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https://www.readbyqxmd.com/read/29777447/integra-%C3%A2-dermal-regeneration-template-and-split-thickness-skin-grafting-a-therapy-approach-to-correct-aplasia-cutis-congenita-and-epidermolysis-bullosa-in-carmi-syndrome
#1
Julian Trah, Christina Has, Ingrid Hausser, Heinz Kutzner, Konrad Reinshagen, Ingo Königs
The association of junctional epidermolysis bullosa with pyloric atresia (JEB-PA) and aplasia cutis congenita (ACC) was described by El Shafie et al. (J Pediatr Surg 14(4):446-449, 1979) and Carmi et al. (Am J Med Genet 11:319-328, 1982). Most patients die in the first weeks of life, and no curative treatment options are available so far. We describe a patient with JEB-PA and ACC (OMIM # 226730) who was treated for extensive areas of ACC by Integra® -Dermal Regeneration Template and split-thickness skin grafting (STSG)...
May 18, 2018: Dermatology and Therapy
https://www.readbyqxmd.com/read/29775550/palliative-sedation-with-oral-medicines-in-an-infant-with-generalized-severe-junctional-epidermolysis-bullosa
#2
Keith P Pasichow, Meg Frizzola, Elissa G Miller
Generalized severe junctional epidermolysis bullosa is a rare mechanobullous skin disorder that is uniformly fatal. We present the case of an infant who received palliative pain management and ultimately proportionate palliative sedation. However, because of the extent of the patient's skin disease, we were unable to provide palliative medication through parenteral routes. We discuss the provision of enteral palliative sedation, including the pharmacology, and creative use of medications to achieve sufficient palliation in this difficult and unique situation...
May 18, 2018: Journal of Palliative Medicine
https://www.readbyqxmd.com/read/29770468/toll-like-receptor-signalling-induces-the-expression-of-serum-amyloid-a-in-epidermal-keratinocytes-and-dermal-fibroblasts
#3
S Morizane, A Kajita, K Mizuno, T Takiguchi, K Iwatsuki
BACKGROUND: Toll-like receptors (TLRs) play critical roles in innate immune response by sensing pathogen- or damage-associated molecular patterns. Epidermal keratinocytes and dermal fibroblasts also produce proinflammatory cytokines and chemokines under stimulation with TLR ligands. Serum amyloid A (SAA) is an essential factor in the pathogenesis of secondary amyloidosis, and also has immunomodulatory functions. SAA are produced mainly by hepatocytes but also by a variety of cells, including immune cells, endothelial cells, synoviocytes, and epidermal keratinocytes...
May 16, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29758284/intradermal-injection-of-bone-marrow-msc-corrects-recessive-dystrophic-epidermolysis-bullosa-in-a-xenograft-model
#4
Clarisse Ganier, Matthias Titeux, Sonia Gaucher, Juliette Peltzer, Marc Le Lorc'h, Jean-Jacques Lataillade, Akemi Ishida-Yamamoto, Alain Hovnanian
No abstract text is available yet for this article.
May 11, 2018: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/29756957/nutritional-outcomes-in-children-with-epidermolysis-bullosa-long-term-follow-up
#5
Ana Paula Caio Zidório, Dryelle Oliveira Dias Leão, Kenia Mara Baiocchi De Carvalho, Eliane Said Dutra
BACKGROUND: some types of epidermolysis bullosa (EB) have extracutaneous manifestations. Manifestations that limit food intake and absorption may compromise nutritional status and increasing nutritional requirements. OBJECTIVES: to investigate the following nutritional status indicators: exclusive breastfeeding duration, problems caused by the introduction of complementary foods, birth weight and length, and growth curves of children with EB. METHODS: assessment was based on the World Health Organization (WHO) growth charts...
January 19, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/29754879/congenital-pyloric-atresia-nine-new-cases-single-center-experience-of-the-long-term-follow-up-and-the-lessons-learnt-over-a-decade
#6
Monal Kansra, V Shankar Raman, Kamal Kishore, Sanat Khanna, Bipin Puri, Abhishek Sharma
BACKGROUND: Congenital pyloric atresia (CPA) is a rare anomaly with an incidence of 1 in 100,000 live births. Depending on the type of anomaly patients can either present in the neonatal period or later in life with subtle nonspecific signs and symptoms. We present our institute's experience in handling these cases over the last decade and highlight lessons learnt. MATERIALS AND METHODS: We retrospectively reviewed records of patients diagnosed with CPA and managed at our centre between Jan 2006 to June 2016...
April 14, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29753707/reduced-microbial-diversity-is-a-feature-of-recessive-dystrophic-epidermolysis-bullosa-involved-skin-and-wounds
#7
Ignacia Fuentes, Christina Guttmann-Gruber, Angeline Su Ling Tay, Josefina Piñón Hofbauer, Simon L I J Denil, Julia Reichelt, Francis Palisson, John E A Common, Andrew P South
No abstract text is available yet for this article.
May 10, 2018: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/29746276/deepithelialized-skin-reduction-preserves-skin-and-nipple-perfusion-in-immediate-reconstruction-of-large-and-ptotic-breasts
#8
Danielle H Rochlin, Dung H Nguyen
BACKGROUND: Women with large and/or ptotic breasts are often not considered candidates for nipple-sparing mastectomy (NSM) and/or immediate breast reconstruction given difficulties avoiding ischemic complications and achieving a favorable aesthetic result. We report a novel technique involving deepithelialized skin reduction to simultaneously reduce the skin envelope and preserve perfusion to the skin and nipple in immediate breast reconstruction for women that fit this high-risk anatomic profile...
April 30, 2018: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/29745997/efficacy-of-human-placental-derived-stem-cells-in-collagen-vii-knockout-recessive-dystrophic-epidermolysis-bullosa-animal-model
#9
Yanling Liao, Larisa Ivanova, Rajarajeswari Sivalenka, Trevor Plumer, Hongwen Zhu, Xiaokui Zhang, Angela M Christiano, John A McGrath, Jodi P Gurney, Mitchell S Cairo
Recessive dystrophic epidermolysis bullosa (RDEB) is a devastating inherited skin blistering disease caused by mutations in the COL7A1 gene that encodes type VII collagen (C7), a major structural component of anchoring fibrils at the dermal-epidermal junction (DEJ). We recently demonstrated that human cord blood-derived unrestricted somatic stem cells promote wound healing and ameliorate the blistering phenotype in a RDEB (col7a1-/- ) mouse model. Here, we demonstrate significant therapeutic effect of a further novel stem cell product in RDEB, that is, human placental-derived stem cells (HPDSCs), currently being used as human leukocyte antigen-independent donor cells with allogeneic umbilical cord blood stem cell transplantation in patients with malignant and nonmalignant diseases...
May 10, 2018: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/29722429/bart-s-syndrome-in-a-family-affected-three-consecutive-generations-with-mutation-c-6007g-a-in-col7a1
#10
Zhiming Chen, Wenbo Bu, Suying Feng, Hongsheng Wang
Bart's syndrome (BS), characterized by aplasia cutis congenita (ACC, also called congenital localized absence of skin) and epidermolysis bullosa (EB), is diagnosed clinically based on the disorder's unique signs and symptoms. We report the case of a family, three members of which presented with ACC at birth and one had blisters on the mucous membranes. The patient was treated conservatively with topical antibacterial ointment and wet gauze dressing. Periodic follow up showed complete healing with minimal scarring...
May 3, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29719822/an-81-year-old-woman-with-recalcitrant-blisters
#11
Daniel C Butler
Epidermolysis bullosa acquisita (EBA) is a rare mucocutaneous blistering disorder with typical onset in adulthood. Diagnosis and management can be difficult owing to the variability in presentation and clinical manifestation. In this case, we explore a case of EBA as well as provide a general overview of the condition and its variants.
January 2018: Dermatopathology (Basel, Switzerland)
https://www.readbyqxmd.com/read/29699768/dilated-cardiomyopathy-in-a-child-with-recessive-dystrophic-epidermolysis-bullosa
#12
A Imbernón-Moya, R Maseda-Pedrero, M Feito, R de Lucas
No abstract text is available yet for this article.
April 23, 2018: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/29689068/role-of-the-keratin-1-and-keratin-10-tails-in-the-pathogenesis-of-ichthyosis-hystrix-of-curth-macklin
#13
Alessandro Terrinoni, Biagio Didona, Sabrina Caporali, Giovanni Chillemi, Alessandro Lo Surdo, Mauro Paradisi, Margherita Annichiarico-Petruzzelli, Eleonora Candi, Sergio Bernardini, Gerry Melino
Ichthyosis Hystrix of Curth-Macklin (IH-CM) is a rare manifestation of epidermolytic ichthyosis (EI) that is characterised by generalised spiky or verrucous hyperkeratosis. The disorder is further distinguished by the presence of binucleated cells in the affected skin, whereas epidermolysis and clumping of tonofilaments, as seen in EI, are absent. While IH-CM is associated with mutations in the keratin 1 (KRT1) gene, reports to date have indicated that mutations in the KRT1 gene result in an aberrant and truncated protein tail, essentially affecting the function of the V2 domain...
2018: PloS One
https://www.readbyqxmd.com/read/29679399/inherited-epidermolysis-bullosa-new-diagnostics-and-new-clinical-phenotypes
#14
Cristina Has, Judith Fischer
Inherited epidermolysis bullosa (EB) is a group of heterogeneous genetic disorders characterized by skin fragility. EB comprises a large spectrum of phenotypes, ranging from severe cutaneous and extracutaneous involvement caused by lack of key adhesion proteins, to mild cutaneous fragility caused by subtle molecular defects. Disease-causing variants in 20 different genes account for the genetic and allelic heterogeneity of EB. Here we discuss the development of laboratory methods that enabled these discoveries and the clinical and molecular features of some new EB entities elucidated during the past 5-6 years...
April 20, 2018: Experimental Dermatology
https://www.readbyqxmd.com/read/29653141/dual-mechanism-of-type-vii-collagen-transfer-by-bone-marrow-mesenchymal-stem-cell-extracellular-vesicles-to-recessive-dystrophic-epidermolysis-bullosa-fibroblasts
#15
Jeffrey McBride, Luis Rodriguez-Menocal, Ambar Candanedo, Wellington Guzman, Marta Garcia-Contreras, Evangelos Badiavas
Recessive dystrophic epidermolysis bullosa (RDEB) is a severe blistering disease resulting from a lack of type VII collagen production. Recent clinical trials have shown efficacy of bone marrow-derived mesenchymal stem cells (BM-MSCs) in the treatment of epidermolysis bullosa, including improved basement membrane restructuring and cutaneous wound healing. The mechanism as to how type VII collagen is transferred from donor stem cell to recipient RDEB cells has not been defined. Here, we submit the model that BM-MSC-derived extracellular vesicles serve at least two roles: 1) to help transport type VII collagen within the extracellular space; and 2) to feed RDEB fibroblasts with messenger RNA that codes for type VII collagen, resulting in COL7A1 translation and synthesis of type VII collagen alpha chain proteins by RDEB fibroblasts...
April 10, 2018: Biochimie
https://www.readbyqxmd.com/read/29648915/response-to-modabber-and-harissi-dagher-s-letter-type-1-boston-keratoprosthesis-for-limbal-stem-cell-deficiency-in-epidermolysis-bullosa
#16
N Geetha Sravani, Ashik Mohamed, Virender S Sangwan
No abstract text is available yet for this article.
April 12, 2018: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/29627521/epidermolysis-bullosa-molecular-pathology-of-connective-tissue-components-in-the-cutaneous-basement-membrane-zone
#17
REVIEW
Cristina Has, Alexander Nyström, Amir Hossain Saeidian, Leena Bruckner-Tuderman, Jouni Uitto
Epidermolysis bullosa (EB), a group of heritable skin fragility disorders, is characterized by blistering, erosions and chronic ulcers in the skin and mucous membranes. In some forms, the blistering phenotype is associated with extensive mutilating scarring and development of aggressive squamous cell carcinomas. The skin findings can be associated with extracutaneous manifestations in the ocular as well as gastrointestinal and vesico-urinary tracts. The phenotypic heterogeneity reflects the presence of mutations in as many as 20 different genes expressed in the cutaneous basement membrane zone, and the types and combinations of the mutations and their consequences at the mRNA and protein levels contribute to the spectrum of severity encountered in different subtypes of EB...
April 5, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29621642/adipose-derived-stem-cells-improve-full-thickness-skin-grafts-in-a-rat-model
#18
Silvana Bellini Vidor, Paula Barros Terraciano, Fernanda Soldatelli Valente, Verônica Machado Rolim, Cristiana Palma Kuhl, Laura Silveira Ayres, Tuane Nerissa Alves Garcez, Natália Emerim Lemos, Cristiano Ely Kipper, Sabrina Beal Pizzato, David Driemeier, Elizabeth Obino Cirne-Lima, Emerson Antonio Contesini
To investigate the effects of heterologous adipose-derived stem cells (ADSCs) on autologous full-thickness skin grafts, we designed a first-intention healing model using Wistar rats. We harvested and sutured two full-thickness skin grafts in the dorsal recipient beds of 15 rats, randomized into three groups. In the treatment group, 1 × 106 ADSCs resuspended in saline solution (200 μL) were administered subcutaneously to the skin graft. The control group received only saline solution subcutaneously, whereas the negative control group did not receive any treatment...
March 28, 2018: Research in Veterinary Science
https://www.readbyqxmd.com/read/29619029/anti-type-vii-collagen-antibodies-are-identified-in-a-subpopulation-of-bullous-pemphigoid-patients-with-relapse
#19
Delphine Giusti, Grégory Gatouillat, Sébastien Le Jan, Julie Plée, Philippe Bernard, Frank Antonicelli, Bach-Nga Pham
Bullous pemphigoid (BP) is an autoimmune bullous skin disease characterized by anti-BP180 and anti-BP230 autoantibodies (AAbs). Mucous membrane involvement is an uncommon clinical feature of BP which may evoke epidermolysis bullosa acquisita, another skin autoimmune disease characterized by anti-type VII collagen AAbs. We therefore evaluated the presence of anti-type VII collagen AAbs in the serum of BP patients with and without mucosal lesions at time of diagnosis and under therapy. Anti-BP180, anti-BP230, and anti-type VII collagen AAbs were measured by ELISA in the serum of unselected patients fulfilling clinical and histo/immunopathological BP criteria at baseline ( n  = 71) and at time of relapse ( n  = 24)...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29616034/specific-inhibition-of-complement-activation-significantly-ameliorates-autoimmune-blistering-disease-in-mice
#20
Sidonia Mihai, Misa Hirose, Yi Wang, Joshua M Thurman, V Michael Holers, B Paul Morgan, Jörg Köhl, Detlef Zillikens, Ralf J Ludwig, Falk Nimmerjahn
Epidermolysis bullosa acquisita (EBA) is an antibody-mediated blistering skin disease associated with tissue-bound and circulating autoantibodies to type VII collagen (COL7). Transfer of antibodies against COL7 into mice results in a subepidermal blistering phenotype, strictly depending on the complement component C5. Further, activation predominantly by the alternative pathway is required to induce experimental EBA, as blistering was delayed and significantly ameliorated only in factor B-/- mice. However, C5 deficiency not only blocked the activation of terminal complement components and assembly of the membrane attack complex (MAC) but also eliminated the formation of C5a...
2018: Frontiers in Immunology
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