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Pseudohypoaldosteronism Medication

  • Author: Alicia Diaz-Thomas, MD, MPH; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: Apr 30, 2014
 

Medication Summary

Drugs used in the management of pseudohypoaldosteronism (PHA) include alkalizing agents, potassium-binding resins, prostaglandin inhibitors, and diuretics.

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Alkalinizing agents

Class Summary

These agents are used for correcting acidosis in children with early childhood hyperkalemia during the first few years of life. Correction of acidosis in pseudohypoaldosteronism type II (PHA-II) does not correct the hyperkalemia.

Sodium bicarbonate (Neut, Brioschi)

 

Sodium bicarbonate is preferred for alkali therapy because it is inexpensive and easy to prepare and does not have to be metabolized by the liver. Unfortunately, sodium bicarbonate is commercially available for oral use only in 325-mg (ie, 5-grain) and 650-mg (ie, 10-grain) tablets, which provide 4 mEq and 8 mEq per tablet, respectively. These tabs can be crushed and added to food or diluted in water to yield a bicarbonate concentration of 1 mEq/mL.

An alternative is to mix an 8-oz box of baking soda in 2.88 L of distilled water to produce a concentration of 1 mEq/mL. It is also feasible to administer an appropriate concentration of the intravenous (IV) product orally.

Citric acid and sodium citrate (Bicitra, Oracit)

 

Citric acid and sodium citrate are systemic alkalinizing agents that have been used to correct the acidosis in PHA; however, they are metabolized by the liver to bicarbonate. Bicitra is extensively used rather than Shohl solution because it does not require mixing by the pharmacist. It provides 1 mEq of sodium bicarbonate per milliliter. Potassium citrate solutions such as Polycitra and Polycitra-K have no use in PHA and should be avoided.

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Antidotes, Other

Class Summary

Potassium-binding resins may be used to control hyperkalemia in patients with PHA.

Sodium polystyrene sulfonate (Kayexalate, Kalexate, Kionex, SPS)

 

Sodium polystyrene sulfonate may be required for control of hyperkalemia in patients with multiple target organ defects (MTOD) PHA type I (PHA-I). The resin partially releases the sodium ions in the large intestine, and these are replaced mole for mole by potassium ions.

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NSAIDs

Class Summary

Prostaglandin inhibitors, like NSAIDs, inhibit the production of prostaglandin by blocking the action of cyclooxygenase (also called prostaglandin synthetase).

Indomethacin (Indocin)

 

Indomethacin has been used in selected cases of MTOD PHA-I and is thought to decrease urinary volume and sodium excretion. Response to indomethacin varies, and some patients may not benefit. Most patients with MTOD PHA-I continue to require sodium supplementation.

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Diuretics, Loop

Class Summary

Diuretics are used to increase the rate of urine formation and output, thereby eradicating fluid overload and controlling hypertension.

Furosemide (Lasix)

 

Furosemide is a loop diuretic that has been effective in the treatment of PHA-II.

Hydrochlorothiazide (Esidrix, HydroDIURIL, Microzide)

 

Thiazide diuretic that has been used occasionally to correct hyperkalemia and hypercalciuria in MTOD PHA-I; however, thiazides should be used with caution because they can exacerbate hypovolemia and salt wastage. Preferred treatment in patients with PHA-II because it can correct hyperkalemia, metabolic acidosis, hypertension, and plasma aldosterone and plasma renin levels. Unlike furosemide, it can also correct hypercalciuria. Does not result in catch-up growth in patients with PHA-II.

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Diuretics, Thiazide

Class Summary

Diuretics are used to increase the rate of urine formation and output, thereby eradicating fluid overload and controlling hypertension.

In general, thiazides should be used with caution, because they can exacerbate hypovolemia and salt wastage.

Hydrochlorothiazide (Microzide)

 

Hydrochlorothiazide is a thiazide diuretic that has occasionally been used to correct hyperkalemia and hypercalciuria in patients with MTOD PHA-I.

Hydrochlorothiazide is the preferred treatment in patients with PHA-II because it can correct hyperkalemia, metabolic acidosis, hypertension, and plasma renin and aldosterone levels. Unlike furosemide, it can also correct hypercalciuria. It does not result in catch-up growth in patients with PHA-II.

Chlorothiazide (Diuril)

 

Chlorothiazide inhibits the reabsorption of sodium in distal tubules, causing increased excretion of sodium and water, as well as of potassium and hydrogen ions.

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Contributor Information and Disclosures
Author

Alicia Diaz-Thomas, MD, MPH Assistant Professor of Pediatrics, University of Tennessee Health Science Center

Alicia Diaz-Thomas, MD, MPH is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Pediatric Endocrine Society, Tennessee Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Jose F Pascual-Y-Baralt, MD Chief, Division of Pediatric Nephrology, San Antonio Military Pediatric Center; Clinical Professor, Department of Pediatrics, University of Texas School of Medicine at San Antonio

Jose F Pascual-Y-Baralt, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, American Society of Pediatric Nephrology, Association of Military Surgeons of the US, International Society of Nephrology

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Robert J Ferry Jr, MD Le Bonheur Chair of Excellence in Endocrinology, Professor and Chief, Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, University of Tennessee Health Science Center

Robert J Ferry Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society

Disclosure: Eli Lilly & Co Grant/research funds Investigator; MacroGenics, Inc Grant/research funds Investigator; Ipsen, SA (formerly Tercica, Inc) Grant/research funds Investigator; NovoNordisk SA Grant/research funds Investigator; Diamyd Grant/research funds Investigator; Bristol-Myers-Squibb Grant/research funds Other; Amylin Other; Pfizer Grant/research funds Other; Takeda Grant/research funds Other

Lynne Lipton Levitsky, MD Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor of Pediatrics, Harvard Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds Other; Eli Lily Grant/research funds PI; NovoNordisk Grant/research funds PI

Arlan L Rosenbloom, MD Adjunct Distinguished Service Professor Emeritus of Pediatrics, University of Florida College of Medicine; Fellow of the American Academy of Pediatrics; Fellow of the American College of Epidemiology

Arlan L Rosenbloom, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Epidemiology, American Pediatric Society, Endocrine Society, Florida Pediatric Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References
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  4. Hogg R, Marks J, Marver D, Frolich J. Long-term observation in a patient with pseudohypoaldosteronism. Pediatr Nephrol. 1991. 5:205-210. [Medline].

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  8. Sheridan MB, Fong P, Groman JD, et al. Mutations in the beta-subunit of the epithelial Na+ channel in patients with a cystic fibrosis-like syndrome. Hum Mol Genet. 2005. 14:3493-3498. [Medline].

  9. Adachi M, Asakura Y, Muroya K, Tajima T, Fujieda K, Kuribayashi E, et al. Increased Na reabsorption via the Na-Cl cotransporter in autosomal recessive pseudohypoaldosteronism. Clin Exp Nephrol. 2010 Apr 8. [Medline].

  10. Sartorato P, Lapeyraque AL, Armanini D, et al. Different inactivating mutations of the mineralocorticoid receptor in fourteen families affected by type I pseudohypoaldosteronism. J Clin Endocrinol Metab. 2003 Jun. 88(6):2508-17. [Medline].

  11. Boyden LM, Choi M, Choate KA, et al. Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities. Nature. 2012 Jan 22. 482(7383):98-102. [Medline]. [Full Text].

  12. Chang SS, Grunder S, Hanukoglu A, et al. Mutations in subunits of the epithelial sodium channel cause salt wasting with hyperkalaemic acidosis, pseudohypoaldosteronism type 1. Nat Genet. 1996 Mar. 12(3):248-53. [Medline].

  13. Mastrandrea LD, Martin DJ, Springate JE. Clinical and biochemical similarities between reflux/obstructive uropathy and salt-wasting congenital adrenal hyperplasia. Clin Pediatr (Phila). 2005. 44:809-812. [Medline].

  14. Perimenis P, Wemeau JL, Vantyghem MC. Hypercalciuria [French]. Ann Endocrinol (Paris). 2005. 66:532-539. [Medline].

 
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Renin angiotensin aldosterone system
Table. Characteristics of Primary Pseudohypoaldosteronism (Types I and II)
Details PHA Type I PHA Type II
Renal PHA-I MTOD PHA-I Early Childhood Hyperkalemia PHA-II
Synonyms Classic PHA of infancy, Cheek and Perry syndrome, autosomal dominant PHA-I, subtype 4 RTA IV Autosomal recessive PHA-I Subtype 5 RTA IV Adolescent hyperkalemic syndrome, Spitzer-Weinstein syndrome, subtype 3 RTA IV Gordon syndrome, mineralocorticoid-resistant hyperkalemia, chloride shunt syndrome
Age Newborn period, infancy Newborn period, infancy Infancy, childhood Childhood Adulthood
Organs Kidney Kidney, sweat glands, salivary glands, colon Kidney Kidney Kidney
Genetics Autosomal dominant, sporadic Autosomal recessive, sporadic Unknown Unknown Autosomal dominant, sporadic
Mechanism Heterozygous MLR mutations (possible) Defective Na transport in organs that contain ENaC Maturation disorder in the number or function of aldosterone receptors Chloride shunt Chloride shunt
Serum potassium High High High High High
Acidosis Present Present Present Present Present
Serum sodium Normal or low Normal or low Normal Normal Normal
PRA* High High Normal or high Normal or low Low
Aldosterone High High Normal or high Normal or low Low
Blood volume Normovolemia, hypovolemia Normovolemia, hypovolemia Normovolemia Hypervolemia Hypervolemia
Blood pressure Normal or low Normal or low Normal or low Normal or low Normal or low
GFR Normal Normal Normal Normal Normal
Salt wasting Renal Renal, sweat or salivary glands, colon Absent Absent Absent
Hypercalciuria Present or absent Absent Absent Present Present
Therapy Na supplementation, K-binding resins High-Na, low-K diet, K-binding resins, hydrochlorothiazide Na bicarbonate, K-binding resins Dietary Na restriction, hydrochlorothiazide Dietary Na restriction, hydrochlorothiazide
Prognosis Outgrow by age 2 y Lifelong therapy Outgrow by age 5 y Lifelong therapy Lifelong therapy
*Plasma renin activity.



ENaC = epithelial sodium channel; GFR = glomerular filtration rate; MLR = mineralocorticoid receptor gene; PHA = pseudohypoaldosteronism; RTA = renal tubular acidosis.



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