000 03562nam a22004575i 4500
001 978-1-59745-384-4
003 DE-He213
005 20140220084512.0
007 cr nn 008mamaa
008 100316s2010 xxu| s |||| 0|eng d
020 _a9781597453844
_9978-1-59745-384-4
024 7 _a10.1007/978-1-59745-384-4
_2doi
050 4 _aRC705-779
072 7 _aMJL
_2bicssc
072 7 _aMED079000
_2bisacsh
082 0 4 _a616.2
_223
100 1 _aMcCormack, Francis X.
_eeditor.
245 1 0 _aMolecular Basis of Pulmonary Disease
_h[electronic resource] :
_bInsights from Rare Lung Disorders /
_cedited by Francis X. McCormack, Ralph J. Panos, Bruce C. Trapnell.
264 1 _aTotowa, NJ :
_bHumana Press,
_c2010.
300 _aX, 434p. 49 illus., 27 illus. in color.
_bonline resource.
336 _atext
_btxt
_2rdacontent
337 _acomputer
_bc
_2rdamedia
338 _aonline resource
_bcr
_2rdacarrier
347 _atext file
_bPDF
_2rda
490 1 _aRespiratory Medicine
505 0 _aA Clinical Approach to Rare Lung Diseases -- Clinical Trials for Rare Lung Diseases -- Idiopathic and Familial Pulmonary Arterial Hypertension -- Lymphangioleiomyomatosis -- Autoimmune Pulmonary Alveolar Proteinosis -- Mutations in Surfactant Protein C and Interstitial Lung Disease -- Hereditary Haemorrhagic Telangiectasia -- Hermansky–Pudlak Syndrome -- Alpha-1 Antitrypsin Deficiency -- The Marfan Syndrome -- Surfactant Deficiency Disorders: SP-B and ABCA3 -- Pulmonary Capillary Hemangiomatosis -- Anti-glomerular Basement Disease: Goodpasture’s Syndrome -- Primary Ciliary Dyskinesia -- Pulmonary Alveolar Microlithiasis -- Cystic Fibrosis -- Pulmonary Langerhans’ Cell Histiocytosis – Advances in the Understanding of a True Dendritic Cell Lung Disease -- Sarcoidosis -- Scleroderma Lung Disease.
520 _aThe study of rare lung disorders enhances our understanding of common pulmonary diseases such as fibrosis and emphysema. Molecular Basis of Pulmonary Disease: Insights from Rare Lung Disorders brings together a panel of distinguished clinicians and molecular scientists who are experts in a range of rare lung diseases and their underlying molecular defects. Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy-to-read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models, clinical presentation, diagnostic approaches, conventional management and treatment strategies, as well as future therapeutic targets and directions. Disorders ranging from the Marfan and Goodpasture’s syndromes to Sarcoidosis and alpha one antitrypsin deficiency are treated in detail. Written for pulmonary clinicians and scientists alike, Molecular Basis of Pulmonary Disease: Insights from Rare Lung Disorders is a comprehensive and invaluable nesource that sheds new light on the molecular mechanisms influencing the clinical presentation and treatment strategies for these debilitating disorders.
650 0 _aMedicine.
650 0 _aPneumology.
650 1 4 _aMedicine & Public Health.
650 2 4 _aPneumology/Respiratory System.
700 1 _aPanos, Ralph J.
_eeditor.
700 1 _aTrapnell, Bruce C.
_eeditor.
710 2 _aSpringerLink (Online service)
773 0 _tSpringer eBooks
776 0 8 _iPrinted edition:
_z9781588299635
830 0 _aRespiratory Medicine
856 4 0 _uhttp://dx.doi.org/10.1007/978-1-59745-384-4
912 _aZDB-2-SME
999 _c110764
_d110764