title: Autosomal dominant polycystic kidney disease with ectopic unilateral multicystic dysplastic kidney.
abstract: BACKGROUND
  Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. In most cases, ADPKD similarly affects bilateral kidneys.
  
  
  METHODS
  Among the 605 ADPKD patients that were followed up by our center, we identified two male patients with unilateral ADPKD. The cases were remarkable because the patients also had ectopia and multicystic dysplasia in the contralateral kidney, which are generally sporadic disease conditions. Both patients tested positive for polycystic kidney disease 1 mutation, but negative for hepatocyte nuclear factor 1 beta mutation. Moreover, the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings. Both patients had started a long-term hemodialysis in their 40s.
  
  
  CONCLUSIONS
  Anatomical and genetic abnormality in patients with ADPKD may be more frequent and complex than previously believed. The compensatory capacity in patients with ADPKD is fragile, and missing one kidney could accelerate the deterioration of renal function.
authors: Division of Nephrology, Kidney Institute of CPLA, Changzheng Hospital, Second Military Medical University, 415 Fengyang Rd, 200003, Shanghai, China.
authors: Jing
authors: J
authors: Xu
authors: Dong-Ping
authors: DP
authors: Chen
authors: Zhi-Guo
authors: ZG
authors: Mao
authors: He-Feng
authors: HF
authors: Huang
authors: Chen-Ming
authors: CM
authors: Xu
authors: Cong-Rong
authors: CR
authors: Wang
authors: Wei-Ping
authors: WP
authors: Jia
authors: Chang-Lin
authors: CL
authors: Mei
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008875:Middle Aged; D021782:Multicystic Dysplastic Kidney / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics