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亨利氏袢处氯化物重吸收缺陷在巴特综合征的发病机制中起主要作用吗?

Does defective chloride reabsorption at the loop of Henle play a major role in the pathogenesis of Bartter's syndrome?

作者信息

Milani L, Pessina A C, Maccà F, Pauletto P, Gatta A

出版信息

Am J Nephrol. 1987;7(1):65-8. doi: 10.1159/000167433.

DOI:10.1159/000167433
PMID:3578377
Abstract

In a patient with typical features of Bartter's syndrome, intrarenal reabsorption of sodium and water at different sites of the nephron, during maximal water diuresis, was studied twice in the course of the disease. During the first study, chloride reabsorption at the loop of Henle and renal diluting ability were not impaired. Chloride reabsorption at the loop of Henle was 0.72 (normal values 0.66-0.90), minimal urine osmolality 71 mosm/kg (normal values less than 75 mosm/kg) and chloride fractional clearance 0.44 (normal values 0.3-6.8). An impaired chloride reabsorption at the loop of Henle was found 6 months later. Chloride reabsorption at the loop of Henle was 0.28, minimal urine osmolality 197 mosm/kg and chloride fractional clearance 8.23. These data suggest that the defect in chloride reabsorption at the loop of Henle is not a pathophysiological abnormality of the syndrome because it appeared at a later time than the other symptoms.

摘要

在一名患有巴特综合征典型特征的患者中,在疾病过程中对其进行了两次研究,以观察在最大水利尿期间肾单位不同部位对钠和水的肾内重吸收情况。在首次研究中,髓袢升支粗段对氯的重吸收及肾脏稀释能力未受损。髓袢升支粗段对氯的重吸收为0.72(正常值为0.66 - 0.90),最低尿渗透压为71 mosm/kg(正常值小于75 mosm/kg),氯分数清除率为0.44(正常值为0.3 - 6.8)。6个月后发现髓袢升支粗段对氯的重吸收受损。髓袢升支粗段对氯的重吸收为0.28,最低尿渗透压为197 mosm/kg,氯分数清除率为8.23。这些数据表明,髓袢升支粗段对氯重吸收的缺陷并非该综合征的病理生理异常,因为它比其他症状出现的时间晚。

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Does defective chloride reabsorption at the loop of Henle play a major role in the pathogenesis of Bartter's syndrome?亨利氏袢处氯化物重吸收缺陷在巴特综合征的发病机制中起主要作用吗?
Am J Nephrol. 1987;7(1):65-8. doi: 10.1159/000167433.
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Renal tubular reabsorption of chloride in Bartter's syndrome and other conditions with hypokalemia.巴特综合征及其他低钾血症情况下肾小管对氯的重吸收
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Evidence for a prostaglandin-independent defect in chloride reabsorption in the loop of Henle as a proximal cause of Bartter's syncrome.作为巴特综合征近端病因的亨利袢氯化物重吸收中前列腺素非依赖性缺陷的证据。
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