Polycystic Kidney Disease in an Elderly Male Patient: A Case Report
Sukumar Naidu *
Department of Research, Healthcare Center, Visakhapatnam, Andhra Pradesh, India.
Mercy Paul
Department of Research, Healthcare Center, Visakhapatnam, Andhra Pradesh, India.
*Author to whom correspondence should be addressed.
Abstract
Polycystic kidney disease truncated as PKD/PCKD, a prominently serious inherited disorder in which groups of cysts (non-cancerous non-functional round fluid filled sacs) formed in the kidney causing abnormal renal tubule structure, making them enlarge and lose their ideal function. The 2 proteins (polycystin 1,2) together form a receptor-ion-channel complex on primary cilia of renal endothelial and epithelial cells. PKD is of 2 types i.e. autosomal recessive and autosomal dominant PKD (ARPKD and ADPKD). The actual reason for cyst formation is not clearly understood but is thought to be related to distortions in signaling pathways that are regulated by intracellular calcium, cyclic adenosine monophosphate (cAMP), Wnt/β-catenin, or planar cell polarity (PCP). The 2 genes effected in ADPKD are PKD1 account for 78% of cases and PKD2 around 15% of cases. About 50% of cases are inherited to ADPKD condition whereas 25% of cases are non-inherited. In the progressed disease state, abnormal cell proliferation causes persistent tubular dilation, fluid secretion (sodium retention), and detachment from main tubule to from cysts.
Keywords: Polycystic, dominant, polycystin, signaling cascade, ADPKD, multiple cysts, arginine vasopressin