Hypoaldosteronism medical therapy: Difference between revisions

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Isolated Hypoaldosteronism: Isolated Hypoaldosteronism from CYP11B2 gene mutation presents in infancy and is treated with 9α-fludrocortisone. In adults treatment is not necessary.  
Isolated Hypoaldosteronism: Isolated Hypoaldosteronism from CYP11B2 gene mutation presents in infancy and is treated with 9α-fludrocortisone. In adults treatment is not necessary.  
Pseudohypoaldosteronism type I: Patients of pseudohypoaldosteronism are resistant to aldosterone or mineralocorticoid therapy and treatment is based on correcting the underlying electrolyte abnormalities with sodium chloride (2 to 8 g/day) and cation-exchange resins.
Thiazide diuretics are used to treat hyperkalemia. In patients with severe hyperkalemia peritoneal dialysis may be done.
Pseudohypoaldosteronism decreases after few years and the therapy may be discontinued. However, these patients require salt supplementation till first 3-4 years of life.





Revision as of 18:27, 22 August 2017

Hypoaldosteronism Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Medical Therapy

  • Pharmacologic medical therapy for hypoaldosteronism include:
    • Fludrocortisone 0.05 to 0.1 mg PO qd in patients with aldosterone deficiency.
    • 0.9% saline for underlying hypovolemia.
    • Furosemide 20 to 40 mg qd to control hyperkalemia.


Disease Name

Patients of hypoaldosteronism have increased levels of potassium which can alter the electrical activity of heart. Therefore ECG should be first obtained to check for any life threatening rhythm.

Hyperreninemic hypoaldosteronism: Secondary Isolated Hypoaldosteronism also known as hyperreninemic hypoaldosteronism is seen in patients with severe underlying illness such as liver cirrhosis or heart failure. The primary focus of the treatment is to treat the underlying condition. Decreased level of aldosterone in patients of hyperreninemic hypoaldosteronism does not lead to any clinical complications and is therefore seldom treated.

Hyporeninemic Hypoaldosteronism: Treatment is aimed at normalizing volume status, plasma potassium and aldosterone levels.

    • Thiazide diuretics: Diuretics are the first-line therapy for patients with severe hyperkalemia and fluid overload (seen in renal impairment or congestive heart failure).
      • Avoid diuretics in patients with signs of hypotension or volume depletion. In these patients sodium bicarbonate (NaHCO3) can be used to increase distal delivery of bicarbonate anion and increase urinary potassium excretion. Sodium bicarbonate (NaHCO3) also corrects underlying metabolic acidosis.
      • Sodium polystyrene sulfonate: It may be used in patients with underlying renal disease and decreased potassium excretion. 1gm of sodium polystyrene sulfonate can remove upto 1 mEq of potassium.
    • Aldosterone analogue: Fludrocortisone in the dose of 0.1-0.3 mg/day.


Isolated Hypoaldosteronism: Isolated Hypoaldosteronism from CYP11B2 gene mutation presents in infancy and is treated with 9α-fludrocortisone. In adults treatment is not necessary.

Pseudohypoaldosteronism type I: Patients of pseudohypoaldosteronism are resistant to aldosterone or mineralocorticoid therapy and treatment is based on correcting the underlying electrolyte abnormalities with sodium chloride (2 to 8 g/day) and cation-exchange resins. Thiazide diuretics are used to treat hyperkalemia. In patients with severe hyperkalemia peritoneal dialysis may be done. Pseudohypoaldosteronism decreases after few years and the therapy may be discontinued. However, these patients require salt supplementation till first 3-4 years of life.


Primary or secondary insufficiency: Use fludrocortisone 0.1 mg daily Reduce dose to 0.05 mg daily if transient hypertension develops Maintenance dosage range: 0.1 mg 3 times weekly to 0.2 mg daily Preferred administration with cortisone or hydrocortisone

Alternate recommendations for primary adrenal insufficiency: Use initial: 0.05 to 0.1 mg PO qd (in combination with hydrocortisone or cortisone). Maintenance dose: 0.05 to 0.2 mg once daily. If hypertension develops, Dose reduction is suggested if hypertension develops Antihypertensive may be necessary in case of uncontrolled hypertension.

Congenital adrenal hyperplasia (21-hydroxylase deficiency): Oral: 0.1 to 0.2 mg daily in combination with hydrocortisone

Orthostatic hypotension Oral: Initial: 0.1 mg daily in conjunction with a high-salt diet and adequate fluid intake May be increased in increments of 0.1 mg per week Maximum dose: 1 mg daily. Note: Doses exceeding 0.3 mg daily may not be beneficial and predispose patient to unwanted side effec

  • 1 Stage 1 - Name of stage
    • 1.1 Primary or secondary adrenal insufficiency

Preferred administration with cortisone or hydrocortisone. (Reduce dose to 0.05 mg daily if transient hypertension develops, maintenance dosage range: 0.1 mg 3 times weekly to 0.2 mg daily)

        • Preferred regimen (2): drug name 500 mg PO q8h for 14-21 days
        • Preferred regimen (3): drug name 500 mg q12h for 14-21 days
        • Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
        • Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
        • Alternative regimen (3): drug name 500 mg PO q6h for 14–21 days
      • 1.1.2 Pediatric
        • 1.1.2.1 (Specific population e.g. children < 8 years of age)
          • Preferred regimen (1): drug name 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
          • Preferred regimen (2): drug name 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
          • Alternative regimen (1): drug name10 mg/kg PO q6h (maximum, 500 mg per day)
          • Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
          • Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
        • 1.1.2.2 (Specific population e.g. 'children < 8 years of age')
          • Preferred regimen (1): drug name 4 mg/kg/day PO q12h(maximum, 100 mg per dose)
          • Alternative regimen (1): drug name 10 mg/kg PO q6h (maximum, 500 mg per day)
          • Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
          • Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
    • 2.1 Specific Organ system involved 2
      • 2.1.1 Adult
        • Preferred regimen (1): drug name 500 mg PO q8h
      • 2.1.2 Pediatric
        • Preferred regimen (1): drug name 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
  • 2 Stage 2 - Name of stage
    • 2.1 Specific Organ system involved 1
      Note (1):
      Note (2):
      Note (3):
      • 2.1.1 Adult
        • Parenteral regimen
          • Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
          • Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
          • Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
        • Oral regimen
          • Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
          • Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
          • Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
          • Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
          • Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
          • Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
      • 2.1.2 Pediatric
      • 2.2.1 Adult
        • Parenteral regimen
          • Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
          • Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
          • Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
        • Oral regimen
          • Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
          • Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
          • Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
          • Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
          • Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
          • Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
      • 2.2.2 Pediatric

e)

          • Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)

References

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