acid-phosphatase and Turner-Syndrome

The study centered on a controversy about whether long-term estrogen replacement therapy may ameliorate the osteoporosis seen in patients with Turner's syndrome. This study comprised 26 adult patients with Turner's syndrome (9 treated and 17 untreated or insufficiently treated) and 12 adult women with pure gonadal dysgenesis (8 untreated and 4 treated). A low bone density below -2 standard deviations from the age- and sex-matched predicted normal mean was documented by dual-photon absorptiometry of the lumbar spine in all the untreated and insufficiently treated patients, but only in 6 treated patients. The biochemical indices of bone resorption (urinary hydroxyproline excretion and plasma tartrate-resistant acid phosphatase activity), as well as osteoblastic function (serum osteocalcin and bone alkaline phosphatase isoenzyme), were significantly increased in untreated and insufficiently treated patients compared with treated patients. A significant negative correlation was found between biochemically documented osteoresorption and spinal bone mineral density corrected for age of the patients. Significant positive correlations were found between serum osteocalcin and bone alkaline phosphatase isoenzyme and between biochemical indices of bone resorption and formation. Although in the patients there was an evidence of a high bone remodeling rate, the rate of bone mass loss seemed to be low, comparable with that seen in oophorectomized women who had already passed their accelerated phase of bone loss. The results indicate that long-term hormonal replacement therapy is justified in gonadal dysgenesis, regardless of the karyotype of the patient, to prevent further bone mass loss.

Topics: Acid Phosphatase; Adult; Alkaline Phosphatase; Bone and Bones; Bone Regeneration; Bone Resorption; Calcium-Binding Proteins; Estrogens; Female; Humans; Hydroxyproline; Isoenzymes; Minerals; Osteocalcin; Turner Syndrome

The case of a woman with Turner's syndrome (45,X karyotype) presenting with secondary amenorrhea is reported. The patient had menstruated regularly from the age of 10 years to the age of 24 years; secondary sex characteristics were normal. Ovarian biopsy showed a fresh, enzymatically normal corpus luteum and only a few follicles. Tissue from the ovary also showed the 45,X karyotype. The patient developed hypergonadotrophic amenorrhea of ovarian origin at the age of 26.

Topics: Acid Phosphatase; Adult; Alkaline Phosphatase; Amenorrhea; Corpus Luteum; Female; Follicle Stimulating Hormone; Humans; Karyotyping; L-Lactate Dehydrogenase; Leucyl Aminopeptidase; Luteinizing Hormone; Lymphocytes; Menstruation; Ovary; Turner Syndrome

Topics: Acid Phosphatase; Adolescent; Adult; Aspartate Aminotransferases; Bone Marrow Cells; Cathepsins; Child; Female; Humans; Muscles; Surface-Active Agents; Turner Syndrome; Tyrosine

Topics: Acid Phosphatase; Adenosine Triphosphatases; Adolescent; Alkaline Phosphatase; Aminopeptidases; Cartilage; Child; DNA; Genetics, Medical; Glucosephosphate Dehydrogenase; Humans; In Vitro Techniques; L-Lactate Dehydrogenase; Malate Dehydrogenase; Mosaicism; Nucleotidases; RNA; Sex Chromatin; Succinate Dehydrogenase; Turner Syndrome