Cyclosporin A may cause injury to undifferentiated glomeruli persisting in patients with Alport syndrome

• Published in: Clinical and experimental nephrology 2014-06, Vol.18 (3), p.492-498
• Main Authors: Sugimoto, Keisuke, Fujita, Shinsuke, Miyazawa, Tomoki, Nishi, Hitomi, Enya, Takuji, Izu, Akane, Wada, Norihisa, Sakata, Naoki, Okada, Mitsuru, Takemura, Tsukasa
• Format: Article
• Language: English
• Subjects: Adolescent; Biopsy; Cell Differentiation; Child; Collagen Type IV - metabolism; Cyclosporine - adverse effects; Cyclosporine - therapeutic use; Female; Follow-Up Studies; Humans; Kidney Diseases - chemically induced; Kidney Diseases - epidemiology; Kidney Diseases - pathology; Kidney Glomerulus - metabolism; Kidney Glomerulus - pathology; Laminin - metabolism; Male; Medicine; Medicine & Public Health; Nephritis, Hereditary - drug therapy; Nephrology; Original Article; Retrospective Studies; Risk Factors; Urology; Young Adult
• ISSN: 1342-1751; 1437-7799
• DOI: 10.1007/s10157-013-0836-2

Background/Aims Alport syndrome (AS) is a renal disorder caused by a genetic abnormality of type IV collagen α3 and α4, or α5 genes and shows a poor prognosis. Since the defect of type IV collagen synthesis disturbs the maturation process of the glomerular capillary loop, residual immature glomeruli persist after birth. The therapeutic efficacy of cyclosporin A (CyA) for AS patients seems to be controversial. We recently noted that renal specimens obtained from a child with AS who was treated with CyA and then developed CyA nephropathy included an increased number of undifferentiated embryonic-type glomeruli. Methods We analyzed renal histologic and immunohistologic findings in children with AS who did ( n  = 3) or did not ( n  = 2) develop CyA-induced nephropathy despite appropriately low serum CyA concentrations (