{"id":10665,"date":"2020-10-07T13:28:09","date_gmt":"2020-10-07T18:28:09","guid":{"rendered":"http:\/\/liceaga.facmed.unam.mx\/revistas\/masalud\/?p=10665"},"modified":"2020-10-07T13:31:33","modified_gmt":"2020-10-07T18:31:33","slug":"fibrosis-quistica","status":"publish","type":"post","link":"https:\/\/massalud.facmed.unam.mx\/index.php\/fibrosis-quistica\/","title":{"rendered":"Fibrosis Qu\u00edstica"},"content":{"rendered":"\n[et_pb_section fb_built=\u00bb1&#8243; _builder_version=\u00bb4.4.8&#8243; custom_padding=\u00bb14px|||||\u00bb][et_pb_row _builder_version=\u00bb4.4.8&#8243; custom_padding=\u00bb0px|||||\u00bb custom_margin=\u00bb|auto|-61px|auto||\u00bb][et_pb_column _builder_version=\u00bb4.4.8&#8243; type=\u00bb4_4&#8243;][et_pb_image src=\u00bbhttp:\/\/liceaga.facmed.unam.mx\/revistas\/masalud\/wp-content\/uploads\/2020\/10\/fibrosis-1-scaled.jpg\u00bb _builder_version=\u00bb4.4.8&#8243; title_text=\u00bbfibrosis\u00bb hover_enabled=\u00bb0&#8243;][\/et_pb_image][\/et_pb_column][\/et_pb_row][et_pb_row _builder_version=\u00bb4.4.8&#8243; custom_padding=\u00bb20px||142px|||\u00bb min_height=\u00bb1024px\u00bb custom_margin=\u00bb|auto|-101px|auto||\u00bb][et_pb_column _builder_version=\u00bb4.4.8&#8243; type=\u00bb4_4&#8243;][et_pb_text _builder_version=\u00bb4.4.8&#8243; hover_enabled=\u00bb0&#8243;]<p style=\"text-align: right;\"><span>Victor Rubio Rojas<\/span><\/p>\n<p><span><\/span><\/p>\n<p><span>Se trata de una enfermedad gen\u00e9tica que produce disfunci\u00f3n de las gl\u00e1ndulas ex\u00f3crinas. \u00c9stas son gl\u00e1ndulas que secretan productos qu\u00edmicos a trav\u00e9s de conductos o tubos y llevan las secreciones producidas a una cavidad corporal, a la luz de un \u00f3rgano o a la superficie corporal.<\/span><\/p>\n<p><span>Esta disfunci\u00f3n glandular conduce a diversas alteraciones gastrointestinales y en las v\u00edas a\u00e9reas produce obstrucci\u00f3n cr\u00f3nica e infecci\u00f3n. Constituye la causa m\u00e1s frecuente de <strong>enfermedad pulmonar grave<\/strong> en la infancia, adem\u00e1s de presentarse con poliposis nasal, pansinusitis, prolapso rectal, colelitiasis e hiperglucemia insulinodependiente.<\/span><\/p>\n<p><span>El cuadro cl\u00ednico respiratorio es producido por la falta de aclaramiento de las bacterias inhaladas lo que produce inflamaci\u00f3n y sobreinfecci\u00f3n por m\u00faltiples g\u00e9rmenes como S. aureus, B. cepaceae, Burkholderia y P. aeruginosa.<\/span><\/p>\n<p><span>Existen algunas caracter\u00edsticas t\u00edpicas que pueden reconocerse seg\u00fan la edad del paciente; as\u00ed, en los <strong>reci\u00e9n nacidos<\/strong> y <strong>hasta al mes de edad<\/strong>, se observa bloqueo del intestino delgado causado por meconio, tambi\u00e9n llamado \u00edleo meconial y coloraci\u00f3n amarilla de la piel y mucosas o ictericia prolongada.<\/span><\/p>\n<p><strong><span>Hasta los dos a\u00f1os<\/span><\/strong><span> con presencia de secreciones lip\u00eddicas en las heces o esteatorrea, retraso del crecimiento, deshidrataci\u00f3n con alteraciones electrol\u00edticas e infecciones respiratorias de repetici\u00f3n.<\/span><\/p>\n<p><span>De los <strong>dos a los doce a\u00f1os <\/strong>con infecciones respiratorias de repetici\u00f3n, p\u00f3lipos y dilataciones bronquiales, tambi\u00e9n llamadas bronquiect\u00e1sias, donde se acumula gran cantidad de secreciones.<\/span><\/p>\n<p><strong><span>Despu\u00e9s de los doce a\u00f1os<\/span><\/strong><span> con insufiencia respiratoria, bronquiectasias, esterilidad, diabetes mellitus asociada a fibrosis quistica, afectaci\u00f3n hep\u00e1tica y litiasis biliar.<\/span><\/p>\n<p><span>Aunque existen diversos <strong>estudios<\/strong> para valorar la funci\u00f3n de cada \u00f3rgano afectado, los criterios <strong>diagn\u00f3sticos<\/strong> para esta enfermedad incluyen una prueba del sudor positiva, confirmaci\u00f3n con estudios de DNA y una o m\u00e1s de las siguientes enfermedades: enfermedad pulmonar cr\u00f3nica, insuficiencia pancre\u00e1tica ex\u00f3crina, antecedentes familiares positivos y tamiz neonatal positivo.<\/span><span style=\"font-size: 15px;\">\u00a0<\/span><\/p>\n<p><span>El <strong>tratamiento<\/strong> va encaminado a controlar las manifestaciones cl\u00ednicas en los distintos aparatos, adem\u00e1s de un r\u00e9gimen nutricional especializado, as\u00ed como a la prevenci\u00f3n, control y tratamiento de las complicaciones respiratorias y digestivas.<\/span><\/p>\n<p><span>Cabe destacar que, si se inicia el tratamiento antes de que la funci\u00f3n pulmonar est\u00e9 severamente deteriorada, cerca del 90% de los pacientes sobreviven despu\u00e9s de los 20 a\u00f1os. La causa m\u00e1s frecuente de muerte se debe a la afectaci\u00f3n respiratoria.<\/span><\/p>\n<p><span><\/span><\/p>\n<p>Referencias:<br \/><br \/><span><\/span><\/p>\n<p>1.- Isabel Largo Garc\u00eda (2009). \u00abFibrosis qu\u00edstica\u00bb. Revista Pediatr\u00eda Electr\u00f3nica 6. ISSN 0718-0918. Archivado desde el original el 3 de marzo de 2016<br \/>2.- Rowe SM, Miller S, Sorscher EJ. \u00abCystic Fibrosis.\u00bb N Engl J Med. 2005 may 12;352(19):1992-2001. <br \/>3.- Eggermont E, De Boeck K. \u00abSmall-intestinal abnormalities in cystic fibrosis patients.\u00bb Eur J Pediatr. 1991 oct;150(12):824-8. Rev.<br \/>4.- Cohn JA, Friedman KJ, Noone PG, Knowles MR, Silverman LM, Jowell PS. Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. N Engl J Med. 1998 sep 3;339(10):653-8.<br \/>5.- Stern, RC. \u00abThe diagnosis of cystic fibrosis.\u00bb N Engl J Med 1997; 336:487.<br \/>6.- Ramalho AS, Beck S, Meyer M, Penque D, Cutting GR, Amaral MD. Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis. Am J Respir Cell Mol Biol. 2002 nov;27(5):619-27. <br \/>7.- Cystic Fibrosis Foundation data (PDF) Archivado el 24 de julio de 2006 en la Wayback Machine.<\/p>\n<p><span><\/span><\/p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section]\n","protected":false},"excerpt":{"rendered":"<p>Es un trastorno hereditario en el cual las gl\u00e1ndulas exocrinas producen secreciones o moco excesivamente. Las gl\u00e1ndulas que suelen afectar con mayor frecuencia son las del p\u00e1ncreas, las del aparato respiratorio y las del sudor.  <\/p>\n","protected":false},"author":1,"featured_media":10666,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"_et_pb_use_builder":"on","_et_pb_old_content":"","_et_gb_content_width":"","_jetpack_newsletter_access":"","_jetpack_dont_email_post_to_subs":false,"_jetpack_newsletter_tier_id":0,"_jetpack_memberships_contains_paywalled_content":false,"_jetpack_memberships_contains_paid_content":false,"footnotes":"","jetpack_post_was_ever_published":false},"categories":[12],"tags":[979,216,976,902,378,931,1097],"class_list":["post-10665","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-descubrir","tag-ictericia","tag-moco","tag-pancreas","tag-pulmonar","tag-pulmones","tag-respiratorio","tag-sistema-respiratorio"],"acf":false,"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.5 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Fibrosis Qu\u00edstica - +Salud FacMed<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/massalud.facmed.unam.mx\/index.php\/fibrosis-quistica\/\" \/>\n<meta property=\"og:locale\" content=\"es_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Fibrosis Qu\u00edstica - +Salud FacMed\" \/>\n<meta property=\"og:description\" content=\"Es un trastorno hereditario en el cual las gl\u00e1ndulas exocrinas producen secreciones o moco excesivamente. 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