Approximately half of people with ADPKD
will require a kidney transplant or dialysis by the age of 54.
To our knowledge, this is the first description of a pure laparoscopic approach to unilateral nephrectomy for ADPKD
in a horseshoe kidney.
Tolvaptan was discovered by Otsuka in Japan, and, if approved by the FDA, would become the first pharmaceutical therapy for patients who suffer from ADPKD
We now understand that ADPKD
and a number of other hereditary kidney diseases are all due to defective cilial proteins.
The trial examined how six months of treatment with sirolimus or conventional therapy affected different measures of kidney volume in 21 patients with ADPKD
who had normal or slightly impaired kidney function.
Between 18% and 22% of ADPKD
patients with intracranial aneurysm have a family history positive for aneurysm.
Tolvaptan is a selective V2 vasopressin receptor antagonist, which had 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 and kidney failure.
The trial, which will take place at approximately 220 sites worldwide, is seeking to enroll approximately 1,300 tolvaptan-naE[macron]ve adult (male and female) patients with ADPKD
between the ages of 18-66, depending on their estimated glomerular filtration rate (eGFR), a renal function test, at time of enrollment.
The data suggest the exciting possibility that targeting the activity of PKD1 may be beneficial for treatment of isolated polycystic liver disease, childhood recessive polycystic kidney disease and even a subset of adult ADPKD
," Somlo said.
As far as his family history is concerned, the patient's father left the family and his mother's side is negative for ADPKD
and Caroli's disease.
Genes associated with proliferation, such as PCNA, c-Myc, and Ki-67, have been found at increased levels in ADPKD
cystic epithelial cell cultures and human cystic tissues.
Profile of patients haemodialysed via IVC catheter Patient Age/sex Nephropathy Indication 1 41/F ADPKD
Occlusive veins 2 28/M Unk Vein thrombosis 3 40/M Unk Occlusive vein 4 20/M CGN Vein thrombosis 5 30/M CGN Vein thrombosis 6 27/F CIN Vein occlusion 7 32/F Unk Vein occlusion Patient Durat.