[Presinusoidal portal hypertension due to portal thrombosis in a patient with Alagille's syndrome]

V Lorenzo-Zúñiga; R M Morillas; R Planas; I Ojanguren; M Broggic; M A Alvarez; S Montoliu; M Rosinach; M Fuensanta; M A Gassull

doi:10.1016/s0210-5705(03)70359-8

[Presinusoidal portal hypertension due to portal thrombosis in a patient with Alagille's syndrome]

Gastroenterol Hepatol. 2003 May;26(5):294-6. doi: 10.1016/s0210-5705(03)70359-8.

[Article in Spanish]

Authors

V Lorenzo-Zúñiga¹, R M Morillas, R Planas, I Ojanguren, M Broggic, M A Alvarez, S Montoliu, M Rosinach, M Fuensanta, M A Gassull

Affiliation

¹ Servicio de Aparato Digestivo. Hospital Universitari Germans Trias i Pujol. Badalona. Barcelona. Spain.

PMID: 12732101
DOI: 10.1016/s0210-5705(03)70359-8

Abstract

We present the case of a 16-year old woman with Alagille's syndrome, who had upper gastrointestinal bleeding due to rupture of esophageal varices secondary to presinusoidal portal hypertension without liver fibrosis. Portal thrombosis is a manifestation previously unreported in association to this syndrome.

Publication types

Case Reports

MeSH terms

Adolescent
Alagille Syndrome / complications*
Alagille Syndrome / genetics
Calcium-Binding Proteins
Combined Modality Therapy
Esophageal and Gastric Varices / etiology
Esophageal and Gastric Varices / surgery
Esophageal and Gastric Varices / therapy
Female
Gastrointestinal Hemorrhage / etiology
Humans
Hypertension, Portal / etiology*
Intercellular Signaling Peptides and Proteins
Membrane Proteins
Portal Vein*
Portasystemic Shunt, Surgical
Primary Myelofibrosis / etiology
Proteins / genetics
Sclerotherapy
Serrate-Jagged Proteins
Splenectomy
Venous Thrombosis / etiology*

Substances

Calcium-Binding Proteins
Intercellular Signaling Peptides and Proteins
Membrane Proteins
Proteins
Serrate-Jagged Proteins