Nephrotic syndrome in infants and children: pathophysiology and management

References

• Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guideline for Glomerulonephritis. Kidney Int. 2012;2:139–274.
   Web of Science ®Google Scholar
• Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int. 1978;13:159–165.
   PubMed Web of Science ®Google Scholar
• D’Agati VD, Kaskel FJ, Falk RJ. Focal segmental glomerulosclerosis. N Engl J Med. 2011;365:2398–2411.10.1056/NEJMra1106556
   PubMed Web of Science ®Google Scholar
• Maas RJ, Deegens JK, Smeets B, et al. Minimal change disease and idiopathic FSGS: manifestations of the same disease. Nat Rev Nephrol. 2016;12:768–776.10.1038/nrneph.2016.147
   PubMed Web of Science ®Google Scholar
• Primary nephrotic syndrome in children: clinical significance of histopathologic variants of minimal change and of diffuse mesangial hypercellularity. A Report of the International Study of Kidney Disease in Children. Kidney Int. 1981;20:765–771.
   PubMed Web of Science ®Google Scholar
• Koskimies O, Vilska J, Rapola J, et al. Long-term outcome of primary nephrotic syndrome. Arch Dis Child. 1982;57:544–548.10.1136/adc.57.7.544
   PubMed Web of Science ®Google Scholar
• Tarshish P, Tobin JN, Bernstein J, et al. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol. 1997;8:769–776.
   PubMed Web of Science ®Google Scholar
• Lombel RM, Gipson DS, Hodson EM, et al. Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol. 2013;28:415–426.10.1007/s00467-012-2310-x
   PubMed Web of Science ®Google Scholar
• Banh TH, Hussain-Shamsy N, Patel V, et al. Ethnic differences in incidence and outcomes of childhood nephrotic ayndrome. Clin J Am Soc Nephrol. 2016;11:1760–1768.10.2215/CJN.00380116
   PubMed Web of Science ®Google Scholar
• Rüth EM, Kemper MJ, Leumann EP, et al. Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr. 2005;147:202–207.10.1016/j.jpeds.2005.03.050
   PubMed Web of Science ®Google Scholar
• Fakhouri F, Bocquet N, Taupin P, et al. Steroid-sensitive nephrotic syndrome: From childhood to adulthood. Am J Kidney Dis. 2003;41:550–557.10.1053/ajkd.2003.50116
   PubMed Web of Science ®Google Scholar
• Kyrieleis HA, Levtchenko EN, Wetzels JF. Long-term outcome after cyclophosphamide treatment in children with steroid-dependent and frequently relapsing minimal change nephrotic syndrome. Am J Kidney Dis. 2007;49:592–597.10.1053/j.ajkd.2007.02.270
   PubMed Web of Science ®Google Scholar
• Kyrieleis HA, Lowik MM, Pronk I, et al. Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children. Clin J Am Soc Nephrol. 2009;4:1593–1600.10.2215/CJN.05691108
   PubMed Web of Science ®Google Scholar
• Chanchlani R, Parekh RS. Ethnic differences in childhood nephrotic syndrome. Front Pediatr. 2016;4:39.
   PubMed Web of Science ®Google Scholar
• McKinney PA, Feltbower RG, Brocklebank JT, et al. Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK. Pediatr Nephrol. 2001;16:1040–1044.10.1007/s004670100021
   PubMed Web of Science ®Google Scholar
• Feehally J, Burden AC, Mayberry JF, et al. Disease variations in Asians in Leicester. QJ Med. 1993;86:263–269.
   PubMedGoogle Scholar
• Feehally J, Kendell NP, Swift PG, et al. High incidence of minimal change nephrotic syndrome in Asians. Arch Dis Child. 1985;60:1018–1020.10.1136/adc.60.11.1018
   PubMed Web of Science ®Google Scholar
• Srivastava T, Simon SD, Alon US. High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol. 1999;13:13–18.10.1007/s004670050555
   PubMed Web of Science ®Google Scholar
• Schlesinger ER, Sultz HA, Mosher WE, et al. The nephrotic syndrome. Its incidence and implications for the community. Am J Dis Child. 1968;116:623–632.10.1001/archpedi.1968.02100020627009
   PubMedGoogle Scholar
• Ingulli E, Tejani A. Racial differences in the incidence and renal outcome of idiopathic focal segmental glomerulosclerosis in children. Pediatr Nephrol. 1991;5:393–397.10.1007/BF01453661
   PubMed Web of Science ®Google Scholar
• Boyer O, Moulder JK, Somers MJ. Focal and segmental glomerulosclerosis in children: a longitudinal assessment. Pediatr Nephrol. 2007;22:1159–1166.10.1007/s00467-007-0493-3
   PubMed Web of Science ®Google Scholar
• Warady BA, Chadha V. Chronic kidney disease in children: the global perspective. Pediatr Nephrol. 2007;22:1999–2009.10.1007/s00467-006-0410-1
   PubMed Web of Science ®Google Scholar
• Sorof JM, Hawkins EP, Brewer ED, et al. Age and ethnicity affect the risk and outcome of focal segmental glomerulosclerosis. Pediatr Nephrol. 1998;12:764–768.10.1007/s004670050542
   PubMed Web of Science ®Google Scholar
• Bonilla-Felix M, Parra C, Dajani T, et al. Changing patterns in the histopathology of idiopathic nephrotic syndrome in children. Kidney Int. 1999;55:1885–1890.10.1046/j.1523-1755.1999.00408.x
   PubMed Web of Science ®Google Scholar
• Mubarak M, Kazi JI, Shakeel S, et al. The spectrum of histopathological lesions in children presenting with steroid-resistant nephrotic syndrome at a single center in Pakistan. Sci World J. 2012;2012:681802.
   Google Scholar
• Andreoli SP. Racial and ethnic differences in the incidence and progression of focal segmental glomerulosclerosis in children. Adv Renal Replace Ther. 2004;11:105–109.10.1053/j.arrt.2003.10.015
   PubMedGoogle Scholar
• Trautmann A, Bodria M, Ozaltin F, et al. Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the podonet registry cohort. Clin J Am Soc Nephrol. 2015;10:592–600.10.2215/CJN.06260614
   PubMed Web of Science ®Google Scholar
• Gadegbeku CA, Gipson DS, Holzman LB, et al. Design of the nephrotic syndrome study network (NEPTUNE) to evaluate primary glomerular nephropathy by a multidisciplinary approach. Kidney Int. 2013;83:749–756.10.1038/ki.2012.428
   PubMed Web of Science ®Google Scholar
• Samuel S, Scott S, Morgan C, et al. The Canadian childhood nephrotic syndrome (CHILDNEPH) project: overview of design and methods. Can J Kidney Health Dis. 2014;1:17.10.1186/2054-3581-1-17
   PubMedGoogle Scholar
• Hussain N, Zello JA, Vasilevska-Ristovska J, et al. The rationale and design of Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT): a prospective cohort study of childhood nephrotic syndrome. BMC Nephrol. 2013;14:765.10.1186/1471-2369-14-25
   Web of Science ®Google Scholar
• Gipson DS, Troost JP, Lafayette RA, et al. Complete remission in the nephrotic syndrome study network. Clin J Am Soc Nephrol. 2016;11:81–89.10.2215/CJN.02560315
   PubMed Web of Science ®Google Scholar
• Büscher AK, Weber S. Educational paper: the podocytopathies. Eur J Pediatr. 2012;171:1151–1160.10.1007/s00431-011-1668-2
   PubMed Web of Science ®Google Scholar
• Wharram BL, Goyal M, Wiggins JE, et al. Podocyte depletion causes glomerulosclerosis: diphtheria toxin-induced podocyte depletion in rats expressing human diphtheria toxin receptor transgene. J Am Soc Nephrol. 2005;16:2941–2952.10.1681/ASN.2005010055
   PubMed Web of Science ®Google Scholar
• Akchurin O, Reidy kJ. Genetic causes of proteinuria and nephrotic syndrome: Impact on podocyte pathobiology. Pediatr Nephrol. 2015;30:221–233.10.1007/s00467-014-2753-3
   PubMed Web of Science ®Google Scholar
• van den Berg JG, Weening JJ. Role of the immune system in the pathogenesis of idiopathic nephrotic syndrome. Clin Sci (Lond). 2004;107:125–136.10.1042/CS20040095
   PubMed Web of Science ®Google Scholar
• Audard V, Larousserie F, Grimbert P, et al. Minimal change nephrotic syndrome and classical Hodgkin's lymphoma: report of 21 cases and review of the literature. Kidney Int. 2006;69:2251–2260.10.1038/sj.ki.5000341
   PubMed Web of Science ®Google Scholar
• Lin CY, Hsu HC. Histopathological and immunological studies in spontaneous remission of nephrotic syndrome after intercurrent measles infection. Nephron. 1986;42:110–115.
   PubMed Web of Science ®Google Scholar
• Reiser J, von Gersdorff G, Loos M, et al. Induction of B7-1 in podocytes is associated with nephrotic syndrome. J Clin Invest. 2004;113:1390–1397.10.1172/JCI20402
   PubMed Web of Science ®Google Scholar
• Furie R, Nicholls K, Cheng TT, et al. Efficacy and safety of abatacept in lupus nephritis: a twelve-month, randomized, double-blind study. Arthritis Rheumatol. 2014;66:379–389.10.1002/art.v66.2
   PubMed Web of Science ®Google Scholar
• Yu CC, Fornoni A, Weins A, et al. Abatacept in B7-1–positive proteinuric kidney disease. N Engl J Med. 2013;369:2416–2423.10.1056/NEJMoa1304572
   PubMed Web of Science ®Google Scholar
• Maas RJ, Deegens JK, Wetzels JF. Permeability factors in idiopathic nephrotic syndrome: historical perspectives and lessons for the future. Nephrol Dial Transplant. 2014;29:2207–2216.10.1093/ndt/gfu355
   PubMed Web of Science ®Google Scholar
• Wada T, Nangaku M. A circulating permeability factor in focal segmental glomerulosclerosis: the hunt continues. Clin Kidney J. 2015;8:708–715.10.1093/ckj/sfv090
   PubMed Web of Science ®Google Scholar
• Deegens JK, Andresdottir MB, Croockewit S, et al. Plasma exchange improves graft survival in patients with recurrent focal glomerulosclerosis after renal transplant. Transpl Int. 2004;17:151–157.10.1111/tri.2004.17.issue-3
   PubMed Web of Science ®Google Scholar
• Zimmerman SW. Increased urinary protein excretion in the rat produced by serum from a patient with recurrent focal glomerular sclerosis after renal transplantation. Clin Nephrol. 1984;22:32–38.
   PubMed Web of Science ®Google Scholar
• Savin VJ, Sharma R, Sharma M, et al. Circulating factor associated with increased glomerular permeability to albumin in recurrent focal segmental glomerulosclerosis. N Engl J Med. 1996;334:878–883.10.1056/NEJM199604043341402
   PubMed Web of Science ®Google Scholar
• Kemper MJ, Wolf G, Müller-Wiefel DE. Transmission of glomerular permeability factor from a mother to her child. N Engl J Med. 2001;344:386–387.10.1056/NEJM200102013440517
   PubMed Web of Science ®Google Scholar
• Gallon L, Leventhal J, Skaro A, et al. Resolution of recurrent focal segmental glomerulosclerosis after retransplantation. N Engl J Med. 2012;366:1648–1649.10.1056/NEJMc1202500
   PubMed Web of Science ®Google Scholar
• Tomizawa S, Maruyama K, Nagasawa N, et al. Studies of vascular permeability factor derived from T lymphocytes and inhibitory effect of plasma on its production in minimal change nephrotic syndrome. Nephron. 1985;41:157–160.
   PubMed Web of Science ®Google Scholar
• Bakker WW, van Dael CM, Pierik LJ, et al. Altered activity of plasma hemopexin in patients with minimal change disease in relapse. Pediatr Nephrol. 2005;20:1410–1415.10.1007/s00467-005-1936-3
   PubMed Web of Science ®Google Scholar
• Lennon R, Singh A, Welsh GI, et al. Hemopexin induces nephrin-dependent reorganization of the actin cytoskeleton in podocytes. J Am Soc Nephrol. 2008;19:2140–2149.10.1681/ASN.2007080940
   PubMed Web of Science ®Google Scholar
• Savin VJ, McCarthy ET, Sharma R, et al. Galactose binds to focal segmental glomerulosclerosis permeability factor and inhibits its activity. Transl Res. 2008;151:288–292.10.1016/j.trsl.2008.04.001
   PubMed Web of Science ®Google Scholar
• McCarthy ET, Sharma M, Savin VJ. Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. Clin J Am Soc Nephrol. 2010;5:2115–2121.10.2215/CJN.03800609
   PubMed Web of Science ®Google Scholar
• Wei C, El Hindi S, Li J, et al. Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis. Nat Med. 2011;17:952–960.10.1038/nm.2411
   PubMed Web of Science ®Google Scholar
• Maas RJ, Wetzels JF, Deegens JK. Serum-soluble urokinase receptor concentration in primary FSGS. Kidney Int. 2012;81:1043–1044.10.1038/ki.2012.32
   PubMed Web of Science ®Google Scholar
• Meijers B, Maas RJ, Sprangers B, et al. The soluble urokinase receptor is not a clinical marker for focal segmental glomerulosclerosis. Kidney Int. 2014;85:636–640.10.1038/ki.2013.505
   PubMed Web of Science ®Google Scholar
• Sinha A, Bajpai J, Saini S, et al. Serum-soluble urokinase receptor levels do not distinguish focal segmental glomerulosclerosis from other causes of nephrotic syndrome in children. Kidney Int. 2014;85:649–658.10.1038/ki.2013.546
   PubMed Web of Science ®Google Scholar
• Maas RJ, Wetzels JF, Deegens JK. Serum suPAR concentrations in patients with focal segmental glomerulosclerosis with end-stage renal disease. Kidney Int. 2014;85:711.10.1038/ki.2013.513
   PubMed Web of Science ®Google Scholar
• Vivarelli M, Massella L, Ruggiero B, et al. Minimal change disease. Clin J Am Soc Nephrol. 2017;12:332–345.10.2215/CJN.05000516
   PubMed Web of Science ®Google Scholar
• Latta K, von Schnakenburg C, Ehrich JH. A meta-analysis of cytotoxic treatment for frequently relapsing nephrotic syndrome in children. Pediatr Nephrol. 2001;16:271–282.10.1007/s004670000523
   PubMed Web of Science ®Google Scholar
• Rheault MN, Zhang L, Selewski DT, et al. AKI in children hospitalized with nephrotic syndrome. Clin J Am Soc Nephrol. 2015;10:2110–2118.10.2215/CJN.06620615
   PubMed Web of Science ®Google Scholar
• Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol. 2012;7:513–520.10.2215/CJN.10131011
   PubMed Web of Science ®Google Scholar
• Kerlin BA, Haworth K, Smoyer WE. Venous thromboembolism in pediatric nephrotic syndrome. Pediatric Nephrol. 2014;29:989–997.10.1007/s00467-013-2525-5
   PubMed Web of Science ®Google Scholar
• Vaziri ND. HDL abnormalities in nephrotic syndrome and chronic kidney disease. Nat Rev Nephrol. 2015;12:37–47.10.1038/nrneph.2015.180
   PubMed Web of Science ®Google Scholar
• Kong X, Yuan H, Fan J, et al. Lipid-lowering agents for nephrotic syndrome. Cochrane Database Syst Rev. 2013;12:CD005425.
   Google Scholar
• Silva JM, Oliveira EA, Marino VS, et al. Premature acute myocardial infarction in a child with nephrotic syndrome. Pediatr Nephrol. 2002;17:169–172.10.1007/s00467-001-0793-y
   PubMed Web of Science ®Google Scholar
• Teoh CW, Robinson LA, Noone D. Perspectives on edema in childhood nephrotic syndrome. Am J Physiol Renal Physiol. 2015;309:F575–F582.10.1152/ajprenal.00229.2015
   PubMed Web of Science ®Google Scholar
• Kapur G, Valentini RP, Imam AA, et al. Treatment of severe edema in children with nephrotic syndrome with diuretics alone – a prospective study. Clin J Am Soc Nephrol. 2009;4:907–913.10.2215/CJN.04390808
   PubMed Web of Science ®Google Scholar
• Kleta R, Bockenhauer D. Bartter syndromes and other salt-losing tubulopathies. Nephron Physiol. 2006;104:p73–p80.10.1159/000094001
   PubMedGoogle Scholar
• Carattino MD, Hughey RP, Kleyman TR. Proteolytic processing of the epithelial sodium channel gamma subunit has a dominant role in channel activation. J Biol Chem. 2008;283:25290–25295.10.1074/jbc.M803931200
   PubMed Web of Science ®Google Scholar
• Svenningsen P, Bistrup C, Friis UG, et al. Plasmin in nephrotic urine activates the epithelial sodium channel. J Am Soc Nephrol. 2009;20:299–310.10.1681/ASN.2008040364
   PubMed Web of Science ®Google Scholar
• Deschênes G, Guigonis V, Doucet A. Molecular mechanism of edema formation in nephrotic syndrome. Arch Pediatr. 2004;11:1084–1094.10.1016/j.arcped.2004.03.029
   PubMed Web of Science ®Google Scholar
• Jackson AA. Albumin in nephrotic syndrome and oedematous malnutrition. Paediatr Int Child Health. 2015;35:77–80.10.1179/2046904715Z.000000000249
   PubMed Web of Science ®Google Scholar
• Coulthard MG. Oedema in kwashiorkor is caused by hypoalbuminaemia. Paediatr Int Child Health. 2015;35:83–89.10.1179/2046905514Y.0000000154
   PubMed Web of Science ®Google Scholar
• Golden MH. Nutritional and other types of oedema, albumin, complex carbohydrates and the interstitium – a response to Malcolm Coulthard’s hypothesis: Oedema in kwashiorkor is caused by hypo-albuminaemia. Paediatr Int Child Health. 2015;35:90–109.10.1179/2046905515Y.0000000010
   PubMed Web of Science ®Google Scholar
• Minimal change nephrotic syndrome in children: deaths during the first 5 to 15 years’ observation. Report of the International Study of Kidney Disease in Children. Pediatrics. 1984;73:497–501.
   PubMed Web of Science ®Google Scholar
• Hahn D, Hodson EM, Willis NS, et al. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev. 2015;3:CD001533.
   Google Scholar
• Hyams JS, Carey DE. Corticosteroids and growth. J Pediatr. 1988;113:249–254.10.1016/S0022-3476(88)80260-9
   PubMed Web of Science ®Google Scholar
• Ng JS, Wong W, Law RW, et al. Ocular complications of paediatric patients with nephrotic syndrome. Clin Exp Ophthalmol. 2001;29:239–243.10.1046/j.1442-9071.2001.00426.x
   PubMed Web of Science ®Google Scholar
• Mishra OP, Basu B, Upadhyay SK, et al. Behavioural abnormalities in children with nephrotic syndrome. Nephrol Dial Transplant. 2010;25:2537–2541.10.1093/ndt/gfq097
   PubMed Web of Science ®Google Scholar
• Hodson EM, Hahn D, Craig JC. Corticosteroids for the initial episode of steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2015;30:1043–1046.10.1007/s00467-015-3106-6
   PubMed Web of Science ®Google Scholar
• Teeninga N, Kist-van Holthe JE, van Rijswijk N, et al. Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome. J Am Soc Nephrol. 2013;24:149–159.10.1681/ASN.2012070646
   PubMed Web of Science ®Google Scholar
• Yoshikawa N, Nakanishi K, Sako M, et al. A multicenter randomized trial indicates initial prednisolone treatment for childhood nephrotic syndrome for two months is not inferior to six-month treatment. Kidney Int. 2015;87:225–232.10.1038/ki.2014.260
   PubMed Web of Science ®Google Scholar
• Sinha A, Saha A, Kumar M, et al. Extending initial prednisolone treatment in a randomized control trial from 3 to 6 months did not significantly influence the course of illness in children with steroid-sensitive nephrotic syndrome. Kidney Int. 2015;87:217–224.10.1038/ki.2014.240
   PubMed Web of Science ®Google Scholar
• Webb NJ, Frew E, Brettell EA, et al. Short course daily prednisolone therapy during an upper respiratory tract infection in children with relapsing steroid-sensitive nephrotic syndrome (PREDNOS 2): protocol for a randomised controlled trial. Trials. 2014;15:147.10.1186/1745-6215-15-147
   PubMed Web of Science ®Google Scholar
• Prospective, controlled trial of cyclophosphamide therapy in children with nephrotic syndrome. Report of the International study of Kidney Disease in Children. Lancet. 1974;2:423–427.
   PubMed Web of Science ®Google Scholar
• Cyclophosphamide treatment of steroid dependent nephrotic syndrome: comparison of eight week with 12 week course. Report of Arbeitsgemeinschaft fur Padiatrische Nephrologie. Arch Dis Child. 1987;62:1102–1106.
   Google Scholar
• Barratt TM, Soothill JF. Controlled trial of cyclophosphamide in steroid-sensitive relapsing nephrotic syndrome of childhood. Lancet. 1970;296:479–482.10.1016/S0140-6736(70)90108-X
   Google Scholar
• Kemper MJ, Altrogge H, Ludwig K, et al. Unfavorable response to cyclophosphamide in steroid-dependent nephrotic syndrome. Pediatr Nephrol. 2000;14:772–775.10.1007/PL00013435
   PubMed Web of Science ®Google Scholar
• Zagury A, de Oliveira AL, de Moraes CA, et al. Long-term follow-up after cyclophosphamide therapy in steroid-dependent nephrotic syndrome. Pediatr Nephrol. 2011;26:915–920.10.1007/s00467-011-1825-x
   PubMed Web of Science ®Google Scholar
• Pravitsitthikul N, Willis NS, Hodson EM, et al. Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children. Cochrane Database Syst Rev. 2013;10:CD002290.
   Google Scholar
• Tarshish P, Tobin JN, Bernstein J, et al. Cyclophosphamide does not benefit patients with focal segmental glomerulosclerosis. A report of the International Study of Kidney Disease in Children. Pediatr Nephrol. 1996;10:590–593.10.1007/s004670050167
   PubMed Web of Science ®Google Scholar
• Szeto C, Gillespie KM, Mathieson PW. Levamisole induces interleukin-18 and shifts type 1/type 2 cytokine balance. Immunology. 2000;100:217–224.10.1046/j.1365-2567.2000.00042.x
   PubMed Web of Science ®Google Scholar
• Levamisole for corticosteroid-dependent nephrotic syndrome in childhood. British Association for Paediatric Nephrology. Lancet. 1991;337:1555–1557.
   PubMedGoogle Scholar
• Dayal U, Dayal AK, Shastry JC, et al. Use of levamisole in maintaining remission in steroid-sensitive nephrotic syndrome in children. Nephron. 1994;66:408–412.
   PubMed Web of Science ®Google Scholar
• Davin JC, Merkus MP. Levamisole in steroid-sensitive nephrotic syndrome of childhood: the lost paradise? Pediatr Nephrol. 2005;20:10–14.10.1007/s00467-004-1615-9
   PubMed Web of Science ®Google Scholar
• Ishikura K, Ikeda M, Hattori S, et al. Effective and safe treatment with cyclosporine in nephrotic children: a prospective, randomized multicenter trial. Kidney Int. 2008;73:1167–1173.10.1038/ki.2008.24
   PubMed Web of Science ®Google Scholar
• Ishikura K, Yoshikawa N, Hattori S, et al. Treatment with microemulsified cyclosporine in children with frequently relapsing nephrotic syndrome. Nephrol Dial Transplant. 2010;25:3956–3962.10.1093/ndt/gfq318
   PubMed Web of Science ®Google Scholar
• Kitano Y, Yoshikawa N, Tanaka R, et al. Ciclosporin treatment in children with steroid-dependent nephrotic syndrome. Pediatr Nephrol. 1990;4:474–477.10.1007/BF00869823
   PubMed Web of Science ®Google Scholar
• Morgan C, Sis B, Pinsk M, et al. Renal interstitial fibrosis in children treated with FK506 for nephrotic syndrome. Nephrol Dial Transplant. 2011;26:2860–2865.10.1093/ndt/gfq813
   PubMed Web of Science ®Google Scholar
• Iijima K, Hamahira K, Tanaka R, et al. Risk factors for cyclosporine-induced tubulointerstitial lesions in children with minimal change nephrotic syndrome. Kidney Int. 2002;61:1801–1805.10.1046/j.1523-1755.2002.00303.x
   PubMed Web of Science ®Google Scholar
• Heisel O, Heisel R, Balshaw R, et al. New onset diabetes mellitus in patients receiving calcineurin inhibitors: a systematic review and meta-analysis. Am J Transplant. 2004;4:583–595.10.1046/j.1600-6143.2003.00372.x
   PubMed Web of Science ®Google Scholar
• Iijima K, Sako M, Oba MS, et al. Cyclosporine C2 monitoring for the treatment of frequently relapsing nephrotic syndrome in children: a multicenter randomized phase II trial. Clin J Am Soc Nephrol. 2014;9:271–278.10.2215/CJN.13071212
   PubMed Web of Science ®Google Scholar
• Roberti I, Vyas S. Long-term outcome of children with steroid-resistant nephrotic syndrome treated with tacrolimus. Pediatr Nephrol. 2010;25:1117–1124.10.1007/s00467-010-1471-8
   PubMed Web of Science ®Google Scholar
• Tanaka R, Yoshikawa N, Kitano Y, et al. Long-term ciclosporin treatment in children with steroid-dependent nephrotic syndrome. Pediatr Nephrol. 1993;7:249–252.10.1007/BF00853209
   PubMed Web of Science ®Google Scholar
• El-Husseini A, El-Basuony F, Mahmoud I, et al. Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome: a single-centre experience. Nephrol Dial Transplant. 2005;20:2433–2438.10.1093/ndt/gfi059
   PubMed Web of Science ®Google Scholar
• Lieberman kV, Tejani A. A randomized double-blind placebo-controlled trial of cyclosporine in steroid-resistant idiopathic focal segmental glomerulosclerosis in children. J Am Soc Nephrol. 1996;7:56–63.
   PubMed Web of Science ®Google Scholar
• Ponticelli C, Rizzoni G, Edefonti A, et al. A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome. Kidney Int. 1993;43:1377–1384.10.1038/ki.1993.194
   PubMed Web of Science ®Google Scholar
• Waldo FB, Benfield MR, Kohaut EC. Therapy of focal and segmental glomerulosclerosis with methylprednisolone, cyclosporine A and prednisone. Pediatr Nephrol. 1998;12:397–400.10.1007/s004670050473
   PubMed Web of Science ®Google Scholar
• Hamasaki Y, Yoshikawa N, Hattori S, et al. Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2009;24:2177–2185.10.1007/s00467-009-1264-0
   PubMed Web of Science ®Google Scholar
• Moudgil A, Bagga A, Jordan SC. Mycophenolate mofetil therapy in frequently relapsing steroid-dependent and steroid-resistant nephrotic syndrome of childhood: current status and future directions. Pediatr Nephrol. 2005;20:1376–1381.10.1007/s00467-005-1964-z
   PubMed Web of Science ®Google Scholar
• Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN, et al. Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatr Nephrol. 2008;23:2013–2020.10.1007/s00467-008-0899-6
   PubMed Web of Science ®Google Scholar
• Gellermann J, Weber L, Pape L, et al. Mycophenolate mofetil versus cyclosporin a in children with frequently relapsing nephrotic syndrome. J Am Soc Nephrol. 2013;24:1689–1697.10.1681/ASN.2012121200
   PubMed Web of Science ®Google Scholar
• Hackl A, Cseprekal O, Gener M, et al. Mycophenolate-mofetil therapy in children with idiopathic nephrotic syndrome. Does therapeutic drug monitoring make a difference? Ther Drug Monit. 2015;38:274–279.
   Web of Science ®Google Scholar
• Guigonis V, Dallocchio A, Baudouin V, et al. Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases. Pediatr Nephrol. 2008;23:1269–1279.10.1007/s00467-008-0814-1
   PubMed Web of Science ®Google Scholar
• Hofstra JM, Deegens JK, Wetzels JF. Rituximab: effective treatment for severe steroid-dependent minimal change nephrotic syndrome? Nephrol Dial Transplant. 2007;22:2100–2102.10.1093/ndt/gfm128
   PubMed Web of Science ®Google Scholar
• Kamei K, Ito S, Nozu K, et al. Single dose of rituximab for refractory steroid-dependent nephrotic syndrome in children. Pediatr Nephrol. 2009;24:1321–1328.10.1007/s00467-009-1191-0
   PubMed Web of Science ®Google Scholar
• Ruggenenti P, Ruggiero B, Cravedi P, et al. Rituximab in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome. J Am Soc Nephrol. 2014;25:850–863.10.1681/ASN.2013030251
   PubMed Web of Science ®Google Scholar
• Ravani P, Magnasco A, Edefonti A, et al. Short-term effects of rituximab in children with steroid- and calcineurin-dependent nephrotic syndrome: a randomized controlled trial. Clin J Am Soc Nephrol. 2011;6:1308–1315.10.2215/CJN.09421010
   PubMed Web of Science ®Google Scholar
• 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;384:1273–1281.10.1016/S0140-6736(14)60541-9
   PubMed Web of Science ®Google Scholar
• Ravani P, Rossi R, Bonanni A, et al. Rituximab in children with steroid-dependent nephrotic syndrome: a multicenter, open-label, noninferiority, randomized controlled trial. J Am Soc Nephrol. 2015;26:2259–2266.10.1681/ASN.2014080799
   PubMed Web of Science ®Google Scholar
• Boren EJ, Cheema GS, Naguwa SM, et al. The emergence of progressive multifocal leukoencephalopathy (PML) in rheumatic diseases. J Autoimmun. 2008;30:90–98.10.1016/j.jaut.2007.11.013
   PubMed Web of Science ®Google Scholar
• Chaumais MC, Garnier A, Chalard F, et al. Fatal pulmonary fibrosis after rituximab administration. Pediatr Nephrol. 2009;24:1753–1755.10.1007/s00467-009-1195-9
   PubMed Web of Science ®Google Scholar
• Sato M, Ito S, Ogura M, et al. Atypical Pneumocystis jiroveci pneumonia with multiple nodular granulomas after rituximab for refractory nephrotic syndrome. Pediatr Nephrol. 2013;28:145–149.10.1007/s00467-012-2286-6
   PubMed Web of Science ®Google Scholar
• Pei SN, Chen CH, Lee CM, et al. Reactivation of hepatitis B virus following rituximab-based regimens: a serious complication in both HBsAg-positive and HBsAg-negative patients. Ann Hematol. 2010;89:255–262.10.1007/s00277-009-0806-7
   PubMed Web of Science ®Google Scholar
• Ardelean DS, Gonska T, Wires S, et al. Severe ulcerative colitis after rituximab therapy. Pediatrics. 2010;126:e243–e246.10.1542/peds.2009-3395
   PubMed Web of Science ®Google Scholar
• Kamei K, Takahashi M, Fuyama M, et al. Rituximab-associated agranulocytosis in children with refractory idiopathic nephrotic syndrome: case series and review of literature. Nephrol Dial Transplant. 2015;30:91–96.10.1093/ndt/gfu258
   PubMed Web of Science ®Google Scholar
• Wang JJ, Mao JH. The etiology of congenital nephrotic syndrome: current status and challenges. World J Pediatr. 2016;12:149–158.10.1007/s12519-016-0009-y
   PubMed Web of Science ®Google Scholar
• Cam H, Taytan Y, Aji DY, et al. Congenital syphilis presenting with nephrotic syndrome and leucocytoclastic vasculitis. J Eur Acad Dermatol Venereol. 2004;18:484–486.10.1111/jdv.2004.18.issue-4
   PubMed Web of Science ®Google Scholar
• Beale MG, Strayer DS, Kissane JM, et al. Congenital glomerulosclerosis and nephrotic syndrome in two infants. Speculations and pathogenesis. Am J Dis Child. 1979;133:842–845.10.1001/archpedi.1979.02130080082017
   PubMedGoogle Scholar
• Besbas N, Bayrakci US, Kale G, et al. Cytomegalovirus-related congenital nephrotic syndrome with diffuse mesangial sclerosis. Pediatr Nephrol. 2006;21:740–742.10.1007/s00467-006-0051-4
   PubMed Web of Science ®Google Scholar
• Miller S, Pallan S, Gangji AS, et al. Mercury-associated nephrotic syndrome: a case report and systematic review of the literature. Am J Kid Dis. 2013;62:135–138.10.1053/j.ajkd.2013.02.372
   PubMed Web of Science ®Google Scholar
• Cil O, Besbas N, Duzova A, et al. Genetic abnormalities and prognosis in patients with congenital and infantile nephrotic syndrome. Pediatr Nephrol. 2015;30:1279–1287.10.1007/s00467-015-3058-x
   PubMed Web of Science ®Google Scholar
• Basker M, Agarwal I, Bendon KS. Congenital nephrotic syndrome-a treatable cause. Ann Trop Paediatr. 2007;27:87–90.10.1179/146532807X170565
   PubMed Web of Science ®Google Scholar
• Lionel AP, Joseph LK, Simon A. Pierson syndrome – a rare cause of congenital nephrotic syndrome. Indian J Pediatr. 2014;81:1416–1417.10.1007/s12098-014-1507-3
   PubMed Web of Science ®Google Scholar
• Lau KK, Chan HH, Massicotte P, et al. Thrombotic complications of neonates and children with congenital nephrotic syndrome. Curr Pediatr Rev. 2014;10:169–176.
   PubMedGoogle Scholar
• Niel O, Thouret MC, Berard E. Anemia in congenital nephrotic syndrome: role of urinary copper and ceruloplasmin loss. Blood. 2011;117:6054–6055.10.1182/blood-2011-02-335844
   PubMed Web of Science ®Google Scholar
• Payne KM, Nelson MR, Petersen MM. Congenital nephrotic syndrome and agammaglobulinemia: a therapeutic dilemma. Ann Allergy Asthma Immunol. 2013;111:142–143.10.1016/j.anai.2013.05.018
   PubMed Web of Science ®Google Scholar
• Jalanko H. Congenital nephrotic syndrome. Pediatr Nephrol. 2009;24:2121–2128.10.1007/s00467-007-0633-9
   PubMed Web of Science ®Google Scholar