Pediatric cholestasis Flashcards
cholestasis is characterized by an elevation in what type of bilirubin?
direct
what is the initial workup for cholestasis?
bilirubin
cholestasis / obstructive pattern
non-cholestatic / hepatocellular pattern
what are the obstructive labs?
direct bilirubin
ALP
GGT
jaundice past two weeks indicates ordering what test?
direct bilirubin
what is the most common cause of cholestasis in children?
biliary atresia
what is the most common cause of death from liver disease in children?
biliary atresia
what is the presentation of intrahepatic hyperbilirubinemia / biliary atresia?
jaundice resolves during first week of life, then reappears around 1 month
what are the lab values for biliary atresia?
elevated direct bilirubin
what is the treatment for biliary atresia?
kasai procedure
what is the goal of the kasai procedure?
intestine used as biliary drainage from smaller bile ducts
definition: cholelithiasis
stones in gallbladder
definition: cholecystitis
inflammed gallbladder
definition: choledocolithiasis
stone in common bile duct
what is the most common cause of intrahepatic cholestasis?
alagille syndrome
what are the features of alagille syndrome?
paucity of interlobular bile ducts
formed normally
progressively lost with age
what are the associated findings of alagille syndrome?
facial findings
peripheral pulmonic stenosis
butterfly vertebrae
medication induced cholestasis is due to what type of medication?
total parenteral nutrition (TPN)
what are the important metabolic causes of cholestasis?
congenital hypothyroidism
congenital panhypopituitarism
UTI and sepsis
galactosemia, autosomal recessive
when is the onset of galactosemia? what is the treatment?
first week
stopping lactose
what is important about medium chain fatty acids?
do not require the biliary system to be absorbed - they go straight to the gut
what is the best type of nutrition for maintaining and gaining weight?
medium chain fatty acids
