RESUMEN
OBJECTIVE: To evaluate uterine development of women with Turner syndrome (TS) receiving conventional medical care. STUDY DESIGN: In a cross-sectional study we used ultrasonography for uterine evaluation in 86 women with TS 18 to 45 years of age participating in an intramural NIH study, and who had abnormal karyotypes in >70% of white blood cells. Outcomes were uterine dimensions and shape. Information on hormone treatment was obtained by personal interview. RESULTS: Twenty-five percent had a mature in size and shape uterus, and 31% had an immature uterus, with the remainder in a transitional category. Twenty percent of all participants were not taking hormone replacement therapy (HRT) in the preceding year. The majority on treatment were taking conjugated estrogens (CE) or oral contraceptives (OC). Factors associated with uterine maturity were history of spontaneous puberty and duration and type of HRT, with estradiol-based treatment being the most effective. The age at starting HRT was not a critical factor. CONCLUSIONS: Women with TS may develop a normal uterus even at a late start of HRT given adequate duration of treatment and regardless of karyotype.
Asunto(s)
Síndrome de Turner/complicaciones , Útero/anatomía & histología , Adolescente , Adulto , Factores de Edad , Anticonceptivos Orales/uso terapéutico , Estudios Transversales , Terapia de Reemplazo de Estrógeno , Estrógenos/uso terapéutico , Estrógenos Conjugados (USP)/uso terapéutico , Femenino , Humanos , Modelos Logísticos , Menarquia , Persona de Mediana Edad , Síndrome de Turner/genética , Ultrasonografía , Útero/diagnóstico por imagenAsunto(s)
Calidad de la Atención de Salud , Síndrome de Turner/complicaciones , Síndrome de Turner/diagnóstico , Adolescente , Adulto , Audiometría/estadística & datos numéricos , Estudios Transversales , Ecocardiografía/estadística & datos numéricos , Femenino , Humanos , Riñón/diagnóstico por imagen , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
BACKGROUND: We recently reported that electrocardiographic abnormalities are common in adults with monosomy X (Turner syndrome), but this issue has not been investigated in girls with Turner syndrome. PATIENTS AND METHODS: We analyzed electrocardiograms in 78 girls with Turner syndrome and 50 age-matched control girls. The girls with Turner syndrome had additional cardiac and metabolic evaluations. RESULTS: Girls with Turner syndrome were more likely to demonstrate > or = 1 electrocardiographic findings including right axis deviation, right ventricular hypertrophy, accelerated atrioventricular conduction, T-wave abnormalities, and a prolonged rate-corrected QT interval. The right-sided findings were associated with partial anomalous pulmonary venous connection, but the etiology of the other findings remains unknown. The rate-corrected QT interval was significantly longer in girls with Turner syndrome (431 +/- 22 vs 407 +/- 21 milliseconds). Twenty-eight girls with Turner syndrome but only 2 controls had a rate-corrected QT interval above the reference range. We found no correlation between body habitus, cardiac dimensions, or metabolic parameters and the rate-corrected QT interval duration in girls with Turner syndrome. CONCLUSIONS: Cardiac conduction and repolarization abnormalities seem to affect both young girls and adults with Turner syndrome equally, suggesting that electrophysiologic defects are intrinsic to the syndrome and indicating that electrocardiogram analysis should be included in evaluating and monitoring even the youngest patients with Turner syndrome. Attention to the rate-corrected QT interval is important, because some common medications may further prolong this interval and increase the risk of arrhythmias.