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Lymphangioleiomyomatosis Mortality in Patients with Tuberous Sclerosis Complex.

Zak S, Mokhallati N, Su W, McCormack FX, Franz DN, Mays M, Krueger DA, Szczesniak RD, Gupta N.

Ann Am Thorac Soc. 2018 Dec 20. doi: 10.1513/AnnalsATS.201807-471RL. [Epub ahead of print] No abstract available.


Moving beyond panaceas in fisheries governance.

Young OR, Webster DG, Cox ME, Raakjær J, Blaxekjær LØ, Einarsson N, Virginia RA, Acheson J, Bromley D, Cardwell E, Carothers C, Eythórsson E, Howarth RB, Jentoft S, McCay BJ, McCormack F, Osherenko G, Pinkerton E, van Ginkel R, Wilson JA, Rivers L 3rd, Wilson RS.

Proc Natl Acad Sci U S A. 2018 Sep 11;115(37):9065-9073. doi: 10.1073/pnas.1716545115. Epub 2018 Aug 23.


The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis.

Miller S, Coveney C, Johnson J, Farmaki AE, Gupta N, Tobin MD, Wain LV, McCormack FX, Boocock DJ, Johnson SR.

Eur Respir J. 2018 Nov 1;52(5). pii: 1800951. doi: 10.1183/13993003.00951-2018. Print 2018 Nov.


Smoking-Related Diffuse Cystic Lung Disease.

Gupta N, Colby TV, Meyer CA, McCormack FX, Wikenheiser-Brokamp KA.

Chest. 2018 Aug;154(2):e31-e35. doi: 10.1016/j.chest.2018.02.027.


The NHLBI LAM Registry: Prognostic Physiologic and Radiologic Biomarkers Emerge From a 15-Year Prospective Longitudinal Analysis.

Gupta N, Lee HS, Ryu JH, Taveira-DaSilva AM, Beck GJ, Lee JC, McCarthy K, Finlay GA, Brown KK, Ruoss SJ, Avila NA, Moss J, McCormack FX; NHLBI LAM Registry Group.

Chest. 2019 Feb;155(2):288-296. doi: 10.1016/j.chest.2018.06.016. Epub 2018 Jun 22.


NKG2D Regulation of Lung Pathology and Dendritic Cell Function Following Respiratory Syncytial Virus Infection.

Liu H, Osterburg AR, Flury J, Huang S, McCormack FX, Cormier SA, Borchers MT.

J Infect Dis. 2018 Oct 20;218(11):1822-1832. doi: 10.1093/infdis/jiy151.


Reply to Yanagisawa: Treatment of Pulmonary Lymphangioleiomyomatosis during Pregnancy.

Gupta N, Johnson SR, Moss J, McCormack FX.

Am J Respir Crit Care Med. 2018 Jun 1;197(11):1507-1508. doi: 10.1164/rccm.201801-0043LE. No abstract available.


Epidemiology of Rare Lung Diseases: The Challenges and Opportunities to Improve Research and Knowledge.

McCarthy C, Lara Gallego B, Trapnell BC, McCormack FX.

Adv Exp Med Biol. 2017;1031:419-442. doi: 10.1007/978-3-319-67144-4_24. Review.


Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline.

Gupta N, Finlay GA, Kotloff RM, Strange C, Wilson KC, Young LR, Taveira-DaSilva AM, Johnson SR, Cottin V, Sahn SA, Ryu JH, Seyama K, Inoue Y, Downey GP, Han MK, Colby TV, Wikenheiser-Brokamp KA, Meyer CA, Smith K, Moss J, McCormack FX; ATS Assembly on Clinical Problems.

Am J Respir Crit Care Med. 2017 Nov 15;196(10):1337-1348. doi: 10.1164/rccm.201709-1965ST.


Unsupervised gene expression analyses identify IPF-severity correlated signatures, associated genes and biomarkers.

Wang Y, Yella J, Chen J, McCormack FX, Madala SK, Jegga AG.

BMC Pulm Med. 2017 Oct 20;17(1):133. doi: 10.1186/s12890-017-0472-9.


Thermal injury of the skin induces G-CSF-dependent attenuation of EPO-mediated STAT signaling and erythroid differentiation arrest in mice.

Noel JG, Ramser BJ, Cancelas JA, McCormack FX, Gardner JC.

Exp Hematol. 2017 Dec;56:16-30. doi: 10.1016/j.exphem.2017.08.005. Epub 2017 Sep 1.


An Official American Thoracic Society Workshop Report: Translational Research in Rare Respiratory Diseases.

Kristof AS, Petrof BJ, Hamid Q, Kolb M, Landry JS, MacKenzie A, McCormack FX, Murawski IJ, Moss J, Rauch F, Rosas IO, Shapiro AJ, Smith BM, Thomas DY, Trapnell BC, Young LR, Zariwala MA; ATS Assembly on Respiratory Cell and Molecular Biology.

Ann Am Thorac Soc. 2017 Aug;14(8):1239-1247. doi: 10.1513/AnnalsATS.201705-406WS.


Mitogenic stimulation accelerates influenza-induced mortality by increasing susceptibility of alveolar type II cells to infection.

Nikolaidis NM, Noel JG, Pitstick LB, Gardner JC, Uehara Y, Wu H, Saito A, Lewnard KE, Liu H, White MR, Hartshorn KL, McCormack FX.

Proc Natl Acad Sci U S A. 2017 Aug 8;114(32):E6613-E6622. doi: 10.1073/pnas.1621172114. Epub 2017 Jul 24.


Differential Ligand Binding Specificities of the Pulmonary Collectins Are Determined by the Conformational Freedom of a Surface Loop.

Rynkiewicz MJ, Wu H, Cafarella TR, Nikolaidis NM, Head JF, Seaton BA, McCormack FX.

Biochemistry. 2017 Aug 8;56(31):4095-4105. doi: 10.1021/acs.biochem.6b01313. Epub 2017 Jul 31.


Summary for Clinicians: Lymphangioleiomyomatosis Diagnosis and Management Clinical Practice Guideline.

Feemster LC, Lyons PG, Chatterjee RS, Kidambi P, McCormack FX, Moss J, Wilson KC, Thomson CC.

Ann Am Thorac Soc. 2017 Jul;14(7):1073-1075. doi: 10.1513/AnnalsATS.201609-685CME. No abstract available.


Apparent Sporadic Lymphangioleiomyomatosis in a Man as a Result of Extreme Mosaicism for a TSC2 Mutation.

Han MK, Tyburczy ME, Darling TN, Kazerooni EA, Myers JL, McCormack FX, Moss J, Kwiatkowski DJ.

Ann Am Thorac Soc. 2017 Jul;14(7):1227-1229. doi: 10.1513/AnnalsATS.201703-229LE. No abstract available.


Evidence of bias in assessment of fisheries management impacts.

Slooten E, Simmons G, Dawson SM, Bremner G, Thrush SF, Whittaker H, McCormack F, Robertson BC, Haworth N, Clarke PJ, Pauly D, Zeller D.

Proc Natl Acad Sci U S A. 2017 Jun 20;114(25):E4901-E4902. doi: 10.1073/pnas.1706544114. Epub 2017 Jun 12. No abstract available.


Is overcrowding a barrier to hand hygiene and how can it be addressed?

Poovelikunnel TT, Duffy F, Finn C, McCormack F, Walsh J, Humphreys H.

J Hosp Infect. 2017 Sep;97(1):30-31. doi: 10.1016/j.jhin.2017.05.026. Epub 2017 Jun 6. No abstract available.


Dendritic Cell Trafficking and Function in Rare Lung Diseases.

Liu H, Jakubzick C, Osterburg AR, Nelson RL, Gupta N, McCormack FX, Borchers MT.

Am J Respir Cell Mol Biol. 2017 Oct;57(4):393-402. doi: 10.1165/rcmb.2017-0051PS. Review.


A Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis.

Lu C, Lee HS, Pappas GP, Dilling DF, Burger CD, Shifren A, Veeraraghavan S, Chapman JT, Parambil J, Ruoss SJ, Young LR, Hammes SR, Kopras EJ, Roads T, Krischer JP, McCormack FX; Trial of an Aromatase Inhibitor in Lymphangioleiomyomatosis Group.

Ann Am Thorac Soc. 2017 Jun;14(6):919-928. doi: 10.1513/AnnalsATS.201610-824OC.


Alcohol and healthy ageing: a challenge for alcohol policy.

Nicholson D, McCormack F, Seaman P, Bell K, Duffy T, Gilhooly M.

Public Health. 2017 Jul;148:13-18. doi: 10.1016/j.puhe.2017.02.021. Epub 2017 Apr 3.


Reply: The ATS/JRS Guidelines on Lymphangioleiomyomatosis: Filling in the Gaps.

McCormack FX, Gupta N, Finlay GR, Young LR, Johnson SR, Cottin V, Wilson KC, Moss J; ATS/JRS Committee on Lymphangioleiomyomatosis.

Am J Respir Crit Care Med. 2017 Sep 1;196(5):660-661. doi: 10.1164/rccm.201702-0417LE. No abstract available.


Spontaneous Pneumothoraces in Patients with Birt-Hogg-Dubé Syndrome.

Gupta N, Kopras EJ, Henske EP, James LE, El-Chemaly S, Veeraraghavan S, Drake MG, McCormack FX.

Ann Am Thorac Soc. 2017 May;14(5):706-713. doi: 10.1513/AnnalsATS.201611-886OC.


Hsp90 regulation of fibroblast activation in pulmonary fibrosis.

Sontake V, Wang Y, Kasam RK, Sinner D, Reddy GB, Naren AP, McCormack FX, White ES, Jegga AG, Madala SK.

JCI Insight. 2017 Feb 23;2(4):e91454. doi: 10.1172/jci.insight.91454.


Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes.

Lee LH, Gasilina A, Roychoudhury J, Clark J, McCormack FX, Pressey J, Grimley MS, Lorsbach R, Ali S, Bailey M, Stephens P, Ross JS, Miller VA, Nassar NN, Kumar AR.

JCI Insight. 2017 Feb 9;2(3):e89473. doi: 10.1172/jci.insight.89473.


Palliative care in general practice: GP integration in caring for patients with advanced cancer.

Le B, Eastman P, Vij S, McCormack F, Duong C, Philip J.

Aust Fam Physician. 2017 Jan/Feb;46(1):51-55.


Lymphangioleiomyomatosis: A Monogenic Model of Malignancy.

Krymskaya VP, McCormack FX.

Annu Rev Med. 2017 Jan 14;68:69-83. doi: 10.1146/annurev-med-050715-104245. Review.


Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective.

Gupta N, Langenderfer D, McCormack FX, Schauer DP, Eckman MH.

Ann Am Thorac Soc. 2017 Jan;14(1):17-25. doi: 10.1513/AnnalsATS.201606-459OC.


NK cell activating receptor ligand expression in lymphangioleiomyomatosis is associated with lung function decline.

Osterburg AR, Nelson RL, Yaniv BZ, Foot R, Donica WR, Nashu MA, Liu H, Wikenheiser-Brokamp KA, Moss J, Gupta N, McCormack FX, Borchers MT.

JCI Insight. 2016 Oct 6;1(16):e87270.


Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.

McCormack FX, Gupta N, Finlay GR, Young LR, Taveira-DaSilva AM, Glasgow CG, Steagall WK, Johnson SR, Sahn SA, Ryu JH, Strange C, Seyama K, Sullivan EJ, Kotloff RM, Downey GP, Chapman JT, Han MK, D'Armiento JM, Inoue Y, Henske EP, Bissler JJ, Colby TV, Kinder BW, Wikenheiser-Brokamp KA, Brown KK, Cordier JF, Meyer C, Cottin V, Brozek JL, Smith K, Wilson KC, Moss J; ATS/JRS Committee on Lymphangioleiomyomatosis.

Am J Respir Crit Care Med. 2016 Sep 15;194(6):748-61. doi: 10.1164/rccm.201607-1384ST.


Rare Lung Diseases: Occasionally the "Horse" Has Stripes.

Kotloff RM, McCormack FX.

Clin Chest Med. 2016 Sep;37(3):xv-xvi. doi: 10.1016/j.ccm.2016.07.001. No abstract available.


Pulmonary Alveolar Microlithiasis.

Saito A, McCormack FX.

Clin Chest Med. 2016 Sep;37(3):441-8. doi: 10.1016/j.ccm.2016.04.007. Epub 2016 Jun 24. Review.


Nonmalignant Adult Thoracic Lymphatic Disorders.

Itkin M, McCormack FX.

Clin Chest Med. 2016 Sep;37(3):409-20. doi: 10.1016/j.ccm.2016.04.004. Review.


Efficacy and Safety of Long-Term Sirolimus Therapy for Asian Patients with Lymphangioleiomyomatosis.

Takada T, Mikami A, Kitamura N, Seyama K, Inoue Y, Nagai K, Suzuki M, Moriyama H, Akasaka K, Tazawa R, Hirai T, Mishima M, Hayashida M, Hirose M, Sugimoto C, Arai T, Hattori N, Watanabe K, Tamada T, Yoshizawa H, Akazawa K, Tanaka T, Yagi K, Young LR, McCormack FX, Nakata K.

Ann Am Thorac Soc. 2016 Nov;13(11):1912-1922.


Prostaglandin-E2 Mediated Increase in Calcium and Phosphate Excretion in a Mouse Model of Distal Nephron Salt Wasting.

Soleimani M, Barone S, Xu J, Alshahrani S, Brooks M, McCormack FX, Smith RD, Zahedi K.

PLoS One. 2016 Jul 21;11(7):e0159804. doi: 10.1371/journal.pone.0159804. eCollection 2016.


Elucidation of Lipid Binding Sites on Lung Surfactant Protein A Using X-ray Crystallography, Mutagenesis, and Molecular Dynamics Simulations.

Goh BC, Wu H, Rynkiewicz MJ, Schulten K, Seaton BA, McCormack FX.

Biochemistry. 2016 Jul 5;55(26):3692-701. doi: 10.1021/acs.biochem.6b00048. Epub 2016 Jun 21.


Keratinocyte growth factor supports pulmonary innate immune defense through maintenance of alveolar antimicrobial protein levels and macrophage function.

Gardner JC, Wu H, Noel JG, Ramser BJ, Pitstick L, Saito A, Nikolaidis NM, McCormack FX.

Am J Physiol Lung Cell Mol Physiol. 2016 May 1;310(9):L868-79. doi: 10.1152/ajplung.00363.2015. Epub 2016 Feb 26.


A Novel Quantitative Computed Tomographic Analysis Suggests How Sirolimus Stabilizes Progressive Air Trapping in Lymphangioleiomyomatosis.

Argula RG, Kokosi M, Lo P, Kim HJ, Ravenel JG, Meyer C, Goldin J, Lee HS, Strange C, McCormack FX; MILES Study Investigators.

Ann Am Thorac Soc. 2016 Mar;13(3):342-9. doi: 10.1513/AnnalsATS.201509-631OC.


Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren Syndrome.

Gupta N, Wikenheiser-Brokamp KA, Fischer A, McCormack FX.

Ann Am Thorac Soc. 2016 Mar;13(3):371-5. doi: 10.1513/AnnalsATS.201511-759BC.


Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment.

Saito A, Nikolaidis NM, Amlal H, Uehara Y, Gardner JC, LaSance K, Pitstick LB, Bridges JP, Wikenheiser-Brokamp KA, McGraw DW, Woods JC, Sabbagh Y, Schiavi SC, Altinişik G, Jakopović M, Inoue Y, McCormack FX.

Sci Transl Med. 2015 Nov 11;7(313):313ra181. doi: 10.1126/scitranslmed.aac8577.



Gupta R, McCormack FX.

Lymphology. 2015 Mar;48(1):52. No abstract available.


Regional Sparing in an Oligemic Lung Segment Supports Hematogenous Spread as a Pathogenic Mechanism in Lymphangioleiomyomatosis.

Gupta N, Han MK, McCormack FX.

Ann Am Thorac Soc. 2015 Aug;12(8):1247-8. doi: 10.1513/AnnalsATS.201503-111LE. No abstract available.


Accuracy of chest high-resolution computed tomography in diagnosing diffuse cystic lung diseases.

Gupta N, Meraj R, Tanase D, James LE, Seyama K, Lynch DA, Akira M, Meyer CA, Ruoss SJ, Burger CD, Young LR, Almoosa KF, Veeraraghavan S, Barker AF, Lee AS, Dilling DF, Inoue Y, Cudzilo CJ, Zafar MA, McCormack FX.

Eur Respir J. 2015 Oct;46(4):1196-9. doi: 10.1183/13993003.00570-2015. Epub 2015 Jul 9. No abstract available.


Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study.

Goldberg HJ, Harari S, Cottin V, Rosas IO, Peters E, Biswal S, Cheng Y, Khindri S, Kovarik JM, Ma S, McCormack FX, Henske EP.

Eur Respir J. 2015 Sep;46(3):783-94. doi: 10.1183/09031936.00210714. Epub 2015 Jun 25.


SP-R210 (Myo18A) Isoforms as Intrinsic Modulators of Macrophage Priming and Activation.

Yang L, Carrillo M, Wu YM, DiAngelo SL, Silveyra P, Umstead TM, Halstead ES, Davies ML, Hu S, Floros J, McCormack FX, Christensen ND, Chroneos ZC.

PLoS One. 2015 May 12;10(5):e0126576. doi: 10.1371/journal.pone.0126576. eCollection 2015.


Diffuse Cystic Lung Disease. Part II.

Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX.

Am J Respir Crit Care Med. 2015 Jul 1;192(1):17-29. doi: 10.1164/rccm.201411-2096CI. Review.


Diffuse Cystic Lung Disease. Part I.

Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX.

Am J Respir Crit Care Med. 2015 Jun 15;191(12):1354-66. doi: 10.1164/rccm.201411-2094CI. Review.


A 25-year-old man with fatigue, dyspnea, and pulmonary cysts.

Mulhall AM, McCormack FX.

Ann Am Thorac Soc. 2015 Jan;12(1):120-3. doi: 10.1513/AnnalsATS.201407-339CC. No abstract available.


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