Paeds Notes Flashcards
Cystic fibrosis: AR/AD?
AR
Cystic fibrosis: 1 in ? affected?
1 in 2000
Cystic fibrosis: 1 in ? carriers?
1 in 22
Life expectancy of cystic fibrosis
Mid 30s
Mutation in cystic fibrosis and which chromosome
CFTR on Chr7. most commonly delta f508
Organism that specifically affects cystic fibrosis
pseudomonas auriginosa
If not through newborn screening, how might CF present?
meconium ileum
2 diagnostic tests for CF
Heel prick Sweat test (>60mmol/l of sweat Cl- supports diagnosis)
Treatment for CF
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
When do you give Abx to tonsillitis/pharyngitis? And what antibiotic for how long?
10 days Pen V if FeverPAIN score:
0-1 = nothing
2-3 = delayed
4-5 = give Abx
Indication for tonsilectomy
7 in one year or 10 in 2 years
scarlet fever cause
Group A strep (think of strep throat)
cause of croup
parainfluenza 3
sometimes RSV
management of croup
oral dex and support
IV adrenaline is emergency
presentation of bacterial tracheitis
viral prodrome 2-5 days then rapid deterioration with continuous biphasic stridor
cause of bacterial tracheitis
S.aureus
management of bacterial trachetiis
cefotaxime + fluclox
?intubation
key feature of diptheria
tonsilitis + grey false membrane over them
management of diptheria
Diptheria antitoxin
erythromycin to patient and contacts
epiglottitis cause
Hib
presentation of epiglottitis
VERY RAPID ONSET
Very high fever, won’t swallow and will drool instead. continuous stridor.
management of epiglotittis
Call anaesthesitst, paediatrician and ENT surgeon for controlled intubation.
Cefotaxime as Abx
age at which viral wheeze tends to stop
5 (most is 2-3)
brittle asthma types
type 1 - variable PEFR
type 2 - sudden attack on normal background
when to discharge after acute asthma
when stable on >4hrly salbutamol
what to discharge acute asthma attack with
- 3-7days pred
- follow up in 1 months in clinic
- inhaler technique
when is acute bronchiolitis worst?
5 days
bronchiolitis cause?
RSV
bronchiolitis prevention?
Monthly pavalizumab
whooping cough timeline
1 week of coryza
3 months of paroxysmal phase
treatment of whooping cough
macrolide
latent TB worldwide number?
2 billion
death from TB / year
2.5 million
investigations for TB (3)
tuberculin
interferon gamma release assay
Zn stain for acid fast bacilli
TB management: latent and active
latent = 3m RI
Active = 2mRIPE and 4mRI
rifampicin, isoniazid, pyrazinamide, ethambutol
pulmonary blood flow before vs after first breath
6x increase
why does the foramen ovale close
blood goes to lung and so left atrial pressure increases
blood excluded from placenta to right atrial pressure decreases
Why are innocent murmurs SASSy?
soft
asymptomatic (incl. sats)
systolic
left sternal edge
flow murmur scenario
fever
anaemia
day 1 murmur (due to physiologic tricuspid regurgitate)
type of murmur:
aortic stenosis
ejection systolic upper right
type of murmur:
ASD
soft systolic and S2 splitting
type of murmur:
pul stenosis
ejection systolic upper left
type of murmur:
VSD
harsh pan systolic left chest
type of murmur:
coarc of aorta
systolic on left chest
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
RL = blue LR = breathless (acyanotic)
tetra RL asd LR tga RL VSD LR PDA LR Eisen RL
tetralogy of fallot
- large VSD
- over-riding aorta
- pulmonary stenosis
- RV hypertrophy
most common congenital heart disease?
VSD (30% of total), and 9 most common account for 80%
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)
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
eisenmenger syndrome
longstanding L2R shunt (e.g. VSD/ASD/PDA) goes unnoticed. This eventually causes pulmonary hypertension and reversal of the shunt, causing cyanosis in adults.
types of ASD and which is more common
primum (Downs)
secumdum (85%)
presentation of ASD
asymptomatic with murmur (fixed S2 splitting and soft systolic left sternal edge)
CXR for ASD
cardiomegaly (RV dilatation - remember its L2R), enlarged pul. arteries, increased pul. vascular markings.
presentation of VSD
asymptomatic with murmur (pansystolic):
- small VSD = loud murmur
- large VSD = quiet murmur
CXR and ECG with VSD
- small VSD = normal
- large VSD = cardiomegaly, enlarged pul arteries, increase pul vast marking, biventricular hypertrophy on ECG
management of VSD
- small = most close spontaneously
- large = heart failure meds (diuretics and ACEi) + surgery at 6m
when is a ductus arteriosus considered failed to have closed?
1 months
presentation of PDA
machinery murmur
collapsing/bounding pulse
breathless
management of PDA
closure by coil
what is the nitrogen washout test
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
management of cyanosed neonate
ABC
IV prostaglandin
Do ECG, Echo and assess cause and need for surgery
CXR with tetralogy of fallot
Small heart with up tilted apex (boot shaped RV hypertrophy)
when is surgery for ToF
6 months
When does TGA present
day 2 of life with rapid deterioration as duct closes (since it’s two parallel circuits at this point)
management of TGA
as with any cyanosed baby (ABC, IV prost, investigate)
balloon atrial septostomy in 20% of cases, arterial switch surgery on day 3-5
atrioventricular septal defect common in?
Downs
How do AVSDs present
antenatal USS
cyanosis at birth
Heart failure 2-3 weeks
no murmur
how does aortic stenosis/pul stenosis occur in babies?
leaflets fused abnormally (as opposed to calcification in elderly
when does coarctation of the aorta present?
normal at first check, circ. collapse at 2-3 days when duct closes
what is coarctation associated with?
Turners
management of hypoplastic left heart syndrome
Norwood operation
WPW Syndrome ECG change
delta wave
presentation of SVT
narrow complex rapid tachycardia 250-300 +/- pulmonary oedema (breathlessness)
management of SVT
RESTORE SINUS
- vagal stimulating manaenovres (carotid massage)
- IV adenosine (in adults its 6,12,12)
- 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
long QT syndrome presentation
sudden LOC during exercise, stress, emotion in late childhood (confused with epilepsy)
can cause sudden death from VT
long QT inheritance and associations
AD inheritance (its structural)
association:
- erythromycin
- electrolyte disorders,
- head injury
mortality of Coxsackie virus myocarditis in children
80%
management of bacterial endocarditis
Benzyl pen + gent +/- surgery
criteria for kawasaki
Fever for >5days and 4/5 of:
- non purulent conjunctivitis
- lymphadenopathy
- maculpapular rash
- extremity involvement (Red finger tips)
- mucositis (strawberry tongue, cracked lips)
management of kawasaki
High dose aspirin
Immuniglobulin
need angiogram
jones diagnostic criteria
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
treatment for rheumatic fever
rest
aspirin
benzylpenicillin followed by Pen V (prevents secondary rheumatic fever)
what is posseting vs regurg vs vomit
small amounts of milk that’s burped up alongside swallowed air. normal in babies. regurg is larger more frequent losses. vomiting is forceful ejection
when does GORD in baby resolve usually
12 months, resolved in almost everyone
does GORD affect weight of baby?
Not usually
complications of GORD (4)
faltering growth oesophagitis (haematemesis, pain, iron def anaemia) pul aspiration (pneumonia, apnoea) Sandifer syndrome (dystonic neck posture to relieve pain)
GORD management
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
incidence of pyloric stenosis
1 in 400
M:F in pyloric stenosis
M>F 4:1
management of pyloric stenosis
Randmstedt pyloromyotomy - can then be fed at 6hrs and discharged at 2 days
what is duodenal atresia associated with (2)
Downs
Polyhydramnios
Investigation for duodenal atresia
Abdo plain film = double bubble sign
management of duodenal atresia
ABC
Drip and suck
Surgery
when does appendicitis typically present
> 5 (under 3 it is vanishingly rare)
how does mesenteric adeninitis present
like appendicitis but without peritonism, guarding, rebound tenderness.
peak age for intussuception
6m-2y
primary vs secondary intusssuception
primary = lymphoid hyperplasia of peters patch secondary = pathological point (meckels, polyp) usually older children
presentation of intussuception
PAROXYSMAL colicky pain where child become pale and draws up legs
bile vomit
redcurrant jelly stools (late sign)
sausage shapes mass in upper quadrant
CI of air insufflation in intusseoction
peritonitis
shock
perforation
known lead pathological point
diagnosis of mecekls diverticulum
technetium scan showing increased uptake by ectopic gastric mucosa
when does the in utero gut return to the abdominal cavity
10w
when does volvulus due to malrotation present
first 1-3 days (90% in first year)
diagnosis of volvulus due to malrotation
urgent upper GI contrast shows corkscrew appearance
what 3 features of chronic abdo pain suggest non-organic cause (which is the case with >90%)
- periodic pain with intervening good health
- periumbilical
- related to school hours (not there in holidays/evenings)
recurrent idiopathic abdo pain definition
pain enough to interrupt ADLs for at least 3 months
what is an abdominal migraine
abdo pain in addition to headaches that comes in attacks (long periods ok and then 12-48hrs of pain)
treatment of abdominal migraine
NSAIDs and triptans
investigation results for eosinophilic oesophagitis
endoscopy with biopsy:
- linear furrows and trachealisation of oesophagus
- eosiniphilic infiltration on biopsy
management of eosinophilic oesopahgitis
swallowed corticosteroids (fluticasone, viscous budesonide)
what is the only abnormal thing if dehydration <5%?
dry mucous membranes
what suggests >10% dehydration as opposed to just 5-10% dehydration?
anuria
coma
low BP (late sign)
Mottled/pale skin
–> tachypnoea occurs in moderate 5-10% dehydration, don’t get caught out
calculating fluid requirement
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
how to rehydrate
try oral
then NG
persistent vomiting = IV rehydration
deaths from gastroenteritis per year
1.5 million
most common virus causing gastroenteritis
rotavirus (60%)
dysentry causes
CASES:
campylobacter amoeba shigella E.coli salmonella
Revise table of gastroenteritis
PDF or desktop
why avoid juice in gastroenetritis
osmotically draws even more water into gut
when are Abx required in gastroenteritis
- sepsis
- salmonella under 6m
- specific infection (cdiff, cholera, shigella, giardia)
advice for parents at home with gastroenteritis in child
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
key question with chronic diarhoea
are they failing to thrive?
yes = CF, coeliac, lactose intolerance
No = overflow constipation, toddler diarrhoea
how to manage toddler diarhoea
fibre down
sugar down
fat up
gastroenteritis with eosinophilia
and Tx
giardiasis
metronidazole
Cows milk protein intolerance vs lactose intolerance
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.
prevalence and incidence of coeliac
1%
1 in 3000
histopathology of coeliac
<3:1 villus height:crypt depth ratio with lymphocyte invasion
how and when does coeliac usually present
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.
screening for coeliac
anti TTG
EMA (endomysial antibodies)
does screening diagnose coeliacs?
no, you need small intestine biopsy
treatment of coealic
remove wheat, rye, barley
complications of coealic (3)
Enteropathy associated T-cell lymphoma
Small bowel lymphoma
ulcerative jejunitis
management of crohns and UC
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
management of longstanding simple constipation
1st line = stool softners (macrogol laxative)
2nd line = stimulant (Senna)
diagnosis of Hirschsprungs
rectal biopsy shows absence of ganglion cells with presence of large acetylcholinesterase positive nerve trunks
management of Hirschspurng
in the 75% of cases it is localised to dial bowel:
- cut it out and form colostomy
- anatomise innervated bowel to anus later on
does hepatitis cause conj or unconj hyperbilirubinaemia
conj
choledocal cyst
dilation og bile duct from gall bladder to duodenum causing a pseudo-obstruction and jaundice
what does jaundice <24hrs mean
probs haemolysis and serious cause (rhesus, ABO, G6PD, HS)
what does jaundice 2d-2w mean
physiological or breast milk jaundice probs
complication of kernicterus
cerebral palsy
learning difficulties
cerebral deafness
complications of chronic liver disease
Nutrition (fat malabsorption, loss of fat soluble vitamins ADEK)
Pruritus
Encephalopathy due to ammonia
cirrhosis/portal hypertension (ascites, varices, spelnomeglay)
what is biliary atresia
progressive fibrosis and obliteration of biliary tree. unknown aetiology but causes chronic liver failure within 2y untreated.
presentation of biliary atresia
mild jaundice
pale stools
faltering growth
hepatosplenomegaly (portal hypertension)
investigations of biliary atresia
LFT - raised conj bili
US - contracted or absent gallbladder
ERCP - diagnostic
management of biliary atresia
surgery (palliative or liver transplant) and fat soluble vitamins supplementation
investigations of A1ATd
plasma anti trypsin is low
presentation of A1ATd
prolonged neonatal jaundice
bleeding (vitKdef)
hepatomegaly
what % of febrile children will have a UTI?
7%
what is the % recurrence of a UTI
12-30%
which bug for UTI is more common in boys
Proteus (can cause phosphate stones)
mild vs severe vesicoureteric reflux
mild = into ureter only severe = dilating renal pelvis and calyces as well
how does VUR lead to hypertension
reflux –> hydronephrosis –> infection will track up easily –> renal scarring –> hypertension
atypical UTI infection: refer or don’t refer
refer urgently for USS
when do you refer for a ‘within 6 weeks’ USS after a UTI
<3m
recurrent
glomerular vs non-glomerular haematuria
glomerular:
brown urine, deformed RBCs, casts +/- proteinuria
most common cause of haematuria
UTI
indications for renal biopsy
persistent proteinuria
recurrent haematuria
abnormal renal function
persistent abnormal complement
investigation of haematuria
Urine MC&S Blood pressure USS kidney and UT Bloods full-panel protein and calcium excretion/ACR
investigation of glomerular haematuria
Urine MC&S Blood pressure USS kidney and UT Bloods full-panel protein and calcium excretion/ACR
PLUS
ESR
complement levels
throat swab
hepB/C screen
presentation of nephritic syndrome
hypertension oliguria (AKI) periorbital oedema/pulmonary oedema haematuria proteinuria
most common cause of nephritic syndrome
80% are post-infectious (group A beta haemolytic strep)
other causes are IgA, HSP, SLE
special investigations of post-strep Glomerulonephritis
C3 (low)
ASOT (high)
Anti-DNAse B (high)
management of post-strep Glomerulonephritis
penicillin 10 day
+ management of AKI
commonest presentations of HSP
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
haematuria following URTI?
IgA nephropathy (1-2days after) in young adult males
OR
Post-streptococcal GN (1-2 weeks after) with low C3 complement
SLE main autoantibody
dsDNA
HUS age group
<5
Causes of HUS
E.coli O157
shigella
presentation of HUS
microangiopathic haemolytic anaemia
acute renal failure
thrombocytopenia
treatment of HUS
Mx is supportive
fluid
blood transfusion
dialysis
how do you best measure proteinuria
urine albumin to creatinine ratio
causes of nephrotic syndrome
MNCS (90%)
FSGS
complication of nephrotic syndrome
thrombosis (haemoconcentration and loss of antithrombin III), infection (loss of Ig in urine), hypvolaemia, hyperlipidaemia
management of nephrotic syndrome
90% respond to steroids
70% will relapse whether frequently or infrequently
penicillin prophylaxis for pneumococcus
gastric protection
immune modulators (tacrolimus, rituximab, levamisole)
investigation to diagnose diabetes insipidus and another to distinguish between types
ADH insensitivity or lack of production - cannot concentrate urine
therefore investigation is serum (high) and urine osmolality (low)
desmopressin test distinguishes
management of DI
central - give desmopressin
nephrogenic - difficult, encourage hydration
management of nocturnal enuresis
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
treatment of the hyperkalaemia in an AKI
cardiac stabiliser:
calcium gluconate
push K+ into cells:
bicarb
insulin/dextrose
salbutamol
get rid of K+:
calcium resonium
dialysis
treatment of the acidosis in an AKI
bicarb
+/- dialysis
stages of CKD
eGFR 1 = >90 2 = 60-90 3a = 45-60 3b = 30-45 4 = 15-30 5 = <15
top 5 causes for CKD in children
renal dysplasia +/- reflux (34) obstructive uropathy (18) glomerular disease (10) congenital nephrotic syndrome (10) PCKD (4)
presentation of CKD
mostly asymptomatic from stages 1-3
Sx include: bone deformity (vit D) hypertension (RAAS) proteinuria anaemia (EPO)
management of CKD
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
definition of hypertension in children
> 95 centile for height age and sex
100 when born, goes up by 2mmHg every year.
4 renal causes of hypertension
scarring
vasculitis
renal failure
coarctation/renal artery stenosis –> low blood to kidneys
what happens if you lower BP in malignant hypertension too quickly
watershed infarcts
where does the fluid in a hydrocele come from
a small gap in the processus vaginalis allows peritoneal fluid in (small version of a hernia defect)
blue spot on testes in acute pain
torsion of hydatid of Morgani
inguinal hernias more common on right or left
right 3x
when should inguinal hernias be surgically reduced (herniotomy) if manual reduction is not possible:
prem baby
infant
child
prior to discharge
within a month
elective
when should you operate on a hydrocele in an infant/child
if persisting beyond 2y
what % of boys have an undescended testes:
newborn
by 3 months
5%
1%
what do you do if the testes are impalpable bilaterally
karyotype for gender
pelvic ultrasound
management of cryptorchidism
orchidopexy at 1y old
time window for testicular torsion
6hrs
RF for testicular torsion
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.
match the following girl boy wolfian mullerina mesonephric paramesonephric
girl, mullerian, mesonephric
at what age are the genitlia fully formed
12 weeks
management of balanatis
warm water cleaning
hydrocortisone cream
imidazole cream
if purulent (bacterial), use fluclox PO
management of balanitis xerotica obliterate (true phimosis secondary to balanitis)
topical steroid
circumcision
behcets
oral ulcer
genital ulcer
arthritis
(anetrior) uveitis
amongst other things
most common cause of cerebral palsy
80% is antenatal stuff:
- cerebrovascular accident
- cortical migration disorder
- maldevelopment
- congenital infection
what % of CP is due to hypoxic ischaemic injuries around birth
only 10%
3 types of CP and where the lesion is
spastic = UMN (pyramidal or corticospinal tract) dystonic = basal ganglia or extrapyramidal tract ataxic = cerebellum
Sx of spastic CP
velocity dependent increased tone
Sx of dystonic CP
chorea
athetosis (Writhing movement)
dystonia (antagonistic contractions of trunk leading to twisting)
Sx of ataxic CP
poor coordination
limb hypotonia
ataxic gate
most common cause of dystonic CP
hyperbilirubinaemia from rhesus disease
which is the ‘worst’ type of CP in terms of GMFCS
dystonic CP, which is usually GMFCS 4 or 5
What are the GMFCS scores
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
what complications do you monitor for in CP
learning difficulty epilepsy visual impairment squint hearing loss speech disorder behaviour disorder undernutrition respiratory problems
medication for spasticity in CP
baclofen
medication for dystonia in CP
trihexyphenidyl
2nd line for dystonia and spasticity in CP
botulinum toxin
surgery option for CP
rhizotomy - cutting part of the tracts in the spine
which members of the MDT are used in managing CP and why
physio - improve family handling of child, advise activities to prevent complications
OT - equipment
dietician
SALT - swallow in early days and language therapy later
Psychologist
age range for febrile convulsions to occur
6m-6y (peak is 18m)
key question to ask in a seizure history in a child
fever
If the following area has a seizure, what Sx will there be? frontal temporal occipital parietal
frontal = motor temporal = aura, dejavu, lip smacking, plucking occipital = vision parietal = sensory
only motor Sx in absence seizure?
eyelid flicking
remission rate of absence epilepsy
80%
Mx of absence epilepsy
sodium valproate/ethosuximide
how does CECTS present
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
Mx of CECTS/benign rolandic epilepsy
very well managed by making sure child has enough sleep. sleep deprivation is a big trigger. unlikely to ever go away though.
typical age for:
absence epilepsy
CECTS
Juvenile myoclonic epilepsy
2-10
7-10
10-20
presentation of juvenile myoclonic epilepsy
myoclonic jerks on awakening (often in morning) in 10-20yo
investigations in epilepsy
ECG (don’t miss arrhythmia like long QT)
EEG
structural imagine: MRI, CT
functional imaging: PET, SPECT
1st line absence epilepsy
sodium valproate
1st line tonic clonic epilepsy
sodium valproate
1st line myoclonic epilepsy
sodium valproate
1st line focal epilepsy
carbamazepine or lamotrigine
2nd line epilepsy
ethosuximide, lamotrigine, clobazam, levetiracetam, topiramate, gabapentin
can children drive if they have epilepsy?
1 year seizure free
when can you stop antiepileptics
2 year seizure free
management of status epilepticus
0min = ABCDE, O2, DEFG 5min = IV lorazepam, buccal midazolam, rectal diazepam 15min = IV lorazepam 25min = phenytoin (or phenobarbitone) 45min = thiopental rapid sequence induction
what makes a febrile convulsion ‘complex’?
if it is focal as opposed to generalised tonic clonic
>15m
twice+ in same illness
do febrile convulsions increase chance of epilepsy?
simple –> no
complex –> slightly
advice for parents after febrile convulsion
1) can’t prevent with antipyretics
2) place in recovery position
3) try to film it
4) call 999 after 5 mins
parixymasal disorder
page 216
what is a breath holding attack and when do they resolve by
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
management of breath holding attack
reassurance
cause of vasovagal syncope
hypotension and decreased cerebral perfusion
how do vasovagal syncope episodes recover
by lying flat
what is a reflex anoxic syncope/seizure
sudden unexpected pain or fear causes a vaguely mediated bradycardia –> limp and falls to ground, pale, clonic jerks. lasts 1 minute with rapid recovery
Mx of reflex anoxic seizure
reassurance
what are parasomnias with examples
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
night terror vs night mare
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
by what age should there no longer be a head lag when pulling up a child to sitting
1 months
what question do you ask yourself when looking at a child with a motor disorder
Central or peripheral
once you’ve figured out a child has a central motor disorder, what’s the next question you ask yourself?
motor cortex (weakness) or basal ganglia (chorea, athetosis=writhing, movement initiation) or cerebellum (DANISHP)
once you’ve figured out a child has a peripheral motor disorder, what’s the next question you ask yourself?
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)
Gower’s sign
using hands and knees to stand up >3yo = duchenne sign
investigation for peripheral motor disorder
EMG nerve biopsy muscle biopsy nerve conduction study plasma CK DNA testing
mutation in spinal muscular atrophy
AR mutation of SMN1 gene for anterior horn cells
SMA aka?
spinal muscle atrophy aka Werdnig-Hoffman disease
prevalence of spinal muscle atrophy
1 in 6000 (2nd most common neuromuscular disease in UK)
What is SMA
progressive weakness and wasting of skeletal muscle cells due to degeneration of LMN in anterior horn of spinal cord
types of SMA
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
in what is the mutation for charcot marie tooth disease
myelin gene
inheritance of most common mutation of charcot maize tooth disease
CMTA.
2/3 AD inheritance
1/3 de novo
how does charcot marie tooth disease present
symmetrical slowly progressive distal muscle wasting (e.g. foot drop). lower limbs more affected than upper limbs
how do you diagnose charcot marie tooth disease
nerve conduction study
nerve biopsy = onion bulb (due to attempts at remyelination)
how does Guillain barre syndrome present
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
management of GBS
supportive
immunoglobulin can help
prognosis of GBS
90% recover, but can take up to 2 years.
LMN vs UMN facial palsy
upper spares upper
management of bells palsy
investigate for lyme disease and HSV
if HSV–>acicilovir
lubricate eye and wear patch if can’t close eye
cause of myaesthenia gravis
antibodies to nACh receptors in NMJ
presentation of myasthenia gravis
after 10yo eye movement problem ptosis loss of facial expression proximal muscle weakness
diagnosis of myasthenia gravis
improvement after neostigmine and identification of antibodies in blood
management of myasthenia gravis
neostigmine
immunosuppressive therapy
plasma exchange in crisis
inheritance of duchenne
X-linked recessive
but 1/3 are de novo
gene that is mutated in duchenne
dystrophin - connects muscle fibre to extracellular matrix - when mutated there is myofibre necrosis
incidence of duchenne
1 in 3000-6000 males
presentation of duchenne
waddling gait language delay towers sign psudoheypertrophy of calves leaning difficulties clumsiness
usual onset of duchenne
3-5
Becker’s muscular dystrophy
like duchenne, but not as bad a mutation so onset is later (11) and not as severe
life expectancy with duchenne
20s - because as breathing muscles become affected you get infections. also cardiomyopathy.
no longer ambulant by 10-14
presentation and age for dermatomyositis
5-10y symmetrical proximal muscle weakness (CK up) heliotrop rash gottron papules fever raised CRP/ESR
management of dermatomyositis
2yrs corticosteroids
immunosuppression
physio
how does friedrich ataxia present
similar to charcot marie tooth: worsening ataxia dysarthria distal wasting of limbs absent reflexes death by 40-50
what is friedrich ataxia caused by
AR mutation of FXN gene –> lack of frataxin protein
headache red flags
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
migraine more common in boys or girls
boys>girls
after puberty its women>men
how do cluster headaches present
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
how do you manage cluster headaches
triptans for acute attack
Ca blockers for prevention
headache type in raised ICP
worse lying down
worse in morning
wakes you up
morning vomiting
Mx of raised ICP
ABCDE
elevate to 25 degrees to help drainage
intubate, mannitol, refer to neuro centre if severe
what brain bleed is classic in NAI (non-accidental injury)
subdural
most common cause of meningitis
viral (enterovirus, EBVm adenosine, mumps)
bacterial causes of meningitis:
0-3m
GBS
Ecoli
Listeria
bacterial causes of meningitis:
3m+
Neisseria meningitides
strep pneumonia
(Haemophilis as well if <6)
kernig vs brudninski
kernig = hamstring stretch brud = neck flexion causes knee flexion
CI to LP
- focal neurology
- coagulopathy
- infection at LP site
- signs of raised ICP
- thrombocytopenia
- cardiorespiratory instability
what is the fluid challenge amount given to children
20ml/kg
what antibiotics are used
<3m = cefotaxime >3m = ceftriaxone
give cipro to rifamp to household contacts
what is X-linked adrenoleukodystrophy a disease of
peroxisome enzyme defect, leading to an accumulation of long chain fatty acids. it causes a neurodegenrative disorder
what is wilsons disease
- AR mutation of Chr 13
- Leads to abhorrent storage of copper in many organs –> liver failure, psychosis, extrapyramidal signs
- Presents in childhood around 12yo
how is wilsons disease diagnosed
Diagnosed by reduced copper/caeruloplasmin in blood and urine (as it’s all in the liver) or by liver biopsy
management of wilsons disease
low copper diet and penicillamine (copper chelation)
diagnosis of type 1
> 1 neurofibroma
6 cafe au lait spots
other things like axillary freckling, optic glioma, lisch nodule, relatives
mutation in neurofibromatosis
AD mutation of NF1 gene, but 50% have a de novo mutation
what is neurofibromatosis associated with
MEN syndromes
features of tuberous sclerosis
depigmented ash leaf shaped patches
angiofibrimata in butterfly distribution on bridge of nsoe
what is sturge weber syndrome
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
4 developmental domains
fine motor
gross motor
hearing and language
social
investigations for developmental delay
urine tests for metabolic problems TFTs chromosome analysis CGH array exome sequencing imaging (MRI)
fever + change in personality
encephalitis
catergories for causes of anaemia
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
what does low reticulocyte mean
red cell production issue
why os bilirubin helpful when looking at cause of anaemia
tells you if haemolysis is the problem
investigation for sickle cell and thalssaemia?
Hb electrophoresis
microcytic
normocytic
macrocytic
definitions
micro <83 fL
normal
macro >96 fL
Micro, normo or macro? iron def B12/folate haemolysis thalaseamia chronic disease hypothyroidism alcohol excess myelodysplasia
micro macro normo/macro micro micro/normo macro macro macro
iron def anaemia:
FBC
blood film
ferritin
microcytic, hypochromic anaemia with low ferritin
if no improvement in iron def anaemia, what should you investigate for
coeliac mockers diverticulum (chronic bleeding)
causes of red cell aplasia (anaemia)
parvovirus B19
diamond-blackfan congenital anaemia
red cell aplasia: reticulocyte count bilirubin Coombs test red cell precursors in bone marrow
Low
Normal
Negative for Abs
Absent
inheritance of hereditary spherocytosis and G6PDd
AD (northern european children)
G6PD = X-linked (African&Mediterranean boys)
How can HS and G6PD present
neonatal jaundice
gallstones
haemolysis after infection
Ix for HS and G6PD
HS = spherocytes on blood film G6PD = G6PD activity
incidence of sickle cell
1 in 2000
inheritance of sickle cell
AR
mutation in sickle cell
glu to val point mutation to form HbS chain
presentation of sickle cell
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
Sickle:
Hb
reticulocytes
LDH
Low
High
High
emergencies in sickle cell?
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
when to transfuse in sickle cell
in the 4 emergencies
long term medication for sickle cell
hydroxycarbamide
types of beta thalassaemia
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
clinical features of beta thalasaemia
- Normal foetal life (as and genes are ok so HbF can be made)
- Anaemia in first few months of life (as HbA take sover)
- Jaundice
- 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 - Splenomegaly/hepatomegaly (due to the extra-medullary haematopoiesis)
Mx of B thal
- Life long regular blood transfusions
- Iron chelation (to prevent iron overload from transfusions) = desferrioxamine
- Folic acid
- Bone marrow transplantation in early life
incidence of leukaemia
3 per 100,000
types of leukaemia in children
80% ALL
20% AML
types of ALL
75% common
15% T-cell
peak incidence of ALL
2-5
Sx and duration of onset of Sx in ALL
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
prevention of tumour lysis syndrome?
allopurinol: prevents uric acid production as all the cells break down
investigations for leukaemia
FBC blood film bone marrow examination clotting screen (?DIC) CXR (?mediastinal disease) LP (?CSF disease) Cytogenetic testing
management for leukaemia
correct abnormalities:
- RBC transfusion
- platelets
Chemotherapy:
- 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 - Some patients get a transplant
bad prognostic indicators for leukaemia
age < 2 years or > 10 years WBC > 20 * 109/l at diagnosis T or B cell surface markers non-Caucasian male sex
prognosis in ALL at 5 years
85%
prognosis of AML at 5 years
60-70%
bleeding:
muscles and joints
vs
mucocutaneous
haemophilia (coagulation cascade problem)
platelet disorders and vWF (primary haemostats and platelet plug formation)
inheritance of haemophilia
X-linked recessive
types of haemophilia
A = factor 8 deficiency B = factor 9 deficiency
when does haemophilia present
end of first year of life when they start crawling/walking/falling
what is the severity spectrum with haemophilia
severity depends on how bad the mutation to the gene is:
severe = sponteneous bleed
moderate = bleed after minor trauma
mild = bleed after surgery
what will the following show for haemophilia:
- aPTT
- PT
- F8&9
- FBC
- long
- normal
- low depending on type a or b
- normal
which factors are involved with:
- intrinsic pathway
- extrinsic pathway
- common pathway
- extrinsic = F7
- intrinsic = F8, 9, 11
- common = F10 and others
what does aPTT and PT test
activated PTT = intrinsic (8, 9, 11)
PT = extrinsic (7)
both = common
treatment for haemophilia in general
special treatment for haemophilia A
recombinant factor when bleeding
avoid aspirin, NSAIDs and IM injections
Hameophilia A can also have desmopressin, which releases endogenous stores of F8
what does vWF do
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
presentation of vWD
bruising
excessive bleeding after surgery
epistaxis or menorrhagia
treatment of vWD
- Tranexamic acid – reduces clot breakdown (a.k.a. an anti-fibrinolytic)
- DDAVP/Desmopressin – this releases endogenous FVIII and vWF. This is good for if you need a short term boost.
- vWF/FVIII concentrate – very expensive so is a last line therapy, but has to be used before things like surgery.
HSP more common in boys or girl
boys 2x
what is ITP
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
what do protein C and S do?
‘put brakes’ on the clotting cascade. so a deficiency means there’s no brake on the cascade and it is a pro-thrombotic state
investigations for a child with a VTE
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
presentation of lymphoma
painless lymphadenopathy over several months B symptoms: - night sweats - fever - weight loss
hodgkin vs non-hodgkin:
histology
age
reed sternberg cells in hodgkin
NHL = childhood
HL = adolescence
investigations for lymphoma
lymph node biopsy
bone marrow biopsy for staging
radiological assessment
bloods (LDH, FBC, urate, Ca)
cure rate for hodgkins
80%
long term complication from hodgkin
secondary malignancy or heart failure from radiotherapy at young age
what is burkitt’s lymphoma
a high grade B cell NHL:
- endemic (african) form is associated with EBV = in face and jaw
- sporadic form = ileocaecal tumours
Li-Fraumeni syndrome mutation
p53 germ line mutation causing early cancer (breast, leukaemia, sarcoma)
mutation causing WIlms tumour
WT1
most common solid tumour in children
brain
what does vomiting with no diarrhoea raise alarm bells of
brain tumour
key feature of medulloblastoma
cerebellar signs
key feature of astrocytoma
supratentorial signs: seizure, hemiplegia, focal signs
from what do neuroblastoma arise?
neural crest tissue in adrenal medulla and sympathetic nervous system (NOT BRAIN)
features of neuroblastoma
abdominal mass pallor, weight loss bone pain, limp hepatomegaly paraplegia proptosis
key investigation in neuroblastoma
urine catecholamines are raised:
raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
what is Wilms tumour
a nephroblastoma = cancer arising from embryonal renal tissue
commonest presentation of wilms tumour
abdominal mass
+/- haematuria
Tx of Wilms
chemo + nephrectomy
80% cure
age group for bone tumours
after puberty
M:F in bone tumours
M > F
presentation of bone tumours
persistent localised bone pain
2 types of bone tumours and most likely place to arise
Osteosarcoma (knee or shoulder)
Ewing’s (femoral diaphysis or hip)
prevalence in UK of T1DM
1 in 500
name a:
rapid
short
intermediate acting insulin
novorapid
humulin-S
humulin-I
what type of insulin regime do children start on?
basal bolus
how do the following affect blood glucose? illness alcohol anxiety/excitement stress menstruation hot weather
up down down up up down down
long term complications of diabetes
macrovascular = HTN, CVD, CHD microvascular = retinopathy, nephropathy, peripheral neuropathy
catergories for osce station looking at ‘assessing a child with diabetes’
assess diabetes - symptoms - complications assess impact on life - school - growth and development - psychological
biochemical cutoffs for DKA
sugar >11.1
blood ketones >3
VBG <7.3 +/- bicarb <15
treatment of DKA
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
causes of hypoglycaemia in infancy
transient neotatal hypoglycaemia
persistent hypoglycaemic hypoerinsulinism of infancy
causes of hypoglycaemia after neonatal period
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
Tx of hypoglycaemia
PO glucose Glucagon injection (if LOC) IV glucose (if glucagon doesn't work)
what is McArdle disease
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
what does RAAS do to:
- BP
- Na
- K
increases BP
increases Na
decreases K
CAH cause
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
CAH presentation
- virilisation of external genitalia
- salt loss adrenal crisis in 1-3 weeks
- tall stature, muscular build, acne
diagnosis of CAH
high 17OHP in blood
management of CAH
- lifelong glucocorticoids
- lifelong mineralocorticoids
- ?surgery to correct genitalia
What is addisons disease
primary adrenal insufficiency, often caused by AI destruction of adrenals
presentation of addisons:
- infants
- older
infants: salt loosing crisis (lowNa, highK, met acidosis)
older: lethargy and pigmentation
diagnosis of addisons
- plasma cortisol
- ACTH
- synacthen
- low cortisol
- high ACTH
- cortisol stays low on synacthen test
Mx of adrenal crisis
IV saline
glucose
hydrocortisone
Ix of cushings syndrome:
- dex suppression
- 24hr urine cortisol
- dex at night doesn’t suppress cortisol at 9am next morning
- high urinary cortisol
what type of T3 do fetuses make
reverse T3 = inactive derivative
incidence of congenital hypithyroidism
1 in 4000
causes of congenital hypothyrpidism
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
what is a Guthrie test
what it looks for in cong hypothy
the neonatal heelprick test done when babies are 5d old
raised TSH
Mx of cong hypothy
lifelong thyroxine from 2-3 weeks of age (otherwishe neurodevelopment will be very slow)
Mx of hyperthyroidism
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)
most common inherited metabolic disorder
phenylketonuria and MCADD (1 in 10,000)
Presentation and management of:
phenylketonuria
learning difficulties, seizures, microcephaly
phenylalanine restricted diet
Presentation and management of:
MCADD
hypoglycaemia if fasting
don’t fast
Presentation and management of:
glutaric aciduria type 1
macrocephaly with encephalopathic crisis 6-18m resulting in dystonic-dyskinetic movements
specialist diet and dialy carnitine
Presentation and management of:
isovaleric acidaemia
metabolic acidosis
low [protein diet and carnitine/glycine
Presentation and management of:
homocystinuria
marfanoid appearance, lens dislocation, osteoporosis, VTE
low protein diet and pyridoxine/folate
maple syrup urine disease
maple syrup smelling urine and encephalopathy in first week of life
low protein diet
causes of ambiguous genitalia:
- if female
- if male
- CAH, prenatal exposure to androgens
- 5areductase deficiency (no DHT from testosterone so can’t virilise), androgen insensitivity, impaired testes
how long is the growth phase for an infantile haemangioma
9m
how long do haemangiomas take to involute
50% by 5y
70% by 7y
Mx of haemangioma
Conservative
propranolol if complications such as obstructed vision, ulceration, pain, bleeding or disfigurement
What is PHACES
variation of infantile haemangiomas including: Posterior fossa defect Haemangioma Arterial abnormalities Cardiac abnormalities Eye abnormalities Sternal clefting
what is sturge weber syndrome
port wine stain (a flat haemangioma) in trigeminal distribution + neurological problems (seizure, developmental delay) + eye problems (blindness, retinal detachment, glaucoma)
incidence of port wine stain
1 in 300
natural progression of port wine stain
darken and thicken with time. permanent.
what are port wine stains caused by
slow flow capillary vascular malformations
what is an exanthem
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
caused of measles
paramyxovirus/morbillivirus
presentation of measles
conjunctivitis koplik spots cough THEN widespread maculopapular/morbilliform rash
complications from measles
encephalitis
pneumonia
otitis media
subacute sclerosing panencephalitus 7 years later (like dementia)
scarlett fever cause
group A strep
scarlett fever presentation
strawberry tongue
sandpaper rash
circumoral pallor
management of scarlett fever
penicillin
cause of rubella
rubella virus
presentation of rubella
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)
complications of scarlett fever
otitis media
pneumonia
glomerulonephritis
rheumatic fever
complications of rubella
congenital rubella syndrome in 1st trimester
diagnosis of rubella
unlike the other exanthems, this needs serology
SSSS cause
S. aureus toxins
SSSS presentation
Erythema following by blistering with Nikolsky’s sign.
No mucosal involvement
Pain, fever, shock (from fluid loss)
Mx of SSSS
IV Abx emolients analgesia dressings fluid and temperature balance
erythema infectiosum AKA and cause
a.k.a 5th disease a.k.a slapped cheek syndrome
caused by parvovirus B19
presentation of erythema infectiosum
slapped cheek for 4 days with circumoral sparing
mesh like net red appearance across buttocks and trunk after a week
roseola infantum presentation and cause
6m old
sudden onset fever which then GOES away AND THEN maculopapular rash appears
HHV6 and HHV7 (think that it is 6th disease)
causes of bullous and non bullous impetigo
non-bullous - S. aureus and Strep pyogenes (group A)
bullous - S. aureus
Mx of periorbital or orbital cellulitis
High dose ceftriaxone
what is a herpetic whitlow
painful erythematous oedematous white pustules on site of broken skin. typically fingers. caused by autoinnoculation
chicken pox cause
VZV (HHV-3)
how many herpes viruses are there and what are they
8: HSV1 - lip and skin lesions HSV2 - genital lesions VZV - chicken pox CMV EBV HHV6 - roseola HHV7 - roseola HHV8 - kaposi
after how long should new lions stop arising gin chicken pox
10 days, after which you worry of immunocompromise
cause of warts
human papillomavirus
how long do molluscum take to go away
up to a year
what is a kerion
a severe pustular ringworm (dermatophyte) infection
what is seborrhoeic dermatitis and aka
aka cradle cap
scales form thick yellow adherent layer on scalp of <3m baby
Mx of cradle cap
emolients
clears scales using ointments containing sulphur and salicylic acid
?topical steroid
What is nappy rash
an irritant contact dermatitis from urine/diarhoea on skin
where does nappy rash occur
Nappy area, but spares the flexures/skin creases
Mx of nappy rash
emollient + ?steroid
genetics in eczema
fillagrin null mutation
Mx of eczema (brief)
soap/irritant avoidance
emollients
steroids
what is erythema nodosum
submit fat inflammation causing tender red nodules on skins
erythema multiforme appearance
target lesions WITH A CENTRAL RED PAPULE
area of body most likely to be injured in a fatal childhood accident
head injury
presentation of brain bleed in children: neuro or shock
compared to adults, relatively more shock because the sutures aren’t fused so there is less mass effect and fewer neuro signs
management of choking
unconscious = CPR
conscious:
child = 5 abdo thrusts
infant = 5 back blows, 5 chest thrusts
when to refer to burns unit
- partial thickness burn >5% of - body surface area
deep partial thickness/full thickness - face, ears, hands, feet, genitalia, perineum, major joint affected
grade of burns
which grades appear dry
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
when is activated charcoal useful in a poisoning
within 1 hour of poisoning to prevent some of it being absorbed
Sx of paracetamol overdose
Early = abdo pain + vomiting
Late (12-24hr) = liver failure
Mx of paracetamol overdose
Serum paracetamol
concentration, INR and LFT
IV NAC if required
Sx of salicylate overdose
Early = tinnitus + vomiting + hyperventilation (respects alkalosis) Late = metabolic acidosis
Mx of salicylate overdose
Serum salicylate
Alkalinisation of urine
Haemodialysis
Sx of alcohol overdose
Hypoglycaemia
Coma
Respiratory failure
Mx of alcohol overdose
Serum ETOH
Treat hypoglycaemia
Support ventilation
what is blount disease
severe progression of unilateral leg bowing (genu varum)
what measurement can be used to see whether someone with knock knees (genu valgum) is normal
Intermalleolar distance <8cm when standing with knees together
pathological cause of genu valgum
JIA
examination for flat feet
Ask to go on tip toes and observe arch
Aldo passively extend big toe
do you refer flat feet?
if there is evidence of a pathological cause:
hyper mobility, contracture, tarsal coalition, JIA
causes of in-toeing:
- infant
- toddler
- child
metatarsus adductus
medial tibial torsion
persistent anteversion of femoral neck
clubfoot name and deformity
talipes equinovarus:
- inversion and supination of foot with plantarflexion
- shorter foot and thin calf muscles
- foot is fixed
incidence and M:F of clubfoot
1 in 1000
male x2
Mx of clubfoot
Ponsetti method = plaster casting and bracing
Severe = corrective surgery
Deformity in talipes calcaneovalgus
dorsiflexion and turned out/everted
definitions: talipes:
- varus
- valgus
- equinus
- calcaneus
turned in (like inversion) turned out (like eversion) like plantarflexion like dorsiflexion
Mx of calcaneovalgus
self corrects with exercises
how does tarsal coalition present
progressively more rigid foot, presenting in pre-adolescent years
Pes cavus deformity
high arched foot.
Pes cavus in older children: association?
Neuromuscular disorder (e.g friedrich ataxia, hereditary motor sensory disorder)
how might DDH present
neonatal screening
Limp/abnoaml gait
type of pain in growing pain
3-12y at night, symmetrical NEVER at start of day doesn't limit activities pGALS normal
score for hypermobility
beighton score
>6/9 is hypermobile
Mx of hypermobility
Investigate for Marfan or Ehlers-Danlos
MDT treatment: PT/OT, orthotics, simple pain relief
what is complex regional pain syndrome and Mx
non-organic dramatic MSK pain with hyperaesthesia and allodynia. Mx is PT based
presentation of transient synovitis
<5yr
hip pain referred to knee with a preceeding URTI
mild fever (not really ill like septic arthritis)
improves in a few days
Mx of transient synovitis
Worried about differential of septic arthritis, so do Xray, FBC, CRP, USS
If unsure joint aspirate and cultures
Presentation of perthes disease
young boy 5-10
insidious onset of limp, hip/knee pain.
Imaging in perthes disease or SUFE
frog leg lateral view Xray of both hips to show AVN
MRI can be helpful
does the hip reossify in perthes disease? Any long term consequences?
Yes, over 18-36 months. Risk of degenrative arthritis in adult life
Mx of perthes
Rest and PT to optimise hip movement
Surgery sometimes used
Presentation of SUFE
hip pain and limp can be insidious or acute. In obese 10-15yr old boy.
Mx of SUFE
urgent referral and surgical pin fixation
cause of chondromalacia patellae
soft cartilage of patella causes pain when tightly opposed to femoral condyles (pain walking up stairs/getting up from chair)
presentation of reactive arthritis
pain, swelling, redness, restricted ROM oligoarthritis FOLLOWING an infection. Usually not ill like in septic arthritis, and usually affects more than one joint.
Mx of reactive arthritis
NSAIDs and reassurance
usually place for septic arthritis
knee
common age for septic arthritis
<2
pathogen for septic artheitis
S. aureus
Mx of septic arthritis
Get joint aspirate and send it off
IV Abx
Surgical washout if no improvement or if in hip
immobilise and then subsequent mobilisation
what is JIA
umbrella term for different types of juvenile arthritides:
- systemic
- oligoarthritis (most common)
- polyarhtirits
- psoriatic
- enthesitis related
What is Still’s disease
systemic arthritis type of JIA where there is arthritis AND fever, rash, hepatosplenomegaly, lymphadenppathy
in polyarthritis type JIA, who is RF+
10-16yo.
the 1-6yo tend to be RF-
which JIA subtype is more common in boys
enthesitis related arthritis
incidence of JIA
1 in 1000
complications of uveitis
posterior synechiae, cataract, glaucoma, band keratopathy
management of uveitis
Steroid (drops, oral, IV)
Methotrexate
Humira
common complication of JIA
chronic anterior uveitis 33%
Need ophthalmological review every 3m
management of JIA
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
common spread type in chilren with osteomyelitis
haematogenous
compared to adults where its usually contiguous (e.g. trauma)
common area for haematogenous spread in osteomyelitis
metaphyseal area of long bone
presentation of osteomyelitis
PAINPAINPAIN pseudo paresis (can't move limb)
Mx of osteomyelitis
several weeks of IV Abx and oral therapy afterwards. main need surgery to debride or drain
common site of osteoid osteoma
spine
what is Scheuermann disease
osteochrontisi of vertebral body
what is adams forward bend test
ask someone with scoliosis to bend over and touch toes
- scoliosis resolves = functional scoliosis
- scoliosis doesn’t resolve = structural scoliosis
what is torticollis
dystonic condition causing abnormal neck position
most common cause of torticollis
sternomastoid tumour, which occurs in first few months of life. presents as a mobile non tender nodule in body of sternocleidomastoid muscle
mutation in achondroplasia
FGFR3 AD
but 50% are new mutations
what is cleidocranial dysostosis
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
what is arthrogryposis
defined as >2 congenital contractures, associated with oligohydramnios or chromosomal dosorders
most common type of osteogenesis imperfecta and presentation
type 1 = AD = fractures in childhood, blue sclera, hearing loss
Mx of OI type 1
bisphosphonates and fracture splinting
presentation of type 2 OI
still born
what is osteopetrosis
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!
marfans syndrome inheritance
AD
risks with marfans syndrome
cardiovascular degeneration of media of vessel walls resulting in dilates aortic root. need echo monitoring
what do you physically give in active vs passive vaccination? which is longer lasting
Passive = antibody. immediate effect but short lasting Active = antigen (because then the body has to actively make an antibody)
CI of giving vaccine
Don’t give to someone in acute illness
Don’t give live vaccine to immunocompromised
how are most primary immunodeficiencys inherites
X linked
SPUR for infections in immunodeficiency
severe
prolonged
unusual
recurrent
what broad spectrum antibiotic should you use in children with sepsis of unknown origin
<1m = cefotaxime + amox >1m = ceftriaxone
presentation of mumps
parotitis (initially unilateral and then bilateral) with fever and malaise –> otalgia + pain on eating
Mx of mumps
supportive
complicaitons of mumps
hearing loss
meningitis/encephalitis
orchitis (unilateral and in post-pubertal children)
presentation of glandular fever
lymphadenpathy tonsilitis + soft palate petechiae fever/malaise splenomegaly 50% rash/jaundice sometimes
specific lab investigations for glandular fever (3)
- abnormal lymphocytes
- +ve mono spot test
- seroconversion with 3 antibodies (viral capsid, IgG and IgM EB nuclear antigen)
what antibiotic do you avoid in EBV infection and why
amoxicillin –> rash
how do enteroviruses present
90% nonspecific febril eillness
D&V
Rash (can be non-blanching petechial)
is hand foot and mouth disease painful
yes, that’s why children present unable to eat or drink. it is a painful vesicular rash
what is herpangina
vesicular and ulcerated lesion son soft palate and uvula caused by enterovirus.
what is hand foot and mouth disease caused by
Picornaviridae family, most commonly:
coxsackie A16
enterovirus 71
Most common cause of meningitis overall
enterovirus
what is bornhol disease
pleural disease: an acute illness caused by enterovirus causing fever, pleuritic chest pain and muscle tenderness. self limiting
MSSA Abx
fluclox
cipro for broader cover
MRSA Abx
Vancomycin
Clindamycin
Most common type of group A strep
Strep pyogenes
+ve coccus
How does toxic shock syndrome occur?
S. aureus or Group A strep produces an exotoxin that acts as a superantigen. can arise uncommonly from any site of infection
presentation of toxic shock syndrome
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
management of toxic shock syndrome
ABCDE in ICU
surgical debridment of obvious areas of infection
Ceftriaxone and clindamycin
IVIG
types of nec fash
type 1 = mixed aerobe and anaerobe
type 2 = strep pyogenes
risk groups for types of nec fash
type 1 = diabetics post op
type 2 = IVDU
presentation of nec fash
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
Mx of nec fash
Benzyl pen + fluclocacillin + metronidazole (to cover anaerobes)
extensive surgical debridement
What do T lymphocytes do
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
types of T lymphocyte and what they do
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
how does HIV infect the CD4 cell
interacts with CD4 TCR alongside CCR5 coreceptor and virus can get into the cell. reverse transciptase enzyme lets virus replicate
4 stages of HIV infection
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
what is hairy leukoplakia and what does it mean
white plaque son side of tongue. it is an AIDS defining EBV infection of the tongue
how do you diagnose children with HIV
>18m = serology to detect IgG against HIV <18m = serology can only confirm exposure due to maternal antibodies
how can you confirm a child is not infected if mum had HIV
Once postnatal antiretroviral therapy is completed –> 2x -ve HIV PCR within first 3m of life
What levels of CD4 indicate:
- normal
- asymptomatic HIV
- AIDS
- about to die
> 500
200-500
<200
<50
what is the transmission rate from mother to baby completely untreated when breastfeeding
25-40%
here in the UK, what advice is given to prevent vertical transmission of HIV
ART during and after pregnancy
post exposure prophylaxis after birth for baby
avoid breast feeding
pre-labour caesarean if viral load is high
cause of lyme disease
spirochete:
Borrelia burgdorferi
presentation of lyme disease
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
Mx for lyme disease
<12y = amox >12y = doxycycline
complicated = IV ceftriaxone
what does cyclical fever every few days raise alarm bells of in someone who has recently been travelling
malaria
typhoid/paratyphoid cause
caused by salmonella group (e.g. salmonella typhi)
presentation of typhoid
- 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
Mx of typhoid
Co-trimoxazole, chloramphenicol, ampicillin
ENT wise, what are gromits used for
to resolve conductive hearing loss Sx in otitis media with effusion (glue ear)
describe limitation of newborn hearing screening
otoacoustic emission test cochlea
audotry brainstem response tests brainstem
neither tests auditory nerve
most common age for acute otitis mediA
6-12 months
what can you do if grommets fail in children
adenoidectomy (remove adenoids which opens Eustachian tube on the mouth end more to allow gunk out)
when do you give antibiotics in otitis media, and what course do you prescribe
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
cause of childhood obstructive sleep apnoea
adenotonsillar hypertrophy
incidence of trisomy 21
1 in 650
most common cause of the reason trisomies occur
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
appearance of trisomy 21
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
% of downs with congebnital heart disease`?
40% AVSD
appearance of Edwards syndrome
small mouth and chin prominent occiput clenched fist with overlapping fingers malformed ears rocker bottom feet and flexed big toe
prognosis with Edwards/pataus syndrome
most die in infancy
appearance of pataus syndrome
small or absent eyes
cleft lip and palate
polydactyl
presentation of turners syndrome
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
what can you give around puberty for a girl with turners
oestrogen replacement therapy to help her go through puberty
what is wrong genetically in fragile X syndrome
trinucleotide repeat in X chromosome
appearance of fragile X
long face
large everted ears
learning difficulty
macro-orchidism
complications of fragile X
mitral valve prolapse
scoliosis
noonan syndrome inheritance
AD
presentation of noonan syndrome
very similar to turners: broad forehead drooping eyelids wide eye distance short webbed neck trident har line pectus excavatum short pulmonary stenosis
Williams syndrome presentation
Short stature Learning difficulties Friendly, extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis
presentation of Prader-willi
hypotonia
hypogonadism
obesity
genetic investgations options from most crude to most precise
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).
% child mortality
4.5% (6 million per year)
top 2 causes of child mortality <5
50% is infectious diseases, of which pneumonia is most common
50% is neonatal death
(if you include 1-15yo, then malignancy accounts for 24%)
at what income level is poverty defined as
60% less than median income
when does a child attain 6/6 vision
5 yo
what is corneal light reflex used for
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
amblyopia?
lazy eye
brain and eye not moving in sync
myopia
hypermetropa
long sighted
short sighted
Short = myopia (its a shorter word)
what is astigmatism
refractive error of the lens meaning there is a constant, slight blur at any distance
what is the UK healthy child programme
from 0-19 years, that is the overarching name for the plan for:
- screening
- immunization
- developmental reviews
- heatlh promotion/education
4 basic human rights of child
from 1989 act:
- survival
- development
- protection
- participation
4 types of abuse
physical (80% mother!)
sexual
emotional
neglect
most common symptom in CFS
other Sx
post-exertional malaise and fatigue
cognitive dysfunction sleep disturbance muscle pain joint pain headaches sore throat
is there a trigger in CFS
yes, usually some sort of viral infection, which because of a genetic predisposition causes CFS to start
TOTT investigation screen
FBC ESR/CRP U/E LFTs CK TTG TFTs ferritin urine dip BP
Management of CFS
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)
is the pain real in chronic pain syndrome
YES, remember pain is an emotion not a physical thing and chronic pain syndrome is a wiring problem leading to allogynia/hyperalgesia
definition of ADHD
- inattention
- hyperactivity
- impulsivity
must be >6m and in at least 2 settings
screening questionnaire for ADHD
connors
ways to gather information when making ADHD diagnosis
parental interview
conors questionnaire
school observation
school reports
Mx of ADHD
non-medical
parent education
school education/special assistance/strategies
CBT
methyphenidate CNS stimulant (SE appetite down). atomoxetine can also be used.
triad in autism spectrum disorder
impaired social interaction
SAL delayed development
routines/ritualistic behaviour
diagnostic tools for autism
ADOS (done by SALT) = Autism Diagnostic Observation Schedule
3DI (parent questionnaire)
is autism associated with epilepsy/seizures?
yes
what is a normal IQ
> 80
<35 is severe learning difficulties
are eating disorders associated with social class
no, but it is a problem of western society
SCOFF questions for anorexia
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
what is lanugo hair
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.
can you have bulimia without being underweight?
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
red flags for anorexia nervisa
BMI<13 weight loss >1kg/week T <34.5 hypotensive <80/50 HR <40 sats <92% or limbs blue purpura electrolyte abnormality
what is refeeding syndrome
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?’`
mortality of anorexia nervisa
5-10%
Mx of refeeding syndrome
vitamin B
stop refeeding and reintroduce slowly
screen for DDH in breech baby with a normal newborn exam?
Yes
cradle cap (Seb dermatitis) treatment
mild = baby oil/shampoo severe = topical hydrocortisone
diagnosis of pertussis
per nasal swab
conservative treatment for chickenpox
topical calamine lotion
what syndrome is neonatal hypotonia most associated with
prader-willi
average age for GOOD pincer grip
cut off age for early pincer grip
12 months
12 months
elfin facies = ?condition
williams syndrome
flattened philtrum = ?condition
foetal alcohol syndrome
asthma, SABA not working. what’s the next step:
<5
>5
<5 = moderate dose ICS
> 5 = low dose ICS
until what age is a phimosis okay?
2y
how much of a fever do children with bronchiolitis have?
low grade (<39). If it’s >39, suspect pneumonia (esp. if focal as opposed to generalised crackles)
Main sequelae of respiratory distress syndrome in premature baby
retinopathy of prematurity
renal failure
paediatric basic life support
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
when do majority of children achieve day and night urinary continence
3-4
initial management of Hirschsprung’s disease
bowel irrigation (whilst biopsy comes back)
Roseola infant cause, presentation, complications and ?exclusion
HHV6 high fever for few days THEN rash nagayama spots on uvula/palate aseptic meningitis/hepatitis exclusion not required
management of exompahlos vs gastroschisis
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
do you grow out of a cows milk allergy
yes, by age 5, but usually by age 1-2
management of cows milk protein allergy
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
risk factors for surfactant lung disease
(prematurity) male sex diabetic mothers caesarean second born of premature twins
turners syndrome heart auscultation sign and why
ejection systolic due to bicuspid aortic valve
dietary advice for CF
high calorie
high fat
pancreatic enzymes every meal
M:F in DDH
female 6x
other RFs are breech, FH, firstborn, oligohydramnios, macrosomia
painless massive GI bleed in 1-2 year old?
rule of 2s?
Meckel’s diverticulum
2% of population
2 feet from ileocaecal valve
2 inches long
viral wheeze age group and management
pre-school children
1st line = SABA + spacer
2nd line = montelukast and inhaled ICS
contraindications for MMR (5)
severe immunosuppresion
allergy to neomycin
other live vaccine <4wks ago
IVIG therapy in past 3m
what is choanal atresia?
presentation?
management?
- 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.
ebsteins anomaly?
presentations?
cause?
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
haemophilia in a girl???
think about turners syndrome (only one X so susceptible to X-linked recessive)
competent use of knife and fork age?
5 years
4 prongs of a fork and one knife
distribution of atopic eczema in an infant?
face and trunk
maternal cause of orofacial clefts?
maternal anti-epileptic use
can you go to school with head lice
yes
full name for mongolian blue spot
congenital dermal melanocytosis
drawing circle
3 years
because you need 3 things: pen, paper and hand
cystic hygroma vs branchial cyst
cystic hygroma = posterior triangle, soft, transilluminate
branchial cyst = lateral in anterior triangle. anechoic on USS.
multiple miliary opacities in both lungs (not TB)?
pneumonia from chickenpox.
one of the 3 main complications of varicella:
- pneumonia
- encephalitis
- disseminated haemorahgic chickenpox
what happens if you miss DDH
arthritis in your 30s
if a mum misses MMR vaccine but now wants it, what can you do
give one now and then in 3m
if >10 or in an outbreak&<10, 1m is ok
fragile X heart problem
mitral valve prolapse
when do you worry about undescended testes
review at GP @3m and make referral to surgeon
surgery by 6m
