McKenzie I M, Heiman D, Winter J S, McKenzie J K
Department of Internal Medicine, University of Manitoba, Winnipeg, Canada.
Clin Invest Med. 1987 Jul;10(4):303-8.
Studies of plasma samples of 3 subjects with Bartter's syndrome were compared to 8 subjects with other conditions. Despite high levels of active renin initially, with low levels of inactive renin, addition of either human nephrectomized plasma or sheep substrate not only increased active renin (by at least 3-fold) but also led to the appearance of large quantities of inactive renin (10-20 times the concentration originally present, much greater than the small increase seen with other plasmas). The activated inactive renin after substrate addition possibly had a larger and more variable molecular size (42,000-48,000) than normal inactive renin (42,500-44,500). Renin substrate in Bartter's plasma was present in similar amounts and had a normal or supranormal angiotensin generation rate with exogenous human renin. Bartter's substrate had a similar molecular weight (55,000) to that found in normal human plasma. The agent in the exogenous substrate preparations causing the increase in apparent active and inactive renin was not ultrafiltrable. However, an acidification procedure that destroyed exogenous substrate also removed the renin-increasing effect. Captopril increased renin but not aldosterone, while amiloride increased aldosterone but not renin. Neither agent improved serum potassium significantly in these patients on indomethacin.
对3名患有巴特综合征的受试者的血浆样本与8名患有其他病症的受试者的血浆样本进行了研究。尽管最初活性肾素水平较高，非活性肾素水平较低，但添加人肾切除血浆或绵羊底物不仅会使活性肾素增加（至少增加3倍），还会导致大量非活性肾素出现（浓度为最初的10 - 20倍，远高于其他血浆中出现的少量增加）。添加底物后激活的非活性肾素的分子大小（42,000 - 48,000）可能比正常非活性肾素（42,500 - 44,500）更大且更具变异性。巴特综合征患者血浆中的肾素底物含量相似，在外源性人肾素作用下，其血管紧张素生成率正常或超常。巴特综合征患者的底物分子量（55,000）与正常人血浆中的相似。外源性底物制剂中导致表观活性和非活性肾素增加的物质不能通过超滤去除。然而，一种破坏外源性底物的酸化程序也消除了肾素增加的效应。卡托普利增加肾素但不增加醛固酮，而氨氯吡咪增加醛固酮但不增加肾素。在服用吲哚美辛的这些患者中，这两种药物均未显著改善血清钾水平。
