Homocysteine ,Folic acid ,Vitamin B12 and Pyridoxine : Effects on Vaso-Occlusive Crisis in Sickle Cell Anemia and Sickle –Thalassemia

Abstract

ABSTRACT:BACKGROUND:Hemoglubiopathies include sickle cell anemia and the Thalassemia . S/ β0- Thalassemia have a clinical course similar to HbSS. Elevated concentration of Homocysteine contribute to thrombosis , a frequent event in sickle cell anemia . Vitamin B12, Pyridoxine , and folic acid deficiencies lead to dangerous increase in plasma Homocysteine.OBJECTIVE:1- To test whether children with sickle cell anemia and Sickle cell – Thalassemia have elevated concentration of serum Homocysteine with diminished level of folate, B6, B12.2- To determine whether hyperomocysteinaemia has a correlation with the frequency of Vaso-occlusive crisis.PATIENTS AND METHOD:A case- control study was carried over a period of one year from Jan.- Dec. 2010 inclusive, 30 patients were collected from the Thalassemia centre in Ibn –AL-Baldy Hospital together with healthy 30 cases, age and sex matched ,were taken from AL- Kadhimiyia Teaching Hospital. Venous blood sample were aspirated from both groups to estimate serum Homocysteine, Folic acid, B12 and B6 level. Statistical analysis was done, using the student T-test ( P. value < 0.05 is considered as statistically significant) . Pearson correlation analysis was performed.RESULTS :The age of the patients range between (5-29) years, the majority of the patients were between ( 10 -19 ) years , 10 cases ( 38 .46 %). More than one half were male ,16 cases ( 61.54 %) . Sickle cell – Thalassemia constitute 20 cases (76.93%). Vaso-occlusive crisis was mainly involving the large joints, 15 cases (57.69 %). Mild attacks constitute more than half of the patients, 16 cases (61.54 %).Homocysteine level was higher in the patients group compared with control group with a mean and standard deviation of (44.52 ± 23.008) and (18.65 ± 4.56)μmol/L respectively . Folic acid level was lower, B12 level was higher, B6 level was lower in the patients group compared with control group with a mean and standard deviation of (11.32± 3.23) and (14.71 ± 3.39)ng /ml, (172.57± 61.34) and (103.45 ± 30.45)pg /ml, (4.43± 3.93) and (10.23 ± 2.30) ng/ml respectively, the results were statistically not significant, P. value > 0.05. Significant inverse correlation was found between Homocysteine level and B6 level. A strong positive correlation between Homocysteine level and the frequency of Vaso-occlusive crisis was found.CONCLUSION:Patients with sickle cell disease have high serum level of Homocysteine with low level of folic acid and pyridoxine. This Hyperomocysteinaemia is significantly inversely correlated with pyridoxine deficiency , but positively correlated with the frequency of Vaso-occlusive crisis.