Pulmonary nodular opacities in children with hereditary hemorrhagic telangiectasia
β Scribed by David Manson; Jeffrey Traubici; Meir Mei-Zahav; Ian MacLuskey; Philip John; Derek Stephens
- Publisher
- Springer-Verlag
- Year
- 2007
- Tongue
- English
- Weight
- 181 KB
- Volume
- 37
- Category
- Article
- ISSN
- 0301-0449
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## Abstract Pulmonary arterial hypertension (PAH) and hereditary hemorrhagic telangiectasia (HHT) are distinct clinical entities caused by germline mutations in genes encoding members of the TGFΞ²/BMP superfamily: __BMPR2__ in PAH and __ACVRL1__, __ENG__, or __SMAD4__ in HHT. When PAH and HHT occasi
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