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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 / Biologia / Aminoacidúria

Aminoacidúria

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Definição

aminoacidúria é uma quantidade anormal de aminoácidos na urina. Os aminoácidos são os blocos de construção das proteínas do corpo.

aminoacidúria é uma condição na qual um ou mais aminoácidos são excretados em quantidades excessivas

É uma característica clínica do distúrbio genético chamado erro inato do metabolismo de aminoácidos.

Os aminoácidos são excretados em condições como:

1. Fenilcetonúria
2. Alcaptonúria
3. Homocistinúria
4. Cistinúria

Um aminoácido é convertido em outro aminoácido na presença de uma enzima. Nessa condição, o gene que codifica essa enzima em particular está ausente desde o nascimento como um distúrbio genético.

Por exemplo, na fenilcetonúria, a fenilalanina é convertida em tirosina pela enzima fenilalanina hidroxilase. Esta enzima não é produzida em indivíduos fenilcetonúricos, de modo que a fenilalanina se acumula e é excretada na urina. Assim causando aminoacidúria.

Aminoacidúria – Urina

aminoacidúria ocorre quando a urina contém quantidades anormalmente elevadas de aminoácidos.

No rim saudável, os glomérulos filtram todos os aminoácidos para fora do sangue, e os túbulos renais então reabsorvem mais de 95% dos aminoácidos filtrados de volta ao sangue.

Na aminoacidúria por transbordamento, concentrações anormalmente altas de aminoácidos no plasma sanguíneo sobrecarregam a capacidade de reabsorção dos túbulos renais, resultando em altas concentrações de aminoácidos na urina. Isso pode ser causado por distúrbios congênitos do metabolismo de aminoácidos, por exemplo, fenilcetonúria. ou pode ser secundário a doença hepática.

Na aminoacidúria renal, os túbulos renais são incapazes de reabsorver os aminoácidos filtrados de volta ao sangue, causando altas concentrações de aminoácidos na urina.

Isso pode ser causado por um defeito nas proteínas de transporte no túbulo renal, por exemplo, como ocorre na doença de Hartnup, ou pode ser devido a danos no túbulo renal, por exemplo, como ocorre na síndrome de Fanconi.

O que é Aminoacidúria?

aminoacidúria é uma condição em que há uma quantidade excessiva de aminoácidos na urina devido a defeitos genéticos nas vias do metabolismo de aminoácidos.

Uma deficiência em uma enzima que resulta em um defeito no metabolismo de aminoácidos é referida como aminoacidúria primária.

Os defeitos nos transportadores moleculares responsáveis pelo transporte e absorção de um aminoácido são classificados como aminoacidúria secundária.

Ambos os tipos de aminoacidúria podem ser hereditários, principalmente em um padrão autossômico recessivo, mas alguns podem ser adquiridos secundariamente a várias doenças, como hiperparatireoidismo, mieloma múltiplo, osteomalácia, raquitismo e hepatite viral.

Alguns exemplos comuns de aminoacidúria primária hereditária são fenilcetonúria clássica, homocistinúria clássica e alcaptonúria. A fenilcetonúria clássica é caracterizada por uma concentração aumentada de fenilalanina e seus subprodutos nos tecidos, plasma e urina devido à deficiência de fenilalanina hidroxilase. Os achados característicos da fenilcetonúria clássica em uma criança não tratada incluem retardo mental, falha em atingir metas iniciais de desenvolvimento, microcefalia, hipopigmentação da pele e do cabelo, convulsões, tremor, hiperatividade e incapacidade de crescimento. A prevenção desses achados em uma criança pode ser feita pelo diagnóstico precoce e início do tratamento dietético antes das 3 semanas de idade.

A homocistinúria clássica é caracterizada por uma concentração aumentada de homocisteína e metionina e uma concentração diminuída de cisteína no plasma e na urina. Isso resulta da redução da atividade da beta-sintase da cistationina.

Indivíduos afetados apresentam o deslocamento de lentes ópticas conhecidas como ectopia lentis, retardo mental, anormalidades esqueléticas, osteoporose e doença arterial prematura. O tratamento consiste em restrição alimentar de proteína e metionina e suplementação com vitaminas B 6, B 12 e folato.

A alcaptonúria é caracterizada por uma concentração aumentada de ácido homogentísico na urina e tecidos conjuntivos devido a uma deficiência de ácido oxidase homogentísico.

Indivíduos afetados são geralmente assintomáticos até os 30 ou 40 anos.

Os três sintomas característicos da alcaptonúria são a presença de urina escura, grande artrite articular e escurecimento das orelhas e outros tecidos cartilaginosos e colágenos.

A prevenção de complicações a longo prazo pode ser feita através da restrição da dieta protéica, especialmente em fenilalanina e tirosina, juntamente com o uso da droga nitisona.

Aminoacidúria
A aminoacidúria dibásica é caracterizada por um comprometimento da função renal

Alguns exemplos comuns de aminoacidúria secundária hereditária são cistinúria, aminoacidúria dibásica e doença de Hartnup.

A cistinúria, devido ao defeito do transportador no rim e no intestino delgado, é caracterizada por reabsorção prejudicada e excreção excessiva de aminoácidos dibásicos cistina, arginina, lisina e ornitina na urina.

A baixa solubilidade da cistina predispõe à formação de cálculos renais, ureteral e vesical, que podem levar à insuficiência renal.

O objetivo do tratamento é evitar a formação de cálculos por diurese alcalina ao longo da vida. Dependendo da apresentação dos indivíduos afetados, o uso de penicilamina e tiopronina, litotripsia por ondas de choque, ureteroscopia, nefrolitotomia percutânea ou cirurgia urológica aberta podem ser considerados.

Aminoacidúria
Os sintomas de aminoacidúria dibásica podem incluir um fígado aumentado

aminoacidúria dibásica é caracterizada por um defeito seletivo na reabsorção de arginina, lisina e ornitina. Os indivíduos afetados podem apresentar aumento do fígado, intolerância a proteínas, hiperamonemia, comprometimento da função renal, osteoporose grave ou alterações estruturais nos pulmões. O tratamento consiste em restrição na dieta protéica e suplementação de citrulina.

A doença de Hartnup é caracterizada por manifestações neurológicas variáveis, como ataxia cerebelar ou delirium, acompanhadas por lesões cutâneas tipo pelagra. Isto é devido a um defeito em um transportador localizado nos rins e intestinos, resultando em um aumento da excreção urinária de alanina, treonina, leucina, isoleucina, asparagina, glutamina, histidina, serina, tirosina, valina, triptofano e fenilalanina. O tratamento inclui uma dieta rica em proteínas e suplementação de nicotinamida.

Fonte: www.indiastudychannel.com/medlineplus.gov/www.wisegeek.org/www.sciencedirect.com/www.ucsfbenioffchildrens.org/www.nicklauschildrens.org

 

 

 

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