Abstract: |
Background: Combined deficits in arginine vasopressin secretion (AVP) and thirst sensation can result in life threatening hyperosmolality and hypernatremia. Complications include seizures, profound volume contraction and renal failure. For tunately, this is an un common clinical condition, with approximately 70 cases reported in the literature over the past 47 years [1]. Defects in AVP secretion and/or synthesis produce central diabetes insipidus (DI), polyuria with polydipsia, hypernatremia and hyperosmolality. Most awake and alert patients with an intact thirst stimulus will "drink" them selves back to a normal serum sodium and osmolality. How ever, if there is concomitant destruction of the osmoreceptors that regulate thirst, osmolal and volume homeostasis can not be maintained. The relationships between urine osmolarity and serum osmolarity and plasma vasopressin levels are vital for distinguishing a reset osmostat from central DI. Methods: After obtaining approval from our institutional review board, we retro spectively re viewed the medical record of a 37-year-old patient who presented to our institution with a serum sodium of 176 mEq/l. Results: Admission laboratory examination revealed: hemoglobin 12.8 g/dl; white blood cell count 4.7 × 103/μl, with a normal differential; random serum glucose 91 mg/dl; sodium 176 mEq/l; plasmaosmolality 366 mOsm/kg; BUN 33 mg/dl; serum creatinine 1 mg/dl; cal cium 9.5 mg/dl; urine specific gravity 1.032; and urine osmolality 1,172 mOsm/kg. An MRI with contrast of the sella/pituitary revealed an enhancing mass centered within the suprasellar cistern and an terior third ventricle, measuring 3.0 × 3.9 × 3.4 cm. The lesion appeared to involve the hypothalamus and displaced the opticchiasm in feriorly. Evalua of pituitary function revealed normal serum levels of thyroid stimulating hormone, AM cortisol, luteinizing hormone, follicle stimulating hormone and prolactin. Figure 1 illustrates the relationship between measured serum AVP levels and serum osmolality. Fig ure 2 shows the re la tionship be tween mea sured urine and se rum osmolality. If the se rum AVP lev els were not avail able, it would ap pear as though the patient had a re set osmostat. The kid neys ap pear to appropriately gener ate maximally concentrated urine at a se rum osmolality above 348 but are unable to below this value. Conclusions: When compared with the normal curve, our patient's AVP levels were lower than ex pected for the corresponding osmolality. This pattern is consistent with a partial cen tral DI. She does not have a reset osmostat. In the presence of significant volume contraction and a reduced GFR, her kidneys produced more concentrated urine despite mark edly de creased central vasopressin production. As the volume con traction abated and the GFR improved, polyuria recurred, de spitepersis tent hyperosmolarity and hypernatremia. © 2011 Dustri-Verlag Dr. K. Feistle. |