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比塞特尔医院门静脉高压症患儿的医学问题。282例研究(1954 - 1981年)(作者译)

[Medical aspects of children with portal hypertension at Bicêtre hospital. A study of 282 cases (1954-1981) (author's transl)].

作者信息

Carlier J C, Martelli H, Ducot B, Alagille D

出版信息

Chir Pediatr. 1982 May-Jun;23(3):167-70.

PMID:6979402
Abstract

282 children with portal hypertension (PHT) were studied. PHT was due to extra-hepatic portal venous obstruction in 40% of children, to intra-hepatic disease in 56% and to post-sinusoídal lesions in 4%. Presenting symptoms, and risk of gastro-intestinal bleeding were analyzed in each etiologic group, as well as the follow-up in children in whom a portal-systemic shunt was not carried out. In portal venous obstruction, gastro-intestinal bleeding occurred frequently and early in life; liver failure and portal systemic encephalopathy did not occur; none of these children died except those with severe congenital heart disease. In children with cirrhosis, gastro-intestinal bleeding occurred twice less frequently and its date depended upon the etiology of cirrhosis; in this group, death was due twice more often to the underlying liver disease than to gastro-intestinal bleeding. In supra-hepatic PHT, the risks of PHT proper are limited except for ascites.

摘要

对282例门静脉高压(PHT)患儿进行了研究。40%的患儿PHT是由肝外门静脉阻塞引起,56%由肝内疾病引起,4%由窦后病变引起。分析了各病因组的临床表现、胃肠道出血风险,以及未进行门体分流术患儿的随访情况。在门静脉阻塞中,胃肠道出血在生命早期频繁发生;未发生肝衰竭和门体性脑病;除患有严重先天性心脏病的患儿外,这些患儿均未死亡。在肝硬化患儿中,胃肠道出血的发生率低两倍,其发生时间取决于肝硬化的病因;在该组中,死于潜在肝脏疾病的频率是死于胃肠道出血的两倍。在肝上型PHT中,除腹水外,PHT本身的风险有限。

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