Arora N K, Lodha R, Gulati S, Gupta A K, Mathur P, Joshi M S, Arora N, Mitra D K
Department of Paediatrics, All India Institute of Medical Sciences, New Delhi.
Indian J Pediatr. 1998 Jul-Aug;65(4):585-91. doi: 10.1007/BF02730901.
Etiological factors associated with portal hypertension in children influence the decision about therapy and the prognosis. This cross-sectional observational study was performed at a tertiary care centre in northern India from January, 1990 to December, 1994. Children below the age of 14 years with suspected portal hypertension were prospectively assembled into a cohort to determine the etiology and clinical profile of portal hypertension. Of the 115 patients with portal hypertension, 76.5% had extrahepatic portal hypertension (EHPH). Remaining 23.5% of the cases had intrahepatic and post-hepatic causes of portal hypertension. Children with EHPH had a significantly earlier onset of symptoms as compared to those with intrahepatic portal hypertension (p = 0.002) and bled significantly more frequently (p = 0.00). Forty per cent of patients with chronic liver disease (CLD) never had jaundice. History suggestive of potential etiological factors could be elicited in only 7% of EHPH patients. The commonest site of block in splenoportal axis was at the formation of the portal vein. An inverse relation of bleeding rates with duration of illness was seen in EHPH. Of the 10 CLD patients in whom liver biopsy could be done, cirrhosis was present in 6 patients. Understanding the natural history of EHPH and portal hypertension due to other etiologies may have significant implications in choosing the appropriate intervention and predicting the outcome.
与儿童门静脉高压相关的病因会影响治疗决策和预后。这项横断面观察性研究于1990年1月至1994年12月在印度北部的一家三级医疗中心进行。对14岁以下疑似门静脉高压的儿童进行前瞻性队列研究,以确定门静脉高压的病因和临床特征。在115例门静脉高压患者中,76.5%患有肝外门静脉高压(EHPH)。其余23.5%的病例有肝内和肝后性门静脉高压病因。与肝内门静脉高压患儿相比,EHPH患儿症状出现明显更早(p = 0.002),出血频率也明显更高(p = 0.00)。40%的慢性肝病(CLD)患者从未出现黄疸。仅7%的EHPH患者能引出提示潜在病因的病史。脾门静脉轴阻塞最常见的部位是门静脉形成处。在EHPH中观察到出血率与病程呈负相关。在10例可进行肝活检的CLD患者中,6例存在肝硬化。了解EHPH和其他病因所致门静脉高压的自然史可能对选择合适的干预措施和预测预后具有重要意义。