What supplements can help manage metabolism and hormones in women with Turner's syndrome?

Hormone Replacement Therapy for Women with Turner's Syndrome

For women with Turner's syndrome, hormone replacement therapy (HRT) with transdermal 17β-estradiol is the recommended supplement to manage metabolism and hormonal balance, with strong evidence supporting its effectiveness for reducing cardiovascular risk, improving bone health, and enhancing quality of life.

Hormone Replacement Therapy Recommendations

Estrogen Therapy

• Transdermal 17β-estradiol is preferred over oral ethinylestradiol or conjugated equine estrogens due to:

  • Better safety profile
  • Avoidance of hepatic first-pass effect
  • Minimal impact on hemostatic factors
  • More beneficial effects on lipids, inflammation markers, and blood pressure 1

• Recommended dosing schedule for adolescents with Turner's syndrome:

Time after start	Transdermal Estradiol	Oral Estradiol
0-6 months	1/8 patch weekly or 1/4 patch for 3-4 days/week	0.5 mg every other day
6-12 months	1/4 patch weekly	0.5 mg every other day
12-18 months	1/2 patch for 3-4 days, 1/4 patch remaining days	0.5 mg daily
18-24 months	1/2 patch weekly	0.5 mg and 1 mg alternating days
>24 months	Full patch weekly	1 mg daily

• For adult women with Turner's syndrome, maintenance dose is typically 100-200 μg/day of transdermal estradiol 1

Progesterone Therapy

• For women with an intact uterus, progesterone must be added to prevent endometrial hyperplasia 2, 1
• Preferred options:
  • Micronized progesterone: 100-200 mg/day for 12-14 days of the month 2
  • Dydrogesterone: 5-10 mg/day for 12-14 days of the month 2
  • Begin cyclic progestogens after at least 2 years of estrogen therapy or when breakthrough bleeding occurs 2

Timing of Therapy

• Initiation: Ages 11-12 years for pubertal induction if FSH is elevated and no spontaneous puberty 2, 1, 3
• Duration: Continue at least until the average age of natural menopause (around 51 years) 1, 4
• Early initiation is crucial for:
  • Proper bone development
  • Cardiovascular protection
  • Uterine development
  • Psychosocial development 2, 3

Monitoring Recommendations

• Initial evaluation: 3-6 months after starting therapy 1
• Annual clinical review thereafter, monitoring:
  • Symptom control and compliance
  • Blood pressure
  • Weight
  • Cardiovascular risk assessment (including lipid profile, fasting glucose, HbA1c)
  • Bone mineral density measurement at diagnosis and if osteoporosis is diagnosed, repeat within 5 years 1, 4

Benefits of HRT for Turner's Syndrome

1. Cardiovascular Health:

   • Reduces risk of ischemic heart disease, hypertension, and stroke 2, 4
   • Improves lipid profile 4, 5

2. Bone Health:

   • Prevents osteoporosis and reduces fracture risk 4, 6
   • Improves bone mineral density 5

3. Metabolic Benefits:

   • Normalizes hyperinsulinemia 5
   • Reduces risk of type 2 diabetes 4
   • Normalizes liver enzymes 4, 5

4. Reproductive System Development:

   • Promotes development of secondary sexual characteristics
   • Supports uterine growth and development 3

Special Considerations

• Cardiovascular Monitoring: Women with Turner's syndrome have increased risk of aortic dilatation and dissection; regular cardiac monitoring is essential 2, 6

• Fertility: Most women with Turner's syndrome are infertile; only 2% have natural pregnancies. Oocyte donation may be an option but carries higher risks of pregnancy complications 7

• Thyroid Function: Regular monitoring recommended due to increased risk of hypothyroidism 4

• Contraindications to HRT:

  • History of breast cancer
  • Active thromboembolic disorders
  • History of arterial thrombotic disease
  • Undiagnosed vaginal bleeding
  • Severe liver disease 1

Common Pitfalls to Avoid

1. Delaying estrogen therapy - can be detrimental to bone and uterine health 3

2. Using oral estrogens in women with cardiovascular risk factors - transdermal route is safer 1

3. Excessive vitamin D supplementation (>4,000 IU daily) - can have adverse effects 1

4. Inadequate progesterone therapy in women with intact uterus - increases risk of endometrial hyperplasia 2, 1

5. Failure to monitor cardiovascular risk factors - Turner's syndrome patients have inherently higher cardiovascular risk 2, 4