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24例溶血尿毒综合征患儿的临床病理研究。西南儿科肾脏病研究组报告。

A clinicopathological study of 24 children with hemolytic uremic syndrome. A report of the Southwest Pediatric Nephrology Study Group.

作者信息

Argyle J C, Hogg R J, Pysher T J, Silva F G, Siegler R L

机构信息

University of Texas Southwestern Medical Center, Dallas.

出版信息

Pediatr Nephrol. 1990 Jan;4(1):52-8. doi: 10.1007/BF00858440.

Abstract

This study reports the pattern of renal injury in 24 North American children with hemolytic uremic syndrome (HUS), and the extent of extrarenal involvement in 9 of these children examined at autopsy. Fifteen of the 24 children were studied during the first 16 days of hospitalization; their renal specimens demonstrated glomerular thrombotic microangiopathy (TMA) in 8 children, cortical necrosis in 1, and varying degrees of glomerular TMA and cortical necrosis in 6 children. Nine of the children were studied after 16 or more days of hospitalization; these patients had prominent renal arterial lesions. Of 9 children examined at autopsy, extrarenal microthrombi were identified in 8. In 4 children who died during the acute phase of the disease, hemorrhagic colonic necrosis (3 children) and pancreatic islet cell necrosis (2 children) were the principal extrarenal lesions encountered. Rare microthrombi were present in the brains of the 3 children who manifested seizures.

摘要

本研究报告了24例北美溶血尿毒综合征(HUS)患儿的肾损伤模式,以及对其中9例患儿进行尸检时肾外受累的程度。24例患儿中有15例在住院的前16天接受了研究;他们的肾脏标本显示,8例患儿出现肾小球血栓性微血管病(TMA),1例出现皮质坏死,6例出现不同程度的肾小球TMA和皮质坏死。9例患儿在住院16天或更长时间后接受了研究;这些患者有明显的肾动脉病变。在接受尸检的9例患儿中,8例发现有肾外微血栓。在疾病急性期死亡的4例患儿中,主要的肾外病变是出血性结肠坏死(3例)和胰岛细胞坏死(2例)。3例出现癫痫发作的患儿大脑中存在罕见的微血栓。

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