Stone R C, Vale P, Rosa F C
Department of Pediatrics, Hospital St. Maria, University of Lisbon, Portugal.
Pediatr Nephrol. 1996 Aug;10(4):501-3. doi: 10.1007/s004670050149.
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.
I型假性醛固酮减少症患者可能会出现对补充氯化钠（NaCl）加阳离子交换树脂治疗无效的严重高钾血症。在一名患有该疾病多靶器官变异型的患者中，尽管给予大剂量的NaCl（每天50 mmol/kg）和阳离子交换树脂（每天6 g/kg），高钾血症仍持续处于危险水平（8.5 mmol/L）。同时还存在高钙尿症。口服所需的液体和补充剂总量无法耐受。尝试使用吲哚美辛（每天2 mg/kg），随后使用氢氯噻嗪（每天2 mg/kg）来进一步纠正失衡。采用血浆钾（K）和钠水平、尿钠/钾比值、肾小管钾梯度（TTKG）以及尿钙/肌酐（Ca/Cr）比值来评估氢氯噻嗪的疗效。治疗期间，血浆钠稳定（137 - 144 mmol/L），钾水平从8.5 mmol/L降至5 mmol/L，尿钠/钾从90降至24，TTKG从0.3升至1.8。Ca/Cr从3.5 mmol/mmol降至1.5 mmol/mmol。阳离子交换树脂的剂量减少，口服液体能够耐受，患者的总体状况有所改善。因此：氢氯噻嗪可用于治疗I型假性醛固酮减少症的严重高钾血症和高钙尿症。
