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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.

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

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

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Transmitted as an autosomal dominant trait, but genetically heterogeneous.

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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.

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

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

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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.

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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.

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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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