BACKGROUND Bronchiolitis obliterans syndrome (BOS) with small-airway pathology and obstructive pulmonary physiology may not be the only form of chronic lung allograft dysfunction (CLAD) after lung transplantation. Characteristics of a form of CLAD consisting of restrictive functional changes involving peripheral lung pathology were investigated. METHODS Patients who received bilateral lung tr...

bronchial responsiveness to bronchodilator medications is usually tested to establish reversibility of airflow obstruction. among the various tests to establish bronchodilator response, fev1, fef25-75% or fef50%, and fvc are the most widely used. in a cross-sectional study, we assessed spirometric responses after administration of bronchodilator in 187 workers with obstructive pattern in spirom...

background: the objective of this study is to assess the safety and preventative effects of aminophylline on the incidence of chronic lung disease (cld) in very premature infants. methods: this was a long follow‑up randomized clinical trial. the prophylactic effect of aminophylline on the incidence of cld was investigated in very premature infants. the study group received aminophylline for the...

BACKGROUND Recent studies have suggested that a restrictive pattern assessed with a single spirometric test is associated with increased morbidity and mortality. This study was undertaken to determine demographic, clinical and mortality profiles of subjects with either a recurrent or an inconsistent restrictive spirometric pattern assessed prospectively. METHODS Data from 2048 adult participa...

Because NMD generally affects the respiratory muscles, most of these patients suffer from restrictive lung disease (RLD). The restrictive component tends to be especially severe in amyotrophic lateral sclerosis, Duchenne’s muscular dystrophy, and spinal muscular atrophy types I and II.8 Even in the earliest stages of these diseases, when few if any respiratory symptoms have been reported, pulmo...

BACKGROUND The allocation of donor lungs for transplantation in Germany was changed on 10 December 2011 to a system based on the Lung Allocation Score (LAS). The aim of the present study is to determine whether this change has prolonged the survival of patients on the transplant waiting list and of those who have undergone lung transplantation (LTx). METHODS We retrospectively compared data f...

IPF is an interstitial lung disease of unknown etiology characterized by a progressive course, a poor prognosis with a median survival of approximately three years, and a lack of evidence that any treatment prolongs survival. While the pathogenesis of IPF is still unclear, increasing evidence points to an unidentified stimulus resulting in repetitive focal injury of alveolar epithelial cells an...

Restrictive cardiomyopathies in the pediatric population have diverse etiologies, including storage diseases like hemosiderosis, glycogenoses and desmin with its associated proteins. Desmin-related myopathy is a rare familial disorder of the cardiac and skeletal muscle characterized by intrasarcoplasmic accumulation of desmin-reactive deposits in the muscle cells. The patients commonly present ...