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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
[Relationship between growth disorders and iron overload in children with beta-thalassemia major]
Yong-Lan Huang1, Sha Liu, Ting Xia
1Department of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou 510180, China. huangylan@tom.com
Insights
Children with beta-thalassemia major often experience short stature, low weight, and delayed sexual development. These growth disorders are frequently linked to iron overload in these patients.
Area of Science:
- Pediatric Hematology
- Endocrinology
- Growth and Development Studies
Context:
- Beta-thalassemia major is a severe inherited blood disorder requiring regular blood transfusions.
- Chronic transfusions lead to iron overload, a significant complication affecting multiple organ systems.
- Understanding growth and development in these children is crucial for managing long-term health outcomes.
Purpose:
- To investigate the prevalence of growth and development issues in children with beta-thalassemia major.
- To explore the correlation between growth disorders and the extent of iron overload.
- To assess parameters like height, weight, and sexual maturation in affected children.
Summary:
- This study examined 50 children with beta-thalassemia major, assessing their growth and development.
- Results indicated high rates of short stature (48%), underweight, and delayed sexual development.
- Elevated serum ferritin levels, indicative of iron overload, were significantly associated with poorer growth parameters and lower hemoglobin levels.
Impact:
- Highlights the significant impact of beta-thalassemia major and its treatment on pediatric growth and development.
- Emphasizes the critical role of monitoring and managing iron overload to mitigate these complications.
- Provides evidence for the link between iron overload and growth deficits, informing clinical practice and patient care strategies.
Objective:
To study the status of growth and development and the relationship between growth disorders and iron overload in children with beta-thalassemia major.
Methods:
Fifty children with beta-thalassemia major and who received blood transfusion therapy regularly (age: 9 months-17 years) were enrolled. They were subjected to a thorough history taking, clinical examinations, and laboratory examinations, including complete blood count, alanine transferasa (ALT) and serum ferritin. The physical growth parameters, such as height and weight, were compared with the reference values of Chinese children.
Results:
Twenty-four patients (48%) were of short stature with height under the 3th percentile. Among them, 15 cases presented with their height and weight both under the 3th percentile. Spontaneous sex development was seen in 7 cases out of 21 over 10-year-old patients. No sex development was found in 4 out of 8 patients who were over 14 years old. The patients with a height under the 10th percentile (n=31) had higher serum ferritin levels (8239.2+/-5865.5 mg/L vs 5028.1+/-3885.7 mg/L; P<0.05) and lower hemoglobin levels (68.2+/-12.3 g/L vs 79.7+/-14.5 g/L; P<0.05) as well as hepatomegaly when compared with those patients with a height over the 10th percentile (n=19). Serum ferritin levels in 20 patients with a weight under the 10th percentile were significantly higher than those in 30 patients with a height over the 10th percentile (9165.5+/-6042.5 mg/L vs 5567.3+/-4447.3 mg/L; P<0.05).
Conclusions:
Short stature, low weight and sex development delay are common in children with beta-thalassemia major. This may be related to iron overload.
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