Medicine Marrow Pg No 845-854 (Endocrinology)
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Medicine Marrow Pg No 845-854 (Endocrinology)

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Questions and Answers

Which of the following symptoms is the most common in the described medical condition?

  • Abdominal pain
  • Palpitation
  • Episodic headache (correct)
  • Profuse sweating

    • Postural hypotension occurs in 75% of the cases of this medical condition.

    True

    What are the three symptoms that make up the symptom triad of the medical condition?

    Episodic headache, palpitation/tachycardia, profuse sweating

    Increased heart rate of more than ______ beats per minute is an indicator of postural hypotension.

    <p>30</p> Signup and view all the answers

    Match the following clinical features with their corresponding frequencies:

    <p>Episodic headache = 95% Postural hypotension = 75% Sustained hypertension = 20% Normal blood pressure = 15%</p> Signup and view all the answers

    What is the most common cause of Cushing's disease?

    <p>Pituitary ACTH-secreting adenoma</p> Signup and view all the answers

    Eosinophilia is associated with a positive infection such as Cushing's Syndrome.

    <p>False</p> Signup and view all the answers

    Name one symptom associated with Cushing's Syndrome.

    <p>Increased intraocular pressure</p> Signup and view all the answers

    The common triad for Cune Albright Syndrome includes precocious puberty, fibrous dysplasia, and __________.

    <p>cafe-au-lait spots</p> Signup and view all the answers

    Match the type of adrenal tumor with its corresponding characteristic:

    <p>Adrenal adenoma = ACTH independent Pituitary adenoma = ACTH dependent Paraneoplastic tumor = Associated with squamous cell carcinoma Adrenal carcinoma = Can cause increased ACTH secretion</p> Signup and view all the answers

    What is the total daily dose of oral hydrocortisone for someone with Addison's disease?

    <p>20 mg</p> Signup and view all the answers

    Fludrocortisone should always be administered regardless of mineralocorticoid deficiency.

    <p>False</p> Signup and view all the answers

    What is the first line treatment given during an Addisonian crisis?

    <p>IV Hydrocortisone 100 mg</p> Signup and view all the answers

    In case of fever or infection, the dose of hydrocortisone should be _____ the regular amount.

    <p>doubled</p> Signup and view all the answers

    Match the following medications with their equivalent conversions:

    <p>0.75 mg Dexamethasone = 4 mg Methylprednisolone 5 mg Prednisolone = 20 mg Hydrocortisone 20 mg Hydrocortisone = 25 mg Cortisol</p> Signup and view all the answers

    What percentage of the adrenal gland is composed of the adrenal medulla?

    <p>20%</p> Signup and view all the answers

    Epinephrine is produced from norepinephrine in the adrenal medulla.

    <p>True</p> Signup and view all the answers

    What is the rate limiting step in the biosynthetic pathway of catecholamines?

    <p>Tyrosine hydroxylase</p> Signup and view all the answers

    The smaller granules in the adrenal medulla mainly contain ______.

    <p>adrenaline</p> Signup and view all the answers

    Match the following metabolites with their respective categories:

    <p>Metanephrine = Measured metabolite of epinephrine VMA = Measured metabolite of catecholamines HVA = Measured metabolite of dopamine Normetanephrine = Measured metabolite of norepinephrine</p> Signup and view all the answers

    Which autoimmune syndrome is most commonly associated with Addison's disease?

    <p>Both A and B</p> Signup and view all the answers

    Addison's disease is caused exclusively by inherited factors.

    <p>False</p> Signup and view all the answers

    Name one clinical feature associated with glucocorticoid deficiency in Addison's disease.

    <p>Asthenia</p> Signup and view all the answers

    Patients with Addison's disease may experience ________ due to mineralocorticoid deficiency.

    <p>hyperkalemia</p> Signup and view all the answers

    Match the following features with their corresponding hormones in Addison's disease:

    <p>Asthenia = Glucocorticoids Hyperkalemia = Mineralocorticoids Loss of libido = Sex Steroids Hyperpigmentation = ACTH</p> Signup and view all the answers

    What is the most common type of pheochromocytoma?

    <p>Adrenal pheochromocytoma</p> Signup and view all the answers

    The ratio of males to females diagnosed with pheochromocytoma is 2:1.

    <p>False</p> Signup and view all the answers

    What is the surest sign of pheochromocytoma?

    <p>metastasis</p> Signup and view all the answers

    Pheochromocytomas are primarily associated with MEN2, VHL, and __________.

    <p>NF-1</p> Signup and view all the answers

    Match the following types of pheochromocytomas with their characteristics:

    <p>Adrenal pheochromocytoma = Produces only norepinephrine Extradrenal pheochromocytoma = Produces adrenaline and has a high malignant potential Sympathetic ganglia = Most common site: Organ of Zuckerkandl Parasympathetic ganglia = Most common site: Carotid body</p> Signup and view all the answers

    Which of the following causes can lead to weakness, fatigue, and weight loss?

    <p>HIV</p> Signup and view all the answers

    Addison's disease is characterized by elevated levels of ACTH.

    <p>True</p> Signup and view all the answers

    What are the two main treatments administered during an Addisonian crisis?

    <p>Fluids and steroids</p> Signup and view all the answers

    In primary adrenal insufficiency, serum potassium levels are typically ______.

    <p>increased</p> Signup and view all the answers

    Match the adrenal insufficiency features with their characteristics:

    <p>Salt craving = Primary adrenal insufficiency Hyperpigmentation = Primary adrenal insufficiency Sodium levels = Both adrenal insufficiencies ACTH levels = Secondary adrenal insufficiency</p> Signup and view all the answers

    What does a petrosal/peripheral ACTH ratio greater than 21 indicate?

    <p>Pituitary adenoma</p> Signup and view all the answers

    Transphenoidal hypophysectomy is a treatment option for patients with pituitary adenoma.

    <p>True</p> Signup and view all the answers

    Name one medication used to prevent relapse in pituitary adenoma treatment.

    <p>Cabergoline</p> Signup and view all the answers

    Symptoms of Para Neoplastic Syndrome include _____ and reduced protein catabolism.

    <p>hyperpigmentation</p> Signup and view all the answers

    Match each treatment option with its corresponding description:

    <p>Transphenoidal surgery = Surgical removal of the pituitary gland Radiotherapy = May be used instead of surgery for patients unfit for surgery Ketoconazole = Medication to prevent relapse in pituitary adenoma treatment Mifepristone = Indicated as a glucocorticoid receptor inhibitor</p> Signup and view all the answers

    What is an early sign of endogenous hypercortisolism?

    <p>Both B and C</p> Signup and view all the answers

    A low dose dexamethasone suppression test (LDDST) is performed after the overnight dexamethasone suppression test (ONDST) indicates cortisol levels are greater than 1.8 µg/dL.

    <p>True</p> Signup and view all the answers

    What indicates that Cushing's Syndrome is ruled out in an overnight dexamethasone suppression test?

    <p>Cortisol &lt; 1.8 µg/dL</p> Signup and view all the answers

    If ACTH levels are greater than ______ pg/mL, this may indicate a paraneoplastic syndrome.

    <p>100</p> Signup and view all the answers

    Match the following test with its purpose:

    <p>Overnight Dexamethasone Suppression Test = Rules out Cushing's Syndrome Low-Dose Dexamethasone Suppression Test = Indicates sustained hypercortisolism ACTH measurement = Identifies pituitary or adrenal causes MRI of brain = Assesses for pituitary tumors</p> Signup and view all the answers

    What treatment option is suggested for a unilateral adrenal adenoma?

    <p>Unilateral adrenalectomy</p> Signup and view all the answers

    A CT scan is used to evaluate for adrenal tumors in patients with ACTH-independent causes.

    <p>True</p> Signup and view all the answers

    What does an ACTH level greater than 10 pg/mL suggest?

    <p>ACTH dependent cause</p> Signup and view all the answers

    For sustained cortisol levels, the ______ test is performed after the overnight dexamethasone suppression test.

    <p>Low-Dose Dexamethasone Suppression Test</p> Signup and view all the answers

    What might indicate a pituitary adenoma?

    <p>Tumor size ≥ 6 mm</p> Signup and view all the answers

    What is the most common cause of childhood Cushing's syndrome?

    <p>Adrenal cause</p> Signup and view all the answers

    Midnight serum cortisol levels above 1.8µg/dL and salivary cortisol levels above 5.5 mmol/L indicate the presence of Cushing syndrome.

    <p>True</p> Signup and view all the answers

    Name one common feature associated with protein catabolism due to glucocorticoid excess.

    <p>Facial plethora</p> Signup and view all the answers

    The condition characterized by obesity, precocious puberty, and increased secretion of adrenal androgen in children is called __________.

    <p>childhood Cushing</p> Signup and view all the answers

    Match the following symptoms of Cushing syndrome with the appropriate descriptions:

    <p>Proximal myopathy = Weakness in proximal muscles of the lower limbs Young onset osteoporosis = Fractures and bone age shortening Violaceous striae = Stretch marks due to collagen loss Bruising &amp; ecchymosis = Easy bleeding and bruising that occur without obvious injury</p> Signup and view all the answers

    Study Notes

    Clinical Features of a Medical Condition

    • This document outlines the clinical features of a medical condition, potentially a type of hypertension or related disorder.

    Pathophysiology

    • Sustained sympathetic activity leads to increased intravascular volume, diabetes mellitus (DM), weight loss, and impaired glomerular filtration rate (GFR) resulting in hypercalcemia and acute abdominal pain.
    • Secondary paraneoplastic polycythemia can be caused by a tumor (Pheochromocytoma) which produces erythropoietin (EPO), leading to secondary paraneoplastic polycythemia.

    Clinical Features

    • Symptoms (triad): Episodic headache (most common symptom, 95%), palpitation/tachycardia, and profuse sweating.
    • Other features: Abdominal pain, palpitation (sinus tachycardia), perspiration, and panic attack (75%).
    • Signs:
      • Blood pressure: Episodic hypertension (75-85%) or normal blood pressure (15%) – this is the most common presentation.
      • Postural hypotension (75%): Measurement involves taking blood pressure in supine position first, then standing for 2-5 minutes; indicators include increased heart rate (>30/minute), decreased systolic blood pressure (>20 mmHg), and decreased diastolic blood pressure (>10 mmHg).
      • Sustained hypertension: Only present in 20% of cases.
      • Resistant young hypertension.

    Cushing's Syndrome

    • Eye: Increased intraocular pressure, cataracts, glaucoma.
    • GIT: GI ulceration.
    • Miscellaneous: Reduced linear growth.
    • Blood and Immune system: Increased white blood cell count (but still prone to infection), inhibits IL-6 (anti-inflammatory action), eosinopenia, and increased thrombosis (hypercoagulable state).

    Causes of Cushing's Syndrome

    • ACTH Dependent (90%):
      • Pituitary ACTH-secreting adenoma (75%): Most common cause; endogenous cause for overall Cushing's disease; micro/macro adenomas.
      • Other Causes (15%): Paraneoplastic Tumor (e.g., squamous cell carcinoma), Adrenal Carcinoma.
    • ACTH Independent (10%): Adrenal adenoma, other causes (most common in toddlers), GSA-Subunit-defect, Primary Pigment nodular adrenal disease (PPNAD), Thymic/bronchial carcinoid, Pheochromocytoma/medullary Ca thyroid.

    Presentation (Adult Cushing's)

    • Sex: Female > Male
    • Diagnosis: Based on discriminatory value of symptoms.

    Cushing's Syndrome Diagnostic Flowchart

    • Initial Evaluation:
      • 8 AM plasma cortisol > 1.8 µg/dL and a loss of diurnal variation suggests endogenous hypercortisolism.
      • Suspected exogenous hypercortisolism: Could be caused by prolonged steroid use, hyperestrogenism, PCOS, and oral contraceptive pills.
      • Suspected endogenous hypercortisolism: Proceed to further testing.
    • Further Testing (Endogenous Hypercortisolism):
      • Overnight Dexamethasone Suppression Test (ONDST): Administer 1 mg oral dexamethasone at bedtime and measure plasma cortisol the next morning.
      • Cortisol < 1.8 µg/dL: Suggests Cushing's is ruled out.
      • Cortisol > 1.8 µg/dL: Suggests endogenous hypercortisolism continues. Proceed to Low-Dose Dexamethasone Suppression Test (LDDST).
      • Low-Dose Dexamethasone Suppression Test (LDDST): Administer 0.5 mg dexamethasone every 6 hours for 2 days. Measure cortisol levels. Sustained cortisol levels indicate the presence of endogenous hypercortisolism.
    • ACTH evaluation:
      • Measure ACTH blood level: ACTH < 10 pg/mL indicates ACTH independent cause; ACTH > 10 pg/mL indicates ACTH dependent cause.
      • ACTH-Independent (Adrenal Causes) Further Evaluation: CT scan (or MRI) of adrenal glands to check for adrenal tumors (adenoma); unilateral adenoma - unilateral adrenalectomy (surgical removal of the affected adrenal); bilateral hyperplasia - bilateral adrenalectomy; risk of ACTH due to -ve feedback - proceed to MRI (or CT scan) of the brain to rule out a pituitary tumor.
      • ACTH Dependent (Pituitary gland or other ACTH producing organs) Further Evaluation: MRI of the brain with gadolinium contrast to assess the pituitary gland for tumors (adenomas) or structural abnormalities; tumor size ≥ 6 mm - consider pituitary adenoma; tumor size < 6 mm - consider other factors; if negative proceed to PET scan; if no mass, inferior petrosal sinus sampling - evaluate for other causes; other tests depend on the results of previous tests.
    • Additional Notes: High Dose Dexamethasone Suppression Test (DST), Para-neoplastic syndrome (PNS), if ACTH levels are greater than 100 pg/mL, this may indicate a paraneoplastic syndrome (PNS). A PET scan may be warranted. This flowchart provides a general guideline for the diagnosis of Cushing's Syndrome, and specific testing and treatment options may vary depending on individual patient circumstances. Consult with a medical professional for accurate diagnosis and treatment.

    Treatment for Addison's Disease

    • Maintenance:
      • Oral Hydrocortisone (DOC): 10 mg (8 to 8:30 am), 5 mg (1 to 1:30 pm), 5 mg (4 to 4:30 pm) - total 20 mg/day.
      • Others: Prednisolone, Dexamethasone.
      • Fludrocortisone: 50-100 µg, if MC deficiency present.
    • Equivalent Conversions: 0.75 mg Dexamethasone = 4 mg Methylprednisolone, 5 mg Prednisolone = 20 mg Hydrocortisone = 25 mg Cortisol.
    • Crisis:
      • IV Hydrocortisone 100 mg immediately + 50 mg IV Q6H.
      • 200 mg IV: 2nd day, 100 mg IV: 3rd day, 50 mg IV: 4th day.
      • Oral Hydrocortisone 25 mg (10, 10, 5) for 1 week.
      • 20 mg (10, 5, 5) as maintenance + Fludrocortisone.
    • Special Considerations: Fever or infection - double the dose; hospitalization - increase dose to 50-75 mg/day; surgery - 50 to 100 mg IV hydrocortisone.
    • IV Fluids: 0.9% NS/DNS IL over 1 hour; 2-3 L over the next 12 hours.

    ADRENAL MEDULLA: PART 1

    • Anatomy & Physiology of Adrenal Gland
      • Anatomy:
        • Development: Neuroectoderm.
        • Composition: Adrenal medulla (20%) and Adrenal cortex (80%).
      • Physiology:
        • Hormones: Catecholamines (pulsatile secretion): 80/20 rule - smaller granules: Adrenaline/Epinephrine (80%), larger dense granules: Noradrenaline (NA)/Norepinephrine (20%).
        • Metabolites: Metanephrine, Vanillyl mandelic acid (VMA), Homovanillic acid (HVA), Normetanephrine.
    • Biosynthetic pathway: Tyrosine → Tyrosine hydroxylase (Rate limiting step) → DOPA → Dopamine → Norepinephrine (NE) → Epinephrine.
    • PNMT (Phenylethanolamine N-methyltransferase): Present only in adrenal gland; extra-adrenal pheochromocytoma - only produce NE; metabolic relation between cortex and medulla - Cortisol (cortex) stimulates PNMT (medulla).

    Addison's Disease

    • Adrenal Insufficiency
      • 1° Adrenal Insufficiency (Addison's Disease)
        • Causes: Autoimmune (most common), inherited, secondary (less common), infective.
    • Clinical Features
      • Size of adrenal gland: Enlarged, normal, atrophic, or asymmetrical.
      • Features: Decreased Glucocorticoids (GC), decreased Mineralocorticoid (MC), increased Sex Steroids (SS), increased Adrenocorticotropic Hormone (ACTH), additional features.

    Pheochromocytoma

    • Age: 4th to 5th decade of life, male:female ratio 1:1.
    • Definition: Neural crest cell tumor producing catecholamines (pulsatile) and catecholamine metabolites.
    • Types: Based on origin: Adrenal pheochromocytoma (75%) - Produce only NA; Extradrenal pheochromocytoma/paraganglioma (25%) - Adrenaline > NA, high malignant potential.
    • Rule of 10 (Obsolete): Bilateral (10%), extraadrenal (10%), familial (10%), malignant (10%).
    • Heading: Young patients, associated with MEN2, VHL, NF-1, usually bilateral except NF-1 - usually unilateral.
    • Surest sign: Metastasis.
    • Succinate dehydrogenase B (SDH-B): ↑ metastatic potential.
    • Adrenomedullin: Produced from endothelium; Functions - vasodilation, inhibit endothelial cell apoptosis (Prevent septic shock).

    Endocrinology

    • Causes of weakness, fatigue & weight loss: TB, HIV, Malignancy, Lymphoma, Addison's disease.
    • Addisonian crisis: Precipitated by infection (Any stress) or abrupt steroid withdrawal.
    • Features: acute abdominal pain, vomiting, constipation.
    • Rx: Fluids + steroids.
    • Hypoglycemia
    • Hypotension/circulatory failure

    Difference between 1° & 2º adrenal insufficiency

    • Features: GC, mc, SS, ACTH, Salt craving, postural hypotension, hyperpigmentation, loss of axillary hair, vitiligo, S.Potassium, and S.Sodium levels.

    Investigations

    • Fasting cortisol levels: 20 µg/dL - ↓; 3-20 µg/dL - ↓ (Synacthen test/ACTH stimulation test).

    Endocrinology Notes - Telegram Channel @back_ed8

    • 848
      • Diagnostic Workup & Treatment of Pituitary & Adrenal Conditions:
        • Inferior petrosal sinus sampling: Measure petrosal/peripheral ACTH ratio; >21 (significant) - Indicative of pituitary adenoma.
        • PET scan: Used to identify tumors.
        • Para Neoplastic Syndrome (PNS): Symptoms - hyperpigmentation, reduced protein catabolism; possible effects - mineralocorticoid action (HTN, hypokalemia).
        • High dose DST (Dexamethasone Suppression Test): Measure cortisol levels after dexamethasone administration; >50% suppression suggests a pituitary cause.
      • Treatment of Pituitary Adenoma:
        • Transphenoidal hypophysectomy: Surgical removal of the pituitary gland.
        • Radiotherapy (and line): May be used with or instead of surgery for patients unfit for traditional surgical approaches.
      • Treatment of relapse or unfit for surgery patients: Transphenoidal surgery (TOC), Radiotherapy (and line).
      • Medications to prevent relapse: Ketoconazole, Pasireotide, Cabergoline, Metyrapone, Mifepristone, Mitotane.
      • Other Related Conditions: Pseudo Cushing syndrome, Adrenal adenoma.

    Endocrinology

    • 846
      • Active space:
        • Protein catabolism (↓AA uptake): Proximal myopathy (bilateral, proximal, symmetrical, LL > UL), facial plethora - Thinning of skin, loss of subcutaneous fat, bruising & ecchymosis, young onset osteoporosis - fractures, Violaceous striae (D/t loss of collagen).
        • Diabetes mellitus: HTN metabolic syndrome.
        • Incidental adrenal mass.
      • Childhood Cushing:
        • Sex: m >> F.
        • Cause: Adrenal cause > Pituitary cause.
        • Features: Less features of protein catabolism, obesity, ↑ Secretion of Adrenal androgen → Precocious puberty.
        • Treatment: Radiotherapy (good response).
      • Diagnosis and Treatment: If midnight serum cortisol ≤1.8µg/dL and midnight salivary cortisol ≤5.5 mmol/L, then no Cushing syndrome is ruled out.
      • Images: Abdominal CT scan, Patient portrait, Adrenal mass.

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    Description

    This quiz covers the clinical features and pathophysiology of hypertension and related disorders. It highlights common symptoms, signs, and their significance in diagnosis. Test your knowledge about these clinical manifestations and their underlying causes.

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