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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Thyroid Function in Egyptian Children with Sickle Cell Anemia in Correlation with Iron Load
Adel A Hagag1, Hassan M El-Asy1, Ibrahim M Badraia1
1Pediatrics Departments, Faculty of Medicine, Tanta University, Tanta, Gharbia, Egypt.
Insights
Children with Sickle Cell Disease (SCD) and iron overload show impaired thyroid function, with lower Free T3 and Free T4 levels. Regular thyroid monitoring is recommended for these vulnerable patients.
Area of Science:
- Endocrinology
- Hematology
- Pediatrics
Background:
- Sickle Cell Disease (SCD) is a genetic disorder affecting hemoglobin, leading to hemolytic anemia, thrombosis, and chronic organ damage, including endocrine dysfunction.
- Iron overload is a common complication in SCD patients, potentially exacerbating organ damage and affecting endocrine function.
Purpose of the Study:
- To investigate thyroid function in children diagnosed with Sickle Cell Disease (SCD) and significant iron overload.
- To explore the correlation between iron load markers (serum ferritin, serum iron, TIBC) and thyroid hormone levels in pediatric SCD patients.
Main Methods:
- A cohort of 40 children with SCD and iron overload (serum ferritin > 1000 ng/ml) was compared to 40 age- and sex-matched healthy controls.
- Comprehensive assessments included complete blood count, hemoglobin electrophoresis, iron studies (serum ferritin, iron, TIBC), and thyroid function tests (FT4, FT3, TSH, TPOAb, TgAb).
Main Results:
- SCD patients exhibited significantly higher serum ferritin and iron levels, and lower Total Iron Binding Capacity (TIBC) compared to controls.
- Patients with SCD showed significantly elevated Thyroid Stimulating Hormone (TSH) and reduced levels of Free Triiodothyronine (FT3) and Free Thyroxine (FT4).
- No significant correlation was found between serum ferritin levels and thyroid hormone levels (FT4, FT3, TSH) in the SCD cohort.
Conclusions:
- Children with Sickle Cell Disease and iron overload are at risk for developing thyroid hormone deficiencies.
- Regular screening of thyroid function is advisable for children with SCD due to their increased susceptibility to hypothyroidism and potential need for hormone replacement therapy.
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.
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