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At least 19 recordsLinked to original sources
PubMed1977

The clinical presentation of Gilbert's disease in 26 patients.

Twenty-six patients with Gilbert's disease (congenital, non-haemolytic unconjugated hyperbilirubinaemia) were analysed regarding their clinical presentation, age at onset of symptoms, sex, frequency of symptoms, family history, race and religion. Seventy-three per cent were men, the mean age at onset of symptoms was 21 years, and frequency of symptoms ranged from 4 times a year to once every 5 years. The symptoms, which were extremely vague, included the following: recurrent asymptomatic jaundice in 74%, malaise in 66%, asthenia in 65%, and vague abdominal distension in 52% of patients. Eight per cent of patients were totally asymptomatic. There did not appear to be any particular race or religious group with a higher incidence of the disorder. No abnormal clinical features apart from mild jaundice were detected. The entirely benign nature of the syndrome is stressed, and a normal life expectancy is the rule. The avoidance of prolonged fasting is the best therapeutic measure, although enzyme induction by phenobarbitone therapy may have some place in the management of symptoms.

Adolescent↗
PubMed1977

Study of the etiology and pathogenesis of low grade nonhemolytic unconjugated hyperbilirubinemia (Gilbert's disease).

172 adolescent and adult patients with low grade nonhemolytic unconjugated hyperbilirubinaemia (n.u.h) were examined. Authors have come to the conclusion of the absence of an acquired (posthepatic) form of n.u.h., i.e of the existence of a single --constitutional--form (Gilbert's disease). Viral hepatitis is not likely to play the role of an etiological factor of n.u.h. but of a factor which manifests a congenital defect of bilirubin metabolism. The study of the glucuronidisation found out that its decrease is an important factor in the pathogenesis of the low grade n.u.h. that therefore cannot opposed to the n.u.h. with glucuronyl transferase deficiency group II according to Arias et al. In 61% of patients a moderately shortened erythrocyte life span has been revealed. However the increased bile pigment production in these cases does not speak against Gilbert's disease. The results of biochemical assays have shown that in n.u.h not only the intrahepatic bilirubin metabolism is disturbed but other functions of the liver cell as well. The morphological study of hepatocytes has revealed certain signs of their dystrophy. Based on their investigation the authors propose to single out a group of hereditary pigment hepatoses which include besides Gilbert's disease the syndromes of Crigler-Najjar, Dubin-Johnson and Rotor.

Adolescent↗
PubMed1977

Does Gilbert's disease exist?

18 454 men and 5471 women attending a screening centre had serum-bilirubin concentrations measured. 2.0% of men and 0.6% of women had concentrations of 25 micronmol/1 (1-5 MG/DL) or above. Both sexes showed skewed distributions for bilirubin concentration, but analysis of the data showed no evidence of bimodality for either sex. This suggests that people who are now diagnosed as having Gilbert's disease may constitute upper end of the normal range rather than a disease state. The term "constitutional hyperbilirubinaemia" is preferable, and its use can be coupled with reassurance to the patient that the condition causes no symptoms.

Bilirubin↗
PubMed1976

Serum gamma-glutamyl transpeptidase: its clinical significance.

Serum gamma-glutamyl transpeptidase (gamma-GT) level was estimated in 132 patients with different liver diseases (chronic persistent and chronic active hepatitis, postnecrotic cirrhosis, chronic alcholic hepatitis and alcoholic cirrhosis, cholestasis syndrome, fatty liver, Gilbert disease) and malignancies with and without liver involvement. The gamma-GT levels were compared with the values for serum bilirubin, transaminases (GOT, GPT) and alkaline phosphatase in the same patients. gamma-GT values were normal in chronic persistent hepatitis and increased in chronic active hepatitis. Very high activities were measured in chronic alcoholic cirrhosis in contrast to postnecrotic cirrhosis. gamma-GT proved to be more sensitive than alkaline phosphate as an index of cholestasis and liver involvement in malignancies. It is suggested that gamma-GT activity offers valuable aid in differential diagnostics of liver-diseases. gamma-GT being an inducible enzyme, its activity may be raised by enzyme inducing drugs also in subjects without liver disease.

Cholestasis↗
PubMed1979

Studies of human liver bilirubin-glycosyl transferase. Bilirubin UDP-xylosyl and UDP-glucuronyl transferase activities in diseased human livers.

The activity of bilirubin UDP-xylosyl transferase as well as UDP-glucuronyl transferase in liver biopsy specimens of 3 control subjects, 42 cases with liver disease and 5 cases with Gilbert's syndrome was measured. Normal values of these enzyme levels were determined to be 142--302 U/kg protein for the former and 260--400 U/kg protein for the latter. Both enzyme levels in acute hepatitis in convalescence and chronic hepatitis were nearly in the normal range. In the cirrhotic liver they tended to a small decrease and patients with Gilbert's syndrome demonstrated significantly decreased enzyme levels. These enzyme levels were only correlated with serum unconjugated bilirubin concentration, but not with the other liver function tests. Finally, both enzyme activities were exactly correlated with each other.

Bilirubin↗
PubMed1975

Estimation of hepatic bilirubin UDP-glucuronyl transferase in patients with noncirrhotic portal fibrosis and liver disease: Significance and limitations.

Using a micromethod, hepatic bilirubin UDP-glucuronyl transferase has been assayed in percutaneous needle biopsy samples obtained from patients with infectious hepatitis, postnecrotic cirrhosis, Gilbert's disease, noncirrhotic portal fibrosis (NCPF), granuloma of the liver, and extrahepatic portal vein obstruction. The results were compared with those obtained from 10 control subjects. Patients with cirrhosis and infectious hepatitis revealed normal bilirubin transferase levels, whereas those with Gilbert's disease showed significantly low enzyme levels. Many patients with NCPF, some with extrahepatic portal vein obstruction, and patients with granulomatous involvement of the liver demonstrated significantly low levels. This low hepatitic-enzyme activity was not associated with hyperbilirubinemia. The mechanism of such low values in NCPF and other disorders is not known. It is postulated that low heaptic-enzyme activity in noncirrhotic portal fibrosis is due to sparse smooth endoplasmic reticulum. This study also emphasizes that serum bilirubin may remain normal with very low hepatic-enzyme activity. Although induction of the microsomal enzyme bilirubin transferase was observed following phenobarbitone administration in noncirrhotic portal fibrosis, this was not apparent in patients with cirrhosis, possibly due to maximal enzyme induction having been achieved by endogenous substrate.

Adult↗
PubMed1975

[Value of a low-calorie diet in the diagnosis of Gilbert's syndrome].

Reduction of the calorie intake to 400 per day was employed in clinching the diagnosis in a case of Gilbert's disease admitted in the course of remission. This diet, in fact, results in a marked increase in serum bilirubin concentration. The non-conjugated form, in fact, reached values 5 times greater than those observed initially, within 24 hr after commencing the diet. Unlike other cases reported in the literature, this increase occurred in spite of an initially normal blood bilirubin level.

Adult↗