Pediatrics in Review
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(Pediatrics in Review. 2009;30:e78-e79. doi:10.1542/10.1542/pir.30-10-e78)
© 2009 American Academy of Pediatrics

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In Brief

Polycystic Kidney Disease

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Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD): The New Horizon For Children Who Have DPKD. Rizk D, Chapman A. Pediatr Nephrol. 2008;23 :1029 –1036[CrossRef][Medline]

Autosomal Recessive Polycystic Kidney Disease. Zerres K. Clin Investig. 1992;70 :794 –801[Medline]

Familial Juvenile Nephronophthisis. Konrad M, Saunier S, Calado J, et al. J Mol Med. 1998;76 :310 –316[CrossRef][Medline]

Congenital Nephropathies and Uropathies. Becker N, Avner E. Pediatr Clin North Am. 1995;42 :1324 –1328

Polycystic kidney disease is the most common hereditary renal malformation. Many malformation syndromes are associated with renal cysts, the primary renal diseases being autosomal dominant polycystic kidney disease (ADPKD), autosomal recessive polycystic kidney disease (ARPKD), and juvenile nephronophthisis (NPH).

ADPKD is the most common inherited renal disease, affecting 1 in 800 live births in the United States. The genetic defect is present in utero, but most affected children are asymptomatic. The usual onset of clinical manifestations occurs from the third through . . . [Full Text of this Article]


Jacques Benun, MD
Carol Lewis, MD
Hasbro Children's Hospital
Providence, RI


Janet R. Serwint, MD, Consulting Editor

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