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musculoskeletal/ neuro Flashcards

1
Q

What AEDs for epilepsy w generalised tonic-clonic seizures?

A

Lamotrigine

sodium valproate

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2
Q

JIA complications

A

if uncontrolled, bone expansion from overgrowth

leg / digit length discrepancy

valgus deformity

advancement of bone age in wrists

chronic anterior uveitis - can lead to sever visual defect. ** regular ophthalmic screening using slit lamp indicated (esp those w oligoarticular disease)

joint flexion contracture (may lead to joint destruction/ need for replacement)

growth failure (generalised or local- premature fusion of epiphyses)

osteoporosis

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3
Q

infantile spasm (West syndrome)

A

4-6mths

triad of infantile spasm, interictal eeg pattern termed hypsarrhythmia, mental retardation

seizure pattern - violent flexor spasms of head, trunk and limbs followed by extension of arms (salaam spasms)

often multiple bursts of 20-30 spasms

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4
Q

Ix of osteomyelitis

A

FBC, WCC, CRP

Blood cultures

Xray

(may show subperiosteal new bone formation + multiple hypodense ares in metaphyseal regions)

USS

(may show periosteal elevation - ‘codmans triangle’)

MRI

(allows differentiation bewteen bone and soft tissue infection)

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5
Q

fitting mx

A

ABC DEFG

ABC approach

do not forget glucose!!

secure airway, give O2, assess B/C, check blood glucose, secure IV Access (large bore cannulae)

DDx

Infection- encephalitis, meningitis, cerebral abscess

malignancy

trauma

metabolic- hypoglycaemia, DKA

febrile convulsion

epileptic fit

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6
Q

pale -> falls to ground -> recovers rapidly

often after pain, discomfort, minor head injurites

due to reflex cardiac astystole secondary to increased vagal response

A

reflex anoxic seizure

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7
Q

Most common Osteomyelitis pathogen cause

A

Staph aureus

Strep / H influenzae also possible

Salmonella (assoc w Sickle cell anaemia - sees both staph and salmonella)

consider TB in immunodeficient child

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8
Q

characteristic skin rash, arthralgia, periarticular oedema, abdo pain and glomerulonephritis

often preceded by URTI

aetiology- antigen exposure increases IgA, which interacts w IgG to activate complement and are deposited in affected organs, precipitating an immune response w vasculitis

A

Henoch schonlein purpura

Clinical diagnosis. Ix includes FBC, renal function, urine dipstick and renal biopsy if indicated.

Risk factors for progressive renal disease are proteinuria, oedema, HTN and deteriorating renal function -> renal biopsy indicated

Most will resolve within 6 wks. NSAIDs help arthritic symptoms.

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9
Q

Generalised hypotonia and marked head lag.

small low set ears, upslanting palpebral fissures (upslanting eyes), prominent epicanthic folds, flat facial profile, protruding tongue

Short neck, single palmar crease, wide sandal gap between 1st and 2nd toe

A

Downs Syndrome T21

95% due to non-dysjunction, 2% robertsonian translocation, 2% mosaicism

learning disability

congenital heart disease- AVSD, TOF, ASD, VSD

GI - duodenal atresia, Hirschsprungs, anal atresia

increased risk of infection, DDH, OME, Alzheimers, epilepsy

diagnosis by karyotyping. By rapid FISH quicker results

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10
Q

Duchenne muscular dystrophy presentation

A

waddling gait

developmental delay (motor +/ language delay, may be global delay)

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11
Q

Acute painful limp differentials (3-10 yrs)

A

Infection (Septic arthritis, osteomyelitis)

Inflammation (Transient synovitis, JIA)

Trauma

Perthes

Malignancy (ALL)

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12
Q

Tx of osteomyelitis

A

Urgent systemic IV Abx

6 wk course

IV for 2 wks (until clinical recovery) then oral for 4

Surgical drainage if does not respond to abx therapy

affected limp initially rested in splint.

+ appropriate pain relief for child

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13
Q

infantile spasms prognosis

A

developmental regression

learning disability

may get epilepsy

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14
Q

Chronic intermittent limp differentials (1-3 yrs)

A

DDH

Talipes

Neuromuscular e.g. Cerebral palsy

JIA

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15
Q

EEG 3 spike wave complex cycles/ s

A

absence seizures

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16
Q

Benign intracranial HTN risk factors

A

obesity, triggered by drugs e.g tetracyclines, cocp, steroids

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17
Q

IX of reactive arthritis

A

FBC WCC CRP/ ESR

Xrays (normal)

Temp (low grade fever)

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18
Q

Benign intracranial HTN Mx

A

reduce weight, avoid precipitating drugs and avoid LP

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19
Q

Perthes Investigations

A

XRay of both hips (incl frog leg view)

(irregular, fragmented, sclerotic femoral head, with decreased size of head)

Bone scan

MRI

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20
Q

chondromalacia patellae

A

softening of the cartilage of the patella

teenage girls

anterior knee pain when walking up or down stairs, rising from sitting

reponds to physiotherapy

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21
Q

Learning difficulties, macroorchidism, macrocephaly, large ears, long face, mitral valve prolapse

A

Fragile X

more commonly in boys

CGG repeats

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22
Q

osteochondritis dissecans

A

pain after exercise

intermittent swelling and locking of knee

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23
Q

Treatment of DDH

A

Conservative (for newborns):

Pavlik harness.

(for 1-2 months. it is a soft positioning device that keeps the thighbone in the socket. helps tighten the ligaments around hip joint and promotes normal hip socket formation.)

Conservative (1-6 months):

Harness/ Craig’s splint

usually worn full time for at least 6 wks, then part-time for additional 6 wks.

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24
Q

risk factors for septic arthritis

A

immunocomp

joint problems/ damage

IVDU

diabetes

SCA (Salmonella)

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25
Q

beckers vs duchenne

A

some functional dystrophin produced

progresses more slowly

avg onset 11 yrs

life expectancy from late 40s to normal

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26
Q

Perthes Treatment

A

if identified early + <50% femoral head affected:

bed rest, analgesia and traction

severe/ late disease:

maintain hip in abduction w plaster or calipers

or

surgery (femoral or pelvic osteotomy)

whcih repositions femoral head within the acetabulum

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27
Q

Reactive arthritis most common pathogens

A

in adolescents: chlamydia trachomatis, neisseria gonorrhoea

in children:

campylobacter

yersinia

samonella

shigella

Also mycoplasma and lyme disease

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28
Q

skull xray shows characteristic rail road track calcification

MRI choice of imaging now

A

sturge weber

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29
Q

Hypotonia, hypogonadism, obesity

A

Prader-Willi

deletion on paternal chromosome 15

characteristic facies, floppy w feeding difficulties and FTT in infancy

rapid weight gain at 1-6 years old. typically have insatiable appetite w food foraging and other behavioural problems

diagnosis by molecular genetic analysis

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30
Q

Mx of JIA

A

aim to induce remission

Managed by specialist paediatric rheumatology MDTs (physio, GP, school, orthopaedics)

education and support for child and family

physio to maintain join function

NSAIDs to relieve symptoms during flares

Joint injections under USS guidance

Methotrexate (give weekly and regular blood monitoring required)

Systemic steroids - avoid if possible to minimize growth suppression and osteoporosis

Biologics - antiTNFa

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31
Q

hydrocephalus tx

A

minimised risk of neuro damage and symptomatic relief

mainstay: insertion of Ventriculoperitoneal VP shunt

ventriculostomy surgery

shunts can malfunction due to blockage or infection (usually w CNS)

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32
Q

perthes complications

A

may be commonly seen in SCA

degenerative osteoarthritis in early adult life

premature fusion of the growth plates

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33
Q

Acute painful limp differentials (11-16 yrs)

A

Mechanical (trauma, overuse, sport injuries)

SUFE

Perthes

Inflammation (RA, JIA)

Knee- osteochondritis dissecans

Malignancy (bone tumours)

Infection (SA, osteomyelitis)

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34
Q

drug side effects: rash, neutropenia, hypoNa, liver enzyme induction interfering w other meds

A

carbamazepine

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35
Q

NICE guidelines for refractory convulsive status epilepticus

A

IV Midazolam/ thopental sodium

+ adequate monitoring and blood levels of AEDs

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36
Q

Most common cause of headaches in children

A

Migraine

tension-type

refractory errors

cluster headaches

secondary:

benign intracranial HTN

raised ICP

infection- meningitis, encephalitis, sinusitis

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37
Q

ash leaf depigmented patches which fluoresce under UV light (woods light)

A

tuberous sclerosis

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38
Q

infantile spasm tx

A

1st line: AED vigabatrin

or steroids

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39
Q

neurofibromatosis type 1

features

A

6 or more cafe au lait spots >5mm in size before puberty, 15mm after puberty

1 or more neurofibroma

optic glioma - can cause visual impairment

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40
Q

port wine stain in distribution of trigeminal n

assoc w similar lesion intracranially

ophthalmic division of trigem n always involved

A

sturge weber syndrome

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41
Q

ethosuximide SE

A

N+V

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42
Q

short stature, learning difficulties, friendly, extroverted personality, transient neonatal hypercalcaemia, supravalvular aortic stenosis

A

Williams syndrome

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43
Q

septic arthritis investigations

A

FBC WCC CRP

Blood cultures

ALWAYS examine joint above and below

USS of joint- effusion

XRay to exclude other trauma/ lesions

MRI - may demonstrate adjacent osteomyelitis

JOINT ASPIRATION under USS guidance and culture is definitive (ideally immediately, before ABx)

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44
Q

osgood schlatter tx

A

rest and analgesia

most resolve w reduced activity and physio for quads strengthening, hamstring stretches and occasionally orthotics

Knee immobilised splint may be helpful

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45
Q

Perthes

A

Avascular necrosis of the femoral head

due to interruption of blood supply,

followed by revascularisation and reossification

mainly affects boys (5:1) of 5-10 yrs

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46
Q

in community, if prolonged seizure (>5 min)

A

1st line; buccal midazolam

if buccal not avail, rectal diazepam

Max 2 doses of first line tx

If continuing seizures, IV phenytoin

and contact anaesthetist - child may need rapid anaesthesia for intubation and ventilation

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47
Q

increased ICP in absence of mass lesion

A

benign intracranial HTN

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48
Q

developmental dysplasia of the hip risk factors

A

female breech (esp vaginal delivery) oligohydramnios multiple pregnancy family history prematurity firstborn child

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49
Q

patellar subluxation

A

medial knee pain due to lateral subluxation of the patella

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50
Q

EEG- hysparrhythmia

salaam spasms

A

west syndrome

infantile spasms

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51
Q

DDH Surgical treatment

A

Surgical (> 6mths):

surgery (closed reduction) + hip spica cast

skin traction may be used for few wks before, as it prepares the soft tissues around the hip for the change in bone positioning.

Surgical (>2 yrs):

usually open surgery necessary + spica cast after

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52
Q

septic arthritis complications

A

adjacent osteomyelitis

sepsis

joint destruction

ankylosis

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53
Q

febrile convulsions investigations

A

examination - obs, temp

focus on cause of fever, exclude meningitis, encephalitis

Full septic/ infection screen may be necessary

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54
Q

febrile convulsion risk factors

A

family history

6mths-5yrs old

viral infection w temp rising rapidly

previous hx of febrile convulsion

(1 in 3 will have another febrile convulsion)

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55
Q

Duchennes Mx

A

appropriate physiotherapy and exercise to maintain muscle power and mobility

passive stretching and night splints to prevent contractures

orthoses to prolong walking

maintaing good posture and exercise reduces risk of scoliosis

scoliosis managed w truncal brace, moulded seat, surgical insertion of spinal rod

self help groups for parents

MDT care: specialist regional centre

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56
Q

Duchenne IX

A

serum CK (markedly elevated)

muscle biopsy showing decreased dystrophin is diagnostic

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57
Q

most common pathogen of septic arthritis

A

staph aureus

Hib was impt cause before Hib immunisation

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58
Q

First non febrile seizure IX

A

ECG - could be cardiac cause for fit e.g. prolonged QT

MRI if focal seizure <2 yo

after 2nd seizure: EEG

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59
Q

prominent occiput, small mouth and chin, low set ears, short sternum, overriding fingers, rocker bottom feet, cardiac (VSD) and renal malformations

A

Edwards Syndrome

Trisomy 18

majority caused by non dysjunction during maternal oogenesis

most do not survive beyond 1 yr

diagnosis - intitial rapid FISH followed by formal chromosomal analysis

median life expectancy around 4 days, although some live for several months

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60
Q

hydrocephalus different causes

A

obstruction to flow of CSF-> leading to dilation of ventricular system

obstruction may be within ventricular system or aqueduct (non communicating) or at arachnoid villi, the site of CSF absorption (communicating)

Causes:

non communicating: congenital malformation (e.g. aqueduct stenosis, stresia of outflow, chiari malformation)

communicating: subarachnoid haemorrhage, meningitis

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61
Q

systemic JIA features

A

fever

rash

lymphadenopathy

hepatosplenomegaly

serositis

anaemia, raised neut/pl, raised CRP/ESR

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62
Q

what is DDH?

A

ranging from dysplasia -> subluxation -> frank dislocation of hip checked in neonatal screening, thereafter presents with limp or abnormal gait. Look out for legs of different length + uneven skin folds on the thigh

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63
Q

DDH Complications

avascular necrosis of the femoral head

pain

osteoarthritis by early adulthood

even w appropriate tx: hip deformity and osteoarthritis may develop later. Esp when tx begins after 2 yrs.

A
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64
Q

webbed neck, pectus excavatum, short stature, pulmonary stenosis

A

Noonan syndrome

autosomal dominant disorder, karyotype normal

short stature, characteristic facies (hypertelorism -usually increased distance between eyes, ptosis, ear abnormalities), short webbed neck, pectus excavatum, undescended testes

associated pulmonary stenosis & ASD, cardiomyopathy, learning difficulties

diagnosis confirmed by mutation analysis of the PTPN11 gene

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65
Q

Duchenne muscular dystrophy - lack of what protein?

A

dystrophin

causing excess of free radicals and myofibre necrosis

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66
Q

Tx for Reactive arthritis

A

complete recovery anticipated

only need pain relief, NSAIDs

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67
Q

clinical features of hydrocephalus

A

infants: as skull sutures not fused, HC disproportionately large, bulging ant fontanelle

advanced sign: fixed downward gaxe or sun setting of eyes

in older children: signs and symptoms of raised ICP

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68
Q

Hydrocephalus IX

A

antenatal USS

Cranial USS for infants

or Imaging with CT or MRI

Head circumferene should be monitored on centile charts

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69
Q

restriction of visual fields, sedation

Drug SEs

A

Vigabatrin

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70
Q

DDH neonatal screen test?

A

Barlow’s (whether hip can be dislocated posteriorly out of the acetabulum) - bring thighs to midline to adduct hip and push on knee backwards) Ortolanis (relocated back w abduction) Screened for at newborn first exam + thereafter (6-8wks)

71
Q

Mx for epileptic seizure

A

always refer on to specialist asap!

must be diagnosed by paediatrician w expertise in epilepsy

discuss w parents what to do in next seizure:

First aid/ safety and injury prevention

safe spot, recovery position, time the seizure, take video if possible

if >5 min, come into hosp, give buccal midazolam

Liaise w school

Offer links to support groups / charities

social support

72
Q

Most common cause of acute hip pain in children (2-12 yo)

following/ alongside viral infection

sudden onset of pain or limp.

pain on movement, decreased ROM, esp int rotation

pain may be referred to knee

Afebrile, does not look ill

A

transient synovitis

73
Q

Chronic intermittent limp (11-16yrs)

A

SUFE (chronic)

JIA

tarsal coalition

74
Q

Benign intracranial HTN features

A

headache, transient diplopia, papilloedema

75
Q

SUFE complications

A

avascular necrosis

premature fusion of epiphyses

76
Q

obese 10-15 yr old boy during adolescent growth spurt with insidious hip pain

(can be acute following minor trauma)

can also be limp, or referred knee pain

A

Slipped upper femoral epiphyses

77
Q

epilepsy investigations

A

take collateral history.

pay attention to specific triggers

clinical exam: check skin for neurocutaneous syndromes and full neuro exam

EEG - after 2nd seizure

or 24h ambulatory eeg

MRI/CT imaging if focal neuro signs

Genetic studies for certain epileptic syndromes

78
Q

if Barlows and ortolanis -ve, but many risk factors?

A

still do USS of hip +

if necessary, can seek specialist ortho surgeon opinion

79
Q

Transient synovitis Tx

A

Rest and analgesia

+ Skin traction

usually resolves within 1 wk

~3% develop perthes

80
Q

Mx of Turners syndrome

A

Refer to genetics team

MDT - paediatric endocrinologist, gynaecologist, psychologist, geneticist, nephrologist, cardiologist

Growth hormone therapy- can help child grow e.g. somatotropin

Oestrogen and Progesterone replacement tx- oestrogen to trigger puberty and then progesterone also started to help girl develop regular periods

If wanting fertility - likely IVF

Psychological therapies for low mood/ self esteem

Learning disability- support

81
Q

Headache mx (when no red flags)

A

thorough history and exam

recurrent headaches common and cause no long term harm

advice on how to live with it

rescue tx- analgesia (paracetamol and NSAIDs) , antiemetics (prochlorperazine, metoclopramide), serotonic agonists (sumitriptan if >12yo)

but beware of medication overuse headache

psychosocial support- stressor ie bullying?

relaxation techniques

82
Q

mx of congenital adrenal hyperplasia

A

during salt losing crisis - urgent tx with IV hydrocortisone, saline and glucose

long term - glucocorticoid replacement e.g. hydrocortisone with increased doses needed for illness/ stress

if salt wasting- also replace fludrocortisone

regular monitoring of growth, skeletal maturity, and androgens to ensure optimal growth and development

83
Q

virilisation of external genitalia in females (clitoral hypertrophy)

virilisation in males (enlarged penis)

salt losing adrenal crisis (low Na)

vomiting, weight loss, floppiness and shock

A

Congenital adrenal hyperplasia

enzyme defect - deficiencies in 21a-hydroxylase (90%), 11B hydoxylase and 17a hydroxylase

decrease in cortisol production and aldosterone -> increased ACTH and subsequent adrenal hyperplasia

auto recessive

diagnosis by detecting biochemical abnormalities during salt wasting crisis- Low Na, High K, metabolic acidosis, hypoglycaemia

+ measuring high lvls of 17a-hydroxyprogesterone, etc

84
Q

what AEDs for infantile spasms?

A

steroids

vigabatrin

85
Q

Gowers sign

A

duchenne muscular dystrophy

86
Q

SUFE investigations

A

XRAY esp lateral view

shows ice cream cone sign

87
Q

micrognathia, posterior displacement of tongue (may cause upper airway obstruction), cleft palate

A

pierre-robin syndrome

88
Q

EEG high amplitude spikes in L centrotemporal region

A

benign rolandic epilepsy

89
Q

lamotrigine SE

A

rash

90
Q

What AED if myoclonic seizures?

A

sodium valproate

consider levetiracetam or topiramate

91
Q

Febrile convulsions Mx

A

reassure parents and provide advice sheet

antipyretics not effect

first aid mx of seizures taught to family:

safe spot, put them in recovery position, don’t restrain them/ put anything in their mouth

if prolonged >5 min, rescue therapy w rectal diazepam/ buccal midazolom

if first episode, MUST be reviewed by consultant paediatrician

92
Q

tuberous sclerosis inheritance

A

auto dominant

93
Q

Head injury mx?

A

ABC

cervical spine immobilisation unless suspiscion of neck injury

94
Q

Transient synovitis ix

A

difficult to differentiate from early septic arthritis

if suspicious, joint aspiration and blood cultures

FBC, WCC, CRP

USS of hip (shows mild effusion)

XRay shows normal joint

95
Q

infantile spasms ix

A

EEG

MRI/CT head- shows diffuse/ localised brain disease in 70%

96
Q

topiramate SEs

A

drowsiness, wtihdrawal, weight loss

97
Q

features that suggest SOL

A

worse in morning/ when lying down/ straining/ coughing

vomiting in the morning

change in mood/ personality/ educational performance

visual field defects

focal neurological signs (e.g. cranial n abnormalities)

papilloedema

98
Q

neurofibromatosis type I inheritance

A

auto dom

99
Q

If on AED and pregnant? Advice?

A

risk of AEDs causing malformations and neuro impairments in unborn child, esp Sodium valproate

offer 5mg/day of folate before/ if any possibility of pregnancy

aim for seizure freedom

100
Q

presentation of septic arthritis

A

acutely unwell, febrile child

with erythematous, warm, acutely tender joint

w reduced or ‘no’ ROM

infant may hold limb still (pseudoparalysis)

may be referred to knee pain

101
Q

What is osgood-schlatter?

A

transient inflammation of the (patellar tendon insertion) growth plate of the tibial tuberosity

often affects adolescent males

worse after exercise (knee pain esp on leg extension)

localised tenderness and swelling over the tibial tuberosity

may be bilateral

102
Q

Markedly painful, immobile limb with fever.

swelling and exquisite tenderness over thigh.

+ skin is erythematous and warm

A

osteomyelitis

103
Q

temporal focal seizures

A

most common

aura, strange warning feelings

lip smacking

automatism (walking in non purposeful manner)

deja vu

jamais vu

consciousness impaired

104
Q

hair loss, weight gain, idiosyncratic liver failure (drug side effects)

A

sodium valproate

105
Q

JIA presentation

A

stiffness after rest (gelliing)

morning joint stiffness and pain

intermittent limp/ avoidance of activities

mono/oligo/polyarticular

joint swelling due to fluid, inflammation

106
Q

Chronic intermittent limp differentials (3-10 yrs)

A

Perthes (chronic)

neuromuscular e.g. CP, Duchenne’s muscular dystrophy

JIA

Trauma (old unidentified fracture)

107
Q

JIA

A

most common chronic inflammatory joint disease in children and adolescents

persistent >6wks joint swelling in absence of infection or any other cause

108
Q

pseudohypertrophy of calves

A

duchenne muscular dystrophy

(replacement of muscle w fat and fibrous tissue)

109
Q

what AEDs used in absence epilepsy/ seizure?

A

ethosuximide

sodium valproate

110
Q

Management of Downs Syndrome

A

Refer to genetics team

refer for dated cardiac assessment, Hip USS, audiology

Long term follow up with MDT team including developmental paediatrician, physiotherapist, specialist nurse etc

Genetic counselling

Parental support e.g. Downs Association

+ education on short term and long term implications of the diagnosis

Test TFTs, audiology, opthalmic assessment yearly

Appropriate education support

111
Q

short stature, webbed neck, wide carrying angle, widely spaced nipples, lymphoedema of hands and feer, spoon shaped nails, excessive pigmented moles

A

Turners Syndrome 45XO

caused by non dysjunction

short stature (cardinal feature)

heart conditions- Coarctation of the arorta and VSD, aortic root dilation + bicuspid aortic valve, renal anomalies

ovarian dysgenesis resulting in delayed puberty, amenorrhoea and infertility

diagnosis by chromosome analysis

112
Q

Migraine common triggers

A

cheese, chocolate, caffeine

menstruation, cocp

stress, late nights

113
Q

angiofibromas in butterfly distribution over bridge of nose and cheeks

A

tuberous sclerosis

114
Q

tonic clonic seizures

A

limb jerking, breathing irregular, cyanosis, tongue biting, urinary incontinence

unconscious or deep sleep to up to several hours later

115
Q

microcephaly, polydactyly, cleft lip/ palate, scalp lesions, small eyes

A

Patau syndrome (trisomy 13)

75% by non dysjunction, 20% robertsonian translocation, 5% mosaicism

usually diagnosed on antenatal scan due to multiple congenital abnormalities incl SGA, microcephaly, cleft lip, polydactyly, micropthalmia (small eyes), cardiac (VSD, ASD), and renal anomalies (fused kidneys) and severe mental retardation

diagnosis via chromosome analysis by amniocentesis, but rapid FISH done to confirm diagnosis quickly

116
Q

tall stature, gynaecomastia in puberty, infertility, small testes

A

Klinefelter’s syndrome 47XXY

infertility most common presentation

hypogonadotrophic hypogonadism - small testes and decreased testosterone production

diagnosis by chromosome analysis

117
Q

what examinations are important in investigating epilepsy?

A

cardiac

neurological

development assessment

118
Q

next step if barlows and ortolani’s postive?

A

USS of hip - allows detailed hip assessment

119
Q

epilepsy mx

A

NICE guidelines or follow individually agreed protocol drawn up by specialist

MDT- specialist epileptic nurse + consultant paediatric neurologist

120
Q

duchenne prognosis

A

life expectancy reduced to late twenties

from resp failure or assoc cardiomyopathy

complications: scoliosis, learning difficulties

121
Q

Septic Arthritis Tx

A

Prolonged course of ABx, initially IV

recommendations vary from 1 -6 wks

Fluclox and Benpen

washing out of joint or surgical drainage may be required

122
Q

If prolonged seizure (>5 min), IV access in place

A

IV lorazepam 1st line

2nd line: iv diazepam or buccal midazolam

+ ABC

MAX 2 doses

If continuing seizures, IV phenytoin

and contact anaesthetist - child may need rapid anaesthesia for intubation and ventilation

123
Q

Duchenne muscular dystrophy

what is it?

A

inherited disorder X-linked recessive

progressive muscle degeneration

124
Q

baby crying, holding breath til blue then losing consciousness

recovers fully and rapidly

A

breath holding attacks

occurs in toddlers when they are upset

behaviour modification therapy, with distraction, may help

125
Q

SUFE assoc w endocrine abnormalities

A

hypothyroidism

hypogonadism

126
Q

Acute painful limp differentials (1-3 yrs)

A

1-3 yrs:

Infection (Osteomyelitis, septic arthritis)

Inflammation (transient synovitis)
Trauma (NAI/ accidental)

Malignancy (Leukaemia, neuroblastoma) - pain may be primarily at night

127
Q

odd sensation at corner of mouth -> twitching of mouth -> rest of ipsilateral face twitches

assoc w excessive salivation, grunting and slurred speech

A

benign rolandic epilepsy

128
Q

traffic light system for fever <5

other

A

Amber - intermediate risk:

age 3-6 months and temp >39

fever >5 days

Rigors

Swelling of limb/ joint

non weight bearing limb

Red - high risk:

Age <3 and temp >38

non blanching rash

bulging fontanelle

neck stiffness

status epilepticus

focal neuro signs

focal seizures

129
Q

Risk factors for slipper upper femoral epiphyses

A

obese children and boys

growth spurt

may present acutely following trauma

130
Q

what should be recorded in all febrile children?

Obs

A

temp, heart rate, resp rate, cap refill time

signs of dehydration (reduced skin turgor, cool extremities) should be looked for

then use NICE traffic light system to determine risk and subsequent mx

131
Q

Traffic Light system

Colour

A

Green - low risk:

Normal colour

Amber - intermediate risk:

pallor reported by parent/ carer

Red - high risk:

pale/ mottled/ ashen/ blue

132
Q

Traffic light system for feverish under 5 children

Activity

A

Green - low risk:

responds normally to social cues, smiles, stays awake, strong normal crying/ not crying

Amber - intermediate risk:

not responding normally to social cues, no smile, decreased activity, wakes only w prolonged stimulation

Red - high risk:

no response to social cues, appears ill to a healthcare professional, does not wake or if roused does not stay awake, weak high pitched or continous cry

133
Q

traffic light system for feverish illness <5

respiratory

A

Amber - intermediate risk:

nasal flaring, RR >50 if aged 6-12 months, RR>40 if > 1yr, SaO2 <95% in air, crackles in the chest

Red - high risk:

RR >60

grunting

]moderate or severe chest indrawing

134
Q

traffic light system for feverish <5

circulation and hydration

A

Amber - intermediate risk:

tachycardia >140 if age 2-5, >150 if 1-2 yrs, >160 if <1 yr

CRT >3s

Dry mucus membranes, poor feeding, reduced UO

Red - high risk:

reduced skin turgor

135
Q

Presentation of acute glomerulonephritis

A

reduced GFR -> decreased urine output and volume overloaded, haematuria and proteinuria.

HTN which may cause seizures.

Oedema, characteristically around the eyes.

136
Q

why is Downs associated with sleep apnoea and snoring?

A

This is due to the low muscle tone in the upper airways and large tongue/adenoids. There is also an increased risk of obesity which in people with Down’s syndrome which is another predisposing factor to snoring.

137
Q

causes of snoring in children

A
  • obesity
  • nasal problems: polyps, deviated septum, hypertrophic nasal turbinates
  • recurrent tonsillitis
  • Down’s syndrome
  • hypothyroidism
138
Q

at what age should a child be smiling?

A

6 wks

refer at 10 wks

139
Q

at what age should a child be laughing and enjoing friendly handling

A

3 months

140
Q

at what age should a child be shy and takes everything to mouth

A

9 months

141
Q

at what age should a child be able to use knife and fork

A

5 years

142
Q

at what age should a child be able to use spoon and fork

A

3 years

143
Q

at what age should a child be competent w spoon and doesnt spill w cup

A

2 years

144
Q

at what age does child develop skills of drinking from cup and using spoon, over a 3 month period

A

12-15 months

145
Q

at what age will child get dressed and undressed independently except for laces and buttons

A

4 years

146
Q

periorbital oedema, leg and ankle oedema, ascites, breathless due to pleural effusions and abdo distension

A

nephrotic syndrome - proteinuria, low plasma albumin and oedema

Ix- Urine dip for protein

possible causes: minimal change disease, secondary to HSP, SLE, infections e.g. malaria, or allergens e.g. bee sting

147
Q

Mx of nephrotic syndrome

A

85-90% steroid sensitive -> resolves with steroid therapy

Give oral steroid (prednisolone) for 4 wks then reduce dose for another 4 wks then stop.

if steroid resistant, refer to paediatric nephrologist. Mx by diuretic therapy, salt restriction, ACEi and sometimes NSAIDs.

148
Q

Complications of nephrotic syndrome

A

Clots - loss of antithrombin III in urine

Infections - loss of Ig -> risk of encapsulated bacteria.

Hypovolaemia - intravasc compartment vol depleted. urinary sodium retention -> low urinary Na (<20mm/L). requires urgent tx w IV albumin as child is at risk of vascular thrombosis and shock

Hypercholesterolaemia - correlates inversely w serum albumin

149
Q

Ix /MX for primary nocturnal enuresis

A

Urine always tested for glucose and protein and for infection.

Mx:

explanation to child and parent. stop punitive measures.

star chart

enuresis alarm - if star chart does not work

desmopressin - short term relief. used first line for >7 who wish to achieve dryness v quickly.

self- help groups - advice and assistance to parents

150
Q

USS of kidneys can look for?

A

urinary tract dilatation, stones, nephrocalcinosis

151
Q

downward slanting palpebral fissures, underdevelopment of lower jaw, + tongue being retracted. external ear is small, rotated, malformed or absent. -> majority have conductive hearing loss

A

treacher collins syndrome

152
Q

acute, unilateral facial n palsy around 2 wks following a viral infection.

mouth droops, pt cannot close one eye.

A

Bells palsy

tx: maintiaining moisture to the affected eye to prevent keratitis. complete spontaneous resolution occurs in about 85% of cases.

153
Q

by what age should infants be able to smile and coo when smiled at or being talked to

A

2 months

154
Q

by what age would a child be able to help dress and undress themselves, with the exception of shoelaces/ buttons. can ride a tricycle and can alternate feet when climbing stairs.

they know their age and gender, but have not porgressed beyond copying a circle.

A

3 years

155
Q

complication of juvenile rheumatoid arthritis

A

iridocyclitis

-> all children should have frequent slit lamp eye examinations.

156
Q

staphylococcal scalded skin disease

aka

Ritter’s Disease of the newborn

A

most commonly in children <5 yrs

due to epidermolytic exotons released by s aureus-> breaking down of the desmosomes. they cleave desmoglein-1 (similar to pemphigus vulgaris)

rash preceded by fever, irritability, erythema and extraordinary tenderness of the skin.

circumoral erythema, crusting of the eyes, mouth and nose, and blisters of the skin can develop. intraoral mucosal surfaces are not affected. (unlike toxic epidermal necrolysis, mucous membranes are spared)

Nikolskys sign positive.

culture of bullae are negative, but source site often positive.

tx: antibiotics to cover s aureus and localized skin care.

157
Q

human bite

  • what organisms may infect it
  • what mx
A

oropharyngeal bacteria, including staphylococcus aureus, viridans streptococci, bacteroides spp and anaerobes.

a patient w infected human bite of the hand requires hospitalization for appropriate drainage procedures, gram stain and culture of the exudate, vigorous cleaning, debridement and appropriate antibiotics.

tx usually w oral coamoxiclav or erythromycin.

the would should be left open and allowed to heal by secondary intention.

158
Q

presents w a herald patch, a single round or oval lesion appearing anywhere on the body. usually about 5-10 days after, a more diffuse rash involving upper extremities and trunk appears.

the rash can appear in a christmas tree pattern on the back.

A

Pityriasis rosea

tx is usually unnecessary but can consist of topical emollients and oral antihistamines

159
Q

neonatal acne

A

peaks at 2-4 wks of age.

the condition results from maternal hormone transmission.

it resolves in a few wks to months, and occasionally is severe enough to require tx w agents such as 2.5% benzoyl peroxide.

160
Q

constricted pupils, bradycardia, muscle fasciculations assoc w sudden onset neurologic symptoms, progressive resp distress, diaphoresis, diarrhoea and overabundant salivation

A

organophosphate poisoning

intake can occur by ingestion, inhalation, or absorption through skin or mucosa.

toxicity depends primarily on the inactivation or inhibition of acetylcholinesterase

tx includes gastric lavage if poison was ingested.

if exposure through contact, maintenance of adequate ventilation and fluid and electrolyte balance.

all symptomatic children should receive atropine.

161
Q

mx of scabies in infants

A

usually present w bullae and pustules.

5% permethrin cream

162
Q

factors that confer high risk to hearing impairments in neonates

A

craniofacial defects (including abnormal ear structure),

neonatal asphyxia,

bacterial meningitis,

congenital infections,

significant hyperbilirubinaemia,

use of ototoxic medications >5 days,

Fhx,

birth weight <1500g,

mechanical ventilation for more than 10days.

163
Q

strabismus tx

A

persistence of a transient or fixed deviation of an eye beyond 4 months requires referral to an ophthalmologist.

infants screened for strabismus by observing the location of a light reflection in the pupils when the pt fixes on a light source.

to prevent monocular blindness and to ensure the development of normal binocular vision, early recognition and treatment of strabismus are essential.

TX: to prevent loss of central vision, accomplished by surgery, eyeglasses, or patching of the normal eye.

164
Q

finding pus in the joint in septic arthritis indicates?

A

immediate surgical drainage and prompt institution of IV abx to avoid serious damage to the joint and permanent loss of function.

most common organism found in staph aureus.

in sexually active adolescents, neisseria gonorrhoea is a common cause.

165
Q

dog bite mx

A

antibiotic prophylaxis should be considered in cat, human, or monkey bites too, and should be given to any bite sustained by an infant, diabetic or immunocompromised person.

166
Q

erythema toxicum neonatorum

A

lesions (1-2mm) are yellow-white and w a surrounding edge of erythema.

examination of the fluid from these lesions demonstrates eosinophils.

No therapy is indicated.

167
Q

Salmon patches aka stork bites

A

flat vascular lesions that appear more prominent when crying.

lesions on the face fade over the first few wks of life.

lesions found over the nucal and occipital areas often persist.

no tx indicated.

168
Q

Pustular melanosis

A

benign self limited disease

more common in darker skin tones.

lesions usually found at birth and consist of 1 -2mm pustules that result in a hyperpigmented lesion upon rupture of the pustule.

the pustular stage of these lesions occur during the first few days of life, w the hyperpigmented stage lasting weeks to months.

no tx indicated.

169
Q

sebaceous naevi

A

small, sharply edged lesions that occur most commonly on the head and neck of infants. yellow orange in color and are slightly elevated. usually hairless.

170
Q

seborrhoeic dermatitis

A

frequently presents as cradle cap in the newborn period.

this rash is commonly greasy, scaly, and erythematous and in smaller children, involve the face, neck, axilla and diaper area. in older children, the rash can be localized to the scalp and intertriginous areas.

pruritus can be marked.

171
Q

SUFE - surgery w internal fixation or open reduction?

A

internal.

open reduction assoc w increased incidence of avascular necrosis of femoral head.

172
Q

osteogenesis imperfecta

inheritance?

A

auto dominant

173
Q

Ash leaf macules + subependymal nodules + infantile spasms

A

Tuberous sclerosis

  • a cause of infantile spasms - treated w vigabatrin

paeds (12 decks)

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