Hyperaldosteronism Primary Definition Treatment Secondary Definition Treatment

Primary Definition Caused by adrenal hyperplasia or an aldosterone-secreting adrenal adenoma (Conn syndrome), resulting in hypertension, hypokalemia, metabolic alkalosis, and low plasma renin. Normal Na+ due to aldosterone escape = no edema due to aldosterone escape mechanism. May be bilateral or unilateral. Treatment: Surgery to remove the tumor and/or spironolactone, a K+-sparing diuretic that acts as an aldosterone antagonist. Secondary Definition Renal perception of low intravascular volume results in an overactive renin-angiotensin system. Due to renal artery stenosis, CHF, cirrhosis, or nephrotic syndrome. Associated with high plasma renin. Treatment Spironolactone.

Hypothyroidism vs. hyperthyroidism Signs / symptoms Heat production Weight / appetite Activity GI Reflexes Myxedema Skin / hair Cardio / pulm Lab findings TSH T3 / T4 Cholesterolemia

Signs / symptoms Heat production _Hypo_: Cold intolerance (decreased heat production) _Hyper_: Heat intolerance (increased heat production) Weight / appetite _Hypo_: Weight gain, decreased appetite _Hyper_: Weight loss, increased appetite Activity _Hypo_: Hypoactivity, lethargy, fatigue, weakness _Hyper_: Hyperactivity GI _Hypo_: Constipation _Hyper_: Diarrhea Reflexes _Hypo_: decreased reflexes _Hyper_: increased reflexes Myxedema _Hypo_: Myxedema (facial/periorbital) _Hyper_: Pretibial myxedema (Graves disease), periorbital edema Skin / hair _Hypo_: Dry, cool skin; coarse, brittle hair _Hyper_: Warm, moist skin; fine hair Cardio / pulm _Hypo_: Bradycardia, dyspnea on exertion _Hyper_: Chest pain, palpitations, arrhythmias, increased number and sensitivity of β-adrenergic receptors Lab findings TSH _Hypo_: increased TSH (sensitive test for 1° hypothyroidism) _Hyper_: decreased TSH (if 1°) T3 / T4 _Hypo_: decreased free T3 and T4 _Hyper_: increased free or total T3 and T4 Cholesterolemia _Hypo_: Hypercholesterolemia (due to decreased LDL receptor expression) _Hyper_: Hypocholesterolemia (due to increased LDL receptor expression)

Subacute thyroiditis (de Quervain) Definition Histology Findings

Definition Self-limited hypothyroidism often following a flu-like illness. May be hyperthyroid early in course. Histology Granulomatous inflammation. Findings Increased ESR, jaw pain, early inflammation, very tender thyroid. de Quer_vain_ is associated with _pain_.

Riedel thyroiditis Definition Findings

Definition Form of hypothyroidism Thyroid replaced by fibrous tissue (hypothyroid). Fibrosis may extend to local structures (e.g., airway), mimicking anaplastic carcinoma. Considered a manifestation of IgG4-related systemic disease. Findings Fixed, hard (rock-like), and painless goiter.

Thyroid storm Definition Findings Treatment

Definition Stress-induced catecholamine surge seen as a serious complication of Graves disease and other hyperthyroid disorders. Findings Presents with agitation, delirium, fever, diarrhea, coma, and tachyarrhythmia (cause of death). May see increased ALP due to increased bone turnover. Treatment: _3_ _P_'s β-blockers (e.g., _P_ropranolol) _P_ropylthiouracil Corticosteroids (e.g., _P_rednisolone).

Endocrine - Pathology Flashcards by Heather Acuff

Cushing syndrome (317)

Etiology
Findings
Diagnosis
- Screening tests include:
- Measure/
- Ectopic secretion

Etiology
- Increased cortisol due to a variety of causes:
  - Exogenous corticosteroids
    - # 1 cause
    - Results in decreased ACTH, bilateral adrenal atrophy.
  - Primary adrenal adenoma, hyperplasia, or carcinoma
    - Results in decreased ACTH, atrophy of uninvolved adrenal gland.
    - Can also present as 1° aldosteronism (Conn syndrome).
  - ACTH-secreting pituitary adenoma [A] (Cushing disease)
    - Paraneoplastic ACTH secretion (e.g., small cell lung cancer, bronchial carcinoids)
    - Results in increased ACTH, bilateral adrenal hyperplasia.
    - Cushing disease is responsible for the majority of endogenous cases of Cushing syndrome.
Findings
- Hypertension, weight gain, moon facies, truncal obesity [B], buffalo hump, hyperglycemia (insulin resistance), skin changes (thinning, striae), osteoporosis, amenorrhea, and immune suppression.
Diagnosis
- Screening tests include: increased free cortisol on 24-hr urinalysis, midnight salivary cortisol, and overnight low-dose dexamethasone suppression test:
- Measure serum ACTH.
  - If decreased, suspect adrenal tumor.
  - If increased, distinguish between Cushing disease and ectopic ACTH secretion with a high-dose (8 mg) dexamethasone suppression test and CRH stimulation test.
- Ectopic secretion
  - Will not decrease with dexamethasone because the source is resistant to negative feedback
  - Will not increase with CRH because pituitary ACTH is suppressed.

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Hyperaldosteronism

Primary
- Definition
- Treatment
Secondary
- Definition
- Treatment

Primary
- Definition
  - Caused by adrenal hyperplasia or an aldosterone-secreting adrenal adenoma (Conn syndrome), resulting in hypertension, hypokalemia, metabolic alkalosis, and **low **plasma renin.
    - Normal Na+ due to aldosterone escape = no edema due to aldosterone escape mechanism.
  - May be bilateral or unilateral.
- Treatment:
  - Surgery to remove the tumor and/or spironolactone, a K+-sparing diuretic that acts as an aldosterone antagonist.
Secondary
- Definition
  - Renal perception of low intravascular volume results in an overactive renin-angiotensin system.
  - Due to renal artery stenosis, CHF, cirrhosis, or nephrotic syndrome.
  - Associated with high plasma renin.
- Treatment
  - Spironolactone.

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Addison disease

Definition
Characterized by/
1° vs. 2°

Definition
- Chronic 1° adrenal insufficiency due to adrenal atrophy or destruction by disease (e.g., autoimmune, TB, metastasis).
- Deficiency of aldosterone and cortisol, causing hypotension (hyponatremic volume contraction), hyperkalemia, acidosis, and skin and mucosal hyperpigmentation [A] (due to MSH, a by-product of increased ACTH production from pro-opiomelanocortin (POMC).
Characterized by Adrenal Atrophy and Absence of hormone production
- Involves All 3 cortical divisions (spares medulla).
Distinguish from 2° adrenal insufficiency (decreased pituitary ACTH production), which has no skin/mucosal hyperpigmentation and no hyperkalemia.

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Waterhouse- Friderichsen syndrome

Acute 1° adrenal insufficiency due to adrenal hemorrhage
Associated with Neisseria meningitidis septicemia, DIC, and endotoxic shock.

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Neuroblastoma

Definition
Source
Most common presentation
Labs

Definition
- The most common tumor of the adrenal medulla in children, usually < 4 years old.
Source
- Originates from neural crest cells [A].
- Occurs anywhere along the sympathetic chain.
Most common presentation
- Abdominal distension
- A firm, irregular mass [B] that can cross the midline (vs. Wilms tumor, which is smooth and unilateral).
- Less likely to develop hypertension.
- Associated with overexpression of N-myc oncogene.
Labs
- Homovanillic acid (HVA), a breakdown product of dopamine, increased in urine.
- Bombesin (+).

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Pheochromocytoma (319)

Etiology
Symptoms
Findings
Treatment

Etiology
- Most common tumor of the adrenal medulla in adults [A] [B].
- Derived from chromaffin cells (arise from neural crest) [C].
- Rule of 10’s:
  - 10% malignant –> 90% benign
  - 10% bilateral –> 90% unilateral
  - 10% extra-adrenal –> 90% in adrenal medulla
  - 10% calcify –> 90% don’t calcify
  - 10% kids –> 90% adults
Symptoms
- Most tumors secrete epinephrine, norepinephrine, and dopamine, which can cause episodic hypertension.
- Associated with von Hippel-Lindau disease, MEN 2A and 2B.
- Symptoms occur in “spells”—relapse and remit.
- Episodic hyperadrenergic symptoms (5 P’s):
  - Pressure (increased BP)
  - Pain (headache)
  - Perspiration
  - Palpitations (tachycardia)
  - Pallor
Findings
- Urinary VMA (a breakdown product of norepinephrine and epinephrine) and plasma catecholamines are increased.
Treatment
- Irreversible α-antagonists (phenoxybenzamine) and β-blockers followed by tumor resection.
- α-blockade must be achieved before giving β-blockers to avoid a hypertensive crisis.

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Hypothyroidism vs. hyperthyroidism

Signs / symptoms
- Heat production
- Weight / appetite
- Activity
- GI
- Reflexes
- Myxedema
- Skin / hair
- Cardio / pulm
Lab findings
- TSH
- T3 / T4
- Cholesterolemia

Signs / symptoms
- Heat production
  - Hypo: Cold intolerance (decreased heat production)
  - Hyper: Heat intolerance (increased heat production)
- Weight / appetite
  - Hypo: Weight gain, decreased appetite
  - Hyper: Weight loss, increased appetite
- Activity
  - Hypo: Hypoactivity, lethargy, fatigue, weakness
  - Hyper: Hyperactivity
- GI
  - Hypo: Constipation
  - Hyper: Diarrhea
- Reflexes
  - Hypo: decreased reflexes
  - Hyper: increased reflexes
- Myxedema
  - Hypo: Myxedema (facial/periorbital)
  - Hyper: Pretibial myxedema (Graves disease), periorbital edema
- Skin / hair
  - Hypo: Dry, cool skin; coarse, brittle hair
  - Hyper: Warm, moist skin; fine hair
- Cardio / pulm
  - Hypo: Bradycardia, dyspnea on exertion
  - Hyper: Chest pain, palpitations, arrhythmias, increased number and sensitivity of β-adrenergic receptors
Lab findings
- TSH
  - Hypo: increased TSH (sensitive test for 1° hypothyroidism)
  - Hyper: decreased TSH (if 1°)
- T3 / T4
  - Hypo: decreased free T3 and T4
  - Hyper: increased free or total T3 and T4
- Cholesterolemia
  - Hypo: Hypercholesterolemia (due to decreased LDL receptor expression)
  - Hyper: Hypocholesterolemia (due to increased LDL receptor expression)

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Hashimoto thyroiditis

Definition
Histologic findings
Findings

Definition
- Most common cause of hypothyroidism in iodine-sufficient regions
- An autoimmune disorder (anti-thyroid peroxidase, antithyroglobulin antibodies).
- Associated with HLA-DR5.
- Increased risk of non-Hodgkin lymphoma.
- May be hyperthyroid early in course due to thyrotoxicosis during follicular rupture.
Histologic findings
- Hürthle cells, lymphoid aggregate with germinal centers [A].
Findings
- Moderately enlarged, nontender thyroid.

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Congenital hypothyroidism (cretinism)

Definition
Findings

Definition
- Severe fetal hypothyroidism due to maternal hypothyroidism, thyroid agenesis, thyroid dysgenesis (most common cause in U.S.), iodine deficiency, dyshormonogenic goiter.
Findings: 6 P’s [B] [C]
- Pot-bellied
- Pale
- Puffy-faced child with Protruding umbilicus
- Protuberant tongue
- Poor brain development

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Subacute thyroiditis (de Quervain)

Definition
Histology
Findings

Definition
- Self-limited hypothyroidism often following a flu-like illness.
- May be hyperthyroid early in course.
Histology
- Granulomatous inflammation.
Findings
- Increased ESR, jaw pain, early inflammation, very tender thyroid.
- de Quervain** is associated with pain.**

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Riedel thyroiditis

Definition
Findings

Definition
- Form of hypothyroidism
- Thyroid replaced by fibrous tissue (hypothyroid).
- Fibrosis may extend to local structures (e.g., airway), mimicking anaplastic carcinoma.
- Considered a manifestation of IgG4-related systemic disease.
Findings
- Fixed, hard (rock-like), and painless goiter.

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Causes of hypothyroidism

Most important
Other

Most important
- Hashimoto thyroiditis
- Congenital hypothyroidism (cretinism)
- Subacute thyroiditis (de Quervain)
- Riedel thyroiditis
Other
- Iodine deficiency [D]
- Goitrogens
- Wolff-Chaikoff effect
- Painless thyroiditis.

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Toxic multinodular goiter

Definition
Jod-Basedow phenomenon

Definition
- Hyperthyroid condition
- Focal patches of hyperfunctioning follicular cells working independently of TSH due to mutation in TSH receptor [A].
- Increased release of T3 and T4.
- Hot nodules are rarely malignant.
Jod-Basedow phenomenon
- Thyrotoxicosis if a patient with iodine deficiency goiter is made iodine replete.

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Graves disease

Definition
Findings

Definition
- Most common cause of hyperthyroidism.
- Often presents during stress (e.g., childbirth).
Findings
- Autoantibodies (IgG) stimulate TSH receptors on thyroid (hyperthyroidism, diffuse goiter)
- Retro-orbital fibroblasts (exophthalmos: proptosis, extraocular muscle swelling [B])
- Dermal fibroblasts (pretibial myxedema).

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Thyroid storm

Definition
Findings
Treatment

Definition
- Stress-induced catecholamine surge seen as a serious complication of Graves disease and other hyperthyroid disorders.
Findings
- Presents with agitation, delirium, fever, diarrhea, coma, and tachyarrhythmia (cause of death).
- May see increased ALP due to increased bone turnover.
Treatment: 3 P’s
- β-blockers (e.g., Propranolol)
- Propylthiouracil
- Corticosteroids (e.g., Prednisolone).

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Thyroid cancer (323)

Treatment
Complications of surgery
Papillary carcinoma
Follicular carcinoma
Medullary carcinoma
Undifferentiated / anaplastic carcinoma
Lymphoma

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Treatment
- Thyroidectomy is treatment option for thyroid cancers and hyperthyroidism.
Complications of surgery
- Hoarseness (due to recurrent laryngeal nerve damage)
- Hypocalcemia (due to removal of parathyroid glands)
- Transection of inferior thyroid artery.
Papillary carcinoma
- Most common, excellent prognosis.
- Empty-appearing nuclei (“Orphan Annie” eyes) [A], psammoma bodies, nuclear grooves.
- Increased risk with RET and BRAF mutations, childhood irradiation.
Follicular carcinoma
- Good prognosis
- Invades thyroid capsule (unlike follicular adenoma)
- Uniform follicles.
Medullary carcinoma
- From parafollicular “C cells”
- Produces calcitonin, sheets of cells in an amyloid stroma [B].
- Associated with MEN 2A and 2B (RET mutations).
Undifferentiated / anaplastic carcinoma
- Older patients
- Invades local structures, very poor prognosis.
Lymphoma
- Associated with Hashimoto thyroiditis.

Primary hyperparathyroidism

Definition
Osteitis fibrosa cystica

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Definition
- Usually an adenoma.
- Hypercalcemia, hypercalciuria (renal stones), hypophosphatemia, increased PTH, increased ALP, increased cAMP in urine.
- Most often asymptomatic.
- May present with weakness and constipation (“groans”), abdominal/flank pain (kidney stones, acute pancreatitis), depression (“psychiatric overtones”).
Osteitis fibrosa cystica
- Cystic bone spaces filled with brown fibrous tissue [A] (bone pain).
“Stones, bones, groans, and psychiatric overtones.”

Hyperparathyroidism

Secondary hyperparathyroidism
Tertiary hyperparathyroidism
Renal osteodystrophy

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Secondary
- 2° hyperplasia due to decreased gut Ca2+ absorption and increased PO₄^3-, most often in chronic renal disease (causes hypovitaminosis D –> decreased Ca2+ absorption).
- Hypocalcemia, hyperphosphatemia in chronic renal failure (vs. hypophosphatemia with most other causes), increased ALP, increased PTH.
Tertiary
- Refractory (autonomous) hyperparathyroidism resulting from chronic renal disease.
- Really increased PTH, increased Ca2+.
Renal osteodystrophy
- Bone lesions due to 2° or 3° hyperparathyroidism due in turn to renal disease.

Hypoparathyroidism

Due to:
Findings
Chvostek sign
Trousseau sign
Pseudohypoparathyroidism (Albright hereditary osteodystrophy)

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Due to:
- Accidental surgical excision of parathyroid glands
- Autoimmune destruction
- DiGeorge syndrome.
Findings
- Hypocalcemia, tetany.
Chvostek sign
- Tapping of facial nerve (tap the Cheek) –> contraction of facial muscles.
Trousseau sign
- Occlusion of brachial artery with BP cuff (cuff the Triceps) –> carpal spasm.
Pseudohypoparathyroidism (Albright hereditary osteodystrophy)
- Autosomal dominant unresponsiveness of kidney to PTH.
- Hypocalcemia, shortened 4th/5th digits, short stature.

Pituitary adenoma

Definition
Nonfunctional vs. functional tumors
Treatment for prolactinoma

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Definition
- Most commonly prolactinoma (benign).
- Adenoma [A] may be functional (hormone producing) or nonfunctional (silent).
Nonfunctional vs. functional tumors
- Nonfunctional tumors present with mass effect (bitemporal hemianopia, hypopituitarism, headache).
- Functional tumor presentation is based on the hormone produced (e.g., prolactinoma: amenorrhea, galactorrhea, low libido, infertility; somatotropic adenoma: acromegaly).
Treatment for prolactinoma
- Dopamine agonists (bromocriptine or cabergoline). .

Acromegaly

Definition
Findings
Diagnosis
Treatment

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Definition
- Excess GH in adults.
- Typically caused by pituitary adenoma.
Findings
- Large tongue with deep furrows, deep voice, large hands and feet, coarse facial features [A], impaired glucose tolerance (insulin resistance).
- Increased GH in children –> gigantism (increased linear bone growth).
- Cardiac failure most common cause of death.
Diagnosis
- Increased serum IGF-1
- Failure to suppress serum GH following oral glucose tolerance test
- Pituitary mass seen on brain MRI.
Treatment
- Pituitary adenoma resection.
- If not cured, treat with octreotide (somatostatin analog) or pegvisomant (growth hormone receptor antagonist).

Diabetes insipidus

Definition
Nephrogenic DI
- Etiology
- Findings
- Diagnosis
- Treatment

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Definition
- Characterized by intense thirst and polyuria with inability to concentrate urine due to lack of ADH.
- Has a central or nephrogenic cause.
Nephrogenic DI
- Etiology
  - Hereditary (ADH receptor mutation)
  - 2° to hypercalcemia, lithium, demeclocycline (ADH antagonist)
- Findings
  - Normal ADH levels
  - Urine specific gravity < 1.006
  - Serum osmolarity > 290 mOsm/L
  - Hyperosmotic volume contraction
- Diagnosis
  - Water restriction test: no change in urine osmolarity
- Treatment
  - HCTZ, indomethacin, amiloride
  - Hydration

Diabetes insipidus

Central DI
- Etiology
- Findings
- Diagnosis
- Treatment

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Central DI
- Etiology
  - Pituitary tumor, autoimmune, trauma, surgery, ischemic encephalopathy, idiopathic
- Findings
  - Decreased ADH
  - Urine specific gravity < 1.006
  - Serum osmolarity > 290 mOsm/L
  - Hyperosmotic volume contraction
- Diagnosis
  - Water restriction test: > 50% increase in urine osmolarity
  - No water intake for 2–3 hr followed by hourly measurements of urine volume and osmolarity and plasma Na+ concentration and osmolarity.
  - DDAVP (ADH analog) is administered if normal values are not clearly reached.
- Treatment
  - Intranasal DDAVP
  - Hydration

SIADH

Definition
Causes include:
Treatment

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Definition
- Syndrome of inappropriate antidiuretic hormone secretion:
  - Excessive water retention
  - Hyponatremia with continued urinary Na+ excretion
  - Urine osmolarity > serum osmolarity
- Body responds to water retention with decreased aldosterone (hyponatremia) to maintain near-normal volume status.
  - Very low serum sodium levels can lead to cerebral edema, seizures.
  - Correct slowly to prevent central pontine myelinolysis.
Causes include:
- Ectopic ADH (small cell lung cancer)
- CNS disorders/head trauma
- Pulmonary disease
- Drugs (e.g., cyclophosphamide)
Treatment
- Fluid restriction, IV hypertonic saline, conivaptan, tolvaptan, demeclocycline.

Hypopituitarism * Definition * Due to: * Treatment

* Definition * Undersecretion of pituitary hormones * Due to: * Nonsecreting pituitary adenoma, craniopharyngioma * **Sheehan syndrome** * Ischemic infarct of pituitary following postpartum bleeding * Usually presents with failure to lactate * **Empty sella syndrome** * Atrophy or compression of pituitary * Often idiopathic * Common in obese women * Brain injury, hemorrhage (pituitary apoplexy) * Radiation * Treatment: * Hormone replacement therapy (corticosteroids, thyroxine, sex steroids, human growth hormone).

Diabetes mellitus * Acute manifestations * Chronic manifestations * Nonenzymatic glycosylation * Small vessel * Large vessel * Osmotic damage * Tests

* Acute manifestations * Polydipsia, polyuria, polyphagia, weight loss, DKA (type 1), hyperosmolar coma (type 2). * Rarely, can be caused by unopposed secretion of GH and epinephrine. * Chronic manifestations * Nonenzymatic glycosylation: * Small vessel disease (diffuse thickening of basement membrane) * --\> retinopathy (hemorrhage, exudates, microaneurysms, vessel proliferation) [A], glaucoma, nephropathy (nodular sclerosis, progressive proteinuria, chronic renal failure, arteriolosclerosis leading to hypertension, Kimmelstiel-Wilson nodules) * Large vessel atherosclerosis, CAD, peripheral vascular occlusive disease, and gangrene * --\> limb loss, cerebrovascular disease. * MI most common cause of death. * Osmotic damage * Sorbitol accumulation in organs with aldose reductase and decreased or absent sorbitol dehydrogenase * Neuropathy (motor, sensory, and autonomic degeneration) * Cataracts * Tests * Fasting serum glucose, oral glucose tolerance test, HbA1c (reflects average blood glucose over prior 3 months).

Type 1 vs. type 2 diabetes mellitus * 1° defect * Insulin necessary in treatment? * Age * Association with obesity? * Genetic predisposition * Association with HLA system? * Glucose intolerance * Insulin sensitivity * Ketoacidosis * β-cell numbers in the islets * Serum insulin level * Classic symptoms of polyuria, polydipsia, polyphagia, weight loss * Histology

* 1° defect * _1_: Autoimmune destruction of β cells * _2_: Increased resistance to insulin, progressive pancreatic β-cell failure * Insulin necessary in treatment? * _1_: Always * _2_: Sometimes * Age (exceptions commonly occur) * _1_: \< 30 yr * _2_: \> 40 yr * Association with obesity? * _1_: No * _2_: Yes * Genetic predisposition * _1_: Relatively weak (50% concordance in identical twins), polygenic * _2_: Relatively strong (90% concordance in identical twins), polygenic * Association with HLA system? * _1_: Yes (HLA-DR3 and 4) * _2_: No * Glucose intolerance * _1_: Severe * _2_: Mild to moderate * Insulin sensitivity * _1_: High * _2_: Low * Ketoacidosis * _1_: Common * _2_: Rare * β-cell numbers in the islets * _1_: Decreased * _2_: Variable (with amyloid deposits) * Serum insulin level * _1_: Decreased * _2_: Variable * Classic symptoms of polyuria, polydipsia, polyphagia, weight loss * _1_: Common * _2_: Sometimes * Histology * _1_: Islet leukocytic infiltrate * _2_: Islet amyloid polypeptide (IAPP) deposits

Diabetic ketoacidosis * Definition * Signs / symptoms * Labs * Complications * Treatment

* Definition * One of the most important complications of diabetes (usually type 1). * Usually due to increased insulin requirements from increased stress (e.g., infection). * Excess fat breakdown and increased ketogenesis from increased free fatty acids, which are then made into ketone bodies (β-hydroxybutyrate \> acetoacetate). * Signs / symptoms * Kussmaul respirations (rapid/deep breathing), nausea/vomiting, abdominal pain, psychosis/ delirium, dehydration. * Fruity breath odor (due to exhaled acetone). * Labs * Hyperglycemia, increased H+, decreased HCO3- (anion gap metabolic acidosis), increased blood ketone levels, leukocytosis. * Hyperkalemia, but depleted intracellular K+ due to transcellular shift from decreased insulin. * Complications * Life-threatening mucormycosis (usually caused by Rhizopus infection), cerebral edema, cardiac arrhythmias, heart failure. * Treatment * IV fluids, IV insulin, and K+ (to replete intracellular stores) * Glucose if necessary to prevent hypoglycemia.

Insulinoma * Definition * Symptoms * Treatment

* Definition * Tumor of β cells of pancreas * --\> overproduction of insulin * --\> hypoglycemia. * Symptoms * Whipple triad of episodic CNS symptoms: lethargy, syncope, and diplopia. * Symptomatic patients have decreased blood glucose and increased C-peptide levels (vs. exogenous insulin use). * Treatment * Surgical resection.

Carcinoid syndrome * Definition * Caused by... * Symptoms * Labs * Treatment

* Definition * Rare syndrome * Most common malignancy in the small intestine. * Not seen if tumor is limited to GI tract (5-HT undergoes first-pass metabolism in liver. * **Rule of _1/3_s:** * **_1/3_** metastasize * **_1/3_** present with 2nd malignancy * **_1/3_** are multiple * Caused by carcinoid tumors (neuroendocrine cells), especially metastatic small bowel tumors, which secrete high levels of serotonin (5-HT). * Symptoms * Recurrent diarrhea, cutaneous flushing, asthmatic wheezing, and right-sided valvular disease. * Labs * Increased 5-hydroxyindoleacetic acid (5-HIAA) in urine, niacin deficiency (pellagra). * Treatment * Resection, somatostatin analog (e.g., octreotide).

Zollinger-Ellison syndrome * Definition * Symptoms

* Definition * Gastrin-secreting tumor of pancreas or duodenum. * May be associated with MEN 1. * Symptoms * Acid hypersecretion causes recurrent ulcers in distal duodenum and jejunum. * Presents with abdominal pain (peptic ulcer disease, distal ulcers), diarrhea (malabsorption).

``` Multiple endocrine neoplasias: MEN 1 (Wermer syndrome) ``` * Characteristics * Comments

* Characteristics * Parathyroid tumors * Pituitary tumors (prolactin or GH) * Pancreatic endocrine tumors—Zollinger-Ellison syndrome, insulinomas, VIPomas, glucagonomas (rare) * Commonly presents with kidney stones and stomach ulcers * Comments * All **_MEN_** syndromes have autosomal **_dominant_** inheritance. * “**All _MEN_ are _dominant_**” (or so they think). * **MEN 1 = _3_ _P_’s** * From cephalad to caudad: **_P_**ituitary, **_P_**arathyroid, and **_P_**ancreas * Remember by drawing a diamond

``` Multiple endocrine neoplasias: MEN 2A (Sipple syndrome) ``` * Characteristics * Comments

* Characteristics * Medullary thyroid carcinoma (secretes calcitonin) * Pheochromocytoma * Parathyroid hyperplasia * Associated with ret gene mutation * Comments * All **_MEN_** syndromes have autosomal **_dominant_** inheritance. * “**All _MEN_ are _dominant_**” (or so they think). * **MEN 2A = _2_ _P_’s** * **_P_**arathyroids and **_P_**heochromocytoma * Remember by drawing a square

Multiple endocrine neoplasias: MEN 2B * Characteristics * Comments

* Characteristics * Medullary thyroid carcinoma (secretes calcitonin) * Pheochromocytoma * Oral/intestinal ganglioneuromatosis (mucosal neuromas). * Associated with marfanoid habitus. * Associated with ret gene mutation * Comments * All **_MEN_** syndromes have autosomal **_dominant_** inheritance. * “**All _MEN_ are _dominant_**” (or so they think). * **MEN 2B = _1_ _P_** * **_P_**heochromocytoma * Remember by drawing a triangle

Endocrine - Pathology Flashcards

(34 cards)