ABSTRACT
The tendency over the last decades in the aerospace industry is to substitute classic metallic materials with new composite materials such as carbon fiber composites (CFC), fiber glass, etc., as well as adding electronic devices to ensure the safety and proper platform operation. Due to this, to protect the aircraft against the Electromagnetic Environmental Effects (E3), it is mandatory to develop accurate electromagnetic (EM) characterization measurement systems to analyze the behavior of new materials and electronic components. In this article, several measurement methods are described to assess the EM behavior of the samples under test: microstrip transmission line for a surface current analysis, free space to obtain intrinsic features of the materials and shielding effectiveness (SE) approaches to figure out how well they isolate from EM fields. The results presented in this work show how the different facilities from the National Institute of Aerospace Technology (INTA) are suitable for such purposes, being capable of measuring a wide variety of materials, depending on the type of test to be carried out.
ABSTRACT
The electromagnetic performance of aerial platforms, which are composed mostly of nonmetallic materials, is a subject of great interest at present time. The behavior of this type of composite structure against electromagnetic environmental effects (E3), such as lightning, is not well-studied as in the case of metalic structures. The purpose of this article is to characterize the joints present in aerial platforms constructed mainly of nonmetallic composite materials. The study of these joints is fundamental because electrical discontinuities or preferential routes can produce changes in the electromagnetic behavior of an aircraft. The proposed measurement system for the characterization of these joints is a microstrip line. The flexibility of the test setup allows for evaluation of different joints in carbon fiber composite (CFC) samples with a different number of plies. Additionally, approximated models of the behavior of the joints as well as the detection of possible defects in the joining process are reported.
ABSTRACT
BACKGROUND: The Prader-Willi syndrome (PWS) is a disease of genetic origin. It is characterized by neonatal hypotonia, hypogonadism, hiperfagia leading to obesity, low stature, developmental delay, moderate mental retardation, abnormal behavior and characteristic facial appearance. It is caused by the loss or the inactivation of paternal genes of the imprinted region 15q11-13. There are different genetic causes: paternal 15q11-q13 deletion in 70% of patients, maternal uniparental disomy in the 20-25% and less than 5% have an imprinting defect. We present the results obtained in the transverse clinical - genetic study of 77 PWS patients. PATIENTS AND METHODS: There has been realized the study of 374 suspected PWS patients. Cytogenetics studies of bands G and hybridization in situ fluorescent (FISH) and molecular genetics analysis of microsatellites, Southern blot, MS-PCR and sequenciation were carried out. Holm's criteria use for the correlation phenotype - genotype in 48 patients. RESULTS: PWS was confirmed in 77 patients, 46 deletion, 16 uniparental disomy, two imprinting defect and 13 only PWS methylation pattern. Significant differences do not observe in the correlation phenotype - genotype. CONCLUSIONS: The frequencies of the molecular alterations, 71.87 % deletion, 25 % UPD and 3.12 % DI, they are similar to described in the literature. It presents the algorithm of diagnosis used with the MS-PCR as rapid technology to confirm PWS.
Subject(s)
Prader-Willi Syndrome/diagnosis , Prader-Willi Syndrome/genetics , Adolescent , Adult , Algorithms , Child , Child, Preschool , Cross-Sectional Studies , Female , Humans , Infant , Male , Young AdultABSTRACT
Prader-Willi syndrome (PWS) and Angelman syndrome (AS) are genetic disorders caused by a deficiency of imprinted gene expression from the paternal or maternal chromosome 15, respectively. This deficiency is due to the deletion of the 15q11-q13 region, parental uniparental disomy of the chromosome 15, or imprinting defect (ID). Mutation of the UBE3A gene causes approximately 10% of AS cases. In this present study, we describe the molecular analysis and phenotypes of two PWS patients and four AS patients with ID. One of the PWS patients has a non-familial imprinting center (IC) deletion and displayed a severe phenotype with an atypical PWS appearance, hyperactivity and psychiatric vulnerability. The other PWS and AS patients did not present genetic abnormalities in the IC, suggesting an epimutation as the genetic cause. The methylation pattern of two AS patients showed a faint maternal band corresponding to a mosaic ID. One of these mosaic patients displayed a mild AS phenotype while the other displayed a PWS-like phenotype.
