Highlights & Basics
- The most common form of nephrotic syndrome in children, characterized by minimal histologic changes in the kidney; 90% of cases are idiopathic.
- Children typically present with peripheral edema, although some are asymptomatic. Diagnosis is usually made on clinical grounds.
- Corticosteroid therapy is the mainstay of treatment. Long-term corticosteroid therapy has significant adverse effects; therefore, if needed, corticosteroid-sparing therapies can be added.
- Renal biopsy is usually reserved for those patients who do not respond to corticosteroid therapy or have frequent relapses, or for the diagnosis of patients <1 year old.
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Citations
Gordillo R, Spitzer A. The nephrotic syndrome. Pediatr Rev. 2009;30:94-104.[Abstract]
Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003 Aug 23;362(9384):629-39.[Abstract]
Shigidi MM. The treatment of relapse in adults with minimal change nephrotic syndrome: myths and facts. Saudi J Kidney Dis Transpl. 2011;22:10-17.[Abstract]
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11. Andreoli SP. Racial and ethnic differences in the incidence and progression of focal segmental glomerulosclerosis in children. Adv Ren Replace Ther. 2004;11:105-109.[Abstract]
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13. International Study of Kidney Disease in Children. 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.[Abstract]
14. Filler G, Young E, Geier P, et al. Is there really an increase in non-minimal change nephrotic syndrome in children? Am J Kidney Dis. 2003;42:1107-1113.[Abstract]
15. Borges FF, Shiraichi L, da Silva MP, et al. Is focal segmental glomerulosclerosis increasing in patients with nephrotic syndrome? Pediatr Nephrol. 2007;22:1309-1313.[Abstract]
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19. 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.[Abstract]
20. Aslam N, Nseir NI, Viverett JF, et al. Nephrotic syndrome in chronic lymphocytic leukemia: a paraneoplastic syndrome? Clin Nephrol. 2000;54:492-497.[Abstract]
21. Shalhoub RJ. Pathogenesis of lipoid nephrosis: a disorder of T-cell function. Lancet. 1974;2(7880):556-560.[Abstract]
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27. Ehrich JH, Brodehl J. Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children. Arbeitsgemeinschaft fur Padiatrische Nephrologie. Eur J Pediatr. 1993;152:357-361.[Abstract]
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30. Colquitt JL, Kirby J, Green C, et al. The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic corticosteroid-resistant nephrotic syndrome: a systematic review. Health Technol Assess. 2007;11:iii-iv, ix-xi, 1-93.[Abstract]
31. Bock ME, Cohn RA, Ali FN. Treatment of childhood nephrotic syndrome with long-term, low-dose tacrolimus. Clin Nephrol. 2013;79:432-438.[Abstract]
32. Hamasaki Y, Yoshikawa N, Nakazato H, et al. Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis. Pediatr Nephrol. 2013;28:765-771.[Abstract]
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34. Palmer SC, Nand K, Strippoli GF. Interventions for minimal change disease in adults with nephrotic syndrome. Cochrane Database Syst Rev. 2008;(1):CD001537.[Abstract][Full Text]
35. Takei I, Nitta K. Rituximab and minimal change nephrotic syndrome: a therapeutic option. Clin Exp Nephrol. 2011;15:641-647.[Abstract]
36. Peters HP, van de Kar NC, Wetzels JF. Rituximab in minimal change nephropathy and focal segmental glomerulosclerosis: report of four cases and review of the literature. Neth J Med. 2008;66:408-415.[Abstract]
37. Bruchfeld A, Benedek S, Hilderman M, et al. Rituximab for minimal change disease in adults: long-term follow-up. Nephrol Dial Transplant. 2014;29:851-856.[Abstract][Full Text]
38. Iwabuchi Y, Takei T, Moriyama T, et al. Long-term prognosis of adult patients with steroid-dependent minimal change nephrotic syndrome following rituximab treatment. Medicine (Baltimore). 2014;93:e300.[Abstract][Full Text]
39. Janardan J, Ooi K, Menahem S. Sustained complete remission of steroid- and cyclophosphamide-resistant minimal-change disease with a single course of rituximab therapy. Clin Kidney J. 2014;7:293-295.[Abstract][Full Text]
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41. Bhimma R, Adhikari M, Asharam K, et al. The spectrum of chronic kidney disease (stages 2-5) in KwaZulu-Natal, South Africa. Pediatr Nephrol. 2008;23:1841-1846.[Abstract]
42. Ruth EM, Kemper MJ, Leumann EP, et al. Children with corticosteroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr. 2005;147:202-207.[Abstract]
43. Pais P, Avner ED. Nephrotic syndrome. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011.
44. Nachman PH, Jennette JC, Falk RJ. Primary glomerular disease. In: Brenner BM, ed. Brenner and Rector's The Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:30.
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46. Shigidi MM. The treatment of relapse in adults with minimal change nephrotic syndrome: myths and facts. Saudi J Kidney Dis Transpl. 2011;22:10-17.[Abstract]
47. Renal Clinicians Group, Renal Unit, Royal Hospital for Sick Children, Yorkhill Division. Guideline for the management of nephrotic syndrome. November 2005. http://www.clinicalguidelines.scot.nhs.uk/ (last accessed 12 September 2017).[Full Text]
48. Gillespie RS. Pediatric nephrology resources for healthcare professionals. http://kidneyweb.net (last accessed 12 September 2017).[Full Text]
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