Individuals with early loss of endogenous estrogen have been shown to have an increased risk of CV mortality PubMed] «>15

Individuals with early loss of endogenous estrogen have been shown to have an increased risk of CV mortality PubMed] «>15

Although data in the adolescent population are lacking and there are no standard screening regimens for CV disease in this population, vigilant monitoring is warranted, and practitioners should help patients optimize CV health. Routine visits should include counseling on tobacco avoidance and appropriate diet and exercise to optimize CV health. Measuring blood pressure at least annually and lipid levels every 5 years is recommended. Patients with Turner syndrome have additional CV risks, including aortic aneurysm. Additional guidelines for patients with Turner syndrome and no obvious CV pathology include either routine cardiac imaging every 5–10 years or focused imaging when transitioning from a pediatric to adult health care provider, before attempting pregnancy, or with the appearance of hypertension to assess for coarctation or aortic stenosis PubMed] [Full Text] «>16. Although early loss of ovarian function has been associated as a risk factor for CV mortality, there are no data indicating that these patients are at an increased risk of CV adverse effects from hormonal therapy PubMed] «>15 PubMed] [Obstetrics & Gynecology]»>17.

Endocrine Disorders

Approximately 20% of adults with idiopathic primary ovarian insufficiency will experience hypothyroidism, most commonly Hashimoto thyroiditis PubMed] [Full Text] «>5 PubMed] [Obstetrics & Gynecology]»>18. Following initial diagnosis of primary ovarian insufficiency, it is appropriate to test thyrotropin levels for the presence of thyroid peroxidase antibodies. Although no recommendations for routine thyroid screening exist in this population, given the high prevalence of this disorder in patients with primary ovarian insufficiency, it is acceptable to test for thyroid disease every 1–2 years. Patients with primary ovarian insufficiency also have a 50% chance of developing adrenal insufficiency if they have adrenal autoimmunity. Patients should be tested for adrenal anti-bodies and if results are positive, should undergo yearly corticotropin stimulation testing. Data are lacking on the follow-up of patients with negative test results PubMed] [Full Text] «>1. Diabetes mellitus, pernicious anemia, myasthenia gravis, rheumatoid arthritis, systemic lupus erythematosus, and dry eye syndrome also have been associated with primary ovarian insufficiency, and testing should be based on symptomatology. Antiovarian antibodies may be present in these patients, but their specificity and pathogenic usefulness has not been validated PubMed] [Obstetrics & Gynecology]»>17.

Patient Counseling

When primary ovarian insufficiency is diagnosed in the adolescent female, the patient and her family are often unprepared for such news with its implications for compromised fertility and impaired self-image and the need for long-term hormonal therapy. It is best to inform the patient and family by having a direct conversation in the office Full Text] «>12. Adolescents may demonstrate myriad emotions ranging from apathy or denial to remorse or sadness, and these emotions may be different from those of their parents or guardians. Practitioners can consider telling the parents separately from their children so that the parents will have an opportunity to understand the diagnosis and adjust their demeanor to be most supportive of their daughters. Parents also can provide valuable insights about their daughters’ ability to appreciate the significance of the diagnosis of primary ovarian insufficiency and guide the clinician or team. Health care providers who make this clinical diagnosis should be mindful of the sensitive nature of this medical condition as well as the cultural significance of the diagnosis within the family unit. Use of the term “premature ovarian failure” can be particularly troubling to a young woman and her family PubMed] «>3. “Insufficiency” is the more accepted term in this population and more truly reflects the possibility of intermittent resumption of function. Patients and their families should be counseled on the effect of the patient’s condition on future fertility. Referrals to a reproductive endocrinology and infertility specialist should be made when desired by the patient and family to further discuss available reproductive treatments. In vitro fertilization with donor oocytes is often the most appropriate treatment; there are otherwise limited therapeutic options. Although the procedure may not be ideal, it may provide some hope for the patient who is told that her fertility is severely compromised. However, this is not a recommended option for patients with Turner syndrome because of the risk of aortic rupture during pregnancy. Psychologic counseling also should be offered because impaired self-esteem and emotional distress have been reported after diagnosis of primary ovarian insufficiency PubMed] «>19 PubMed] [Full Text] «>20 PubMed] [Full Text] «>21. Because many patients will use the Internet to learn more about their diagnoses, referral to appropriate sources for support is an efficient means to enhance patient care (see Resources).