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Sample records for juvenile dermatomyositis dm

  1. Dermatomyositis (DM)

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    ... Favorite Name: Category: Share: Yes No, Keep Private Dermatomyositis Share | Dermatomyositis (DM) is a rare inflammatory muscle disease that ... drugs, and infectious agents) in genetically predisposed individuals. Dermatomyositis can occur with other connective tissue disorders such ...

  2. Dermatomyositis (Juvenile)

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    ... Am A Patient / Caregiver Diseases & Conditions Dermatomyositis (Juvenile) Dermatomyositis (Juvenile) Fast Facts Patients with JDM have varying ... What are common signs and symptoms of juvenile dermatomyositis? The most common signs and symptoms of JDM ...

  3. Juvenile Dermatomyositis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Anthony Emeka Madu

    2013-01-01

    Full Text Available Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature.

  4. Juvenile dermatomyositis in pregnancy.

    Science.gov (United States)

    Madu, Anthony Emeka; Omih, Edwin; Baguley, Elaine; Lindow, Stephen W

    2013-01-01

    Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature.

  5. The Paediatric Rheumatology International Trials Organisation provisional criteria for the evaluation of response to therapy in juvenile dermatomyositis

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Pistorio, Angela; Ravelli, Angelo

    2010-01-01

    To develop a provisional definition for the evaluation of response to therapy in juvenile dermatomyositis (DM) based on the Paediatric Rheumatology International Trials Organisation juvenile DM core set of variables.......To develop a provisional definition for the evaluation of response to therapy in juvenile dermatomyositis (DM) based on the Paediatric Rheumatology International Trials Organisation juvenile DM core set of variables....

  6. The Paediatric Rheumatology International Trials Organisation provisional criteria for the evaluation of response to therapy in juvenile dermatomyositis

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Pistorio, Angela; Ravelli, Angelo

    2010-01-01

    To develop a provisional definition for the evaluation of response to therapy in juvenile dermatomyositis (DM) based on the Paediatric Rheumatology International Trials Organisation juvenile DM core set of variables....

  7. [JUVENILE DERMATOMYOSITIS AND CALCINOSIS].

    Science.gov (United States)

    Zhvania, M

    2015-01-01

    Juvenile Dermatomiositis (JD) is autoimmune disease that progresses with time; JD's main differentiated syndromes are rash on the skin, poor function of muscles, and often developing invalidism. If the health practitioners manage to diagnose the JD on an early stage and prescribe the adequate treatment the disease will not progress aggressively. This approach is tangible for practical rheumatology and pediatric. The article aims to present the reasons of the development of the JD and calcinosis. The study based on the description of the patients with JD. There are distinguished the main symptoms of the disease in children: frequent and acute developments of muscles calcinosis, occasionally with diffuse character followed with hypotrophy of the muscles, contractures and invalidism. One of the patient cases that describe the article is the thirteen-year boy with JD indicating repeated sequence of the disease, with diffusive calcinosis, cellulitis followed with secondary infection and impaired vision.

  8. Juvenile dermatomyositis in a Nigerian girl

    Science.gov (United States)

    Adelowo, Olufemi; Nwankwo, Madu; Olaosebikan, Hakeem

    2014-01-01

    Juvenile dermatomyositis is an autoimmune connective tissue disease occurring in children less than 16 years old. It is part of a heterogeneous group of muscle diseases called idiopathic Iiflammatory myopathies. It had previously been reported in black Africans resident in UK. However, there is no documented case reported from Africa. The index sign of heliotrope rashes is often difficult to visualise in the black skin. An 11-year-old Nigerian girl presenting with clinical, laboratory and histopathological features of juvenile dermatomyositis is presented here. It is hoped that this case will heighten the index of suspicion of this condition among medical practitioners in Africa. PMID:24706700

  9. Muscle pathology in juvenile dermatomyositis

    Directory of Open Access Journals (Sweden)

    Edenilson Eduardo Calore

    Full Text Available OBJECTIVE: To study muscle biopsies, using histochemistry, on ten children with infantile dermatomyositis. DESIGN: Series of ten patients (of whom eight patients had received treatment and two had not were submitted to muscle biopsy in order to diagnose possible inflammatory myopathy or to detect recurrences. PLACE OF DEVELOPMENT OF THE STUDY: Public Health Service of São Paulo State. PARTICIPANTS: children with clinical features of inflammatory myopathy. INTERVENTION: biopsies were performed on the vastus lateralis using local anesthetic. Histochemistry was performed according to standardized methods. RESULTS: Architectural changes of the muscle fibers, necrosis of variable intensity and accentuated evidence of regeneration were observed in patients who had not received treatment (2 cases and in one case where muscular weakness persisted in spite of corticosteroid therapy. Necrosis and regeneration were minimal or absent in cases treated for one year or more (4 cases. In 3 cases with clinical and laboratorial recurrences, muscle necrosis and architectural changes were detected. CONCLUSIONS: It was concluded that muscle biopsy could aid in diagnosing infantile dermatomyositis as well as in detecting recurrences even in cases without clinical activity of the disease.

  10. [Lung is also involved in juvenile dermatomyositis].

    Science.gov (United States)

    Pouessel, G; Thumerelle, C; Nève, V; Santangelo, T; Flammarion, S; Pruvot, I; Tillie-Leblond, I; Deschildre, A

    2014-07-01

    Juvenile dermatomyositis is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Lung involvement in inflammatory myopathies is well described in adults, involving mostly interstitial lung disease, aspiration pneumonia and alveolar hypoventilation. We propose to describe its specificities in children. Pulmonary involvement may be asymptomatic and therefore must be systematically screened for. In case of clinical or functional respiratory abnormality, a chest computed tomographic (CT) scan is necessary. In children, a decrease of respiratory muscle strength seems common and should be systematically and specifically searched for by non-invasive and reproducible tests (sniff test). Interstitial lung disease usually associates restrictive functional defect, impairment of carbon monoxide diffusion and interstitial lung disease on CT scan. As in adults, the first-line treatment of juvenile dermatomyositis is based on corticosteroids. Corticosteroid resistant forms require corticosteroid bolus or adjuvant immunosuppressive drugs (methotrexate or cyclosporine). There is no consensus in pediatrics for the treatment of diffuse interstitial lung disease. Complications of treatment, including prolonged steroid therapy, are frequent and therefore a careful assessment of the treatments risk-benefit ratio is necessary, especially in growing children.

  11. Clinical features and outcome in a Danish cohort of juvenile dermatomyositis patients

    DEFF Research Database (Denmark)

    Mathiesen, P R; Zak, M; Herlin, Troels

    2010-01-01

    To assess disease characteristics and outcome in Danish juvenile dermatomyositis (JDM) patients (1977-2007).......To assess disease characteristics and outcome in Danish juvenile dermatomyositis (JDM) patients (1977-2007)....

  12. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma.

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    McCann, Liza J; Pain, Clare E

    2016-02-01

    Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

  13. Long-term outcome in patients with juvenile dermatomyositis

    DEFF Research Database (Denmark)

    Mathiesen, P; Hegaard, H; Herlin, Troels

    2012-01-01

    To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome.......To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome....

  14. Aerobic training in persons who have recovered from juvenile dermatomyositis

    DEFF Research Database (Denmark)

    Riisager, M; Mathiesen, P R; Vissing, J

    2013-01-01

    A recent study has shown that 36 persons who had recovered from juvenile dermatomyositis (JDM) have on average an 18% decrease in maximal oxygen uptake. The objective of this study was to investigate the effect of a 12-week aerobic training program in this group, and assess whether aerobic training...

  15. Exercise in action for JUVENILE DERMATOMYOSITIS : Steps towards understanding

    NARCIS (Netherlands)

    Habers, G.E.A.

    2015-01-01

    This thesis describes studies in the field of juvenile dermatomyositis (JDM), a chronic inflammatory disease in which the microvasculature is attacked by the immune system. Clinical characteristics include symptoms of muscles (e.g. weakness) and skin (e.g. Gottron’s papules over the extensor joint s

  16. Rapid improvement of calcinosis in juvenile dermatomyositis with alendronate therapy.

    Science.gov (United States)

    Ambler, Geoffrey R; Chaitow, Jeffrey; Rogers, Maureen; McDonald, David W; Ouvrier, Robert A

    2005-09-01

    A 6-year-old boy with improving juvenile dermatomyositis (JDM) developed severe and debilitating calcinosis, unresponsive to diltiazem and probenecid. Alendronate produced dramatic improvement within 1 month and by 12 months calcinosis had virtually resolved. The response was followed by bone mineral content measurements.

  17. Juvenile Dermatomyositis: New Developments in Pathogenesis, Assessment and Treatment

    OpenAIRE

    2009-01-01

    Juvenile dermatomyositis (JDM) is a rare, potentially life-threatening systemic autoimmune disease primarily affecting muscle and skin. Recent advances in the recognition, standardised assessment and treatment of JDM have been greatly facilitated by large collaborative research networks. Through these networks, a number of immunogenetic risk factors have now been defined, as well as a number of potential pathways identified in the aetio-pathogenesis of JDM. Myositis-associated and myositis-sp...

  18. Near-infrared spectroscopy during exercise and recovery in children with juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, G.E.; Knikker, R. de; Brussel, M. Van; Hulzebos, E.; Stegeman, D.F.; Royen, A. Van; Takken, T.

    2013-01-01

    INTRODUCTION: We hypothesized that microvascular disturbances in muscle tissue play a role in the reduced exercise capacity in juvenile dermatomyositis (JDM). METHODS: Children with JDM, children with juvenile idiopathic arthritis (clinical controls), and healthy children performed a maximal increme

  19. Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis.

    LENUS (Irish Health Repository)

    Gunawardena, H

    2009-06-01

    OBJECTIVE: The identification of novel autoantibodies in juvenile dermatomyositis (DM) may have etiologic and clinical implications. The aim of this study was to describe autoantibodies to a 140-kd protein in children recruited to the Juvenile DM National Registry and Repository for UK and Ireland. METHODS: Clinical data and sera were collected from children with juvenile myositis. Sera that recognized a 140-kd protein by immunoprecipitation were identified. The identity of the p140 autoantigen was investigated by immunoprecipitation\\/immunodepletion, using commercial monoclonal antibodies to NXP-2, reference anti-p140, and anti-p155\\/140, the other autoantibody recently described in juvenile DM. DNA samples from 100 Caucasian children with myositis were genotyped for HLA class II haplotype associations and compared with those from 864 randomly selected UK Caucasian control subjects. RESULTS: Sera from 37 (23%) of 162 patients with juvenile myositis were positive for anti-p140 autoantibodies, which were detected exclusively in patients with juvenile DM and not in patients with juvenile DM-overlap syndrome or control subjects. No anti-p140 antibody-positive patients were positive for other recognized autoantibodies. Immunodepletion suggested that the identity of p140 was consistent with NXP-2 (the previously identified MJ autoantigen). In children with anti-p140 antibodies, the association with calcinosis was significant compared with the rest of the cohort (corrected P < 0.005, odds ratio 7.0, 95% confidence interval 3.0-16.1). The clinical features of patients with anti-p140 autoantibodies were different from those of children with anti-p155\\/140 autoantibodies. The presence of HLA-DRB1*08 was a possible risk factor for anti-p140 autoantibody positivity. CONCLUSION: This study has established that anti-p140 autoantibodies represent a major autoantibody subset in juvenile DM. This specificity may identify a further immunogenetic and clinical phenotype within the

  20. Dermatomyositis: Diagnosis

    Science.gov (United States)

    ... Meet our Partners How to Get Involved Donate Dermatomyositis (DM) Share print email share facebook twitter google ... As with other muscle diseases, a doctor diagnoses dermatomyositis (DM) by considering an individual’s history, family medical ...

  1. Juvenile Dermatomyositis Diagnosed by {sup 99m}Tc-HDP Three-phase Bone Scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ja Hye; Song, Ho Chun; Yoo, Su Ung; Ha, Jung Min; Chong, A Ri; Oh, Jong Ryool; Min, Jung Joon; Bom, Hee Seung; Jeong, Eun Hui; Lee, Min Chul [Chonnam National University Hospital, Gwangju (Korea, Republic of)

    2009-12-15

    Juvenile dermatomyositis is a common inflammatory muscle disease of childhood, characterized by weakness in proximal muscles and specific skin rash. In case of juvenile dermatomyositis without characteristic clinical features, non-invasive imaging tools such as {sup 99m}Tc-HDP three-phase bone scan are very helpful in diagnostic workup of myopathies. We report a case of 13-year old female with juvenile dermatomyositis, in which {sup 99m}Tc-HDP three-phase bone scan was useful in diagnosis and assessing therapy response.

  2. Calcinosis in juvenile dermatomyositis: frequency, risk factors and outcome.

    Science.gov (United States)

    Saini, Isha; Kalaivani, Mani; Kabra, Sushil Kumar

    2016-07-01

    The aim was to retrospectively estimate the prevalence of calcinosis in patients with juvenile dermatomyositis (JDM) and to identify risk factors associated with development of calcinosis in these patients. Retrospective chart review of 39 children diagnosed with JDM between 2004 and 2015 in a tertiary care hospital was done. Patients were divided into two groups, depending on the presence or absence of calcinosis, and the two groups were compared with respect to demographic, clinical, laboratory and therapeutic characteristics. Calcinosis developed in nine (23.1 %) patients. Delay in diagnosis and initiation of treatment, prolonged duration of disease, the presence of joint contractures and cardiac involvement were significantly associated with increased frequency of calcinosis. Six out of nine (66.7 %) patients with calcinosis received alendronate therapy, out of which four showed partial reduction in calcinosis. In one case, surgical removal of tumorous clumps was done. Calcinosis remains a common complication of JDM. We found an association between calcinosis and delay in diagnosis and initiation of treatment, prolonged duration of disease and cardiac involvement. Our study suggests that alendronate may be beneficial in management of calcinosis of JDM.

  3. Nodular cystic fat necrosis with calcification in a patient with juvenile dermatomyositis.

    Science.gov (United States)

    Ferenczi, Katalin; Berke, Adrienne; Makkar, Hanspaul S

    2014-01-01

    Nodular cystic fat necrosis is a rare, benign form of encapsulated fat necrosis with distinct histology, characterized by cystic fat necrosis with lipomembranous changes and, in later stages, calcification. We report the case of a 7-year-old child with juvenile dermatomyositis who presented with three asymptomatic, firm, mobile nodules on the arms and neck. Histology was consistent with nodular cystic fat necrosis with prominent calcification. This is an unusual presentation of this entity because it has never been previously reported in association with juvenile dermatomyositis.

  4. Dermatomiosite juvenil e linfoma de Hodgkin: uma rara associação Juvenile dermatomyositis and Hodgkin disease: a rare association

    Directory of Open Access Journals (Sweden)

    André Cavalcanti

    2007-12-01

    Full Text Available A dermatomiosite (DM em adultos está freqüentemente associada com câncer. Já na faixa etária pediátrica, a dermatomiosite juvenil (DMJ é predominantemente idiopática, e sua associação com neoplasia é rara e com base apenas em relatos de casos. Embora rara, a presença de neoplasia em pacientes com DMJ deve ser sempre suspeitada quando houver manifestações clínicas atípicas e alterações laboratoriais incomuns. A seguir, descrevemos e discutimos um caso de DMJ e linfoma de Hodgkin em uma adolescente.Dermatomyositis (DM in adults is frequently associated with cancer. In contrast, during childhood juvenile dermatomyositis (JDM is predominantly idiopathic and its association with neoplasia is rare and based only in case reports. Although rare, the presence of neoplasia in JDM patients must always be suspected in face of atypical clinical manifestations and uncommon laboratorial findings. We describe and discuss a case of JDM and Hodgkin disease in an adolescent.

  5. [Juvenile dermatomyositis and new autoantibodies: Cases and review].

    Science.gov (United States)

    Guarella, M; Jurquet, A-L; Retornaz, K; Bardin, N; Chastang, M-C; Desjonquere, M; Fabien, N; Belot, A

    2015-12-01

    Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children. Its diagnosis is usually made on a clinical basis following the criteria of Bohan and Peter (1975). Recently, the presence of myositis-specific autoantibodies (MSAs) have started to be associated with specific outcome in adult patients; the diagnosis and prognosis value of these autoantibodies remains to be identified in children. We report four cases of JDM with MSAs focusing on clinical, biological, and radiological manifestations, and then we describe associated treatment. The cohort comprises four girls with an average age of 8.5 years. The time to diagnosis was 1 week to 4 months. For these patients, the immunologic study found one patient positive for the MDA5 antibody (or CADM 140), one positive for the TIF1γ antibody (or p155/140), and two patients positive for the NXP2 antibody (or p140/MJ). Each patient showed specific and characteristic cutaneous manifestations. For example, the girl positive for the TIF1γ antibody presented the most severe skin disease with urticaria, face edema, and vascularity of the neck and shoulders. However, regarding muscular features, proximal weakness was present in most of the cohort, except for the child positive for the MDA5 antibody, who presented no sign of muscular disease at the beginning with low CK levels. Importantly, acute pancreatitis also affected this patient. Concerning radiological indications, muscular MRI evidenced hyperinflammation, a sign of diffuse myositis, in all these patients. Treatments consisted in corticosteroids together with methotrexate or mycofenolate mofetil associated or not with intravenous immunoglobulin therapy. This report highlights the importance of systematic detection and analysis of MSA in diagnosis and characterization of JDM, and describes a new approach that would allow more focused treatments and be a useful predictor of clinical complications and prognosis in JDM-affected subjects.

  6. Consensus-based recommendations for the management of juvenile dermatomyositis.

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    Enders, Felicitas Bellutti; Bader-Meunier, Brigitte; Baildam, Eileen; Constantin, Tamas; Dolezalova, Pavla; Feldman, Brian M; Lahdenne, Pekka; Magnusson, Bo; Nistala, Kiran; Ozen, Seza; Pilkington, Clarissa; Ravelli, Angelo; Russo, Ricardo; Uziel, Yosef; van Brussel, Marco; van der Net, Janjaap; Vastert, Sebastiaan; Wedderburn, Lucy R; Wulffraat, Nicolaas; McCann, Liza J; van Royen-Kerkhof, Annet

    2017-02-01

    In 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile dermatomyositis (JDM) is a rare disease within the group of paediatric rheumatic diseases (PRDs) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment regimens differ throughout Europe. To provide recommendations for diagnosis and treatment of JDM. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of 19 experienced paediatric rheumatologists and 2 experts in paediatric exercise physiology and physical therapy, mainly from Europe. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using nominal group technique. Recommendations were accepted if >80% agreement was reached. In total, 7 overarching principles, 33 recommendations on diagnosis and 19 recommendations on therapy were accepted with >80% agreement among experts. Topics covered include assessment of skin, muscle and major organ involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRD. Within this remit, recommendations for the diagnosis and treatment of JDM have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JDM throughout Europe. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  7. Quantitative muscle ultrasonography in the follow-up of juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, G.E.; Brussel, M. Van; Bhansing, K.J.; Hoppenreijs, E.P.A.H.; Janssen, A.J.; Royen-Kerkhof, A. Van; Pillen, S.

    2015-01-01

    INTRODUCTION: We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM). METHODS: Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT)

  8. QUANTITATIVE MUSCLE ULTRASONOGRAPHY IN THE FOLLOW-UP OF JUVENILE DERMATOMYOSITIS

    NARCIS (Netherlands)

    Habers, G. Esther A.; van Brussel, Marco; Bhansing, Kavish J.; Hoppenreijs, Esther P.; Janssen, Anjo J. W. M.; van Royen-Kerkhof, Annet; Pillen, Sigrid

    2015-01-01

    Introduction: We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM). Methods: Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT)

  9. Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis : a randomised trial

    NARCIS (Netherlands)

    Ruperto, Nicolino; Pistorio, Angela; Oliveira, Sheila; Zulian, Francesco; Cuttica, Ruben; Ravelli, Angelo; Fischbach, Michel; Magnusson, Bo; Sterba, Gary; Avcin, Tadej; Brochard, Karine; Corona, Fabrizia; Dressler, Frank; Gerloni, Valeria; Apaz, Maria T; Bracaglia, Claudia; Cespedes-Cruz, Adriana; Cimaz, Rolando; Couillault, Gerard; Joos, Rik; Quartier, Pierre; Russo, Ricardo; Tardieu, Marc; Wulffraat, Nico; Bica, Blanca; Dolezalova, Pavla; Ferriani, Virginia; Flato, Berit; Bernard-Medina, Ana G; Herlin, Troels; Trachana, Maria; Meini, Antonella; Allain-Launay, Emma; Pilkington, Clarissa; Vargova, Veronika; Wouters, Carine; Angioloni, Simona; Martini, Alberto

    2016-01-01

    BACKGROUND: Most data for treatment of dermatomyositis and juvenile dermatomyositis are from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the efficacy and safety of prednisone alone with that of prednisone plus either methotrexate or ciclosporin in children with

  10. Milk of calcium fluid collections in juvenile dermatomyositis: MR characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Samson, C.; Soulen, R.L. [Wayne State Univ., Detroit, MI (United States). Dept. of Radiology; Gursel, E. [Department of Plastic Surgery, Wayne State University School of Medicine, 3390 John R. Street, Detroit, MI 48201 (United States)

    2000-01-01

    Children with dermatomyositis may have extensive subcutaneous and intermuscular calcium-laden fluid collections referred to as ''milk of calcium.'' The distinctive MR appearance of such collections in an upper extremity of a 16-year-old girl is presented. MR can differentiate these collections from abscesses and guide appropriate therapy. (orig.)

  11. Comparison of the Utility and Validity of Three Scoring Tools to Measure Skin Involvement in Patients With Juvenile Dermatomyositis

    OpenAIRE

    Campanilho-Marques, R; Almeida, B.; Deakin, C; Arnold, K; Gallot, N.; De Iorio, M.; Nistala, K; Pilkington, C.A.; Wedderburn, L. R.; Juvenile Dermatomyositis Research Group

    2016-01-01

    OBJECTIVE: To compare the abbreviated Cutaneous Assessment Tool (CAT), Disease Activity Score (DAS), and Myositis Intention to Treat Activity Index (MITAX) and correlate them with the physician's 10-cm skin visual analog scale (VAS) in order to define which tool best assesses skin disease in patients with juvenile dermatomyositis. METHODS: A total of 71 patients recruited to the UK Juvenile Dermatomyositis Cohort and Biomarker Study were included and assessed for skin disease using the CAT, D...

  12. Dermatomyositis: Signs and Symptoms

    Science.gov (United States)

    ... Meet our Partners How to Get Involved Donate Dermatomyositis (DM) Share print email share facebook twitter google ... Signs and Symptoms What happens to someone with dermatomyositis? For many decades, DM was considered “ polymyositis with ...

  13. The Paediatric Rheumatology International Trials Organisation provisional criteria for the evaluation of response to therapy in juvenile dermatomyositis.

    Science.gov (United States)

    Ruperto, Nicolino; Pistorio, Angela; Ravelli, Angelo; Rider, Lisa G; Pilkington, Clarissa; Oliveira, Sheila; Wulffraat, Nico; Espada, Graciela; Garay, Stella; Cuttica, Ruben; Hofer, Michael; Quartier, Pierre; Melo-Gomes, Jose; Reed, Ann M; Wierzbowska, Malgorzata; Feldman, Brian M; Harjacek, Miroslav; Huppertz, Hans-Iko; Nielsen, Susan; Flato, Berit; Lahdenne, Pekka; Michels, Harmut; Murray, Kevin J; Punaro, Lynn; Rennebohm, Robert; Russo, Ricardo; Balogh, Zsolt; Rooney, Madeleine; Pachman, Lauren M; Wallace, Carol; Hashkes, Philip; Lovell, Daniel J; Giannini, Edward H; Gare, Boel Andersson; Martini, Alberto

    2010-11-01

    To develop a provisional definition for the evaluation of response to therapy in juvenile dermatomyositis (DM) based on the Paediatric Rheumatology International Trials Organisation juvenile DM core set of variables. Thirty-seven experienced pediatric rheumatologists from 27 countries achieved consensus on 128 difficult patient profiles as clinically improved or not improved using a stepwise approach (patient's rating, statistical analysis, definition selection). Using the physicians' consensus ratings as the "gold standard measure," chi-square, sensitivity, specificity, false-positive and-negative rates, area under the receiver operating characteristic curve, and kappa agreement for candidate definitions of improvement were calculated. Definitions with kappa values >0.8 were multiplied by the face validity score to select the top definitions. The top definition of improvement was at least 20% improvement from baseline in 3 of 6 core set variables with no more than 1 of the remaining worsening by more than 30%, which cannot be muscle strength. The second-highest scoring definition was at least 20% improvement from baseline in 3 of 6 core set variables with no more than 2 of the remaining worsening by more than 25%, which cannot be muscle strength (definition P1 selected by the International Myositis Assessment and Clinical Studies group). The third is similar to the second with the maximum amount of worsening set to 30%. This indicates convergent validity of the process. We propose a provisional data-driven definition of improvement that reflects well the consensus rating of experienced clinicians, which incorporates clinically meaningful change in core set variables in a composite end point for the evaluation of global response to therapy in juvenile DM. Copyright © 2010 by the American College of Rheumatology.

  14. Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies

    DEFF Research Database (Denmark)

    Schwartz, TThomas W; Diederichsen, L. P.; Lundberg, Ingrid E.

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM ( JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also...

  15. Recurrent Multiple Squamous Cell Carcinomas on the Scalp in a Patient with Juvenile Dermatomyositis

    Science.gov (United States)

    Sato, Yota; Fujimura, Taku; Kambayashi, Yumi; Tsukada, Akira; Hidaka, Takanori; Tanita, Kayo; Haga, Takahiro; Hashimoto, Akira; Aiba, Setsuya

    2017-01-01

    Both long-term administration of immunosuppressive agents and chronic inflammatory conditions, such as autoimmune disease, could be risk factors for the development of cutaneous squamous cell carcinoma (cSCC). In this report, we present a case of recurrent multiple cSCC on the scalp in a patient with juvenile dermatomyositis who had been administered cyclosporine and Predonine since she was a 1-year-old infant. Interestingly, immunohistochemical staining revealed IL-17-producing cells adjacent to IL-17R-expressing atypical keratinocytes. Our present case suggested that IL-17/IL-17R signaling might contribute to the carcinogenesis of cSCC. PMID:28203172

  16. Juvenile dermatomyositis with anti-signal recognition particle antibodies: a case report

    Directory of Open Access Journals (Sweden)

    Sofia Almeida

    2016-04-01

    Full Text Available Serologic investigation has been explored in inflammatory myopathies in order to define subgroups that can help us predict clinical course, treatment and prognosis. The level of similarity between juvenile and adult myopathies regarding the presence of myositis-specific autoantibodies has not been fully elucidated. We report the case of a 8-year-old girl who presented with a rapid progression of muscle weakness and cutaneous signs consistent with the diagnosis of juvenile dermatomyositis and whose serologic testing revealed the presence of anti-signal recognition particle (SRP antibodies. So far these antibodies have been described mostly in adult subsets, frequently associated with poorer outcomes and rarely related to cutaneous manifestations. The knowledge of the degree of overlap between paediatric and adult SRP positive myopathies may improve the medical care we provide to these children.

  17. Calcinosis in juvenile dermatomyositis : a possible role for the vitamin K-dependent protein matrix Gla protein

    NARCIS (Netherlands)

    Van Summeren, M. J. H.; Spliet, W. G. M.; Van Royen-Kerkhof, A.; Vermeer, C.; Lilien, M.; Kuis, W.; Schurgers, L. J.

    2008-01-01

    Objectives. The aims of the present study were to investigate whether the calcification inhibitor matrix Gla protein (MGP) is expressed in muscle biopsies of patients with juvenile dermatomyositis (JDM), and whether different forms of MGP are differentially expressed in JDM patients with and without

  18. Dermatomyositis (Juvenile)

    Science.gov (United States)

    ... most inflammatory myopathies is unknown; however, the leading theory is that the body's immune system mistakenly directs ... is to minimize inflammation, improve function, and prevent disability. The treatment should be early and requires a ...

  19. Calcinose na infância, um desafio terapêutico Calcinosis in juvenile dermatomyositis, a therapeutic challenge

    Directory of Open Access Journals (Sweden)

    Tânia Caroline Monteiro de Castro

    2007-02-01

    Full Text Available A calcinose é uma complicação comum e incapacitante da dermatomiosite. Ela constitui uma característica marcante da doença, ocorrendo principalmente em pacientes pediátricos. Pouco se sabe a respeito de sua fisiopatologia e não existe um tratamento reconhecido universalmente. Este trabalho relata dois casos de dermatomiosite juvenil e calcinose grave com melhora clínica com o uso do alendronato e do diltiazen em um paciente e da imunoglobulina endovenosa em outro.Calcinosis is a common and debilitating complication of dermatomyositis. It is a hallmark of the disease, occurring mainly in pediatric patients. Little is known about its pathophysiology, and there is no universally recognized treatment. This is a report of two children with juvenile dermatomyositis and severe calcinosis who showed improvement with the use of alendronate and diltiazen in one patient, and intravenous immunoglobulin in the other.

  20. Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease

    OpenAIRE

    Kim, Susan; Kahn, Philip; Robinson, Angela B; Lang, Bianca; Shulman, Andrew; Oberle, Edward J.; Schikler, Kenneth; Curran, Megan Lea; Barillas-Arias, Lilliana; Spencer, Charles H; Rider, Lisa G; Huber, Adam M.

    2017-01-01

    Background Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript...

  1. Fatores de risco associados à calcinose na dermatomiosite juvenil Risk factors associated with calcinosis of juvenile dermatomyositis

    Directory of Open Access Journals (Sweden)

    Adriana M. E. Sallum

    2008-02-01

    calcinosis in children and adolescents with juvenile dermatomyositis. METHODS: A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale, pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies, gastrointestinal problems (gastroesophageal reflux and/or heart disease (pericarditis and/or myocarditis; laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase; and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. RESULTS: Calcinosis was identified in 23 (43% patients, and in six (26% patients it had emerged prior to diagnosis while in 17 (74% it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01 and pulmonary (p = 0.02 involvement and the need for one or more immunosuppressor (methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide to treat juvenile dermatomyositis (p = 0.03 were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95%CI 1.59-152.2 and the use of one or more immunosuppressor (OR = 4.01; 95%CI 1.08-14.87 were independently associated with the presence of calcinosis. CONCLUSIONS: Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment.

  2. 重症幼年皮肌炎的治疗进展%Therapeutic advances on juvenile dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    孙广超; 曾华松

    2013-01-01

    The current management for juvenile dermatomyositis includes the initial use of corticosteroids followed by various conventional second-line treatments such as methotrexate and azathioprine.Intravenous immunoglobulin is a reasonable short-term treatment with proven benefit.Cyclosporine or tacrolimus have shown efficacy in juvenile dermatomyositis including those patients with interstitial lung disease,whereas mycophenolate mofetil is effective in both polymyositis and refractory dermatomyositis.The curative effect of biological agents needs to be further studied.%当前幼年皮肌炎的治疗以皮质激素及各种传统的免疫抑制剂(如甲氨蝶呤和硫唑嘌呤)为主.静脉注射免疫球蛋白是一种有效的短期治疗方法.环孢霉素和他克莫司对皮肌炎包括肺间质病变有效,而霉酚酸酯则对多发性肌炎和难治性皮肌炎有效.生物制剂的疗效有待于进一步验证.

  3. Lower extremity lipedema, upper extremity lipodystrophy and severe calcinosis complicating juvenile dermatomyositis.

    Science.gov (United States)

    Pavlov-Dolijanovic, Slavica R; Vujasinovic Stupar, Nada Z; Gavrilov, Nikola; Seric, Srdjan

    2014-11-01

    Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood. Significant proportions of patients have residual weakness, muscle atrophy, joint contractures, and calcinosis. Recently, new clinical findings, such as lipodystrophy accompanied with increased fat deposition in certain areas, have been reported. So far, it is not known whether the redistribution of body fat may be the type of lipedema of lower extremity. We describe a 39-year-old woman who was diagnosed with JDM at the age of 7. Later she developed symmetrical lipodystrophy of upper extremities and symmetrical lipedema of lower extremities (making 2 and 58.3 % of total body fat mass, respectively), with multiple calcified nodules in the subcutaneous tissues. These nodules gradually increased in size despite therapy. Capillaroscopy findings showed scleroderma-like abnormalities. ANA and anti-U1RNP antibodies were positive. Similar cases with simultaneous occurrence of the lipedema of lower extremities, lipodystrophy of upper extremities, and severe calcinosis complicating JDM have not been published so far. We showed that the calcinosis and lipodystrophy were associated with short duration of active disease. Also, we display case that raises the question whether it is possible overlapping autoimmune diseases revealed during follow-up.

  4. Efficacy of thalidomide in a girl with inflammatory calcinosis, a severe complication of juvenile dermatomyositis

    Directory of Open Access Journals (Sweden)

    Inayama Yoshiaki

    2010-02-01

    Full Text Available Abstract We report a 14-year-old girl with juvenile dermatomyositis (JDM complicated by severe inflammatory calcinosis successfully treated with thalidomide. She was diagnosed as JDM when she was 4 years old after a few months of increasing lethargy, muscle pain, muscle weakness, and rash. During three months, clinical manifestations and abnormal laboratory findings were effectively treated with oral prednisolone. However, calcinosis was recognized 18 months after disease onset. Generalized calcinosis rapidly progressed with high fever, multiple skin/subcutaneous inflammatory lesions, and increased level of CRP. Fifty mg/day (1.3 mg/kg day of oral thalidomide was given for the first four weeks, and then the dose was increased to 75 mg/day. Clinical manifestations subsided, and inflammatory markers had clearly improved. Frequent high fever and local severe pain with calcinosis were suppressed. The levels of FDP-E, IgG, and tryglyceride, which were all elevated before the thalidomide treatment, were gradually returned to the normal range. Over the 18 months of observation up to the present, she has had no inflammatory calcinosis, or needed any hospitalization, although established calcium deposits still remain. Her condition became painless, less extensive and less inflammatory with the CRP level below 3.08 mg/dL. Recent examination by whole-body 18F-FDG-PET-CT over the 15 months of thalidomide treatment demonstrated fewer hot spots around the subcutaneous calcified lesions.

  5. Nursing for children with juvenile dermatomyositis%儿童皮肌炎的护理

    Institute of Scientific and Technical Information of China (English)

    邵淑芳

    2007-01-01

    @@ 皮肌炎(juvenile dermatomyositis,JDM)是一种以横纹肌和皮肤广泛非化脓性炎症为特征的多系统受累的疾病,该病为儿童少见病,且危害大,如不正确治疗护理易发生严重后果.儿童皮肌炎临床表现和病理改变与成人不同[1],尤以发热、并发上呼吸道感染、甲周红斑或毛细血管扩张方面的表现较明显[2].在英国和美国,估计每年发病人数为4~5人/100万[1].基于本病具有的临床表现及5%的患儿有严重的后遗症的特性[2],2005年1月-2006年9月,我科对10例JDM患儿进行了精心的治疗及护理,收到良好效果,现报道如下.

  6. Duration of chronic inflammation alters gene expression in muscle from untreated girls with juvenile dermatomyositis

    Directory of Open Access Journals (Sweden)

    Gordish-Dressman Heather

    2008-07-01

    Full Text Available Abstract Background To evaluate the impact of the duration of chronic inflammation on gene expression in skeletal muscle biopsies (MBx from untreated children with juvenile dermatomyositis (JDM and identify genes and biological processes associated with the disease progression, expression profiling data from 16 girls with active symptoms of JDM greater than or equal to 2 months were compared with 3 girls with active symptoms less than 2 months. Results Seventy-nine genes were differentially expressed between the groups with long or short duration of untreated disease. Genes involved in immune responses and vasculature remodelling were expressed at a higher level in muscle biopsies from children with greater or equal to 2 months of symptoms, while genes involved in stress responses and protein turnover were expressed at a lower level. Among the 79 genes, expression of 9 genes showed a significant linear regression relationship with the duration of untreated disease. Five differentially expressed genes – HLA-DQA1, smooth muscle myosin heavy chain, clusterin, plexin D1 and tenomodulin – were verified by quantitative RT-PCR. The chronic inflammation of longer disease duration was also associated with increased DC-LAMP+ and BDCA2+ mature dendritic cells, identified by immunohistochemistry. Conclusion We conclude that chronic inflammation alters the gene expression patterns in muscle of untreated children with JDM. Symptoms lasting greater or equal to 2 months were associated with dendritic cell maturation and anti-angiogenic vascular remodelling, directly contributing to disease pathophysiology.

  7. 儿童皮肌炎35例临床分析%Clinical analysis of 35 patients with juvenile dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    丁艳; 张燕; 蒙秉新

    2012-01-01

    目的 研究儿童皮肌炎(Juvenile dermatomyositis,JDM)的临床特点.方法 采用回顾性分析方法对1990年4月-2010年3月在本院住院的35例JDM患者进行临床资料分析. 结果 35例JDM患者有较多并且较严重的并发症如化脓性感染、肺结核、钙质沉着、肌肉挛缩、心脏受累等,激素等治疗后完全缓解31例,部分缓解2例,未愈1例,死亡2例.结论 JDM是慢性活跃性疾病,结局好坏与治疗是否合适有关,严重的并发症仍然普遍存在.%Objective To analyze the clinical characteristics of juvenile dermatomyositis. Methods Thirty-five patients with juvenile dermatomyositis in Hainan Provincial Hospital were analyzed retrospectively. Results Most of Juvinilee dermatomyositis patients with severe complications, such as pyogenic infections, pulmonary tuberculosis, calcinosis, muscle contractures and heart involvement, et al. Thirty-one of the 35 patients with juvenile dermatomyositis were totlly relieved,2 partial relieved,one failed and 2 died after treated with coorticosteroids. Conclusion Juvenile dermatomyositis is a chronic inflammatory illness. Functional outcomes are associated with therpied chosen and serious complications are still common.

  8. Minor sperm abnormalities in young male post-pubertal patients with juvenile dermatomyositis

    Directory of Open Access Journals (Sweden)

    A.J.P. Moraes

    2008-12-01

    Full Text Available The objective of the present study was to identify sperm abnormalities in young male patients with juvenile dermatomyositis (JDM. In 2005, 18 male JDM patients, diagnosed according to the criteria of Bohan and Peter, were followed at the Pediatric Rheumatology Unit and Rheumatology Division, of our Institution. Of the 18 males, 11 were pre-pubertal and 7 were post-pubertal. Two of 7 post-pubertal JDM male patients were excluded: one for orchidopexy for cryptorchidism and the other for testicular ectopia in the left testis. The remaining 5 post-pubertal JDM patients were prospectively evaluated on the basis of two semen analyses, according to the World Health Organization (WHO, urologic evaluation, testicular Doppler ultrasound hormone profile. The data of the JDM patients were compared with those of 5 age-matched healthy controls. The median age 18, was similar in JDM patients and controls. All JDM patients had teratozoospermia (abnormal sperm morphology, as did 4 (80% of the controls. One of JDM patients had previous oligoasthenoteratozoospermia treated with intravenous cyclophosphamide with normalization of the number and concentration of the sperm after 5 years. All sperm parameters (sperm concentration, total sperm count and total motile sperm count by WHO, and sperm morphology by Kruger strict criteria, testicular volumes by Prader orchidometer and ultrasound, and hormones were similar in JDM patients compared with controls. The frequency of anti-sperm antibodies was similar in both groups. All JDM patients had minor sperm abnormalities in the head, midpiece, and/or tail of spermatozoids. Serial semen analyses in larger study populations are necessary to identify the extent and duration of sperm abnormalities in male patients with idiopathic inflammatory myopathies.

  9. A hyper-ferritinemia syndrome evolving in recurrent macrophage activation syndrome, as an onset of amyopathic juvenile dermatomyositis: a challenging clinical case in light of the current diagnostic criteria.

    Science.gov (United States)

    Poddighe, Dimitri; Cavagna, Lorenzo; Brazzelli, Valeria; Bruni, Paola; Marseglia, Gian Luigi

    2014-11-01

    Juvenile dermatomyositis is an immune-mediated inflammatory multi-system disease involving mainly striated muscles and skin. Typical dermatological features are fundamental to establish the diagnosis, especially whenever the myopathy is very mild or absent, as it occurs in the form called as amyopathic juvenile dermatomyositis. Sometimes, systemic rheumatic diseases can develop a hyperferritinemia syndrome characterized by hemophagocytosis, namely macrophage activation syndrome, which represents a severe and life-threatening complication. Here, we describe a complex clinical history characterized by a hyper-ferritinemia syndrome after infectious mononucleosis, leading to recurrent episodes of macrophage activation syndrome. Finally, the late onset of several skin changes brought to a diagnosis of amyopathic juvenile dermatomyositis.

  10. A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis

    Science.gov (United States)

    Yoshida, Yoichiro; Takahashi, Hironori; Oka, Hideharu; Kajihama, Aya; Nakau, Koichi; Sugimoto, Masaya; Minami-Hori, Masako; Azuma, Hiroshi

    2017-01-01

    Juvenile-onset systemic sclerosis (jSSc) is a rare condition, having unique characteristic features compared to adult-onset SSc. Although cardiac involvement (CI) is known as a leading cause of mortality overall in SSc, the importance of CI in jSSc has not been emphasized. Here we present a 13-year-old female with jSSc overlapped with dermatomyositis (DM) complicated CI. She developed skin thickness and induration, Raynaud's phenomenon, digital pitting scars in fingertips, and skeletal myositis. Oral prednisolone and pulse methotrexate treatment led to the improvement of skin findings; however two weeks after the initiation she suddenly presented with muscle pain and dyspnea within a few days. Cardiac investigations then showed pericardiac effusion and diastolic dysfunction due to significant biventricular hypertrophy causing heart failure. As pericardiac effusion and exacerbation of skeletal myositis were evident, steroid pulse therapy was initiated. Unexpectedly, not only the myositis but also the CI including diastolic dysfunction was improved. She thereafter followed a favorable clinical course without reactivation of the CI or cardiac fibrosis. As a conclusion, close attention to CI must be paid in jSSc patients, especially when skeletal muscle involvement is evident and immunosuppressive therapy may be effective for CI in jSSc in cases where it occurs abruptly. PMID:28316854

  11. Clinical analysis of 35 patients with juvenile dermatomyositis.%儿童皮肌炎35例临床分析

    Institute of Scientific and Technical Information of China (English)

    丁艳; 张燕; 蒙秉新

    2011-01-01

    Objective To provide theoretic basis for clinical characteristics of Juvenile Dermatomyositis. Methods Thirty-five patients with Juvenile Dermatomyositis from our hospital were analyzed retrospectively. Results There were so many and serious complications such as pyogenic infections -, pulmonary tuberculosis tcalcinosis <,contracturesand and heart involved et al. In the Thirty-five patients with Juvenile Dermatomyositis,Thirty-one,two,one and two persons had experienced complete remission,partial remission,no response and death respectively. Conclusion Juvenile dermatomyositis is a chronic inflammatory illness. Functional outcomes have become good with modem treatments and serious complications are still common.%目的分析儿童皮肌炎(Juvenile dermatomyositis,JDM)的临床特点.方法 采用回顾性分析方法对1990年4月~2010年3月在本院住院的35例JDM患者临床资料进行分析.结果 35例JDM患者有较多并且较严重的并发症如化脓性感染、肺结核、钙质沉着、肌肉挛缩、心脏受累等,激素等治疗后完全缓解31例,部分缓解2例,未愈1例,死亡2例.结论 JDM是慢性活跃性疾病,预后与治疗是否合适有关,严重的并发症仍然普遍存在.

  12. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

    Science.gov (United States)

    Rider, Lisa G; Aggarwal, Rohit; Pistorio, Angela; Bayat, Nastaran; Erman, Brian; Feldman, Brian M; Huber, Adam M; Cimaz, Rolando; Cuttica, Rubén J; de Oliveira, Sheila Knupp; Lindsley, Carol B; Pilkington, Clarissa A; Punaro, Marilynn; Ravelli, Angelo; Reed, Ann M; Rouster-Stevens, Kelly; van Royen-Kerkhof, Annet; Dressler, Frank; Magalhaes, Claudia Saad; Constantin, Tamás; Davidson, Joyce E; Magnusson, Bo; Russo, Ricardo; Villa, Luca; Rinaldi, Mariangela; Rockette, Howard; Lachenbruch, Peter A; Miller, Frederick W; Vencovsky, Jiri; Ruperto, Nicolino

    2017-05-01

    To develop response criteria for juvenile dermatomyositis (DM). We analyzed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. At a consensus conference, experts considered 14 top candidate criteria based on their performance characteristics and clinical face validity, using nominal group technique. Consensus was reached for a conjoint analysis-based continuous model with a total improvement score of 0-100, using absolute percent change in core set measures of minimal (≥30), moderate (≥45), and major (≥70) improvement. The same criteria were chosen for adult DM/polymyositis, with differing thresholds for improvement. The sensitivity and specificity were 89% and 91-98% for minimal improvement, 92-94% and 94-99% for moderate improvement, and 91-98% and 85-86% for major improvement, respectively, in juvenile DM patient cohorts using the IMACS and PRINTO core set measures. These criteria were validated in the PRINTO trial for differentiating between treatment arms for minimal and moderate improvement (P = 0.009-0.057) and in the RIM trial for significantly differentiating the physician's rating for improvement (P Rheumatology.

  13. Design of the muscles in motion study : a randomized controlled trial to evaluate the efficacy and feasibility of an individually tailored home-based exercise training program for children and adolescents with juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, Esther A.; van Brussel, Marco; Langbroek-Amersfoort, Anneli C.; van Royen-Kerkhof, Annet; Takken, Tim

    2012-01-01

    Background: Juvenile dermatomyositis (JDM) is a rare, often chronic, systemic autoimmune disease of childhood, characterized by inflammation of the microvasculature of the skeletal muscle and skin. Prominent clinical features include significant exercise intolerance, muscle weakness, and fatigue.

  14. Design of the muscles in motion study : a randomized controlled trial to evaluate the efficacy and feasibility of an individually tailored home-based exercise training program for children and adolescents with juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, Esther A.; van Brussel, Marco; Langbroek-Amersfoort, Anneli C.; van Royen-Kerkhof, Annet; Takken, Tim

    2012-01-01

    Background: Juvenile dermatomyositis (JDM) is a rare, often chronic, systemic autoimmune disease of childhood, characterized by inflammation of the microvasculature of the skeletal muscle and skin. Prominent clinical features include significant exercise intolerance, muscle weakness, and fatigue. De

  15. Developing a provisional, international minimal dataset for Juvenile Dermatomyositis: for use in clinical practice to inform research.

    Science.gov (United States)

    McCann, Liza J; Arnold, Katie; Pilkington, Clarissa A; Huber, Adam M; Ravelli, Angelo; Beard, Laura; Beresford, Michael W; Wedderburn, Lucy R

    2014-01-01

    Juvenile dermatomyositis (JDM) is a rare but severe autoimmune inflammatory myositis of childhood. International collaboration is essential in order to undertake clinical trials, understand the disease and improve long-term outcome. The aim of this study was to propose from existing collaborative initiatives a preliminary minimal dataset for JDM. This will form the basis of the future development of an international consensus-approved minimum core dataset to be used both in clinical care and inform research, allowing integration of data between centres. A working group of internationally-representative JDM experts was formed to develop a provisional minimal dataset. Clinical and laboratory variables contained within current national and international collaborative databases of patients with idiopathic inflammatory myopathies were scrutinised. Judgements were informed by published literature and a more detailed analysis of the Juvenile Dermatomyositis Cohort Biomarker Study and Repository, UK and Ireland. A provisional minimal JDM dataset has been produced, with an associated glossary of definitions. The provisional minimal dataset will request information at time of patient diagnosis and during on-going prospective follow up. At time of patient diagnosis, information will be requested on patient demographics, diagnostic criteria and treatments given prior to diagnosis. During on-going prospective follow-up, variables will include the presence of active muscle or skin disease, major organ involvement or constitutional symptoms, investigations, treatment, physician global assessments and patient reported outcome measures. An internationally agreed minimal dataset has the potential to significantly enhance collaboration, allow effective communication between groups, provide a minimal standard of care and enable analysis of the largest possible number of JDM patients to provide a greater understanding of this disease. This preliminary dataset can now be developed into

  16. Cutaneous signs of classical dermatomyositis.

    Science.gov (United States)

    Auriemma, M; Capo, A; Meogrossi, G; Amerio, P

    2014-10-01

    Idiopathic immune myopathies (IIM) are an heterogeneous group of autoimmune muscle disorders characterized by progressive muscle involvement. Dermatomyositis (DM) is the most common form of IIM. It is a multisystem disorder characterized by symmetric proximal, extensor, inflammatory myopathy, vascular involvement and a characteristic cutaneous eruption. Six types of DM have been identified: idiopathic, juvenile (JDM), cancer-related other autoimmune diseases-related, iatrogenic DM and amyopathic DM. Cutaneous manifestations of DM are the most important aspect of this disease and can precede from several months to years muscle or systemic involvement. Three groups of signs have been described: pathognomonic, highly characteristic and compatible. Although differences exist among the different clinical presentation of skin lesions, they share common histological findings including the presence of interface dermatitis with epidermal atrophy, basement membrane degeneration, vacuolar alteration of basal keratinocytes, and dermal changes consisting of interstitial mucin deposition and a sparse lymphocytic infiltrate. DM is a serious disease; the correct evaluation of any skin lesion suggesting an early diagnosis is of utmost importance. Skin signs may, also, represent a marker of treatment efficacy even though systemic symptoms worsening may not always be followed by more severe skin lesions.

  17. Osteoporosis grave con aplastamientos vertebrales en dermatomiositis juvenil: Efecto del tratamiento con alendronato oral Severe osteoporosis with vertebral crushes in juvenile dermatomyositis: Effect of oral alendronate therapy

    Directory of Open Access Journals (Sweden)

    Cristina Tau

    2007-02-01

    Full Text Available Los glucocorticoides son usados comúnmente para el tratamiento de enfermedades inflamatorias, autoinmunes, enfermedades malignas, y en la prevención de rechazo de órganos trasplantados. Un efecto secundario frecuente del tratamiento prolongado es la pérdida de masa ósea que se produce por varios mecanismos y es causa de osteoporosis y fracturas vertebrales. El tratamiento con disfosfonatos ha sido propuesto para esta situación. Presentamos un caso clínico de osteoporosis grave en una niña con dermatomiositis juvenil, que respondió favorablemente al tratamiento con disfosfonatos orales.Glucocorticoids are used for the treatment of inflammatory and autoimmune diseases, cancer, and in prevention of organ rejects. A frequent secondary effect of longterm treatment with corticoids is the loss of bone mass, caused by several mechanisms: decrease in the intestinal calcium absorption, increase of the renal calcium excretion at the distal renal tubule, suppressive effect on the osteoblast and also in apoptosis of osteoclasts, inhibition in local production of IGF I (Insulin-like growth factor and IGFBPs (binding IGF I proteins necessary for bone metabolism, and decrease on osteocalcin production. Longterm treatment with corticoids is associated with osteoporosis and vertebral fractures. To improve this condition, treatment with bisphosphonates has been proposed. We present here a clinical case of a girl with dermatomyositis and severe osteoporosis with vertebral crushes, who responded well to oral bisphophonate treatment.

  18. Frequência elevada de calcinose em dermatomiosite juvenil: estudo de fatores de risco High frequency of calcinosis in juvenile dermatomyositis: a risk factor study

    Directory of Open Access Journals (Sweden)

    Gleice Clemente

    2012-08-01

    Full Text Available OBJETIVO: Avaliar a frequência de calcinose em pacientes com dermatomiosite juvenil, bem como estudar possíveis fatores de risco para essa manifestação. MÉTODOS: Revisão de prontuários de 34 pacientes, com ênfase nas características demográficas, clínicas e laboratoriais, tipo de tratamento e adesão, tipo de evolução (monocíclico, crônico e policíclico e gravidade da doença. Os pacientes foram separados em grupos: aqueles que desenvolveram calcinose (até o sexto mês de acompanhamento ambulatorial e após seis meses de acompanhamento e os que não desenvolveram calcinose. Vinte e sete pacientes fizeram dois exames de capilaroscopia periungueal (CPU, os quais foram considerados alterados quando era encontrado padrão escleroderma. RESULTADOS: A média de idade de início dos sintomas dos 34 pacientes foi de 6,5 anos, e o tempo até o diagnóstico foi de 1,2 anos. Setenta por cento eram meninas. Metade dos pacientes teve curso monocíclico da doença, e apenas 14,7% tiveram vasculite grave. Quase 90% dos pacientes que realizaram CPU tiveram alteração na primeira avaliação, e 74% tiveram alteração na segunda avaliação, com uma média de 1,6 anos entre as duas. Dezesseis (47,1% pacientes apresentaram calcinose. Não houve associação entre as variáveis analisadas e o desenvolvimento da calcinose. CONCLUSÃO: Não conseguimos demonstrar a presença de fatores de risco para calcinose, apesar de termos encontrado uma frequência dessa complicação em cerca de metade dos pacientes com dermatomiosite juvenil.OBJECTIVE: To assess the frequency of calcinosis in patients with juvenile dermatomyositis, and the possible risk factors for that manifestation. METHODS: Medical record review of 34 patients, with an emphasis on the following characteristics: demographic, clinical and laboratory data; type of treatment; adherence to treatment; disease course (monocyclic, chronic and polycyclic; and disease severity. Patients were

  19. Cutaneous dermatomyositis in the era of biologicals.

    Science.gov (United States)

    Wright, Natalie A; Vleugels, Ruth Ann; Callen, Jeffrey P

    2016-01-01

    Dermatomyositis (DM) is a systemic inflammatory condition characterized by cutaneous and muscle findings, in addition to potential involvement of other organ systems. A distinct subtype of DM exists that is categorized by cutaneous findings with absent or minimal muscle involvement, referred to as clinically amyopathic dermatomyositis or dermatomyositis sine myositis. A variety of topical, immunosuppressive, and immunomodulatory therapies have been utilized to treat cutaneous DM. The advent of biological agents including tumor necrosis factor-α antagonists, intravenous immunoglobulin, rituximab, and others has allowed for the use of these agents with varying degrees of success for the treatment of cutaneous DM.

  20. [Role of magnetic resonance imaging in the diagnosis of juvenile dermato-myositis and polymyositis in Chinese children].

    Science.gov (United States)

    Lai, J M; Wu, F Q; Zhou, Z X; Yuan, X Y; Su, G X; Li, S N; Yan, Y C; Zhu, J; Kang, M

    2016-10-02

    Objective: To evaluate the utility of magnetic resonance imaging (MRI) in diagnosis of juvenile dermatomyositis and polymyositis (JDM-PM) in children. Method: Fifty-four patients with JDM-PM in the active stage were enrolled in the study group. Twelve patients with benign acute childhood myositis and forty patients with juvenile idiopathic arthritis (JIA) complicated with myositis were enrolled as controls. MRI imaging of thighs was performed in all patients, fast spin echo T1WI, T2WI, and STIR were obtained in all patients.Muscle biopsy was performed in 41/54 patients with JDM-PM. We compared the value of MRI in diagnosis of JDM-PM with muscle biopsy, electromyography and serum aspartate transaminase (AST), alanine transaminase (ALT), creatine kinase (CK), isoenzyme of creatine kinase (CKMB), lactate dehydrogenase (LDH), hydroxybutyrate dehydrogenase (HBDH) levels. Continuous normally distributed variables were reported as means and continuous non-normally distributed variables as median. Chi-square test and Fisher exact test were used to test differences between MRI and other categorical variables. Result: A total of 54 patients were included. Twenty-seven patients were male and the others were female. Average age of the patients was (7.1±3.5) years (2-13 years); 45(83%) paitests were JDM cases and 9(17%) patients had JPM. All patients had MRI examination. Of the 54 patients, 53 had multiple myositis; 10 out of 50 (19%) patients received second MRI after treatment, 6 out of 10 patients had normal findings, 4 patients showed obviously improved images; 41 out of 54 patients underwent muscle biopsy; 22 out of 41 patients had inflammatory cells infiltration and muscle fiber degeneration. The results of the muscle enzyme tests are as follows: 27 (50%) patients had elevated AST, 24 (44%) patients had elevated ALT, 22 (41%) patients had elevated CK, 18(33%) patients had elevated CKMB, and LDH rose in 30 (56%) patients, HBDH rose in 28(52%) patients. These results

  1. Cryopyrin-Associated Autoinflammatory Syndromes (CAPS) - Juvenile

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  2. Increased Fas and Bcl-2 Expression on Peripheral Blood T and B Lymphocytes from Juvenile-Onset Systemic Lupus Erythematosus, but not from Juvenile Rheumatoid Arthritis and Juvenile Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Bernadete L. Liphaus

    2006-01-01

    Full Text Available Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE, juvenile rheumatoid arthritis (JRA and juvenile dermatomyositis (JDM. Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal–Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

  3. Increased Fas and Bcl-2 expression on peripheral blood T and B lymphocytes from juvenile-onset systemic lupus erythematosus, but not from juvenile rheumatoid arthritis and juvenile dermatomyositis.

    Science.gov (United States)

    Liphaus, Bernadete L; Kiss, Maria H B; Carrasco, Solange; Goldenstein-Schainberg, Claudia

    2006-01-01

    Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE), juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDM). Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC) were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal-Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

  4. The pathogenesis of interstitial lung disease (ILD) associated with polymyositis (PM) and dermatomyositis (DM)%多发性肌炎(PM)/皮肌炎(DM)相关的肺间质病变发(ILD)病机制的研究进展

    Institute of Scientific and Technical Information of China (English)

    张立

    2015-01-01

    肺间质病变(interstitial lung disease,ILD)是多发性肌炎(polymyositis,PM)/皮肌炎(dermatomyositis,DM)的常见并发症,预后不良且死亡率高,是PM/DM患者住院和死亡的重要原因.PM/DM相关ILD发病机制目前仍不清楚;治疗上仍以激素为主,尚缺乏有效的治疗方法,近年来,对ILD的发病机制和治疗已成为一个研究热点.本文就参与发病的自身抗体、免疫细胞、细胞因子、组织蛋白酶、遗传因素学等方面对PM/DM相关的ILD的发病机制研究作一简要综述.

  5. A Fatal Complication of Dermatomyositis: Spontaneous Pneumomediastinum

    Directory of Open Access Journals (Sweden)

    Ezgi Demirdöğen Çetinoğlu

    2016-04-01

    Full Text Available Interstitial lung disease (ILD is a negative prognostic factor associated with increased morbidity and mortality in patients with dermatomyositis (DM. Spontaneous pneumomediastinum is a rare complication of DM and it can be fatal. We present a 48-year-old woman with DM and ILD complicated by pneumomediastinum without pneumothorax and subcutaneous emphysema.

  6. A Fatal Complication of Dermatomyositis: Spontaneous Pneumomediastinum

    OpenAIRE

    Ezgi Demirdöğen Çetinoğlu; Ediz Dalkılıç; Muharrem Erol; Nilüfer Aylin Acet; Ahmet Ursavaş; Ercüment Ege

    2016-01-01

    Interstitial lung disease (ILD) is a negative prognostic factor associated with increased morbidity and mortality in patients with dermatomyositis (DM). Spontaneous pneumomediastinum is a rare complication of DM and it can be fatal. We present a 48-year-old woman with DM and ILD complicated by pneumomediastinum without pneumothorax and subcutaneous emphysema.

  7. The evolving spectrum of polymyositis and dermatomyositis--moving towards clinicoserological syndromes: a critical review.

    Science.gov (United States)

    Tansley, Sarah; Gunawardena, Harsha

    2014-12-01

    The idiopathic inflammatory myopathies: polymyositis (PM) and dermatomyositis (DM) have been historically defined by broad clinical and pathological criteria. These conditions affect both adults and children with clinical features including muscle weakness, skin disease and internal organ involvement. Over the last few years, it has become increasingly apparent that using a clinico-serological approach, both DM and PM can be defined into more homogeneous subsets. A large number of antibodies are directed against cytoplasmic or nuclear components involved in key regulatory intra-cellular processes including protein synthesis, translocation and gene transcription within this disease spectrum. In addition, these autoantibodies are found in patients with clinical features other than myositis, in particular 'idiopathic' interstitial pneumonia emphasizing that these patients may in fact be a formes-frustes of autoimmune connective tissue disease. Other important findings are the identification of specific autoantibodies in both cancer-associated dermatomyositis, clinically amyopathic dermatomyositis and juvenile dermatomyositis, which previously were classically described as antibody-negative clinical subsets. Finally, work has highlighted how target autoantigens identified in the myositis-connective tissue disease overlap share common cellular mechanisms, which provides us with further insights into disease pathogenesis.

  8. Increased presence of FOXP3+ regulatory T cells in inflamed muscle of patients with active juvenile dermatomyositis compared to peripheral blood.

    Directory of Open Access Journals (Sweden)

    Yvonne Vercoulen

    Full Text Available Juvenile dermatomyositis (JDM is an immune-mediated inflammatory disease affecting the microvasculature of skin and muscle. CD4+ CD25+ FOXP3+ regulatory T cells (Tregs are key regulators of immune homeostasis. A role for Tregs in JDM pathogenesis has not yet been established. Here, we explored Treg presence and function in peripheral blood and muscle of JDM patients. We analyzed number, phenotype and function of Tregs in blood from JDM patients by flow cytometry and in vitro suppression assays, in comparison to healthy controls and disease controls (Duchenne's Muscular Dystrophy. Presence of Tregs in muscle was analyzed by immunohistochemistry. Overall, Treg percentages in peripheral blood of JDM patients were similar compared to both control groups. Muscle biopsies of new onset JDM patients showed increased infiltration of numbers of T cells compared to Duchenne's muscular dystrophy. Both in JDM and Duchenne's muscular dystrophy the proportion of FOXP3+ T cells in muscles were increased compared to JDM peripheral blood. Interestingly, JDM is not a self-remitting disease, suggesting that the high proportion of Tregs in inflamed muscle do not suppress inflammation. In line with this, peripheral blood Tregs of active JDM patients were less capable of suppressing effector T cell activation in vitro, compared to Tregs of JDM in clinical remission. These data show a functional impairment of Tregs in a proportion of patients with active disease, and suggest a regulatory role for Tregs in JDM inflammation.

  9. Dermatomyositis Sine Myositis with Membranoproliferative Glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Mohammad Bagher Owlia

    2012-01-01

    Full Text Available Dermatomyositis (DM is an autoimmune disease that is characterized by involvement of proximal musculature and skin. We report a 52-year-old woman with a 6-year history of dermatomyositis sine myositis, who developed lower extremity edema and proteinuria. Pathological examination of renal biopsy showed membranoproliferative glomerulonephritis. She received steroid, cyclophosphamide, and mycophenolate mofetil. Over the 9 to 10 months after the beginning of treatment, the proteinuria was improved.

  10. PANNICULITIS IN DERMATOMYOSITIS

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    O. A. Antelava

    2016-01-01

    Full Text Available Dermatomyositis (DM and polymyositis (PM belong to idiopathic inflammatory myopathies (IIM and are characterized by inflammatory injury to the skeletal muscles. In DM, unlike PM, there is skin injury that serves as a pathognomonic sign of this condition. Panniculitis (PN is one of the rare cutaneous manifestations in this disease.Objective: to investigate the clinical and laboratory characteristics of IIM accompanied by PN and to elaborate guidelines for managing these patients.Subjects and methods. Examinations were made in 318 patients (75 men and 243 women aged 18 to 80 years who were diagnosed with IIM (mean disease duration of 18.97±7.4 months and followed up at the V.A. Nasonova Research Institute of Rheumatology in 1996 to 2015.Results and discussion. In 12 (3.8% of the 318 patients, lobular PN that was associated with the diagnosis of DM verified in all cases. The clinical picture in this patient group was also characterized by skin injury as erythema on the face and trunk and Gottron’s papules (100%, periungual capillaritis (91.7%, ulceronecrotic vasculitis (57.3%, periorbital edema (75%, fever (41.7%, alopecia (50%, and joint involvement (25%.Conclusion. The development of PN is associated with the acute period of DM and the emergence of new foci is related to an exacerbation of this disease, which requires active therapy. 

  11. [Infantile dermatomyositis. Clinical aspects and prospective treatments].

    Science.gov (United States)

    Cordone, G; Buoncompagni, A; Ciccone, O; Minetti, C; Fasce, L; Scribanis, R; De Langlade, E

    1991-10-01

    Juvenile dermatomyositis (DMI) is a rare illness of multisystem inflammatory disease of unknown etiology. It is usually considered as a part of the spectrum of polymyositis/dermatomyositis (PM/DM), and is distinguished from the adult form in having a number of different clinical features as well as distinctive pathologic findings. Although the clinical findings and course of the disease, laboratory evaluation and therapy have been reviewed, many of these studies are old and may not accurately reflect the current status of the disease as it is now defined and treated. Generally it is first diagnosed by the appearance of typical signs in both the skin and muscles. The clinical picture shows early signs of heterogeneity and is confirmed by these latter developments. Its classification falls into three groups: acute single cycle, chronic polycycle and continued chronic; this sickness invariably evolves into one or another of these three. Our current work involves 15 cases of DMI; 8 have acute monocycle, 4 chronic polycycle and 3 continued chronic. This illustrates the clinical and biological heterogenousness of this illness which in itself presents extreme difficulties when considering possible treatment. From our experience, high dose of corticosteroida intraveins have had the best result. Where cases have been acute at the beginning of the sickness and where there has been complications, particularly those of a bronco-pulmonary nature (we treated 3 such cases) it has proven to be beneficial to use corticosteroid together with the immunosuppresant: ciclofosfamide. The use of ciclosporina in one case who had resisted other forms of therapy has appeared to be effective, though this has yet to be verified.

  12. Subcutaneous Emphysema, Pneumomediastinum and Pneumothorax in a Patient with Dermatomyositis

    Science.gov (United States)

    Bakhshaee, Mehdi; Jokar, Mohammad Hassan; Mirfeizi, Zahra; Atabati, Elham; Tarighat, Somayeh

    2017-01-01

    Introduction: Spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema are rare, but serious complications of inflammatory myopathies and occur more commonly in DM than PM. complications of dermatomyositis (DM) and polymyositis (PM), both of which can be fatal. Case Report: A 20-year-old woman was admitted with neck pain, dyspnea, cough, and fever. She had been diagnosed with dermatomyositis 21 months prior. A thorax computed tomography (CT) scan revealed ground glass opacities in her lungs, pneumomediastinum, pneumothorax, and subcutaneous emphysema. Despite intensive immunosuppressive therapy, clinical deterioration and radiological progression were observed, ultimately the patient died. Conclusion: During the care for a patient with dermatomyositis, the otorhinolaryngologist should be cautious of rapidly progressive and fatal neck subcutaneous emphysema. For a patient with dermatomyositis and with normal bronchoscopy and esophagoscopy, the main treatment is control of dermatomyositis with medical therapy. Therefore, a tracheostomy and/or mechanical ventilation may not be necessary.

  13. Subcutaneous Emphysema, Pneumomediastinum and Pneumothorax in a Patient with Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Mehdi Bakhsaee

    2017-03-01

    Full Text Available Introduction:Spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema are rare, but serious complications of inflammatory myopathies and occur more commonly in DM than PM. complications of dermatomyositis (DM and polymyositis (PM, both of which can be fatal. Case Report:A 20-year-old woman was admitted with neck pain, dyspnea, cough, and fever. She had been diagnosed with dermatomyositis 21 months prior. A thorax computed tomography (CT scan revealed ground glass opacities in her lungs, pneumomediastinum, pneumothorax, and subcutaneous emphysema. Despite intensive immunosuppressive therapy, clinical deterioration and radiological progression were observed, ultimately the patient died. Conclusion:During the care for a patient with dermatomyositis, the otorhinolaryngologist should be cautious of rapidly progressive and fatal neck subcutaneous emphysema. For a patient with dermatomyositis and with normal bronchoscopy and esophagoscopy, the main treatment is control of dermatomyositis with medical therapy. Therefore, a tracheostomy and/or mechanical ventilation may not be necessary.

  14. Subcutaneous Emphysema, Pneumomediastinum and Pneumothorax in a Patient with Dermatomyositis

    OpenAIRE

    Mehdi Bakhsaee; Mohammad Hassan Jokar; Zahra Mirfeizi; Elham Atabati; Somayeh Tarighat

    2017-01-01

    Introduction:Spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema are rare, but serious complications of inflammatory myopathies and occur more commonly in DM than PM. complications of dermatomyositis (DM) and polymyositis (PM), both of which can be fatal. Case Report:A 20-year-old woman was admitted with neck pain, dyspnea, cough, and fever. She had been diagnosed with dermatomyositis 21 months prior. A thorax computed tomography (CT) scan revealed ground glass opacities i...

  15. Dermatomyositis, Gottron's papule (image)

    Science.gov (United States)

    This is Gottron's sign, seen in dermatomyositis (an inflammatory disease of the muscles and skin). Violet-colored inflammation over the knuckles is an important diagnostic finding in dermatomyositis, since ...

  16. Four dermatomyositis-specific autoantibodies-anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5-in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort.

    Science.gov (United States)

    Bodoki, Levente; Nagy-Vincze, Melinda; Griger, Zoltán; Betteridge, Zoe; Szöllősi, Lászlóné; Dankó, Katalin

    2014-12-01

    Idiopathic inflammatory myopathies (IIMs) are chronic systemic autoimmune diseases characterised by symmetrical, proximal muscle weakness. Dermatomyositis represents one subset of IIMs, in which skin rashes are present in addition to muscle weakness. Myositis-specific antibodies can only be detected in myositis, and they are directed against specific proteins found in the cytoplasm or in the nucleus of cells. With this case-based article, we introduce the recently detected anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5 antibodies that form various clinical groups. These antibodies could be detected in patients with dermatomyositis. The myositis-specific autoantibodies of three hundred and thirty-seven Hungarian patients with IIM were detected. Retrospective analysis of the clinical findings has also been introduced by revision of the medical history. We had twelve patients with anti-TIF1γ positivity, four patients with anti-NXP2 positivity and four patients with anti-SAE positivity. We did not have any positive anti-MDA5 patients. The most relevant clinical findings were similar to those seen in previously published reports. Eleven of the twelve patients with anti-TIF1γ positivity had classical dermatomyositis. Three of the twelve anti-TIF1γ patients had cancer during the disease progression. This was two out of four for the anti-NXP2 subgroup and one in four for the anti-SAE subgroup. In two juvenile dermatomyositis cases, typical ulceration was seen in patients with anti-TIF1γ positivity. The frequency of pulmonary fibrosis during the disease progression was 2/12, 1/4 and 1/4 in anti-TIF1γ, anti-NXP2 and anti-SAE, respectively. Other extra-muscular manifestations, such as arthralgia, dysphagia, dysphonia and dyspnoea, were also detectable. The myositis subgroups determined by these myositis-specific autoantibodies differ from each other in their symptoms, prognosis and therapy responsiveness. Their detection is helpful for the preparation of an adequate

  17. Nasopharyngeal carcinoma and Dermatomyositis: a case report

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    P. Sfriso

    2011-09-01

    Full Text Available Nasopharyngeal carcinoma has long been reported as the predominant type of cancer associated with dermatomyositis in many several Asian countries, including Hong Kong, Singapore, and Southern-Cina. Dermatomyositis is one of the idiopathic inflammatory myopathies showing characteristic cutaneous manifestations. Reviews from the western literature have demonstrated that certain cancers, such as ovarian and breast carcinoma in women and lung and prostate carcinoma in men, are highly associated with DM relative to the general population. We report the case of a Caucasian Italian patient with nasopharyngeal carcinoma and dermatomyositis. Considering the rarity of nasopharyngeal carcinoma among whites, both the detection and the report of each new case are noteworthy in defining the geographic and ethnic distribution of this tumor.

  18. Clinical analysis on juvenile dermatomyositis combined with soft-tissue calcification%幼年皮肌炎合并软组织钙化临床分析

    Institute of Scientific and Technical Information of China (English)

    李晓云; 田恒创; 谷文佳; 王春艳; 兰洁

    2015-01-01

    目的 探讨幼年皮肌炎(JDM)合并软组织钙化临床特点. 方法 回顾性分析47例JDM患者合并软组织钙化(皮下钙化组)特点,并与89例非皮下钙化组患者(非皮下钙化组)对比.结果 皮下钙化组Gotton征、肌挛缩和关节功能障碍比例高于非皮下钙化组[87.23%(41/47)比43.82%(39/89)、68.09%(32/47)比21.35%(19/89)],差异有统计学意义(P1.5 mg/kg)、发病后应用免疫抑制比例及发病时肌酸激酶水平低于非皮下钙化组[17.02%(8/47)比68.54%(61/89)、25.53%(12/47)比88.76%(79/89)、(566.45±240.41) U/L比(1 680.12±656.50)U/L],差异有统计学意义(P<0.05). 结论 JDM合并皮肤Gotton征者更易发生皮下钙化;皮下钙化患者发生肌挛缩和关节功能障碍概率高于非皮下钙化患者;对于发病时肌酸激酶并未明显升高者,或许更易发生皮下钙化;早期积极应用激素、免疫抑制剂治疗可以减轻或预防后期钙质沉着的发生或发展.%Objective To analyze the clinical features of juvenile dermatomyositis (JDM) combined with soft-tissue calcification. Methods Forty-seven patients with JDM combined with soft-tissue calcification (soft-tissue calcification group) were retrospectively analyzed, and they were contrasted with 89 patients with non-calcification (non-calcification group). Results The rates of Gotton signe, muscle contracture and joint dysfunction in soft-tissue calcification group were significantly higher than those in non-calcification group:87.23% (41/47) vs. 43.82% (39/89) and 68.09% (32/47) vs. 21.35% (19/89), and there were statistical differences (P<0.05). The dosage of glucocorticoid (conversion of prednisone measuring more than 1.5 mg/kg), rate of using immunodepressant, level of creatine kinase in soft-tissue calcification group were significantly lower than those in non-calcification group:17.02%(8/47) vs. 68.54%(61/89), 25.53%(12/47) vs. 88.76%(79/89), (566.45±240.41) U/L vs. (1 680.12±656.50) U/L, and there were

  19. A case of dermatomyositis with rhabdomyolysis, rescued by intravenous immunoglobulin.

    Science.gov (United States)

    Mizoguchi, Fumitaka; Takada, Kazuki; Ishikawa, Kinya; Mizusawa, Hidehiro; Kohsaka, Hitoshi; Miyasaka, Nobuyuki

    2015-07-01

    We describe a case of severe dermatomyositis (DM) complicated by rhabdomyolysis, acute tubular necrosis, and hemophagocytosis. The case failed to respond to corticosteroids, but showed rapid and significant improvement after the addition of intravenous immunoglobulin (IVIG). While the prognosis of DM is poor when it is complicated by rhabdomyolysis, the early administration of IVIG has the potential to be the cornerstone of its management.

  20. Dermatomyositis revealing breast cancer: report of a case.

    Science.gov (United States)

    Lamquami, Safae; Errarhay, Sanae; Mamouni, Nisrine; Bouchikhi, Chahrazad; Banani, Abdelaziz

    2015-01-01

    Dermatomyositis (DM) is a rare connective corresponding to an inflammatory disease of skeletal muscles. Paraneoplastic origin must always be sought, primarily gynecological tumor in women, but the investigations are often made difficult by the fact that a primary tumor is often not detectable at the time of the cutaneous manifestations. This approach includes in addition to the monitoring report at regular intervals of 6 to 12 months for two years after diagnosis. We report a case of Dermatomyositis revealing breast cancer.

  1. Diffuse Muscular Pain, Skin Tightening, and Nodular Regenerative Hyperplasia Revealing Paraneoplastic Amyopathic Dermatomyositis due to Testicular Cancer

    OpenAIRE

    Sarah Norrenberg; Valérie Gangji; Véronique Del Marmol; Soyfoo, Muhammad S.

    2012-01-01

    Paraneoplastic dermatomyositis (DM) associated with testicular cancer is extremely rare. We report the case of a patient with skin tightening, polymyalgia, hypereosinophilia, and nodular regenerative hyperplasia revealing seminoma and associated paraneoplastic DM.

  2. Diffuse Muscular Pain, Skin Tightening, and Nodular Regenerative Hyperplasia Revealing Paraneoplastic Amyopathic Dermatomyositis due to Testicular Cancer.

    Science.gov (United States)

    Norrenberg, Sarah; Gangji, Valérie; Del Marmol, Véronique; Soyfoo, Muhammad S

    2012-01-01

    Paraneoplastic dermatomyositis (DM) associated with testicular cancer is extremely rare. We report the case of a patient with skin tightening, polymyalgia, hypereosinophilia, and nodular regenerative hyperplasia revealing seminoma and associated paraneoplastic DM.

  3. Diffuse Muscular Pain, Skin Tightening, and Nodular Regenerative Hyperplasia Revealing Paraneoplastic Amyopathic Dermatomyositis due to Testicular Cancer

    Directory of Open Access Journals (Sweden)

    Sarah Norrenberg

    2012-01-01

    Full Text Available Paraneoplastic dermatomyositis (DM associated with testicular cancer is extremely rare. We report the case of a patient with skin tightening, polymyalgia, hypereosinophilia, and nodular regenerative hyperplasia revealing seminoma and associated paraneoplastic DM.

  4. Dermatomyositis and HIV

    Directory of Open Access Journals (Sweden)

    Mamata Chand

    2016-12-01

    Full Text Available HIV has been linked to several autoimmune disorders since its emergence in the 1980s. By affecting different cells and pathways in the immune system, HIV induces the development of certain autoimmune diseases while prohibiting the emergence of others. Dermatomyositis has been rarely described in patients with HIV. We present a case of dermatomyositis in a patient with HIV and explore the pathogenesis of autoimmune disorders in HIV focusing on dermatomyositis.

  5. Successful resection of liver metastasis detected by exacerbation of skin symptom in a patient with dermatomyositis accompanied by rectal cancer: a case report and literature review

    OpenAIRE

    Ono, Kosuke; Shimomura, Manabu; Toyota,Kazuhiro; Kagimoto, Atsushi; Tsukiyama, Naofumi; Shishida, Masayuki; Oishi, Koichi; Miyamoto, Kazuaki; SHIBATA, Satoshi; Ikeda, Masahiro; Sadamoto, Seiji; Takahashi, Tadateru

    2017-01-01

    Background Dermatomyositis (DM) is a rare syndrome that belongs to the group of idiopathic inflammatory myopathies. The association between DM and malignancy is well recognized, and the severity of DM symptoms has been linked to the progression of metastatic disease. Case presentation We report the case of a 42-year-old man that was diagnosed with dermatomyositis (DM) and rectal cancer. Proctectomy was performed, and DM symptoms were resolved postoperatively. One year and 9 months after the s...

  6. A case of Amyopathic Dermatomyositis associated with Interstitial Pulmonary Disease.

    Science.gov (United States)

    Ugan, Y; Sahin, M; Dogru, A; Bayram, D; Ceyhan, A M; Tunc, S E

    2015-01-01

    Inflammatory myopathies are a heterogeneous group of diseases with unknown etiology characterized by inflammation of the skeletal muscles and proximal muscle weakness. Dermatomyositis (DM) is an idiopathic inflammatory myopathy with characteristic cutaneous findings such as heliotrope rash, Gottron's sign, Gottron's papules, shawl sign and machinist hand. Amyopathic dermatomyositis (ADM) is a rare but well-recognized clinical subtype of DM, constituting aproximately 10-20% of patients with this disease. It generally manifests only pathognomonic skin findings without clinical and laboratory evidence of muscle involvement. In this report, we present a rare case of ADM associated with interstitial pulmonary disease.

  7. Tamoxifen- Induced Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Bandyopadhyay Debabrata

    1997-01-01

    Full Text Available A 38 year old woman developed dermatomyositis after radical mastectomy and tamoxifen therapy for metastatic breast carcinoma. The possibility of the drug as the underlying cause of the connective tissue disease is discussed.

  8. Dermatomyositis panniculitis: a case report.

    Science.gov (United States)

    Carroll, Melissa; Mellick, Nick; Wagner, Godfrey

    2015-08-01

    Dermatomyositis-related panniculitis is a rare cutaneous manifestation of dermatomyositis. There are few reported cases in the medical literature. We present the case of a 60-year-old woman with a 2-year history of dermatomyositis and recent biopsy-confirmed panniculitis treated with prednisone, cyclophosphamide and i.v. immunoglobulin.

  9. Scleromyxedema concurrent with dermatomyositis and paraproteinemia: A case report

    OpenAIRE

    M N Starovoitova; O V Desinova; Guseva, N. G.

    2015-01-01

    The paper describes the case of a female patient who had at least three diseases: scleromyxedema, dermatomyositis/polymyositis (DM/PM), and paraproteinemia (monoclonal gammopathy). The concurrence of these diseases determines the atypism of their clinical and morphological patterns, as well as refractoriness to performed therapy. Scleromyxedema preceded DM followed by paraproteinemia, which does not rule out its presence in an earlier period when the patent was not carefully examined. The spe...

  10. Respiratory disorders in patients with polymyositis/dermatomyositis

    Directory of Open Access Journals (Sweden)

    Olga Alekseyevna Antelava

    2014-01-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are rare disorders characterized by inflammatory lesions in skeletal muscles. These diseases include polymyositis (PM, dermatomyositis (DM, and inclusion body myositis, which exhibit clinicoimmunological heterogeneity and give different response to therapy. The most frequent manifestation in PM/DM patients is respiratory system dysfunction. The developing respiratory disorders are varied and may outpace the presentation of muscle pathology.

  11. Dermatomyositis related to the relapse of cervical cancer

    Directory of Open Access Journals (Sweden)

    Marta Stawczyk-Macieja

    2015-04-01

    Full Text Available Dermatomyositis (DM is a rare syndrome which belongs to the group of idiopathic inflammatory myopathies (IIM. The diagnosis of DM in adults is an indication for diagnostic evaluation towards malignancy. The exacerbation of clinical symptoms or laboratory markers of DM may indicate the relapse of neoplasm, therefore close follow-up visits of patients are obligatory. We present the case of a woman with a two-month history of progressive muscle weakness, dysphagia and oedemo-erythematous skin lesions limited to the face and trunk. The patient was diagnosed with DM associated with the relapse of cervical cancer.

  12. Design of the muscles in motion study: a randomized controlled trial to evaluate the efficacy and feasibility of an individually tailored home-based exercise training program for children and adolescents with juvenile dermatomyositis

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    Habers Esther A

    2012-06-01

    Full Text Available Abstract Background Juvenile dermatomyositis (JDM is a rare, often chronic, systemic autoimmune disease of childhood, characterized by inflammation of the microvasculature of the skeletal muscle and skin. Prominent clinical features include significant exercise intolerance, muscle weakness, and fatigue. Despite pharmacological improvements, these clinical features continue to affect patients with JDM, even when the disease is in remission. Exercise training is increasingly utilized as a non-pharmacological intervention in the clinical management of (adult patients with chronic inflammatory conditions; however no randomized controlled trials (RCT have been performed in JDM. In the current study, the efficacy and feasibility of an exercise training program in patients with JDM will be examined. Methods/design Subjects (n = 30 will include 8–18 year olds diagnosed with JDM. The intervention consists of an individually tailored 12-weeks home-based exercise training program in which interval training on a treadmill is alternated with strength training during each session. The program is based on previous literature and designed with a defined frequency, intensity, time, and type of exercise (FITT principles. Primary outcome measures include aerobic exercise capacity, isometric muscle strength, and perception of fatigue. The study methodology has been conceived according to the standards of the CONSORT guidelines. The current study will be a multi-center (4 Dutch University Medical Centers RCT, with the control group also entering the training arm directly after completion of the initial protocol. Randomization is stratified according to age and gender. Discussion The current study will provide evidence on the efficacy and feasibility of an individually tailored 12-week home-based exercise training program in youth with JDM. Trial registration Medical Ethics Committee of the University Medical Center Utrecht, the Netherlands: 11–336

  13. Clinical presentation and evaluation of dermatomyositis

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    Umaima Marvi

    2012-01-01

    Full Text Available Dermatomyositis (DM is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50-70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron′s papules, Gottron′s sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, "mechanic′s hands", palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35-40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.

  14. Clinically Amyopathic Dermatomyositis Complicated by Pleural Effusion Case Report, Literature Review, and Proposed Mechanism.

    Science.gov (United States)

    Wu, Ying; Chhaya, Sheetal; Hurowitz, Bert; Ardiles, Thomas; Carlson, Richard

    2015-07-01

    Polymyositis-dermatomyositis (PM-DM) is a chronic inflammatory disorder that mainly involves muscles and skin. Clinically amyopathic dermatomyositis (CADM) is a unique subset of PM-DM with typical skin manifestations but little or no evidence of musculoskeletal involvement. Many cases of dermatomyositis and CADM are associated with internal malignancy, but pulmonary manifestations can also been seen; the most common of which is interstitial lung disease. Pleural effusion is a rare complication and may be difficult to differentiate from other causes, such as infections, heart failure, or malignancy. We report a patient with CADM complicated by rapidly progressive pleural effusions. Based on findings of this patient, as well as literature review, we suggest that the etiology of massive pleural effusion in this setting is most likely related to local immune pleuritis associated with underlying interstitial lung disease due to dermatomyositis. Optimal management should be individualized and may include immunosuppressive agents, as well as antimicrobials, and potentially other agents.

  15. Traditional Cardiovascular Risk Factors and Coronary Artery Calcification in Adults With Polymyositis and Dermatomyositis

    DEFF Research Database (Denmark)

    Diederichsen, Louise P; Diederichsen, Axel C P; Simonsen, Jane A

    2015-01-01

    OBJECTIVE: To determine the occurrence of traditional cardiovascular (CV) risk factors and coronary artery calcification (CAC) in adults with polymyositis (PM) or dermatomyositis (DM) compared to healthy controls and to assess the association between CV risk factors, PM/DM, and CAC score. METHODS...

  16. [Dermatomyositis-update].

    Science.gov (United States)

    Volc-Platzer, B

    2015-08-01

    Dermatomyositis is a rare idiopathic inflammatory myopathy that affects adults and children, mostly female. Hallmarks of the disease are myositis with necrosis, regeneration and perifascicular atrophy accompanied by a typical skin rash with heliotrope erythema, Gottron's sign, Gottron's papules and nail fold changes with splinter hemorrhage. Typical skin symptoms may appear 6 months up to 2 years before muscle involvement (amyopathic dermatomyositis). New myositis-specific antibodies may allow clinicoserologic correlations within a heterogeneous clinical spectrum. Autoantibody profiles, subtype of myositis, overlap with other collagen vascular disorders and/or malignancy (paraneoplastic dermatomyositis) as well as age of the patients all have a considerable impact on course and prognosis. Infections, drugs and tumors may trigger activation of T and B cells, plasmacytoid dendritic cells, overproduction of type I interferons and complement-mediated endothelial cell damage resulting in vasculopathy. UV radiation may also trigger dermatomyositis. Oral corticosteroids (1.5-2.0 mg/kg body weight/day) are the mainstay of treatment until improvement of muscle symptoms and/or normalization of muscle enzymes with subsequent slow tapering. Corticosteroids may be given as monotherapy or combined with steroid-sparing immunosuppressive agents' i.e. azathioprine, methotrexate, mycophenolate mofetil or high-dose intravenous immunoglobulins. Prognosis has improved considerably since use of high-dose corticosteroids, from 50 to 90% response rate. New therapies with biologicals (anti-CD20-, anti-TNFalpha-, anti-interferon antibodies) and Janus kinase inhibitors are currently being evaluated.

  17. Dermatomyositis and polymyositis: Clinical presentation, autoantibodies, and pathogenesis.

    Science.gov (United States)

    Mammen, Andrew L

    2010-01-01

    Dermatomyositis (DM) and polymyositis (PM) are autoimmune myopathies characterized clinically by proximal muscle weakness, muscle inflammation, extramuscular manifestations, and frequently, the presence of autoantibodies. Although there is some overlap, DM and PM are separate diseases with different pathophysiological mechanisms. Furthermore, unique clinical phenotypes are associated with each of the myositis-specific autoantibodies (MSAs) associated with these disorders. This review will focus on the clinical features, pathology, and immunogenetics of PM and DM with an emphasis on the importance of autoantibodies in defining unique phenotypes and, perhaps, as clues to help elucidate the mechanisms of disease.

  18. Duodenal perforation in childhood dermatomyositis

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    Magill, H.L.; Hixson, S.D.; Whitington, G.; Igarashi, M.; Hannissian, A.

    1984-01-01

    Perforation of the duodenum is an uncommon, but serious complication which may occur in children with dermatomyositis. In this disease vasculitis may involve the bowel to a variable extent and result in radiologic manifestations of intestinal injury ranging from benign pneumatosis intestinalis to signs of bowel perforation. We report two children with dermatomyositis in whom perforation of the second portion of the duodenum occurred. This serious complication should be considered in any child with dermatomyositis when extraluminal gas is suspected on abdominal radiographs.

  19. Cancer risk in dermatomyositis: a systematic review of the literature.

    Science.gov (United States)

    Di Rollo, D; Abeni, D; Tracanna, M; Capo, A; Amerio, P

    2014-10-01

    The association between idiopathic inflammatory myopathy (IIM) and cancer has been extensively studied in adults. Many epidemiological studies demonstrated this association, which appears stronger for dermatomyositis (DM) than for polymyositis (PM). The first case suggesting an association between cancer and DM was reported in 1916. At present the reported incidence of cancer association with DM varies widely, from less than 7% to over 30%. Many early evidences came from case reports, but this association was later confirmed in case-control as well as in population-based studies. Ovarian cancer or breast cancer in females and lung cancer in males are the main malignancies associated with DM. Given the frequency of the association of dermatomyositis with cancer, for cost-effectiveness reasons it might be important to develop simple and appropriate diagnostic tests for identification of patients with DM, who may be at higher risk of developing a malignancy. Clinicians should plan follow-up schedules to optimize both cancer detection and treatment, and thus to improve patient survival. Many different clinical and serological signs have been suggested as possible predictive factors for malignancy in dermatomyositis: age, increased erythrocyte sedimentation rate (ESR), presence of cutaneous leukocytoclastic vasculitis, cutaneous rash and skin lesions as cutaneous necrosis and periungueal erythemas, neoplastic markers or dysphagia. The results of the different studies are quite discordant. Therefore, we conducted a systematic review of the scientific literature to evaluate the level of the risk of cancer in patients with dermatomyositis and to explore whether certain patient characteristics may be linked to different levels of cancer risk.

  20. Polymyositis and dermatomyositis: Disease spectrum and classification

    Directory of Open Access Journals (Sweden)

    Siba P Raychaudhuri

    2012-01-01

    Full Text Available Muscle inflammation and weakness are the key features of idiopathic inflammatory myopathies (IIMs. In addition IIMs are frequently associated with cutaneous and pulmonary involvement. In clinical practice the three common inflammatory myopathies we come across are polymyositis (PM, dermatomyositis (DM and inclusion body myositis (IBM. The Bohan and Peter criteria combine clinical, laboratory, and pathologic features to define PM and DM. They did not recognize inclusion body myositis (IBM or other inflammatory myopathies, such as granulomatous and eosinophilic myositis. Thus the disease spectrum is wide and IIMs are a heterogeneous group of autoimmune disorders. To address these issues in this article we have discussed the currently developing newer classifications of IIMs.

  1. Polymyositis and dermatomyositis.

    Science.gov (United States)

    Dalakas, Marinos C; Hohlfeld, Reinhard

    2003-09-20

    The inflammatory myopathies, commonly described as idiopathic, are the largest group of acquired and potentially treatable myopathies. On the basis of unique clinical, histopathological, immunological, and demographic features, they can be differentiated into three major and distinct subsets: dermatomyositis, polymyositis, and inclusion-body myositis. Use of new diagnostic criteria is essential to discriminate between them and to exclude other disorders. Dermatomyositis is a microangiopathy affecting skin and muscle; activation and deposition of complement causes lysis of endomysial capillaries and muscle ischaemia. In polymyositis and inclusion-body myositis, clonally expanded CD8-positive cytotoxic T cells invade muscle fibres that express MHC class I antigens, which leads to fibre necrosis via the perforin pathway. In inclusion-body myositis, vacuolar formation with amyloid deposits coexists with the immunological features. The causative autoantigen has not yet been identified. Upregulated vascular-cell adhesion molecule, intercellular adhesion molecule, chemokines, and their receptors promote T-cell transgression, and various cytokines increase the immunopathological process. Early initiation of therapy is essential, since both polymyositis and dermatomyositis respond to immunotherapeutic agents. New immunomodulatory agents currently being tested in controlled trials may prove promising for difficult cases.

  2. Electrocardiographic changes in dermatomyositis and polymyositis

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    Leticia Miranda Alle Deveza

    2016-04-01

    Full Text Available ABSTRACT Introduction: Cardiac involvement is frequent in inflammatory myopathies. Electrocardiogram (ECG may show evidence of this involvement and its changes should be well-known and described. Objectives: Due to the lack of studies in the literature, we conducted an analysis of the ECG findings in patients with dermatomyositis (DM and polymyositis (PM, comparing them with a control group. Methods: This cross-sectional study compared the ECG of 86 individuals with no rheumatic disorders (controls with 112 patients (78 DM and 34 PM, during 2010 to 2013. The ECG findings between DM and PM were also compared. Results: Demographic characteristics, comorbidities and ECG abnormalities were similar between controls and patients (p > 0.05, except for a higher frequency of left ventricular hypertrophy (LVH in patients (10.7% vs. 1.2%, p = 0.008. Demographic characteristics, comorbidities, clinical and laboratory manifestations, were also similar between the groups PM and DM, except for the presence of cutaneous lesions only in DM. One third of the patients had ECG abnormalities, which were more prevalent in PM than DM (50% vs. 24.4%, p = 0.008. LVH, left atrial enlargement, rhythm and conduction abnormalities were more frequent in PM than DM (p < 0.05 for all, especially the left anterior fascicular block. Conclusions: We showed distinct ECG changes between DM and PM and a higher frequency of LVH in patients compared to controls. Investigation of cardiac involvement should be considered even in asymptomatic patients, especially PM. Further studies are necessary in order to determine the correlation of ECG findings with other complementary tests, clinical manifestations, disease activity and progression to other cardiac diseases.

  3. Recurrent pneumatosis intestinalis in a patient with dermatomyositis

    OpenAIRE

    2013-01-01

    A 51-year-old woman with dermatomyositis (DM) on chronic immunosuppressive therapy was hospitalised for evaluation of haematuria. Surprisingly, abdominal imaging demonstrated pneumoperitoneum and pneumatosis intestinalis (PI). Her abdominal examination and white cell count were normal, but she subsequently developed nausea and fever. Owing to concern for perforation, a hemicolectomy was performed. Pathology revealed PI without inflammatory, ischaemic or neoplastic features, and she recovered ...

  4. Muscle biopsy findings predictive of malignancy in rare infiltrative dermatomyositis.

    Science.gov (United States)

    Uchino, Makoto; Yamashita, Satoshi; Uchino, Katsuhisa; Mori, Akira; Hara, Akio; Suga, Tomohiro; Hirahara, Tomoo; Koide, Tatsuya; Kimura, En; Yamashita, Taro; Ueda, Akihiko; Kurisaki, Ryoichi; Suzuki, Junko; Honda, Shoji; Maeda, Yasushi; Hirano, Teruyuki; Ando, Yukio

    2013-05-01

    The characteristic pathological muscular findings of polymyositis (PM) and dermatomyositis (DM) have been shown to reflect their different pathogeneses. Here, we characterized the muscle biopsy findings of PM and DM patients with or without malignancy. We evaluated the muscle biopsy findings of 215 consecutive PM and DM patients admitted to our hospital between 1970 and 2009. Pathology of the lesion biopsy sections was classified into 3 types: endomysial infiltration-type, perivascular infiltration-type, and rare-infiltrative-type. There was no difference between the muscle pathology of PM patients with and without malignancy. However, the incidence of rare-infiltrative type muscle pathology in DM patients with malignancy was significantly higher than in those without such tumors (p=0.0345). The incidence of rare-infiltrative type muscle pathology may be a predictive marker of DM with malignancy. Copyright © 2012 Elsevier B.V. All rights reserved.

  5. Dermatomyositis as the first manifestation of gallbladder adenocarcinoma: case report and literature overview.

    Science.gov (United States)

    Jurcic, Petra

    2015-03-27

    Dermatomyositis (DM) is characterized by pathognomic cutaneous manifestations (heliotrope rash, periorbital edema, Gottron's papules) and proximal muscle weakness. In this paper, I will present the case of a 48-year-old female patient whose dermatomyositis was initially diagnosed as vasculitis. Following the patient's inadequate response to corticosteroid treatment, clinical and radiologic examinations were performed, showing inoperable gallbladder adenocarcinoma. Although initial chemotherapy led to regression, the dermatomyositis developed an independent course with new pathological changes leading to the progression of the disease. I will also present an overview of case reports in English published so far. Gallbladder carcinoma should be added to the list of malignancies with dermatomyositis and has to be excluded by relevant investigation in women.

  6. High levels of serum hyaluronic acid in adults with dermatomyositis

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    Alana Ausciutti Victorino

    2015-04-01

    Full Text Available Background / objectives. Hyaluronic acid (HA is rarely described in dermatomyositis (DM. Thus, we determined any clinical association of serum levels of hyaluronic acid (HA in patients with dermatomyositis (DM. Materials and Methods. This cross-sectional single-center analysis 75 DM and 75 healthy individuals, during the period from January 2012 to July 2013. An anti-HA antibody assay was performed using specific ELISA/EIA kits, according to the manufacturer’s protocol. Results. The patients with DM and control subjects had comparable demographic distributions (p>0.05. The median time duration between disease diagnosis and initial symptoms was 6.0 [3.0-12.0] months, with a median DM disease duration of 4.0 [1.0-7.0] years. The median level of serum HA was significantly increased in patients with DM compared to the control group [329.0 (80.0-958.0 vs. 133.0 (30.0-262.0 ng/mL, respectively; p0.05. Serum HA also did not correlate with gender, ethnicity, auto-antibodies or drug use (p>0.05, but did correlate with cutaneous features, such as photosensitivity (p=0.001, “shawl” sign (p=0.018, “V-neck” sign (p=0.005 and cuticular hypertrophy (p=0.014. Conclusions. A high level of serum AH was observed in DM compared to healthy individuals. In DM, HA did not correlate to demographic, auto-antibodies and therapy parameters. However, HA correlated specifically with some cutaneous features, suggesting that this glycosaminoglycan could be involved in modulating cutaneous inflammation in this population. More studies are necessary to understand the correlation between AH and patients with DM.

  7. Rare association of chronic lymphocytic thyroiditis with dermatomyositis.

    Science.gov (United States)

    Charalabopoulos, Konstantinos; Mittari, Evanthia; Peschos, Dimitrios; Golias, Christos; Charalabopoulos, Alexander; Tsanou, Eleni; Batistatou, Anna

    2006-05-01

    Autoimmune thyroid disease (AITD) has been associated with other autoimmune diseases such as chronic urticaria, insulin-dependent diabetes mellitus, Sjøgren's syndrome, inflammatory bowel disease, rheumatoid arthritis, multiple sclerosis, systemic lupus erythematosus, psoriasis, ankylosing spondylitis, and dermatomyositis (DM). AITD is a common disorder affecting primarily women, and both genetic and environmental factors are included in its pathogenesis. DM is considered an autoimmune disease of the muscles and skin. Although AITD is the most common cause of hypothyroidism, to the best of our knowledge, only three cases of DM and AITD in the same patient have been reported in the last 40 years. We consider that both are developed from the same autoimmune background. Herein, we present a case of a 30-year-old man with a 4-year history of AITD who was diagnosed as suffering as well from DM.

  8. Diagnosis of dermatomyositis and polymyositis: a study of 102 cases

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    SCOLA ROSANA HERMINIA

    2000-01-01

    Full Text Available Patients with dermatomyositis (DM or polymyositis (PM were studied retrospectively. The patients were divided into four groups: definite PM 24, probable PM 19, definite DM 34 and mild-early DM 25 cases. PM patients complained more often proximal muscle weakness [p <0.01]. DM patients complained more arthralgia [p <0.05], dysphagia [p <0.03] and weight loss [p <0.04]. Five patients had a malignant neoplasm and 9 had other connective-tissue disease. DM presented higher ESR than PM [p <0.002]. PM presented more significant increase in creatine kinase (CK [p <0.02] and in alanine aminotransferase (ALT [p <0.001] levels. Electromyography showed myopathic pattern in 76%. Muscle biopsy was the definitive test. Perifascicular atrophy was more frequent in definite DM than in mild-early DM group [p <0.03]. CONCLUSION: A small association with connective-tissue diseases and neoplasms was found. DM and PM are clinically different. DM presents systemic involvement affecting the skin, developing more severe arthralgia, dysphagia and weight loss and presenting higher values of ESR. PM presents a restricted and more significant involvement of muscles generating more weakness complaints and higher levels of serum muscle enzymes.

  9. Cutaneous manifestations of dermatomyositis and their management.

    Science.gov (United States)

    Callen, Jeffrey P

    2010-06-01

    Dermatomyositis is a condition with pathognomonic and characteristic cutaneous lesions. This article describes the skin manifestations observed in patients with dermatomyositis, their differential diagnosis, their relationship to internal disease (particularly malignancy), and their management.

  10. Cardiac Abnormalities in Adult Patients With Polymyositis or Dermatomyositis as Assessed by Noninvasive Modalities

    DEFF Research Database (Denmark)

    Diederichsen, L P; Simonsen, J A; Diederichsen, A C

    2016-01-01

    OBJECTIVE: Cardiac events are a major cause of death in patients with idiopathic inflammatory myopathies. The study objective was in a controlled setting to describe cardiac abnormalities by noninvasive methods in a cohort of patients with polymyositis (PM) or dermatomyositis (DM) and to identify...

  11. Optimal management of interstitial lung disease associated with dermatomyositis/polymyositis: lessons from the Japanese experience

    OpenAIRE

    Kurasawa K; Arai S

    2014-01-01

    Kazuhiro Kurasawa,1,2 Satoko Arai2 1Center of Rheumatic Diseases, Dokkyo Medical University, Mibu, Tochigi, Japan; 2Department of Clinical Immunology, Dokkyo Medical University, Mibu, Tochigi, Japan Abstract: Interstitial lung disease (ILD) is a serious complication in dermatomyositis (DM) and polymyositis (PM). In Japan, patients with DM/PM develop acute life-threatening ILD with high frequency. Physicians in Japan have shown the following: refractory acute/subacute (A/S)-ILD is not a rare ...

  12. MR imaging in amyopathic dermatomyositis

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    Lam, W.W.M.; Chan, Y.L.; So, N.M.C.; Metreweli, C. [Chinese Univ. of Hong Kong, Shatin (China). Dept. of Diagnostic Radiology and Organ Imaging; Chan, H.; Fung, J.W.K. [Chinese Univ. of Hong Kong, Shatin (China). Dept. of Medicine

    1999-01-01

    Purpose: Amyopathic dermatomyositis is a distinct clinical entity with cutaneous involvement but no myopathy. We conducted a prospective study to investigate the role of MR imaging in these patients. Material and Methods: Out of 40 Chinese patients presenting with dermatomyositis, based on clinical assessment and normal serum muscle enzymes 10 were diagnosed as having amyopathic dermatomyositis. These 10 patients underwent MR imaging for evaluation of any subclinical muscle involvement. Results: Three patients demonstrated abnormal signal intensity in muscles on both T2- and fat suppression sequences. Thus, one-third of patients with dermatomyositis and clinically normal muscles may have detectable muscle inflammation on MR images, indicating that MR has a potential role for locating the relevant biopsy site and for longitudinal follow up. Six of the 10 patients had malignant disease diagnosed before or after diagnosis of the cutaneous manifestation. Nasopharyngeal carcinoma was the most common malignant disease in this group of patients. Conclusion: MR imaging is recommended for demonstrating subclinical muscle involvement in patients with the clinical diagnosis of amyopathic dermatomyosits. We also recommend screening for malignancy, particularly nasopharyngeal carcinoma, in Southern Chinese patients with dermatomyositis. (orig.)

  13. Polymyositis and dermatomyositis as a risk of developing cancer.

    Science.gov (United States)

    Jakubaszek, Michał; Kwiatkowska, Brygida; Maślińska, Maria

    2015-01-01

    Polymyositis (PM) is an idiopathic inflammatory myopathy that affects striated muscles. Dermatomyositis (DM) is an idiopathic inflammatory myopathy with presence of skin symptoms. Both are characterized by acute or subacute onset, symmetrical proximal muscle weakness, the presence of mononuclear cell infiltrates of the muscles and increased activity of muscle enzymes. The treatment still remains glucocorticoids and disease-modifying drugs. Symptoms of PM/DM can be a signal of developing cancer. Known risk factors for cancer in patients with PM/DM are older age, male gender, dysphagia, skin necrosis, cutaneous vasculitis, rapid onset of the disease, elevated creatinine kinase (CK) and C reactive protein (CRP), and an increase in the erythrocyte sedimentation rate (ESR). Recently three new myositis-specific autoantibodies (MSA) predicting the risk of cancer have been discovered: melanoma differentiation-associated protein 5 (anti-MDA-5), transcription intermediary factor 1γ (TIF-1γ), and nuclear matrix protein NXP-2.

  14. Recurrent pneumatosis intestinalis in a patient with dermatomyositis.

    Science.gov (United States)

    Zarbalian, Yousef; von Rosenvinge, Erik C; Twadell, William; Mikdashi, Jamal

    2013-08-23

    A 51-year-old woman with dermatomyositis (DM) on chronic immunosuppressive therapy was hospitalised for evaluation of haematuria. Surprisingly, abdominal imaging demonstrated pneumoperitoneum and pneumatosis intestinalis (PI). Her abdominal examination and white cell count were normal, but she subsequently developed nausea and fever. Owing to concern for perforation, a hemicolectomy was performed. Pathology revealed PI without inflammatory, ischaemic or neoplastic features, and she recovered uneventfully. Her immunosuppressive therapy was discontinued. Six months later, a follow-up CT of the abdomen revealed recurrence of PI. As she was asymptomatic, she was managed conservatively with resolution of PI on subsequent imaging. PI is characterised by the presence of gas within the wall of the intestine. Its aetiology is often unclear but this case highlights the association between PI and both immunosuppressive therapy and DM. A review of PI in patients with DM suggests that clinically stable patients may be observed, while avoiding surgical intervention.

  15. Amyopathic dermatomyositis: definitions, diagnosis, and management.

    Science.gov (United States)

    Bailey, Elizabeth E; Fiorentino, David F

    2014-12-01

    Amyopathic dermatomyositis can be a challenging diagnosis because patients lack traditional muscle findings. "Clinically amyopathic" dermatomyositis (CADM) accounts for the presence of subclinical muscle disease in some of these patients. These patients represent a substantial minority of dermatomyositis cases and have similar co-morbidities to "classic" dermatomyositis patients, including interstitial lung disease and malignancy. Clinically amyopathic dermatomyositis patients should not be considered as a distinct clinical entity from "classic" dermatomyositis, as they share antibody sub-types and associated co-morbidities, likely representing clinical spectrum of a common disease. It is essential for the clinician to be familiar with the clinical presentation of clinically amyopathic dermatomyositis, in order to facilitate early, accurate diagnosis and appropriate clinical management.

  16. {sup 18F} FDG PET Demonstration of Cancer Recurrence Presenting as Dermatomyositis in a Rare Case of Primary Pleural Lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang Mi; Kim, Yu Kyeong; Lee, Jong Jin; Lee, Yoon Jong; Kim, Hye Ryung; Choe, Gheeyoung [Seoul National Univ. College of Medicine/Bundang Hospital, Seoul (Korea, Republic of); Lee, Jeong Won [Jeju National Univ. Hospital, Jeju (Korea, Republic of)

    2011-03-15

    Dermatomyositis (DM) or polymyositis (PM) are possibly considered to have an association with malignancies. We describe a case of dermatomyositis in which {sup 18F} fluorodeoxyglucose (FDG)positron emission tomography (PET) was able to detect cancer recurrence earlier than any other modality in a patient with a history of primary pleural lymphoma, a very rare condition of malignancy. Further, a typical finding of dermatomyositis is diffuse hypermetabolism in the bilateral proximal shoulder and pelvic girdle areas was shown on {sup 18F} FDG PET, which can implicate the inflammatory process in the skeletal muscle in dermatomyosistis. This case well illustrates the characteristic {sup 18F} FDG findings of dermatomyositis as well as a capability of {sup 18F} FDG PET in detection of recurrence of lymphoma, even in a rare condition.

  17. ANTISYNTHETASE SYNDROME IS THE MOST SEVERE SUBTYPE OF POLYMYOSITIS/DERMATOMYOSITIS:DESCRIPTION OF CASES

    Directory of Open Access Journals (Sweden)

    Olga Alekseyevna Antelava

    2009-01-01

    Full Text Available Polymyositis (PM and dermatomyositis (DM are autoimmune skeletal muscle diseases of unknown etiology, which are referred to as systemic connective tissue diseases and united under the common term Tidiopathic inflammatory myopathiesy. The most severe subtype of PM/DM is the antisynthetase syndrome that is characterized by a certain sympathocomplex, including interstitial lung lesion that is one of the most common visceral changes. Of interest are the specific features of the antisynthetase syndrome, its onset, the course and pulmonary manifestations of fibrosing alveolitis, unlike the classical course of PM/DM. Two clinical cases of the antisynthetase syndrome are given.

  18. Treatment of early and refractory dermatomyositis with infliximab: a report of two cases.

    Science.gov (United States)

    Dold, Sylvia; Justiniano, Maria E; Marquez, Javier; Espinoza, Luis R

    2007-07-01

    The idiopathic inflammatory myopathies embody the largest group of acquired and potentially treatable causes of skeletal muscle weakness. The three major groups of this disorder are polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. Corticosteroids continue to be the mainstay of initial treatment in the majority of cases of PM/DM. The treatment of refractory disease can be challenging despite the utilization of the medications currently available. We report two patients with refractory DM who were treated with infliximab. We describe their presentation, clinical course, treatment, and outcomes.

  19. Consenso em reumatologia pediátrica: parte II - definição de melhora clínica para o lúpus eritematoso sistêmico e dermatomiosite juvenil Consensus in pediatric rheumatology: part II - definition of clinical improvement in systemic lupus erythematosus and juvenile dermatomyositis

    Directory of Open Access Journals (Sweden)

    Claudia Machado

    2005-02-01

    development of the core response variables for disease activity in Juvenile Systemic Lupus Erythematosus (JSLE and Juvenile Dermatomyositis (JDM as well as the preliminary definition of improvement for both. METHODS AND RESULTS: The core response domains and variables for JSLE were: 1 disease activity global assessment by the physician = 10 cm VAS; 2 health related to quality of life = CHQ-PF50 physical score; 3 disease activity global assessment by parents/patients = 10 cm VAS; 4 kidney assessment = 24 h proteinuria; 5 global disease activity assessment by a specific and valid tool (SLEDAI or ECLAM. The preliminary definition of improvement for JSLE was: 2 of the 5 measures with at least 50% of improvement and no more than 1 worse by more than 30%, which cannot be the 24 h proteinuria. The core response domains and variables for JDM were: 1 disease activity global assessment by the physician = 10 cm VAS; 2 muscle strength assessment by a specific tool (CMAS - Childhood Myositis Assessment Scale = 0-52; 3 functional assessment by a valid tool (CHAQ; 4 disease activity global assessment by parents/patients = 10 cm VAS; 5 health related to quality of life (CHQ-PF50 physical score; 6 global disease activity assessment by specific and valid tool (DAS - Disease Activity Score. The preliminary definition of improvement for JDM was: 3 of the 6 measures with at least 20% of improvement and no more than 1 worse by more than 30%, which cannot be the muscle strength by CMAS. CONCLUSIONS: All the variables were tested and have shown good statistical responsiveness and discriminative validity to be used in therapeutic trials.

  20. [Report of an amyopathic dermatomyositis clinical case].

    Science.gov (United States)

    Monteiro, Paulo; Duarte, Cátia; Salvador, Maria João; Malcata, Armando

    2009-01-01

    The authors report a clinical case of a 41-years-old man with astenia and myalgias maintained for many years without an accurate diagnosis. Amyopathic dermatomyositis was diagnosed based on the presence of typical cutaneous lesions and the absence of myositis. Subsequent evidence of subclinical myositis allowed the diagnosis of hypomyophatic dermatomyositis. Finally, the diagnosis of classical dermatomyositis could be made when the patient presented elevated levels of muscle enzimes. There was a good clinical response to imunossupressor therapy and the patient remains asymptomatic. Differences in the clinical management of amyopathic, hypomyopathic and classic dermatomyositis are discussed.

  1. Effect of early treatment in polymyositis and dermatomyositis

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    Naji Peyman

    2010-01-01

    Full Text Available Background : Idiopathic inflammatory myopathies, dermatomyositis (DM and polymyositis (PM are rare but are potentially treatable. Aim : To compare the effect of early and late treatment in patients with PM and DM. Materials and Methods : The study included all the adult patients with definite diagnosis of PM or DM treated for at least 12 months. The patients were divided into two groups: Early Group - treatment within three months and Late Group - treatment after three months. The number of patients with positive therapeutic response, remission in less than one year and the mean time elapsed for reaching the remission were assessed and compared between the two groups. Chi-square test, Fisher′s exact test, t-test and Pearson correlation test were used for data analysis. Results : The analysis included 65 patients, 42 with DM and 23 with PM. Late Group included 24 patients (seven PM and 17 DM, while Early Group included 41 patients (16 PM and 25 DM. Positive therapeutic response, remission rate within one year was higher in Early Group (80% vs. 46%, P: 0.004. The mean time needed to achieve remission was much less with early treatment (5.5 vs. 11.9 months, P: 0.003. The relapse rate was also lower in Early Group (5% vs. 25%, P < 0.02. The comparison of treatment outcomes showed the same results in both PM and DM, but it was statistically significant in patients with DM. Conclusions : Early treatment in patients with PM and DM is associated with higher remission rates, shorter treatment period and low complication rates.

  2. Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil*

    Science.gov (United States)

    Ortigosa, Luciena Cegatto Martins; dos Reis, Vitor Manuel Silva

    2014-01-01

    BACKGROUND Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small. PMID:25184910

  3. Juvenile idiopatiske inflammatoriske myopatier

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    Helga Sanner

    2009-10-01

    Full Text Available Juvenile idiopathic inflammatory myopathies (JIIM is a group of rare autoimmune systemic diseases in children and adolescents, characterized by chronic skeletal muscle inflammation. Unlike in adults, dermatomyositis (JDM is by far the most common of the idiopathic inflammatory myopathies in children and adolescents. The hallmark of JDM is calcinosis, lipodystrophy and vasculitis, findings that differs the juvenile form of dermatomyosits from the adult form. JDM is still diagnosed and classified by Bohan and Peter’s criteria from 1975. There are limited data on long time outcome of this disease

  4. [The heart in dermatomyositis and polymyositis ].

    Science.gov (United States)

    Badui, E; Mintz, G; Robles, E

    1986-01-01

    We studied eleven consecutive patients: eight with Dermatomyositis (DM) and three with Polymyositis (PM) from the cardiological point of view through non invasive methods. Nine patients (82%) had some kind of cardiopulmonary complications as shown by any of the used methods. eight (73%) referred some kind of cardiopulmonary symptoms, mainly dyspnea; Physical examination; in seven (64%) was abnormal, detecting increased second pulmonary sound in four (36%), findings of mitral valve prolapse (MVP) in two (18%) and in two (18%) S3 gallop; Electrocardiogram: in seven (64%) was abnormal; six (55%) had some kind of heart enlargement corresponding four (36%) to right atrial or ventricular hypertrophy (RAH & RVH) and two (18%) to left ventricular hypertrophy (LVH), three (27%) had incomplete or complete right bundle branch block, one (9%) had bifascicular block and one (9%) left anterior hemiblock. Two (18%) had sinus tachycardia and two (18%) atrial premature contractions; d) chest ray: six (55%) were abnormal, among them, three (27%) had pulmonary fibrosis, three (27%) had RAH and/or RVH, two (18%) had LVH and one (9%) pericardial effusion; e) Echocardiogram: was abnormal in eight (73%), corresponding three (27%) to RVH, three to MVP which has been considered rare, in two (18%) congestive cardiomyopathy, in two (18%) pericardial effusion and in one (9%) type "A" paradoxical septal movement.

  5. C5b-9 deposits on endomysial capillaries in non-dermatomyositis cases.

    Science.gov (United States)

    Braczynski, Anne K; Harter, Patrick N; Zeiner, Pia S; Drott, Ulrich; Tews, Dominique-Suzanne; Preusse, Corinna; Penski, Cornelia; Dunst, Maika; Weis, Joachim; Stenzel, Werner; Mittelbronn, Michel

    2016-01-01

    Deposits of the terminal-membrane-attack-complex (MAC) C5b-9 on perfascicular endomysial capillaries are generally regarded as diagnostic hallmark of dermatomyositis (DM). Although the pathophysiology is not clear, C5b-9 deposits on capillaries seem to be associated with microinfarctions and vascular damage. Here, we report on a series of 19 patients presenting with C5b-9 accumulation on endomysial capillaries in the absence of features for DM. To decipher differences in the capillary C5b-9 accumulation pattern between DM and non-DM cases, we assessed the extent of endomysial capillary C5b-9 deposits related to capillary density and extent of myofiber necrosis by immunohistochemistry in 12 DM and 8 control patients. We found similar numbers of C5b-9-positive myofibers in both DM and non-DM C5b-9(+) cases. The distribution pattern differed as DM cases showed significantly more perifascicular capillary C5b-9 deposits as compared to non-DM cases, which presented stronger endomysial capillary C5b-9 deposits in a diffuse pattern. While total capillary density was not differing, DM patients displayed significantly more C5b-9(+) necrotic fibers as compared to non-DM C5b-9(+). In summary, endomysial capillary C5b-9 deposits are present in a variety of non-DM cases, however with differing distribution pattern. In conclusion, capillary C5b-9(+) deposits should be assessed critically, taking into consideration the distribution pattern.

  6. Optimal management of interstitial lung disease associated with dermatomyositis/polymyositis: lessons from the Japanese experience

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    Kurasawa K

    2014-11-01

    Full Text Available Kazuhiro Kurasawa,1,2 Satoko Arai2 1Center of Rheumatic Diseases, Dokkyo Medical University, Mibu, Tochigi, Japan; 2Department of Clinical Immunology, Dokkyo Medical University, Mibu, Tochigi, Japan Abstract: Interstitial lung disease (ILD is a serious complication in dermatomyositis (DM and polymyositis (PM. In Japan, patients with DM/PM develop acute life-threatening ILD with high frequency. Physicians in Japan have shown the following: refractory acute/subacute (A/S-ILD is not a rare complication in DM and amyopathic DM (ADM; anti-anti-melanoma differentiation-associated gene 5 (anti-MDA5 antibody (Ab is closely related to A/S-ILD with poor outcomes in DM/ADM; and poor prognostic factors in A/S-ILD in DM/PM are ADM, DM with low creatine kinase elevation, positivity for anti-MDA5 Ab, serum ferritin elevation, and consolidation with ground-glass opacities on high-resolution computed tomography. There are subtypes in DM/PM-ILD: refractory DM/ADM A/S-ILD positive for anti-MDA5 Ab with poor prognosis; DM A/S-ILD with glucocorticoid (GC resistance; PM A/S-ILD with GC sensitivity; chronic ILD positive for anti-aminoacyl-tRNA-synthetases (anti-ARS Abs with GC responsiveness; and C-ILD negative for anti-ARS Abs. For patients with A/S-ILD with poor prognosis, initial combination therapy with cyclosporine and cyclophosphamide in addition to GC has been developed, which rescues 50%–80% of the patients, although elucidation of the efficacy of the combination therapy is required. A/S-ILD with potentially fatal outcomes is found worldwide, not only in Japan. Clinicians caring for patients with DM/PM should be cautious when dealing with A/S-ILD and treat the patients based on clinical subtypes. Keywords: interstitial lung disease, dermatomyositis, polymyositis, management, cyclosporine, cyclophosphamide

  7. A rare case of dermatomyositis revealed during pregnancy with good outcome.

    Science.gov (United States)

    Awatef, Kelati; Salim, Gallouj; Zahra, Mernissi Fatima

    2016-01-01

    There are only few case reports in the literature documenting outcome of pregnancy in patients with DM in contrast with those of other connective tissue diseases especially when dermatomyositis is revealed during pregnancy, most of the publications reported a poor prognosis for both the mother and the fetus, yet, in our case we confirmed the results of the rare recent cases that have tended to show a good outcome, after the treatment with glucocorticoid and immunosuppressant therapy.

  8. Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis

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    Sudhir Babu Karri

    2015-01-01

    Full Text Available Aims: To study the histological features on muscle biopsy and correlate them with clinical features, other laboratory data in adult patients to make a diagnosis of dermatomyositis (DM, applying the European Neuromuscular center (ENMC criteria. Materials and Methods: Adult patients who fulfilled clinical, laboratory, and muscle biopsy findings according to ENMC criteria for DM during the period 2010-2013 were included in the study. Cryostat sections of muscle biopsy were reviewed with emphasis on Perifascicular atrophy (PFA, perivascular/endomysial inflammation. Muscular dystrophies and metabolic myopathies were excluded by appropriate immunohistochemistry and special stains. Results: The diagnosis of adult DM was made in 45 patients out of 170 clinically suspected idiopathic inflammatory myopathies. These included 33 definite, 4 probable, 7 possible sine dermatitis, and 1 amyopathic DM. All patients with definite DM had typical rash and proximal muscle weakness and muscle biopsy showed PFA with or without inflammation. Thirteen patients had quadriparesis, neck muscle weakness, dysphagia/dysphonia at presentation. Patients with probable DM had rash and showed perivascular/endomysial inflammation with no PFA. Possible DM sine dermatitis showed PFA with perivascular/endomysial infiltrates. One patient of amyopathic DM had typical heliotrope rash and characteristic skin biopsy. Conclusions: Histological features are important for the diagnosis of DM. Relying on PFA for diagnosis of definite DM underestimates the true frequency of DM.

  9. Whole-body /sup 67/Ga scintigraphy in dermatomyositis

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    Hiraki, Yoshio; Okazaki, Yoshio; Murakami, Kiminori; Inoue, Nobuhiro; Noriyasu, Toshiaki; Takeda, Yoshihiro; Morimoto, Setsuo; Aono, Kaname

    1987-10-01

    The presence or absence of abnormal accumulation of gallium-67 in soft tissues was studied in 11 patients undergoing /sup 67/Ga scintigraphy out of 25 patients with dermatomyositis and polymyositis (DM-PM) who had visited our hospital during the period between July 1981 and March 1987 and met the diagnostic criteria of muscle biopsy, etc. A definite image of abnormal accumulation was obtained by /sup 67/Ga scintigraphy in 3 of the patients. Although the positive site tended to be in agreement with the site of muscular symptoms in the DM-PM active stage, the accumulation was not necessarily correlated with the variations in creatine phosphokinase. From these results, it seems necessary to keep in mind the possibility that gallium-67 may also accumulate abnormally in the soft tissue lesion owing to the pathogenic process specific to DM-PM when /sup 67/Ga scintigraphy is undertaken for the purpose of screening, etc., for complication by a malignant tumor in DM-PM patients

  10. Association of HLA-DRB1 Alleles with Polymyositis/ Dermatomyositis in Northern Chinese Hans

    Institute of Scientific and Technical Information of China (English)

    翟宁; 张庆瑞; 韩秀萍; 宋芳吉

    2002-01-01

    The HLA system is a highly polymorphic antigen and genetic system in human,and different population has different distribution of HLA alleles. Polymyositis/dermatomyositis (PM/DM) are idiopathic inflammatory myopathies that are characterized clinically by proximal muscle weakness, elevations of serum muscle enzymes, especially creatine kinase (CK), and typical abnormal findings on electromyography. Significant association has been reported between PM/DM and HLA-DR3/DRB1 0301, DRB8/DRB1 08, DR6 and DR2.

  11. Sequential change of high-resolution CT findings of interstitial lung disease in polymyositis and dermatomyositis

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    Tsukada, Hiroshi; Furuizumi, Naoya; Akita, Shinichi; Oda, Junichi; Sakai, Kunio; Suzuki, Eiichi; Emura, Iwao (Niigata Univ. (Japan). School of Medicine)

    1994-01-01

    Sequential changes of interstitial lung disease in fourteen patients of polymyositis/dermatomyositis (PM/DM) were followed up by high-resolution CT (HRCT). Most frequent CT findings were intense lung attenuation (ILA) with volume loss and slightly increased lung attenuation (SILA). Open lung biopsy was performed in a case with ILA shadow which revealed so-called usual interstitial pneumonia (UIP). Most intense ILA and SILA shadows resolved after steroid therapy. Some of ILA, however, reappeared and accompanied more prominent volume loss findings than before treatment. We think HRCT findings of interstitial lung disease in PM/DM may indicate prognosis of these diagnoses to some degree. (author).

  12. Serum Interleukin-6 Expression Level and Its Clinical Significance in Patients with Dermatomyositis

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    Min Yang

    2013-01-01

    Full Text Available Objective. To analyze serum interleukin-6 (IL-6 expression level and its clinical significance in patients with dermatomyositis. Methods. Blood samples from 23 adult patients with dermatomyositis (DM, 22 with systemic lupus erythematosus (SLE, 22 with rheumatoid arthritis (RA, 16 with Sjögren's syndrome (SS, and 20 healthy controls were collected. The IL-6 concentration was detected by chemiluminescence immunoassay. Correlations between IL-6 expression levels and clinical features or laboratory findings in patients with DM were investigated. Results. IL-6 expression level of DM patients was significantly higher than that of normal controls, significantly lower than that of RA patients, and slightly lower than that of SLE or SS patients with no significant differences. The incidence of fever was significantly higher in the IL-6 elevated group. Serum ferritin (SF and C-reactive protein (CRP were positively correlated with IL-6. Conclusions. IL-6 plays a less important role in DM than in RA. IL-6 monoclonal antibodies may have poor effect in patients with DM.

  13. [Pneumomediastinum during a dermatomyositis: a rare entity].

    Science.gov (United States)

    Majdoub, Senda; Zemni, Houcem; Zaghouani, Houneida; Ben Salem, Halima Houda; Amara, Habib; Bakir, Dajla; Kraeim, Chakib

    2016-01-01

    Dermatomyositis is a connective characterized by inflammation of skeletal muscle with cutaneous manifestations. Their etiologies, still unknown, associate environmental and genetic factors. Among lung complications described, interstitial pneumopathies are common complications. Other complications are rarely reported as pneumomediastinum. We report a case of pneumomediastinum with aeric in massive skin dissection occurred in a patient with dermatomyositis. We will discuss the frequency, causes and pathophysiology of the disease according to the literature.

  14. Dermatomyositis as an Expression of Paraneoplastic Syndrome

    OpenAIRE

    Mejía-López, María Gimena; Hospital San José-Fundación Universitaria de Ciencias de la Salud; Alvarado-Ospina, Yury Alejandra; Gutiérrez, Juan Martín; Hospital Universitario San Ignacio; Fernández-Ávila, Daniel G.; Hospital Universitario San Ignacio; Díaz, María Claudia; Hospital Universitario San Ignacio

    2014-01-01

    Dermatomyositis is an inflammatory myopathy with cutaneous compromise that has been linked to neoplastia. We introduce a 74 year old patient diagnosed with dermatomyositis who presented with dysphagia and significant weight loss, which lead to the search of a malignant association, with presence of an advanced neoplasic process requiring palliative management with no primary etiology. Due to the increased incidence ofdermatomyositis in cancer patients compared to the general population, it is...

  15. Chylothorax in dermatomyositis complicated with interstitial pneumonia.

    Science.gov (United States)

    Isoda, Kentaro; Kiboshi, Takao; Shoda, Takeshi

    2016-11-24

    Chylothorax is a disease in which chyle leaks and accumulates in the thoracic cavity. Interstitial pneumonia and pneumomediastinum are common thoracic manifestations of dermatomyositis, but chylothorax complicated with dermatomyositis is not reported. We report a case of dermatomyositis with interstitial pneumonia complicated by chylothorax. A 77-year-old woman was diagnosed as dermatomyositis with Gottron's papules, skin ulcers, anti-MDA5 antibody and rapid progressive interstitial pneumonia. Treatment with betamethasone, tacrolimus and intravenous high-dose cyclophosphamide was initiated, and her skin symptoms and interstitial pneumonia improved once. However, right-sided chylothorax began to accumulate and gradually increase, and at the same time, her interstitial pneumonia began to exacerbate, and skin ulcers began to reappear on her fingers and auricles. Although her chylothorax improved by fasting and parenteral nutrition, she died due to further exacerbations of dermatomyositis and interstitial pneumonia in spite of steroid pulse therapy, increase in the betamethasone dosage, additional intravenous high-dose cyclophosphamide and plasma pheresis. An autopsy showed no lesions such as malignant tumors in the thoracic cavity. This is the first report of chylothorax complicated by dermatomyositis with interstitial pneumonia.

  16. Exacerbation of Dermatomyositis with Recurrence of Rectal Cancer: A Case Report

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    Yuka Nagano

    2015-11-01

    Full Text Available Dermatomyositis (DM is a rare idiopathic inflammatory myopathy characterized by cutaneous and muscle manifestations. The association between DM and malignancy has been well recognized for many years. The clinical course of paraneoplastic DM may be affected by malignancies, although the cause and effect relationship between exacerbation of DM and cancer progression is uncertain. Herein, we report a 44-year-old woman who presented with progressive DM associated with rectal cancer. After curative resection of rectal cancer, DM symptoms resolved. Three months after surgery, blood test surveillance showed elevation of serum carcinoembryonic antigen levels, although the patient remained asymptomatic. One month later she had a DM flare-up, and multiple lung and liver metastases were found. She immediately underwent cancer chemotherapy with prednisolone therapy for DM. However, her condition deteriorated and she was unable to swallow. Percutaneous endoscopic gastrostomy was constructed, allowing alimentation and oral delivery, which made it possible to keep her on chemotherapy. She had remarkable response for unresectable metastases 8 weeks after the administration of chemotherapy. Seven months after onset of recurrence, her condition improved considerably and she had stable disease. Moreover, she can now eat food of soft consistency. Our case provides further support for the clinical importance of cancer chemotherapy for patients who have progressive DM and unresectable rectal cancer.

  17. Exacerbation of Dermatomyositis with Recurrence of Rectal Cancer: A Case Report.

    Science.gov (United States)

    Nagano, Yuka; Inoue, Yasuhiro; Shimura, Tadanobu; Fujikawa, Hiroyuki; Okugawa, Yoshinaga; Hiro, Junichiro; Toiyama, Yuji; Tanaka, Koji; Mohri, Yasuhiko; Kusunoki, Masato

    2015-01-01

    Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by cutaneous and muscle manifestations. The association between DM and malignancy has been well recognized for many years. The clinical course of paraneoplastic DM may be affected by malignancies, although the cause and effect relationship between exacerbation of DM and cancer progression is uncertain. Herein, we report a 44-year-old woman who presented with progressive DM associated with rectal cancer. After curative resection of rectal cancer, DM symptoms resolved. Three months after surgery, blood test surveillance showed elevation of serum carcinoembryonic antigen levels, although the patient remained asymptomatic. One month later she had a DM flare-up, and multiple lung and liver metastases were found. She immediately underwent cancer chemotherapy with prednisolone therapy for DM. However, her condition deteriorated and she was unable to swallow. Percutaneous endoscopic gastrostomy was constructed, allowing alimentation and oral delivery, which made it possible to keep her on chemotherapy. She had remarkable response for unresectable metastases 8 weeks after the administration of chemotherapy. Seven months after onset of recurrence, her condition improved considerably and she had stable disease. Moreover, she can now eat food of soft consistency. Our case provides further support for the clinical importance of cancer chemotherapy for patients who have progressive DM and unresectable rectal cancer.

  18. Dermatomyositis

    Science.gov (United States)

    ... Translational Research Research at NINDS Focus on Research Alzheimer's & Related Dementias Bioengineering Epilepsy Health Disparities Neural Interfaces Parkinson's Disease Spinal Cord Injury Stem Cells Traumatic Brain Injury Trans-Agency Activities Interagency Research ...

  19. Dermatomyositis

    Science.gov (United States)

    ... a muscle disease. The doctor can determine the distribution of the disease by testing different muscles. Magnetic ... ed. New York, N.Y.: The McGraw-Hill Companies; 2013. http://www.accessmedicine.com/resourceTOC.aspx?resourceID= ...

  20. Preface Gi4DM

    NARCIS (Netherlands)

    Zlatanova, S.; Sithole, G.; Nakagawa, M.; Zhu, Q.

    2015-01-01

    Gi4DM 2015 marks the 10th edition of the Geoinformation for Disaster Management series of conferences. The first conference in 2005 was held in the aftermath of the 2004 Indian Ocean earthquake and tsunami which claimed the lives of over 220000 civilians. While Geoinformatics has a long been used in

  1. Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases

    Science.gov (United States)

    2010-06-25

    Rheumatoid Arthritis; Spondyloarthritis; Systemic Lupus Erythematosus (SLE); Dermatomyositis (DM); DMixed Connective Tissue Disease; Systemic Vasculitis; Systemic Sclerosis (SSc); Sjögren's Syndrome; Antiphospholipid Syndrome; Juvenile Idiopathic Arthritis; Juvenile SLE; Juvenile DM

  2. MR findings of polymyositis / dermatomyositis

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    Lee, Hak Soo; Joo, Kyung Bin; Moon, Won Jin; Lee, Tae Hee; Park, Ki Ho; Park, Dong Woo; Hahm, Chang Kok [Hanyang Univ. College of Medicine, Seoul (Korea, Republic of)

    1998-07-01

    The purpose of this study was to evaluate the MR findings and useful sequences in Polymyositis/ Dermatomyositis, and to correlate MR findings with disease activity. Materials and Methods: The study included nine clinically proven cases of Polymyositis/Dermatomyositis, eight involving the thigh and one, the shoulder (2 cases, 1 follow-up). The contrast between affected and normal muscles and difference in signal intensity ratio in the muscle groups were retrospectively evaluated on Gd-enhanced T1WI and T2WI. We also evaluated the magnitude of involvement of muscle groups, fatty replacement of muscle and change of subcutaneous fat layer, and correlated signal intensity ratio with serum level of muscle enzymes. Differences in signal intensity ratio and the frequency of chemical shift artifact were evaluated on T2WI as active and inactive groups classified according to clinical findings, and the chemical shift artifact was correlated with the finding of Gd-enhanced T1WI. Except in the case of one shoulder, statistical analysis was assessed by the Anova test and-test. Results: On Gd-enhanced T1WI and T2WI contrast was 0.54 and 0.82, respectively and p value was 0.02. With regard to difference in signal intensity ratios of muscle groups, as seen on Gd-enhanced T1WI and T2WI, p valves were 0.07 and < 0.01, respectively. Muscle involvement was thus clearly visualized on T2WI. The order of frequency of involved muscle groups was vastus muscles, gluteus maximus, sartorius muscles, adductor muscles, gracilis muscle, and hamstring muscles. Fatty replacement and subcutaneous fatty change were visualized in five cases and one, respectively. The correlation coefficient between the signal intensity seen on T2WI and muscle enzymes was 0.59 (CPK) and 0.52 (LDH). The chemical-shift artifact was detected in both clinical groups (four active two inactive) and corresponded to one case of muscle involvement and five of perimuscular edema, as seen on Gd-enhanced T1WI. Conclusion: T2WI is

  3. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

    Science.gov (United States)

    Aggarwal, Rohit; Rider, Lisa G; Ruperto, Nicolino; Bayat, Nastaran; Erman, Brian; Feldman, Brian M; Oddis, Chester V; Amato, Anthony A; Chinoy, Hector; Cooper, Robert G; Dastmalchi, Maryam; Fiorentino, David; Isenberg, David; Katz, James D; Mammen, Andrew; de Visser, Marianne; Ytterberg, Steven R; Lundberg, Ingrid E; Chung, Lorinda; Danko, Katalin; García-De la Torre, Ignacio; Song, Yeong Wook; Villa, Luca; Rinaldi, Mariangela; Rockette, Howard; Lachenbruch, Peter A; Miller, Frederick W; Vencovsky, Jiri

    2017-05-01

    To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM). Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. The PAPRIKA (Potentially All Pairwise Rankings of All Possible Alternatives) method determined the relative weights of core set measures and conjoint analysis definitions. The performance characteristics of the definitions were evaluated on patient profiles using expert consensus (gold standard) and were validated using data from a clinical trial. The nominal group technique was used to reach consensus. Consensus was reached for a conjoint analysis-based continuous model using absolute percent change in core set measures (physician, patient, and extramuscular global activity, muscle strength, Health Assessment Questionnaire, and muscle enzyme levels). A total improvement score (range 0-100), determined by summing scores for each core set measure, was based on improvement in and relative weight of each core set measure. Thresholds for minimal, moderate, and major improvement were ≥20, ≥40, and ≥60 points in the total improvement score. The same criteria were chosen for juvenile DM, with different improvement thresholds. Sensitivity and specificity in DM/PM patient cohorts were 85% and 92%, 90% and 96%, and 92% and 98% for minimal, moderate, and major improvement, respectively. Definitions were validated in the clinical trial analysis for differentiating the physician rating of improvement (P < 0.001). The response criteria for adult DM/PM consisted of the conjoint analysis model based on absolute percent change in 6 core set measures, with thresholds for minimal, moderate, and major improvement. © 2017, American College of Rheumatology.

  4. [Myositis and the skin: cutaneous manifestations of dermatomyositis].

    Science.gov (United States)

    Jinnin, Masatoshi

    2013-11-01

    Idiopathic inflammatory myopathies include dermatomyositis, polymyositis, and inclusion body myositis. Among them, cutaneous manifestations are observed most frequently in dermatomyositis. While dermatomyositis commonly affects the skin and muscles, it can also affect the lungs and other organs. Dermatomyositis presenting clinically and histopathologically with typical cutaneous lesions, but without myositis, is called amyopathic dermatomyositis. Given that the Bohan and Peter criteria cannot distinguish amyopathic dermatomyositis, understanding the characteristic skin manifestations may be essential for diagnosing this condition. The cutaneous manifestations of dermatomyositis are thought to be the result of the Koebner phenomenon, vasculopathy, or photosensitivity; manifestations include various eruptions, such as heliotrope rush, Gottron's sign, Gottron's papules, mechanic's hand, nail-fold bleeding, skin ulcer, vasculitis, flagellate erythema, V-sign, and Shawl sign. The presence of multiple types of eruptions can help diagnose the disease. Several skin diseases, including adult Still's disease, contact dermatitis, and sarcoidosis, can mimic the cutaneous manifestations of dermatomyositis. Skin biopsy is useful for differential diagnoses. Histopathologically, dermatomyositis of the skin is characterized by liquefaction degeneration, vacuolar degeneration, edema, and mucin deposition. Dermatologists, neurologists, and rheumatologists are responsible for the diagnosis and management of dermatomyositis, in cooperation with pulmonologists, pediatricians, and pathologists. This review aims to provide clinicians with recent findings regarding skin involvement in dermatomyositis.

  5. Dermatomyositis associated with hepatitis B virus-related hepatocellular carcinoma.

    Science.gov (United States)

    Yang, Suh Yoon; Cha, Bong Ki; Kim, Gihyeon; Lee, Hyun Woong; Kim, Jae Gyu; Chang, Sae Kyung; Kim, Hyung Joon

    2014-03-01

    Dermatomyositis is an idiopathic inflammatory myopathy with typical cutaneous manifestations. It has been proposed that dermatomyositis may be caused by autoimmune responses to viral infections. Previous studies have shown an association between dermatomyositis and malignant tumors such as ovarian cancer, lung cancer, and colorectal cancer. However, a chronic hepatitis B virus (HBV) infection associated with dermatomyositis and hepatocellular carcinoma (HCC) has been very rarely reported. Here, we report a rare case of dermatomyositis coinciding with HBV-associated HCC. A 55-year-old male was confirmed to have HCC and dermatomyositis based on proximal muscle weakness, typical skin manifestations, elevated muscle enzyme levels, and muscle biopsy findings. This case suggests that HCC and/or a chronic HBV infection may be factors in the pathogenesis of dermatomyositis through a paraneoplastic mechanism.

  6. [Dermatomyositis and Panniculitis: the function of immunoglobulins].

    Science.gov (United States)

    Abdelhafidh, Nadia Ben; Toujeni, Sana; Kefi, Asma; Bousetta, Najeh; Sayhi, Sameh; Gharsallah, Imen; Othmani, Salah

    2016-01-01

    Panniculitis is an inflammatory disease of subcutaneous adipose tissue which is rarely associated with dermatomyositis. It can occur before, after or simultaneously with muscle damage. In most cases, the evolution of panniculitis and of other dermatomyositis affections is favorable with corticosteroids and/or immunosuppressants. We report the case of a 48 year-old patient who developed panniculitis lesions 2 months before having muscular signs. Skin involvement was resistant to corticosteroid treatment associated with immunosuppressants drugs. This led to the use of polyvalent immunoglobulin treatment improving both skin and muscle damage.

  7. An overview of polymyositis and dermatomyositis.

    Science.gov (United States)

    Findlay, Andrew R; Goyal, Namita A; Mozaffar, Tahseen

    2015-05-01

    Polymyositis and dermatomyositis are inflammatory myopathies that differ in their clinical features, histopathology, response to treatment, and prognosis. Although their clinical pictures differ, they both present with symmetrical, proximal muscle weakness. Treatment relies mainly upon empirical use of corticosteroids and immunosuppressive agents. A deeper understanding of the molecular pathways that drive pathogenesis, careful phenotyping, and accurate disease classification will aid clinical research and development of more efficacious treatments. In this review we address the current knowledge of the epidemiology, clinical characteristics, diagnostic evaluation, classification, pathogenesis, treatment, and prognosis of polymyositis and dermatomyositis.

  8. Cutaneous Manifestations in Dermatomyositis: Key Clinical and Serological Features-a Comprehensive Review.

    Science.gov (United States)

    Muro, Yoshinao; Sugiura, Kazumitsu; Akiyama, Masashi

    2016-12-01

    Dermatomyositis (DM) is a common idiopathic inflammatory myopathy. The pathogenesis is considered to be microangiopathy affecting skin and muscle. The cutaneous manifestations of DM are the most important aspect of this disease, and their correct evaluation is important for early diagnosis. The skin signs are various: Some are pathognomonic or highly characteristic, and others are compatible with DM. Recently, DM has been categorized into several disease subsets based on the various autoantibodies present in patients. Sometimes, characteristic cutaneous manifestations are strongly associated with the presence of specific autoantibodies. For example, anti-Mi-2 antibody is associated with the classic features of DM, including heliotrope rash, Gottron's papules, the V-neck sign, the shawl sign, cuticular overgrowth, and photosensitivity. Frequent cutaneous features in anti-transcriptional intermediary factor 1 gamma (TIF1γ)-positive patients are diffuse photoerythema, including "dusky red face," while skin ulcerations, palmar papules (inverse Gottron), diffuse hair loss, panniculitis, and oral pain and/or ulcers are sometimes associated with anti-melanoma differentiation-associated gene 5 product (MDA5) antibody. Here, we review important cutaneous manifestations seen in patients with DM, and we examine the relationship between the skin changes and myositis-associated autoantibodies. Correct evaluation of cutaneous manifestations and myositis-associated autoantibodies should help the clinician in the early diagnosis of DM, for a quick recognition of cutaneous signs that may be the symptom of onset before muscle inflammation.

  9. Interferon and biologic signatures in dermatomyositis skin: specificity and heterogeneity across diseases.

    Directory of Open Access Journals (Sweden)

    David Wong

    Full Text Available BACKGROUND: Dermatomyositis (DM is an autoimmune disease that mainly affects the skin, muscle, and lung. The pathogenesis of skin inflammation in DM is not well understood. METHODOLOGY AND FINDINGS: We analyzed genome-wide expression data in DM skin and compared them to those from healthy controls. We observed a robust upregulation of interferon (IFN-inducible genes in DM skin, as well as several other gene modules pertaining to inflammation, complement activation, and epidermal activation and differentiation. The interferon (IFN-inducible genes within the DM signature were present not only in DM and lupus, but also cutaneous herpes simplex-2 infection and to a lesser degree, psoriasis. This IFN signature was absent or weakly present in atopic dermatitis, allergic contact dermatitis, acne vulgaris, systemic sclerosis, and localized scleroderma/morphea. We observed that the IFN signature in DM skin appears to be more closely related to type I than type II IFN based on in vitro IFN stimulation expression signatures. However, quantitation of IFN mRNAs in DM skin shows that the majority of known type I IFNs, as well as IFN g, are overexpressed in DM skin. In addition, both IFN-beta and IFN-gamma (but not other type I IFN transcript levels were highly correlated with the degree of the in vivo IFN transcriptional response in DM skin. CONCLUSIONS AND SIGNIFICANCE: As in the blood and muscle, DM skin is characterized by an overwhelming presence of an IFN signature, although it is difficult to conclusively define this response as type I or type II. Understanding the significance of the IFN signature in this wide array of inflammatory diseases will be furthered by identification of the nature of the cells that both produce and respond to IFN, as well as which IFN subtype is biologically active in each diseased tissue.

  10. [Dermatomyositis. Presentation of a mild to moderate case with early dysphagia].

    Science.gov (United States)

    Sánchez Carbonell, M; Climent Antolí, H; Leandro Fonseca, A L; Paricio Talayero, J M; Escrivá Tomás, P

    2015-01-01

    We report the case of a twelve year old female patient with juvenile dermatomyositis. The diagnosis was made in our unit a few days after starting with proximal muscular weakness and a skin rash. A combination of oral corticosteroids and methotrexate was administered. There was an initial improvent in the skin lesions, but with no changes in the muscle weakness. The appearance of dysphagia a few days after starting the treatment led us to add three pulses of parenteral methylprednisolone to her initial treatment. We report the case to illustrate the difficulties in deciding initial treatment options.

  11. Ubiquitous CM and DM

    Science.gov (United States)

    Crowley, Sandra L.

    2000-01-01

    Ubiquitous is a real word. I thank a former Total Quality Coach for my first exposure some years ago to its existence. My version of Webster's dictionary defines ubiquitous as "present, or seeming to be present, everywhere at the same time; omnipresent." While I believe that God is omnipresent, I have come to discover that CM and DM are present everywhere. Oh, yes; I define CM as Configuration Management and DM as either Data or Document Management. Ten years ago, I had my first introduction to the CM world. I had an opportunity to do CM for the Space Station effort at the NASA Lewis Research Center. I learned that CM was a discipline that had four areas of focus: identification, control, status accounting, and verification. I was certified as a CMIl graduate and was indoctrinated about clear, concise, and valid. Off I went into a world of entirely new experiences. I was exposed to change requests and change boards first hand. I also learned about implementation of changes, and then of technical and CM requirements.

  12. [Porokeratosis in a patient with dermatomyositis].

    Science.gov (United States)

    Monteagudo-Sánchez, B; Ginarte, M; Durana, C; Labandeira, J; de las Heras, C; Cacharrón, J M

    2006-12-01

    There are several reports of porokeratosis in the context of immmunosuppressive diseases. These mainly include organ transplant, HIV infection, lymphomas and some inflammatory and autoimmune diseases commonly treated with immunosuppresive drugs or chemotherapy. Disseminated superficial actinic porokeratosis is the clinical variant of porokeratosis that most frequently develops in immunosuppressive states. We report a case of porokeratosis in a woman with dermatomyositis.

  13. Paraneoplastic Dermatomyositis in Hepatocellular Carcinoma with Colonic Perforation: A Case Report

    Science.gov (United States)

    Miyata, Naoteru; Emoto, Katsura; Dei, Yoshiaki; Tomiyasu, Kazuhiro; Ishiyama, Ryoko; Horie, Tomofumi; Sakai, Gen; Tahara, Toshiyuki

    2016-01-01

    Background Dermatomyositis (DM) is an autoimmune disease characterized by cutaneous Gottron papules, heliotrope rash, and proximal myopathy. It may also present as a paraneoplastic syndrome that can complicate a variety of different cancers, such as lung, cervical, and breast cancer. However, the association with hepatocellular carcinoma (HCC) is extremely rare. Moreover, to our knowledge, there are no previous reports of colonic perforation following steroid pulse treatment for a DM patient. Case Summary A 61-year-old male complained of a skin rash that began in his neck and spread to his face and abdomen. On physical examination, the patient was also found to have symmetrical proximal muscle weakness, abdominal pain, heliotrope rash in the periorbital skin, and poikiloderma on his face and abdomen. Serum level of muscle enzymes was remarkably increased. Muscle examination revealed symmetrical proximal weakness. The diagnosis of DM was made, and steroid treatment was started for symptomatic relief. A search for causative malignancy revealed HCC. Despite steroid therapy for DM, his symptoms did not improve. Additionally, C-reactive protein elevation was seen along with severe abdominal pain on day 14 of admission. Shortly after this, the patient died of septic shock due to suppurative peritonitis after perforation of the ascending colon. Conclusion Here, we present a rare case of DM caused by non-hepatitis-associated advanced HCC with colonic perforation. The cause of colonic perforation is still unclear. This case demonstrates the need to carefully monitor abdominal pain in DM patients as symptoms can be masked by steroid therapy.

  14. [MHC class I antigens, CD4 and CD8 expressions in polymyositis and dermatomyositis].

    Science.gov (United States)

    Graça, Carla Renata; Kouyoumdjian, João Aris

    2015-01-01

    To analyze the frequencies of the expression of major histocompatibility complex class I (MHC-I) antigens, and CD4 and CD8 cells in skeletal muscle in polymyositis (PM) and dermatomyositis (DM). This was a retrospective study of 34 PM cases, 8 DM cases, and 29 control patients with non-inflammatory myopathies. MHC-I antigens were expressed in the sarcolemma and/or sarcoplasm in 79.4% of PM cases, 62.5% of DM cases, and 27.6% of controls (CD4 expression was observed in 76.5%, 75%, and 13.8%, respectively). There was a high suspicion of PM/DM (mainly PM) in patients in whom MHC-I antigens and CD4 were co-expressed. In 14.3% of PM/DM cases, we observed MHC-I antigens expression alone, without inflammatory cells. MCH-I antigens expression and CD4 positivity might add to strong diagnostic suspicion of PM/DM. No cellular infiltration was observed in 14.3% of such cases. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  15. Juvenile idiopathic inflammatory myopathies: the value of magnetic resonance imaging in the detection of muscle involvement

    Directory of Open Access Journals (Sweden)

    Maria Odete Esteves Hilário

    2000-03-01

    Full Text Available CONTEXT: One of the major current challenges related to juvenile idiopathic inflammatory myopathy is the search for highly sensitive and specific non-invasive methods for diagnosis as well as for follow-up. OBJECTIVES: The aim of our study was to describe typical magnetic resonance imaging findings and to investigate the usefulness of this method in detecting active muscle disease in juvenile dermatomyositis and juvenile systemic lupus erythematosus patients. DESIGN: Transverse study, blinded assessment. SETTING: University referral unit (Pediatric Rheumatology section, Department of Pediatrics, Universidade Federal de São Paulo / Escola Paulista de Medicina. SAMPLE: Thirteen patients (9 girls with dermatomyositis, as well as 13 patients (12 girls with juvenile systemic lupus erythematosus and 10 normal children (5 girls, were enrolled in the study. MAIN MEASUREMENTS: Qualitative and quantitative analyses of gluteus maximus, quadriceps, adductors and flexors were performed and evaluated by two radiologists, blinded to all clinical information. Spin-echo in T1, DP, T2 and IR was used in all MRI images. RESULTS: The different muscle groups presented non-uniform involvement in the patients. The patients with dermatomyositis presented acute and chronic muscular alterations, while those with lupus presented only chronic myopathy, especially atrophy. In the dermatomyositis group, the major alterations were found in the gluteus and flexor regions (signal intensity and fat replacement. The signal intensity was increased in all acute myopathies. CONCLUSION: The qualitative and quantitative resonance analyses are useful in detecting clinically active disease in patients with dermatomyositis.

  16. Dermatomysitis: Medical Management

    Science.gov (United States)

    ... Meet our Partners How to Get Involved Donate Dermatomyositis (DM) Share print email share facebook twitter google ... activity. Myositis and You: A Guide to Juvenile Dermatomyositis for Patients, Families and Healthcare Providers , is a ...

  17. When and how should the patients with dermatomyositis be assessed for interstitial lung disease?

    Institute of Scientific and Technical Information of China (English)

    GUO Li-shao; ZHANG Jian-zhong

    2010-01-01

    @@ Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies with unknown etiology.The incidence of both diseases is estimated at 4 to 10 cases per million people per year~1.Classical DM patients display characteristic cutaneous manifestations,proximal muscle weakness,and laboratorial evidence of myositis.Skin manifestations include the classic periorbital heliotrope,cutaneous Gottron's papules and signs,and violaceous erythema of the upper trunk and extremities.Muscle involvement may be revealed by elevated muscle-derived enzymes such as creatine kinase (CK),lactate dehydrogenase (LDH),aldolase,alanine aminotransferase (ALT) or aspartate transaminase (AST).Additional testing,including electromyography (EMG),magnetic resonance imaging (MRI),or even muscle biopsies,may be performed if other tests are inconclusive~2.

  18. High-resolution CT study of interstitial lung disease in polymyositis and dermatomyositis

    Energy Technology Data Exchange (ETDEWEB)

    Tomii, Keisuke; Iwata, Takekuni; Oida, Kazukiyo (Tenri Hospital, Nara (Japan)) (and others)

    1992-01-01

    High-resolution CT scans of lung parenchyma were obtained in 8 patients with pure polymyositis or dermatomyositis (PM-DM) with interstitial lung involvement. The most frequent findings were subpleural curved band-like shadows (A 2), which were demonstrated in 7 patients (88%). Open lung biopsy was performed in a case of A 2 shadow, which revealed non-specific interstitial pneumonia (chronic interstitial pneumonia, not otherwise specified). Most of the A 2 shadows gradually shrinked with steroids or immunosuppressants, and then disappeared completely or changed into subpleural curvilinear shadows (A 1). Peri-bronchovascular shadows (B) were detected in 4 patients (50%). Acute exacerbation had occurred in the two of them, but after all every B shadow reduced or disappeared with therapy. We think A 2 and B shadows are characteristic HRCT findings of interstitial lung disease in PM-DM and their prognosis is fairly good. (author).

  19. [Dermatomyositis in a family of Working Kelpies].

    Science.gov (United States)

    Röthig, A; Rüfenacht, S; Welle, M M; Thom, N

    2015-01-01

    Eight members of a family of Working Kelpies were presented with signs compatible with dermatomyositis. Alopecia, crusts, ulcerations of the skin, depigmentation of nasal planum and lips, onychodystrophy and atrophy of the masticatory muscles were present with varying degree. Histopathology of the skin, but not from muscles was performed in three dogs and confirmed the clinical diagnosis. Different immunomodulating drugs (steroids, cyclosporine, mycophenolate mofetil, pentoxifylline, doxycyline/niacinamid, omega-3 fatty acids and vitamin E) were used with variable success. Dermatomyositis is an immune-mediated disease and a genetic predisposition is known in humans and certain canine breeds, mainly Shetland Sheepdogs and Collies, but also for the Beauceron. The responsible genes have not been identified so far. It is assumed that the Working Kelpie derives from the Collie which could explain a hereditary predisposition in the Kelpie.

  20. New Onset of Dermatomyositis/Polymyositis during Anti-TNF-α Therapies: A Systematic Literature Review

    Directory of Open Access Journals (Sweden)

    Alexandra Maria Giovanna Brunasso

    2014-01-01

    Full Text Available We performed a systematic search of databases from 1990 to 2013 to identify articles concerning the new onset of dermatomyositis/polymyositis (DM/PM in patients treated with anti-TNF-α therapy. We retrieved 13 publications describing 20 patients where the new onset of DM/PM after anti-TNF-α therapy was recorded. 17 patients were affected by rheumatoid arthritis (RA, one by Crohn’s disease, one by ankylosing spondilytis, and one by seronegative arthritis. In 91% of the cases antinuclear autoantibodies were detected after the introduction of anti-TNF-α therapy. In 6 patients antisynthetase antibodies were detected and other clinical findings as interstitial lung disease (ILD were recorded. Improvement of DM/PM after anti-TNF suspension (with the concomitant use of other immunosuppressors was recorded in 94% of cases. The emergence of DM/PM and antisynthetase syndrome seem to be associated with the use of anti-TNF-α agents, especially in patients with chronic inflammatory diseases (mainly RA with positive autoantibodies before therapy initiation. In particular, physicians should pay attention to patients affected by RA with positive antisynthetase antibodies and/or history of ILD. In those cases, the use of the TNF-α blocking agents may trigger the onset of PM/DM or antisynthetase syndrome or may aggravate/trigger the lung disease.

  1. IL-1RN VNTR Polymorphism in Adult Dermatomyositis and Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Zornitsa Kamenarska

    2014-01-01

    Full Text Available Polymorphisms in the cytokine genes and their natural antagonists are thought to influence the predisposition to dermatomyositis (DM and systemic lupus erythematosus (SLE. A variable number tandem repeat (VNTR polymorphism of 86 bp in intron 2 of the interleukin-1 receptor antagonist (IL-1RN gene leads to the existence of five different alleles which cause differences in the production of both IL-1RA (interleukin-1 receptor antagonist and IL-1β. The aim of this case-control study was to investigate the association between the IL-1RN VNTR polymorphism and the susceptibility to DM and SLE in Bulgarian patients. Altogether 91 patients, 55 with SLE and 36 with DM, as well as 112 unrelated healthy controls, were included in this study. Only three alleles were identified in both patients and controls ((1 four repeats, (2 two repeats, and (3 five repeats. The IL-1RN*2 allele (P=0.02, OR 2.5, and 95% CI 1.2–5.4 and the 1/2+2/2 genotypes were found prevalent among the SLE patients (P=0.05, OR 2.6, and 95% CI 1–6.3. No association was found between this polymorphism and the ACR criteria for SLE as well as with the susceptibility to DM. Our results indicate that the IL-1RN VNTR polymorphism might play a role in the susceptibility of SLE but not DM.

  2. Subscapular bursitis as a rare manifestation of dermatomyositis: a case report

    Science.gov (United States)

    Kim, Kyung Rok; Konig, Maximilian F.; Park, Jin Kyun

    2015-01-01

    Dermatomyositis (DM) is characterized by proximal muscle weakness and characteristic skin rash. Pain is a less common feature and usually indicates inflammation of extramuscular structures such as fascia. Here we report a rare case of subscapular bursitis in a 48-year-old woman with DM. She initially presented with severe, sharp, stabbing pain in her right shoulder that worsened with shoulder movement. Magnetic resonance imaging (MRI) revealed inflammation in the right subscapular bursae. A few months later, the patient developed periungual erythema, Gottron’s papules, and shawl sign with muscle pain in her thighs. DM was diagnosed based on the presence of interface dermatitis on skin biopsy and diffuse muscle inflammation on MRI. Bursitis and myalgia responded incompletely to nonsteroidal anti-inflammatory drugs but promptly to corticosteroids. Here we report a case of subscapular bursitis as a rare manifestation of DM. Pain in patients with DM may warrant physicians to evaluate for the presence of additional inflammatory processes in the perimuscular structures. PMID:27708933

  3. Elevated serum levels of neopterin in adult patients with polymyositis/dermatomyositis.

    Science.gov (United States)

    Samsonov, M Y; Nassonov, E L; Tilz, G P; Geht, B M; Demel, U; Gurkina, G T; Shtutman, V Z; Guseva, A G; Wachter, H; Fuchs, D

    1997-06-01

    We determined serum concentrations of neopterin, soluble tumour necrosis factor (55 kDa) receptor (sTNF-R) and soluble interleukin-2 receptor (sIL-2R) in plasma of 44 patients with polymyositis (PM)/dermatomyositis (DM), including 15 patients with primary PM, 13 patients with primary DM, and 16 patients with myositis and systemic sclerosis in overlap. Concentrations of neopterin, sTNF-R and sIL-2R were measured using commercially available immunoassays. Serum neopterin was increased in 35 of 44 PM/DM patients (80%), sTNF-R in 14 (32%) and sIL-2R in 18 (41%) patients, respectively. There were significant correlations between serum neopterin and sTNF-R, sIL-2R and erythrocyte sedimentation rate (all P < 0.001). Neopterin, as well as sTNF-R and sIL-2R, did not correlate with clinical (neuromuscular and activities of daily living scores) and laboratory (creatine kinase levels) manifestations of myositis. Increased serum levels of neopterin were associated with non-muscular manifestations of PM/DM. In conclusion, serum neopterin appears to be a useful laboratory marker for ongoing immune activation and global disease activity in PM/DM.

  4. Dermatomyositis followed by pemphigus foliaceus: a case report and possible mechanism

    Directory of Open Access Journals (Sweden)

    Ahmad Tuffaha

    2009-10-01

    Full Text Available Dermatomyositis (DM and Pemphigus foliaceus (PF are two autoimmune diseases that are rarely reported in the same patient. DM is an idiopathic inflammatory myopathy with characteristic skin manifestations. Muscle involvement manifests as proximal weakness. Muscle tenderness may occur but is not a regular feature. Classic dermatological findings in DM include Gottron’s papules, heliotrope erythema, the shawl sign, and facial erythema. The etiology of DM remains unknown; some studies have reported an association with histocompatability antigens, environmental agents (e.g. virus, drugs and autoimmunity.1,2 Several autoantibodies have been identified, but their routine use in diagnosis has not yet been defined. These autoantibodies have been termed myositis-specific antibodies and include Anti-Mi-2, Anti-Jo-1, antisignal recognition protein and anti-Ku.2 Pemphigus foliaceus, which is the least severe form of pemphigus, is characterized by crusted, scaly sores, or fragile blisters. Oral or other mucocutaneous blisters do not occur in this disorder. Circulating antibodies in PF bind to desmoglein-1 protein rather than to the desmoglein-3 protein affected in pemphigus vulgaris (PV. Unlike the blisters seen in PV, the lesions in PF may itch and may easily be confused with eczema or non-specific dermatitis. We report a case of a 53-year old African American female who presented with DM and later developed PF. Narbutt et al. reported a similar case of a patient who presented with these two conditions.3

  5. [The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis'

    NARCIS (Netherlands)

    Hoogendijk, J.E.; Bijlsma, J.W.J.; Engelen, B.G.M. van; Lindeman, E.J.M.; Royen-Kerkhof, A. van; Rie, M.A. de; Visser, M. de; Jennekens, F.G.I.

    2005-01-01

    This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a

  6. Pyruvate kinase M2 and the mitochondrial ATPase Inhibitory Factor 1 provide novel biomarkers of dermatomyositis: a metabolic link to oncogenesis

    OpenAIRE

    Santacatterina, Fulvio; Sánchez-Aragó, María; Catalán-García, Marc; Garrabou, Glòria; de Arenas, Cristina Nuñez; Grau, Josep M.; Cardellach, Francesc; Cuezva, José M.

    2017-01-01

    Background Metabolic alterations play a role in the development of inflammatory myopathies (IMs). Herein, we have investigated through a multiplex assay whether proteins of energy metabolism could provide biomarkers of IMs. Methods A cohort of thirty-two muscle biopsies and forty plasma samples comprising polymyositis (PM), dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) and control donors was interrogated with monoclonal antibodies against proteins of energy metabolism using...

  7. Juvenile angiofibroma

    Science.gov (United States)

    Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor; Juvenile nasal angiofibroma; JNA ... Juvenile angiofibroma is not very common. It is most often found in adolescent boys. The tumor contains ...

  8. Implications in the difference of anti-Mi-2 and -p155/140 autoantibody prevalence in two dermatomyositis cohorts from Mexico City and Guadalajara.

    Science.gov (United States)

    Petri, Marcelo H; Satoh, Minoru; Martin-Marquez, Beatriz T; Vargas-Ramírez, Raul; Jara, Luis J; Saavedra, Miguel A; Cruz-Gonzalez, Claudia; Andrade-Ortega, Lilia; Vera-Lastra, Olga; Salazar-Páramo, Mario; Prieto-Parra, Rosa E; Gonzalez-Lopez, Laura; Gamez-Nava, Jorge I; Ramírez-Sánchez, Hermes U; Chan, Jason Y F; Ross, Steven J; Chan, Edward K L; Vázquez-Del Mercado, Mónica

    2013-04-04

    Autoantibodies and clinical manifestations in polymyositis/dermatomyositis (PM/DM) are affected by both genetic and environmental factors. The high prevalence of DM and anti-Mi-2 in Central America is thought to be associated with the high UV index of the area. The prevalences of autoantibodies and the clinical manifestations of PM/DM were evaluated comparing two cohorts in Mexico. Ninety-five Mexican patients with PM/DM (66 DM, 29 PM; 67 Mexico City, 28 Guadalajara) were studied. Autoantibodies were characterized by immunoprecipitation using 35S-methionine labeled K562 cell extract. Clinical information was obtained from medical records. DM represented 69% of PM/DM and anti-Mi-2 was the most common autoantibody (35%), followed by anti-p155/140 (11%); however, anti-Jo-1 was only 4%. The autoantibody profile in adult-onset DM in Mexico City versus Guadalajara showed striking differences: anti-Mi-2 was 59% versus 12% (P = 0.0012) whereas anti-p155/140 was 9% versus 35% (P = 0.02), respectively. A strong association of anti-Mi-2 with DM was confirmed and when clinical features of anti-Mi-2 (+) DM (n = 30) versus anti-Mi-2 (-) DM (n = 36) were compared, the shawl sign (86% versus 64%, P Guadalajara, which have a similar UV index. This suggests roles of factors other than UV in anti-Mi-2 antibody production.

  9. Survival benefit associated with early cyclosporine treatment for dermatomyositis-associated interstitial lung disease.

    Science.gov (United States)

    Go, Dong Jin; Park, Jin Kyun; Kang, Eun Ha; Kwon, Hyun Mi; Lee, Yun Jong; Song, Yeong Wook; Lee, Eun Bong

    2016-01-01

    Interstitial lung disease (ILD) is the most common cause of death in dermatomyositis (DM). Cyclosporine A (CsA) has shown to be effective in DM-associated ILD (DM-ILD). This study aimed to define the optimal time of CsA administration. A total of 47 patients with DM-ILD, who were treated with CsA at Seoul National University Hospital between January 1998 and June 2013, were enrolled. ILD was diagnosed based on typical chest high-resolution computed tomography (HRCT) findings. Patients with early and delayed CsA treatment were compared in regard to the mortality and ILD progression on HRCT. The early (n = 16) and the delayed treatment group (n = 31) did not differ in regard to baseline clinical characteristics including HRCT scores and pulmonary function. Patients with clinically amyopathic DM (CADM) were more common in the early treatment group. The mortality rate was significantly lower in the early treatment group than in the delayed treatment group (p = 0.009). The survival benefit of early CsA treatment remained significant even after adjusting for age, degree of dyspnea, CADM status, and the year of CsA treatment (hazard ratio 0.057, 95 % confidence interval 0.007-0.472). CsA stabilized disease progression on HRCT in the early treatment group (p = 0.738). Delay in CsA treatment is associated with a worse survival in patients with DM-ILD. Early CsA treatment should be considered at DM-ILD diagnosis especially in patients at a higher risk of developing a rapidly progressive ILD.

  10. Clinical characteristics of anti-SAE antibodies in Chinese patients with dermatomyositis in comparison with different patient cohorts.

    Science.gov (United States)

    Ge, Yongpeng; Lu, Xin; Shu, Xiaoming; Peng, Qinglin; Wang, Guochun

    2017-03-15

    This study aimed to analyze the clinical features of anti-SAE antibodies in Chinese myositis patients in comparison with different cohorts. The anti-SAE antibodies were tested in myositis patients and in control subjects. Long-term follow-up was conducted on the antibody-positive patients. Anti-SAE antibodies were exclusively present in 12 out of 394 (3.0%) adult dermatomyositis (DM) patients. Of the anti-SAE-positive DM patients, 75% had distinctive diffuse dark-red or pigment-like skin rashes, and 67% of these patients experienced mild muscle weakness. Muscular biopsies showed mild pathological manifestations. Compared with the antibody-negative group, the average age of dermatomyositis onset in the antibody-positive group was higher, and dysphagia occurred more frequently noted (p = 0.012). Only 9 patients received follow-up, 7 experienced improvement after treatment. The anti-SAE antibody levels correlated with improved disease condition. The anti-SAE antibody was found exclusively in adult DM patients, occurring infrequently in Chinese patients. In addition to a diffuse dark-red or pigment-like skin rash and mild muscular weakness, common symptoms included propensity for developing dysphagia. Serum levels of the anti-SAE antibody correlated with myositis disease activity, and anti-SAE-positive patients were responsive to treatment.

  11. The role of type I interferons and other cytokines in dermatomyositis.

    Science.gov (United States)

    Arshanapalli, Ashish; Shah, Mihir; Veerula, Vindhya; Somani, Ally-Khan

    2015-06-01

    Much work has been done to unveil the mechanisms behind the pathogenesis of dermatomyositis (DM) - mainly those involving certain pathogenic cytokines, termed "pathokines" as the principal cytokines involved. Recently, it has become clear that a group of cytokines known as type I interferons (IFN-Is) play a significant role in the development of DM. We review the literature published between 1946 and 2014 using an Ovid Medline database search to provide an update on the role of IFN-Is and other cytokines in the pathogenesis of DM. We provide information about the genes and proteins induced by IFN-Is and potential mechanisms by which these downstream products relate to clinical disease activity. We also explore findings of other autoimmune phenomena that may contribute to disease onset and activity including T-helper 17 (Th17) cells and associated interleukins, as well as autoantibodies. Finally, we provide a brief update on current treatment options for DM as well as some new immunomodulatory treatment modalities in development.

  12. A Case of Dermatomyositis and Anti-EJ Autoantibody with Chronic Intestinal Pseudoobstruction Successfully Treated with Octreotide

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    Chiho Yamada

    2016-01-01

    Full Text Available Chronic intestinal pseudoobstruction (CIPO is a serious complication in patients with connective tissue disease (CTD and is sometimes life-threatening or fatal despite intensive medical treatment. Here, we report a patient with dermatomyositis (DM and anti-EJ autoantibody who developed CIPO that was improved by octreotide. Because her abdominal pain and bloatedness were so severe and persistent, we introduced octreotide to relieve symptoms. In this case, continuous intravenous administration as well as long-acting subcutaneous injection of octreotide was effective for treating CIPO.

  13. Unusual sequelae of adult-onset dermatomyositis

    Science.gov (United States)

    Naffaa, Mohammad Ebrahim; Bishara, Rema; Braun-Moscovici, Yolanda; Balbir-Gurman, Alexandra

    2014-01-01

    A 44-year-old woman diagnosed with dermatomyositis 5 years ago based on progressive proximal muscle weakness, elevated creatine kinase, typical findings on electromyography and muscle biopsy. Despite the treatment, in contrast to improvement in her muscle symptoms, the heliotrope rash of her eyelids persisted. After several years, the patient developed multiple limited skin retraction lesions with hyperpigmentation on both lower limbs. Palpation of these lesions revealed dry, cold and very firm skin on both thighs and calves, particularly in the distal areas. X-ray and ultrasound imaging of the calves showed multiple subcutaneous calcifications in the distal muscles. PMID:25085949

  14. 临床无肌病性皮肌炎的诊断及治疗%The diagnosis and treatment of clinically amyopathic dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    刘宇; 王雷; 刘玉峰

    2012-01-01

    临床无肌病性皮肌炎被认为是皮肌炎的一种少见类型,是指具有皮肌炎的典型皮损,临床上不表现为肌无力,肌酶谱正常或仅轻度异常,分为无肌病性皮肌炎和低肌病性皮肌炎.部分患者与严重的肺部病变及恶性肿瘤相关,而部分严重患者对糖皮质激素治疗不敏感,故早期发现和正确认识该病并给予合理的干预,对改善患者预后非常重要.%Clinically amyopathic dermatomyositis(C-ADM) is a rare form of dermatomyositis (DM), in which typical skin changes contrast with minimal or absent muscle abnormalities, including amyopathic dermatomyositis (ADM) and hypomyopathic dermatomyositis (HDM). In some cases, it is associated with severe lung lesions and malignant tumors. Patients with severe disease of this kind are unresponsive to gtucocorticoids. So early detection and correct understanding of the disease, and a proper intervention to it is very important for improving patient outcomes. In this paper, the diagnosis and treatment of clinically amyopathic dermatomyositisare are summarized as follows.

  15. Activation of the wnt/β-Catenin Signaling Pathway in Polymyositis, Dermatomyositis and Duchenne Muscular Dystrophy

    Science.gov (United States)

    Liu, Fuchen; Liang, Zonglai; Xu, Jingwen; Li, Wei; Zhao, Dandan; Zhao, Yuying

    2016-01-01

    Background and Purpose The wnt/β-catenin signaling pathway plays a critical role in embryonic development and adult-tissue homeostasis. Recent investigations implicate the importance of wnt/β-catenin signaling in normal wound healing and its sustained activation being associated with fibrogenesis. We investigated the immunolocalization and activation of wnt/β-catenin in polymyositis (PM), dermatomyositis (DM), and Duchenne muscular dystrophy (DMD). Methods Immunofluorescence staining and Western blot analysis of β-catenin were performed in muscle specimens from 6 PM, 8 DM, and 6 DMD subjects. The β-catenin/Tcf4 DNA-binding activity in muscle was studied using an electrophoretic mobility shift assay (EMSA), and serum wnt/β-catenin/Tcf transcriptional activity was measured using a luciferase reporter gene assay. Results Immunoreactivity for β-catenin was found in the cytoplasm and nuclei of muscle fibers in PM, DM, and DMD. The protein level of β-catenin was elevated, and EMSA analysis confirmed the activation of wnt/β-catenin signaling. The transcriptional activities of β-catenin/Tcf in the circulation were increased in patients with PM, DM, and DMD, especially in those with interstitial lung disease, and these transcriptional activities decreased when PM or DM patients exhibited obvious clinical improvements. Conclusions Our findings indicate that wnt/β-catenin signaling is activated in PM, DM, and DMD. Its activation in muscle tissue and the circulation may play a role in modulating muscle regeneration and be at least partly involved in the process of muscle and pulmonary fibrosis. PMID:27165423

  16. Paraneoplastic Dermatomyositis in Hepatocellular Carcinoma with Colonic Perforation: A Case Report

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    Naoteru Miyata

    2016-09-01

    Full Text Available Background: Dermatomyositis (DM is an autoimmune disease characterized by cutaneous Gottron papules, heliotrope rash, and proximal myopathy. It may also present as a paraneoplastic syndrome that can complicate a variety of different cancers, such as lung, cervical, and breast cancer. However, the association with hepatocellular carcinoma (HCC is extremely rare. Moreover, to our knowledge, there are no previous reports of colonic perforation following steroid pulse treatment for a DM patient. Case Summary: A 61-year-old male complained of a skin rash that began in his neck and spread to his face and abdomen. On physical examination, the patient was also found to have symmetrical proximal muscle weakness, abdominal pain, heliotrope rash in the periorbital skin, and poikiloderma on his face and abdomen. Serum level of muscle enzymes was remarkably increased. Muscle examination revealed symmetrical proximal weakness. The diagnosis of DM was made, and steroid treatment was started for symptomatic relief. A search for causative malignancy revealed HCC. Despite steroid therapy for DM, his symptoms did not improve. Additionally, C-reactive protein elevation was seen along with severe abdominal pain on day 14 of admission. Shortly after this, the patient died of septic shock due to suppurative peritonitis after perforation of the ascending colon. Conclusion: Here, we present a rare case of DM caused by non-hepatitis-associated advanced HCC with colonic perforation. The cause of colonic perforation is still unclear. This case demonstrates the need to carefully monitor abdominal pain in DM patients as symptoms can be masked by steroid therapy.

  17. 多发性肌炎和皮肌炎%Multiple myositis and dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    邹和建

    2004-01-01

    @@ 多发性肌炎(polymyositis, PM)和皮肌炎(dermatomyositis, DM)是一组主要累及横纹肌,呈慢性非化脓性炎症改变,并以肌无力为特征的自身免疫性结缔组织疾病,前者仅有肌肉病变而无皮肤损害,后者常具特征性皮肤表现.女性发病多于男性,女与男之比约为2:1.本病病因不明,发病与病毒感染、免疫异常、遗传及肿瘤等因素有关.

  18. A Case of Amyopathic Dermatomyositis with Pneumomediastinum and Subcutaneous Emphysema

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    Aslıhan Gürün Kaya

    2015-01-01

    Full Text Available A 34-year-old man was admitted with dyspnea, cough, and fever. Thorax computed tomography revealed ground glass opacities and pneumomediastinum. The patient was diagnosed as amyopathic dermatomyositis due to skin lesions and radiological findings. Despite immunosuppressive treatment clinical deterioration and radiological progression were observed and the patient died because of severe hypoxemic respiratory failure. The patient presented with extremely rare occurrence of pneumomediastinum and subcutaneous emphysema in amyopathic dermatomyositis with a poor prognosis.

  19. Elevated Erythrocyte Sedimentation Rate Is Predictive of Interstitial Lung Disease and Mortality in Dermatomyositis: a Korean Retrospective Cohort Study.

    Science.gov (United States)

    Go, Dong Jin; Lee, Eun Young; Lee, Eun Bong; Song, Yeong Wook; Konig, Maximilian Ferdinand; Park, Jin Kyun

    2016-03-01

    Interstitial lung disease (ILD) is a major cause of death in patients with dermatomyositis (DM). This study was aimed to examine the utility of the erythrocyte sedimentation rate (ESR) as a predictor of ILD and prognostic marker of mortality in patients with DM. One hundred-and-fourteen patients with DM were examined, including 28 with clinically amyopathic DM (CADM). A diagnosis of ILD was made based on high resolution computed tomography (HRCT) scans. The association between elevated ESR and pulmonary impairment and mortality was then examined. ILD was diagnosed in 53 (46.5%) of 114 DM patients. Cancer was diagnosed in 2 (3.8%) of 53 DM patients with ILD and in 24 (92.3%) of those without ILD (P < 0.001). The median ESR (50.0 mm/hour) in patients with ILD was significantly higher than that in patients without ILD (29.0 mm/hour; P < 0.001). ESR was inversely correlated with forced vital capacity (Spearman ρ = - 0.303; P = 0.007) and carbon monoxide diffusing capacity (ρ = - 0.319; P = 0.006). DM patients with baseline ESR ≥ 30 mm/hour had significantly higher mortality than those with ESR < 30 mm/hour (P = 0.002, log-rank test). Patients with a persistently high ESR despite immunosuppressive therapy was associated with higher mortality than those with a normalized ESR (P = 0.039, log-rank test). Elevated ESR is associated with increased mortality in patients with DM due to respiratory failure. Thus, monitoring ESR should be an integral part of the clinical care of DM patients.

  20. The Heterogeneity of Juvenile Myositis

    Science.gov (United States)

    Rider, Lisa G.

    2007-01-01

    Juvenile myositis is a heterogeneous group of systemic autoimmune diseases, in which clinical and serologic subgroups result in subsets of patients with distinct clinical manifestations, disease courses, immunogenetic associations, responses to therapy, and prognoses. A newly identified autoantibody of unknown specificity, anti-p155, is myositis-associated and seen in up to 20 – 30% of juvenile and adult DM patients. HLA DRB1*0301 and its linked allele DQA1*0501 have been identified as the major immunogenetic risk factor for juvenile and adult DM in both European- and African- American patients, and DQA1*0301 is an additional risk factor in European American patients. Several DQA1 alleles also are protective for juvenile DM. Environmental risk factors are poorly understood, but growing evidence suggests a role for infectious agents and ultraviolet radiation. The current therapy of juvenile DM consists of corticosteroids and other immunosuppressive agents, with the adjunctive treatment of cutaneous manifestations and rehabilitation. Therapeutic trials of biologic agents, including anti-TNFα and anti-CD20, may aid in developing promising new therapies for these disorders. PMID:17317616

  1. An atypical case of dermatomyositis associated with chromophobe renal cell carcinoma.

    Science.gov (United States)

    George, Michael David; Lahouti, Arash H; Christopher-Stine, Lisa

    2016-01-19

    Dermatomyositis and polymyositis are the major idiopathic inflammatory myopathies in adults. They are associated with an elevated risk of malignancy. However, renal tumours have rarely been described in dermatomyositis patients. We report the case of a 27-year-old Caucasian man with chromophobe renal cell cancer (ChRCC) and antinuclear matrix protein (NXP-2)-associated dermatomyositis. To the best of our knowledge, there are no previous reports of ChRCC presenting with dermatomyositis.

  2. Retinoschisis (Juvenile)

    Science.gov (United States)

    ... here Home › Eye Conditions Listen Retinoschisis What is Juvenile Retinoschisis? Juvenile retinoschisis is an inherited disease diagnosed in childhood ... degeneration of the retina. What are the symptoms? Juvenile retinoschisis, also known as X-linked retinoschisis, occurs ...

  3. Wong-Type Dermatomyositis Showing Porokeratosis-Like Changes (Columnar Dyskeratosis: A Case Report and Review of the Literature

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    Nicole Umanoff

    2015-01-01

    Full Text Available Background: Wong-type dermatomyositis (DM exhibits simultaneous pityriasis rubra pilaris (PRP features. Case Report: A 50-year-old woman presented with a heliotrope rash, Gottron's papules, and a poikilodermic, erythematous rash in shawl distribution without evidence of muscle weakness. Despite topical corticosteroids, the eruption progressed 9 months later to include generalized hyperkeratotic follicular papules, islands of sparing, and atrophic macules with a collarette of scale suggestive of porokeratosis. Mild dysphonia was the only sign of muscle weakness. Serology showed positive ANA. Histopathology revealed interface dermatitis with dermal mucin and melanophages, irregular psoriasiform hyperplasia, alternating mounds of para- and orthokeratosis, and tiers of dyskeratotic cells (columnar dyskeratosis. Systemic corticosteroid therapy was not tolerated; acitretin diminished the hyperkeratosis. While hyperpigmentation persisted, no progression of cutaneous or muscular symptoms has occurred after 22 months of follow-up and cessation of the therapy. Overall, her course did not differ from the natural history documented in the literature review of Wong-type DM. The most similar case also exhibited pseudocornoid lamella changes. Conclusion: Wong-type DM is a clinicopathologic DM-PRP hybrid that can also exhibit porokeratosis-like features best described as columnar dyskeratosis. Recognizing these types of lesions in DM is warranted in order to make an accurate assessment of their prognostic significance.

  4. Brief Report: Power Doppler Ultrasonography for Detection of Increased Vascularity in the Fascia: A Potential Early Diagnostic Tool in Fasciitis of Dermatomyositis.

    Science.gov (United States)

    Yoshida, Ken; Nishioka, Makiko; Matsushima, Satoshi; Joh, Kensuke; Oto, Yosuke; Yoshiga, Masayuki; Otani, Kazuhiro; Ito, Haruyasu; Hirai, Kenichiro; Furuya, Kazuhiro; Ukichi, Taro; Noda, Kentaro; Kingetsu, Isamu; Kurosaka, Daitaro

    2016-12-01

    We previously demonstrated that fasciitis is a common lesion of dermatomyositis (DM) that is detectable early after disease onset by en bloc muscle biopsy combined with magnetic resonance imaging (MRI). Power Doppler ultrasonography (PDUS) is a useful method for detection of inflammation and vascularity in rheumatic diseases. We undertook this study to determine whether fasciitis was detectable by PDUS in patients with DM. We prospectively evaluated 7 patients with DM and 7 patients with polymyositis (PM) for the detection of fasciitis with PDUS. MRI and PDUS were both performed in all patients. Fasciitis was histologically confirmed by en bloc biopsy. Among all patients with DM, 4 showed signs of fasciitis on MRI, while increased blood flow signals were observed along the fascia by PDUS in 6 DM patients, including 4 patients with early disease (fascia. Immunohistochemical staining for CD31 indicated abnormal neovascular proliferation in the fascia in patients with DM. None of the PM patients showed signs of fasciitis or increased vascularity by MRI, PDUS, or en bloc biopsy. In our limited population, PDUS was useful for the detection of fasciitis associated with DM, especially in the early stage of disease. The increased blood flow signal as detected by PDUS is involved in angiogenesis accompanying fasciitis in patients with DM. © 2016, American College of Rheumatology.

  5. 皮肌炎/多发性肌炎合并肺间质性病变患者的护理%Nursing for dermatomyositis or polymyositis patients when combined with interstitial lung disease

    Institute of Scientific and Technical Information of China (English)

    金妙娟; 杜红卫

    2010-01-01

    @@ 皮肌炎(dermatomyositis,DM)和多发性肌炎(polymyositis,PM)是以横纹肌和/或皮肤受累为主的系统性自身免疫性疾病,病变可同时累及全身各组织器官,其中肺问质病变(interstitial lungdisease,ILD)是其常见的并发症,发生率为5%~30%~([1]).

  6. A Case of New-Onset Dermatomyositis in the Second Trimester of Pregnancy: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Tayfun Akalin

    2016-01-01

    Full Text Available Dermatomyositis (DM, a subtype of idiopathic inflammatory myopathies (IIMs, is characterized by skin rash, proximal muscle weakness, and inflammatory infiltrates in the muscle tissue. The peak incidence of the disease is at the age of 50–60 years, and only 14% of the patients with IIMs are estimated to present during reproductive years. Because of the limited pregnancy experience in patients with IIMs, little is known regarding the effects of DM on pregnancy or vice versa. We herein report a 40-year-old woman who developed DM in the second trimester of her pregnancy and did not respond to treatment with methylprednisolone. Her pregnancy was terminated at the 32nd week of gestation, due to preeclampsia and fetal distress. She delivered a healthy baby and improved rapidly after delivery. We have searched PubMed for relevant articles and reviewed previously published cases.

  7. Anti-MDA5 dermatomyositis mimicking psoriatic arthritis.

    Science.gov (United States)

    Cabezas-Rodríguez, Iván; Morante-Bolado, Isla; Brandy-García, Anahy; Queiro-Silva, Rubén; Mozo, Lourdes; Ballina-García, Francisco Javier

    2016-12-28

    Dermatomyositis causes inflammation and damage of muscle and skin, and sometimes involves internal organs, especially lung parenchyma. Patients with dermatomyositis still represent a diagnostic challenge because of the rarity of this disease and the lack of specificity of some of its cutaneous manifestations. Herein, we describe the case of a patient with dermatomyositis, initially diagnosed as psoriatic arthritis, in which the performance of anti-melanoma differentiation-associated gene 5 (MDA5) antibodies was decisive to establish a definitive diagnosis. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  8. Chronic cutaneous varicella zoster virus infection complicating dermatomyositis.

    Science.gov (United States)

    Hoesly, Fridolin J; Sluzevich, Jason C

    2014-04-01

    Chronic cutaneous varicella zoster virus (VZV) infection has not been previously reported or characterized as a complication of dermatomyositis. Two patients with non-malignancy-associated dermatomyositis, treated with long-term prednisone and methotrexate, developed persistent, painless ulcers ultimately established to be secondary to chronic VZV. The absence of pain or a history suggestive of acute VZV, and the lack of characteristic histopathology, resulted in a lengthy delay in diagnosis. Polymerase chain reaction and tissue immunohistochemistry were positive for VZV, and treatment with valacyclovir resulted in complete clearance. Diagnostic testing for VZV should thus be considered in the evaluation of ulcerative lesions in patients with dermatomyositis. The increased incidence of acute VZV in combination with the nature and duration of immunosuppressive treatment in this patient population may be contributory.

  9. Clinical significance of soluble CD163 in polymyositis-related or dermatomyositis-related interstitial lung disease.

    Science.gov (United States)

    Enomoto, Yasunori; Suzuki, Yuzo; Hozumi, Hironao; Mori, Kazutaka; Kono, Masato; Karayama, Masato; Furuhashi, Kazuki; Fujisawa, Tomoyuki; Enomoto, Noriyuki; Nakamura, Yutaro; Inui, Naoki; Suzuki, Daisuke; Ogawa, Noriyoshi; Nakashima, Ran; Mimori, Tsuneyo; Iwashita, Toshihide; Suda, Takafumi

    2017-01-19

    Macrophage activation is involved in the pathogenesis of polymyositis (PM)/dermatomyositis (DM). CD163, a scavenger receptor expressed on the surface of activated macrophages, mediates anti-inflammatory functions. This study aimed to evaluate the clinical significance of soluble CD163 (sCD163) in PM/DM-related interstitial lung disease (ILD). The main subjects were 48 patients with PM/DM-related ILD. As controls, 10 patients with PM/DM without ILD and 20 healthy volunteers were enrolled. In patients with PM/DM-related ILD, the baseline characteristics and clinical course were obtained through a review of patient medical records. Serum sCD163 levels at ILD diagnosis were quantified by enzyme-linked immunosorbent assay, which were compared with the other baseline clinical factors and evaluated for potential as a prognostic biomarker. In addition, immunohistochemistry analysis using anti-human CD163 antibody was performed on the lung sections of two patients with DM-related ILD (a survivor and non-survivor, respectively) and one patient with early-stage lung cancer as a normal control. The median value of serum sCD163 in patients with PM/DM-related ILD was 818 ng/mL, which was higher than that of PM/DM patients without ILD and healthy volunteers (716 ng/mL and 340 ng/mL, respectively). Significant but mild correlations with serum sCD163 levels were observed for serum C-reactive protein levels (r = 0.322) and % predicted forced vital capacity (r = -0.301) in patients with PM/DM-related ILD. A Cox proportional hazard model demonstrated that patients with PM/DM-related ILD and higher sCD163 levels had worse prognosis (age-adjusted and gender-adjusted hazard ratio per 100 ng/mL increase 1.27, 95% confidence interval 1.11-1.45, P CD163-positive macrophages was evident in the lungs of patients with DM-related ILD. Especially, the finding was more severe in the non-survivor's lung. Serum sCD163 might be a potential biomarker for predicting the severity and

  10. The role of high-dose immune globulin intravenous in the treatment of dermatomyositis.

    Science.gov (United States)

    Dalakas, Marinos C

    2006-04-01

    Dermatomyositis (DM) is a complement-mediated microangiopathy affecting skin and muscle resulting in skin abnormalities, including subcutenous calcifications, muscle weakness and disability. The disease generally responds to steroids or immunosuppressive drugs, but a number of patients are resistant or partially responsive to these therapies, prompting us to examine the efficacy of IVIg. A double-blind placebo-controlled study demonstrated that IVIg is very effective in improving both the muscle strength and the skin rash. The clinical benefit, which was impressive in patients with early disease, was associated with improvement in the muscle cytoarchitecture. Quantitative histological studies in repeated muscle biopsies showed a statistically significant increase in the size of muscle fibers and the number of capillaries with normalization of the capillary diameter. Resolution of the aberrant immunopathological parameters, including interception of complement activation products and downregulation of T cells, ICAM-I, VCAM, TGF-beta and MHC-I molecules, was also noted. Further, a number of immunoregulatory and structural genes were modified in the patients' muscle biopsies after therapy. The study concluded that IVIg is an effective second-line therapy for patients with DM incompletely responding to steroids.

  11. Clinical features of amyopathic dermatomyositis%无肌病性皮肌炎八例临床特征分析

    Institute of Scientific and Technical Information of China (English)

    周晓洁; 袁双龙; 周蕾; 巩路

    2010-01-01

    The clinical features of 8 cases with amyopathic dermatomyositis (ADM) wereretrospectively compared with those of 53 cases with typical dermatomyositis ( DM). Incidence of interstitiallung disease, joint pain, fever, concurrent cancer were 4/8, 3/8, 3/8 and 0 in ADM group and those were 40% , 34% , 32% and 11 % in DM group respectively. The prevalence of interstitial lung disease in ADMgroup was significantly higher than that in DM group, which indicates that ADM patient should be properlytreated.%收集近4年皮肌炎患者61例,其中8例无肌病性皮肌炎患者(观察组),53例典型皮肌炎患者(对照组),分析两组患者的临床特点.结果 示观察组肺问质病变、关节痛、发热者分别为4例、3例、3例;对照组肺间质病变、关节痛、发热、肿瘤者分别为21例(40%)、18例(34%)、17例(32%)和6例(11%).无肌病性皮肌炎者肺间质病变的发生率较高,虽没有明显肌病表现,但可累及内脏.

  12. Polymyositis and dermatomyositis associated with malignancy%多发性肌炎、皮肌炎与恶性肿瘤

    Institute of Scientific and Technical Information of China (English)

    徐文俊; 李惠

    2007-01-01

    多发性肌炎(polymyositis,PM)、皮肌炎(dermatomyositis,DM)常并发恶性肿瘤,其高危因素包括45岁以上男性、恶性红斑、无肌病症状、伴有系统损害、糖皮质激素反应差等.两者伴发的机制可能为:病毒感染、交叉免疫反应、免疫功能紊乱、遗传因素等.

  13. 皮肌炎患者合并恶性肿瘤的临床分析%Clinical analysis patients with dermatomyositis complicated with malignant tumor

    Institute of Scientific and Technical Information of China (English)

    谢红付; 朱武

    2000-01-01

    @@ 皮肌炎(dermatomyositis,DM)是一种结缔组织疾病,以皮肤和肌肉的炎症为主要表现,伴发恶性肿瘤的概率高.为了探讨湖南地区皮肌炎合并肿瘤的情况,我们回顾性分析了我科1990年~1998年住院病例的临床资料,现报道如下.

  14. 成人皮肌炎伴钙质沉积六例临床分析%The study of clinical manifestations of calcinosis in adult dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    江薇; 章璐; 王国春

    2015-01-01

    Objective To review the literature of calcinosis in adult dermatomyositis(DM)and find out the characters of DM.Methods We use the clinical data of 6 patients of calcinosis in adult dermatomyositis diagnosed in our hospital from Feburary,2008 to December,2014.Results Among 6 pa-tients,there were 1 male and 5 female.The mean age of 6 patients diagnosed dermatomyositis was(24 ±10) years and the average time of development of calcium deposition was(35 ±36)months.The most common site of calcium deposition was limbs,followed by trunk and armpits.3 cases complicated with femoral head necrosis and 1 case had nuclear matrix protein 2 (NXP-2)antibody positive.All of them were given pamidronate treatment,3 cases’patients were effectively treated.Conclusion Calcinosis in dermatomyo-sitis associated with earlier year of onset,longer duration,repeated activity of disease and positive NXP-2. Pamidronate is an apparently safe and effective treatment for calcinosis management in adult dermatomyositis.%目的:回顾相关文献并研究成人皮肌炎伴钙质沉积的病例特点。方法分析2008年2月~2014年12月我科收治的6例成人皮肌炎合并钙质沉积患者临床特点及治疗随访情况。结果6例患者男性1例,女性5例;皮肌炎平均起病年龄(24±10)岁;出现钙质沉积平均时间(35±36)个月;钙质沉积最常见出现的部位在四肢,其次在躯干和腋窝;3例合并股骨头坏死;1例抗核基质蛋白2(NXP-2)抗体阳性;6例患者均给予帕米膦酸钠治疗,3例治疗有效。结论起病年龄小、病程长、病情反复活动以及抗 NXP-2抗体阳性与皮肌炎钙质沉积有关,帕米膦酸钠治疗有效且无明显不良反应。

  15. "Sick sinus syndrome in dermatomyositis"--a case report.

    Science.gov (United States)

    Badui, E

    1987-11-01

    A forty-five-year-old woman with a history of dermatomyositis for the past fifteen years developed sick sinus syndrome during an exacerbation of her illness. This association has not been previously described in the literature, as far as the author can determine.

  16. Dermatomyositis som markør for lungecancer

    DEFF Research Database (Denmark)

    Petersen, Bibi; Bygum, Anette

    2009-01-01

    A 76-year-old female was referred because of violaceous dermatitis on sun-exposed skin. She had associated muscle weakness, dysphagia, dysarthria and reported an unintended weight loss. The clinical presentation gave a suspicion of dermatomyositis, and diagnostic procedures revealed a small-cell ...

  17. Positive whole-body /sup 67/Ga scintigraphy in dermatomyositis

    Energy Technology Data Exchange (ETDEWEB)

    Smith, W.P.; Robinson, R.G.; Gobuty, A.H.

    1979-07-01

    Gallium-67 has proven useful in the evaluation of the extent of several malignancies, including bronchogenic carcinoma, Hodgkin's disease, and lymphoma. Many infectious processes also yield positive scans, including sarcoidosis, pneumonia, pyelonephritis, and active tuberculosis. We report a patient in whom whole-body /sup 67/Ga scintimaging led to the diagnosis of dermatomyositis.

  18. Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis

    Energy Technology Data Exchange (ETDEWEB)

    Bonnefoy, Olivier; Ferretti, Gilbert; Calaque, Olivier; Coulomb, Max; Begueret, Hugues; Beylot-Barry, Marie; Laurent, Francois E-mail: francois.laurent@chu-bordeaux.fr

    2004-03-01

    Objective: A retrospective study was carried out in two institutions to determine serial changes in the pattern, distribution, and extent of interstitial lung disease (ILD) associated with polymyositis (PM)-dermatomyositis (DM) using HRCT. Subjects and methods: Twenty patients with PM-DM and clinical suspicion of ILD who underwent at least two serial HRCT examinations were retrospectively evaluated by two readers. Patients were classified according to the dominant CT pattern which was correlated with clinical evolution and underlying histology when available (n=6). Results: Patients were classified into four groups according to the dominant pattern: ground-glass attenuation and reticulation (group 1, n=9); honeycombing (group 2, n=4); airspace consolidation (group 3, n=4), and normal or almost normal lung (group 4, n=3). Under medical treatment, serial HRCT showed that the extent of areas of ground-glass opacities (group 1) decreased in five patients, stabilized in two, and increased in two. Pathologic findings demonstrated usual interstitial pneumonia (UIP) in two cases and unspecified interstitial pneumonia in one. In group 2, extent of honeycombing increased in three cases and stabilized in one. In group 3, dramatic resolution of airspace consolidation occurred in three cases. Clinical deterioration with extensive consolidation at CT and diffuse alveolar damage (DAD) at histology occurred in one patient of each of the three previous groups. Lesions stayed invisible or progressed slightly in the fourth group. Conclusion: In ILD associated with PM-DM, clinical deterioration and DAD in the follow-up can be observed whatever the HRCT pattern. However, unfavorable evolution is constant when honeycombing is present at the initial CT.

  19. Juvenile Scleroderma

    Science.gov (United States)

    Juvenile Scleroderma INTRODUCTION Every parent will experience a moment of panic when told their child has scleroderma. ... in all their family members as well. CONCLUSION Juvenile scleroderma can be unsettling for the child and ...

  20. Role of Toll-like receptors and retinoic acid inducible gene I in endogenous production of type I interferon in dermatomyositis.

    Science.gov (United States)

    Li, Ling; Dai, Tingjun; Lv, Jingwei; Ji, Kunqian; Liu, Junling; Zhang, Bin; Yan, Chuanzhu

    2015-08-15

    To explore the possible mechanisms implicated in the endogenous production of type I interferons within the muscle tissue of dermatomyositis (DM) patients. We detected the co-localization of plasmacytoid dendritic cells (pDCs) with Toll-like receptors (TLRs) and retinoic acid inducible gene (RIG)-I by immunohistochemistry and immunofluorescence. Western blotting confirmed the expression of TLRs and RIG-I. TLR-3 and RIG-I was preferentially expressed in the perifascicular atrophy fibers of DM. TLR-7 was only in inflammatory infiltrates of a few DM patients. TLR-4 and TLR-9 was expressed mainly in inflammatory infiltrates. Immunofluorescence showed extensive co-localization of BDCA-2 with TLR-9 and little co-localization with TLR-7. Western blotting showed upregulation of expression of TLRs and RIG-I in DM compared with the controls. Our findings indicate that endogenous production of type I IFN in DM is generated by pDCs, mainly through the TLR-9 pathway and in part by TLR-7. TLR-3 and RIG-I are implicated in the formation of perifascicular atrophy in DM.

  1. 1例以咳嗽气促为主诉的皮肌炎并文献复习%In 1 Cases with Cough Shortness of Breath with Chief Complaint of Dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    陈英

    2014-01-01

    Idiopathic inflammatory myopathies (I M) is a heterogeneous disease mainly af ecting proximal limb muscles . Polymyositis (PM) and dermatomyositis (DM) is the most common in I M. Organ damage within PM/DM is an important factor influencing the prognosis of the disease. We reported a case of dermatomyositis with pulmonary manifestations as the main performance, and combined with clinical review of literature data as fol ows.%特发性炎性肌病(idiopathic inflammatory myopathies,I M),是一组以四肢近端肌肉受累为突出表现的异质性疾病。其中以多发性肌炎(polymyositis,PM)和皮肌炎(dermatomyositis,DM)最为常见。 PM/DM伴器官损害是影响疾病预后的重要因素。现报道1例以肺部表现为主要表现的皮肌炎且治疗失败的病例,结合临床复习文献资料如下。

  2. A mild form of dermatomyositis as a prodromal sign of lung adenocarcinoma: a case report

    OpenAIRE

    Papakonstantinou, Eleni; Kapp, Alexander; Raap, Ulrike

    2016-01-01

    Background Dermatomyositis is an idiopathic connective tissue disease characterized by specific cutaneous findings and inflammatory lesions in the muscle biopsy. An association between dermatomyositis and malignancy, including breast, ovarian, lung and colon cancer was recognized many years ago, with an incidence of malignancy in approximately 20 % of cases. Dermatomyositis is hypothesized to be an autoimmune reaction against factors or hormones secreted by the tumor; however, the exact autoi...

  3. Dermatomyositis presenting with symptomatic dermographism and raised troponin T: a case report

    Directory of Open Access Journals (Sweden)

    Rahim Kartini F

    2009-07-01

    Full Text Available Abstract Introduction Dermatomyositis is an important inflammation of skin and muscles. Generalised itch is frequent in the condition; however, symptomatic dermographism has not previously been reported as a presenting feature. Case presentation A 32-year-old Caucasian Scottish woman was diagnosed with dermatomyositis after initial presentation with symptomatic dermographism. No underlying neoplasm was found and her condition was successfully treated with systemic corticosteroids and high-dose human immunoglobulin infusions. At presentation, her troponin T and creatine phosphokinase enzymes were highly raised. Conclusion Symptomatic dermographism may be a presenting feature of dermatomyositis. Dermatomyositis is one of many conditions that can result in a raised troponin T.

  4. Nasopharyngeal carcinoma in dermatomyositis patients: A 10-year retrospective review in Hospital Selayang, Malaysia

    Science.gov (United States)

    Teoh, J.W.; Yunus, Razif M.; Hassan, Faridah; Ghazali, Norazmi; Abidin, Zainal A.Z.

    2014-01-01

    Aim The objective of our review is to investigate the association between dermatomyositis patients and nasopharyngeal carcinoma (NPC) together with the clinical presentation of the patients and their management in otorhinolaryngology. Background NPC is a malignant disease with good prognosis on early diagnosis. However, the relationship between the dermatomyositis and NPC and its management is not well defined. Materials and methods A 10-year retrospective review of case records of 21 dermatomyositis patients seen in Otorhinolaryngology Department of Hospital Selayang from January 2000 to November 2010. Results These patients ranged from 19 to 74 years old and a total of 8 (38%) out of 21 adults with dermatomyositis were detected to have malignancy. Five out of 8 patients had NPC (62.5%). The mean age of patients with NPC and dermatomyositis was 48 years. NPC is diagnosed in 4 out of 5 patients (80%) in the first year of diagnosis of dermatomyositis. The clinical findings of the examination of nasopharynx ranged from hyperemia to exophytic nasopharyngeal mass. Histologically, it is only related to NPC of WHO types II and III. Conclusions There is a strong relationship between dermatomyositis and malignancy, especially NPC. Clinicians should have a high index of suspicion for malignancy in all dermatomyositis patients. Rigid nasoendoscopies and biopsies, serum Epstein–Barr viral capsid IgA antibody and imaging studies are helpful in detecting NPC in dermatomyositis patients. PMID:25184058

  5. Delusional and psychotic disorders in juvenile myotonic dystrophy type-1.

    Science.gov (United States)

    Jacobs, Delphine; Willekens, Diane; de Die-Smulders, Christine; Frijns, Jean-Pierre; Steyaert, Jean

    2017-06-01

    We investigated the clinically derived hypothesis of a relatively high incidence of delusional and psychotic disorders in adolescents with juvenile Myotonic Dystrophy type-1 (DM1). Twenty-seven subjects of age 16-25 with juvenile DM1 and their parents were invited to have a clinical psychiatric interview, and to complete an ASEBA behavior checklist (YSR, ASR, CBCL, and ABCL). We diagnosed a Delusional Disorder in 19% of our patients and a Psychotic Disorder not otherwise specified in another 19%. These two groups of patients had a significantly worse level of clinically defined general functioning. It is clinically relevant to investigate in patients with juvenile DM the symptom of delusions and the presence of a delusional and psychotic disorder, and to consider the presence of juvenile DM in youngsters presenting with such a thought disorder. These disorders compromise the general functioning of the subjects and are often to some extent treatable. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  6. Role of the Promoter Polymorphism IL-6 −174G/C in Dermatomyositis and Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Maria Hristova

    2013-01-01

    Full Text Available The promoter polymorphism −174G/C within the interleukin-6 gene (IL-6 has been reported to have a functional importance through the modulation of IL-6 gene expression in vitro and in vivo. IL-6 is thought to play an important role in autoimmune diseases and the effect of its receptor inhibitor—tocilizumab—has been recently studied. The aim of this case-control study was to investigate the association between the interleukin-6 −174G/C single nucleotide polymorphism and the susceptibility to dermatomyositis (DM and systemic lupus erythematosus (SLE in Bulgarian patients. Altogether, 87 patients—52 with SLE and 35 with DM—as well as 80 unrelated healthy controls were included in this study. All of them were analyzed by restriction fragment length polymorphism analysis (RFLP. The GG genotype and the G allele appeared to be associated with SLE, especially in women. None of the genotypes showed an association with DM. However, the G allele appeared to be associated with muscle weakness and it is a risk factor for elevated muscle enzymes. Our results indicate that IL-6 −174G/C polymorphism might be associated with the susceptibility to SLE especially in women. Although it is not associated with DM, it seems that IL-6 −174G/C polymorphism could modulate some clinical features in the autoimmune myopathies.

  7. Controlled studies with high-dose intravenous immunoglobulin in the treatment of dermatomyositis, inclusion body myositis, and polymyositis.

    Science.gov (United States)

    Dalakas, M C

    1998-12-01

    There are three major subsets of the inflammatory myopathies: polymyositis (PM), dermatomyositis (DM), and inclusion-body myositis (IBM). High-dose intravenous immunoglobulin (IVIg) has been tried in controlled clinical trials in patients with DM and IBM but not with PM. In patients with DM that is resistant or partially responsive to conventional therapies, IVIg was very effective. The treated patients experienced dramatic improvement not only in muscle strength but also of their skin rash. Repeated muscle biopsies with quantitative histologic studies showed the IVIg-treated patients had a statistically significant improvement of the muscle cytoarchitecture, with resolution of the aberrant immunopathologic parameters. In two controlled clinical trials conducted in IBM patients, IVIg showed marginal improvements in muscle strength which were nonsignificant. However, a few IBM patients had a definite clinical improvement with increased activities of daily living, but when analyzed within the entire IVIg-treated group, their total gains in muscle strength did not reach statistical significance compared to the placebo-treated group. Of interest is that certain muscle groups in the IVIg-treated patients, such as the muscles of swallowing, showed significant improvement compared to those of the placebo-treated patients, implying mild regional effects. In PM, uncontrolled trials have shown improvements in muscle strength, but the controlled clinical trial is still ongoing.

  8. Idiopathic Inflammatory Myopathies: an Update on Classification and Treatment with Special Focus on Juvenile Forms.

    Science.gov (United States)

    Pagnini, Ilaria; Vitale, Antonio; Selmi, Carlo; Cimaz, Rolando; Cantarini, Luca

    2017-02-01

    Juvenile inflammatory myopathies represent a heterogeneous group of rare and potentially fatal disorders of unknown aetiology, characterised by inflammation and proximal and symmetric muscle weakness. Beyond many similarities, specific clinical, laboratoristic and histopathologic features underlie different subsets with distinguishing demographic, prognostic and therapeutic peculiarities. Over time, several forms of inflammatory idiopathic myopathies have been described, including macrophagic myofascitis, immune-mediated necrozing myopathy and the spectrum of amyopathic dermatomyositis that include hypomyopathic dermatomyositis, inclusion body myositis and cancer-associated myositis occurring almost exclusively in adults. However, juvenile dermatomyositis is the most frequent in childhood, whereas polymyositis is relatively more frequent in adults. The aetiology is nowadays widely unclear; however, current theories contemplate a combination of environmental triggers, immune dysfunction and specific tissue responses involving muscle, skin and small vessels endothelium in genetically susceptible individuals. Myositis-specific autoantibodies, found almost exclusively in patients with myositis and myositis-associated autoantibodies, detectable both among patients with myositis and in subjects suffering from other autoimmune diseases, have an important clinical role because of their relation to specific clinical features, response to therapy and prognosis. The gold standard treatment for juvenile dermatomyositis is represented by corticosteroids, along with adjunctive steroid-sparing immunosuppressive therapies, which are used to counteract disease activity, prevent mortality, and reduce long-term disability. Further treatment approach such as biologic agents and autologous stem cell transplantation are emerging during the last years, in particular in patients difficult to treat and with poor prognosis. Therefore, a highly medical specialised approach is required for

  9. Dermatomyositis-like syndrome in x-linked agammaglobulinemia

    Directory of Open Access Journals (Sweden)

    Pedro David Carvalho

    2016-01-01

    Full Text Available Primary immunodeficiencies (PIDs encompass more than 250 different pathological conditions. X-linked agammaglobulinemia (XLA has been occasionally associated with cutaneous and muscular manifestations resembling dermatomyositis, often termed dermatomyositis-like syndrome (DLS. This syndrome has been associated with cutaneous, muscular and central nervous system manifestations, accompanying a persistent infection by an Echovirus. According to sixteen previously reported cases, this syndrome has a poor prognosis. We report the case of a 27-years old male, with XLA and DLS, successfully treated with 6 cycles of human immunoglobulin and methotrexate. Clinical symptoms improved dramatically with a complete resolution of the musculoskeletal manifestations. Despite this clinical response, prognosis should remain reserved. The evolution of this syndrome remains unpredictable and therapeutic options are limited. To the best of our knowledge, there are only a few reports of similar cases which have survived so many months after the diagnosis.

  10. Juvenile Judge

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    SHANG Xiuyun was among the first sitting judges when the juvenile court was set up in Beijing 10 years ago. With enriched experience she has altered the way judges ask questions in court. She began the practice of inviting juvenile offenders, their parents, relatives, friends and teachers to the juvenile court to work hand in hand in dealing with cases: Facing their relatives and friends and hearing their heartfelt words, juvenile offenders would often be touched, thus bringing forth a positive attitude toward life.

  11. Genetic association study of TNFAIP3, IFIH1, IRF5 polymorphisms with polymyositis/dermatomyositis in Chinese Han population.

    Directory of Open Access Journals (Sweden)

    Si Chen

    Full Text Available BACKGROUND: Single-nucleotide polymorphisms (SNPs in the TNFAIP3, IFIH1, and IRF5 genes have been associated with several auto-inflammation diseases, while the susceptibility between these genes and idiopathic inflammatory myopathies (IIMs were not reported. This study aimed to investigate whether TNFAIP3, IFIH1, and IRF5 gene polymorphisms confer susceptibility for the IIMs in Chinese Han population. METHODS: A large case-control study of Chinese subjects with polymyositis (PM (n = 298 and dermatomyositis (DM (n = 530 was accomplished. 968 healthy and ethnically matched controls were available for comparison. Six SNPs in the TNFAIP3 region (rs2230926 and rs5029939, the IFIH1 gene (rs1990760 and rs3747517 and the IRF5 region (rs4728142 and rs729302 were assessed and genotyped using the Sequenom MassArray iPLEX platform. RESULTS: Our study indicated a strong allele association was observed in PM/DM and PM patients for rs2230926 (OR: 1.61, 95%CI: 1.20-2.16, P(c = 7.5×10(-3; OR: 1.88, 95%CI: 1.30-2.74, P(c = 4.0×10(-3, respectively and rs5029939 (OR: 1.64, 95%CI: 1.21-2.21, P(c = 6.0×10(-3; OR: 1.88, 95%CI: 1.28-2.76, P(c = 5.5×10(-3, respectively. And rs2230926 and rs5029939 were significantly associated with interstitial lung disease (ILD in PM/DM and PM patients (P(c = 0.04 and P(c = 0.016; P(c = 0.02 and P(c = 0.03, respectively. In addition, rs4728142 allele and genotype had significant association with PM/DM patients (P(c = 0.026 and P(c = 0.048, respectively. Further analysis with three logistic regression genetic models revealed statistically significant difference in the genotypic distribution in the PM/DM, PM or DM patients when the additive and dominant models were used. CONCLUSIONS: This was the first study to reveal TNFAIP3 and IRF5 polymorphisms were associated with PM/DM patients or these patients with ILD, indicating that TNFAIP3 and IRF5 might be the susceptibility gene for PM/DM

  12. Juvenile Arthritis

    Science.gov (United States)

    Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

  13. Anti-MDA5-Positive Dermatomyositis Presenting as Fever of Unknown Origin.

    Science.gov (United States)

    Lee, Lori W; Narang, Neera S; Postolova, Anna; Seminara, Nicole; Kantor, Molly A

    2016-12-01

    Dermatomyositis is a chronic systemic autoimmune disease characterized by inflammatory infiltrates in the skin and muscle. The wide variability in clinical and serologic presentation poses a diagnostic challenge for the internist. Appreciation of the clinical variants of dermatomyositis allows for expedient diagnosis and avoidance of diagnostic error. We illustrate these challenges with the case of a 51-year-old Vietnamese-American man who initially presented with fever of unknown origin in the absence of overt skin and muscle manifestations. The diagnosis of dermatomyositis was not evident on several clinical encounters due to the absence of these hallmark symptoms. We review the variable clinical manifestations of a subtype of dermatomyositis associated with an autoantibody against melanoma differentiation-associated protein 5 (anti-MDA5) and suggest consideration of dermatomyositis as a diagnosis in patients presenting with systemic illness and markedly elevated ferritin, even in the absence of elevated muscle enzymes and classic autoantibodies.

  14. [Juvenile scleroderma].

    Science.gov (United States)

    de Mâcedo, Patrícia Andrade; Shinjo, Samuel Katsuyuki; Goldenstein-Schainberg, Cláudia

    2008-01-01

    Juvenile scleroderma is a rare childhood condition characterized by fibrosis of the skin and internal organs. Clinical manifestations of childhood scleroderma are different from adult disease and early recognition, correct classification and treatment can improve long-term outcome. This review explores the most recent actualizations on clinical manifestations, classification criteria, treatment options and prognosis of juvenile scleroderma. There are two main forms of the disease: localized scleroderma and systemic sclerosis. Localized scleroderma is the most common form in children and mostly restricted to the skin. Juvenile diffuse systemic sclerosis is related to visceral involvement and cardiac disease which is the main cause of death in these patients. The outcome of juvenile systemic sclerosis is better compared with the adult form. Treatment remains a medical challenge and the EULAR task force proposed an approach to juvenile scleroderma treatment based on expert's opinion and guidelines used for the treatment of adults. Larger studies on childhood scleroderma are warranted.

  15. Compare study of clinical features in polymyositis and dermatomyositis%多发性肌炎和皮肌炎临床特点分析

    Institute of Scientific and Technical Information of China (English)

    宣丹; 盛君; 李志; 毛桐俊; 徐亮; 陆进明

    2011-01-01

    AIM: To study the difference of clinical charactertics between polymyositis and dermatomyositis patients. METHODS: 11 cases of polymyositis and 21 cases of dermatomyositis were included in this study. Their muscle strength, serum creatine phosphokinase (CPK) levels, erythrocyte sedimentation rate (ESR),C-reactive protein (CRP), hospitalization times,the quadriceps muscle MRI and high-resolution lung CT (HRCT) were compared. RESULTS:There were no statistic differences among muscle strength, ESR, CRP, hospitalization times,hoarseness and dysphagia in the two groups(P>0.05). The level of CPK in polymyositis patients was significantly higher than that in dermatomyositis patients [(3032±2973)vs(390±947) U/L (P=0. 015)]. But the rate of incidence of interstitial pneumoniin in dermatomyositis patients was significantly higher than that in polymyositis patients (P = 0. 021), and the therapy effect of glucocorticoids in polymyositis patients was significantly better than that in dermatomyositis patients (P=0. 013). 4 deaths were all dermatomyositis patients, 3 cases died with interstitial pneumoniin developed respiratory failure and 1 cases died with rhabdomyolysis developed acute renal failure. CONCLUSION: The level of CPK in dermatomyositis patients is higher than polymyositis patients, but more complicated that in by interstitial pneumoniin and poor prognosis.%目的:对比分析多发性肌炎(PM)和皮肌炎(DM)的临床特征,提高临床认识、改善预后.方法:收集2006年8月至2009年8月间住院的PM和DM患者共32例,对其年龄、住院时间、肌力、血清肌酸磷酸激酶(CPK)水平、红细胞沉降率(ESR)、C反应蛋白(CRP)和股四头肌MRI、肺高分辩率CT(HRCT)进行比较分析.结果:DM组和PM组患者在年龄、肱二头肌力、股四头肌力、住院时间、ESR、CRP方面差异均无统计学意义(P>0.05).CPK水平PM组(3032±2973)U/L显著高于DM组(390±947)U/L,差异有统计学意义(P=0.015).DM和PM患者颈肌

  16. Dermatomyositis and Polymyositis in the Intensive Care Unit: A Single-Center Retrospective Cohort Study of 102 Patients.

    Directory of Open Access Journals (Sweden)

    Jin-Min Peng

    Full Text Available Patients with idiopathic inflammatory myopathies (IIMs are sometimes complicated with life-threatening conditions requiring intensive care unit (ICU admission. In the past, owing to the low incidence of IIM, little was known about such patients. Our aim was to investigate the clinical features and outcomes of these patients and identify their risk factors for mortality.A retrospective study was performed of IIM patients admitted over an 8-year period to the medical ICU of a tertiary referral center in China. We collected data regarding demographic features, IIM-related clinical characteristics, reasons for admission, organ dysfunction, and outcomes. Independent predictors of ICU mortality were identified through multivariate logistic regression analysis.Of the 102 patients in our cohort, polymyositis (PM, dermatomyositis (DM, and clinically amyopathic dermatomyositis (CADM accounted for 23.5%, 64.7%, and 11.7% respectively. The median duration from the onset of IIM to ICU admission was 4.3 months (interquartile range [IQR], 2.6-9.4 months. Reasons for ICU admission were infection alone (39.2%, acute exacerbation of IIM alone (27.5%, the coexistence of both (27.5%, or other reasons (5.8%. Pneumonia accounted for 97% of the infections; 63.2% of infections with documented pathogens were caused by opportunistic agents. Rapid progressive interstitial lung disease (RP-ILD was responsible for 87.5% of acute exacerbation of IIM. The median Acute Physiology and Chronic Health Evaluation II (APACHE II score on ICU day 1 was 17 (IQR 14-20. On ICU admission, acute respiratory failure (ARF was the most common type (80.4% of organ failure. The mortality rate in the ICU was 79.4%. Factors associated with increased ICU mortality included a diagnosis of DM (including CADM, a high APACHE II score, the presence of ARF, a decreased PaO2/FiO2 ratio, and a low lymphocyte count at the time of ICU admission.The outcome of IIM patients admitted to the ICU was extremely

  17. Picometer-Resolution MEMS Segmented DM Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Microelectromechanical systems (MEMS) technology has the potential to create deformable mirrors (DM) with 10^4 actuators that have size, weight, and power...

  18. Picometer-Resolution MEMS Segmented DM Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Microelectromechanical systems (MEMS) technology has the potential to create deformable mirrors (DM) with 10^4 actuators that have size, weight, and power...

  19. Thermodynamics of diffusive DM/DE systems

    Science.gov (United States)

    Haba, Z.

    2017-04-01

    We discuss the energy density, temperature and entropy of dark matter (DM) and dark energy (DE) as functions of the scale factor a in an expanding universe. In a model of non-interacting dark components we repeat a derivation from thermodynamics of the well-known relations between the energy density, entropy and temperature. In particular, the entropy is constant as a consequence of the energy conservation. We consider a model of a DM/DE interaction where the DM energy density increase is proportional to the particle density. In such a model the dependence of the energy density and the temperature on the scale factor a is substantially modified. We discuss (as a realization of the model) DM which consists of relativistic particles diffusing in an environment of DE. The energy gained by the dark matter comes from a cosmological fluid with a negative pressure. We define the entropy and free energy of such a non-equilibrium system. We show that during the universe evolution the entropy of DM is increasing whereas the entropy of DE is decreasing. The total entropy can increase (in spite of the energy conservation) as the DM and DE temperatures are different. We discuss non-equilibrium thermodynamics on the basis of the notion of the relative entropy.

  20. Beyond the MHC: A canine model of dermatomyositis shows a complex pattern of genetic risk involving novel loci

    Science.gov (United States)

    Evans, Jacquelyn M.; Hill, Cody M.; Anderson, Kendall J.

    2017-01-01

    Juvenile dermatomyositis (JDM) is a chronic inflammatory myopathy and vasculopathy driven by genetic and environmental influences. Here, we investigated the genetic underpinnings of an analogous, spontaneous disease of dogs also termed dermatomyositis (DMS). As in JDM, we observed a significant association with a haplotype of the major histocompatibility complex (MHC) (DLA-DRB1*002:01/-DQA1*009:01/-DQB1*001:01), particularly in homozygosity (P-val = 0.0001). However, the high incidence of the haplotype among healthy dogs indicated that additional genetic risk factors are likely involved in disease progression. We conducted genome-wide association studies in two modern breeds having common ancestry and detected strong associations with novel loci on canine chromosomes 10 (P-val = 2.3X10-12) and 31 (P-val = 3.95X10-8). Through whole genome resequencing, we identified primary candidate polymorphisms in conserved regions of PAN2 (encoding p.Arg492Cys) and MAP3K7CL (c.383_392ACTCCACAAA>GACT) on chromosomes 10 and 31, respectively. Analyses of these polymorphisms and the MHC haplotypes revealed that nine of 27 genotypic combinations confer high or moderate probability of disease and explain 93% of cases studied. The pattern of disease risk across PAN2 and MAP3K7CL genotypes provided clear evidence for a significant epistatic foundation for this disease, a risk further impacted by MHC haplotypes. We also observed a genotype-phenotype correlation wherein an earlier age of onset is correlated with an increased number of risk alleles at PAN2 and MAP3K7CL. High frequencies of multiple genetic risk factors are unique to affected breeds and likely arose coincident with artificial selection for desirable phenotypes. Described herein is the first three-locus association with a complex canine disease and two novel loci that provide targets for exploration in JDM and related immunological dysfunction. PMID:28158183

  1. Juvenile Prostitution.

    Science.gov (United States)

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  2. Juvenile Prostitution.

    Science.gov (United States)

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  3. Decreased miR-7 expression in the skin and sera of patients with dermatomyositis.

    Science.gov (United States)

    Oshikawa, Yuka; Jinnin, Masatoshi; Makino, Takamitsu; Kajihara, Ikko; Makino, Katsunari; Honda, Noritoshi; Nakayama, Wakana; Inoue, Kuniko; Fukushima, Satoshi; Ihn, Hironobu

    2013-05-01

    Expression of microRNA (miRNA) in the skin in dermatomyositis has not previously been studied in detail. In this study, we performed miRNA array analysis using miRNAs purified from dermatomyositis-involved skin and normal skin, and found that several miRNAs were up- or down-regulated in dermatomyositis skin. Among them, we focused on miR-7, one of the most down-regulated miRNAs in dermatomyositis skin. Total miRNAs were purified from serum, and hsa-miR-7 levels were measured with quantitative real-time PCR using the specific primer. Serum levels of miR-7 were significantly decreased in patients with dermatomyositis compared with normal subjects or patients with other autoimmune diseases. Thus, serum miR-7 levels might be a possible diagnostic marker for dermatomyositis. Clarifying the up- or down-stream events of down-regulated miR-7 in patients with dermatomyositis may lead to further understanding of the disease and a new therapeutic approach.

  4. Juvenile myasthenia

    Directory of Open Access Journals (Sweden)

    Knežević-Pogančev Marija

    2011-01-01

    Full Text Available Introduction. Juvenile myasthenia is a chronic autoimmune neuromuscular disease characterized by varying degrees of fluctuating, painless muscle weakness and rapid fatigue of any muscles under voluntary control. Juvenile myasthenia is a form of myasthenia appearing in adolescent age, representing 10% to 15% of all cases of myasthenia gravis. Juvenile myasthenia is presented by a defect in the transmission of nerve impulses to muscles, resulting from a breakdown in the normal communication between nerves and muscles. In myasthenia, antibodies produced by the body’s own immune system block, alter, or destroy the receptors for acetylcholine. Juvenile myasthenia is neither directly inherited nor is it contagious. Signs and Symptoms. The first noticeable symptoms may be eye muscle weakness, difficulty in swallowing, or slurred speech. Juvenile myasthenia usually affects muscles innervated by the cranial nerves (face, lips, tongue, neck and throat, but it can affect any muscle group. Symptoms vary in type and severity with typical periods of exacerbation interspersed with periods of remission. When the muscles necessary for breathing are affected, a patient is said to be in a myasthenic crisis, which is a life-threatening situation. Disease Outcome and Treatment. Juvenile myasthenia produces sporadic but progressive weakness and abnormal fatigability of striated (skeletal muscles, exacerbated by exercise and repeated movement, but improved by rest and anticholinesterase drugs. Juvenile myasthenia follows an unpredictable course of recurring exacerbations and periodic remissions. With current therapies, however, most cases of juvenile myasthenia are not as serious as the name implies. Although there is no known cure, drug treatment has improved prognosis and allows patients to lead relatively normal lives, except during exacerbations.

  5. Renal cell carcinoma-associated adult dermatomyositis treated laparoscopic nephrectomy

    Directory of Open Access Journals (Sweden)

    Elizabeth Nevins

    2013-01-01

    Full Text Available A 77-year-old female, who suffered from rheumatoid arthritis and hypothyroidism, developed severe muscle weakness. Clinical features, blood results and muscle biopsy suggested a possible diagnosis of dermatomyositis. A computed tomography of the chest, abdomen and pelvis showed a solid mass in the left kidney. She underwent a left laparoscopic nephrectomy and histology confirmed conventional (clear cell renal cell carcinoma. She recovered slowly and almost back to normal life after 6 months. Early appreciation of the typical skin rash may provide a clue to the diagnosis and screening for neoplasm may improve prognosis.

  6. Practice and Educational Gaps in Lupus, Dermatomyositis, and Morphea.

    Science.gov (United States)

    Fett, Nicole M; Fiorentino, David; Werth, Victoria P

    2016-07-01

    Patients with skin-predominant lupus erythematosus, dermatomyositis, and morphea should be evaluated, treated, and followed by dermatologists who can take primary responsibility for their care. Many academic centers have specialized centers with dermatologists who care for these patients. Patients with skin-predominant lupus erythematosus should be followed regularly with laboratory tests to detect significant systemic disease. Antibody tests can help determine the risks for individual patients. Patients with morphea rarely progress to systemic disease, but therapies can be helpful in treating and preventing progression of disease.

  7. Dermatomyositis and myastenia gravis: An uncommon association with therapeutic implications.

    Science.gov (United States)

    Sangüesa Gómez, Clara; Flores Robles, Bryan Josué; Méndez Perles, Clara; Barbadillo, Carmen; Godoy, Hildegarda; Andréu, José Luis

    2015-01-01

    The association of dermatomyositis with myasthenia gravis (MG) is uncommon, having been reported so far in only 26 cases. We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne. The patient developed acute proximal weakness, shoulder pain and elevated creatine-kinase (CK). He also developed generalized facial erythema and Gottron's papules. Laboratory tests showed positive antinuclear and anti-Mi2 antibodies. Further analysis confirmed CK levels above 1000 U/l. The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discusssed.

  8. Increased Expression of the NOD-like Receptor Family, Pyrin Domain Containing 3 Inflammasome in Dermatomyositis and Polymyositis is a Potential Contributor to Their Pathogenesis

    Institute of Scientific and Technical Information of China (English)

    Xi Yin; Gen-Cheng Han; Xing-Wei Jiang; Qiang Shi; Chuan-Qiang Pu

    2016-01-01

    Background:Dermatomyositis (DM) and polymyositis (PM) are common inflammatory myopathies whose immunopathogenic mechanisms remain poorly understood.The NOD-like receptor family,pyrin domain containing 3 (NLRP3) inflammasome is a type of cytoplasmic multiprotein inflammasome and is responsible for the activation of inflammatory reactivations.Responding to a wide range of exogenous and endogenous microbial or sterile stimuli,NLRP3 inflammasomes can cleave pro-caspase-1 into active caspase-1,which processes the pro-inflammatory cytokines pro-interleukin (IL)-1 β and pro-IL-18 into active and secreted IL-1 β and IL-18.The NLRP3 inflammasome is implicated in infectious and sterile inflammatory diseases.However,it remains unclear whether it is involved in the pathogenesis of DM/PM,which we aim to address in our research.Methods:In this study,22 DM/PM patients and 24 controls were recruited.The protein and RNA expression of IL-1β,IL-18,NLRP3,and caspase-1 in serum and muscle samples were tested and compared between the two groups.Results:The serum IL-1β and IL-18 levels were significantly higher in DM/PM patients than those in the controls by enzyme linked immunosorbent assay (ELISA,DM vs.control,25.02 ± 8.29 ng/ml vs.16.49 ± 3.30 ng/ml,P < 0.001; PM vs.control,26.49 ± 7.79 ng/ml vs.16.49 ± 3.30 ng/ml,P < 0.001).Moreover,the real-time quantitative reverse transcription-polymerase chain reaction (qRT-PCR) showed that DM/PM patients exhibited higher RNA expression of IL-1 β,IL-18,and NLRP3 in the muscle (for IL-1β,DM vs.control,P =0.0012,PM vs.control,P =0.0021; for IL-18,DM vs.control,P =0.0045,PM vs.control,P =0.0031; for NLRP3,DM vs.control,P =0.0017,PM vs.control,P =0.0006).Moreover,the protein expression of NLRP3 and caspase-1 in muscle samples of DM/PM patients were also significantly elevated compared to that in the muscles of the controls.Conclusions:Our findings demonstrate that the NLRP3 inflammasome is implicated in the pathogenesis of DM

  9. Consequences of DM/antiDM oscillations for asymmetric WIMP darkmatter

    DEFF Research Database (Denmark)

    Cirelli, M.; Panci, P.; Servant, G.

    2012-01-01

    Assuming the existence of a primordial asymmetry in the dark sector, a scenario usually dubbed Asymmetric Dark Matter (aDM), we study the effect of oscillations between dark matter and its antiparticle on the re-equilibration of the initial asymmetry before freeze-out, which enable efficient...... a primordial asymmetry of the same order as the baryon asymmetry naturally gets the correct relic abundance if the DM-number-violating Delta(DM) = 2 mass term is in the similar to meV range. The re-establishment of annihilations implies that constraints from the accumulation of aDM in astrophysical bodies...

  10. Consequences of DM/antiDM oscillations for asymmetric WIMP darkmatter

    DEFF Research Database (Denmark)

    Cirelli, M.; Panci, P.; Servant, G.;

    2012-01-01

    Assuming the existence of a primordial asymmetry in the dark sector, a scenario usually dubbed Asymmetric Dark Matter (aDM), we study the effect of oscillations between dark matter and its antiparticle on the re-equilibration of the initial asymmetry before freeze-out, which enable efficient...... annihilations to recouple. We calculate the evolution of the DM relic abundance and show how oscillations re-open the parameter space of aDM models, in particular in the direction of allowing large (WIMP-scale) DM masses. A typical WIMP with a mass at the EW scale (similar to 100 GeV - 1TeV) presenting...

  11. Increasing recognition of dermatomyositis with subcutaneous edema - is this a poorer prognostic marker?

    Science.gov (United States)

    Tu, Jenny; McLean-Tooke, Andrew; Junckerstorff, Reimar

    2014-01-15

    Subcutaneous edema as a presenting feature of dermatomyositis has infrequently been described and is thought to signify a more aggressive disease course. We report a case involving a 38-year-old man who presented with significant subcutaneous edema involving his neck and upper body; he later developed clinical features and biopsy results consistent with dermatomyositis. Only sixteen previous cases of dermatomyositis with subcutaneous edema involving adults have been published in the literature and we aim to review disease progression, prognosis, and optimal treatment of the condition.

  12. Juvenile idiopathic arthritis

    Science.gov (United States)

    Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... The cause of juvenile idiopathic arthritis (JIA) is not known. It ... illness . This means the body attacks and destroys healthy body ...

  13. Juvenile Polyposis Syndrome

    Science.gov (United States)

    ... Types of Cancer > Juvenile Polyposis Syndrome Request Permissions Juvenile Polyposis Syndrome Approved by the Cancer.Net Editorial Board , 12/2015 What is juvenile polyposis syndrome? Juvenile polyposis syndrome (JPS) is a ...

  14. Dermatomyositis, polymyositis and immune-mediated necrotising myopathies.

    Science.gov (United States)

    Luo, Yue-Bei; Mastaglia, Frank L

    2015-04-01

    Dermatomyositis, polymyositis and immune-mediated necrotising myopathy are major forms of idiopathic inflammatory myopathy. We review here recent developments in understanding the pathology and pathogenesis of these diseases, and characterisation of autoantibody biomarkers. Dermatomyositis is traditionally considered to be due to a complement-mediated microangiopathy but the factors responsible for complement activation remain uncertain. Recent studies have emphasised the importance of the type I interferon pathway in the pathogenesis of the disease and have identified autoantibodies with specificities for different clinical subgroups of patients. Polymyositis is characterised by a cytotoxic T cell response targeting as yet unidentified muscle antigens presented by MHC Class I molecules, and can occur in isolation but is more often part of a multi-systemic overlap syndrome. The immune-mediated necrotising myopathies are heterogeneous and are distinguished from polymyositis by the sparseness of inflammatory infiltrates and recognition of an association with specific autoantibodies such as anti-SRP and anti-HMGCR in many cases. This article is part of a Special Issue entitled: Neuromuscular Diseases: Pathology and Molecular Pathogenesis.

  15. Medical resource utilization in dermatomyositis/polymyositis patients treated with repository corticotropin injection, intravenous immunoglobulin, and/or rituximab.

    Science.gov (United States)

    Knight, Tyler; Bond, T Christopher; Popelar, Breanna; Wang, Li; Niewoehner, John W; Anastassopoulos, Kathryn; Philbin, Michael

    2017-01-01

    Dermatomyositis and polymyositis (DM/PM) are rare, incurable inflammatory diseases that cause progressive muscle weakness and can be associated with increased medical resource use (MRU). When corticosteroid treatment is unsuccessful, patients may receive intravenous immunoglobulin (IVIg), rituximab, or repository corticotropin injection (RCI). This study compared real-world, non-medication MRU between patients treated with RCI and those treated with IVIg and/or rituximab for DM/PM. Claims of DM/PM patients were analyzed from the combination of three commercial health insurance databases in the United States from July 2009 to June 2014. Patients treated with RCI were propensity score matched to those treated with IVIg, rituximab, and both (IVIg+rituximab) based on demographics, prior clinical characteristics, and prior MRU. Per-patient per-month (PPPM) MRU and costs were compared using Poisson regression and generalized linear modeling, respectively. One-hundred thirty-two RCI, 1,150 IVIg, and 562 rituximab patients had an average age of 52.6, 46.6, and 51.7 years, respectively, and roughly two-thirds were female. After matching, there were no significant differences in demographics or prior clinical characteristics. RCI patients had fewer PPPM hospitalizations (0.09 vs 0.17; P=0.049), shorter length of stay (LOS; 3.24 days vs 4.55 days; P=0.004), PPPM hospital outpatient department (HOPD) visits (0.60 vs 1.39; PMRU and costs than those treated with IVIg and/or rituximab, particularly in the hospital setting where significant costs are incurred.

  16. Rituximab as a first-line agent for the treatment of dermatomyositis.

    LENUS (Irish Health Repository)

    2012-02-01

    B cells may play a pivotal role in the pathophysiology of DM, and reports have claimed that targeting B cells is a viable treatment option in patients with dermatomyositis. A 20-year-old girl presented in October 2007, with few weeks\\' history of proximal muscle weakness. Gottron\\'s papules were noted on her knuckles. She had normal inflammatory markers and negative autoantibody screen. Her CPK was 7,000 U\\/L (normal range 0-170) with an LDH of 1,300 U\\/L (normal range 266-500). EMG and muscle biopsy was consistent with active myositis. She had normal pulmonary function tests. HRCT showed no interstitial lung disease. She was started with 60 mg glucocorticoids (1 mg\\/kg), with a good clinical response. However, any attempt to taper down the steroid dose led to recurrence of her symptoms. The options of available immunosuppressive therapies, including the experimental usage of rituximab, were discussed with her; averse to long-term systemic treatments, she opted to try a course of rituximab. She had rituximab 1,000 mg on days 0 and 14, and her glucocorticoids were tapered in next few weeks. Now, 24 months since her rituximab infusions, she remains in complete clinical and biochemical remission and is naive to other immunosuppressive agents apart from glucocorticoids and rituximab. Depleting peripheral B cells with rituximab (one course) in our patient has led not only to complete resolution of muscle and skin disease (induction) but also remains off all immunosuppressives including glucocorticoids.

  17. Dermatomyositis and clinically amyopathic dermatomyositis complicated with interstitial lung disease: clinical analysis of 43 cases%43例皮肌炎和临床无肌病型皮肌炎合并肺间质病变的临床分析

    Institute of Scientific and Technical Information of China (English)

    周琳; 曹华; 郑捷

    2014-01-01

    目的:探讨皮肌炎(dermatomyositis,DM)和临床无肌病性皮肌炎(clinically amyopathic dermatomyositis,CADM)合并肺间质病变(interstitial lung disease,ILD)患者的胸部影像学表现与临床特征、实验室检查及预后的关系.方法:收集43例DM/CADM合并ILD患者的临床资料,根据肺高分辨率CT结果将患者分为2组,G1组患者的肺高分辨率CT影像主要表现为网格影及纤维条索影,G2组则主要表现为磨玻璃样、蜂窝状病变.比较2组间的临床特征、实验室检查及预后.结果:G2组患者发热、低氧血症的发生率、铁蛋白、乳酸脱氢酶水平、死亡率显著高于G1组:而G2组患者血清肌酸激酶水平、肌电图及肌肉活检阳性率均显著低于G1组.结论:在合并ILD的DM/CADM患者中,肺高分辨率CT影像特征与ILD病情密切相关,以网格影及纤维条索影为主的患者ILD病情轻,预后好,而磨玻璃样、蜂窝状病变为主的患者ILD病情重、预后差.

  18. Amyopathic Dermatomyositis: A Concise Review of Clinical Manifestations and Associated Malignancies.

    Science.gov (United States)

    Udkoff, Jeremy; Cohen, Philip R

    2016-10-01

    Amyopathic dermatomyositis is a rare, idiopathic, connective tissue disease that presents with dermatologic lesions of classic dermatomyositis but lacks the myopathy of this disease. Cutaneous manifestations may include Gottron's sign, heliotrope rash, and characteristic patterns of poikiloderma. There is a substantial risk for developing interstitial lung disease or malignancy in patients with amyopathic dermatomyositis. A literature review of amyopathic dermatomyositis was performed using the PubMed medical database. The key features of amyopathic dermatomyositis, including autoantibodies, clinical presentation and dermatologic manifestations, epidemiology, history, associated malignancies, management, and pathogenesis, are summarized in this review. Cancer (solid tumor) (73/79, 89 %) and hematologic malignancies (9/79, 11 %) were reported in 79 patients, with three patients having more than one malignancy. In addition, there were six patients with amyopathic dermatomyositis who had tumor of unknown primary, and eight patients with cancer-associated amyopathic dermatomyositis for whom no additional details were provided. From the group of 73 tumors for whom primary origin and sex were available, malignancy of the genitourinary organs (24/73, 33 %), aerorespiratory organs (15/73, 21 %), and breast (14/73, 19 %) were the most commonly observed solid organ tumors. Tumors of the genitourinary organs (15/48, 31 %) and breast (14/48, 29 %) were the most frequent neoplasms in women, accounting for 29 of 48 (60 %) cancers, with the most common sites being breast (14/48, 29 %), ovary (8/48, 17 %), and cervix or uterus (5/48, 10 %). In men, tumors of the aerorespiratory (9/25, 36 %) and genitourinary (9/25, 36 %) tracts were the most common neoplasms, accounting for 72 % (18/25) of cancers; the most common sites of primary malignancy were nasopharyngeal (6/25, 24 %), bladder (4/25, 16 %), and either colorectal, lung or prostate cancer (three cancers each

  19. Paraneoplastic SIADH and Dermatomyositis in Cervical Cancer: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Guy Jones

    2009-11-01

    Full Text Available We present the first known case of a patient with cervical squamous cell carcinoma complicated by paraneoplastic syndromes of both dermatomyositis and inappropriate secretion of antidiuretic hormone (SIADH. The patient in this case presented with generalized body pain and vaginal bleeding. Her cervical cancer was diagnosed as stage IIB by physical exam, imaging, and cervical biopsy, her dermatomyositis was confirmed by muscle and skin biopsy, and her SIADH was diagnosed based on laboratory findings.

  20. Dermatomyositis presenting with symptomatic dermographism and raised troponin T: a case report

    OpenAIRE

    Rahim Kartini F; Dawe Robert S

    2009-01-01

    Abstract Introduction Dermatomyositis is an important inflammation of skin and muscles. Generalised itch is frequent in the condition; however, symptomatic dermographism has not previously been reported as a presenting feature. Case presentation A 32-year-old Caucasian Scottish woman was diagnosed with dermatomyositis after initial presentation with symptomatic dermographism. No underlying neoplasm was found and her condition was successfully treated with systemic corticosteroids and high-dos...

  1. Serum IL8 and mRNA level of CD11b in circulating neutrophils are increased in clinically amyopathic dermatomyositis with active interstitial lung disease.

    Science.gov (United States)

    Zou, Jing; Chen, Jie; Yan, Qingran; Guo, Qiang; Bao, Chunde

    2016-01-01

    The objective of this study is to assess serum IL8 and the potential activity of circulating neutrophils on relative messenger RNA (mRNA) levels and their relationship with disease activity in clinically amyopathic dermatomyositis (CADM) associated with interstitial lung disease (ILD). We studied 18 CADM patients and compared them with 18 classic dermatomyositis (DM) patients and 18 healthy control subjects. Serum IL8 level and mRNA expressions of neutrophils (chemokine (C-X-C motif) receptor 1 (CXCR1), cluster of differentiation molecule 11b (CD11b), cluster of differentiation 64 (CD64), myeloid cell leukemia 1 (MCL1), interleukin-18 (IL18)) were detected. The overproduction of serum IL8 level was most significant in the CADM group with active period. The mRNA expressions of CD11b, IL18, and MCL1 were greatly increased in the neutrophils in patients with CADM compared with DM or healthy controls. Up-expressions of CD11b, IL18, and MCL1 were detected in the neutrophils in CADM patients of active period compared with remission period. A positive correlation was found between CD11b mRNA level and high-resolution computed tomography (HRCT) score, in CADM associated with ILD. Serum IL8 level and mRNA levels of CD11b, MCL1, and IL18 in circulating neutrophils are related with the disease activity of CADM-ILD. The mRNA level of CD11b is positively correlated with HRCT score in CADM-ILD.

  2. Disease course and therapeutic approach in dermatomyositis: A four-center retrospective study of 100 patients.

    Science.gov (United States)

    Johnson, Nicholas E; Arnold, W David; Hebert, Donald; Gwathmey, Kelly; Dimachkie, Mazen M; Barohn, Richard J; McVey, April L; Pasnoor, Mamatha; Amato, Anthony A; McDermott, Michael P; Kissel, John; Heatwole, Chad R

    2015-08-01

    Dermatomyositis is a life-altering inflammatory disorder of skin and muscle. Details regarding the natural course of this disorder, the effects of specific therapies on its progression, and the optimal therapeutic dosage and duration of prednisone are limited. We performed a retrospective medical record review of dermatomyositis patients at four medical centers. All patients were over the age of 21 and had a clinical diagnosis of dermatomyositis with pathological confirmation. We reviewed average muscle strength, corticosteroid use, creatine kinase levels, and supplemental immunosuppressant use during the 36-month period following each patient's initial assessment. One hundred patients participated with an average age of 50.1 years. Average muscle strength improved and prednisone requirements lessened six months after initial assessment. There was no difference in the mean change in muscle strength or cumulative corticosteroid use over 36 months among those initially treated with methotrexate, mycophenolate mofetil, pulse IVIG, or azathioprine. There was a 5% mortality rate in dermatomyositis patients due to infections. Treated dermatomyositis patients demonstrate the most significant improvement in strength during the first six-to-twelve months following their initial clinical assessment. Additional prospective studies are needed to determine the relative benefit of select immunosuppressant agents in preserving strength and reducing corticosteroid use in dermatomyositis.

  3. A rarity that can lead to a casualty - A retrospective study of 12 cases of Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Matilda Naesström

    2015-09-01

    Full Text Available Aims: Lesions of the skin are omnipresent in Internal Medicine practice. The varying etiopathology when facing multiple system involvement may pose a challenge when it comes to diagnostics and management, especially when faced with less common skin diseases. Dermatomyositis is a rare skin disorder that manifests on the skin and in muscle; it also comes with a higher risk of comorbid cancers. Therefor we present the cases of dermatomyositis diagnosed at our departmet during the last 17 years, with the specific attention to ocurrance of oncological processes. Method: A retrospective study was performed on 12 cases hospitalized between 1996 to 2011 due to dermatomyositis. The analysis was based on the course of the disease, clinical picture, treatment and frequency of neoplasms. Results: Within those 12 patients (in addition to dermatomyositis five patients had concomitant oncological process. The tumors of these five patients were located in discrete anatomical locations. The oncological process occured before, during, or after the appearance of dermatomyositis. Conclusions: The combination of hallmark signs and symptoms seen in dermatomyositis are specific for the disease. Physicians need to be better informed about this rare, yet important disease, because it can be considered a paraneoplastic process.

  4. Pulmonary function and autoantibodies in a long-term follow-up of juvenile dermatomyositis patients

    DEFF Research Database (Denmark)

    Mathiesen, Pernille Raasthøj; Buchvald, Frederik Fouirnaies; Nielsen, Kim G

    2014-01-01

    Objectives. Pulmonary disease is a rare complication in JDM, described in only a few studies. This long-term follow-up study aimed to (i) describe pulmonary involvement in a national cohort of JDM patients estimated by conventional spirometry, (ii) compare pulmonary impairment with overall JDM ou...

  5. Early detection of colon cancer by increased serum level of Krebs von den Lungen-6 in a patient with dermatomyositis-associated interstitial pneumonia.

    Science.gov (United States)

    Fukuhara, Naoko; Tanino, Yoshinori; Sato, Suguru; Fukuhara, Atsuro; Uematsu, Manabu; Nikaido, Takefumi; Misa, Kenichi; Sato, Yasuko; Saito, Junpei; Wang, Xintao; Munakata, Mitsuru

    2015-09-14

    Krebs von den Lungen-6 (KL-6) is a high-molecular-weight glycoprotein which is elevated in serum of patients with interstitial pneumonia (IP). Serum KL-6 level is clinically used for the diagnosis of IP as well as the evaluation of its disease activity. KL-6 is originally identified when exploring novel soluble antigens in patients with lung cancer, and is known to be elevated in patients with several malignant tumors. The risk of malignant tumors is high in IP patients with polymyositis and dermatomyositis (PM/DM), and follow-up of KL-6 levels may allow earlier detection of such tumors. However, to date, there are only a few reports showing the usefulness of following-up serum KL-6 levels for finding malignant tumors in IP patients with PM/DM. Here, we described the first patient in whom increased serum KL-6 led to the diagnosis of colon cancer during follow-up of DM-associated IP.

  6. Juvenile Spondyloarthritis

    Science.gov (United States)

    Gmuca, Sabrina; Weiss, Pamela F.

    2015-01-01

    Purpose of review To provide a comprehensive update of the pathogenesis, diagnostic imaging, treatments, and disease activity measurements of juvenile spondyloarthritis (JSpA). Recent findings Genetic and microbiome studies have provided new information regarding possible pathogenesis of JSpA. Recent work suggests that children with JSpA have decreased thresholds for pain in comparison to healthy children. Additionally, pain on physical examination and abnormalities on ultrasound of the entheses are not well correlated. Treatment guidelines for juvenile arthritis, including JSpA, were published by the American College of Rheumatology and are based on active joint count and presence of sacroiliitis. Recent studies have established the efficacy of tumor necrosis factor inhibitors in the symptomatic treatment of axial disease, though their efficacy for halting progression of structural damage is less clear. Newly developed disease activity measures for JSpA include the Juvenile Arthritis Disease Activity Score and the JSpA Disease Activity index. In comparison to other categories of juvenile arthritis, children with JSpA are less likely to attain and sustain inactive disease. Summary Further microbiome and genetic research may help elucidate JSpA pathogenesis. More randomized therapeutic trials are needed and the advent of new composite disease activity measurement tools will hopefully allow for the design of these greatly needed trials. PMID:26002028

  7. Clinical analysis and immunological characteristics of patients with dermatomyositis and thyroid dysfunction%皮肌炎合并甲状腺功能异常的临床及免疫学特征分析

    Institute of Scientific and Technical Information of China (English)

    姚海红; 李玉慧; 张学武; 栗占国

    2011-01-01

    Objective:To study the clinical and immunological features of dermatomyositis (DM) com plicated with thyroid dysfunction. Methods: Between 1993 and 2008, dermatomyositis was diagnosed in 70 patients referred to the Department of Rheumatology of Peking University People' s Hospital. Clinical and laboratory data including antibodies, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) , immunoglobulin, protein electrophoresis, and serum lipid levels, etc. were retrospectively analyzed. Results: 48% of DM patients had their thyroid function impaired, most of whom suffered from subclinical hypothyroidism. DM patients with thyroid dysfunction were more likely to have higher levels of CRP and IgG as well as higher incidences of fever and erythra. Conclusion: Subclinical thyroid dysfunction with DM is not rare. Common immunological background may be responsible for this phenomenon and it should not be neglected.%目的:探讨合并甲状腺功能异常的皮肌炎(dermatomyositis,DM)患者临床及免疫学特征.方法:回顾性分析70例DM患者的临床表现、肌酶水平、自身抗体、红细胞沉降率(erythrocyte sedimentation rate,ESR)、C反应蛋白(C-reactive protein,CRP)、免疫球蛋白、血脂等指标,依据甲状腺功能情况进行分组比较.结果:70例DM患者中甲状腺功能异常者达48%,主要表现为甲状腺功能减低,但患者多无甲状腺功能减低的临床症状.与甲状腺功能正常组比较,甲状腺功能异常组的患者多见发热、V型征、Gottron征,也常出现CRP、IgG升高,两组患者系统器官受累情况及肿瘤发生率比较差异无统计学意义.结论:DM患者甲状腺功能异常的发生率高,常表现为亚临床型甲状腺功能减低,可能与两种疾病存在共同的免疫机制有关,临床上应予重视.

  8. 皮肌炎/多发性肌炎病因及发病机制的研究进展%Advancement in the Study of Pathogenesis of Dermatomyositis/Polymyositis

    Institute of Scientific and Technical Information of China (English)

    谭艳平; 刘志刚

    2016-01-01

    皮肌炎(dermatomyositis,DM)和多发性肌炎(polymyositis,PM)是一组主要累及皮肤和横纹肌的自身免疫性皮肤病.目前确切病因尚不清楚,认为可能与遗传、感染、恶性肿瘤和免疫因素等相关.近年来,随着对该病遗传学研究的不断深入,对其发病机制有了更进一步的认识,本文将重点从遗传学方面对DM/PM的病因及发病机制的进展做一综述.

  9. Medical image of the week: subcutaneous calcification in dermatomyositis

    Directory of Open Access Journals (Sweden)

    Natt B

    2016-12-01

    Full Text Available A 36-year old woman was referred to our Interstitial Lung Disease (ILD clinic for evaluation of dyspnea. A high-resolution CT scan of the chest showed perivascular reticular and ground glass opacities with air trapping, consistent with non-specific interstitial pneumonitis (Figure 1. She was diagnosed with connective tissue associated ILD. On review of previous images extensive subcutaneous calcifications were seen (Figure 2. Calcinosis is an uncommon manifestation of dermatomyositis in adults (1. It is usually seen around areas of frequent trauma like the hands and elbows. In her case, a pelvic inflammatory disease may have been a trigger for this calcinosis. Calcinosis is a difficult complication to treat with some success seen with diltiazem, aluminum hydroxide, and even alendronate in children. Surgical excision may be required in some cases.

  10. Serum level of DNase1l3 in patients with dermatomyositis/polymyositis, systemic lupus erythematosus and rheumatoid arthritis, and its association with disease activity.

    Science.gov (United States)

    Zhao, Qi; Yang, Chunshu; Wang, Jianing; Li, Yujia; Yang, Pingting

    2016-12-30

    DNase1l3 is an endonuclease to degrade the chromatin of apoptotic or necrotic cells. Serum DNase1l3 may fulfill the function of clearance of chromatin released into the circulation by dying cells, which can trigger autoimmune responses. To date, it remains unclear whether serum DNase1l3 level associates with the pathogenesis of autoimmune diseases. Sixty-eight patients with dermatomyositis/polymyositis (DM/PM, n = 30), systemic lupus erythematosus (SLE, n = 20) and rheumatoid arthritis (RA, n = 18), as well as 26 healthy blood donors were enrolled in the present study. Serum levels of DNase1l3 were quantified by enzyme-linked immunosorbent assay. DNASE1L3 activity in serum was estimated by the capability of serum to digest nucleosomal DNA. Clinical, biochemical, serological and other markers of disease activity (CRP, ESR, C3, C4, anti-Jo-1 and anti-dsDNA, etc.) were measured by standard laboratory procedure. We found a decrease in DNase1l3 level in the DM/PM and SLE patients, resulting in the reduction in serum activity to digest nucleosome DNA. In contrast, the level and activity of DNase1l3 remained unchanged in the RA patients. The DNase1l3 level was relatively lower in the DM/PM patients with anti-Jo-1 antibody and interstitial lung disease, and in the SLE patients with SLE disease activity index higher than 6, renal involvement and anti-dsDNA antibody. DNase1l3 level negatively correlated with CRP and IgG in the PM/DM patients and correlated with ESR in the SLE patients. We found a significant reduction in serum DNase1l3 level in DM/PM and SLE, which may associate with clinic features and disease activity.

  11. TI DM270 PMP解决方案

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    @@ TI PMP特点 TI提供系列PMP解决方案,包括TI DM270/DM275/DM320/DM342/DM420等数字媒体处理器及不同操作系统(linux或wince等)的PMP方案.另外特别值得关注的是微软公司和TI合作推出了PMC(便携式媒体中心)-Ⅱ代.

  12. Juvenile Justice in Milwaukee

    Science.gov (United States)

    Williams, Gary L.; Greer, Lanetta

    2010-01-01

    Historically, there have been several attempts made to address issues surrounding juvenile delinquency. The Wisconsin Legislature outlines the objectives of the juvenile justice system in the Juvenile Justice Code in s. 939.01, ?to promote a juvenile justice system capable of dealing with the problem of juvenile delinquency, a system which will…

  13. Sex differential association of dermatomyositis with Sjögren syndrome

    Science.gov (United States)

    Tseng, Chia-Chun; Chang, Shun-Jen; Tsai, Wen-Chan; Ou, Tsan-Teng; Wu, Cheng-Chin; Sung, Wan-Yu; Hsieh, Ming-Chia; Yen, Jeng-Hsien

    2017-01-01

    BACKGROUND: Although dermatomyositis and Sjögren syndrome share serologic autoantibodies and genetic polymorphisms, population data about the incidence of Sjögren syndrome in patients with dermatomyositis is unavailable. We performed a nationwide cohort study to explore the potential relation between dermatomyositis and Sjögren syndrome and, if an association exists, to elucidate whether it varies by sex. METHODS: We identified all patients with newly diagnosed dermatomyositis from the Registry of Catastrophic Illness Database in Taiwan between Jan. 1, 1998, and Dec. 31, 2011. Each patient was matched to, at most, 5 control patients from the National Health Insurance Research Database by age, sex and entry date. Cox regression was used to calculate the hazard ratio (HR) and 95% confidence interval (CI) of Sjögren syndrome after adjusting for age, sex, rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis. RESULTS: A total of 1602 patients with dermatomyositis and 7981 control patients were enrolled in the study. There was a positive association of having Sjögren syndrome among patients with dermatomyositis after adjusting for age, sex, rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis (HR 2.67, 95% CI 2.01–3.54). The association was more pronounced in the male cohort (HR 2.69, 95% CI 1.19–6.09). INTERPRETATION: We found a sex differential association of Sjögren syndrome among patients with dermatomyositis independent of age and concomitant autoimmune disease. Further studies are required to determine the clinical importance of this association for both outcomes and therapeutic options. PMID:28246264

  14. Juvenile xanthogranuloma.

    Science.gov (United States)

    Singh, R; Ghazali, W

    1992-05-01

    Juvenile xanthogranuloma is a benign cutaneous growth presenting as papules or nodules. It is characterized by an intradermal collection of lipid-laden macrophages and varying degrees of fibroblastic proliferation. We have recently observed two patients with xanthogranulomas: one was found to have a papular type and the second patient had multiple nodular growths. We present these cases, which should be considered in the differential diagnosis of skin nodules.

  15. Identification of 13 DB + dM and 2 DC + dM binaries from the Sloan Digital Sky Survey

    NARCIS (Netherlands)

    Besselaar, E.J.M. van den; Roelofs, G.H.A.; Nelemans, G.A.; Augusteijn, T.; Groot, P.J.

    2005-01-01

    We present the identification of 13 DB + dM binaries and 2 DC + dM binaries from the Sloan Digital Sky Survey (SDSS). Before the SDSS only 2 DB + dM binaries and 1 DC + dM binary were known. At least three, possibly 8, of the new DB + dM binaries seem to have white dwarf temperatures well above 30 0

  16. Pre-treatment ferritin level and alveolar-arterial oxygen gradient can predict mortality rate due to acute/subacute interstitial pneumonia in dermatomyositis treated by cyclosporine a/glucocorticosteroid combination therapy: a case control study [corrected].

    Directory of Open Access Journals (Sweden)

    Kentaro Isoda

    Full Text Available BACKGROUND: Acute/subacute interstitial pneumonia in dermatomyositis (DM-A/SIP is a disease associated with a poor prognosis that resists treatment with glucocorticosteroids (GC and progresses rapidly in a period of weeks to months to death. We retrospectively studied outcomes, prognostic factors, and their relations with survival rate in patients with DM-A/SIP treated with early cyclosporine A (CSA/GC combination therapy and 2-hour postdose blood concentration monitoring. METHODS: This study comprised 32 DM-A/SIP patients who were simultaneously treated with CSA and prednisolone. Clinical and laboratory findings were compared between those who died due to DM-A/SIP and those surviving 24 weeks after beginning of therapy. Prognostic factors were extracted, and their relations with the survival rate were evaluated. RESULTS: Of the 32 DM-A/SIP patients, 25 survived, 5 died of DM-A/SIP, and 2 died of infections. In those who died due to DM-A/SIP, ferritin level and the alveolar-arterial oxygen gradient were significantly increased compared with the survivors (P<0.001 and P = 0.002, respectively. Multivariate analyses showed that ferritin and alveolar-arterial oxygen gradient were independent prognostic factors of poor outcome. The survival rate 24 weeks after beginning of treatment was significantly lower in those with a ferritin level of ≥ 600 ng/ml and alveolar-arterial oxygen gradient of ≥ 45 Torr (P<0.001 and P<0.001, respectively. All patients with both prognostic factors died, and the outcome was significantly poorer in these patients than in those with one or neither of the prognostic factors (P<0.001. CONCLUSIONS: We identified pre-treatment high serum ferritin level and high alveolar-arterial oxygen gradient as poor prognostic factors in DM-A/SIP patients undergoing early CSA/GC combination therapy and showed that the outcomes were poor in patients with both factors.

  17. A Novel Induction Algorithm for DM

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    DM usually means an efficient knowledge discovery from database, and the immune algorithm is a biological theory-based and global searching algorithm. A novel induction algorithm is proposed here which integrates a power of individual immunity and an evolutionary mechanism of population. This algorithm does not take great care of discovering some classifying information, but unknown knowledge or a predication on higher level rules. Theoretical analysis and simulations both show that this algorithm is prone to the stabilization of a population and the improvement of entire capability, and also keeping a high degree of preciseness during the rule induction.

  18. Dermatomiositis juvenil

    OpenAIRE

    Goldaracena, Pablo; Pérez, Federico

    2008-01-01

    La dermatomiositis juvenil (DMJ) es una enfermedad multi sistémica de etiología desconocida, caracterizada por una vasculitis que ocasiona una inflamación no supurativa del músculo estriado y lesiones cutáneas distintivas. La cobertura de los criterios de Bohan y Peter establece el diagnóstico: exantema patognomónico junto a debilidad muscular proximal simétrica, elevación sérica de enzimas musculares, s...

  19. Medical resource utilization in dermatomyositis/polymyositis patients treated with repository corticotropin injection, intravenous immunoglobulin, and/or rituximab

    Directory of Open Access Journals (Sweden)

    Knight T

    2017-05-01

    Full Text Available Tyler Knight,1 T Christopher Bond,1 Breanna Popelar,2 Li Wang,3 John W Niewoehner,4 Kathryn Anastassopoulos,1 Michael Philbin4 1Covance Market Access Services Inc., Gaithersburg, MD, 2Xcenda, LLC, Palm Harbor, FL, 3STATinMED Research, Ann Arbor, MI, 4Mallinckrodt, LLC, Hazelwood, MO, USA Background: Dermatomyositis and polymyositis (DM/PM are rare, incurable inflammatory diseases that cause progressive muscle weakness and can be associated with increased medical resource use (MRU. When corticosteroid treatment is unsuccessful, patients may receive intravenous immunoglobulin (IVIg, rituximab, or repository corticotropin injection (RCI. This study compared real-world, non-medication MRU between patients treated with RCI and those treated with IVIg and/or rituximab for DM/PM.Methods: Claims of DM/PM patients were analyzed from the combination of three commercial health insurance databases in the United States from July 2009 to June 2014. Patients treated with RCI were propensity score matched to those treated with IVIg, rituximab, and both (IVIg+rituximab based on demographics, prior clinical characteristics, and prior MRU. Per-patient per-month (PPPM MRU and costs were compared using Poisson regression and generalized linear modeling, respectively.Results: One-hundred thirty-two RCI, 1,150 IVIg, and 562 rituximab patients had an average age of 52.6, 46.6, and 51.7 years, respectively, and roughly two-thirds were female. After matching, there were no significant differences in demographics or prior clinical characteristics. RCI patients had fewer PPPM hospitalizations (0.09 vs 0.17; P=0.049, shorter length of stay (LOS; 3.24 days vs 4.55 days; P=0.004, PPPM hospital outpatient department (HOPD visits (0.60 vs 1.39; P<0.001, and PPPM physician office visits (2.01 vs 2.33; P=0.035 than IVIg. RCI had fewer PPPM HOPD visits (0.56 vs 0.92; P<0.001 than rituximab. Patients treated with RCI had shorter LOS (2.18 days vs 5.15; P<0.001 and less PPPM HOPD

  20. A case of dermatomyositis associated with mechanic's hand.

    Science.gov (United States)

    Watanabe, C; Okubo, Y; Ohi, T; Koga, M; Abe, H; Tawara, K; Tsuboi, N; Hayashi, T

    2000-11-01

    A 67-year-old man was referred to the Department of Internal Medicine at Tokyo Medical University with interstitial pneumonia in July 1999. He presented with keratotic plaques on both palsm and on the ventral and lateral sides of his fingers. Erythematous keratosis was observed on the dosal aspect of his fingers and metatarsophalangeal (MP) joints. Edematous erythema was seen on the patient's chest, back, and the extensor surfaces of his arms. Electromyography revealed a myogenic pattern and an increased level of myogenic enzymes was found in the blood. Histological findings of the ventral sides of his fingers showed hyperkeratosis and parakeratosis of the dermal tissue and liquefaction degeneration of the basal layer at the papilla. Based on these findings, the patient was given a diagnosis of dermatomyositis associated with mechanic's hand. A systemic examination confirmed interstitial pneumonia and carcinoma of the duodenal papilla. Mechanic's hand is a type of dermatitis associated with myopathy first reported by Stahl et al. in patients with collagen disease. We report herein the first documented case of mechanic's hand in Asians.

  1. Quantitative analysis of Esophageal Transit of Radionuclide in Patients with Dermatomyositis-Polymyositis

    Energy Technology Data Exchange (ETDEWEB)

    Chung, June Key; Lee, Myung Chul; Koh, Chang Soon [Seoul National University College of Medicine, Seoul (Korea, Republic of); Lee, Myung Hae [Asan Medical Center, Seoul (Korea, Republic of)

    1989-07-15

    Esophageal transit of radionuclide was quantitatively analyzed in 29 patients with dermatomyositis-polymyositis Fourteen patients (48.3%) showed retention of tracer in oropharynx. The mean value of percent retention of oropharynx was 15.5+16.6%. Esophageal dysfunction was found in 19 patients (65.5%). Among them 4 showed mild, 12 showed moderate and 3 showed severe esophageal dysfunction. Dysphagia was found in 11 patients (37.9%), which was closely related to percent retention of oropharynx. Quantitative analysis of esophageal transit of radionuclide seemed to be a useful technique for evaluation of dysphagia in patients with dermatomyositis-polymyositis.

  2. A Case of Dermatomyositis with Esophageal Fistula in Whom Blind Mucosal Biopsy Detected Occult Oropharyngeal Carcinoma

    Directory of Open Access Journals (Sweden)

    Miho Kabuto

    2014-11-01

    Full Text Available We present a case of anti-transcription intermediary factor 1 (anti-TIF-1 antibody-positive dermatomyositis with concomitant esophageal fistula and extensive truncal erythema. The characteristic cutaneous features and presence of anti-TIF-1 antibodies were predictive for internal malignancy. However, repeated examinations for internal malignancy showed none, and blind mucosal biopsy was needed to diagnose oropharyngeal carcinoma. We should note the possibility of occult nasopharyngeal carcinoma and consider performing blind mucosal biopsy in dermatomyositis with esophageal fistula, especially with extensive truncal erythema.

  3. A mild form of dermatomyositis as a prodromal sign of lung adenocarcinoma: a case report.

    Science.gov (United States)

    Papakonstantinou, Eleni; Kapp, Alexander; Raap, Ulrike

    2016-02-06

    Dermatomyositis is an idiopathic connective tissue disease characterized by specific cutaneous findings and inflammatory lesions in the muscle biopsy. An association between dermatomyositis and malignancy, including breast, ovarian, lung and colon cancer was recognized many years ago, with an incidence of malignancy in approximately 20 % of cases. Dermatomyositis is hypothesized to be an autoimmune reaction against factors or hormones secreted by the tumor; however, the exact autoimmune mechanism of the disease pathogenesis remains unknown. Here we report a case of a woman with dermatomyositis who was diagnosed with lung adenocarcinoma in the setting of weight loss, progressive fatigue and muscle weakness. A 43-year-old Caucasian woman was referred to our hospital by her physician for suspected contact dermatitis since she described mild itching sensations in her arms and legs as her major symptom. A physical examination revealed erythematous papular lesions over her metacarpophalangeal and proximal interphalangeal joints together with a periungual involvement with redness, hyperkeratosis and capillary telangiectasia along the distal nailfolds on her hands. She was unaware of these features and they did not seem to bother her. A thorough examination of her medical history, however, revealed more symptoms. Pain and weakness in the muscles of her proximal extremities and neck flexor muscles led to difficulty in raising her arms and climbing stairs. At the same time she experienced swallowing difficulties and reported an uncharacteristic weight loss of 10 kg in the last 3 months. The results of laboratory tests showed increased values of serum creatine kinase and myoglobin. An electromyogram, a skin biopsy and a muscle biopsy confirmed the diagnosis of dermatomyositis. A computed tomography of her thorax showed a nodular mass in the upper lobe of her right lung. A histological examination of the lung biopsy showed an adenocarcinoma of moderate differentiation. She was

  4. [Calcinosis universalis associated with dermatomyositis. A report on five cases (author's transl)].

    Science.gov (United States)

    Godeau, P; Herson, S; Ghozlan, R; Bletry, O; Richard, C; Herreman, G

    Five patients with dermatomyositis developed calcinosis universalis, the calcinosis lesions being diffuse in three cases and localized to a subcutaneous site in the other two. The diffuse lesions developed in young subjects with severe dermatomyositis, and increased progressively during recovery from the muscle disease. Treatment was ineffective. Subcutaneous calcifications can be detected at an early stage by technetium pyrophosphate scintigraphy, while the fluid nature of the swellings due to a local inflammatory reaction is proved by ultrasonography. Good results are obtained after excision of those calcified masses requiring removal because of their size, site, or superinfection.

  5. Clinical analysis of 114 cases of dermatomyositis%皮肌炎114例临床分析

    Institute of Scientific and Technical Information of China (English)

    丁峰; 张芳; 刘花香; 李兴福

    2001-01-01

    Objective To investigate the clinic characteristic of dermatomyositis (DM).Method One hundred and fourteen cases of DM were collected from the Qi Lu Hospital of Shandong University from 1994 to 1997.The criteria for the diagnosis of DM were based on the report of Bohan and Peter in 1975.Results The disease can be seen in all ages.Women are affected more frequently than men.Muscle weakness of low limb was observed in 80% cases at the onset.The abnormity of biopsy was seen in all cases.The muscle enzymes including lactic dehydrogenase (LDH),creatine kinase (CK),alanine aminotransferase (ALT) and aspartate aminotransferase (AST) all went up;positive rates were 97%,93%,57% and 54% respectively.There were 26 out of 26 DM patients (100%) with abnormal biopsy;66 out of 69 (95.6%) positive electromyography (EMG);5 out of 114 complicated with malignancy,15 died of pulmonary infection,tumor or heart failure.Conclusion The onset of DM isn′t typical.Early diagnosis should take muscle weakness,muscle enzymes,EMG and biopsy into account.Poor prognostic factors in DM were malignancy,pulmonary infection and heart failure.%目的探讨皮肌炎的临床特点。方法对114例皮肌炎患者进行临床分析。结果该病可见于各个年龄组,女性略多于男性,80%患者以下肢无力症状为首发。通过对26例患者肌肉活检,阳性率达100%。对乳酸脱氢酶(LDH)、肌酸激酶(CK)、丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(AST)等肌肉酶学的检查,其阳性率分别为97%、93%、57%、54%。肌电图检查的阳性率为95.6%,主要表现为肌源性损害。15%患者有肺部炎症表现,4%患者合并肿瘤,年龄均在40岁以上。15例死亡,死因分别为感染、肿瘤及心力衰竭。结论首发症状不典型,对于不明原因的皮损患者,应注意有无四肢近端无痛性肌无力的表现,结合肌肉酶学、肌电图及肌肉活检等检查,早期诊断并不困难。对于40岁以上的患

  6. The clinical utility of serum IL-35 in patients with polymyositis and dermatomyositis.

    Science.gov (United States)

    Yin, Liguo; Ge, Yongpeng; Yang, Hanbo; Peng, Qinglin; Lu, Xin; Zhang, Yamei; Wang, Guochun

    2016-11-01

    The objectives of this study are to assess the levels of serum Interleukin-35 (IL-35) in patients with idiopathic inflammatory myopathies (IIMs) and to evaluate the association between IL-35 levels and IIM-related features. Serum IL-35 was detected in 76 patients with dermatomyositis (DM), 28 patients with polymyositis (PM), 98 disease controls (40 rheumatoid arthritis (RA), 34 systemic lupus erythematosus (SLE), 12 systemic sclerosis (SSc), and 12 sjogren syndrome (SS)), and 43 healthy controls by ELISA. Follow-up was conducted on 34 patients. Serum IL-35 was higher in myositis (PM/DM) patients than in healthy controls (median 76.6 pg/ml [interquartile range (IQR) 57.9-136.2] vs. 29.9 pg/ml (IQR 21.9-65.5), P < 0.001) and disease controls. Serum IL-35 in IIM patients negatively correlated with disease duration moderately (r = -0.35, P < 0.01). Patients with dysphagia had higher IL-35 than those without (median149.35 pg/ml (IQR 87.97-267.32) vs. 70.72 pg/ml (IQR 54.49-123.42), P = 0.001). Cross-sectional correlation analysis showed a weak positive correlation between serum IL-35 and CK (r = 0.293, P = 0.003), moderate positive correlation with erythrocyte sedimentation rate (ESR) (r = 0.304, P = 0.002), serum ferritin (SF) (r = 0.467, P = 0.001) and LDH levels (r = 0.401, P < 0.001). Additionally, serum IL-35 was higher in patients who were positive for anti-HMGCR (median 292.04 pg/ml (IQR 67.9-442.86) vs. 74.66 pg/ml (IQR 57.24-131.32), P = 0.038) and anti-SRP antibody (median 130.33 pg/ml (IQR 88.04-481.28) vs. 73.06 pg/ml (IQR 56.78-134.28), P = 0.009) than in negative patients, respectively. Follow-up study showed that changes in IL-35 levels after treatment correlated with changes in MYOACT scores moderately (r = 0.375, P = 0.029). These data indicate that increased serum IL-35 could act as a disease activity marker and as a risk factor for esophageal involvement in IIM. IL-35 may participate in the pathophysiological

  7. Implementation of SMV based on DM642%基于DM642的SMV实现

    Institute of Scientific and Technical Information of China (English)

    王小兵; 黄冰; 胡巍; 胡斌

    2010-01-01

    介绍了变速率语音算法SMV的DSP实时实现技术.根据标准SMV的要求优化算法的定点C程序,减少占用空间,改善码本的搜索时间,最终在DM642硬件开发平台上运行成功,话音质量符合通信要求.

  8. Dermatomyositis associated with hepatocellular carcinoma in an elderly female patient with hepatitis C virus-related liver cirrhosis

    Institute of Scientific and Technical Information of China (English)

    Nobuyuki Toshikuni; Rikako Torigoe; Mikio Mitsunaga; Akiyoshi Omoto; Koji Nakashima

    2006-01-01

    A 79-year-old female patient with hepatitis C virusrelated liver cirrhosis was diagnosed as having hepatocellular carcinoma (HCC) with a diameter of 2.0 cm. She refused therapy for HCC. Nine months after the diagnosis, she developed dermatomyositis when the HCC enlarged to a diameter of 6.0 cm. She underwent therapy for dermatomyositis, and then transcatheter arterial chemoembolization for HCC. Although the manifestations of dermatomyositis improved and entire tumor necrosis was achieved, she died of pneumonia 2 mo after the treatment of HCC. HCC and/or chronic hepatitis C virus infection might be involved in the pathogenesis of dermatormyositis.

  9. 水疱性皮肌炎合并间质性肺炎1例%Vesicle formation in dermatomyositis complicated with interstitial pneumonia:a case report

    Institute of Scientific and Technical Information of China (English)

    韩建文

    2014-01-01

    We reported a 39-year-old man with vesiculo-bullous dermatomyositis. He reported a two-month history of widespread erythema with muscle weakness and blisters on arms for one week. The myocar-dial enzymes in serum were elevated. Both the electromyography and muscle biopsy supported dermatomyosi-tis. A skin biopsy from the right arm showed subepidermal vesicles. High-resolution computed tomography ( HRCT) scans showed interstitial lung disease. He was diagnosed as vesiculo-bullous DM with aggressive interstitial pneumonia.%报道1例以水疱为皮肤表现的皮肌炎。患者男,39岁。因眼睑水肿性紫红斑,全身多处紫红色斑伴肌无力2个月,上肢水疱1周入院。化验血清心肌酶升高,肌电图提示肌源性损害。上肢水疱处组织病理示:表皮下水疱。肺部CT显示间质性肺炎。

  10. Acute myocardial infarction with normal coronary arteries in a patient with dermatomyositis. Case report.

    Science.gov (United States)

    Badui, E; Valdespino, A; Lepe, L; Rangel, A; Campos, A; Leon, F

    1996-08-01

    The authors present a forty-year-old man, with a history of dermatomyositis for the past twelve years, managed intermittently with prednisone. During an exacerbation of his illness he developed an acute anterior wall myocardial infarction with normal coronary arteries. A literature review indicates this represents a rare association.

  11. Acute lyme infection presenting with amyopathic dermatomyositis and rapidly fatal interstitial pulmonary fibrosis: a case report

    Directory of Open Access Journals (Sweden)

    Nguyen Hanh

    2010-06-01

    Full Text Available Abstract Introduction Dermatomyositis has been described in the setting of lyme infection in only nine previous case reports. Although lyme disease is known to induce typical clinical findings that are observed in various collagen vascular diseases, to our knowledge, we believe that our case is the first presentation of acute lyme disease associated with amyopathic dermatomyositis, which was then followed by severe and fatal interstitial pulmonary fibrosis only two months later. Case presentation We present a case of a 64-year-old African-American man with multiple medical problems who was diagnosed with acute lyme infection after presenting with the pathognomonic rash and confirmatory serology. In spite of appropriate antimicrobial therapy for lyme infection, he developed unexpected amyopathic dermatomyositis and then interstitial lung disease. Conclusions This case illustrates a potential for lyme disease to produce clinical syndromes that may be indistinguishable from primary connective tissue diseases. An atypical and sequential presentation (dermatomyositis and interstitial lung disease of a common disease (lyme infection is discussed. This case illustrates that in patients who are diagnosed with lyme infection who subsequently develop atypical muscular, respiratory or other systemic complaints, the possibility of severe rheumatological and pulmonary complications should be considered.

  12. Human leukocyte Antigen-DM polymorphisms in autoimmune diseases.

    Science.gov (United States)

    Alvaro-Benito, Miguel; Morrison, Eliot; Wieczorek, Marek; Sticht, Jana; Freund, Christian

    2016-08-01

    Classical MHC class II (MHCII) proteins present peptides for CD4(+) T-cell surveillance and are by far the most prominent risk factor for a number of autoimmune disorders. To date, many studies have shown that this link between particular MHCII alleles and disease depends on the MHCII's particular ability to bind and present certain peptides in specific physiological contexts. However, less attention has been paid to the non-classical MHCII molecule human leucocyte antigen-DM, which catalyses peptide exchange on classical MHCII proteins acting as a peptide editor. DM function impacts the presentation of both antigenic peptides in the periphery and key self-peptides during T-cell development in the thymus. In this way, DM activity directly influences the response to pathogens, as well as mechanisms of self-tolerance acquisition. While decreased DM editing of particular MHCII proteins has been proposed to be related to autoimmune disorders, no experimental evidence for different DM catalytic properties had been reported until recently. Biochemical and structural investigations, together with new animal models of loss of DM activity, have provided an attractive foundation for identifying different catalytic efficiencies for DM allotypes. Here, we revisit the current knowledge of DM function and discuss how DM function may impart autoimmunity at the organism level.

  13. Acute Exacerbation of Polymyositis/Dermatomyositis Associated Interstitial Lung Disease%多发性肌炎/皮肌炎相关的间质性肺疾病急性加重的研究进展

    Institute of Scientific and Technical Information of China (English)

    郝秀; 李振华

    2009-01-01

    @@ 结缔组织病相关的间质性肺疾病的死亡率高,在美国每年约有1600人死于结缔组织病相关的间质性肺疾病,约占所有间质性肺疾病死亡人数的25%[1],死因多为间质性肺疾病的急性加重,而多发性肌炎/皮肌炎(polymyositis/dermatomyositis,PM/DM)是结缔组织病中合并肺间质疾病发病率较高的类型,且最容易发生间质性肺疾病的急性加重[2].

  14. What Is Juvenile Arthritis?

    Science.gov (United States)

    ... Analgesics for Osteoarthritis (Report from AHRQ) Joint Replacement Surgery: Health Information Basics for You and Your Family NIH Pediatric Rheumatology Clinic Health Information Juvenile Arthritis Find a Clinical Trial Journal Articles Juvenile Arthritis PDF Version Size: 123 KB ...

  15. Juvenile Delinquency: An Introduction

    Science.gov (United States)

    Smith, Carolyn A.

    2008-01-01

    Juvenile Delinquency is a term which is often inaccurately used. This article clarifies definitions, looks at prevalence, and explores the relationship between juvenile delinquency and mental health. Throughout, differences between males and females are explored. (Contains 1 table.)

  16. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted.

  17. Differences in aberrant expression and splicing of sarcomeric proteins in the myotonic dystrophies DM1 and DM2.

    Science.gov (United States)

    Vihola, Anna; Bachinski, Linda L; Sirito, Mario; Olufemi, Shodimu-Emmanuel; Hajibashi, Shohrae; Baggerly, Keith A; Raheem, Olayinka; Haapasalo, Hannu; Suominen, Tiina; Holmlund-Hampf, Jeanette; Paetau, Anders; Cardani, Rosanna; Meola, Giovanni; Kalimo, Hannu; Edström, Lars; Krahe, Ralf; Udd, Bjarne

    2010-04-01

    Aberrant transcription and mRNA processing of multiple genes due to RNA-mediated toxic gain-of-function has been suggested to cause the complex phenotype in myotonic dystrophies type 1 and 2 (DM1 and DM2). However, the molecular basis of muscle weakness and wasting and the different pattern of muscle involvement in DM1 and DM2 are not well understood. We have analyzed the mRNA expression of genes encoding muscle-specific proteins and transcription factors by microarray profiling and studied selected genes for abnormal splicing. A subset of the abnormally regulated genes was further analyzed at the protein level. TNNT3 and LDB3 showed abnormal splicing with significant differences in proportions between DM2 and DM1. The differential abnormal splicing patterns for TNNT3 and LDB3 appeared more pronounced in DM2 relative to DM1 and are among the first molecular differences reported between the two diseases. In addition to these specific differences, the majority of the analyzed genes showed an overall increased expression at the mRNA level. In particular, there was a more global abnormality of all different myosin isoforms in both DM1 and DM2 with increased transcript levels and a differential pattern of protein expression. Atrophic fibers in DM2 patients expressed only the fast myosin isoform, while in DM1 patients they co-expressed fast and slow isoforms. However, there was no increase of total myosin protein levels, suggesting that aberrant protein translation and/or turnover may also be involved.

  18. Advancement in the study of pathogenesis of polymyositis/dermatomyositis%多发性肌炎/皮肌炎病因和发病机制研究进展

    Institute of Scientific and Technical Information of China (English)

    赵云; 吕玲

    2009-01-01

    多发性肌炎 (polymyositis,PM)和皮肌炎 (dermatomyositis,DM)是累及全身骨骼肌为主的慢性炎症性肌病,其确切发病机制未明.随着对该病研究的不断深入和新的研究技术的使用,近年来人们对该病发病机制的认识不断加深,大都认为本病与自身免疫异常有关,非免疫机制亦有所研究.但目前国内外尚无全面描述这些进展的相关综述,本文就该病发病机制的研究进展做一综述,并将各种机制假说有机联系起来以使人们更加深刻地认识该病.%Polymyositis (PM) and dermatomyositis (DM) are inflammatory myopathies and slowly advanced of unknown etiology that affect the skeletal muscles.With the advancement of the constant research on the disease and use of new technology,people have a penetrating realization about the pathogenesis. The disease is thought to be associated with autoimmune as well as nonimmune mechanism. By now, there is no overall summary on the newest advancement of the pathogenesis.We summarized the progress on the pathogenesis of the disease in order to make it more clear to physicians.

  19. Juvenile Arrests, 2007. Juvenile Justice Bulletin

    Science.gov (United States)

    Puzzanchera, Charles

    2009-01-01

    This Bulletin summarizes 2007 juvenile crime and arrest data reported by local law enforcement agencies across the country and cited in the FBI report, "Crime in the United States 2007." The Bulletin describes the extent and nature of juvenile crime that comes to the attention of the justice system. It serves as a baseline for comparison for…

  20. Differences in aberrant expression and splicing of sarcomeric proteins in the myotonic dystrophies DM1 and DM2

    OpenAIRE

    2010-01-01

    Aberrant transcription and mRNA processing of multiple genes due to RNA-mediated toxic gain-of-function has been suggested to cause the complex phenotype in myotonic dystrophies type 1 and 2 (DM1 and DM2). However, the molecular basis of muscle weakness and wasting and the different pattern of muscle involvement in DM1 and DM2 are not well understood. We have analyzed the mRNA expression of genes encoding muscle-specific proteins and transcription factors by microarray profiling and studied s...

  1. Juvenile polyposis syndrome

    NARCIS (Netherlands)

    L.A.A. Brosens; D. Langeveld; W.A. van Hattem; F.M. Giardiello; G.J.A. Offerhaus

    2011-01-01

    Juvenile polyposis syndrome is a rare autosomal dominant syndrome characterized by multiple distinct juvenile polyps in the gastrointestinal tract and an increased risk of colorectal cancer. The cumulative life-time risk of colorectal cancer is 39% and the relative risk is 34. Juvenile polyps have a

  2. Association of tumor necrosis factor α (TNF-α) and interleukin 10 (IL-10) gene polymorphisms in dermatomyositis patients: a pilot study.

    Science.gov (United States)

    Hristova, Maria; Dourmishev, Lyubomir; Kamenarska, Zornitsa; Kaneva, Radka; Vinkov, Anton; Mitev, Vanio

    2012-01-01

    TNF-α and IL-10 single nucleotide polymorphisms have been implicated in various autoimmune diseases but the results are still quite controversial. This case-control study aimed to investigate the association between six TNF-α and five IL-10 polymorphisms with dermatomyositis. The -857CC and +489GG genotypes showed a weak association with dermatomyositis when the analysis was carried out for the whole cohort but they appeared to be a significant risk factor for the development of dermatomyositis in women. The TNF-α -1031CC genotype was found only among dermatomyositis patients. The TNF-α -1031C/-863C/-857C-308G/+489G haplotype showed a significant association with dermatomyositis in women. The IL-10 -3575TT genotype and T allele showed an association with dermatomyositis. The frequency of the IL-10 -2763CC genotype and C allele was higher among dermatomyositis patents and it was associated with an increased OR. Haplotype analysis showed an association between the IL-10 -3575T/-2763C haplotype and dermatomyositis. In conclusion, our results indicate that both TNF-α and IL-10 polymorphisms are associated with the development of dermatomyositis in Bulgarian patients.

  3. EW scale DM models with dark gauge symmetries

    CERN Document Server

    Ko, P

    2016-01-01

    In this talk, I describe a class of electroweak (EW) scale dark matter (DM) models where its stability or longevity are the results of underlying dark gauge symmetries: stable due to unbroken local dark gauge symmetry or topology, or long-lived due to the accidental global symmetry of dark gauge theories. Compared with the usual phenomenological dark matter models (including DM EFT or simplified DM models), DM models with local dark gauge symmetries include dark gauge bosons, dark Higgs bosons and sometimes excited dark matter. And dynamics among these fields are completely fixed by local gauge principle. The idea of singlet portals including the Higgs portal can thermalize these hidden sector dark matter very efficiently, so that these DM could be easily thermal DM. I also discuss the limitation of the usual DM effective field theory or simplified DM models without the full SM gauge symmetry, and emphasize the importance of the full SM gauge symmetry and renormalizability especially for collider searches for...

  4. Juveniles on trial.

    Science.gov (United States)

    Quinn, Kathleen M

    2002-10-01

    This article describes common forensic evaluations requested of juvenile court mental health evaluators. There has been a legal shift toward criminalization of juvenile court, with a greater emphasis on rights, abandonment of the rehabilitative model, and greater movement of adolescents into the adult criminal court. A resulting shift has been the redefinition of juvenile court forensic evaluations toward the specificity of adult forensic work. The challenge for evaluators is to refine their knowledge of the forensic standards and bring knowledge of development, assessment, and diagnosis in juveniles and interview techniques appropriate to juveniles to improve the evaluation and forensic reports.

  5. Study on the Cardiac Damage in the Patients with Polymyositis/Dermatomyositis%多发性肌炎/皮肌炎患者心脏损害的分析和评价

    Institute of Scientific and Technical Information of China (English)

    胡绍先; 何培根

    2001-01-01

    探讨多发性肌炎(PM)/皮肌炎(DM)患者心脏损害的表现及其意义。以临床资料、血清肌酶谱、胸片、心电图、超声心动图等指标为依据,对我院1992~1998年住院治疗的45例PM/DM患者进行分析和评价。23例伴发心脏损害(51.1%),病变累及心包、心肌、心内膜和冠状动脉,临床表现多种多样,心脏损害者中3例因心力衰竭死亡。表明心脏损害为PM/DM最常见的合并症,也是本病常见的死亡原因之一,应引起临床医师的足够重视。%To investigate the significance of cardiac damage in the patients with polymyositis (PM)/dermatomyositis (DM),45 cases of PM/DM in our hospital from 1992 to 1998 were retrospectively analyzed in the aspects of the clinical data,muscle zymogram,X-ray plain films,electrocardiograph,ultrasonic cardiogram. It was found that cardiac damage (51.1 %) occurred in 23 out of 45 cases of PM/DM. Pericardium,cardiac muscle,endocardium and coronary artery were involved. Clinical manifestations were various. Among the cases of cardiac damage,3 died of heart failure. It was indicated that cardiac damage is the most common complication and also one of the common death causes of PM/DM.

  6. Dermatomiosite e polimiosite: da imunopatologia à imunoterapia (imunobiológicos Dermatomyositis and polymyositis: from immunopathology to immunotherapy (immunobiologics

    Directory of Open Access Journals (Sweden)

    Samuel Katsuyuki Shinjo

    2013-02-01

    Full Text Available As miopatias inflamatórias idiopáticas (MII, das quais fazem parte a dermatomiosite (DM e a polimiosite (PM, são doenças sistêmicas crônicas associadas a alta morbidade e incapacidade funcional. O tratamento atual baseia-se na corticoterapia e no uso de imunossupressores, porém uma parcela considerável dos pacientes é refratária à terapia tradicional. Isso tem levado à tentativa de uso de imunobiológicos nesses pacientes, tendo por fundamento a fisiopatogênese das MII. Do ponto de vista imunopatológico, há diferenças entre PM e DM: a primeira está mais relacionada à imunidade celular, enquanto na segunda o papel humoral parece mais importante. Em ambas, porém, são descritas concentrações elevadas de interleucinas pró-inflamatórias (TNF, IL-1, IL-6 e aumento da expressão de moléculas relacionadas à coestimulação dos linfócitos T - nessas condições, parece racional o uso da terapia biológica. Considerando os imunobiológicos disponíveis, são escassos os dados de trabalhos abertos na literatura, compostos principalmente por séries e relatos de casos. Os bloqueadores do TNF apresentam resultados conflitantes sem evidência de boa resposta ao tratamento. A terapia anti-CD20 possui os resultados mais promissores. É extremamente escassa a informação sobre o bloqueio da coestimulação do linfócito T e a terapia anti- IL-6, que impede qualquer consideração. Dessa maneira, o uso de imunobiológicos em MII ainda permanece como fronteira a ser explorada. A terapia biológica pode ter papel relevante no tratamento das MII refratárias à terapia convencional; no entanto, novos estudos prospectivos com base em parâmetros objetivos de resposta ao tratamento são necessários. Até o momento, a terapia anti-CD20 parece ser a mais promissora no tratamento das MII refratárias.Idiopathic inflammatory myopathies (IIM, which include dermatomyositis (DM and polymyositis (PM, are chronic systemic diseases associated with

  7. Konfirmasi spesifitas GAD65 terhadap anti-GAD65 pada tikus DM dan pasien DM tipe 1

    Directory of Open Access Journals (Sweden)

    Aulanni’a Aulanni’a

    2012-02-01

    Full Text Available The use of glutamic acid decarboxylase (GAD65 from bovine brain has been studied to obtain basic knowledge and diagnosis and prediction of Type 1 Diabetes Mellitus (DM patients. The importance of GAD65 in DM diagnosis based on its patogenesis. One of the autoimmune marker that can be used to detect beta-pancreas destruction in Diabetes Type I is the antibody to glutamic acid decarboxylase (GAD65. Most of the pre-diabetic patients indicate the reactive autoantibody to GAD65. For early detection of anti-GAD65 in the serum of the patient, human recombinat GAD65 has been succeed to be used. However this is not economical, therefore, it is necessary to find the alternative source of cheaper GAD65. The aim of this research is to develop an early detection kit of Type 1 DM based on antibody- GAD65, since the longest patient suffering from DM has higher probability to be complicated, especially for uncured patients. The anti- GAD65 antibodies induced by anti-GAD65 synthetized and labelled by alkaline phosphatase can be used as reagent detection early DM patients. The ten patients of DM as samples (positive of anti-GAD65 and five rats of DM were positive with western blott technique using reagents as result of this research. It can be concluded, GAD65 enzyme isolated from bovine brain induced anti-GAD65 production and have possibilities to be packaged in a diagnostic kit for patient pre DM.

  8. Juvenile polyposis syndrome

    Institute of Scientific and Technical Information of China (English)

    Lodewijk AA Brosens; Danielle Langeveld; W Arnout van Hattem; Francis M Giardiello; G Johan A Offerhaus

    2011-01-01

    Juvenile polyposis syndrome is a rare autosomal dominant syndrome characterized by multiple distinct juvenile polyps in the gastrointestinal tract and an increased risk of colorectal cancer.The cumulative life-time risk of colorectal cancer is 39% and the relative risk is 34.Juvenile polyps have a distinctive histology characterized by an abundance of edematous lamina propria with inflammatory cells and cystically dilated glands lined by cuboidal to columnar epithelium with reactive changes.Clinically, juvenile polyposis syndrome is defined by the presence of 5 or more juvenile polyps in the colorectum,juvenile polyps throughout the gastrointestinal tract or any number of juvenile polyps and a positive family history of juvenile polyposis.In about 50%-60% of patients diagnosed with juvenile polyposis syndrome a germline mutation in the SMAD4 or BMPR1A gene is found.Both genes play a role in the BMP/TGF-beta signalling pathway.It has been suggested that cancer in juvenile polyposis may develop through the so-alled "landscaper mechanism" where an abnormal stromal environment leads to neoplastic transformation of the adjacent epithelium and in the end invasive carcinoma.Recognition of this rare disorder is important for patients and their families with regard to treatment,follow-up and screening of at risk individuals.Each clinician confronted with the diagnosis of a juvenile polyp should therefore consider the possibility of juvenile polyposis syndrome.In addition, juvenile polyposis syndrome provides a unique model to study colorectal cancer pathogenesis in general and gives insight in the molecular genetic basis of cancer. This review discusses clinical manifestations, genetics, pathogenesis and management of juvenile polyposis syndrome.

  9. Generalized subcutaneous edema as a rare manifestation of dermatomyositis: clinical lesson from a rare feature.

    LENUS (Irish Health Repository)

    Haroon, Muhammad

    2011-04-01

    Generalized subcutaneous edema is a very rare manifestation of inflammatory myopathies. A 61-year-old woman presented with classic signs and symptoms of dermatomyositis. She was also noted to have generalized edema that was so florid that an alternative diagnosis was considered. Her disease was resistant to corticosteroids, azathioprine, and mycophenolate mofetil. Intravenous administration of immunoglobulins was started because of marked worsening of her disease-muscle weakness, generalized anasarca, and involvement of her bulbar muscles. This led to dramatic resolution of her subcutaneous edema and significant improvement of her skin and muscle disease. As the initial screen for malignancy was negative, a positron emission tomography-computed tomography scan was requested, which interestingly showed a metabolically active cervical tumor. Anasarca is an unusual manifestation of dermatomyositis. In treatment-refractory cases, it seems reasonable to consider positron emission tomography scan in excluding underlying malignant disease.

  10. Successful Immunoglobulin Treatment in Severe Cryptogenic Organizing Pneumonia Caused by Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Dong Hoon Lee

    2015-08-01

    Full Text Available In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.

  11. A case of dermato-fasciitis: amyopathic dermatomyositis associated with fasciitis.

    Science.gov (United States)

    Tsuruta, Yuko; Ikezoe, Koji; Nakagaki, Hideaki; Shigeto, Hiroshi; Kawajiri, Masakazu; Ohyagi, Yasumasa; Kira, Jun-Ichi

    2004-04-01

    Amyopathic dermatomyositis (ADM) is characterized by the typical cutaneous features of dermatomyositis and minor involvement of the skeletal muscles. A 50-year-old woman had fever, reddening and pain in the distal part of all four limbs, and cutaneous findings such as Gottron's papules and periorbital heliotrope. She showed no muscle weakness or atrophy, and her serum creatine kinase was within the normal range. Electromyography showed no myopathic pattern. Magnetic resonance imaging (MRI) recorded abnormal hyperintensity in the fascia and muscle of the tibialis anterior. A biopsy from the tibialis anterior muscle showed fasciitis and mild myopathic changes with focal perivascular infiltration. This patient also presented with interstitial pneumonitis, although evaluation for malignancy was negative. With steroid therapy, her symptoms and MRI abnormality disappeared within 2 months. This case is therefore considered to be a variant of ADM, presenting as dermato-fasciitis.

  12. Application and Evaluation of EMG in Diagnosing Polymyositis and Dermatomyositis%多发性肌炎与皮肌炎的肌电图特征分析

    Institute of Scientific and Technical Information of China (English)

    蔡慧敏

    2005-01-01

    目的探讨多发性肌炎(polymyositis,PM)、皮肌炎(dermatomyositis,DM)的肌电图特征.方法对65例PM和DM患者的肌电图进行分析.结果肌电图示肌源性损害55例,神经源性损害2例.肌源性损害主要表现为插入电位延长(19.29%)、出现自发电位(45.18%)、运动单位电位(MUP)时限缩(67.54%)、MUP波幅降低(17.98%)、多相波增多(42.11%)、重收缩波型异(41.67%).股四头肌、胫前肌异常率显著高于三角肌、肱二头肌(P<0.05),胫前肌MUP时限缩短,多相波增多阳性率最高.结论肌电图检查是诊断PM和DM的重要手段,选择股四头肌、胫前肌进行EMG检查阳性率高.

  13. Detection of multifocal osteonecrosis in an adolescent with dermatomyositis using whole-body MRI

    Energy Technology Data Exchange (ETDEWEB)

    Castro, Tania C.M.; Terreri, Maria Teresa A.; Hilario, Maria Odete E. [Federal University of Sao Paulo, Department of Pediatrics, Division of Allergy, Clinical Immunology and Rheumatology, Sao Paulo, SP (Brazil); Lederman, Henrique [Federal University of Sao Paulo, Image Diagnosis Department, Sao Paulo (Brazil); Kaste, Sue C. [St. Jude Children' s Research Hospital, Department of Radiological Sciences, Division of Diagnostic Imaging, Memphis, TN (United States)

    2010-09-15

    Osteonecrosis is a well-recognized complication of corticosteroid use resulting in significant morbidity, often requiring surgical intervention. Whole-body MRI is a promising method that allows imaging of the whole patient in a reasonable time without the use of ionizing radiation. This technique has the potential for evaluating nonmalignant multifocal skeletal disease like osteonecrosis. This case highlights the value of whole-body MR in an adolescent with dermatomyositis who developed multifocal osteonecrosis. (orig.)

  14. The Effects of 12 Weeks Regular Aerobic Exercise on Brain-derived Neurotrophic Factor and Inflammatory Factors in Juvenile Obesity and Type 2 Diabetes Mellitus

    OpenAIRE

    Lee, Sung Soo; Yoo, Jae Ho; Kang, Sung; Woo, Jin Hee; Shin, Ki Ok; Kim, Kwi Beak; Cho, Su Youn; Roh, Hee Tae; Kim, Young Il

    2014-01-01

    [Purpose] The purpose of this study was to investigate the effects of 12 weeks regular aerobic exercise on brain-derived neurotrophic factor (BDNF) and inflammatory factors in juvenile obesity and type 2 diabetes mellitus (T2DM). Obesity and T2DM, typically common among adults, have recently become more prevalent in the Korean juvenile population, affecting not only their lipid profiles and oxidant stress levels, but also their BDNF and inflammatory factor levels. [Subjects] This study enroll...

  15. The evidence for immunotherapy in dermatomyositis and polymyositis: a systematic review.

    Science.gov (United States)

    Vermaak, Erin; Tansley, Sarah L; McHugh, Neil J

    2015-12-01

    Dermatomyositis and polymyositis are rare chronic inflammatory disorders with significant associated morbidity and mortality despite treatment. High-dose corticosteroids in addition to other interventions such as immunosuppressants, immunomodulators, and more recently, biologics are commonly used in clinical practice; however, there are no clear guidelines directing their use. Our objective was to systematically review the evidence for immunotherapy in the treatment of dermatomyositis and polymyositis. Relevant studies were identified through Embase and PubMed database searches. Trials were selected using pre-determined selection criteria and then assessed for quality. Randomized controlled trials and experimental studies without true randomization and including adult patients with definite or probable dermatomyositis or polymyositis were evaluated. Any type of immunotherapy was considered. Clinical improvement, judged by assessment of muscle strength after 6 months, was the primary outcome. Secondary outcomes included IMACS definition of improvement, improvements in patient and physician global scores, physical function, and muscle enzymes. Twelve studies met eligibility criteria. Differences in trial design, quality, and variable reporting of baseline characteristics and outcomes made direct comparison impossible. Although no treatment can be recommended on the basis of this review, improved outcomes were demonstrated with a number of agents including methotrexate, azathioprine, ciclosporin, rituximab, and intravenous immunoglobulin. Plasmapheresis and leukapheresis were of no apparent benefit. More high-quality randomized controlled trials are needed to establish the role of immunosuppressive agents in the treatment of these conditions and the clinical context in which they are most likely to be beneficial.

  16. Paniculitis en paciente con dermatomiositis Panniculitis in a patient with dermatomyositis

    Directory of Open Access Journals (Sweden)

    Mariana Arias

    2011-02-01

    Full Text Available La paniculitis es una manifestación cutánea infrecuente de la dermatomiositis. Puede preceder hasta en 14 meses a otras manifestaciones de la dermatomiositis. En todos los casos, la miositis y la paniculitis presentan mejoría simultánea durante el tratamiento. Describimos una paciente de sexo femenino de 30 años que presenta lesiones clínica e histológicamente compatibles con paniculitis luego de 2 meses de que el compromiso muscular y cutáneo permitió el diagnóstico de dermatomiositis. Las lesiones cutáneas remitieron con el tratamiento esteroideo.Panniculitis is a rarely reported clinical finding in dermatomyositis. It may precede the other manifestations associated with dermatomyositis by as much as 14 months. In all cases, myositis and panniculitis improve simultaneously during treatment. The present report describes the case of a 30-year-old female patient with clinical and histopathological findings consistent with panniculitis two months after the onset of the muscle and cutaneous symptoms that permitted diagnosis of dermatomyositis. The skin lesions regressed following steroid treatment.

  17. Paraneoplastic Dermatomyositis with Cutaneous and Myopathic Disease Responsive to Adrenocorticotropic Hormone Therapy

    Science.gov (United States)

    Mancuso, Christopher; Lal, Karan; Dicostanzo, Damian; Gropper, Charles

    2017-01-01

    Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron’s sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy. The authors present a case report of paraneoplastic dermatomyositis associated with triple negative, BRCA-1 positive, invasive intraductal carcinoma of the breast, whose myopathic and cuteanous symptoms were recalcitrant to high-dose corticosteroid therapy. Herein, the authors describe the first reported case of the use of an injectable adrenocorticotropic hormone agonist gel in a patient with myopathic paraneoplastic disease that achieved clinical resolution of both myopathic and cutaneous symptoms, but subseuqently developed significant hyperpigmentation of her face suspected to be secondary to a chemotherapeutic-induced pigmentary change which was augmented by adrenocorticotropic hormone therapy. PMID:28210382

  18. DM-Ice: Current Status and Future Prospects

    CERN Document Server

    Pettus, Walter C

    2015-01-01

    DM-Ice is a program towards the first direct detection search for dark matter in the Southern Hemisphere with a 250 kg-scale NaI(Tl) crystal array. It will provide a definitive understanding of the modulation signal reported by DAMA by running an array at both Northern and Southern Hemisphere sites. A 17 kg predecessor, DM-Ice17, was deployed in December 2010 at a depth of 2457 m under the ice at the geographic South Pole and has concluded its 3.5 yr data run. An active R&D program is underway to investigate detectors with lower backgrounds and improved readout electronics; two crystals with 37 kg combined mass are currently operating at the Boulby Underground Laboratory. We report on the final analyses of the DM-Ice17 data and describe progress towards a 250 kg DM-Ice experiment.

  19. Development of three-dimensional memory (3D-M)

    Science.gov (United States)

    Yu, Hong-Yu; Shen, Chen; Jiang, Lingli; Dong, Bin; Zhang, Guobiao

    2016-10-01

    Since the invention of 3-D ROM in 1996, three-dimensional memory (3D-M) has been under development for nearly two decades. In this presentation, we'll review the 3D-M history and compare different 3D-Ms (including 3D-OTP from Matrix Semiconductor, 3D-NAND from Samsung and 3D-XPoint from Intel/Micron).

  20. Dermatomyositis as a complication of interferon-α therapy: a case report and review of the literature.

    Science.gov (United States)

    Shiba, Hideyuki; Takeuchi, Tohru; Isoda, Kentaro; Kokunai, Yasuhito; Wada, Yumiko; Makino, Shigeki; Hanafusa, Toshiaki

    2014-09-01

    Autoimmune disorder is one of the important side effects of interferon-α therapy. Some polymyositis cases as complication of interferon-α therapy were reported, but dermatomyositis were rarely. We report a case of dermatomyositis as a complication of interferon-α therapy for hepatitis C. A 52-year-old Japanese man was treated by combination therapy with pegylated interferon-α-2b and ribavirin for hepatitis C. Three months after the initiation of therapy, he showed erythema in the posterior cervical to dorsal and anterior cervical to thoracic regions, weight loss, general malaise, muscle pain, and severe increase in levels of muscle enzymes. We made a diagnosis of dermatomyositis according to these clinical features, proximal muscle-predominant myogenic change on electromyography, and infiltration of monocytes and CD4+-dominant lymphocytes on skin biopsy, although myositis-associated antibodies were absent. He was successfully treated with intravenous immunoglobulin and tacrolimus in addition to glucocorticoid. This is a very rare case of dermatomyositis associated with interferon-α therapy. We reviewed several similar published cases and the association of dermatomyositis and type I interferon.

  1. The cfinical and pathological features of hypomyopathic dermatomyositis: a report of 6 cases%低肌病性皮肌炎的临床和肌肉病理特点六例分析

    Institute of Scientific and Technical Information of China (English)

    张晓雷; 洪道俊; 张卓莉; 栗占国; 张巍; 袁云

    2012-01-01

    Objective To explore the clinical and pathological features of hypomyopathic dermatomyositis (HDM).Methods Six cases of HDM among 62 cases of dermatomyositis diagnosed between January 2008 and June 2010 were collected and analyzed.Four were women and 2 were men.The age of onset was between 45 and 65 years old,with disease duration of more than 6 months.All HDM patients presented with DM-specific skin lesions,but without muscle weakness.Serum creatine kinase (CK) level was within normal limits in 5 and mild elevated in 1.Electromyogram showed myogenic changes in 4 cases and normal in the other 2 patients.Muscle biopsies were carried out in the left biceps branchii in all of them.The muscle sections were processed with standard histological procedures, enzyme histochemistrical and immunohistochemical stains were carried out.The antibodies used for immunohistochemical stains were antiCD8,anti-CD20,anti-CD68,and anti-MHC-Ⅰ monoclonal antibodies.Results Muscle biopsies showed a variable amount of macrophages and B-lymphocytes infiltrating in the peri-mysium and fascicle area.Three of them presented with mild peri-fascicular fiber atrophy with fiber necrosis and regeneration.Lipid droplets increased mildly in muscle fibers.Capillaries distributing around perifascicular regions were darkly stained in nonspecific esterase stain.Perifascicular fibers showed immune-reactivity to MHC-Ⅰ antibody.Conclusion The myopathological changes of HDM mimic classical dermatomyositis in some cases.HDM might be an intermediate presentation between pure amyopathic dermatomyositis and classical dermatomyositis.%目的 了解低肌病性皮肌炎的临床表现和肌肉病理改变特点.方法 收集我院2008年1月至2010年6月行肌肉活检的62例皮肌炎患者,按照低肌病性皮肌炎的诊断标准筛选6例,其中4例患者肌电图呈肌源性损害.同时对6例患者进行肱二头肌活检,标本进行组织学、酶组织化学染色和免疫组织化学染色,后者

  2. The Clinical Phenotypes of the Juvenile Idiopathic Inflammatory Myopathies

    Science.gov (United States)

    Shah, Mona; Mamyrova, Gulnara; Targoff, Ira N.; Huber, Adam M.; Malley, James D.; Rice, Madeline Murguia; Miller, Frederick W.; Rider, Lisa G.

    2013-01-01

    Abstract The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical

  3. PERBEDAAN POLA KONSUMSI DAN STATUS GIZI ANTARA REMAJA DENGAN ORANG TUA DIABETES MELITUS (DM) DAN NON DM

    OpenAIRE

    Marine, Denov; Adiningsih, Sri

    2017-01-01

    Adolescents prefer to consume high fat and high sugar food compare to healthy food such as fruit and vegetable. High fat and high sugar food are risk factor of diabetes mellitus development, especially in adolescents whose parents had diabetes mellitus. The aim of this study was to analyze the difference of eating habit and nutritional status between adolescents with DM parents and non DM parents. This was a cross-sectional research included 42 adolescents as sample that divided into 21 adole...

  4. Dermatomiosite recém-diagnosticada em idosos como preditiva de malignidade Newly diagnosed dermatomyositis in the elderly as predictor of malignancy

    Directory of Open Access Journals (Sweden)

    Fernando Henrique Carlos de Souza

    2012-10-01

    Full Text Available OBJETIVO: Os sintomas da dermatomiosite (DM podem ser um indício da existência de um câncer oculto. Melhorar a detecção precoce é essencial, porém não há estudos avaliando em curto prazo os fatores preditivos para a doença. MÉTODO: Estudo retrospectivo, monocêntrico, incluindo pacientes com DM definida (pelo menos quatro dos cinco critérios de Bohan e Peter, 1975, no período entre 1991 e 2011. A presença de malignidade foi limitada a um período de até 12 meses após o diagnóstico da doença. RESULTADOS: Houve 12 casos de neoplasias em 139 pacientes (pele, trato gastrintestinal, próstata, tireoide, mama, pulmão e trato geniturinário. Os pacientes com neoplasia tiveram maior média de idade que os controles (56,8 ± 15,7 vs. 40,3 ± 13,1 anos, respectivamente, P = 0,004; odds ratio 1,09; intervalo de confiança de 95%: 1,04-1,14. Não foram observadas diferenças estatísticas em relação a gênero, etnia, frequência de sintomas constitucionais, envolvimento de órgãos e sistemas e/ou alterações laboratoriais. CONCLUSÃO: Na DM recém-diagnosticada, a idade tardia ao diagnóstico foi um fator preditivo de malignidade.OBJECTIVE: Dermatomyositis (DM symptoms may be a clue to the existence of a hidden cancer. Enhancing early detection is essential, but there are no studies evaluating short-term predictive factors in this disease. METHODS: This is a singlecenter retrospective study, including patients diagnosed with DM meeting at least four of the five Bohan and Peter's criteria (1975, from 1991 to 2011. This study assessed malignancies occurring in up to 12 months after the diagnosis of DM. RESULTS: Neoplasm was found in 12 out of 139 patients (skin, gastrointestinal tract, prostate, thyroid, breast, lungs, and genitourinary tract. Patients with neoplasm had a higher mean age than controls (56.8 ± 15.7 vs. 40.3 ± 13.1 years, respectively, P = 0.004, odds ratio 1.09; 95% confidence interval: 1.04-1.14. No statistical

  5. Juvenile giant fibroadenoma

    Directory of Open Access Journals (Sweden)

    Vipul Yagnik

    2011-07-01

    Full Text Available Fibroadenomas are benign solid tumor associated with aberration of normal lobular development. Juvenile giant fibroadenoma is usually single and >5 cm in size /or >500 gms in weight. Important differential diagnoses are: phyllodes tumor and juvenile gigantomastia. Simple excision is the treatment of choice.

  6. Renewing Juvenile Justice

    Science.gov (United States)

    Macallair, Daniel; Males, Mike; Enty, Dinky Manek; Vinakor, Natasha

    2011-01-01

    The Center on Juvenile and Criminal Justice (CJCJ) was commissioned by Sierra Health Foundation to critically examine California's juvenile justice system and consider the potential role of foundations in promoting systemic reform. The information gathered by CJCJ researchers for this report suggests that foundations can perform a key leadership…

  7. Philanthropist in Juvenile Reformatory

    Institute of Scientific and Technical Information of China (English)

    HAN NIU

    2007-01-01

    @@ On the afternoon of February 1, 2007, Chen Guangbiao, a noted philanthropist, found himself in the Jiangsu Provincial Juvenile Reformatory in Jurong City for a ceremony to donate two buses, 100 computers, and 100 desks and 100 chairs for the juvenile offenders to use in their study.

  8. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. Th

  9. Juvenile Confinement in Context

    Science.gov (United States)

    Mendel, Richard A.

    2012-01-01

    For more than a century, the predominant strategy for the treatment and punishment of serious and sometimes not-so-serious juvenile offenders in the United States has been placement into large juvenile corrections institutions, alternatively known as training schools, reformatories, or youth corrections centers. America's heavy reliance on…

  10. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. Th

  11. File list: Oth.ALL.10.dm.AllCell [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.ALL.10.dm.AllCell dm3 TFs and others dm All cell types SRX160967,SRX160968,SRX3...35496,SRX495787,SRX495790,SRX495786,SRX335495 http://dbarchive.biosciencedbc.jp/kyushu-u/dm3/assembled/Oth.ALL.10.dm.AllCell.bed ...

  12. File list: Oth.CeL.50.dm.AllCell [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.CeL.50.dm.AllCell dm3 TFs and others dm Cell line SRX160968,SRX160967,SRX495786...,SRX495787,SRX335495,SRX335496,SRX495790 http://dbarchive.biosciencedbc.jp/kyushu-u/dm3/assembled/Oth.CeL.50.dm.AllCell.bed ...

  13. File list: Oth.CeL.05.dm.AllCell [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.CeL.05.dm.AllCell dm3 TFs and others dm Cell line SRX160967,SRX495786,SRX160968...,SRX495790,SRX335495,SRX335496,SRX495787 http://dbarchive.biosciencedbc.jp/kyushu-u/dm3/assembled/Oth.CeL.05.dm.AllCell.bed ...

  14. File list: Oth.CeL.10.dm.AllCell [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.CeL.10.dm.AllCell dm3 TFs and others dm Cell line SRX160967,SRX160968,SRX335496...,SRX495787,SRX495790,SRX495786,SRX335495 http://dbarchive.biosciencedbc.jp/kyushu-u/dm3/assembled/Oth.CeL.10.dm.AllCell.bed ...

  15. Juvenile mammary papillomatosis; Papilomatosis juvenil mamaria

    Energy Technology Data Exchange (ETDEWEB)

    Alvarez, M.; Jimenez, A. V. [Hospital Reina Sofia. Cordoba (Spain)

    2001-07-01

    Juvenile mammary papillomatosis is a benign proliferative disease of young patients, generally under 30 years of age. The most frequent clinical presentation is the existence of an elastic and mobile lymph node of the breast. Anatomopathologically, it is characterized because it presents ductal epithelial hyperplasia, sometimes with marked atypia, and there are numerous cysts having different sizes among the findings. It has been associated with an increase in the incidence of breast cancer, both in the patient herself as well as her family. We review the literature on the subject and present the mammographic and ultrasonographic findings of a 22 year old woman diagnosed of juvenile mammary papillomatosis. (Author) 12 refs.

  16. Design Overview of the DM Radio Pathfinder Experiment

    CERN Document Server

    Silva-Feaver, Maximiliano; Cho, Hsiao-Mei; Dawson, Carl; Graham, Peter; Irwin, Kent; Kuenstner, Stephen; Li, Dale; Mardon, Jeremy; Moseley, Harvey; Mule, Richard; Phipps, Arran; Rajendran, Surjeet; Steffen, Zach; Young, Betty

    2016-01-01

    We introduce the DM Radio, a dual search for axion and hidden photon dark matter using a tunable superconducting lumped-element resonator. We discuss the prototype DM Radio Pathfinder experiment, which will probe hidden photons in the 500 peV (100 kHz)-50 neV (10 MHz) mass range. We detail the design of the various components: the LC resonant detector, the resonant frequency tuning procedure, the differential SQUID readout circuit, the shielding, and the cryogenic mounting structure. We present the current status of the pathfinder experiment and illustrate its potential science reach in the context of the larger experimental program.

  17. Electroweak supersymmetric dark matter annihilation in DM rate at NLO

    Energy Technology Data Exchange (ETDEWEB)

    Schmiemann, Saskia; Klasen, Michael; Kovarik, Karol; Steppeler, Patrick [Institut fuer Theoretische Physik, Universitaet Muenster (Germany); Herrmann, Bjoern [LAPTh, Universite Savoie Mont Blanc, CNRS (France); Harz, Julia [CNRS, UMR 7589, LPTHE, Paris (France); Sorbonne Universites, Institut Lagrange de Paris (ILP) (France); Sorbonne Universites, UPMC Univ Paris 06, UMR 7589, LPTHE (France)

    2016-07-01

    Today there are several pieces of evidence for dark matter. One well-known experiment is the measurement of the Dark Matter relic density by the Planck satellite. The talk introduces the 'Dark Matter at next-to-leading order' (DM rate at NLO) project which provides predictions for the dark matter relic density in the MSSM including higher-order corrections. After an introduction of the project DM rate at NLO, I shortly speak about the calculation of the electroweak processes. The main focus lies on the effects of the electroweak tree-level processes on the relic density of neutralinos within selected scenarios.

  18. Design Overview of the DM Radio Pathfinder Experiment

    OpenAIRE

    Silva-Feaver, Maximiliano; Chaudhuri, Saptarshi; Cho, Hsiao-Mei; Dawson, Carl; Graham, Peter; Irwin, Kent; Kuenstner, Stephen; Li, Dale; Mardon, Jeremy; Moseley, Harvey; Mule, Richard; Phipps, Arran; Rajendran, Surjeet; Steffen, Zach; Young, Betty

    2016-01-01

    We introduce the DM Radio, a dual search for axion and hidden photon dark matter using a tunable superconducting lumped-element resonator. We discuss the prototype DM Radio Pathfinder experiment, which will probe hidden photons in the 500 peV (100 kHz)-50 neV (10 MHz) mass range. We detail the design of the various components: the LC resonant detector, the resonant frequency tuning procedure, the differential SQUID readout circuit, the shielding, and the cryogenic mounting structure. We prese...

  19. Experience on Treatment of Dermatomyositis%皮肌炎证治

    Institute of Scientific and Technical Information of China (English)

    周宝宽; 周探

    2012-01-01

    Objective : To summarize the experience of Chief Physician ZHOU Bao-kuan on the treatment of dermatomyositis based on syndrome differentiation. Methods iThe etiology, pathogenesis, types, therapeutic methods and prescriptions of dermatomyositis were expounded by the way of medical cases. Result :The etiology and pathogenesis were that innate weakness failed to bear the invasion of wind-dampness-heat, and the accumulated heat changed into toxin, resulting in the syndrome of exuberance of heat-toxin. Or innate weakness failed to bear the invasion of damp-cold, and exuberant yin-cold blocked the meridians, resulting in the syndrome of stagnation of damp-cold. Or endogenous pathogens, such as improper diet or overstrain, leaded to deficient qi and blood, which failed to circulate smoothly but stagnate, resulting in the syndrome of qi and blood deficiency. Conclusion : The treatment of dermatomyositis based on syndrome differentiation is effective.%目的:总结周宝宽主任医师辨证论治皮肌炎经验.方法:以医案形式阐述皮肌炎的病因病机、证型、治法、方药.结果:皮肌炎的病因病机为禀赋不耐,风湿热邪侵袭,蕴积化热,热极化毒形成毒热炽盛证;禀赋不耐,寒湿之邪侵袭,阴寒偏盛,痹阻经络形成寒湿凝滞证;饮食劳倦内伤,气血虚弱,运行不畅,气血痹阻,形成气血亏虚证.结论:辨证论治皮肌炎有一定疗效.

  20. A case of interstitial lung disease associated with clinically amyopathic dermatomyositis: radiologic-pathologic correlation.

    Science.gov (United States)

    Okubo, Gosuke; Noma, Satoshi; Nishimoto, Yuko; Sada, Ryuichi; Kobashi, Yoichiro

    2013-01-01

    This case report describes a 64-year-old woman with interstitial lung disease associated with clinically amyopathic dermatomyositis. Chest computed tomography revealed consolidations along bronchovascular bundles in the periphery of the lower lungs. Interstitial lung disease developed acutely, and the patient died 3 months after the clinical diagnosis. An autopsy was performed, and a large section of the lung specimen was prepared. Various interstitial lesions including organizing pneumonia, cellular and fibrotic nonspecific interstitial pneumonia, and diffuse alveolar damage were seen in the large section. Correlating the large section and computed tomography images was useful for determining the distribution of diffuse alveolar damage.

  1. LikeDM: likelihood calculator of dark matter detection

    CERN Document Server

    Huang, Xiaoyuan; Yuan, Qiang

    2016-01-01

    With the large progresses of searching for dark matter (DM) particles from indirect and direct methods, we develop a numerical tool which enables fast calculation of the likelihood of specified DM particle models given a number of observational data, such as charged cosmic rays from space-borne experiments (e.g., PAMELA, AMS-02), $\\gamma$-rays from Fermi space telescope, and the underground direct detection experiments. The purpose of this tool, \\likedm\\ --- likelihood calculator of dark matter detection, is to bridge the particle model of DM and the observational data. The intermediate steps between these two, including the astrophysical backgrounds, the propagation of charged particles, the analysis of Fermi $\\gamma$-ray data, as well as the DM velocity distribution and the nuclear form factor, have been dealt with in the code. We release the first version (v1.0) focusing on the constraints of charged cosmic and gamma rays and the direct detection part will be implemented in the next version. This manual de...

  2. Antigen list: dm3 [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available dm3 TFs and others GFP SRX016140,SRX016156,SRX016162,SRX016166,SRX016170,SRX025466,SRX025470,SRX025476,...SRX030958,SRX030960,SRX030962,SRX030964,SRX032116,SRX032121,SRX032125,SRX033313,SRX03331...5,SRX041381,SRX320155,SRX320156,SRX661230,SRX661232,SRX737176,SRX750072,SRX750083 ...

  3. Juvenil idiopatisk arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2002-01-01

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis...

  4. Juvenile Rockfish Recruitment Cruise

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — In 1983, the groundfish analysis project began a series of yearly cruises designed to assess the annual abundance of juvenile rockfish along the central California...

  5. Juvenile Idiopathic Arthritis

    Science.gov (United States)

    ... Physical Therapy Regular Exercise en español Artritis idiopática juvenil It may begin with a swollen knuckle, a ... may suddenly appear and disappear, developing in one area and then another. High fevers that tend to ...

  6. Juvenile Sex Offenders.

    Science.gov (United States)

    Ryan, Eileen P; Otonichar, Joseph M

    2016-07-01

    Sexual offending by juveniles accounts for a sizable percentage of sexual offenses, especially against young children. In this article, recent research on female juvenile sex offenders (JSOs), risk factors for offending in juveniles, treatment, and the ways in which these youth may differ from general delinquents will be reviewed. Most JSOs do not go on to develop paraphilic disorders or to commit sex offenses during adulthood, and as a group, they are more similar to nonsexual offending juvenile delinquents than to adult sex offenders. Recent research has elucidated some differences between youth who commit sex offenses and general delinquents in the areas of atypical sexual interests, the use of pornography, and early sexual victimization during childhood.

  7. Pneumatosis cystoides intestinalis in patients with antinuclear antibody negative systemic lupus erythematosus and dermatomyositis: report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Soo Yeon; Cho, On Koo; Koh, Byung Hee; Kim, Yong Soo; Song, Soon Young [College of Medicine, Hanyang University, Seoul (Korea, Republic of)

    2007-04-15

    Pneumatosis cystoides intestinalis (PCI) occurring in association with collagen vascular disease is an unusual combination that presents with intramural gas in the gastrointestinal tract. We report two cases of PCI, one with antinuclear antibody (ANA) negative SLE and the other with dermatomyositis, with a review of the relevant literature.

  8. Juvenile Spondyloarthritis Treatment Recommendations

    OpenAIRE

    Tse, Shirley; Burgos-Vargas, Ruben; Colbert, Robert A

    2012-01-01

    No specific recommendations for the treatment of juvenile spondyloarthritis have been established. Important differences exist in how spondyloarthritis begins and progresses in children and adults, supporting the need for pediatric-specific recommendations. Recently published recommendations for the treatment of juvenile arthritis consider children with sacroiliitis in a separate group, and allow for more accelerated institution of a TNF inhibitor depending on disease activity and prognostic ...

  9. Parenting and juvenile delinquency

    OpenAIRE

    Hoeve, Machteld

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. The first study addressed a meta-analysis on parenting characteristics and styles in relation to delinquency. In this meta-analysis, previous manuscripts were systematically analyzed, computing mean ...

  10. Association of anti-glomerular basement membrane antibody disease with dermatomyositis and psoriasis: case report

    Directory of Open Access Journals (Sweden)

    Natália Pereira Machado

    Full Text Available CONTEXT: Anti-glomerular basement membrane (anti-GBM antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis. CASE REPORT: A 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.

  11. Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis.

    Science.gov (United States)

    Xu, Y; Yang, C S; Li, Y J; Liu, X D; Wang, J N; Zhao, Q; Xiao, W G; Yang, P T

    2016-01-01

    Clinically amyopathic dermatomyositis (CADM) is a unique subset of dermatomyositis, showing a high incidence of lung involvements. The aim of this study is to identify risk factors, other than melanoma differentiation-associated protein (MDA)-5, for developing rapidly progressive-interstitial lung disease (RP-ILD) in patients with CADM. Forty CADM patients, in whom 11 patients developed RP-ILD, were enrolled. Clinical features and laboratory findings were compared between the patients with and without RP-ILD. We found that skin ulceration, CRP, serum ferritin, anti-MDA5 Ab, and lymphocytopenia were significantly associated with ILD. Multivariate logistic regression analysis indicated that anti-MDA5 Ab(+), elevated CRP, and decreased counts of lymphocyte were independent risk factors for RP-ILD, which can provide a precise predict for RP-ILD in CADM patients. When anti-MDA5 Ab(+) was removed from the multivariate regression model, using skin ulcerations, elevated serum ferritin and decreased counts of lymphocyte can also precisely predict RP-ILD. Except for MDA-5, more commonly available clinical characteristics, such as skin ulcerations, serum ferritin, and count of lymphocyte may also help to predict prognosis in CADM.

  12. Primary biliary cirrhosis--autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

    Science.gov (United States)

    Pamfil, Cristina; Candrea, Elisabeta; Berki, Emese; Popov, Horațiu I; Radu, Pompilia I; Rednic, Simona

    2015-03-01

    Autoimmune liver diseases may be associated with extrahepatic autoimmune pathology. We report the case of a 52-year old woman who initially presented to the gastroenterology department for extreme fatigue, pale stools, dark urine and pruritus. Laboratory tests showed significant cholestasis and elevation of aminotransferase levels. Immunological tests revealed positive antinuclear (ANA=1:320) and antimitochondrial antibodies (AMA=1:40) with negative anti-smooth muscle and liver kidney microsomal type 1 antibodies. The biopsy was compatible with overlap syndrome type 1. The patient was commenced on immunosuppressive therapy according to standard of care (azathioprine 50mg, ursodeoxycholic acid and prednisone 0.5mg/kg), with moderate biochemical improvement. She subsequently developed proximal symmetrical weakness and cutaneous involvement and was diagnosed with biopsy-proven dermatomyositis. The immunosuppressive regimen was intensified to 150 mg azathioprine. At the three-month follow-up, her symptoms subsided and aminotransferases and muscle enzymes normalized. Upon further investigation the patient was diagnosed with autoimmune thyroiditis and antiphospholipid syndrome. To our knowledge, this is the first case of primary biliary cirrhosis - autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

  13. Dermatomyositis Leading to Necrotizing Vasculitis: A Perfect Response to Applied Therapy.

    Science.gov (United States)

    Akbaryan, Mahmood; Darabi, Farideh; Soltani, Zahra

    2016-12-01

    Dermatomyositis is an idiopathic inflammatory myopathy that cause skin and muscle complications. The ethiology is not understood well yet. Released cytokines including interferon and interleukins are suggested to make inflammatory responses in the skin or muscle. Muscle weakness and skin lesions including heliotrope rash, shawl sign and Gottron's papules are the most common symptoms. A biopsy (muscle or skin) is always the most reliable method for diagnosis. Corticosteroids in association with immunosuppressive agents are used as standard treatment. The patient was a 30 years old woman who got involved with dermatomyositis for 10 years. She has been under therapy with Methotrexate, Prednisolon and Azathioprine until she came to us suffering from progressive skin lesions. Experiments and examinations were normal except the lesions and detected lipoatrophy. Because of immune cells infiltration and observations necrotizing vasculitis was diagnosed. After three month of high dose prednisolon and intravenous cyclophosphamide therapy the lesions vanished remarkable. True and immediate diagnosis gives physicians the chance not only to assess the best treatment but have adequate time to apply the procedure. However shortening the therapy and diminishing morbidity of the disease need more investigations and efforts.

  14. Malignancy associated dermatomyositis/polymyositis%恶性肿瘤相关性皮肌炎/多发性肌炎

    Institute of Scientific and Technical Information of China (English)

    蔡云雅; 方红

    2012-01-01

    Dermatomyositis/polymyositis is an inflammatory muscle disease of unknown cause,and carries elevated risks of cancers.The most associated cancers are ovarian cancer,breast cancer,lung cancer,gastroenteric cancer,and so on.Nasopharyngeal cancer is more commonly associated with dermatomyositis/polymyositis in Asian countries.It is revealed that older age at onset,the male sex,increased erythrocyte sedimentation rate and severe skin lesions are associated with a high risk of malignancy in patients with dermatomyositis/polymyositis.The pathogenesis of malignancy associated with dermatomyositis/polymyositis is thought to be a possible antigenic similarity between regenerating myoblasts and some cancer cell populations.An autoantibody against a protein with a relative molecular mass of 155 000 has proved to be a serologic marker of cancer-associated dermatomyositis/polymyositis.%皮肌炎/多发性肌炎是一种原因不明的炎性肌肉疾病,常伴发恶性肿瘤,常见的为卵巢癌、乳腺癌、肺癌、胃肠道肿瘤等,而鼻咽癌在亚洲国家较常见.恶性肿瘤相关皮肌炎/多发性肌炎可能的高危因素包括高龄、男性患者、血沉增高、严重的皮损等,其发病机制可能为肿瘤细胞与肌细胞之间相似抗原的交叉免疫反应等.血清自身抗-155抗体是癌症相关性皮肌炎/多发性肌炎的血清学监测指标.

  15. Modified DM Models for Aging Networks Based on Neighborhood Connectivity

    Institute of Scientific and Technical Information of China (English)

    WEI Du-Qu; LIN Min; LUO Xiao-Shu; WANG Gang; ZOU Yan-Li; CHEN Tian-Lun

    2008-01-01

    Two modified Dorogovtsev-Mendes (DM) models of aging networks based on the dynamics of connecting nearest-neighbors are introduced. One edge of the new site is connected to the old site with probabilityekt-αas in the DM's model, where the degree and age of the old site are k and t, respectively. We consider two eases, I.e. The other edges of the new site attaching to the nearest-neighbors of the old site with uniform and degree connectivity probability, respectively. The network structure changes with an increase of aging exponent α. It is found that the networks can produce scale-free degree distributions with small-world properties. And the different connectivity probabilities lead to the different properties of the networks.

  16. Hyperuricaemia and the metabolic syndrome in type 2 DM

    Directory of Open Access Journals (Sweden)

    Ogbera Anthonia O

    2010-04-01

    Full Text Available Abstract Background Elevated serum uric acid levels (SUA have been associated with an increased risk of cardiovascular diseases and the metabolic syndrome (MetS and are often reported to be higher in females than in males. The aim of this report is to determine the prevalence and clinical correlates of hyperuricaemia and also to evaluate associations with the MetS in people with type 2 diabetes mellitus (DM. Methods This was a cross-sectional study conducted in people with type 2 DM in Lagos, Nigeria. Hyperuricaemia was defined by cut-off values of > 7 mg/dl for men and > 6 mg/dl for women. The diagnosis of MetS was made using the new definition by the American Heart Association and other related bodies. Clinical and biochemical parameters were compared between subjects with hyperuricaemia and normouricaemia. Statistical analysis included usage of Student's t test, Pearson correlation coefficients, multivariate regression analysis and chi square. Results 601 patients with type 2 DM aged between 34-91 years were recruited for the study. The prevalence rates of hyperuricaemia and the MetS were 25% and 60% respectively. The frequency of occurrence of hyperuricaemia was comparable in both genders (59% vs 41%, p = 0.3. Although, the prevalence of the MetS in subjects with hyperuricaemia and normouricaemia was comparable (61 vs 56%, p = 0.1, a higher proportion of hyperuricaemic subjects had 3 or more components of the Mets compared with normouricaemic subjects. Possible predictors of hyperuricaemia include central obesity, smoking and elevated serum triglycerides (TG. SUA levels were found to be positively and significantly associated with serum TG (r = 0.2, p = 0.0001 and total cholesterol (r = 13, p = 0.001. Conclusion The prevalence of hyperuricaemia in subjects with type 2 DM is comparable in both genders and possible predictors of hyperuricaemia are potentially modifiable. SUA is positively and significantly associated with serum TG and total

  17. In vitro mapping of Myotonic Dystrophy (DM) gene promoter

    Energy Technology Data Exchange (ETDEWEB)

    Storbeck, C.J.; Sabourin, L. [Univ. of Ottawa (Canada); Baird, S. [Children`s Hospital of Eastern Ontario, Ottawa (Canada)] [and others

    1994-09-01

    The Myotonic Dystrophy Kinase (DMK) gene has been cloned and shared homology to serine/threonine protein kinases. Overexpression of this gene in stably transfected mouse myoblasts has been shown to inhibit fusion into myotubes while myoblasts stably transfected with an antisense construct show increased fusion potential. These experiments, along with data showing that the DM gene is highly expressed in muscle have highlighted the possibility of DMK being involved in myogenesis. The promoter region of the DM gene lacks a consensus TATA box and CAAT box, but harbours numerous transcription binding sites. Clones containing extended 5{prime} upstream sequences (UPS) of DMK only weakly drive the reporter gene chloramphenicol acetyl transferase (CAT) when transfected into C2C12 mouse myoblasts. However, four E-boxes are present in the first intron of the DM gene and transient assays show increased expression of the CAT gene when the first intron is present downstream of these 5{prime} UPS in an orientation dependent manner. Comparison between mouse and human sequence reveals that the regions in the first intron where the E-boxes are located are highly conserved. The mapping of the promoter and the importance of the first intron in the control of DMK expression will be presented.

  18. A Forming Pair of Dwarf Galaxies and Its DM Halo

    Science.gov (United States)

    Pustilnik, S. A.; Brinks, E.; Thuan, T. X.; Izotov, Yu.; Lipovetsky, V.

    SBS 0335-052 and its companion 0335-052W are shown to be a unique pair of currently forming dwarf galaxies associated with a huge HI cloud (Izotov et al. 1997, Lipovetsky et al. 1997, Thuan et al. 1997, Pustilnik et al. 1997). We present the analysis of the velocity field of this HI cloud, obtained with the VLA, and the model of its rotation curve to derive the total mass distribution in this system. We argue that this gas cloud is rotationally supported in the gravitational potential of a massive DM halo, and discuss the implications of this fact for galaxy formation and evolution scenarios. This unique system apparently preserves the original unperturbed structure of its DM halo and is thus very valuable to confront halo properties with model predictions. The parameters of the DM halo and neutral gas set limits, based on observational evidence, to the range of physical conditions under which a pristine hydrogen cloud can survive as a stable object over cosmlogical time scales. We discuss the possible effect of the massive spiral galaxy at the projected distance of about 100/h kpc to these forming galaxies.

  19. Immune dysfunction in patients with diabetes mellitus (DM).

    Science.gov (United States)

    Geerlings, S E; Hoepelman, A I

    1999-12-01

    Patients with diabetes mellitus (DM) have infections more often than those without DM. The course of the infections is also more complicated in this patient group. One of the possible causes of this increased prevalence of infections is defects in immunity. Besides some decreased cellular responses in vitro, no disturbances in adaptive immunity in diabetic patients have been described. Different disturbances (low complement factor 4, decreased cytokine response after stimulation) in humoral innate immunity have been described in diabetic patients. However, the clinical relevance of these findings is not clear. Concerning cellular innate immunity most studies show decreased functions (chemotaxis, phagocytosis, killing) of diabetic polymorphonuclear cells and diabetic monocytes/macrophages compared to cells of controls. In general, a better regulation of the DM leads to an improvement of these cellular functions. Furthermore, some microorganisms become more virulent in a high glucose environment. Another mechanism which can lead to the increased prevalence of infections in diabetic patients is an increased adherence of microorganisms to diabetic compared to nondiabetic cells. This has been described for Candida albicans. Possibly the carbohydrate composition of the receptor plays a role in this phenomenon.

  20. Is mortalin a candidate gene for T1DM ?

    Science.gov (United States)

    Johannesen, Jesper; Pie, Angeles; Karlsen, Allan Ertmann; Larsen, Zenia Marian; Jensen, Allan; Vissing, Henrik; Kristiansen, Ole Peter; Pociot, Flemming; Nerup, Jørn

    2004-01-01

    Mortalin has been found to be up-regulated by 2D-protein gel analysis in isolated rodent islets exposed to cytokines. In islets from two rat strains with different sensitivity to the toxic effects of cytokines we observed a significant difference in IL-1beta mediated mortalin expression. Constitutive over-expression of rat mortalin in NIH3T3 cells reduced cellular survival in accordance with mortalin being associated to cellular senescence. Hence we consider the gene encoding for mortalin at chromosome 5q31.1 a putative candidate gene in cytokine induced beta-cell destruction. We scanned the human mortalin gene for polymorphisms and identified three novel polymorphisms. Neither the SNPs individually nor as constructed haplotypes showed disease association tested by (E)TDT in a Danish type 1 diabetes (T1DM) population. Furthermore, we tested the D5S500 microsatelite located close to 5q31.1 without finding linkage to (T1DM). In conclusion, the functional data identifying a difference in mortalin expression in IL-1beta stimulated islets between two rat strains and over-expression of mortalin in NIH3T3 cells associated with decreased viability suggests a functional role for mortalin in cytokine mediated beta cell destruction; however, the identified polymorphisms did not reveal any association in the presence of linkage disequilibrium of mortalin to T1DM in the Danish population.

  1. Vocational Teachers' Role in Serving Juvenile Offenders.

    Science.gov (United States)

    Meers, Gary D.

    1983-01-01

    Educators need to understand the juvenile justice system to understand what juvenile offenders go through while completing their sentences. This article reviews cases and juvenile charge classifications, and presents a model for alternative sentencing options for juveniles. (JOW)

  2. 甲氨蝶呤治疗多发性肌炎/皮肌炎的系统评价%Methotrexate in the treatment of polymyositis/dermatomyositis:A qualitative systematic review

    Institute of Scientific and Technical Information of China (English)

    田小兰; 王冬雪; 王国春

    2012-01-01

    Objective:To summarize and evaluate published information on the effectiveness and adverse effects of methotrexate (MTX)in treating patients with polymyositis or dermatomyositis (PM/DM).Methods: A pubmed search from January 1966 to August 2011 was conducted with the specified search strategy, using "myositis"or "dermatomyositis"or "polymyositis"or "inflammatory myositis"and "methotrexate"as the docuterm,and also a search was conducted in CNKI and Wanfang Database for Chinese documents.In all,403 references were retrieved.We selected 148 of them to do the intensive reading by reading the abstracts.Results: Of the 16 articles selected (n=359),there were 4 randomized controlled trials of 161 patients,one prospective trial of 6 patients,8 respective studies of 176 patients,and 3 case series of 16 patients.MTX had confirmatory effectiveness in the treatment of PM/DM.Compared with azathioprine and ciclosporin A,the safety of MTX was more affirmative.Conclusion:As the important second-line therapy of PM/DM, MTX is effective confirmatively.lt is quite meaningful to use MTX at the early stage of refractory myositis.MTX therapy in combination of corti-costeroids is superior to steroid treatment alone.Side effects are common but well tolerable.%目的:总结和评价甲氨蝶呤(MTX)在多发性肌炎/皮肌炎(PM/DM)治疗中的有效性和副作用.方法:选择美国国立医学图书馆的Pubmed数据库、中国期刊全文数据库(CNKI)和万方数据库,文献发表时间截止至201 1年8月,英文检索词为“myositis”或“dermatomyositis”或“polymyositis”或“inflammatory myositis”和“methotrexate”,获取文献398篇;中文检索关键词“肌炎”和“甲氨蝶呤”,获取文献4篇,手工检索获取文献1篇.通过阅读摘要选择148篇文献进一步阅读全文.结果:纳入文献16篇,病例359例,其中包括随机对照试验研究4篇(n=161)、前瞻性研究1篇(n=6)、回顾性研究8篇(n=176)和个案总结3篇(n=16).综合

  3. Trunk asymmetry in juveniles

    Directory of Open Access Journals (Sweden)

    Triantafyllopoulos Georgios

    2008-09-01

    Full Text Available Abstract Background Trunk asymmetry (TA is a common phenomenon in children, but its incidence in juveniles is not known. The present cross sectional study reports TA in normal juveniles and provides data which describe the evolution of TA from early childhood to adolescence. Materials and methods The scoliometer readings in both standing and sitting forward bending position (FBP of 3301 children, (1645 boys, and 1656 girls aged from 3 to 9 years old were studied. TA was quantified by measuring angle of trunk rotation (ATR and children were categorized as symmetric (ATR = 0°, mild asymmetric (ATR 1° – 6° and severely asymmetric (ATR ≥ 7°. The difference of TA between standing and sitting FBP as well as differences between boys and girls in frequency of TA were also calculated. The scoliometer readings were analyzed by age to reveal at which age the juvenile pattern of TA changes into the adolescent one. Results 74.2% of boys and 77% of girls were symmetric (ATR = 0° in the thoracic region in standing FBP, while 82.7% of boys and 84.1% of girls were symmetric in the thoracic region in sitting FBP. Juvenile girls are more symmetric than boys but severe TA was found almost the same between the two genders. A significant reduction in the frequency of mild TA from standing into sitting FBP, in all the examined regions in both boys and girls was found, but in severe TA this reduction is very small. Analysing scoliometer readings by age it appears that significant TA changes take place between 8–9 years of age for boys and between 6–7 and 8–9 years for girls. TA in boys is changing into the adolescent pattern at a later age than in girls. Conclusion Juveniles were found more symmetric than adolescents, who were studied previously in a different study. Furthermore, juvenile girls were found more symmetric than boys. Juvenile TA pattern seems to be in accordance with the higher incidence of juvenile idiopathic scoliosis in boys. Furthermore

  4. Different familial association patterns of autoimmune diseases between juvenile-onset systemic lupus erythematosus and juvenile rheumatoid arthritis.

    Science.gov (United States)

    Huang, Chun-Mei; Yang, Yao-Hsu; Chiang, Bor-Luen

    2004-04-01

    The aim of this study was to determine if the prevalence of autoimmune disorders in the relatives of patients with systemic lupus erythematosus (SLE) is greater than that of relatives of patients with juvenile rheumatoid arthritis (JRA). Interviews were used to obtain histories of the following autoimmune disorders among living or deceased first-, second-, and third-degree relatives of 91 SLE and 110 JRA families: ankylosing spondylitis, SLE, rheumatoid arthritis (RA), JRA, multiple sclerosis, juvenile dermatomyositis, Sjögren's syndrome, myasthenia gravis, psoriasis, and thyroid diseases. There were statistically significant differences between the SLE and JRA probands in mean age and gender ratio (19.1 +/- 4.8 vs 14.0 +/- 5.5 years; M (male)/F (female): 17/74 vs 62/48, pJRA families (11.8%), but not statistically significantly so. The mean age (18.0 +/- 5.3 vs 14.0 +/- 4.3 years), mean age at diagnosis (13.4 +/- 4.3 vs 7.9 +/- 3.9 years) and gender ratio (F/M, 16/3 vs 5/8) of the patients with affected relatives between these 2 groups all had statistically significant differences. A higher prevalence of SLE in relatives was found in SLE families than in JRA cases. Furthermore, this study revealed a higher incidence of autoimmune disorders among second- and third-degree relatives of SLE or JRA probands versus first-degree ones, especially sisters (including 1 pair of twins) and the maternal aunt in SLE families. These data demonstrate that the prevalence of autoimmune disorders in the relatives of patients with SLE is greater than those of relatives of patients with JRA. This suggests that clinically different autoimmune phenotypes may share common susceptibility genes, which may act as risk factors for autoimmunity.

  5. Juvenile Incarceration and Health.

    Science.gov (United States)

    Barnert, Elizabeth S; Perry, Raymond; Morris, Robert E

    2016-03-01

    Addressing the health status and needs of incarcerated youth represents an issue at the nexus of juvenile justice reform and health care reform. Incarcerated youth face disproportionately higher morbidity and higher mortality compared to the general adolescent population. Dental health, reproductive health, and mental health needs are particularly high, likely as a result of lower access to care, engagement in high-risk behaviors, and underlying health disparities. Violence exposure and injury also contribute to the health disparities seen in this population. Further, juvenile incarceration itself is an important determinant of health. Juvenile incarceration likely correlates with worse health and social functioning across the life course. Correctional health care facilities allow time for providers to address the unmet physical and mental health needs seen in this population. Yet substantial challenges to care delivery in detention facilities exist and quality of care in detention facilities varies widely. Community-based pediatricians can serve a vital role in ensuring continuity of care in the postdetention period and linking youth to services that can potentially prevent juvenile offending. Pediatricians who succeed in understanding and addressing the underlying social contexts of their patients' lives can have tremendous impact in improving the life trajectories of these vulnerable youth. Opportunities exist in clinical care, research, medical education, policy, and advocacy for pediatricians to lead change and improve the health status of youth involved in the juvenile justice system.

  6. Perbandingan kadar hs-CRP pada penderita DM tipe 2 yang dislipidemi dan nondislipidemi

    OpenAIRE

    Pasaribu, Juli Y.M

    2016-01-01

    Introduction : DM type 2 is a global health problem which the prevalence is getting more increased. One of the complications DM type 2 is the coronary artery disease (CAD). CAD is caused by the endothelial dysfunction which later becomes atherosclerosis. Besides DM, dyslipidemia might cause the endothelial dysfunction. DM and dyslipidemia, each of them might cause the endothelium dysfunction, furthermore, if it is found that the DM patients suffer dyslipidemia, it will increase the risk of th...

  7. Extending juvenility in grasses

    Energy Technology Data Exchange (ETDEWEB)

    Kaeppler, Shawn; de Leon Gatti, Natalia; Foerster, Jillian

    2017-04-11

    The present invention relates to compositions and methods for modulating the juvenile to adult developmental growth transition in plants, such as grasses (e.g. maize). In particular, the invention provides methods for enhancing agronomic properties in plants by modulating expression of GRMZM2G362718, GRMZM2G096016, or homologs thereof. Modulation of expression of one or more additional genes which affect juvenile to adult developmental growth transition such as Glossy15 or Cg1, in conjunction with such modulation of expression is also contemplated. Nucleic acid constructs for down-regulation of GRMZM2G362718 and/or GRMZM2G096016 are also contemplated, as are transgenic plants and products produced there from, that demonstrate altered, such as extended juvenile growth, and display associated phenotypes such as enhanced yield, improved digestibility, and increased disease resistance. Plants described herein may be used, for example, as improved forage or feed crops or in biofuel production.

  8. DERMATOMIOSITIS JUVENIL Y EMBARAZO

    OpenAIRE

    Evans M,Gregorio; Poulsen R,Ronald; Blanco R,Romiely; Luna V,Viviana

    2002-01-01

    La dermatomiositis juvenil es un desorden inflamatorio crónico multisistémico del tejido conectivo. Tiene una incidencia de 2-3/100.000/año. Con la disminución en la mortalidad experimentada en los últimos decenios, la atención está cifrada en la morbilidad a largo plazo y en las alteraciones funcionales. Con un tratamiento agresivo los niños con dermatomiositis juvenil generalmente tienen un futuro promisorio, sin incapacidad o con incapacidad mínima. La mortalidad actualmente se estima cerc...

  9. [Characterization of a thermophilic Geobacillus strain DM-2 degrading hydrocarbons].

    Science.gov (United States)

    Liu, Qing-kun; Wang, Jun; Li, Guo-qiang; Ma, Ting; Liang, Feng-lai; Liu, Ru-lin

    2008-12-01

    A thermophilic Geobacillus strain DM-2 from a deep-subsurface oil reservoir was investigated on its capability of degrading crude oil under various conditions as well as its characters on degrading hydrocarbons in optimal conditions. The results showed that Geobacillus strain DM-2 was able to degrade crude oil under anoxic wide-range conditions with pH ranging from 4.0 to 10.0, high temperature in the range of 45-70 degrees C and saline concentration ranging from 0.2% to 3.0%. Furthermore, the optimal temperature and pH value for utilizing hydrocarbons by the strain were 60 degrees C and 7.0, respectively. Under such optimal conditions, the strain utilized liquid paraffine emulsified by itself as its carbon source for growth; further analysis by gas chromatography (GC) and infrared absorption spectroscopy demonstrated that it was able to degrade n-alkanes (C14-C30), branched-chain alkanes and aromatic hydrocarbons in crude oil and could also utilize long-chain n-alkanes from C16 to C36, among of which the degradation efficiency of C28 was the highest, up to 88.95%. One metabolite of the strain oxidizing alkanes is fatty acid.While utilizing C16 as carbon source for 5 d, only one fatty acid-acetic acid was detected by HPLC and MS as the product, with the amount of 0.312 g/L, which indicated that it degraded n-alkanes with pathway of inferior terminal oxidation,and then followed by a beta-oxidation pathway. Due to its characters of efficient emulsification, high-performance degradation of hydrocarbons and fatty-acid production under high temperature and anoxic condition, the strain DM-2 may be potentially applied to oil-waste treatment and microbial enhanced heavy oil recovery in extreme conditions.

  10. Identifying Bank Frauds Using CRISP-DM and Decision Trees

    Directory of Open Access Journals (Sweden)

    Bruno Carneiro da Rocha

    2010-10-01

    Full Text Available This article aims to evaluate the use of techniques of decision trees, in conjunction with the managementmodel CRISP-DM, to help in the prevention of bank fraud. This article offers a study on decision trees, animportant concept in the field of artificial intelligence. The study is focused on discussing how these treesare able to assist in the decision making process of identifying frauds by the analysis of informationregarding bank transactions. This information is captured with the use of techniques and the CRISP-DMmanagement model of data mining in large operational databases logged from internet banktransactions.

  11. Effects of Growth Temperature and Postharvest Cooling on Anthocyanin Profiles in Juvenile and Mature Brassica oleracea.

    Science.gov (United States)

    Socquet-Juglard, Didier; Bennett, Alexandra A; Manns, David C; Mansfield, Anna Katharine; Robbins, Rebecca J; Collins, Thomas M; Griffiths, Phillip D

    2016-02-24

    The effects of growth temperatures on anthocyanin content and profile were tested on juvenile cabbage and kale plants. The effects of cold storage time were evaluated on both juvenile and mature plants. The anthocyanin content in juvenile plants ranged from 3.82 mg of cyanidin-3,5-diglucoside equivalent (Cy equiv)/g of dry matter (dm) at 25 °C to 10.00 mg of Cy equiv/g of dm at 16 °C, with up to 76% diacylated anthocyanins. Cold storage of juvenile plants decreased the total amount of anthocyanins but increased the diacylated anthocyanin content by 3-5%. In mature plants, cold storage reduced the total anthocyanin content from 22 to 12.23 mg/g after 5 weeks of storage in red cabbage, while the total anthocyanin content increased after 2 weeks of storage from 2.34 to 3.66 mg of Cy equiv/g of dm in kale without having any effect on acylation in either morphotype. The results obtained in this study will be useful for optimizing anthocyanin production.

  12. Implementing VLPR systems based on TMS320DM642

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    This paper gives a practical schema for using DSP boards to construct Vehicle License Plate Recognition (VLPR) modules that could be embedded in any Intelligent Transportation System (ITS). Using DSP can avoid the heavy investment in dedicated VLPR system and improve the computational power compared to PC software environment. Low cost, high computational power, and high flexibility of DSP provide the License Plate Recognition System (LPRS) an excellent cost-effective solution to execute the major part of the recognition tasks. This paper describes a successful implementation of VLPR system based on Texas Instruments (TI)'s TMS320DM642. The DSP board acquires video (which could be output to a monitor for surveillance)from a camera, captures images from the video, locates and recognizes the license plates in images, and then sends the recognized results and related images after compression to a host PC through the network. Finally, the overall software is optimized according to the features of DM642 chip. Experiments showed that the DSP VLPR system performs well on the local license plates, and that the processing speed and accuracy can meet the requirement of practical applications.

  13. Juvenile Battens Disease.

    Science.gov (United States)

    Gayton, Romayne

    1987-01-01

    Ten children diagnosed with juvenile Battens disease were tested over a three-year period in general intelligence, memory, listening and speech, motor skills, and general learning. Results showed that the patients followed a predetermined pattern but that the time span for development of memory, communication, and behavior problems varied greatly.…

  14. Juvenile Victimization and Delinquency.

    Science.gov (United States)

    Esbensen, Finn-Aage; Huizinga, David

    1991-01-01

    Demographic characteristics of juvenile victims of crime and a potential relationship between victimization and self-reported delinquency are examined for 877 adolescents from a large midwestern city. Lifetime victimization rates (LVRs) are higher for those involved in delinquency, and LVRs rise with age and higher levels of delinquent behavior.…

  15. Juvenile Battens Disease.

    Science.gov (United States)

    Gayton, Romayne

    1987-01-01

    Ten children diagnosed with juvenile Battens disease were tested over a three-year period in general intelligence, memory, listening and speech, motor skills, and general learning. Results showed that the patients followed a predetermined pattern but that the time span for development of memory, communication, and behavior problems varied greatly.…

  16. Juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Prakken, Berent; Albani, Salvatore; Martini, Alberto

    2011-01-01

    Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years. Pivotal studies in the past 5 years have led to substantial progress in various areas, ranging from disease classification to new treatments. Gene expres

  17. Early Illness Features Associated with Mortality in the Juvenile Idiopathic Inflammatory Myopathies

    Science.gov (United States)

    Huber, Adam M.; Mamyrova, Gulnara; Lachenbruch, Peter A.; Lee, Julia A.; Katz, James D.; Targoff, Ira N.; Miller, Frederick W.; Rider, Lisa G.

    2015-01-01

    Objectives Because juvenile idiopathic inflammatory myopathies (JIIM) are potentially life-threatening systemic autoimmune diseases, we examined risk factors for JIIM mortality. Methods Mortality status was available for 405 patients (329 juvenile dermatomyositis [JDM], 30 juvenile polymyositis [JPM], 46 juvenile connective tissue disease–associated myositis [JCTM]) enrolled in nationwide protocols. Standardized mortality ratios (SMR) were calculated using United States population statistics. Cox regression was used to assess univariable associations with mortality, and random survival forest (RSF) classification and Cox regression for multivariable associations. Results Of 17 deaths (4.2% overall mortality), 8 (2.4%) were in JDM patients. Death was related to the pulmonary system, primarily interstitial lung disease (ILD), in 7 patients, gastrointestinal in 3, multisystem in 3, and of unknown etiology in 4 patients. The SMR for JIIM overall was 14.4 [95% confidence interval (CI) 12.2, 16.5] and 8.3 [95% CI 6.4, 10.3] for JDM. The top mortality risk factors in the univariable analysis included clinical subgroup (JCTM, JPM), anti-synthetase autoantibodies, older age at diagnosis, ILD and Raynaud’s phenomenon at diagnosis. In multivariable analyses, clinical subgroup, illness severity at onset, age at diagnosis, weight loss and delay to diagnosis were the most important predictors from RSF; clinical subgroup and illness severity at onset were confirmed by multivariable Cox regression. Conclusions Overall mortality was higher in JIIM patients, and several early illness features were identified as risk factors. Clinical subgroup, anti-synthetase autoantibodies, older age at diagnosis, and ILD are also recognized as mortality risk factors in adult myositis. PMID:24151254

  18. A Study on Clinical Activity of Myositis by the Use of Radioisotope Bone Scan in the Patients with Dermatomyositis - polymyositis

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Sung Jae; Koh, Chang Soon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1982-03-15

    To evaluate the diagnostic usefulness of radioisotope bone scan and clinical activity of myositis, bone scan using {sup 99m}Tc-Methylene diphosphonate was serially done before and after treatment with prednisolone in 10 patients with well- documented dermatomyositis-polymyositis. The observed results were as follows. 1. In all 10 patients before treatment with prednisolone, the bone scans showed evidence of increased muscle uptake. Muscle uptake was markedly increased in 4 patients, moderately increased in 3 patients and minimally increased in 3 patients. 2. The site of increased muscle uptake was consistent with the site of clinically involved muscle which was weak and tender. 3. The degree of muscle uptake correlated with the severity of the muscle weakness and tenderness at the scan was done. In all 10 patients treated with high dose prednisolone, muscle uptake was decreased following therapy. Above results suggest the radioisotope bone scanning may be useful in the diagnosis and treatment of patient with dermatomyositis-polymyositis.

  19. 多发性肌炎或皮肌炎心脏损害的临床分析%The clinical analysis of cardiac manifestation in polymyositis or dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    佟胜全; 周新福; 张奉春

    2005-01-01

    目的分析多发性肌炎或皮肌炎(polymyositis/dermatomyositis,PM/DM)心脏损害的特征,探讨心脏损害发生的危险因素.方法收集423例PM/DM的临床资料,分析心脏损害的发生率、心脏损害的类型以及可能诱发心脏损害的危险因素.结果心脏损害的发生率为42.6%(180/423),表现为心肌缺血、心律失常、心包炎、心肌损害、肺动脉高压、瓣膜病变、传导阻滞、肢导低电压和其他心脏损害;多因素分析发现血沉是心脏损害发生的危险因素.结论心脏损害是PM/DM的常见并发症,表现复杂多样.血沉增快提示心脏损害的危险性增加,而与年龄、性别及病程长短等因素无关.

  20. Clinical characteristics and survival analysis of patients with dermatomyositis combined malignant tumor%皮肌炎合并恶性肿瘤的临床资料分析

    Institute of Scientific and Technical Information of China (English)

    蔡娅菲; 梁燕; 李芳

    2014-01-01

    Objective To analyze the clinical characteristics and survival time of patients with dermatomyositis ( DM) combined with malignant tumor .Methods Twenty-four patients with dermatomyositis associated with malignant tumor were enrolled;the clinical data were collected during hospitalization ( including the first symptoms , skin rash, muscular per-formance etc.) and serological indicators (including related antibody, muscle enzyme spectrum, erythrocyte sedimentation rate, and C reactive protein ) were used for data analysis and 3 year follow-up after discharge were performed .Results The 24 cases of dermatomyositis associated with malignant tumor patients were aged between 45-87 years old, male and female ratio was 1∶1.18, including 6 lung cancer cases and 6 nasopharyngeal carcinoma cases (both were 25.0%), 3 cases (12.5%) of breast cancer, 2 cases of ovarian cancer and 2 cases of thymic carcinoma (both were 8.3%), 1 case of renal cell carcinoma , 1 case of gastric cancer , 1 case of cervical cancer , 1 case of intestinal tumor , 1 case of metastasis ( unknown primary tumor ) (both were 4.2%).Among the 24 patients, 13 cases were malignant erythema (54.2%), loss of body mass in 10 cases (41.7%);laboratory examination of anti-nuclear antigem(ANA) positive in 22 cases (91.7%), Jo-1 antibodies was nega-tive in all of these cases.During the follow-up period, 16 cases were dead (66.7%), 8 cases (33.3%) were still alive.The main causes of death include dermatomyositis complications , tumor metastasis related multiple organ failure , pulmonary infec-tion related severe respiratory failure , fulminant hepatitis;all patients were followed up for 3 years, 1 year’s survival rate was 50.0%, 3 year’s survival rate was 33.3%.Conclusion Lung cancer and nasopharyngeal carcinoma were the most common malignant tumors in dermatomyositis patients , followed by breast cancer , ovarian cancer , breast cancer , its revealed high fa-tality rate.Malignant erythema , loss of body weight

  1. HRCT findings and the response to the treatment for interstitial pneumonia in patients with polymyositis and dermatomyositis

    Energy Technology Data Exchange (ETDEWEB)

    Abo, Hitoshi [Toyama Prefectural Central Hospital (Japan); Kobayashi, Takeshi; Shibata, Yoshihiro; Matsui, Osamu

    2002-01-01

    HRCT findings and the response to the treatment for interstitial pneumonia were evaluated retrospectively in 9 patients with polymyositis and dermatomyositis. Ground-glass attenuation and consolidation were observed most frequently on HRCT images. The response to the treatment for cases with ground-glass attenuation and consolidation were initially tend to improve, but considerable different in the long run. Therefore we considered the response to the treatment would be difficult to expect on each case. (author)

  2. Aberrant intracellular metabolism of T-DM1 confers T-DM1 resistance in HER2-positive gastric cancer cells.

    Science.gov (United States)

    Wang, Hongbin; Wang, Wenqian; Xu, Yongping; Yang, Yong; Chen, Xiaoyan; Quan, Haitian; Lou, Liguang

    2017-04-07

    T-DM1 (Kadcyla), an antibody-drug conjugate (ADC) consisting of HER2-targeted monoclonal antibody trastuzumab linked to anti-microtubule agent mertansine (DM1), has been approved for the treatment of HER2-positive metastatic breast cancer. To date, acquired resistance arises to be a major obstacle to T-DM1 treatment, and mechanisms remain incompletely understood. In the present study, we established a T-DM1-resistant N87-KR cell line from HER2-positive N87 gastric cancer cells to investigate mechanisms of acquired resistance and develop strategies for overcoming it. Although the kinetics of binding, internalization, and externalization of T-DM1 were the same in N87-KR cells and N87 cells, N87-KR was strongly resistant to T-DM1, but remained sensitive to both trastuzumab and DM1. T-DM1 failed to inhibit microtubule polymerization in N87-KR cells. Consistently, lysine-MCC-DM1, the active T-DM1 metabolite that inhibits microtubule polymerization, accumulated much lesser in N87-KR cells. Furthermore, lysosome acidification, achieved by V-ATPase, was much diminished in N87-KR cells. Notably, treatment of sensitive N87 cells with the V-ATPase-selective inhibitor Baf-A1 induced T-DM1 resistance, suggesting that aberrant V-ATPase activity decreases T-DM1 metabolism, leading to T-DM1 resistance in N87-KR cells. Interestingly, HER2-targeted ADCs containing a protease-cleavable linker, such as hertuzumab-vc-MMAE, were capable of efficiently overcoming this resistance. Our results demonstrate for the first time that a decrease in T-DM1 metabolites induced by aberrant V-ATPase activity contributes to T-DM1 resistance, which could be overcome by HER2-targeted ADC containing different linkers, including a protease-cleavable linker. Accordingly, we propose that V-ATPase activity in lysosomes is a novel biomarker for predicting T-DM1 resistance. This article is protected by copyright. All rights reserved.

  3. 皮肌炎合并甲状腺功能异常的临床分析%Clinical analysis of dermatomyositis associated with thyroid dysfunction

    Institute of Scientific and Technical Information of China (English)

    田小兰; 张思功; 舒晓明; 章璐; 王国春

    2013-01-01

    Objective To study the clinical and laboratory data of dermatomyositis (DM)complicated with thyroid dysfunction.Methods A retrospective analysis of 112 patients with DM was conducted to compare the clinical manifestations,laboratory features and mortality differences between the group with thyroid dysfunction and the group without.Results 48.2% of DM patients had thyroid dysfunction.The most common subgroup of thyroid dysfunction was euthyroid sick syndrome (ESS) (31.3%),followed by hypothyroidism (10.7%) and hyperthyroidism (6.3%).DM patients with ESS were more likely to have higher incidence of interstitial lung disease (ILD) and arthritis,as well as higher levels of C-reactive protein(CRP) and ferritin (Fet),and lower level of hemoglobin (HGB) (P < O.05),and showed a higher incidence of deaths (P =0.006).The subgroup with hypothyroidism were prone to have gastrointestinal involvement and proteinuria,with higher levels of IgM,thyroid peroxidase antibodies(TPOAb) and thyroglobulin antibodies (TGAb)(P < 0.05).The hyperthyroidism group had a significant higer level of cholesterol(CHO) (P < 0.05).The incidence of DM associated with AITD was 5.4%.The most commonly seen AITD was Hashimoto's thyroiditis(HT) (3.6%).Conclusions Thyroid dysfunction is common in DM.ESS was most frequently seen and exhibited a higher incidence of deaths.AITD associated with DM is not rare.The most commonly seen type is HT.%目的 探讨合并甲状腺功能异常的皮肌炎(DM)患者临床及实验室检查特征.方法 回顾性分析112例DM患者,比较甲状腺功能异常组与正常组患者临床表现、实验室检查特点及死亡率差异.结果 112例DM患者中甲状腺功能异常者达48.2%,最常见表现为正常甲状腺病态综合征(ESS)(31.3%),其次是甲状腺功能减退(简称甲减)(10.7%)和甲状腺功能亢进(简称甲亢)(6.3%).与正常组相比,ESS组患者多见合并关节炎和间质性肺疾病(ILD),

  4. STT Doubles with Large DM - Part IV: Ophiuchus and Hercules

    Science.gov (United States)

    Knapp, Wilfried; Nanson, John

    2016-04-01

    The results of visual double star observing sessions suggested a pattern for STT doubles with large DM of being harder to resolve than would be expected based on the WDS catalog data. It was felt this might be a problem with expectations on one hand, and on the other might be an indication of a need for new precise measurements, so we decided to take a closer look at a selected sample of STT doubles and do some research. We found that like in the other constellations covered so far (Gem, Leo, UMa, etc.) at least several of the selected objects in Ophiuchus and Hercules show parameters quite different from the current WDS data.

  5. SELF MANAGEMENT INTERVENTION INCREASING COMPLIANCE IN PATIENT WITH DM

    Directory of Open Access Journals (Sweden)

    Siti Nur Kholifah

    2017-01-01

    Full Text Available Introduction: Diabetes mellitus (DM was a degenerative disease which often found in the community. Diabetes was caused by unhealthy habits, such as overeating, lack of exercise, and stress. The purpose of this study was to identify selfmanagement as one of the interventions that can improve treatment compliance in patients with diabetes. Method: This study was used quasy experiments non randomized pretest-posttest design. Samples were 20 families who lived with type 2 diabetes patient. Variable independent was self management intervention and variable dependent was patient complience. Data were collected by using interview, food recall, and observation on behavioral change. Data then analyzed by using paired t-test with α≤0.05. Results: The results had showed that before intervention only 3 (15 % respondents who obey diabetes diet, then increase to 19 (95 % respondents after intervention with p value=0.000. Patient’s medication compliance also increased, from 6 (30 % respondents before intervention to 20 (100% respondents after intervention, with p value= 0.000. Patient compliance on exercise also increase from 2 (10% respondents before intervention, become 19 (95% respondents after intervention, with p value=0.000. Discussion: Self management intervention could improve patient’s knowledge, problem-solving skills, and self-effi cacy. Self management should be done after the patient had understand their disease and realized the importance of self-care. Community health nurses were expected to implement self management as one of nursing intervention, so that patient compliance on their treatment can be increased. Key words: Self management intervention, compliance, patient DM type 2

  6. Case report of anti-transcription intermediary factor-1-γ/α antibody-positive dermatomyositis associated with gastric cancer and immunoglobulin G4-positive pulmonary inflammatory pseudotumor.

    Science.gov (United States)

    Ito, Kotaro; Imafuku, Shinichi; Hamaguchi, Yasuhito; Fujimoto, Manabu; Nakayama, Juichiro

    2013-07-01

    Dermatomyositis is a rare connective tissue disease often associated with internal malignancy and interstitial pneumonitis. Serologically, various auto-antibodies (Ab) are associated with dermatomyositis. Anti-transcription intermediary factor-1-γ/α (TIF-1-γ/α) Ab was recently identified as an auto-Ab and was observed mostly in cancer-associated dermatomyositis. IgG4-related disease is a newly described entity characterized by increased serum IgG4 levels and IgG4-positive plasma cell infiltration with fibrosis in organs such as the pancreas and parotid gland. IgG4-related disease also includes inflammatory pseudotumors in various organs. We report herein a 59-year-old Japanese man who had dermatomyositis complicated with a gastric cancer and an IgG4-related pulmonary inflammatory pseudotumor. He manifested typical classical Gottron's papules on the fingers, V-sign erythema on the chest, flagellate erythema on the back, nail fold bleeding and facial erythema. Serum levels of anti-TIF-1-γ/α Ab were positive as assessed by immunoprecipitation assay. He also had bilateral swelling of the parotid gland, and an excised specimen of the lung showed inflammatory pseudotumor with IgG4-positive plasma cells. As far as we know, this case is the first to report the association of IgG4-related disease and TIF-1-γ/α-positive dermatomyositis. Further accumulation of such cases is required to elucidate the mechanism of this association.

  7. Late Onset Juvenile Xanthogranuloma

    Directory of Open Access Journals (Sweden)

    Punithwavathy K

    1999-01-01

    Full Text Available A 19 year old female was seen with multiple skin coloured and hyperpigmented macules, discrete as well as grouped papules and nodules of varying sizes distributed over the face, neck, extensor and flexor aspects of both upper and lower extremities including joints. The trunk was spared. Some of the lesions showed features of spontaneous regression. Investigations confirmed the diagnosis of juvenile xanthogranuloma. Lesions regressed satisfactorily with liquid nitrogen cryotherapy.

  8. Juvenile Incarceration and Health

    OpenAIRE

    Barnert, ES; R Perry; Morris, RE

    2015-01-01

    © 2015. Addressing the health status and needs of incarcerated youth represents an issue at the nexus of juvenile justice reform and health care reform. Incarcerated youth face disproportionately higher morbidity and higher mortality compared to the general adolescent population. Dental health, reproductive health, and mental health needs are particularly high, likely as a result of lower access to care, engagement in high-risk behaviors, and underlying health disparities. Violence exposure a...

  9. Juvenile Ultracool Dwarfs

    CERN Document Server

    Rice, Emily L; Cruz, Kelle; Barman, Travis; Looper, Dagny; Malo, Lison; Mamajek, Eric E; Metchev, Stanimir; Shkolnik, Evgenya L

    2011-01-01

    Juvenile ultracool dwarfs are late spectral type objects (later than ~M6) with ages between 10 Myr and several 100 Myr. Their age-related properties lie intermediate between very low mass objects in nearby star-forming regions (ages 1-5 Myr) and field stars and brown dwarfs that are members of the disk population (ages 1-5 Gyr). Kinematic associations of nearby young stars with ages from ~10-100 Myr provide sources for juvenile ultracool dwarfs. The lowest mass confirmed members of these groups are late-M dwarfs. Several apparently young L dwarfs and a few T dwarfs are known, but they have not been kinematically associated with any groups. Normalizing the field IMF to the high mass population of these groups suggests that more low mass (mainly late-M and possibly L dwarf) members have yet to be found. The lowest mass members of these groups, along with low mass companions to known young stars, provide benchmark objects with which spectroscopic age indicators for juvenile ultracool dwarfs can be calibrated and...

  10. Dermatomyositis With pneumomediastinum%皮肌炎并发纵隔气肿

    Institute of Scientific and Technical Information of China (English)

    朱剑; 黄烽

    2001-01-01

    Objective: To acknowledge the possible associated factors of dermatomyositis and pneumomediastinurn.Methods: Two patients with dermatomyositis coaplicating spontaneous pneumomediatinum were presented andthe related literatures were reviewed. Resuts:By analysing the clinical information of 13 cases from literatures and our own cases, we found that 72.7% of these patients had normal CPK at the time when pneumomediastinum happened; 38. 4% had remittent complications and 46. 2% died; 53. 8% had previouse pulmonary diseases. There were obvious skin changes in the two cases. Moreover, one of ours and other 4 patients from literatures had multiple skin ulcers indicating the cutanuous vasculitis was active. Conclusion: Dermatomyositis patients with previous pulmonary diseases, normal or slightly elevated serum muscular enzymes and dominant skin changes are more likely to suffer from complication of pneumomediastinurn than those with obvious muscular damage and polymyositis patients. The reason is probably because of the existence of pulmonary fibrosis and active vasculitis.%目的:提高对皮肌炎并发纵隔气肿的各种临床相关因素的认识。方法:分析两例皮肌炎并发纵隔气肿患者的详细临床资料及复习有关文献,了解各种临床表现与发生这种并发症之间的可能联系。结果:对本文及通过MEDL1NE查阅的国外文献记载的13例患者临床资料进行了总结。发生纵隔气肿时有72.7%CPK完全正常,肌炎处于临床缓解期;38.4%纵隔气肿呈反复发作,46.2%死亡,表明皮肌炎并发纵隔气肿的预后不佳。53.8%既往有肺部疾病,提示纵隔气肿的发生的确与肺部原有病变及肺纤维化有密切关系。本文两位患者皮肤仍有明显的病变,其中1例和文献报道的4例患者还有多发皮肤溃疡,表明皮肤的血管炎仍处于活动状态。结论:既往有肺部疾患、血清肌酶不高或轻度升高、以皮肤改变为主的

  11. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... Home Health Conditions juvenile idiopathic arthritis juvenile idiopathic arthritis Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  12. Genetics Home Reference: juvenile primary osteoporosis

    Science.gov (United States)

    ... Home Health Conditions juvenile primary osteoporosis juvenile primary osteoporosis Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Juvenile primary osteoporosis is a skeletal disorder characterized by thinning of ...

  13. Genetics Home Reference: juvenile myoclonic epilepsy

    Science.gov (United States)

    ... Home Health Conditions juvenile myoclonic epilepsy juvenile myoclonic epilepsy Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Juvenile myoclonic epilepsy is a condition characterized by recurrent seizures (epilepsy). ...

  14. Clinical and Pathological Analysis of Dermatomyositis Combined with Cardiac Orifice Cancer%皮肌炎合并贲门癌的临床及病理特点分析

    Institute of Scientific and Technical Information of China (English)

    康增军; 王芳; 李娜

    2013-01-01

    目的 分析皮肌炎(DM)合并贲门癌患者的临床及病理特点.方法 收集1例DM合并贲门癌患者的临床表现及开放式骨骼肌活体组织检查(活检)、组织化学染色病理与手术切除贲门病变组织的病理结果资料,进行回顾性分析.结果 本例为老年男性患者,临床表现为四肢近端肌肉伴吞咽肌无力2个月.查体:双上眼睑轻度水肿性紫红斑,咀嚼肌、吞咽肌肌力弱,髂腰肌、四肢近端肌肌力Ⅲ+级、远端肌肌力Ⅴ-级.实验室检查:红细胞沉降率46 mm/h、血肌酸激酶145 U/L、补体C4 0.44 g/L,均增高,C反应蛋白水平正常.肌电图:部分被检肌肉可见震颤、正相电位与波幅低、时限窄多相电位.活检骨骼肌组织化学染色光镜病理:可见典型束周肌纤维萎缩,肌内膜、肌束膜、血管周围炎性细胞浸润,未见坏死肌纤维.病理诊断为DM.电子纤维胃镜示:贲门灶状黏膜腺体重度不典型增生,可疑癌变.手术切除的贲门病变组织病理诊断为腺癌.结论 骨骼肌活检病理诊断是确诊DM的必备条件.中老年DM患者易并发恶性肿瘤,应积极行相关肿瘤筛查.%Objective To analyze clinical and pathological features of patients with dermatomyositis ( DM ) combined with cardiac orifice cancer. Methods Clinical data, skeletal muscle biopsy, pathological results of histochemistry staining and surgical resection of cardiac tissues of a patient with DM combined with cardiac orifice cancer were retrospectively analyzed. Results Clinical manifestations of the elderly male patient were limbs proximal muscle progressive weakness and mild dysphagia for more than 2 months. Clinical examination showed a heliotrope rash on the upper eyelids with mild oedema, the strength of masse-ter and swallowing muscle were weak, muscle strength score of iliopsoas and limbs proximal muscles were III + , of distal muscles were V -. Laboratory examination; erythrocyte sedimentation rate ( ESR ) 46 mm

  15. Juvenile Justice Bulletin: Aftercare Services. Juvenile Justice Practices Series.

    Science.gov (United States)

    Gies, Steve V.

    This bulletin examines aftercare services that provide youth with comprehensive health, education, family, and vocational services upon their release from the juvenile justice system. Aftercare can be defined as reintegrative services that prepare out-of-home placed juveniles for reentry into the community by reestablishing the necessary…

  16. DM100 AND DM1200 MELTER TESTING WITH HIGH WASTE LOADING GLASS FORMULATIONS FOR HANFORD HIGH-ALUMINUM HLW STREAMS

    Energy Technology Data Exchange (ETDEWEB)

    KRUGER AA; MATLACK KS; KOT WK; PEGG IL; JOSEPH I

    2009-12-30

    This Test Plan describes work to support the development and testing of high waste loading glass formulations that achieve high glass melting rates for Hanford high aluminum high level waste (HLW). In particular, the present testing is designed to evaluate the effect of using low activity waste (LAW) waste streams as a source of sodium in place ofchemical additives, sugar or cellulose as a reductant, boehmite as an aluminum source, and further enhancements to waste processing rate while meeting all processing and product quality requirements. The work will include preparation and characterization of crucible melts in support of subsequent DuraMelter 100 (DM 100) tests designed to examine the effects of enhanced glass formulations, glass processing temperature, incorporation of the LAW waste stream as a sodium source, type of organic reductant, and feed solids content on waste processing rate and product quality. Also included is a confirmatory test on the HLW Pilot Melter (DM1200) with a composition selected from those tested on the DM100. This work builds on previous work performed at the Vitreous State Laboratory (VSL) for Department of Energy's (DOE's) Office of River Protection (ORP) to increase waste loading and processing rates for high-iron HLW waste streams as well as previous tests conducted for ORP on the same waste composition. This Test Plan is prepared in response to an ORP-supplied statement of work. It is currently estimated that the number of HLW canisters to be produced in the Hanford Tank Waste Treatment and Immobilization Plant (WTP) is about 12,500. This estimate is based upon the inventory ofthe tank wastes, the anticipated performance of the sludge treatment processes, and current understanding of the capability of the borosilicate glass waste form. The WTP HLW melter design, unlike earlier DOE melter designs, incorporates an active glass bubbler system. The bubblers create active glass pool convection and thereby improve heat

  17. Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis

    Directory of Open Access Journals (Sweden)

    Marcela Gran Pina Cruellas

    2013-07-01

    Full Text Available OBJECTIVE: To analyze the prevalence of myositis-specific and myositis-associated autoantibodies and their clinical correlations in a large series of patients with dermatomyositis/polymyositis. METHOD: This cross-sectional study enrolled 127 dermatomyositis cases and 95 polymyositis cases. The disease-related autoantibody profiles were determined using a commercially available blood testing kit. RESULTS: The prevalence of myositis-specific autoantibodies in all 222 patients was 34.4%, whereas myositis-associated autoantibodies were found in 41.4% of the patients. The most frequently found autoantibody was anti-Ro-52 (36.9%, followed by anti-Jo-1 (18.9%, anti-Mi-2 (8.1%, anti-Ku (4.1%, anti-SRP (3.2%, anti-PL-7 (3.2%, anti-PL-12 (2.7%, anti-PM/Scl75 (2.7%, and anti-PM/Scl100 (2.7%. The distributions of these autoantibodies were comparable between polymyositis and dermatomyositis, except for a higher prevalence of anti-Jo-1 in polymyositis. Anti-Mi-2 was more prevalent in dermatomyositis. Notably, in the multivariate analysis, anti-Mi-2 and anti-Ro-52 were associated with photosensitivity and pulmonary disorders, respectively, in dermatomyositis. Anti-Jo-1 was significantly correlated with pulmonary disorders in polymyositis. Moreover, anti-Ro-52 was associated with anti-Jo-1 in both diseases. No significant correlation was observed between the remaining autoantibodies and the clinical and/or laboratory findings. CONCLUSIONS: Our data are consistent with those from other published studies involving other populations, although certain findings warrant consideration. Anti-Ro-52 and anti-Jo-1 were strongly associated with one another. Anti-Ro-52 was correlated with pulmonary disorders in dermatomyositis, whereas anti-Jo-1 was correlated with pulmonary alterations in polymyositis.

  18. Miastenia gravis juvenil Juvenile myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Oscar Papazian

    2009-01-01

    Full Text Available La miastenia gravis juvenil (MGJ es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos o sostenidos (posturas y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nervios craneales, especialmente los extraoculares y elevadores de los párpados, tienen más tendencia a la debilidad muscular persistente que los inervados por otros pares craneales y las extremidades. Las formas clínicas de presentación son generalizadas, oculares y respiratorias. El diagnóstico se sospecha mediante la anamnesia, la fatiga anormal se comprueba mediante el examen físico y la estimulación eléctrica iterativa del nervio que inerva al músculo afectado pero no paralizado. Se corrobora mediante la administración de inhibidores de la acetilcolin esterasa (IACE que al aumentar la cantidad de acetilcolin en la hendidura sináptica, corrigen la fatiga o la debilidad muscular transitoriamente. Se hace el diagnóstico de certeza mediante la demostración sérica de anticuerpos contra los receptores de acetilcolin (ACRA. El tratamiento es a largo plazo sintomático con IACE y etiopatogénico con inmunosupresores, plasmaféresis, gamma globulina endovenosa y timectomía. El curso es crónico. La remisión espontánea o después de tratamiento sintomático o etiopatogénico ocurre entre 1-10 años respectivamente. La mortalidad es prácticamente nula aun durantes las crisis miastenias gracias a la educación de padres, pacientes y público en general sobre el tema, al desarrollo del sistema de respuesta rápida de auxilio domiciliario y las unidades de cuidados intensivos y el empleo de la ventilación asistida profiláctica, plasmaféresis y

  19. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

    Science.gov (United States)

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-09-01

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.

  20. [A case of clinical overlap syndrome of rheumatoid arthritis and amyopathic dermatomyositis with multiple pulmonary injuries].

    Science.gov (United States)

    Liu, Shuang; An, Yuan; Jia, Yuan; Li, Zhan-guo

    2014-10-18

    Autoimmune diseases can cause various kinds of lung injuries. Clinical features of a case of overlap syndrome with multiple pulmonary injuries were investigated, and the treatment experiences discussed. The patient suffered from rheumatoid arthritis (RA) at first, and then had a lobectomy surgery due to the rheumatoid nodules in her right lung. A year later her disease was diagnosed as amyopathic dermatomyositis (ADM) with typical Gottron's sign, craftsmen hands and rapid-progressive organizing pneumonia (OP). After a combined treatment with glucocorticoids (GCs) and cyclophosphamide (CTX), her OP became better but lung infections progressed. Her lung infections eventually were cured after we used antibiotics and antifungal treatment while we ceased to use CTX and reduced the dosage of GCs. The clinical feature of the patient was overlap syndrome with a variety of lung injuries, such as pulmonary rheumatoid nodules, OP, secondary bronchopleural fistula and lung infections. Its diagnosis and treatment experiences could improve our understanding of pulmonary manifestations of connective tissue disease and improve our diagnosis and treatment level.

  1. Oropharyngeal dysphagia as dominant and life-threatening symptom in dermatomyositis

    Directory of Open Access Journals (Sweden)

    Đaković Zorana

    2009-01-01

    Full Text Available Background. Dysphagia can be a serious problem in patients with inflammatory myopathies. It may be associated with nutritional deficit, aspiration pneumonia, and poor prognosis. Case report. We presented a 60-year-old male, suffering from difficulty in swallowing, pain and weaknes in the proximal parts of his extremities, and skin manifestation. Laboratory findings showed increased creatine kinase and aldolase. Antinuclear antibodies to HEP-2 subtrate revealed titer of 1:40. Electromyoneurography demonstrated evidence of a proximal myopathy. A muscle biopsy revealed myositis. The baruim swallow test was remarkable for regurgitation, and nasal emerging of barium. Nuclear magnetic resonance images of cranium was normal. Tumor markers CEA, and Ca 19-9 were increased. A dose of 1 mg daily prednisolone was administered and percutaneous enteral feeding was performed. Two months later, the patient developed febrile state, aspiration pneumonia, and died due to respiratory failure. Conclusion. In cases of dermatomyositis with the serious dysphagia, percutaneous endoscopic gastrostomy should be performed as soon as possible. Owerall survival rate is low, even with an adequate therapy administration. Inflammatory myopathies should be considered in any patient with oropharyngeal dysphagia.

  2. Ultraviolet Radiation Exposure is Associated with Clinical and Autoantibody Phenotypes in Juvenile Myositis

    Science.gov (United States)

    Shah, Mona; Targoff, Ira N.; Rice, Madeline M.; Miller, Frederick W.; Rider, Lisa G.

    2013-01-01

    Objective Genetic and environmental factors may contribute to the etiology of the juvenile idiopathic inflammatory myopathies (JIIM), systemic autoimmune diseases characterized by muscle and skin inflammation. We investigated the association between ultraviolet radiation (UVR) exposure and the clinical and autoantibody expression of JIIM. Methods We assessed the relationship between UVR exposure in the month before symptom onset and prevalence of juvenile dermatomyositis (JDM) versus polymyositis (JPM) in 298 patients. Among JDM patients, the association between UVR exposure and myositis autoantibodies was assessed. Regression models were stratified by sex and race. The association between regional UV index in U.S. geoclimatic zones and the clinical and autoantibody subgroups was examined by weighted least squares regression analysis. Results We observed increasing odds of JDM compared with JPM per unit increase in the patients’ highest UV index in the month before symptom onset in girls (OR = 1.18; 95% CI = 1.00–1.40). The average and highest UV indices were associated with increasing odds of anti-p155/140 autoantibodies, which was strongest in white males (OR 1.30 and 1.23, respectively). No association was observed between the UV index and anti-MJ autoantibodies or patients without myositis autoantibodies. Across US geoclimatic regions, the average UV index was associated with increasing odds of JDM and anti-p155/140 autoantibodies but decreasing odds of anti-MJ autoantibodies. Conclusion Short-term UVR exposure prior to illness onset may have a role in the clinical and serologic expression of juvenile myositis. Research examining mechanisms of UVR in JIIM pathogenesis is suggested from these findings. PMID:23658122

  3. Juvenile hyaline fibromatosis.

    Science.gov (United States)

    Larralde, M; Santos-Muñoz, A; Calb, I; Magariños, C

    2001-01-01

    Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.

  4. Juvenile Justice in Rural America.

    Science.gov (United States)

    Jankovic, Joanne, Ed.; And Others

    Producing a much-needed organized body of literature about rural juvenile justice, 14 papers (largely from the 1979 National Symposium on Rural Justice) are organized to identify current issues, identify forces causing changes in current systems, review programs responding to rural juvenile justice problems, and provide planning models to aid…

  5. Juvenile arthritis and uveitis.

    Science.gov (United States)

    Kanski, J J

    1990-01-01

    The association between juvenile arthritis and uveitis is reviewed. Some children with the HLA-B27 related spondyloarthropathies develop anterior uveitis. About 20% of patients with juvenile rheumatoid arthritis (JRA) who are negative for IgM rheumatoid factor develop a frequently bilateral, nongranulomatous chronic anterior uveitis. Risk factors for uveitis in JRA patients are: female gender, pauciarticular onset of arthritis, presence of circulating antinuclear antibodies, and the antigens HLA-DW5 and HLA-DPw2. Uveitis is rare after seven years or more have elapsed from the onset of arthritis. The visual prognosis in patients with uveitis is good in 25% and fair in 50%. The remaining 25% develop visual impairment from complicated cataract and/or secondary inflammatory glaucoma. The potential benefit of cytotoxic agents in the treatment of intractable uveitis is outweighed by the risk of serious side effects. The management of secondary inflammatory glaucoma is unsatisfactory, but the results of treatment of complicated cataracts by lensectomy-vitrectomy are good.

  6. Aggressive juvenile mandibular fibromatosis.

    Science.gov (United States)

    Ivanov, Georgi P; Atanasov, Dimitar T; Anavi, Beniamin L

    2013-01-01

    Aggressive juvenile fibromatosis of the jawbones is a rare tumor presenting as infiltrative mass with unpredictable evolution. We report herein a 17-year-old student with a 6-month history of radiologically proven resorption of a part of the mandible, lingual displacement of tooth 34 and malocclusion. Alveolar ridge resorption and three dark-brown foci in the bone were seen after the tooth was extracted. Histological study showed the tumor tissue to have a bundle-like structure; immunohistochemically it was positive for vimentin, smooth muscle actin, beta-catenin, Ki-67 (5%), and negative for desmin and cytokeratin 34bE12. The golden standard in the diagnostics of desmoid fibromatoses is the nuclear or membrane expression of beta-catenin, which is found in 90% of the cases. Differential diagnosis include mandibular fibroma, well-differentiated fibrosarcoma, fibrosing histiocytoma, and infiltration from adjacent soft-tissue tumor. Aggressive juvenile fibromatosis should be managed by radical excision. Local recurrences are not rare, but metastases do not develop. In rare cases this type of fibromatosis has been known to regress spontaneously. Aggressive fibromatosis is a diagnostic challenge, since it remains in the grey zone between benign and malignant lesions of the oral cavity.

  7. Adult-onset nemaline rods in a patient treated for suspected dermatomyositis: study with two-dimensional electrophoresis

    Energy Technology Data Exchange (ETDEWEB)

    Danon, M.J.; Giometti, C.S.; Manaligod, J.R.; Perurena, O.H.; Skosey, J.L.

    1981-12-01

    A 65-year-old woman with progressive muscle weakness and a diffuse rash of three years' duration was examined. Muscle tissue was studied with histochemical techniques, phase-contrast microscopy, electron microscopy, and two-dimensional electrophoresis. Histochemical studies showed numerous nemaline rods, with a normal ratio of types I and II fibers. Two-dimensional electrophoresis revealed abnormalities in the myosin light chain and tropomyosin protein patterns when compared with normal and diseased muscle biopsy samples, including those from two patients with adult-onset dermatomyositis.

  8. Adiponectin may improve osseointegration of dental implants in T2DM patients.

    Science.gov (United States)

    Bai, Yun; Yin, Guozhu; Luo, En

    2011-08-01

    Type 2 diabetes mellitus (T2DM) is the most common form of diabetes. Compared with the general population, a higher failure rate is seen in T2DM patients. There is also evidence that chronically high levels of plasma glycemia leads to inflammatory effect and a negative influence on bone formation and remodeling, and reduce osseointegration of implants. Recently studies reveal that adiponectin is an insulin-sensitizing adipokine, and closely associated with T2DM. Adiponectin has potent anti-inflammatory properties and has been shown to increase bone density by inhibiting osteoclast formation and promoting the formation of osteoblasts. We therefore hypothesize systemically infused or locally used adiponectin could accelerate osseointegration of dental implants in T2DM. Our hypothesis could help to create an option to improve success ratio of dental implants in T2DM by the replenishment of adiponectin in T2DM patients.

  9. Juvenile justice mental health services.

    Science.gov (United States)

    Thomas, Christopher R; Penn, Joseph V

    2002-10-01

    As the second century of partnership begins, child psychiatry and juvenile justice face continuing challenges in meeting the mental health needs of delinquents. The modern juvenile justice system is marked by a significantly higher volume of cases, with increasingly complicated multiproblem youths and families with comorbid medical, psychiatric, substance abuse disorders, multiple family and psychosocial adversities, and shrinking community resources and alternatives to confinement. The family court is faced with shrinking financial resources to support court-ordered placement and treatment programs in efforts to treat and rehabilitate youths. The recognition of high rates of mental disorders for incarcerated youth has prompted several recommendations for improvement and calls for reform [56,57]. In their 2000 annual report, the Coalition for Juvenile Justice advocated increased access to mental health services that provide a continuum of care tailored to the specific problems of incarcerated youth [58]. The specific recommendations of the report for mental health providers include the need for wraparound services, improved planning and coordination between agencies, and further research. The Department of Justice, Office of Juvenile Justice and Delinquency Prevention has set three priorities in dealing with the mental health needs of delinquents: further research on the prevalence of mental illness among juvenile offenders, development of mental health screening assessment protocols, and improved mental health services [59]. Other programs have called for earlier detection and diversion of troubled youth from juvenile justice to mental health systems [31,56]. Most recently, many juvenile and family courts have developed innovative programs to address specific problems such as truancy or substance use and diversionary or alternative sentencing programs to deal with first-time or nonviolent delinquents. All youths who come in contact with the juvenile justice system

  10. IceCube Events from Heavy DM decays through the Right-handed Neutrino Portal

    CERN Document Server

    Ko, P

    2015-01-01

    The recently observed IceCube PeV events could be due to heavy dark matter (DM) decay. In this paper, we propose a simple DM model with extra $U(1)_X$ gauge symmetry and bridge it with standard model particles through heavy right-handed neutrino. The Dirac fermion DM $\\chi$ with mass ~5 PeV can dominantly decay into a dark Higgs ($\\phi$), the SM Higgs ($h$) and a neutrino ($\

  11. Performance Study of CM/DM Discrimination Network for Conducted EMI Diagnosis

    Institute of Scientific and Technical Information of China (English)

    ZHAOYang; SEEKyeYak

    2003-01-01

    This paper compares the discrimination capability for four different types of Common-mode and Differential-mode Discrimination networks (CM/DM DN). The insertion losses and mode rejection performances for the four CM/DM DNs are measured. Based on the measured results, the CM/DM DN with the best discrimination capability is chosen for the purpose of conducted EMI diagnosis. A practical example is shown to demonstrate the ease of EMI diagnosis, with the aid of the chosen CM/DM DN.

  12. Organizacijska kultura v podjetju dm drogerie markt d.o.o.

    OpenAIRE

    Renko, Jure

    2016-01-01

    Organizacijska kultura pomembno vpliva na uspešnost posameznika v organizaciji in uspeh podjetja pri strankah. V diplomskem delu smo preučili organizacijsko kulturo v podjetju dm drogerie markt d.o.o., ki je del koncerna dm. Management v koncernu dm se zaveda pomembnosti tega kompleksnega in dinamičnega pojava. V dm Slovenija smo naredili raziskavo s pomočjo anketnega vprašalnika, kjer smo izmerili trenutno in želeno organizacijsko kulturo z vidika zaposlenih in s stališča lastnikov. Na podla...

  13. Toward electroweak scale cold dark matter with local dark gauge symmetry and beyond the DM EFT

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Pyungwon, E-mail: pko@kias.re.kr [School of Physics, Korea Institute for Advanced Study 85 Hoegiro, Dongdaemun-gu, Seoul 02455 (Korea, Republic of)

    2016-06-21

    In this talk, I describe a class of electroweak (EW) scale dark matter (DM) models where its stability or longevity are the results of underlying dark gauge symmetries: stable due to unbroken local dark gauge symmetry or topology, or long-lived due to the accidental global symmetry of dark gauge theories. Compared with the usual phenomenological dark matter models (including DM EFT or simplified DM models), DM models with local dark gauge symmetries include dark gauge bosons, dark Higgs bosons and sometimes excited dark matter. And dynamics among these fields are completely fixed by local gauge principle. The idea of singlet portals including the Higgs portal can thermalize these hidden sector dark matter very efficiently, so that these DM could be easily thermal DM. I also discuss the limitation of the usual DM effective field theory or simplified DM models without the full SM gauge symmetry, and emphasize the importance of the full SM gauge symmetry and renormalizability especially for collider searches for DM.

  14. Miranda Rights: Implications for Juveniles with Disabilities

    Science.gov (United States)

    Katsiyannis, Antonis; Barrett, David E.; Losinski, Mickey L.

    2011-01-01

    Juvenile delinquency in the United States has been a persistent concern for decades. Consequently, because more juveniles have been referred to juvenile court and the arrest rate of preteen offenders has increased to almost three times that of older youth, the persistent and often controversial issue of the capacity of juvenile offenders to waive…

  15. Juvenile prison in parallel legislation

    Directory of Open Access Journals (Sweden)

    Lutovac Mitar

    2016-01-01

    Full Text Available The need for punishment of juveniles occurred from the time when there was no clear line separating them from the adult criminal population. At the same time, the evolution of the juvenile punishment is not in itself involve substantial changes to their criminal status. On the contrary, the status of minors in society did not show serious differences regarding the status of young adults, as well as the adult elderly. On the other hand, on the ground of their punishment is recorded deviations that go in the direction of application of mild corporal punishment. Closing the minor was performed in a physically separate parts of the general penal institutions with the use of a lower degree of restrictions while serving juvenile prison. Due to the different treatment of minors during the evolution of their criminal status leads to their different treatment in comparative law. That is why we are witnessing the existence of numerous differences in the juvenile punishment in some countries in the world. On the European continent there is a wide range of different legal solutions when it comes to punishing juveniles. There are considerable differences in the procedure pronouncing juvenile prison and in particular penal treatment of juveniles in penitentiary institutions. For these reasons, the author has decided to show the basic statutory provisions in the part that relates to the issue of punishment of minors in the legislation of individual countries.

  16. Update on juvenile myasthenia gravis.

    Science.gov (United States)

    Liew, Wendy K M; Kang, Peter B

    2013-12-01

    Juvenile myasthenia gravis is a relatively rare autoimmune neuromuscular disorder. The pathophysiology of juvenile myasthenia gravis is similar to that of adult myasthenia gravis, though there remain important differences regarding presentation and therapeutic options. We review the pathophysiology, clinical presentation, and treatment options for juvenile myasthenia gravis. Randomized clinical studies of myasthenia gravis have been carried out primarily in adult populations. As juvenile myasthenia gravis is rare, it has been difficult to collect prospective randomized controlled data to evaluate treatment outcomes and efficacy. A recent retrospective series suggests that, as in adult myasthenia gravis, thymectomy is a viable therapeutic option for selected cases of generalized juvenile myasthenia gravis. This is corroborated by the clinical experience of the authors in a referral center with a cohort of patients affected by juvenile myasthenia gravis over a number of years. Recent studies illustrate that some, but not all, adult research on myasthenia gravis is applicable to children and adolescents with juvenile myasthenia gravis. Adult research can inform pediatric studies, but should not be regarded as a substitute for dedicated research in those populations.

  17. Diagnosis and treatment of clinically amyopathic dermatomyositis (CADM): a case series and literature review.

    Science.gov (United States)

    Gil, Bornstein; Merav, Lidar; Pnina, Langevitz; Chagai, Grossman

    2016-08-01

    The objective of this study was to report the clinical course of a cohort of patients with clinically amyopathic dermatomyositis (CADM) in correlation to the presence or absence of anti-melanoma differentiation-associated gene 5 (MDA-5) antibody. Five patients with CADM presented to our rheumatology unit between September 1, 2011 and March 31, 2014. We hereby present their clinical course, laboratory findings, imaging modalities, functional tests, and treatments regimens. Our cohort included five patients, with a mean age of 41.8 ± 17.7. Three patients, all anti-MDA-5 antibody positive, developed rapidly progressive interstitial lung disease (ILD) within 4.3 ± 4.5 months of presentation. Two of these patients succumbed to their disease within 30 months of diagnosis despite intensive immunosuppressive therapy. The third anti-MDA-5-positive patient with ILD is still stable, 20 months from disease onset, on massive combination therapy. One patient developed CADM associated with the anti-p155/140 antibody, a year after completing chemotherapy for non-seminomatous germ cell tumor. He presented with a benign clinical course with no evidence of ILD and no recurrence of malignancy after 20 months of follow-up. The fifth patient in our cohort, who is anti-MDA-5 negative and has no evidence of malignancy, also enjoys a benign clinical course. The presence of anti-MDA-5 antibodies in CADM patients is associated with rapidly progressive ILD and a poor prognosis. The serologic profile of patients with CADM should be routinely evaluated and integrated with clinical data in the management of these patients.

  18. Juvenile chronic arthritis.

    Science.gov (United States)

    Southwood, T R; Woo, P

    1995-05-01

    The nomenclature and classification criteria for arthritis in children should be dealt with initially as separate issues, although they are undoubtedly intertwined. The classification criteria should aim to delineate homogeneous patient populations, yet should be flexible enough to incorporate advances in disease knowledge. It should be recognized that arriving at an international consensus for classification criteria will merely provide a set of operational definitions to facilitate research, and not a set of diagnostic criteria. Indeed the only point to obtaining consensus is to begin a process of systematic ongoing review of the criteria. The labels attached to any of these diseases should facilitate accurate communication. In view of the heterogeneous nature of childhood arthritis, consideration should be given to using a broad umbrella term such as juvenile or childhood arthritis only for communicating with the lay public. Medical nomenclature should be formulated to reflect accurately homogeneous subgroups of arthritis, and should not artificially proscribe a relationship between paediatric and adult disease.

  19. [Juvenile idiopathic arthritis].

    Science.gov (United States)

    Herlin, Troels

    2002-08-19

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis. In addition to the clinical characteristics, genetic and biochemical differences suggest that JIA could be regarded as a general term covering various diseases. Complications described are uveitis, temporomandibular joint affection and growth disturbances. The therapeutic strategy should be planned individually according to age, subtype and disease activity and carried out as teamwork with several specialties. Drugs showing significant effectiveness in controlled studies are primarily methotrexate and sulphasalazine. An immunomodulating agent, etanercept, a soluble TNF alpha-receptor fusion protein, has shown a promising effect in severe polyarticular JIA refractory to methotrexate treatment.

  20. Juvenile hyaline fibromatosis

    Directory of Open Access Journals (Sweden)

    Jayashree Krishnamurthy

    2011-01-01

    Full Text Available Juvenile hyaline fibromatosis is a rare, autosomal-recessive disease characterized by papular and nodular skin lesions, gingival hyperplasia, joint contractures and bone involvement in variable degrees. It is a connective tissue disorder with aberrant synthesis of glycosaminoglycans by fibroblasts. We report a 5-year-old female born of first-degree consanguineous marriage who presented with multiple, recurrent, painless, variable-sized nodules. Fine needle aspiration cytology smears and the subsequent histopathological examination from the nodules showed benign spindle cells in a Periodic acid Schiff-positive myxoid background. The disease has a relentlessly progressive course, with most patients surviving only up to the 4 th decade. As of now, there is no specific treatment for this disorder. Genetic counseling is essential to explain to parents about a 25% chance of having a diseased baby in any pregnancy. With the gene being mapped recently, techniques for antenatal diagnosis are likely to be established.

  1. Juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Krupa H Bhatt

    2014-01-01

    Full Text Available Juvenile Idiopathic Arthritis (JIA is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential.

  2. Juvenile homosexual homicide.

    Science.gov (United States)

    Myers, Wade C; Chan, Heng Choon Oliver

    2012-01-01

    Limited information exists on juvenile homosexual homicide (JHH), that is, youths who perpetrate sexual homicides against same-sex victims. Only a handful of cases from the United States and internationally have been described in the literature. This study, the first of its kind, examines the epidemiology, victimology, victim-offender relationship, and weapon-use patterns in JHH offenders using a large U.S. database on homicide spanning three decades. The data for this study were derived from the Federal Bureau of Investigation's Supplementary Homicide Reports (SHRs) for the years 1976 through 2005. A total of 93 cases of JHH were identified. On average, three of these crimes occurred annually in the U.S., and there was a marked decline in its incidence over the study period. Ninety-five percent were male offender-male victim cases and 5% were female offender-female victim cases. JHH offenders were over-represented amongst all juvenile sexual murderers, similar to their adult counterparts. The majority of these boys were aged 16 or 17 and killed adult victims. They were significantly more likely to kill adult victims than other age groups, to be friends or acquaintances of the victims, and to use contact/edged weapons or firearms. Most offenders killed same-race victims, although Black offenders were significantly more likely than White offenders to kill interracially. A case report is provided to illustrate JHH. Further research is needed to promote our understanding of the pathogenesis, etiology, and associated risk factors for this aberrant form of murder by children.

  3. Successful polymyxin B hemoperfusion treatment associated with serial reduction of serum anti-CADM-140/MDA5 antibody levels in rapidly progressive interstitial lung disease with amyopathic dermatomyositis.

    Science.gov (United States)

    Teruya, Aoi; Kawamura, Kodai; Ichikado, Kazuya; Sato, Shinji; Yasuda, Yuko; Yoshioka, Masakazu

    2013-12-01

    Clinically amyopathic dermatomyositis (CADM), a subtype of dermatomyositis with subtle or no muscle involvement, is occasionally accompanied by fatal, rapidly progressive interstitial lung disease (RP-ILD) that is resistant to aggressive immunosuppressive therapy. The presence of anti-CADM-140/MDA5 antibodies is diagnostic for patients with dermatomyositis (particularly CADM) and is known to be strongly associated with the pathogenesis, disease activity, and mortality of RP-ILD. Polymyxin-B direct hemoperfusion (PMX-DHP), originally developed for the removal of endotoxin, has been demonstrated to be effective for treating various types of acute respiratory failure. We describe a patient with amyopathic dermatomyositis who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 autoantibodies, was resistant to combined steroid and immunosuppressant therapy, and was treated successfully with PMX-DHP. To our knowledge, this is the first case to indicate a serial reduction of anti-CADM-140/MDA5 autoantibodies, associated with clinical improvement, following PMX-DHP. Early intervention using PMX-DHP may improve the prognosis of RP-ILD accompanied by CADM.

  4. Clinical analysis of 6 cases with amyotrophic dermatomyositis%无肌病性皮肌炎6例临床分析

    Institute of Scientific and Technical Information of China (English)

    宋星慧

    2013-01-01

      目的探讨无肌病性皮肌炎(amyotrophic dermatomyositis,ADM)的临床特点和预后.方法对6例ADM患者的临床资料进行回顾性分析.结果6例ADM患者均出现皮肌炎特征性皮肤损害,2例患者合并肺间质病变,其中1例出现急性进展性间质性肺炎死于呼吸衰竭.1例在随访过程中发现肺癌.结论 ADM常合并肺间质病变和肿瘤,临床医师需提高对ADM的认识,一旦发现应积极治疗.%Objective To investigate the clinical feature and prognosis of amyopathic dermatomyositis(ADM). Methods Six case of amyotrophic dermatomyositis were studied retrospectively. Results Six amyopathic dermatomyositis had typical cutaneous vasculitis. Interstitial lung disease(ILD)occurred in two patients,and one of them died of respiratory failure resulting from rapidly progressive ILD. One case cured malignancy. Conclusions ILD and malignancy often cured in ADM. We should enhace the understanding of ADM.

  5. Recurrent dermatomyositis manifesting as a sign of recurrent transitional cell carcinoma of urinary bladder: Long-term survival

    Directory of Open Access Journals (Sweden)

    John Fitzpatrick

    2014-01-01

    Full Text Available The association between urological malignancies and paraneoplastic syndromes has been well documented. We report a case of recurrent dermatomyositis manifesting as a sign of metastatic recurrence of non-muscle-invasive transitional cell carcinoma of the bladder, a relationship which has only been referred to in a few reports. The case highlights a few important clinical challenges; firstly, the importance of thorough investigation for underlying malignancy in patients with dermatomyositis, as successful treatment of such malignancy can lead to resolution of paraneoplastic symptoms, and secondly, a high index of suspicion of recurrence in cases where paraneoplastic manifestations recur. Metastatic pulmonary recurrence without local evidence of disease at a follow-up of 4 years makes this case unique. Moreover, in the light of our experience and reported literature, a framework is suggested to approach such a diagnostic dilemma in the future. Description of the case will guide clinicians in the future, in case they encounter such an unusual clinical scenario. This could also serve as a hypothesis-generating source for designing future research as well.

  6. Do juvenile Amphiprion ocellaris (Pisces

    DEFF Research Database (Denmark)

    Brolund, Thea Marie; Nielsen, Lis Engdahl; Arvedlund, Michael

    2003-01-01

    . This is contrary to the settling mechanisms of the damselfish D. aruanus and D. reticulatus, and of the temperate herring Clupea harengus. Hence the results emphasize the variation of sensory abilities and behaviours in fish larvae and juveniles. It is not an area prone for generalizations.......Juvenile anemonefish Amphiprion ocellaris were tested in two behavioural laboratory set-ups for their ability to visually or chemically recognize conspecifics. Individuals of two other species of anemonefish, A. clarkii and Dascyllus aruanus, were also used as test specimens for recognition....... The results indicate that juvenile A. ocellaris recognize conspecifics visually rather than by olfaction. This is contrary to their finding mechanism of their host anemone. However, the results also indicate that the juvenile A ocellaris are neither attracted nor deterred by the presence of conspecifics...

  7. Bilateral, independent juvenile nasopharyngeal angiofibroma

    DEFF Research Database (Denmark)

    Mørkenborg, Marie-Louise; Frendø, M; Stavngaard, T;

    2015-01-01

    BACKGROUND: Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating...... from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning...... embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. CONCLUSION: This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral...

  8. Editor's Shelf: International Juvenile Titles.

    Science.gov (United States)

    Mitchell-Powell, Brenda

    1994-01-01

    Provides an annotated list of international juvenile picture books and notes those that emphasize text over pictures. The 49 titles present international perspectives for educators, librarians, and parents seeking materials with alternative cultural content. The majority are folk tales. (SLD)

  9. Myotonic dystrophy (DM1) and dysphagia: the need for dysphagia management guidelines and an assessment tool

    NARCIS (Netherlands)

    LaDonna, K.A.; Koopman, W.J.H.; Venance, S.L.

    2011-01-01

    Myotonic dystrophy (DM1) is the most prevalent muscular dystrophy occurring in adulthood. DM1 is a multi-systemic disorder resulting in early-onset cataracts, cardiac rhythm problems, muscle weakness, ptosis, and cognitive and psychiatric manifestations. Dysphagia is one of the most problematic

  10. Comparative experimental and theoretical investigations of the DM neutron moisture probe

    DEFF Research Database (Denmark)

    Ølgaard, Povl Lebeck; Haahr, Vagner

    1967-01-01

    Theoretical and experimental investigations of the Danish produced DM subsurface moisture probe have been carried out at the Research Establishment Risö, and the results obtained are presented in this paper. The DM probe contains an Am-Be fast neutron source and has a glass scintillator containi...

  11. Registration of an oilseed sunflower germplasm HA-DM1 resistant to sunflower downy mildew

    Science.gov (United States)

    HA-DM1 (Reg. No.xxx, PI 674793) sunflower (Helianthus annuus L.) germplasm was developed and released cooperatively by the USDA-ARS, Sunflower and Plant Biology Research Unit and the North Dakota Agricultural Experiment Station in 2015. HA-DM1 is a BC2F4 derived oilseed maintainer line from the cros...

  12. Interference effects of two scalar boson propagators on the LHC search for the singlet fermion DM

    CERN Document Server

    Ko, P

    2016-01-01

    A gauge invariant UV-completion for singlet fermion DM interacting with the standard model (SM) particles involves a new singlet scalar. Therefore the model contains two scalar mediators, mixtures of the SM Higgs boson and a singlet scalar boson. Collider phenomenology of the interference effect between these two scalar propagators is studied in this work. This interference effect can be either constructive or destructive in the DM production cross section depending on both singlet scalar and DM masses, and it will soften the final state jets in the full mass region. Applying the CMS mono-jet search to our model, we find the interference effect plays a very important role in the DM search sensitivity, and the DM production cross section of our model is more than one order of magnitude below the LHC sensitivity at current stage.

  13. Interference effects of two scalar boson propagators on the LHC search for the singlet fermion DM

    Science.gov (United States)

    Ko, P.; Li, Jinmian

    2017-02-01

    A gauge invariant UV-completion for singlet fermion DM interacting with the standard model (SM) particles involves a new singlet scalar. Therefore the model contains two scalar mediators, mixtures of the SM Higgs boson and a singlet scalar boson. Collider phenomenology of the interference effect between these two scalar propagators is studied in this work. This interference effect can be either constructive or destructive in the DM production cross section depending on both singlet scalar and DM masses, and it will soften the final state jets in the full mass region. Applying the CMS mono-jet search to our model, we find the interference effect plays a very important role in the DM search sensitivity, and the DM production cross section of our model is more than one order of magnitude below the LHC sensitivity at current stage.

  14. 酸性皂洗剂DM-1530应用实践%The application practice of the acid soaping agent DM-1530

    Institute of Scientific and Technical Information of China (English)

    季金霞; 胡灿辉; 吴少新

    2009-01-01

    酸性皂洗剂DM-1530将酸中和与皂洗合二为一,可以替代醋酸起到中和作用,而且皂洗效果极佳.以大红色纯棉双面针织布为例,分析了不同pH值、DM-1530用量、水的硬度对DM-1530皂洗效果的影响,并通过生产实践,介绍了传统工艺和DM-1530工艺的流程,比较分析了二者的pH值变化情况、皂洗牢度以及生产成本.表明,使用DM-1530可省去皂洗前的酸中和工艺,节约能源,减少污水排放,缩短工艺流程,提高工作效率,皂洗效果良好.

  15. Sjögren's Syndrome

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  16. Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  17. HCV and Rheumatic Disease

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  18. Find a Rheumatologist

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  19. Carpal Tunnel Syndrome

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  20. Spinal Stenosis

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  1. Osteonecrosis of the Jaw (ONJ)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  2. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  3. Antinuclear Antibodies (ANA)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  4. Pregnancy and Rheumatic Disease

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  5. Neck Pain

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  6. Glucocorticoid-Induced Osteoporosis

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  7. Immune System and Its Link to Rheumatic Diseases

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  8. NSAIDs (Nonsteroidal Anti-inflammatory Drugs)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  9. American College of Rheumatology

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  10. Giant Cell Arteritis

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  11. Metabolic Myopathies

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  12. Calcium Pyrophosphate Deposition (CPPD)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  13. Joint Injection/Aspiration

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  14. 皮肌炎与肺间质病变%Dermatomyositis and lung mesenchyma affection

    Institute of Scientific and Technical Information of China (English)

    陈欢; 谢红付

    2004-01-01

    肺间质病变(interstitial lung disease,ILD)是皮肌炎(dermatomyositis,DM)的严重合并症之一,可危及生命。为提高DM合并ILD的早期诊断率,现将我院1998-2001年住院病例的临床资料分析如下。

  15. Peritendinous calcinosis of calcaneus tendon associated with dermatomyositis: correlation between conventional radiograph, ultrasound, magnetic resonance imaging and gross surgical pathology; Calcinose peritendinea do tendao calcaneo associada a dermatomiosite: correlacao entre radiografia convencional, ultra-sonografia, ressonancia magnetica e macroscopia cirurgica

    Energy Technology Data Exchange (ETDEWEB)

    Rosa, Ana Claudia Ferreira; Gomide, Lidyane Marques de Paula; Lemes, Marcella Stival [Universidade Federal de Goias (UFG), Goiana, GO (Brazil). Faculdade de Medicina. Hospital das Clinicas; Costa, Edegmar Nunes; Rocha, Valney Luiz da [Universidade Federal de Goias (UFG), Goiania, GO (Brazil). Faculdade de Medicina. Dept. de Ortopedia; Machado, Marcio Martins; Santos Junior, Rubens Carneiro dos; Barros, Nestor de; Cerri, Giovanni Guido [Universidade Federal de Goias (UFG), Goiania, GO (Brazil). Faculdade de Medicina. Dept. de Radiologia; Sernik, Renato Antonio [Sao Paulo Univ., SP (Brazil). Hospital das Clinicas. Inst. de Radiologia; Nunes, Rodrigo Alvarenga [Universidade do Vale do Sapucai (UNIVAS), Pouso Alegre, MG (Brazil). Faculdade de Ciencias Medicas; Albieri, Alexandre Daher [Hospital de Acidentados de Goiania, GO (Brazil)

    2006-01-15

    Interstitial calcinosis is an uncommon condition in which there is either localized or widely disseminated deposition of calcium in the skin, subcutaneous tissues, muscles, and tendons. Calcinosis is often associated with collagen diseases, scleroderma and dermatomyositis. The authors report a case of interstitial calcinosis associated with dermatomyositis studied with conventional radiograph, ultrasound and magnetic resonance imaging, and correlate the imaging findings with the results of surgical pathology gross examination. (author)

  16. JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    I N Sartika

    2012-11-01

    Full Text Available Juvenile rheumatoid arthritis (JRA is the most common rheumatic condition in children. JRA is defined as persistent arthritis in 1 or more joints for at least 6 weeks, with the onset before age 16 years. The etiology of JRA is unknown. Antigen activated CD4+ T cell stimulate monocytes, macrophages, and synovial fibroblasts to produce the cytokines Interleukin-1 (IL-1, IL-6, and tumor necrosis factor ? (TNF-? and to secrete matrix metalloproteinases, which lead to chronic inflammation due to infiltration of inflammatory cell, angiogenesis, destruction of cartilage and bone with pannus formation. The 3 major subtypes of JRA are based on the symptoms at disease onset and are designated systemic onset, pauciarticular onset, and polyarticular onset. For all patients, the goals of therapy are to decrease chronic joint pain and suppress the inflammatory process. Poor prognostic have been observed in patients with polyarticular onset, rheumatoid factor, persistent morning stiffness, tenosynovitis, involvement of the small joints, rapid appearance of erosions, active late onset childhood, subcutaneous nodules, or antinuclear antibody.

  17. The use of magnetic resonance imaging in the diagnosis and evaluation of dermatomyositis: report of two cases and review of the literature; Papel da ressonancia magnetica no diagnostico e na avaliacao da dermatomiosite: relato de dois casos e revisao da literatura

    Energy Technology Data Exchange (ETDEWEB)

    Vieira, Renata La Rocca; Silva, Jader Jose da; Amaral, Denise Tokechi do; Fernandes, Artur da Rocha Correa [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. de Diagnostico por Imagem]. E-mail: relarocca@uol.com.br

    2005-04-15

    Dermatomyositis is an autoimmune inflammatory disorder that usually affects skeletal muscle causing muscle weakness in the thighs and pelvic girdle. The diagnosis and the determination of disease activity are based on clinical and laboratorial tests. Magnetic resonance imaging has been described in recent studies as a useful method for the diagnosis of dermatomyositis, in guided muscle biopsy as well as in the determination of disease activity. We report two cases of dermatomyositis with MRI findings and present a review of the literature. (author)

  18. A case of adult onset disseminated juvenile xanthogranuloma

    Directory of Open Access Journals (Sweden)

    Havva Hilal Ayvaz

    2016-01-01

    Full Text Available Juvenile xanthogranuloma (JX is a rare, benign, non-Langerhans histiocytic proliferative disease that etiology is unknown. It is usually seen in children and infants. JX in adult is very rare. A 41-year-old female patient was admitted to our clinic with papules on her face, torso and extremities. A few lesions had occured 3 years ago on her face, they disseminated all over her body after having a traffic accident one year ago which for she had operations and she also concurrently was diagnosed asdiabetes mellitus (DM. Based on clinical and histopathological findings, the diagnosis of JX was made. There is no systemic involvement of JX detected. JX seen in adults are very rare and usually associated with hematological malignancy. The present case is a rare adult onset disseminated JX case without malignancy

  19. STUDY OF PREVALENCE OF THYROID DYSFUNCTION IN TYPE 2 DM IN RURAL POPULATION

    Directory of Open Access Journals (Sweden)

    Nanjil Kumaran

    2015-08-01

    Full Text Available Thyroid disease and diabetes are the two common endocrine disorders encountered in the clinical practice. Type 2 DM is a growing problem in our country and we have observed that many patients are associated with thyroid dysfunction later in their life. The aim of the study is to investigate the prevalence of thyroid dysfunction in patients with Type 2 DM in rural population. METHODS AND MATERIALS : A total of 100 patients attending diabetic clinic in Rajah Muthiah Medical College and Hospital were enrolled in the study and were randomized into test and control groups.70 Type 2 DM patients aged more than 18 years of both genders, Type II DM was diagnosed as pati ents who initially respond to OHA were included in the test group. Patient with thyroid dysfunction, Type 1 DM, chronic kidney disease were excluded. 30 Patients age matched control group without any history of diabetes and thyroid disease was taken for the study. RESULTS : In this study abnormal thyroid function was seen in 17 patients of Type 2 DM and remaining had normal thyroid function. The study shows prevalence of thyroid dysfunction is more in males compared with females. 10 out of 29 (34.4% males had thyroid dysfunction compared with that 7 out of 41 (17% females are suffering from thyroid dysfunction. CONCLUSION : There is growing evidence of an association between thyroid dysfunction and diabetes. Our study shows that higher prevalence of abnormal thyroid hormone levels in Type 2 DM.

  20. Investigation of the interaction of deltamethrin (DM) with human serum albumin by multi-spectroscopic method

    Science.gov (United States)

    Wang, Jiaman; Ma, Liang; Zhang, Yuhao; Jiang, Tao

    2017-02-01

    The interaction of Deltamethrin (DM) with human serum albumin (HSA) under the condition of simulating human blood pH environment (pH = 7.4) was investigated by fluorescence, UV-Vis absorbance and circular dichroism (CD) spectroscopy. It was shown that DM was a static quencher of HSA. The binding constants (Ka) are 3.598 × 104 L mol-1 (25 °C); the thermodynamic parameters (ΔH = -3.269 × 104 kJ mol-1, ΔS = -22.81 kJ mol-1 k-1, ΔG = -25889.8 kJ mol-1) obtained with the thermodynamic equation. The hydrogen bond and Vander Waals were the main driving force. The effect of DM on the conformation of HSA was observed by three-dimensional (3D) fluorescence and circular dichroism spectra, indicating that the interaction between DM and HSA was achieved through the binding of DM with the tryptophan and tyrosine residues of HSA. The study on the interaction of DM and Bovine Serum Albumin (BSA) was researched and compared. Difference exists in the interactions of with each of the serum albumins. We will verify and supplement that DM residue in animals and human metabolism, toxicology and other mechanisms are different.

  1. Reduced expression of the normal DMPK allele in a congenital DM patient

    Energy Technology Data Exchange (ETDEWEB)

    Funanage, V.L.; Carango, P.; Moses, R.M.; Marks, H.G. [Alfred I. duPont Institute, Wilmington, DE (United States)

    1994-09-01

    Both adult-onset and congenital myotonic dystrophy (DM) are autosomal dominant disorders caused by triplet repeat expansions within the 3{prime} untranslated region of the DM protein kinase (DMPK) gene. The size of the repeat region shows a positive correlation with disease severity; in general, the triplet repeat expansions in congenital DM patients are larger than those found in adult DM individuals. In an adult DM patient, the expanded allele of 133 repeats reduced both the synthesis and processing of DMPK mRNA, whereas expression from the unexpanded allele remained unaffected. However, in both muscle and skin tissues from a congenital DM individual, DMPK mRNA expression from the unexpanded allele was also reduced. This reduced expression was maintained in fibroblasts cultured from a skin biospy of the patient; however, normal expression of the unexpanded allele occurred in cultured myoblasts established from this patient`s muscle biopsy. To determine if the expanded repeat exerts a trans effect on DMPK gene expression, we have separated the normal and mutant DMPK alleles from the cogenital DM skin fibroblasts by somatic cell hybridization. Hybrid clones containing only the normal DMPK gene still produced reduced levels of DMPK mRNA, indicating that the reduced expression from the normal allele is due to a cis effect. Cultured skin fibroblasts from the congenital DM patient were exposed to 5-azacytidine to determine if demethylation of the DMPK gene could restore proper expression of the normal allele. We are currently analyzing DMPK mRNA levels in these cells and determining if a difference in the methylation patterns of the normal DMPK alleles from adult and congenital DM patients accounts for this effect.

  2. [Study of a DNA sequence from brine shrimp artemia containing a novel DM domain].

    Science.gov (United States)

    Zeng, Hui; Song, Wen Qin; Chen, Rui Yang

    2004-10-01

    Sex-determining mechanisms are highly variable between phyla. However, there is an apparent exception in which structurally and functionally related genes control sex determination in different phyla: the sexual regulators DSX of Drosophila melanogaster and MAB-3 of Caenorhabditis elegans both containing a DNA-binding motif, DM domain. Proteins containing the domain may also play a role in sexual development of vertebrates. For examples, both the human DMRT1 (doublesex and mab-3 related transcription factor 1) gene and mouse Dmrt1 gene are necessary for male development. In this paper, through the degenerated PCR, a DNA fragment ADM was amplified out from genomic DNA of brine shrimp, Artemia sinica from YunCheng Salt Lake, Shanxi, China and Artemia parthenogenetica from GaHai, Qinghai, China, respectively. ADM encodes 47 amino acids and is highly homologous to amino acid sequence of the known DM domains. By comparing total of 27 DM domains in distant related species, a phylogenic tree of DM domain was constructed. In the tree, these DM domains were divided into different branches according to their subtypes. Among the DM domains that were compared, ADM is most homologous to the DM domain contained in human DMRT3 and mouse Dmrt3, which shares 83% identity between them. In addition, the same length of ADM could also be amplified out from cDNA of Artemia sinica and Artemia parthenogenetica, which indicated that ADM was expressed and located in one exon. The DM domain in brine shrimp reported here would make it possible for cloning the full-length cDNA containing the DM domain and further elucidating their functions.

  3. Dm5-HT2B: Pharmacological Characterization of the Fifth Serotonin Receptor Subtype of Drosophila melanogaster

    Directory of Open Access Journals (Sweden)

    Wolfgang Blenau

    2017-05-01

    Full Text Available Serotonin (5-hydroxytryptamine, 5-HT is an important regulator of physiological and behavioral processes in both protostomes (e.g., insects and deuterostomes (e.g., mammals. In insects, serotonin has been found to modulate the heart rate and to control secretory processes, development, circadian rhythms, aggressive behavior, as well as to contribute to learning and memory. Serotonin exerts its activity by binding to and activating specific membrane receptors. The clear majority of these receptors belong to the superfamily of G-protein-coupled receptors. In Drosophila melanogaster, a total of five genes have been identified coding for 5-HT receptors. From this family of proteins, four have been pharmacologically examined in greater detail, so far. While Dm5-HT1A, Dm5-HT1B, and Dm5-HT7 couple to cAMP signaling cascades, the Dm5-HT2A receptor leads to Ca2+ signaling in an inositol-1,4,5-trisphosphate-dependent manner. Based on sequence similarity to homologous genes in other insects, a fifth D. melanogaster gene was uncovered coding for a Dm5-HT2B receptor. Knowledge about this receptor’s pharmacological properties is very limited. This is quite surprising because Dm5-HT2B has been attributed to distinct physiological functions based on genetic interference with its gene expression. Mutations were described reducing the response of the larval heart to 5-HT, and specific knockdown of Dm5-HT2B mRNA in hemocytes resulted in a higher susceptibility of the flies to bacterial infection. To gain deeper understanding of Dm5-HT2B’s pharmacology, we evaluated the receptor’s response to a series of established 5-HT receptor agonists and antagonists in a functional cell-based assay. Metoclopramide and mianserin were identified as two potent antagonists that may allow pharmacological interference with Dm5-HT2B signaling in vitro and in vivo.

  4. Myotonic dystrophy type 1 (DM1): a triplet repeat expansion disorder.

    Science.gov (United States)

    Kumar, Ashok; Agarwal, Sarita; Agarwal, Divya; Phadke, Shubha R

    2013-06-15

    Myotonic dystrophy is a progressive multisystem genetic disorder affecting about 1 in 8000 people worldwide. The unstable repeat expansions of (CTG)n or (CCTG)n in the DMPK and ZNF9 genes cause the two known subtypes of myotonic dystrophy: (i) myotonic dystrophy type 1 (DM1) and (ii) myotonic dystrophy type 2 (DM2) respectively. There is currently no cure but supportive management helps equally to reduce the morbidity and mortality and patients need close follow up to pay attention to their clinical problems. This review will focus on the clinical features, molecular view and genetics, diagnosis and management of DM1.

  5. Qualidade de vida e sintomas depressivos em adolescentes com Diabetes Mellitus tipo 1 (DM1)

    OpenAIRE

    Gabriela de Oliveira Pintar

    2015-01-01

    O objetivo geral deste trabalho foi avaliar a presença de sintomas depressivos e as possíveis influências do Diabetes Mellitus tipo I (DM1) na qualidade de vida de adolescentes com esta doença. Para alcançar tal objetivo foram avaliados adolescentes com DM1 (58) e sem DM1 (61) completando um total de 119 adolescentes avaliados; de ambos os sexos. Os locais de coleta de dados foram o Ambulatório de Endocrinologia da Criança e do Adolescente (ECA) do Hospital das Clínicas da Faculdade de Medici...

  6. Studies on the s_dm.t=f verb form in Classical Egyptian

    OpenAIRE

    Zonhoven, Ludovicus Martinus Johannes

    1997-01-01

    This study is devoted to some synchronic aspects of the sDm.t=f verb form, primarily its meaning and uses in Classical Egyptian. In the introduction some attention is paid to the history of the studies of the form and its origin, an aspect which will receive no further consideration. In accordance with present common opinion the sDm.t=f is here considered to belong to the suffix conjugation. Ch. I is primarily concerned with the active Dr sDm.t=f construction, but begins with a general introd...

  7. Juveniles tried as adults: the age of the juvenile matters.

    Science.gov (United States)

    Semple, Jaclyn K; Woody, William Douglas

    2011-08-01

    Serious juvenile crimes require evaluation of a child as a criminal defendant in adult court. In such cases, it is crucial to understand jurors' attitudes, biases, and ability to follow legal instructions and maintain fairness. 308 undergraduate psychology students served as mock jurors, were randomly separated into four groups, and each group read the same realistic summary of a trial with the defendant's age presented as 13, 15, 17, or 21 years. Participants were asked to render guilty or not guilty verdicts and, if guilty, to suggest sentences. Chi-squared analysis indicated 13- and 15-year-old defendants were convicted less often than 17- and 21-year-old defendants, showing that jurors distinguished between juvenile defendants of different ages, but not minors and adults as defined by law. Additional analysis showed that age did not affect sentencing recommendations. Decision processes jurors use for juveniles tried as adults are discussed.

  8. Forensic aspects of juvenile violence.

    Science.gov (United States)

    Haller, L H

    2000-10-01

    The juvenile justice system was created because it was recognized that youthful offenders needed to be managed differently from adults. They were to receive habilitation services instead of punishment. It is now more than a century since the creation of the first juvenile court. After 67 years, the US Supreme Court, in Kent v United States stated that the model was not working because juveniles in the criminal justice system received no treatment and they had no rights. Because the issue that had been appealed was the lack of rights (not lack of treatment), the Court mandated that juveniles, like adults, be given certain rights. The following year, in In re Gault, the Court expanded these rights. Subsequent Supreme Court cases have dealt with these kinds of issues--that is, whether juvenile offenders are entitled to the same rights as adults and subject to the same penalties. The Supreme Court has never heard a "right to treatment" case, which is the other part of the juvenile court system. Cases have been brought in lower courts (e.g., Nelson v. Heyne, 1972) alleging inadequate treatment services, but no national impact has resulted. Thus, in general, children in the juvenile court system do not have an enforceable right to treatment and can obtain only what services are available in their jurisdictions. The services often are woefully inadequate. Sentencing a youth to probation, with the requirement that he or she participate in counseling or mental health treatment, is meaningless if services are not available. Community-based, model programs that provide effective treatment do exist. They are, as yet, the rare exception rather than the norm and, therefore, are not available to most youthful offenders. Incarcerated juveniles, obviously, cannot avail themselves of community programs. Litigation to give these youth the same rights as adults in penal institutions is not the answer because incarcerated adults don't have a right to treatment, only a right to be free

  9. 基于DM365的便携监控系统解决方案%PortabIe Monitoring System SoIution Based on DM365

    Institute of Scientific and Technical Information of China (English)

    桑赫; 林岩; 张烽

    2015-01-01

    本文提出了一种基于TMS320 DM365芯片的便携监控系统解决方案。设计以 TMS320 DM365为核心硬件设备,在满足基本功能的基础上,尽量减小体积、降低功耗。编写了基于嵌入式 Linux系统的应用程序,完成了音频/视频采集、存储等功能,并设计了有效的电源管理方案,实现了便携监控系统与 Android手机间的数据交互,提升了该系统的易用性。%A portable monitoring system based on TMS320DM365 chip is proposed.The design takes TMS320DM365 as the core of the hardware devices,it minimizes the size and the power consumption as possible while satisfying the basic functions.The collection and storage of audio/video,effective power management scheme and data exchange between portable monitoring device and Android mobile phone are realized through writing application programs based on the embedded Linux system,so the usability of the system is enhanced.

  10. Juvenile Correctional Institutions Library Services: A Bibliography.

    Science.gov (United States)

    McAlister, Annette M.

    This bibliography lists citations for 14 articles, books, and reports concerned with library services in juvenile correctional institutions. A second section lists 21 additional materials on adult correctional libraries which also contain information relevant to the juvenile library. (KP)

  11. Muscles in motion : a randomized controlled trial on the feasibility, safety and efficacy of an exercise training programme in children and adolescents with juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, G. Esther A.; Bos, G. J. F. Joyce; van Royen-Kerkhof, Annet; Lelieveld, Otto T. H. M.; Armbrust, Wineke; Takken, Tim; van Brussel, Marco

    Objective. To study for the first time in a randomized controlled trial the feasibility, safety and efficacy of an exercise training programme in children and adolescents with JDM. Methods. Patients were randomly assigned to the Intervention Group (IG; n = 14) or Waiting Control Group (WCG; n = 12).

  12. Muscles in motion: a randomized controlled trial on the feasibility, safety and efficacy of an exercise training programme in children and adolescents with juvenile dermatomyositis.

    Science.gov (United States)

    Habers, G Esther A; Bos, G J F Joyce; van Royen-Kerkhof, Annet; Lelieveld, Otto T H M; Armbrust, Wineke; Takken, Tim; van Brussel, Marco

    2016-07-01

    To study for the first time in a randomized controlled trial the feasibility, safety and efficacy of an exercise training programme in children and adolescents with JDM. Patients were randomly assigned to the Intervention Group (IG; n = 14) or Waiting Control Group (WCG; n = 12). The intervention comprised an individually tailored 12-week home-based exercise programme of treadmill interval training and strength exercises. The efficacy of the IG over usual care (WCG) was examined with mixed linear regression (intention-to-treat). Effect sustainability during 12 weeks follow-up was also examined. Seventy-five percent of the participants completed the intervention. Reasons for discontinuation were motivation/fatigue, recurrent infections and increasing physical complaints. No hospitalizations occurred and immune suppressive therapy remained stable or decreased in the patients who participated in the intervention. The estimated marginal means after the intervention period were significantly in favour of the IG compared with the WCG for standing long jump distance [difference between groups (95% CI): 13 cm (2-23)], the 30-s number of push-ups [8 (3-13)] and sit-ups [4 (0.4-8)], and the parent Childhood Health Assessment Questionnaire 30 + 8 score [-0.13 (-0.24 to - 0.01)] and effects sustained at follow-up. A trend was seen for the maximal oxygen uptake divided by body mass during maximal exercise treadmill testing; the IG scored 3.0 ml/kg/min (-1.3 to 7.3) higher compared with the WCG. Other outcomes (e.g. isometric muscle strength and perception of fatigue) did not differ between IG and WCG. Exercise training is of value in the clinical management of JDM. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  13. Muscles in motion : a randomized controlled trial on the feasibility, safety and efficacy of an exercise training programme in children and adolescents with juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, G Esther A; Bos, G J F Joyce; van Royen-Kerkhof, Annet; Lelieveld, Otto T H M; Armbrust, Wineke; Takken, Tim; van Brussel, Marco

    OBJECTIVE: To study for the first time in a randomized controlled trial the feasibility, safety and efficacy of an exercise training programme in children and adolescents with JDM. METHODS: Patients were randomly assigned to the Intervention Group (IG; n = 14) or Waiting Control Group (WCG; n = 12).

  14. Muscles in motion : a randomized controlled trial on the feasibility, safety and efficacy of an exercise training programme in children and adolescents with juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, G Esther A; Bos, G J F Joyce; van Royen-Kerkhof, Annet; Lelieveld, Otto T H M; Armbrust, Wineke; Takken, Tim; van Brussel, Marco

    2016-01-01

    OBJECTIVE: To study for the first time in a randomized controlled trial the feasibility, safety and efficacy of an exercise training programme in children and adolescents with JDM. METHODS: Patients were randomly assigned to the Intervention Group (IG; n = 14) or Waiting Control Group (WCG; n = 12).

  15. Clinical Analysis of 64 cases of Dermatomyositis%皮肌炎64例临床分析

    Institute of Scientific and Technical Information of China (English)

    骆志成; 武三卯; 秦元麟

    2003-01-01

    对64例住院治疗的皮肌炎患者的临床资料进行了回顾分析,并对最近的20例患者进行了随访.结果表明男女之比为1:2,平均年龄为45.7岁,其中41岁以上占68.8%;病程10天~4年,平均1.3年.大多数无明确诱因,首发症状以皮炎为多(68.8%),合并恶性肿瘤者占12.5%.活动期血清肌酶升高,以CPK的LDH为著,随症状减轻,血清肌酶水平趋于正常.采用以糖皮质激素或合并免疫抑制剂为主的综合治疗,多数患者的病情能得到有效控制,临床痊愈14例,显效36例,好转9例,无效5例.提示在皮肌炎患者的诊治过程中,应进行全面的体格检查和相关实验室检查,并进行追踪观察,以便制定合理的治疗方案并及时发现内恶性肿瘤.%The clincal materials of 64 hospitalized patients with dermatomyositis were analyzed retrospectively, furthermore, the prognosis of the last 20 patients were evaluated by way of follow- up, The data indicated that sex ratio was 1: 2 for male to female, with a mean age of 45.7 years. The mean course from onset to diagnosis was 1.3 years, ranged from 10 days to 4 years. Most patients (84.4%) were found with no definite etiological factor.Dermatitis as the first presentation of dermatomyositis was seen in 68.8%of patients. Internal malignancies were found in 12.5% (8/64)of patients. Of serum levels of muscle enzymes, elevations of CPK and LDH were predominant during the active stage of dermatomyositis, and their levels went to mormal range as the disease alleviated. The diseases were well controlled in most patients treated with gtucocorticoid or glucocorticoid in combination with immunosuppressuve agent. Of outcomes, 14 patients recovered from, 36 patients improved prominently,9 patients progressed,and 5 patients tailed in treatment. It is suggested that systematic physical examination and related laboratory tests should be performed during the period of diagnosis and treatment of dermatomyositis so that rational

  16. Myositis in mixed connective tissue disease: a unique syndrome characterized by immunohistopathologic elements of both polymyositis and dermatomyositis Miosite na doença mista do tecido conectivo: achados imunopatológicos de polimiosite e dermatomiosite

    Directory of Open Access Journals (Sweden)

    Maria Angela A.G. Vianna

    2004-12-01

    Full Text Available OBJECTIVE: To characterize the inflammatory cells, the expression pattern of adhesion molecules (ICAM-1 and VCAM-1, membrane attack complex (C5b-9, and major histocompatibility complex (MHC antigens in muscle biopsy of mixed connective tissue disease (MCTD. METHOD: We studied 14 patients with MCTD, and compared to 8 polimyositis (PM patients, 5 dermatomyositis (DM and 4 dystrophies. Inflammatory cells were examined for CD4+, CD8+, memory and naïve T cells, natural killer cells, and macrophages. Expression of MHC-I and -II, ICAM-1, VCAM-1 and C5b -9 were characterized on muscle fibers and vessels. RESULTS: Morphological analysis displayed a pattern of PM. Immunohistochemical study revealed a decreased number of capillaries, predominance of CD4+ and B cells in perivascular regions and predominance of CD8+ and CD45RO+ in endomysial regions. The expression of MHC-I on vessels and on degenerated muscle fibers, MHC-II expression on vessels and perifascicular muscle fibers, and the expression of ICAM-1 / VCAM-1 on endothelial cells indicated both vascular and cellular-immune mediated processes causing the muscular lesion. CONCLUSION:Our findings revealed a mixed mechanism in MCTD, both vascular involvement as DM, and cell-mediated like PM.OBJETIVO: Caracterizar as células do infiltrado inflamatório, o padrão de expressão das moléculas de adesão (ICAM-1 e VCAM-1, complexo de ataque à membrana (C5b-9 e antígenos de histocompatibilidade maior (MHC em biópsias musculares de patientes com doença mista do tecido conectivo (DMTC. MÉTODO: Foram estudados14 pacientes com DMTC e comparadas com 8 pacientes com polimiosite (PM, 5 com dermatomiosite (DM e 4 com distrofias. As células inflamatórias foram caracterizadas como CD4+, CD8+, células T de memória (CD45RO+ e virgens, células "natural killer" e macrófagos. As expressões de MHC-I e -II, ICAM-1, VCAM-1 e C5b-9 foram caracterizadas em fibras musculares e vasos. RESULTADOS:A análise morfol

  17. 儿童皮肌炎伴钙质沉着1例

    Institute of Scientific and Technical Information of China (English)

    徐学刚; 张亚芹; 李纪峰

    2011-01-01

    @@ 皮肌炎(dermatomyositis, DM)是一种亚急性或慢性结缔组织病,以全身广泛性血管炎为特点,主要表现为皮肤损害及横纹肌非化脓性炎症.1有儿童期和40~60岁两个发病高峰,报道的儿童皮肌炎(Juvenile dermatomyositis, JDM)发病率为0.8~4.1/1 000 000.

  18. Juvenile technologies in foreign publications

    Directory of Open Access Journals (Sweden)

    Shpagina E.M.

    2012-09-01

    Full Text Available The article provides the review of foreign publications, concerning the juvenile technologies used in France, Canada, Germany and Switzerland. The paper presents legal, social and psychotherapeutic aspects of juvenile judiciary in foreign countries. The authors paid special attention to the complexity of approaches to young children and teenagers who found themselves in complicated life circumstances or got into trouble with the law. The article gives examples of using the following techniques: cognitive-behavioral intervention, mediation, family therapy (including family background and family history, relations theory, narrative practices, utilization of «emotional intelligence» resources.

  19. Evolution of Juvenile Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Ye.V. Prohorov

    2013-03-01

    Full Text Available Evolution of juvenile ankylosing spondylitis tend to follow a more frequent involvement in the pathological process of elbow and ankle joints, development of enthesiopathies, changes of intraarticular meniscal horns, forming of Baker’s cysts, cartilage flaps and systemic osteoporosis, and total value of all these signs 13 times exceeds thereof in patients with with the debut of disease in adulthood, but for juvenile ankylosing spondylitis vertebral lesion is less common. Age dimorphism of the use of certain groups of drugs and physiotherapy facilities is observed.

  20. On the Prevention of Juvenile Crime

    Science.gov (United States)

    Lelekov, V. A.; Kosheleva, E. V.

    2008-01-01

    Crimes committed by juveniles are among the most urgent social problems. Juvenile crime is as prevalent as crime itself is, and it has not been solved completely in any society and cannot be solved through law enforcement measures alone. In this article, the authors discuss the dynamics and structure of juvenile crime in Russia and present data…

  1. School-Related Characteristics of Male Juveniles.

    Science.gov (United States)

    Sapp, Gary L.; Abbott, Gypsy A.

    School-related characteristics of 256 male juveniles under the jurisdiction of a Family Court system were examined by perusing court records and conducting individual interviews with the juveniles. Results indicated that most juveniles last attended eighth grade, more than 81% had failed at least once, and more than half had fought frequently at…

  2. Intensive Reading Instruction in Juvenile Correctional Settings

    Science.gov (United States)

    Williams, Jacob L.; Wexler, Jade; Roberts, Greg; Carpenter, Clint

    2011-01-01

    Despite 60 years of evidence linking juvenile illiteracy and delinquency, practitioners and policymakers have been painfully slow in the implementation of evidence-based reading interventions for incarcerated juveniles. We will present the Texas Juvenile Justice Tiered Instructional Model, an evidence-based reading program model created…

  3. Sex Differences in Attributions of Juvenile Delinquency.

    Science.gov (United States)

    Sagatun, Inger J.

    This paper is an application of attribution theory to the processing of juvenile delinquents in an attempt to understand the differential treatment of female and male offenders within the juvenile justice system. The paper explores the attributions of juvenile delinquency both by male and female minors, by male and female parents, and by male and…

  4. Do Juveniles Bully More than Young Offenders?

    Science.gov (United States)

    Ireland, Jane L.

    2002-01-01

    Study compares bullying behavior among juvenile and young offenders. Ninety-five male juvenile and 196 male young offenders completed two questionnaires, measuring bullying directly and behaviors indicative of "being bullied" or of "bullying others". Juveniles perceived a higher extent of bullying and reported significantly…

  5. The Juvenile Court: Changes and Challenges.

    Science.gov (United States)

    Feld, Barry C.

    2000-01-01

    Explores the changes in the juvenile court system, in particular, the juvenile waiver and sentencing laws, as it transformed from a social welfare agency into a type of criminal court system for young offenders. Addresses whether states should create an integrated juvenile and criminal justice system. (CMK)

  6. Reforming Our Expectations about Juvenile Justice

    Science.gov (United States)

    Rodriguez, Pamela F.; Baille, Daphne M.

    2010-01-01

    Typing the term "juvenile justice reform" into a Google[TM] search will result in 60 pages of entries. But what is meant by juvenile justice reform? What does it look like? How will one know when it is achieved? This article defines juvenile justice reform, discusses the principles of effective reform, and describes the practice of juvenile…

  7. Mobilizing Communities To Prevent Juvenile Crime. Juvenile Justice Bulletin.

    Science.gov (United States)

    Bownes, Donna; Ingersoll, Sarah

    Through Title V Incentive Grants for Local Delinquency Prevention Programs (Community Prevention Grants), the Office of Juvenile Justice and Delinquency Prevention (OJJDP) allocated $20 million in fiscal year 1997 to states to complement law enforcement and justice system efforts by helping local communities foster strong families and nurture…

  8. Approaching the treatment of dermatomyositis from a new perspective of the theory of collateral disease%从络病学说新视角论治皮肌炎

    Institute of Scientific and Technical Information of China (English)

    王连祥

    2014-01-01

    从中医络病理论探讨皮肌炎的中医病机及治疗,从络病论治皮肌炎是中医药治疗这一难治性疾病取得疗效突破的切入点。根据皮肌炎的发病特点、病位、传变规律、临床症状及疾病的特点等,认为络脉病变贯穿于皮肌炎发生发展。从络病学说和皮肌炎的现代研究出发,揭示了二者间存在密切联系;并分5个证型从络病论治皮肌炎。%To explore the pathogenesis and the treatment of dermatomyositis from the theory of collateral disease. Investigating the treatment of dermatomyositis from the perspective of the theory of collateral disease maybe produce a breakthrough in dealing with this intractable disease. According to the onset characteristics, the transmission, clinical symptoms and characteristics of dermatomyositis, collateral disease is thought to be existed persistently in the occurrence and the development of dermatomyositis. Studies of the theory of collateral disease and modern studies of dermatomyositis reveal that there is a close relationship between collateral disease and dermatomyositis, moreover dermatomyositis can be differentiated into 5 patterns of syndrome from the theory of collateral disease.

  9. Juvenile Justice and Substance Use

    Science.gov (United States)

    Chassin, Laurie

    2008-01-01

    Laurie Chassin focuses on the elevated prevalence of substance use disorders among young offenders in the juvenile justice system and on efforts by the justice system to provide treatment for these disorders. She emphasizes the importance of diagnosing and treating these disorders, which are linked both with continued offending and with a broad…

  10. Genetics in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Albers, Heleen Marion

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is a non-common disease in children that can persist into adulthood. JIA is considered to be an auto-immune disease. Genetic factors play a role in the pathogenesis. In a new cohort of JIA patients from North-West European descent genetic candidate gene associatio

  11. Juvenile Courts. Creation and development

    Directory of Open Access Journals (Sweden)

    Montserrat GONZÁLEZ FERNÁNDEZ

    2013-11-01

    Full Text Available This paper studies the creation of Juvenile or Children's Courts in Spain, analysing their reasons and aims, as well as the ethical and political connotations present on their way of acting. Their history and the one of the institutions that complement them is built from the legislation, writings and ideas of their promoters.

  12. Juvenile Diabetes and Rehabilitation Counseling.

    Science.gov (United States)

    Stone, J. Blair; Gregg, Charles H.

    1981-01-01

    Severe complications of diabetes are more likely to occur with the juvenile diabetic and problems of psychosocial adjustment are recurring and difficult. Implications for the rehabilitation counselor are discussed in terms of employment considerations, the effects of complications, genetic counseling, and cooperation with other professionals.…

  13. Case Report: Juvenile Tophaceous Gout

    Directory of Open Access Journals (Sweden)

    Seyma Gunes

    2016-01-01

    Full Text Available Gout is a metabolic disease that manifests as recurrent arthritis. Its incidance increases with age. Clinical findings include recurrent acute arthritis, tophus at joints and tissues, uricacid stones and gouty nephropathy. Tophi is a late period complication of arthritis. In this casereport we presented  a patient with early-onset juvenile tophaceous gout.

  14. Do juvenile Amphiprion ocellaris (Pisces

    DEFF Research Database (Denmark)

    Brolund, Thea Marie; Nielsen, Lis Engdahl; Arvedlund, Michael

    2003-01-01

    . This is contrary to the settling mechanisms of the damselfish D. aruanus and D. reticulatus, and of the temperate herring Clupea harengus. Hence the results emphasize the variation of sensory abilities and behaviours in fish larvae and juveniles. It is not an area prone for generalizations....

  15. [Sex-linked juvenile retinoschisis].

    Science.gov (United States)

    François, P; Turut, P; Soltysik, C; Hache, J C

    1976-02-01

    About 13 observations of sexe linked juvenile retinoschisis, the authors describe the ophthalmoscopic, fluorographic and functional aspects of the disease whose caracteristics are:--its sexe linked recessive heredity; --its clinical characterestics associating: a microcystic macular degeneration, peripheral retinal lesions, vitreous body alterations, --an electroretinogram of the negative type.

  16. Sense and Antisense DMPK RNA Foci Accumulate in DM1 Tissues during Development.

    Directory of Open Access Journals (Sweden)

    Lise Michel

    Full Text Available Myotonic dystrophy type 1 (DM1 is caused by an unstable expanded CTG repeat located within the DMPK gene 3'UTR. The nature, severity and age at onset of DM1 symptoms are very variable in patients. Different forms of the disease are described, among which the congenital form (CDM is the most severe. Molecular mechanisms of DM1 are well characterized for the adult form and involve accumulation of mutant DMPK RNA forming foci in the nucleus. These RNA foci sequester proteins from the MBNL family and deregulate CELF proteins. These proteins are involved in many cellular mechanisms such as alternative splicing, transcriptional, translational and post-translational regulation miRNA regulation as well as mRNA polyadenylation and localization. All these mechanisms can be impaired in DM1 because of the deregulation of CELF and MBNL functions. The mechanisms involved in CDM are not clearly described. In order to get insight into the mechanisms underlying CDM, we investigated if expanded RNA nuclear foci, one of the molecular hallmarks of DM1, could be detected in human DM1 fetal tissues, as well as in embryonic and neonatal tissues from transgenic mice carrying the human DMPK gene with an expanded CTG repeat. We observed very abundant RNA foci formed by sense DMPK RNA and, to a lesser extent, antisense DMPK RNA foci. Sense DMPK RNA foci clearly co-localized with MBNL1 and MBNL2 proteins. In addition, we studied DMPK sense and antisense expression during development in the transgenic mice. We found that DMPK sense and antisense transcripts are expressed from embryonic and fetal stages in heart, muscle and brain and are regulated during development. These results suggest that mechanisms underlying DM1 and CDM involved common players including toxic expanded RNA forming numerous nuclear foci at early stages during development.

  17. Mitochondrial DNA backgrounds might modulate diabetes complications rather than T2DM as a whole.

    Directory of Open Access Journals (Sweden)

    Alessandro Achilli

    Full Text Available Mitochondrial dysfunction has been implicated in rare and common forms of type 2 diabetes (T2DM. Additionally, rare mitochondrial DNA (mtDNA mutations have been shown to be causal for T2DM pathogenesis. So far, many studies have investigated the possibility that mtDNA variation might affect the risk of T2DM, however, when found, haplogroup association has been rarely replicated, even in related populations, possibly due to an inadequate level of haplogroup resolution. Effects of mtDNA variation on diabetes complications have also been proposed. However, additional studies evaluating the mitochondrial role on both T2DM and related complications are badly needed. To test the hypothesis of a mitochondrial genome effect on diabetes and its complications, we genotyped the mtDNAs of 466 T2DM patients and 438 controls from a regional population of central Italy (Marche. Based on the most updated mtDNA phylogeny, all 904 samples were classified into 57 different mitochondrial sub-haplogroups, thus reaching an unprecedented level of resolution. We then evaluated whether the susceptibility of developing T2DM or its complications differed among the identified haplogroups, considering also the potential effects of phenotypical and clinical variables. MtDNA backgrounds, even when based on a refined haplogroup classification, do not appear to play a role in developing T2DM despite a possible protective effect for the common European haplogroup H1, which harbors the G3010A transition in the MTRNR2 gene. In contrast, our data indicate that different mitochondrial haplogroups are significantly associated with an increased risk of specific diabetes complications: H (the most frequent European haplogroup with retinopathy, H3 with neuropathy, U3 with nephropathy, and V with renal failure.

  18. Clinical significance of combined detection of three myositis-specific autoantibodies in the diagnosis of dermatomyositis%联合检测3种肌炎特异性自身抗体在皮肌炎诊断中的意义

    Institute of Scientific and Technical Information of China (English)

    吴云娟; 张晓军; 张缪佳

    2013-01-01

    Objective To detect the myositis-specific autoantibodies (MSAs) in serum samples from the patients with dermatomyositis (DM),and evaluate their values in the diagnosis of DM.Methods Serum samples were collected from 24 healthy persons,38 patients with DM,14 with polymyositis and 20 with other connective tissue diseases,including 6 cases of Sjogren's syndrome,7 systemic lupus erythematosus,4 systemic sclerosis and 3 rheumatoid arthritis.Then,the anti-CADM (clinically amyopathic dermatomyositis)-140 antibody,anti-P155/140 antibody,and anti-SAE (small ubiquitin-like modifier enzyme) antibody in these serum samples were determined by enzyme-linked immunosorbent assay (ELISA),and the positive rates of these antibodies and their correlation with patients' clinical manifestation were analyzed.Results The anti-CADM-140 antibody was detected from 4 out of 38 (10.5%) patients with DM,and the patients were characterized by fever,Gottron syndrome,interstitial lung disease (ILD) and exercise-induced asthma.The anti-P155/140 antibody was also detected from 10.5% of patients with DM,who were characterized by Gottron syndrome,Heliotrope rash,ILD,dysphagia and malignancies.Three out of 38 (7.9%) DM patients were positive for anti-SAE antibody and were characterized by Gottron sign,myalgia,arthritis,arthralgia and dysphagia.When 3 antibodies were combined for the diagnosis of DM,the sensitivity,specificity,positive predictive rate,negative predictive rate and Youden index were 28.9%,100%,100%,68.2%and 0.289,respectively.Conclusion The combined detection of anti-CADM-140 antibody,anti-P155/140 antibody and anti-SAE antibody may increase the diagnosis specificity of DM,and also help to improve the differential diagnosis of DM.%目的 测定皮肌炎(DM)患者血清中的肌炎特异性抗体(MSAs),探讨MSAs在诊断DM中的价值.方法 采集38例DM、14例多发性肌炎、20例其他结缔组织病患者(6例干燥综合征、7例SLE、4例系统性硬化症、3

  19. Serum Concentrations of Transforming Growth Factor-Beta 1 in Predicting the Occurrence of Diabetic Retinopathy in Juvenile Patients with Type 1 Diabetes Mellitus

    Directory of Open Access Journals (Sweden)

    Katarzyna Zorena

    2013-01-01

    Full Text Available In the present study, we have decided to evaluate if serum transforming growth factor-beta 1 (TGF-β1 concentrations may have diagnostic value in predicting the occurrence of diabetic retinopathy (DR in juvenile patients with type 1 diabetes mellitus (T1DM. The study included 81 children and adolescents with T1DM and 19 control subjects. All study participants had biochemical parameters examined, underwent an eye examination, and 24-hour blood pressure monitoring. Moreover, serum concentrations of TGF-β1 were measured. The group of patients with T1DM and nonproliferative diabetic retinopathy (NPDR had statistically significant higher serum levels of TGF-β1 (P=0.001 as compared to T1DM patients without retinopathy as well as the healthy control subject. The threshold serum TGF-β1 concentrations which had a discriminative ability to predict the presence of DR were calculated using the receiver operating characteristic (ROC curves analysis and amounted to 443 pg/ml. The area under the ROC curve (AUCROC was 0.80, and its population value was in the range of 0.66 to 0.94. The sensitivity and specificity were calculated to be 72% and 88%, respectively. Our results suggest that TGF-β1 serum concentrations may be an additional parameter in predicting the occurrence of DR in juvenile patients with T1DM.

  20. 皮肌炎的诊断与治疗%Diagosis and Treatment of Dermatomyositis

    Institute of Scientific and Technical Information of China (English)

    Jeffrey P Callen

    2007-01-01

    @@ 1 简介 1.1 背景 皮肌炎(DM)是一种以皮损为特征的特发性炎性肌病(IIM).1975年,Bohan和Peter首先提出了皮肌炎和多发性肌炎(PM)的诊断与分型标准.5条标准中有4条与肌肉病变有关,如:渐进的近端对称性肌力减弱、肌酶水平升高、肌电图表现异常及肌肉活组织样本检查异常.

  1. Juvenile Crime, Juvenile Justice. Panel on Juvenile Crime: Prevention, Treatment, and Control.

    Science.gov (United States)

    McCord, Joan, Ed.; Widom, Cathy Spatz, Ed.; Crowell, Nancy A., Ed.

    This book discusses patterns and trends in crimes committed by children and adolescents, analyzing youth crime as a subset of general crime and studying the impact of race and gender. It evaluates different approaches to forecasting future crime rates. Data come from a national panel that examined what is known about juvenile crime and its…

  2. Microstructure evolution and kinetic analysis of DM hot-work die steels during tempering

    Energy Technology Data Exchange (ETDEWEB)

    Zhou Qingchun, E-mail: qingchun@shu.edu.cn [School of Materials Science and Engineering, Shanghai University, Shanghai 200072 (China); Wu Xiaochun; Shi Nannan; Li Junwan; Min Na [School of Materials Science and Engineering, Shanghai University, Shanghai 200072 (China)

    2011-07-15

    Highlights: {yields} Hardness of the DM steels decreases continuously with increasing temperature. {yields} Increasing temperatures results in intra-lath carbides precipitate and coarsen significantly. {yields} The values of n indicate a growth mechanism in coarsening of large spheroids on dislocations. {yields} Mn in DM steels dissolves into carbides and retards the coarsening of carbides at high temperature. - Abstract: Utilizing the hardness measurement, an attempt is made to study the tempering kinetics of DM hot-work die steels, combined with scanning electron microscopy (SEM) and transmission electron microscopy (TEM). The results demonstrate that the increasing of tempering temperature will result in precipitation and coarsening of the intra-lath spherical carbides. Meanwhile, it is significantly observed the dissolution of needle-like Mo2C carbides with increasing of tempering temperatures. The growth mechanism of carbides is coarsening of large spheroids after complete and impingement precipitation on dislocations. In addition, the activation energy {Delta}H of DM steels during additional tempering between 620 deg. C and 700 deg. C is slightly higher than that of the diffusion of usual alloying elements (e.g. Cr, V, Mo and W) in ferrite, which may be attributed to a higher Mn content in DM steels that dissolves into carbides and retardsthe coarsening of carbides at high temperature.

  3. Complementarity of DM searches in a consistent simplified model: the case of Z{sup ′}

    Energy Technology Data Exchange (ETDEWEB)

    Jacques, Thomas [SISSA and INFN,via Bonomea 265, 34136 Trieste (Italy); Katz, Andrey [Theory Division, CERN,CH-1211 Geneva 23 (Switzerland); Département de Physique Théorique and Center for Astroparticle Physics (CAP),Université de Genève, 24 quai Ansermet, CH-1211 Genève 4 (Switzerland); Morgante, Enrico; Racco, Davide [Département de Physique Théorique and Center for Astroparticle Physics (CAP),Université de Genève, 24 quai Ansermet, CH-1211 Genève 4 (Switzerland); Rameez, Mohamed [Département de Physique Nucléaire et Corpusculaire,Université de Genève, 24 quai Ansermet, CH-1211 Genève 4 (Switzerland); Riotto, Antonio [Département de Physique Théorique and Center for Astroparticle Physics (CAP),Université de Genève, 24 quai Ansermet, CH-1211 Genève 4 (Switzerland)

    2016-10-14

    We analyze the constraints from direct and indirect detection on fermionic Majorana Dark Matter (DM). Because the interaction with the Standard Model (SM) particles is spin-dependent, a priori the constraints that one gets from neutrino telescopes, the LHC, direct and indirect detection experiments are comparable. We study the complementarity of these searches in a particular example, in which a heavy Z{sup ′} mediates the interactions between the SM and the DM. We find that for heavy dark matter indirect detection provides the strongest bounds on this scenario, while IceCube bounds are typically stronger than those from direct detection. The LHC constraints are dominant for smaller dark matter masses. These light masses are less motivated by thermal relic abundance considerations. We show that the dominant annihilation channels of the light DM in the Sun and the Galactic Center are either bb̄ or tt̄, while the heavy DM annihilation is completely dominated by Zh channel. The latter produces a hard neutrino spectrum which has not been previously analyzed. We study the neutrino spectrum yielded by DM and recast IceCube constraints to allow proper comparison with constraints from direct and indirect detection experiments and LHC exclusions.

  4. Complementarity of DM Searches in a Consistent Simplified Model: the Case of Z'

    CERN Document Server

    Jacques, Thomas; Morgante, Enrico; Racco, Davide; Rameez, Mohamed; Riotto, Antonio

    2016-01-01

    We analyze the constraints from direct and indirect detection on fermionic Majorana Dark Matter (DM). Because the interaction with the Standard Model (SM) particles is spin-dependent, a priori the constraints that one gets from neutrino telescopes, the LHC and direct detection experiments are comparable. We study the complementarity of these searches in a particular example, in which a heavy $Z'$ mediates the interactions between the SM and the DM. We find that in most cases IceCube provides the strongest bounds on this scenario, while the LHC constraints are only meaningful for smaller dark matter masses. These light masses are less motivated by thermal relic abundance considerations. We show that the dominant annihilation channels of the light DM in the Sun are either $b \\bar b$ or $t \\bar t$, while the heavy DM annihilation is completely dominated by $Zh$ channel. The latter produces a hard neutrino spectrum which has not been previously analyzed. We study the neutrino spectrum yielded by DM and recast Ice...

  5. Routine daily physical activity and glucose variations are strongly coupled in adults with T1DM.

    Science.gov (United States)

    Farabi, Sarah S; Carley, David W; Cinar, Ali; Quinn, Lauretta

    2015-12-01

    Type 1 Diabetes (T1DM) is characterized by altered glucose homeostasis resulting in wide glucose variations throughout a 24-h period. The relationship between routine daily physical activity and glucose variations has not been systematically investigated in adults with T1DM. The objectives of this study were to characterize and quantify the relationship between routine daily activity and glucose variations in a small group of adults with T1DM. Adults with T1DM treated with an insulin pump were recruited for the study. Over a 3-day period, glucose variations were monitored with a continuous glucose monitoring system (CGMS) and routine daily physical activity was assessed using an accelerometer-based physical activity-monitoring band. Simultaneous glucose and physical activity data for one 24-h period were used for analysis. Cross-correlation function and wavelet coherence analyses were employed to quantify the coupling between physical activity and glucose. Twelve subjects were included in the analysis. Cross-correlation function analysis revealed strong coupling between activity and glucose. Wavelet Coherence demonstrated that slower oscillations (120-340 min) of glucose and physical activity exhibited significantly greater coherence (F = 12.6, P < 0.0001) than faster oscillations (10 and 120 min). Physical activity and glucose demonstrate strong time and frequency-dependent coupling throughout a 24-h time period in adults with T1DM.

  6. Replacing single-view mediolateral oblique (MLO) digital mammography (DM) with synthesized mammography (SM) with digital breast tomosynthesis (DBT) images: Comparison of the diagnostic performance and radiation dose with two-view DM with or without MLO-DBT.

    Science.gov (United States)

    Kang, Hyo-Jin; Chang, Jung Min; Lee, Joongyub; Song, Sung Eun; Shin, Sung Ui; Kim, Won Hwa; Bae, Min Sun; Moon, Woo Kyung

    2016-11-01

    To evaluate the diagnostic performance and radiation dose of single view cranio-caudal (CC) digital mammography (DM) plus mediolateral oblique (MLO) digital breast tomosynthesis (DBT) combined with synthesized mammography (SM) in comparison with two-view DM with or without DBT. This study was approved by our institutional review board, and informed consent was obtained from 130 women. Paired two-view DM and single MLO-DBT with SM images were acquired, and four independent retrospective reading sessions of different combinations of DM, SM and DBT were performed for the presence of malignant tumors using jackknife alternative free-response receiver operator curve (JAFROC) methods. The diagnostic performances and average glandular dose (AGD) were compared between different combinations of DM, SM and DBT. Of 159 lesions in 130 patients, 27 were malignant. When using MLO-DBT with SM instead of MLO-DM, a significantly higher sensitivity (P=0.016) and specificity (P=0.012) were noted than with two-view DM, and comparable figure of merit (FOM), sensitivity, and specificity to two-view DM with DBT were noted. The mean AGD of CC-DM plus MLO-DBT with SM was 5.78mGy±1.06 per patient, which was significantly lower than that with two-view DM with MLO-DBT (8.45mGy±1.32; P <0.001) and slightly higher than that with two-view DM (5.30mGy±0.63). The combined use of CC-DM plus MLO-DBT with SM showed higher sensitivity and specificity to two-view DM with a smaller AGD increment and comparable diagnostic performance to that of two-view DM with MLO-DBT with a significantly lower mean AGD. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  7. A Peptide/MHCII conformer generated in the presence of exchange peptide is substrate for HLA-DM editing

    OpenAIRE

    Andrea Ferrante; Jack Gorski

    2012-01-01

    The mechanism of HLA-DM (DM) activity is still unclear. We have shown that DM-mediated peptide release from HLA-DR (DR) is dependent on the presence of exchange peptide. However, DM also promotes a small amount of peptide release in the absence of exchange peptide. Here we show that SDS-PAGE separates purified peptide/DR1 complexes (pDR1) into two conformers whose ratio is peptide K d-dependent. In the absence of exchange peptide, DM only releases peptide from the slower migrating conformer. ...

  8. Dark matter, dark radiation and Higgs phenomenology in the hidden sector DM models

    CERN Document Server

    Ko, P

    2015-01-01

    I present a class of hidden sector dark matter (DM) models with local dark gauge symmetries, where DM is stable due to unbroken local dark gauge symmetry, or due topology, or it is long-lived because of some accidental symme- tries, and the particle contents and their dynamics are completely fixed by local gauge symmetries. In these models, one have two types of natural force mediators, dark gauge bosons and dark Higgs boson, which would affect DM and Higgs phenomenology in important ways. I discuss various phenomenological issues including the GeV scale gamma-ray excess from the galactic center (GC), (in)direct detection signatures, dark radiation, Higgs phenomenology and Higgs inflation assisted by dark Higgs.

  9. Separation and Purification of Total Phloroglucinols inDryopteris crassirhizoma with DM-130 Macroporous Adsorption Resin

    Institute of Scientific and Technical Information of China (English)

    Jin Zhe; Huang Jian-ping; Wang He-meng; Ju han-xun; Ren Si-rui; Chang Ying

    2015-01-01

    To improve the purity of the total phloroglucinols fromDryopteris crassirhizoma extracts, the separation and purification conditions of the total phloroglucinols fromDryopteris crassirhizoma were studied with DM-130 macroporous adsorption resin in this study. Adsorption rate, elution rate and purity of the total phloroglucinols were used as indexes to investigate the adsorption and desorption capacity of the total phloroglucinols with DM-130 macroporous adsorption resin. Through the study, the optimum sample concentration of the total phloroglucinols and maximum sample volume were 1.5 mg• mL-1 and 7 BV (210 mL), respectively. The optimum desorption conditions were achieved by using 80% ethanol as desorption solvent at elution flow rate of 1.0 mL• min-1. The result showed DM-130 macroporous adsorption resin performed effective adsorption and desorption. After purification, the purity of the total phloroglucinols increased by 11.5-fold.

  10. DM dimensioning for the next generation AO systems : strategies and rules

    Science.gov (United States)

    Costille, A.; Conan, J.-M.

    2007-07-01

    Adaptive optics (AO) provides a real time correction of turbulence and allows to improve the angular resolution of the astronomical telescopes. The design of AO systems and of its key components (deformable mirror (DM), wave-front sensors...) is very important to provide the best performance. The definition rules are well known for 10m class telescopes. However the study of the next generation of telescopes, especially the Extremely Large Telescope (ELT), associated with the development of new technological solutions, particularly concerning DMs, require to refine and in some cases to reconsider these rules. Both turbulent parameters (seeing, outer scale ...), telescope effects, system design and performance requirements have to be considered in order to define the DM key parameters (stroke, inter-actuator stroke, influence function ...). We present a study based on the combination of analytical expressions and numerical simulation results. We do not give absolute rules of design but we present key aspects to keep in mind when designing the DM.

  11. Plant insecticide L-canavanine repels Drosophila via the insect orphan GPCR DmX.

    Directory of Open Access Journals (Sweden)

    Christian Mitri

    2009-06-01

    Full Text Available For all animals, the taste sense is crucial to detect and avoid ingesting toxic molecules. Many toxins are synthesized by plants as a defense mechanism against insect predation. One example of such a natural toxic molecule is L-canavanine, a nonprotein amino acid found in the seeds of many legumes. Whether and how insects are informed that some plants contain L-canavanine remains to be elucidated. In insects, the taste sense relies on gustatory receptors forming the gustatory receptor (Gr family. Gr proteins display highly divergent sequences, suggesting that they could cover the entire range of tastants. However, one cannot exclude the possibility of evolutionarily independent taste receptors. Here, we show that L-canavanine is not only toxic, but is also a repellent for Drosophila. Using a pharmacogenetic approach, we find that flies sense food containing this poison by the DmX receptor. DmXR is an insect orphan G-protein-coupled receptor that has partially diverged in its ligand binding pocket from the metabotropic glutamate receptor family. Blockade of DmXR function with an antagonist lowers the repulsive effect of L-canavanine. In addition, disruption of the DmXR encoding gene, called mangetout (mtt, suppresses the L-canavanine repellent effect. To avoid the ingestion of L-canavanine, DmXR expression is required in bitter-sensitive gustatory receptor neurons, where it triggers the premature retraction of the proboscis, thus leading to the end of food searching. These findings show that the DmX receptor, which does not belong to the Gr family, fulfills a gustatory function necessary to avoid eating a natural toxin.

  12. DM235 (sunifiram): a novel nootropic with potential as a cognitive enhancer.

    Science.gov (United States)

    Ghelardini, C; Galeotti, N; Gualtieri, F; Romanelli, M N; Bucherelli, C; Baldi, E; Bartolini, A

    2002-06-01

    DM235 (sunifiram), a new compound structurally related to piracetam, prevented the amnesia induced by scopolamine (1.5 mg kg(-1) i.p.), after intraperitoneal (0.001-0.1 mg kg(-1)) or oral (0.01-0.1 mg kg(-1)) administration, as shown by a passive avoidance test in mice. The antiamnesic effect of DM235 was comparable to that of well-known nootropic drugs such as piracetam (30-100 mg kg(-1) i.p.), aniracetam (100 mg kg(-1) p.o.) or rolipram (30 mg kg(-1) p.o.). DM235 also prevented mecamylamine (20 mg kg(-1) i.p.)-, baclofen (2 mg kg(-1) i.p.)- and clonidine (0.125 mg kg(-1) i.p.)-induced amnesia in the same test. In the Morris water maze test with rats, scopolamine (0.8 mg kg(-1) i.p.) inhibited the reduction of escape latency in both acquisition and retention/retraining tests. DM235 (0.1 mg kg(-1) i.p.), 20 min before each daily acquisition training, prevented the scopolamine-induced memory impairment. DM235 (1 mg kg(-1) i.p.) also reduced the duration of pentobarbitone-induced hypnosis in mice without modifying the induction time of hypnosis. At the highest effective doses, the investigated compound neither impaired motor coordination (rota-rod test), nor modified spontaneous motility and inspection activity (Animex and hole board tests). These results indicate that DM235, a compound structurally related to piracetam, is a novel nootropic endowed with the capability to prevent cognitive deficits at very low doses. Indeed, its potency is about 1,000 times higher than that of the most active piracetam-like compounds.

  13. Polyuria with the Concurrent manifestation of Central Diabetes Insipidus (CDI) & Type 2 Diabetes Mellitus (DM)

    OpenAIRE

    Shin, Hyun-Jong; Kim, Jae-ha; Yi, Joo-Hark; Han, Sang-Woong; Kim, Ho-Jung

    2012-01-01

    We report a rare case of the concurrent manifestation of central diabetes insipidus (CDI) and type 2 diabetes mellitus (DM). A 56 year-old man was diagnosed as a type 2 DM on the basis of hyperglycemia with polyuria and polydipsia at a local clinic two months ago and started an oral hypoglycemic medication, but resulted in no symptomatic improvement at all. Upon admission to the university hospital, the patient's initial fasting blood sugar level was 140 mg/dL, and he showed polydipsic and po...

  14. 8 CFR 236.3 - Detention and release of juveniles.

    Science.gov (United States)

    2010-01-01

    ... 8 Aliens and Nationality 1 2010-01-01 2010-01-01 false Detention and release of juveniles. 236.3... Aliens Prior to Order of Removal § 236.3 Detention and release of juveniles. (a) Juveniles. A juvenile is defined as an alien under the age of 18 years. (b) Release. Juveniles for whom bond has been posted,...

  15. The combined effect of the T2DM susceptibility genes is an important risk factor for T2DM in non-obese Japanese: a population based case-control study

    Directory of Open Access Journals (Sweden)

    Yamakawa-Kobayashi Kimiko

    2012-02-01

    Full Text Available Abstract Background Type 2 diabetes mellitus (T2DM is a complex endocrine and metabolic disorder. Recently, several genome-wide association studies (GWAS have identified many novel susceptibility loci for T2DM, and indicated that there are common genetic causes contributing to the susceptibility to T2DM in multiple populations worldwide. In addition, clinical and epidemiological studies have indicated that obesity is a major risk factor for T2DM. However, the prevalence of obesity varies among the various ethnic groups. We aimed to determine the combined effects of these susceptibility loci and obesity/overweight for development of T2DM in the Japanese. Methods Single nucleotide polymorphisms (SNPs in or near 17 susceptibility loci for T2DM, identified through GWAS in Caucasian and Asian populations, were genotyped in 333 cases with T2DM and 417 control subjects. Results We confirmed that the cumulative number of risk alleles based on 17 susceptibility loci for T2DM was an important risk factor in the development of T2DM in Japanese population (P P P = 0.88 for trend. Conclusions Our findings indicate that there is an etiological heterogeneity of T2DM between obese/overweight and non-obese subjects.

  16. Uveitis in juvenile chronic arthritis.

    Science.gov (United States)

    Kanski, J J

    1990-01-01

    About 20% of patients with juvenile chronic arthritis develop uveitis which is frequently bilateral. Risk factors for uveitis are: female gender, pauciarticular onset of arthritis, presence of circulating antinuclear antibodies, and the antigens HLA-DW5 and HLA-DPw2. The visual prognosis in patients with uveitis is good in 25% and fair in 50%. The remaining 25% develop cataract and/or glaucoma. The management of glaucoma is unsatisfactory, but the results of cataract surgery by lensectomy are good.

  17. Clinical analysis of gastrointestinal involvement in polymyositis and dermatomyositis%多发性肌炎和皮肌炎消化道受累的临床研究

    Institute of Scientific and Technical Information of China (English)

    姜丽丽; 卢昕

    2012-01-01

    Objective:To investigate the incidence,clinical feature,risk factors of gastrointestinal involvement in polymyositis(PM)and dermatomyositis (DM) .Methods: Clinical and laboratory data of the patients diagnosed with PM or DM between Mar. 1986 and Jun.2010 in the hospital were collected and compared in patients with and without gastrointestinal involvement.Results:Out of 192 patients there were 82 cases (42.7%)with gastrointestinal involvement.Of which 71 had dysphagia,8 had oesophageal hiatus disorder,8 had esophagitis, 10 had oe-sophageal motility disorders, 6 had weakened peristole, 2 had decreased gastric tonus, 1 had pseudomembranous enteritis and 1 had pneumatosis cystoides intestinalis.There were no statistical significance between the two groups in diagnosis (PM/DM)and sexuality, but the average onset age was younger in the group with gastrointestinal involvement than the group without gastrointestinal involvement, and the incidence of difficulty in raising head was higher in the former group than the latter one,while the incidence of interstitial lung disease was lower in the latter group (all P<0.05).No significant difference were observed in other clinical features like general fatigue, Gottron' s sign, heliotrope erythra, neither in laboratory parameters like creatinekinase, erythrocyte sedimentation rate or C reative protein.There were no significant difference in types of gastrointestinal involvement between patients with PM and those with DM.Conclusion:Gastrointestinal involvement more frequently occurred in younger PM/DM patients and those with difficulty in raising heads,while ILD seems negative correlated with gastrointestinal involvement in PM/DM patients.%目的:研究多发性肌炎(PM)和皮肌炎(DM)合并消化道受累的发生率、临床表现特点及危险因素.方法:回顾性分析中日友好医院风湿免疫科1986年3月~ 2010年6月住院的192例PM/DM患者的临床表现、辅助检查、实验室参数的特点,比较伴消

  18. Juvenile morphology in baleen whale phylogeny.

    Science.gov (United States)

    Tsai, Cheng-Hsiu; Fordyce, R Ewan

    2014-09-01

    Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

  19. Juvenile morphology in baleen whale phylogeny

    Science.gov (United States)

    Tsai, Cheng-Hsiu; Fordyce, R. Ewan

    2014-09-01

    Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

  20. 基于DM365的人脸检测系统的设计与实现%Design and implementation of human facedetection based on DM365

    Institute of Scientific and Technical Information of China (English)

    张健; 蒋挺

    2012-01-01

    随着安防、人机交互等需求的提出,人脸检测已经成为模式识别领域的一个研究热点,日益受到人们的重视。近年来,随着嵌入式系统的快速发展,出现了基于嵌入式设备的人脸检测系统,由于其具有体积小、成本低、布设方便的优点,逐渐得到了人们的青睐,所以取得了很大的发展。本文主要研究和开发了基于德州仪器DM365视频监控设备的人脸检测系统,针对 Adaboost 的人脸检测算法进行了研究,利用改进的带加权判决函数的级联分类器来提高检测准确率,并给出了在DM365嵌入式视频监控设备上的实现。%The face detection algorithm, currently, receives an increasing attention, and has become a hot topic in the field of the pattern recognition with the requirement of the security guards against theft and the human-computer interaction. With the rapid development of the embedded system, the face detection system based on embedded platform emerged in recent years. And this system has earned the favor of users and developed rapidly due to its advantages of tiny volume, low cost and convenient layout. In this paper, we researched and developed a face detection system based on DM365. The AdaBoost algorithm was improved by employing a cascade classifier with weighted decision function. This improved algorithm enhanced accuracy rate of the detection and was implemented on DM365.