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Allogeneic ABCB5-positive Stem Cells For Treatment Of Epidermolysis Bullosa PubMed articles on BioPortfolio. Our PubMed references draw on over 21 million records from the medical literature. Here you can see the latest Allogeneic ABCB5-positive Stem Cells For Treatment Of Epidermolysis Bullosa articles that have been published worldwide.
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Recessive dystrophic epidermolysis bullosa (RDEB) is a hereditary blistering disorder due to lack of type VII collagen (C7). At present, treatment is mainly supportive.
Defining markers of different phenotypic states in melanoma is important for understanding disease progression, determining the response to therapy, and defining the molecular mechanisms underpinning phenotype-switching driven by the changing intratumor microenvironment. The ABCB5 transporter is implicated in drug-resistance, and has been identified as a marker of melanoma-initiating cells. Indeed ongoing studies are using ABCB5 to define stem cell populations. However, we show here that the ABCB5 is a dire...
Epidermolysis bullosa pruriginosa (EBP; MIM#604129) is a rare clinical subtype of autosomal dominant (or less commonly recessive) dystrophic epidermolysis bullosa (DEB). In addition to usual manifestations of DEB including trauma-induced skin fragility, milia and nail dystrophy, EBP features severe pruritus, prurigo nodularis (PN) and lichen simplex chronicus-like lesions which may resemble other dermatoses and thereby complicate clinical diagnosis. Like other types of DEB, EBP results from heterozygous or ...
Inherited epidermolysis bullosa (EB) consists of different genetically heterogeneous skin and mucosal disorders due to a number of defects in the synthesis of proteins involved in the adhesion of the epithelium to the connective tissues; the oral cavity is commonly involved but there are very few published data about potential treatment..
Epidermolysis bullosa with pyloric atresia (EB-PA) is a rare, autosomal recessive form of epidermolysis bullosa characterized by mucocutaneous fragility, intestinal obstruction, and frequent urologic and renal abnormalities. The clinical course is typically lethal in the first few weeks of life, with an overall mortality of nearly 80%. Mutations in ITGB4, ITGA6, and PLEC1, which encode hemidesmosome components β4 integrin, α6 integrin, and plectin, respectively, are most commonly implicated.
Impaired growth and anaemia are major extracutaneous complications of epidermolysis bullosa (EB), but data on their development are lacking.
Chronic pruritus causes major morbidity in epidermolysis bullosa (EB). The substance P-neurokinin 1 receptor (SP-NK1) pathway is a promising target for treating EB-related pruritus.
A spectrum of skin disease severity exists in patients with recessive dystrophic epidermolysis bullosa (RDEB).
Inherited epidermolysis bullosa is a skin fragility disorder typified by blister formation following minor trauma. Four major EB types are distinguished based on the level of cleavage within the skin. Among these, epidermolysis bullosa simplex (EBS) is characterized by blister formation within the basal epidermis. EBS is the most heterogeneous EB type with mutations in seven different genes and a spectrum of clinical manifestations, ranging from widespread life-threatening skin and mucosal involvement to mi...
Dystrophic epidermolysis bullosa is a major form of epidermolysis bullosa and may be inherited as an autosomal dominant or recessive trait, with associated mutations in the COL7A1 gene. Here, we describe a consanguineous Pakistani family with four affected individuals suffering from recessive dystrophic epidermolysis bullosa. Exome sequencing of the proband's DNA revealed a homozygous missense variant (c.8038G>A:p.Gly2680Ser) in COL7A1 which cosegregated with disease in the family. The emergence of this par...
Epidermolysis bullosa (EB) comprises a group of hereditary disorders characterized by mechanical fragility of the skin and mucous membranes, with the development of blisters and vesicles in response to minimum tissue friction. Recessive dystrophic epidermolysis bullosa (RDEB) with generalized involvement is the most common subtype in the oral cavity. The present study was carried out to investigate dental implant survival, peri-implant tissue condition, patient satisfaction, and the impact of treatment upon...
Epidermolysis Bullosa (EB) is a group of genetic conditions resulting in skin and mucosal membrane fragility. EB is characterised by chronic wounds and scarring, consequent functional limitations and high levels of pain. In its most severe forms, life expectancy is significantly foreshortened.
To describe a case of epidermolysis bullosa occurring in a children's hospital in the South of Brazil, evidencing the nursing care provided to this newborn diagnosed with this pathology.
To carry out genetic testing and prenatal diagnosis for a family affected with recessive dystrophic epidermolysis bullosa (RDEB).
Epidermolysis bullosa (EB) is a rare inherited blistering disease, with an incidence of 19.6 cases per million live births . The term inherited EB encompasses multiple heterogenous genodermatoses, grouped by a shared fragility of epithelial lined tissues, particularly the skin . The pathophysiology of EB is well understood. However, there is no cure; management is supportive. Protective dressings are used to promote blister healing and avoid secondary infection.
Next-generation sequencing (NGS) is helpful in diagnosing complex genetic disorders and phenotypes, particularly when more than one overlapping condition is present. From a large cohort of 362 families with clinical manifestations of skin and mucosal fragility, referred by several major medical centers, one patient was found by NGS to have two overlapping heritable skin diseases, recessive dystrophic epidermolysis bullosa (RDEB; COL7A1 mutations) and acrodermatitis enteropathica (AE; SLC39A4 mutations). The...
Dystrophic epidermolysis bullosa (DEB) is a skin blistering disease caused by mutations in the COL7A1 gene encoding the anchoring fibril-constituent collagen VII. Secondary to skin fragility, DEB manifests as chronic wounds and progressive soft tissue fibrosis. As a consequence of a chronically injured and stiffened dermal microenvironment people with severe DEB are prone to develop early-onset metastatic cutaneous squamous cell carcinomas (cSCCs). Dermal fibrosis in DEB is paradigmatic of injury- and infla...
To evaluate the efficacy of a biocellulose, a carboxymethyl cellulose and a normal saline wound dressing in the wound care management of epidermolysis bullosa (EB) skin wounds.
Epidermolysis bullosa simplex (EBS) is a genetic skin disorder characterized by mechanical fragility and blistering of the skin. Localized EBS (EBS-loc: OMIM 131800) is the mildest variant, with blistering occurring only in the hands and feet. EBS is mainly caused by autosomal dominant mutations in the KRT5 or KRT14 gene, encoding keratin 5 (K5) or keratin 14 (K14), respectively. Here, we report a patient with EBS-loc harboring a novel mutation in KRT5, highlighting that changes affecting the charge of amin...
Single and repeated intra-articular injections in the tarsocrural joint with allogeneic and autologous equine bone marrow-derived mesenchymal stem cells are safe, but did not reduce acute inflammation in an experimental interleukin-1β model of synovitis.
Allogeneic and autologous bone marrow-derived mesenchymal stem cells (BMDMSCs) have been administered in equine joints for their anti-inflammatory effects. However, allogeneic BMDMSC offer multiple clinical and practical advantages. Therefore, it is important to determine the relative effectiveness of allogeneic versus autologous BMDMSCs.
Autologous chimeric antigen receptor (CAR) T cells have changed the therapeutic landscape in haematological malignancies. Nevertheless, the use of allogeneic CAR T cells from donors has many potential advantages over autologous approaches, such as the immediate availability of cryopreserved batches for patient treatment, possible standardization of the CAR-T cell product, time for multiple cell modifications, redosing or combination of CAR T cells directed against different targets, and decreased cost using...