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Inheritance of the gene in a homozygous form or a compound heterozygous form with another abnormal Hb often results in clinical disease.
The disease is characterized by chronic debilitating complications often interrupted by the episodes of crises (acute complications) and periods of wellness (steady state).
Subscales 1, 2, 4, and 7 assess physical health; subscales 3 and 5 assess emotional health, subscale 6 assesses social well-being while subscale 8 assesses general health. The higher the score, the better the quality of life tested by the subscale.
The study was approved by the Ethics Committee of the University of Benin Teaching Hospital, Benin City as well as the management of the Sickle Cell Centre, Edo State.
Fifty-nine (96.7%) of the SCD respondents were hemoglobin (Hb) SS and 2 (3.3%) were Hb SC.
The physical function, limitations due to physical health, general health, and pain scores were significantly lower in the SCD population compared to the controls (P = 0.000, 0.000, 0.000, and 0.002, respectively.
Afr J Med Health Sci [serial online] 2016 [cited 2018 Jul 18];-5. 2016/15/2/80/197965Sickle cell disease (SCD) is a hereditary disease of hemoglobin (Hb) due to the inheritance of an S gene on the b globin chain.
The disorder is due to a mutation at position six of the b globin chain resulting in the substitution of glutamic acid by valine.
Medical Students of the University of Benin, Benin City who had knowledge of their Hb phenotype were recruited as controls.
Methodology: The HRQo L of SCD subjects was evaluated using the Medical Outcome Study 36-item survey tool.