营养失调与治疗杂志

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营养与健康 2017：患有先天性单功能肾的儿童的饮食疗法 - Zeev Katzir - 特拉维夫大学

泽夫·卡齐尔

背景：肾损伤、蛋白尿和高血压是遗传性肾病 (CSFK) 的显著特征。肾损伤在 10 岁左右出现，到 30 岁时需要透析。少量蛋白质摄入可减轻动物未经试验模型、单肾切除患者以及肾移植捐赠者和接受者的肾损伤。低盐摄入有助于控制血压。 目的：迄今为止，尚未描述有关 CSFK 患者的相关数据。我们决定研究蛋白质限制和低钠饮食对这一特定人群的蛋白尿、肾功能和高血压的长期预防作用。方法：在前瞻性观察暴露系列中纳入了 28 名 CSFK 儿童。在营养师监督下，按照父母的安排，限制蛋白质和盐：0.85 倍推荐每日摄入量 (RDA)。通过以下方式确认：监测尿素氮和体重；测量 24 小时尿钠。补体（8-22 岁和卑微的 15.8 岁）包括临床、生长发育评估、肾功能和尿蛋白排泄。 患者和方法：

本研究共纳入 28 名儿童，均因超声检查发现 CSFK 而送至我们儿科肾病门诊观察 14 年，疑似诊断均通过肾脏动态扫描确诊。

 

蛋白质和盐限制：自断奶（母乳喂养或工业配方奶粉）以来，在营养师的持续监督下，对每位患者开始限制 0.85 × 推荐每日摄入量 (RDA)。通过 (a) 监测尿素氮 (g/24 小时) 和体重来验证 (a) 蛋白质和 (b) 盐限制的合规性，与以下公式一致：

每日蛋白质摄入量（g ）=[（尿尿素氮（g / 24h ）+ 0.031 ×体重（kg ）]× 6.25每日蛋白质摄入量（g）=[（尿尿素氮（g/24h）+ 0.031 ×体重（kg） ] ×6.25

 

The follow-up included twice a year visits within the first 3 years then once-a-year visit. Twenty-four-hour urine collections were done from the age of three years. Just in case of doubtful accuracy of the gathering, another one was performed, after further explanation. Patients and fogeys were evaluated and instructed on each visit by paediatric nephrologists and dieticians. The entire period of follow-up was 22 years. Twenty-three completed the study, as young adults.

Statistical Analysis:

Analysis of knowledge was administered using SPSS 11.0 statistical analysis software (SPSS Inc, Chicago, IL). Outcome variables (serum creatinine, rates of RDA for protein intake and for salt intake) as descriptive statistics were calculated and reported as mean ± variance.

 

Results:

Baseline data showed normal physical assessment, blood pressure kidney functions and urinalysis. There was one patient with ipsilateral kidney malformation and two with systemic pathologies. Adherence to dietary restrictions: 89 ±8% for protein and 93±5% salt. At the top of follow up: two patients had chronic renal disorder (CKD) grade I. Not one person had hypertension, proteinuria or growing and enlargement worsening.

 

One child was uninephrectomized (6 months old), due to UPJ stenosis, which caused severe hydronephrosis and hydroureter (right kidney). This kidney, actually, wasn't working at birth.

 

Adherence to the diet regime is expressed because the rate of protein and salt restriction targets, consistent with urine measurements described within the Methods section. Visit numbers represent 4- to 5-year intervals between each, when 1 is that the first presentation with the diagnosis of CSFK. Adherence to dietary restrictions was 89 ± 8% for protein and 93 ± 5% for salt.

 

The number of CSFK patients in our one-center observation study was larger than expected. as long as the annual birth rate in our municipal area was 20/1000 at the start of the study (30% to 80% quite within the Western world and Asia) leads us to suppose that this condition is more frequent than had been considered thus far (around 1/180 in our regional population vs 1/2031). it's according to our assumption that “real” incidence within the past was also much greater (see the Introduction section).

 

Arguably, lack of an impact group may be a certain limitation of our study. However, studies summarizing unfavorable outcome of youngsters with CSFK do exist, during which none deals with preventive or preservative therapeutic strategy.They can be considered historical controls for our work, considering identical population characteristics and nutritional habits, supported Israeli data of daily consumption of protein and salt among children. Furthermore, identified as exposure series observation (exposure = CSFK), its favorable final outcome is valid: only 8.6% with mild renal injury, none had proteinuria, hypertension, and growth-retardation. One cannot ignore the effect of the intervention.

 

2 名患有 I 级慢性肾病 (CKD) 的例外患者代表了已知的肾损伤风险因素 CAKUT（患者 19），以及由于自 17 岁（患者 7）开始的 8 年随访时间相对较短而缺乏足够的观察和营养指导。他们的尿蛋白排泄量（<400 mg/24 h）不受运动的影响（15 mg/dL，无论是在静息状态下还是在运动后），与上述结果同时单独评估。

 

我们没有发现本例患者的影像学结果（包括 CRG 或正常大小的肾脏）与随访结束时的肾脏损伤之间存在相关性。所有患者均显示正常的发育、体重和身高增长。

 

综上所述，我们得出结论，严格随访并控制饮食监督，轻度限制蛋白质和盐分，可预防 CSFK 患者出现肾损伤、蛋白尿和高血压。这种疗法对生长发育没有不利影响。

 

结论：严格的随访和有节制的饮食管理，轻度限制蛋白质和盐摄入，可以避免 CSFK 出现肾损伤、蛋白尿和高血压，且不会对生长发育造成损害。

该研究部分在2017 年 2 月 20 日至 21 日于德国柏林举行的第九届^国际 营养与健康大会上发表。