Ch. 356 - Cholestasis Flashcards
Neonatal cholestasis is defined biochemically as
prolonged elevation of the serum levels of conjugated bilirubin beyond the 1st 14 days of life
T/F infants initially regarded as having neonatal hepatitis, with a patent biliary system shown on cholangiography, can later develop biliary atresia
T
Hepatobiliary disease can be the initial manifestation of ___ and ___
homozygous α1-antitrypsin de ciency or of cystic fibrosis
T/F The hepatitis viruses (A, B, C) rarely cause neonatal cholestasis.
T
The final and critical step in evaluating neonates with cholestasis is to ___
differentiate extrahepatic biliary atresia from neonatal hepatitis
___ is a rare autosomal recessive genetic disorder marked by progressive degeneration of the liver and kidneys
Zellweger (cerebrohepatorenal) syndrome
___ 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)
Test for suspected neonatal cholestasis: Indicates cholestasis
Serum bili fractionation
Test for suspected neonatal cholestasis: Indicates bile flow into intestine
Assessment of stool color
Test for suspected neonatal cholestasis: Confirms cholestasis
Urine and serum bile acids measurement
Test for suspected neonatal cholestasis: Indicates severity of hepatic dysfunction
Albumin, PT/PTT
Test for suspected neonatal cholestasis: Suggests or excludes PiZZ
Alpha 1 antitrypsin phenotype
Test for suspected neonatal cholestasis: Suggests or excludes endoocrinopathy
FT4 TSH
Test for suspected neonatal cholestasis: Distinguishes biliary atresia; suggests alternative diagnosis
Liver biopsy
Test for suspected neonatal cholestasis: Documents bile duct latency or obstruction
Hepatobiliary scintigraphy
Test for suspected neonatal cholestasis: Suggests or excludes choledochal cyst
UTZ
Biliary atresia on UTZ
Triangular cord sign
Cholestasis is defined as
Conjugated bilirubin elevation of any degree
T/F Cholestasis is always pathologic in infants
T
Form of idiopathic familial intrahepatic cholestasis associated with lymphedema of lower extremities
Aagenaes syndrome
MC syndrome with intrahepatic bile duct paucity
Alagille syndrome
Progressive obliterative cholangiopathy
Biliary atresia
MC form of biliary atresia
Obliteration of entire extrahepatic biliary tree at or above the porta hepatis
Triangular cord sign of biliary atresia represents
Cone-shaped fibrotic mass cranial to the bifurcation of the portal vein
Most valuable procedure in the evaluation of neonatal hepatobiliary diseases and provides the most reliable discriminatory evidence
Percutaneous liver biopsy
Neonatal hepatitis vs biliary atresia: Bile ductular proliferation, bile plugs, portal or perilobular edema and fibrosis, basic hepatic lobular architecture intact
Biliary atresia
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
T/F All patients with suspected biliary atresia should undergo EL and direct cholangiography to determine presence/absence of obstruction
T
Success rate for establishing good bile flow after Kasai is much higher if performed before
8 weeks of life
Chronic cholestasis, pruritus, and xanthomas seen in neonatal cholestasis is related to the accumulation of
Cholesterol and bile acids in serum and tissues
Increase bile flow or interrupt enterohepatic circulation of bile acids thus decrease xanthomas and ameliorate pruritus of chronic cholestasis
UDCA
First step in management of patients with ascites
Rule out SBP and restrict Na intake
Diuretic of choice for ascites in neonatal cholestasis
Spironolactone
