Septo-optic Dysplasia- Case Report

The clinical triad of septo-optic dysplasia (SOD) comprises the absence of the septum pellucidum, congenital optic nerve dysplasia, and multiple endocrine disorders. When any two of these factors are present, the condition is defined as an incomplete form of SOD. The authors report the case of an incomplete form of SOD in a 9-year-old boy with low vision and nystagmus present from birth. The bilateral ophthalmoscopic examination revealed small papillae with double contour images. Magnetic resonance imaging showed hypoplasia of the optic nerve bilaterally, chiasm and absence of the septum pellucidum.674653655De Morsier, G., Études sur les dysraphies cranio-encéphaliques. III. Agénésie du septum pellucidum avec malformation du tractus optique: La dysplasie septo-optique (1956) Schweiz Arch Neurol Psychiatr, 77 (1-2), pp. 267-292. , Texto inglês: [Studies on malformation of cranio-encephalic sutures.III. Agenesis of the septum lucidum with malformation of the optic tractKaplan, S., Grumbach, M.M., Hoyt, W., The syndrome of hypopituitary dwarfism, hypoplasia of optic nerves and malformation of prosencephalon: Report of 6 patients (1970) Pediatr Res, 4, pp. 480-481Lippe, B., Kaplan, S.A., La Franchi, S., Septo-optic dysplasia and maternal age (1979) Lancet, 2 (8133), pp. 92-93Purdy, F., Maternal factors in septo-optic dysplasia (1979) J Pediatr, 95 (4), p. 661Lambert, S.R., Hoyt, C.S., Narahara, M.H., Optic nerve hypoplasia (1987) Surv Ophthalmol, 32 (1), pp. 1-9Davis, G.V., Shock, J.P., Septo-optic dysplasia associated with see-saw nystagmus (1975) Arch Ophthalmol, 93 (2), pp. 137-139Williams, J., Brodsky, M.C., Griebel, M., Glasier, C.M., Caldwell, D., Thomas, P., Septo-optic dysplasia: The clinical insignificance of an absent septum pellucidum (1993) Dev Med Child Neurol, 35 (6), pp. 490-501Willnow, S., Kiess, W., Butenandt, O., Dörr, H.G., Enders, A., Strasser-Vogel, B., Endocrine disorders in septo-optic dysplasia (De Morsier syndrome) - evaluation and follow up of 18 patients (1996) Eur J Pediatr, 155 (3), pp. 179-18

Spironolactone treatment attenuates vascular dysfunction in type 2 diabetic mice by decreasing oxidative stress and restoring NO/GC signaling

Type 2 diabetes (DM2) increases the risk of cardiovascular disease. Aldosterone, which has pro-oxidative and pro-inflammatory effects in the cardiovascular system, is positively regulated in DM2. We assessed whether blockade of mineralocorticoid receptors (MR) with spironolactone decreases reactive oxygen species (ROS)-associated vascular dysfunction and improves vascular nitric oxide (NO) signaling in diabetes. Leptin receptor knockout [LepRdb/LepRdb (db/db)] mice, a model of DM2, and their counterpart controls [LepRdb/LepR+, (db/+) mice] received spironolactone (50 mg/kg body weight/day) or vehicle (ethanol 1%) via oral per gavage for 6 weeks. Spironolactone treatment abolished endothelial dysfunction and increased endothelial nitric oxide synthase (eNOS) phosphorylation (Ser1177) in arteries from db/db mice, determined by acetylcholine-induced relaxation and Western Blot analysis, respectively. MR antagonist therapy also abrogated augmented ROS-generation in aorta from diabetic mice, determined by lucigenin luminescence assay. Spironolactone treatment increased superoxide dismutase-1 and catalase expression, improved sodium nitroprusside and BAY 41-2272-induced relaxation, and increased soluble guanylyl cyclase (sGC) β subunit expression in arteries from db/db mice. Our results demonstrate that spironolactone decreases diabetes-associated vascular oxidative stress and prevents vascular dysfunction through processes involving increased expression of antioxidant enzymes and sGC. These findings further elucidate redox-sensitive mechanisms whereby spironolactone protects against vascular injury in diabetes

Influence of the pavement surface on the vibrations induced by heavy traffic in road bridges

The irregularity of the pavement surface governs the traffic-induced vibrations in road bridges, but it is either ignored or simulated by means of ideal pavements that differ significantly from real cases. This work presents a detailed dynamic analysis of a heavy truck crossing a 40-m span composite deck bridge using on-site measurements of different existing road profiles, as well as code-based ideal pavements. By activating or deactivating certain spatial frequency bands of the pavement, it is observed that the ranges 0.2 - 1 and 0.02 - 0.2 cycles/m are critical for the comfort of the pedestrians and the vehicle users, respectively. Well maintained roads with low values of the displacement Power Spectral Density (PSD) associated with these spatial frequency ranges could reduce significantly the vibration on the sidewalks and, specially, in the vehicle cabin. Finally, a consistent road categorisation for vibration assessment based on the PSD of the pavement irregularity evaluated at the dominant frequencies is proposed