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Phase 3a > Paeds > Flashcards

Paeds Flashcards

1
Q

what are the 3 fetal shunts

A

ductus venosus
foramen ovale
ductus arteriosus

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2
Q

what does the ductus venosus connect

A

umbilical vein to inferior vena cava so blood can bypass the liver

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3
Q

what does the foramen ovale connect

A

right and left atrium so blood can bypass the right ventricle and pulmonary circulation

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4
Q

what does the ductus arteriosus connect

A

pulmonary artery with aorta so blood can bypass the pulmonary circulation

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5
Q

how does the foramen ovale shut

A

1st breath expands alveoli, decreases pulmonary vascular resistance, decreases right atrium pressure so left atrium pressure is high, squashing atrium septum - seals after few weeks to become the fossa ovalis

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6
Q

how does the ductus arteriosus close

A

increased blood oxygenation drops amount of circulating prostaglandins - becomes ligaments arteriosum

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7
Q

how does the ductus venosus close

A

immediately stops function because umbilical cord is clamped - becomes ligamentum venosum

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8
Q

what are innocent/flow murmur

A

common in children
fast blood flow through areas of the heart during systole

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9
Q

features of an innocent murmur

A

soft
short
systolic
symptomless
situation dependent - quieter when standing, appears when unwell etc

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10
Q

prompts for investigation of a murmur

A

louder than 2/6
diastolic
louder on standing
other symptoms e.g. failure to thrive

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11
Q

where would you hear mitral regurgitation murmur

A

5th intercostal space, midclavicular line

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12
Q

where would you hear a tricuspid regurgitation murmur

A

5th intercostal space, left sternal border

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13
Q

where would you hear a ventricular septal defect murmur

A

left lower sternal border

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14
Q

where would you hear an aortic stenosis murmur

A

2nd intercostal space, right sternal border

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15
Q

where would you hear a pulmonary stenosis murmur

A

2nd intercostal space, left sternal border

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16
Q

where do you hear a hypertrophic obstructive cardiomyopathy murmur

A

4th intercostal space on left sternal border

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17
Q

what type murmur do you hear in mitral reurgitation

A

pan systolic

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18
Q

what murmur do you hear in tricuspid regurgitation

A

pan systolic

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19
Q

what murmur do you hear in a ventricular septal defect

A

pan systolic

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20
Q

what murmur do you hear in aortic stenosis

A

ejection systolic

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21
Q

what murmur do you hear in pulmonary stenosis

A

ejection systolic

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22
Q

what murmur do you hear in hypertrophic obstructive cardiomyopathy

A

ejection systolic

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23
Q

what is a splitting second heart sound

A

pulmonary valve closes slightly later than aortic valve

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24
Q

what type of murmur do you hear in tetralogy of fallot

A

ejection systolic

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25
Q

where do you hear the murmur in tetralogy of fallot

A

2nd intercostal space, left sternal border

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26
Q

what can cause right to left shunt/cyanotic heart disease

A

tetralogy of fallot
transposition of the great arteries
truncus
tricuspid abnormalities
total anomalous pulmonary venous return

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27
Q

when does a PDA usually stop functioning and then close

A

stops functioning 1-3 days after birth
closes within 2-3 weeks

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28
Q

what causes/is a potential risk factor for PDA

A

rubella
prematurity

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29
Q

pathophysiology caused by a PDA

A

aorta pressure > pulmonary vessels
left to right shunt
pulmonary hypertension and right sided heart strain
right ventricular hypertrophy
eventually left ventricular hypertrophy

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30
Q

what type murmur do you get in PDA

A

on 1st heart sound but may continue into second
continuous crescendo-decrescendo machinery murmur

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31
Q

what are the 3 types of ASD

A

ostium secondum
patent foramen ovale
osmium primum

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32
Q

what is the most common ASD

A

ostium secondum

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33
Q

complication of ASD

A

stroke (when patient has a DVT)
atrial fibrillation/flutter
pulmonary hypertension
eisenmenger syndrome

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34
Q

type of murmur heard in ASD

A

mid-systolic, crescendo-decrescendo murmur, fixed split second heart sound

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35
Q

Where is murmur heard in ASD

A

upper left sternal border

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36
Q

what conditions are commonly associated with a VSD

A

Downs syndrome
turners syndrome

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37
Q

what skin changes will a patient have in Eisenmengers syndrome and why

A

become cyanotic due to right to left shunt
bone marrow responds to hypoxia - polycythemia
plethoric complexion

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38
Q

what is coarctation of the aorta

A

narrowing o the aortic arch usually around the ductus arteriosus

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39
Q

what condition is coarctation of the aorta associated with

A

turners

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40
Q

presentation of someone with coarctation of the aorta

A

weak femoral pulses
high blood pressure in some limbs, lower in others

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41
Q

murmur heard in coarctation of the aorta and where

A

systolic
below left clavicle and left scapula

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42
Q

management of coarctation of aorta

A

prostaglandin E immediately after birth to keep ductus arteriosus open until surgical repair

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43
Q

associations with pulmonary valve stenosis

A

tetralogy of fallot
William syndrome
Noonan syndrome
congenital rubella syndrome

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44
Q

what are the component of tetralogy of fallot

A

ventricular septal defect
overriding aorta
pulmonary stenosis
right ventricular hypertrophy

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45
Q

risk factors for tetralogy of fallot

A

maternal rubella infection
increased maternal age
diabetic mother
alcohol consumption in pregnancy

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46
Q

what is seen on chest X-ray in tetralogy of fallot

A

boot-shaped heart

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47
Q

what are tet spells

A

intermittent periods where right to left shunt causing a cyanotic episode

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48
Q

how will an older child stand in a tet spell

A

squat

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49
Q

what is Ebsteins anomaly

A

tricuspid valve set lower in right side of the heart
causing a bigger right atrium and small right ventricle

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50
Q

what is ebsteins anomaly associated with

A

right to left shunt in ASD
Wolf-Parkinson-White syndrome
lithium use

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51
Q

what would the heart sound like in Ebsteins anomaly

A

gallop rhythm (addition of 3rd and 4th heart sound)

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51
Q

what would the heart sound like in Ebsteins anomaly

A

gallop rhythm (addition of 3rd and 4th heart sound)

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52
Q

what is transposition of the great arteries

A

attachments of aorta and pulmonary trunk are swapped

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53
Q

what does immediate survival depend on in transposition of the great arteries

A

having a shunt e.g. PDA, ASD, VSD

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54
Q

management of transposition of great arteries

A

prostaglandin infusion to maintain ductus arteriosus
balloon septostomy in foramen ovale to creat large ASD
cardiopulmonary bypass is only definitive treatment

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55
Q

what is bronchiolitis usually caused by

A

RSV (respiratory syncytial virus)

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56
Q

in what age are you likely to see/consider bronchiolitis

A

most common under 6 months
consider in under 1 year
consider in ex-premature babies with chronic lung disease under 2

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57
Q

what is the pathophysiology behind bronchiolitis

A

smallest amount of inflammation and mucus in airway causes air to circulate back to alveoli

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58
Q

presentation of bronchiolitis

A

coryza symptoms
signs of respiratory distress
dyspnoea, tachypnoea, apnoeas
poor feeding
mild fever <39

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59
Q

what will you hear on auscultation of bronchiolotis

A

wheeze and crackles on auscultation

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60
Q

what are signs of respiratory distress

A

raised respiratory rate
use of accessory muscles
intercostal and subcostal recessions
nasal flaring
head bobbing
tracheal tugging
cyanosis
abnormal airway noises

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61
Q

what is the typical course of bronchiolitis infection

A

coryzal symptoms - half spontaneously get better
chest symptoms 1-2 days after
day 3/4 worst
lasts 7-10 days
recovered within 2-3 weeks

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62
Q

what does bronchiolitis make you more likely to have in childhood

A

viral induced wheeze

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63
Q

when would you admit someone with bronchiolitis

A

<3 months
pre-existing condition e.g. prematurity, downs, cf
50-75% less normal milk intake
clinical dehydration
resp rate >70
oxygen sats <92%
moderate to severe respiratory distress
apnoeas
parents not confident in ability to manage

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64
Q

what can be given to high risk babies to prevent from getting RSV

A

palivizumab

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65
Q

how is viral induced wheeze different to astha

A

present before 3
no atopic history
only occurs during viral infections

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66
Q

how would you classify moderate asthma

A

peak flow >50% predicted
normal speech
no features

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67
Q

how would you classify severe asthma

A

peak flow <50% predicted
saturations <92%
unable to complete sentences in one breath
signs of respiratory distress
resp rate > 40 in 1-5 years old, >30 in >5 years old
heart rate >140 in 1-5 years old, >125 in >5 years old

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68
Q

how would you classify life threatening asthma

A

peak flow < 33% predicted
saturations <92%
exhaustion and poor respiratory effort
hypotension
silent chest
cyanosis
altered consciousness/confusion

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69
Q

what is the step up order of acute asthma management

A

salbutamol inhalers
nebulisers with salbutamol/ipratoprium bromide
oral prednisolone (1mg/kg once a day for 3 days)
IV hydrocortisone
IV magnesium sulfate
IV salbutamol
IV aminophylline

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70
Q

what is the step-down of salbutamol

A

10 puffs 2 hourly
10 puffs 4 hourly
6 puffs 4 hourly
4 puffs 6 hourly

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71
Q

what is the medical management of chronic asthma in a patient under the age of 5

A

SABA

8 week trial moderate dose ICS
(if fine after stay on SABA
if recurs within 4 weeks, start on ICS
if recurs after 4 weeks repeat trial)

add LRTA

refer to specialist

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72
Q

what is the medical management of chronic asthma in a patient aged 5-12

A

SABA
low dose ICS
LRTA
medium dose ICS
stop LRTA, start LABA
MART
referral to specialist

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73
Q

what is the medical management of chronic asthma in a patient > 12 years old

A

(same as adult)
SABA
low dose ICS
LABA
medium dose ICS
oral leukotriene receptor antagonist/oral theophylline/inhaled LAMA (tiotropium)
high dose ICS
combine treatments from step 5, oral salbutamol?
referral to specialist
oral steroids at low dose under guidance

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74
Q

presentation of pneumonia

A

cough - typically wet or productive
high fever > 38.5
tachypnoea/tachycardia
increased work of breathing
lethargy
delirium

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75
Q

chest signs of pneumonia

A

bronchial breast sounds
focal coarse crackles
dullness to percuss

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76
Q

bacterial causes of pneumonia

A

streptococcus pneumonia
group A strep
group B strep
staph aureus
haemophilus influenza
mycoplasma pneumonia

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77
Q

viral causes of pneumonia

A

RSV
parainfluenza
influenza

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78
Q

treatment for bacterial pneumonia

A

amoxicillin
+ macrolide to cover atypical (mono therapy if penicillin allergy)

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79
Q

when would you commonly see croup

A

6 months - 2 years

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80
Q

most common cause of croup

A

parainfluenza

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81
Q

presentation of croup

A

increased work of breathing
barking cough
hoarse voice
stridor
low grade fever

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82
Q

management of croup

A

single dose of 150mcg/kg dexamethasone (can be repeated if required after 12 hours)
if no response = oxygen, nebulised budesonide, nebulised adrenalin, intubation/ventilation

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83
Q

most common cause of epiglottitis

A

haemophilus influenza type B

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84
Q

presentation of epiglottitis

A

sore throat and stridor
drooling
tripod position
high fever
difficulty or painful swallowing
muffled voice

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85
Q

what would you see on investigation for epiglottitis

A

thumb/thumbprint sign on lateral X-ray of the neck

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86
Q

management of epiglottitis

A

prep for intubation/tracheostomy
ceftriaxone
dexamethasone

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87
Q

complication of epiglottitis

A

epiglottic abscess

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88
Q

what causes laryngomalacia

A

aryepiglottic folds are shortened pulling on epiglottis

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89
Q

what shape is seen in laryngomalacia

A

omega shape

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90
Q

presentation of laryngomalacia

A

~ 6 months
inspiratory stridor
more prominent when feeding, upset, lying on back or URTI

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91
Q

what causes whooping cough

A

bordetella pertussis

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92
Q

presentation of whooping cough

A

mild coryzal symptoms
paroxysmal cough starts after a week
loud inspiratory whoop
may get apnoeas

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93
Q

how do you diagnose whooping cough

A

nasopharyngeal swap with PCR testing/bacterial culture
if had cough > 2 weeks - anti-pertussis toxin immunoglobulin G

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94
Q

management of whooping cough

A

notifiable disease
macrolide antibiotics (clarithromycin <1 month, azithromycin or clarithromycin >1 month, erythromycin in pregnant women), co-trimoxazole as alternative
prophylactic antibiotics to close contacts
resolves within 8 weeks, long lasting cough

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95
Q

complication of whooping cough

A

bronchiectasis

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96
Q

prevention of chronic lung disease of prematurity/bronchopulmonary dysplasia

A

corticosteroids in mothers showing signs of prematurity
CPAP at birth
caffeine to stimulate respiratory effort
don’t over oxygenate

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97
Q

what causes cystic fibrosis

A

autosomal recessive condition = gene mutation of cystic fibrosis transmembrane conductance regulatory gene on chromosome 7 (delta-F508) with codes for chloride cellular channels

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98
Q

what are the key consequences in cystic fibrosis

A
  • thick pancreatic and biliary secretions blocking ducts resulting in lack of digestive enzymes e..g pancreatic lipase
  • low volume thick airway secretions reducing airway clearance = bacterial colonisation and susceptibility to airway infection
  • congenital bilateral absence of the vas deferens
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99
Q

how would cystic fibrosis present

A

often picked up on newborn blood spot test
Meconium ileus - not passing meconium within 24 hours leading to distention and vomiting
can present later in childhood with recurrent LRTI, failure to thrive or pancreatitis

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100
Q

symptoms of cystic fibrosis

A

chronic cough
thick sputum production
recurrent respiratory tract infections
steatorrhoea
salty sweat
poor height/weight gain

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101
Q

signs of cystic fibrosis

A

low weight/height
nasal polyps
finger clubbing
crackles/wheeze on auscultation
abdominal distention

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102
Q

gold standard diagnostic test for cystic fibrosis

A

sweat test
pilocarpine applied to skin, electrolodes either side, sweat absorbed
diagnosis if chlorine >60

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103
Q

common microbial colonisers in cystic fibrosis and what measures are taken

A

staph aureus - long term prophylactic flucloxacillin
pseudomonas - long term nebulised antibiotics e.g. tobramycin, oral ciprofloxacin

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104
Q

causes of clubbing in children

A

hereditary
cyanotic heart disease
IE
CF
TB
IBD
liver cirrhosis

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105
Q

what type of inheritance pattern if primary ciliary ddyskinesia/Kartagner’s syndrome

A

autosomal recessive

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106
Q

what is primary ciliary dyskinesia/Kartagner’s syndrome

A

affects motile cilia of cells in body, notably in respiratory = build up of mucus = more likely for infection, affects cilia in Fallopian tubes = reduced or absent fertility

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107
Q

what is Kartagner’s triad

A

paranasal sinusitis
bronchiectasis
situs inversus (internal organs on other side of body)

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108
Q

how do you diagnose primary ciliary dyskinesia/Kartagners syndrome

A

sample of ciliated epithelium of upper airway via nasal brushing or bronchoscopy to examine action of the cilia

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109
Q

what are abdominal migraines

A

central abdominal pain > 1 hour
associated nausea/pallor/headache/photophobia/aura
usually develop traditional migraines when older

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110
Q

how do you treat acute attack of abdominal migraine

A

low stimulus environment
paracetamol
ibuprofen
sumatriptan

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111
Q

preventative medications of abdominal migraine

A

pizotifen
propanolol
cyproheptadine
flunarizine (CCB)

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112
Q

what are signs of problematic reflux

A

chronic cough
hoarse cry
distress after feeding
reluctance to feed
pneumonia
poor weight gain
> 1 year old - heartburn, pain, bloating, nocturnal cough

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113
Q

what is Sandifer’s syndrome

A

brief episodes of normal movements
torticollis = forceful contraction of neck muscles causing twisting of neck
dystonia = abnormal muscle contractions causing twisting movement, arching of back or unusual postures

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114
Q

what is pyloric stenosis

A

hypertrophy of the ring of smooth muscle connecting stomach and duodenum causing food to be ejected into oesophagus when stomach peristalsis

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115
Q

presentation of pyloric stenosis

A

first few weeks of life
hungry, thin and pale baby failing to thrive
projectile vomiting
olive size feeling in abdomen

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116
Q

what would a blood gas show in pyloric stenosis

A

hypochlorite metabolic alkalosis

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117
Q

how do you diagnose pyloric stenosis

A

abdominal ultrasound

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118
Q

how do you treat pyloric stenosis

A

laparoscopic pyloromyotomy - Ramstedt’s operation

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119
Q

what is gastritis

A

inflammation of stomach presenting with nausea and vomtiing

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120
Q

what is enteritis

A

inflammation of the intestines presenting with diarrhoea

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121
Q

what is gastroenteritis

A

inflammation of the stomach all the way to the intestines presenting with nausea, vomiting and diarrhoea

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122
Q

what is the most common cause of gastroenteritis

A

viral - rotavirus, norovirus

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123
Q

what is the main concern in gastroenteritis

A

dehydration

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124
Q

how are you likely to get e.coli gastroenteritis

A

contact with infected faeces, unwashed salads or contaminated water

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125
Q

how are you likely to get campylobacter jejuni

A

travellers diarrhoea
raw/uncooked poultry, untreated water, unpasteurised milk

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126
Q

incubation period of campylobacter jejuni and when should it resolve by

A

incubation = 2-5 days
resolve after 3-6 days

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127
Q

what do you give if a person has e.coli gastroenteritis

A

no antibiotics - increased risk of HUS which is increased in e.coli due to shiga toxin

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128
Q

what would you give if a person had severe campylobacter jejuni

A

azithromycin or ciprofloxacin

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129
Q

how are you likely to get shigella gastroenteritis

A

faeces contaminating drinking water, swimming pools and food

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130
Q

what is the incubation period for shigella gastroenteritis and when would it usually resolve by

A

incubation = 1-2 days
resolve within 1 week

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131
Q

how are you likely to get salmonella gastroenteritis

A

raw eggs, poultry or food contaminated with infected faeces of small animals

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132
Q

what type of bacteria is bacillus cereus

A

gram positive rod

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133
Q

how are you likely to get bacillus cereus

A

inadequately cooked food/food not immediately refrigerated after cooking e.g. fried rice

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134
Q

how are you likely to get yersinia entercolitica gastroenteritis

A

raw or undercooked pork

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135
Q

antibodies associated with coeliac disease

A

anti-TTG and anti-EMA

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136
Q

genetic associations with coeliac disease

A

HLA-DQ2
HLA-DQ8

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137
Q

how would diagnose coeliac disease

A

stay on gluten containing diet
endoscopy and intestinal biopsy showing crypt hypertrophy and villous atrophy

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138
Q

how would you characterise signs of Crohns

A

NESTS
no blood or mucus
entire GI tract
skip lesions on endoscopy
terminal ileum most affected/transmural inflammation
smoking is a risk factor

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139
Q

how would you characterise signs of ulcerative colitis

A

CLOSEUP
continuous inflammation
limited to colon and rectum
only superficial mucosa affected
smoking is protective
excrete blood and mucus
use aminosalicylates
primary sclerosis cholangitis association

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140
Q

medication to induce remission in crohns

A

steroids = 1st line (oral pred/IV hydrocortisone)
immunosuppressants - azathioprine/methotrexate/infliximab

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141
Q

medication to maintain remission in crohns

A

1st line = azathioprine/mercaptopurine
alternatives = methotrexate/infiximab

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142
Q

medication to induce remission in ulcerative colitis

A

1st line = aminosalicylate (mesalazine)
2nd line = corticosteroids
severe = 1st line - IV corticosteroids, 2nd line = IV ciclosporin

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143
Q

medication to maintain remission in ulcerative colitis

A

aminoosalicylate
azathioprine
mercaptopurine

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144
Q

surgical management of ulcerative colitis

A

panproctocolectomy - permanent ileostomy or ileoanal anastomosis (J pouch)

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145
Q

what is biliary atresia

A

section of bile duct narrowed or absent so in cholestasis bile not transported from liver to bowel so excretion of conjugated of bilirubin is prevented

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146
Q

what is the presentation of biliary atresia

A

presents shortly after birth
jaundice (suspect in >14 days in term babies, >21 days in premature)

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147
Q

management of biliary atresia

A

Kasai portoenterostomy (attach section of small intestine to opening of liver)

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148
Q

presentation of intestinal obstruction

A

persistant vomiting - may be bilious
abdominal pain/distention
failure to pass stools/wind
abnormal bowel sounds (high pitched/tinkling in early obstruction, absent later)

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149
Q

what would you expect to see on an abdominal xray in intestinal obstruction

A

dilated loops of bowels proximal to obstruction
collapsed loops of bowel distal to obstruction
absence of air in the rectum

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150
Q

what is the pathophysiology behind Hirschsprung’s Disease

A

absence of parasympathetic ganglion cells in distal bowel/rectum

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151
Q

what is it called if the entire colon is affected in Hirschsprung’s disease

A

total colonic aganglionosis

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152
Q

associations with Hirschsprung’s disease

A

family history
downs syndrome
neurofibromatosis
Waardenburg syndroom (pale blue eyes, hearing loss, patches of white skin and hair)
multiple endocrine neoplasia type II

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153
Q

presentation of Hirschsprung’s disease

A

delay in passing meconium
chronic constipation since birth
abdominal pain and distention
vomiting
poor weight gain/failure to thrive

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154
Q

what is Hirschsprung-Associated Enterocolitis

A

inflammation and obstruction occurring in neonates with Hirschsprungs

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155
Q

presentation of HAEC

A

2-4 weeks after birth
fever
abdominal distention
bloody diarrhoea
sepsis

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156
Q

complication of HAEC

A

toxic megacolon and perforation

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157
Q

diagnostic test for HAEC

A

rectal biopsy showing absence of ganglionic cells

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158
Q

what is intususception

A

where the bowel invaginate/teloscopes into itself which thickens size of bowel and narrows lumen

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159
Q

when would you likely see intussusception

A

infants aged 6 months - 2 years
more common in boys

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160
Q

associations with intussusception

A

concurrent viral illness
Henoch-Schonlein purpura
cystic fibrosis
intestinal polyps
mocked diveritculum

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161
Q

presentation of intussusception

A

redcurrant jelly stool
mass in RUQ = sausage shaped
severe, colicky abdominal pain
pale, lethargic, unwell
vomiting
intestinal obstruction

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162
Q

peak incidence of appendicitis

A

10-20 years

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163
Q

signs/symptoms of appendicitis

A

abdominal pain (starts centrally, moves to RIF)
tenderness in McBurney’s point
loss of appetite
nausea/vomiting
Rovsing’s sign
guarding

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164
Q

what is Rovsings sign

A

palpation of LIF causes pain in RIF

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165
Q

what 2 signs of appendicitis indicates it is more likely peritonitis

A

rebound tenderness
percussion tenderness

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166
Q

what may trigger type 1 diabetes

A

coxsackie B virus and enterovirus

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167
Q

3 characteristic symptoms of a patient presenting with type 1 diabetes

A

polyuria
polydipsia
weight loss

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168
Q

treatment for severe hypoglycaemia

A

IV dextrose and IM glucagon

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169
Q

long term macrovascular complications in type 1 diabetes

A

coronary artery disease
peripheral ischaemia
stroke
hypertension

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170
Q

long term microvascular complications in type 1 diabetes

A

peripheral neuropathy
retinopathy
glomerulosclerosis

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171
Q

what is adrenal insufficiency

A

adrenal glands not producing enough steroid hormones e.g. cortisol and aldosterone

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172
Q

what is primary adrenal insufficiency

A

Addisons - adrenal glands been damaged (autoimmune)

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173
Q

what is secondary adrenal insufficiency

A

inadequate ACTH
damage to pituitary gland - hypoplasia, surgery, infection, loss of blood flow, radiotherapy

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174
Q

what is tertiary adrenal insufficiency

A

inadequate CRH release by hypothalamus
can be causes by long term oral steroids (>3 weeks)

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175
Q

features of adrenal insufficiency in babies

A

lethargy
vomiting
poor feeding
hypoglycaemia
jaundice
failure to thrive

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176
Q

features of adrenal insufficiency in older children

A

nausea or vomiting
poor weight gain/weight loss
reduced appetite
abdominal pain
muscle weakness or cramps
developmental delay/poor academic performance
bronze hyperpigmentation to skin

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177
Q

why do you get bronze hyperpigmentation to the skin in adrenal insufficiency

A

ACTH stimulates melanocytes

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178
Q

blood results seen in addisons

A

low cortisol
low aldosterone
high ACTH
high renin

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179
Q

blood results seen in seocndary adrenal insufficiency

A

low cortisol
low ACTH
normal aldosterone
normal renin

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180
Q

diagnostic test for adrenal insufficiency

A

short synacthen test

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181
Q

management of adrenal insufficiency

A

hydrocortisone
fludocortisone

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182
Q

what would be the presentation of Addisonian crisis/adrenal crisis

A

reduced consciousness
hypotension
hypoglycaemia, hyponatraemia and hyperkalaemia

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183
Q

what is congenital adrenal hyperplasia

A

deficiency of 21-hydroxylase enzyme which converts progesterone into aldosterone and cortisol so instead gets converted into testosterone

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184
Q

what is the inheritance pattern of congenital adrenal hyperplasia

A

autosomal recessive

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185
Q

presentation of severe congenital adrenal hyperplasia

A

hyponatraemia, hyperkalaemia, hypoglycaemia
in females - virilised/ambiguous genitalia, enlarged clitoris

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186
Q

presentation of females in mild congenital adrenal hyperplasia

A

tall for their age
facial hair
absent periods
deep voice
early puberty
hyperpigmentation

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187
Q

presentation of males in mild congenital adrenal hyperplasia

A

tall for their age
deep voice
large penis
small testicles
early puberty

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188
Q

2 causes of growth hormone deficiency

A

congenital - genetic mutation in GH1 or GHRHR, or empty sella syndrome
acquired - infection, trauma, surgery

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189
Q

what is empty sella syndrome

A

pituitary gland underdeveloped or damaged

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190
Q

presentation of growth hormone deficiency

A

micropenis
hypoglycaemia
severe jaundice
poor growth 2/3
short stature
slow development of movement and strength
delayed puberty

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191
Q

investigation to diagnose growth hormone deficiency

A

growth hormone stimulation test
- measure response to medications that should stimulate release of growth hormone e.g. glucagon, insulin, arginine and clonidine

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192
Q

treatment given in growth hormone deficiency

A

daily subcutaneous growth hormone - somatropin

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193
Q

what is it called when the thyroid is underdeveloped

A

dysgenesis

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194
Q

what is it called when the thyroid is fully developed but it doesn’t produce enough hormone

A

dyshormongenesis

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195
Q

how is congenital hypothyroidism diagnosed

A

newborn blood spot screening test

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196
Q

presentation of congenital hypothyroidism

A

prolonged neonatal jaundice
poor feeding
constipation
increased sleeping
reduced activity
slow growth and development

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197
Q

associations with acquired hypothyroidism/hashimotos

A

antithyroid peroxidase antibodies (anti-TPO)
antithyroglobulin autoantibodies

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198
Q

presentation of acquired hypothyroidism

A

fatigue/low energy
poor growth
poor weight gain
poor school performance
constipation
dry skin and hair loss

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199
Q

management of hypothyroidism

A

levothyroxine

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200
Q

when do you do USS’s in UTIs

A

< 6 months with 1st UTI - do USS within 6 weeks or during if atypical
recurrent - USS within 6 weeks
atypical - USS during

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201
Q

when do you test dimercaptosuccinic acid (DMSA) in UTI

A

4-6 months after to asses for damage

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202
Q

when do you do vesico-uteric reflux test in UTIs

A

recurrent/atypical in <6 months

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203
Q

what is vulvovaginitis

A

inflammation and irritation of the vulva and vagina

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204
Q

when would you often see vulvovaginitis

A

girls aged 3-10

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205
Q

what can vulvovaginitis be exacerbated by

A

wet nappies
use of chemicals/soaps
tight clothing
poor hygiene
constipation
threadworms
pressure
highly chlorinated pools

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206
Q

what is the classic triad of nephrotic syndrome

A

proteinuria
hypoalbuminaemia
oedema

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207
Q

2 main types of nephritic syndrome

A

post-streptococcal glomerulonephritis
IgA nephorpathy

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208
Q

signs seen in nephritic syndome

A

haematuria
proteinuria (less than nephrotic)

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209
Q

triad of Haemolytic Uraemia Syndrome

A

haemolytic anaemia
acute kidney injury
thrombocytopenia

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210
Q

what is enuresis

A

involuntary urination

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211
Q

what age is nocturnal enuresis typically controlled by

A

3-4 years

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212
Q

what age is diurnal enuresis typically controlled by

A

2 years

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213
Q

management of enuresis

A

desmopression taken at bedtime
oxybutynin
imipramine

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214
Q

inheritance pattern of polycystic kidney disease seen in children

A

autosomal recessive

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215
Q

pathophysiology of polycystic kidney disease

A

mutation of PKHD1 on chromosome 6
- codes for fibrocysin/polyductin protein complex
- responsible for creation of tubules and maintenance of healthy epithelial tissue in kidneys, liver and pancreas

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216
Q

features of polycystic kidney disease

A

cystic enlargement of renal collecting ducts
oligohydramnios, pulmonary hypoplasia and Potters syndrome

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217
Q

what is Potter syndrome

A

lack of amniotic fluid causing underdeveloped ear cartilage, low set ears, flat nasal bridge and skeletal abnormalities

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218
Q

what is multicystic dysplastic kidney

A

one kidney made up of many cysts, other normal
cystic one will atrophy and disappear before age of 5

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219
Q

what is Wilms tumour

A

tumour in kidney affecting children typically under 5

220
Q

what is a posterior urethral valve

A

tissue at proximal end of urethra causing obstruction of urine output

221
Q

what would be seen on an abdominal ultrasound in posterior urethral valve

A

enlarged, thickened bladder
bilateral hydronephrosis

222
Q

what is cryptochidism

A

undescended testes

223
Q

complications of undescended testes

A

testicular torsion
infertility
testicular cancer

224
Q

risk factors for undescended testes

A

family history
low birth weight
small for gestational age
prematurity
maternal smoking during pregnancy

225
Q

management of undescended testes

A

most will descend in 3-6 months
urologist if not by 6 months
orchidopexy between 6-12 months

226
Q

what is hypospadias

A

where the urethral meatus is abnormally displaced to the ventral side of the penis towards to scrotum

227
Q

what is epispadias

A

where the meatus is displaced to dorsal side

228
Q

what is a hydrocele

A

collection of fluid in the tunica vaginalis that surrounds the testes

229
Q

what happens in a simple hydrocele

A

common in newborn males
fluid will get reabsorbed over time

230
Q

what happens in a communicating hydrocele

A

tunica vaginalis is connected with the peritoneal cavity via the processes vaginalis
hydrocele can fluctuate in size

231
Q

what would be seen on examination of a hydrocele

A

soft, smooth, non-tender swelling around one of the testes
transilluminate

232
Q

what are the 5 components of the APGAR score

A

appearance
pulse
grimace
activity
respiration

233
Q

what is the scoring criteria for appearance in APGAR score

A

0 - blue/pale centrally
1 - blue extremities
2 - pink

234
Q

what is the scoring criteria for pulse in the APGAR score

A

0 = absent
1= <100
2 = >100

235
Q

what is the scoring criteria for grimace in the APGAR score

A

0 = no response
1 = little response
2 = good response

236
Q

what is the scoring criteria for activity in the APGAR score

A

0 = floppy
1 = flexed arms and legs
2 = active

237
Q

what is the scoring criteria for respiration in the APGAR score

A

0 = absent
1 = slow/irregular
2 = strong/crying

238
Q

positives of delayed umbilical cord clamping

A

improved haemoglobin, iron stores and blood pressure
reduction in intraventricular haemorrhage and necrotising enterocolitis

239
Q

negative of delayed cord clamping

A

increase in neonatal jaundice

240
Q

when should the newborn blood spot test be done

A

day 5 (8 at latest)

241
Q

what does the newborn blood spot test screen for

A

sickle cell disease
cystic fibrosis
phenylketonuria
congenital hypothyroidism
medium chain acyl-coA dehydrogenase deficiency
maple syrup urine disease
isovaleric acidaemia
glutamic acuduria type 1
homocystin

242
Q

what is Barlows test

A

baby on back, hips adducted and flexed at 90 degrees and knees bent to 90 degrees
apply gentle downwards pressure on knees through femur to see if femoral head will dislocate posteriorly

243
Q

what is Ortolani test

A

baby on back with hip and knees flexed
gentle pressure to abduct hips and apply pressure behind legs to see if hips will dislocate anteriorly

244
Q

what is Moro reflex

A

when rapidly tipped backward the arms and legs will extend

245
Q

what is rooting reflex

A

tickling the cheek will cause the baby to turn towards the stimulus

246
Q

what is caput succedaneum

A

fluid collecting on scalp outside of periosteum
pressure to area of scalp during traumatic, prolonged or instrumental delivery

247
Q

what is a cephalohaemaotma

A

collection of blood between the skull and periosteum
lump does not cross suture line
resolves in few months
risk of anemia and jaundice

248
Q

what is Erbs palsy

A

injury to C5/C6 nerves in brachial plexus during birth

249
Q

what is seen in the arm in Erbs palsy

A

waiter tip appearance
weakness of should abduction, external rotation, arm flexion and finger extension

250
Q

most communist cause of neonatal sepsis

A

group b streptococcus

251
Q

when would you initiate antibiotics in neonatal sepsis

A

2 or more clinical features or 1 red flag

252
Q

red flags for neonatal sepsis

A

confirmed or suspected sepsis in mother
signs of shock
seizures
term baby needing mechanical ventilation
respiratory distress > 4 hours after birth
presumed sepsis in another baby in a multiple pregnancy

253
Q

what is given in neonatal sepsis

A

benzylpenicillin and gentamycin

254
Q

what causes hypoxic-ischaemic encephalopathy

A

maternal shock
intrapartum haemorrhage
prolapsed cord
nuchal cord

255
Q

presentation of mild grade HIE

A

poor feeding, irritable, hyper-alert
resolves within 24 hours
normal prognosis

256
Q

presentation of moderate grade HIE

A

poor feeding, lethargic, hypotonic, seizures
can take weeks to resolve
~ half develop cerebral palsy

257
Q

presentation of severe grade HIE

A

reduced consciousness, apnoeas, flaccid and reduced/absent reflexes
50% mortality
~90% develop cerebral palsy

258
Q

what can help treat HIE

A

therapeutic hypothermia

259
Q

what is kernicterus

A

brain damage due to high bilirubin levels

260
Q

what is seen on a chest X-ray in respiratory distress syndrome

A

ground glass appearance

261
Q

risk factors for necrotising entercolitis

A

low birth weight/premature
formula feeds
respiratory distress and assisted ventilation
sepsis
PDA

262
Q

presentation of nectrosiing entercolitis

A

intolerance to feeds
vomiting - green bile
generally unwel
distended, tender abdomen
absent bowel sounds
blood in stools

263
Q

what would be seen on an abdominal X-ray for diagnosis of necrotising entercolitis

A

dilated loops of bowel
bowel wall oedema
pneumatosis intestinalis
pneumoperitoneum
gas in portal veins

264
Q

when would signs and symptoms present in neonatal abstinence syndrome

A

3-72 hours for opiates, diazepam, SSRIs and alcohol
24 hours - 21 days for methadone and other benzos

265
Q

CNS symptoms of neonatal abstinence syndrome

A

irritability
increased tone
high pitched cry
not settling
tremors
seizures

266
Q

vasomotor and respiratory symptoms of neonatal abstience syndrome

A

yawning
sweating
unstable temperature
tachypnoea

267
Q

metabolic and gastrointestinal symptoms of neonatal abstinence syndrome

A

poor feeding
regurgitation or vomiting
hypoglycaemia
loose stools with small nappy area

268
Q

what do you give for opiate withdrawal in neonatal abstinence syndrome

A

oral morphine sulfate

269
Q

what do you give for non-opiate withdrawal in neontaal abstinence syndrome

A

oral phenobarbitone

270
Q

symptoms of fetal alcohol syndrome

A

microcephaly
thin upper lip
smooth flat philtrum
short palpebral fissure
learning and behavioural disabilities

271
Q

features of congenital rubella syndrome

A

congenital cataracts
congenital heart disease
learning disability
hearing loss

272
Q

risk factors for sudden infant deaths syndrome

A

prematurity
low birth weight
smoking during pregnancy
male baby

273
Q

normal course of puberty in girls

A

development of breast buds, then pubic hair
start periods around2 years after the start of puberty

274
Q

normal course of puberty in boys

A

enlargement of testicles, then penis
gradual darkening of the scrotum
development of pubic hair
deepening of the voice

275
Q

what causes hypergonadotrophic hypogonadism

A

previous damage to gonads
congenital absence of testes or ovaries
Klinefelters syndrome
turners syndrome

276
Q

genetics in Klinefelters syndrome

A

male has additional X chromosome
47 XXY

277
Q

features of Klinefelter syndrome

A

taller height
wider hips
gynaecomastia
weaker muscles
small testicles
reduced libido
shyness
subtle learning difficulties

278
Q

genetics of turners syndrome

A

female has single X chromosome

279
Q

features of turners syndrome

A

short stature
webbed neck
widely spaced nipples
high arching palate
ptosis
cubitus valgus - abnormal feature of elbow
underdeveloped ovaries
late or incomplete puberty

280
Q

inheritance of Noonan syndrome

A

autosomal dominant

281
Q

features of Noonan syndrome

A

short stature
broad forehead
ptosis
hypertelorism - wide space between eyes
prominent nasolabial folds

282
Q

inheritance of Marfan syndrome

A

autosomal dominant

283
Q

features of Marfan syndrome

A

tall stature
long neck, limbs, fingers
hyper mobility
pectus carinatum or excavatum

284
Q

inheritance / genetic of fragile x syndrome

A

x linked (males always affected, females can vary)
mutation in FMR1

285
Q

features of fragile x syndrome

A

delay in speech and language development
long narrow face
large ears and testicles
hypermobility
ADHD, autism
seizures

286
Q

genetics behind Prader-Willi syndrome

A

loss of functional gene on proximal arm of chromosome 15 inherited from father

287
Q

features of Prader-Willi syndrome

A

constant insatiable hunger
hypotonia
hypogonadism
mild-moderate learning disability
fair, soft skin prone to bruising
almond shaped eyes

288
Q

genetics behind Angelman syndrome

A

loss of function of the uBE3A gene, deletion on chromosome 15

289
Q

key features of angelman syndrome

A

unusual fascination with water
happy demeanour
widely spaced teeth

290
Q

genetics behind William Syndrome

A

deletion of material on one copy of chromosome 7

291
Q

key features of William syndrome

A

very sociable
starburst eyes
wide mouth with big smile

292
Q

associated conditions with William syndrome

A

supravalvular aortic stenosis
hypercalcaemia

293
Q

what is cerebral palsy

A

non-progressive, permanent neurological problems resulting from damage to the brain around the time of birth

294
Q

antenatal causes of cerebral palsy

A

maternal infections
trauma during pregnancy

295
Q

perinatal causes of cerebral palsy

A

birth asphyxia
preterm birth

296
Q

post natal causes of cerebral palsy

A

meningitis
severe neonatal jaundice
head injury

297
Q

4 types of cerebral palsy

A

spastic
dyskinetic
ataxic
mixed

298
Q

what happens in spastic cerebral palsy

A

hypertonia and reduced function due to damage to UPN ( can be monoplegia, hemiplegia, diplegia and quadriplegia)

299
Q

what happens in dyskinetic cerebral palsy

A

hyper and hypo Tonia, athetoid movements and oro-motor problems
damage to basal ganglia

300
Q

what happens in ataxic cerebral palsy

A

problems with coordinated movement due to damage to cerebellum

301
Q

what is a concomitant squint

A

differences in control of extra ocular muscles

302
Q

what is a paralytic squint

A

paralysis in one or more of extra ocular muscles

303
Q

what is esotropia

A

inward positioned squint

304
Q

what is exotropia

A

outward positioned squint

305
Q

most common cause of hydrocephalus

A

aqeductal stenosis

306
Q

presentation of hydrocephalus

A

increased occipito-frontal circumference
bulging anterior fontanelle
poor feeding and vomiting
poor tone
sleepiness

307
Q

treatment for hydrocephalus

A

ventriculoperitoneal shunt

308
Q

what is craniosynostosis

A

where the skulls sutures close permanently leading to raised ICP

309
Q

inheritance of Duchennes muscular dystrophy

A

x linked recessive

310
Q

what is Gower’s sign

A

specific technique to stand up from lying down with proximal muscle weakness
hands and knees, push hips upwards and backwards, shift weight backwards, bring hands to knees, walk hands up body

311
Q

inheritance of spinal muscle atrophy

A

autosomal recessive

312
Q

what does spinal muscle atrophy affect

A

lower motor neurons

313
Q

1st line for depression in children

A

fluoxetine

314
Q

2nd line for depression in children

A

sertraline or citalopram

315
Q

1st line for anxiety in children

A

sertraline

316
Q

medications for ADHD

A

central nervous system stimulants
methylphenidate, dexamfetamine, atomoxetine

317
Q

what is Russell’s sign

A

calluses on the knuckles, seen in bulimia

318
Q

what electrolyte results would you see in referring syndrome

A

hypomagnesaemia
hypokalaemia
hypophopsphataemia

319
Q

what is copropraxia

A

type of complex tic
obscene gestures

320
Q

what is coprolalia

A

type of complex tic
obscene words

321
Q

when would physiologic anaemia of infancy present

A

6-9 weeks

322
Q

when would physiologic anaemia of infancy present

A

6-9 weeks

323
Q

inheritance/genetics of sickle cell disease

A

autosomal recessive
abnormal gene for beta-globin on chromosome 11

324
Q

general management of sickle cell disease

A

penicillin as antibiotic prophylaxis
hydroxycarbamide (stimulates fetal haemoglobin production)

325
Q

what happens in a vaso-occulsive sickle cell crisis

A

cells clog capillaries and cause distal ischaemia
dehydration and raised haematocrit

326
Q

what happens in a splenic sequestration sickle cell crisis

A

cells block blood flow within spleen

327
Q

what happens in an aplastic sickle cell crisis

A

temporary loss of creation of new blood cells
triggered by parvovirus B19

328
Q

inheritance of thalassaemia

A

autosomal recessive

329
Q

chromosome affected in alpha thalassaemia

A

16

330
Q

chromosome affected in beta thalassaemia

A

11

331
Q

inheritance of hereditary spherocytosis

A

autosomal dominant

332
Q

inheritance of G6PD deficiency

A

x linked recessive

333
Q

what can G6PD deficiency be triggered by

A

fava beans

334
Q

what is the presentation of G6PD deficiency

A

neonatal jaundice
Heinz bodies seen on blood film

335
Q

how do you confirm diagnosis of anaphylaxis

A

measure serum mast cell tryptase within 6 hours of event

336
Q

what is Digeorge syndrome

A

CATCH 22
congenital heart disease
abnormal facial appearance
thymus gland incompletely developed
cleft palate
hypoparathyroidism
22nd chromosome affected

337
Q

inactivated vaccinations

A

polio
flu
hep a
rabies

338
Q

subunit and conjugate vaccinations

A

pneumococcus
meningococcus
hep b
pertussis
hib
hpv
shingles

339
Q

live attenuated vaccinations

A

MMR
BCG
chickenpox
nasal influenza
rotavirus

340
Q

what vaccinations are given at 8 weeks

A

6 in 1
meningococcal type B
rota virus

341
Q

what vaccinations are given at 12 weeks

A

6 in 1
pneumococcal
rotavirus

342
Q

what vaccinations are given at 16 weeks

A

6 in 1
meningococcal type B

343
Q

what vaccinations are given at 1 year

A

2 in 1
pneumococcal
MMR
meningococcal type B

344
Q

what vaccination is given yearly between years 2 and 8

A

influenza nasal vaccine

345
Q

what vaccinations are given at 3 years 4 months

A

4 in 1
MMR

346
Q

what vaccination is given aged 12-13

A

HPV (2 doses 6-24 months apart)

347
Q

what vaccinations are given aged 14 years

A

3 in 1
meningococcal groups A, C, W, Y

348
Q

what vaccinations are in the 6 in 1

A

diptheria
tetanus
pertussis
polio
Hib
hep B

349
Q

what vaccinations are in the 2 in 1

A

hib
meningococcal type c

350
Q

what vaccinations are in the 4 in 1

A

diphtheria
tetanus
pertussis
polio

351
Q

what vaccinations are in the 3 in 1

A

diptheria
tetanus
polio

352
Q

most common cause of meningitis in > 3 months

A

neisseria meningitidis

353
Q

most common cause of meningitis in neonates

A

group b strep

354
Q

treating meningitis in < 3 months

A

cefotaxime plus amoxicillin

355
Q

treating meningitis in > 3 months

A

ceftriaxone

356
Q

what is added to meningitis treatment if risk of penicillin resistant pneumococcal infection

A

vancomycin

357
Q

most common cause of encephalitis

A

HSV (type 1 in children, 2 in neonates)

358
Q

contraindications for LP

A

GCS <9
Haemodynamically unstable
active seizures or post ictal

359
Q

treatment for encephalitis

A

acyclovir

360
Q

treatment for encephalitis if caused by CMV

A

ganiclovir

361
Q

testing for infectious mononucleosis/glandular fever

A

heterophiles antibodies (takes 6 weeks to be produced)
monospot test - react to RBCs from horses
Paul-Bunnel- from sheep

362
Q

incubation period of mumps

A

14-25 days

363
Q

what HIV level indicates having a normal vaginal delivery is best

A

<50

364
Q

what HIV level indicates the need to consider a c-section

A

5-400

365
Q

what HIV indicates the need for a C-section

A

> 400

366
Q

what is given in c-section if the viral load of HIV is unknown/>10000

A

IV zidovudine

367
Q

what does HBsAg mean in Hep B

A

active infection

368
Q

what does HBeAg mean in Hep B

A

marker of viral replication, implies high infectivity

369
Q

what does HBcAB mean in Hep B

A

past or current infection

370
Q

what does HBsAb mean in Hep B

A

implies vaccination or past/current infection

371
Q

what does HBV DNA mean in Hep B

A

direct count of viral load

372
Q

most common cause of tonsilitis

A

group A strep (pyogenes)

373
Q

treatment for tonsilitis

A

phenoxymethylpenicillin, clarithromycin if true penicillin allergy

374
Q

centor criteria for tonsilitis

A

fever >38
tonsillar exudates
absence of cough
tender anterior cervical lymph nodes

375
Q

feverPain score for tonsilitis

A

fever
purulence
attended within 3 days
inflammed tonsils
no cough or coryza

376
Q

what is trismus

A

unable to open mouth

377
Q

what type of voice do you get in a quinsy/peritonsillar abscess

A

hot potato voice

378
Q

indications for tonsillectomy

A

7 or more in 1 year
5 per year in 2 year
3 per year in 3 years
recurrent abscesses > 2 episodes
enlarged tonsils causing difficulty breathing

379
Q

most common cause of otitis media

A

strep pneumoniae

380
Q

management of otitis media

A

amoxillin for 5 days

381
Q

what is glue ear

A

otitis media with effusion

382
Q

what would you see on glue ear

A

dull tympanic membrane with air bubbles

383
Q

management of glue ear

A

grommets

384
Q

congenital causes of hearing loss

A

maternal rubella or CMV
genetic deafness
associated syndrome e.g Down syndrome

385
Q

perinatal causes of hearing loss

A

hypoxia during or after birth
prematurity

386
Q

causes of hearing loss after birth

A

jaundice
meningitis/encephaltiis
otitis media/glue ear
chemotherapy

387
Q

where do nosebleeds originate from

A

Kiesselbach’s plexus/Littles area

388
Q

how do you treat tongue tie

A

frenotomy

389
Q

what is a cystic hygroma and where would you see it

A

cyst filled with lymphatic fluid typically located in posterior triangle of neck on left hand side

390
Q

what causes of thyroglossal cyst

A

thyroglossal duct persists

391
Q

key differential of a thyroglossal cysts

A

ectopic thyroid tissue

392
Q

how would you tell it is a thyroglossal cyst

A

moves with movement of tongue

393
Q

what is a branchial cyst

A

round, soft, cystic swelling between angle of the jaw and sternocleidomastoid in anterior triangle

394
Q

common causes of hip pain aged 0-4

A

septic arthritis
developmental dysplasia of the hip
transient synovitis

395
Q

common causes of hip pain aged 5-10

A

septic arthritis
transient synovitis
perthes disease

396
Q

common causes of hip pain aged 10-16

A

septic arthritis
slipped upper femoral epiphysis
JIA

397
Q

types of growth plate fractures

A

SALTR
straight across
above
below
through
crush

398
Q

what is the most common cause of septic arthritis

A

staphylococcus aureus
(neisseria gonnorhoea in sexually active teenagers)

399
Q

most common cause of hip pain aged 3-10

A

transient synovitis following recent viral URTI

400
Q

what is Perthes disease

A

disruption of blood flow to the femoral head causing avascular necorisis

401
Q

who’s most commonly affected by Perthes disease

A

boys 5-8

402
Q

what causes Perthes disease

A

minor trauma - slipped upper femoral epiphysis

403
Q

how do you manage Perthes disease

A

keep hip in external rotation

404
Q

most common cause of osteomyelitis

A

staphylococcus aureus

405
Q

gold standard investigation for osteomyelitis

A

MRI

406
Q

what would you see on an X-ray in osteosarcoma

A

fluffy appearance
sunburst appearance

407
Q

what are the 2 types of talipes

A

equinovarus
calcaneolvalgus

408
Q

what would you get in equinovarus talipes

A

plantar flexion an supination

409
Q

what would you get in calcaneolvalgus talipes

A

dorsiflexion and pronation

410
Q

how do you treat talipes

A

Ponseti method

411
Q

best investigation for developmental dysplasia of the hip

A

ultrasound

412
Q

what is the management of developmental dysplasia of the hip

A

Pavlik harness < 6 months

413
Q

what is achondroplasia

A

disproportionate short stature

414
Q

what is the inheritance pattern of achondroplasia

A

autosomal dominant

415
Q

what is the genetic abnormality in achondroplasia

A

FGFR3 on chromosome 4

416
Q

what is Osgood-Schlatters disease

A

inflammation at the tibial tuberosity where the patella ligament inserts

417
Q

what is osteogenesis imperfecta

A

brittle bones prone to fracture due to malformation of collagen

418
Q

presentation of osteogenesis imperfecta

A

hyper mobility
blue/grey sclera

419
Q

when would you diagnose JIA

A

> 6 weeks in <16 year old

420
Q

what are the 5 types of JIA

A

systemic JIA/Stills disease
polyarticular
oligoarticular
enthesitis
juvenile psoriatic arthritis

421
Q

characteristic sign of systemic JIA/stills disease

A

subtle salmon pink rash

422
Q

complication of Systemic JIA/Stills

A

macrophage activation syndrome
low ESR, acutely unwell, DIC

423
Q

what is polyarticular JIA

A

5 or more joints

424
Q

what is oligoarticular JIA

A

4 or less joints
girls < 6

425
Q

what is enthesitis related arthritis

A

males > 6
HLA-B27

426
Q

scoring system for Ehlers Danlos Syndrome

A

beighton score - one score for each side of the body
palms flat on floor with straight legs
elbows hyperextend
knees hyperextend
thumb can bend to touch forearm
little finger hyperextends > 90 degrees

427
Q

what is POTS

A

postural othrostatic tachycardia syndrome
result of autonomic dysfunction

428
Q

what is Henoch-Schonlein purpura

A

Upper airway infection or gastroenteritis triggers an IgA vasculitis

429
Q

classic 4 features of HSP

A

purpura
joint pain
abdominal pain
renal involvement (–> nephrotic syndrome)

430
Q

what is Kawasaki disease

A

systemic medium sized vessel vasculitis

431
Q

who does Kawasaki disease more commonly affect

A

< 5 years
more common in Asian children
more common in boys

432
Q

presentation of Kawasaki disease

A

persistant high fever > 5 days
widespread erythematous maculopapular rash and desquamation
strawberry tongue
cracked lips
bilateral conjunctivitis
cervical lymphadenopathy

433
Q

complication of Kawasaki disease

A

coronary artery aneurysms

434
Q

management of Kawasaki disease

A

high dose aspirin
IvIgG

435
Q

what causes rheumatic fever

A

group a beta-haemolytic strep - pyogenes (from tonsilitis)

436
Q

investigation for rheumatic fever

A

ASO antibody

437
Q

diagnosis criteria for rheumatic fever

A

Jones criteria ‘‘JONES-FEAR”

438
Q

Major criteria for rheumatic fever

A

JONES
joint arthritis
organ inflammation
nodules
erythema marginatum rash
sydenham chorea

439
Q

minor criteria for rheumatic fever

A

FEAR
fever
ECG changes - prolonged PR - without carditis
Arthralgia without arthritis
raised inflammatory markers

440
Q

steroid ladder for eczema

A

hydrocortisone
clobetasone
betamethasone
clobetasol

441
Q

common bacterial infection cause in eczema and treatment

A

staphylococcus aureus
flucloxacillin

442
Q

most common type of psoriasis in children

A

guttate
small raised papules over trunk and limbs
triggered by strep infection, stress or medications

443
Q

what is Auspitz sign

A

small points of bleeding when plaques are scraped off in psoriasis

444
Q

what is Koebner phenomenon

A

development of psoriatic lesions to area of skin affected by trauma

445
Q

management of psoriasis

A

topical steroids
calcipotriol
dithranol
phototherapy with narrow band ultraviolet b light

446
Q

what is a macule

A

flat marks

447
Q

what is a papule

A

small lumps

448
Q

what is a pustule

A

small lumps with yellow pus

449
Q

what are comedomes

A

skin coloured papule representing blocked pulosebaceous units

450
Q

what are black heads

A

comedones with black pigmentation in centre

451
Q

what are ice pick scars

A

indentations in skin remaining after acne lesions heal

452
Q

what are hypertrophic scars

A

small lumps in the skin remaining after acne lesions heal

453
Q

what are rolling scars

A

irregular wave-like irregularities of the skin that remain after acne lesions heal

454
Q

management of acne

A

topical benzoyl peroxide
topical retinoids
topical antibiotics
oral antibiotics
oral contraceptive pill

455
Q

what are the 6 red rashes for viral exanthemas

A

measles
scarlet fever
rubella
dukes disease
parvovirus b19
roseola infantum

456
Q

what causes scarlet fever

A

group A strep infection

457
Q

characteristic signs for scarlet fever

A

strawberry tongue
sandpaper skin
red-pink blotchy macular rash

458
Q

how do you treat scarlet fever

A

phenoxymethylpenicilin

459
Q

characteristic sign of parvovirus b19

A

slapped cheek syndrome

460
Q

what causes roseola infantum

A

HHV-6

461
Q

key sign in erythema multiforme

A

target lesions

462
Q

what causes hand foot and mouth disease

A

coxsackie A virus

463
Q

what causes pityriasis rosea

A

HHV 6/7

464
Q

characteristic sign in pityriasis rosea

A

herald patch

465
Q

what is the fungus called that causes ringworm

A

trichophyton

466
Q

where the types of ringworm affect - tines capitis

A

scalp

467
Q

where the types of ringworm affect - tinea pedis

A

affects feet

468
Q

where the types of ringworm affect - tinea cruris

A

groin

469
Q

where the types of ringworm affect - tines corporis

A

body

470
Q

what is onchomycosis

A

fungal nail infection caused by ringworm

471
Q

classic location of scabies

A

between finger webs

472
Q

what is panniculitis

A

inflammation of the subcutaneous fat on shins seen in erythema nodosum

473
Q

what causes impetigo

A

staphylococcus auerus

474
Q

appearance of impetigo rash

A

golden crust appearance

475
Q

what is Nikolsky sign

A

in bullous impetigo - staphylococcus scaled skin syndorome - gentle rubbing of skin causes it to peel away

476
Q

appropriate test to confirm pyloric stenosis diagnosis

A

test feed

477
Q

managing cows milk protein intolerance for formula fed baby

A

trial of extensively hydrolysed formula

478
Q

investigation of choice for intussuception

A

abdominal ultrasound

479
Q

what would you see on abdominal ultrasound in intussusception

A

bull’s eye sign

480
Q

what does bowel sounds in a respiratory exam indicate

A

diaphragmatic hernia

481
Q

what do you need to monitor in methylpredinate

A

growth every 6 months

482
Q

when would you likely see benign rolandic epilepsy

A

aged 4-12

483
Q

what is benign rolandic epilepsy

A

seizures commonly occurring at night
partial seizure - can progress to secondary generalised
child is well

484
Q

what would an ECG show in benign rolandic epilepsy

A

centro-temporal spikes

485
Q

what is the genetic abnormality in Patau syndrome

A

trisomy 13

486
Q

key features of Patau syndrome

A

polydactyly
cleft palate and lips
microcephaly

487
Q

how do you differentiate between infantile spasms and spasms due to colic

A

will be distressed between between spasms in infantile spasms, in colic will be distressed during spasm

488
Q

investigation in infantile spasm

A

ECG - hyperarrythmias seen in West syndrome

489
Q

what would you hear in venous hums and where

A

continuous blowing murmur heard under clavicles

490
Q

what would you hear in stills murmur and where

A

low pitched sound heard at lower left sternal border

491
Q

most common complication of measles

A

otitis media

492
Q

complication of fragile X syndrome

A

mitral valve prolapse

493
Q

what is chondromalacia patellae

A

softening of the cartilage of the patella

494
Q

what group would you likely see chondromalacia patellae

A

teenage girls

495
Q

characteristic sign of chondromalacia patellae

A

anterior knee pain walking up and down stairs and when rising from sitting

496
Q

what is characteristic of osteochondritis dissecans

A

swelling and locking

497
Q

what are newborns offered if their hearing exam is abnormal

A

auditory brainstem response test

498
Q

how do you treat mycoplasma pneumoniae

A

erythromycin

499
Q

what is given to promote ductus arteriosus closure

A

ibuprofen or indomethacin

500
Q

cause of painless massive GI bleed in 1-2 years

A

Meckel diverticulum

501
Q

management of necrotising enterocolitis

A

laparotomy

502
Q

management for intussecption

A

pneumatic reduction under fluoroscopic guidance

503
Q

why don’t you give NSAIDs in chicken pox

A

increases risk of necrotising fasciitis

504
Q

most common cardiac pathology associated with Duchennes

A

dilated cardiomyopathy

505
Q

common complication of roseola infantum

A

febrile convulsions

506
Q

management for intestinal malrotation

A

Ladd’s procedure

507
Q

investigation of choice for stable Meckel diverticulum

A

technetium scan

508
Q

what would a chest X-ray show in transient tachypnoea of a newborn

A

hyperinflation of lungs and fluid in the horizontal fissures

509
Q

what happens when taking amoxicillin for glandular fever

A

maculopapular pruritic rash develops

510
Q

when can a child with scarlet fever return to school

A

24 hours after starting antibiotics

511
Q

when can a child with measles return to school

A

4 days after onset of rash

512
Q

when can a child with whooping cough return to school

A

48 hours after starting antibiotics

513
Q

when can a child with rubella return to school

A

5 days after onset of rash

514
Q

where should the pulse be checked in infant bLS

A

brachial and femoral

515
Q

triad of shaken baby syndrome

A

retinal haemorrhages
subdural haematoma
brain swelling

516
Q

adrenaline dose for anaphylaxis

A

< 6 = 50
6-12 = 300
12+ = 500

517
Q

what is a naevus flammeus

A

port wine stain
present from birth and grows with the child

518
Q

what are the contraindications for waiting to prescribe antibiotics for acute otitis media

A

AOM in hearing ear
cochlear implant
bilateral AOM <2 years old
ruptured tympanic membrane

519
Q

what is the name of the surgical procedure used to treat hirschsprung’s

A

Swenson

520
Q

what is the name of the surgical procedure used to treat Meckel divetituclum if symptomatic

A

wedge excision

521
Q

what is the most commonest cause of a convergent squint

A

hypermetropia

522
Q

when would you roughly count someone as having severe DKA/10% dehydration

A

ph < 7.1

523
Q

most common cause of death in measles

A

pneumonia

524
Q

what can you give if mild bleeding in ITP

A

tranexamic acid

525
Q

what do you give a child <3 months for a UTI

A

cefuroxime

526
Q

what do you give an unwell child >3 months for pyelonephritis

A

cefuroxime

527
Q

what do you give a well child > 3 months for pyelonephritis

A

cefalexin or co-amox

528
Q

what do you give an unwell child >3 months for UTI

A

cefuroxime

529
Q

what do you give a well child >3 months for UTI

A

trimethoprim

530
Q

fluids given to a 0-1 day old

A

50-60ml/kg

531
Q

fluids given to a 2 day old

A

70-80ml/kg

532
Q

fluids given to a 3 day old

A

80-100ml/kg

533
Q

fluids given to a 4 day old

A

100-120ml/kg

534
Q

fluids given to a 5-28 day old

A

120-150ml/kg

535
Q

Second line medication for ADHD

A

lisdexamfetamine

536
Q

common ECG finding at a paediatric cardiac arrest

A

asystole

537
Q

most common dysarthrythmia in paeds

A

supra ventricular tachycardia

538
Q

common cause of late onset neonatal sepsis

A

staph aureus

539
Q

what differentiates IgA nephropathy from post-strep glomerulonpehritis

A

IgA few days after infection, post-strep is few weeks

540
Q

what is Prehns sign

A

in testicular torsion, elevation doesn’t relieve pain

541
Q

triad for ADHD

A

inattention
hyperactivity
impulsivity

542
Q

when is a macrolide no longer indicated for whooping cough

A

when child has had cough >21 days

543
Q

difference between periorbital and orbital cellulitis

A

orbital is a life threatening event
pain on movement, vision changes and proptosis

544
Q

when would you give 40mmol/L KCL until in dka

A

until glucose levels are <14

545
Q

medication for infantile spasms

A

prednisolone
vigabatrin

546
Q

inheritance of Kallmans

A

autosomal recessive

547
Q

what is raised in CAH

A

17a-hydroxyprogesterone

548
Q

poor prognostic factors in ALL

A

being male
<2 or >10
WBC >20x10^9 on diagnosis
T or B cell surface markers
non-Caucasian

Phase 3a (7 decks)

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