Acute immune complex mediated glomerulonephritis in a Chinese girl with Wiskott-Aldrich syndrome variant.
This study presents a 12-year-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 isohaemagglutinins, recurrent infections and high IgE. She developed hematuria about one month prior to admission. For her severe thrombocytopenia splenectomy was performed and proved to be effective. At the time of splenectomy, renal biopsy was done and showed proliferative glomerulonephritis with coarse granular deposition of immunoglobulins (IgA and IgM) and C3. Electron microscopy demonstrated granular electron-dense deposits in the glomerulus, indicating an immune complex glomerulonephritis.
D000917:Antibody Formation; D000936:Antigen-Antibody Complex / Q000276:immunology*; D001706:Biopsy; D002633:Chemotaxis; D002648:Child; D003176:Complement C3 / Q000032:analysis; D005260:Female; D005455:Fluorescent Antibody Technique; D014644:Genetic Variation; D005921:Glomerulonephritis / Q000150:complications / Q000276:immunology*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007678:Kidney Glomerulus / Q000473:pathology; D008264:Macrophages / Q000276:immunology; D008854:Microscopy, Electron; D009504:Neutrophils / Q000276:immunology; D014165:Transfer Factor / Q000627:therapeutic use; D014923:Wiskott-Aldrich Syndrome / Q000150:complications / Q000235:genetics*
D000936:Antigen-Antibody Complex; D003176:Complement C3; D007070:Immunoglobulin A; D007075:Immunoglobulin M; D014165:Transfer Factor