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Molecular Basis of Pulmonary Disease Insights from Rare Lung Disorders

:	Bruce C. Trapnell, Ralph J. Panos, Francis X. McCormack
:	Francis X. McCormack, Ralph J. Panos, Bruce C. Trapnell
:	Molecular Basis of Pulmonary Disease Insights from Rare Lung Disorders
:	Humana Press
:	9781597453844
:	1
:	CHF 198.40
:

:	Klinische Fächer
:	English

:	438
:	Wasserzeichen/DRM
:	PC/MAC/eReader/Tablet
:	PDF

The study of rare lung disorders enhances our understanding of common pulmonary diseases such as fibrosis and emphysema. Molecular Basis of Lung Disease: Insights from Rare 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 titrypsin deficiency are treated in detail. Written for pulmonary clinicians and scientists alike, Molecular Basis of Lung Disease: Insights from Rare 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.

	Preface	6
	Contents	8
	Contributors	10
	1 A Clinical Approach to Rare Lung Diseases	13
	Introduction	13
	Diagnostic Evaluation	15
	History	15
	Breathlessness	16
	Chest Discomfort	16
	Respiratory Sounds or Noises	17
	Medical History	17
	Family/Social History	17
	Occupational/Environmental History	17
	Review of Systems	18
	Physical Examination	18
	Inspection	18
	Palpation and Percussion	18
	Auscultation	19
	Imaging Studies	19
	Physiologic Studies	20
	Other Studies	21
	Pulmonary Differential Diagnosis of Rare or Unusual Conditions	22
	References	41
	2 Clinical Trials for Rare Lung Diseases	43
	Introduction	44
	A Hierarchy of Study Designs	46
	Summary	49
	References	49
	3 Idiopathic and Familial Pulmonary Arterial Hypertension	51
	Historical Review of Pulmonary Arterial Hypertension	52
	Clinical History	52
	Development of Clinical Classifications	52
	Epidemiology of Pulmonary Arterial Hypertension	54
	Incidence and Natural History	54
	Prognostic Factors	54
	Clinical Assessment of Pulmonary Arterial Hypertension	55
	Defining Characteristics	55
	Clinical History	55
	Physical Examination Findings	55
	Diagnostic Testing	56
	Electrocardiography	56
	Chest Radiography	57
	Doppler Echocardiography	57
	Evaluation of the Etiology of Pulmonary Hypertension	58
	Pulmonary Function Testing	59
	Evaluation for Thromboembolic Disease	59
	Radiographic Testing	59
	Serologic and Laboratory Testing	60
	MRI	60
	Confirmation of Presence of Pulmonary Arterial Hypertension (PAH)	60
	Pathology	61
	Genetics	63
	History	63
	Identification of BMPR2 Gene Mutations	64
	Genetic Testing and Counseling	66
	Potential Modifiers and Other Genes	66
	Pathogenesis	67
	Endothelial Dysfunction	68
	Nitric Oxide	69
	Prostacyclin	69
	Thromboxane	70
	Endothelin	70
	Growth Factors	70
	Serotonin	71
	Bone Morphogenic Proteins (BMPs), Transforming Growth Factor- (TGF-), and Smad Signaling	71
	Vasoactive Intestinal Peptide (VIP)	72
	Tenascin-C (TN-C)	72
	Elastase	73
	S100A4	73
	Inflammation	73
	Viral Infection	73
	Ion Channels	74
	Rho-Kinase	74
	Current Therapies for Pulmonary Hypertension	75
	General Measures	75
	Conventional Therapies	75
	Calcium Channel Blockers	76
	Targeted Pulmonary Arterial Hypertension Therapies	76
	Prostanoids	76
	Endothelin Receptor Antagonists	77
	Phosphodiesterase Inhibitors	78
	Interventional Procedures	78
	Future Directions	79
	Diagnosis and Management	79
	Pharmacotherapies	79
	Genetics	80
	Pathobiology and Cell-Based Therapies	80
	References	81
	4 Lymphangioleiomyomatosis	97
	Introduction	98
	Epidemiology	98
	Genetic Basis and Molecular Pathology	100
	Overview	100
	Tuberous Sclerosis Complex-Associated LAM	100
	Sporadic LAM	101
	LAM and the mTOR Signaling Cascade	102
	The Cell of Origin of LAM	104
	Why Does LAM Occur in Women?	105
	Why Is LAM Associated with Cystic Lung Disease?	106
	Clinical Presentation	107
	Physical Examination	108
	Diagnosis	108
	Symptomatic Women Without a Prior Chest CT	108
	Symptomatic or Asymptomatic Patient with Cystic Change on a High-Resolution Chest CT	109
	Pathology	109
	Physiology	111
	Radiology	111
	Clinical Course and Management	112
	Pulmonary Function	112
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