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- 저자 : ( Munkhbaatar Tsendsuren ) (검색결과 1 건)
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Severe Indirect Hyperbilirubinemia Patient with NT-211G>A Variant of UGT1A1 Gene
( Baterdene Dashnyam ),( Naran Uyanga ),( Munkhbaatar Tsendsuren ),( Enkhtuya Derem ),( Gantuya Balgan ),( Nyam Biziya ) 대한간학회 2016 춘·추계 학술대회 (KASL) Vol.2016 No.1
Aims: Hereditary abnormalities of uridine diphosphate glucuronosyltransferase 1A1 (UGT1A1) gene is associated with unconjugated hyper- bilirubinemia worldwide. Methods: Here we report one case of rapid onset of severe hemolysis and severe indirect hyperbilirubinemia in patient with nt-211 G>A mutation in UGT1A1 gene during the initial phase of combined interferon and ribavirin therapy for chronic viral hepatitis C. A 23-year-old Mongolian male developed chronic viral hepatitis C three years ago. His baseline laboratory data were hemoglobin 16.1 g/dl, MCV 86.8 fL, prothrombin time INR 1.0, AST 27 IU/L, ALT 27 IU/L, total bilirubin 1.4 mg/dL, direct bilirubin 0.4 mg/dL. His HCV RNA was genotype 1b and viral load was 3,063,289 IU/mL. The analysis of UGT1A1 gene revealed a homogeneous nt-211 G to A mutation. In the second week of the combined Peginterferon alfa-2a 100mcg plus Ribavirin 1200mg therapy, the patient developed increased jaundice with total bilirubin 4.9 mg/dL, direct bilirubin 1.9 mg/dL, hemoglobin 14.0 g/dL. Reducing the dosage of Ribavirin to 1000 mg daily, the total bilirubin went down to 1.9 mg/dL, direct bilirubin 0.9 mg/dL, hemoglobin 11.0 g/dL and HCV RNA became undetectable in the fourth week. The dosage of Ribavirin was further reduced to 800 mg daily. The total bilirubin level remained steady at 1.9 mg/dL, direct bilirubin 0.6 mg/dL and hemoglobin 10.4 g/dL in the eighth week. In the 22nd week, the patient is discontinued from the combination therapy due to severe depression, and the total bilirubin was 1.2 mg/dL, direct bilirubin 0.4 mg/dL and hemoglobin 11.4 g/dL. The patient achieved a sustained virological response despite the early termination of the combination therapy. Results: The hemolysis was improved by reducing dosage of ribavirin. The patient had sustained virological response despised the severe indirect hyperbilirubinemia. Conclusions: UGT1A1 abnormalities should be assessed when hyperbilirubinemia is observed during the initial phase of combined interferon and ribavirin therapy.
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