Cystic fibrosis adult care: consensus conference report.

2004;125;1S-39S Chest and David Rodman James R. Yankaskas, Bruce C. Marshall, Beth Sufian, Richard H. Simon * Cystic Fibrosis Adult Care html http://chestjournal.chestpubs.org/content/125/1_suppl/1S.full. and services can be found online on the World Wide Web at: The online version of this article, along with updated information ISSN:0012-3692 ) http://chestjournal.chestpubs.org/site/misc/reprints.xhtml ( of the copyright holder. may be reproduced or distributed without the prior written permission Northbrook, IL 60062. All rights reserved. No part of this article or PDF by the American College of Chest Physicians, 3300 Dundee Road, 2004 Physicians. It has been published monthly since 1935. Copyright CHEST is the official journal of the American College of Chest

[1]  J. Lloyd-Still,et al.  Crohn's disease and cystic fibrosis , 1994, Digestive Diseases and Sciences.

[2]  B. Thorsteinsson,et al.  Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis , 1992, European Journal of Pediatrics.

[3]  S. Dutta,et al.  Critical examination of therapeutic efficacy of a ph-sensitive enteric-coated pancreatic enzyme preparation in treatment of exocrine pancreatic insufficiency secondary to cystic fibrosis , 1988, Digestive Diseases and Sciences.

[4]  W. Walker Pediatric Gastrointestinal Disease , 2004 .

[5]  F. Ratjen,et al.  A double-blind placebo controlled trial with oral ambroxol and N-acetylcysteine for mucolytic treatment in cystic fibrosis , 1985, European Journal of Pediatrics.

[6]  R. Glass,et al.  Bone changes associated with cystic fibrosis , 2004, Skeletal Radiology.

[7]  Susan M Sawyer,et al.  Transition to adult health care for adolescents and young adults with chronic conditions: position paper of the Society for Adolescent Medicine. , 2003, The Journal of adolescent health : official publication of the Society for Adolescent Medicine.

[8]  L. Saiman,et al.  Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. , 2003, JAMA.

[9]  P. Sammut,et al.  Effectiveness and tolerability of high‐dose salmeterol in cystic fibrosis , 2002, Pediatric pulmonology.

[10]  K. Kerr,et al.  Transmission of colistin‐resistant Pseudomonas aeruginosa between patients attending a pediatric cystic fibrosis center , 2002, Pediatric pulmonology.

[11]  M. Rosenthal,et al.  Long term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trial , 2002, The Lancet.

[12]  M. Hodson,et al.  A randomised clinical trial of nebulised tobramycin or colistin in cystic fibrosis , 2002, European Respiratory Journal.

[13]  M. Corey,et al.  How useful is fecal pancreatic elastase 1 as a marker of exocrine pancreatic disease? , 2002, The Journal of pediatrics.

[14]  D. Salvatore,et al.  Effects of salmeterol on arterial oxyhemoglobin saturations in patients with cystic fibrosis , 2002, Pediatric pulmonology.

[15]  S. Bell,et al.  Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial , 2002, Thorax.

[16]  F R Adler,et al.  Survival effect of lung transplantation among patients with cystic fibrosis. , 2001, JAMA.

[17]  J. P. Sy,et al.  A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. , 2001, The Journal of pediatrics.

[18]  W. Wells,et al.  Living donor lung transplantation: selection, technique, and outcome. , 2001, Transplantation proceedings.

[19]  H. Chaun,et al.  Colonic disorders in adult cystic fibrosis. , 2001, Canadian journal of gastroenterology = Journal canadien de gastroenterologie.

[20]  J. Lipuma,et al.  Disproportionate distribution of Burkholderia cepacia complex species and transmissibility markers in cystic fibrosis. , 2001, American journal of respiratory and critical care medicine.

[21]  M. Corey,et al.  Treatment of vitamin K deficiency in cystic fibrosis: Effectiveness of a daily fat-soluble vitamin combination. , 2001, The Journal of pediatrics.

[22]  L. Wong,et al.  Long-term comparative trial of positive expiratory pressure versus oscillating positive expiratory pressure (flutter) physiotherapy in the treatment of cystic fibrosis. , 2001, The Journal of pediatrics.

[23]  J. Malagelada,et al.  Gastrointestinal, Liver, and Pancreatic Involvement in Adult Patients with Cystic Fibrosis , 2001, Pancreas.

[24]  S. Cunningham,et al.  Bronchoconstriction following nebulised colistin in cystic fibrosis , 2001, Archives of disease in childhood.

[25]  N. Chalasani,et al.  A meta‐analysis of endoscopic variceal ligation for primary prophylaxis of esophageal variceal bleeding , 2001, Hepatology.

[26]  J. Adams,et al.  Effect of intravenous pamidronate on bone mineral density in adults with cystic fibrosis , 2001, Thorax.

[27]  A. Galli-Tsinopoulou,et al.  Long-term Prospective Study of the Effect of Ursodeoxycholic Acid on Cystic Fibrosis-related Liver Disease , 2001, Journal of clinical gastroenterology.

[28]  J. Luce,et al.  Perspectives on care at the close of life. Management of dyspnea in patients with far-advanced lung disease: "once I lose it, it's kind of hard to catch it... ". , 2001, JAMA.

[29]  B. Hollis,et al.  Diminished and erratic absorption of ergocalciferol in adult cystic fibrosis patients. , 2001, The American journal of clinical nutrition.

[30]  T. Liou,et al.  Predictive 5-year survivorship model of cystic fibrosis. , 2001, American journal of epidemiology.

[31]  J. Yankaskas,et al.  Outcomes of intensive care unit care in adults with cystic fibrosis. , 2001, American journal of respiratory and critical care medicine.

[32]  P. Wilcox,et al.  Predictability of oxygen desaturation during sleep in patients with cystic fibrosis : clinical, spirometric, and exercise parameters. , 2001, Chest.

[33]  H. Becker,et al.  Low-flow oxygen and bilevel ventilatory support: effects on ventilation during sleep in cystic fibrosis. , 2001, American journal of respiratory and critical care medicine.

[34]  A. Knox,et al.  Respiratory diseases in pregnancy c 4 Series , 2001 .

[35]  A. Togias,et al.  Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population. , 2000, JAMA.

[36]  J. Milunsky,et al.  Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes. , 2000, Chest.

[37]  A. Ionescu,et al.  Bone density, body composition, and inflammatory status in cystic fibrosis. , 2000, American journal of respiratory and critical care medicine.

[38]  J. Renner,et al.  Efficacy of pamidronate for osteoporosis in patients with cystic fibrosis following lung transplantation. , 2000, American journal of respiratory and critical care medicine.

[39]  E. Widerman,et al.  Health status and sociodemographic characteristics of adults receiving a cystic fibrosis diagnosis after age 18 years. , 2000, Chest.

[40]  S. Williams,et al.  Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. , 2000, Radiographics : a review publication of the Radiological Society of North America, Inc.

[41]  P. Jowell,et al.  Cystic fibrosis mutations and genetic predisposition to idiopathic chronic pancreatitis. , 2000, The Medical clinics of North America.

[42]  R. Prescott,et al.  Elective versus symptomatic antibiotic treatment in cystic fibrosis patients with chronic Pseudomonasinfection of the lungs , 2000, Thorax.

[43]  M R Kosorok,et al.  Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. , 2000, The New England journal of medicine.

[44]  M. Corey,et al.  A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. , 2000, The Journal of pediatrics.

[45]  S. Conway,et al.  Evaluation of fecal pancreatic elastase‐1 as a measure of pancreatic exocrine function in children with cystic fibrosis , 2000, Pediatric pulmonology.

[46]  T. Therneau,et al.  Ursodeoxycholic acid for primary biliary cirrhosis , 2000, The Lancet.

[47]  D. Frangolias Osteoporosis in cystic fibrosis : pathogenesis and clinical features , 2000 .

[48]  W. Warwick,et al.  Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. , 2000, American journal of respiratory and critical care medicine.

[49]  S. Veeraraghavan,et al.  Lung transplantation for cystic fibrosis: an update and practical considerations for referring candidates. , 1999, Current opinion in pulmonary medicine.

[50]  J. Adams,et al.  Low bone mineral density in adults with cystic fibrosis , 1999, Thorax.

[51]  D. Johns,et al.  Hypoxemia and hypercapnia during exercise and sleep in patients with cystic fibrosis. , 1999, Chest.

[52]  A. Quittner,et al.  Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report. , 1999, Diabetes research and clinical practice.

[53]  B. Dousset,et al.  Outcome of cystic fibrosis-associated liver cirrhosis: management of portal hypertension. , 1999, Journal of hepatology.

[54]  L. Tsui,et al.  Proportion of cystic fibrosis gene mutations not detected by routine testing in men with obstructive azoospermia. , 1999, JAMA.

[55]  P. Hanly,et al.  Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia. , 1999, Chest.

[56]  N. S. Murthy,et al.  Comparison of endoscopic ligation and propranolol for the primary prevention of variceal bleeding. , 1999, The New England journal of medicine.

[57]  R. Stern,et al.  Timing of referral for lung transplantation for cystic fibrosis: overemphasis on FEV1 may adversely affect overall survival. , 1999, Chest.

[58]  L. Donnelly,et al.  Atypical presentation of Clostridium difficile colitis in patients with cystic fibrosis. , 1999, AJR. American journal of roentgenology.

[59]  Murray D. Altose,et al.  Dyspnea. Mechanisms, assessment, and management: a consensus statement. American Thoracic Society. , 1999, American journal of respiratory and critical care medicine.

[60]  M S Pepe,et al.  Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group. , 1999, The New England journal of medicine.

[61]  P. Sammut,et al.  Effects of standard and high doses of salmeterol on lung function of hospitalized patients with cystic fibrosis , 1999, Pediatric pulmonology.

[62]  S. Williams,et al.  Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis , 1999, Gut.

[63]  R. Sokol,et al.  Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. , 1999, Journal of pediatric gastroenterology and nutrition.

[64]  R. Aris,et al.  Altered Calcium Homeostasis in Adults with Cystic Fibrosis , 1999, Osteoporosis International.

[65]  G. Giles,et al.  CFTR ΔF508 carrier status, risk of breast cancer before the age of 40 and histological grading in a population‐based case‐control study , 1998 .

[66]  J. Elborn,et al.  Nutrition in adults with cystic fibrosis. , 1998, Clinical nutrition.

[67]  M. Knowles,et al.  Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. , 1998, The New England journal of medicine.

[68]  M. Schwarz,et al.  Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. , 1998, The New England journal of medicine.

[69]  B. Marshall,et al.  Basic therapies in cystic fibrosis. Does standard therapy work? , 1998, Clinics in chest medicine.

[70]  W. Levinson,et al.  Detection of prostate and colon cancer. , 1998, JAMA.

[71]  J. Emerson,et al.  Microbiology of sputum from patients at cystic fibrosis centers in the United States. , 1998, Clinical infectious diseases : an official publication of the Infectious Diseases Society of America.

[72]  M. Estenne,et al.  International guidelines for the selection of lung transplant candidates. The International Society for Heart and Lung Transplantation, the American Thoracic Society, the American Society of Transplant Physicians, the European Respiratory Society. , 1998, Heart & lung : the journal of critical care.

[73]  A. Moran,et al.  Abnormal glucose metabolism in cystic fibrosis. , 1998, The Journal of pediatrics.

[74]  R. Lindsay,et al.  Bone mass and vitamin D deficiency in adults with advanced cystic fibrosis lung disease. , 1998, American journal of respiratory and critical care medicine.

[75]  S. Kudoh,et al.  Improvement of survival in patients with diffuse panbronchiolitis treated with low-dose erythromycin. , 1998, American journal of respiratory and critical care medicine.

[76]  W J Warwick,et al.  Risk of death in cystic fibrosis patients with severely compromised lung function. , 1998, Chest.

[77]  M. Corey,et al.  Uncertainty in the diagnosis of cystic fibrosis: possible role of in vivo nasal potential difference measurements. , 1998, The Journal of pediatrics.

[78]  G. Cutting,et al.  The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. , 1998, The Journal of pediatrics.

[79]  M. Rosenthal,et al.  Long-term azithromycin may improve lung function in children with cystic fibrosis , 1998, The Lancet.

[80]  J. Renner,et al.  Increased Rate of Fractures and Severe Kyphosis: Sequelae of Living into Adulthood with Cystic Fibrosis , 1998, Annals of Internal Medicine.

[81]  F. Accurso,et al.  Enteral nutrition for patients with cystic fibrosis: comparison of a semi-elemental and nonelemental formula. , 1998, The Journal of pediatrics.

[82]  P. König,et al.  A placebo‐controlled, double‐blind trial of the long‐term effects of albuterol administration in patients with cystic fibrosis , 1998, Pediatric pulmonology.

[83]  B. Strandvik,et al.  A two‐year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis–associated liver disease , 1998, Hepatology.

[84]  M. Walshaw,et al.  Prevalence and Mechanisms of Gastro-Oesophageal Reflux in Adult Cystic Fibrosis Patients , 1998, Journal of the Royal Society of Medicine.

[85]  R. Sokol,et al.  Effect of ursodeoxycholic acid therapy on hepatic function in children with intrahepatic cholestatic liver disease. , 1998, Journal of pediatric gastroenterology and nutrition.

[86]  Keith McNeil,et al.  International guidelines for the selection of lung transplant candidates. The American Society for Transplant Physicians (ASTP)/American Thoracic Society(ATS)/European Respiratory Society(ERS)/International Society for Heart and Lung Transplantation(ISHLT). , 1998, American journal of respiratory and critical care medicine.

[87]  M. Häusler,et al.  First adult patient with fibrosing colonopathy , 1998, American Journal of Gastroenterology.

[88]  J. Yankaskas,et al.  Lung transplantation in cystic fibrosis: consensus conference statement. , 1998, Chest.

[89]  L. Wong,et al.  Long-term comparative trial of conventional postural drainage and percussion versus positive expiratory pressure physiotherapy in the treatment of cystic fibrosis. , 1997, The Journal of pediatrics.

[90]  M. King,et al.  Effect of increasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis. , 1997, Thorax.

[91]  Y. Ouchi,et al.  Osteoporosis in lung transplantation candidates with end-stage pulmonary disease. , 1997, The American journal of medicine.

[92]  T. Church,et al.  Fecal occult blood screening in the Minnesota study: sensitivity of the screening test. , 1997, Journal of the National Cancer Institute.

[93]  E. Dickson,et al.  Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. , 1997, Gastroenterology.

[94]  D. Gozal Nocturnal ventilatory support in patients with cystic fibrosis: comparison with supplemental oxygen. , 1997, The European respiratory journal.

[95]  M. E. Wohl,et al.  End-of-life care in cystic fibrosis. , 1997, Pediatrics.

[96]  M. King,et al.  Rheology of cystic fibrosis sputum after in vitro treatment with hypertonic saline alone and in combination with recombinant human deoxyribonuclease I. , 1997, American journal of respiratory and critical care medicine.

[97]  J. Lloyd-Still,et al.  High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. , 1997, The New England journal of medicine.

[98]  M. Rosenfeld,et al.  Gender gap in cystic fibrosis mortality. , 1997, American journal of epidemiology.

[99]  C. Bijleveld,et al.  Low-dose versus high-dose ursodeoxycholic acid in cystic fibrosis-related cholestatic liver disease. Results of a randomized study with 1-year follow-up. , 1997, Scandinavian journal of gastroenterology.

[100]  S. Fitzsimmons,et al.  Pregnancy in patients with cystic fibrosis , 1997, Pediatric pulmonology. Supplement.

[101]  B. Griffith,et al.  Influence of panel-reactive antibody on survival and rejection after lung transplantation. , 1997, The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.

[102]  G. Saggese,et al.  Bone Demineralization in Cystic Fibrosis: Evidence of Imbalance between Bone Formation and Degradation , 1997, Pediatric Research.

[103]  T. Imperiale,et al.  Primary prophylaxis of variceal bleeding in cirrhosis: a cost-effectiveness analysis. , 1997, Gastroenterology.

[104]  G. Lepage,et al.  Ursodeoxycholic acid improves the hepatic metabolism of essential fatty acids and retinol in children with cystic fibrosis. , 1997, The Journal of pediatrics.

[105]  P. Cole,et al.  Sodium chloride increases the ciliary transportability of cystic fibrosis and bronchiectasis sputum on the mucus-depleted bovine trachea. , 1997, The Journal of clinical investigation.

[106]  D. Kaplan,et al.  Transition of care provided for adolescents with special health care needs. American Academy of Pediatrics Committee on Children with Disabilities and Committee on Adolescence. , 1996, Pediatrics.

[107]  B. Melosky,et al.  Colonic carcinoma in two adult cystic fibrosis patients. , 1996, Canadian journal of gastroenterology = Journal canadien de gastroenterologie.

[108]  Charles A. Johnson,et al.  Effects of 12-Week Administration of Dornase Alfa in Patients with Advanced Cystic Fibrosis Lung Disease , 1996 .

[109]  M. E. Wohl,et al.  Chronic pain in cystic fibrosis. , 1996, Pediatrics.

[110]  A. Prince,et al.  Antibiotic susceptibility of multiply resistant Pseudomonas aeruginosa isolated from patients with cystic fibrosis, including candidates for transplantation. , 1996, Clinical infectious diseases : an official publication of the Infectious Diseases Society of America.

[111]  E. Shane,et al.  Osteoporosis in lung transplantation candidates with end-stage pulmonary disease. , 1996, The American journal of medicine.

[112]  B. Ramsey,et al.  Management of pulmonary disease in patients with cystic fibrosis. , 1996, The New England journal of medicine.

[113]  P. Sammut,et al.  The effects of albuterol on the lung function of hospitalized patients with cystic fibrosis. , 1996, American journal of respiratory and critical care medicine.

[114]  K. Hardy,et al.  Noninvasive clearance of airway secretions. , 1996, Respiratory care clinics of North America.

[115]  R. Amin,et al.  Aerosolized recombinant human DNase in hospitalized cystic fibrosis patients with acute pulmonary exacerbations. , 1996, American journal of respiratory and critical care medicine.

[116]  A. Giunta,et al.  Ursodeoxycholic acid for liver disease associated with cystic fibrosis: A double‐blind multicenter trial , 1996, Hepatology.

[117]  M. Corey,et al.  Determinants of mortality from cystic fibrosis in Canada, 1970-1989. , 1996, American journal of epidemiology.

[118]  J. Carlin,et al.  Bronchoalveolar lavage or oropharyngeal cultures to identify lower respiratory pathogens in infants with cystic fibrosis , 1996, Pediatric pulmonology.

[119]  S. Anderson,et al.  Airway responsiveness to hyperosmolar saline challenge in cystic fibrosis: A pilot study , 1996, Pediatric pulmonology.

[120]  T. Egan,et al.  Severe osteoporosis before and after lung transplantation. , 1996, Chest.

[121]  P. Okunieff,et al.  Cystic fibrosis hetero–and homozygosity is associated with inhibition of breast cancer growth , 1996, Nature Medicine.

[122]  P. Shah,et al.  The effects of recombinant human DNase on neutrophil elastase activity and interleukin-8 levels in the sputum of patients with cystic fibrosis. , 1996, The European respiratory journal.

[123]  D. Dreyfus,et al.  Cystic fibrosis 3849+10kb C > T mutation associated with severe pulmonary disease and male fertility. , 1996, American journal of respiratory and critical care medicine.

[124]  M. Schöni,et al.  Pilot study to assess the effect of inhaled corticosteroids on lung function in patients with cystic fibrosis. , 1996, The Journal of pediatrics.

[125]  J. Morton,et al.  Short‐term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis , 1996, Pediatric pulmonology.

[126]  R. Marcus,et al.  Correlates of osteopenia in patients with cystic fibrosis. , 1996, Pediatrics.

[127]  C. Madsen,et al.  Bone density in children and adolescents with cystic fibrosis. , 1996, The Journal of pediatrics.

[128]  H. Carty,et al.  Osteopenia in adults with cystic fibrosis. , 1995, The American journal of medicine.

[129]  D. Mack,et al.  Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis. , 1995, The Journal of pediatrics.

[130]  C. Lamers,et al.  Genetic and clinical features of patients with cystic fibrosis diagnosed after the age of 16 years. , 1995, Thorax.

[131]  D. Ashby,et al.  Fibrosing colonopathy in cystic fibrosis: results of a case-control study , 1995, The Lancet.

[132]  M. Morgan,et al.  Postprandial versus preprandial blood glucose monitoring in women with gestational diabetes mellitus requiring insulin therapy. , 1995, The New England journal of medicine.

[133]  P. Durie,et al.  Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus Committee. , 1995, The Journal of pediatrics.

[134]  P. König,et al.  Short‐term and long‐term effects of albuterol aerosol therapy in cystic fibrosis: A preliminary report , 1995, Pediatric pulmonology.

[135]  W. Warwick,et al.  Cystic fibrosis-associated colitis and fibrosing colonopathy. , 1995, The Journal of pediatrics.

[136]  R. Blum Transition to adult health care: setting the stage. , 1995, The Journal of adolescent health : official publication of the Society for Adolescent Medicine.

[137]  M Claustres,et al.  Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens. , 1995, The New England journal of medicine.

[138]  D. Schidlow,et al.  A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. , 1995, The Journal of pediatrics.

[139]  M. Knowles,et al.  In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. , 1995, Human gene therapy.

[140]  K. McCoy,et al.  Prolonged parenteral nutrition for cystic fibrosis patients. , 1995, Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition.

[141]  P. J. Byard,et al.  Effect of high-dose ibuprofen in patients with cystic fibrosis. , 1995, The New England journal of medicine.

[142]  J. Lammers,et al.  The effects of the inhaled corticosteroid budesonide on lung function and bronchial hyperresponsiveness in adult patients with cystic fibrosis. , 1995, Respiratory medicine.

[143]  D. Brooks,et al.  Chest physical therapy management of patients with cystic fibrosis. A meta-analysis. , 1995, American journal of respiratory and critical care medicine.

[144]  M. Corey,et al.  The risk of cancer among patients with cystic fibrosis. Cystic Fibrosis and Cancer Study Group. , 1995, The New England journal of medicine.

[145]  G. Gores,et al.  Ursodeoxycholate (UDCA) inhibits the mitochondrial membrane permeability transition induced by glycochenodeoxycholate: a mechanism of UDCA cytoprotection. , 1995, The Journal of pharmacology and experimental therapeutics.

[146]  J. Littlewood Abdominal pain in cystic fibrosis. , 1995, Journal of the Royal Society of Medicine.

[147]  J. Kanis,et al.  DIAGNOSIS OF OSTEOPOROSIS , 2016 .

[148]  R. Sokol Fat-soluble vitamins and their importance in patients with cholestatic liver diseases. , 1994, Gastroenterology clinics of North America.

[149]  J C Olsen,et al.  A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations. , 1994, The New England journal of medicine.

[150]  D. Gozal,et al.  Comparison of high frequency chest compression and conventional chest physiotherapy in hospitalized patients with cystic fibrosis. , 1994, American journal of respiratory and critical care medicine.

[151]  H. Fuchs,et al.  Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. , 1994, The New England journal of medicine.

[152]  J. Nerup,et al.  Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections , 1994, Acta paediatrica.

[153]  R. Henderson,et al.  Kyphosis and fractures in children and young adults with cystic fibrosis. , 1994, The Journal of pediatrics.

[154]  M. Merli,et al.  Effect of a Medium Dose of Ursodeoxycholic Acid with or without Taurine Supplementation on the Nutritional Status of Patients with Cystic Fibrosis: A Randomized, Placebo‐Controlled, Crossover Trial , 1994, Journal of pediatric gastroenterology and nutrition.

[155]  D. Vergani,et al.  Interleukin-1cx, soluble interleukin-2 receptor, andIgGconcentrations incystic fibrosis treated withprednisolone , 1994 .

[156]  Z. Nagy,et al.  Microsurgical epididymal sperm aspiration and intracytoplasmic sperm injection: a new effective approach to infertility as a result of congenital bilateral absence of the vas deferens * , 1994 .

[157]  Rosen Transition from Pediatric to Adult-Oriented Health Care for the Adolescent with Chronic Illness or Disability. , 1994, Adolescent medicine.

[158]  M. Goldstein,et al.  Results in the United States with Sperm Micro-Aspiration Retrieval Techniques and Assisted Reproductive Technologies , 1994 .

[159]  J. Costantino,et al.  Lipid levels in adults with cystic fibrosis. , 1994, Journal of the American Dietetic Association.

[160]  M. Ferrari,et al.  Analysis of risk factors for the development of liver disease associated with cystic fibrosis. , 1994, The Journal of pediatrics.

[161]  J. Udall,et al.  Meconium ileus equivalent in children and adults. , 1994, The American journal of gastroenterology.

[162]  G. Steinkamp,et al.  Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis. , 1994, The Journal of pediatrics.

[163]  A. Stecenko,et al.  Clinical Pharmacology of Pancreatic Enzymes in Patients with Cystic Fibrosis and In Vitro Performance of Microencapsulated Formulations , 1994, Journal of clinical pharmacology.

[164]  J. Abbott,et al.  Treatment compliance in adults with cystic fibrosis. , 1994, Thorax.

[165]  J. Nerup,et al.  Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications , 1994, Acta paediatrica.

[166]  P. Friend,et al.  Liver transplantation for hepatic cirrhosis in cystic fibrosis. , 1996, Journal of the Royal Society of Medicine.

[167]  D P Orr,et al.  Transition from child-centered to adult health-care systems for adolescents with chronic conditions. A position paper of the Society for Adolescent Medicine. , 1993, The Journal of adolescent health : official publication of the Society for Adolescent Medicine.

[168]  A. Swerdlow,et al.  A cohort study of cystic fibrosis and malignancy. , 1993, British Journal of Cancer.

[169]  B. Kerem,et al.  Mild cystic fibrosis and normal or borderline sweat test in patients with the 3849 + 10 kb C → T mutation , 1993, The Lancet.

[170]  D. Heuman,et al.  Hepatoprotective properties of ursodeoxycholic acid. , 1993, Gastroenterology.

[171]  C. A. Vaz Fragoso,et al.  Review of the clinical efficacy of theophylline in the treatment of chronic obstructive pulmonary disease. , 1993, The American review of respiratory disease.

[172]  A. Giunta,et al.  Failure of ursodeoxycholic acid to dissolve radiolucent gallstones in patients with cystic fibrosis , 1993, Acta paediatrica.

[173]  D S Rosen,et al.  Transition to adult health care for adolescents and young adults with cancer , 1993, Cancer.

[174]  M. Knowles,et al.  Cystic fibrosis foundation consensus conference report on pulmonary complications of cystic fibrosis , 1993, Pediatric pulmonology.

[175]  D. Fryback,et al.  Wisconsin cystic fibrosis chest radiograph scoring system. , 1993, Pediatrics.

[176]  S. Kelsey,et al.  The prognostic value of exercise testing in patients with cystic fibrosis. , 1992, The New England journal of medicine.

[177]  I. Hawkins,et al.  Transjugular intrahepatic portosystemic shunt in a child with cystic fibrosis. , 1992, AJR. American journal of roentgenology.

[178]  J. Popovich,et al.  Respiratory physiology in pregnancy. , 1992, Clinics in chest medicine.

[179]  S. Nasr,et al.  Transition program from pediatric to adult care for cystic fibrosis patients. , 1992, The Journal of adolescent health : official publication of the Society for Adolescent Medicine.

[180]  A. Giunta,et al.  Ursodeoxycholic acid therapy in cystic fibrosis—associated liver disease: A dose‐response study , 1992, Hepatology.

[181]  G. Cleghorn,et al.  Nutritional Rehabilitation in Cystic Fibrosis: A 5 Year Follow‐up Study , 1992, Journal of pediatric gastroenterology and nutrition.

[182]  C. Figarella,et al.  Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chronic cholestasis. , 1992, The Journal of pediatrics.

[183]  M. Corey,et al.  Prediction of mortality in patients with cystic fibrosis. , 1992, The New England journal of medicine.

[184]  M. White,et al.  Congenital bilateral absence of the vas deferens. A primarily genital form of cystic fibrosis. , 1992 .

[185]  A. Giunta,et al.  Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with cystic fibrosis and associated liver disease , 1992, Hepatology.

[186]  V. Chernick,et al.  Acute bronchodilator response to a combination of beta-adrenergic and anticholinergic agents in patients with cystic fibrosis. , 1992, The Journal of pediatrics.

[187]  M. Dueholm,et al.  N-acetylcysteine by metered dose inhaler in the treatment of chronic bronchitis: a multi-centre study. , 1992, Respiratory medicine.

[188]  N. Grace Prevention of initial variceal hemorrhage. , 1992, Gastroenterology clinics of North America.

[189]  Geddes Dm,et al.  Cystic fibrosis and pregnancy. , 1992, Journal of the Royal Society of Medicine.

[190]  J. Littlewood Cystic fibrosis: gastrointestinal complications. , 1992, British medical bulletin.

[191]  R. Kotloff,et al.  Fertility and pregnancy in patients with cystic fibrosis. , 1992, Clinics in chest medicine.

[192]  P M Farrell,et al.  Nutritional assessment and management in cystic fibrosis: a consensus report. The Consensus Committee. , 1992, The American journal of clinical nutrition.

[193]  L. Tsui,et al.  Cystic fibrosis gene mutation in two sisters with mild disease and normal sweat electrolyte levels. , 1991, The New England journal of medicine.

[194]  J. Britton,et al.  Cystic fibrosis: current survival and population estimates to the year 2000. , 1991, Thorax.

[195]  R. Gibson,et al.  Predictive value of oropharyngeal cultures for identifying lower airway bacteria in cystic fibrosis patients. , 1991, The American review of respiratory disease.

[196]  M. Deneyer,et al.  Esophageal pH Monitoring Data During Chest Physiotherapy , 1991, Journal of pediatric gastroenterology and nutrition.

[197]  E. Mellits,et al.  A controlled trial of long-term bronchodilator therapy in cystic fibrosis. , 1991, Chest.

[198]  M. Yacoub,et al.  Non-invasive mechanical ventilation for cystic fibrosis patients--a potential bridge to transplantation. , 1991, The European respiratory journal.

[199]  H. Levison,et al.  Appendicitis in cystic fibrosis. , 1991, Archives of disease in childhood.

[200]  B. Rosenstein,et al.  Risks of alternate-day prednisone in patients with cystic fibrosis. , 1991, Pediatrics.

[201]  C. Lamers,et al.  Omeprazole enhances the efficacy of pancreatin (pancrease) in cystic fibrosis. , 1991, Annals of internal medicine.

[202]  S. Shak,et al.  Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. , 1990, Proceedings of the National Academy of Sciences of the United States of America.

[203]  R. Stern,et al.  Person-to-person transmission of Pseudomonas cepacia between patients with cystic fibrosis , 1990, The Lancet.

[204]  M. Corey,et al.  Effects of cisapride in patients with cystic fibrosis and distal intestinal obstruction syndrome. , 1990, The Journal of pediatrics.

[205]  D. Schidlow,et al.  Life beyond pediatrics. Transition of chronically ill adolescents from pediatric to adult health care systems. , 1990, The Medical clinics of North America.

[206]  K. Setchell,et al.  Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis. , 1990, The Journal of pediatrics.

[207]  M. Lentze,et al.  Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis. , 1990, Gut.

[208]  R. Stern,et al.  The spectrum of appendiceal disease in cystic fibrosis. , 1990, Journal of pediatric surgery.

[209]  J. Davis,et al.  Meta-analysis of value of propranolol in prevention of variceal haemorrhage , 1990, The Lancet.

[210]  A. Stecenko,et al.  Treatment failure after substitution of generic pancrelipase capsules. Correlation with in vitro lipase activity. , 1990, JAMA.

[211]  D M Eddy,et al.  Screening for osteoporosis. , 1990, Annals of internal medicine.

[212]  J. Zitsman Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis , 1990 .

[213]  Q. Summers,et al.  Nebulized ipratropium in the treatment of acute asthma. , 1990, Chest.

[214]  M. Hodson,et al.  Effect of chest physiotherapy on oxygen saturation in patients with cystic fibrosis. , 1990, Thorax.

[215]  M. Hodson,et al.  LIVER DISEASE AND BILEDUCT ABNORMALITIES IN ADULTS WITH CYSTIC FIBROSIS , 1989, The Lancet.

[216]  C. Denning,et al.  Diabetic microangiopathy in patients with cystic fibrosis. , 1989, Pediatrics.

[217]  F. Cerny,et al.  Relative effects of bronchial drainage and exercise for in-hospital care of patients with cystic fibrosis. , 1989, Physical therapy.

[218]  S. Weintraub,et al.  The inhaled bronchodilators ipratropium bromide and metaproterenol in adults with CF. , 1989, Chest.

[219]  P. Swender,et al.  Breast-milk composition in women with cystic fibrosis: report of two cases and a review of the literature. , 1989, The American journal of clinical nutrition.

[220]  A. Coates,et al.  Nocturnal home oxygen in the treatment of hypoxemic cystic fibrosis patients. , 1989, The Journal of pediatrics.

[221]  C. Koch,et al.  N-acetylcysteine in cystic fibrosis and Pseudomonas aeruginosa infection: clinical score, spirometry and ciliary motility. , 1989, The European respiratory journal.

[222]  P. McGrath,et al.  Assessment of readiness to transfer to adult care for adolescents with cystic fibrosis. , 1989, Children's health care : journal of the Association for the Care of Children's Health.

[223]  M. Gappa,et al.  Long‐term tobramycin aerosol therapy in cystic fibrosis , 1989, Pediatric pulmonology.

[224]  M. Corey,et al.  Long‐term effects of inhaled tobramycin in patients with cystic fibrosis colonized with Pseudomonas aeruginosa , 1989, Pediatric pulmonology.

[225]  M. Corey,et al.  Role of conventional physiotherapy in cystic fibrosis. , 1988, The Journal of pediatrics.

[226]  B. Bresnihan Cystic fibrosis, chronic bacterial infection and rheumatic disease. , 1988, British journal of rheumatology.

[227]  M. Boechat,et al.  Osteoporosis in cystic fibrosis. , 1988, The Journal of pediatrics.

[228]  John F. Murray,et al.  Textbook of Respiratory Medicine , 1988 .

[229]  S. Finkelstein,et al.  Diabetes mellitus associated with cystic fibrosis. , 1988, The Journal of pediatrics.

[230]  M. Hodson,et al.  Vitamin D and parathyroid hormone and bone mineralisation in adults with cystic fibrosis. , 1988, Thorax.

[231]  K. Gaskin,et al.  Liver disease and common-bile-duct stenosis in cystic fibrosis. , 1988, The New England journal of medicine.

[232]  M. Corey,et al.  A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. , 1988, Journal of clinical epidemiology.

[233]  J. Gamble,et al.  Back pain and spinal deformity in cystic fibrosis. , 1987, American journal of diseases of children.

[234]  Arnold L. Smith,et al.  Antibiotic Pharmacokinetics in Cystic Fibrosis , 1987, Clinical pharmacokinetics.

[235]  B. Bresnihan,et al.  Episodic arthropathy in adult cystic fibrosis. , 1987, The Quarterly journal of medicine.

[236]  N. Høiby,et al.  Colistin inhalation therapy in cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infection. , 1987, The Journal of antimicrobial chemotherapy.

[237]  K. Hardy,et al.  Clinical features, survival rate, and prognostic factors in young adults with cystic fibrosis. , 1987, The American journal of medicine.

[238]  A. Eklund,et al.  No penetration of orally administered N-acetylcysteine into bronchoalveolar lavage fluid. , 1987, European journal of respiratory diseases.

[239]  B. Jonson,et al.  Long‐term Effects of Physical Exercise on Working Capacity and Pulmonary Function in Cystic Fibrosis , 1987, Acta paediatrica Scandinavica.

[240]  D. A. Cook,et al.  A controlled trial of nebulized aminoglycoside and oral flucloxacillin versus placebo in the outpatient management of children with cystic fibrosis. , 1987, British journal of diseases of the chest.

[241]  H. Carty,et al.  Gastrografin in acute meconium ileus equivalent. , 1986, Archives of disease in childhood.

[242]  C. Doershuk,et al.  The Interaction of 2 Diseases: Diabetes Mellitus and Cystic Fibrosis , 1986, Medicine.

[243]  S. Borriello,et al.  Asymptomatic carriage of Clostridium difficile in patients with cystic fibrosis. , 1986, Journal of clinical pathology.

[244]  D. Stringer,et al.  TREATMENT OF DISTAL INTESTINAL OBSTRUCTION SYNDROME IN CYSTIC FIBROSIS WITH A BALANCED INTESTINAL LAVAGE SOLUTION , 1986, The Lancet.

[245]  C. Pantin,et al.  Prednisolone in the treatment of airflow obstruction in adults with cystic fibrosis. , 1986, Thorax.

[246]  A. Cohen,et al.  Evaluation of pulmonary hypertrophic osteoarthropathy in cystic fibrosis. A comprehensive study. , 1986, American journal of diseases of children.

[247]  H. Colten,et al.  ALTERNATE-DAY PREDNISONE REDUCES MORBIDITY AND IMPROVES PULMONARY FUNCTION IN CYSTIC FIBROSIS , 1985, The Lancet.

[248]  M. Corey,et al.  Age-related alterations in immunoreactive pancreatic lipase and cationic trypsinogen in young children with cystic fibrosis. , 1985, The Journal of pediatrics.

[249]  M. McKenna,et al.  Hypovitaminosis D and response to supplementation in older patients with cystic fibrosis. , 1985, The Quarterly journal of medicine.

[250]  P. König,et al.  A longitudinal study of bronchodilator responsiveness in cystic fibrosis. , 1985, The American review of respiratory disease.

[251]  M. Miller,et al.  NEBULISED COLOMYCIN FOR EARLY PSEUDOMONAS COLONISATION IN CYSTIC FIBROSIS , 1985, The Lancet.

[252]  G. Polgar,et al.  Bronchodilators increase airway instability in cystic fibrosis , 1985, The American review of respiratory disease.

[253]  O. Rennert,et al.  Reproductive defects in patients of both sexes with cystic fibrosis: a review. , 1985, Annals of clinical and laboratory science.

[254]  D. Orenstein,et al.  Oxygen saturation during exercise in cystic fibrosis. , 2015, The American review of respiratory disease.

[255]  V. Hubbard,et al.  Use of oral contraceptives in women with cystic fibrosis. , 1984, Chest.

[256]  D. Schidlow,et al.  Arthritis in cystic fibrosis. , 1984, Archives of disease in childhood.

[257]  M. Hodson,et al.  Hypertrophic osteoarthropathy in cystic fibrosis. , 1984, British medical journal.

[258]  L. Landau,et al.  Nebulized gentamicin in children and adolescents with cystic fibrosis , 1984, Australian paediatric journal.

[259]  P. Durie,et al.  Colipase and lipase secretion in childhood-onset pancreatic insufficiency: Delineation of patients with steatorrhea secondary to relative colipase deficiency , 1984 .

[260]  A. Coates,et al.  Immediate and long-term effects of chest physiotherapy in patients with cystic fibrosis. , 1983, The Journal of pediatrics.

[261]  J. Dempsey,et al.  Ventilation and oxygenation changes during sleep in cystic fibrosis. , 1983, Chest.

[262]  N. Høiby,et al.  FREQUENT ANTIBIOTIC THERAPY IMPROVES SURVIVAL OF CYSTIC FIBROSIS PATIENTS WITH CHRONIC PSEUDOMONAS AERUGINOSA INFECTION , 1983 .

[263]  V. Gill,et al.  Isolation rate and toxigenic potential of Clostridium difficile isolates from patients with cystic fibrosis. , 1983, The Journal of infectious diseases.

[264]  J. Eisenberg,et al.  INHALED ANTIBIOTICS IN CYSTIC FIBROSIS , 1983, The Lancet.

[265]  B. Rosenstein,et al.  Incidence of Distal Intestinal Obstruction Syndrome in Cystic Fibrosis , 1983, Journal of pediatric gastroenterology and nutrition.

[266]  S. Husby,et al.  CHRONIC PSEUDOMONAS AERUGINOSA LUNG INFECTION IN CYSTIC FIBROSIS , 1983, Acta paediatrica Scandinavica.

[267]  E. Coles,et al.  COELIAC DISEASE AND MALIGNANCY , 1983, The Lancet.

[268]  T. De,et al.  Parenteral nutrition before gastrointestinal surgery. , 1982 .

[269]  R. Grand,et al.  Gastrointestinal manifestations of cystic fibrosis: a review. , 1981, Gastroenterology.

[270]  M. Zach,et al.  EFFECT OF SWIMMING ON FORCED EXPIRATION AND SPUTUM CLEARANCE IN CYSTIC FIBROSIS , 1981, The Lancet.

[271]  M. Hodson,et al.  AEROSOL CARBENICILLIN AND GENTAMICIN TREATMENT OF PSEUDOMONAS AERUGINOSA INFECTION IN PATIENTS WITH CYSTIC FIBROSIS , 1981, The Lancet.

[272]  L. Cohen,et al.  Cystic Fibrosis and Pregnancy: A National Survey , 1981 .

[273]  M. Corey,et al.  Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis. , 1980, Gut.

[274]  T. Boat,et al.  Heart failure in cystic fibrosis. Treatment and prognosis of cor pulmonale with failure of the right side of the heart. , 1980, American journal of diseases of children.

[275]  H. Kressel,et al.  Meconium ileus equivalent: treatment with Hypaque enema. , 1980, Diagnostic imaging.

[276]  R. Mazess,et al.  Demineralization in cystic fibrosis detected by direct photon absorptiometry. , 1979, American journal of diseases of children.

[277]  S. Soong,et al.  The chest roentgenogram in cystic fibrosis: a new scoring system. , 1979, Pediatrics.

[278]  T. Hahn,et al.  Reduced serum 25-hydroxyvitamin D concentration and disordered mineral metabolism in patients with cystic fibrosis. , 1979, The Journal of pediatrics.

[279]  P. di Sant'Agnese,et al.  Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature , 1979 .

[280]  P. di Sant'Agnese,et al.  Assisted ventilation for patients with cystic fibrosis. , 1978, JAMA.

[281]  R. Kraemer,et al.  RELATIVE UNDERWEIGHT IN CYSTIC FIBROSIS AND ITS PROGNOSTIC VALUE , 1978, Acta paediatrica Scandinavica.

[282]  M. Yaron,et al.  Letter: Levamisole in rheumatoid arthritis. , 1976, Lancet.

[283]  M. Rawlins,et al.  HOW DO DOCTORS LEARN ABOUT DRUGS? , 1975, The Lancet.

[284]  C. Roy,et al.  Fecal chymotrypsin: a reliable index of exocrine pancreatic function in children. , 1973, The Journal of pediatrics.

[285]  H. Shwachman,et al.  Water and electrolytes in cervical mucus from patients with cystic fibrosis. , 1973, Fertility and sterility.

[286]  E. Oppenheimer,et al.  Cervical mucus in cystic fibrosis: a possible cause of infertility. , 1970, American journal of obstetrics and gynecology.

[287]  S. Rao,et al.  Acute effects of nebulization of N-acetylcysteine on pulmonary mechanics and gas exchange. , 1970, The American review of respiratory disease.

[288]  H. Shwachman,et al.  Reproductive failure in males with cystic fibrosis. , 1968, The New England journal of medicine.

[289]  S. Eidelman,et al.  Oral administration of n-acetyl cysteine in the prophylaxis of "meconium ileus equivalent". , 1967, The Journal of pediatrics.

[290]  P. Rhodes,et al.  Acute Effects of Aerosolized Acetylcysteine Upon Spirometric Measurements in Subjects With and Without Obstructive Pulmonary Disease , 1966 .

[291]  A. Barber EXPENDITURE ON N.H.S. PRACTICE PREMISES. THE MINISTER'S LETTER. , 1964, Lancet.

[292]  J. Gustafson,et al.  Cystic Fibrosis , 2009, Journal of the Iowa Medical Society.

[293]  Izant Rj,et al.  MECONIUM ILEUS EQUIVALENT. , 1963 .

[294]  Nutrition Board,et al.  RECOMMENDED DIETARY ALLOWANCES. , 1964, Clinical pediatrics.