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 contin...

Descripción completa

Detalles Bibliográficos
Autores Principales: De la Hoz Mendoza, Ingrid Rosana, Santana Fontalvo, Milena María
Otros Autores: Espitaleta Vergara, Zilac
Formato: Trabajo de grado (Bachelor Thesis)
Lenguaje:Español (Spanish)
Publicado: 2017
Materias:
Acceso en línea:http://hdl.handle.net/10901/10226
id ir-10901-10226
recordtype dspace
institution Universidad Libre de Colombia
collection DSpace
language Español (Spanish)
topic Síndrome nefrótico corticoresistente
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Esteroides
Corticosteroides
Inhibidores de la calcineurina
Steroid-sensitive nephrotic syndrome
Focal segmental glomerulosclerosis
NPHS2 gene mutation (podocin)
Corticosteroids
Calcineurin inhibitors
SINDROME NEFROTICO
CORTICOESTEROIDES
spellingShingle Síndrome nefrótico corticoresistente
Glomeruloesclerosis focal y segmentaria
Podocina (gen NPHS2)
Corticoides
Esteroides
Corticosteroides
Inhibidores de la calcineurina
Steroid-sensitive nephrotic syndrome
Focal segmental glomerulosclerosis
NPHS2 gene mutation (podocin)
Corticosteroids
Calcineurin inhibitors
SINDROME NEFROTICO
CORTICOESTEROIDES
De la Hoz Mendoza, Ingrid Rosana
Santana Fontalvo, Milena María
Síndrome nefrótico resistente a corticoides en pediatría, actualización
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.
author2 Espitaleta Vergara, Zilac
author_facet Espitaleta Vergara, Zilac
De la Hoz Mendoza, Ingrid Rosana
Santana Fontalvo, Milena María
format Trabajo de grado (Bachelor Thesis)
author De la Hoz Mendoza, Ingrid Rosana
Santana Fontalvo, Milena María
author_sort De la Hoz Mendoza, Ingrid Rosana
title Síndrome nefrótico resistente a corticoides en pediatría, actualización
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
publishDate 2017
url http://hdl.handle.net/10901/10226
_version_ 1678816455829749760
spelling ir-10901-102262020-09-15T22:16:56Z Síndrome nefrótico resistente a corticoides en pediatría, actualización Steroid-resistant nephrotic syndrome in pediatrics, an update De la Hoz Mendoza, Ingrid Rosana Santana Fontalvo, Milena María Espitaleta Vergara, Zilac Parga Lozano, Carlos Síndrome nefrótico corticoresistente Glomeruloesclerosis focal y segmentaria Podocina (gen NPHS2) Corticoides Esteroides Corticosteroides Inhibidores de la calcineurina Steroid-sensitive nephrotic syndrome Focal segmental glomerulosclerosis NPHS2 gene mutation (podocin) Corticosteroids Calcineurin inhibitors SINDROME NEFROTICO CORTICOESTEROIDES 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. 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. 2017-07-25T21:19:40Z 2017-07-25T21:19:40Z 2016 info:eu-repo/semantics/bachelorThesis Tesis de Especialización info:eu-repo/semantics/acceptedVersion http://hdl.handle.net/10901/10226 spa http://creativecommons.org/licenses/by-nc-nd/2.5/co/ Atribución-NoComercial-SinDerivadas 2.5 Colombia OpenAccess Abierto (Texto Completo) PDF application/pdf Barranquilla reponame:Repositorio Institucional Universidad Libre instname:Universidad Libre 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 . Sandalio, A. Síndromes nefróticos congénitos y hereditarios. Rev. Cubana de Pediatría 2011;83(1):87-102 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 Mortazavi, 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 2011:7 167–171 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 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 Luna-Fabritius, A., Nokkala, E., Sarkamo, V. Finlandia y su historia: una presentación., Istor. Primavera. 2012;12(48):3-9 Cañás, L. Historia y Etnografía de una Colonia Menonita. Editorial Antropofagia, Buenos Aires, 2005 Bolaños, L., Castaño, I. Características clínicas e histológicas del Sindrome Nefrótico Primario, Colombia Médica 2005;(36):29-33 .Kiryluk, K., Li, Y., Scolari, F., Sanna-Cherchi, S., Choi, M., Verbitsky, M. et al. Discovery of new risk loci for IgA nephropathy implicates genes involved in immunity against intestinal pathogens. Nat Genet. 2014; 46:1187-96 Bello, P. Síndrome nefrótico en el primer año de vida; An Pediatr Contin. 2014;12(1):17-24 Azócar, M. Síndrome nefrótico congénito por mutación del gen de la nefrina. Caso clínico, Rev Chil Pediatr. 2011;82(5):426-431 Caridi, G., Trivelli, A., Sanna-Cherchi, S., Perfumo, F., Ghiggeri, GM. Familial forms of nephrotic syndrome. Pediatr Nephrol. 2010;25(2):241-52 Tryggvason, K., Patrakka, J., Wartiovaara, J. Hereditary proteinuria syndromes and mechanisms of proteinuria. N Engl J Med. 2006;354(13):1387-401 Stanescu, HC., Arcos-Burgos, M., Medlar, A., Bockenhauer, D., Kottgen, A., Dragomirescu, L. et al. Risk HLA-DQA1 and PLA (2) R1 alleles in idiopathic membranous nephropathy. N Engl J Med. 2011; 364:616-26 Hladunewich, M., Avila-Casado, C., Gipson, D. Focal Segmental Glomerulosclerosis, National Kidney Foundation Primer on Kidney Diseases, 6a Edic, 2014, cap 18, 170-175 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 Steroid-resistant nephrotic syndrome in children, Kidney International Supplements (2012) 2, 172–176; Chapter 4 Andolino, TP., Reid-Adam, J. Nephrotic Syndrome, Pediatrics in Review, 2015;36(3) Lane, J., Langman, C., Finberg, L. Pediatric Nephrotic Syndrome, Medscape, Updated. 2015 .Basu, B., Mahapatra, T., Mondal, N. Mycophenolate Mofetil Following Rituximab in Children with Steroid-Resistant Nephrotic Syndrome, Pediatrics. 2015;136(1 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 .Iijima, K., Sako, M., Nozu, K. et. al. Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial. Lancet. 2014; 384:1273–81 Wu, B., Mao, J., Shen, H. et al. Triple immunosuppressive therapy in steroidresistant nephrotic syndrome children with tacrolimus resistance or tacrolimus sensitivity but frequently relapsing. Nephrology. 2015; 20:18–24. doi:10.1111/nep.12351 Greenbaum LA, Benndorf R, Smoyer WE. Childhood nephrotic syndrome– current and future therapies. Nat Rev Nephrol. 2012; 8:445–458 Reiser, J., Glassock, R., Fervenza, F. Epidemiology, classification, and pathogenesis of focal segmental glomerulosclerosis, uptodate, 2015 Kitiyakara, C., Eggers, P., Kopp, JB. Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. Am J Kidney Dis. 2004; 44:815 Filler, G. Robinson, L. Should we stop dosing steroids per body surface área for nephrotics? Pediatr Nephrol. 2016; 31:519–522 Zaffanello, M., Franchini, M. Thromboembolism in childhood nephrotic syndrome: a rare but serious complication. Hematology. 2007;12(1):69-73 Braun, D., Sadowski, C., Kohl, S. Lovric, S., Astrinidis, S. et al. Mutations in nuclear pore genes NUP93, NUP205 and XPO5 cause steroid resistant nephrotic syndrome. Nature Genetics. 2016;48(4) 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 Genovese, G., Friedman, DJ., Ross, MD., Lecordier, L., Uzureau, P. et al. Association of trypanolytic ApoL1 variants with kidney disease in African Americans. Science. 2010; 329:841-5 Okamoto, K., Tokunaga, K., Doi, K., Fujita, T., Suzuki, H., Katoh, T. et al. Common variation in GPC5 is associated with acquired nephrotic syndrome. Nat Genet. 2011; 43:459-63 Gbadegesin, RA., Winn, MP., Smoyer, WE. Genetictestingin nephrotic syndrome–challenges and opportunities. Nat Rev Nephrol. 2013; 9:179-84 Sampson, MG. Opportunities and Challenges of Genotyping Patients with Nephrotic Syndrome in the Genomic Era. Seminars in Nephrology, 2015;35(3):212–221 .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 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 Rood, IM., Deegens, JK., Wetzels, JF., Genetic causes of focal segmental glomerulosclerosis: implications for clinical practice. Nephrol Dial Transplant. 2012; 27:882-90. Mishra, O., Singh, A., Abhinay, A., Gopeshwar, P. et al., WT1 Mutations in Steroid-Resistant Idiopathic Nephrotic Syndrome, Saudi J Kidney Dis Transpl 2016;27(2):417-418 Lombel, R., Hodson, E., Gipson, D., Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO. Pediatr Nephrol.2013; 28:409–414 Pais, P, Avner, E., Nephrotic Syndrome, Nelson Textbook of Pediatrics, Vol 2. 20a ed. Canada: Elsevier; 2015. p. 2521-2528 Gordillo, R., Spitzer, A. The Nephrotic Syndrome. Pediatr. Rev. 2009;30;94-105 DOI: 10.1542/pir.30-3-94 Srivastava, R., Aggarwal, V. Steroid resistant nephrotic Syndrome in children. Apollo Medicine. 2005;2(3) Ortiz, R. Síndrome nefrótico pediátrico. Protoc diagn ter pediatr. 2014; 1:283-301 Gordillo, G., Exeni, R., De La Cruz, J. Nefrologia pediatrica. España: Ediciones Elsevier; 2009 Elie, V., Fakhoury, M., Deschênes, G., Jacqz-Aigrain, E. Physiopathology of idiopathic nephrotic syndrome: lessons from glucocorticoids and epigenetic perspectives. Pediatr Nephrol. 2012;27(8):1249-56 Van den Berg, JG., Weening, JJ. Role of the immune system in the pathogenesis of idiopathic nephrotic syndrome. Clin Sci (Lond). 2004;107(2):125-36 Wei, C., Trachtman, H., Li, J., Dong, C., Friedman, AL., Gassman, JJ. Circulating suPAR in two cohorts of primary FSGS. J Am Soc Nephrol. 2012;23(12):2051-9 Benoit, G., Machuca, E., Antignac, C. Hereditary nephrotic syndrome: a systematic approach for genetic testing and a review of associated podocyte gene mutations. Pediatr Nephrol. 2010;25(9):1621-32 Anderson, S., Komers, R., Brenner, BM. Renal and Systemic Manifestations of Glomerular Disease. Brenner BM. Brenner and Rector's The Kidney. 8th ed. Philadelphia: Saunders Elsvie; 2008;26 Saland, JM., Ginsberg, H., Fisher, EA. Dyslipidemia in pediatric renal disease: epidemiology, pathophysiology, and management. Curr Opin Pediatr. 2002;14(2):197-204 .Loscalzo, J. Venous thrombosis in the nephrotic syndrome. N Engl J Med. 2013;368(10):956-8. 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. Wu, HM., Tang, JL., Sha, ZH., Cao, L., Li, YP. Interventions for preventing infection in nephrotic syndrome. Cochrane Database Syst Rev. 2004. CD003964. 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 Bodria, M., Ozaltin, F., Gheisari, A., Melk, A., Azocar, M. et al. Spectrum of steroid-resistant and congenital nephrotic syndrome in children: The PodoNet registry cohort. Agnes Trautmann. Clin J Am Soc Nephrol. 2015;10(4):592 Jungraithmayr, TC., Hofer, K., Cochat, P., Chernin, G., Cortina, G., Fargue, S., et al. Screening for NPHS2 mutations may help predict FSGS recurrence after transplantation. J Am Soc Nephrol. 2011; 22:579-85 Koboldt, DC., Steinberg, KM., Larson, DE., Wilson, RK., Mardis, ER. The nextgeneration sequencing revolution and its impact on genomics. Cell. 2013; 155:27-38 Toward precision medicine: building a knowledge network for biomedical research and a new taxonomy of disease the National Academies Collection. Bethesda: National Institutes of Health; 2011. Collins, FS., Varmus, H., A new initiative on precision medicine. N Engl J Med. 2015; 372:793-5 Saleem, MA. New developments in steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2013; 28:699-709. Manolio, TA. Bringinggenome-wide association findings into clinical use. Nat Rev Genet. 2013; 14:549-58 Gbadegesin, RA., Adeyemo, A., Webb, NJ., Greenbaum, LA., Abeyagunawardena, A., Thalgahagoda, S. et al. HLA-DQA1 and PLCG2 are candidate risk loci for childhood-onset steroid- sensitive nephrotic syndrome. J Am Soc Nephrol. 2014 Huyghe, JR., Jackson, AU., Fogarty, MP., Buchkovich, ML., Stančáková, A., Stringham, HM., et al. Exomearray analysis identifies new loci and low frequency variants in Fluencing insulin processing and secretion. Nat.Genet.,2013;45:197- 201 Li, Y., Sidore, C., Kang, HM., Boehnke, M., Abecasis, GR. Low- coverage sequencing: implications for design of complex trait association studies. Genome Res. 2011; 21:940-51 Lee, S., Abecasis, GR., Boehnke, M., Lin, X. Rare-variant association analysis: study designs and statistical tests. Am J Hum Genet. 2014; 95:5-23 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) Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO Clinical Practice Guideline for Glomerulonephritis. Kidney Int Suppl. 2012; 2:139- 274 Che, R., Zhang, A. Mechanisms of Glucocorticoid Resistance in Idiopathic Nephrotic Syndrome. Kidney Blood Press Res. 2013; 37:360-378. DOI: 10.1159/000350163. Kopp, JB., Nelson, GW., Sampath, K., Johnson, RC., Genovese, G., An P, et al. APOL1 genetic variants in focal segmental glomerulosclerosis and HIVassociated nephropathy. J Am Soc Nephrol. 2011; 22:2129-37 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 Jafar, T., Prasad, N., Agarwal, V., Mahdi, A., Gupta, A., Sharma, RK., Negi, MP., Agrawal, S. MDR-1 gene polymorphisms in steroid-responsive versus steroidresistant nephrotic syndrome in children. 2011. Nephrol Dial Transplant. 26:3968–3974 Jinks, RN., Puffenberger, EG., Baple, E., Harding, B., Crino, P., Fogo, AB., Wenger, O, et al. Recessive nephrocerebellar syndrome on the Galloway-Mowat syndrome spectrum is caused by homozygous protein-truncating mutations of WDR73. Epub. 2015;138 (Pt 8):2173-90. 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 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 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. .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 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 .Tomiyama, H., Yamashina, A. Non-invasive vascular function tests: their pathophysiological background and clinical application. Circ J. 2010; 74:24–33 Shalaby, SA., El Idrissy, HM., Safar, RA., Hussein, ST. Glucocorticoid receptors and the pattern of steroid response in idiopathic nephrotic syndrome. Arab J. Nephrol Transplant. 2013; 5:13–17 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) Fogo, A., Lusco, M., Najafian, B., Alpers, Ch. AJKD Atlas of Renal Pathology: Congenital Nephrotic Syndrome of Finnish Am J Kidney Dis. 2015;66(3): e11-e1 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) Cawood, TJ., Bashir, M., Brady, J., et al. Urinary collagen IV and pGST: potential biomarkers for detecting localized kidney injury in diabetes-a pilot study. Am J Nephrol. 2010; 32:219–225 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 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 Bennett, MR., Piyaphanee, N., Czech, K., et al. NGAL distinguishes steroid sensitivity in idiopathic nephrotic syndrome. Pediatr Nephrol. 2012; 27:807–812. Nishida, M., Kawakatsu, H., Okumura, Y., et al. Serum and urinary neutrophil gelatinase-associated lipocalin levels in children with chronic renal diseases. Pediatr Int. 2010; 52:563–568 Hodson, EM., Craig, JC. Corticosteroid therapy for steroid sensitive nephrotic syndrome in children: dose or duration? J Am Soc Nephrol. 2013; 24:7–9 Hahn, D., Hodson, E., Willis, N., Craig, J. Corticosteroid therapy for nephrotic síndrome in children, The cocrhane Library, Cochrane Kidney and Transplant Group Cochrane Database of Systematic Reviews. 2015;3. Husen, M., Kemper, M. New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol. 2011; 26:881–892 .Buescher, AK., Kranz, B., Buscher, R., Hildebrandt, F., Dworniczak, B., Pennekamp, P., Kuwertz-Broking, E., Wingen, AM., John, U., Kemper, M., Monnens, L., Hoyer, PF., Weber, S., Konrad, M. Immunosuppression and renal outcome in congenital and pediatric steroid-resistant nephrotic syndrome. Clin J Am Soc Nephrol. 2010 Nikibakhsh, A., Mahmoodzadeh, H., Karamyyar, M., Hejazi, S., Noroozi, M., Macooie, A. Treatment of Steroid and Cyclosporine-Resistant Idiopathic Nephrotic Syndrome in Children. International Journal of Nephrology. 2011. ID 930965 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 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 Hamasaki, Y., Yoshikawa, N., Nakazato, S., Sasaki, S., Iijima, K., Nakanishi, K. et al. Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis. Pediatr Nephrol. 2013; 28:765–771 .Bensman, A., Niaudet, P. Non-immunologic mechanisms of calcineurin inhibitors explain its antiproteinuric effects in genetic glomerulopathies. Pediatr Nephrol. 2010; 25:1197–1199 .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) 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 Prytuła, A., Iijima, K., Kamei, K., et al. Rituximab in refractory nephrotic syndrome. Pediatr Nephrol. 2010;25(3):461–468 Magnasco, A., Ravani, P., Edefonti, A., Murer, L., Ghio, L., Belingheri, M. et al. Rituximab in children with resistant idiopathic nephrotic syndrome. J Am Soc Nephrol. 2012; 23:1117–1124 Pascual, J., Royuela, A., Galeano, C., Crespo, M., Zamora, J. Very early steroid withdrawal or complete avoidance for kidney transplant recipients: A systematic review. Nephrol. Dial. Transplant. 2012; 27: 825–32 Kim, J., Patnaik, N., Chorny, N., Frank, R., Infante, L., Sethna, C. Second-line immunosuppressive treatment of childhood nephrotic syndrome: A single-center experience. Nephron Extra. 2014; 4: 8–17 McCarthy, ET., Sharma, M., Savin, V. Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. Clin J Am Soc Nephrol. 2010; 5:2115–2121 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 Kopac, M., Meglic, A., Rus, RR. Partial remission of resistant nephrotic syndrome after oral galactose therapy. Ther Apher Dial. 2011; 15:269–272 Liern, M., De Reyes, V., Fayad, A., Vallejo, G. Uso del sirolimus en pacientes con síndrome nefrótico córtico-resistente primario. Nefrología. 2012;32(3):321-8 Tumlin, JA., Miller, D., Near, M., Selvaraj, S., Hennigar, R., Guasch, A. A prospective, open-label trial of sirolimus in the treatment of focal segmental glomerulosclerosis. Clin J Am Soc Nephrol. 2006; 1:109- 16 Fervenza, FC., Fitzpatrick, PM., Mertz, J., Erickson, SB., Liggett, S., Popham, S. et al. Acute rapamycin nephrotoxicity in native kidneys of patients with chronic glomerulopathies. Nephrol Dial Transplant. 2004;19(5):1288-92 Bomback AS., Radhakrishnan J. Treatment of nephrotic síndrome with adrenocorticotropic hormone (ACTH). Discov Med. 2011; 12: 91–96 Bomback AS., Tumlin JA., Baranski J., Bourdeau JE., Besarab A., Appel AS., et al. Treatment of nephrotic syndrome with adrenocorticotropic hormone (ACTH) gel. Drug Des Devel Ther. 2011; 5: 147–153 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
score 11,36867