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Liver bilirubin UDP-glucuronosyltransferase activity in chronic nonhemolytic unconjugated hyperbilirubinemia of adults. 1998

A Watanabe, and H Wakabayashi, and Y Kuwabara, and H Yamamoto, and S Hattori, and T Tsuji
Third Department of Internal Medicine, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Japan.

Ten adult patients with chronic nonhemolytic unconjugated (indirect) hyperbilirubinemia were analyzed by determining bilirubin uridine diphosphate-glucuronosyltransferase activity according to a more physiological and sensitive method (9 control cases, 0.457 +/- 0.163 nmole/mg protein/min). There was no overlap of the enzyme activities of 2 cases with Crigler-Najjar syndrome (type II) (0.006 nmole/mg protein/min on average) and 6 cases with Gilbert's syndrome (0.051 +/- 0.016 nmole/mg protein/min). The enzyme activities in 2 patients with post-hepatitic hyperbilirubinemia were within the normal range. A new classification of nonhemolytic unconjugated hyperbilirubinemia in adults is proposed according to the results of this enzyme activity and the recent data on the gene mutation of this enzyme.

UI MeSH Term Description Entries
D007565 Jaundice A clinical manifestation of HYPERBILIRUBINEMIA, characterized by the yellowish staining of the SKIN; MUCOUS MEMBRANE; and SCLERA. Clinical jaundice usually is a sign of LIVER dysfunction. Icterus,Jaundice, Hemolytic,Hemolytic Jaundice,Hemolytic Jaundices,Jaundices, Hemolytic
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009154 Mutation Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations. Mutations
D002908 Chronic Disease Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2). Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D003414 Crigler-Najjar Syndrome A familial form of congenital hyperbilirubinemia transmitted as an autosomal recessive trait. It is characterized by icterus and brain damage caused by a glucuronyl transferase deficiency in the liver and faulty bilirubin conjugation. Crigler Najjar Syndrome,Crigler Najjar Syndrome, Type 1,Crigler-Najar Syndrome,Crigler-Najjar Syndrome, Type I,Familial Nonhemolytic Unconjugated Hyperbilirubinemia,Hereditary Unconjugated Hyperbilirubinemia,Crigler Najar Syndrome,Crigler Najjar Syndrome, Type I,Crigler Najjar Syndromes,Hereditary Unconjugated Hyperbilirubinemias,Hyperbilirubinemia, Hereditary Unconjugated,Najjar Syndrome, Crigler,Syndrome, Crigler Najjar,Unconjugated Hyperbilirubinemia, Hereditary
D005260 Female Females
D005878 Gilbert Disease A benign familial disorder, transmitted as an autosomal dominant trait. It is characterized by low-grade chronic hyperbilirubinemia with considerable daily fluctuations of the bilirubin level. Constitutional Liver Dysfunction,Familial Nonhemolytic Jaundice,Gilbert Syndrome,Gilbert's Disease,Gilbert's Syndrome,Gilbert-Lereboullet Syndrome,Hyperbilirubinemia 1,Hyperbilirubinemia I,Hyperbilirubinemia, Arias Type,Meulengracht Syndrome,Unconjugated Benign Bilirubinemia,Arias Type Hyperbilirubinemia,Arias Type Hyperbilirubinemias,Disease, Gilbert,Disease, Gilbert's,Gilberts Disease,Gilberts Syndrome,Hyperbilirubinemia 1s,Hyperbilirubinemias, Arias Type,Syndrome, Gilbert,Syndrome, Gilbert's
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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