Ulick Syndrome

Ulick syndrome—better known today as Apparent Mineralocorticoid Excess (AME)—is a rare, inherited condition where the kidneys are “tricked” into acting like the hormone aldosterone is very high, even though actual aldosterone is low. This happens because the enzyme 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2) is weak or missing. That enzyme normally turns cortisol (active) into cortisone (inactive) inside kidney cells. When the enzyme does not work, cortisol piles up and turns on the mineralocorticoid receptor (MR), which raises blood pressure, wastes potassium, and causes metabolic alkalosis. AME is autosomal recessive and caused by HSD11B2 gene variants. PubMed Central+2BioMed Central+2 Children (and sometimes adults) develop early, resistant high blood pressure, low potassium, and alkalosis. If untreated, this can lead to kidney damage and growth problems. PubMed Central

Ulick syndrome is a rare, inherited blood-pressure disease. It happens when the body cannot safely switch the hormone cortisol into its calmer form cortisone inside the kidney. Normally an enzyme called 11β-hydroxysteroid dehydrogenase type 2 (short name 11β-HSD2) makes this switch. When this enzyme is weak or missing, too much cortisol reaches the mineralocorticoid receptor in the kidney. The kidney then acts as if there is too much aldosterone (salt-retaining hormone), even though aldosterone is low. This “false aldosterone” effect causes the body to hold onto salt and water, lose potassium, and raise blood pressure from a young age. Doctors first described this condition in 1979, and it is often called “apparent mineralocorticoid excess” because it looks like aldosterone is high when it is not. PubMed+2Oxford Academic+2

Key features are: resistant high blood pressure in children or young adults, low blood potassium, metabolic alkalosis, very low renin and low aldosterone, and a high ratio of cortisol to cortisone breakdown products in urine. Most cases are due to harmful changes (variants) in the HSD11B2 gene that makes the 11β-HSD2 enzyme. PubMed Central+1

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Other names

• Apparent Mineralocorticoid Excess (AME)

• Syndrome of Apparent Mineralocorticoid Excess (SAME)

• Ulick syndrome (after the describing author)

• 11β-hydroxysteroid dehydrogenase type 2 deficiency (11β-HSD2 deficiency)

• HSD11B2-related hypertension

• “Licorice-like” hypertension (for acquired, licorice-induced cases that mimic AME) PubMed+2Oxford Academic+2

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Types

1. Classic (severe) congenital AME
   This is the typical, early-onset form in infants or children. The enzyme activity is very low. Children show failure to thrive, thirst and urination problems, and severe high blood pressure with low potassium. Orpha

2. Non-classic (milder or late-onset) AME
   Here, some enzyme function remains. High blood pressure may appear later (teens or adults), and low potassium is less constant. Genetic testing can still show HSD11B2 variants. PubMed

3. Acquired “AME-like” states
   Certain substances inhibit 11β-HSD2 even when the gene is normal. The leading example is licorice (glycyrrhizic/glycyrrhetinic acid). Some medicines and medical conditions can also tip cortisol balance and imitate AME. These cases improve when the trigger is removed. PubMed+1

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Causes

1. HSD11B2 gene mutations (autosomal recessive)
   Inherited changes reduce or stop 11β-HSD2 activity, letting cortisol overstimulate kidney mineralocorticoid receptors. This is the main cause of classic AME. PNAS

2. Compound heterozygous HSD11B2 variants
   Two different damaging variants, one from each parent, can cause disease with early hypertension and low potassium. PubMed

3. Consanguinity (parents related by blood)
   Increases the chance both parents carry the same recessive HSD11B2 variant, raising the risk of affected children. Frontiers

4. Population clusters
   Some populations show more reported cases due to founder variants or consanguinity patterns. Frontiers

5. Licorice/glycyrrhizic acid intake
   Licorice and some herbal products contain glycyrrhizin/glycyrrhetinic acid, which inhibits 11β-HSD2 and produces an AME-like picture: high BP, low potassium, low renin/aldosterone. PubMed+1

6. Carbenoxolone and related derivatives
   These compounds are pharmacologic 11β-HSD inhibitors and can mimic AME when used or abused. Oxford Academic

7. Very high cortisol states (Cushing physiology)
   Excess cortisol can overwhelm 11β-HSD2 activity so cortisol reaches mineralocorticoid receptors, imitating mineralocorticoid excess. (General pathophysiology covered in reviews.) PubMed Central

8. ACTH-driven cortisol excess from medicines
   Some anti-cancer drugs (e.g., abiraterone) increase ACTH and shift steroid balance, causing mineralocorticoid excess signs. Cureus

9. Certain antifungals (medication-induced)
   Clinical reports show mineralocorticoid-excess physiology during antifungal therapy; stopping the drug and adding salt restriction/MR blockade reverse it. Kidney News

10. Chronic high-salt intake (worsens phenotype)
    Dietary salt aggravates hypertension when mineralocorticoid signaling is high; low-salt diet is part of care. Kidney News

11. Dehydration/volume contraction
    Can intensify renin-angiotensin suppression pattern seen in AME, revealing the phenotype more strongly (clinical principle supported by reviews). PubMed Central

12. Prematurity/early kidney vulnerability (hypothesis in reviews)
    Immature renal handling of steroids may unmask enzyme deficits earlier (noted in expert reviews of monogenic hypertension). Frontiers

13. Obesity or metabolic stress (exacerbating factor)
    Metabolic stress may alter cortisol metabolism and raise BP burden in genetic AME (discussed in reviews). PubMed Central

14. Kidney disease progression in untreated AME
    Prolonged salt retention and hypokalemia may damage kidneys, feeding back into worse BP and electrolyte control. PubMed

15. Pregnancy (physiologic cortisol changes)
    Cortisol rises in pregnancy and may worsen BP control in women with unrecognized AME. PubMed

16. Exogenous glucocorticoids without mineralocorticoid blockade
    Some regimens can unmask electrolyte/BP problems if not balanced—management nuance noted in case literature. PubMed Central

17. Adrenal incidentalomas ruled out (differential)
    Not a cause of AME itself, but mistaken as primary aldosteronism; imaging is used to exclude these when evaluating causes of low-renin hypertension. enbpr.org

18. High cortisol to cortisone ratio due to enzyme inhibition by foods/supplements
    Beyond licorice, some products may contain glycyrrhizin-like chemicals; careful history is needed. Oxford Academic

19. Genetic modifiers of steroid metabolism (research area)
    Variants in other enzymes may influence severity in HSD11B2 deficiency (discussed in genetic overviews). PNAS

20. Brain 11β-HSD2 involvement (salt appetite)
    Experimental deletion of HSD11B2 in the brain increases salt appetite, which can worsen BP control in humans with AME who already crave salt. AHA Journals

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Common symptoms and signs

1. High blood pressure (often from childhood)
   Hypertension is persistent and hard to control because the kidney acts as if aldosterone is high. PubMed Central

2. Low potassium (hypokalemia)
   Potassium is wasted in urine, leading to weakness, cramps, palpitations, or constipation. PubMed Central

3. Headaches
   High blood pressure commonly causes headaches, especially in children with severe early-onset disease. Orpha

4. Fatigue and muscle weakness
   Low potassium reduces normal muscle and nerve function, so day-to-day activity feels tiring. PubMed Central

5. Polydipsia (excessive thirst)
   Salt and water retention with kidney tubule stress make children very thirsty. Orpha

6. Polyuria (frequent urination)
   The kidneys handle large urine volumes; bed-wetting or nocturia can occur. Orpha

7. Failure to thrive or poor growth
   Untreated AME can slow growth due to chronic illness burden and electrolyte problems. Orpha

8. Salt craving
   Some patients crave salty foods; this can worsen blood pressure. AHA Journals

9. Dizziness or faintness
   Sudden BP changes or severe hypokalemia may cause light-headedness.

10. Visual problems from hypertensive retinopathy
    Very high BP can damage eye blood vessels, causing blurred vision or headaches.

11. Palpitations or irregular heartbeat
    Hypokalemia increases the risk of heart rhythm changes; an ECG may show changes.

12. Constipation
    Low potassium slows gut muscle activity.

13. Numbness or tingling
    Potassium imbalance can produce nerve symptoms.

14. Swelling (edema) is usually mild
    Salt retention is present, but many patients mainly show high BP rather than obvious swelling.

15. Complications if untreated
    Risk of left ventricular hypertrophy, kidney damage, and stroke rises over time; early recognition prevents this. PubMed

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Diagnostic tests

A) Physical examination

1. Accurate blood pressure measurement (multiple visits)
   Measure sitting and standing, both arms, using the right cuff size. Persistent elevation in a child/young adult with low renin/aldosterone suggests AME. PubMed Central

2. Growth chart and nutrition review
   Plot height/weight; children with classic AME may show poor growth if not treated. Orpha

3. Volume status check
   Look for signs of salt retention (e.g., mild edema) and dehydration; both can affect BP readings.

4. Fundoscopic (eye) exam
   Checks for hypertensive retinopathy from long-standing high BP.

5. Cardiovascular exam
   Listen for abnormal heart sounds or signs of left ventricular strain from severe hypertension.

B) Manual/bedside tests

6. Ambulatory Blood Pressure Monitoring (24-hour ABPM)
   Confirms sustained hypertension, detects nighttime patterns, and assesses treatment response.

7. Orthostatic vitals
   BP and heart rate while lying/sitting/standing help assess autonomic and volume effects.

8. Diet and product history focusing on licorice and herbs
   Ask about candies, teas, tobacco, herbal medicines, or supplements containing licorice or glycyrrhizin, which can mimic AME. BioMed Central

9. Medication review
   Screen for drugs that shift steroid balance (e.g., abiraterone) or inhibit 11β-HSD2 (case-based reports for antifungals), and stop the culprit where possible. Cureus+1

10. Family assessment
    Ask about consanguinity and relatives with early hypertension or low potassium to support a genetic cause. Frontiers

C) Laboratory and pathological tests

11. Serum electrolytes and acid–base status
    Look for low potassium and metabolic alkalosis—the typical biochemical pattern. PubMed Central

12. Plasma renin activity (PRA) or renin concentration
    Low renin is characteristic because the kidney “thinks” aldosterone is high. PubMed

13. Plasma aldosterone
    Usually low or low-normal in AME, helping distinguish it from primary aldosteronism where aldosterone is high. PubMed

14. 24-hour urinary steroid profile with metabolite ratios
    Diagnostic hallmark: high cortisol-to-cortisone metabolite ratio, classically (THF + allo-THF) / THE elevated. This reflects impaired 11β-HSD2 activity. PubMed+1

15. Urinary free cortisone (very low) and urinary free cortisol (relatively higher)
    Confirms the same pattern using free steroids. ScienceDirect

16. Genetic testing for HSD11B2
    Identifies pathogenic variants (classic and non-classic forms), allows family counseling, and guides lifelong care. PNAS

17. Kidney function tests and urine chemistry (including calcium)
    Monitors for kidney injury and hypercalciuria/nephrocalcinosis; thiazides may be considered if hypercalciuria is present. Revista Nefrología

18. Cortisol and ACTH (context-specific)
    Helps rule out Cushing physiology or medicine-related steroid changes that can imitate AME. PubMed Central

D) Electrodiagnostic tests

19. Electrocardiogram (ECG)
    Checks for arrhythmias or characteristic changes of hypokalemia (e.g., U waves), and for left ventricular hypertrophy from chronic hypertension.

20. Ambulatory ECG/Holter (when palpitations present)
    Assesses rhythm over 24–48 hours to detect ectopy caused by low potassium or severe hypertension.

E) Imaging tests

21. Renal ultrasound
    Screens for kidney size/structure and nephrocalcinosis; monitors chronic effects of untreated disease. Revista Nefrología

22. Echocardiography
    Assesses left ventricular hypertrophy or reduced function caused by long-standing high BP; can improve with proper treatment. PubMed

23. Retinal photography
    Documents hypertensive retinopathy, useful in children who may not report visual changes.

24. Adrenal imaging (CT/MRI) only to exclude mimics
    Used when the history suggests primary aldosteronism or other adrenal causes; in AME, adrenals are not overproducing aldosterone. enbpr.org

25. Renal artery Doppler (when indicated)
    Rules out renovascular hypertension in the differential diagnosis.

Treatment overview

Treatment goals are: lower blood pressure, correct potassium, protect kidneys, and avoid triggers. The most supported medicines are MR blockers (spironolactone, eplerenone) and ENaC blockers (amiloride). Low-dose dexamethasone can help in some patients by reducing cortisol production (turning down the drive on the “wrong” receptor), but results vary. In severe kidney failure, kidney transplantation is curative for the metabolic defect because the new kidney has normal 11β-HSD2. PubMed+3New England Journal of Medicine+3ScienceDirect+3

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Non-pharmacological treatments (therapies & practical measures)

Each item explains what it is, purpose, and mechanism—in simple words with references to the AME literature or hypertension care.

1. Strict salt (sodium) restriction
   What: Reduce daily salt intake (avoid salty snacks, processed foods).
   Purpose: Lower blood pressure and reduce potassium loss.
   Mechanism: Less sodium reaching the kidney’s collecting duct reduces sodium reabsorption through ENaC and lowers the “volume” signal that drives hypertension in AME. Salt restriction is first-line in low-renin, mineralocorticoid-type hypertension. PubMed Central

2. Avoid licorice and “natural” products with glycyrrhizin
   What: Read labels; avoid licorice candy, some herbal teas, and chewing tobacco with licorice.
   Purpose: Prevent acquired enzyme blockade that copies AME.
   Mechanism: Glycyrrhetinic acid inhibits 11β-HSD2, letting cortisol stimulate MR, raising blood pressure and lowering potassium. Stopping licorice removes the block. PubMed Central+2Oxford Academic+2

3. Adequate potassium in the diet
   What: Emphasize potassium-rich foods if your doctor approves (bananas, oranges, lentils), closely monitoring labs if on MRAs/amiloride.
   Purpose: Support normal muscle and heart function; help correct hypokalemia.
   Mechanism: Dietary potassium opposes sodium reabsorption and lowers blood pressure modestly; medical monitoring is essential to avoid hyperkalemia when using potassium-sparing drugs. PubMed Central

4. DASH-style eating pattern
   What: Vegetables, fruits, whole grains, low-fat dairy; minimal processed foods.
   Purpose: Blood pressure control and kidney protection.
   Mechanism: Low sodium, higher potassium/magnesium, and fiber improve vascular function and BP—especially helpful in mineralocorticoid-type hypertension. PubMed Central

5. Hydration and heat-illness prevention
   What: Regular fluids; avoid dehydration.
   Purpose: Prevent extra renin-angiotensin-aldosterone activation and kidney stress.
   Mechanism: Steady volume reduces the kidney’s drive to reabsorb sodium aggressively. PubMed Central

6. Routine home blood pressure monitoring
   What: Use a validated cuff; track morning/evening readings.
   Purpose: Catch rises early and guide safe medicine adjustments with the care team.
   Mechanism: More data improves control in resistant hypertension and protects kidneys. PubMed Central

7. Genetic counseling for the family
   What: Meet a genetics professional to discuss inheritance and testing.
   Purpose: Plan for siblings and future pregnancies.
   Mechanism: AME is autosomal recessive; counseling helps families understand carrier status and options. PubMed Central

8. Medication label checks (OTC & herbal)
   What: Review decongestants, NSAIDs, and supplements with clinicians.
   Purpose: Avoid agents that raise BP or hurt kidneys, or that interact with MRAs/eplerenone (CYP3A).
   Mechanism: Some drugs increase sodium retention or potassium; others inhibit CYP3A and raise eplerenone levels. FDA Access Data

9. Weight management and physical activity
   What: Age-appropriate exercise; nutrition to maintain healthy BMI.
   Purpose: Lower BP and reduce cardiovascular risk over time.
   Mechanism: Exercise improves vascular tone; modest weight loss reduces BP in most hypertensive states. PubMed Central

10. Kidney stone/nephrocalcinosis prevention
    What: Hydration, dietary counseling, and thiazide consideration if hypercalciuria is present (the pill part is pharmacologic; the lifestyle parts are not).
    Purpose: Protect kidneys long-term.
    Mechanism: Less calcium in urine (with diet and, if needed, thiazides) lowers stone risk linked to AME. Revista Nefrología

11. Regular kidney function and electrolytes checks
    What: Scheduled labs (creatinine, eGFR, potassium, acid-base status).
    Purpose: Detect progression early and adjust care promptly.
    Mechanism: Tight monitoring prevents dangerous potassium swings from AME and its treatments. PubMed Central

12. School and sports planning
    What: Share a brief plan with teachers/coaches (hydration, symptom awareness).
    Purpose: Keep kids safe and active.
    Mechanism: Early recognition of dizziness or cramps prompts fast care. PubMed Central

13. Pregnancy planning (for adults with AME or carriers)
    What: Pre-conception counseling with nephrology/obstetrics.
    Purpose: Choose BP meds safe in pregnancy and plan close monitoring.
    Mechanism: Mineralocorticoid-type hypertension needs individualized plans before conception. PubMed Central

14. Limit added sugars and ultra-processed foods
    What: Reduce sweetened drinks and packaged snacks.
    Purpose: BP and weight control; avoid stealth sodium.
    Mechanism: Many packaged foods hide salt; sugar-sweetened beverages worsen BP over time. PubMed Central

15. Sleep hygiene and sleep apnea screening if needed
    What: Regular schedule; consider screening if snoring/daytime sleepiness.
    Purpose: Better BP control.
    Mechanism: Treating sleep apnea lowers BP in resistant hypertension. PubMed Central

16. Stress-reduction skills (mind-body)
    What: Breathing, relaxation, or mindfulness practices.
    Purpose: Complement medical therapy and improve adherence.
    Mechanism: Reduces sympathetic tone, modestly aiding BP control. PubMed Central

17. Sun/heat precautions with diuretics
    What: Shade, hats, fluids in hot weather.
    Purpose: Avoid dehydration and electrolyte shifts.
    Mechanism: Heat-related volume loss can worsen AME BP patterns. PubMed Central

18. Food-drug interaction awareness (eplerenone)
    What: Avoid grapefruit and strong CYP3A inhibitors if on eplerenone.
    Purpose: Prevent dangerous rises in drug levels and potassium.
    Mechanism: Eplerenone is metabolized by CYP3A; inhibitors raise exposure. FDA Access Data+1

19. Family screening (BP and electrolytes)
    What: Check siblings/relatives for hypertension and low potassium.
    Purpose: Detect unrecognized cases early.
    Mechanism: Autosomal recessive disorders cluster in families; early care protects kidneys. PubMed Central

20. Clear “sick-day” rules
    What: When to hold/adjust meds during vomiting/diarrhea, and when to get labs.
    Purpose: Avoid sudden potassium or kidney changes.
    Mechanism: Fluid loss + potassium-sparing drugs can be risky; plans keep it safe. PubMed Central

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Drug treatments

Important safety note: Doses below are general adult ranges from drug labels and clinical references for hypertension; AME care must be individualized (especially for children, pregnancy, kidney disease). Always titrate with clinician supervision and frequent potassium/creatinine checks.

1. Eplerenone (MR antagonist)
   Dose/time: 50 mg once daily; may increase to 50 mg twice daily if BP response is inadequate; adjust with CYP3A interactions/CKD. Take at the same time daily.
   Purpose: First-line to block MR more selectively (fewer anti-androgen effects vs spironolactone).
   Mechanism: Blocks cortisol’s inappropriate activation of MR in AME.
   Side effects: Hyperkalemia, kidney function changes; avoid strong CYP3A inhibitors; caution with grapefruit. NCBI+3Drugs.com+3Medscape Reference+3

2. Spironolactone (MR antagonist)
   Dose/time: 25–100 mg/day in 1–2 doses; titrate every ~2 weeks; higher than 100 mg/day usually adds little for BP.
   Purpose: Classic MR blocker to lower BP and correct potassium wasting.
   Mechanism: Direct MR antagonism in kidney.
   Side effects: Hyperkalemia, gynecomastia, menstrual irregularities, GI upset; adjust with CKD. FDA Access Data+2Drugs.com+2

3. Amiloride (ENaC blocker)
   Dose/time: 5–10 mg/day in 1–2 doses (with food); reduce dose in CKD.
   Purpose: Lowers BP and raises potassium by blocking sodium entry in the collecting duct.
   Mechanism: Inhibits ENaC, countering mineralocorticoid effects downstream of MR.
   Side effects: Hyperkalemia (especially with MRAs/ACEI/ARB), GI upset. DailyMed+2Drugs.com+2

4. Triamterene (ENaC blocker)
   Dose/time: (Adult hypertension) commonly 50–100 mg twice daily (often in combo with HCTZ) per standard references.
   Purpose: Alternative ENaC blocker if amiloride not tolerated.
   Mechanism: Same as amiloride—ENaC inhibition.
   Side effects: Hyperkalemia, kidney stones (rare), photosensitivity. Revista Nefrología

5. Hydrochlorothiazide (thiazide diuretic)
   Dose/time: 12.5–25 mg once daily.
   Purpose: Add-on for BP control and to reduce hypercalciuria/nephrocalcinosis risk in AME.
   Mechanism: Distal tubule sodium-chloride cotransporter inhibition; increases calcium reabsorption in kidney.
   Side effects: Low sodium, low potassium (watch closely if combined with MRAs/ENaC blockers), photosensitivity, glucose/lipid effects. Revista Nefrología

6. Low-dose dexamethasone (glucocorticoid)
   Dose/time: Small doses (e.g., 0.25–0.5–1 mg at night) are reported; response is variable.
   Purpose: Reduce ACTH-driven cortisol production so less cortisol reaches MR in the kidney.
   Mechanism: Central suppression of endogenous cortisol synthesis.
   Side effects: Cushingoid features, glucose intolerance, infection risk, growth effects in children—so specialist oversight is essential. PubMed+2SciELO+2

7. Potassium chloride (oral supplementation)
   Dose/time: Individualized (common starting 20–40 mEq/day in divided doses), adjusted by labs.
   Purpose: Correct symptomatic hypokalemia during initiation or adjustment of therapy.
   Mechanism: Replaces potassium lost in urine from mineralocorticoid-like action.
   Side effects: GI irritation, hyperkalemia risk when combined with MRAs/amiloride—close monitoring. PubMed Central

8. Amlodipine (calcium-channel blocker)
   Dose/time: 5–10 mg once daily.
   Purpose: Add-on antihypertensive if BP remains high.
   Mechanism: Arterial vasodilation.
   Side effects: Edema, flushing, headache. (Adjunctive—does not target AME mechanism directly.) FDA Access Data

9. ACE inhibitors (e.g., lisinopril)
   Dose/time: Titrate per standard hypertension dosing.
   Purpose: Add-on BP control and kidney protection if needed.
   Mechanism: Blocks RAAS; helpful even with low renin in some patients.
   Side effects: Cough, angioedema (rare), hyperkalemia—caution with MRAs/amiloride. PubMed Central

10. ARBs (e.g., losartan)
    Dose/time: Titrate per hypertension labeling.
    Purpose: Alternative to ACEI if cough occurs; adjunct for BP control.
    Mechanism: AT1 receptor blockade; renoprotective effects.
    Side effects: Hyperkalemia risk with potassium-sparing agents. PubMed Central

11. Beta-blockers (e.g., atenolol/metoprolol)
    Purpose/mechanism: Reduce heart rate and cardiac output; used when additional BP control is needed.
    Note: Choice is individualized; monitor for fatigue and asthma symptoms. PubMed Central

12. Central agents (e.g., clonidine)
    Purpose/mechanism: Decrease sympathetic tone; short-term bridge in severe hypertension.
    Caution: Sedation, rebound hypertension if stopped abruptly. PubMed Central

13. Loop diuretics (e.g., furosemide)
    Purpose/mechanism: For volume overload or CKD; increase sodium and water excretion.
    Caution: Can worsen potassium loss; typically paired with potassium-sparing agents in AME. PubMed Central

14. Combination amiloride/HCTZ
    Purpose/mechanism: Balances BP lowering and potassium conservation.
    Caution: Dosing must be individualized; still monitor potassium and kidney function closely. Drugs.com

15. Eplerenone (pediatric/CKD adjustments)
    Note: In CKD (eGFR 30–49), some guidance starts 25 mg every other day and titrates cautiously; avoid if eGFR < 30. NCBI

16. Spironolactone (pediatric/CKD adjustments)
    Note: Lower starting doses and slower titration with reduced eGFR to limit hyperkalemia. Follow label cautions. FDA Access Data

17. Dexamethasone—non-responders
    Some genotypes respond poorly to MRAs but do respond to low-dose dexamethasone; others show little benefit. Careful trial only with specialist oversight. PubMed

18. Thiazide add-on specifically for hypercalciuria
    In AME with stones/nephrocalcinosis, thiazides reduce urinary calcium and help protect kidneys. Revista Nefrología

19. Post-transplant antihypertensive plan
    After kidney transplant, BP and electrolytes often normalize, but some patients still need standard BP agents while immunosuppression is adjusted. PubMed+1

20. Careful drug–drug interaction management
    Especially for eplerenone (CYP3A) and potassium-sparing combinations—this is an active part of therapy, not optional. FDA Access Data+1

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Dietary molecular supplements

There are no miracle supplements for AME. A few nutrients can support BP, electrolytes, or kidney health when used with medical therapy and lab monitoring.

1. Potassium chloride (medical supplement, not a vitamin)
   Dose: individualized (often 20–40 mEq/day split), only with clinician guidance.
   Function/mechanism: Replaces potassium losses; helps correct alkalosis.
   Note: High-risk for hyperkalemia when combined with MRAs/amiloride—monitor labs. PubMed Central

2. Magnesium
   Dose: typical 200–400 mg elemental/day if low; adjust to GI tolerance.
   Function: Low magnesium can worsen potassium loss; repletion helps stabilize potassium.
   Mechanism: Supports renal potassium handling; may aid BP modestly. PubMed Central

3. Calcium/Vitamin D (if deficient)
   Function: Bone health in children with chronic alkalosis; address deficiency only—avoid excess.
   Mechanism: Correcting deficiency is good for bones and overall growth; not an AME cure. PubMed Central

4. Omega-3 fatty acids (fish oil)
   Function: Small BP benefit and cardioprotection in general hypertension.
   Mechanism: Vascular and anti-inflammatory effects; adjunct only. PubMed Central

5. Dietary nitrate sources (leafy greens, beet)
   Function: Can give modest BP reductions in general populations.
   Mechanism: Nitric-oxide related vasodilation; food approach is safer than pills. PubMed Central

6. Fiber (psyllium/whole grains)
   Function: Supports weight and BP control; improves metabolic health. PubMed Central

7. Probiotics (food-based, e.g., yogurt)
   Function: Small, variable BP effects; safe as food in most people. PubMed Central

8. CoQ10 (optional)
   Function: Some studies show small BP reductions; discuss with clinician for interactions. PubMed Central

9. Citrus/fruit potassium sources
   Function: Food-first potassium (if safe with your meds) may help BP; monitor labs. PubMed Central

10. Sodium-free flavoring (herbs/spices)
    Function: Helps sustain long-term low-salt eating, a cornerstone in AME care. PubMed Central

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Immunity booster / regenerative / stem-cell drugs

There are no approved immunity boosters, regenerative medicines, or stem-cell drugs to treat Ulick syndrome/AME. The only disease-level “curative” therapy reported is kidney transplantation, which corrects the enzyme defect in the kidney and normalizes cortisol-to-cortisone handling because the transplanted kidney expresses healthy 11β-HSD2. Any claims of stem-cell drugs for AME would be unsupported at this time. New England Journal of Medicine+1

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Procedures / surgeries

1. Kidney transplantation (definitive option in ESRD)
   Procedure: Surgical placement of a donor kidney.
   Why: Replaces the defective renal enzyme with a normal one; corrects the AME defect and often normalizes BP and electrolytes. New England Journal of Medicine+2PubMed+2

2. Arteriovenous fistula or graft creation (if hemodialysis is needed before transplant)
   Why: Provides long-term vascular access for dialysis while awaiting transplant (bridge therapy). PubMed Central

3. Peritoneal dialysis catheter placement (dialysis option before transplant)
   Why: Home dialysis route for kidney failure as a bridge to transplant. PubMed Central

4. Kidney biopsy (diagnostic procedure when indicated)
   Why: Evaluate unexplained kidney decline or suspected nephrocalcinosis-related damage. PubMed Central

5. (Not recommended) Adrenalectomy
   Why not: AME is not due to aldosterone overproduction; removing adrenal glands does not fix the kidney enzyme problem and is generally inappropriate. PubMed Central

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Prevention tips

1. Low-salt diet from early childhood to prevent BP elevation. PubMed Central

2. Avoid all licorice products/herbals that block 11β-HSD2. PubMed Central

3. Regular BP and lab checks (potassium, bicarbonate, creatinine). PubMed Central

4. Family genetic counseling/testing to identify affected or carrier relatives. PubMed Central

5. Hydration and sick-day plans to avoid sudden potassium swings. PubMed Central

6. Weight control and activity to lower lifetime CV risk. PubMed Central

7. Check all medications/supplements with clinicians (CYP3A inhibitors with eplerenone, NSAIDs, decongestants). FDA Access Data

8. Kidney stone prevention if hypercalciuria: fluids, diet, (thiazide if prescribed). Revista Nefrología

9. Sleep-apnea screening in resistant hypertension. PubMed Central

10. Pregnancy planning with high-risk obstetrics and nephrology. PubMed Central

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When to see a doctor (red flags)

• New or worsening high blood pressure, headaches, or vision changes

• Muscle weakness, cramps, or palpitations (possible low or high potassium)

• Reduced urination, swelling, or signs of dehydration

• Children with poor growth or fatigue

• Any fainting, chest pain, or severe headache—seek urgent care

• Before starting any new medicine or supplement, especially if taking eplerenone/spironolactone/amiloride because of potassium risks. PubMed Central

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What to eat and what to avoid

1. Eat: Fresh vegetables, fruits (watch potassium with your doctor), whole grains, beans, lentils. Avoid: Highly salted packaged foods, instant noodles, chips. PubMed Central

2. Choose: Home-cooked meals; use herbs/spices without salt. Avoid: Salt substitutes with potassium unless your clinician approves. PubMed Central

3. Drink: Water regularly. Avoid: Sugary drinks and energy drinks. PubMed Central

4. Include: Low-fat dairy or fortified alternatives. Avoid: Excessive cheese/cured meats (salt). PubMed Central

5. Snack: Unsalted nuts/seeds (if appropriate). Avoid: Salted/roasted nuts. PubMed Central

6. Pick: Fresh poultry/fish. Avoid: Processed meats (sausages, deli meats). PubMed Central

7. Flavor: Lemon, garlic, pepper, herbs. Avoid: Soy sauce, ketchup, pickles (hidden sodium). PubMed Central

8. Be careful with: High-potassium foods if labs already high on MRAs/amiloride—follow your plan. PubMed Central

9. Absolutely avoid: Licorice (candies, teas, herbal tonics). PubMed Central

10. If on eplerenone: Avoid grapefruit and discuss any CYP3A inhibitors with your clinician. FDA Access Data

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Frequently Asked Questions

1) Is Ulick syndrome the same as AME?
Yes. “Ulick syndrome” is the older eponym; “Apparent Mineralocorticoid Excess (AME)” is the modern name. Both mean 11β-HSD2 deficiency due to HSD11B2 variants. PubMed Central

2) Why is aldosterone low if my blood pressure is high?
The kidney “thinks” aldosterone is high because cortisol, not aldosterone, is turning on the MR. As a result, renin and aldosterone are suppressed. PubMed Central

3) How do doctors confirm AME?
Typical picture is early hypertension + hypokalemia + low renin/aldosterone; then steroid metabolite testing and HSD11B2 gene testing confirm it. PubMed Central

4) What medicines work best?
MR blockers (eplerenone, spironolactone) and ENaC blockers (amiloride) address the mechanism; low-dose dexamethasone helps some patients by lowering cortisol production. PubMed Central+1

5) Can a kidney transplant cure AME?
Yes—because the transplanted kidney has normal 11β-HSD2. Many cases show normalization of cortisol metabolism and electrolytes after transplant. New England Journal of Medicine+1

6) Do I need to stop licorice forever?
Yes. Licorice blocks 11β-HSD2 and can dangerously raise BP and lower potassium—even in people without AME. PubMed Central

7) Is there a role for gene or stem-cell therapy now?
No approved therapies yet. Research continues, but the standard of care is medical management and, if needed, kidney transplant. PubMed Central

8) Why might dexamethasone work for some and not others?
Different HSD11B2 mutations and patient factors mean variable response; it lowers cortisol production but doesn’t fix the kidney enzyme itself. PubMed

9) What are the biggest medication risks?
High potassium (especially when combining eplerenone/spironolactone/amiloride or adding ACEI/ARB), kidney function changes, and drug interactions (CYP3A for eplerenone). FDA Access Data

10) Will my child grow normally?
Blood pressure control and potassium correction improve growth outcomes; spironolactone therapy has been associated with improved growth in case series. ScienceDirect

11) Can adults present with AME?
Yes. Some late-onset or “non-classic” cases occur with milder variants. AHA Journals

12) How common is AME in specific populations?
Cases are reported worldwide; clustering has been described in certain groups (e.g., Omani, Native American families), often with consanguinity. Frontiers

13) If my BP is controlled, do I still need labs?
Yes. Potassium and kidney function must be checked regularly, especially on potassium-sparing therapies. PubMed Central

14) Are thiazide diuretics bad for my potassium?
They can lower potassium, so they’re used carefully—but they have a specific role if you have hypercalciuria/nephrocalcinosis. Revista Nefrología

15) Does grapefruit matter?
Yes—avoid it with eplerenone because of CYP3A metabolism (interaction risk). FDA Access Data

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The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: September 21, 2025.