eMedicine Specialties > Pediatrics: General Medicine > Endocrinology
Pseudohypoaldosteronism: Treatment & Medication
Updated: Jul 11, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Patients with hypovolemia and shock should receive fluid resuscitation with isotonic sodium chloride solution at 20 mL/kg over 30-60 minutes. Fluid bolus may be repeated until signs of improved perfusion to vital organs are observed. Patients with severe hyperkalemia should receive intravenous calcium gluconate 10% (0.5-1 mL/kg) to protect the heart muscle and sodium bicarbonate to shift potassium intracellularly until cation exchange resins start to lower serum potassium. The use of glucose (0.5-1 g/kg) and insulin (0.1 U/kg) intravenously over 30 minutes should also be considered in severe hyperkalemia.
- Renal pseudohypoaldosteronism type I (PHA-I): Patients with renal PHA-I have a characteristic lack of improvement despite administration of large doses of mineralocorticoids. Therapy consists of fluid and sodium supplementation, with requirements being higher early in infancy and tending to diminish over time. Large doses may be necessary to correct serum electrolyte abnormalities. Sodium chloride supplementation is followed by significant clinical improvement and correction of electrolyte abnormalities. Expansion of ECF increases the renal tubular flow and sodium chloride delivery to the distal nephron, thus creating a favorable gradient for secretion of potassium despite the lack of mineralocorticoid action.
- Multiple target organ defects (MTOD) PHA-I: Although administration of exogenous mineralocorticoids is ineffective in correcting the abnormalities in this disorder, ingestion of a high-sodium and low-potassium diet is generally effective in preventing volume depletion and in partially reducing, but not completely correcting, the hyperkalemia. Patients may require oxygen for episodes of dyspnea and cyanosis associated with lower respiratory tract infections.
- PHA-II: Restriction of dietary sodium in some patients has resulted in normalization of blood pressure, plasma potassium, aldosterone, renin, and urinary calcium levels. However, correction of acidosis with bicarbonate does not correct the hyperkalemia.
Surgical Care
- No surgical management is needed in most cases.
Consultations
- Endocrinologist
- Nephrologist
Diet
- Renal PHA-I: Sodium chloride supplementation during infancy can reverse hyponatremia and hyperkalemia, improve symptoms, and permit improved growth. Ingestion of a high-sodium (10-15 mEq/kg/d) and low-potassium (0.6 mEq/kg/d) diet is generally effective in preventing both volume depletion and hyperkalemia. After infancy, reduction or discontinuation of sodium chloride supplementation is possible when patients develop an appetite for salt and are asymptomatic while eating a normal diet. Symptoms may recur with salt restriction in older children and adults.
- MTOD PHA-I: Dietary sodium supplementation (10-15 mEq/kg/d) and a low-potassium (0.6 mEq/kg/d) diet are recommended. Patients have a poor response to sodium chloride supplementation alone.
- PHA-II: Dietary sodium and potassium restriction may correct the hyperkalemia and acidosis.
Activity
- No restrictions are necessary once adequate replacement therapy is instituted.
Medication
Alkalinizing agents
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
Preferred choice of alkali therapy because it is inexpensive, easy to prepare, and does not have to be metabolized by the liver. Unfortunately, commercially available PO sodium bicarbonate is available only in 325-mg (ie, 5 grains) and 650-mg (ie, 10 grains) tabs, which provide 4 and 8 mEq/tab, respectively. These tabs can be crushed and added to food or diluted in water to yield a concentration of 1 mEq of bicarbonate per 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. May also administer appropriate concentration of IV product PO.
Adult
Not applicable; PHA-I only occurs in newborns and infants
Pediatric
3-6 mEq/kg/d PO divided tid/qid
Urinary alkalinization (induced by increased sodium bicarbonate concentrations) may decrease serum levels of lithium, tetracyclines, chlorpropamide, methotrexate, and salicylates; increases levels of amphetamines, pseudoephedrine, flecainide, anorexiants, mecamylamine, ephedrine, quinidine, and quinine
Respiratory alkalosis; hypocalcemia; hypochloremia; hypernatremia; severe pulmonary edema; unknown abdominal pain
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
History of congestive heart failure or renal impairment; hypertension; concurrent corticosteroids; monitor acid-base balance; monitor electrolytes and pH; PO ingestion has caused gastric rupture from gas production
Citric acid and sodium citrate (Bicitra, Oracit)
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. Provides 1 mEq of sodium bicarbonate per mL. Potassium citrate solutions such as Polycitra and Polycitra-K have no use in PHA and should be avoided.
Adult
Not applicable; PHA-I only occurs in newborns and infants
Pediatric
3-6 mEq (3-6 mL)/kg/d of alkali PO divided tid/qid
Decreases therapeutic levels of lithium, chlorpropamide, methotrexate, tetracyclines, and salicylates because of urinary alkalinization; increases toxicity of aluminum hydroxide, amphetamines, pseudoephedrine, ephedrine, quinine, and quinidine because of urinary alkalinization
Metabolic alkalosis; hypocalcemia; acute dehydration; myocardial damage
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Sodium citrate may exacerbate conditions that require sodium restriction (ie, congestive heart failure, hypertension, pulmonary or peripheral edema, toxemia of pregnancy); increases risk of urolithiasis because of increased renal calcium excretion
Potassium-binding resins
These agents may be used in patients with PHA to successfully control hyperkalemia.
Sodium polystyrene sulfate (Kayexalate)
May be required in patients with MTOD PHA-I to control hyperkalemia. The resin partially releases the sodium ions in the large intestine, and these are replaced mole for mole by potassium ions.
Adult
15 g PO in water or 70% sorbitol qd/qid
Alternatively, 60 g PR q6H as retention enema (30-60 min)
Pediatric
1 g/kg/dose PO/NG q6h
Alternatively, may administer PR as retention enema q6h
Systemic alkalosis may occur if administered concurrently with magnesium hydroxide, aluminum carbonate or similar antacids, and laxatives
Documented hypersensitivity; hypokalemia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Nausea, vomiting, electrolyte abnormalities, hypocalcemia, constipation, and colonic necrosis
Prostaglandin inhibitors
These agents inhibit the production of prostaglandin by blocking the action of cyclooxygenase (also called prostaglandin synthetase).
Indomethacin (Indocin, Indochron E-R)
Has been used in selected cases of MTOD PHA-I and is thought to decrease urinary volume and sodium excretion. Response varies, and some patients may not benefit. Most patients with MTOD PHA-I continue to require sodium supplementation.
Adult
25 mg PO bid/tid; not to exceed 200 mg/d
Pediatric
1-3 mg/kg/d PO divided tid; not to exceed 4 mg/kg/d or 200 mg/d
Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Documented hypersensitivity; asthma; GI bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Category D in third trimester of pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur (discontinue if persistent leukopenia, granulocytopenia, or thrombocytopenia is present)
Diuretic agents
These agents are used to increase the rate of urine formation and output, thus eradicating fluid overload and controlling hypertension.
Furosemide (Lasix)
Loop diuretic that has been effective in the treatment of PHA-II.
Adult
20-80 mg/d PO
20-40 mg/d IV/IM
Pediatric
2-5 mg/kg/d PO divided bid/tid
Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently with this medication
Documented hypersensitivity; anuria; PHA-I (because increased diuresis can exacerbate hypovolemia)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypokalemia, hyperuricemia, hypotension, and hyperglycemia; can intensify hypercalciuria in PHA-II, increasing the risk of nephrolithiasis
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.
Adult
25-100 mg/d PO
Pediatric
1-5 mg/kg/d PO divided bid
May decrease effects of anticoagulants, antigout agents, and sulfonylureas; may increase toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, loop diuretics, lithium, diazoxide, digitalis, amphotericin B, and nondepolarizing muscle relaxants
Documented hypersensitivity; anuria; renal decompensation
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypokalemia, hypovolemia, hyperuricemia, SLE, and salt wastage
More on Pseudohypoaldosteronism |
| Overview: Pseudohypoaldosteronism |
| Differential Diagnoses & Workup: Pseudohypoaldosteronism |
 Treatment & Medication: Pseudohypoaldosteronism |
| Follow-up: Pseudohypoaldosteronism |
| References |
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Further Reading
Keywords
pseudohypoaldosteronism, PHA, pseudohypoaldosteronism type I, PHA-I, Cheek and Perry syndrome, renal pseudohypoaldosteronism type I, AD renal PHA-I, multiple target organ pseudohypoaldosteronism, MTOD PHA-I, autosomal recessive PHA-I, AR PHA-I, early childhood hyperkalemia, renal tubular acidosis subtypes 4 and 5, RTA, pseudohypoaldosteronism type II, PHA-II, Gordon syndrome, adolescent hyperkalemic syndrome, Spitzer-Weinstein syndrome, mineralocorticoid-resistant hyperkalemia, renal tubular acidosis type IV subtype 3, metabolic acidosis, hypervolemia, renal salt wasting, hypotension, hypertension, chloride shunt syndrome, renal tube defects, short stature, urolithiasis, obstructive uropathy, urinary tract infection, tubulointerstitial nephritis, sickle cell nephropathy, systemic lupus erythematosus, amyloidosis, neonatal medullary necrosis, unilateral renal vein thrombosis, failure to thrive, adolescent hyperkalemic syndrome
