Budd-Chiari syndrome in children: Report of 22 cases

Gentil-Kocher, S.; Bernard, Olivier; Brunelle, Françis; Hadchouel, Michelle; Maillard, J N; Valayer, J; Hay, Jean‐Marie; Alagille, D

doi:10.1016/s0022-3476(88)80524-9

Cited by 91 publications

(75 citation statements)

References 36 publications

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“…Patients with HVCS often have associated endophlebitis of medium-sized HVs and PVs. The associated ischemic liver damage eventually results in development of veno-portal type of cirrhosis (9)(10)(11). Development of LC within 3-9 months of liver biopsy for acute diseases has been documented in patients with sinusoidal obstruction syndrome (SOS) (12, 13), Budd-Chiari syndrome (BCS) (14), and HVCS (11).…”

Section: Liver Damage In Sinusoidal Hypertensionmentioning

confidence: 99%

Hepatic Venous Outflow Obstruction: Suggestion of a New Classification

Shrestha¹

2017

jrenhep

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Hepatic venous outflow obstruction (HVOO) is common in developing countries. It is a serious condition that clinically manifests with ascites from sinusoidal hypertension, and carries the risk of high mortality or development of liver cirrhosis. In the past, the eponym Budd-Chiari Syndrome (BCS) was used synonymously for HVOO. In the West, hepatic vein (HV) thrombosis caused by prothrombotic disorders is the main cause of HVOO. In the East, obliterative disease of hepatic portion of inferior vena cava induced by bacterial infection, now renamed hepatic vena cava syndrome, is the common cause of HVOO. These two diseases with different etiology, epidemiology, and natural history are at present grouped together under BCS causing much confusion. Sinusoidal obstruction syndrome, another important cause of HVOO at the level of the sinusoid and terminal HV, was left out in the classification of HVOO. In this article, the pathophysiology of sinusoidal hypertension is described, the term BCS is redefined, and a new classification of HVOO is suggested.

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Section: Liver Damage In Sinusoidal Hypertensionmentioning

confidence: 99%

Hepatic Venous Outflow Obstruction: Suggestion of a New Classification

Shrestha¹

2017

jrenhep

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“…El caso que se comenta muestra algunas variaciones respecto al cuadro tipico de adultos, pues no tenia dolor abdominal y no se identifico causa o factores predisponentes, en concordancia con lo descrito en otra serie de 22 nifios, todos los cuales teni'an hepatomegalia, 13 (59%) ascitis, 7 (32%) compromise de talla y peso, pero solo 2 (9%) dolor abdominal 14 .…”

Section: Comentariounclassified

“…El estudio flebografico permite identificar la obstruccion de la vena inferior y de las venas suprahepaticas y observar la circulacion colateral, habitualmente presente, pero no muestra la extension de la trombosis. La ecografia puede ser de gran ayuda, mostrando la Iesi6n venosa que compromete las venas suprahepa"-ticas y la extension de esta 1 ' 4 ' 13 , siendo considerada por algunos como el metodo inicial mas util, por revelar alteraciones virtualmente en todos los casos 14 . La cintigrafia es otra te"cnica diagnostica que puede ser de utilidad en SBC.…”

Section: Comentariounclassified

“…La biopsia de hi'gado constituye uno de los pilares en el diagnostico del SBC 2 ' 3 ' 6 " 8 ' 14 , donde los hallazgos mas caracten'sticos son gran congestion pasiva, sinusoides dilatados y atrofia celular en las zonas centrolobulillares. Necrosis, fibrosis e inflamacion son poco frecuentes, aunque pacientes con largo tiempo de evolution pueden tener acentuada fibrosis a semejanza de lo que ocurre en la cirrosis cardi'aca, lo que dificulta el diagnostico.…”

Section: Comentariounclassified

“…No hemos encontrado otras descripciones de quilotorax y quiloascitis en casos similares. El transplante hepatico tambien ha sido propuesto como posibilidad de tratamiento, pero no ha sido aun bien evaluada 14 ' 24 .…”

Section: Comentariounclassified

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