Tratamiento de reposición enzimática en la insuficiencia pancreática exocrina asociada a la fibrosis quística. Grupo de trabajo de Fibrosis Quística de la Sociedad Latinoamericana de Gastroenterología, Hepatología y Nutrición Pediátrica (LASPHGAN)
Keywords:
fibrosis quística, insuficiencia pancreática exocrina, malabsorción, reposición enzimáticaAbstract
La fibrosis quística (FQ) es un trastorno hereditario, de las glándulas de secreción exocrina, considerada la causa más frecuente de insuficiencia pancreática exocrina (IPE) en la infancia. Esta revisión resume el panorama del tratamiento de reposición enzimática en la IPE asociada a la FQ, las formulaciones disponibles, su dosificación y modo de administración, así como las limitaciones y desafíos actuales y las posibles áreas del desarrollo futuro
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Contreras Bolivar V, Olveira Fuster C, Blasco Alonso J, et. al. Actualización en nutrición en la fibrosis quística. Nutr Clin Med 2019; XIII (1): 19-44. DOI: 10.7400/NCM.2019.13.1.5071
Calvo-Lerma J, Hulst J, Boon M, Colombo C, et al. Clinical validation of an evidence-based method to adjust Pancreatic Enzyme Replacement Therapy through a prospective interventional study in paediatric patients with Cystic Fibrosis. PLoS ONE 2019; 14(3):1-16 e0213216. DOI: 10.1371/journal.pone.0213216
Altman K, McDonald C, Michel S, Maguiness K. Nutrition in cystic fibrosis: From the past to the present and into the future. Pediatric Pulmonology 2019; 54: S56–S73.
Singh VK, Schwarzenberg SJ. Pancreatic insufficiency in Cystic Fibrosis. J Cyst Fibros 2017;16: S70 -S78.
Can Gan, Yan-Hua Chen, Ling Liu, et. al. Efficacy and safety of pancreatic enzyme replacement therapy on exocrine pancreatic insufficiency: a meta-analysis. Oncotarget 2017; 8(55): 94920-9493.
Tabori H, Arnold C, Jaudszius A, et al. Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings. PloS One 2017; 12(5): e0174463.
Capurso G, Traini M, Piciucchi M, et al. Exocrine pancreatic insufficiency: prevalence, diagnosis, and management. Clin Exp Gastroenterol 2019; 12: 129–139.
Castellani C, Duff AJA, Bell SC, et al. ECFS best practice guidelines: the 2018 revision. J Cyst Fibros 2018;17(2):153-78.
Stalling A, Tindal A, Mascarenha M, et al. Improved residual fat malabsorption and growth in children with cystic fibrosis treated with a novel oral structured lipid supplement: A randomized controlled trial. PLoS ONE 2020; 15(5):1-13 e0232685. https://doi.org/10.1371/journal.pone.0232685
Heubi JE, Schaeffer D, Ahrens RC, et al. Safety and efficacy of a novel microbial lipase in patients with exocrine pancreatic insufficiency due to cystic fibrosis: a randomized controlled clinical trial. J Pediatr 2016 176: 156-161.e1.
Turck D, Braegger CP, Colombo C, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr 2016; 35(3): 557–77. pmid:27068495
Food and Drug Administration. Guidance for Industry: Exocrine Pancreatic Insufficiency Drug Products—Submitting New Drug Applications; Withdrawal of Guidance. Federal Register 2020/ Vol. 85, No. 44.
Somaraju URR, Solis Moya A. Pancreatic enzyme replacement therapy for people with cystic fibrosis. Cochrane Database of Syst Rev 2020, 8(8): CD008227. DOI: 10.1002/14651858.CD008227.pub4.
Kuhn R, Gelrud A, Munck A, Caras S. CREON (Pancrelipase Delayed-Release Capsules) for the Treatment of Exocrine Pancreatic Insufficiency. Adv Ther 2010; 27(12): 2-22. DOI 10.1007/s12325-010-0085-7.
Konstan MW, Accurso FJ, Nasr SZ, et al. Efficacy and safety of a unique enteric-coated bicarbonate-buffered pancreatic enzyme replacement therapy in children and adults with cystic fibrosis. Clin Investig (Lond). 2013; 3(8): 723–729.
Assis DN, Freedman SD. Gastrointestinal Disorders in Cystic Fibrosis Clin Chest Med 2016; 37:109–118.
Freedman S, Orenstein D, Black P, et al. Increased fat absorption from enteral formula through an in-line digestive cartridge in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2017;65: 97–101.
Stevens J, Wyatt C, Brown P, et. al. Absorption and safety with sustained use of RELIZORB. Evaluation (ASSURE) Study in Patients With Cystic Fibrosis Receiving Enteral Feeding. J Pediatr Gastroenterol Nutr 2018; 67: 527–532.
Konstan M, Wagener J, WilschanskiM, et al. A comparison of liprotamase, a non-porcine pancreatic enzyme replacement therapy, to porcine extracted pancrelipase in a noninferiority randomized clinical trial in patients with cystic fibrosis. Clin Invest(Lond) 2018; 8(4): 14-154.
Lahiri T, Hempstead SE, Brady C, et. al. Clinical Practice Guidelines from the Cystic Fibrosis Foundation for Preschoolers with Cystic Fibrosis. Pediatrics 2016; 1(37): 1-26.
Gelfond D, Ma C, Semler J, et. al. Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule. Dig Dis Sci 2013; 58(8): 2275 -81.
Borowitz D. CFTR, bicarbonate, and the pathophysiology of cystic fibrosis. Pediatr Pulmonol 2015; 50(Suppl. 40): S24 -30.
Schwarzenberg SJ, Hempstead SE, McDonald CM, et. al. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. Journal of Cystic Fibrosis 2016; 15: 724 -735.
Highlights of prescribing information for PERTZYE. ReferenceID. 4578388. https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/022175s006lbl.
Shlieout G, Koerner A, Maffert M, et al. Administration of CREON pancrelipase pellets via gastrostomy tube is feasible with no loss of gastric resistance or lipase activity. An in vitro study. Clin Drug Investig 2011; 31 (7): e1 - e7.
Boullata JI, Clarke JL, Stone A, et. al. Optimizing clinical and cost outcomes for patients on enteral nutrition support for treatment of exocrine pancreatic insufficiency: proceedings from an expert advisory board meeting. Popul Health Manag 2019; 22(S1): S1 -S10.
Ratchford TL, Teckman JH, Patel DR. Gastrointestinal pathophysiology and nutrition in cystic fibrosis, Expert Rev Gastroenterol Hepatol 2018; 12(9): 853 – 862. DOI: 10.1080/17474124.2018.1502663
Declercq D, Meerhaeghe SV, Marchand S, et al: The nutritional status in CF: being certain about the uncertainties. Clinical Nutrition ESPEN 29. 2019; 29: 15-21.
Ooi CY, Durie PR. Cystic Fibrosis from the Gastroenteroloist’s perspective. Nat Rev Gastroenterol Hepatol 2016; 13(3): 175 – 85.
Wilschanski M, Braegger C, Colombo C, et al. Highlights of the ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants and children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2016; 63(6): 671-675.
Kalnins D, Wilschanski M. Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies. Drug Des Devel Ther 2012; 6:151-61.
Nakajima K, Oshida H, Muneyuki T, et al. Pancrealipase: an evidence-based review of its use for treating pancreatic exocrine insufficiency. Core Evid 2012; 7: 77-91.
Crowley J, Croining K, Mullane D, et al. Restoration of exocrine pancreatic function in child with lumacaftor/ivacaftor therapy in cystic fibrosis. J Cyst Fibros 2022; 21(2): 264. DOI:10.1016/j.jcf.2021.08.032.Epub 2021 set 10.