Defective lipid metabolism in sickle cell anaemia subjects in vaso-occlusive crisis

Akinlade, Kehinde Sola; Adewale, Christiana Odunayo; Rahamon, Sheu Kadiri; Fasola, F A; Olaniyi, John Ayodele; Atere, Adedeji David

doi:10.4103/0300-1652.140388

Cited by 15 publications

(16 citation statements)

References 22 publications

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“… 23 Also, it was reported recently that an elevated level of triglycerides, which is already known to be linked with endothelial dysfunction, is an independent predictor of pulmonary hypertesion in patients with SCA. 29 Raised triglyceride levels is an important cardiovascular risk factor for atherogenesis, as other abnormalities of the lipid profile are much more likely and are a readily available complement to atheromatous plaque formation and progression.…”

Section: Discussionmentioning

confidence: 99%

Electrocardiographic abnormalities and dyslipidaemic syndrome in children with sickle cell anaemia

Adegoke

Okeniyi

Akintunde

2016

CVJA

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SummaryBackgroundLipid and electrocardiographic (ECG) abnormalities have been reported in adults with sickle cell anaemia (SCA) and may reflect underlying structural and/ or functional damage. However, the relationship between ECG and lipid abnormalities among children with sickle cell disease is not fully understood.ObjectivesTo compare the steady-state lipid and ECG abnormalities in children with SCA to the controls and examine the hypothesis that lipid abnormalities are closely related to electrocardiographic abnormalities, and therefore are a reflection of cardiac damage among these children.Methods:Clinical, laboratory and ECG profiles of 62 children with SCA and 40 age- and gender-matched haemoglobin AA controls were compared. The influence of clinical characteristics, lipids profiles, markers of haemolysis, and renal and hepatic dysfunction on ECG pattern in children with SCA was then determined.ResultsThe patients had lower average diastolic and mean arterial blood pressure, total cholesterol and low-density lipoprotein cholesterol (LDL-C) levels than the controls, (p = 0.001, 0.002, 0.000 and 0.000, respectively). The mean triglyceride level was significantly higher (p < 0.001), while high-density lipoprotein cholesterol (HDL-C) levels were comparable (p = 0.858). The cases were about six times more likely to have left ventricular hypertrophy than the controls (OR = 6.4, 95% CI = 2.7–15.6, p = 0.000). Haematocrit level had a negative correlation with QTC (r = –0.3, p = 0.016) and QT intervals (r = – 0.3, p = 0.044). Triglyceride levels had a positive correlation with the PR interval (r = 0.3, p = 0.012), while serum alanine transferase (ALT) concentrations had an inverse correlation with PR interval (r = –0.3, p = 0.015). There was no statistical difference in the sociodemographic and clinical characteristics of the SCA children with or without ECG abnormalities. However, the mean triglyceride and serum ALT levels in those with ECG abnormalities were significantly higher than those without (p = 0.007 and 0.045, respectively).ConclusionLipid and ECG abnormalities are common in children with SCA. Elevated triglyceride and serum ALT levels are possible biochemical markers of ECG abnormalities in these patients.

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Section: Discussionmentioning

confidence: 99%

Electrocardiographic abnormalities and dyslipidaemic syndrome in children with sickle cell anaemia

Adegoke

Okeniyi

Akintunde

2016

CVJA

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“…In the general population, low HDL is a recognized risk factor of cardiovascular disease but low HDL also remains a common feature of pulmonary hypertension which is the main cardiovascular disorder associated with SCD [27]. SCD patients with low HDL are more likely to have received more blood transfusions, an indication of the severity of the patient's condition [28]. …”

Section: Discussionmentioning

confidence: 99%

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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“…However, there is evidence that pulmonary hypertension, for instance, seems to be associated to hemolysis, VO, and elevated triglyceride levels in a large cohort of individuals with sickle cell disease (SCD) with increased tricuspid regurgitant velocity [ 20 ]. In addition, during vasoocclusive crises, patients with SCA presented total cholesterol, triglyceride, and LDL-C levels significantly decreased whereas HDL-C levels were increased when compared to steady state [ 21 ]. Also, during steady state, HDL-C levels were found to be associated to nitric oxide metabolites (NOm) and fetal hemoglobin (HbF) levels [ 18 ], two of the most important prognostic biomarkers in SCA.…”

Section: Introductionmentioning

confidence: 99%

Investigation of Lipid Profile and Clinical Manifestations in SCA Children

et al. 2020

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Introduction. Clinical complications in sickle cell anemia (SCA) are heterogeneous and involve several molecules. It has been suggested that SCA individuals present a dyslipidemic phenotype and that lipid parameters are associated with severe clinical complications, such as pulmonary hypertension. We sought to investigate associations between lipid parameters and clinical manifestations, as well as other laboratory parameters in a population of pediatric SCA patients. Methods. Our cross-sectional evaluation included 126 SCA patients in steady state and who were not undergoing lipid-lowering therapy. Hematological and biochemical parameters were characterized, and previous clinical manifestations were investigated. Results. Total cholesterol and low-density lipoprotein cholesterol (LDL-C) levels were increased in patients with a previous history of pneumonia, which also positively correlated with HbS levels. Decreased LDL-C levels were also associated with leg ulcers and anemia. Elevated high-density lipoprotein cholesterol (HDL-C) levels were associated with pain crises, increased viscosity, and decreased hemolysis. Several studies have determined that lipids play a role in the vascular impairment seen in SCA, which was corroborated by our findings. Conclusions. In sum, our results suggest that total cholesterol, HDL-C, and LDL-C levels are associated with hemolysis and anemia markers and, most importantly, with clinical complications related to vasculopathy in SCA.

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Defective lipid metabolism in sickle cell anaemia subjects in vaso-occlusive crisis

Cited by 15 publications

References 22 publications

Electrocardiographic abnormalities and dyslipidaemic syndrome in children with sickle cell anaemia

Electrocardiographic abnormalities and dyslipidaemic syndrome in children with sickle cell anaemia

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

Investigation of Lipid Profile and Clinical Manifestations in SCA Children

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