title: [Regression of glomerulosclerosis in a girl with C3 nephropathy].
abstract: Regression of glomerular sclerosis was documented in experimental models as a result of RAA system blocking and/or cyclosporin A (CsA) treatment. Here we present a case of a girl suffering from nephrotic syndrome (NS) in whom unusual healing of glomerular changes appeared. First time the girl was admitted to our department at the age of 4 year with 2 years history of steroid-dependent NS. Kidney biopsy studied in light microscopy revealed 11 glomeruli among which 5 were totally sclerosed and the others showed mesangial proliferation and segmental mesangial matrix expansion. Diffuse interstitial mononuclear cells infiltration was also visible. In fluorescence microscopy only granular C3 and fibrinogen deposits were seen. C3 nephropathy was diagnosed and cyclophosphamide therapy started. As no effects appeared, CsA was introduced together with angiotensin converting enzyme inhibitor (ACEI) enalapril for two years without any relapse during treatment. Prednisone was gradually reduced and finally stopped after a year. On the second biopsy performed to assess CsA nephrotoxicity among 16 glomeruli evaluated in light microscopy only 1 was sclerosed, while the others presented just mild mesangial proliferation. No interstitial changes were found. Fluorescence showed IgA and IgM added to glomerular C3 deposits. CsA was then stopped and after 4 months relapses and steroid-dependency appeared again, so CsA was reintroduced. Actually the girl is 8-year-old, remission of NS has been observed for six months. As a conclusion we would like to suggest that treatment with CsA and ACEI may cause glomerular healing even in a case of advanced glomerulosclerosis.
authors.affiliation: Z Oddziału Nefrologii ze Stacja, Dializ Specjalistycznego Samodzielnego Publicznego Zakładu Opieki Zdrowotnej nad Dzieckiem i Młodzieza, w Szczecinie. pednefrolszczecin@wp.pl
authors.forename: Tomasz
authors.initials: T
authors.lastname: Jarmoliński
authors.forename: Barbara
authors.initials: B
authors.lastname: Borucka
authors.forename: Aldona
authors.initials: A
authors.lastname: Wozniak
mesh_terms: D000806:Angiotensin-Converting Enzyme Inhibitors / Q000008:administration & dosage*; D002675:Child, Preschool; D003176:Complement C3 / Q000378:metabolism*; D003520:Cyclophosphamide / Q000008:administration & dosage; D016572:Cyclosporine / Q000008:administration & dosage*; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000473:pathology; D012074:Remission Induction; D013997:Time Factors
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D003176:Complement C3; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003520:Cyclophosphamide