الفهرس 
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Abstract
Thalassemia is an inherited autosomal recessive blood disease that originated in the Mediterranean region.In thalassemia the genetic defect, which could be either mutation or deletion, results in reduced rate of synthesis of one of the globin chains causing the formation of abnormal hemoglobin molecule.
Patients with severe thalassemia require medical treatment, and a blood transfusion regimen was the first measure effective in prolonging life.
Blood transfusion should be initiated at an early age when the child is symptomatic and after an initial period of observation to assess wether the child can maintain an acceptable level of Hb without trasnsfusion.
Today, regular blood transfusion combined with well-monitored chelation therapy has become the standard therapy and has drastically changed the outlook for this population of patients.
This study (case control study) was designed to assess the level of vit D in patient with beta thalassemia. It included 50 children with Beta Thalassemia major., 29 males(58%) and 21 females (42%) with age ranging from 2 to 15 years. Twenty healthy unrelated individuals served as control group. Both patients and controls were recruited from Faculty of Medicine, Beni Suef University.
Each studied case was subjected to detailed history taking( age of diagnosis of thalassemia , rate of blood transfusion, chelation therapy and its type, Presence of complications of thalassemia, complications that could be attributed to vitamin D deficiency) ,General examination(Vital sigs , weight and height,Signs of heart disease,Mongoloid features), Abdominal examination and.Musculoskeletal system : to detect bone pain, tenderness,deformity, pathological fractures, muscle weakness.
laboratory investigations has been carried out as well including CBC, Retics ,Hemoglobin electrophoresis ,Serum ferritin ,Serum calcium & phosphorus levels ,Serum Alkaline phosphatase.
Specific laboratory test:Serum 25 hydroxy vitamin D3 level by Enzyme-linked immunosorbent assay( ELISA). There was a highly statistically significant difference between patients and controls as regards vitamin D deficiency (P<0.001). None of the 20controls were having vitamin D defiieny.
There were highly statistically differences between thalassemic patients and control groups in laboratory finding as regard serum calcium level, phosphorus level ( P<0.001) in both. Also there was a statistically significant difference between patients and controls as regards alkaline phosphatase level ( P=0.04).
Cases recorded many bone complication as bone pain, tenderness ,deformity ,pathological frature.
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There was a statistically significant difference between vitamin D deficiency and non deficiency patients as regards vitamin D level (P<0.001), serum calcium (P=0.008), both were lower in the deficient patients. No statistically significant difference between two groups as regards other parameters (P>0.005).
The current study also revealed a statistically significant difference between vitamin D deficiency and non deficiency patients as regards bony tenderness (P=0.01), bony deformity (P=0.03), splenomegaly (P=0.01) and patients having splenectomy (P=0.002).
There was a statistically significant negative correlation between vitamin D level and( age as r-value (-0.71), P<0.001.- duration of patient illness as r-value (-0.72), P<0.001.- weight as r-value (-0.70), P<0.001.- level and height as r-value (-0.70), P<0.001).
There was a statistically significant positive correlation between vitamin D level and serum calcium r-value (0.49), P<0.001.
There wasn’t any statistically significant correlation between vitamin D level and( serum phosphorus as P=0.75.- and serum alkaline phosphatase level as P=0.68)
We conclude that high prevalence of vitamin D deficiency occurs in thalassemic children and adolescents that may largely contribute to their bone diseases. Maintaning normal serum level of 25-OH D through oral intake of vitamin D and early correction of VDD by oral or parental use of vitamin D may significantly improve their bone mineral accretion and prevent bone disease .