Gill J R, Bartter F C
Am J Med. 1978 Nov;65(5):766-72. doi: 10.1016/0002-9343(78)90794-5.
Maximal free-water clearance was measured in five patients with Bartter's syndrome and in five patients with the hypokalemic alkalosis of persistent psychogenic vomiting. Hypokalemic alkalosis, hyperreninemia, hyperaldosteronism and excessive renal production of prostaglandin E2 were present in the patients with both disorders. Maximal free water clearance was abnormally low, in association with a high clearance of chloride, in all the patients with Bartter's syndrome; it was normal in all the patients with psychogenic vomiting. In the patients with Bartter's syndrome, apparent distal delivery of proximal tubular fluid was inversely related to glomerular filtration rate and was excessive only in those patients with a low glomerular filtration rate. Patients with psychogenic vomiting showed mean distal fractional chloride reabsorption of 0.92 +/- 0.04 (standard error [SE]). In the patients with Bartter's syndrome, distal fractional reabsorption of chloride was 0.49 +/- 0.08 and was the same (0.46 +/- 0.06) during inhibition of prostaglandin synthesis with indomethacin therapy. Thus, a prostaglandin-independent defect in chloride reabsorption in the loop of Henle is the most proximal cause for the abnormalities in Bartter's syndrome thus far identified.
对5例巴特综合征患者和5例持续性精神性呕吐所致低钾性碱中毒患者进行了最大自由水清除率测定。两种疾病的患者均存在低钾性碱中毒、高肾素血症、醛固酮增多症以及肾脏前列腺素E2生成过多。所有巴特综合征患者的最大自由水清除率均异常降低，同时伴有氯清除率升高；而所有精神性呕吐患者的最大自由水清除率均正常。在巴特综合征患者中，近端肾小管液的表观远端输送与肾小球滤过率呈负相关，且仅在肾小球滤过率较低的患者中过高。精神性呕吐患者的远端氯分数重吸收平均值为0.92±0.04（标准误[SE]）。在巴特综合征患者中，氯的远端分数重吸收为0.49±0.08，在用吲哚美辛抑制前列腺素合成治疗期间，该值相同（0.46±0.06）。因此，迄今为止所确定的，亨氏袢中氯重吸收的前列腺素非依赖性缺陷是巴特综合征异常的最根本原因。
