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溶血尿毒综合征的临床血液学特征

A clinico-hematological profile of hemolytic-uremic syndrome.

作者信息

Dayal R, Agarwal S, Prasad R, Mathur P P, Agarwal A, Anand A

机构信息

Department of Paediatrics, SN Medical College, Agra, India.

出版信息

Southeast Asian J Trop Med Public Health. 1993;24 Suppl 1:280-3.

PMID:7886599
Abstract

Hemolytic uremic syndrome (HUS) is defined as microangiopathic hemolytic anemia, thrombocytopenia and uremia. It is an important cause of acute renal failure (ARF) in children all over the world. The present study was carried out to assess the incidence, clinical presentation, hematological and biochemical profile of children presenting with HUS from 1987 to 1990. Out of the 100 cases who presented with ARF 22 had HUS. A majority of these children were males below 1 year of age, and had a prodromal phase of mainly gastrointestinal manifestations lasting for about a week. Anemia was a constant feature followed by bleeding diathesis, mainly melena and purpura. Neurological manifestations included altered sensorium, irritability, coma, hypertensive encephalopathy and convulsions. Renal problems mainly included oliguria, hypertension, hematuria and edema. Investigations revealed thrombocytopenia and microangiopathic hemolytic anemia in all cases. Evidence of disseminated intravascular coagulation (DIC) was observed in 3 cases as decreased fibrinogen levels, increased fibrinogen degradation products and deranged clotting studies. Blood biochemistry revealed azotemia in all cases, hyponatremia in 5 cases, hypernatremia in 3 cases and hyperkalemia in 12 cases. Stool culture showed the presence of Shigella in 8, E. coli in 6 and Klebsiella in 4 cases. Out of 22 cases of HUS, 15 were treated conservatively; of these 2 died. Both of these deaths were due to DIC 7 children were put on peritoneal dialysis; only 1 child died in this group. Factors affecting the outcome were duration of oliguria, levels of blood urea and presence of encephalopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

溶血尿毒综合征(HUS)的定义为微血管病性溶血性贫血、血小板减少症和尿毒症。它是全球儿童急性肾衰竭(ARF)的一个重要病因。本研究旨在评估1987年至1990年期间患溶血尿毒综合征儿童的发病率、临床表现、血液学和生化特征。在100例急性肾衰竭患儿中,22例患有溶血尿毒综合征。这些儿童大多数为1岁以下男性,有前驱期,主要表现为胃肠道症状,持续约一周。贫血是一个持续特征,其次是出血倾向,主要为黑便和紫癜。神经学表现包括意识改变、易怒、昏迷、高血压脑病和惊厥。肾脏问题主要包括少尿、高血压、血尿和水肿。检查发现所有病例均有血小板减少症和微血管病性溶血性贫血。3例观察到弥散性血管内凝血(DIC)证据,表现为纤维蛋白原水平降低、纤维蛋白原降解产物增加和凝血研究紊乱。血液生化检查显示所有病例均有氮质血症,5例低钠血症,3例高钠血症,12例高钾血症。粪便培养显示8例有志贺菌,6例有大肠杆菌,4例有克雷伯菌。22例溶血尿毒综合征患儿中,15例接受保守治疗,其中2例死亡。这2例死亡均因弥散性血管内凝血。7例儿童接受腹膜透析,该组仅1例死亡。影响预后的因素为少尿持续时间、血尿素水平和是否存在脑病。(摘要截选至250词)

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A case of hemolytic uremic syndrome associated with Epstein-Barr virus infection.一例与爱泼斯坦-巴尔病毒感染相关的溶血尿毒综合征病例。
Korean J Intern Med. 1998 Jul;13(2):131-5. doi: 10.3904/kjim.1998.13.2.131.