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Journal of Tropical Pediatrics 1992 38(4):196-198; doi:10.1093/tropej/38.4.196
© 1992 by Oxford University Press
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Sickle Cell/ß-Thalassemia in North Jordan

N. Bashir*, M. Barkawi** and L. Sharif***

*Department of Biochemistry, School of Medicine, University of Science and Technology Irbid, Jordan
**Pediatrics Department, Princess Basma Hospital Jordan,
***Veterinary Clinical Sciences Department, Jordan University of Science and Technology Irbid, Jordan

Clinical and haematological features of 50 patients with sickle cell/ß-thalassemia (SB0 or SB+) are investigated. Total haemoglobin value was not significantly different (P>0.05) in both types. Haemoglobin F and S were significantly higher (P<0.05) in SB0 than SB+ while haemoglobin A2 level was lower in SB0 than SB+. One SB0 case with exceptionally high HbF (32 per cent) with severe clinical course was found. In this case, the high HbF did not ameliorate the clinical severity. Heterogeneity in each type of sickle cell/ß-thalassemia is discussed. The differentiation of SB0 and sickle cell disease is best made on the basis of family study.


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