning stiffness >1hr - Arthritis in at least 3 joints - Hand arthritis - Systemic arthritis - Rheumatoid nodules - Rheumatoid factor +ve - Erosions on XR ```

Question 149

How is psoriatic arthritis diagnosed?

Answer 149

Inflammatory arthritis in the presents of psoriasis OR Inflammatory arthritis with dactylics OR psoriatic nail changes + first degree relative with psoriasis - Arthritis = asymmetrical large joint (knee, ankles) or small joint polyarthritis (MCP, PIP, DIP) - Dactylics, tendonitis, uveitis

Question 150

How is enthesitis related arthritis diagnosed

Answer 150

Arthritis with enthesitis (inflammation of tendons, ligamentous or muscular insertion on to bones) OR Arthritis alone or enthesitis alone with 2 of: - Sacroiliac joint tenderness - Inflammatory spinal pain - HLAB27 +ve - First degree relative FHx of uveitis - Age of onset >6 years

Question 151

How might enthesitis related arthritis present?

Answer 151

- Oligo or polyarthritis in lower limbs - Enthesitis - Spinal pain - Systemic features - Acute anterior uveitis - Plantar fascitis

Question 152

What might you see on an X-ray of someone with enthesitis related arthritis?

Answer 152

Romanus lesions = small erosions of corners of vertebral bodies

Question 153

What signs might you see in someone with systemic onset JIA?

Answer 153

- Spiking daily fever - Salmon pink rash --> macular, urticarial - Lymphadenopathy - Hepatosplenomeagly - Polyserositis --> pericarditis, pleurites, sterile peritonitis - Arthritis --> after 6 months, oligo or polyarthritis

Question 154

Describe the non medical treatment for JIA

Answer 154

1. Information 2. Education 3. SUpport 4. Liaison with school 5. Exercise and physiotherapy

Question 155

Describe the medical treatment for JIA

Answer 155

1. NSAIDs --> ibuprofen, naproxen, diclofenac 2. Inraarticular steroid injection 3. Methotrexate 4. Anti TNF --> etanercept

Question 156

What must you screen for in a patient with JIA?

Answer 156

Uveitis --> every 3 months | - Often asymptomatic in children

Question 157

Give 5 potential consequences that can occur if you fail to treat JIA

Answer 157

1. Damage 2. Deformity 3. Disability 4. Pain 5. Bony overgrowth 6. Uveitis 7. Ligament laxity

Question 158

Name a severe, potentially life threatening complicated of systemic onset JIA

Answer 158

Macrophage activation syndrome (MAS)

Question 159

What signs might you see in someone with macrophage activation syndrome (MAS)?

Answer 159

- High fever - Hepatosplenomegly - CNS dysfunction - Purpuric rash - Cytopaenia

Question 160

What is the treatment for macrophage activation syndrome (MAS)?

Answer 160

Supportive treatment | Steroids

Question 161

Define osteoporosis in children

Answer 161

>1 vertebral crush fracture OR Bone density <2.0 and either 2 or more long bone fractures by 10y/o or >3 fracture by 19 y/o

Question 162

Describe the aetiology of osteoporosis in children

Answer 162

1. Inherited/congenital --> osteogenesis imperfecta, inborn errors, idiopathic 2. Acquired --> drug induce, malabsorption, immobilisation

Question 163

What investigation might you do to determine fi a child has osteoporosis?

Answer 163

DEXA scan

Question 164

How do you manage oestoporosis?

Answer 164

- Calcium and vitamin D supplements - Regular weight bearing exercise - Minimise oral corticosteroids - Sometimes bisphosphonates

Question 165

What is osteogenesis imperfecta?

Answer 165

AD inherited osteoporotic condition that leads to bone weakness in children Due to defect in type 1 collagen genes

Question 166

Describe the inheritance of osteogenesis imperfecta

Answer 166

AD inheritance in 90%

Question 167

Give 5 signs of osteogenesis imperfecta

Answer 167

1. Bone fragility 2. Fractures 3. Deformity 4. Impaired motility --> ligamentous laxity, sarcopenia 5. Poor growth 6. Deafness 7. Blue sclera

Question 168

What classifications is used in osteogenesis imperfecta?

Answer 168

``` Sillence classification I = Mild II = Lethal III = Progressively deforming, severe IV = Moderate ```

Question 169

Describe the treatment for osteogenesis imperfecta

Answer 169

- MDT approach --> PT, OT, specialists, surgeons - Bisphosphonates --> pamidronate - Surgery if needed

Question 170

What are bisphosphonates mode of action?

Answer 170

Inhibit osteoclasts - In osteogenesis imperfecta they Increase LS and TB bone mass, reduce fracture frequency, increase vertebral height, suppress bone markers, reduce pain, increase overall mobility and have no adverse effect on growth

Question 171

What is rickets?

Answer 171

A disorder of bone mineralisation --> under mineralisation leading to bone weakness

Question 172

What is the main cause of rickets?

Answer 172

Vitamin D deficiency - Lack on sunlight - Poor nutrition = including prolonged unsupplemented breast feeding - Intestinal malabsorption = CF, coeliac

Question 173

What does vitamin D do?

Answer 173

1. Increase calcium absorption from the gut 2. Increased calcium release from the bone 3. Increases calcium reabsorption at the kidneys 4. Maternal vitamin D influences bone size and mass in childhood

Question 174

Give 3 sources of Vitamin D

Answer 174

1. Sunlight 2. Cereals 3. Egg yolk 4. Oily fish 5. Spreads

Question 175

Give 4 signs of Rickets

Answer 175

1. Metaphyseal swelling 2. Bony deformities --> bowing legs 3. Motor delay 4. Hypotonia 5. Fractures 6. Craniotabes --> ping pong sensation of the skull 7. Splayed and frayed metaphyses 8. Harrison sulcus

Question 176

What investigations might you do to determine if a child has Rickets?

Answer 176

1. Serum biochemistry --> low PO4, Raised AlkP 2. Bone profile 3. Measure Vitamin D levels --> low with raised PTH 4. XR --> cupping and fraying of metaphyses

Question 177

What vitamin D level suggests skeletal ill health?

Answer 177

<10 nmol/L >25 nmol/L = possible adverse effects >50 nmol/L = no obvious adverse effects

Question 178

Describe the treatment of Rickets

Answer 178

- Treat underlying problem | - If vitamin D deficiency, give Adcal D3

Question 179

Secretion fo which hormone will increase in response to low calcium?

Answer 179

PTH | - Increase PTH = bone resorption, Ca reabsorption at kidneys and Ca absorption at gut

Question 180

Name an organism that commonly causes osteomyelitis in children

Answer 180

Staph aureus

Question 181

Give 3 signs of osteomyelitis in children

Answer 181

1. Joint pain 2. Lethargy 3. Fever

Question 182

What investigations might you do on child who you suspect has an infected joint?

Answer 182

- XR - MRI - Blood cultures - JOint aspirate

Question 183

How would you treat a child with osteomyelitis?

Answer 183

IV cefuroxime or IV flucloxicillin | 6 weeks of treatment, can switch to PO when improving

Question 184

Define UTI

Answer 184

Growth of bacteria within the urinary tract (10^5 organism/ml grown on culture of appropriate sample)

Question 185

What is the importance of diagnosing UTI's in children?

Answer 185

- Can cause significant acute illness - 50% rate of reoccurrence - 50% have structural abnormality - Long term complications --> kidney scarring, HTN, CKD

Question 186

What is the most common causative organism of UTI in children?

Answer 186

E. coli Can also have Klebsiella, proteus (B>G), pseudomonas (can indicate structural abnormality)

Question 187

Give 3 signs of UTI in infants

Answer 187

1. Fever 2. Lethargy 3. Irritability 4. Poor feeding or failure to thrive 5. Vomiting

Question 188

Give 3 signs of UTI in children

Answer 188

1. Dysuria 2. Frequency 3. Abdominal pain or loin tenderness 4. Fever with or without rigors

Question 189

What investigations might you do on a child who you suspect has a UTI?

Answer 189

1. Urine dip --> nitrates, 2. MCS on clean catch urine Further investigations (if indicated) 3. USS KUB ---> obstruction, scarring, structural abnormalities 4. DMSA --> renal scarring 5. MCUG --> function of bladder and reflux

Question 190

When would you do a USS KUB on a child with a UTI?

Answer 190

- <6 months old m - Recurrent UTIs - Atypical UTI = seriously ill/septic, poor urine flow, abdominal or bladder mass, raised creatinine, failure to respond to Abx within 48 hrs, infection with non E.coli organism

Question 191

What is the treatment for UTI in children?

Answer 191

IV cefuroxime | Switch to PO Trimethoprim if stable

Question 192

How would you treat a UTI that has been caused by ESBL E.coli?

Answer 192

Give Meropenem | - ESBL bacteria are resistant to all penicillin and cephalosporins

Question 193

What is oedema?

Answer 193

Increase in interstitial fluid | Clinically = swelling, pitting oedema, facial puffiness, ascites, pleural effusions, pulmonary oedema

Question 194

Give 3 causes of oedema

Answer 194

1. Impaired lymph drainage --> lynmphoedema 2. Impaired venous drainage --> venous obstruction 3. Lower oncotic pressure (low albumin/protein) --> malnutrition, liver failure, nephrotic syndrome 4. Salt and water retention --> impaired GFR, HF

Question 195

Give 3 causes of proteinuria in children

Answer 195

1. Orthostatic proteinuria 2. Glomerular abnormalities --> nephrotic or nephritic syndrome 3. Increased glomerular filtration pressure 4. Reduced renal renal mass 5. HTN 6. Tubular proteinuria 7. Infection

Question 196

Why does nephrotic syndrome occur?

Answer 196

Podocyte processes leak protein into urine

Question 197

What is nephrotic syndrome characterised by?

Answer 197

1. Heavy proteinuria = frothy urine 2. Hypoalbuminaemia (<35g/L) 3. Oedema --> pitting, gravitational, facial swelling

Question 198

Give 3 causes of nephrotic syndrome

Answer 198

1. Minimal change disease (85%) 2. Focal segmental glomerulosclerosis 3. Membranoproliferative glomerulonephritis 4. Membranous glomerulonephritis 5. Congenital 6. Secondary to --> HSP, SLE, malaria, allergens

Question 199

What investigations would you do in someone with suspected nephrotic syndrome?

Answer 199

- Urine dipstick --> protein - FBC, ESR, U+Es, creatinine, albumin (hypo), alb:Cr >250mg/mmol - Complement levels --> C3 and C4 - Antistreptolysin O or anti-DNAase B titres and throat swab, cANCA, ANA - Urine MS&C --> haematuria/casts - Urinary electrolytes --> sodium concentration - Hep B and C screen - Malaria screen (if travel abroad)

Question 200

Name the 3 types of nephrotic syndrome

Answer 200

1. Steroid sensitive nephrotic syndrome = 85-90% 2. Steroid resistant nephrotic syndrome 3. Congenital nephrotic syndrome

Question 201

Give 3 signs suggestive of steroid sensitive nephrotic syndrome

Answer 201

- 1 to 10 years old (peak = 2-5y/o) - Norma BP - NO macroscopic haematuria - Normal renal function - Normal complement levels - NO features to suggest nephritis - Respond to steroids - Histology = "minimal change" usually

Question 202

What is steroid sensitive nephrotic syndrome often precipitated by?

Answer 202

Respiratory infections

Question 203

How do you treat steroid sensitive nephrotic syndrome?

Answer 203

- Standard course of prednisolone for 1st episode = 60mg/m2 OD for 4 weeks then 40mg/m2 on alternate days for 4 weeks - Consider salt and water restriction, diuretics, Pen V, measles/varicella/pnuemonoccoal immunisations - Renal biopsy if unresponsive or atypical features

Question 204

What is the prognosis of steroid sensitive nephrotic syndrome?

Answer 204

``` 1/3 = resolve directly 1/3 = infrequent relapses 1/3 = frequent relapses = steroid dependent ```

Question 205

Give 3 features suggestive of steroid resistant nephrotic syndrome

Answer 205

1. Elevated BP 2. Haematuria 3. May have impaired renal function 4. Features may suggest nephritis 5. Failure to respond to steroids

Question 206

Give 3 causes of steroid resistant nephrotic syndrome

Answer 206

1. Focal segmentaal glomerulosclerosis 2. Membranoproliferative glomerulonephritis 3. Membranous nephropathy

Question 207

Give 2 features of congenital nephrotic syndrome

Answer 207

- Presents in 1st 3 months of life - Mostly recessively inherited - More commonly in consanguineous families - Associated with high mortality --> complications of hypoalbuminaemia

Question 208

Give 3 non glomerular causes of haematuria

Answer 208

1. Infection 2. Trauma to genitalia, urinary tract or kidneys 3. Stone 4. Tumours 5. Sickle cell disease 6. Bleeding disorders 7. Renal vein thrombosis

Question 209

Give 3 glomerular causes of haematuria

Answer 209

1. Actue GN 2. Chronic GN 3. IgA nephropathy 4. Familial nephritis --> Alport syndrome 5. Thin basement membrane syndrome

Question 210

What is nephritis?

Answer 210

Increased glomerular cellularity restricts glomerular blood flow and therefore filtration is decreased

Question 211

What are the main features of nephritis?

Answer 211

1. Macroscopic haematuria 2. Salt and water retention --> HTN, oedema 3. Proteinuria 4. Impaired GFR

Question 212

Give 3 causes of acute nephritis

Answer 212

1. Post infectious 2. Vasculitis --> HSP, SLE, Wegner granulomatosis 3. Anti-glomerular basement membrane disease (good pasture syndrome) = rare

Question 213

How do you treat nephritis?

Answer 213

Treat water and electrolyte balance | Diuretics

Question 214

Name a complication of nephritis

Answer 214

Rapidly progressive glomerulonephritis may occur = irreversible renal failure if left intreated - renal biopsy, immunosuppression, plasma exchange

Question 215

What is acute post streptococcal glomerulonephritis?

Answer 215

- Cause of nephritis - 1-2 weeks after nasopharyngeal or skin infection - Caused by group A beta haemolytic streptococcus

Question 216

What are the features of acute post streptococcal glomerulonephritis?

Answer 216

- Clinical nephritis 10 days post infection - Haematuria - Oedema - Decreased urine output - HTN

Question 217

What investigations would you do for someone with suspected acute post streptococcal glomerulonephritis?

Answer 217

- FBC --> mild normochromic, normocytic anaemia - U&Es --> increased urea and creatinine, (hyperkalaemia, acidosis) - Immunology --> raised ASOT/antiDNAse B titre, low C3, C4 - Throat/other swabs - Urinalysis --> haematuria, proteinuria, microscopy

Question 218

Describe the treatment of acute post streptococcal glomerulonephritis

Answer 218

- Fluid balance --> salt and fluid restriction, diuretics - Correction fo other imbalances --> K+, acidosis - Dialysis - Penicillin --> treatment of strep infection

Question 219

What is Henoch-Schoenlein Purpura?

Answer 219

A systemic vasculitis where there is deposition of IgA complexes

Question 220

What is HSP characterised by?

Answer 220

- Purpuric, raised, palpable red rash, normally in extensors --> butt, back of legs/arms - Arthralgia - Periarticular oedema - Abdominal pain - Glomerulonephritis - HSP nephritis --> IgA deposition, haematuria, proteinuria, nephrotic syndrome, acute nephritis, renal impairment, HTN

Question 221

Who is most likely to get HSP?

Answer 221

- Occurs between 3-10 years old - More common in boys - More common in winter - Often preceded by URTI

Question 222

Give examples of children who are at increased risk of cancer

Answer 222

1. Down's syndrome children --> risk of leukaemia 2. Immunocompromised children --> risk of lymphoma 3. Neurofibromatosis 1

Question 223

Name 3 embryonal tumours

Answer 223

1. Wilm's tumour 2. Neuroblastoma 3. Rhabdomyosarcoma

Question 224

Give 3 possible presentations of paediatric malignant disease

Answer 224

1. Localised mass --> lymphadenopthy, organomegaly, soft tissue/bony mass 2. Problems with disseminated disease --> bone marrow infiltration 3. Problems from localised mass --> airway obstruction, raised ICP, focal neurology

Question 225

An abnormal red reflex in a child may be a sign of what paediatric malignancy?

Answer 225

Retinoblastoma

Question 226

What type of leukaemia is most common in children?

Answer 226

Acute Lymphoblastic Leukaemia (ALL) | - Peak incidence = 2-5 years old

Question 227

What is ALL?

Answer 227

Malignant proliferation of 'pre-B' or T cell lymphoid precursors

Question 228

Give 4 signs of ALL

Answer 228

1. Anaemia 2. Bleeding 3. Bruising 4. Bone/limb pain 5. Fever 6. Infections 7. Fatigue 8. Lymphadenopathy 9. Hepatosplenomegaly

Question 229

What investigations might you do in a child to determine whether they have ALL?

Answer 229

1. Blood film --> blastocytes 2. Serum chemistry --> anaemia, low WCC, low platelets 3. CXR 4. BM aspirate 5. Lumbar puncture

Question 230

Describe the treatment of ALL

Answer 230

- Correct anaemia, low platelets, WBC - Allopurinol --> protect against cell lysis - 5 phase combination chemotherapy (2 years for girls, 3 years for boys) - A stem cell transplant = high risk or relapsed patients

Question 231

What are the 5 phases of chemotherapy in the treatment of ALL?

Answer 231

1. Induction 2. Consolidation 3. Interim maintenance 4. Intensification 5. Maintenance

Question 232

What is AML?

Answer 232

Malignant proliferation of myeloid cell precursors - Presentation = pancytopaenia, lymphadenopathy - Treatment = All-trans retinoic acid followed by 4 courses of intensive myeloablative chemo, BMT in 1st remission

Question 233

Give 5 signs fo CNS malignancy in children?

Answer 233

1. Headache --> early morning, worse on lying down 2. Vomiting --> early morning 3. Papilloedema 4. Visual disturbances 5. Squint 6. Nystagmus 7. Ataxia 8. Behavioural/personality changes 9. Infants = separation of sutures/tense fontanelle, increase HC, developmental delay/regression DEPENDS ON SITE

Question 234

Give 2 signs of a supratentorial tumour

Answer 234

= cortex 1. Seizures 2. Hemiplegia 3. Focal neuro signs

Question 235

Give 2 signs of a midline tumour

Answer 235

1. Bitemporal hemianopia | 2. Pituitary failure --> growth failure, DI, weight gain

Question 236

Give 2 signs of a cerebellar/IVth ventricle tumour

Answer 236

1. Truncal axtia 2. Coordination difficulties 3. Nystagmus

Question 237

Give 2 signs of a brainstem tumour

Answer 237

1. CN defects 2. Pyramidal signs 3. Cerebellar signs 4. Often NO raised ICP

Question 238

Give the 5 types of CNS tumour

Answer 238

1. Pilocytic astrocytoma (40%) --> benign to highly malignant GBM, surgical removal 2. Medulloblastoma (20%) 3. Ependyoma (8%) 4. Pontine glioma (6%) --> survival = 6-12 months 5. Craniopharyngioma (4%)

Question 239

Describe the general treatment for CNS malignancies

Answer 239

1. Surgical resection + VP shunt (reduces risk of coning) 2. Chemotherapy 3. Radiotherapy

Question 240

Give 5 signs that lymphadenopathy may be due to malignancy rather than a benign e.g. infection, AI

Answer 240

1. Enlarging node without infective cause 2. Persistently enlarged 3. Unusual site --> e.g. supra-clavicular 4. B symptoms --> e.g. fever, weight loss 5. Abnormal CXR

Question 241

What is seen on a lymph node biopsy of someone with Hodgkins lymphoma?

Answer 241

Reed Sternberg cells

Question 242

What staging is used in lymphoma?

Answer 242

Ann Arbor - 1 = single site - 2 = more than one site on one side - 3 = on both sides of the diaphragm - 4 = disseminated disease

Question 243

What is the treatment for lymphoma?

Answer 243

- Combination chemotherapy +/- radiation | - High dose therapy and BMT for those who have relapsed

Question 244

Give 5 differentials for abdominal mass in children

Answer 244

1. Hepatoblastoma 2. Wilm's tumour 3. Neuroblastoma 4. Lymphoma/leukaemia 5. Constipation/bowel obstruction 6. Enlarged kidneys --> polycystic

Question 245

What investigations might you do on a child with an abdominal mass?

Answer 245

- USS - CT/MRI - Biopsy

Question 246

What is a neuroblastoma?

Answer 246

Tumours arising from neural crest tissue in the adrenal medulla and sympathetic nervous system - Most common <5 y/o

Question 247

Give 2 signs of a neuroblastoma tumour

Answer 247

1. Abdominal mass --> often cross the midline and envelops major vessels and lymph nodes 2. Pallor 3. Symptoms of metastases --> bone pain, weight loss, limp, hepatomegaly 4. Lymphadenopathy 5. Pancytopenia --> anaemia, infections, bruising

Question 248

What investigations would you do in someone suspected to have a neuroblastoma?

Answer 248

- Urinary catecholamine levels --> raised - Biopsy - Bone marrow biopsy - MIBG scan --> maps metastatic growth

Question 249

Describe the treatment for a neuroblastoma

Answer 249

- Surgery --> localised primary tumour often resectable - Chemotherapy --> before and/or after - Radiotherapy for high risk groups/relapse

Question 250

What is a Wilm's tumour?

Answer 250

Nephroblastoma = malignancy arising from embryonal renal tissue - 80% before 5 y/o

Question 251

Give 3 signs of a Wilm's tumour

Answer 251

1. Abdominal mass --> not likely to cross midline 2. Abdominal pain 3. Anorexia 4. Haematuria 5. Anaemia 6. HTN

Question 252

Describe the treatment for a Wilm's tumour

Answer 252

- Chemotherapy --> before or after surgery - Surgery --> nephrectomy to partial nephrectomy - Radiotherapy --> for residual abdominal or pulmonary disease

Question 253

Describe the aetiology of retinoblastoma

Answer 253

1. Mutations in RB1 (tumour suppressor gene) located on chromosome 13 2. Familial (bilateral disease) 3. Sporadic

Question 254

Give 3 signs of a retinoblastoma

Answer 254

1. Loss of red reflex (white reflex noted) 2. Pain around the eye 3. Poor vision 4. Squint

Question 255

Describe the treatment for retinoblastoma

Answer 255

Laser therapy Chemotherapy and radiotherapy Surgery --> repair or removal

Question 256

How may a malignant bone tumour present?

Answer 256

1. Persistent localised bone pain 2. swelling 3. Pathological fracture

Question 257

Name 2 types of malignant bone tumour

Answer 257

1. Ewings = more common in younger children | 2. Osteogenic sarcoma = more common

Question 258

Describe the treatment of a malignant bone tumour

Answer 258

- Combination chemo - Resection - Radiotherapy --> in Ewings

Question 259

Give 3 short term side effects of chemotherapy

Answer 259

1. N+V 2. Alopecia 3. Immunosuppression --> infection 4. Bone marrow suppression --> anaemia, brushing, infections

Question 260

Give 3 long term effects of chemotherapy

Answer 260

1. Endocrine related --> growth and development 2. Intellectual 3. Fertility problems 4. Psychological issues 5. Cardiac and renal toxicity

Question 261

Where are red blood cells made?

Answer 261

In the bone marrow | - Stimulated by erythropoietin (produced by kidneys)

Question 262

What are the normal levels of haemoglobin in a) Neonates b) 1-12 months c) 1-12 years?

Answer 262

a) Neonates = <14g/dl b) 1-12 months = <10g/dl c) 1-12 years = <11g/dl

Question 263

Give 3 causes of anaemia due to decreased production of RBC

Answer 263

a) Ineffective erythropoiesis 1. Iron/folic acid deficiency 2. Chronic inflammation --> JIA 3. Chronic renal failure b) Red cell aplasia 4. Parvovirus B19 infection 5. Congenital 6. Fanconi anaemia 7. Aplastic anaemia

Question 264

Give 3 causes of anaemia due to increased consumption of RBC

Answer 264

= Haemolysis 1. Red cell membrane disorders --> hereditary spherocytosis 2. Red cell enzyme disorders --> G6PD deficiency 3. Haemoglobinopathies --> thalassaemia, sickle cell 4. Immune --> haemolytic disease of the newborn, AI haemolytic anaemia

Question 265

Give 3 causes of anaemia due to increased loss of RBC

Answer 265

1. Bleeding 2. Burns 3. Splenomegaly

Question 266

Give 3 causes of microcytic anaemia

Answer 266

1. Iron deficiency 2. Thalassaemia 3. Sideroblastic anaemia --> congenital or heavy metal poisoning

Question 267

Give 3 causes of normocytic anaemia

Answer 267

1. Chronic inflammation = anaemia of chronic disease 2. Acute bleed 3. Renal failure 4. Transient erhtyroblastocytopenia of childhood

Question 268

Give 3 causes of macrocytic anaemia

Answer 268

1. B12 deficiency 2. Folate deficiency 3. Hypothyroidism 4. Bone marrow failure

Question 269

What investigations would you do for someone with suspected anaemia?

Answer 269

1. FBC and blood film 2. Reticulocytes 3. TSH 4. B12, folate, ferritin 5. LFTs --> bilirubin 6. U&Es 7. Hb electrophoresis

Question 270

What is the most common cause of anaemia in children?

Answer 270

Iron deficiency = microcytic, hypochromic

Question 271

Give 3 causes of iron deficiency anaemia

Answer 271

1. Poor intake --> particularly those who are breast fed, or high intake of cows milk, picky toddlers 2. Malabsorption 3. Blood loss 4. Increased requirement

Question 272

Give 3 signs of anaemia in children

Answer 272

1. Pallor --> conjunctival, tongue, palmar creases 2. Irritability 3. Lethargy 4. Tachycardia 5. SOB

Question 273

What is the treatment of iron deficiency anaemia?

Answer 273

- Oral ferrous sulphate | - Increase iron in diet --> red meat, liver, kidney, oily fish

Question 274

Describe the inheritance patten of hereditary spherocytosis

Answer 274

Autosomal dominant | - Mutations in genes for protein of red cell membrane (spectrum, ankyrin, band 3)

Question 275

Give 3 possible symptoms of hereditary spherocytosis

Answer 275

1. Jaundice 2. Anaemia 3. Splenomegaly 4. Aplastic crisis 5. Gallstones

Question 276

How can hereditary spherocytosis be treated?

Answer 276

- Oral Folic acid | - Splenectomy

Question 277

Describe the inheritance pattern of G6PD deficiency

Answer 277

X linked recessive red cell enzymopathy

Question 278

How can G6PD deficiency present?

Answer 278

- Neonatal jaundice - Chronic non spherocytic haemolytic anaemia - Intermittent episodes fo intravascular haemolytic - Sporadic/acute haemolysis

Question 279

How can acute haemolysis in G6PD deficiency be triggered?

Answer 279

- Drugs --> antimalarials, quinolones, aspirin - Broad (fava) beans - Infection

Question 280

What might you see on a blood film of someone with G6PD deficiency?

Answer 280

Bite cells and hemighosts

Question 281

How is G6PD deficiency managed?

Answer 281

- Education about acute haemolysis and precipitants to avoid - Transfusions and renal support

Question 282

Describe the inheritance pattern of thalassaemia

Answer 282

Autosomal recessive | - Affects those from Indian subcontinent, Mediterranean and middle east most commonly

Question 283

Give 3 signs of beta thalassaemia major

Answer 283

1. Severe anaemia 2. Jaundice 3. Splenomegaly, hepatomegaly 4. Failure to thrive/growth failure 5. Bossing of skull, maxillary overgrowth

Question 284

Describe the management of beta thalassaemia major

Answer 284

1. Genetic counselling 2. Regular blood transfusions 3. Bone marrow transplant = cure 4. Endocrine supplementation, fertility help, bone health

Question 285

What is a complications beta thalassaemia major?

Answer 285

Iron overload --> cardiac filature, cirrhosis, DM, infertility, growth failure - Treatment = iron chelation = S/C deferoxamine or oral deferasirox

Question 286

What is sickle cell disease?

Answer 286

Variant Hb chain = HbS | - Point mutation on codon 6 of the B globin gene causing glutamine to change to valine

Question 287

Describe the inheritance pattern seen in sickle cell disease

Answer 287

Autosomal recessive

Question 288

What RBC abnormalities occur in sickle cell?

Answer 288

- Fragility - Prone to haemolysis - Occlusion of small vessels - Sickle shaped - Reduced lifespan

Question 289

Name 3 consequences of sick cell disease

Answer 289

1. Anaemia 2. Infection 3. Painful crises 4. Stroke 5. Acute chest syndrome --> pain, hypoxia, fever, +/- CXR signs 6. Splenic sequestration = pooling of blood in spleen 7. Aplastic crisis --> can be precipitated by parvovirus B19

Question 290

What is the affect of sickle cell disease on Hb and reticulocyte count?

Answer 290

- Low Hb | - Raised reticulocyte count

Question 291

Describe the treatment for sick cell disease

Answer 291

- Avoid exposure to cold, dehydration, excessive exercise, stress, hypoxia - Prevention of crises and infection --> daily oral penicillin - Oral folic acid - Hydroxycarbamide (hydroxyurea) --> increased HbF - Transfusions - Stem cell transplant = cure

Question 292

What is aplastic anaemia?

Answer 292

= bone marrow failure | Characterised by reduction or absence of all 3 main lineages in bone marrow leading to peripheral blood pancytopenia

Question 293

How does aplastic anaemia present?

Answer 293

1. Anaemia = low RBC 2. Infection = low WBC (especially neutrophils) 3. Bruising and bleeding = thrombocytopenia

Question 294

Give 3 causes of bone marrow failure syndrome

Answer 294

1. Congenital --> Fanconi's anaemia 2. Acquired aplasia anaemia 3. Bone marrow infiltration --> leukaemia, lymphoma, neuroblastoma

Question 295

What are the 5 main components for homeostasis to work?

Answer 295

1. Platelet --> number and function 2. Coagulation factors 3. Coagulation inhibitors 4. Fibrinolysis 5. Vascular integrity --> initiate and limit coagulation

Question 296

What are the liver dependent clotting factors?

Answer 296

2, 7, 9 and 10

Question 297

Give 3 causes of thrombocytopenia due to decreased production of platelets

Answer 297

1. Congenital thrombocytopenia 2. Infiltration of bone marrow --> leukaemia, metastases, lymphoma 3. Reduced platelet production by bone marrow --> low B12/folate, reduced TPO, medication (methotrexate, chemo), alcohol, infection, AI 4. Dysfunctional production of platelets in BM --> myelodysplasia

Question 298

Give 3 causes of thrombocytopenia due to increased destruction of platelets

Answer 298

1. Autoimmune --> ITP, SLE, primary/secondary 2. Hypersplenism --> portal HTN, splenomegaly 3. Drug related immune destruction --> heparin induced 4. Consumption of platelets --> DIC, TTP, haemolytic uraemic syndrome, congenital heart disease

Question 299

What diagnosis would you suspect in a child with a single figure platelet count but is otherwise well?

Answer 299

``` Immune Thrombocytopenia (ITP) - Normal blood film and clotting but very low platelets ```

Question 300

Why does ITP occur?

Answer 300

Usually due to destruction of circulating platelets by anti platelet IgG autoantibodies

Question 301

What can trigger acute ITP?

Answer 301

Viral infection --> occurs 1-2 weeks after | - Secondary ITP = malignancies (CLL), infection (HIV, hep C)

Question 302

How do you manage ITP?

Answer 302

Majority are self limiting within 6-8 weeks | - Oral prednisolone, IV anti-d or IV IgG occasionally

Question 303

What is disseminated intravascular coagulation (DIC)?

Answer 303

Coagulation pathway activation leading to diffuse fibrin deposition in the microvasculature and consumption of coagulation factors and platelets

Question 304

Give 3 possible causes of DIC

Answer 304

1. Severe sepsis or shock 2. Extensive tissue damage --> trauma/burns 3. Malignancy

Question 305

What would you see in the investigations of someone with DIC?

Answer 305

- Prolonged prothrombin time and APTT - Low platelets - Low fibrinogen - High D dimers

Question 306

How do you treat DIC?

Answer 306

- Treat underlying condition - Platelet transfusion - Fresh frozen plasma --> contains clotting factors - Cryoprecipitate --> contains fibrinogen and some clotting factors

Question 307

Name 2 coagulopathies

Answer 307

1. Haemophilia | 2. Von Willebrand disease

Question 308

Describe the inheritance pattern seen in haemophilia

Answer 308

X linked recessive

Question 309

Haemophilia A is due to a deficiency in which clotting factor?

Answer 309

Factor VIII (8)

Question 310

Haemophilia B is due to a deficiency in which clotting factor?

Answer 310

Factor IX (9)

Question 311

Give 3 signs of haemophilia

Answer 311

1. Easy bruise 2. Haematomas 3. Mouth bleeding 4. Joint bleeds --> prophylaxis needs to be given to prevent

Question 312

Give 2 possible complications fo treatment for haemophilia

Answer 312

1. Inhibitors --> antibodies to factor 8 or 9 so reduce treatment effect 2. Vascular access becomes more difficult

Question 313

Briefly describe the pathophysiology behind Von Willebrand disease

Answer 313

Bleeding disorder due to an abnormality of vWF vWF acts as an adhesive bridge between platelets and the damaged sub-endothelium Without it takes longer for bleeding to stop --> defective platelet plug formation

Question 314

Describe the inheritance pattern seen in vWD

Answer 314

Autosomal dominant

Question 315

Describe the treatment for vWD

Answer 315

No cure - Mild = DDAVO --> causes secretion of factor VIII and vWF into plasma - Severe = plasma derived factor VIII concentrate

Question 316

Give 3 signs of thrombocytopenia

Answer 316

1. Petechial rash 2. Bruising 3. Bleeding

Question 317

What does a low reticulocyte count indicate?

Answer 317

A production problem --> infection, renal disease, drugs, marrow failure/infiltration

Question 318

What does a high reticulocyte count indicate?

Answer 318

A degradation problem --> bleeding, haemolysis

Question 319

Give 5 determinants of growth

Answer 319

1. Parental phenotype and genotype 2. Pregnancy factors 3. Nutrition 4. Psychosocial deprivation 5. Hormones

Question 320

When is growth velocity fastest?

Answer 320

IN utero and in infancy

Question 321

When does growth end?

Answer 321

When the epiphyses fuse

Question 322

What is hypochondroplasia?

Answer 322

A developmental disorder resulting in short limbs

Question 323

What is the main factor driving infant growth?

Answer 323

Nutrition

Question 324

Give 5 differentials for short stature

Answer 324

1. Constitutional delay 2. Slow maturation 3. Delayed puberty 4. Idiopathic 5. Environmental --> psychosocial 6. Nutrition 7. Skeletal disease 8. Physical disease --> coeliac, IBD, CHD 9. Turner syndrome 10. Endocrine pathology

Question 325

What can cause an increased final height?

Answer 325

1. Androgen/oestrogen excess 2. GH excess 3. Marfan's 4. Kleinfelters

Question 326

Name the scale that is used to describe physical development based on external sex characteristics

Answer 326

Tanner scale

Question 327

Define thelarche

Answer 327

Breast development

Question 328

Define adrenarche

Answer 328

Maturation of the adrenal gland --> androgen production --> body odour and mild acne

Question 329

Define pubarche

Answer 329

Growth of pubic hair

Question 330

What is the first sign of puberty in boys?

Answer 330

First ejaculation and testicular size >3ml

Question 331

Define delayed puberty

Answer 331

The absence of secondary sexual characteristics by 14 in females or 16 in males = Hypogonadism

Question 332

What is the most likely cause fo delayed puberty in boys?

Answer 332

Constitutional delay --> runs in the family

Question 333

What must you rule out as a cause of delayed puberty in girls?

Answer 333

Turner syndrome (45XO)

Question 334

What is primary gonadal failure?

Answer 334

Hypergonadotrophic hypogonadism

Question 335

What is the affect of hypergonadotrophic hypogonadism on a) FSH/LH b) Oestrogen/Testosterone?

Answer 335

= High basal and stimulated gonadotrophin levels a) High LH, FSH and GnRH b) Low testosterone/oestrogen

Question 336

Give 4 causes of primary gonadal failure

Answer 336

1. Chromosomal disorders --> turners, Klinefelter's 2. Gonadal dysgenesis 3. Disorders of steroid biosynthesis --> CSH 4. Chemotherapy or gonadal radiotherapy 5. Gonadal infection --> mumps orchitis 6. Gonadal trauma/torsion

Question 337

What is secondary and tertiary hypogonadism?

Answer 337

Hypogonadotrophic hypogonadism

Question 338

What is the affect of hypogonadotrophic hypogonadism on a) FSH/LH b) Oestrogen/Testosterone?

Answer 338

= Low/undetectable basal and stimulated gonadotrophin levels a) low GnRH, LH and FSH b) high testosterone/oestrogen

Question 339

Give 3 causes of secondary/tertiary hypogonadism

Answer 339

1. Kallman's syndrome 2. Congenital hypopituitarism 3. Syndromic associations --> Prader-WIlli 4. Intracranial tumours 5. Cranial irradiation 6. TBI 7. Anorexia, excessive exercise

Question 340

How might you investigate delayed puberty?

Answer 340

- FBC - U&Es - TFTs - LH/FSH - Karyotyping

Question 341

How do you manage delayed puberty?

Answer 341

- Constitutional delay = short course of sex steroid therapy - Permanent gonadotrophin deficiency or gonadal failure = long term sex steroids gradually increasing over 2-3 years --> IM testosterone (boys), oral ethinylestradiol + progesterone (girls)

Question 342

Give 3 potential consequences of delayed puberty

Answer 342

1. Psychological problems 2. Reproduction defects 3. Reduced bone mass

Question 343

What is Turners syndrome?

Answer 343

45 XO | = Hypergonadotrophic hypogonadism

Question 344

Give 5 signs of Turner Syndrome

Answer 344

1. Delayed puberty 2. Short stature 3. Recurrent otits media 4. Webbed neck 5. CV and renal malformations 6. Low posterior hairline 7. Widely spaced nipples 8. Ovarian dysgenesis resulting in infertility

Question 345

How do you treat Turner Syndrome?

Answer 345

- Growth hormone therapy - Oestrogen replacement for development of secondary sexual characteristics at the time of puberty (infertility will persist)

Question 346

What is Klinefelter's Syndrome?

Answer 346

``` 47 XXY Primary hypogonadism (hypergonadotrophic hypogonadism) ```

Question 347

Give 3 signs of Klinefelter Syndrome

Answer 347

1. Azoospermia = infertility, no sperm 2. Gynaecomastia 3. Testicular size <5ml 4. Reduces pubic hai 5. Tall stature

Question 348

What is Kallman syndrome?

Answer 348

There is a congenital deficiency of GnRH meaning the pituitary isn't stimulated to release FSH and LH this leads to secondary gonadal failure

Question 349

What inheritance pattern is seen in Kallman Syndrome?

Answer 349

X linked recessive or dominant

Question 350

75% of people with which syndrome may have anosmia?

Answer 350

Kallman Syndrome

Question 351

Define precocious puberty

Answer 351

The onset of secondary sexual characteristics before 8 (in girls) or 9 (in boys)

Question 352

What must you rule out as a cause of precocious puberty in boys?

Answer 352

Brain tumour

Question 353

What is True precious puberty?

Answer 353

Central precocious puberty = gonadotrophin dependent | - Increase in GnRH

Question 354

Give 3 causes of true precocious puberty

Answer 354

1. Idiopathic (most often girls) 2. Intracranial tumours --> astrocyroma, craniopharyngioma 3. Other CNS lesions --> hydrocephalus, arachnoid cyst, TBI 4. Secondary central PP = early maturation of HPG axis --> CAH

Question 355

What is precocious pseudopuberty?

Answer 355

Peripheral precocious puberty = gonadotrophin independent | - Secondary sexual characteristics but no central activation of gonads

Question 356

Give 2 causes of precocious pseudopuberty

Answer 356

1. Gonadal --> McCune-Albright syndrome, Ovarian tumour, testicular tumour 2. Adrenal --> CAH, tumour 3. HCG secreting tumour 4. Itraogenic --> exogenous sex steroid administration

Question 357

What investigations might you do in someone who present with precocious puberty?

Answer 357

- Examination --> Tanner scale, height/weight, growth records, parents heights, abdominal/testicular/neuro exam - Plasma LH and FSH - Plasma sex hormones --> oestrogen, testosterone - Other serum androgens --> 17-OH progesterone, DHEAS, androstenedione - Urine steroid profile - GnRH test - Imaging = Bone age XR, pelvic US, abdo US, MRI brain

Question 358

How would you treat precocious puberty?

Answer 358

GnRH analogues to suppress pulsatility of GnRH secretion --> SC or IM every 3 months

Question 359

What is Androgen Insensitivity Syndrome?

Answer 359

46XY - Occurs due to defects in the androgen receptor and results in a spectrum of under virilisied phenotypes - Complete = deletion of gene --> completely female phenotype - Partial = wide spectrum of phenotypic expression

Question 360

Name the test that can pick up congenital hypothyroidism

Answer 360

Guthrie test (heel prick, newborn screening blood spot)

Question 361

What is the most common cause of hypothyroidism a) Worldwide b) In the UK c) In a consanguineous pedigree?

Answer 361

a) Worldwide = Iodine deficiency b) In the UK = Thyroid dysgenesis (maldescent of thyroid, athyrosis) c) In a consanguineous pedigree = Dyshormonogenesis (inborn error of thyroid hormone synthesis)

Question 362

Give 4 signs of congenital hypothyroidism

Answer 362

1. Feeding probelms and failure to thrive 2. Prolonged jaundice 3. Cold and mottled skin 4. Hoarse cry 5. Hypotonia 6. Constipation 7. Low temperature 8. Large fontanelle, broadened and flattened nose 9. Goitre

Question 363

What is the treatment for a baby with congenital hypothyroidism?

Answer 363

Levothyroxine starting at 2-3 weeks of age | - Neonatal dose = 10-15 mag/kg OD

Question 364

What is a complication of untreated congenital hypothyroidism?

Answer 364

Congenital iodine deficiency syndrome/cretinism = severely stunted physical and mental growth - Delayed mental development, learning difficulties, poor coordination

Question 365

A 10-day-old baby presents with collapse and shock. They have low sodium, high potassium and are found to be in metabolic acidosis, what is the most likely diagnosis?

Answer 365

Congenital adrenal hyperplasia | - The baby is having a salt-losing adrenal crisis

Question 366

What is Congenital Adrenal Hyperplasia (CAH)?

Answer 366

An autosomal recessive disease that is more common cause of insufficient cortisol and mineralocorticoid secretion - Most often due to absence of enzyme 21-hydroxylase

Question 367

How may CAH presetn?

Answer 367

1. Mineralocorticoid deficiency = renal salt wasting --> severe dehydration 2. Glucocorticoid deficiency = adrenal crisis with hypoglycaemia and hypotension in setting of an intercurrent illness or physiological stress 3. Females = ambiguous genitalia --> clitomegaly, degree of labia fusion, position of urethra/urogenital sinus 4. Males = salt loss (80%) or tall stature and precocious puberty (20%)

Question 368

How can CAH be diagnosed biochemically?

Answer 368

- Raised levels of 17-alpha-hydroxy-progesterone - Low serum Na - High serum K+ - Metabolic acidosis - Hypoglycaemia

Question 369

Why might someone with CAH have low sodium and high potassium?

Answer 369

They are unable to produce aldosterone

Question 370

Describe the treatment for CAH

Answer 370

- Immediate = Fluid, IV/IM hydrocortisone, glucose - Lifelong hydrocortisone and fludrocortisone - Monitor growth, skeletal maturity, plasma androgens and 17a hydroxyprogesterone

Question 371

What is a complication of CAH?

Answer 371

Adrenal crisis = urgent treatment with IV saline, glucose and hydrocortisone

Question 372

What would you expect the fasting plasma glucose level to be in the following a) Diabetes b) Impaired glucose tolerance c) Euglycaemia?

Answer 372

a) Diabetes = >7 mmol/l b) Impaired glucose tolerance = 5.7-7 mmol/l c) Euglycaemia = 3.5-5.6 mmol/l

Question 373

What would you expect the post OGTT plasma glucose level to be in the following a) Diabetes b) Impaired glucose tolerance?

Answer 373

a) Diabetes = >11.1 mmol/l | b) Impaired glucose tolerance = 7.8-11 mmol/l

Question 374

What would you expect the HbA1c (%) to be in the following a) Diabetes b) Impaired glucose tolerance c) Euglycaemia?

Answer 374

a) Diabetes = >6.5% b) Impaired glucose tolerance = 5.7-6.4% c) Euglycaemia = 4-5.6%

Question 375

Name 2 genes that have been identified inc asking T1DM

Answer 375

1. HLADR3 | 2. HLADR4

Question 376

Briefly describe the pathophysiology of T1DM

Answer 376

AI destruction of pancreatic B cells so absence of insulin --> Insulin deficiency --> glycogenolysis, gluconeogenesis, ketogenesis --> increased ketone and glucose production --> vomiting, osmotic diuresis, acidosis --> fluid and electrolyte depletion --> cellular dysfunction, cerebral oedema, shock

Question 377

Give 3 symptoms fo T1DM

Answer 377

1. Wight loss 2. Polydipsia 3. Polyuria 4. Lethargic 5. Skin infections

Question 378

How do you treat T1DM?

Answer 378

Insulin therapy Carb counting Lifestyle advice --> sex, drugs, alcohol, contraception, driving Diet --> reduced refined carbs, no more than 30% fat Education --> exercise, sick day rules, blood glucose monitoring, hypoglycaemia recognition

Question 379

What do you need to monitor in DM?

Answer 379

1. Hba1c 2. Retinopathy --> eye test every year 3. Neuropathy --> foot checks 4. Nephrophathy --> bloods, urine 5. BP checks 6. Annual bloods

Question 380

Briefly describe the pathophysiology behind DKA

Answer 380

No insulin --> lipolysis --> FFA's --> oxidised in liver -> ketone bodies --> ketoacidosis Glucose is not metabolised or stored so the body goes into a 'starvation state'

Question 381

What is DKA characterised by?

Answer 381

1. Acidosis, pH <7.3 2. Raised ketones >3 3. Hyperglycaemia >11.1 random

Question 382

Give 3 symptoms of DKA

Answer 382

1. Unwell 2. Lethargic 3. Nausea 4. Vomiting 5. Abdominal pain 6. Dehydration

Question 383

Give 3 signs of DKA

Answer 383

1. Kassmaul breathing 2. Subcostal and intercostal recessions 3. tachycardia 4. Hypotension 5. Dry mucous membranes 6. Ketotic breath

Question 384

Describe the management of DKA

Answer 384

1. Fluid resuscitation = 0.9% NACl --> corrected gradually over 48 hours 2. Insulin infusion 3. Potassium replacement and cardiac monitoring

Question 385

Why is important to correct fluid balance slowing over 48 hours in someone with DKA?

Answer 385

Rapid fluid resuscitation can lead to cerebral oedema

Question 386

Give 3 possible complications of DKA

Answer 386

1. Cerebral oedema 2. Shock 3. Hypokalaemia --> arrhythmias 4. Aspiration 5. Thrombus

Question 387

Define hypoglycaemia

Answer 387

Someone with blood glucose <4 mmol/L in diabetes

Question 388

Give 5 hypoglycaemic symptoms

Answer 388

``` Autonomic symptoms 1. Irritable 2. Hungry 3. Nauseous 4. Shakey 5. Anxious 6. Sweaty 7. Pallor Neuroglycopenic symptoms 8. Dizzy 9. Headache 10. Confused 11. Slurred speech 12. Hearing or visual problems ```

Question 389

Define a Tic

Answer 389

Sudden twitches, movements or sounds that people do repeatedly, they are involuntary

Question 390

Define a stereotypies

Answer 390

Repetitive rhythmic movements or sounds --> body rocking, head nodding, finger tapping, arm/hand flapping, waving, pacing

Question 391

Give 3 red flag symptoms of a headache being a SOL

Answer 391

1. Worse lying down, with cough or straining 2. Wakes up child 3. Associated confusion and/or morning or persistent N+V 4. Change in personality., behaviour or educational performance

Question 392

Give 3 red flag physical signs that may suggest a SOL

Answer 392

1. Growth failure 2. Visual field defects 3. Squint 4. Cranial nerve abnormality 5. Torticollis 6. Abnormal coordination or gait 7. Papilloedema 8. Bradycardia 9. Cranial bruits

Question 393

Give 2 examples of primary headaches

Answer 393

1. Migraine 2. Tension 3. Cluster 4. Other --> cough, extertional headache

Question 394

Give 2 examples of a secondary headaches

Answer 394

1. Raised ICP 2. Space occupying lesion 3. Trauma 4. Infection

Question 395

Briefly describe tension headaches

Answer 395

Symmetrical, gradual onset, tightness/band/pressure

Question 396

Briefly describe migraine without aura

Answer 396

1-72 hours, bilateral, pulsatile over temporal or frontal area, GI disturbances, photophobia or phonophobia

Question 397

Briefly describe migraine with aura

Answer 397

Migraine preceded by visual, sensory or motor aura

Question 398

What is cerebral palsy?

Answer 398

An umbrella term covering a group of motor impairments syndrome secondary to non-progressive lesions or anomalies in the brain arising in the early stages of development - Chronic disorder of movement and/or posture that present early (before 2y/o) and continues throughout life

Question 399

Give 3 causes of cerebral palsy

Answer 399

1. 80% antenatal --> hypoxia, congenital infection, haemorrhage, ischaemia, genetic syndrome/structural maldevelopment 2. 10% peri-natal --> hypoxia, infection, haemorrhage 3. 10% postnatal --> hypoxia, infection (meningitis, encephalitis), haemorrhage, encephalopathy, trauma, hydrocephalus, hyperbilirubinaemia

Question 400

How does cerebral palsy often present?

Answer 400

- Abnormal limb/trunk posture and tone in infancy with delayed motor milestone - Feeding difficulties - Abnormal gait once walking is achieved - Asymmetric hand function before 12 months - Primitive reflexes may persist

Question 401

Cerebral palsy is characterised according to neurological features, what are the categories?

Answer 401

1. Spastic --> hemiplegic, quadriplegic, diplegia 2. Dyskinetic 3. Ataxic

Question 402

Briefly describe spastic cerebral palsy

Answer 402

Damage to UMN pathway --> increased spasticity with associated brisk tendon reflexes and extensor plantar responses - Clasp knife phenomenon - Ankle plantar flexion - Limited adduction and internal rotation of hip flexion - Wrist flexed and pronated - Shoulder adducted - Bulbar muscle may be spastic (dysphagia, dribbling)

Question 403

Briefly describe the 3 types of spastic cerebral palsy

Answer 403

1. Hemiplegic --> Unilateral involvement of arm and leg, arm more affected than leg with face spared (causes = neonatal stroke) 2. Quadriplegic --> all 4 limbs affected, trunk involved, poor head control, low central tone, seizures, microcephaly, intellectual impairment (causes = hypoxic-ischaemic encephalopathy) 3. Diplegia --> all 4 limbs, legs affected more than arms (cause = periventricular brain damage with preterm birth)

Question 404

Briefly describe dyskinetic cerebral palsy

Answer 404

- Muscle tone variable and primitive motor reflex patterns predominate, chorea, athetosis, dystonia - Intellect may be relatively unimpaired - Present with floppiness, poor trunk control and delayed motor development in infancy - Causes = hypoxic-ischaemic encephalopathy, hyperbilirubinaemia (due to rhesus disease of newborn)

Question 405

Briefly describe ataxic cerebral palsy

Answer 405

- Early trunk and limb hypotonia, poor balance, delayed motor development - Incoordinate movement, intention tremor and taxis gait = later signs - Causes = genetic, acquired brain injury

Question 406

Why might the clinical signs of cerebral plays change over time?

Answer 406

As brain matures but the underlying aetiology is not progressive

Question 407

How might you investigate a child who is presenting with signs of cerebral palsy?

Answer 407

1. US scan and MRI brain 2. Genetic testing 3. TORCH screen 4. Metabolic tests 5. Neurophysiological tests 6. Histopathology

Question 408

What drug can be used to treat hypertonia in children with cerebral palsy?

Answer 408

Botox for muscle spasticity

Question 409

Describe the support that is offered to someone with cerebral palsy

Answer 409

1. Physiotherapists --> mobility and hand function 2. SALT --> communication 3. Feeding support 4. Sleeping support Holistic, child focussed and with an MDT approach

Question 410

Define seizure

Answer 410

Sudden disturbance of neurological function caused be an abnormal or excessive neuronal discharge - Epileptic or non-epileptic

Question 411

Define epileptic seizure

Answer 411

Excessive, unsynchronised neuronal discharges in the brain cause paroxysmal changes in behaviour, sensation or cognitive processes

Question 412

Give 3 possible causes of epileptic seizures

Answer 412

1. Idiopathic (70-80%) 2. Secondary --> cerebral dysgeneis/malformation, vascular occlusion, damage (congenital infection, hypoxic-ischaemic encephalopathy, haemorrhage) 3. Cerebral tumour 4. Neurodegenerative disorder 5. Neurocutaneous syndrome 6. Congenital --> NF, TORCH, tuberous sclerosis

Question 413

Give 3 possible causes of non epileptic seizures

Answer 413

1. Febrile seizures 2. Metabolic --> hypoglycaemia, hypocalcaemia, hypomagnzsia, hypo/hypernatraemia 3. Head trauma 4. Meningitis/encephalitis 5. Poisons/toxins

Question 414

What are febrile seizures?

Answer 414

An epileptic seizures accompanied by a fever in the absence of intracranial infection due to bacterial meningitis or viral encephalitis = Brief generalised tonic-clonic seizure

Question 415

What is the prognosis if febrile seizures?

Answer 415

- 30-40% further febrile seizures - Simple = no effect on intellectual performance or risk of developing epilepsy - Complex (focal, prolonged, repeated in same illness) = 4-12% risk of subsequent epilepsy

Question 416

What is a neonatal seizure?

Answer 416

Typically repetitive, rhythmic (clonic) movements of the limbs which persist despite restraint and are often accompanied by eye movements and changes in respiration

Question 417

Name 3 possible causes of neonatal seizures

Answer 417

1. Hypoxic-ischaemic encephalopathy 2. Cerebral infarction 3. Septicaemia/meningitis 4. Metabolic --> hypoglycaemia, hypo/hypernatraemia 5. Intracranial haemorrhage 6. Cerebral malformations 7. Drug withdrawal 8. Congenitaal infection 9. Kernicterus

Question 418

How long do epileptic seizures tend to last for?

Answer 418

30-120 seconds

Question 419

Give 3 signs of epileptic seizures

Answer 419

1. Movement 2. Tongue biting 3. Head turning 4. Muscle pain

Question 420

What is a generalised seizure?

Answer 420

Discharge arises from both hemispheres | LOC, no warning, symmetrical, bilateral seizure discharge on EEG or varying asymmetry

Question 421

Give 3 examples of generalised seizures

Answer 421

1. Absence 2. Myoclonic 3. Tonic 4. Generalised tonic clonic 5. Atonic

Question 422

What are absence seizures?

Answer 422

Seizures where there is a transient loss of consciousness with an abrupt onset and termination Momentary unresponsiveness with motor arrest Last <30s, usually <10s Developmentally normal but can interfere with school

Question 423

What can absence seizures evolve into?

Answer 423

Juvenile myoclonic epilepsy (JME)

Question 424

How might you investigates suspected absence seizures?

Answer 424

EEG = 3Hz spike and wave | Observe episode = hyperventilation, ask child to blow on windmill

Question 425

What medications can be given to treat absence seizures?

Answer 425

Ethosuxamide or Sodium Valporate --> Lamotrigine

Question 426

What would you expect to see in a myoclonic seizure?

Answer 426

Isolated muscle jerking | - Brief, often repetitive, jerking movements of the limbs, neck or trunk

Question 427

What would you expect to see in a tonic seizure?

Answer 427

Generalised increase in tone

Question 428

What would you expect to see in a atonic seizure?

Answer 428

Transient sudden loss of muscle tone

Question 429

What would you expect to see in a generalised tonic-clonic seizure?

Answer 429

Sudden onset rigid phase followed by a convulsion in which the muscle jerk rhythmically - Other symptoms = LOC, tongue biting, urine incontinence

Question 430

What AED is offered to those suffering from generalised seizures?

Answer 430

Sodium valproate --> lamotrigine --> carbamazepine

Question 431

What is a focal seizure?

Answer 431

Seizure that arises from one part of 1 hemisphere +/- LOC +/- aura

Question 432

What might you see in a frontal focal seizure?

Answer 432

Involves motor and premotor cortex - Clonic movements --> Jacksonian March, Todds palsy - Asymmetrical tonic seizures - Atonic seizures

Question 433

What might you see in a temporal focal seizure?

Answer 433

= most common - Aura/warning signs = smell, taste abnormalities, distortions of shape/sounds - Automatism = lip smacking, chewing, fumbling - Deja vu, jamais vu - Emotional disturbance --> terror, panic, anger, elation

Question 434

What might you see in a occipital focal seizure?

Answer 434

Visual phenomena --> spot, line, flashes

Question 435

What might you see in a parietal focal seizure?

Answer 435

Sensory disturbances --> tingling, numbness

Question 436

What AED is offered to those suffering from focal seizures?

Answer 436

Carbamazepine --> lamotrigine --> sodium valproate

Question 437

What are the signs of juvenile myoclonic epilepsy?

Answer 437

Clumsiness (myoclonic seizures) and GTCS that occur shortly after waking and are often provoked by sleep deprivation - Onset in 2nd decade

Question 438

What is West syndrome?

Answer 438

Infantile spasms - 4 to 6 months - Violent flexor spasm of head, trunk and limbs by extension of the arms - Lasts 1-2 s, often multiple bursts of 20-30s, often on waking - EEG = hypsarrhythmia - Treatment = vigabatrin or corticosteroids - Prognosis = most lose skills, develop learning disability or epilepsy

Question 439

What investigations might you do in someone presenting with seizures?

Answer 439

1. Eye witness accounts/viedo 2. ECG 3. EEG 4. MRI or CT --> identify possible causes 5. Functional --> PET, SPECT

Question 440

Why must you do an ECG in those suffering from seizures?

Answer 440

To check for arrhythmia as the causes --> long QT syndrome

Question 441

What surgery can be done to treat epilepsy?

Answer 441

- Vagal nerve stimulation - Resection fo affected area - Hemispherectomy

Question 442

Give 2 possible side effects of Ethosuximide

Answer 442

- Drowsiness - N+V - Rash - Headaches

Question 443

Give 2 possible side effects of Sodium Valproate

Answer 443

1. Appetite increases --> increase in weight 2. Liver failure --> monitor LFTs over 1st 6months 3. Pancreatitis 4. Reversible hair loss 5. Oedema 6. Ataxia 7. Teratogenicity, Tremor, Thrombocytopaenia 8. Encephalopathy --> due to increased ammonia

Question 444

Give 2 possible side effects of Lamotrigine

Answer 444

1. Skin rash 2. Diplopia, blurred vision 3. Levels affected by enzyme inhibitors/inducers

Question 445

Give 2 possible side effects of Carbamazepine

Answer 445

1. Rash 2. Neutropenia 3. Hyponatraemia 4. Ataxia 5. Liver enzyme induction

Question 446

What is status epilepticus?

Answer 446

Seizure lasting 5 minutes or repeated seizures for 30 minutes without recovery of consciousness

Question 447

Describe the treatment for status epilepticus

Answer 447

- ABCDE - 5 mins: Benzodiazepine e.g. IV lorazepam - 10mins later: Further benzodiazepine - Prepare PR paraldehyde - Prepare phenytoin - Call for senior support - 10mins later: IV phenytoin

Question 448

How long do non-epileptic seizures tend to last for?

Answer 448

1-20 minutes | - Very pale --> falls to floor --> hypoxia may induce brief generalised tonic-clonic seizure --> rapid recovery

Question 449

Give 3 signs of non-epileptic seizures

Answer 449

1. Eyes closed 2. Talking/crying 3. Pelvic thrusting

Question 450

Name 3 conditions that are commonly diagnosed as being epilepsy?

Answer 450

1. Sandifer syndrome 2. Bengin neonatal slöe myoclonus 3. Syncope

Question 451

What is syncope?

Answer 451

Insufficient blood or O2 supply to the brain causes paroxysmal changes in behaviour, sensation and cognitive processes - Clonic movements can occur

Question 452

What non neurological disease is Sandifer Syndrome associated with?

Answer 452

GORD | - Patients present with GORD and a characteristic neck movement disorder

Question 453

Name 3 possible causes of Paroxysmal disorders

Answer 453

1. Breath holding attack = Cries --> holds breath --> goes blue --> goes limp --> sometimes LOC --> rapid spontaneous recovery 2. reflex anoxic (non-epileptic) seizures 3. Syncope 4. Migraine 5. Benign paroxysmal vertigo 6. Cardiac arrhythmia 7. Pseudoseizures 8. Tics

Question 454

What are the 2 main types of Strabismus?

Answer 454

1. Manifest = esotropia, exotropia, hypertropia, hypotropia | 2. Latent = esophoria, exophoria, hyperphoria, hypophoria

Question 455

Name and briefly describe the types of manifest strabismus?

Answer 455

1. Esotropia/convergent = one eye straight ahead, one eye turned inwards towards nose 2. Exotropia/divergent = one eye straight ahead, one eye turning outwards 3. Hypertropia = deviating eye is pointing upwards 4. Hypotropia = deviating eye is pointing downwards

Question 456

Name and briefly describe the types of latent strabismus?

Answer 456

When eyes are straight when both eyes are open, but a deviation of the visual axes can be elicited when each eye is covered 1. Esophoria = tendency for eye to turn inwards under the cover 2. Exophoria = tendency for eye to turn outwards under the cover 3. Hyperphoria = tendency for eye to deviate upwards under the cover 4. Hypophoria = tendency for eye to deviate downwards under the cover

Question 457

Describe the aetiology of strabismus

Answer 457

1. Multifactorial --> hereditary and refractive error (need glasses) 2. Unknown cause 3. Secondary to loss o vision 4. Cerebral palsy 5. Febrile illness can trigger it 6. Primary neurological aetiology (SOL) = rare --> brain tumour, stroke

Question 458

What is more concerning a paralytic squint or a non paralytic squint?

Answer 458

Paralytic squints = SOL so never correct - Non paralytic = malalignment usually, can be corrected

Question 459

Name 2 ways in which strabismus can be investigates?

Answer 459

1. Cover test = will determine presence and direction --> Eye that is covered is the one that turns and then when cover removed eyes swap 2. Corneal reflections --> Shinning pen torch into eye to see corneal reflections = reflections in different positions in the 2 eyes - It is important to assess visual acuity too

Question 460

What type of squint may require neurological-imaging?

Answer 460

Paralytic squints --> as may be due to SOL (Brain tumour)

Question 461

What does the management of strabismus aim to achieve?

Answer 461

1. To restore comfortable binocular single vision 2. To achieve/restore good alignment of the 2 eyes 3. To eliminate diplopia

Question 462

Give 3 conservative management techniques for strabismus

Answer 462

1. Glasses 2. Prism for diplopia 3. Orthoptic exercises 4. Eye patch --> over the good eye

Question 463

Give 2 other management techniques for strabismus

Answer 463

1. Surgery | 2. Botox

Question 464

Define ambylopia

Answer 464

Defective visual acuity which persists after correction of the refractive error and removal of any pathology

Question 465

Describe the treatment for ambylopia

Answer 465

1. Refractive adaptation = wear appropriate glasses for 16 weeks 2. Occlusion of better seeing eye 3. Atropine drops in better eye

Question 466

Describe the epidemiology of ADHD

Answer 466

5% school aged children | M:F = 4:1 (1:1 into adulthood)

Question 467

Describe the aetiology of ADHD

Answer 467

- Genetic and environmental - Neuroanatomical and neurochemical factors too - CNS insults --> Fetal alcohol syndrome, premature, NF, LBW

Question 468

What are the 3 core behaviours of ADHD?

Answer 468

1. Hyperactivity 2. Inattention 3. Impulsivity

Question 469

Give 3 signs of hyperactivity in ADHD

Answer 469

1. Squirms and fidgets 2. Cannot remain seated 3. Talks excessively 4. Noisy 5. Girls = often small things --> giggling, hair twisting, talk too much, overreacting

Question 470

Give 3 signs of inattention in ADHD

Answer 470

1. Easily distracted 2. Not listening 3. Mind wandering 4. Struggling at school 5. Forgetful 6. Organisational problems 7. Difficulty sustaining attention 8. Loses important items 9. Makes careless mistakes

Question 471

Give 3 signs of impulsivity in ADHD

Answer 471

1. Blurts out answers 2. Difficulty waiting turns 3. Interrupts 4. When older = alcohol, drugs, car accidents, pregnancy

Question 472

What is the diagnostic criteria for ADHD?

Answer 472

6/9 inattentive symptoms and 6/9 hyperactivity/impulsivity symptoms - Present before 12 years and occur in >1 place and in a primary setting - Clear evidence that symptoms interfere with social/academic function

Question 473

How does ADHD typically present in primary school age children (6-12 y/o)?

Answer 473

- Hyperactivity most often seen - Distractibility - Motor restlessness - Impulsive and disruptive behaviour - Associated = learning disorders, low self-esteem, repetition of classes

Question 474

How does ADHD typically present in adolescent age children (13-17 y/o)?

Answer 474

- Difficulty in planning and organisation - Persistent inattention - Reduction of motor restlessness - Associated = aggressive, antisocial behaviour, alcohol/drug problems, emotional problems, accidents

Question 475

What tools can be used in order to diagnose ADHD?

Answer 475

1. Clinical interview = any RFs --> pregnancy, birth, development, social Hx 2. ADHD nurse classroom observation 3. Questionnaires (SNAP) 4. Quantitative behavioural analysis

Question 476

Describe the treatment for ADHD

Answer 476

1. Education 2. Parenting programmes and school support 3. Medications --> stimulant medications and non-stimulant medications or combined

Question 477

Give an examples of a stimulant medications

Answer 477

Increase amounts of dopamine released in the brain Methylpenidate - Short acting = Ritalin - Long acting = Delmosart SE = HTN, loss of appetite, psychiatric symptoms

Question 478

Give an examples of a non-stimulant medications

Answer 478

Reduce breakdown of norepinephrine in the brain Atomoxetine (SNRI) Guanfacine

Question 479

Describe the epidemiology of ASD

Answer 479

1% prevalence | Boys > girls

Question 480

Give 4 signs of ASD

Answer 480

1. Communication problems 2. Social interaction difficulties 3. Social imagination difficulties 4. Sensory issues

Question 481

What communication problems might a child with ASD show?

Answer 481

- Lack of desire to communicate at all - Communicating needs only - Disordered or delayed language - Repeats speech/echolalia - No understanding of jokes, very literal - Poor non verbal communication --> gesture, body language

Question 482

What social interaction problems might a child with ASD show?

Answer 482

- Struggles to understand social roles - Often no desire to interact with others - Touches inappropriately, plays alone, poor eye contact, finds it hard to take turns - No understanding of unspoken social rules

Question 483

What social imagination problems might a child with ASD show?

Answer 483

- Struggles with change - Obsessions/rituals - Repetitive with play - Unable to play or write imaginatively

Question 484

Describe the treatment for ASD

Answer 484

- Education and games to encourage social communication - Visual aids and timetables - Parenting workshops and school liaison No medications available

Question 485

When can strawberry marks/haemangiomas be dangerous?

Answer 485

Usually self limiting but beware over eye, in airway - Limit ability of eye to develop - Can use BB to treat them --> only when justified not for cosmetic reasons

Question 486

What condition are cafe au last patches associated with?

Answer 486

Neurofibromatosis

Question 487

What is chicken pox caused by?

Answer 487

Varicella zoster virus

Question 488

How does chicken pox present?

Answer 488

High contagious rash and fever - 200–500 lesions start on head and trunk, progress to peripheries - Appear as crops of papules, vesicles with surrounding erythema and pustules at different times for up to 1 week

Question 489

What is the treatment for chicken pox?

Answer 489

Usually self limiting Treatment = symptomatic Severe or immunocompromised = IV acyclovir

Question 490

Give 2 possible complications of chicken pox

Answer 490

1. Secondary bacterial infection with staph or group A strep --> TTS, necrotising fasciitis 2. Encephalitis 3. Purpura fulminans

Question 491

What happens if chicken pox is reactivated?

Answer 491

Shingles (herpes zoster) = widespread itchy apolar rash in dermatomal distribution

Question 492

How does measles present?

Answer 492

Prodromal CCCK = cough, coryza, conjunctivitis, Koplik spots - Fever, coryza and non itchy maculopapular rash

Question 493

What is Slapped Cheek Syndrome?

Answer 493

= fifth disease, parvovirus B19 - Viral infection common in children 6-10 - Self limiting 1-3 weeks - Usually asymptomatic or erythema infectiosum (viraemic phase followed by distinctive bright red rash to develop on both cheeks), once rash appears infection no longer contagious

Question 494

What is hand, foot and mouth caused by?

Answer 494

Coxsackie or enterovirus 71

Question 495

How does mumps present?

Answer 495

- Incubation period is 15-24 days and infectivity up to 7 days after onset of parotid swelling - Onset = fever, malaise, parotitis - Only one side swollen initially --> bilateral over a few days Usually self limiting

Question 496

Give 2 possible complications of mumps

Answer 496

1. Boys become infertile | 2. Hearing loss

Question 497

Describe Molluscum contagiosum?

Answer 497

= Common pox viral infection - Pink umbilicate papules, presenting as clusters of small round papules - Exacerbated by active eczema or topical steroids - Common in children --> warm, overcrowded environment - Self limiting within a year

Question 498

Describe Impetigo

Answer 498

Causes by staph aureus or strep, localised and very contagious - Superficial rapidly spreading initially clear blisters that develop into straw-coloured dirty looking lesions with yellow crusting - Topical treatment (mupirocin), oral Abx if severe

Question 499

What is scaled skin/red man syndrome

Answer 499

Exotoxin mediated epidermolysis secondary to Staph aureus infection (reaction to staph toxin) - Extensive tender erythema with flaccid superficial blisters/bullae - Erosions and +ve Nikolsky sign - Crusting around eyes, mouth and fever - Treatment = IV anti-staph Abx

Question 500

What is scarlet fever?

Answer 500

Reaction to strep toxin | - Highly contagious bacterial infection affecting children between 2-8 years

Question 501

How does scarlet fever present?

Answer 501

Prodrome = fever, committing, abdominal pain Exanthematous phase = fine red rash (feels like sandpaper) in neck and chest spreading to flexors, strawberry tongue, anterior cervical lymphadenopathy

Question 502

How is scarlet fever treated?

Answer 502

Penicillin V for 10 days

Question 503

3-year-old, 7-day history of high fevers. Now developed red eyes, a rash, sore mouth and throat. Miserable and unwell with a diffuse maculopapular rash on his torso. He has bilateral injected conjunctiva, red cracked lips and a strawberry tongue. 3x2cm cervical swelling and swollen reddened palms. Give 3 differentials

Answer 503

1. Kawasaki disease 2. Scarlet fever 3. Shingles/chickenpox 4. Measles - ask about immunisation history

Question 504

What is Kawasaki disease?

Answer 504

Multi-system disease characterised by systemic vasculitis

Question 505

What is the diagnostic criteria for Kawasaki disease?

Answer 505

``` Fever for >5 days AND not explained by another disease AND 4/5 of the following: - Conjunctivitis (bilateral) - Cracked lips/strawberry tongue - Cervical lymphadenopathy - Rash - Swollen and red extremities ```

Question 506

What mighty see on the blood results in a patient with Kawasaki disease?

Answer 506

High CRP, WCC and ESR | High platelet count

Question 507

Why must you always do a Echo when a patient presents with Kawasaki disease?

Answer 507

Potential complication = coronary artery aneurysm | - Need ECHO at presentation, 2 weeks and 6 weeks

Question 508

Describe the treatment for Kawasaki disease

Answer 508

IV immunoglobulin | High dose Aspirin (1st 14 days) then low dose (until 6 week ECHO)

Question 509

Why do you give high dose aspirin to children with Kawasaki disease?

Answer 509

To prevent thrombosis | - These children have thrombocytosis so are at risk of thrombosis

Question 510

What is choanal atresia?

Answer 510

Failure of nose to canalise Presentation = cyclical going blue --> crying pink, stop crying blue Treatment - dilation +/- stent insertion

Question 511

Name 2 congenital problems with the throat

Answer 511

1. Laryngeal atresia | 2. Laryngomalcia

Question 512

How does laryngomalacia present?

Answer 512

- Normal voice, stridor worse on feeing and exertion - Worse when supine - Failure to thrive - Increased work of breathing

Question 513

What are the criteria for considering a tonsillectomy?

Answer 513

1. >7 episodes of acute tonsillitis in a year | 2. Obstructive sleep apnoea or sleep deprived breathing

Question 514

Give 3 potential consequences of hearing loss?

Answer 514

1. Speech and language delay 2. Social problems --> behaviour issues 3. Academic underachievement 4. Social isolation

Question 515

How does hearing loss in children often present?

Answer 515

1. Parental concern 2. Speech, behavioural or educational problems 3. Incidentally on screening

Question 516

What are the 3 types of hearing loss?

Answer 516

1. Conductive hearing loss 2. Sensorineural hearing loss 3. Mixed

Question 517

Where does conductive hearing loss stem from?

Answer 517

From abnormalities of the ear canal or the middle ear

Question 518

Give 3 causes of conductive hearing loss

Answer 518

1. Otitis media 2. Glue ear 3. Ear wax 4. Perforated ear drum 5. Eustachian tube dysfunction --> Downs, cleft palate

Question 519

Describe the management of conductive hearing loss

Answer 519

- Wait = most resolve on their own - Grommet insertion - Temporary hearing aid - Permanent conductive losses = bone anchored hearing aid can be fitted

Question 520

Where does sensorineural hearing loss stem from?

Answer 520

Caused by lesion in cochlea or auditory nerve (inner ear) and is usually present at birth

Question 521

Give 3 risk factors for sensorineural hearing loss

Answer 521

1. Family history 2. SCBU 3. Consanguinity

Question 522

Give 3 possible causes of sensorineural hearing loss

Answer 522

1. Genetic 2. Antenatal and perinatal --> congenital infection, preterm 3. Postnatal --> meningitis, encephalitis, head injury, drugs, neurodegenerative disorders

Question 523

Describe the management of sensorineural hearing loss

Answer 523

- Usually hearing aids | - Cochlear implants

Question 524

How would you manage mixed hearing loss?

Answer 524

- Address conductive problem first | - Hearing aids

Question 525

When is hearing tested in children?

Answer 525

1. Newborn hearing screen 2. School entry hearing rest 3. Long term monitoring done in high risk groups --> CF, CMV, Downs, cleft, head trauma

Question 526

Is the newborn hearing screen an objective or subjective test?

Answer 526

Objective = response or no response | - If concerns --> follow up with evoked response audiometry

Question 527

What are the 3 aims of hearing testing in children?

Answer 527

1. Measuring hearing threshold (dB) 2. To be frequency specific (Hz) 3. Obtain single ear information if possible

Question 528

Name 4 types of subjective hearing testing

Answer 528

1. Behavioural observational audiometry 2. Distraction testing 3. Visual reinforcement audiometry 4. Performance testing and play audiometry

Question 529

What is otitis media?

Answer 529

Infection of the inner ear

Question 530

Name 2 organisms that can cause acute otitis media

Answer 530

1. Strep pneumoniae | 2. H. influenzae

Question 531

Give 3 symptoms of acute otitis media

Answer 531

1. Pain 2. Fever 3. Generally unwell 4. Otorrhoea

Question 532

What would you see on examination of someone with otitis media?

Answer 532

Red, bulging tympanic membrane | Loss of normal high reflex

Question 533

Describe the treatment for acute otitis media

Answer 533

- Watch and what - Analgesia - If recurrent = board spec Abx - Consider grommet

Question 534

Give 2 potential complications of acute otitis media

Answer 534

1. Extracranial = mastoiditis, TM perforation | 2. Intracranial = meningitis, abscess

Question 535

What is glue ear?

Answer 535

Otitis media + effusion | - Often asymptomatic except hearing loss

Question 536

What causes glue ear?

Answer 536

Infection | - 45% follow acute OM

Question 537

How do you manage glue ear?

Answer 537

- Conservative - Grommets - Hearing aids --> alternative to surgical intervention - Surgical = if hearing loss 25-30 dB on 2 occasions 2/12 apart

Question 538

When might a grommet be indicated?

Answer 538

1. Recurrent acute OM 2. Chronic glue ear 3. Eustachian tube dysfunction

Question 539

What is cholesteatoma?

Answer 539

Erosive condition affection middle ear and mastoid - Can lead to life threatening intracranial infection - Offensive discharge, conductive hearing loss, vertigo, facial nerve palsy (rare) - Treatment = Surgery and Abx

Question 540

Define vomiting

Answer 540

Physical act that results in a forceful expulsion of gastric contents (aided by a sustained contraction of the abdominal muscles and the diaphragm at a time when the cardia of the stomach is raised, and the pylorus is contracted)

Question 541

Define regurgitation

Answer 541

Effortless expulsion of gastric contents (healthy infants and older children who eat in excess)

Question 542

Define rumination

Answer 542

Frequent regurgitation of ingested food (largely behavioural)

Question 543

Define posseting

Answer 543

Small volume vomits during or between feeds in otherwise well child

Question 544

Give 5 differentials for vomiting

Answer 544

1. GORD 2. Infection --> gastroenteritis 3. Food allergy/intolerance 4. Intestinal obstruction 5. Appendicitis 6. Coeliac disease 7. Over feeding --> common in bottle fed infants 8. Necrotising enterocolitis 9. Malrotation - biliary vomit 10. DKA

Question 545

Give 3 causes of intestinal obstruction that can cause vomiting

Answer 545

1. Pyloric stenosis 2. Duodenal atresia 3. Intussusception 4. Hirschsprung's

Question 546

Give 3 important neonatal causes of vomiting

Answer 546

1. Malrotation 2. Hirschsprung's disease/meconium ileus/intestinal atresia 3. Necrotising enterocolitis 4. Infection

Question 547

How might malrotation in a neonate present?

Answer 547

Obstruction with bilious vomiting | Abdominal pain and tenderness

Question 548

When does malrotation normally present?

Answer 548

First 1-3 days of life

Question 549

Any presenting with dark green vomiting needs what urgent investigation?

Answer 549

An urgent upper GI contrast study to assess intestinal rotation

Question 550

Give a potential consequence of malrotation

Answer 550

SMA blood supply to small intestine can be compromised --> infarction

Question 551

What is pyloric stenosis?

Answer 551

A disease characterised by hypertrophy of the pyloric muscle causing gastric outlet obstruction

Question 552

When does pyloric stenosis normally present?

Answer 552

Between 2 and 7 weeks (around 4 weeks)

Question 553

Give 3 signs of pyloric stenosis

Answer 553

1. Vomiting --> projectile, milky, straight after feeding 2. Hunger after vomiting 3. Weight loss 4. Visible gastric peristalsis and palpable mass on test feed (often RUQ)

Question 554

What investigations mighty you do in a neonate you suspect to have pyloric stenosis?

Answer 554

1. U+E 2. Blood gas = hypokalaemia, hyponatraemia 3. USS --> hypertrophy to pyloric sphincter

Question 555

What would a blood gas on a neonate with pyloric stenosis show?

Answer 555

Metabolic alkalosis = low K+ and Cl- | Baby has vomited up all HCl and kidneys go into overdrive --> increased K+ secretion

Question 556

How would you treat a neonate with pyloric stenosis?

Answer 556

- IV fluids and correct electrolyte disturbances - Repeat gases to monitor alkalosis - Stop feeding to stop vomiting - Pyloromyotomy once stable

Question 557

What is intussusception?

Answer 557

Where proximal bowel telescopes into a distal segment --> obstruction, inflammation, bloody stools

Question 558

When does intussusception usually present?

Answer 558

6 months of age (3 months to 2 years)

Question 559

Give 3 signs of intussusception

Answer 559

1. Colickly pain --> draws legs up 2. Vomiting --> milky then green 3. Abdominal mass = sausage shaped in RUQ 4. Red currant jelly stool

Question 560

What investigations might you do on a child who you suspect has an intussusception?

Answer 560

- USS = target sign | - Abdominal XR = distended small bowel

Question 561

Describe the treatment for intussusception

Answer 561

1. Air enema (unless peritonitis is present) 2. Analgesia 3. IV fluids if shocked 4. Surgery

Question 562

What is duodenal atresia?

Answer 562

A congenital absence or complete closure of the duodenum | Causes intestinal obstruction in neonates

Question 563

On an abdominal XR on a neonate duodenal atresia what would you expect to see?

Answer 563

A double bubble sign

Question 564

What syndrome is associated with duodenal atresia?

Answer 564

20-40% have Down's syndrome

Question 565

Give 5 causes of bilious vomiting in children

Answer 565

1. Volvulus 2. Hirschsprung's 3. Necrotising enterocolitis 4. Intussusception 5. Bowel obstruction 6. Strangulated hernia 7. Adhesions

Question 566

Give 4 important causes of vomiting in infants

Answer 566

1. GORD 2. Food intolerance 3. Pyloric stenosis 4. Intussusception 5. Strangulated hernia 6. Raised ICP 7. Infection

Question 567

Give 4 important causes of vomiting in older children

Answer 567

1. Acute appendicitis 2. Strangulated hernia 3. Pancreatitis 4. Cyclical vomiting syndrome 5. DKA 6. Medications, alcohol, illicit drugs 7. Post operative 8. Infection

Question 568

What is GORD (gastro-oesophageal reflux)

Answer 568

Involuntary passage of gastric contents into the oesophagus with or without regurgitation or vomiting Disease = presence of troublesome Sx and/or complications of persistent GOR

Question 569

Give 3 warning signs of GORD

Answer 569

1. Bilious vomiting 2. Persistent forceful vomiting 3. New onset vomiting >6 months 4. Failure to thrive 5. Diarrhoea/constipation 6. Fever 7. HSM

Question 570

Give 3 severe symptoms/signs of GORD

Answer 570

1. Faltering growth 2. Oesophagitis +/- strictures 3. Apnoea 4. Aspiration 5. Iron deficiency anaemia

Question 571

What investigations might you do for someone with suspected GORD?

Answer 571

1. pH --> 24 hours oesophageal monitoring 2. Barium swallow and meal 3. Endoscopy

Question 572

How do you treat GORD?

Answer 572

- Position = 30 degrees head up prone position after feeds - Thicken feeds - Change feeds - Drugs --> antacid, H2 blocker (ranitidine), PPI (omeprazole) - Surgery --> Nissan fundoplication

Question 573

What is an allergy?

Answer 573

Immune mediated response with symptoms and signs that occur within 2 hours of infection of specific food and fully resolve within 24 hours in a sensitised individual

Question 574

What is an intolerance?

Answer 574

Abdominal non immune response to specific foods or additives, primarily characterised by GU symptoms (discomfort, pain, diarrhoea)

Question 575

Give 3 symptoms of cow's milk allergy?

Answer 575

1. Vomiting 2. Abdominal pain 3. Diarrhoea 4. Malabsorption 5. Intestinal bleeding 6. Urticaria and lip swelling

Question 576

What is the treatment for cow's milk allergy?

Answer 576

Specialised formula feeds --> hydrolysed or amino acid feeds

Question 577

A 6-month old breast fed infant developed widespread urticaria immediately after the first formula feed. a) What investigation might you do? b) What is the most likely diagnosis?

Answer 577

a) Skin-prick test for cow's milk | b) IgE mediated cow's milk allergy

Question 578

A 4-month-old infant, formula fed since birth, has loose stools and faltering growth. Skin prick test to cow's milk is negative but elimination of cow's milk results in resolution of symptoms which return on trial re-introduction. What is the most likely diagnosis?

Answer 578

Non-IgE mediated cow's milk allergy

Question 579

Briefly describe the pathophysiology behind lactose intolerance

Answer 579

A deficiency of intestinal lactase prevents the hydrolysis of lactose The osmotic load of the unabsorbed lactose causes secretion of fluid and electrolytes until osmotic equilibrium is reaches --> diarrhoea

Question 580

Give 3 causes of lactose intolerance

Answer 580

1. Primary = deficiency in lactase 2. secondary = small intestine injury from infection, coeliac, IBD 3. Post infective lactose intolerance

Question 581

Give 2 symptoms of lactose intolerance

Answer 581

1. Explosive watery stools 2. ABdominal distension m 3. Flactulance 4. Audible bowel sounds

Question 582

What test can be done to check for lactose intolerance?

Answer 582

Hydrogen breath test

Question 583

What is constipation?

Answer 583

Infrequent passage of stool associated with pain and difficulty, or delay in defecation

Question 584

What is encopresis?

Answer 584

Involuntary faecal soiling or incontinence secondary to chronic constipation = overflowing diarrhoea

Question 585

What is the criteria used to diagnose functional constipation?

Answer 585

ROME III criteria - 2 or fewer defecations per week - At least 1 episode of decal incontinence per week - Retentive posturing or stool retentive - Painful or hard bowel movements - Presence of a large faecal mass in the rectum - Large diameter stools that may obstruct the toilet

Question 586

Give 3 red flag symptoms of constipation

Answer 586

1. Delayed passage of meconium (>24 hours) --> Hirschsprung's 2. Fever, vomiting, bloody diarrhoea 3. Failure to thrive 4. Tight, empty rectum with presence of palpable abdominal faecal mass

Question 587

How do you manage constipation?

Answer 587

- Diet.fluids and exercise - Behavioural advice m - Medications --> softeners, bulking agents, stimulants, enema - Disimpaction regime for overflow diarrhoea

Question 588

Give 4 causes of diarrhoea

Answer 588

1. Overflow diarrhoea due to constipation 2. Chronic no-specific diarrhoea 3. Malabsorption 4. Enteric infection 5. IBD 6. Drug induced 7. Non-intestinal --> hyperthyroid, neuroblastoma

Question 589

Give 2 infection causes of diarrhoea

Answer 589

1. Virus --> adenovirus, rotavirus, norovirus 2. Bacterial --> campylobacter jejuni, salmonella, E.coli 3. Protozoa --> giardiasis

Question 590

How might diarrhoea present?

Answer 590

1. Diarrhoea +/- bloody stool 2. Fever +/- vomiting 3. Dehydration --> sunken fontanelle, dry mucus membranes, abnormal skin turgor, decreased consciousness, no tears, prolonged CRT

Question 591

How do you manage diarrhoea?

Answer 591

Oral rehydration solution --> IV is in shock or deteriorating Abx --> bacterial gastroenteritis complicated by septicaemia or systemic infections NO antiemetics or anti-motility drugs

Question 592

With which HLA molecules is coeliac disease often associated with?

Answer 592

DQ2 and DQ8

Question 593

Briefly describe the pathophysiology behind Coeliac Disease

Answer 593

o T cell mediated AI disease of the small bowel o Gliadin from gluten deaminated by tissue transglutaminase --> increases immunogenicity o Gliadin recognised by HLA-DQ2 receptor on APC --> inflammatory response o Plasma cells produce anti-gliadin and tissue transglutaminase --> T cells/cytokine activated o Consequences = villous atrophy and compensatory crypt hyperplasia --> malabsorption

Question 594

Give 3 risk factors for developing Coeliac Disease

Answer 594

``` 1. Family history 2, T1DM 3. Down's syndrome 4. IgA deficiency 5. Often associated with other AI disease ```

Question 595

Give 3 symptoms of coeliac disease

Answer 595

1. Bloating 2. Abdominal pain 3. Diarrhoea and steatorrhoea 4. Dermatitis herpetiformis 5. Dental enamel defects 6. Neonatal failure to thrive, buttock wasting, irritability at 8-24 months

Question 596

Describe the diagnostic criteria for coeliac disease

Answer 596

1. Characteristic histology after endoscopy and duodenal biopsy = villous atrophy, crypt hyperplasia, increased lymphocytes 2. Positive serology, tTG and anti-gliadin antibodies 3. Symptom resolution with gluten exclusion 4. Resolution of antibodies

Question 597

Give 2 possible complications of coeliac disease

Answer 597

1. Osteoporosis --> DEXA scans 2. Anaemia 3. Secondary lactose intolerance

Question 598

Give 2 examples of Inflammatory bowel disease (IBD)

Answer 598

1. Crohn's disease | 2. Ulcerative Colitis

Question 599

Describe Crohn's disease

Answer 599

A type of inflammatory bowel disease that affects anywhere from the mouth to the anus (mainly terminal ileum) It is us characterised by patchy (skip lesions), non caseating granulomatous inflammation that can affect any layer of the bowel wall (transmural) causing cobblestone damage (deep ulcers and fissures)

Question 600

Give 3 symptoms of Crohn's disease

Answer 600

1. Ulcers 2. General ill-health = severe, fatigue, malaise 3. Growth failure/weight loss 4. Abdominal pain 5. Diarrhoea

Question 601

Give 3 extra-intestinal signs o f Crohn's disease

Answer 601

1. Oral ulcers 2. Uveitis 3. Arthralgia 4. Erythema nodosum 5. Clubbing

Question 602

How do you treat Crohn's disease?

Answer 602

- Smoking cessation - Corticosteroids = remission, nutrition optimisation, hydration - Immunomodulators - Biologics --> infliximab - Surgical resection

Question 603

Give 2 possible complications of Crohn's disease

Answer 603

1. Small bowel obstruction 2. Abscess/fistulae formation 3. Perforation 4. Anal skin tag/fissure/fistula 5. Malnutrition

Question 604

Describe Ulcerative Colitis

Answer 604

A type of IBD that starts at the rectum and spreads proximally but only affects the colon Continuous inflammation that is confined to the mucosa Pseudopolyps

Question 605

Give 3 symptoms of UC

Answer 605

1. Episodic/chronic diarrhoea +/- blood/mucus 2. PR bleeding 3. Colicky pain 4. Weight loss 5. Growth failure

Question 606

Give 2 extraintestinal signs of UC

Answer 606

1. Erythema nodosum 2. Arthritis 3. Iritis and episcleritis 4. CLubbing and orał ulcers

Question 607

How do you treat UC?

Answer 607

- Smoking protects - Induction = Aminosalicylates (mesalamine) --> corticosteroids - Remission = Aminosalicylates --> 6MP/Azathioprine - Surgical resection

Question 608

Give 2 possible complications of UC

Answer 608

1. Toxic dilatation --> perforation risk 2. Increased risk of colorectal cancer 3. Ankylosing spondylitis 4. Uveitis, episcleritis, iritis 5. Primary sclerosing cholangitis (PSC)

Question 609

What investigations might you want to do on someone who you suspect has IBD?

Answer 609

1. ESR and CRP 2. Measure Hb --> often microcytic anaemia (IDA) 3. Low serum albumin 4. Endoscopy 5. Biopsy

Question 610

What histological findings would you see on a biopsy taken from someone with IBD?

Answer 610

1. Mucosal inflammation 2. Crypt damage 3. Ulceration

Question 611

Describe the treatment for IBD

Answer 611

1. Steroids 2, Dietary modifications 3. Immunosuppressants --> methotrexate and infliximab

Question 612

Give 3 benefits of using immunosuppressants as opposed to steroids of the treatment of IBD

Answer 612

1. Safe 2. Effective 3. Steroid sparing 4. Prevent relapse

Question 613

What is guarding?

Answer 613

Contraction of abdominal wall musculature, classically in response to underlying peritoneal inflammation

Question 614

Describe the difference between voluntary and involuntary guarding

Answer 614

Voluntary = contraction of abdominal wall musculature in anticipation of a painful stimulus Involuntary = 'true' guarding = conduced at the reflex arc level (most needs surgery)

Question 615

Give 3 GI causes of abdominal pain

Answer 615

1. IBS 2. Constipation 3. Gastritis 4. Peptic ulcer 5. Malrotation 6. Appendicitis 7. IBD 8. Abdominal migraine

Question 616

Give 3 gynaecological causes of abdominal pain

Answer 616

1. Dysmenorrhoea 2. PID 3. Ectopic pregnancy 4. Ovarian torsion

Question 617

Give 3 Hepatobiliary causes of abdominal pain

Answer 617

1. Hepatitis 2. Gallstones 3. Pancreatitis

Question 618

Give 2 urinary causes of abdominal pain

Answer 618

1. UTI | 2. PUJ obstruction

Question 619

Give 3 surgical causes of acute abdominal pain

Answer 619

1. Appendicitis 2. Non specific abdominal pain 3. Ovarian pathology 4. Meckel's diverticulum 5. Pancreatitis 6. Intussusception

Question 620

Give 3 signs of appendicitis

Answer 620

1. Anorexia 2. Pyrexia 3. Abdominal pain --> initially central and colicky to RIF 4. Vomiting 5. RIF rebound tenderness

Question 621

Why is central trauma and bruising to the abdomen a concern?

Answer 621

Bruising and trauma to the abdomen is a concern because mid-line structures such as the pancreas, spleen, bowel and liver may be damaged

Question 622

If you suspect trauma and abdominal organ damage what investigation might you do?

Answer 622

CT

Question 623

Give 3 differentials for abdominal pain and rectal bleeding

Answer 623

1. Anal fissures 2. Haemorrhoids 3. Polyps 4. Infective causes 5. Meckel diverticulum If melaena = gastritis or duodenal ulcer

Question 624

What is Meckel diverticulum a remnant of?

Answer 624

Vitelline duct (joins yolk sac to midgut lumen in foetus)

Question 625

Give 3 signs of Meckel diverticulum

Answer 625

1. Severe rectal bleeding 2. Intussusception 3. Volvulus

Question 626

How would you treat Meckel diverticulum?

Answer 626

Surgical resection

Question 627

Give 3 differentials for abdominal mass

Answer 627

1. Constipation 2. Appendicitis 3. Organomegaly 4. Nephroblastoma/Wilm's tumour

Question 628

Define faltering growth

Answer 628

Failure to gain adequate weight or achieve adequate growth during infancy and early childhood NICE defines it as weight that has fallen down 2 centime lines

Question 629

Give 3 nonorganic causes of faltering growth?

Answer 629

1. Feeding problems --> insufficient breast milk, poor techniques 2. Lack of regular feeding times 3. Difficulty feeding 4. Low socioeconomic status 5. Maternal depression 6. Neglect or child cabuse

Question 630

Give 2 organic causes of faltering growth

Answer 630

1. Impaired suck/swallow --> promoter dysfunction, cerebral palsy, cleft palate 2. Crohn's disease 3. Cystic fibrosis 4. Liver disease 5. Chronic renal failure 6. Malabsorption

Question 631

Give 2 causes of faltering growth due to increased requirements

Answer 631

1. Chronic illness --> CHD, CKD, CF 2. Thyrotoxicosis 3. Malignancy 4. Chronic infection --> HIV, immune deficiency, TB

Question 632

Why is prompt intervention important when managing a child with faltering growth?

Answer 632

Prompt intervention can avoid problems such as cognitive delay, feeding and behavioural problems and low maternal self-esteem

Question 633

You do a lumbar puncture and find, turbid, raised WCC (polymorphs) raised proteins and low glucose, is this likely to be due to a bacterial or a viral infection?

Answer 633

Bacterial

Question 634

You do a lumbar puncture and find clear, raised WCC (lymphocytes), normal proteins and normal glucose, is this likely to be due to a bacterial or a viral infection?

Answer 634

Viral

Question 635

Give 2 contraindications for a lumbar puncture

Answer 635

1. Cardiorespiratory instability 2. Focal neurological signs 3. Signs of raised ICP 4. Coagulopathy 5. Thrombocytopenia 6. Local infection at LP site

Question 636

Give 2 viral causes of meningitis

Answer 636

1. Enterovirus 2. Adenovirus 3. Mumps

Question 637

What bacteria commonly causes meningitis in children?

Answer 637

- Neonatal = GBS, E.coli, mysterious monocytogenes - 1 month - 6 years = Neisseria meningitidis, S. pneumonia, H. influenzae - >6 years = Neisseria meningitidis

Question 638

How does a child with meningitis present?

Answer 638

- Non specific in <18 months = fever, poor feeding, vomiting, irritability - Older child = neck stiffness - Bacterial meningitis = neck stiffness, bulging fontanelle, decreased consciousness, status epilepticus - Non blanching purpuric rash = meingincoccal sepsis

Question 639

Why does a child with meningococcal septicaemia develop a rash?

Answer 639

Due to bacterial endotoxins damaging blood vessels --> vasculitis --> bleeding into SC tissue --> thrombosis and DIC

Question 640

What investigations might you do on a child who you suspect has meningitis?

Answer 640

- Thorough history and examination - Blood cultures - Lumbar puncture

Question 641

Describe the treatment for a child with meningitis

Answer 641

- IV ceftriaxone (80mg/kg/od) or cefotaxime (50mg/kg/od) - Dexamethasone --> reduce risk of long term complications - Prophylaxis is needed to prevent further spread

Question 642

Name 2 drugs that can be given as meningitis prophylaxis

Answer 642

1. Rifampicin | 2. Ciprofloxacin

Question 643

Who must you notify when you diagnose a child with meningitis?

Answer 643

Public Health England

Question 644

Define sepsis

Answer 644

Dysregulated host response leading to end organ dysfunction

Question 645

What is a septic screen?

Answer 645

Checks for infection, in particular meningitis 1. Urine sample 2. Blood tests 3. Lumbar puncture

Question 646

Give 2 causes of encephalitis

Answer 646

1. Herpes simplex virus 2. Enterovirus 3. Post infectious encephalopathy --> following chickenpox 4. Slow virus infection --> HIV

Question 647

Give 2 signs of encephalitis

Answer 647

1. Fever 2. Altered consciousness 3. Behavioural changes 4. Seizures

Question 648

How do you treat encephalitis?

Answer 648

Parenteral high dose acyclovir

Question 649

What is Toxic Shock Syndrome caused by?

Answer 649

Toxin producing Staph aureus and group A strep

Question 650

What is TSS characterised by?

Answer 650

1. Fever >39 degree 2. Hypotension 3. Diffuse erythematous, macular rash

Question 651

Give 3 causes of immune deficiency

Answer 651

1. Primary = intrinsic defect in immune system --> inherited X linked or AR disorders 2. Incurrent bacterial or viral infection 3. Malignancy 4. Malnutrition 5. HiB infection 6. Immunosuppressive therapy 7. Splenectomy

Question 652

Give 3 signs of immune deficiency

Answer 652

1. Frequent infections 2. Infection with unusual organisms 3. Severe infections 4. Failure to thrive 5. Extensive candidiasis

Question 653

Who would you investigate for Severe immune deficiency?

Answer 653

- Children who present with Severe, Prolonged, Unusual or Recurrent (SPUR) infections - Those with family history

Question 654

Name 5 vaccine preventable diseases

Answer 654

1. Tetanus 2. Diphtheria 3. Whooping cough 4. Polio 5. Measles 6. Mumps 7. Rubella

Question 655

Give an example of a live, attenuated vaccine

Answer 655

1. MMR 2. BCG 3. Rotavirus

Question 656

Give an example of an inactivated vaccine

Answer 656

1. Influenza | 2. Diphtheria

Question 657

How do infants get HIV?

Answer 657

Due to vertical transmission = intrauterine, intrapartum and postpartum (breastfeeding)

Question 658

How are children likely to be affected by HIV?

Answer 658

- Infections - Asymptomatic - Lymphadenopathy - Parotitis - Candidiasis

Question 659

How is HIV diagnosed in children?

Answer 659

- Children <18 months born to infected month = transplacental maternal IgG HIV antibodie --> +ve test confirms exposure NOT HIV infection - <18 months most affective test = HIV DNA PCR --> 2 -ve within first 3 months of life, at least 2 weeks after completion of postnatal antiretroviral therapy = NOT infected - Children >18 months = antibody detection

Question 660

How is HIV managed in children?

Answer 660

- HIV infant medication starts at 4 weeks - Antiretroviral therapy = 2 nucleoside reverse transcriptase inhibitor (Abacavir, Didanosine) AND 1 non-nucleoside reverse transcriptase inhibitor (Efavirenz) OR 1 protease inhibitor (Atazanavir)

Question 661

Name the most common congenital infections

Answer 661

TORCH - Toxoplasma gondii - Other = Parvovirus, varicella zoster, syphillis - Rubella - CMV - Herpes simplex

Question 662

A baby makes many adaptations to ex-utero life, give 4 CV adaptations

Answer 662

1. Closure of foetal shunts --> foramen oval, ductus arteriosus 2. Perfusion of the lungs 3. Fall in pulmonary artery pressure and increase in systemic BP 4. Increase in cardiac output

Question 663

A baby makes many adaptations to ex-utero life, give 2 respiratory adaptations

Answer 663

1. Foetal lung fluid removed, filled with air 2. Surfactant released 3. Gaseous exchange

Question 664

A baby makes many adaptations to ex-utero life, aside from CV and respiratory give 4 adaptations

Answer 664

1. Control of own movements 2. Independent hormonal responses 3. Thermoregulation 4. Feeding 5. Immunocompetence 6. Conversion to adult haemoglobin

Question 665

Give 5 medical problems that preterm infants may suffer from

Answer 665

1. Respiratory distress syndrome 2. Apnoea and bradycardia 3. Patent ductus arteriosus 4. Infection 5. Jaundice 6. Intraventricular haemorrhage 7. Cystic periventricular leukomalacia 8. Necrotising enterocolitis 9. Retinopathy of prematurity 10. Hypothermia

Question 666

What long term problems can a pre term baby develop?

Answer 666

1. Retinopathy of prematurity 2. Chronic lung disease 3. Osteopenic bones 4. Neurodevelopmental delays

Question 667

Give 3 functions of surfactant

Answer 667

1. Reduces surface tension 2. Prevents alveoli collapse 3. Allows homogenous aeration

Question 668

When is surfactant produced?

Answer 668

From 34 weeks gestation | - Production increases rapidly 2 weeks before birth

Question 669

Where is surfactant produced?

Answer 669

By T2 pneumocytes

Question 670

What can mothers be given antenatally to prevent surfactant deficiency?

Answer 670

Steroids can be given to the mother to encourage foetal surfactant release

Question 671

Give 3 causes of surfactant deficiency

Answer 671

1. Respiratory distress syndrome 2. Chronic lung disease of prematurity 3. Non compliant lungs 4. Unequal aeration 5. Reduced lung volume

Question 672

Give 3 signs of respiratory distress in a child

Answer 672

1. Tachypnoea, RR = 40-60 2. Subcostal and intercostal recession 3. Stridor 4. Tracheal tug 5. Cyanosis

Question 673

How would you manage respiratory distress in a neonate?

Answer 673

1. O2 2. Intubate 3. CO2 monitoring 4. Surfactant therapy 5. Nasal CPAP and mechanical ventilation 6. Fluids if indicated

Question 674

Briefly describe the pathophysiology of chronic lung disease of prematurity

Answer 674

There is reduced lung volume and reduced alveolar SA --> diffusion defect This often leads to recurrent hospital admissions and increased mortality

Question 675

What is apnoea of prematurity

Answer 675

When a preterm infant has no repository drive due to lack of myelination around the brain stem (not fully myelinated until 32/34 weeks) --> they forget to breathe - Often associated with bradycardia

Question 676

Describe the treatment for apnoea of prematurity?

Answer 676

1. Nasal CPAP | 2. Stimulation = caffeine

Question 677

Give 2 benefits of breast feeding for the infant

Answer 677

1. Reduces the risk infection 2. Reduces the risk of allergic disease 3. Reduces the risk of GORD 4. Increased IQ and cognition

Question 678

Give 2 benefits of breast feeding for the mother

Answer 678

1. Reduces the risk of some types of cancer --> breast, uterine, ovarian, endometrial 2. Reduces the risk of PPH and postnatal depression 3. Optimum child spacing 4. Less food/medical expenses

Question 679

How would you feed a baby born at 27 weeks?

Answer 679

NG tube feeds | - At 35 weeks you can start breast/bottle feeding as this is when suckling reflex develops

Question 680

Give 5 signs of hepatic dysfunction in children

Answer 680

1. Encephalopathy 2. Jaundice 3. Epistaxis 4. Ascites 5. Varices 6. Spider naevi 7. Bruising 8. Palmar erythema 9 Clubbing 10. Malnutrition and faltering growth

Question 681

Give 3 reasons why newborns become jaundiced

Answer 681

1. RBC lifespan is shorter --> increased bilirubin production 2. Hepatic bilirubin metabolism is less efficient --> septic immaturity 3. Absence of gut flora impedes elimination of bile pigment

Question 682

Give 3 causes of high levels of unconjugated bilirubin

Answer 682

1. Haemolytic Disease 2. Phsyiological 3. Infection/sepsis 4. Breast bilk jaundice 5. CF 6. Hypothyroidism

Question 683

Describe the treatment for high levels of unconjugated bilirubin

Answer 683

1. Phototherapy | 2. Exchange transfusion

Question 684

How does phototherapy work in treatment jaundice?

Answer 684

Light converts unconjugated bilirubin into a water soluble pigment

Question 685

When might exchange transfusion be used in the treatment of jaundice?

Answer 685

If bilirubin rises to very dangerous levels and phototherapy alone is not effective

Question 686

What area of the brain does high levels of unconjugated bilirubin affects?

Answer 686

The basal ganglia

Question 687

Why are high levels of unconjugated bilirubin concerning in a neonate?

Answer 687

Unconjudgated bilirubin is fat soluble and can diffuse into brain tissue High levels can lead to kernicterus and then cerebral palsy

Question 688

What is kernicterus?

Answer 688

Brian damage due to high levels fo unconjugated bilirubin

Question 689

Give 3 signs of kernicterus

Answer 689

1. Lethargy progressing to hypertonia then hypotonia 2. Poor feeding 3. Fever 4. High-pitched cry 5. Opisthotonos (spasm of the muscles causing backward arching of the head, neck, and spine) 6. Seizures 7. Coma

Question 690

Give 2 possible complications of kernicterus

Answer 690

1. Deafness 2. Gaze disturbance 3. Cerebral palsy 4. Death

Question 691

When is jaundice most likely to occurs

Answer 691

Physiological Janice is common | Appears after 24 hours, peaks around 3 or 4 days and usually resolves by 14 days

Question 692

What are the likely causes of jaundice in a neonate who is <24 hours old?

Answer 692

1. Sepsis 2. Haemolytic causes --> haemolytic disease of the newborn, rhesus haemolytic disease, hereditary spherocytosis, G6PD deficiency 3. TORCH infections

Question 693

Why is it important to identify a neonate with haemolytic cause of their jaundice?

Answer 693

The bilirubin is unconjugated and if allowed to reach high levels could cause kernicterus

Question 694

Give 3 causes of jaundice at 24 hours to 2 weeks of age

Answer 694

1. Physiological jaundice 2. Breast milk jaundice 3. Infection --> UTI 4. Congenital hypothyroidism 5. Haemolytic cause

Question 695

Give 2 causes of jaundice > 2 weeks of age due to unconjugated hyperbilirubinaemia

Answer 695

1. Infection 2. Physiological or Breast milk jaundice 3. Hypothyroidism 4. Haemolytic disease

Question 696

Give 2 causes of jaundice > 2 weeks of age due to conjugated hyperbilirubinaemia

Answer 696

1. Biliary atresia | 2. Neonatal hepatitis syndrome

Question 697

What is biliary atresia?

Answer 697

When there is progressive fibrosis and obliteration of the extra-hepatic and intra-hepatic biliary tree Chronic liver failure and death can occur within 2 years

Question 698

Give 3 signs of biliary atresia

Answer 698

1. Prolonged neonatal jaundice (develops after 2 days) 2. Failure to thrive 3. Pale white stools and dark urine = obstructive jaundice 4. Hepatomegaly

Question 699

What investigations might you do to determine whether a child has biliary atresia?

Answer 699

1. Measure transcutaneous bilirubin = conjugated bilirubin raised 2. LFTs would be abnormal 3. ERCP imaging

Question 700

What is the treatment for biliary atresia?

Answer 700

- Surgery to bypass fibrotic ducts = hepatoportoenterostomy (Ksai procedure) - Nutrition and vitamin supplementation - If unsuccessful or late presentation = liver transplant

Question 701

Give 2 causes of neonatal hepatitis

Answer 701

1. Idiopathic = 80% 2. CMV 3. Rubella 4. Hepatitis A, B or C

Question 702

What investigations would you do on someone with suspected neonatal hepatitis?

Answer 702

Liver biopsy = multinucleated giant cells and rosette formation LFTs = increase unconjugated and conjugated bilirubin

Question 703

Why are preterm babies at increased risk of infection?

Answer 703

Active IgG transfer happens in the last 3 months of pregnancy

Question 704

Shat Abx would you prescribe empirically to reduce the risk of neonatal infection in a preterm baby?

Answer 704

- Benzylpenicillin (50mg/kg BD) | - Gentamicin (5mg/kg OD)

Question 705

What is necrotising enterocolitis?

Answer 705

Inflammation and necrosis of a portion of the GI tract

Question 706

What can increase the risk of necrotising enterocolitis?

Answer 706

IUGR

Question 707

What can decreased the risk of necrotising enterocolitis?

Answer 707

Breast feeding and probiotics

Question 708

Give 3 signs of necrotising enterocolitis

Answer 708

1. Feed intolerance 2. Vomiting 3. Distended abdomen 4. Stool contains fresh blood 5. Shock

Question 709

How can you treat necrotising enterocolitis?

Answer 709

Stop oral feeding | Broad spectrum Abx

Question 710

Give the cause of retinopathy of prematurity

Answer 710

Hyperoxic insult | - Can lead to blindness

Question 711

How could you treat retinopathy of prematurity?

Answer 711

Laser therapy

Question 712

Give 2 reasons why preterm infants are particularly vulnerable to hypothermia

Answer 712

1. Large SA relative to mass 2. Thin and heat permeable skin 3. Little fat for insulation - Temps are maintained using incubators

Question 713

Give 4 differentials for the acute scrotum

Answer 713

1. Testicular torsion 2. Epididymo-orchitis 3. Trauma 4. Acute hydrocele 5. Idiopathic scrotal oedema 6. Torsion of Hydatid/appendix of testicle ('blue dot')

Question 714

Give 5 signs and symptoms of testicular torsion

Answer 714

1. Severe, constant pain 2. Sudden onset pain 3. Vomiting 4. Tenderness 5. Swelling 6. Redness

Question 715

In what age groups is testicular torsion more common?

Answer 715

Neonates and peri-pubertal

Question 716

How is testicular torsion managed?

Answer 716

Surgery --> within 6 hours to save testicle

Question 717

What is hypospadias?

Answer 717

Displacement of urethral meatus (ventral), hooded foreskin and a bend in the shaft (chordee = ventral curve)

Question 718

Give 2 differentials for a groin swelling in children

Answer 718

1. Hernia | 2. Hydrocele

Question 719

Give 3 signs of an inguinal hernia

Answer 719

1, Can't get above it 2. Reducible 3. Often indirect in children Will need surgical repair

Question 720

Give 3 signs of a hydrocele

Answer 720

1. Can get above it 2. Confined to scrotum 3. Non reducible Should resolve by 2 years old

Question 721

How are undescended testes classified?

Answer 721

1. Retractile = Testis can be manipulated into the bottom of the scrotum without tension but subsequently retracts into inguinal region, pulled up the cremasteric muscle (corrects with age) 2. Palpable = Palpable in the groin but cannot be manipulated into the scrotum 3. Impalpable = NO testis felt on examination

Question 722

What investigations do you do for someone with undescended testes

Answer 722

1. US | 2. Hormonal tests = for bilateral impalpable testes

Question 723

How do you treat undescended testes?

Answer 723

- Laparoscopy for impalpable | - Orchidopexy = surgical placement of testis in scrotum

Question 724

At what point will testes not descend spontaneously?

Answer 724

After 6 months of age

Question 725

Give 3 differentials for head and neck lumps in children

Answer 725

1. Lymphadenopathy 2. Thyroglossal cyst (moves when stick tongue out) 3. Goitre 4. Malignancy 5. Branchial arch remnants 6. Dermoid cysts

Question 726

What are the red flag signs for head/neck lumps in children?

Answer 726

>2cm for >2 weeks and enlarging

Question 727

Define childhood disability

Answer 727

Someone who has a physical or mental impairment that results in a marked, pervasive limitation on activity e.g. Downs, Cerebral plasy

Question 728

Give 3 factors that influence childhood disability

Answer 728

1. Environment 2. Social background 3. Impairment

Question 729

What is cystic fibrosis?

Answer 729

Defect in CFTR gene (Chr 7) --> affecting pancreas, lungs, liver and gonads, thickens secretions

Question 730

Give 5 signs of cystic fibrosis

Answer 730

1. Neonate = meconium ileus, fralure to thrive 2. Cough = loose, productive or purulent sputum 3. Wheeze 4. Recurrent infections 5. Haemoptysis 6. Pancreatic insufficiency 7. Male infertility (absent vas deferens) 8. Maldigestion and malabsorption 9. Clubbing

Question 731

How is cystic fibrosis diagnosed?

Answer 731

- Newborn blood spot test = Guthrie test - Sweat test - Raised immunoreactive trypsin - Genetic testing

Question 732

How is cystic fibrosis managed?

Answer 732

- Physio - Prophylactic oral Abx - Nebulised DNAase or hypertonic saline - BIlateral lung transplant - Pancreatic replacement therapy - High calorie diet

Question 733

Give 2 complications of cystic fibrosis

Answer 733

1. DM | 2. Liver disease

Question 734

What is Duchenne Muscular Dystrophy?

Answer 734

X linked recessive muscle degeneration disorder due to deletion at Xp21 causing no functional dystrophin - Increase creatinine kinase

Question 735

How does DMD present?

Answer 735

Around 4 years old - Difficulty standing = Gowers sign (need to turn prone to rise) - Waddling gait - Language delay - Calf pseudohypertrophy - Respiratory failure - Scoliosis - Learning difficulties = 1/3

Question 736

How do you manage DMD?

Answer 736

- Exercise - Passive stretching and night splints - Orthotics - Physio - Surgical repair of scoliosis

Question 737

What conditions does the newborn blood spot (Guthrie test) screen for?

Answer 737

1. Cystic fibrosis 2. Congenital hypothyroidism 3. Sickle Cell Disease 6 Metabolic diseases --> MCADD, phenylketonuria and maple syrup disease etc

Question 738

Name 3 diseases with AD inheritance

Answer 738

1. ADPKD 2. Huntington disease 3. Marfan's

Question 739

Give 3 characteristics of AD inheritance

Answer 739

1. Vertical transmission 2. Mutation transmitted from Male to male 3. Every generation affected 4. Both males and females affected 5. 50% chance of inheritance

Question 740

Name 3 disease with AR inheritance

Answer 740

1. Cystic Fibrosis 2. Sickle Cell Disease 3. Haemochromatosis 4. Phenylketonuria

Question 741

Give 3 characteristics of AR inheritance

Answer 741

1. Both patients must be carriers 2. Often only one generation is affected 3. 2/3 carrier risk for unaffected siblings

Question 742

Give an example of an X linked disease

Answer 742

Duchenne and Becker muscular dystrophy

Question 743

Give 3 characteristics of X linked inheritance

Answer 743

1. Male > female affected 2. NO male to male transmission 3. 50% of daughters are carriers and 50% of sons are affected

Question 744

Give 4 example son non-mendelian inheritance

Answer 744

1. Multifactoral --> neural tube defects 2. Mitochondrial 3. Genomic imprinting 4. Gonadal mosaicism

Question 745

Give an example of a neural tube defect

Answer 745

Spina Bifida | - More likely to happen is there is folic acid deficiency or maternal deficiency

Question 746

Give 2 exmaples of genomic imprinting

Answer 746

1. Prader Willi = Caused by a deletion in the paternally inherited chromosome 15 (15q11-13) or maternal uniparental disomy 2. Angelmans Syndrome = Caused by a deletion in the Maternally inherited chromosome 15 or paternal uniparental disomy

Question 747

Give 2 symptoms of Prader Will syndrome

Answer 747

1. Neonatal hypotonia 2. Poor feeding 3. Moderate developmental delay and learning difficulties 4. Hyperphagia and obesity

Question 748

Give 2 symptoms of Angelmans Syndrome

Answer 748

1. "Happy puppet" = unprovoked laughing.clapping 2. Microcephaly 3. Severe cognitive impairment 4. Seizures 5. Ataxia 6. Broad based gait

Question 749

What is gonadal mosaicism?

Answer 749

Gonadal mosaicism is when there are two different populations of cells in the gonads One population is normal and the other is mutated All gametes from the mutated line are affected

Question 750

Why does Down's syndrome occur?

Answer 750

Autosomal trisomy due to 1. Meiotic non disconjunction (94%) 2. Translocation (5%) 3. Moasiacism (1%)

Question 751

Give 3 signs of Down's syndrome

Answer 751

1. Learning/developmental delay 2. Hypotonia 3. Short stature 4. CHD 5. Brushfield spots 6. Single palmar crease 7. Flat occiput 8. Wide 'sandal' gap between big and 2nd toe

Question 752

What is Edwards syndrome and give 2 features?

Answer 752

Trisomy 18 1. Low birthweight 2. Prominent occiput 3. Small mouth and chin 4. Short sternum 5. Flexed, overlapping fingers 6. ‘Rocker-bottom’ feet 7. Cardiac and renal malformations

Question 753

What is Patau Syndrome and give 2 features

Answer 753

Trisomy 13 1. Structural defect of brain 2. Scalp defects 3. Small eyes (microphthalmia) and other eye defects 4. Cleft lip and palate 5. Polydactyly 6. Cardiac and renal malformations

Question 754

Define child development

Answer 754

The biological, psychological and emotional changes that occur between birth and adolescence as the individual processes from dependency to crashing autonomy It is a continuous process with a predictable sequence however each childs development is unique

Question 755

Give 5 influences on a child's development

Answer 755

1. Genetic factors 2. Stimulating environment 3. Pregnancy factors --> premature, mums health 4. Healthy attachment 5. Medical conditions 6. Abuse/neglect/domestic violence 7. Healthy peer relationships 8. Education 9. Nutrition 10. Parenting style

Question 756

What are the 4 domains of child development?

Answer 756

1. Gross Motor 2. Fine motor and vision 3. Speech, language and hearing 4. Social interaction and self-care skills

Question 757

What are the developmental milestones for gross motor function?

Answer 757

- 3m = lifts head on tummy - 6m = chest up with arm support, can sit unsupported - 8m = crawling - 9m = pulls to stand - 12m = walking - 2 years = walking up stairs - 3 years = jumping - 4 years = hopping - 5 years = rides a bike

Question 758

With regards to gross motor development, at what age would you expect a child to do the following a) Walking b) Jumping c) Crawling d) Walking up stairs?

Answer 758

a) Walking = 12 months b) Jumping = 3 years c) Crawling = 8 months d) Walking up stairs = 2 years

Question 759

What are the developmental milestones for fine motor and visual function?

Answer 759

- 4m = grabs an object using both hands - 8m = takes objects in each hand - 12m = scribbles with crayons e.g. circle, cross, square - 18m = builds a tower of 2 cubes - 3 years = builds a tower of 8 cubes

Question 760

With regards to fine motor and visual development, at what age would you expect a child to do the following a) Drawing with crayons b) Building a tower of 8 cubes c) Takes an object in each hand d) Builds a tower of 2 cubes

Answer 760

a) Drawing with crayons = 12m b) Building a tower of 8 cubes = 3 years c) Takes an object in each hand = 8m d) Builds a tower of 2 cubes = 18m

Question 761

What are the developmental milestones for speech, language and hearing?

Answer 761

- 3m = laughs and squeals - 9m = can make sounds such as 'dada' and 'mama' - 12m = can say one word - 2 years = can form short sentences and name body parts - 3 years = speech is mainly understandable - 4 years = knows colours and can count - 5 years = knows the meaning of words

Question 762

With regards to speech, language and hearing, at what age would you expect a child to do the following a) Form short sentences and name body parts b) Knows colours and can count c) Laughs and squeals d) Has mainly understandable speech

Answer 762

a) Forms short sentences and name body parts = 2 years b) Knows colours and can count = 4 years c) Laughs and squeals = 3 months d) Has mainly understandable speech = 3 years

Question 763

What are the developmental milestones for social interaction and self-care skills?

Answer 763

- 6 weeks = smiles - 6 months = finger feeds - 9m = waves bye-bye - 12m = uses cutlery - 2 years = undresses, feeds toys - 3 years = plays with others, names a friend - 4 years = dresses with no help, plays a board game

Question 764

With regards to social interaction and self-care skills, at what age would you expect a child to do the following a) Uses cutlery b) Plays with others, names a friend c) Smiles d) Waves bye-bye

Answer 764

a) Uses cutlery = 12 months b) Plays with others, names a friend = 3 years c) Smiles = 6 weeks d) Waves bye-bye = 9 months

Question 765

What does 'The healthy child programme' encourage?

Answer 765

1. Encourages care to keep children healthy and safe 2. Promotes healthy eating and activity 3. Identifies problems in children's development 4. Identifies 'at risk' families for more support 5. Ensures children are prepared for school

Question 766

When does universal surveillance of developmental occur?

Answer 766

- Neonatal examination (NIPE) --> within 72 hours - Blood spot --> 5-9 days, midwife - Baby review (14 days old) --> health visitor - 6-8 week check --> GP - At 1 year (for development problems) - Between 2-2.5 years (for development problems)

Question 767

Give 2 examples of concerning child development with regards to gross motor function

Answer 767

1. Not sitting by 12 months | 2. Not walking by 18 months

Question 768

Give an example of concerning child development with regards to fine motor function

Answer 768

Hand preference before 18 moths --> neuro condition like cerebral palsy

Question 769

Give a speech and language examples that may suggest concerning child development

Answer 769

No clear words by 18 months --> hearing/learning disability? ASD?

Question 770

Give two examples of concerning child development with regards to social development

Answer 770

1. No response to carers interaction by 8 weeks 2. No smiling by 3 months --> vision impairment? 3. No interest in playing by 3 years

Question 771

Give 3 red flags in development

Answer 771

1. Regression of development 2. Poor health/growth 3. Significant family history 4. Findings on examination --> microcephaly, dysmorphic features 5. Safeguarding indicators

Question 772

Give 5 causes of developmental delay

Answer 772

1. Genetics 2. Pregnancy 3. Factors around birth 4. Factors in childhood 5. Environmental

Question 773

Give 2 examples of genetic causes of developmental delay

Answer 773

1. Chromosomal disorders --> Downs 2. Single gene disorders --> Duchenne 3. Polygenic --> ASD, DHD 4. Micro-deletions or micro duplications

Question 774

Give 2 examples of pregnancy related causes of developmental delay

Answer 774

1. Congenital infections --> CMV, HIV 2. Exposure to drugs/alcohol --> FAS 3. MCA infarct

Question 775

Give 2 examples of birth related causes of developmental delay

Answer 775

1. Prematurity | 2. Birth asphyxia (due to hypoxia)

Question 776

Give 2 examples of childhood medical causes of developmental delay

Answer 776

1. Infections --> meningitis 2. Chronic illness 3. hearing or visual impairment 4. Acquired brain injury

Question 777

Give 2 examples of environmental causes of developmental delay

Answer 777

1. Abuse and neglect | 2. Low stimulation

Question 778

How might you investigate someone's child development if you suspected that there was something wrong?

Answer 778

Thorough history and examination Tailor any investigations to the child e.g. - Boys not walking by 18m check creatinine kinase for Duchenne - Focal neurological signs -> MRI brain - Genetic testing - Unwell, failure to thrive -> metabolic investigations There is no 'developmental screen', investigations need to be tailored towards to the child

Question 779

Name 3 primitive reflexes

Answer 779

1. Moro 2. Grasp 3. Rooting 4. Stepping response 5. Asymmetrical tonic neck reflex

Question 780

What is the Moro reflex?

Answer 780

The startle reflex = primitive reflex - Response due to a sudden loss of support, sudden extension of the head causes symmetrical extension then flexion of the arms

Question 781

Name 2 postural reflexes

Answer 781

1. Labyrinthine righting 2. Postural suport 3. Lateral propping 4. Parachute

Question 782

Why should primitive reflexes disappear?

Answer 782

Primitive reflexes should gradually disappear as postural reflexes develop - Essential for good motor development

Question 783

Why is it concerning is primitive reflexes persist?

Answer 783

May be a sign of CNS dysfunction

Question 784

Define Self harm

Answer 784

Wide range of things that people do to hurt themselves in a deliberate and usually hidden way, which are damaging - Act with intent to hurt self but no intention to kill self

Question 785

Give 3 predisposing factors for a child developing depression

Answer 785

1. Family history 2. Stress in pregnancy 3. Poor attachment 4. Poverty 5. Isolation

Question 786

Give 3 precipitating factors for a child developing depression

Answer 786

1. Trauma 2. Drugs 3. Infections 4. Puberty 5. Exam stress 6. Sexual abuse 7. Bullying

Question 787

Give 3 perpetuating factors for a child developing depression

Answer 787

1. Chronic illness 2. Malnutrition 3. Ongoing neglect 4. Ongoing poverty

Question 788

Give 4 symptoms of depression

Answer 788

1. Persistent sadness or low mood 2. Loss of interest or enjoyment 3. Fatigue 4. Poor or increased sleep 5. Poor or increased appetite 6. Low concentration 7. Low self confidence 8. Feelings for guilt and self blame 9. Hopelessness

Question 789

Describe the non medical and medical treatment of depression

Answer 789

Non medical = Education, CBT, IPT, family therapy | Medical = fluoxetine, sertraline, citalopram

Question 790

What is the diagnostic criteria for anorexia?

Answer 790

Deliberately keeping weigh below 85% of expected - Restricted dietary choice - Excessive exercise - Induced vomiting, use of appetite suppressants and diuretics Also have dread of fatness and can have endocrine effects (amennorhoea)

Question 791

Give 5 risk factors for developing anorexia

Answer 791

1. Social pressure 2. Perfectionist character traits 3. Family attitudes towards food 4. Low self esteem 5. Occupation/interests 6. Family history 7. Past of present life evens --> abuse, illness, grief

Question 792

What screening tool can be used for investigation earring problems?

Answer 792

SCOFF • Do you make yourself Sick because you’re uncomfortably full? • Do you worry that you’ve lost Control over how much you eat? • Have you recently lost more than 6kg (about One stone) in 3 months? • Do you believe you’re Fat when others say you’re thin? • Would you say that Food dominates your life?

Question 793

Describe the treatment for anorexia

Answer 793

1. Family therapy 2. IPT 3. CBT Weight restoration at 0.5kg/week - Monitor for re-feeding syndrome

Question 794

Give 5 ways in which radiological imaging for a child differs to imaging an adult

Answer 794

1. Size 2. Growth plates 3. Skull sutures 4. Ossification 5. Congenital problems --> dextrocardia, osteogensis imperfecta

Question 795

What night an abdominal XR be indicated on a child?

Answer 795

1. Abdominal distension --> obstruction 2. Abdominal pain of unknown cause 3. Constipation

Question 796

What analgesia is appropriate for a paediatric fracture?

Answer 796

Paracetamol | Oromorph

Question 797

Give 5 causes of dehydration

Answer 797

1. Dysphagia --> cerebral palsy, developmental delay 2. VOmiting 3. Behavioural/Psychiatric 4. Neglect 5. Burns/Sepsis 6. Cystic fibrosis 7. Kidney disease --> renal tubular disease, renal dysplasia

Question 798

Give 4 signs of dehydration

Answer 798

1. thirst 2. Dry mucous membranes 3. Restlessness, irritability 4. Sunken eyes/fontanelle 5. Reduced skin turgor 6. Decreased urine output 7. Reduced consciousness 8. Cold, mottled peripheries 9. Anuria

Question 799

How much should you feed to a neonate?

Answer 799

``` 150ml/kg/day Day 1 = 60ml/kg/day Day 2 = 90ml/kg/day Day 3 = 120ml/kg/day Day 4 onwards = 150ml/kg/day ```

Question 800

What fluid should you give to a neonate?

Answer 800

10% dextrose

Question 801

What fluid should you give to children?

Answer 801

0.9% Sodium Chloride + 5% glucose +/- KCl

Question 802

How do you calculate the rate at which to give maintenance fluids to a child?

Answer 802

100ml/Kg/day for first 10Kg 50ml/Kg/day for the next 10Kg 20ml/Kg/day for every Kg after that

Question 803

When taking a history from a teenager, the HEADDS framework can be used, describe this framework

Answer 803

``` Home Education Activity Drugs/alcohol Depression/suicide Sexuality ```

Question 804

Give 4 risk factors for child abuse

Answer 804

1. Lack of parental support, parental indifference or intolerance 2. Parental mental health probelms 3. Parental alcohol or drug abuse 4. Domestic violence within the family 5. Young parental age 6. Failure of child to meet parental expectations/aspirations 7. Poverty

Question 805

Name the 4 broad categories of child abuse

Answer 805

1. Physical injury --> bruises, scratches, burns, fractures 2. Sexual abuse --> behaviour change, physical symptoms e.g. bleeding, STI, pregnancy 3. Emotional abuse --> relationships high in criticism and low in warmth 4. Neglect --> care that does not meet the needs of a child

Question 806

How does child abuse present?

Answer 806

- Disclosure - Injury observed --> at school - Incidental findings

Question 807

How would you manage a child whom you suspect is a victim of child abuse?

Answer 807

- Thorough history --> ensure good documentation, are there any discrepancies? - Examination --> use body charts - FBC, clotting, swabs, bone profile, skeletal survey - Social services assessment +/- police input