epidermolysis bullosa/albumină

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Enteral iron absorption in patients with recessive dystrophic epidermolysis bullosa

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Background/objectives: To determine whether iron was being enterally absorbed in anemic patients with recessive dystrophic epidermolysis bullosa (RDEB). Methods: Anemic

Correlates of low bone mass in children with generalized forms of epidermolysis bullosa.

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BACKGROUND Epidermolysis bullosa (EB) is a family of rare, heterogeneous, genetic disorders characterized by fragility of the skin and mucous membranes. Reduced bone mass and fractures have been recognized as complications of generalized forms of EB. OBJECTIVE We sought to describe the range and to

[A case of recessive dystrophic epidermolysis bullosa associated with dwarfism with special reference to pathophysiological role of growth hormone].

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Epidermolysis bullosa is a group of disorders whose common primary feature is the formation of blisters following trivial trauma. Recessive dystrophic epidermolysis bullosa (RDEB), a subtype of epidermolysis bullosa, is frequently associated with growth retardation. This growth retardation has been

[Surgical treatment of esophageal stenosis with recessive dystrophic epidermolysis bullosa--esophageal dilatation using balloon catheter method].

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We report the details of our treatment of 5 patients with recessive dystrophic epidermolysis bullosa (RDEB). Good results were obtained by esophageal dilatation using the balloon catheter method. Prior to the operation, we corrected the patients' nutritional status with oral supplementation of

Diffuse membranoproliferative glomerulonephritis with focal sclerosis and renal amyloidosis in an adult male with autosomal dominant dystrophic epidermolysis bullosa: a case report.

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Previous reports of glomerular disease in adult patients with autosomal dominant dystrophic epidermolysis bullosa (EB) are limited and include post-infectious glomerulonephritis, IgA nephropathy, amyloidosis, and leukocytoclastic vasculitis. To our knowledge, membranoproliferative glomerulonephritis

Biocompatibility of electrospun human albumin: a pilot study.

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Albumin is rarely used for electrospinning because it does not form fibres in its native globular form. This paper presents a novel method for electrospinning human albumin from a solution containing pharmaceutical grade protein and 25% polyethylene oxide (PEO) used as the fibre-forming agent. After

Salivary function of persons with hereditary epidermolysis bullosa.

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Oral alterations of the hard and/or soft tissues are commonly associated with the different types of epidermolysis bullosa (EB). The relationship of oral soft and hard tissue changes to the disease mechanisms in different EB types remains to be elucidated. The purpose of this investigation was to

The chemical composition of tooth enamel in junctional epidermolysis bullosa.

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The junctionalis form of epidermolysis bullosa (EBJ) is associated with a number of clinical problems involving tooth enamel, including increased susceptibility to caries. The aim here was to carry out a chemical characterization of the enamel of teeth from EBJ patients compared with that of

Vitamin and trace metal levels in recessive dystrophic epidermolysis bullosa.

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BACKGROUND In recessive dystrophic epidermolysis bullosa (RDEB), a good nutritional balance is necessary to obtain healing of the chronic wounds. However, involvement of the oral mucosa and oesophagus stenosis may be responsible for severe nutritional deficiencies. OBJECTIVE In order to propose an

A phase II randomized vehicle-controlled trial of intradermal allogeneic fibroblasts for recessive dystrophic epidermolysis bullosa.

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BACKGROUND Chronic wounds are a major source of morbidity and mortality in generalized severe recessive dystrophic epidermolysis bullosa (RDEB-GS). OBJECTIVE This was a phase II double-blinded randomized controlled trial of intralesional allogeneic cultured fibroblasts in suspension solution versus

Immunogenicity of decidual stromal cells in an epidermolysis bullosa patient and in allogeneic hematopoietic stem cell transplantation patients.

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Allogeneic mesenchymal stromal cells (MSCs) are widely used in regenerative medicine, but little is known about their immunogenicity. In this study, we monitored the therapeutic and immunogenic effects of decidual stromal cells (DSCs) from term placentas when used as a therapy for generalized severe

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

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Impaired growth and anaemia are major extracutaneous complications of epidermolysis bullosa (EB), but data on their development are lacking.To determine the clinical course of growth and anaemia in children with EB and clarify the impact of nutritional

High levels of "complexed" interleukin-6 in human blood.

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The biochemical nature of endogenous interleukin-6 (IL-6) as it exists in human serum or plasma was investigated. Serum from a patient following bone marrow (BM) transplantation and fresh plasma samples from patients with epidermolysis bullosa or psoriasis, as well as from normal volunteers, were

Autoimmunity to type VII collagen in SKH1 mice is independent of regulatory T cells.

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Epidermolysis bullosa acquisita is an autoimmune blistering disease characterized by circulating and skin basement membrane-bound IgG autoantibodies to type VII collagen, a major structural protein of the dermal-epidermal junction. Regulatory T cells (T(reg)) suppress self antigen-mediated

NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via a unique subdomain within the fibronectin-like repeats.

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Type VII collagen, the major component of anchoring fibrils, consists of a central collagenous triple-helical domain flanked by two noncollagenous, globular domains, NC1 and NC2. Approximately 50% of the molecular mass of the molecule is consumed by the NC1 domain. We previously demonstrated that

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