Know the differential diagnosis for neonatal cholestasis.
Understand how to evaluate the neonate with conjugated hyperbilirubinemia.
Know the therapeutic management of neonates with cholestasis.
Neonatal cholestasis is defined as conjugated hyperbilirubinemia developing within the first 90 days of extrauterine life.
Conjugated bilirubin exceeds 1.5 to 2.0 mg/dl.Conjugated bilirubin generally exceeds 20% of the total bilirubin.
Basic distinction is between:
Extrahepatic biliary atresia
Bile duct stenosis
Spontaneous perforation of the bile duct
Inspissated bile/mucus plug
Extrinsic compression of the bile duct
Jaundice,Scleral icterus,Hepatomegaly,Acholic stools & Dark urine
History and physical examination (includes exam of stool color)
CBC and reticulocyte count
Electrolytes, BUN, creatinine, calcium, phosphate
SGOT, SGPT, GGT, alkaline phosphatase
Total and direct bilirubin
Total protein, albumin, cholesterol, PT/PTT
Tests for infectious causes
Indicated cultures of blood, urine, CSF
TORCH titers, RPR/VDRL
Urine for CMV
Hepatitis B and C serology
Protein electrophoresis, alpha-1-antitrypsin level and phenotype
Thyroid function tests
Urine/serum amino acids
Review results of newborn metabolic screen
Urine reducing substances
Urine bile acids
Patient should be NPO to increase likelihood of visualizing the gallbladder
Feeding with exam may demonstrate a functioning gallbladder