In this study, we report a model of spontaneous cyst formation in vitro and a procedure to obtain large quantities of cysts from polycystic rat kidney cells. Furthermore, we assess the effects of epidermal growth factor, a modulator of morphogenesis, and of taxol, a stabilizer of microtubules, which has recently been proposed as a useful treatment of human polycystic kidney disease (PKD). It is...

Fifty per cent of the offspring of adults with the adult (dominant) form of polycystic kidney disease are carriers of the abnormal gene. Clinical symptoms and signs before adolescence are rare, but renal ultrasonography may detect evidence of cyst formation. Twenty two children, all offspring of parents with known adult polycystic kidney disease, have undergone renal ultrasonography. In six cas...

BACKGROUND/AIMS Cystic epithelia in polycystic kidney disease display features similar to malignant cells. Thiazolidinediones have been shown to have anti-neoplastic properties, therefore we tested the hypothesis that pioglitazone reduces cyst formation, improves renal function, and prolongs survival in a mouse model of polycystic kidney disease. METHODS PC-Pkd1-KO mice, which have homozygous...

PURPOSE We report our experience with bilateral hand-assisted laparoscopic nephrectomy in patients with adult polycystic kidney disease. MATERIALS & METHODS Between November 2009 and November 2010, 3 patients with adult polycystic kidney disease underwent bilateral hand-assisted laparoscopic nephrectomy in our institution. Indications for bilateral nephrectomy included recurrent cyst hemorrha...

We report a case of an unusual prenatal presentation of polycystic kidneys associated with multiple skeletal limb defects, including polydactyly, syndactyly, bilateral agenesis of the tibia, and club foot. The ultrasonographic picture was consistent with a diagnosis of polycystic kidney disease, either the adult onset autosomal dominant type (ADPKD) or the early onset autosomal recessive form (...

The many hundreds of cysts that grow and expand and ultimately overwhelm and destroy polycystic kidneys arise from the slow but unrelenting proliferation of tubular epithelial cells, eventually giving rise to very large, thin-walled, fluid-filled structures. The growth of these cystic bodies requires two processes: Cell proliferation and fluid secretion. Cyst epithelial cells seem to have a uni...

Short rib-polydactyly syndrome (SRPS) is a group of lethal skeletal dysplasia of an autosomal recessive inheritance characterized by markedly narrow ribs, micromelia, and multiple anomalies of major organs. We report a case of type IV SRPS with uncommon associations of polydactyly and bilateral polycystic kidneys, in a 28 week old female fetus. She was born dead to a 28 year old mother, showing...

Polycystic kidney disease (Autosomal Dominant Kidney Disease, ADPKD) is the most common genetic of kidney, and measurement Total Volume (TKV) in clinical research this essential to study progression ADPKD. At present, volume segmentation polycystic kidneys mainly relies on doctors manually outline boundary radiological image. This process time-consuming, labor-intensive, inefficient, subjective...

Because the kidneys in patients with autosomal dominant polycystic kidney disease (ADPKD) are usually supplied by well-developed arteries, the authors attempted renal contraction therapy in such patients with renal transcatheter arterial embolization (TAE) using intravascular coils. In most patients with marked nephromegaly, renal TAE was effective. However, in patients with marked hepatomegaly...

Autosomal-dominant polycystic kidney disease (ADPKD) is a common cause of end-stage renal disease, and no approved treatment is available in the United States to slow disease progression. The mammalian target of rapamycin (mTOR) signaling pathway is aberrantly activated in renal cysts, and while mTOR inhibitors are highly effective in rodent models, clinical trials in ADPKD have been disappoint...