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目的了解伴新月体形成的原发性IgA肾病(IgAN)患儿临床与病理特点。方法选择2000年1月至2011年1月在温州医学院附属育英儿童医院经肾活检确诊为原发性IgAN且病历资料完整的患儿78例,根据是否伴新月体形成分为伴新月体形成的IgAN组(C组)和不伴新月体形成的IgAN组(NC组),并比较两组结果。结果 78例原发性IgAN患儿中男性57例,女性21例;年龄平均(9.32±3.16)岁。C组33例(42.3%),NC组45例(57.7%)。与NC组相比,C组肾活检前病程更短(2.15±4.06)个月对(9.87±19.09)个月,P<0.05],肉眼血尿发生率更高(30/33对25/45,P<0.01),24h尿蛋白定量更多(93.08±82.75)mg/(kg·d)对(44.92±68.44)mg/(kg·d),P<0.05],且表现为大量蛋白尿者更多(19/31对10/42P<0.01),肾功能损害者较多(8/33对2/45,P<0.01)。在肾脏病理改变上,C组中-重度系膜增生、球囊粘连、毛细血管内皮增生均显著多于NC组(P均<0.01)。结论儿童伴新月体形成的原发性IgAN临床表现、病理改变较重,临床上应提高对此类型IgAN的认识,争取早期肾活检,早期诊断,积极治疗,在急性期控制疾病进展以改善预后。

Clinicopathological characteristics of the primary IgA nephropathy with crescentic formation in children

Objective To understand the clinical and pathological characteristics of the primary IgA nephropathy(IgAN) with crescentic formation in children.Methods Clinicopathological data of 78 children with primary IgAN were analyzed.These patients were divided into two groups according to with or without crescents,and their results were compared.Results There were 57 male and 21 female patients,with a mean age of(9.32 ± 3.16) years old ;33 of them were in Group C(with crescents) and 45 were in Group NC(without crescents).Compared with Group NC,there were significant differences in the course of disease (2.15 ± 4.06) m vs(9.87±19.09) m,P< 0.05 ],macrohematuria(30/33 vs 25/45,P< 0.01),the levels of 24hrs proteinuria (93.08 ± 82.75) mg/(kg · d) vs(44.92 ± 68.44) mg/(kg · d),P< 0.05 ],the case number of severe proteinuria(19/31 vs 10/42,P< 0.01) and occurence of renal impairment(8/33 vs 2/45,P< 0.01) in Group C.In renal pathology,significantly more cases in Group C had moderate-severe mesangial proliferation,endocapillary proliferation and tuft adhesion than in Group NC(P< 0.01).Immunofluorescence of both Group C and Group NC showed no significant difference.Conclusion The clinical manifestation and renal lesions of IgAN with crescentic formation in childhood are worse than those without,thus we should improve the understanding of this type of IgAN so as to make early diagnosis,treatment and improve the prognosis of this disease.