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.
Minimal change disease (MCD) is the most common form of nephrotic syndrome (a clinical condition characterized by heavy proteinuria, edema, hypoalbuminemia, and hyperlipidemia) affecting children. Studies in the US have shown that, between the ages of 2 and 8 years, 90% of children with nephrotic syndrome have minimal histologic changes in the kidney, hence the name minimal change disease. 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.
History and exam
Professor and Chief Medical Director
Pediatric Dialysis & Renal Transplantation
Department of Pediatrics
Blair E. Batson Hospital for Children
University of Mississippi Medical Center
MPD has acted as a consultant for Retrophin Pharmaceuticals.
Dr Mehul P. Dixit would like to gratefully acknowledge Dr Robert Mathias, a previous contributor to this topic. RM declares that he has no competing interests.
Associate Professor of Paediatrics and Paediatric Nephrologist
Department of Maternal and Child Health
Nelson R. Mandela School of Medicine
University of KwaZulu-Natal
RB is an author of a reference cited in the monograph.
Professor of Pediatrics
Vice Chairman for Affiliate & Network Affairs
Chief, Section on Nephrology
Division of Pediatric Nephrology
Children's Hospital at Montefiore of Albert Einstein College of Medicine
FJK declares that he has no competing interests.
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