Positron emission tomography demonstrates restricted myocardial perfusion and decreased flow reserve in areas of ventricular non-compaction in children. The myocardial perfusion defects in non-compacted areas may be the cause of myocardial damage and possibly form the basis of arrhythmias and pump failure.

Bone loss and osteoporotic fractures are common in cardiac transplant recipients. To compare two prophylactic medical regimens after heart transplantation, 26 consecutive heart transplant recipients were randomized to receive either continuous oral calcitriol (0.5 microg/day) combined with nasal salmon calcitonin (200 U/day) for the first 3 months (group A) or intermittent intravenous pamidronate (0.5 mg/kg body weight) every third month (group B). Bone mineral density (BMD) and biochemical indices of bone turnover were measured at baseline and 3, 6, 12, and 18 months after transplantation. The mean pretransplant BMD, measured by dual energy X-ray absorptiometry (DXA) was significantly lower in the patients compared with age-matched healthy controls. During the first year of treatment, rates of bone loss at the lumbar spine and femoral neck were slightly but significantly slower in the patients treated with pamidronate, but there was no longer a significant difference between the two groups after 18 months of heart transplantation. Irrespective of the mode of osteoporosis prevention, osteocalcin levels increased whereas urinary deoxypyridinoline decreased after transplantation, and significant bone loss was observed in both treatment groups. We found no relationship between initial BMD, markers of bone turnover, cumulative glucocorticoid dose, or cyclosporine levels and the rate of bone loss after cardiac transplantation. In summary, we found that the rapid and severe bone loss following heart transplantation could be attenuated by two preventive measures, pamidronate or calcitriol with calcitonin.

In patients with adrenal hirsutism or enzyme deficiencies in steroidogenesis, elevated adrenal androgens could be normalized by dexamethasone. We were interested to see if dexamethasone would be as effective as cyproterone acetate in treating hirsutism in selected patients with adrenal pathogenesis. Therefore 28 patients with hirsutism of adrenal origin or enzyme deficiency were treated cyclically either with cyproterone acetate and ethinylestradiol (2 mg cyproterone acetate + 0.035 mg ethinyl-estradiol days 1-21, +10 mg cyproterone acetate days 1-15) (n = 15) or with 0.25-0.5 mg dexamethasone daily at 10 pm (n = 13). In the dexamethasone group there was a significant drop in dehydroepiandrosterone and dehydroepiandrosterone sulfate levels within 9 months, but there was a diminution in hirsutism in only four women (31%); in four out of seven menstrual irregularities decreased. In the cyproterone acetate group hirsutism diminished significantly in 66% (n = 10) without suppression of adrenal androgens. Weight gain occurred in a few cases in both groups; other side effects developed in 33% in the cyproterone acetate group. Preselection of patients with hirsutism is useful with respect to diagnosis; adrenal pathogenesis should not generally indicate dexamethasone treatment of hirsutism unless there is a desire for pregnancy, because cyproterone acetate is a more powerful agent in reducing hair growth.

Of 218 women with hirsutism 16 (7%) were found to have partial 21-hydroxylase deficiency, while 38 (17%) had partial 3 beta-hydroxysteroid dehydrogenase deficiency. Six women (3%) had a steroid constellation which resembled that of an augmented adrenarche. In the women with enzyme deficiency over-weight and abnormal menstruations were more frequent (50%) than in those without such deficiency (33%). The degree of hirsutism and age at diagnosis were similar in those with and those without partial enzyme deficiency. Furthermore, the diagnosis of partial enzyme deficiency could only be made with certainty by the ACTH stimulation test, because with sole measurement of basal levels (17-hydroxyprogesterone and 21-desoxycortisol in 21-hydroxylase deficiency, and 17-hydroxypregnenolone and dehydroepiandrosterone in 3 beta-hydroxysteroid dehydrogenase deficiency) the enzyme defects are in most instances not revealed.

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