We report the case of a 49-year-old lady who presented with hypertension, breathlessness and malaise. She was thrombocytopenic, with polycystic kidneys on imaging, and was found to have nephrotic syndrome. Serological results were consistent with systemic lupus erythematosus (SLE) and a renal biopsy confirmed WHO class V lupus nephritis. This is the first reported case of nephrotic syndrome due...

AIM Autosomal dominant polycystic kidney disease (ADPKD) is characterized by an imbalance between tubular epithelial cell proliferation and apoptosis. We have previously shown that the mammalian target of rapamycin (mTOR) signalling pathway is aberrantly activated in the cystic kidneys of Han:SPRD rats with ADPKD. Because the Akt kinase is an upstream regulator of mTOR, we hypothesized that the...

Dear Sir, Autosomal dominant polycystic kidney disease (ADPKD) is a multi-systemic and progressive disorder characterized by formation of cysts in ductal organs particularly the kidneys, liver, pancreas, gastro-intestinal tract, the central nervous system and the cardiovascular system.[1,2] The disease may occur anytime in life, including in utero in contrast with its previous name “adult polyc...

We present a case of autosomal recessive polycystic kidney disease diagnosed at 28 weeks' gestation by ultrasonographic examination and magnetic resonance imaging (MRI). The fetal kidneys were symmetrically enlarged and highly echogenic by ultrasonographic examination and showed high-signal intensity on T2-weighted images by MRI. Cystic lesions were recognized by neither examination. In additio...

Polycystic kidney disease (PKD) is a genetic disorder characterized by aberrant renal epithelial cell proliferation and formation progressive growth of numerous fluid-filled cysts within the kidneys. Previously, we showed that there elevated Notch signaling compared to normal cells contributes cystic cells. Quinomycin A, bis-intercalator peptide, has previously been shown target pathway inhibit...

Autosomal dominant polycystic kidney disease, a leading cause of end-stage renal disease in adults, is characterized by progressive focal cyst formation in the kidney. Embryonic lethality of Pkd1-targeted mice limits the use of these mice. Here we developed a floxed allele of Pkd1 exons 2-6. Global deletion mutants developed polyhydramnios, hydrops fetalis, polycystic kidney and pancreatic dise...

Autosomal dominant polycystic kidney disease (ADPKD) is a genetically heterogeneous disorder characterized by focal cyst formation from any part of the nephron. The molecular bases include germinal mutation of either PKD1 or PKD2 genes, enhanced expression of several protooncogenes, alteration of the TGF-alpha/EGF/EGF receptor (EGFR) axis, and disturbed regulation of proliferative/apoptosis pat...

Renal Lymphangiectasia (RLM) is a very infrequent benign lymphatic disorder. RLM often misdiagnosed for other disorders suchas cystic renal masses, usually polycystic kidneys. Although parenthetically found in different cases, probable cause of renalfailure and hypertension undiagnosed victims. The etiology unclear results from the formation masses anddilatation perinephric channels. In sinus, ...

Ali, Shujath M., Victoria Y. Wong, Kristine Kikly, Todd A. Fredrickson, Paul M. Keller, Walter E. DeWolf, Jr., Dennis Lee, and David P. Brooks. Apoptosis in polycystic kidney disease: involvement of caspases. Am. J. Physiol. Regulatory Integrative Comp. Physiol. 278: R763– R769, 2000.—Polycystic kidney disease (PKD) is characterized by the development of large renal cysts and progressive loss o...

Expression of kidney injury molecule-1 (Kim-1) is rapidly upregulated following tubular injury, constituting a biomarker for acute kidney damage. We examined the renal localization of Kim-1 expression in PKD/Mhm (polycystic kidney disease, Mannheim) (cy/+) rats (cy: mutated allel, +: wild type allel), an established model for autosomal dominant polycystic kidney disease, with chronic, mainly pr...