Bilirubin and paranitrophenol glucuronyl transferase activities and ultrastructural aspect of the liver in patients with chronic hemolytic anemias.
The hepatic ultrastructural aspect and the hepatic bilirubin (Bil-GT) and paranitrophenol (PNP-GT) glucuronyl transferase activities were studied in twenty subjects with a chronic hemolytic anemia (HA) and five subjects with an aplastic anemia. In chronic HA: the hepatic ultrastructural aspect showed mitochondrial abnormalities affecting structure (paracrystalline inclusions), size (giant mitochondria) and shape (irregular mitochondria); there was a decrease of Bil-GT activity in 80 percent of the patients. No connection could be shown between the HA etiology and the degree of the enzymatic activity decrease. The physiopathological mechanism of this enzymatic activity decrease is unknown; no deficiency of PNP-GT was observed; in chronic aplastic anemias there does not seem to be any modification, either of Bil-GT activity, or of hepatic PNP-GT activity. It is concluded that Bil-GT is often decreased in HA and that neither Bil-GT measurement nor the ultrastructural aspect of the liver distinguish chronic HA from Gilbert's syndrome.
D000741:Anemia, Aplastic / Q000201:enzymology / Q000473:pathology; D000743:Anemia, Hemolytic / Q000201:enzymology* / Q000473:pathology; D001663:Bilirubin; D005965:Glucuronates; D006602:Hexosyltransferases / Q000378:metabolism*; D006801:Humans; D008099:Liver / Q000201:enzymology* / Q000648:ultrastructure; D008930:Mitochondria, Liver / Q000648:ultrastructure; D009596:Nitrophenols
D005965:Glucuronates; D009596:Nitrophenols; D006602:Hexosyltransferases; D001663:Bilirubin