Abstract: | Red cell survival was determined in 20 patients with idiopathic unconjugated hyperbilirubinæmia without overt signs of hæmolysis (Gilbert's syndrome) by means of a double isotope technique with the use of diisopropylfluorophosphate (DF32P) and chromium (51Cr) simultaneously. Red cell life span was significantly reduced in 42% of patients. However, the degree of hæmolysis was not sufficient to produce hyperbilirubinæmia in persons with normal hepatic function. The incidence of familial involvement in patients with reduced red cell survival was not significantly different from that in patients with normal red cell survival. Similarly, there was no difference between the groups with respect to symptomatology, age of onset of jaundice, or results of laboratory investigations. It is concluded that the hyperbilirubinæmia in this syndrome is more likely to be due to a defect in hepatic handling of bilirubin than to compensated hæmolysis. This defect appears to occur whether or not there is a reduced red cell survival. It remains to be shown why some patients with this syndrome have a reduced red cell survival while others do not. |