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https://www.readbyqxmd.com/read/28343327/osteolytic-variant-poems-syndrome-an-uncommon-presentation-of-osteosclerotic-myeloma
#1
Michael S Clark, Benjamin M Howe, Katrina N Glazebrook, Michelle L Mauermann, Stephen M Broski
Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome, a form of osteosclerotic myeloma, is a multisystem disease related to a monoclonal plasma cell proliferative disorder. Osseous lesions are most commonly sclerotic on radiographs and computed tomography (CT), demonstrate low T1 and T2 signal intensity on magnetic resonance imaging (MRI), and have variable degrees of avidity on positon emission tomography (PET) imaging using 18-fluorodeoxyglucose ((18)F-FDG). We present three cases of POEMS syndrome manifesting as osteolytic lesions with indolent features, including well-defined thin sclerotic rims, no cortical disruption or periosteal reaction, no associated soft-tissue mass, and a periarticular location, all features that could lead to misinterpretation as benign bone lesions...
March 25, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28343168/potential-skin-involvement-in-als-revisiting-charcot-s-observation-a-review-of-skin-abnormalities-in-als
#2
Bastien Paré, François Gros-Louis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motor neurons of the brain and spinal cord, leading to progressive paralysis and death. Interestingly, many skin changes have been reported in ALS patients, but never as yet fully explained. These observations could be due to the common embryonic origin of the skin and neural tissue known as the ectodermal germ layer. Following the first observation in ALS patients' skin by Dr Charcot in the 19th century, in the absence of bedsores unlike other bedridden patients, other morphological and molecular changes have been observed...
March 25, 2017: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/28343013/inflammatory-muscle-disease-a-new-landscape
#3
Alain Meyer, Béatrice Lannes, Joëlle Goetz, Andoni Echaniz-Laguna, Dan Lipsker, Laurent Arnaud, Thierry Martin, Jacques Eric Gottenberg, Bernard Geny, Jean Sibilia
Greater accuracy in clinical descriptions combined with advances in muscle histology and immunology have established that inflammatory muscle diseases (IMDs) resemble inflammatory joint diseases in that they constitute a highly heterogeneous group of conditions. The topographic distribution, severity, and tempo of onset vary widely, and the histological findings distinguish at least five different profiles, which may reflect different pathophysiological processes. Most IMDs are connective tissue diseases that can affect multiple organs, among which the most common targets are the skin, joints, and lungs...
March 22, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28342637/first-line-rituximab-combined-with-short-term-prednisone-versus-prednisone-alone-for-the-treatment-of-pemphigus-ritux-3-a-prospective-multicentre-parallel-group-open-label-randomised-trial
#4
Pascal Joly, Maud Maho-Vaillant, Catherine Prost-Squarcioni, Vivien Hebert, Estelle Houivet, Sébastien Calbo, Frédérique Caillot, Marie Laure Golinski, Bruno Labeille, Catherine Picard-Dahan, Carle Paul, Marie-Aleth Richard, Jean David Bouaziz, Sophie Duvert-Lehembre, Philippe Bernard, Frederic Caux, Marina Alexandre, Saskia Ingen-Housz-Oro, Pierre Vabres, Emmanuel Delaporte, Gaelle Quereux, Alain Dupuy, Sebastien Debarbieux, Martine Avenel-Audran, Michel D'Incan, Christophe Bedane, Nathalie Bénéton, Denis Jullien, Nicolas Dupin, Laurent Misery, Laurent Machet, Marie Beylot-Barry, Olivier Dereure, Bruno Sassolas, Thomas Vermeulin, Jacques Benichou, Philippe Musette
BACKGROUND: High doses of corticosteroids are considered the standard treatment for pemphigus. Because long-term corticosteroid treatment can cause severe and even life-threatening side-effects in patients with this disease, we assessed whether first-line use of rituximab as adjuvant therapy could improve the proportion of patients achieving complete remission off-therapy, compared with corticosteroid treatment alone, while decreasing treatment side-effects of corticosteroids. METHODS: We did a prospective, multicentre, parallel-group, open-label, randomised trial in 25 dermatology hospital departments in France (Ritux 3)...
March 22, 2017: Lancet
https://www.readbyqxmd.com/read/28342387/isolation-and-characterization-of-a-novel-putative-human-polyomavirus
#5
Tarik Gheit, Sankhadeep Dutta, Javier Oliver, Alexis Robitaille, Shalaka Hampras, Jean-Damien Combes, Sandrine McKay-Chopin, Florence Le Calvez-Kelm, Neil Fenske, Basil Cherpelis, Anna R Giuliano, Silvia Franceschi, James McKay, Dana E Rollison, Massimo Tommasino
The small double-stranded DNA polyomaviruses (PyVs) form a family of 73 species, whose natural hosts are primarily mammals and birds. So far, 13 PyVs have been isolated in humans, and some of them have clearly been associated with several diseases, including cancer. In this study, we describe the isolation of a novel PyV in human skin using a sensitive degenerate PCR protocol combined with next-generation sequencing. The new virus, named Lyon IARC PyV (LIPyV), has a circular genome of 5269 nucleotides. Phylogenetic analyses showed that LIPyV is related to the raccoon PyV identified in neuroglial tumours in free-ranging raccoons...
March 22, 2017: Virology
https://www.readbyqxmd.com/read/28340341/tridimensional-visualization-and-analysis-of-early-human-development
#6
Morgane Belle, David Godefroy, Gérard Couly, Samuel A Malone, Francis Collier, Paolo Giacobini, Alain Chédotal
Generating a precise cellular and molecular cartography of the human embryo is essential to our understanding of the mechanisms of organogenesis in normal and pathological conditions. Here, we have combined whole-mount immunostaining, 3DISCO clearing, and light-sheet imaging to start building a 3D cellular map of the human development during the first trimester of gestation. We provide high-resolution 3D images of the developing peripheral nervous, muscular, vascular, cardiopulmonary, and urogenital systems...
March 23, 2017: Cell
https://www.readbyqxmd.com/read/28340187/biofilm-adherence-and-hydrophobicity-as-virulence-factors-in-malassezia-furfur
#7
Letizia Angiolella, Claudia Leone, Florencia Rojas, Javier Mussin, María de Los Angeles Sosa, Gustavo Giusiano
Malassezia species are natural inhabitants of the healthy skin. However, under certain conditions, they may cause or exacerbate several skin diseases. The ability of this fungus to colonize or infect is determined by complex interactions between the fungal cell and its virulence factors. This study aims to evaluate "in vitro" the hydrophobicity levels, the adherence on a plastic surface and the biofilm formation of 16 clinical isolates of Malassezia furfur. Cellular surface hydrophobicity (CSH) levels were determined by two-phase system...
March 9, 2017: Medical Mycology: Official Publication of the International Society for Human and Animal Mycology
https://www.readbyqxmd.com/read/28340090/predictive-value-of-european-scleroderma-group-activity-index-in-an-early-scleroderma-cohort
#8
Tatiana Nevskaya, Murray Baron, Janet E Pope
Objective.: To estimate the effect of disease activity, as measured by the European Scleroderma Research Group Activity Index (EScSG-AI), on the risk of subsequent organ damage in a large systemic sclerosis (SSc) cohort. Methods.: Of 421 SSc patients from the Canadian Scleroderma Research Group database with disease duration of ⩽ 3 years, 197 who had no evidence of end-stage organ damage initially and available 3 year follow-up were included. Disease activity was assessed by the EScSG-AI with two variability measures: the adjusted mean EScSG-AI (the area under the curve of the EScSG-AI over the observation period) and persistently active disease/flare...
March 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/28339994/a-longitudinal-cohort-study-of-the-anti-synthetase-syndrome-increased-severity-of-interstitial-lung-disease-in-black-patients-and-patients-with-anti-pl7-and-anti-pl12-autoantibodies
#9
Iago Pinal-Fernandez, Maria Casal-Dominguez, Julio A Huapaya, Jemima Albayda, Julie J Paik, Cheilonda Johnson, Leann Silhan, Lisa Christopher-Stine, Andrew L Mammen, Sonye K Danoff
Objective.: The aim was to study the prevalence, rate of appearance and severity of clinical features in patients with different anti-synthetase syndrome (ASyS) autoantibodies. Methods.: All Johns Hopkins Myositis Longitudinal Cohort subjects positive for any ASyS autoantibodies were included. Clinical information, including symptoms, signs, strength, creatine kinase concentrations and pulmonary function tests, were prospectively collected. The standardized mortality and cancer rates and the rate of appearance and intensity of the different organ manifestations were assessed using univariate and multivariate analysis and compared between ASyS autoantibodies...
March 4, 2017: Rheumatology
https://www.readbyqxmd.com/read/28339785/a-food-debris-like-component-in-the-urine-sediment-from-a-urostomy-pouch
#10
Michiko Yamashita, Masaki Hotta, Kiyoshi Yoshino, Yoh Hidaka, Tadashi Kimura
Microscopic examination of urine sediment is a basic, common method of detecting diseases of the urinary tract. We experienced a case involving a patient who had developed a fever after undergoing a urinary-diversion operation. Results of the microscopic examination of the urine collected from the urostomy pouch of the patient showed a food debris-like component. Based on this finding, we suspected a fistula between the urinary and intestinal tracts. However, after performing an experiment to verify the results, we determined that no fistula was present...
March 2, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/28339447/chronic-localized-back-pain-due-to-posterior-cutaneous-nerve-entrapment-syndrome-pocnes-a-new-diagnosis
#11
Oliver B Boelens, Robert C Maatman, Marc R Scheltinga, Kees van Laarhoven, Rudi M Roumen
Most patients with chronic back pain suffer from degenerative thoracolumbovertebral disease. However, the following case illustrates that a localized peripheral nerve entrapment must be considered in the differential diagnosis of chronic back pain. We report the case of a 26-year-old woman with continuous excruciating pain in the lower back area. Previous treatment for nephroptosis was to no avail. On physical examination the pain was present in a 2 x 2 cm area overlying the twelfth rib some 4 cm lateral to the spinal process...
March 2017: Pain Physician
https://www.readbyqxmd.com/read/28339128/life-course-of-atopy-and-allergy-related-disease-events-in-tropical-sub-saharan-africa-a-birth-cohort-study
#12
Swaib A Lule, Harriet Mpairwe, Margaret Nampijja, Florence Akello, Joyce Kabagenyi, Benigna Namara, Gyaviira Nkurunungi, Dennison Kizito, Joseph Kahwa, Lawrence Muhangi, Stephen Nash, Moses Muwanga, Emily L Webb, Alison M Elliott
BACKGROUND: In high-income countries, allergy related-diseases (ARDs) follow a typical sequence, the "Atopic March". Little is known about the life-course of ARDs in the markedly different, low-income, tropical environment. We describe ARDs in a tropical, African birth cohort. METHODS: Ugandan children were followed from birth to nine years. ISAAC questionnaires were completed at intervals; doctor-diagnosed ARDs were recorded throughout follow-up. Skin prick tests (SPTs) were done at three and nine years...
March 24, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28338755/red-blotch-disease-alters-grape-berry-development-and-metabolism-by-interfering-with-the-transcriptional-and-hormonal-regulation-of-ripening
#13
Barbara Blanco-Ulate, Helene Hopfer, Rosa Figueroa-Balderas, Zirou Ye, Rosa M Rivero, Alfonso Albacete, Francisco Pérez-Alfocea, Renata Koyama, Michael M Anderson, Rhonda J Smith, Susan E Ebeler, Dario Cantu
Grapevine red blotch-associated virus (GRBaV) is a major threat to the wine industry in the USA. GRBaV infections (aka red blotch disease) compromise crop yield and berry chemical composition, affecting the flavor and aroma properties of must and wine. In this study, we combined genome-wide transcriptional profiling with targeted metabolite analyses and biochemical assays to characterize the impact of the disease on red-skinned berry ripening and metabolism. Using naturally infected berries collected from two vineyards, we were able to identify consistent berry responses to GRBaV across different environmental and cultural conditions...
January 27, 2017: Journal of Experimental Botany
https://www.readbyqxmd.com/read/28338588/-vertical-midface-lifting-with-periorbital-anchoring-in-the-management-of-lower-eyelid-retraction-a-ten-year-clinical-retrospective-study
#14
Michele Pascali, Chiara Botti, Valerio Cervelli, Giovanni Botti
BACKGROUND: Lower eyelid retraction can be the unfortunate result of aesthetic surgery, trauma, disease or simply aging processes. The purpose of this article is to assess whether midface lifting based on purely vertical repositioning constitutes an effective procedure for its correction. METHODS: A retrospective study was carried out on 199 patients (311 eyelids) operated on between January 2004 and January 2014. The various causes of eyelid retraction in this population comprised cosmetic blepharoplasty (56...
March 3, 2017: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28338219/human-orf-complicated-by-epidermolysis-bullosa-acquisita
#15
E Zuelgaray, C Salle de Chou, J Gottlieb, M Battistella, M D Vignon-Pennamen, M Bagot, F Guibal, J D Bouaziz
Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after Orf infection including erythema multiforme. A few cases of auto-immune bullous dermatosis complicating Orf disease have been reported to date, usually characterized by tense blisters eruptions with or without mucosal involvement, linear deposition of C3, IgG and/or IgA along the basement membrane and negativity of indirect immunofluorescence analysis and ELISA assays (performed in 4 of 11 reported cases) against target antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin-332 antibodies...
March 24, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28338216/the-diversified-function-and-potential-therapy-of-ectopic-olfactory-receptors-in-non-olfactory-tissues
#16
REVIEW
Zhe Chen, Hong Zhao, Nian Fu, Linxi Chen
Olfactory receptors (ORs) are mainly distributed in olfactory neurons and play a key role in detecting volatile odorants, eventually resulting in the production of smell perception. Recently, it is also reported that ORs are expressed in non-olfactory tissues including heart, lung, sperm, skin and cancerous tissues. Interestingly, ectopic ORs are associated with the development of diseases in non-olfactory tissues. For instance, ectopic ORs initiate the hypoxic ventilatory responses and maintain the oxygen homeostasis of breathing in the carotid body when oxygen levels decline...
March 24, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28338081/a-distinct-bacterial-dysbiosis-associated-skin-inflammation-in-ovine-footrot
#17
Grazieli Maboni, Adam Blanchard, Sara Frosth, Ceri Stewart, Richard Emes, Sabine Tötemeyer
Ovine footrot is a highly prevalent bacterial disease caused by Dichelobacter nodosus and characterised by the separation of the hoof horn from the underlying skin. The role of innate immune molecules and other bacterial communities in the development of footrot lesions remains unclear. This study shows a significant association between the high expression of IL1β and high D. nodosus load in footrot samples. Investigation of the microbial population identified distinct bacterial populations in the different disease stages and also depending on the level of inflammation...
March 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28337860/clinical-features-and-long-term-outcomes-of-105-granulomatosis-with-polyangiitis-patients-a-single-center-experience-from-north-india
#18
Aman Sharma, Godasi S R S N K Naidu, Manish Rathi, Roshan Verma, Manish Modi, Benzeeta Pinto, Kusum Sharma, Varun Dhir, Manphool Singhal, Mahesh Prakash, Ritambhra Nada, Naresh K Panda, Ranjana W Minz
AIM: To describe the clinical features, treatment and long-term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA). METHODS: Clinical details, Birmingham Vasculitis Activity Score (BVAS-v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories...
March 24, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28337853/long-term-efficacy-and-tolerability-of-mycophenolate-mofetil-therapy-in-diffuse-scleroderma-skin-disease
#19
Daniel Boulos, Gene-Siew Ngian, Anton Rajadurai, Kathleen Elford, Wendy Stevens, Susanna Proudman, Claire Owen, Janet Roddy, Mandana Nikpour, Peter Youssef, Catherine Hill, Joanne Sahhar
OBJECTIVES: To assess the long-term efficacy and tolerability of mycophenolate mofetil (MMF) in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: Patients enrolled in the Australian Scleroderma Cohort study with dcSSc and baseline modified Rodnan skin score (mRSS) ≥ 12 who were treated for a minimum of 12 months with MMF for the primary indication of skin disease were included and their prospectively collected data retrieved. Change in mRSS, the proportion with a clinically significant improvement (reduction in mRSS ≥ 5 from baseline) and adverse effects due to therapy were determined...
March 24, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28337469/a-case-of-autoinflammatory-skin-and-bone-disease-flared-by-a-change-in-osteoporosis-management
#20
Larry K Heard, Vanessa N Richardson, Caroline M Lewis, Loretta S Davis
No abstract text is available yet for this article.
March 2017: JAAD Case Reports
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