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

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VCS-352 exam Dumps Source : Administration of Storage Foundation and HA 6.0 for Windows

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

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Veritas Administration of Storage Foundation

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How collaboration apps foster digital transformation

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Symantec this week introduced that it has greater three of its flagship products – Veritas Storage foundation, Veritas Cluster File materiel and Veritas Cluster Server – with assist for migrating statistics into and out of stalwart situation drives and the skill to reclaim unused storage. moreover, the business has more suitable the failover capabilities of Veritas Cluster File system to work 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 stalwart situation 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 besides 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 exercise 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 succession | true 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 Plan

Coming up with a viable governance strategy is captious and requires a observant balancing act.

Attend any SharePoint conference and you'll see several sessions on the schedule highlighting the import of SharePoint governance. Inevitably, speakers construe a fable that resonates with the vast universe of organizations that exercise SharePoint. "We launched SharePoint, it grew devotion an out-of-control wildfire, and now, they exigency to attach 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 discontinue the problem from transpiring in the first place.

Nearly half of those responding to a survey last year by the Association for Information and Information Management (AIIM) said it has become easier to find SharePoint governance resources. At the same time, 30 percent still 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 tangled documents and records management platform, into a full-blown enterprise content management system complete with multi-dimensional gregarious tools. The governance model, once simple -- though generally ignored by IT executives and SharePoint administrators -- now requires a observant balancing act.

The prevalent SharePoint pyramid (see design 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 gregarious collaboration, the bring your own device (BYOD) revolution and the common challenge of SharePoint site sprawl. A well-executed governance artery compares the various elements of governance and balances security with the current evolution of business mobility (see design 2).

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

Why Bother?Governance plans are insurance. But, devotion changing insurance providers, governance is a pain. It consumes resources, those who presumably already gain full-time responsibilities. Also, it requires vigilance, dedication and a general commitment to excellence -- everyone of which their organizations strive to embody, but at a macro-level. The trickle-down result rarely reaches the SharePoint trenches. So why create everyone 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 business improvement" is a more accurate description of the perpetual cycle of improvement in a SharePoint implementation supported by a solid SharePoint governance artery and an active governance committee.

Second, define measurable milestones for improvement. Without a clear declaration of what constitutes improvement, even the most well-intentioned governance artery will topple flat. Each policy and procedure outlined in a SharePoint governance artery should live a quantifiable milestone. For example, as SharePoint implementations become larger and less centralized, organizations struggle to "stop" business 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 kisser significant competing priorities, nearly half of everyone organizations still faith 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 import can't live over-stressed.

SharePoint governance committees, the voice of the SharePoint governance plan, are not only comprised of representatives from the business and IT sides of the organization, but besides executives and organizational champions. Individuals, regardless of their position or role at an organization, who lead by instance 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 still 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 gain the uphold and infrastructure in position to paw everyone aspects of the business. An effectual governance committee depends on its capacity to invent and, perhaps more important, implement decisions and policies around everyone aspects of SharePoint technology and information. Second, they're not given the resources, both financial 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 finish 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 near with the imperfection of any business process, but any combination of these flaws can prove deadly 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 import of a governance artery that distinguishes IT governance, application management and information management.

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

Governance plans should include the following core elements:

  • Operational Management
  • Technical Operations
  • Site and Security Administration
  • Content Administration
  • Personal and gregarious 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 everyone the roles and related responsibilities necessary to sprint an effectual SharePoint solution.

    Technical Operations: Technical operations outline 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 besides 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 besides 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 exercise cases for different site templates in SharePoint. It besides dictates the procedures for the following:

  • Site provisioning
  • Site de-provisioning
  • Site ownership and responsibilities associated with ownership
  • Permissions structures and best practices
  • Different exercise 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 attach 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 sweep of topics, including:

  • Defining the differences between business-critical and non-critical content
  • Creating guidelines for the exercise 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 exercise cases for lists, libraries, pages and different Web Parts

  • Recycle your used tech gear | true questions and Pass4sure dumps

    In springtime, people’s fancies may turn to love, but their to-do list turns to cleaning. invent this the year that you finally recycle everyone 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 piece to boost the 27 percent electronics recycling rate in the U.S.—just in time for another spring constant, Earth Day.

    Half of everyone electronic waste is linked to consumer electronics. Why recycle at all?

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

    But if the environmental concerns don’t grab you, regard the business 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 waste annually—so imagine the job potential that’s still 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 used electronics.

    Now, don’t you want to avoid breaking the law? Don’t you besides want to employ people and withhold the planet antiseptic by recycling your used stuff? Of course you do, so let’s pick up started.

    Before you pick up rid of any of your electronics

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

    Don't forget the exiguous things devotion deauthorizing iTunes-running computers, for example.

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

    If you’re recycling a smartphone, finish a complete reset of your phone to wipe out its data and restore it to its factory settings. Before you finish this, however, invent certain 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. everyone it does is reformat the space on the drive, and the data can live snagged using tools designed for disaster recovery.

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

    You exigency to pick up a disk-wiping program, preferably one that meets the U.S. Department of Defense’s Media Sanitation Guidelines, and you exigency to set aside a few hours. What a valid disk-wiping program will finish is write everyone 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 exercise Apple’s built-in Disk Utility, or they can cough up $25 for Mireth Technology’s ShredIt X. Windows users on a budget can delete their drives using the free Eraser 6.0.10.

    If you’re tempted to skip this step, regard 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 financial 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 used MacBook and using it for evildoing are low, why not invent 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 memory card, you’ll exigency to wipe the card too. The best artery to finish this is to stick any memory card back in the camera and exercise the camera’s built-in “format card” option to wipe the memory card antiseptic of everyone data.

    If you’re getting rid of memory sticks, USB sparkle 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 used printers, scanners, or fax machines, you’ll exigency to purge the appliance’s memory. The valid word is, most consumer-grade printers, scanners, and fax machines don’t gain internal hard drives. (Most of the time, the document you’re printing or scanning is stored in RAM in the appliance’s hardware.) everyone you gain to finish is disconnect your appliance from any computer, and then turn it on and retract through the settings until you find the option to reset everything to factory settings. That should flush out any residual data or personal information devotion your fax number.

    Now, on to recycling

    Broadly speaking, you gain 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 advantage to dropping off your gear is that you don’t exigency to really artery 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 gain set up recycling options for everything from batteries to toner cartridges to mobile phones. Below, some of the most ubiquitous:

    You can bring your used iPod to any Apple Retail Store and pick up 10 percent off a newfangled one. (Note: this does not apply to iPod shuffles).

    GreenpeaceIf you thought computers from the late 1990s were homely on the desktop, wait til you see 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 bare ink and toner cartridge through its own recycling program—and you’ll even pick up back $2 in Staples rewards for each cartridge you recycle.)

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

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

    Some big chains gain recycling programs as well. OfficeMax, for example, will purchase your used printer/toner cartridges, as will Walgreens.

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

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

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

    A third option that’s cropping up across the U.S. is the electronics recycling fundraiser. You’ll often see 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 everyone the electronics you faith to drop off. The advantage here is that you don’t gain to retract chasing everyone over the position to drop off your stuff. The disadvantage is that Lincoln Middle School’s first clarinet chair may finish a mighty job hustling your used printer cartridges out of your car trunk, but she probably can’t construe you whether there’s a decent data-management policy in position at the recycling endpoint.

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

    Apple will purchase everyone sorts of your used 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 used 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 used products include BlackBerry, Hewlett-Packard, Lenovo, and Verizon Wireless.

    If you just want to toss everything in one giant box and never regard 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 near free—getting rid of your Jurassic monitor starts at $35.

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

    If you gain relatively modern, working electronics and you want to pick up them into the hands of people who can exercise 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 belt of the country. Another organization to regard is the World Computer Exchange, which sells low-cost refurbished desktop computers and peripherals available to developing countries.

    There’s besides a crop of charities seeking to pass along mobile phones to people who desperately exigency 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 withhold in reason that if you’re giving to a registered nonprofit organization, you may pick up some tax benefits from your donation (you’ll exigency a receipt with the company’s Tax ID on it).

    This story, "Recycle your used 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 | true questions and Pass4sure dumps

    All patients received weekly infusions of recombinant human α-L-iduronidase for 52 weeks. The imply 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 tolerable 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 flush of 1 percent of normal.

    Five patients (Patients 1, 4, 5, 6, and 7) had momentary 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 genesis 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 stern 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 momentary 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 momentary 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 indigenous 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 everyone 10 patients IgG antibodies developed to Chinese-hamster-ovary cell proteins that were present as a trail impurity in the enzyme preparation, but clinically Important 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 corpse 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 momentary increase in the size of her liver. The upper bounds of the 95 percent assurance interval of typical values (i.e., within the typical sweep 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 groundwork line in nine patients and by 5 percent in one patient at 52 weeks; the imply abate was 25 percent (P<0.001). By 26 weeks, the size of the liver was typical for corpse weight and age in eight patients (Figure 1). In the two patients (Patients 6 and 9) who had the largest liver size relative to corpse weight at groundwork line, the size of the liver was immediate to typical at 52 weeks (3.2 and 3.3 percent of corpse weight, respectively).

    In eight patients the size of the spleen decreased by 13 to 42 percent from groundwork line. The imply abate was 20 percent among everyone 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 imply reduction was 63 percent (range, 53 to 74 percent; P<0.001). The excess urinary glycosaminoglycan excretion (that above the upper confine of the typical value for age) was reduced by a imply 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 still greater than typical in everyone 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 imply of 6.0 cm (5 percent) in the six prepubertal patients, and their imply 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 everyone 10 patients, corpse weight increased by a imply of 3.2 kg (9 percent), and the imply increase was 4.2 kg (17 percent) among the 6 prepubertal patients. In these six patients, the imply 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. imply Changes in the Restriction of sweep 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 imply degrees of restriction in the sweep 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 groundwork line. The values limn the disagreement between the typical maximal sweep 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 groundwork line. The imply abate in joint restriction was 28 degrees in the birthright shoulder (P< 0.001) and 26 degrees in the left shoulder (P=0.002) (Figure 2A). Among everyone 10 patients, the degree of restriction of elbow extension decreased by a imply of 7.0 degrees in the birthright 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 imply of 3.2 degrees on the birthright (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 groundwork line, Patients 5, 9, and 10 could not flex their shoulders beyond 100 degrees, and the sweep of motion increased by 21 to 51 degrees after treatment. The improvements in the sweep 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 abate 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 imply apnea–hypopnea index from 2.1 to 1.0 event per hour. Three of these seven (Patients 2, 6, and 9) had clinically Important sleep apnea, and in everyone three this disorder improved during treatment. In Patient 2, the apnea–hypopnea index decreased from 4.5 events per hour at groundwork 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 groundwork line, Patient 6 required continuous positive airway pressure at night because of stern 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 character of sleep, and decreased daytime somnolence.

    Cardiac Function Figure 3. Figure 3. Changes in newfangled 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. newfangled 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 sort of activity or at rest. The disagreement between pretreatment scores and scores at 52 weeks was significant (P=0.002).

    The newfangled York Heart Association functional classification was determined by serial interviews with the patients. everyone 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 groundwork line, Patient 6 had atrial flitter and clinical signs of cardiac failure, including dyspnea at repose and pitting edema. After 12 weeks of treatment, he had sinus cadence with first-degree screen and his dyspnea at repose and pitting edema had resolved.

    Symptomatic Changes

    Before treatment, everyone 10 patients reported a lack of endurance and limitations in their capacity to effect daily activities, but exercise tolerance was not formally tested. During treatment, everyone patients had improved endurance and fewer limitations in their capacity to effect 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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