Mastocytosis is a heterogeneous group of diseases defined by an increased number and accumulation of mast cells, and often also by signs and symptoms of mast cell activation. Disease subtypes range from indolent to rare aggressive forms. Mastocytosis affects people of all ages and has been considered rare; however, it is probably underdiagnosed with potential severe implications. Diagnosis can be challenging and symptoms may be complex and involve multiple organ-systems. In general it is advised that patients should be referred to centres with experience in the disease offering an individualized, multidisciplinary approach. We present here consensus recommendations from a Nordic expert group for the diagnosis and general management of patients with mastocytosis.
Objectives to examine whether MrI assessed inflammation and damage in the wrist of patients with early rheumatoid arthritis (rA) are associated with patient-reported outcomes (pros). Methods Wrist and hand MrIs of 210 patients with early rA from two investigator-initiated, randomised controlled studies (CIMEStrA/opErA) were assessed according to the outcome Measures in rheumatology rA MrI score (rAMrIS) for synovitis, tenosynovitis, osteitis, bone erosions and joint space narrowing (JSn) at baseline, 1 and 5 years follow-up. these features, and changes therein, were assessed for associations with health assessment questionnaires (HAQ), patient global visual analogue scales (VAS-ptGlobal) and VAS-pain using Spearman's correlations, generalised estimating equations and univariate/multivariable linear regression analyses. MrI features were further tested for trends against specific hand-related HAQ items using Jonckheere trend tests. results MrI inflammation, but not damage, showed statistically significant associations with HAQ, VASptGlobal and VAS-pain for status and change scores, independently of C reactive protein and swollen joint count. MrI-assessed synovitis was most consistently associated with pros, particularly VAS-ptGlobal and VAS-pain. MrI-assessed synovitis and tenosynovitis mean scores were positively associated with patientreported difficulty to cut meat and open a milk carton (p<0.01), and similar patterns were seen for other hand-related HAQ items. Incorporating metacarpophalangeal joints in the analyses did not strengthen the associations between MrI pathology and pros. Conclusions MrI-assessed inflammation, but not damage, in early rA wrists is associated with patientreported physical impairment, global assessment of disease activity and pain and influences the physical function in the hand. trial registration number nCt00660647.
The authors studied the relation between residual angulation at the time of healing and final orientation of the distal radius as well as the clinical outcome in patients after Salter-Harris type II epiphyseal plate injury of the distal radius. Eighty-five patients were reviewed with a median follow-up of 8.5 years. Anteroposterior and lateral radiographs were taken at follow-up. The mobility of both wrists and forearms was examined, together with grip strength. Pain with activities and sports was evaluated. At follow-up, 73 patients (86%) were anatomically normal on radiographs; the remaining 12 patients had an incomplete remodeling of the volar and/or radial inclination of the distal radius. Premature closure of the growth plate in the distal radius or ulna did not occur in any of these children. Complete remodeling was seen in children aged up to 10 years in all but one patient. Remodeling after Salter-Harris type II epiphyseal plate injury occurs in all age groups, but the potential is greater in children up to 10 years of age. The incomplete remodeling does not seem to have any substantial long-term negative effect on mobility of the wrist and grip strength.
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