| Background: IgA nephropathy is the most common form of glomerulonephritis in the world. In adult, fifteen to 40 percent of patients eventually will progress to end stage renal disease after twenty years of follow-up. Indeed, IgA nephropathy is the main cause of end-stage renal disease in patients with primary glomerular disease. Until recently, there has been no enough knowledge about childhood IgA nephropathy, especially, there is no effective treatment available in children with IgA nephropathy and the therapy of IgA nephropathy in children remains controversial.Objective: In order to know more about childhood IgA nephropathy, to slow the progression of IgA nephropathy and to improve the prognosis, we investigated the clinical and pathological features of childhood primary IgA nephropahy in Hangzhou region and analyzed the association among clinical manifestations, renal histological changes andprognosis. Meanwhile, the risk factors associated with renal lesions were studied.Patients: Forty-six children with IgA nephropathy were confirmed by renal biopsy during Aug lst of 1994 to July 30th of 2004 in Affiliated Children's Hospital of Zhejiang University. Immnofluorescence studies demonstrating a predominant deposition of IgA within mesangial regions are essential to establish a definitive diagnosis of IgA nephropathy (the mean intensity of IgA stain ≥++),except for Schonlein-Henoch purpra, systemic lupus erythematosus, chronic liver disease, et al. Methods: Under the B-type ultrasound conducting, renal biopsies were performed with tru-cut auto-fine needles. Biopsy specimens obtained were stained by HE, PAS, PASM and Masson. IgA, IgG, IgM, C3, C4, C1q and fibrin were detected by direct immunofluorescence examination. HBsAg and HBcAg were also detected. Electromicroscopic examination was performed in some children. Clinical classification was evaluated according to criteria set by Children Nephrology of Chinese Medical Society in 2000 zhuhai meeting. Renal histo-pathological changes were evaluated according to Lee's grading system. Treatment was used according to different clinical type and Lee's grade. The association of initial clinical manifestations and renal histological changes withprognosis was analyzed in 46 cases with childhood primary IgA nephropathy. They were at follow-up with duration from ten months to ten years. Statistical analysis was performed using Statistical Packages for Social Science version 12.0. The risk factors associated with renal lesions were analyzed using multiple Logistic regression. Result: 1.Clinical characteristics: 31 cases were male, and 15 cases were female. There was a male: female ratio 2.07:1. 46 children with IgA nephropathy were diagnosed at age rang from six to fifteen years old. 19 of the 46 children (41.3%) with primary IgA nephropathy presented with macroscopic hematuna at the first onset of the disease, edema was in 41.3%. 8 of the 46 patients (17.4%) had no obvious symptoms and their microscopic hematuna and/or proteinuria were discovered during routine screening. 56.5% (26/46) of the patients had been recurrent episodes of macroscopic hematuna in the course of the disease. Isolated hematuria (32.6%) was the most common clinical type, while nephrotic syndrome (28.3%) was the second. 2. Histo-pathological features: The most common renal pathological alteration by light microscopy is focal or diffuse expansion of mesangial regions, including mesangial cells and matrix. Some patients companied with capsular adhesions, a few patients had crescent formation and few of them haddiffuse endocapillary proliferations and segmental sclerosis. In addition to the glomerular alterations, a variety of tubulo-interstitial changes may be identified, including tubular edema, tubular atrophy and interstitial inflammation. Interstitial fibrosis and vascular lesions may hardly be seen. Severe lesions at the time of biopsy were usually less. Most cases had Lee's grade II (43.5%) and grade 111(45.7%) histological lesions at diagnosis. IgA depositions with IgM (65.2%) w...
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