Autoantibodies to eight of the aminoacyl-transfer RNA synthetases-the most well-recognized of which is anti-histidyl (Jo-1)-have all been implicated in the pathogenesis of antisynthetase syndrome (AS). AS is characterized by varying degrees of interstitial lung disease, myositis, arthropathy, fever, Raynaud's phenomenon, and mechanic's hands, and the morbidity and mortality of the disease are usually linked to the pulmonary findings. The value of a lung biopsy in AS cannot be overemphasized, as it serves to describe the underlying etiology of the interstitial lung disease, guide therapy, and estimate prognosis. Muscle disease shares many clinical features of polymyositis, yet histologically, the inflammatory involvement resembles that of dermatomyositis. Because inflammatory arthritis mimics rheumatoid arthritis, AS should be considered in atypical cases. Corticosteroids are the mainstay of acute therapy, although treatment often requires immunosuppressant medications such as cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, or rituximab.
We performed a genomewide search for linkage in an extended Dutch family with hereditary vascular retinopathy associated with migraine and Raynaud phenomenon. Patients with vascular retinopathy are characterized by microangiopathy of the retina, accompanied by microaneurysms and telangiectatic capillaries. The genome search, using a high throughput capillary sequencer, revealed significant evidence of linkage to chromosome 3p21.1-p21.3 (maximum pairwise LOD score 5.25, with D3S1578). Testing of two additional families that had a similar phenotype, cerebroretinal vasculopathy, and hereditary endotheliopathy with retinopathy, nephropathy, and stroke, revealed linkage to the same chromosomal region (combined maximum LOD score 6.30, with D3S1588). Haplotype analysis of all three families defined a 3-cM candidate region between D3S1578 and D3S3564. Our study shows that three autosomal dominant vasculopathy syndromes with prominent cerebroretinal manifestations map to the same 3-cM interval on 3p21, suggesting a common locus.
Introduction
Published curricula to teach communication skills for postgraduate fellows in oncology are few in number despite the fact that oncologists conduct many difficult discussions with their patients and their families. Such discussions may include disclosing initial diagnosis or relapse of a patient's cancer or relaying a poor prognosis or change to palliative care.
Methods
An eight-module course on communication in oncology practice was delivered over 2 months for palliative and oncology fellows and radiation oncology residents. Learners were given a precourse survey in which they were asked to rate their proficiency in various communication tasks. Each learner then participated in a videotaped precourse objective structured clinical exam (OSCE) on breaking bad news with standardized patients (SPs). The course took place over 8 weeks with weekly didactics and role-play. At the end of the course, a second OSCE took place. After the course was completed, the fellows again filled out a proficiency survey.
Results
Twenty-two learners participated over 2 years of this course. Participants reported a significant increase in perceived competence in all areas on the postcourse survey. SP feedback on OSCEs pre- and postcourse indicated improvement in skills for learners. Pre- and postcourse OSCE video assessment revealed a significant improvement in global communication skills.
Discussion
Initial data show that this course successfully improved communication skills and increased fellows' comfort level across several domains of communication. Future directions include validating our assessment tool, expanding the topic base, and investigating the impact on practice after course completion.
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