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Phase 3A > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

what are 3 characteristic signs of pneumonia on examination?

A

bronchial breath sounds
focal coarse crackles
dullness to percussion

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2
Q

what is the most common cause of pneumonia in children?

A

streptococcus pneumonia

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3
Q

what is the most common viral cause of pneumonia?

A

respiratory syncytial virus (RSV)

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4
Q

what is the typical x-ray finding in s. aureus pneumonia?

A

pneumatocoeles (round air filled cavities) and multilobe consolidation

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5
Q

what are 2 bacteria that are more likely to cause pneumonia in pre/un-vacinated children?

A

GBS
Haemophilus influenza

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6
Q

which bacterial pneumonia can cause extrapulmonary manifestations and what are they?

A

Mycoplasma pneumonia
Can cause erythema multiforme

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7
Q

what is the management of pneumonia?

A

1st - amoxicillin

+/- macrolide (erythro, clarithro, azithro) for atypicals or allergy

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8
Q

what is the name of the condition where people cannot convert IgM to IgG so cannot form long term immunity?

A

immunoglobulin class-switch recombination deficiency

VACCINES DONT WORK!

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9
Q

what age group is typically affected by croup?

A

6 months - 2years

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10
Q

what is the pathophysiology of croup?

A

upper resp tract Ix causing oedema of the larynx

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11
Q

what is the most common pathogenic cause of croup?

A

parainfluenza virus

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12
Q

what are 2 key features of croup caused by parainfluenza virus?

A

improves in <48 hours
responds well to dexamethasone

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13
Q

what are 3 other common pathogenic causes of croup

A

influenza
adenovirus
RSV

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14
Q

what is a possible cause of croup especially in pre/unvaxinated children?

A

diphtheria - leads to epiglottitis!!! high mortality!

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15
Q

what are 5 symptoms of croup?

A

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

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16
Q

what is the treatment for croup?

A

supportive

oral dexamethasone - single dose 150 mcg/Kg (can repeat at 12 hours)

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17
Q

what is the stepwise management of severe croup?

A

oral dexamethasone
O2
Neb budesonide
Neb adrenaline
Intubation and ventilation

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18
Q

what age is virally induced wheeze typical in?

A

<3 years

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19
Q

what is the pathophysiology of viral induced wheeze?

A

viral infection causes inflammation and oedema which reduces space for air flow in a greater proportion due to small size of child’s airways- Poiseuilles law

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20
Q

what is the management of viral induced wheeze?

A

same as acute asthma

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21
Q

what kind of wheeze is heard in asthma and viral wheeze?

A

expiratory wheeze throughout

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22
Q

what is moderate asthma?

A

peak flow >50% predicted
normal speech
otherwise well

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23
Q

what is severe asthma? (6)

A

peak flow <50% predicted
sats <92%
unable to complete sentences
signs of respiratory distress
RR >40 1-5years, >30 5+ years
HR >140 1-5 years, >125 5+ years

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24
Q

what is life threatening asthma?

A

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

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25
Q

what is a physical finding on the chest wall in severe chronic asthma?

A

harrison sulus - indentation in chest wall along 6th rib can be bilateral or unilateral

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26
Q

what is the acute management of asthma/viral wheeze?

A

Escalating Bronchodilators - Salbutamol, ipratropium bromide, magnesium sulphate, aminophylline

PLUS - Steroids - prednisone (oral) or Hydrocortisone IV

ABx - if suspected

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27
Q

what is the stepwise progression of bronchodilators in acute asthma?

A

IHR/Neb salbutamol (10 puffs every 2 hours or repeat nebs every 20-30 mins)
IHR/Neb ipratropium bromide
IV magnesium sulphate
IV aminophylline

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28
Q

what on spirometry suggests an obstructive picture?

A

reversibility
FEV1 <80%, FEV1:FVC <0.7

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29
Q

what are 4 investigations for asthma?

A

spirometry
direct bronchial challenge
fractional exhaled nitric oxide
peak flow variability

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30
Q

what is the long term management of asthma over 5?

A

Reliever - SABA (salbutamol)
Preventer
1 - ICS
2 - ICS + LTRA (leukotriene receptor antagonist - montelukast)
3 - ICS + LABA (-LTRA)
4 - MART (maintenance and reliever therapy, steroid + (S)LABA)

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31
Q

which LABA can be used as a short acting agonist also?

A

folmeterol

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32
Q

can ICS cause restricted growth?

A

can reduced adult hight with long term use by up to 1cm . dose dependent effect

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33
Q

what is the most common cause of bronchiolitis?

A

Respiratory syncytial virus (RSV)

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34
Q

in what age group does bronchiolitis occur?

A

<1 year, most common <6 months can be up to 2 years

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35
Q

what are 8 typical signs of bronchiolitis?

A

coryzal symptoms - URTI
signs of resp distress
dyspnoea
tachypnoea
poor feeding
mild fever
apnoea
wheeze and crackles

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36
Q

what are 8 signs of respiratory distress?

A

raised RR
use of accessory muscles
intercostal and subcostal recession
nasal flaring
head bobbing
tracheal tugging
cyanosis
abnormal airway noise

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37
Q

what is the typical course of RSV bronchiolitis?

A

coryzal symptoms
chest symptoms 1-2 days later
worst day 3-4
symptoms usually last 7-10 days

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38
Q

what are reasons for admission in bronchiolitis?

A

<3 months, pre-existing conditions
<50-70% of normal milk intake
clinical dehydration
RR >70
O2 <92%
resp distress
apnoea
parent not able to manage/access medical help at home

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39
Q

what is the management of bronchiolitis?

A

ensure adequate intake - NG, IVs
saline nasal drops, nasal suctioning
O2
ventilation support

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40
Q

2 signs of poor ventilation of a cap gas?

A

rising pCO2
falling pH

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41
Q

what can be given as prevention of RSV infection?

A

Palivizumab

monthly injection as prevention of bronchiolitis caused by RSV given to high risk babies. Provides passive protection

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42
Q

what age does laryngomalacia occur at?

A

infants peaking at 6 months - inspiratory stridor exacerbated by lying, feeding, upset => no coinciding resp distress

problem resolves as larynx matures - around 1.5 years

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43
Q

what chromosome is CF carried on?

A

chromosome 7

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44
Q

mutations on what gene causes CF?

A

Cystic fibrosis transmembrane conductance regulatory gene

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45
Q

what channels are affected in CF?

A

chloride channels

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46
Q

what is often the first sign of CF?

A

meconium ileus - not passing meconium in 24 hours, abdo distention, vomiting

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47
Q

what are 7 causes of clubbing in children?

A

hereditary
cyanotic heart disease
infective endocarditis
CF
TB
IBD
Liver cirhosis

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48
Q

what is the diagnostic chloride concentration on sweat test for CF?

A

> 60 mmol/L

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49
Q

which are 2 bacteria which are especially difficult to treat in CF?

A

pseudomonas aeruginosa
Burkholderia cepacia

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50
Q

what Abx can be used to treat pseudomonas?

A

tobramycin nebs or oral ciprofloxacin

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51
Q

what is the management of CF?

A

multidisciplinary

chest physio
exercise
high calorie diet
CREON tablets - for pancreatic insufficiency
prophylactic flucloxacillin
Tx chest infections
bronchodilators
DNase nebs - help clear secretions
hypertonic saline nebs
vaccinations

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52
Q

what is the average life expectancy for CF?

A

47 years

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53
Q

what infection typically causes epiglottitis?

A

HiB

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54
Q

what are 8 symptoms of epiglottitis?

A

sore throat/pain swallowing
stridor
drooling
tripod position
high fever
muffled voice
scared and quiet
septic and unwell

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55
Q

what investigation can be done in suspected epiglottitis?

A

lateral xray of neck

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56
Q

what is the management of epiglottitis?

A

alert senior paediatrician and anaesthetist

ABx - cefriaxone
steroids - dexamethasone

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57
Q

what is a complication of epiglottitis?

A

epiglottic abscess

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58
Q

what is chronic lung disease of prematurity (bronchopulmonary dysplasia)?

A

typically <28 weeks gestation suffer from resp distress syndrome and require O2 or intubation and ventilation at birth. Diagnosed on CXR when infant required O2 >36 weeks gestational age

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59
Q

what are 5 features of chronic lung disease of prematurity?

A

low O2 sats
increased work of breathing
poor feeding and weight gain
crackles and wheeze
increased susceptibility to infection

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60
Q

what can reduce risk of chronic lung disease of prematurity?

A

corticosteroids (betamethasone) in premature labour mothers <36 weeks
CPAP rather than intubation
Caffeine to stimulate resp effort
avoid over-oxygenating

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61
Q

what is the most common pathogen of otitis media?

A

strep pneumoniae

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62
Q

what is the typical presentation of otitis media?

A

ear pain
reduced hearing
symptoms of URTI

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63
Q

what is the first line Abx for otitis media?

A

1 - amoxicillin 5 days

erythro, clarithro

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64
Q

what is glue ear?

A

otitis media with effusions - middle ear become full of fluid due to a blockage in the eustacion tube causing loss of hearing

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65
Q

what can be seen on otoscopy in glue ear?

A

dull tympanic membrane with air bubbles or a visible fluid level

can look normal

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66
Q

what is the natural course of glue ear?

A

usually resolves within 3 months without treatment

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67
Q

how long does it usually take grommets to fall out?

A

1 year

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68
Q

what are 3 common congenital causes of deafness?

A

maternal rubella or CMV
genetic deafness
associated syndromes - downs

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69
Q

what are 2 common perinatal causes of deafness?

A

prematurity
hypoxia during or after birth

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70
Q

what are 4 common post natal causes of deafness?

A

jaundice
meningitis and encephalitis
otitis media or glue ear
chemo

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71
Q

what is the range of normal hearing on an audiogram?

A

all readings between 0-20 dB

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72
Q

what are 3 symptoms additional to tonsillitis symptoms that can indicate quinsy?

A

trismus - unable to open mouth
change in voice (hot potato voice)
swelling and erythema surrounding tonsils

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73
Q

what is the most common pathogen in quinsy?

A

strep pyogenes (A) and Haemophilus influenzae

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74
Q

what is the management of quinsy?

A

drainage and Abx (co-amox)
?dex

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75
Q

what is preorbital cellulitis?

A

infection of upper and lower eyelid which causes red how skin swelling around eyelid and eye - distinguish from orbital cellulitis with CT scan

usually no pain or reduction in eye movements, no change in vision or abnormal pupillary response

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76
Q

what is the management of preorbital cellulitis?

A

systemic Abx

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77
Q

what is squint also known as?

A

strabismus - misalignment of the eyes causing double vision

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78
Q

what are 5 causes of squint?

A

idiopathic
hydrocephalus
cerebral palsy
space occupying lesion
trauma

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79
Q

what are 2 tests for squint?

A

cover test
herschberg’s test

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80
Q

what is the management of squint?

A

occlusive patch or atropine drops in good eye

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81
Q

what are the 3 foetal circulation shunts?

A

ductus venosus
foramen ovalae
ductus arteriosus

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82
Q

what is the ductus venosus?

A

shunt connecting umbilical vein to inferior vena cava allowing foetal blood to bypass liver

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83
Q

what is the foramen ovale?

A

shunt connecting R to L atrium which allows blood to bypass R ventricle and pulmonary circulation

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84
Q

what is the ductus arteriosus?

A

shunt connecting pulmonary artery and aorta which allows blood to bypass the pulmonary circulation

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85
Q

are atrial septal defects cyanotic?

A

no - unless R sided pressure becomes so great due to pulmonary hypertension the shunt reverses - EISENMENGER SYNDROME

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86
Q

what is eisenmenger syndrome?

A

when a shunt reverses due to pulmonary HTN causing R sided pressure > L sided pressure leading to pulmonary circulation bypass and cyanosis. develops after 1-2 years with large shunts and in adulthood with small

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87
Q

what are 4 complications of atrial septal defects?

A

stroke - with DVT - clot bypasses lungs
AF or atrial flutter
Pulmonary hypertension and r sided HF
Eismenger syndrome

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88
Q

what are 3 types of atrial septal defect?

A

Ostium secondum
patent foramen ovale
ostium primum - tend to causes Av valve defects also

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89
Q

what is the murmur in ASD?

A

mid systolic crescendo-decrescendo murmur loudest at upper left sternal border with a fixed split second heart sound

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90
Q

what are 5 typical ASD symptoms?

A

SOB
difficulty feeding
poor eight gain
LRTIs
complications - heart failure, stroke

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91
Q

what can be seen on examination in eisenmengers syndrome?

A

cyanosis
clubbing
dyspnoea
plethoric complection (due to polycythaemia)

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92
Q

what is the management of eisenmengers syndrome?

A

Heart lung transplant

Management of pulmonary HTN, polycythaemia and thrombosis

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93
Q

what genetic conditions are associated with VSDs?

A

Downs and turners

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94
Q

what 3 defects can cause eisenmenger’s syndrome?

A

ASD
VSD
patent duct arteriosus

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95
Q

what is the murmur is VSD?

A

pan-systolic more prominent at left lower sternal border in 3/4 intercostal space. May be systolic thrill

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96
Q

what are the 3 causes of pan-systolic murmur?

A

VSD
mitral regurg
tricuspid regurg

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97
Q

how long does it usually take for the ductus arteriosus to close?

A

1-3 days to stop functioning, 2-3 weeks to fully close

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98
Q

what kind of shunt is patent duct arteriosus?

A

left to right - from aorta to pulmonary vessels

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99
Q

what murmur is heard in patent duct arteriosus?

A

continuous crescendo -decrescendo machinery murmur

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100
Q

what causes an ejection systolic murmur? (3)

A

aortic stenosis
pulmonary stenosis
hypertrophic obstructive cardiomyopathy

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101
Q

what 2 conditions that cause cyanotic heart disease?

A

transposition of the great arteries
Tetralogy of fallot

Eisenmengers transformation

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102
Q

what condition is coarctation of the aorta associated with?

A

turners syndrome

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103
Q

what is the presentation of coarctation of the aorta?

A

weak femoral pulses - upper limb BP higher than lower limb
Systolic murmur below left clavicle
tachypnoea
poor feeding
grey, floppy baby

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104
Q

what is the management of coarctation of the aorta?

A

surgery
in high risk - prostaglandin E to keep ductus arteriosus open till surgery

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105
Q

what cardiac condition causes cyanosis at birth?

A

transposition of the great arteries

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106
Q

what is the management of transposition of the great arteries?

A

prostaglandins - maintain ductus arteriosus
Balloon septostomy - insert balloon catheter into foramen ovale to create largeASD
open heart surgery - arterial switch

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107
Q

what are the 4 pathologies in tetralogy of fallot?

A

Pulmonary valve stenosis
Right ventricular hypertrophy
Overriding Aorta
VSD

PROVe

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108
Q

what kind of shunt is there in tetralogy of fallot?

A

left to right - cyanotic

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109
Q

what are 4 risk factors for tetralogy of fallot?

A

rubella infection
increased age of mother
alcohol in pregnancy
diabetic mother

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110
Q

what investigations can be used for septal defects?

A

echo + doppler flow studies

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111
Q

what can be seen on chest x-ray in tetralogy of fallot?

A

boot shaped heart due to right ventricular hypertrophy

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112
Q

what are 6 manifestations of tetralogy of fallot?

A

cyanosis
clubbing
poor feeding
poor weight gain
ejection systolic murmur loudest in pulmonary area
tet spells

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113
Q

what are tet spells?

A

seen in tetralogy of fallot where shunt becomes temporarily worse causing cyanotic episode - usually during waking, exertion, crying

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114
Q

what is the management of tet spells?

A

squatting or pulling knees to chest - increases systemic vascular resistance

O2
beta blockers - relax R ventricle
IV fluids
morphine - decrease resp drive
sodium bicarb - buffer metabolic acidosis
phenylephrine infusion - increase systemic vascular resistance

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115
Q

what is the management of tetralogy of fallot?

A

Prostaglandins - maintain ductus arteriosus

surgery

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116
Q

what is ebstein’s anomaly?

A

R tricuspid valve lower than normal causing R ventricle to be smaller causing poor flow to pulmonary vessels - usually presents after closing of ductus arteriosus

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117
Q

what 4 conditions are associated with pulmonary valve stenosis?

A

tetralogy of fallot
william syndrome
noonan syndrome and turners syndrome
congenital rubella syndrome

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118
Q

what is rheumatic fever?

A

autoimmune condition triggered by antibodies to group A beta-haemolytic strep - typically strep pyogenes causing tonsilitis. The process usually occurs 2-4 weeks after initial infection

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119
Q

what is the presentation of rheumatic fever? (7)

A

fever
joint pain - migratory of large joints
erythema marginatum rash
shortness of breath
chorea
firm painless nodules
carditis - tachy/brady, murmurs, pericardial rub, heart failure

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120
Q

what are 3 investigations for rheumatic fever?

A

throat swab and culture
Anti streptococcal antibodies (ASO) titres
ECHO, ECG, CXR

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121
Q

what criteria is used to diagnose rheumatic fever?

A

jones criteria

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122
Q

what is the jones criteria?

A

for diagnosing rheumatic fever

TWO of JONES
Joint arthritis
Organ inflammation (carditis)
Nodules
Erythema marginatum rash
Sydenham chorea

OR
One JONES and TWO FEAR
Fever
ECG changes (prolonged PR)
Arthralgia
Raised inflammatory markers

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123
Q

what is the management of rheumatic fever?

A

Abx - Benzathine benzylpenicilin or phenoxymethylpenicillin
NSAIDs or salicates (joint pain)
Aspirin and steroids - carditis
Heartfailure - Diuretics +/- ACEi
Chorea - carbamazepine

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124
Q

what are 3 complications of rheumatic fever?

A

recurrence
valvular heart disease - mitral stenosis
chronic heart failure

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125
Q

what are 6 signs of problematic reflux in babies?

A

chronic cough
hoarse cry
distress after feeding
reluctance to feed
pneumonia
poor weight gain

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126
Q

what advice can be given for GORD in babies?

A

small frequent meals
burping regularly
not overfeeding
keep baby upright after feeding

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127
Q

what kind of vomiting is present in GORD?

A

effortless mainly after feeding

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128
Q

what 3 treatments can be given in babies with GORD?

A

gaviscon mixed with feeds
thickened milk or anti-reflux formula
PPIs

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129
Q

what is a key feature of pyloric stenosis?

A

projectile vomiting

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130
Q

when does pyloric stenosis present?

A

first few weeks (4-6 weeks) of life baby failing to thrive with projectile vomiting

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131
Q

what 3 things can be found on examination of a baby with pyloric stenosis?

A

observation of stomach peristalsis
firm round ‘olive like’ mass in upper abdomen

hypochloric (low Cl-) metabolic alkalosis on blood gas

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132
Q

how is pyloric stenosis dianosed?

A

Abdo USS

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133
Q

what is the management of pyloric stenosis?

A

laparoscopic pyloromyotomy - Ramstedt’s operation

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134
Q

what are 2 common viral causes of gastroenteritits?

A

rotavirus
norovirus

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135
Q

which e.coli produces shiga toxin?

A

E.coli 0157

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136
Q

what does shiga toxin cause?

A

abdo cramps, bloody diarrhoea, vomiting - can lead to haemolytic uraemic syndrome

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137
Q

what is the most common cause of bacterial gastroenteritis?

A

campylobacter jejuni

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138
Q

where does campylobacter come from?

A

raw/improperly cooked poultry
untreated water
unpasteurised milk

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139
Q

what is the treatment of campylobacter?

A

azithromycin or ciprofloxacin

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140
Q

which bacteria causes gastroenteritis after food has not been refrigerated quickly (rice)?

A

bacillus cereus - diarrhoea resolving in 24 hours

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141
Q

what is the treatment of Giardiasis?

A

metronidazole

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142
Q

what are 4 possible complications of gastroenteritis?

A

lactose intolerance
IBS
reactive arthritis
GBS

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143
Q

what is toddlers diarrhoea?

A

chronic watery diarrhoea in well <5 year olds which can often be helped by changes in diet

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144
Q

what are the 4fs of toddler’s diets?

A

fat - shouldnt have low fat diet
Fluid - not sugary drinks, not too much water
Fruit + fruit juice - gut cannot absorb fructose or sorbitol easily
Fibre - 12-18g per day

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145
Q

what is persistent diarrhoea?

A

> 2 weeks

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146
Q

what is chronic diarrhoea?

A

> 4 weeks

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147
Q

what is encopresis?

A

foecal incontinence

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148
Q

what are 5 causes of faecal incontinence?

A

spina bifida
hirschprungs disease
cerebral palsy
overflow incontenence - stress, abuse
Constipation with overflow

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149
Q

what are 5 lifestyle factors that may contribute to constipation in children?

A

habitual not opening bowels
low fibre diet
poor fluid intake
sedentary lifestyle
psychosocial problems

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150
Q

what are 8 secondary causes of constipation in children?

A

hirschprungs disease
CF
Hypothyroid
Spinal cord lesions
sexual abuse
Intestinal obstruction
anal stenosis
cow milk intolerance

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151
Q

what is the first line laxative in children?

A

movicol

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152
Q

when is the peak incidence of apendicitis?

A

10-20 years

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153
Q

where is McBurney’s point?

A

one third of the way from the anterior superior iliac spine and the umbilicus

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154
Q

what is rovsing’s sign?

A

for apendicitis

when palpation in LIF causes pain in RIF

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155
Q

what are 6 classical features of apendicitis?

A

loss of appetite
nausea and vomiting
rovsing’s sign
guarding
rebound tenderness
percussion tenderness

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156
Q

what are 5 key differentials for apendicitis?

A

ectopic pregnancy
ovarian cysts
meckel’s diverticulum
mesenteric adenitis
appendix mass

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157
Q

what is meckel’s divertculum?

A

malformation of distal ileum present in 2% of population that can bleed, become inflamed, rupture or cause volvutus, intussusception or obstruction

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158
Q

what are 5 complications of apendicectomy?

A

bleeding, infection, pain, scars
damage to bowel or bladder
removal of normal appendix
anaesthetic risks
VTE

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159
Q

what are the 5 key features of Crohn’s? (mneumonic)

A

NESTS

No blood or mucus (less than UC)
Entire GI tract
Skip lesions
Terminal ileum most affected + Trans mural
Smoking risk factor

weight loss, strictures, fistulae

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160
Q

what are the 7 key features of UC? (mneumonic)

A

CLOSEUP

Continuous inflammation
Limited to colon and rectum
Only superficial mucosa
Smoking protective
Excrete blood and mucus
Use aminosalicylates
Primary sclerosing cholangitis

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161
Q

what are extra-intestinal manifetations of IBD?

A

finger clubbing
erythema nodosum
pyoderma gangrenosum
episcleritis and iritis
inflammatory arthritis
primary sclerosing cholangitis (UC)

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162
Q

what is one stool test that can be used for IBD but only in adults?

A

faecal calprotectin

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163
Q

what is the acute management of crohn’s?

A

1 - steroids (oral pred or IV hydrocortisone)

+ immunosuppression under specialist - azathioprine, mercaptopurine, methotrexate, infliximab

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164
Q

what is the long term management of crohn’s?

A

1 - azithioprine or mercaptopurine

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165
Q

what is the management of mild-moderate acute UC?

A

1 - aminosalcylates - mesalazine

2 - corticosteroids

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166
Q

what is the management of severe acute UC?

A

1 - IV corticosteroids (hydrocortisone)

2 - IV ciclosporin

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167
Q

what is the long term management of UC?

A

aminosalicylate - mesalazine

or azathioprine or meraptopurine

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168
Q

what is the name of surgery to remove the colon and rectum sometimes used in UC?

A

panproctocolectomy

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169
Q

what is the most common congenital abnormality of the small bowel?

A

meckel’s diverticulum

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170
Q

when do people usually present with meckel’s diverticulum?

A

<2 years

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171
Q

what are 2 antibodies in coeliac disease?

A

anti-tissue transglutaminase (anti-TTG)

anti-endomysial (anti-EMA)

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172
Q

What skin manifestation can be a sign of coeliac disease?

A

dermatitis herpetiformis - itchy blistering skin rash usually on abdomen

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173
Q

what are 3 neurological signs associated with celiac disease?

A

peripheral neuropathy
cerebellar ataxia
epilepsy

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174
Q

what are 2 findings on endoscopy in coeliac disease?

A

crypt hypertrophy
villous atrophy

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175
Q

what are 6 conditions associated with coeliac?

A

T1DM
thyroid disease
autoimmune hepatitis
primary billiary cirrhosis
primary sclerosing cholangitis
downs syndrome

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176
Q

what are 7 complications of untreated coeliac disease?

A

vitamin deficiency
anaemia
osteoporosis
ulcerative jenunitits
enteropathy associated t-cell lymphoma
non-hodgekin lymphoma
small bowl adenocarcinoma

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177
Q

what is faltering growth?

A

fall in weight across:
1+ centiles if birth weight below 9th
2+ centiles if birth weight between 9th-91st
3+ centiles if birth weight 91+ centiles

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178
Q

what are 5 causes of failure to thrive?

A

inadequate nutritional intake
difficulty feeding
malabsorption
increased energy requirements
inability to process nutrition

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179
Q

what are 5 causes of inadequate nutritional intake in children failing to thrive?

A

maternal malabsorption if breastfeeding
IDA
family or parental problems
neglect
availability of food

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180
Q

what are 4 causes of difficulty feeding?

A

poor suck (cerebral palsy)
cleft lip/palate
genetic conditions w/ abnormal face structure
pyloric stenosis

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181
Q

what are 5 causes of malabsorption in children failing to thrive?

A

CF
coeliac disease
cows milk intolerance
chronic diarrhoea
IBD

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182
Q

what are 4 causes of increased energy requirements in children failing to thrive?

A

Hyperthroidism
chronic disease - CF, CHD
malignancy
chronic infections - HIV, immunodeficiency

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183
Q

what are 2 causes of inability to process nutrients in children failing to thrive?

A

inborn errors of metabolism
T2DM

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184
Q

how do you calculate mid-parental height?

A

average of parents height

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185
Q

what is anthropometry?

A

in nutritional assessment - includes weight, height mid-upper arm circumference and skinfold thickness

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186
Q

what is marasmus?

A

severe malnutrition due to inadequate energy intake in all forms. More common <1 year olds and causes muscle wasting and loss of subcutaneous fat but no oedema

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187
Q

what is kwashiorkor?

A

Severe malnutrition due to protein deficiency but with adequate energy intake. Leads to oedema, ascites and enlarged liver with fatty infiltrates

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188
Q

what is Hirschprungs disease?

A

congenital condition where nerve cells of the myenteric (auerbach’s) plexus are absent causing a lack of peristalsis of the large bowel

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189
Q

what is the key pathopysiology of hirschprungs disease?

A

absence of parasympathetic ganglion cells in the colon

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190
Q

what are 4 syndrome that can be associated with hirschprung’s?

A

downs
neurofibromatosis
waardenburg syndrome
multiple endocrine neoplasia type II

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191
Q

what are 5 presentations of hirschprungs disease?

A

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

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192
Q

what is hirschsprung associated enterocolitis?

A

inflammation and obstruction of the intestines in 20% of neonates with hirschprungs presenting usually between 2-4 weeks with fever, abdo distension, diarrhoea (w/ blood usually) and features of sepsis

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193
Q

what is the management of hirschsprungs associated enterocolitis?

A

Abx
fluid resuscitation
decompression of obstruction

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194
Q

how is hirschsprungs disease diagnosed?

A

rectal biopsy + histology for absence of ganglionic cells

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195
Q

what is the management of hirschsprungs disease?

A

surgical resection of aganglionic bowel

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196
Q

what age group does intussusception occur most commonly in?

A

6 months - 2 years

more common in boys

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197
Q

what is intussusception?

A

when the bowel invaginates into itself causing bowel obstruction

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198
Q

what 5 things are risk factors for intussusception?

A

concurrant viral illness
henoch-schonlein purpura
CF
intestinal polyps
meckel diverticulum

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199
Q

what are 6 presentations of intussusception?

A

sever colicky abdo pain
pale, lethargic unwell child
redcurrant jelly stool
sausage shaped RUQ mass
vomiting
intestinal obstruction

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200
Q

what is the first line investigation for intussusception?

A

USS

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201
Q

what is the treatment for intussusception?

A

1 - therapeutic enema - contrast, water or air

2- surgery

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202
Q

what are 4 complications of intussusception?

A

obstruction
gangrenous bowel
perforation
death

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203
Q

what are 8 causes of bowel obstruction in children?

A

meconium ileus
hirschprungs
oesophageal atresia
duodenal atresia
intussusception
imperforate anus
malrotation of the intestines + volvulus
strangulated hernia

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204
Q

what can be seen on abdo xray in obstruction?

A

proximal dilated bowel loops
distal collapsed bowel loops
absence of air in rectum

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205
Q

when does biliary atresia present?

A

shortly after birth

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206
Q

what kind of bilirubin is high in biliary atresia?

A

conjugated bilirubin

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207
Q

what is the 1st line investigation for billiary atresia?

A

conjugated and unconjugated bilirubin

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208
Q

what is classed as persistent jaundice in babies?

A

> 14 days term babies
21 days premies

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209
Q

what is the management of billiary atresia?

A

surgery - kasai portoenterostomy

often require full liver transplant

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210
Q

what is henoch-schonlein purpura?

A

autoimmune condition usually triggered by URTI which causes inflammation of capilaries leading to purpuric rash typically on lower legs, joint pain and GI symptoms. Can also cause kidney damage

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211
Q

what are the 3 key features of Henoch-schonlein purpura?

A

purpuric rash on legs
joint pain
GI symptoms

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212
Q

what is an abdominal migraine?

A

severe central abdo pain >1 hour with normal examination
may also have nausea, vom, anorexia, pallor, headache, photophobia, aura

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213
Q

what is the acute management of abdo migraine?

A

paracetamol
ibuprofen
sumatriptan

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214
Q

what is the prophylaxis of abdo migraine?

A

1 - pizotifen - serotonin agonist

propanolol
cyproheptadine - antihistamine
flunarazine - Ca channel blocker

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215
Q

what is gastroschisis?

A

a birth defect causing abdominal organs to be located outside abdomen due to defect in abdominal wall

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216
Q

what are 2 examination findings in diaphragmatic hernia?

A

apex beat displaced to r side of chest
poor air entry in L chest

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217
Q

what is the management of diaphragmatic hernia?

A

NG tube suction to prevent intrathoracic bowel distention and surgical repair - high mortality due to underdeveloped lungs in utero

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218
Q

who are umbilical hernias more common in?

A

children of african descent

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219
Q

what are 2 classes of symptoms of cow milk protein allergy?

A

GI symptoms - bloating, wind, abdo pain, diarrhoea , vomiting

Allergic symptoms - hives, angio-oedema, cough/wheeze, sneezing, watery eyes, eczema, anaphylaxis

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220
Q

what formula can be used in cow milk protein allergy?

A

hydrolysed formulas or in severe cases elemental amino acid formulas

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221
Q

what is the difference between cow milk intolerance and allergy?

A

intolerance has GI symptoms but no allergic symptoms unlike allergy

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222
Q

what immunoglobulin causes rapid cows milk protein allergy?

A

IgE

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223
Q

what are choledochal cysts?

A

congenital cyst dilations of the billary tree more commonly affecting females which have a small risk of malignancy

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224
Q

what is the classic triad of choledochal cysts?

A

abdo pain
jaundice
abdo mass

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225
Q

what is neonatal hepatitis syndrome?

A

idiopathic prolonged neonatal jaundice and hepatic inflammation which may cause low birth weight and faltering growth

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226
Q

what is the usual presentation of colic?

A

sudden inconsolable crying/screaming accompanied by drawing knees to chest and passign excessive gas

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227
Q

what 2 findings indicate pyelonephritis over uti?

A

temp >38 degrees
loin pain/tenderness

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228
Q

what is the management for < 3 months with fever?

A

immediate IV Abx - cefotaxime+ amoxicillin

+ full septic screen

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229
Q

what a full septic screen in <3 months?

A

bloods - FBC, CRP, cultures, lactate
urine dip
CXR
LP
? cap gas

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230
Q

when should children with UTIs get an USS within 6 weeks?

A

if first UTI <6 months
recurrent UTIs
atypical utis

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231
Q

what investigation can be done for defects in renal tissue and scars after UTI?

A

DMSA scan (dimercaptosuccinic acid) - injection of radioactive material using gamma camera done 4-6 months after infection

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232
Q

what investigation can be done to visualise urinary anatomy, vesicouteric reflux and urethral obstruction in children <6 months?

A

micturating cystourethrogram (MCUG)
- catheterise child and inject contrast then take x rays

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233
Q

what is vesico-uteric reflux?

A

where there is a developmental abnormality of the vesicouteric junction where the ureters are displaced laterally and enter the bladder directly allowing urine to reflux back up ureters and to the kidney causing dilatation and increased risk of infection and scaring

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234
Q

what is the management of pyelonephritis in children >3 months?

A

oral trimethoprim 7 days or IV co-amox 2-4 days

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235
Q

what is the management of UTI in children >3 months?

A

oral ABx - trimeth or nitro 3 days

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236
Q

what is enuresis?

A

involuntary urination

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237
Q

by what age do children usually get control of day time bladder function?

A

2 years

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238
Q

by what age do children usually stop bed wetting?

A

3-4 years

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239
Q

what are 5 possible causes of primary nocurnal enuresis?

A

overactive bladder - frequent small volume urination prevents development of bladder capacity
fluid intake prior to bed
failure to wake
psychological distress
secondary causes - chronic constipation, UTI, learning disability, cerebral palsy

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240
Q

what is primary nocturnal enuresis?

A

wetting the bed having never stopped

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241
Q

what is secodnary nocturnal enuresis?

A

wetting the bed having previously stopped for >6 months

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242
Q

what are 5 causes of secondary nocturnal enuresis?

A

UTI
constipation
T1DM
psychosocial problems
abuse

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243
Q

what is diurnal enuresis?

A

day time wetting self

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244
Q

what are 3 management options for nocturnal enuresis?

A

Eneurisis alarm - 1st line
desmopressin (taken at bedtime)
oxybutinin - anticholinergic
imipramine - tricyclic antidepressant

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245
Q

what age range is nephrotic syndrome most common in?

A

2-5 years

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246
Q

what is the classical triad of nephrotic syndrome?

A

low serum albumin
high urine protein (3+ or >3 grams)
oedema

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247
Q

what are 6 signs of nephrotic syndrome?

A

low serum albumin
high urine protein
oedema
deranged lipid profile
high BP
hyper-coagulability

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248
Q

what is the most common cause of nephrotic syndrome in children?

A

minimal change disease

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249
Q

what are 4 secondary causes of nephrotic syndromes?

A

intrinsic kidney disease - focal segmental glomerulosclerosis, mebranoproliferative glomerulonephitis

Henoch schonlein purpura
diabetes
infection - HIV, hepatitis, malaria

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250
Q

what can be seen on urinalysis in minimal change?

A

small molecular weight proteins and hyaline casts

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251
Q

what is the first line management of minimal change disease?

A

high dose corticosteroids - prednisolone

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252
Q

what is the management of steroid resistant nephrotic syndrome?

A

ACEi
immunosupressants - cyclosporine, tacrolimus, rituximab

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253
Q

what is the general management of nephrotic syndromes (5)?

A

high dose steroids - pred
low salt diet
diuretics
albumin infusion
antibiotic prophylaxis

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254
Q

what are 5 complications of nephrotic syndromes?

A

hypovolaemia and low BP
thrombosis
infection - kidneys leak immunoglobulins
acute or chronic renal failure
relapse

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255
Q

what is the pathophysiology of nephrotic syndromes?

A

the basement membrane of the glomerulus becomes highly permeable to proteins allowing them to leak from the blood into the urine

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256
Q

what is the pathophysiology of nephritic syndromes?

A

inflammation of the nephrons

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257
Q

what are 3 consequences of nephritic syndromes?

A

reduction in kidney function
haematuria
proteinuria (less than nephrotic)

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258
Q

what are the two most common causes of nephritis in children?

A

post-streptococcal glomerulonephritis
IgA nephropathy

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259
Q

what is the pathophysiology of post-streptococcal glomerulonephritis?

A

1-3 weeks after b-haemolytic strep (strep pyogenes) infection e.g tonsilitis immune complexes get stuck in the glomeruli and cause inflammation leading to AKI

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260
Q

what 2 investigations can be done to test for strep in post-strep glomerulonephritis?

A

positive throat swab
anti-streptolysin antibody titres

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261
Q

what is the management of nephritis?

A

supportive mainly

manage complications - IgA may need immunosupression

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262
Q

what is IgA nephropathy a complication of?

A

Henoch-schonlein purpura (IgA vasculitis)

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263
Q

what is the pathophysiology of IgA nephropathy?

A

IgA deposits in nephrons cause inflammation

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264
Q

what 2 things can be seen on biopsy in IgA nephropathy?

A

IgA deposits
Glomerular mesangial proliferation

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265
Q

what age group is usually affected with IgA nephropathy?

A

teens and young adults

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266
Q

what usually triggers haemolytic uraemic syndrome?

A

shinga toxin (from e.coli 0157 or shingella)

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267
Q

what is the classical triad of haemolytic uraemic syndrome?

A

haemolytic anaemia
AKI
thrombocytopenia

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268
Q

what increases the risk of haemolytic uraemic syndrome?

A

use of antibiotics and loperamide to treat gastroenteritis caused by e.coli0157 or shingella

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269
Q

how long after onset of gastroenteritis do symptoms of haemolytic uraemic syndrome usually start?

A

5 days

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270
Q

what are 8 presentations of haemolytic uraemic syndrome?

A

reduced urine output
haematuria or dark brown urine
abdo pain
lethargy and irritablility
confusion
oedema
HTN
bruising

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271
Q

what is the management of haemolytic uraemic syndrome?

A

supportive - renal dialysis, antihypertensives, maintain fluid balance, blood transfusion

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272
Q

what is hypospadias?

A

condition where uretheral meatus is displaced to underside of penis

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273
Q

what is epispadias?

A

condition where the urethral meatus is displaced to the top side of the penis

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274
Q

what is chordee?

A

where head of penis is bent downwards

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275
Q

what are 3 complications of hypospadias?

A

difficulty directing urination
cosmetic and psychological concerns
sexual dysfunction

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276
Q

what is phemosis?

A

pathological non-retration of foreskin

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277
Q

what condition is the most common cause of phimosis?

A

balantitis xerotica obliterans

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278
Q

what reduction in renal function classes as AKI?

A

<0.5 ml/Kg/hour over 6 hours

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279
Q

what category of cause is most common in childhood AKI?

A

pre-renal

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280
Q

what are the 2 most common causes of intra-renal failure in children?

A

haemolytic uraemic syndrome
acute tubular necrosis

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281
Q

what is stage 1 ckd?

A

eGFR >90 ml/min per 1.73 m2

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282
Q

what is stage 2 ckd?

A

eGFR 60-89

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283
Q

what is stage 3 ckd?

A

eGFR 30-59

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284
Q

what is stage 4 eGFR?

A

eGFR 15-29

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285
Q

what is stage 5 ckd?

A

eGGR <15 ml/min per 1.73m2

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286
Q

what 8 presenting features of severe CKD?

A

anorexia and lethargy
polydipsia and polyuria
faltering growth
bony deformities from renal rickets
hypertension
acute on chronic renal failure
proteinuria
normochomic normocytic anaemia

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287
Q

what type of hypersensitivity reaction in anaphylaxis?

A

type 1

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288
Q

what immunoglobulin causes anaphylaxis?

A

IgE

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289
Q

what is the pathophysiology of anaphylaxis?

A

igE stimulates mast cells to rapidly release histamine in mast cell degranulation

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290
Q

what is the key feature of anaphylaxis vs non-anaphylactic allergy?

A

anaphylaxis causes compromise of airway, breathing or circulation

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291
Q

what are the 4 allergic symptoms?

A

urticaria
itching
angio-oedema
abdominal pain

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292
Q

what is the management of anaphylaxis?

A

ABCDE

IM adrenaline
Antihistamines - chlorphenamine or certirizine
steroids - hydrocortisone

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293
Q

what can be a complication of anaphylaxis?

A

biphasic reaction - second anaphylactic reaction after treatment

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294
Q

what investigation can be done for anaphylaxis?

A

serum mast cell tryptase - within 6 hours of event

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295
Q

what are 5 reasons someone with a non-anaphylactic allergic reaction may get an epipen?

A

asthma requiring ICS
poor access to medical tx
adolescents - higher risk
nut/sting allergies
significant co-morbidities

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296
Q

what is the skin sensitisation theory of allergy?

A

there is a break in the infants skin that allows allergens from the environment to react with the immune system but there is no contact with the allergen through the GI tract

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297
Q

what is the classification system for hypersensitivity reactions?

A

cooms and gell

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298
Q

what is a type 1 hypersensitivity reaction?

A

IgE antibodies trigger mast cells and basophils to degranulate causing an immediate reaction - food allergy reactions

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299
Q

what is a type 2 hypersensitivity reaction?

A

IgG and IgM mediated activating the complement system leading to direct damage of local cells - haemolytic disease of newborn, transfusion reactions

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300
Q

what is a type 3 hypersensitivity reaction?

A

immune complexes accumulate and cause damage to local tissues - autoimmune conditions

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301
Q

what is a type 4 hypersensitivity reaction?

A

cell mediated caused by T lymphocytes which are inappropriately activated causing inflammation and damage to local tissues - organ transplant rejection, contact dermatitis

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302
Q

what are 3 investigations for allergies?

A

skin prick test
RAST testing - measure of allergen specific IgE
food challenge - gold standard

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303
Q

what hypersensitivity reaction is allergic rhinits?

A

type 1 IgE

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304
Q

what is the presentation of allergic rhinitis?

A

runny, bocked, itchy nose
sneezing
itchy, red, swollen eyes

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305
Q

what are 3 non-sedating antihistamines?

A

certirizine
loratadine
fexofenadine

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306
Q

what are 2 sedating anti-histamines?

A

chlorpenamine
promethazine

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307
Q

what can be taken as prophylactic for allergic rhinits?

A

nasal corticosteroids - fluticasone, mometasone

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308
Q

what are 3 live vaccinations?

A

BCG
MMR
nasal flu vaccine

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309
Q

where is eczema usually found?

A

flexor surfaces

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310
Q

what are 2 thick emolients?

A

50;50 ointment
hydromol

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311
Q

what is the steroid cream ladder?

A

mild - hydrocortisone 0.5,1,2.5%
moderate - eumovate 0.05%
potent - betamethasone 0.1%
very potent - clobetasol propionate 0.05%

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312
Q

what is the most common bacterial infection in eczema?

A

s aureus

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313
Q

what abx can be used to treat bacterial infection of eczema?

A

flucloxacillin

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314
Q

what is eczema herpeticum?

A

viral skin infection caused by HSV-1 or varicella zoster

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315
Q

what is the presentation of eczema herpeticum?

A

eczema sufferer developed widespread painful vesicular rash with fever, lethargy, irritability, reduced oral intake and swollen lymph nodes

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316
Q

What is the management of eczema herpeticum?

A

aciclovir

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317
Q

what is stevens-johnson syndrome and toxic epidermal necrosis?

A

a disproportionate immune response causing epidermal necrosis resulting in blistering and shedding of top layer of skin - SJS <10% of body, TEN >10% of body

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318
Q

what are 5 medications that can cause stevens-johnson syndrome?

A

anti-epileptics(lamotrigine, phenotoin)
antibiotics
allopurinol
NSAIDs
Penicillin

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319
Q

what are 7 infections that can cause stevens-johnson syndrome?

A

herpes
HIV
Mumps
FLu
EBV
mycoplasma pneumonia
CMV

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320
Q

what is the natural history of stevens-johnson syndrome?

A

starts with fever, cough, sore throat, mouth, eyes and skin
then develop purple/red rash which blisters
the after a few days skin sheds leaving raw tissue underneath

can also affect internal organs

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321
Q

what is the management of stevens-johnson syndrome?

A

admit to derm/burns unit

steroids
immunoglobulins
immunosuppression

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322
Q

what are 3 complications of stevens-johnson syndrome?

A

secondary infection
permanent skin damage and scarring
visual complications with eye involvement

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323
Q

what are 6 triggers for acute urticaria?

A

allergies
contact with chemicals, latex or nettles
medications
viral infection
insect bites
rubbing skin

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324
Q

what are the 3 different types of chronic urticaria?

A

chronic idiopathic urticaria
chronic inducible urticaria
autoimmune urticaria

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325
Q

what are 6 triggers for chronic inducible urticaria?

A

sunlight
temperature change
exercise
strong emotions
hot/cold weather
pressure

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326
Q

what is the management of urticaria?

A

Antihistamines - fexofenadine

if problematic - anti-leukotrienes (montelukast), omalizumab, cyclosporin

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327
Q

what can cafe-au-lait spots be a sign of?

A

neurofibromatosis type 1 (if have 6+)

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328
Q

what 3 things can port wine marks rarely be a sign of?

A

sturge-weber syndrome
klippel trenaunay syndrome
macrocephaly-capillary malformation

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329
Q

what are mongolial blue spots?

A

blue/black macular discolouration at base of spine and on buttocks or thighs - fade over first few years of life

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330
Q

what is osteogenesis imperfecta?

A

genetic condition also known as brittle bone disease caused by mutation in formation of collagen

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331
Q

what are 8 features associated with osteogenesis imperfecta?

A

hypermobility
blue/grey sclera
triangular face
short stature
deafness from early adulthood
dental problems
bone deformities
joint and bone pain

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332
Q

what is the pathophysiology of rickets?

A

deficiency in vitamin D/calcium which results in defective bone mineralisation

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333
Q

what is the name of the genetic version of rickers?

A

hereditary hypophasphataemic rickets

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334
Q

what are 8 presentations of rickets?

A

lethargy
bone pain
swollen wrists
bone deformuty
poor growth
dental problems
muscle weakness
pathological or abnormal fractures

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335
Q

what are 5 bone deformities in rickets?

A

bowing of legs
knock knees
rachitic rosary - expanded ribs causing lumps on chest
craniotabes - soft skull, delayed closing
delayed teeth

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336
Q

what investigation is done for vitamin d deficiency?

A

serum 25-hydroxyvitamin D - <25nmol/L = deficiency

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337
Q

what are 5 investigations that can be done for rickets?

A

serum 25-hydroxyvitamin D
serum calcium
serum phosphate
serum alkaline phosphatase
parathyroid hormone

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338
Q

what is the most common cause of hip pain in children 3-10 years?

A

transient synovitis

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339
Q

what is transient synovitis often associated with?

A

preceeding viral upper resp tract infection

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340
Q

what age is septic arthitis most common in?

A

<4 years

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341
Q

what is teh most common causative organism of septic arthritis?

A

staph aureus

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342
Q

what are 4 investigations of the synovial fluid in septic arthritis?

A

gram staining
crystal microscopy
culture
antibiotic sensitivities

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343
Q

what is the most common causative organism of osteomyelitis?

A

s.aureus

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344
Q

what are 6 risk factors for osteomyelitis?

A

open fracture
orthopaedic surgery
immunocompromised
sickle cell
HIV
TB

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345
Q

what is perthes disease?

A

disruption of blood flow to femoral head causing avascular necrosis of the epiphysis of the femur

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346
Q

what age group does perthes disease occur most commonly in?

A

5-8 years - mostly in boys

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347
Q

what is slipped upper femoral eiphysis? (SUFE)

A

when head of femur displaces along growth plate

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348
Q

who is SUFE more common in?

A

boys aged 8-15
obese children

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349
Q

how do people with SUFE prefer to hold their hip?

A

in external rotation and have limited movement of hip

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350
Q

what is the management of SUFE?

A

surgery to correct femoral head position

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351
Q

what is osgood-schlatter disease?

A

inflammation of the tibial tuberosity where patella ligament inserts causing anterior knee pain in adolescents - usually unilateral but can be bilateral. causes hard lump at front of knee

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352
Q

what are 3 presentations of osgood-schlatter?

A

visible or palpabl hard tender lump at tibial tuberosity
pain in anterior knee
pain exacerbated by activity, kneeling or extension of knee

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353
Q

what is a complication of osgood-schlatters?

A

avulsion fracture - tibial tuberosity separated from tibia

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354
Q

what is the management of osgood-schlaters?

A

reduction in physical activity
ice
NSAIDs

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355
Q

what is developmental dysplasia of the hip?

A

structural abnormality n developmental of foetal bones leading to instability of te hips and tendency for sublaxation or dislocation

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356
Q

what are 7 risk factors for developmental dysplasia of the hip?

A

FHx 1st degree relative
breech presentation 36 weeks
multiple pregnancy
female
oligohydramnios
prematurity
macrosomnia

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357
Q

what are 5 findings that may suggest developmental dysplasia of the hip on neonatal examination?

A

different leg lengths
restricted hip abduction on one side
significant bilateral restriction in hip abduction
difference in knee level with flexed hips
clunking of hips

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358
Q

what are two special test for developmental dysplasia of the hio?

A

ortolani test
barlow test

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359
Q

what is the ortolani test?

A

for developmental dysplasia of the hip

hips and knees flexed, hips gently abducted and pushed from the back to see if they will dislocate anteriorly

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360
Q

what is barlow test?

A

for developmental dysplasia of the hip

hips and knees flexed and gentle pressure put on knees through femur to see if hips will dislocate posteriorly

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361
Q

what investigation can be done for developmetal dysplasia of the hips?

A

USS

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362
Q

what is the management for developmental dysplasia of the hips?

A

Pavlik harness (<6 months) for 6-8 weeks

surgery if harness fails or >6 months

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363
Q

what are the 5 subtypes of juvenile idiopathic arthritis?

A

systemic JIA
polyarticular JIA
Oligoarticular JIa
enthesitis related arthritis
juvenile psoriatic arthritis

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364
Q

what is systemic juvenile idiopathic arthritis also known as?

A

Still’s disease

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365
Q

what are 8 manifestations of systemic JIA?

A

subtle salmon pink rash
high swinging fevers
enlarged lymph nodes
weight loss
joint inflammation and pain
splenomegally
muscle pain
pleuritis and pericarditis

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366
Q

what is are 2 key complication of systemic JIA?

A

macrophage activation syndrome - severe inflammatory responce causing DIC, anaemia, thrombocytopenia, bleeding, non-blanching rash

chronic anterior uveitis

367
Q

what are 4 key non-infective causes of fever in children > 5 days?

A

kawasaki disease
still disease (systemic JIA)
rheumatic fever
leukaemia

368
Q

what is polyarticular JIA?

A

idiopathic inflammatory arthritis in 5+ joints - equivalent of RhA in adults though most children are seronegative

369
Q

what is oligoarticular JIA?

A

monoarthritis affecting <4 joints, classically associated with anterior uveitis. most common in gils under 6

370
Q

what is enthesitis related arthritis?

A

paediatric seronegative spondyloarthropathies. inflammatory arthritis + enthesitis (inflammation of tendon insertion points)

usually HLA-B27 +ve

371
Q

what are 5 signs of juvenile psoriatic arthritis?

A

places of psoriasis
nail piitting
onycholysis (seperation of nail bed)
dactylitis
enthesitis

372
Q

what is the management of juvenile idiopathic arthritis?

A

NSAIDs
steroids
DMARDs
Biologics - TNF inhibitors, infliximab etc

373
Q

what age group does kawasaki’s usually affect?

A

<5 years

374
Q

what is a key complication of kawasaki disease?

A

coronary artery aneurysm

375
Q

what is the pathophysiology of kawasaki disease?

A

systemic medium sized vessel vasculitis?

376
Q

what are 7 key features of kawasaki?

A

high (>39) fever >5 days
widespread erythematous maculopapular rash on trunk
Oedema of hands and feed preceeding desquamation
Strawberry tongue and cracked lips
cervical lymphadenopathy
bilateral conjunctivitis
arthritis

377
Q

what are 5 investigations for kawasaki?

A

FBC - anaemia, leukocytosis, thrombocytosis
LFTS - hypoalbuminaemia, elevated enzymes
raised inflammatory markers (particularly esr)
Urinaralysis - wt cells without infection
ECHO

378
Q

what happens in the acute phase of kawasakis?

A

most unwell for 1-2 weeks
Fever, rash, lymphadenopathy

379
Q

what happens in the subacute phase of kawasakis?

A

weeks 2-4
acute symptoms settle
desquamation, strawberry tongue and arthralgia
occur - there is risk of coronary artery aneurysms forming

380
Q

what happens in the covalescent stage of kawasakis?

A

week 2-4
remaining symptoms settle

381
Q

what is the management of kawasaki disease?

A

high dose aspirin - reduce risk of throbosis
IV Ig to reduce risk of coronary artery aneurysm

382
Q

what is Reye’s syndrome

A

a preceeding viral infection usually treated with ASPIRIN followed by acute encephalopathy and hepatic dysfunction in children and young people

383
Q

what follow up is needed with kawasakis?

A

ECG and ECHO

384
Q

what cells produce surfactant?

A

type II pneumocytes

385
Q

when does surfactant start to be produced?

A

between 24-34 weeks gestation

386
Q

what is required to keep the ductus arteriosus open?

A

prostaglandins

387
Q

what can extended hypoxia lead to during birth?

A

hypoxic-ischaemic encephalopathy => cerebral palsy

388
Q

what are 3 issues of neonatal resuscitation?

A

large SA to weight ratio - cold
born wet and loose heat rapidly
meconium aspiration

389
Q

what are 5 principles of neonatal resuscitation?

A

warm baby
calculate APGAR score
stimulate breathing
inflation breaths
chest compressions

390
Q

what can be used to manage hypoxic ischaemic encephalopathy?

A

therapeutic hypothermia

391
Q

when should you calculate the APGAR score?

A

1, 5 and 10 minutes during resus

392
Q

how do you stimulate breathing in neonatal resussitation?

A

dry vigorously with towel
place baby head in neutral
check for airway obstruction and consider aspiration if gasping or unable to breathe

393
Q

how should inflation breaths be given in neonates?

A

2 cycles of 5 inflation breaths
if no response 30s of ventilation breaths can be used
then chest compressions coordinated with ventilation breaths

394
Q

what should be used for inflation breaths in preterm vs term babies?

A

preterm - air and O2
term - just air

395
Q

when should you start chest compressions in a neonate?

A

if HR <60 bpm despite resus and inflation breaths

396
Q

what is the APGAR score?

A

for neonatal resus

Appearance (skin colour)
Pulse
Grimmace (to stimulation)
Activity (tone)
respiration

397
Q

what is the scoring for appearance in APGAR?

A

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

398
Q

what is the scoring for pulse in APGAR?

A

Absent = 0
<100 = 1
>100 = 2

399
Q

what is the scoring for grimmace in APGAR?

A

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

400
Q

what is the scoring for activity in APGAR?

A

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

401
Q

what is the scoring for respiration in APGAR?

A

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

402
Q

how long should delayed cord clamping be?

A

1 minute

403
Q

under what gestation can be affected by respiratory distress syndrome?

A

< 32 weeks

404
Q

what xray changes are seen in respiratory distress syndrome?

A

ground glass appearance

405
Q

what are 4 types of ventilatory support that may be needed by premature neonates?

A

intubation and ventilation
endotracheal surfactant
CPAP
supplementary oxygen

406
Q

what are 6 short term complications of respiratory distress syndrome?

A

penumothorax
infection
apnoea
intraventricular haemorrhage
pulmonary haemorrhage
necrotising enterocolitis

407
Q

what are 3 long term complications of respiratory distress syndrome?

A

chronic lung disease of prematurity
retinopathy of prematurity
neurological, hearing or visual impairment

408
Q

what are 4 causes of hypoxic ischaemic encephalopathy?

A

maternal sock
intrapartum haemorrhage
prolapsed cord
nucal cord

409
Q

what is the staging for hypoxic ischaemic encephalopathy called?

A

sarnat staging

410
Q

what is the presentation of mild hypoxic ischaemic encephalopathy?

A

poor feeding, generally irritable, hyper-alert

resolves within 24 hours
normal prognosis

411
Q

what is moderate hypoxic ischaemic encephalopathy?

A

poor feeding, lethargy, hypotonic, seizures

can take weeks to resolve
40% develop cerebral palsy

412
Q

what is severe hypoxic ischaemic encephalopathy?

A

reduced consciousness, apnoea, flaccid, reduced or absent reflexes
50% mortality
90% develop cerebral palsy

413
Q

what temperature is therapeutic hypothermia cooled to and for how long?

A

33-34 degrees for 72 hours

414
Q

when can physiological jaundice be present?

A

from 2-10 days of age

415
Q

what are 8 causes of increased production of bilirubin in neonates?

A

haemolytic disease of newborn
ABO incompatibility
Haemorrhage
intraventricular haemorrhage
cephalo-haemorrhage
polycythaemia
sepsis and DIC
G6PD deficiency

416
Q

what are 6 causes of decreased bilirubin clearance in neonates?

A

prematurity
breast milk jaundice
neonatal cholestasis
extrahepatic biliary atresia
endocrine disorders
gilbert syndrome

417
Q

when is jaundice always pathological?

A

in first 24 hours of life

can be sign of sepsis

418
Q

what is kernicterus?

A

brain damage due to high unconjugated bilirubin

419
Q

what causes haemolytic disease of the newborn?

A

rhesus incompatability

420
Q

what is prolonged neonatal jaundice?

A

14+ days in full term babies
21+ days in premature babies

421
Q

what test is done for autoimmune haemolysis?

A

direct coombs test

422
Q

what is used to monitor neonatal jaundice?

A

treatment threshold charts

423
Q

what is the usual treatment of neonatal jaundice?

A

phototherapy with blue light (450nm)

424
Q

what are 3 complications of kernictus?

A

cerebral palsy
learning disability
deafness

425
Q

what is the medical management of supraventricular tachycardia?

A

adenosine - after valsalva manoeuvres

426
Q

what is necrotising enterocolitis?

A

disorder affecting premature neonates where part of bowel becomes necrotic and can lead to perforation

427
Q

what are 5 risk factors for necrotising enterocilitis?

A

very low birth weight or v premature
formula feeds
resp distress and assisted ventilation
sepsis
patent ductus arteriosus and congenital heart disease

428
Q

what are 6 features of necrotising enterocolitis?

A

intolerance to feeds
vomiting green bile
distended tender abdomen
blood in stool
absent bowel sounds
generally unwell

429
Q

what investigation is good for diagnosing necrotising enterocolitis?

A

abdo Xray - supine and lateral

430
Q

what can be seen on xray with necrotising enterocolitis?

A

dilated bowel loops
bowel wall oedema
pneumatosis intestinalis - gas in bowel wall
pneumoperitoneum - free gas in peritoneum
gas in portal veins

431
Q

what is the management of necrotising enterocolitis?

A

nil by mouth
iv fluids
total parenteral nutrition
antibiotics
NG tube - to drain fluid and gas

SURGERY

432
Q

what are 8 complications of necrotising enterocolitis?

A

perforation and peritonitis
sepsis
death
strictures
abcess formation
recurrence
long term stoma
short bowel syndrome

433
Q

what are the torch congenital infections?

A

Toxoplasmosis
rubella
cytomegalavirus
herpes simplex
HIV

434
Q

at what gestation is the risk of congenital rubella highest?

A

< 3 months

435
Q

what are 3 features of congenital rubella?

A

Classic triad of - deafness, blindness (congenital cataracts) and congenital heart disease

also learning disability

436
Q

what are 6 features of congenital cytomegalovirus?

A

foetal growth restriction
microcephaly
hearing loss
vision loss
learning disability
seizures

437
Q

what is the classic triad of congenital toxoplasmosis gondii?

A

intracranial calcification
hydrocephalus
chorioretinitis (type of posterior uveitis)

438
Q

what are 6 manifestations of neonatal herpes?

A

external herpes lesions
liver involvement
encephalitis
sezures, tremmors, lethargy, irritability

439
Q

what are 5 complications of chicken pox in pregnancy?

A

foetal varicellar zoster syndrome
pneumonitis
hepatitis
encephalitis
severe neonatal infection

440
Q

what are 6 mamifestations of congenital varicella syndrome?

A

foetal growth restriction
microcephaly
hydrocephalus, learnign disability
scars and skin changes
limb hypoplasia
cataracts and chorioretinitis

441
Q

what is given to a baby immediatly after birth?

A

vitamin K IM injection

442
Q

what 9 conditions are tested for in the blood spot test?

A

sickle cell
CF
congenital hypothyroidism
phenylketonuria
medium chain acy-COA dehydrogenase deficiency
maple syrup urine disease
isovaleric acidaemia
glutaric aciduria type 1
homocystin

443
Q

at what age does the heel prick test happen?

A

5 days old

444
Q

how long does the heel prick take to come back?

A

6-8 weeks

445
Q

when is the NIPE examination done?

A

<72 hours of birth

446
Q

when is the NIPE repeated?

A

6-8 weeks by GP

447
Q

when does the ductus arteriosus close?

A

1-3 days

448
Q

how small should the difference in pre-ductal and post-ductal O2 saturations be in the NIPE?

A

<2%
pre-ductal - R hand
post ductal - feet

449
Q

what is the name of oedema of the scalp outside the periosteum due to trauma in birth which crosses suture lines?

A

caput succedaneum

450
Q

what is the name of a collection of blood between the skull and periosteum due to trauma in delivery which does not cross suture lines?

A

cephalohaematoma

451
Q

what is erbs palsy?

A

damage to C5/6 brachial plexus ileading to lweakness in shoulder abduction and external rotation, arm flexion and finger extension

may have a ‘waiters tip appearance’

452
Q

what are 6 risk factors for neonatal sepsis?

A

vaginal GBS colonisation
GBS sepsis in previous baby
Maternal sepsis, chorioamniotitis or fever
prematurity
premature ROM
prolonged rupture of membranes

453
Q

what are 6 red flags for neonatal sepsis?

A

confirmed/suspected sepsis in mother
signs of sock
seizures
term baby needing mechanical ventilation
resp distress starting 4+ hours after burth
presumed sepsis in other multiple

454
Q

what are 10 clinical features of neonatal sepsis?

A

fever
reduced tone and activity
poor feeding
resp distress or apnoea
vomiting
tachy or brady
hypoxia
jaundice <24 hours
seizures
hypoglycaemia

455
Q

what is the nice treatment for neonatal sepsis?

A

monitor if 1 risk factor/clinical feature

start antibiotics within one hour if 2 risk factors/clinical features or 1 red flag

456
Q

what antibiotic should be given in neonatal sepsis?

A

benzylpenicillin or gentamycin

cefotaxime if lower risk

457
Q

what is the ongoing management of neonatal sepsis?

A

check CRP at 24 hours and blood cultures at 36 hours

consider stopping Abx if blood cultures negative and CRP <10 at day 2

458
Q

what counts as extreme preterm?

A

<28 weeks

459
Q

what counts as very preterm?

A

28-32 weeks

460
Q

what counts as moderate/late preterm?

A

32-37 weeks

461
Q

what are 10 early complications of prematurity?

A

respiratory distress syndrome
hypothermia
hypoglycaemia
poor feeding
apnoea and bradycardia
neonatal jaundice
intraventricular haemorrhage
retinopathy of prematurity
necrotising enterocolitis
immature immune system and infection

462
Q

what are 5 long term complications of prematurity?

A

chronic lung disease of prematurity
learnign an dbehvioural difficulties
susceptibility to infections - particularly resp
hearing and visual impairement
cerebral palsy

463
Q

what is apnoea?

A

stopping breathing >20 seconds or shorter periods with oxygen desaturation or bradycardia

464
Q

what is the causes of apnoea in neonates?

A

due to immaturity of the autonomic nervous system

465
Q

what is the management of neonatal apnoea?

A

tactile stimulation
IV caffeine

466
Q

what is the pathophysiology of retinopathy of prematurity?

A

retinal blood vessel formation is stimulated by hypoxia which is the normal condition of the retina during pregnancy so with early exposure to oxygen in prematurity this can stop retinal blood vessel development and cause retinal detachment

467
Q

who should screening for retinopathy of prematurity be offered to?

A

<32 weeks gestation
<1.5kg

468
Q

what is the management of retinopathy of prematurity?

A

transpupillary laser photocoagulation

cyotherapy
injections of intravitreal VEGF inhibitors
Surgery if retinal detachment

469
Q

how is a diagnosis of meconium aspiration confirmed?

A

CXR

470
Q

what are 3 risk factors for meconium aspiration?

A

gestational age >42 weeks
foetal distress
APGAR score <7

471
Q

what is the management for suspected HSV encephalitis?

A

aciclovir

472
Q

How do you get infected with listeria?

A

consumption of dairy products, raw vegetables, meats and refrigerated foods

passed from mother to baby placentally

473
Q

what are clinical manifestations of neonatal listeriosis?

A

abortion
prematurity
still birth
neonatal sepsis

474
Q

how can listeria infection be diagnosed prenatally and postnatally?

A

culture or PCR of blood, cervix or amniotic fluid of mother prenatally

culture/PCR of blood, CSF, gastric aspirate, meconium of neonate

475
Q

what is the management of neonatal listeriosis?

A

Ampicillin +/- gentamicin

476
Q

what is maternal hyperthyroidism associated with?

A

foetal tachycardia
small for gestational age
prematurity
still birth
congenital malformations

477
Q

what is maternal hypothyroidism associated with?

A

lower IQ and impaired psychomotor development

478
Q

what are 6 risk factors for neonatal hypoglycaemia?

A

IUGR
preterm
born to mothers with diabetes
hypothermic
polycythaemic
ill for any reason

479
Q

what are 6 symptoms of neonatal hypoglycaemia?

A

jitters
irritability
apnoea
lethargy
drowsiness
seizures

480
Q

what counts as hypoglycaemia in neonates?

A

<2.6 mmol/L

481
Q

what is the pathophysiology of cleft lip and palate?

A

lip - failure of fusion of the frontonasal and maxillary processes
Palate - failure of fusion of palatine processes and nasal septum

482
Q

when is a cleft lip and palate usually fixed?

A

lip - 3 months
palate - 6 months - 1 year

483
Q

what are 2 USS signs in pregnancy of oesophageal atresia?

A

polyhydramnios
no stomach bubble

484
Q

what is a clinical sign of oesophageal atresia in neonates?

A

persistant salivation and drooling

485
Q

what is the name of a group of midline congenital defects like oesophageal atresia?

A

VACTERL association

Vertebral
anorectal
cardiac
tracheo-oesophageal
renal
limb

anomalies

486
Q

what is the management of oesophageal atresia?

A

continuous suction of secretions to avoid aspiration until surgery is available

487
Q

what is an exomphalos?

A

where abdominal contents protrude through umbilical ring covered by transparent sac

488
Q

what congenital condition is duodenal atresia associated with?

A

downs syndrome

489
Q

what is an x-ray/USS sign of duodenal atresia?

A

‘double bubble’ of of distention of stomach and duodenal cap with absence of air distally

490
Q

what are 3 obstetric complications of diabetes in pregnancy?

A

polyhydramnios
preeclampsia
early and late foetal loss

491
Q

what are 3 foetal complications of diabetes in pregnancy?

A

congenital malformations
IUGR
macrosomia

492
Q

what are 4 neonatal complications of diabetes in pregnancy?

A

neonatal hypoglycaemia
respiratory distress syndrome
hypertrophic cardiomyopathy
polycythaemia

493
Q

what is scoliosis?

A

lateral curvature of frontal plane of spine. In severe cases can lead to cardiorespiratory failure from curvature of chest

494
Q

what is torticollis?

A

Head twisting to the side also called wry neck. The most common cause in infants is a sternomastoid tumour.

495
Q

what is the incubation period for mumps?

A

14-25 days

496
Q

what is the presentation of mumps?

A

prodromal fever, muscle aches, lethargy, reduced appetite, headache and dry mouth for a few days

followed by parotid swelling

usually self limiting after around a week

497
Q

what are 4 complications of mumps?

A

pancreatitis
orchitis
meningitis
sensorineural hearing loss

498
Q

How is mumps diagnosed?

A

salivary PCR
salivary or blood antibodies

499
Q

what is the management for mumps?

A

supportive - self limiting infection

500
Q

what is the rash and its spread in chicken pox?

A

widespread erythematous raised vesicular blistering lesions - starts as macules then papules then vesicles
Starts on trunk or face and spreads outwards over 2-5 days the crusts after 5 days

501
Q

what are 5 complications of chicken pox?

A

bacterial superinfection (necrotising faciitis)
dehydration
conjunctival lesions
pneumonia
encephalitis (presents with ataxia)

502
Q

How long can it take someone to become symptomatic after exposure to chicken pox?

A

10 days - 3 weeks

503
Q

what is the presentation of measles?

A

fever, coryza and conjunctivitis
followed 2 days later by greyish white spots on the buccal mucosa - KOPLIK SPOTS
followed by rash 3-5 days post fever, classically starting behind ears
self limiting after 7-10 days

504
Q

what sign on examination is pathognomonic for measles?

A

Koplik spots - grey/white spots on buccal mucosa that appear 2 days after fever

505
Q

what is the rash like in measles and how does it spread?

A

starts on face (classically behind ears) 3-5 days after fever and then spreads to rest of body

erythematous macular rash with flat lesions

506
Q

what is the incubation period of measles?

A

10-12 days

507
Q

what are 8 complications of measles?

A

pneumonia
diarrhoea
dehydration
encephalitis
meningitis
hearing loss
vision loss
death

508
Q

what pathogen causes scarlet fever?

A

exotoxins from group a strep (strep pyogenes)

509
Q

what is the rash in scarlet fever and how does it spread?

A

red-pink blotchy macular rash with rough SANDPAPER skin
Starts on trunk and spreads outwards

may also have flushed cheeks

510
Q

what are 7 features of scarlet fever?

A

sandpaper rash
fever
lethargy
flushed face
sore throat
Strawberry tongue
cervical lymphadenopathy

511
Q

what is the management of scarlet fever?

A

phenoxymethylpenicillin (penicillin V) for 10 days

512
Q

what are 3 conditions associated with scarlet fever?

A

post-streptococcal glomerulonephritis
acute rheumatic fever
tonsillitis - strep throat

513
Q

how long is the incubation time for rubella?

A

2 weeks

514
Q

what is the rash in rubella and how does it spread?

A

Erythematous macular rash (milder than measles)
starts on face and spreads to body
lasts 3 days

515
Q

what are 2 rare complications of rubella?

A

thrombocytopenia
encephalitis

516
Q

what are 5 presenting features of rubella?

A

erythematous macular rash
mild fever
joint pain
sore throat
lymphadenopathy

517
Q

what pathogen causes slapped cheek syndrome?

A

Parvovirus b19

518
Q

what is the presentation of parvovirus B19?

A

fever, coryza and non-specific viral symptoms

followed 2-5 days later by diffuse bright red rash on both cheeks (as though slapped)
a few days later a reticular mildly erythematous affecting trunk and limbs - may be raised and itchy

519
Q

what are 4 complications of parvovirus?

A

aplastic anaemia
encephalitis or meningitis
pregnancy complications - foetal death
rare - hepatitis, myocarditis, nephritis

520
Q

what pathogen causes roseola infantum?

A

HHV-6/7

521
Q

what is the presentation of roseola infantum?

A

presents 1-2 weeks after high fever for 3-5 days which the settles and is followed by a rash

522
Q

what is the rash like in roseola infantum?

A

mild erythematous macular rash on arms, legs, trunk and face that isn’t itchy

523
Q

what pathogen causes whooping cough?

A

bordetella pertussis - gram neg

524
Q

what is the presentation of whooping cough?

A

mild coryzal symptoms and low grade fever, dry cough
followed by more severe coughing fits after 1 week so much so people may vomit, fait or have a pneumothorax

525
Q

what are 3 ways to diagnose whooping cough?

A

nasopharangeal PCR or culture
anti-pertussis toxin IgG if cough present >2 weeks

526
Q

what is the management of whooping cough?

A

1 - macrolides - azithromycin, erythromycin, clarithromycin within first 21 days

2 - Co-trimoxazole

527
Q

what is a complication of whooping cough?

A

bronchiectasis

528
Q

what can be given to vulnerable contacts of people with whooping cough?

A

prophylactic antibiotics

529
Q

what 2 pathogen cause impetigo?

A

staph aureus - most common
strep pyogenes

530
Q

what is the characteristic sign of impetigo?

A

golden crusted rash/skin infection

531
Q

what are the two different types of impetigo?

A

bullous - 1-2cm fluid filled vesicles which grow and burst to form golden crusts. may be itchy
non-bullous - golden crusts

532
Q

what pathogen always causes bullous impetigo?

A

staph aureus

533
Q

what is the management of non-bullous impetigo?

A

1st line - hydrogen peroxide cream 1%
2nd - If unsuitable - topical fusidic acid
3rd - oral flucloxacillin - when more widespread/unwell

534
Q

what is the name of severe bullous impetigo?

A

staphylococcus scalded skin syndrome

535
Q

what is the management of bullous impetigo?

A

abx - usually flucloxacillin

536
Q

what are 6 complications of impetigo?

A

cellulitis
sepsis
scarring
post-streptococcal glomerulonephritis
staphylococcus scalded skin syndrome
scarlet fever

537
Q

what bacteria causes diphtheria?

A

corynebacterium diphtheriae

538
Q

what are 5 features of diphtheria?

A

sore throat + difficulty swallowing
fever
lymphadenopathy
SOB
pseudomembrane on tonsils and mucosa of pharynx larynx and nose

539
Q

what is the management of diphtheria?

A

1 - diphtheria antitoxin
Abx - azithromycin, erythromycin, clarithromycin,

540
Q

what is the pathogenic cause of scalded skin syndrome?

A

staph aureus epidermolytic toxins

541
Q

what age group is usually affected by scalded skin syndrome?

A

<5 years

542
Q

what sign is positive in scalded skin syndrome?

A

Nikolysky sign - gentle rubbing of skin causes it to peel away

543
Q

what is a major acute complication of polio?

A

acute flaccid paralysis

544
Q

what is a possible long term complication of polio?

A

post-poliomyelitis syndrome - weakness and fatigue of muscle groups affected in acute illness

545
Q

what is the main cause of viral encephalitis in neonates?

A

HSV-2 - genital herpes

546
Q

what is the most common cause of viral encephalitis?

A

HSV-1 - from cold sores

547
Q

what is the treatment for encephalitis caused by CMV?

A

ganciclovir

548
Q

what are 4 complications of encephalitis?

A

change in personality, memory or cognition
leaning disability
headache
movement disorders

549
Q

what is the most common cause of toxic shock syndrome?

A

group A strep toxins

or s.aureus toxins

550
Q

what are 6 presenting features of toxic shock?

A

severe diffuse of localised pain in an extremity
fever
localised swelling or erythema
hypotension
diffuse, scarlatina-like red rash
muscle weakness

551
Q

what is the management of strep toxic shock?

A

clindamycin + penzylpenicillin or vancomycin

552
Q

what is the management for staph toxic shock?

A

clindamycin + oxacillin or vancomycin

553
Q

what are 4 complications of toxic shock?

A

bacteraemia
acute resp distress
DIC
renal failure

554
Q

what is the management for oral candidaiasis?

A

miconazole gel
nystatin suspension
fluconazole tablets

555
Q

what does cutaneous candida rash look like?

A

sore and itchy pustular or papular erythematous rash with an irregular edge and SATELLITE lesions

often in flexural areas

556
Q

what is the management of nappy rash?

A

1 - topical hydrocortisone 1% if inflamed

topical imidazoles (clotrimazole), oral antibiotics if severely inflammed

557
Q

what causes hand foot and mouth disease?

A

coxsackie a virus

558
Q

what is the incubation period for hand foot and mouth?

A

3-5 days

559
Q

what is the typical history for hand foot and mouth disease?

A

resp illness + fever
1-2 days later - mouth ulcers then red painful possibly itchy blistering spots across body

560
Q

what is the management for hand foot and mouth?

A

supportive - will resolve in 7-10 days without treatment

561
Q

what are 3 complications of hand foot and mouth disease?

A

dehydration
bacterial superinfection
encephalitis

562
Q

what kind of bacteria in Neisseria meningitidis?

A

gram negative diplococci

563
Q

what is the most common cause of meningitis in neonates?

A

group B strep

564
Q

what are 5 non-specific signs of meningitis in neonates?

A

hypotonia
poor feeding
lethargy
hypothermia
bulging fontanelle

565
Q

what is kernig’s test?

A

for meningitis

lie patient on back and flex one hip an dknee 90 degrees then straighten knee with hip still flexed - +ve if spinal pain and resistance to movement

566
Q

what is brudzinskis test?

A

for meningitis

lie patient on back and use hand to lift head and neck to flex chin to chest +ve if causes patient to flex hips and knees

567
Q

what is the treatment of sus bacterial meningitis in the community?

A

benzylpenicillin

568
Q

what is the management of sus bacterial meningitis in <3 months?

A

cefotaxime + amoxicilin (for listeria)

569
Q

what is the management for sus bacterial meningitis in >3 months?

A

ceftriaxone

+ dexomethasone QDS for 4 days

570
Q

what is the prophylaxis for contact with bacterial meningitis?

A

single dose ciprofloxacin

571
Q

what are 3 common causes of viral meningitis?

A

HSV
enterovirus
VZV

572
Q

where should an LP be performed?

A

L3/4

573
Q

what is the CSF picture in bacterial meningitis?

A

cloudy
high protein
Low glucose
high neutrophils
culture +ve

574
Q

what is the CSF picture in viral menigitis?

A

clear
mild raise/normal protein
normal glucose
high lymphocytes
-ve culture

575
Q

what are 5 complications of meningitis?

A

hearing loss
seizures
cognitive impairment
memory loss
cerebral palsy

576
Q

what are 7 causes of cerebral palsy?

A

antenatal - maternal infection, trauma in preggo
perinatal - birth asphyxia, pre-term birth
postnatal - meningitis, severe neonatal jaundice, head injury

577
Q

what are the 4 types of cerebral palsy?

A

spastic
dyskinetic
ataxic
mixed

578
Q

what is spastic cerebral palsy?

A

hypertonia and reduced function due to UMN damage

579
Q

what is dyskinetic cerebral palsy?

A

problems controlling muscle tone with hyper and hypotonia casing athetoid (slow writing) movements and oro-motor problems

due to basal ganglia damage

580
Q

what is ataxic cerebral palsy?

A

problems with coordinated movement due to cerebellar damage

581
Q

what are 6 signs of cerebral palsy?

A

failure to meet milestones
increased/decreased tone
hand preference <18 months
problems with coordination, speech or walking
feeding or swallowing problems
learning difficulties

582
Q

what are 6 complications of cerebral palsy?

A

learning difficulty
epilepsy
kyphoscoliosis
muscle contractures
hearing/visual impairement
GORD

583
Q

what is the management for cerebral palsy?

A

multidisciplinary

physio
OT
speech and language
dietician
orthopaedics

Meds - muscle relaxants - baclofen, antiepileptics, glycopyrronium bromide - drooling

584
Q

what age group do febrile convulsions occur in?

A

ages 6 months - 5 years

585
Q

what are simple febrile convulsions?

A

one generalised tonic clonic <15 minutes

586
Q

what are 5 possible causes of global developmental delay?

A

downs
fragile X
foetal alcohol syndrome
rett syndrome
Metabolic disorders

587
Q

what are 5 possible causes of gross motor delay?

A

cerebral palsy
ataxia
myopathy
spina bifida
visual impairement

588
Q

what are 5 possible causes of fine motor delay?

A

dyspraxia
cerebral palsy
muscular dystrophy
visual impairment
congenital ataxia

589
Q

what are 6 possible causes of language delay?

A

social circumstance
hearing impairement
learning disability
neglect
autism
cerebral palsy

590
Q

what are 3 possible causes of social delay?

A

emotional and social neglect
parenting issues
autism

591
Q

what are the 4 areas of development?

A

gross motor
fine motor and vision
language and hearing
personal and social

592
Q

what is the gross motor development of a 4 month old?

A

start supporting head and keep in line with body

593
Q

what is the gross motor development at 6 months?

A

Maintain sitting position usually supported as unbalanced

594
Q

what is the gross motor development at 15 months?

A

walk unaided

594
Q

what is the gross motor development at 9 months?

A

sit unsupported
start crawling
maintain standing and bouncing position wile supported

594
Q

what is the gross motor development at 12 months?

A

standing and cruising

594
Q

what is the gross motor developement at 18 months?

A

squat and pick things up

595
Q

what is the gross motor development at 2 years?

A

run and kick ball

596
Q

what is the gross motor development at 3 years?

A

climb stairs one foot at time
stand on one leg
ride tricycle

597
Q

what is the gross motor development of a 4 year old?

A

hop
climb stairs normally

598
Q

what are 8 red flags of developemental milestones?

A

lost development
unable to hold object 5 months
not sitting unsupported at 12 months
not standing independently 18 months
not walking 2 years
not running 2.5 years
no words 18 months
no interest in others 18 months

599
Q

what is social development at 6 weeks?

A

smiles

600
Q

what is social development at 3 months?

A

communicates pleasure

601
Q

what is social development at 6 months?

A

curious and engages with people

602
Q

what is social development at 9 months?

A

apprehensive around strangers

603
Q

what is social development at 12 months

A

engages with others
pointing and handing objects
waves bye
claps

604
Q

what is social development at 2 years?

A

interest in others beyond parents
parallel play

605
Q

what is social development by 3 years?

A

play with others
bowel control

606
Q

what is social development by 4 years?

A

has best friend
dry at night
dresses self
imaginative play

607
Q

what is language development at 3 months?

A

cooing

608
Q

what is language development at 9 months?

A

babbling

609
Q

what is language development at 12 months?

A

say 1 word in context
follows simple instructions

610
Q

what is language development at 18 months?

A

5-10 words

611
Q

what is language development at 2 years?

A

combines 2 words

612
Q

what is language development at 3 years?

A

basic 3 word sentences

613
Q

what is language development at 4 years?

A

tells stories

614
Q

what is fine motor development at 8 weeks?

A

fixes and attempts to follow
recognises faces

615
Q

what is fine motor development at 6 months?

A

palmar grasp

616
Q

what is fine motor development at 9 months?

A

scissor grasp between thumb and forefinger

617
Q

what is fine motor development at 12 months?

A

pincer grasp with tip of thumb and forefinger
scribbles randomly

618
Q

what is fine motor development at 14-18 months?

A

clumsily use cutlery
tower of 2-4 blocks

619
Q

what is the first and second line management for tonic-clonic seizures?

A

1 - sodim valporate
2 - lamotrigine or carbamezapine

620
Q

what is the 1st and 2nd line management for focal seizures?

A

1 - carbamexapine or lamotragine
2 - sodium valporate or levetiracetam (keppra)

621
Q

where do focal seizures start?

A

temporal lobes

622
Q

what is the first line management for absence seizures?

A

ethosuximide

623
Q

what are atonic seizures?

A

drop attacks with breif lapses of muscle tone usually lasting <3 mins

may be indicative of lennox-gastaut syndrome

624
Q

what is the 1st and 2nd line management of atonic seizures?

A

1 - sodium valporate
2 - lamotrigine

625
Q

what is the management of myoclonic seizues?

A

1 - sodium valporate

others - lamotrigine, levetiracetam or topiramate

626
Q

when do infantile spasms usually start?

A

around 6 months

627
Q

what is the prognosis for infantile spasms?

A

1/3rd die by 25
1/3rd keep seizures
1/3rd seizure free

628
Q

what are 2 treatment options for infantile spasms?

A

pred
vigabitrin

629
Q

what are 4 side effects of sodium valporate?

A

teratogenic
liver damage and hepatitis
hair loss
tremor

630
Q

what are 3 side effects of carbamazepine?

A

agranulocytosis
aplastic anaemia
P450 drug interactions

631
Q

what are 3 side effects of phenytoin?

A

folate and vitamin d deficiency
megaloblastic anaemia
osteomalacia

632
Q

what are 2 side effects of ethosuximide?

A

night terrors
rashes

633
Q

what are 2 side effects of lamotrigine?

A

stevens-johnson syndrome
leukopenia

634
Q

what is status epilepticus?

A

seizure lasting >5 mins
2 or more seizures without regaining consciousness

635
Q

how is status epilepticus managed?

A

IV lorazepam
buccal midazolam
rectal diazepam

IV phenytoin can be used if seizure persists

636
Q

what chromosome is affected in sickle cell anaemia?

A

chromosome 11

637
Q

what are 10 complications of sickle cell?

A

anaemia
increased risk of infection
chronic kidney disease
sickle cell crisis
acute chest syndrome
stroke
avascular necrosis
pulmonary hypertension
gallstones
priaprism (painful persistent erections)

638
Q

what are 4 things that can trigger sickle cell crisis?

A

dehydration
infection
stress
cold weather

639
Q

what is the management of sickle cell crisis?

A

low threshold for admission
treat infections
keep warm
good hydration
analgesia

640
Q

what is vaso-occlusive crisis?

A

sickle shaped RBCs clog capillaries causing distal ischaemia

presents with pain and swelling in hands, feet, chest or back

can cause priapism in men

641
Q

what is splenic sequestration crisis?

A

blood flow to spleen blocked by sickle cells causing acutely enlarged painful spleen which can lead to severe anaemia and hypovolaemic shock

management - supportive, blood transfusion, fluid resus

642
Q

what is an aplastic crisis?

A

complication of sickle cell temporary absence of creation of RBCs
usually triggered by parvovirus B19

643
Q

what is acute chest syndrome?

A

vaso-oclussive crisis due to sickle cells of the blood vessels of the lungs causing fever, SOB, chest pain, cough and hypoxia

644
Q

what is the management of acute chest syndrome?

A

analgesia
good hydration
Abx
blood transfusion for anaemia
incentive spirometry
resp support

645
Q

what is the management for sickle cell?

A

avoid triggers
vaccines
Abx prophylaxis (penicillin V)
hydroxycarbamine
crizanluzumab
blood transfusion
bone marrow transplant

646
Q

what medication can be used in sickle cell to stimulate foetal haemoglobin production?

A

hydroxycarbamide

647
Q

what are 8 signs of thallasaemia?

A

microcytic anaemia
splenomegally
pronounced forehead and malar eminences
jaundice
gallstones
poor growth and development
fatigue
pallor

648
Q

what investigation can be used to detect thallasaemia?

A

haemoglobin electrophoresis

649
Q

what are the two types of thalassaemia?

A

alpha or beta

650
Q

what is thalassaemia minor?

A

carriers with one faulty gene

causes mild microcytic anaemia usually only requires monitoring

651
Q

what is thalassamia intermedia?

A

either two defective geners or one and one deletion

causes more significant microcytic anaemia
patients require monitoring and blood transfusions which may require iron chelation

652
Q

what is thalassaemia major?

A

homozygous for the deletion genes

severe anaemia and failure to thrive in early childhood

severe microcytic anaemia
splenomegaly
bone deformities

regular transfusions, iron chelation, splenectomy

653
Q

what is klinefelter syndrome?

A

when a male has a additional X chromosomes making them 47XXY

654
Q

what is the presentation of Kleinfelters syndrome? (8)

A

normal development as male till puberty then

taller height
wider hips and gynaecomasitia
weaker muscles
small testes
reduced libido
shyness
infertility
subtle learning difficulties

655
Q

what is the management of kleinfelters syndrome?

A

testosterone injections
advanced IVF techniques
breast reduction

656
Q

what are 4 things that at at increased risks in kleinfelters?

A

breast cancer (more than other men)
osteoporosis
diabetes
anxiety and depression

657
Q

what is turners syndrome?

A

females with single X chromosome on chromosome 45 -> 45XO

658
Q

what are 9 features of turner syndrome?

A

short stature
webbed nack
high arching palate
downward sloping eyes with ptosis
broadchest and with wide spaced nipples
cubitus valgus
underdeveloped ovaries
late/incomplete puberty
infertile

659
Q

what are 9 conditions associated with turners syndrome?

A

recurrent otitis media
recurrent UTI
Cardiac - coarctation of aorta, pulmonary stenosis
hypothyroid
hypertension
obesity
diabetes
osteoporosis
learning difficulties

660
Q

what are 3 managements for turners?

A

growth hormone therapy for height
oestrogen and progesterone for female secondary sex characteristics
fertility treatment

661
Q

what are 8 dysmorphic features of downs syndrome?

A

hypotonia
brachycephaly (small head with flat back)
short neck
short stature
flattened face and nose
prominent epicanthic folds
upward sloping palpebral fissures
single palmar crease

662
Q

what are 8 complications of downs?

A

recurrent otitis media and deafness (eustachion tube abnormalities)
visual problems
hypothyroidism
cardiac defects - ASD, VSD, PDA, tetralogy
atlantoaxial instability
leukaemia - AML (most common) and ALL
Alzheimers
Hirschprungs disease and duodenal atresia

663
Q

what cardiac defects are more likely in downs?

A

ASD
VSD
PDA
Tetralogy of fallot

664
Q

what nuchal thickness is indicative of downs?

A

> 6mm

665
Q

what is the combinded test for downs syndrome?

A

11-14 weeks
USS for neucal transleucency
bloods - B-HCG, PAPPA

666
Q

what is the tripple test?

A

between 14-20 weeks

maternal bloods - B-HCG, alpha-fetoprotein, serum oestrodiol

667
Q

what is the quadruple test for downs syndrome?

A

14-20 weeks

HCG, AFP, Total Oestrodiol
+ inhibin-A

668
Q

what are 2 ways of antenatal testing for downs?

A

chorionic villus sampling
amniocentesis

669
Q

what are 4 routine follow ups needsd in downs?

A

regular thyroid checks - 2 yearly
echo
regular audiometry
regular eye checks

670
Q

what is the average life expectancy for downs?

A

60 years

671
Q

what is the genetics underlying patau syndrome?

A

trisomy 13

672
Q

what genetics cause Edwards syndrome?

A

trisomy 18

673
Q

What are 7 key features of Edwards syndrome syndrome?

A

Rocker(rounded)-bottom feet
low birth weight
prominent occiput
small mouth and chin
short sternum
flexed overlapping fingers
cardiac and renal malformations

674
Q

what are 6 features of Pataus?

A

structural defects of brain
scalp defects
small eyes and eye defects
cleft lip and palate
polydactyly
cardiac and renal malformations

675
Q

what gene is affected in fragile X syndrome?

A

fragile X mental retardation 1 gene on X chromosome

males are always affected but females vary

676
Q

what are 8 features of fragile X?

A

intellectual disability
long narrow face
large ears
large testes after puberty
hypermobile joints
ADHD
ASD
Seizures

677
Q

what gene is affected in Angelman syndrome?

A

UBE3A gene on chromosome 15

678
Q

what are 6 key features of Angelman syndrome?

A

fascination with water
happy demeanour
wide spaced mouth and teeth
learning and developmental delay
Fair skin, light hair, blue eyes
epilepsy

679
Q

what is the genetics of prader-willi?

A

loss of functional genes on proximal arm of chromosome 15 inhertied from father

680
Q

what are 6 key features of prader-willi?

A

constant insatiable hunger
hypotonia
mild/moderate learnign disability
hypogonadism
dysmorphic - narrow forehead, almond eyes, thin upper lip, downturned mouth
mental health problems

681
Q

what is the management of prader willi?

A

growth hormone - for improving muscle development an body composition

dieticians, education support, psych, physio, OT

682
Q

what are 8 features of noonan syndrome?

A

short stature
broad forehead
downward sloping eyes with ptosis
hypertelorism (widespaced eyes)
prominent nasolabial folds
low set ears
webbed neck
widely spaced nipples

683
Q

what are 6 conditions associated with noonan syndrome?

A

congenital heart disease
Undescended testes
learning disability
lymphoedema
increased risk of leukaemia and neuroblastoma

684
Q

what is the cause of william syndrome?

A

deletion on chromosome 7 - usually random rather than inherited

685
Q

what are 8 features of william syndrome?

A

broad forehead
starburst iris
flattened nasal bridge
long philtrum
wide mouth and spaced teeth
small chin
sociable and trusting personality
mild learning disability

686
Q

what are 4 conditions associated with william syndrome?

A

supravalvular aortic tenosis
hypercalcaemia
ADHD
hypertension

687
Q

what is the management for williams sydrome?

A

echo and BP monitoring
low calcium diet

688
Q

what is gower’s sign?

A

children with proximal weakness get onto their hands and knees then go into downward dog and push themselves up using their hands

MUSCULAR DYSTROPHY

689
Q

what is the name of the sign that is the way children with muscular dystrophy get up?

A

Gower’s sig

690
Q

what are 7 types of muscular dystrophy?

A

duchennes muscular dystrophy
beckers muscular dystrophy
faciosapulohumeral muscular dystrophy
oculopharyngeal muscular dystrophy
limb-girdle muscular dystrophy
emery-dreifuss muscular dystrophy

691
Q

what is the inheritance for duchennes MD?

A

X-linked recessive

692
Q

what is the prognosis for duchennes MD?

A

25-35 years

693
Q

what is the management of duchennes MD?

A

oral steroids - slow progress of muscle weakness

creatine supplements improve muscle strength

694
Q

when do symptoms start to appear in duchennes MD?

A

3-5 years

695
Q

when do symptoms start to appear in beckers MD?

A

8-12 years

696
Q

when does myotonic dystrophy usually present?

A

adulthood

697
Q

what are 4 key features of myotonic dystrophy?

A

progressive muscle weakness
prolonged muscle contractions
cataracts
cardiac arrhythmia

698
Q

what are 4 key features of facioscapulohumeral muscular dystrophy?

A

weakness around face progressing to shoulders and arms
sleeping with eyes slightly open
weak pursing lips
unable to blow out cheeks without air leaking from mouth

699
Q

what are 3 key features of oculopharyngeal MD?

A

bilateral ptosis
restricted eye movements
swallowing problems

usually presents in alate adulthood

700
Q

what is limb girdle MD?

A

presents in teenage years with progressive weakness around limb girdles - hips and shoulders

701
Q

what is emery-dreifuss MD?

A

presents in childhood with contractures which restricts range of movement
also progressive weakness and wasting of muscles starting with upper arms and lower legs

702
Q

what chromosome and what gene is affected in CF?

A

chromosome 7

cystic fibrosis transmembrane conductance regulatory gene

703
Q

what does the CF transmembrane conductance regulatory gene code for?

A

Chloride channels

704
Q

what are 3 features of CF?

A

thick pancreatic and biliary secretions causing blockages
low vllume thick airway secretions
congenital bilateral absence of vas deferens

705
Q

what is the inheritance of CF?

A

autosomal recessive

706
Q

what are 7 causes of clubbing in children?

A

hereditary clubbing
cyanotic heart disease
infective endocarditis
CF
TB
IBD
Liver cirrhosis

707
Q

what are 3 key tests for CF?

A

newborn blood spot test
sweat test - GOLD
genetic testing

708
Q

what is the diagnostic chloride conc for CF on sweat test?

A

60 mmol/L

709
Q

what is a key problematic coloniser for people with CF?

A

pseudomonas aeruginosa

710
Q

what is treatment for pseudomonas infection in CF?

A

long termtobramycin nebs

or oral ciprofloxacin

711
Q

what is the management for CF?

A

multidisciplinary

chest physio
exercise
high calory diet
creon tablets
prophylactic flucloxacillin
tx infections
bronchodilators
DNase Nebs
hypertonic saline nebs
vaccination

712
Q

what is the median life expectancy for CF?

A

47 years

713
Q

what bacteria causes TB?

A

mycobacterium tuberculosis

714
Q

what stain is required in TB?

A

Zeihl-neelson stain

acid-fast bacilli that turn red against blue background on Z-N

715
Q

what are 8 symptoms of TB?

A

cough
haemoptysis
lethargy
fever/night sweats
wt loss
lymphadenopathy
erythema nodosum
spinal pain

716
Q

what are 2 investigations for TB?

A

mantoux test
interferon gamma release assay

717
Q

what is the mantoux test?

A

for TB
inject tuberculin proteins into intradermal space and measure size of injection site at 72 hours - >5mm = positive

718
Q

what is the appearance of disseminated miliary TB on CXR?

A

millet seeds uniformally distributed

719
Q

what is the treatment for active TB?

A

RIPE

Rifampicin - 6 months
Isoniazid - 6 months
Pyrazinamide - 2 months
Ethanbutol - 2 months

720
Q

what is the treatment of latent TB?

A

isoniazid and rifampicin 3 months
or isoniazid for 6 months

721
Q

what are 3 side effect of rifampicin?

A

red/orange piss and tears

reduced CYP450 drug effectiveness - COCP

hepatotoxic

722
Q

what should be prescribed with isoniazid?

A

pyridoxine (vit B6)

723
Q

what are 2 side effects of isoniazid?

A

peripheral neuropathy
hepatotoxic

724
Q

what are 2 side effects of pyrazinamide?

A

hyperuricaemia => gout and kidney stones
hepatotoxic

725
Q

what are 2 side effects of ethambutol?

A

colour blindness
reduced visual acuity

726
Q

what are 3 risks of undescended testes?

A

testicular torsion
infertility
testicular cancer

727
Q

what are 5 risk factors for undescended testes?

A

FHx
low birth weight
small for gestational age
prematurity
maternal smoking

728
Q

what is the management for unilateral undescended testes?

A

watch and wait for 3 months then refer

orchidopexy between 6-12 months

729
Q

what is the usual presentation of testicular torsion?

A

teenage boy
unilateral testicular pain
abdo pain and vomiting
often triggered by sport

730
Q

what are 5 examination findings in testicular torsion?

A

firm swollen testicle
elevated esticle
absent cremasteric reflex
abnormal testicular lie
rotation so epididymis is not posterior

731
Q

what is the name of the deformity that makes testicular torsion more likely?

A

bell-clapper deformity - testicle hangs in more horizontal position

732
Q

what is the management for testicular torsion?

A

nil by mouth
analgesia
urgent senior urology assessment
surgical exploration of scrotum
orchiopexy
orchidectomy if needed

733
Q

what sign may be seen on USS for testicular/ovarian torsion?

A

whirlpool sign - spiral appearance of spermatic cord and blood vessels

734
Q

what are 6 presentations of congenital hypothyroidism?

A

usually picked up on blood spot

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

735
Q

what antibody is present in hashimotos?

A

antithyroid peroxidase antibodies and antithroglobulin antibodies

736
Q

what causes congenital adrenal hyperplasia?

A

congenital deficiency in 21-hydroxylase enzyme causing underproduction of cortisol and aldosterone and overproduction of androgens (testosterone)

Causes increased sodium and potassium excretion

737
Q

what is the inheritance pattern for congenital adrenal hyperplasia?

A

autosomal recessive

738
Q

what is the presentation of congenital adrenal hyperplasia in severe cases?

A

enlarged clitoris in females
hyponatraemia
hyperkalaemia
hypoglycaemia

poor feeding
vomiting
dehydration
arrhythmias

SKIN HYPERPIGMENTATION

739
Q

what is the presentation of congenital adrenal hyperplasia in mild cases?

A

female
- tall for age
- facial hair
- absent periods
- deep voice
- early puberty
Males
- tall or age
- deep voice
- large penis
- small testicles
- early puberty

740
Q

what is the management of congenital adrenal hyperplasia?

A

cortisol replacement - hydrocortisone
aldosterone replacement - fludrocortisone

virilised genitals corrective surgery

741
Q

what is the inheritance pattern for androgen insensitivity syndrome?

A

X-linked recessive due to mutation on androgen receptor gene on X-chromosome in XY males

742
Q

what is the pathophysiology of androgen insensitivity syndrome?

A

cells are unresponsive to androgen hormones due to a lack of androgen receptors leading to excess androgens which are converted to oestrogen resulting in female secondary sexual characteristics and external female phenotypes from birth. Internally there are testes in the abdo/inguinal canal but no internal female organs due to the testes releasing anti-Mullerian hormone

743
Q

what are 2 presentations of androgen insensitivity syndrome?

A

inguinal hernias
primary amenorrhoea

744
Q

what will hormone bloods be like in androgen insensitivity syndrome?

A

Raised LH
normal/raised FSH
Normal/raised testosterone for a man
Raised oestrogen for a man

745
Q

what is the management for androgen insensitivity syndrome?

A

bilateral orchidectomy - testicular tumours
oestrogen therapy
vaginal dilators and surgery

746
Q

what age does Wilms tumours usually present in?

A

<5 years

747
Q

what are 7 presentations of Wilms tumours?

A

abdo pain
haematuria
lethargy
fever
HTN
Weight loss
Abdo mass

748
Q

What is the first line Ix for a Wilms tumour?

A

USS abdo

749
Q

what is the management of Wilms tumour?

A

surgical excision +/- nephrectomy

+/- adjuvant chemo/radio

750
Q

what is the prognosis for Wilms tumour?

A

up to 90% cure in early stages

751
Q

what is the most common leukaemia in children?

A

1st - ALL

2nd - AML

752
Q

what age is the peak incidence of ALL?

A

2-3 years

753
Q

what age is the peak incidence of AML?

A

<2 years

754
Q

what are 4 conditions that are risk factors for leukaemia?

A

Down syndrome
kleinfelter syndrome
Noonan syndrome
faconi’s anaemia

755
Q

what is the prognosis for ALL?

A

80% cure rate

756
Q

what are 7 complications of chemo?

A

failure of treatment
stunted growth and development
immunodeficiency and infection
neurotoxicity
infertility
secondary malignancy
cardiotoxicity

757
Q

what causes Idiopathic thrombocytopenic purpura?

A

type 2 hypersensitivity reaction that casues antibodies to target and destroy platelets either spontaneously or triggered due to viral infection

758
Q

what age group does idiopathic thrombocytopenic purpura present in?

A

<10 years

759
Q

what is the management for severe idiopathic throbocytopenic purpura?

A

prednisolone
IVIG
blood transfusion if required
platelet transfusion may work temporarily

760
Q

what are 4 complications of ITP?

A

chronic ITP
anaemia
intracrania and subrachnoid haemorrhage
GI bleeds

761
Q

what are 5 causes of anaemia in infants?

A

physiological anaemia
anaemia of prematurity
blood loss
haemolysis
twin-twin transfusion syndrome

762
Q

what is physiological anaemia of infancy?

A

normal dip in haemoglobin at 6-9 weeks due to high O2 at birth

763
Q

what are 4 reasons for anaemia of prematurity?

A

less time in utero getting iron from mum
RBC production cant keep up with growth
reduced EPO
blood tests

764
Q

what is the treatment for helminth infection?

A

albendazole or mebendazole

765
Q

what can interfere with iron absorption?

A

acid from stomach is required to comvert iron into ferrous (Fe2+) form so PPIs can interfere with absorption

also coeliac or chrons

766
Q

what test can be used to identify haemolytic disease of the newborn?

A

direct coombs test

767
Q

what deficiency causes haemophilia A?

A

factor VIII

768
Q

what deficiency causes haemophilia B?

A

factor IX

769
Q

what is the inheritance pattern for haemophilia?

A

X-linked recessive

770
Q

what are 3 complications of haemophilia?

A

intracranial haemorrhage
haemarthrosis
compartment syndrome

771
Q

what is von willebrand factor?

A

a glycoprotein important in platelet adhesion and aggregation (formation of platelet plug)

772
Q

what are the 3 types of von-willebrand factor disease?

A

1 - partial deficiency
2 - reduced function
3 - complete deficiency

773
Q

what is the acute management of von willebrand disease?

A

desmopressin (stimulated release of vWF from endothelial cells)
Tranexamic acid
vWF infusion +/- factor VIII

774
Q

what are 5 options for heavy menstrual periods?

A

tranexamic acid
mefanamic acid
mirena coil
COCP
norethisterone

775
Q

what is faconi anaemia?

A

rare inherited disorder of gradual bone marrow failure and birth defects eventually leading to aplastic anaemia

776
Q

what is kallmann syndrome and 3 features?

A

an X linked disorder
Causes hypogonadotrophic hypogonadism due to failure of GnRH release

Delayed puberty
Low sex hormones, LH and FSH
differentiating feature of INABILITY TO SMELL

777
Q

what are the three key areas affected by Autism?

A

deficits in
social interaction
communication and behaviour

778
Q

what are 6 possible social diferences in autism?

A

lack of eye contact
delay in smiling
avoids physical contact
unable to read non-verbal cues
difficulty establishing friendships
no desire to share attention (play with others)

779
Q

what are 4 communication diferences in autism?

A

delay, absence or regression in language
lack of appropriate non-erbal communication
difficulty with imaginative or imitative behaviours
repetitive use of words or phrases

780
Q

what are 6 behavioural differences in autism?

A

greater interest in things than people
sterotypical repetitive movements
intensive deep interests
repetitive behaviours and fixed routines
anxiety and distress with deviation from routine
extremely restricted food preferences

781
Q

what are 6 features of ADHD?

A

very short attention span
quick moving from one activity to another
quick loss of interest in task or inability to persist with challenging task
constantly moving or fidgeting
impulsive behaviour
disruptive or rule breaking

782
Q

what are 3 ADHD meds?

A

methyphenidate - ritalin
dexamfetamine
atomoxetine

783
Q

what are 8 features of annorexia?

A

excessive wt loss
amenorrhoea
lanugo hair
hypokalaemia
hypotension
hypothermia
changes in mood
cardiac complications

784
Q

what are 3 cardiac complications of anorexia?

A

arrythmias
cardiac atrophy
sudden cardiac death

785
Q

what are 7 features of bulimia?

A

alkalosis on Blood gas
hypokalaemia
erosion of teeth
swollen salivary glands
mouth ulcers
GORD
calluses on knuckles (russels sign)

786
Q

what are 3 micronutrient deficiencies due to starvation in eating disorders?

A

hypomagnesaemia
hypokalaemia
hypophosphataemia

787
Q

what is the management of eating disorders to avoid refeeding syndromes?

A

slowly refeed
magnesium, potassium, posphate and glucose monitoring
fluid balance monitoring
ECG monitoring
supplementations with electrolytes, B vitamins and thyamine

788
Q

what is the first line antidepressant in children?

A

Fluoxetine 10-20mg

789
Q

what are 8 signs of dehydration?

A

appear unwell
altered consciousness
sunken eyes
tachycardia
tachypnoea
reduced skin turgor
dry mucous membranes
decreased urine outpt

790
Q

what is the first line maintenance fluid choice in children >28 days?

A

0.9% NaCl + 5% glucose

791
Q

what is the first line fluid choice in neonate <28 days?

A

if well 10% dextrose if unwell seek advice

792
Q

what is the formula for maintenance fluids in >28 days of life?

A

100 mk/kg/day 1st 10kg
50ml/kg/day 2nd 10kg
20 ml/kg/day >20kg

793
Q

what is the maintenance fluid requirements for <28 days?

A

day 1 - 50-60 ml/kg/day
2 - 70-80 ml/kg/day
3 - 80-100 ,l/kg/day
4 - 100-120 ml/kg/day
5-28 - 120-150 ml/kg/day

794
Q

what is the calculation for percentage dehydration?

A

(well weight - current weight)/well weight

X100

795
Q

how do you calculate fluid deficit?

A

% dehydration X weight (kG) X 10

796
Q

how do you calculate total fluid requirement?

A

maintenance fluid + fluid deficit

797
Q

what fluids should be given for resucitation?

A

0.9% NaCl
10 ml/Kg <10 mins

798
Q

what is the normal ages to start to develop secondary sexual characteristics in males and females?

A

males - 9 years
females - 8 years

development before this age is precocious puberty

799
Q

what are are 8 complications of obesity?

A

Slipped upper femoral epiphysis (SUFE)
idiopathic intracranial hypertension
hypoventilation syndrome
fatty liver disease
T2DM
PCOS
HTN
abnormal blood lipids

800
Q

what medication can be given to severely obese children >12 years?

A

orlistat

801
Q

what are 4 causes of gonadotrophin (pituitary) dependant precocious puberty?

A

idiopathic/familial
CNS abnormalities - congenital, tumours (neurfibromatosis, craniopharyngioma)
Hypothyroism

802
Q

what are 5 gonadotrophin independent causes of precocious puberty?

A

adrenal tumour
congenital adrenal hyperplasia
ovarian/testicular tumour (granulosa/leydig cell)
Exogenous sex steroids

803
Q

what age is usually affected by premature breast development (thelarche) absent of any other signs of precocious puberty?

A

6 months - 2 years

fluctuating development of breast buds which is self limiting and does not require treatment

804
Q

what is premature pubarche (adrenarche)?

A

when pubic hair develops precociously with no other signs of sexual development most commonly caused by accentuation of normal maturation androgen. More common in non-white population. Leads to increased risk of PCOS in adult life.

805
Q

what is delayed puberty in males and females?

A

> 14 years in females
15 years in males

806
Q

what are 7 causes of delayed puberty?

A

congenital/familial - most common
hypogonadotrophic hypogonadism
Hypergonadotrophic hypogonadism

807
Q

what are 4 causes of hypogonadotrophic hypogonadism?

A

systemic disease - CF, asthma, crohns
Hypothalamo-pituitary disorders - tumours, kallman syndrome, pituitary dysfunction
acquired hypothyroidism

808
Q

what are 3 causes of hypergonadotrophic hypogonadism?

A

chromosomal abnormalities - klinefelter syndrome, turner syndrome
steroid hormone enzyme deficiencies
acquired gonadal damage

809
Q

what is the most common solid tumour in children?

A

brain tumour

810
Q

what is the most common brain tumour in children?

A

astrocytomas - raneg from benign to the highly malignant glioblastoma multiforme

811
Q

what is the clinical presentation of brain tumour?

A

persistant/recurrent vomiting
problems with balance, coordination or walking
behaviour change
abnormal eye movement
seizures
abnormal head positioning
headache
blurred/double vision
lethargy
deteriorating school work/developmental delay
increasing head size in infants

812
Q

what is medulloblastoma?

A

2nd most common childhood brain tumour

arises in midline of posteriod fossa and may spread through CSF to spinal mets

813
Q

what is an ependymoma?

A

brain tumour that behaves much like medulloblastoma but arises in posterior fossa

814
Q

what is a brainstem glioma?

A

malignant brain tumour with very poor prognosis

815
Q

what is a craniophryngioma?

A

developmental tumour arising from squamous remnant of rathke pouch - non-malignant but locally invasive

816
Q

what age group is affected by neuroblastoma?

A

<6 years

817
Q

where do neuroblastomas arise from?

A

neural crest tissue in adrenal medulla and sympathetic nervous system

818
Q

what is the presentation of neuroblastoma?

A

mostly abdo mass but primary tumour may be anywhere along sympathetic chain from neck to pelvis

pallor, wt loss, abdo mass, hepatomegally, bone pain, limp

819
Q

what investigations can be does for neuroblastoma?

A

urinary catecholamine metabolite levels (VMA/HVA)

BIOPSY

820
Q

what is the inheritance pattern for retinoblastomas and what chromosome is it found on?

A

autosomal dominant - incomplete penetrance

chromosome 13

821
Q

what are 2 presenting features of retinoblastoma?

A

red reflex turns white
squint

822
Q

what is the most common liver tumour in children?

A

hepatoblastoma

usually presents with bloating and abdo mass

823
Q

when does the palmar reflex usually disappear?

A

2-6 months

824
Q

when does the sucking reflex usually start?

A

around 32 weeks gestation - not fully developed until 36 weeks

825
Q

how long does the moro reflex last?

A

around 2 months

826
Q

how long does the stepping reflex last?

A

around 2 months

827
Q

how long does the rooting reflex last?

A

around 4 months

828
Q

when does osteosarcoma usually present?

A

10-20 year olds

829
Q

what is the most common bone to be affected by osteosarcoma?

A

femur

tibia and humorous also common

830
Q

what are 5 symptoms of bone tumour?

A

persistent bone pain
pain worse at night - disturb or wake from sleep
bone swelling
palpable mass
restricted joint movement

831
Q

what is a classical x-ray finding of bone tumour?

A

periosteal reaction causing ‘sun-burst’ appearance of bone

832
Q

what can be seen on LFTs that suggest bone tumour?

A

raised ALP

833
Q

what are 2 complications of bone tumours?

A

pathological fractures
metastasis

834
Q

what is the 1st line investigation for bone tumour?

A

urgent (in 48 hours) x-ray and specialist assesment

835
Q

what is the most common bone cancer in children?

A

osteosarcoma

836
Q

what is the second most common bone cancer in children?

A

ewing sarcoma

837
Q

what does use of NSAIDs in chicken pox increase risk of?

A

necrotising fasciitis - increase risk of bacterial infection

838
Q

what is the infectivity period of chicken pox?

A

from 4 days pre-rash to around 5 days after rash appears when all crusted over

839
Q

when is the meningitis B vaccine given?

A

2 months, 4 months, 12 months

840
Q

what is the proper name for threadworms?

A

enterobius vermicularis

841
Q

what is the management for threadworms?

A

Mebendazole single dose for whole household

only for >6 months

842
Q

what does a blood gas with pyloric stenosis look like?

A

Hypochloraemia
Hypokalaemia
elevated bicarb

843
Q

what are 5 causes of obesity in children?

A

downs syndrome
prader-willi syndrome
growth hormone deficiency
hypothyroid
cushings syndrome

844
Q

what are 5 consequences of obesity in children?

A

Ortho - SUFE, MSK Pains, Blounts disease
Psycho - bullying, low self esteem
sleep apnoea
benign intracranial hypertension
long term - T2DM, hypertension, IHD

845
Q

what is the first line management for tonic clonic seizures in kids?

A

sodium valporate

846
Q

what is the second line management for tonic clonic seizures in kids?

A

lamotrigine or levetiracitam

847
Q

where in the brain do focal seizures start?

A

temporal lobes

affect hearing, speech, memory and emotion may cause people to do things on autopilot

848
Q

what is the management for focal seizures?

A

1 - lamotrigine or levitiracitam

2 - carbamazepine, oxcarbazepine, zonisamide.

3 - lacosamide

849
Q

what is the 1st line management for absence seizures?

A

1 - ethosuximide

2 - sodium valporate/lamotrigine/levetiracitam

850
Q

what syndrome is atonic seizures linked to?

A

Lennox-Gastaut syndrome

851
Q

what is the mangement for atonic seizures?

A

1 - sodium valporate
2 - lamotrigine

852
Q

what is the 1st line management for myoclonic epilspsy?

A

sodium valporate/levetiracitam (girls)

853
Q

what is the 2nd line management for myoclonic seizures?

A

lamotrigine, levetiracetam or topiramat

854
Q

what is the management for infantile spasms?

A

Prednisolone
Vigabatrin

855
Q

what are 4 side effects of sodium valporate?

A

teratogenic
liver damage/hepatitis
Hair loss
tremmor

856
Q

what is the MOA of sodium valporate?

A

increases activitiy of GABA

857
Q

what are 3 side effects of carbamezapine?

A

Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions

858
Q

what are 3 side effects of phenytoin?

A

Folate and vitamin D deficiency
Megaloblastic anaemia (folate deficiency)
Osteomalacia (vitamin D deficiency)

859
Q

what are 2 side effects of ethosuximide?

A

Night terrors
Rashes

860
Q

what are 2 side effects of lamotrigine?

A

Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.
Leukopenia

861
Q

what is the management of status epilepticus in hospital?

A

IV lorazepam x2
IV phenytoin/phenobarbital
ITU

862
Q

what is the medical management of seizure in the community?

A

Buccal midazolam
Rectal diazepam

863
Q

what inheritance pattern is there in achondropasia?

A

autosomal dominant

864
Q

what mutation causes achondroplasia?

A

mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene

865
Q

what are 5 features of achondroplasia?

A

short limbs (rhizomelia) with shortened fingers (brachydactyly)
large head with frontal bossing and narrow foramen magnum
midface hypoplasia with a flattened nasal bridge
‘trident’ hands - gap between middle and ring finger
lumbar lordosis

866
Q

what is the management of asthma <5 years?

A

SABA
+ ICS for 8 weeks
+ leukotriene receptor antagonist (LTRA)

refer to secondary care

867
Q

what is a paeds ICS low dose?

A

<200 micrograms

868
Q

what is a paeds ICS high dose?

A

> 400 micrograms

869
Q

what are 2 conditions associated with hypospadias?

A

cryptorchidism
inguinal hernia

870
Q

what medication can be used in CF patients who are homozygous for the delta F508 mutation?

A

Lumacaftor/Ivacaftor (Orkambi)

871
Q

what is a venous hum?

A

innocent murmur heard just below clavicles of blood returning to heart

872
Q

what is stills murmour?

A

innocent murmur of Low-pitched sound heard at the lower left sternal edge

873
Q

what are 8 features of innocent murmurs?

A

soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area
may vary with posture
localised with no radiation
no diastolic component
no thrill
no added sounds (e.g. clicks)
asymptomatic child
no other abnormality

874
Q

what is the management of asymptomatic neonatal hypoglycaemia?

A

encourage feeding + monitor

875
Q

what is the management of symptomatic neonatal hypoglycaemia?

A

SCBU
IV 10% dextrose

876
Q

Is perthes usually unilateral or bilateral?

A

unilateral - only bilateral 10% of time

877
Q

How is perthes disease diagnosed?

A

X-ray

878
Q

what vaccines are given at 8 weeks?

A

6 in 1 (DTaP, IPV, Hib, Hep B),
Men B
Rotavirus

879
Q

what vaccines are given at 12 weeks?

A

6 in 1 (DTaP, IPV, Hib, Hep B)
Pneumococcal
Rotavirus

880
Q

what vaccines are given at 16 weeks?

A

6in1(DTaP,IPV,Hib,HepB)
MenB.

881
Q

what vaccines are given at 1 year?

A

Hib/Men C
Pneumococcal booster
MMR
Men B booster.

882
Q

what vaccines are given at 3 years (4 months)?

A

DTap/IPV - Diptheria, Tetenus, Petussus, Polio
MMR.

883
Q

what vaccine is given at 12-13 years?

A

HPV

884
Q

what vaccines are given at 14 years?

A

Tetanus, diphtheria, polio
Men ACWY.

885
Q

what is in the 6 in 1 vaccine?

A

Diphtheria
Tetanus
Pertussis
Hib
Hepatitis B
Inactivated Polio Vaccine

886
Q

when is the 6 in 1 vaccine given?

A

8, 12 and 16 weeks

887
Q

when is the MMR vaccine given?

A

12 months and 3 years

888
Q

When is the rotavirus vaccine given?

A

8 and 12 weeks

889
Q

when is the men B vaccine given?

A

8 and 16 weeks
booster at 1 year

890
Q

what are the two main sanctuary sites for leukaemia?

A

CNS
Testes

most likely to have secondary malignancies

891
Q

what is the dose of insulin infusion in DKA?

A

0.1 units/kg/h

892
Q

what are 6 blood results that might be seen in DKA?

A

Acidotic
Hyperglycaemia
Ketonaemia
Low Bicarb
raised creatinine
raised potassium

893
Q

what is a key complication of rapid correction of DKA?

A

Cerebral oedema

894
Q

what is the management for cerebral oedema?

A

slowing IV fluids
IV mannitol
IV hypertonic saline

895
Q

what is mild DKA?

A

pH 7.2- 7.29 or bicarbonate < 15 mmol/L. Assume 5%
dehydrationwh

896
Q

what is moderate DKA?

A

pH 7.1-7.19 or bicarbonate < 10 mmol/L. Assume 7%
dehydration

897
Q

what is severe DKA?

A

pH less than 7.1 or serum bicarbonate < 5 mmol/L.
Assume 10% dehydration

898
Q

what should be given in DKA and shock?

A

20 ml/kg bolus of 0.9% Sodium
Chloride over 15 minutes

899
Q

what fluid bolus should be given in DKA not in shock?

A

10 ml/kg 0.9% sodium chloride

900
Q

when should the fluid bolus be subtracted from the resusitation fluids in DKA?

A

when not in shock

901
Q

what fluids should be used in DKA?

A

0.9% sodium chloride with 20 mmol potassium chloride in each 500ml
bag until glucose <14mmol/L

902
Q

what is DiGeorge syndrome and it’s features?

A

22q11.2 deletion

congenital heart disease (e.g. tetralogy of Fallot), learning difficulties, hypocalcaemia, recurrent viral/fungal diseases, cleft palate

903
Q

what is the name of deep rapid breathing in acidosis to rid body of CO2?

A

kassmaul breathing - seen in DKA

904
Q

what is benign rolandic epilepsy?

A

twitching numbness or tingling episodes usually at night or when tired

904
Q

When does neonatal hypoglycaemia require treatment?

A

if symptomatic or BM <1 mmol/L

905
Q

what is the mangement of neonatal hypoglycaemia?

A

2.5 mg/Kg 10% dextrose

906
Q

what is the management of foecal impaction?

A

1 - macrogol laxative (movicol)
2 - senna if doesnt work after 1 week

use lactulose if macrogol isn’t tolerate

907
Q

what surgery is done in Hirschprungs?

A

Swensen’s procedure

908
Q

what is the most common cause of convergent squint?

A

hypermetropia (long sightedness)

909
Q

what extra feature is present in pentology of fallot?

A

ASD

Phase 3A (7 decks)

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