[Presinusoidal portal hypertension due to portal thrombosis in a patient with Alagille's syndrome].
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.
Servicio de Aparato Digestivo. Hospital Universitari Germans Trias i Pujol. Badalona. Barcelona. Spain.
V
V
Lorenzo-Zúñiga
R M
RM
Morillas
R
R
Planas
I
I
Ojanguren
M
M
Broggic
M A
MA
Alvarez
S
S
Montoliu
M
M
Rosinach
M
M
Fuensanta
M A
MA
Gassull
D000293:Adolescent; D016738:Alagille Syndrome / Q000150:complications* / Q000235:genetics; D002135:Calcium-Binding Proteins; D003131:Combined Modality Therapy; D004932:Esophageal and Gastric Varices / Q000209:etiology / Q000601:surgery / Q000628:therapy; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology*; D036341:Intercellular Signaling Peptides and Proteins; D008565:Membrane Proteins; D011169:Portal Vein; D011170:Portasystemic Shunt, Surgical; D055728:Primary Myelofibrosis / Q000209:etiology; D011506:Proteins / Q000235:genetics; D015911:Sclerotherapy; D000072099:Serrate-Jagged Proteins; D013156:Splenectomy; D020246:Venous Thrombosis / Q000209:etiology*
D002135:Calcium-Binding Proteins; D036341:Intercellular Signaling Peptides and Proteins; D008565:Membrane Proteins; D011506:Proteins; D000072099:Serrate-Jagged Proteins