No abstract available.
Diagnosis* ; Polycystic Kidney, Autosomal Dominant*

No abstract available.
Hernia, Obturator* ; Polycystic Kidney, Autosomal Dominant*

No abstract available.
Carcinoma, Renal Cell* ; Kidney Diseases* ; Kidney* ; Polycystic Kidney, Autosomal Dominant

No abstract available.
Carcinoma, Renal Cell* ; Humans ; Polycystic Kidney, Autosomal Dominant* ; Translocation, Genetic

PURPOSE: The purpose of this study was to determine the value of the intrarenal resistive index (RI),measured by Doppler sonography, in order to assess intrarenal vascular resistance in autosomal dominant polycystickidney disease (ADPKD) patients. MATERIALS AND METHODS: In 26 patients with ADPKD, RI was measured by Dopplersonography and correlated with the presence of hypertension, renal function (creatinine clearance) and anatomicalrenal severity index (RSI), thus indicating renal morphologic abnormalities during B-mode sonography . RESULTS:RI was significantly higher in 18 hypertensive ADPKD patients (0.64+/-0.65) (Mean+/-1SD; range: 0.52-0.74) than ineight normotensive patients (0.59+/-0.50) ( 0.48-0.64) (p<0.05). Statistically significant inverse correlation wasfound between RI values and creatinine clearance (r=-0.45, p<0.05), and statistically significant correlation wasfound between RI values and RSI, indicating the degree of renal parenchymal involvement. CONCLUSION: RIcorrelates with the development of hypertension, renal function and renal morphologic abnormality scoring by RSIduring B-mode Doppler sonography, and measured in this way may thus be used to assess renal vaseular resistance inADPRD patients.
Creatinine ; Humans ; Hypertension, Renal ; Polycystic Kidney, Autosomal Dominant* ; Vascular Resistance

Because autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic abnormalities seen in today's medical practice, many internists will likely treat patients affected by this condition. Genetic abnormalities have been increasingly recognized, and the pathophysiology of the disease is beginning to be unraveled. Because of advances in imaging technology, surrogate markers for disease progression have allowed clinical studies of newer therapeutic agents to proceed. In the near future, therapies for this common genetic disease may be available to either prevent or stabilize the disease course for many affected individuals.
Humans ; *Polycystic Kidney, Autosomal Dominant/complications/diagnosis/genetics/therapy ; Prognosis

The discovery of the genes and their respective proteins that are associated with autosomal dominant polycystic kidney disease (ADPKD) has revolutionized the field of ADPKD biology. Recent studies indicate that the pathogenesis of ADPKD is linked to abnormalities in the primary cilium in the kidney. Inactivation of ciliary proteins in the postnatal kidney has uncovered novel roles of primary cilia in regulating tubular growth and repair after injury. Furthermore, defective tubular repair after injury may contribute to the progression of ADPKD. Studies of signaling pathways that are perturbed in ADPKD have identified potential targets for pharmacological therapy. Better understanding of the downstream consequences of ADPKD mutations has identified a number of therapeutic targets that are now being tested in preclinical and clinical trials. The author summarized recent insights in the pathogenesis of ADPKD including the genetics of ADPKD, the properties of the respective polycystin proteins, the role of cilia, some cell-signaling pathways and new therapeutic interventions.
Biology ; Cilia ; Kidney ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant ; Proteins ; Vasopressins

Some cases of renal malignancy associated with adult (autosomal dominant) polycystic kidney disease have been reported. Most of these malignancies were diagnosed as renal cell carcinoma. But the case of transitional cell carcinoma has not been reported. We report a case of renal pelvic and urethral transitional cell carcinoma associated with adult polycystic kidney.
Adult* ; Carcinoma, Renal Cell ; Carcinoma, Transitional Cell* ; Humans ; Kidney ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant*

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease. There are some reports in the literature concerning unilateral ADPKD. However, in adults, only a few cases of unilateral ADPKD with agenesis of contralateral kidney have been reported. We present a case of unilateral ADPKD with agenesis of contralateral kidney in a 66-yr-old man. Radiographic images showed the enlarged right kidney with multiple variable-sized cysts and the absence of the left kidney. The diagnosis of ADPKD was confirmed by the family screening. The patient received maintenance hemodialysis for endstage renal disease. We report a case of unilateral ADPKD associated with contralateral renal agenesis in a 66-yr-old male patient with a literature review.
Abdomen/pathology ; Aged ; Female ; Human ; Kidney/abnormalities* ; Male ; Pedigree ; Polycystic Kidney, Autosomal Dominant/diagnosis* ; Polycystic Kidney, Autosomal Dominant/pathology* ; Polycystic Kidney, Autosomal Dominant/physiopathology ; Radiopharmaceuticals/metabolism ; Technetium Tc 99m Dimercaptosuccinic Acid/metabolism

Kidney transplantation is the preferred treatment in end stage renal disease for autosomal dominant polycystic kidney disease (ADPKD) patients. Removal of the native kidney is not usually recommended for ADPKD patients during a transplantation procedure because the operation time may be prolonged or the risk of bleeding may be higher. Therefore, native kidney removal is indicated for patients with chronic pain by enlarged kidney, frequent complications from cysts, such as infection or bleeding, and renal tumor development. Here, we report a case of a patient whose native kidneys were removed during a kidney transplantation procedure, and multifocal adenomas were identified in the removed kidneys after the procedure.
Adenoma* ; Chronic Pain ; Hemorrhage ; Humans ; Kidney ; Kidney Failure, Chronic ; Kidney Transplantation* ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Dominant*