Ch 15 Pathoma Endocrine Pathology Flashcards
Endocrine system is a group of __ that maintain body __. Functions by release of __ that travel via __ to distant organs. ___ mechanisms control hormone release.
glands; homeostasis; hormones; blood; feedback
Pituitary adenoma is a benign/malignant tumor of anterior/posterior pituitary cells. May be functional (___) or nonfunctional (___).
benign; anterior; hormone-producing; silent
Nonfunctional pituitary adenomas often present with __. Name 3 presentations and why they occur
mass effect; bilateral hemianopsia (due to compression of the optic chiasm); hypopituitarism (due to compression of normal pituitary tissue); headache
Functional pituitary adenomas present with features based on __ produced. Name 6, and which is the most common
hormone; 1) growth hormone cell adenoma; 2) ACTH cell adenoma; 3) TSH cell; 4) LH producing; 5) FSH producing adenoma (the last three are rare); 6) prolactinoma (most common)
Prolactinoma presents as __ and __ (females) or as __ and __ (males). Treatment is ___ (e.g. __ or __) to suppress prolactin production (shrinks tumor) or __ for larger lesions.
galactorrhea; amenorrhea; decreased libido; headache (amenorrhea and decreased libido are due to negative feedback inhibiting release of GnRH); dopamine agonist; bromocriptine; cabergoline; surgery
Growth hormone cell adenoma causes __ in children. There is increased __ growth, since the __ are not fused. It causes __ in adults, which presents with enlarged __ of these 3 areas, growth of __ organs leading to dysfunction (e.g. __) and enlarged __.
gigantism; linear bone; epiphyses; acromegaly; bones; hands, feet, and jaw; visceral (cardiac failure - most common cause of death in these pts); tongue
With a growth hormone cell adenoma, secondary ___ is often present (GH induces __). GH cell adenomas are diagnosed by elevated __ and __ levels along with lack of __ suppression by __. Tx is __ (__ analog that suppresses GH release), __ antagonists, or __.
diabetes mellitus; liver gluconeogenesis; GH; insulin growth factor-1 (IGF-1); GH; oral glucose; octreotide; somatostatin; GH receptor; surgery
ACTH cell adenomas secrete __ leading to __.
ACTH; Cushing syndrome
Hypopituitarism is insufficient production of __ by the __. Symptoms arise when greater than __% of the __ is lost. Name 3 causes.
hormones; anterior pituitary gland; 75%; pituitary parenchyma; 1) pituitary adenoma (adults) or craniopharyngioma (children); 2) Sheehan syndrome; 3) Empty sella syndrome
Pituitary adenomas (adults) and craniopharyngiomas (children) can cause hypopituitarism due to __ or __.
mass effect; pituitary apoplexy (bleeding into an adenoma)
Sheehan syndrome is __-related __ of the __. Gland doubles in size during __, but __ does not increase significantly. Blood loss during __ precipitates __. Presents with these 3 symptoms
pregnancy; infarction; pituitary gland; pregnancy; blood supply; parturition; infarction; 1) poor lactation; 2) loss of pubic hair; 3) fatigue
Empty sella syndrome is a __ defect of the __. __ of the arachnoid and CSF into the sella __ and __ the pituitary gland. Pituitary gland is __ (empty sella) on imaging.
congenital; sella; herniation; compresses and destroys; absent
__ and __ are made in the hypothalamus and then transported via __ to the posterior pituitary for release. Name 3 disorders.
ADH; oxytocin; axons; 1) central diabetes insipidus; 2) nephrogenic insipidus; 3) SIADH
ADH acts on the __ and __ of the __ to promote free water excretion/retention. Oxytocin mediates __ during labor and __ in __.
distal tubules; collecting ducts; kidney; retention; uterine contraction; release of breast milk (let-down); lactating mothers
Central diabetes insipidus is a __ deficiency. Due to __ or __ pathology (name general 4 causes). __ test fails to increase urine osmolality (useful for diagnosis). Treatment is ___.
ADH; hypothalamic; posterior pituitary; tumor, trauma, infection or inflammation; water deprivation test; desmopressin (ADH analog)
Clinical features of both central and nephrogenic diabetes insipidus are based on ___. Name 3.
loss of free water; 1) polyuria and polydipsia w/life threatening dehydration; 2) hypernatremia and high serum osmolality; 3) low urine osmolality and specific gravity
Nephrogenic diabetes insipidus is impaired __ to __. Due to __ or __ (eg __ and __). Clinical features are similar to central diabetes insipidus but there is/is no response to __.
renal response; ADH; inherited mutations; drugs (lithium and demeclocycline); is no; desmopressin
Syndrome of inappropriate ADH secretion is excessive/insufficient ADH secretion. Most often due to __ (e.g. __). Name 3 other causes.
excessive; ectopic production; small cell carcinoma of the lung; 1) CNS trauma; 2) pulmonary infxn; 3) drugs (e.g. cyclophosphamide)
Clinical features of SIADH are based on __ of free water. Name 2. Treatment of SIADH is __ or __.
retention; 1) hyponatremia and low serum osmolality; 2) mental status changes and seizures (hyponatremia leads to neuronal swelling and cerebral edema); free water restriction; demeclocycline
Thyroglossal duct cyst is a cystic __ of __. The thyroid develops at the __ and then travels along the __ to the __. The thyroglossal duct normally __. A persistent duct, however, may undergo __. Presents as a ___.
dilation; thyroglossal duct remnant; base of tongue; thryoglossal duct; anterior neck; involutes; cystic dilation; anterior neck mass
Lingual thyroid is the __ of thyroid tissue at the __. Presents as a __.
persistence; base of the tongue; base of tongue mass
Hyperthyroidism is increased levels of circulating __. It increases basal metabolic rate (due to increased ___), and it increases sympathetic nervous system activity (due to increased __).
thyroid hormone; synthesis of Na/K ATPase; expression of B1 adrenergic receptors
Name at least 5 of the 12 clinical features of hyperthyroidism
1) WL despite inc appetite; 2) heat intolerance/sweating; 3) tacky w/inc CO; 4) arrythmia (afib) esp in elderly; 5) tremor, anxiety, insomnia, heightened emotions; 6) staring gaze with lid lag; 7) diarrhea w/malabsorption; 8) oligomenorrhea; 9) bone resorption w/hypercalcemia (risk for osteoporosis); 10) dec muscle mass w/weakness; 11) hypocholesterolemia; 12) hyperglycemia (due to gluconeogenesis and glycogenolysis)
Hyperthyroidism leads to hyper/hypocholesterolemia, hyper/hypoglycemia, diarrhea/constipation, and heat/cold intolerance
hypocholesterolemia; hyperglycemia (due to gluconeogenesis and glycogenolysis); diarrhea; heat intolerance
What is the most common cause of hyperthyroidism?
Graves disease
In Graves disease, a __ (__) stimulates __ (type _ hypersensitivity). This leads to increased synthesis and release of __. Classically occurs in women/men of __ age (__ years). Treatment involves these 3.
autoantibody; IgG; TSH receptor; type II; thyroid hormone; women; childbearing age (20-40 yrs); 1) beta blockers (block SNS effects); 2) thiamides (blocks peroxidase, prevents TH synthesis); 3) radiodine ablation
Name 3 clinical features of graves disease?
1) hyperthyroidism; 2) diffuse goiter (constant TSH stimulation leads to thyroid hyperplasia and hypertrophy); 3) exophthalmos and pretibial myxedema (not due to hyperthyroid - due to antibody that stimulates TSH receptor)
Exopthalmos and pretibial myxedema occur in graves disease because __ behind the orbit and overlying the __ express the __. TSH activation results in __ buildup, inflammation, __, and edema leading to exophthalmos and pretibial myxedema.
fibroblasts; shin; TSH receptor; glycosaminoglycan (chondroitin sulfate and hyaluronic acid); fibrosis
In Graves disease, irregular __ with __ colloid and chronic __ are seen on histology. Lab findings include H/N/L total and free T4, H/N/L TSH, hyper/hypocholesterolemia, and increased/decreased serum glucose
follicles; scalloped (pictured); inflammation; high T4; low TSH (free T3 down regulates TRH receptors in the AP to decrease TSH release); hypocholesterolemia; increased
___ is a potentially fatal complication of Graves disease. It occurs due to elevated __ and massive __ excess, usually in response to __ (e.g. surgery or childbirth). Presents with these 3 symptoms. Treatment is __, __, and __.
Thyroid storm; catecholamines; hormone; stress; 1) arrhythmia; 2) hyperthermia; 3) vomiting w/hypovolemic shock; propylthiouracil (PTU), B-blockers, and steroids
Propylthiouracil (PTU) inhibits __-mediated __, __, and __ steps of thyroid hormone synthesis, as well as __ conversation of __ to __.
peroxidase; oxidation, organification, and coupling; peripheral; T4 to T3
Multinodular goiter is a enlarged/shrunken thyroid gland with multiple __. Due to relative __. Usually nontoxic (euthyroid). Rarely, regions become __ leading to T4 release and hyperthyroidism (‘__’ goiter).
enlarged; nodules; TSH-independent; toxic
___ is hypothyroidism in neonates and infants. __ is hypothyroidism in older children and adults.
Cretinism; myxedema
Cretinism is hyper/hypothyroidism in __ and __. Characterized by these 5. Thyroid hormone is required for normal __ and __ development
hypothyroidism; neonates; infants; 1) mental retardation; 2) short stature w/skeletal abnormalities; 3) coarse facial features; 4) enlarged tongue; 5) umbilical hernia; brain and skeletal
Causes of cretinism include these 4.
1) maternal hypothyroidism during early pregnancy; 2) thyroid agenesis; 3) dyshormonogenetic goiter; 4) iodine deficiency
Dyshormonogenetic goiter is due to a __ defect in __ production. Most commonly involves __ enzyme.
congenital; thyroid hormone; thyroid peroxidase
Myxedema is hyper/hypothyroidism in __ or __. Clinical features are based on decreased __ and decreased __. Name 5 of 9 clinical features.
hypothyroid; older children; adults; basal metabolic rate; sympathetic nervous system activity; 1) myxedema; 2) weight gain despite normal appetite; 3) slowing of mental activity; 4) muscle weakness; 5) cold intolerance w/decreased sweating; 6) bradycardia w/dec CO, leading to SOB and fatigue; 7) oligomenorrhea; 8) hypercholesterolemia; 9) constipation
The most common causes of myxedema are __ and __. Name 3 others.
iodine deficiency; Hashimoto thyroiditis; 1) drugs (lithium); 2) surgical removal; 3) radio ablation of the thyroid
Hypothyroid causes weight gain/loss, heat/cold intolerance, tachy/bradycardia, hyper/hypocholesterolemia, diarrhea/constipation. Does hyper or hypothyroid cause oligomenorrhea?
weight gain (despite normal appetite); cold intolerance; bradycardia w/dec CO; hypercholesterolemia; constipation BOTH!
Hashimoto thyroiditis is __ destruction of the thyroid gland. Associated with HLA-__. Initially may present as __ (due to __ damage). Progresses to __. H/N/L T4 and H/N/L TSH. __ and __ antibodies are often present (sign of thyroid damage).
autoimmune; DR5; hyperthyroidism; follicle (TH leaks out); hypothyroidism; low T4; high TSH; antithyroglobulin and antithyroid peroxidase
Hashimoto thyroiditis presents as chronic inflammation with __ and __ cells (__ metaplasia of cells that line follicles) is seen on histology. Increased risk for __. Presents as a enlarging/shrunken thyroid gland late in disease course.
germinal centers; Hurtle cells; eosinophilic; B-cell (marginal zone) lymphoma; enlarging
Subacute granulomatous (___) thyroiditis is granulomatous thyroiditis that follows a ___. Presents as a ___ with transient hyper/hypothyroidism. Self-limited. Rarely may progress to hyper/hypothyroidism.
De quervain; viral infxn; tender thyroid (tender is unique); hyperthyroidism; hypothyroidism
Riedel fibrosing thyroiditis is chronic __ with extensive __ of the thyroid gland. Presents as hyper/hypothyroidism with a ‘___’, tender/nontender thyroid gland. __ may extended to involve local structures (e.g. __). Clinically mimics ___, but pts are younger and malignant cells are absent.
inflammation; fibrosis; hypothyroidism; hard as wood; nontender; Fibrosis; airway; anaplastic carcinoma
Thyroid neoplasia usually presents as __, __ nodule. Thyroid nodules are more likely to be benign/malignant. __ uptake studies are useful to further characterize nodules. Increased uptake (hot nodule) is seen in __ or __. Decreased uptake (cold nodule) is seen in __ and __.
distinct, solitary; benign; 131I radioactive; Graves disease; nodular goiter; adenoma; carcinoma
Biopsy of thyroid neoplasia is performed by __. Name the 5 types of thyroid neoplasia. What is the most common type of thyroid carcinoma? Which type is associated with psammoma bodies?
fine needle aspiration (FNA); 1) follicular adenoma; 2) papillary carcinoma (most common type of thyroid carcinoma); 3) follicular carcinoma; 4) medullary carcinoma; 5) anaplastic carcinoma; papillary carcinoma
Follicular adenoma is a benign/malignant proliferation of __ surrounded by __. Usually __, less commonly, may secrete __.
benign; follicles; fibrous capsule; non-functional; thyroid hormone
Papillary carcinoma is the most common type of thyroid carcinoma (__%) of cases. ___ in childhood is a major risk factor. Comprised of __ lined by cells with __, “___” nuclei and __. Papillae are often associated with __. Often spreads to __. Prognosis is excellent/poor.
80; exposure to ionizing radiation; papillae; clear; Orphan Annie eye; nuclear grooves; psammoma bodies (pictured); cervical (neck) LNs; excellent (10-yr survival > 95%)
Follicular carcinoma is benign/malignant proliferation of __ surrounded by __ with __. __ helps distinguish follicular carcinoma from follicular adenoma. Entire __ must be examined microscopically (FNA only examines cells and not the __, hence a distinction btwn the 2 cannot be made by FNA).
malignant; follicles; fibrous capsule; invasion through the capsule; invasion through the capsule; capsule; capsule;
In follicular carcinoma metastases generally occurs hematogenously/lymphatically. Name 4 carcinomas that break the rule and spread hematogenously.
hematogenously; 1) follicular carcinoma of the thyroid; 2) RCC; 3) hepatocellular carcinoma; 4) choriocarcinoma
Medullary carcinoma is a benign/malignant proliferation of ___. Comprises __% of thyroid carcinomas. __ cells are neuroendocrine cells that secrete __, which lowers serum calcium by increasing renal calcium excretion but is inactive at normal physiologic levels.
malignant; parafollicular C cells; 5; C cells; calcitonin;
High levels of calcitonin produced by tumor (__ carcinoma) may lead to __. Calcitonin often deposits within the tumor as __. Biopsy reveals sheets of __ in a __.
medullary; hypocalcemia; amyloid; malignant cells; amyloid stroma
Familial cases of medullary carcinoma are often due to __ and __, which are associated with mutations in the __ oncogene. Both result in __ and __, while 2A also results in __, and 2B may also result in ___. Detection of the __ mutation warrants ___.
multiple endocrine neoplasia (MEN) 2A and 2B; RET; medullary carcinoma and pheochromocytoma; parathyroid adenoma - 2A; ganglioneuromas of the oral mucosa (2B)
Anaplastic carcinoma is a differentiated/undifferentiated benign/malignant tumor of the thyroid. Usually seen in __. Often invades __, leading to __ or __. Good/poor prognosis. __ clinically mimics it, but those pts are younger and malignant cells are absent
undifferentiated; malignant; elderly; local structures; dysphagia; respiratory compromise; poor; Riedel fibrosing thyroiditis
In the parathyroid glands, __ cells regulate serum free (ionized) ___ via parathyroid hormone secretion. It has these 3 effects (in 3 different locations). Increased serum ionized calcium levels provide __ feedback to __ PTH secretion.
chief; calcium; 1) increases bone osteoclast activity releasing calcium and phosphate; 2) increases small bowel absorption of calcium and phosphate (indirectly by activating vitamin D); 3) increases renal calcium reabsorption (distal tubule) and decreases phosphate reabsorption (proximal tubule); negative; decrease
Primary hyperparathyroidism is due to a disorder of the __. Most common cause is __ (gt 80%). Name 2 other less common causes.
parathyroid gland; parathyroid adenoma; sporadic parathyroid hyperplasia; parathyroid carcinoma
Parathyroid adenoma is a benign/malignant neoplasm, usually involving 1/2/3/4 glands. Most often results in __. However, may present w/consequences of increased __ and __, such as these 5
benign; 1; asymptomatic hypercalcemia; PTH; hypercalcemia; 1) nephrolithiasis (calcium oxalate stones); 2) nephrocalcinosis (metastatic calcification of renal tubules); 3) CNS disturbances (depression and seizures); 4) constipation, peptic ulcer disease, acute pancreatitis; 5) osteitis fibrosa cystica (pictured)
Nephrocalcinosis (pictured) from primary hyperparathyroidism is caused by dystrophic/metastatic calcification of __, potentially leading to __ and __. Osteitis fibrosa cystica is __ of bone leading to __ and __ spaces.
metastatic; renal tubules; renal insufficiency; polyuria; resorption; fibrosis; cystic
Lab findings from a parathyroid adenoma include H/N/L PTH, H/N/L serum calcium, H/N/L serum phosphate, H/N/L urinary cAMP, H/N/L serum alkaline phosphatase. Treatment involves __.
increased PTH; increased Ca; low phosphate; high urinary cAMP; high alk phos (alk phos needed to lay down bone - high b/c PTH works by activating osteoblasts, (which then activate osteoclasts)); surgical removal of affected gland
Secondary hyperparathyroidism is __ production of __ due to a disease process intrinsic/extrinsic to the PT gland. Most common cause is __.
excess; PTH; extrinsic; chronic renal failure
Renal insufficiency leads to inc/dec phosphate excretion. Inc/dec serum phosphate binds __, which decreases it, stimulating __. Increased PTH leads to __ (contributing to renal osteodystrophy). Lab findings include H/N/L PTH, serum Ca, serum phosphate, alk phos.
decreased; increased; free calcium; parathyroid glands; bone resorption; high PTH; low serum Ca; high serum phosphate; high alk phos
Hypoparathyroidism is low __. Name 3 causes.
PTH; 1) autoimmune damage to the parathyroids; 2) surgical excision; 3) DiGeorge’s syndrome (failure to develop 3rd and 4th pharyngeal pouches)
Hypoparathyroidism presents with symptoms related to low __. Name 2 clinical signs/symptoms. Labs reveal H/N/L PTH level and H/N/L serum calcium.
serum calcium; 1) numbness and tingling (particularly circumoral); 2) muscle spasms (tetany); low PTH; low calcium
Low serum calcium can cause muscle spasms (__), which may be elicited with filling of a blood pressure cuff (__ sign) or tapping on the facial nerve (__ sign).
tetany; Trousseau sign; Chvostek sign
Pseudohypoparathyroidism is due to __. Labs reveal H/N/L calcium with H/N/L PTH. Autosomal dominant form is associated with short __ and short __ and __.
end-organ resistance to PTH; low calcium; high PTH; stature; 4th and 5th digits (autosomal dominant form is associated with Gs-GPCR that is non-responsive)
Endocrine pancreas is composed of clusters of cells termed ___. A single islet consists of multiple cell types, each producing one type of hormone. Insulin is secreted by __ cells, which lie in the center/periphery of the islets. Glucagon is secreted by __ cells.
islets of Lagerhans; beta; center; alpha
Insulin is a major anabolic/catabolic hormone. It up regulates insulin-dependent glucose transporter protein (__) on __ and __. Glucose uptake by __ decreases serum glucose. Increased glucose uptake by tissues leads to increased __, __ and __.
anabolic; GLUT4; skeletal muscle; adipose tissue; GLUT4; glycogen synthesis; protein synthesis; lipogenesis
Glucagon is secreted by __ cells. It opposes __ in order to increase/decrease blood glucose levels (e.g. in states of fasting) via __ and __.
alpha; insulin; increase; glycogenolysis; lipolysis
Type I diabetes mellitus is a __ deficiency leading to a metabolic disorder characterized by __. Due to __ destruction of __ cells by __ (type __ hypersensitivity).
insulin; hyperglycemia; autoimmune; beta; T lymphocytes; IV
Type I DM is characterized by inflammation of __. Associated with HLA-__ and HLA-__. Autoantibodies against __ are often present (sign of damage) and may be seen years before clinical disease develops.
islets; DR3; DR4; insulin
Type 1 DM manifests in __ with clinical features of __ deficiency. Name 3. What is the treatment? Patients are at risk for __.
childhood; insulin; 1) high serum glucose; 2) weight loss, low muscle mass, and polyphagia (inc hunger); 3) polyuria, polydipsia, and glycosuria; lifelong insulin; diabetic ketoacidosis
High serum glucose in T1DM is due to a lack of __ leading to decreased __ by fat and skeletal muscle. WL, low muscle mass, and polyphagia occur because unopposed __ leads to __, __, and __, which further exacerbate hyperglycemia.
insulin; glucose uptake (GLUT4); glucagon; gluconeogenesis, glycogenolysis, lipolysis
Polyuria, polydipsia, and glycosuria occur in T1DM because hyperglycemia exceeds renal ability to __. Excess filtered glucose leads to __.
resorb glucose; osmotic diuresis
Diabetic ketoacidosis is characterized by excessive serum __. Often arises with __ (e.g. infxn); __ stimulates glucagon secretion increasing __ (also inc these 2), which leads to increased __. Liver converts __ to ketone bodies (name 2).
ketones; stress; epinephrine; lipolysis (gluconeogensis and glycogenolysis); free fatty acids (FFAs); FFAs; beta-hydroxybutyric acid and acetoacetic acid
DKA results in hyper/hypoglycemia, __, and hyper/hypokalemia. It presents with these 6 symptoms.
hyperglycemia (unopposed glucagon! gt 300); anion gap metabolic acidosis; hyperkalemia (K/H reversal from acidosis, insulin helps drive K in to cells); 1) Kussmaul respiration; 2) dehydration; 3) nausea; 4) vom; 5) mental status changes; 6) fruity smelling breath (due to acetone)
Treatment of DKA is __ (corrects __ from polyuria), __, and replacement of __.
fluid; dehydration; insulin (counter effects of glucagon and force K in to cells); electrolytes (potassium - lose a lot in urine and insulin forces it in to cells, so there will be low K)
Type 2 DM is end-organ __ leading to a metabolic disorder characterized by __. Affects __% of US population, with a rising incidence. Arises in __-aged, __ adults. __ leads to decreased numbers of __. There is a strong/weak genetic predisposition.
insulin resistance; hyperglycemia; 5-10%; middle-aged; obese; obesity; insulin receptors; strong (stronger than it is for type I)
Clinical features of T2DM include __, __, and __, but disease is often __. Diagnosis is made by measuring __ (normal is __). To get a diagnosis you need one of these 3.
polyuria; polydipsia; hyperglycemia; clinically silent; glucose levels; 70-120; 1) random glucose gt 200; 2) fasting glucose gt 126; 3) glucose tolerance test w/a serum glucose level gt 200 2 hrs after glucose loading
In T2DM there is risk for developing __ coma. High glucose (gt __) leads to life-threatening __ with __ and __. __ are absent due to small amounts of circulating insulin.
hyperosmolar non-ketotic coma; 500; diuresis; hypotension; coma; ketones
NEG of hemoglobin produces __, a marker of glycemic control.
glycated hemoglobin (HbA1c)
Long term diabetes can cause osmotic damage. __ freely enters into __ cells (which myelinated peripheral nerves), __ of retinal blood vessels, and the __. __ enzyme converts glucose to __, resulting in osmotic damage. Leads to these 4 complications.
glucose; Schwann cells (doesn’t need insulin to uptake glucose); pericytes; lens; Aldose reductase; sorbitol; 1) peripheral neuropathy; 2) impotence; 3) blindness (leading cause of blindness in developed world); 4) cataracts
Pancreatic endocrine neoplasms are tumors of __. Account for lt __% of pancreatic neoplasms. Often a component of __, along with __ and __. Name 4.
islet cells; 5%; MEN1; parathyroid hyperplasia; pituitary adenomas; 1) insulinomas; 2) gastrinomas; 3) somatostatinomas; 4) VIPomas
Insulinomas present as episodic __ with __ changes that are relieved by administration of __. Diagnosed by H/N/L serum glucose, H/N/L insulin, and H/N/L C-peptide
hypoglycemia; mental status changes; glucose; low glucose (less than 50); high insulin; high C-peptide (indicated endogenous insulin production)
Gastrin goes to __ cells of stomach and causes them to produce __. Somatostatin inhibits __ and __ production. Cholecystokinin is needed for __ of the __. Vasoactive intestinal peptide (VIP) inhibits __ secretion.
parietal; acid (by H+ pumps); gastrin and cholecystokinin (CCK); contraction of the gall bladder; gastric acid
Gastrinomas present as __-resistant __ (__ syndrome). __ may be multiple and can extend in to the jejunum. Somatostatinomas present as __ (due to inhibition of __) and __ with __ ( due to inhibition of __).
treatment; peptic ulcers; Zollinger-Ellision; ulcers achlorhydria (low acid production); gastrin; cholelithiasis with steatorrhea; cholecystokinin (CCK) (can’t digest fat without bile)
VIPomas secrete excessive __ leading to these 3 symptoms.
vasoactive intestinal peptide; 1) watery diarrhea; 2) hypokalemia; 3) achlorhydria
Adrenal cortex is composed of 3 layers. Name them and the class of hormone they produce.
1) glomerulosa produces mineralcorticoids (aldosterone); 2) fasciculata produces glucocorticoids (cortisol); 3) reticularis produces androgens (DHEA) - it get sweeter as you get deeper
Adrenal cortical hormones are derived from __. Sequential enzyme modifications produces __, __, and __. Each layer secretes distinct hormones based on __ present in that layer.
cholesterol; mineralcorticoids, glucocorticoids, and androgens; enzymes
Cushing syndrome is excess __. Name 6 clinical features.
cortisol; 1) muscle weakness w/thin extremities; 2) moon facies, buffalo hump, and truncal obesity; 3) abdominal striae; 4) HTN often w/hypokalemia and metabolic alkalosis; 5) osteoporosis; 6) immune suppression
In cushing’s, high __ (due to high glucose) increases storage of __ centrally/peripherally, resulting in these 3 clinical features
insulin; fat; centrally; 1) moon facies; 2) buffalo hump; 3) truncal obesity
High cortisol increases sensitivity of peripheral vessels to __. At very high levels, cortisol cross-reacts with __ receptors. These two things can cause __ often with __ and __ in Cushing patients.
catecholamines; mineralcorticoid (aldosterone is not increased); hypertension; hypokalemia; metabolic alkalosis
Diagnosis of Cushing syndrome is based on ___ (increased), ___ (increased) and ___test.
24-hour urine cortisol level; late night salivary cortisol level; low-dose dexamethasone suppression
Name 4 causes of Cushing syndrome. Which one is Cushing disease? What do the adrenal glands look like on imaging?
1) exogenous glucocorticoids (bilateral atrophy); 2) ACTH-secreting pituitary adenoma (aka Cushing disease - bilateral hyperplasia); 3) ectopic ACTH secretion (paraneoplastic) (bilateral hyperplasia); 4) primary adrenal adenoma, hyperplasia or carcinoma (contralateral atrophy or bilateral nodular hyperplasia)
High dose dexamethasone suppresses __ production by a __ (serum cortisol is lowered) but does not suppress __ production (serum cortisol remains high).
ACTH; pituitary adenoma; ectopic ACTH (like from small cell carcinoma or carcinoid) (this is how you differentiate the ACTH-depedent high cortisol causes)
Which cause of Cushing syndrome may have high androgens present? which may cause hyper pigmentation? Which are ACTH-independent? ACTH-dependent? What are two sources of ectopic ACTH production?
ACTH-secreting pituitary adenoma and ectopic ACTH secretion; hyperpig: ectopic ACTH secretion; indep: exogenous glucocorticoids and primary adrenal adenoma; dep: first two listed above; small cell carcinoma or carcinoid
Hyperaldosteronism classically presents as __, __ and __. Aldosterone increases absorption of __ and secretion of __ (in __ cells) and __ (in __ cells) in the distal tubule and collecting duct. Increased __ expands plasma volume leading to __. Edema is often present/absent, however due to ___.
HTN; hypokalemia; metabolic alkalosis; sodium; potassium; principal cells; hydrogen ions; alpha-intercalated cells; sodium; htn; absent; aldosterone escape
Primary hyperaldosteronism is most commonly due to __. __ (__ syndrome) and __ are two less common causes. There is high/low aldosterone and high/low renin. First line tx is __ (e.g. __ or __). __ are surgically resected.
bilateral adrenal hyperplasia; adrenal adenoma (Conn syndrome); adrenal carcinoma (rare); high; low (inc perfusion pressure down regulates renin); mineralcorticoid receptor antagonists (eplerenone and spironolactone); adenomas
Secondary hyperaldosteronism is seen with activation of __ (e.g. __ or __). High/low aldosterone and high/low renin are seen. Renal artery stenosis is commonly caused by __ in elderly males or __ in young women, which then activates the __
renin-angiotensin system; renovascular HTN or CHF); high; high; atherosclerosis; fibromuscular dysplasia; RAA system
Familial hyperaldosteronism can rarely be due to __. Aberrant expression (pattern of inheritance) of ___ in the fasculata. Presents in children/adults as hyper/hypotension, hyper/hypokalemia, high/low aldosterone and high/low renin. Responds to __; confirmed with __.
glucocorticoid-remediable aldosteronism (GRA); autosomal dominant; aldosterone synthase; children; HTN; hypokalemia (+/-); low; dexamethasone; genetic testing
___ syndrome mimics hyperaldosteronism. Decreased degradation of __ (pattern of inheritance) in collecting tubules. Classically presents as hyper/hypotension, hyper/hypokalemia, and metabolic acidosis/alkalosis in young patient.
Liddle; sodium channels; autosomal dominant; HTN, hypokalemia, metabolic alkalosis
Liddle syndrome has high/low aldosterone and high/low renin. Treatment is __ (__or __), which block tubular __. __ is not effective.
low; low; potassium sparing diuretics (amiloride or triamterene); sodium channels; spironolactone (Liddle syndrome mimics hyperaldosteronism w/dec degradation of sodium channels in collecting tubules)
Congenital adrenal hyperplasia is characterized by __ defects (pattern of inheritance) in __ production. There is high/low ACTH (due to inc/dec neg feedback) leading to __. __ and __ may be inc or dec depending on defect.
enzymatic defects; autosomal recessive; cortisol; high; decreased; bilateral adrenal hyperplasia; mineralcorticoids and androgens
Cholesterol –__– pregenelone; what enzymes are needed to make aldosterone? what enzymes are needed to make cortisol? what enzymes are needed to make androstenodione and DHEA? Pregenelone and progesterone are converted to __ and __ by __.
desmolase; 21-OHase, 11-OHase, aldo synthase; 17-OHase, 21-OHase, and 11-OHase; 17-OHase; 17-OH pregnelone and 17-OH progesterone by 17-OHase (precursors in cortisol and DHEA production) (all need 3HSD as well)
What is the most common cause of congenital adrenal hyperplasia?
21-hydroxylase deficiency
11-OHase deficiency is biochemically similar to __ deficiency, but weak __ are increased. Leads to __ (__retention) with mild __. __ and __ are low.
21-hydroxylase; mineralcorticoids; HTN; sodium; hypokalemia; renin and aldosterone
17-hydroxylase deficiency leads to decreased __ and __. Weak __ are increased leading to __ and mild __. __ and __ are low. Decreased __ (adrenal and gonads) lead to primary __ and lack of __ in females or __ in males.
cortisol; androgens; mineralcorticoids; hypertension; hypokalemia; renin and aldosterone; androgens; amenorrhea; pubic hair; pseudohermaphroditism
17-hydroxylase deficiency leads to decreased __ and __. Weak __ are increased leading to __ and mild __. __ and __ are low. Decreased __ (adrenal and gonads) lead to primary __ and lack of __ in females or __ in males.
cortisol; androgens; mineralcorticoids; hypertension; hypokalemia; renin and aldosterone; androgens; amenorrhea; pubic hair; pseudohermaphroditism
Screening for congenital adrenal hyperplasia involves serum __ levels. Those levels are increased in __ deficiency/s and decreased in __ deficiency/s
17-hydroxyprogesterone; 21 and 11-OHase deficiency (since 17-OH progesterone cannot be converted to cortisol and aldosterone); 17-hydroxylase (b/c 17-hydroxylase is what is needed to make 17-OH-prog
Adrenal insufficiency is __ of adrenal hormones. Acute insufficiency most commonly arises secondary to __. Presents as __ and __. __ syndrome is a classic cause of acute adrenal insufficiency.
deficiency; abrupt withdrawal of glucocorticoids; weakness; hypotension (shock); Waterhouse-Friderichsen
Waterhouse-Friedrichsen syndrome is a classic cause of __. Characterized by __ of the __, classically due to __ in young children with __ infection.
acute adrenal insufficiency; hemorrhagic necrosis; adrenal glands; DIC; Neisseria meningitidis
Chronic adrenal insufficiency (__ disease) is due to progressive destruction of __. Name 3 common causes and a fun fact.
Addison; adrenal glands; 1) autoimmune destruction (most common cause in West); 2) TB (MCC in developing world); 3) metastatic carcinoma (lung loves to go to adrenals)
Clinical features of chronic renal insufficiency include hyper/hypotension, hyper/hyponatremia, hyper/hypovolemia, hyper/hypokalemia, metabolic acidosis/alkalosis, and these 4 other symptoms. Which symptoms help distinguish primary from secondary insufficiency?
hypotension (cortisol dec), hyponatremia, hypovolemia, hyperkalemia (aldosterone dec), metabolic acidosis; 1) weakness; 2) hyperpigmentation (very high ACTH stimulates melanocytes due to MSH also being a derivative of POMC); 3/4) vom/diarrhea; hyper pigmentation (high ACTH - means primary problem) and hyperkalemia (low aldosterone mean prim prob)
Adrenal medulla is composed of __-derived __ cells. Main physiologic source of __ (__ and __). What is the name of a tumor of the chromaffin cells?
neural crest; chromaffin; catecholamines; epi and norepi; pheochromocytoma
Pheochromocytoma is a tumor of __ cells. Clinical features are due to increased __. Name 5. It is diagnosed by increased serum __ and increased 24-hr urine __ and __.
chromatin cells; serum catecholamines; 1) episodic HTN; 2) headache; 3) palpitations; 4) tachycardia; 5) sweating; metanephrines; metanephrines and vanillylmandelic acid (epi and norepi are broken down in to metanephrines and further into VMA)
Treatment of pheochromoctyoma is __. Catecholamines may leak into bloodstream upon manipulation of the tumor. __ (irreversible _-blocker) is administered perioperatively to prevent a __.
surgical excision; phenoxybenzamine; alpha; hypertensive crisis
Pheochromocytomas often follow the rule of 10s: 10% are these 4 things. Most common location outside of the medulla is __ or __.
10% bilateral; 10% familial; 10% malignant; 10% located outside of the adrenal medulla; bladder wall; organ of Zuckerkandl at the inferior mesenteric artery root
Pheochromoctyomas are associated with __, __ disease, and __.
MEN2A and 2B (as is medullary carcinoma of thyroid); von Hippel-Landau disease (as is hemangioblastoma of cerebellum, RCC); neurofibromatosis type 1
In long term DM II, NEG of small vessels (arterioles) leads to __. Involvement of __ arterioles leads to __, resulting in small, __ kidneys with a __ surface.
hyaline arteriolosclerosis; renal; glomerulosclerosis; scarred; granular
