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Living with Pseudohypoaldosteronism, type 2

A condition that causes high potassium and high blood pressure even though the kidneys work normally.

Understanding Pseudohypoaldosteronism, type 2

Pseudohypoaldosteronism type 2 (PHA2) is caused by problems regulating sodium and potassium in the body.

People have high blood pressure and high blood potassium (hyperkalemia) despite normal kidney function. Age at onset varies, from infancy to adulthood. High potassium usually comes first, and high blood pressure develops later. Blood also tends to have extra chloride and acid. Some people have high calcium in the urine.

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • High blood pressure (hypertension)
  • Nausea and vomiting
  • Extreme tiredness
  • Muscle weakness
  • High potassium in the blood (hyperkalemia)
  • Slow growth

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists and kidney specialists.

  3. Ask which tests can help

    Your care team will decide which exams and tests can confirm the diagnosis.

Care & treatment

Care team
Genetic specialists (geneticists); Kidney specialists (nephrologists)
Expert care
Ask about multidisciplinary care centers for rare conditions
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with National Kidney Foundation and other patient organizations
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Would genetic testing help us find the cause?
How should potassium and blood pressure be monitored?
Which specialists, such as a kidney doctor, should I see?

Quick facts

Symptoms usually startany time in life
InheritanceAutosomal Dominant, Autosomal Recessive

GARD lists both autosomal dominant and autosomal recessive inheritance. It doesn't name a gene here.

GeneticKidney

Also known as: chloride shunt syndrome; familial hyperkalemic hypertension; gordon hyperkalemia-hypertension syndrome; hyperkalemia-hypertension syndrome, gordon type; hypertensive hyperkalemia; mineralocorticoid resistant hyperkalemia

Patient organizations

Source: NIH GARD · Pseudohypoaldosteronism, type 2GARD ID 4553 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Pseudohypoaldosteronism, type 2.

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  • Recruiting studies from ClinicalTrials.gov
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  • Eligibility is confirmed by the study team

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