A STUDY OF BONE AND JOINT INVOLVEMENT IN THALASSEMIA MAJOR
(Abstract release date: 05/19/16)
EHA Library. Kakkar S. 06/09/16; 133043; E1494
Dr. Shruti Kakkar
Contributions
Contributions
Abstract
Abstract: E1494
Type: Eposter Presentation
Background
Thalassemia major is an inherited hemoglobinopathy causing imbalance in globin chain synthesis, ineffective erythropoiesis and increased peripheral haemolysis. Thalassemia patients have a variety of bone and joint disorders like bone pain, deformity, growth failure, rickets, scoliosis, spinal deformity, nerve compression, pathological fractures, osteopenia and osteoporosis. There is a paucity of data about the extent of bone and joint disorders in thalassemia in developing countries.
Aims
1. To determine bone and joint involvement in thalassemia major.2. To evaluate serum biochemical parameters of bone formation and resorption and their relation with bone and joint formation.3. To evaluate DEXA scan scores of Thalassemia patients and find a correlation, if any, with daily physical activity.
Methods
The study was carried out on 40 thalassemia patients between 5-18 years of age under regular follow up at our centre. All patients were interviewed as per a well-structured proforma for symptoms relating to bone and joint disease as described by the patient/parents in past one year and physical activity by QAPACE questionnaire. Venous blood samples were drawn under aseptic conditions and used for estimation of biochemical parameters (calcium, phosphorus, alkaline phosphatase, vitamin D, parathyroid hormone, thyroid hormone and serum ferritin). Enrolled subjects were scanned for bone mineral density (BMD) at femur neck, trochanter and Ward's angle using dual energy X ray absorptiometry.
Results
A total of 40 patients were enrolled in the study (M:F = 80:20). Half the patients (n=20) had symptoms pertaining to bone and joint disease. The most common symptom was leg pain (42.50%), followed by backache (22.50%), generalized weakness (20%) and joint pains (20%). Asymptomatic patients had greater mean serum vitamin D as compared to the symptomatic patients (22.2 vs 15.08 ng/ml)(p= 0.225). Five patients were detected to have osteopenia, all above 10 years of age. The mean BMD in symptomatic patients at femur neck, trochanter and ward's angle was 0.781, 0.639 and 0.735 g/cm2 respectively as compared to 0.754, 0.635 and 0.722 g/cm2 in asymptomatic patients. The difference was not statistically significant. Patients with low BMD had lower mean pretransfusion Hb (< 9 g/dl) (p= 0.01).Eighty percent of osteopenic patients had sedentary lifestyle as compared to 44% of patients with normal BMD. Bone mineral density did not have any correlation with vitamin D levels, serum ferritin and type of chelation therapy.
Conclusion
Pre transfusion hemoglobin of more than 9 g/dL has a protective role in maintaining good bone health and bone mineral density and helps to prevent osteopenia. Engaging thalassemia patients in physical activity has a positive effect on bone mineral density. Low vitamin D levels contribute to symptoms of bone and joint involvement in thalassemia and the same needs to be determined and supplemented.
Session topic: E-poster
Keyword(s): Osteoporosis, Thalassemia
Type: Eposter Presentation
Background
Thalassemia major is an inherited hemoglobinopathy causing imbalance in globin chain synthesis, ineffective erythropoiesis and increased peripheral haemolysis. Thalassemia patients have a variety of bone and joint disorders like bone pain, deformity, growth failure, rickets, scoliosis, spinal deformity, nerve compression, pathological fractures, osteopenia and osteoporosis. There is a paucity of data about the extent of bone and joint disorders in thalassemia in developing countries.
Aims
1. To determine bone and joint involvement in thalassemia major.2. To evaluate serum biochemical parameters of bone formation and resorption and their relation with bone and joint formation.3. To evaluate DEXA scan scores of Thalassemia patients and find a correlation, if any, with daily physical activity.
Methods
The study was carried out on 40 thalassemia patients between 5-18 years of age under regular follow up at our centre. All patients were interviewed as per a well-structured proforma for symptoms relating to bone and joint disease as described by the patient/parents in past one year and physical activity by QAPACE questionnaire. Venous blood samples were drawn under aseptic conditions and used for estimation of biochemical parameters (calcium, phosphorus, alkaline phosphatase, vitamin D, parathyroid hormone, thyroid hormone and serum ferritin). Enrolled subjects were scanned for bone mineral density (BMD) at femur neck, trochanter and Ward's angle using dual energy X ray absorptiometry.
Results
A total of 40 patients were enrolled in the study (M:F = 80:20). Half the patients (n=20) had symptoms pertaining to bone and joint disease. The most common symptom was leg pain (42.50%), followed by backache (22.50%), generalized weakness (20%) and joint pains (20%). Asymptomatic patients had greater mean serum vitamin D as compared to the symptomatic patients (22.2 vs 15.08 ng/ml)(p= 0.225). Five patients were detected to have osteopenia, all above 10 years of age. The mean BMD in symptomatic patients at femur neck, trochanter and ward's angle was 0.781, 0.639 and 0.735 g/cm2 respectively as compared to 0.754, 0.635 and 0.722 g/cm2 in asymptomatic patients. The difference was not statistically significant. Patients with low BMD had lower mean pretransfusion Hb (< 9 g/dl) (p= 0.01).Eighty percent of osteopenic patients had sedentary lifestyle as compared to 44% of patients with normal BMD. Bone mineral density did not have any correlation with vitamin D levels, serum ferritin and type of chelation therapy.
Conclusion
Pre transfusion hemoglobin of more than 9 g/dL has a protective role in maintaining good bone health and bone mineral density and helps to prevent osteopenia. Engaging thalassemia patients in physical activity has a positive effect on bone mineral density. Low vitamin D levels contribute to symptoms of bone and joint involvement in thalassemia and the same needs to be determined and supplemented.
Session topic: E-poster
Keyword(s): Osteoporosis, Thalassemia
Abstract: E1494
Type: Eposter Presentation
Background
Thalassemia major is an inherited hemoglobinopathy causing imbalance in globin chain synthesis, ineffective erythropoiesis and increased peripheral haemolysis. Thalassemia patients have a variety of bone and joint disorders like bone pain, deformity, growth failure, rickets, scoliosis, spinal deformity, nerve compression, pathological fractures, osteopenia and osteoporosis. There is a paucity of data about the extent of bone and joint disorders in thalassemia in developing countries.
Aims
1. To determine bone and joint involvement in thalassemia major.2. To evaluate serum biochemical parameters of bone formation and resorption and their relation with bone and joint formation.3. To evaluate DEXA scan scores of Thalassemia patients and find a correlation, if any, with daily physical activity.
Methods
The study was carried out on 40 thalassemia patients between 5-18 years of age under regular follow up at our centre. All patients were interviewed as per a well-structured proforma for symptoms relating to bone and joint disease as described by the patient/parents in past one year and physical activity by QAPACE questionnaire. Venous blood samples were drawn under aseptic conditions and used for estimation of biochemical parameters (calcium, phosphorus, alkaline phosphatase, vitamin D, parathyroid hormone, thyroid hormone and serum ferritin). Enrolled subjects were scanned for bone mineral density (BMD) at femur neck, trochanter and Ward's angle using dual energy X ray absorptiometry.
Results
A total of 40 patients were enrolled in the study (M:F = 80:20). Half the patients (n=20) had symptoms pertaining to bone and joint disease. The most common symptom was leg pain (42.50%), followed by backache (22.50%), generalized weakness (20%) and joint pains (20%). Asymptomatic patients had greater mean serum vitamin D as compared to the symptomatic patients (22.2 vs 15.08 ng/ml)(p= 0.225). Five patients were detected to have osteopenia, all above 10 years of age. The mean BMD in symptomatic patients at femur neck, trochanter and ward's angle was 0.781, 0.639 and 0.735 g/cm2 respectively as compared to 0.754, 0.635 and 0.722 g/cm2 in asymptomatic patients. The difference was not statistically significant. Patients with low BMD had lower mean pretransfusion Hb (< 9 g/dl) (p= 0.01).Eighty percent of osteopenic patients had sedentary lifestyle as compared to 44% of patients with normal BMD. Bone mineral density did not have any correlation with vitamin D levels, serum ferritin and type of chelation therapy.
Conclusion
Pre transfusion hemoglobin of more than 9 g/dL has a protective role in maintaining good bone health and bone mineral density and helps to prevent osteopenia. Engaging thalassemia patients in physical activity has a positive effect on bone mineral density. Low vitamin D levels contribute to symptoms of bone and joint involvement in thalassemia and the same needs to be determined and supplemented.
Session topic: E-poster
Keyword(s): Osteoporosis, Thalassemia
Type: Eposter Presentation
Background
Thalassemia major is an inherited hemoglobinopathy causing imbalance in globin chain synthesis, ineffective erythropoiesis and increased peripheral haemolysis. Thalassemia patients have a variety of bone and joint disorders like bone pain, deformity, growth failure, rickets, scoliosis, spinal deformity, nerve compression, pathological fractures, osteopenia and osteoporosis. There is a paucity of data about the extent of bone and joint disorders in thalassemia in developing countries.
Aims
1. To determine bone and joint involvement in thalassemia major.2. To evaluate serum biochemical parameters of bone formation and resorption and their relation with bone and joint formation.3. To evaluate DEXA scan scores of Thalassemia patients and find a correlation, if any, with daily physical activity.
Methods
The study was carried out on 40 thalassemia patients between 5-18 years of age under regular follow up at our centre. All patients were interviewed as per a well-structured proforma for symptoms relating to bone and joint disease as described by the patient/parents in past one year and physical activity by QAPACE questionnaire. Venous blood samples were drawn under aseptic conditions and used for estimation of biochemical parameters (calcium, phosphorus, alkaline phosphatase, vitamin D, parathyroid hormone, thyroid hormone and serum ferritin). Enrolled subjects were scanned for bone mineral density (BMD) at femur neck, trochanter and Ward's angle using dual energy X ray absorptiometry.
Results
A total of 40 patients were enrolled in the study (M:F = 80:20). Half the patients (n=20) had symptoms pertaining to bone and joint disease. The most common symptom was leg pain (42.50%), followed by backache (22.50%), generalized weakness (20%) and joint pains (20%). Asymptomatic patients had greater mean serum vitamin D as compared to the symptomatic patients (22.2 vs 15.08 ng/ml)(p= 0.225). Five patients were detected to have osteopenia, all above 10 years of age. The mean BMD in symptomatic patients at femur neck, trochanter and ward's angle was 0.781, 0.639 and 0.735 g/cm2 respectively as compared to 0.754, 0.635 and 0.722 g/cm2 in asymptomatic patients. The difference was not statistically significant. Patients with low BMD had lower mean pretransfusion Hb (< 9 g/dl) (p= 0.01).Eighty percent of osteopenic patients had sedentary lifestyle as compared to 44% of patients with normal BMD. Bone mineral density did not have any correlation with vitamin D levels, serum ferritin and type of chelation therapy.
Conclusion
Pre transfusion hemoglobin of more than 9 g/dL has a protective role in maintaining good bone health and bone mineral density and helps to prevent osteopenia. Engaging thalassemia patients in physical activity has a positive effect on bone mineral density. Low vitamin D levels contribute to symptoms of bone and joint involvement in thalassemia and the same needs to be determined and supplemented.
Session topic: E-poster
Keyword(s): Osteoporosis, Thalassemia
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