Work

Unraveling restrictive chronic lung disease in sickle cell disease [Editorial]

Year: 2013

Type: editorial

Abstract: A LEADING CAUSE of decreased life expectancy in adults with sickle cell disease (SCD) is acute and chronic lung disease (CLD).CLD historically encompassed both pulmonary f brosis and pulmonary hypertension; 1 however, we now understand that these are distinct entities that may not necessarily coexist in SCD patients.While the latter has been the subject of thorough investigations that have determined its high prevalence (approximately 10% by right heart catheterization), strong association with chronic hemolytic anemia, and its relevance as a risk factor for early mortality in SCD, 2-5 the former has not been studied comprehensively in the modern era.

Source: The International Journal of Tuberculosis and Lung Disease

DOI: 10.5588/ijtld.13.0509

Authors: Shilpa Jain, Mark T. Gladwin, Enrico M. Novelli

Institutions: University of Pittsburgh, Pulmonary and Allergy Associates

Language: English

Topic: Hemoglobinopathies and Related Disorders

Subfield: Genetics

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

Funder: National Heart, Lung, and Blood Institute