Abstract
Background
This study examined the structural outcomes for joints of boys with severe hemophilia A receiving frequency/dose‐escalated primary prophylaxis using magnetic resonance imaging (MRI), and the importance of interval MRI changes.
Methods
Forty‐six subjects (27 with interval studies) were evaluated by radiographs (X‐rays) and mid‐ and end‐of‐study MRIs (using the International Prophylaxis Study Group scale), as part of the Canadian Hemophilia Prophylaxis Study. The primary outcome was the presence of MRI osteochondral findings.
Results
The median (range) time on study at the end‐of‐study MRI examination was 9.6 (4.8–16.0) years, during which 18 of 46 subjects (39%) had osteochondral changes in at least one joint. An interval change in MRI score of at least 1 point was observed in 44% of joints (43 ankles, 21 elbows, 4 knees); at least one joint showed this change in all 27 subjects. Self‐reported interval hemarthrosis was associated with a higher likelihood of interval osteochondral change (odds ratio [OR], 1.49; 95% confidence interval [CI] = 1.08–2.06). Presence of synovial hypertrophy or hemosiderin on interval MRIs was associated with an OR of 4.71 (95% CI, 1.92–11.57) and 5.25 (95% CI, 2.05–13.40) of later osteochondral changes on MRI.
Discussion
MRI changes were seen in 39% of subjects. Interval index joint bleeding was associated with an increased risk of later MRI changes, and earlier soft‐tissue changes were associated with subsequent osteochondral changes.
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Details
; Pullenayegum, Eleanor 3 ; Blanchette, Victor S 4
; Doria, Andrea S 1
; Feldman, Brian M 5
; Carcao, Manuel 4 ; Rivard, Georges E 6
; Israels, Sara J 7
; Chan, Anthony K 8
; Steele, MacGregor 9
; Cloutier, Stephanie 10 ; Klaassen, Robert J 11
; Price, Victoria E 12 ; Sinha, Roona 13 ; Laferriere, Nicole 14 ; Paradis, Elizabeth 15 ; Wu, John K M 16 ; Babyn, Paul 17 1 Department of Medical Imaging, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
2 Child Health Evaluative Sciences, The Hospital for Sick Children Research Institute, Toronto, ON, Canada
3 Child Health Evaluative Sciences, The Hospital for Sick Children Research Institute, Toronto, ON, Canada; Institute of Health Policy, Management & Evaluation, The Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada
4 Child Health Evaluative Sciences, The Hospital for Sick Children Research Institute, Toronto, ON, Canada; Department of Pediatrics, University of Toronto, Toronto, ON, Canada; Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada
5 Child Health Evaluative Sciences, The Hospital for Sick Children Research Institute, Toronto, ON, Canada; Institute of Health Policy, Management & Evaluation, The Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada; Department of Pediatrics, University of Toronto, Toronto, ON, Canada; Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada
6 Division of Hematology/Oncology, Department of Pediatrics, CHU Sainte‐Justine, Montreal, QC, Canada
7 Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, MB, Canada
8 Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, ON, Canada
9 Section of Pediatric Hematology, Department of Pediatrics, University of Calgary and Alberta Children's Hospital, Calgary, AB, Canada
10 Centre de l’hémophilie de l'est du Québec, Hôpital de l'Enfant‐Jésus, Université Laval, Québec, QC, Canada
11 Division of Hematology Oncology, Children’s Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
12 Division of Pediatric Hematology/Oncology, IWK Health Centre, Halifax, NS, Canada
13 Saskatchewan Bleeding Disorders Program, Royal University Hospital, Saskatoon, SK, Canada
14 Division of Hematology/Oncology, Thunder Bay Regional Cancer Care, Thunder Bay, ON, Canada
15 Health Sciences North, Sudbury, ON, Canada
16 Division of Hematology/Oncology/BMT, Department of Pediatrics, UBC & BC Children’s Hospital, Vancouver, BC, Canada
17 Department of Medical Imaging, University of Saskatchewan, Saskatoon, SK, Canada




