Recessive (infantile) polycystic kidney disease is a rare inherited disorder with cystic dilations of the renal collecting ducts frequently associated with hepatic involvement. The gene responsible for this disease, PKHD1, located on the short arm of chromosome 6, has recently been identified. Autosomal recessive polycystic kidney disease is a rare entity with an incidence of 1:10,000 to 1:40,0...

Tuberous sclerosis complex (TSC) is a multisystemic inherited autosomal dominant disease characterized by the development of hamartomas in the brain and kidneys. In about 2% of patients, polycystic kidney disease is present, which may result in different stages of renal insufficiency. Acute kidney failure has not been reported in infants with TSC. We report a female infant with TSC who was admi...

Polycystic kidney and liver disease was seen in a stillborn white-tailed deer (Odocoileus virginianus) fawn. Bilaterally enlarged kidneys were characterized by severe dilatation of all renal tubules. Glomeruli were sparse, small, and located within a dilated Bowman's capsule. The liver was characterized by marked periportal fibrosis, biliary hyperplasia, and bile duct ectasia with dilated ducts...

BACKGROUND Patients with end-stage renal failure due to huge autosomal dominant polycystic kidney disease usually have an umbilical hernia and rectus abdominis diastasis, which are very troublesome. Pretransplant bilateral nephrectomy techniques does not manage the umbilical hernia and rectus abdominis diastasis. We report our experience in performing bilateral nephrectomy and repairing the rec...

INTRODUCTION We report an instructive case of incidental renal cell carcinoma in a patient with autosomal dominant polycystic kidney disease who underwent simultaneous bilateral native nephrectomy and living donor renal transplantation. CASE PRESENTATION A 57-year-old Asian man with end-stage kidney disease due to autosomal dominant polycystic kidney disease received a living kidney graft fro...