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Results: 1 to 20 of 89

1.

Recent advances in cystic fibrosis.

Milla CE, Moss RB.

Curr Opin Pediatr. 2015 Apr 17. [Epub ahead of print]

PMID:
25888148
2.

Lung matrix and vascular remodeling in mechanically ventilated elastin haploinsufficient newborn mice.

Hilgendorff A, Parai K, Ertsey R, Navarro E, Jain N, Carandang F, Peterson J, Mokres L, Milla C, Preuss S, Alcazar MA, Khan S, Masumi J, Ferreira-Tojais N, Mujahid S, Starcher B, Rabinovitch M, Bland R.

Am J Physiol Lung Cell Mol Physiol. 2015 Mar 1;308(5):L464-78. doi: 10.1152/ajplung.00278.2014. Epub 2014 Dec 24.

PMID:
25539853
3.

Clinical features of childhood primary ciliary dyskinesia by genotype and ultrastructural phenotype.

Davis SD, Ferkol TW, Rosenfeld M, Lee HS, Dell SD, Sagel SD, Milla C, Zariwala MA, Pittman JE, Shapiro AJ, Carson JL, Krischer JP, Hazucha MJ, Cooper ML, Knowles MR, Leigh MW.

Am J Respir Crit Care Med. 2015 Feb 1;191(3):316-24. doi: 10.1164/rccm.201409-1672OC.

PMID:
25493340
4.

Study design considerations for evaluating the efficacy and safety of pancreatic enzyme replacement therapy in patients with cystic fibrosis.

Konstan MW, Borowitz D, Mayer-Hamblett N, Milla C, Hendeles L, Murray S, Kronmal RA, Casey S, Rose LM, Morgan WJ, Ramsey BW.

Clin Investig (Lond). 2013 Aug;3(8):731-741.

5.

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
6.

Laterality defects other than situs inversus totalis in primary ciliary dyskinesia: insights into situs ambiguus and heterotaxy.

Shapiro AJ, Davis SD, Ferkol T, Dell SD, Rosenfeld M, Olivier KN, Sagel SD, Milla C, Zariwala MA, Wolf W, Carson JL, Hazucha MJ, Burns K, Robinson B, Knowles MR, Leigh MW.

Chest. 2014 Nov;146(5):1176-86. doi: 10.1378/chest.13-1704.

PMID:
24577564
7.

Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype.

Knowles MR, Ostrowski LE, Leigh MW, Sears PR, Davis SD, Wolf WE, Hazucha MJ, Carson JL, Olivier KN, Sagel SD, Rosenfeld M, Ferkol TW, Dell SD, Milla CE, Randell SH, Yin W, Sannuti A, Metjian HM, Noone PG, Noone PJ, Olson CA, Patrone MV, Dang H, Lee HS, Hurd TW, Gee HY, Otto EA, Halbritter J, Kohl S, Kircher M, Krischer J, Bamshad MJ, Nickerson DA, Hildebrandt F, Shendure J, Zariwala MA.

Am J Respir Crit Care Med. 2014 Mar 15;189(6):707-17. doi: 10.1164/rccm.201311-2047OC.

8.

A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor.

Char JE, Wolfe MH, Cho HJ, Park IH, Jeong JH, Frisbee E, Dunn C, Davies Z, Milla C, Moss RB, Thomas EA, Wine JJ.

PLoS One. 2014 Feb 10;9(2):e88564. doi: 10.1371/journal.pone.0088564. eCollection 2014.

9.

Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial.

Davies J, Sheridan H, Bell N, Cunningham S, Davis SD, Elborn JS, Milla CE, Starner TD, Weiner DJ, Lee PS, Ratjen F.

Lancet Respir Med. 2013 Oct;1(8):630-8. doi: 10.1016/S2213-2600(13)70182-6. Epub 2013 Sep 10.

PMID:
24461666
10.

In vivo readout of CFTR function: ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glands.

Wine JJ, Char JE, Chen J, Cho HJ, Dunn C, Frisbee E, Joo NS, Milla C, Modlin SE, Park IH, Thomas EA, Tran KV, Verma R, Wolfe MH.

PLoS One. 2013 Oct 24;8(10):e77114. doi: 10.1371/journal.pone.0077114. eCollection 2013.

11.

Zebrafish Ciliopathy Screen Plus Human Mutational Analysis Identifies C21orf59 and CCDC65 Defects as Causing Primary Ciliary Dyskinesia.

Austin-Tse C, Halbritter J, Zariwala MA, Gilberti RM, Gee HY, Hellman N, Pathak N, Liu Y, Panizzi JR, Patel-King RS, Tritschler D, Bower R, O'Toole E, Porath JD, Hurd TW, Chaki M, Diaz KA, Kohl S, Lovric S, Hwang DY, Braun DA, Schueler M, Airik R, Otto EA, Leigh MW, Noone PG, Carson JL, Davis SD, Pittman JE, Ferkol TW, Atkinson JJ, Olivier KN, Sagel SD, Dell SD, Rosenfeld M, Milla CE, Loges NT, Omran H, Porter ME, King SM, Knowles MR, Drummond IA, Hildebrandt F.

Am J Hum Genet. 2013 Oct 3;93(4):672-86. doi: 10.1016/j.ajhg.2013.08.015.

12.

Pricing for orphan drugs: will the market bear what society cannot?

O'Sullivan BP, Orenstein DM, Milla CE.

JAMA. 2013 Oct 2;310(13):1343-4. doi: 10.1001/jama.2013.278129. No abstract available.

PMID:
24084916
13.

Standardizing nasal nitric oxide measurement as a test for primary ciliary dyskinesia.

Leigh MW, Hazucha MJ, Chawla KK, Baker BR, Shapiro AJ, Brown DE, Lavange LM, Horton BJ, Qaqish B, Carson JL, Davis SD, Dell SD, Ferkol TW, Atkinson JJ, Olivier KN, Sagel SD, Rosenfeld M, Milla C, Lee HS, Krischer J, Zariwala MA, Knowles MR.

Ann Am Thorac Soc. 2013 Dec;10(6):574-81. doi: 10.1513/AnnalsATS.201305-110OC.

14.

Anti-PcrV antibody in cystic fibrosis: a novel approach targeting Pseudomonas aeruginosa airway infection.

Milla CE, Chmiel JF, Accurso FJ, VanDevanter DR, Konstan MW, Yarranton G, Geller DE; KB001 Study Group.

Pediatr Pulmonol. 2014 Jul;49(7):650-8. doi: 10.1002/ppul.22890. Epub 2013 Sep 9.

PMID:
24019259
15.

Novel CFTR variants identified during the first 3 years of cystic fibrosis newborn screening in California.

Prach L, Koepke R, Kharrazi M, Keiles S, Salinas DB, Reyes MC, Pian M, Opsimos H, Otsuka KN, Hardy KA, Milla CE, Zirbes JM, Chipps B, O'Bra S, Saeed MM, Sudhakar R, Lehto S, Nielson D, Shay GF, Seastrand M, Jhawar S, Nickerson B, Landon C, Thompson A, Nussbaum E, Chin T, Wojtczak H; California Cystic Fibrosis Newborn Screening Consortium.

J Mol Diagn. 2013 Sep;15(5):710-22. doi: 10.1016/j.jmoldx.2013.05.006. Epub 2013 Jun 28.

16.

Cystic fibrosis in the era of genomic medicine.

Milla CE.

Curr Opin Pediatr. 2013 Jun;25(3):323-8. doi: 10.1097/MOP.0b013e328360dbf5. Review.

PMID:
23652683
17.

Polyvinylpyrrolidone microneedles enable delivery of intact proteins for diagnostic and therapeutic applications.

Sun W, Araci Z, Inayathullah M, Manickam S, Zhang X, Bruce MA, Marinkovich MP, Lane AT, Milla C, Rajadas J, Butte MJ.

Acta Biomater. 2013 Aug;9(8):7767-74. doi: 10.1016/j.actbio.2013.04.045. Epub 2013 May 3.

18.

Exome sequencing identifies mutations in CCDC114 as a cause of primary ciliary dyskinesia.

Knowles MR, Leigh MW, Ostrowski LE, Huang L, Carson JL, Hazucha MJ, Yin W, Berg JS, Davis SD, Dell SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla CE, Olivier KN, Turner EH, Lewis AP, Bamshad MJ, Nickerson DA, Shendure J, Zariwala MA; Genetic Disorders of Mucociliary Clearance Consortium.

Am J Hum Genet. 2013 Jan 10;92(1):99-106. doi: 10.1016/j.ajhg.2012.11.003. Epub 2012 Dec 20.

19.

Role of Sonic Hedgehog in idiopathic pulmonary fibrosis.

Bolaños AL, Milla CM, Lira JC, Ramírez R, Checa M, Barrera L, García-Alvarez J, Carbajal V, Becerril C, Gaxiola M, Pardo A, Selman M.

Am J Physiol Lung Cell Mol Physiol. 2012 Dec 1;303(11):L978-90. doi: 10.1152/ajplung.00184.2012. Epub 2012 Sep 28.

20.

Quantitative analysis of the human airway microbial ecology reveals a pervasive signature for cystic fibrosis.

Blainey PC, Milla CE, Cornfield DN, Quake SR.

Sci Transl Med. 2012 Sep 26;4(153):153ra130.

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