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Goljan Endocrine
| Question | Answer |
|---|---|
| Overactive endocrine syndrome | most often adenomas; use suppression tests (most do not suppress) |
| Tumors that suppress | prolactinoma (bromocriptine), pituitary Cushings (high dose dexamethasone) |
| Underactive endocrine syndrome | autoimmune destruction MCC; stimulation tests |
| Hypopituitarism adults | non-functioning adenoma MCC, Sheehan’s postpartum necrosis (stop lactation) |
| Hypopituitarism in children | craniopharyngioma (Rathke’s pouch remnant) MCC; visual field defects |
| S/S ↓ FSH and LH | amenorrhea, ↓ testosterone in male |
| Growth hormone functions | muscle growth, gluconeogenesis; release of insulin growth factor (IGF) |
| IGF | synthesized in liver; bone and cartilage growth |
| S/S ↓ GH/IGF in children | growth retardation; ↓ height and weight |
| Sleep and arginine infusion | stimulation tests for GH and IGF |
| S/S ↓ GH/IGF in adults | hypoglycemia |
| S/S ↓ TSH | secondary hypothyroidism; ↓ T4, ↓ TSH; muscle weakness, dry skin |
| S/S ↓ ACTH | secondary hypocortisolism; ↓ cortisol, ↓ ACTH; fatigue; hypoglycemia |
| Metyrapone | stimulation test for ACTH reserve |
| Metyrapone | blocks adrenal 11-hydroxylase → ↑ ACTH and 11-deoxycortisol (proximal to block) |
| Metyrapone test ↓ ACTH and 11-deoxycortisol | pituitary/hypothalamic dysfunction |
| Metyrapone test ↑ ACTH and 11-deoxycortisol | Addison's disease |
| Diabetes insipidus | loss ADH (central), refractory to ADH (nephrogenic); always diluting urine |
| Central diabetes insipidus (CDI) | ↓ UOsm and ↑ POsm with water deprivation; vasopressin causes ↑ UOsm > 50% |
| Causes CDI | pituitary stalk transection, hypothalamic lesion (site for ADH synthesis) |
| Nephrogenic diabetes insipidus (NDI) | ↓ UOsm and ↑ POsm with water deprivation; vasopressin causes ↑ UOsm < 50% |
| Causes NDI | lithium, demeclocycline, nephrocalcinosis, severe hypokalemia |
| Gigantism | GH secreting pituitary adenoma before epiphyses have fused |
| Acromegaly | GH secreting pituitary adenoma after epiphyses have fused |
| S/S acromegaly | cardiomyopathy; large hands, feet, jaw; hyperglycemia |
| Prolactin | inhibited by dopamine |
| Prolactinoma | MC pituitary tumor; secondary amenorrhea and galactorrhea; prolactin inhibits GnRH |
| Rx | surgery or bromocriptine (dopamine analog) |
| Other causes hyperprolactinemia | primary hypothyroidism, drugs |
| Inappropriate ADH syndrome | hyponatremia <120 mEq/L; ↑ UOsm (always concentrating urine) |
| Causes | small cell carcinoma lung, CNS injury, chlorpropamide |
| Rx | restrict water; demeclocycline in small cell carcinoma |
| Serum T4 | ↑ or ↓ in free hormone or thyroid binding globulin (TBG) |
| ↑ Serum T4 and normal TSH | ↑ TBG; due to ↑ in estrogen |
| ↑ Serum T4 and ↓ TSH | thyrotoxicosis |
| ↓ Serum T4 and normal TSH | ↓ TBG; due to anabolic steroids |
| ↓ Serum T4 and ↑ TSH | primary hypothyroidism |
| ↓ Serum T4 and ↓ TSH | secondary hypothyroidism |
| TSH | negative feedback with T4 and T3; best screening test |
| I131 uptake | ↑ in Graves; ↓ in thyroiditis, patient taking excess thyroid, hypothyroidism |
| Cold nodule | non-functioning nodule; no uptake I131 |
| Hot nodule | functioning nodule; ↑ uptake I131 |
| Thyroglossal duct cyst | midline cystic mass |
| Branchial cleft cyst | cyst in anterolateral neck |
| Acute/subacute thyroiditis | painful thyroid; early thyrotoxicosis; ↓ I131 uptake |
| Hashimoto’s thyroiditis | MCC hypothyroidism; HLA Dr3/Dr5; inhibitory IgG TSH receptor antibody |
| Hashimoto’s thyroiditis | ↑ anti microsomal and thyroglobulin antibodies |
| S/S | muscle weakness, periorbital puffiness, ↓ reflexes, diastolic hypertension, constipation, dry skin |
| Lab | ↓ T4, ↑ TSH |
| Cretinism | maternal hypothyroidism before fetal thyroid developed, genetic disorder |
| S/S | mental retardation; short stature and increased weight; coarse skin |
| Thyrotoxicosis | any cause ↑ thyroid hormone activity; Graves disease, excess hormone, thyroiditis |
| Hyperthyroidism | ↑ synthesis thyroid hormone; Graves disease and toxic nodular goiter |
| Graves disease | autoantibody against TSH receptor (type II reaction); HLA Dr3 |
| S/S unique to Graves | exophthalmos, pretibial myxedema |
| S/S thyrotoxicosis | tachycardia/atrial fibrillation, systolic hypertension, diarrhea, brisk reflexes |
| Lab thyrotoxicosis | ↑ T4, ↓ TSH, ↑ glucose, ↑ calcium |
| I131 uptake | ↑ Graves, toxic nodular goiter; ↓ thyroiditis, excess hormone, hypothyroidism |
| Rx Graves disease | ß-blocker; drug to decrease hormone synthesis (propylthiouracil) |
| Toxic nodular goiter | hyperthyroidism; develops out of a multinodular goiter; no exophthalmos |
| Goiter | enlarged thyroid; iodine deficiency MCC; relative thyroid hormone deficiency |
| S/S | rapid enlargement due to hemorrhage into cyst; Rx thyroxine |
| Solitary thyroid nodule woman | most often benign (cyst) |
| Solitary thyroid nodule man or child | often malignant |
| Papillary carcinoma thyroid | MC thyroid cancer; radiation exposure; psammoma bodies |
| Follicular carcinoma thyroid | invades blood vessels |
| Medullary carcinoma thyroid | parafollicular cells; calcitonin; amyloid (calcitonin conversion) |
| MEN I syndrome | 3 P's; pituitary tumor, parathyroid adenoma, pancreatic tumor (ZE or ß-islet cell tumor) |
| MEN IIa syndrome | 2 P's; medullary carcinoma thyroid, pheochromocytoma, parathyroid adenoma |
| MEN IIb syndrome | 1 P; medullary carcinoma thyroid, pheochromocytoma, mucosal neuromas |
| Alkalotic pH | tetany with normal total calcium, ↓ ionized calcium and ↑ PTH |
| Hypoalbuminemia | ↓ total calcium, normal ionized calcium and PTH |
| Tetany | ↓ ionized calcium level; threshold potential comes closer to resting potential |
| S/S | thumb adducts into palm, twitching after tapping of facial nerve |
| PTH | maintains ionized Ca2+; ↑ Ca2+ renal reabsorption; ↓ phosphate/bicarbonate reabsorption in kidneys |
| Primary HPTH | ↑ Ca2+, hypophosphatemia, ↑ PTH |
| Cause | adenoma MCC, hyperplasia, cancer |
| S/S | renal stone, peptic ulcers, pancreatitis, hypertension, metastatic calcification |
| Secondary HPTH | ↓ Ca2+, ↑ PTH; hypovitaminosis D from renal failure MCC |
| Malignancy-induced hypercalcemia | ↑ Ca2+, ↓ PTH; all other non-parathyroid causes same results |
| Causes hypercalcemia | osteolytic lesions, sarcoidosis, ↑ vitamin D, PTH-related peptide, myeloma |
| Tertiary HPTH | hypercalcemia developing from secondary HPTH |
| Primary hypoparathyroidism | ↓ Ca2+ and ↓ PTH |
| Causes | previous thyroid surgery, autoimmune, DiGeorge syndrome |
| S/S | tetany; calcification basal ganglia |
| Pseudohypoparathyroidism | ↓ Ca2+ with normal to ↑ PTH; end-organ resistance to PTH |
| Other causes ↓ Ca2+ | hypomagnesemia (↓ PTH), ↓ vitamin D, DiGeorge |
| ↓ Ca2+ and ↓ PTH | primary hypoparathyroidism |
| ↓ Ca2+ and ↑ PTH | secondary hyperparathyroidism |
| ↑ Ca2+ and ↑ PTH | primary hyperparathyroidism |
| ↑ Ca2+ and ↓ PTH | malignancy induced hypercalcemia; other causes hypercalcemia |
| Waterhouse-Friderichsen syndrome | meningococcemia with bilateral adrenal hemorrhage due to DIC |
| Addison’s disease | autoimmune destruction adrenal cortex MCC, adrenogenital syndrome, metastasis |
| S/S | hypotension (salt loss), hyperpigmentation (ACTH), hypoglycemia |
| Lab | ↓ sodium, ↓ cortisol, ↑ potassium, ↑ ACTH |
| Adrenogenital syndrome | AR; enzyme deficiency; hypocortisolism; hyperpigmentation from ↑ ACTH |
| 21-Hydroxylase deficiency | ↑ 17 KS, ↓ 17 OH, lose salt, hypotension; female pseudohermaphrodite |
| 11-Hydroxylase deficiency | ↑ 17 KS, ↑ 17 OH, retain salt, hypertension; female pseudohermaphrodite |
| 17-Hydroxylase deficiency | ↓ 17 KS, ↓ 17 OH, retain salt, hypertension; male pseudohermaphrodite |
| MCC Cushings | long-term corticosteroid therapy |
| Tests Cushings syndrome | low/high dose dexamethasone suppression; urine free cortisol (best test) |
| Normal dexamethasone suppression | cortisol analogue; ↓ ACTH and ↑ cortisol |
| Pituitary Cushings | MCC Cushing’s; ACTH secreting pituitary tumor |
| Lab | low dose dexamethasone not suppress cortisol; high dose suppresses |
| Adrenal Cushings | adrenal adenoma secreting cortisol; suppressed ACTH |
| Lab | no suppression with low/high dose dexamethasone |
| Ectopic Cushings | ACTH secreting small cell carcinoma of lung; high ACTH and cortisol levels |
| Lab | no suppression with low/high dose dexamethasone |
| S/S Cushings | purple stria, truncal obesity, hypertension, DM |
| Primary aldosteronism | benign adenoma in zona glomerulosa |
| S/S | hypertension and muscle weakness (hypokalemia), no pitting edema |
| Lab | hypernatremia, hypokalemia, metabolic alkalosis, ↑ urine K+ and Na+ |
| Pheochromocytoma | benign tumor in adrenal medulla in adults |
| Associations | von Hippel Lindau, neurofibromatosis, MEN IIa and IIb |
| S/S | labile hypertension, anxiety, sweating, headache |
| Lab | ↑ 24 hr urine for VMA and metanephrines |
| Neuroblastoma | malignant tumor adrenal medulla child; widespread metastasis; hypertension |
| ß-islet cell tumor (insulinoma) | benign tumor; hypoglycemia, ↑ insulin and C-peptide |
| Patient taking excess insulin | hypoglycemia, ↑ insulin, ↓ C-peptide |
| Glucagonoma | malignant α-islet cell tumor; hyperglycemia and rash |
| Zollinger Ellison syndrome | malignant islet cell tumor secreting gastrin; peptic ulcers |
| Somatostatinoma | malignant δ islet cell tumor; DM, malabsorption, cholelithiasis, achlorhydria |
| VIPoma | malignant islet cell tumor; diarrhea, hypokalemia, achlorhydria |
| DM | organ damage correlates with glycemic control |
| Type 1 | young, thin person; no insulin; HLA DR3/4; insulitis; islet cell antibodies; ketoacidosis |
| Type 2 | older person; obese; relative insulin deficiency (↓ insulin receptors, postreceptor problems) |
| Type 2 | family history; fibrotic islet cells with amyloid; hyperosmolar nonketotic coma |
| ↑ Non-enzymatic glycosylation | glucose attaches to amino acids in basement membranes |
| Non-enzymatic glycosylation | ↑ vessel permeability producing hyaline arteriolosclerosis |
| Osmotic damage | glucose converted into sorbitol by aldose reductase |
| Osmotic damage | lens (cataracts), Schwann cell (neuropathy), pericytes retinal vessels (microaneurysms) |
| Pathogenesis hyperglycemia | ↑ gluconeogenesis (most important), glycogenolysis |
| Pathogenesis hyperlipidemia | no insulin to stimulate capillary lipoprotein lipase; ↑ chylomicrons/VLDL |
| Pathogenesis ketoacidosis | ↑ oxidation fatty acids with excess acetyl CoA; liver synthesis ketone bodies |
| Most commons due to DM | neuropathy, blindness, CRF, hyperglycemia, non-traumatic amputation |
| Glycosylated HbA1c | measure of long term glycemic control (8-12 weeks) |
| Gestational DM | ↑ placental size, human placental lactogen |
| Complications | macrosomia (↑ muscle/fat from insulin), RDS, newborn hypoglycemia (↑ insulin) |
| Hypoglycemia | insulin/oral hypoglycemics MCC, liver disease; carnitine deficiency |
| Carnitine deficiency | no ß-oxidation of fatty acids; all cells compete for glucose |