Wiskott-Aldrich syndrome with membranous nephropathy in a child
10.3760/cma.j.cn441217-20240118-00123
- VernacularTitle:Wiskott-Aldrich综合征并发幼儿期膜性肾病1例
- Author:
Shouhuan CHEN
1
;
Jing CHEN
;
Shicong YANG
;
Yuxin PEI
;
Xiaoyun JIANG
;
Zhilang LIN
Author Information
1. 中山大学附属第一医院儿科，儿童肾脏风湿病中心，广州 510080
- Keywords:
Child;
Wiskott-Aldrich syndrome;
Glomerulonephritis, membranous;
Nephrotic syndrome;
Hematopoietic stem cell transplantation
- From:
Chinese Journal of Nephrology
2024;40(10):815-817
- CountryChina
- Language:Chinese
-
Abstract:
Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive genetic disorder characterized by thrombocytopenia, eczema, recurrent infections, and susceptibility to autoimmune diseases. The renal complication of WAS is mainly manifested as IgA nephropathy. Membranous nephropathy (MN) in children mostly has secondary factors, and its occurrence is related to immune disorders. This paper reports a case of WAS in an infant with bleeding tendency and recurrent infections, complicated with nephrotic syndrome during the course, confirmed as MN by pathological examination. After treatment with glucocorticoids, tacrolimus and hematopoietic stem cell transplantation, nephrotic syndrome was manifested as steroid-resistant and partially sensitive to tacrolimus. Proteinuria continued to relieve 3 months after hematopoietic stem cell transplantation and renal function remained stable. WAS complicated with MN is extremely rare, and its renal prognosis is still unclear, which deserves the attention of clinicians.
