ACUTE IMMUNE-COMPLEX MEDIATED GLOMERULONEPHRITIS IN A CHINESE GIRL WITH WISKOTT-ALDRICH SYNDROME VARIANT

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  • 1 January 1984
    • journal article
    • research article
    • Vol. 53  (1) , 74-78
Abstract
This study presents a 12-yr-old girl with Wiskott-Aldrich syndrome variant, who developed acute glomerulonephritis without history of transfer factor therapy and the efficacy of splenectomy for the control of the patient''s thrombocytopenia. The patient presented with eczema, severe thrombocytopenia and immunodeficiency. The impaired immunity was featured by impaired delayed hypersensitivity and lymphoproliferative response to nonspecific mitogen, low serum IgM, low isohemagglutinins, recurrent infections and high IgE. She developed hematuria .apprx. 1 mo. prior to admission. Severe thrombocytopenia splenectomy proved effective. Renal biopsy at the time of splenectomy showed proliferative glomerulonephritis with coarse granular deposition of IgA and IgM and [complement] C3. EM demonstrated granular electron-dense deposits in the glomerulus, indicating an immune complex glomerulonephritis.