SciELO - Scientific Electronic Library Online

 
vol.35 número1Hepatectomía en dos tiempos para las metástasis hepáticas de origen colorrectalNeurofibromatosis orbitotemporal gigante índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Servicios Personalizados

Revista

Articulo

Links relacionados

Compartir


Revista Médica del Uruguay

versión impresa ISSN 0303-3295versión On-line ISSN 1688-0390

Resumen

FERREIRA, Joaquín et al. Budd-Chiari syndrome associated to antiphospholipid syndrome. Rev. Méd. Urug. [online]. 2019, vol.35, n.1, pp.150-166.  Epub 01-Mar-2019. ISSN 0303-3295.  https://doi.org/10.29193/rmu.35.8.

Introduction:

Budd-Chiari is a rare condition characterized by the occlusion of the hepatic vein flow out. It is frequently associated to a pro-thrombotic state. Diagnosis and treatment constitute a challenge for clinical doctors. This study aims to report a clinical case of Budd-Chiari syndrome secondary to an antiphospholipid syndrome, a rather unusual association in literature.

Clinical case:

31 year old woman, with 6 years of evolution ascites, evidencing a general affection in the last year. Clinical examination: lucid, universal hyperbilirubinemia (jaundice), painful hepatomegaly, splenomegaly and tension ascites. Laboratory tests: hepatogram with cholestatic pattern, cholinesterase, and reduced prothrombin rate. Abdominal Doppler revealed: severe ascites, irregular hepatomegaly, increased echogenicity. Splenomegaly. Occlusion of the middle suprahepatic vein, narrowing of the right suprahepatic vein. Llow speed scarcely phasic flow, inverse in a few branches, hepatofugal. Fibrogastroduodenoscopy: esophagic varices grade III. Ascites secondary to portal hypertension was suspected, probable Budd Chiari syndrome. Antibodies IgM â2glicoprotein 1 were positive. Diagnosis of antiphospholipid syndrome. Liver transplant was performed with a good evolution of the patient.

Discussion and conclusions:

Budd-Chiari syndrome is more frequent in women between 35 and 50 years old. It needs to be regarded as a cause of liver conditions, in particular when there is no other evident cause. Hematologic diseases are the prothrombotic states more frequently associated to Budd-Chiari syndrome in the western world. Treatment must be based on the clinical presentation. Liver transplant is a rescue/salvage measure when all other treatments fail.

Palabras clave : Budd-Chiari Syndrome; Antibodies, antiphospholipid; Case reports.

        · resumen en Español | Portugués     · texto en Español     · Español ( pdf )