Published online July 27, 2009
PEDIATRICS Vol. 124 No. 2 August 2009, pp. 747-757 (doi:10.1542/peds.2008-1559)

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SPECIAL ARTICLE

Management of Childhood Onset Nephrotic Syndrome

Debbie S. Gipson, MD, MS^a, Susan F. Massengill, MD^b, Lynne Yao, MD^c, Shashi Nagaraj, MD^d, William E. Smoyer, MD^e,f, John D. Mahan, MD^f, Delbert Wigfall, MD^g, Paul Miles, MD^h, Leslie Powell, RN, CPNP^a, Jen-Jar Lin, MD, PhD^d,i, Howard Trachtman, MD^j and Larry A. Greenbaum, MD, PhD^k

^a Division of Nephrology and Hypertension, Department of Medicine and Pediatrics, University of North Carolina, Chapel Hill, North Carolina
^b Pediatric Nephrology, Levine Children's Hospital at Carolinas Medical Center, Charlotte, North Carolina
^c Department of Pediatric Nephrology, Inova Fairfax Hospital for Children, Falls Church, Virginia
^d Department of Pediatric Nephrology, Wake Forest University Medical Center, Winston-Salem, North Carolina
^e Department of Pediatric Nephrology, University of Michigan, Ann Arbor, Michigan
^f Department of Pediatric Nephrology, Nationwide Children's Hospital, Columbus, Ohio
^g Division of Nephrology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina
^h American Board of Pediatrics, Chapel Hill, North Carolina
ⁱ Department of Pediatric Nephrology, East Carolina University, Greenville, North Carolina
^j Department of Pediatric Nephrology, Schneider Children's Hospital, New Hyde Park, New York
^k Department of Pediatric Nephrology, Emory University and Children's Healthcare of Atlanta, Atlanta, Georgia

The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967. The characteristics of children presenting with nephrotic syndrome have changed over recent decades with greater frequency of the challenging condition focal segmental glomerulosclerosis and a greater prevalence of obesity and diabetes mellitus, which may be resistant to glucocorticoids in the former and exacerbated by long-term glucocorticoid therapy in the latter 2 conditions. The Children's Nephrotic Syndrome Consensus Conference was formed to systematically review the published literature and generate a children's primary nephrotic syndrome guideline for use in educational, therapeutic, and research venues.

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Key Words: proteinuria • pediatric • nephrosis • kidney disease

Abbreviations: ISKDC—International Study of Kidney Disease in Children • MCNS—minimal-change nephrotic syndrome • FSGS—focal segmental glomerulosclerosis • Up/c—urine protein/creatinine ratio • BID—twice daily • ACE-I—angiotensin-converting enzyme inhibitor • ARB—angiotensin receptor blocker • HMG-CoA—3-hydroxy-3-methylglutaryl coenzyme A

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Accepted Nov 26, 2008.

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