Minimal change disease

The most common form of nephrotic syndrome in children, characterised by minimal histological changes in the kidney; 90% of cases are idiopathic.

Children typically present with peripheral oedema, 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.

Minimal change disease (MCD) is the most common form of nephrotic syndrome (a clinical condition characterised by heavy proteinuria, oedema, hypoalbuminaemia, and hyperlipidaemia) affecting children.[1]Gordillo R, Spitzer A. The nephrotic syndrome. Pediatr Rev. 2009;30:94-104. http://www.ncbi.nlm.nih.gov/pubmed/19255123?tool=bestpractice.com [2]Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003 Aug 23;362(9384):629-39. http://www.ncbi.nlm.nih.gov/pubmed/12944064?tool=bestpractice.com Studies in the US have shown that, between the ages of 2 and 8 years, 90% of children with nephrotic syndrome have minimal histological changes in the kidney, hence the name minimal change disease.[1]Gordillo R, Spitzer A. The nephrotic syndrome. Pediatr Rev. 2009;30:94-104. http://www.ncbi.nlm.nih.gov/pubmed/19255123?tool=bestpractice.com [2]Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003 Aug 23;362(9384):629-39. http://www.ncbi.nlm.nih.gov/pubmed/12944064?tool=bestpractice.com MCD is typically primary (idiopathic) but may also be secondary to another condition. Although it usually presents in childhood, it can occur in adults. MCD is also known as lipoid nephrosis or nil disease.