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https://www.readbyqxmd.com/read/27908312/calcinosis-in-poly-dermatomyositis-clinical-and-laboratory-predictors-and-treatment-options
#1
Micaela Fredi, Francesca Bartoli, Ilaria Cavazzana, Angela Cerebelli, Nice Carabellese, Angela Tincani, Minoru Satoh, Franco Franceschini
OBJECTIVES: We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM). METHODS: We carried out a retrospective analysis of a cohort of myositis patients attending our clinic between January 2013 and May 2014. RESULTS: 74 patients (58 females, 16 males) with PM (30 cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis (1 case) were enrolled...
November 14, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27835954/characteristics-and-outcome-of-children-with-juvenile-dermatomyositis-in-cape-town-a-cross-sectional-study
#2
Lawrence Owino Okong'o, Monika Esser, Jo Wilmshurst, Christiaan Scott
BACKGROUND: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. METHODS: We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa...
November 11, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27833984/a-case-of-a-generalized-symptomatic-calcinosis-in-systemic-sclerosis
#3
Cristian Radu Jecan, Ştefan Iulian Bedereag, Ruxandra Diana Sinescu, Valentin Titus Grigorean, Cristina Nicoleta Cozma, Anca Bordianu, Ioan Petre Florescu
Calcinosis consists of abnormal calcium deposition in soft tissues, which appears often in patients with limited systemic sclerosis, being one of the criteria of CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome. With a long evolution, the aim of the treatment is to control the symptoms and prevent complications. In this article, we present the challenging management of a profuse lesion of calcinosis in a patient with systemic sclerosis. We describe the case of a 52-year-old woman with systemic sclerosis and CREST syndrome who was admitted in our Department with multiple painful and disabling tumoral masses, situated in nearly all joints...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27693175/esophageal-abnormalities-in-juvenile-localized-scleroderma-is-it-associated-with-other-extracutaneous-manifestations
#4
Clarissa C M Valões, Glaucia V Novak, Juliana B Brunelli, Katia T Kozu, Ricardo K Toma, Clovis A Silva
OBJECTIVE: To assess esophageal involvement (EI) in juvenile localized scleroderma (JLS) population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. METHODS: For a period of 31 years, 5,881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia) and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring...
September 17, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27660938/a-novel-approach-in-the-treatment-of-calcinosis-cutis
#5
Robert Hawkins, Ricky Mehta
No abstract text is available yet for this article.
October 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/27622522/calciphylaxis-and-martorell-hypertensive-ischemic-leg-ulcer-same-pattern-one-pathophysiology
#6
Jürg Hafner
This review presents a closer look at four diseases which are probably closely related to one another pathophysiologically: (a) calciphylaxis (distal pattern); (b) calciphylaxis (proximal pattern); (c) Martorell hypertensive ischemic leg ulcer; (d) calciphylaxis with normal renal and parathyroid function (synonym: eutrophication). The four diseases have largely the same risk factors: (1) arterial hypertension, (2) diabetes mellitus (types 1 and 2), (3) secondary or tertiary hyperparathyroidism (in end-stage kidney disease) and (4) oral anticoagulation with vitamin K antagonists...
September 14, 2016: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/27608765/bilateral-strio-pallido-dentate-calcinosis-fahr-s-disease-report-of-seven-cases-and-revision-of-literature
#7
Elisabetta Savino, Cecilia Soavi, Eleonora Capatti, Massimo Borrelli, Giovanni B Vigna, Angelina Passaro, Giovanni Zuliani
BACKGROUND: Fahr's disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term "Fahr's syndrome" is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome...
September 8, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27606883/the-erbium-yag-laser-a-new-technique-for-the-treatment-of-scrotal-calcinosis
#8
Markus Meissner, Johannes Kleemann, Roland Kaufmann, Falk Ochsendorf
Scrotal calcinosis is a rare local entity characterized by multiple, calcified and painless nodules of the scrotal skin and was first described by Lewinski in 1883 (Figure 1a).(1) It normally appears in men aged 20-40 years.(2) For a long time, the pathological origin of this disease was unclear. In the last years it became more and more obvious, that dystrophic calcification of epithelial cysts involving inflammation, rupture, calcification and finally obliteration of the cyst wall is the main pathological correlate for scrotal calcinosis...
September 8, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27494629/safety-and-efficacy-of-extracorporeal-shock-wave-therapy-eswt-in-calcinosis-cutis-associated-with-systemic-sclerosis
#9
Sandra Blumhardt, Diana P Frey, Martin Toniolo, Hatem Alkadhi, Ulrike Held, Oliver Distler
OBJECTIVES: Calcinosis cutis is a frequent, difficult to treat manifestation of systemic sclerosis (SSc) associated with high morbidity. The aim of this prospective, controlled, monocentric study was to assess safety and efficacy of extracorporeal shock wave therapy (ESWT) for calcinosis cutis of the finger in SSc patients. METHODS: A 12-week proof of concept study in which 4 SSc patients with calcinosis cutis were treated at one painful finger with high-energy, focused ESWT, in 3 sessions with one-week interval between each session...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27437158/assessment-and-surgical-treatment-of-calcinosis-of-the-shoulder-associated-with-crest-syndrome
#10
R Manohara, S J Breusch
We report an unusual case of a 65-year-old lady with CREST syndrome with multiple upper and lower limb calcinosis, who presented with severe shoulder pain and stiffness, with widespread intra- and extra-articular calcinosis, which was refractory to conservative measures. We were able to identify the main cause of her symptoms through serial diagnostic injections as calcific biceps tendinosis. We will discuss her assessment and surgical management and the pathophysiology and various treatment modalities for managing the soft tissue calcinosis in rheumatological diseases...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27247203/is-rituximab-an-effective-treatment-of-refractory-calcinosis
#11
Maria Dubos, Kim Ly, Clothilde Martel, Anne Laure Fauchais
Calcinosis, the deposition of calcified material in soft tissues, is frequently seen in systemic sclerosis and dermatomyositis. Treatment options are limited, with disappointing results. Some recent case reports suggest that rituximab may be an attractive therapeutic option. In case 1, a 54-year-old woman who presented with rheumatoid arthritis in association with scleromyositis was treated with rituximab for rheumatoid arthritis. Despite this, she developed multiple progressive calcinosis, necessitating extracorporeal shock wave lithotripsy to limit calcinosis extension and pain...
May 31, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27241706/calcinosis-in-systemic-sclerosis-subsets-distribution-and-complications
#12
Francesca Bartoli, Ginevra Fiori, Francesca Braschi, Laura Amanzi, Cosimo Bruni, Jelena Blagojevic, Silvia Bellando-Randone, Laura Cometi, Carolina de Souza Mueller, Serena Guiducci, Laura Rasero, Francesco Epifani, Daniel E Furst, Marco Matucci-Cerinic
OBJECTIVE: To retrospectively analyse the features of calcinosis in a cohort of SSc patients. METHODS: Charts of SSc patients attending the Ulcer Unit of the Rheumatology Department, University of Florence and presenting a clinical suspicion of calcinosis were considered in the study. Data on clinical history, including recent skin changes, and clinical examination of all areas with suspected calcinosis, radiological imaging of the calcinotic area, demographics and SSc-related organ involvement and pain measured by a visual analogue scale were recorded...
September 2016: Rheumatology
https://www.readbyqxmd.com/read/27164190/phenotypic-and-genotypic-characterization-and-treatment-of-a-cohort-with-familial-tumoral-calcinosis-hyperostosis-hyperphosphatemia-syndrome
#13
Mary Scott Ramnitz, Pravitt Gourh, Raphaela Goldbach-Mansky, Felasfa Wodajo, Shoji Ichikawa, Michael J Econs, Kenneth E White, Alfredo Molinolo, Marcus Y Chen, Theo Heller, Jaydira Del Rivero, Patricia Seo-Mayer, Bita Arabshahi, Malaka B Jackson, Sarah Hatab, Edward McCarthy, Lori C Guthrie, Beth A Brillante, Rachel I Gafni, Michael T Collins
Familial tumoral calcinosis (FTC)/hyperostosis-hyperphosphatemia syndrome (HHS) is a rare disorder caused by mutations in the genes encoding fibroblast growth factor-23 (FGF23), N-acetylgalactosaminyltransferase 3 (GALNT3), or KLOTHO. The result is functional deficiency of, or resistance to, intact FGF23 (iFGF23), causing hyperphosphatemia, increased renal tubular reabsorption of phosphorus (TRP), elevated or inappropriately normal 1,25-dihydroxyvitamin D3 (1,25D), ectopic calcifications, and/or diaphyseal hyperostosis...
October 2016: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/27163355/topical-sodium-thiosulfate-a-treatment-for-calcifications-in-hyperphosphatemic-familial-tumoral-calcinosis
#14
Jérémy Jost, Claire Bahans, Marie Courbebaisse, Tu-Anh Tran, Agnès Linglart, Karelle Benistan, Anne Lienhardt, Hadile Mutar, Elodie Pfender, Voa Ratsimbazafy, Vincent Guigonis
CONTEXT: Hyperphosphatemic familial tumoral calcinosis (HFTC) and hyperphosphatemia hyperostosis syndrome (HHS) are rare diseases characterized by hyperphosphatemia and ectopic calcifications or recurrent episodes of diaphysitis. In the setting of metabolic or inflammatory diseases, recent data suggest that systemic administration of sodium thiosulfate (STS) could be effective in the treatment of ectopic calcifications but may also be poorly tolerated (digestive symptoms, metabolic acidosis)...
July 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27136920/systemic-lupus-erythematosus-related-hypercalcemia-with-ectopic-calcinosis
#15
REVIEW
Lidan Zhao, Linfang Huang, Xuan Zhang
We report a case of a 39-year-old female with active systemic lupus erythematosus who complained of lethargy and weakness with a moderate renal impairment. Hypercalcemia was confirmed by laboratory examination. Her X-ray revealed significant ectopic calcinosis in subcutaneous tissue of bilateral hands, and Tc-99(m) methylene diphosphonate bone scan revealed a remarkably intense uptake of bilateral lungs. She had no evidence suggestive of other diseases related to hypercalcemia such as hyperparathyroidism and malignancy...
July 2016: Rheumatology International
https://www.readbyqxmd.com/read/27088846/a-23-year-old-patient-with-secondary-tumoral-calcinosis-regression-after-subtotal-parathyroidectomy-a-case-report
#16
Katharina E Niemann, Feride Kröpil, Martin F Hoffmann, Marlon O Coulibaly, Thomas A Schildhauer
INTRODUCTION: Tumoral calcinosis (TC) is a rare disorder defined by hyperphosphatemia and ectopic calcifications in various locations. The most common form of TC is associated with disorders such as renal insufficiency, hyperparathyroidism, or hypervitaminosis D. The primary (hereditary) TC is caused by inactivating mutations in either the fibroblast growth factor 23 (FGF23), the GalNAc transferase 3 (GALNT3) or the KLOTHO (KL) gene. PRESENTATION OF CASE: We report here a case of secondary TC in end-stage renal disease...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27007612/calcinosis-in-juvenile-dermatomyositis-frequency-risk-factors-and-outcome
#17
Isha Saini, Mani Kalaivani, Sushil Kumar Kabra
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...
July 2016: Rheumatology International
https://www.readbyqxmd.com/read/26992073/worsening-of-calcinosis-cutis-with-teriparatide-treatment-in-two-osteoporotic-patients
#18
A F Echeverri, F E Ospina, C A Cañas, A Agualimpia, J P Suso, G J Tobón, F Bonilla-Abadía
We present two cases of patients with systemic autoimmune diseases (one with dermatomyositis and the second one with CREST syndrome), who presented a worsening of calcinosis cutis after treatment of osteoporosis with teriparatide. To our knowledge, this association is not described in the literature and might be considered in the spectrum of adverse reactions to teriparatide. This article is protected by copyright. All rights reserved.
March 16, 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/26901061/sterile-%C3%AE-motif-domain-containing-9-is-a-novel-cellular-interacting-partner-to-low-risk-type-human-papillomavirus-e6-proteins
#19
Jia Wang, Crystal Dupuis, Stephen K Tyring, Michael P Underbrink
Low-risk type human papillomavirus (HPV) 6 and 11 infection causes recurrent respiratory papillomatosis (RRP) and genital warts. RRP is the most common benign tumor of the larynx in children with frequent relapses. Repeated surgeries are often needed to improve vocal function and prevent life-threatening respiratory obstruction. Currently, there are no effective treatments available to completely eliminate these diseases, largely due to limited knowledge regarding their viral molecular pathogenesis. HPV E6 proteins contribute to cell immortalization by interacting with a variety of cellular proteins, which have been well studied for the high-risk type HPVs related to cancer progression...
2016: PloS One
https://www.readbyqxmd.com/read/26833515/advances-in-pathogenesis-and-treatment-of-systemic-sclerosis
#20
Christopher P Denton
Systemic sclerosis is the most severe disease within the scleroderma spectrum and is a major medical challenge with high mortality and morbidity. There have been advances in understanding of pathogenesis that reflect the interplay between immune-inflammatory processes and vasculopathy and fibrosis. It can be regarded as a disease of connective tissue repair and this leads to organ-based complications. However the aetiology and triggering events remain to be elucidated. Treatment is available for many aspects of the disease although the available therapies are not curative and some complications remain very challenging, especially non-lethal manifestations such as fatigue, calcinosis and anorectal dysfunction...
February 2016: Clinical Medicine: Journal of the Royal College of Physicians of London
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