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Notch signaling inactivation by small molecule γ-secretase inhibitors restores the multiciliated cell population in the airway epithelium.
Vladar EK, Kunimoto K, Rojas-Hernandez LS, Spano JM, Sellers ZM, Joo NS, Cooney RA, Axelrod JD, Milla CE. Vladar EK, et al. Among authors: milla ce. Am J Physiol Lung Cell Mol Physiol. 2023 Jun 1;324(6):L771-L782. doi: 10.1152/ajplung.00382.2022. Epub 2023 Apr 11. Am J Physiol Lung Cell Mol Physiol. 2023. PMID: 37039381 Free PMC article.
In summary, we demonstrate that GSI administration is a promising therapeutic to restore multiciliated cells and potentially improve epithelial function in a wide range of chronic lung diseases.NEW & NOTEWORTHY Our findings show that low-dose, short-term topical or sys …
In summary, we demonstrate that GSI administration is a promising therapeutic to restore multiciliated cells and potentially improve epithel …
Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review.
Shapiro AJ, Zariwala MA, Ferkol T, Davis SD, Sagel SD, Dell SD, Rosenfeld M, Olivier KN, Milla C, Daniel SJ, Kimple AJ, Manion M, Knowles MR, Leigh MW; Genetic Disorders of Mucociliary Clearance Consortium. Shapiro AJ, et al. Among authors: milla c. Pediatr Pulmonol. 2016 Feb;51(2):115-32. doi: 10.1002/ppul.23304. Epub 2015 Sep 29. Pediatr Pulmonol. 2016. PMID: 26418604 Free PMC article. Review.
These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long-term therapeutics in PCD patients....
These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagno …
Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study.
Chilvers MA, Davies JC, Milla C, Tian S, Han Z, Cornell AG, Owen CA, Ratjen F. Chilvers MA, et al. Among authors: milla c. Lancet Respir Med. 2021 Jul;9(7):721-732. doi: 10.1016/S2213-2600(20)30517-8. Epub 2021 Jan 28. Lancet Respir Med. 2021. PMID: 33516285 Clinical Trial.
BACKGROUND: The safety and efficacy of 24 weeks of lumacaftor-ivacaftor combination therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation was previously shown in two phase 3 studies. Here, we report long-term safety and efficacy …
BACKGROUND: The safety and efficacy of 24 weeks of lumacaftor-ivacaftor combination therapy in children aged 6-11 years with cystic fibrosis …
Association of nutritional status and pulmonary function in children with cystic fibrosis.
Milla CE. Milla CE. Curr Opin Pulm Med. 2004 Nov;10(6):505-9. doi: 10.1097/01.mcp.0000138995.08494.69. Curr Opin Pulm Med. 2004. PMID: 15510058 Review.
Therefore, aggressive nutritional support aiming at achieving normal growth patterns should lead to adequate development of lung function and maintenance of pulmonary health. However, more research is required with long-term longitudinal studies to better identify the most …
Therefore, aggressive nutritional support aiming at achieving normal growth patterns should lead to adequate development of lung function an …
Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
Flume PA, Biner RF, Downey DG, Brown C, Jain M, Fischer R, De Boeck K, Sawicki GS, Chang P, Paz-Diaz H, Rubin JL, Yang Y, Hu X, Pasta DJ, Millar SJ, Campbell D, Wang X, Ahluwalia N, Owen CA, Wainwright CE; VX14-661-110 study group. Flume PA, et al. Lancet Respir Med. 2021 Jul;9(7):733-746. doi: 10.1016/S2213-2600(20)30510-5. Epub 2021 Feb 10. Lancet Respir Med. 2021. PMID: 33581080
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability, and efficacy of tezacaftor-ivacaftor in participants aged 12 years or older with cystic fibrosis who were homozygous or heterozygous for t …
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability …
Lung clearance index is sensitive to small airway disease in pediatric lung transplant recipients.
Kao JE, Zirbes JM, Conrad CK, Milla CE. Kao JE, et al. Among authors: milla ce. J Heart Lung Transplant. 2017 Sep;36(9):980-984. doi: 10.1016/j.healun.2017.05.004. Epub 2017 May 10. J Heart Lung Transplant. 2017. PMID: 28651906
BACKGROUND: The principal obstacle to long-term survival after lung transplant is chronic lung allograft dysfunction (CLAD), which primarily affects the small airways. ...
BACKGROUND: The principal obstacle to long-term survival after lung transplant is chronic lung allograft dysfunction (CLAD), which pr …
Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis.
Ren CL, Borowitz DS, Gonska T, Howenstine MS, Levy H, Massie J, Milla C, Munck A, Southern KW. Ren CL, et al. Among authors: milla c. J Pediatr. 2017 Feb;181S:S45-S51.e1. doi: 10.1016/j.jpeds.2016.09.066. J Pediatr. 2017. PMID: 28129812
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive for Pseudomonas aeruginosa. Most studies have only reported short-term outcomes in the first 1-3 years of life; the long-term …
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive …
Comparison of settings used for high-frequency chest-wall compression in cystic fibrosis.
Kempainen RR, Milla C, Dunitz J, Savik K, Hazelwood A, Williams C, Rubin BK, Billings JL. Kempainen RR, et al. Among authors: milla c. Respir Care. 2010 Jun;55(6):695-701. Respir Care. 2010. PMID: 20507651 Free article. Clinical Trial.
CONCLUSIONS: In adult CF patients, single-session higher-pressure/variable-frequency HFCWC resulted in greater sputum expectoration by wet weight, but not other differences, compared to the commonly used lower-pressure/mid-frequency settings. Longer-term comparisons are ne …
CONCLUSIONS: In adult CF patients, single-session higher-pressure/variable-frequency HFCWC resulted in greater sputum expectoration by wet w …
Long-term effects of aerosolised rhDNase on pulmonary disease progression in patients with cystic fibrosis.
Milla CE. Milla CE. Thorax. 1998 Dec;53(12):1014-7. doi: 10.1136/thx.53.12.1014. Thorax. 1998. PMID: 10195070 Free PMC article.
BACKGROUND: After multiple studies, including clinical trials, suggested some mild clinical benefits from the use of rhDNase by patients with cystic fibrosis, a widespread acceptance of the drug has followed. However, long-term effects, specifically on lung disease progres …
BACKGROUND: After multiple studies, including clinical trials, suggested some mild clinical benefits from the use of rhDNase by patients wit …
12 results