A study on demographic and clinical profile of children with extra hepatic portal venous obstruction and with special reference to thrombophilic factors

Abstract

Background: Extra-hepatic portal vein obstruction (EHPVO) due to portal vein thrombosis is an important cause of portal hypertension in several region including India. The cause of thrombosis in these patients remains unclear. Objective of the study was to study the demographic features, etiology, clinical, laboratory findings with special reference to thrombophilic factors like protein C, protein S and antithrombin III deficiency in children with EHPVO.Methods: The prospective analysis of 62 patients of EHPVO (<14 years of age) was done in the Department of Hepatology, SCB medical College, Cuttack. After detailed history, clinical examination, Ultrasound abdomen /color Doppler and Upper GI endoscopy, the subjects were analyzed for any deficiency of thrombophilic factors like protein C, protein S and antithrombin III.Results: A total of 62 patients (37 Male, 25 Female) with mean age of 8.3+3.1 years were studied. Growth retardation was present in the form of wasting (alone) 20.9%, stunting (alone) 25.8% and both wasting and stunting was found in 9.8% cases. History of neonatal, umbilical sepsis and umbilical vein catheterization was found in 15.9% and 10.2% of cases respectively. Haemorrhage from oesophageal varices was prevalent symptoms in 85.9% patients. Splenomegaly was found in 91.9% patients and ascites in 9.4% patients. 47 patients studied for protein C, S and antithromibin III. 14 patients were found to have thrombophilia: protein C deficiency in 9, protein S deficiency in 8, Antithrombin III deficiency in 6.Conclusions: The etiology of EHPVO in the majority of patients remain still unclear. It is commonly associated impaired somatic growth. The risk of EHPVO increases in the presence of thrombophilia, resulting from deficiency of naturally occurring anticoagulant proteins like Protein C, Protein S and Antithrombin III.

A Study on Antithromin III Deficiency in Children with Extrahepatic Portal Hypertension Attending a Tertiary Care Hospital in Bangladesh

“…Table X showed that no significant association was observed between EHPVO and non EHPVO for antithrombin III deficiency. However, the odds for EHPVO due to low antithrombin III level were 12.25 (95% CI: 17,18 The wide variation in the frequencies of antithrombin III deficiency implies the impact of geographic and ethnic variations among patients as the mentioned studies were done in different parts of the world like Denmark and India respectively.…”

Surgical outcome of extrahepatic portal venous obstruction: Audit from a tertiary referral centre in Eastern India

“…Liver function is mostly well preserved, unless compromised by reduced hepatic cell mass and synthetic dysfunction, as rarely seen with advanced age, prolonged duration of disease, and development of SBC from PB [7]. Neonatal exchange transfusion through umbilical catheterisation, omphalitis, congenital abnormalities, childhood sepsis, and prothrombotic disorders, like deficiencies in the naturally occur-ring anticoagulants (protein c, protein s, and antithrombin III) and factor V leiden and prothrombin gene mutations, are some of the suspected reasons [2].…”

“…Extra hepatic portal venous obstruction (EHPVO) is the most common cause of non-cirrhotic portal hypertension (NCPH) in Indian children, particularly affecting lower socio-economic strata [1]. Neonatal and childhood septic conditions, and prothrombotic disorders are some of the suspected reasons [2]. Upper gastrointestinal (GI) bleeding is most common presentation of EHPVO.…”