Membranoproliferative glomerulonephritis with partial lipodystrophy: discordant occurrence in identical twins.

Abstract

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.

• Authors: W Reichel, J Köbberling, H Fischbach, F Scheler
• Journal: Klinische Wochenschrift (Klin Wochenschr) Vol. 54 Issue 2 Pg. 75-81 (Jan 15 1976) ISSN: 0023-2173 [Print] Germany
• PMID: 129586 (Publication Type: Case Reports, Journal Article)

Chemical References

• Complement C3
• Complement C4
• Fibrin Fibrinogen Degradation Products
• Creatinine

Topics

• Adolescent
• Chickenpox (complications)
• Child
• Complement C3 (deficiency, metabolism)
• Complement C4 (analysis)
• Creatinine (blood)
• Diseases in Twins
• Female
• Fibrin Fibrinogen Degradation Products (analysis)
• Glomerulonephritis (complications, genetics, immunology)
• Humans
• Immune Complex Diseases
• Kidney Glomerulus (pathology)
• Lipodystrophy (complications, genetics, immunology)
• Membranes
• Nephrotic Syndrome
• Pregnancy
• Proteinuria
• Twins, Monozygotic