Homocysteine, folic acid, vitamin B[12] and pyridoxine: effects on vaso-occlusive crisis in sickle cell anemia and sickle-thalassemia

ABSTRACT

Hemoglubiopathies include sickle cell anemia and the Thalassemia. S/ beta 0-Thalassemia have a clinical course similar to HbSS. Elevated concentration of Homocysteine contribute to thrombosis, a frequent event in sickle cell anemia. Vitamin B[12], Pyridoxine, and folic acid deficiencies lead to dangerous increase in plasma Homocysteine. 1-To test whether children with sickle cell anemia and Sickle cell-Thalassemia have elevated concentration of serum Homocysteine with diminished level of folate, B[6], B[12]. 2-To determine whether hyperomocysteinaemia has a correlation with the frequency of Vaso-occlusive crisis. 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, B[12] and B[6] level. Statistical analysis was done, using the student T-test [P. value<0.05 is considered as statistically significant]. Pearson correlation analysis was performed. 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] micro mol/L respectively. Folic acid level was lower, B[12] 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. 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. Hemoglubiopathies are diseases caused by genetic