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Preparing your E-Poster
Please review the E-Poster format requirements carefully when preparing your E-Poster. Should your E-Poster not meet the mentioned requirements, it may not be displayed as described above.
E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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Abstract titles should be brief and reflect the content of the abstract.
Hyperkalemia is an electrolyte abnormality that can be life-threatening in severe cases. The complication rate of hyperkalemia increases when renal function is impaired, but pseudohyperkalemia should be suspected when renal function is normal. Familial pseudohyperkalemia is a cause of pseudohyperkalemia, but it is a relatively rare disease with only two cases reported in Japan. Here, we report a case of familial pseudohyperkalemia that was genetically diagnosed.
The patient was a woman in her 20s who visited our hospital due to a potassium level of 9.3 mmol/L at another clinic despite normal renal function and no symptoms. Potassium level was in the normal range when we measured at our own hospital. We suspected familial pseudohyperkalemia as one of the diagnoses. To confirm our diagnosis, we measured the patient’s blood in the following conditions. Samples were stored as whole blood in tubes with a separating agent, and storage conditions were set at 4 degrees, 23 degrees, and 37 degrees Celsius. And potassium levels were measured after 0 hours, 1 hour, 3 hours, and 6 hours, respectively.
A sample stored at 4 degrees Celsius for 6 hours showed a significant increase in potassium levels by 8.9 mmol/L. Her mother, in her 60s, also showed a similar increase in potassium level at low temperatures, and genetic testing revealed a mutation in the ABCB6 gene (c1123C>T), leading to a diagnosis of familial pseudohyperkalemia. Furthermore, we showed that erythrocyte morphology does not change even at low temperatures, the potassium level does not increase at 32 degrees Celsius, and the risk of patients with this disease developing hyperkalemia even if they undergo hypothermia therapy is low.
Patients with familial pseudohyperkalemia may develop hypokalemia due to inappropriate potassium-lowering therapy. This disease should be considered in cases of hyperkalemia with normal renal function or discrepancies in serum potassium between outsourced and in-house tests.
