Walser M, Hill S, Tomalis E A
Department of Pharmacology and Molecular Sciences, Johns Hopkins School of Medicine, Baltimore, MD 21205, USA.
Am J Kidney Dis. 1996 Sep;28(3):354-64. doi: 10.1016/s0272-6386(96)90492-8.
Optimal dietary protein intake for adults with the nephrotic syndrome has not been established; very low-protein diets are believed to be contraindicated. Sixteen patients with the nephrotic syndrome were nevertheless prescribed a very low protein diet (0.3 g/kg) supplemented by 10 to 20 g/d essential amino acids (or, in a few cases, ketoacids) for an average of 10 months (range, 1 to 36 months). In 11 patients with initial glomerular filtration rates (GFRs) < or = 30 mL/min/3 m2 of height (ht)2, significant but modest improvement was seen (on the average) in proteinuria, serum albumin, and serum cholesterol; all 11 eventually went on to dialysis. The other five patients, with initial GFRs of 32 to 69 ml/min/3 m2 of ht2, had either focal segmental glomerulosclerosis, diabetic nephropathy, or, in one patient, both. The nephrotic syndrome associated with these disorders rarely remits spontaneously. However, during the following 3 to 15 months mean proteinuria decreased from 9.3 to 1.9 g/d, mean serum albumin increased from 2.5 g/dL to 3.8 g/dL, and mean serum cholesterol decreased from 415 mg/dL to 255 mg/dL (all P < 0.001). The GFR either remained constant or increased. Four of these five patients have resumed normal or nearly normal diets and remain in remission or near-remission for 6 to 24 months. We conclude that severe protein restriction plus an essential amino acid supplement may induce prolonged remission in adults with the nephrotic syndrome provided that GFR is not severely reduced. The mechanism of this paradoxical response to protein restriction remains to be determined.
成人肾病综合征的最佳膳食蛋白质摄入量尚未确定;极低蛋白饮食被认为是禁忌的。然而,16例肾病综合征患者被规定采用极低蛋白饮食(0.3g/kg),并补充10至20g/d的必需氨基酸(少数情况下为酮酸),平均持续10个月(范围为1至36个月)。在11例初始肾小球滤过率(GFR)≤30mL/min/3m²身高(ht)²的患者中,蛋白尿、血清白蛋白和血清胆固醇(平均)有显著但适度的改善;所有11例最终都进行了透析。另外5例患者,初始GFR为32至69ml/min/3m²ht²,患有局灶节段性肾小球硬化症、糖尿病肾病,其中1例同时患有这两种疾病。与这些疾病相关的肾病综合征很少自发缓解。然而,在接下来的3至15个月中,平均蛋白尿从9.3g/d降至1.9g/d,平均血清白蛋白从2.5g/dL升至3.8g/dL,平均血清胆固醇从415mg/dL降至255mg/dL(所有P<0.001)。GFR要么保持不变,要么升高。这5例患者中有4例已恢复正常或接近正常饮食,并在缓解或接近缓解状态下维持6至24个月。我们得出结论,严重的蛋白质限制加必需氨基酸补充剂可能会使肾病综合征成人患者获得长期缓解,前提是GFR没有严重降低。这种对蛋白质限制的矛盾反应机制仍有待确定。