Plasma lipoprotein alterations and morphologic changes with lipid deposition in the kidney of patients with hepatorenal syndrome.
Four patients with advanced liver disease and progressive renal failure compatible with the diagnosis of hepatorenal syndrome have been studied. All four patients had low lecithin:cholesterol acyltransferase activity in plasma, and the concentration of cholesteryl esters was markedly reduced. The main lipoprotein classes were abnormal with an increased content of polar lipids. Electron microscopy of negatively stained lipoproteins from two of the patients (H.K. and I.A.) revealed large particles with layered membranes (700 to 2000 A in diameter) corresponding to the large molecular weight fraction of the low density lipoproteins. These structures were not present in the low density lipoproteins from the other two patients. In the renal biopsy from H.K. and in the necropsy specimen from I.A.  deposition of osmophilic material was found in the glomeruli (especially located subendothelially), in the basement membrane, and in the mesangial regions. The deposits were similar to those we have previously described in patients with familial lecithin:cholesterol acyltransferase deficiency and most probably represent cholesterol and phospholipids. It is suggested that the renal deposition of lipid may be related to the large molecular weight low density lipoprotein fraction and that the mechanisms involved in this lipid deposition are similar to those occurring in familial lecithin:cholesterol acyltransferase deficiency.
D005260:Female; D006801:Humans; D007668:Kidney / Q000378:metabolism* / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000378:metabolism* / Q000473:pathology; D050356:Lipid Metabolism; D008074:Lipoproteins / Q000097:blood*; D008107:Liver Diseases / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D007862:Phosphatidylcholine-Sterol O-Acyltransferase / Q000097:blood; D013577:Syndrome
D008074:Lipoproteins; D007862:Phosphatidylcholine-Sterol O-Acyltransferase