Aetiology and Management of Extrahepatic Portal Vein Obstruction in Children: King's College Hospital Experience

El-hamid, Nehal Abd; Taylor, Rachel M; Marinello, Daniela; Mufti, G J; Patel, Raj; Mieli‐Vergani, Giorgina; Davenport, Mark; Dhawan, Anil

doi:10.1097/mpg.0b013e31817b6eea

Cited by 79 publications

(72 citation statements)

References 34 publications

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“…As observed in the present study, PVT does not show gender predominance and its more common initial manifestations in children are upper gastrointestinal bleeding and splenomegaly, which corroborates results reported in the literature (1,6,41,47,50,53) . In the present casuistic, upper gastrointestinal bleeding was the main initial clinical manifestation in 52.7% of patients, followed by splenomegaly in 36.4%.…”

Section: Discussionsupporting

confidence: 81%

“…Approximately 79% of the children diagnosed with PVT will show at least one episode of upper gastrointestinal bleeding during their lives (2,41) . In the literature there are few reports on PVT in children and adolescents, being some of them also studies about casuistic descriptions and others about specific characteristics of the disease as thrombophilia (1,17,20,32,43,45,48,53,54,55) . Due to the scarcity of studies in the pediatric age group and the importance of the theme, this study`s objective is to describe the clinical characteristics, diagnosis, evolution and treatment of the portal hypertension in children and adolescents with diagnosis of portal vein thrombosis assisted at the Pediatric Hepatology Clinic of the Hospital das Clínicas of Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.…”

Section: Introductionmentioning

confidence: 99%

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Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

Ferri

Ferreira

Fagundes

et al. 2012

Arq. Gastroenterol.

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-Context -Portal vein thrombosis refers to a total or partial obstruction of the blood flow in this vein due to a thrombus formation. It is an important cause of portal hypertension in the pediatric age group with high morbidity rates due to its main complication -the upper gastrointestinal bleeding. Objective -To describe a group of patients with portal vein thrombosis without associated hepatic disease of the Pediatric Hepatology Clinic of the Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil with emphasis on diagnosis, presentation form and clinical complications, and the treatment of portal hypertension. Methods -This is a descriptive study of a series of children and adolescents cases assisted from January 1990 to December 2010. The portal vein thrombosis diagnosis was established by ultrasound. Results -Of the 55 studied patients, 30 (54.5%) were male. In 29 patients (52.7%), none of the risk factors for portal vein thrombosis was observed. The predominant form of presentation was the upper gastrointestinal bleeding (52.7%). In 20 patients (36.4%), the initial manifestation was splenomegaly. During the whole following period of the study, 39 patients (70.9%) showed at least one episode of upper gastrointestinal bleeding. The mean age of patients in the first episode was 4.6 ± 3.4 years old. The endoscopic procedure carried out in the urgency or electively for search of esophageal varices showed its presence in 84.9% of the evaluated patients. The prophylactic endoscopic treatment was performed with endoscopic band ligation of varices in 31.3% of patients. Only one died due to refractory bleeding. Conclusions -The portal vein thrombosis is one of the most important causes of upper gastrointestinal bleeding in children. In all non febrile children with splenomegaly and/or hematemesis and without hepatomegaly and with normal hepatic function tests, it should be suspect of portal vein thrombosis. Thus, an appropriate diagnostic and treatment approach is desirable in an attempt to reduce morbidity and mortality.

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Section: Discussionsupporting

confidence: 81%

Section: Introductionmentioning

confidence: 99%

Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

Ferri

Ferreira

Fagundes

et al. 2012

Arq. Gastroenterol.

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“…Protein C activity was low in 20% patients; protein S was low in 2 patients. 16 Antithrombin was normal factor V Leiden mutation were absent in all cases. According to study by Yaccha et al from India, out of 19 children with portal vein thrombosis they had studied alone Protein C deficiency was found in 5 children and combined protein C & elevated anticardiolipin antibody in 3 children.…”

Section: Resultsmentioning

confidence: 99%

“…28 According to Sharma et al factor V leiden and prothrombin gene mutations are infrequent in Indian patients with EHPVO. 16 Out study showed significant deficiency of protein C, protein S and antithrombin III 19.1%, 17% and 12.7% patients respectively out of total 47 patients studied for this.…”

Section: Resultsmentioning

confidence: 99%

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

Jena

Mohanty

Patra

et al. 2017

Int J Community Med Public Health

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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.

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Therapeutic Upper GI Endoscopy

Gershman

Vargas

Wyllie

et al. 2011

Practical Pediatric Gastrointestinal Endoscopy

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Aetiology and Management of Extrahepatic Portal Vein Obstruction in Children: King's College Hospital Experience

Abstract: The aetiology of EHPVO in the majority of patients remains unknown. Sclerotherapy and banding are effective treatments for bleeding varices with good long-term outcome. Procoagulant state is an infrequent cause of EHPVO in children.

Cited by 79 publications

References 34 publications

Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

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

Therapeutic Upper GI Endoscopy

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