Sunday, September 06, 2009

HPS lung lavages

It's been a tough few weeks for all of us. I know it makes me feel better when I can see that our efforts are showing results and things are indeed moving forward. The following abstract is from the Journal of Respitory Critical Care Medicine. It's the result of many of you who went to the NIH to undergo lung lavages.

As the NIH breaks the blind on the drug trial, and those that were on active drug come back for lung lavages, we'll be working on the next step.

Way to go everyone!

Alveolar Macrophage Dysregulation in Hermansky-Pudlak Syndrome Type-1.Rouhani FN, Brantly ML, Markello TC, Helip-Wooley A, O'Brien K, Hess R, Huizing M, Gahl WA, Gochuico

BR.Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, United States.

RATIONALE: Individuals with Hermansky-Pudlak syndrome type 1 (HPS-1), an autosomal recessive disorder characterized by defective biogenesis of lysosome-related organelles, develop an accelerated form of progressive fibrotic lung disease. The etiology of pulmonary fibrosis associated with HPS-1 is unknown. OBJECTIVES: To investigate the potential pathogenesis of pulmonary fibrosis in HPS-1, lung cells and proteins from individuals with HPS-1 were studied. METHODS: Forty-one subjects with HPS-1 with and without pulmonary fibrosis were evaluated with pulmonary function tests, high-resolution computed tomography scan, and bronchoscopy. Bronchoalveolar lavage cells and analytes were analyzed. MEASUREMENTS AND MAIN RESULTS: Concentrations of total bronchoalveolar lavage cells and alveolar macrophages were significantly higher in epithelial lining fluid from subjects with HPS-1 with and without pulmonary fibrosis compared to healthy research volunteers. Concentrations of cytokines and chemokines (i.e., monocyte chemoattractant protein-1, macrophage inflammatory protein-1 alpha, granulocyte-macrophage colony-stimulating factor) in alveolar epithelial lining fluid were significantly higher in subjects with HPS-1 with and without pulmonary fibrosis compared to healthy research volunteers (P<0.001). p="0.001," and ="0.011,">

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