1. Symposium for Patients & Caregivers

2. Hormonal Imbalances Laura Knecht, MD Adult Endocrinologist Medical Director, Barrow Pituitary Center

3. Functions of the Hypothalamus Secretes hormones-releasing and inhibitory effects on the pituitary gland Anti diuretic hormone (ADH) Dopamine Oxytocin Somatostatin Corticotropin Releasing Hormone (CRH) Thyrotropin Releasing Hormone (TRH) Gonadotropin Releasing Hormone (GnRH) Growth Hormone Releasing Hormone (GHRH)

4. Anti Diuretic Hormone (ADH) ADH (vasopressin) made in the hypothalamus ADH stored in posterior pituitary gland Works at kidney to resorb water Reabsorbing water regulates sodium levels in the blood Lack of ability to reabsorb water leads to increased thirst and urination

5. Dopamine Released by the Hypothalamus Travels down the pituitary stalk Continuous release inhibits the release of prolactin from pituitary

6. Oxytocin Acts at breast for milk let-down Acts at uterus to aid in contractions

8. Somatostatin Inhibits growth hormone release from pituitary Inhibits TSH release from pituitary

9. Corticotropin Releasing Hormone (CRH) Acts at pituitary to release adrenocorticotropic hormone (ACTH) ACTH acts at adrenal glands to secrete cortisol (stress hormones)

11. Thyrotropin Releasing Hormone (TRH) Acts at thyroid to secrete TSH (Thyroid Stimulating Hormone) Acts at pituitary to release prolactin TSH acts at thyroid to release T4, T3 which controls metabolic activities

13. Gonadotropin Releasing Hormone (GnRH) Acts at pituitary to secrete FSH (Follicular Stimulating Hormone) and LH (Luteinizing Hormone) Acts at ovaries and testicles to secrete Estrogen, Progesterone, and Testosterone

15. Growth Hormone Releasing Hormone (GHRH) Acts at pituitary to secrete Growth Hormone Growth hormone acts at liver to produce IGF-1 Acts at bones, muscles, cartilage

18. Growth Hormone Deficiency In children, short stature Diminished muscle mass Increased fat mass Increased LDL Increased inflammatory markers (IL-6, CRP) Increased cardiac disease Decreased bone mineral density Diminished quality of life

19. Treatment Growth hormone deficiency Recombinant human growth hormone Increased muscle mass Decreased fat ? Improvement in bone mineral density Improved quality of life

20. Hypogonadism Causes hypogonadism In women Inability to ovulate Oligo/amenorrhea Estradiol deficiency Decreased bone mineral density In men Testicular hypofunction Infertility Decreased testosterone (energy/libido) Decreased bone mineral density

21. Treatment LH/FSH deficiency Men - testosterone replacement if not interested in fertility Cannot follow LH If interested in fertility, can be treated w/ gonadotropins or GnRH, HCG Check sperm count Women - estradiol-progestin replacement if not interested in fertility If interested in fertility, can be treated w/ pulsatile GnRH or gonadotropins Effects of testosterone still being studied

22. Prolactin/Oxytocin Deficiency Inability to lactate after delivery Difficulty with uterine contractions

23. Treatment Not available Not indicated

24. Hypothyroidism Central hypothyroidism Fatigue Heat/cold intolerance Decreased appetite Puffy face Dry skin Bradycardia Relaxation of deep tendon reflexes Anemia

25. Treatment TSH deficiency Levothyroxine (synthroid, levoxyl, unithroid, armour) Normalize free T4 – mid range (TSH not helpful) Treat adrenal insufficiency first

26. Adrenal Insufficiency Cortisol deficiency Mild Fatigue Anorexia Weight loss Decreased libido Hypoglycemia Eosinophilia Severe Vascular collapse Loss of peripheral vascular tone Death

27. Treatment ACTH deficiency Administer hydrocortisone 20-30 mg/d in varying regimens Dexamethasone/prednisone (0.5-1mg, 5-7.5mg) have longer action Increase in times of stress Cannot measure serum ACTH, cortisol, urinary cortisol Mineralocorticoid replacement unnecessary Can unmask central DI w/ polyuria Can increase blood pressure, renal flow, and decrease bone mineral density

28. Diabetes Insipidus Can occur prior to surgery, around time of surgery, after surgery Can be temporary or permanent

29. Diabetes Insipidus - Symptoms Increased thirst Craving ice water Increased urination Every 30-60 minutes Night time urination 5-6x/night Increased sodium levels Above upper limit of normal

30. Diabetes Insipidus - Treatment If intact thirst center, can drink Can drink to thirst Usually desire ice water Avoid significantly increased sodium loads Tomato juice, V8, pickles, high salt foods

31. Diabetes Insipidus - Treatment If hypothalamus damaged, may not have desire to drink Can schedule drinking times With meals, at mid-morning (10am), mid-afternoon (3pm)

32. Diabetes Insipidus – Treatment DDAVP (Desmopressin) Oral 0.1-0.2mg by mouth 2-3x/day Half-life of 8-12 hours Intranasal 10mcg spray 1-2 sprays 2-3x/day Longer half-life of 12 hours More potent Need to coordinate inhalation

33. Diabetes Insipidus – Treatment DDAVP (Desmopressin) Subcutaneous Rarely necessary as outpatient Avoids absorption issues IV Avoids absorption issues Used in hospital around time of surgery

34. A Special Thanks to our Sponsors Aesculap Barrow Neurological Institute @ St. Joseph’s Hospital Barrow Neurological Institute @ Phoenix Children’s Hospital Great Council for the Improved Hope for Hypothalamic Hamartoma Foundation KARL STORZ Endoskope