Severe pulmonary hypertension in histiocytosis X: long-term improvement with bosentan
نویسندگان
چکیده
منابع مشابه
Severe pulmonary hypertension in histiocytosis X: long-term improvement with bosentan.
Pulmonary Langerhans’ cell histiocytosis (LCH)/histiocytosis X is a rare smoking-related interstitial lung disease that predominantly affects adults aged 20–40 yrs. It is commonly associated with irreversible airflow obstruction and highly variable clinical outcomes. The disease may regress spontaneously or following smoking cessation; however, in 20–30% of cases it progresses to chronic respir...
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Pulmonary arterial hypertension (PAH) secondary to pulmonary Langerhans cell histiocytosis (PLCH) is known to be a relatively common complication and is associated with a poor prognosis. However, the optimal therapeutic approach for these cases remains to be established. A 57-year-old man visited our hospital because of a progressive dry cough. A thoracic computed tomography examination showed ...
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A case report of histiocytosis X in a 2 years old infaut, primarily admitted a for jaundice is discussed. Major clinical manifestations included: Jaundice, bilateral otitis media. pallor and loss of weight. Clinical examination revealed hepatosplenomegaly. lymphadenopathies and sever anemia. Seborrr.eic eruptions and ptechias appeared over the scalp and other parts of body surface a few days ...
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Bosentan has proven 4-month efficacy in patients with HIV-associated pulmonary arterial hypertension (PAH-HIV). Herein, the long-term outcome of unselected PAH-HIV patients treated with first-line bosentan is described. Data for 59 consecutive World Health Organization (WHO) functional class II-IV PAH-HIV patients treated with first-line bosentan between May 2002 and July 2007 were analysed. HI...
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OBJECTIVES This study investigated the long-term outcome of children with pulmonary arterial hypertension (PAH) treated with bosentan therapy, with or without concomitant prostanoid therapy. BACKGROUND Bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, improves hemodynamics and exercise capacity in adults with PAH; however, limited data are available on its long-term effects in chi...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2010
ISSN: 0903-1936,1399-3003
DOI: 10.1183/09031936.00004810