Abstract

Autosomal dominant polycystic kidney disease(ADPKD) is the most common hereditary renal disease, which has an incidence of 1/1 000 to 1/500 in live births, was characterized by renal enlargement along with renal tubular cystic dilatation, approximately half of affected individuals reach end stage renal disease(ESRD) by 60 years of age, accounting for 7%-10% of patients with ESRD.ADPKD could be diagnosed in children and even in utero, early identification and intervention in children would ameliorate the progression of ADPKD to ESRD.Therefore, it is imperative for pediatricians to recognize the early clinical manifestations and outcome of the disease.The aim of this review will focus on the early identification, management and outcome in childhood. Key words: Autosomal dominant polycystic kidney disease; Early identification; Outcome

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