Defective thirst mechanism secondary to a hypothalamic lesion: Studies in a child with adipsia, polyphagia, obesity, and persistent hyperosmolality

Adipsic diabetes insipidus following pituitary surgery for a macroprolactinoma

“…In addition there is considerable morbidity associated with this rare condition, including polyphagia and obesity [22], sleep apnoea [10,22], hypersomnolence [23] and venous thrombosis [24] during episodes of dehydration. Adipsic diabetes insipidus should be considered to be a rare complication of extensive surgery for large pituitary macroadenomas.…”

Hypertriglyceridemia in Infants and Children with Hypernatremia

“…(18)(19)(20)(21)(22)(23)(24)(25)(26)(27) Serum lipids are rarely measured in patients with hypernatremia. Hypertriglyceredemia in association with hypernatremia was reported in few children.…”

“Essential” hypernatremia due to ineffective osmotic and intact volume regulation of vasopressin secretion

“…Zierler, in reviewing this subject in 1958, concluded that this increased total solute concentration of the body fluids was usually the result of relatively deficient fluid intake in obtunded or comatose patients rather than a specific distortion of water regulating mechanisms (1). While this is true in most instances, a group of patients with neurologic lesions and sustained hyperosmolality has been described with more complex and specific disturbances in water metabolism (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). In such patients lesions involving the hypothalamic-neurohypophyseal area have frequently been documented (2-7, 10-12, 14, 17, 18) or strongly suspected from associated clinical findings (8,9,13,16).…”

“…In such patients lesions involving the hypothalamic-neurohypophyseal area have frequently been documented (2-7, 10-12, 14, 17, 18) or strongly suspected from associated clinical findings (8,9,13,16). Abnormalities in water metabolism associated with such lesions appear to result from (a) impaired thirst (2)(3)(4)(5)(6)(7)19), (b) impaired antidiuretic hormone production (diabetes insipidus) (20), or (c) altered regulation of antidiuretic hormone (ADH) secretion (21). Since the neuronal "centers" for thirst and ADH production and secretion lie in close proximity to each other in the anterior hypothalamus (22), these disturbances may occur either singly or in combination.…”

“…Since the neuronal "centers" for thirst and ADH production and secretion lie in close proximity to each other in the anterior hypothalamus (22), these disturbances may occur either singly or in combination. Conscious patients with hypothalamic lesions and sustained hyperosmolality have been described in whom adipsia or hypodipsia appeared to be the sole or predominant abnormality (2)(3)(4)(5)(6)(7). In such patients, like those reviewed by Zierler (1), hyperosmolality was primarily the result of inadequate fluid intake and was corrected by fluid administration (2)(3)(4)(5)(6).…”