Ch. 356 - Cholestasis Flashcards Preview

Nelson - The Digestive System > Ch. 356 - Cholestasis > Flashcards

Flashcards in Ch. 356 - Cholestasis Deck (33):
1

Neonatal cholestasis is defined biochemically as

prolonged elevation of the serum levels of conjugated bilirubin beyond the 1st 14 days of life

2

T/F infants initially regarded as having neonatal hepatitis, with a patent biliary system shown on cholangiography, can later develop biliary atresia

T

3

Hepatobiliary disease can be the initial manifestation of ___ and ___

homozygous α1-antitrypsin de ciency or of cystic fibrosis

4

T/F The hepatitis viruses (A, B, C) rarely cause neonatal cholestasis.

T

5

The final and critical step in evaluating neonates with cholestasis is to ___

differentiate extrahepatic biliary atresia from neonatal hepatitis

6

___ is a rare autosomal recessive genetic disorder marked by progressive degeneration of the liver and kidneys

Zellweger (cerebrohepatorenal) syndrome

7

___ is a rapidly progressive disease characterized by increased iron deposition in the liver, heart, and endocrine organs without increased iron stores in the reticuloendothelial system

Neonatal iron storage disease (neonatal hemochromatosis)

8

Test for suspected neonatal cholestasis: Indicates cholestasis

Serum bili fractionation

9

Test for suspected neonatal cholestasis: Indicates bile flow into intestine

Assessment of stool color

10

Test for suspected neonatal cholestasis: Confirms cholestasis

Urine and serum bile acids measurement

11

Test for suspected neonatal cholestasis: Indicates severity of hepatic dysfunction

Albumin, PT/PTT

12

Test for suspected neonatal cholestasis: Suggests or excludes PiZZ

Alpha 1 antitrypsin phenotype

13

Test for suspected neonatal cholestasis: Suggests or excludes endoocrinopathy

FT4 TSH

14

Test for suspected neonatal cholestasis: Distinguishes biliary atresia; suggests alternative diagnosis

Liver biopsy

15

Test for suspected neonatal cholestasis: Documents bile duct latency or obstruction

Hepatobiliary scintigraphy

16

Test for suspected neonatal cholestasis: Suggests or excludes choledochal cyst

UTZ

17

Biliary atresia on UTZ

Triangular cord sign

18

Cholestasis is defined as

Conjugated bilirubin elevation of any degree

19

T/F Cholestasis is always pathologic in infants

T

20

Form of idiopathic familial intrahepatic cholestasis associated with lymphedema of lower extremities

Aagenaes syndrome

21

MC syndrome with intrahepatic bile duct paucity

Alagille syndrome

22

Progressive obliterative cholangiopathy

Biliary atresia

23

MC form of biliary atresia

Obliteration of entire extrahepatic biliary tree at or above the porta hepatis

24

Triangular cord sign of biliary atresia represents

Cone-shaped fibrotic mass cranial to the bifurcation of the portal vein

25

Most valuable procedure in the evaluation of neonatal hepatobiliary diseases and provides the most reliable discriminatory evidence

Percutaneous liver biopsy

26

Neonatal hepatitis vs biliary atresia: Bile ductular proliferation, bile plugs, portal or perilobular edema and fibrosis, basic hepatic lobular architecture intact

Biliary atresia

27

Neonatal hepatitis vs biliary atresia: Severe diffuse hepatocellular disease with distortion of lobular architecture, marked infiltration with inflammatory cells, and focal hepatocellular necrosis

Neonatal hepatitis

28

T/F All patients with suspected biliary atresia should undergo EL and direct cholangiography to determine presence/absence of obstruction

T

29

Success rate for establishing good bile flow after Kasai is much higher if performed before

8 weeks of life

30

Chronic cholestasis, pruritus, and xanthomas seen in neonatal cholestasis is related to the accumulation of

Cholesterol and bile acids in serum and tissues

31

Increase bile flow or interrupt enterohepatic circulation of bile acids thus decrease xanthomas and ameliorate pruritus of chronic cholestasis

UDCA

32

First step in management of patients with ascites

Rule out SBP and restrict Na intake

33

Diuretic of choice for ascites in neonatal cholestasis

Spironolactone