Study of Some Biochemical Parameters in Iraqi Male Children with Thalassemia

Thalassemia is a term that refers to a group of genetic disorders characterized by a defect in the synthesis of hemoglobin. It is sometimes called Mediterranean anemia. Many biochemical changes in the blood accompany this disease. In this research, some biochemical parameters were measured in thalassemic patients and compared with healthy control group. These parameters include serum Iron, ferritin, TIBC, hemoglobin, uric acid, albumin, calcium, transferrin, and transferrin saturation percentage. The results of the research showed that there is a significant increase (P<0.05) in serum iron and ferritin in thalassemic patients group in comparison with healthy control group. A significant decrease (P<0.05) in serum uric acid, TIBC, transferrin protein concentration, and hemoglobin. There is no significant difference (P>0.05) in serum albumin and calcium in both groups. The results of this research can be explained by different mechanisms involving the hemolysis of erythrocytes and consequence precipitation of iron in the tissues. In addition, the hypoxia may be one of the reasons about the biochemical changes in thalassemic patients. Monitoring the measured parameters may be useful in the prognosis and follow up of the thalassemic patients. Introduction Thalassemia is a term that refers to a group of genetic disorders characterized by insufficient production of hemoglobin, wherein there is a defect in the synthesis of hemoglobin. It is sometimes called Mediterranean anemia. To understand how thalassemia affects the human body, we must first understand a little about how blood is made. If the body doesn't produce enough of alpha and a beta chains of globin, the red blood cells do not form properly and cannot carry sufficient oxygen. The result is anemia that begins in early childhood and lasts throughout life. Genes involved are those that control the production of alpha and beta globins contained in hemoglobin [1]. The two main types of thalassemia, alpha and beta, are named for the two protein chains that make up normal hemoglobin. Since thalassemia is not a single disorder but a group of related disorders that affect the human body in different ways. Thalassemia can be classified according to symptoms or to the genes affected. Thalassemia major that is studied in this work is inherited from both parents. Beta thalassemia tends to be more common in people from many ancestry including Mediterranean and Arabian Peninsula. Thalassemia in Iraq is a real problem due mainly to the deficiency in the equipments and drugs during different periods of lack of security and wars. Thalassemia is a problem in many areas in Iraq [2]. Of 1064 couples recruited from the Public Health Laboratory in Basra, southern Iraq, about 5% had beta-thalassemia trait and the carriers of major beta-gobin disorders comprised 11.48%. These defects constitute a real health problem and necessitate a management p lan and public health education for early diagnosis and therapy . In Iraq-Najaf IBN ALHAITHAM J. FO R PURE & APPL. SCI VO L. 22 (4) 2009 Government, till October-2007 there are 288 patients' files who are still treating in the thalassemia Unit in AL-Zahra'a Teaching Hospital. The patients are more dependent on blood transfusions, the more likely he or she is to be classified as thalassemia major. Biochemical changes may include; serum iron, increased/normal total iron binding capacity (TIBC), decreased/normal ferritin, increased/normal and increased transferrin saturation percentage (TISP). These factors in addition to others are studied in Iraqi patients in this work in addition to uric acid, calcium and albumin which are not studied extensively in thalassemic patients. Transferrin acts as iron transport protein between sites of absorption, storage, and use . Ferritin is a ubiquitous protein in which the only clearly defined function is the sequestration and storage of iron [3]. Ferritin synthesis may be induced by iron . The serum ferritin is a universally available and well-standardized measurement that has been the single most important laboratory measure of iron status during the past quarter century. The well-known limitation of the serum ferritin is the elevation in values independent of iron status that occur with acute or chronic inflammation, malignancy, liver diseases, and alcoholism .Therefore, transferrin-bound iron and transferrin saturation must be measured in the same serum sample with ferritin protein to distinguish iron status from inflammation. Just as a high serum ferritin protein may mean inflammation rather than iron overload, a low serum iron may mean inflammation rather than iron deficiency. Only if both serum iron and serum ferritin protein go in the same direction (i.e., both go up or down) can reasonably assess iron status from them. There is no treatment when asymptomatic. Different methods for treating Thalassemia are available Partial sp lenectomy was associated with a dramatic reduction of mortality in the Iraqi patients [2]. Thalassemia in Iraq was studied in different areas at different fields of study [4], but the complete picture about the chemical changes in Iraqi thalassemic patients is not understood yet. The aim of the study is to identify variation in biochemical changes in thallasemic patients in comparison with healthy control group. These parameters include serum Iron, ferritin, TIBC, hemoglobin, albumin, calcium, uric acid and TISP. Materials and Methods 1-Patients: Forty-five male children with thalassemias have participated in this research. Age range was (6-10 years) from AL-Zahraa Teaching Hospital for Obstetrics and Children during the period from 1-7-2007 till 1-10-2007. Thirty non-thalassemic children were taken as control group. 2-Biochemical (measurements): Blood was aspirated from individuals in the morning and collected in plain tubes for serum in order to estimate the following parameters. Hemoglobin electrophoresis shows; Hgb A decreased, Hgb F increased, and Hgb A2 variable. Biochemical Analysis Serum Iron was estimated by using colorimetric method[5], total Iron Binding Capacity were estimated by using colorimetric method by the following method: An excess of iron is added to the serum iron to saturate the transferrin. The unbound iron is precipitated with basic magnesium carbonate. After centrifugation the iron in the supernatant is determined). Hemoglobin was estimated by using colorimetric method. Calcium was estimated by using colorimetric method using O-Cresolphtaleine complex compound, at alkaline pH, Albumin was estimated by using colorimetric method by binding to the BCG dye to produce a blue green color. Uric acid was used for estimation by using colorimetric method. The Ferritin Quantitative Test is based on a solid phase enzyme-Linked IBN ALHAITHAM J. FO R PURE & APPL. SCI VO L. 22 (4) 2009 immunosorbent assay (ELISA). The assay system utilizes one rabbit anti-ferritin antibody for solid phase (microtitre wells) immobilization and a mouse monoclonal anti-ferritin antibody in the antibody-enzyme (horseradish peroxidase) conjugate solution. Transferrrin saturation percentage (TISP) was calculated by dividing serum iron concentration by TIBC (17) concentrations, using the formula: serum iron (μmol/L)/transferrin (g/L) x 3.98. The formula is based on the maximal binding of 2 mol Fe/mol of transferrin and a molecular weight of 79,570 Dalton for transferrin [6]. As shown in the formula : 98 . 3 * % . ) / ( . ) / ( saturation n Transferri L umol Iron S L g n Transferri  Biostatistical analysis: The results were expressed as (mean±standard deviation ). Pooled t-test was used for the comparison of a significant difference between the healthy and control groups in the measured parameters. Correlation coefficient (r) was also used for searching about any correlation between the parameters.