BACKGROUND In healthy volunteers, the single-breath diffusing capacity of the lung for carbon monoxide (DLCO) decreases and DLCO normalized per liter alveolar volume (VA; DLCO/VA) increases if VA is decreased. We hypothesized that comparison of DLCO/VA with its predicted value at predicted total lung capacity (TLC) will result in an underestimation of the diffusion disorder in patients with a r...

OBJECTIVE . To assess the efficacy and tolerability of bosentan in pulmonary arterial hypertension secondary to systemic sclerosis (SSc-PAH) including patients with restrictive lung disease. METHODS We retrospectively reviewed 23 SSc-PAH patients with PAH at baseline [PA systolic pressure (PASP) >or= 45 mm Hg by echocardiogram or mean PA pressure > 25 mm Hg at rest by cardiac catheterization]...

Background—An elevated Tl lung uptake after stress is related to an adverse prognosis. Methods and Results—The functional and prognostic significance of resting Tl lung uptake was assessed in 124 consecutive patients with ischemic heart disease and ejection fraction #35% undergoing rest-redistribution tomography to evaluate myocardial viability. Tl lung uptake significantly correlated with pulm...

OBJECTIVE To characterize the pulmonary dysfunction in patients with nephropathic cystinosis after renal transplantation. DESIGN Cross-sectional analysis of consecutive adult patients. PATIENTS Twelve adult, nephropathic cystinosis patients and 3 adult, ocular, nonnephropathic cystinosis patients admitted to the National Institutes of Health Clinical Center. RESULTS The 12 nephropathic cy...

We developed a simplified automated algorithm to interpret noninvasive gas exchange in healthy subjects and patients with heart failure (HF, n = 12), pulmonary arterial hypertension (PAH, n = 11), chronic obstructive lung disease (OLD, n = 16), and restrictive lung disease (RLD, n = 12). They underwent spirometry and thereafter an incremental 3-minute step test where heart rate and SpO2 respira...

BACKGROUND A study was performed to determine the elastic equilibrium volume (Vr) of the respiratory system in patients with chronic obstructive pulmonary disease (COPD). METHODS Voluntary relaxed expiration from total lung capacity (TLC) was studied in three groups of subjects: seven patients with severe chronic airways obstruction (COPD), 10 normal subjects, and 15 subjects with restrictive...

Langerhans cells are dendritic that form the antigenic barrier of human body. They occur in nearly any tissue but most prevalent skin, submucosa bronchial tree, and other mucosae. Cell Histiocytosis (LCH) develops when these damage tissues which they reside through a combination inflammatory monoclonal stimulation. The pulmonary variant LCH involves lung parenchyma creates wide variety disturba...