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Items: 1 to 20 of 78

1.

Dominant-negative mutations in human IL6ST underlie hyper-IgE syndrome.

Béziat V, Tavernier SJ, Chen YH, Ma CS, Materna M, Laurence A, Staal J, Aschenbrenner D, Roels L, Worley L, Claes K, Gartner L, Kohn LA, De Bruyne M, Schmitz-Abe K, Charbonnier LM, Keles S, Nammour J, Vladikine N, Maglorius Renkilaraj MRL, Seeleuthner Y, Migaud M, Rosain J, Jeljeli M, Boisson B, Van Braeckel E, Rosenfeld JA, Dai H, Burrage LC, Murdock DR, Lambrecht BN, Avettand-Fenoel V, Vogel TP; Undiagnosed Diseases Network, Esther CR, Haskologlu S, Dogu F, Ciznar P, Boutboul D, Ouachée-Chardin M, Amourette J, Lebras MN, Gauvain C, Tcherakian C, Ikinciogullari A, Beyaert R, Abel L, Milner JD, Grimbacher B, Couderc LJ, Butte MJ, Freeman AF, Catherinot É, Fieschi C, Chatila TA, Tangye SG, Uhlig HH, Haerynck F, Casanova JL, Puel A.

J Exp Med. 2020 Jun 1;217(6). pii: e20191804. doi: 10.1084/jem.20191804.

PMID:
32207811
2.

Effects of repleting organic phosphates in banked erythrocytes on plasma metabolites and vasoactive mediators after red cell exchange transfusion in sickle cell disease.

Lopez Domowicz DA, Welsby I, Esther CR Jr, Zhu H, Marek RD, Lee G, Shah N, Poisson JL, McMahon TJ.

Blood Transfus. 2020 Mar 17:1-8. doi: 10.2450/2020.0237-19. [Epub ahead of print]

3.

Variable cellular ivacaftor concentrations in people with cystic fibrosis on modulator therapy.

Guimbellot JS, Ryan KJ, Anderson JD, Liu Z, Kersh L, Esther CR, Rowe SM, Acosta EP.

J Cyst Fibros. 2020 Feb 7. pii: S1569-1993(20)30035-7. doi: 10.1016/j.jcf.2020.01.011. [Epub ahead of print]

PMID:
32044246
4.

Positional impairment of gas exchange during diaphragm pacing alleviated by increasing amplitude settings in congenital central hypoventilation syndrome.

Chada A, Leu RM, Perez IA, Esther CR Jr, Kasi AS.

J Clin Sleep Med. 2020 Mar 15;16(3):459-462. doi: 10.5664/jcsm.8232. Epub 2020 Jan 14.

PMID:
31992416
5.

Challenging scenarios in nontuberculous mycobacterial infection in cystic fibrosis.

Martiniano SL, Esther CR, Haworth CS, Kasperbauer SH, Zemanick ET, Caverly LJ.

Pediatr Pulmonol. 2020 Feb;55(2):521-525. doi: 10.1002/ppul.24604. Epub 2019 Dec 10.

PMID:
31821718
6.

American Thoracic Society 2019 Pediatric Core Curriculum.

Moore PE, Boyer D, Perkins R, Katz ES, Castro-Codesal ML, MacLean JE, Akil N, Esther CR Jr, Kaslow J, Lewis TC, Krone KA, Quizon A, Simpson R, Benscoter D, Spielberg DR, Melicoff E, Kuklinski CA, Blatter JA, Dy J, Rettig JS, Horani A, Gross J.

Pediatr Pulmonol. 2019 Dec;54(12):1880-1894. doi: 10.1002/ppul.24482. Epub 2019 Aug 27. Review.

PMID:
31456278
7.

Identifying Biomarkers in Pediatric Rare Lung Disease. chILD Grows Up.

Vece TJ, Esther CR.

Am J Respir Crit Care Med. 2019 Dec 15;200(12):1458-1459. doi: 10.1164/rccm.201908-1594ED. No abstract available.

8.

Chronic E-Cigarette Use Increases Neutrophil Elastase and Matrix Metalloprotease Levels in the Lung.

Ghosh A, Coakley RD, Ghio AJ, Muhlebach MS, Esther CR Jr, Alexis NE, Tarran R.

Am J Respir Crit Care Med. 2019 Dec 1;200(11):1392-1401. doi: 10.1164/rccm.201903-0615OC.

PMID:
31390877
9.

Highlights from the 2018 North American cystic fibrosis conference.

Martiniano SL, Daines CL, Dellon EP, Esther CR Jr, Muhlebach MS, Ong T, Rabinowitz EC, Toprak D, Zemanick ET.

Pediatr Pulmonol. 2019 Jul;54(7):941-948. doi: 10.1002/ppul.24356. Epub 2019 May 15.

PMID:
31091021
10.

Use of inhaled imipenem/cilastatin in pediatric patients with cystic fibrosis: A case series.

Jones LA, Doucette L, Dellon EP, Esther CR, McKinzie CJ.

J Cyst Fibros. 2019 Jul;18(4):e42-e44. doi: 10.1016/j.jcf.2019.04.017. Epub 2019 May 3.

PMID:
31060800
11.

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis.

Esther CR Jr, Muhlebach MS, Ehre C, Hill DB, Wolfgang MC, Kesimer M, Ramsey KA, Markovetz MR, Garbarine IC, Forest MG, Seim I, Zorn B, Morrison CB, Delion MF, Thelin WR, Villalon D, Sabater JR, Turkovic L, Ranganathan S, Stick SM, Boucher RC.

Sci Transl Med. 2019 Apr 3;11(486). pii: eaav3488. doi: 10.1126/scitranslmed.aav3488.

12.

Persistent Wheeze in Infants: A Guide for General Pediatricians.

Yu Y, Esther CR Jr, Ren CL, Loughlin C.

Pediatr Ann. 2019 Mar 1;48(3):e110-e114. doi: 10.3928/19382359-20190222-02.

PMID:
30874818
13.

An Improved Inhaled Mucolytic to Treat Airway Muco-obstructive Diseases.

Ehre C, Rushton ZL, Wang B, Hothem LN, Morrison CB, Fontana NC, Markovetz MR, Delion MF, Kato T, Villalon D, Thelin WR, Esther CR Jr, Hill DB, Grubb BR, Livraghi-Butrico A, Donaldson SH, Boucher RC.

Am J Respir Crit Care Med. 2019 Jan 15;199(2):171-180. doi: 10.1164/rccm.201802-0245OC.

14.

Use of telavancin in adolescent patients with cystic fibrosis and prior intolerance to vancomycin: A case series.

Bernstein AT, Leigh MW, Goralski JL, Esther CR Jr, McKinzie CJ.

J Cyst Fibros. 2018 Nov;17(6):e48-e50. doi: 10.1016/j.jcf.2018.08.003. Epub 2018 Aug 28.

PMID:
30170755
15.

Iron concentration in exhaled breath condensate decreases in ever-smokers and COPD patients.

Ghio AJ, Soukup JM, McGee J, Madden MC, Esther CR.

J Breath Res. 2018 Aug 21;12(4):046009. doi: 10.1088/1752-7163/aad825.

PMID:
30079894
16.

Tracheostomy in children: Epidemiology and clinical outcomes.

Sanders CD, Guimbellot JS, Muhlebach MS, Lin FC, Gilligan P, Esther CR Jr.

Pediatr Pulmonol. 2018 Sep;53(9):1269-1275. doi: 10.1002/ppul.24071. Epub 2018 Jul 3.

PMID:
29968973
17.

Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding.

Trimble A, McKinzie C, Terrell M, Stringer E, Esther CR Jr.

J Cyst Fibros. 2018 Nov;17(6):779-782. doi: 10.1016/j.jcf.2018.05.009. Epub 2018 Jun 1.

18.

Highlights from the 2017 North American Cystic Fibrosis Conference.

Martiniano SL, Toprak D, Ong T, Zemanick ET, Daines CL, Muhlebach MS, Esther CR Jr, Dellon EP.

Pediatr Pulmonol. 2018 Jul;53(7):979-986. doi: 10.1002/ppul.24000. Epub 2018 Apr 16. Review.

PMID:
29660839
19.

Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children.

Muhlebach MS, Zorn BT, Esther CR, Hatch JE, Murray CP, Turkovic L, Ranganathan SC, Boucher RC, Stick SM, Wolfgang MC.

PLoS Pathog. 2018 Jan 18;14(1):e1006798. doi: 10.1371/journal.ppat.1006798. eCollection 2018 Jan.

20.

Transition and post-transition metals in exhaled breath condensate.

Ghio AJ, Madden MC, Esther CR.

J Breath Res. 2018 Feb 7;12(2):027112. doi: 10.1088/1752-7163/aaa214. Review.

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