Evaluation of the vitamin D and biomedical statuses of young children with β-thalassemia major at a single center in southern China

Abstract

BackgroundIn young children, β-thalassemia major (β-TM) is associated with potentially severe clinical characteristics, including poor growth, feeding difficulties, hepatosplenomegaly, bone metabolic disorders, and skeletal abnormalities.MethodsIn this study, we reviewed the demographic and clinical characteristics (e.g., age, sex, duration of blood transfusion and chelating therapy, and vitamin supplementation) and serum biomarker levels (e.g., iron accumulation, bone metabolism, liver, kidney, and thyroid function markers) of 32 patients that received regular blood transfusion at a single center in southern China with the aim of stratifying the risk of severe complications such as osteopenia, endocrinopathies, and multi-organ failures.ResultsAlthough all patients exhibited moderately to strongly elevated serum ferritin levels, this biomarker was significantly higher in children older than ≥5 years, compared to younger children (*p < 0.05, 1512 ± 192.6 vs. 2337 ± 299.8 ng/ml, Mann-Whitney U test). Older children had a significantly lower 25-hydroxy vitamin D3 (25(OH)D3) level, compared to younger children (**p < 0.01, 34.25 ± 11.06 vs. 23.05 ± 9.95 ng/ml, Mann-Whitney U test). No age-related differences were observed in serum calcium, phosphorus, and PTH levels. Regarding liver function, the serum alanine aminotransferase (ALT) level was significantly increased in children older than ≥5 years, compared to younger children (*p < 0.05, 19.17 ± 2.44 vs. 43.45 ± 9.82I U/ml, Mann-Whitney U test). However, no age-related differences were observed in the serum levels of other liver or kidney and thyroid biomarkers.ConclusionsOur results suggest that in older children, hepatic iron overload may be associated with a low serum concentration of 25(OH)D3, an indicator of vitamin D deficiency and altered bone metabolism. Iron accumulation may also be associated with a higher concentration of ALT, a sensitive marker of liver malfunction. These findings may provide important clinical indications of the need for intervention to prevent severe complications in children with β thalassemia.

Assessment of the Nutritional Status, Bone Mineralization, and Anthropometrics of Children with Thalassemia Major

“…Our study revealed that 6.9% of patients have osteopenia and 6.9% have osteoporosis. Vitamin D deficiency frequency was 72.41% and was similar to prior studies, which have reported as 54%, 69.8%, and 90% 10 , 15 , 16 . Vitamin D deficiency is thought to be related to hepatic dysfunction and defective vitamin D hydroxylation due to iron overload 17 .No significant relationship or correlation was determined between vitamin D and ferritin.…”

“…Vitamin D deficiency is thought to be related to hepatic dysfunction and defective vitamin D hydroxylation due to iron overload 17 .No significant relationship or correlation was determined between vitamin D and ferritin. Yu et al’s 16 study revealed normal vitamin D levels in patients <5 years and that the deficiency progressively increased with age. Our study revealed no significant difference between the age groups.…”

“…Our study revealed no significant difference between the age groups. Bone metabolism defects have been demonstrated in many studies; however, their cause remains controversial 15 , 16 , 17 . The risk of developing endocrine disorders increases as ferritin increases 16 , 17 , 18 , 19 .…”

“…Bone metabolism defects have been demonstrated in many studies; however, their cause remains controversial 15 , 16 , 17 . The risk of developing endocrine disorders increases as ferritin increases 16 , 17 , 18 , 19 . The study conducted by Casale et al 14 revealed hypoparathyroidism in 2.3% of patients.…”

Nutrition in Thalassemia: A Systematic Review of Deficiency, Relations to Morbidity, and Supplementation Recommendations

“…Both Nakavachara and Viprakasit 61 and Fung et al 62 found that non-transfusion-dependent subjects with Thal had significantly lower 25(OH)D than transfusion-dependent subjects, perhaps due to infrequent visits with medical personnel and inadequate nutrition monitoring. Lumbar spine bone mineral density (BMD) positively correlated with 25(OH)D. 42,63,64 Both Claster et al 12 and Wood et al 57 found that age was negatively correlated with 25(OH)D. Furthermore, Yu et al 24 found older subjects with Thal to have lower absolute 25(OH) D, perhaps related to reduced time spent outdoors compared with younger subjects. In contrast, Vogiatzi et al 65 observed that adolescents with Thal residing in the United States had lower 25(OH)D than children or adults, possibly related to increased time indoors at school.…”