Highlights & Basics
- Minimal change disease (MCD) is the most common form of idiopathic nephrotic syndrome (NS) in children (70% to 90% of cases) and is characterized by minimal histologic changes in the kidney. In adults, MCD is the cause of NS in 10% to 15% of cases.
- Children typically present with peripheral edema, associated with heavy proteinuria, hypoalbuminemia, and hyperlipidemia, although some are asymptomatic. Adults have similar symptoms and signs but may also have hypertension, hematuria, and acute kidney injury at the point of diagnosis.
- Corticosteroid therapy is the mainstay of treatment. Long-term corticosteroid therapy has significant adverse effects; therefore, if needed, corticosteroid-sparing therapies can be added. However, most children outgrow MCD and stop having relapses during their teenage years.
- In children ages up to 12 years, treatment is typically empiric, with kidney biopsy usually reserved for those patients who do not respond to corticosteroid therapy or have frequent relapses, or for diagnosis in infants ages <1 year old. Kidney biopsy is required for diagnosis in adults.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Citations
Vivarelli M, Massella L, Ruggiero B, et al. Minimal change disease. Clin J Am Soc Nephrol. 2017 Feb 7;12(2):332-45.[Abstract][Full Text]
Trautmann A, Boyer O, Hodson E, et al. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2023 Mar;38(3):877-919.[Abstract][Full Text]
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4s):S1-276.[Full Text]
Hogan J, Radhakrishnan J. The treatment of minimal change disease in adults. J Am Soc Nephrol. 2013 Apr;24(5):702-11.[Abstract]
1. Vivarelli M, Massella L, Ruggiero B, et al. Minimal change disease. Clin J Am Soc Nephrol. 2017 Feb 7;12(2):332-45.[Abstract][Full Text]
2. Trautmann A, Boyer O, Hodson E, et al. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2023 Mar;38(3):877-919.[Abstract][Full Text]
3. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4s):S1-276.[Full Text]
4. Gordillo R, Spitzer A. The nephrotic syndrome. Pediatr Rev. 2009;30:94-104.[Abstract]
5. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003 Aug 23;362(9384):629-39.[Abstract]
6. Su S, Yu J, Wang Y, et al. Clinicopathologic correlations of renal biopsy findings from northeast China: A 10-year retrospective study. Medicine (Baltimore). 2019 Jun;98(23):e15880.[Abstract][Full Text]
7. Veltkamp F, Rensma LR, Bouts AHM, et al. Incidence and relapse of idiopathic nephrotic syndrome: meta-analysis. Pediatrics. 2021 Jul;148(1):e2020029249.[Abstract][Full Text]
8. Ranganathan S. Pathology of podocytopathies causing nephrotic syndrome in children. Front Pediatr. 2016;4:32.[Abstract][Full Text]
9. Baqi N, Singh A, Balachandra S, et al. The paucity of minimal change disease in adolescents with primary nephrotic syndrome. Pediatr Nephrol. 1998;12:105-107.[Abstract]
10. Horinouchi T, Nozu K, Iijima K. An updated view of the pathogenesis of steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2022 Sep;37(9):1957-65.[Abstract][Full Text]
11. Davin JC. The glomerular permeability factors in idiopathic nephrotic syndrome. Pediatr Nephrol. 2016 Feb;31(2):207-15.[Abstract][Full Text]
12. Hackl A, Zed SEDA, Diefenhardt P, et al. The role of the immune system in idiopathic nephrotic syndrome. Mol Cell Pediatr. 2021 Nov 18;8(1):18.[Abstract][Full Text]
13. Shalhoub RJ. Pathogenesis of lipoid nephrosis: a disorder of T-cell function. Lancet. 1974;2(7880):556-60.[Abstract]
14. Kim SH, Park SJ, Han KH, et al. Pathogenesis of minimal change nephrotic syndrome: an immunological concept. Korean J Pediatr. 2016 May;59(5):205-11.[Abstract][Full Text]
15. Watts AJB, Keller KH, Lerner G, et al. Discovery of autoantibodies targeting nephrin in minimal change disease supports a novel autoimmune etiology. J Am Soc Nephrol. 2022 Jan;33(1):238-52.[Abstract][Full Text]
16. Jia X, Yamamura T, Gbadegesin R, et al. Common risk variants in NPHS1 and TNFSF15 are associated with childhood steroid-sensitive nephrotic syndrome. Kidney Int. 2020 Nov;98(5):1308-22.[Abstract][Full Text]
17. Lane BM, Cason R, Esezobor CI, et al. Genetics of childhood steroid sensitive nephrotic syndrome: an update. Front Pediatr. 2019;7:8.[Abstract][Full Text]
18. Fogo AB, Lusco MA, Najafian B, et al. AJKD atlas of renal pathology: minimal change disease. Am J Kidney Dis. 2015 Aug;66(2):376-7.[Full Text]
19. Murugapandian S, Mansour I, Hudeeb M, et al. Epidemiology of glomerular disease in southern Arizona: review of 10-year renal biopsy data. Medicine (Baltimore). 2016;95:e3633.[Abstract][Full Text]
20. 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;13:159-65.[Abstract]
21. 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]
22. Aslam N, Nseir NI, Viverett JF, et al. Nephrotic syndrome in chronic lymphocytic leukemia: a paraneoplastic syndrome? Clin Nephrol. 2000;54:492-497.[Abstract]
23. Teeninga N, Schreuder MF, Bokenkamp A, et al. Influence of low birth weight on minimal change nephrotic syndrome in children, including a meta-analysis. Nephrol Dial Transplant. 2008;23:1615-20.[Abstract][Full Text]
24. Korbet SM, Whittier WL. Management of adult minimal change disease. Clin J Am Soc Nephrol. 2019 Jun 7;14(6):911-3.[Full Text]
25. Hogan J, Radhakrishnan J. The treatment of minimal change disease in adults. J Am Soc Nephrol. 2013 Apr;24(5):702-11.[Abstract]
26. Li S, Zhang M, Chen L, et al. An implication of relationship between tuberculosis and primary nephrotic syndrome. Int J Artif Organs. 2015 Apr;38(4):178-83.[Abstract]
27. Azukaitis K, Palmer SC, Strippoli GF, et al. Interventions for minimal change disease in adults with nephrotic syndrome. Cochrane Database Syst Rev. 2022 Mar 1;3(3):CD001537.[Abstract][Full Text]
28. Beck LH Jr, Ayoub I, Caster D, et al. KDOQI US commentary on the 2021 KDIGO clinical practice guideline for the management of glomerular diseases. Am J Kidney Dis. 2023 Aug;82(2):121-75.[Abstract][Full Text]
29. Schijvens AM, Teeninga N, Dorresteijn EM, et al. Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis. Eur J Pediatr. 2021 Sep;180(9):2849-59.[Abstract][Full Text]
30. Webb NJA, Woolley RL, Lambe T, et al. Long term tapering versus standard prednisolone treatment for first episode of childhood nephrotic syndrome: phase III randomised controlled trial and economic evaluation. BMJ. 2019 May 23;365:l1800.[Abstract][Full Text]
31. Hahn D, Samuel SM, Willis NS, et al. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev. 2024 Aug 22;8(8):CD001533.[Abstract]
32. Trautmann A, Vivarelli M, Samuel S, et al. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2020 Aug;35(8):1529-61.[Abstract][Full Text]
33. Liu ID, Willis NS, Craig JC, et al. Interventions for idiopathic steroid-resistant nephrotic syndrome in children. Cochrane Database Syst Rev. 2019 Nov 21;2019(11):CD003594.[Abstract][Full Text]
34. Larkins NG, Liu ID, Willis NS, et al. Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children. Cochrane Database Syst Rev. 2020 Apr 16;4(4):CD002290.[Abstract][Full Text]
35. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4s):S1-S276.[Abstract][Full Text]
36. Haws RM, Baum M. Efficacy of albumin and diuretic therapy in children with nephrotic syndrome. Pediatrics. 1993;91:1142-6.[Abstract]
37. Homik J, Suarez-Almazor ME, Shea B, et al. Calcium and vitamin D for corticosteroid-induced osteoporosis. Cochrane Database Syst Rev. 2000;1998(2):CD000952.[Abstract][Full Text]
38. International Study of Kidney Disease in Children. 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.[Abstract]
39. 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]
40. 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]
41. Hjorten R, Anwar Z, Reidy KJ. Long-term outcomes of childhood onset nephrotic sndrome. Front Pediatr. 2016;4:53.[Abstract][Full Text]
42. 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]
43. Parker K, Ragy O, Hamilton P, et al. Thromboembolism in nephrotic syndrome: controversies and uncertainties. Res Pract Thromb Haemost. 2023 Aug;7(6):102162.[Abstract][Full Text]
44. KidneyWeb. Pediatric nephrology resources for healthcare professionals [internet publication].[Full Text]
