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ITER vacuum vessel structural analysis completion during manufacturing phase
Martinez, J.M.,Alekseev, A.,Sborchia, C.,Choi, C.H.,Utin, Y.,Jun, C.H.,Terasawa, A.,Popova, E.,Xiang, B.,Sannazaro, G.,Lee, A.,Martin, A.,Teissier, P.,Sabourin, F.,Caixas, J.,Fernandez, E.,Zarzalejos, North-Holland 2016 Fusion engineering and design Vol.109 No.1
Some years ago, analyses were performed by ITER Organization Central Team (IO-CT) to verify the structural integrity of the ITER vacuum vessel baseline design fixed in 2010 and classified as a Protection Important Component (PIC). The manufacturing phase leads the ITER Organization domestic agencies (IO-DA) and their contracted manufacturers to propose detailed design improvements to optimize the manufacturing or inspection process. These design and quality inspection changes can affect the structural margins with regards to the Codes&Standards and thus oblige to evaluate one more time the modified areas. This paper proposes an overview of the additional analyses already performed to guarantee the structural integrity of the manufacturing designs. In this way, CT and DAs have been strongly involved to keep the considerable margins obtained previously which were used to fix reasonable compensatory measures for the lack of In Service Inspections of a Nuclear Pressure Equipment (NPE).
Özlem Akgün Doğan,Pelin Özlem Şimşek Kiper,Gülen Eda Utine,Mehmet Alikaşifoğlu,Koray Boduroğlu 대한가정의학회 2017 Korean Journal of Family Medicine Vol.38 No.2
Williams syndrome (OMIM #194050) is a rare, well-recognized, multisystemic genetic condition affecting approxi-mately 1/7,500 individuals. There are no marked regional differences in the incidence of Williams syndrome. The syndrome is caused by a hemizygous deletion of approximately 28 genes, including ELN on chromosome 7q11.2. Prenatal-onset growth retardation, distinct facial appearance, cardiovascular abnormalities, and unique hyperso-cial behavior are among the most common clinical features. Here, we report the case of a patient referred to us with distinct facial features and intellectual disability, who was diagnosed with Williams syndrome at the age of 37 years. Our aim is to increase awareness regarding the diagnostic features and complications of this recognizable syn-drome among adult health care providers. Williams syndrome is usually diagnosed during infancy or childhood, but in the absence of classical findings, such as cardiovascular anomalies, hypercalcemia, and cognitive impair-ment, the diagnosis could be delayed. Due to the multisystemic and progressive nature of the syndrome, accurate diagnosis is critical for appropriate care and screening for the associated morbidities that may affect the patient’s health and well-being.
Guvenoglu, Merve,Simsek-Kiper, Pelin Ozlem,Kosukcu, Can,Taskiran, Ekim Z.,Saltik-Temizel, Inci Nur,Gucer, Safak,Utine, Eda,Boduroglu, Koray The Korean Society of Pediatric Gastroenterology 2022 Pediatric gastroenterology, hepatology & nutrition Vol.25 No.6
Congenital diarrheal disorders (CDDs) with genetic etiology are uncommon hereditary intestinal diseases characterized by chronic, life-threatening, intractable watery diarrhea that starts in infancy. CDDs can be mechanistically divided into osmotic and secretory diarrhea. Congenital tufting enteropathy (CTE), also known as intestinal epithelial dysplasia, is a type of secretory CDD. CTE is a rare autosomal recessive enteropathy that presents with intractable neonatal-onset diarrhea, intestinal failure, severe malnutrition, and parenteral nutrition dependence. Villous atrophy of the intestinal epithelium, crypt hyperplasia, and irregularity of surface enterocytes are the specific pathological findings of CTE. The small intestine and occasionally the colonic mucosa include focal epithelial tufts. In 2008, Sivagnanam et al. discovered that mutations in the epithelial cell adhesion molecule (EpCAM, MIM# 185535) were the genetic cause of CTE (MIM# 613217). More than a hundred mutations have been reported to date. Furthermore, mutations in the serine peptidase inhibitor Kunitz type 2 (SPINT2, MIM# 605124) have been linked to syndromic CTE. In this study, we report the case of a 17-month-old male infant with congenital diarrhea. Despite extensive etiological workup, no etiology could be established before admission to our center. The patient died 15 hours after being admitted to our center in a metabolically decompensated state, probably due to a delay in admission and diagnosis. Molecular autopsy with exome sequencing revealed a previously reported homozygous missense variant, c.757G>A, in EpCAM, which was confirmed by histopathological examination.