儿童慢性马兜铃酸肾病3例临床病理分析及文献复习  

Clinical and pathological analysis of 3 children with chronic aristolochic acid nephropathy and review of the literature

作　　者：张焱[1] 姚勇[1] 黄建萍[1] 刘景城[1] 肖慧捷[1] 杨霁云[1] 

机构地区：[1]北京大学第一医院儿科,北京100034

出　　处：《中国实用儿科杂志》2007年第8期616-620,共5页Chinese Journal of Practical Pediatrics

摘　　要：目的通过报告3例慢性马兜铃酸肾病,增加对其临床与病理特征的认识。方法对北京大学第一医院儿科收治的3例慢性马兜铃酸肾病患儿进行回顾性分析,总结其临床和病理特点,并复习相关文献。结果3例慢性马兜铃酸肾病患儿,男1例,女2例,年龄为10岁、14岁和16岁;分别因乙型肝炎、颅咽管瘤术后脑水肿和紫癜性肾炎服用含马兜铃酸成分的中草药或中成药4～8个月,于开始服用2个月至6年后发病;3例患儿均以不同程度的肾功能损害伴近-远端肾小管受损为主要表现,其中2例以贫血为首发症状,肾功能损害严重,均已达到肾衰竭尿毒症期,另1例以糖尿为首发表现,肾功能损害较轻,同时表现继发性Fanconi综合征。3例肾病理均以广泛性或多灶状寡细胞性间质纤维化和肾小管萎缩为特征,肾小球病变轻微,免疫荧光除2例因基础疾病见IgA(++～+++)外,均未见明显免疫复合物沉积。2例患儿停用含有马兜铃酸的药物后肾功能仍呈不同程度的进行性恶化,其中1例放弃治疗半年后死于消化道大出血,1例等待肾替代治疗;另1例肾功能损伤较轻者,停用含有马兜铃酸的药物后经小剂量糖皮质激素和营养肾小管治疗病情改善,肾功能及肾小管功能恢复,仍在临床随访中。结论儿童慢性马兜铃酸肾病临床特点是渐进性肾功能损害和肾小管功能受损,肾脏病理特征为广泛的寡细胞性间质纤维化和肾小管萎缩;在停服含马兜铃酸药物后肾功能恶化常不可逆转,最终导致终末期肾病;小剂量糖皮质激素对于缓解早期轻症者的病情可能有益。预防该病的关键在于提高对含马兜铃酸中药肾毒性的认识。Objective Many cases of aristolochic acid nephropathy （AAN） in adults had been reported throughout the world, but chronic aristolochic acid nephropathy （ CAAN ） had seldom been described in children. We reported 3 children with CAAN diagnosed recently to investigate the clinical and pathological characteristics of the disease in children. Methods The 3 cases were studied retrospectively, and relevant literatures was reviewed. Results Three children with CAAN ,including 1 boy and 2 girls ,aged 10,14 and 16 years respectively ,were referred to our department of pediatrics. They received traditional Chinese herb medicine containing aristolochic acid 4 to 8 months for different basal diseases including chronic aggressive hepatitis B, hydrocephalus secondary to the operation of craniopharyngioma and purpura nephritis, and suffered from CAAN from 2 months to 6 years after they began receiving the Chinese medication. The main manifestation of our patients was renal failure of various degree accompanied with proximal and distal tubular disfunction. Two of them began with anemia and suffered from serious renal failure,which had achieved uremia. The onset of another case was glucosuria with renal function impaired mildly, and she presented secondary Fanconi＇s syndrome simultaneously. Their pathological characteristics were diffuse paucicellular interstitial fibrosis and marked tubular atrophy with mild glomerular impairment. After withdrawal of the Chinese herb medicine containing aristolochic acid, renal failure in two of the three patients attenuated progressively. One of them died of gastrointestinal massive haemorrhage half a year later,another one was going to receive renal replacement treatment. Another case, whose renal function was impaired mildly, received low dosagesteroid and renal tubular nutrition therapy after withdrawal of the Chinese herb medicine containing aristolochic acid, her renal and tubular function ameliorated gradually. This patient isstill in follow-up so far. Conclusion The clinical fe

关 键 词：马兜铃酸 肾损害 儿童 

分 类 号：R692.5[医药卫生—泌尿科学]