Paediatrics

Question 1

Foetal shunts

1. Ductus venosus
2. Foramen ovale
3. Ductus arteriosus
4. Patent - cause
5. Patent - presentation

Answer 1

1. Connects umbilical vein to IVC; bypass liver
2. Connects RA to LA; bypass RV / pulm circulation
3. Connects pulm artery to aorta; bypass pulm circulation
4. Rubella, prematurity
5. Asymptomatic, or if large L-R shunt then SOB, difficulty feeding, poor weight gain, LRTI (monitor with echo until 1y)

Question 2

Murmurs

1. Innocent (flow, during systole) murmurs - features
2. Refer if
3. Pan-systolic - differentials
4. Ejection-systolic - differentials
5. Split 2nd heart sound (S2) - cause
6. Atrial septal defect
7. Ventricular septal defect
8. Patent ductus arteriosus
9. Coarctation of the aorta
10. Pulmonary stenosis

Answer 2

1. Soft, short, systolic, symptomless, situation-dependent (quieter standing, worse if unwell)
2. Loud (+ on standing), diastolic, other symptoms
3. VSD, MR, TR
4. HOCM (4th IC, left sternal border), AS, PS (ToF)
5. Pulmonary valve closes slightly later than aortic valve
6. Mid-systolic, crescendo-decrescendo murmur, loudest at upper left sternal border, fixed split S2
7. Pan-systolic, 3/4th IC, left sternal border, + thrill
8. Normal S1, continuous crescendo-decrescendo “machinery” murmur (may continue during S2)
9. Systolic, below left clavicle/scapula
10. Ejection systolic, loudest in pulmonary area (2nd IC, left sternal border)

Question 3

Septal defects - atrial and ventricular

1. Features
2. Complications - atrial
3. Complications - ventricular
4. Ventricular - associated with which syndromes (2)
5. Atrial - associated with what structural defect
6. Structural defect causes what on auscultation
7. Eisenmenger syndrome - causes (3)
8. Pathophysiology/features
9. Management - pre-heart/lung transplant

Answer 3

1. NO cyanosis, but right overload/strain, leading to right HF + pulmonary HTN
2. Stroke post-VTE, AF/flutter,
3. Higher risk of IE (prophylactic ABX in surgery)
4. Down’s / Turner’s syndrome
5. Ebsteins anomaly (low tricuspid valve so bigger RA and smaller RV), causing R-L shunt. Linked to WpW
6. ‘Gallop’ rhythm - 3rd and 4th heart sounds
7. ASD, VSD, patent ductus arteriosus
8. Pulmonary pressure greater than systemic pressure, so shunt reverses (becomes R-L) so blood bypasses lungs + patient becomes cyanotic
9. Oxygen, sildenafil (pulmonary HTN), venesection (polycythaemia), anticoagulation (thrombosis)

Question 4

Coarctation of the aorta (no shunt)

1. Associated condition
2. Signs
3. If critical at birth, give what before surgery
4. Pulmonary valve stenosis - associated diseases
5. Signs
6. Tetralogy of Fallot - 4 problems
7. Risk factors
8. CXR - defining feature
9. Tet spells - what
10. Another cyanotic HD with shunt

Answer 4

1. Turner’s syndrome
2. No cyanosis, weak femoral pulses, systolic murmur below left clavicle/scapula, LV heave post-hypertrophy, underdeveloped left arm/legs, headache/nose bleeds
3. Prostaglandin E to keep DA open (do same in ToF)
4. ToF, William/Noonan/congenital rubella syndromes
5. Cyanosis, palpable thrill in pulmonary area, RV heave from hypertrophy, raised JVP with giant a waves
6. VSD and overriding (right-leaning) aorta, alongside pulmonary valve stenosis (so RV deoxygenated blood more likely to go into aorta), causing RV hypertrophy
7. Rubella, old/diabetic/alcohol drinking mother
8. ‘Boot-shaped’ heart from RV thickening
9. Acute worsening, can happen when waking / crying / on exertion. Squat increases SVR so blood goes to lungs
10. Transposition of great arteries

Question 5

Acute SOB

1. Bronchiolitis - commonest age, + causes
2. Auscultation
3. Management - SUPPORTIVE
4. What makes more severe
5. Pneumonia - commonest bacteria + virus
6. If unvaccinated
7. ABX - 1st line
8. Recurrent LRTIs - differentials
9. Croup - age group, common causative virus
10. Presentation
11. Mild vs moderate vs severe
12. Management
13. Epiglottitis - causative organism
14. Presentation
15. Lateral X-ray - finding

Answer 5

1. < 1 year (3-6 months), RSV (also adenovirus, rhinovirus)
2. Harsh breath sounds, fine inspiratory wheeze, dry cough, crackles
3. Minimal handling, adequate intake, nasal saline drops/suction, O2 if <92%
4. Bronchopulmonary dysplasia, CHD, CF
5. Streptococcus pneumoniae, RSV
6. Group B streptococcus, haemophilus influenzae
7. Amoxicillin, + macrolide if atypical suspected (or PI)
8. Reflux, aspiration, neurological/heart disease, asthma, CF, primary ciliary dyskinesia, immune deficiency
9. 6-24 months, parainfluenza virus
10. ‘Barking’ cough, hoarse, stridor, low fever
11. Mild (cough but no stridor/RD at rest), moderate (add RD at rest), severe (add agitation/lethargy)
12. Maybe admit, supportive, oral dexamethasone (single dose), if worse then O2 + nebulised adrenaline
13. Haemophilus influenza type B (HIB)
14. Sore throat, stridor, drooling, tripod, high fever
15. ‘Thumbprint sign’ from oedematous epiglottis

Question 6

Wheeze

Asthma

1. Acute - moderate
2. Severe
3. Life-threatening
4. Management ‘O SHIT ME’
5. Can discharge when
6. Long term management - 3rd line
7. Viral wheeze (expiratory, global) - features vs asthma
8. If focal wheeze, consider what (3)

Answer 6

1. Peak flow >50%
2. PF <50%, sats <92%, no sentences, respiratory distress, RR >40 if 1-5, >30 if >5, HR >140 if 1-5, >125 if >5
3. PF <33%, sats <92%, exhaustion, poor respiratory effort, hypotension, silent chest, cyanosis, reduced GCS
4. Oxygen, Salbutamol (back to back), Hydrocortisone / prednisolone (1mg/kg for 3 days), ipratropium, Theophylline infusion (only in ITU as need daily levels, U+Es, ECG monitor), Magnesium IV, Escalate to CC
5. Child well on maximum 6 puffs salbutamol 4 hourly
6. LTRA (if under 5), LABA (if 5+)
7. <3 years old, no atopy, only during viral infections
8. Inhaled foreign body, lesion, focal infection

Question 7

Other paediatric respiratory conditions

1. Laryngomalacia - cause
2. Presentation
3. Whooping cough - causative organism
4. Presentation
5. Diagnosis
6. Management
7. Key complication
8. Chronic lung disease of prematurity (CLDP) - aka
9. Diagnosis
10. Prevention
11. To complete respiratory exam, do what (3)

Answer 7

1. Supraglottic larynx causes partial airway obstruction
2. Chronic inspiratory stridor, worse if feeding, upset, lying back or during URTIs (no respiratory distress)
3. Bordetella pertussis (gram negative bacteria)
4. Coryzal symptoms for a week, then coughing fits, inspiratory ‘whoop’ when coughing ends (lasts 8 weeks)
5. NP/nasal swab with PCR testing or bacterial culture
6. Notifiable disease, supportive, ABX (macrolide or co-trimoxazole, + prophylactic to vulnerable close contacts)
7. Bronchiectasis
8. Bronchopulmonary dysplasia
9. CXR changes, + needs O2 after 36 weeks GA
10. Corticosteroids e.g. betamethasone to mother if showing signs of labour < 36 weeks
11. Vital signs, growth chart, ENT

Question 8

Cystic fibrosis (autosomal recessive)

1. CFTR mutation - commonest type
2. How many are carriers, how many born with CF
3. Consequence - GI
4. Respiratory
5. Reproductive
6. 1st sign of CF, + associated signs
7. Other signs
8. Diagnosis - 1st
9. Gold-standard - confirming 1st result
10. Genetic testing for CFTR gene - when
11. Common colonisers
12. Management - medical
13. Monitoring/screening requirements
14. Primary ciliary dyskinesia - aka, inheritance, linked with
15. Kartagener’s triad (not all will have all 3)
16. Diagnostic test

Answer 8

1. Delta-F508 (codes for particular chlorine channel)
2. 1 in 25 are carriers, 1 in 2500 born with CF
3. Blocked ducts (thick pancreas/biliary secretions) so lack of digestive enzymes e.g. lipase in tract, so malabsorption, steatorrhoea, abdo pain, failure to thrive
4. Thick secretions, less airway clearance, bacterial colonisation, frequent infections
5. Male infertility (bilateral absent vas deferens)
6. Meconium ileus (with abdo distention + vomiting)
7. Nasal polyps, finger clubbing, crackles/wheeze
8. Newborn heel prick (immune reactive trypsinogen)
9. Sweat test (pilocarpine), diagnose if Cl- >60mmol/l
10. Pregnancy (amniocentesis/CVS) / shortly after birth
11. Staph. aureus, pseudomonas, h. influenza, klebsiella
12. CREON tablets, prophylactic flucloxacillin, bronchodilators, nebulisers (dornase alpha, saline), vaccinations (pneumococcal, varicella, influenza)
13. Regular sputum/CXR, monitor for DM (50% need insulin), osteoporosis (DEXA), USS abdo, low vit D, liver failure (30%)
14. Kartagener’s, autosomal recessive, consanguinity
15. Paranasal sinusitis, bronchiectasis, situs inversus
16. Ciliated epithelium sample, assess ciliary function (from nasal brushing / bronchoscopy)

Question 9

Cardiac abnormality - summary

1. Acyanotic, with shunt
2. Acyanotic, no shunt
3. Cyanotic, with shunt
4. Cyanotic, no shunt
5. Symptoms
6. Signs
7. Complications

Answer 9

1. ASD, VSD, PDA
2. Coarctation of aorta
3. ToF, TGA
4. Severe pulm. stenosis, T/P atresia, hypoplastic L heart
5. Problems breast feeding, failure to thrive, syncope, squatting, oedema, sweating
6. Murmur, high HR/RR, clubbing, cyanosis (esp. feeding)
7. Infective endocarditis, paradoxical embolism, polycythaemia, pulmonary HTN

Question 10

Respiratory - main causes of problems

1. Neonates
2. Infants
3. Under 5
4. Over 5
5. Respiratory distress - signs
6. Bronchiolitis vs pneumonia

Answer 10

1. Resp. distress syndrome (premature, less surfactant, atelectesis + low O2/high CO2 - CXR ‘ground glass’; apnoea (20s + low HR, give IV caffeine)
2. Bronchiolitis, pneumonia, croup, GI reflux/aspiration, HF
3. Viral induced wheeze, croup, pneumonia
4. Asthma, pneumonia
5. Tachypnoea, head bobbing, tracheal tug, abdominal movements to aid ventilation, sub/intercostal recession
6. Pneumonia - focal signs, higher grade fever

Question 11

Seizures

1. GTC - 1st line
2. Focal
3. Myoclonic
4. Absence
5. Atonic
6. MRI to rule out structural pathology if (3)
7. Key blood test if afebrile seizure
8. Febrile convulsion - cause (+ other differentials)
9. Simple - definition
10. Complex - definition
11. Management
12. Reoccurs how often

Answer 11

1. Sodium valproate (SEs: liver damage, hair loss)
2. Carbamazepine (agranulocytosis, P450 inducer, aplastic anaemia) / lamotrigine (SJS, leukopaenia)
3. Valproate
4. Ethosuximide (night terrors, rash) / valproate
5. Valproate (3 minutes, maybe Lennox-Gastaut synd)
6. 1st seizure <2yo, focal, don’t respond to 1st line
7. Blood glucose
8. Viral (+ infection, epilepsy, SOL, syncope, trauma)
9. GTC, <15 minutes, once during febrile illness.
10. Focal/partial, >15 minutes, >1x during febrile illness
11. Antipyretic, reassure parents, safety netting, benzodiazepines if > 15 minutes
12. 1 in 6

Question 12

Shaken baby syndrome

1. Triad
2. 1st line investigations

Answer 12

1. Retinal haemorrhage, subdural haematoma, encephalopathy
2. CT scan, FBC, LFT, clotting, toxicology, urinalysis,
   LP (if stable/not contraindicated)

Question 13

Lyme disease

1. Presentation
2. Neurological manifestations (3)
3. Diagnosis confirmed via
4. Management

Answer 13

1. Erythema migrans, fatigue, lymphadenopathy, myalgia, headache, fever
2. Meningitis, facial palsy, cerebellar ataxia
3. Antibody testing
4. Amoxicillin / doxycycline

Question 14

Cerebral palsy

1. Definition
2. Causative factors (3)
3. Types (3) and what has been damaged
4. MRI - indications
5. Hypoxic ischaemia encephalopathy - causes
6. How to stage
7. Management

Answer 14

1. Mixed neurological disorder (mostly motor/posture), caused by non-progressive injury to developing brain in the first two years of life
2. Antenatal (80%), hypoxic ischaemic injury during delivery, post-natal (IC haemorrhage, head injury, sepsis)
3. Spastic (cortex), extrapyramidal (basal ganglia), ataxic (cerebellum)
4. Rule out neurodegenerative/metabolic conditions if aetiology unclear or signs of regression
5. Maternal shock, intrapartum haemorrhage, prolapsed cord, nuchal cord
6. Sarnat Staging
7. Therapeutic hypothermia

Question 15

Meningitis

1. Non-specific presentation in babies
2. Investigations
3. Bacterial - commonest organisms in children (2)
4. Commonest in neonates
5. Antibiotics - prehospital
6. Hospital if <3 months
7. > 3 months
8. If risk of penicillin-resistant pneumococcal infection (e.g. recent foreign travel or antibiotic use)
9. Steroids - what, when, why
10. Viral - commonest causes (3)
11. Investigations
12. Management

Answer 15

1. Hypotonia/thermia, poor feeding, lethargy, bulging fontanelles
2. LP, blood culture, blood for meningococcal PCR
3. Neisseria meningitidis (gram-negative diplococcus), Strep. pneumoniae (gram-positive pneumococcus)
4. Group B streptococcus (for neonatal sepsis too)
5. IM benzylpenicillin
6. Cefotaxime AND amoxicillin
7. Ceftriaxone (80mg/kg) or cefotaxime
8. Vancomycin
9. Dexamethasone 4x/d if >3mo + LP positive for bacteria - anti-sensorineural hearing loss/neuro damage
10. HSV, enterovirus, VZV
11. CSF sample for HSZ/enterovirus/VZV PCR
12. Supportive, + aciclovir (clinical/CSF positive decision)

Question 16

Encephalitis

1. Commonest in neonates
2. Commonest in children
3. Presentation
4. Investigations
5. Management

Answer 16

1. HSV-2
2. HSV-1 (+ VZV, CMV, EBV, adenovirus, influenza)
3. Focal neurology/seizures, altered consciousness, unusual behaviour, fever
4. LP with viral PCR, CT if not (GCS <9, haemodynamically unstable, active seizures, post-ictal)
5. Aciclovir

Question 17

Hydrocephalus

1. Commonest cause
2. Other causes
3. Presentation
4. Complication of VP shunt (management)

Answer 17

1. Aqueductal stenosis
2. Arachnoid cysts, Arnold-Chiari malformation
3. Big head, bulging anterior fontanelle, sleepiness, poor tone, poor feeding/vomiting
4. Infection, peri-operative intraventricular haemorrhage, blockage, over-drainage, outgrowing (replace every 2y)

Question 18

Muscular dystrophy

1. Sign suggesting proximal muscle weakness
2. Duchenne MD (X-linked recessive) - caused by
3. Present at what age, with what
4. Management - medical
5. Name if dystrophin gene less severely affected

Answer 18

1. Gower’s sign (when standing up from lying position)
2. Defective gene for dystrophin on X-chromosome
3. 3-5yo, weakness around pelvic muscles
4. Oral steroids (slow progression), creatine supplements
5. Becker’s MD (symptoms at 8-12yo)

Question 19

Diarrhoea

1. Differential diagnosis
2. Viral - commonest causes (2)
3. E.coli - presentation, management
4. Traveller’s diarrhoea - organism, management
5. If lymphadenopathy - organism
6. Haemolytic uraemic syndrome (HUS) - triad
7. Cause
8. Features
9. Investigations
10. Management

Answer 19

1. IBD, IBS, gastroenteritis, appendicitis, coeliac disease, lactose intolerance
2. Rotavirus, norovirus (adenovirus more subacute)
3. Cramps, bloody diarrhoea, vomiting, NO ABX
4. Campylobacter jejuni, give azithromycin/ciprofloxacin
5. Yersinia (gram-negative bacillus), mesenteric lyphadenitis so Ddx in appendicitis
6. Microangiopathic non-immune haemolytic anaemia,
   thrombocytopenia, AKI
7. E.coli (+ shigella) - shiga toxin
8. Diarrhoea, severe abdominal pain, vomiting
9. Stool culture, FBC, blood film, G+S, COOM, LDH, coagulation screen, biochemistry
10. Supportive - RRT, anti-HTN, fluid balance, transfusions

Question 20

Constipation

1. History
2. Idiopathic - 1st line laxative
3. Secondary causes
4. Red flags
5. Intestinal obstruction - signs
6. Causes - neonate
7. Causes - slightly older

Answer 20

1. <3/week, hard, ‘rabbit dropping’, straining, pain, retentive posturing, overflow soiling
2. Movicol
3. Hirschsprung’s, CF, hypothyroid, spinal cord lesion, sexual abuse, intestinal obstruction, anal stenosis, cow’s milk intolerance
4. Meconium ileus (Hirschprungs, CF), vomiting (intestinal obstruction/Hirschprungs), ribbon stool/abnormal anus (anal stenosis), abnormal lower back/buttocks (spina bifida, sacral agenesis), failure to thrive (coeliac disease, hypothyroid, safeguarding), acute severe abdominal pain + bloating (obstruction, intussusception)
5. Absolute constipation (stool/flatus), vomiting (projectile, bile stained), abdominal pain/distention, abnormal bowel sounds (high pitched/tinking at first, then absent)
6. Meconium ileus, hirschprungs, oesophageal/duodenal atresia, imperforate anus
7. Intussusception, malrotation/volvulus, strangulated hernia

Question 21

Coeliac disease

1. What is it
2. Antibodies
3. Endoscopy finding
4. Presentation
5. Neurological signs (3) - rare
6. Linked autoimmune diseases
7. Associated malignancies (3)

Answer 21

1. Autoimmune disease, inflammatory response to gluten
2. Anti-TTG, anti-endomysial (EMA) (check IgA levels 1st)
3. Crypt hypertrophy, jejunal villous atrophy
4. Failure to thrive, diarrhoea, fatigue, weight loss, mouth ulcers, anaemia (iron/B12/folate deficiency), dermatitis herpetiformis on abdomen
5. Peripheral neuropathy, cerebellar ataxia, epilepsy
6. T1DM, thyroid, hepatitis, PBC, PSC
7. Enteropathy-associated T-cell lymphoma (EATL) of the intestine, non-hodgkin lymphoma, small bowel adenocarcinoma (rare)

Question 22

Intussusception

1. Presentation
2. Associated conditions
3. Gold-standard investigation
4. Management - medical
5. Surgical - indication (3)

Hirschprung’s disease

6. Diagnosis
7. Associated conditions
8. Main complication
9. Necrotising entercolitis - presentation
10. Bloods
11. AXR finding

Answer 22

1. Severe colicky abdominal pain, ‘Red currant jelly’ stool, pale/lethargic, palpable RUQ ‘sausage shaped’ mass, vomiting, intestinal obstruction - in 6-24mo boys
2. Viral illness, HSP, CF, intestinal polyps, Meckel D
3. Abdominal USS + contrast enema
4. Fluid resuscitation, broad IV ABX (co-amoxiclav), pneumatic reduction therapeutic enema
5. Failed enema, highly distended abdomen, peritonitis
6. Rectal biopsy - congenital absence of mesenteric parasypathetic ganglion cells (colonic aganglionosis)
7. Down’s/Waardenburg syndrome, NF, MEN 2
8. Hirschprung-associated entercolitis (HAEC) - fever, abdominal distention, bloody diarrhoea, sepsis within 2-4 weeks of birth - leads to toxic megacolon/perforation
9. Premature, won’t feed, tender/distended abdomen,
   no bowel sounds, blood in stool
10. Metabolic acidosis, low platelets
11. Pneumatosis intestinalis (gas in bowel wall)

Question 23

Fluids

1. Bolus - amount (vs in trauma)
2. Maintenance - fluid of choice
3. Total fluids in 24 hours - A + B + C

Answer 23

1. 20 ml/kg of 0.9% NaCl (10ml/kg in trauma)
2. 0.9% NaCl + 5% dextrose
3. A (100 ml/kg first 0-10 kg)
   B (50 ml/kg 10-20 kg)
   C (20 ml/kg 20 kg+)

Question 24

Jaundice

1. Prolonged - definition
2. Think pathological if prolonged or (2)
3. Haemolytic anaemia - causes (3)
4. Hepatic causes (3)
5. Post-hepatic causes (3)
6. History - important questions (3)
7. Associated symptom
8. Bedside test
9. Gold-standard test
10. Other tests
11. Emergency presentation
12. Biliary atresia (narrow/absent bile duct) - blood test
13. Management

Answer 24

1. > 2 weeks after birth (physiological resolves by then)
2. Within first 24 hours, serum bilirubin <95 percentile
3. G6PD, Rhesus disease, ABO incompatibility
4. Sepsis, hepatitis, TORCH infections
5. Biliary atresia, choledochal cysts, bilary stricture
6. Age at onset, how marked is it, stool/urine colour
7. Brushing
8. Transcutaenous bilirubinometer
9. Serum bilirubin
10. Direct Coombs (haemolysis), haematocrit, FBC, (polycythaemia, anaemia) reticulocyte count, blood smear, blood groups (ABO)
11. Kernicterus - acute bilirubin encephalopathy
12. High conjugated bilirubin (can make but not excrete)
13. Kasai portoenterostomy, liver transplant

Question 25

GORD 1. Presentation - babies 2. Vomiting - differentials Red flags 3. Intestinal obstruction 4. Pyloric stenosis 5. Peptic ulcer, oesophagitis or varices 6. Meningitis/raised ICP 7. Gastroenteritis 8. Cows milk protein allergy 9. Sandifer syndrome 10. Other more serious differentials

Answer 25

1. Chronic cough, hoarse cry, feeding distress/reluctance, pneumonia (aspiration), poor weight gain 2. Overfeeding, GORD, pyloric stenosis, gastritis, appendicitis, other infections, obstruction 3. Projectile, bile-stained, keep nothing down, distention 4. Projectile, keeping nothing down 5. Haematemsis, melaena 6. Reduced GCS, bulging fontanelle, neuro signs 7. Blood in stool, diarrhoea 8. Blood in stool, rash, angioedema, other signs of allergy 10. Torticollis + dystonia associated with GORD 11. Infantile spasms (West syndrome), seizures

Question 26

Pyloric stenosis 1. Presentation 2. Vomiting - description 3. Examination finding post-feeding 4. Blood gas finding 5. Diagnosis 6. Management - surgical

Answer 26

1. First few weeks of life; hungry, thin, pale, failing to thrive 2. Projectile, keeping nothing down 3. Firm, round mass in upper abdomen 'large olive' from pyloric hypertrophy 4. Hypochloraemic metabolic alkalosis 5. Abdominal USS 6. Laparoscopic pyloromyotomy ('Ramstedt’s operation')

Question 27

Abdominal pain 1. Surgical causes 2. Medical causes 3. Appendicitis - presentation 4. Diagnosis 5. Important differentials

Answer 27

1. Appendicitis (central to RIF), intussusception, bowel obstruction 2. HSP (with non-blanching rash on legs/buttocks), tonsillitis (central), pneumonia (upper), constipation (low), pyelonephritis (loin), mesenteric adenitis (low/RIF) 3. Loss of appetite, N+V, Rovsing's sign, guarding, rebound/percussion tenderness 4. Clinical + raised inflammatory markers, CT if likely other cause, USS if F, diagnostic laparoscopy 5. Ectopic, ovarian cyst, Meckel's diverticulum (distal ileum problem causing volvulus/intussusception)

Question 28

Type 1 Diabetes Mellitus 1. Potential triggering viruses (2) 2. Insulin - functions (2) 3. Glucagon - made where, due to what, functions (2) 4. Symptoms of hyperglycaemia 5. Antibodies associated with islet cell destruction (3) 6. Tests to check for other autoimmune diseases (2) 7. DKA - diagnosis (3 parts) 8. In children, correct dehydration over how long

Answer 28

1. Coxsackie B virus, enterovirus 2. Causes cells to absorb glucose from blood and use as fuel, tells liver/muscle cells to absorb glucose from blood to store as glycogen 3. Alpha cells, if low BM, tells liver to do glycogenolysis + gluconeogenesis (cortisol also tells liver to do this) 4. Polyuria, polydipsia, weight loss, bedwetting, recurrent infections 5. Anti-insulin anti-GAD, anti-islet cell antibodies 6. TFTs/TPO, anti-TTG 7. Hyperglycaemia >11, ketosis >3, acidosis <7.3 8. 48 hours, to reduce risk of cerebral oedema

Question 29

Adrenal insufficiency 1. Primary - cause 2. Bloods 3. Management - medical 4. Other advice/management 5. Addisonian crisis - bloods 6. Secondary - cause 7. Bloods 8. Tertiary - cause 9. Congenital adrenal hypoplasia - inheritance, deficiency 10. Bloods 11. Presentation - female neonate 12. Older child 13. Why skin pigmentation 14. Management

Answer 29

1. Autoimmune adrenal gland damage (Addison's) 2. Low cortisol (even after synacthen administered), high ACTH, low aldosterone, high renin 3. Hydrocortisone (replaces C), fludrocortisone (A) 4. Steroid card, emergency ID tag, when unwell (increase steroids, monitor BM) 5. Hypotension, low BM, low Na+, high K+ 6. Low ACTH made (pituitary damage/hypoplasia) 7. Low cortisol, low ACTH, normal aldosterone/renin 8. Low CRH made in hypothalamus (stopped steroids) 9. Autosomal recessive, 21-hydroxylase enzyme (usually converts progesterone into aldosterone/cortisol) 10. High testosterone (converted from progesterone), low adrenal cortisol/aldosterone 11. Virilised 'ambiguous' genitalia, low Na+/BM, high K+ 12. Skin pigmentation, tall, deep voice, small testicles/absent periods, early puberty 13. ACTH produced due to low cortisol; byproduct of this is melanocyte-stimulating hormone 14. Hydrocortisone, fludrocortisone, surgery for virilism

Question 30

Growth hormone deficiency 1. GH - produced where, stimulates what 2. Congenital deficiency - causes 3. Secondary deficiency - causes 4. Presentation 5. GH stimulation test - which medications used (4) 6. Management (tertiary)

Answer 30

1. Anterior pituitary, insulin-like growth factor 1 (IGF-1) 2. Hypothalamus/pituitary gland problem (GH1/GHRHR gene mutations, empty sella syndrome) 3. Infection, trauma, surgery 4. Micropenis, severe jaundice, hypoglycaemia, small 5. Glucagon / insulin / arginine / clonidine 6. Daily SC somatropin (GH)

Question 31

Delayed puberty 1. Scale used to determine stage of puberty 2. Hypogonadism (low oestrogen/testosterone) - 2 types 3. How to assess for constitutional delay

Answer 31

1. Tanner 2. Hypogonadotrophic (low FSH/LH) - hypothalamus / pituitary bad (low GH, high prolactin, Kallman - no smell) Hypergonadotrophic (high FSH/LH but no response by gonads) - gonad absence/damage, Klinefelter, Turner 3. Wrist x-ray

Question 32

UTI 1. Symptoms 2. Pyelonephritis - diagnosis 3. Urinalysis 4. Management - stat IV ABX (ceftriaxone) when 5. If >3 months + otherwise well 6. Recurrent UTI - investigations 7. Other test if < 6 months, + what for 8. Investigation 4-6m post-UTI if atypical/recurrent

Answer 32

1. Fever, lethargy, irritable, suprapubic pain, dysuria 2. Fever >38, loin pain/tenderness 3. Dipstick (nit/leucocytes, send MSU to lab if 1/2 positive) 4. <3 months old, acutely unwell, persistent vomiting (also do septic screen) 5. PO ABX - trimetho/nitrofurantoin/cefalexin/amoxicillin 6. Abdo USS within 6 weeks for all <6 months after 1st UTI + >6 months if recurrent. USS during illness if aytypical UTI 7. Micturating Cystourethrogram, for vesico-ureteric reflux 8. DMSA (dimercaptosuccinic acid) scan - look for scarring

Question 33

Nephrotic syndrome (basement membrane malfunction) 1. Classic presentation 2. Classic finding triad 3. Other clinical findings 4. Commonest paediatric cause (90% under 10yo) 5. Findings from this cause 6. Other secondary disease causes 7. Investigations - general 8. Investigations for secondary cause 9. Management 10. Complications

Answer 33

1. Frothy urine, generalised oedema, pallor 2. Hypoalbuminaemia (<25g/L), oedema, proteinuria (3+ urine dip or >1g/m2/day) 3. Deranged lipids, HTN, hypercoagulability 4. Minimal change disease (isolated nephrotic syndrome) 5. Renal biopsy normal, small molecular weight proteins + hyaline casts on urinalysis 6. Intrinsic kidney disease (FSGS, membranoproliferative), systemic illness (HSP, DM, infection) 7. Plasma albumin, urine dipstick/24 hour urine protein, lipids, U+E, TFT, FBC 8. Complement levels (SLE), auto-antibodies, HbA1C 9. 4 weeks high dose prednisolone (if steroid resistant, use ACE-i / immunosuppressants), low salt diet, diuretics 10. Hypovolaemia, infection, thrombosis, renal failure

Question 34

Leukaemia - general 1. Bone marrow failure - triad 2. Tissue infiltration - triad 3. Symptoms 4. 1st line investigations 5. Specialist investigations 6. Commonest order in children (+ peak ages) 7. Abnormal bruising - other differentials 8. Specific complications of management

Answer 34

1. Pancytopaenia - thrombocytopenia, anaemia, leukopenia 2. Bone pain, hepatosplenomegaly, lymphandenopathy 3. Fatigue, fever, failure to thrive, anaemia, petechiae, abnormal bruising, bleeding, lymphadenopathy, hepatosplenomegaly 4. FBC, blood film, CXR, clotting screen 5. Bone marrow aspirate, LP 6. ALL (2-3y), AML (< 2y), CML (< 1y, + Ph chromosome) 7. HSP, NAI 8. Stunted growth, neuro/cardiotoxicity, infertility, secondary malignancy (AML)

Question 35

Kawasaki disease 1. AKA 2. Features (5) 3. Raised inflammatory marker 4. Management

Answer 35

1. Mucocutaneous lymph node syndrome 2. High grade fever >5 days, conjunctival injection, cervical lymphadenopathy, bright red cracked lips, strawberry tongue, red palms/soles 3. ESR 4. IV aspirin, maybe IV IG

Question 36

Anaemia in children 1. Commonest general cause 2. Causes of above (3) 3. Serious SE of low iron 4. Blood film - results 5. Management

Answer 36

1. Iron deficiency anaemia 2. Insufficient intake, malabsorption, chronic blood loss 3. Decreased cognitive/psychomotor performance 4. Hypo-chromic, microcytic cells 5. Diet advice (+ limit milk), iron replacement (continue 2-3 months after target levels met), transfusion (rare)

Question 37

Sickle cell anaemia 1. Inheritance 2. Dysfunctional protein 3. Up to what month is this not a problem (+ why) 4. Trait vs symptomatic 5. Diagnosis 6. Sickle cell crisis - cause 7. Triggers 8. Types (4) 9. Acute chest syndrome - definition 10. Management - acute 11. Management - long-term 12. Hereditary spherocytosis (AD) - presentation 13. Blood results 14. Management

Answer 37

1. Autosomal recessive (gene on chromosome 11) 2. Beta globin chain - RBCs deform when deoxygenated (HbS instead of HbB - normal 2x HbA, 2x HbB) 3. 6 months (foetal Hb (2 gamma chains instead of 2 B) still in circulation 4. 1 HbS (aysmptomatic), vs 2 abnormal variants 5. Haemoglobin electrophoresis 6. Deformed RBCs get stuck 7. Hypoxia, cold, dehydration, exercise, stress, infection 8. Vaso-occlusive (pain), sequestration (spleen pain, hypovolaemia), aplastic (from parvovirus B19), haemolytic (jaundice, commoner with G6PD defiency) 9. Fever/respiratory symptoms + new infiltrates on CXR 10. Analgesia (no NSAIDs if renal impairment), oxygen, keep warm/hydrated, treatment of any infection 11. Prophylactic penicillin V, vaccines UTD, close monitoring, hydroxyurea/carbamide, marrow transplants (can be curative) 12. Jaundice (intermittent), gallstones, failure to thrive, hepatosplenomegaly, aplastic crisis (parvovirus) 13. Spherocytes, reticulocytosis, raised mean corpuscular Hb concentration (MCHC) 14. Folate supplements, splenectomy /cholecystecomy

Question 38

Thalassaemia 1. Inheritance pattern, what part of Hb affected 2. Type of anaemia 3. Clinical features - general 4. Alpha - chromosome, specific features, management 5. Beta - chromosome 6. B-major - definition 7. B-intermedia = 8. B-minor = 9. What cells may indicate thalassaemia

Answer 38

1. Autosomal recessive; Globin chain - alpha / beta 2. Microcytic 3. Delayed growth, congestive HF, splenomegaly, bone deformity, increased risk of infections, iron overload 4. 16, pronounced forehead, blood/marrow transfusions, splenectomy 5. 11 6. 2 deletion genes (no B-globin production) - severe microcytic anaemia, splenomegaly, bone deformities. Regular transfusion, chelation, spleen out, BM transplant 7. 2 defective/deletion genes, occasional transfusions 8. Carrier of one abnormal, mild microcytic, monitoring 9. Target cells

Question 39

Anaemia in children - causes 1. RBC destruction 2. RBC loss 3. RBC slow production

Answer 39

1. G6PD, sickle cell, thalassaemia, drug induced/viral haemolytic, physiological anaemia of newborn 2. Haemorrhagic disease of newborn, cow's milk protein enteropathy, clotting disorders, menstruation 3. Iron deficiency, chemotherapy, leukaemia

Question 40

Idiopathic thrombocytopenic purpura (ITP) 1. What it is 2. Clinical features 3. History 4. Investigation 5. Management - general 6. Management if severe (platelets <10) 7. Platelet count should be normal within how long 8. Other differential for acute purpura with low platelets

Answer 40

1. Virus leads to/spontaenous development of platelet membrane glycoprotein AB - so autoimmune platelet destruction (type II hypersensitivity) 2. Thrombocytopaenia (other bloods normal), petechial rash, purpura bruising/ecchymoses, strange bleeding 3. Child <10 years old, post-viral, symptoms over 1-2 days 4. Bone marrow aspirate before giving corticosteroids 5. Avoid high bleeding risk activities - contact sports, invasive procedures, NSAIDs/aspirin, safety netting 6. IV Ig, PO prednisolone / transfusions (blood/platelets) 7. 3 months (monitor until normal again) 8. Acute lymphoblastic leukaemia

Question 41

Henoch-Schonlein Purpura 1. AKA 2. Precipitants (2) 3. Clinical features 4. Rash - description 5. Acute investigations 6. Management 7. Follow up tests (2) 8. Complications 9. Other differentials for purpura with NORMAL platelets

Answer 41

1. IgA vasculitis 2. Infection (URTI/gastroenteritis), vaccination 3. Commonest in <10yo, purpura, arthralgia (knee/ankle), abdominal pain, IgA nephritis (haematuria, proteinuria) 4. Raised, purpuric (buttocks + limb extensor surfaces) 5. BP, urine dip, renal function, albumin, FBC, clotting 6. Symptomatic (analgesia) NO NSAIDs IF RENAL IMPAIRMENT 7. BP, urine dip 8. GI haemorrhage, intussusception, infarction. 9. Viral infection, NAI, meningitis, septicaemia

Question 42

Other childhood cancers 1. Along sympathetic chain / adrenal glands 2. In kidney 3. Central skeleton 4. Long bones 5. Rhabdomyosarcoma - can be found where (4)

Answer 42

1. Neuroblastoma 2. Nephroblastoma (Wilm's tumour) 3. Ewing's sarcoma 4. Osteosarcoma 5. Bladder, pelvis, nasopharynx, parameningeal

Question 43

Enuresis 1. Age at which should be dry in day 2. Age at which should be dry at night 3. Primary nocturnal 4. Causes 5. Management 6. Secondary nocturnal 7. Causes 8. Diurnal 9. Pharmacological management

Answer 43

1. 2 years 2. 3-4 years 3. Never managed to be dry at night 4. Normal variant (FH), overactive bladder, fluid intake, failure to wake, chronic constipation 5. Lifestyle change, encouragement 6. Have previously been dry for >6 months 7. UTI, constipation, T1DM, psychosocial 8. In daytime - urge (overactive bladder) and stress 9. Desmopressin (NE), oxybutinin (overactive bladder), imipramine

Question 44

Other renal/urological diseases 1. ARPKD (chromosome 6) - presentation 2. Lack of amniotic fluid leads to 3. Other feature 4. Multicystic dysplastic kidney (MCDK) - diagnosis 5. Wilms tumour - presentation, management 6. Posterior urethral valve - signs on antenatal USS 7. Presentation

Answer 44

1. Oligohydramnios + PKs in collecting ducts (foetal USS) 2. Potter syndrome (underdeveloped ear cartilage, low set ears, flat nasal bridge, skeletal abnormalities), foetal pulmonary hypoplasia 3. Liver fibrosis 4. Antenatal USS (one kidney cystic, one normal) 5. Child <5yo with abdominal mass/pain, haematuria, lethargy, fever, HTN, weight loss - nephrectomy 6. Oligohydramnios, hydronephrosis 7. Young boy, difficulty urinating, recurrent UTI

Question 45

Systems review - history 1. General 2. Cardiorespiratory 3. GI 4. GU 5. Neurological 6. ENT 7. Birth history 8. Specific feeding questions 9. Social

Answer 45

1. Fever, weight loss, night sweat, growth, alert, feeding 2. Cough, wheeze, stridor, sweating, dyspnoea, cyanosis 3. Feeding, abdo pain, N+V, diarrhoea, constipation 4. Wet nappies, frequency, urgency, continence, dysuria 5. Seizures, headaches, abnormal movements 6. Sore throat, sore ear, noisy breathing 7. Pregnancy issues, born at term, birth weight, mode of delivery, perinatal issues, neonatal issues 8. Breast/bottle fed, type of formula, cow's milk, weaning (normal 6-12 months) 9. Who's at home, smokers, family tree, nursery/school, any social services involvement

Question 46

Genetic profiles 1. Edward's syndrome 2. Patau's syndrome 3. Klinefelter syndrome 4. Turner's syndrome (+ feature) 5. Fragile X - clinical features 6. Lynch syndrome - inheritance, high risk of what Subsequent surveillance 7. Familial adenomatous polyposis - mutation, what Surveillance 8. Prader-Willi - clinical features

Answer 46

1. Trisomy 18 2. Trisomy 13 3. XXY 4. XO - short stature, webbed neck, wide carrying angle, horseshoe kidney, coarctation of aorta, infertility 5. LDs, large low ears, long thin face, high palate, autism, macroorchidism, hypotonia, mitral valve prolapse 6. AD; high risk of colorectal + endometrial carcinoma Colonoscopy every 1-2 years from age 25 7. APC gene mutation; leads to 100% colon cancer risk Colonoscopys every 5 years if have FAP but no polyps 8. Hypotonia in infancy, short, LDs, hypogonadism, infertility, childhood obesity, behavioural problems

Question 47

Chicken pox 1. Cause 2. Presentation - initial 3. Then what 4. Infectious from when to when 5. Management - supportive 6. VZ immunoglobulin (VZIG) if 7. Consider IV aciclovir if 8. Common complication, + what cause 9. Other complications 10. Shingles - what it is

Answer 47

1. VZV primary infection, spread via respiratory route 2. Initially a fever 3. Itchy rash, starts on head/trunk then spreads (macular, then papular , then vesicular); mild systemic upset 4. 4 days before rash to 5 days after rash first appeared (when lesions are dry/crusted over) 5. Keep cool, trim nails, camamile lotion, off school 6. Immunocompromised, or newborns with peripartum exposure 7. If chickenpox then develops in above patients 8. Secondary bacterial infection (group A strep) 9. Pneumonia (miliary), encephalitis (cerebellar involvement) disseminated haemorrhagic chickenpox, arthritis, nephritis, pancreatitis 10. Reactivation of dormant VZV in dorsal root ganglion

Question 48

Other infectious diseases - presentation 1. Measles - prodrome (3), causative virus 2. Full presentation 3. Mumps 4. Rubella 5. Scarlet fever 6. Hand, foot + mouth disease 7. Infectious mononucleosis - cause 8. Presentation 9. Produces what after up to 6 weeks, test via what (2) 10. Complications

Answer 48

1. Irritable, conjunctivitis, fever (Paramyxovirus) 2. Koplik spots (white spots on buccal mucosa) Maculopapular rash - starts behind the ear, spreads to whole body, becomes blotchy and confluent 3. Fever, malaise, myalgia, parotitis (earache/pain if eats) 4. Pink maculopapular rash (on face, spreads everywhere, fades by day 3-5) Lymphadenopathy (posterior auricular and suboccipital) 5. Fever, malaise, tonsillitis, strawberry tongue Fine punctuate erythema sparing the face 6. Vesicles (mouth, palms/soles), mild systemic upset 7. EBV 8. Sore throat, itchy rash post-amoxicillin/cephalosporin 9. Heterophile antibodies; Monospot/Paul-Bunnell tests 10. Splenic rupture, GN, haemolytic anaemia, thrombocytopaenia, Burkitt's lymphoma

Question 49

Development - normal 1. Primitive reflexes (4) 2. Gross M - age to roll + sit unsupported (curved back) 3. Stand unaided 4. Walk - unsteady vs steady 5. Run, then jump/kick ball 6. Fine M - palmar grasp 7. Crude-fine pincer grip 8. Building blocks - tower of 2, then 3 9. Speech/language - turns to own name + babbling 10. Few words 11. 2 part commands, then what/where, then 3 part commands and why/when/how 12. Talks about past/present/future 13. Social - stranger anxiety (peaks + lost when) 14. Shares

Answer 49

1. Moro (<3 months) Grasp (<3 months) Stepping (<4, >12 months) Asymmetrical tonic neck reflex (< 6 months) 2. 5-6 months 3. 10 months 4. 12 vs 15 months 5. 18 months, then 2 years 6. 6 months 7. 9-10 months 8. 15 months, then 18 months 9. 3-4 months 10. 12 months 11. 2 years, then 3 years, then 4 years 12. 5 years 13. Peaks 8-9 months, loses at 2 years 14. 3 years

Question 50

Developmental delay 1. Broad causes (3) 2. C/M - examples 3. M - examples 4. Ch - examples 5. Global delay - causes 6. Gross motor 7. Fine motor 8. Language 9. Personal/social

Answer 50

1. Cerebral/muscular, metabolic, chromosomal 2. Cerebral palsy, Duchenne's muscular dystrophy 3. Foetal alcohol, epilepsy, inborn (mitochondrial) 4. Down's, Edward's, Patau's, Fragile X 5. Metabolic and chromosomal conditions 6. CP, ataxia, vision, DMD, myopathy, spina bifida 7. CP, ataxia, visual impairment 8. Deafness, learning disability, neglect, autism 9. Emotional/social neglect, parenting issues, autism

Question 51

Ortho - brief 1. Hip disorders - limping child - investigations 2. 0-4 years (3) 3. 5-10 years (3) 4. 10-16 years 5. Knee pain - Osgood–Schlatter disease - rest, NSAIDs 6. Patellar tendonitis 5. Talipes equinovarus - positional abnormality 6. Talipes calcaneovalgus 7. Management 8. Osteogenesis imperfecta - features

Answer 51

1. Bilateral hip x-ray, FBC, CRP, metabolic panel (SUFE) 2. DDH, septic arthritis, transient sinovitis 3. Septic arthritis, transient sinovitis, Perthe's disease 4. Septic arthritis, SUFE, Perthe's disease 5. Tibial epiphyseal inflammation causing prominent tibial tuberosity + knee pain on exertion (sporty 10-15yo boys) 6. Athletic teenage boys; chronic anterior knee pain that worsens after running, sub-patellar tenderness 5. Plantar flexion and supination 6. Dorsiflexion and pronation 7. Ponseti method - physio, achilles tenotomy, brace 8. Easy fractures, lax ligaments, blue/grey sclera, triangular face, deafness from early adulthood

Question 52

Psychiatry - differences in paediatrics 1. 1st line antidepressant 2. Medication in OCD 3. ADHD - 1st line medication + SEs (6 week trial) 4. Monitoring requirements

Answer 52

1. Fluoxetine 10mg 2. SSRIs 3. Methylphenidate (dopamine/norepinephrine RI); abdominal pain, nausea, dyspepsia 4. ECG at start (potentially cardiotoxic), weight and height every 6 months in children

Question 53

Failure to thrive - general causes 1. Structural barrier to adequate nutrition (3) 2. Malabsorption (4) 3. Inadequate nutrition (4) 4. Increased energy requirements (4) 5. Cannot process nutrients properly (1)

Answer 53

1. Abnormal facial structure, cleft, pyloric stenosis 2. Cystic fibrosis, coeliac, cow's milk protein allergy, chronic diarrhoea 3. Maternal malabsorption (breastfeeding), iron deficiency anaemia, neglect, poverty 4. Hyperthyroidism, chronic disease, malignancy, chronic infection (HIM, immunodeficiency) 5. Inborn errors of metabolism

Question 54

APGAR score 1. Measured out of 2. Done when 3. Stands for 4. A 5. P 6. G 7. A 8. R

Answer 54

1. 10 2. 1, 5, 10 minutes 3. Appearance, pulse, grimmace, activity, respiration 4. Central cyanosis (0), peripheral cyanosis (1), pink (2) 5. Absent (0), <100 (1), >100 (2) 6. No response (0), little response (1), good response (2) 7. Floppy (0), flexed limbs (1), active (2) 8. Absent (0), slow/irregular (1), strong/crying (2)

Question 55

Blood spot (heel prick) screening 1. When 2. Which diseases (9) 3. How long for results to come back

Answer 55

1. 5-8 days 2. Sickle cell, CF, hypothyroid, 6x metabolism (phenylketonuria, MCADD, maple syrup urine disease, isovaleric acidaemia, glutaric aciduria T1, homocystinuria) 3. 6-8 weeks

Question 56

Birth injuries 1. Caput seccendaneum 2. Cephalohaematoma (traumatic subperiosteal haematoma) 3. Facial paralysis - cause, management (forceps) 4. Erb's palsy - injured area 5. Associated with 6. Clinical features 7. Other injury associated with shoulder dytocia

Answer 56

1. Scalp oedema (outside periosteum), crosses suture lines 2. Blood between skull + periosteum, does not cross suture lines; increased risk of anaemia/jaundice 3. From forceps; CN 7 function returns over few months 4. C5/C6 nerves in the brachial plexus 5. Shoulder dytocia, traumatic birth 6. 'Waiter's tip' - shoulder internal rotation, elbow extension, wrist flexed facing backwards (pronation) 7. Clavicle fracture

Question 57

Childhood vaccines 1. '6-in-1' vaccine - components 2. Given when 3. 8 weeks - other vaccines given (3) 4. 12 weeks (1) 5. 16 weeks (2) 6. 1 year (4) 7. 3 years 4 months (2) 8. 12-13 years (1) 9. 14 years (2) 10. Yearly flu jabs between what ages

Answer 57

1. Diphtheria, tetanus, pertussis, polio, HI B, hepatitis B 2. 8, 12 and 16 weeks of age (2, 3, 4 months) 3. Pneumococcal (PCV), rotavirus, meningitis B (Men B) 4. Rotavirus 5. PCV, Men B 6. PCV, Men B, Hib/Men C, MMR 7. MMR, 4-in-1 preschool booster (DTP, pertussis/WC) 8. HPV vaccine 9. Men ACWY, 3-in-1 teenage booster (DTP) 10. 2-10 years