Cystic Fibrosis. Etiology, Diagnosis and Treatments

Book Description:

Cystic Fibrosis (also known as CF, mucovoidosis, or mucoviscidosis) is a hereditary disease affecting the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure. Abnormally thick mucus results in frequent lung infections. Diminished secretion of pancreatic enzymes is the main cause of poor growth, greasy stools, and deficiency in fat-soluble vitamins. Males can be infertile due to the condition congenital bilateral absence of the vas deferens. Often, symptoms of CF appear in infancy and childhood. Meconium ileus is a typical finding in newborn babies with CF.Individuals with CF can be diagnosed prior to birth by genetic testing. Newborn screening tests are increasingly common and effective(although false positives may occur, and children need to be brought in for a sweat test to distinguish disease vs carrier status). The diagnosis of CF may be confirmed if high levels of salt are found during a sweat test, although some false positives may occur.There is no known cure for CF. This new book presents important research in the field.

Editors: Paul N. Leatte

Table of Contents:

Preface

• Newborn screening for cystic fibrosis
  (Anne Deucher and Iris Schrijver, Departments of Pathology, Stanford University School of Medicine, Stanford, CA, and others)
• Cystic fibrosis and respiratory viral infections
  (Dennis Wat, Adult Cystic Fibrosis Unit, Papworth Hospital, Cambridge, United Kingdom)
• Cystic fibrosis, pseudomonas aeruginosa infection, and relevance of innate immune response: challenge for hematopoietic stem cell transplantation
  (Massimo Conese, Department of Biomedical Sciences, University of Foggia, Italy)
• Neutrophil elastase-mediated modulation of pathophysiology in cystic fibrosis lung disease
  (Xin Xu, German Luy, Janelle Chiasera, Amit Gaggar and Karen Bernard, University of Alabama at Birmingham, Birmingham, Alabama, and others)
• Genotypic hetereogeneity of the molecular basis of cystic fibrosis: the paradigm of lithuanian population genetic testing
  (Sergio Giannattasio, Antonella Bobba, Paolo Lattanzio, Nicoletta Guaragnella, Vaidutis Kucinskas, Ersilia Marra, CNR Istituto di Biomembrane e Bioenergetica, Italy, and others)
• Serum transferrin microheterogeneity in cystic fibrosis
  (E. Marklová, Z. Albahri, H. Vanícek1, V. Vávrová, Dept of Paediatrics, University Hospital, Hradec Králové, and others)
• Effects of the lack of transport of thiocyanate in cystic fibrosis lung disease
  (Melanie Childers, Alan Himmel, Jim Caldwell, Share International Foundation Sequim, WA)
• Treatments for cystic fibrosis: the role of adherence, importance and burden
  (Lynn B. Myers, Department of Psychology, School of Social Sciences, Brunel University, UK)
• Cystic Fibrosis – update on diagnosis and treatment
  (Melinda Solomon and Hartmut Grasemann, Division of Respiratory Medicine, Department of Paediatrics, The Hospital for Sick Children, University of Toronto and others)
• How effective are nutritional interventions in children and adults with cystic fibrosis?
  (Helen McCabe, Newcastle Upon Tyne Hospitals, UK and Helen White, Dept Nutrition and Dietetics, Leeds Metropolitan University, Leeds, UK)
• Sweat test: recommendations for good practice and analytical criteria of the methods used
  (Rota Michèle, Borgard Jean-Pierre, Sitruk-Khalfon Dominique, Marchand Martine Feldmann Delphine, Vassault Anne, Nguyen-Khoa Thao, Biochemistry Laboratory, France, and others)
• New trends in the inhibition of Pseudomonas aeruginosa quorum sensing activity
  (Mario Zucca, Sabrina Crivellaro, and Dianella Savoia, Department of Clinical and Biological Sciences, University of Torino, Italy)
• Ocular surface changes in patients with cystic fibrosis
  (Malgorzata Mrugacz, Department of Pediatric Ophthalmology Medical University of Bialystok, Poland)
• Pseudo-Bartter Syndrome in cystic fibrosis
  (Mehmet Kose, Nural Kiper, Department of Pediatrics, Erciyes University, and others)
• Management of cystic fibrosis-related arthritis
  (Judith Thornton, Satyapal Rangaraj and Daniel Hawley, Greater Manchester, Lancashire & South Cumbria Medicines for Children Research Network, Manchester, UK and others)

Index