BONE AND CALCIUM-METABOLISM IN WERNERS-SYNDROME

In order to compare the bone aging process in Werner's syndrome, a dis ease characterized by premature aging, and that in natural aging, we h ave assessed the bone status in a total of 19 cases (11 males and eigh t postmenopausal females) with Werner's syndrome. The spinal deformity index was determined for a total of 87 vertebral bodies from nine mal e patients and for a total of 61 vertebral bodies from six female pati ents. In the male patients, 15 vertebral deformities among 87 vertabra e in seven patients were observed, and the incidences of patients bear ing deformity and deformed vertebrae were 77.8 (7/9) and 17.2% (15/87) , respectively. In the female patients, the corresponding incidences w ere 50.0% (3/6) and 4.9% (3/61), respectively. The chi(2)-values for t he incidences of patients bearing deformity and deformed vertebrae in the male and the female patients were 1.25 (P = 0.26) and 5.24 (P < 0. 03), respectively. The metacarpal cortical thickness (MCI) was also me asured on hand X-ray films, and the Z scores for MCI in the male (n = 9) and female (n = 3) patients were -0.8 +/- 0.3 and -1.6 +/- 0.3, res pectively. This gender-based difference in the MCI was not statistical ly significant but, the Z scores for MCI in both the males and the fem ales were significantly smaller than those of corresponding age-matche d controls (P < 0.05 and 0.01, respectively). The serum levels of calc ium, intact PTH, 25-OH vitamin D and 1,25(OH)(2) vitamin D in three ma le and three female Werner's syndrome patients were within the referen ce ranges with one exception. The serum intact osteocalcin levels in t he male (2.2 +/- 0.6 ng/ml) and female (2.6 +/- 0.8 ng/ml) patients we re lower than the age-matched control values (P < 0.05 for males) and the postmenopausal control values (P < 0.05 for females). In conclusio n, the incidence of spinal bone deformity was higher in males with Wer ner's syndrome than that in female patients. The cortical bone thickne ss decreased in both the male and the female patients. A low serum int act osteocalcin level was a characteristic finding in the male and in post-menopausal Werner's syndrome, so that the bone changes such as ve rtebral fracture incidence and the serum intact osteocalcin level in W erner's syndrome may differ from those in normal aging in the general population.