Proesmans W
Department of Pediatrics, University Hospital, University of Leuven, Belgium.
Kidney Blood Press Res. 1996;19(3-4):205-8. doi: 10.1159/000174075.
The hemolytic uremic syndrome is the most frequent cause of acute renal failure in childhood. In the vast majority of patients, the syndrome of acute hemolysis, thrombopenia and renal dysfunction is preceded by an episode of diarrhea with or without bloody stools. This colitis is caused by different strains of Escherichia coli which produce shiga like toxins. These toxins are responsible for both hemolysis and renal disease. There are good reasons for distinguishing patients with D (+) HUS and those without prodromal diarrhea [D (-) HUS], especially since the outcome in the latter group is less predictable and on average fairly unfavorable. D (+) HUS has also been labeled 'typical HUS' and D (-) HUS as 'atypical HUS'. This has led to some oversimplification, in that atypical has become synonymous with poor outcome. Our experience comprises 20 D (-) HUS patients, of whom 14 did extremely well. Scrutinizing the data of our patients lead to the conclusion that, within the D (-) group, some have a 'typical course' and display complete cure whereas those with an 'atypical course' either die or have severe sequelae.
溶血尿毒综合征是儿童急性肾衰竭最常见的病因。绝大多数患者在急性溶血、血小板减少和肾功能不全综合征出现之前，会有一次伴有或不伴有血便的腹泻发作。这种结肠炎是由产生志贺样毒素的不同大肠杆菌菌株引起的。这些毒素导致溶血和肾脏疾病。区分D（+）溶血尿毒综合征患者和无前驱性腹泻的患者[D（-）溶血尿毒综合征]是有充分理由的，特别是因为后一组患者的预后较难预测且平均相当不利。D（+）溶血尿毒综合征也被称为“典型溶血尿毒综合征”，D（-）溶血尿毒综合征被称为“非典型溶血尿毒综合征”。这导致了一些过度简化，即非典型已成为预后不良的同义词。我们的经验包括20例D（-）溶血尿毒综合征患者，其中14例恢复得非常好。仔细研究我们患者的数据得出的结论是，在D（-）组中，一些患者有“典型病程”并完全治愈，而那些有“非典型病程”的患者要么死亡要么有严重后遗症。
