法属西印度群岛镰状细胞病队列和普通人群的血脂谱。
Lipid profiles in French West Indies sickle cell disease cohorts, and their general population.
机构信息
Université des Antilles, CHU de Pointe-à-Pitre, Guadeloupe, Université Sorbonne Paris Cité, Université Paris Diderot, Inserm, INTS, Unité Biologie Intégrée du Globule Rouge UMR_S1134, laboratoire d'Excellence GR-Ex, Paris, France.
Centre d'investigation Clinique Antilles Guyane, Inserm/DGOS CIC 14-24, enceinte de l'Institut Pasteur, Pointe-À-Pitre, Guadeloupe, France.
出版信息
Lipids Health Dis. 2018 Mar 5;17(1):38. doi: 10.1186/s12944-018-0689-5.
BACKGROUND
The pathophysiology of sickle cell disease (SCD) and the variability of its clinical expression remain not fully understood, whether within or between different SCD genotypes. Recent studies have reported associations between lipid levels and several SCD complications. If lipid levels have been previously described as low in sickle cell anemia (SCA), few data have been provided for sickle cell SC disease (SCC). We designed our epidemiological study to isolate lipid levels and profiles by genotype in Guadeloupian cohorts of SCA and SCC adult patients, at steady state. We compared SCD lipid levels with those of the Guadeloupian general population (GGP), and analyzed potential associations between lipid levels and SCD complications (vaso-occlusive crises, acute chest syndrome and osteonecrosis).
METHODS
Lipids, apolipoproteins, biological variables and anthropometric evaluation, were collected at steady state from medical files for 62 SCC and 97 SCA adult patients. Clinical SCD complications were collected from the clinical files. Analysis was conducted by genotype for all variables.
RESULTS
Different SCC and SCA lipid profiles, both distinct from their GGP's, were identified. Compared to SCC and GGP, higher triglyceride (TG) levels were observed in SCA patients, independent of hydroxyurea, hemolysis, gender, age, body mass index (BMI), abdominal obesity and clinical nutritional status. Our survey highlights also subsequent anthropometrical phenotypes, with an over-representation of abdominal obesity with normal BMI in SCA patients, and affecting almost exclusively females in both genotypes. Moreover, more frequent positive history of acute chest syndrome (ACS) was observed in SCA patients with TG level higher than 1.50 g/l, and of osteonecrosis in SCC patients having non high-density lipoprotein-cholesterol level (Non HDL-C) higher than 1.30 g/l.
CONCLUSIONS
This study reveals that SCA and SCC patients exhibit distinct lipid profiles and suggests that high TG and Non HDL-C levels are associated with past histories of ACS and osteonecrosis in SCA and SCC patients, respectively.
背景
镰状细胞病（SCD）的病理生理学及其临床表现的变异性在不同 SCD 基因型内或之间仍不完全清楚。最近的研究报告了血脂水平与几种 SCD 并发症之间的关联。如果以前已经描述过镰状细胞贫血（SCA）中的血脂水平低，那么对于镰状细胞 SC 病（SCC）则很少有数据提供。我们设计了这项流行病学研究，旨在分离稳态下瓜德罗普 SCA 和 SCC 成年患者的基因型的血脂水平和谱。我们将 SCD 的血脂水平与瓜德罗普普通人群（GGP）进行了比较，并分析了血脂水平与 SCD 并发症（血管阻塞危象、急性胸部综合征和骨坏死）之间的潜在关联。
方法
从稳态下的医疗档案中收集了 62 例 SCC 和 97 例 SCA 成年患者的血脂、载脂蛋白、生物学变量和人体测量评估。从临床档案中收集了临床 SCD 并发症。对所有变量按基因型进行分析。
结果
我们确定了不同的 SCC 和 SCA 血脂谱，它们都与 GGP 不同。与 SCC 和 GGP 相比，SCA 患者的甘油三酯（TG）水平更高，这与羟基脲、溶血、性别、年龄、体重指数（BMI）、腹部肥胖和临床营养状况无关。我们的调查还突出了随后的人体测量表型，SCA 患者中 BMI 正常的腹部肥胖发生率过高，而且这两种基因型中几乎仅发生在女性。此外，TG 水平高于 1.50 g/l 的 SCA 患者中更频繁出现急性胸部综合征（ACS）阳性病史，而 Non HDL-C 水平高于 1.30 g/l 的 SCC 患者中更频繁出现骨坏死。
结论
这项研究表明 SCA 和 SCC 患者表现出不同的血脂谱，并表明高 TG 和 Non HDL-C 水平与 SCA 和 SCC 患者过去的 ACS 和骨坏死病史相关。
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