Paeds Flashcards
name 5 conditions tested for by the guthrie test?
sickle cell hypothyroidism CF maple syrup urine disease phenylketonuria
what does the APGAR score test for?
Appearance- colour Pulse Grimace Activity - muscle tone Respiratory effort
7-10 good
0-3 low
what are some complications of premature birth?
NEC RDS- surfactant deficiency retinopathy of prematurity PDA jaudince- liver immaturity intraventricular haemorrhage
what are the ways to categorise neonatal jaundice?
present at birth/appears in first 24 hours- pathological
appears after 24 hours and resolves by 14 days - physiological
continues after 14 days- prolonged
causes of pathological jaundice?
rhesus d incompatibility/ABO
G6PD-deficiency
Infection - congenital (CMV, syphilis, rubella, toxoplasmosis, herpes)
Spherocytosis
what is the pathophysiology of rhesus/ABO incompatibility?
mother is rhesus -ve, baby is rhesus +ve (due to rhesus +ve father), sensitisation event occurs where mother is exposed to foetal blood -> causes body to produces auto-antibodies against rhesus +ve RBC’s, which then cross the placenta and destroy the foetal RBC’s leading to physiological jaundice. The same occurs if a mothers blood type is O and the foetus is either A or B.
investigations of suspected rhesus incompatibility?
rhesus negative mothers and O blood group - indirect coombs test
foetal blood sampling/doppler USS- can show foetal anaemia + increased reticulocyte
direct coomb test after birth to check
management of incompatbility ABO or rhesus?
transfusion with group O negative blood
anti-D immunoglobulin
what is the danger of prolonged jaundice in neonates?
can develop kernicterus - bilirubin crosses blood/brain barrier which can lead to cerebral palsy (non-progressive permanent cerebral insult)
mode of inheritance of G6PD deficiency?
X-linked recessive
pathophysiology of G6PD deficiency?
reduced G6PD leads to reduction in glutathione which causes increased susceptibility of RBC to oxidative stress
management of G6PD deficiency in neonates?
exchange transfusion
phototherapy
causes of physiological jaundice in neonate?
immature hepatic bilirubin conjugation
infection: sepsis/UTI
haemolytic disorders
breast milk jaundice
management of neonatal jaundice?
blue light phototherapy
exchange transfusion if very high
investigations of neonatal jaundice?
serum bilirubin to monitor
causes of prolonged neonatal jaundice?
unconjugated:
-infection
-breast milk
-haemolytic anaemia
-hypothyroidism
conjugated:
-bile duct obstruction - biliary atresia
neonatal hepatitis
what is biliary atresia?
complete or partial obstruction of the extrahepatic biliary tree within the first 3 months of life
signs and symptoms of biliary atresia?
neonatal prolonged jaundice - often 8 weeks after birth (can have an episode of transient jaundice at birth which then resolves, followed by further jaundice 8 weeks later) pale stools dark urine failure to thrive hepatomegaly + splenomegaly
investigations of biliary atresia?
serum bilirubin - normal conjugated bilirubin- abnormally high serum alpha-1-antitrypsin CF sweat test USS of biliary tree percutaneous liver biopsy with intraoperative cholangioscopy
management of biliary atresia?
surgical intervention
pathophysiology of neonatal respiratory distress syndrome?
widespread alveolar collapse due to surfactant deficiency (immature lungs in neonate- surfactant starts to be produced at 24 weeks). This leads to inadequate gas exchange.
RF for RDS?
premature birth
C-section delivery
maternal diabetes
past hx
presentation of RDS?
nasal flaring grunting tachypnoea tracheal tug intercostal recession
investigations and findings in RDS?
CXR: bilateral diffuse ground glass appearance, reduced lung volume