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Paediatrics Flashcards

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1
Q

What are the most common causes of pneumonia?

A
  • Neonates = group B strep
  • Infants = strep pneumoniae
  • Children = strep pneumoniae/staph aureus/group A strep
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2
Q

What is the investigation/finding and management for pneumonia?

A
  • CXR = consolidation
  • Supportive management
  • Oxygen (if severe)
  • Abx (amoxicillin/co-amoxiclav/erythromycin)
  • Mycoplasma pneumonia = macrolides (azithromycin/erythromycin)
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3
Q

Describe croup

A
  • 6 months - 3 years
  • Peak incidence at 2 years
  • More common in males
  • Parainfluenza virus
  • 1-4 day history
  • Sx worse at night
  • Barking cough
  • Hoarse voice
  • Stridor
  • Fever/rhinorrhoea
  • Single oral dose of dexamethasone or prednisolone
  • Nebulised adrenaline
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4
Q

Describe roseola infantum

A
  • Self limiting
  • Human herpes virus type 6
  • Febrile illness followed by rash with blanching papules
  • Patient is well
  • No exclusion from school
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5
Q

What are red flag sx in respiratory?

A
  • Drowsiness/decreased consciousness
  • Tachypnoea (>60)
  • Intercostal recession
  • Cyanosis
  • Laboured breathing
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6
Q

How is croup severity calculated?

A

Westley Croup Score
- Mild = 0-2
- Moderate = 3-5
- Severe = 6-11
- Impending respiratory failure = 12-17

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7
Q

What criteria is included in the Westley Croup Score?

A
  • SaO2 <92%
  • Stridor
  • Retractions
  • Air entry
  • Consciousness
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8
Q

What is a common differential diagnosis for croup?

A

Epiglottitis (shorter time course and more severe)

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9
Q

Describe acute epiglottitis

A
  • Haemophilus influenzae type B (Hib)
  • Acute onset (hours)
  • Tripoding (lean forward with mouth open and tongue out)
  • Sore throat/SOB/fever
  • Drooling
  • Stridor
  • DON’T EXAMINE THROAT
  • CXR (thumb sign)
  • O2
  • Nebulised adrenaline
  • Secure airway/tracheostomy
  • Fluids
  • Abx (if underlying infection) e.g. ceftriaxone
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10
Q

What is an important preventative measure for acute epiglottitis?

A
  • 6-in-1 DTaP/IPV/Hib vaccination
  • At 8/12/16 weeks
  • Booster at 1 year
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11
Q

Describe asthma

A
  • Risk factors = genetics (atopy)/prematurity/low birth weight/not breastfed/exposure to allergens
  • Episodic wheeze
  • Dry cough (often worse at night)
  • Chest tightness
  • SOB
  • Reduced peak flow
  • Diurnal variability
  • FEV1 reduced
  • FVC normal
  • FEV1:FVC <70%
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12
Q

What is the long-term management for asthma in children?

A
  1. SABA PRN (salbutamol)
  2. ICS (beclomethasone)
  3. LRTA (montelukast)
  4. Stop LRTA and add LABA (salmeterol)
  5. Switch ICS/LABA for ICS MART (formoterol and ICS)
  6. Add separate LABA
  7. Increase ICS dose and refer
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13
Q

What is the long-term amanagement for asthma in children <5?

A
  1. SABA PRN (salbutamol)
  2. SABA + 8 week trial of ICS (restart if sx reoccur within 4 weeks)
  3. Refer
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14
Q

What is the management for acute asthma?

A

Acute - O SHIT ME
1. Oxygen
2. Salbutamol nebulisers (SABA)
3. Hydrocortisone IV or oral prednisolone (steroids)
4. Ipratropium bromide nebulisers (SAMA)
5. Theophylline/aminophylline
6. IV magnesium sulphate
7. Escalate

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15
Q

What is the criteria for life threatening asthma?

A
  • SpO2 <92%
  • PEFR <33% predicted
  • Silent chest
  • Poor respiratory effort
  • Altered consciousness
  • Agitation/confusion
  • Exhaustion
  • Cyanosis
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16
Q

Describe viral induced wheeze

A
  • RSV/rhinovirus
  • Evidence of viral illness (fever/cough/coryzal sx 1-2 days prior)
  • SOB
  • Signs of respiratory distress
  • Expiratory wheeze throughout chest
  • Manage same as asthma
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17
Q

Describe bronchiolitis

A
  • Children <2 (3-6 months)
  • Very common
  • Peaks in winter/spring
  • RSV
  • Risk factors = breastfeeding <2 months/smoke exposure/older siblings who attend nursery/school/chronic lung disease of prematurity
  • 2-5 day history
  • Coryzal symptoms (fever/nasal congestion/rhinorrhoea)
  • Cough
  • Reduced feeding
  • Bilateral wheeze
  • Bilateral crepitations
  • Nasopharyngeal aspirate/throat swab
  • Supportive management/Palivizumab
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18
Q

What is cystic fibrosis?

A

Autosomal recessive genetic condition caused by mutation of CFTR gene on chromosome 7

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19
Q

What are common microbial colonisers in CF?

A
  • Staph aureus
  • Pseudomonas aeruginosa
  • Haemophilus influenzae
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20
Q

How does CF affect the respiratory tract?

A
  • Recurrent LRTIs
  • Chronic cough
  • Thick sputum
  • Crackles and wheeze
  • Nasal polyps
  • Chest hyperinflation
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21
Q

How does CF affect the pancreas?

A
  • Pancreatitis
  • Pancreatic insufficiency (steatorrhoea due to lack of fat digesting enzymes)
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22
Q

How does CF affect the GI tract?

A
  • Bowel obstruction in-utero (meconium ileus)
  • Cholestasis in-utero (neonatal jaundice)
  • Distal intestinal obstruction syndrome
  • Steatorrhoea (due to lack of fat digesting enzymes)
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23
Q

How does CF affect the reproductive tract?

A

Congenital bilateral absence of vas deferens = male infertility

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24
Q

What are investigations for CF?

A
  • Genetic testing for CFTR gene (amniocentesis/CVS/blood test)
  • Meconium ileus + abdominal distension + vomiting
  • Newborn heel prick test
  • Bloods = immunoreactive trypsinogen (high)
  • Sweat test (cause skin to sweat and test Cl- conc)
  • CXR (hyperinflation)
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25
What is the lifestyle management for CF?
- Patient/family education - Avoid other CF px - Chest physiotherapy - Vitamin A/D/E supplements - High calorie and high fat diet - CREON tablets (pancreatic enzyme supplementation)
26
What is the cause of whooping cough?
Bacterium Bordetella Pertussis (gram -ve bacillus)
27
What are the clinical features of whooping cough?
- Rhinitis/conjunctivitis - Irritability/sore throat/fever/dry cough - Severe paroxysms of coughing followed by inspiratory gasp (whoop) often followed by going blue and vomiting
28
What are investigations for whooping cough?
- Culture of nasopharyngeal aspiration/swab - Anti-pertussis toxin IgG serology - Anti-pertussis toxin detection in oral fluid - FBC (lymphocytosis)
29
What is the management for whooping cough?
- Macrolide abx (clarithromycin <1 month, azithromycin/clarithromycin >1 month, co-trimoxazole 2nd line) - Isolation for 21 days after sx onset or 2 days after abx - Supportive management
30
What are important preventative measures for whooping cough?
- Vaccinations given at 2/3/4 months (8/12/16 weeks) = 6-in-1 - Booster at 3 years and 4 months = 4-in-1 - Prophylactic abx for close contacts at high risk
31
What is otitis media and what are common causes of it?
Infection of middle ear Bacterial: - Strep pneumoniae Viral: - RSV - Rhinovirus
32
What are the clinical features of otitis media?
- Ear pain - Reduced hearing - Bulging tympanic membrane - General sx of URTI - Balance issues/vertigo - Discharge (if tympanic membrane perforated)
33
What are the investigations for otitis media?
- Examine ears and throat - Otoscope - to visualise tympanic membrane (normal = pearly-grey/translucent/slightly shiny; otitis media = bulging/red/inflamed looking membrane)
34
What is the management for otitis media?
- Analgesia - Most cases resolve without abx within 3 days but can last up to 1 week - Abx (amoxicillin 1st line then erythromycin/clarithromycin)
35
What are complications of otitis media?
- Otitis media with effusion (glue ear) - Hearing loss - Perforated eardrum - Recurrent infection - Mastoiditis
36
What are the clinical features of glue ear?
- Difficulty hearing - Retracted eardrum - Sensation of pressure inside ear (accompanied by popping/crackling noises) - Disequilibrium/vertigo
37
What is the investigation for glue ear?
Otoscopy: - Tympanic membrane appears dull - Light reflex lost (indicates fluid in middle ear) - Bubble seen behind TM
38
What is the management for glue ear?
- Some cases resolve within 3 months - Hearing aid insertion - Surgery - myringotomy and grommet insertion - Surgery - adenoidectomy
39
What are grommets?
Small tubes inserted into the tympanic membrane to allow fluid from the middle ear to drain through the tympanic membrane to the ear canal - usually fall out within a year
40
What type of hearing loss is more common in children?
Conductive - recurrent ear infections or insertion of foreign objects into ear canal
41
What are some causes of congenital deafness?
- Maternal rubella/CMV infection during pregnancy - Genetic deafness - Associated conditions e.g. Down's syndrome
42
What are some causes of perinatal deafness?
- Prematurity - Hypoxia during/after birth
43
What are some causes of deafness after birth?
- Jaundice - Meningitis/encephalitis - Otitis media/glue ear - Chemotherapy
44
What are some causes of sensorineural deafness?
- Exposure to loud noise - Injury - Disease - Drugs - Inherited condition
45
What are some causes of conductive deafness?
- Ear wax/foreign object - Fluid/infection/bone abnormality/eardrum
46
What are the investigations for hearing loss?
- UK newborn hearing screening programme (NHSP) - Audiometry
47
What is periorbital cellulitis and what are common causes of it?
Eyelid/skin infection of periorbital soft tissue superficial to orbital septum - Staph aureus (most common) - Strep pneumoniae/haemophilus influenzae
48
What are risk factors for periorbital cellulitis?
- Male - Previous sinus infection - Lack of Hib infection - Recent eyelid injury
49
What are the clinical features of periorbital cellulitis?
- Eyelid erythema - Eyelid oedema - Hot skin around eyelids/eye - Normal vision - Normal ocular motility with no pain
50
What are the investigations for periorbital cellulitis?
- CT sinus and orbits with contrast
51
What is the management for periorbital cellulitis?
Empirical abx e.g. cefotaxime/clindamycin
52
What is a common differential diagnosis for periorbital cellulitis?
Orbital cellulitis - muscles of orbit affected usually due to bacterial sinusitis - LIFE-THREATENING
53
What is a squint and what are some common causes of it?
Misalignment of the eye a.k.a strabismus - Hydrocephalus - Cerebral palsy - Space-occupying lesions e.g. retinoblastoma - Trauma
54
What is amblyopia?
In squint, lazy eye becomes progressively more passive and has reduced function
55
What is the main feature of a squint and what is the management?
- Diplopia - Occlusive patch over good eye - Atropine drops in dominant eye (causes blurred vision)
56
What does the foramen ovale connect?
Left and right atria in foetuses - can bypass pulmonary circulation
57
What does the ductus arteriosus connect?
Pulmonary artery and aorta in foetuses - can bypass pulmonary circulation
58
What is the most common septal defect?
Ventricular septal defect
59
What are risk factors for VSD?
- Prematurity - Genetic conditions e.g. Down's syndrome/Edward's/Patau - Fhx
60
What are the clinical features specific to VSD?
- Asx - Pansystolic murmur in LLSE --> transmits to USE and axillae
61
What are the investigations for VSD?
- Echo - CXR (large pulmonary arteries/cardiomegaly) - ECG
62
What is the management for VSD?
- Many close spontaneously - Surgery - Diuretics (to relieve pulmonary congestion) - ACEi (to reduce systemic pressure)
63
What are complications of VSD?
- Eisenmenger's - Endocarditis - HF
64
What are risk factors for ASD?
- Maternal smoking in 1st trimester - Fhx - Maternal diabetes/rubella
65
What are the types of ASD?
- Ostium primum (AV valve) - Ostium secundum (centre of atrial septum) - Sinus venosus (superior/inferior vena with right atrium connection)
66
What are the clinical features specific to ASD?
- Asx - Fixed, widely split S2 sound - Ejection systolic murmur in pulmonary area - Recurrent chest infections
67
What are the investigations for ASD?
- CXR (large pulmonary arteries/cardiomegaly) - Echo - ECG
68
What is the management for ASD?
- Most close spontaneously - Surgery
69
What are complications of ASD?
- Eisenmenger's - AF - Stroke from DVT
70
What are the risk factors for patent ductus arteriosus?
- Female - Prematurity
71
What are the clinical features for patent ductus arteriosus?
- Continuous machinery murmur in pulmonary area - Collapsing pulse - Heaving apex beat - Left subclavicular thrill
72
What are the investigations for patent ductus arteriosus?
- CXR (cardiomegaly) - Echo - ECG
73
What is the management for patent ductus arteriosus?
- Surgery (cardiac catheterisation) - Indomethacin/ibuprofen (inhibits prostaglandins and stimulates closure)
74
What are complications of patent ductus arteriosus?
- Eisenmenger's - Differential cyanosis
75
What are generic clinical features of septal defects?
- Poor feeding/failure to thrive - Tachypnoea/dyspnoea - Active precordium - Thrill - Gallop rhythm - Hepatomegaly - Oedema
76
What are the clinical features specific to AVSD?
Murmur arises from valvular regurgitation
77
What are the investigations for AVSD?
- Echo - Screen children for Down's syndrome
78
What is the management for AVSD?
Surgery
79
What is a complication of AVSD?
Pulmonary vascular disease
80
Briefly describe the pathophysiology of coarctation of aorta
- Collateral circulation forms to increase flow to lower part of body - Intercostal arteries become dilated and tortuous
81
What are the clinical features of coarctation of aorta?
- Weak femoral pulses - Pre and post ductal difference in saturations - Murmur over back - Collapse/acidosis
82
What are the investigations for coarctation of aorta?
- 4 limb BP (discrepancy between upper and lower limbs) - CXR (rib notching) - CT/MRI
83
What is the management for coarctation of aorta?
Surgery - angioplasty and stent insertion
84
What are complications of coarctation of aorta?
- HTN - Re-coarctation - CHF - Intracerebral haemorrhage - Bacterial endocarditis
85
What are the clinical features of aortic stenosis?
- Weak pulses - Thrill palpable in suprasternal region and carotid area - Ejection systolic murmur in aorta area - Collapse/acidosis
86
What is the management for aortic stenosis?
Aortic valve replacement surgery
87
What are the clinical features of pulmonary stenosis?
- Ejection systolic murmur in LUSE (often radiates to back) - Right ventricular heave
88
What is the management for pulmonary stenosis?
Balloon valvuloplasty
89
What are the 3 main cyanotic heart conditions?
- Transposition of the great arteries - Tetralogy of Fallot - Ebstein's anomaly
90
What is transposition of the great arteries?
Heart defect in which the aorta and pulmonary artery have swapped over
91
What are risk factors for transposition of the great arteries?
- Maternal diabetes - Males - Increased maternal age - Maternal rubella - Alcohol consumption
92
What are clinical features of transposition of the great arteries?
- Cyanosis/acidosis/collapse/death - Right ventricular heave - Loud S2 sound - Systolic murmur - Respiratory distress/tachycardia/poor feeding/poor weight gain/sweating
93
What are the investigations for transposition of the great arteries?
- Antenatal USS - CXR ('egg on a string' due to narrowed mediastinum and cardiomegaly) - Low SATS - Metabolic acidosis
94
What is the management for transposition of the great arteries?
- Prostaglandin infusion (to maintain ductus arteriosus) - Surgery
95
What conditions make up tetralogy of fallot?
- Pulmonary stenosis - Overriding aorta - Ventricular septal defect - Right ventricular hypertrophy
96
What are risk factors for tetralogy of fallot?
- Male - Maternal diabetes/rubella - Alcohol consumption - Increased maternal age - Associated with 22q11 deletion
97
What are the clinical features of tetralogy of fallot?
- Cyanosis - Acidosis - Collapse/death - Irritability - Clubbing - Poor feeding/weight gain - Ejection systolic murmur in pulmonary area - Tet spells
98
What are tet spells?
Intermittent symptomatic periods where right to left shunt becomes temporarily worsened --> irritability/cyanosis/SOB/reduced consciousness/seizures/death
99
What are the investigations for tetralogy of fallot?
- Check for 22q deletion - Echo - CXR (boot-shaped heart due to right ventricular thickening) - MRI/cardiac catheter
100
What is the management for tetralogy of fallot?
- Surgery - Prostaglandin infusion (to maintain ductus arteriosus) - Morphine (to reduce respiratory drive) - Tet spells (O2/beta blockers/fluids/morphine/sodium bicarbonate/phenylephrine infusion)
101
What are complications of tetralogy of fallot?
- Pulmonary regurgitation - Right HF - Death
102
What is Ebstein's anomaly?
Congenital heart condition in which tricuspid valve is set lower in the right side of the heart causing a bigger right atrium and smaller right ventricle
103
What are the causes of Ebstein's anomaly?
- Genetics - Lithium (teratogenic)
104
What conditions are associated with Ebstein's anomaly?
- ASD - Wolff-Parkinson-White Syndrome
105
What are the clinical features of Ebstein's anomaly?
- Evidence of HF (e.g. oedema) - Gallop rhythm - 3rd and 4th heart sounds - Cyanosis - SOB/tachypnoea - Poor feeding - Collapse/cardiac arrest
106
What is the investigation for Ebstein's anomaly?
Echo
107
What is the management of Ebstein's anomaly?
- Treat arrhythmia/HF - Prophylactic abx (prevent IE) - Surgical correction
108
What are risk factors for rheumatic fever?
- Female - Children - Poverty - Overcrowding/poor hygiene - Fhx - D8/17B cell antigen positivity
109
What is the cause of rheumatic fever?
Strep pyogenes - systemic infection that occurs 2-4 weeks after pharyngitis due to cross-reactivity to strep pyogenes
110
What are the clinical features of rheumatic fever?
- Tachycardia - Murmur - Pericardial rub - Erythema marginatum - Prolonged PR interval - Fever/fatigue - Arthritis - Chest pain/SOB - Chorea
111
What criteria is used to diagnose rheumatic fever?
Jones diagnostic criteria - 1 positive test + 2 major criteria (or 1 major + 1 minor criteria)
112
What are the investigations for rheumatic fever?
- Throat culture for group A beta-haemolytic strep (strep pyogenes) = positive - Anti-streptolysin O (ASO) = elevated - Anti-deoxyribonuclease B (anti-DNASE B) titre = elevated
113
What are the major criteria for rheumatic fever?
- Carditis (tachycardia/murmur/pericardial rub/cardiomegaly) - Polyarthritis - Erythema marginatum (red rash with raised edges and clear centre) - Sydenham's chorea - Subcut nodules
114
What are the minor criteria of rheumatic fever?
- Fever - Raised CRP/ESR - Arthralgia - Prolonged PR interval - Previous rheumatic fever
115
What are common differential diagnoses for rheumatic fever?
- Septic arthritis - Reactive arthropathy - Infective endocarditis - Myocarditis
116
What is the management for rheumatic fever?
- Bed rest until CRP is normal for 2 weeks consistently - Abx = benzylpenicillin/phenoxymethylpenicillin/amoxicillin - Aspirin/NSAIDs - Assess for emergency valve replacement - Glucocorticoids/diuretics (if severe carditis) - Haloperidol/diazepam (for chorea)
117
What is the long-term management for rheumatic fever?
Secondary prophylaxis with: - IM benzylpenicillin every 3-4 weeks - Oral phenoxymethylpenicillin BD - Oral sulfadiazine or azithromycin
118
What are risk factors for IE?
- Male - IVDU - Immunocompromised - Congenital/acquired heart disease - Abnormal valves (e.g. regurgitant/prosthetic) - Poor dental hygeine - Pacemaker - IV cannula - Previous IE
119
What are the most common cause of IE?
- Staph aureus (IVDU/diabetes/surgery) = MOST COMMON - Strep viridans (poor dental health) - Staph epidermis (prosthetic valves) - Pseudomonas aeruginosa - Strep bovis - Enterococci - Coxiella burnetii
120
What are the clinical features of IE?
- Anaemia - Splenomegaly - Clubbing - New murmur - Sepsis - Generic sx (fever/fatigue/loss of appetite/rigors/night sweats/malaise/weight loss)
121
What are the clinical features more specific to IE?
- Embolic skin lesions - Petechiae - Splinter haemorrhages - Osler nodes - Janeway lesions - Roth spots
122
What are the investigations for IE?
- Fever + new murmur = suspect IE - Echo (gold standard) = vegetation - Blood cultures - Bloods - ECG - CXR
123
What criteria is used to diagnose IE?
Modified Duke's Criteria (2 major criteria/1 major + 3 minor criteria/5 minor criteria)
124
What are the major criteria for IE?
- Pathogen grown from blood cultures - Evidence of endocarditis on echo or new valve leak
125
What are the minor criteria for IE?
- Predisposing heart condition/IVDU - Fever - Vascular phenomena (Janeway lesions/major arterial emboli/intracranial haemorrhage) - Immune phenomena (Osler's nodes/Roth spots/glomerulonephritis) - Positive blood cultures that don't meet major criteria - Echo findings consistent with IE but don't meet major criteria
126
What is the management for IE?
Abx - First line = flucloxacillin, ampicillin (rifampicin if staph) and gentamicin - MRSA = vancomycin, rifampicin and gentamicin - Benzylpenicillin and gentamicin
127
When would surgery be required in IE?
- Valve replacement - Remove/replace infected devices - Remove large vegetation at risk of embolising - If complications e.g. severe valve damage
128
What are the most common causes of UTIs in children?
- E Coli (most common) - Klebsiella - Staph saprophyticus
129
What are the risk factors for UTIs?
- Age <1 year - Females - Caucasian - Previous UTI - Voiding dysfunction - Vesicoureteral reflux - Sexual abuse - Spinal abnormalities - Constipation - Immunosuppression
130
What is the difference between cystitis and pyelonephritis?
- Cystitis - lower renal tract (bladder/urethra) - Pyelonephritis - upper renal tract (renal pelvis/kidneys)
131
What are common clinical features of UTIs in children <3 months?
- Fever - Vomiting - Lethargy - Irritability
132
What are common clinical features of UTIs in preverbal children >3 months?
- Fever - Abdominal pain/loin tenderness - Vomiting - Poor feeding
133
What are common clinical features of UTIs in verbal children >3 months?
- Frequency/dysuria - Dysfunctional voiding - Changes to continence - Abdominal pain/loin tenderness
134
What are the investigations for UTIs?
- Urine microscopy - Urine culture - Urine dipstick (leucocytes/nitrites) - Imaging (USS/dimercaptosuccinic acid/micturating cystourethrogram)
135
What is the management for UTIs in infants <3 months?
- Minimum 2-4 days IV abx followed by oral - Trimethoprim/nitrofurantoin/cephalosporin/amoxicillin
136
What is the management for UTIs in systematically well children?
- 3 days oral/IV abx - Return if no better after 24-48 hours for reassessment
137
What is the management for UTIs in systematically unwell children (fever >38 +/- loin pain/tenderness)?
- 7-10 days oral/IV abx - Ciprofloxacin/co-amoxiclav
138
What are complications of UTIs?
- Renal scarring/damage - HTN - Renal insufficiency/failure - Recurrence
139
Who is vesicoureteral reflux most common in?
Girls
140
What are the clinical features of vesicoureteral reflux?
Usually only becomes apparent following UTI: - Fever - Vomiting - Reduced appetite - Foul smelling urine - Abdominal pain whilst voiding - Urgency/frequency
141
What are the investigations for vesicoureteral reflux?
- Antenatal scan = dilated ureter - Micturating cystography (a.k.a voiding cystourethrogram) - USS KUB
142
What is the management for vesicoureteral reflux?
- Improving bladder function and infection monitoring - Abx - Surgery (rare)
143
What are causes/risk factors of primary nocturnal enuresis?
- Variation of normal development - Fhx of delayed dry nights - Overactive bladder - Fluid intake - Failure to wake - Psychological distress
144
What are causes/risk factors of secondary nocturnal enuresis?
More indicative of underlying illness - UTI - Constipation - T1DM - New psychosocial problems - Maltreatment
145
What is the management for nocturnal enuresis?
- Reassurance that it is likely to resolve - Lifestyle changes (reduce fluid intake in evenings/pass urine before bed/etc.) - Encouragement/positive reinforcement - Treat underlying cause - Enuresis alarms - Medication
146
What medication can be given for nocturnal enuresis?
- Desmopressin (ADH - reduces volume of urine produced by kidneys) - Oxybutynin (anticholinergic - reduces contractility of bladder - helpful in urge incontinence) - Imipramine (TCA - may relax bladder and lighten sleep)
147
What is nephrotic syndrome and who is it most common in?
- Increased permeability of GBM - Most common in males - Most common between ages 2-5
148
What is the most common cause of nephrotic syndrome in children?
Minimal change disease - May be secondary to intrinsic kidney disease (focal segmental glomerulosclerosis/membranoproliferative glomerulonephritis) - May be secondary to underlying systemic illness (HSP/diabetes/infection e.g. HIV/hepatitis/malaria)
149
What are the clinical features of nephrotic syndrome?
- *Proteinuria (frothy urine) - *Hypoalbuminemia - *Oedema (pitting) - Hyperlipidaemia
150
What are the investigations for nephrotic syndrome?
- Bloods (renal function/elevated lipids/low serum albumin) - Urine dipstick (proteinuria) - Urine protein:creatinine ratio - Renal biopsy
151
What is the management for nephrotic syndrome?
- Prednisolone (if steroid sensitive) - Fluid/salt restriction - Loop diuretics - Treat cause - ACEis/ARBs (to reduce protein loss)
152
What are complications of nephrotic syndrome?
- Hyperlipidaemia (give statins) - VTE (give heparin)
153
What is nephritic syndrome and what are the most common causes of it in children?
- Inflammation within kidney - Post-streptococcal glomerulonephritis - IgA nephropathy (a.k.a Buerger's disease)
154
What are the main clinical features of nephritic sydrome?
- Haematuria - Oliguria - Proteinuria - HTN
155
What are the investigations for nephritic syndrome?
- Urine dipstick = haematuria - Bloods = elevated ESR/CRP - Renal biopsy
156
What is the management for nephritic syndrome?
- Treat cause - BP control (ACEis/ARBs) - Corticosteroids
157
What are complications of nephritic syndrome?
- AKI - Decreased resistance to infection
158
What is hypospadias and what is it commonly associated with?
- Condition in which urethral meatus is abnormally displaced to the ventral side of the penis (towards scrotum) - Often associated with chordee (head of penis bends downwards) and abnormally formed foreskin
159
What is the management for hypospadias?
Surgery to correct position of meatus and straighten penis
160
What are the complications of hypospadias?
- Difficulty directing urination - Cosmetic/psychological concerns - Sexual dysfunction
161
What is haemolytic uraemic syndrome and what is the most common cause/risk factor?
- Condition in which there is thrombosis in small blood vessels throughout the body - Usually triggered by shiga toxins from either E coli or shigella - Most often affects children following an episode of gastroenteritis (loperamide increases risk)
162
What are the clinical features of haemolytic uraemic syndrome?
- Diarrhoea that turns bloody - Fever - Abdominal pain - Lethargy - Pallor - Oliguria - Haematuria - HTN - Bruising - Jaundice - Confusion
163
What are the investigations and treatment for haemolytic uraemic syndrome?
- Stool culture - Self-limiting - Supportive treatment (IV fluids/treat HTN/blood transfusion/haemodialysis)
164
What is global developmental delay indicative of?
- Down's syndrome - Fragile X syndrome - Foetal alcohol syndrome - Rett syndrome - Metabolic disorders
165
What is gross motor delay indicative of?
- Cerebral palsy - Ataxia - Myopathy - Spinal bifida - Visual impairment
166
What is fine motor delay indicative of?
- Dyspraxia - Cerebral palsy - Muscular dystrophy - Visual impairment - Congenital ataxia
167
What is language delay indicative of?
- Social circumstances (e.g. exposure to multiple languages) - Hearing impairment - Learning disability - Neglect - Autism - Cerebral palsy
168
What is personal/social delay indicative of?
- Emotional/social neglect - Parenting issues - Autism
169
Which seizures are most common in children?
Absence seizures
170
What are the clinical features of generalised tonic-clonic seizures?
- Loss of consciousness - Tonic (rigidity) and clonic (rhythmic jerking) phase - Tongue biting/incontinence/groaning/irregular breathing - Post-ictal period (confusion/drowsiness/irritability)
171
What are the clinical features of focal seizures?
- Hallucinations - Memory flashbacks - Deja vu
172
What are the clinical features of absence seizures?
- Blank/stare into space - Abruptly back to normal - Unaware of surroundings and unresponsive - Last 10-20 seconds
173
What are the clinical features of myoclonic seizures?
- Remain conscious - Sudden brief muscle contractions
174
What are the clinical features of tonic/atonic seizures?
- Sudden tension/stiffness
175
What are the investigations for epilepsy?
- EEG - MRI - Blood electrolytes/glucose/cultures/LP
176
What is the criteria required for a diagnosis of epilepsy?
- At least 2 unprovoked seizures occurring more than 24 hours apart - 1 unprovoked seizure and a probability of further seizures - At least 2 seizures in a setting of reflex epilepsy
177
What is the management for an acute seizure?
- Recovery position - Place something soft under head - Remove anything that could cause injury - Note start and end time of seizures - Call ambulance if seizures lasts >5 minutes
178
What medication is used for generalised tonic-clonic seizures?
- Sodium valproate - Lamotrigine - Carbamazepine
179
What medication is used for focal seizures?
- Lamotrigine - Levetiracetam
180
What medication is used for absence seizures?
Ethosuximide
181
What medication is used for myoclonic seizures?
- Sodium valproate - Levetiracetam
182
What medication is used for tonic/atonic seizures?
- Sodium valproate - Lamotrigine
183
When is something diagnosed with epilepsy remission?
- Individuals who had an age-dependent epilepsy syndrome but are now past the applicable age - Individuals have remained seizure-free for at least 10 years off anti-seizure medications
184
What are side effects of sodium valproate?
- Teratogenic - Liver damage - Hair loss - Tremors
185
What are side effects of carbamazepine?
- Agranulocytosis - Aplastic anaemia
186
What are side effects of ethosuximide?
- Night tremors - Rashes - N+V
187
What are side effects of lamotrigine?
- DRESS syndrome (drug reaction with eosinophilia and systemic symptoms) - Leukopenia
188
What is status epilepticus?
- Medical emergency - Seizures last >5 minutes or 2+ seizures without regaining consciousness
189
What is the management for status epilepticus?
- Secure airway - O2 - Assess cardiac/respiratory function - IV lorazepam - Buccal midazolam/rectal diazepam (community)
190
By definition, when can febrile convulsions occur?
Only in children between the ages of 6 months and 5 years
191
What are risk factors for febrile convulsions?
- Fhx - Socio-economic class - Winter season - Zinc/iron deficiency
192
What are the clinical features of simple febrile convulsions?
- Generalised tonic-clonic seizures - Last <15 minutes - Only occur once during single febrile illness
193
What are the clinical features of complex febrile convulsions?
- Focal seizures - Last >15 minutes - Occur multiple times during same febrile illness
194
What is the management for febrile convulsions (and a complication)?
- Identify/manage source of infection - Simple analgesia - Parental education *increased risk of developing epilepsy in future
195
What is eczema?
Chronic atopic condition caused by defects in the normal continuity of the skin barrier leading to inflammation in the skin
196
What are risk factors for eczema?
- Genetics/fhx - Environmental triggers (changes in temperature/dietary products/washing powders/cleaning products/emotional events/stress)
197
What are the clinical features of eczema?
- Dry, red, itchy, sore patches of skin over flexor surfaces and on face/neck - Episodic with flares
198
What is the management of eczema?
- Emollients (E45/diprobase) - Thicker emollients (cetraben/topical steroids e.g. hydrocortisone/betnovate) - Avoid hot baths/scratching/scrubbing skin - Topical tacrolimus/methotrexate - IV abx - Oral steroids
199
What is Stevens-Johnson Syndrome?
Disproportional immune response causing epidermal necrosis resulting in blistering and shedding of the top layer of the skin
200
What is the difference between Stevens-Johnson Syndrome and toxic epidermal necrolysis?
- Stevens-Johnson syndrome (SJS) - affects <10% of body surface area - Toxic epidermal necrolysis (TEN) - affects >10% of body surface area
201
What medications can cause SJS?
- Anti-epileptics - Abx - Allopurinol - NSAIDs
202
What infections can cause SJS?
- HSV - Mycoplasma pneumoniae - CMV - HIV
203
What are the clinical features of SJS?
- Purple/red rash that blisters and breaks away to leave raw tissue underneath - Pain/blistering/shedding to lips/mucous membranes - Inflammation/ulceration of eyes - Fever/cough/sore throat/itchy skin
204
What is the management for SJS?
- Supportive care - Steroids/immunoglobulins/immunosuppressants
205
What are the complications of SJS?
- Secondary infections e.g. cellulitis/sepsis - Permanent skin damage - Visual complications
206
What is the management for allergic rhinitis (IgE mediated type I hypersensitivity reaction)?
- Avoid triggers - Oral antihistamines (cetirizine/loratadine/fexofenadine/chlorphenamine/promethazine) - Nasal corticosteroids sprays (fluticasone/mometasone) - Nasal antihistamines
207
What is urticaria/angioedema?
- Urticaria - swelling of surface of skin (hives/welts) - Angioedema - swelling of deeper layers of skin caused by build-up of fluid
208
What is the management for urticaria?
- Antihistamines (fexofenadine) - Oral steroids Severe: - Antileukotrienes (montelukast) - Omalizumab (targets IgE) - Cyclosporin
209
What are the clinical features of anaphylaxis?
- Rapid onset - ABC compromise - Urticaria - Itching - SOB/wheeze - Swelling of larynx --> stridor - Tachycardia - Collapse
210
How is anaphylaxis confirmed?
Serum mast cell tryptase (highest within 6 hours of event)
211
What is the management for anaphylaxis?
- ABCDE - IM adrenaline - Antihistamines - Steroids
212
What are the most common types of birthmarks?
- Pigmented = cells containing melanin collected together in one area (brown/black) - Vascular = vessels that have not formed properly (red/blue)
213
What are causes/risk factors for nappy rash?
Friction between skin and nappy and contact with urine/faces (most common between 9-12 months) - Delayed changing of nappies - Irritant soap products/vigorous cleaning - Diarrhoea - Oral abx - Prematurity
214
What is the difference between petechiae and purpura?
- Petechiae = small/non-blanching/red spots on skin caused by burst capillaries - Purpura = large/non-blanching/red/purple macules/papules caused by leaking of blood from vessels under skin
215
What are the most common causes of a rash?
- Candida - Meningococcal septicaemia - HSP - ITP - Leukaemia - HUS - Mechanical (strong coughing/vomiting/breath holding) - Trauma - Viral illness
216
What are risk factors for GORD in infants?
Common in infants - Prematurity - Fhx - Obesity - Hiatus hernia - Neurodisability e.g. cerebral palsy
217
What are risk factors for pyloric stenosis?
- Male - First born - Fhx
218
What are the clinical features of pyloric stenosis?
- First few weeks of life - Projectile vomiting - Poor weight gain/failure to thrive - Dehydration/constipation - Firm, round, 'olive sized' mass in upper abdomen (hypertrophic pyloric muscle)
219
What are the investigations for pyloric stenosis?
- Abdominal USS (thickened pylorus) - Blood gas (hypochloremic, hypokalemic, metabolic alkalosis )
220
What is the management for pyloric stenosis?
- Correct metabolic imbalances (NaCl) - Fluids - NG tube and aspiration of stomach - Laparoscopic pyloromyotomy (Ramstedt's operation) - incision made in smooth muscle of pylorus to widen canal
221
What are common causes of gastroenteritis?
- Viral (most common) - rotavirus/norovirus/adenovirus - Bacterial - c. jejuni/e. coli - Parasitic
222
What are the clinical features of gastroenteritis?
- Sudden onset diarrhoea with/without vomiting (risk of dehydration) - Abdominal pain/cramps - Mild fever
223
When does appendicitis peak?
Peak incidence at age 10-20 years
224
What type of hernia is most common in children?
Indirect inguinal hernias (more common in boys/prematurity)
225
What are the clinical features of an inguinal/inguino-scrotal hernia?
- Cannot 'get above' mass - Reducible when lying flat - Does not transilluminate - Positive cough reflex
226
What are the main complications of a hernia?
- Recurrence - Strangulation - Incarceration - Bowel obstruction
227
Which IBD is more common in children?
Crohn's disease
228
What are the clinical features of UC?
CLOSE UP: - Continuous inflammation - Limited to colon/rectum - Only superficial mucosa affected - Smoking protective - Excrete blood/mucus - Use aminosalicylates - PSC
229
What are the clinical features of Crohn's?
NESTS: - No blood/mucus - Entire GI tract - Skip lesions - Terminal ileum most affected/transmural inflammation - Smoking is a RF
230
What are the investigations for IBD?
- GOLD STANDARD = Endoscopy with biopsy - Raised faecal calprotectin - Raised CRP
231
What medications are used for Crohn's?
- Steroids (oral prednisolone/IV hydrocortisone) - Immunosuppressant (azathioprine/methotrexate/infliximab/adalimumab/mercaptopurine)
232
What medications are used for UC?
- Aminosalicylates (mesalazine) - Steroids (prednisolone/hydrocortisone) - IV ciclosporin - Azathioprine/mercaptopurine
233
What are the investigations for coeliac disease?
- Must be carried out whilst patient remains on gluten-containing diet - Antibodies (anti-TTG and anti-EMA) - Endoscopy and biopsy (crypt hypertrophy/villous atrophy)
234
What are common causes of poor weight gain/failure to thrive?
- Inadequate nutritional intake (maternal malabsorption/neglect/poverty) - Difficulty feeding (cleft lip/palate/pyloric stenosis) - Malabsorption (CF/coeliac/IBD) - Increased energy requirements (hyperthyroidism/malignancy/HIV) - Inability to process nutrition (T1DM)
235
What is the difference between marasmus and kwashiorkor?
- Marasmus = result of diet with total calorie insufficiency - Kwashiorkor = result of diet with sufficient calorie intake but insufficient protein intake (worse prognosis)
236
What are the clinical features marasmus/kwashiorkor?
- Wasting - Loss of body fat/muscle - Stunted growth - Kwashiorkor = bilateral pitting oedema/ascites - Marasmus = inadequate weight relative to height
237
What is Hirschsprung's?
Congenital condition in which nerve cells (parasympathetic ganglion cells) of the myenteric plexus (a.k.a Auerbach's plexus) are absent in the distal bowel and rectum
238
What are risk factors for Hirschsprung's?
- Male - Fhx - Other conditions (Down's/neurofibromatosis/etc.)
239
What are the clinical features of Hirschsprung's?
- Failure to pass meconium - Empty rectal vault - Palpable faecal mass in left lower abdomen (DRE may ease sx) - Abdominal pain/distension - Vomiting - Poor weight gain/failure to thrive
240
What is Hirschsprung-associated enterocolitis?
Life threatening condition - inflammation/obstruction of intestine - Presents with 2-4 weeks of birth - Fever - Abdominal distension - Diarrhoea with blood - Features of sepsis
241
What are the investigations for Hirschsprung's?
- AXR - Rectal suction biopsy (absence of ganglionic cells) - Contrast enema (short transition zone between proximal/distal colon and small rectal diameter)
242
What is the management for Hirschsprung's?
- Fluid resus - Bowel decompression - NG tube - IV abx - Surgery (Swenson, Soave, Dunhamel pull through surgery - remove aganglionic section of bowel)
243
What is the difference between intussusception and malrotation/volvulus?
Intussusception - one piece of bowel telescopes inside another leading to ischaemia and bowel obstruction Malrotation and volvulus - twisting loop of bowel leading to intestinal obstruction
244
What are risk factors for intussusception?
- CF - Meckel's diverticulum - HSP - Rotavirus vaccine >23 weeks
245
Who is intussusception most common in?
- Most common cause of obstruction in neonates - 3 months-3 years (most commonly <1) - Most common in distal ileum at ileocecal junction
246
What are the clinical features of intussusception?
- Colic abdominal pain/distension - Pallor - Sausage-shaped mass palpable in RUQ - Redcurrant jelly stools - Shock - Peritonitis (guarding/rigidity/pyrexia)
247
What are the investigations for intussusception?
- USS (target-shaped mass) - AXR (distended small bowel/absence of gas in large bowel)
248
What are the clinical features of malrotation/volvulus?
- Abdominal pain - Bilious vomiting - Caecum at midline - Reflux sx
249
What are the investigations for malrotation/volvulus?
- Barium enema - AXR with contrast (double bubble sign - dilated stomach and proximal small bowel)
250
What is the management for intussusception/malrotation/volvulus?
- IV fluids - Air insufflation/air enema (intussusception) - Surgery - Ladd's procedure (malrotation) - Broad spectrum abx e.g. gentamicin
251
What is Meckel's Diverticulum?
Congenital diverticulum of small intestine containing ilea/gastric/pancreatic mucosa
252
What are the clinical features of Meckel's Diverticulum?
- Abdominal pain - Rectal bleeding
253
What are the investigations for Meckel's Diverticulum?
- X-ray - USS - Laparoscopy
254
What is the management of Meckel's Diverticulum?
- Surgical removal/resection
255
What are complications of Meckel's Diverticulum?
- Risk of peptic ulceration - Obstruction due to intussusception/volvulus
256
What is colic?
Frequent, prolonged and intense crying or fussiness in a healthy infant (usually worst between 4-6 weeks)
257
What are the clinical features of colic?
- Hard to soothe/settle baby - Clenched fists - Red in face - Curling up legs/arching back
258
Who is biliary atresia more common in?
- Females - Associated with CMV
259
What are the clinical features of biliary atresia?
- Jaundice - Dark urine/pale stools - Disturbed appetite - Hepatosplenomegaly - Abnormal growth
260
What are the investigations for biliary atresia?
- Serum bilirubin = high conjugated bilirubin - Raised LFTs - Alpha 1 antitrypsin (rule out) - Sweat test (rule out CF) - USS
261
What is the management for biliary atresia?
Surgery (Kasai Portoenterostomy) - section of SI attached to opening of liver (where bile duct normally attaches)
262
What is Kawasaki's disease?
Mucocutaneous lymph node syndrome - systemic medium-sized vessel vasculitis
263
Who is Kawasaki's disease most common in?
- Typically <5 years - Asian children, particularly Korean/Japanese - Males
264
What are the clinical features of Kawasaki's disease?
- Persistent high fever >39 C for >5 days - Widespread erythematous maculopapular rash - Desquamation on palms/soles - Strawberry tongue - Cracked lips - Cervical lymphadenopathy - Bilateral conjunctivitis
265
What are the investigations for Kawasaki's disease?
- Bloods (FBC/LFTs/ESR/CRP) - Urinalysis (raised WCC without infection) - Echo
266
What is the management for Kawasaki's disease?
- High dose aspirin (usually avoided in children but Kawasaki's is one of exceptions) - IV immunoglobulins
267
What is the most common complication of Kawasaki's disease?
Coronary artery aneurysm
268
What are the clinical features of measles?
- Sx start 10-12 days after exposure - Fever/coryzal sx - Conjunctivitis - Koplik spots (blue/white spots on inside of cheek) - Maculopapular rash
269
What is the management for measles?
- Self-resolving - Supportive treatment - Avoid school - Notify public health - Vaccination
270
What is the cause of chickenpox and what is the incubation period?
- Varicella zoster virus (VZV) - 21 days
271
What are the clinical features of chickenpox?
- Widespread, erythematous, raised, vesicular, blistering lesions - Fever - Itch - General fatigue/malaise
272
What is the management for chickenpox?
- Self-limiting - Calamine lotion - Avoid school until lesions have crusted over
273
What are the clinical features of rubella?
- Low grade fever - Erythematous maculopapular rash - Joint pain - Sore throat - Lymphadenopathy
274
What is the management for rubella?
- Self-limiting - Avoid school - Notify public health
275
What is a complication of rubella in pregnancy?
Congenital rubella syndrome: - Deafness - Blindness - Congenital heart disease
276
What is scalded skin syndrome?
Condition caused by a type of staph aureus that produces epidermolytic toxins (enzymes that break down proteins that hold skin cells together)
277
Who is scalded skin syndrome most common in?
Children <5 years
278
What are the clinical features of scalded skin syndrome?
- Generalised patches of erythema on skin - Skin looks thin/wrinkled - Formation of bullae - Fever - Irritability - Lethargy - Dehydration - Nikolsky sign (areas of epidermis separate on gentle pressure leaving denuded areas of skin which dry and heal without scarring)
279
What is the management for scalded skin syndrome?
- IV abx - Analgesia - Fluid and electrolyte balance
280
What are the clinical features of TB?
- Asx - Lethargy - Fever/night sweats - Weight loss - Cough with/without haemoptysis - Hilar lymphadenopathy
281
What are the investigations for TB?
- Ziehl-Neelsen stain (bright red) - Mantoux test (tuberculin injected) - CXR (patchy consolidation/pleural effusions/hilar lymphadenopathy) - Bacterial cultures - IGRA (blood test)
282
What are the complications of TB medication?
- Rifampicin = red/orange discolouration of secretions - Isoniazid = peripheral neuropathy - Pyrazinamide = hyperuciaemia --> gout ^ all associated with hepatotoxicity - Ethambutol = colour blindness/reduced visual acuity
283
What is the management for TB?
- BCG vaccine - Contact tracing RIPE: - Rifampicin for 6 months - Isoniazid for 6 months - Pyrazinamide for 2 months - Ethambutol for 2 months
284
What are the clinical features of HIV?
- Most remain asx - Lymphadenopathy (mild) - Recurrent bacterial infections (moderate) - Chronic diarrhoea (moderate) - Lymphocytic interstitial pneumonitis (moderate) - Pneumocystis jirovecii pneumonia (severe) - Severe faltering growth (severe) - Encephalopathy (severe)
285
What are the investigations for HIV?
- Antibody screen - Viral load
286
What is the management for HIV?
- Antiretroviral therapy - Normal childhood vaccines - Prophylactic co-trimoxazole - Prophylactic zidovudine/lamivudine/nevirapine
287
What are the common causes of bacterial meningitis in children?
- Neonates = group B strep - Children <2 = strep pneumoniae
288
What are the common causes of viral meningitis in children?
- Enteroviruses (echovirus/coxsackie) - HSV - VZV
289
What are the common causes of fungal meningitis in children?
- Cryptococcus - Candida
290
What are the clinical features of meningitis?
- Non-blanching rash (meningococcal septicaemia) - Fever - Neck stiffness - Vomiting - Headache - Photophobia - Altered consciousness - Seizures
291
What are the investigations for meningitis?
- Lumbar puncture for CSF - Blood cultured - Meningococcal PCR - Kernig's test - Brudzinski's test
292
What is the difference between CSF in bacterial vs viral meningitis?
- Bacterial = cloudy/high protein/low glucose/neutrophils/culture - Viral = clear/normal protein/normal glucose/lymphocytes
293
What is the management for meningitis?
- Bacterial (community) = stat IM injection of benzylpenicillin - Bacterial (hospital) = IV cefotaxime + IV amoxicillin - Post exposure prophylaxis = ciprofloxacin - Viral = aciclovir - Notify public health
294
What are common causes of encephalitis in children?
- HSV1 (children - cold sores) - HSV2 (neonates - genital herpes contracted during birth) - VZV - CMV
295
What are the investigations for encephalitis?
- LP for CSF - CT - MRI - HIV testing
296
What is the management for encephalitis?
- Aciclovir (HSV/VZV) - Ganciclovir (CMV)
297
What is the cause and risk factors for slapped cheek syndrome?
- Parvovirus B19 virus - Immunocompromised - Pregnant - Haematological conditions (sickle cell anaemia/thalassaemia)
298
What are the clinical features of slapped cheek syndrome?
- Asx - Mild fever - Coryza - Non specific sx e.g. muscle aches/lethargy - Rash after 2-5 days (diffuse bright red on both cheeks followed by reticular mildly erythematous rash)
299
What are the investigations for slapped cheek syndrome?
- Serology testing for parvovirus - FBC/reticulocyte count
300
What is the management for slapped cheek syndrome?
- Self-limiting - Supportive treatment
301
What are common causes of impetigo?
- Staph aureus (most common) - causes bullous impetigo which is most common in neonates and children <2 - Strep pyogenes
302
What are the clinical features of impetigo?
Golden crust = characteristic Non-bullous: - Typically around nose/mouth - Exudate from lesions dries to form golden crust - Few/non systemic sx Bullous: - Fluid-filled vesicles - Vesicles grow and burst to form golden crust - Painful/itchy - Systemic sx (fever)
303
What are the investigations for impetigo?
Swabs
304
What is the management for impetigo?
- Topical fusidic acid - Antiseptic cream - Oral flucloxacillin - Avoid school
305
What is the main complication of impetigo?
Scalded skin syndrome
306
What is toxic shock syndrome?
Life-threatening condition caused by an infection (toxin producing staph aureus and group A strep)
307
What are the clinical features of toxic shock syndrome?
- Fever >39 C - Hypotension - Diffuse erythematous macular rash - Mucositis (conjunctivae/oral mucosa/genital mucosa) - Vomiting and diarrhoea
308
What is the management for toxic shock syndrome?
- Intensive care support - Abx (e.g. ceftriaxone + clindamycin)
309
What is the cause of scarlet fever and who is it most common in?
- Toxic producing strains of strep pyogenes - Common in those between 2-8 years - Higher risk in neonates/immunocompromised/concurrent chickenpox/influenza
310
What are the clinical features of scarlet fever?
- Sore throat/fever/fatigue/headache - N+V - Pinpoint, sandpiper-like, blanching rash - Strawberry tongue - Cervical lymphadenopathy
311
What is the management for scarlet fever?
- Oral abx e.g. benzylpenicillin for 10 days - Notify public health
312
What is Coxsackie's disease?
a.k.a hand, foot and mouth disease - caused by Coxsackie A virus
313
What are the clinical features of hand, foot and mouth disease?
- Typical viral URTI sx (sore throat/fever/cough) - Small mouth ulcers - Blistering red spots across body - Itchy
314
What is the management for hand, foot and mouth disease?
- Self-limiting - Supportive management
315
What are risk factors for undescended testes (cryptorchidism)?
- Fhx - Low birth weight - Small for gestational age - Prematurity - Maternal smoking
316
What is the management for undescended testes?
- Watch and wait (will usually descend in first 3-6 months) - Surgical orchidopexy
317
What are complications of undescended testes?
Higher risk of: - Testicular torsion - Infertility - Testicular cancer
318
What is a risk factor for testicular torsion?
Bell-Clapper deformity: - Absence of fixation between testicle and tunica vaginalis - Testicle hangs in horizontal position - Able to rotate within tunica vaginalis, twisting at spermatic cord
319
What are the clinical features of testicular torsion?
- Rapid onset unilateral testicular pain - Abdominal pain - Vomiting - Firm, swollen testicle - Elevated/retracted testicle - Absent cremasteric reflex - Abnormal testicular lie - Prehn's sign negative
320
What is the investigation for testicular torsion?
Scrotal USS - whirlpool sign (spiral appearance of spermatic cord and blood vessels)
321
What is the management for testicular torsion?
- NBM - Analgesia - Orchiopexy (within 6 hours) - Orchidectomy if delayed surgery/necrosis
322
What is pica?
Eating disorder in which patients eat things not usually considered food e.g. dirt/clay/rocks/paper/crayons/hair/chalk
323
Who is pica more common in?
- ASD - Intellectual disabilities - OCD - Schizophrenia - Malnutrition/hunger - Stress (i.e. due to abuse/neglect)
324
What is the criteria for pica?
- Eating non-food items - Doing so for >1 month - Abnormal behaviour for child's age/developmental stage - Has risk factor
325
What are complications of pica?
- Iron deficiency anaemia - Lead poisoning - Constipation/diarrhoea - Intestinal obstruction - Mouth/teeth injuries
326
What are common causes of hypothyroidism?
Congenital: - Dysgenesis (underdeveloped thyroid gland) - Dyshormonogenesis (thyroid gland not producing enough hormone) Acquired: - Hashimoto's thyroiditis
327
What are the clinical features of hypothyroidism?
- Prolonged neonatal jaundice - Poor feeding/growth - Constipation - Increased sleeping/fatigue - Weight gain
328
What are the investigations for hypothyroidism?
- Newborn blood spot screening test - TFTs - Thyroid USS - Thyroid antibodies (anti-TPO/anti-thyroglobulin)
329
What is the management for hypothyroidism?
Levothyroxine
330
What is the most common cause of congenital adrenal hyperplasia?
Congenital deficiency of 21-hydroxylase enzyme (autosomal recessive)
331
Describe the pathophysiology of congenital adrenal hyperplasia?
- Deficiency of 21-hydroxylase (enzyme responsible for converting progesterone into aldosterone/cortisol) - Progesterone unable to become aldosterone/cortisol so more converted into testosterone
332
What are the clinical features of congenital adrenal hyperplasia in females?
- Tall for age - Facial hair - Absent periods - Deep voice - Early puberty - Virilised (ambigious) genitalia and enlarged clitoris
333
What are the clinical features of congenital adrenal hyperplasia in males?
- Tall for age - Deep voice - Large penis - Small testicles - Early puberty
334
What are the investigations/management for congenital adrenal hyperplasia?
- Low aldosterone --> replacement (fludrocortisone) - Low cortisol --> replacement (hydrocortisone) - High testosterone - Corrective surgery for virilised genitalia
335
What is Kallmann syndrome?
Genetic condition causing hypogonadotropic hypogonadism resulting in failure to start puberty
336
What are the clinical features of Kallmann syndrome?
- Anosmia - Delayed/incomplete puberty - Undescended/partially descended testicles - Small penile size - Facial defects e.g. cleft lip/palate - Short fingers/toes
337
What are the investigations and management for Kallmann syndrome?
- Bloods/MRI hypothalamus/pituitary gland - Hormone replacement therapy
338
Who does androgen insensitivity syndrome affected and how?
- Only affects genetically male people - X-linked recessive - Mutation in androgen receptor gene - Cells unable to respond to androgen hormone so converted into oestrogen --> female characteristics
339
What are the clinical features of androgen insensitivity syndrome?
- Female phenotype (breast tissue/female external genitalia) - Testes in abdomen/inguinal canal --> inguinal hernias - Absence of uterus/upper vagina/cervix/fallopian tubes/ovaries - Lack of pubic/facial hair - Taller than female average - Infertile - Primary amenorrhoea
340
What are the investigations for androgen insensitivity syndrome?
- Raised LH - Normal/raised FSH - Normal/raised testosterone - Raised oestrogen
341
What is the management for androgen insensitivity syndrome?
- Bilateral orchidectomy (increased risk of testicular cancer if not removed) - Oestrogen therapy - Vaginal dilators/vaginal surgery - Patients generally raised as female
342
What bone tumours are most common in children?
- Osteogenic sarcoma (osteosarcoma) - Ewing's sarcoma more common in younger children
343
What are the clinical features of bone tumours?
- Patients generally well - Persistent/localised bone pain - Bone swelling - Palpable mass - Restricted joint movements
344
What are the investigations for bone tumours?
- XRAY (poorly defined lesions in bone with destruction of normal bone and 'fluffy' appearance) - XRAY (periosteal reaction - irritation of lining of bone - 'sun burst' appearance) - Bloods (raised ALP) - CT/MRI/PET - Bone scan - Bone biopsy
345
What is the management/complications for bone tumours?
- Surgery (resection/amputation) - Chemotherapy/radiotherapy - Pathological bone fractures - Metastasis
346
What are risk factors for brain tumours in children and how common are they?
- Fhx - Neurofibromatosis - Tuberous sclerosis - Leading cause of childhood cancer deaths in UK - almost always primary tumour
347
What are the 5 main types of brain tumours in children?
- Astrocytoma - Medulloblastoma - Ependymoma - Craniopharyngioma - Brainstem glioma
348
What are the clinical features of brain tumours?
- Raised ICP (headache/coughing/papilloedema) - Focal neurological signs - Behavioural changes/altered GCS - Back pain - Peripheral weakness of arms/legs - Bladder/bowel dysfunction
349
What are the investigations/management/complications of brain tumours?
- MRI - Surgery (+/- chemotherapy/radiotherapy) - Metastasis
350
What are the clinical features of hepatoblastomas?
- Abdominal distension/mass - Pain - Jaundice
351
What are the investigations and management for hepatoblastomas?
- Elevated a-fetoprotein - Good prognosis - Surgery/chemotherapy/liver transplant
352
Which types of leukaemia are most common in children?
- Acute lymphoblastic leukaemia (ALL) - peaks in ages 2-3 years - Acute myeloid leukaemia (AML) - peaks ages <2
353
What are the clinical features of leukaemia?
- Cancer sx (fatigue/weight loss/night sweats/etc.) - Petechiae/abnormal bruising/bleeding - Hepatosplenomegaly - Lymphadenopathy
354
What are the investigations for leukaemia?
- FBC (anaemia/leukopenia/thrombocytopenia/high WCC) - Blood film (blast cells) - BM biopsy - Lymph node biopsy - CXT/CT/LB (staging)
355
What is the management for leukaemia?
- Chemotherapy/radiotherapy - Surgery/BM transplant
356
What is a neuroblastoma and when does it usually present?
- Tumour arising from neural crest tissue in adrenal medulla and sympathetic nervous system - Usually occurs before 5 years of age
357
What are the clinical features of neuroblastomas?
- Abdominal mass - Weight loss - Pallor - Hepatomegaly - Lymphadenopathy - Periorbital bruising - Skin nodules
358
What are the investigations/management/complications of neuroblastomas?
- Raised urinary catecholamine levels - Biopsy/BM sampling - Generally good prognosis - Surgery (+/- chemotherapy/radiotherapy) - Metastasis
359
When do retinoblastomas usually present and what are the clinical features?
- Most cases present in first 3 years of life - White pupillary reflex replaces red one - Squint
360
What are the investigations/management/complications of retinoblastomas?
- MRI - Preserve vision/chemotherapy/laser treatment - Visual impairment/secondary malignancy
361
What is Wilms tumour and who is it most common in?
- Nephroblastoma (renal tumour) - Most common renal tumour in children - Often presents before 5 years
362
What are the clinical features of Wilms tumours?
- Large abdominal mass - Abdominal pain - Weight loss - Haematuria - HTN - Lethargy/fever
363
What are the investigations/management/complications of Wilms tumours?
- USS - CT/MRI - Biopsy - Chemotherapy/surgery (nephrectomy) - Metastasis
364
What is Angelman syndrome?
Genetic condition caused by loss of function of UBE3A gene on chromosome 15
365
What are the clinical features of Angelman syndrome?
- Delayed development/learning disability - Severe delay/absence of speech development - Ataxia - Fascination with water - Happy demeanour/inappropriate laughter - Hand flapping - Wide mouth with widely space teeth - Microcephaly - ADHD/epilepsy
366
What is Edward's Syndrome and what are the clinical features?
Trisomy 18 - Low birthweight - Small mouth/chin - Short sternum - Flexed/overlapping fingers - Rocket-bottom feet - Cardiac/renal malformations
367
What is Klinefelter syndrome and who is affected?
Additional X chromosome ONLY IN MEN - 47 XXY - 48 XXXY (rare) - 49 XXXXY (rare)
368
What are the clinical features of Klinefelter syndrome?
- Tall stature - Wide hips - Gynaecomastia - Weaker muscles - Small testicles - Reduced libido - Shyness - Subtle learning difficulties (particularly speech/language)
369
What is the management for Klinefelter syndrome?
- Testosterone injections - Breast reduction surgery - Speech and language therapy - Occupational therapy - Physiotherapy
370
What are the complications of Klinefelter syndrome?
- Infertility - Increased risk of breast cancer/osteoporosis/diabetes/anxiety and depression
371
What is Turner syndrome and who is affected?
Single X chromosome ONLY IN WOMEN - 45XO
372
What are the clinical features of Turner syndrome?
- Short stature - Webbed neck - High arching palate - Downward sloping eyes with ptosis - Broad chest with widely spaced nipples - Cubitus valgus - Overdeveloped ovaries - Late/incomplete puberty - Ejection systolic murmur
373
What is the management of Turner syndrome?
- Growth hormone therapy - Oestrogen/progesterone
374
What are the complications of Turner syndrome?
- Infertility - Associated with recurrent otitis media/UTIs/coarctation of aorta/aortic stenosis/hypothyroidism/HTN/obesity/diabetes/osteoporosis/learning disabilities
375
What are the most common types of muscular dystrophy and when do they present?
- Duchenne's = 3-5 years - Becker's = 8-12 years - Myotonic dystrophy - Facioscapulohumeral muscular dystrophy - Oculopharyngeal muscular dystrophy - Limb-girdle muscular dystrophy - Emery-Dreifuss muscular dystrophy
376
Describe the pathophysiology of Duchenne's/Becker's muscular dystrophy
- X linked recessive - Defective gene for dystrophin (protein that helps to hold muscles together) - Becker's = gene less severely affected than Duchenne's
377
What are the clinical features of muscular dystrophy?
- Gower's sign (stand up from lying down using a specific technique) - Progressive weakness - Waddling gait - Language delay - Pseudohypertrophy of calves - Slow/clumsy
378
What is the management for Duchenne's/Becker's muscular dystrophy?
- Exercise - Night splints/passive stretching - Oral steroids - Creatinine supplementation
379
What is the life expectancy of Duchenne's muscular dystrophy?
- 25-35 years - Usually due to cardiac/respiratory complications (dilated cardiomyopathy)
380
What are the clinical features of Noonan syndrome?
- Short stature - Broad forehead - Downward sloping eyes with ptosis - Wide space between eyes - Low set ears - Webbed neck - Widely spaced nipples
381
What are the complications of Noonan syndrome?
Associated with: - Congenital heart disease (pulmonary stenosis/HCM/ASD) - Cryptorchidism --> infertility - Learning disability - Bleeding disorders - Lymphoedema - Increased risk of leukaemia/neuroblastoma
382
What is Patau syndrome and what are the clinical features?
Trisomy 13 - Structural defects of brain - Scalp defects - Small eyes/eye defects - Cleft lip/palate
383
What is Prader Will Syndrome?
Genetic condition caused by loss of functional genes of proximal arm of chromosome 15
384
What are the clinical features of Prader Willi Syndrome?
- Constant insatiable hunger --> obesity - Hypotonia - Learning disability - Soft skin prone to bruising - Anxiety - Dysmorphic features - Narrow forehead - Almond shaped eyes - Squint - Thin upper lip - Hypogonadism
385
What is Williams Syndrome?
Genetic condition caused by deletion of genetic material on one copy of chromosome 7
386
What are the clinical features of Williams Syndrome?
- Elfin facies - Broad forehead - Starburst eyes (star-like pattern on iris) - Flattened nasal bridge - Long philtrum - Wide mouth with widely spaced teeth - Small chin - Very social trusting personality - Mild learning disability
387
What are complications of Williams Syndrome?
Associated with: - Supravalvular aortic stenosis/pulmonary stenosis - ADHD - HTN - Hypercalcaemia
388
What are risk factors for developmental dysplasia of hip?
- Fhx - Breech presentation - Multiple pregnancy - Oligohydramnios
389
What are the clinical features of developmental dysplasia of hip?
- Hip asymmetry - Reduced range of motion in hip - Limp/abnormal gait - Weakness - Recurrent subluxation/dislocation - Early degenerative changes
390
What are the investigations for developmental dysplasia of hip?
- Newborn examination - Leg length - Restricted hip abduction on one side - Difference in knee level when hips flexed - Ortolani and Barlow tests - clicking - USS - XRAY
391
What is the management for developmental dysplasia of hip?
- Pavlik harness - Surgery
392
What is discoid meniscus and who is it most common in?
- Congenital anatomical defect of menisci (2 pads of cartilage in knee joint) - Usually lateral meniscus affected - More common in Asian people
393
What are the clinical features of discoid meniscus?
- Can be asx - Activity related pain - Effusions - Joint line tenderness - Mechanical sx - Increased incidence of tears - Instability in meniscus --> snapping knee
394
What are the investigations/management for discoid meniscus?
- XRAY - MRI - Observation/conservative management - Surgery if tears/instability
395
Which types of juvenile idiopathic arthritis are more common in children?
- Oligoarticular JIA = more common in girls <6 years - Enthesitis related arthritis = more common in boys >6 years (can be caused by traumatic stress e.g. repetitive strain during sports)
396
Briefly describe the main types of JIA
- Systemic JIA - Polyarticular JIA = 5+ joints, inflammatory - tends to be more symmetrical - Oligoarticular JIA = 4 or less joints, usually only affects single joint, tends to affect larger joints e.g. knee/ankle - Enthesitis related arthritis = inflammatory arthritis and enthesitis (inflammation of point where tendon inserts into bone) - Juvenile psoriatic arthritis
397
What are the clinical features of JIA?
- Joint pain - Swelling - Stiffness
398
What are the clinical features of systemic JIA?
- Subtle pink rash - High swinging fevers - Enlarged lymph nodes - Weight loss - Joint inflammation/pain - Splenomegaly - Muscle pain - Pleuritis/pericarditis
399
What are the clinical features of polyarticular JIA?
- Mild fever - Anaemia - Reduced growth
400
What are the clinical features of oligoarticular JIA?
- Anterior uveitis
401
What are the clinical features of enthesitis related arthritis?
- Signs of psoriasis/IBD - Anterior uveitis - Tender to localised palpation of entheses
402
What are the clinical features of juvenile psoriatic arthritis?
- Associated with psoriasis - Plaques of psoriasis - Nail pitting - Onycholysis - Dactylitis - Enthesitis
403
What criteria is required for a diagnosis of JIA?
- Arthritis without any other cause - Lasts >6 weeks - Patient <16 years
404
What are the investigations for systemic JIA?
- ANA = -ve - RF = -ve - Raised CRP/ESR/platelets/serum ferritin
405
What are the investigations for polyarticular JIA and juvenile psoriatic arthritis?
RF = -ve
406
What are the investigations for oligoarticular JIA?
- ANA = +ve - RF = -ve - Inflammatory markers normal/mildly elevated
407
What are the investigations for enthesitis related arthritis?
- MRI - Majority have HLA-B27 gene
408
What is the management for JIA?
- NSAIDs - Oral/IM/intra-articular steroids - DMARDs (methotrexate/sulfasalazine/leflunomide) - Biological therapy (etanercept/infliximab/adalimumab - TNF inhibitors)
409
What is Osgood-Schlatter Disease and who is it most common in?
- Inflammation at tibial tuberosity where patellar ligament inserts - Common cause of anterior knee pain in adolescents - Typically occurs in males aged 10-15
410
What are the clinical features of Osgood-Schlatter Disease?
- Visible/palpable lump below knee (initially tender due to inflammation but becomes hard/non-tender) - Gradual onset of sx - Pain in anterior aspect of knee - Pain exacerbated by physical activity/kneeling/extension of knee
411
What is the management of Osgood-Schlatter Disease?
- Sx usually resolve over time - Reduction in physical activity - Ice - NSAIDs - Stretching/physiotherapy
412
What is osteogenesis imperfecta?
Autosomal dominant genetic condition resulting in brittle bones that are prone to fractures
413
What are the clinical features of osteogenesis imperfecta?
- Recurrent/inappropriate fractures - Joint/bone pain - Blue/grey sclera - Hypermobility - Triangular face - Deafness - Dental problems - Bone deformities e.g. bowed legs/scoliosis
414
What are the investigations/management of osteogenesis imperfecta?
- XRAY - Genetic testing - Bisphosphonates - Vit D supplementation - Physiotherapy/occupational therapy - Management of fractures
415
What is osteomyelitis, what is the most common cause and who is it most common in?
- Infection in bone and bone marrow - Staph aureus - More common in males <10 years/open bone fractures/orthopaedic surgery/immunocompromised/sickle cell anaemia/HIV/TB
416
What are the clinical features of osteomyelitis?
- Systemic sx - Refusal to use limb/weight bear - Pain - Swelling - Tenderness
417
What are the investigations for osteomyelitis?
- XRAY - MRI (gold standard) - Bone scan - Bloods (CRP/ESR/WCC) - Blood cultures - BM aspiration
418
What is the management/complications of osteomyelitis?
- Abx therapy - Surgery - drainage/debridement of infected bone - Complication = septic arthritis
419
What is Perthes Disease and who is it most common in?
- Disruption of blood flow to the femoral head causing avascular necrosis of the bone - Usually idiopathic - Most common in boys aged 5-8 years
420
What are the clinical features of Perthes Disease?
- Slow onset of pain in hip/groin - Limp - Restricted hip movements - Referred pain to knee - No history of trauma - Hyperactivity and short stature
421
What are the investigations/management for Perthes Disease?
- XRAY/bloods/MRI/technetium bone scan - Conservative management (bed rest/traction/crutches/analgesia/physiotherapy) - Surgery
422
What is rickets?
Condition in which there is defective bone mineralisation causing soft and deformed bones
423
What are the causes and risk factors for rickets?
- Vit D/calcium deficiency - Hereditary hypophosphatemic rickets - RF = darker skin/low exposure to sunlight/colder climates/lack of time spent outdoors/malabsorption disorders
424
What are the clinical features of rickets?
- Lethargy - Bone pain - Poor growth - Dental problems - Muscle weakness - Bone deformities (bowed legs/knock knees/rachitis rosary/craniotabes/delayed teeth)
425
What are the investigations for rickets?
- Serum 25-hydroxyvitamin D - XRAY - Low serum calcium/phosphate - High serum ALP/PTH - Bloods (FBC/ESR/CRP/LFTs/TFTs) - Malabsorption screen
426
What is the management for rickets?
- Prevention (400IU supplements) - Ergocalciferol - Vitamin D/calcium supplementation
427
What is scoliosis and what are the clinical features?
Lateral curvature of spine - One shoulder higher than other - One shoulder blade more prominent than other - One hip more prominent than other (uneven waistline) - Changed position of spine/chest/pelvis
428
What are the investigations/management/complications of scoliosis?
- Visible curvature/XRAY/MRI - Observation/bracing/surgery - Lung/heart problems
429
What are common causes of septic arthritis and who is it most common in?
- Staph aureus - N. gonorrhoea/group A strep/haemophilus influenzae/e. coli - Most common in children <4 years
430
What are the clinical features of septic arthritis?
- Usually one joint affected (knee/hip) - Rapid onset - Hot/red/swollen/painful joint - Refusal to weight bear - Stiffness/reduced ROM - Systemic sx (fever/lethargy/sepsis)
431
What are the investigations/management of septic arthritis?
- Joint aspiration sent for gram staining/crystal microscopy/culture/abx sensitivities - Aspirate joint + empiric IV abx + specific abx - Surgical drainage and washout
432
What is slipped femoral epiphysis and who is it most common in?
- Head of femur displaced along growth place - More common in males/obese children/ages 8-15 years
433
What are the clinical features of slipped femoral epiphysis?
- Adolescent, obese male undergoing a growth spurt - History of minor trauma which may trigger onset of sx - Hip/groin/thigh/knee pain - Restricted hip ROM/movement - Painful limp
434
What are the investigations/management of slipped femoral epiphysis?
- XRAY/bloods/CT/MRI/technetium bone scan - Surgery to return femoral head to correct position and fix in place
435
What is torticollis?
Persistent tilting of the head to one side
436
What are the causes of congenital torticollis?
Associated with birth trauma/antenatal complications - Congenital muscular torticollis (caused by shortening and fibrosis of sternocleidomastoid) - Cervical spine malformations - Chiari malformations - Spina bifida
437
What are the causes of acquired torticollis?
Typically occurs after 4-6 months - Usually results from SCM/trapezius muscle injury/inflammation - Retropharyngeal abscess - C-spine injury - CNS tumour - Spinal epidural haematoma
438
What are the clinical features of torticollis?
- Chin pointing towards opposite side/ear and head tilted to affected shoulder Red flag symptoms: - Neck stiffness - Fever/drooling/vomiting - Pain increasing/unremitting/disturbing sleep - Recent trauma - Repeated hospital attendances with persistence of sx - Gait disturbance - History of headaches/change in behaviour
439
What are the investigations/management of torticollis?
- XRAY/CT/MRI/USS neck - Physiotherapy/analgesia/treat underlying cause
440
Why might resuscitation be necessary in neonates?
- Large SA:weight ratio --> get cold easily - Babies are born wet --> lose heat rapidly - If born through meconium, may have it in mouth/airway
441
What are the steps to resuscitation of babies?
- Warm baby - Calculate APGAR score - Stimulate breathing - Inflation breaths - Chest compressions - Severe situations - Delayed umbilical cord clamping
442
How are babies warmed in neonatal resuscitation?
- Vigorous drying - Warm delivery room/heat lamp - Placed in plastic bag
443
What is an APGAR score?
Used as an indicator of neonatal resuscitation progression - scored 0-2 - Appearance (skin colour) - Pulse - Grimmace (response to stimulation) - Activity (muscle tone) - Respiration
444
How is breathing stimulated in neonatal resuscitation?
- Dry vigorously - Place head in neutral position to keep airway open - Check for airway obstruction - consider aspiration
445
How are chest compressions and inflation breaths given in neonatal resuscitation?
- 2 cycle of 5 inflation breaths lasting 3 seconds each - Chest compression with ventilation breaths if no response - 3:1 ratio
446
What severe situations may be required in neonatal resuscitation?
- IV drugs - Intubation - Therapeutic hypothermia with active cooling
447
What is the significance of delayed umbilical cord clamping in neonatal resuscitation?
- Blood in placenta after birth - Delayed clamping allows time for blood to enter neonate's circulation (placental transfusion)
448
What are the effects of delayed umbilical cord clamping?
- Improve Hb - Improved iron - Improved BP - Reduced intraventricular haemorrhage risk - reduced necrotising enterocolitis risk - Increased neonatal jaundice
449
What are the main complications of slow neonatal resuscitation?
- Hypoxic ischaemic encephalopathy (HIE) - Cerebral palsy
450
Who does respiratory distress syndrome typically occur in?
Premature neonates (usually <32 weeks) due to insufficient surfactant
451
What is the investigation and management for respiratory distress syndrome?
- CXR (ground glass appearance) - Dexamethasone given to mothers (increases surfactant production) - Endotracheal surfactant - CPAP - Intubation/ventilation/oxygen
452
What are the complications of respiratory distress syndrome?
Short term: - Pneumothorax - Infection - Apnoea Long term: - Chronic lung disease of prematurity - Retinopathy of prematurity - Neurological/hearing/visual impairment
453
What is bronchopulmonary dysplasia?
Lung damage due to pressure and volume trauma of artificial ventilation, O2 toxicity and infection
454
What is the investigation and management for bronchopulmonary dysplasia?
- CXR (widespread areas of opacification +/- lung collapse) - CPAP - O2 - Corticosteroids
455
What are the risk factors for meconium aspiration?
- >42 weeks - Foetal distress - Thick meconium - Maternal HTN/diabetes/pre-eclampsia/smoking/etc.
456
What are the clinical features of meconium aspiration?
- Tachypnoea - Tachycardia - Cyanosis - Grunting - Nasal flaring - Recession (intercostal/supraclavicular/tracheal tug) - Hypotension
457
What are the investigations for meconium aspiration?
- CXR (increased lung volumes/asymmetrical patchy pulmonary opacities/pleural effusions/pneumothorax/etc.) - Infection markers (FBC/CRP/blood cultures) - ABG - Oximetry - Echo - Cranial USS
458
What is the management for meconium aspiration?
- Observation - Routine care (warm/O2 monitoring/bloods/fluids) - Ventilation/O2 therapy - Abx - Surfactant - Inhaled nitric oxide - Corticosteroids
459
What are the causes of hypoxic ischaemic encephalopathy?
- Maternal shock - Intrapartum haemorrhage - Prolapsed cord - Nuchal cord
460
How is hypoxic ischaemic encephalopathy graded?
Mild = poor feeding/general irritability/hyper-alert/resolves within 24 hours Moderate = poor feeding/lethargic/hypotonic/seizures/takes weeks to resolve Severe = reduced consciousness/apnoeas/flaccid/reduced/absent reflexes
461
What is the management for hypoxic ischaemic encephalopathy?
- Supportive care (resuscitation/ventilation/circulatory support/nutrition/acid base balance/treat seizures) - SEVERE = therapeutic hypothermia
462
What is the main complication of hypoxic ischaemic encephalopathy?
Cerebral palsy
463
What are the TORCH infections?
- Toxoplasmosis - Other (syphilis/varicella/mumps/parvovirus/HIV) - Rubella - CMV - HSV
464
What are the clinical features of a neonatal toxoplasmosis infection?
- Intracranial calcification - Hydrocephalus - Chorioretinitis
465
What are the clinical features of a neonatal varicella infection?
- Foetal growth restriction - Microcephaly/hydrocephalus/learning disability - Scars/significant skin changes - Limb hypoplasia - Cataracts/chorioretinitis
466
What are the clinical features of a neonatal rubella infection?
- Congenital cataracts - Congenital heart disease (PDA/pulmonary stenosis) - Learning disability - Hearing loss
467
What are the clinical features of a neonatal CMV infection?
- Foetal growth restriction - Microcephaly - Hearing/vision loss - Learning disability/seizures
468
How can varicella/rubella infections be prevented in neonates?
- Vaccines before or after pregnancy - Varicella - oral aciclovir for mother if present within 24 hours and >20 weeks gestation
469
Who is jaundice more common in?
- Normal after birth (no access to placenta --> normal rise in bilirubin) - More common in breastfed babies
470
What are some causes of jaundice due to increased bilirubin production?
- Haemolytic disease of newborn - ABO incompatibility - Haemorrhage - Sepsis/DIC - Polycythaemia - G6PD deficiency
471
What are some causes of jaundice due to decreased bilirubin clearance?
- Prematurity - Breast milk jaundice (components of breast milk inhibit liver's ability to process bilirubin) - Neonatal cholestasis - Extrahepatic biliary atresia
472
What are the clinical features of jaundice and when does it become prolonged?
- Mild yellowing of skin and sclera - Prolonged if lasts >14 days in full term babies or >21 days in premature babies
473
What are the investigations for jaundice?
- If presents in first 24 hours of life = urgent investigation/management required (indicates sepsis) - FBC/blood film - Conjugated bilirubin - Blood type testing - Direct coombs tets - TFTs - Blood/urine cultures - G6PD levels
474
What is the management for jaundice?
- Phototherapy (blue light - converts unconjugated bilirubin into isomers that can be excreted in bile/urine without conjugation) - Exchange transfusion
475
What is the main complication of jaundice?
Kernicterus - brain damage due to high bilirubin levels (bilirubin can cross blood-brain barrier)
476
What are the clinical features and complications of Kernicterus?
- Floppy, drowsy baby with poor feeding - Cerebral palsy - Learning disability - Deafness
477
When does necrotising enterocolitis commonly present and how common is it?
- Commonly presents in first 2 weeks of life - Most common surgical emergency in neonates
478
What are the risk factors for necrotising enterocolitis?
- Low birth weight/premature - Formula feeding - Respiratory distress/assisted ventilation - Sepsis - Patent ductus arteriosus
479
What are the clinical features of necrotising enterocolitis?
- Intolerance to feeds - Vomiting (particularly with green bile) - Generally unwell - Distended/tender abdomen - Absent bowel sounds - Blood in stool
480
What are the investigations for necrotising enterocolitis?
- Bloods (FBC/CRP/blood gas/culture) - AXR (dilated bowel loos/bowel wall oedema/pneumatosis intestinalis (gas in bowel wall)/pneumoperitoneum (indicates perforation - Rigler's sign (both sides of bowel visible))) - USS (air in portal system/ascites/perforation)
481
What is the management for necrotising enterocolitis?
- NBM + IV fluids + TPN + Abx (cefotaxime) - Bowel decompression (NG tube inserted to drain fluid/gas from GI tract) - Surgery (remove necrotic tissue)
482
What are the complications of necrotising enterocolitis?
- Perforation/peritonitis - Sepsis - Death
483
What is gastroschisis?
Abdominal organs protrude outside abdomen without protective membrane
484
What are the risk factors for gastroschisis?
- Younger mothers (<20 years) - Maternal smoking - Environmental exposure (e.g. nitrosamines) - Maternal cyclooxygenase inhibitors use (e.g. aspirin/ibuprofen)
485
What are the clinical features of gastroschisis?
- Abdominal organs herniated outside of abdominal cavity (often to the right of the umbilical cord) and no membrane covering contents - Swollen/inflamed/thickened/short intestines - Thick fibrous peel seen over contents (secondary to inflammation from amniotic fluid exposure) - Small abdominal cavity - Associated with intestinal malrotation/intestinal atresia - Malabsorption/hypomotility
486
What are the investigations for gastroschisis?
- Prenatal USS (echogenic and dilated bowel loops freely floating in amniotic cavity) - Alpha-fetoprotein (typically elevated)
487
What is the management for gastroschisis?
- Sterile/clear covering over contents - Surgery (immediate)
488
What is exomphalos/omphalocele and how is it managed?
- Abdominal defect in which abdominal contents protrude through abdominal wall but are covered by an amniotic sac - Staged repair with completion at 6-12 months
489
What is oesophageal atresia?
Birth defect in which the upper part of the oesophagus does not connect with the lower oesophagus/stomach
490
What are the risk factors for oesophageal atresia?
- Polyhydramnios (excess amniotic fluid in pregnancy) - Developmental problems regarding kidneys/heart/spine - Often happens alongside tracheo-oesophageal fistula
491
What are the clinical features of oesophageal atresia?
- Coughing - Choking - Turning blue - Inability to feed
492
What are the investigations for oesophageal atresia?
- Antenatal USS (excess amniotic fluid) - Attempt to pass NG tube - XRAY
493
What is the management for oesophageal atresia?
- Surgery - IV TPN + NG tube
494
What are risk factors for bowel atresia?
- Twin/multiple birth - Premature/low birth weight
495
What are the clinical features for bowel atresia?
- Appear well at birth - Vomiting when feeding - Soft, distended abdomen - Jaundice - May not pass meconium/small amount
496
What are the investigations and management for bowel atresia?
- Antenatal USS (polyhydramnios) - XRAY +/- contrast scan/enema (blockage) - Surgery - IV TPN + NG tube
497
What are clinical features of hypoglycaemia?
- Shaking/sweating - Irritability - Pale - Sudden behaviour changes - Clumsy/jerky movements
498
What is the management for hypoglycaemia in neonates?
- If asymptomatic, encourage normal feeding and monitor glucose - If symptomatic or very low, admit to neonatal unit and give IV infusion of 10% dextrose
499
Who is hypoglycaemia most common in?
- Newborns - Usually temporary
500
What are risk factors for Group B Strep infection?
- Premature - Previous baby with GBS infection - Fever during labour - Positive GBS urine/swab test during pregnancy - Waters broken >24 hours before birth
501
What are the clinical features of Group B Strep infection?
- Noisy breathing - Sleepy/unresponsive - Inconsolable crying - Being unusually floppy - Poor feeding - High/low temperature - Changes in skin colour
502
What is the management for Group B Strep infection?
- IV abx in labour - IV abx
503
Describe listeria infection
- Bacterial infection much more common in pregnancy - Typically transmitted by unpasteurised dairy products/processed meats/contaminated foods - Usually asx or flu-like symptoms - Pregnant women advised to avoid high risk foods e.g. blue cheese - Complications = pneumonia/meningoencephalitis/miscarriage/foetal death/neonatal infection
504
What are the causes of HSV encephalitis?
- HSV-1 (from cold sores) = more common in children - HSV-2 (from genital herpes) = more common in neonates
505
What are the clinical features of HSV encephalitis?
- Altered consciousness - Acute onset of focal neurological sx - Acute onset of focal seizures - Fever
506
What are the investigations and management for HSV encephalitis?
- LP and CSF sent for viral PCR testing - CT/MRI - EEG - Swabs to determine causative organism - HIV testing - Management = IV aciclovir
507
What is cleft lip/palate?
Cleft lip - a split/open section of the upper lip Cleft palate - defect in the hard/soft palate at the roof of the mouth, leaving an opening between the mouth and nasal cavity
508
What is the management for cleft lip/palate?
- Ensure baby can eat/drink (specifically shaped bottles/teats) - Surgery
509
What are the causes of microcytic anaemia?
TAILS: - Thalassaemia - Anaemia of chronic disease - Iron deficiency anaemia - Lead poisoning - Sideroblastic anaemia
510
What are the causes of normocytic anaemia?
AAAHH: - Acute blood loss - Anaemia of chronic disease - Aplastic anaemia - Haemolytic anaemia - Hypothyroidism
511
What are the causes of megaloblastic macrocytic anaemia?
B12/folate deficiency
512
What are the causes of normoblastic macrocytic anaemia?
- Alcohol - Reticulocytosis - Hypothyroidism - Liver disease - Drugs e.g. azathioprine
513
What are common causes of anaemia in infants?
- Physiologic anaemia of infancy - Iron deficiency anaemia - Anaemia of prematurity - Haemolysis (haemolytic disease of newborn)
514
What are the investigations and management for anaemia?
- Blood film - MCV - Hb - Treat cause
515
What is thalassaemia?
Genetic defect in protein chains that make up Hb (autosomal recessive) - Alpha thalassaemia = defects in alpha globin chains (gene on chromosome 16) - Beta thalassaemia - defects in beta globin chains (gene on chromosome 11 - minor/intermedia/major)
516
What are the clinical features of thalassaemia?
- Fatigue - Pallor - Jaundice - Gallstones - Bone deformities/poor growth - Splenomegaly - Pronounced forehead/malar eminences
517
What are the investigations for thalassaemia?
- FBC - Hb electrophoresis - DNA testing - Pregnancy screening test
518
What is the management for thalassaemia?
- Monitor FBC - Monitor for complications - Blood transfusions - Splenectomy - BM transplant
519
What is a complication of thalassaemia?
Iron overload
520
What is haemolytic disease of newborn?
Blood disorder in which a mother and baby's blood types are incompatible (rhesus D negative)
521
What are the clinical features of haemolytic disease of newborn?
- Jaundice - Severe anaemia - Compensatory splenomegaly/hepatomegaly
522
What is the investigation and management for haemolytic disease of newborn?
- Direct Coombs test (DCT) - Prevention of sensitisation with Rh immune globulin - Intrauterine transfusion
523
Who is sickle cell anaemia more common in?
Patients from areas traditionally affected by malaria - Africa/India/Middle East/Caribbean
524
Describe the genetics of sickle cell anaemia
Autosomal recessive - affects gene for beta globin on chromosome 11 - Sickle cell trait = one copy (usually asx) - Sickle cell disease - two copies
525
What are the investigations for sickle cell anaemia?
- Newborn blood spot screening (at around 5 days old) - High risk pregnant women offered testing
526
What is the management for sickle cell anaemia?
- Vaccinations - Abx prophylaxis (phenoxymethylpenicillin) - Hydroxycarbamide (stimulates HbF) - Crizanlizumab (monoclonal antibody that targets P-selectin to stop RBCs from sticking to blood vessel wall)
527
What is the main complication of sickle cell anaemia?
Sickle cell crisis: - Vaso-occlusive crisis (RBCs clog capillaries --> distal ischaemia) - Splenic sequestration crisis (RBCs block flow within spleen) - Aplastic crisis (temporary absence of creation of new RBCs) - Acute chest syndrome (vessels supplying lungs become clogged with RBCs)
528
What is Fanconi anaemia?
- Rare genetic disease characterised by bone marrow failure syndromes - Mutations in FA genes (23 different FA genes) - BM failure --> aplastic anaemia
529
What are the clinical features for Fanconi anaemia?
- Failure to thrive/growth deficiency - Skeletal anomalies (no thumb/radius bone) - Abnormal skin pigmentation (cafe au lait spots) - Structural anomalies of kidneys/heart/GI tract/urinary tract/genitals/etc. - Small head/eyes - Unexplained fatigue - Frequent infections - Frequent nosebleeds - Easy bruising - Blood in stool/urine
530
What are the investigations and management for Fanconi anaemia?
- Bloods/BM testing - Blood/platelet transfusions - WCC support with growth factor (G-CSF = granulocyte colony stimulating factor) - Androgen - BM transplant = definitive treatment
531
What are the complications of Fanconi anaemia?
Higher risk of: - Myelodysplastic syndrome - Leukaemia - Tumours
532
Who is haemophilia most common in?
Haemophilia A = factor VIII deficiency (more common) Haemophilia B = factor IX deficiency - Mainly affects men (X linked recessive)
533
What are the clinical features of haemophilia?
- Haematoma - Hemarthrosis - Frequent spontaneous bleeding into muscles/joints - Bleeding following injury/spontaneous bleeding - Easy bruising - Haematuria - Epistaxis
534
What are the investigations for haemophilia?
- PTT (normal - not in extrinsic pathway) - vWF (normal) - APTT (prolonged - intrinsic pathway) - Reduced plasma factor VIII/IX
535
What is the management for haemophilia?
- IV factor VIII/IX - Fresh frozen plasma - Desmopressin (boosts activity) - Hep A/B vaccinations - Exercise/avoid contact sports/avoid aspirin
536
What is a complication of haemophilia?
Joint deformities and arthritis from recurrent bleeding into joints
537
Who is Von Willebrand Disease more common in?
- Most common hereditary coagulopathy - More common in females - Poorer prognosis in blood type O
538
What are the clinical features of Von Willebrand Disease?
- Epistaxis - Menorrhagia - Spontaneous bleeding
539
What are the investigations for Von Willebrand Disease?
- FBC - Fibrinogen - Platelet count (normal) - Clotting screen - Plasma vWF (decreased) - Factor VIII levels (may be decreased)
540
What is the management for Von Willebrand Disease?
- Stop any antiplatelet drugs/NSAIDs - Tranexamic acid - COC - Desmopressin - Platelet transfusions - Family screening
541
What are the clinical features of anaemia?
- Tiredness - SOB - Headaches/dizziness/palpitations - Pale skin/conjunctival pallor - Tachyardia/rasied RR
542
What are causes of iron deficiency anaemia?
- Dietary insufficiency - Loss of iron e.g. menorrhagia - Inadequate absorption e.g. IBD - PPIs (stomach acid keeps it in soluble ferrous form)
543
What are the clinical features of iron deficiency anaemia?
- Pica - Hair loss - Koilonychia - Angular cheilitis - Atrophic glossitis - Brittle hair/nails
544
What are the investigations for iron deficiency anaemia?
- FBC - Blood film - Serum ferritin/iron/total iron binding capacity
545
What is the management for iron deficiency anaemia?
- Iron supplementation e.g. ferrous fumarate/sulphate (causes constipation/black stool) - Blood transfusion
546
What is immune thrombocytopenic purpura?
- Type II hypersensitivity reaction - Low platelet count causing a non-blanching purpuric rash
547
What are the investigations of immune thrombocytopenic purpura?
Diagnosis of exclusion: - Often preceded by viral illness - Acute onset - No other concerning features - Normal examination except bruising/petechiae - FBC/platelet count/blood film
548
What is the management for immune thrombocytopenic purpura?
- Treatment rarely required unless bleeding - Steroids - IVIG - TPO-RA (thrombopoietin receptor agonists)
549
What are the most common birth injuries in neonates?
- Caput succedaneum - Cephalohematoma
550
Describe caput succedaneum
- Caused by pressure on scalp during birth - Results in subcutaneous extraperiosteal collection of fluid - Presents as oedematous swelling/bruising over scalp that crosses suture lines - Usually reduces over a few days
551
Describe cephalohematomas
- Occurs after spontaneous vaginal delivery/following trauma from forceps/ventouse - Causes haemorrhage between skull and periosteum - Presents as swelling of scalp that doesn't cross suture lines - Reduces over a few weeks/months
552
Which intracranial haemorrhage is most common in neonates?
- Subarachnoid haemorrhage - Intraventricular haemorrhages more common in pre-term infants
553
What is the most common cause of a subdural haemorrhage in neonates?
Use of forceps
554
What are the clinical features of intracranial haemorrhages in neonates?
- No obvious head trauma/swelling - Irritability/convulsions over first 2 days of life
555
At what ages are vaccinations given?
- 8 weeks - 12 weeks - 16 weeks - 1 year - 2-15 years - 3 years 4 months - 12-13 years - 14 years
556
What vaccinations are given at 8 weeks?
- 6-in-1 (DTaP/IPV/Hib/HepB) - Rotavirus - MenB
557
What vaccinations are given at 12 weeks?
- 6-in-1 (DTaP/IPV/Hib/HepB) - Pneumococcal - Rotavirus
558
What vaccinations are given at 16 weeks?
- 6-in-1 (DTaP/IPV/Hib/HepB) - MenB
559
What vaccinations are given at 1 year?
- Hib/MenC - MMR - Pneumococcal - MenB
560
What vaccinations are given at 2-15 years?
Flu vaccine
561
What vaccinations are given at 3 years 4 months?
- MMR - 4-in-1 pre-school booster (DTaP/IPV)
562
What vaccinations are given at 12-13 years?
HPV
563
What vaccinations are given at 14 years?
- 3-in-1 teenage booster (Td/IPV) - MenACWY
564
Describe paediatric IV fluid prescribing
- Usually 0.9% sodium chloride + 5% glucose - 1000ml/kg for first 10kg - 500ml/kg for next 10kg - 20ml/kg for next 1kg
Phase 3a
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