Síndrome nefrótico resistente a corticoides en pediatría, actualización

El síndrome nefrótico (SN) constituye la glomerulopatia más frecuente en pediatría. La incidencia anual del SN idiopático en niños, a nivel mundial se ha estimado en 2-7 por cada 100.000 niños. Las manifestaciones clínicas son derivadas de la hipoalbuminemia. El pilar del tratamiento continúa siendo...

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Autores:
De la Hoz Mendoza, Ingrid Rosana
Santana Fontalvo, Milena María
Tipo de recurso:
Trabajo de grado de pregrado
Fecha de publicación:
2016
Institución:
Universidad Libre
Repositorio:
RIU - Repositorio Institucional UniLibre
Idioma:
spa
OAI Identifier:
oai:repository.unilibre.edu.co:10901/10226
Acceso en línea:
https://hdl.handle.net/10901/10226
Palabra clave:
Síndrome nefrótico
Pediatría
Síndrome nefrótico en niños
Steroid-sensitive nephrotic syndrome
Focal segmental glomerulosclerosis
NPHS2 gene mutation (podocin)
Corticosteroids
Calcineurin inhibitors
SINDROME NEFROTICO
CORTICOESTEROIDES
Síndrome nefrótico corticoresistente
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Esteroides
Corticosteroides
Inhibidores de la calcineurina
Rights
openAccess
License
http://creativecommons.org/licenses/by-nc-nd/2.5/co/
id RULIBRE2_31ce2e059f074952df6b0abbcdc971b4
oai_identifier_str oai:repository.unilibre.edu.co:10901/10226
network_acronym_str RULIBRE2
network_name_str RIU - Repositorio Institucional UniLibre
repository_id_str
dc.title.spa.fl_str_mv Síndrome nefrótico resistente a corticoides en pediatría, actualización
dc.title.eng.fl_str_mv Steroid-resistant nephrotic syndrome in pediatrics, an update
title Síndrome nefrótico resistente a corticoides en pediatría, actualización
spellingShingle Síndrome nefrótico resistente a corticoides en pediatría, actualización
Síndrome nefrótico
Pediatría
Síndrome nefrótico en niños
Steroid-sensitive nephrotic syndrome
Focal segmental glomerulosclerosis
NPHS2 gene mutation (podocin)
Corticosteroids
Calcineurin inhibitors
SINDROME NEFROTICO
CORTICOESTEROIDES
Síndrome nefrótico corticoresistente
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Esteroides
Corticosteroides
Inhibidores de la calcineurina
title_short Síndrome nefrótico resistente a corticoides en pediatría, actualización
title_full Síndrome nefrótico resistente a corticoides en pediatría, actualización
title_fullStr Síndrome nefrótico resistente a corticoides en pediatría, actualización
title_full_unstemmed Síndrome nefrótico resistente a corticoides en pediatría, actualización
title_sort Síndrome nefrótico resistente a corticoides en pediatría, actualización
dc.creator.fl_str_mv De la Hoz Mendoza, Ingrid Rosana
Santana Fontalvo, Milena María
dc.contributor.advisor.none.fl_str_mv Espitaleta Vergara, Zilac
Parga Lozano, Carlos
dc.contributor.author.none.fl_str_mv De la Hoz Mendoza, Ingrid Rosana
Santana Fontalvo, Milena María
dc.subject.spa.fl_str_mv Síndrome nefrótico
Pediatría
Síndrome nefrótico en niños
topic Síndrome nefrótico
Pediatría
Síndrome nefrótico en niños
Steroid-sensitive nephrotic syndrome
Focal segmental glomerulosclerosis
NPHS2 gene mutation (podocin)
Corticosteroids
Calcineurin inhibitors
SINDROME NEFROTICO
CORTICOESTEROIDES
Síndrome nefrótico corticoresistente
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Esteroides
Corticosteroides
Inhibidores de la calcineurina
dc.subject.subjectenglish.eng.fl_str_mv Steroid-sensitive nephrotic syndrome
Focal segmental glomerulosclerosis
NPHS2 gene mutation (podocin)
Corticosteroids
Calcineurin inhibitors
dc.subject.lemb.spa.fl_str_mv SINDROME NEFROTICO
CORTICOESTEROIDES
dc.subject.proposal.spa.fl_str_mv Síndrome nefrótico corticoresistente
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Esteroides
Corticosteroides
Inhibidores de la calcineurina
description El síndrome nefrótico (SN) constituye la glomerulopatia más frecuente en pediatría. La incidencia anual del SN idiopático en niños, a nivel mundial se ha estimado en 2-7 por cada 100.000 niños. Las manifestaciones clínicas son derivadas de la hipoalbuminemia. El pilar del tratamiento continúa siendo de preferencia a la terapia con corticoides. Dependiendo de la respuesta a los corticoides, se reconocen dos categorías: el Síndrome nefrótico corticosensible (el 90% de los casos) y los que no logran una respuesta al tratamiento con corticoides: el síndrome nefrótico corticoresistente (SNCR, del 10-20% de los casos). La mayoría de los pacientes con SNCR tienen una Glomeruloesclerosis focal y segmentaria (GEFS), la cual está asociada con un 50% de riesgo de enfermedad renal terminal dentro de los 5 años del diagnóstico, por lo que se recomienda la biopsia renal en pacientes con SNCR. Es de resaltar, la importancia de las pruebas genéticas, por el hecho que ciertas mutaciones resultan en la no respuesta a la terapia con corticosteroides, siendo la mutación del gen NPHS2, (podocina) la principal causa descrita en niños con esta condición. Los inhibidores de la calcineurina (ICN) son recomendados como la terapia inicial para estos pacientes, pero confieren un riesgo de inmunosupresión y nefrotoxicidad adicional en el tiempo. Se incluyen en las últimas investigaciones fármacos como corticoides a altas dosis o en pulsos, ciclofosfamida, Mofetil micofenolato, Rituximab; solos o en combinación, obteniendo resultados variables e imprecisos por muestras pequeñas de pacientes en estos estudios; así como el uso experimental de terapias como la galactosa, que, aunque no hay evidencia de su uso, puede constituirse en una medida preventiva en los pacientes con GEFS y por tanto con SNCR. Con este artículo se pretende realizar una revisión actualizada de la literatura mundial y nacional acerca del SNCR en pediatría, en la búsqueda de nuevos enfoques de diagnóstico y tratamiento.
publishDate 2016
dc.date.created.none.fl_str_mv 2016
dc.date.accessioned.none.fl_str_mv 2017-07-25T21:19:40Z
dc.date.available.none.fl_str_mv 2017-07-25T21:19:40Z
dc.type.local.spa.fl_str_mv Tesis de Especialización
dc.type.hasversion.spa.fl_str_mv info:eu-repo/semantics/acceptedVersion
dc.type.coar.spa.fl_str_mv http://purl.org/coar/resource_type/c_7a1f
dc.type.driver.spa.fl_str_mv info:eu-repo/semantics/bachelorThesis
format http://purl.org/coar/resource_type/c_7a1f
status_str acceptedVersion
dc.identifier.uri.none.fl_str_mv https://hdl.handle.net/10901/10226
dc.identifier.instname.spa.fl_str_mv instname:Universidad Libre
dc.identifier.reponame.spa.fl_str_mv reponame:Repositorio Institucional Universidad Libre
url https://hdl.handle.net/10901/10226
identifier_str_mv instname:Universidad Libre
reponame:Repositorio Institucional Universidad Libre
dc.language.iso.none.fl_str_mv spa
language spa
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spelling Espitaleta Vergara, ZilacParga Lozano, CarlosDe la Hoz Mendoza, Ingrid RosanaSantana Fontalvo, Milena MaríaBarranquilla2017-07-25T21:19:40Z2017-07-25T21:19:40Z2016https://hdl.handle.net/10901/10226instname:Universidad Librereponame:Repositorio Institucional Universidad LibreEl síndrome nefrótico (SN) constituye la glomerulopatia más frecuente en pediatría. La incidencia anual del SN idiopático en niños, a nivel mundial se ha estimado en 2-7 por cada 100.000 niños. Las manifestaciones clínicas son derivadas de la hipoalbuminemia. El pilar del tratamiento continúa siendo de preferencia a la terapia con corticoides. Dependiendo de la respuesta a los corticoides, se reconocen dos categorías: el Síndrome nefrótico corticosensible (el 90% de los casos) y los que no logran una respuesta al tratamiento con corticoides: el síndrome nefrótico corticoresistente (SNCR, del 10-20% de los casos). La mayoría de los pacientes con SNCR tienen una Glomeruloesclerosis focal y segmentaria (GEFS), la cual está asociada con un 50% de riesgo de enfermedad renal terminal dentro de los 5 años del diagnóstico, por lo que se recomienda la biopsia renal en pacientes con SNCR. Es de resaltar, la importancia de las pruebas genéticas, por el hecho que ciertas mutaciones resultan en la no respuesta a la terapia con corticosteroides, siendo la mutación del gen NPHS2, (podocina) la principal causa descrita en niños con esta condición. Los inhibidores de la calcineurina (ICN) son recomendados como la terapia inicial para estos pacientes, pero confieren un riesgo de inmunosupresión y nefrotoxicidad adicional en el tiempo. Se incluyen en las últimas investigaciones fármacos como corticoides a altas dosis o en pulsos, ciclofosfamida, Mofetil micofenolato, Rituximab; solos o en combinación, obteniendo resultados variables e imprecisos por muestras pequeñas de pacientes en estos estudios; así como el uso experimental de terapias como la galactosa, que, aunque no hay evidencia de su uso, puede constituirse en una medida preventiva en los pacientes con GEFS y por tanto con SNCR. Con este artículo se pretende realizar una revisión actualizada de la literatura mundial y nacional acerca del SNCR en pediatría, en la búsqueda de nuevos enfoques de diagnóstico y tratamiento.The nephrotic Syndrome is the most common cause of glomerulonephropathy in pediatrics. The estimated worldwide annual incidence of INS (idiopathic nephrotic syndrome) in children is 2 -7 per 100.000 children. Clinical findings are derived from hypoalbuminemia. The main therapy remains to be corticosteroids. According to the response to the corticosteroids therapy, there are two categories: Steroid-sensitive nephrotic syndrome (90% of the cases) and the ones that do not respond to steroids: steroid-resistant nephrotic syndrome (10 - 20% of the cases). Most of the patients that have a corticoid-resistant nephrotic syndrome have a focal segmental glomerulosclerosis, which is associated with a 50% risk of terminal renal disease within the next five years of the diagnosis, so renal biopsy is recommended in these patients. Another important factor is genetic testing, this is because some mutations are associated with a failure in the response to steroids, an example is the NPHS2 gene mutation (podocin) which is the most common described cause of NS. Calcineurin inhibitors are recommended as the best initial therapy in these cases, but they do conferee a high risk of immunosuppression and nephrotoxicity at long term. Recent researches include high dose or pulses of corticosteroids, cyclophosphamide, mycophenolate mofetil, and rituximab, alone or in combination as alternate option obtaining variable and imprecise results due to small sample sizes in the studies; as well as galactose, although there is yet not evidence of its use, it may become a preventive alternative for patients with focal Glomerular sclerosis and resulting steroid resistant Nephrotic syndrome. With this article we introduce an updated review of the national and international literature about CRNS in Pediatrics, looking forward to achieve new alternatives for its diagnosis and more efficient treatments.PDFapplication/pdfspahttp://creativecommons.org/licenses/by-nc-nd/2.5/co/Atribución-NoComercial-SinDerivadas 2.5 Colombiainfo:eu-repo/semantics/openAccesshttp://purl.org/coar/access_right/c_abf2Síndrome nefróticoPediatríaSíndrome nefrótico en niñosSteroid-sensitive nephrotic syndromeFocal segmental glomerulosclerosisNPHS2 gene mutation (podocin)CorticosteroidsCalcineurin inhibitorsSINDROME NEFROTICOCORTICOESTEROIDESSíndrome nefrótico corticoresistenteGlomeruloesclerosis focal y segmentariaPodocina (gen NPHS2)CorticoidesEsteroidesCorticosteroidesInhibidores de la calcineurinaSíndrome nefrótico resistente a corticoides en pediatría, actualizaciónSteroid-resistant nephrotic syndrome in pediatrics, an updateTesis de Especializacióninfo:eu-repo/semantics/acceptedVersionhttp://purl.org/coar/resource_type/c_7a1finfo:eu-repo/semantics/bachelorThesisAguilar, M., Zepeda, C., Ibarra, M., Sánchez, J., Luna, R., Mendoza, M. et al. Síndrome nefrótico corticoresistente: 15 años de experiencia en el Hospital de Pediatría, Centro Médico Nacional Siglo XXI. Bol. Med Hosp Infant Mex 2012;69(5):411-417. Sandalio, A. Síndromes nefróticos congénitos y hereditarios. Rev. Cubana de Pediatría 2011;83(1):87-102Banaszak B, Banaszak P. The increasing incidence of initial steroid resistance in childhood nephrotic syndrome. Pediatr Nephrol 2011 [citado 10 de enero de 2012]. Disponible en: http://www.Springerlink.com/DOI10.1007/s00467-011- 2083-7Mortazavi, F., Soleimani, Y. et al. Steroid response pattern and outcome of pediatric idiopathic nephrotic syndrome: a single-center experience in northwest Iran, Therapeutics and Clinical Risk Management 2011:7 167–171Azocara, M., Vegab, A., Farfánc, M., Cano, F. Identificación de variantes del gen NPHS2 en niños con síndrome nefrótico corticoresistente, Rev Chil Pediatr. 2016;87(1):31---36Atehortúa, PS., Piedrahíta, V., Vanegas, JJ., Vélez, C., et al. 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Psychosocial aspects of nephrotic syndrome among children and their caregivers, Middle East Current Psychiatry. 2013; 20:164–171ORIGINAL44190034.pdf44190034.pdfTrabajo de Gradoapplication/pdf1453583http://repository.unilibre.edu.co/bitstream/10901/10226/1/44190034.pdf5aba6af3899a020c0d7a9d4aa06ed731MD51LICENSElicense.txtlicense.txttext/plain; charset=utf-81748http://repository.unilibre.edu.co/bitstream/10901/10226/2/license.txt8a4605be74aa9ea9d79846c1fba20a33MD52THUMBNAIL44190034.pdf.jpg44190034.pdf.jpgIM Thumbnailimage/jpeg5604http://repository.unilibre.edu.co/bitstream/10901/10226/3/44190034.pdf.jpge7d09a07a89fe58bf793dc6af9cdcc99MD5310901/10226oai:repository.unilibre.edu.co:10901/102262022-10-11 12:08:27.007Repositorio Institucional Unilibrerepositorio@unilibrebog.edu.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