Oakes Robert S, Siegler Richard L, McReynolds Markham A, Pysher Theodore, Pavia Andrew T
Pediatric Nephrology, University of Utah School of Medicine, Salt Lake City, Utah, USA.
Pediatrics. 2006 May;117(5):1656-62. doi: 10.1542/peds.2005-0785.
Describe the cause of deaths among patients with postdiarrheal hemolytic uremic syndrome (HUS) and identify predictors of death at the time of hospital admission.
Case-control study of 17 deaths among patients with HUS identified from the Intermountain HUS Patient Registry (1970-2003) compared against all nonfatal cases.
Of the 17 total deaths, 15 died during the acute phase of disease. Two died because treatment was withdrawn based on their preexisting conditions, and 1 died because of iatrogenic cardiac tamponade; they were excluded from analysis. Brain involvement was the most common cause of death (8 of 12); congestive heart failure, pulmonary hemorrhage, and hyperkalemia were infrequent causes. Presence of prodromal lethargy, oligoanuria, or seizures and white blood cell count (WBC) >20 x 10(9)/L or hematocrit >23% on admission were predictive of death. In multivariate analysis, elevated WBC and elevated hematocrit were independent predictors. The combination of prodromal dehydration, oliguria, and lethargy and admission WBC values >20 x 10(9)/L and hematocrit >23% appeared in 7 of the 12 acute-phase deaths.
Diarrheal HUS patients presenting with oligoanuria, dehydration, WBC >20 x 10(9)/L, and hematocrit >23% are at substantial risk for fatal hemolytic uremic syndrome. Such individuals should be referred to pediatric tertiary care centers.
描述腹泻后溶血性尿毒症综合征(HUS)患者的死亡原因,并确定入院时的死亡预测因素。
对从山间HUS患者登记处(1970 - 2003年)确定的17例HUS患者死亡病例进行病例对照研究,并与所有非致命病例进行比较。
在17例死亡病例中,15例死于疾病急性期。2例因基于其原有病情而停止治疗死亡,1例因医源性心脏压塞死亡;这3例被排除在分析之外。脑受累是最常见的死亡原因(12例中的8例);充血性心力衰竭、肺出血和高钾血症是较少见的原因。前驱期嗜睡、少尿或惊厥以及入院时白细胞计数(WBC)>20×10⁹/L或血细胞比容>23%可预测死亡。在多变量分析中,白细胞计数升高和血细胞比容升高是独立的预测因素。前驱期脱水、少尿和嗜睡以及入院时白细胞计数>20×10⁹/L和血细胞比容>23%的组合出现在12例急性期死亡病例中的7例。
出现少尿、脱水、白细胞计数>20×10⁹/L和血细胞比容>23%的腹泻型HUS患者发生致命性溶血性尿毒症综合征的风险很高。此类患者应转诊至儿科三级护理中心。