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This book is a comprehensive reference on diffuse cystic lung diseases (DCLDs). DCLDs are a group of pathophysiologically heterogenous processes that are characterized by the presence of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the pulmonary parenchyma. In recent years, tremendous advancements have been made in these diseases leading to improved understanding of the underlying pathophysiology, and improved outcomes with targeted therapies. The authors, who are leading experts in the field, delineate DCLDs as a separate category distinct from other interstitial lung diseases, and have created this textbook specifically dedicated to this disease group.
This book begins with a chapter introducing the definition and classification of DCLDs. Subsequent chapters address the pathogenic mechanisms underlying pulmonary cyst formation and provide a detailed overview of the radiological and pathological features of DCLDs. The common as well as uncommon causes of DCLDs are comprehensively reviewed in individual chapters, as are the varied clinical presentations and extrapulmonary manifestations, and approaches to management and treatment. The book culminates in a final chapter that presents a practical algorithmic approach to diagnosis that progresses from least invasive to most invasive approaches. This textbook provides a one-stop, comprehensive and integrated, clinical, radiologic, and pathologic overview of DCLDs that will be as useful to the practicing clinician as it is to the clinical investigator.
List of contents
Definition and Classification of Diffuse Cystic Lung Diseases.- Mechanisms of Lung Cyst Formation.- Radiologic Evaluation of Cystic Lung Disease.- Pathological Evaluation of Pulmonary Cysts.- Pathophysiology of Lymphangioleiomyomatosis.- Smoking-induced Diffuse Cystic Lung Diseases.- Birt-Hogg-Dubé Syndrome.- Lymphocytic Interstitial Pneumonitis and Follicular Bronchiolitis.- Amyloidosis and Immunoglobulin Deposition Disease.- Infectious Etiologies of Diffuse Cystic Lung Diseases.- Neoplasms that Present as Multicystic Lung Disease.- Congenital and Developmental Causes of Cystic Lung Disease.- Rare Causes of Cystic Lung Disease.- Mimics, Impersonators and Semblances of Pulmonary Cysts.- Extrapulmonary Manifestations of Diffuse Cystic Lung Diseases.- Clinical Presentation and Management of Pleural Disease in Diffuse Cystic Lung Diseases.- Air Travel in Diffuse Cystic Lung Diseases.- Approach to the Diagnosis and Management of Diffuse Cystic Lung Diseases.
About the author
Nishant Gupta, MD, MS is Associate Professor in the Division of Pulmonary, Critical Care, and Sleep Medicine at the University of Cincinnati. He is the Director of the Interstitial Lung Disease Program at the University of Cincinnati and the International Rare Lung Disease Clinic Network.
Kathryn A. Wikenheiser-Brokamp, MD, PhD is Associate Professor in the Division of Pathology & Laboratory Medicine and the Perinatal Institute Division of Pulmonary Biology at Cincinnati Children's Hospital Medical Center. She is also Associate Professor in the Department of Pathology & Laboratory Medicine at the University of Cincinnati and Pulmonary Pathologist for the Interstitial Lung Disease program at the University of Cincinnati.
Francis X. McCormack, MD is Taylor Professor and Director of the Division of Pulmonary, Critical Care, and Sleep Medicine at the University of Cincinnati. He is also volume editor of
Molecular Basis of Pulmonary Disease (2010).
