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Abstract: SA-PO715

Spontaneous Development of Diabetes Insipidus in a Healthy Female: A Rare Case of Central Diabetes Insipidus in the Nephrology Clinic

Session Information

Category: Fluid, Electrolytes, and Acid-Base Disorders

  • 1102 Fluid, Electrolyte, and Acid-Base Disorders: Clinical


  • Mitchell, Allison, The University of Texas Medical Branch at Galveston Development Office, Galveston, Texas, United States
  • Kassem, Hania, The University of Texas Medical Branch at Galveston Development Office, Galveston, Texas, United States

Diabetes Insipidus (DI) is a rare complication resulting from vasopressin (AVP) deficiency [central (CDI)] or AVP resistance [nephrogenic (NDI)] leading to polydipsia, polyuria, and hypernatremia. We present a case of CDI in the nephrology clinic setting including diagnosis, imaging, and treatment.

Case Description

A 46-year-old female presented to nephrology clinic with polydipsia and polyuria that began 3 months prior. Her primary complaints were extreme thirst requiring consumption of approximately 20L of water over 2 to 3 days with large volume urination every hour. Initial laboratory findings were consistent with diabetes insipidus: high-normal sodium (Na) of 145, creatinine of 0.55mg/dL, low urine osmolality of 140 mOsm/kg, and low serum AVP (which resulted 2 weeks later). Due to high suspicion for CDI, patient was started on a trial of oral desmopressin 0.1mg daily which was increased over two weeks to desmopressin 0.2mg BID. Patient subsequently improved with resolution of symptoms and labs showing: normal serum Na (140 mmol/L) with improved urine osmolality (494 mOsm/kg). MRI brain showed diffuse thickening of the pituitary infundibulum in the absence of posterior pituitary bright spot and a small meningioma over the left anterior temporal lobe supporting a diagnosis of Langerhans cell histiocytosis (LCH). Patient was referred to neurology and oncology for further evaluation.


LCH is a rare disorder characterized by abnormal proliferation of Langerhans cells, histiocyte-like cells, of unknown cause. This disorder is rarely seen in adults (1-2 per million) and is twice as likely to be diagnosed in males. LCH affects the central nervous system by affecting the pituitary infundibulum leading to the initial presentation of DI. An elevated serum Na along with dilute urine in a patient with polyuria and polydipsia was strongly suggestive of DI, which led to the decision of work up and treatment without requiring a confirmatory water deprivation test. Desmopressin dosing titration required close monitoring with patient counseling about decreasing fluid intake once urine output decreases and being aware of the signs of hyponatremia. This was a unique case in the outpatient nephrology setting of a rare disease in an unlikely patient demographic.