Name 2 major conditions in which the Adrenal glands are hyperfunctioning.

Adrenal Hypercortisolism

Primary Hyperaldosteronism

T/F 

The adrenals cannot be seen on standard abdominal plain films but can be seen on CT

TRUE

The medulla of the Adrenals produces [blank]

The cortex of the Adrenals produces [blank]

Catecholamines

Steroids

Are there syndromes in which the adrenal medulla dosn't make enough catecholamines?

Not really because other organs make plenty

What are Catecolamines

fight or flight hormones

exp:  Epi, NorEpi, Dopamine

They are made from Phenylalanine and Tyrosine

What is Cushing's Syndrome?

Adrenal Cortical Hyperfunction

What is Cushing's Disease?

Adrenal Cortical Hyperfunctio due Specifically to an

ACTH - producing Pituitary adenoma

Who usually gets Cushing's Syndrome?

And what is the most common etiology?

Females 25-45

Iatrogenic (administeration of exogenous glucocorticoids)

What 6 Hormones does the Anterior Pituitary Secrete?

What 2 Hormones does the Posterior Pituitary Secrete?

Anterior Pituitary:  LH, FSH, ACTH, TSH, GH, PRL

Posterior Pituitary:  Vasopressin, Oxytocin

What 2 Neoplastic processes can cause Adrenal Hypercortisolism?

Functioning Pituitary Adenoma

(this secretes excess ACTH which leads to b/l Adrenal Hyperplasia)

Cortisol-secreting Adenoma, Carcinoma, or nodular hyperplasia (results in hypercortisolism)

What is the most common cause of endogenous overproduction of adrenal hormones?

Functioning adrenal adenoma

(60-80% of endogenous etiology)

What is a Paraneoplastic cause of Adrenal Hypercortisolism?

An ectopic ACTH secreting tumor  (often in the lungs)

Who gets Pseudocushing's syndrome?

Alcoholics

Very obese individuals

What is the Diff Diagnosis for Cushing's syndrome?

ACTH dependent cushings syndrome

Cushing's disease

Ectopic ACTH

Ectopic CRH

ACTH independent cushings syndrome

iatrogenic

adrenal adenoma

micronodular hyperplasia

macronodular hyperplasia

Hypercortisolism presents with the followins symptoms:

(regardless of etiology)

• Muscle weakness, fatigability, and osteoporosis
• Cutaneous striae and easy bruisability
• Increased hepatic gluconeogenesis and insulin resistance\overt T2DM in <20% of patients
• Visceral obesity, interscapular fat, and moon facies
• Hypertension
• Emotional changes, depression, confusion and psychosis

Describe the overnight Dexamethasone suppresion test

Dexamethasone, which is like cortisol, decreases the amount of ACTH released by the pituitary gland, which in turn decreases the amount of cortisol released by the adrenal glands.

After taking a dose of dexamethasone, cortisol levels often stay abnormally high in people who have Cushing's syndrome. Occasionally other conditions (such as major depression, alcoholism, stress, obesity, kidney failure, pregnancy, or uncontrolled diabetes) can keep cortisol levels from going down after taking a dose of dexamethasone.

The night before the blood test, you will take a pill containing dexamethasone. The next morning, the cortisol level in your blood will be measured. If your cortisol level remains high, Cushing's syndrome may be the cause

What are the tests you would order when suspecting Cushing's syndrome?

• Dexamethasone suppression test: overnight, low-dose and high-dose versions
• Plasma ACTH levels
• 24 hour urinary free cortisol levels
• ACTH and CRH challenge tests
• Metyrapone test
• MRI and CT studies of the sella and abdomen

Describe the ACTH and CRH challenge test

Describe the metyrapone test

 It is a test to perform when suspecting Cushing's syndrome

decrease the amount of Cortosol to see if ACTH rises apropriately.

How do you treat Adrenal Hypercortisolism?

Primary goal is to LOWER CORTISOL

• Major goal is to reduce cortisol levels
• Surgical removal
• Pituitary adenoma
• Adrenal adenoma, carcinoma or hyperplasia
• Any ectopic sources
• Irradiation of the pituitary can be considered
• Medical (chemical) adrenalectomy

What are some important aspects of managing somone who has adrenal hypercortisolism?

wean them off of steroids post gland reduction treatment

try to prevent medication dependence

Long term monitoring of cortisol levels for signs of return

You don't want to take mitotane while pregant or breastfeeding

What is hyperaldostronism?

A condition in which there is hyper secretion of the mineralcorticoid Aldosterone

Aldosterone is usually made by the adrenal cortex

What is Conn's Disease

Hyperaldosteronism that is Specifically due to an Adrenal Adenoma

What is the relationship between primary and secondary hyperaldosteronism and HTN?

HTN is RARELY ever associate/caused by primary hyperaldosteronism

however

HTN can cause secondary hyperaldosteronism via stenosis of the renal arteries.

What are the etiologies of Primary Hyperaldosteronism?

Adrenal Adenoma (conn's dz) (usually the cause)

B/L adrenal cortical nodular hyperplasia

more frequent in women

usually 30-50

What are the etiologies of secondary hyperaldosteronism?

Renal artery stenosis

HTN

Edema

S&S of Hyperaldosteronism

• Diastolic hypertension (can be severe)
• Headaches
• Potassium depletion (hypokalemia)
• Weakness and fatigue
• Other associated signs and symptoms include:
  • Left ventricular hypertrophy
  • EKC changes
  • Cardiac arrhythmias
  • Polyuria and polydipsia
  • Renal failure edema can occur

Aldosterone

secreted by adrenal cortex

Aldosterone is a hormone that increases the reabsorption of sodium and water and the release (secretion) of potassium in the kidneys

What tests are you going to order if you suspect Hyperaldosteronism?

• Discontinue any diuretics if present
• Serum potassium levels
• Plasma rennin levels
• Plasma aldosterone levels
• CT or MRI imaging of the abdomen

List the 5 disorders for Adrenal Cortical Hypofunction

Primary Adrenal Insufficiency

Secondary Adrenal Insuficiency

Acute Adrenal Insuficiency

Hypoaldosteronism

Congenital Adrenal Hyperplasia

What is Primary Adrenal Insufficiency

and what can cause it?

Addison's Dz

Progressive destrucion if the Adrenals (>90%)

all sorts of things can cause it

#1 is autoimmune destruction (85%)(adrenalitis)

#2 vascular disease (hemorrhage)

Bacterial Infection (TB) used to be most common in us but no longer

What are the S&S of primary adrenal insuficiency?

• Asthenia, fatigability, weakness,
• Weight loss
• Hyperpigmentation
• Hypotension
• GI abnormalities such as anorexia, nausea and vomiting

What testing will you order if you suspect Primary adrenal insuficiency?

• Serum cortisol levels
• Serum electrolytes
• ACTH stimulation test
• Abdominal plain film (calcification)
• Abdominal CT scan

How do you treat primary adrenal insuficiency?

Adrenal cortical steroid replacement

What are your expected management needs / complications

for primary adrenal insuficiency?

• Fatal if untreated or poorly compliant
• Monitor serum electrolytes
• Monitor bone density
• Adrenal function will not recover
• Relatively normal life expectancy is possible with appropriate treatment
• Plan of action in the even to adrenal crisis

Explane Acute Adrenal Crisis

acute adrenal crisis can manifest with vomiting, abdominal pain, and hypovolemic shock. When not promptly recognized, adrenal hemorrhage, seen in the image below, can be a cause of adrenal crisis.

What is secondary Adrenal insuficiency?

What are its causes?

Adrenal insuficiency due to the suppression or loss of ACTH.

Etiologies:  damage to ant pituitary or hypothalamus,  medicinal suppresion of ACTH

• Iatrogenic disorder (rapid cessation of glucocorticoid therapy or pituitary irradiation)
• Traumatic disorder (surgical removal of a pituitary tumors or destructive lesions such as pituitary tumors)
• Vascular disease (pituitary hemorrhage or infarction)
• Deposition diseases (sarcoidosis and amyloidosis)

What are the S&S  of secondary adrenal insuficiency?

Same as primary only no hyperpigmentation

(so weakness, fatigue, anorexia, weight loss)

What are you going to order for tests if you suspect secondary adrenal insuficiency?

How will you treat it?

• Serum cortisol levels
• Serum electrolytes
• ACTH stimulation test
• Abdominal plain film (calcification)
• Abdominal CT scan

Treatment:  similar to primary adrenal insuficiency --- hormone replacement.

What is Acute adrenal Insuficiency?

And what are its etiologies?

"Adrenal Crisis"

life threatening emergency

Septic Shock

Hemorrhage

(usually associated with infection in infants - known as Waterhouse-Friderichsen Syndrome) (also anticoagulaiton or DIC in adults)

Rapid withdrawl of Steroid medication

What are some major risk factors for developing an Adrenal Crisis?

• Infection, trauma or stress in those with chronic adrenal insufficiency
• Heavy steroid useage
• Congenital adrenal hyperplasia
• Treatment with steroid inhibiting drugs, i.e. ketoconazole

What does Adrenal Crisis Look Like?

How do you treat it?

• Shock
• Nausea, vomiting and abdominal pain
• Lethargy and somnolence
• Hypovolemic vascular collapse

• Replacement of electrolytes and glucose
• Hormone replacement
• Vasoconstriction if necessary

What is Hypoaldosteronism?

What does it look like?

D: under production of aldosterone from the adrenal gland (rare)

congenital enzyme disorder 

renal tubular disorder.

• Weight loss
• Gastrointestinal disturbances
• Low blood pressure
• Salt craving
• Dizziness and lightheadedness
• Palpitations
• Low blood sodium, high potassium (hyperkalemia, hyponatremia)
  
• Increased blood renin levels

What is Congenital Adrenal Hyperplasia?

CAH represents a family of recessive mutations that affect the adrenal steroid biosynthesis pathways.

Damming of the cortisol biosynthetic pathway results in precursor built-up and spill-over into the androgen pathway --> virilization

21-hydroxylase deficiency is most common (salt wasting form with low aldosterone and cortisol)

11b-hydroxylase deficiency (non-salt waisting) 11-deoxycorticosterone and 11-deoxycortisol build up in the adrenal gland; these compounds have aldosterone-like properties and trigger both hypertension and hypokalemia

Describe the presentation of 21-hydroxylase deficiency

Describe the presentation of 11-hydroxylase deficiency

• episodic acute adrenal insufficiency
• hyponatremia
• hyperkalemia
• dehydration
• vomiting
• virilization

• hypertension
• hypernatermia
• hypokalemia
• virilization

What are you going to do to diagnose CAH?

• Physical exam
• Routine serum chemistries
• Urine 17-ketosteroids
• Plasma DHEA (Dehydroepiandrosterone)
• Amnioic fluid 17-hydroxyprogesterone

How will you treat CAH?

• Adrenal cortical hormone replacement
• Suppression of ACTH production is necessary
• When prenatal diagnosis is possible, ACTH suppression can be initiated during pregnancy
• Monitoring of skeletal growth and maturation for the effects of glucocorticoids
• Reconstructive surgery can be used to correct virilization in cases of ambiguous genitalia

Name 2 kinds of Adrenal Masses

Benign Non-functional Adrenal Mass

Mallignant Adrenal Mass

What is a benign non-functional adrenal adenoma

Nonfunctional adrenal adenomas are well-circumscribed lesions in the gland.When detected on routine screening they are termed “incidentalomas”.

90% of incidentalomas are non-functional

30-50% of masses will rep Metastasis

What is Malignant adrenal carcinoma

can be of any size

• Very rare but quite lethal
• Probability of an adrenal mass being a carcinoma is <0.01%
• More likely to be functional than adenomas
• Metastasis to region lymph nodes
• Hematogenous spread to lungs and other viscera

What does malignant adrenal carcinoma look like

• Large size, mass effect possible
• Irregular margins
• Inhomogeneity
• Calcifications present on imaging

What is an example of Adrenal medullary Hyperfunction

Pheochromocytomas are neoplasms of the adrenal medulla and surrounding paraganglia that can produce catecholamines as well as a few peptide hormones.

most are benign but those of paragaglia are more likely to become malignant

• Extra-adrenal locations typically involve the autonomic ganglia and region of the hypogastric plexus
• Right-sided predominance
• Tumors contain chromaffin cells
• Less than 10% are malignant
• Produce norepinephrine, epinephrine and dopamine

How do Pheochromycytomas present?

• Episodic hypertension similar to seizures or anxiety disorders
• Unresponsive hypertension
• Headaches, excessive sweating and palpitations
• Sense of impending doom
• Chest and abdominal pain can be present
• Mild to moderate weight loss
• Cardiac abnormalities
• Paroxysms or crisis occurs in over half of patients

What tests will you order to diagnose Pheochromocytomas?

Urinary tests

• Vanillylmandelic acid
• Metanephrines
• Catecholamines
• Creatine

Phentoamine test during crisis if necessary

Glucagon test (potentially life-threatening)

CT and MRI imaging

How would you treat Pheochromocytomas?

• alpha-receptor blockade
• Surgical removal with proper precautions

What might help you identify Pheochromocytoma on radiograph?

Focal areas of low attenuation within the mass on the CT images.

Represent cystic areas containing fluid.

What is MEN2 Syndrome?

MEN 2 is a rare familial cancer syndrome caused by mutations in the RET proto-oncogene. Inherited as an autosomal dominant disorder, MEN 2 has 3 distinct subtypes, including MEN 2A, MEN 2B, and familial medullary thyroid carcinoma – only (FMTC-only). The subtypes are defined by the combination of tissues affected. Developmental abnormalities may also be present. By age 70 years, the penetrance rate is 70%. Genetic testing and clinical surveillance beginning in childhood provide the opportunity to treat the devastating and sometimes fatal complications of this disorder

what are some microscopic feature you would expect to see of a thyroid slide

note vast number of follicles

note the coloid in the follicles

each follicle is surounded by cuboidal epithelium which make the coloid.

note parafollicular cells or C cells which make calcitonin and are in the interfolicular space... their cytoplasm is lighter and they are bigger.

tumor of Parafolicular cells

medullary carcinoma???

Thyroid Hormones

thiosine derivatives

condensation reaction required to make functional hormone

T4 (4 iodines)

T3 (3 iodines)... this is functional

You need iodine

GI system needs to absorbe and change to iodide for circulation... it is in very low concentration.... so you have to have active transport to get it out of circulation and into follicular cell

it is degraded in Liver

it is excreted in kidney

what are the proteins that bind Thyroid hormone?

Thyroglobulin Binding Protein (most)

Transthyretin

Albumin

Increase

Pregnancy

estrogen therapy

Hepatitis

chronic heroin abuse

Decrease

Steroid use

Nephrotic syndrome (can't hold onto protein)

Thyroid hormone

activating enzyme

T4 --> T3

Deiodinase Type 1,2,3

type 1 is the more active one in the perifery

type 2 is found in the pituitary ... feedback regulation

type 3 is more of an inhibitory (inactivates t3,t4)

Different enzymes leads to the possibility of un sinced sensitivities

Activation of Thyroid hormone receptors does what???

Increase metabolic activity

How is Thyroid Hormone Regulated?

Thyrotoxicosis

over amount of thyroid hormone in you

hyperthyroidism means that the glad is making too much

cretinism is when hypothyroidism is when it occurs in kids

Myxedema ....hypothyroidism in older child or adult... also seems to being refering specifically to the edema

Thyroiditis... thyroid is infected or is being degraded by the immune system.

Graves Disease

primary cause of hyperthyroidism

Diffuse hyperplasia of cells

Hashimoto's Thyroiditis

Anatomical Abnormalities of the Thyroid gland

what are they?

Goiter

Diffuse:  from iodine deficiency (non-toxic)

multinodular: toxic, euthyroid)

Solitary nodule (euthyroid or toxic)...not a goiter

How does hypothyroidism lead to high cholesterol?

Thyroid Hormone has what effects on the heart?

chronotropic and inotropic effects

(speeds it up and increases the force of contraction

why are patietns with hyperthyroidism weak?

because they are breaking down lots of protien  (not just fat)  in their hypermetabolic state.

Bruit over Thyroid is diagnostic for?

Grave's Dz

what is the most common disconjucate gaze (diplopia) finding of Graves

cant move up and to the right

inferior rectus gets scared and cant contract

what is an early sign of ischemid damage damamge to the optic nerve associated with Grave's dz?

Loss of color vision

What is the natural hx of Graves dz if untreated

Slow progression to eventual death

Or

Because of the infalmation... the gland can burn itself out over years...and then become hypothyroid

T/F 

HCG can stimulate the thyroid

True

it looks enough like TSH

Thyrotoxicosis

indicates an over abundance of thyroid hormone in circulation but does not indicate the etiology

What Etiologies of Thyrotoxicosis will present as high radioactive iodine uptake?

Graves Dz

Toxic adenoma

Toxic multinodular goiter

Iodine induced thyrotoxosis

Hashimoto's Thyroiditis

what is the goal% of HBA1C  in a Diabetic under your care?

7%

What causes the thyroid gland to produce too much thyroid hormone in Grave's dz?

An autoantibody that stimulates the TSH receptors on the follicular cells of the gland

What is the primary cause of Thyrotoxicosis?

Grave's Dz

What are some symptoms of Grave's Dz?

• Heat intolerance
• Weight loss >weight gain
• Increased sweating
• Cardiac palpitations and achycardia
• Diarrhea Amenorrhea/light menses
• Tremor Weakness and Fatigue
• Nervousness Irritability
• Insomnia

What are some clinical signs of Grave's Dz

• Thinning of hair
• Proptosis, Lid lag or lid retraction - “stare”, chemosis
• Goiter, bruits
• Flushed, moist skin
• Increased reflexes
• Pretibial myxedema
• Vary with duration of the disease and age of the patient
• Note specifically Graves ophthalmopathy and thyroid dermopathy

What tests will you order to Diagnose Grave's Dz?

• TSH levels
• T4 levels
• T3 if necessary
• ^99mTc, ¹²³I, or ¹³¹I scan of the thyroid if necessary
• MRI or CT of the head (sella) if necessary

What are the treatment options for Grave's Dz

• Reducing thyroid hormone synthesis with antithyroid drugs
• Iodides (Wolff-Chaikoff effect)
• Thionamides (blocks organification of the iodides)
• Beta-blockers
• Reducing thyroid tissue with radioiodine (¹³¹I)treatment (RAI)
• Surgical resection, when necessary

What are your management and complication concers with Grave's Dz?

• Monitor thyroid function tests
• Radiation safety
• Pregnancy and breast feeding issues
• Thyrotoxic crisis (thyroid storm)
• Wolff-Chaikoff effect
• Ophthalmopathy
• Lupus-like syndrome and agranulocytosis side-effect profile of thioamide treatment

what is the blood supply to the the thyroid?

Superior Thyroid arteryies --- external carotid

Inferior thyroid arteries  --- thyrocervical trunk off of subclavian

Thyroid ima artery --- the right subclavian carotid trunk

What cells of what organ make Calcitonin and what does Calcitonin do?

Parafolicular cells or light cells of the thyroid make Calcitonin.

This hormone lowers blood calcium levels by inhibiting bone resorption. The secretion of calcitonin results from elevation of blood calcium concentration above normal levels

Thyroid receptor

Binding constant for T3 is 10 higher than for T4

Local conversion of T4 to T3 by type II deiodinase

In general, it stimulates protein synthesis

Organs effected most by Thyroid receptor: Liver, anterior pit, cerebral cortex

It stimulates growth, differentiation, and general metabolism

Stimulation of the thyroid receptor causes elevation of what?

Na-K pump

Gluconeogenic enzymes

Respiratory enzymes

Myosin heavy chain

Beta Adrenergic Receptors

and more

ie it increases metabolic activity

What are some major actions of Thyroid hormone?

What Stimulates Thyroid hormone and what suppresses it?

in terms of regulation

Stimulation:  Thyrotropin Releasing Hormone

Suppression:  Somatostatin and Dopamine

Glucocorticoids

IL-1, IL-2, TNF

Thyroid Antibodies ?

What is the most common cause of Hypothyroidism?

Hashimoto's Thyroiditis

Cytotoxic T lymphocyte-associated antigen-4 (CTLA4)

Protein-tyrosine phosphatase 22 (PTPN22)

A Carcinoma of the Thyroid shows "undifferentiated aggressive tumors" cells

what will you call it?

Anaplastic Carcinoma

A Carcinoma of the Thyroid shows

interstitial “C” cells, neuroendocrine tumors

what will you call it

Medullary Carcinoma

If you had a Thyroid Carcinoma

that show follicles of colloid

what will you call it?

Follicular Carcinoma

If you have a Thyroid Carcinoma that shows

Papillary folds of epithelium what will you call it?

Pappilary Carcinoma

What are the signs and symptoms for HYPOTHYROID?

Tired, week, fatigued

Social withdrawl and Depression

weight gain and hoarseness

Cold intolerance and Reynaud's phenomenon

Decreased Sweating

Thick Coarse hair

Facial Myxedema

Loss of lateral 3rd of eyebrow

Cold, dry, thickened skin

Constipation

What usually causes Subacute Thyroiditis?

What usually causes Acute Thyroiditis

Viral infections or post viral inflamation via cellular mimicry

Bacterial or fungal infection, radiation therapy or drug therapy (amiodarone)

What do the following refer to

• Granulomatous thyroiditis
• De Quervain thyroiditis
• Painful thyroiditis

Subactue Thyroiditis

(the most comon cause of painful thyroiditis)

What is the Presentation of Subacute Thyroiditis

• Painful, enlarged thyroid (goiter); pain can refer to jaw or ear
• Odynophagia frequently present
• Fever can be present
• Malaise and URT infection signs and symptoms can precede the presentation of thyroid-related features
• Thyrotoxicosis can be present initially
• Hypothyroidism characterizes later stages of the disease

What is the #1 cause of hypothyroid in kids

Hashimoto's thyroiditis

True or False

Hashimoto's thyroiditis is more common in the older adult and more common in women

true

what testing would you order/ test results would you expect to get in a patient who has

Hashimoto's Thyroiditis

• Decreased free T4
• Decreased total T3
• Elevated TSH
• Positive Auto antibodies (in 95% of Hashimoto’s)
• RAIU of less than 10%
• Elevated cholesterol
• Anemia
• Elevated
• CPK Decreased sodium
• Elevated LDH
• Elevated AST
• Elevated ESR

How would you treat Hashimoto's Thyroiditis

Give them Levothyroxine replacement thyroid hormone

How would you manage someone with Hashimoto's thyroiditis

• Follow-up levels of TSH
• Prevention and Health Promotion:
• Compliance issues
• Pregnancy issues
• Dosage adjustment in the elderly
• Myxedema coma
• Myxedema heart
• Cardiac disease of hypothyroidism
• Anemia of hypothyroidism

what are the classic signs and symptoms of Hashimoto's thyroiditis

• Weakness, tiredness, lethargy, fatigue, cold intolerance, headache, loss of taste or smell, deafness, hoarseness, absence of sweating, modest weight gain
• muscle cramps /aches/ pains
• dyspnea, slow speech, angina pain, constipation, menorrhagia, galactorrhea
• Thin brittle nails, thinning of the skin, pallor, puffiness of face/eyelids, yellowing of skin, thinning of outer eyebrows, thickening of tongue, peripheral edema
• Pleural/peritoneal/pericardial effusions, decreased DTRs, “myxedema heart”, bradycardia, hypertension, goiter if primary hypothyroidism
• Goiter can be the initial symptom

What are some causes of congenital thyroiditis

who tends to get it

thyroid dysgenesis

thyroid ectopia

defective synthesis of thyroxine

defects in iodide transport

defects in thyroid peroxidase

defect in thyroglobulin

Maternal antibodies

Maternal Medications

Girls > Boys

How do you test

How do you treat

what are the complications

• Neonatal screening tests are important
• TSH levels
• T4 levels

T4 replacement

Mental retardation

What is the primary cause of iatrogenic thyroiditis

how do you treat it

how do you manage it

Hypothyroidism post surgical removal of thyroid tissue surgically or radiation (takes about 3 months to present)

levothyroxine

TSH level monitoring

What is Thyroid Resistance Syndrome

Mutation in the Thyroid hormone Receptor beta gene

results in Clinical Hyopthyroidism but high TSH levels and high Thyroid Hormone levels

What are some common S&S of thyroid resistance syndrome

How would you test for it

How would you treat it

• Goiter
• Attention deficit disorder
• Mild reduction in IQ
• Delayed skeletal maturation
• Tachycardia

• TSH levels
• T3 and T4 levels
• TSH challenge test with T3

We don't know how to treat

What type of cancer is the most common cancer of the Endocrine system?

Thyroid cancer

Who gets thyroid cancer and in whom is it most severe

risk increases as you age

risk increases if you are a woman

disease is most severe in kids and men

your patient has a thyroid nodule that takes up radioactive iodine and is thus "hot"

what is the likely malignancy status

most likely benign

Are solitary thyroid nodules more likely to be malignant or benign

Are thyroid nodules in men and kids more likely to be benign or malignant?

malignant

malignant

What is the pathogenesis of a Toxic solitary thyroid nodule?

acquisition of an activating mutation in a TSH-R protein or its signaling pathway

How would you test a solitary thyroid nodule

and how would you treat it?

Serum TSH

Thyroid Scan

• Radioiodine ablation
• Surgical ablation of the nodule
• Antithyroid drugs for initial stabilization of thyroid function if necessary
• Ethanol injections

toxic nodule can become malignang

treatment can cause rebound hypothyroid

A solitary nodule of the thyroid is termed a

Toxic adenoma

What is a goiter?

A goiter is an non-nodular enlargement of the thyroid gland usually due to either defects in biosynthesis of thyroid hormones or in the availability of iodine.

What is a Non-toxic Multinodular Goiter?

Nontoxic multinodular goiters are non-functional in nature. However, when chronic, these goiters can eventually develop hyperfunction, thereby evolving into a Toxic Multinodular Goiter.

they are far more common in women

most patients are euthroid with high to normal TSH

What is a toxic multinodular Goiter?

Toxic multinodular goiters are similar to the nontoxic goiters in appearance but contain autonomously functioning nodules.

commonly assoc with mutation in TSH receptor

What is a follicular adenoma of the Thyroid?

Follicular adenomas are typically benign, discrete, solitary nodules that, for the most part are non-functional. The few that do form thyroid hormones are then referred to a toxic adenomas.

Painless mass

What is a Papillary thyroid carcinoma

most common type of thyroid cancer

presents 20-40

Cytology shows psamomma bodies and papillary structure

rapidly expanding, usually free moving but ominous if fixed

What is a Follicular Thyroid Carcinoma?

A single, well-circumscribed lesion containing some follicles with colloid. Lesions may be very large, exceeding the extent of the gland and causing a mass effect in the neck.

2nd most common form of Thyroid cancer

women over 40

Slowly Enlarging Painless nodule

What is an Anaplastic Thyroid Carcinoma?

They are the least differentiated and most aggresive neoplasm of the Thyroid

Prognosis is very poor

Survival less than 6 months

What is a Medullary Thyroid Carcinoma?

Neoplasms derived from the neuroendocrine parafollicular cells (C cells) in the stroma of the thyroid from medullary carcinomas.

• Secrete calcitonin
• Can also secrete carcinoembryonic antigen, somatostatin, serotonin, vasoactive intestinal polypeptide
• Some tumors occur in the setting of the MEM type 2 syndromes
• Mutations in the RET protooncogene

Myxedema Coma

Myxedema coma is a loss of brain function as a result of severe, longstanding low level of thyroid hormone in the blood (hypothyroidism). Myxedema coma is considered a life-threatening complication of hypothyroidism

a very serious complication of hypothyroidism that is virtually unique to the elderly.

What are some unique aspects of Thyroid Diease in the Elderly?

There is some natural degenertaion

Myxedema Coma is a serious life threatining complication in the elderly

Common medications in the elderly effect the hypothalamic-pituitary-thyroid axis

"Apathetic Hypothyroidism"

 What are some important aspects of Hypothyroidism in the Pregnant Woman?

Several hormonal factors in pregnancy interact with the hypothalamic-pituitary-thyroid axis to alter thyroid hormone levels. Adequate control of the thyroid axis during pregnancy is extremely important due to the severe consequences hypothyroidism can have on the developing fetus.

Dx: they require increased doses or replacement hormone?

25% increase in their baseline dose of Thyroid hormone replacement

goal is to reach normal or slightly normal levels of TSH hormone/ also, check cord at birth for thyroid hormone levels

What are some sequelae to the infant from Hyperthyroidism in mom during pregnancy?

• Hypothyroidism is associated with abnormal fetal development secondary to
• Poor placental maturation
• Spontaneous abortions
• Congenital defects
• Mental retardation
• Increased rate of stillbirths

What is Sick Euthroid Syndrome

An acutely Ill patient can present with depressed TSH and circulating Thyroid hormone.  This is due to altered cytokine levels

It is very common in hospital patients

You would expect depressed T3 but normal T4 and TSH

rT3 is elevated due to the impairment of converting T4 to T3 in the peripheral tissue and decreased clearence of rT3

Low total T4 but Free T4 is normal

Dont treat with replacement hormone(controversy)

What is the most common cause of Goiter outside of the US?

Iodine Deficiency

What causes a goiter?

Too much TSH or Too Sensitive to TSH

often that is due to no feedback from TH

so anything that decreases TH an lead to a goiter

What is the Evolution of the Non-toxic Goiter?

2 phases: Hyperplastic phase,  Colloid Involution phase

• In Nontoxic Diffuse Goiter, considered the early stages of the disease, the entire gland expands in size.
• Over time the diffuse enlargement will develop a nodularity creating a Nontoxic Multinodular Goiter.
• Occasionally the nontoxic multinodular goiters can become functional forming a Toxic Multinodular Goiter.

How do you test for a Non-toxic Goiter?

How do you treat it?

• Thyroid function tests
• Patients are euthyroid
• Generally T3 is normal but T4 can be low normal
• TSH can in the upper range of normal
• TPO antibodies can be useful
• Urinary iodine can be useful
• Ultrasound only if the presence of a nodule is in question

Treat with iodide or TH replacment.  Only remove if necessary due to untoward mass efects.

Under what cricumstances would you biopsy a goiter?

Only if you suspect a Neoplasm

Becasue Toxic Multinodular Goiters

are functional.... they present differently than non toxic goiters....

How do they present?

• Subclinical hyperthyroidism
• Atrial fibrillations
• Palpitations
• Tachycardia
• Nervousness
• Tremor
• Weight loss

What testing will you do/ what results will you expect to find in a patient whom you suspect has

Toxic Multinodular Goiter

And how would you treat?

• Thyroid function tests
• TSH levels are low
• T4 can be normal or slightly increased
• T3 is often elevated compared to T4
• Thyroid scan

• Antithyroid drugs
• Radioiodine
• Surgery if necessary and permissible

Histology of the Pancreas

The islets of Langerhans contain the endocrine portion of the pancreas. Each islet contains at least four different types of cells: alpha, beta, delta and gamma.

Anatomy of the Pancreas

The pancreas is located in the upper portion of the abdominal cavity on the posterior body wall of the lesser sac. It is a retroperitoneal organ surrounded by a thin, loosely organized capsule.

What is T1DM

Intolerance to glucose accompanied by hypoinsulinemia secondary to the autoimmune destruction of beta-cells in the islets of the pancreas results in the clinical presentation of type 1 diabetes mellitus (T1DM).

Autoimmune destruction of beta-cells in pancreas --> No insulin --> hyperglycemia/glucose intolerance

What are some possible Etiologies for the descruction of the Beta-cells of the pancreas in T1DM?

• Viruses
• Diet
• Nitrosamines (possibly)
• Environmental toxins
• Emotional and physical stress

If autoimune... it's called "insulinitis"

What percentage of Beta-cells need to be destroyed before you develop symptoms of T1DM?

80-85%

What Autoantibodies can be present in T1DM?

• Islet cell autoantibodies (ICA)
• Glutamic acid decarboxylase (GAD65)
• Insulin antibodies
• Antibodies ot tyrosine phosphatase

How does T1DM tend to Present?

• Clinical presentation can be sudden
• Typically a short history of weight loss, thirst and fatigue
• Ketoacidosis can be the presenting feature
• Polyuria and polydipsia

What tests will you order if you suspect T1DM?

• Fasting blood glucose
• Glycosylated hemoglobin
• Microalbumin testing in the urine
• Serum BUN and creatinine
• Serum potassium
• Urine glucose
• Urine ketones
• Lipid profile

Explain the BUN test

The most common cause of an elevated BUN, azotemia, is poor kidney function, 

A greatly elevated BUN (>60 mg/dL) generally indicates a moderate-to-severe degree of renal failure. Impaired renal excretion of urea may be due to temporary conditions such as dehydration or shock, or may be due to either acute or chronic disease of the kidneys themselves.

An elevated BUN in the setting of a relatively normal creatinine may reflect a physiological response to a relative decrease of blood flow to the kidney (as seen in heart failure or dehydration) without indicating any true injury to the kidney. However, an isolated elevation of BUN may also reflect excessive formation of urea without any compromise to the kidneys.

Increased production of urea is seen in cases of moderate or heavy bleeding in the upper gastrointestinal tract (e.g. from ulcers). The nitrogenous compounds from the blood are resorbed as they pass through the rest of the GI tract and then broken down to urea by the liver. Enhanced metabolism of proteins will also increase urea production, as may be seen with high protein diets, steroid use, burns, or fevers.

When the ratio of BUN to creatinine (BUN:Cr) is greater than 20, the patient is suspected of having prerenal azotemia. This means that the pathologic process is unlikely to be due to intrinsic kidney damage.

A low BUN usually has little significance, but its causes include liver problems, malnutrition (insufficient dietary protein), or excessive alcohol consumption. Overhydration from intravenous fluids can result in a low BUN. Normal changes in renal bloodflow during pregnancy will also lower BUN.

Urea itself is not toxic. This was demonstrated by Johnson et al. by adding large amounts of urea to the dialysate of hemodialysis patients for several months and finding no ill effects.^[1]. However, BUN is a marker for other nitrogenous waste. Thus, when renal failure leads to a buildup of urea and other nitrogenous wastesuremia), an individual may suffer neurological disturbances such as altered cognitive function (encephalopathy), impaired taste (dysgeusia) or loss of appetite (anorexia). The individual may also suffer from nausea and vomiting, or bleeding from dysfunctional platelets. Prolonged periods of severe uremia may result in the skin taking on a grey discolouration or even forming frank urea crystals ("uremic frost") on the skin.

Because multiple variables can interfere with the interpretation of a BUN value, GFR and creatinine clearance are more accurate markers of kidney function. Age, sex, and weight will alter the "normal" range for each individual, including race. In renal failure or chronic kidney disease (CKD), BUN will only be elevated outside "normal" when more than 60% of kidney cells are no longer functioning. Hence, more accurate measures of renal function are generally preferred to assess the clearance for purposes of medication dosing.

How do you treat T1DM?

Insuline therapy tailored to individual

What are the expected longterm management requirements and complications for someone who had T1DM

• Diabetic ketoacidosis
• Hyperosmolar nonketotic state
• Hypoglycemia with neuroglycopenia
• Co-morbidities
  • Hypertension
  • Hyperlipidemia
• Long-term complications include
  • Diabetic retinopathy
  • Diabetic neuropathy
  • Diabetic macro- and microvasculopathy
  • Diabetic nephropathy

What is T2DM?

Impaired tolerance to glucose and hyperinsulinemia secondary to the development of resistance to the action of insulin constitutes diabetes mellitus type 2 (T2DM)Eventual beta-cell stress and demise create a conversion from hyper- to hypoinsulinemia in some individuals.

90% of Diabetic pt's have this type

What testing would you order  If you suspected

T2DM?

• Fasting plasma glucose levels
• Glucose tolerance test
• Glycosylated hemoglobin levels
• Lipid profile
• Plasma urea, creatinine and electrolytes

Treatment for T2DM

• Initial treatment should include lifestyle changes such as exercise and diet
• Oral antidiabetic medication
• Insulin therapy if needed

What are the long term management issues and complications of T2DM?

Weight managment

other complications are same as T1DM

DM secondary to Genetic Defect

also called Mature onset diabetes of the young

disorder in the synthesis, handiling, excretion of insuline

Rare

S&S of T2DM present in the young people

usually Ketosis resistant

What is Gestational DM (GDM)?

Intolerance to glucose can manifest during pregnancy, where it is termed gestational diabetes (GDM).

• Normal increase in the physiologic levels of maternal insulin resistance during gestation stimulated in part by the increase in human chorionic somatomammotropin and estrogen
• Physiological increase in maternal insulin production
• Risk of perinatal loss and neonatal morbidity is increased when GDM went undetected
  • Fetal hyperinsulinemia
  • Increased fetal size (macrosomia)

T/F

women with GDM are more likely to develop T2DM later

true

How does GDM Present?

• Typically asymptomatic and detected by screening
• Can present as the initial onset of diabetes in an individual with unrecognized pregestational T2DM

How do  you test for GDM?

And How do you Treat?

• Risk assessment
• A screening test with a 50 gram oral glucose challenge with plasma glucose determinations
• Oral glucose tolerance test

• Diet
• Insulin therapy (controversial)
• Exercise (moderate only)

Monitor their Ketones and their blood glucose

What is Diabetic Ketoacidosis

Excessive elevation of plasma levels of glucose accompanied by depressed level of insulin and elevated levels of the counter-regulatory hormones can lead to the metabolic production of ketones creating ketoacidosis in the diabetic. DKA is an acute complication of hyperglycemia.

What is the Natural Hx of DKA?

It represents a huge portion of Diabetes related hospital admissions.

It is often the initial sign leading to diagnosis of DM type 1

Excess of free fatty acids  get converted to keytones in via the liver and then you just pee them out.

How does DKA present?

• Nausea and vomiting can be prominent features
• Severe abdominal pain
• Hypotension
• Hypreglycemia and glucosuria
• Hypovolemia
• Tachycardia
• Kussmaul respiration (deep labored breathing)
• Ketone breath
• Lethargy and CNS depression evolving into coma

They can get cerebral edema

How do you test for DKA?

• Plasma glucose level
• Plasma electrolytes (anion gap)
• Plasma chemistry, BUN and creatinine

What is Nonketotic Hyperosmotic State? (NKHS)

Hyperglycemia, insulin deficiency and reduced fluid intake can create a hyperosmotic plasma with serious consequences.NKHS is an acute complication of hyperglycemia.

can lead to Diabetic Coma

T/F

NKHS presents most frequently in T2DM in the elderly

true

What are some risk factors for developing NKHS?

• Infection
• Restriction or restraint of motion
• Extremes of age
• Excessive sedation
• Physiologic stress
• Polypharmacy
• Social isolation

Presentation of NKHS

• Severe hyperglycemia, hyperosmolarity and dehydration
• Relative absence of ketoacidosis (anion gap can be increased)
• Days to weeks of increasing polyuria and polydipsia
• Eventual hypovolemia, hypotension, organ hypoperfusion and tachycardia
• Depressed mental status can be present
• Heavy glucosuria with little to no ketouria or ketonemia
• Weight loss, weakness, visual disturbance and leg camps can also be present
• Physical exam finds profound dehydration, poor tissue tugor, soft sunken eyes, cool extremities and thready pulse
• Neurological signs such as seizure and hemiparesis occur in up to 19% of cases
• Coma is rare unless the osmolarity exceeds 320 mOsm/L

At what osmolarity in NKHS does coma become likely?

Coma is rare unless the osmolarity exceeds 320 mOsm/L

What testing would you get for  NKHS?

• Plasma glucose levels
• Electrolytes and the anion gap

How will you treat NKHS?

• Treat the underlying cause, such as infection
• Volume expander if hypotension is present
• Administer insulin if necessary
• Replace electrolytes

What are the complications of NKHS?

• Beware of cerebral edema
• Thromboembolic events
• Disseminated intravascular coagulation
• Pulmonary aspiration
• Rhabdomyolysis
• Pancreatitis

What is Secondary Hyperglycemia?

Hyperglycemia can develop secondarily in patients with other diseases such as pancreatitis due to the decreased production of insulin from the beta-cells in the islets.

• Fibrocalculous pancreatitis
• Chronic pancreatitis
• Pancreatic neoplasms
• Cystic fibrous
• Hemochromatosis
• Pancreatic trauma

Acute Complications of Hyperglycemia can be life threataning...  What are they?

Acute Complications:

• The two most important acute complications are (both are treated elsewhere as separate disease states):
  • Diabetic ketoacidosis (DKA)
  • Nonketotic Hyperosmolar State(NKHS)

Chronic Complications of Hyperglycemia can be extrememly debilitating.... What are they?

• Important chronic complications are:
  • Diabetic retinopathy
  • Diabetic nephropathy
  • Diabetic neuropathy
  • Diabetic gastroparesis, constipation and diarrhea
  • Diabetic genitourinary dysfunction
  • Diabetic cardiovascular disease
  • Lower extremity complications (Foot)
  • Diabetic infections

T/F

Long term complicaitons of DM regardless of how well you control your blood sugar Are inevitable.

True

Hypoglycemia

The defence of euglycemia is multifidus; however in disease states these defences can breakdown and hypoglycemia result.Three major types of hypoglycemia are recognized clinically:

• Postprandial or reactive hypoglycemia
• Fasting hypoglycemia
• Iatrogenic or diabetic hypoglycemia.

Who most comonly get hypoglycemia?

Hypoglycemia most commonly occurs during the course of treatment of patients with diabetes mellitus

What's the deal with Hypoglycemia in the New Born?

The brain of the newborn is the biggest source of glucose consumption in the body. Hypoglycemia can due permanent damage to the growth and development of the brain. The defence of euglycemia involves the autonomic nervous system and the hypothalamic-pituitary-adrenal axis to stimulate glycogenolysis and gluconeogenesis. A critical point occurs when the infant shifts from maternal sources of energy to its own supplies. Dysregulation of the protective axis that helps make this shift in energy sources can result in hypoglycemia.

What are the treatment approaches for

Hypoglycemia in the newborn?

• Acute hypoglycemia requires normalization of blood glucose levels, can be intravenous when necessary
• Persistent hyperinsulinemia
  • Pharmacological approach
    • Oral diazoxide
    • Somatostatin analogues
    • Calcium channel blockers
  • Surgical approach
    • Can require subtotal pancreatectomy

What are the major Etiologies of Hypoglycemia in the New Born

• Hyperinsulinemia
• Endocrine deficiency
  • Corticosteroids
  • Panhypopituitarism
• Substate limited
  • Ketotic hypoglycemia ***
• Glycogen storage diseases
• Disorders of gluconeogenesis

T/F 

The risk for hypoglycemia in the newborn is indirectly proportional with the birth weight.

True

What does Whipple's Triad indicate

and what is it?

Hypoglycemia

• Symptoms consistent with hypoglycemia
• Low plasma glucose concentration
• Relief of symptoms after plasma glucose is raised

What are the neurologic symptoms

What are the Neurogenic responses of

Hypoglycemia

• Neuroglycopenic symptoms
  • Behavioral changes
  • Confusion
  • Fatigue
  • Seizures
  • Loss of consciousness
• Neurogenic responses
  • Palpitations, tremor and anxiety
  • Sweating, hunger, and paresthesia

the most important structure for regulating blood calcium is the [blank]

parathyroid gland

What are the four organs/structures that control bood calcium levels and how do they do it?

Parathyroid gland secretes Parathyroid Hormone.  PTH stimulates osteoclasts to break down bone and increase blood calcium and it stimulated kidneys to make active vit D

Kidney -- site for vit D activation. the enzyme there (1 alpha hydroxylase converts 25 hydroxy Vit D to 125 hydroxy Vit D the active form)

Gut -- the active Vit D acts on the gut to increase calcium absorption

Bone --

What is the negative feed back mech for blood calcium?

If blood calcium gets too high, it directly inhibits the Parathyroid gland from secreting more PTH.

What is more likely to cause symptoms of hypercalcemia:

the degree of calcium elevation

or

the rapidity by which it increased

The speed.

slow gradual increases of Calcium tend not to be symptomatic

What is the normal level of calcium in the blood?

At what level can you start getting symptoms?

At what level are you at risk for becoming comatose or stuperose?

8.5-10.5 mg/dl

> 11 or 12 mg/dl

> 13 mg/dl

What types of symptoms does hyperCalcemia cause?

Neurologic

Cardiovascular

Gastrointestinal (most common)

Renal

What are the Neurologic symptoms of HyperCalcemia?

Lethergy, Confusion, Coma,

What are the Cardiovascular symptoms of Hypercalcemia?

Increases BP

Decreases HR

Shortens QT interval on EKG

What are the Gastrointestinal Effects of HyperCalcemia?

Anorexia

Nausea

Vomiting

Constipation

Pancreatitis

What are the Renal effects of HyperCalcemia?

Polyuria

damage via calcium deposits

Stones

What are the different etiologies of HyperCalcemia?

Primary Hyperparathyroidism (one overactive gland/adenoma)

Parathyroid Hyperplasia (primary hyperparathyroidism when all 4 glands are overactive)

Malignancy secreting Parathyroid related Protein

Paget's Dz (increased activity of osteoclasts)

Lymphoma or Granulomatous dz (increased 1alpha hydroxylase from lymphocytes leading to high active vit D and high absorption of Calcium

Familial Hyperuric hypercalcemia (kidneys are too sensitive to pth and they reabsorbe too much calcium) (usually not severe)

Thiazide diuretics (increase calcium reabsorption)

Bone destroying Cancer (causing release of calcium into blood)

HyperCalcemia of Immobility (increased break down of bone)

51 asymptomatic female

labs show:  Calcium (11.1), Phosphorous (2.3)

so high calcium low phosphorous

What is the most likely diagnosis?

Primary Hyperparathyroidism

this is the #1 cause of mild asymptomatic hypercalcemia in the general population

it causes increased calcium and decreased phosphorous (incresed excretion in kidneys)

it is the only etiology in where you will find high PTH

How will you confirm primary Hyper parathyroidism?

serum PTH: high

serum Phosphorous: low

Serum Calcium: high

Urine calcium: high (because there is just more of it even though more is being reabsorbed in the kidneys)

Alkalyn phosphatase: high (a marker for osteoblast activity which is high to combat increased osteoclast activity)

Urinary Cyclic AMP: high (permiability in kidneys increased by PTH)

radioactive imaging will show

What is the cause of primary hyper parathyroidism?

85% -- one abnormal PT gland --> high calcium --> feedback suppresion and shrinking of other 3 glands. it is a benign tumor... an Adenoma

Remove single gland

15% -- all glands are overfunctioning "parathyroid hyperplasia"

Remove all glands or use another treatment

When do you treat Primary Hyperparathyroidism?

(remember it is a Non-Progressive Dz)

When they are young (<50) [so that you don't have to follow up on them forever]

When they are symptomatic or have hx of symptoms

When their bone densitiy is greater than 2 standard deviations below normal

If Calcium > 12

If Urinary Calcium is high [risk for stone}

How can you use radioactive imaging to help diagnose wether you have one overactive PTgland or 4 overactive PTglands in primary hyperparathyroidism?

you can just have a look during surgery: hyperactive glands should be hypertrophied

radioactive subs will stay in over active glands longer so 2 images ... one where everything lights up and a second where the overactive gland still lights up

but... you should check PTH levels before and after surgury and they should fall by 80% if you got what you needed

Osteitisfibrosis cystica

phenomenon of primary hyperparathyroidism

where you have cysts in the bone where the mineral has leached out.

most common in hand, distal radius and ulna

72 year old woman with anorexia, nausea, vomiting, lethargy, confusion, low BP, low HR, short QT interval

Ca: 15.6, PH: 3.2, BUN and Creat suggest liver failure(from dehydration probably)

What do you do at this point?

Later... you get low PTH levels and R breast mass and high PTRP level

What is the diagnosis?

You don't spend time finding a cause... this is a Medical Emergency.

1st: Hydrate them with saline (this will also dilute the ca)

2nd: monitor electrolyes

3rd: give loop diuretic to increase ca excretion

4th: give drug to decrease osteoclastic activity.  could give dialysis

She has malignancy related hypercalcemia to to parathyroid related protein.

36 year old man SOB, nonproductive cough, hyalar lymphadenopathy, alveolar difusion problems

ca: 11

phos: 3.6

pth: low

what is your diagnosis and how is it treated?

Granulomatous dz (Sarcoidoisis)  leading to increased 1alpha hydroxylase activity and more 125 hydroxy vitamine D and too much calcium absorption from gut.

you can give them glucocorticoids which will inhibit macrophages and lymphocytes

What are the 3 main causes of HypoCalcemia

and what are their lab values for

PTH level

Phosphorous level

Hypoparathyroidism [low PTH, high PHO]

Resistance to PTH action or pseudo hypoparathyroidism [high PTH, high Phosphorous]

Vitamine D defficiency [high PTH, low phosphorous]

What are the etiologies of HypoCalcemia?

Hypoparathyroidism

Parathyroid agenisis

Defective PTH receptors

Low Magnesium [diarrhea, alcoholic,diabetic (magnesium importatn for pth action and secretion)

resistance to 1 alpha hydroxylase (so no active vit D)

Vitamine D defficiency

Renal failure (no proper conversion of vit D to active form)

What are the S&S of HypoCalcemia?

Iritation of Nervous system

carpal pedal spasms

seizures (ca level of 4 or5)

cataracts

wide QT interval

heart failure

megaloblastic anemia

Concept of Free calcium and correcting for protein

most calcium is bound to albumin but only free calcium is active

so, in situations where albumin is low or high you want to correct your total calcium reading so that you do not diagnose them with high or low calcium

free calcium wil increase in hypoalbuminemia and Acidosis

you can order an ionized calcium test

or you can correct it yourself (total calcium will decrease by 0.8 for each gram of decreased albumen) 

So if you had a total ca of 10 and an albumin of 4g and your albumin decreased to 3 grams your total calcium reading would show 9.2 even though free calcium would be normal so you would add 0.8 to correct the calcium reading for the protein

How do you treat Hypocalcemia?

You give them 2-3 grams of Calcium per day with a goal of keeping their calcium level at 8.5-9.5 so that they don't make stones

calcium carbonate is 40/60 calcium/carbonate so you have to give them like 8g of calcium carbonate/day to get the dose of calcium you desire.

you don't want them to be peeing out > 400 units of ca

you can give them calcitiol???  active vitamine D

woman in 20's with short stature and shorter 4th and 5th digits, round face, and hx of seizures

low Ca and High Phosphorous

high PTH

whats your diagnosis?

Pseudohypoparathyroidism

What is pseudo pseudo hypoparathyroidism?

form of pseudo hypoparathyroidism in which the kidney still responds properly to PTH so they have normal levels of calcium but they still have the phenotypic effects due their bone not responding to PTH

interestingly:

when mom passes on Pseudohypoparathyroid kids will get pseudohypoparathyroid

when Dad passes on pseudohypoparathyroid, kids will get Psudopseudohypoparathyroid

MEN 1

(remeber that this is a better dz than MEN 2 becasue the neoplasms are benign)

Parathyroid

[parathyroid hyperplasia]

Pituitary

[prolactinoma most common]

Pancreatic

[mostly gastrinomas (zollinger-ellison syndrome of multiple refractory peptic ulcers in atypical locations) or Insulinomas]

MEN 2

malignant

Parathyroid

[hyperplasia ... this is rarer feature of men2]

Thyroid Tumor

[medulary thyroid tumor of C-cells]

Pheochromocytoma

[adrenal medulary tumor]

Type A -- look normal but they have the 3 things (extracellular domain is affected)

Type B -- have typical physical paearance with oral tumors long legs, more aggresive thyroid tumor. (intracellular domain affected)

Familial Medullary cancer (only have the thyroid cancer

High calcitonin: diarrhea, flushing and more

PGA (polyglandular autoimmune syndrome)

Type 1

Starts in kids

mucocutaneous candidiasis

hypopituitarism

Adrenal insuficiency

(plus: nail problems teeth problems, low calc highphosph, low cortisol, low sugar

AIRE gene chrom 21

PGA (polyglandular autoimmune syndrome)

Type 2

Starts as Adult

autoimmune destruction of glands

adrenal insufficiency (addison's dz)

Hypothyroid (hashimoto's)

Diabetes

plus; viteleigo...