Unconjugated Hyperbilirubinemia Questions & Answers

Updated: May 21, 2019
  • Author: Hisham Nazer, MBBCh, FRCP, DTM&H; Chief Editor: BS Anand, MD  more...
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Questions & Answers

Overview

What causes unconjugated hyperbilirubinemia?

What are the signs and symptoms of unconjugated hyperbilirubinemia?

How is Crigler-Najjar syndrome type 1 diagnosed?

How is Crigler-Najjar syndrome type 2 diagnosed?

How is Gilbert syndrome diagnosed?

How are total serum bilirubin levels used to diagnose physiologic neonatal jaundice?

How are total serum bilirubin levels used to diagnose breast milk jaundice?

How is ineffective erythropoiesis characterized?

What are the treatment options for Crigler-Najjar syndrome type 1?

What are the treatment options for Crigler-Najjar syndrome type 2?

Which medications are used in the treatment of Gilbert syndrome?

When is phototherapy indicated for the treatment of unconjugated hyperbilirubinemia?

What is unconjugated hyperbilirubinemia?

What is Gilbert syndrome?

What is the biochemistry of bilirubin relative to unconjugated hyperbilirubinemia?

What leads to an increased production of bilirubin in unconjugated hyperbilirubinemia?

What causes impaired hepatic bilirubin uptake in unconjugated hyperbilirubinemia?

How is physiologic jaundice characterized in newborns?

What are the types of nonphysiologic jaundice in unconjugated hyperbilirubinemia?

What causes ABO/Rh incompatibility leading to neonatal jaundice?

What are the inherited defects of bilirubin conjugation in unconjugated hyperbilirubinemia?

What is Crigler-Najjar syndrome?

What are the forms of Crigler-Najjar syndrome?

What is the pathophysiology of unconjugated hyperbilirubinemia?

What is the pathophysiology of ineffective erythropoiesis in unconjugated hyperbilirubinemia?

What is pathophysiology of neonatal jaundice in unconjugated hyperbilirubinemia?

Which factors contribute to the development of physiologic jaundice in unconjugated hyperbilirubinemia?

What is the pathophysiology of nonphysiologic jaundice in unconjugated hyperbilirubinemia?

What is the pathophysiology of impaired conjugation of bilirubin in unconjugated hyperbilirubinemia?

What is the pathophysiology of Gilbert syndrome?

What is the role of UGT mutations in the pathophysiology of Gilbert syndrome?

What causes increased bilirubin production in unconjugated hyperbilirubinemia?

What causes impaired hepatic bilirubin uptake in unconjugated hyperbilirubinemia?

What causes impaired bilirubin conjugation in unconjugated hyperbilirubinemia?

What is the incidence of unconjugated hyperbilirubinemia in the US?

What is the global incidence of unconjugated hyperbilirubinemia?

What are the racial predilections of unconjugated hyperbilirubinemia?

How does the incidence of unconjugated hyperbilirubinemia vary by sex?

At what age do the symptoms of unconjugated hyperbilirubinemia first appear?

What is the prognosis of breast milk jaundice in unconjugated hyperbilirubinemia?

What is the prognosis of maternal serum jaundice in unconjugated hyperbilirubinemia?

What is the prognosis of Crigler-Najjar syndrome type 1?

What is the prognosis of Crigler-Najjar syndrome type 2?

What is the prognosis of Gilbert syndrome?

What is the prognosis of ineffective erythropoiesis (ELB production)?

What should be included in patient education about unconjugated hyperbilirubinemia?

Presentation

What is the clinical history of ineffective erythropoiesis (ELB production)?

What is the clinical history of Crigler-Najjar (CN) syndrome type 1?

What is the clinical history of Crigler-Najjar (CN) syndrome type 2?

What is the clinical history of Gilbert syndrome?

What is the clinical history of physiologic and nonphysiologic neonatal jaundice in unconjugated hyperbilirubinemia?

Which physical findings suggest Crigler-Najjar syndrome?

Which physical exam findings are characteristic of Gilbert syndrome?

DDX

What guidelines have been published for the diagnosis and treatment of unconjugated hyperbilirubinemia?

Which diseases are associated with ineffective erythropoiesis?

What other causes of jaundice should be included in the differential diagnoses for infants with unconjugated hyperbilirubinemia?

What are diagnostic considerations in Gilbert syndrome?

Which conditions should be considered in the differential diagnoses of unconjugated hyperbilirubinemia?

What are the differential diagnoses for Unconjugated Hyperbilirubinemia?

Workup

What is the significance of elevated total serum bilirubin level in the evaluation of unconjugated hyperbilirubinemia?

How is Crigler-Najjar syndrome diagnosed?

Which lab results suggest Crigler-Najjar syndrome type 1 syndrome?

Which lab results suggest Crigler-Najjar syndrome type 2 syndrome?

Which liver function testing results are characteristic of Crigler-Najjar syndrome?

What is the role of percutaneous liver biopsy in the diagnosis of Crigler-Najjar syndrome?

How is Gilbert syndrome diagnosed?

Which findings are diagnostic of Gilbert syndrome?

What is the role of a fasting test in the workup of Gilbert syndrome?

What is the role of a nicotinic acid test in the workup of Gilbert syndrome?

What is the role of a phenobarbital test in the workup of Gilbert syndrome?

What is the role of a radiolabeled chromium test in the workup of Gilbert syndrome?

What is the role of thin-layer chromatography in the workup of Gilbert syndrome?

What is the role of a drug-clearance test in the workup of Gilbert syndrome?

What is the role of polymerase chain reaction (PCR) assay in the workup of Gilbert syndrome?

What is the role of imaging studies in the workup of Gilbert syndrome?

What is the role of percutaneous liver biopsy in the workup of Gilbert syndrome?

What peak total serum bilirubin level is diagnostic of physiologic jaundice in unconjugated hyperbilirubinemia?

At what bilirubin level is phototherapy indicated for breast milk jaundice in unconjugated hyperbilirubinemia?

Which lab results are characteristic of ineffective erythropoiesis (ELB production) in unconjugated hyperbilirubinemia?

Treatment

What are treatment options for Gilbert syndrome?

What are the treatment options for Crigler-Najjar syndrome?

What is the role of phototherapy in the treatment of severe unconjugated neonatal hyperbilirubinemia?

How is unconjugated hyperbilirubinemia prevented?

What is the therapeutic response to nonsurgical correction of bilirubin levels in Crigler-Najjar syndrome type 1?

What is the efficacy of phototherapy for the treatment of Crigler-Najjar syndrome type 1?

What is the role of plasma exchange transfusion in the treatment of Crigler-Najjar syndrome type 1?

What is the role of gene therapy in the treatment of Crigler-Najjar syndrome type 1?

Why is long-term monitoring required in the management of Crigler-Najjar syndrome type 1?

What are the nonsurgical options for Crigler-Najjar syndrome type 2?

What are the nonsurgical options for Gilbert syndrome?

How is phototherapy administered in the treatment of nonphysiologic jaundice in unconjugated hyperbilirubinemia?

When is liver transplantation indicated in the treatment of unconjugated hyperbilirubinemia?

What is the efficacy of hepatocyte transplantation for the treatment of unconjugated hyperbilirubinemia?

Medications

Which medications are indicated in the treatment of unconjugated hyperbilirubinemia?

Which medications in the drug class Antipsychotics, Phenothiazine are used in the treatment of Unconjugated Hyperbilirubinemia?

Which medications in the drug class Gallstone-Solubilizing Agents are used in the treatment of Unconjugated Hyperbilirubinemia?

Which medications in the drug class Calcium Salts are used in the treatment of Unconjugated Hyperbilirubinemia?

Which medications in the drug class Lipid-Lowering Agents are used in the treatment of Unconjugated Hyperbilirubinemia?

Which medications in the drug class Anticonvulsants are used in the treatment of Unconjugated Hyperbilirubinemia?