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Cystic Fibrosis

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Table of Contents

Contributing Authors vPreface viiAcknowledgments ixIntroduction xiii1 The Basic Defect 1Daniel J. Weiner, Raymond A. Frizzell, and David M. Orenstein2 Making the Diagnosis 13Jonathan E. Spahr, David M. Orenstein, and Daniel J. Weiner3 The Respiratory System 21Daniel J. Weiner and David M. Orenstein4 The Gastrointestinal Tract 83Douglas S. Lindblad, Daniel J. Weiner, and David M. Orenstein5 Other Systems 103Daniel J. Weiner and David M. Orenstein6 Nutrition 113Judith A. Fulton, Iris Yann, Daniel J. Weiner, and David M. Orenstein7 Hospitalization and Other Special Treatments 135Jonathan E. Spahr, David M. Orenstein, Daniel J. Weiner,and Louise T. Bauer8 Transplantation 153Geoffrey Kurland, Jonathan E. Spahr, Daniel J. Weiner,and David M. Orenstein9 Daily Life 195Jonathan E. Spahr, David M. Orenstein, and Daniel J. Weiner10 Exercise 205David M. Orenstein and Daniel J. Weiner11 Genetics 221Barbara Karczeski, Garry R. Cutting, and David M. Orenstein12 The Family 235David M. Orenstein and Daniel J. Weiner13 The Teenage Years 245Jonathan E. Spahr, David M. Orenstein, and Daniel J. Weiner14 Cystic Fibrosis and Adulthood 265Joseph M. Piliewski, David M. Orenstein, Jonathan E. Spahr,and Daniel J. Weiner15 Death and Cystic Fibrosis 287Elisabeth P. Dellon and David M. Orenstein16 Research and Future Treatments 295Christopher Penland, David M. Orenstein, and Daniel J. Weiner17 The Cystic Fibrosis Foundation 311Robert J. Beall A P P E N D I C E SA Glossary of Terms 319B Medications 325C Airway Clearance Techniques 363D Some High Calorie Recipes 375E The History of Cystic Fibrosis 385F Bibliography and Resources 387G Cystic Fibrosis Care Centers in the United States 391H Cystic Fibrosis Care Centers Worldwide 411Index 445

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