Dr Om Lakhani

Interprets the postural stimulation test to detect angiotensin II‑unresponsive aldosterone‑producing adenoma (APA) or familial hyperaldosteronism type I (FH‑I) when aldosterone fails to rise with upright posture. Triggered by clinical cues such as “postural stimulation test shows no aldosterone increase”, “suspected APA with non‑responsive aldosterone”, or “evaluating FH‑I in young patients with early‑onset hypertension and hypokalemia”.

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Recommends confirmatory testing with corticotropin stimulation test in patients presenting with clinical symptoms or signs suggesting PAI (e.g., fatigue, hypotension, hyponatremia, hyperpigmentation) when the patient's condition permits safe testing. Use when a clinician identifies suggestive features and circumstances allow outpatient stimulation testing without imminent need for emergency glucocorticoids.

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Initiates a 6‑month trial of DHEA replacement in women with primary adrenal insufficiency (PAI) who have persistent low libido, depressive symptoms, or low energy despite otherwise optimized glucocorticoid and mineralocorticoid therapy. If the patient does not report a sustained beneficial effect after 6 months, discontinue DHEA.

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Suggests a trial of dehydroepiandrosterone (DHEA) replacement in women with primary adrenal insufficiency (PAI) who report low libido, depressive symptoms, and/or low energy levels despite otherwise optimized glucocorticoid and mineralocorticoid replacement. Consider when a woman with PAI presents with persistent low libido, depressive symptoms, and/or low energy levels after glucocorticoid and mineralocorticoid doses have been titrated to clinical targets.

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This skill outlines the emergency management of suspected adrenal crisis, recommending immediate parenteral hydrocortisone (100 mg adult, 50 mg/m² pediatric) plus fluid resuscitation, then 200 mg/24 h (50–100 mg/m²/24 h pediatric) hydrocortisone. Trigger when patient presents with hypotension, hyponatremia, hyperkalemia, or unexplained shock suggestive of adrenal insufficiency.

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Recommends immediate IV hydrocortisone stress dosing for patients with suspected adrenal crisis presenting with severe adrenal insufficiency symptoms such as hypotension, hyponatremia, hyperkalemia, or acute abdominal pain. Use when clinician observes signs of adrenal crisis (e.g., syncope, delirium, marked abdominal tenderness) prior to diagnostic test results.

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Determines follow‑up interval for patients with primary adrenal insufficiency (PAI) based on age: annual visits for adults and children, and every 3–4 months for infants. Triggered when establishing routine endocrine care after PAI diagnosis or during stable management.

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Recommends initiating glucocorticoid therapy in all patients with confirmed primary adrenal insufficiency (PAI). Use when PAI diagnosis is confirmed by subnormal cortisol response to ACTH stimulation test or elevated ACTH with low cortisol.

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Suggests monitoring DHEA replacement in women with primary adrenal insufficiency (PAI) by measuring morning serum DHEAS levels before the daily dose, targeting the mid‑normal range. Consider when evaluating low libido, depressive symptoms, or low energy despite optimized glucocorticoid and mineralocorticoid replacement.

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Monitors glucocorticoid replacement in primary adrenal insufficiency (PAI) patients using clinical assessment of body weight, postural blood pressure, energy levels, and signs of frank glucocorticoid excess. Trigger phrases include "PAI patient on glucocorticoid replacement," "assess for weight gain or insomnia," and "evaluate for postural hypotension or fatigue."

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Suggests that pregnant patients with primary adrenal insufficiency be monitored for clinical signs of glucocorticoid over- or under-replacement such as normal weight or gain, fatigue, postural hypotension or hypertension, and hyperglycemia, with at least one review each trimester. Use when managing a pregnant patient with PAI to assess glucocorticoid dosing adequacy.

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Recommends initiating hydrocortisone replacement at a total starting dose of 8 mg/m2 body surface area per day, given in three or four divided doses, for children with confirmed primary adrenal insufficiency (PAI). Use when starting glucocorticoid therapy in pediatric PAI patients, adjusting the dose according to individual clinical response, growth, and need for stress coverage.

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Suggests using prednisolone 3–5 mg/d orally once or twice daily as a glucocorticoid alternative in primary adrenal insufficiency. Consider this option when evaluating glucocorticoid therapy, particularly in patients with reduced medication compliance.

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In pregnant women with primary adrenal insufficiency (PAI), the guideline suggests using hydrocortisone over cortisone acetate, prednisolone, or prednisone for glucocorticoid replacement. It recommends against dexamethasone due to its lack of placental inactivation, which can expose the fetus to excess glucocorticoid exposure.

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Suggests using morning cortisol ≤140 nmol/L (5 µg/dL) in combination with ACTH as a preliminary test suggestive of primary adrenal insufficiency when a corticotropin stimulation test is not feasible. Use when patients present with indicative symptoms such as hypotension, hyponatremia, hyperkalemia, or fatigue and corticotropin stimulation testing cannot be performed.

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This skill recommends simultaneous measurement of plasma renin and aldosterone in patients with primary adrenal insufficiency (PAI) to determine the presence of mineralocorticoid deficiency. It is triggered when assessing for mineralocorticoid deficiency in PAI, such as in patients with unexplained hyponatremia, hyperkalemia, or salt craving despite glucocorticoid replacement.

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The Endocrine Society recommends a standard-dose corticotropin (ACTH) stimulation test to confirm primary adrenal insufficiency when clinical suspicion arises from symptoms such as hypotension, hyponatremia, hyperkalemia, or unexplained fatigue. Dosing is weight/age based: 250 µg for adults and children ≥2 years, 15 µg/kg for infants, and 125 µg for children <2 years.

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Recommends hydrocortisone stress dosing during the active phase of labor for pregnant patients with primary adrenal insufficiency, using a regimen similar to major surgical stress. Trigger phrases: "active phase of labor", "cervix dilation ≥4 cm", "contractions every 5 minutes", "PAI patient in labor".

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Determines when to avoid aldosterone suppression testing in hypertensive patients with a positive PA screen who have normokalemia and suppressed aldosterone below assay‑specific cutoffs (<11 ng/dL immunoassay or <8 ng/dL LC‑MS/MS), indicating low likelihood of primary aldosteronism. Use when a clinician asks whether to skip confirmatory suppression testing based on low pretest probability.

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Recommends performing a dexamethasone suppression test to evaluate for autonomous cortisol secretion in patients with primary aldosteronism and adrenal adenoma. Triggered when a clinician considers whether to perform a dexamethasone suppression test in a patient with PA and adrenal adenoma.

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Recommends spironolactone as first-line mineralocorticoid receptor antagonist for initial medical therapy of primary aldosteronism due to low cost and widespread availability. Use when a clinician queries which MRA to start for a patient with PA requiring medical therapy.

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Recommends using mineralocorticoid receptor antagonists rather than epithelial sodium-channel inhibitors for medical treatment of primary aldosteronism. Use when a clinician asks whether to use an MRA or ENaC inhibitor for medical treatment of PA.

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In patients with primary aldosteronism receiving mineralocorticoid receptor antagonist therapy, the guideline recommends titrating the MRA dose upward to raise renin when blood pressure remains uncontrolled and renin is suppressed. Consider this step when hypertension is not at goal despite MRA therapy and plasma renin activity (or direct renin concentration) is low.

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Recommends correcting serum potassium to the laboratory reference range and repeating aldosterone-to-renin ratio (ARR) screening when the initial screen is negative in a hypertensive patient with hypokalemia. Use when a clinician encounters a negative PA screen in a hypertensive patient with hypokalemia and wonders whether to repeat testing after potassium repletion.

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