Rc. Stone et al., EFFECT OF HYDROCHLOROTHIAZIDE IN PSEUDOHYPOALDOSTERONISM WITH HYPERCALCIURIA AND SEVERE HYPERKALEMIA, Pediatric nephrology, 10(4), 1996, pp. 501-503
Severe hyperkalemia resistant to treatment with sodium chloride (NaCl)
supplements plus cation exchange resins can be found in pseudohypoald
osteronism type I. In a patient with the multiple target organ variant
of this condition, hyperkalemia persisted at dangerous levels (8.5 mm
ol/l) despite large doses of NaCl (50 mmol/kg per day) and cation exch
ange resins (6 g/kg per day). Hypercalciuria was also present. The tot
al 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 gradie
nt (TTKG), and urinary calcium/creatinine (Ca/Cr) ratio were used to e
valuate 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 res
ins was decreased, oral fluids were tolerated, and the patient's gener
al condition improved. Hence: hydroclorothiazide can be useful in the
treatment of severe hyperkalemia and hypercalciuria of pseudohypoaldos
teronism type I.