Función pulmonar en pacientes de 6 a 17 años con fibrosis quística entre los años 2020-2021 en la ciudad de Barranquilla (Atl, Co)

datacite.rightshttp://purl.org/coar/access_right/c_16ec
dc.contributor.advisorStand Niño, Iván Guillermo
dc.contributor.advisorNúñez-Bravo, Narledis
dc.contributor.authorGuerrero Sabagh, Mercy María
dc.contributor.authorSalcedo Doria, Alexander
dc.date.accessioned2023-05-19T20:45:37Z
dc.date.available2023-05-19T20:45:37Z
dc.date.issued2023
dc.description.abstractLa fibrosis quística es una enfermedad de carácter autosómico recesivo. Su incidencia es variable de acuerdo con la población estudiada. En Latinoamérica la incidencia de esta enfermedad es poco conocida y se estima en uno por cada 6000 recién nacidos vivos. Existe un alto índice de subdiagnostico de esta patología en países en vía de desarrollo. La esperanza de vida de estos pacientes mejora conforme ha mejorado el diagnóstico y tratamiento oportuno. Objetivo: Evaluar la función pulmonar en la población de 6-17 años con diagnóstico de Fibrosis Quística en seguimiento entre los años 2020-2021 en la ciudad de Barranquilla (Atl, CO). Materiales y Métodos: Se realizó un estudio de tipo descriptivo transversal, con el fin de caracterizar y evaluar la función pulmonar de los pacientes pediátricos con fibrosis quística en Barranquilla. Se realizó en 19 niños con FQ en seguimiento con neumología pediátrica y cumplieron los criterios de inclusión. Se detalló a través de estadística descriptiva, con medidas de tendencia central y de dispersión, los resultados se mostraron en medidas de porcentaje, media y desviación estándar (SD). Resultados: Se evaluaron variables sociodemográficas de los pacientes como la edad, sexo, IMC, encontrando como grupo etario predominante los pacientes entre 6 y 10 años, además el sexo predominante fue el femenino con un 52.6% de la población. El 52.6% de los pacientes tuvo un peso bajo para la edad. En los aspectos clínicos el mas frecuente fue el hipocratismo digital en un 84% de los casos, el 73.7% tuvo una exacerbación respiratoria, y un porcentaje importante de los pacientes tuvieron infecciones en el ultimo año. Se encontró alteración en la mayoría de las pruebas de función pulmonar.spa
dc.description.abstractCystic fibrosis is an autosomal recessive disease. Its incidence is variable according to the population studied. In Latin America the incidence of this disease is little known and is estimated at per 6000 live newborns. There is a high rate of subdiagnosis of this pathology in developing countries. The life expectancy of these patients improves as timely diagnosis and treatment has improved. Objective: To evaluate lung function in the population aged 6-17 years with a diagnosis of Cystic Fibrosis under follow-up between the years 2020-2021 in the city of Barranquilla (Atl, CO). Materials and methods: A cross-sectional descriptive study was carried out in order to characterize and evaluate the lung function of pediatric patients with cystic fibrosis in Barranquilla. It was carried out in 19 children with CF who were being followed up by pediatric pulmonology and met the inclusion criteria. It was detailed through descriptive statistics, with measures of central tendency and dispersion, the results were shown in measures of percentage, mean and standard deviation (SD). Results: Sociodemographic variables of the patients such as age, sex, and BMI were evaluated, finding patients between 6 and 10 years as the predominant age group, in addition the predominant sex was female with 52.6% of the population. 52.6% of the patients had a low weight for their age. In clinical aspects, the most frequent was digital clubbing in 84% of cases, 73.7% had a respiratory exacerbation, and a significant percentage of patients had infections in the last year. Abnormalities were found in most pulmonary function tests.eng
dc.format.mimetypepdf
dc.identifier.urihttps://hdl.handle.net/20.500.12442/12417
dc.language.isospa
dc.publisherEdiciones Universidad Simón Bolívarspa
dc.publisherFacultad de Ciencias de la Saludspa
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacionaleng
dc.rights.accessrightsinfo:eu-repo/semantics/restrictedAccessspa
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subjectFunción pulmonarspa
dc.subjectFibrosis quísticaspa
dc.subjectNiñosspa
dc.subjectLung functioneng
dc.subjectCystic fibrosiseng
dc.subjectChildreneng
dc.titleFunción pulmonar en pacientes de 6 a 17 años con fibrosis quística entre los años 2020-2021 en la ciudad de Barranquilla (Atl, Co)spa
dc.type.driverinfo:eu-repo/semantics/other
dc.type.spaOtros
dcterms.referencesShteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. Lancet. 2021;397(10290):2195-2211.eng
dcterms.referencesSchrijver I., Pique L., Graham S., Pearl M., Cherry A., Kharrazi M.: The Spectrum of CFTR variants in nonwhite cystic fibrosis patients: implications for molecular diagnostic testing. J Mol Diagn 2016; 18:39-50.eng
dcterms.referencesBurgel P.R., Bellis G., Olesen H.V., Viviani L., Zolin A., Blasi F., et. al.: Future trends in cystic fibrosis demography in 34 European countries. Eur Respir J 2015; 46:133-141.eng
dcterms.referencesKeyeux G, Rodas C, Bienvenu T, Garavito P, Vidaud D, Sanchez D, Kaplan JC, Aristizabal G. Cftr mutations in patients from colombia: Implications for local and regional molecular diagnosis programs. Hum Mutat 2003; 22:259.eng
dcterms.referencesJay LM, Mateus H, Fonseca D, Restrepo CM, Keyeux G. Pcr-heteroduplex by grouping: Rapid screening carrier method for cystic fibrosis f508del mutation in colombia. 2009.eng
dcterms.referencesFajac I., Sermet-Gaudelus I.: Mucoviscidose: nouvelles thérapeutiques ciblant la protéine CFTR. Rev Mal Respir 2013; 30: 255-261.eng
dcterms.referencesSosnay PR, Siklosi KR, Van Goor F, et al. Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet. 2013;45(10):1160-1167.eng
dcterms.referencesNaehrig S, Chao CM, Naehrlich L. Cystic Fibrosis. Dtsch Arztebl Int. 2017;114(33-34):564-574.eng
dcterms.referencesCollaco J.M., Blackman S.M., McGready J., Naughton K.M., Cutting G.R.: Quantification of the relative contribution of environmental and genetic factors to variation in cystic fibrosis lung function. J Pediatric 2010; 157: pp. 802-807.eng
dcterms.referencesElborn J. S. (2016). Cystic fibrosis. The Lancet 388 (10059), 2519–2531. 10.1016/s0140-6736(16)00576-6eng
dcterms.referencesKünzi L, Easter M, Hirsch MJ, Krick S. Cystic Fibrosis Lung Disease in the Aging Population. Front Pharmacol. 2021;15 (12):601438.eng
dcterms.referencesSonneveld N, Stanojevic S, Amin R, Aurora P, Davies J, Elborn JS, Horsley A, Latzin P, O’Neill K, Robinson P, et al. Lung clearance index in cystic fibrosis subjects treated for pulmonary exacerbations. Eur Respir J 2015; 46:1055–1064.eng
dcterms.referencesRanganathan SC, Hall GL, Sly PD, Stick SM, Douglas TA; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST-CF). Early lung disease in infants and pre-school children with cystic fibrosis: what have we learnt and what should we do about it? Am J Respir Crit Care Medeng
dcterms.referencesCystic Fibrosis Foundation. 2018 patient registry annual data report, 2019.eng
dcterms.referencesEarnest, A., Salimi, F., Wainwright, C.E. et al. Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry. Sci Rep 10, 17421. 2020eng
dcterms.referencesVeit G, Avramescu RG, Chiang AN, et. al.: From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol Biol Cell 2016; 27: 424-433.eng
dcterms.referencesVázquez Cordero C, Baranda García F. Diagnóstico. En: Salcedo Posadas A, Gartner S, Girón Moreno RM, García Novo MD, editores. Tratado de Fibrosis Quística. 1ª ed. España: Justim; 2012. 109-122.spa
dcterms.referencesO'Sullivan BP, Freedman SD. Cystic fibrosis. Lancet. 2009;373(9678):1891-1904.eng
dcterms.referencesGuo J, Garratt A, Hill A. Worldwide rates of diagnosis and effective treatment for cystic fibrosis. J Cyst Fibros. 2022;21(3):456-462.eng
dcterms.referencesBoyle MP, De Boeck K. A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect [published correction appears in Lancet Respir Med. 2013;1(2):101].eng
dcterms.referencesAlonso Ramos MJ, Telleria Orriols JJ. Relación fenotipo-genotipo. Genes modificadores. En: Salcedo Posadas A, Gartner S, Girón Moreno RM, García Novo MD, editores. Tratado de Fibrosis Quística. 1ª ed. España: Justim; 2012. p. 63-71.spa
dcterms.referencesRandell SH, Boucher RC: Effective mucus clearance is essential for respiratory health. Am J Respir Cell Mol Biol 2006; 35: 20-28.eng
dcterms.referencesButton B, Cai L-H, Ehre C, et. al.: A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia. Science 2012; 337: 937-941.eng
dcterms.referencesBirket SE, Chu KK, Liu L, et. al.: A functional anatomic defect of the cystic fibrosis airway. Am J Respir Crit Care Med 2014; 190: 421-432.eng
dcterms.referencesGustafsson JK, Ermund A, Ambort D, et. al.: Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype. J Exp Med 2012; 209: 1263-1272.eng
dcterms.referencesPallagi P, Hegyi P, Rakonczay Z: The physiology and pathophysiology of pancreatic ductal secretion: the background for clinicians. Pancreas 2015; 44: 1211-1233.eng
dcterms.referencesWilschanski M, Novak I. The cystic fibrosis of exocrine pancreas. Cold Spring Harb Perspect Med. 2013;3(5): a009746. Published 2013 May 1.eng
dcterms.referencesKhoury O., Barrios C., Ortega V., Atala A., Murphy S.V.: Immunomodulatory cell therapy to target cystic fibrosis inflammation. Am J Respir Cell Mol Biol 2018; 58: 12-20.eng
dcterms.referencesScambler T, Holbrook J, Savic S, McDermott MF, Peckham D. Autoinflammatory disease in the lung [published online ahead of print. Immunology. 2018;154(4):563-573.eng
dcterms.referencesWine JJ, Hansson GC, König P, Joo NS, Ermund A, Pieper M. Progress in understanding mucus abnormalities in cystic fibrosis airways. J Cyst Fibros. 2018;17(2S): S35-S39.eng
dcterms.referencesMall MA, Stahl M, Graeber SY, Sommerburg O, Kauczor HU, Wielpütz MO. Early detection and sensitive monitoring of CF lung disease: Prospects of improved and safer imaging. Pediatr Pulmonol. 2016;51(S44): S49-S60.eng
dcterms.referencesRosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr. 1998;132(4):589-95eng
dcterms.referencesFarrell PM, White TB, Ren CL, et al. Diagnóstico de fibrosis quística: Directrices de consenso de la Fundación de Fibrosis Quística. J Pediatr 2017; 181S:S4.spa
dcterms.referencesNoël S, Sermet-Gaudelus I. Mucoviscidosis: fisiopatología, genética, aspectos clínicos y terapéuticos. EMC Pediatr. 2020;55(1):1-23.spa
dcterms.referencesOoi CY, Dupuis A, Ellis L, Jarvi K, Martin S, Ray PN, Steele L, Kortan P, Gonska T, Dorfman R, Solomon M, Zielenski J, Corey M, Tullis E, Durie P. Does extensive genotyping and nasal potential difference testing clarify the diagnosis of cystic fibrosis among patients with single-organ manifestations of cystic fibrosis? Thorax. 2014;69(3):254-60.eng
dcterms.referencesQuinton P, Molyneux L, Ip W, Dupuis A, Avolio J, Tullis E, Conrad D, Shamsuddin AK, Durie P, Gonska T. β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis. Am J Respir Crit Care Med. 2012;186(8):732-9.eng
dcterms.referencesChillón M, Casals T, Mercier B, Bassas L, Lissens W, Silber S, Romey MC, Ruiz-Romero J, Verlingue C, Claustres M, et al. Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens. N Engl J Med. 1995 1;332(22):1475-80.eng
dcterms.referencesCastellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F, Sermet-Gaudelus I, Southern KW, Barben J, Flume PA, Hodková P, Kashirskaya N, Kirszenbaum MN, Madge S, Oxley H, Plant B, Schwarzenberg SJ, Smyth AR, Taccetti G, Wagner TOF, Wolfe SP, Drevinek P. ECFS best practice guidelines: the 2018 revision. J Cyst Fibros. 2018;17(2):153-178.eng
dcterms.referencesChen Q, Shen Y, Zheng J. A review of cystic fibrosis: Basic and clinical aspects. Animal Model Exp Med. 2021;4(3):220-232.eng
dcterms.referencesRafeeq MM, Murad HAS. Cystic fibrosis: current therapeutic targets and future approaches. J Transl Med. 2017;15(1):84.eng
dcterms.referencesMogayzel P.J., Naureckas E.T., Robinson K.A., Mueller G., Hadjiliadis D.: Pulmonary clinical practice guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2013; 187: 680-689.eng
dcterms.referencesFrayman K., Armstrong D., Grimwood K., Ranganathan S.: The airway microbiota in early cystic fibrosis lung disease. Pediatr Pulmonol 2017; 52: 1384-1404.eng
dcterms.referencesFielbaum O, MANEJO ACTUAL DE LA FIBROSIS QUÍSTICA, Revista Médica Clínica Las Condes, 2017; 28 (1): 60-71spa
dcterms.referencesRingshausen FC, Hellmuth T, Dittrich AM. Was ist gesichert in der Therapie der Mukoviszidose? [Evidence-based treatment of cystic fibrosis]. Internist (Berl). 2020;61(12):1212-1229.eng
dcterms.referencesSnell G., Reed A., Stern M., Hadjiliadis D.: The evolution of lung transplantation for cystic fibrosis: a 2017 update. J Cyst Fibros 2017; 16: 553-564.eng
dcterms.referencesCastellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F, Sermet-Gaudelus I, Southern KW, Barben J, Flume PA, Hodková P, Kashirskaya N, Kirszenbaum MN, Madge S, Oxley H, Plant B, Schwarzenberg SJ, Smyth AR, Taccetti G, Wagner TOF, Wolfe SP, Drevinek P. ECFS best practice guidelines: the 2018 revision. J Cyst Fibros. 2018;17(2):153-178.eng
dcterms.referencesColombo C, Ellemunter H, Houwen R, Munck A, Taylor C, Wilschanski M; ECFS. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros. 2011;10 Suppl 2: S24-8eng
dcterms.referencesBrown, Sheena D. MSCR; White, Rachel, PA-C; Tobin, Phil DHSc, PA-C. Mantenlos respirando: Fisiopatología, diagnóstico y tratamiento de la fibrosis quística. JAAPA 2017; 30(5): 23-27spa
dcterms.referencesDinwiddie R. Lung function in paediatrics. Lung function testing in children. Allergol Immunopathol (Madr). 2010;38(2): 88 – 91eng
dcterms.referencesP. Nixon, M.L. Joswiak, F.J. Fricker. A six-minute walk test for assessing exercise tolerance in severely ill children. J Pediatr. 1996;129: 362-366eng
dcterms.referencesA.M. Li, J. Yin, C.C. YU, T. Tsang, E. Wong, D. Chan, et al. The six-minute walk test in healthy children: reliability and validity. Eu Respir J. 2013; 25:1057-1060eng
dcterms.referencesAurora RN, Zak RS, Karippot A, Lamm CI, Morgenthaler TI, Auerbach SH, et al. Practice parameters for the respira- tory indications for polysomnography in children. Sleep. 2011; 34:379-88.eng
dcterms.referencesAlonso-Álvarez ML, Canet T, Cubell-Alarco M, Estivill E, Fernández-Julián E, Gozal D, et al. Documento de consenso del síndrome de apneas-hipopneas durante el sueño en niños. Arch Bronconeumol. 2011; 47:2-18.spa
dcterms.referencesLevesque ME, Montesi SB, Sharma A. Diffuse parenchymal abnormalities in acutely dyspneic patients. A pattern-based approach. J Thorac Imaging 2015; 30: 220-232.eng
dcterms.referencesNakatani S. [Echocardiography]. Nihon Rinsho. 2006;64(5):867-73.eng
dcterms.referencesHudaverdi M, Hamilton-Craig C, Platts D, Chan J, Burstow DJ. Echocardiography for the clinician: a practical update. Intern Med J. 2010;40(7):476-85.eng
dcterms.referencesLascano-Vaca Y, Ortiz-Prado E, Gomez-Barreno L, Simbaña-Rivera K, Vasconez E, Lister A, Arteaga-Espinosa ME, Pérez GF. Clinical, genetic and microbiological characterization of pediatric patients with cystic fibrosis in a public Hospital in Ecuador. BMC Pediatr. 2020; 20(1):111.eng
dcterms.referencesCalella P, Valerio G, Brodlie M, Donini LM, Siervo M. Cystic fibrosis, body composition, and health outcomes: a systematic review. Nutrition. 2018;55:131-139.eng
dcterms.referencesKonstan MW, Pasta DJ, VanDevanter DR, Wagener JS, Morgan WJ; Scientific Advisory Group and the Investigators and Coordinators of ESCF. Epidemiologic Study of Cystic Fibrosis: 25 years of observational research. Pediatr Pulmonol. 2021;56(5):823-836.eng
dcterms.referencesLiu K, Xu W, Xiao M, Zhao X, Bian C, Zhang Q, Song J, Chen K, Tian X, Liu Y, Xu KF, Zhang X. Characterization of clinical and genetic spectrum of Chinese patients with cystic fibrosis. Orphanet J Rare Dis. 2020; 15(1):150.eng
dcterms.referencesWalicka-Serzysko K, Postek M, Milczewska J, Sands D. Lung function deterioration in school children with cystic fibrosis. Pediatr Pulmonol. 2020;55(11):3030-3038eng
dcterms.referencesSilva Filho LV, Castaños C, Ruíz HH. Cystic fibrosis in Latin America-Improving the awareness. J Cyst Fibros. 2016;15(6):791-793.eng
dcterms.referencesGale S, Maynor S, Ortega J. Caracterización de los pacientes con Fibrosis Quística diagnosticados por cloruros en Sudor. Acta Pediátrica Hondureña. 2016;6 (2)spa
dcterms.referencesOlivo P, Flores O, Rosero C. Correlación de los valores espirométricos con el puntaje clínico de Shwachman y el puntaje radiológico de Brasfield, en la evaluación a pacientes con diagnóstico de fibrosis quística, atendidos en consulta externa del Hospital “Eugenio Espejo” de Quito, año 2014. Rev Fac Cien Med. 2014;39(2):25–30spa
oaire.versioninfo:eu-repo/semantics/acceptedVersion
sb.programaEspecialización en Pediatríaspa
sb.sedeSede Barranquillaspa

Archivos

Bloque original
Mostrando 1 - 2 de 2
Cargando...
Miniatura
Nombre:
PDF_Resumen.pdf
Tamaño:
241.42 KB
Formato:
Adobe Portable Document Format
No hay miniatura disponible
Nombre:
PDF.pdf
Tamaño:
698.51 KB
Formato:
Adobe Portable Document Format

Colecciones