Nasal potential difference in non-classic cystic fibrosis - Long term follow up

Pediatric Pulmonology

Volumn 43, Issue 6, 2008, Pages 545-549

  Jaron, Ranit ^a   Yaakov, Yasmin ^a   Rivlin, Joseph ^b   Blau, Hannah ^c   Bentur, Lea ^d   Yahav, Yakov ^e   Kerem, Eitan ^a   Bibi, Haim ^b   Picard, Elie ^f   Wilschanski, Michael ^a  

^a HADASSAH HEBREW UNIVERSITY MEDICAL CENTER   (Israel)

^b CARMEL MEDICAL CENTER   (Israel)

^c SCHNEIDER CHILDREN'S HOSPITAL   (United States)

^d RAMBAM MEDICAL CENTER   (Israel)

^e SHEBA MEDICAL CENTER   (Israel)

^f SHAARE ZEDEK MEDICAL CENTER   (Israel)

Author keywords

Long term follow up; Nasal potential difference; Non classic cystic fibrosis; Questionable cystic fibrosis

Indexed keywords

ARTICLE; CLINICAL FEATURE; CONTROLLED STUDY; CYSTIC FIBROSIS; DISEASE COURSE; FOLLOW UP; HUMAN; LONG TERM CARE; MAJOR CLINICAL STUDY; POTENTIAL DIFFERENCE; PRIORITY JOURNAL; SINUSITIS;

CYSTIC FIBROSIS; DISEASE PROGRESSION; FOLLOW-UP STUDIES; HUMANS; MEMBRANE POTENTIALS; NASAL MUCOSA; REPRODUCIBILITY OF RESULTS;

EID: 44949253090     PISSN: 87556863     EISSN: 10990496     Source Type: Journal    
DOI: 10.1002/ppul.20807     Document Type: Article

References (21)

1. Lebecque P, Leal T, De Boeck C, Jaspers M, Cuppens H, Cassiman JJ. Mutations of the cystic fibrosis gene and intermediate sweat chloride levels in children. Am J Resp Crit Care Med 2002;165:757-761.
2. Highsmith WE, Burch LH, Zhou Z, Olsen JC, Boat TE, Spock A, Gorvoy JD, Quittell L, Friedman KJ, Silverman LM, Boucher RC, Knowles MR. A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations. N Engl J Med 1994;331:974-980.
3. Augarten A, Kerem BS, Yahav Y, Noiman S, Rivlin Y, Tal A, Blau H, Ben-Tur L, Szeinberg A, Kerem E. Mild cystic fibrosis and normal or borderline sweat test in patients with the 3849+10kb C->T mutation. Lancet 1993;342:25-26.
4. Pradal U, Castellani C, Delmarco A, Mastella G. Nasal potential differnce in congenital bilateral abscence of the vas deferns. Am J Respir Crit Care Med 1998;158:896-901.
5. Sharer N, Schwarz M, Malone G, Howarth A, Painter J, Super M, Braganza J. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med 1998;339:645-652.
6. Widerman E, Millner L, Sexauer W, Fiel S. Health status and sociodemographic characteristics of adults receiving a cystic fibrosis diagnosis after age 18 years. Chest 2000;118:427-433.
7. Gilljam M, Ellis L, Corey M, Zielenski J, Durie P, Tullis DE. Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood. Chest 2004;126:1215-1224.
8. McCloskey M, Redmond AOB, Hill A, Elborn JS. Clinical features associated with a delayed diagnosis of cystic fibrosis. Respiration 2000;67:402-407.
9. Boyle MP. Nonclassic cystic fibrosis and CFTR-related diseases. Curr Opin Pulm Med 2003;9:498-503.
10. Wallis C. Atypical cystic fibrosis - diagnostic and management dilemmas. J R Soc Med 2003;96:2-10.
11. Knowles MR, Carson JL, Collier AM, Gatzy JT, Boucher RC. Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo. Am Rev Respir Dis 1981;124:484-490.
12. Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr 1998;132:589-595.
13. Wilschanski M, Famini H, Strauss-Liviatan N, Rivlin J, Blau H, Bibi H, Bentur L, Yahav Y, Springer H, Kramer MR, Klar A, Ilani A, Kerem B, Kerem E. Nasal potential difference measurement in patients with atypical cystic fibrosis. Eur Respir J 2001;17:1208-1215.
14. Yaakov Y, Kerem E, Yahav Y, Rivlin J, Blau H, Bentur L, Aviram M, Picard E, Bdolah-Abram T, Wilschanski M. Reproducibility of nasal potential difference measurements in cystic fibrosis. Chest 2007;132:1219-1226.
15. Farrell PM. The meaning of early diagnosis in a new era of CF care. Pediatrics 2007;119:156-157.
16. Lai HJ, Cheng Y, Farrell PM. The survival advantage of patients with cystic fibrosis diagnosed through neonatal screening: evidence from the United States Cystic Fibrosis Foundation registry data. J Pediatr 2005; S57-S63.
17. Farrell PM, Lai HJ, Li Z, Kosorok MR, Laxova A, Green CG, Collins J, Hoffman G, Laessig R, Rock MJ, Splaingard ML. Evidence on improved outcomes with early diagnosis of cystic fibrosis through neonatal screening: enough is enough! J Pediatr 2005;147:S30-S36.
18. Narzi L, Terraguti G, Stamato A, Narzi F, Valentini SB, Lelli A, Delaroche I, Lucarelli M, Strom R, Ouattrucci S. Does cyctic fibrosis neonatal screening detect atypical CF forms? Extended genetic characterization and 4-year clinical follow-up. Clin Genet 2007;72:39-46.
19. Delmarco A, Pradal U, Cabrini G, Bonizzato A, Mastella G. Nasal potaential difference in cystic fibrosis patient presenting borderline sweat test. Eur Respir J 1997;10:1145-1149.
20. Coste A, Giridon E, Louis S, Pruliere-Escabasse V, Goossens M, Peynegre R, Escudier E. Atypical sinusitis in adults must lead to looking for cystic fibrosis and primary ciliary dyskinesia. Laryngoscpe 2004;114:839-843.
21. Raman V, Clary R, Siegrist KL, Zehnbauer B, Chatila TA. Increased prevalence of mutations in the cystic fibrosis transmembrane conductance regulator in children with chronic rhinosinusitis. Pediatrics 2002;109:E13.