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Items: 25

1.

Nasal potential difference in suspected cystic fibrosis patients with 5T polymorphism.

Aalbers BL, Yaakov Y, Derichs N, Simmonds NJ, De Wachter E, Melotti P, De Boeck K, Leal T, Tümmler B, Wilschanski M, Bronsveld I.

J Cyst Fibros. 2019 Jul 19. pii: S1569-1993(19)30822-7. doi: 10.1016/j.jcf.2019.07.001. [Epub ahead of print]

PMID:
31331863
2.

GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1).

Davies JC, Van de Steen O, van Koningsbruggen-Rietschel S, Drevinek P, Derichs N, McKone EF, Kanters D, Allamassey L, Namour F, de Kock H, Conrath K.

J Cyst Fibros. 2019 Sep;18(5):693-699. doi: 10.1016/j.jcf.2019.05.006. Epub 2019 May 27.

3.

Real life practice of sweat testing in Europe.

Cirilli N, Southern KW, Buzzetti R, Barben J, Nährlich L, Munck A, Wilschanski M, De Boeck K, Derichs N; of the ECFS Diagnostic Network Working Group.

J Cyst Fibros. 2017 Sep 27. pii: S1569-1993(17)30881-0. doi: 10.1016/j.jcf.2017.09.002. [Epub ahead of print]

PMID:
28964647
4.

Diagnosis of Cystic Fibrosis in Nonscreened Populations.

Sosnay PR, White TB, Farrell PM, Ren CL, Derichs N, Howenstine MS, Nick JA, De Boeck K.

J Pediatr. 2017 Feb;181S:S52-S57.e2. doi: 10.1016/j.jpeds.2016.09.068.

PMID:
28129813
5.

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.

Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, Howenstine M, McColley SA, Rock M, Rosenfeld M, Sermet-Gaudelus I, Southern KW, Marshall BC, Sosnay PR.

J Pediatr. 2017 Feb;181S:S4-S15.e1. doi: 10.1016/j.jpeds.2016.09.064. Erratum in: J Pediatr. 2017 May;184:243.

6.

Cystic Fibrosis Diagnostic Challenges over 4 Decades: Historical Perspectives and Lessons Learned.

Farrell PM, White TB, Derichs N, Castellani C, Rosenstein BJ.

J Pediatr. 2017 Feb;181S:S16-S26. doi: 10.1016/j.jpeds.2016.09.067.

PMID:
28129808
7.

Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): A new designation and management recommendations for infants with an inconclusive diagnosis following newborn screening.

Munck A, Mayell SJ, Winters V, Shawcross A, Derichs N, Parad R, Barben J, Southern KW; ECFS Neonatal Screening Working Group.

J Cyst Fibros. 2015 Nov;14(6):706-13. doi: 10.1016/j.jcf.2015.01.001. Epub 2015 Jan 24.

8.

CFTR functional measurements in human models for diagnosis, prognosis and personalized therapy: Report on the pre-conference meeting to the 11th ECFS Basic Science Conference, Malta, 26-29 March 2014.

Beekman JM, Sermet-Gaudelus I, de Boeck K, Gonska T, Derichs N, Mall MA, Mehta A, Martin U, Drumm M, Amaral MD.

J Cyst Fibros. 2014 Jul;13(4):363-72. doi: 10.1016/j.jcf.2014.05.007. Epub 2014 Jun 2. Review. No abstract available.

9.

Nasal potential difference measurements in diagnosis of cystic fibrosis: an international survey.

Naehrlich L, Ballmann M, Davies J, Derichs N, Gonska T, Hjelte L, van Konigsbruggen-Rietschel S, Leal T, Melotti P, Middleton P, Tümmler B, Vermeulen F, Wilschanski M; ECFS Diagnostic Network Working Group.

J Cyst Fibros. 2014 Jan;13(1):24-8. doi: 10.1016/j.jcf.2013.08.006. Epub 2013 Sep 7.

10.

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.

Derichs N.

Eur Respir Rev. 2013 Mar 1;22(127):58-65. doi: 10.1183/09059180.00008412. Review.

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.

Immunochemical analysis of mutant CFTR in lung explants.

van Barneveld A, Zander I, Hyde R, Länger F, Simon A, Krüger M, Ballmann M, Derichs N, Tümmler B.

Cell Physiol Biochem. 2012;30(3):587-95. doi: 10.1159/000341440. Epub 2012 Jul 25.

13.

Functional fluorescently labeled bithiazole ΔF508-CFTR corrector imaged in whole body slices in mice.

Davison HR, Taylor S, Drake C, Phuan PW, Derichs N, Yao C, Jones EF, Sutcliffe J, Verkman AS, Kurth MJ.

Bioconjug Chem. 2011 Dec 21;22(12):2593-9. doi: 10.1021/bc2004457. Epub 2011 Nov 18.

14.

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.

15.

Recommendations for the classification of diseases as CFTR-related disorders.

Bombieri C, Claustres M, De Boeck K, Derichs N, Dodge J, Girodon E, Sermet I, Schwarz M, Tzetis M, Wilschanski M, Bareil C, Bilton D, Castellani C, Cuppens H, Cutting GR, Drevínek P, Farrell P, Elborn JS, Jarvi K, Kerem B, Kerem E, Knowles M, Macek M Jr, Munck A, Radojkovic D, Seia M, Sheppard DN, Southern KW, Stuhrmann M, Tullis E, Zielenski J, Pignatti PF, Ferec C.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S86-102. doi: 10.1016/S1569-1993(11)60014-3.

16.

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
17.

Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching.

Derichs N, Jin BJ, Song Y, Finkbeiner WE, Verkman AS.

FASEB J. 2011 Jul;25(7):2325-32. doi: 10.1096/fj.10-179549. Epub 2011 Mar 22.

18.

Airway surface liquid depth imaged by surface laser reflectance microscopy.

Thiagarajah JR, Song Y, Derichs N, Verkman AS.

J Gen Physiol. 2010 Sep;136(3):353-62. doi: 10.1085/jgp.201010490. Epub 2010 Aug 16.

19.

Functional analysis of F508del CFTR in native human colon.

van Barneveld A, Stanke F, Tamm S, Siebert B, Brandes G, Derichs N, Ballmann M, Junge S, Tümmler B.

Biochim Biophys Acta. 2010 Nov;1802(11):1062-9. doi: 10.1016/j.bbadis.2010.08.001. Epub 2010 Aug 7.

20.

Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data.

Derichs N, Sanz J, Von Kanel T, Stolpe C, Zapf A, Tümmler B, Gallati S, Ballmann M.

Thorax. 2010 Jul;65(7):594-9. doi: 10.1136/thx.2009.125088.

PMID:
20627915
21.

Phenotypic characterisation of patients with intermediate sweat chloride values: towards validation of the European diagnostic algorithm for cystic fibrosis.

Goubau C, Wilschanski M, Skalická V, Lebecque P, Southern KW, Sermet I, Munck A, Derichs N, Middleton PG, Hjelte L, Padoan R, Vasar M, De Boeck K.

Thorax. 2009 Aug;64(8):683-91. doi: 10.1136/thx.2008.104752. Epub 2009 Mar 23.

PMID:
19318346
22.

Evaluation of portal venous gas detected by ultrasound examination for diagnosis of necrotising enterocolitis.

Dördelmann M, Rau GA, Bartels D, Linke M, Derichs N, Behrens C, Bohnhorst B.

Arch Dis Child Fetal Neonatal Ed. 2009 May;94(3):F183-7. doi: 10.1136/adc.2007.132019. Epub 2008 Sep 11.

PMID:
18786962
23.

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.

24.

Homozygosity for L997F in a child with normal clinical and chloride secretory phenotype provides evidence that this cystic fibrosis transmembrane conductance regulator mutation does not cause cystic fibrosis.

Derichs N, Schuster A, Grund I, Ernsting A, Stolpe C, Körtge-Jung S, Gallati S, Stuhrmann M, Kozlowski P, Ballmann M.

Clin Genet. 2005 Jun;67(6):529-31. No abstract available.

PMID:
15857421
25.

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

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