Advertisement

International Journal of Hematology

, Volume 83, Issue 3, pp 224–228 | Cite as

Acute Painful Crises of Sickle Cell Disease in Egyptian Children: Predictors of Severity for a Preventive Strategy

  • Mohammad Al-Haggar
  • Hala Al-Marsafawy
  • Nabeel Abdel-Razek
  • Rizk Al-Baz
  • Abdel-Hamid Mostafac
Review Article

Abstract

The objective of this study was to predict which infants with sickle cell disease (SCD) are prone to develop severe painful crises. In a mixed hospital- and community-based population (76 cases), demographic data, SCD diagnostic parameters, and basal blood counts were correlated with 2 indices of SCD severity: pain rate (average number of days of painful episodes per year of follow-up) and serious life-threatening complications, such as hyperhemolytic crises. Data were analyzed blind to these indices.The Student t test, analysis of variance, and Pearson correlation were used to determine association with pain rate. Discriminant analysis was used for the prediction of SCD severity. Pain rate was significantly high in hemoglobin SS patients, especially in those with an early onset of dactylitis. There were statistically significant negative correlations of pain rate with basal hemoglobin level, hematocrit, percent hemoglobin F, and arterial oxygen saturation (P < .01 for all correlations).The top 3 predictors of SCD severity were (in descending order) genotype, basal hemoglobin level, and early dactylitis. Severe forms of SCD could be predicted in early infancy with 100% accuracy by using the basal diagnostic parameters for the disease. These infants should be closely monitored with special attention to ventilation status, even before the development of dactylitis.

Key words

Sickle cell disease Painful crises Prediction 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Miller ST, Sleeper LA, Pegelow CH, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. 2000;342:83–89.CrossRefPubMedGoogle Scholar
  2. 2.
    Kaul DK, Fabry ME, Nagel RL. The pathophysiology of vascular obstruction in the sickle syndromes. Blood Rev. 1996;10:29–44.CrossRefPubMedGoogle Scholar
  3. 3.
    Marouf R, Gupta R, Haider MZ, Al-Wazzan H, Adekile AD. Avascular necrosis of the femoral head in adult Kuwaiti sickle cell disease patients. Acta Haematol. 2003;110:11–15.CrossRefPubMedGoogle Scholar
  4. 4.
    Rahimi Z, Karimi M, Haghshenass M, Merat A. β-Globin gene cluster haplotypes in sickle cell patients from southwest Iran. Am J Hematol. 2003;74:156–160.CrossRefPubMedGoogle Scholar
  5. 5.
    Homi J, Levee L, Higgs D, Thomas P, Serjeant G. Pulse oximetry in a cohort study of sickle cell disease. Clin Lab Haematol. 1997;19:17–22.CrossRefPubMedGoogle Scholar
  6. 6.
    Needleman JP, Franco ME, Varlotta L, et al. Mechanisms of nocturnal oxyhemoglobin desaturation in children and adolescents with sickle cell disease. Pediatr Pulmonol. 1999;28:418–422.CrossRefPubMedGoogle Scholar
  7. 7.
    Wilkey O, Evans JPM, Telfer PT, Kirkham FJ. Predictors of nocturnal hypoxaemia in children with sickle cell disease [abstract]. Arch Dis Child. 2002;86(suppl 1)A12.Google Scholar
  8. 8.
    Haregrave DR, Wade A, Evans JPM, Hewes DKM, Kirkham FJ. Nocturnal oxygen saturation and painful sickle cell crises in children. Blood. 2003;101:846–848.CrossRefGoogle Scholar
  9. 9.
    Fairbanks VK, Klee GG. Biochemical aspects of hematology. In: Teitz NW, ed. Textbook of Clinical Chemistry. Philadelphia, Pa: WB Saunders; 1986:1540–1541.Google Scholar
  10. 10.
    Kim HC, Atwater J, Schwartz E. Separation of hemoglobins. In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA, eds. Hematology. 4th ed. New York, NY: McGraw Hill; 1990:1711–1714.Google Scholar
  11. 11.
    Echler G. Determination of glucose-6-phosphate dehydrogenase levels in red cell preparations. Am J Med Technol. 1983;49:259–262.PubMedGoogle Scholar
  12. 12.
    Al-Arrayed S, Hafadh N, Amin S, Al-Mukhareq H, Sanad H. Student screening for inherited blood disorders in Bahrain. East Mediterr Health J. 2003;9:344–352.PubMedGoogle Scholar
  13. 13.
    Keser I, Sanlioglu AD, Manguoglu E, et al. Molecular analysis of beta-thalassemia and sickle cell anemia in Antalya. Acta Haematol. 2004;111:205–210.CrossRefPubMedGoogle Scholar
  14. 14.
    Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease: rates and risk factors. N Engl J Med. 1991;325:11–16.CrossRefPubMedGoogle Scholar
  15. 15.
    Inati A, Taher A, Bou Alawi W, et al. β-Globin gene cluster haplotypes and HbF levels are not the only modulators of sickle cell disease in Lebanon. Eur J Haematol. 2003;70:79–83.CrossRefPubMedGoogle Scholar
  16. 16.
    Udezue E, Girshab AM. Differences between males and females in adults sickle cell pain crises in eastern Saudi Arabia. Ann Saudi Med. 2004;24:179–182.CrossRefPubMedGoogle Scholar
  17. 17.
    Graves PA, Kedar A, Koshy M, et al: RheothRx (Poloxamer 188) injection for the acute painful episode of sickle cell disease: a pilot study. Blood. 1997;90:2041–2046.Google Scholar
  18. 18.
    Cooper-Effa M, Blount W, Kaslow N, Rothenberg R, Eckman J. Role of spirituality in patients with sickle cell disease. J Am Board Fam Pract. 2001;14:116–122.PubMedGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2006

Authors and Affiliations

  • Mohammad Al-Haggar
    • 1
  • Hala Al-Marsafawy
    • 1
  • Nabeel Abdel-Razek
    • 1
  • Rizk Al-Baz
    • 2
  • Abdel-Hamid Mostafac
    • 3
  1. 1.Paediatrics Department, Faculty of MedicineMansoura UniversityMansouraEgypt
  2. 2.Mansoura University Children’s Hospital(Genetics Laboratories)Mansoura
  3. 3.Clinical Pathology Department, Faculty of MedicineAl-Azhar UniversityCairoEgypt

Personalised recommendations