Ch. 356 - Cholestasis

Question 1

Neonatal cholestasis is defined biochemically as

Answer 1

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

Question 2

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

Answer 2

T

Question 3

Hepatobiliary disease can be the initial manifestation of ___ and ___

Answer 3

homozygous α1-antitrypsin de ciency or of cystic fibrosis

Question 4

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

Answer 4

T

Question 5

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

Answer 5

differentiate extrahepatic biliary atresia from neonatal hepatitis

Question 6

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

Answer 6

Zellweger (cerebrohepatorenal) syndrome

Question 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

Answer 7

Neonatal iron storage disease (neonatal hemochromatosis)

Question 8

Test for suspected neonatal cholestasis: Indicates cholestasis

Answer 8

Serum bili fractionation

Question 9

Test for suspected neonatal cholestasis: Indicates bile flow into intestine

Answer 9

Assessment of stool color

Question 10

Test for suspected neonatal cholestasis: Confirms cholestasis

Answer 10

Urine and serum bile acids measurement

Question 11

Test for suspected neonatal cholestasis: Indicates severity of hepatic dysfunction

Answer 11

Albumin, PT/PTT

Question 12

Test for suspected neonatal cholestasis: Suggests or excludes PiZZ

Answer 12

Alpha 1 antitrypsin phenotype

Question 13

Test for suspected neonatal cholestasis: Suggests or excludes endoocrinopathy

Answer 13

FT4 TSH

Question 14

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

Answer 14

Liver biopsy

Question 15

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

Answer 15

Hepatobiliary scintigraphy

Question 16

Test for suspected neonatal cholestasis: Suggests or excludes choledochal cyst

Answer 16

UTZ

Question 17

Biliary atresia on UTZ

Answer 17

Triangular cord sign

Question 18

Cholestasis is defined as

Answer 18

Conjugated bilirubin elevation of any degree

Question 19

T/F Cholestasis is always pathologic in infants

Answer 19

T

Question 20

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

Answer 20

Aagenaes syndrome

Question 21

MC syndrome with intrahepatic bile duct paucity

Answer 21

Alagille syndrome

Question 22

Progressive obliterative cholangiopathy

Answer 22

Biliary atresia

Question 23

MC form of biliary atresia

Answer 23

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

Question 24

Triangular cord sign of biliary atresia represents

Answer 24

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

Question 25

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

Answer 25

Percutaneous liver biopsy

Question 26

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

Answer 26

Biliary atresia

Question 27

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

Answer 27

Neonatal hepatitis

Question 28

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

Answer 28

T

Question 29

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

Answer 29

8 weeks of life

Question 30

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

Answer 30

Cholesterol and bile acids in serum and tissues

Question 31

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

Answer 31

UDCA

Question 32

First step in management of patients with ascites

Answer 32

Rule out SBP and restrict Na intake

Question 33

Diuretic of choice for ascites in neonatal cholestasis

Answer 33

Spironolactone