Open Access Medical Books



Edited by Dinesh Sriramulu .

Open Access .

The book ‘Cystic Fibrosis – Renewed Hopes Through Research’ emerged as a result of the contribution of interesting chapters by the experts in response to the invitation by Intech Open Access Publisher. This open-to-all initiative enables dissemination of scientific knowledge to a wide range of readers through an unrestricted access to book contents. Publication via virtual media enables constant update of information on a regular basis whenever appropriate.
Cystic fibrosis, also known as mucoviscidosis, is an autosomal recessive multi-system genetic disease that occurs predominantly among Caucasians. Though found highest among Irish population, the incidence of this disease among Africans and Asians, who are normally least-affected, is on the rise probably due to social aspects and it is also attributable to improved early diagnostic methods available. This disease is characterized by an abnormal transport of chloride and sodium across epithelium that leads to thickening of secretions mainly in the lungs, pancreas, liver and intestine. The multifarious nature of this disease pushes individuals with cystic fibrosis towards mortality.
Unlike in the classical textbook, the collection of chapters in this book explains the complexity of the disease, microbiology and immunological aspects, therapeutic options and disease management that encompasses topics from basic science to patient care. The contributors of chapters of this book are well-established members of the worldwide scientific community, which is focused on an ultimate goal of finding a cure for cystic fibrosis condition. Recent developments, in the study and treatment of disease organized in the form of chapters in the book, emphasizes the dominating role of interdisciplinary participation. Initial chapters of the book focused on topics dedicated to prognosis, radiology, gender differences and reproductive complications associated with the disease. It is followed by chapters on genetic, biochemical and molecular aspects of the disease target, the Cystic Fibrosis Transmembrane conductance Regulator protein channel. The collection of chapters under microbiology and immunology covers typical and atypical infections that are secondary in nature and one of the major causative of mortality of cystic fibrosis patients. A special emphasis has been given to therapeutic developments to include recent findings with respect to steps towards cure. This set of chapters show the inevitable need for an interdisciplinary approach in cystic fibrosis research. The final part of the book is dedicated to focus on the importance of management aspects in order to achieve success in the treatment of this complex disease and care of patients.
More recent findings, especially at the molecular level, have changed our knowledge and understanding about cystic fibrosis and have dramatically increased the life expectancy of the patients. Owing to the pleiotropic nature of cystic fibrosis condition, medical research should focus more on translational research, then finding a common cure for cystic fibrosis will be attainable in the near future. With this approach, a cohort- or patient-specific treatment strategy backed by an intense bench-to-bedside research flow is the only feasible option to cater to the needs of patients in order to lead, at least, a near-normal life. After all, by definition, “Health is a state of completephysical, mental and social well-being, and not merely the absence of a disease or infirmity.”
On behalf of me and the publisher, we anticipate that this book will instill the interest of readers in understanding the clinical and social aspects of a complex disease such as cystic fibrosis.
I humbly thank the INTECH for appointing me as the Editor of this book and for publishing the same. I thank all the authors for their valuable contributions without which this book would not have been materialized. I would like to thank Helmholtz Centre for Infection Research – Braunschweig, Germany, which served as the knowledge centre for me to gain expertise in this field. Finally, I thank Dr. Lakshmy  Dinesh for her continuous support in shaping various aspects of this book.

Dinesh D. Sriramulu

Helmholtz Centre for Infection Research, Braunschweig,


Part 1 Cystic Fibrosis – A Complex Syndrome.

  1 The Prognosis of Cystic Fibrosis – 
A Clinician’s Perspective 3 Patrick Lebecque

  2 Radiological Features of Cystic Fibrosis 31 Iara Maria Sequeiros and Nabil A. Jarad

  3 The Cystic Fibrosis ‘Gender Gap’: Past Observations 
Present Understanding and Future Directions 51 Sanjay H. Chotirmall, Catherine M. Greene, Brian J. Harvey and Noel G. McElvaney

  4 Cystic Fibrosis and Infertility 67 Maria do Carmo Pimentel Batitucci, Angela Maria Spagnol Perrone and Giselle Villa Flor Brunoro

Part 2 CFTR – Genetics and Biochemistry .

  5 The Genetics of CFTR: Genotype – Phenotype Relationship, 
Diagnostic Challenge and Therapeutic Implications 91 Marco Lucarelli, Silvia Pierandrei, Sabina Maria Bruno and Roberto Strom

  6 Biochemical and Molecular Genetic Testing Used in 
the Diagnosis and Assessment of Cystic Fibrosis 123 Donovan McGrowder

Part 3 Microbiology and Immunology .

  7 Pseudomonas aeruginosa Biofilm Formation in 
the CF Lung and Its Implications for Therapy 153 Gregory G. Anderso

  8 Outcome and Prevention of Pseudomonas aeruginosa- 
Staphylococcus aureus Interactions During Pulmonary Infections in Cystic Fibrosis 181 Gabriel Mitchell and François Malouin

  9 Infection by Non Tuberculous 
Mycobacteria in Cystic Fibrosis 207 María Santos, Ana Gil-Brusola and Pilar Morales

  10 Atypical Bacteria in the CF Airways: Diversity, 
Clinical Consequences, Emergence and Adaptation 225 Marchandin Hélène, Michon Anne-Laure and Jumas-Bilak Estelle

  11 Viral Respiratory Tract Infections in Cystic Fibrosis 253 
Dennis Wat

  12 Immune Dysfunction in Cystic Fibrosis 273 Yaqin Xu and Stefan Worgall

Part 4 Therapeutic Options .

  13 Channel Replacement Therapy for Cystic Fibrosis 291 
John M. Tomich, Urška Bukovnik, Jammie Layman and Bruce D. Schultz

  14 Improving Cell Surface Functional Expression of F508 CFTR:
A Quest for Therapeutic Targets 333 Yifei Fan, Yeshavanth K. Banasavadi-Siddegowda and Xiaodong Wang

  15 Fine Tuning of CFTR Traffic and 
Function by PDZ Scaffolding Proteins 359 Florian Bossard, Emilie Silantieff and Chantal Gauthier

  16 CFTR Gene Transfer and Tracking 
the CFTR Protein in the Airway Epithelium 379 Gaëlle Gonzalez, Pierre Boulanger and Saw-See Hong

  17 VIP as a Corrector of CFTR Trafficking and Membrane Stability 397 Valerie Chappe and Sami I. Said

  18 Pharmacological Potential of 
PDE5 Inhibitors for the Treatment of Cystic Fibrosis 413 Bob Lubamba, Barbara Dhooghe, Sabrina Noël and Teresinha Leal

  19 Pharmacological Modulators of Sphingolipid Metabolism 
for the Treatment of Cystic Fibrosis Lung Inflammation 439 M.C. Dechecchi, E. Nicolis, P. Mazzi, M. Paroni, F. Cioffi, A. Tamanini,V. Bezzerri, M. Tebon, I. Lampronti, S. Huang, L. Wiszniewski, M.T. Scupoli, A. Bragonzi, R. Gambari, G. Berton and G. Cabrini

Part 5 Disease Management .

  20 The Importance of Adherence and 
Compliance with Treatment in Cystic Fibrosis 455 Rosa Patricia Arias-Llorente, Carlos Bousoño García and Juan J. Díaz Martín

  21 Improving the Likelihood of Success in Trials and 
the Efficiency of Delivery of Mucolytics and Antibiotics 473 Carlos F. Lange

  22 Airways Clearance Techniques in Cystic Fibrosis:
Physiology, Devices and the Future 493 Adrian H. Kendrick

  23 The Physiotherapist’s Use of Exercise in 
the Management of Young People with Cystic Fibrosis 519 Allison Mandrusiak and Pauline Watter

  24 Exercise Performance and 
Breathing Patterns in Cystic Fibrosis 541 Georgia Perpati . 

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Published by: Unknown - Saturday, January 26, 2013


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