Paediatrics

Question 1

Childhood vaccinations at age 8 weeks / 2 months

Answer 1

Diphtheria/tetanus/pertussis/Haemophilus influenza type B(HiB)/ Hep B (6 in 1 vaccine)
Men B
Rotavirus (oral)

Question 2

Childhood vaccinations at age 12 weeks / 3 months

Answer 2

Diphtheria/tetanus/pertussis/Haemophilus influenza type B/ Hep B (6 in 1 vaccine)
Pneumococcal conjugate vaccine (PCV)
Rotavirus

Question 3

Childhood vaccinations at age 16 weeks / 4 months

Answer 3

Diphtheria/tetanus/pertussis/Haemophilus influenza type B/ Hep B (6 in 1 vaccine)
Men B

Question 4

Childhood vaccinations at age 12-13 months

Answer 4

Hib B/Men C
PCV
MMR
Men B

Question 5

Childhood vaccinations at age 3 yrs 4 months

Answer 5

Diphtheria, tetanus, pertussis, polio

MMR

Question 6

Childhood vaccinations at age 12-13 yrs

Answer 6

HPV types 6,11,16,18 (both boys and girls)

Question 7

Childhood vaccinations at age 14 yrs

Answer 7

Men ACWY

Tetanus/diphtheria/polio

Question 8

Developmental milestone: sits without support

Answer 8

7 months (>9 months = red flag)

Question 9

Developmental milestone: stands independently

Answer 9

12 months (>12 months = red flag)

Question 10

Developmental milestone: Walks unsupported

Answer 10

13 - 15 months (>18 months = red flag)

Question 11

Developmental milestone: Pincer grip

Answer 11

10 months (>12 months = red flag)

Question 12

Developmental milestone: Drawing

Answer 12

2 1/2 yrs

Question 13

Developmental milestone: 2-3 words / understands name

Answer 13

1 year

Question 14

Developmental milestone: 6-10 words

Answer 14

15 - 18 months

Question 15

Developmental milestone: startling at loud noise & follows face

Answer 15

2 months (>3 months = red flag)

Question 16

Developmental milestone: smiling

Answer 16

6 weeks (>8 weeks = red flag)

Question 17

Developmental milestone: plays near others but not with them

Answer 17

2 yrs

Question 18

Developmental milestone: joining two words

Answer 18

2 yrs (>2 yrs = red flag)

Question 19

Developmental milestone: stranger fear

Answer 19

7 months (>10 months = red flag)

Question 20

Paeds BLS compression to breath ratio

Answer 20

15:2

Question 21

First step of paeds BLS

Answer 21

5 rescue breaths before commencing CPR

Question 22

Peads foreign body airway obstruction (FBAO)

Answer 22

5 back blows 
5 thrusts (abdo if >1yr & chest if <1yr)

Question 23

Neonatal life support Step 1

Answer 23

dry & stimulate baby

Question 24

Neonatal life support Step 2

Answer 24

5x inflation breaths if still gasping/not breathing with open airway

Question 25

Neonatal life support Step 3

Answer 25

CPR if not breathing/ HR < 60 | ratio 3:1 (compression : ventilation)

Question 26

APGAR score groups

Answer 26

``` 0-3 = bad 4-6 = moderate 7-10 = good ```

Question 27

Risk factors for Sudden Infant Death Syndrome (SIDS)

Answer 27

``` prone sleeping parental smoking prematurity bed sharing male sex ```

Question 28

Protective factors for SIDS

Answer 28

breast feeding | supine sleeping

Question 29

Most common genetic cause fo trisomy 21

Answer 29

meiotic non-disjunction of chromes 21 (known as full trisomy 21), has 3 full chromosome 21

Question 30

Risk factors for trisomy 21

Answer 30

↑maternal age, previous child with down syndrome

Question 31

Physical features of trisomy 21

Answer 31

``` hypotonia epicanthal folds brushfield spots on iris flat nasal bridge single palmar crease small low set ears short stature ```

Question 32

Trisomy 21 investigations to confirm diagnosis

Answer 32

chromosomal karyotype | fluorescent in situ hybridisation (FISH)

Question 33

Congenital heart defects associated with trisomy 21

Answer 33

atrioventricular septal defect (AVSD) most common | ASD, VSD, tetralogy of fallot

Question 34

Screening of congenital heart defects in trisomy 21

Answer 34

all neonates require an echo

Question 35

Congenital GI defects in trisomy 21

Answer 35

duodenal / oesophageal atresia | imperforate anus

Question 36

How frequent is trisomy 21 related hearing loss

Answer 36

90% of pts have some extend of hearing loss

Question 37

Hearing screen for trisomy 21

Answer 37

Screen hearing at birth, 6 months, 12 months and annually there after

Question 38

Trisomy 21 related visual problem at birth

Answer 38

congenital cataracts (absent red reflex - leukocoria)

Question 39

Thyroids screening for trisomy 21

Answer 39

annual TFTs | ↑ risk of hypothyroidism

Question 40

Trisomy 21 related cancer

Answer 40

↑ risk of acute lymphoid leukaemia & acute myeloid leukaemia

Question 41

Most common trisomy 21 related sleep problem

Answer 41

Obstructive sleep apnoea (60% of children) | sleep study recommended age 3-4 yrs

Question 42

Orthopaedics disorders associated with trisomy 21

Answer 42

atlanto-axial instability | scoliosis

Question 43

Monitoring tests for Trisomy 21 pts

Answer 43

hearing screen: at birth, 6 months, 12 months and then annually TFTs: at birth, 6 months, annually Eye screening: at birth, 6 months, then yearly till age 5 Sleep study: age 3-4 echo: at birth coeliac disease screen

Question 44

leading cause of death in trisomy 21 pts after age 40

Answer 44

dementia

Question 45

Trisomy 13 (patau syndrome) presentation

Answer 45

microcephaly polydactyly small eyes midline facial defects

Question 46

Trisomy 13 (patau syndrome) prognosis

Answer 46

very poor, usually stillborn/spontaneously aborted | if born alive usually dead in a week

Question 47

which is more common Trisomy 18 (edward syndrome) vs Trisomy 13 (patau syndrome)

Answer 47

``` Trisomy 18 (edward syndrome) is 2nd most common autosomal trisomy Trisomy 13 (patau syndrome) is 3rd most common autosomal trisomy ```

Question 48

Most common management of Trisomy 21/18/13 if detected prenatally

Answer 48

abortion of the foetus

Question 49

Trisomy 18 (edward syndrome) presentation

Answer 49

``` low birth weight microcephaly micrognathia prominent occiput small facial features (microstomia/micropthalmia) overlapping fingers rocker bottom feet ```

Question 50

Trisomy 18 (edward syndrome) prognosis

Answer 50

often stillbirth/death in labour | <10% survive to age 1 year

Question 51

Fragile X inheritance pattern

Answer 51

X linked dominant inheritance

Question 52

Fragile X presentation

Answer 52

``` intellectual disability macrocephaly long face large ears macro-orchidist hyperflexibility ```

Question 53

Fragile X genetic cause

Answer 53

expansion of CGG triplet repetition on the FMR1 gene

Question 54

Noonan syndrome

Answer 54

Autosomal dominate | Features: webbed neck, pectus excavatum, short stature, low set ears

Question 55

Pierre-Robin syndrome

Answer 55

Features: micrognathia, posterior displaced tongue (can cause airway obstruction)

Question 56

Prader-Willi syndrome

Answer 56

Features: hypotonia, hypogonadism, obesity, T2DM

Question 57

Williams syndrome

Answer 57

Features: elfin face, learning difficulties, friendly/extroverted personality, Supravalvular aortic stenosis

Question 58

Cri du Chat syndrome

Answer 58

Features: characteristic cat like cry, feeding difficulties, poor weight gain

Question 59

DiGeorge syndrome

Answer 59

Features: CHD, abnormal facies, cleft palate, developmental delay

Question 60

Turner syndrome genetic cause

Answer 60

complete or partial absence of second X chromosome in females, 45XO/45X

Question 61

Turner syndrome features

Answer 61

``` poor growth short stature lymphoedema of hands and feet webbed neck shielded chest wide spaced nipples wide carrying angle absent pubertal development infertility ```

Question 62

Turner syndrome associated cardiac abnormalities

Answer 62

bicuspid aortic valve | coarctation of the aorta

Question 63

Hormone levels in turner syndrome

Answer 63

LH & FHS ↑ Antimullerian hormone↓

Question 64

Klinefelter's syndrome genetic cause

Answer 64

males with extra X chromosome (47XXY)

Question 65

Klinefelter's syndrome features

Answer 65

``` tall & slender physique wide hips small firm testes impotence ↓ facial & pubic hair gynaecomastia learning disability ```

Question 66

Hormone levels in Klinefelter's syndrome

Answer 66

↓ testosterone, LH & FHS ↑

Question 67

Klinefelter's syndrome associated cardiac abnormalities

Answer 67

↑ risk of mitral valve prolapse

Question 68

cancer associated with Klinefelter's syndrome

Answer 68

↑ risk of male breast cancer & germ cell tumours

Question 69

Klinefelter's syndrome management

Answer 69

``` testosterone replacement (as pt enter puberty) intracytoplasmic sperm injection if they are trying to conceive ```

Question 70

Neurofibromatosis (NF) types

Answer 70

NF-1: chromosome 17 gene mutation NF-2: chromosome 22 gene mutations

Question 71

Neurofibromatosis type 1 presentation

Answer 71

cafe au lait spots axillary & inguinal freckles neurofibromas Iris hamartomas

Question 72

Neurofibromatosis type 2 presentation

Answer 72

``` bilateral acoustic neuromas/vestibular schwanomas cafe au lait spots juvenile cataracts meningiomas intracranial schwanomas (multiple) ```

Question 73

Investigating Neurofibromatosis (NF)

Answer 73

MRI/CT scan | genetic testing

Question 74

Differentiating Neurofibromatosis (NF) types 1 and 2

Answer 74

NF-1 : more common in children, usually >6 cafe au lait spots NF-2 : more common in adults, usually <6 cafe au lait spots

Question 75

Neurofibromatosis (NF) differential diagnosis

Answer 75

tuberous sclerosis | which is differentiated by presence of ash-leaf spots (seen under UV light), retinal hamartomas, epilepsy

Question 76

Cystic fibrosis (CF) genetics

Answer 76

abnormalities in salt and water transport across epithelial surfaces due to mutations in CF transmembrane conductance regulator (CFTR) gene on chromes 7 most common mutation in caucasians = DF508 (delta F508)

Question 77

Ethnic group is at ↑ risk of cystic fibrosis

Answer 77

white population | ↓ incidence in africans/hispanic/asian

Question 78

Pathophysiology of cystic fibrosis

Answer 78

impaired salt & water transport by epithelial cells leads to thick sticky excretions, which can affect the pancreas/GI tract/Bilairy tree/resp system/sweat

Question 79

Presentation of cystic fibrosis in neonates

Answer 79

meconium ileus/delayed passage of meconium | prolonged jaundice

Question 80

Presentation of cystic fibrosis in older children

Answer 80

``` recurrent chest infections/chronic pulmonary disease wet sounding cough purulent sputum wheezes nasal polyps failure to thrive steatorrhoea ```

Question 81

Investigating cystic fibrosis

Answer 81

found on heelprick test (day 5-7 after birth) Sweat test (↑ Cl- concentration >60mmol/L) genetic testing CXR Lung function test (FEV1/FVC ↓) stool elastase (for pancreatic dysfunction)

Question 82

Common pathogens in cystic fibrosis pts

Answer 82

``` staph aureus H influenzas pseudomonas aeroginosa aspergillus Burkholderia cepacia ```

Question 83

Managing respiratory problems in cystic fibrosis pts

Answer 83

chest physio & mucous clearance techniques ABx mucolytics e.g. dornase alfa

Question 84

Management of pancreatic insufficiency in cystic fibrosis

Answer 84

confirm via stool elastase test | pancreatic enzyme therapy

Question 85

Recommended diet for cystic fibrosis pts

Answer 85

high fat, high protein, high calorie diet

Question 86

Fertility problems related to cystic fibrosis

Answer 86

almost all males suffer from azoospermia so should be counselled on IVF for conception

Question 87

Biological treatments of cystic fibrosis

Answer 87

lumacaftor & Ivacaftor | to treat homozygous pts for DF508, help increase CFTR protein numbers

Question 88

Presentation of meconium ileus

Answer 88

24-48h post birth with failure to pass meconium, abdo distension and bilious vomiting NB seen in ~20% of CF pts

Question 89

Management of meconium ileus

Answer 89

surgical decompression

Question 90

Genetic cause of sickle cell disease

Answer 90

autosomal recessive gene defect of the 17th nucleotide of the beta chain leading to the substitution of valine instead of glutamic acid

Question 91

screening for sickle cell disease

Answer 91

part of the routine heel prick test screening on day 5-7 after birth

Question 92

timeline of presentation for sickle cell disease

Answer 92

symptomatic presentation between age 3-6 months as HbF levels fall

Question 93

presentation of sickle cell disease

Answer 93

``` jaundice pallor anaemia growth restriction failure to thrive lethargy systolic flow murmur ```

Question 94

The spleen in sickle cell disease

Answer 94

↑ risk of infection by encapsulated organisms e.g. pneumococcus highest risk of overwhelming infection in <3y/o recurrent infections lead to autosplenectomy

Question 95

Vaso-occlusive crisis in sickle cell

Answer 95

most common type of crisis | obstruction of micro circulation by sickle cells leading to ischaemia

Question 96

precipitating factors of vaso-occlusive crisis in sickle cell

Answer 96

cold, infection, dehydration, exertion

Question 97

presentation of vaso-occlusive crisis in sickle cell

Answer 97

swollen joints pain tachypnoea priapism if major vessels may present as thrombotic stroke/acute sickle chest syndrome

Question 98

aplastic crisis in sickle cell

Answer 98

temporary cessation of erythropoiesis leading to severe anaemia

Question 99

trigger of aplastic crisis in sickle cell

Answer 99

parvovirus B19 infection

Question 100

presentation of aplastic crisis in sickle cell

Answer 100

generally rapid drop in Hb over 1 week with spontaneous recovery may have high output congestive HF

Question 101

sequestration crisis in sickle cell

Answer 101

sudden enlargement of spleen leading to ↓ Hb & circulatory collapse which causes hypovolaemic shock

Question 102

at risk groups for sequestration crisis in sickle cell

Answer 102

young children snd babies

Question 103

presentation of sequestration crisis in sickle cell

Answer 103

↓ Hb, ↑reticulocytes, splenomegaly

Question 104

management of recurrent sequestration crisis in sickle cell

Answer 104

splenectomy

Question 105

differentiating sequestration crisis in sickle cell & aplastic crisis in sickle cell

Answer 105

aplastic = ↓ Hb but no ↑reticulocytes | sequestration ↓ Hb, ↑reticulocytes, splenomegaly

Question 106

Acute chest crisis in sickle cell

Answer 106

vaso-occlusive crisis of the lungs

Question 107

diagnostic criteria for Acute chest crisis in sickle cell

Answer 107

new pulmonary infiltrates on CXR + ≥ 1 of - cough - fever - sputum production - tachypnoea - dyspnoea - hypoxia

Question 108

investigating sickle cell disease

Answer 108

``` FBC (↓ Hb, ↑reticulocytes) blood film (sickle cells) haemoglobin electrophoresis (to confirm diagnosis, absence of HbA, but presence of HbS) ```

Question 109

general management of sickle cell disease

Answer 109

Patient & parental education folic acid/zinc/Vit D supplementation unconjugated pneumococcal vaccine (from age 2) oral penicillin prophylaxis hydroxycarbamide (,2y/o to ↓ frequency of crisis by stimulation HbF) blood transfusion

Question 110

treatment of sickle cell disease in pregnancy

Answer 110

prophylactic LMWH due ↑ risk of prematurity/neonatal death/low birth weight low dose aspirin 75mg from 12 weeks

Question 111

Duchenne muscular dystrophy (DMD) inheritance

Answer 111

X-linked recessive

Question 112

Duchenne muscular dystrophy (DMD) pathophysiology

Answer 112

mutation leads to absence of dystrophin protein leading to muscle degeneration/necrosis with muscle being replaced by adipose tissue

Question 113

Presentation of Duchenne muscular dystrophy (DMD)

Answer 113

``` delayed motor milestones calf hypertrophy waddling gait/inability to run Gower's sign (climbing up legs when standing up) heel contractures lordosis ↓ tendon reflexes ↓muscle tone ```

Question 114

Investigating Duchenne muscular dystrophy (DMD)

Answer 114

``` Creatine kinase (↑10-100x normal levels) genetic testing muscle biopsy (check for dystrophin protein) ```

Question 115

Normal test result excluding Duchenne muscular dystrophy (DMD)

Answer 115

normal creatine kinase excludes diagnosis of DMD

Question 116

Managing Duchenne muscular dystrophy (DMD) early stages

Answer 116

Physiotherapy knee-foot-ankle orthosis corticosteroids e.g. prednisolone (prolongs ambulation)

Question 117

Managing Duchenne muscular dystrophy (DMD) late stages

Answer 117

mobility aids respite care NIV regular cardiology reviews (6 monthly)

Question 118

Spinal muscular atrophy (SMA)

Answer 118

slow progressive atrophy & weakness of limb muscles due to SMN1 gene mutation, leading to lower motor neurone weakness generally autosomal recessive

Question 119

Features of Spinal muscular atrophy (SMA)

Answer 119

``` muscle weakness & wasting preserved intellect flaccid weakness hypotonia ↓ tendon reflexes fasciculation's ```

Question 120

most common type of Spinal muscular atrophy (SMA)

Answer 120

SMA type II most common (chronic infantile SMA)

Question 121

Investigating Spinal muscular atrophy (SMA)

Answer 121

``` creatine kinase (normal) genetic testing ```

Question 122

Charcot-Marie-Tooth disease

Answer 122

heterogenous group of peripheral neuropathies which is the most common inherited neuromuscular disorder usually autosomal dominant inheritance

Question 123

Presentation of Charcot-Marie-Tooth disease

Answer 123

slowly progressive (CMT1 most common onset by age 10) muscle weakness & wasting (starting in intrinsic foot muscles) muscle weakness spreads to lower legs & thigh sensory loss following same pattern as muscle weakness (especially ↓vibration & light touch) generalised tendon areflexia

Question 124

Necrotising enterocolitis (NEC)

Answer 124

ischaemic inflammatory bowel necrosis | most common GI emergency in neonates

Question 125

Risk factors for necrotising enterocolitis (NEC)

Answer 125

prematurity ↓ birth weight PDA

Question 126

presentation of Necrotising enterocolitis (NEC)

Answer 126

``` classically in preterm infants within first 2 weeks of life abdo distension altered stool pattern bloody mucoid stool bilious vomiting ↓ bowel sounds lethargy ```

Question 127

investigating necrotising enterocolitis (NEC)

Answer 127

FBC ABG/VBG baseline biochem AXR (dilated bowel loops, intramural gas, Ringler sign)

Question 128

AXR findings in necrotising enterocolitis (NEC)

Answer 128

dilated bowel loops bowel wall oedema intramural gas (pneumatosis intestinalis) portal venous gas Rigler sign Football sign (air outlining falciform ligament)

Question 129

managing necrotising enterocolitis (NEC)

Answer 129

``` Nil by mouth NG tube to decompress bowel IV fluids/parenteral nutrition IV ABx (cefotaxime +metronidazole/clindamycin) surgery ```

Question 130

Exomphalos (omphalocele)

Answer 130

abdo contents herniated into umbilical cord via umbilical ring, viscera covered by a membrane

Question 131

Gastroschisis

Answer 131

abdominal contents herniating into amniotic sac without covering membrane usually to right side of umbilicus

Question 132

Investigating gastroschisis & exomphalos

Answer 132

``` maternal AFP (↑) USS ```

Question 133

Managing exomphalos

Answer 133

surgical repair fo defect, abdominal contents returned to abdomen

Question 134

Managing gastroschisis

Answer 134

surgical repair | requires more pre-op care than exomplhalos with IV fluids, radiant heaters etc

Question 135

congenital diaphragmatic hernia

Answer 135

incomplete fusion of diaphragm leading to herniation of abdo contents into thorax which causes pulmonary hypoplasia

Question 136

common side of congenital diaphragmatic hernia

Answer 136

left side | liver plugs space on right

Question 137

congenital diaphragmatic hernia prenatal presentation

Answer 137

often prenatal diagnosis (polyhydraminos & via routine USS)

Question 138

congenital diaphragmatic hernia presentation

Answer 138

``` presents soon after birth cyanosis tachypnoea chest wall asymmetry absent breath sounds on affected side bowel sounds audible on chest wall ```

Question 139

Managing congenital diaphragmatic hernia

Answer 139

``` intubate & venitalte at minimal pressures orogastric tube (to locate stomach on X-ray) surgery to fix diaphragm ```

Question 140

Hirschsprung's disease

Answer 140

congenital condition characterised by partial/complete colonic functional obstruction associated with absence of parasympathetic ganglion cells = tonically constricted lamina = functional obstruction

Question 141

Disease associated with Hirschsprung's disease

Answer 141

Down's syndrome

Question 142

Hirschsprung's disease initial presentation

Answer 142

failure to pass meconium in first 48h of life repeated bilious vomiting abdo distension explosive passage of liquid & foul smelling stools (especially post PR exam) failure to thrive

Question 143

later presentation of Hirschsprung's disease

Answer 143

chronic constipation resistant to treatment

Question 144

investigating Hirschsprung's disease

Answer 144

AXR contrast enema (contracted distal bowel & dilated proximal bowel) rectal biopsy

Question 145

Hirschsprung's disease treatment

Answer 145

Surgery

Question 146

Hirschsprung's disease prognosis

Answer 146

most pts acquire normal fecal continence & normal bowel habits

Question 147

Meckel's diverticulum

Answer 147

true diverticulum due to failure of the vitelline duct to obliterate most common congenital abnormality of the small bowel

Question 148

Location of Meckel's diverticulum

Answer 148

in distal ileum close to ileocaecal valve

Question 149

Meckel's diverticulum rule of 2's

Answer 149

occurs in 2% of population within 2 feet from ileocaecal valve 2 inches long

Question 150

Meckel's diverticulum presentation

Answer 150

generally asymptomatic painless Gi bleeding = haematochezia intractable constipation

Question 151

Meckel's diverticulum investigations

Answer 151

AXR (for bowel obstruction) Meckel's scan CT abdo

Question 152

Intussusception epidemiology

Answer 152

usually seen age 3-12 months | peak age 9 months

Question 153

commonest part of bowel affected by Intussusception

Answer 153

ileocaecal region | lead point is often an enlarged lymph node (peters patch) in terminal ileum

Question 154

Intussusception presentation

Answer 154

``` paroxysmal colicky abdo pain drawing knees up to chest crying early vomiting blood PR (red current jelly stools) child normal between bouts of pain ```

Question 155

Investigating Intussusception

Answer 155

Abdo USS (target sign/doughnut sign) AXR diagnostic enema

Question 156

Mangement of Intussusception

Answer 156

drip & suck (IV fluids & NG tube) radiological reduction via air enema surgical reduction

Question 157

Umbilical hernia

Answer 157

herniation of peritoneal sac covered with skin | generally spontaneously resolves in most children

Question 158

Umbilical hernia presentation

Answer 158

bulge at umbilicus with overlying skin | easily reducible

Question 159

Umbilical hernia management

Answer 159

generally observed till age 4-5 yrs if small | surgical closure if large (if asymptomatic then by age 2-3 yrs)

Question 160

Inguinal hernia

Answer 160

due to patent processus vaginalis allowing abdominal contents to herniate into inguinal canal, presenting as bulge lateral to pubic tubercle

Question 161

Inguinal hernia management

Answer 161

urgent surgical management indicated due to ↑ risk of strangulation

Question 162

pyloric stenosis

Answer 162

infantile hypertrophic pyloric stenosis due to hypertrophy of the pyloric sphincter narrowing the pyloric canal

Question 163

risk factors for pyloric stenosis

Answer 163

first born male family history exposure to erythromycin in first 2 weeks of life

Question 164

time of presentation of pyloric stenosis

Answer 164

between 2-8 weeks of age | rare after 12 weeks of age

Question 165

Presentation of pyloric stenosis

Answer 165

non bilious vomiting within 30-60 minute of feeding projectile vomit upper abdominal mass (olive) baby remains hungry after feed frequency/intensity of vomiting ↑ over several days

Question 166

investigating pyloric stenosis

Answer 166

ABG/VBG (hypochloraemic, hypokalaemic metabolic alkalosis) | Abdo USS

Question 167

classic blood gas finding for pyloric stenosis

Answer 167

hypochloraemic, hypokalaemic metabolic alkalosis

Question 168

Management of pyloric stenosis

Answer 168

Ramstedt's pyloromyotomy

Question 169

Biliary atresia

Answer 169

progressive idiopathic necroinflammatory process involving the extra hepatic biliary tree leading to fibrosis

Question 170

time of presentation of biliary atresia

Answer 170

usually between 2-8 weeks after birth

Question 171

presentation of biliary atresia

Answer 171

``` usually seen in term infant with normal birth weight persistent jaundice play stools/dark urine failure to thrive hepatosplenomegaly ```

Question 172

Investigations for biliary atresia

Answer 172

total & conjugated bilirubin (↑ conjugated bilirubin) LFTs (generally ↑, disproportionate ↑ GGT) Liver histology (gold standard)

Question 173

management biliary atresia

Answer 173

``` surgery (portoenterostomy), best outcome if surgery before 8 weeks of age ursodeoxycholic acid (to encourage bile flow) ```

Question 174

oesophageal atresia

Answer 174

blind ending oesophagus presenting with frothing at mouth, feeding difficulties, chocking, respiratory distress

Question 175

oesophageal atresia investigation

Answer 175

``` antenatal USS (polyhydraminos, smaller/no stomach bubble) antenatal MRI (small stomach, oesophageal pouch) ```

Question 176

oesophageal atresia management

Answer 176

surgery | suctioning oesophageal pouch till surgery

Question 177

Risk factors of duodenal/oesophageal atresia

Answer 177

related to trisomy 21/13/18 twins NB duodenal atresia is particularly related to trisomy 21

Question 178

duodenal atresia presentation

Answer 178

abdo distension bilious/non-bilious vomiting presents in first days of life

Question 179

Investigations for duodenal atresia

Answer 179

AXR (double bubble sign)

Question 180

Volvulus/midgut rotation

Answer 180

a spectrum of rotation & fixation disturbances of the intestines occurring during embryonic development, volvulus is complete twisting of a loop of intestines

Question 181

Presentation of volvulus

Answer 181

``` rapid onset bilious/non bilious vomiting lactataemia metabolic acidosis oligouria hypotension feeding intolerance ```

Question 182

Volvulus/midgut rotation investigations

Answer 182

``` Volvulus = clinical diagnosis AXR (double bubble sign, air fluid levels) contrast studies (duodenojejunal junction displaced in malrotation) ```

Question 183

Managing Volvulus/midgut rotation

Answer 183

``` surgical treatment (even of asymptomatic malrotation due to risk fo volvulus) Ladd's procedure = treatment of choice ```

Question 184

hydrocele

Answer 184

collection of serous fluid between the layers of tunica vaginalis around testicles/along spermatic cord usually disappears age 1-2 yrs

Question 185

most common hydrocele in children

Answer 185

communicating hydrocele i.e. patent processus vaginalis allowing fluid to drain into scrotum from peritoneum

Question 186

presentation of hydrocele

Answer 186

scrotal enlargement (non tender, smooth cystic swelling, below/anterior to testicle) transluminates with pen torch swelling confined to scrotum i.e. able to get above it

Question 187

managing hydrocele

Answer 187

clinical diagnosis usually self limiting, so observe repair if not resolves after 2 yrs of age

Question 188

cryptorchidism

Answer 188

unilateral/bilaterally undescended testes, i.e. not present within the dependent portion of the scrotal sac

Question 189

when to refer cryptorchidism

Answer 189

specialist referral age 6 months (corrected) if still undescended with surgical correction in next year

Question 190

presentation of cryptorchidism

Answer 190

testes may be palpable in upper portion of scrotum/inguinal canal or may be absent (indicates intra-abdominal location)

Question 191

Management of cryptorchidism

Answer 191

if still undescended by age 3 months =pathological referral to specialist before 6 months surgical repair by 12-18 months of age

Question 192

cryptorchidism complications

Answer 192

risk of infertility if delayed diagnosis (especially >2y/o) ↑ risk of testicular torsion ↑ of testicular cancer

Question 193

Hypospadias

Answer 193

congenital abnormality of penis where urethral opening is somewhere along ventral aspect of penis

Question 194

Hypospadias common location

Answer 194

90% have meatus on/near glans = distal hypospadias

Question 195

Hypospadias associated condition

Answer 195

if associated with cryptorchidism then may suggest disorder of sexual differentiation

Question 196

Posterior urethral valves

Answer 196

cause obstruction of urethra | most common cause if UTI in male infants

Question 197

Posterior urethral valves presentation

Answer 197

poor, intermittent dribbling urine stream | ±frequent UTIs

Question 198

Posterior urethral valves complications

Answer 198

can cause high pressure & detrusor hypertrophy leading to vesicoureteric reflux causing hydronephrosis

Question 199

Posterior urethral valves diagnostics

Answer 199

``` voiding cystourethrography (VCUG) = gold standard postnatally NB most prenatal diagnosis ```

Question 200

Posterior urethral valves treatment

Answer 200

endoscopic ablation = gold standard

Question 201

meconium aspiration syndrome (MAS)

Answer 201

respiratory distress in the newborn due to presence of meconium in the trachea usually secondary to foetal hypoxia

Question 202

meconium aspiration syndrome (MAS) risk factors

Answer 202

``` post term infants (>42 weeks) fetal distress oligohydramnios chorioamnionitis NB rare in <34 weeks gestation ```

Question 203

meconium aspiration syndrome (MAS) presentation

Answer 203

``` meconium/green stained amniotic fluid green/blueish staining of skin at birth floppy baby ↓ APGAR score rapid/laboured/absent breathing bradycardia signs of post maturity (skin peeling, long stained nails) ```

Question 204

managing meconium aspiration syndrome (MAS)

Answer 204

suctioning O2/ventilatory support surfactant (if severe)

Question 205

infant respiratory distress syndrome (IRDS)

Answer 205

know as surfactant deficient lung disease or hyaline membrane disease usually seen in preterm infants due to surfactant deficiency

Question 206

Risk factors for infant respiratory distress syndrome (IRDS)

Answer 206

prematurity male infant c-section delivery without maternal labour maternal diabetes

Question 207

Presentation of infant respiratory distress syndrome (IRDS)

Answer 207

``` tachypnoea nasal flaring grunting intercostal/subcostal recession cyanosis ↓ air entry on auscultation ```

Question 208

infant respiratory distress syndrome (IRDS) investigations

Answer 208

ABG (↓ PaO2, ↑PaCO2, metabolic/respiratory acidosis) CXR (air bronchograms, reticular granular pattern) echo (to rule out congenital heart disease)

Question 209

Prevention of infant respiratory distress syndrome (IRDS)

Answer 209

``` maternal corticosteroids (dexamethasone) tocolytics (to delay premature delivery by ~48h while giving steroids) e.g. nifedipine ```

Question 210

infant respiratory distress syndrome (IRSD) management

Answer 210

surfactant replacement therapy (via ET tube) | O2/assisted ventilation

Question 211

Transient tachypnoea of the newborn (TTN)

Answer 211

delayed absorption of fluid in the lungs leading to ineffective gas exchange commonest cause of respiratory distress in newborn period

Question 212

Presentation of Transient tachypnoea of the newborn (TTN)

Answer 212

``` tachypnoea (DUHH ITS in the fucking name) respiratory distress (nasal flaring/grunting/subcostal/intercostal recession, crackles) ```

Question 213

Transient tachypnoea of the newborn (TTN) investigations

Answer 213

ABG (↓ PaO2, ↑PaCO2) | CXR (hyperinflation, fluid in horizontal fissure, prominent perihilar vascular markings)

Question 214

Transient tachypnoea of the newborn (TTN) management

Answer 214

supplementary O2 | usually self limiting on 1-2 days

Question 215

Persistent pulmonary hypertension of the newborn (PPHN)

Answer 215

defined as failure of the normal circulatory transition that occurs after birth, i.e. ≥ 1 of the fetal shuts fail to close leading to persistently elevated pulmonary pressures

Question 216

Risk factors for Persistent pulmonary hypertension of the newborn (PPHN)

Answer 216

male gender C-section large for gestational age babies asthma

Question 217

Persistent pulmonary hypertension of the newborn (PPHN) presentation

Answer 217

``` tachypnoea respiratory distress (nasal flaring/grunting/subcostal/intercostal recession, crackles) loud S2 right ventricular heave cyanosis ```

Question 218

Persistent pulmonary hypertension of the newborn (PPHN) investigations

Answer 218

echocardiogram = definitive diagnostic test (↑ pulmonary artery pressure, tricuspid regard, altered RV size/function)

Question 219

Retinopathy of prematurity (ROP)

Answer 219

proliferative disorder of immature retinal vasculature, due to vasculature of retina only reaching periphery at age of 1 month and being susceptible to oxidative damage most common cause of preventable childhood visual impairment

Question 220

Risk factors for Retinopathy of prematurity (ROP)

Answer 220

prematurity (especially , <32 weeks) low birth weight (especially ≤1250g) O2 therapy respiratory distress

Question 221

Management of Retinopathy of prematurity (ROP)

Answer 221

laser photocoagulation (treatment of choice)

Question 222

screening for Retinopathy of prematurity (ROP)

Answer 222

all infants born <32 weeks/ <1501g get weekly/fortnightly screening

Question 223

hypoxic ischaemic encephalopathy (HIE)

Answer 223

the neurological sequelae of perinatal asphyxia

Question 224

Diagnostic criteria of hypoxic ischaemic encephalopathy (HIE)

Answer 224

Metabolic acidosis with pH <7 Base deficit -12 APGAR score = 5 at 10 min & continued need for resuscitation presence of multi organ failure evidence of encephalopathy (hypotonia, abnormal oculomotor/pupillary movements, weak/absent suck reflex, seizures)

Question 225

hypoxic ischaemic encephalopathy (HIE) causes

Answer 225

maternal haemodynamic compromise uterine conditions e.g. uterine rupture placental/umbilical cord conditions e.g. abruption infection

Question 226

Management of hypoxic ischaemic encephalopathy (HIE)

Answer 226

therapeutic cooling (to minimise secondary brain injury), within 6 h of birth

Question 227

Periventricular/intraventricular haemorrhage

Answer 227

haemorrhage related to perinatal stress affecting the highly vascularised subependymal germinal matrix commonest intracranial haemorrhage in newborns

Question 228

Risk factors for periventricular/intraventricular haemorrhage

Answer 228

``` prematurity lowe birth weight IRDS hypoxia sepsis ```

Question 229

Presentation of Periventricular/intraventricular haemorrhage

Answer 229

``` diminished/absent mono reflex hypotonia lethargy apnoea poor/absent suck shrill cry convulsions bulging/tense fontanelle posturing ↓GCS ```

Question 230

Periventricular/intraventricular haemorrhage investigations

Answer 230

transfontanelle USS = investigation of choice

Question 231

Periventricular/intraventricular haemorrhage management

Answer 231

mainly supportive

Question 232

Neonatal hypoglycaemia

Answer 232

hypoglycaemia is commonly seen in first 24h post birth

Question 233

Neonatal hypoglycaemia threshold

Answer 233

<2.6mmol/L | severe <1.4mmol/L

Question 234

causes of persistent/severe neonatal hypoglycaemia

Answer 234

``` preterm large for gestational age hypothermia neonatal sepsis gestational diabetes ```

Question 235

neonatal hypoglycaemia presentation

Answer 235

``` often asymptomatic jittery seizures tachypnoea weak cry drowsy hypotonia poor feeding ```

Question 236

Management of neonatal hypoglycaemia

Answer 236

If asymptomatic: encourage feeding & monitor blood glucose If symptomatic: buccal glucagon IV glucose (10%) generally slow infusion but may require bolus if LOC/seizures

Question 237

define neonatal sepsis

Answer 237

serious bacterial/viral infection in the blood of babies within 28 days of birth

Question 238

time frame of neonatal sepsis

Answer 238

``` early onset (EOS): within 72h of birth late onset (LOS): 7-28 days after birth ```

Question 239

Causative organisms for neonatal sepsis

Answer 239

Group B strep (75% of EOS) | E.coli

Question 240

Risk factors for neonatal sepsis

Answer 240

mother with previous Group B Strep infection prematurity low birth weight maternal chorioamnionitis

Question 241

Presentation of neonatal sepsis

Answer 241

``` subacute onset of respiratory distress tachycardia apnoea jaundice seizures ↓ feeding lethargy fever (often fluctuates) ```

Question 242

Investigating neonatal sepsis

Answer 242

Blood cultures CRP (↑, helps guide management & monitor progress) urine MC&S LP

Question 243

Management of neonatal sepsis

Answer 243

IV benzylpenicilin & gentamicin (usually 10 days) | guided by CRP & culture results (if both negative stop ABx after 48h)

Question 244

physiological neonatal jaundice

Answer 244

``` due to RBC breakdown & immature liver function usually presents 2-3 days post birth & resolves by day 10 very common (up to 40% of neonates) ```

Question 245

early neonatal jaundice

Answer 245

presents within 24h of birth ALWAYS PATHOLOGICAL causes include haematological disease (e.g. ABO incompatibility), congenital infection

Question 246

prolonged neonatal jaundice

Answer 246

jaundice that persists >14 days in term/ >21 days in premature infants causes: hypothyroidism, breast milk jaundice, Gi problems (e.g. biliary atresia)

Question 247

causes of conjugated hyperbilirubinaemia in the newborn

Answer 247

biliary atresia cystic fibrosis neonatal hepatitis

Question 248

Risk factors for significant neonatal jaundice

Answer 248

``` male gender gestational diabetes low birth weight jaundice in first 24h of life previous sibling requiring phototherapy preterm ```

Question 249

presentation of neonatal jaundice

Answer 249

jaundice (usually first visible in face & forehead) drowsiness neurological signs (muscle tone changes, seizures, altered cry) NB neurological signs are Red flags and must be treated to prevent kernicterus

Question 250

kernicterus

Answer 250

chronic bilirubin encephalopathy with deposition of unconjugated bilirubin in the basal ganglia and/or brain stem nuclei leads to cerebral paresis, movement disorders, intellectual impairment

Question 251

Investigating neonatal jaundice

Answer 251

``` bilirubin levels (serum bilirubin or transcutaneous bilirubinmeter) LFTs infection screen Haemolysis screen TFTs ```

Question 252

Managing neonatal jaundice

Answer 252

``` phototherapy (as per treatment threshold on bilirubin charts) exchange transfusion (as per bilirubin chart threshold) ```

Question 253

haemolytic disease of the newborn

Answer 253

due to transplacental passage of maternal antibodies causing immune haemolytic of the fetal/neonatal RBCs may be due to naturally occurring antibodies e.g. ABO or sensitising events e.g. Rhesus alloimmunisation

Question 254

presentation of haemolytic disease of the newborn

Answer 254

hydrops fetalis/polyhydramnios jaundice pallor hepatosplenomegaly

Question 255

investigating haemolytic disease of the newborn

Answer 255

indirect coombs test (+ve for antibodies) foetal blood sampling (Rh typing) FBC (↓Hb, ↑reticulocytes)

Question 256

Neural tube defects (NTDs)

Answer 256

abnormal closure of the neural plates results in NTDs

Question 257

risk factors for Neural tube defects (NTDs)

Answer 257

family history folate deficiency anti-epileptic drugs (sodium valproate, carbamazepine)

Question 258

investigating Neural tube defects (NTDs)

Answer 258

MRI = gold standard USS (antenatal screening) AFP ↑ (antenatal screening at 16-18 weeks)

Question 259

Prevention of Neural tube defects (NTDs)

Answer 259

folic acid supplementation (400 mcg/day for everyone until 12 weeks of gestation, 5mg if high risk)

Question 260

risk factors for congenital heart defects

Answer 260

``` family history consanguineous unions intrauterine infection e.g. rubella drugs/toxins e.g. lithium or alcohol lack of folic acid gestational diabetes ```

Question 261

most common congenital heart defect

Answer 261

Ventricular petal defect (VSD)

Question 262

Ventricular petal defect (VSD) presentation

Answer 262

if small = asymptomatic if larger = presents around 5-6 weeks post birth exercise intolerance feeding intolerance harsh systolic murmur loudest at sternal edge (pan systolic)

Question 263

Investigations for congenital heart defects

Answer 263

CXR ECG Echocardiogram

Question 264

Atrial septal defect (ASD) presentation

Answer 264

generally missed in children and diagnosed in adults | soft systolic ejection murmur at left sternal edge and fixed S2 splitting

Question 265

Atrioventricular septal defect (AVSD) presentation

Answer 265

``` similar to ASD &VSD dyspnoea cyanosis signs of HF in newborn pan systolic murmur ```

Question 266

Complication of Atrioventricular septal defect (AVSD)

Answer 266

life long follow up needed due to development of AV valve regurgitation and left ventricular outflow obstruction

Question 267

Patent ductus arteriosus (PDA) presentation

Answer 267

``` failure to thrive difficulty feeding poor growth continuous machinery murmur loudest in left infraclavicular area/upper left sternal edge hyperactive precordium systolic thrill at left sternal edge ```

Question 268

Patent ductus arteriosus (PDA) management in preterms

Answer 268

indomethacin used to stop prostaglandin production & causing duct to close

Question 269

General management of congenital heart defects

Answer 269

Surgical closure especially if symptomatic

Question 270

Coarctation of the aorta presentation

Answer 270

``` poor feeding lethargy tachypnoea congestive HF shock BP in upper limbs > lower limbs systolic murmur in left infraclavicular area ```

Question 271

coarctation of the aorta management in neonates

Answer 271

Prostaglandin E1 to maintain ductus arteriosus | surgery/balloon angioplasty to repair defect

Question 272

tetralogy of fallot (TOF)

Answer 272

right ventricular hypertrophy ventricular septal defect right ventricular outflow tract obstruction overriding aorta

Question 273

tetralogy of fallot (TOF) presentation

Answer 273

poor feeding breathlessnes agitation dyspnoea on exertion squatting to rest while exercising (characteristic for R→L shunt) Tet spells (intense crying, cyanosis, ↓intensity of murmur) ejection systolic murmur

Question 274

tetralogy of fallot (TOF) management

Answer 274

Prostaglandin E1 to maintain patent ductus arteriosus if unwell asap surgical repair if unwell surgical repair age 3-6 months if not unwell

Question 275

transposition of the great arteries (TGA)

Answer 275

not compatible with life if no communication between the 2 circuits e.g. VSD/ASD/PDA

Question 276

transposition of the great arteries (TGA) presentation

Answer 276

``` cyanosis soon after birth tachypnoea respiratory distress congestive HF (if large VSD) single loud S2, no murmur ```

Question 277

Truncus arteriosus

Answer 277

single outflow tract | presents as congestive HF & cyanosis

Question 278

Phimosis

Answer 278

non retractile foreskin associated with infection & scarring

Question 279

presentation of phimosis

Answer 279

``` ballooning during micturition non retractile foreskin recurrent balanophosthitis painful micturition recurrent UTI's swelling/erythema/tenderness of prepuce ```

Question 280

Management of phimosis

Answer 280

expectant management if <2yrs old | plastic surgery/circumcision if persistent >2y/o

Question 281

paraphimosis

Answer 281

foreskin retracted & left behind glans leading to vascular engorgement & oedema of the distal glans medical emergency, may need circumcision

Question 282

Balnatitis/balanophosthitis

Answer 282

inflammation of the glans or glans & prepuce usually due to poor personal hygiene in uncircumcised males

Question 283

Cleft lip/palate

Answer 283

relatively common congenital abnormality with a complex aetiology but it is associated with chromosomal/teratogenic syndromes ~30% have associated syndromes

Question 284

cleft lip/palate presentation

Answer 284

obvious gap in newborn lip (usually upper lip) gap in palate on palpation of roof of mouth difficulty feeding (especially from bottle) poor weight gain

Question 285

Cleft lip/palate mangement

Answer 285

surgical repair in a stepwise fashion lip closure at 3 months palate closure at 6-12 months

Question 286

anorectal malformations (ARMs)

Answer 286

range of conditions generally diagnosed in early neonatal period relatively common congenital abnormality

Question 287

presentation of anorectal malformations (ARMs)

Answer 287

absence/abnormal location of anus look for anal pit in males looks for 3 classic peritoneal openings in females

Question 288

anorectal malformations (ARMs) management

Answer 288

early surgical management crucial

Question 289

Cerebral palsy

Answer 289

umbrella term for non progressive disease of the brain originating during the antenatal/neonatal/early postnatal period when brain neuronal connections are still evolving

Question 290

Classifications of cerebral palsy

Answer 290

Spastic : intermittently ↑ tone, pathological reflexes Athetoid/dyskinetic: hyperkinesia (storm movements), dsytonia, chorea, athetosis ataxic: loss of orderly muscular coordination = abnormal force/rhythm/accuracy of movements mixed type: a combination of the above

Question 291

cerebral palsy presentation

Answer 291

``` ↓ APGAR score delayed developmental milestones (especially motor & speech) retention of primitive reflexes spasticity/clonus contractures toe walking muscle tone abrnomalities ```

Question 292

cerebral palsy associated conditons

Answer 292

learning disability ~50% communication difficulty ~50% epilepsy ~30%

Question 293

Management of cerebral palsy

Answer 293

``` MDT approach psychological support is key oral baclofen/diazepam for spasticity botulin toxin for focal spasticity mobility aids & orthotics ```

Question 294

chickenpox infection

Answer 294

caused by varicella zoster virus, highly infectious but generally only mild to moderate disease which is self limiting

Question 295

presentation of chickenpox

Answer 295

pyrexia itchy vesicular rash (generally starts centrally, like dew drops on rose pedals) generally crops of lesions that eventually crust over headache malaise

Question 296

chickenpox school exclusion

Answer 296

school exclusion & avoidance of pregnant woman until all lesions are crusted over

Question 297

Measles

Answer 297

highly infectious disease caused by measles virus which has been becoming more frequent due to ↓ uptake of MMR vaccine

Question 298

Measles presentation

Answer 298

prodrome of fever/irritability/conjunctivitis/coryza/cough koplik spots (white spots on oral mucosa) maculopapular rash (spreading from behind ears/head down to trunk) high fever

Question 299

Measles school exclusion

Answer 299

5 days from onset of rash

Question 300

measles complications

Answer 300

pneumonia otitis media encephalitis subacute sclerosing panencephalitis

Question 301

chickenpox complications

Answer 301

varicella pneumonia | encephalitits

Question 302

Mumps

Answer 302

acute systemic infectious disease caused by an RNA paramyxovirus which spreads via respiratory droplets usually seen in school aged children

Question 303

Mumps presentation

Answer 303

non specific symptoms e.g. mails, fever, headache, myalgia unilateral/bilateral parotitis orchitis (swollen oedematous scrotum)

Question 304

Mumps school exclusion

Answer 304

5 days of school exclusions from onset of parotitis

Question 305

Complications of mumps

Answer 305

epididymo-orchitis (sub fertility/infertility) defaness (not permanent) pancreatitis

Question 306

Rubella presentation

Answer 306

prodrome (low grade fever, headache, mild conjunctivitis) maculopapular rash (starts on face then spreads, fades after 3-5 days) cervical/suboccipital/postauricular lymphadenopathy *

Question 307

erythema infectiosum other names

Answer 307

fifth disease, slapped cheek syndrome

Question 308

cause of slapped cheek syndrome (erythema infectiosum)

Answer 308

parvovirus B19

Question 309

presentation of erythema infectiosum

Answer 309

prodrome (fever, malaise, headache, rhinitis, sore throat) bright red macular erythema of bilateral cheeks sparing the nasal bridge/perioral areas arthralgia/arthritis

Question 310

complications of parvovirus B19 infection

Answer 310

transient aplastic crisis due to ↑RBC turnover e.g. in sickle cell pts, thalassaemia etc

Question 311

Scarlet fever

Answer 311

exotoxin mediated disease secondary to bacterial function by an erythrogenic toxin producing strain of strep pyogenes

Question 312

normal age of presentation of scarlet fever

Answer 312

peak at 4y/o | usually seen between 2-6 y/o

Question 313

scarlet fever presentation

Answer 313

sudden onset fever lasting 24-48h rash (appears on second day of illness, starting on neck/chest, coarse 'sandpaper' texture with fine punctuate erythema i.e. pinhead) strawberry tongue

Question 314

Management of scarlet fever

Answer 314

Notifiable disease | 10 days of penicillin V (azithromycin if penicillin allergic)

Question 315

school exclusion for scarlet fever

Answer 315

return 24h after initiating Abx

Question 316

hand-foot-mouth disease

Answer 316

viral illness of childhood caused by cocksackie virus A16 | usually seen in children <10y/o

Question 317

presentation of hand-foot-mouth disease

Answer 317

``` prodrome (low fever, cough, abdo pain, malaise, loss of appetite) oral lesions (begin as macule that develop into vesicles and then erode) skin lesions (on palms/soles & in between fingers/toes, starts as macule progressing to vesicles) ```

Question 318

school exclusion for hand-foot-mouth disease

Answer 318

not necessary

Question 319

molluscum contagiosum presentation

Answer 319

rash (pearly papule with central umbilication, firm & smooth, usually found in clusters on trunk/extremities) NB >50 lesions/facial lesions imply immunocompromise

Question 320

Roseola infantum (sixth disease)

Answer 320

common febrile illness of childhood caused by Human herpes virus 6 (HHV 6) usually seen in children between 6 months and 2 years of age

Question 321

Presentation of roseola infantum

Answer 321

3-5 days of high fever (40°C), peaks in early evening exanthema presents after fever resolves (2-5mm pink-red macules/papules) maybe be associated with febrile seizures

Question 322

pertussis (whooping cough)

Answer 322

URTI caused by bordetella pertussis which is characterised by a severe cough NOTIFIABLE DISEASE even when suspected only on clinical grounds

Question 323

Presentation of pertussis

Answer 323

prodrome: 2-3 days of coryza dry hacking cough (usually worse at night, may cause vomiting) inspiratory whoop (caused by forced inspiration against closed glottis after coughing fit)

Question 324

Management of pertussis

Answer 324

macrolide Abx e.g. clarithromycin

Question 325

School exclusion for pertussis

Answer 325

21 days from onset of symptoms or 48h after Abx started

Question 326

croup (laryngotracheobronchitis)

Answer 326

URTI seen in infants & toddlers usually between 6 months and 3 years of age caused by parainfluenza virus

Question 327

Croup presentation

Answer 327

general non specific URTI cough (seal like/barking = characteristic) stridor respiratory distress (nasal flaring, inter/subcostal recession/tracheal tug/tachypnoea) lasts 3-7 days

Question 328

croup scoring system

Answer 328

westley score

Question 329

croup mangement

Answer 329

single dose oral dexamethasone (0.15mg/kg) avoid agitating child (i.e. do not examine throat) admit if moderate/severe croup

Question 330

Bronchiolitis

Answer 330

Lower respiratory tract infection caused by respiratory syncytial virus (RSV) generally seen in infants under the age of 2 with peak incidence between 3-6 months of age

Question 331

Risk factors for severe bronchiolitis

Answer 331

``` prematurity low birth weight age <12 weeks congenital heart disease chronic lung disease ```

Question 332

Croup presentation

Answer 332

prodrome: 1-3 day history of coryza symptoms persistent cough (seal like/barking = characteristic) stridor respiratory distress (nasal flaring, inter/subcostal recession/tracheal tug/tachypnoea) wheeze/crackles poor feeding rhinitis fever (<39°C)

Question 333

croup management

Answer 333

single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity ``` supportive therapy (O2, feeding support) self limiting, lasting 3-7days ```

Question 334

Epiglottitis

Answer 334

a cellulitis of the supra glottis with the potential to cause airway compromise caused by haemophilia influenzas type B (HiB) rare but serious infection

Question 335

epiglottitis presentation

Answer 335

``` sore throat odynophagia fever inability to swallow secretions = drooling muffled voice tripod positioning ```

Question 336

epiglottitis investigations

Answer 336

fibre-optic laryngoscopy = gold standard | lateral neck X-ray (thumb sign)

Question 337

Management of epiglottis

Answer 337

oral/IV ABx (usually IV) | may require intubation/tracheostomy

Question 338

Impetigo

Answer 338

common superficial skin infection cause by strep pyogenes or staph aureus

Question 339

Bullus impetigo presentation

Answer 339

thin roofed bullae, tend to rupture spontaneously | often painful

Question 340

Non bullous impetigo presentation

Answer 340

tiny pustules/vesicles which rapidly erode into a honey coloured crusted plaque especially on face, around the mouth & nose

Question 341

Management of impetigo

Answer 341

hydrogen peroxide 1% cream topical Abs (fusidic acid) Oral flucloxacillin if systemically unwell (erythromycin if penicillin allergy)

Question 342

impetigo school exclusion

Answer 342

until all lesions crusted over & healed | or 48h after commencing ABx

Question 343

Laryngomalacia

Answer 343

congenital abnormality of the larynx that predisposes to supraglottic collapse during the inspiratory phase commonest cause of stridor in infants often associated with GORD

Question 344

Mesenteric adenitis

Answer 344

inflammation of mesenteric lymph nodes after viral illness | presents as fever, RLQ pain, nausea, vomiting

Question 345

Kallman's syndrome

Answer 345

delayed puberty secondary to hypogonadotrophic hypogonadism x-linked recessive trait presents with anosmia, delayed puberty, cryptorchidism ↓ sex hormone levels, ↓/normal FSH/LH

Question 346

Kawasaki disease

Answer 346

idiopathic self limiting vasculitis usually seen in children age 6 months to 5 years more common in the asian population

Question 347

Kawasaki disease presentation

Answer 347

``` Fever (≥39°C for ≥5days) conjunctival injections polymorphous rash erythema/oedema of palms/soles ±desquamation cervical lympadenopathy bright red cracked lips strawberry tongue ```

Question 348

Kawasaki disease diagnosis

Answer 348

clinical | no diagnostic tests available

Question 349

Management of kawasaki disease

Answer 349

``` Aspirin (high dose) IV immunoglobulin (IVIG) ```

Question 350

complications of kawasaki

Answer 350

coronary artery aneurysm (screen with echo)

Question 351

Henoch-Schönlein Purpura (HSP)

Answer 351

IgA mediated mediated autoimmune hypersensitivity vasculitis of unknown aetiology usually seen between the ages of 4-6 years most common vasculitis of childhood

Question 352

Henoch-Schönlein Purpura (HSP) presentation

Answer 352

``` often preceded by URTI or GI infection arthralgia ± oedema abdo pain ± nausea & vomiting palpable, non blanching purpuric rash over buttocks & extensors surfaces of arms and legs renal disease ```

Question 353

Management of Henoch-Schönlein Purpura (HSP)

Answer 353

NSAIDs for joint pain consider corticosteroids if nephrotic range proteinuria monitor renal function (BP & urinalysis at 6 & 12 months)

Question 354

Febrile seizures

Answer 354

a seizure occurring in a febrile child between the ages of 6 months and 5 years without other underlying causes

Question 355

Peak incidence of febrile seizures

Answer 355

18 months of age

Question 356

simple febrile seizure

Answer 356

generalised tonic clonic seizure lasting <15min and do not recur within 24h/the same febrile illness

Question 357

complex febrile seizure

Answer 357

≥1 of the following: - focal seizure/focal features - duration between 15-30min - recurrence within 24h/same febrile illness

Question 358

febrile status epilepticus

Answer 358

seizure >30 min duration

Question 359

Trigger for febrile seizures

Answer 359

rapidly rising temp (usually early in illness)

Question 360

presentation of febrile seizures

Answer 360

normal post ictal examination generally tonic clonic seizures lasting <5min quick recovery of consciousness no sequelae

Question 361

Referring a febrile seizure pt

Answer 361

``` if 1st febrile seizure unable to exclude serious illness complex seizure <18 months old status epilepticus ```

Question 362

Management of febrile seizures

Answer 362

mainly parental education e.g. call 999 if seizure >5min give PR diazepam/buccal midazolam if >5min & repeat at 10 min consider IV phenytoin if still continuing

Question 363

Complications of febrile seizures

Answer 363

reoccurrence in 1/3 pts | ↑ risk of developing epilepsy

Question 364

otitis media

Answer 364

infection of the middle ear space occurring often in childhood

Question 365

presentation of otitis media

Answer 365

``` otalgia (tugging at ear in younger children) irritability sleep disturbance fever malaise ```

Question 366

Investigations for otitis media

Answer 366

otoscaopy (bulging tympanic membrane, red/yellow/cloudy tympanic membrane, air fluid level behind tympanic membrane, discharge in auditory canal if tympanic membrane perforation)

Question 367

Management of otitis media

Answer 367

admit if <3 months & temp > 38°C / acute complication e.g. meningitis suspected antipyretics & analgesia Abx ( amoxicillin 1 st line, or erythromycin if penicillin allergy)

Question 368

when to give ABx in otitis media

Answer 368

if ≥ 4 days duration & no improvement, systemically unwell, immunocompromised, perforation & discharge, <2y/o with bilateral AOM

Question 369

Meningococcal disease causative organism

Answer 369

Neisseria meningitidis (gram -ve, commensal in nasopharynx)

Question 370

Meningococcal disease

Answer 370

leading cause of infective deaths in early childhood mostly seen in <5 y/o peak age is in under 1 y/o

Question 371

Presentation of Meningococcal disease

Answer 371

``` rapid onset of illness (febrile illness >24h unlikely to be meningococcal disease) fever, irritability, leg pain, headache stiff neck photophobia back rigidity bulging fontanelle seizures altered mental status ↓ level of consciousness non blanching purpuric rash hypotonia ```

Question 372

Investigating Meningococcal disease

Answer 372

clinical suspicion Blood cultures lumbar puncture

Question 373

Management of Meningococcal disease

Answer 373

< 3 months = cefotaxime/ceftriaxone +ampicillin/amoxcillin (to cover listeria) >3 months = cefotaxime/ceftriaxone anticoagulation for DIC

Question 374

complications of Meningococcal disease

Answer 374

sensorineural hearing loss Disseminated intravascular coagulation ↑ ICP Waterhouse- Friedrich syndrome

Question 375

testicular torsion presentation

Answer 375

``` sudden onset intense scrotal pain pain associated with nausea & vomiting pain preceded by sport/physical activity absent cremaster reflex scrotal swelling/oedema/erythema ```

Question 376

testicular torsion investigations

Answer 376

DO NOT delay surgical exploration if clinical suspicion is high

Question 377

Management of testicular torsion

Answer 377

``` scrotal exploration (emergency surgery) bilateral orchioplexy ```

Question 378

Appendicitis in children <4 y/o

Answer 378

uncommon but often presents as perforation if it does occur

Question 379

Appendicitis presentation

Answer 379

abdo pain ( typically starting centrally then moving to RLQ/RIF, constant pain with intermittent cramps which is aggravated by movement) anorexia nausea & vomiting Rovsing's sign positive (palpation of LLQ = ↑ pain in RLQ as inflamed peritoneum is stretched)

Question 380

Investigations for appendicitis

Answer 380

abdo USS/ CT abdomen FBC CRP (↑)

Question 381

Managing appendicitis

Answer 381

appendectomy (laparoscopically)

Question 382

Risk factors for paediatric asthma

Answer 382

``` atopy family history allergies passive smoking prematurity low birthweight ```

Question 383

presentation of paediatric asthma

Answer 383

wheezing episodes/SOB/chest tightness/cough (symptoms often worse at night/early morning)

Question 384

Investigating paediatric asthma

Answer 384

spirometry (↓FEV1/FVC ratio) response to bronchodilators on spirometry peak expiratory flow (↓) CXR (normal)

Question 385

managing paediatric asthma step 1

Answer 385

Inhaled short acting beta agonist (SABA) e.g. salbutamol

Question 386

managing paediatric asthma step 2

Answer 386

SABA + low dose inhaled corticosteroids

Question 387

managing paediatric asthma step 3 | NB Step 2 = SABA + low dose inhaled corticosteroids

Answer 387

add leukotriene receptor antagonist (LTRA)

Question 388

managing paediatric asthma step 4 | NB Step 3 = Trial of LTRA

Answer 388

stop LTRA if ineffective and add LABA

Question 389

Acute asthma in children management

Answer 389

Salbutamol via inhaler, or nebuliser with O2 if hypoxic ipratropium bromide oral/IV corticosteroids nebuliser/IV magnesium sulphate

Question 390

sign of life threatening asthma

Answer 390

``` SpO2 <92%, PEF < 33% silent chest cyanosis poor respiratory effort confusion agitation LOC ```

Question 391

sign of severe asthma

Answer 391

``` SpO2 <92% HR >125 if >5y/o or >140 if 2-5y/o RR >30 if >5y/o or >40 if 2-5y/o use of accessory muscles inability to complete full sentences ```

Question 392

causative organism for septic arthritis in children

Answer 392

staph aureus

Question 393

Most commonly affected joint for septic arthritis in children

Answer 393

hip, knee, ankle

Question 394

presentation of septic arthritis in children

Answer 394

easy to miss as localised signs may be absent hot/swollen/painful joint restricted movement of affected joint unwillingness to weight bear/move affected joint fever

Question 395

investigating septic arthritis in children

Answer 395

joint aspirate for culture/MC&S (↑WCC) FBC/CRP/blood cultures/U&Es/LFTs USS/X-ray of joint

Question 396

managing septic arthritis in children

Answer 396

joint drainage & Abx (according to local guidelines but often flucloxacillin)

Question 397

Kocher diagnostic criteria for septic arthritis in children

Answer 397

fever >38.5 °C non weight bearing ↑WCC ↑ESR/CRP

Question 398

Slipped upper femoral epiphysis (SUFE)/slipped capital femoral epiphysis (SCFE)

Answer 398

due to weakness in proximal femoral growth plate allowing displacement of the capital femoral epiphysis most common hip disorder in adolescent age group

Question 399

SUFE epidemiology

Answer 399

more common in boys | peaks around age 13 for boys and age 11.5 for girls

Question 400

SUFE risk factors

Answer 400

OBESITY** endocrine disorders (e.g. hypothyroidism) puberty male gender

Question 401

SUFE presentation

Answer 401

``` hip/groin discomfort/pain limp abnormal gait leg externally rotated loss of internal rotation of leg ↓ Range of movement may have knee pain ```

Question 402

SUFE investigations

Answer 402

bilateral AP X-ray (kleins line does not intersect femoral head) lateral frog leg X-ray ( +ve Bloombergs sign)

Question 403

Management of SUFE

Answer 403

immobilise/non weight bearing provide analgesia surgical referral single screw internal fixation

Question 404

Classical presentation of SUFE

Answer 404

overweight/obese teenage boy

Question 405

transient synovitis

Answer 405

self limiting inflammatory disorder common in young children often triggered by a viral infection e.g. URTI Peak incidence age 5-6 yrs

Question 406

Presentation of transient synovitis

Answer 406

``` discomfort/pain (less severe than septic arthritis, often still able to weight bear) limp abnormal gait ↓ Range of movement positive log roll systemically well ```

Question 407

managing transient synovitis

Answer 407

analgesia rest usually self limiting

Question 408

Perthes disease

Answer 408

self limiting disease of the femoral head with necrosis, collapse, repair and remodelling , essentially avascular necrosis of the nucleus of the proximal femoral epiphysis

Question 409

Epidemiology of perthes disease

Answer 409

typically unilateral 5:1 male:female ratio usually seen in boys aged 4-8 yrs

Question 410

presentation of perthes disease

Answer 410

``` hip pain progressively worsening over several weeks limp stiffness ↓ Range of movement no history of trauma ```

Question 411

investigating perthes disease

Answer 411

bilateral hip X-ray (femoral collapse & fragmentation) lateral frog leg X-ray (widening of joint space) both X-rays show (flattening of femoral head, and ↓ femoral head size)

Question 412

managing perthes disease

Answer 412

physiotherapy | surgery if >6y/o

Question 413

Developmental dysplasia of the hip

Answer 413

spectrum of mild dysplasia with stable hip to established hip dysplasia with subluxation and instability affects 1-3% of newborns

Question 414

Developmental dysplasia of the hip risk factors

Answer 414

``` female gender (6:1 female:male ratio) breech presentation family history oligohydramnios macrosomia ```

Question 415

Developmental dysplasia of the hip presentation

Answer 415

often on NIPE examination/6 week baby check positive ortolans's and Barlow's test (i.e. able to dislocated & relocate the hip joint) limited hip abduction

Question 416

Screening for developmental dysplasia of the hip

Answer 416

USS screening for all pts with the following - first degree relative with history of hip problems in early life - breech presentation at/after 36 weeks gestation - multiple pregnancy All babies are screened view Barlow/Ortolani test at NIPE/6week check

Question 417

Investigating developmental dysplasia of the hip

Answer 417

USS of hips | Hip X-ray if >6months of age

Question 418

developmental dysplasia of the hip management

Answer 418

stabilisation with Pavlik harness if <5months | if failed stabilisation/>6 months old =surgery

Question 419

juvenile idiopathic arthritis (JIA)

Answer 419

collection of chronic paediatric arthropathies characterised by onset <16 yrs and presence of objective arthritis for >6 weeks

Question 420

Presentation of juvenile idiopathic arthritis (JIA)

Answer 420

``` > 6 week duration joint pain erythema joint swelling fever morning stiffness ↓ ROM pink salmon coloured rash ```

Question 421

managing juvenile idiopathic arthritis (JIA)

Answer 421

NSAIDs steroids methotrexate (1st line if >1 joint affected)

Question 422

Osgood-Schlatter disease

Answer 422

overuse syndrome of the paediatric population typically affecting young athletes during adolescent growth spurts more common in boys one of the most common causes of knee pin in active adolescents

Question 423

Osgood-Schlatter disease presentation

Answer 423

gradual pain/swelling below knee pain worse on activity/better at rest pain over tibial tuberosity

Question 424

Toddlers diarrhoea

Answer 424

commonest cause fo chronic diarrhoea in young children | characterised by undigested food in stool & systemically well child

Question 425

Cow's milk protein intolerance

Answer 425

one of the commonest allergic disorders in young children, usually seen in 1st year of life (especially formula fed infants

Question 426

Cow's milk protein intolerance presentation

Answer 426

``` failure to thrive ↓ growth velocity skin reaction (urticaria/angioedma) Nausea & vomiting diarrhoea abdo pain ```

Question 427

gastroenteritis in children

Answer 427

frequently rotavirus | very common in <5y/o

Question 428

Managing gastroenteritis in children

Answer 428

encourage fluid intake & continue feeding offer ORS if ↑ risk of dehydration IV fluids if dehydrated

Question 429

IV fluids for dehydrated children

Answer 429

if shocked 100ml/kg | if not shocked 50ml/kg

Question 430

coeliac disease

Answer 430

systemic autoimmune disease triggered by dietary gluten peptides HLA DQ2 & HLA DQ8 associated

Question 431

coeliac disease presentation

Answer 431

``` failure to thrive diarrhoea abdo distension faltering growth persistent mouth ulcers ```

Question 432

investigating coeliacs disease

Answer 432

total IgA IgA-tTG (tissue transglutaminase) endomysial antibody small bowel biopsy

Question 433

GORD in children

Answer 433

very common especially in <1y/o due to immature sphincters NB smalll amounts of asymptomatic effortless regurgitation after feeding (possetting is normal)

Question 434

Presentation of GORD in infants

Answer 434

``` stressful meal times long feeds inappropriate volume of feeds regurgitation vomiting irritability at meal times ```

Question 435

managing GORD in infants

Answer 435

feeding position (30°, head up) review feeding volume trial of gaviscon

Question 436

constipation in children presentation

Answer 436

``` difficult/painful defectation straining long interval between stools faecal incontinence anal fissure ```

Question 437

Red flags for constipation

Answer 437

``` symptoms from birth/in first weeks of life overflow soiling >48h to pass meconium ribbon stools leg weakness abdo distension + vomiting abnormal appearance of anus abnormal reflexes ```

Question 438

Managing constipation in children

Answer 438

1st line: paediatric movicol 2nd line: add stimulant laxative if no dissimpaction after 2 weeks e.g. senna or decussate 3rd line: if still not working substitute stimulant laxative for lactulose

Question 439

Reflex anoxic seizures

Answer 439

syncopal episodes secondary to emotional/painful stimuli usually seen in children aged 6 months - 2yrs 30-60 sec on tonic jerking of limbs but no post ictal phase no treatment needed

Question 440

Breath holding spells

Answer 440

vigorous crying followed by blocked respiration resulting in cyanosis, LOC, opisthotonic posturing

Question 441

Infantile spasms (west syndrome)

Answer 441

seen in babies aged 4-8months Salaam attacks of head bobbing/torso flexion/arm flexion usually spasm occur in clusters (ie repeated 50x), generally symmetrical & bilateral CT (diffuse/localised brain disease) & EEG Treatment = vigabatrin poor prognosis

Question 442

absence seizures in childhood

Answer 442

onset usually age 3-10yrs presenting with behavioural arrest/starring attacks lasting 5-10 secs, with no aura or post-octal phase Management : 1st line = sodium valproate/ethosuximide/lamotrigine

Question 443

Tonic clonic seizures in childhood

Answer 443

contraction of limbs followed by extension & arching of back lasting 10-30 seconds cyanosis & apnoea post ictal phase up to 30 min tongue biting common Management: lamotrigine or levetiracetam

Question 444

Benign rolandic epilepsy/benign focal epilepsy

Answer 444

children aged 4-10yrs, more common in boys nocturnal seizures especially on waking characterised by facial twitching & aphasia most children outgrow these & no treatment required

Question 445

Status epilepticus in children

Answer 445

prolonged seizure >30min or recurrent seizures without full return to baseline between seizures

Question 446

managing status epilepticus in children

Answer 446

at 5 min = Buccal midazolam/rectal diazepam (0.5mg/kg) repeat at 5-10 min after first dose at ~25min = phenytoin IV (20mg/kg) over 20 min if not terminated = RSI & intubation

Question 447

causative organisms for paediatric sepsis

Answer 447

neonates <72h after birth = group B strep neonates 72h-28 days after birth = coagulase -ve staph infants/young children = staph pneumoniae, neisseria meningitidis

Question 448

Abx for paediatric sepsis

Answer 448

cefotaxime/ceftriaxone + gentamicin is commonly used

Question 449

contraindications for Lumbar puncture

Answer 449

``` coma suspected ↑ ICP cardiovascular/respiratory compromise suspected cerebral herniation coagulopathy/thrombocytopenia local infection ```

Question 450

Septic screen in children

Answer 450

``` Blood cultures urine MC&S Lumbar puncture ABG CXR/AXR FBC/CRP/U&Es/LFTs/Coagulation ```

Question 451

definition of neutropenic sepsis

Answer 451

neutrophil count <500 cells/microlitre (<0.5x10^9) in a patient having anticancer therapy + temp >38.5°C or other clinical features of sepsis

Question 452

Abx management of neutropenic sepsis

Answer 452

Tazocin (piperacillin + tazobactam)

Question 453

Pneumonia in children causative organism

Answer 453

strep pneumonia = most common causative organism

Question 454

first line Abx for penumonia in children

Answer 454

amoxicillin

Question 455

UTI in children causative organisms

Answer 455

E. coli most common

Question 456

UTI presentation in children

Answer 456

``` Fever (>39°C) lethargy poor feeding frequency dysuria abdo pain ```

Question 457

UTI investigation for children

Answer 457

clean catch urine for urinalysis (+ve nitrites, +ve leukocytes) & MC&S (↑WBC, diagnostic culture)

Question 458

management of UTI in children

Answer 458

<3 months old = refer to peads >3 months = cephalosporin/co-amoxiclav for 7-10 days

Question 459

recurrent UTIs in children

Answer 459

should raise question of child abuse

Question 460

Continence in children

Answer 460

majority of children achieve day & night time continence at age 3-4

Question 461

enuresis

Answer 461

involuntary discharge of urine day/night/both in a child ≥5 y/o in absence of congenital/acquired defects of the nervous system of the urinary tract

Question 462

primary vs secondary enuresis

Answer 462

primary = never previously acquired continence secondary = in a child who has previously been dry for >6months

Question 463

Management of enuresis

Answer 463

1st line = advice on fluid intake & toileting behaviour 2nd line= reward system 3rd line = enuresis alarm 4th line = desmopressin (particularly in 7y/o for short term control e..g a sleep over/school trip)

Question 464

Haemophilia

Answer 464

X-linked recessive bleeding disorder Haemophilia A = factor VII deficiency (more common) Haemophilia B = factor IX deficiency present with recurrent/severe bleeding, easy bruising, pronged bleeding episodes, recurrent nose bleeds Investigations: aPTT (↑), plasma factor VIII/IX (↓/absent), PT (normal) ``` Management: Type A (prophylactic factor VIII) Type B (recombinant factor IX) ```

Question 465

Neuroblastoma

Answer 465

solid tumour arising from the developing sympathetic nervous system majority of pts diagnosed by age 5 location usually adrenal/paraspinal sites presents with abdo distension, abdo mass, fatigue, bone pain, constipation, hepatomegaly, weakness paralysis ``` FBC ( pancytopenia), USS abdo (shows mass), CT/MRI urinary catecholamines ( VMA/HVA ↑) ```

Question 466

Wilms tumour/nephroblastoma

Answer 466

most common renal malignancy of childhood presenting between age 2-5 presents as asymptomatic abdominal mass, flank pain, UTIs, haematuria, pallor Urinalysis (↑RBC, ↑protein), abdo USS, CT/MRI management: nephrectomy followed by chemo

Question 467

Osteosarcoma

Answer 467

most common primary bone malignancy in children, usually seen age 13-16 presents with worsening bone pain over weeks/months, which is worse at night, firm mass/swelling, limping 75% in knee/proximal humerus X-ray (sunburst appearance of calcified soft tissue, conman triangle of subperiosteal bone), ALP (↑), LDH (↑) management: surgery + chemo

Question 468

Ewings sarcoma

Answer 468

rare cancer, usually occurs in teenagers presents as mass/swelling in long bones, pelvis or skull with pain, erythema, weight loss, malaise X-ray (bone destruction and overlying onion skin layers of periosteal bone), biopsy Management: chemo (VIDE)

Question 469

brain tumours in children

Answer 469

brain = most common site of solid tumours in children, mainly infratentorial, astorcytomas & medulloblastomas are most common presents with headache, vomiting, gait abnormalities, co-ordination problems, papiloedma Headache (worse in morning, frequent, gradually working) MRI/CT head Management: surgery/chemo/radiotherapy

Question 470

Retinoblastoma

Answer 470

embryonal tumour of the retina usually seen in <5y/o with a peak at 18 months of age presents with leukocoria (absent red reflex), strabismus, visual problems, pseudo-orbital cellulitis, ocular pain (indicates advanced disease) fundoscopy and examination under GA, MRI Management: enucleation & implant

Question 471

Leukaemia in childhood

Answer 471

most common diagnosed cancer in children, with peak incidence between 2-3 yrs of age Acute lymphoblastic leukaemia (ALL) = 80% of childhood leukaemia Acute myeloid leukaemia (AML) = 15% of childhood leukaemia, most common in infants presents with anaemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy often vague non specific symptoms FBC/blood film (pancytopenia, ↑blast cells) bone marrow aspirate/biopsy CT/MRI management: chemotherapy allogenic bone marrow transplant

Question 472

Prognosis of childhood leukaemia

Answer 472

leading cause of death in childhood | ALL better prognosis than AML

Question 473

Childhood lymphoma

Answer 473

lymphoproliferative disorder, non-hodgkins type is more common in children presents with painless, progressive lymphadenopathy (rubbery, asymmetrical), fatigue, fever, malaise, night sweats, ↓weight FBC (thrombocytopenia, neutropenia), excisions node biopsy (diagnostic) CT/MRI chest/abdo/pelvis Management: watchful waiting, chemotherapy, radiotherapy

Question 474

ADHD

Answer 474

a disorder of inattention, hyperactivity, impulsivity which affects ~5% of children usually diagnosed at age 3-7 more common in boys

Question 475

Diagnostic criteria for ADHD

Answer 475

≥ 6 of the following symptoms which have persisted for ≥6months to a degree that is maladaptive & inconsistent with developmental level symptoms present in ≥2 settings Inattention: Poor attention to details at work/school, Difficulty sustaining attention during tasks, Does not listen when spoken to directly, Inability to complete tasks/instructions at work/school, Struggles to organize tasks/activitie, Prefers to avoid tasks that require a high amount of mental effort, Loses things necessary to complete tasks, Easily distracted, Forgetful Hyperactivity & impulsivity: Fidgets or squirms in seat, Often leaves their seat during inappropriate situations, Restless, Unable to carry out tasks quietly, Talks excessively, Answers questions prematurely or for others, Difficulty waiting their turn, Interrupts other people

Question 476

management of ADHD in children

Answer 476

CAMHS referral education & advice regular exercise & healthy diet pharmacological Methylphenidate (monitor heigh & weight 6 monthly) lisdexamfetamine or atomoxetine (2nd line)

Question 477

Monitoring children on pharmacological therapy for ADHD

Answer 477

Monitor height & weight | monitor BP & ECG (drugs potentially cardiotoxic)

Question 478

Autism spectrum disorder (ASD)

Answer 478

a disorder characterised by persistent impairments of social communication, restricted, repetitive and stereotyped patterns of behaviours/interests/activities more common in males

Question 479

Presentations of autism spectrum disorder (ASD)

Answer 479

impaired social communication & interaction language delay/regression verbal & non-verbal communication impairment repetitive, rigid, stereotyped interests/behaviours/activities motor stereotypes (e.g. rocking, spinning) feeding difficulties sensory interests (e.g. examining objects closely)

Question 480

Investigations for autism spectrum disorder (ASD)

Answer 480

ASD screenign tools

Question 481

Management of autism spectrum disorder (ASD)

Answer 481

early educational & behavioural interventions SALT occupational therapy family support and counselling

Question 482

Rickets

Answer 482

changes caused by deficient mineralisation of the growth plates of long bones presents with slow growth rate, bone pain, bony deformities (bow legs, knock knees), late dentition management: Calcium & Vit D supplementation, phosphate salts

Question 483

Assessing puberty stages

Answer 483

Tanner stages are used in boys and girls

Question 484

Normal onset of puberty

Answer 484

Males: 12-13 y/o Females: usually round 11y/o

Question 485

First sign of puberty in boys

Answer 485

testicular enlargement

Question 486

First sign of puberty in girls

Answer 486

breast development

Question 487

Delayed puberty in boys

Answer 487

absence of testicular development i.e. testicular volume <4ml at age 14

Question 488

Delayed puberty in females

Answer 488

absence of breast development by age 13 or primary amenorrhoea with normal breast development by age 15

Question 489

average age of menarche

Answer 489

13 years

Question 490

Causes for delayed puberty

Answer 490

central Constitutional delay of growth and puberty (CDGP), chronic disease, malnutrition, excessive physical exercise, hypothyroidism Peripheral Boys: testicular damage, Prader-willi/Noonas/Klinefelters Girls: Turner, PCOS

Question 491

Precocious puberty in boys

Answer 491

in boys before age 9

Question 492

Precocious puberty in girls

Answer 492

in girls before age 8 | NB 5-10x more common in women)

Question 493

Types of Precocious puberty

Answer 493

gonadotrophin dependent (central precocious puberty) most common, premature HPG axis activation ↑FSH/LH e.g. CNS tumours gonadotrophin independent (precocious pseudo puberty) les common, production of sex independent of HPG axis ↓FSH/LH ↑ testosterone/estradiol e.g. congenital adrenal hyperplasia

Question 494

Investigating precocious puberty

Answer 494

FSH/LH levels testosterone oestrogen USS pelvis

Question 495

paediatric fluid resuscitation

Answer 495

20ml/kg NaCl 0.9% over 10 minutes | if ≥40-60ml/kg required call PICU

Question 496

Paediatric maintenance fluids

Answer 496

100ml/kg/24h for first 10kg 50ml/kg/24h for second 10 kg (11-20kg) 20ml/kg/24h for every kg >20kg to calculate rate rate (ml/h) = total daily requirements/24

Question 497

Dehydration fluids in children

Answer 497

50ml/kg (~5%) deficit add 50ml/kg/24h as fluid replacement on top of maintenance 100ml/kg (~10%) deficit add 100ml/kg/24h as fluid replacement on top of maintenance

Question 498

Red flags for developmental milestones

Answer 498

developmental regression/loss of previously acquired skills/milestones parental concerns hand preference in infant <1y/o marked hyper/hypotonia lack of response to sound/visual stimuli poor interaction with parent/other children

Question 499

Red flags for headaches

Answer 499

``` headache waking them up at night/present at waking worse on coughing/bending over persistent vomiting fever/neck stiffness/photophobia confusion altered level of consciousness change in gait/balance seizures focal neurology school absences due to headache ```

Question 500

Non accidental injuries red flags

Answer 500

no history of trauma unwitnessed trauma injury incompatible with developmental stage delayed presentation changing history fractures in child <1y/o multiple fractures presence of other injuries fractures of different ages injuries to sites not usually exposed to trauma lack of concordance between given MOI and injury

Question 501

Fever red flags

Answer 501

``` ≥38°C in <3 months old ≥39°C in 3-6 month old non blanching rash/mottled skin child appears unwell to HCP ↓ fluid intake/ ↓ urine output does not wake when roused/does not stay awake signs of dehydration bulging fontanelle seizures persistent fever >5days ```

Question 502

Features of innocent murmurs

Answer 502

systolic musical/vibratory quality does not radiate Vary with respiration & positioning

Question 503

Migraine in children

Answer 503

very common (~10%), most common cause of primary headache in children

Question 504

Presentation of migraine in children

Answer 504

headache lasting 4-72h usually unilateral, usually frontal/temporal with a pulsating/throbbing character may be associated with nausea & vomiting, photophobia or photophobia

Question 505

Managing migraine in children

Answer 505

NSAIDs (ibuprofen) | NB Ibuprofen is more effective than paracetamol

Question 506

Tension headache in children

Answer 506

2nd most common cause of headache in children, can be episodic or chronic

Question 507

Triggers for tension headaches

Answer 507

stress (physiological, social, emotional) disturbed sleep pattern mental tension

Question 508

Presentation of tension headaches

Answer 508

generalised bilateral headache, usually less severe than migraine, pressure like non-throbbing pain, often occipital/frontal described as tight band around head

Question 509

Managing tension headaches in children

Answer 509

advice on triggers simple analgesia TCAs (for recurrent/chronic TTH)

Question 510

Fetal alcohol syndrome presentation

Answer 510

failure of growth (↓ head circumference, ↓ weight, ↓ length), irreversible (i.e. stunted for life) craniofacial abnormalities (microcephaly, flat philtre, micro/retrognathia, microphtalmia, cleft lip/palate) presence of congenital abnormalities (e.g. congenital heart defects) neurodevelopment abnormalities ( e..g developmental delay, learning difficulties, mental health problems, behavioural problems)

Question 511

Diamond blackfan syndrome

Answer 511

congenital hypoplastic anaemia that usually presents in infancy presents with severe/life threatening in first few months of life as severe hypo plastic microcytic anaemia treated with ciclosporin A + prednisolone

Question 512

Caput succedaneum

Answer 512

poorly defined subcutaneous collection of serosanguinous fluid,soft puffy swelling, spreads over suture lines and midline soft puffy swelling due to prolonged labour or use of venous delivery

Question 513

Cephalohaematoma

Answer 513

subperiosteal haematoma that develops shortly after birth, usually in parietal region, does not spread across suture lines may cause jaundice as haematoma resolves

Question 514

Erbs Palsy

Answer 514

injury to upper trunk of the brachial plexus (C5/C6) causes loss of motion of shoulder with limp arm that is adducted & internally rotated absent bicep reflex, intact grasp reflex due to excessive lateral traction on neck during labour

Question 515

Klumpke's palsy

Answer 515

injury to lower trunk of brachial plexus (C7/C8/T1) causes paralysis & weakness of hand, loss of grasp reflex, may cause Horner's due to excessive traction on arm during delivery

Question 516

Juvenile idiopathic arthritis (JIA)

Answer 516

arthritis occurring in someone who is less than 16 years old that lasts for more than 6 weeks. Systemic onset JIA is a type of JIA which is also known as Still's disease presentation includes pyrexia, salmon-pink rash, lymphadenopathy, arthritis, uveitis, anorexia and weight loss ANA may be positive (especially in oligoarticular JIA) rheumatoid factor is usually negative NBP pauciarticular JIA refers to cases where 4 or less joints are affected. It accounts for around 60% of cases of JIA