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Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancy.
Kerby GS, Wagner BD, Popler J, Hay TC, Kopecky C, Wilcox SL, Quinones RR, Giller RH, Accurso FJ, Deterding RR. Kerby GS, et al. Among authors: accurso fj. Pediatr Pulmonol. 2013 Oct;48(10):1008-15. doi: 10.1002/ppul.22718. Epub 2012 Nov 20. Pediatr Pulmonol. 2013. PMID: 23169677
Initial FEV0.5 and FRC z-scores correlated with RA O2 sat (r = 0.60 and -0.49) at short-term follow up (6-12 months). Most measurements of RVRTC correlated with FEV1 (n = 5) measured 4-5 years later (r > 0.50). ...
Initial FEV0.5 and FRC z-scores correlated with RA O2 sat (r = 0.60 and -0.49) at short-term follow up (6-12 months). Most measuremen …
Long term effects of denufosol tetrasodium in patients with cystic fibrosis.
Ratjen F, Durham T, Navratil T, Schaberg A, Accurso FJ, Wainwright C, Barnes M, Moss RB; TIGER-2 Study Investigator Group. Ratjen F, et al. Among authors: accurso fj. J Cyst Fibros. 2012 Dec;11(6):539-49. doi: 10.1016/j.jcf.2012.05.003. Epub 2012 Jun 8. J Cyst Fibros. 2012. PMID: 22682898 Free article. Clinical Trial.
Urinary desmosine: a biomarker of structural lung injury during CF pulmonary exacerbation.
Laguna TA, Wagner BD, Starcher B, Luckey Tarro HK, Mann SA, Sagel SD, Accurso FJ. Laguna TA, et al. Among authors: accurso fj. Pediatr Pulmonol. 2012 Sep;47(9):856-63. doi: 10.1002/ppul.22525. Epub 2012 Mar 19. Pediatr Pulmonol. 2012. PMID: 22431382 Free PMC article.
The non-invasive measurement of urinary desmosine (UDes), a breakdown product of elastin, may be reflective of ongoing lung injury and may serve as a biomarker of active short-term damage during pulmonary exacerbation. Our objectives were to measure desmosine in the urine …
The non-invasive measurement of urinary desmosine (UDes), a breakdown product of elastin, may be reflective of ongoing lung injury and may s …
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.
Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, De Boeck K, Donaldson SH, Dorkin HL, Dunitz JM, Durie PR, Jain M, Leonard A, McCoy KS, Moss RB, Pilewski JM, Rosenbluth DB, Rubenstein RC, Schechter MS, Botfield M, Ordoñez CL, Spencer-Green GT, Vernillet L, Wisseh S, Yen K, Konstan MW. Clancy JP, et al. Among authors: accurso fj. Thorax. 2012 Jan;67(1):12-8. doi: 10.1136/thoraxjnl-2011-200393. Epub 2011 Aug 8. Thorax. 2012. PMID: 21825083 Free PMC article. Clinical Trial.
Additional data are needed to determine how improvements detected in CFTR function secondary to VX-809 in the sweat gland relate to those measurable in the respiratory tract and to long-term measures of clinical benefit. CLINICAL TRIAL NUMBER: NCT00865904....
Additional data are needed to determine how improvements detected in CFTR function secondary to VX-809 in the sweat gland relate to those me …
Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis.
Nick JA, Chacon CS, Brayshaw SJ, Jones MC, Barboa CM, St Clair CG, Young RL, Nichols DP, Janssen JS, Huitt GA, Iseman MD, Daley CL, Taylor-Cousar JL, Accurso FJ, Saavedra MT, Sontag MK. Nick JA, et al. Among authors: accurso fj. Am J Respir Crit Care Med. 2010 Sep 1;182(5):614-26. doi: 10.1164/rccm.201001-0092OC. Epub 2010 May 6. Am J Respir Crit Care Med. 2010. PMID: 20448091 Free PMC article.
RATIONALE: Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed with classic manifestations in childhood, and nonclassic phenotypes typically diagnosed as adults. ...OBJECTIVES: Examine effects of age at d …
RATIONALE: Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed w …
Cystic Fibrosis Foundation practice guidelines for the management of infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome during the first two years of life and beyond.
Cystic Fibrosis Foundation; Borowitz D, Parad RB, Sharp JK, Sabadosa KA, Robinson KA, Rock MJ, Farrell PM, Sontag MK, Rosenfeld M, Davis SD, Marshall BC, Accurso FJ. Cystic Fibrosis Foundation, et al. Among authors: accurso fj. J Pediatr. 2009 Dec;155(6 Suppl):S106-16. doi: 10.1016/j.jpeds.2009.09.003. J Pediatr. 2009. PMID: 19914443 Free PMC article.
Mutations in the CFTR gene can cause CF, but not all CFTR mutations are disease-causing. The term CFTR-related metabolic syndrome (CRMS) is proposed to describe infants identified by hypertrypsinogenemia on NBS who have sweat chloride values <60 mmol/L and up to 2 CFTR …
Mutations in the CFTR gene can cause CF, but not all CFTR mutations are disease-causing. The term CFTR-related metabolic syndrome (CR …
Association of socioeconomic status with the use of chronic therapies and healthcare utilization in children with cystic fibrosis.
Schechter MS, McColley SA, Silva S, Haselkorn T, Konstan MW, Wagener JS; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis; North American Scientific Advisory Group for ESCF. Schechter MS, et al. J Pediatr. 2009 Nov;155(5):634-9.e1-4. doi: 10.1016/j.jpeds.2009.04.059. Epub 2009 Jul 16. J Pediatr. 2009. PMID: 19608199 Free PMC article.
Sputum desmosine during hospital admission for pulmonary exacerbation in cystic fibrosis.
Laguna TA, Wagner BD, Luckey HK, Mann SA, Sagel SD, Regelmann W, Accurso FJ. Laguna TA, et al. Among authors: accurso fj. Chest. 2009 Dec;136(6):1561-1568. doi: 10.1378/chest.09-0217. Epub 2009 Jun 30. Chest. 2009. PMID: 19567495 Free PMC article.
The noninvasive measurement of desmosine, a breakdown product of elastin, may reflect ongoing lung injury and serve as a biomarker of short-term damage. Our objectives were to measure desmosine in the sputum of patients with CF hospitalized for treatment of a pulmonary exa …
The noninvasive measurement of desmosine, a breakdown product of elastin, may reflect ongoing lung injury and serve as a biomarker of short- …
Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes.
Sontag MK, Hammond KB, Zielenski J, Wagener JS, Accurso FJ. Sontag MK, et al. Among authors: accurso fj. J Pediatr. 2005 Sep;147(3 Suppl):S83-8. doi: 10.1016/j.jpeds.2005.08.005. J Pediatr. 2005. PMID: 16202790
Finally, infants with persistent hypertrypsinogenemia and borderline sweat test results need long-term follow-up....
Finally, infants with persistent hypertrypsinogenemia and borderline sweat test results need long-term follow-up....
Late diagnosis defines a unique population of long-term survivors of cystic fibrosis.
Rodman DM, Polis JM, Heltshe SL, Sontag MK, Chacon C, Rodman RV, Brayshaw SJ, Huitt GA, Iseman MD, Saavedra MT, Taussig LM, Wagener JS, Accurso FJ, Nick JA. Rodman DM, et al. Among authors: accurso fj. Am J Respir Crit Care Med. 2005 Mar 15;171(6):621-6. doi: 10.1164/rccm.200403-404OC. Epub 2004 Dec 10. Am J Respir Crit Care Med. 2005. PMID: 15591474
This is the largest cohort of older patients with CF described to date, and our findings indicate that patients diagnosed as adults differ distinctly from survivors of long-term CF diagnosed as children....
This is the largest cohort of older patients with CF described to date, and our findings indicate that patients diagnosed as adults differ d …
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