Cystic fibrosis: Benefits and clinical outcome

Journal of Inherited Metabolic Disease

Volumn 30, Issue 4, 2007, Pages 544-555

  McKay, Karen O ^a,b  

^a UNIVERSITY OF SYDNEY   (Australia)

^b CHILDREN S HOSPITAL AT WESTMEAD   (Australia)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBIOTIC THERAPY; CLINICAL TRIAL; CONFERENCE PAPER; CONTROLLED CLINICAL TRIAL; CYSTIC FIBROSIS; DISEASE SEVERITY; HEALTH PROGRAM; HOSPITALIZATION; HUMAN; LUNG DISEASE; LUNG FUNCTION TEST; MALNUTRITION; NEWBORN; NEWBORN SCREENING; OUTCOME ASSESSMENT; PANCREAS INSUFFICIENCY; PSEUDOMONAS AERUGINOSA; QUALITY OF LIFE; RANDOMIZED CONTROLLED TRIAL; RESPIRATORY TRACT INFECTION; THORAX RADIOGRAPHY; TREATMENT DURATION;

CLINICAL TRIALS; COHORT STUDIES; CYSTIC FIBROSIS; GENETIC PREDISPOSITION TO DISEASE; HUMANS; INFANT, NEWBORN; LUNG; LUNG DISEASES; NEONATAL SCREENING; NUTRITIONAL SCIENCES; PSEUDOMONAS INFECTIONS; REGISTRIES; RESEARCH DESIGN; TREATMENT OUTCOME;

EID: 34548514218     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-007-0620-0     Document Type: Conference Paper

References (36)

1. Accurso FJ, Sokol RJ, Hammond KB, Abman SH (1991) Early respiratory course in infants with cystic fibrosis: Relevance to newborn screening. Pediatr Pulmonol 7: S42-s45.
2. Accurso FJ, Sontag MK, Wagener JS (2005) Complications associated with symptomatic diagnosis in infants with cystic fibrosis. J Paediatr 147: s37-s41.
3. Assael BM, Castellani C, Ocampo MB, Iansa P, Callegaro A, Valsecchi MG (2002) Epidemiology and survival analysis of cystic fibrosis in an area of intense neonatal screening over 30 years. Am J Epidemiol 156: 397-401.
4. Chatfield S, Owen G, Ryley HC, et al (1991) Neonatal screening for cystic fibrosis in Wales and the West Midlands: Clinical assessment after 5 years of screening. Arch Dis Child 66: 29-33.
5. Corrigan JJ, Taussig LM, Beckerman R, Wagener JS (1981) Factor II (prothrombin) coagulant activity in immunoreactive protein: Detection of vitamin K deficiency and liver disease in patients with cystic fibrosis. J Pediatr 99: 254-257.
6. Dankert-Roelse JE, te Meerman GJ (1995) Long term prognosis of patients with cystic fibrosis in relation to early detection by neonatal screening and treatment in a cystic fibrosis centre. Thorax 50: 712-718.
7. Dankert-Roelse JE, te Meerman GJ, Martjin A, ten Kate LP, Knol K (1989) Survival and clinical outcome in patients with cystic fibrosis, with or without neonatal screening. J Pediatr 114: 362-367.
8. Danks DM, Allan JL, Anderson CM (1965) A genetic study of fibrocystic disease of the pancreas. Ann Human Gen 28: 323-356.
9. Davis PB (2006) Cystic fibrosis since 1938. Am J Respir Crit Care Med 173: 475-482.
10. Doull IJM, Ryley HC, Weller P, Goodchild MC (2001) Cystic fibrosis-related deaths in infancy and the effect of newborn screening. Pediatr Pulmonol 31: 363-366.
11. Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson RL (2002) Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol 34: 91-100.
12. Farrell PM, Bieri JG, Fratononi JF, Wood RE, di Sant'Agnese PA (1977) The occurrence and effects of human vitamin E deficiency - a study in patients with cystic fibrosis. J Clin Invest 60: 233-241.
13. Farrell PM, Kosorok MR, Laxova A, et al (1997a) Nutritional benefits of neonatal screening for cystic fibrosis. N Engl J Med 337: 963-969.
14. Farrell PM, Shen G, Splaingard M, et al (1997b) Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis. Pediatrics 100: e2.
15. Farrell PM, Kosorok MR, Rock MJ, et al (2001) Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Pediatrics 107: 1-13.
16. Farrell PM, Li Z, Kosorok MR, et al (2003) Bronchopulmonary disease in children with cystic fibrosis after early or delayed diagnosis. Am J Respir Crit Care Med 168: 1100-1108.
17. Farrell PM, Lai HCJ, Li Z, et al (2005) Evidence on improved outcomes with early diagnosis of cystic fibrosis through neonatal screening: enough is enough! J Pediatr 147: S30-s36.
18. Grosse SD, Rosenfeld M, Devine OJ, Lai HJ, Farrell PM (2006) Potential impact of newborn screening for cystic fibrosis on child survival: A systematic review and analysis. J Pediatr 149: 362-366.
19. Koscik RL, Farrell PM, Kosorok MR, et al (2004) Cognitive function of children with cystic fibrosis: Deleterious effect of early malnutrition. Pediatrics 113: 1549-1558.
20. Lai HC, Kosorok MR, Sondel SA, et al (1998) Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: Evaluation of various criteria used to identify malnutrition. J Pediatr 132: 478-485.
21. Lai HCJ, Cheng Y, Cho H, Kosorok MR, Farrell PM (2004) Association between initial disease presentation, lung disease outcomes, and survival in patients with cystic fibrosis. Am J Epidemiol 159: 537-546.
22. Mastella G, Zanolla L, Castellani C, et al (2001) Neonatal screening for cystic fibrosis: Long-term clinical balance. Pancreatology 1: 531-537.
23. McKay KO, Waters DL, Gaskin KJ (2005) The influence of newborn screening for cystic fibrosis on pulmonary outcomes in New South Wales. J Pediatr 147: S47-s50.
24. Mérelle ME, Dankert-Roelse JE, Dezateux C, Lees C, Nagelkerke A, Southern KW (2001a) Newborn screening for cystic fibrosis. Cochrane Database of Systematic Reviews Issue 3. Art No: CD00142. doi: 10.1002/ 14651858.CD001402.
25. Mérelle ME, Schouten JP, Gerritsen J, Dankert-Roelse JE (2001b) Influence of neonatal screening and centralized treatment on long-term clinical outcome and survival of CF patients. Eur Respir J 18: 306-315.
26. Rosenstein BJ, Zeitlin PL (1998) Cystic fibrosis. The Lancet 351: 277-282.
27. Saiman L (2004) Microbiology of early CF lung disease. Pediatr Respir Rev 5: S367-369.
28. Sims EJ, McCormick J, Mehta G, Mehta A (2005a) Neonatal screening for cystic fibrosis is beneficial even in the context of modern treatment. J Pediatr 147: S42-s46.
29. Sims EJ, McCormick J, Mehta G, Mehta A (2005b) Newborn screening for cystic fibrosis is associated with reduced treatment intensity. J Pediatr 147: 306-311.
30. Siret D, Bretaudeau G, Branger B, et al (2003) Comparing the clinical evolution of cystic fibrosis screened neonatally to that of cystic fibrosis diagnosed from clinical symptoms: A 10 year retrospective study in a French Region (Brittany). Pediatr Pulmonol 35: 342-349.
31. Solomons NW, Wagonfield JB, Rieger C, et al (1981) Some biochemical indices of nutrition in treated cystic fibrosis patients. Am J Clin Nutr 34: 462-474.
32. Tsui LC, Buchwald M, Barker D, et al (1985) Cystic fibrosis locus defined by a genetically linked polymorphic DNA marker. Science 230: 1054-1057.
33. Wang SS, FitzSimmons SC, O'Leary LA, Rock MJ, Gwinn ML, Khoury MJ (2001) Early diagnosis of cystic fibrosis in the newborn period and risk of Pseudomonas aeruginosa acquisition in the first 10 years of life: A registry-based longitudinal study. Pediatrics 107: 274-279.
34. Waters DL, Wilcken B, Irwig L, et al (1999) Clinical outcomes of newborn screening for cystic fibrosis. Arch Dis Child Fetal Neonatal Ed 80: F1-F7.
35. Wilcken B, Chalmers G (1985) Reduced morbidity in patients with cystic fibrosis detected by neonatal screening. The Lancet 326: 1319-1321.
36. Wilmott RW, Tyson SL, Matthew DJ (1985) Cystic fibrosis survival rates: the influences of allergy and Pseudomonas aeruginosa. Arch Dis Child 139: 669-671.