Budd-Chiari Syndrome in a Boy With β Thalassemia Major (A Study of a New Case)

Guirat-Dhouib, Nawel; Mellouli, Fethi; Abdelaali, Alya; Ouederni, Monia; Guedri, R.; Yousr, Dridi; Askri, Mouna; Béjaoui, Mohamed

doi:10.1097/mph.0b013e3182880784

Cited by 1 publication

(1 citation statement)

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“…18 Cases of hepatic vein thrombosis (Budd-Chiari syndrome) have been described in TM. 19 In sickle cell disease: Naik RP et al recently reported in 1,523 SCD patients, aged ≥15 years and cumulating 8,862 years of follow-up, an incidence rate for first venous thromboembolism of 5.2 events/1000 person-years, and a cumulative incidence of 11.3% by age 40. 20 A specific vascular-related hepatic complication, named sickle cell intrahepatic cholestasis (SCIC), has been described in SCD.…”

Section: Vascular Obstructionmentioning

confidence: 99%

Management of Liver Complications in Haemoglobinopathies

Brissot

Loréal

2014

Thalassemia Reports

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Liver complications in haemoglobinopathies (thalassaemia and sickle cell disease) are due to several factors, dominated (beside chronic viral infections, not considered here) by chronic iron overload, biliary obstruction and venous thrombosis. Whereas the latter two factors can cause acute hepatic syndromes, all three mechanisms - when becoming chronic- can produce fibrosis and cirrhosis and even, in thalassaemia, hepatocellular carcinoma. These chronic hepatic complications are an indirect consequence of the significant improvement in life expectancy due to the overall amelioration of disease management. The diagnostic approach has benefited from non invasive (biochemical and imaging) approaches which have considerably reduced the indication of liver biopsy. The therapeutic management involves relatively efficient curative medical, endoscopic or surgical methods, but should rest primarily on preventive measures focused on the haematological causative factors but also on hepatic co-morbidities. This chapter will focus on hepatic complications in thalassaemia and sickle cell disease (SCD), without considering the complications related to virus B or C infections which will be described in another chapter.

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Section: Vascular Obstructionmentioning

confidence: 99%