NEONATAL CHOLESTASIS

Posted by e-Medical PPT
Learning Objectives
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 etiologies
Intrahepatic etiologies

EXTRAHEPATIC ETIOLOGIES
Extrahepatic biliary atresia
Choledochal cyst
Bile duct stenosis
Spontaneous perforation of the bile duct
Cholelithiasis
Inspissated bile/mucus plug
Extrinsic compression of the bile duct

CLINICAL PRESENTATION
Jaundice,Scleral icterus,Hepatomegaly,Acholic stools & Dark urine

EVALUATION

Basic evaluation
 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
Ophthalmologic examination

Metabolic work-up
Protein electrophoresis, alpha-1-antitrypsin level and phenotype
Thyroid function tests
Sweat chloride
Urine/serum amino acids
Review results of newborn metabolic screen
Urine reducing substances
Urine bile acids

Radiological evaluation
Ultrasonography
Patient should be NPO to increase likelihood of visualizing the gallbladder
Feeding with exam may demonstrate a functioning gallbladder
Hepatobiliary scintigraphy

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