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https://www.readbyqxmd.com/read/27914124/atypical-neonatal-marfan-syndrome-with-p-glu1073lys-mutation-of-fbn1-the-first-case-in-korea
#1
Ju Sun Heo, Joo Young Song, Eun Young Choi, Eun Hee Kim, Ji Hee Kim, So Eun Park, Ji Hyun Jeon
Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna...
January 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/27913771/safety-assessment-of-methyl-glucose-polyethers-and-esters-as-used-in-cosmetics
#2
Wilbur Johnson, Bart Heldreth, Wilma F Bergfeld, Donald V Belsito, Ronald A Hill, Curtis D Klaassen, Daniel C Liebler, James G Marks, Ronald C Shank, Thomas J Slaga, Paul W Snyder, F Alan Andersen
The Cosmetic Ingredient Review Expert Panel (Panel) reviewed the safety of methyl glucose polyethers and esters which function in cosmetics as skin/hair-conditioning agents, surfactants, or viscosity increasing agents. The esters included in this assessment are mono-, di-, or tricarboxyester substituted methyl glucosides, and the polyethers are mixtures of various chain lengths. The Panel reviewed available animal and clinical data, including the molecular weights, log Kows, and other properties in making its determination of safety on these ingredients...
November 2016: International Journal of Toxicology
https://www.readbyqxmd.com/read/27903963/cdh1-regulates-e2f1-degradation-in-response-to-differentiation-signals-in-keratinocytes
#3
Randeep K Singh, Lina Dagnino
The E2F1 transcription factor plays key roles in skin homeostasis. In the epidermis, E2F1 expression is essential for normal proliferation of undifferentiated keratinocytes, regeneration after injury and DNA repair following UV radiation-induced photodamage. Abnormal E2F1 expression promotes nonmelanoma skin carcinoma. In addition, E2F1 must be downregulated for proper keratinocyte differentiation, but the relevant mechanisms involved remain poorly understood. We show that differentiation signals induce a series of post-translational modifications in E2F1 that are jointly required for its downregulation...
November 26, 2016: Oncotarget
https://www.readbyqxmd.com/read/27903823/in%C3%A2-vitro-skin-models-and-tissue-engineering-protocols-for-skin-graft-applications
#4
REVIEW
Lucas B Naves, Chetna Dhand, Luis Almeida, Lakshminarayanan Rajamani, Seeram Ramakrishna
In this review, we present a brief introduction of the skin structure, a concise compilation of skin-related disorders, and a thorough discussion of different in vitro skin models, artificial skin substitutes, skin grafts, and dermal tissue engineering protocols. The advantages of the development of in vitro skin disorder models, such as UV radiation and the prototype model, melanoma model, wound healing model, psoriasis model, and full-thickness model are also discussed. Different types of skin grafts including allografts, autografts, allogeneic, and xenogeneic are described in detail with their associated applications...
November 30, 2016: Essays in Biochemistry
https://www.readbyqxmd.com/read/27903244/cryopreservation-of-dermal-fibroblasts-and-keratinocytes-in-hydroxyethyl-starch-based-cryoprotectants
#5
Yahaira Naaldijk, Adiv A Johnson, Annett Friedrich-Stöckigt, Alexandra Stolzing
BACKGROUND: Preservation of human skin fibroblasts and keratinocytes is essential for the creation of skin tissue banks. For successful cryopreservation of cells, selection of an appropriate cryoprotectant agent (CPA) is imperative. The aim of this study was to identify CPAs that minimize toxic effects and allow for the preservation of human fibroblasts and keratinocytes in suspension and in monolayers. RESULTS: We cryopreserved human fibroblasts and keratinocytes with different CPAs and compared them to fresh, unfrozen cells...
December 1, 2016: BMC Biotechnology
https://www.readbyqxmd.com/read/27891590/bi-allelic-iars-mutations-in-a-child-with-intra-uterine-growth-retardation-neonatal-cholestasis-and-mild-developmental-delay
#6
Naama Orenstein, Karin Weiss, Stephanie N Oprescu, Rivka Shapira, Dvora Kidron, Lina Vanagaite-Basel, Anthony Antonellis, Maximilian Muenke
Recently, bi-allelic mutations in cytosolic isoleucyl-tRNA synthetase (IARS) have been described in three individuals with growth delay, hepatic dysfunction, and neurodevelopmental disabilities. Here we report an additional subject with this condition identified by whole-exome sequencing. Our findings support the association between this disorder and neonatal cholestasis with distinct liver pathology. Furthermore, we provide functional data on two novel missense substitutions and expand the phenotype to include mild developmental delay, skin hyper-elasticity, and hypervitaminosis D...
November 28, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27888034/a-proactive-approach-to-penicillin-allergy-testing-in-hospitalized-patients
#7
Justin R Chen, Scott A Tarver, Kristin S Alvarez, Trang Tran, David A Khan
BACKGROUND: Penicillin allergy testing is underutilized in inpatients despite its potential to immediately impact antibiotic treatment. Although most tested patients are able to tolerate penicillin, limited availability and awareness of this tool leads to the use of costly and harmful substitutes. OBJECTIVE: We established an inpatient service at a large academic hospital to identify and test patients with a history of penicillin allergy with the goals of removing inaccurate diagnoses, reducing the use of beta-lactam alternatives, and educating patients and clinicians about the procedure...
November 22, 2016: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/27878297/hydroxyproline-containing-collagen-peptide-derived-from-the-skin-of-the-alaska-pollack-inhibits-hiv-1-infection
#8
In Seung Jang, Sun Joo Park
The human immunodeficiency virus (HIV) is a lentivirus that results in acquired immunodeficiency syndrome (AIDS). HIV treatment involving chemical therapeutic agents has improved the quality of life of HIV/AIDS patients. The present study demonstrates that a hydroxyproline-containing marine collagen peptide (APHCP) derived from Alaska pollack inhibits HIV‑1 infection in the MT-4 human T cell‑line. APHCP inhibited HIV-1IIIB-induced cell lysis, syncytia formation, reverse transcriptase activity and viral p24 production at non‑cytotoxic concentrations; however, APHCP did not inhibit HIV‑2ROD infection in MT‑4 cells...
November 16, 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27875945/3d-printing-articular-cartilage-recapitulating-the-complexity-of-native-tissue
#9
Ting Guo, Josephine Lembong, Lijie Grace Zhang, John P Fisher
In the past few decades, the field of tissue engineering combined with rapid prototyping (RP) techniques has been successful in creating biological substitutes that mimic tissues. Its applications in regenerative medicine have drawn efforts in research from various scientific fields, diagnostics, and clinical translation to therapies. While some areas of therapeutics are well developed, such as skin replacement, many others such as cartilage repair can still greatly benefit from tissue engineering and RP due to the low success and / or inefficiency of current existing, often surgical treatments...
November 22, 2016: Tissue Engineering. Part B, Reviews
https://www.readbyqxmd.com/read/27872793/improved-methods-to-produce-tissue-engineered-skin-substitutes-suitable-for-the-permanent-closure-of-full-thickness-skin-injuries
#10
Danielle Larouche, Laurence Cantin-Warren, Maxime Desgagné, Rina Guignard, Israël Martel, Akram Ayoub, Amélie Lavoie, Robert Gauvin, François A Auger, Véronique J Moulin, Lucie Germain
There is a clinical need for skin substitutes to replace full-thickness skin loss. Our group has developed a bilayered skin substitute produced from the patient's own fibroblasts and keratinocytes referred to as Self-Assembled Skin Substitute (SASS). After cell isolation and expansion, the current time required to produce SASS is 45 days. We aimed to optimize the manufacturing process to standardize the production of SASS and to reduce production time. The new approach consisted in seeding keratinocytes on a fibroblast-derived tissue sheet before its detachment from the culture plate...
2016: BioResearch Open Access
https://www.readbyqxmd.com/read/27866883/comparison-of-contraction-among-three-dermal-substitutes-morphological-differences-in-scaffolds
#11
Keijiro Hori, Atsuyoshi Osada, Tsukasa Isago, Hiroyuki Sakurai
Various kinds of synthetic dermal substitutes are produced and used in clinical application and contribute to wound bed preparation for subsequent skin grafting. Although their appearance and outcomes are different, the criteria for the selection of proper synthetic dermal substitutes is not well defined yet. In this study, we focused on the contraction of dermal substitutes and compared the morphological differences in scaffolds. A marked contraction was observed with Pelnac(®) compared to Integra(®) and Terudermis(®) in vitro...
November 17, 2016: Burns: Journal of the International Society for Burn Injuries
https://www.readbyqxmd.com/read/27862914/in-vivo-biocompatibility-vascularization-and-incorporation-of-integra-%C3%A2-dermal-regenerative-template-and-flowable-wound-matrix
#12
Thomas Später, Florian S Frueh, Wolfgang Metzger, Michael D Menger, Matthias W Laschke
Integra(®) matrix wound dressing (MWD) is used for the reconstruction of full-thickness skin defects. For the treatment of complex wounds, this dermal substitute is available as a flowable wound matrix (FWM) of identical composition. To clarify whether variations in sample preparation and consistency affect the biocompatibility and tissue incorporation, we herein compared MWD and FWM. The matrices were characterized using scanning electron microscopy and histology. Moreover, they were implanted in mouse dorsal skinfold chambers to analyze their in vivo performance over 2 weeks...
November 14, 2016: Journal of Biomedical Materials Research. Part B, Applied Biomaterials
https://www.readbyqxmd.com/read/27861978/circulating-cla-t-cells-in-atopic-dermatitis-and-their-possible-role-as-peripheral-biomarkers
#13
REVIEW
Tali Czarnowicki, Luis F Santamaria-Babí, Emma Guttman-Yassky
Cutaneous lymphocyte-associated antigen (CLA(+) ) T-cells are specialized for skin homing and represent the main T-cell population in atopic dermatitis (AD) lesions. CLA(+) is expressed on the surface of circulating CD45RO(+) memory T-cells and most skin infiltrating T-cells. Mechanistic studies and thus treatment advancements are limited by the need of large number of skin biopsies. Circulating CLA(+) T-cells may be a reliable surrogate marker of the inflammatory events occurring in the skin, and thus the evaluation of CLA(+) T-cells in the blood may eliminate the need for skin biopsies...
November 8, 2016: Allergy
https://www.readbyqxmd.com/read/27826471/integra-based-reconstruction-of-large-scalp-wounds-a-case-report-and-systematic-review-of-the-literature
#14
Maxwell B Johnson, Alex K Wong
BACKGROUND: Large complex scalp wounds that have traditionally required free vascularized tissue transfer have been successfully reconstructed with skin substitutes such as Integra. Although there are multiple reports of Integra-based reconstructions of scalp wounds, there has not been a comprehensive assessment of this body of literature that critically examines this method. Our goal was to conduct a systematic review to determine the effectiveness of Integra-based reconstructions of scalp wounds, with emphasis on large defects...
October 2016: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/27826004/cell-infiltrative-hydrogel-fibrous-scaffolds-for-accelerated-wound-healing
#15
Xin Zhao, Xiaoming Sun, Lara Yildirimer, Qi Lang, Zhi Yuan William Lin, Reila Zheng, Yuguang Zhang, Wenguo Cui, Nasim Annabi, Ali Khademhosseini
: Development of natural protein-based fibrous scaffolds with tunable physical properties and biocompatibility is highly desirable to construct a three-dimensional (3D), fully cellularized scaffold for wound healing. Herein, we demonstrated a simple and effective technique to construct electrospun 3D fibrous scaffolds for accelerated wound healing using a photocrosslinkable hydrogel based on methacryloylated gelatin (GelMA). We found that the physical properties of the photocrosslinkable hydrogel including water retention, stiffness, strength, elasticity and degradation can be tailored by changing the light exposure time...
November 4, 2016: Acta Biomaterialia
https://www.readbyqxmd.com/read/27824929/tissue-kallikrein-inhibitors-based-on-the-sunflower-trypsin-inhibitor-scaffold-a-potential-therapeutic-intervention-for-skin-diseases
#16
Wenjie Chen, Veronica A Kinsler, Derek Macmillan, Wei-Li Di
Tissue kallikreins (KLKs), in particular KLK5, 7 and 14 are the major serine proteases in the skin responsible for skin shedding and activation of inflammatory cell signaling. In the normal skin, their activities are controlled by an endogenous protein protease inhibitor encoded by the SPINK5 gene. Loss-of-function mutations in SPINK5 leads to enhanced skin kallikrein activities and cause the skin disease Netherton Syndrome (NS). We have been developing inhibitors based on the Sunflower Trypsin Inhibitor 1 (SFTI-1) scaffold, a 14 amino acids head-to-tail bicyclic peptide with a disulfide bond...
2016: PloS One
https://www.readbyqxmd.com/read/27817210/evaluation-of-a-new-topical-skin-protectant-rd1433-for-the-prevention-and-treatment-of-incontinence-associated-dermatitis
#17
Hazem Matar, Joanne Larner, Vanessa Viegas, Sneha Kansagra, Karen L Atkinson, Satyajit Shetage, John T Skamarauskas, Baveetharan Theivendran, Virginia S Goldman, Robert P Chilcott
CONTEXT: Incontinence-associated dermatitis (IAD) is a type of moisture-associated dermatitis caused by repeated skin exposure to urine or stool. A product that could mitigate such symptoms would have a significant impact on cost of care and patients' quality of life. OBJECTIVE: This study compared the clinical efficacy of RD1433 and a comparator product (Vaseline®) in preventing and treating experimental IAD skin lesions. MATERIALS AND METHODS: For the "prevention" part of the study, skin sites in eight human volunteers were treated daily for five days with either RD1433 or Vaseline® immediately prior to synthetic urine exposure...
November 6, 2016: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/27816673/comparison-of-balb-c-and-cba-j-mice-for-the-local-lymph-node-assay-using-bromodeoxyuridine-with-flow-cytometry-llna-brdu-fcm
#18
Yong Sun Lee, Jung-Sun Yi, Souk Jin Seo, Joo Hwan Kim, Mi-Sook Jung, Im-Kwon Seo, Ilyoung Ahn, Kyungyuk Ko, Tae Sung Kim, Kyung Min Lim, Soojung Sohn
The local lymph node assay using 5-bromo-2-deoxyuridine (BrdU) with flow cytometry (LLNA: BrdU-FCM) is a modified LLNA that is used to identify skin sensitizers by counting BrdU-incorporated lymph node cells (LNCs) with flow cytometry. Unlike other LLNA methods (OECD TG 429, 442A and 442B) in which the CBA/J mouse strain is used, LLNA: BrdU-FCM was originally designed to be compatible with BALB/c, a mouse strain that is more widely used in many countries. To justify the substitution of CBA/J for BALB/c, the equivalence of the test results between two strains shall be established prior to the official implementation of LLNA: BrdU-FCM...
November 2, 2016: Regulatory Toxicology and Pharmacology: RTP
https://www.readbyqxmd.com/read/27806249/effects-of-macrolide-antibiotics-on-th1-cell-and-th2-cell-development-mediated-by-langerhans-cells
#19
Katsuhiko Matsui, Saki Tamai, Reiko Ikeda
: - Background: It is well known that Langerhans cells (LCs) work as the primary orchestrators in the polarization of the immune milieu towards a T helper type 1 (Th1) or a Th2 immune response. In this study, we investigated the effects of macrolide antibiotics on Th1 cell and Th2 cell development mediated by LCs. METHODS: LC-like dendritic cells (LDCs) were generated from mouse bone marrow cells and used as substitutes for LCs. Mice were primed with ovalbumin (OVA) peptide-pulsed LDCs, which had been treated with each macrolide antibiotic, via the hind footpad...
July 2016: Journal of Pharmacy & Pharmaceutical Sciences: a Publication of the Canadian Society for Pharmaceutical Sciences
https://www.readbyqxmd.com/read/27795427/the-glycoprotein-b-cytoplasmic-domain-lysine-cluster-is-critical-for-varicella-zoster-virus-cell-cell-fusion-regulation-and-infection
#20
Edward Yang, Ann M Arvin, Stefan L Oliver
: The conserved glycoproteins gB and gH-gL are essential for herpesvirus entry and cell-cell fusion induced syncytia formation, a characteristic of Varicella Zoster Virus (VZV) pathology in skin and sensory ganglion. VZV syncytia formation, which has been implicated in the painful condition of postherpetic neuralgia, is regulated by the cytoplasmic domains of gB (gBcyt) via an immunoreceptor tyrosine-based inhibition motif (ITIM) and gH (gHcyt). A lysine cluster (K894, K897, K898, and K900) in the VZV gBcyt was identified by sequence alignment to be conserved among alphaherpesviruses suggesting a functional role...
October 26, 2016: Journal of Virology
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