Defects in Postabsorptive Plasma Homeostasis of Fatty Acids in Sickle Cell Disease

Buchowski, Maciej S.; Swift, Larry L.; Akohoue, Sylvie A.; Shankar, Sadhna; Flakoll, Paul J.; Abumrad, Naji N.

doi:10.1177/0148607107031004263

Cited by 18 publications

(28 citation statements)

References 22 publications

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“…We observed no significant difference in non-HDL-C levels among our participants. This to our knowledge is the first report on non-HDL-C levels in SCD and thus gives credence to the established evidence that cardiovascular disease in sickle cell disease is mostly due to pulmonary hypertension and not atherosclerosis associated with elevated levels of TC, HDL, and LDL [9, 18, 19]. …”

Section: Discussionsupporting

confidence: 69%

“…These two morbid processes disturb lipid homeostasis which in turn may lead to atherosclerosis in these patients [7, 8]. Abnormal lipid homeostasis, as well as other haematological disorders, has been reported in SCA and this has been suggested to have the potential to alter membrane fluidity and functions of red blood cells (RBC) in individuals with SCD [9–11]. …”

Section: Introductionmentioning

confidence: 99%

“…Earlier studies in patients with SCD recorded a significant increase in triglyceride (TG) levels and decreased levels of total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), and low-density lipoprotein cholesterol (LDC-C) [9]. This remains a cause for concern as it is associated with increased mortality [10].…”

Section: Introductionmentioning

confidence: 99%

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Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

et al. 2016

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Background. Abnormal lipid homeostasis in sickle cell disease (SCD) is characterized by defects in plasma and erythrocyte lipids and may increase the risk of cardiovascular disease. This study assessed the lipid profile and non-HDL cholesterol level of SCD patients. Methods. A hospital-based cross-sectional study was conducted in 50 SCD patients, in the steady state, aged 8–28 years, attending the SCD clinic, and 50 healthy volunteers between the ages of 8–38 years. Serum lipids were determined by enzymatic methods and non-HDL cholesterol calculated by this formula: non-HDL-C = TC-HDL-C. Results. Total cholesterol (TC) (p = 0.001) and high-density lipoprotein cholesterol (HDL-C) (p < 0.0001) were significantly decreased in cases compared to controls. The levels of non-HDL-C, low-density lipoprotein cholesterol (LDL-C), and triglyceride (TG) were similar among the participants. The levels of decrease in TC and HDL were associated with whether a patient was SCD-SS or SCD-SC. Systolic blood pressure and diastolic blood pressure were each significantly associated with increased VLDL [SBP, p = 0.01, OR: 0.74 (CI: 0.6–0.93); DBP, p = 0.023, OR: 1.45 (CI: 1.05–2.0)]. Conclusion. Dyslipidemia is common among participants in this study. It was more pronounced in the SCD-SS than in SCD-SC. This dyslipidemia was associated with high VLDL as well as increased SBP and DBP.

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Section: Discussionsupporting

confidence: 69%

Section: Introductionmentioning

confidence: 99%

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Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

et al. 2016

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“…There is a general alteration in the lipid balance of SCA patients but the underlying mechanisms have been poorly understood [8]. Lower plasma cholesterol levels in SCA patients compared to healthy controls were reported by many studies [9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Hypocholesterolemia is associated negatively with hemolysate lipid peroxidation in sickle cell anemia patients

et al. 2010

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The oxidative stress levels in plasma and hemolysate and cholesterol levels in plasma of sickle cell anemia patients, carriers and controls were evaluated. A total of 40 cases-17 patients, 13 carriers and 10 controls-were involved in the study. Plasma and hemolysate malondialdehyde (MDA) were detected via thiobarbituric acid reaction with a fluorimetric detector by high-performance liquid chromatography system. Plasma cholesterol was determined by enzymatic colorimetric method. Mean MDA levels of SCA patients were higher than those of the carriers' and healthy children's both in plasma and in hemolysate (P < 0.005). The mean plasma and hemolysate MDA levels were 25.3 ± 1.6 nmol/l and 86.7 ± 19.3 nmol/l in patients, 19.1 ± 0.8 nmol/l and 54.1 ± 10.8 nmol/l in carriers and 19.6 ± 0.8 nmol/l and 56.8 ± 9.3 nmol/l in healthy children. Mean plasma total cholesterol levels were 92.1 ± 19.1 mg/dl in patients, 116.2 ± 23.3 mg/dl in carriers and 126.6 ± 16.4 mg/dl in controls (P < 0.005). There was a significant negative correlation of -0.520 between hemolysate MDA and plasma cholesterol levels in patients (P < 0.05). The degree of correlation increased up to -0.782 (P = 0.008) in the patients with HbSS phenotype. This negative correlation between MDA and cholesterol may imply a potential association between oxidative stress and hypocholesterolemia in sickle cell anemia.

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“…Our observation of significantly elevated fasting serum TAG concentration is in accordance with a study that reported 22% higher plasma TAG in Nigerian children with homozygous SCD compared with controls (Erasmus, Olukoga, & Ojuawo, ). Studies have also shown that patients with SCD, without documented vaso‐occlusive crises for at least 3 months, had elevated fasting plasma concentrations of TAG when compared with control participants matched for age, weight, and body fat (Buchowski et al, ; Zorca et al, ). The documented increase in fasting plasma TAG concentrations in SCD patients needs further investigation.…”

Section: Discussionmentioning

confidence: 99%

Decreased Serum Levels of Sphingomyelins and Ceramides in Sickle Cell Disease Patients

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et al. 2018

Lipids

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Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle-cell disease (SCD). This study was aimed at identifying the levels of C16-C24 CerPCho and C16-C24 CER in serum obtained from SCD patients and controls. Circulating levels of neutral sphingomyelinase (N-SMase) activity, ceramide-1-phosphate (C1P), and sphingosine-1-phosphate (S1P) were also determined. Blood was collected from 35 hemoglobin (Hb)A volunteers and 45 homozygous HbSS patients. Serum levels of C16-C24 CerPCho and C16-C24 CER were determined by an optimized multiple reaction monitoring (MRM) method using ultrafast liquid chromatography (UFLC) coupled with tandem mass spectrometry (MS/MS). Serum activity of N-SMase was assayed by standard kit methods, and C1P and S1P levels were determined by enzyme-linked immunosorbent assay. A significant decrease was observed in the serum levels of C18-C24 CerPCho in patients with SCD compared to controls. No significant difference was found in C16 CerPCho levels between the two groups. Very-long-chain C22-C24 CER were significantly decreased in SCD, while long-chain C16-C20 CER levels showed no significant difference between SCD patients and controls. Significant positive correlation was found between the serum total cholesterol levels and C18-C24 CerPCho and C22-C24 CER in SCD patients. Patients with SCD had significantly elevated serum activity of N-SMase as well as increased circulating levels of C1P and S1P compared to controls. The decrease in serum levels of C18-C24 CerPCho in patients with SCD was accompanied by decreased levels of C22-C24 CER. Future studies are needed to understand the role of decreased CerPCho and CER in the pathophysiology of SCD.

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Defects in Postabsorptive Plasma Homeostasis of Fatty Acids in Sickle Cell Disease

Cited by 18 publications

References 22 publications

Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

Hypocholesterolemia is associated negatively with hemolysate lipid peroxidation in sickle cell anemia patients

Decreased Serum Levels of Sphingomyelins and Ceramides in Sickle Cell Disease Patients

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