Desenvolvimento de ferramenta vocacionada para o dimensionamento de estruturas metálicas de acordo com a NP EN 1993-1-1

Dissertação de mestrado integrado em Engenharia Civil

Aortopulmonary window. Clinical and surgical assessment of 18 cases

OBJECTIVE: Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We evaluated our experience with this anomaly and compared it with 296 cases reported in the literature. METHODS: Retrospective study of 18 patients diagnosed as having APW (age range from 13 days to 31 years, 13 (72.2%) females), divided into two groups: Group A (GA): 10 patients with isolated APW, and Group B (GB): 8 patients with associated lesions. RESULTS: Heart failure occurred in 14 patients, and cyanosis in 3: 2 from GB (tetralogy of Fallot - TF, and double outlet right ventricle - DORV), and one from GA with pulmonary hypertension. In 5 patients from GA the diagnosis of mitral regurgitation was made based on a systolic murmur and LV hypertrophy on the EKG. In GB, clinical findings were determined by the associated defect. Diagnosis was established by echocardiography in 11 (61.2%) of the patients. In 3 patients, a wrong diagnosis of mitral regurgitation was made, in 1 a patent ductus arteriosus was diagnosed and in 3 others, the diagnosis of APW was masked by other important associated defects (2 cases of DORV and 1 case of TF). The diagnosis was made by catheterization in 3 (16.6%) patients, by surgery in 3 (16.6%) and by necropsy in 1 (5.5%). Corrective surgery was performed in 14 (77.7%) patients, with one immediate death and good long-term follow-up in the remaining patients. CONCLUSION: APW can be confused with other defects. Clinical findings, associated with an adequate echocardiogram can provide the information for the correct diagnosis.

Recurrence of atrial septal defect in three generations

Beginning with a patient presenting with an atrial septal defect (ASD) of the secundum type, the genealogy was identified in four affected individuals who belonged to three successive generations of the same family. The defects were visually confirmed in all individuals and were found to be anatomically similar. No other congenital malformations were present in these individuals. The genealogy was identified in 1972, when ASD recurred in two generations, and it was concluded that the mechanism of transmission was autosomal recessive. The fifth individual, identified 21 years later, and having an anomaly identical to that of the others, was the child of a couple who had no consaguinity and whose mother was a member of the previously studied genealogy. Considering the absence of phenotype in the parents and the rarity of the ASD gene in the general population, the occurrence of the uniparental disomy for this family nucleus, and the same autosomal recessive mechanism of transmission by this affected individual is possible. This study reports the familial occurrence of ASD by genetic mechanisms of transmission, emphasizing the necessity for genetic-clinical studies in members of the familial nucleus in order to detect new carriers, who usually are asymptomatic, thereby allowing for early and adequate treatment of individuals who may be affected.

Critical aortic stenosis in the neonate. Clinical assessment and surgical outcome in 18 patients

OBJECTIVE - To assess neonates with aortic stenosis with early decompensation operated upon. (LCO) (CHF). METHODS - A and retrospective study analyzing 6 neonates with LCO, group I (GI), and 12 neonates with CHF, group II (GII). Clinical radiographic, electrocardiographic and echocardiographic findings also provided comparative bases for the study, as did surgical and evolutional findings. RESULTS - The mean ages at hospitalization and surgery (p = 0.0031) were 14.3 and 14.8 days in GI and 35.4 and 42.8 days in GII, respectively. Cardiac murmurs were more intense in GII (p = 0.0220). The aortic ring was smaller in GI (8.0 ± 2.5mm) as compared to GII (11.4±1.4mm) (p = 0.2882). Ventricular function was reduced to 18±5.5% and 33.3±7.6% in GI and GII, respectively (p = 0.0162). Aortic atresia, however, was present only in 2 neonates in GI. Five of 6 patients in GI died but all patients in GII survived (p=0.0007). In the latter group, 84.6% of the patients were in functional class I (FC-I) in the long-term follow-up, with moderate residual lesions in 6 neonates, discrete residual lesions in 4, and reoperation in 2. CONCLUSION - Aortic stenosis is a severe anomaly of the neonate, whose immediate evolution depends on the pre-operative anatomic and functional findings, and the late evolution essentially depends on the anatomic features of the valve.

Experimental analysis of Maritime pine and Iroko single shear dowel-type connections

"Available online 21 March 2016"

Experimental procedures for the identification of material properties of intervertebal disc - a contribution for the development of biomimetic substitutes for the nucleus pulposus

Tese de Doutoramento em Engenharia Mecânica.

Epidemiological study of congenital heart defects in children and adolescents: analysis of 4,538 cases

OBJECTIVE: To analyze the frequency and prevalence of congenital heart defects in a tertiary care center for children with heart diseases. METHODS: We carried out an epidemiological assessment of the first medical visit of 4,538 children in a pediatric hospital from January 1995 to December 1997. All patients with congenital heart defects had their diagnoses confirmed at least on echocardiography. The frequency and prevalence of the anomalies were computed according to the classification of sequential analysis. Age, weight, and sex were compared between the groups of healthy individuals and those with congenital heart defects after distribution according to the age group. RESULTS: Of all the children assessed, 2,017 (44.4%) were diagnosed with congenital heart disease, 201 (4.4%) with acquired heart disease, 52 (1.2%) with arrhythmias, and 2,268 (50%) were healthy children. Congenital heart diseases predominated in neonates and infants, corresponding to 71.5% of the cases. Weight and age were significantly lower in children with congenital heart defects. Ventricular septal defect was the most frequent acyanotic anomaly, and tetralogy of Fallot was the most frequent cyanotic anomaly. CONCLUSION: Children with congenital heart defects are mainly referred during the neonatal period and infancy with impairment in gaining weight. Ventricular septal defect is the most frequent heart defect.

Desórdenes Congénitos de la Glicosilación (CDG). Primera investigación Argentina de patologías genéticas causadas por defectos en la biosíntesis de las glicoproteínas: estudios clínicos, bioquímicos y moleculares para la identificación y caracterización de las distintas variantes

La caracterización de nuevas patologías causadas por defectos en la glicosilación de proteínas se ha incrementado exponencialmente en los últimos cinco años. Los Desórdenes Congénitos de la Glicosilación ó Congenital Disorders of Glycosylation, sigla en inglés, CDG, comprenden defectos en la biosíntesis de las glicoproteínas, ya sea en la vía de la N-glicosilación, como así también de la O-glicosilación proteica. La presentación fenotípica es multisistémica, existen más de 500 genes que codifican para proteínas implicadas en procesos de glicosilación, poniendo de manifiesto la importancia crucial de la glicobiología en los procesos celulares. La mayoría de los CDG conocidos hasta el momento son defectos de N-glicosilación (clasificadas CDG-Ia hasta CDG-Im y CDG-IIa hasta CDG-IIf), aunque están siendo descritas alteraciones de O-glicosilación, como causa primaria de diferentes distrofias musculares, condrodisplasias, mucolipidosis I y II; Síndrome de Exostosis Múltiple Hereditario (EMH), trastornos de la migración neuronal e incluso existen defectos combinados de N- y O-glicosilación. Presentan una mortalidad infantil elevada, de aproximadamente un 25% por infecciones graves o fallos orgánicos. Las principales manifestaciones clínicas son: retraso psicomotor, convulsiones, hipotonía axial, estrabismo e hipoplasia cerebelosa, entre las características más frecuentes, acompañadas en algunos casos por dismorfias, hepatopatía, coagulopatía, enteropatía, entre otras manifestaciones, sin existir un patrón único de expresión clínica y pudiendo observarse manifestaciones inusuales de la enfermedad. Existe en nuestro medio un sub-diagnóstico de estas patologías, atribuible al desconocimiento de la gran variabilidad fenotípica y a la falta de metodologías para su diagnóstico. El estudio de alteraciones de la glicosilación proteica permitirá la identificación de diferentes clases de CDG como responsables de síndromes clínicos no explicados e incluso el hallazgo de nuevas variantes de estas patologías en nuestro medio. Como Objetivo General, se desea contribuir al desarrollo de un capítulo inédito en Latinoamérica, en el área de las Enfermedades Metabólicas Hereditarias, desde los diferentes aspectos: clínico, bioquímico y molecular, conjuntamente con la aplicación de criterios cada vez más amplios para la detección de CDG en nuestro medio y el conocimiento de los aspectos fisiopatogénicos propios de estas enfermedades.

Особенности локальной магнитной аномалии Цкалцминда-Уреки

შავი ზღვის სანაპიროზე ანომალიური გეომაგნიტური ველის ძველი და ახალი მნიშვნელობების შედარების მიზნით ჩატარებულია პროფ. მ. ნოდიას 1936-38 წლების მაგნიტურ-საძიებო ექსპედიციების შედეგებისა და 2000-2005 წლებში გეოფიზიკის ინსტიტუტის საველე-საექსპედიციო რაზმის მიერ მიღებული მონაცემების შედარებითი ანალიზი. გამოყოფილია გურიის რეგიონული გეომაგნიტური ანომალიის შემადგენელი ელემენტები ომფარეთისა და წყალწმინდა-ურეკის ლოკალური ანომალიები. შესრულებულია ამ ანომალიებია ფართობითი მაგნიტური აგეგმვა და კონტურირება. თეორიულად შეფასებულია სანაპირო ზოლში განთავსებულ წყალწმინდა-ურეკის ანომალიაში დამაგნიტებული ქვიშის არაერთგვაროვანი წარმონაქმნების, ქედებისა და ცალკეული ბორცვების არსებობით გამოწვეული შესაძლო მაგნიტური ეფექტი. აღნიშნული ლოკალური მაგნიტური ანომალიების განსაკუთრებულად არაერთგვაროვანი, წვრილმასშტაბოვანი სტრუქტურა მიანიშნებს მათ კავშირზე სიღრმულ გეოლოგიურ სტრუქტურებთან.

«Геомагнитная аномалия» широтного распределения электронной концентрации F2 слоя ионосферы

მსოფლიო ქსელის იონოსფერული სადგურების კრიტიკული სიხშირეების - fmin f0E, f0El, f0F2, დედამიწის ეკვატორთან ახლომდებარე გეომაგნიტური ობსერვატორიების (რომლებიც მოიცავენ ჩრდილოეთისა და სამხრეთის განედებს j≈±300-ს) დედამიწის მაგნიტური ველის X მდგენელისა და დედამიწის ახლომდებარე კოსმოსურ სივრცეში განხორციელებული ექსპერიმენტების კვლევის შედეგები, ერთობრივი კომპლექსური ანალიზი.

Модель локальной геомагнитной аномалии Цкалцминда-Уреки

მოცემულია წყალწმინდა-ურეკის საკურორტო ზონაში ჩატარებული მაგნიტომეტრიული და ელექტრომეტრიული გაზომვების გეოფიზიკური ინტერპრეტაციის შედეგები, რომელთა საფუძველზეც ჩატარებულია ამ ლოკალური მაგნიტური ანომალიის თვისობრივი ანალიზი.

Цителицкароиская магнитная аномалия и её геологическая интерпретация на основе новых геолого-гоеомагнитных данных

ახალი გეომაგნიტური და გეოლოგიური ინფორმაციების საფუძველზე დადგენილია, რომ წითელწყაროს მაგნიტური ანომალიის წყაროა ბაიოსის პორფირიტული წყების ქანები. ამასთან ერთად, გამოთქმულია მოსაზრება საკვლევი რეგიონის გეოტექნიკური განვითარების და მაგნიტური ანომალიის წარმოშობის გეოლოგიური ისტორიის შესახებ.

Heat and mass transfer in tubular inorganic membranes

Inorganic membranes, permeation, diffusion, heat transfer, mass transfer, axial dispersion

Electrophysiological Studies and Radiofrequency Ablations in Children and Adolescents with Arrhythmia

Background:Radiofrequency ablation is the standard non-pharmacological treatment for arrhythmias in pediatric patients. However, arrhythmias and their associated causes have particular features in this population.Objective:To analyze the epidemiological characteristics and findings of electrophysiological diagnostic studies and radiofrequency ablations in pediatric patients referred to the Electrophysiology Unit at Instituto de Cardiologia do Rio Grande do Sul, in order to characterize the particularities of this population.Methods:Cross-sectional study with 330 electrophysiological procedures performed in patients aged less than 20 years between June 1997 and August 2013.Results:In total, 330 procedures (9.6% of the overall procedures) were performed in patients aged less than 20 years (14.33 ± 3.25 years, age range 3 months to 19 years), 201 of which were males (60.9%). A total of 108 (32.7%) electrophysiological diagnostic studies were performed and of these, 48.1% showed abnormal findings. Overall, 219 radiofrequency ablations were performed (66.3%) with a success rate of 84.8%. The presence of an accessory pathway was the most prevalent finding, occurring in 158 cases (72.1%), followed by atrioventricular nodal reentrant tachycardia (16.8%), typical atrial flutter (3.1%) and extrasystoles originating from the right ventricular outflow tract (2.7%). Three patients developed complications during ablation (1.4%). Among congenital heart diseases, which occurred in 51 (15.4%) patients, atrial sept defect was the most frequent (27.4%), followed by ventricular sept defect (25.4%) and Ebstein's anomaly (17.6%).Conclusion:Electrophysiological study and radiofrequency ablation are effective tools for diagnosis and treatment of arrhythmias in the pediatric population.

Flow and transport in electrochromatography

Analyte retention, analyte transport, axial dispersion, adsorption, charge-selectivity, concentration polarization, confocal laser scanning microscopy, donnan-exclusion, electrical double layer; electrochromatography; electrohydrodynamics, electrokinetic instability, electroosmosis; electroosmotic flow; electroosmotic mobility, electroosmotic perfusion, electrophoresis, hierarchical porous media, hydrodynamic flow, induced-charge electroosmosis, ion-permselectivity, ion-permselective transport, monolith, nonequilibrium electrical double layer, nonequilibrium electrokinetic effects, nonlinear electroosmosis, plate height, plate number, porous media, pore-scale dispersion, refractive index matching, space charge effects, sphere packing, quantitative imaging, wall effect, zeta-potential