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Outstanding Group of Companies and Individuals Receive FlexTech's FLEXI Awards for Flexible Electronics Innovation, R&D, and Leadership
... MONTEREY Calif. March 3 2016 /PRNewswire/...Logo - a href http://photos.prnewswire.com/prnh/20160303/340208LOGO ...This year's Innovation Award goes to two companies one in manufacturi...Blue Spark Technologies wins the other Innovation FLEXI Award for...
MONTEREY, Calif., March 3, 2016 /PRNewswire/ -- FlexTech, a SEMI Strategic Association Partner, awarded five FLEXI Awards in the categories of Innovation, Research & Development, Leadership in Education, and, in a category new this year, Industry Leadership. This is the 9th year of the FLEXI Awards and the winners join a select group of companies and individuals from past years. Judging was done on a set of nominations based on a pre-described set of criteria, by a panel of non-affiliated, independent, industry experts.
Logo - http://photos.prnewswire.com/prnh/20160303/340208LOGO
This year's Innovation Award goes to two companies, one in manufacturing and one in consumer products. The products were awarded for their outstanding design and ingenuity, with strong market adoption and significant revenue generation. The first FLEXI Award for Innovation was awarded to Sensor Films Inc. (SFI), for their Starlight Digital Manufacturing Platform, which enables a modular approach to flexible and hybrid electronic manufacturing with large area deposition of functional and decorative materials on cut-sheet, flexible, porous or rigid substrates and is scalable to roll to roll production. Peter Hessney, SFI's president noted, "We are truly grateful to be recognized by the FlexTech Alliance as the 2016 FLEXI Innovation Award winner. The award represents everything we at SFI having been working towards in bringing an additive manufacturing prototyping and production system to the emerging market of functional printing."
Blue Spark Technologies wins the other Innovation FLEXI Award for their TempTraq® product, the only available Bluetooth®, wearable temperature monitor in the form of a soft, comfortable patch that provides caregivers with continuous body temperature data for up to 24 hours to either an Apple® or Android™ compatible mobile device through the free TempTraq app. "It is an honor to be recognized by FlexTech Alliance not only for our flexible printed battery technology, but also the design and ingenuity of the application," said John Gannon, president and chief executive officer of Blue Spark Technologies. "We are enthusiastic about the future products that will further expand on the innovative technology that powers TempTraq, which has been so widely received in the consumer space."
Scientists at the University of Massachusetts Lowell received the FLEXI Award for Research & Development for research conducted at the Printed Electronics Research Collaborative (PERC) and the Raytheon-UMass Lowell Research Institute (RURI). This award is given to the nominee evaluated highest for the research approach, originality and the commercial potential for expanding the scope for flexible or printed electronics or manufacturing processes or a real-world problem. The PERC/RURI organization was recognized for its development of the novel BST ink, a ferroelectric nano-ink for printing electrostatically-tunable dielectrics on plastic substrates using direct-ink writing methodologies. The BST ink allows for realizing an all-printed high-frequency voltage variable capacitor for flexible, frequency-agile microwave applications such as phased array antennas and radar systems.
The Technology Leadership in Education Award was bestowed upon the National Science Foundation Center for High Rate NanoManufacturing at Northeastern University (CHN). The CHN in Boston has educated more than 130 students, half at the Ph.D. level on the topics of directed assembly-based printing, synthesis of organic semiconductor, and polymers engineering, leading to the development of the world's first printer for nanoscale electronics on flexible or rigid substrates. CHN has had an impact on established and emerging industries in sensors, electronics, bio/medical, energy, and materials fields by educating the next generation's professional workforce. The CHN publishes extensively in industry journals, created tutorials and other programs to educate the general public, as many as 840,000 individuals. The award recognizes outstanding contributions to the industry through education and workforce development activities. "The CHN is honored to receive the FLEXI Award for Leadership in Education," says Ahmed Busnaina, the founding director of the Center and a professor of Industrial Engineering. "We are proud of our many accomplishments in manufacturing and nano-scale printing such as graduating more than 130 students, more than 100 patents and developing the world's first nanoscale 3D printer for electronics and sensors."
2016 marks the inaugural year of the Industry Leadership Award to recognize and honor an individual who has shown particular dedication to building awareness of the capabilities and innovations which advanced flexible, hybrid and printed electronics bring to the broader field of electronics. As well, the individual has demonstrated outstanding thought leadership and vision for the industry in public forums, and association activity. The judges and FlexTech wish to acknowledge these contributions by Daniel R. Gamota, Ph.D., currently vice president of Strategic Capabilities in the Engineering & Technology Services Organization at Jabil. Dan has devoted many hours to the genesis of the industry through strategic roadmap development activities and standards work, including organizations such as IEC, IPC, iNEMI, IEEE, A*STAR, and the WTEC panel at the National Science Foundation. Dan commands thought leadership for the industry at numerous public forums and through several peer reviewed flexible and printed electronics publications. In accepting the award, Dan stated, "No one person could have much success without the cooperation of others in the emergence of a disruptive technology such as FHE. It has been a great pleasure in working with many of you in the advancement of the technology and the products they spawn."
Also receiving awards during the evening were student poster contestants. First place was awarded to Yasmin Afsar, Levent Aygun and Can Wu from Princeton University. Their posters and research were entitled: ZnO versus a-Si thin-film oscillator circuits: metrics for design and comparison; High-Frequency ZnO Schottky Diodes for Inductive Non-Contact Interfaces for Hybrid Flexible Electronics/IC integration; and Sensor Data Compression by Large-area Electronics to Reduce Large-area to VLSI Physical Interface Connections in Hybrid Systems, respectively.
Second place poster was awarded to Francisco Suarez from North Carolina State University, for his poster entitled Flexible Thermoelectric Device Using Bulk Materials.
Third place was awarded to Yuvraj Singh from Purdue University for his work on Reliability Enhancement of Active Circuits on Flexible Substrates.
About FlexTech Alliance
FlexTech, a SEMI Strategic Association Partner, is focused on growth, profitability, and success throughout the manufacturing and distribution chain of flexible, printed electronics, by developing solutions for advancing these technologies from R&D to commercialization. Visit FlexTech at www.flextech.org.
Heidi Hoffman
SOURCE FlexTech Alliance
Copyright©2016 PR Newswire.
1. Outstanding research on improving animal welfare in science
2. Leibniz Prizes 2014: DFG honors 11 outstanding researchers
3. AGU honors outstanding journalists
4. Institute of Medicine honors members for outstanding service
5. Eliassen Groups Biometrics & Data Solutions Team Expands to Support Growth
6. J. Craig Venter Institute Policy Group Releases New Report: "DNA Synthesis and Biosecurity: Lessons Learned and Options for the Future"
7. Dragon Capital Group Received a Contract for Multi-Format Naked Eye 3D System
8. Gefängnisbehörde von Virginia benennt Track Group als Partner für elektronische Überwachung
9. Virginia Department of Corrections Selects Track Group As Electronic Monitoring Partner
10. SoundView Technology Group Issues NXT-ID (NXTD) Update; Scaling Up
11. Global Smart Mobile Devices Fingerprint Sensors Market 2015-2019: Key Vendors are Authentec, Validity Sensors, Fingerprint Cards, Next Biometrics Group. IDEX, and Qualcomm


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Metropolis Records presents more KMFDM Classic re-releases. These long out of print essentials from the band's Wax Trax! Records days have been unavailable for years. Now, Metropolis Records is putting them out in the order in which they debuted. The next releases to be made available are two of the aces in the deck of the KMFDM back catalog, 1995's Nihil and 1996's Xtort. These records contain a veritable who's who of hard alternative music at the time. KMFDM leader and founder Sascha K. conducts a crew featuring the likes of Chris Connelly (Ministry, Rev. Co.), Raymond Watts (Pig), Bill Rieflin (Ministry) and F.M. Einheit (Einsturzende Neubauten). While KMFDM has always been about creating a legacy of quality and never focused on mainstream success, it must be noted that Nihil contains the hit single "Juke Joint Jezebel" and Xtort features the indie favorites "Power" and "Son Of A Gun". These albums have both been given the deluxe treatment which includes all new booklets that have been expanded to 24 pages to include some exciting new material. All KMFDM Classic re-releases have been digitally remastered, presenting their songs in a quality previously unavailable. Documenting the history of KMFDM the accompanying CD booklets all include the original album art (most by famed KMFDM co-conspirator Brute!), in-depth liner notes, quotes from Sascha K., lyrics for each song, never before seen photos of the band, full recording and production credits and more. All work on these releases, from the remastering to the packaging, has been done with the highest level of participation from Sascha K. These releases join KMFDM's first six albums, What Do You Know, Deutschland?, Don't Blow Your Top, UAIOE, Naïve, Money and Angst which are already available from Metropolis Records. Look for the remaining KMFDM Classics, Symbols and Adios, coming soon. The next phase of the legacy begins, KMFDM is back to prove that the rules do not apply! Celebrate chaos, get into the noise!
Ultra 4:34 $ 0.89 buy mp3
Juke Joint Jezebel 5:40 $ 0.89 buy mp3
Flesh 5:02 $ 0.89 buy mp3
Beast 5:07 $ 0.89 buy mp3
Terror 4:50 $ 0.89 buy mp3
Search & Destroy 3:26 $ 0.89 buy mp3
Disobedience 4:43 $ 0.89 buy mp3
Revolution 4:27 $ 0.89 buy mp3
Brute 4:25 $ 0.89 buy mp3
Trust 4:42 $ 0.89 buy mp3
Nihil 2:04 $ 0.89 buy mp3

redpajama_set_name: RedPajamaCommonCrawl


Something I meant to do a while ago, but I'm doing it now… I've uploaded the list of 100 ways we suggested to save the city. The list ranges from the entirely possible to completely imagined, but each one might just make this city a better place, or at least make you feel a little bit better about this city.
There's a different background colour for each of the 100 ideas, each of which was taken from a Google Map of Windsor. The colour didn't show up so well in our projection, but it was there.
We'll also upload the little Max patch we wrote to do the performance … it's quite easy to use, but maybe would eventually be better to be something written in Processing?
Download the PDF for your enjoyment.
As part of FAM Fest 09, we did a projection performance on the roof of Metro Cleaners accessed from Empire Lounge in downtown Windsor.
For about an hour and a half, we presented our 100 Ways to Save the City and then asked for ideas from the folks on the ground, at Phog, and on the Twitterverse.
After the jump, there's 160-something photos from all the ideas that were projected on Saturday night.
Last night out a window in the county, the new projector at night.
Today, finishing our list of 100 ways to save the city.
It's going to run as a presentation in Keynote, the easiest failsafe solution. Though, we might try to open it up on Twitter somehow later tonight.
And, speaking of tonight, the weather is looking good. No rain!!!! We'll be projecting across from Phog, look up above Empire Lounge and you'll see us. Tonight is marks Day 2 of FAM Fest, hope to see you out and about.
Saw a good deal on FutureShop's website, thanks to Mike, and had to place the order. While this projector is still nowhere near bright enough for Cross-Border Communication, I couldn't pass up a 2800 lumens projector on sale for half price.
It'll debut tomorrow night at our projection performance for FAM Fest.
We met last night and started our list of 100 Ways to Save the City for our projection on Saturday night as part of FAM Fest.
We're also spent some time putting together a rough schedule for 2009 Micro-Residencies, which will likely start in November. If you've thought about applying, but haven't yet, now would be a great time to put forward an idea!
Projection Site for Another Project?
I've been meaning to take a look at this location for a potential projection project that we're looking into for Harvesting the F.A.M. The wall is dark brick, but we need to work out the logistics of projecting onto such a surface anyways for another project we're working towards.
What exactly we'll be projecting is still up the air, but we plan to be on the roof of Empire Lounge for an hour or so on one of the nights of the F.A.M. Fest. We'll keep you posted.


text:
var mongoose = require('mongoose');

// local requires
var connections = require('./connections');

// variable declarations
var databaseUrls = [
	'mongodb://localhost/doubletest1',
	'mongodb://localhost/doubletest2'
];

// callback function declarations
var connectToDabases = function(url){
	connections.add(mongoose.createConnection(url));
};

// function declarations
var connect = function(){
	databaseUrls.forEach(connectToDabases);
};

var getConnections = function(){
	return connections;
};

// main
exports.connect = connect;
exports.get = getConnections;

redpajama_set_name: RedPajamaGithub


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Read This: The inside story of SNL's insane, baffling "Potato Chip" sketch
(Photo: NBC)
It is a long-standing tradition on Saturday Night Live to allow the last sketch of the evening, the one that airs just before the goodbye waves, to be bizarre and conceptual and even off-putting in a way that the rest of the show isn't. In his SNL reviews, Dennis Perkins files these under the heading "What the hell is that thing?"—The Ten-To-Oneland Report. A perfect example of a "Ten-To-Oneland" sketch, perhaps a textbook case, is "Potato Chip," a Jason Sudeikis-Will Forte scene originally written for a November 2009 episode with Taylor Swift. It was cut after dress rehearsal but finally aired during a Blake Lively episode the very next month.
The premise is extremely simple: During a job interview at NASA, interviewer Forte leaves astronaut candidate Sudeikis alone for a few minutes with explicit instructions not to touch the potato chips on Forte's desk. Sudeikis immediately eats one of the chips, and when Forte returns, he is apoplectic with rage. What distinguishes the sketch are the intensity of the performances, the stubborn oddity of the characters, the theatrical staging, and a propensity for gross-out humor. For anyone wondering how this strange sketch ever aired on national television, Uproxx's Mike Ryan has assembled an oral history of "Potato Chip," complete with testimony from Sudeikis, Forte, co-writer John Solomon, and former SNL head writer Seth Meyers.
Open www.hulu.com
The origins of "Potato Chip" are surprisingly complex, drawing on such diverse sources as Cool Hand Luke, the plays of Tennessee Williams, Colonel Sanders, and Forte's fascination with NASA. Most of the credit for the scene, the interviewees agree, belongs to Forte, who championed "Potato Chip" during SNL table reads with his 100-percent committed performance. That was enough to win over Meyers, who gave the sketch the go-ahead twice. Even today, he considers himself a "Potato Chip" fan. Sudeikis brought a few of his own ideas to the scene, including the hemorrhoid donut his character would carry and a final, dramatic shot in which his character stares longingly at Forte and Lively through a window. According to Solomon, Forte didn't care whether or not the audience laughed at the sketch or not. He just wanted a strong reaction. Since "Potato Chip" climaxes with Sudeikis vomiting into Forte's hand, it's safe to say he got one.


STAMFORD, CT - January 2002 Cuisinart, a leading supplier of culinary appliances and professional quality cookware, has unveiled its Custom 14™ Food Processor, model no. DFP-14BC, in brushed stainless. The new model joins several other product introductions in brushed chrome and brushed stainless, offering consumers a full spectrum of appliances in either look.
"We have seen a continuing trend towards brushed stainless accents in the kitchen," says Mary Rodgers, Director of Marketing Communications for Cuisinart and Waring. "By offering the DFP-14 in brushed stainless, consumers can accessorize their homes with Cuisinart products for a uniform look in home design."
The DFP-14BC has a spacious 14-cup work bowl for extra large culinary tasks. It has both a large and small feed tube for slicing and shredding fruits and vegetables, either whole or pre-trimmed. The food processor comes with Cuisinart's signature Metal "S" blade, as well as a special dough blade that is powerful enough to knead a batch of dough with ease. The 14-cup food processor includes Cuisinart's patented 4mm Slicing Disc and Medium Shredding Disc, spatula, and 20-minute how-to video, as well as a collection of 75 recipes from the Cuisinart Kitchen. The new model is compatible with all existing Cuisinart specialty blades and discs. All parts are dishwasher safe.
The DFP-14BC is backed by a five-year full motor warranty and a three-year limited warranty on the entire unit.


Experience the heat of walnut wood and champagne gold that this pretty accent table can bring to your house. This elegant geometric table has an angled twisted metal base crafted of perforated metal with a polished finish. The square table top is solid wood with a warm walnut finish. This table has a 10.25 inch square base and stands 30.5 inches tall. This pedestal table is designed to suit perfectly for your entryway to drop your keys as you walk within the door, as a side table for your living room, or whilst Up to date nightstand next on your bed in a tight space.


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Sloth moves into slow lane at Banham Zoo as there is lot of monkeying around
Published: 7:00 AM August 12, 2016
A male Linne's two-toed sloth and a group of gelada monkeys are now calling Banham Zoo their home - Credit: Archant
He may not be the quickest of animals at Banham Zoo but this male Linne's two-toed sloth is proving a hit with visitors to the attraction's Eureka sub-tropical enclosure.
The sloth can be seen hanging from branches above visitors in a specially adapted section of the enclosure.
The species from South America is managed within a European breeding programme and the zoo has already been in contact in the hope of acquiring a female for the enclosure.
A zoo spokesman said: 'The zoo has equipped his living space with a network of branches, enabling him to live in the area directly above the zoo's visitors, with its lush flora and warm year-round temperatures, it has proved to be a perfect habitat for its latest occupant.'
The zoo has plans to expand its volunteer base with specific Eureka explorers – visitor engagement volunteers who will be based within the sub-tropical house and will inform guests about the myriad of plants and animals living in this exciting area.
Another addition to the zoo's menagerie has seen four male gelada monkeys make the journey down from Edinburgh Zoo to a large grassed enclosure that previously housed colobus monkeys.
Gelada live in the Ethiopian Highlands with large populations found in the Simien Mountains and primarily eat grass.
The zoo spokesman added: 'The four males have settled in well and are making full use of the large open topped grassed enclosure which is very well suited to their needs.'


What Troll is Behind the AltCDP Twitter Page?
As the campaign for the next Chair of the California Democratic Party heats up, social media feeds supporting individual candidates running are super-blooming like California poppies in Lancaster. The feeds are fun to watch, because any question about a certain candidate is immediately viewed as an attack on that candidate by supporters.
There is a particularly interesting Twitter feed called AltCDP, which some claim is actually run by one of the four senior CDP officials running the party in the wake of former chair Eric Bauman's resignation. Some are calling on public denials in order to out the identity. Good luck with that.
Orange County is no stranger to Internet trolls; best to ignore them.
The current charge de jour on AltCDP is that an unnamed senior party official is pressuring those suing Bauman to amend their lawsuit to soften it but having the effect of harming these staffers all over again.
Go to the Twitter feed and check it out.
Meanwhile, CDP chair candidate Kimberly Ellis announced earlier this month that she's raised $350K in her bid to become the next chair. And a website has emerged with information suggesting she's violating campaign finance reporting from her 2017 bid.
BERKELEY, Calif. (March 6, 2019): Te…am Ellis is proud to announce it has surpassed the midway point of its fundraising goal, having raised $350K from individual donors since January 2019 — expecting to complete its total funding goal of $650K by the end of April.
Team Ellis established a $650K goal for the five month campaign, which includes three programmatic components, including a $1000 paid internship with a focus on Gen Z + Millennials, four full-time, benefited regional campaign directors to provide delegate member-services, $50K in grants for Central Committees in hotly contested Congressional and Legislative districts, and live streamed statewide issue forums.
At Team Ellis, our fundraising philosophy is simple and straight forward… we believe in publicly financed elections and the eradication of big money in politics. But until this goal is achieved, we believe it is our responsibility to take as many dollars from as many people as we can – BUT – to never take any dollar that comes with strings attached.
Practically speaking, this means our campaign will only seek out and solicit individual donors or political organizations whose values are aligned with our own. Our experience in fundraising teaches us that corporations and corporate PACs generally never give political money unless they want something in return – and since we don't believe in pay to play – our campaign has no intention of receiving any corporate donations.
But should the world's best corporate citizen come along and want to help us turn a Republican seat into a reliably progressive district for the next couple decades by banking all the costs out of the goodness of its heart without expecting anything in return, then our campaign is willing to talk… well, just as long as they're not charter schools, tobacco and oil companies, the private prison industry, big Pharma and the like. Thanks but no thanks; donations from them are non-starters for our campaign.
Note: Ellis was asked — twice — about contributions from high tech millionaires at a DPOC meeting in 2017 and ignored the question completely. Misha Houser asked the questions.
Ellis violated the Internal Revenue Code by never filing regular contribution and expenditure reports (known as Form 8872).
Kimberly Ellis' campaign organization was of course created to influence the election of the CDP Chair's race, which is the office of a political organization. She was therefore required to file reports pursuant to the Internal Revenue Code.
The race for chair is turning into a nasty and dirty campaign; whoever wins deserves the support from California Democrats to unit all factions of the party to defeat Republican candidates from President to Dog Catcher.
The Republican Party is the political equivalent of the human centipede.


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What channel is MeTV on dishnetwork?
Channel 247
Starting October 1, 2019, MeTV can be seen on Dish Network Channel 247.
What is the schedule for MeTV?
5:30pm. Remind Me. Happy Days. Bride & Gloom (s8, ep13)
6:00pm. Remind Me. M*A*S*H. Deal Me Out (s2, ep13)
6:30pm. Remind Me. M*A*S*H.
7:00pm. Remind Me. The Andy Griffith Show.
8:00pm. Remind Me. Old Man Carter (s1, ep23)
8:30pm. Remind Me. Eb Returns (s3, ep16)
9:00pm. Remind Me. Hogan's Heroes.
What channel is 218 on Dish?
Folk TV (FOLK)
Where is the MeTV Channel?
MeTV's operations are located in Weigel Broadcasting's corporate headquarters on North Halsted Street in Chicago, Illinois.
Why can't I get MeTV anymore?
Many U.S. television stations are moving to new frequencies over the next year. If you watch television using an over-the-air antenna, you may need to make adjustments to your television set to receive MeTV. Cable and satellite viewers of MeTV are not impacted.
What directv channel is MeTV?
MeTV is not a national channel. It is usually broadcasted as a sub-channel. If you are, getting sub-channels in your area with your DIRECTV service, well and good….What channel is MeTV for DIRECTV?
Channel name
Channel Number
MeTV New York 33
KAZA LA 54-1
How can I watch MeTV for free?
Lucky and more 100% free on MeTV's website. The streaming on MeTV's website is powered by Hulu, but you won't find a ton of Hulu branding for most shows except a "powered by Hulu logo" on the bottom of the page.
What shows are on MeTV tonight?
MeTV Network
3:00 pm The Rifleman I Take This Woman – Season 5 Episode 6
3:30 pm The Rifleman The Assailants – Season 5 Episode 7
4:00 pm Wagon Train The Jane Hawkins Story – Season 4 Episode 10
5:00 pm Adam-12 Log 91—You're Not the First Guy's Had the Problem – Season 1 Episode 5
What channel is bet on Dish 2020?
BET on DISH Network: Channel 124.
Is MeTV a free channel?
The best thing about MeTV? Well, all the classic television shows you love. But also, it's free! Most of America is able to receive MeTV in crisp 1080p over the air.
Why can't I get MeTV on DirecTV?
If you are using DirecTV and wondering whether you can access MeTV, the answer is yes – but it's also no. DirecTV allows users to access MeTV as they are broadcast as the Digi-nets (the OTA ones), but only if the local station carries the network on the -1 channel.
Where to watch MeTV?
Where to watch METV. You can receive METV over the air on channel 4-2, 10-3, or 11-3. Just like 10/11 and NBC Nebraska, METV is broadcast free, over-the-air for viewers to receive using an antenna. In order to receive digital television, a digital tuner is needed to decode the signal. METV is home to classic television like I Love Lucy ,…
What channel is MeTV on?
MeTV was originally developed as a programming block that debuted on January 6, 2003, on Class A television station WFBT-CA (channel 23) in Chicago, Illinois, an independent station owned by Weigel that otherwise maintained a format featuring programming aimed at the market 's various ethnic demographics.
What channel is me on Dish?
V Me on DISH Network – Channel 846. V-ME was launched as the first public access satellite television channel dedicated entirely to Spanish language programming for households in the United States. The DISH TV channel features original programming celebrating the Spanish language and culture as well as a variety of dubbed programs.
What station is Me TV on?
Answer Wiki. Me TV is one of the channels offered on U-Verse (channel 136 in the Dallas area, likely similar for other markets as it is a national channel).
Is it worth it to make your own Greek yogurt?
What is tawteen process?


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The brothers Jan and Václav Fišers from Turnov. Their alleged voyage to Venice, five years of efforts to seize the local secret of secrecy, return to Turnov, dozens of wasted attempts and then - finally success! In 1711 they made the first glass for the production of artificial stones.
It was in 1712, when Elias Müller built his glassworks on the estate of the Harrach Counts in Jilemnice. It continued in the traditional production of glass, which was produced on the border of the Giant Mountains and the Jizera Mountains in the middle of the 14th century. Even today, you can see not only metallurgical production and blowing glass, but also a hundred-year-old grinding shop. It is driven by a water turbine and transmissions used to distribute the driving force.
Elias Palme, one of the most famous luminaire manufacturers, continued the tradition of chandeliers in Kamenický Šenov. Industrial production of chandeliers was started in 1724 by Josef Palme in Prácheň.
Surely you would not find in the whole Principle and in the broad surroundings of a person who would say a crooked word about Jan Šourek. After all, it was as if he had come to the Principle of Prosperity and Welfare. As soon as he took possession of the reeve, life in the village began to turn for the better. He was one of the first to venture into the world for business.
Josef Riedel, king of the Jizera Mountains glassmakers, was born in Hejnice in 1816. He was the representative of the sixth generation of the glass family and imprinted his footprint indelibly on the slopes of the Jizera Mountains.
Josef Pfeiffer - Jablonec entrepreneur and mayor, who was involved in the development of jewelery exports. Everything is confirmed at the imperial court and in 1866 Jablonec became a town during his reign.
People can make sense of time and numbers, but not us, beads. However, I have heard that they have been blowing me in the mountains for more than a hundred years. As a bead would put it, that is a long time. You, who have never seen how a bead is born, you don´t know how beautiful life can be....
In 1920 the first Czech glass school was founded in Železný Brod, thanks to which the town became a center of glass art.
Precious stones from the Bohemian Paradise
THE GLASSHOUSE IN NOVÝ SVĚT
ELIAS PALME
ELDORADO OF BEADS AND SEED BEADS
JOSEF RIEDEL
FRIEDRICH EGERMANN
JOSEF PFEIFFER
BLOWN BEAD and its way to the tree
MELTED GLASS SCULPTURE
Lusatian Mountains (Lužické hory)
Giant Mountains (Krkonoše)
Jizera Mountains (Jizerské hory)
Zásada
Hejnice, Frýdlant and surroundigs
Bohemian Paradise (Český ráj)
How did glass Christmas decorations get from Železný Brod to New York?
Christmas Concerts in Crystal Temple
Pearl Christmas decorations from Poniklá are on the UNESCO World Heritage List
LASVIT glass headquarters winner of the Dezeen Awards 2020
Unveiling a unique glass sculpture
The winning work of the Crystal Master 2020
Museum programs for children online
Turnov: Gloria musaealis 2019
Glass fountain at the state chateau in Zákupy
TRENDY.DESIGN.PRODUCTION
Crystal Temple
The museum crystal in Jablonec is finished
11/9 11/9
Glass Town - Glass Craft Festival
Open without advance booking Small workshop /studio Exhibition, Museum, Art Gallery Also suitable for groups over 40 guests Glassworks Restaurant in the glassworks Jewellery / Fashion Jewellery studio Glass or Jewellery school
Bystro Design
Čangel Glass
Crystalex CZ
Florianova huť - Petr Červený
Glass Museum Kamenický Šenov
Glass Museum Nový Bor
Glassworks Jílek
Hoineff glass art
Kamenický Šenov: Secondary School of Glassmaking
LASVIT - GLASS HOUSE
LASVIT AJETO
Memory Crystal
NATĚLO
Novotny Glass
Nový Bor: Higher Vocational School of Glass and Secondary School
Pačinek Glass
Preciosa Lighting
Prousek exklusive lighting
SKLO.
Svojkov Glassworks, Jiří Haidl
TGK - Technology, Glass and Art
Vydry studio
Glassworks and private brewery Novosad & Son
Rautis
AG Plus
Arcon Bijoux / Collegium Trade
ArtCrystal Tomeš
Atlas Bijoux
Bead Game
Cultural and Information Center Riedelova vila Desná
Decor by Glassor
Evans Atelier
Glass Pesničák
Glassunicum
Goat farm Pěnčín
IQLANDIA
Jablonec nad Nisou: Secondary School of Applied Arts and Higher Vocational School
Martin Gőrner, Lužické sklo LsG
Museum of Glass and Jewellery in Jablonec nad Nisou
PALACE PLUS
Perlex Bijoux Jablonec
Preciosa Beauty
Preciosa Ornela Desná
Preciosa Ornela Zásada
Ralton
Spider Glass
U KAHANU
Čambalová Pavlína
Detesk
Galerie Granát
Glass Studio Oliva - Oliva Glass
Hela-glass
JEWSTONE
Kamila Parsi
Ladislav Ševčík Bohemia Crystal
Lhotský
Misamo
Museum of the Bohemian Paradise in Turnov
Stefany jewelry
Town Museum
Turnov: Secondary School of Applied Arts and Higher Vocational School
Wranovsky Crystal
Železný Brod: Secondary School of Glassmaking
CZ EN DE PL
PlacesPlaces
See & ExperienceSee & Experience
Glassworks Everyday Glassware Lighting Christmas Decorations Jewellery, Bijou Glass Sculptures
There are places in the world that have magic power. There is always success, no matter what you do. Even if there are some obstacles, it all goes well in the end. One such place is Nový Svět in the Giant Mountains. Today, it is a part of Harrachov, but at the turn of the 17th and 18th century, it was just a settlement, where the Müller family decided to build a glasshouse. The reason why Ellias Müller built the glassworks was that three surrounding glasshouses ended their production. This was quite common. When there was no wood, the glassworks closed down. A new glasshouse was then built in another place with enough wood. Nevertheless, Ellias had a dream. He wanted to build a glasshouse that wouldn´t end like the others. In the end, he was successful. The Nový Svět glassworks still functions today. It has been operating for more than three hundred years. This may be the oldest continuously operating glassworks in the world. Let´s be honest, Ellias Müller and his sons would never succeed without help. Only owners of large estates had an opportunity to transport wood from remote locations. In the case of Nový Svět, it was the Harrach noble family that moved to the Giant Mountains area in the first year of the 18th century, when they bought the town of Jilemnice with its estate.
And so, Elias built that glasshouse. To tell the truth, he did not pay for the construction. He had to borrow money from the Vicar of Rokytnice. That is why, for some time, the glasshouse was called "Vicar´s glasshouse". However, he repaid it very soon, because the glasshouse did very well. The glassmaking master Müller stubbornly insisted on quality, which turned out to be a good move. He couldn´t know that he started the tradition of quality that became "the alpha and omega" of the glass factory. When Elias died in 1730, his oldest son Jan Josef Müller became his successor. He was also doing well, and the prestige of the local glassworks was growing. The Harrach family witnessed the success of the glassworks and was very proud that the glass factory located on their estate produces high-quality and demanded products. Glassmaking was a prospective industry, and they would join it as well if they had time. However, politics consumed their time. The Harrach family worked in the service of the Viennese court across the whole of Europe. Thanks to that, they had great knowledge and experience. They got familiar with the latest trends in glass production.
And politics did not leave them alone, not even in the Giant Mountains. In 1742, The Austrian Empire lost most of Silesia, and the Giant Mountains, until then understood as one territory, were now divided by a border. Immediately, the smuggling spread and the customs officers could not prevent it, not even with most refined measures. The glass smugglers brought the glass to Prussia for refining and then the same way back, but at a much higher price. The only chance to stop that was to create a complete production and refinement area in the Austrian part of the mountains. The Harrach family that had a strong position in Vienna reacted very quickly. First, they started successful textile production, and then glass production. In 1764, Arnošt Quido, Count Harrach, buys the glasshouse in Nový Svět from Jan Josef Müller. He leaves Müller in charge of technical management of the glassworks. Great experts fill other positions. The traditional high quality remains intact. In addition to several already existing refining workshops, more were soon added. In Nový Svět, the final product only needs to be packed and shipped to customers. The Harrach family´s contacts contribute to the company´s sales.
Only four years left until the end of the century, when another member of the noble family gives up his political career to be more active in the property management of the estate in the Giant Mountains. It is Jan Nepomuk Arnošt. It is necessary because there was a windstorm that destroyed huge areas of forests, and the glassworks in Nový Svět ceased to be profitable. There was a fear that the glassworks might close. That would be a disaster for local people and a very unfortunate thing for the family fortune. The Harrach family did not want to let that happen. They had a strong social conscience. Jan Nepomuk started cleverly, like his father before him. First, he bet on experts in glassworks management. At the same time, he made several changes that are considered ground-breaking in Czech glassmaking. He published "the Conditions of Employment", established regular weekly wages, and prohibited the substitution of money in kind. The oldest collective contract in the history of Czech glassmaking was created. Soon afterwards, he established a pension fund for glassmakers as well. Everything seemed good again when thunder rumbled over Austria, and state bankruptcy struck like lightning. The glassworks employees got into a very bad position, and a riot was in the air. Count Harrach called all the employees to discuss the situation. General costs were ruining workers, and Harrach was worried that the prices of goods would rise if he increased the wages and that the glassworks couldn´t compete in world markets. He demonstrated with examples of many glasshouses in the area that disappeared. He said it is better to have poorly paid work than no work at all. That is true, but tell that to a father of fourteen. Eventually, they find a compromise - the wages would increase, but only for some time. The Count knew that he couldn't just stand and watch and that to protect the glassworks, he needs to invest in and modernize the glasshouse. Also, he agrees to list rewards for suggestions for technical improvements. He founds a drawing school as well.
And the family line continues with Jan´s nephew František Arnošt - Count Harrach. Nevertheless, it looked like the successions of the estate was cursed. Less than a year after his succession, Josef has to deal with a catastrophe – the glassworks burned down almost completely. Others would give up, but not Harrach! He built a new glasshouse from stone. He said to himself, stone cannot burn, and once again it turned out great. The new factory achieves great success and prosperity. Over twenty years, the number of employees doubled. As the positions of glassworkers improve, so does their security. The Harrach family is very smart social-wise, and so they work on a system of pension funding. It is not just empathetic, but tactic as well They knew, that this way they would prevent the experts from leaving to the competition.
Who is the next Harrach in charge of the glassworks? – Jan Nepomuk František, Count Harrach. Do you think that he will be excluded from the line of disasters? Sadly not. He has been the administrator for three years when the fire strikes again. The building is made of stone, but it is still damaged. In this time of crisis, Jan Nepomuk shows his unique abilities – he manages to fully restore production just thirteen months from the fire. Everybody is involved – even local cutters and grinders help with delivering wood for the construction. Even later, Jan Nepomuk shows his talent as an administrator, great landlord and amazing businessman. Moreover, he is an active Czech patriot, and his goal is the development of the Giant Mountains area. He advocates for road network improvement, expansion of the railway network and a boom in tourism, which means more money for the region. He goes beyond managing the glassworks, further than his predecessors. He knows his way around glassworks´ operations, considers and approves new designs of products, recognizes the quality and expertise of employees. The best employees are appropriately motivated and rewarded. He is also a very gifted exhibition organizer. He gets repeatedly asked to organize the Austrian panel at world exhibitions, and his great merit was the jubilee exhibition in 1891 in Prague. Harrach´s pavilion and glass exposition in the central pavilion was at the centre of attention, as was the case of exhibitions in London or Antwerp. Jan Nepomuk promotes Czech industry at every opportunity, which is a rarity at the time. He uses Czech inscriptions in his expositions, even at world exhibitions.
At the time, the innovation of the glasshouse is truly legendary. The buyers from all over the world try to visit Nový Svět to see what is new. Local production exports in addition to all of Europe, to North and South America, Russia, India and Australia. And the quality is of course top. In the 1880s, over five hundred people work in the glassworks. Twenty years later, the products are processed in seven cutting workshops, twenty-two painting workshops and ten engraving workshops. It was as if the prosperity and success of the glassworks couldn't be stopped. However, death took Jan Nepomuk in 1909,. His funeral at his beautiful family tomb in Dolní Branná was a manifestation of loyalty. There were several thousand people in the coffin parade, and the sadness was sincere. The most famous epoch of the glassworks in Nový Svět ends, as the owner´s relationship with the glassworks did. Not one of his successors managed to do what Jan Nepomuk accomplished. Well, maybe one did. But it took a long time.
It´s not that John´s son Otto didn't want to. But unfortunately, he was taking over his magnificent inheritance at a bad time. The First World War starts. Otto does what he can, he is really trying so his employees and their families wouldn´t suffer. He doesn´t hesitate to use his own assets. After the end of the Great War, Czechoslovakia is established, and Otto faces the same situation as his predecessors with Silesia. Once again, part of his estate is located in a foreign country. He fails to revive the glassworks, not even in the better years, in the time of the crisis, the glassworks nearly closed. However, remember that Nový Svět is a magical place. And so, the glassworks, mainly thanks to a national loan, survived. When the glassworks situation improved a little bit, Otto died. For his son Jan Nepomuk Antonín, it was a bitter pill to swallow. The Munich Agreement follows shortly afterwards, then the annexation of the Sudetenland with the condition that every company has to be led by Germans. There is only one way to keep the family estate. Despite the Harrach family considering themselves Czech, they are forced to accept imperial citizenship. It helps for a little while, but the Germans have other plans. If it isn´t possible this way, let´s find another way. They put their man – Rudolf Endler in the position of the director of the glassworks, and in 1943, Count Harrach is forced to sell the glassworks to Endler, for a much lower price. Jan Nepomuk clears out the office, and at the same time eagerly organizes some legal activities. This war cannot last forever, and then the Harrach family would return to Nový Svět, for sure. And so, he asked for all samples to be packed and hidden in a room behind the sample room and orders the door to be walled up. He, however, did not know, that just two years later, he would die, suffering from dysentery, in a prisoner camp. What he also cannot know is that this forced sale then makes it impossible for the Harrach family to get the glasshouse back, after the restoration of democracy. With him, the family loses the glassworks.
The Czechoslovak state becomes the third owner of the glassworks after the red coup. The glasshouse gradually becomes a part of three companies, functioning and keeping up the level of quality of its founder Ellias. Its products are sold by Skloexport all around the world. This was the case until 1993 when the decision is made to offer the glass factory to a new private owner. At that happy moment, after the family Müller and Harrach, a third family takes over the glassworks – the Novosads. A lawyer, as well as a learned glassmaker, František Novosad, decides to accept a new challenge in his life, after twenty-three years in chandelier production in Kamenický Šenov. Under his rule, the glassworks flourished. However, František knew that it may not always be like that. He felt that something is about to happen, and that it is necessary to maintain balance. He believed the activities of Skloexport were slowly ending, and Czech glassmakers were amazing artists and producers, but not such good businessmen. Unfortunately, his sad vision came true, and the sales of glassworks decreased. He was in charge of the oldest glassworks and wouldn't give up. At the time, when there were only seven microbreweries in the Czech Republic, he started to build the eighth one. A brewery next to glassworks - that was a great idea. He additionally reconstructed the old stone Manor House and placed exhibits there from the secret room behind the showroom. He now has a museum with a comprehensive collection of glassworks production, which is a world rarity. And the tourists were coming from all around the world. Thanks to that, the glassworks managed to survive and nowadays, the whole complex has also a beer spa. The attendance is so high that any Czech chateau would do anything to get it.
The glasshouse in Nový Svět works sufficiently. Thanks to the foresight of its owners, the glassworks holds to a high quality and manages to compete, with its higher prices, with cheap Turkish, Chinese and Vietnamese glass. Maybe, it is because the production is more or less the same as hundred years ago. The preserved cutting workshop, another unique place, is not only an open-air museum but a fully functioning and operating workshop. The technology from 1895, based on driving a water turbine and cutting and grinding using natural grinding wheels is still functioning. All the excursions that visit this place are very impressed. And what about the glassworks? The glass has been hand-blown here until today, and it is mesmerizing to watch.
František Novosad knows very well to whom he can pass on his work. He believes that property is not to be owned, but to be preserved for future generations. It is a noble approach. His son Petr and small grandson Jakub are his guarantees. Could it be any other way at this magical place?
The stories unfold and intertwine. In 2013, the Harrach family held a family gathering in Jilemnice estate, and almost sixty family members came to visit. I wonder, what did they think about the family of Novosad taking care of Nový Svět?
Well, the Harrach family invited both the Novosad father and son to the event. Mrs Eleonora, who remembered the times when the Harrachs owned the glassworks, welcomed them, introduced and noted that she is happy that the former glory of the Harrach family is still shining, is in great shape and still focuses on quality. František Novosad is a worthy successor to Jan Nepomuk František, Count Harrach.
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Q: Cannot rollback to a suggested edit which was 'improved' by a reviewer This question of mine has had an edit suggested, which was improved by one of the reviewers. Because of this, both the suggested edit itself (revision 2) and the improved edit (revision 3) are added to the revision history.
I decided I like the suggested edit more than the improved one, so I'd like to rollback to revision 2. I get the familiar popup 'Are you sure you want to rollback', but when I confirm, the page reloads, but there is no rollback. (Normally, you'd see the new revision on top of the list.)
I double-checked with @rene in chat and it's happening for him as well. He mentioned that the suggested edit only added a tag - the rollback mechanism might be checking just the body and title of the question.
It might be that it's not intended at all to rollback to that revision, but then the rollback link should be disabled/hidden.
Based on a SEDE query I found another case where an improved tag-only edit cannot be rolled back to:

*

*Organizer.displayName is null for some users

*Compare String using tMap

*Rearrangement based on matching text in Excel?
It's not consistent, though, as for some other questions in that query (e.g. this one) the rollback works as intended.

A: There's a bit of history to this bug. But the problem boils down to one of establishing a consistent chronology when actions can happen concurrently:

*

*An Improved suggested edit always applies two edits simultaneously: the suggested edit, and the improved version. However, the Improved version must be considered a "later" revision.

*It is possible for two editors to independently submit an edit to the same post simultaneously, or at least close enough to one another that the processing overlaps. Which one should be considered "later" is debatable, but we should try to pick the same one consistently.

*Due to how edits are stored (title, tags and body each a separate row in PostHistory) and the existence of a grace period for edits, it is entirely possible for multiple revisions to share the same timestamp while having interleaved IDs for their component parts.

The canonical solution to this is the method used to generate the revision history (/posts/[id]/revisions):

*

*Group PostHistory rows by RevisionGUID

*Order them first by the lowest creation date for each group, then by the lowest ID for each group

For your post, that looks something like this. This occasionally produces some very weird results, but in normal situtations it does the right thing and does it consistently.
However, there was a different routine responsible for retrieving a specific revision (for editing or rolling back), and it used a much different approach, relying on the CreationDate of a specific revision's PostHistory entries being greater than all those preceding it and less than all those following. This broke pretty reliably whenever those assumptions didn't hold, which happens consistently when Improve is used.
The fix here involves establishing a consistent chronology using more or less the same approach as the one used to generate the revision history itself, and then relying on that to compose a specific revision. Something like this.

redpajama_set_name: RedPajamaStackExchange


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Local MP supports pub campaign
New battle against greenbelt housing
Options for Aire Valley crossing "known by Christmas"
'All we want for Christmas is the Skipton-Colne railway'
Ilkley Parking Survey
'Time for devolution deep breath'
From Yorkshire Post, 18th July 2017
Written by James Reed, Political Editor
ILKLEY'S MP John Grogan has stepped in to a row over redundancy pay at an Ilkley care home.Mr Grogan visited Hollycroft Care Home in Hebers Ghyll Drive after its closure was announced in June. During that visit Mr Grogan said AN MP has called for a "deep breath" to be taken over the issue of Yorkshire devolution after a minister suggested the south of the region should be grateful for the powers and money it has been promised. Keighley MP John Grogan urged council leaders, MPs and ministers to return to the issue with "open minds" after the summer and held out hope that a single deal to given Yorkshire more control over its own affairs could yet be struck.
He was speaking after Northern Powerhouse Minister Jake Berry told MPs it was time for people in South Yorkshire to "learn to take yes for an answer" and press ahead with their existing devolution deal.
The Government has expressed frustration at the failure to implement the Sheffield City Region deal first agreed in 2015 which included £30m a year in extra funding and the creation of an elected mayor for the area.
Chesterfield and Bassetlaw have withdrawn and any move to press ahead with a revised deal covering just the four South Yorkshire councils was put on hold at a meeting on Monday.
Doncaster and Barnsley are keen to explore alternative options including striking a deal for the whole of Yorkshire.
Mr Berry has rejected that idea and insisted the current deal must go ahead and other parts of Yorkshire put forward separate proposals.
Speaking in the Commons, Mr Berry said: "As a proud Lancastrian, it is not for me, nor is it for Government, to tell Yorkshire what devolution deal it should have.
"However, I gently point out that in 2015, Barnsley, Doncaster, Rotherham and Sheffield asked for powers from the Government and we gave them to them; they asked for new money from the Government and we gave it to them and they asked to have an election next May and we gave it to them.
"When will the people of South Yorkshire learn to take yes for an answer?"
Mr Berry was responding to Mr Grogan's suggestion that the minister had been "not entirely wise" to reject the case for a Yorkshire-wide devolution deal during a visit to Leeds last week.
Mr Grogan, who was previously the MP for Selby and was re-elected to the Commons in June, told The Yorkshire Post: "Clearly South Yorkshire devolution is not going to proceed as planned.
"I think over the summer holidays everybody needs to take a deep breath and come back with an open mind.
"From my few weeks back in Westminster it is already clear to me that Yorkshire is in danger of missing out big time to those areas which already have a devolution deal.
" I am an eternal optimist and I think it is still possible to get cross party agreement to proceed on a county-wide basis."
Greater Manchester, the West Midlands, Merseyside and Tees Valley are among the areas which elected mayors in May as part of devolution deals giving them more money and decision-making powers in areas such as business support, skills and transport.


When you ask for answers, you are just seeking instructions. Don't look for a turn by turn plan. A psychic reading is truly a method for you (by means of somebody else) to utilize unused senses to get some insight.
Whenever you want to get an e-mail psychic reading, you get a specific question and send it to the psychic. By utilizing email psychic readings, you know ahead of time how much it is going to cost.
In no way let a psychic reading bypass your typical sense and intelligence. If you are interested in free online reading, do not be reluctant to try an online psychic reading.
There are many various methods to check ones psychic abilities. As more people become mindful of using their psychic energy, more psychic tests are developed. We all have psychic powers to one degree or another. Utilizing this energy and welcoming it can assist it to grow more powerful all the time. There are a couple of psychic tests you can take to assist see how psychic you really are at this time.
The most popular psychic test is the Zener Cards. This test was actually developed by Dr. J.B. Rhine. Dr. Zener was a psychologist who carried out statistical experimental tests in extra sensory perception. The Zener cards have symbols or photos on them. How the test works is that the cards are mixed and after that the topic was asked to determine the symbol and guess or photo on the back of each card that was held up. There is now an online version that was later on established and has actually since ended up being incredibly popular.
The Zener cards are extremely well understood, they are not essential to truly check ones psychic capability. Anyone can do psychic tests from house by merely making their own cards and testing themselves.
Psychic tests can be enjoyable to practice especially with a buddy. If you do not appear to be extremely excellent at them right now, be careful not to get dissatisfied. All of us have the capability to use psychic energy and by setting the intention to establish your own, you will marvel how quickly your energy establishes. Try several psychic tests and practice the ones you are greatest at. Anything and everything is possible even being psychic. Rely on yourself and your instinct. Your energy will just get stronger.
You can attempt to sense when someone is coming closer to you by feeling his or her energy pattern. You can likewise begin to feel the energy that is kept in objects and see if you can inform anything about the individual who owns or owned the things simply by touching it.
If you have not had a lot of psychic experiences before, this might seem unbelievable, but relax, hold the product in your hand and picture it plainly in your mind, remembering that the energy of the individual is in the things. There's really a detailed process that can help you to "check out" objects, which you can learn quickly in just a matter of days.
In the beginning a few of this information is sure to be unreliable, incomplete or in some way puzzled. As with any other ability, it takes practice to take advantage of this energy and to understand what it is trying to inform you.
When attempting to develop your psychic abilities is that we are all energy and the universe is the very same energy, the most essential thing you can remember. There is no need to connect to a greater power or aircraft of presence to take advantage of your psychic abilities-it is already all within you.
A good phone psychic these days is most likely to have his or her existence understood online so if you have a preferred psychic that you desire to get a reading from, look up his or her site. You can likewise look for companies that use a complimentary psychic reading online when you sign up for their newsletters on their site.
If you're not extremely specific about the experience of the phone psychic you'll be talking to, you need to also check out the brand-new and up and coming psychics who are providing to do complimentary psychic chats in exchange for some lip service. There are a lot of psychics out there looking to break into the market so if you have the time to spare, take advantage of these complimentary readings.
You can just have the first part of the checking out complimentary, however who wants that, since as soon as you have had that then you will be wishing to know more about what is coming up for you. After a few minute you will aspire to have more as 5 minutes goes so quickly. If you have been believing about have a free online psychic reading but were not sure what to do, then this could be the best method for you.
All you do is search the web and see what sites are offering free readings and then take a look at the fine print and go from there. You will more than most likely have to pay for the remainder of the reading either through your phone costs or through your charge card. It doesn't really matter how you pay, just that you understand what you are getting. Generally a reading is around 20 minutes typically so if you get the very first 5 minutes free then you will still need to spend for the remainder of the reading need to you want the psychic to continue. The rates vary from psychic to psychic so take a look prior to you have your reading done so you understand precisely how much it may cost you.
In the end a totally free online reading suggests that you will get some of the reading free however not all of it. If this is for you or not, as long as you know that then you can go from there and decide.


When you're living in a college dorm room, you're going to have the overhead light lighting your entire room. While that's great, it's also a problem at night. The light switch for that overhead dorm light won't be conveniently placed by your dorm bed. Instead, it will probably be at the entrance to your dorm room. Know what that means? That means a very slow walk back to your dorm bed at night that may also involve hitting your knee on your dorm desk. Avoid that with cheap dorm supplies like our Peel N Stick Night Light.
Our Peel N Stick Night Light is battery operated, which is essential in a college dorm room. After all, electrical outlets are hard enough to come by as it is. The Peel N Stick nature of this dorm necessities item allows you to place it wherever you want to in your college dorm room so that it is convenient for both you and your dorm roommate. This dorm essentials item is perfect for whether you're walking to your dorm bed after turning out the overhead light or for when you need to get up in the night to use the dorm bathroom. Plus, when it's not in use it can even add to your dorm room decor!


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Despite co-signs from Tricky Stewart and The-Dream and a major deal with Def Jam, the ladies of Electrik Red still have yet to make their mark on the charts. With the release of their debut album, How to Be a Lady (Vol. 1), postponed till May 26th, the eclectic girl group is pushing their single (and best shot at a hit) So Good at radio and music video outlets. Now, just how confident are Naomi, Sarah, Lesley, and Binki that they'll achieve success this time around? They've already released the official remix, for which they've snagged up the biggest groupie-magnet in music today—that's right, Weezy F. Baby (sans Autotune) shows up to flaunt his heartthrob status, knowing that once a woman's been with him, nobody else will do! For some vinyl hard cider (yes, I did coin that phrase), pick up How to Be a Lady (Vol. 1) when it drops April 21... then get ready for even more of Weezy's showoff antics on rock album Rebirth set to drop June 9.


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Q: Formatting a drive that is protected with BitLocker I am planning to do a complete format on my computer. My HDD (which everything is located) is encrypted with BitLocker. A few months ago I tried to manually decrypt the drive to not cause any error before format but it literally took ages to even decrypt. Since I don't care about the data, is it OK for me to format a Bitlocker encrypted drive? Will it cause any problems or errors?

A: There's nothing at all wrong with formatting a BitLocker-protected drive. Doing so removes BitLocker - in a sense, when you encrypt the disk you are "Formatting it with BitLocker" because BitLocker takes over the space where filesystem metadata usually goes and moves it elsewhere - and it will be as if you'd just formatted any other disk.
You will of course lose all data, even if you don't do a full format - and I recommend only doing a quick format because, as @MichaelKjorling pointed out, a full one takes approximately "ages" - because it will no longer be possible to decrypt the disk's old data. I'm guessing you're fine with that, though.

A: If you are not worried about date, simply format it. Bitlocker does not stop you from doing so. It won't cause any problems or errors.


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Смітсон Теннант — англійський науковець, хімік.
 Девід Теннант — шотландський актор, п'ятиразовий лауреат британської Національної телевізійної премії, премій «Еммі» (2013), БАФТА Уельс (2007) та БАФТА Шотландія (2014) та ін.
 Ніл Теннант — соліст британського дуету Pet Shop Boys.
 Вікторія Теннант — британська кіно- та телеакторка.

Теннант — місто в штаті Айова
 Теннант — переписна місцевість у штаті Каліфорнії

redpajama_set_name: RedPajamaWikipedia


This bag is in the Rustic Ranch range produced by Sew Easy.
It is made from a black quilted fabric with an owl design. There is a large exterior pocket that is trimmed with yellow and the interior of the bag is lined with a yellow fabric. There is an interior zipped pocket. There are two handles for carrying the bag. It fastens with a magnetic catch inside the top of the bag.


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New PS2 TT-Superbikes Game: Release Date 1 Week Later
Jester Interactive Publishing, creators of PS2 games, and Duke Marketing, the powersport specialists, today announce that the "TT Superbikes" PlayStation 2 game release date for UK & Ireland has been moved from 22 April to 29 April 2005.
Clark Evans, Director of Jester, said: "Once again we apologise for this short delay, which is due to the unexpected demise of our UK distributor Big Ben and the time taken to establish Duke as the new supplier for the UK. One additional week is needed for Duke to take over the UK retail orders; ensuring stock is delivered to all retailers on the same release date, which will now be the 29th April.
Peter Duke commented: "We have been in discussion with the major retailers who are sympathetic to our being appointed as distributor at very short notice. We are anxious to ensure sufficient stock is ready for the release date and this proved to be impossible for April 22nd due to the high level of initial demand. After up to a year of anticipation another week is a small price to pay to ensure that all UK retailers receive stock at the same time."
TT Superbikes for PlayStation 2 (SRP £34.99) is now released on 29 April 2005 and is available from leading outlets or direct from Duke Video: tel 01624 640000, fax 01624 640001 or email mail@dukevideo.com. Visit www.dukevideo.com online.
For further information, to arrange review copies or digital packshots please contact Gavin Smith at Duke Video: Tel 01624 640013, fax 01624 640001 or email mktg@dukevideo.com or email ttinfo@jesterinteractive.com. Visit www.jesterinteractive.com


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Throwback Thursday – The Car Phone
September 25th, 2014 TOSS C3 Throwback Thursday car phone, old tecnology, Throwback Thursday
Back in the 80's or 90's you may have seen one of these rare creatures in wild, an ordinary telephone mounted on the center console of a car. Back then these car phones were very expensive and seen as a luxurious high end piece of technology. It seems strange to say that now since we all carry around mini computers in our pockets with our smart phones, but 25 years ago this was the tool of the rich and famous.
The car phone actually led AT&T to develop the Mobile Telephone Service in 1947, it was only implemented in around 100 highway towns but was then upgraded in 1965 while cellular technology was being developed. The main problem with the car phone was that the idea for a cellular device came a year after the first car phones appeared. Then in 1984 there was a switch to analog cellular services and mobile communications shifted to cellular technology making the car phone obsolete. Despite their short life cycle we can still thank the car phone for starting the mobile phone trend and being a catalyst for the smart phones and mobile technology we enjoy today.
Believe it or not there are still some car phones floating around today where cell service is very limited. They are rare, but you can still find some people who can say "I'm calling you from the car!" and mean it.
Written By: Sam Watkinson
Marketing Associate TOSS C3


When you purchase your massage recliner the shipping company will deliver it right to your doorstep, usually within 1-2 weeks from the day your order is placed. It's in a pretty big box(es). You will be responsible for unpacking it, assembling it, and then cleaning up all the packing materials. It's not hard to do, but the thought of doing all that intimidates some folks. Others just don't have the time or energy to do it. So, for those of you who don't want to deal with any of that when your new massage chair arrives, then White Glove Service is just what the doctor ordered!
Just check the "White Glove Service" option to include in your shopping cart when adding your chair (if White Glove Service is included with the chair you are purchasing, it will already be checked off). Once you've done that, the only time you'll have to lift a finger is to push the "Place Order Now" button here on the website and to push the remote control buttons of your brand new chair. We will not only have your chair delivered to your home, but we will have it unpacked, assembled, put exactly where you want it in your home, and then have all the cardboard and packing material cleaned up (by the way, you might want to hold onto the original packing material, since you'll need it if, for some reason, you want to return your chair within 90 days).
IMPORTANT! This is a separate service from the regular shipping costs. We highly recommend this wonderful option for your benefit. The cost of this service is $199.99 per chair. Expect delivery to take anywhere from 2-4 weeks for delivery when ordering White Glove Service. If you decide to add white glove delivery after your purchase and after your chair has already shipped from the warehouse, you will be responsible for any additional costs incurred by making the change (and it almost certainly will cost substantially more than the white glove price we offer on our website).


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Lesch Nyhan Syndrome
Lesch Nyhan syndrome (LNS) is a hereditary disorder of purine metabolism which consists in a severe form of hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency that leads to a characteristic uric acid overproduction (UAO), as well as neurological and behavioral problems.
WIKIDATA, CC BY 3.0
The clinical manifestation of LNS has an early onset, as many patients begin to show clear signs within the first six months of life. Urate crystal formation, the direct consequence of increased uric acid levels, is the first sign to appear, manifesting itself indirectly through the appearance of orange colored deposits called "orange sands" in the diapers of affected children. Unfortunately, even though this can be an important significant clue for the future manifestation of the disorder, it is rarely diagnosed and taken into consideration. The appearance of crystals, in fact, occurs later during late teenagerhood or early adulthood after distressful episodes of pain and swelling, especially in the joints [7], which tend to increase in frequency as the disorder progresses on. It is important to note that in older children the development of sodium urate in cartilaginous tissues can also be observed.
Stone formation is another sign which appears very early. The stones in the urinary tracts might determine the presence of blood in the urine, in a condition known as hematuria, as well as the occurrence of infections.
Other musculoskeletal problems include the appearance of severe muscle spasms, characterized by the tendency of the back to severely arch, combined with the tendency of head and heels to bend backward, in a condition known as opisthotonos. These are associated with other characteristic events such as hip dislocation, fractures, abnormal spine curvature (scoliosis), and permanent joint fixation in flexed positions.
The neurological signs of LNS usually appears later in life, at around 12 years of age. They classically include involuntary writhing movements of arms and legs (dystonia) as well as repetitive movements performed with no apparent reason such as flexing the fingers, raising or lowering shoulders, and grimacing the face. Other neurological signs include those which show the failure of the correct developmental process, such as delays in reaching important stages like crawling, sitting upright and walking. Many also show problems correlated with muscle tone, with muscles appearing with either an abnormally high tone (hypertonia) or an abnormally high rigidity (spasticity). It is also possible to observe deep tendon reflexes (hyperreflexia) or the incapability by the side of the children of holding the head in an upright position. Mental retardation can also appear after a while, but this important sign might be difficult to detect due to poorly articulated speech (dysarthria).
One of the most important behavioral problems, observed in around 85% of the cases, is self-mutilation, which tends to appear very early usually within the first 2-3 years of life. These behavioral problems can include repeated biting of lips, fingers, and hands, frequent banging of the head on walls or other hard objects, and scratching of the face, which are often combined with aggressiveness, vomiting, and spitting. Irritability and screaming are also frequent.
AS previously indicated, the clinical signs of LNS mostly appear in men, even though many females might develop gout later in life as a result of untreated uric acid accumulation in the blood [8].
We report on a 7-year-old boy with Lesch-Nyhan syndrome, lacking self-mutilative behavior, who was erroneously diagnosed as having athetotic cerebral palsy. [ncbi.nlm.nih.gov]
DOID:1919 - Lesch-Nyhan syndrome A purine-pyrimidine metabolic disorder characterized by mental retardation, spastic cerebral palsy, choreoathetosis, uric acid urinary stones, and self-destructive biting of fingers and lips that has_material_basis_in [xenbase.org]
Because Lesch-Nyhan syndrome can mimic cerebral palsy, it is important to screen children with an initial diagnosis of cerebral palsy for Lesch-Nyhan syndrome, especially if the prenatal and perinatal courses were normal. [contemporarypediatrics.com]
Neurological problems, especially general learning disability, spastic cerebral palsy and choreoathetosis. Behavioural disorders. [patient.info]
Megaloblastic anemia is frequent and may be severe. Microcytic anemia may occur. UAO may result in joint inflammation, gouty arthritis and urolithiasis. Renal failure or acidosis occur rarely. [orpha.net]
2016 2017 2018 2019 Billable/Specific Code Applicable To Amino-acid deficiency anemia Orotaciduric anemia Type 1 Excludes Lesch-Nyhan syndrome ( E79.1 ) Arthropathy M12.9 - see also Arthritis ICD-10-CM Diagnosis Code M12.9 Arthropathy, unspecified 2016 [icd10data.com]
Because a lack of HPRT causes the body to poorly utilize vitamin B12, some boys may develop a rare disorder called megaloblastic anemia. Treatment Treatment for LNS is symptomatic. [ninds.nih.gov]
Short Stature
stature Decreased body height Small stature [ more ] 0004322 Testicular atrophy Testicular degeneration 0000029 Vomiting Throwing up 0002013 X-linked recessive inheritance 0001419 Showing of 31 | Last updated: 6/1/2019 Lesch Nyhan syndrome is inherited [rarediseases.info.nih.gov]
Muscle Hypotonia
These include uncontrollable movements in the arms and legs (dystonia), repetitive movements (chorea) such as raising and lowering of the shoulders, and/or facial grimacing, and low muscle tone or soft muscles (hypotonia). [thinkgenetic.com]
It affects males and is characterized by neurologic defects, moderate mental retardation, muscle hypotonia, and a tendency for self-mutilation (self-biting of lips, tongue, and fingertips). [icd10data.com]
Manifestations of the disease begin to appear at 3–6 months of age with muscle hypotonia and difficulties in maintaining the sitting position and supporting the head. [mdpi.com]
Self-Mutilation
Affected children are rarely able to resist self-mutilation. [contemporarypediatrics.com]
Lesch-Nyhan syndrome is a very rare X-linked recessive disorder characterized by mental retardation, spasticity resembling cerebral palsy, choreo-athetosis, self-mutilation and hyperuricemia. Self-mutilative behavior is a hallmark of the disease. [ncbi.nlm.nih.gov]
Major manifestations include mental retardation and self-destructive behavior resulting in self-mutilation through biting and scratching. [oooojournal.net]
Self-Inflicted Wound
He was referred to the Dental Unit from the Department of Pediatric Neurology for evaluation and management of self-inflicted wounds on fingers, lips and cheeks associated with weight loss and decreased food intake. [ncbi.nlm.nih.gov]
Main symptoms and co-morbidities for Lesch-Nyhan syndrome include: Various degree of physical disability Delayed growth and puberty Gouty arthritis with flare-ups Kidney and bladder stones Infections of self-inflicted wounds Megaloblastic anaemia Cardiovascular [gii.co.jp]
This syndrome is characterized clinically by mental retardation, chorea, athetosis, hyperuricemia, uricosuria and self-mutilation. [ncbi.nlm.nih.gov]
[…] uncontrolled aggressive and/or compulsive actions, muscle weakness (hypotonia), uncontrolled spastic muscle movements, and neurological problems such as involuntary writhing movements of the arms and legs (athetosis) and purposeless repetitive movements (chorea [web.archive.org]
Chorea — Involuntary, rapid, jerky movements. Chorionic villus sampling (CVS) — A procedure used for prenatal diagnosis at 10-12 weeks gestation. [medical-dictionary.thefreedictionary.com]
Spastic Gait
There is another LNV, HPRT Salamanca, that is characterized by spastic gait, mental retardation, and skeletal abnormalities.4,6 We describe a family with 3 affected individuals with LNV. [jamanetwork.com]
Legs were increasingly spastic. There was no self-injurious or impulsive behavior. On examination he appeared normal. Speech was readily intelligible and of normal syntax and cadence. He walked without assistance with a spastic gait. [dx.doi.org]
Syndrome of mild mental retardation, spastic gait, and skeletal malformations in a family with partial deficiency of hypoxanthine-guanine phosphoribosyltransferase. Pediatrics 79: 713-717, 1987. [ncbi.nlm.nih.gov]
Choreoathetoid Movements
Pharmacologic treatment of agitation and choreoathetoid movements is variably successful. Diazepam is sometimes useful in reducing agitation; antipsychotic agents have shown mixed results. [contemporarypediatrics.com]
They have in addition a variety of neurological abnormalities including mental retardation, spastic cerebral palsy, and involuntary, choreoathetoid movements. Involved patients have unusual, compulsive, aggressive behavior. [link.springer.com]
Hence, he usually presented with marked motor incoordination, hyperreflexia, hyperkinetic choreoathetoid movements, and self-harm behavior with autoinflicted lesions and ataxia, all of which were intensified by stress. [cureus.com]
Abnormal Gait
gait and poor speech. [jamanetwork.com]
Indifference to Pain
Kapasi AY, Trasi SS, Khopkar US, Wadhwa SL (1992) Congenital indifference to pain. Indian J Dermatol Venereol Leprol 58: 273-276. Sawyer RJ, Richmond MN, Hickey JD, Jarratt JA (2000) Peripheral nerve injuries associated with anaesthesia. [heraldopenaccess.us]
Measurement of HPRT enzyme and its function in blood and tissues is the definitive diagnosis for LNS. This is usually confirmed by the identification of mutations in the HPRT1 gene.
Lab Studies
Uric acid overproduction can be easily revealed with blood and urine studies usually performed in clinical practices. However, it has to be remembered that sometime hyperuricemia can be caused by other conditions, while many patients affected by LNS might have levels of uric acid within the normal ranges. Therefore, uric acid levels cannot be used to confirm the disorder. Particularly in use are the 24-hour samples, even though they can be very difficult to collect. The calculation of the ratio between urinary uric acid and creatinine from urine specimen can be used as alternative method, but this approach is not as informative as the previous one.
No evident structural malformation in the brain of the patients affected by LNS can be detected by using neuroimaging studies such as CT scan and MRI [6]. However, they might reveal small reductions in brain volume, which frequently go unnoticed in routine imaging studies. The brain volume loss especially occurs in the area of the basal ganglia and frontal cortex, without involving occipital lobes and cerebellum [9]. Sometime, complications can reach the spinal cord, especially in the cervical portions, causing degenerative joint disease that damage the spinal cord itself and the emerging nerve roots nearby.
Experts also recommend the use of noninvasive imaging studies for the kidneys and the urogenital system, especially to detect in advance the occurrence of infections and stone formation when they still remain asymptomatic. On this regard, ultrasonography is highly recommended in most of the cases.
Nephrolithiasis
Death is usually from aspiration pneumonia or complications of nephrolithiasis. Sometimes there is sudden death of unknown cause. [patient.info]
Nephrolithiasis can occur in children who are younger than 6 years; subcutaneous tophi of urates and gouty arthritis may develop later. The chief complication is renal failure secondary to nephrolithiasis. [contemporarypediatrics.com]
Affects 1 per 380,000 individuals, which are nearly all male given the X-linked inheritance 1. hyperuricemia-related symptoms such as gouty arthritis and uric acid nephrolithiasis 1,2 neurological symptoms which can include extrapyramidal signs such as [radiopaedia.org]
Hyperuricemia leads to hyperuricuria and uric acid nephrolithiasis. The underlying defect is a deficiency of hypoxanthine-guanine-phosphoribosyl transferase. [ncbi.nlm.nih.gov]
Hyperuricosuria
We report on a 16-day-old male with metabolic acidosis, hyperuricemia, hyperuricosuria, and nephrocalcinosis caused by Lesch-Nyhan syndrome. [ncbi.nlm.nih.gov]
Uric Acid Increased
The laboratory tests showed hyperuricemia, urinary uric acid increased, and deficiency of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT) that belongs to purine metabolism. [scielo.br]
Treatment is mostly aimed at addressing specific symptoms, through a strategy of coordinated efforts performed with the intervention of several specialists.
The pharmacological approach is mostly based on the use of allopurinol, which helps control the excessive amounts of uric acid in the blood and tissues [10]. However, this drug has no influence on the behavior and the neurological state of the patient, which remains negatively affected.
Stone formation can be treated using extracorporeal shock wave lithotripsy [11], a particular procedure which consists in immersing patients in water and treating them with high energy shock waves. Thanks to this approach, stones eventually dissolve and the many little pieces produced are finally eliminated through urine.
Neurological problems are usually addressed by using baclofen, benzodiazepines, or diazepam. However, no drug treatment has proved to be fully and evenly effective so far in dealing with this clinical side of LNS. On the contrary, behavior modification techniques have turned out to be useful in dealing with self-injurious behavior, especially when combined with the use of physical restrain of hips, chest, and elbows, or the use of mouth guards to avoid biting fingers and lips. Behavioral abnormalities can some time be treated with drugs. On this regard, it is important to remember depakote (sodium valproate), gabapentin, baclofen, and carbamazepine. Benzodiazepines should also be added to this list, as it can effectively treat the anxiety frequently associated with LNS.
The clinical consequences of LNS are very severe. Many patients die after aspiration pneumonia and the aftermaths of chronic nephrolithiasis and renal failure. Even with the best clinical treatment, just a few patients reach 40 years of age and many remain confined to wheelchairs [4].
LNS is a classical X-linked disorder, in other words a pathological condition which is caused by one or more mutations of the genes present in the X chromosome [3], one of the two sex-determining chromosomes (or allosomes) in many animal species. These types of genetic disorders mostly occur in males, as they have one single X chromosome. Therefore, to see the phenotypic expression of these mutations, it is sufficient to have one single mutated gene. On the contrary, LSN appears much less frequently in females, as they mostly have two X chromosomes. Therefore, in a female the expression of a X-linked mutation, which appears as a recessive character, occurs only when the two copies of the gene in both X chromosomes are mutated. Thus, females more frequently end up becoming simple carriers of the disorder.
As previously stated, the gene involved in the development of LNS is HPRT1, located on the long arm of the X chromosome in the Xq27 position. Its mutations inevitably result into a severe deficiency of HPRT and an abnormally high uric acid accumulation in the blood.
The prevalence of LNS has been estimated over the years to vary in the range going from 1 case for every 235,000 to 1 case for every 380,000 live births [4]. The disorder has been observed in all races and ethnic groups, with no particular predilection. As stated above, because of its X-linked nature, the disorder mostly appears in males rather than females, which mainly remain simple carriers. Affected individuals are diagnosed very early in life, usually before the age of 4. It is important to note that at the moment the prevalence of the HPRT deficiency is still unknown.
HPRT is involved in the recycling process of purine bases. If the function of this enzyme is compromised, the purine recycling process is compromised as well, and as a result the purine bases are degraded and excreted as uric acid. This then leads to an increase in the synthesis of the purines, perhaps for some still undetected compensation mechanism. These two factors combined, the failure of the recycling process and the increased purine synthesis, are lastly responsible for the uric acid overproduction [1].
The direct consequence of uric acid overproduction is persistent hyperuricemia which, as the uric acid is already near the solubility limit, finally causes uric acid precipitation and the subsequent crystallization in the several tissues in the body. Crystallization, in turn, leads to the formation of the tophi along skin surface, especially in the joints, and the inflammatory reaction called gouty arthritis. Another direct consequence to remember is the increased excretion by the side of kidneys into the urogenital system, which in turn increases the risk of stone formation into the urinary tracts, and therefore the risk of the associated urine flow blocks and infections.
As to the pathogenesis of the behavioral and neurological signs, the mechanism that finally leads to these problems have not been completely understood yet [5]. However, some neurochemical and neuroimaging studies have underlined the presence of significant abnormalities associated with the dopamine neuron function in the basal ganglia. In addition, neuropathological studies suggest the presence of a neurodevelopmental defect with no signs of degenerative process [6], that must somehow be connected with the behavioral and neurological profile. Nevertheless, the question about the mechanism underlying the effects of the HPRT over the dopamine systems and therefore the appearance of the behavioral and neurological consequences is still hotly debated among experts.
As LNS is a genetic disorder, no specific measure can be suggested to prevent it. However, genetic counseling is highly recommended, as it might allow parents and physicians to organize a treatment plan in advance.
Lesch Nyhan Syndrome (LNS) is a genetic disorder which is characterized by three major symptoms, as indicted previously: a characteristic overproduction of uric acid, combined with neurological and behavioral problems [1]. The over production of uric acid is usually associated with hyperuricemia and on the long run, if left untreated, it might lead to nephrolithiasis and associated complications such as renal failure, gouty arthritis, and deposits of uric acid on the surface of the skin called tophi. Dystonia appears to be the main neurological complications, but it can be combined with other problems such as spasticity, ballismus, choreoathetosis, and hyperreflexia. Lastly, the behavioral problems include intellectual disability, often combined with aggressive and impulsive behaviors which often cause self-injuries.
In addition to the full form of the disorder, characterized by the full manifestation of all the clinical signs underlined, milder forms have also been observed over the years [2]. In these milder variants, while uric acid overproduction is likely to be always present, affected individuals can show no self-injurious behaviors, normal levels of cognition, and no dystonia at all. These cases are collectively referred to as LNS variants and their occurrence can only be diagnosed by screening HPRT gene and revealing HPRT deficiency.
Unfortunately, treatment is limited and mainly based on the use of products such as allopurinol which controls uric acid overproduction and reduces the possibility of developing nephrolithiasis and gouty arthritis. Neurological and behavioral problems, instead, are frequently addressed with a combination of behavioral modification techniques and pharmacological treatment based on drugs like baclofen and benzodiazepines.
Lesch Nyhan syndrome (LNS) is a genetic disorder which consists in a disruption of the normal mechanism to build and break down purines, one of the components of DNA and RNA.
LNS is caused by mutations of the gene for hypoxanthine guanine phosphoribosyltransferase, one of the enzyme responsible for purine recycle process. If its function is compromised, purines can no longer be recycled, and as consequence are turned into uric acid which begins to build up throughout the body. The hypoxanthine guanine phosphoribosyltransferase gene can be found as recessive train carried by the X chromosome. For this reason, the disorder is predominantly observed in males.
Motor symptoms are the first to be noticed, usually between the first months of life, especially as delays in motor development, hypotonia, or spasticity. These are then followed by involuntary movements, especially between the sixth and the eighteenth month of age.
Hyperuricaemia, the abnormally high level of uric acid in the blood, is another important clinical sign of LNS. This is usually associated with the appearance of orange sands throughout the skin surface, as a consequences of the formation of uric acid crystals and gout.
These initial signs are then followed by neurological and behavioral problems, which eventually lead to the appearance of self-injurious behaviors towards the age of 12 years.
The diagnosis of LNS is largely made with a thorough clinical evaluation based on the history of the patient and specialized blood tests. The tests are used especially to detect the high concentration of uric acid in the blood, even though the final diagnosis has to be confirmed with a genetic analysis to reveal the mutations on the HPRT 1 gene associated with LNS.
The most important drug used against LNS is allopurinol, employed to treat hyperuricaemia and its complications. Other important drugs include baclofen and benzodiazepines, used in combination to treat spasticity, and diazepam.
No treatment has proved to be fully effective against neurological and behavioral problems, which can be best addressed by using behavioral modification techniques. Furthermore, while treating the patient it is very useful to use physical restrains and month guards to avoid physical damages due to self-injurious behaviors.
Jinnah HA, Friedmann T. Lesch-Nyhan disease and its variants. Scriver CR, Sly WS, Childs B, Beaudet AL, et al, eds. The Molecular and Metabolic Bases of Inherited Disease. 6th ed. New York, NY: McGraw-Hill; 2000. Chapter 107.
Jinnah HA, Ceballos-Picot I, Torres RJ, et al. Attenuated variants of Lesch-Nyhan disease. Brain. 2010 Feb 22.
Jinnah HA, De Gregorio L, Harris JC, et al. The spectrum of inherited mutations causing HPRT deficiency: 75 new cases and a review of 196 previously reported cases. Mutat Res. 2000 Oct; 463(3):309-26.
Torres RJ, Puig JG. Hypoxanthine-guanine phosophoribosyltransferase (HPRT) deficiency: Lesch-Nyhan syndrome. Orphanet J Rare Dis. 2007 Dec 8; 2:48.
Visser JE, Bar PR, Jinnah HA. Lesch-Nyhan disease and the basal ganglia. Brain Res Brain Res Rev. 2000 Apr; 32(2-3):449-75.
Jinnah HA, Visser JE, Harris JC, et al. Delineation of the motor disorder of Lesch-Nyhan disease. Brain. 2006 May; 129(Pt 5):1201-17.
van der Zee SP, Schretlen ED, Monnens LA. Megaloblastic anaemia in the Lesch-Nyhan syndrome. Lancet. 1968 Jun 29; 1(7557):1427.
Jinnah H A, Friedmann T. Lesch-Nyhan disease and its variants.In: Scriver C R, Beaudet A L, Sly W S, Valle D. (eds.) : The Metabolic & Molecular Bases of Inherited Disease. Vol. II. 8th ed. New York: McGraw-Hill 2001. P. 2537.
Bakay B, Nissinen E, Sweetman L, et al. Utilization of purines by an HPRT variant in an intelligent, nonmutilative patient with features of the Lesch-Nyhan syndrome. Pediat Res. 1979; 13:1365-1370.
Torres RJ, Prior C, Puig JG. Efficacy and safety of allopurinol in patients with hypoxanthine-guanine phosphoribosyltransferase deficiency. Metabolism. 2007; 56:1179-1186.
Morino M, Shiigai N, Kusuyama H, et al. Extracorporeal shock wave lithotripsy and xanthine calculi in Lesch-Nyhan syndrome. Pediatr Radiol. 1992; 22:304.


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Winner will be contacted via e-mail after giveaway ends, and will have 48 hours to respond before a new winner is chosen. One entry per household, winning entry will be verified. Odds of winning depend on valid entries received. A Modern Day Fairy Tale is not responsible for prize delivery. So Easy Being Green, Viva Veltoro, and the It's Fall Y'all participating bloggers are not responsible for sponsors who do not fulfill prize obligations.
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Tilmans, O; Bühler, T; Poese, I; Vissicchio, S; Vanbever, L; (2018) Stroboscope: Declarative Network Monitoring on a Budget. In: Banerjee, S and Seshan, S, (eds.) Proceedings of 15th USENIX Symposium on Networked Systems Design and Implementation - 2018. (pp. pp. 467-482). USENIX Association: Renton, WA.
For an Internet Service Provider (ISP), getting an accurate picture of how its network behaves is challenging. Indeed, given the carried traffic volume and the impossibility to control end-hosts, ISPs often have no other choice but to rely on heavily sampled traffic statistics, which provide them with coarse-grained visibility at a less than ideal time resolution (seconds or minutes). We present Stroboscope, a system that enables finegrained monitoring of any traffic flow by instructing routers to mirror millisecond-long traffic slices in a programmatic way. Stroboscope takes as input high-level monitoring queries together with a budget and automatically determines: (i) which flows to mirror; (ii) where to place mirroring rules, using fast and provably correct algorithms; and (iii) when to schedule these rules to maximize coverage while meeting the input budget. We implemented Stroboscope, and show that it scales well: it computes schedules for large networks and query sizes in few seconds, and produces a number of mirroring rules well within the limits of current routers. We also show that Stroboscope works on existing routers and is therefore immediately deployable.