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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 Real 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 equipment and Veritas Cluster Server – with assist for migrating statistics into and out of strong state 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 flow of facts from Tier 1 to Tier 0 strong state 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 also be used with IBM XIV and 3PAR InServ Storage Servers to reclaim storage skill as it is decided to be 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 use of the 'write equal' SCSI command.

furthermore, Symantec has stronger the Cluster File system and Cluster Server with support 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 be 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 series | killexams.com 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 Plan | killexams.com real questions and Pass4sure dumps

In-Depth

Build a Successful SharePoint Governance Plan

Coming up with a viable governance strategy is critical and requires a careful balancing act.

Attend any SharePoint conference and you'll see several sessions on the schedule highlighting the importance of SharePoint governance. Inevitably, speakers tell a story that resonates with the vast universe of organizations that use SharePoint. "We launched SharePoint, it grew like an out-of-control wildfire, and now, they need to put 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 stop 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 complex documents and records management platform, into a full-blown enterprise content management system complete with multi-dimensional social tools. The governance model, once simple -- though generally ignored by IT executives and SharePoint administrators -- now requires a careful 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 large SharePoint implementations including management of social collaboration, the bring your own device (BYOD) revolution and the common challenge of SharePoint site sprawl. A well-executed governance plan compares the various elements of governance and balances security with the current evolution of business mobility (see Figure 2).

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

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

Consider the high-costs of implementing SharePoint -- some estimates suggest 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 plan 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 plan will fall flat. Each policy and procedure outlined in a SharePoint governance plan should be 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 be addressed with sound governance policies and socialization.

In this example, success in governance can be 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 face significant competing priorities, nearly half of all organizations still rely 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 importance can't be 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 also 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 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 have the support and infrastructure in place to touch all aspects of the business. An effective governance committee depends on its ability to make and, perhaps more important, enforce decisions and policies around all 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 do 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 come with the imperfection of any business process, but any combination of these flaws can prove fatal 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 importance of a governance plan that distinguishes IT governance, application management and information management.

Governance plans will mature as an organization's SharePoint implementation matures. Likewise, governance plan content will have different points of emphasis at different organizations. For example, Office 365 and SharePoint online governance plans are less technical, but place a heavy emphasis on social content and other collaborative tools available in the online SharePoint environment. In such cases, technical governance is less significant because Microsoft bears the burden of many of the technical governance decisions that an organization with an on-premises SharePoint solution would otherwise have to resolve.

Governance plans should include the following core elements:

  • Operational Management
  • Technical Operations
  • Site and Security Administration
  • Content Administration
  • Personal and Social 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 all the roles and related responsibilities necessary to run an effective 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 also 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 also suggests, rightly so, different types of SharePoint sub-sites can, and likely should be governed in different ways. This makes for a potentially complex, but ultimately more thorough picture of content governance. Site and security administration articulates the use cases for different site templates in SharePoint. It also dictates the procedures for the following:

  • Site provisioning
  • Site de-provisioning
  • Site ownership and responsibilities associated with ownership
  • Permissions structures and best practices
  • Different use 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 put 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 range of topics, including:

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

  • Recycle your old tech gear | killexams.com real questions and Pass4sure dumps

    In springtime, people’s fancies may turn to love, but their to-do list turns to cleaning. Make this the year that you finally recycle all 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 be 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 all electronic waste is linked to consumer electronics. Why recycle at all?

    Sure, it would be easier just to dump all your old, unwanted electronic stuff in the trash. However, old computers and their related peripherals contain a lot of heavy metals—lead, cadmium, mercury—that are bad for people’s health when they get into the soil and water. In addition, when old electronics hit the trash heap, they’re out of a recycling stream that can cut the energy costs for production of future electronics.

    But if the environmental concerns don’t grab you, consider 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 old electronics.

    Now, don’t you want to avoid breaking the law? Don’t you also want to employ people and keep the planet clean by recycling your old stuff? Of course you do, so let’s get started.

    Before you get rid of any of your electronics

    The first step on your recycling journey: Make sure that none of your personal data will be leaving the house along with your soon-to-be-discarded electronics. As a bare minimum, make sure you do the following:

    Don't forget the little things like deauthorizing iTunes-running computers, for example.

    If you’re using Apple’s iTunes to manage your digital music and video, be sure you deauthorize any device you’re recycling. “But that computer is dead” is no excuse: Apple expects you to simply deauthorize all 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 use it wisely.

    If you’re recycling a smartphone, do a complete reset of your phone to wipe out its data and restore it to its factory settings. Before you do this, however, make sure 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. All it does is reformat the space on the drive, and the data can be 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 need to get a disk-wiping program, preferably one that meets the U.S. Department of Defense’s Media Sanitation Guidelines, and you need to set aside a few hours. What a good disk-wiping program will do is write all 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 use Apple’s built-in Disk Utility, or they can cough up $25 for Mireth Technology’s ShredIt X. Windows users on a budget can erase their drives using the free Eraser 6.0.10.

    If you’re tempted to skip this step, consider 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 old MacBook and using it for evildoing are low, why not make them even lower? (Some people recommend physically drilling holes in hard-drive platters, but even that may not be enough.)

    If you’re recycling a digital camera and its attendant memory card, you’ll need to wipe the card too. The best way to do this is to stick any memory card back in the camera and use the camera’s built-in “format card” option to wipe the memory card clean of all data.

    If you’re getting rid of memory sticks, USB flash drives, and other small 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 old printers, scanners, or fax machines, you’ll need to purge the appliance’s memory. The good news is, most consumer-grade printers, scanners, and fax machines don’t have internal hard drives. (Most of the time, the document you’re printing or scanning is stored in RAM in the appliance’s hardware.) All you have to do is disconnect your appliance from any computer, and then turn it on and go through the settings until you find the option to reset everything to factory settings. That should flush out any residual data or personal information like your fax number.

    Now, on to recycling

    Broadly speaking, you have 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 need to really plan 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 have set up recycling options for everything from batteries to toner cartridges to mobile phones. Below, some of the most ubiquitous:

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

    GreenpeaceIf you thought computers from the late 1990s were ugly 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 empty ink and toner cartridge through its own recycling program—and you’ll even get back $2 in Staples rewards for each cartridge you recycle.)

    Hewlett-Packard has also partnered with Staples as a drop-off point for its old computing equipment and printer cartridges. Again, you can drop off your old equipment 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 large chains have recycling programs as well. OfficeMax, for example, will take your old printer/toner cartridges, as will Walgreens.

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

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

  • Do you accept electronic devices from individuals, or do you work only with businesses?
  • What are your policies on destroying personal data that may still be on used computers or cell phones?
  • Do you destroy storage media?
  • Can I get a record of the methods used on my old electronics?
  • What environmental management guidelines do you follow?
  • What percentage of the materials you collect are recycled and what percentage is disposed?
  • What fees will you charge for disposing of computer monitors? Televisions? Or do you charge a flat fee for a carload of items? (The first time I used a recycling center, 11 years ago, I paid $35 to get rid of two laptops. That experience made me appreciate the free programs set up by manufacturers.)
  • A local recycler-as-fundraiser program typically takes the kinds of small electronics found around the house, but won't take 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 all the electronics you care to drop off. The advantage here is that you don’t have to go chasing all over the place to drop off your stuff. The disadvantage is that Lincoln Middle School’s first clarinet chair may do a great job hustling your old printer cartridges out of your car trunk, but she probably can’t tell you whether there’s a decent data-management policy in place at the recycling endpoint.

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

    Apple will take all sorts of your old 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 old 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 old products include BlackBerry, Hewlett-Packard, Lenovo, and Verizon Wireless.

    If you just want to toss everything in one giant box and never think 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 come 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 think about giving it away instead of sending it on its way to its next life. But before you make plans to gift your local preschool with a Performa 6115, stop and consider whether your donation is going to be 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, do it and save the folks at the nonprofits the effort.

    If you have relatively modern, working electronics and you want to get them into the hands of people who can use 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 area of the country. Another organization to consider is the World Computer Exchange, which sells low-cost refurbished desktop computers and peripherals available to developing countries.

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

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

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

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

    All patients received weekly infusions of recombinant human α-L-iduronidase for 52 weeks. The mean 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 average 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 level 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 beginning 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 severe 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 moderate 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 native 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 all 10 patients IgG antibodies developed to Chinese-hamster-ovary cell proteins that were present as a trace 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 body 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 be due to a concomitantly taken medication and that resolved by week 30. This episode was thought to account for the transient increase in the size of her liver. The upper bounds of the 95 percent confidence interval of normal values (i.e., within the normal range 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 base line in nine patients and by 5 percent in one patient at 52 weeks; the mean decrease was 25 percent (P<0.001). By 26 weeks, the size of the liver was normal for body weight and age in eight patients (Figure 1). In the two patients (Patients 6 and 9) who had the largest liver size relative to body weight at base line, the size of the liver was close to normal at 52 weeks (3.2 and 3.3 percent of body weight, respectively).

    In eight patients the size of the spleen decreased by 13 to 42 percent from base line. The mean decrease was 20 percent among all 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 mean reduction was 63 percent (range, 53 to 74 percent; P<0.001). The excess urinary glycosaminoglycan excretion (that above the upper limit of the normal value for age) was reduced by a mean 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 normal in all 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 mean of 6.0 cm (5 percent) in the six prepubertal patients, and their mean 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 all 10 patients, body weight increased by a mean of 3.2 kg (9 percent), and the mean increase was 4.2 kg (17 percent) among the 6 prepubertal patients. In these six patients, the mean 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. Mean Changes in the Restriction of Range 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 mean degrees of restriction in the range 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 base line. The values represent the difference between the normal maximal range 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 base line. The mean decrease in joint restriction was 28 degrees in the right shoulder (P< 0.001) and 26 degrees in the left shoulder (P=0.002) (Figure 2A). Among all 10 patients, the degree of restriction of elbow extension decreased by a mean of 7.0 degrees in the right 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 mean of 3.2 degrees on the right (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 base line, Patients 5, 9, and 10 could not flex their shoulders beyond 100 degrees, and the range of motion increased by 21 to 51 degrees after treatment. The improvements in the range 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 decrease 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 mean 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 all three this disorder improved during treatment. In Patient 2, the apnea–hypopnea index decreased from 4.5 events per hour at base 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 base line, Patient 6 required continuous positive airway pressure at night because of severe 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 quality 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 type of activity or at rest. The difference 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. All 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 base line, Patient 6 had atrial flutter and clinical signs of cardiac failure, including dyspnea at rest and pitting edema. After 12 weeks of treatment, he had sinus rhythm with first-degree block and his dyspnea at rest and pitting edema had resolved.

    Symptomatic Changes

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