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
Key diagnostic factors
- facial or generalized edema
- age >1 year but <8 years
Other diagnostic factors
- normal BP
- absence of hematuria
- history of recent viral illness
- history of lymphoma or leukemia
- age >1 year but <8 years
- Hodgkin lymphoma
- recent viral illness
- hepatitis B or C infection
- low birth weight (LBW)
1st investigations to order
- 24-hour urine protein
- urine protein/creatinine ratio
- serum albumin level
- serum lipid profile
- serum complement levels
- serum electrolyte panel
- complete blood count
- glomerular filtration rate (GFR)
- serum LFTs
- renal ultrasound
Investigations to consider
- chest x-ray
- renal biopsy
frequent relapse or corticosteroid-dependent or severe corticosteroid adverse effects
Mehul P. Dixit, MD, DM (Nephrology)
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.
Rajendra Bhimma, MD
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.
Frederick J. Kaskel, MD
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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