Delayed Puberty Topics in Adolescent Gynecology Michael Wolfe, M.D. University of Kansas School of Medicine Department of ...
Objectives <ul><li>Review normal pubertal development </li></ul><ul><li>Discuss common etiologies of delayed puberty </li>...
Normal Puberty <ul><li>Thelarche </li></ul><ul><li>Adrenarche </li></ul><ul><li>Growth Spurt </li></ul><ul><li>Menarche </...
Tanner Staging – Breast Development
Tanner Staging – Pubic Hair
Prepubertal Period <ul><li>3 changes in the low endocrine state of childhood occur </li></ul><ul><ul><li>Adrenarche </li><...
What is delayed puberty? <ul><li>Defined clinically as the absence or incomplete development of secondary sexual character...
Etiologies <ul><li>Hypergonadotropic Hypogonadism (43%) </li></ul><ul><ul><li>Ovarian failure with normal or abnormal kary...
Hypergonadotropic Hypogonadism <ul><li>Postmenopausal levels of FSH and LH </li></ul><ul><li>Gonadal Dysgenesis </li></ul>...
Hypogonadotropic Hypogonadism Reversible <ul><li>Physiologic delay / Functional Hypothalamic Amenorrhea </li></ul><ul><li>...
Hypogonadotropic Hypogonadism Irreversible <ul><li>GnRH deficiency </li></ul><ul><li>Hypopituitarism, pituitary adenomas, ...
Eugonadism <ul><li>Mullerian Agenesis </li></ul><ul><li>Vaginal Septum </li></ul><ul><li>Imperforate Hymen </li></ul><ul><...
Review <ul><li>Classification by gonadotropin levels </li></ul><ul><ul><li>Hypergonadotropic hypogonadism </li></ul></ul><...
Evaluation <ul><li>History </li></ul><ul><li>Physical Exam </li></ul><ul><li>Imaging Studies </li></ul>
Initial Labs <ul><li>Uterus Absent </li></ul><ul><ul><li>Karyotype </li></ul></ul><ul><ul><li>Serum testosterone </li></ul...
Progestational Challenge <ul><li>Positive withdrawal bleeding </li></ul><ul><ul><li>Suggests intact outflow tract </li></u...
Evaluation after estrogen/progesterone <ul><li>Measure gonadotropin levels (FSH and LH) </li></ul><ul><ul><li>Elevated Gon...
Gonadotropin Levels <ul><li>Elevated gonadotropins </li></ul><ul><li>Normal gonadotropins </li></ul><ul><li>Low gonadotrop...
Treatment Strategies <ul><li>Correct underlying pathology </li></ul><ul><li>Prevent complications of disease process </li>...
Summary <ul><li>Normal Puberty </li></ul><ul><li>Delayed Puberty </li></ul><ul><ul><li>Hypergonadotropic Hypogonadism (ele...
Resources <ul><li>Clinical Gynecologic Endocrinology and Infertility .6 th  ed. Speroff, Leon, et al. Lippincott Williams ...
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Delayed Puberty Topics in Adolescent Gynecology Delayed Puberty Topics in Adolescent Gynecology

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Delayed Puberty Topics in Adolescent Gynecology Delayed Puberty Topics in Adolescent Gynecology

  1. 1. Delayed Puberty Topics in Adolescent Gynecology Michael Wolfe, M.D. University of Kansas School of Medicine Department of Obstetrics and Gynecology
  2. 2. Objectives <ul><li>Review normal pubertal development </li></ul><ul><li>Discuss common etiologies of delayed puberty </li></ul><ul><li>Discuss evaluation and management of primary amenorrhea </li></ul>
  3. 3. Normal Puberty <ul><li>Thelarche </li></ul><ul><li>Adrenarche </li></ul><ul><li>Growth Spurt </li></ul><ul><li>Menarche </li></ul>
  4. 4. Tanner Staging – Breast Development
  5. 5. Tanner Staging – Pubic Hair
  6. 6. Prepubertal Period <ul><li>3 changes in the low endocrine state of childhood occur </li></ul><ul><ul><li>Adrenarche </li></ul></ul><ul><ul><li>Decreased repression of the “gonadostat” </li></ul></ul><ul><ul><li>Gradual amplification of peptide-peptide and peptide-steroid interactions leading to gonadarche </li></ul></ul>
  7. 7. What is delayed puberty? <ul><li>Defined clinically as the absence or incomplete development of secondary sexual characteristics by an age at which 95% of children of that sex and culture have initiated sexual maturation </li></ul><ul><li>In the U.S. the National Center for Health Statistics states that the upper 95% for females is age 12, with breast development being the first sign </li></ul>
  8. 8. Etiologies <ul><li>Hypergonadotropic Hypogonadism (43%) </li></ul><ul><ul><li>Ovarian failure with normal or abnormal karyotype </li></ul></ul><ul><li>Hypogonadotropic Hypogonadism (31%) </li></ul><ul><ul><li>Reversible vs. irreversible </li></ul></ul><ul><li>Eugonadism (26%) </li></ul><ul><ul><li>Anatomic abnormalities of the target organ or outflow tract </li></ul></ul>
  9. 9. Hypergonadotropic Hypogonadism <ul><li>Postmenopausal levels of FSH and LH </li></ul><ul><li>Gonadal Dysgenesis </li></ul><ul><ul><li>45X </li></ul></ul><ul><ul><li>46XX </li></ul></ul><ul><ul><li>46XY </li></ul></ul><ul><li>Mosaicism </li></ul>
  10. 10. Hypogonadotropic Hypogonadism Reversible <ul><li>Physiologic delay / Functional Hypothalamic Amenorrhea </li></ul><ul><li>Weight loss/anorexia </li></ul><ul><li>Primary hypothyroidism </li></ul><ul><li>Prolactinoma </li></ul><ul><li>Congenital adrenal hyperplasia </li></ul><ul><li>Cushing’s syndrome </li></ul>
  11. 11. Hypogonadotropic Hypogonadism Irreversible <ul><li>GnRH deficiency </li></ul><ul><li>Hypopituitarism, pituitary adenomas, malignant pituitary tumors </li></ul><ul><li>Craniopharyngioma </li></ul><ul><li>Congenital CNS defects </li></ul>
  12. 12. Eugonadism <ul><li>Mullerian Agenesis </li></ul><ul><li>Vaginal Septum </li></ul><ul><li>Imperforate Hymen </li></ul><ul><li>Androgen Insensitivity Syndrome </li></ul>
  13. 13. Review <ul><li>Classification by gonadotropin levels </li></ul><ul><ul><li>Hypergonadotropic hypogonadism </li></ul></ul><ul><ul><li>Hypogonadotropic hypogonadism </li></ul></ul><ul><ul><li>Eugonadism </li></ul></ul><ul><li>Disorders by compartment </li></ul><ul><ul><li>Compartment 1: Uterus, outflow tract </li></ul></ul><ul><ul><li>Compartment 2: Ovary </li></ul></ul><ul><ul><li>Compartment 3: Anterior Pituitary </li></ul></ul><ul><ul><li>Compartment 4: CNS, Hypothalamus </li></ul></ul>
  14. 14. Evaluation <ul><li>History </li></ul><ul><li>Physical Exam </li></ul><ul><li>Imaging Studies </li></ul>
  15. 15. Initial Labs <ul><li>Uterus Absent </li></ul><ul><ul><li>Karyotype </li></ul></ul><ul><ul><li>Serum testosterone </li></ul></ul><ul><li>Uterus Present </li></ul><ul><ul><li>TSH, Prolactin, Progestational Challenge </li></ul></ul><ul><ul><li>FSH, LH, estradiol </li></ul></ul><ul><ul><li>Other labs </li></ul></ul>
  16. 16. Progestational Challenge <ul><li>Positive withdrawal bleeding </li></ul><ul><ul><li>Suggests intact outflow tract </li></ul></ul><ul><ul><li>Likely due to anovulation </li></ul></ul><ul><ul><li>Functional ovary, pituitary, and CNS is confirmed </li></ul></ul><ul><li>Negative withdrawal bleeding </li></ul><ul><ul><li>Administer hormonal cycle of estrogen with progestational agent </li></ul></ul><ul><ul><li>May skip this step if certain of normal uterus </li></ul></ul>
  17. 17. Evaluation after estrogen/progesterone <ul><li>Measure gonadotropin levels (FSH and LH) </li></ul><ul><ul><li>Elevated Gonadotropins </li></ul></ul><ul><ul><li>Normal Gonadotropins </li></ul></ul><ul><ul><li>Low Gonadotropins </li></ul></ul>
  18. 18. Gonadotropin Levels <ul><li>Elevated gonadotropins </li></ul><ul><li>Normal gonadotropins </li></ul><ul><li>Low gonadotropins </li></ul>
  19. 19. Treatment Strategies <ul><li>Correct underlying pathology </li></ul><ul><li>Prevent complications of disease process </li></ul><ul><li>Sex steroids </li></ul>
  20. 20. Summary <ul><li>Normal Puberty </li></ul><ul><li>Delayed Puberty </li></ul><ul><ul><li>Hypergonadotropic Hypogonadism (elevated FSH, LH) </li></ul></ul><ul><ul><li>Hypogonadotropic Hypogonadism (low FSH, LH) </li></ul></ul><ul><ul><li>Eugonadism </li></ul></ul><ul><li>History and Physical Exam </li></ul><ul><li>Work-up: TSH, Prolactin, Progestational Challenge, Estrogen/Progesterone cycle, gonadotropin assays, imaging studies </li></ul>
  21. 21. Resources <ul><li>Clinical Gynecologic Endocrinology and Infertility .6 th ed. Speroff, Leon, et al. Lippincott Williams and Wilkins, 1999. </li></ul><ul><li>UpToDate, 2004 </li></ul>

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