الفهرس 
SICKLE CELL DISEASEOnly 14 pages are availabe for public view
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Abstract
In the present study we have evaluated 30 patients with sickle cell disease and sickle beta thalassemia who were regularly attending the pediatric Hematology Clinic, Ain Shams University.
There were 19 patients with sickle beta thalassemia and 11 patients with sickle cell disease, according to their initial hemoglobin electrophoresis.
Data was recorded regarding demographic data, frequency of blood transfusion /year, transfusion index, Frequency of hospital admission, Hydroxyurea treatment, dose and complications, laboratory investigations were done including CBC, hemoglobin electrophoresis at the time of study entry, markers of hemolysis (retics, LDH, total and indirect bilirubin)
L glutamine supplementation was given to all studied patients in adose of 0.6g/kg/day for 4 weeks.
All the clinical and laboratory investigations were done one year before and one year after therapy.
As regard the demographic data, the mean age of all studied patients was 13.07+6.97 years. There were 19 male patients (63.3%), and 11 female patient (36.7%).
The mean duration of illness of the studied patients was 9.29 + 6.82 years.
The mean hemoglobin level of the studied patients before glutamine therapy was 8.08 + 1.70 g/dl whereas, the mean hemoglobin level after therapy was 8.7 + 1.66 g/dl
In the current study, 21 (70 %) of the studied patients were using hydroxyurea : 14 patients (46.7 %) received dose of 15-30 mg/kg/day, whereas : 7 patients (23.3%) received dose > 30 mg/kg/day.
The current study reported that there was no statistical significant difference between the two groups(sickle cell anemia and sickle beta thalassemia) as regards the clinical and the laboratory parameters either before or after treatment (P>0.05).
Also, the current study shows that there was no statistical significant difference between the other two groups (patient receiving hydroxyurea and patient not receiving hydroxyurea) as regards the clinical and the laboratory parameters either before or after treatment (P>0.05).
Patients with Sickle cell anemia showed significantly higher Hb S than those with Sickle beta Thalassemia (P<0.05).
As regard all patients There was a significant increase in Hb with decreased transfusion index and frequency of transfusion (P<0.05). There was a significant decrease in frequency of Vaso-occlusive crisis frequency of hospital admission/ year
Both studied groups showed decreased total , indirect bilirubin, reticulocyte and LDH after treatment but this did not reach statistical significant (P<0.05).
Further more, standard deviation score of weight and height significantly increased after therapy with decreased transfusion index (P<0.05).
There was a significant decrease in frequency of Vaso-occlusive crisis frequency of hospital admission/ year
In conclusion, L –glutamine is an inexpensive compound , its supplementation improve hemoglobin level, decrease frequency of hospital admission and frequency of blood transfusion
It is also considered as an effective way to promote the growth of children with sickle cell disease as well as improvement in chronic pain level and frequency of vaso-occlusive crisis in patients with SCD with no difference in clinical and laboratory response between patients with sickle cell anemia and sickle Beta thalassemia , and no additive effect of the use of hydroxyurea treatment .