Liver involvement in hereditary hemorrhagic telangiectasia may lead to high-output cardiac failure. Few data have been reported on orthotopic liver transplantation (OLT) for these patients. In this paper, we describe two patients treated by OLT as a salvage procedure for cardiac failure, and we revi
ALK-1 mutations in liver transplanted patients with hereditary hemorrhagic telangiectasia
โ Scribed by Loukas Argyriou; Robert Pfitzmann; Lars-Erik Wehner; Stefan Twelkemeyer; Peter Neuhaus; Karim Nayernia; Wolfgang Engel
- Publisher
- John Wiley and Sons
- Year
- 2005
- Tongue
- English
- Weight
- 81 KB
- Volume
- 11
- Category
- Article
- ISSN
- 1527-6465
- DOI
- 10.1002/lt.20544
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Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by mucocutaneous and visceral telangiectasia. Hepatic involvement with vascular malformations may lead to portal hypertension, biliary ischemia, and high-output cardiac failure. Liver transplantation is indica
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant and age-dependent vascular disorder originated by mutations in Endoglin (ENG) or activin receptor-like kinase-1 (ALK1, ACVRL1) genes. The first large series HHT analysis in Spanish population has identified mutations in 17 unrelate
unclear, and further experience in a clinical trial setting is required to clarify this. Lastly, it is exciting to ponder that vascular endothelial growth factor antagonists may have a role in reducing symptoms in the substantial number of HHT patients who are symptomatic but do not require liver tr
Hereditary hemorrhagic telangiectasia (HHT), or Osler-Rendu-Weber syndrome, is a heterogeneous inherited disorder characterized by multi-systemic vascular dysplasia and wide variation in its phenotypic expression. Hepatic manifestation is seen in about 8 to 30 % of the patients. The molecular basis
## Abstract Pulmonary arterial hypertension (PAH) and hereditary hemorrhagic telangiectasia (HHT) are distinct clinical entities caused by germline mutations in genes encoding members of the TGFฮฒ/BMP superfamily: __BMPR2__ in PAH and __ACVRL1__, __ENG__, or __SMAD4__ in HHT. When PAH and HHT occasi