BACKGROUND Hypocholesterolemia is the most frequently encountered lipid abnormality in sickle cell disease (SCD). We enrolled pediatric patients to determine the relationships between lipid profile and parameters of hemolysis, oxidative stress and chronic inflammation in SCD. METHODS The study involved 35 pediatric SCD patients and 19 healthy controls. Patients were crisis-free and had not received transfusions for the last 3 months. Total cholesterol, triglyceride, HDL-C, LDL-C, VLDL-C, apolipoprotein A1, apolipoprotein B, LCAT, LDH, bilirubin, haptoglobin, iron, ferritin, hemin, serum amyloid A (SAA), myeloperoxidase (MPO), uric acid, ALT and GGT levels were evaluated in patients' blood. RESULTS Patients had hypocholesterolemia depicted by lower levels of total cholesterol, HDL-C, LDL-C, as well as Apolipoprotein A1 and Apolipoprotein B compared to controls. The chronic hemolysis of SCD was evident in patients by higher LDH and bilirubin and almost undetectable haptoglobin levels. Hemin levels (as a measure of oxidized heme) were significantly increased in patients with SCD. Inflammation markers, SAA and MPO, were significantly increased in the patients as well. There were negative correlations between HDL-C and LDH, and Apo A1 and SAA. Hemin was positively correlated to MPO. CONCLUSION Hemolysis was associated with decreased HDL -C, and Inflammation was linked to decreased apolipoprotein A1 levels in our SCD patients. Therefore, we suggest that the HDL particle is altered during the course of the disease. The altered HDL in SCD may become dysfunctional and result with a slowing down of the reverse cholesterol transport.

中文翻译：

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镰状细胞病中HDL颗粒改变：胆固醇含量降低与溶血有关，而载脂蛋白A1降低则与炎症有关。

背景低胆固醇血症是镰状细胞病（SCD）中最常见的脂质异常。我们招募了儿科患者，以确定脂质谱与溶血参数，SCD中的氧化应激和慢性炎症参数之间的关系。方法该研究涉及35名儿科SCD患者和19名健康对照。患者没有危机，并且在过去3个月内未接受输血。总胆固醇，甘油三酸酯，HDL-C，LDL-C，VLDL-C，载脂蛋白A1，载脂蛋白B，LCAT，LDH，胆红素，触珠蛋白，铁，铁蛋白，血红素，血清淀粉样蛋白A（SAA），髓过氧化物酶（MPO），尿酸评估患者血液中的酸，ALT和GGT水平。结果患者的低胆固醇血症表现为总胆固醇，HDL-C，LDL-C，以及载脂蛋白A1和载脂蛋白B与对照组相比。LDH和胆红素升高以及触珠蛋白水平几乎无法检测到，SCD的慢性溶血在患者中很明显。SCD患者的血红素水平（作为氧化血红素的量度）显着增加。患者体内的炎症标志物SAA和MPO也显着增加。HDL-C和LDH与Apo A1和SAA之间呈负相关。血红素与MPO呈正相关。结论溶血与HDL -C降低有关，炎症与SCD患者载脂蛋白A1水平降低有关。因此，我们建议在疾病过程中改变HDL颗粒。SCD中HDL的改变可能会导致功能障碍，并导致胆固醇逆向转运减慢。