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

页码：1495 / 1500

页数：6

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