Baehler R W, Work J, Kotchen T A, McMorrow G, Guthrie G
Am J Med. 1980 Dec;69(6):933-8. doi: 10.1016/s0002-9343(80)80022-2.
There is no agreement concerning the primary pathogenetic event leading to Bartter's syndrome. Free water clearance and distal fractional chloride reabsorption were abnormally low in our patient with Bartter's syndrome. This series of investigations in this patient with Bartter's syndrome and hypomagnesemia was undertaken to determine if the defect in chloride transport in the ascending limb and the associated renal potassium wasting was specifically related to potassium depletion, increased prostaglandin production or magnesium depletion. Neither potassium repletion, indomethacin administration nor magnesium repletion had an effect on the defect in free water clearance or in distal fractional chloride reabsorption. However, magnesium infusion eliminated renal potassium wasting. These observations suggest that the proximate cause of Bartter's syndrome in this patient is a primary defect in the reabsorption of sodium chloride in the ascending limb and not renal potassium wasting. however, hypomagnesemia may contribute to the renal potassium wasting seen in this syndrome.
关于导致巴特综合征的原发性致病事件尚无定论。我们这位患有巴特综合征的患者的自由水清除率和远端氯化物重吸收分数异常低。对这位患有巴特综合征和低镁血症的患者进行了这一系列研究,以确定升支中氯化物转运缺陷及相关的肾钾流失是否与钾缺乏、前列腺素生成增加或镁缺乏有特定关联。补钾、给予吲哚美辛或补镁均未对自由水清除率缺陷或远端氯化物重吸收分数产生影响。然而,输注镁消除了肾钾流失。这些观察结果表明,该患者巴特综合征的直接原因是升支中氯化钠重吸收的原发性缺陷,而非肾钾流失。不过,低镁血症可能导致了该综合征中所见的肾钾流失。