Management of Hyperbilirubinemia in Adolescents

Initial Diagnostic Workup

The American Academy of Pediatrics recommends fractionated bilirubin testing to distinguish conjugated from unconjugated hyperbilirubinemia, as this distinction drives all subsequent diagnostic and therapeutic decisions 1, 2
Complete blood count with peripheral smear is essential to evaluate for hemolysis, according to the American Academy of Pediatrics 3, 1
Reticulocyte count, haptoglobin, and LDH are necessary to assess hemolytic processes, as recommended by the American Academy of Pediatrics 1
Liver function tests, including ALT, AST, alkaline phosphatase, GGT, and albumin, help differentiate hepatocellular injury from cholestatic patterns, as suggested by the American Association for the Study of Liver Diseases 1
Prothrombin time/INR is crucial to evaluate hepatic synthetic function, according to the American Association for the Study of Liver Diseases 1

G6PD deficiency testing is essential, as G6PD deficiency accounts for 31.5% of kernicterus cases and can present with acute hemolytic crises triggered by oxidative stress, according to the American Academy of Pediatrics 3, 1
The American Academy of Pediatrics recommends evaluating for hereditary spherocytosis, sickle cell disease, or other hemoglobinopathies through blood smear morphology and hemoglobin electrophoresis, although standard hemolysis markers have poor sensitivity and specificity 1
Crigler-Najjar syndrome type II presents with moderate unconjugated hyperbilirubinemia (6-20 mg/dL) that responds to phenobarbital administration, as reported by the American Association for the Study of Liver Diseases 4
Crigler-Najjar syndrome type I causes severe unconjugated hyperbilirubinemia (>20 mg/dL) unresponsive to phenobarbital and requires urgent liver transplantation evaluation to prevent kernicterus, according to the American Association for the Study of Liver Diseases 4

Abdominal ultrasonography is the initial imaging modality to differentiate extrahepatic biliary obstruction from intrahepatic parenchymal disease, as recommended by the American College of Radiology 1
Autoimmune hepatitis requires immunosuppression with prednisone with or without azathioprine, with liver transplantation needed in 10-20% of pediatric cases, according to the American Association for the Study of Liver Diseases 4
Primary sclerosing cholangitis can be diagnosed with MRCP if ultrasonography shows ductal abnormalities, as reported by the American Association for the Study of Liver Diseases 4

The American Academy of Pediatrics advises against relying on visual assessment alone to gauge bilirubin severity, especially in patients with darker skin pigmentation 3
The American Association for the Study of Liver Diseases recommends not attributing low albumin solely to liver disease, as sepsis, inflammation, and malnutrition also reduce albumin levels 1
Correct prolonged INR with vitamin K supplementation before attributing coagulopathy exclusively to hepatic dysfunction, as suggested by the American Association for the Study of Liver Diseases 1
G6PD levels can be falsely elevated during acute hemolysis, so repeat testing at 3 months if deficiency is suspected but initial testing is normal, according to the American Academy of Pediatrics 2