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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 |