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Results: 1 to 20 of 35

1.

Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, Sagel SD, Khan U, Mayer-Hamblett N, Van Dalfsen JM, Joseloff E, Ramsey BW; GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network.

Am J Respir Crit Care Med. 2014 Jul 15;190(2):175-84. doi: 10.1164/rccm.201404-0703OC.

PMID:
24927234
[PubMed - indexed for MEDLINE]
2.

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. No abstract available.

PMID:
24882694
[PubMed - in process]
3.

Design and Implementation of INSPPIRE.

Morinville VD, Lowe ME, Ahuja M, Barth B, Bellin MD, Davis H, Durie PR, Finley B, Fishman DS, Freedman SD, Gariepy CE, Giefer MJ, Gonska T, Heyman MB, Himes R, Husain S, Kumar S, Ooi CY, Pohl JF, Schwarzenberg SJ, Troendle D, Werlin SL, Wilschanski M, Yen E, Uc A.

J Pediatr Gastroenterol Nutr. 2014 Sep;59(3):360-4. doi: 10.1097/MPG.0000000000000417.

PMID:
24824361
[PubMed - in process]
4.

VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface.

Eckford PD, Ramjeesingh M, Molinski S, Pasyk S, Dekkers JF, Li C, Ahmadi S, Ip W, Chung TE, Du K, Yeger H, Beekman J, Gonska T, Bear CE.

Chem Biol. 2014 May 22;21(5):666-78. doi: 10.1016/j.chembiol.2014.02.021. Epub 2014 Apr 10.

PMID:
24726831
[PubMed - in process]
5.

Does integration of various ion channel measurements improve diagnostic performance in cystic fibrosis?

Ooi CY, Dupuis A, Gonska T, Ellis L, Ni A, Jarvi K, Martin S, Ray P, Steele L, Kortan P, Dorfman R, Solomon M, Zielenski J, Corey M, Tullis E, Durie P.

Ann Am Thorac Soc. 2014 May;11(4):562-70. doi: 10.1513/AnnalsATS.201311-412OC.

PMID:
24697796
[PubMed - in process]
6.

An infant with vomiting, diarrhea, and failure to thrive. Chylomicron retention disease.

Mouzaki M, Vresk L, Gonska T.

Gastroenterology. 2014 Apr;146(4):912, 1137-8. doi: 10.1053/j.gastro.2013.11.035. Epub 2014 Feb 19. No abstract available.

PMID:
24560855
[PubMed - indexed for MEDLINE]
7.

Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention.

Molinski SV, Gonska T, Huan LJ, Baskin B, Janahi IA, Ray PN, Bear CE.

Genet Med. 2014 Aug;16(8):625-32. doi: 10.1038/gim.2014.4. Epub 2014 Feb 20.

PMID:
24556927
[PubMed - in process]
8.

Total pancreatectomy and islet autotransplantation in chronic pancreatitis: recommendations from PancreasFest.

Bellin MD, Freeman ML, Gelrud A, Slivka A, Clavel A, Humar A, Schwarzenberg SJ, Lowe ME, Rickels MR, Whitcomb DC, Matthews JB; PancreasFest Recommendation Conference Participants, Amann S, Andersen DK, Anderson MA, Baillie J, Block G, Brand R, Chari S, Cook M, Cote GA, Dunn T, Frulloni L, Greer JB, Hollingsworth MA, Kim KM, Larson A, Lerch MM, Lin T, Muniraj T, Robertson RP, Sclair S, Singh S, Stopczynski R, Toledo FG, Wilcox CM, Windsor J, Yadav D.

Pancreatology. 2014 Jan-Feb;14(1):27-35. doi: 10.1016/j.pan.2013.10.009. Epub 2013 Nov 13.

PMID:
24555976
[PubMed - in process]
9.

The intestinal epithelial insulin-like growth factor-1 receptor links glucagon-like peptide-2 action to gut barrier function.

Dong CX, Zhao W, Solomon C, Rowland KJ, Ackerley C, Robine S, Holzenberger M, Gonska T, Brubaker PL.

Endocrinology. 2014 Feb;155(2):370-9. doi: 10.1210/en.2013-1871. Epub 2013 Nov 21.

PMID:
24265452
[PubMed - indexed for MEDLINE]
10.

Does extensive genotyping and nasal potential difference testing clarify the diagnosis of cystic fibrosis among patients with single-organ manifestations of cystic fibrosis?

Ooi CY, Dupuis A, Ellis L, Jarvi K, Martin S, Ray PN, Steele L, Kortan P, Gonska T, Dorfman R, Solomon M, Zielenski J, Corey M, Tullis E, Durie P.

Thorax. 2014 Mar;69(3):254-60. doi: 10.1136/thoraxjnl-2013-203832. Epub 2013 Oct 21.

PMID:
24149827
[PubMed - indexed for MEDLINE]
11.

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.

PMID:
24022019
[PubMed - indexed for MEDLINE]
12.

Detection, evaluation and treatment of diabetes mellitus in chronic pancreatitis: recommendations from PancreasFest 2012.

Rickels MR, Bellin M, Toledo FG, Robertson RP, Andersen DK, Chari ST, Brand R, Frulloni L, Anderson MA, Whitcomb DC; PancreasFest Recommendation Conference Participants.

Pancreatology. 2013 Jul-Aug;13(4):336-42. doi: 10.1016/j.pan.2013.05.002. Epub 2013 May 17.

PMID:
23890130
[PubMed - indexed for MEDLINE]
Free PMC Article
13.

Severely elevated transaminases in an adolescent male with anorexia nervosa.

Smith RW, Korenblum C, Thacker K, Bonifacio HJ, Gonska T, Katzman DK.

Int J Eat Disord. 2013 Nov;46(7):751-4. doi: 10.1002/eat.22162. Epub 2013 Jul 23.

PMID:
23881604
[PubMed - indexed for MEDLINE]
15.

β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis.

Quinton P, Molyneux L, Ip W, Dupuis A, Avolio J, Tullis E, Conrad D, Shamsuddin AK, Durie P, Gonska T.

Am J Respir Crit Care Med. 2012 Oct 15;186(8):732-9. doi: 10.1164/rccm.201205-0922OC. Epub 2012 Aug 2.

PMID:
22859523
[PubMed - indexed for MEDLINE]
16.

Comparing the American and European diagnostic guidelines for cystic fibrosis: same disease, different language?

Ooi CY, Dupuis A, Ellis L, Jarvi K, Martin S, Gonska T, Dorfman R, Kortan P, Solomon M, Tullis E, Durie PR.

Thorax. 2012 Jul;67(7):618-24. doi: 10.1136/thoraxjnl-2011-201454. Epub 2012 Apr 15.

PMID:
22504961
[PubMed - indexed for MEDLINE]
17.

Role of cystic fibrosis transmembrane conductance regulator in patients with chronic sinopulmonary disease.

Gonska T, Choi P, Stephenson A, Ellis L, Martin S, Solomon M, Dupuis A, Dorfman R, Zielenski J, Ooi CY, Weiser W, Durie PR, Tullis E.

Chest. 2012 Oct;142(4):996-1004.

PMID:
22423042
[PubMed - indexed for MEDLINE]
18.

Definitions of pediatric pancreatitis and survey of present clinical practices.

Morinville VD, Husain SZ, Bai H, Barth B, Alhosh R, Durie PR, Freedman SD, Himes R, Lowe ME, Pohl J, Werlin S, Wilschanski M, Uc A; INSPPIRE Group.

J Pediatr Gastroenterol Nutr. 2012 Sep;55(3):261-5. Erratum in: J Pediatr Gastroenterol Nutr. 2013 Apr;56(4):459. Abu-Al-Haija, Maisam [corrected to Abu-El-Haija, Maisam].

PMID:
22357117
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Early in vivo testing to assess new therapeutic interventions in CF patients.

Gonska T.

Curr Pharm Des. 2012;18(5):663-73. Review.

PMID:
22229557
[PubMed - indexed for MEDLINE]
20.

Type of CFTR mutation determines risk of pancreatitis in patients with cystic fibrosis.

Ooi CY, Dorfman R, Cipolli M, Gonska T, Castellani C, Keenan K, Freedman SD, Zielenski J, Berthiaume Y, Corey M, Schibli S, Tullis E, Durie PR.

Gastroenterology. 2011 Jan;140(1):153-61. doi: 10.1053/j.gastro.2010.09.046. Epub 2010 Nov 9.

PMID:
20923678
[PubMed - indexed for MEDLINE]

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