Membranoproliferative glomerulonephritis with partial lipodystrophy: discordant occurrence in identical twins.
The course of disease of a patient with membranoproliferative glomerulonephritis and partial lipodystrophy is described. The case is further characterized by a deficiency of C3 and C3- activator, by normal values of C4, by evidence of the nephritogenic factor, by raised fibrin degradation products and by an unselective proteinuria. The course of the glomerulonephritis runs parallel to a pronounced susceptibility to infection (at first varicella, tonsillitis and measles, later pneumonia, meningitis, encephalitis and hepatitis). On account of a nephrotic syndrome and an initative impairment of the renal function, a cytostatic treatment was begun, which although raising the C3 level did not influence the further course of the disease. As the patient has a healthy identical twin sister without lipodystrophy, who shows no reduction in C3 and no nephritogenic factor, this case proves that these diseases are acquired and not genetically determined.
D000293:Adolescent; D002644:Chickenpox / Q000150:complications; D002648:Child; D003176:Complement C3 / Q000172:deficiency / Q000378:metabolism; D003181:Complement C4 / Q000032:analysis; D003404:Creatinine / Q000097:blood; D004200:Diseases in Twins; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005921:Glomerulonephritis / Q000150:complications / Q000235:genetics* / Q000276:immunology; D006801:Humans; D007105:Immune Complex Diseases; D007678:Kidney Glomerulus / Q000473:pathology; D008060:Lipodystrophy / Q000150:complications / Q000235:genetics* / Q000276:immunology; D008566:Membranes; D009404:Nephrotic Syndrome; D011247:Pregnancy; D011507:Proteinuria; D014430:Twins, Monozygotic
D003176:Complement C3; D003181:Complement C4; D005338:Fibrin Fibrinogen Degradation Products; D003404:Creatinine