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New guidelines issued for nephrotic syndrome in children

Research Activities, December 2009, No. 352

New guidelines have been released on the diagnosis and management of childhood nephrotic syndrome. The syndrome, which can lead to kidney failure, is characterized by large amounts of protein in the urine, increased cholesterol levels, and fluid retention. Development of the guidelines was prompted by changes in the incidence of different forms of the condition, with increases over the past decade in one type (focal segmental glomerulosclerosis). This type of nephrotic syndrome is less responsive to glucocorticoid treatment (the gold standard initial treatment) and also has a greater risk for progressive kidney failure. In addition, the greater prevalence of obesity and diabetes among U.S. children may be exacerbated by long-term glucocorticoid (steroid) therapy.

The current recommendations are based on an extensive literature search, analysis of the data, and expert opinion from a consensus study group on the topic. The guidelines offer recommendations to physicians for the initial evaluation of children with nephrotic syndrome who are aged 1 to 18 years old. Important to its treatment and management are defining key descriptors of nephrotic syndrome management, including remission, relapse, frequently relapsing, steroid-dependent, and steroid-resistant. The guidelines offer definitions for these categories of treatment response, which form the basis of implementing appropriate therapy. Clinicians will also find a section on complications, including growth disturbances, lipid abnormalities, infection, and blood clots. For children who are responsive to steroids, the guidelines recommend an initial, 12-week glucocorticoid regimen in order to decrease the chance of a relapse.

The guidelines also note that children who are overweight at the start of steroid therapy are more likely to remain overweight after treatment. The use of glucocorticoids may impair growth and increase body mass index. Both patients and their caregivers need to be educated in the complex treatment of this chronic condition. They should receive specialized instruction in the proper administration of medications, how to deal with dietary restrictions, and the need for ongoing medical monitoring.

The study was supported in part by the Agency for Healthcare Research and Quality (HS10397). See "Management of childhood onset nephrotic syndrome," by Debbie S. Gipson, M.D., M.S., Susan F. Massengill, M.D., Lynne Yao, M.D., and others, in the August 2009 Pediatrics 124(2), pp. 747-757.

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Page last reviewed December 2009
Internet Citation: New guidelines issued for nephrotic syndrome in children: Research Activities, December 2009, No. 352. December 2009. Agency for Healthcare Research and Quality, Rockville, MD.