Search results for "neon"
showing 10 items of 760 documents
Combined effect of AAV-U7-induced dystrophin exon skipping and soluble activin Type IIB receptor in mdx mice.
2012
Adeno-associated virus (AAV)-U7-mediated skipping of dystrophin-exon-23 restores dystrophin expression and muscle function in the mdx mouse model of Duchenne muscular dystrophy. Soluble activin receptor IIB (sActRIIB-Fc) inhibits signaling of myostatin and homologous molecules and increases muscle mass and function of wild-type and mdx mice. We hypothesized that combined treatment with AAV-U7 and sActRIIB-Fc may synergistically improve mdx muscle function. Bioactivity of sActRIIB-Fc on skeletal muscle was first demonstrated in wild-type mice. In mdx mice we show that AAV-U7-mediated dystrophin restoration improved specific muscle force and resistance to eccentric contractions when applied a…
Joint contractures in the absence of inflammation may indicate mucopolysaccharidosis
2009
Abstract Background Undiagnosed patients with the attenuated form of mucopolysaccharidosis (MPS) type I often have joint symptoms in childhood that prompt referral to a rheumatologist. A survey conducted by Genzyme Corporation of 60 European and Canadian rheumatologists and pediatric rheumatologists demonstrated that Bone and joint manifestations are prominent among most patients with MPS disorders. These life-threatening lysosomal storage diseases are caused by deficient activity of specific enzymes involved in the degradation of glycosaminoglycans. Patients with attenuated MPS disease often experience diagnostic delays. Enzyme replacement therapy is now commercially available for MPS I (l…
Efecto del precondicionamiento hipóxico en la respuesta a un insulto hiperóxico en el sistema nervioso central de ratones recién nacidos
2017
Mammal fetal life elapses in a low oxygen environment relative to the extra uterine. Interestingly, in the human being partial pressure of oxygen increases from fetal 3.3 kPa (25–30 mmHg) to 10.5 kPa (75–85 mmHg) in the first minutes after birth in the newly born infant. This rapid and brisk increase in oxygen availability to tissue generates a physiologic oxidative stress. However, both the lungs and the antioxidant defense system do not mature until late in gestation. Therefore, preterm infants often need respiratory support and oxygen supplementation in the delivery room to achieve postnatal stabilization. The use of supplementary oxygen will cause oxidative stress and damage. In experim…
Efectos de las diferentes ondas de flujo y de la pausa teleinspiratoria sobre la oxigenación y ventilación en modelo animal
2015
Clásicamente, la elección de modo ventilatorio en el neonato ha sido el flujo decelerado (DF) o ventilación mecánica por presión (VCP). Se ha sugerido que la onda de flujo decelerada mejora el intercambio de gases y la ventilación perfusión al compararla con la onda de flujo cuadrada (SF) o ventilación controlada por volumen (VCV). Aunque la superioridad de la DF comparada con la SF no ha sido todavía demostrada durante la ventilación de los neonatos. En el presente trabajo se han estudiado por primera vez los efectos sobre la oxigenación y ventilación de ambos flujos, decelerado y cuadrado, con y sin pausa tele- inspiratoria (EIP). Así como el efecto sobre la mecánica respiratoria y la hem…
Intrauterine and Neonatal Exposure to Opioids: Toxicological, Clinical, and Medico-Legal Issues
2023
Opioids have a rapid transplacental passage (i.e., less than 60 min); furthermore, symptoms characterize the maternal and fetal withdrawal syndrome. Opioid withdrawal significantly impacts the fetus, inducing worse outcomes and a risk of mortality. Moreover, neonatal abstinence syndrome (NAS) follows the delivery, lasts up to 10 weeks, and requires intensive management. Therefore, the prevention and adequate management of NAS are relevant public health issues. This review aims to summarize the most updated evidence in the literature regarding toxicological, clinical, and forensic issues of intrauterine exposure to opioids to provide a multidisciplinary, evidence-based approach for managing …