Summary. The development of arthropathy is a serious complication of severe haemophilia. With the use of prophylaxis, bleeds can be prevented and arthropathy delayed. We investigated whether an individually tailored prophylactic regimen can prevent arthropathy and whether it had a similar effect on orthopaedic outcome compared with that of a high-dose regimen. Efficacy was determined clinically and by radiographs of six major joints. Prophylaxis was started in 70 patients at a mean age of 4´1 years. Mean follow-up was 15´6 years (range 8±24´5 years). The mean factor VIII consumption was 2319 IU/kg/year. The mean number of joint bleeds was 3´5/year and the mean clinical score (maximum score 90) was 1´0, with a mean Pettersson joint score (maximum score 78) of 3´0 at a mean age of 13´5 years. In conclusion, long-term, early-onset, individualized prophylaxis in haemophilia is feasible and prevents arthropathy.
Computed tomographic angiography and MRA are not reproducible or sensitive enough to rule out renal artery stenosis in hypertensive patients. Therefore, DSA remains the diagnostic method of choice. *For a list of the other investigators and research coordinators who participated in RADISH, see the Appendix.
Our results suggest that initial staging of malignant lymphoma using whole-body MRI (without DWI and with DWI) equals staging using CT in the majority of patients, whereas whole-body MRI never understaged relative to CT. Furthermore, whole-body MRI mostly correctly overstaged relative to CT, with a possible advantage of using DWI.
• Accurate staging is important for treatment planning and assessing prognosis • Whole-body MRI-DWI could be a good radiation-free alternative to FDG-PET/CT • Interobserver agreement of whole-body MRI-DWI is good • Agreement between whole-body MRI and the FDG-PET/CT reference standard is good • Most discrepancies were caused by suboptimal accuracy of size measurements on MRI.
Our early results indicate that overall interobserver agreement on whole-body MRI-DWI findings is moderate to good. Overall agreement between whole-body MRI-DWI and FDG PET/CT is moderate. In the care of patients with newly diagnosed lymphoma, staging with whole-body MRI-DWI does not result in underestimation of stage relative to the results with FDG PET/CT. In a minority of patients, reliance on whole-body MRI-DWI leads to clinically important overstaging relative to the results with FDG PET/CT. FDG PET/CT remains the reference standard for lymphoma staging until larger-scale studies show that use of whole-body MRI-DWI results in correct staging in this minority of cases.
Introduction
Recurrent joint bleeding is the hallmark of haemophilia. Synovial hypertrophy observed with Magnetic Resonance Imaging (MRI) is associated with an increased risk of future joint bleeding.
Aim
The aim of this study was to investigate whether point‐of‐care ultrasound (POC‐US) is an accurate alternative for MRI for the detection of early joint changes.
Methods
In this single centre diagnostic accuracy study, bilateral knees and ankles of haemophilia patients with no or minimal arthropathy on X‐rays were scanned using POC‐US and 3 Tesla MRI. POC‐US was performed by 1 medical doctor, blinded for MRI, according to the “Haemophilia Early Arthropathy Detection with Ultrasound” (HEAD‐US) protocol. MRIs were independently scored by 2 radiologists, blinded for clinical data and ultrasound results. Diagnostic accuracy parameters were calculated with 95% confidence intervals (CI).
Results
Knees and ankles of 24 haemophilia patients (96 joints), aged 18‐34, were studied. Synovial hypertrophy on MRI was observed in 20% of joints. POC‐US for synovial tissue was correct (overall accuracy) in 97% (CI: 91‐99) with a positive predictive value of 94% (CI: 73‐100) and a negative predictive value of 97% (CI: 91‐100). The overall accuracy of POC‐US for cartilage abnormalities was 91% (CI: 83‐96) and for bone surface irregularities 97% (CI: 91‐99).
Conclusion
POC‐US could accurately assess synovial hypertrophy, bone surface irregularities and cartilage abnormalities in haemophilia patients with limited joint disease. As POC‐US is an accurate and available alternative for MRI, it can be used for routine evaluation of early joint changes.
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