Wiskott-Aldrich syndrome with IgA nephropathy: a case report and literature review

被引：13
|
作者：
Liu, Chia-Hung [1 ,2 ]
Wu, Kang-Hsi [2 ,3 ]
Lin, Tze-Yi [3 ,4 ]
Wei, Chang-Ching [2 ,3 ]
Lin, Ching-Yuang [2 ,3 ]
Chen, Xian-Xiu [2 ,3 ]
Lee, Wen-I [5 ]
机构：
[1] Taichung Armed Forces Gen Hosp, Dept Paediat, Taichung, Taiwan
[2] China Med Univ Hosp, Dept Paediat, Div Nephrol, Taichung 40402, Taiwan
[3] China Med Univ, Taichung, Taiwan
[4] China Med Univ Hosp, Dept Pathol, Taichung 40402, Taiwan
[5] Chang Gung Univ, Primary Immunodeficiency Care & Res PICAR Inst, Dept Paediat Allergy Immunol & Rheumatol, Chang Gung Childrens & Mem Hosp,Coll Med, Tao Yuan, Taiwan
来源：
INTERNATIONAL UROLOGY AND NEPHROLOGY | 2013年 / 45卷 / 05期
关键词：
Antibiotic prophylaxis; IgA nephropathy; Splenectomy; Wiskott-Aldrich syndrome; RENAL-TRANSPLANTATION; GLOMERULONEPHRITIS; PATIENT; SPLENECTOMY;
D O I：
10.1007/s11255-012-0178-0
中图分类号：
R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];
学科分类号：
1002 ; 100201 ;
摘要：
The pathogenesis of renal involvement in Wiskott-Aldrich syndrome (WAS) is unclear and renal outcome is generally poor in such situations. Here we present the case of an 8-year-old boy with WAS who developed hematuria, proteinuria, and declining renal function that did not improve with the combined use of immunosuppressive agents and angiotensin-converting-enzyme inhibitor. Renal pathology revealed IgA nephropathy (IgAN). The patient underwent splenectomy for refractory thrombocytopenia. The proteinuria remitted and renal function improved after splenectomy, long-term antibiotic prophylaxis, and tapering of immunosuppressive agents.
引用
收藏
页码：1495 / 1500
页数：6
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