Background: Maffucci syndrome is a rare genetic disorder associated with the development of multiple enchondromas and soft tissue cavernous hemangiomas, as well as an increased risk of malignant tumors. Case Description: Here we report a case of Maffucci syndrome in a patient who presented with a giant left frontal lobe tumor. Molecular genetic analysis of the tumor revealed an isocitrate dehydrogenase (IDH) mutation p.R132H (c.395C>A) mutation in the IDH1 gene and a heterozygous duplication of the CDKN2A genes. Conclusions: The presence of an IDH1 mutation is notable because this mutation is frequently seen in glial tumors and other neoplasms, and its co-occurrence with Maffucci syndrome may represent a novel risk factor for the development of gliomas. This case underscores the importance of genetic testing in patients with Maffucci syndrome who present with central nervous system tumors, as well as the need for further research to understand the relationship between IDH1 mutations and the development of gliomas in this population.
Aircraft noise and methods of its reduction are considered. Brief information about noise sources and acceptable value are described. Regulatory documents, the noise standard established by ICAO are also considered. Recommendations for reducing aircraft noise are presented. The whole body is susceptible to noise. Even at a relatively low, but constant noise level, a person may experience discomfort, irritability, and headaches. Especially dangerous is sudden noise that violates the psychological comfort of a person (takeoff and landing, areas of the route of supersonic aircraft). The result of the specific effects of noise on the hearing organs are diseases such as sensorineural hearing loss, manifested by persistent hearing loss. The development of sensorineural hearing loss is associated with prolonged and regular levels of aircraft noise. Pilots are exposed to this noise level, the noise level fluctuates in the range of 90-109 dB, and in the cockpits of helicopters - in the range of 100-118 dB. Aircraft noise around airports and airways covers the entire area of the territory, including the residential area. When the airport is close to the city, there are problems associated with a high level of aircraft noise, which creates discomfort for the human body.
English is the international language of science and technology. English is not often the first language of technical documentation readers. Most are limited in knowledge of the English language. sentence structure and the large number of meanings and synonyms that many English words possess are complex. Correctly understanding the maintenance and operation documentation is very important in aviation, it also affects the work of safety to protect human lives. The purpose of this article is to highlight the importance of the ASD-STE100 document for students of the Academy of Civil Aviation in Kazakhstan. In this document, we will consider the relevance of applying this document during training in the aviation industry, as well as touch on the requirements for proficiency in technical English and summarize the basic rules aimed at facilitating the study and understanding of the English language.
Pineal region tumors are classically presented with vertical gaze palsy, hypothalamic symptoms, or, more often, hydrocephalus, by obstructing the aqueduct. This case report describes a patient with obstructive hydrocephalus and a hearing loss as an initial complaint of pineal region tumor. The patient started to behave aggressively after falling and stopped hearing what his family was saying. Although he could read and speak, most of the time he was not able to understand where he is and attempted multiple times to escape from the hospital. Detailed neurological examination was significant only for slight horizontal nystagmus. The first operation was the placement of external ventricular drainage and the second one was tumor removal. Aggressive behavior stopped after the first operation and hearing loss completely resolved after tumor removal.
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