Transcript Laura Knecht, MD - Barrow Hypothalamic Hamartoma Blog

Symposium for Patients
& Caregivers
Hormonal Imbalances
Laura Knecht, MD
Adult Endocrinologist
Medical Director, Barrow Pituitary Center
Functions of the Hypothalamus
• Secretes hormones-releasing and inhibitory
effects on the pituitary gland
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Anti diuretic hormone (ADH)
Dopamine
Oxytocin
Somatostatin
Corticotropin Releasing Hormone (CRH)
Thyrotropin Releasing Hormone (TRH)
Gonadotropin Releasing Hormone (GnRH)
Growth Hormone Releasing Hormone (GHRH)
Anti Diuretic Hormone (ADH)
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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
Dopamine
• Released by the Hypothalamus
• Travels down the pituitary stalk
• Continuous release inhibits the release of
prolactin from pituitary
Oxytocin
• Acts at breast for milk let-down
• Acts at uterus to aid in contractions
Somatostatin
• Inhibits growth hormone release from pituitary
• Inhibits TSH release from pituitary
Corticotropin Releasing Hormone
(CRH)
• Acts at pituitary to release adrenocorticotropic
hormone (ACTH)
• ACTH acts at adrenal glands to secrete cortisol
(stress hormones)
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
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
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
Growth Hormone Deficiency
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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
Treatment
• Growth hormone deficiency
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Recombinant human growth hormone
Increased muscle mass
Decreased fat
? Improvement in bone mineral density
Improved quality of life
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
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
Prolactin/Oxytocin Deficiency
• Inability to lactate after delivery
• Difficulty with uterine contractions
Treatment
• Not available
• Not indicated
Hypothyroidism
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Central hypothyroidism
Fatigue
Heat/cold intolerance
Decreased appetite
Puffy face
Dry skin
Bradycardia
Relaxation of deep tendon reflexes
Anemia
Treatment
• TSH deficiency
• Levothyroxine (synthroid, levoxyl, unithroid, armour)
• Normalize free T4 – mid range (TSH not helpful)
• Treat adrenal insufficiency first
Adrenal Insufficiency
• Cortisol deficiency
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Fatigue
Anorexia
Weight loss
Decreased libido
Hypoglycemia
Eosinophilia
• Severe
• Vascular collapse
• Loss of peripheral vascular tone
• Death
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
Diabetes Insipidus
• Can occur prior to surgery, around time of
surgery, after surgery
• Can be temporary or permanent
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
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
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)
Diabetes Insipidus – Treatment
DDAVP (Desmopressin)
• Oral
• 0.1-0.2mg by mouth 2-3x/day
• Half-life of 8-12 hours
• Intranasal
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10mcg spray 1-2 sprays 2-3x/day
Longer half-life of 12 hours
More potent
Need to coordinate inhalation
Diabetes Insipidus – Treatment
DDAVP (Desmopressin)
• Subcutaneous
• Rarely necessary as outpatient
• Avoids absorption issues
• IV
• Avoids absorption issues
• Used in hospital around time of surgery
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