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...
Methods and Materials
Thirty one boys with haemophilia A (severe 24,
mean age 11.3 ±3.7) who are followed at paediatic Haemophilia Comprehensive Care Centre (HCCC) in Aghia Sophia Children’s Hospital,
Greece were evaluated with DXA and pQCT. An informed consent was obtained from all study participants and/or their parents.
Height and weight was measured by standard methods.
Tanner stage was determined forenitalia and/or pubic hair by inspection. Whole body less head (TBLH ,ROI of total body after...
All parameters matched for age,
height and weight.
Spinal DXA BMC and BMD were strongly and positively correlated with pQCT SSI at both radii and right tibia and weakly associated with pQCT cortical density at both radii and tibiae.
Whole body DXA BMC was strongly correlated with strength at the left tibia and moderately at the right and weakly associated with pQCT total density of both hands.
Both spinal and wholly body DXA BMC and BMD and height were strongly correlated with pQCT cortical...
Childhood and adolescence are critical periods for skeletal development and body mass gain and are characterize by increase of trabecular and cortical bone density and cortical bone dimensions.
Physical activity stimulates bone remodelling and can increase bone mass and strength in pre- to early pubertal.
Recent studies showed positive association between exercise during growing years and bone apposition and strength. Hemarthrosis and reduced physical activity can alter the normal skeletal...
Bone properties and muscle strength of young haemophilia patients.
Haemophilia 2005;11(4):380–6 2.
] White GC,
Definitions in hemophilia.
Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis.
Thromb Haemost 2001;85(3):560 3....
Alexia Balanika ,Consultant Radiologist ,Computed Tomography Department ,General Hospital of Athens "Asclepeion Voulas",Greece Panagiota Xafaki,
Pediatrist and Haematologist,Haemophilia Centre/Haemostasis Unit,
Aghia Sophia Children's Hospital,
Greece Christos Baltas Consultant Radiologis,Radiology Imaging Department ,General Hospital of Athens " G.Gennimmatas",Greece Helen Pergantou ,Consultant Consultant Pediatrist and Haematologist,
Chlidren's Hospitalof Ahtens "Agia Sofia" Helen...