Discrepancy in patterns of myocardial involvement in beta-thalassaemia vs. sickle cell anaemia.

Introduction: Different mechanisms contribute to myocardial dysfunction in sickle cell disease [SCD] and beta thalassaemia major [TM]. TM mainly involves the highly vascular subepicardium by iron load and SCD mainly operates by inducing ischaemia in the relatively ischaemic subendocardium. The aim of this article was to determine if pattern of left ventricular [LV] dysfunction differ among the two groups of patients.Methods: Forty TM and 40 SCD patients and 40 age- and surface area-matched controls were subjected to conventional echocardiography, 2D Speckle tracking myocardial layer strain discriminating echocardiography (MLSD-STE) which is able to discriminate if myocardial dysfunction is predominantly subepicardial or subendocardial and 3D echocardiography for ejection fraction assessment as well as haemoglobin, ferritin, and lactate dehydrogenase levels.Results: TM patients had a deeper subepicardial dysfunction while SCD had prevalent subendocardial dysfunction, epicardial GLS (TM: -10.9 ± 2 vs. SCD: 19.9 ± 1.7; p value < 0.01); endocardial GLS (TM: -19.9 ± 1.7 vs. SCD: -10.6 ± 1.6, p value < 0.01).Conclusion: This study points towards divergent microcirculatory mechanisms in the pathogenesis of myocardial dysfunction in haemoglobinopathies. It shows predominant subendocardial dysfunction with underlying ischaemia of SCD and prevalent subepicardial iron-induced injury in cases of TM.

Review of 16 cases of aneurysmal bone cyst in the proximal femur treated by extended curettage and cryosurgery with reconstruction using autogenous nonvascularized fibula graft.

BACKGROUND: Aneurysmal bone cyst (ABC) is an aggressive benign lesion that may sometimes be difficult to treat. Lesions that occur in the proximal femur require to be addressed aggressively because of the high rate of local recurrence and the risk of fracture. Few articles report the experience of management of ABC in the proximal femur. This location presents a surgical challenge due to its anatomical and biomechanical peculiarity. METHODS: We retrospectively reviewed 16 cases with ABC in the proximal femur that were treated in our institution between 2005 and 2014, utilizing extended curettage and liquid nitrogen as adjuvant therapy and reconstruction using nonvascularized autogenous fibular strut graft. The mean follow-up period was 50.5 months. Five cases presented as recurrent cases, while four cases presented with pathological fractures. RESULTS: The functional score ranged from 70 to 90 on modified Musculoskeletal Tumor Society score with a mean score of 81.25, which is considered to be excellent function. Time for graft incorporation ranged from 10 to 12 weeks. One case got local recurrence, and one case got early surgical site infection. CONCLUSIONS: Extended curettage and cryotherapy represent a recommended approach for management of ABC in the proximal femur with favorable results.