NEUMOLOGÍA PEDIÁTRICA

Revista Neumología Pediátrica | Contenido disponible en www.neumologia-pediatrica.cl 42 Neumol Pediatr 2023; 18 (2): 40 - 42 Actualización en Fibrosis Quística: Diabetes relacionada a fibrosis quística en la era de los moduladores del CFTR 1. Prentice B., Nicholson M., Lam GY. Cystic Fibrosis Related Diabetes in the Era of Modulators: A Scoping Review. PaediatricRespiratoryReviews 2022; 22: 526-542DOI: https:// doi.org/10.1016/j.prrv.2022.11.005. 2. Moran A., Dunitz J., Nathan B., Saeed A., Holme B., Thomas W. Cystic Fibrosis related Diabetes: current trends in prevalence, incidence, and mortality. Diabetes care 2009;32:1626-31. 3. Norris A.W., Larson K., Merjaneh L., Sanda S., Yi Y., Sun X. et al. Survival in a bad neighborhood: pancreatic islets in Cystic Fibrosis. J.Endocrinol JOE-18-0468.RI (2019) doi:10.1530/ JOE-18-0468. 4. Khan D., Kelsey R., Maheshwari R., Stone V., Hasib A., Manderson F. et al. Short termCFTR inhibition reduces islet area in C57BL/6mice. Sci Rep 2019 (9): 11244-54. 5. Bellin M.D., Laguna T., Leschyshyn J., Regelman W., Dunitz J., Moran A. Insulin secretion improves in cystic fibrosis following Ivacaftor correction of CFTR: a small pilot study. Pediatr Diabetes 2013;14: 417-421. 6. Tsabari R., Elyashar H., Cohen-Cymberknoh M.,Breuer O., Armoni S., Livnat G., et al. CFTR potentiator therapy ameliorates impaired insulin secretion in CF patients with a gating mutation. J Cyst Fibrosis 2016;15: e25-27. 7. Dagan A., Cohen-Cymberknoh M., Steinberg M., Levine H., Vilozni D., Betzalel Y., el al. Ivacaftor for the p.Ser549Arg (S549R) gatingmutation.The Israeli experience. RespirMed 2017;131:225-228. 8. Thomassen J.C., Mueller M.I., Alcazar M.A., Rietschel E., Van Koningsbruggen-Rietschel S.. Effect of Lumacaftor/Ivacaftor on glucose metabolism and Insulin secretion in Phe508del homozygous Cystic Fibrosis patients. J Cyst Fibros 2018;17:271-275. 9. Korten I., Kieninger E., Krueger L., Bullo M., Flük C., Latzin P., et al. Short term effects of Elexacaftor/Tezacaftor/Ivacaftor combination on Glucose tolerance in young people with Cystic Fibrosis. An observational pilot study. Front Pediatr 2022; 10: 852551. 10. Petersen M.C., Begnel L., Wallendorf M., Litvin M. Effect of Elexacaftor/Texacaftor/Ivacaftor on body weight and metabolic parameters in adult Cystic Fibrosis. J Cyst Fibros 2022;21: 265-271. 11. Bessonova L., Volkova N., Higgins M., Bengtsson L., Tian S., Simard C., et al. Data from the US and UK Cystic Fibrosis registries support disease modification by CFTR modulation with Ivacaftor. Thorax 2018;73: 731-740. 12. Volkova N., Moy K., Evans J., Campbell D., Tian S., Simard C.,et al. Disease progression in patients with Cystic Fibrosis treated with Ivacaftor. Data from national US and UK registries. J Cyst Fibros 2020;19: 68-79. 13. Missgault B., Chatron E., Reynaud Q., Touzet S., Abely M., Melly M., et al. Effect of one year Lumacaftor/Ivacaftor treatment on glucose tolerance abnormalities in Cystic Fibrosis patients J Cyst Fibros 2020;19: 712-716. 14. Moheet A., Beisang D., Zhang L., Sagel S., Van Dalfsen J., Heltshe S., et al. Lumacaftor/Ivacaftor therapy fails to increase Insulin secretion in F508del/F508del Cystic Fibrosis patients. J Cyst Fibros 2021;20: 333-338. 15. Gaines H., Jones K., Lim J., Medhi N., Chen S., Scofield S. Effect of CFTRmodulator therapy on Cystic Fibrosis Related Diabetes. J Diabetes Complications 2021;35:107845. 16. Scully K., Marchetti P., Sawicki G., Uluer A., Cernadas M., Cagnina R., et al. The effect of Elexacaftor/Tezacaftor/Ivacaftor onglycemia in adultswithCystic Fibrosis. J Cyst Fibros 2022;21: 258-263. 17. Szenpetery S., Foil K., Hendrix S., Gray S., Mingora C., Head B., et al. A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus. J Cyst Fibros 2022;21:721-724. 18. Fortner C. N., Seguin J.M., Kay D.M. Normal pancreatic function and false negative CF newborn screen in a child born to amother taking CFTRmodulator therapy during pregnancy. J Cyst Fibros 2021;20: 835-836. REFERENCIAS desarrollo de disglicemias y DRFQ. El uso precoz y continuo de moduladores del CFTR podría prevenir estas complicaciones, aunque faltan más estudios enfocados en el momento óptimo de intervención en estos pacientes. El autor declara no tener conflictos de interés.

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