pituitrin and Empty-Sella-Syndrome

Topics: Aged; Drug Therapy, Combination; Empty Sella Syndrome; Female; Humans; Injections, Spinal; Leukemia, Promyelocytic, Acute; Magnetic Resonance Imaging; Neurophysins; Protein Precursors; Vasopressins

A 63-year-old female with known empty sella syndrome underwent coronary artery bypass grafting surgery. She became hypotensive immediately postoperatively and this did not respond to fluid resuscitation and inotropic therapy. Surgical re-exploration was undertaken and did not reveal any surgical cause. Pulmonary artery catheterisation confirmed a profound vasodilatory component to her shock. We believe this was due to unmasking of posterior pituitary hypofunction, in particular vasopressin insufficiency, due to metabolic stress. This rapidly corrected with an exogenous vasopressin infusion.

Topics: Coronary Artery Bypass; Empty Sella Syndrome; Female; Humans; Hypotension; Middle Aged; Shock, Surgical; Vasopressins

Hyponatremia as the presenting manifestation of empty sella syndrome is rare. There is little clinical experience in the management of this problem and its possible therapeutic complications. We herein report on a 44-year-old woman with a past history of massive postpartum hemorrhage who was admitted because of hyponatremia and disturbed consciousness. Initial biochemical data suggested the effects of antidiuretic hormone, but fluid restriction alone offered limited benefit. Later, hormonal levels indicated hypopituitarism. Magnetic resonance imaging and cisternography led to a diagnosis of empty sella. Although glucocorticoid substitution was initiated and the clinical condition initially improved, possible myelinolysis subsequently became a complication. With early recognition and immediate replacement of hypotonic fluid, the patient completely recovered. We report this case to illustrate the fact that glucocorticoid substitution and concurrent fluid restriction can probably lead to myelinolysis in empty sella syndrome patients. We suggest that the serum sodium level should be frequently monitored and that much more attention should be paid to the neurologic signs when substituting glucocorticoids in these patients, even though the increment in the serum sodium level is acceptable. Once possible myelinolysis develops, early recognition is critical, and the immediate replacement of hypotonic fluid is suggested.

Topics: Adult; Empty Sella Syndrome; Female; Humans; Hyponatremia; Myelinolysis, Central Pontine; Vasopressins

Gitelman disease was diagnosed in two unrelated children with hypokalemic metabolic alkalosis and growth failure (a boy and a girl aged 7 mo and 9.5 y, respectively, at clinical presentation) on the basis of mutations detected in the gene encoding the thiazide-sensitive NaCl cotransporter of the distal convoluted tubule. GH deficiency was demonstrated by specific diagnostic tests in both children. Hypertonic saline infusion tests showed a partial vasopressin deficiency in the girl and delayed secretion of this hormone in the boy. Magnetic resonance imaging revealed an empty sella in both cases. Up to now, hypomagnesemia and hypocalciuria have been considered obligatory criteria for the diagnosis of Gitelman disease; however, our two patients had hypomagnesemia and hypocalciuria in less than half the determinations. GH replacement treatment was associated with a good clinical response in both children. It appears that these cases represent a new phenotype, not previously described in Gitelman disease, and that the entity may be considered a new complex hereditary renal tubular-pituitary syndrome.

Topics: Bartter Syndrome; Carrier Proteins; Child; Empty Sella Syndrome; Female; Human Growth Hormone; Humans; Kidney Tubules; Male; Pituitary Gland; Sodium-Potassium-Chloride Symporters; Syndrome; Vasopressins