Pulmonary Manifestations of Neuromuscular Diseases.

Neuromuscular disorders comprise a phenotypically diverse group of inherited and acquired diseases; however, they share common pathophysiologic mechanisms which produce significant respiratory complications. Respiratory and bulbar muscle weakness gives rise to ineffective cough, swallowing dysfunction results in aspiration-related lung disease, and abnormal muscle tone produces chest wall deformities - all of which ultimately leads to repeated chest infections and chronic respiratory failure. In this article, the authors describe the respiratory manifestations of neuromuscular disease and their underlying pathophysiological mechanisms.

MMP-8 promotes polymorphonuclear cell migration through collagen barriers in obliterative bronchiolitis.

Increased levels of MMP-8 (neutrophil collagenase) have been reported in OB, but the biological role of MMP-8 in OB is not known. MMP-8 is an interstitial collagenase highly expressed by polymorphonuclear leukocytes, which are prominent in early OB. Here, we show that MMP-8 promotes migration of PMNs through the collagen-rich matrix in a mouse heterotopic airway transplant model of OB.