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Journal ArticleDOI

Magnetic resonance imaging in teenagers and young adults with limited haemophilic arthropathy: baseline results from a prospective study.

TLDR
Haemosiderin seemed associated with the time between assessment and last bleed; joints that had suffered a bleed long before MRI had hardly haemOSiderin, while those with a recent bleed showed haemosIDERin, suggesting joint damage may be reversible.
Abstract
The clinical relevance of subtle changes on magnetic resonance imaging (MRI) for evaluating haemophilia treatment is unknown. To determine the relationship of findings on MRI with joint function and bleeding in joints with apparently very mild arthropathy, a prospective study was performed. Knees and ankles of 26 patients, 13-26 years, were scanned. Two blinded radiologists scored the MRI (IPSG consensus score) and the radiography [Pettersson score (PS)]. Clinical function (HJHS) was scored by one physiotherapist. Life-time number of bleeds was collected from patient files. Of 104 joints scanned, three were excluded because of previous arthrodesis or trauma. Remaining 101 MRI scores correlated weakly with clinical function (r = 0.27, P = 0.01) and less with lifetime number of bleeds (r = 0.16, P = 0.14). MRI scores were 0 in 58 joints, including 27 with major bleeds. In three joints of patients playing intensive sports MRI showed minor changes (MRI score = 1) in the absence of bleeds. Agreement was reasonable between PS and MRI score (r = 0.41, P < 0.01). In 30% of joints, MRI detected abnormalities in soft-tissue and cartilage, while PS was 0 points. No evidence of occult haemorrhages was found. Instead, we found no abnormalities on MRI in 43 joints with a history of repeated joint bleeding. Haemosiderin seemed associated with the time between assessment and last bleed; joints that had suffered a bleed long before MRI had hardly haemosiderin, while those with a recent bleed showed haemosiderin, suggesting joint damage may be reversible. Abnormalities detected by MRI, but not by PS were minor and their clinical implications are not yet clear.

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Citations
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Journal ArticleDOI

Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens.

TL;DR: Initial data support that adult patients with already existing advanced joint arthropathy benefit from tertiary prophylaxis with significantly lowered number of bleeds, almost complete absence of target joints, and less time off from work.
Journal ArticleDOI

Using the Haemophilia Joint Health Score for assessment of teenagers and young adults: exploring reliability and validity.

TL;DR: In this paper, the authors assess reliability and explore validity of the Haemophilia Joint Health Score (HJHS) in teenagers and young adults with hahemophilia.
Journal ArticleDOI

Controlled, cross-sectional MRI evaluation of joint status in severe haemophilia A patients treated with prophylaxis vs. on demand

TL;DR: MRI scores identified pathologic joint status with high sensitivity and primary prophylaxis demonstrated protective effects against joint deterioration compared with secondary proPHylaxis, which had lower annualized joint bleeds and MRI scores vs. the on‐demand group.
References
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Journal ArticleDOI

The efficacy of diagnostic imaging.

TL;DR: The authors discuss the assessment of the contribution of diagnostic imaging to the patient management process and presents a hierarchical model of efficacy as an organizing structure for appraisal of the literature on efficacy of imaging.
Journal ArticleDOI

A radiologic classification of hemophilic arthropathy.

TL;DR: A new, exclusively radiologic classification of hemophilic arthropathy is based on a review of 54 patients who had had repeated radiologic examinations of all great joints before the institution of any specific treatment.
Journal ArticleDOI

Hemophilia joint health score reliability study

TL;DR: This study is the first in a series to assess the psychometric properties of the HJHS, a promising new measure of joint health in boys with haemophilia.
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