SUMMARY Eighteen males, 17 of whom were members of a single family, affected with angiokeratoma corporis diffusum were examined in detail to determine the extent of clinical variation of the expression of what was almost certainly the same X-linked mutation in each. The commonest symptom was episodic bouts of severe, painful dysaesthesia in hands and feet. This was a major complaint of 12, a minor complaint of 5, and absent in 1. In over half the subjects, the skin rash that is considered a characteristic sign of the disease was absent or inconspicuous. All exhibited mild clubbing of fingers and toes, and 15 showed variable limitation of active and passive extension of the 5th fingers bilaterally. Only 2 (aged 36 and 47) had evidence of significant renal disease. Electrocardiograms showed abnormally short PR intervals in 4, and right ventricular conduction disturbances in 5.
The effects of centrifugation for various lengths of time were investigated on circulating levels of luteinizing hormone (LH) and testosterone in male rats. In a chronic 52-day experiment, centrifugation at 4.1 G significantly reduced LH and testosterone levels for the entire period. Centrifugation at 2.3 G had less effect inasmuch as LH levels were not significantly decreased and testosterone levels were significantly reduced only during the first few days of centrifugation. In more acute experiments, centrifugation at 4.1 G for 4 h resulted in reduced testosterone levels, whereas centrifugation for 15 min did not significantly alter the hormone levels. These results indicate that centrifugation can decrease circulating LH and testosterone levels if the gravitational force is of sufficient magnitude and is maintained for a period of hours. Chronic centrifugation may also inhibit the acute excitatory response of LH to handling and ether stress.
Reproductive function and sexual behavior were examined in female rats subjected to a restricted watering schedule. In 4- or 5-day cycling rats, water was supplied only from 09.00 to 10.00 h each day for a minimum of 1 month. A tendency towards prolonged estrous cycles was noted, with 5-day cycles predominating, regardless of the pretreatment cyclical pattern. Water-restricted 5-day rats became sexually receptive earlier on the day of proestrus than did controls. Barbiturate was more effective in blocking ovulation and the plasma LH concentration was lower at 16.00 h on the day of proestrus following water restriction. Plasma corticosterone levels were elevated just prior to watering. Immature females placed on the same restricted watering schedule as the adults showed no significant difference in age at vaginal opening, a delay in the appearance of the first estrus, and significantly more 5-day cycles than controls. It is suggested that the changes observed are related to alterations in adrenocortical activity mediated by the action of adrenal progesterone and/or corticosteroids on gonadotropin secretion and sexual behavior.
Plasma levels of LH were measured in young sexually mature (2- to 5-month-old) and aged (13- to 20-month-old) female C57BL/6 mice, Syrian hamsters and Wistar rats using a radioimmunoassay (RIA) and a radioreceptor assay (RRA). There were no statistically significant differences when comparing data from the two assays when examining young oestrous and dioestrous mice. Aged oestrous and dioestrous mice exhibited significantly higher levels of LH as measured by RIA than by RRA. Levels of LH analysed by RIA were also higher in aged mice compared with those in younger mice. Comparing LH hamsters produced similar results. In contrasts, aged pseudopregnant rats exhibited significantly lower levels of plasma LH than younger dioestrous females and there were no differences in RIA and RRA values. There were also no differences when comparing data from the two assays in younger rats. The mean (+/- S.E.M.) RRA: RIA ratios of 0.96 +/- 0.05 (oestrous) and 0.96 +/- 0.04 (dioestrous) for younger mice and 0.92 +/- 0.08 for younger hamsters were significantly higher than the ratios of 0.66 +/- 0.05, 0.72 +/-0.05 and 0.71 +/- 0.05 respectively of their aged counterparts. The mean RRA: RIA ratios in the two age groups of rats were almost identical (0.79 +/- 0.05 and 0.80 +/- 0.10). These studies suggested that a portion of the higher LH levels detectable by RIA in some aged female rodents results from qualitative changes in the LH molecule.
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