WFRF:(Danielsson Olof 1963 )
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| Fältnamn | Indikatorer | Metadata |
|---|---|---|
| 000 | 06599nam a2200469 4500 | |
| 001 | oai:DiVA.org:liu-132768 | |
| 003 | SwePub | |
| 008 | 161123s2016 | |||||||||||000 ||eng| | |
| 020 | a 9789176856413q print | |
| 024 | 7 | a https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-1327682 URI |
| 024 | 7 | a https://doi.org/10.3384/diss.diva-1327682 DOI |
| 040 | a (SwePub)liu | |
| 041 | a engb eng | |
| 042 | 9 SwePub | |
| 072 | 7 | a vet2 swepub-contenttype |
| 072 | 7 | a dok2 swepub-publicationtype |
| 100 | 1 | a Danielsson, Olof,d 1963-u Linköpings universitet,Avdelningen för neuro- och inflammationsvetenskap,Medicinska fakulteten4 aut0 (Swepub:liu)oloda25 |
| 245 | 1 0 | a The Clinical and Pathological Spectrum of Idiopathic Inflammatory Myopathies :b Implications for pathogenesis, classification and diagnosis |
| 264 | 1 | a Linköping :b Linköping University Electronic Press,c 2016 |
| 300 | a 131 s. | |
| 338 | a electronic2 rdacarrier | |
| 490 | 0 | a Linköping University Medical Dissertations,x 0345-0082 ;v 1551 |
| 520 | a Background: Idiopathic inflammatory myopathies (IIM) constitute a heterogeneous group of diseases with severe consequences for the life of affected patients. Dermatomyositis, polymyositis and inclusion body myositis (IBM) are the classical representatives of this group. The treatments given today often have limited effects, and are taken at the cost of side effects. Major obstacles in the search for more effective treatments are; (1) an incomplete understanding of the disease mechanisms, (2) difficulties to delineate homogeneous disease groups for clinical studies and (3) the sometimes challenging task to diagnose these diseases.Aims: We addressed a number of “loose ends” in the areas of pathogenesis, classification and diagnosis; mechanisms of muscle fiber degeneration in IIM, with a focus of programmed cell death (apoptosis) and invasion of muscle fibers by inflammatory cells (partial invasion); protecting and mediating factors present in muscle; the association of other diseases with IIM, in particular celiac disease ; the evaluation of two classification systems and laboratory methods for increased diagnostic performance.The studies: We included 106 patients, diagnosed at the Neuromuscular unit in Linköping, Sweden, with pathological muscle findings consistent with IIM. The incidence in the county of Östergötland (during 5 years) was 7.3 per million/year (3 patients each year). Of 88 patients with confirmed IIM 4 (4.5 %) had celiac disease, 33 (38%) had an associated systemic inflammatory disease and 5 (5.7 %) had a malignancy. Ninety-nine patients were included for a comparison of two classification systems using criteria of the European Neuromuscle Centre (Amato/ENMC), and the widely used Bohan and Peter classification, both with the addition of IBM according to Griggs et al. Using the Amato/ENMC criteria the most prevalent diagnostic group after IBM (30%) was nonspecific myositis (23%), followed by polymyositis (20%) and dermatomyositis 17%). A substantial number of patients meeting Bohan and Peter (or Griggs) criteria were excluded by Amato/ENMC criteria, most (21/23) due to lack of detectable muscle weakness. Extended muscle sectioning increased the sensitivity of a muscle biopsy by 15 % and the specificity by 22%, and showed an overlap between disease groups. Muscle biopsies from patients with IIM and controls were used to investigate pathological findings considered specific for disease groups, and for the presence of programmed cell death (apoptosis) and disease protecting and mediating factors in muscle. The presence of apoptotic muscle fiber nuclei was detected in muscle with partial invasion (however not in the invaded fibers) in the presence of granzyme B and CD8+ cytotoxic T cells. The major apoptosis inhibiting protein Bcl-2 was shown to be constitutionally expressed in healthy muscle but weakened in IIM.Conclusion: We present apoptosis as a possible disease mechanism in parallel with partial invasion of fibers. Furthermore, partial invasion may not be a suitable distinguishing feature in the pathogenesis, or for classification and diagnosis of IIM. We also introduce the anti-apoptotic Bcl-2 as a possible relevant muscle fiber protecting factor. A more extensive pathological work-up improves classification and diagnosis of IIM. The proposed Amato/ENMC creates a substantial portion of patients with non-specific or unclassified myositis. Associated diseases are common in IIM, and also include celiac disease. | |
| 650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Klinisk laboratoriemedicin0 (SwePub)302232 hsv//swe |
| 650 | 7 | a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Clinical Laboratory Medicine0 (SwePub)302232 hsv//eng |
| 650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Neurologi0 (SwePub)302072 hsv//swe |
| 650 | 7 | a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Neurology0 (SwePub)302072 hsv//eng |
| 650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Reumatologi och inflammation0 (SwePub)302102 hsv//swe |
| 650 | 7 | a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Rheumatology and Autoimmunity0 (SwePub)302102 hsv//eng |
| 650 | 7 | a NATURVETENSKAPx Biologix Immunologi0 (SwePub)106052 hsv//swe |
| 650 | 7 | a NATURAL SCIENCESx Biological Sciencesx Immunology0 (SwePub)106052 hsv//eng |
| 700 | 1 | a Ernerudh, Jan,c Professoru Linköpings universitet,Avdelningen för neuro- och inflammationsvetenskap,Medicinska fakulteten,Region Östergötland, Klinisk immunologi och transfusionsmedicin4 ths0 (Swepub:liu)janer15 |
| 700 | 1 | a Vrethem, Magnus,c Professoru Linköpings universitet,Avdelningen för neuro- och inflammationsvetenskap,Medicinska fakulteten,Region Östergötland, Neurologiska kliniken i Linköping4 ths0 (Swepub:liu)magvr07 |
| 700 | 1 | a Solders, Göranu Department of Clinical Neuroscience, Karolinska Institutet, Stockholm4 opn |
| 710 | 2 | a Linköpings universitetb Avdelningen för neuro- och inflammationsvetenskap4 org |
| 856 | 4 | u https://doi.org/10.3384/diss.diva-132768y Fulltext |
| 856 | 4 | u https://liu.diva-portal.org/smash/get/diva2:1049178/FULLTEXT01.pdfx primaryx Raw objecty fulltext |
| 856 | 4 | u https://liu.diva-portal.org/smash/get/diva2:1049178/COVER01.pdfy cover |
| 856 | 4 | u https://liu.diva-portal.org/smash/get/diva2:1049178/PREVIEW01.jpgx Previewy preview image |
| 856 | 4 | u http://liu.diva-portal.org/smash/get/diva2:1049178/FULLTEXT01 |
| 856 | 4 8 | u https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-132768 |
| 856 | 4 8 | u https://doi.org/10.3384/diss.diva-132768 |
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