SAT-284 Sex Hormone Replacement Therapy in Turner Syndrome and the Impact on Morbidity and Mortality

Background: In Turner Syndrome (TS) it is recommended to induce puberty around 11-12 years of age, by initiating hormone replacement therapy (HRT), when premature ovarian failure is diagnosed. However, evidence for the long-term effects on morbidity and mortality is sparse and it is unknown how many TS receive HRT as recommended according to guidelines. Aim: To describe the incidence of cardiovascular and endocrine related morbidity and mortality, and the association with HRT in TS. Design: Nationwide epidemiological study using Danish registries on medications and healthcare contacts. Methods: We identified 1.156 women with Turner Syndrome diagnosed from 1960-2014 using The Danish Cytogenetic Central Registry and linked them with the Danish National Patient Register and the Medication Statistics Register. Statistics Denmark randomly identified 115.578 controls matched on gender and age. We used Stratified Cox regression to analyze morbidity, mortality and prescriptions, computing proportional hazard ratios (HR). Results: Women with TS had more than doubled risk of cardiovascular diseases such as arrhythmia, heart failure, hypertension, ischemic heart disease, stroke, and endocrine diseases such as thyroid disorders. Similar a 4-fold increase in the risk of diabetes type I and II and osteoporosis was seen. Many TS (17-33% depending on karyotype) never received HRT. Overall mortality in TS was 3-fold increased. Among TS with 45,X receiving HRT, mortality was lower than among HRT non-treated 45,X (HR 5.0 (3.0-8.2) vs. HR 3.0 (1.9-4.5)). We saw a significant reduction in use of antihypertensive medication (HR 0.5 (95% CI 0.4-0.7)), antidiabetics (HR 0.4 (95% CI 0.2-0.9)) and thyroid hormones (HR 0.5 (95% CI 0.3-0.9)) in HRT treated 45,X women compared to HRT non-treated 45,X women. Conclusion: 17-33% of TS women never received HRT. Mortality is increased 3-fold in all TS compared with controls, and 45,X women that did receive HRT had a lover mortality than those who did not. HRT have a beneficial effect on endocrine conditions, hypertension and stroke.