الفهرس 
Sickle Cell Disease: Pathogenesis and ComplicationsOnly 14 pages are availabe for public view
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Abstract
Background: Bone involvement is the commonest clinical manifestation of sickle cell disease both in the acute setting such as painful vaso-occlusive crises, and as a source of chronic, progressive disability such as avascular necrosis. Osteonecrosis of the femoral head in sickle cell disease has an insidious onset, and in the majority of patients progresses to femoral head collapse with rapid destruction of the hip joint. Early screening for femoral head AVN and identification of risk factors would help in placement of appropriate measures to prevent osteonecrosis in high risk patients.
Objective: To screen for femoral head osteonecrosis in sickle cell disease patients using conventional radiography and MRI for hip joints. To study the association between MR findings with disease status and hematologic parameters.
Patients and Methods: This cross sectional study was carried out on 30 patients with sickle cell disease (HbSS disease, HbS\beta0 thalassemia, HbS\beta+ thalassemia) in their steady state who attend the Hematology clinic, Pediatrics Hospital, Ain-Shams University. The osteonecrosis was defined using the Ficat and Arlet classification system for femoral head osteonecrosis and Mitchell classification system for femoral shaft osteonecrosis. Using these classification systems the participants were divided into two groups: the “AVN positive” consisting of patients with AVN, and the “AVN negative” without AVN. Both groups were evaluated in search for factors associated with osteonecrosis of the femoral head.
Results: All study cases were subjected to revision of their hospital records for clinical and laboratory details related to their disease and radiological examination was conducted including plain x ray and MRI for hip joints, upper femurs and lumbosacral spine.
Results showed prevalence of osteonecrosis affecting vertebrae, femoral head and shaft is 43.30%, with 60% of them had femoral head osteonecrosis and 3 of them with bilateral disease. Femoral shaft osteonecrosis was a significant finding with 40% prevalence.
Conclusion: No association between osteonecrosis and gender, disease status, MCV, high hematocrit or hemoglobin was observed. However, a significant direct relationship was found between osteonecrosis and higher mean of age.