STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE

The nephrotic Syndrome is the most common cause of glomerulonephropathy in pediatrics. 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 s...

Descripción completa

Detalles Bibliográficos
Autores Principales: De la Hoz Mendoza, Ingrid, Santana Fontalvo, Milena, Espitaleta Vergara, Zilac, Parga, Carlos
Formato: Artículo (Article)
Lenguaje:Español (Spanish)
Publicado: Universidad Libre 2016
Materias:
id ir-10901-14696
recordtype dspace
institution Universidad Libre de Colombia
collection DSpace
language Español (Spanish)
topic Síndrome nefrótico
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Inhibidores de la calcineurina
spellingShingle Síndrome nefrótico
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Inhibidores de la calcineurina
De la Hoz Mendoza, Ingrid
Santana Fontalvo, Milena
Espitaleta Vergara, Zilac
Parga, Carlos
STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE
description The nephrotic Syndrome is the most common cause of glomerulonephropathy in pediatrics. 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, and the ones that do not respond to steroids: steroid-resistant nephrotic syndrome. 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, 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, additionally 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. 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.
format Artículo (Article)
author De la Hoz Mendoza, Ingrid
Santana Fontalvo, Milena
Espitaleta Vergara, Zilac
Parga, Carlos
author_facet De la Hoz Mendoza, Ingrid
Santana Fontalvo, Milena
Espitaleta Vergara, Zilac
Parga, Carlos
author_sort De la Hoz Mendoza, Ingrid
title STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE
title_short STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE
title_full STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE
title_fullStr STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE
title_full_unstemmed STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE
title_sort steroid-resistant nephrotic syndrome in pediatrics, an update
publisher Universidad Libre
publishDate 2016
_version_ 1678817229700857856
spelling ir-10901-146962020-09-15T22:16:53Z STEROID-RESISTANT NEPHROTIC SYNDROME IN PEDIATRICS, AN UPDATE SÍNDROME NEFRÓTICO RESISTENTE A CORTICOIDES EN PEDIATRÍA, ACTUALIZACIÓN De la Hoz Mendoza, Ingrid Santana Fontalvo, Milena Espitaleta Vergara, Zilac Parga, Carlos Síndrome nefrótico Glomeruloesclerosis focal y segmentaria Podocina (gen NPHS2) Corticoides Inhibidores de la calcineurina The nephrotic Syndrome is the most common cause of glomerulonephropathy in pediatrics. 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, and the ones that do not respond to steroids: steroid-resistant nephrotic syndrome. 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, 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, additionally 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. 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. El síndrome nefrótico (SN) constituye la glomerulopatía más frecuente en pediatría.  Las manifestaciones   clínicas   son   derivadas de la hipoalbuminemia.  El pilar del tratamiento continúa siendo de preferencia la terapia con corticoides.  Dependiendo de la respuesta a los corticoides, se clasifica en síndrome nefrótico corticoresistente (SNCR) y corticosensible.  La mayoría de los pacientes con SNCR tienen una Glomeruloesclerosis focal y segmentaria, asociada con un 50% de riesgo de enfermedad renal terminal, por lo que se recomienda la biopsia renal. Es importante la realización pruebas genéticas, ya que ciertas mutaciones resultan en corticorresistencia, siendo la mutación del gen NPHS2 (podocina) la más relacionada. Se incluyen las últimas investigaciones del tratamiento de esta entidad, los inhibidores de la calcineurina recomendados como terapia inicial, así como corticoides a altas dosis o en pulsos, ciclofosfamida, Mofetil micofenolato, Rituximab; obteniendo resultados variables por muestras pequeñas de pacientes en estos estudios. 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.  2016-07-05 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion https://revistas.unilibre.edu.co/index.php/biociencias/article/view/2538 10.18041/2390-0512/bioc..2.2538 spa https://revistas.unilibre.edu.co/index.php/biociencias/article/view/2538/1965 /*ref*/Aguilar, 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 2. Banaszak 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/s00467-011-2083-7 3. Andolino, TP., Reid-Adam, J. Nephrotic Syndrome, Pediatrics in Review, 2015;36(3) 4. Atehortúa, PS., Piedrahíta, V., Vanegas, JJ., Vélez, C., et al. Evolución a largo plazo de los niños con diagnóstico de síndrome nefrótico atendidos en el Hospital Universitario San Vicente de Paúl, Medellín, entre enero de 1960 y diciembre de 2009, Iatreia. 2013;26(2):127-135 5. Lane, J., Langman, C., Finberg, L. Pediatric Nephrotic Syndrome, Medscape, Updated. 2015 6. Basu, B., Mahapatra, T., Mondal, N. Mycophenolate Mofetil Following Rituximab in Children with Steroid-Resistant Nephrotic Syndrome, Pediatrics. 2015;136(1) 7. Azocara, 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- 36 8. Sandalio, A. Síndromes nefróticos congénitos y hereditarios. Rev. Cubana de Pediatría 2011;83(1):87-102 9. Steroid-resistant nephrotic syndrome in children, Kidney International Supplements (2012) 2, 172–176; Chapter 4 10. Chiou, YH., Wang, LY., Wang, T.H., Huang, SP. Genetic polymorphisms influence the steroid treatment of children with idiopathic nephrotic syndrome. Pediatr Nephrol. 2012;(27):1511–1517 11. Pais, P, Avner, E., Nephrotic Syndrome, Nelson Textbook of Pediatrics, Vol 2. 20a ed. Canada: Elsevier; 2015. p. 2521-2528 12. Gordillo, R., Spitzer, A. The Nephrotic Syndrome. Pediatr. Rev. 2009;30;94-105 DOI: 10.1542/pir.30-3-94 13. Ortiz, R. Síndrome nefrótico pediátrico. Protoc diagn ter pediatr. 2014; 1:283-301 14. Lovric, S., Fang, H., Vega-Warner, V., Sadowski, CE., Gee, HY., Halbritter, J, et al. Rapid detection of monogenic causes of childhood-onset steroid-resistant nephrotic syndrome. Clin J Am Soc Nephrol. 2014; 9:1109-16 15. Lipska, BS., Iatropoulos, P., Maranta, R., Caridi, G., Ozaltin, F., Anarat, A. et al. Genetic screening in adolescents with steroid- resistant nephrotic syndrome. Kidney Int. 2013; 84:206-13 16. Rood, IM., Deegens, JK., Wetzels, JF., Genetic causes of focal segmental glomerulosclerosis: implications for clinical practice. Nephrol Dial Transplant. 2012; 27:882-90. 17. Joshi, S., Andersen, R., Jespersen, B., Rittig, S. Genetics of steroid-resistant nephrotic syndrome: a review of mutation spectrum and suggested approach for genetic testing. Acta Pædiatrica. 2013; 102:844–856. DOI:10.1111/apa.12317 18. Sadowski, C.E. et al. A single-gene cause in 29.5% of cases of steroid-resistant nephrotic syndrome. J. Am. Soc. Nephrol. 2015; 26:1279–1289 19. Wu, HM., Tang, JL., Sha, ZH., Cao, L., Li, YP. Interventions for preventing infection in nephrotic syndrome. Cochrane Database Syst Rev. 2004. CD003964. 20. Basiratnia, M., Yavarian, M., Torabinezhad, S., Erjaee, A. NPHS2 Gene in Steroid-resistant Nephrotic Syndrome Prevalence, Clinical Course, and Mutational Spectrum in South-West Iranian Children. Iranian Journal of Kidney Diseases. 2013;7(5) 21. Ferraris, JR., Argibay, PF., Costa, L., Jimenez, G., Coccia, PA., Ghezzi, LF. et al. Influence of CYP3A5 polymorphism on tacrolimus maintenance doses and serum levels after renal transplantation: age dependency and pharmacological interaction with steroids. 2011. Pediatr Transplant. 15:525–532 22. Collins, FS., Varmus, H., A new initiative on precision medicine. N Engl J Med. 2015; 372:793-5 23. Zhou, T-B., Qin, Y-H., Su, L-N., Lei, F-Y., Huang, W-F., et al. ACE I/D Gene Polymorphism Can’t Predict the Steroid Responsiveness in Asian Children with Idiopathic Nephrotic Syndrome: A Meta-Analysis. PLoS ONE. 2011; 6(5): e19599. doi: 10.1371/journal.pone.0019599 24. Prasun, P., Prasad, N., Tripathi, G., et al. Association of angiotensin-converting enzyme gene I/D polymorphism with steroid responsiveness in childhood nephrotic syndrome. Indian J Nephrol. 2011; (21):26–29. 25. Kapoor, K., Saha, A., Dubey, N., Goyal, P., Suresh, C., Batra, V., Upadhayay, U. Subclinical non-autoimmune hypothyroidism in children with steroid resistant nephrotic syndrome. Clin Exp Nephrol.2014;18:(1)13–117. DOI 10.1007/s10157-013-0800-1 26. Candan, C., Canpolat, N., Gökalp, S., Yıldız, N., Turhan, P., Taşdemir, M. et al. Subclinical cardiovascular disease and its association withrisk factors in children with steroid-resistant nephrotic síndrome, Pediatr Nephrol. 2014; 29:95–102 27. Hammad, A., Yahia, S., Samy, M., Bakr, A., El-farahaty, R. Low expression of glucocorticoid receptors in children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2013; 28:759–763. DOI 10.1007/s10157-013-0800. 28. Hladunewich, M., Avila-Casado, C., Gipson, D. Focal Segmental Glomerulosclerosis, National Kidney Foundation Primer on Kidney Diseases, 6a Edic, 2014, cap 18, 170-175 29. Niaudet, P., Avner, E., Harmon, W, N. Yoshikawa. Steroid-Sensitive Idiopathic Nephrotic Syndrome in Children. En: Springer‐Verlag Berlin Heidelberg, editors. Pediatric Nephrology. Chap. 24, 6th ed. 2009. 30. Bienias, B., Zaja, M., Borze, H., Sikora, P., Wieczorkiewicz-Płaza, A., Wilczyn´ska, B. Early Markers of Tubulointerstitial Fibrosis in Children with Idiopathic Nephrotic Syndrome. Eng. Medicine. 2015;94(42) 31. Wu, Y., Su, T., Yang, L., et al. Urinary neutrophil gelatinase-associated lipocalin: a potential biomarker for predicting rapid progression of drug-induced chronic tubulointerstitial nephritis. Am J Med Sci. 2010; 339:537–542 32. Ko, GJ., Grigoryev, DN., Linfert, D., et al. Transcriptional analysis of kidneys during repair from AKI reveals possible roles for NGAL and KIM-1 as biomarkers of AKI-to-CKD transition. Am J Physiol Renal Physiol. 2010;298: F1472–F1483 33. Xia, ZK., Jin, Y., Lu, L., The Nephrology Group, Pediatric Association Branch of Chinese Medical Association. Guide interpretation for children with steroid resistant nephrotic syndrome. Chin. J. Pediatr. 2010; 48:72–5 34. Wu, B., Mao, J., Shen, H. et al. Triple immunosuppressive therapy in steroid-resistant nephrotic syndrome children with tacrolimus resistance or tacrolimus sensitivity but frequently relapsing. Nephrology. 2015; 20:18–24. doi:10.1111/nep.12351 35. Sgambat, K., Banks, M., Moudgil, A. Effect of galactose on glomerular permeability and proteinuria in steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2013; 28:2131–2135 36. Lombel, R., Hodson, E., Gipson, D., Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO. Pediatr Nephrol.2013; 28:409–414 37. Husen, M., Kemper, M. New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol. 2011; 26:881–892 38. Gulati, A., Sinha, A., Jordan, SC. et al. Efficacy and safety of treatment with rituximab for difficult steroid-resistant and -dependent nephrotic syndrome: multicentric report. Clin J Am Soc Nephrol. 2010;5(12):2207–2212 39. Prytuła, A., Iijima, K., Kamei, K., et al. Rituximab in refractory nephrotic syndrome. Pediatr Nephrol. 2010;25(3):461–468 40. Nickavar, A., Safarzadeh, A., Sotoudeh, K., Otukesh, H., Hooman, N. Mycophenolate Mofetil for Treatment of Idiopathic Nephrotic Syndrome in Children. Iranian Journal of Kidney Diseases. 2012;6(5) 41. Sgambat, K., Banks, M., Moudgil, A. Effect of galactose on glomerular permeability and proteinuria in steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2013; 28:2131–2135 42. Hogan, J., Bomback, A., Mehta, K., Canetta, P., Rao, M., Appel, G., et al. Treatment of Idiopathic FSGS with Adrenocorticotropic Hormone Gel. Clin J Am Soc Nephrol. 2013; 8: 2072–2081 43. Zyadaa, F., Elbatrawyd, A., Khalifad, D., Walyb, S., Mouawadb, E., Ahmedc, D. Psychosocial aspects of nephrotic syndrome among children and their caregivers, Middle East Current Psychiatry. 2013; 20:164–171 Derechos de autor 2018 Biociencias https://creativecommons.org/licenses/by-sa/4.0/ http://creativecommons.org/licenses/by-nc-nd/2.5/co/ Atribución-NoComercial-SinDerivadas 2.5 Colombia application/pdf Universidad Libre Biociencias; Vol 11 No 2 (2016): Biociencias; 17-28 Biociencias; Vol. 11 Núm. 2 (2016): Biociencias; 17-28 2390-0512 0124-0110
score 11,373924