beta - Thalassemia Intermedia with Severe Bone Abnormality
Journal: International Journal of Science and Research (IJSR) (Vol.10, No. 9)Publication Date: 2021-09-05
Authors : I Ketut Suyasa; I Gusti Ngurah Wien Aryana; Hendra Aryudi Hamzah;
Page : 1183-1186
Keywords : Case report; Pathologic fracture; Bone abnormalities; beta - Thalassemia;
Abstract
Introduction: beta - thalassemia (betath) is a congenital hematologic disorder that affects the production of beta - globin chains, resulting in impaired haemoglobin function. betath is classified into beta - Thalassemia minor, beta - Thalassemia intermedia (betati), and beta - Thalassemia major. Bone abnormalities are common including osteoporosis and fracture in betati patients. This case study aims to highlightthe features of bone abnormalities in beta - thalassemia intermedia patients. Case Description: A 25 - year - old woman with beta - Thalassemia intermedia (betati) presented to our clinic with pain on her right knee and inability to bear her weight, after stumbling and falling during her way to the bathroom. Patient had previous history of trauma affecting her left leg. On radiographic examination, we found a right distal femoral fracture and a left subtrochanteric fracture. The patient was treated by packed red cell transfusion, internal fixation, and bisphosphonate. After two months of evaluation, the fractures had started to unite and the patient had improved her range of motion of her both leg. Bone abnormalities are common in betati patients including pathologic fracture in long bones. These patients should be treated comprehensively in order to improve bone density andlower the risk of fractures.
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