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Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

Overview of attention for article published in Respiratory Medicine Case Reports, November 2014
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Title
Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy
Published in
Respiratory Medicine Case Reports, November 2014
DOI 10.1016/j.rmcr.2014.11.005
Pubmed ID
Authors

Adam May, Garvan Kane, Eunhee Yi, Robert Frantz, Robert Vassallo

Abstract

Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH.

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The data shown below were compiled from readership statistics for 13 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 13 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 4 31%
Student > Doctoral Student 2 15%
Other 2 15%
Student > Master 1 8%
Professor 1 8%
Other 0 0%
Unknown 3 23%
Readers by discipline Count As %
Medicine and Dentistry 7 54%
Social Sciences 1 8%
Nursing and Health Professions 1 8%
Unknown 4 31%