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1.
Clin Res Hepatol Gastroenterol ; 44(4): 491-496, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32819872

RESUMO

Chronic non cirrhotic extrahepatic portal vein obstruction (EHPVO) refers to the cavernomatous transformation of the portal vein (the so-called "portal cavernoma") which occurs following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic [1]. Risk factors and associated diseases should be investigated (chapter 1). Indeed, the presence of a thrombophilic alteration, in particular myeloproliferative neoplasm impacts prognosis and determine a causal treatment.


Assuntos
Hipertensão Portal/diagnóstico , Hipertensão Portal/terapia , Veia Porta/anormalidades , Doença Crônica , Humanos , Guias de Prática Clínica como Assunto
14.
Clin Res Hepatol Gastroenterol ; 44(4): 480-485, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32253162

RESUMO

Sinusoidal obstruction syndrome (SOS), previously known as veno-occlusive disease, is characterized by concentric and non-thrombotic obstruction of the sinusoid and central vein lumen with no identified primitive or thrombotic hepatic vein lesions. The initial lesion is a result of endothelial denudation, corresponding to the migration of damaged sinusoidal cells to the central veins of the hepatic lobules, leading to sinusoidal and veno-occlusive congestive obstruction. SOS may be associated with other lesions such as centrilobular perisinusoidal fibrosis, peliosis, or nodular regenerative hyperplasia. The first cases of SOS were documented in 1920 in South Africa, after ingestion of food sources contaminated by pyrrolizidine alkaloids. SOS is a well-known complication of hematopoietic stem cell transplantation (HSCT). Numerous toxins and drugs have been associated with SOS, mainly chemotherapies and immunosuppressive therapies, as well as total body or liver irradiation and ABO mismatch platelet transfusion. The pathogenesis of this entity remains unknown.


Assuntos
Hepatopatia Veno-Oclusiva/diagnóstico , Hepatopatia Veno-Oclusiva/terapia , Humanos , Guias de Prática Clínica como Assunto
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