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Health Condition

Cystic Fibrosis

About This Condition

Cystic fibrosis (CF) is an inherited disease that results in impaired transport of chloride into and out of cells. The digestive and respiratory systems are most affected. 

The most common manifestation of cystic fibrosis is frequent respiratory infection. Impaired digestion and malabsorption due to pancreatic insufficiency and blocked liver ducts is often seen as well. Management of this condition requires the help of a qualified doctor.

Symptoms

Symptoms include a persistent cough with thick and often greenish-colored mucus, failure to grow normally, recurrent sinus and bronchial infections, and frequent, bulky, foul-smelling stools. Infants may experience a set of acute symptoms, including a distended abdomen, failure to pass stool, and vomiting. Although the course of the disease is highly variable, largely dependent upon the severity and frequency of respiratory infections, CF inevitably leads to debility and death. Average survival is to age 31.

Other Therapies

Treatment typically includes a daily regimen of physical therapy that consists of pounding on the chest to loosen mucus.

References

1. Littlewood JM, Wolfe SP. Control of malabsorption in cystic fibrosis. Paediatr Drugs 2000;2:205-22.

2. Borowitz DS, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus committee. J Pediatr 1995;127:681-4.

3. Vyas H, Matthew DJ, Milla PJ. A comparison of enteric coated microspheres with enteric coated tablet pancreatic enzyme preparations in cystic fibrosis. A controlled study. Eur J Pediatr 1990;149:241-3.

4. Taylor CJ, Hillel PG, Ghosal S, et al. Gastric emptying and intestinal transit of pancreatic enzyme supplements in cystic fibrosis. Arch Dis Child 1999;80:149-52.

5. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805-22 [review].

6. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537-40.

7. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33-41.

8. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805-22 [review].

9. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537-40.

10. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33-41.

11. Chase HP, Dupont J. Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis. Lancet 1978;2:236-8.

12. Rosenlund ML, Selekman JA, Kim HK, Kritchevsky D. Dietary essential fatty acids in cystic fibrosis. Pediatrics 1977;59:428-32.

13. Lloyd-Still JD, Simon SH, Wessel HU, Gibson LE. Intravenous linoleic acid supplementation in children with cystic fibrosis. Pediatrics 1979;64:50-2.

14. Lawrence R, Sorrell T. Eicosapentaenoic acid in cystic fibrosis: evidence of a pathogenic role for leukotriene B4. Lancet 1993;342:465-9.

15. Darling PB, Lepage G, Leroy C, et al. Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatr Res 1985;19:578-82.

16. Belli DC, Levy E, Darling PB, et al. Taurine improves the absorption of a fat meal in patients with cystic fibrosis. Pediatrics 1987;80:517-23.

17. Thompson GN, Robb TA, Davidson GP. Taurine supplementation, fat absorption, and growth in cystic fibrosis. J Pediatr 1987;111:501-6.

18. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805-22 [review].

19. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537-40.

20. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33-41.

21. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805-22 [review].

22. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537-40.

23. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33-41.

24. Krebs NF, Sontag M, Accurso FJ, Hambidge KM. Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr 1998;133:761-4.

25. Abdulhamid I, Beck FWJ, Millard S, et al. Effect of zinc supplementation on respiratory tract infections in children with cystic fibrosis. Pediatr Pulmonol 2008;43:281-7.

26. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805-22 [review].

27. Wilson DC, Pencharz PB. Nutrition and cystic fibrosis. Nutrition 1998;14:792-5 [review].

28. Schneiderman-Walker J, Pollock SL, Corey M, et al. A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J Pediatr 2000;136:304-10.

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The information presented by Healthnotes is for informational purposes only. It is based on scientific studies (human, animal, or in vitro), clinical experience, or traditional usage as cited in each article. The results reported may not necessarily occur in all individuals. Self-treatment is not recommended for life-threatening conditions that require medical treatment under a doctor's care. For many of the conditions discussed, treatment with prescription or over the counter medication is also available. Consult your doctor, practitioner, and/or pharmacist for any health problem and before using any supplements or before making any changes in prescribed medications. Information expires December 2018.

Drugs used to treat CYSTIC FIBROSIS. Select drug name to view medication information and pricing