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From girl to young Lady: Growing up with Turner syndrome. Tala Dajani, MD MPH FAAP FACP FACE Pediatric Endocrinology of Phoenix Presentation and info at: www.drdajani.com. Objectives. Discuss adulthood considerations Review care schedule for teens and adults Discuss transition plans.

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from girl to young lady growing up with turner syndrome

From girl to young Lady: Growing up with Turner syndrome

Tala Dajani, MD MPH FAAP FACP FACE

Pediatric Endocrinology of Phoenix

Presentation and info at: www.drdajani.com

objectives
Objectives
  • Discuss adulthood considerations
  • Review care schedule for teens and adults
  • Discuss transition plans
adulthood considerations
Adulthood Considerations
  • Hormones: thyroid, growth, female hormones
  • Bone health
  • Heart Health
  • Adult responsibility
hormones thyroid
Hormones: Thyroid
  • Thyroid
    • Autoimmune thyroid disease: Hashimoto thyroiditis
    • Hypothyroidism
      • Fatigue
      • Weakness
      • Weight
      • Coarse, dry hair
      • Dry, rough pale skin
      • Hair loss
      • Cold intolerance
      • Constipation
    • Screening: yearly thyroid lab screen
growth hormone
Growth Hormone
  • Poor growth
    • Untreated average final adult height is 4 feet 7 inches
    • SHOX gene deficiency: short stature homeobox
      • Skeletal development
      • End organ resistance: skeletal dysplasia
  • Growth hormone therapy
    • GH therapy started early, estrogen treatment could be initiated at a younger, more age-appropriate time without compromising adult height
    • GH therapy should generally begin as soon as growth failure occurs
    • GH benefits: skeletal bone strength, cholesterol, muscle strength
    • GH treatment of girls with TS does not affect ascending or descending aortic diameter above the increase related to the larger body size. (J ClinEndocrinolMetab 91: 1785–1788, 2006)
female hormone replacement
Female Hormone Replacement
  • Ovarian failure
    • Hormonal replacement therapy should begin at a normal pubertal age and be continued until the age of 50 yr
    • Female sex hormones
      • Muscle and bone strength
      • Sex drive
      • Energy
      • Sense of well being.
      • Estrogen may play a role in memory and mood
      • Protective effect against heart disease
estrogen replacement
Estrogen Replacement
  • Hormone replacement therapy (HRT) for:
    • Healthy bones: osteoporosis prevention
    • Development of secondary sexual characteristics.
    • Initiated between ages 12-15 years
    • Introduced to the body to mimic body’s natural pubertal progression and course
    • Best dose and optimal HRT is individualized by care provider
estrogen and growth timing
Estrogen and Growth Timing
  • Estrogen start decision means start of pubertal development
  • Puberty marks the end of childhood growth
  • Estrogen therapy over time leads to growth plate fusion and completion of bone growth
  • Full growth potential is balanced with timing of starting puberty
  • Estrogen continues after growth hormone discontinued
hrt choices
HRT Choices
  • Start low/ slow and graduate dose to mimic natural puberty
  • Forms of HRT
    • Oral estrogen: natural conjugated Equine estrogens
    • Estrogen Patches: Skin patches are like plasters which allow estrogen to be slowly absorbed through the skin.
    • Contraceptive pill: contain ethinyloestradiol
bioidentical hormone therapy
Bioidentical hormone therapy
  • Bioidentical estrogen: Estrace, Estraderm, Estrasorb, Climara, Vivelle or Femring
  • Bio-identical progesterone: Prometrium
  • No company has yet put bio-identical estradiol and progesterone into one combined product
  • Compounded preparations: estriol, estrone, estradiol, testosterone, progesterone and dehydroepiandrosterone (DHEA)
  • Menopausal symptoms
hrt risks
HRT Risks
  • Low estrogen levels in women
    • Osteoporosis
    • Heart disease.
    • HRT helps maintain bone health and reduce the risk of heart disease.
  • Replace hormones that the women’s bodies should be making—hormones that they need for their overall health.
  • HRT taken by women with certain health conditions is different than that taken my post-menopausal women.
  • The risks associated with post-menopausal HRT do not apply to pre-menopausal women taking HRT.
bone health
Bone Health
  • Untreated
    • Childhood: significant deficit in cortical
    • Adolescence: significant osteopenia
  • Treated
    • Long-term GH therapy: absence of osteopenia.
    • Long-term estrogen therapy: improved bone density but less than is also treated with GH
    • The data indicate that long-term GH treatment during the prepubertal and early to midpubertal years optimizes BMD and improves the prognosis for adequate peak bone mass being achieved after a puberty induced with exogenous estrogen
  • 1000 mg of elemental calcium daily in the preteen years
  • 1200–1500 mg of elemental calcium daily after 11 yr of age
heart health
Heart Health
  • Congenital heart defects: ~ 20 %
  • Cholesterol abnormalities:
    • Improved with GH and estrogen
    • Hypertension should be aggressively
  • Cardiac imaging, preferably magnetic resonance imaging
    • Performed at diagnosis
    • Repeated at 5- to 10-yr intervals to assess for congenital heart abnormalities and the emergence of aortic dilatation, a precursor to aortic dissection
prevention guidelines
Prevention Guidelines
  • Once
    • Karyotype
    • Renal ultrasound
    • Pelvic ultrasound
    • Comprehensive educational evaluation in early childhood to identify potential attention-deficit or nonverbal learning disorders.
  • Yearly evaluation
    • Height, weight, blood pressure, auscultation of the heart
    • Blood work: Creatinine, blood urea nitrogen, ASAT, -GT, TSH, FT4, total cholesterol, low-density cholesterol, high-density cholesterol, triglycerides, glucose, HbA1c, urine dipstick analysis
  • Every 3-5 years
    • Celiac serology
    • Audiogram
    • Cardiac ultrasound, including electrocardiogram
    • MRI aorta (thoracic and abdominal)
    • Bone mineral density measurement (DEXA Scan)
adult self care
Adult Self-care
  • Steps towards independent healthcare behaviors
    • Knowledge of health history and health needs
    • Personal health records
    • Making appointments
    • Managing prescriptions
    • Adult care plan
develop support systems
Develop Support Systems
  • Woman's friends are important to both her mental well-being and her physical health.
  • Support groups
health records care plan
Health Records/ Care Plan
  • Surgeries
  • Cardiac status
  • Kidney status
  • Medications and dosages
  • Allergies
  • Hearing status
  • Prevention checklist
conclusion
Conclusion

Early diagnosis and intervention

Get girls involved early in their care and decisions

Maintain good records

Normalize hormone levels

Presentation and info at: drdajani.com