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Patient # 1

Patient # 1. 50 year old male Chief complaints: Fatigue sweating of hands and feet Increasing shoe size Joint pains Headache. Patient #1 (2). Pertinent family history No family history of Pituitary tumors Hypercalcemia Pancreatic tumors Past medical history

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Patient # 1

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  1. Patient # 1 • 50 year old male • Chief complaints: • Fatigue • sweating of hands and feet • Increasing shoe size • Joint pains • Headache

  2. Patient #1 (2) • Pertinent family history • No family history of • Pituitary tumors • Hypercalcemia • Pancreatic tumors • Past medical history • s/p carpal tunnel surgery • Physical findings

  3. GH Excess Enlargement of hands and feet Thick skin Skin tags Sweating Sleep Apnea Carpal Tunnel Syndrome Glucose intolerance Osteoarthritis Colonic Polyps Tumor-related Headache Visual field defect Loss of pituitary function Gonadotrophins TRH - hypothyroid ACTH - Addison’s Acromegaly - Signs and Symptoms

  4. Acromegaly Frontal Bossing Chin Protrusion

  5. Acromegaly: Large Hands

  6. Bone and Soft Tissue Manifestationsof Acromegaly

  7. Acromegaly A patient with marked macroglossia. This can cause severe sleep apnea which can be associated with cardiac arrhythmias and sudden death.

  8. Acromegaly: Skin Tags

  9. Acromegaly: Slow changes over years

  10. Acromegaly: Slow changes over years

  11. Initial Test to Diagnose Acromegaly?

  12. Diurnal Variation in Pulsitile Growth Hormone Secretion

  13. Dopamine Glucose Alpha adrenergic Opiates GABA + Regulation of GH Secretion TRH SS GRH GH Stimulation IGF-1 Inhibition

  14. Post Prandial Acromeg. Control Screening Growth Hormone Levels in Acromegaly Fasting GH Upper “Normal” Acromeg. Control

  15. Additional Tests to Diagnose Acromegaly • IGF-1 (Insulin-like Growth Factor 1) • IGF-BP3 (IGF binding protein 3) • Advantages: • Single blood level • No diurnal variation • Disadvantages • Some overlap with normal

  16. Definitive Test for Acromegaly • Oral Glucose Tolerance Test (OGTT)

  17. Acromegaly Control Oral Glucose Tolerance Test GH Upper “Normal” 0 30 60 90 1200 Time (minutes)

  18. Acromegaly: Diagnosis Clinical suspicion of acromegaly 1-2hr postprandial GH, IGF1 and IGFBP3 Normal Abnormal 2 hr OGTT with GH levels Normal Acromegaly Excluded Abnormal Acromegaly diagnosed

  19. Growth-Hormone ExcessEtiology • 98%: GH-producing pituitary tumor • 2%: Ectopic GHRH secretion • Small cell lung cancer • Bronchial or intestinal carcinoid tumors • Pancreatic islet cell tumor • Pheochromocytoma

  20. Acromegaly: Diagnosis Clinical suspicion of acromegaly 1-2hr postprandial GH, IGF1 and IGFBP3 Normal Abnormal 2 hr OGTT with GH levels Normal Acromegaly Excluded Abnormal Pituitary MRI Normal Octreoscan Abnormal Site-specific CT/MRI TREATMENT

  21. Pituitary Macroadenoma (Sagittal MRI Scan) Optic Chiasm Pituitary Tumor

  22. Normal Visual Fields 90 70 50 50 70 90 O.S. O.D.

  23. Bitemporal hemianopsia due to Pituitary Tumor 90 70 50 30 30 50 70 90 O.S. O.D.

  24. Visual Field Defects Caused by Pituitary Tumor From Sandoz slide set

  25. Acromegaly (1) • Prevalence: 40-50 / 106 • Incidence: 3-4 / 106 • Mean age of onset: 32 years • Mean age at diagnosis: 42 years • Prognosis: 2x increased mortality if not treated • Cure rate: Greatly reduced if tumor invades cavernous sinus

  26. Acromegaly -- Treatment

  27. Acromegaly: Treatment options Transsphenoidal Surg. Radiotherapy Somatostatin Dopaminergic Micro Macro Analog (Cabergoline) GH <1 mcg/l Normal IGF1 82% 47% 75% (20 years) 50-65% 10-20% Recurrence 5-10% * Late response inconv. & cost Low efficacy Complications Hypopit. 15% >50% None None Other DI- 2-3% Neuro deficits Gallstones Nausea, hypotens. * At 10 years, Longer-term recurrence probably higher

  28. Dopamine Glucose Alpha adrenergic Opiates GABA + Regulation of GH Secretion TRH SS GRH GH Stimulation IGF-1 Inhibition

  29. Pegvisomant - GH receptor antagonist Normalization of GH 90% Normalization of IGF1 80-90% Tumor growth Rare Long-term effect Unknown

  30. Invasive Acromegaly: Treatment Algorithm Pituitary Adenoma < 1 cm >1 cm Consider preoperative somatostatin analog Transphenoidal surgery Post prand GH >1 mcg/l And/or IGF-1 elevated Post-prand. GH <1 mcg/l IGF-1 normal somatostatin analog or dopaminergic Progressive therapy if post-prand. GH >1 mcg/l and IGF-1 not normal somatostatin analog, GHR antagonist or dopaminergic Annual Follow-up Combination therapy Adapted from Melmed ESAP, 1999 Radiation Therapy

  31. Patient #3 • 35 year old woman • Chief complaint • Amenorrhea for 6 months • Galactorrhea • Otherwise healthy • Past medical history • Family History • Physical findings

  32. Patient #3 • Prolactin -- 5000 pmol/l (nl < 900 pmol/l) • CT - consistent with microadenoma

  33. Differential Diagnosis of Hyperprolactinemia • Medications • Alpha-methyldopa, reserpine • Phenothiazines, butyrophenones, • benzamides (metoclopramide, sulpride) Estrogens • H2-receptor blockers (cimetidine) • Opiates • Hypothyroidism • Decreased dopamine delivery to pituitary • Pituitary, suprasellar and hypothalamic lesions • Radiation damage to the hypothalamus

  34. Differential Diagnosis of Hyperprolactinemia • Prolactin levels > 11,000 pmol/l is usually indicative of macroprolactinoma. • Stalk compression, medications, hypothyroidism and stress usually result in prolactin levels < 2,000 and virtually always less than 6,500 pmol/l. • Microprolactinomas, mass lesions compressing the pituitary stalk frequently present with similar prolactin levels.

  35. Women: Amenorhea 57-90 % Oligomenorrhea 10-28 % Regular menses 9-15 % Galactorrhea 30-80 % Headache 40 % Visual field defect<25 % Hirsutism 19 % Men: Decreased libido 75-100 % Impotence 68-100 % Headache 70 % Visual field defect 36-70 % Galactorrhea 10-30 % Gynecomastia 4-50 % Hyperprolactinemia: Clinical Presentation

  36. Response Recurrence Surgery Microprolactinoma 60-80% 50% Macroprolactinoma 10-30% ~100% RadiotherapyNormalization of PRL after ~10 years Medical Therapy Microprolactinoma >90% Macroprolactinoma 50-80% Prolactinoma: Results of Treatment

  37. Clinical Evaluation of Hyperprolactinemia Increased fasting, resting prolactin levels < 6,500 pmol/l > 6,500 pmol/l Exclude: Stress Renal failure Medications Hypothyroidism CT or MRI CT or MRI "Non-functioning" macroadenoma Micro- prolactinoma Macro- prolactinoma Surgery and/or Radiation Dopaminergic Therapy

  38. Patient #3: A.L. - History • 58 year old male • Presenting symptoms (3 months): • Decreased vision • Weight loss • Nausea • Dizziness • Impotence • Occasional diarrhea • Physical examination: • Bitemporal hemianopsia • Atrophic testes

  39. A.L. - Laboratory Data • Blood count, electrolytes, liver and kidney functions - Normal • Endocrine tests: • Prolactin - 50,400 pmol/l (N <450) • T4 - 46 nmol/l (N 60 - 160) • T3 - 2.0 nmol/l (N 1.2 - 3) • TRH test: TSH increased from 1.2 to 7.2 mU/l with delayed curve • ACTH test: Cortisol 108 to 617 µmol/l @ 60 minutes (Normal basal 200-700) • Testosterone - <0.9 nM/l (Nl 7-30) • LH - 4.1 U/l (3-15) • FSH - 1.2 U/l (1-10)

  40. Optic Chiasm Pituitary Macroadenoma AL- Pre-treatment MRI

  41. A.L. - Pre-treatment Visual Fields 24/3/89 O.S. O.D.

  42. A.L. - Treatment • Diagnosis: • Macroprolactinoma • Hypopituitarism: • Thyroid axis • Adrenal axis • Gonadotrophin axis • Treatment: • Postpone surgery • Bromocriptine in increasing doses • Cortisol, thyroid and testosterone replacement

  43. 80,000 30 Prolactin (pmol/l) 25 Bromocriptine (mg/d) 60,000 20 Prolactin pmol/l Bromocriptine Dose (mg/d) 40,000 15 10 20,000 5 0 0 April-90 Aug-89 April-89 Jan - 93 Dec-91 A.L. - Prolactin Levels During Treatment

  44. A.L. - Post-treatment Visual Fields 26/10/89 O.S. O.D.

  45. Optic chiasm AL- MRI Post-treatment

  46. A.L. - Long-term Follow-up • CT, MRI: • Complete tumor regression - empty sella • Complete normalization of visual fields • Pituitary functions: • Complete normalization of all axes • Prolactin levels: • 130 - 650 pmol/l (N < 450) on 1.25 - 0.625 mg/d bromocriptine

  47. Pt# 4 • 55 year old male • Coma • Blood pressure normal, no edema • Hyponatremia • Normokalemia • Past medial history • Viral syndrome 2 days before entry • Weight loss • Nausea • Progressive impotence, weakness and fatigue • Not taking any medications • Liver/Kidney function normal • Chest x-ray normal

  48. Patient #4 (2) • Additional tests: • Urine sodium -- 50 mEq/l • Presumptive Diagnosis

  49. Syndrome of Inappropriate ADH (SIADH) • Clinical findings: • Hyponatremia • Euvolemia (mild volume expansion) • Normokalemia • Diagnosis: • Hyponatremia • Inappropriately elevated urine sodium • No volume depletion or severe volume expansion • Etiology: • Glucocorticoid deficiency • Hypothyroidism • Pulmonary lesions • CNS lesions • Drugs (Chlorpropamide and others)

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