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...

Thirty one hemophilic A boys (severe 24, mean age 11.2 years,7 subjects (22.6%) with FVIII inhibitors) followed at hemopihilic unit of our paediatic hospital with an informed consent from all study participants and/or their parents. Clinical data about joint bleeding were collected from the patients’medical records and height and weight were measured by standard methods. All patients underwent pQCT scan (XCT-2000; Stratec, Pforzheim, Germany, software version 5.50) at both radii (at 4% and 60% site of radius length ) and both tibiae (at 4% and...

Statistical analysis pQCT bone parameters were compared between left and right with the paired t-test, while between children with and without inhibitors and between target and no-target joint with the independent samples t-test. The level of significance was set at the 0.05 level. Thirty one children with haemophilia A (severe 24, moderate 6, mild 1, mean age 11,2 years range: 6-19) were included in the study. An arm target-joint (elbow: 3 left/2 right) was found at 5 children while12 children, at least one leg target-joint...

Haemophilia is a bleeding disorder in which there is impairment of blood clotting or coangulation control.In bleeding disorders there is an increase of haematopoietic growth factors that provokes osteoclasts proliferation, bone absortption and a negative influence in bone mass. Reduced bone mineral density in children with haemophilia is multi-factorial and it predisposes to the presence of osteoporosis later in life .The main causes of low bone mass in haemophilia include limited physical activity ,muscle hematomas and atrophy, joint deformities and arhtropathy. Our result showed a...

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, Athens, 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 Platokouki ,Consultant Pediatrist and Haematologist,Haemophilia Centre/Haemostasis Unit, Aghia Sophia Children's Hospital, Athens, Greece Nikolaos Kelekis, Proffessor of Radiology, 2nd Rdiology Department, University Hospital of Athens "Attikon",Greece Olympia Papakonstantinou, Associated Proffessor of Radiology, 2nd Radiology...

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