1. Vital signs. Fever may indicate infectious cholangitis or viral hepatitis
2. Growth parameters. Poor weight gain and linear growth stunting can occur with chronic liver disease, endocrine abnormalities, and malabsorptive conditions. Weight may be falsely elevated due to ascites or edema, and organomegaly. Consider anthropometric measurements in that setting.
3. Physical findings. Jaundice, scleral icterus, hepatosplenomegaly, ascites, peripheral edema, caput medusa, xanthomas, palmar erythema, telangiectasia, male gynecomastia, peripheral wasting, hemorrhoids or occult blood in stool, clubbing, changes on neurologic exam (includes mental status, pupillary size, aster-ixis, hyperreflexia or hyporeflexia, clonus, Babinski sign).
B. Laboratory Data. Evaluation is guided by clinical presentation.
1. Blood. CBC with platelets and reticulocyte count, electrolytes, glucose, BUN, creatinine, liver enzymes (ALT, AST, alkaline phosphatase, GGTP, 5'-nucleotidase), direct and total bilirubin, PT and PTT, albumin, ammonia, cholesterol, TORCH titers, EBV, CMV, hepatitis B and C, ^-antitrypsin level and pheno-type, ceruloplasmin, serum ferritin and iron, RBC galactose-1-phosphate uridyl transferase, and thyroid function tests. Consider blood culture, serum amino acids, and serum bile acids.
2. Urine. Urinalysis, including reducing substances. Consider urine culture, urine amino acids, urine organic acids, urine bile acids. Suspect galactosemia if urinalysis has reducing substance without glucosuria.
3. Other workup. Sweat chloride analysis to evaluate for CF.
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