Orphan Lung Diseases

The second edition comprehensively reviews the wide field of rare pulmonary diseases. Issues such as less common diseases affecting the airways, systematic disorders with lung involvement, interstitial lung diseases, and many other orphan conditions of the lungs are explored in this book. The progress and advances made in the field and the limited number of patients presenting each condition makes it very difficult for clinicians to be up-to-date in this field. Readers will discover how to diagnose and manage these rare orphan diseases. Orphan Lung Diseases: A Clinical Guide to Rare Lung Disease is a practical, informative book written by a team of international authors with much experience in rare pulmonary diseases Orphan Lung Diseases: A Clinical Guide to Rare Lung Disease, second edition provides synthesized and easily accessible information about the main orphan lung diseases, to aid clinicians in charge of patients with rare disease, or who consider the diagnosis of a rare disease in their patient. It is a practical, informative guide written by a team of international experienced authors in rare pulmonary diseases.

This book has been replaced by "Rare Diseases of the Respiratory System", ISBN 978-1-84984-167-2 A respiratory physician is only likely to see a few orphan lung diseases each year or even during their career. However, it is essential that specialists are able identify, confirm and diagnosis orphan lung disease in a patient. This Monograph comprehensively covers the most common and/or complex orphan lung diseases. It should be seen as a solid companion to the respiratory specialist each time they need to consider a diagnosis of an orphan disease.

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!

Orphan Lung Diseases: A Clinical Guide to Rare Lung Disease provides a comprehensive, clinically focused textbook on rare and so-called ‘orphan’ pulmonary diseases. The book is oriented towards the diagnostic approach, including manifestations suggesting the disease, diagnostic criteria, methods of diagnostic confirmation, and differential diagnosis, with an overview of management.

European Respiratory Monograph (ERM) is the quarterly book series from the European Respiratory Society (ERS). Each Monograph covers a specific area of respiratory medicine, providing in-depth reviews that give clinicians at all levels a concise, comprehensive guide to symptoms, diagnosis and treatment.

Difficult to Diagnose Rare Diffuse Lung Disease presents the theoretical basis and practical aspects of differential diagnoses of rare lung diseases with the use of new method of probe-based confocal laser endomicroscopy (alveoscopy) of the distal respiratory region. Each chapter describes signs and symptoms of the disease and its typical and atypical manifestations. The book contains full color illustrations, including high-resolution histological microphotographs, CT-scans and confocal laser endomicroscopy images. In combination, these elements make this book an invaluable reference and guide for pulmonary researchers, pulmonologists, radiologists, and pathologists who wish to broaden their spectrum of knowledge in rare lung diseases. Highlights the new method of probe-based confocal laser endomicroscopy (alveoscopy) of the distal respiratory region, opening new horizons in the minimally invasive diagnosis of lung diseases Discusses current treatment strategies in accordance with clinical guidelines, including data from the latest clinical trials Presented in tabular format to aid in the diagnostic process

Diffuse parenchymal lung disease (DPLD) represents a large and heterogeneous group of disorders. Although new insights into the pathogenesis and new techniques such as high-resolution-CT have led to a better understanding of DPLD, clinical management remains a challenge. This volume is designed to become a valuable aid in the diagnosis and management of DPLD. It starts with general topics such as a new classification system for DPLD, the general diagnostic approach, and clinical evaluation of the patient by radiology, histological patterns and bronchoalveolar lavage. This is followed by consideration of some basic aspects such as the genetics of DPLD, the principles of granuloma formation, pulmonary fibrogenesis and vasculitis, as well as novel treatment approaches. A large part of the book consists of disease-specific chapters, which discuss granulomatous disorders, idiopathic pulmonary fibrosis and other entities of idiopathic interstitial pneumonia, the collagen vascular diseases, drug-induced infiltrative lung disease as well as orphan diffuse lung diseases including Langerhans' cell histiocytosis, lymphangioleiomyomatosis and pulmonary alveolar proteinosis. Special chapters on DPLD in children and lung transplantation for end-stage fibrosis complete this comprehensive publication. Written by internationally known experts, this well-organized volume is recommended to a wide audience including respiratory physicians in training, practice and research as well as radiologists and pulmonary pathologists.

Removing the guesswork associated with Interstitial Lung Disorders (ILDs) and bronchiolar disorders, Interstitial Pulmonary and Bronchiolar Disorders addresses the issues faced by pulmonologists in treating these disorders. Divided into sections based on the disease type (granulomatous, pneumonias, bronchiolar disorders, vasculitis, and orphan lung disease), each disorder is covered from epidemiological, pathogenic, clinical, and radiographic perspectives. The only text on the market today that deals with the entire spectrum of ILDs, this handy, one-stop reference includes: a special focus on treatment and the proper use of treatment options, including in depth coverage of the most common and potentially dangerous means of treatment emerging concepts in patient care discussion of lung diseases affecting the survivors of 9/11