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Skin fragility

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https://www.readbyqxmd.com/read/28728858/-dress-complicated-by-hemophagocytic-lymphohistiocytosis-in-an-infant-treated-for-congenital-toxoplasmosis
#1
S Rioualen, J Dufau, C Flatres, P Lavenant, L Misery, J-M Roué
BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe disease that may be complicated by hemophagocytic lymphohistiocytosis but this is rarely described in children. PATIENTS AND METHODS: We report the case of a 5-week old infant hospitalized in a pediatric intensive care unit for hemophagocytic lymphohistiocytosis with prolonged fever, splenomegaly, cytopenia, fibrinogen≤1.5g/L, ferritin≥500μg/L, and soluble IL-2 receptor≥2400U/mL...
July 17, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28726572/successful-forearm-prosthesis-fitting-in-a-patient-with-epidermolysis-bullosa-dystrophica-case-report
#2
Carl Verduyn, Marie-Anne Morren, Marguerite Stas, Friedl Sinnaeve, Katarina Segers, Carlotte Kiekens
BACKGROUND: Epidermolysis bullosa dystrophica is a rare dermatological disease characterized by extreme skin fragility and elevated risk of developing a squamous cell carcinoma. In some cases, amputation of a limb is necessary. Case description and methods: A 37-year-old man with recessive, severe generalized epidermolysis bullosa dystrophica developed a squamous cell carcinoma on the right forearm requiring a below-elbow amputation. Preoperative advice concerning indication and level of amputation was given...
July 1, 2017: Prosthetics and Orthotics International
https://www.readbyqxmd.com/read/28719827/mixed-evidence-for-the-potential-of-non-invasive-transcutaneous-vagal-nerve-stimulation-to-improve-the-extinction-and-retention-of-fear
#3
A M Burger, B Verkuil, H Fenlon, L Thijs, L Cools, H C Miller, B Vervliet, I Van Diest
Extinction memories are fragile and their formation has been proposed to partially rely on vagus nerve activity. We tested whether stimulating the auricular branch of the vagus (transcutaneous VNS; tVNS) accelerates extinction and reduces spontaneous recovery of fear. Forty-two healthy students participated in a 3-day fear conditioning study, where we tested fear acquisition (day 1), fear extinction (day 2) and the retention of the extinction memory (day 3). During extinction, participants were randomly allocated to receive tVNS or sham stimulation concurrently with each CS presentation...
July 8, 2017: Behaviour Research and Therapy
https://www.readbyqxmd.com/read/28714284/egg-albumen-as-a-fast-and-strong-medical-adhesive-glue
#4
Kaige Xu, Yuqing Liu, Shousan Bu, Tianyi Wu, Qiang Chang, Gurankit Singh, Xiaojian Cao, Chuang Deng, Bingyun Li, Gaoxing Luo, Malcolm Xing
Sutures penetrate tissues to close wounds. This process leads to inflammatory responses, prolongs healing time, and increases operation complexity. It becomes even worse when sutures are applied to stress-sensitive and fragile tissues. By bonding tissues via forming covalent bonds, some medical adhesives are not convenient to be used by surgeons and have side effects to the tissues. Here egg albumen adhesive (EAA) is reported with ultrahigh adhesive strength to bond various types of materials and can be easily used without any chemical and physical modifications...
July 17, 2017: Advanced Healthcare Materials
https://www.readbyqxmd.com/read/28693674/the-imperative-for-regenerative-agriculture
#5
Christopher J Rhodes
A review is made of the current state of agriculture, emphasising issues of soil erosion and dependence on fossil fuels, in regard to achieving food security for a relentlessly enlarging global population. Soil has been described as "the fragile, living skin of the Earth", and yet both its aliveness and fragility have all too often been ignored in the expansion of agriculture across the face of the globe. Since it is a pivotal component in a global nexus of soil-water-air-energy, how we treat the soil can impact massively on climate change - with either beneficial or detrimental consequences, depending on whether the soil is preserved or degraded...
March 1, 2017: Science Progress
https://www.readbyqxmd.com/read/28690212/focal-adhesions-in-the-skin-lessons-learned-from-skin-fragility-disorders
#6
Cristina Has, Yinghong He
Focal adhesions are large multiprotein cell-matrix adhesion complexes, which regulate multiple cellular functions, such as adhesion and migration. Their biological significance in skin is underscored by two genetic disorders, the Kindler syndrome and the interstitial lung disease, nephrotic syndrome and epidermolysis bullosa, in which mutations affect focal adhesion proteins, kindlin-1 and the integrin α3 subunit, respectively. Here we provide an overview of what we learned from the study of the molecular mechanisms of these diseases...
June 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28672133/unstable-fragility-fractures-of-the-ankle-in-the-elderly-transarticular-steinmann-pin-or-external-fixation
#7
R P J Meijer, J A Halm, T Schepers
INTRODUCTION: Because of poor skin conditions and comorbidity, open reduction and internal fixation in ankle fractures is frequently contra-indicated in the elderly. This study reports the results of two temporary fixation types in fragility fractures in the older patient: transarticular Steinmann pin fixation and external fixation. METHODS: Patients aged over 60 treated with a Steinmann pin or external fixation were retrospectively included. Patient, fracture and treatment characteristics were collected...
April 27, 2017: Foot
https://www.readbyqxmd.com/read/28652794/epidemiology-of-fragile-skin-internet-based-surveys-in-mexico-and-russia
#8
Jorge Ocampo-Candiani, Nikolay Murashkin, Asmaa Zkik, Markéta Saint Aroman, Pierre Wolkenstein
BACKGROUND: Fragile skin is a poorly understood skin condition, particularly in the general adult population. There are currently limited epidemiological data on the prevalence of fragile skin in adults. The objectives of this study were to assess the prevalence of perceived fragile skin across different skin types in representative samples of the general adult populations in Mexico and Russia, and to identify skin characteristics associated with perceived fragile skin. METHODS: Two identical cross-sectional surveys, using a short online self-administered questionnaire, were conducted on samples of recruited individuals that were representative of the general Mexican and Russian populations...
2017: Clinical, Cosmetic and Investigational Dermatology
https://www.readbyqxmd.com/read/28647894/keratin-gene-mutations-influence-the-keratinocyte-response-to-dna-damage-and-cytokine-induced-apoptosis
#9
Tina Zupancic, Gregor Sersa, Hans Törmä, Ellen Birgitte Lane, Harald Herrmann, Radovan Komel, Mirjana Liovic
The keratin filament cytoskeleton is vital to the normal function of epithelial cells. It provides structural support and regulates different aspects of cell metabolism. Mutations in keratins 5 and 14 cause a skin fragility disorder, epidermolysis bullosa simplex (EBS). Patients with severe EBS have an increased cumulative risk for basal cell carcinoma. In this study, we tested how keratin 5 and 14 mutant EBS patient-derived keratinocytes behave in the face of two different types of stressors that are able to induce cell death: ionizing radiation and cytokines TNF-α and TRAIL...
June 24, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28641957/kindler-syndrome-complicated-by-invasive-squamous-cell-carcinoma-of-the-palate
#10
H Souldi, M Y Bajja, M Mahtar
INTRODUCTION: Kindler syndrome is a very rare, autosomal recessive genodermatosis characterized by skin fragility and photosensitivity in infancy with progressive poikiloderma. CASE REPORT: We report the case of a young woman with a history of Kindler syndrome predominantly characterized by extensive involvement of the oropharyngeal mucosa. The patient presented with an ulcerative lesion of the palate. Computed tomography and biopsy concluded on unresectable invasive squamous cell carcinoma of the hard palate...
June 19, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28640219/topical-application-of-trisodium-ascorbyl-6-palmitate-2-phosphate-actively-supplies-ascorbate-to-skin-cells-in-an-ascorbate-transporter-independent-manner
#11
Shuichi Shibuya, Ikuyo Sakaguchi, Shintaro Ito, Eiko Kato, Kenji Watanabe, Naotaka Izuo, Takahiko Shimizu
Ascorbic acid (AA) possesses multiple beneficial functions, such as regulating collagen biosynthesis and redox balance in the skin. AA derivatives have been developed to overcome this compound's high fragility and to assist with AA supplementation to the skin. However, how AA derivatives are transferred into cells and converted to AA in the skin remains unclear. In the present study, we showed that AA treatment failed to increase the cellular AA level in the presence of AA transporter inhibitors, indicating an AA transporter-dependent action...
June 22, 2017: Nutrients
https://www.readbyqxmd.com/read/28612338/nutritional-rickets-and-osteomalacia-in-the-twenty-first-century-revised-concepts-public-health-and-prevention-strategies
#12
REVIEW
Suma Uday, Wolfgang Högler
PURPOSE OF REVIEW: Nutritional rickets and osteomalacia are common in dark-skinned and migrant populations. Their global incidence is rising due to changing population demographics, failing prevention policies and missing implementation strategies. The calcium deprivation spectrum has hypocalcaemic (seizures, tetany and dilated cardiomyopathy) and late hypophosphataemic (rickets, osteomalacia and muscle weakness) complications. This article reviews sustainable prevention strategies and identifies areas for future research...
June 13, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28611842/betulin-based-oleogel-to-improve-wound-healing-in-dystrophic-epidermolysis-bullosa-a-prospective-controlled-proof-of-concept-study
#13
Agnes Schwieger-Briel, Dimitra Kiritsi, Christoph Schempp, Cristina Has, Hauke Schumann
INTRODUCTION: Skin fragility and recurrent wounds are hallmarks of hereditary epidermolysis bullosa (EB). Treatment options to accelerate wound healing are urgently needed. Oleogel-S10 contains a betulin-rich triterpene extract from birch bark. In this study, we tested the wound healing properties of topical Oleogel-S10 in patients with dystrophic EB. METHODS: We conducted an open, blindly evaluated, controlled, prospective phase II pilot trial in patients with dystrophic EB (EudraCT number 2010-019945-24)...
2017: Dermatology Research and Practice
https://www.readbyqxmd.com/read/28592938/thermal-burn-in-a-30-minute-old-newborn-report-on-the-youngest-patient-with-iatrogenic-burn-injury
#14
L Abboud, G Ghanimeh
Burns in infants are rare. The majority of neonatal burns occur in the hospital setting. The immaturity of their immune system, their fragile and thin skin, difficulties in resuscitation, engraftment paucity limited by donor sites, and long-term complications make taking care of burned newborns extremely difficult. We present the case of a newborn burned 30 minutes after his birth over a total body surface of 35%, when the hot water bottle used in the hospital accidentally burst. This is the earliest iatrogenic burn in a newborn reported to date...
March 31, 2017: Annals of Burns and Fire Disasters
https://www.readbyqxmd.com/read/28584224/erratum-ectodermal-dysplasia-skin-fragility-syndrome-with-a-new-mutation
#15
(no author information available yet)
[This corrects the article DOI: 10.4103/ijdvl.IJDVL_411_16].
July 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/28561874/a-review-of-52-pedigrees-with-epidermolysis-bullosa-simplex-identifying-ten-novel-mutations-in-krt5-and-krt14-in-australia
#16
Emma N Kim, Adam G Harris, Linda J Bingham, Wenfei Yan, John C Su, Dedee F Murrell
Epidermolysis bullosa simplex (EBS) is a rare heritable skin fragility disorder, most commonly caused by dominant mutations in KRT5 and KRT14. EBS shows clinical heterogeneity with localised, intermediate and generalised severe forms, which tend to correlate with the location and nature of the disease causing mutations. We therefore aimed to identify the KRT5 and KRT14 mutations in patients diagnosed with EBS in Australia, and explore in depth the genotype to the phenotype correlations in patients with novel variants...
May 31, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28560784/reversible-and-cachexia-associated-feline-skin-fragility-syndrome-in-three-cats
#17
Nicla Furiani, Ilaria Porcellato, Chiara Brachelente
BACKGROUND: Feline skin fragility syndrome (FSFS) is an acquired disorder characterized by altered collagen production resulting in an extremely thin and fragile skin. FSFS is associated with diseases characterized by excessive steroidal hormones that can inhibit collagen synthesis. It is also described concomitantly with severe inflammatory, infectious or neoplastic conditions where the pathogenesis remains largely unknown. OBJECTIVES: To describe three cases of FSFS in cats that become cachectic secondary to different causes without glucocorticoid involvement...
May 31, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28554884/ichthyosis-linearis-circumflexa-with-bamboo-hair-challenges-in-the-diagnosis-and-management
#18
Tanvi Dev, Marwaha Raman Kumar, Gomathy Sethuraman
A 15-year-old boy had persistent and refractory erythroderma since early childhood. His parents noticed polycyclic skin lesions and hair fragility around the age of 5 years. He was treated by a local untrained practitioner for more than 3 years without any significant improvement, and he developed weight gain, thinning of skin, muscle weakness and growth retardation. He was evaluated in 2015 and found to have iatrogenic Cushing's disease with severe skeletal complications and pituitary-adrenal-gonadal suppression, which persisted despite gradual withdrawal of steroids...
May 28, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28549954/amlexanox-enhances-premature-termination-codon-read-through-in-col7a1-and-expression-of-full-length-type-vii-collagen-potential-therapy-for-recessive-dystrophic-epidermolysis-bullosa
#19
Velina S Atanasova, Qiujie Jiang, Marco Prisco, Christina Gruber, Josefina Piñón Hofbauer, Mei Chen, Cristina Has, Leena Bruckner-Tuderman, John A McGrath, Jouni Uitto, Andrew P South
Recessive dystrophic epidermolysis bullosa (RDEB) is a rare monogenic blistering disorder caused by lack of functional type VII collagen, leading to skin fragility and subsequent trauma-induced separation of the epidermis from the underlying dermis. 46% of RDEB patients harbor at least one premature termination codon (PTC) mutation in COL7A1 and previous studies have shown that aminoglycosides are able to overcome RDEB PTC mutations by inducing "read-through" and incorporation of an amino acid at the PTC site...
May 23, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28548491/deposition-of-a-sorbent-into-a-recession-on-a-solid-support-provides-a-new-mechanically-robust-solid-phase-micro-extraction-device
#20
Justen J Poole, Jonathan J Grandy, Miao Yu, Ezel Boyaci, German A Gomez Rios, Nathaly Reyes-Garcés, Barbara Bojko, Harmen Vander Heide, Janusz Pawliszyn
To date, solid-phase microextraction (SPME) fibers used for in-vivo bio-analysis can be too fragile and flexible, which limits suitability for direct tissue sampling. As a result, these devices often require a sheathing needle to pre-puncture robust sample matrices and protect the extraction phase from mechanical damage. To address this limitation, a new SPME device is herein presented which incorporates an extraction phase recessed into the body of a solid needle. This device requires no additional support or shielding during puncture events through protective tissue...
May 26, 2017: Analytical Chemistry
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