Pediatric cholestasis Flashcards

1
Q

cholestasis is characterized by an elevation in what type of bilirubin?

A

direct

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2
Q

what is the initial workup for cholestasis?

A

bilirubin
cholestasis / obstructive pattern
non-cholestatic / hepatocellular pattern

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3
Q

what are the obstructive labs?

A

direct bilirubin
ALP
GGT

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4
Q

jaundice past two weeks indicates ordering what test?

A

direct bilirubin

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5
Q

what is the most common cause of cholestasis in children?

A

biliary atresia

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6
Q

what is the most common cause of death from liver disease in children?

A

biliary atresia

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7
Q

what is the presentation of intrahepatic hyperbilirubinemia / biliary atresia?

A

jaundice resolves during first week of life, then reappears around 1 month

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8
Q

what are the lab values for biliary atresia?

A

elevated direct bilirubin

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9
Q

what is the treatment for biliary atresia?

A

kasai procedure

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10
Q

what is the goal of the kasai procedure?

A

intestine used as biliary drainage from smaller bile ducts

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11
Q

definition: cholelithiasis

A

stones in gallbladder

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12
Q

definition: cholecystitis

A

inflammed gallbladder

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13
Q

definition: choledocolithiasis

A

stone in common bile duct

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14
Q

what is the most common cause of intrahepatic cholestasis?

A

alagille syndrome

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15
Q

what are the features of alagille syndrome?

A

paucity of interlobular bile ducts
formed normally
progressively lost with age

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16
Q

what are the associated findings of alagille syndrome?

A

facial findings
peripheral pulmonic stenosis
butterfly vertebrae

17
Q

medication induced cholestasis is due to what type of medication?

A

total parenteral nutrition (TPN)

18
Q

what are the important metabolic causes of cholestasis?

A

congenital hypothyroidism
congenital panhypopituitarism
UTI and sepsis
galactosemia, autosomal recessive

19
Q

when is the onset of galactosemia? what is the treatment?

A

first week

stopping lactose

20
Q

what is important about medium chain fatty acids?

A

do not require the biliary system to be absorbed - they go straight to the gut

21
Q

what is the best type of nutrition for maintaining and gaining weight?

A

medium chain fatty acids