Peripheral quantitative computed tomography (pQCT) reveals alterations in the three-dimensional bone structure in children with haemophilia.
Children with haemophilia are at risk of suboptimal bone mass accrual and low bone mineral density (BMD). We recently demonstrated that although BMD in Finnish children with haemophilia was within the normal range, their whole body BMD was significantly lower and hypercalciuria more prevalent than in controls. This study sought to determine the bone structure and strength in physically active children with haemophilia. To investigate the underlying mechanisms in this group, we conducted a case-control study to assess bone structure and strength by peripheral quantitative computed tomography (pQCT) at the radius. The study group comprised 29 patients (mean age 12.2 years) and 46 age-matched controls. Children with haemophilia had decreased total BMD Z-score at the distal radius (P ≤ 0.001), but increased cortical bone density at the proximal radius (P ≤ 0.001). Total bone area at the proximal radius was significantly lower in children with haemophilia (P = 0.002), whereas there were no differences in cortical bone area or in polar Strength-Strain Index, a parameter of bone strength, between the patients and controls. Patients with mild to moderate haemophilia and on-demand treatment had inferior bone strength compared to those with moderate to severe haemophilia and prophylaxis. Our findings suggest altered skeletal development in patients with haemophilia in the radius, resulting in smaller bone size and higher cortical bone density. Importantly, bone strength at the radius appears equal to healthy children. Prophylactic treatment seems to have a beneficial effect on bone health.
Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland. susanna.ranta@ki.se
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Ranta
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Viljakainen
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Mäkipernaa
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Mäkitie
D000293:Adolescent; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D015519:Bone Density / Q000502:physiology; D001842:Bone and Bones / Q000033:anatomy & histology / Q000502:physiology*; D016022:Case-Control Studies; D002648:Child; D005260:Female; D006467:Hemophilia A / Q000000981:diagnostic imaging* / Q000188:drug therapy; D002836:Hemophilia B / Q000000981:diagnostic imaging* / Q000188:drug therapy; D006801:Humans; D008297:Male; D011884:Radius / Q000502:physiology; D014057:Tomography, X-Ray Computed; D055815:Young Adult
D001779:Blood Coagulation Factors