This book is for every individual with cystic fibrosis and their families, and for the caregivers, researchers, and the many volunteers who have helped to further progress in the treatment and understanding of this disease. This book is also for historians and those interested in the story of a voluntary health organization whose insightful leadership successfully grew effective, wide ranging programs and developed a strategy of collaboration with associated agencies and interests that made for efficient and dramatic progress.

Cystic fibrosis used to be thought of as a respiratory and digestive disease, with a uniformly and rapidly fatal outcome. The spectrum of the disease has broadened into the mild atypical case, presenting in middle age, with the potential for complications in virtually every system of the body. In the past few years there has been an explosion of knowledge of the basic science of the defect. The editors have therefore invited the leading scientists and clinicians in the field of cystic fibrosis to describe the recent advances in this disease. Although there are many 'Recent Advances' texts, previous books have been selective in their choice of topics. This book is the first to cover the entire field of this complex disease, and encompasses the rapidly moving topics of the basic molecular and cellular biology as well as the recent multi-system, multi-disciplinary advances in the clinical care of patients. The authors have been charged with writing only about new developments and not to rehash old literature. The bulk of the references is therefore less than five years old. This book addresses all professionals who treat cystic fibrosis and want to have an up-date of new findings in the field, particularly of those outside their immediate specialisation. It will also be useful for basic researchers interested in related scientific areas and the clinical context of their work.

Cystic Fibrosis has seen dramatic advances in treatment since the last edition, including targeted cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators for most CFTR gene abnormalities. This new fifth edition is an update and expansion of the rapid clinical and scientific advances in improving prognosis, and the impact of COVID-19, all of which has transformed conventional models of care. It covers basic science, such as how detailed understanding of the biology of the CFTR gene and protein has led to novel and beneficial therapies, as well as all aspects of clinical management in high-, middle- and low-income settings and the voices of individuals with CF from across the world. It will be a useful reference for clinicians, including all levels of trainees, across the whole multidisciplinary team, scientists and students. Key Features • Follows an appealing organization of chapters, by developing fundamental knowledge of the reader before moving on to more complex or developing topics. • Presents a comprehensive, authoritative and up-to-date text, integrating fundamental science and clinical aspects of cystic fibrosis providing an attractive read for clinicians, trainee doctors and scientists. • Draws on global expertise and reflects best evidence-based practice from experts conducting cutting-edge clinical and basic science research from around the world.

This international and authoritative work, which brings together current knowledge in the field of cystic fibrosis, has become established in previous editions as a leading reference in the field. The third edition continues to provide everything that the clinician or allied health professional treating patients with cystic fibrosis will need in a single manageable volume. Thoroughly revised and updated throughout, it reflects the significant advances that have been made in the field since the second edition published in 2000. Cystic Fibrosis evaluates in detail the basic science that underlies the disease and its progression, putting it into a clinical context. Diagnostic and clinical aspects are covered in depth, as are monitoring the condition and the importance of multi-disciplinary care, reflected in the sections into which the new edition has been sub-divided to improve accessibility. Future developments, including novel therapies, are covered in a concluding section. The clinical areas have been much expanded, with the introduction of separate chapters covering sleep, lung mechanics and the work of breathing, upper airway disease, insulin deficiency and diabetes, bone disease, and sexual and reproductive issues. A new section on monitoring discusses the use of databases to improve patient care, and covers monitoring in different age groups, exercise testing and the outcomes of clinical trials in these areas. Separate chapters are devoted to paramedical issues, including nursing, physiotherapy, psychology, and palliative and spiritual care. Throughout, the emphasis is on providing an up-to-date and balanced review of both the clinical and basic sciences aspects of the subject, and to reflect the multi-disciplinary nature of the cystic fibrosis care team. Drawing on the expertise of a team of international specialists from a variety of backgrounds, the third edition of Cystic Fibrosiswill continue to find a broad readership among respiratory physicians, paediatricians, specialist nurses and other health professionals working with patients with cystic fibrosis.

20th Century Revolutions in Technology

Cystic fibrosis (CF) is one of the most common fatal hereditary diseases. The discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the stage for unraveling the pathogenesis of CF lung disease, continuous refinement of symptomatic treatments and the development of mutation-specific therapies, which are now becoming available for a subgroup of patients. This ERS Monograph provides an update on all aspects of CF lung disease, from infancy to adulthood, including current concepts on disease process, improvements in early diagnosis and monitoring, therapeutic approaches, and patient care. The book highlights important recent developments and discusses the next steps that will be required for further improvement of the life expectancy and quality of life of CF patients. It will be an essential reference for basic and clinical scientists and all members of the CF team.

Vols. for 1963- include as pt. 2 of the Jan. issue: Medical subject headings.

Respiratory disorders in infants and children are challenging problems for every clinician involved in the management of these patients. This book summarises recent advances in the management of children. The scholarly contributions of the distinguished contributors represent a careful and comprehensive synthesis of current knowledge in pathogenesis, diagnosis and treatment.

This book chronicles the life and accomplishments of Dorothy Hansine Andersen, a pioneering American pathologist and pediatrician who was the first person to define, diagnose, and treat cystic fibrosis. Divided into three parts, the book begins by detailing Anderson’s early life, including being orphaned as an adolescent, her college career, and her laborious start in the medical field. Part II then examines Andersen’s role in defining the new disease “cystic fibrosis of the pancreas” and her career of active engagement in various clinical pursuits and research, both in pathology and pediatrics. Chapters in this section also discuss the numerous attempts made by others to minimize Andersen’s work through gender bias and the Matilda Effect. The book concludes by reviewing the foundations laid for CF, Andersen’s legacy, and her terminal illness. Featuring an engaging narrative style, Dorothy Hansine Andersen is a historically relevant, invaluable text for anyone interested in the life of Dorothy Anderson and the nascence of cystic fibrosis diagnoses.