Abstract：
Autosomal recessive polycystic kidney disease (ARPKD) is the rare and usually early-onset form of polycystic kidney disease with a typical clinical presentation of enlarged cystic kidneys and liver involvement with congenital hepatic fibrosis or Caroli syndrome. ARPKD remains a clinical challenge in pediatrics, frequently requiring continuous and long-term multidisciplinary treatment. In this review, we aim to give an overview over clinical aspects of ARPKD and recent developments in our understanding of disease progression, risk patterns, and treatment of ARPKD.
摘要：
常染色体隐性遗传性多囊肾病（ARPKD）是多囊肾病的罕见且通常是早发性形式，具有典型的囊性肾脏增大和肝脏受累先天性肝纤维化或Caroli综合征的临床表现。ARPKD仍然是儿科的临床挑战，经常需要连续和长期的多学科治疗。在这次审查中,我们的目的是概述ARPKD的临床方面以及我们对疾病进展的理解的最新进展，风险模式，ARPKD的治疗。
