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Items: 26

1.

Is sweat chloride predictive of severity of cystic fibrosis lung disease assessed by chest computed tomography?

Caudri D, Zitter D, Bronsveld I, Tiddens H.

Pediatr Pulmonol. 2017 Sep;52(9):1135-1141. doi: 10.1002/ppul.23739. Epub 2017 Jun 6.

PMID:
28586522
2.

β2-Adrenergic receptor agonists activate CFTR in intestinal organoids and subjects with cystic fibrosis.

Vijftigschild LA, Berkers G, Dekkers JF, Zomer-van Ommen DD, Matthes E, Kruisselbrink E, Vonk A, Hensen CE, Heida-Michel S, Geerdink M, Janssens HM, van de Graaf EA, Bronsveld I, de Winter-de Groot KM, Majoor CJ, Heijerman HG, de Jonge HR, Hanrahan JW, van der Ent CK, Beekman JM.

Eur Respir J. 2016 Sep;48(3):768-79. doi: 10.1183/13993003.01661-2015. Epub 2016 Jul 28.

3.

Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis.

Dekkers JF, Berkers G, Kruisselbrink E, Vonk A, de Jonge HR, Janssens HM, Bronsveld I, van de Graaf EA, Nieuwenhuis EE, Houwen RH, Vleggaar FP, Escher JC, de Rijke YB, Majoor CJ, Heijerman HG, de Winter-de Groot KM, Clevers H, van der Ent CK, Beekman JM.

Sci Transl Med. 2016 Jun 22;8(344):344ra84. doi: 10.1126/scitranslmed.aad8278.

PMID:
27334259
4.

Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations.

Dekkers JF, Van Mourik P, Vonk AM, Kruisselbrink E, Berkers G, de Winter-de Groot KM, Janssens HM, Bronsveld I, van der Ent CK, de Jonge HR, Beekman JM.

J Cyst Fibros. 2016 Sep;15(5):568-78. doi: 10.1016/j.jcf.2016.04.007. Epub 2016 May 5.

5.

Relationship between lung function and Modified Shuttle Test performance in adult patients with cystic fibrosis: a cross-sectional, retrospective study.

Doeleman WR, Takken T, Bronsveld I, Hulzebos EH.

Physiotherapy. 2016 Jun;102(2):184-8. doi: 10.1016/j.physio.2015.10.015. Epub 2015 Dec 12.

PMID:
27101722
6.

Emphysema Is Common in Lungs of Cystic Fibrosis Lung Transplantation Patients: A Histopathological and Computed Tomography Study.

Mets OM, Roothaan SM, Bronsveld I, Luijk B, van de Graaf EA, Vink A, de Jong PA.

PLoS One. 2015 Jun 5;10(6):e0128062. doi: 10.1371/journal.pone.0128062. eCollection 2015.

7.

Inhibition of Heat-Stable Toxin-Induced Intestinal Salt and Water Secretion by a Novel Class of Guanylyl Cyclase C Inhibitors.

Bijvelds MJ, Loos M, Bronsveld I, Hellemans A, Bongartz JP, Ver Donck L, Cox E, de Jonge HR, Schuurkes JA, De Maeyer JH.

J Infect Dis. 2015 Dec 1;212(11):1806-15. doi: 10.1093/infdis/jiv300. Epub 2015 May 21.

PMID:
25999056
8.

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Kerem E, Konstan MW, De Boeck K, Accurso FJ, Sermet-Gaudelus I, Wilschanski M, Elborn JS, Melotti P, Bronsveld I, Fajac I, Malfroot A, Rosenbluth DB, Walker PA, McColley SA, Knoop C, Quattrucci S, Rietschel E, Zeitlin PL, Barth J, Elfring GL, Welch EM, Branstrom A, Spiegel RJ, Peltz SW, Ajayi T, Rowe SM; Cystic Fibrosis Ataluren Study Group.

Lancet Respir Med. 2014 Jul;2(7):539-47. doi: 10.1016/S2213-2600(14)70100-6. Epub 2014 May 15.

9.

A functional CFTR assay using primary cystic fibrosis intestinal organoids.

Dekkers JF, Wiegerinck CL, de Jonge HR, Bronsveld I, Janssens HM, de Winter-de Groot KM, Brandsma AM, de Jong NW, Bijvelds MJ, Scholte BJ, Nieuwenhuis EE, van den Brink S, Clevers H, van der Ent CK, Middendorp S, Beekman JM.

Nat Med. 2013 Jul;19(7):939-45. doi: 10.1038/nm.3201. Epub 2013 Jun 2.

PMID:
23727931
10.

Influence of perfusate temperature on nasal potential difference.

Bronsveld I, Vermeulen F, Sands D, Leal T, Leonard A, Melotti P, Yaakov Y, de Nooijer R, De Boeck K, Sermet I, Wilschanski M, Middleton PG; European Cystic Fibrosis Society – Diagnostic Network Working Group.

Eur Respir J. 2013 Aug;42(2):389-93. doi: 10.1183/09031936.00097712. Epub 2012 Oct 25.

11.

CFTR biomarkers: time for promotion to surrogate end-point.

De Boeck K, Kent L, Davies J, Derichs N, Amaral M, Rowe SM, Middleton P, de Jonge H, Bronsveld I, Wilschanski M, Melotti P, Danner-Boucher I, Boerner S, Fajac I, Southern K, de Nooijer RA, Bot A, de Rijke Y, de Wachter E, Leal T, Vermeulen F, Hug MJ, Rault G, Nguyen-Khoa T, Barreto C, Proesmans M, Sermet-Gaudelus I; European Cystic Fibrosis Society Clinical Trial Network Standardisation Committee.

Eur Respir J. 2013 Jan;41(1):203-16. doi: 10.1183/09031936.00057512. Epub 2012 Aug 9. Review.

12.

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.

Thorax. 2012 Jan;67(1):12-8. doi: 10.1136/thoraxjnl-2011-200393. Epub 2011 Aug 8.

13.

New clinical diagnostic procedures for cystic fibrosis in Europe.

De Boeck K, Derichs N, Fajac I, de Jonge HR, Bronsveld I, Sermet I, Vermeulen F, Sheppard DN, Cuppens H, Hug M, Melotti P, Middleton PG, Wilschanski M; ECFS Diagnostic Network Working Group; EuroCareCF WP3 Group on CF diagnosis.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S53-66. doi: 10.1016/S1569-1993(11)60009-X. Review.

14.

Measurement of ion transport function in rectal biopsies.

Hug MJ, Derichs N, Bronsveld I, Clancy JP.

Methods Mol Biol. 2011;741:87-107. doi: 10.1007/978-1-61779-117-8_7.

PMID:
21594780
15.

Assessment of CFTR function in homozygous R117H-7T subjects.

de Nooijer RA, Nobel JM, Arets HG, Bot AG, van Berkhout FT, de Rijke YB, de Jonge HR, Bronsveld I.

J Cyst Fibros. 2011 Sep;10(5):326-32. doi: 10.1016/j.jcf.2011.03.009. Epub 2011 Apr 19.

16.

Transient correction of the basic defect in sweat glands in an individual with cystic fibrosis carrying the complex CFTR allele F508del-R553Q.

Tümmler B, Stanke F, Bronsveld I, Veeze H, Ballmann M.

Thorax. 2009 Feb;64(2):179-80. doi: 10.1136/thx.2008.096123.

PMID:
19176844
17.

Diversity of the basic defect of homozygous CFTR mutation genotypes in humans.

Stanke F, Ballmann M, Bronsveld I, Dörk T, Gallati S, Laabs U, Derichs N, Ritzka M, Posselt HG, Harms HK, Griese M, Blau H, Mastella G, Bijman J, Veeze H, Tümmler B.

J Med Genet. 2008 Jan;45(1):47-54. doi: 10.1136/jmg.2007.053561.

18.

Fat absorption in cystic fibrosis mice is impeded by defective lipolysis and post-lipolytic events.

Bijvelds MJ, Bronsveld I, Havinga R, Sinaasappel M, de Jonge HR, Verkade HJ.

Am J Physiol Gastrointest Liver Physiol. 2005 Apr;288(4):G646-53. Epub 2004 Nov 4.

19.

Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers.

De Jonge HR, Ballmann M, Veeze H, Bronsveld I, Stanke F, Tümmler B, Sinaasappel M.

J Cyst Fibros. 2004 Aug;3 Suppl 2:159-63. Review.

20.

Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients.

Derichs N, Mekus F, Bronsveld I, Bijman J, Veeze HJ, von der Hardt H, Tummler B, Ballmann M.

Pediatr Res. 2004 Jan;55(1):69-75. Epub 2003 Nov 6.

PMID:
14605249
21.

Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings.

Bronsveld I, Mekus F, Bijman J, Ballmann M, de Jonge HR, Laabs U, Halley DJ, Ellemunter H, Mastella G, Thomas S, Veeze HJ, Tümmler B.

J Clin Invest. 2001 Dec;108(11):1705-15.

22.

Categories of deltaF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics.

Mekus F, Ballmann M, Bronsveld I, Bijman J, Veeze H, Tümmler B.

Twin Res. 2000 Dec;3(4):277-93.

PMID:
11463149
23.

Residual chloride secretion in intestinal tissue of deltaF508 homozygous twins and siblings with cystic fibrosis. The European CF Twin and Sibling Study Consortium.

Bronsveld I, Mekus F, Bijman J, Ballmann M, Greipel J, Hundrieser J, Halley DJ, Laabs U, Busche R, De Jonge HR, Tümmler B, Veeze HJ.

Gastroenterology. 2000 Jul;119(1):32-40.

PMID:
10889152
24.

Clinical presentation of exclusive cystic fibrosis lung disease.

Bronsveld I, Bijman J, Mekus F, Ballmann M, Veeze HJ, Tümmler B.

Thorax. 1999 Mar;54(3):278-81.

25.

Cystic-fibrosis-like disease unrelated to the cystic fibrosis transmembrane conductance regulator.

Mekus F, Ballmann M, Bronsveld I, Dörk T, Bijman J, Tümmler B, Veeze HJ.

Hum Genet. 1998 May;102(5):582-6.

PMID:
9654209
26.

Exocrine pancreatic alterations in long-lived surviving cystic fibrosis mice.

Ip WF, Bronsveld I, Kent G, Corey M, Durie PR.

Pediatr Res. 1996 Aug;40(2):242-9.

PMID:
8827772

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