Non-Cirrhotic Portal Hypertension in Children -A Report of Six Cases-

孩童非硬化性肝門脈高血壓症－六病例報告－

江文山(Man-Shan Kong) ； 張美惠(Mei-Hwei Chang) ； 李鍾祥(Chung-Hsiang Lee) ； 陳秋江(Chiu-Chiang Chen) ； 呂鴻基(Hung-Chi Lue) ； 許輝吉(Hey-Chi Hsu) ； 徐劍耀(Chien-Yao Hsu) ； 陳維昭(Wei-Jao Chen)

《Acta Paediatrica Sinica》 27卷3期 (1986/06) Pp. 260-270

摘要

非硬化性肝門脈高血壓症乃一成人及小孩少見的疾病，此疾病常較肝硬化有較好的預後，本篇報告6例兒童非硬化性肝門脈高血壓症病例，年齡在第一次住院時為3到13歲，3例男孩診斷為肝門靜脈栓塞病例，一男性為先天性肝臟纖維化，2例女性，一為巴德奇亞利氏症，1例為部份肝臟結節轉化病例，肝臟結節轉化症十分罕見，少於10例曾發表在文獻上。
食道靜脈瘤出血及脾臟腫大為最常見的臨床症狀，五位病人追蹤的時間由3個月到6年不等，2例接受外科血液分流手術的病人死亡，死因1例為腸阻塞合併壞死，另1例為腹內感染，3例在追踪3到6年後仍健在，但1例仍有食道出血現象。

並列摘要

Non cirrhotic portal hypertension is not common in either children or adults. It usually has a more optimistic prognosis than portal hypertension in cirrhosis of the liver. Six children, aged 3 to 13 years at their first visit, with non-cirrhotic portal hypertension are reported here. There were three boys with portal vein thrombosis, one boy with congenital hepatic fibrosis, one girl with Budd-chiari syndrome, and one girl with partial nodular transformation of the liver. Partial nodular transformation of the liver is very rare. Less than 10 cases has been reported in the English literature.
Esophageal varies with bleeding and splenomegaly were the most common presenting features. Five patients were followed for three months to six years. Two patients died: one from strangulation of the bowels and, the other from intraabdominal infection after portocaval shunt. Three are still living after three to six year follow up. Esophageal varices with bleeding is still a problem in one of them.

並列關鍵字

non-cirrhotic portal hypertension ； portal vein thrombosis ； congenital hepatic fibrosis ； partial nodular transformation of the liver