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Pathology 2 - Block 3 - Part 5

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davidwurbel7's version from 2016-08-01 04:20

Parathyroid Disorders

Question Answer
Chief cells synthesize thisParathormone (PTH)
Parathormone (PTH) is synthesized by these cellsChief Cells
Functions of this activates osteoclasts (mobilizes Ca+2 from bone), Increases resorption of calcium from renal tubules. Increases the conversion of vitamin D to its active form. Increases Ca+2 absorption from GIT. Increases urinary phosphate excretionParathormone (PTH)
PTH secretion is controlled by thisFree Ionized Calcium
Calcium is bound to this protein in the bloodAlbumin
Causes decreased total serum calcium. Normal free Ca levels, normal PTH. No evidence of tetanyHypoalbuminemia
This increases negative charges on albumin. Extra negative charges bind some of the free Ca. Total serum calcium remains normal. Decreased free calcium, increased PTH. Patient develops tetanyAlkalosis
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(Insert 10)
Question Answer
Unregulated overproduction of PTH by the parathyroid leading to hypercalcemia. MCC of hypercalcemia in general population. Most often occurs in women older than 50 years of agePrimary Hyperparathyroidism
The most common cause of Primary Hyperparathyroidism is thisParathyroid Adenoma
A benign tumor, Solitary & well circumscribed. Right inferior parathyroid gland is most often involved. Composed of sheets of chief cells with no intervening fat. Remainder of the gland plus all other glands show atrophyParathyroid adenoma
Most cases of this are asymptomatic. Discovered accidentally by finding hypercalcemia on biochemical profilePrimary Hyperparathyroidism
Clinical findings include Calcium oxalate stones = nephrolithiasis, Nephrocalcinosis (causes polyuria and renal failure. Peptic ulcer disease: calcium stimulates gastrin release which increases gastric acid. Acute pancreatitis: calcium activates phospholipases. Constipation: Most common. Cystic and hemorrhagic bone lesion. Caused by increased osteoclastic activity. Commonly involves the jaw and fingers. Aggregates of osteoclasts, reactive giant cells and hemorrhage mimicking neoplasm called the "brown tumor" of hyperparathyroidismPrimary Hyperparathyroidism
The most common cause of this is chronic renal failureSecondary Hyperparathyroidism
The most common cause of this is accidental removal during thyroid surgeryPrimary Hypoparathyroidism
This is required for PTH activationMagnesium
This condition has a congenital absence of parathyroid glandsDi George Syndrome
The clinical findings include Tetany, Calcification of basal ganglia, Cataracts, Candida infection and Prolonged QT intervalPrimary Hypoparathyroidism
This is due to decreased ionized calcium in primary hypoparathyroidismTetany
Sex linked inherited disorder characterized by end organ resistance (kidney) to PTHPseudohypoparathyroidism
Clinical findings include Mental retardation, Calcification of basal ganglia, Short 4th and 5th metacarpals and Short stature and other skeletal abnormalitiesPseudohypoparathyroidism
Cause of hypocalcemia do to calcium bound to fatty acids in enzymatic fat necrosisAcute pancreatitis
The most common cause of hypocalcemiaHypoalbuminemia
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Lab Findings
Question Answer
PTH: Elevated Ca+2: Elevated PO4: DecreasedPrimary Hyperparathyroidism
PTH: Decreased Ca+2: Increased PO4: DecreasedParathyroid Malignancy
PTH: Decreased Ca+2: Decreased PO4: ElevatedPrimary Hypoparathyroidism
PTH: Elevated Ca+2: Decreased PO4: ElevatedVitamin D Deficiency/Renal Failure
PTH: Normal/Elevated Ca+2: Decreased PO4: ElevatedPseudohypoparathyroidism
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Adrenal Glands 1

Question Answer
Secreted by anterior pituitary. Stimulates growth of adrenal cortex. Stimulates release of Glococorticoids (mainly), Andogens and MineralocorticoidsAdrenocorticotropic hormone (ACTH)
The outer most layer of the adrenal cortexZona Glomerulosa
Zona Glomerulosa produces this hormoneAldosterone
The middle layer of the adrenal cortexZona Fasciulata
Zona Fasciulata produces this hormoneCortisol
The inner most layer of the adreal cortexZona Reticularis
Zona Reticularis produces this hormoneAndrogens
Tumor of the adrenal medulla in adultsPheochomocytoma
Tumor of the adrenal medulla in childrenNeuroblastoma
Excess of aldosteroneHyperaldosteronism
Excess of cortisolCushing’s Syndrome
Excess of androgensAdrenogenital Syndrome
This hormone is involved in regulation of metabolism, Counteracts the action of insulin, Supports blood pressure and vasomotor tone. Also involved in regulation of behavior and immunosuppressionCortisol
Due to a pituitary adenoma. Increased ACTH and cortisol. Bilateral adrenal hyperplasiaPituitary Cushing Syndrome (Cushing disease)
Most often due to an adrenal adenoma producing cortisol. Decreased ACTH and increased cortisolAdrenal Cushing Syndrome
Due to administration of exogenous cortisol. Decreased ACTH. Bilateral adrenal atrophyIatrogenic Cushing Syndrome
Usually small cell carcinoma of lung. Ectopic ACTH production. Markedly increased ACTH and cortisolEctopic Cushing Syndrome
Clinical findings include weight gain, Fat deposition in Face, Upper back, Trunk and thin extremities, Purple abdominal striae, Glucose intolerance/diabetes, Hypertension, Hirsutism, Osteoporosis, Plethoric face, menstrual irregularities, acne, easy bruisability, depression and insomniaCushing’s Syndrome
High dose Dexamethasone suppression test is able to suppress ACTHPituitary Cushing's
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Lab Values
Question Answer
Cortisol: Elevated ACTH: Normal to Elevated Low dose Dexamethasone suppression test: Not Suppressed High dose Dexamethasone suppression test: SuppressedPituitary Cushing's
Cortisol: Elevated ACTH: Decreased Low dose Dexamethasone suppression test: Not Suppressed High dose Dexamethasone suppression test: Not SuppressedAdrenal Cushing's
Cortisol: Elevated ACTH: Markedly Elevated Low dose Dexamethasone suppression test: Not Suppressed High dose Dexamethasone suppression test: Not SuppressedEctopic Cushing's
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Question Answer
Most commonly due to aldosterone producing benign adenoma in zona glomerulosaPrimary Hyperaldosteronism
Primary Hyperaldosteronism is also called thisConn's Disease
While taking the BP, the thumb adducts into the palm. This is a sign of tetanyTrousseau’s Sign
Clinical findings include Muscle weakness, Tetany, Polyuria, polydipsia and Hypertension. Laboratory findings include Hypernatremia, Hypokalemia, Increased plasma aldosterone level. Decreased plasma renin activity, Aldosterone/Renin ratio increased and Metabolic alkalosisPrimary Hyperaldosteronism
A consequence of the stimulation of the adrenal cortex by the renin-angiotensin systemSecondary Hyperaldosteronism
Congestive heart failure, Atherosclerosis of the aorta and Renal artery stenosis are conditions associated with thisSecondary Hyperaldosteronism
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Lab Values
Question Answer
Aldosterone: Elevated Na+: Elevated K+: Decreased Blood pH: Increased Renin: Decreased Ald/Renin Ratio: IncreasedPrimary Hyperaldosteronism
Aldosterone: Elevated Na+: Elevated K+: Decreased Blood pH: Increased Renin: Increased Ald/Renin Ratio: DecreasedSecondary Hyperaldosteronism
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Question Answer
Characterized by excess production of androgens causing virilization in females and precocious puberty in malesAdrenogenital Syndromes
Group of autosomal recessive disorders. Characterized by deficiency or total lack of enzyme required for synthesis of cortical steroidsCongenital Adrenal Hyperplasia
Clinical findings includes ambiguous genitalia in females, precocious puberty in males, hypotension and decrease in 17-OH21-Hydroxylase Deficiency
Clinical findings includes ambiguous genitalia in females, precocious puberty in males, hypertension and increase in 17-OH11-Hydroxylase Deficiency
Clinical findings includes ambiguous genitalia in females, precocious puberty in males, mild hypotension and slight decrease in 17-OH in an older child or adolescentPartial 21-Hydroxylase Deficiency
Clinical findings includes ambiguous genitalia in females, precocious puberty in males, mild hypertension and slight increase in 17-OH in an older child or adolescentPartial 11-Hydroxylase Deficiency
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Adrenal Glands 2

Question Answer
A state of inadequate cortisol and aldosterone production be the adrenal glandsAdrenocortical Insufficiency
Life threatening condition due to low cortisolAcute Adrenocortical Insufficiency
The most common cause of acute adrenocortical insufficiency is thisSudden Withdraw of Corticosteroid
An infection by this bacteria can lead to Waterhouse-Friderichesen syndromeNeisseria
Bilateral hemorraghorric necrosis of the adrenal glands due to bacterial infectionWaterhouse-Friderichesen Syndrome
Clinical findings include hypotension, abdominal pain, nausea and vomiting, often fever, hyponatremia, hyperkalemia and eosinophiliaAcute Adrenocortical Insufficiency
Clinical findings include profound fatigue, anorexia, weight loss, hypotension, nausea and vomiting, hyponatremia, hyperkalemia, eosinophilia, skin hyperpigmentationChronic Adrenocortical Insufficiency
Bilateral hemorrhagic infarction of adrenal glands with acute adrenal insufficiency. Usually associated with septicemia from N. meningitidis, purpuric rash. Eventually patients develop Endotoxic shock which results in DIC. Commonly occurs in children. Often fatalWaterhouse-Friderichesen Syndrome
Destruction of the adrenal cortex, leading to a deficiency of glucocorticoids, mineralocorticoids and androgensAddison's Disease
Weakness, Hypotension, Skin hyperpigmentation. Lab findings: Hyponatremia, hyperkalemia, metabolic acidosis (decreased aldosterone). Hypoglycemia. Treatment: steroid and fluid replacementAddison's Disease
Yellow to orange color. Can be functional or nonfunctionalAdrenal Adenoma
Yellow but contain area of hemorrhage and necrosis. Can be functional or nonfunctionalAdrenal Cortical Carcinoma
Most commonly occur in patients between 30-50 yrs of age. Derived from chromaffin cells. Tumors cells secrete Catecholamine causing episodic hypertensionPheochromocytoma
Clinical findings include Hypertension (sustained with occasional bursts), Palpitations, Sense of apprehension and anxiety, Drenching Sweats, Headache and chest painPheochromocytoma
Metabolic products of EpinephrineMetanephrine
Metabolic products of NorepinephrineVanillylmandelic Acid (VMA)
Metabolic product of dopamineHomovanillic Acid (HVA)
A pheochromocytoma is composed of large cells that are pink and arranged in nests with capillaries in betweenZell-Ballen Pattern
Tumor composed of polygonal to spindle cells arranged in small nests. Malignancy excluded by absence of vessel invasion and metastasis. Ultra-structurally: contain dense core neurosecretory granulesPheochromocytoma
Composed of small, primitive-appearing   cells with dark nuclei, scant cytoplasm, and poorly defined cell borders growing in solid sheetsNeuroblastoma
Tumor cells are concentrically arranged around central space filled with neuropilHomer-Wright Pseudorosettes
A Malignant tumor from adrenal medulla. Primarily seen in children younger than 5 years of age. Deletion or rearrangement of short arm of chromsome 1 leading to amplification of N-myc oncogene. Presentation: Palpable abdominal mass, Hypertension. Metastasis to skin and boneNeuroblastoma
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MEN Syndromes

Question Answer
Parathyroid tumor (hypercalcemia), Pancreatic tumor (gastrin) and Pituitary adenoma (prolactin , ACTH)MEN-1
Medullary carcinoma of thyroid (calcitonin), Pheochromocytoma (catecholamines) and Parathyroid tumor (hypercalemia)MEN-2a
Medullary carcinoma of thyroid (calcitonin), Pheochromocytoma (catecholamines) and Marfanoid habitus, Mucosal neuromasMEN-2b
Linked to mutations in MEN I geneMEN 1
Linked to mutations in RET protooncogeneMEN 2a and MEN2b
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