SÍNDROME NEFRÓTICO RESISTENTE A CORTICOIDES EN PEDIATRÍA, ACTUALIZACIÓN
DOI:
https://doi.org/10.18041/2390-0512/bioc..2.2538Palabras clave:
Síndrome nefrótico, Glomeruloesclerosis focal y segmentaria, Podocina (gen NPHS2), Corticoides, Inhibidores de la calcineurinaResumen
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.
Descargas
Los datos de descarga aún no están disponibles.
Referencias
1. 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
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
Descargas
Publicado
2016-07-05
Número
Sección
ARTÍCULOS DE REVISIÓN
Licencia
-
Reconocimiento — Debe reconocer adecuadamente la autoría, proporcionar un enlace a la licencia e indicar si se han realizado cambios<. Puede hacerlo de cualquier manera razonable, pero no de una manera que sugiera que tiene el apoyo del licenciador o lo recibe por el uso que hace.
-
NoComercial — No puede utilizar el material para una finalidad comercial.
-
CompartirIgual — Si remezcla, transforma o crea a partir del material, deberá difundir sus contribuciones bajo la misma licencia que el original.
- No hay restricciones adicionales — No puede aplicar términos legales o medidas tecnológicas que legalmente restrinjan realizar aquello que la licencia permite.
Cómo citar
SÍNDROME NEFRÓTICO RESISTENTE A CORTICOIDES EN PEDIATRÍA, ACTUALIZACIÓN. (2016). Biociencias, 11(2), 17-28. https://doi.org/10.18041/2390-0512/bioc..2.2538
