Endocrine Pathology Flashcards
Patient presents to clinic with hypertension, weight gain, moon facies, truncal obesity, buffalo hump, hyperglycemia, striae. You suspect _____. What is the #1 exogenous cause?
Cushing’s syndrome; steroids
What are 3 endogenous causes of Cushing’s syndrome? State whether ACTH inc/dec for each.
Cushing’s disease (inc ACTH from pituitary adenoma), ectopic ACTH (inc ACTH from non-pituitary tissue), adrenal adenoma/carcinoma/nodular adrenal hyperplasia (dec ACTH)
What are some examples of nonpituitary tissue making ACTH?
Small cell lung cancer, bronchial carcinoids
Is cortisol elevated/suppressed during a dexamethasone suppression test for ACTH-pituitary tumor?
With low dose dex: remains elevated; with high dose dex: suppressed
Is cortisol elevated/suppressed during a dexamethasone suppression test for ectopic ACTH-pituitary tumor?
With low dose dex: remains elevated; with high dose dex: remains elevated
Is cortisol elevated/suppressed during a dexamethasone suppression test for cortisol-producing tumor?
With low dose dex: remains elevated; with high dose dex: remains elevated
Patient with adrenal hyperplasia who presents with HTN, hypokalemia, metabolic alkalosis, and low plasma renin. What is the treatment?
Patient has primary hyperaldosteronism. Surgery to remove the tumor and/or spironolactone
Secondary hyperaldosteronism is associated with high/low plasma renin. What is the treatment?
High; spironolactone
How does a patient with Addison’s disease clinically present?
Hypotension, hyperkalemia, acidosis, skin hyperpigmentation (due to MSH, a by-product of inc ACTH production from POMC)
T/F: Addison’s disease only affects the outer 2 cortical divisions.
FALSE: Involves all 3 cortical divisions (spares medulla)
What are 2 clinical features that distinguish primary from secondary adrenal insufficiency?
Unlike primary, secondary does not have skin hyperpigmentation and no hyperkalemia
What is Waterhouse-Friderichsen syndrome?
Acute primary adrenal insufficiency due to adrenal hemorrhage. Associated with Neisseria meningitidis septicemia, DIC, endotoxic shock
What is the Rule of 10’s for pheochromocytoma?
10% malignant, bilateral, extra-adrenal, calcify, kids
Most pheochromocytomas secrete which 3 hormones?
Epinephrine, NE, dopamine
How is pheochromocytoma diagnosed and treated?
Dx: Urinary VMA and plasma catecholamines are elevated; Treatment: Tumor surgically removed only after effective alpha and beta blockade
T/F: Pheochromocytoma is associated with neurofibromatosis type 1, MEN types 2A and 2B.
TRUE
T/F: When treating pheochromocytoma, beta blockers must be given first to avoid a hypertensive crisis.
FALSE: Irreversible alpha-antagonists must be given first to avoid a hypertensive crisis
What are the 5 P’s of pheochromocytoma (episodic hyperadrenergic symptoms)?
Pressure (elevated blood pressure), pain (headache), perspiration, palpitations (tachycardia), pallor
T/F: In neuroblastoma, HVA is elevated in urine.
TRUE
Patient presents with weight gain, constipation, cold intolerance, decreased appetite, lethargy and fatigue. On exam, some facial myxedema is noted and patient has decreased reflexes, bradycardia. You suspect hypothyroidism. What is the most sensitive test for primary hypothyroidism?
TSH (increased, also see decreased free T4)
Patient presents with heat intolerance, weight loss, diarrhea. Increased reflexes and pretibial myxedema seen on exam. Would you expect TSH, free or total T3 and T4 to be inc or dec?
dec TSH, inc free or total T3 and T4
Patient presents with moderately enlarged, nontender thyroid. Histology shoes Hurthle cells and lymphocytic infiltrate with germinal centers. What are the antibodies associated with this disease?
Antithyroglobulin antibodies (this is Hashimoto’s thyroiditis)
What are the 5 P’s associated with cretinism?
Pot-bellied, Pale, Puffy-faced child with Protruding umbilicus and Protuberant tongue
Patient with recent flu-like illness presents with jaw pain and a very tender thyroid. Histology shows granulomatous inflammation. What is a lab finding associated with the disease and treatment?
Inc ESR; self limiting
T/F: Patients with subacute thyroiditis may be hyperthyroid early in course.
TRUE
T/F: Riedel’s thyroiditis presents as a fixed, hard, painful goiter.
FALSE: Painless
Fill in the blank: Toxic multinodular goiter is focal patches of hyperfunctioning follicular cells working independently of TSH due to a _____.
Mutation in TSH receptor
Hot nodules are rarely malignant/benign.
Malignant
What are some clinical findings associated with Graves’ disease?
Proptosis, pretibial myxedema, inc in connective tissue deposition, diffuse goiter
What is thyroid storm?
Stress-induced catecholamine surge leading to death by arrhythmia
What type of thyroid cancer is associated with Orphan Annie’s eyes and psammoma bodies on histology?
Papillary carcinoma
Calcitonin is produced in which type of thyroid cancer?
Medullary carcinoma
What malignancy is associated with Hashimoto’s thyroiditis?
Lymphoma
Which type of thyroid cancer is associated with the best prognosis: papillary, medullary, follicular, or medullary?
Papillary carcinoma
T/F: Primary hyperparathyroidism is usually an adenoma.
TRUE
Hypercalcemia is seen with primary/secondary hyperparathyroidism while hypocalcemia is seen with primary/secondary hyperparathyroidism.
Primary; secondary
Urine cAMP is inc/dec in primary hyperparathyroidism.
Inc
What is osteitis fibrosa cystica?
Cystic bone spaces filled with brown fibrous tissue, leads to bone pain
What is renal osteodystrophy?
Bone lesions due to secondary or tertiary hyperparathyroidism due to renal disease
What are some causes of hypoparathyroidism?
Accidental surgical excision (thyroid surgery), autoimmune destruction, DiGeorge syndrome
Describe the 2 clinical signs associated with hypoparathyroidism.
Chvostek’s sign: tapping of facial nerve leads to contraction of facial muscles; Trousseau’s sign: occlusion of brachial artery with BP cuff leads to carpal spasm
Patient presents with decreased PTH, hypocalcemia, shortened 4th/5th digits, and short stature. Name the genetic disease.
Pseudohypoparathyroidism (Albright’s hereditary osteodystrophy)
Patient presents with amenorrhea, galactorrhea, low libido and has been undergoing treatment for infertility. Bitemporal hemianopia is observed on exam. What is the treatment?
Dopamine agonists (bromocriptine or cabergoline)
Patient presents with large tongue, deep voice, large hands and feet, coarse facial features. Patient also with insulin resistance DM. What is the diagnosis and treatment for this disease?
Dx: increased serum IGF-1; Treatment: pituitary adenoma resection followed by somatostatin analog if not cured
What is the difference between central and nephrogenic DI.
Central is due to lack of ADH while nephrogenic is due to lack of renal response to ADH
Compare the different etiologies of central and nephrogenic DI.
Central: pituitary tumor, trauma, surgery, histiocytosis X; Nephrogenic: hereditary or secondary to hypercalcemia, lithium, demeclocycline
What are the findings consistent with DI (urine specific gravity and serum osmolality)?
Urine specific gravity 290mOsm/L
How is DI diagnosed? What is the test used to distinguish central from nephrogenic DI?
Water deprivation test: urine osmolality does not increase; response to desmopressin distinguishes central vs. nephrogenic
What is the treatment for central DI?
Adequate fluid intake; intranasal desmopressin
What is the treatment for nephrogenic DI?
Adequate fluid intake; hydrochlorothiazide, indomethacin, amiloride
T/F: In SIADH, serum osmolarity > urine osmolarity
FALSE: Urine osmolarity > serum osmolarity
What are some causes of SIADH?
Ectopic ADH (small cell lung cancer), CNS disorders/head trauma, pulmonary disease, drugs (eg. cyclophosphamide)
How is SIADH treated?
Fluid restriction, IV saline, conivaptan, tolvaptan, demeclocycline
Patient with significant postpartum bleeding presents with failure to lactate. What is the diagnosis?
Sheehan’s syndrome: Ischemic infarct of pituitary following postpartum bleeding
What is empty sella syndrome?
Atrophy or compression of pituitary, often idiopathic, common in obese women
Patient presents with polydipsia, polyuria, polyphagia, weight gain. What tests would you consider ordering to diagnose this patient?
Fasting serum glucose, oral glucose tolerance test, HbA1c
What are some small vessel disease manifestations of DM?
Retinopathy, glaucoma, nephropathy
What are some large vessel disease manifestations of DM?
Large vessel atherosclerosis, CAD, peripheral vascular occlusive disease, gangrene
How does osmotic damage occur in diabetics? What are 2 clinical manifestations?
Sorbitol accumulation in organs with aldose reductase; neuropathy and cataracts
State whether the following is more commonly associated with type 1 or type 2 DM: autoimmune destruction of beta cells; insulin treatment is always necessary; associated with obesity; low insulin sensitivity; low beta cell numbers in the islets; islet amyloid deposit.
Type 1; 1; 2; 2; 1; 2
Patient with DM1 presents with deep, rapid breathing, nausea, vomiting, abdominal pain, fruity breath odor. How do you treat this patient?
IV fluids, IV insulin, K+ (to replete intracellular stores); glucose if necessary to prevent hypoglycemia
What would you see on the labs for a patient in DKA?
Hyperglycemia, inc H+, dec HCO3- (anion gap metabolic acidosis), inc blood ketone levels, leukocytosis, hyperkalemia
Explain how a patient in DKA would have hyperkalemia on labs but depleted intracellular K+.
This is due to transcellular shift from decreased insulin
What are some complications of DKA?
Mucormycosis (life-threatening), Rhizopus infection, cerebral edema, cardiac arrhythmias, heart failure
Patient presents with recurrent diarrhea, cutaneous flushing, asthmatic wheezing, and right-sided valvular disease. Increased 5-HIAA is seen in urine. How should you treat the patient?
Somatostatin analog (eg. octreotide) (patient has carcinoid syndrome)
What is the Rule of 1/3’s associated with carcinoid syndrome?
1/3 metastasize, 1/3 present with 2nd malignancy, 1/3 multiple
What is the most common tumor of the appendix?
Carcinoid syndrome
Patient presents with recurrent ulcers. On upper endoscopy, stomach shows rugal thickening with acid hypersecretion. What is the most likely diagnosis?
Zollinger-Ellison syndrome
Name the tumors associated with MEN1 (Wermer’s syndrome).
Pituitary, Parathyroid, Pancreas
Name the tumors associated with MEN2A (Sipple’s syndrome).
Parathyroid, Pheochromocytoma, medullary thyroid carcinoma
Name the tumors associated with MEN2B.
Pheochromocytoma, medullary thyroid carcinoma, oral/intestinal ganglioneuromatosis (associated with marfanoid habitus)
What is the inheritance pattern of all MEN syndromes?
Autosomal dominant
MEN2A and 2B are associated with what gene mutation?
Ret gene mutation