Paeds Flashcards
what is croup
viral laryngotracheobronchitis
stridor caused by laryngeal oedema and secretions
which organisms cause croup
parainfluenza viruses
when is croup more common
autumn
what are the symptoms/signs of croup
stridor
barking cough
fever
coryzal symptoms
how is croup diagnosed
clinically
what might you see on cxr of croup
PA view shows subglottic narrowing (steeple sign)
what treatment for croup
oral dex 0.15mg/kg
admit if stridor at rest
what emergency treatment for croup
high flow o2
nebulised adrenaline
what causes epiglottitis
haemophilus influenzae type b
what are symptoms of epiglottitis
fever
stridor
drooling
tripod position
how is epiglottitis diagnosed
- clinical by trained airway staff
what sign on epiglottitis xray
lateral view - thumb sign - swelling of epiglottis
what management of epiglottitis
immediate senior involvement
endotracheal intubation may be necessary to secure airway
oxygen
cefotaxime IV
what type of vasculitis is kawasaki
medium vessel
what are symptoms of kawasaki
- fever resistant to antipyretics
- cervical lymphadenopathy
- conjunctival injection
- bright red cracked lips
- strawberry tongue
- palmar, sole erythema, desquamation
how is kawasaki managed
high-dose aspirin
IVIg
echocardiogram to screen for coronary artery aneurysm
How is duchenne’s transmitted, what gene?
x linked recessive inherited disorder of dystrophin genes on xp21
what are the symptoms of duchennes
- proximal muscle weakness
- calf pseudohypertrophy
- Gower’s sign
- some patients have intellectual impairment
what investigations for muscular dystrophies
genetic testing (replaced muscle biopsy) creatinine kinase
what management for muscular dystrophies
supportive
oral steroids can delay progression weakness
creatinine supplements can give some improvement in muscle strength
what condition associated with duchenne’s
associated with dilated cardiomyopathy
how is beckers muscular dystrophy inherited
x linked recessive, although dystrophin gene not as severely affected as in duchennes
how is beckers different to duchennes and how are they managed
symptoms appear later (8+ y) in becker than duchenne
mgmt similar to duchennes
what is immune thrombocytopaenic purpura
immune mediated reduction in platelet count
what are antibodies directed against in ITP and what type of hypersensitivity reaction is this
GP IIb/IIIa or Ib-V-IX complex
type 2 hypersensitivity
how does ITP present
usually following viral illness or vaccination
bruising
purpuric rash
bleeding less common (gingival/epistaxis)
how is ITP investigated
FBC - isolated thrombocytopaenia
blood film
bone marrow studies only if atypical features such as lymphadenopathy, splenomegaly or if failure to resolve
when would bone marrow studies be indicated in ITP and what would they show
lymph node enlargement
splenomegaly
failure to respond to treatment/resolve
megakaryocytes
what mgmt of ITP
usually resolves by 6mths
oral/IV steroids
IVIg
platelet transfusion if active haemorrhage
explain pathophys of hirschsprung’s
aganglionic segment of bowel due to developmental failure of parasympathetic auerbach’s and meissner plexuses
uncoordinated peristalsis -> obstruction
what is hirschsprung’s associated with
down’s
more common in males
how would hirschsprungs present
failure to pass meconium by 3 days
constipation, abdo distension
how is hirschsprungs diagnosed
AXR
gold standard: rectal biopsy
how is hirschsprung’s managed in the immediate setting and definitively
immediate: rectal irrigation to prevent enterocolitis
definitive - surgical resection of affected segment of bowel
what is congenital diaphragmatic hernia
incomplete formation of diaphragm
herniation of abdominal viscera into thoracic cavity causing pulmonary hypoplasia and hypertension
how would congenital diaphragmatic hernia present
respiratory distress
tinkling bowel sounds in thorax
absent heart sounds on left hand side
what is best initial mgmt of congenital diaphragmatic hernia
intubate and ventilate
NG tube to prevent air going into the stomach
what is gastroschisis?
congenital defect in anterior abdominal wall just lateral to the umbilicus
what is the management of gastroschisis
vaginal delivery
immediate surgical correction (within 4 hours)
what is exomphalos
abdo contents protrude through abdo wall but are covered in amniotic sac covered in amniotic membrane and peritoneum
how is exomphalos managed
c-section to decrease chance of rupture
staged surgical correction
what is exomphalos associated with
down’s
beckwith wiedemann syndrome
cardiac and renal problems
what is gastroschisis associated with
socioeconomic deprivation - smoking, drinking during preg
how is candidal nappy rash treated
topical imidazole
how do infantile spasms present
flexion of head arms trunk followed by extension of arms
progressive mental handicap
what investigations for infantile spasms
EEG - hypsarrhythmia
CT - diffuse/localised brain disease eg. tuberous sclerosis
how is infantile spasms treated
vigabatrin
ACTH/pred
what is necrotising enterocolitis
disorder affecting prem neonates where a segment of bowel becomes necrotic
what RFs for nec
very low birth weight formula feed respiratory distress/assisted ventilation sepsis PDA and congenital heart disease
how would NEC present
bilious vomiting intolerance to feeds distended tender abdomen blood in stools absent bowel sounds
how would perforated NEC present
peritonitis and shock, severely unwell neonate
what investigations for NEC
AXR
bloods - FBC, CBG, blood cultures, CRP
what would AXR show in NEC
- dilated loops of bowel
- gas in the bowel wall (pneumatosis intestinalis)
- bowel wall oedema
- gas in the peritoneal cavity if perforated (pneumoperitoneum)
- gas in portal veins
how is NEC managed
- NBM, IV fluids, total parenteral nutrition
- antibiotics
- immediate referral to neonatal surgical team for:
- surgical removal of necrotic bowel
what would CBG of NEC show
metabolic acidosis
what is pityriasis versicolour (aka tinea versicolour)
superficial cutaneous fungal infection caused by malassezia furfur
how does pityriasis versicolour present
affects trunks
hypopigmented/pink/brown lesions
scaly
mild pruritus
what are RFs for pityriasis versicolour
- immunosuppression
- cushing’s disease
- malnutrition
what treatment for pityriasis versicolour
topical ketoconazole
if persistent - oral itraconazole
what is pityriasis rosea
acute self-limiting rash affecting young adults
thought to be linked to HHV-7 (herpes hominis)
how does pityriasis rosea present
- some prodromal viral illness
- herald patch on trunk
- followed by erythematous oval scaly patch in fir tree distribution
how is pityriasis rosea treated
self-limiting within 6-12 weeks
what are RFs for developing epilepsy following febrile convulsions
- family history of epilepsy
- complex febrile seizures
- background of neurodevelopmental disorder
what is biliary atresia
atresia/occlusion of biliary ducts
how does biliary atresia present
- obstructive jaundice after 2 weeks
- dark urine and pale stool
- appetite and growth disturbance
what signs would you find on examination of biliary atresia
- hepatosplenomegaly
- jaundice
- abnormal growth
- cardiac murmurs (associated with cardiac abnormalities)
what investigations for biliary atresia
- serum bilirubin - total bilirubin may be normal, but conjugated is VERY HIGH
- LFTs cannot differentiate between causes of jaundice
what management of biliary atresia
surgical - kasai portoenterostomy
or liver transplant
what complications of biliary atresia
cirrhosis
portal hypertension
liver failure
why does biliary atresia not cause kernicterus
liver is still able to conjugate bilirubin. conjugated bilirubin is unable to cross BBB
what vitamin deficiencies are associated with biliary atresia and how would this present
ADEK, fat soluble vitamins
failure to thrive
bleeding
what is osteomalacia
softening of bones secondary to vit D deficiency
which organism causes measles
RNA paramyxovirus
what are the symptoms of measles
prodromal:
- fever
- irritability
- conjunctivitis
- koplik spots
- rash - macpap starts behind ears and spreads, becomes confluent
- some desquamation sparing palms and soles
10% get diarrhoea
what are the complications of measles
otitis media pneumonia encephalitis subacute sclerosing panencephalitis corneal ulceration/keratoconjunctivitis diarrhoea increased incidence of appendicitis myocarditis
what investigation for measles
IgM antibodies
what mgmt for measles
supportive
if preg/immunosuppressed admit
what mgmt for measles contacts
MMR vax within 72 hours (vax immunity develops faster than natural immunity)
what causes mumps
RNA paramyxovirus
what symptoms of mumps
- fever
- malaise/muscle aches
- parotitis - ear pain, pain on eating. unilateral -> bilateral
what complications of mumps
- orchitis
- hearing loss (unilateral, transient)
- meningoencephalitis
- panencephalitis
- pancreatitis
what treatment for mumps
supportive, PHE notification
when is mumps infectious
7 days before parotitis and 9 days after
how is mumps spread
droplets
respiratory tract epithelial cells -> parotid cells -> other tissues
what topical Abx if resistance suspected for impetigo
mupirocin
oral fluclox/erythromycin if systemic infection
what is bullous impetigo
- caused by staph aureus bacteria releasing epidermolytic toxins causing vesicles to form
vesicles burst and release exudate forming golden crust without scarring
what is staphylococcus scalded skin syndrome
extensive bullous impetigo
what treatment for bullous impetigo/staph scalded skin syndrome
oral/IV fluclox
what complications of impetigo if caused by strep pyogenes
post-streptococcal glomerulonephritis
scarlet fever
what causes rubella
togavirus
what are the symptoms of rubella
fever
macpap rash starts on face and spreads
lymphadenopathy - suboccipital and post-auricular
what are the complications of rubella
arthritis
thrombocytopaenia
encephalitis
myocarditis
what are the features of congenital rubella syndrome
- sensorineural deafness
- congenital heart disease eg PDA
- congenital cataracts
- salt and pepper chorioretinopathy
- micropthalmia
- purpuric skin lesions
- cerebral palsy
- growth retardation
- hepatosplenomegaly
how should cases of rubella in preg be managed
discuss with local health protection unit
avoid contact with other pregnant women
offer MMR vaccine post-natally
what causes chickenpox
varicella zoster virus
how is chickenpox spread
respiratory
how does chickenpox present
fever
itchy rash - mac then pap then vesicular
mild systemic upset (fatigue, malaise)
how is chickenpox managed
trim nails, keep cool
calamine lotion/chlorphenamine for itching
how is chickenpox isolated?
until all lesions have dried and crusted over, usually about 5 days from onset of rash
what are the complications of chickenpox
- secondary bacterial infection
- group A strep -> necrotising fasciitis
- pneumonia
- encephalitis - ataxia
- disseminated haemorrhagic chickenpox
- pancreatitis
- arthritis
- nephritis
- conjunctival lesions
how does chickenpox encephalitis present
ataxia
where does chickenpox virus lie dormant
sensory dorsal root ganglion and cranial nerves
how do you manage chickenpox exposure in preg
check blood urgently for varicella antibodies
if none
<20 weeks - VZIG immediately
>20 weeks - aciclovir 10-14 days post exposure
how do you manage confirmed chickenpox in preg
seek specialist advice
if rash - acyclovir. use with caution in <20 weeks
how does fetal varicella syndrome present
skin scarring microcephaly micropthalmia limb hypoplasia learning difficulties
what are the complications of maternal chickenpox exposure
fetal varicella syndrome
severe neonatal varicella
neonatal shingles
who is vaccinated against whooping cough and when
children and pregnant women 16-32 weeks
how does whooping cough present
fever cough lasting 14+ days cough, worse at night, paroxysmal cough inspiratory whoop post-tussive vomiting apnoeic attacks in young infants
how is pertussis diagnosed
nasal swab with PCR/bacterial culture
how is pertussis managed
- if under 6m, admit
- notifiable disease
- macrolide if <21 days (clarithromycin, erythromycin in preg)
which antibiotics for pertussis in normal/preg
clarithromycin
erythromycin if preg
co-trimoxazole
what are the complications of whooping cough
- bronchiectasis
- subconjunctival haemorrhages
- pneumonia
- seizures
- pneumothorax
how should household contacts of pertussis be managed
prophylactic antibiotics (clari/erythromycin) offer pertussis vaccine
how long exclusion for whooping cough
21 days from onset of cough
48 hours after starting Abx
what are the causes of cerebral palsy
antenatal:
- maternal infections
- trauma in pregnancy
perinatal
- birth asphyxiation
- pre-term birth
postnatal:
- meningitis
- head injury
- severe neonatal jaundice
what are the different types of cerebral palsy
spastic (UMN damage)
dyskinetic (basal ganglia damage)
ataxic (cerebellar damage)
mixed
what symptoms of spastic cerebral palsy
spasticity, UMN signs - hyperreflexia, hypertonia, power same or reduced
what symptoms of dyskinetic cerebral palsy?
athetoid movements
problems controlling muscle tone (hyper and hypotonia)
oro-motor problems
what symptoms of ataxic cerebral palsy?
problems with coordinated movement and cerebellar signs
what is extrapyramidal cerebral palsy?
another word for dyskinetic cerebral palsy
so is athetoid cerebral palsy
how is cerebral palsy managed
multi-disciplinary team approach
- baclofen PO/ intrathecal for spasticity
- botox for spasticity
- selective dorsal rhizotomy for spasticity
- tenotomy for contractures
- glycopyrrhonium for secretions
- antiepileptics for seizures
what treatment for hypoxic-ischaemic encephalopathy (HIE)
therapeutic cooling to target of 33-34, rectal temp probe
follow up by paediatrician and MDT
what can cause HIE?
- maternal shock
- intrapartum haemorrhage
- cord prolapse
- nuchal cord
what are the symptoms/signs of HIE?
mild:
- irritabilty
- hyper-alert
- poor feeding
moderate:
- poor feeding
- hypotonic
- seizures
- lethargic
severe:
- flaccid, reduced/absent reflexes
- reduced consciousness
- apnoeas
what management of GORD in infancy
advise re: positioning, burping after feeds, sleep on back
trial of feed thickeners
alginates, but not alongside thickeners
PPI
what causes of neonatal jaundice in first 24 hours
- rhesus haemolytic disease
- ABO haemolytic disease
- hereditary spherocytosis
- G6PD deficiency
- sepsis
- TORCH
Toxoplasmosis
CMV
Rubella
Syphillis
Hepatitis
Herpes
what causes of neonatal jaundice 24h-14 days
physiological, haemolysis of fetal RBC
breast milk jaundice
breastfeeding jaundice
infection
what causes of neonatal jaundice after 14 days
unconjugated - hypothyroidism, infection - UTI
conjugated - biliary atresia/neonatal hepatitis
what causes kernicterus
high levels of unconjugated bilirubin cross BBB, depositing in the deep grey matter of brain and in basal ganglia causing encephalopathy
what are some possible features of chronic kernicterus
cerebral palsy sensorineural hearing loss gaze palsy learning difficulties death
how would acute kernicterus present
lethargy irritability abnormal muscle tone/posture convulsions apnoeic episodes
what is physiological jaundice
fetal RBC have shorter lifespan than adult FBC (70 days vs 120)
increased RBC breakdown increases circulating bilirubin
hepatic bilirubin metabolism is less efficient in first few days of life
jaundice
what is breast milk jaundice
not fully understood, thought to be due to levels of beta-glucoronidase in breast milk, blocking UGT, decreased metabolism of bilirubin causing jaundice
what is breastfeeding jaundice
poor feeding, presents alongside dehydration
poor feeding causes reduced bilirubin excretion, increases enterohepatic circulation, more bilirubin enters system
what is crigler-najjar
Congenital unconjugated hyperbilirubinaemia
no UGT enzyme activity
no conjugation of bilirubin
causes kernicterus and d
what is gilbert syndrome
autosomal recessive condition causing defective conjugation of bilirubin
unconjugated hyperbilirubinaemia (not in urine)
presents with jaundice in fasting/illness/exercise
no treatment necessary
what is g6pd deficiency
x-linked recessive g6pd deficiency
causing increased oxidative stress and intravascular haemolysis
what triggers in g6pd
fava beans
sulfa drugs, ciprofloxacin
anti-malarials
infections
what is associated with g6pd
splenomegaly
gallstones
what investigations for g6pd
blood film - heinz bodies, bite and blister cells
g6pd enzyme assay
what investigations for neonatal jaundice
FBC and blood film
conjugated bilirubin
blood type testing of mother and baby for ABO/Rh incompatibility
direct coomb’s test (direct antiglobulin for haemolysis)
TFT
blood cultures and urine dip
G6PD enzyme assay
which brain haemorrhage is most common in prem infants and not associated with NAI
intraventricular haemorrhage
what mgmt for neonatal jaundice
dxfjkhs
what is tof
PROV Pulmonary stenosis Right ventricular hypertrophy Overriding aorta Ventricular septal defect
