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目的总结我院儿科22年来儿童原发性IgA肾病的发病率、发病诱因、临床分型和病理分级情况及临床分型与病理分级间的关系。方法回顾性分析256例原发性IgA肾病患儿的临床分级、病理分级、实验室检查及临床分型与病理分级间的关系。结果儿童IgA肾病占同期肾活检患儿的14·2%;IgA肾病的诱发因素依次为呼吸道感染126例(占49·2%),胃肠道感染43例(占16·8%)、慢性扁桃体炎24例(占9·4%)和劳累12例(占4·7%);临床分型中孤立性血尿型134例(占52·3%),肾病综合征型63例(24·6%),血尿和蛋白尿型39例(15·2%);病理改变以Ⅲ级为主(147例,57·4%),免疫沉积物荧光检查系膜区均有IgA沉积(100%),免疫病理分型以IgA+IgG型最多105例(占41%),IgA+IgG+IgM型77例(占30%)、单纯IgA型43例(占17%)、IgA+IgM型31例(占12%);肾脏病理损害的程度与免疫沉积物的种类有关,伴有IgM沉积者肾脏病理损害的程度较重。结论随着肾活检的广泛开展,IgA肾病的检出率逐步提高,IgA肾病临床表现的多样性及病理改变的程度、免疫沉积物的种类对指导治疗与判断预后具有重要的意义。
Objective To summarize the incidence of primary IgA nephropathy in children in our hospital for 22 years, the causes of the disease, the clinical classification and pathological grading, and the relationship between clinical classification and pathological grade. Methods A retrospective analysis of 256 cases of primary IgA nephropathy in children with clinical grade, pathological grading, laboratory tests and clinical classification and the relationship between pathological grade. Results IgA nephropathy in children accounted for 14.2% of children with renal biopsy in the same period. The predisposing factors of IgA nephropathy were respiratory tract infection in 126 cases (49.2%), gastrointestinal tract infection in 43 cases (16.8%), chronic There were 24 cases (accounting for 9.4%) of tonsillitis and 12 cases of fatigue (4.7%). Among them, 134 cases (52.3%) of isolated hematuria and 63 cases of nephrotic syndrome (147 cases, 57.4%). Immunofluorescence showed IgA deposition in the mesangium area (100% ). Immunohistopathology showed that IgA + IgM was the most common in 105 cases (41%), IgA + IgG + IgM in 77 cases (30%), simple IgA in 43 cases (17%), IgA + (12%). The extent of renal pathological damage was related to the type of immune deposits. Patients with IgM deposition had a more severe renal pathological lesion. Conclusion With the extensive renal biopsy, the detection rate of IgA nephropathy gradually increased. The clinical manifestations of IgA nephropathy and the degree of pathological changes, the type of immune deposits for guiding the treatment and prognosis of great significance.