Solomon L R, Bobinski H, Astley P, Goldby F S, Mallick N P
Q J Med. 1982 Summer;51(203):251-70.
The pathophysiology of Bartter's syndrome affecting seven adults has been investigated. (1) Saralasin infusion caused a fall in blood pressure in all patients, suggesting that angiotensin was contributing to the maintenance of blood pressure. (2) Following a water load, urinary chloride concentrations and osmolality were both low. No positive evidence for a defect in chloride reabsorption in the ascending limb of the loop of Henle was obtained. (3) The effect of high and low dietary sodium on plasma sodium, potassium, chloride, magnesium, renin activity, aldosterone, 6-keto-PGF1a, thromboxane B2, urinary kallikrein, platelet function and erythrocyte membrane cation transport were studied. A variety of responses was observed. Sodium restriction increased (or sodium loading decreased), plasma renin activity, aldosterone, 6-keto-PGF1a, urinary kallikrein and the platelet aggregation abnormality in some, but not all, individuals. (4) Treatment with indomethacin was undertaken in all patients and studied in detail in one patient. There was weight gain, increase in plasma sodium and potassium, decrease in capillary pH, positive sodium and potassium balance, and decrease in plasma renin activity, 6-keto-PGF1a, thromboxane B2 and urinary kallikrein. Hypomagnesaemia and excessive urinary magnesium loss persisted unchanged. (5) A variety of abnormalities of erythrocyte membrane cation transport was found and these persisted during high- and low-sodium, and high-potassium intakes; and during treatment with indomethacin, despite correction of intracellular sodium and potassium concentrations. Bartter's syndrome is associated with an abnormality of erythrocyte membrane sodium and potassium transport. Many of the other metabolic abnormalities may be the consequence of potassium and sodium depletion.
对7名成年巴特综合征患者的病理生理学进行了研究。(1) 输注沙拉新使所有患者血压下降，提示血管紧张素有助于维持血压。(2) 水负荷后，尿氯浓度和渗透压均降低。未获得髓袢升支氯化物重吸收缺陷的确切证据。(3) 研究了高钠和低钠饮食对血浆钠、钾、氯、镁、肾素活性、醛固酮、6-酮-前列环素F1a、血栓素B2、尿激肽释放酶、血小板功能和红细胞膜阳离子转运的影响。观察到多种反应。钠限制（或钠负荷增加）使部分但并非所有个体的血浆肾素活性、醛固酮、6-酮-前列环素F1a、尿激肽释放酶和血小板聚集异常增加。(4) 所有患者均接受吲哚美辛治疗，并对1例患者进行了详细研究。结果显示体重增加、血浆钠和钾升高、毛细血管pH降低、钠和钾呈正平衡，以及血浆肾素活性、6-酮-前列环素F1a、血栓素B2和尿激肽释放酶降低。低镁血症和尿镁过度丢失持续不变。(5) 发现了多种红细胞膜阳离子转运异常，这些异常在高钠和低钠饮食、高钾摄入期间持续存在；在吲哚美辛治疗期间，尽管细胞内钠和钾浓度得到纠正，异常仍持续存在。巴特综合征与红细胞膜钠和钾转运异常有关。许多其他代谢异常可能是钾和钠耗竭的结果。
