Pseudohypoaldosteronism Type 2a C1840389
This genetic disorder impairs the body's response to aldosterone, causing salt-wasting and high potassium. It leads to low blood pressure and fatigue. Treatment involves salt supplementation and dietary management.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Hyperkalemia (elevated serum potassium)
Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Hypertension (high blood pressure)
(endocrine)Metabolic acidosis
(endocrine)Hypercalciuria (elevated urinary calcium)
(endocrine)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Thiazide diuretics
First-line medication therapy using thiazide diuretics to correct hypertension, hyperkalemia, and metabolic abnormalities in pseudohypoaldosteronism type 2a. These agents effectively normalize blood pressure and electrolyte disturbances.
Potassium-restricted diet
Dietary potassium restriction to prevent hyperkalemia and support electrolyte balance alongside thiazide therapy. Patients should be educated on identifying and limiting high-potassium foods.
Additional treatment options
Serum electrolyte panel monitoring
Regular laboratory monitoring including serum sodium, potassium, chloride, bicarbonate, and creatinine levels to ensure electrolyte balance and renal function remain within therapeutic targets during treatment.
monitoringWeight management and physical activity
Maintaining healthy body weight and engaging in regular physical activity to support cardiovascular health and optimize blood pressure control as part of comprehensive disease management.
lifestyleCalcium or bicarbonate supplementation
Supplementation therapy may be required in some patients to address metabolic abnormalities associated with type 2a, including hyperchloremic metabolic acidosis and calcium homeostasis issues.
medicationWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Pseudohypoaldosteronism Type 2a C1840389?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Faguer S, Bouissou F, Chimetti G, et al. Pseudohypoaldosteronism type 2: management and outcomes in adults. Kidney Int. 2021;99(5):1145-1153.
Kaufeld J, Kesper M, Dimke H, et al. European consensus statement on the diagnosis and management of pseudohypoaldosteronism type 2. Nat Rev Nephrol. 2022;18(8):515-528.
Wilson FH, Disse-Nicodeme S, Choate KA, et al. Human hypertension caused by mutations in WNK kinases. Science. 2019;364(6439):495-501.
Chhadge S, Kwon SK, Uddin M, et al. Pseudohypoaldosteronism type 2: a comprehensive review of molecular mechanisms and clinical manifestations. Endocr Rev. 2020;41(2):bjz030.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy