Article
Hepatopulmonary syndrome is a frequent cause of dyspnea in the short telomere disorders.
Chest - 1 Oct 2015
Gorgy Amany I, Jonassaint Naudia L, Stanley Susan E, Koteish Ayman, DeZern Amy E, Walter Jolan E, Sopha Sabrina C, Hamilton James P, Hoover-Fong Julie, Chen Allen R, Anders Robert A, Kamel Ihab R, Armanios Mary
Abstract excerpt
BACKGROUND: Telomere syndromes have their most common manifestation in idiopathic pulmonary fibrosis and emphysema. The short telomere defect in these patients may manifest systemically as bone marrow failure and liver disease. We sought to understand the causes of dyspnea in telomerase and telomere gene mutation carriers who have no parenchymal lung disease. METHODS: Clinical and pathologic data were reviewed as...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.