Pituitary Adenomas

What is the major PE finding?

**Skin changes areas of hyperpigmentation

What are some more symptoms and the cause?

Orthostatic hypotension, hyponatremia, hyperkalmeia due to aldosterone deficiency and hypovolemia limit BP control and effective excretiong of proper watse products

What does Norepinephrine and Epinephrine impact?

Norepinephrine causes hypertension Epinephrine causes tachyarrhythmias

Do pts get their normal vision back?

Normalization of visual fields is observed in as many as 90% of pts

What can you do however?

Obtain acurate 24 hour Urine collection volume of <2L/24 hours rules out Diabetes insipidus

*What happens in Pheochromocytoma?**

Tumor that arises from adrenal medulla. Secrete both epinephrine and norepinephrine

What is Secondary Adrenocortical Insufficiency?

due to pituitary gland disorder. Lack of ACTH thus pts lack characteristic skin coloration changes

What about the face and base of the neck?

Often develop a rounded face and collections of at on the upper back and at the base of the neck (Buffalo Hump)- classic

What is hypernatremia?

elevated sodium level in the blood

What do you use to control HTN?

start w/ alpha blockers. phenoxybenzamine

Tx?

surgery

labs?

Prolactin level increased

What is an example of a secretory pituitary adenoma..that causes boobs

Prolactinoma: prolactin-secreting pituitary adenoma

What would be the pt's chief complaint?

"I have acute attacks of H/A, perspiration, palpitations, anxiety"

What happens in paragangliomas?

"extra-adrenal pheochromocytomas" Located along sympathetic nervous chain. Secrete norepinephrine

Tx of Pheochromocytoma? (2)

1. Surgical resection of tumor 2. Must treat HTN and tachyarrhythmias medically prior to surgery.

Info: Acute coronary syndrome-secondary to vasoconstriction MI, TIA, CVA Aortic aneurysms may dissect or rupture

...

How rare is this disease? When does it happen?(age) Male over female?

1 in 100,000 30s thru 50s M=F

What is Addison disease characterized by? (2)

1. Chronic deficiency or cortisol and often aldosterone 2. Elevation of serum ACTH

What are the four diagnostic techniques for Addison's disease?

1. Decreased Cortisol and Aldosterone levels 2. ACTH challenge test 3. An elevated blood level of ACTH 4. CT or MRI of Adrenal glands

What is the tx of Addison's Disease? (think of fixing the two problems)

1. Hydrocortisone (DOC)- to replace cortisol 2. Fludrocortisone acetate- to replace aldosterone

What percentage of reported cases of Addison's disease are caused by autoimmune disorders? (immune system makes antibodies that attack the body's own tissues or organs and slowly destroy them

70%

Adrenal insufficiency occurs when at least ______% of the adrenal cortex has been destroyed

90%

What is the etiology of Addison Disease? percentage?

Autoimmune adrenal insufficiency= 80% TB- most common world wide HIV Chronic fungal infections

Why do you have chronic deficiency of cortisol and often aldosterone (glucocorticoids and minerlocorticoids respecivetly) ? What's another name?

Bc adrenal cortices are dysfunctional. Adrenal insufficiency or hypocortisolism

Clinical Manifestations chart- CMDT

Blood pressure-HTN(severeormild), paraoxysmal or sustained; othrostasis, hypotension/shock, normotension Cardiovascular-palpitations, dysrhythmias, chest pain, acute coronary syndrome, cardiomyopathy, HF, cardiac paraganliomas Gastrointestinal- abdominal pain, nausea, comiting, wt. loss, intestinal ischemia; pancreatitits, cholecystitis, jaundice; rupture of abdominal aneurysm; constipation, toxic megacolon

Imaging of Pheochromocytoma

CT w/o contrast or MRI of abdomen (huge adrenals)

What are the clinical manifestations? (3) Can they be lethal?

Can be lethal unless they are diagnosed and tx appropriately 1. Catastrophic HTN crisis 2. Potentially fatal cardiac arrhythmia 3. Other symptoms-mimic anxiety attack

What are the symptoms and cause?

Chronic Fatigue, Muscle weakness, anorexia and wt. loss due to decreased cortisol>improper metabolism

Manifestation of symptoms described as "Acute onset of symptoms"?

HTN Severe headache Cardiac palpitations Anxiety tremor Tachyarrhythmias

What would you see?

Hand creases, dental-gingival margins, buccal and vaginal mucosa, lips, areola, pressure points such as elbows, knees, changes are also seen on scares and skin folds

Is Addison disease (Primary Adrenal insufficiency) common?

No. It's rare

What is another symptom and cause?

Hyperpigmentation of skin due to increased corticotropin levels due to decreased feedback

what are some more symptoms and cause?

Hyponatremia (demonstrated by salt craving) due to wt. loss and vomiting

What are triggers of Pheochromocytoma?..and Paraganglioma

IV contrast dye Anesthesia Surgical Procedures episodes can also be triggered by emotional stress, exercise, lifting, bending Drugs: MAO inhibitors, caffeine, nicotine, decongestants, amphetamins, cocaine, IV contrast, epinephrine

What is the tx?

IV fluids

Lab findins of Pheochromocytoma

Plasma fractionate free metanephrines-single most sensitive test 24-hour urine specimens- methanephrines (a metabolite of epinephrine) and catecholamines (epinephrine and norepinephrine)

Explain wt gain #2

Progressive weight gain.

Why sis it important to do the 24 hour urine test in addition to the serum test?

Since catecholamines secretion tends to fluctuate over time, 24-hour urine test for metanephrines or catecholamines may detect excess production that is missed with a blood test

Why would you have an elevation of serum ACTH?

bc pituitary is still working

What are most cases caused by?

gradual destruction of the adrenal cortex by body's own immune system

How common are these conditions?

incidence- 2-3 new cases per million population are dx annually

What is ACTH challenge test?

measure the amount of cortisol and aldosterone in the blood and urine and then document a lack of the normal increase in the levels of these two hormones after administration of ACTH given by injection

What is the result of this?

often both glucocorticoid (Cortisol) and mineralocorticoid (aldosterone) hormones are lacking because both are made in the adrenal cortex

what is the adrenal cortex?

outer layer of the adrenal glands

Are Prolactin-secreting pituitary adenomas common?

they are the most common and account for approx 30% of all pituitary tumors.

What's special about the limbs?

thin

What should you expect the symptoms to be then?

thus the symptoms are a collection of sympathetic nervous system hyperactivity

What is Pheochromocytoma and Paraganglioma?

tumor of the sympathetic nervous system

How does Addison disease occur?

w/ destruction or dysfunction of adrenal cortices

Explain Symptoms of androgen excess #4. What is it called?

Hirsutism-growth of coarse body hair in a male pattern

When does acromegaly occur?

after closure of epiphyses

Is it due to more salt intake?

noooooo, b/c water level is so low

Tx of Hyperprolactinemia? (2) main points

1. Medical Management <--start 2. Surgical management <--then go here

What is this due to?

Arises from the compression of the optic nerve by the tumor at the optic chiasm.

What are some of these illnesses?

Asthma rheumatoid arthritits Systemic lupus Inflammatory bowel disease S/P transplant *****Prolonged exposure to elevated levels of corticosteroids

The vast majority of Pituitary Adenomas are benign or malignant?

Benign

What is this loss of vision called?

Bitemporal Hemianopia

What is medical management of hyperprolactinemia?

Bromocriptine mesylate (drug of choice)

How are pts able to maintain fluid balance?

By continuing to ingest large volumes of water

What are some causes?

Damage to hypothalamus of pituitary stalk by tumor encephalopathy Surgical or accidental trauma Infection-encephalitits Metastases to pituitary more likely to cause than pituitary adenomas

What is diabetes Insipidus caused by?

Deficiency of vasopressin (ADH)

How do you classify them?

Depends upon when it occurs. Is the pt skeletally mature or immature? CLOSURE OF GROWTH PLATES?

How is cortisol secreted? (pattern)

Diurnal pattern, being highest upon awakening and lowest at bedtime

What is Bromocriptine mesylate? What does it do?

Dopamine agonist. Shrinks the prolactinoma.

Explain Glucose intolerance #5

Exces cortisolcan cause elevation of blood glucose levels. can lead to diabetes

Explain HTN and Cardiovascular disease #1

Excess cortisol raises blood pressure and puts stress on the heart and vascular system. Pt may suffer from any complication associated w/ HTN or DM. MI, CVA

What are the symptoms of Cushing's a result from?

Excess of cortisol

What would you find on PE w/ a pt w/ hyperprolactinemia?

Galactorrhea in females/gynecomastia in males visual-field defects

Explain Psychologic symptoms. #6

Over half of all pts with Cushing's syndrome have psychologic symptoms that range from loss of emotional control and depression to panic attacks and paranoia

People w/ visual field defects would complain of what?

c/o vision changes. Loss of temporal vision

What is the job of Coritsol?

counters insulin effects, tending to cause hyperglycemia by inhibiting insulin secretion and by increasing hepatic gluconeogenesis

How much is fluid intake increased by?

from 2L to 20 L per day

What is 24-hour Urinary Free Cortisol Level?

if Dexamethasone suppression test is abnormal. Findings suggestive of Cushing's is >3-4 times normal

When does Gigantism happen?

in youth, before closure of epiphyses

how much can Bromocriptine mesylate lower the prolactin level?

it can lower the prolactin level in 70-100% of pts

Explain galactorrhea

lactation in the absence of nursing. due to the direct physiologic effect of prolactin on breast epithelial cells

Where is IGF-1 released from?

liver

What is Cushing's Syndrome?

manifestation of excessive or high dose corticosteroids. Commonly an unfortunate side effect when due to medicaiton taken to treat illnesses

Cushing's Disease?

manifestations of hypercortisolism often due to ACTH hypersecretion by a benign adenoma in the pituitary

What would the indicate Cushing's Disease of Dexamethasone suppression test?

no change in coritsol on low-dose dexamethaone

What is also often associated with this? (manifested on EOM exam)

ocular muscle palsy and headache

WHere is ADH secreted from?

posterior pituitary gland

Explain why sexual dysfunction happens?

prolactin suppresses GnRH, causing a decrease in leteinizing hormone and follicle-stimulating hormone, ultimately leading to decreased serum testosterone levels and hypogonadism

What does reproductive dysfunction result from?

prolactin suppression of gonadotropin-releasing hormone (GnRH)-puberty

What does ADH do again?

regulates the body's retention of water by acting to increase water absorption in the collecting ducts of the kidney nephron. thus a lack of ADH reults in large quantities of dilute urine being expelled

Tx of Cushing's syndrome?

slowly ween off steroid

what does that mean? (what's the color/concentration?)

urine is clear- non-concentrated

When should you perform surgery?

when there is a failure to improve w/in 1-3 months

Explain Skin Changes # 3

Skin tends to become thin, fragile, and more susceptible to bruises and infections. Wounds heal poorly, and wide, reddish-purple streaks

What does Aldosterone due?

Stimulates the renal tubule to reabsorb sodium and excrete potassium, thereby protecting against hypovolemia and hyperkalemia

what are the reddish-purple streaks called? why?

Striae. B/c thinning of skin

What is the difference between Cushing's Syndrome/ Disease?

Syndrome is what we do-by giving lots of corticosteroids-long term

What is the percentage of Carcinoma? and Mestastases to pituitary?

0.1% to 0.2% of growths. Uncommon, seen in elderly. Usually from breast and lung CA

What are the four diagnostic techniques?

1. Dexamethasone suppression test (screening test) 2. 24-Hour Urinary Free Cortisol level 3. CT adrenals 4. MRI Pituitary

What are the clinical findings in women? (3)

1. Galactorrhea 2. reproductive dysfunction 3. Visual field changes, headache

There are 7 symtpoms. What are they?

1. HTN and Cardiovascular Disease 2. Wt. gain 3. Skin changes 4. Symptoms of androgen excess 5. Glucose intolerance 6. Psychologic symptoms 7. Infections

By remembering the job of cortisol in the body you can understand the symptoms******* list the jobs (5)

1. Help maintain BP and cardiovascular function 2. Helps slow the immune system's inflammatory response 3. Helps balance the effects of insulin in breaking down sugar for energy 4. helps regulate the metabolism of proteins, carbohydrates, and fats 5. Helps maintain proper arousal and sense of well-being

What are 5 functions of cortisol?

1. Helps maintain BP and cardiovascular funciton 2. Helps slow the immune system's inflammatory response 3. helps balance the effects of insulin in breaking down sugar for energy 4. helps regulate the metabolism of proteins, carbohydrates, and fats 5. Helps maintain proper arousal and sense of well-being

What are 2 clinical manifestations of Diabetes Insipidus?

1. Intense Thirst 2. Polyuria

What are the 3 effects of suppression of GnRH / reprod. dysfu.?

1. Oligomenorrhea 2. Infertility 3. Amenorrhea (in non-pregnant pts)- diminished menorrhea

What are some examples of manifestations associated w/ hormone hypersecretion or hyposecretion in secreting adeomas? (4)

1. Prolactin (hyper) 2. Growth Hormone (Hyper) or (hypo) 3. TSH (hyper) 4. Antidiuretic hormone (hypo from posterior pituitary)

What are the two types of Pituitary Adenomas?

1. Secreting 2. Non-secreting?

What are three manifestations of pituitary adenomas?

1. Visual Field Defects 2. headache 3. Manifestations associated w/ hormone hypersecretion or hyposecretion in secreting adenoma. 1 and 2 are space occupying 3-endocrine effect

What are the two categories of pituitary adenomas?

1. non-secreting pituitary tumors -> do not make extra hormones 2. Secretary pituitary adenomas - tumors secrete one or more hormones in excess ( usually found due to hormonal imbalances that affect bodily functions. they may be relatively small when detected.)

What are the 4 complaints of men with prolactin-secreting pituitary adenomas?

1. sexual dysfunction-erectile dysfunction, decreased libido 2. Gynecomastia w/o galactorrhea 3. Visual problems- visual field defects 4. Headache

What percent of adenomas are secretory?

50%

How much do tumors usually shrink?

50% of their original size in approx 90% of pts treated for macroadenomas for 1 yr

What can Cushing's disease also be cuased by?

ACTH secreting tumors such as small cell lung cancer- but most commonly it is due to pituitary adenoma

What is Primary Adrenal Insufficiency? What's the name? Which President had this?

Addison's Disease. JFK. pts have all symptoms including increased ACTH levels due to decreased feedback which causes characteristic skin coloration changes

What is Dexamethasome Supression test (screening test)?

Administer dexamethasone at 11 pm and then serum collected for cortisol at 9 am the next day.

What is that major mineralocorticoid secreted by outer layer of the adrenal cortex?

Aldosterone

What is the major glucocorticoid secreted by adrenal gland?

Cortisol

Expain Infections #7

Cortisol syppresses the immune system, and ppl w/ Cushing's syndrome may develop infections more frequently

What would be a normal result of Dexamethasone suppression test?

DECREASE in cortisol levels upon administration of low-dose dexamethasone

What are some features of acromegaly?

Hands enlarge, doughy, moist handshake Fingers widen, carpel tunnel syndrome Facial features: Bones and sinuses of skull enlarge, hat size increases, mandible more prominent-causing malocclusion, tooth spacing widens, macroglossia-hypertrophy of pharyngear and laryngeal tissue-causes deep, coarse voice

What can occur in pts w/o access to water or a damaged hypothalamic thirst center/altered thirst sensation?

Hypernatremia and dehydration

What is the pathway?

Hypothalamus sends corticotropin releasing hormone (CRH) to pituitary gland. CRH caused the anterior pituitary to secrete ACTH, which then stimulate the adrenal glands, which they respond by releasing cortisol into the bloodsteam

What are the lab findings?

IGF-1 (increase over 5 times normal) -diagnose GH stimulates the release for Insulin-like growth Factor-1 (IGF-1)

What happens when pts have Diabetes Insipidus?

Increase thirst and passage of large quantities of urine of low specific gravity

Why do you administer dexamethasone?

It is an exogenous steroid thtat provides negative feedback to the pituitary to suppress the secretion of ACTH

Is Diabetes insipidus common? Is it excessive secreting or deficient?

It is uncommon. Deficiency!!!!

Diagnosis of prolactiin-secreting pituitary adenoma?

MRI to evaluate for presence of tumor Serum evidence of hyperprolactinemia

Imaging?

MRI- shows pituitary tumor (90% are benign)

Tx of diabetes Insipidus? (2)

Mild-no tx other than adequate fluid intake Desmopressin acetate (DDAVP)-tx of choice

Do pts develop all of the symptoms? How do they sypmtoms progress?

Most pts deveop at least a few of these symptoms, and the symptoms typically worsen over time.

If it's past 3 months and the prolactin levels are the same..what do you do next?

Move on to surgery.

What is Acromegaly/Giantism?

Pituitary adenoma causing increase in release of Growth Hormone from anterior pituitary

Examples of Secretory Pitutiary adenomas

Pituitary adenomas have specific signs and symptoms that are primarily related to the endocrinopathies produced by hypersecretion

What are the two Adrenal Insufficiencies?

Primary and Secondary

Is it rare? Who does it affect most?

Rare Most commonly affects adults aged 20-50 F>M 5:1

What is the diagnosis of Diabetes Insipidus?

There is no single diagnostic lab test

How common are Pituitary Adenomas?

They are common often benign tumors of the pituitary gland. It is estimated that up to 10% of ppl will have a pituitary adenoma ( which might never have caused a problem) by the time of their death.

What is prolactin's primary function again?

To enhance breast development during pregnancy and to induce lactation

What is performed?

Transsphenoidal removal of pituitary tumor. Up through the nose.

What is the tx of Cushing's disease?

Transsphenoidal resection of pituitary adenoma

Where does it normally affect? What is this called?

Truncal obesity. Affects the face, neck, trunk, abdomen

What is the most common symptom?

Weight gain

When can people develop Pituitary adenomas?

at any age. Most pituitary adenomas are in the front part (anterior lobe) of the pituitary gland