Abstract

Patterns of bone loss in the axial and the appendicular skeleton were studied in 48 women with streak gonad syndrome (age: between 14-38 years, mean: 25.5 years). Bone mineral content was measured in vivo at the lumbar spine and right femoral neck by dual photon absorptiometry and at the midradius and distal radius by single photon absorptiometry. The bone mineral status of 20 patients was evaluated by radiomorphometrical indices of the metacarpals, femorals and lumbar spine, too. Mean bone mineral content and radiomorphometrical indices were significantly lower in patients with streak gonad syndrome than in age-matched normal subjects at all three scanning sites. The patients lost cortical and trabecular bone mineral content at the same rate, suggesting that bone loss in streak gonad syndrome is a generalized phenomenon. In spite of serious bone loss osteoporotic fractures were not observed in the patients, in contrast to patients with pathological post-menopausal osteoporosis having equal degree of bone deficiency. The authors did not find any relationship between the occurrence of osteoporosis and the karyotype of the patients. It is suggested that the osteoporosis in streak gonad syndrome--at least after puberty--is a consequence of gonadal hormone deficiency.