Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Nebraska Medical Center and Children's Hospital & Medical Center, Omaha, Nebraska, USA.
Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Pediatr Blood Cancer. 2023 Jul;70(7):e30397. doi: 10.1002/pbc.30397. Epub 2023 Apr 26.
Nutritional deficiencies are prevalent in sickle cell disease (SCD) and may be associated with worse pain outcomes. Gut dysbiosis has been reported in patients with SCD and may contribute to both nutritional deficiencies and pain.
We tested the association of nutrition, fat-soluble vitamin (FSV) deficiency, and gut microbiome composition on clinical outcomes in SCD. Second, we measured the association between diet and exocrine pancreatic function on FSV levels.
Using case control design, we enrolled children with SCD (n = 24) and matched healthy controls (HC; n = 17, age, sex, race/ethnicity). Descriptive statistics summarized demographic and clinical data. Wilcoxson-rank tests compared FSV levels between cohorts. Regression modeling tested the association between FSV levels and SCD status. Welch's t-test with Satterthwaite adjustment evaluated associations between microbiota profiles, SCD status, and pain outcomes.
Vitamin A and D levels were significantly decreased in participants with HbSS as compared to HC (vitamin A, p = < .0001, vitamin D, p = .014) independent of nutritional status. FSV correlated with dietary intake in SCD and HC cohorts. Gut microbial diversity was reduced in hemoglobin SS (HbSS) compared to hemoglobin SC (HbSC) and HC, p = .037 and .059, respectively. The phyla Erysipelotrichaceae and Betaproteobacteria were higher in SCD children reporting the highest quality-of-life (QoL) scores (p = .008 and .049, respectively), while Clostridia were higher in those with lower QoL scores (p = .03).
FSV deficiencies and gut dysbiosis are prevalent in children with SCA. Gut microbial composition is significantly different in children with SCD with low QoL scores.
营养缺乏在镰状细胞病（SCD）中很常见，可能与更严重的疼痛结果有关。据报道，SCD 患者存在肠道菌群失调，这可能导致营养缺乏和疼痛。
我们检测了营养、脂溶性维生素（FSV）缺乏和肠道微生物群落组成与 SCD 临床结局的相关性。其次，我们测量了饮食与外分泌胰腺功能对 FSV 水平的关系。
采用病例对照设计，我们纳入了 24 名 SCD 患儿（病例组）和 17 名匹配的健康对照者（对照组，年龄、性别、种族/民族匹配）。描述性统计分析总结了人口统计学和临床数据。Wilcoxson-rank 检验比较了两组 FSV 水平。回归模型检测了 FSV 水平与 SCD 状态的关系。Welch's t 检验和 Satterthwaite 调整用于评估微生物群谱、SCD 状态和疼痛结果之间的关系。
与对照组相比，HbSS 组的维生素 A 和 D 水平显著降低（维生素 A，p<0.0001，维生素 D，p=0.014），且独立于营养状况。FSV 与 SCD 和 HC 队列的饮食摄入相关。与 HbSC 和 HC 相比，血红蛋白 SS（HbSS）患者的肠道微生物多样性降低，分别为 p=0.037 和 0.059。在报告生活质量（QoL）评分最高的 SCD 患儿中，Erysipelotrichaceae 和 Betaproteobacteria 菌属较高（分别为 p=0.008 和 0.049），而 Clostridia 菌属在 QoL 评分较低的患者中较高（p=0.03）。
FSV 缺乏和肠道菌群失调在 SCA 儿童中很常见。生活质量较低的 SCD 儿童的肠道微生物组成存在显著差异。
