Kinder CIS Flashcards by Briana Tillman

Secondary Hypertension causes

Renal parenchymal disease
Renal artery stenosis
Obstructive sleep apnea
Endocrine:
- Hyperaldosteronism
- Thyroid disease
- Cushing disease
Pheochromocytoma
Coarctation of the aorta
CNS (Brain Tumor)
Medications (NSAID)
Polycythemia
Pre-eclampsia
Hypercalcemia (renal dysfunction and vasoconstriction)

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what is essential hypertension?

idiopathic

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Primary Aldosteronism – Who to screen

Blood pressure ≥ 150/100 mm Hg on 3 separate occasions
Drug Resistant Hypertension
BP > 140/90 mm Hg that is resistant to 3 medications including a diuretic
Controlled blood pressure on 4 medications
Hypertension and hypokalemia
Hypertension with an adrenal incidentaloma
Hypertension and sleep apnea
Hypertension with a family history of early onset hypertension or stroke age under 40
Hypertension and first-degree relative with primary aldosteronism

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testing aldosteronism- Test Preparation

Correct hypovolemia and hypokalemia
Stop spironolactone, eplerenone, amiloride, potassium wasting diuretics, licorice, and chewing tobacco at least one month before testing
Patient should be ambulatory and sitting upright with a morning sample
Substitute antihypertensives:
Verapamil, hydralazine, and doxazosin

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Etiologies of Primary Hyperaldosteronism

Unilateral aldosterone-producing adenoma ≅ 50%
Bilateral Adrenal Hyperplasia ≅ 50%

Rarer:
Inherited glucocorticoid-remedial aldosteronism
- ACTH instead of angiotensin II regulated
- Suppressed by low dose glucocorticoids
Unilateral adrenal hyperplasia
Bilateral aldosterone producing adenomas
Adrenocortical carcinoma

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tests to confirm primary hyperaldosteronism

Oral Salt Loading Test – most common test
Saline Suppression Test
Fludrocortisone Suppression Test

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Oral Salt Loading Test

6 gm salt per day for 3 days
Maintain normokalemia
24-hour urinary aldosterone > 12 mcg/day suggestive of primary aldosteronism

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Saline Suppression Test

2 liters normal saline infusion over 4 hours

Plasma aldosterone ≥ 10 ng/dL confirms primary aldosteronism

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Fludrocortisone Suppression Test

Fludrocortisone 0.1 mg po q 6 hours for 4 days
NaCl 1800 mg po tid, High Salt Diet, Supplemental Potassium
Plasma aldosterone > 6 ng/dL day 4 consistent with primary aldosteronism

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Adrenal Venous Sampling

Best test to determine if small adenoma on CT is secreting aldosterone
Differentiate between bilateral or unilateral hyperplasia

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treating adrenal hyperplasia

Treat hypertension and hypokalemia with spironolactone and oral potassium supplementation

Unilateral laparoscopic adrenalectomy for unilateral adrenal hyperplasia or unilateral aldosterone producing adenoma

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11-β-hydroxysteroid dehydrogenase 2

Enzyme expressed in aldosterone-selective epithelial tissues such as kidney, colon, salivary, and sweat glands.
Oxidizes cortisol to the inactive metabolite cortisone preventing illicit activation of the mineralcorticoid receptor.
Glycyrrhizinic acid in black licorice inactivates 11-β-HSD 2 causing pseuodohyperaldosteronism
Syndrome of apparerent mineralocorticoid excess is caused by a genetically inherited deficiency of 11-β-HSD 2

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Liddle Syndrome

Autosomal Dominant
Dysregulation of epithelial sodium channel (ENaC) due to a genetic mutation that causes the channels to no longer be degraded appropriately
High number of channels present in the collecting ducts lead to pseudohyperaldosteronism (↓PRA, ↓PAC)

Treatment is with potassium sparing diuretics

Amiloride
Triamterene

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17 alpha hydroxylase deficiency

mineralocorticoids up
cortisol and sex hormones down

hypertension
hypokalemia
decreased DHT

male: ambiguous genitalia, pseudo-hermaphroditism
female: lack of 2ndary sexual characteristics

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21-hydroxylase deficiency

decreased mineralocorticoids, decreased cortisol
increased sex hormones

hypotension
hyperkalemia
increased renin activity
increased 17-hydroxyprogesterone

presents in infancy

salt wasting
precocious puberty
female: virilization

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11 beta hydroxylase enzyme deficiency

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decreased aldosterone
increased 11-deoxycorticosterone

decreased cortisol
increased sex hormones

hypertension with low renin

female virilization

Hirsutism

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Due to decreased sex hormone binding globulin leading to increased free testosterone

Decreased estrogen (e.g. prolactinoma)
Insulin
Growth Hormone (acromegaly)
Obesity (especially PCOS)
Hypothyroidism
Glucocorticoids (Cushings)
Androgens
Nephrotic Syndrome

causes of hirsutism

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PCOS (70-80% of cases)
- Irregular menses, central obesity, infertility, acanthosis nigricans, and insulin resistance

Idiopathic hyperandrogenemia (6-15% of cases)
- Clinical and biochemical signs of hyperandrogenemia with regular ovulatory cycles

Idiopathic hirsutism (50% of mild cases)
- Normal androgens, normal ovulation, normal appearing ovaries

Congenital Adrenal Hyperplasia (< 5% of cases)
- Menstrual dysfunction and anovulation

Rare etiologies of hirsutism

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Acromegaly
Cushing syndrome
Hyperprolactinemia
Hyperthecosis
Thyroid dysfunction
Glucocorticoid resistance

Drugs
- Anabolic Steroids
- Androgenic steroids
- Valproic acid
Androgen secreting adrenal or ovarian tumor

Labs for patients with hirsutism

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Fasting Glucose/Insulin
TSH
Prolactin 
IGF-1
24 hour urine Cortisol

Testosterone
DHEAS
17OHP

of those last three tests:
Idiopathic or hereditary - all normal
PCOS (LH/FSH > 2) - testosterone mildly increased
CAH - all increased (17 OHP > 500* ng/dL = CAH)

Virilization:

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Increased muscularity, temporal alopecia, deepening voice, acne, clitoromegaly - Adrenal, Ovaries, CAH

Labs usually show increase of:
testosterone (ovarian neoplasm)
DHEAS (adrenal neoplasm)
androstenedione (adrenal or ovarian)

Labs for patients with virilization

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Ovarian tumor
total testosterone greatly increased with normal
DHEAS and 17 OHP. (Total Testosterone > 200 ng/dL points to ovary or adrenal)
Adrenal tumor
greatly increased DHEAS (> 700ng/dL)
CAH
Total Testosterone, DHEAS, and 17 OHP = all increased.
(17 OHP > 500 ng/dL = diagnostic)

Labs for patients with hirsutism or virilization

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** Total testosterone > 200 ng/dL = ovarian neoplasm

Serum androstenedione > 1000 ng/dL = adrenal or ovarian neoplasm

** DHEAS > 700mcg/dL = adrenal neoplasm or CAH

17-hydroxyprogesterone = CAH **(> 500 ng/dL is diagnostic); CAH usually has base line > 300ng/dL with ACTH stimulated to > 1000 ng/dL)

ACTH and 24 hour urine cortisol

LH:FSH > 2 (PCOS)

TSH, prolactin,

Treatment of Hirsutism

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Combination Oral Contraceptives

Antiandrogens
- Spironolactone
- Finasteride (5-alpha reductase inhibitor)
- Metformin (reduces hepatic gluconeogenesis)
—- Lowers peripheral insulin sensitivity
—- Lowers insulin levels
—- Not great for hirsutism
GnRH agonists (decrease gonadotropins by constant stimulation)

Pheochromocytoma symptoms

Classic Triad of Symptoms: Paroxysmal headache, palpitations, and sweating Other paroxysmal symptoms: Anxiety, pallor/flushing, syncope, tremor, blurred vision, heat intolerance, nausea, vomiting, abdominal pain, diarrhea, constipation, polyuria, polydipsia, weight loss, fatigue, chest pain, low grade fever

labs to confirm pheochromocytoma

24 hour Urinary Fractionated Metanephrines or Plasma Free Metanephrines - Metaneprhine (epinephrine metabolite) - Normetanephrine (norepinephrine metabolite) Other testing - 24 hour urine catecholamines - -- Norepinephrine, epinephrine, and dopamine - 24 hour urine vanilylmandelic acid (norepineprhine metabolite) , homovanillic acid (dopamine metabolite)

pheochromocytoma treatment goals

get blood pressure and heart rate down less than 1 PVC every 5 minutes on EKG No S-T segment changes or T-wave inversions on EKG

meds for pheochromocytoma

Alpha-adrenergic blocker - 1st Phenoxybenzamine Beta-adrenergic blocker – 2nd Atenolol Increase intake of salt and fluid for volume expansion if needed

Precipitators of adrenergic crisis

``` Postural changes or increase in intra-abdominal pressure Exertion, Trauma Foods/Beverages --- Banana, cheese, caffeine Emotional Stress Urination(bladder pheochromocytoma) Drugs --- Corticosteroids, metoclopramide, phenothiazines, tricyclic antidepressants, sympathomimetics, morphine, nasal decongestants, glucagon, chemotherapeutic agents Induction of anesthesia Manipulation of the tumor ```

where pheochromocytomas arise

80-85% of pheochromocytomas arise in the adrenal 10-20% : symphathetic ganglia in pelvis, mediastinum, or neck Along cranial nerves Vagus (parasympathetic) Most common site in the abdomen is at the origin of the inferior mesenteric artery

Pheochromocytoma Rules of 10

``` 10% Extra-adrenal 10% Bilateral 10% Malignant 10% Children 10% Familial 10% not associated with hypertension 10% contain calcification ```

Genetic Disorders associated with Pheochromocytomas

MEN 2A MEN 2B Von Hippel Lindau Syndrome Hereditary Paraganglioma-Pheochromocytoma Syndromes

Multliple Endocrine Neoplasia 2A (Autosomal Dominant)

Mutation of germline RET (neural crest cells) Pheochromocytoma Parathyroid Tumors Medullary Thyroid Cancers

Multiple Endocrine Neoplasia 2B (Autosomal Dominant)

Mutation of germline RET Medullary Thyroid Cancers Pheochromocytoma Mucosal Neuroma Intestinal ganglioneuromas Muscle, joint, and spinal problems – Marfinoid body habitus Facial Features including swollen lips, and thick eyelids

Von Hippel-Lindau Syndrome – Autosomal Dominant

``` Retinal angiomatosis Cerebral hemangioblastoma Pheochromocytoma Renal cyst/Carcinoma Pancreatic cysts Endolymphatic sac tumor Bilateral papillary cystadenomas of the epididymis in men and broad limagment of the uterus in women Genetic: VHL ```

Hereditary Paraganglioma-Pheochromocytoma Syndromes

Autosomal Dominant Paragangliomas in the skull base, upper mediastinum, and neck are usually parasympathetic and generally do not secrete. Paragangliomas in the lower mediastinum, abdomen, and pelvis are typically associated with the sympathetic nervous system and hypersecrete catecholamines

Gynecomastia etiologies

``` Physiologic - 25% - Neonatal, pubertal, or aging - 90 % newborns, 50% of boys during puberty, 65% of hospitalized men age 50-80 Idiopathic – 25% Medication- 10-25% Cirrhosis 8 % Hypogonadism Tumors Hyperthyroidism Chronic Renal Insufficiency Obesity ``` ``` Drugs: Cimetidine Finasteride Cannabinoids Omeprazole Ranitidine Spironolactone Diazepam Phenytoin Phenothiazines Opioids ```

Testing for gynecomastia

Pubertal Gynecomastia Does not need further testing in most cases ``` Testing when etiology unclear: Liver and kidney function Thyroid function Testosterone, LH, FSH, and Prolactin Serum estradiol Beta-HCG, AFP - rule out germ cell tumors DHEA-Sulfate – adrenal androgens ``` Testicular Ultrasound - Testicular mass - Gynecomastia > 5 cm - Elevated hCG - Elevated estradiol - Unclear etiology for gynecomastia Breast Cancer suspicion - Breast ultrasound and mammogram - Biopsy Abdominal CT if adrenal tumor suspected

treatment of gynecomastia

Pubertal boys reassurance and watchful waiting Asymptomatic men with long standing gynecomastia no treatment necessary Stop offending medications Medications - Antiestrogens – tamoxifen or raloxifene - Androgens – danazol Surgery

Kinder CIS Flashcards

(39 cards)