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新生儿孤立性肝血管瘤并发心力衰竭、消耗性凝血病、微血管病性溶血性贫血和梗阻性黄疸。病例报告及文献复习。

Solitary hepatic hemangioma in a newborn infant complicated by cardiac failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. Case report and review of the literature.

作者信息

Linderkamp O, Höpner F, Klose H, Riegel K, Hecker W C

出版信息

Eur J Pediatr. 1976 Dec 9;124(1):23-9. doi: 10.1007/BF00452410.

Abstract

A newborn infant with a large hepatic hemangioma developed congestive heart failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. The patient was mildly heparinized (250 units per kg and day) and underwent successful resection of the tumor without lobectomy at the age of 3 days. Blood volume increased from 93.9 ml/kg at the age of 5 h to 124.2 ml/kg prior to surgery. Red-cell mass simultaneously decreased from 53.8 to 39.4 ml/kg. The increase of blood volume is explained by congestive heart failure, the decrease of red-cell mass by intravascular coagulation within the tumor resulting in formation of thrombi and microangiopathic hemolytic anemia. A review of the literature on infants with symptoms caused by an intrahepatic hemangioma during the first month of life confirms that surgical intervention is the treatment of choice for infants with giant solitary hemangioma of the liver.

摘要

一名患有巨大肝血管瘤的新生儿出现了充血性心力衰竭、消耗性凝血病、微血管病性溶血性贫血和梗阻性黄疸。该患者接受了轻度肝素化治疗(每千克体重每日250单位),并在3日龄时成功切除肿瘤,未进行肝叶切除术。血容量从出生5小时时的93.9毫升/千克增加到手术前的124.2毫升/千克。红细胞量同时从53.8毫升/千克降至39.4毫升/千克。血容量增加是由充血性心力衰竭导致的,红细胞量减少是由于肿瘤内血管内凝血形成血栓以及微血管病性溶血性贫血所致。对有关出生后第一个月内由肝内血管瘤引起症状的婴儿的文献综述证实,手术干预是患有巨大孤立性肝血管瘤婴儿的首选治疗方法。

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