三例新生儿马兜铃酸肾病临床特点及长期随访分析  

Clinical characteristics and long-term follow-up analysis of three cases with newborn aristolochic acid nephropathy

作　　者：李正红[1] 魏珉[1] 丁国芳[1] 王丹华[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院儿科,100730

出　　处：《中华儿科杂志》2011年第11期814-817,共4页Chinese Journal of Pediatrics

摘　　要：目的总结新生儿木通中毒所致马兜铃酸肾病的临床特点和转归。方法回顾性分析3例新生儿因木通所致马兜铃酸肾病的临床表现、治疗和转归情况。结果3例患儿均在服用含有木通的中药后出现呕吐、腹泻及尿量减少，出现急性肾功能衰竭、肾小球及肾小管损害。实验室检查表现为高血钾、低血钠，肌酐、尿素氮增高及代谢性酸中毒。轻度肾小球损害，表现为蛋白尿及血β2微球蛋白增高。肾小管功能损害表现为碱性尿，尿β2微球蛋白增高，尿糖、尿酮体及尿氨基酸阳性。经过对症治疗后肾功能在3～4周恢复正常，5～8个月尿蛋白转阴，3个月一1年尿氨基酸转阴，9个月～3年尿糖转阴，5．0～5．5年尿pH值降至7．0。5．5～6．0年后停止服用枸橼酸合剂。3例患儿随访12年，前11年内3例患儿的血清肌酐均在正常范围内，但近期随访显示2例患儿的血清肌酐升高，例3的血清肌酐正常。3例患儿估算肾小球滤过率（eGFR）均有所降低，其中例1、2的eGFR低于90ml／（min·1．73m^2），近6年的eGFR每年降低1．1ml/（min·1．73m^2）及0．6ml／（min·1．73m^2），1例患儿的eGFR较前无明显降低。3例患儿血气正常，尿常规阴性，但血、尿β2微球蛋白仍较高。尿N-乙酰B葡萄糖苷酶（NAG）降至正常后又有所增高。结论新生儿马兜铃酸肾病可引起急性肾功能衰竭和肾小管功能损害，经过对症治疗肾功能可在短期内恢复正常，但肾小球滤过率呈缓慢下降趋势且肾小管损害可持续多年，需要长期随访。Objective To summarize the clinical characteristics and prognosis of newborn aristolochic acid nephropathy induced by akebia. Method Retrospective analysis of clinical manifestations, therapy and prognosis was made upon data of 3 newborn infants with renal function lesion induced by akebia. Result Three infants who were fed with Chinese herbal medicines containing akebia trifoliate suffered from acute renal failure, renal glomerular and tubular injury, with symptoms of vomiting, diarrhea, and oliguria. Laboratory tests manifested hyperpotassemia, hyponatremia, elevation of serum creatinine and urea nitrogen, and metabolic acidosis. Renal glomerular lesion was mild, presented with proteinuria and increased serum β2 microglobin. Renal dysfunction was manifested with alkaline urine, glucosuria, positiveness of urine glucose, ketone and aminoaciduria, and increased urine β2 microglobin excretion. After symptomatic treatment for 3 to 4 weeks, the renal function of these infants recovered. Proteinuria, aminoaciduria and glucosuria turned negative within 5 to 8 months, 3 months to 1 year, and 9 months to 3 years, respectively. Urine pH decreased to 7.0 after 5.0-5.5 years. All cases took citric acid mixtures for 5.5 to 6 years. A 12- years follow-up demonstrated that serum creatinine of 3 cases were within normal range during the first 11 years of life, however recent follow-up showed increased serum creatinine of case 1 and case 2, except for serum creatinine of case 3 remained normal. The estimated glomerular filtration rate （eGFR） of all the 3 cases decreased. Among which, eGFR of case 1 and case 2 were lower than 90 [ ml/（ min . 1.73 m2 ） ], and decreasedl. 1 [ml/（min .1.73 m2）] and 0.6 [ml/（min . 1.73 m2）] per year during recent six years, respectively. No obvious decrease of eGFR was observed in case 3. Blood gas analysis and urine routine were normal, yet blood and urine β2 microglobin excretion were still high. Urinary N-acetyl-β-D- glucosaminidase increased again after having returned to nor

关 键 词：木通 马兜铃酸 肾病 婴儿 新生 随访研究 

分 类 号：R726.9[医药卫生—儿科]