25 endocrine

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<ul><li> 1. ENDOCRINE</li></ul> <p> 2. JOHN MINARCIK, MD minarcik@gmail.com http://snurl.com/pathology OPTIONAL youtube.com histopathology youtube.com shotgun histology 3. CLASSICAL ALGORHYTHM PITUITARYANTERIORPOSTERIOR THYROID PARATHYROID PANCREAS (endo.) ADRENALCORTEXMEDULLA DEGENERATION(aka, involution) INFLAMMATION NEOPLASMBENIGNMALIGNANT 4. BETTER ALGORHYTHM NON-NEOPLASTIC HYPER-function HYPO-function NEOPLASTIC FUNCTIONAL NON-FUNCTIONAL Functional endocrinemalignancies areRARE. Why? PITUITARY ANTERIOR POSTERIOR THYROID PARATHYROID PANCREAS (endo.) ADRENAL CORTEX MEDULLA 5. FEEDBACK SYSTEMS HYPOTHALAMUS ANTERIOR PITUITARY ENDOCRINE GLAND END ORGAN HYPOTHALAMUS 6. HORMONESPOLYPEPTIDE (2ndMESSENGER)STEROID (DIRECTon NUCLEUS) 7. ACIDOPHILSBASOPHILSCHROMOPHOBESAXONSAXONS and PITUI-cytesAIP 8. ANTERIOR PITUITARY ACIDOPHILSGROWTH HORMONEPROLACTIN BASOPHILSTSHACTHLH, FSH 9. POSTERIORPITUITARY OXYTOCIN (contractsuterine smooth muscle) VASOPRESSIN (ADH)(vasoconstriction, gluconeogenesis,platelet aggregation, release ofFactor-VIII and vWb factor,concentrates urine, main effects onkidney and brain) 10. PITUITARY PATHOLOGY CLINICAL FEATURES, mimic the endocrineeffects or mass effects) FUNCTIONING ADENOMAS HYPO-PITUITARISM POSTERIOR PITUITARY SYNDROMES HYPOTHALAMIC (SUPRASELLAR) TUMORS 11. CLINICAL FEATURES HYPER: growth, lactation, thyroid,adrenal cortex HYPO: growth, thyroid, adrenalcortex MASS EFFECT: visual fields, brain 12. GALACTORRHEA 13. GIGANTISM(excesssomatotropin[GH]BEFOREepiphysealclosure) 14. ACROMEGALY:(excesssomatotropin[GH] AFTERepiphysealclosure) 15. MOONFACIESBUFFALOHUMPSTRIAE 16. BITEMPORALHEMIANOPSIA 17. HYPO-pituitarism Pituitary tumors, functional or not. NON-pituitary tumors, primary or metastatic Pituitary surgery, of course Radiation, of course Apoplexy, i.e., sudden hemorrhage Sheehans syndrome (Post-partum ischemicnecrosis) Cysts (Rathkes cleft) Empty sella syndrome, (is NOT a disease) Genetic defects (pit-1 gene mutations) 18. POSTERIOR pituitaryDIABETESINSIPIDUSSIADH (Syndrome ofInappropriate Andi-Diuretic Hormone) 19. DIABETES INSIPIDUS ADH deficiency Head trauma, tumors,inflam. hypothal/pit Hyperdiureses withLOW sp.gr. 20. Inappropriate ADH ADH EXCESSHyponatremia, cerebral edema,neurologic symptomsNeoplasms, esp. Small Cell CA.NON-neoplastic lung diseasesPosterior pituitary injury 21. 15-25grams 22. HYPER-THYROIDISM aka, thyrotoxicosis Diffuse Nodular Adenoma Carcinoma Neonatal Secondary to TSH pituitary adenoma 23. HYPER-THYROIDISM HYPERMETABOLISM Tachycardia, palpitations Increased T3, T4 Goiter Exophthalmos Tremor GI hypermotility Thyroid storm, life threatening 24. HYPO-THYROIDISM 1 Developmental 1 Surgery, I-131, external radiation 1 Auto-immune (i.e., Hashimotos) 1 Iodine deficiency 1 Li+, iodides, p-aminosalicylates 2 (pituitary) 3 (hypothalamic, rare) 25. HYPO-THYROIDISM Cretinism Severe retardation CNS/Musc-skel Short stature Protruding tongue Umbilical hernia Myxedema (coma) Sluggishness Cool skin 26. THYROIDITIS Hashimoto (Auto-Immune) (Lymphoidfollicles with germinal centers), MOSTCOMMON cause of acquired hypothyroidismin USA Subacute Granulomatous (DeQuervain) Subacute Lymphocytic (just likeHashimotos but NO fibrosis and nogerminal centers), often post-partum 27. GRAVES DISEASE(aka, diffuse toxic goiter) HYPERTHYROIDISM EXOPHTHALMOS PRE-TIBIAL MYXEDEMA Autoimmune, auto-antibodies to TSH 28. SCALLOPING 29. GRAVES DISEASE(aka, diffuse toxic goiter)PLUMMER DISEASE(aka, nodular toxic goiter)HARDER TO TREATSurgPTU (Propyl Thio Uracil)I-131 30. GOITERS(aka, thyromegaly, diffuse or nodular) IODINE deficiency Increased TSH Goitrogens, e.g., cabbage, Brusselssprouts, cauliflower, turnips, cassava) Associated with HYPO thyroidismeventually, NOT hyperthyroidism 31. GOITER 32. Thyroid Neoplasms Nodules vs. true neoplasms Adenomas vs. Carcinomas 33. NODULES Solitary vs. Multiple Younger vs. Older Male vs. Female Hx. neck radiation vs. NO Rx. Cold vs. HOT (really NOT-cold) 34. NEOPLASMS ADENOMASFOLLICULARHRTHLE(oxyphilic) CARCINOMASFOLLICULARPAPILLARYMEDULLARY(AMYLOID)ANAPLASTIC(worst) 35. HRTHLE CELL ADENOMA, note atypia 36. ORPHAN ANNIE CELLS in PAPILLARY CARCINOMA 37. MEDULLARY CARCINOMA of the thyroid with HYALINIZATION, i.e.,AMYLOID!!! 38. HYALINIZATION showing APPLE GREENbirefringence in CONGO RED stain, i.e., AMYLOID 39. BIOLOGIC BEHAVIOR Papillary CA lymph nodes Follicular CA bloodvessels, bone 40. 35-40 mg 41. PTH HYPOCALCEMIA is MAINSTIMULUS (9-10.5 mg/dl) ANTAGONIZES CALCITONIN 42. PARATHYROID DISORDERS HYPER-PRIMARY (usually adenomas)SECONDARY (LOW CA++ of RenalFailure) HYPO-: Surgical, congenital,familial, idiopathic PSEUDO-HYPO-(end organ resistance) 43. HYPER-PARATHYROIDISM Bone pain, fractures Nephrolithiasis Constipation, ulcers, gallstones Depression, lethargy Weakness, fatigue Valve calcifications 44. HYPO-PARATHYROIDISM Neuromuscular irritability Mental status change Parkinsonism like effects Lens calcification* (paradox) Widened QT interval Defective, carious, teeth 45. ADRENAL CORTEX Glomerulosa (Salt), mineralocorticoids ALDOSTERONE Fasciculata (Sugar), glucocorticoids CORTISOL Reticularis (Sex), gonadocorticoids ANDROGENS, ESTROGENS 46. 4 g. 47. SALTSUGARSEXSTRESS 48. HYPERADRENALISM HYPERALDOSTERONISM CUSHING SYNDROME(CORTISOL) ADRENOGENITAL(VIRILIZING) SYNDROME 49. CUSHING SYNDROME CENTRAL OBESITY MOON FACIES WEAKNESS HIRSUTISM HYPERTENSION DIABETES OSTEOPOROSIS STRIAE 50. MOONFACIESBUFFALOHUMPSTRIAE 51. CUSHING SYNDROME PITUITARY ACTH INCREASE TUMOR ACTH INCREASE HYPERPLASIA OF CORTEX ADENOMA OF CORTEX CARCINOMA OF CORTEXEXOGENOUSSTEROIDS (90%) 52. PRIMARYHYPERALDOSTERONISM(Conns Syndrome) Na+ RETENTION K+ EXCRETION HYPERTENSION 53. PRIMARYHYPERALDOSTERONISM CORTICAL NEOPLASM CORTICAL HYPERPLASIA FAMILIAL (rare) 54. SECONDARYHYPERALDOSTERONISM DECREASED RENAL PERFUSION EDEMA (HEART, LIVER, KIDNEY) PREGNANCY 55. ADRENOGENITALSYNDROME VIRILIZATION/feminization CORTICAL NEOPLASM CORTICAL HYPERPLASIA 21-Hydroxylase Deficiency 56. ADRENAL INSUFFICIENCY PRIMARY ACUTE(ADRENAL CRISIS) PRIMARY CHRONIC(ADDISON DISEASE) SECONDARY (PITUITARY) 57. PRIMARY ACUTE RAPID WITHDRAWAL OF STEROIDS MASSIVE ADRENAL HOMORRHAGE(WATERHOUSE-FRIDERICHSEN, if itfollows infection and shock)Newborns with DIFFICULT DELIVERYANTICOAGULANT RXPOSTSURGICAL DIC PATIENTS 58. PRIMARY CHRONIC Most of Addison disease is auto-immuneadrenalitis INFECTIONS METASTASES GENETIC DISORDERS 59. NEOPLASMS ADENOMAS of ADRENALCORTEX CARCINOMAS of ADRENALCORTEX 60. ADRENAL MEDULLA PHEOCHROMOCYTOMAS, aka,primary tumors of the adrenal medulla 10% arise in an MEN setting10% are EXTRA-adrenal 10% are bilateral 10% are malignant10% are in childhood You can only call them malignant if theymetastasize 61. PHEO 62. TWO crucially important pointsspecific for endocrine tumors: 1. FUNCTIONING carcinomas arevery RARE in ANY endocrinegland. Why? (KEY principle ofoncology) 2. Benign adenomas may haveextremely bizarre nuclei, but aremost usually BENIGN!!! 63. MEN-1, aka, Wermer Syndrome(3 Ps) HYPERPARATHYROIDISM,chiefly hyperplasiaPancreatic endocrinetumorsPituitary adenoma, usuallyprolactinoma 64. MEN-2 MEN-2A (SIPPLE): Pheo,Medullary CA., Parathyroidhyperplasia MEN-2B: NOhyperparathyroidism, butneuromas present Familial Medullary Thyroid CA 65. PINEAL GLANDPINEALOMASPINEOBLASTOMASPINEOCYTOMAS 66. ENDOCRINEPANCREAS 67. ExocrineEndocrineIsletsAlpha CellsBeta CellsDelta Cells(suppressinsulin andglucagon)PancreaticPolypeptide(PP) cellsEpsilonCells makegherlin 68. DIABETES MELLITUS 16 Million in the USA 1 Million/yr 50K people die of it peryear in the USA 69. How to Diagnose Dm: Glucose &gt;200 Or. Fasting glucose &gt;126 trice Or. Post-prandial glucose &gt; 200, 2 hrsAFTER standard OGTT (OralGlucose Tolerance Test) 70. * MODY might be regardedas the third typeTWO* Types of DM1 Genetic Autoimmune Childhood (juvenile)onset Antibodies to betacells Beta cell depletion NON-OBESEpatients2 Genetic, but diff. fromType 1 NOT autoimmune Adult, or maturityonset, e.g., 40s, 50s Insulin may be low,BUT, peripheralresistance to insulin isthe main factor OBESE patients 71. DmPOLY-POLY-POLY- 72. INSULIN FAT IN-creased glucose uptake IN-creased lipogenesis DE-creased lipolysis MUSCLE IN-creased glucose uptake IN-creased glycogen synthesis IN-creased protein synthesis LIVER DE-creased gluconeogenesis IN-creased glycogen synthesis IN-creased lipogenesis 73. PATHOGENESIS 1 T-Lymphocytesreacting againstpoorly definedbeta cellantigens Inflammatoryinflitrate,chronic, i.e.,INSULITIS 2 Diet Life Style Obesity INSULINRESISTANCE Beta cells UN-ableto adapt to thelong termdemands of insulinresistance 74. MODY (Maturity OnsetDiabetes of the Young) Multiple types 2-5% of diabetics Primary beta cell defects Multiple genetic mechanisms,especially GLUCOKINASEmutations 75. PANCREAS in Dm 76. PANCREAS in Dm 77. COMPLICATIONS MACRO-VASCULAR disease, i.e.,ASCVD MICRO-VASCULAR disease, kidneys,retina, nerves IMMUNE related problems,INFECTIONS, e.g., TB, pneumonia,pyelonephritis, candida, etc. 78. COMPLICATIONS ADVANCED GLYCATION collagen, laminin, polypeptides, GBM(glomerular basement membrane) ACTIVATION of PROTEIN KINASE C,VEGF, endothelin-1, increased ECM,decreased fibrinolysis, inflam.cytokines INTRACELLULAR HYPERGLYCEMIA 79. COMPLICATIONSMORPHOLOGY (MACRO-vascular) Atherosclerosis MICRO-vascular Retinopathy Nephropathy- glomerular, vascular, KW Neuropathy Infections 80. ATHEROSCLEROSIS 81. ATHEROSCLEROSIS 82. RETINOPATHY in DmShows microaneurysms,areas of hemorrhage,cotton wool spots, hardexudates, venous beading,neovascularization, retinaldetachment, vitreousdetachment, pre retinalhemorrhage 83. NEPHROPATHYKimmelstiel-Wilson (KW)KidneysIsNodularglomerulosclerosis 84. NEPHROPATHYNEPHROSCLEROSIS 85. NEPHROPATHYGBM thickening 86. NEPHROPATHYDiffuseMesangialSclerosis 87. INFECTIONS in Dm SKIN TUBERCULOSIS PNEUMONIA PYELONEPHRITIS CANDIDA 88. NEOPLASMS of theEndocrine Pancreas Islet cell tumors Beta cells INSULINOMAS (NOT rare) Alpha cells GLUCAGONOMAS (rare) Delta cells SOMATOSTATINOMAS(rare)GASTRINOMAS, producingZOLLINGER-ELLISON SYNDROME,consisting of increased acid and ulcers </p>

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