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467 Cards in this Set

  • Front
  • Back
What are the two basic types of hormonal activity?
Interact with cell surface receptors producing 2nd messengers--cAMP, increased Ca2+

Interact with intracellular receptors
If there is a decrease in the amount of hormone produced, what does this usually mean?
Autoimmune
If there is an increase in the amount of hormone produced, what does this usually mean?
Adenoma
What are the ways the endocrine system can be disturbed?
Impaired Synthesis
Impaired release
Abnormal interaction between hormones and target tissues
Abnormal target organ response
What is the master gland involved in the endocrine system?
The pituitary gland
What are the two portions in the pituitary gland?
The anterior portion and the posterior portion
Where is the anterior pituitary gland derived from?
Rathke's pouch--oral ectoderm
Where is the posterior pituitary gland derived from?
Diencephalon
What portion of the pituitary is the adenohypophysis?
The anterior pituitary
What portion of the pituitary is the neurohypophysis?
The posterior pituitary
Where does the pituitary gland sit?
In the sella turcica
What do the somatotrophs produce?
Growth hormone
What do the lactotrophs produce?
Prolactin
What do the corticotrophs produce?
ACTH, MSH, POMC
What do the thyrotrophs produce?
TSH
What do gonadotrophs produce?
FSH and LH
How does the hypothalamus control the anterior pituitary?
By the portal venous system that links the hypothalamus and the anterior lobe
Why does the posterior pituitary not need a portal venous system?
Because it is directly connected to the brain
What does the hypothalamus contain?
Releasing hormones that act on the anterior pituitary
What happens during pregnancy to the anterior pituitary?
It doubles in size because of the increase need in hormones
What inhibits prolactin?
Dopamine
What is the only anterior pituitary hormone that has an inhibitory effect?
Prolactin
What are the two hormones released from the posterior pituitary?
Oxytocin and Vasopressin (ADH)
What do gonadotrophs produce?
FSH and LH
How does the hypothalamus control the anterior pituitary?
By the portal venous system that links the hypothalamus and the anterior lobe
Why does the posterior pituitary not need a portal venous system?
Because it is directly connected to the brain
What does the hypothalamus contain?
Releasing hormones that act on the anterior pituitary
What happens during pregnancy to the anterior pituitary?
It doubles in size because of the increase need in hormones
What inhibits prolactin?
Dopamine
What is the only anterior pituitary hormone that has an inhibitory effect?
Prolactin
What are the two hormones released from the posterior pituitary?
Oxytocin and Vasopressin (ADH)
Where are oxytocin and ADH made?
In the hypothalamus and released by the posterior pituitary
What is hyperpituitarism?
Excess secretion of trohpic hormones
What things can cause hyperpituitarism?
Adenooma (most common)
Hyperplasia
Carcinoma (VERY RARE!)
What is hypopituitarism?
Deficiency of trophic hormones--usually caused by destructive processes
What do pituitary local mass effects produce?
Visual disturbances and also exert effects on the sella turcica
What are diseases of the posterior pituitary?
Diabetes Insipdis
Inappropriate ADH secretion
Are pituitary adenomas usually benign or malignant?
Usually benign
In what ways do pituitary adenomas cause problems?
By mass effects (visual disturbances) or by production of hormones (prolactin or ACTH)
The larger a tumor is......
The less likely it is to be producing hormones
What is the most common cause of hyperpituitarism?
Adenoma arising in the anterior lobe
What is a functional pituitary adenoma?
Secrete active hormones
What is a silent pituitary adenoma?
Cells are producing hormones but they don't have clinical effects
What is a nonfunctional adenoma?
No evidence of hormonal production; usually gets recognized by mass effects
Who usually gets pituitary adenomas?
4th to 6th decade
What are the most common hormones secreted in pituitary adenomas?
Prolactin
ACTH
Growth Hormone
What percentage of clinically detected pituitary adenomas are responsible for intracranial neoplasms?
10%
What do 25% of routine autopsies find?
Microadenomas (<1cm)--rarely have a hormonal output
What type of pituitary adenomas have the GNAS1 mutation?
Somatotroph cell adenomas
What MEN commonly has pitutiary adenomas?
MEN-1 (Wermer Syndrome)
What oncogenes are seen in aggressive tumors?
RAS and c-MYC
What is the most common somatotroph tumor?
GHRH-40%
What is a large pituitary adenoma usually producing?
NOTHING
What type of cells are usually seen in a pituitary adenoma?
Monomorphic cells
What is the most common type of pituitary adenoma?
Prolactinoma (20-30%)
What is the classic triad of symptoms seen in a prolactinoma?
Amenorrhea
Galactorrhea
Infertility
Do proalactinomas usually cause symptoms in men?
Not usually--but can--impotence, ha, visual loss
Who are the prolactinomas the most prevalent in?
Women with adult onset amenorrhea
What level of prolactin is diagnostic of a prolactinoma?
No absolute level--levels >1000 are usually suggestive of a macroadenoma
How is a prolactinoma usually treated?
With Dopamine agonists and surgical removal if necessary
What is the 2nd most common pituitary adenoma?
Growth Hormone (Somatotroph)
What are the two subtypes of growth hormone adenomas?
Densely granulated (acidophilic)
Sparsely granulated (chromophobe)
What is the densely granulated growth hormone adenoma?
Reacts with GH antibody and has cytokeratin; tumors with both growth hormone and prolactin
What is the sparsely granulated growth hornome adenoma?
Weak reaction with GH antibody
What happens if there is persistent secretion of growth hormone?
Stimulates hepatic secretion of IGF-1
If the growth hormone adenoma occurs before puberty, what is the result?
Gigantism--occurs before the epiphyseal plate closes
If the growth hormone adenoma occurs after puberty, what is the result?
Acromegaly--after ephysieal plate closed
What is the most common etiology of acromegaly?
Growth hormone secreting adenoma--95%
What causes acromegaly besides a growth hormone adenoma?
Small cell carcinoma of the lung
What are the major characteristics of acromegaly?
Organomegaly
Spade like hands
Frontal bossing
Big nose
How is acromegaly diagnosed?
Elevated serum GH and IGF-1
How do we test for acromegaly?
Give a glucose load
If the patient does have acromegaly, what will be the result of the glucose load test?
GH will not fall rapidly as it normally does with administration of glucose
What are the classic complaints of patient's who have acromegaly?
"My wedding ring won't go on"....."My hat doesn't fit anymore"
What is another common type of somatotroph adenoma that is nearly as common as growth hormone adenoma?
Corticotroph cell adenoma
What are corticotroph adenomas usually at the time of diagnosis?
Microadenomas
What type of tumors are usually seen in corticotroph adenomas?
Densely granulated
What does the excess ACTH production in a corticotroph adenoma lead to?
Cushing's disease
What is Cushing's disease?
Increase in ACTH and an increase in cortisol
What is Cushing's syndrome?
Just an increase in cortisol
What is Nelson's syndrome?
A large adenoma that develops after an adrenalectomy--loss of negative feedback
What is hypopituitarism?
Reduction in normal hormonal output
How much of the pituitary is required to be lost before symptoms are present?
About 75%
What are some clinical findings that are seen with hypopituitarism?
Hypothyroid
Amenorrhea
Infertility
Pallor
Lactation failure
Hypoadrenalism
What are some causes of hypopituitarism?
Tumors/lesions including Rathke's pouch
Pituitary surgery
Pituitary apoplexy
Ischemic Necrosis
Empty sella syndrome
What is Sheehan syndrome?
Post-partum necrosis of the pituitary--changes in blood volume after delivery
Who is most commonly affected by empty sella syndrome?
Obese multips
What are some presenting signs of Sheehan's syndrome?
Fatigue
Not bonding well
(resembles pp depression)
What is empty sella syndrome?
No tissue seen in the sella turcica
What are the two types of posterior pituitary syndrome?
Diabetes Insipidus and SIADH
What is the ADH level in Diabetes Insipidus?
Low--excess water being excreted
What is the ADH level in SIADH?
High--not enough water is urine
What are the subclasses of Diabetes Insipidus?
Neurogenic (Central)
Nephrogenic
Primary Polydipsia
Gestational diabetes insipidus
What is nerogenic or central diabetes insipidus?
The posterior pituitary fails to secrete ADH due to injury
What is nephrogenic diabetes insipidus?
The kidney fails to respond to ADH
What is primary polydipsia diabetes insipidus?
Abnormal thirst causes patient to consume excessive amounts of water
What is gestational diabetes insipidus?
Increased metabolism of ADH which causes deficiency
What is a good way to tell if the patient has neurogenic (central) or nephrogenic diabetes insipidus?
Give the patient ADH
What is a usual cause of nephrogenic diabetes insipidus?
Drugs
What are the clinical findings in patients with diabetes insipidus?
Polyuria--dilute urine with low specific gravity
Polydipsia
HYPERNATREMIA
In SIADH, do patient's have dilute or concentrated urine?
Concentrated urine--unable to make dilute urine
What are the clinical findings in SIADH?
HYPONATREMIA
Fluid overload
Weakness
Confusion
Irritability and hostility
What is the most common cause of hospital acquired hyponatremia?
SIADH
What are the two causes of SIADH?
Abnormal regulation of post pituitary secretion of ADH--high levels of ADH

Ectopic secretion of ADH by a malignant tumor
What is the most common cause of ectopic secretion of ADH?
Small cell carcinoma
Hyponatremia occurs how often in hospitalized patients over the age of 65?
25% of the time with half of those having evident SIADH
What are the two types of suprasellar tumors?
Gliomas
Craniopharyngioma
What is a craniopharyngioma?
Derived from remnants of Rathke's pouch
Is a craniopharyngioma slow or fast growing?
Slow growing
Where do craniopharyngiomas occur?
In the hypothalamic region above the sella turcica
What are common symptoms seen in craniopharyngiomas?
Diabetes Insipidus and visual disturbances
What is unique about the epidemiology of craniopharyngiomas?
It has a bimodal age distribution---Children 5-15 years and adults 50 and up
What type of tumors do craniopharyngiomas typically form?
Cystic tumors
What do craniopharyngiomas arrise from?
Epithelial neoplasm dervied from Rathke's pouch
What does a non palpable thryoid mean?
Normal size
Where is the most common place for ectopic thyroid tissue?
Lingual
What are C cells and what do they secrete?
C cells are parafollicular cells and they secrete calcitonin
What is the most abundant thyroid hormone produced?
T4
What is the most metabolically active hormone produced?
T3
Which of the thyroid hormones has a much shorter half life?
T3
What is a goiter?
Enlargement of the thyroid gland
What is a diffuse nontoxic goiter?
A goiter that involves the entire thyroid gland and is not functional
What is the most common cause of a diffuse goiter?
Iodine deficiency
What is the thyroid state in patients with a diffuse goiter?
Usually euthyroid
What is a multinodular goiter?
A diffuse goiter that has recurrent episodes of hyperplasia and involution resulting in a mulitnodular goiter
What is thyroiditis?
Inflammation of the thyroid gland
What is the problem with a asymptomatic, nontoxic goiter?
Nothing--purely cosmetic
What does the epithelium look like in multinodular goiters?
"Flattened" epithelium
What is thyrotoxicosis?
Hypermetabolic state caused by high levels of circulating T3 and T4
What is subclinical hyperthyroid?
An asympotmatic patient with a low TSH but normal T3 and T4
What is exophthalmos?
Accumulation of loose connective tissue in the soft tissue of the posterior orbit
What is thryoid storm?
ABRUPT onset of thyrotoxicosis
What are the clinical findings in thyrotoxicosis?
Increased BMR
Tachycardia
Tremor
Weight loss
Intolerance to heat
Nervousness
What are three signs found in 50% of older patients?
Tachycardia
Fatigue
Weight loss
Who normally presents with thyrotoxicosis?
Women
What are the four causes of Sustained thryotoxicosis?
Primary
Secondary
Tertiary
Exogenous
What is primary sustained thyrotoxicosis?
Overactivity of the thyroid gland--the most common
What is secondary sustained thyrotoxicosis?
Excessive TSH
What is tertiary sustained thyrotoxicosis?
Caused by hypothalamic disorders
What is exogenous sustained thyrotoxicosis?
Caused by taking thyroid hormones when you don't really need them
What is the cause of transient or temporary thyrotoxicosis?
Thyroiditis
How do you distinguish between thyrotoxicosis and thyroiditis?
Thyroiditis won't light up on a thryoid scan
What is the main cause of Primary Hyperthyroidism?
Graves Disease--85% (Diffuse Hyperplasia)
Besides Graves Disease, what are other causes of primary hyperthyroidism?
Toxic Multinodular Goiter
Toxic Adenoma/Carcinoma
Drug-Induced Goiter
What is critical to obtain when taking a patient's history who is presenting with hyperthyroidism?
The medication history--Amiodarone, Iodides, and Lithium can all cause hyperthyroidism
Thyroiditis, Struma ovarii, and exogenous hyperthryoidism are all causes of what?
Thyrotoxicosis
What are two examples of Exogenous Hyperthyroidism?
Iatrogenic and Factitious Thyroidism
What is Iatrogenic Hyperthryoidism?
Excessive administration of thyroxine by physicians
What is Factitious Thyrotoxicosis?
Result of patient self administering excessive doses of thryoxine--for weight loss or malingering
What is the typical age of onset and who is mostly affected by hyperthyroidism?
Women between 40-60 years of age
What is the classic triad of symptoms seen in Graves Disease?
Hyperthyroidism
Exophthalmos
Pretibial Myxedema
What is the typical age of onset for Graves Disease?
Ages 20-40
Is there a genetic component associated with Graves Disease?
Yes
What type of disorder is Graves Disease?
Autoimmune disorder
What antibodies are produced in Graves Disease?
TSI--binds to the TSH receptor and triggers release of T3 and T4
What is the vast majority of thyroiditis caused be?
Autoimmune disorder
What is the most common cause of hypothyroidism?
Hashimoto Thyroiditis
What type of thyroiditis is believed to be caused by a viral infection?
Subacute Granulomatous Thyroiditis (DeQuervain)
What do most patients with DeQuervain present with?
An antecedent respiratory infection
What are the symptoms of DeQuervain?
PAINFUL, enlarged thyroid that resolves
Difficutly swallowing
Symptoms of hyperthyroidism
What is the recovery like in DeQuervain?
Almost always complete and rarely progresses to chronic thyroiditis and hypothyroidism
What type of thryoiditis is associated with post partum?
Subacute Lymphocytic Thyroiditis
What type of goiter is associated with subactue lymphocyctic thyroiditis?
Painless goiter
What symptoms are associated with subacute lymphocytic thyroiditis?
Typical thyrotoxicosis symptoms--tremor, palpitations, tachycardia, weakness, and fatigue
Are the symptoms mild or severe in subactue lymphocyctic thyroiditis?
Usually mild and transient--develop over 1-2 weeks and last for 2-8 weeks
What is the difference between subacute lymphocyctic and DeQuervian thyroiditis?
A minority of patients may progress to hypothyroidism in subacute lymphocyctic thyroiditis
What is the rare variant of subacute lymphocyctic thyroiditis?
Reidel Struma
What happens in Reidel Struma?
Complete replacement of the thyroid gland by fibrous tissue
What is subclinical hypothryoidism?
An asymptomatic patient with a high measured sensitive TSH and a normal T3 and T4
What is cretinism?
Hypothyroidism developing in infancy or early childhood--severe mental retardation--short stature, coarse facies, and protruding tongue
What are the two main causes of hypothyroidism?
Autoimmune disorder and postablative surgery
What is the most common cause of hypothyroidism?
Hashimoto's Thyroiditis
Who is normally affected by Hashimoto's?
Usually older women
How does a patient with Hashimoto's present?
Initially with hyperthyroidism but progresses to hypothyroidism
What type of antibodies are produced in Hashimoto's?
Anti-TPO or anti-thyroglobulin
What is a classic cell seen in Hashimoto's?
Hurthle cells--eosinophilic and stain pink
What are the preferred tests for the functional state of the thyroid?
TSH and FT4
If your TSH test is elevated, what does this suggest?
Hypothyroid
If you TSH test is decreased, what does this suggest?
Hyperthyroid
If the FT4 test is high, what does this suggest?
Hyperthyroid
If the FT4 test is low, what does this suggest?
Hypothyroid
What is the preferred test for autoimmune thyroiditis?
Anti-TPO antibody
What is the preferred test for Graves disease?
Thyrotropin receptor anitbodies (TRA)
To confirm a diagnosis of hyperthryoidism, what test would you order?
Anti-TPO and/or Anti-TRA )TSI, TGI)
If the TSH is low but the FT4 is normal, what test would you order next?
Free T3
To confirm hypothyroidism, what test would you order?
Anti-TPO antibody
What is a solitary thyroid nodule?
Palpable discrete mass within an otherwise normal thyroid
What accounts for 90% of solitary nodules?
Non-neoplastic disorders--Nodular hyperplasia, Colloid cysts, and Foci of thryoiditis
What percentage of solitary nodules are cacinoma?
Less than 1%
What are the risk factors for a solitary nodule containing carcinoma?
Solitary nodule vs. mulitple
Nodules in younger patients
Nodules in males
History of radiation tx to neck
What type of nodules are more likely to contain carcinoma?
Non-functioning or "cold" nodules
What type of nodules are almost always benign?
Functioning or "warm" nodules
What does a radionuclide thyroid scan tell us?
The functionality of the thyroid
What other tests can be done to evaluate solitary nodules?
Thryoid function tests
U/S
Fine Needle Aspiration Biopsy
Does a thryoid that is hypoactive or hyperactive take up a lot of iodine?
Hyperactive thyorid takes up a lot of iodine
Does a thyroid that is hypoactive or hyperactive take up a only a small amount of iodine?
Hypoactive thyroid takes up a small amount of iodine
What is a normal amount of iodine uptake seen in a euthryoid patient?
6-7%
Is a "cold" nodule non-functioning or functioning?
Non-functioning--meaning it is not secreting hormone
Is a "warm" nodule non-functioning or functioning?
Functioning--releasing hormone--takes up dye because it doesn't suppress the entire gland
What does a "hot" nodule do to the thyroid gland?
Turns the gland off so you won't see any iodine uptake
Is a multinodular goiter a good or a bad sign?
Usually a good sign
What test remarkably reduces the amount of thryoid nodules that need to be removed?
Fine Needle Aspiration Biospy
What does the term adenoma refer to?
A single thryoid nodule or mass--can also be called uninodular
Are the vast majority of adenomas functional or non-functional?
Non-functional--designated non-toxic
What do follicular adenomas look like?
Well-circumscribed, solid, gray-white to red-brown
Do follicular adenomas have a capsule?
Yes
What does the term "toxic" mean in reference to a follicular adenoma?
Inidicates that the adenoma is producing thyroid hormone independent of TSH
What is papillary carcinoma of the thyroid?
Malignant neoplasm with numerous, irregular, finger like projections of fibrous stroma that is covered with surface layer of neoplastic epithelia cells
What is the most common type of thyroid carcinoma?
Papillary carcinoma
Are papillary carcinomas small or large and are they usually unifocal or multifocal?
They vary in size and are most often multifocal
What size are the neoplasms that are designated as carcinomas?
1-3 cm
What are the neoplasms that are <1.0 cm in size termed?
Microcarcinomas--rarely assoicated with metastisis
!/3 of patients with papillary carcinoma of the thyroid present with what?
Clinically evident enlarged lymph nodes
Where is the primary diesease of papillary carcinoma confined to in 90% of the patients?
Confined to the neck
What is the 5-year and 10-year survival rate of papillary carcinoma of the thyroid?
5 year-105%
10 year-95%
What type of cell is typically seen in papillary carcinoma of the thyroid?
Orphan Annie cells
What should all papillary neoplasms of the thryoid be considered as?
Malignant--papillary adenomas do not exist
Besides Orphan Annie Cells, what is another characteristic thing seen in Papillary carcinoma?
Psammoma bodies--areas of calcification
If you collect a cervical sample from a patient and there are lymph nodes present, what must you rule out?
Papillary carcinoma of the thryoid
Does papillary carcinoma have a variant form assoiciated with it?
Yes--Follicular variant of papillary carcinoma
What is the median age of diagnosis of Follicular Carcinoma of the Thyroid?
6th decade of life
What is a risk factor assoiciated with follicular carcinoma?
Radiation exposure and also increased frequency with iodine deficiency
Does follicular carcinoma usually present with lymph node involvement?
No--occurs in less than 10% at presentation
How does follicular carcinoma tend to spread?
Hematogenously
What percentage of patients with follicular carcinoma present with distant metastisis?
15% overall
What percentage of patients with follicular carcinoma develop metastisis and where?
5-40% develop metastisis to the lung and bone
What is the 10-year survival rate of patients with follicular carcinoma?
90%
Why has the prevalence of follicular carcinoma changed over the years?
Decreasing incidence
Increased recognition of the follicular variant of papillary carcinoma
15% of normal thryoid glands have thryoid follicles in the thyroid capsule (less diagnosis of follicular carc.)
What two settings does medullary carcinoma of the thyroid occur in?
Familial and sporadic
What are the familial medullary carcinoma (MEN) characteristics?
Typically bilateral
Associated with other benign or malignant endocrine tumors
More common in children
Much earlier age of onset
What are the sporadic form of medullary carcinoma charactersitics?
Unilateral
Presents age 40 to 60
How is the medullary carcinoma tumor idenitifed histologically?
By the presence of amyloid
What do all medullary tumors produce?
Calcitonin
What is the 10-year survival rate associated with medullary carcinoma?
65%--worse prognosis than papillary or follicular cancer
What are the characteristics of anaplastic carcinoma of the thyroid?
Rapidly progressive, lethal rare cancer that occurs in older individuals (mean age of onset is 65)
What is the mean survial for a diagnosis of anaplastic carcinoma of the thryoid?
6 months
What do patients diagnosed with anaplastic carcinoma frequently have a history of?
Long standing goiter
Prior papillary thryoid carcinoma
Concurrent papillary carcinoma
Out of the 10 cases of thryoid cancer/100,000, how many of them are anaplastic carcinoma?
<10%
How does anaplastic carcinoma appear histologically?
Wildly undifferentiated
What is the best way to tell if the parathyroid glands have increased in size?
Weight
What does the relative size neoplasia clue tell us?
4 Big glands--Hyperplasia
1 Big gland + 3 Average--Adenoma
How is the parathyroid gland regulated?
By the free calcium level
What are the metabolic effects of the parathyroid?
Stimulates osteoclasts
Increases calcium resorption in the GI and kidney
Converts Vit D to an active form in the kidney
Increases phophate excretion
What is the most common cause of hypercalcemia?
Primary hyperparathyroidism
What is the most common cause of symptomatic hypercalcemia?
Malignancy
What is primary hyperparathyroidism known as?
The disease of bones, stones, groans, and moans
What are the causes of hyperparathyroidism?
Parathyroid adenoma (80%)
Parathyroid Hyperplasia
Parathyroid Carcinoma
What is the most common symptom of hyperparathyroidism?
Kidney stones
What is osteitis fibrosa cystica?
"Brown tumor"; Osteoclast burrowing themselves in bone--seen in hyperparathryoidism
Where is a common location for brown tumors to occur?
The jaw
What is osteitis fibrosis cytica sometimes referred to?
von Recklinghausen disease of bone
What is the age associated with the greatest incidence of hyperparathyroidism?
age 40-65
What are some diseases associated with hyperparathyroidism?
Diabetes mellitus type 2
Thyroid Disease
What are parathyroid adenomas?
Solid neoplams that are almost always solitary nodules
Where do parathyroid adenomas typically occur?
Superior parathryoid glands
What is the normal weight of a prathyroid gland?
30 mg
What is the weight of a parathyroid adenoma?
Anywhere from 300-3000 mg
How do pituitary adenomas typically appear?
Reddish-brown appearance and measure from 1-4 cm in dimension
What are the most common cell type seen in PTH adenoma?
Cheif cells
What is the 2nd most common form of primary hyperparathyroidism?
Parathyroid hyperplasia
How is the diagnosis of parathyroid carcinoma made?
By properties seen in the gland--Necrosis, hemorrhage, and attachment
How do the cells in parathyroid carcinoma typically appear?
Uniform
What is secondary hyperparathyroidism caused by?
Renal failure
Is the calcium low or high in secondary hyperparathyroidism?
Low--hypocalcemia
What does the low calcium level in secondary hyperparathyroidism stimulate?
Secretion of PTH--causing hyperplasia of the parathyroid glands
What are the symptoms of hyperparathyroidism?
Fatigue, muscle weakness, nausea, bone pain, and fractures
What do patients with a CCr of less than 40 have histological evidence of?
Hyperparathyroid bone disease
What is the most common cause of hypoparathyroidism?
Surgical removal
What is the most important thing to do following thyroid surgery?
Must monitor the calcium levels after surgery
What are other causes of hypoparathyroidism?
Familial
Idiopathic
Hypomagnesemia
Replacement of destruction of normal PTH tissue
In who are the adrenal glands abnormally large compared to the rest of the body?
Fetal/Newborns
What is Cushing syndrome?
High cortisol levels
What are most causes of Cushing's syndrome the result of?
Administration of exogenous glucocorticoids
What is the most common cause of endogenous Cushing syndrome?
Primary hypothalamic-pituitary disease associated with hypersecretion of ACTH
What is it called when there is high levels of cortisol and ACTH?
Cushing disease
What is the cause of primary aldosteronism/hyperaldosteronism?
Excess production of aldosterone
What are the symptoms of hyperaldosteronism?
Hypertension
Hypokalemia
Decreased renin activity
What is the most common cause of hyperaldosteronism?
Adenoma--Conn syndrome
What is primary acute adrenocortical insufficiency?
Acute failure of the adrenal cortex
What can cause acute adrenocorticol insuffiency?
Stress
Too rapid withdrawal from exogenous corticosteroids
Massive Adrenal Hemorrhage
What is Waterhouse-Friderichsen Syndrome?
Massive adrenal hemorrhage
What are the causes of Waterhouse-Friderischsen Syndrome?
Bacterial infection
Septicemia--usually Nisseria meningiditis
Shock
DIC
What are the causes of Primary Chronic Adrenocorticol Insufficiency--Addison's Disease?
Autoimmue adrenalitis
AIDS
Tuberculosis
Metastatic cancer
What are the symptoms of Addison's Disease?
Fatigue/Weakness
GI symptoms
Hyperpigmentation
Hypotension
Hyperkalemia
Hypoglycemia
Which is more common, to get a primary tumor of the adrenal gland or to get metastatic cancer of the adrenal gland?
Metastatic cancer of the adrenal gland
What causes secondary chronic adrenocorticol insuffiency?
Any disorder of the hypothalamus or pituitary which reduces the output of ACTH
What symptom is different in secondary chronic adrenocorticol insuffiency from primary?
Hypopigmentation
What is the "PEARL" of secondary adrenocortical insuffiency?
Hypopigmentation
What is the one constant finding in secondary insufficiency?
Moderate to marked reduction in adrenal size
What is an adrenocortical adenoma?
Benign neoplasm of adrenal cortical cells
What is the most common adrenocortical adenoma?
Aldosterone producing adenomas
Who is most likely to have adrenocortical adenoma?
Females in their 3rd to 5th decade
Are adrenocortical carcinomas common or rare?
Rare--between 0.05% and 0.2% of all cancers
What can adrenocortical carcinomas be confused with?
Renal tumors--ususally large when discovered
Who does adrenocortical carcinoma affect and what is the prognosis?
Affects any age----Poor prognosis (mean survival 2 years)--may be functional (secreting hormones)
What is the most common type of cancer causing metastisis to the adrenal glands?
Lung cancer
What is a pheochromocytoma?
Adrenal medulla neoplasm
What is the most common symptom of a pheochromocytoma?
Hypertension
What is characteristic about the "attacks" seen in pheochromocytoma?
They are sudden severe attacks assoiciated with release of cathecholamines
What is the "Pluin triad" seen in pheochromocytoma?
Headache
Sweating
Palpitations
What are the laboratory findings of a pheochromocytoma?
Increased urinary free catecholamines and metabolites (VMA, metanephrines)
What are Robbins Rule of 10's for pheochromocytoma?
10% arise in association with familial syndromes
10% are extra-adrenal
10% of non-familial are bilateral
10% are biologically malignant
10% arise in childhood
Where do 10% of pheochromocytomas occur?
In the aortosympathetic system
What is characteristic of a pheochromocytoma seen histologically?
"Zellballen" nests of cells
What is MEN-1?
Werner Syndrome
What organs are affected in Werner Syndrome?
Parathyroid, Pancreas, Pituitary
What is MEN-2?
Sipple Syndrome
What is assoicated with Sipple Syndrome/MEN-2?
Meduallry carcinoma of the thyroid, Pheochromocytoma, and Hyperplasia of the PTH
What gene locus is mutant in MEN-2?
RET
What gene locus is mutant in MEN-1?
MEN
What is associated with MEN-2B?
Mucosal veroma and medullary carcinoma and pheochromocytoma
What do alpha cells of the pancreas secrete?
Glucagon
What do beta cells of the pancreas secrete?
Insulin
What do gamma cells of the pancreas secrete?
Somatostatin
What is the most important stimulus that triggers insulin synthesis?
Glucose
What is insulin's principle function?
To increase the rate of glucose transport into the cells of the body
What is the anabolic effect of insulin?
To increase synthesis and reduce degradation of glycogen, lipids, and proteins
What is Type 1 Diabetes Mellitus?
An absolute deficiency of insulin caused by an autoimmune destruction of beta cells (IDDM)
What is Type 2 Diabetes Mellitus?
Resistance to insulin and inadequate secretory response by the pancreatic beta cells
What is MODY?
A primary defect in beta cell function without beta cell destruction
What is gestational diabetes?
Any degree of glucose intolerance during pregnancy
What is the ADA criteria for the diagnosis of Diabetes Mellitus?
DM symptoms--polyuria, polydipsia, and unexplained weight loss PLUS a random plasma glucose greater than 200

OR

Fasting plasma glucose >126 repeated on a different day

OR

OGTT 2-hr PG >200
What is the intial diagnostic testing done for Diabetes Mellitus?
Fasting plasma glucose
What is the confirmatory test for diabetes mellitus?
Repeat fasting glucose on a different day
What is a monitoring test that can be done for Diabetes Mellitus?
Hemoglobin A1c--done following intial diagnosis and quarterly until patient is well controlled--once patient is well controlled can be done 2x a year

Self monitoring BG
Dipstick
What numbers are diagnostic diabetes mellitus?
126 or greater--provisional diagnosis of diabetes until confirmed by a test on a different day

110-125--impaired fasting glucose--represents an intermediate group "at risk"
What type of diabetes is insulinitis often associated with?
Type 1
What type of diabetes is amyloid often associated with?
Type 2
Who is typically affected by Type 1 Diabetes?
Children--usually less than 20 years old
Who is typically affected by Type 2 Diabetes?
Usually adults
What is the clincal presentation of a patient with Type 1 diabetes?
<20 years
Polyuria, polydipsia, polyphagia
Skinny
What is the clincal presentation of a patient with Type 2 diabetes?
>30 years
Common family history
Obese
Can be asymptomatic
3 P's
Blurred vision
Tingling in the feet
Which type of diabetes mellitus has a definite genetic component?
MODY--usually autosomal dominant
What is gestational diabetes?
Normal pregnancy creates a condition of impaired target organ response to insulin or insulin resistance--similar to type 2
Are gestational diabetics affected for life?
Not usually--glucose returns to normal rapidly
What are the maternal clinical associations with GDM?
More likely to develop hypertension and preeclampsia
Increased risk for C/S
Increased risk for PTL
Increased UTI's
Higher incidence of PP hemorrhage
What are the fetal clinical associations with GDM?
Macrosomia
Higher incidence of birth trauma--shoulder dystocia
What are the macrovascular complications seen in diabetes?
Atherosclerosis
MI
Stroke
Gangrene
What are the microvascular complications seen in diabetes?
Capillary dysfunction
Retinopathy
Nephropathy
Neuropathy

**Leading cause of blindness and renal failure**
What is the #1 cause of limb amputation?
Diabetes Mellitus
What is the #1 cause of death in diabetics?
Myocardial infarction
What are the syndromes associated with pancreatic endocrine neoplasms?
Insulinoma (Hyperinsulinism)
Gastrinoma (Zollinger-Ellison)
MEN syndrome
What is the criteria used to diagnose pancreatic endocrine neoplasm malignancy?
Metastases to lymph nodes or distant site
Vascular invasion
Gross invasion of adjacent tissues
What are the two types of pancreatic endocrine neoplasms?
Functioning and Non-functioning
What percentage of non-functioning pancreatic tumors have clinical evidence of metastisis?
90%
What is the best predictor of metastasis in pancreatic tumors?
The polypeptide secreted by the tumor?
What is the most common pancreatic neoplasm?
Insulinoma--Hyperinsulinsim
Are insulinomas most often benign or malignant?
Benign--90%
What is the clinical triad associated with pancreatic neoplasms?
Attacks of hypoglycemia
Confusion, stupor, loss of consciousness
Attacks precipitated by fasting or exercise--relieved by administering glucose
What is a gastrinoma?
Islet cell carcinoma secreting gastrin
Where do gastrinomas usually arise?
Duodenum, pancreas, or near pancreas
About how many gastrinomas are malignant
About 50%
What are the symptoms assoiciated with a gastrinoma?
Peptic ulcer disease
Diarrhea
What are the syndromes associated with pancreatic endocrine neoplasms?
Insulinoma (Hyperinsulinism)
Gastrinoma (Zollinger-Ellison)
MEN syndrome
What is the criteria used to diagnose pancreatic endocrine neoplasm malignancy?
Metastases to lymph nodes or distant site
Vascular invasion
Gross invasion of adjacent tissues
What are the two types of pancreatic endocrine neoplasms?
Functioning and Non-functioning
What percentage of non-functioning pancreatic tumors have clinical evidence of metastisis?
90%
What is the best predictor of metastasis in pancreatic tumors?
The polypeptide secreted by the tumor?
What is the most common pancreatic neoplasm?
Insulinoma--Hyperinsulinsim
Are insulinomas most often benign or malignant?
Benign--90%
What is the clinical triad associated with pancreatic neoplasms?
Attacks of hypoglycemia
Confusion, stupor, loss of consciousness
Attacks precipitated by fasting or exercise--relieved by administering glucose
What is a gastrinoma?
Islet cell carcinoma secreting gastrin
Where do gastrinomas usually arise?
Duodenum, pancreas, or near pancreas
About how many gastrinomas are malignant
About 50%
What are the symptoms assoiciated with a gastrinoma?
Peptic ulcer disease
Diarrhea
What are the syndromes associated with pancreatic endocrine neoplasms?
Insulinoma (Hyperinsulinism)
Gastrinoma (Zollinger-Ellison)
MEN syndrome
What is the criteria used to diagnose pancreatic endocrine neoplasm malignancy?
Metastases to lymph nodes or distant site
Vascular invasion
Gross invasion of adjacent tissues
What are the two types of pancreatic endocrine neoplasms?
Functioning and Non-functioning
What percentage of non-functioning pancreatic tumors have clinical evidence of metastisis?
90%
What is the best predictor of metastasis in pancreatic tumors?
The polypeptide secreted by the tumor?
What is the most common pancreatic neoplasm?
Insulinoma--Hyperinsulinsim
Are insulinomas most often benign or malignant?
Benign--90%
What is the clinical triad associated with pancreatic neoplasms?
Attacks of hypoglycemia
Confusion, stupor, loss of consciousness
Attacks precipitated by fasting or exercise--relieved by administering glucose
What is a gastrinoma?
Islet cell carcinoma secreting gastrin
Where do gastrinomas usually arise?
Duodenum, pancreas, or near pancreas
About how many gastrinomas are malignant
About 50%
What are the symptoms assoiciated with a gastrinoma?
Peptic ulcer disease
Diarrhea
What are the syndromes associated with pancreatic endocrine neoplasms?
Insulinoma (Hyperinsulinism)
Gastrinoma (Zollinger-Ellison)
MEN syndrome
What is the criteria used to diagnose pancreatic endocrine neoplasm malignancy?
Metastases to lymph nodes or distant site
Vascular invasion
Gross invasion of adjacent tissues
What are the two types of pancreatic endocrine neoplasms?
Functioning and Non-functioning
What percentage of non-functioning pancreatic tumors have clinical evidence of metastisis?
90%
What is the best predictor of metastasis in pancreatic tumors?
The polypeptide secreted by the tumor?
What is the most common pancreatic neoplasm?
Insulinoma--Hyperinsulinsim
Are insulinomas most often benign or malignant?
Benign--90%
What is the clinical triad associated with pancreatic neoplasms?
Attacks of hypoglycemia
Confusion, stupor, loss of consciousness
Attacks precipitated by fasting or exercise--relieved by administering glucose
What is a gastrinoma?
Islet cell carcinoma secreting gastrin
Where do gastrinomas usually arise?
Duodenum, pancreas, or near pancreas
About how many gastrinomas are malignant
About 50%
What are the symptoms assoiciated with a gastrinoma?
Peptic ulcer disease
Diarrhea
What are the main causes of Adult Respiratory Distress Syndrome?
Pulmonary infection
Gastric aspiration
Sepsis
Trauma
What happens to the lungs in ADRS?
Diffuse damage to alveolar capillary walls mediated by cytokines--IL-8, IL-1, and TNF--which lead to PMNs
Who usually gets ARDS?
Patients who are already sick
What is the clinical presentation of ARDS?
Rapid onset in already ill patients
Dyspnea and tachypnea
Cyanosis and respiratory failure
Diffuse bilateral infiltrates
Is ARDS associated with a high or low mortality rate?
High--60%
How does ARDS typically stain?
Pink--staining proteins--hyaline membrane
What is idiopathic ARDS?
Acute Interstital Pneumonia (AIP)--Hamman-Rich Syndrome
What is the difference between AIP and ARDS?
AIP has no associated causative disorder
What are the two categories of Chronic Diffuse Lung Disease?
Obstructive and Restrictive
What are obstructive diseases?
Diseases that limit the rate of flow due to resistance
What is the FEV1 level in obstructive diseases?
Markedly reduced
What are examples of obstructive lung diease?
Emphysema
Chronic Bronchitis
Bronchiectasis
Asthma
What are restrictive diseases?
Disease that limit the total lung capacity and residual volume with near normal flow rate
What is the FEV1 rate in restrictive diseases?
Mildly reduced
What are examples of restrictive diseases?
Chest wall disorders
Obesity
ARDS
Interstitial fibrosis
Pneumoconioses
What are the characteristics of obstructive lung diseases?
Air flow obstruction
Increased compliance
Decreased elasticity
Increased TLC
Barrell chested
Can't get air out
Decreased FEV
What are the characteristics of restrictive lung diseases?
Can't get air in
Decreased compliance
Increased elasticity
Decreased TLC
Decreased FEV
What are the two main types of emphysema?
Centriacinar/centrilobular (most common)
Panacinar/panlobular
Which type of emphysema is caused by smoking?
Centriacinar
Where in the lung is centriacinar emphysema seen?
Upper lobes/apices
What the main cause of panacinar/panlobular emphysema?
Alpha-1 antitrypsin and smoking
Where in the lung is panacinar/panlobular emphysema mostly seen?
Lower lobes/anterior
What are the localized types of emphysema?
Irregular/paracicatrical
Distal acinar/paraseptal
Which type of emphysema is associated with a damaged lung?
Distal acinar/paraseptal--spontaneous pneumothorax in young adults
What does emphysema destroy in the lungs?
The alveolar wall
What enzyme is deficient in emphysema?
Alpha-1 antitrypsin
What is the "primary villan" in emphysema?
Neutrophil secreted elastase
Do emphysema patients have adequate gas exchange?
Yes--Pink Puffers
Are emphysema patients acidotic or alkalotic?
Acidotic--can move CO2 outW
What is chronic bronchitis?
Defined as 3 months of productive cough/year for 2 consecutive years
What is the most common cause of chronic bronchitis?
SMOKING
Do chronic bronchitis patients have hyper or hypo secretion of mucus?
Hypersecretion of mucus
Is the Reid Index elevated or decreased in chronic bronchitis?
Elevated due to hypertrophy of submucosal glands
What is the most common finding of chronic bronchitis?
Destruction of the bronchus--small airways can also be involved
What often overshadows the lung problem in chronic bronchitis?
Cor pulmonale
Do chronic bronchitis patients have good or bad gas exchange?
Bad gas exchange--"Blue Bloaters"
What is the Reid Index?
Ratio of the thickness of mucous glands to wall from epithelium to cartilage
What is an abnormal Reid Index and with what disease is it usually seen in?
>0.4 in Chronic Bronchitis
What age group is typically affected by chronic bronchitis?
40-45
What age group is typically affected by emphysema?
50-75
Do chronic bronchitis patients have dyspnea?
Mild late in the disease
Do emphysema patients have dyspnea?
Severe early in the disease
What kind of cough is seen in chronic bronchitis?
Productive cough with copious sputum
What kind of cough is seen in emphysema?
Late cough with scant sputum
Are infections common in chronic bronchitis?
Yes
Are infections common in emphysema?
No--occasional
Is cor pulmonale more common in chronic bronchitis or emphysema?
Chronic bronchitis--rarely seen in emphysema
Is airway resistance increased in chronic bronchitis or empysema?
Chronic bronchitis
Is the elastic recoil low in emphysema or chronic brochitis?
Emphysema
What is seen on a chest x-ray of chronic bronchitis?
Prominent vessels and a large heart
What is seen on a chest x-ray of emphysema?
Hyperinflation and a small heart
Who are the blue bloaters?
Chronic Bronchitis
Who are the pink puffers?
Emphysema
What is asthma?
Chronic inflammatory disorder of airways--recurrent wheezing or breathlessness
When is asthma most often a problem?
At night and early morning
What are inducers of asthma?
Allergens
Exercise
Cold
Anxiety
Is asthma associated with bronchoconstriction or bronchodilation?
Episodic bronchoconstriction
What is status asthmaticus?
Prolonged severe attacks
What is atopic asthma?
Genetic tendency to develop IgE antibodies to inhaled allergens--usually seen in childhood
What is the pathogenesis of atopic asthma?
Mucosal mast cells react releasing inflammatory meidators--Stimulation of vagal receptors causing bronchoconstriction
What is non-atopic asthma?
Associated with pulmonary infections that are usually viral--no allergic indicators
What is drug induced asthma?
Asthma usually caused by aspirin
What is the morphology associated with asthma?
Overinflated lungs
Over production of thick mucous
Thickened bronchial basement membranes
Eosinophils in bronchial wall
Hypertrophy of submucosal glands and smooth muscle
Goblet cell metaplasia
What is pathognomonic for asthma?
Charcot Leyden Crystals--dead eosinophils

Curschmann spirals
What is bronchiectasis?
Permanent dilation of bronchi and bronchioles
What causes bronchiectasis?
Tissue destruction secondary to infection
What are the characteristics of restrictive lung diseases?
Can't get air in
Decreased TLC
Decreased residual volume
Decreased FEV
What is classic at the end stage of restrictive diseases?
Honeycomb lung
What is the pathogenesis of Idiopathic Pulmonary Fibrosis?
Unknown
What is another name for Idiopathic Pulmonary Fibrosis?
Cryptogenic Fibrosing Alveolitis
What happens to the pneumocytes in Idiopathic Pulmonary Fibrosis?
Type I pneumocytes die and Type II hyperplasia
What types of things are involved in Idiopathic Pulmonary Fibrosis?
Inflammation
Cytokines
Macrophages
Fibroblast proliferation
Collagen deposition
What are the symptoms of IPF?
Insidious, unpredictable
Dyspnea
Dry cough
Cyanosis

**Mean survival 3 years**
What leads to fibrosis in IFP?
Repeated healing and scarring
What is Cryptogenic Organizing Pneumonia?
COP/BOOP--Unknown etiology

Possibly an unusual reaction to known infection and inflammatory process
Is the pneumonia organized or unorganized in COP/BOOP?
Organized pneumonia--all connective tissue is the same age ad no interstital fibrosis--NO HONEYCOMB LUNG
Is COP/BOOP treatable?
Yes--usually responds to steroid treatment
What type of cells are usually seen in COP/BOOP?
Masson's bodies