BACKGROUND & AIMS Sickle cell disease (SCD), a genetic disorder resulting from the presence of a mutated hemoglobin S (HbS), has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of SCD and health-related quality of life (HRQoL) in adult patients. METHODS In this longitudinal study, we recruited and prospectively followed 62 adults with SCD (aged ≥18 years) for a median of 93 months. At entry and follow-up, patients provided medical and dietary history, had a physical examination and anthropometric measurements, assessed protein-energy intake, measurement of micronutrient levels, estimation of SCD severity score, and determination of the HRQoL (SF-26v2). The study outcome was a composite of hospitalization due to SCD crises or death. RESULTS At baseline, 42 (67.74%) patients had macro and, or micro-undernutrition (Group A), and 20 (32.26%) were well nourished. (Group B). The BMI and most anthropometric measurements were significantly lower in SCD patients compared to control subjects. Seventy percent of SCD patients had vitamin D, vitamin B12, and zinc deficiencies. Thirty-six under-nourished patients (86%) had gastrointestinal disorders. During follow-up, 46 patients (74.19%) developed one or more vaso-occlusive pain crises or other SCD related complications that required hospitalization. Significant differences in most SF-36v2 domains existed between well-nourished and undernourished SCD patients. Protein-energy and micronutrient deficiencies were independent predictors of severe SCD and mortality. Correction of undernutrition and hydroxyurea therapy improved SCD severity scores and HRQoL. CONCLUSIONS Patients with sickle cell disease have various degrees of macro and micro deficiencies, which increase SCD severity and hospitalizations and reduce the health-related quality of life. Early diagnosis and prompt correction of macro and micronutrient deficiencies need to be incorporated in the standard of care of SCD patients to improve the disease outcomes.

中文翻译：

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营养对镰状细胞病成人疾病严重程度和健康相关生活质量的影响：一项前瞻性研究。

背景和目的 镰状细胞病 (SCD) 是一种由突变的血红蛋白 S (HbS) 引起的遗传疾病，在世界范围内分布，并在儿童和成人中引起显着的发病率和死亡率。很少有研究涉及成人 SCD 严重程度的决定因素；因此，我们调查了营养对成年患者 SCD 结局和健康相关生活质量 (HRQoL) 的影响。方法 在这项纵向研究中，我们招募并前瞻性地跟踪了 62 名 SCD 成人（年龄≥18 岁），中位时间为 93 个月。在入组和随访时，患者提供病史和饮食史，进行体格检查和人体测量，评估蛋白质能量摄入，测量微量营养素水平，估计 SCD 严重程度评分，并确定 HRQoL (SF-26v2)。研究结果是因 SCD 危机或死亡而住院的综合结果。结果 基线时，42 名（67.74%）患者存在宏观和/或微量营养不足（A 组），20 名（32.26%）患者营养良好。（B组）。与对照组相比，SCD 患者的 BMI 和大多数人体测量值显着降低。70% 的 SCD 患者存在维生素 D、维生素 B12 和锌缺乏症。36 名营养不良患者 (86%) 患有胃肠道疾病。在随访期间，46 名患者 (74.19%) 出现了一种或多种血管闭塞性疼痛危象或其他需要住院治疗的 SCD 相关并发症。大多数 SF-36v2 结构域在营养良好和营养不良的 SCD 患者之间存在显着差异。蛋白质能量和微量营养素缺乏是严重 SCD 和死亡率的独立预测因素。纠正营养不良和羟基脲治疗可改善 SCD 严重程度评分和 HRQoL。结论 镰状细胞病患者存在不同程度的宏观和微观缺陷，这增加了 SCD 的严重程度和住院率，降低了与健康相关的生活质量。需要将早期诊断和及时纠正大量和微量营养素缺乏症纳入 SCD 患者的护理标准，以改善疾病结果。这会增加 SCD 的严重程度和住院率，并降低与健康相关的生活质量。需要将早期诊断和及时纠正大量和微量营养素缺乏症纳入 SCD 患者的护理标准，以改善疾病结果。这会增加 SCD 的严重程度和住院率，并降低与健康相关的生活质量。需要将早期诊断和及时纠正大量和微量营养素缺乏症纳入 SCD 患者的护理标准，以改善疾病结果。