Michelson J, Cohen A
Department of Pediatric Orthopaedic Surgery, Children's Hospital of Philadelphia, Pennsylvania.
J Orthop Trauma. 1988;2(1):29-32. doi: 10.1097/00005131-198802000-00007.
Thalassemia major, a disorder of hemoglobin synthesis, contributes to skeletal deformities, marked osteopenia, and frequent fractures. Hypertransfusion therapy in the last 20 years has prolonged the life expectancy of thalassemics and decreased the orthopaedic manifestations of the disease. This study examines the incidence and treatment of fractures in hypertransfused thalassemics in whom the hemoglobin is kept above 8 g/dl. Over a 5-year period, there were eight fractures in 38 patients, which yields an incidence that is approximately 70% above the fracture rate in the general population. With the exception of one patient, all of the fractures healed in the expected time utilizing nonoperative treatment. The results of this study indicate that, although thalassemics are at moderately high risk for sustaining fractures, they respond well to standard casting methods. However, the iron overload found in thalassemia can deplete the body of vitamin C, which can subsequently retard fracture healing. Attention must, therefore, be paid to replenishing vitamin C very cautiously in order to avoid this complication.
