title: Effect of hydrochlorothiazide in pseudohypoaldosteronism with hypercalciuria and severe hyperkalemia.
abstract: Severe hyperkalemia resistant to treatment with sodium chloride (NaCl) supplements plus cation exchange resins can be found in pseudohypoaldosteronism type I. In a patient with the multiple target organ variant of this condition, hyperkalemia persisted at dangerous levels (8.5 mmol/l) despite large doses of NaCl (50 mmol/kg per day) and cation exchange resins (6 g/kg per day). Hypercalciuria was also present. The total volume of fluids and supplements required was not tolerated orally. Indomethacin (2 mg/kg per day) and later hydrochlorothiazide (2 mg/kg per day) were tried to further correct imbalance. Plasma potassium (K) and Na levels, the urinary Na/K ratio, transtubular potassium gradient (TTKG), and urinary calcium/creatinine (Ca/Cr) ratio were used to evaluate the effect of hydrochlorothiazide. Under treatment, plasma Na was stable (137-144 mmol/l), K levels decreased from 8.5 to 5 mmol/l, urinary Na/K from 90 to 24, and TTKG increased from 0.3 to 1.8. Ca/Cr decreased from 3.5 to 1.5 mmol/mmol. The dosage of cation exchange resins was decreased, oral fluids were tolerated, and the patient's general condition improved. Hence: hydroclorothiazide can be useful in the treatment of severe hyperkalemia and hypercalciuria of pseudohypoaldosteronism type I.
authors.affiliation: Department of Pediatrics, Hospital St. Maria, University of Lisbon, Portugal.
authors.forename: R C
authors.initials: RC
authors.lastname: Stone
authors.forename: P
authors.initials: P
authors.lastname: Vale
authors.forename: F C
authors.initials: FC
authors.lastname: Rosa
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D002118:Calcium / Q000652:urine*; D004232:Diuretics / Q000627:therapeutic use; D005260:Female; D005665:Furosemide / Q000627:therapeutic use; D006801:Humans; D006852:Hydrochlorothiazide / Q000627:therapeutic use*; D006947:Hyperkalemia / Q000188:drug therapy* / Q000209:etiology; D007213:Indomethacin / Q000627:therapeutic use; D007231:Infant, Newborn; D011188:Potassium / Q000097:blood / Q000652:urine; D011546:Pseudohypoaldosteronism / Q000150:complications / Q000188:drug therapy* / Q000652:urine; D006178:Romani People; D012964:Sodium / Q000097:blood / Q000652:urine; D049993:Sodium Chloride Symporter Inhibitors / Q000627:therapeutic use*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D004232:Diuretics; D049993:Sodium Chloride Symporter Inhibitors; D006852:Hydrochlorothiazide; D005665:Furosemide; D012964:Sodium; D011188:Potassium; D002118:Calcium; D007213:Indomethacin