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Kenan Barut, Pinar Ozge Avar Aydin, Amra Adrovic, Sezgin Sahin, Ozgur Kasapcopur
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty...
January 5, 2017: Clinical Rheumatology
Rohit Aggarwal, Priyadarshini Loganathan, Diane Koontz, Zengbiao Qi, Ann M Reed, Chester V Oddis
OBJECTIVE: The aim was to assess the efficacy of rituximab for the cutaneous manifestations of adult DM and JDM. METHODS: Patients with refractory adult DM (n = 72) and JDM (n = 48) were treated with rituximab in a randomized placebo-phase-controlled trial [either rituximab early drug (week 0/1) or rituximab late arms (week 8/9), such that all subjects received study drug]. Stable concomitant therapy was allowed. Cutaneous disease activity was assessed using the Myositis Disease Activity Assessment Tool, which grades cutaneous disease activity on a visual analog scale...
November 11, 2016: Rheumatology
Naoteru Miyata, Katsura Emoto, Yoshiaki Dei, Kazuhiro Tomiyasu, Ryoko Ishiyama, Tomofumi Horie, Gen Sakai, Toshiyuki Tahara
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...
September 2016: Case Reports in Oncology
Patrick P J Mulder, Susannah L de Witte, Geert M Stoopen, Jan van der Meulen, Piet G van Wikselaar, Erik Gruys, Maria J Groot, Ron L A P Hoogenboom
To investigate the potential transfer of pyrrolizidine alkaloids (PAs), laying hens were fed for 14 days with diets containing 0.5% of dried common ragwort, common groundsel, narrow-leaved ragwort or viper's bugloss, or 0.1% of common heliotrope. This resulted in total PA levels in feed of respectively 5.5, 11.1, 53.1, 5.9 and 21.7 mg kg(-)(1), with varying composition. PAs were transferred to eggs, in particular yolk, with steady-state levels of respectively 12, 21, 216, 2 and 36 µg kg(-)(1). Overall transfer rates for the sum of PAs were estimated between 0...
December 2016: Food Additives & Contaminants. Part A, Chemistry, Analysis, Control, Exposure & Risk Assessment
Hagop S Atamian, Nicky M Creux, Evan A Brown, Austin G Garner, Benjamin K Blackman, Stacey L Harmer
Young sunflower plants track the Sun from east to west during the day and then reorient during the night to face east in anticipation of dawn. In contrast, mature plants cease movement with their flower heads facing east. We show that circadian regulation of directional growth pathways accounts for both phenomena and leads to increased vegetative biomass and enhanced pollinator visits to flowers. Solar tracking movements are driven by antiphasic patterns of elongation on the east and west sides of the stem...
August 5, 2016: Science
Efi Levizou, Aris Kyparissis
A novel type of heliotropic leaf movement is presented for Capparis spinosa L., a summer perennial shrub of Mediterranean and arid ecosystems. In contrast to plants that demonstrate uniform diaheliotropic and/or paraheliotropic movement for all their foliage, the alternate leaves of C. spinosa follow different movement patterns according to their stem azimuth and the side of the stem that they come from (cluster). Additionally, leaf movement for each cluster may not be uniform throughout the day, showing diaheliotropic characteristics during half of the day and paraheliotropic characteristics during the rest of the day...
September 2016: Tree Physiology
Naomi Yoshida, Shinjiro Kaieda, Kumi Tomozoe, Morihiro Tajiri, Daisuke Wakasugi, Masaki Okamoto, Masaki Tominaga, Hiroaki Ida, Tomoaki Hoshino
A 62-year-old man presented with heliotrope rash, Gottron's sign, and mild muscle weakness. Both of his lung fields showed interstitial changes that worsened rapidly. He was diagnosed with clinical amyopathic dermatomyositis with rapidly progressive interstitial lung disease. The patient died of respiratory failure, despite the administration of immunosuppressive therapy. Autopsy revealed diffuse alveolar damage. An antibody analysis, which was performed postmortem, detected the presence of anti-melanoma differentiation-associated gene (MDA)-5 antibodies...
2016: Internal Medicine
Jeremy Udkoff, Philip R Cohen
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...
October 2016: American Journal of Clinical Dermatology
Yao-Fan Fang, Yeong-Jian Jan Wu, Chang-Fu Kuo, Shue-Fen Luo, Kuang-Hui Yu
This study aims to investigate the prevalence and predictive risk factors of malignancy in patients with polymyositis (PM) and dermatomyositis (DM). The medical records of 192 PM/DM patients followed up in a medical center between January 2000 and December 2013 were reviewed. Among the 192 patients, 33 patients (17.2 %) had associated cancer. Both PM and DM are significantly associated with cancer, although the risk of cancer appears to be somewhat higher among patients with DM (23.0 %) than among those with PM (8...
August 2016: Clinical Rheumatology
Xinyu Nan, Abimbola Adike, Lawrence Rice, Viviane Bunin
A 55-year-old woman presented with a 3-week history of weakness. On examination, she had a heliotrope rash, V-neck and shawl signs. There was symmetric proximal muscle weakness, and 2/5 strength in upper and lower extremities. Laboratory values showed CK 5836, CRP 14.9, erythrocyte sedimentation rate 49, lactate dehydrogenase 633, negative antinuclear antibodies and anti-Jo1 antibodies. Muscle biopsy and immunohistochemistry findings were consistent with dermatomyositis. Treatment with high-dose steroids, cyclophosphamide and intravenous immunoglobulin (IVIG) was started, without improvement of symptoms...
April 28, 2016: BMJ Case Reports
Xiaomin Yu, Dongyan Qin, Dehua Ma, Qin Yao
A 27-year-old female patient presented with a 3-month history of bilateral orbital and facial edema accompanied by skin erythema and heliotrope rash. The left lesion was more critical than the right. Limb muscles were occasionally sore. On physical examination, scattered hemorrhagic rashes were observed on the patient's face and neck. Upon laboratory testing, creatine kinase (CK) was markedly elevated at 1,543.2 U/l, while CK-MB isoenzyme (27.4 U/l), α-hydroxybutyric dehydrogenase (188.4 U/l) and aspartate aminotransferase (65...
April 2016: Oncology Letters
M Manie
Inflammatory myopathy (IM) is a rubric term to describe a heterogeneous group of muscle diseases typified by dermatomyositis and polymyositis. The current classifications are unsatisfactory, but IM associated with other connective tissue diseases (CTDs), such as systemic lupus erythematosus, underlying malignancy and HIV, should also be included. Although uncommon, IM should always be considered in a patient who presents with proximal weakness of gradual onset and has raised serum muscle enzymes. The diagnosis may be obvious if the patient has diagnostic skin signs such as heliotropic rash (peri-orbital discoloration) and Gottron’s lesions (typically on the extensor surfaces of the fingers)...
December 2015: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
Sukesh Sukumaran, Tiffany Palmer, Vini Vijayan
No abstract text is available yet for this article.
April 2016: Journal of Pediatrics
Y Ugan, M Sahin, A Dogru, D Bayram, A M Ceyhan, S E Tunc
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...
November 2015: La Clinica Terapeutica
Qiang Zhang, Yihan Xiong, Haiyan An, Konstantin Boucke, Georg Treusch
Taking advantage of robust facet passivation, we unveil a laser "fossil" buried within a broad area laser diode (LD) cavity when the LD was damaged by applying a high current. For the first time, novel physical phenomena have been observed at these dramatically elevated energy densities within the nanoscale LD waveguide. The observation of the laser "fossil" is interpreted with different mechanisms, including: the origination of bulk catastrophic optical damage (COD) due to locally high energy densities, heliotropic COD growth, solid-liquid-gas phase transformations, strong longitudinal phonon cooling effect on the molten COD wave front, and the formation of patterns due to laser lateral modes...
2016: Scientific Reports
Kayo Nakamura, Noriko Nakatsuka, Masatoshi Jinnin, Takamitsu Makino, Ikko Kajihara, Katsunari Makino, Noritoshi Honda, Kuniko Inoue, Satoshi Fukushima, Hironobu Ihn
Fms-like tyrosine kinase 3 (Flt-3) is a cytokine receptor expressed on the surface of bone-marrow progenitor of hematopoietic cells. Flt-3 ligands are produced by peripheral blood mononuclear cells, and found in various human body fluids. Flt-3 signal is involved in the regulation of vessel formation as well as B cell differentiation, suggesting that Flt-3 signal contributes to the pathogenesis of vascular abnormalities and immune dysregulation in rheumatic diseases. The aim of the present study is to examine serum Flt-3 ligand levels in patients with various rheumatic diseases, and to evaluate the possibility that serum Flt-3 ligand levels can be a useful disease marker...
October 2015: Bioscience Trends
Yanina J Jansen, Peter Janssens, Anne Hoorens, Max S Schreuer, Teofila Seremet, Sofie Wilgenhof, Bart Neyns
A 61-year-old man was diagnosed with stage IIIB BRAF V600E mutant melanoma in October 2012. He was treated with a combination therapy of dabrafenib and trametinib. He remained in complete remission for 18 months and the treatment was well tolerated after dose reduction because of pyrexia. In March 2013, he developed bilateral pitting edema of the legs with an erythematous, slightly infiltrated rash on his back and upper arms. His face was edematous, with a heliotrope rash-like aspect. Eye examination showed bilateral blepharitis...
December 2015: Melanoma Research
Mukta Sharma, Aditi Prashar, Rajiv Tuli, Rattan K Sharma, Vikram K Mahajan
Several ophthalmic manifestations in patients with dermatomyositis have been described but involvement of the retina is rare. A 39-year-old man diagnosed with dermatomysitis had developed sudden painless visual loss in the left eye. He was non-diabetic and non-hypertensive. He was on treatment with systemic prednisolone (50 mg/day) and methotrexate (7.5 mg/day) for dermatomyositis. His visual acuity was finger counting at 2 feet in the left eye and 6/6 in the right eye. Left fundus showed slight blurring of the optic disc margins, large peripapillary flame-shaped haemorrhages and extensive area of opacification suggestive of central retinal artery occlusion (CRAO) with sparing of the area supplied by the cilioretinal artery...
September 25, 2015: International Journal of Rheumatic Diseases
B Volc-Platzer
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...
August 2015: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Yoshinao Muro, Kazumitsu Sugiura, Masashi Akiyama
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...
December 2016: Clinical Reviews in Allergy & Immunology
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