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Membranoproliferative glomerulonephritis Type 3 associated with Kabuki syndrome.
MedLine Citation:
PMID:  23149249     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
First described in Japanese patients, Kabuki syndrome (KS) is a congenital condition associated with multiple anomalies and mental retardation. Although urological and immune abnormalities are common in KS, immune complex nephritis such as membranoproliferative glomerulonephritis (MPGN) has not yet been reported. We describe the first reported case of a KS patient with common variable immunodeficiency (CVI) and recurrent otitis media who developed severe proteinuria and hematuria due to MPGN Type 3 detected during the school urinary screening program in Japan. The patient was intravenously treated with methylprednisolone pulses followed by alternate-day prednisolone and an angiotensin receptor blocker. The patient showed remarkable improvement in both histological and urinary analyses. This case report suggests that immune abnormalities associated with KS can play an important role in the development of MPGN. Urinalysis should be regularly performed in KS patients with hypogammaglobulinemia and/or recurrent infection.
Authors:
Naoto Nishizaki; Shuichiro Fujinaga; Daishi Hirano; Hitohiko Murakami; Kouichi Kamei; Yoshiyuki Ohtomo; Toshiaki Shimizu; Kazunari Kaneko
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-14
Journal Detail:
Title:  Clinical nephrology     Volume:  -     ISSN:  0301-0430     ISO Abbreviation:  Clin. Nephrol.     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0364441     Medline TA:  Clin Nephrol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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