Background: Osteopathy is an important cause of morbidity in β-thalassemia major (TM). Although many of the etiopathological factors implicated in thalassemic osteoporosis commence in early disease phases during childhood, limited information exists on bone turnover in children with TM. This study was conducted with the objective to compare bone turnover markers (BTMs) in thalassemic children at different ages.
Methods: In a cross sectional case control study, 47 children (age range, 1.5-18 years) with TM were recruited. BTMs were compared to eighteen age- and sex-matched healthy controls and to 16 adults (age range, 19.67-31.08 years) with TM.
Results: Thalassemic children displayed unbalanced bone turnover with an increased bone resorption (shown by high levels of tartrate-resistant acid phosphatase 5b (TRACP5), receptor activator of nuclear factor-kappa B ligand (sRANKL) and sRANKL/osteoprotegerin (OPG) ratio) and a decreased bone neoformation (shown by low levels of osteocalcin (OC)) when compared to healthy children. TRACP5b was the only BTMs studied that showed a significant correlation with age in thalassemic children. For the whole thalassemic children group, regression analyses showed an influence of sex hormones replacement therapy on TRACP5b; pretransfusion hemoglobin and splenectomy on sRANKL; pretransfusion hemoglobin on sRANKL/OPG; and pretransfusion hemoglobin and serum ferritin on OC.
Conclusion: The present study confirms that TM has profound effects on bone metabolism starting from early childhood. The early onset of bone turnover disturbances in TM indicates the need to investigate possible option to intervene early.
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http://dx.doi.org/10.1016/j.pedneo.2016.08.012 | DOI Listing |
J Family Med Prim Care
November 2024
Department of Oral Medicine, Diagnosis and Radiology, Yogita Dental College and Hospital, Khed, Ratnagiri, Maharashtra, India.
Background: Thalassemia is a hemoglobinopathy-associated genetic disease resulting due to defective synthesis of globin chains, causing defects in the skeletal and oral structures.
Aim: This cross-sectional study was designed to analyze the prevalence of dental caries, oral health status, malocclusion status, and dental treatment needs in thalassemic children.
Materials And Methods: Institutional Ethical committee clearance was obtained before starting the study.
Cureus
October 2024
Department of Family and Community Medicine, College of Medicine, Taibah University, Medina, SAU.
Background: Thalassemia is a group of genetic disorders that result in a lack of hemoglobin (Hb) production. Children with thalassemia rely on regular blood transfusions for survival, which places a significant burden on their families and negatively impacts their quality of life (QOL).
Objective: This study aimed to assess QOL from the perspective of parents of thalassemic children in Medina City, Saudi Arabia.
Purpose: The purpose of this study was to assess serum homocysteine levels and their relationship with serum vitamin B12, folate, and ferritin levels in transfusion-dependent thalassemic children. This study was proposed due to a paucity of literature regarding the status of homocysteine levels in thalassemic children and their relationship with the levels of vitamins and iron overload (serum ferritin values).
Methodology: A descriptive observational study was conducted on transfusion-dependent thalassemic children aged 1-18 years, who were registered at the Thalassemia Day Care Centre (TDCC), Umaid Hospital, Dr.
Mymensingh Med J
October 2024
Dr Jannatul Ferdousi, Department of Paediatrics, Mymensingh Medical College Hospital, Mymensingh, Bangladesh; E-mail:
Most of the thalassemic children of Bangladesh are receiving repeated blood transfusion. But they do not receive chelation therapy due to financial constraints. As a result, iron overload occurs in various organs of these children.
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