The spectrum of autosomal dominant polycystic kidney disease in children and adolescents

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• 作者：Bharathi V. Reddy ; Arlene B. Chapman
• 关键词：ADPKD ; Adolescents ; Children ; Hypertension ; Total kidney volume
• 刊名：Pediatric Nephrology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：32
• 期：1
• 页码：31-42
• 全文大小：
• 刊物类别：Medicine
• 刊物主题：Pediatrics; Nephrology; Urology;
• 出版者：Springer Berlin Heidelberg
• ISSN：1432-198X
• 卷排序：32

文摘

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. It is characterized by the development of renal cysts and kidney enlargement and ultimately leads to renal failure typically in the sixth decade of life. Although most patients are asymptomatic until well into adulthood, renal cysts develop much earlier, often in utero. Significant renal anatomic and cystic expansion typically occurs before clinical manifestations in children and young adults with AKPKD. The cyst burden detected by imaging represents the minority of cyst burden, and renal and cardiovascular abnormalities are the most common manifestations in children with ADPKD. Here we review the molecular pathogenesis of ADPKD, discuss the screening, diagnosis and clinical manifestations of this renal disorder in childhood and adolescents and review treatment options and potential therapies currently being tested.