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Journal of the National Medical Association logoLink to Journal of the National Medical Association
. 2003 Sep;95(9):813–817.

Reduced cholesterol levels in African-American adults with sickle cell disease.

Jaimie Shores 1, John Peterson 1, Dorothy VanderJagt 1, Robert H Glew 1
PMCID: PMC2594470  PMID: 14527048

Abstract

In a recent study of boys and girls with sickle cell disease (SCD) in Nigeria, a common finding with this genetic hematologic disorder was a marked reduction in total cholesterol. Epidemiologic studies have identified a relation between low serum levels of total cholesterol (< 130 mg/dL) and increased mortality from all causes. We were interested in knowing if hypocholesterolemia was present in African-American adults with SCD. We therefore compared the plasma lipid profiles of the 16 men and 20 women with SCD who received care at the University of Texas Medical Branch at Galveston between 1996 and 2001 with those of 2,415 gender-matched African Americans who were seen at the same hospital but who did not have SCD. The age-adjusted mean total cholesterol concentrations of the SCD males and females were 147 +/- 42 mg/dL and 179 +/- 36 mg/dL, compared to male and female control values of 200 +/- 75 mg/dL and 216 +/- 61 mg/dL, respectively. These differences between SCD subjects and controls were statistically significant (p < 0.001). The LDL-cholesterol levels of the men and women with SCD (68 +/- 28 mg/dl and 95 +/- 33 mg/dl, respectively) were also significantly reduced relative to the controls (121 +/- 58 mg/dl and 128 +/- 54 mg/dl, respectively, p = 0.001). The triglyceride levels of the men with SCD were much reduced relative to the male controls (102 +/- 34 mg/dL versus 194 +/- 215 mg/dL, p = 0.02), but were not different between the SCD females and their control group. The HDL-cholesterol levels of the SCD subjects and the controls were not different. These results indicate that total cholesterol and LDL-cholesterol concentrations are significantly reduced in adult men and women with SCD in the United States, and should heighten interest in the implication that low levels of cholesterol might exacerbate the medical problems inherent in this genetic disease.

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Selected References

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  1. Furbee James W., Jr, Francone Omar, Parks John S. In vivo contribution of LCAT to apolipoprotein B lipoprotein cholesteryl esters in LDL receptor and apolipoprotein E knockout mice. J Lipid Res. 2002 Mar;43(3):428–437. [PubMed] [Google Scholar]
  2. Glew R. H., Casados J., Huang Y. S., Chuang L. T., VanderJagt D. J. Correlation of the fatty acid composition and fluid property of the cholesteryl esters in the serum of Nigerian children with sickle cell disease and healthy controls. Prostaglandins Leukot Essent Fatty Acids. 2003 Jan;68(1):61–68. doi: 10.1016/s0952-3278(02)00275-2. [DOI] [PubMed] [Google Scholar]
  3. Glueck C. J., Kuller F. E., Hamer T., Rodriguez R., Sosa F., Sieve-Smith L., Morrison J. A. Hypocholesterolemia, hypertriglyceridemia, suicide, and suicide ideation in children hospitalized for psychiatric diseases. Pediatr Res. 1994 May;35(5):602–610. [PubMed] [Google Scholar]
  4. Goldstein J. L., Brown M. S. Regulation of the mevalonate pathway. Nature. 1990 Feb 1;343(6257):425–430. doi: 10.1038/343425a0. [DOI] [PubMed] [Google Scholar]
  5. Jonas A. Lecithin cholesterol acyltransferase. Biochim Biophys Acta. 2000 Dec 15;1529(1-3):245–256. doi: 10.1016/s1388-1981(00)00153-0. [DOI] [PubMed] [Google Scholar]
  6. Oforofuo I. A., Adedeji M. O. Effect of sickle-cell gene expression on plasma cholesterol in a Nigerian population. Clin Biochem. 1994 Dec;27(6):505–508. doi: 10.1016/0009-9120(94)00049-2. [DOI] [PubMed] [Google Scholar]
  7. Saha N., Samuel A. P. Sickle cell gene and liver functions in a Sudanese population. Acta Haematol. 1982;68(1):65–67. doi: 10.1159/000206951. [DOI] [PubMed] [Google Scholar]
  8. Schatz I. J., Masaki K., Yano K., Chen R., Rodriguez B. L., Curb J. D. Cholesterol and all-cause mortality in elderly people from the Honolulu Heart Program: a cohort study. Lancet. 2001 Aug 4;358(9279):351–355. doi: 10.1016/S0140-6736(01)05553-2. [DOI] [PubMed] [Google Scholar]
  9. Song Y. M., Sung J., Kim J. S. Which cholesterol level is related to the lowest mortality in a population with low mean cholesterol level: a 6.4-year follow-up study of 482,472 Korean men. Am J Epidemiol. 2000 Apr 15;151(8):739–747. doi: 10.1093/oxfordjournals.aje.a010272. [DOI] [PubMed] [Google Scholar]
  10. VanderJagt D. J., Bonnett C., Okolo S. N., Glew R. H. Assessment of the bone status of Nigerian children and adolescents with sickle cell disease using calcaneal ultrasound and serum markers of bone metabolism. Calcif Tissue Int. 2002 Jul 24;71(2):133–140. doi: 10.1007/s00223-001-1107-x. [DOI] [PubMed] [Google Scholar]
  11. VanderJagt D. J., Kanellis G. J., Isichei C., Patuszyn A., Glew R. H. Serum and urinary amino acid levels in sickle cell disease. J Trop Pediatr. 1997 Aug;43(4):220–225. doi: 10.1093/tropej/43.4.220. [DOI] [PubMed] [Google Scholar]
  12. VanderJagt D. J., Okolo S. N., Rabasa A. I., Glew R. H. Bioelectrical impedance analysis of the body composition of Nigerian children with sickle cell disease. J Trop Pediatr. 2000 Apr;46(2):67–72. doi: 10.1093/tropej/46.2.67. [DOI] [PubMed] [Google Scholar]
  13. el-Hazmi M. A., Jabbar F. A., Warsy A. S. Cholesterol and triglyceride level in patients with sickle cell anaemia. Scand J Clin Lab Invest. 1987 Jun;47(4):351–354. [PubMed] [Google Scholar]

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