Renal accumulation of glycosphingolipids. Report of a case and a review of literature.
A 62-year-old woman gave clinical manifestation of liver cirrhosis. Urinary protein was false positive, no uremia was found and renal changes were entirely overlooked. Deposition of abundant lipids (globoside and ceramide trihexoside) was found in the kidneys; essentially degenerative changes of the tubular epithelia were noted. These renal changes were compared with those in Fabry's disease.
D000328:Adult; D002518:Ceramides / Q000378:metabolism*; D000795:Fabry Disease / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006028:Glycosphingolipids / Q000378:metabolism*; D006801:Humans; D007668:Kidney / Q000378:metabolism* / Q000473:pathology; D008064:Lipidoses / Q000473:pathology; D008103:Liver Cirrhosis / Q000473:pathology; D008297:Male; D008875:Middle Aged
D002518:Ceramides; D006028:Glycosphingolipids