Atypical Presentation of Gordon Syndrome and Its Management: A Report of Three Patients

Sushil Mehandru, Attiya Haroon, Vikas Singh, Avais Masud, Eric J. Costanzo, Mayurkumar Patel, Elmer Sadiang-Abay, Arif Asif


Gordon syndrome (GS) usually presents in children with hyperkalemia, hypertension and hyperchloremic metabolic acidosis. However, it has been reported in adults, where diagnosis can be missed in the absence of vigilance for electrolytes and acid base abnormalities. GS is traditionally managed with hydrochlorothiazide and restricted sodium diet. We report adult patients with GS, who were successfully treated with a potassium-restricted diet. Three female patients (aged 70, 61, and 79 years) presented to the clinic with untreated isolated hyperkalemia for several years. Patients did not complain of headache, palpitation, chest pain, polyuria or hematuria. Upon examination, two patients were normotensive and one patient had hypertension (150/90 mm Hg). Laboratory analysis revealed hyperkalemia (6.2, 6.1 and 6.2 mEq/L), serum bicarbonate level of 30, 27 and 28 mEq/L, hyperchloremia (108, 107 and 94 mEq/L) and serum normal creatinine (0.90, 0.8 and 0.78 mg/dL), based upon normal reference lab values. Plasma aldosterone and renin were normal. Based on patients history, physical examination, laboratory findings and absence of other causes of hyperkalemia such as the history of the use of NSAID, ACE inhibitors (angiotensin receptor blockers, aldosterone receptor blockers, and beta-blockers), and the absence of diabetes mellitus and other causes of hyporeninemic hypoaldosteronism, a diagnosis of GS was made. All three patients were treated with a restricted potassium diet contrary to a low sodium diet and hydrochlorothiazide. A 4-week follow-up showed normal serum potassium levels (4.2, 4.6 and 4.9 mEq/L) in all three patients. While GS has been reported previously in adults and can present with normal blood pressure, its management by a restricted potassium diet has not been reported, to the best of our knowledge. GS should be included in the differential diagnosis of adult patients presenting with unexplained hyperkalemia.

J Med Cases. 2017;8(8):252-255


Gordon syndrome; Pseudohypoaldosteronism; Hyperkalemia

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