Síndrome Nefrótico “De la teoría al manejo”

Contenido principal del artículo

Gustavo Carvajal-Barrios
Natalia Mejía
Luz Estella González Ch
Adriana Florez
Carlos M. Restrepo
Ricardo Gastelbondo

Resumen

El síndrome nefrótico (SN) es la manifestación más frecuente de las glomerulopatías primarias no inflamatorias en niños y se caracteriza proteinuria masiva con hipoalbuminemia profunda, con hiperlipidemia y edema secundarios.El SN de etiología primaria entre 15 al 30% de causa de enfermedad renal crónica terminal (ERCT) en Colombia. Dadas las diferencias en el tratamiento instaurado entre pediatras y nefrólogos pediatras, en las dosis y la duración  del primer tratamiento, la presencia de  efectos adversos asociados a la inmunosupresión, así como los resultados finales, según el centro médico y el recurso humano disponible, se ha realizado esta revisión en un intento de ampliar el conocimiento de la enfermedad, unificando los avances genéticos que han caracterizado el pronóstico así como las nuevas opciones terapéuticas en el manejo de los pacientes difíciles.


 


 


 


 

Descargas

Los datos de descargas todavía no están disponibles.

Detalles del artículo

Cómo citar
Carvajal-Barrios, G., Mejía, N., González Ch, L. E., Florez, A., Restrepo, C. M., & Gastelbondo, R. (2020). Síndrome Nefrótico: “De la teoría al manejo”. Pediatría, 52(3), 94–107. https://doi.org/10.14295/p.v52i3.137
Sección
Artículos de Revisión

Citas

Nishi S, Ubara Y, Utsunomiya Y, Okada K, Obata Y, Kai H, et al. Evidence-based clinical practice guidelines for nephrotic syndrome 2014. Clin Exp Nephrol. 2016 jun.;20(3):342-370.

Cattran DC, Feehally J, Cook HT, Liu ZH, Fervenza FC, Mezzano SA, et al. Kidney disease: Improving global outcomes (KDIGO) glomerulonephritis work group. KDIGO clinical practice guideline for glomerulonephritis. Kidney International Supplements. 2012 jun.;2(2):139-274. doi:10.1038/kisup.2012.9.

Lombel RM, Gipson DS, Hodson EM. Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol. 2013;28:415-426.

Chopra B, Tomas L. Nephrotic Syndrome. Hosp Med Clin. 2014;3(2):e245-e254.

Nishi S, Ubara Y, Utsunomiya Y, Okada K, Obata Y, Kai H, et al. Evidence-based clinical practice guidelines for nephrotic syndrome 2014. Clin Exp Nephrol. 2016;20(3):342-370.

Cattran DC, Feehally J, Cook HT, Liu ZH, Fervenza FC, Mezzano SA, et al. Kidney disease: Improving global outcomes (KDIGO) glomerulonephritis work group. KDIGO clinical practice guideline for glomerulonephritis. Kidney International Supplements. 2012;2(2):139-274. doi:10.1038/kisup.2012.9.

Lombel RM, Gipson DS, Hodson EM. Treatment of steroidsensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol. 2013;28:415-426.

Chopra B, Tomas L. Nephrotic Syndrome. Hosp Med Clin. 2014;3(2):e245-e254.

Jiang M, Xiao Z, Rong L, Xu Y, Chen L, Mo Y, et al. Twenty-eightyear review of childhood renal diseases from renal biopsy data: a single centre in China. Nephrology (Carlton). 2016;21(12):10031009.

Prasad Yadav S, Shankar Shah G, Prakash Mishra O, Baral N. Pattern of Renal Diseases in Children: A Developing Country Experience. Saudi J Kidney Dis Transpl. 2016;27(2):371-376.

Dogra S, Kaskel F. Steroid-resistant nephrotic syndrome: a persistent challenge for pediatric nephrology. Pediatr Nephrol. 2017;32(6):965-974.

Piedrahita Echeverry VM, Prada Meza MC, Vanegas Ruiz JJ, Echeverry CV, Serna Higuita LM, Serrano Gayub AK. Causas de enfermedad renal crónica en niños atendidos en el Servicio de Nefrología Pediátrica del Hospital Universitario San Vicente de Paúl, de Medellín, Colombia, entre 1960 y 2010. Iatreia. 2011;24(4):347-352.

Gastelbondo Amaya R, Pombo L, Bejarano R, Diago J. Etiología y estado actual de la insuficiencia renal crónica (IRC) en pediatría. Rev Col Ped. 1998;33(1):9-13.

El Bakkali L, Rodrigues Pereira R, Kuik DJ, Ket JCF, Van Wijk JAE. Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literatura. Pediatr Nephrol. 2011;26(8):1241-1246.

Ishikura K, Matsumoto S, Sako M, Tsuruga K, Nakanishi K, Kamei K, et al. Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy. Clin Exp Nephrol. 2015;19(1):6-33.

McKinney PA, Feltbower RG, Brocklebank JT, Fitzpatrick MM. Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK. Pediatr Nephrol 2001;16(12):10401044.

Hahn D, Hodson EM, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database of Systematic Reviews. 2015;(3): CD001533. doi:10.1002/14651858. CD001533.pub5.

Atehortúa Baena PS, Piedrahíta Echeverry V, Vanegas Ruiz JJ, Vélez Echeverri C, Serna Higuita LM, Serrano Gayubo AK, 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.

Pasini A, Aceto G, Ammenti A, Ardissino G, Azzolina V, Bettinelli A, et al. Best practice guidelines for idiopathic nephrotic syndrome: recommendations versus reality. Pediatr Nephrol. 2015;30(1):91-101.

Samuel S, Scott S, Morgan C, Dart A, Mammen C, Parekh R. The Canadian Childhood Nephrotic Syndrome (CHILDNEPH) project: overview of design and methods. Can J Kid H Dis. 2014;1:1.

Wang CS, Greenbaum LA. Nephrotic Syndrome. Pediatr Clin N Am. 2019 febr.;66(1):73-85.

Joshi S, Andersen R, Jespersen B, Rittig S. Genetics of steroidresistant nephrotic syndrome: a review of mutations spectrum and suggested approach for genetic testing. Acta Paediatr. 2013;102(9):844-56.

Greenbaum L; Benndorf R; Smoyer W. Childhood nephrotic syndrome--current and future therapies. Nat. Rev. Nephrol. 2012;8(8):445-458.

Brown EJ, Pollak MR, Barua M. Genetic testing for nephrotic syndrome and FSGS in the era of next-generation sequencing. Kidney Int. 2014;85(5):1030-8. doi:10.1038/ki.2014.48.

Lipska BS, Iatropoulos P, Maranta R, Caridi G, Ozaltin F, Anarat A. Genetic screening in adolescents with steroid-resistant nephrotic syndrome. Kidney Int. 2013;84(1):206-13. doi:10.1038/ ki.2013.93.

Rheault MN. Nephrotic and Nephritic Syndrome in the Newborn. Clin Perinatol. 2014;41(3):605-18. doi:10.1016/j. clp.2014.05.009.

National Center for Biotechnology Information [Internet]. Genetic Testing Registry (GTR) [citado 2017 nov.]. Disponible en http://www.ncbi.nlm.nih.gov/sites/GeneTests/.

Jhaveri KD, Shah HH, Calderon K, Campenot ES, Radhakrishnan J. Glomerular diseases seen with cancer and chemotherapy: a narrative review. Kidney Int. 2013;84(1):34-44.

Ruebner RL, Copelovitch L, Evageliou NF, Denburg MR, Belasco JB, Kaplan BS. Nephrotic syndrome associated with tyrosine kinase inhibitors for pediatric malignancy: case series and review of the literature. Pediatr Nephrol. 2014;29(5):863-869.

Ganguli A, Sawinski D, Berns JS. Kidney diseases associated with haematological cancers. Nat. Rev Nephrol. 2015;11(8):478490.

Woodrick R, Ruderman EM. Safety of biologic therapy in rheumatoid arthritis. Nat. Rev. Rheumatol. 2011;7(11):639-652.

Stoll ML, Cron RQ. Treatment of Juvenile Idiopathic Arthritis in the Biologic Age. Rheum Dis Clin North Am. 2013;39(4):751-66. doi:10.1016/j.rdc.2013.05.004.

Kataria A, Trasande L, Trachtman H. The effects of environmental chemicals on renal function. Nat. Rev. Nephrol. Nat Rev Nephrol. 2015;11(10):610-25.

Grahammer F, Benzing T, Huber TB. New insights into mechanisms of glomerular injury and repair from the 10th International Podocyte Conference 2014. Kidney Int. 2015;87(5):885-93. doi: 10.1038/ki.2015.5.

Welsh G, Saleem M. The podocyte cytoskeleton—key to a functioning glomerulus in health and disease. Nat Rev Nephrol. 2011;8(1):14-21. doi:10.1038/nrneph.2011.151.

Grahammer F, Schell C, Huber TB. The podocyte slit diaphragmfrom a thin grey line to a complex signalling hub. Nat. Rev. Nephrol. 2013;9(10):587-598.

Perico L, Conti S, Benigni A, Remuzzi G. Podocyte-actyne dynamics in health and disease. Nat Rev Nephrol. 2016;12(11):692-710.

Vivarelli M, Masella L, Rugiero B, Emma F. Minimal change disease. Clin J Am Soc Nephrol. 2017;12(2):332-345.

Bertelli R, Bonanni A, Di Donato A, Cioni M, Ravani P, Ghiggeri GM. Regulatory T cells and minimal change nephropathy: in the midst of a complex network. Clin Exp Immunol. 2016 ;183(2):166-74.

D’Agati VD., Alster JM, Jennette JC, Thomas DB, Pullman J, Savino DA, et al. Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes. Clin J Am Soc Nephrol. 2013;8(3):399-406. doi:10.2215/CJN.06100612.

Li F, Zheng C, Zhong Y, Zeng C, Xu F, Yin R, et al. Relationship between Serum Soluble Urokinase Plasminogen Activator Receptor Level and Steroid Responsiveness in FSGS. Clin J Am Soc Nephrol. 2014;9(11):1903-1911.

Couser W. Primary Membranous Nephropathy. Clin J Am Soc Nephrol. 2017;12(6):1-15.

Ayalon R, Beck LH Jr. Membranous nephropathy: not just a disease for adults. Pediatr Nephrol. 2015;30(1):31-9.

Ruggenenti P, Fervenza F, Remuzzi G. Treatment of membranous nephropathy: time for a paradigm shift. Nat Rev Nephrol. 2017;13(9):563-579.

Alchi B, Jayne D. Membranoproliferative glomerulonephritis. Pediatr Nephrol. 2010;25(8):1409-1418.

González LE, Gastelbondo AR, Meza AL. Síndrome Nefrótico en: Pediatría práctica. Bogotá: Universidad El Bosque; 2014.

Trimarchi H, Duboscq C. Proteinuria: A Cross Road Where the Complement and the Plasminogen-plasmin Systems Meet. J Integr Nephrol Androl. 2016;3(2):37-47.

Trimarchi H. Plasmin, urokinase plasminogen activator receptor and amiloride in the nephrotic syndrome. Argentina: Hospital Británico de Buenos Aires; 2015.

Pasini A, Benetti E, Conti G, Ghio L, Lepore M, Massella L, et al. The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I - Diagnosis and treatment of the first episode and the first relapse. Ital J Pediatr. 2017;43(1):41. doi:10.1186/ s13052-017-0356-x.

Wei CC, Yu IW, Lin HW, Tsai AC. Occurrence of infection among children with nephrotic syndrome during hospitalizations. Nephrology (Carlton). 2012;17(8):681-8.

Eneman B, Levtchenko E, Heuvel VBD, Van Geet C, Freson K. Platelet abnormalities in nephrotic syndrome. Pediatr Nephrol. 2016;31(8):1267-79.

Alshami A, Roshan A, Catapang M, Jöbsis JJ, Kwok T, Polderman N, et al. Indications for kidney biopsy in idiopathic childhood nephrotic syndrome. Pediatr Nephrol 2017; 32(10):1897-1905.

Larkins N, Kim S, Craig J, Hodson E. Steroid-sensitive nephrotic syndrome: an evidence-based update of immunosuppressive treatment in children. Arch Dis Child. 2016;101(4):404-8. doi:10.1136/archdischild-2015-308924.

Hansen KE, Kleker B, Safdar N, Bartels CM. A systematic review and meta-analysis of glucocorticoid-induced osteoporosis in children. Semin Arthritis Rheum. 2014;44(1):47-54.

Tsampalieros A, Gupta P, Denburg ML, Shults J, Zemel BS, Mostoufi-Moab S, et al. Glucocorticoid Effects on Changes in Bone Mineral Density and Cortical Structure in Childhood Nephrotic Syndrome. J Bone Miner Res. 2013;28(3):480-488.

El-Mashad GM, El-Hawy MA, El-Hefnawy SM, Mohamed SM. Bone mineral density in children with idiopathic nephrotic síndrome. J Pediatr (Rio J). 2017;93(2):142-147.

Banerjee S, Basu S, Sen A, Sengupta J. The effect of vitamin D and calcium supplementation in pediatric steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2017;32(11):2063-2070.

Fu HD, Qian GL, Jiang ZG. Comparison of second-line immunosuppressants for childhood refractory nephrotic

syndrome: a systematic review and network meta-analysis. J Investig Med. 2016;65(1):1-7.

Abeyagunawardena AS, Karunadasa U, Jayaweera H, Thalgahagoda S, Tennakoon S, Abeyagunawardena S. Efficacy of higher-dose levamisole in maintaining remission in steroiddependant nephrotic síndrome. Pediatr Nephrol. 2017;32(8):1363-1367.

Taner Elmas A, lmaz Tabel Y, Nalbantoglu Elmas Ö. Short- and long-term efficacy of levamisole in children with steroidsensitive nephrotic syndrome. Int Urol Nephrol. 2013;45(4):10471055.

Cattran DC, Alexopoulos E, Heering P, Hoyer PF, Johnston A, Meyrier A. Cyclosporin in idiopathic glomerular disease associated with the nephrotic syndrome: Workshop recommendations. Kidney Int. 2007;72(12):1429-47.

Hamasaki Y, Komaki F, Ishikura K, Hamada R, Sakai T, Hataya H, et al. Nephrotoxicity in children with frequently relapsing nephrotic syndrome receiving long-term cyclosporine treatment. Pediatr Nephrol. 2017;32(8):1383-1390.

Jahan A, Prabha R, Chaturvedi S, Mathew B, Fleming D, Agarwal I. Clinical efficacy and pharmacokinetics of tacrolimus in children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2015;30(11):1961-7.

Querfeld U, Weber LT. Mycophenolate mofetil for sustained remission in nephrotic syndrome. Pediatr Nephrol. 2018;33(12):2253-2265.

Tullus K. Marks SD. Indications for use and safety of rituximab in childhood renal diseases. Pediatr Nephrol. 2013;28(7):10011009.

Takei T, Nitta K. Rituximab and minimal change nephrotic syndrome: a therapeutic option. Clin Exp Nephrol. 2011;15(5):641-647.

Iijima K, Sako M, Nozu K. Rituximab for nephrotic syndrome in children. Clin Exp Nephrol. 2017;21(2):193-202.

Yu CC, Fornoni A, Weins A, Hakroush S, Maiguel D, Sageshima J, et al. Abatacept in B7-1-positive proteinuric kidney disease. N Engl J Med. 2013;369(25):2416-2423.

Trachtman H, Gipson DS, Somers M, Spino C, Adler S, Holzman L. Randomized Clinical Trial Design to Assess Abatacept in Resistant Nephrotic Syndrome. Kidney Int Rep. 2017;3(1):115121. doi:10.1016/j.ekir.2017.08.013.

Trimarchi H. Abatacept and Glomerular Diseases: The Open Road for the Second Signal as a New Target is Settled Down. Recent Pat Endocr Metab Immune Drug Discov. 2015;9(1):2-14.

Trachtman H, Vento S, Herreshoff E, Radeva M, Gassman J, Stein DT. Efficacy of galactose and adalimumab in patients with resistant focal segmental glomerulosclerosis: report of the Font clinical trial group. BMC Nephrology. 2015;16:111.

Wang CS, Liverman RS, Garro R, George RP, Glumova A, Karp A, et al. Ofatumumab for the treatment of childhood nephrotic syndrome. Pediatr Nephrol. 2017;32(5):835-841.

Ravani P, Bonanni A, Ghiggeri GM. Randomised controlled trial comparing ofatumumab to rituximab in children with steroiddependent and calcineurin inhibitor-dependent idiopathic nephrotic syndrome: study protocol. BMJ Open. 2017;7(3):e013319.

Basu B. Ofatumumab for Rituximab-Resistant Nephrotic Syndrome. N Engl J Med. 2014;370(13):1268-70.

Bonanni A, Bertelli E, Moscatelli A, Lampugnani E, Bodria M, Ravani P, et al. Ofatumumab-associated acute respiratory manifestations: clinical characteristics and treatment. Br J Clin Pharmacol. 2016;82(4):1146-1148.

Kaku Y, Ohtsuka Y, Komatsu Y, Ohta T, Nagai T, Kaito H, et al. Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: general therapy. Clin Exp Nephrol. 2015;19(1):34-53.

Geier P, Strojil J, Kutrova K, Potomkova J, Feber J. Diuretics for treating oedema in nephrotic syndrome. Cochrane Database of Systematic Reviews. 2010;9: CD008665. doi:10.1002/14651858. CD008665.

Kister de Toledo LA, Beisl Noblat AC, Do Nascimento HFI, Araújo L, Noblat CB. Economic evaluation of human albumin use in patients with nephrotic syndrome in four Brazilian public hospitals: pharmacoeconomic study. Sao Paulo Med J. 2017; 135(2):92-9.

Tanzi M, Gardner M, Megellas M, Lucio S, Restino M. Evaluation of the appropriate use of albumin in adult and pediatric patients. Am J Health Syst Pharm. 2003;1;60(13):1330-5.

Pradel V, Tardieu S, Micallef J, Signoret A, Villano P, Gauthier L, et al. Use of albumin in three French university hospitals: is prescription monitoring still useful in 2004?. Pharmacoepidemiol Drug Saf. 2007;16(1):79-85.

Lane ER, Hsu EK, Murray KF. Management of ascites in children. Expert Rev Gastroenterol Hepatol. 2015;9(10):1281-92.

Hughes HK, Kahl L. Manual Harriet Lane de pediatría. 20.a ed. Barcelona: Elsevier; 2015.

Silvey M, Brandão LR. Risk Factors, Prophylaxis, and Treatment of venous Thromboembolism in Congenital Heart Disease Patients. Front. Pediatr. 2017;5:146.

Dasi Carpio M. Trastornos trombóticos de la coagulación: trombosis y tratamiento antitrombótico en niños. An Pediatr Contin. 2007;5(4):189-96 189.

Giglia TM, Witmer C, Procaccini DE, Byrnes JW. Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care Anticoagulation and Thrombolysis. Pediatr Crit Care Med. 2016;17(3 Suppl 1):S77-88.

Monagle P, Chan AKC, Goldenberg NA, Ichord RN, Journeycake JM, Nowak-Göttl U, et al. Antithrombotic therapy in neonates and children: antithrombotic therapy and prevention of thrombosis 9th ed: American College of Chest physicians evidence-based clinical practice guidelines. Chest. 2012;141(2 Suppl):e737S-801S.

Rajpurkar M, Biss T, Amankwah EK, Martinez D, Williams S, Van Ommen CH, et al. Pulmonary embolism and in situ pulmonary artery thrombosis in paediatrics. A systematic review. Thromb Haemost. 2017;117(6):1199-1207.

Ramiz S, Rajpurkar M. Pulmonary Embolism in Children. Pediatr Clin N Am. 2018;65(3),495-507.

Raffini L. Thrombolysis for intravascular thrombosis in neonates and children. Curr Opin Pediat. 2009;21(1):9-14.

Wanner C, Tonelli M, Kidney Disease: Improving Global Outcomes Lipid Guideline Development Work Group Members. KDIGO Clinical Practice Guideline for Lipid Management in CKD: summary of recommendation statements and clinical approach to the patient. Kidney Int. 2014;85(6):1303-9. doi:10.1038/ki.2014.31.

Tullus K. Dyslipidemia in children with CKD: should we treat with statins? Pediatr Nephrol. 2012;27(3):357-362.

Fan J, Li Z, Wu T, Chen H. Lipid-lowering agents for nephrotic syndrome. Cochrane Database of Systematic Reviews. 2005;3:CD005425. doi:10.1002/14651858.CD005425.

Chauhan A, Paunikar P. Update on pediatric hyperlipidemia. Curr Opin Pediatr. 2014;26(2):252-258.

Lamaida N, Capuano E, PintoL, Capuano E, Capuano R, Capuano V. The safety of statins in children. Acta Paediatr. 2013;102(9):85762.

Belay B, Belamarich PF, Tom-Revzon C, PharmD BS. The Use of Statins in Pediatrics: Knowledge Base, Limitations, and Future Directions. Pediatrics. 2007;119(2):370-80.

Braamskamp MJ, Wijburg FA, Wiegman A. Drug Therapy of Hypercholesterolaemia in Children and Adolescents. Drugs. 2012;72(6):759-72.

Nakamura A, Niimi R, Kurosaki K, Yanagawa Y. Factors influencing cardiovascular risk following termination of glucocorticoid therapy for nephrotic syndrome. Clin Exp Nephrol. 2010;14(5):457-462.

Saland J, Pierce CB, Mitsnefes MM, Flynn JT, Goebel J, Kupferman JC, et al. Dyslipidemia in children with chronic kidney disease. Kidney Int. 2010;78(11):1154-63. doi:10.1038/ki.2010.311.

Kogon AJ, Pierce CB, Cox C, Brady TM, Mitsnefes MM, Warady BA. Nephrotic-range proteinuria is strongly associated with poor blood pressure control in pediatric chronic kidney disease. Kidney Int. 2014;85(4):938-44. doi:10.1038/ki.2013.352.

Sharma B, Saha A, Dubey NK, Kapoor K, Anubhuti, Batra VV. Endothelial dysfuntion in children with idiopathic nephrotic syndrome. Atherosclerosis. 2014;233(2):704-6. doi:10.1016/j. atherosclerosis.

Soto-Rivera CL, Agus MS, Sawyer JE, Macrae DJ. Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care Chronic Heart Failure. Pediatr Crit Care Med. 2016;17(3 Suppl 1):S59-68.

Rubin LG, Levin MJ, Ljungman P, Davies EG, Avery R,T omblyn M, et al. 2013 IDSA Clinical Practice Guideline for Vaccination of the Immunocompromised Host. Clin Infect Dis. 2014;58(3):30918. doi:10.1093/cid/cit816.

Chi C, Patel P, Pilishvili T, Moore M, Murphy T, Strikas R. Guidelines for vaccinating dialysis patients and patients with chronic kidney disease. U.S: Department of Health & Human Services; 2012.

Mellado Peña MJ, Moreno-Pérez D, Ruíz Contreras J, Hernández-Sampelayo T, Navarro Gómez ML. Documento de consenso de la Sociedad Española de Infectología Pediátrica y el Comité Asesor de Vacunas de la Asociación Española de Pediatría para la vacunación en inmunodeprimidos. Anales de Pediatría(Barc). 2012;75(6):413.e1-413.e22.

Artículos más leídos del mismo autor/a

Artículos similares

1 2 > >> 

También puede {advancedSearchLink} para este artículo.