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青少年溶血性尿毒症综合征

Hemolytic-uremic syndrome in adolescents.

作者信息

Siegler R L, Pavia A T, Cook J B

机构信息

Division of Nephrology, University of Utah School of Medicine, Salt Lake City, USA.

出版信息

Arch Pediatr Adolesc Med. 1997 Feb;151(2):165-9. doi: 10.1001/archpedi.1997.02170390055010.

Abstract

OBJECTIVE

To compare the epidemiological characteristics, clinical features, and outcome of adolescents with hemolytic-uremic syndrome (HUS) with those of children with HUS.

DESIGN

A retrospective descriptive study using data stored in the computerized Utah HUS registry.

SETTING

The HUS registry contains data on postdiarrheal and nondiarrheal HUS cases since 1970 in which the patients were younger than 18 years of age at the time of diagnosis and includes virtually all Utah cases as well as those referred from surrounding states.

PATIENTS

Seventeen adolescents (age, 12-17 years) and 276 younger patients from September 30, 1970, through December 5, 1993, who met the diagnostic criteria for HUS.

MAIN OUTCOME MEASURES

Age, sex, seasonality, prodromal features (eg, antecedent diarrhea), laboratory values, hospital course, outcome, and chronic sequelae.

RESULTS

The 17 adolescent patients, who composed 5.8% of the study population, experienced a course of the disease that was similar to that of the younger patients. Diarrhea preceded HUS in approximately 90% of the patients in both groups. Laboratory values were similar in teenagers and younger patients. The hospital courses were also similar; seizures occurred in almost 20%, and hypertension and oligoanuric renal failure occurred in most. Two (12%) of the teenagers and 7 (2.4%) of the younger patients died during the acute phase of the syndrome (P = .09); almost 50% of both groups experienced 1 or more chronic renal sequelae. End-stage renal disease has occurred in 1 (5.8%) of the teenagers and 6 (2.2%) of the children. At follow-up, 1 or more years (median, 5 years) after the onset of HUS, hypertension was present in 22% of the teenagers and 6.7% of the preteens (P = .14). A below-normal glomerular filtration rate was seen in approximately 30% of both groups; proteinuria was noted in approximately 25% of both groups. Approximately 10% of both groups had a combination of proteinuria and a low glomerular filtration rate and are, therefore, at risk for eventual end-stage renal disease.

CONCLUSIONS

In our region of the Intermountain West, HUS in adolescents closely resembles that seen in children and the outcome is more favorable than that experienced by adults.

摘要

目的

比较青少年溶血尿毒综合征(HUS)与儿童HUS的流行病学特征、临床特点及转归。

设计

一项回顾性描述性研究,使用存储在犹他州HUS计算机登记系统中的数据。

背景

该HUS登记系统包含自1970年以来腹泻后及非腹泻后HUS病例的数据,这些病例诊断时年龄小于18岁,几乎涵盖了犹他州所有病例以及周边州转诊的病例。

患者

1970年9月30日至1993年12月5日期间,17例青少年(年龄12 - 17岁)和276例年龄较小的患者,均符合HUS诊断标准。

主要观察指标

年龄、性别、季节性、前驱特征(如前期腹泻)、实验室检查值、住院病程、转归及慢性后遗症。

结果

17例青少年患者占研究人群的5.8%,其疾病病程与年龄较小患者相似。两组中约90%的患者HUS发病前有腹泻。青少年和年龄较小患者的实验室检查值相似。住院病程也相似;近20%的患者发生惊厥,大多数患者出现高血压和少尿性肾衰竭。17例青少年中有2例(12%)、276例年龄较小患者中有7例(2.4%)在综合征急性期死亡(P = 0.09);两组中近50%的患者有1种或更多慢性肾脏后遗症。青少年中有1例(5.8%)、儿童中有6例(2.2%)发生终末期肾病。在HUS发病1年或更长时间(中位时间5年)后的随访中,22%的青少年和6.7%的儿童期前青少年存在高血压(P = 0.14)。两组中约30%的患者肾小球滤过率低于正常;两组中约25%的患者有蛋白尿。两组中约10%的患者既有蛋白尿又有低肾小球滤过率,因此有发生终末期肾病的风险。

结论

在我们美国西部山间地区,青少年HUS与儿童HUS非常相似,且转归比成人更有利。

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