Abstract
We have previously described high set osmotic threshold (OT) for vasopressin release and deficient thirst in a patient with KS (ESPE-meeting 1980). In the present study the OT was evaluated by an isovolemic infusion of 5% NaCl in 7 patients with KS. The OT was defined at the plasma osmolality (Posm) at which the free water clearance (CH2O) abruptly fell. The OT was abnormally high in 5 patients, 291-296mOsm/kg. It was normal in one, 287mOsm/kg, and abnormally low in one patient, 270mOsm/kg. These values are compared to the OT in 73 normal volunteers previously reported (A.M.M & M.M), 287.3 ± 0.9(SD) mOsm/kg. The 5 patients with high set OT denied thirst at Posms as high as 296-316mOsm/kg. Normal response of volume regulation of vasopressin release was demonstrated by an appropriate fall in CH2O during water deprivation. Normal response of baroreceptors was shown by a fall in CH2O and a rise in plasma ADH during infusion of the hypotensive agent trimethaphan in 2 patients. It is concluded that the hypothalamic involvement in KS include deficient thirst sensation and abnormal setting of the osmoreceptors, that can be at an abnormal high or low setting.
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Hochberg, Z., Moses, A., Benderli, A. et al. Abnormal osmoreceptors in Kallman's syndrome (KS). Pediatr Res 15, 1562 (1981). https://doi.org/10.1203/00006450-198112000-00161
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DOI: https://doi.org/10.1203/00006450-198112000-00161
