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VCS-352 Administration of Storage Foundation and HA 6.0 for Windows

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Test Code : VCS-352
Test name : Administration of Storage Foundation and HA 6.0 for Windows
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: 286 real Questions

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Veritas Administration of Storage Foundation

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Symantec this week introduced that it has greater three of its flagship products – Veritas Storage foundation, Veritas Cluster File equipment and Veritas Cluster Server – with assist for migrating statistics into and out of strong condition drives and the skill to reclaim unused storage. moreover, the trade has more suitable the failover capabilities of Veritas Cluster File system to travail with structured statistics in Oracle, Sybase or IBM DB2 applications.

Symantec calls 'SpywareGuard' and 'Antivirus' properly scareware

Veritas Storage groundwork now supports the non-disruptive current of facts from Tier 1 to Tier 0 strong condition drives. Its dynamic storage tiering know-how helps heterogeneous arrays.

To improve potential utilization, Symantec added what it calls the thin Reclamation API. This API may too live used with IBM XIV and 3PAR InServ Storage Servers to reclaim storage skill as it is decided to live unused. traditionally as applications write information, actual storage is allocated. brief information are created, records is modified or deleted, resulting in a file device size which is lower than the physical storage in the array. the skinny Reclamation API enables a number file gadget to intelligently talk with the array to reclaim house linked to file deletions. house reclamation makes spend of the 'write equal' SCSI command.

furthermore, Symantec has stronger the Cluster File system and Cluster Server with uphold and more suitable availability for Oracle environments. Cluster File device and Cluster Server now give concurrent access to information and require only the utility to live moved, resulting in failover of single-illustration databases in seconds.

The enhancements are available now.

This story, "Symantec Enhances Veritas Storage basis" turned into firstly published through community World.

Copyright © 2009 IDG Communications, Inc.

VERITAS Storage basis Dynamic Multi-Pathing alternative - ( v. 5.1 ) - fundamental protection train | real Questions and Pass4sure dumps

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Administration of Storage Foundation and HA 6.0 for Windows

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Build a Successful SharePoint Governance blueprint | real questions and Pass4sure dumps


Build a Successful SharePoint Governance Plan

Coming up with a viable governance strategy is faultfinding and requires a heedful balancing act.

Attend any SharePoint conference and you'll contemplate several sessions on the schedule highlighting the significance of SharePoint governance. Inevitably, speakers command a account that resonates with the vast universe of organizations that spend SharePoint. "We launched SharePoint, it grew infatuation an out-of-control wildfire, and now, they necessity to build that fire out," is the typical refrain. So it goes, organization after organization -- SharePoint governance was an afterthought. SharePoint governance is the "solution" after the problem is already out-of-hand, rather than the mechanism designed to halt the problem from transpiring in the first place.

Nearly half of those responding to a survey eventual year by the Association for Information and Information Management (AIIM) said it has become easier to find SharePoint governance resources. At the very time, 30 percent noiseless felt their organizations had "big issues" with governance and security in their SharePoint implementations.

The traditionally accepted top-down SharePoint governance model, however, is far less valuable in an era where it has evolved from a complex documents and records management platform, into a full-blown enterprise content management system complete with multi-dimensional sociable tools. The governance model, once simple -- though generally ignored by IT executives and SharePoint administrators -- now requires a heedful balancing act.

The prevalent SharePoint pyramid (see figure 1), where governance is tightly controlled at the top, and less controlled at the bottom, isn't an accurate depiction of the current struggles facing organizations with big SharePoint implementations including management of sociable collaboration, the bring your own device (BYOD) revolution and the common challenge of SharePoint site sprawl. A well-executed governance blueprint compares the various elements of governance and balances security with the current evolution of trade mobility (see figure 2).

[Click on image for larger view.] figure 1. The traditional top-down SharePoint governance model. In an era where sociable content and collaboration is continually important, this approach is no longer as effective. Source: Microsoft [Click on image for larger view.] figure 2. effectual SharePoint governance requires governance committees find a balance between different types of SharePoint content, including sociable content, in order to manage it more efficiently.

Why Bother?Governance plans are insurance. But, infatuation changing insurance providers, governance is a pain. It consumes resources, those who presumably already acquire full-time responsibilities. Also, it requires vigilance, dedication and a general commitment to excellence -- total of which their organizations strive to embody, but at a macro-level. The trickle-down upshot rarely reaches the SharePoint trenches. So why create total the fuss?

Consider the high-costs of implementing SharePoint -- some estimates hint that an organization of 3,000 employees will spend more than $4 million in SharePoint-related costs over a three-year period. Combine that with the fact that many organizations are turning to SharePoint as their enterprise content management (ECM) platform, which houses mission-critical and sensitive data, and suddenly an insurance policy designed to protect and potentially improve your investment isn't a terrible idea.

Steps for SuccessFirst, forget the phrase "successful SharePoint governance." Success implies completion -- and governance is never complete. "Governed SharePoint trade improvement" is a more accurate description of the perpetual cycle of improvement in a SharePoint implementation supported by a solid SharePoint governance blueprint and an dynamic governance committee.

Second, define measurable milestones for improvement. Without a transparent declaration of what constitutes improvement, even the most well-intentioned governance blueprint will Fall flat. Each policy and procedure outlined in a SharePoint governance blueprint should live a quantifiable milestone. For example, as SharePoint implementations become larger and less centralized, organizations struggle to "stop" trade units from placing duplicate content in sub-site after sub-site. This is a specific problem that can live addressed with sound governance policies and socialization.

In this example, success in governance can live demonstrated by continually measuring and benchmarking the number of duplicate documents in the system. As the number goes down, it becomes evident that the SharePoint governance policy addressing this issue is successful.

Governance committees should continually document and measure metrics to demonstrate the extent to which their governing process is improving the overall SharePoint enterprise.

Third, create a governance committee empowered to actually govern. According to the 2014 IT Priorities Survey from Protiviti Inc., while IT Executives visage significant competing priorities, nearly half of total organizations noiseless depend on IT to deploy, configure and launch SharePoint. Information governance and management programs rank in the top five among the significant priorities for CIOs and fellow IT executives -- topped only by the significance of IT project management, virtualization and cloud computing. SharePoint governance is only one aspect of the larger information governance puzzle, but given organization's commitments to collaborative technologies such as SharePoint, its significance can't live over-stressed.

SharePoint governance committees, the voice of the SharePoint governance plan, are not only comprised of representatives from the trade and IT sides of the organization, but too executives and organizational champions. Individuals, regardless of their position or role at an organization, who lead by example and are role models to their fellow colleagues, are invaluable assets in any SharePoint governance planning process.

Despite adherence to this mix-composition plan, many governance committees noiseless fail in their quest to draft and effectively implement SharePoint governance. The problem is three-fold. One, these governance committees aren't recognized as legitimate policy-making bodies in the organization. They're generally considered project-based committees, not governing bodies. The distinction is critical. Project committees live in silos; governing bodies acquire the uphold and infrastructure in set to palpate total aspects of the business. An effectual governance committee depends on its aptitude to design and, perhaps more important, enforce decisions and policies around total aspects of SharePoint technology and information. Second, they're not given the resources, both monetary and human, to socialize SharePoint governance via multiple channels. Last, participants aren't recognized as going above and beyond their typical job responsibilities to participate in an activity that's for the improvement of the entire organization.

"Often clients attain some of the things the best practices guidance recommends despite not having a governance team, but it is hit or miss and the misses gradually erode at the integrity of the project and the system," says SharePoint MVP Doug Hemminger.

Most governance committees suffer from at least one of these flaws, and perhaps can compensate for the shortcomings that arrive with the imperfection of any trade process, but any combination of these flaws can prove mortal to the best-intentioned committees.

The fourth step for success is to write appropriate, concise, thorough and useful content. Microsoft highlighted in a TechNet Library article the significance of a governance blueprint that distinguishes IT governance, application management and information management.

Governance plans will develope as an organization's SharePoint implementation matures. Likewise, governance blueprint content will acquire different points of stress at different organizations. For example, Office 365 and SharePoint online governance plans are less technical, but set a ponderous stress on sociable content and other collaborative tools available in the online SharePoint environment. In such cases, technical governance is less significant because Microsoft bears the cross of many of the technical governance decisions that an organization with an on-premises SharePoint solution would otherwise acquire to resolve.

Governance plans should include the following core elements:

  • Operational Management
  • Technical Operations
  • Site and Security Administration
  • Content Administration
  • Personal and sociable Administration
  • The extent to which each of the broad categories is defined will depend entirely on the maturity of an organization's SharePoint implementation.

    Operational Management: Operational management defines not only the membership of the governance committee, but more important, its role and responsibility to the larger SharePoint community. It defines total the roles and related responsibilities necessary to sprint an effectual SharePoint solution.

    Technical Operations: Technical operations contour the basic technical structures, requirements and quotas for the SharePoint environment. It references existing documents defining SharePoint architecture or existing IT policies such as uptime and backup requirements. It too defines the authentication mechanisms by which users access the site -- if necessary, defining in detail the differences between internal and external users and the processes for provisioning and de-provisioning access for users.

    Site and Security Administration: The aforementioned TechNet Library article too suggests, rightly so, different types of SharePoint sub-sites can, and likely should live governed in different ways. This makes for a potentially complex, but ultimately more thorough picture of content governance. Site and security administration articulates the spend cases for different site templates in SharePoint. It too dictates the procedures for the following:

  • Site provisioning
  • Site de-provisioning
  • Site ownership and responsibilities associated with ownership
  • Permissions structures and best practices
  • Different spend cases for Yammer groups vs. SharePoint sites
  • Content Administration: Content administration is typically the most difficult element to define for governance committees, largely because by the time governance is build in place, defining a vast wasteland of content is an overwhelming proposition. Information management is more easily addressed when the SharePoint implementation is rooted in a foundation of sound information architecture. Content administration covers a wide orbit of topics, including:

  • Defining the differences between business-critical and non-critical content
  • Creating guidelines for the spend of metadata, content types and workflows
  • Determining review cycles for content, and policies enforcing archiving and deleting of content
  • Legal considerations regarding document and content retention, and the associated retention policies
  • Administration and spend cases for lists, libraries, pages and different Web Parts

  • Recycle your stale tech gear | real questions and Pass4sure dumps

    In springtime, people’s fancies may whirl to love, but their to-do list turns to cleaning. design this the year that you finally recycle total the ancient MP3 players, toner cartridges, ethernet cables, and bulky monitors out of the closets, garages, and spare rooms where they’ve been lurking. Your home will feel more modern, and you’ll live doing your Part to boost the 27 percent electronics recycling rate in the U.S.—just in time for another spring constant, Earth Day.

    Half of total electronic blow is linked to consumer electronics. Why recycle at all?

    Sure, it would live easier just to dump total your old, unwanted electronic stuff in the trash. However, stale computers and their related peripherals hold a lot of ponderous metals—lead, cadmium, mercury—that are nasty for people’s health when they acquire into the soil and water. In addition, when stale electronics hit the trash heap, they’re out of a recycling stream that can crop the energy costs for production of future electronics.

    But if the environmental concerns don’t grab you, admiration the trade benefits to recycling electronics: According to the Institute for Local Self-Reliance, compared to disposal, computer reuse created 296 jobs per every 10,000 tons of material disposed of each year. The Electronics TakeBack Coalition estimates that the U.S. generates approximately 1.7 million tons of electronic blow annually—so imagine the job potential that’s noiseless there. And if those arguments don’t sway you, maybe the long arm of the law can: It’s illegal in 25 U.S. states to simply trash your stale electronics.

    Now, don’t you want to avoid breaking the law? Don’t you too want to employ people and maintain the planet cleanly by recycling your stale stuff? Of course you do, so let’s acquire started.

    Before you acquire rid of any of your electronics

    The first step on your recycling journey: design positive that no one of your personal data will live leaving the house along with your soon-to-be-discarded electronics. As a bare minimum, design positive you attain the following:

    Don't forget the puny things infatuation deauthorizing iTunes-running computers, for example.

    If you’re using Apple’s iTunes to manage your digital music and video, live positive you deauthorize any device you’re recycling. “But that computer is dead” is no excuse: Apple expects you to simply deauthorize total your devices via the iTunes app, then reauthorize the ones that are noiseless alive (and hanging around after the purge). Note that the “deauthorize all” option works only once a year, so spend it wisely.

    If you’re recycling a smartphone, attain a complete reset of your phone to wipe out its data and restore it to its factory settings. Before you attain this, however, design positive that you’ve got your data backed up someplace else, and that you’ve double-checked the manufacturer's instructions on how to reset your phone.

    If you’re recycling a computer or a hard drive, don’t just assume that deleting files will wipe the data off your system, because it won’t. total it does is reformat the space on the drive, and the data can live snagged using tools designed for cataclysm recovery.

    In 2003, graduate students at MIT were able to pull more than 5000 credit card numbers, personal and corporate monetary records, and medical records from so-called “wiped” drives

    You necessity to acquire a disk-wiping program, preferably one that meets the U.S. Department of Defense’s Media Sanitation Guidelines, and you necessity to set aside a few hours. What a well-behaved disk-wiping program will attain is write total sorts of data on your hard disk multiple times, effectively obscuring your original data with newer, nonpersonal stuff. This takes some time, so don’t procrastinate and leave it to the morning of your planned recycling run.

    Mac users looking for disk-wiping programs can spend Apple’s built-in Disk Utility, or they can cough up $25 for Mireth Technology’s ShredIt X. Windows users on a budget can cancel their drives using the free Eraser 6.0.10.

    If you’re tempted to skip this step, admiration this: In 2003, graduate students at MIT bought 158 used hard drives—approximately 60 percent of them reformatted and 45 percent with no files on them at all—and they were able to pull more 5000 credit card numbers, personal and corporate monetary records, and medical records from these “wiped” drives. While the odds of a graduate student at an elite technical university pulling your personal data off that stale MacBook and using it for evildoing are low, why not design them even lower? (Some people recommend physically drilling holes in hard-drive platters, but even that may not live enough.)

    If you’re recycling a digital camera and its attendant reminiscence card, you’ll necessity to wipe the card too. The best passage to attain this is to stick any reminiscence card back in the camera and spend the camera’s built-in “format card” option to wipe the reminiscence card cleanly of total data.

    If you’re getting rid of reminiscence sticks, USB scintillate drives, and other minute storage drives, reformat the drive, preferably with a disk-writing program. Remember, throwing out files doesn’t delete the data.

    If you’re getting rid of stale printers, scanners, or fax machines, you’ll necessity to purge the appliance’s memory. The well-behaved word is, most consumer-grade printers, scanners, and fax machines don’t acquire internal hard drives. (Most of the time, the document you’re printing or scanning is stored in RAM in the appliance’s hardware.) total you acquire to attain is disconnect your appliance from any computer, and then whirl it on and depart through the settings until you find the option to reset everything to factory settings. That should flush out any residual data or personal information infatuation your fax number.

    Now, on to recycling

    Broadly speaking, you acquire three recycling options: You can drop off your unwanted stuff in person somewhere; you can ship it away; or you can donate it to a willing organization.

    Drop it off in person. The odds to dropping off your gear is that you don’t necessity to really blueprint ahead and print out prepaid labels (as you would if you were shipping things away); you just toss your stuff in the car and go. Several national retail chains acquire set up recycling options for everything from batteries to toner cartridges to mobile phones. Below, some of the most ubiquitous:

    You can bring your stale iPod to any Apple Retail Store and acquire 10 percent off a new one. (Note: this does not apply to iPod shuffles).

    GreenpeaceIf you thought computers from the late 1990s were unsightly on the desktop, wait til you contemplate several thousand of them in a landfill.

    Dell has set up a printer recycling program in conjunction with the office supply chain Staples, where you can drop off Dell-branded used ink and toner cartridges at participating locations. The program is free. (Staples takes any brand of blank ink and toner cartridge through its own recycling program—and you’ll even acquire back $2 in Staples rewards for each cartridge you recycle.)

    Hewlett-Packard has too partnered with Staples as a drop-off point for its stale computing equipment and printer cartridges. Again, you can drop off your stale equipment for free.

    Acer works with Best Buy’s in-store recycling program. Check Acer’s website to contemplate what programs the computer manufacturer offers in your state.

    Some big chains acquire recycling programs as well. OfficeMax, for example, will occupy your stale printer/toner cartridges, as will Walgreens.

    Finally, who among us doesn’t acquire a ratking of cables that you’ve been holding on to since the Clinton administration? Happily, most electronics recycling places will occupy any and total cables you have, as well, because the cables acquire copper that can live stripped out. If you really want to brighten the day of whoever is stuck dealing with your cables, occupy the time to untangle them, sort them by size, and spend twist ties to design compact puny bundles of each cable.

    You’ve too got the option of dropping off your unwanted stuff at a local recycling center. LG, Sony, and Toshiba total provide location-based lookups for electronics recycling centers that occupy their wares. You can too find one through eStewards, or Greener Gadgets. When you attain find a center, convene ahead and examine the following questions:

  • Do you accept electronic devices from individuals, or attain you travail only with businesses?
  • What are your policies on destroying personal data that may noiseless live on used computers or cell phones?
  • Do you blow storage media?
  • Can I acquire a record of the methods used on my stale electronics?
  • What environmental management guidelines attain you follow?
  • What percentage of the materials you collect are recycled and what percentage is disposed?
  • What fees will you freight for disposing of computer monitors? Televisions? Or attain you freight a flat fee for a carload of items? (The first time I used a recycling center, 11 years ago, I paid $35 to acquire rid of two laptops. That undergo made me value the free programs set up by manufacturers.)
  • A local recycler-as-fundraiser program typically takes the kinds of minute electronics create around the house, but won't occupy televisions or other appliances.

    A third option that’s cropping up across the U.S. is the electronics recycling fundraiser. You’ll often contemplate schools or civic groups doing these, where a coordinating group, such as a middle-school orchestra's booster club, charges a nominal fee of $25 or so for total the electronics you keeping to drop off. The odds here is that you don’t acquire to depart chasing total over the set to drop off your stuff. The handicap is that Lincoln Middle School’s first clarinet chair may attain a distinguished job hustling your stale printer cartridges out of your car trunk, but she probably can’t command you whether there’s a decent data-management policy in set at the recycling endpoint.

    Ship it away. The Web is chockablock with sites that engage to pay you cash money for your stale junk. Some manufacturers and retailers acquire gotten in on the game too. Apple, for example—working in conjunction with PowerOn Services—promises a gift card with whatever cash value your stale computers, iPhones, or iPads had. (And if your gear doesn’t qualify, it’ll recycle them for you at no charge.)

    Apple will occupy total sorts of your stale gear.

    Other tech vendors provide free shipping and hassle-free recycling: Dell’s mailback program provides a prepaid mailing label and a pickup by FedEx to whisk away your stale PCs. Motorola will foot the bill if you want to send it any brand of mobile phone, tablet, or accessory and it’ll recycle its branded cordless phones, modems, and routers for free.

    Other major brands that will provide prepaid mailing labels to recycle their stale products include BlackBerry, Hewlett-Packard, Lenovo, and Verizon Wireless.

    If you just want to toss everything in one giant box and never believe of it again, check out the services GreenDisk offers. It’ll recycle almost anything (but draws the line at electric toothbrushes). Note that this convenience does not arrive free—getting rid of your Jurassic monitor starts at $35.

    Donate it. If your older gear is noiseless in working order, you may want to believe about giving it away instead of sending it on its passage to its next life. But before you design plans to gift your local preschool with a Performa 6115, halt and admiration whether your donation is going to live useful to any given organization. Phones, computers, and printers that don’t travail aren’t going to magically fire up once they’ve been tossed in the Goodwill bin; if you can recycle them on your own, attain it and reclaim the folks at the nonprofits the effort.

    If you acquire relatively modern, working electronics and you want to acquire them into the hands of people who can spend them, start with the Cristina Foundation, which matches donors of used computers and other electronic hardware to select a local charity or school in their locality of the country. Another organization to admiration is the World Computer Exchange, which sells low-cost refurbished desktop computers and peripherals available to developing countries.

    There’s too a crop of charities seeking to pass along mobile phones to people who desperately necessity them—groups such as the National Coalition Against Domestic Violence, HopeLine, Cellphones for Soldiers, Operation Gratitude, Hope Phones, Secure the Call, and the 911 Cell Phone Bank.

    And maintain in mind that if you’re giving to a registered nonprofit organization, you may acquire some tax benefits from your donation (you’ll necessity a receipt with the company’s Tax ID on it).

    This story, "Recycle your stale tech gear" was originally published by TechHive.

    To remark on this article and other PCWorld content, visit their Facebook page or their Twitter feed.

    Enzyme-Replacement Therapy in Mucopolysaccharidosis I | real questions and Pass4sure dumps

    All patients received weekly infusions of recombinant human α-L-iduronidase for 52 weeks. The matter activity of α-L-iduronidase in leukocytes was 0.04 U per milligram before treatment and 4.98 U per milligram, or 15 percent of normal, an dispassionate of seven days after an infusion of α-L-iduronidase (i.e., immediately before the next infusion). Enzyme activity was not detectable in brushings of buccal mucosa before treatment, but seven days after an infusion, it reached a flat of 1 percent of normal.

    Five patients (Patients 1, 4, 5, 6, and 7) had transient urticaria on the trunk, face, arms, and legs during an infusion given during week 4 or later, and in four patients (Patients 1, 5, 6, and 7) it recurred during subsequent infusions dawn at or after week 20. During these episodes, urticaria began midway through an infusion but resolved soon after the infusion was completed. In the four patients with recurrent urticaria, the episodes eventually became less frequent and less austere and finally stopped. In three patients (Patients 1, 5, and 6), the urticaria was accompanied by angioedema (thickening of the tongue and tightening of the throat) on a total of nine occasions and by qualify transient hypoxemia on three occasions. Patient 8 had one episode of facial swelling without urticaria during the infusion at week 47. These symptoms usually resolved about one hour after the infusion was stopped. In patients with recurrent urticaria, the rate of enzyme infusion was decreased or the dose temporarily reduced, and they were given increased medications such as diphenhydramine before, and in some cases during, an infusion.

    Four patients (Patients 2, 7, 8, and 9) had biochemical evidence of complement activation during infusions given at weeks 6 and 12, as evidenced by decreased serum total complement activity (i.e., decreased CH100) and decreased C3 or C4 concentrations after infusions. Although these patients were usually asymptomatic, Patient 8 had fever, chills, and “fussy behavior” on three occasions. By week 26, there was no complement activation during infusion in any patient. In the four patients who had transient complement activation, serum α-L-iduronidase antibodies were detected by week 8, but the antibody levels subsequently declined or became undetectable. The antibodies did not immunoprecipitate autochthonous enzyme and did not inhibit enzyme activity in vitro (Anand VA, Kakkis ED: unpublished data), nor did they alter efficacy in vivo on the basis of urinary glycosaminoglycan excretion. In total 10 patients IgG antibodies developed to Chinese-hamster-ovary cell proteins that were present as a vestige impurity in the enzyme preparation, but clinically necessary adverse events were not correlated with the presence or titer of these antibodies. There were no abnormalities in blood counts, serum chemical values, or urinalysis during treatment. Other mucopolysaccharidosis-related complications during treatment consisted of cervical subluxation requiring cervical fusion, mitral-valve replacement with coronary bypass grafting, repeated lumbar fusion, and a ventriculoperitoneal shunt, each in one patient.

    Reduction in Lysosomal Storage Figure 1. Figure 1. Changes in Liver Size in Patients with Mucopolysaccharidosis I during α-l-Iduronidase Therapy.

    Liver size was measured in terms of volume and expressed as the percentage of carcass weight, given a density of 1 g per milliliter of tissue. Patient 9 had an episode of hepatitis at 26 weeks that was believed to live due to a concomitantly taken medication and that resolved by week 30. This episode was thought to account for the transient augment in the size of her liver. The upper bounds of the 95 percent assurance interval of unvarying values (i.e., within the unvarying orbit for age, as adapted from the data of Stocker and Dehner23) are 3.5 percent for boys 5 to 12 years of age, 3.2 percent for girls 5 to 12 years of age, 2.2 percent for boys 13 to 17 years of age, 2.7 percent for girls 13 to 17 years of age, 2.6 percent for men 18 years of age or older, and 2.9 percent for women 18 years of age or older.

    Liver volume decreased by 19 to 37 percent from foundation line in nine patients and by 5 percent in one patient at 52 weeks; the matter lessen was 25 percent (P<0.001). By 26 weeks, the size of the liver was unvarying for carcass weight and age in eight patients (Figure 1). In the two patients (Patients 6 and 9) who had the largest liver size relative to carcass weight at foundation line, the size of the liver was nearby to unvarying at 52 weeks (3.2 and 3.3 percent of carcass weight, respectively).

    In eight patients the size of the spleen decreased by 13 to 42 percent from foundation line. The matter lessen was 20 percent among total 10 patients (P<0.001).

    Urinary glycosaminoglycan excretion declined rapidly after 3 to 4 weeks of treatment, and by 8 to 12 weeks it was 60 to 80 percent below the base-line values. At 52 weeks, the matter reduction was 63 percent (range, 53 to 74 percent; P<0.001). The excess urinary glycosaminoglycan excretion (that above the upper limit of the unvarying value for age) was reduced by a matter of 80 percent in these patients. The results were confirmed by an assay of uronic acids and N-sulfate (a test specific for heparan sulfate). Electrophoretic studies of urine revealed a significant reduction in the excretion of heparan sulfate and dermatan sulfate, but the excretion of dermatan sulfate was noiseless greater than unvarying in total patients.

    Clinical Studies Increases in Height and Weight Table 2. Table 2. Height and Weight of Six Prepubertal Patients before Enzyme-Replacement Therapy and after One Year of Therapy.

    The height increased by a matter of 6.0 cm (5 percent) in the six prepubertal patients, and their matter rate of growth in height increased from 2.80 cm per year to 5.17 cm per year during treatment (P=0.01) (Table 2). For total 10 patients, carcass weight increased by a matter of 3.2 kg (9 percent), and the matter augment was 4.2 kg (17 percent) among the 6 prepubertal patients. In these six patients, the matter rate of growth in weight increased from 1.66 kg per year before treatment to 3.83 kg per year during treatment (P=0.04) (Table 2).

    Range of Motion Figure 2. Figure 2. matter Changes in the Restriction of orbit of Motion of Shoulder Flexion (Panel A), Elbow Extension (Panel B), and Knee Extension (Panel C) in Patients with Mucopolysaccharidosis I during α-l-Iduronidase Therapy.

    The matter degrees of restriction in the orbit of motion of right- and left-shoulder flexion are not shown for two patients, because shoulder flexion was not evaluated in these two patients at foundation line. The values depict the contrast between the unvarying maximal orbit of motion for age and the measured value.

    Restriction of shoulder flexion decreased during treatment in six of the eight patients in whom it was evaluated at foundation line. The matter lessen in joint restriction was 28 degrees in the privilege shoulder (P< 0.001) and 26 degrees in the left shoulder (P=0.002) (Figure 2A). Among total 10 patients, the degree of restriction of elbow extension decreased by a matter of 7.0 degrees in the privilege elbow (P=0.03) and 7.0 degrees in the left elbow (P=0.007) (Figure 2B). The degree of restriction of knee extension decreased by a matter of 3.2 degrees on the privilege (P=0.10) and 3.0 degrees on the left (P=0.09) in the 10 patients (Figure 2C).

    Analysis in individual patients revealed that the joints with the greatest degree of restriction before treatment had the greatest improvement. For example, at foundation line, Patients 5, 9, and 10 could not flex their shoulders beyond 100 degrees, and the orbit of motion increased by 21 to 51 degrees after treatment. The improvements in the orbit of motion were accompanied by patient-reported increases in physical activities, such as being able to wash their hair, hold a hamburger normally, hang from monkey bars, and play sports better.

    Airway Function

    Seven of the 10 patients had apnea, and these 7 had a lessen in the number of episodes of apnea and hypopnea during treatment, from 155 per night to 60 per night (a 61 percent decrease), with a change in the matter apnea–hypopnea index from 2.1 to 1.0 event per hour. Three of these seven (Patients 2, 6, and 9) had clinically necessary sleep apnea, and in total three this disorder improved during treatment. In Patient 2, the apnea–hypopnea index decreased from 4.5 events per hour at foundation line to 0.4 event per hour at 26 weeks, and the total length of time during sleep in which oxygen desaturation fell below 89 percent decreased from 48 minutes to 1 minute per night. At foundation line, Patient 6 required continuous positive airway pressure at night because of austere desaturation (with continuous positive airway pressure, the oxygen saturation was below 89 percent during 61 of 368 minutes of sleep). After 52 weeks of treatment, oxygen saturation was less than 89 percent for only 8 of 332 minutes of sleep, and continuous positive airway pressure was not used. Patient 9 had an apnea–hypopnea index of 9.5 events per hour before treatment and 4.0 events per hour after 26 weeks of treatment. In Patient 8, the initial apnea–hypopnea index of 0.1 event per hour increased to 3.1 events per hour at 26 weeks and to 9.3 events per hour at 52 weeks for reasons that were unclear. Eight of 10 patients or their families reported that their breathing had improved, and 5 of 7 reported quieter nighttime breathing, an improved attribute of sleep, and decreased daytime somnolence.

    Cardiac Function Figure 3. Figure 3. Changes in New York Heart Association (NYHA) Functional Class in Patients with Mucopolysaccharidosis I during α-l-Iduronidase Therapy.

    The changes in scores were based on information obtained from serial interviews with the patients. New York Heart Association class I indicates no symptoms with ordinary activity; class II, symptoms with ordinary activity and a slight limitation of activity; class III, symptoms with less-than-ordinary activity and marked limitation of activity; and class IV, symptoms with any ilk of activity or at rest. The contrast between pretreatment scores and scores at 52 weeks was significant (P=0.002).

    The New York Heart Association functional classification was determined by serial interviews with the patients. total 10 patients reported an improvement by one or two classes (Figure 3), but there was no objective data from echocardiographic studies to verify direct cardiac benefit. The improved functional scores may reflect improvements in aspects of the disease other than cardiac function. When base-line echocardiograms were compared with those obtained after 52 weeks of treatment, tricuspid regurgitation or pulmonic regurgitation was decreased in four patients, but mitral regurgitation worsened in two patients (Patients 2 and 7). At foundation line, Patient 6 had atrial flitter and clinical signs of cardiac failure, including dyspnea at rest and pitting edema. After 12 weeks of treatment, he had sinus meter with first-degree shroud and his dyspnea at rest and pitting edema had resolved.

    Symptomatic Changes

    Before treatment, total 10 patients reported a want of endurance and limitations in their aptitude to execute daily activities, but exercise tolerance was not formally tested. During treatment, total patients had improved endurance and fewer limitations in their aptitude to execute daily activities, and after 26 weeks of treatment, many were able to walk farther, run, and play sports. Patients 3, 4, and 5 reported the resolution of severe, incapacitating headaches after 6 to 12 weeks of treatment.

    Ophthalmic Changes

    The extent of corneal clouding did not change in any of the eight patients with this problem. Several patients reported decreased photophobia or conjunctival irritation. Visual acuity improved from 20/1000 to 20/200 (in one eye) in one patient and slightly in two others.

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