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

Background: The chronic hemolytic anemia experienced by sickle cell disease (SCD) patients leads to adverse effects on oxygen transport by the blood and to a decrease in oxygen availability for peripheral tissues. Limited tissue oxygen availability has the potential to modify events of intracellular...

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Published in:JPEN. Journal of parenteral and enteral nutrition 2007-07, Vol.31 (4), p.263-268
Main Authors: Buchowski, Maciej S., Swift, Larry L., Akohoue, Sylvie A., Shankar, Sadhna M., Flakoll, Paul J., Abumrad, Naji
Format: Article
Language:English
Subjects:
Adolescent
Adult
Anemia, Sickle Cell - blood
Anemia, Sickle Cell - metabolism
Anemias. Hemoglobinopathies
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Case-Control Studies
Diseases of red blood cells
Emergency and intensive care: metabolism and nutrition disorders. Enteral and parenteral nutrition
Erythrocyte Membrane - chemistry
Erythrocyte Membrane - metabolism
Fasting - blood
Fasting - metabolism
Fatty Acids, Nonesterified - blood
Fatty Acids, Nonesterified - chemistry
Female
Hematologic and hematopoietic diseases
Humans
Infusions, Parenteral
Intensive care medicine
Lipid Metabolism - physiology
Male
Medical sciences
Middle Aged
Phospholipids - blood
Phospholipids - chemistry
Postprandial Period - physiology
Triglycerides - blood
Triglycerides - chemistry
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Summary:Background: The chronic hemolytic anemia experienced by sickle cell disease (SCD) patients leads to adverse effects on oxygen transport by the blood and to a decrease in oxygen availability for peripheral tissues. Limited tissue oxygen availability has the potential to modify events of intracellular metabolism and, thus, alter lipid homeostasis. Methods: The impact of SCD on plasma fatty acid homeostasis was determined in 8 African American SCD patients and in 6 healthy African American control subjects under postabsorptive conditions and during a 3-hour IV infusion of a nutrient solution containing lipid, glucose, and amino acids. Results: SCD patients had higher fasting levels of plasma nonesterified fatty acids (NEFA), triglycerides, and phospholipids than healthy controls. Similarly, SCD patients had higher fasting levels of fatty acids in plasma triglycerides and phospholipids than healthy controls. Infusion of nutrients resulted in equivalent plasma NEFA profiles, total NEFA, and triglycerides in SCD patients and controls. However, the plasma phospholipid concentrations and fatty acid composition of plasma triglycerides and phospholipids were significantly higher in SCD patients; in particular, plasma pools of oleic acid were consistently increased in SCD. Plasma free oleic acid levels were elevated basally, leading to increased oleic acid content in triglycerides and phospholipids both postabsorptively and during nutrient infusion. Conclusions: There is an underlying defect in lipid metabolism associated with SCD best manifested during the fasting state. This abnormality in lipid homeostasis has the potential to alter red blood cell (RBC) membrane fluidity and function in SCD patients. Sickle cell disease (SCD) is a genetic disorder usually characterized by marked clinical severity and perturbations in energy and nutrient metabolism. We found there is an underlying defect in lipid metabolism associated with SCD that is more apparent as the metabolic state progresses from postabsorptive to nutrient availability.
ISSN:0148-6071
1941-2444
DOI:10.1177/0148607107031004263