A constitutional unconjugated hyperbilirubinemia combined with indocyanine green intolerance: A new functional disorder?
✍ Scribed by Hideki Ohkubo; Kunio Okuda; Shinji Iida
- Book ID
- 102850355
- Publisher
- John Wiley and Sons
- Year
- 1981
- Tongue
- English
- Weight
- 558 KB
- Volume
- 1
- Category
- Article
- ISSN
- 0270-9139
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✦ Synopsis
The kinetics of plasma clearance of indocyanine green and bromosulfophthalein were studied in 49 consecutive patients with chronic unconjugated hyperbilirubinemia. Forty-four patients had Gilbert's syndrome whereas five patients had impaired hepatic uptake of indocyanine green and virtually normal hepatic bromosulfophthalein uptake. There was no difference in bilirubin metabolism between the two groups. A family study of the patients with impaired indocyanine green uptake revealed that the defect appears to be transmitted as an autosomal dominant trait associated with inheritance of impaired bilirubin metabolism. These patients manifest a novel disorder of organic anion transport which, like Gilbert's syndrome, is also characterized by chronic mild unconjugated hyperbilirubinemia. Gilbert's syndrome is a relatively common disorder characterized by mild chronic unconjugated hyperbilirubinemia. It has long been thought that patients with Gilbert's syndrome have normal hepatic transport of organic anions other than bilirubin. Recently, Berk et al. (1) and Martin et al. ( 2) demonstrated that some patients with otherwise typical Gilbert's syndrome have abnormalities in the hepatic transport of indocyanine green (ICG) and bromosulfophthalein (BSP); patients were classified into three groups, GS I, GS 11, and GS 111, according to the magnitude of impairment in BSP metabolism. These authors emphasized functional heterogeneity in patients with Gilbert's syndrome with respect to defects in hepatic transport of organic anions, and postulated the existence of subgroups with defective hepatic uptake of organic anions other than bilirubin.
During the past 7 years, we encountered five patients with otherwise typical Gilbert's syndrome who had impaired hepatic transport of ICG, which exceeded results in patients with GS 111. We studied the hepatic transport of several organic anions in these patients in comparison with suLgroups GS I to I11 to assess whether these