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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.

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

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

Transmitted as an autosomal dominant trait, but genetically heterogeneous.

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.

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

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

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.

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.

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.

Gereda JE, Bonilla-Felix M, Kalil B, et al: Neonatal presentation of Gordon syndrome. J Pediatr 129:615, 1996.  [PubMed: 8859273]
Mansfield TA, Simon DB, Farfel Z, et al: Multilocus linkage of familial hyperkalaemia and hypertension, pseudohypoaldosteronism type II, to chromosomes 1q31-42 and 17p11-q21. Nat Genet 16:202, 1997.  [PubMed: 9171836]

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