Radiosterilized Pig Skin, Silver Nanoparticles and Skin Cells as an Integral Dressing Treatment for Burns: Development, Pre-Clinical and Clinical Pilot Study.

Radiosterilized pig skin (RPS) has been used as a dressing for burns since the 1980s. Its similarity to human skin in terms of the extracellular matrix (ECM) allows the attachment of mesenchymal stem cells, making it ideal as a scaffold to create cellularized constructs. The use of silver nanoparticles (AgNPs) has been proven to be an appropriate alternative to the use of antibiotics and a potential solution against multidrug-resistant bacteria.

Development of Radiosterilized Porcine Skin Electrosprayed with Silver Nanoparticles Prevents Infections in Deep Burns.

Extensive burns represent a significant challenge in biomedicine due to the multiple systemic and localized complications resulting from the major skin barrier loss. The functionalization of xenografts with nanostructured antibacterial agents proposes a fast and accessible application to restore barrier function and prevent localized bacterial contamination. Based on this, the objective of this work was to functionalize a xenograft by electrospray deposition with silver nanoparticles (AgNPs) and to evaluate its antibiofilm and cytotoxic effects on human fibroblasts.

Effects of mesenchymal stem cell culture on radio sterilized human amnion or radio sterilized pig skin in burn wound healing.

Deep second and third degree burns treatment requires fibroblasts, keratinocytes and other skin cells in order to grow new dermis and epidermis. Cells can proliferate, secrete growth factors and extracellular matrix required to repair the damaged tissue. Radiosterilized human amnion and radiosterilized pig skin have been used as natural origin skin dressings for burned patients.

Severe anemia in patients with Propionic acidemia is associated with branched-chain amino acid imbalance.

Background: Propionic acidemia (PA), an inborn error of metabolism, is caused by a deficiency in propionyl-CoA carboxylase. Patients have to follow a diet restricted in the propiogenic amino acids isoleucine (Ile), valine (Val), methionine (Met) and threonine (Thr); proper adherence can prevent and treat acute decompensation and increase life expectancy. However, chronic complications occur in several organs even though metabolic control may be largely maintained.

Long-term follow-up with filter paper samples in patients with propionic acidemia.

Background: Propionic acidemia (PA) is an inherited disorder caused by deficiency of propionyl CoA carboxylase. Most patients with this disorder are diagnosed during the neonatal period because of severe metabolic acidosis and hyperammonemia. Patients are required to undergo blood and urine analysis at least 3 to 4 times per year, depending on age and metabolic control.

Allogeneic Limbal Epithelial Transplantation Modified With Solid Platelet-Rich Plasma for Bilateral Limbal Stem Cell Deficiency.

Purpose: To present successful management of bilateral limbal stem cell deficiency (LSCD) by using an allogeneic limbal epithelial stem cell transplantation together with solid activated platelet-rich plasma (PRP).

Methods: A 59-year-old man with a history of bilateral LSCD due to penicillin-induced Stevens-Johnson Syndrome suffered from a lime corneal burn in his right eye, leading to a total LSCD with severely reduced visual acuity. After stabilizing the ocular surface, we performed an allogeneic limbal epithelial transplantation from a cadaveric donor using an autologous clot of PRP to cover the limbal grafts to nourish the ocular surface microenvironment.

Clinical and molecular diagnosis of non-phosphomannomutase 2 N-linked congenital disorders of glycosylation in Spain.

The congenital disorders of glycosylation (CDG) are defects in glycoprotein and glycolipid glycan synthesis and attachment. They affect multiple organ/systems, but non-specific symptoms render the diagnosis of the different CDG very challenging. Phosphomannomutase 2 (PMM2)-CDG is the most common CDG, but advances in genetic analysis have shown others to occur more commonly than previously thought.

Overview on radiation and tissue banking in Latin America.

The International Atomic Energy Agency (IAEA) played an important role in the establishment of new tissue banks and the improvement of already existing ones in Latin America. The Agency strongly supported, through regional, interregional and national technical cooperation projects, providing equipment, expert missions and training for the production and application of human tissues for transplantation. From 1999 to 2005 five regional courses were given in Buenos Aires under the modality of 1-year distance learning training courses and 1-week face to face courses.

Silver-pig skin nanocomposites and mesenchymal stem cells: suitable antibiofilm cellular dressings for wound healing.

Background: Treatment of severe or chronic skin wounds is an important challenge facing medicine and a significant health care burden. Proper wound healing is often affected by bacterial infection; where biofilm formation is one of the main risks and particularly problematic because it confers protection to microorganisms against antibiotics. One avenue to prevent bacterial colonization of wounds is the use of silver nanoparticles (AgNPs); which have proved to be effective against non-multidrug-resistant and multidrug-resistant bacteria.

Generation of Two Biological Wound Dressings as a Potential Delivery System of Human Adipose-Derived Mesenchymal Stem Cells.

Human adipose-derived mesenchymal stem cells (hADMSCs) are believed to be potential key factors for starting the regenerative process after tissue injury. However, an efficient method of delivering these regenerative cells to an external wound site is still lacking. Human amnion and pig skin have long been used as skin wound dressings for the treatment of burns and other skin lesions.

Urea cycle disorders in Spain: an observational, cross-sectional and multicentric study of 104 cases.

Background: Advances in the diagnosis and treatment of urea cycle disorders (UCDs) have led to a higher survival rate. The purpose of this study is to describe the characteristics of patients with urea cycle disorders in Spain.

Methods: Observational, cross-sectional and multicenter study.

Liver transplantation for classical maple syrup urine disease: long-term follow-up.

Objectives: The aim of the study was to evaluate indications, results, and clinical and neurological evolution in children who have undergone liver transplantation for classical maple syrup urine disease (MSUD).

Methods: Descriptive study of liver transplantation for MSUD between 1991 and 2012. Eight patients were transplanted.

[Importance of early diagnosis of phenylketonuria in women and control of phenylalanine levels during pregnancy].

The phenylalanine hydroxylase (PAH) in the liver hydroxylates phenylalanine from the diet. Fetuses depend for the hydroxylation of phenylalanine the maternal metabolism , fetal maturity does not come until week 26. Though the women with PAH deficiency (phenylketonuria, PKU) not adequately hydroxylate phenylalanine diet so their blood levels are high.

A novel regulatory defect in the branched-chain α-keto acid dehydrogenase complex due to a mutation in the PPM1K gene causes a mild variant phenotype of maple syrup urine disease.

This article describes a hitherto unreported involvement of the phosphatase PP2Cm, a recently described member of the branched-chain α-keto acid dehydrogenase (BCKDH) complex, in maple syrup urine disease (MSUD). The disease-causing mutation was identified in a patient with a mild variant phenotype, involving a gene not previously associated with MSUD. SNP array-based genotyping showed a copy-neutral homozygous pattern for chromosome 4 compatible with uniparental isodisomy.

A novel congenital disorder of glycosylation type without central nervous system involvement caused by mutations in the phosphoglucomutase 1 gene.

Recent years have seen great advances in our knowledge of congenital disorders of glycosylation (CDG), a clinically and biochemically heterogeneous group of genetic diseases caused by defects in the synthesis (CDG-I) or processing (CDG-II) of glycans that form glycoconjugates. This paper reports a new subtype of non-neurological CDG involving the impaired cytoplasmic biosynthesis of nucleotide sugars needed for glycan biosynthesis. A patient presented with muscle fatigue, elevated creatine kinase, growth hormone deficiency, and first branchial arch syndrome.

Use of irradiated human amnion as a matrix for limbal stem cell culture.

Several ocular diseases affect the corneal surface; the development of effective technologies for the treatment of corneal lesions has brought about an improvement in the quality of life of affected patients. The aim of this study is to culture and characterize limbal stem cells cultured on gamma ((60)Co) radiosterilized human amnion (RHA). Limbal stem cells were isolated from ten preserved samples of corneal transplant.

[Epidemiological study of the metabolic diseases with homocystinuria in Spain].

Objectives: To determine the prevalence of homocystinuria in Spain and to establish the measures and mechanisms to ensure its prevention, diagnosis and treatment.

Material And Methods: A national cross-sectional survey was conducted by means of a questionnaire sent to 35 hospitals in which children and adult patients are treated.

Results: Using the questionnaires submitted by 25 physicians from 16 centres, 75 patients were identified: 41 transsulphuration defects (one deceased), 27 remethylation (six deaths) and 7 without a syndromic diagnosis.

Physical development in patients with phenylketonuria on dietary treatment: a retrospective study.

Objectives: To evaluate the growth and physical development in patients with phenylalanine hydroxylase deficiency who follow exclusively dietary treatment.

Methods: Anthropometric measurements of 160 patients with hyperphenylalaninemia who were followed at our center over a 25 year period were obtained. Only patients treated exclusively with a protein-restrictive diet supplemented with amino acid mixtures were included.