Rare diseases · Sign or symptom
Hyperkalemia
Increased circulating potassium concentration
HP:0002153
What it means
The concentration of potassium(1+) in the blood circulation is above the upper limit of normal.
Rare diseases that can present with this27
Very common80–99%
7- 46,XY difference of sex development-adrenal insufficiency due to CYP11A1 deficiency
- Early-onset familial hypoaldosteronism
- Familial hypoaldosteronism
- Familial pseudohyperkalemia
- Generalized pseudohypoaldosteronism type 1
- Inherited isolated adrenal insufficiency due to partial CYP11A1 deficiency
- Pseudohypoaldosteronism type 2
Common30–79%
8- Acute adrenal insufficiency
- Addison disease
- Classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency
- Congenital adrenal hyperplasia due to 3-beta-hydroxysteroid dehydrogenase deficiency
- Congenital lipoid adrenal hyperplasia due to STAR deficency
- Familial glucocorticoid deficiency
- Hyperkalemic periodic paralysis
- X-linked adrenal hypoplasia congenita
Sometimes5–29%
11- Ethylene glycol poisoning
- Exercise-induced malignant hyperthermia
- Genetic recurrent myoglobinuria
- Glycogen storage disease due to aldolase A deficiency
- Hemorrhagic fever-renal syndrome
- Infection-related hemolytic uremic syndrome
- Late-onset familial hypoaldosteronism
- Lysosomal acid lipase deficiency
and 3 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions, and Orphanet separately records 4 diseases where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Elevated potassium(1+) concentration · Elevated potassium(1+) level · Elevated potassium(1+) level in blood · Increased potassium(1+) concentration · Increased potassium(1+) level · Increased potassium(1+) level in blood
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.