Carlier J C, Martelli H, Ducot B, Alagille D
Chir Pediatr. 1982 May-Jun;23(3):167-70.
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.
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。