Haemolytic uraemic syndrome: prognostic factors in children over 3 years of age
Haemolytic uraemic syndrome: prognostic factors in children over 3 years of age
复制标题
溶血性尿毒综合征：3岁以上儿童的预后因素
DOI：
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发表时间：
1995
期刊：
影响因子：
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通讯作者：
Renée Habib
中科院分区：
文献类型：
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作者：
C. Renaud;P. Niaudet;M. Gagnadoux;M. Broyer;Renée Habib
以往的研究表明，原发性溶血性尿毒综合征（HUS）的发病年龄是一个具有预后意义的特征，3岁以下的儿童患者的预后比年龄较大的儿童好得多。为了解释这种差异，我们分析了1955年至1990年间在我科就诊的42例3岁以上HUS患儿的临床和病理特征。根据前驱腹泻的存在，我们将患者分为两组：21名儿童表现为腹泻相关（典型或D+）形式的HUS，而21名儿童表现为非腹泻相关（非典型或D-）形式。在42名儿童中，20名（47.5%）进展为终末期肾衰竭。然而，我们的研究表明，发病年龄本身并不是HUS的预后特征。儿童和婴儿之间结果的差异很可能与3岁以上儿童中HUS非典型亚群的高发病率有关，这一亚群在婴儿中非常罕见。这种疾病的不祥特征是：（1）没有肾前驱症状，（2）尿量正常，（3）明显的蛋白尿，（4）高血压，（5）复发或复发的发生，（6）肾活检时出现广泛和严重的小动脉改变。非典型形式的溶血尿毒综合征预后差，因此在病程中应尽早使用新鲜冷冻血浆输注和/或血浆置换。
Previous studies have shown that age at onset of primary haemolytic uraemic syndrome (HUS) is a feature of prognostic significance, the disease being of much better outcome in paediatric patients younger than 3 years than in older children. In an attempt to find an explanation for such a difference, we analysed the clinical and pathological features of 42 children over 3 years of age who presented with HUS between 1955 and 1990 in our department. On the basis of the presence of a prodromal diarrhoea, we divided our patients into two groups: 21 children presented with the diarrhoea-associated (typical or D+) form of HUS, whereas 21 had the non-diarrhoea-associated (atypical or D-) form. Of the 42 children, 20 (47.5%) progressed to end-stage renal failure. However, our study shows that age at onset of HUS is not a prognostic feature per se. The difference in outcome between children and infants is most likely related to the high incidence of the atypical subset of HUS in children over 3 years, a subset that is very uncommon in infants. The ominous features which characterise this form of the disease are: (1) the absence of a diarrhoeal prodrome, (2) normal urine output, (3) marked proteinuria, (4) hypertension, (5) the occurrence of relapses or recurrences and (6) the presence of widespread and severe arteriolar changes on renal biopsy. The poor prognosis of the atypical form of HUS warrants the use of fresh-frozen plasma infusions and/or plasma exchange as early as possible in the course of the disease.
影响因子：
158.5
作者：
BELL, WR;BRAINE, HG;KICKLER, TS
通讯作者：
KICKLER, TS