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146 Cards in this Set

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