Open access peer-reviewed Edited Volume

Cystic Fibrosis - Facts, Management and Advances

Prashant Mohite

Royal Brompton and Harefield NHS Foundation Trust


Anna Reed

Royal Brompton and Harefield NHS Foundation Trust

Andre Simon

Royal Brompton and Harefield NHS Foundation Trust


Cystic Fibrosis Genetic Disorder CFTR Protein Pathophysiology Diagnosis Respiratory System Complications Digestive System Chronic Stable Disease Acute Exacerbations

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About the book

Cystic fibrosis is a genetic disorder affecting multiple systems in the body with various levels of severity. Over the last few decades the patterns of presentation, the aspects of diagnosis and approaches to its management have changed substantially. Rather an acute disease in the children it is now a chronic condition in the adults. In the developed world there are more adults than children affected with the disease now. As more affected children are reaching adulthood fighting the early encroachments of the disease, the burden on the healthcare is ever increasing given the natural history of cystic fibrosis that is riddled with exacerbations and complications requiring constant clinical input, healthcare professional visits, and frequent hospital admissions.

The treatment of cystic fibrosis has also displayed a monumental change over the years. The gene therapy that remained on the horizon for some good years is now a reality in clinical practice. The current management is focused on preventing disease complications rather than treating them.Today the medical fraternity is facing new challenges in the management of cystic fibrosis. The disease is exhibiting as a chronic affair with slow-deteriorating stable phases interspersed with acute exacerbations. This mandates a close observation and timely intervention at early signs of exacerbations to prevent catastrophic complications. The cystic fibrosis transforms lungs into a thriving bed for the variety of microbial flora that slowly develops resistance to multiple antibiotics. Advances in the lung transplantation technique, immunosuppression as well as post-operative management have led to an increase in the survival of lung transplant recipients qualifying more and more recipients as a candidate for re-transplantation.

Cystic fibrosis affecting multiple systems naturally involves multiple specialties in its management - genetics, microbiology, neonatology, pediatrics, diabetology, gastroenterology, infertility, psychiatry, respiratory, thoracic surgery, and transplant surgery are some to be named. This book is intended for the clinicians, medical professionals, healthcare assistants and trainees in these specialties. With few available books covering this subject, this book aims to be different. Chapters should cover the topics in entirety without missing onto the basics, at the same time reaching out to the recent advances in the field. Chapters should show uniformity, consistency and similar standards in their presentation and material and therefore authors are urged to follow the general guidelines of authorship.

Publishing process

Book initiated and editor appointed

Date completed: November 6th 2019

Applications to edit the book are assessed and a suitable editor is selected, at which point the process begins.

Chapter proposals submitted and reviewed

Deadline Extended: Open for Submissions

Potential authors submit chapter proposals ready for review by the academic editor and our publishing review team.

Approved chapters written in full and submitted

Deadline for full chapters: May 1st 2020

Once approved by the academic editor and publishing review team, chapters are written and submitted according to pre-agreed parameters

Full chapters peer reviewed

Review results due: July 20th 2020

Full chapter manuscripts are screened for plagiarism and undergo a Main Editor Peer Review. Results are sent to authors within 30 days of submission, with suggestions for rounds of revisions.

Book compiled, published and promoted

Expected publication date: September 18th 2020

All chapters are copy-checked and typesetted before being published. IntechOpen regularly submits its books to major databases for evaluation and coverage, including the Clarivate Analytics Book Citation Index in the Web of ScienceTM Core Collection. Other discipline-specific databases are also targeted, such as Web of Science's BIOSIS Previews.

About the editor

Prashant Mohite

Royal Brompton and Harefield NHS Foundation Trust

Dr. Prashant Mohite is a qualified cardiothoracic surgeon currently working as a Specialty Doctor in Heart and Lung Transplantation at Royal Brompton and Harefield Foundation Trust, London. He has been involved in the development of several programs at Harefield Hospital including Organ Care Systems for heart and lungs, Ex-vivo lung perfusion, Awake ECLS, ECLS as a bridge to lung transplant and DCD heart transplantation. He is active in research, has represented my Institute in multiple national and overseas meetings as an invited speaker as well as through voluntary abstract submissions. He is always keen to share the experience, has published more than 125 articles (Orcid id: 0000-0001-5533-3089) in renowned journals and am a frequent reviewer for some of them. He is also an experienced reviewer and has been frequently chosen as a reviewer by renowned journals including the Journal of heart-lung transplantation, Transplant International, Artificial organs. He is closely studying Cystic Fibrosis patients as they are referred to our institute for medicinal as well as surgical management.

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Introducing your Author Service Manager

Mr. Mateo Pulko

As an Author Service Manager my responsibilities include monitoring and facilitating all publishing activities for authors and editors. From chapter submission and review, to approval and revision, copyediting and design, until final publication, I work closely with authors and editors to ensure a simple and easy publishing process. I maintain constant and effective communication with authors, editors and reviewers, which allows for a level of personal support that enables contributors to fully commit and concentrate on the chapters they are writing, editing, or reviewing. I assist authors in the preparation of their full chapter submissions and track important deadlines and ensure they are met. I help to coordinate internal processes such as linguistic review, and monitor the technical aspects of the process. As an ASM I am also involved in the acquisition of editors. Whether that be identifying an exceptional author and proposing an editorship collaboration, or contacting researchers who would like the opportunity to work with IntechOpen, I establish and help manage author and editor acquisition and contact.

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