Thyroid Function in Egyptian Children with Sickle Cell Anemia in Correlation with Iron Load.

BACKGROUND: Sickle Cell Disease (SCD) is characterized by defective hemoglobin synthesis, hemolytic anemia, frequent thrombosis and chronic organ damage including endocrine organs. AIM: To assess thyroid function in children with SCD in correlation and iron load. PATIENTS AND METHOD: This study was conducted on 40 children with SCD with iron overload (serum ferritin more than 1000 ng/ml) including 22 males and 18 females with their ages ranging from 11-14 years and mean age value of 11.63±1.36 years and 40 healthy children of matched age and sex as a control group. For all patients; complete blood count, hemoglobin electrophoresis, serum ferritin, serum iron, iron binding capacity and thyroid function including Free Thyroxine (FT4), Free Triiodothyronine (FT3), Thyroid Stimulating Hormone (TSH), Thyroid Peroxidase Antibody (TPOAb) and Thyroglobulin Antibody (TgAb) were done. RESULTS: Significantly higher serum ferritin and iron and significantly lower Total Iron Binding Capacity (TIBC) were found in patients compared with controls (mean serum ferritin was 1665.2±1387.65ng/ml in patients versus 192.55±107.2ng/ml in controls with p-value of 0. 007, mean serum iron was 164±83.9 ug/dl in patients versus 89.5±4.5ug/dl in controls with p-value of 0.039, mean TIBC was 238±44.5ug/dl in patients versus 308±11ug/dl in controls with p-value of 0.001). Significantly higher serum TSH and significantly lower Free T3 and Free T4 were found in patients compared with controls with no significant correlation between thyroid hormones and serum ferritin (mean serum TSH was 4.61±1.2 µIU/mL in patients versus 2.11 ± 0.54 µIU /mL in controls with p-value of 0. 045, mean serum FT3 was 2.61 ±1.3 pg/mL versus 3.93±0.47pg/mL in controls with p-value of 0.027, mean serum FT4 was 0.91±0.174 ng/dL versus 1.44± 0.164 ng/dLin controls with p-value of 0.047, r = - 0. 008 and p-value was 0. 973 for correlation between free T4 and serum ferritin, r = -0. 028 and p-value was 0. 9 for correlation between TSH and serum ferritin and r= - 0.259 and p-value was 0.27 for correlation betweenT3 and serum ferritin). There were no significant differences between patients and controls regarding thyroid peroxidase antibody and thyroglobulin antibody (mean serum thyroid peroxidase antibody was 22.45± 4.32 in patients versus 22.45 ± 3.21 in controls with p-value of 0.98 while mean serum thyroglobulin antibody was 12.32 ± 2.65 in patients versus 12.99 ± 2.34 in controls with p-value of 0.76. CONCLUSION: Thyroid hormones deficiency may occur in some patients with SCD. RECOMMENDATIONS: Regular assessment of thyroid function in children with SCD may be recommended as they are more vulnerable to develop hypothyroidism and may require replacement therapy.

Immune Status 'in Children with Beta Thalassemia' in Correlation 'with Iron Overload': Single Center Egyptian Study.

BACKGROUND: 'Beta thalassemia is inherited hemoglobin disorder resulting in chronic hemolytic anemia that requires lifelong transfusion therapy'. 'Repeated blood transfusions and RBCs hemolysis are the main causes of iron overload', which in addition to immune abnormalities, are common predisposing factors to infections in patients with thalassemia. The Aim of this Work: The aim of this work was to study immune status including T lymphocyte subsets and serum immunoglobulin levels 'in children with beta- thalassemia in correlation with iron overload'. PATIENTS AND METHODS: The present 'study was conducted on 40 children with beta thalassemia major under follow up at Hematology Unit, Pediatric Department, Tanta University' 'including 24 males and 16 females with mean' age value of 9. 22 ± 3.9 years and 20 'healthy children of matched age and sex as a control group'. All children included in the study were subjected to; 'complete blood count, Hb electrophoresis, serum iron status', T cell subsets including CD3, CD4 and CD8 and serum immunoglobulin levels including IgM, IgA and IgG. RESULTS: 'Pallor and jaundice were the most common presenting' clinical manifestations. Infective episodes 'were significantly higher in patients' compared with controls. There were significantly lower Hb, MCV and MCH levels and significantly higher WBCs and platelets counts, reticulocytes and lymphocytes percentage in patients than controls and no significant differences in MCHC between patients and controls. Serum ferritin and iron were 'significantly higher but TIBC was significantly lower in' patients than controls. CD3, CD4 and IgM were significantly lower but CD8, IgG, and IgA 'were significantly higher in patients than controls' with negative correlation between CD3, CD4, IgM and ferritin and positive correlation between CD8, IgG, IgA and ferritin. CONCLUSION: Iron overload can affect humeral and cell mediated immunity in patients with beta thalassemia with reduction of IgM, CD3 and CD4 and elevation of CD8, IgG, and IgA. RECOMMENDATIONS: Regular follow up of patients with beta thalassemia for detection of iron overload as it affects humeral and cell mediated immunity.

Protective Role of Silymarin on Hepatic and Renal Toxicity Induced by MTX Based Chemotherapy in Children with Acute Lymphoblastic Leukemia.

BACKGROUND: ALL is the most common childhood malignancy. The children with ALL are treated with methotrexate (MTX) based chemotherapy protocols. MTX causes unpredictable serious hepatic and renal side effects. Silymarin has antioxidant and anti-inflammatory activities and stimulates tissue regeneration. This study aims to evaluate the protective effects of Silymarin on MTX-based chemotherapy-induced Hepatic and renal toxicity in children with ALL. PATIENTS AND METHODS: 80 children with newly diagnosed ALL were enrolled in the study. They were randomly divided into two groups. Group I included 40 children with ages ranging from 4-13 years and the mean age of 6.85± 2.89 years, who received Silymarin 420 mg/day in 3 divided doses for one week after each MTX dose. Group II included 40 children, with ages ranging from 4-12 years and the mean age of 7.30±2.6 years, who received placebo for one week after MTX therapy. For all patients liver functions including serum bilirubin, total proteins, albumin, globulin and albumin-globulin ratio, alkaline phosphatase, ALT and AST, prothrombin time and activity and renal functions including blood urea and serum creatinine, serum cystatin C and urinary N-acetyl-beta-D-glucosaminidase were done to assess hepatic and renal toxicity before and after chemotherapy. RESULTS: There were no significant differences between group I and II as regard liver and renal functions before chemotherapy. After chemotherapy, there were significantly higher values of ALT and AST and alkaline phosphatase, and significantly lower Prothrombin activity in group II compared with group I. No significant differences between group I and II were found in total bilirubin, serum protein, and albumin levels. There was significantly lower blood urea, serum creatinine, and cystatin C and urinary N-acetyl-beta-D-glucosaminidase in group I compared with group II. CONCLUSION: Silymarin improved some hepatic and renal functions in children with ALL who received MTX-based chemotherapy protocols. RECOMMENDATIONS: Extensive multicenter studies could be recommended to prove the hepatic and renal protective effects of Silymarin in patients with ALL who received MTX-based chemotherapy protocols.