Non-enteropathic hemolytic uremic syndrome: Causes and short-term course
Non-enteropathic hemolytic uremic syndrome: Causes and short-term course
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DOI：
10.1053/j.ajkd.2004.02.010
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发表时间：
2004-06-01
影响因子：
13.2
通讯作者：
Trachtman, H
中科院分区：
文献类型：
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作者：
Constantinescu, AR;Bitzan, M;Trachtman, H
背景：非腹泻性或肺炎链球菌相关溶血性尿毒症综合征（HUS）是一组异质性疾病。本研究的目的是：(1)描述非肠病性溶血性尿毒综合征的发病率、病因、人口统计学特征、住院过程和短期结局；(2)比较非肠性溶血性尿毒综合征患者与同一临床地点诊断和治疗的肠性或腹泻相关溶血性尿毒综合征（D+ HUS）患儿同期队列的结果；(3)确定区分这两组的临床或实验室特征，并预测非肠病性溶血性尿毒综合征患者的疾病严重程度和短期预后。方法：从1997年至2001年筛选的D+ HUS患者中收集数据，这些患者参加了SYNSORB Pk （SYNSORB Biotech Inc ., Calgary, Alberta, Canada）的多中心试验，但由于缺乏腹泻前体症状而不符合条件。记录了以下特征：年龄；性;种族的;前驱症状;原因;血红蛋白、红细胞压积和血小板计数的最低值；透析的使用；以及住院时间。结果：247例溶血性尿毒综合征患儿中有27例为非肠病性溶血性尿毒综合征（11%）。24名患者（15名男孩，9名女孩）的医疗记录完整，可用于审查，包括研究队列。平均发病年龄为4.2±0.9 （SE）岁。肺炎链球菌感染9例（38%）。17例患者（71%）透析40+/-27天。住院时间中位数为22天（范围2至71天）。肺炎链球菌相关溶血性尿毒综合征患儿的住院时间比其他非肠病性溶血性尿毒综合征患儿长，但所有肺炎链球菌相关溶血性尿毒综合征患儿均恢复了肾功能。与参加SYNSORB Pk临床试验的D+溶血性尿毒综合征患者相比，非肠病性溶血性尿毒综合征患儿需要透析治疗的次数更多（24 / 17对145 / 59，P=0.025），住院时间更长（中位数，22天对9天，P=0.002）。结论：(1)非肠病性溶血性尿毒综合征的发生率约为D+溶血性尿毒综合征的十分之一；(2)与D+型溶血性尿毒综合征患者相比，非肠病性溶血性尿毒综合征患者更需要透析治疗，急性发作期间住院时间是D+型溶血性尿毒综合征患者的两倍以上；(3)肺炎链球菌感染占非肠病性溶血性尿毒综合征病例的近40%；(4)尽管肺炎链球菌相关性溶血性尿毒综合征的短期病程不如其他类型的非肠病性溶血性尿毒综合征或D+型溶血性尿毒综合征，但这些患者肾功能恢复的长期预后似乎较好。
Background: Nondiarrheal or Streptococcus pneumoniae-related hemolytic uremic syndrome (HUS) represents a heterogeneous group of disorders. This study was performed to: (1) describe the current incidence, causes, demographic features, hospital courses, and short-term outcomes of non-enteropathic HUS; (2) compare findings in patients with non-enteropathic HUS with those obtained from a contemporaneous cohort of children with enteropathic or diarrhea-associated HUS (D+ HUS) diagnosed and treated at the same clinical sites; and (3) identify clinical or laboratory features that differentiate these 2 groups and predict disease severity and the short-term outcome in patients with non-enteropathic HUS. Methods: Data were collected from patients screened between 1997 and 2001 for enrollment in a multicenter trial of SYNSORB Pk (SYNSORB Biotech Inc, Calgary, Alberta, Canada) in D+ HUS, but who were ineligible because of lack of a diarrhea prodrome. The following features were recorded: age; sex; ethnicity; prodromal symptoms; cause; nadir values for hemoglobin, hematocrit, and platelet count; use of dialysis; and length of hospitalization. Results: Twenty-seven of 247 children with HUS had non-enteropathic HUS (11%). Twenty-four patients (15 boys, 9 girls), whose medical records were complete and available for review, comprise the study cohort. Mean age at onset was 4.2+/-0.9 (SE) years. Infection caused by S pneumoniae was diagnosed in 9 patients (38%). Dialysis was performed in 17 patients (71%) for 40+/-27 days. Median length of hospitalization was 22 days (range, 2 to 71 days). Children with S pneumoniae-related HUS had a longer hospital stay than those with other causes of non-enteropathic HUS, but all patients with S pneumoniae-related HUS recovered kidney function. Dialysis therapy was required more often (17 of 24 versus 59 of 145 children; P=0.025) and hospital stays were longer (median, 22 versus 9 days; P=0.002) in children with non-enteropathic HUS compared with patients with D+ HUS who were enrolled in the SYNSORB Pk clinical trial. Conclusion: (1) The incidence of non-enteropathic HUS is approximately one tenth that of D+ HUS; (2) patients with non-enteropathic HUS require dialysis therapy more often and are hospitalized more than twice as long during the acute episode compared with those with D+ HUS; (3) infection caused by S pneumoniae accounts for nearly 40% of cases of non-enteropathic HUS; and (4) although S pneumoniae-related HUS is associated with a less favorable short-term course than other types of non-enteropathic HUS or D+ HUS, the long-term prognosis for recovery of renal function appears to be good in these patients.