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  lüll Physiopathology of hereditary polyuric states: a molecular view of renal  function Bichet DGSwiss Med Wkly  2012[]; 142 (ä): w13613Recent discoveries have shed new light on the understanding of water metabolism:  (1.) in addition to hypothalamic osmoreceptor cells expressing a TRPV1 variant,  there are peripheral TRPV4 receptors sensing tonicity in the portal vein and  changing central vasopressin secretion and peripheral autonomic activity; (2.)  the central osmoregulatory gain of angiotensin action participates in the  non-osmotic release of vasopressin induced by hypovolaemia; (3.) prostaglandins  EP2 receptors on principal cells of the collecting ducts positively regulate  urine concentration mechanisms. These new developments are important clinically  for the understanding of hereditary polyuric states. We recommend sequencing of  the nephrogenic diabetes insipidus genes in all affected patients. This genomic  information is key to the routine care of patients with congenital polyuria and,  as in other genetic diseases, reduces health costs and confers psychological  benefits on patients and families.|Arginine Vasopressin/biosynthesis/physiology[MESH]|Diabetes Insipidus/*genetics/physiopathology[MESH]|Humans[MESH]|Kidney/*physiopathology[MESH]|Neurons, Afferent/metabolism[MESH]|Osmolar Concentration[MESH]|Polyuria/genetics/*physiopathology[MESH]|Sodium/blood[MESH]|TRPV Cation Channels/metabolism[MESH]|Water-Electrolyte Balance[MESH] |