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Bone mineral density in primarily preadolescent children with hemoglobin E/β-thalassemia with different severities
Pairunyar Nakavachara1, Praewvarin Weerakulwattana1, Julaporn Pooliam2
1Division of Pediatric Endocrinology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Insights
Most children with hemoglobin E/β-thalassemia have normal bone mineral density (BMD). However, transfusion-dependent (TD) patients showed more lumbar spine low bone mass than non-transfusion-dependent (NTD) patients.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Metabolic Bone Disease
Background:
- Children with β-thalassemia major and intermedia often exhibit low bone mass.
- Data on bone mineral density (BMD) in children with transfusion-dependent (TD) or non-transfusion-dependent (NTD) hemoglobin (Hb) E/β-thalassemia are limited.
Purpose of the Study:
- To determine the prevalence of low bone mass in preadolescent children with NTD and TD Hb E/β-thalassemia.
- To identify factors associated with low bone mass in these pediatric populations.
Main Methods:
- Dual-energy X-ray absorptiometry (DXA) was used to measure lumbar spine (LSBMD) and total body (TBBMD).
- The study included 59 children with NTD Hb E/β-thalassemia and 50 with TD Hb E/β-thalassemia.
- Bone mineral density was adjusted for height age and bone age.
Main Results:
- The prevalence of low bone mass was relatively low in both groups (NTD: 1.7%-10.2%; TD: 4%-14%).
- The NTD group had significantly lower total body BMD Z-scores (height age adjusted) compared to the TD group.
- Low lumbar spine bone mass (bone age adjusted) was significantly more prevalent in the TD group than the NTD group.
Conclusions:
- Most children with Hb E/β-thalassemia have normal bone mineral density.
- Patients with NTD Hb E/β-thalassemia exhibited lower total body BMD compared to TD patients.
- Low bone mass, particularly in the lumbar spine, was more pronounced in TD Hb E/β-thalassemia patients.
Background:
Children with β-thalassemia major and β-thalassemia intermedia frequently have low bone mass. However, studies of bone mineral density (BMD) in children with transfusion-dependent (TD) or non-transfusion-dependent (NTD) hemoglobin (Hb) E/β-thalassemia are scarce.
Objectives:
To determine the prevalence of low bone mass among mostly preadolescent children with NTD and TD Hb E/β thalassemia and the related factors.
Methods:
We investigated the BMD of the lumbar spine (LSBMD) and total body (TBBMD), measured by dual-energy X-ray absorptiometry, of 59 children with NTD Hb E/β-thalassemia and 50 with TD Hb E/β-thalassemia.
Results:
The median age of the patients was 10.4 (6.2-13.5) years in the NTD group and 10.3 (5.9-14.1) years in the TD group. These children had a relatively low prevalence of low bone mass (NTD: 1.7%-10.2%; TD: 4%-14%). The values varied with the bone site measured and the BMD size-adjustment method used (height age vs. bone age). The NTD group had significantly lower TBBMD Z-scores (adjusted for height age) than the TD group. The proportion of patients with low lumbar spine bone mass (adjusted for bone age) was significantly higher for the TD group than for the NTD group.
Conclusions:
Our study demonstrates that most children with either disease had normal BMD. Patients with the NTD form had a lower TBBMD than those with the TD form. Low bone mass affected the lumbar spine of patients with TD Hb E/β-thalassemia more than those with the NTD form.
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