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Year2 GI Exam II > Neonatal Cholestasis CIS - Brandau/Tieman > Flashcards

Neonatal Cholestasis CIS - Brandau/Tieman Flashcards

1
Q

Case 10 week old F, jaundice, C section, breast fed and yellow from birth, 2 weeks ago switched to formula feeding

  • colicky and fussy, no fever
  • no vomiting/diarrhea
  • stools - light yellow
  • distended abdomen, increased veins on abdomen, yellow sclera, systolic murmur sternal border
  • hepatomegaly
  • total bilirubin 10.1 and direct 6.3
  • elevated ALT/AST
A

neonatal cholestasis - elevation in direct bilirubin

DDx - infection, hepatitis, metabolic, endocrine, genetic, obstructive

NOT physiologic jaundice - not before 36 hours - gone by 10 days of life - and is indirect bilirubin**

NOT breast milk jaundice - prolonged indirect bilirubin**

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2
Q

jaundice at birth

A

needs to be worked up

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3
Q

left sternal border murmur

A

pulmonary valve

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4
Q

unconjugated bilirubin

A

indirect

  • cross BBB
  • carried by albumin
  • kernicterus
  • destruction of heme
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5
Q

jaundice in an otherwise health infant

A

physiologic jaundice

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6
Q

physiologic jaundice

A

gone by 10 days

-indirect jaundice high

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7
Q

breast milk jaundic

A

prolonged indirect bilirubin

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8
Q

clue for toxo infection

A

pet cat

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9
Q

endocrine before 2 months old

A

hypothyroidism - very common picked up on screening

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10
Q

viral neonatal cholestasis

A 
adenovirus
CMV - 2 months sensorineural hearing loss
coxsackie - myocarditis
EBV
enterovirus
hep A, B, C
herpes
HIV
parvo
reovirus
rubella
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11
Q

bacterial neonatal cholestasis

A

UTI
sepsis
listerosis
TB

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12
Q

spirochete neonatal cholestasis

A

syphilis

leptospirosis

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13
Q

parasite neonatal cholestasis

A

toxoplasmosis
malaria
toxocariasis
histoplasmosis

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14
Q

congenital rubella syndrome

A
  • heart disease
  • petechiae/purpura rash
  • microcephaly
  • eye - cataracta, nystagmus, glaucoma
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15
Q

congenital syphilis

A

dracula teeth
nasal chondritis - snuffles

early congenital - before 2yo - hepatosplenomegaly
-nasal chondritis - snuffles

late congenital - after 2yo - craniofacial malformation, dental changes, deafnes

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16
Q

toxoplasmosis

A

most common intrauterine infection - developing countries

posterior uveitis

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17
Q

posterior uveitis

A

toxoplasmosis

destruction of retina

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18
Q

metabolic neonatal stasis

A 
galactosemia
glycogen storage disease
FA oxidation - SCAD
urea cycle defects
tyrosinemia
lysosomal storage diseases
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19
Q

ammonia levels

A

urea cycle defects

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20
Q

galactosemia

A

brain damage, cataracts, jaundice, enlarged liver, kidney damage

unmetabolized milk sugars build up and damage liver, eyes, kidneys, and brain

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21
Q

glycogen storage type IV

A

branching enzyme deficiency
-auto recessive

glycogen with fewer branching points

progressive liver failure early in life

22
Q

tyrosinemia

A

cabbage like odor to skin

liver problems - enlarged, jaundice, bleed and bruise easily, swelling of legs and abdomen

can lead to necessary liver transplant

23
Q

gaucher disease type 2

A

acute and infantile

hepatomegaly and splenomegaly

hydrops fetalis

anemia thrombocytopenia

bulbar palsies, hypertonic

arthrogyrposis - joint contractures

24
Q

genetic neonatal cholestasis

A

CF
trisomy 13, 18, 21
turner
alpha1 antitrypsin

25
Q

edwards syndrome

A

trisomy 18

26
Q

patau syndrome

A

trisomy 13

27
Q

turner syndrome

A

web neck in newborn

wide nipples

spots on skin

28
Q

alpha1 antitrypsin deficieny

A

damage lung and liver

neutrophil elastase problems

29
Q

congenital hypothyroidism

A

jaundice

hypotonic

large tongue

delayed closure fontanelles**

coarse facial features

mental retardation problems if don’t pick up

30
Q

diagnosis congeintal hypothyroid

A

screen - T< 10%, TSH elevation

measure TSH and free T4

  • high TSH and low T4
  • primary**
  • low TSH and low T4
  • secondary**(central)
31
Q

arteriohepatic dysplasia

A

aliagille syndrome

cholestatic liver disease

  • pulmonary valvular stenosis
  • vasculopathy
  • renal disease
32
Q

most common cause of neonatal cholestasis

A

idiopathic neonatal hepatitis 35%

extrahepatic biliary atresia 30%

alpha 1 antitrypsin 10%

33
Q

systemic retention of biliary constituents as result of failure of formation or flow of bile

A

cholestasis

34
Q

intrahepatic

A

failure of formation of bile at hepatocyte level - elevated transaminases and indirect bilirubin

35
Q

extrahepatic

A

obstructive of bile ducts - elevated alk phos, GGTP, and direct bilirubin

36
Q

neonatal obstructive jaundice

A

must be considered jaundice after 14 days old**

37
Q

physiologic jaundice

A

does not extend beyond 2 weeks

38
Q

acholic stool

A

yellow

  • because no bilirubin
  • neonatal obstructive jaundice
39
Q

most common cause neonatal obstructive jaundice

A

biliary atresia**

also choledochal cyst and infections

40
Q

biliary atresia

A

post-natal destruction of extrahepatic bile ducts with resultant injury and fibrosis of intrahepatic bile ducts

more in females

unknown etiology**

41
Q

most common cause of hepatic death and need for liver transplant

A

biliary atresia

42
Q

first in work up for biliary atresia

A

ultrasound**
diagnosis - lab, US, MRC, liver biopsy

liver biopsy - cholestasis in canaliculi and some fibrosis

MRC - magnetic resonance cholangiography

43
Q

Tx for biliary atresia

A

porto-enterostomy - kasal procedure

if done less than 60 days of age

44
Q

intraoperative cholangiogram

A

looking for ductal system

45
Q

kasai procedure

A

porto-enterostomy
-for biliary atresia

needs to be done first 30-45 days (80% success) - beyond only 20%**

if fails - to liver transplant**

46
Q

choledochal cyst

A

can cause obstructive jaundice

5 types - type 5 is carolis disease

majority found <10yo

obstructive jaundice, acholic stools, pruritis, abdomen pain, and or fever

47
Q

tx of choledocal cyst

A

should be resected - risk of malignancy and cholangitis

48
Q

diagnosis of choledochal cyst

A

typical lab pattern of obstructive jaundice, US, MRCP, and ERCP

49
Q

type II choledochal cyst

A

easiest to resect

50
Q

carolis disease

A

type V choledochal cysts

intrahepatic cysts - hard to resect

need liver transplant

Year2 GI Exam II (18 decks)

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