Keywords:
Osteoporosis, Metabolic disorders, Screening, Diagnostic procedure, CT, Musculoskeletal system, Musculoskeletal joint, Bones
Authors:
A. Balanika, P. XAFAKI, C. Baltas, H. Pergantou, H. PLATOKOUKI, N. L. Kelekis, O. Papakonstantinou; Athens/GR
DOI:
10.1594/essr2018/P-0060
Purpose
Hemophilia A is a rare X -linked inherited bleeding disorder,
almost exclusively in males,
due to a deficiency of clotting factor VIII.
Hemophilic children have a great risk of bleeding spotaneously or after minor trauma.Because of hemarthrosis and hypokinisia,
hemophilic children are prone to reduced bone mass accrual.
Dual-energy X-Ray absorptiometry ( DXA) is the most widely used method for the assessment of bone mineral content (BMC) in pediatric studies because of the assessment of large volumes of bone with short time scans and low radiation dose.The performance of posteroanterior lumbar spine (LS) and whole-body (TBLH) DXA is recommended by the International Society for Clinical Densitometry (ISCD) in children with chronic diseases that may affect bone health. But DXA is 2-dimensional technique,
therefore it influences by body size and it provides an incomplete assessment of compartmental bone mineral distribution and bone geometry.
Peripheral quantitative computed tomography (pQCT) is the gold standard technique for the assessment of bone geometry ,
density and strength.
The aim of this study is to evaluate bone status in children with haemophilia A by whole body and spinal DXA and to correlate its findings and clinical data with pQCT parameters in the same patients.