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4th Year > Paeds Notes > Flashcards

Paeds Notes Flashcards

1
Q

Cystic fibrosis: AR/AD?

A

AR

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2
Q

Cystic fibrosis: 1 in ? affected?

A

1 in 2000

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3
Q

Cystic fibrosis: 1 in ? carriers?

A

1 in 22

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4
Q

Life expectancy of cystic fibrosis

A

Mid 30s

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5
Q

Mutation in cystic fibrosis and which chromosome

A

CFTR on Chr7. most commonly delta f508

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6
Q

Organism that specifically affects cystic fibrosis

A

pseudomonas auriginosa

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7
Q

If not through newborn screening, how might CF present?

A

meconium ileum

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8
Q

2 diagnostic tests for CF

A 
Heel prick
Sweat test (>60mmol/l of sweat Cl- supports diagnosis)
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9
Q

Treatment for CF

A 
MDT approach
• Education
• Respiratory:
o Monitor FEV1 and symptoms
o Physiotherapy
o Nebulisers
o Prophylactic and rescue antibiotics
• Nutritional/ GI
o Monitor: weight/ regularly asses diet, OGTT (Over 12) o Replacement enzymes
o Multivitamin
o High calorie/ high protein diet
• Psychological
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10
Q

When do you give Abx to tonsillitis/pharyngitis? And what antibiotic for how long?

A

10 days Pen V if FeverPAIN score:
0-1 = nothing
2-3 = delayed
4-5 = give Abx

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11
Q

Indication for tonsilectomy

A

7 in one year or 10 in 2 years

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12
Q

scarlet fever cause

A

Group A strep (think of strep throat)

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13
Q

cause of croup

A

parainfluenza 3

sometimes RSV

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14
Q

management of croup

A

oral dex and support

IV adrenaline is emergency

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15
Q

presentation of bacterial tracheitis

A

viral prodrome 2-5 days then rapid deterioration with continuous biphasic stridor

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16
Q

cause of bacterial tracheitis

A

S.aureus

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17
Q

management of bacterial trachetiis

A

cefotaxime + fluclox

?intubation

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18
Q

key feature of diptheria

A

tonsilitis + grey false membrane over them

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19
Q

management of diptheria

A

Diptheria antitoxin

erythromycin to patient and contacts

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20
Q

epiglottitis cause

A

Hib

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21
Q

presentation of epiglottitis

A

VERY RAPID ONSET

Very high fever, won’t swallow and will drool instead. continuous stridor.

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22
Q

management of epiglotittis

A

Call anaesthesitst, paediatrician and ENT surgeon for controlled intubation.
Cefotaxime as Abx

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23
Q

age at which viral wheeze tends to stop

A

5 (most is 2-3)

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24
Q

brittle asthma types

A

type 1 - variable PEFR

type 2 - sudden attack on normal background

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25
Q

when to discharge after acute asthma

A

when stable on >4hrly salbutamol

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26
Q

what to discharge acute asthma attack with

A
  • 3-7days pred
  • follow up in 1 months in clinic
  • inhaler technique
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27
Q

when is acute bronchiolitis worst?

A

5 days

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28
Q

bronchiolitis cause?

A

RSV

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29
Q

bronchiolitis prevention?

A

Monthly pavalizumab

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30
Q

whooping cough timeline

A

1 week of coryza

3 months of paroxysmal phase

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31
Q

treatment of whooping cough

A

macrolide

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32
Q

latent TB worldwide number?

A

2 billion

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33
Q

death from TB / year

A

2.5 million

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34
Q

investigations for TB (3)

A

tuberculin
interferon gamma release assay
Zn stain for acid fast bacilli

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35
Q

TB management: latent and active

A

latent = 3m RI

Active = 2mRIPE and 4mRI

rifampicin, isoniazid, pyrazinamide, ethambutol

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36
Q

pulmonary blood flow before vs after first breath

A

6x increase

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37
Q

why does the foramen ovale close

A

blood goes to lung and so left atrial pressure increases

blood excluded from placenta to right atrial pressure decreases

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38
Q

Why are innocent murmurs SASSy?

A

soft
asymptomatic (incl. sats)
systolic
left sternal edge

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39
Q

flow murmur scenario

A

fever
anaemia
day 1 murmur (due to physiologic tricuspid regurgitate)

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40
Q

type of murmur:

aortic stenosis

A

ejection systolic upper right

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41
Q

type of murmur:

ASD

A

soft systolic and S2 splitting

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42
Q

type of murmur:

pul stenosis

A

ejection systolic upper left

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43
Q

type of murmur:

VSD

A

harsh pan systolic left chest

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44
Q

type of murmur:

coarc of aorta

A

systolic on left chest

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45
Q

R to L presentation
L to R presentation

R to L or L to R:

  • tetralogy of fallot
  • ASD
  • transposition of great arteries
  • VSD
  • PDA
  • Eisenmenger
A 
RL = blue
LR = breathless (acyanotic)
tetra RL
asd LR
tga RL
VSD LR
PDA LR
Eisen RL
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46
Q

tetralogy of fallot

A
  1. large VSD
  2. over-riding aorta
  3. pulmonary stenosis
  4. RV hypertrophy
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47
Q

most common congenital heart disease?

A

VSD (30% of total), and 9 most common account for 80%

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48
Q

how do the following present?

L to R shunt (VSD, PDA, ASD)

R to L shunt (ToF, TGA)

Mixing shunts (ASD+VSD)

Outflow obstruction in well child (Pul or aortic stenosis)

Left heart outflow obstruction (coarctation of aorta, hypoplastic left heart, interruption of aortic arch)

A

L to R shunt = breathless

R to L shunt = blue

Mixing shunts = blue and breathless

Outflow obstruction in well child = asymptomatic murmur

Left heart outflow obstruction = sudden circulatory collapse with some absent pulses

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49
Q

eisenmenger syndrome

A

longstanding L2R shunt (e.g. VSD/ASD/PDA) goes unnoticed. This eventually causes pulmonary hypertension and reversal of the shunt, causing cyanosis in adults.

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50
Q

types of ASD and which is more common

A

primum (Downs)

secumdum (85%)

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51
Q

presentation of ASD

A

asymptomatic with murmur (fixed S2 splitting and soft systolic left sternal edge)

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52
Q

CXR for ASD

A

cardiomegaly (RV dilatation - remember its L2R), enlarged pul. arteries, increased pul. vascular markings.

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53
Q

presentation of VSD

A

asymptomatic with murmur (pansystolic):

  • small VSD = loud murmur
  • large VSD = quiet murmur
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54
Q

CXR and ECG with VSD

A
  • small VSD = normal

- large VSD = cardiomegaly, enlarged pul arteries, increase pul vast marking, biventricular hypertrophy on ECG

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55
Q

management of VSD

A
  • small = most close spontaneously

- large = heart failure meds (diuretics and ACEi) + surgery at 6m

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56
Q

when is a ductus arteriosus considered failed to have closed?

A

1 months

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57
Q

presentation of PDA

A

machinery murmur
collapsing/bounding pulse
breathless

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58
Q

management of PDA

A

closure by coil

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59
Q

what is the nitrogen washout test

A

aims: “does this blue kid have congenital heart problem?”
method: 100% oxygen for 10 mins
results: if right radial artery ppO2 is still low, congenital heart disease can be diagnosed

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60
Q

management of cyanosed neonate

A

ABC
IV prostaglandin
Do ECG, Echo and assess cause and need for surgery

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61
Q

CXR with tetralogy of fallot

A

Small heart with up tilted apex (boot shaped RV hypertrophy)

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62
Q

when is surgery for ToF

A

6 months

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63
Q

When does TGA present

A

day 2 of life with rapid deterioration as duct closes (since it’s two parallel circuits at this point)

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64
Q

management of TGA

A

as with any cyanosed baby (ABC, IV prost, investigate)

balloon atrial septostomy in 20% of cases, arterial switch surgery on day 3-5

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65
Q

atrioventricular septal defect common in?

A

Downs

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66
Q

How do AVSDs present

A

antenatal USS
cyanosis at birth
Heart failure 2-3 weeks
no murmur

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67
Q

how does aortic stenosis/pul stenosis occur in babies?

A

leaflets fused abnormally (as opposed to calcification in elderly

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68
Q

when does coarctation of the aorta present?

A

normal at first check, circ. collapse at 2-3 days when duct closes

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69
Q

what is coarctation associated with?

A

Turners

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70
Q

management of hypoplastic left heart syndrome

A

Norwood operation

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71
Q

WPW Syndrome ECG change

A

delta wave

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72
Q

presentation of SVT

A

narrow complex rapid tachycardia 250-300 +/- pulmonary oedema (breathlessness)

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73
Q

management of SVT

A

RESTORE SINUS

  1. vagal stimulating manaenovres (carotid massage)
  2. IV adenosine (in adults its 6,12,12)
  3. DC cardio version

LONG TERM

  • flecainide or sotalol

90% don’t have further attack so stop treatment at 1 yr

If WPW present, need ablation

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74
Q

long QT syndrome presentation

A

sudden LOC during exercise, stress, emotion in late childhood (confused with epilepsy)

can cause sudden death from VT

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75
Q

long QT inheritance and associations

A

AD inheritance (its structural)

association:

  • erythromycin
  • electrolyte disorders,
  • head injury
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76
Q

mortality of Coxsackie virus myocarditis in children

A

80%

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77
Q

management of bacterial endocarditis

A

Benzyl pen + gent +/- surgery

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78
Q

criteria for kawasaki

A

Fever for >5days and 4/5 of:

  • non purulent conjunctivitis
  • lymphadenopathy
  • maculpapular rash
  • extremity involvement (Red finger tips)
  • mucositis (strawberry tongue, cracked lips)
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79
Q

management of kawasaki

A

High dose aspirin
Immuniglobulin
need angiogram

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80
Q

jones diagnostic criteria

A

for rheumatic fever. Need 2 major or 1 major /2minor AND evidence of previous strep infection (scarlett fever or positive throat swab)

Major:
carditis (murmur, CCF, cardiomegaly, friction rub, echo)
poly arthritis
-erythema marginatum
subcutaneous nodule
sydenham's chorea
minor:
fever
arthralgia
prolonged PR interval
high ESR/CRP
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81
Q

treatment for rheumatic fever

A

rest
aspirin
benzylpenicillin followed by Pen V (prevents secondary rheumatic fever)

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82
Q

what is posseting vs regurg vs vomit

A

small amounts of milk that’s burped up alongside swallowed air. normal in babies. regurg is larger more frequent losses. vomiting is forceful ejection

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83
Q

when does GORD in baby resolve usually

A

12 months, resolved in almost everyone

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84
Q

does GORD affect weight of baby?

A

Not usually

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85
Q

complications of GORD (4)

A 
faltering growth
oesophagitis (haematemesis, pain, iron def anaemia)
pul aspiration (pneumonia, apnoea)
Sandifer syndrome (dystonic neck posture to relieve pain)
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86
Q

GORD management

A

Conservative:

  • feed little and often
  • upright after feeds
  • food thickener

Medical:

  • antacid
  • ranitidine or omeprazole

Surgical
- Nissen fundoplication if underlying disorder (CP) to wrap fundus around sphincter

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87
Q

incidence of pyloric stenosis

A

1 in 400

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88
Q

M:F in pyloric stenosis

A

M>F 4:1

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89
Q

management of pyloric stenosis

A

Randmstedt pyloromyotomy - can then be fed at 6hrs and discharged at 2 days

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90
Q

what is duodenal atresia associated with (2)

A

Downs

Polyhydramnios

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91
Q

Investigation for duodenal atresia

A

Abdo plain film = double bubble sign

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92
Q

management of duodenal atresia

A

ABC
Drip and suck
Surgery

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93
Q

when does appendicitis typically present

A

> 5 (under 3 it is vanishingly rare)

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94
Q

how does mesenteric adeninitis present

A

like appendicitis but without peritonism, guarding, rebound tenderness.

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95
Q

peak age for intussuception

A

6m-2y

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96
Q

primary vs secondary intusssuception

A 
primary = lymphoid hyperplasia of peters patch
secondary = pathological point (meckels, polyp) usually older children
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97
Q

presentation of intussuception

A

PAROXYSMAL colicky pain where child become pale and draws up legs

bile vomit
redcurrant jelly stools (late sign)
sausage shapes mass in upper quadrant

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98
Q

CI of air insufflation in intusseoction

A

peritonitis
shock
perforation
known lead pathological point

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99
Q

diagnosis of mecekls diverticulum

A

technetium scan showing increased uptake by ectopic gastric mucosa

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100
Q

when does the in utero gut return to the abdominal cavity

A

10w

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101
Q

when does volvulus due to malrotation present

A

first 1-3 days (90% in first year)

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102
Q

diagnosis of volvulus due to malrotation

A

urgent upper GI contrast shows corkscrew appearance

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103
Q

what 3 features of chronic abdo pain suggest non-organic cause (which is the case with >90%)

A
  1. periodic pain with intervening good health
  2. periumbilical
  3. related to school hours (not there in holidays/evenings)
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104
Q

recurrent idiopathic abdo pain definition

A

pain enough to interrupt ADLs for at least 3 months

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105
Q

what is an abdominal migraine

A

abdo pain in addition to headaches that comes in attacks (long periods ok and then 12-48hrs of pain)

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106
Q

treatment of abdominal migraine

A

NSAIDs and triptans

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107
Q

investigation results for eosinophilic oesophagitis

A

endoscopy with biopsy:

  • linear furrows and trachealisation of oesophagus
  • eosiniphilic infiltration on biopsy
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108
Q

management of eosinophilic oesopahgitis

A

swallowed corticosteroids (fluticasone, viscous budesonide)

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109
Q

what is the only abnormal thing if dehydration <5%?

A

dry mucous membranes

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110
Q

what suggests >10% dehydration as opposed to just 5-10% dehydration?

A

anuria
coma
low BP (late sign)
Mottled/pale skin

–> tachypnoea occurs in moderate 5-10% dehydration, don’t get caught out

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111
Q

calculating fluid requirement

A

Fluids required = maintenance fluid PLUS fluid deficit

maintenance fluid:
first 10kg = 100ml/kg
second 10kg = 50ml/kg
thereafter = 20ml/kg

fluid deficit = estimated % dehydration as a % of body weight (e.g. 10% dehydrated if 7.5kg = 750ml extra)

THIS IS ALL NORMAL 0.9% SALINE

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112
Q

how to rehydrate

A

try oral
then NG
persistent vomiting = IV rehydration

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113
Q

deaths from gastroenteritis per year

A

1.5 million

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114
Q

most common virus causing gastroenteritis

A

rotavirus (60%)

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115
Q

dysentry causes

A

CASES:

campylobacter
amoeba
shigella
E.coli
salmonella
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116
Q

Revise table of gastroenteritis

A

PDF or desktop

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117
Q

why avoid juice in gastroenetritis

A

osmotically draws even more water into gut

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118
Q

when are Abx required in gastroenteritis

A
  • sepsis
  • salmonella under 6m
  • specific infection (cdiff, cholera, shigella, giardia)
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119
Q

advice for parents at home with gastroenteritis in child

A 
diarrhoea lasts 5-7d
vomiting 1-2d
safety net dehydration symptoms
lactose intolerance can follow GE
exclude from school until 48h last D or V
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120
Q

key question with chronic diarhoea

A

are they failing to thrive?

yes = CF, coeliac, lactose intolerance

No = overflow constipation, toddler diarrhoea

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121
Q

how to manage toddler diarhoea

A

fibre down
sugar down
fat up

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122
Q

gastroenteritis with eosinophilia

and Tx

A

giardiasis

metronidazole

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123
Q

Cows milk protein intolerance vs lactose intolerance

A

milk protein intolerance is an ALLERGY, so comes with atopic individuals. these people can’t have it anymore and symptoms subside after cessation.

lactose intolerance is common after gastroentertisis (when superficial lactase enzymes are stripped from bowel). these take a break from lactose but can return after Sx subside.

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124
Q

prevalence and incidence of coeliac

A

1%

1 in 3000

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125
Q

histopathology of coeliac

A

<3:1 villus height:crypt depth ratio with lymphocyte invasion

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126
Q

how and when does coeliac usually present

A

CLASSICALLY

8-24 months

faltering growth, abdo distension, buttock wasting, abnormal stools, irritable

ATYPICALLY

later in life with mild non specific GI Sx and low growth or anaemia.

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127
Q

screening for coeliac

A

anti TTG

EMA (endomysial antibodies)

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128
Q

does screening diagnose coeliacs?

A

no, you need small intestine biopsy

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129
Q

treatment of coealic

A

remove wheat, rye, barley

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130
Q

complications of coealic (3)

A

Enteropathy associated T-cell lymphoma
Small bowel lymphoma
ulcerative jejunitis

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131
Q

management of crohns and UC

A

o Crohn’s disease:
♣ First line:
- Immunosuppression (steroids, azathioprine)
- Anti-inflammatory (5-ASA a.k.a. mesalazine)
♣ Second line:
- Add other immunosuppressants (mercaptopurine, methotrexate + folate)
♣ Third line:
- Add anti-TNF biologic (infliximab, adalimumab)
♣ Surgery may be needed at any stage if there is perforation, obstruction or toxic megacolon.
o Ulcerative colitis:
♣ First line:
- Topical 5-ASA (suppository/enema)
- Oral 5-ASA or steroids
♣ Second line:
- Add anti-TNF biologic (infliximab, adalimumab)
♣ Colectomy can be needed at any stage in fulminant or very refractory disease

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132
Q

management of longstanding simple constipation

A

1st line = stool softners (macrogol laxative)

2nd line = stimulant (Senna)

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133
Q

diagnosis of Hirschsprungs

A

rectal biopsy shows absence of ganglion cells with presence of large acetylcholinesterase positive nerve trunks

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134
Q

management of Hirschspurng

A

in the 75% of cases it is localised to dial bowel:

  • cut it out and form colostomy
  • anatomise innervated bowel to anus later on
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135
Q

does hepatitis cause conj or unconj hyperbilirubinaemia

A

conj

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136
Q

choledocal cyst

A

dilation og bile duct from gall bladder to duodenum causing a pseudo-obstruction and jaundice

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137
Q

what does jaundice <24hrs mean

A

probs haemolysis and serious cause (rhesus, ABO, G6PD, HS)

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138
Q

what does jaundice 2d-2w mean

A

physiological or breast milk jaundice probs

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139
Q

complication of kernicterus

A

cerebral palsy
learning difficulties
cerebral deafness

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140
Q

complications of chronic liver disease

A

Nutrition (fat malabsorption, loss of fat soluble vitamins ADEK)
Pruritus
Encephalopathy due to ammonia
cirrhosis/portal hypertension (ascites, varices, spelnomeglay)

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141
Q

what is biliary atresia

A

progressive fibrosis and obliteration of biliary tree. unknown aetiology but causes chronic liver failure within 2y untreated.

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142
Q

presentation of biliary atresia

A

mild jaundice
pale stools
faltering growth
hepatosplenomegaly (portal hypertension)

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143
Q

investigations of biliary atresia

A

LFT - raised conj bili
US - contracted or absent gallbladder
ERCP - diagnostic

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144
Q

management of biliary atresia

A

surgery (palliative or liver transplant) and fat soluble vitamins supplementation

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145
Q

investigations of A1ATd

A

plasma anti trypsin is low

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146
Q

presentation of A1ATd

A

prolonged neonatal jaundice
bleeding (vitKdef)
hepatomegaly

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147
Q

what % of febrile children will have a UTI?

A

7%

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148
Q

what is the % recurrence of a UTI

A

12-30%

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149
Q

which bug for UTI is more common in boys

A

Proteus (can cause phosphate stones)

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150
Q

mild vs severe vesicoureteric reflux

A 
mild = into ureter only
severe = dilating renal pelvis and calyces as well
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151
Q

how does VUR lead to hypertension

A

reflux –> hydronephrosis –> infection will track up easily –> renal scarring –> hypertension

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152
Q

atypical UTI infection: refer or don’t refer

A

refer urgently for USS

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153
Q

when do you refer for a ‘within 6 weeks’ USS after a UTI

A

<3m

recurrent

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154
Q

glomerular vs non-glomerular haematuria

A

glomerular:

brown urine, deformed RBCs, casts +/- proteinuria

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155
Q

most common cause of haematuria

A

UTI

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156
Q

indications for renal biopsy

A

persistent proteinuria
recurrent haematuria
abnormal renal function
persistent abnormal complement

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157
Q

investigation of haematuria

A 
Urine MC&amp;S
Blood pressure
USS kidney and UT
Bloods full-panel
protein and calcium excretion/ACR
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158
Q

investigation of glomerular haematuria

A 
Urine MC&amp;S
Blood pressure
USS kidney and UT
Bloods full-panel
protein and calcium excretion/ACR

PLUS

ESR
complement levels
throat swab
hepB/C screen

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159
Q

presentation of nephritic syndrome

A 
hypertension
oliguria (AKI)
periorbital oedema/pulmonary oedema
haematuria
proteinuria
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160
Q

most common cause of nephritic syndrome

A

80% are post-infectious (group A beta haemolytic strep)

other causes are IgA, HSP, SLE

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161
Q

special investigations of post-strep Glomerulonephritis

A

C3 (low)
ASOT (high)
Anti-DNAse B (high)

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162
Q

management of post-strep Glomerulonephritis

A

penicillin 10 day

+ management of AKI

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163
Q

commonest presentations of HSP

A

rash on legs and extensor surface (urticarial then maculopapular then purpuric) = 100%

GI Sx (colicky pain, bloody diarrhoea) = 75%

Joint pain and oedema = 50%

Haematuria, oedema are less common

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164
Q

haematuria following URTI?

A

IgA nephropathy (1-2days after) in young adult males

OR

Post-streptococcal GN (1-2 weeks after) with low C3 complement

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165
Q

SLE main autoantibody

A

dsDNA

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166
Q

HUS age group

A

<5

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167
Q

Causes of HUS

A

E.coli O157

shigella

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168
Q

presentation of HUS

A

microangiopathic haemolytic anaemia
acute renal failure
thrombocytopenia

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169
Q

treatment of HUS

A

Mx is supportive
fluid
blood transfusion
dialysis

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170
Q

how do you best measure proteinuria

A

urine albumin to creatinine ratio

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171
Q

causes of nephrotic syndrome

A

MNCS (90%)

FSGS

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172
Q

complication of nephrotic syndrome

A

thrombosis (haemoconcentration and loss of antithrombin III), infection (loss of Ig in urine), hypvolaemia, hyperlipidaemia

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173
Q

management of nephrotic syndrome

A

90% respond to steroids
70% will relapse whether frequently or infrequently

penicillin prophylaxis for pneumococcus

gastric protection

immune modulators (tacrolimus, rituximab, levamisole)

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174
Q

investigation to diagnose diabetes insipidus and another to distinguish between types

A

ADH insensitivity or lack of production - cannot concentrate urine

therefore investigation is serum (high) and urine osmolality (low)

desmopressin test distinguishes

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175
Q

management of DI

A

central - give desmopressin

nephrogenic - difficult, encourage hydration

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176
Q

management of nocturnal enuresis

A

advice (reassurance, Stop punishments, use reward charts for agreed behaviour not dry nights e.g. toilet before bed)
<7 = enuresis alarm
>7 = desmopressin if short term control or alarm hasn’t worked or alarm

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177
Q

treatment of the hyperkalaemia in an AKI

A

cardiac stabiliser:
calcium gluconate

push K+ into cells:
bicarb
insulin/dextrose
salbutamol

get rid of K+:
calcium resonium
dialysis

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178
Q

treatment of the acidosis in an AKI

A

bicarb

+/- dialysis

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179
Q

stages of CKD

A 
eGFR
1 = >90
2 = 60-90
3a = 45-60
3b = 30-45
4 = 15-30
5 = <15
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180
Q

top 5 causes for CKD in children

A 
renal dysplasia +/- reflux (34)
obstructive uropathy (18)
glomerular disease (10)
congenital nephrotic syndrome (10)
PCKD (4)
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181
Q

presentation of CKD

A

mostly asymptomatic from stages 1-3

Sx include:
bone deformity (vit D)
hypertension (RAAS)
proteinuria
anaemia (EPO)
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182
Q

management of CKD

A

high calorie, low protein diet (but enough to grow well), avoiding milk as phosphate leads to 2’ hyperpara

EPO, vit D and growth hormone

dialysis/transplant in stage 5

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183
Q

definition of hypertension in children

A

> 95 centile for height age and sex

100 when born, goes up by 2mmHg every year.

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184
Q

4 renal causes of hypertension

A

scarring
vasculitis
renal failure
coarctation/renal artery stenosis –> low blood to kidneys

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185
Q

what happens if you lower BP in malignant hypertension too quickly

A

watershed infarcts

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186
Q

where does the fluid in a hydrocele come from

A

a small gap in the processus vaginalis allows peritoneal fluid in (small version of a hernia defect)

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187
Q

blue spot on testes in acute pain

A

torsion of hydatid of Morgani

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188
Q

inguinal hernias more common on right or left

A

right 3x

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189
Q

when should inguinal hernias be surgically reduced (herniotomy) if manual reduction is not possible:
prem baby
infant
child

A

prior to discharge
within a month
elective

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190
Q

when should you operate on a hydrocele in an infant/child

A

if persisting beyond 2y

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191
Q

what % of boys have an undescended testes:
newborn
by 3 months

A

5%

1%

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192
Q

what do you do if the testes are impalpable bilaterally

A

karyotype for gender

pelvic ultrasound

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193
Q

management of cryptorchidism

A

orchidopexy at 1y old

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194
Q

time window for testicular torsion

A

6hrs

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195
Q

RF for testicular torsion

A

Bell clapper deformity (no posterior attachment of the gubernaculum). It usually happens bilaterally. This is why you attach both tests to the scrotum when untorting one side.

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196
Q 
match the following
girl
boy
wolfian
mullerina
mesonephric
paramesonephric
A

girl, mullerian, mesonephric

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197
Q

at what age are the genitlia fully formed

A

12 weeks

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198
Q

management of balanatis

A

warm water cleaning
hydrocortisone cream
imidazole cream

if purulent (bacterial), use fluclox PO

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199
Q

management of balanitis xerotica obliterate (true phimosis secondary to balanitis)

A

topical steroid

circumcision

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200
Q

behcets

A

oral ulcer
genital ulcer
arthritis
(anetrior) uveitis

amongst other things

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201
Q

most common cause of cerebral palsy

A

80% is antenatal stuff:

  • cerebrovascular accident
  • cortical migration disorder
  • maldevelopment
  • congenital infection
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202
Q

what % of CP is due to hypoxic ischaemic injuries around birth

A

only 10%

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203
Q

3 types of CP and where the lesion is

A 
spastic = UMN (pyramidal or corticospinal tract)
dystonic = basal ganglia or extrapyramidal tract
ataxic = cerebellum
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204
Q

Sx of spastic CP

A

velocity dependent increased tone

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205
Q

Sx of dystonic CP

A

chorea
athetosis (Writhing movement)
dystonia (antagonistic contractions of trunk leading to twisting)

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206
Q

Sx of ataxic CP

A

poor coordination
limb hypotonia
ataxic gate

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207
Q

most common cause of dystonic CP

A

hyperbilirubinaemia from rhesus disease

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208
Q

which is the ‘worst’ type of CP in terms of GMFCS

A

dystonic CP, which is usually GMFCS 4 or 5

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209
Q

What are the GMFCS scores

A 
1 - no limitation to walking
2 - some limitation to walking
3 - walks with handheld aid
4 - self transported in wheelchair
5 - transported in manual wheelchair
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210
Q

what complications do you monitor for in CP

A 
learning difficulty
epilepsy
visual impairment
squint
hearing loss
speech disorder
behaviour disorder
undernutrition
respiratory problems
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211
Q

medication for spasticity in CP

A

baclofen

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212
Q

medication for dystonia in CP

A

trihexyphenidyl

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213
Q

2nd line for dystonia and spasticity in CP

A

botulinum toxin

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214
Q

surgery option for CP

A

rhizotomy - cutting part of the tracts in the spine

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215
Q

which members of the MDT are used in managing CP and why

A

physio - improve family handling of child, advise activities to prevent complications
OT - equipment
dietician
SALT - swallow in early days and language therapy later
Psychologist

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216
Q

age range for febrile convulsions to occur

A

6m-6y (peak is 18m)

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217
Q

key question to ask in a seizure history in a child

A

fever

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218
Q 
If the following area has a seizure, what Sx will there be?
frontal
temporal
occipital
parietal
A 
frontal = motor
temporal = aura, dejavu, lip smacking, plucking
occipital = vision
parietal = sensory
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219
Q

only motor Sx in absence seizure?

A

eyelid flicking

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220
Q

remission rate of absence epilepsy

A

80%

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221
Q

Mx of absence epilepsy

A

sodium valproate/ethosuximide

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222
Q

how does CECTS present

A

childhood epilepsy with centre-temporal spikes a.k.a benign rolandic epilepsy:

  • focal onset seizure
  • 1/2min, often nocturnal and wake child with gurgling sound
  • speech arrest and hypersalivation
  • hemifacial motor seizure
  • 50% progress to gen ton clon
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223
Q

Mx of CECTS/benign rolandic epilepsy

A

very well managed by making sure child has enough sleep. sleep deprivation is a big trigger. unlikely to ever go away though.

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224
Q

typical age for:
absence epilepsy
CECTS
Juvenile myoclonic epilepsy

A

2-10
7-10
10-20

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225
Q

presentation of juvenile myoclonic epilepsy

A

myoclonic jerks on awakening (often in morning) in 10-20yo

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226
Q

investigations in epilepsy

A

ECG (don’t miss arrhythmia like long QT)
EEG
structural imagine: MRI, CT
functional imaging: PET, SPECT

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227
Q

1st line absence epilepsy

A

sodium valproate

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228
Q

1st line tonic clonic epilepsy

A

sodium valproate

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229
Q

1st line myoclonic epilepsy

A

sodium valproate

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230
Q

1st line focal epilepsy

A

carbamazepine or lamotrigine

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231
Q

2nd line epilepsy

A

ethosuximide, lamotrigine, clobazam, levetiracetam, topiramate, gabapentin

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232
Q

can children drive if they have epilepsy?

A

1 year seizure free

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233
Q

when can you stop antiepileptics

A

2 year seizure free

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234
Q

management of status epilepticus

A 
0min = ABCDE, O2, DEFG
5min = IV lorazepam, buccal midazolam, rectal diazepam
15min = IV lorazepam
25min = phenytoin (or phenobarbitone)
45min = thiopental rapid sequence induction
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235
Q

what makes a febrile convulsion ‘complex’?

A

if it is focal as opposed to generalised tonic clonic
>15m
twice+ in same illness

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236
Q

do febrile convulsions increase chance of epilepsy?

A

simple –> no

complex –> slightly

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237
Q

advice for parents after febrile convulsion

A

1) can’t prevent with antipyretics
2) place in recovery position
3) try to film it
4) call 999 after 5 mins

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238
Q

parixymasal disorder

A

page 216

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239
Q

what is a breath holding attack and when do they resolve by

A

resolves by 18 months

precipitated by crying/temper tantrum, child takes deep breath and stops breathing, becoming deeply acyanotic, floppy and can have clonic jerks. it lasts no more than a few minutes and there is no post-ictal phase

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240
Q

management of breath holding attack

A

reassurance

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241
Q

cause of vasovagal syncope

A

hypotension and decreased cerebral perfusion

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242
Q

how do vasovagal syncope episodes recover

A

by lying flat

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243
Q

what is a reflex anoxic syncope/seizure

A

sudden unexpected pain or fear causes a vaguely mediated bradycardia –> limp and falls to ground, pale, clonic jerks. lasts 1 minute with rapid recovery

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244
Q

Mx of reflex anoxic seizure

A

reassurance

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245
Q

what are parasomnias with examples

A

Parasomnias are a category of sleep disorders that involve abnormal movements, behaviors, emotions, perceptions, and dreams that occur while falling asleep, sleeping, between sleep stages, or during arousal from sleep.

night terrors
sleepwalking
sleedtalking
confusional arounsals
narcolepsy
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246
Q

night terror vs night mare

A

terror = early in night in stage 4 sleep, children confused, disorientated but still asleep, no recollection, more distressing to watch

nightmare = late in night in REM, children then wake up and are aware of having a bad dream, distressing for child

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247
Q

by what age should there no longer be a head lag when pulling up a child to sitting

A

1 months

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248
Q

what question do you ask yourself when looking at a child with a motor disorder

A

Central or peripheral

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249
Q

once you’ve figured out a child has a central motor disorder, what’s the next question you ask yourself?

A 
motor cortex (weakness)
or
basal ganglia (chorea, athetosis=writhing, movement initiation)
or
cerebellum (DANISHP)
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250
Q

once you’ve figured out a child has a peripheral motor disorder, what’s the next question you ask yourself?

A

anterior horn cell (signs of denervation:weakness, loss of reflexes, fasciculations, wasting)
or
neuromuscular transmission problem (diurnal worsening as ACh depletes)
or
primary muscle disease (proximal muscle wasting)
or
peripheral neuropathy (specific pattern)

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251
Q

Gower’s sign

A

using hands and knees to stand up >3yo = duchenne sign

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252
Q

investigation for peripheral motor disorder

A 
EMG
nerve biopsy
muscle biopsy
nerve conduction study
plasma CK
DNA testing
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253
Q

mutation in spinal muscular atrophy

A

AR mutation of SMN1 gene for anterior horn cells

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254
Q

SMA aka?

A

spinal muscle atrophy aka Werdnig-Hoffman disease

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255
Q

prevalence of spinal muscle atrophy

A

1 in 6000 (2nd most common neuromuscular disease in UK)

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256
Q

What is SMA

A

progressive weakness and wasting of skeletal muscle cells due to degeneration of LMN in anterior horn of spinal cord

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257
Q

types of SMA

A

0 - survive a few months
1 - flaccid paralysis, absent reflexes, weak cry, poor suck, never sit. death from respiratory failure by 1y
2 - present 3-15m. can sit but never walk
3 - present after 1 y. can walk

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258
Q

in what is the mutation for charcot marie tooth disease

A

myelin gene

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259
Q

inheritance of most common mutation of charcot maize tooth disease

A

CMTA.
2/3 AD inheritance
1/3 de novo

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260
Q

how does charcot marie tooth disease present

A

symmetrical slowly progressive distal muscle wasting (e.g. foot drop). lower limbs more affected than upper limbs

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261
Q

how do you diagnose charcot marie tooth disease

A

nerve conduction study

nerve biopsy = onion bulb (due to attempts at remyelination)

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262
Q

how does Guillain barre syndrome present

A

ascending symmetrical weakness 2-3 weeks post-infection (URTI or campylobacter gastroenteritis) that gets worse over 2-3 weeks. if left long enough, will stop diaphragm and need to ventilate

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263
Q

management of GBS

A

supportive

immunoglobulin can help

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264
Q

prognosis of GBS

A

90% recover, but can take up to 2 years.

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265
Q

LMN vs UMN facial palsy

A

upper spares upper

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266
Q

management of bells palsy

A

investigate for lyme disease and HSV

if HSV–>acicilovir

lubricate eye and wear patch if can’t close eye

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267
Q

cause of myaesthenia gravis

A

antibodies to nACh receptors in NMJ

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268
Q

presentation of myasthenia gravis

A 
after 10yo
eye movement problem
ptosis
loss of facial expression
proximal muscle weakness
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269
Q

diagnosis of myasthenia gravis

A

improvement after neostigmine and identification of antibodies in blood

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270
Q

management of myasthenia gravis

A

neostigmine
immunosuppressive therapy
plasma exchange in crisis

271
Q

inheritance of duchenne

A

X-linked recessive

but 1/3 are de novo

272
Q

gene that is mutated in duchenne

A

dystrophin - connects muscle fibre to extracellular matrix - when mutated there is myofibre necrosis

273
Q

incidence of duchenne

A

1 in 3000-6000 males

274
Q

presentation of duchenne

A 
waddling gait
language delay
towers sign
psudoheypertrophy of calves
leaning difficulties
clumsiness
275
Q

usual onset of duchenne

A

3-5

276
Q

Becker’s muscular dystrophy

A

like duchenne, but not as bad a mutation so onset is later (11) and not as severe

277
Q

life expectancy with duchenne

A

20s - because as breathing muscles become affected you get infections. also cardiomyopathy.
no longer ambulant by 10-14

278
Q

presentation and age for dermatomyositis

A 
5-10y
symmetrical proximal muscle weakness (CK up)
heliotrop rash
gottron papules
fever
raised CRP/ESR
279
Q

management of dermatomyositis

A

2yrs corticosteroids
immunosuppression
physio

280
Q

how does friedrich ataxia present

A 
similar to charcot marie tooth:
worsening ataxia
dysarthria
distal wasting of limbs
absent reflexes
death by 40-50
281
Q

what is friedrich ataxia caused by

A

AR mutation of FXN gene –> lack of frataxin protein

282
Q

headache red flags

A 
early morning headaches
waking up at night
worse headache ever had
rapid onset
neck stiffness
TMJ pain
rash
focal neurological signs
increasing head circumference
altered conscious level
personality change
worse lying down
283
Q

migraine more common in boys or girls

A

boys>girls

after puberty its women>men

284
Q

how do cluster headaches present

A

non pulsatile severe behind the eye pain. can cause unilateral redness/swelling of eye. attacks occur in clusters a few times a day for a period of weeks

285
Q

how do you manage cluster headaches

A

triptans for acute attack

Ca blockers for prevention

286
Q

headache type in raised ICP

A

worse lying down
worse in morning
wakes you up
morning vomiting

287
Q

Mx of raised ICP

A

ABCDE
elevate to 25 degrees to help drainage
intubate, mannitol, refer to neuro centre if severe

288
Q

what brain bleed is classic in NAI (non-accidental injury)

A

subdural

289
Q

most common cause of meningitis

A

viral (enterovirus, EBVm adenosine, mumps)

290
Q

bacterial causes of meningitis:

0-3m

A

GBS
Ecoli
Listeria

291
Q

bacterial causes of meningitis:

3m+

A

Neisseria meningitides
strep pneumonia
(Haemophilis as well if <6)

292
Q

kernig vs brudninski

A 
kernig = hamstring stretch
brud = neck flexion causes knee flexion
293
Q

CI to LP

A
  • focal neurology
  • coagulopathy
  • infection at LP site
  • signs of raised ICP
  • thrombocytopenia
  • cardiorespiratory instability
294
Q

what is the fluid challenge amount given to children

A

20ml/kg

295
Q

what antibiotics are used

A 
<3m = cefotaxime
>3m = ceftriaxone

give cipro to rifamp to household contacts

296
Q

what is X-linked adrenoleukodystrophy a disease of

A

peroxisome enzyme defect, leading to an accumulation of long chain fatty acids. it causes a neurodegenrative disorder

297
Q

what is wilsons disease

A
  • AR mutation of Chr 13
  • Leads to abhorrent storage of copper in many organs –> liver failure, psychosis, extrapyramidal signs
  • Presents in childhood around 12yo
298
Q

how is wilsons disease diagnosed

A

Diagnosed by reduced copper/caeruloplasmin in blood and urine (as it’s all in the liver) or by liver biopsy

299
Q

management of wilsons disease

A

low copper diet and penicillamine (copper chelation)

300
Q

diagnosis of type 1

A

> 1 neurofibroma
6 cafe au lait spots
other things like axillary freckling, optic glioma, lisch nodule, relatives

301
Q

mutation in neurofibromatosis

A

AD mutation of NF1 gene, but 50% have a de novo mutation

302
Q

what is neurofibromatosis associated with

A

MEN syndromes

303
Q

features of tuberous sclerosis

A

depigmented ash leaf shaped patches

angiofibrimata in butterfly distribution on bridge of nsoe

304
Q

what is sturge weber syndrome

A

a neurological condition where you get:

  • mental retardation
  • seizures
  • port wine stain in trigeminal distribution
  • glaucoma

effectively, it is a birthmark (capillary malformation) that affects the skin and brain

305
Q

4 developmental domains

A

fine motor
gross motor
hearing and language
social

306
Q

investigations for developmental delay

A 
urine tests for metabolic problems
TFTs
chromosome analysis
CGH array
exome sequencing
imaging (MRI)
307
Q

fever + change in personality

A

encephalitis

308
Q

catergories for causes of anaemia

A

Don’t make enough
- iron def, folic acid def, chronic renal failure

RBCs get destroyed
- hereditary spherocytosis, G6PD, thalassaemia, sickle, immune things like haemolytic disease

Blood loss
- fetomaternal bleeding, meckel diverticulum, vWF disease

309
Q

what does low reticulocyte mean

A

red cell production issue

310
Q

why os bilirubin helpful when looking at cause of anaemia

A

tells you if haemolysis is the problem

311
Q

investigation for sickle cell and thalssaemia?

A

Hb electrophoresis

312
Q

microcytic
normocytic
macrocytic
definitions

A

micro <83 fL
normal
macro >96 fL

313
Q 
Micro, normo or macro?
iron def
B12/folate
haemolysis
thalaseamia
chronic disease
hypothyroidism
alcohol excess
myelodysplasia
A 
micro
macro
normo/macro
micro
micro/normo
macro
macro
macro
314
Q

iron def anaemia:
FBC
blood film
ferritin

A

microcytic, hypochromic anaemia with low ferritin

315
Q

if no improvement in iron def anaemia, what should you investigate for

A 
coeliac
mockers diverticulum (chronic bleeding)
316
Q

causes of red cell aplasia (anaemia)

A

parvovirus B19

diamond-blackfan congenital anaemia

317
Q 
red cell aplasia:
reticulocyte count
bilirubin
Coombs test
red cell precursors in bone marrow
A

Low
Normal
Negative for Abs
Absent

318
Q

inheritance of hereditary spherocytosis and G6PDd

A

AD (northern european children)

G6PD = X-linked (African&Mediterranean boys)

319
Q

How can HS and G6PD present

A

neonatal jaundice
gallstones
haemolysis after infection

320
Q

Ix for HS and G6PD

A 
HS = spherocytes on blood film
G6PD = G6PD activity
321
Q

incidence of sickle cell

A

1 in 2000

322
Q

inheritance of sickle cell

A

AR

323
Q

mutation in sickle cell

A

glu to val point mutation to form HbS chain

324
Q

presentation of sickle cell

A

i. Acute – splenic crisis, stroke, venous thrombus, priapism, acute chest syndrome, PE.
ii. Chronic – gallstones, blindness, kidney failure, leg ulcers, bony pain
iii. Hyposplenism causing infection

325
Q

Sickle:
Hb
reticulocytes
LDH

A

Low
High
High

326
Q

emergencies in sickle cell?

A

Acute chest syndrome
A vaso-occlusive crisis of pulmonary vasculature pulmonary infiltrate, visible on a chest X-ray. Requires close monitoring and potentially a transfusion to the % of HbS

Neurological deficits
Probably a stroke. Difficult to be picked up as these patients don’t present like usual stroke victims who are old + atheromatous. Needs usual stroke care and a transfusion to the % of HbS

Infection
Encapsulated bacteria pose a big risk to sickle patients who have badly functioning spleens, especially in children. Prophylactic penicillin is used to prevent this

Acute splenic sequestration
Spleen rapidly expands and Hb drops rapidly

327
Q

when to transfuse in sickle cell

A

in the 4 emergencies

328
Q

long term medication for sickle cell

A

hydroxycarbamide

329
Q

types of beta thalassaemia

A 
carrier = one normal, one faulty beta haemoglobin gene. can still make HbA (=alpha+beta)
intermedia = both faulty but still working. variable Sx.
major = no functioning HbA, will die if untreated
330
Q

clinical features of beta thalasaemia

A
  1. Normal foetal life (as and genes are ok so HbF can be made)
  2. Anaemia in first few months of life (as HbA take sover)
  3. Jaundice
  4. Medullary hyperplasia (as bone marrow tries desperately to make RBCs) leading to:
    i. Facial changes due to meduallry hyperplasia and frontal bossing
    ii. ‘Hair on end’ appearance of cranium due to medullary expansion
  5. Splenomegaly/hepatomegaly (due to the extra-medullary haematopoiesis)
331
Q

Mx of B thal

A
  1. Life long regular blood transfusions
  2. Iron chelation (to prevent iron overload from transfusions) = desferrioxamine
  3. Folic acid
  4. Bone marrow transplantation in early life
332
Q

incidence of leukaemia

A

3 per 100,000

333
Q

types of leukaemia in children

A

80% ALL

20% AML

334
Q

types of ALL

A

75% common

15% T-cell

335
Q

peak incidence of ALL

A

2-5

336
Q

Sx and duration of onset of Sx in ALL

A

over several weeks:

  • general malaise and anorexia
  • bone marrow infiltration (anaemia so pale and tired, neutropenia so infections, thrombocytopaenia so bruising petechiae and nose bleeds)
  • hepatosplenomegaly and lymphadenopathy as RES tries to take over blood production.

acutely as DIC

337
Q

prevention of tumour lysis syndrome?

A

allopurinol: prevents uric acid production as all the cells break down

338
Q

investigations for leukaemia

A 
FBC
blood film
bone marrow examination
clotting screen (?DIC)
CXR (?mediastinal disease)
LP (?CSF disease)
Cytogenetic testing
339
Q

management for leukaemia

A

correct abnormalities:

  • RBC transfusion
  • platelets

Chemotherapy:

  1. Multidrug chemotherapy in 3 phases:
    i. Induction phase – 3 months’ intense chemotherapy to induce remission
    ii. Consolidation phase – 4 months’ intense chemotherapy to consolidate the remission
    iii. Maintenance phase – 2 years less intense chemotherapy to maintain remission
  2. Some patients get a transplant
340
Q

bad prognostic indicators for leukaemia

A 
age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex
341
Q

prognosis in ALL at 5 years

A

85%

342
Q

prognosis of AML at 5 years

A

60-70%

343
Q

bleeding:
muscles and joints
vs
mucocutaneous

A

haemophilia (coagulation cascade problem)

platelet disorders and vWF (primary haemostats and platelet plug formation)

344
Q

inheritance of haemophilia

A

X-linked recessive

345
Q

types of haemophilia

A 
A = factor 8 deficiency
B = factor 9 deficiency
346
Q

when does haemophilia present

A

end of first year of life when they start crawling/walking/falling

347
Q

what is the severity spectrum with haemophilia

A

severity depends on how bad the mutation to the gene is:
severe = sponteneous bleed
moderate = bleed after minor trauma
mild = bleed after surgery

348
Q

what will the following show for haemophilia:

  • aPTT
  • PT
  • F8&9
  • FBC
A
  • long
  • normal
  • low depending on type a or b
  • normal
349
Q

which factors are involved with:

  • intrinsic pathway
  • extrinsic pathway
  • common pathway
A
  • extrinsic = F7
  • intrinsic = F8, 9, 11
  • common = F10 and others
350
Q

what does aPTT and PT test

A

activated PTT = intrinsic (8, 9, 11)

PT = extrinsic (7)

both = common

351
Q

treatment for haemophilia in general

special treatment for haemophilia A

A

recombinant factor when bleeding
avoid aspirin, NSAIDs and IM injections

Hameophilia A can also have desmopressin, which releases endogenous stores of F8

352
Q

what does vWF do

A

it lives in the serum and when there is an injury it binds to the exposed collagen. it then also binds to platelets in the plasma and activates them forming the primary platelet plug

it also stabilises F8 in the plasma

353
Q

presentation of vWD

A

bruising
excessive bleeding after surgery
epistaxis or menorrhagia

354
Q

treatment of vWD

A
  1. Tranexamic acid – reduces clot breakdown (a.k.a. an anti-fibrinolytic)
  2. DDAVP/Desmopressin – this releases endogenous FVIII and vWF. This is good for if you need a short term boost.
  3. vWF/FVIII concentrate – very expensive so is a last line therapy, but has to be used before things like surgery.
355
Q

HSP more common in boys or girl

A

boys 2x

356
Q

what is ITP

A

Immune (or idiopathic) thrombocytopenic purpura (ITP) is an immune-mediated reduction in the platelet count. IgG Antibodies are directed against the glycoprotein IIb/IIIa (like abciximab) or Ib-V-IX complex.

Immune thrombocytopenia (ITP) in children
it is typically more acute than in adults
equal sex incidence
may follow an infection or vaccination
presents as petechiae, purpura, bruising,
usually runs a self-limiting course over 1-2 weeks

Chronic ITP
more common in young/middle-aged women
tends to run a relapsing-remitting course

357
Q

what do protein C and S do?

A

‘put brakes’ on the clotting cascade. so a deficiency means there’s no brake on the cascade and it is a pro-thrombotic state

358
Q

investigations for a child with a VTE

A

95% it is due to an underlying disorder, so must go looking for it:

  • protein C and S levels
  • antithrombin assay
  • PCR for factor V leiden and prothrombin mutation
359
Q

presentation of lymphoma

A 
painless lymphadenopathy over several months
B symptoms:
- night sweats
- fever
- weight loss
360
Q

hodgkin vs non-hodgkin:
histology
age

A

reed sternberg cells in hodgkin
NHL = childhood
HL = adolescence

361
Q

investigations for lymphoma

A

lymph node biopsy
bone marrow biopsy for staging
radiological assessment
bloods (LDH, FBC, urate, Ca)

362
Q

cure rate for hodgkins

A

80%

363
Q

long term complication from hodgkin

A

secondary malignancy or heart failure from radiotherapy at young age

364
Q

what is burkitt’s lymphoma

A

a high grade B cell NHL:

  • endemic (african) form is associated with EBV = in face and jaw
  • sporadic form = ileocaecal tumours
365
Q

Li-Fraumeni syndrome mutation

A

p53 germ line mutation causing early cancer (breast, leukaemia, sarcoma)

366
Q

mutation causing WIlms tumour

A

WT1

367
Q

most common solid tumour in children

A

brain

368
Q

what does vomiting with no diarrhoea raise alarm bells of

A

brain tumour

369
Q

key feature of medulloblastoma

A

cerebellar signs

370
Q

key feature of astrocytoma

A

supratentorial signs: seizure, hemiplegia, focal signs

371
Q

from what do neuroblastoma arise?

A

neural crest tissue in adrenal medulla and sympathetic nervous system (NOT BRAIN)

372
Q

features of neuroblastoma

A 
abdominal mass
pallor, weight loss
bone pain, limp
hepatomegaly
paraplegia
proptosis
373
Q

key investigation in neuroblastoma

A

urine catecholamines are raised:

raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels

374
Q

what is Wilms tumour

A

a nephroblastoma = cancer arising from embryonal renal tissue

375
Q

commonest presentation of wilms tumour

A

abdominal mass

+/- haematuria

376
Q

Tx of Wilms

A

chemo + nephrectomy

80% cure

377
Q

age group for bone tumours

A

after puberty

378
Q

M:F in bone tumours

A

M > F

379
Q

presentation of bone tumours

A

persistent localised bone pain

380
Q

2 types of bone tumours and most likely place to arise

A

Osteosarcoma (knee or shoulder)

Ewing’s (femoral diaphysis or hip)

381
Q

prevalence in UK of T1DM

A

1 in 500

382
Q

name a:
rapid
short
intermediate acting insulin

A

novorapid
humulin-S
humulin-I

383
Q

what type of insulin regime do children start on?

A

basal bolus

384
Q 
how do the following affect blood glucose?
illness
alcohol
anxiety/excitement
stress
menstruation
hot weather
A 
up
down
down
up
up
down
down
385
Q

long term complications of diabetes

A 
macrovascular = HTN, CVD, CHD
microvascular = retinopathy, nephropathy, peripheral neuropathy
386
Q

catergories for osce station looking at ‘assessing a child with diabetes’

A 
assess diabetes
- symptoms
- complications
assess impact on life
- school
- growth and development
- psychological
387
Q

biochemical cutoffs for DKA

A

sugar >11.1
blood ketones >3
VBG <7.3 +/- bicarb <15

388
Q

treatment of DKA

A

1) ABCDE
2) rehydrate with 0.9% saline
3) insulin infusion (+ add 5% glucose when <14mmol/L)
4) correct potassium
5) later, establish PO fluids and SC insulin. identify cause of DKA

389
Q

causes of hypoglycaemia in infancy

A

transient neotatal hypoglycaemia

persistent hypoglycaemic hypoerinsulinism of infancy

390
Q

causes of hypoglycaemia after neonatal period

A

fasting hypoglycaemia:

  • with insulin excess (overtreatment, insulinoma)
  • without insulin excess (hormone def, liver disease, inborn errors of metabolism/ GSDV)

Non-fasting hypoglycaemia:
- maternal diabetes, galactosaemia, fructose intolerance

391
Q

Tx of hypoglycaemia

A 
PO glucose
Glucagon injection (if LOC)
IV glucose (if glucagon doesn't work)
392
Q

what is McArdle disease

A

most common type of glycogen storage disorder (i.e. inborn error of metabolism that causes fasting hypoglycaemia without insulin excess). GSD V. characterized by exercise intolerance relieved by rest

393
Q

what does RAAS do to:

  • BP
  • Na
  • K
A

increases BP
increases Na
decreases K

394
Q

CAH cause

A

21-a-hydroxylase deficiency in 90% of cases. This leads to:

  • no aldosterone
  • no cortisol
  • buildup of 17OHP substrate that shunts towards making too much testosterone
395
Q

CAH presentation

A
  • virilisation of external genitalia
  • salt loss adrenal crisis in 1-3 weeks
  • tall stature, muscular build, acne
396
Q

diagnosis of CAH

A

high 17OHP in blood

397
Q

management of CAH

A
  • lifelong glucocorticoids
  • lifelong mineralocorticoids
  • ?surgery to correct genitalia
398
Q

What is addisons disease

A

primary adrenal insufficiency, often caused by AI destruction of adrenals

399
Q

presentation of addisons:

  • infants
  • older
A

infants: salt loosing crisis (lowNa, highK, met acidosis)
older: lethargy and pigmentation

400
Q

diagnosis of addisons

  • plasma cortisol
  • ACTH
  • synacthen
A
  • low cortisol
  • high ACTH
  • cortisol stays low on synacthen test
401
Q

Mx of adrenal crisis

A

IV saline
glucose
hydrocortisone

402
Q

Ix of cushings syndrome:

  • dex suppression
  • 24hr urine cortisol
A
  • dex at night doesn’t suppress cortisol at 9am next morning

- high urinary cortisol

403
Q

what type of T3 do fetuses make

A

reverse T3 = inactive derivative

404
Q

incidence of congenital hypithyroidism

A

1 in 4000

405
Q

causes of congenital hypothyrpidism

A

1) iodine deficiency (most common worldwide but not in UK)
2) maldescent of thyroid from tongue to larynx
3) pituitary dysfunction
4) inborn error of thyroid syntheiss

406
Q

what is a Guthrie test

what it looks for in cong hypothy

A

the neonatal heelprick test done when babies are 5d old

raised TSH

407
Q

Mx of cong hypothy

A

lifelong thyroxine from 2-3 weeks of age (otherwishe neurodevelopment will be very slow)

408
Q

Mx of hyperthyroidism

A

beta blocker for Sx
carbimazole = pro-drug that blocks thyroid peroxidase enzyme that puts idone on thyroglobulin to make T3 and T4
propiothyiouriaciol = blocks T4 to T3 conversion

block (radiotherapy thyroidectomy) and replace (thyroxine)

409
Q

most common inherited metabolic disorder

A

phenylketonuria and MCADD (1 in 10,000)

410
Q

Presentation and management of:

phenylketonuria

A

learning difficulties, seizures, microcephaly

phenylalanine restricted diet

411
Q

Presentation and management of:

MCADD

A

hypoglycaemia if fasting

don’t fast

412
Q

Presentation and management of:

glutaric aciduria type 1

A

macrocephaly with encephalopathic crisis 6-18m resulting in dystonic-dyskinetic movements

specialist diet and dialy carnitine

413
Q

Presentation and management of:

isovaleric acidaemia

A

metabolic acidosis

low [protein diet and carnitine/glycine

414
Q

Presentation and management of:

homocystinuria

A

marfanoid appearance, lens dislocation, osteoporosis, VTE

low protein diet and pyridoxine/folate

415
Q

maple syrup urine disease

A

maple syrup smelling urine and encephalopathy in first week of life

low protein diet

416
Q

causes of ambiguous genitalia:

  • if female
  • if male
A
  • CAH, prenatal exposure to androgens

- 5areductase deficiency (no DHT from testosterone so can’t virilise), androgen insensitivity, impaired testes

417
Q

how long is the growth phase for an infantile haemangioma

A

9m

418
Q

how long do haemangiomas take to involute

A

50% by 5y

70% by 7y

419
Q

Mx of haemangioma

A

Conservative

propranolol if complications such as obstructed vision, ulceration, pain, bleeding or disfigurement

420
Q

What is PHACES

A 
variation of infantile haemangiomas including:
Posterior fossa defect
Haemangioma
Arterial abnormalities
Cardiac abnormalities
Eye abnormalities
Sternal clefting
421
Q

what is sturge weber syndrome

A

port wine stain (a flat haemangioma) in trigeminal distribution + neurological problems (seizure, developmental delay) + eye problems (blindness, retinal detachment, glaucoma)

422
Q

incidence of port wine stain

A

1 in 300

423
Q

natural progression of port wine stain

A

darken and thicken with time. permanent.

424
Q

what are port wine stains caused by

A

slow flow capillary vascular malformations

425
Q

what is an exanthem

A 
a rash that appears abruptly affecting several areas of the skin simultaneously.
6 classic ones are:
measles
scarlett fever
rubella
staph scalded skin
erythema infectiosum/slapped cheek
roseola
426
Q

caused of measles

A

paramyxovirus/morbillivirus

427
Q

presentation of measles

A 
conjunctivitis
koplik spots
cough
THEN
widespread maculopapular/morbilliform rash
428
Q

complications from measles

A

encephalitis
pneumonia
otitis media
subacute sclerosing panencephalitus 7 years later (like dementia)

429
Q

scarlett fever cause

A

group A strep

430
Q

scarlett fever presentation

A

strawberry tongue
sandpaper rash
circumoral pallor

431
Q

management of scarlett fever

A

penicillin

432
Q

cause of rubella

A

rubella virus

433
Q

presentation of rubella

A

prodrome, e.g. low-grade fever
rash: maculopapular, initially on the face before spreading to the whole body, usually fades by the 3-5 day
lymphadenopathy: suboccipital and postauricular
Forschemer’s spots (petechiae on hard palate)

434
Q

complications of scarlett fever

A

otitis media
pneumonia
glomerulonephritis
rheumatic fever

435
Q

complications of rubella

A

congenital rubella syndrome in 1st trimester

436
Q

diagnosis of rubella

A

unlike the other exanthems, this needs serology

437
Q

SSSS cause

A

S. aureus toxins

438
Q

SSSS presentation

A

Erythema following by blistering with Nikolsky’s sign.
No mucosal involvement
Pain, fever, shock (from fluid loss)

439
Q

Mx of SSSS

A 
IV Abx
emolients
analgesia
dressings
fluid and temperature balance
440
Q

erythema infectiosum AKA and cause

A

a.k.a 5th disease a.k.a slapped cheek syndrome

caused by parvovirus B19

441
Q

presentation of erythema infectiosum

A

slapped cheek for 4 days with circumoral sparing

mesh like net red appearance across buttocks and trunk after a week

442
Q

roseola infantum presentation and cause

A

6m old
sudden onset fever which then GOES away AND THEN maculopapular rash appears

HHV6 and HHV7 (think that it is 6th disease)

443
Q

causes of bullous and non bullous impetigo

A

non-bullous - S. aureus and Strep pyogenes (group A)

bullous - S. aureus

444
Q

Mx of periorbital or orbital cellulitis

A

High dose ceftriaxone

445
Q

what is a herpetic whitlow

A

painful erythematous oedematous white pustules on site of broken skin. typically fingers. caused by autoinnoculation

446
Q

chicken pox cause

A

VZV (HHV-3)

447
Q

how many herpes viruses are there and what are they

A 
8:
HSV1 - lip and skin lesions
HSV2 - genital lesions
VZV - chicken pox
CMV
EBV
HHV6 - roseola
HHV7 - roseola
HHV8 - kaposi
448
Q

after how long should new lions stop arising gin chicken pox

A

10 days, after which you worry of immunocompromise

449
Q

cause of warts

A

human papillomavirus

450
Q

how long do molluscum take to go away

A

up to a year

451
Q

what is a kerion

A

a severe pustular ringworm (dermatophyte) infection

452
Q

what is seborrhoeic dermatitis and aka

A

aka cradle cap

scales form thick yellow adherent layer on scalp of <3m baby

453
Q

Mx of cradle cap

A

emolients
clears scales using ointments containing sulphur and salicylic acid
?topical steroid

454
Q

What is nappy rash

A

an irritant contact dermatitis from urine/diarhoea on skin

455
Q

where does nappy rash occur

A

Nappy area, but spares the flexures/skin creases

456
Q

Mx of nappy rash

A

emollient + ?steroid

457
Q

genetics in eczema

A

fillagrin null mutation

458
Q

Mx of eczema (brief)

A

soap/irritant avoidance
emollients
steroids

459
Q

what is erythema nodosum

A

submit fat inflammation causing tender red nodules on skins

460
Q

erythema multiforme appearance

A

target lesions WITH A CENTRAL RED PAPULE

461
Q

area of body most likely to be injured in a fatal childhood accident

A

head injury

462
Q

presentation of brain bleed in children: neuro or shock

A

compared to adults, relatively more shock because the sutures aren’t fused so there is less mass effect and fewer neuro signs

463
Q

management of choking

A

unconscious = CPR
conscious:
child = 5 abdo thrusts
infant = 5 back blows, 5 chest thrusts

464
Q

when to refer to burns unit

A
  • partial thickness burn >5% of - body surface area
    deep partial thickness/full thickness
  • face, ears, hands, feet, genitalia, perineum, major joint affected
465
Q

grade of burns

which grades appear dry

A

superficial = epidermis only
partial = into dermis. can be superficial or deep type depending on depth
full thickness = into subcutaneous fat and tissue

superficial and full are dry, other is moist

466
Q

when is activated charcoal useful in a poisoning

A

within 1 hour of poisoning to prevent some of it being absorbed

467
Q

Sx of paracetamol overdose

A

Early = abdo pain + vomiting

Late (12-24hr) = liver failure

468
Q

Mx of paracetamol overdose

A

Serum paracetamol
concentration, INR and LFT

IV NAC if required

469
Q

Sx of salicylate overdose

A 
Early = tinnitus + vomiting + hyperventilation (respects alkalosis)
Late = metabolic acidosis
470
Q

Mx of salicylate overdose

A

Serum salicylate

Alkalinisation of urine
Haemodialysis

471
Q

Sx of alcohol overdose

A

Hypoglycaemia
Coma
Respiratory failure

472
Q

Mx of alcohol overdose

A

Serum ETOH

Treat hypoglycaemia
Support ventilation

473
Q

what is blount disease

A

severe progression of unilateral leg bowing (genu varum)

474
Q

what measurement can be used to see whether someone with knock knees (genu valgum) is normal

A

Intermalleolar distance <8cm when standing with knees together

475
Q

pathological cause of genu valgum

A

JIA

476
Q

examination for flat feet

A

Ask to go on tip toes and observe arch

Aldo passively extend big toe

477
Q

do you refer flat feet?

A

if there is evidence of a pathological cause:

hyper mobility, contracture, tarsal coalition, JIA

478
Q

causes of in-toeing:

  • infant
  • toddler
  • child
A

metatarsus adductus
medial tibial torsion
persistent anteversion of femoral neck

479
Q

clubfoot name and deformity

A

talipes equinovarus:

  • inversion and supination of foot with plantarflexion
  • shorter foot and thin calf muscles
  • foot is fixed
480
Q

incidence and M:F of clubfoot

A

1 in 1000

male x2

481
Q

Mx of clubfoot

A

Ponsetti method = plaster casting and bracing

Severe = corrective surgery

482
Q

Deformity in talipes calcaneovalgus

A

dorsiflexion and turned out/everted

483
Q

definitions: talipes:
- varus
- valgus
- equinus
- calcaneus

A 
turned in (like inversion)
turned out (like eversion)
like plantarflexion
like dorsiflexion
484
Q

Mx of calcaneovalgus

A

self corrects with exercises

485
Q

how does tarsal coalition present

A

progressively more rigid foot, presenting in pre-adolescent years

486
Q

Pes cavus deformity

A

high arched foot.

487
Q

Pes cavus in older children: association?

A

Neuromuscular disorder (e.g friedrich ataxia, hereditary motor sensory disorder)

488
Q

how might DDH present

A

neonatal screening

Limp/abnoaml gait

489
Q

type of pain in growing pain

A 
3-12y
at night, symmetrical
NEVER at start of day
doesn't limit activities
pGALS normal
490
Q

score for hypermobility

A

beighton score

>6/9 is hypermobile

491
Q

Mx of hypermobility

A

Investigate for Marfan or Ehlers-Danlos

MDT treatment: PT/OT, orthotics, simple pain relief

492
Q

what is complex regional pain syndrome and Mx

A

non-organic dramatic MSK pain with hyperaesthesia and allodynia. Mx is PT based

493
Q

presentation of transient synovitis

A

<5yr
hip pain referred to knee with a preceeding URTI
mild fever (not really ill like septic arthritis)
improves in a few days

494
Q

Mx of transient synovitis

A

Worried about differential of septic arthritis, so do Xray, FBC, CRP, USS

If unsure joint aspirate and cultures

495
Q

Presentation of perthes disease

A

young boy 5-10

insidious onset of limp, hip/knee pain.

496
Q

Imaging in perthes disease or SUFE

A

frog leg lateral view Xray of both hips to show AVN

MRI can be helpful

497
Q

does the hip reossify in perthes disease? Any long term consequences?

A

Yes, over 18-36 months. Risk of degenrative arthritis in adult life

498
Q

Mx of perthes

A

Rest and PT to optimise hip movement

Surgery sometimes used

499
Q

Presentation of SUFE

A

hip pain and limp can be insidious or acute. In obese 10-15yr old boy.

500
Q

Mx of SUFE

A

urgent referral and surgical pin fixation

501
Q

cause of chondromalacia patellae

A

soft cartilage of patella causes pain when tightly opposed to femoral condyles (pain walking up stairs/getting up from chair)

502
Q

presentation of reactive arthritis

A

pain, swelling, redness, restricted ROM oligoarthritis FOLLOWING an infection. Usually not ill like in septic arthritis, and usually affects more than one joint.

503
Q

Mx of reactive arthritis

A

NSAIDs and reassurance

504
Q

usually place for septic arthritis

A

knee

505
Q

common age for septic arthritis

A

<2

506
Q

pathogen for septic artheitis

A

S. aureus

507
Q

Mx of septic arthritis

A

Get joint aspirate and send it off
IV Abx
Surgical washout if no improvement or if in hip
immobilise and then subsequent mobilisation

508
Q

what is JIA

A

umbrella term for different types of juvenile arthritides:

  • systemic
  • oligoarthritis (most common)
  • polyarhtirits
  • psoriatic
  • enthesitis related
509
Q

What is Still’s disease

A

systemic arthritis type of JIA where there is arthritis AND fever, rash, hepatosplenomegaly, lymphadenppathy

510
Q

in polyarthritis type JIA, who is RF+

A

10-16yo.

the 1-6yo tend to be RF-

511
Q

which JIA subtype is more common in boys

A

enthesitis related arthritis

512
Q

incidence of JIA

A

1 in 1000

513
Q

complications of uveitis

A

posterior synechiae, cataract, glaucoma, band keratopathy

514
Q

management of uveitis

A

Steroid (drops, oral, IV)
Methotrexate
Humira

515
Q

common complication of JIA

A

chronic anterior uveitis 33%

Need ophthalmological review every 3m

516
Q

management of JIA

A

Paediatric rheumatologist!

PT
Analgesia (ibuprofens/naproxen NOT opiod or COX2)
Immunosuppression (MTX - no alcohol, folate and LFT monitoring monthly - steroids or biologics)

3/12 opthalm review
encourage to do everything even during flare

517
Q

common spread type in chilren with osteomyelitis

A

haematogenous

compared to adults where its usually contiguous (e.g. trauma)

518
Q

common area for haematogenous spread in osteomyelitis

A

metaphyseal area of long bone

519
Q

presentation of osteomyelitis

A 
PAINPAINPAIN
pseudo paresis (can't move limb)
520
Q

Mx of osteomyelitis

A

several weeks of IV Abx and oral therapy afterwards. main need surgery to debride or drain

521
Q

common site of osteoid osteoma

A

spine

522
Q

what is Scheuermann disease

A

osteochrontisi of vertebral body

523
Q

what is adams forward bend test

A

ask someone with scoliosis to bend over and touch toes

  • scoliosis resolves = functional scoliosis
  • scoliosis doesn’t resolve = structural scoliosis
524
Q

what is torticollis

A

dystonic condition causing abnormal neck position

525
Q

most common cause of torticollis

A

sternomastoid tumour, which occurs in first few months of life. presents as a mobile non tender nodule in body of sternocleidomastoid muscle

526
Q

mutation in achondroplasia

A

FGFR3 AD

but 50% are new mutations

527
Q

what is cleidocranial dysostosis

A

AD disorder. absence of part or all fo clavicles and delayed closure of anterior fontanelle. can bring shoulder in from of chest and have short stature.

STRANGER THINGS BOY ACTOR

528
Q

what is arthrogryposis

A

defined as >2 congenital contractures, associated with oligohydramnios or chromosomal dosorders

529
Q

most common type of osteogenesis imperfecta and presentation

A

type 1 = AD = fractures in childhood, blue sclera, hearing loss

530
Q

Mx of OI type 1

A

bisphosphonates and fracture splinting

531
Q

presentation of type 2 OI

A

still born

532
Q

what is osteopetrosis

A

MARBLE BONE DISEASE!

carbonic anhydrase and proton pump are not working,
meaning osteoclasts cannot properly resorb bone. This means you get
extremely dense bones with high mineral content that are brittle. Also,
patients get problems with anaemia/thrombocytopaenia because there’s no space for the
haematopoietic cells!

533
Q

marfans syndrome inheritance

A

AD

534
Q

risks with marfans syndrome

A

cardiovascular degeneration of media of vessel walls resulting in dilates aortic root. need echo monitoring

535
Q

what do you physically give in active vs passive vaccination? which is longer lasting

A 
Passive = antibody. immediate effect but short lasting
Active = antigen (because then the body has to actively make an antibody)
536
Q

CI of giving vaccine

A

Don’t give to someone in acute illness

Don’t give live vaccine to immunocompromised

537
Q

how are most primary immunodeficiencys inherites

A

X linked

538
Q

SPUR for infections in immunodeficiency

A

severe
prolonged
unusual
recurrent

539
Q

what broad spectrum antibiotic should you use in children with sepsis of unknown origin

A 
<1m = cefotaxime + amox
>1m = ceftriaxone
540
Q

presentation of mumps

A

parotitis (initially unilateral and then bilateral) with fever and malaise –> otalgia + pain on eating

541
Q

Mx of mumps

A

supportive

542
Q

complicaitons of mumps

A

hearing loss
meningitis/encephalitis
orchitis (unilateral and in post-pubertal children)

543
Q

presentation of glandular fever

A 
lymphadenpathy
tonsilitis + soft palate petechiae
fever/malaise
splenomegaly 50%
rash/jaundice sometimes
544
Q

specific lab investigations for glandular fever (3)

A
  1. abnormal lymphocytes
  2. +ve mono spot test
  3. seroconversion with 3 antibodies (viral capsid, IgG and IgM EB nuclear antigen)
545
Q

what antibiotic do you avoid in EBV infection and why

A

amoxicillin –> rash

546
Q

how do enteroviruses present

A

90% nonspecific febril eillness
D&V
Rash (can be non-blanching petechial)

547
Q

is hand foot and mouth disease painful

A

yes, that’s why children present unable to eat or drink. it is a painful vesicular rash

548
Q

what is herpangina

A

vesicular and ulcerated lesion son soft palate and uvula caused by enterovirus.

549
Q

what is hand foot and mouth disease caused by

A

Picornaviridae family, most commonly:
coxsackie A16
enterovirus 71

550
Q

Most common cause of meningitis overall

A

enterovirus

551
Q

what is bornhol disease

A

pleural disease: an acute illness caused by enterovirus causing fever, pleuritic chest pain and muscle tenderness. self limiting

552
Q

MSSA Abx

A

fluclox

cipro for broader cover

553
Q

MRSA Abx

A

Vancomycin

Clindamycin

554
Q

Most common type of group A strep

A

Strep pyogenes

+ve coccus

555
Q

How does toxic shock syndrome occur?

A

S. aureus or Group A strep produces an exotoxin that acts as a superantigen. can arise uncommonly from any site of infection

556
Q

presentation of toxic shock syndrome

A

fever >39 degrees
hypotension (hence the shock)
diffuse macular rash
multi-organ dysfunction:
- mucositis, D/v, renal liver impairment, cltoiting abnormalities, low GCS
- 1-2 weeks later you get desqamation of palms, soles, fingers

557
Q

management of toxic shock syndrome

A

ABCDE in ICU
surgical debridment of obvious areas of infection
Ceftriaxone and clindamycin
IVIG

558
Q

types of nec fash

A

type 1 = mixed aerobe and anaerobe

type 2 = strep pyogenes

559
Q

risk groups for types of nec fash

A

type 1 = diabetics post op

type 2 = IVDU

560
Q

presentation of nec fash

A

intensely tender red area that is acute and rapidly progressing. in 3-5 days there will be skin breakdown and bullae, following by gangrene and anaesthesia in the area

561
Q

Mx of nec fash

A

Benzyl pen + fluclocacillin + metronidazole (to cover anaerobes)

extensive surgical debridement

562
Q

What do T lymphocytes do

A

recognize antigen using the t cell receptor in the context of the major histocompatibility complex.

alongside B lymphocytes that produce antibodies, they make up the adaptive immune system

563
Q

types of T lymphocyte and what they do

A

CD4 helper

  • uses MHCII and when with antigen releases cytokines with two types of response:
    1) Th1 = macrophage activation
    2) humoral immunity

CD8 cytotoxic
- uses MHCI and kills virally infected cells using perforins and granzymes and interferons

564
Q

how does HIV infect the CD4 cell

A

interacts with CD4 TCR alongside CCR5 coreceptor and virus can get into the cell. reverse transciptase enzyme lets virus replicate

565
Q

4 stages of HIV infection

A

Stage 1 = acute seroconversion where viral load goes up and causes a non specific viral picture

stage 2&3 = continuous turnover of viruses and cells. is asymptmatic and lasts up to 10 years but CD4 count slowly drops. some lymphadenopathy maybe.

stage 4 = AIDS. weird infections kill you due to lack of CD4 T cells

566
Q

what is hairy leukoplakia and what does it mean

A

white plaque son side of tongue. it is an AIDS defining EBV infection of the tongue

567
Q

how do you diagnose children with HIV

A 
>18m = serology to detect IgG against HIV
<18m = serology can only confirm exposure due to maternal antibodies
568
Q

how can you confirm a child is not infected if mum had HIV

A

Once postnatal antiretroviral therapy is completed –> 2x -ve HIV PCR within first 3m of life

569
Q

What levels of CD4 indicate:

  • normal
  • asymptomatic HIV
  • AIDS
  • about to die
A

> 500
200-500
<200
<50

570
Q

what is the transmission rate from mother to baby completely untreated when breastfeeding

A

25-40%

571
Q

here in the UK, what advice is given to prevent vertical transmission of HIV

A

ART during and after pregnancy
post exposure prophylaxis after birth for baby
avoid breast feeding
pre-labour caesarean if viral load is high

572
Q

cause of lyme disease

A

spirochete:

Borrelia burgdorferi

573
Q

presentation of lyme disease

A

50% have tick bite in past couple weeks

erythematous macule at site of tick bite that spreads with a bright leading edge = erythema migrans

fever, malaise, myalgia, arthralgia, lymphadenopathy

If disseminates:

  • CN palsy
  • meningitis
  • arthritis
  • myocarditis/heart block
574
Q

Mx for lyme disease

A 
<12y = amox
>12y = doxycycline

complicated = IV ceftriaxone

575
Q

what does cyclical fever every few days raise alarm bells of in someone who has recently been travelling

A

malaria

576
Q

typhoid/paratyphoid cause

A

caused by salmonella group (e.g. salmonella typhi)

577
Q

presentation of typhoid

A
  • initially systemic (fever, malaise, arthralgia)
  • relative bradycardia
  • abdominal pain, distension
  • constipation: although Salmonella is a recognised cause of diarrhoea, constipation is more common in typhoid
  • rose spots: present on the trunk in 40% of patients, and are more common in paratyphoid
578
Q

Mx of typhoid

A

Co-trimoxazole, chloramphenicol, ampicillin

579
Q

ENT wise, what are gromits used for

A

to resolve conductive hearing loss Sx in otitis media with effusion (glue ear)

580
Q

describe limitation of newborn hearing screening

A

otoacoustic emission test cochlea
audotry brainstem response tests brainstem
neither tests auditory nerve

581
Q

most common age for acute otitis mediA

A

6-12 months

582
Q

what can you do if grommets fail in children

A

adenoidectomy (remove adenoids which opens Eustachian tube on the mouth end more to allow gunk out)

583
Q

when do you give antibiotics in otitis media, and what course do you prescribe

A

5 days amox if:

  • Symptoms lasting more than 4 days or not improving
  • Systemically unwell but not requiring admission
  • Immunocompromise or high risk of complications secondary to significant heart, lung, kidney, liver, or neuromuscular disease
  • Younger than 2 years with bilateral otitis media
  • Otitis media with perforation and/or discharge in the canal
584
Q

cause of childhood obstructive sleep apnoea

A

adenotonsillar hypertrophy

585
Q

incidence of trisomy 21

A

1 in 650

586
Q

most common cause of the reason trisomies occur

A

94% is…

meiotic nondisjunction = fail to separate during anaphase of either meiosis 1 or 2. this leads to a gamete with 2x chromosome 21 for eg, which wen feritlised becomes trisomy

5% is translocation
1% is a mosaic of trisomy

587
Q

appearance of trisomy 21

A 
round face with flat nasal bridge
epicanthic folds
small mouth and protruding tongue
hypotonic
flat occipur
single palmar crease
incurved 5th finger
wide sandal gap
588
Q

% of downs with congebnital heart disease`?

A

40% AVSD

589
Q

appearance of Edwards syndrome

A 
small mouth and chin
prominent occiput
clenched fist with overlapping fingers
malformed ears
rocker bottom feet and flexed big toe
590
Q

prognosis with Edwards/pataus syndrome

A

most die in infancy

591
Q

appearance of pataus syndrome

A

small or absent eyes
cleft lip and palate
polydactyl

592
Q

presentation of turners syndrome

A 
lymphedema of hands and feet in neonate
webbed neck
spoon shaped nails
small stature
widely spaced nipples
ovarian dysgenesis (infertility)
coarctation of aorta
hypothyroidism
renal abnormalities
pigmented moles
593
Q

what can you give around puberty for a girl with turners

A

oestrogen replacement therapy to help her go through puberty

594
Q

what is wrong genetically in fragile X syndrome

A

trinucleotide repeat in X chromosome

595
Q

appearance of fragile X

A

long face
large everted ears
learning difficulty
macro-orchidism

596
Q

complications of fragile X

A

mitral valve prolapse

scoliosis

597
Q

noonan syndrome inheritance

A

AD

598
Q

presentation of noonan syndrome

A 
very similar to turners:
broad forehead
drooping eyelids
wide eye distance
short webbed neck
trident har line
pectus excavatum
short
pulmonary stenosis
599
Q

Williams syndrome presentation

A 
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
600
Q

presentation of Prader-willi

A

hypotonia
hypogonadism
obesity

601
Q

genetic investgations options from most crude to most precise

A

karyotyping
DNA PCR (analysis of small samples)
CGH array (compares sample to known array to see if chinks are added or missing)
Mutation analysis (point mutations)
Next gen sequencing (whole exome sequencing)

whole genome sequencing is reserved for research tool as it looks at introns and exons (100,000 genome project).

602
Q

% child mortality

A

4.5% (6 million per year)

603
Q

top 2 causes of child mortality <5

A

50% is infectious diseases, of which pneumonia is most common

50% is neonatal death

(if you include 1-15yo, then malignancy accounts for 24%)

604
Q

at what income level is poverty defined as

A

60% less than median income

605
Q

when does a child attain 6/6 vision

A

5 yo

606
Q

what is corneal light reflex used for

A

Detection of a squint may be made by the corneal light reflection test - holding a light source 30cm from the child’s face to see if the light reflects symmetrically on the pupils

607
Q

amblyopia?

A

lazy eye

brain and eye not moving in sync

608
Q

myopia
hypermetropa
long sighted
short sighted

A

Short = myopia (its a shorter word)

609
Q

what is astigmatism

A

refractive error of the lens meaning there is a constant, slight blur at any distance

610
Q

what is the UK healthy child programme

A

from 0-19 years, that is the overarching name for the plan for:

  • screening
  • immunization
  • developmental reviews
  • heatlh promotion/education
611
Q

4 basic human rights of child

A

from 1989 act:

  1. survival
  2. development
  3. protection
  4. participation
612
Q

4 types of abuse

A

physical (80% mother!)
sexual
emotional
neglect

613
Q

most common symptom in CFS

other Sx

A

post-exertional malaise and fatigue

cognitive dysfunction
sleep disturbance
muscle pain
joint pain
headaches
sore throat
614
Q

is there a trigger in CFS

A

yes, usually some sort of viral infection, which because of a genetic predisposition causes CFS to start

615
Q

TOTT investigation screen

A 
FBC
ESR/CRP
U/E
LFTs
CK
TTG
TFTs
ferritin
urine dip
BP
616
Q

Management of CFS

A

making diagnosis helps

advice is two fold:

SLEEP
- anchor wake up time for cortisol hit

ACTIVITY
- avoid boom bust. must pace yourself. the coloured diary charts help.

CBT/graded exercise therapy is the next step (with a specialit)

617
Q

is the pain real in chronic pain syndrome

A

YES, remember pain is an emotion not a physical thing and chronic pain syndrome is a wiring problem leading to allogynia/hyperalgesia

618
Q

definition of ADHD

A
  1. inattention
  2. hyperactivity
  3. impulsivity

must be >6m and in at least 2 settings

619
Q

screening questionnaire for ADHD

A

connors

620
Q

ways to gather information when making ADHD diagnosis

A

parental interview
conors questionnaire
school observation
school reports

621
Q

Mx of ADHD

A

non-medical

parent education
school education/special assistance/strategies
CBT

methyphenidate CNS stimulant (SE appetite down). atomoxetine can also be used.

622
Q

triad in autism spectrum disorder

A

impaired social interaction
SAL delayed development
routines/ritualistic behaviour

623
Q

diagnostic tools for autism

A

ADOS (done by SALT) = Autism Diagnostic Observation Schedule

3DI (parent questionnaire)

624
Q

is autism associated with epilepsy/seizures?

A

yes

625
Q

what is a normal IQ

A

> 80

<35 is severe learning difficulties

626
Q

are eating disorders associated with social class

A

no, but it is a problem of western society

627
Q

SCOFF questions for anorexia

A

sick - do you make yourself sick because youre too full
control - do you worry you’ve lost control of eating
one stone - have you ever lost one stone in a short space of time
fat - you you believe you are fat when others say you are thin
food - does food dominate your life

628
Q

what is lanugo hair

A

a condition characterized by excessive hair growth in certain areas of the body. Lanugo is actually a sign that the body is trying to protect itself during the starvation process by trapping in heat that muscles and fat can no longer provide.

629
Q

can you have bulimia without being underweight?

A

yes. in fact, you must have a BMI >17.5 to have bulimia nervosa. if you have <17.5, then you have BN and AN

630
Q

red flags for anorexia nervisa

A 
BMI<13
weight loss >1kg/week
T <34.5
hypotensive <80/50
HR <40
sats <92% or limbs blue
purpura
electrolyte abnormality
631
Q

what is refeeding syndrome

A

Refeeding syndrome is a syndrome consisting of metabolic disturbances that occur as a result of reinstitution of nutrition to patients who are starved, severely malnourished or metabolically stressed due to severe illness. When too much food and/or liquid nutrition supplement is consumed during the initial four to seven days of refeeding, this triggers synthesis of glycogen, fat and protein in cells, to the detriment of serum (blood) concentrations of potassium, magnesium, and phosphorus.[1][2] Cardiac, pulmonary and neurological symptoms can be signs of refeeding syndrome. The low serum minerals, if severe enough, can be fatal.

‘caused by low phosphate?’`

632
Q

mortality of anorexia nervisa

A

5-10%

633
Q

Mx of refeeding syndrome

A

vitamin B

stop refeeding and reintroduce slowly

634
Q

screen for DDH in breech baby with a normal newborn exam?

A

Yes

635
Q

cradle cap (Seb dermatitis) treatment

A 
mild = baby oil/shampoo
severe = topical hydrocortisone
636
Q

diagnosis of pertussis

A

per nasal swab

637
Q

conservative treatment for chickenpox

A

topical calamine lotion

638
Q

what syndrome is neonatal hypotonia most associated with

A

prader-willi

639
Q

average age for GOOD pincer grip

cut off age for early pincer grip

A

12 months

12 months

640
Q

elfin facies = ?condition

A

williams syndrome

641
Q

flattened philtrum = ?condition

A

foetal alcohol syndrome

642
Q

asthma, SABA not working. what’s the next step:

<5
>5

A

<5 = moderate dose ICS

> 5 = low dose ICS

643
Q

until what age is a phimosis okay?

A

2y

644
Q

how much of a fever do children with bronchiolitis have?

A

low grade (<39). If it’s >39, suspect pneumonia (esp. if focal as opposed to generalised crackles)

645
Q

Main sequelae of respiratory distress syndrome in premature baby

A

retinopathy of prematurity

renal failure

646
Q

paediatric basic life support

A 
hello hello?
can i get some help
open airway
check for breathing
5 rescue breaths (omitted in adults)
check for BRACHIAL/FEMORAL (not carotid) pulse
15:2 CPR
647
Q

when do majority of children achieve day and night urinary continence

A

3-4

648
Q

initial management of Hirschsprung’s disease

A

bowel irrigation (whilst biopsy comes back)

649
Q

Roseola infant cause, presentation, complications and ?exclusion

A 
HHV6
high fever for few days THEN rash
nagayama spots on uvula/palate
aseptic meningitis/hepatitis
exclusion not required
650
Q

management of exompahlos vs gastroschisis

A

exompahlos = bowel out of abdomen with peritoneal/amniotic covering –> staged closure with completion at 6-12m

gastroschisis = defect in abdominal wall just lateral to umbilical cord. gut out of body with no covering –> immediate correction

651
Q

do you grow out of a cows milk allergy

A

yes, by age 5, but usually by age 1-2

652
Q

management of cows milk protein allergy

A

formula fed –> use extensive hydrolysed formula (eHF), or even amino acid formula if severe and eHF doesn’t work

breast fed –> carry on breast feeding but mum to stop milk products. then use eHF from 6-12 months after breast stops

653
Q

risk factors for surfactant lung disease

A 
(prematurity)
male sex
diabetic mothers
caesarean
second born of premature twins
654
Q

turners syndrome heart auscultation sign and why

A

ejection systolic due to bicuspid aortic valve

655
Q

dietary advice for CF

A

high calorie
high fat
pancreatic enzymes every meal

656
Q

M:F in DDH

A

female 6x

other RFs are breech, FH, firstborn, oligohydramnios, macrosomia

657
Q

painless massive GI bleed in 1-2 year old?

rule of 2s?

A

Meckel’s diverticulum
2% of population
2 feet from ileocaecal valve
2 inches long

658
Q

viral wheeze age group and management

A

pre-school children

1st line = SABA + spacer
2nd line = montelukast and inhaled ICS

659
Q

contraindications for MMR (5)

A

severe immunosuppresion
allergy to neomycin
other live vaccine <4wks ago
IVIG therapy in past 3m

660
Q

what is choanal atresia?
presentation?
management?

A
  • posterior nasal airway occluded by soft tissue or bone
  • presents with episodes of cyanosis worst when feeding that disappears when crying. unilateral subtype may go unnoticed. (Remember all newborn babies are obligate nasal breathers so having a blocked nose is bad)
  • penetration procedures.
661
Q

ebsteins anomaly?
presentations?
cause?

A

tricuspid valve is lower and floppy so RA is massive and there is tricuspid regurg (pan-syslotic) and tricuspid stenosis (mid-diastolic)

caused by lithium in pregnancy

662
Q

haemophilia in a girl???

A

think about turners syndrome (only one X so susceptible to X-linked recessive)

663
Q

competent use of knife and fork age?

A

5 years

4 prongs of a fork and one knife

664
Q

distribution of atopic eczema in an infant?

A

face and trunk

665
Q

maternal cause of orofacial clefts?

A

maternal anti-epileptic use

666
Q

can you go to school with head lice

A

yes

667
Q

full name for mongolian blue spot

A

congenital dermal melanocytosis

668
Q

drawing circle

A

3 years

because you need 3 things: pen, paper and hand

669
Q

cystic hygroma vs branchial cyst

A

cystic hygroma = posterior triangle, soft, transilluminate

branchial cyst = lateral in anterior triangle. anechoic on USS.

670
Q

multiple miliary opacities in both lungs (not TB)?

A

pneumonia from chickenpox.

one of the 3 main complications of varicella:

  • pneumonia
  • encephalitis
  • disseminated haemorahgic chickenpox
671
Q

what happens if you miss DDH

A

arthritis in your 30s

672
Q

if a mum misses MMR vaccine but now wants it, what can you do

A

give one now and then in 3m

if >10 or in an outbreak&<10, 1m is ok

673
Q

fragile X heart problem

A

mitral valve prolapse

674
Q

when do you worry about undescended testes

A

review at GP @3m and make referral to surgeon

surgery by 6m

4th Year (10 decks)

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