Rare and Orphan Lung Diseases, An Issue of Clinics in Chest Medicine
1. Auflage
Erschienen am 16. August 2016
100 Seiten
978-0-323-46274-7 (ISBN)
Beschreibung
Drs. Robert Kotloff and Francis McCormack have assembled an expert team of authors on the topic of Rare and Orphan Lung Diseases. Articles include: Lymphangioleiomyomatosis, Pulmonary Lymphangiomatosis, Langerhans Cell Histiocytosis and other Histiocytic Diseases of the Lung, Pulmonary Alveolar Proteinosis, Pulmonary Alveolar Microlithiasis, Primary Ciliary Dyskinesia, Birt-Hogg-Dube Syndrome, Hermansky-Pudlak Syndrome, Hereditary Hemorrhagic Telangiectasia, Non-CF Bronchiectasis, Eosinophilic Lung Diseases, Benign Metastasizing Leiomyomata, and more!
Weitere Details
Weitere Ausgaben
Andere Ausgaben
Robert Kotloff | Francis X. McCormack
Rare and Orphan Lung Diseases, An Issue of Clinics in Chest Medicine: Volume 37-3
Volume 37-3
Buch
08/2016
Elsevier
129,50 €
Versand in 15-20 Tagen
Inhalt
- Front Cover
- Rare and Orphan Lung Diseases
- Copyright
- Contributors
- EDITORS
- AUTHORS
- Contents
- Preface: Rare Lung Diseases: Occasionally the "Horse" Has Stripes
- Lymphangioleiomyomatosis
- Plastic Bronchitis
- Nonmalignant Adult Thoracic Lymphatic Disorders
- Langerhans Cell Histiocytosis and Other Histiocytic Diseases of the Lung
- Pulmonary Alveolar Proteinosis Syndrome
- Pulmonary Alveolar Microlithiasis
- Primary Ciliary Dyskinesia
- Lymphocytic Interstitial Pneumonia
- Birt-Hogg-Dubé Syndrome
- a1-Antitrypsin Deficiency
- Hermansky-Pudlak Syndrome
- Hereditary Hemorrhagic Telangiectasia
- Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease
- Eosinophilic Lung Diseases
- Hyper-IgE Syndromes and the Lung
- Immunoglobulin G4-Related Disease and the Lung
- Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Neuroendocrine Hyperplasia of Infancy
- Benign Metastasizing Leiomyoma
- CME Accreditation Page
- PROGRAM OBJECTIVE
- TARGET AUDIENCE
- LEARNING OBJECTIVES
- ACCREDITATION
- DISCLOSURE OF CONFLICTS OF INTEREST
- UNAPPROVED/OFF-LABEL USE DISCLOSURE
- TO ENROLL
- METHOD OF PARTICIPATION
- CME INQUIRIES/SPECIAL NEEDS
- CLINICS IN CHEST MEDICINE
- FORTHCOMING ISSUES
- December 2016
- March 2017
- June 2017
- RECENT ISSUES
- June 2016
- March 2016
- December 2015
- Preface: Rare Lung Diseases: Occasionally the "Horse" Has Stripes
- Lymphangioleiomyomatosis
- Key points
- INTRODUCTION
- PATHOLOGIC EVALUATION
- Histology
- Lymphangioleiomyomatosis Cell Biology
- Circulating Cells: Lymphangioleiomyomatosis as a Metastatic Neoplasm
- Tuberous Sclerosis Complex Genes and Mammalian Target of Rapamycin Activation
- PRESENTATION AND DIAGNOSTIC STRATEGY
- Presentation
- Diagnostic Strategy
- MANAGEMENT AND DISEASE COURSE
- Initial Assessment and Prognosis
- General Principles of Management
- Pregnancy and Contraception
- Tuberous Sclerosis-Related Clinical Problems
- Bronchodilators
- Mammalian Target of Rapamycin Activation Inhibitors
- Pneumothorax
- Chylous Complications
- Angiomyolipomas
- Meningioma
- Advanced Disease
- SUMMARY
- REFERENCES
- Plastic Bronchitis
- Key points
- INTRODUCTION
- PATIENT EVALUATION OVERVIEW
- DISEASE ASSOCIATIONS
- PHARMACOLOGIC TREATMENT OPTIONS
- NONPHARMACOLOGIC AND SURGICAL TREATMENT OPTIONS
- EVALUATION OF OUTCOMES AND LONG-TERM RECOMMENDATIONS
- THE FUTURE
- REFERENCES
- Nonmalignant Adult Thoracic Lymphatic Disorders
- Key points
- INTRODUCTION
- THORACIC LYMPHANGIOMAS (MICROCYSTIC AND MACROCYSTIC LYMPHATIC MALFORMATIONS)
- LYMPHANGIOLEIOMYOMATOSIS
- PRIMARY PULMONARY LYMPHATIC ANOMALY
- GENERALIZED LYMPHATIC ANOMALY WITH PULMONARY INVOLVEMENT
- GORHAM-STOUT DISEASE
- KAPOSIFORM LYMPHANGIOMATOSIS
- CHYLOPTYSIS AND PLASTIC BRONCHITIS
- PULMONARY LYMPHANGIECTASIA
- THE YELLOW NAIL SYNDROME
- SUMMARY AND FUTURE DIRECTIONS
- REFERENCES
- Langerhans Cell Histiocytosis and Other Histiocytic Diseases of the Lung
- Key points
- INTRODUCTION
- EPIDEMIOLOGY AND DEMOGRAPHICS
- PATHOLOGY AND PATHOPHYSIOLOGY
- CLINICAL FEATURES AND DIAGNOSTIC EVALUATION
- IMAGING AND PULMONARY FUNCTION TESTING
- APPROACH TO THE DIAGNOSIS OF PULMONARY LANGERHANS CELL HISTIOCYTOSIS
- MANAGEMENT
- ERDHEIM-CHESTER DISEASE
- ROSAI-DORMAN DISEASE (SINUS HISTIOCYTOSIS WITH MASSIVE LYMPHADENOPATHY)
- SUMMARY
- REFERENCES
- Pulmonary Alveolar Proteinosis Syndrome
- Key points
- INTRODUCTION
- PATHOGENESIS
- CLINICAL PRESENTATION
- DIAGNOSIS
- Radiographic Findings
- Laboratory Findings
- Pulmonary Function Testing
- Bronchoscopy, Bronchoalveolar Lavage, Microbiologic Culture
- Histopathology
- Biomarkers
- NATURAL HISTORY
- THERAPY
- Current Therapy
- Experimental Approaches
- KNOWLEDGE GAPS AND FUTURE RESEARCH
- ACKNOWLEDGMENTS
- REFERENCES
- Pulmonary Alveolar Microlithiasis
- Key points
- INTRODUCTION
- EPIDEMIOLOGY
- PATHOLOGY
- Molecular Pathogenesis
- Histology
- CLINICAL FEATURES
- Symptoms and Signs
- Clinical Testing
- Radiology
- Extrapulmonary Disease
- Associated Diseases
- DIAGNOSIS
- DIFFERENTIAL DIAGNOSIS
- PROGNOSIS
- PHARMACOLOGIC TREATMENT OPTIONS
- Etidronate
- Steroid Hormones
- Bronchoalveolar Lavage
- Oxygen Therapy and Vaccinations
- SURGICAL TREATMENT OPTIONS
- Lung Transplantation
- SUMMARY
- REFERENCES
- Primary Ciliary Dyskinesia
- Key points
- INTRODUCTION
- STRUCTURE AND FUNCTION OF MOTILE CILIA
- ULTRASTRUCTURAL AND FUNCTIONAL DIAGNOSTIC TESTS
- GENETIC TESTING FOR DIAGNOSIS
- EARLY FEATURES OF CLINICAL DISEASE
- LUNG DISEASE
- NONPULMONARY MANIFESTATIONS
- MANAGEMENT OF LUNG DISEASE
- MANAGEMENT OF OTOLARYNGOLOGIC MANIFESTATIONS
- FUTURE DIRECTIONS
- ACKNOWLEDGMENTS
- REFERENCES
- Lymphocytic Interstitial Pneumonia
- Key points
- INTRODUCTION
- PATHOGENESIS
- EPIDEMIOLOGY
- CLINICAL PRESENTATION
- RADIOGRAPHIC MANIFESTATIONS
- PULMONARY FUNCTION TESTS
- DIAGNOSIS
- PATHOLOGIC FEATURES
- LYMPHOCYTIC INTERSTITIAL PNEUMONIA AND LYMPHOMA: DIFFERENTIATING FEATURES
- TREATMENT AND PROGNOSIS
- SUMMARY
- REFERENCES
- Birt-Hogg-Dubé Syndrome
- Key points
- INTRODUCTION
- GENETICS AND PATHOGENESIS
- Folliculin and Its Role as a Tumor Suppressor
- Mechanism of Action of Folliculin in Tumor Formation
- Role of Folliculin in Formation of Pulmonary Cysts
- CLINICAL MANIFESTATIONS
- Pulmonary
- Renal
- Cutaneous
- Other Clinical Manifestations
- RADIOGRAPHIC FEATURES OF BIRT-HOGG-DUBÉ SYNDROME
- Pulmonary
- Renal
- HISTOPATHOLOGIC FEATURES OF BIRT-HOGG-DUBÉ SYNDROME
- Pulmonary
- Renal
- Skin
- DIAGNOSTIC APPROACH
- MANAGEMENT
- Management of Skin Lesions
- Management of the Pulmonary Aspects of Birt-Hogg-Dubé Syndrome
- Management of the Renal Manifestations of Birt-Hogg-Dubé Syndrome
- SUMMARY
- REFERENCES
- a1-Antitrypsin Deficiency
- Key points
- INTRODUCTION
- GENETICS OF a1-ANTITRYPSIN DEFICIENCY
- EPIDEMIOLOGY OF a1-ANTITRYPSIN DEFICIENCY
- DIAGNOSIS OF a1-ANTITRYPSIN DEFICIENCY
- PATHOPHYSIOLOGY
- PULMONARY MANIFESTATIONS OF a1-ANTITRYPSIN DEFICIENCY
- NATURAL HISTORY OF a1-ANTITRYPSIN DEFICIENCY-RELATED LUNG DISEASE
- LIVER DISEASE IN a1-ANTITRYPSIN DEFICIENCY
- VASCULITIS IN a1-ANTITRYPSIN DEFICIENCY
- PANNICULITIS AND OTHER PROPOSED DISEASE ASSOCIATIONS WITH a1-ANTITRYPSIN DEFICIENCY
- TREATMENT OF a1-ANTITRYPSIN DEFICIENCY
- Augmentation Therapy
- Lung Transplantation
- Lung Volume Reduction
- FUTURE DIRECTIONS
- REFERENCES
- Hermansky-Pudlak Syndrome
- Key points
- INTRODUCTION
- EPIDEMIOLOGY
- PATHOGENESIS
- DIAGNOSIS
- Clinical Features
- Diagnostic Testing
- Platelet electron microscopy
- Genetic testing
- Diagnosis of Interstitial Lung Disease
- High-resolution computed tomography of the chest
- NATURAL HISTORY AND PROGNOSIS
- CLINICAL MANAGEMENT
- Management of Pulmonary Disease
- Pirfenidone in Hermansky-Pudlak Syndrome with Pulmonary Fibrosis
- Lung Transplantation
- Additional Recommendations for Pulmonary Management
- Management of Extrapulmonary Complications
- Skin and eye protection
- Bleeding complications
- Inflammatory bowel disease
- FUTURE DIRECTIONS
- SUMMARY
- REFERENCES
- Hereditary Hemorrhagic Telangiectasia
- Key points
- INTRODUCTION
- GENETICS
- DIAGNOSIS
- PULMONARY INVOLVEMENT
- Pulmonary Arteriovenous Malformations
- Bronchial Telangiectasias
- Pulmonary Hypertension
- NASAL INVOLVEMENT
- CENTRAL NERVOUS SYSTEM INVOLVEMENT
- GASTROINTESTINAL INVOLVEMENT
- HEPATIC INVOLVEMENT
- SUMMARY
- REFERENCES
- Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease
- Key points
- INTRODUCTION
- PULMONARY VENO-OCCLUSIVE DISEASE
- History
- Pathology
- Epidemiology
- Risk Factors
- Genetic Factors
- Clinical Features
- Imaging
- Laboratory Studies
- Hemodynamics
- Prognosis and Therapy
- PULMONARY CAPILLARY HEMANGIOMATOSIS
- History
- Pathology
- Epidemiology
- Risk Factors
- Genetic Factors
- Clinical Presentation
- Imaging
- Laboratory Studies
- Hemodynamics
- Prognosis
- Therapy
- PULMONARY VENO-OCCLUSIVE DISEASE AND PULMONARY CAPILLARY HEMANGIOMATOSIS: SPECTRUM OF A SINGLE DISEASE OR DISTINCT ENTITIES?
- Histologic Overlap
- Genetic Overlap
- PULMONARY VENO-OCCLUSIVE DISEASE AND PULMONARY CAPILLARY HEMANGIOMATOSIS: DIAGNOSTIC APPROACH
- SUMMARY
- ACKNOWLEDGMENTS
- REFERENCES
- Eosinophilic Lung Diseases
- Key points
- DEFINITION AND CLASSIFICATION
- Definition
- Classification
- PATHOPHYSIOLOGY
- Recruitment of Eosinophils to the Lung
- Eosinophils and Immunity
- IDIOPATHIC CHRONIC EOSINOPHILIC PNEUMONIA
- Epidemiology and Risk Factors
- Clinical Description
- Chest Imaging
- Laboratory Findings
- Pathogenesis
- Lung Function
- Pathology
- Diagnosis
- Treatment and Outcome
- IDIOPATHIC ACUTE EOSINOPHILIC PNEUMONIA
- Epidemiology and Risk Factors
- Clinical Description
- Chest Imaging
- Laboratory Findings
- Lung Function
- Pathology
- Diagnosis
- Treatment and Outcome
- EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS
- Definition
- Epidemiology and Risk Factors
- Clinical Description
- Chest Imaging
- Laboratory Findings
- Lung Function
- Pathology
- Diagnosis
- Treatment and Outcome
- ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS
- Epidemiology and Pathogenesis
- Clinical Description
- Chest Imaging
- Laboratory Findings
- Pathology
- Diagnosis
- Treatment and Outcome
- OTHER EOSINOPHILIC LUNG DISEASES
- Idiopathic Hypereosinophilic Syndromes
- Idiopathic Hypereosinophilic Obliterative Bronchiolitis
- Eosinophilic Pneumonias in Parasitic Diseases
- Eosinophilic Pneumonias Induced by Drugs and Toxics
- Radiation Therapy
- Miscellaneous
- PRACTICAL APPROACH TO DIAGNOSIS AND TREATMENT
- REFERENCES
- Hyper-IgE Syndromes and the Lung
- Key points
- INTRODUCTION
- AUTOSOMAL-DOMINANT HYPER-IGE SYNDROMES (JOB SYNDROME)
- Pulmonary Features
- Other Clinical Features
- Laboratory Findings
- Genetics and Pathogenesis
- Management
- DOCK8 DEFICIENCY
- Clinical Features
- Laboratory Findings
- Genetics and Pathogenesis
- Management
- PGM3 DEFICIENCY
- Clinical Features
- Laboratory Findings
- Genetics and Pathogenesis
- Management
- SUMMARY
- REFERENCES
- Immunoglobulin G4-Related Disease and the Lung
- Key points
- INTRODUCTION
- EPIDEMIOLOGY
- PATHOGENESIS
- Immunologic Mechanisms
- Immunoglobulin G4
- Genetic Predisposition
- Microorganisms
- PATHOLOGY
- Clinical Manifestations
- Symptoms
- Immunoglobulin G4-Related Lung Disease
- Parenchymal Lung Manifestations
- Airway Manifestations
- Pulmonary Vascular Manifestations
- Mediastinal Manifestations
- Pleural Manifestations
- DIAGNOSTIC EVALUATION
- Immunoglobulin G4 Immunostain
- Serum Immunoglobulin G4 Level
- Other Laboratory Tests
- BRONCHOALVEOLAR LAVAGE
- TREATMENT
- Corticosteroids
- Steroid-Sparing Immunosuppressive Agents
- B-Cell Depletion Therapy
- PROGNOSIS
- SUMMARY
- REFERENCES
- Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Neuroendocrine Hyperplasia of Infancy
- Key points
- INTRODUCTION
- DIFFUSE IDIOPATHIC NEUROENDOCRINE EPITHELIAL CELL HYPERPLASIA
- Imaging
- Airway abnormalities
- Pulmonary nodules
- Biochemical Testing
- Physiology
- Pathology
- Management and Treatment
- NEUROENDOCRINE HYPERPLASIA OF INFANCY
- Imaging
- Physiology
- Pathology
- Management and Treatment
- Pathogenesis
- SUMMARY AND DISCUSSION
- REFERENCES
- Benign Metastasizing Leiomyoma
- Key points
- INTRODUCTION
- CLINICAL PRESENTATION
- IMAGING STUDIES
- PATHOLOGY
- PATHOGENESIS
- DIAGNOSIS
- TREATMENT
- PROGNOSIS
- SUMMARY
- REFERENCES
- Index
