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ADPKDAutosomal Dominant Polycystic Kidney Disease
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ADPKD is thought to affect around 60,000 people in the UK and 205,000 people in Europe, and leads to the development of multiple fluid-filled cysts in the kidneys, which can result in chronic and acute pain, hypertension and kidney failure.
A 45-year-old woman with ESRD secondary to ADPKD was evaluated for native nephrectomy due to insufficient space within the iliac fossa for renal transplantation (Fig.
Tolvaptan is a selective V2 vasopressin receptor antagonist that has been hypothesized to slow the progression of ADPKD by reducing the development and growth of kidney cysts, which are characteristic of the disease and often associated with pain, hypertension, decreased kidney function and ultimately, kidney failure.
It turns out that ADPKD is due to a mutation in a gene that directs the synthesis of a protein found in the cell membrane, which is a critical component of the primary cilia.
Early research reveals that certain signals within cells may contribute to cyst growth in ADPKD patients.
Estimates of the frequency of aneurysms in ADPKD patients range from 10% to 41%.
ADPKD is the most common inherited cause of kidney failure, accounting for about 5 percent of end-stage renal disease in the U.
1] The company reached an agreement with the United States Food & Drug Administration (FDA) on a Special Protocol Assessment (SPA) for the design and planned analysis of this multi-center, placebo-controlled, double-blind, parallel-group trial designed to compare the efficacy and safety of tolvaptan (45 to 120 mg per day split-dose) in subjects with chronic kidney disease between late stage 2 and early stage 4 due to ADPKD.
The researchers wanted to know how liver-only polycystic disease was related to ADPKD.
4] but only 4 case reports of ADPKD and Caroli's disease, with 2 reported postoperative mortalities (Table 1).
Nevertheless, in the recent years of ADPKD study, the tubular renal cell cilia have also been implicated in cystogenesis.
A 41-year-old woman with ESRD secondary to ADPKD was treated by dialysis for 10 years, including continuous ambulatory peritoneal dialysis (CAPD) for 1 year, followed by haemodialysis.