生活方式因素包括饮食和急性间歇性卟啉症的生化生物标志物：来自挪威北部病例对照研究的结果。

Lifestyle factors including diet and biochemical biomarkers in acute intermittent porphyria: Results from a case-control study in northern Norway.

机构信息

Department of Laboratory Medicine, Nordland Hospital Trust, Bodø, Norway; Institute of Clinical Medicine, UiT The Arctic University of Norway, Tromsø, Norway; Research Laboratory, Nordland Hospital Trust, Bodø, Norway.

Department of Laboratory Medicine, Nordland Hospital Trust, Bodø, Norway.

出版信息

Mol Genet Metab. 2019 Nov;128(3):254-270. doi: 10.1016/j.ymgme.2018.12.006. Epub 2018 Dec 10.

DOI:10.1016/j.ymgme.2018.12.006

PMID:30583995

Abstract

BACKGROUND

Lifestyle factors, including a low intake of carbohydrates, dieting, alcohol consumption, cigarette smoking and stress are some of the possible triggers of attacks in acute intermittent porphyria (AIP). The influence of lifestyle factors, including energy intake, diet and alcohol consumption on the biochemical disease activity in AIP and biochemical nutritional markers were examined.

METHODS

A case-control study with 50 AIP cases and 50 controls matched for age, sex and place of residence was performed. Dietary intake was registered using a food diary in 46 matched pairs. Symptoms, alcohol intake, stress and other triggering factors of the last AIP attack were recorded on questionnaires. Porphyrin precursors, liver and kidney function markers, vitamins, diabetogenic hormones and other nutritional biomarkers were analyzed by routine methods. The Wilcoxon matched-pairs signed rank test was used to compare the cases vs. controls. The Spearman's rank correlation coefficient was used on the cases.

RESULTS

Increasing total energy intake was negatively correlated with the biochemical disease activity. The intake of carbohydrates was lower than recommended, i.e., 40 and 39% of total energy intake in the AIP cases and controls, respectively. The plasma resistin level was significantly higher (p = .03) in the symptomatic than asymptomatic cases. Plasma insulin was lower in those with high porphobilinogen levels. The intake of sugar and candies were higher in the AIP cases with low U-delta aminolevulinic acid (ALA) levels (p = .04). Attacks were triggered by psychological stress (62%), physical strain (38%), food items (24%) and alcohol (32%) in the 34 symptomatic cases. Alcohol was used regularly by 88% of the cases (3.2 g ethanol/day) and 90% of the controls (6.3 g/day), but the intake was significantly lower in symptomatic than in asymptomatic cases (p = .045).

CONCLUSION

A high intake of energy, sugar and candies and a higher insulin level were associated with a lower biochemical disease activity. The resistin level was higher in the symptomatic than the asymptomatic cases. AIP patients drink alcohol regularly, but the intake was significantly lower in the symptomatic cases.

TRIAL REGISTRATION

ClinicalTrials.gov Identifier: NCT01617642.

摘要

背景

生活方式因素，包括碳水化合物摄入不足、节食、饮酒、吸烟和压力，是急性间歇性血卟啉症（AIP）发作的可能诱因之一。本研究旨在探讨生活方式因素（包括能量摄入、饮食和饮酒）对 AIP 患者生化疾病活动和生化营养标志物的影响。

方法

采用病例对照研究，纳入 50 例 AIP 患者和 50 例年龄、性别和居住地相匹配的对照。46 对匹配的病例和对照使用食物日记记录饮食摄入情况。通过问卷调查记录 AIP 发作的症状、饮酒量、压力和其他诱发因素。采用常规方法分析卟啉前体、肝肾功能标志物、维生素、致糖尿病激素和其他营养生物标志物。采用 Wilcoxon 配对符号秩检验比较病例与对照，Spearman 秩相关系数用于病例分析。

结果

总能量摄入增加与生化疾病活动呈负相关。碳水化合物摄入量低于推荐量，分别为 AIP 患者和对照的总能量摄入的 40%和 39%。与无症状病例相比，有症状病例的血浆抵抗素水平显著升高（p = .03）。高卟啉原水平患者的血浆胰岛素水平较低。AIP 患者中，低 U-δ-氨基乙酰丙酸（ALA）水平与糖和糖果摄入量较高有关（p = .04）。34 例有症状病例中，心理压力（62%）、体力消耗（38%）、食物（24%）和酒精（32%）是发作的诱因。88%的病例（3.2g 乙醇/天）和 90%的对照（6.3g/天）经常饮酒，但在有症状病例中，饮酒量明显低于无症状病例（p = .045）。