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获得性肾囊肿性疾病:终末期肾病儿童中一种未被充分认识的疾病。

Acquired cystic kidney disease: an under-recognized condition in children with end-stage renal disease.

机构信息

Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA.

出版信息

Pediatr Nephrol. 2018 Jan;33(1):41-51. doi: 10.1007/s00467-017-3649-9. Epub 2017 Apr 25.

Abstract

Acquired cystic kidney disease (ACKD) is a condition that occurs predominantly in patients with end-stage renal disease (ESRD). In contrast to hereditary cystic kidney disease, ACKD is characterized by the presence of multiple small cysts in bilaterally small kidneys. Limited pediatric data suggest a high incidence (21.6-45.8%) of ACKD in children on dialysis, comparable to that in adults, with an increased frequency associated with a longer duration of dialysis. Recent research has shed light on the pathogenesis of ACKD, such as activation of proto-oncogenes. Although most patients with ACKD are asymptomatic, the condition can be complicated by renal cell carcinoma. Routine surveillance should therefore be considered in at-risk populations.

摘要

获得性肾囊肿病(ACKD)主要发生于终末期肾病(ESRD)患者。与遗传性囊性肾病不同,ACKD 的特征是双侧小肾脏中存在多个小囊肿。有限的儿科数据表明,透析儿童的 ACKD 发病率较高(21.6-45.8%),与成人相当,且随着透析时间的延长,发病率也随之增加。最近的研究揭示了 ACKD 的发病机制,如原癌基因的激活。虽然大多数 ACKD 患者无症状,但该疾病可能会并发肾细胞癌。因此,应考虑对高危人群进行常规监测。

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