Keywords:
Musculoskeletal system, Extremities, Paediatric, CT-Quantitative, Comparative studies, Screening, Osteoporosis, Metabolic disorders, Haematologic diseases
Authors:
A. Balanika, P. XAFAKI, C. Baltas, H. Pergantou, H. PLATOKOUKI, N. L. Kelekis, O. Papakonstantinou; Athens/GR
DOI:
10.1594/ecr2018/C-1605
Aims and objectives
Hemophilia is an X-linked congenital bleeding disorder caused by deficiency of factor VIII (FVIII) in hemophilia A or factor IX (FIX) in hemophilia B.
Hemophilic patients suffer from recurrent spontaneous bleeding mainly of the large synovial joints leading to hemophilic arthropathy.The severity of bleeding is inversely correlated with clotting factor level and the use of prophylactic clotting factor diminishes the risk of haemarthrosis.
In children and older hemophilic patients is found low bone mineral density BMD with the majority of studies have used dual X-ray absorptiometry to measure BMD in haemophilic males.
Since dual X-ray absorptiometry provides no information on the bone microarchitecture and strength the aim of this study is to investigate location-specific bone material properties in hemophilic children and their correlation with clinical data using peripheral quantitative computed tomography (pQCT).