De Franceschi L, Cappellini M D, Graziadei G, Manzato F, Olivieri O, Corrocher R, Fiorelli G, Beuzard Y, Brugnara C
Department of Internal Medicine, University of Verona, Italy.
Haematologica. 1998 Feb;83(2):118-25.
Reduced serum or erythrocyte Mg have been reported in human beta thalassemia. These deficiencies may play a role in the cellular abnormalities characteristic of this disorder. We have therefore studied the effect of dietary Mg supplementation in patients with beta thalassemia intermedia in order to establish whether it improves the abnormalities of thalassemic erythrocytes.
Plasma and erythrocyte Mg were determined in 11 patients with b thalassemia intermedia, not requiring chronic transfusion therapy, and in 17 normal controls. Inclusion criteria included normal renal and liver function and performance status of 70% or greater. Seven patients were enrolled for the Mg supplementation study, after the appropriate informed consent was obtained. They were given a starting dose of 0.6 mEq/kg/day of magnesium pidolate, divided into two oral daily doses, for four weeks. In a 70-kg subject, a daily Mg dose of 42 mEq corresponds to 504 mg of Mg, with the daily Mg intake of normal subjects being 418 +/- 120 mg for males and 343 +/- 94 mg for females. After 28 days of treatment, five of the patients continued the protocol with a daily dosage increased to 1.2 mEq magnesium pidolate/kg/day, divided into two oral administrations, for an additional four weeks.
In patients with untransfused beta thalassemia intermedia we found reduced erythrocyte Mg (in mmol/kg Hb, 6.12 +/- 1.5, n = 11 vs. 8.69 +/- 0.89, n = 17, respectively, p < 0.0001) and normal serum Mg. In the seven patients given oral Mg supplements, at Mg dosages of 0.6 mEq/kg/day we observed significant increases in erythrocyte Mg, and significant improvement in some of the characteristic abnormalities of beta that erythrocytes (increased Na-K pump, KCl cotransport, cell dehydration, increased osmotic resistance). These changes were maintained in the 5 patients who were treated with 1.2 mEq of Mg/kg/day. Follow-up studies showed a return to baseline conditions. There were no signs of Mg toxicity, with the only side effect being diarrhea, which was generally mild, but led to discontinuation for one patient after the first four weeks.
These data indicate that dietary Mg supplementation improves some of the characteristic cellular function abnormalities of b thalassemia intermedia. The possible therapeutic value of this strategy should be further tested in these patients.
据报道，人类β地中海贫血患者血清或红细胞镁含量降低。这些缺乏可能在该疾病特征性的细胞异常中起作用。因此，我们研究了饮食中补充镁对中间型β地中海贫血患者的影响，以确定其是否能改善地中海贫血红细胞的异常。
测定了11例不需要长期输血治疗的中间型β地中海贫血患者及17名正常对照者的血浆和红细胞镁含量。纳入标准包括正常的肝肾功能以及70%或更高的体能状态。在获得适当的知情同意后，7例患者被纳入镁补充研究。他们开始服用匹多酸镁，剂量为0.6 mEq/kg/天，分两次口服，共四周。对于一名体重70kg的受试者，每日镁剂量42 mEq相当于504mg镁，正常受试者每日镁摄入量男性为418±120mg，女性为343±94mg。治疗28天后，5例患者继续该方案，每日剂量增加至1.2 mEq匹多酸镁/kg/天，分两次口服，再持续四周。
在未经输血的中间型β地中海贫血患者中，我们发现红细胞镁含量降低（分别为6.12±1.5 mmol/kg Hb，n = 11；与8.69±0.89 mmol/kg Hb，n = 17，p < 0.0001），而血清镁正常。在7例口服镁补充剂的患者中，当镁剂量为0.6 mEq/kg/天时，我们观察到红细胞镁显著增加，并且β地中海贫血红细胞的一些特征性异常有显著改善（钠钾泵活性增加、钾氯协同转运增加、细胞脱水、渗透压抵抗力增加）。这些变化在5例接受每日1.2 mEq镁/kg治疗的患者中得以维持。随访研究显示恢复到基线状态。没有镁中毒的迹象，唯一的副作用是腹泻，通常较轻，但有一名患者在最初四周后因腹泻停药。
这些数据表明，饮食中补充镁可改善中间型β地中海贫血的一些特征性细胞功能异常。该策略可能的治疗价值应在这些患者中进一步测试。
