1. AMENORRHEA Paul Beck, MD, FACOG, FACS

2. Incidence of Primary Amenorrhea Less than.1% Puberty Breast: 10.8 +/- 1.10 yrs. Pubic Hair:11.0 +/- 1.21 yrs. Menarche12.9 +/- 1.2 yrs.

3. Onset of Puberty and Menstruation Ratio of fat to both total body weight and lean body weight Moderate obesity (20 – 30 % above ideal body weight) = earlier menarch Malnutrition (anorexia nervosa, starvation) = delay Prepubertal strenuous exercise (less total body fat) = delay e.g. ballet dancers, swimmers, runners

4. Diagnostic Evaluation by Compartments IOutflow Tract (uterus – vagina) IIOvary IIIAnterior Pituitary IVCNS – Hypothalamus (environment and psyche)

5. Evaluation History/Physical Psychiatric, family history-genetic abnormalities, nutritional status, growth/development Secondary sexual characteristics Presence of breasts – normal reproductive tract (uterus, vagina)

6. Evaluation Categories Breast Absent – Uterus Present Breast Present – Uterus Present Breast Present – Uterus Absent Breast Absent – Uterus Absent

7. Initial Tests for Amenorrhea Progesterone challenge TSHProlactin TSH elevated – hypothyroid Prolactin elevated (MRI – 100 ng/ml)

8. Progesterone Challenge Positive withdrawal bleed Normal prolactin Normal TSH Diagnosis = annovulation Treatment: monthly progesterone/O.C.

9. Progesterone Negative Withdrawal FSH/LH FSH/LH normal – estrogen/progesterone cycle If negative = end organ defect If FSH/LH high = ovarian failure Estrogen – positive withdrawal, FSH normal or low, MRI sella = no path Diagnosis: hypothalamic amenorrhea

10. Chromosome Evaluation for Ovarian Failure If the patient is under age 30 – karyotype Y chromosome/excision of gonadal area Problem – gonadal tumor – malignant 30% do not develop virilization, therefore even normal appearing female needs karyotype to exclude Y After age 30 = premature menopause

11. Selected Blood Test for Autoimmune Disease Calcium, phosphorus Fasting blood sugar A.M. cortisol Free T 4 – TSH Thyroid antibodies CBC – ESR – CRP Total protein A/G ratio Rheumatoid factor Antinuclear antibody

12. Specific Disorders IOutflow- imperforate hymen, ashermans mullerian agenesis, androgen insensitivity syndrome IIOvary - can be primary or secondary amenorrhea 40% of primary amenorrhea have gonadal streaks Of the 40%, 50% = 45,X 25% = mosaics 25% = mosaics 25% = 46 XX Secondary amenorrhea patients have many karyotypes

13. Specific Disorders (continued) Turner syndrome Gonadal dysgenesis Gonadal agenesis Savage syndrome Premature ovarian failure Radiation therapy Alkylating agents

14. Compartment III Anterior pituitary disorders Tumors – large bitemperal hemianopsia Small tumors – visual defects- rare Craniopharyngioma – calcification x-ray may produce blurring of vision AcromegalyCushings Pituitary prolactin adenomas (micro/macro) Sheehan’s syndrome

15. Compartment IV CNS disorders Hypothalamic amenorrhea – problem is a GNRH pulsatile secretion Anorexia/Bulemia/weight loss – 25% (onset – 10 – 30 years) Exercise

16. Etiology of Amenorrhea Breast – Absent Breast – Present Uterus Absent Uterus Present 17, 20 desmolase deficiency 1. Gonadal failure turner 45X 17 a hydroxylase deficiency 46xy Gonadal dysgenisis Agonadism 17 a hydroxylase deficiency with 46XX 2. Hypothalamic failure 3. Pituitary failure AIS (T.F.) Hypothalamic, pituitary, ovarian pt uterine etiology Mullerianagenesis