kidney-nephrology-2017-scientifictracks-abstracts

19 / 33

Page 51
Notes:
conferenceseries.com
Volume 3, Issue 3 (Suppl) J Kidney, an open access journal
ISSN:2472-1220 Kidney & Nephrology 2017
August 28-30, 2017
August 28-30, 2017 Philadelphia, USA
15th Annual Congress on
Kidney: Nephrology & Therapeutics
Successful cyclosporin a therapy for diffuse mesangial sclerosis associated withWT1 mutations
Koji Nagatani
Uwajima City Hospital, Japan
Wilms’ tumor suppressor gene 1 (WT1) mutations are found in Denys-Drash syndrome, Frasier syndrome and
isolated diffuse mesangial sclerosis; these mutations lead to the occurrence of diffuse mesangial sclerosis (DMS) and
focal segmental glomerulosclerosis. Nephrotic syndrome (NS) caused due to DMS is unresponsive to drug therapy and is
characterized by rapid progression to end-stage renal disease. Here, we report a case of a 3 years and 5 months old girl with
NS caused due to DMS who responded favorably to cyclosporin A (CsA) and angiotensin-I converting enzyme inhibitor
(ACE-I). The light microscopic findings of the renal biopsy before CsA therapy revealed the early stage of DMS, which showed
small glomerulus with diffuse mesangial matrix increase and mesangial hypercellularity and hyperplastic podocytes. However,
prominent epithelial proliferation was not found in the specimen. CsA therapy induced a dose-dependent decrease in her
urinary protein/creatinine ratio and resulted in partial remission of NS and maintenance of normal renal function for over 3
years. The second biopsy at 3 years old revealed the improvement on the light microscopic findings. CsA may be effective for
DMS with WT1 mutations, if therapy is started before creatinine levels increase and in the early stage of DMS. In children with
WT1 mutation, CsA therapy may prevent prompt progression to end-stage renal disease.
Biography
Koji Nagatani has completed his graduation from Hamamatsu University, School of Medicine, Shizuoka, Japan in 1998 and belonged to Ehime University Graduate
School of Medicine, Department of Pediatrics. He is a Member of The Japanese Society for Pediatric Nephrology, Japanese Society of Nephrology, International
Pediatric Nephrology Association and International Society of Nephrology. He is the Director of Department of Pediatrics, Uwajima City Hospital, Japan
Koji Nagatani, J Kidney 2017, 3:3 (Suppl)
DOI: 10.4172/2472-1220-C1-002