The immunosuppressive agent sirolimus exerts an antiproliferative effect by inhibiting mammalian target of rapamycin (mTOR). Because excessive proliferation of the biliary epithelium is a prominent feature of the polycystic liver that accompanies autosomal dominant polycystic kidney disease (ADPKD), we hypothesized that sirolimus may benefit patients with this disorder. We retrospectively measu...

The basic biochemical mechanisms underlying many heritable human polycystic diseases are unknown despite evidence that most cases are caused by mutations in members of several protein families, the most prominent being the polycystin gene family, whose products are found on the primary cilia, or due to mutations in posttranslational processing and transport. Inherited polycystic kidney disease,...

Objective: We examined the relationship between insulin resistance and vascular function in three insulin resistant states (type 2 diabetes mellitus, non-HIV lipodystrophic diabetes, and non-diabetic polycystic ovary syndrome (PCOS)) and in healthy controls. Research Design and Methods: The population included 12 women with type 2 diabetes, 6 with lipodystrophic diabetes, 10 with polycystic ova...

BACKGROUND Liver cysts have been estimated to occur in 5% of the population. Multiple liver cysts can also be part of the polycystic disease complex. Only symptomatic or complicated cysts need surgery. Traditionally, laparotomy is the procedure of choice. We present our experiences with laparoscopic management of both symptomatic multiple liver cysts and polycystic liver disease. METHODS Betw...

Polycystic ovarian syndrome (PCOS) is the most common female endocrine disorder, affecting approximately 5%-10% of all females and 4-6% of adolescent girls and young women. PCOS is a hormonal disorder that involves multiple organ systems within the body and is believed to be fundamentally caused by insensitivity to insulin.1 It can be diagnosed in all phases of life in girls as young as 8-9 yea...

OBJECTIVES: Polycystic ovary syndrome is a heterogeneous endocrine disorder that affects reproductive-age women. The mechanisms underlying the endocrine heterogeneity and neuroendocrinology of polycystic ovary syndrome are still unclear. In this study, we investigated the expression of the kisspeptin system and gonadotropin-releasing hormone pulse regulators in the hypothalamus as well as facto...

UNLABELLED To evaluate the relevance of body mass index (BMI), luteinizing hormone (LH), follicle-stimulating hormone (FSH) and LH/FSH ratio with clinical symptoms in polycystic ovary syndrome (PCOS) women. METHODS We reviewed the medical records of all women visited in the PCOS Clinic of Babol (Iran) from 2008 to 2012. A retrospective cross-sectional study was conducted on 175 PCOS women; ag...

BACKGROUND Polycystic ovarian syndrome (PCOS) is a controversial endocrine pathology and, recently, it has been suggested that the condition is hereditary. The aim of this study was to prospectively determine in daughters of patients with PCOS, by ultrasonographic (US) and colour Doppler analyses, the incidence of polycystic ovaries and search any correlation with specific hormonal parameters. ...

OBJECTIVE Limited data suggest that menstrual cycle abnormalities are more pronounced in younger and more obese patients with polycystic ovary syndrome (PCOS). We aimed to evaluate the association between menstrual cycle pattern and age, obesity and PCOS phenotype in a large population of women with PCOS. DESIGN We studied 1,297 women with PCOS and divided them according to: a) age in ≤ 20, 2...

Autosomal recessive polycystic kidney disease is a truly catastrophic monogenetic disease, causing death and end stage renal disease in neonates and children. Using PCK female rats, an orthologous model of autosomal recessive polycystic kidney disease harboring mutant Pkhd1, we tested the hypothesis that intravenous renal cell transplantation with normal Sprague Dawley male kidney cells would i...