Sano M, Shichiri M, Ida T, Sasaki S, Tsukagoshi H
Department of Neurology, School of Medicine, Tokyo Medical University.
Intern Med. 1994 Jan;33(1):1-5. doi: 10.2169/internalmedicine.33.1.
We studied three siblings with Bartter's syndrome associated with hypomagnesemia; two of them showing marked hypomagnesemia and the other mild hypomagnesemia. Urinary potassium, sodium and chloride excretions were determined and distal fractional chloride reabsorption and free water clearance on water loading test were compared before and after magnesium supplementation. Baseline urinary potassium and magnesium excretions were elevated in spite of the decreased plasma levels, whereas distal fractional chloride reabsorption and free water clearance were depressed in all patients. Magnesium repletion resulted in significant decrease in urinary potassium, sodium and chloride and subsequent increase in plasma potassium in all patients. However, neither distal fractional chloride reabsorption nor free water clearance was affected. Hypomagnesemia may contribute to urinary potassium wasting and aggravate urinary sodium and chloride wasting in familial Bartter's syndrome by a mechanism independent of the defect in free-water formation by the active reabsorption of chloride in Henle's loop.
我们研究了三名患有巴特综合征并伴有低镁血症的兄弟姐妹;其中两人表现出明显的低镁血症,另一人则为轻度低镁血症。测定了尿钾、钠和氯的排泄量,并比较了镁补充前后水负荷试验中远端氯分数重吸收和自由水清除率。尽管血浆水平降低,但基线尿钾和镁排泄量仍升高,而所有患者的远端氯分数重吸收和自由水清除率均降低。补充镁后,所有患者的尿钾、钠和氯均显著减少,随后血浆钾升高。然而,远端氯分数重吸收和自由水清除率均未受影响。低镁血症可能通过一种独立于亨利袢中氯主动重吸收形成自由水缺陷的机制,导致家族性巴特综合征患者尿钾浪费,并加重尿钠和氯浪费。