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NDT Advance Access published online on August 29, 2006
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfl394
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© The Author [2006]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Received January 4, 2006
Accepted June 6, 2006

Original Article

Spectrum of clinical features and type IV collagen {alpha}-chain distribution in Chinese patients with Alport syndrome

Gong Wei 1, Liu Zhihong 1 *, Chen Huiping 1, Zeng Caihong 1, Chen Zhaohong 1, and Li Leishi 1

1 Research Institute of Nephrology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China

* To whom correspondence should be addressed.
Liu Zhihong, E-mail: zhihong{at}21cn.net



  Abstract

Background. Alport syndrome (AS) is a clinically and genetically heterogeneous nephropathy. The goal of the present study is to delineate clinical characteristics and the distribution of type IV collagen chains in Chinese AS patients and to identify any {alpha}(IV)-chain expression and clinical phenotype correlation.

Methods. A total of 126 biopsy-proven patients meeting immunofluorescence criteria for the diagnosis of AS were investigated retrospectively.

Results. Microscope haematuria associated with proteinuria was observed as the initial symptom in 77.8% of the patients; 59.8% showed hearing impairment and 22.9% had ocular abnormalities. Renal biopsies from 118 patients revealed mesangial proliferative glomerulonephritis (61.9%) and focal and segmental sclerosis glomerulonephritis (37.3%). Ten different distribution patterns for the type IV collagen {alpha}-chains were found in the kidney; six of these are presented here for the first time. Based on renal immunofluorescence findings, 113 patients (89.7%) were classified as X-linked dominant inherited AS (XLAS) and 13 (10.3%) as autosomal recessive AS (ARAS). The XLAS group was divided into typical and non-typical subgroups according to the expression patterns for the {alpha}3(IV)-chain. Clinical phenotypes were more severe in XLAS patients than in ARAS patients and the prognosis was poorer in typical XLAS patients than non-typical XLAS patients.

Conclusion. In China, the incidence of XLAS is 89.7% and 10.3% for ARAS. Chinese patients with AS have various distribution patterns of type IV collagen {alpha}-chains. The distribution pattern of type IV collagen {alpha}-chains in the kidney may correspond to the severity of the clinical phenotype.

Keywords: Alport syndrome (AS); indirect immunofluorescence; phenotype; type IV collagen.
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