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10.1016/j.ccm.2016.04.012 http://scihub22266oqcxt.onion/10.1016/j.ccm.2016.04.012 C4987498!4987498!27514596     free     free     free       Clin+Chest+Med 2016 ; 37 (3): 505-11 Nephropedia Template TP {{tp|p=27514596|t=2016. Hermansky-Pudlak Syndrome |pdf=|usr=}}{{27514596}} gab.com Text Hermansky-Pudlak Syndrome JO: Clin Chest Med http://www.kidney.de/pget.php?&tw=1&pmid=27514596 #FOAvip Hermansky-Pudlak Syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diathesis, granulomatous colitis, and highly penetrant pulmonary fibrosis in some subtypes, including HPS-1, HPS-2, and HPS-4. HPS pulmonary fibrosis exhibits many of the clinical, radiologic, and histologic features found in Idiopathic Pulmonary Fibrosis, but occurs at a younger age. All patients with oculocutaneous albinism and easy bruising or bleeding should be screened for HPS, though the degree of albinism is variable and can be subtle. All adult patients with HPS should be screened for pulmonary involvement with chest computerized tomography. Lung biopsy is not required for diagnosis in HPS and is contraindicated because of bleeding complications. Despite knowledge of the underlying genetic defects, there are currently no definitive therapeutic or preventative approaches for HPS pulmonary fibrosis other than lung transplantation. The Hermansky?Pudlak Syndrome Network, Inc. (http://www.hpsnetwork.org) is a support organization available for patients with HPS. Delineating molecular mechanisms responsible for fibrotic susceptibility in HPS holds promise for development of targeted therapies. Twit Text FOAVip Hermansky-Pudlak Syndrome ????Clin Chest Med http://www.kidney.de/pget.php?&tw=1&pmid=27514596 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=27514596 #FOAvip English Wikipedia <ref>{{Cite pmid|27514596}}</ref> Hermansky-Pudlak Syndrome #MMPMID27514596 El-Chemaly S; Young LR Clin Chest Med 2016[Sep]; 37 (3): 505-11 PMID27514596show ga Hermansky-Pudlak Syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diathesis, granulomatous colitis, and highly penetrant pulmonary fibrosis in some subtypes, including HPS-1, HPS-2, and HPS-4. HPS pulmonary fibrosis exhibits many of the clinical, radiologic, and histologic features found in Idiopathic Pulmonary Fibrosis, but occurs at a younger age. All patients with oculocutaneous albinism and easy bruising or bleeding should be screened for HPS, though the degree of albinism is variable and can be subtle. All adult patients with HPS should be screened for pulmonary involvement with chest computerized tomography. Lung biopsy is not required for diagnosis in HPS and is contraindicated because of bleeding complications. Despite knowledge of the underlying genetic defects, there are currently no definitive therapeutic or preventative approaches for HPS pulmonary fibrosis other than lung transplantation. The Hermansky?Pudlak Syndrome Network, Inc. (http://www.hpsnetwork.org) is a support organization available for patients with HPS. Delineating molecular mechanisms responsible for fibrotic susceptibility in HPS holds promise for development of targeted therapies. äHermansky-Pudlak Syndrome (epmc).pdf DeepDyve Pubget Overpricing