ABSTRACT
Long-term, low-dosage androgen treatment of patients with Turner syndrome results in more rapid growth and significantly greater adult height than in control patients who receive only estrogen for pubertal development. Seventeen patients treated with oxandrolone for one year and ten treated for two years had significantly greater growth velocities during than before treatment. Mean adult height of 25 patients treated with oxandrolone, fluoxymesterone, or both was significantly taller than the height of adult patients with Turner syndrome treated with estrogen only. Excessive skeletal maturation was not generally observed.
Subject(s)
Oxandrolone/therapeutic use , Turner Syndrome/drug therapy , Body Height/drug effects , Bone and Bones/drug effects , Drug Administration Schedule , Drug Therapy, Combination , Fluoxymesterone/administration & dosage , Fluoxymesterone/pharmacology , Fluoxymesterone/therapeutic use , Growth/drug effects , Humans , Karyotyping , Mosaicism , Oxandrolone/administration & dosage , Oxandrolone/pharmacology , Retrospective StudiesSubject(s)
Adrenal Hyperplasia, Congenital , Adrenocortical Hyperfunction/genetics , Genetic Carrier Screening , Steroid Hydroxylases/deficiency , Adrenocortical Hyperfunction/blood , Adrenocortical Hyperfunction/etiology , Adrenocorticotropic Hormone , Homozygote , Humans , Hydroxyprogesterones/blood , Progesterone/bloodABSTRACT
Two teen-age XY brothers with mental retardation, short stature, obesity, genital abnormalities, and contractures of their hands are described. They have generalized osteoporosis and a history of frequent fractures. Their endocrinologic evaluation was normal except for mild glucose intolerance and delayed, but normal puberty. Although these brothers are similar to individuals with Prader-Willi syndrome, their unusual hand contractures, clinically significant osteoporosis, and lack of hypotonia indicate that they represent a different entity.