Cystic Fibrosis: Multimedia

References

1. Davis PB, Drumm M, Konstan MW. Cystic fibrosis. Am J Respir Crit Care Med. Nov 1996;154(5):1229-56. [Medline].

2. Collaco JM, Vanscoy L, Bremer L, et al. Interactions between secondhand smoke and genes that affect cystic fibrosis lung disease. JAMA. Jan 30 2008;299(4):417-24. [Medline].

3. Sharma GD, Doershuk CF, Stern RC. Erosion of the wall of the frontal sinus caused by mucopyocele in cystic fibrosis. J Pediatr. May 1994;124(5 Pt 1):745-7. [Medline].

4. Cystic Fibrosis Genetic Analysis Consortium. Cystic fibrosis mutation database-. CFMDB Statistics. Available at http://www.genet.sickkids.on.ca/cftr/StatisticsPage.html. Accessed September 25, 2008.

5. LeGrys VA, Yankaskas JR, Quittell LM, Marshall BC, Mogayzel PJ Jr. Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines. J Pediatr. Jul 2007;151(1):85-9. [Medline].

6. Comeau AM, Accurso FJ, White TB, et al. Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics. Feb 2007;119(2):e495-518. [Medline]. [Full Text].

7. Hale JE, Parad RB, Comeau AM. Newborn screening showing decreasing incidence of cystic fibrosis. N Engl J Med. Feb 28 2008;358(9):973-4. [Medline].

8. Ren CL, Brucker JL, Rovitelli AK, Bordeaux KA. Changes in lung function measured by spirometry and the forced oscillation technique in cystic fibrosis patients undergoing treatment for respiratory tract exacerbation. Pediatr Pulmonol. Apr 2006;41(4):345-9. [Medline].

9. Yankaskas JR, Mallory GB Jr. Lung transplantation in cystic fibrosis: consensus conference statement. Chest. Jan 1998;113(1):217-26. [Medline].

10. Liou TG, Adler FR, Cox DR, Cahill BC. Lung transplantation and survival in children with cystic fibrosis. N Engl J Med. Nov 22 2007;357(21):2143-52. [Medline].

11. Allen J, Visner G. Lung transplantation in cystic fibrosis--primum non nocere?. N Engl J Med. Nov 22 2007;357(21):2186-8. [Medline].

12. [Best Evidence] Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med. Jan 19 2006;354(3):241-50. [Medline].

13. [Best Evidence] Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. Jan 19 2006;354(3):229-40. [Medline].

14. [Best Evidence] Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. Nov 15 2007;176(10):957-69. [Medline].

15. Gibson RL, Emerson J, McNamara S, Burns JL, Rosenfeld M, Yunker A. Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med. Mar 15 2003;167(6):841-9. [Medline]. [Full Text].

16. Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. Oct 1 2003;290(13):1749-56. [Medline].

Further Reading

Keywords

cystic fibrosis, CF, CFTR, mucoviscidosis, cystic disease of the pancreas, chronic respiratory infection, pancreatic enzyme insufficiency, airway obstruction, lung infection, lung inflammation, exocrine gland function, progressive lung disease, end-stage lung disease, meconium ileus, distal intestinal obstruction syndrome, DIOS, fecal impaction, intussusception, obstructive cirrhosis, splenomegaly, hypersplenism, malnutrition, hepatic steatosis, right heart failure, gallstones, bronchitis, bronchiolitis, bronchiectasis, cor pulmonale, hemoptysis, pneumothorax, mucocele, mucopyocele, sinusitis, nasal polyps, intestinal obstruction syndrome, rectal prolapse, peptic ulcer, gastroesophageal reflux, volvulus, intestinal atresia, perforation, meconium peritonitis, amenorrhea, delayed sexual development, sterility, hydrocele, rhinitis, scoliosis, kyphosis

Contributor Information and Disclosures

Author

Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center
Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland
Disclosure: Nothing to disclose.

Medical Editor

Susanna A McColley, MD, Director of Cystic Fibrosis Center; Head, Division of Pulmonary Medicine; Associate Professor, Department of Pediatrics, Children's Memorial Medical Center of Chicago, Northwestern University
Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Consulting fee Consulting; Novartis Consulting fee Consulting; Altus Consulting fee Consulting; Axcan Scandi Consulting fee Consulting; Boston Scientific Consulting fee Consulting

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center
Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, and Christian Medical & Dental Society
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching