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Here is a comprehensive, clinically focused review of rare and ´orphan´ pulmonary diseases. Covers diagnostic criteria, methods of confirmation and differential diagnosis, with an overview of management. Illustrations include high resolution CT chest imaging.

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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.

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1. Orphan Lung Diseases: From definition to organisation of Care.- 2. The Challenge of clinical research in orphan lung diseases.- 3. Chronic bronchiolitis in adults.- 4. Diffuse bronchiectasis of genetic origin.- 5. Allergic bronchopulmonary mycosis.- 6. Orphan Tracheopathies.- 7. Amyloidosis and the respiratory tract.- 8. Churg-Strauss syndrome: A clinical and therapeutic approach.- 9. Granunomatosis with polyangiitis (Wegener s granylomatosis).- 10. Alveolar Hemorrhage.- 11. Pulmonary Involvement in Takayasu Arteritis and Beh‡et Disease.- 12. Pulmonary vascular manifestations of hereditary hemorrhagic.- 13. Hepatopulmonary syndrome.- 14. Non-Langerhans cell histiocytosis -including Erdheim-Chester disease- and the lung.- 15. Idiopathic eosinophilic pneumonia.- 16. Multiple cystic lung disease.- 17. Lymphangioleiomyomatosis.- 18. Pulmonary alveolar Proteinosis.- 19. Exogenous lipoid pneumonia.- 20. Pulmonary alveolar microlithiasis.- 21. Pulmonary Fibrosis and the many faces of UIP.- 22. The syndrome of combined pulmonary fibrosis and emphysema.- 23. Non-specific, Unclassifiable, and Rare Idiopathic Interstitial Pneumonia.- 24. Organizing Pneumonias.- 25. Interstitial lung disease in systemic sclerosis.- 26. Interstitial lung disease in connective tissue diseases other than systemic sclerosis.- 27. Interstitial Lung Disease in Undifferentiated Forms of Connective Tissue Disease.- 28. Langerhans cell Granulomatosis and smoking-related interstitial lung diseases.- 29. Rare causes and the spectrum of hypersensitivity penumonitis.- 30. Chronic beryllium disease and other insterstitial lung diseases of occupational origin.- 31. Lymphoproliferative lung disorders.- 32. Pulmonary manifestations of hematological malignancies.- 33. Pulmonary hypertension in orphan lung diseases.- 34. Orphan and rare drug-induced and iatrogenic respiratory disease.- 35. From cancer mimicking orphan lung disease to orphan thoracic oncology.- 36. Rare Diffuse lung diseases of genetic origin.

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