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.

Background Information: Cystic fibrosis (CF) is the most common fatal genetic disease affecting Caucasians, with an incidence of 1:3000 live births. With the advances in treatment, there has been an important improvement in life expectancy. It is important for the radiologist to be familiar with the multi-systemic manifestations of CF, and to recognize its complications which will likely become more common as patients live longer. Educational Goals/Teaching Points: After reviewing this presentation, participants will be able to recognize the wide spectrum of imaging manifestations in adults with CF, as well as its most common complications. They will be aware of the appropriate radiological follow-up in these patients. They will learn how to improve their radiology reports to better help clinicians. Key Anatomic/Physiologic Issues and Imaging Findings/Techniques: We will begin with a brief review of the genetics and pathophysiology of CF. We will discuss the spectrum of imaging manifestations in adults using different modalities from X-rays to MRI, reviewing pulmonary as well as extrapulmonary disease. There will be a brief review of the suggestive signs of CF that can be observed with prenatal ultrasound. We will review the treatment approach, including lung transplantation and its complications in CF patients. We will make some suggestions to improve radiology reports by listing items that clinicians usually want to know with these patients. Conclusion: CF is a common genetic disease with which modern radiologists should be familiar, especially those working in endemic regions. Knowledge of its multi-systemic manifestations and most common complications is important to better help pneumologists and to provide appropriate patient care.

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 in the Light of New Research provides the latest research and clinical evidence that will be useful for clinicians, scientists and researchers to further their knowledge around this fascinating condition. The authors have brought along their expertise and wealth of knowledge to produce this book, including the basic science that underlies the disease, the burden of bacterial and viral infections, immunologic aspects of CF, a variety of clinical measurements to predict prognosis and novel therapies including gene therapy. This book will be invaluable and entertaining for anyone who is involved in the care of patients with cystic fibrosis.

Cystic fibrosis, a genetic disorder in children and young adults, is a multisystemic disease that mainly affects the lungs. Advances and improvements in the diagnosis and management of this condition have led to increased overall and symptom-free survival in cystic fibrosis patients. This book examines recent advances in the field and presents an evidence-based approach to the management of cystic fibrosis.

Cystic fibrosis (CF), also known as munoviscidosis, is a chronic genetic disease involving the dysfunction of the exocrine glands. This dysfunction is manifested as a deficiency in pancreatic enzymes, accumulation of mucous in airways, and excess salt in sweat. Cystic fibrosis was previously restricted to infancy and childhood but developments in medical treatment and therapy have extended survival considerably. This book includes within its scope research aimed at understanding the genetic linkage in cystic fibrosis, as well as improving the diagnosis and treatment of this disease in both children and adults. Leading-edge scientific research from throughout the world is presented.

Nutritional therapies have been key early interventions, and remain central to the well-being and survival of patients with cystic fibrosis. The nature of the disease causes significant alterations in a patient’s ability to process and assimilate nutrients. Furthermore, many factors contribute to higher metabolic demands throughout a patient’s life. In combination, maldigestion, malabsorption, and increased metabolic demands pose a high hurdle for the patient to overcome in order to maintain optimal nutritional status. Nutrition in Cystic Fibrosis: A Guide for Clinicians is an excellent resource for physicians, nurses and dietitians who deliver care for patients with cystic fibrosis. The book provides an introduction to cystic fibrosis and nutritional assessments. It will also serve as a comprehensive guide to the nutritional monitoring and management of patients with cystic fibrosis including special populations within cystic fibrosis that require additional considerations. The chapters are written by experts in their fields and include the most up to date scientific and clinical information. Nutrition in Cystic Fibrosis: A Guide for Clinicians targets pediatric and adult pulmonologists and gastroenterologists, residents and fellows, internists, pediatricians, nurses, dietitians and general practitioners who treat patients with cystic fibrosis.

Cystic Fibrosis: The Facts provides a much needed simple and understandable source book about this disease. It is aimed at those living with Cystic Fibrosis (CF), either themselves or members of their families or their friends. The book explains clearly what is happening to the body in CF, what causes it and what treatment options are available for the different aspects of the disease. There are more detailed chapters for those wanting to find out about the genetics of the disease and specific aspects such as how it affects life choices and employment. It looks to the future in terms of potential new therapies for CF and provides useful information on organizations that can provide help and further information across those areas of the world where the disease is prevalent.

This pocketbook is a concise companion for all health care professionals in respiratory medicine, paediatrics, and primary care who manage, or come across, patients with cystic fibrosis.