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Showing "Gentamicin Sulfate Injectable Junctional Epidermolysis Bullosa" PubMed Articles 1–25 of 1,100+

Reproductive alternatives for patients with dystrophic epidermolysis bullosa.

Epidermolysis bullosa describes a group of skin conditions caused by mutations in genes encoding proteins related to dermal-epidermal adhesion. In the United States, 50 cases of epidermolysis bullosa per 1 million live births are estimated, 92% of which classified as simplex, 5% dystrophic, 1% junctional and 2% non-classified. Dystrophic epidermolysis bullosa is associated with autosomal, dominant and recessive inheritance. Epidermolysis bullosa causes severe psychological, economic and social impacts, and ...

Epidermolysis bullosa with pyloric atresia consistently demonstrates concurrent low intra-basal epidermal and lamina lucida cleavage planes: a survey of six cases.

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.

Natural history of growth and anaemia in children with epidermolysis bullosa: A retrospective cohort study.

Impaired growth and anaemia are major extracutaneous complications of epidermolysis bullosa (EB), but data on their development are lacking.

Treatment of epidermolysis bullosa pruriginosa-associated pruritus with dupilumab.

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 ...

Whole-exome sequencing in a consanguineous Pakistani family identifies a mutational hotspot in the COL7A1 gene, causing recessive dystrophic epidermolysis bullosa.

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...

A Phase I/II open-label trial of intravenous allogeneic mesenchymal stromal cell therapy in adults with recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is a hereditary blistering disorder due to lack of type VII collagen (C7). At present, treatment is mainly supportive.

The psychological functioning of children with Epidermolysis Bullosa (EB) and its relationship with specific aspects of disease.

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.

Nursing care of a newborn with epidermolysis bullosa: a case report.

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.

Phase 2 Trial of a Neurokinin-1 Receptor Antagonist for the Treatment of Chronic Itch in Epidermolysis Bullosa Patients: A Randomized Clinical Trial.

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.

Oral rehabilitation with dental implants in patients with recessive dystrophic epidermolysis bullosa: A retrospective study with 2-15 years of follow-up.

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...

Opposing effects of STAT3 targeting in epidermolysis bullosa.

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...

Clinical efficacy of biocellulose, carboxymethyl cellulose and normal saline dressing in epidermolysis bullosa.

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.

Amino acid charge and epidermolysis bullosa simplex severity: Genotype-phenotype correlations.

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...

Successful treatment of refractory epidermolysis bullosa acquisita with intravenous immunoglobulin and dapsone.

Epidermolysis Bullosa Pruriginosa: An Unusual Presentation of a Simplex Variant.

Epidermolysis Bullosa Simplex keratinocytes show disturbed mitochondrial positioning and activity.

EBGene trial: Patient pre-selection outcomes for the European GENEGRAFT ex vivo phase I/II gene therapy trial for recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited skin fragility disorder caused by loss-of-function mutations in COL7A1 encoding type VII collagen (C7), the major component of anchoring fibrils (AFs) that ensure dermal-epidermal adherence. From birth, RDEB patients endure lifelong skin and mucosal blistering with both local and systemic complications including aggressive metastatic squamous cell carcinomas (SCC). Currently, treatments are only symptomatic although clinical studies of novel ...

Combined anti-inflammatory and low dose antiproliferative therapy for squamous cell carcinomas in recessive dystrophic epidermolysis bullosa.

Cutaneous squamous cell carcinoma (SCC) is the most severe, life-limiting complication of recessive dystrophic epidermolysis bullosa (RDEB). In RDEB, collagen VII deficiency results in permanent cutaneous damage, favoring bacterial colonization, inflammation and scarring. Particularities of SCCs in RDEB are: early onset , multiple SCCs , frequent localization on hands and feet , rapid progression, with metastatic SCC being the main cause of death in RDEB . Driver mutations emerging as a result of apolipopro...

Genomics-based treatment in a patient with two overlapping heritable skin disorders: Epidermolysis bullosa and acrodermatitis enteropathica.

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...

Boron, a Trace Mineral, Alleviates Gentamicin-Induced Nephrotoxicity in Rats.

The present study was considered to assess the protective effects of boron (B) on gentamicin-induced oxidative stress, proinflammatory cytokines, and histopathological changes in rat kidneys. Rats were split into eight equal groups which were as follows: control (fed with low-boron diet); gentamicin group (100 mg/kg, i.p.); B, B, and B (5, 10, and 20 mg/kg B, i.p.) groups; gentamicin (100 mg/kg, i.p.) plus B, B, and B (5, 10, and 20 mg/kg B, i.p.) groups. B was given to rats 4 days before the gentamicin tre...

Foot care in Epidermolysis bullosa: Evidence-based Guideline.

To provide service providers and users with an evidence-based set of current best practice guidelines for people and their families and carers, living with Epidermolysis bullosa (EB). A systematic literature review relating to the podiatric care of patients with EB was undertaken. Search terms were used, for which the most recent articles relating to podiatric treatment were identified as early as 1979 to present day, across seven electronic search engines: Medline, Wiley online library, Google Scholar, Ath...

Base editor correction of COL7A1 in recessive dystrophic epidermolysis bullosa patient-derived fibroblasts and iPSCs.

Genome editing represents a promising strategy for therapeutic correction of COL7A1 mutations that cause recessive dystrophic epidermolysis bullosa (RDEB). DNA cleavage followed by homology-directed repair (HDR) using an exogenous template has previously been used to correct COL7A1 mutations. HDR rates can be modest and the double-strand DNA breaks that initiate HDR commonly result in accompanying undesired insertions and deletions (indels). To overcome these limitations, we applied an A•T→G•C adenine...

Human mesenchymal stromal cells engineered to express collagen VII can restore anchoring fibrils in recessive dystrophic epidermolysis bullosa skin graft chimeras.

Recessive dystrophic epidermolysis bullosa (RDEB) is a debilitating genodermatosis caused by loss-of-function mutations in COL7A1 encoding type VII collagen (C7), the main component of anchoring fibrils (AFs) at the dermal-epidermal junction (DEJ). With no curative treatments presently available, retrovirally-transduced autologous epidermal grafts and intradermal lentivirally-engineered fibroblast injections are being investigated. Alternative approaches aim to infuse allogeneic mesenchymal stromal cells (M...

Synergistic Ototoxicity of Gentamicin and Low-Dose Irradiation: Molecular Basis and Clinical Significance.

Inner ear structures may be included in the radiation fields when irradiation is used to treat patients with head and neck cancers. These patients may also have concurrent infections that require gentamicin treatment. Radiation and gentamicin are both potentially ototoxic, and their combined use has been shown to result in synergistic ototoxicity in animals.

Efficient and Robust Highly Branched Poly(β-Amino Ester)/Minicircle COL7A1 Polymeric Nanoparticles for Gene delivery to Recessive Dystrophic Epidermolysis Bullosa Keratinocytes.

Recessive dystrophic epidermolysis bullosa (RDEB) is a severe congenital skin fragility disease caused by COL7A1 mutations that result in type VII collagen (C7) deficiency. Herein, we report a synergistic polyplex system that can efficiently restore C7 expression in RDEB keratinocytes. A highly branched multifunctional poly(β-amino ester) (HPAE), termed as HC32-122, was optimized systematically as the high-performance gene delivery vector for keratinocytes, achieving much higher transfection capability tha...

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