Diagnostic Considerations

An important variant of primary hypodipsia is the syndrome of essential hypernatremia. An impaired osmotic regulation of aqueous vasopressin (AVP) and thirst characterizes essential hypernatremia; however, AVP synthesis and secretion is intact in response to hemodynamic stimuli. Patients may present with mild stable elevations of serum sodium concentration, consistent with the upward regulation of the osmostats, or with severe fluctuations in serum sodium concentration, consistent with complete loss of osmoregulatory function.

Differential Diagnoses

Workup

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Media Gallery

Anatomic relationships between pituitary and hypothalamic areas of interest with respect to regulation of antidiuretic hormone (ADH) secretion and thirst sensation. AN = Anterior (hypothalamic) nucleus; AP = Anterior pituitary; OC = optic chiasm; OVLT = Organum vasculosum of lamina terminalis; PA = Preoptic (hypothalamic) area; PP = Posterior pituitary; PVN = Paraventricular (hypothalamic) nucleus; SON = Supraoptic (hypothalamic) nucleus.

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Author

Vikas R Dharnidharka, MD, MPH Professor and Chief, Division of Pediatric Nephrology, Hypertension and Pheresis, Vice-Chair for Clinical Investigation, Department of Pediatrics, St Louis Children's Hospital, Washington University in St Louis School of Medicine

Vikas R Dharnidharka, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: CareDx<br/>Received research grant from: CareDx.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Adrian Spitzer, MD Clinical Professor Emeritus, Department of Pediatrics, Albert Einstein College of Medicine

Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD Tenured Professor of Pediatrics, The First Isaac A Abt, MD, Professor of Kidney Diseases (Emeritus), Northwestern University, The Feinberg School of Medicine; Staff Nephrologist, The Ann and Robert H Lurie Children’s Hospital of Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Clinical Research, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Pediatric Nephrology, Association of Clinical Scientists, Cochrane Collaboration, International Bone and Mineral Society, International Conferences on Calcium Regulating Hormones, International Pediatric Nephrology Association, International Society of Nephrology, International Society of Renal Nutrition and Metabolism, Midwest Society for Pediatric Research, Pediatric Academic Societies, ROCK (Research on Calculus Kinetics) Society, Society for Pediatric Research

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Horizon Pharmaceuticals); Dicerna Pharmaceuticals<br/>Incyte Pharmaceuticals; Eli Lilly for: Federation bio; Dicerna pharmaceuticals.

Additional Contributors

Uri S Alon, MD Director of Bone and Mineral Disorders Clinic and Renal Research Laboratory, Children's Mercy Hospital of Kansas City; Professor, Department of Pediatrics, Division of Pediatric Nephrology, University of Missouri-Kansas City School of Medicine

Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research

Disclosure: Nothing to disclose.

Adipsia Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

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