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QUESTION 1 You have a hybrid Exchange Server 2016 organization. Some of the mailboxes in the research department are hosted on-premises. Other mailboxes in the research department are stored in Microsoft Office 365. You need to search the mailboxes in the research department for email messages that contain a specific keyword in the message body. What should you do? A. From the Exchange Online Exchange admin center, search the delivery reports. B. Form the on-premises Exchange center, search the delivery reports. C. From the Exchange Online Exchange admin SY0-401 exam center, create a new In-Place eDiscovery & Hold. D. From the Office 365 Compliance Center, create a new Compliance Search. E. From the on-premises Exchange admin center, create a new In-Place eDiscovery & Hold. Correct Answer: E QUESTION 2 You have an Exchange Server 2016 organization. You plan to enable Federated Sharing. You need to create a DNS record to store the Application Identifier (AppID) of the domain for the federated trust. Which type of record should you create? A. A B. CNAME C. SRV D. TXT Correct Answer: D QUESTION 3 Your company has an Exchange Server 2016 200-310 exam Organization. The organization has a four- node database availability group (DAG) that spans two data centers. Each data center is configured as a separate Active Directory site. The data centers connect to each other by using a high-speed WAN link. Each data center connects directly to the Internet and has a scoped Send connector configured. The company's public DNS zone contains one MX record. You need to ensure that if an Internet link becomes unavailable in one data center, email messages destined to external recipients can 400-101 exam be routed through the other data center. What should you do? A. Create an MX record in the internal DNS zone B. B. Clear the Scoped Send Connector check box C. Create a Receive connector in each data center. D. Clear the Proxy through Client Access server check box Correct Answer: AQUESTION 4 Your network contains a single Active Directory forest. The forest contains two sites named Site1 and Site2. You have an Exchange Server 2016 organization. The organization contains two servers in each site. You have a database availability group (DAG) that spans both sites. The file share witness is in Site1. If a power failure occurs at Site1, you plan to mount the databases in Site2. When the power is restored in Site1, you Cisco CCNP Security 300-207 exam SITCS need to prevent the databases from mounting in Site1. What should you do? A. Disable AutoReseed for the DAG. B. Implement an alternate file share witness. C. Configure Datacenter Activation Coordination (DAC) mode. D. Force a rediscovery of the EX200 exam network when the power is restored. Correct Answer: C QUESTION 5 A new company has the following: Two offices that connect to each other by using a low-latency WAN link In each office, a data center that is configured as a separate subnet Five hundred users in each office You plan to deploy Exchange Server 2016 to the network. You need to recommend which Active Directory deployment to use to support the Exchange Server 2016 deployment What is the best recommendation to achieve the goal? A. Deploy two forests that each contains one site and one site link. Deploy two domain controllers to each forest. In each forest configure one domain controller as a global catalog server B. Deploy one forest that contains one site and one site link. Deploy four domain controllers. Configure all of the domain controllers as global catalog servers. C. Deploy one forest that contains two sites and two site links. Deploy two domain controllers to each site in each site, configure one domain controller as a global catalog server D. Deploy one forest that contains two sites and one site link. Deploy two domain controllers to each site. Configure both domain controllers as global catalog servers Correct Answer: C QUESTION 6 How is the IBM Content Template Catalog delivered for installation? A. as an EXE file B. as a ZIP file of XML files C. as a Web Appli cati on Archive file D. as a Portal Application Archive file Correct Answer: D QUESTION 7 Your company has a data center. The data center contains a server that has Exchange Server 2016 and the Mailbox server role installed. Outlook 300-101 exam anywhere clients connect to the Mailbox server by using thename outlook.contoso.com. The company plans to open a second data center and to provision a database availability group (DAG) that spans both data centers. You need to ensure that Outlook Anywhere clients can connect if one of the data centers becomes unavailable. What should you add to DNS? A. one A record B. two TXT records C. two SRV records D. one MX record Correct Answer: A QUESTION 8 You have an Exchange Server 2016 EX300 exam organization. The organization contains a database availability group (DAG). You need to identify the number of transaction logs that are in replay queue. Which cmdlet should you use? A. Test-ServiceHealth B. Test-ReplicationHealth C. Get-DatabaseAvailabilityGroup D. Get-MailboxDatabaseCopyStatus Correct Answer: D QUESTION 9 All users access their email by using Microsoft Outlook 2013 From Performance Monitor, you discover that the MSExchange Database\I/O Database Reads Average Latency counter displays values that are higher than normal You need to identify the impact of the high counter values on user connections in the Exchange Server organization. What are two client connections 400-051 exam that will meet performance? A. Outlook on the web B. IMAP4 clients C. mobile devices using Exchange ActiveSync D. Outlook in Cached Exchange ModeE. Outlook in Online Mode Correct Answer: CE QUESTION 10 You work for a company named Litware, Inc. that hosts all email in Exchange Online. A user named User1 sends an email message to an Pass CISCO 300-115 exam - test questions external user User 1 discovers that the email message is delayed for two hours before being delivered. The external user sends you the message header of the delayed message You need to identify which host in the message path is responsible for the delivery delay. What should you do? A. Review the contents of the protocol logs. B. Search the message tracking logs. C. Search the delivery reports 200-355 exam for the message D. Review the contents of the application log E. Input the message header to the Exchange Remote Connectivity Analyzer Correct Answer: E QUESTION 11 You have an Exchange Server 2016 organization. The organization contains three Mailbox servers. The servers are configured as shown in the following table You have distribution group named Group1. Group1 contains three members. The members are configured as shown in the following table. You discover that when User1 sends email messages to Group1, all of the messages are delivered to EX02 first. You need to identify why the email messages sent to Group1 are sent to EX02 instead. What should you identify? A. EX02 is configured as an expansion server. B. The arbitration mailbox is hosted 300-320 exam on EX02.C. Site2 has universal group membership caching enabled. D. Site2 is configured as a hub site. Correct Answer: A
Home / Saúde / Síndrome de Barth

Síndrome de Barth

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Definição

A síndrome de Barth (BTHS) é um distúrbio genético grave ligado ao cromossomo X, que afeta principalmente homens.

O que é

A síndrome de Barth, ou BTHS, é um distúrbio genético raro, sério e às vezes fatal, que afeta homens.

Herdada da mãe, a síndrome de Barth altera o gene BTHS no cromossomo X e pode causar problemas no coração, na imunidade e na capacidade do macho de se desenvolver.

Seu início é tipicamente observado no nascimento ou em poucos meses após o nascimento.

O diagnóstico precoce da síndrome de Barth é complicado, mas crítico.

Acredita-se que a síndrome de Barth ocorra em aproximadamente um em cada 200.000 nascimentos, mas acredita-se que seja altamente sub-diagnosticada devido à sua natureza complicada.

As ferramentas diagnósticas incluem exames de sangue para determinar a contagem de glóbulos brancos, urinálise para determinar a presença de um aumento de ácido orgânico tipicamente observado com BTHS, um ecocardiograma ou ultrassonografia cardíaca para avaliar a estrutura e função ou condição do coração, e Análise de Sequenciação de DNA para determinar a anormalidade do gene BTHS.

Alguns sintomas graves da síndrome de Barth podem incluir cardiomiopatia ou defeitos cardíacos e fraqueza, neutropenia ou uma deficiência nos glóbulos brancos que diminui a capacidade do organismo de combater infecções e um aumento anormal de um ácido orgânico na urina.

Outros problemas que podem ocorrer incluem uma diminuição do apetite e diarreia frequente, o que pode levar a um ganho de peso lento, úlceras recorrentes, respiração difícil, fadiga extrema, dores de cabeça crônicas ou dores no corpo e distúrbios leves de aprendizado. Meninos que sofrem da síndrome de Barth podem demonstrar hipotonia ou mau tônus muscular e controle, e estatura encurtada ou corpo pequeno.

Osteoporose ou amolecimento dos ossos e hipoglicemia também podem ocorrer.

Complicações da síndrome de Barth incluem graves infecções bacterianas, como o corpo demonstra uma diminuição da capacidade de combater infecções e insuficiência cardíaca congestiva, o que prejudica a capacidade do organismo para fornecer fluxo sanguíneo suficiente para atender às necessidades do corpo e pode resultar em morte.

Não há tratamento especial ou cura para a síndrome de Barth, portanto, aliviar e controlar os sintomas é crucial.

O monitoramento constante dos sintomas e o tratamento dos sintomas cardíacos, infecções e problemas nutricionais à medida que ocorrem e alterando a dieta, se necessário, são ações comuns.

Ocasionalmente, a cirurgia pode corrigir defeitos cardíacos.

Com um bom monitoramento cardíaco ou cardíaco, acredita-se que pelo menos 75% dos pacientes apresentem uma melhora gradual, o que aumenta a chance de normalizar a função cardíaca geral.

A avaliação imediata e o tratamento de processos infecciosos também podem aumentar a expectativa de vida.

Descrição

A síndrome de Barth é uma condição rara caracterizada por um coração aumentado e enfraquecido (cardiomiopatia dilatada), fraqueza nos músculos usados para o movimento (miopatia esquelética), infecções recorrentes devido a um pequeno número de glóbulos brancos (neutropenia) e baixa estatura.

A síndrome de Barth ocorre quase exclusivamente no sexo masculino.

Nos homens com síndrome de Barth, a cardiomiopatia dilatada está freqüentemente presente ao nascimento ou se desenvolve nos primeiros meses de vida.

Com o tempo, o músculo cardíaco fica cada vez mais enfraquecido e é menos capaz de bombear o sangue.

Indivíduos com síndrome de Barth podem ter fibras elásticas no lugar de fibras musculares em algumas áreas do músculo cardíaco, o que contribui para a cardiomiopatia. Essa condição é chamada de fibroelastose endocárdica.

Isso resulta em espessamento do músculo e prejudica sua capacidade de bombear o sangue.

Em pessoas com a síndrome de Barth, os problemas cardíacos podem levar à insuficiência cardíaca.

Em casos raros, a cardiomiopatia melhora com o tempo e os indivíduos afetados eventualmente não apresentam sintomas de doença cardíaca.

Na síndrome de Barth, a miopatia esquelética, particularmente dos músculos mais próximos do centro do corpo (músculos proximais), geralmente é perceptível desde o nascimento e causa tônus muscular baixo (hipotonia).

A fraqueza muscular freqüentemente causa atraso nas habilidades motoras, como rastejar e caminhar. Além disso, os indivíduos afetados tendem a sentir cansaço extremo (fadiga) durante atividades físicas extenuantes.

A maioria dos homens com a síndrome de Barth tem neutropenia. Os níveis de glóbulos brancos podem ser consistentemente baixos (persistentes), podem variar de normais a baixos (intermitentes), ou podem alternar entre episódios regulares de normais e baixos (cíclicos). A neutropenia torna mais difícil para o corpo combater invasores estranhos, como bactérias e vírus, por isso os indivíduos afetados têm um risco aumentado de infecções recorrentes.

Recém-nascidos com a síndrome de Barth são geralmente menores que o normal, e seu crescimento continua lento ao longo da vida.

Alguns meninos com esta condição experimentam um surto de crescimento na puberdade e são de estatura média quando adultos, mas muitos homens com síndrome de Barth continuam a ter baixa estatura na vida adulta.

Homens com a síndrome de Barth freqüentemente apresentam características faciais distintas, incluindo bochechas proeminentes. Os indivíduos afetados normalmente têm inteligência normal, mas muitas vezes têm dificuldade em realizar tarefas que envolvem habilidades matemáticas ou visuais-espaciais, como quebra-cabeças.

Os homens com a síndrome de Barth apresentam níveis aumentados de uma substância chamada ácido 3-metilglutacônico no sangue e na urina. A quantidade do ácido não parece influenciar os sinais e sintomas da condição. A síndrome de Barth é parte de um grupo de distúrbios metabólicos que podem ser diagnosticados pela presença de níveis aumentados de ácido 3-metilglutacônico na urina (acidúria 3-metilglutacônica).

Embora a maioria das características da síndrome de Barth esteja presente no nascimento ou na infância, os indivíduos afetados podem não ter problemas de saúde até mais tarde na vida.

A idade em que os indivíduos com síndrome de Barth apresentam sintomas ou são diagnosticados varia muito. A gravidade dos sinais e sintomas entre os indivíduos afetados também é altamente variável.

Homens com síndrome de Barth têm uma expectativa de vida reduzida.

Muitas crianças afetadas morrem de insuficiência cardíaca ou infecção na infância ou na primeira infância, mas aquelas que vivem na idade adulta podem sobreviver até o final de seus 40 anos.

Fonte: ghr.nlm.nih.gov/rarediseases.info.nih.gov/www.wisegeek.org/www.barthsyndrome.org/www.childrenshospital.org/www.brainfacts.org/www.brainfacts.org

 

 

 

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QUESTION 1 You are managing a SharePoint farm in an Active Directory Domain Services (AD DS) domain. You need to configure a service account to use domain credentials. What should you do first? A. Configure the service account to use a domain user account. B. Register the domain credentials in the Farm Administrators group. C. Configure the service account to use a local user account. D. Register the domain credentials as a SharePoint managed account. Correct Answer: D Explanation QUESTION 2 A company named Contoso, Ltd. has a SharePoint farm. The farm has one Search service application configured. The Search service application contains five crawl databases, all of which contain content. Contoso plans to crawl knowledge base content from its partner company Litware, Inc. The knowledge base content must be stored in a dedicated crawl database.https://www.pass4itsure.com/az-103.html You need to configure crawling. What should you do? A. 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The domain\superuser account needs to have a User Policy set for that gives it Full Control to the entire web application. D: If you are using any type of claims based authentication you will need to use Windows PowerShell. And Windows PowerShell is the hipper more modern and sustainable option anyway. 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You need to minimize the amount of log data written to the disk. Which two actions should you perform? (Each correct answer presents part of the solution. Choose two.) A. Set the log event level to Information. B. Set the log event level to Verbose. C. Set the log trace level to Medium. D. Set the log trace level to Verbose. E. Set the log event level to Warning. F. Set the log trace level to Monitorable. Correct Answer: EF Explanation Explanation/Reference: E: Event Levels Warning, Level ID 50 Information, Level ID: 80 Verbose, Level ID: 100 F: Trace levels: Monitorable: 15 Medium: 50 Verbose: 100 Note: When using the Unified Logging System (ULS) APIs to define events or trace logs, one of the values you must supply is the ULS level. Levels are settings that indicate the severity of an event or trace and are also used for throttling, to prevent repetitive information from flooding the log files. 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