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146 Cards in this Set
- Front
- Back
What hormones are produced by the basophils of the anterior pituitary?
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- FSH
- LH - ACTH - TSH ** B-FLAT ** |
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What hormones are produced by the acidophils of the anterior pituitary?
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- Growth hormone
- Prolactin |
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What is the only hypothalamic hormone (pro-hormone) that is inhibitory in nature?
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Dopamine (Prolactin is under tonic inhibition by dopamine)
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What is the anterior lobe of the pituitary (adenohypophysis) derived from embryologically?
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Rathke's Pouch (upward extension of the oral canal)
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What is the posterior lobe of the pituitary (neurohypophysis) derived from embryologically?
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Outpouching on the floor of the 3rd ventricle (consists of pituicytes and axonal process from the hypothalamus)
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What is the most common cause of hyperpituitarism (increased secretion of stimulatory hormones)?
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Pituitary adenoma (of the anterior lobe)
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What visual field abnormality is commonly associated with pituitary masses?
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Bitemporal hemianopsia (defects in lateral (temporal) fields
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Germline mutations in which tumor suppressor genes are strongly associated with pituitary adenoma?
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MEN-1 (Chromosome 11q13, most commonly GH-, prolactin-, or ACTH- secreting tumors)
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What histological finding is indicative of pituitary adenoma?
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Absence of usual reticulin supporting network
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What physical/clinical findings are associated with prolactinoma (prolactin secreting pituitary adenoma)?
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- Galactorrhea
- Amenorrhea - Sexual dysfunction - Infertility |
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What is the most frequent type of hyperfunctioning pituitary adenoma?
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Prolactinoma (may undergo dystrophic calcifications, resulting in a "pituitary stone"
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What term refers to any disorder that interferes with dopamine delivery from hypothalamus to prolactin secreting cells in anterior pituitary?
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Stalk effect (may result in a mild increase in serum prolactin; mild prolactinemia)
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What type of pituitary adenoma expresses both GH and prolactin?
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Bihormonal mammosomatotroph adenomas
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What are the physical/clinical findings associated with GH adenoma?
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- Gigantism (if prior to closue of epiphyseal plates)
- Acromegaly (if after close of epiphyseal plates) |
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What pituitary tumor is usually microadenomas and basophilic at the time of diagnosis?
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Corticotroph cell adenomas (excess ACTH production)
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What term refers to hypercortisolism of any cause?
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Cushing Syndrome
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What term refers to hypercortisolism as a result of excess production of ACTH?
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Cushing disease
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What term refers to sudden hemorrhage into pituitary?
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Pituitary apoplexy (may cause sudden onset of excruciating headache, diplopia, and hypopituitarism (neurosurgical emergency)
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What syndrome is the most common cause of ischemic necrosis of anterior pituitary?
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Sheehan syndrome (Post-partum necrosis of anterior pituitary)
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What posterior pituitary syndrome is characterized by polyuria due to inability of the kidney to resorb water from the urine?
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Diabetes insipidous (ADH deficiency, central ADH deficiency, nephrogenic - renal tubular unresponsiveness to ADH)
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What posterior pituitary syndrome is characterized by persistent secretion of ADH resulting in the inability to secrete a dilute urine?
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SIADH (Syndrome of Inappropriate ADH)
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What are the clinical findings associated with SIADH?
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- Hyponatremia
- Cerebral edema, neurological dysfunction |
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What syndrome may result from paraneoplastic secretion of ADH by small cell carcinoma of the lung?
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SIADH
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What hypothalamic supersellar tumor is derived from vestigial remnants of Rathke's pouch?
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Craniopharyngiomas (cause viusal disturbances in adults; growth retardation due to pituitary hypofunction and GH deficiency)
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What cells have neuroendocrine and photosensory functions?
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Pineocytes
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What type of tumor are most pineal gland tumors?
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Germinomas
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Which pinealoma is poorly differentiated and highly malignant?
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Pinealoblastoma
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Which pinealoma is well differentiated and indolent?
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Pineocytoma
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What cells of the thyroid line follicles and secrete T3 and T4?
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Follicular epithelial cells (columnar follicular epithelial cells = active; cuboidal follicular epithelial cells = inactive)
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What cells of the thyroid are neuroendocrine in origin and secrete calcitonin?
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Parafollicular cells (secrete calcitonin)
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What is the best overall screening test for thyroid function?
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Serum TSH
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What pathology is associated with a nodule that has increased uptake of radioactive iodine ("Hot" Nodule)?
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Toxic nodular goiter
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What pathologies are associated with a nodule that has decreased uptake of radioactive iodine ("Cold" Nodule)?
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Cyst and cancer
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What term refers to the failed descent of thyroid anlage from the base of the tongue that usually represents all of the thyroid tissue?
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Lingual Thyroid
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What are the clinical findings associated with acute thyroiditis?
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- Fever
- Painful cervical adenopathy - Initial Thyrotoxicosis from gland destruction |
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What etiology is associated with acute thyroiditis?
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Bacterial infection (i.e. Staphylococcus aureus)
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What is the most common cause of a painful thyroid gland?
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Subacute granulomatous thyroiditis (granulomatous inflammation with multinucleated giant cells)
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What etiology is associated with subacute granulomatous thyroiditis?
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Viral infection (i.e Coxsackievirus)
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What is the most common cause of hypothyroidism?
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Hasimoto's thyroiditis
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What term refers to the initial thyrotoxicosis that results from Hashimoto's thyroiditis?
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Hashitoxicosis
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What type of thyroiditis is characterized by fibrous replacement of gland and surrounding tissue?
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Reidel's thyroiditis (hard, fixed thyroid mass that mimics thyroid carcinoma)
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What type of thyroiditis is autoimmune in nature and develops postpartum?
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Subacute painless lymphocytic thyroiditis
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What is cretinism?
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Hypothyroidism in infancy or early childhood that results in severe mental retardation, increased weight, and short stature
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What is the most common cause of cretinism?
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Maternal hypothyroidism (especially if occurs before fetal thyroid is developed; the brain requires thyroxine for maturation)
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What are the laboratory findings associated with primary hypothyroidism?
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- Decreased T4 & T3
- Increased TSH |
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What is most common cause of congenital hypothyroidism?
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Iodine deficiency (worldwide most common cause)
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What clinical findings fall under the umbrella of myxedema (adult hypothyroidism)?
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- Lethargy
- Fatigue - Cold intolerance - Weight gain - Periorbital puffiness - Hoarse voice - Cardiomyopathy - Mental slowness - Dry brittle hair - Coarse yellow skin - Large tongue |
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What is the treatment for hypothyroidism?
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- Levothyroxine sodium
- Bring serum TSH into the normal range |
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What term refers to excess thyroid hormone regardless of the cause?
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Thyrotoxicosis
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What term refers to excess thyroid hormones due to increased thyroid hormone synthesis by the thyroid gland?
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Hyperthyroidism
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What is the most common cause of hyperthyroidism and thyrotoxicosis?
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Grave's disease (female dominant autoimmune disease)
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What disease results from thyroid-stimulating (IgG) antibodies against TSH receptor?
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Grave's disease
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What physical/clinical findings are unique to Grave's disease?
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- Exopthalmos
- Pretibial myxedema |
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What test should be ordered in a patient with atrial fibrillation to rule out hyperthyroidism?
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Serum TSH test
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Which thyroid disease is characterized by diastolic hypertension?
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Hypothyroidism (due to retention of sodium and water)
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Which thyroid disease is characterized by systolic hypertension?
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Hyperthyroidism and Thyrotoxicosis (
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What are the physical/clinical findings associated with thyrotoxicosis?
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- Weight loss (good appetite)
- Fine tremor of the hands - Heat intolerance - Diarrhea - Anxiety - Lid stare - Sinus tachycardia - Atrial fibrillation - Systolic hypertension |
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What are the thyroid hormone levels associated with Grave's disease?
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- Decreased TSH
- Increased T4 and T3 - Increased uptake of radioactive iodine (I131) |
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What is non-toxic goiter?
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Thyroid enlargement from excess colloid
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What is the most common cause of non-toxic goiter?
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Endemic type (due to iodine deficiency)
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What is toxic multinodular goiter (Plummer's disease)?
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One or more nodules in multinodular goiter become TSH independent
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What is the first step in the management of a thyroid nodule?
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Fine needle aspiration
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What is the most common benign tumor of the thyroid gland?
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Follicular adenoma (surrounded by a complete capsule; "Cold" Nodule)
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What is the most common cancer of the thyroid gland?
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Papillary adenocarcinoma (associated with radiation exposure) - overall has a good prognosis
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What are unique histological findings associated with Papillary adenocarcinoma of the thyroid?
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- Psammoma bodies (dystrophically calcified cancer cells)
- Empty appearing nuclei (Orphan Annie nuclei) |
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What type of thyroid cancer favors hematogenous spread rather than lymphatic spread?
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Follicular carcinoma (neoplastic follicles invade blood vessels)
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What type of thyroid cancer is associated with autosomal dominant mutations in MEN IIa and IIb?
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Medullary Carcinoma (Familial type ~ 20% of cases)
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What endocrine syndrome is comprised of thyroid medullary carcinoma, hyperparathyroidism, & pheochromocytoma?
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MEN II (aka MEN IIa)
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What endocrine syndrome is comprised of thyroid medullary carcinoma, mucosal neuromas (lips/tongue), & pheochromocytoma?
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MEN III (MEN IIb)
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What thyroid tumor is derived from parafollicular C cells?
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Medullary carcinoma
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What type of thyroid tumor most often develops from Hashimoto's thyroiditis?
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Primary B-cell malignant lymphoma
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What type of thyroid cancer most often occurs in elderly women?
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Anaplastic thyroid cancer (rapidly aggressive and uniformly fatal)
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What mutations are associated with Papillary thyroid carcinoma?
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Mutations in RET gene (RET/PTC fusion proteins) and mutations in BRAF gene.
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What mutations are associated with Follicular carcinoma of the thyroid?
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- Activation of PI-3K/AKT pathway (RAS mutations)
- PAX8:PPARG translocation |
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What are "Water Clear" cells?
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Chief cells containing abundant glycogen (Chief Cells of the Parathyroid)
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What are "Oxyphil" cells?
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Chief cells containing numerous mitochondria
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What are the metabolic functions of parathyroid hormone?
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- Increase calcium absorption in the early distal tubule
- Increase conversion of Vit. D to acitve Vit. D in the kidney - Increase urinary excretion of phosphate - Increase intestinal absorption of calcium PTH = Phosphate trashing hormone |
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What is the most common cause of symptomatic hypercalcemia?
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Malignancy
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What is the most common cause of asymptomatic hypercalcemia?
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Primary hyperparathyroidism
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What protein is secreted by many solid tumors resulting in increased bone resorption?
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PTH-related protein (PTHrP)
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What disease state refers to autonomous spontaneous overproduction of PTH by parathyroid?
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Primary hyperparathyroidism (most common non-malignant cause of hypercalcemia)
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What disease state refers to secondary phenomena in patients with chronic renal insufficiency?
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Secondary and tertiary hyperparathyroidism
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What is the most common cause of primary hyperparathyroidism?
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Adenoma - 85% of cases (sheets of chief cells with no intervening adipose)
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What autosomal dominant mutations are associated with Familial hypocalciuric hypercalcemia?
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Inactivating mutations in calcium sensing receptor gene (CASR)
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What is a malignant diagnosis of parathyroid carcinoma base upon?
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- Local invasiveness
- Metastasis |
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What etiology associated with primary hyperparathyroidism involves all (or multiple) glands?
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Primary parathyroid hyperplasia (usually a chief cell hyperplasia)
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What is the most common manifestation of primary hyperparathyroidism?
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Increase in serum ionized calcium
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What are the renal findings associated with symptomatic hyperparathyroidism ("renal stones")?
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- Calcium stones
- Nephocalcinosis (cause polyuria and renal failure) |
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What are the gastrointestinal findings associated with symptomatic hyperparathyroidism ("abdominal groans")?
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- Peptic Ulcer disease (PUD)
- Acute pancreatitis - Constipation |
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What are the bone findings associated with symptomatic hyperparathyroidism ("painful bones")?
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- Osteoporosis
- Osteitis fibrosa cystica (cystic and hemorrhagic bone lesion) - Fractures |
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What is osteitis fibrosa cystica?
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Cystic and hemorrhagic bone lesion as a result on increased osteoclastic activity stimulated by hyperparathyroidism.
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What are the laboratory findings associated with hyperparathyroidsim?
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- Increased PTH
- Increased calcium (hypercalcemia) - Decreased phosphorous (hypophosphatemia) |
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What is the most common cause of secondary hyperparathyroidism?
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Renal failure (CRI, CKI, CKD)
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What is hypoparathyroidism?
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Decreased secretion of PTH accompanied by hypocalcemia.
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What is the most common cause of hypoparathyroidism?
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Surgery: inadvertent removal of all glands
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What is the most common pathologic cause of hypocalcemia in the hospital?
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Hypomagnesemia (magnesium if a cofactor required for adenylate cyclase; cAMP is required for PTH activation)
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What are the causes of hypoparathyroidism?
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- Previous thyroid surgery
- Autoimmune hypoparathyroidism - DiGeorge syndrome (absence of parathyroid glands) |
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What term refers to the clinical finding of hypoparathyroidism that result from tapping along the course of the facial nerve resulting in contractions of muscles of eye, mouth, & nose?
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Chvostek sign (tapping along course of facial nerve resulting in contractions of muscles of eye, mouth, and nose)
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Occluding circulation of forearm and hand by inflating blood pressure cuff about the arm resulting in a carpal spasm?
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Trousseau sign (indication of hypoparathyroidism)
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What are the clinical findings associated with hypoparathyroidism?
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- Emotional instability, anxiety, depression
- Tetany - Calcification of basal ganglia (increased phosphorous drive calcium into brain tissue) - QT prolongation - |
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What disease/deficiency results from end organ resistance to PTH?
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Pseudohypoparathyroidism
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What is the function of the chromaffin cells of the adrenal medulla?
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Synthesize and secrete catecholamine (epinephrine and norepinephrine).
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What neoplasm is derived from the chromaffin cells of the adrenal medulla?
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Pheochromocytoma
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What are the episodic hyperadrenergic symptoms associated with pheochromocytoma (5Ps)?
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- Pressure (elevated BP = HTN)
- Pain (headache) - Perspiration - Palpitations (tachycardia) - Pallor |
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What is the Rule of 10s associated with pheochromocytoma?
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- 10% extra-adrenal
- 10% malignant - 10% bilateral - 10% not associated with HTN - 25% associated with germline mutations |
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What are the germline mutations associated with pheochromocytomas?
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- SDHB, SDHC, SDHD
- RET - NF1 - VHL |
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What are the laboratory findings associated with pheochromocytoma?
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- Increased urinary catecholamines, VMA, and metanephrine
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What is a the treatment for an isolated benign pheochromocytoma?
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Surgical excision after pre-operative and intraoperative medication with phenoxybenzamine.
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What is a paraganglioma?
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Tumors of extra-adrenal paraganglia
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What term refers to a paraganglioma of the carotid body?
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Carotid body tumor
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What term refers to a paraganglioma of the jugulotympanic body?
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Chemodectoma
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What hormones are produced by the zona glomerulosa of the adrenal cortex (outermost layer)?
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- Mineralocorticoids (i.e. Aldosterone) regulates "salt" (sodium/potassium)
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What hormones are produced by the zona fasiculata of the adrenal cortex (middle layer)?
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Glucocorticoids (i.e. cortisol) regulates "sugar" (gluconeogenesis)
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What hormones are produced by the zona reticularis of the adrenal cortex (innermost layer)?
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Sex steroids (i.e. estrogens and androgens) regulates "sex"
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What is the most common cause of hypercortisolism (Cushing syndrome)?
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Exogenous (iatrogenic) ingestion of steroids.
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What is the most common cause of ACTH-independent Cushing syndrome?
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Primary adrenal neoplasms (i.e. adenomas & carcinomas) increase serum cortisol and decrease ACTH.
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A majority of the cases of adrenal hyperplasia are ACTH-dependent or ACTH-independent?
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Majority are ACTH-dependent
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What happens to the adrenal glands morphologically as a result of exogenous Cushing syndrome?
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Atrophy of the adrenal glands due to lack of stimulation by ACTH.
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What are the Cushignoid symptoms associated with Cushing syndrome?
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- Moon facies
- Truncal obesity - Buffalo hump - Osteoporosis - Thinning of skin - Abdominal striae (purple striae) - Amenorrhea |
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What test is used to diagnose and individual with hypercortisolism?
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- 24 hr urine free cortisol (increase in 24 hr urine free cortisol and loss of diurnal pattern of cortisol secretion are diagnostic of hypercortisolism)
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What effect does dexamethasone have on an individual without hypercortisolism?
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Suppress cortisol
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What will happen to cortisol levels following administration of dexxamethasone if the cause of the hypercortisolism is a pituitary tumor?
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- No Change in cortisol (fail to suppress) following low dose dex
- Decrease in cortisol (suppressed) following high dose dex |
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What will happen to cortisol levels following administration of dexamethasone if the cause of the hypercortisolism is ectopic in origin?
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- No change in cortisol (fail to suppress) following both low and high dose dexamethasone.
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What will happen to cortisol levels following administration of dexamethasone if the cause of the hypercortisolism is an adrenal tumor?
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- No change in cortisol (fail to suppress) following both low and high dose dexamethasone.
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What group of syndromes is characterized by chronic increase in aldosterone resulting in sodium retention and potassium excretion, HTN, & sometimes hypokalemia?
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- Hyperaldosteronism
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What clinical findings are associated with primary hyperaldosteronism?
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- Decrease in plasma renin
- Increase in blood pressure |
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What are the three causes of primary hyperaldosteronism?
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- Bilateral idiopathic hyperaldosteronism
- Adrenal cortical neoplasm - Glucocorticoid suppressible |
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What is the most common cause of primary hyperaldosteronism?
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- Bilateral idiopathic hyperaldosteronism
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What adrenal cortical neoplasm is most likely to cause primary hyperaldosteronism?
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Solitary aldosterone-secreting adenoma (Conn syndrome)
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What are the main clinical manifestations associated with primary hyperaldosteronism?
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- Excess aldosterone resulting in sodium retention and potassium excretion
- HTN - primary hyperaldosteronism may be the most common cause of secondary HTN |
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What type of hyperaldosteronism results in response to activation of the renin-angiotensin system?
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Secondary hyperaldosteronism (increase in plasma renin)
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What adrenocortical neoplasm is most likely to be associated with virilization?
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Androgen-secreting carcinomas of the adrenal cortex.
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What adrenocortical enzyme deficiency associated with 90% of cases of adrenal virilism presents with increase in testosterone, decrease in aldosterone, and decrease cortisol?
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21-hydroxylase deficiency
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What type of syndrome results from a complete lack of 21-hydroxylase?
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Salt-wasting syndrome (virtually no mineralocorticoid synthesis and deficient cortisol synthesis)
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What clinical findings are a associated with Salt-wasting syndrome?
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- Hyponatremia & hyperkalemia
- Clitoral enlargement in females (virilization) |
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What syndrome results from a partial deficiency in 21-hydroxylase function?
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Non-classical adrenal virilism
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What syndrome is characterized by rapid onset of adrenocortical insufficiency as as result of massive adrenal hemorrhage?
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Waterhouse-Friderichsen syndrome
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What is the underlying etiologic agent associated with Waterhouse-Friderichsen syndrome?
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Neisseria meningitidis (mostly occurs in children)
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What autoimmune adrenalitis is characteiized by autoimmune adrenal insufficiency, other AI disorders, mucocutaneous candidiasis, and skin disorders?
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Autoimmune polyendocrine syndrome 1 (APS1)
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What autoimmune adrenalitis is characterized by autoimmune adrenal insuffciency, other AI disorders without mucocutaneous candidiasis and skin disorder?
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Autoimmune polyendocrine syndrome 2 (APS2)
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What clinical physical findings helps distinguish between primary and secondary adrenal insufficiency?
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Skin hyperpigmentation (observed in primary not secondary adrenal insufficiency).
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What laboratory findings are associated with primary adrenal insufficiency?
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- Increased ACTH
- Decreased glucose **No response to exogenous ACTH due to destruction of adrenal cortex** |
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What laboratory findings are associated with secondary adrenal insufficiency?
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- Decreased ACTH
**Exogenous ACTH causes an increase in plasma cortisol** |
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What three tumors (3Ps) are associated with MEN-1 (Wermer's Syndrome)?
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- Parathyroid tumors
- Pituitary tumors - Pancreatic endocrine tumors **Commonly presents with kidney stones (hyperparathyroidism induced hypercalemia) and stomach ulcers (ZES gastrinoma induced recurrent ulcers)** |
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What three tumors (2Ps) are associated with MEN-2A (Sipple's Syndrome)?
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- Medullary Carcinoma (increased secretion of calcitonin)
- Parathyroid tumors - Pheochromocytoma |
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What three tumors (1P) are associated with MEN-2B syndrome?
|
- Medullary Carcinoma (increased secretion of calcitonin)
- Pheochromocytoma - Mucosal neuromas/ganglioneuromas **Marfanoid habitus is also associated with MEN-2B syndrome** |