Gordon Hyperkalemia-Hypertension Syndrome

At a glance

Rare form of familial hypertension. Syndrome is characterized by hyperkalemia, metabolic acidosis, suppressed plasma renin activity, and hyperchloremia, but no renal failure. Positive effects of thiazide diuretics are associated.

Synonyms

Hypertensive Hyperkalemia; Familial Hyperpotassemia Hypertension Pseudohypoaldosteronism Type II Syndrome; PHA II Syndrome.

History

Genetic disorder first described by Paver and Pauline in 1964 and singularized by Gordon in 1970.

Genetic inheritance

Transmitted as an autosomal dominant trait, but genetically heterogeneous.

Pathophysiology

Caused by mutations in WNK4 (a serine threonine protein kinase) (17q21) or WNK1 (a lysine deficient protein kinase) (12p). An additional locus is probably located on 1q. A resistance to aldosterone regarding potassium, but not sodium transport, and a generalized cellular defect in transmembrane potassium transport, has been evocated to explain this disease.

Diagnosis

Based on the clinical findings (hyperkalemia, metabolic acidosis, absent plasma renin activity, hyperchloremia) in a patient with hypertension, already presenting in childhood.

Clinical aspects

Besides the main features, muscular weakness and periodic paralysis have been described.

Precautions before anesthesia

Hyperkalemia is a specific electrolyte disturbance that has numerous implications for the administration of an anesthetic. Evaluate renal function and electrolytes. Arterial blood gas analysis can be useful. Evaluate cardiac function because of hypertension and hyperkalemia (clinical, ECG, echocardiography). Check for signs of muscle weakness.

Anesthetic considerations

Perioperative cardiac monitoring (including invasive arterial blood pressure measurement if necessary) has to be considered. Hyperkalemia has to be corrected before surgery. Central regional anesthesia should be considered.

Pharmacological implications

Both insulin and bicarbonates have no effect on hyperkalemia. Thiazide diuretics can treat main features. Potassium-free intravenous solutions with reduced sodium concentration might be preferable.

References