Hemolytic‐uremic Syndrome: An Analysis of the Natural History and Prognostic Features
Hemolytic‐uremic Syndrome: An Analysis of the Natural History and Prognostic Features
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溶血尿毒症综合征：自然史和预后特征分析
DOI：
10.1111/j.1651-2227.1984.tb09962.x
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发表时间：
1984
期刊：
影响因子：
--
通讯作者：
H. Mathieu
中科院分区：
文献类型：
--
作者：
Chantal Loirat;E. Sonsino;A. V. Moreno;G. Pillion;Mercier Jc;F. Beaufils;H. Mathieu
1974年至1981年间，收治了67例溶血尿毒综合征（HUS）患儿。其中，52例（78%）年龄小于3岁。所有患儿均发生急性肾功能衰竭，48例（72%）需要腹膜透析. 20例患者的病因各异，从细菌和病毒感染（分别为7例和5例）到肾放射化疗（2例）和既存肾小球病（1例）。5例（7%）儿童在急性期死亡。对56例病例进行长期随访（平均3年3个月），结果显示，37例儿童（60%）迄今未出现功能性后遗症，8例（13%）仅出现轻度后遗症，3例（5%）正在接受反复血液透析，3例患有重度慢性肾衰竭和高血压（HBP），5例（8%）患有HBP和正常肾功能。虽然所有年龄组的恢复率约为60%，但3岁以上儿童的死亡率和严重后遗症是3岁以下儿童的两倍（42%）。37例肾组织学检查显示皮质坏死12例，肾小球血栓性微血管病（TMA）22例，动脉血栓性微血管病3例。所有动脉TMA病例的预后都很差，58%的病例表现为皮质坏死。
Sixty‐seven children with hemolytic‐uremic syndrome (HUS) were admitted between 1974 and 1981. Of these, 52 (78 %) were aged less than 3 years. All children had acute renal failure and 48 (72 %) required peritoneal dialysis. The etiology in twenty cases varied from bacterial and viral infections (7 and 5 cases, respectively) to renal irradiation with chemotherapy (2) and preexisting glomerulopathy (1). 5 (7 %) children died during the acute phase of the illness. Long‐term follow‐up (mean 3 years 3 months) of 56 cases showed that 37 children (60 %) had so far experienced no functional sequelae and 8 (13 %) only mild sequelae while 3 (5%) were on iterative hemodialysis, 3 had severe chronic renal failure and high blood pressure (HBP) and 5 (8 %) had HBP and normal kidney function. While the recovery rate was approximately 60 % in all age groups, the mortality rate and serious after‐effects were twice as frequent (42 %) in children over 3 years of age as in those less than 3. Renal histology (total of 37) showed 12 cases of cortical necrosis, 22 of glomerular thrombotic microangiopathy (TMA) and 3 arterial TMA. Prognosis was poor for all cases of arterial TMA and 58 % of those exhibiting cortical necrosis.