Last week, former police officer Derek Chauvin was sentenced to 22 and a half years in prison for the May 2020 killing of George Floyd. While his sentence marked a historic court ruling, those closest to Floyd are still coming to terms with his loss. Former NBA champion Stephen Jackson is one of those people, and during a recent podcast, the former NBA player became emotional while discussing the tragic death of his childhood friend.
RELATED: Stephen Jackson Disses Donald Sterling In Rap Song
RELATED: Derek Chauvin Finally Breaks His Silence With Message To George Floyd’s Family
“The tears will never go away because I really lost family,” Jackson said on Portal A’s “Charges With Rex Chapman” podcast.
Both Jackson and Floyd were raised in Houston, Texas. Jackson said that during the height of the Floyd protests, his phone rang off the hook with supporters, but, one year later, those supporting voices have lessened.
“Hearing Matt [Barnes] talk, it just took me back to that place,” a tearful Jackson said. “I’m still doing the work… my tears are still here.”
Jackson said he considered Floyd a brother, and his loss was felt throughout his immediate family.
“The tears will never go away, my momma will tell you… she even thought [George Floyd] was her son at times when she saw him, that’s how much we looked alike.” He said. “These tears are not fake, they’re real, so when I feel it they come out.”
Chauvin was sentenced to 270 months – 22 and a half years – for the murder of Floyd, with 199 days credit for time already served. Even though Chauvin was found guilty of three counts, he could only be sentenced on the most serious crime — second-degree murder under Minnesota law.
“I ask about him all the time,” Gianna Floyd said of her father during her victim impact statement. “I miss him and I love him.”
The seven-year-old was the first of four family members to speak during Chauvin’s sentencing. When asked what she would do if she ever saw her dad again, she replied, “I want to play with him.”
Floyd’s nephew, Brandon Williams, asked that the maximum penalty be imposed.
“What was going through your head when you had your knee on my brother’s neck?” Terrence Floyd asked Chauvin. Like Williams, he asked that his brother’s murderer receive the maximum sentence.
Philonise Floyd says nightmares of his older brother begging for his life haunt him to this day.
“George’s life mattered,” Philonise said. “My family and I have been given a life sentence, we will never be able to get George back.”
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Former NBA Player Stephen Jackson Still Grieving Over Childhood Friend George Floyd was originally published on wzakcleveland.com
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In the middle of two weeks’ leave from the workplace, and slowly spinning down.
I roast T-bone steaks and wash floors and thin out the vegetable garden.
I listen to the scimitar-billed Wood Hoopooes and read the news.
I paint the outer walls of the house and burn Myrrh and Rose to the New Moon.
I commiserate with canine companions feeling poorly and clean out overstuffed bedside drawers.
I look at pictures of tornado damage in the United States and read the complete works of H P Lovecraft.
I dream with a strange aura.
And, above and beneath all, I am held in wonder and awe by the recurring thought which slips across my mind every day:
That I entered the River of Time over 54 years ago, and –miribile visu – I am still progressing down it.
Pic: Original art by Wang Nong

domain: artistic


text:
The German national team manager Joachim Low is reportedly keen to persuade Bayern Munich youngster Jamal Musiala to represent them over England.
The highly-rated 17-year-old looks a huge prospect after his breakthrough in the Bayern team, with Liverpool and Manchester United already emerging as transfer suitors for the former Chelsea starlet.
MORE: How Solskjaer convinced Van de Beek to snub Real Madrid & Bayern Munich for Man Utd transfer despite limited opportunities
Now Sport Bild claim there could be another England vs Germany row over Musiala, with Low eager to ensure he is fast-tracked into the German national side.
Musiala is able to represent either country due to being born in Germany but spending most of his life living in the UK.
The teenager currently plays for England at Under-21 level, but has previously also represented Germany’s Under 16s.
It remains to be seen where Musiala will ultimately decide his loyalty lies.

domain: news


text:
Ilford, United Kingdom
I was born and brought up in Melton Mowbray, Leicestershire. Since graduating from art school I have lived and worked in London in the UK.
I have worked in many areas of art over the years. Disciplines into which I have ventured include painting & drawing, iPad art, photography, image & text, short stories, poetry and performance.
The abstract art in this selection reflects a variety of interests and stages of development. I have chosen pieces that I think especially lend themselves to reproduction through the high quality process known as giclee printing. The prints on show are made to archival quality whilst remaining at an economical price by virtue of their small scale.
Work arrives through its own logic and develops under its own momentum; and whilst sources and content are diverse, themes are still discernible.
The viewer takes from the work whatever it gives to them, however close or distant it may be in relation to my initial inspiration and ideas.


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The Alchemist Code is Now Available on iOS and Android
The official launch of the strategy RPG, The Alchemist Code by gumi is now live on iOS and Android! It’s a full-scale, gorgeous SRPG on mobile device and is based on the Japanese mobile game, “For Whom The Alchemist Exist” (誰ガ為のアルケミスト) and is prepared to captivate a new audience. Easy-to-learn, this turn-based RPG has a deep job and level system reminiscent of classic turn-based SRPGs. Players will have to be aware of casting times for magical spells, and which types of attacks are strong or weak versus their foes as they scale beautiful stages, collecting treasures and defeating their foes.
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domain: game


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BCS: The Attack of the One-Loss Teams Is Upon Us
This week the BCS lost Clemson and Kansas State from the unbeaten ranks. There are now only five undefeated teams left, not counting Houston, and after next week there will be no more than four.
You know what that means? Which team is the best one-loss team? There is a very solid chance that we may have to start comparing these teams. So, with that in mind, who gets the nod right now? Also, bear in mind that either LSU or Alabama will be joining this group Saturday night.
The Sooners had a pretty rough October 22nd hiccup against Texas Tech that nobody saw coming.
Be that as it may, they came back by exposing and overmatched Kansas State team. We all kinda thought the Wildcats would take a loss soon, but not the 58-17 beating they got from the Sooners this past weekend.
I would give the Sooners a slight inside track, especially since they still have Bedlam against No. 3 Oklahoma State on the schedule.
Arkansas has a rough road of competition ahead and needs help.
The Razorbacks have already logged a loss to Alabama. They play SEC Eastern hopeful South Carolina next and still have LSU to end the season. That's a lot of strikes already.
They must win out, hope that Alabama loses to LSU and someone else, and win the SEC Championship. That's probably unlikely.
I like the prospects of Oregon.
Yes, Oregon lost pretty handily to LSU in their season opener, but that obviously was early. The Ducks are getting better every week and gaining momentum. They still have Stanford in what is looking like the game of the year in the PAC-12. They also have to face the winner of the South Division if they should win out.
The offense seems to be clicking, the defense is playing better, and they're now returning from injury offensive stars Darron Thomas and LaMichel James.
If the SEC and Big XII cannibalize themselves, the Ducks may be sitting there waiting.
South Carolina lost it's best player (Marcus Lattimore) and starting quarterback (Stephen Garcia), but they've been playing stellar defense and that could go a long way.
Just ask the Ol' Ball Coach, the last two wins haven't been pretty (14-12 and 14-3), but a win is a win. Their schedule isn't horrible, but Arkansas will be going hard test this week and Clemson in the rivalry game could be a great matchup and they play FCS Citadel before the Tigers as a prep game.
Georgia could still win the East Division, but the Gamecocks still have a solid shot with their defense.
Nebraska's lone loss is doing nothing to help them as Wisconsin can't get out of their own way now of late.
They do, however, have two ranked opponents left—No. 15 Michigan and No. 16 Penn State—that few expected to be in such position at this point in the season. That will do wonders to overcome strength of schedule issues.
The Huskers still have to rely on Taylor Martinez, who has issues throwing the ball, but their defense seems to be finding it's identity again. If they can keep things together on offense they could run the table in the Legends Division and have either a rematch with Wisconsin or play Penn State in the Big Ten Championship.
I was just starting to believe Clemson even had better athletes than SEC teams and they lose a game they probably shouldn't have.
Not to take anything away from Georgia Tech, but Clemson can absolutely fly at every position. Their schedule still sets up nicely even with the showdown against South Carolina.
They still control their own destiny for the ACC Championship game and would get a shot at either Virginia Tech or Georgia Tech.
Aside from the game against Clemson, Virginia Tech has looked like the Frank Beamer teams we've always known.
The game against Georgia Tech will most likely decide who goes to the ACC Championship from the Coastal Division. They have North Carolina and Virginia to finish the season and neither should pose much of a threat.
The Hokies have been flying under the radar since the loss to Clemson, but this team could quickly enter the BCS conversation with a strong finish.
I love the Kansas State story this year, but in all honesty they're probably the biggest longshot of this list.
The Wildcats got hammered by a very motivated Oklahoma team and they still have Oklahoma State, Texas and Texas A&M to finish the season. That would be a difficult slate for even the likes of LSU or Alabama to deal with.
I know they only have one loss now, but the Wildcats should be thrilled with a split of the last four games of their schedule.
The Big Ten has suddenly given us many contenders and Michigan is one that many didn't expect to see at the table.
The Wolverines have played well and only have one loss to a very strong and large Michigan State team.
They can run with anybody on offense. That was illustrated by the comeback against Notre Dame. Their problem is that they can't stop everybody. Notre Dame ran up and down them and Michigan State pushed them around.
Nebraska poses the biggest threat, but don't overlook Ohio State. The Buckeyes have had a rough season and would like nothing better than to ruin their season.
Penn State, much like Michigan, wasn't expected to be here at this point.
The Nittany Lions are the polar opposite of the Wolverines. They have gotten here on the back of a very strong defense and sometimes (see this past weekend's 10-7 victory against Illinois) struggle to score any points.
The Lions still haven't settled on a quarterback, but have Saturday off to prepare for their showdown against the Huskers.
Penn State controls their own destiny in the Leaders Division and could force themselves in the BCS talk by running the table.


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Floor Cleaner Market: Summary
The Global Floor Cleaner Market is projected to reach a value of USD 16251.23 million by 2027 at a CAGR of 4.9%.
The floor cleaner is being used to remove allergens as well as to make the environment sanitary. Its also help to reduce the amount of accumulated dirt and to keep stains from setting into the floor, which will lead to result health environment. Global floor cleaner market is growing at a rapid pace with the growing demand for herbal and non-toxic surface cleaners. However, the limited penetration in developing countries are expected to hamper the growth of the market.
Floor Cleaner Market: Market Dynamics
Growing Demand for Herbal and Non-Toxic Surface Cleaners
- There has been growing demand for herbal and non-toxic surface cleaners is play a significant role for the floor cleaner market. This will lead to cleans stains, kills germs as well as acts as an insect repellent.
- There herbal and non-toxic cleaner’s helps to keep the air clean, as per the U.S. Environmental Protection Agency, air quality inside the many homes is excepted to be two to five times more polluted than the air just outside our homes.
- The ITC Limited, Nimyle herbal is a 100% natural action floor cleaner which has the power of neem. It is also has a chlorine free formulation as well as 100% natural action to get germ free floors for children & pets protecting.
- For example, the organica thinksafe natural floor cleaner liquid is an eco-friendly, baby safe, pet friendly and citrus fresh. This is also excepted to provide about 99.9% protection from germs.
- Supportive Government Policies
- Limited Penetration in Developing Countries
Floor Cleaner Market: Key Segments
- The Floor Cleaner Market is majorly segments into type, and form.
- On the basis of type, the market is bifurcated into ceramic floor cleaners, marble floor cleaners, wooden floor cleaners and multisurface floor cleaners. The ceramic floor cleaners is projected to get the largest share. It is has been widely used for roofs, floors, as well as for the tabletops.
- On the basis of form, the market is sub segmented into liquid form and powder form. The liquid form is excepted to get higher market share. It has been majorly prefer by the various consumer throughout the globe as it is easily dissolve in water.
Floor Cleaner Market: Key Regions
On the basis of region, the market is divided into North America, Europe, Asia-Pacific, Latin America, and Middle East & Africa. North America accounted for the major market share, as the region is heavy investments in infrastructural development. However, Asia Pacific is projected grow at the fastest growth rate with the growing construction activities in the residential sector which is projected to increasing population has boost the real estate market in this region.
Floor Cleaner Market: Report Scope
The report on the floor cleaner market covers a deep dive analysis of historic, recent and current market trends. Furthermore, market share/ranking analysis of key players, market dynamics, competition landscape, country-wise analysis for each region covered and the entire supply chain dynamics are covered through the below segmentation.
Historical/Estimated/Forecasted Market Size Years
2020-2027 (2020 estimated year, forecast up to 2027)
Value (USD Million)
Type and Form
North America, Asia-Pacific, Europe, Middle East & Africa, South America
Key Companies Covered
- ACCO Brands Corporation (Company Overview, Financial Overview, Product Synopsis, Key Developments, SWOT Analysis, & Key Strategy)
- Church & Dwight Co., Inc.
- Colgate-Palmolive Company
- Henkel AG & Co KGaA
- Kao Corporation
- Procter & Gamble Co
- S. C. Johnson & Son, Inc.
- Zep, Inc
- Other Key Companies
Floor Cleaner Market: Report Segmentation
For the scope of the report, In-depth segmentation is offered by Forencis Research
Floor Cleaner Market, by Type
- Ceramic Floor Cleaners
- Marble Floor Cleaners
- Wooden Floor Cleaners
- Multisurface Floor Cleaners
Floor Cleaner Market, by Form
Floor Cleaner Market, by Region
- North America
- South Korea
- Rest of Asia-Pacific
- Rest of Europe
- Middle East & Africa
- Saudi Arabia
- Rest of Middle East & Africa
- South America
- Rest of South America
Key Questions Answered By The Report
- What are the prominent developments affecting the market worldwide and its growth?
- What will be the impact of the developments in the market on the industry and on the market players in the future?
- What are the evolving variations of the market globally?
- What are the evolving functions of the market globally?
- What are the prominent aspects that will affect market growth globally during the study period?
- Who are the key players operating in the market across the globe?
- How are the key players leveraging in the present market scenario worldwide?
- Floor Cleaner Market Providers
- Floor Cleaner Market Solution and Services Suppliers, Distributors and Dealers
- End-Users of Floor Cleaner Market
- Government Bodies and Intellectual Institutions
- Associations and Industrial Organizations
- Research & Consulting Service Providers
- Research & Development Organizations
- Investment Banks & Private Equity Firms
60+ MARKET TABLES AND 30+ FIGURES WILL BE INCLUDED IN THE FLOOR CLEANER MARKET STUDY

domain: economics


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text:
We started the Labor Day Weekend with a distance race ahead of the Stingray Point Regatta at Fishing Bay Yacht Club. The boat I was to be sailing on wasn’t here on Friday so I jumped on a J120 called Bogey from Hampton Yacht Club with Steve, Daryl, Jim, Vicky, Amy, Tom, Rob, Mitchel and other Tom. This was my first time on a J120 which is almost like a 40′ version of a J105 and also has an asymmetrical spinnaker. My position was at the mast working with Tom on the bow and also providing some local knowledge as one of the only few who had sailed in this area before.
The wind was out of the NNE at 14 to 16 and we saw some periods of 8 while we were out there and some shiftiness. The race started in the mouth of the Piankatank River and we sailed about 4 miles to the south entrance of the Rappahannock River.
We had a great start right on the boat and we could just about lay the mark from there so we only spent a short time on starboard before tacking over for the long leg out. On the way out we crossed a current line where we could clearly see where the water from the Rappahannock met the water from the rest of the bay. We arrived at the mark just behind Nanuq with Excitation and Voodoo 2 just behind us.
The next leg took us straight upwind to another entrance mark of the Rappahannock River. To our surprise the cruising class was also using this mark as a leg of their course, only they were rounding in the opposite direction. We arrived at the mark at the exact same time as Running Tide and we were on Starboard so they made a wide rounding to avoid us. I can’t say I’ve ever been in that situation in a sailing race before and I’m not 100% sure how all of the rules might apply.
From there we went back to the mark we had just came from and then did another lap around both of them. We got to some spinnaker sailing and made up some ground on Voodoo on the first downwind, but ceded it upwind by being on the wrong side of the course.
The last two legs were reaches back towards Stove Point. Nanuq was first over the line and well ahead followed by Voodoo 2 just ahead of us. Excitation and Osprey were a bit behind us. We would go on to finish on corrected time 4th behind Osprey, Excitation and Voodoo 2 with Nanuq behind us.
I had a lot of fun and it was a great day of racing. Thanks Steve and the rest of the crew on Bogey for having me aboard!

domain: hobby


text:
While at EPCOT yesterday, I took the monorail to get a look at the construction going on, including at Journey of Water, Inspired by Moana (scheduled to open in later 2023).
First, here is a view of the ongoing work next to Guest Relations.
In World Celebration, CommuniCore Hall has changed a lot since my last time on the monorail in December.
A first glimpse of Moana, Journey of Water can be seen.
There was a lot going on across the site.
The flower quilt for the upcoming EPCOT International Flower & Garden Festival (March 1st, 2023) was planted a few days ago.
CommuniCore Hall can be seen beyond it.
We pass by the World Nature restrooms.
And then back around toward Moana, Journey of Water.
The area is looking lush. Te Fiti can be seen in the background.
I expect to take more photos next week and will post then. With the EPCOT International Flower & Garden Festival taking shape, I will be spending even more time at EPCOT in the coming weeks.

domain: geography


text:
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Welcome to USPS. Find information on our most convenient and affordable shipping and mailing services. Use our quick tools to find locations, calculate prices, look up a Zip Code, and get Track amp; Confirm info.
2018 9 6ensp;0183;ensp;At the top left, the capital city of Sudan, Khartoum, is located at the convergence of the Blue Nile and the White Nile. Although the Blue Nile is much shorter than the White Nile, it contributes about 80% of the flow of the river.
2001 7 13ensp;0183;ensp;Beijing 2008 Olympic Games Mount Olympus Meets the Middle Kingdom The Games of the XXIX Olympiad, involving some 200 Olympic committees and as many as 13,000 accredited athletes competing in 28 different sports, were auspiciously scheduled to begin at 808 pm on the eighth day of the eighth month of 2008 in
2013 4 21ensp;0183;ensp;African Archaeology section on the latest news about archaeological discoveries in Africa
Join the Nasdaq Community today and get free, instant access to portfolios, stock ratings, real time alerts, and more
2018 8 15ensp;0183;ensp;About our postgraduate scholarship program. Developing the next generation of world class scientists and engineers is critical to building Australia's future science, technology, engineering and mathematics (STEM) and innovation capable workforce, and CSIRO is contributing to this by increasing our engagement in education, training and
Background A large multinational mining corporation with operations spanning Africa, Asia, and Australia. The client received a large block for gold exploration in Sudan, which was highly inaccessible.
2018 9 6ensp;0183;ensp;This detailed astronaut photograph highlights sand dunes in the Fachi Bilma erg (sand sea) in the central eastern part of the Tenere Desert. The Tenere occupies much of southeastern Niger and is considered to be part of the larger Sahara Desert, which stretches across northern Africa.
2018 8 22ensp;0183;ensp;one thought on joy behar on the view admits to touching robin williams inappropriately
2018 9 8ensp;0183;ensp;Location of South Sudan (dark blue) in Africa (light blue amp; dark grey) in the African Union (light blue)
Get the latest science news and technology news, read tech reviews and more at ABC News.
2018 9 7ensp;0183;ensp;Gold is a chemical element with symbol Au (from Latin aurum) and atomic number 79, making it one of the higher atomic number elements that occur naturally. In its purest form, it is a bright, slightly reddish yellow, dense, soft, malleable, and ductile metal.Chemically, gold is a transition metal and a group 11 element.It is one of the
2018 3 18ensp;0183;ensp;A desert is a landscape or region that receives very little precipitation. Deserts can be defined as areas that receive an average annual precipitation of less than 250 mm (10 in), or as areas in which more water is lost than falls as precipitation.
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The Xbox One X release will have to deal with limited supply
Like any major console launch, stock is going to be an issue when it comes to supplying everyone.
And it looks like things will not be any different for Microsoft Xbox One X release.
Reports today are claiming that retailers are getting a much more limited supply than they have requested, meaning things could get a little tight.
Trade Magazine MCV UK has spoken to several retailers over the situation, stating: “Speaking with four other retailers, both indies and major chains, there was a general feeling that stock was in short supply when compared to the Xbox One's launch.”
And speaking to Exertis, the current distributor, it was revealed that allocation is indeed limited ahead of the console’s November release date.
"We are seeing very high demand for Xbox One X and while we are doing our best to keep up with that demand, unfortunately it is the case that, as with any new console launch, there is a limited amount of stock available,” Exertis told MCV UK.
“This is not specific to independent retailers and we are finding that every retailer would wish to have more Xbox One X stock for launch, which is reflective of the strong pre-order performance we are seeing.
“What we are doing is working very hard to keep supply flowing in the weeks immediately following launch, and we are hopeful that additional stock will be available in the subsequent weeks."
The Xbox One X is already known to be one of Microsoft's most successful console pre-order campaigns, although no exact figures have been shared.
The upgraded Microsoft console will be launching next month, with a release date of November 7 and a UK price of £449.99.
Microsoft says the Xbox One X is the world’s most powerful console, boasting six teraflops of power and native 4K gaming.
It’s launching more than a year after the PS4 Pro was released - which also offers 4K gaming and HDR visuals.
And one analyst is predicting a close battle for living room supremacy between the two powerful consoles.
Lewis Ward, IDC Research Director for Gaming and VR/AR, spoke to GamesTM about the Xbox One X.
Ward Predicted: “No, the XB1X won’t be enough for Microsoft to catch up. It should give PlayStation 4 Pro a run for its money in 2018 in the United States and the United Kingdom, however.
“I’m projecting about four million XB1X consoles shipped from Q4 2017 through the end of 2018, and about five million PlayStation 4 Pro consoles shipped in the same timeframe.
“The way shipments break down globally, however, that implies a very tight race in the United States and the United Kingdom.”
While Ward is still predicting the PS4 Pro to outsell the Xbox One X, it’s a much closer battle the fight between the PS4 and Xbox One overall.


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Seville - An Andalusian Gem
horas Duración estimada
Discover the most iconic landmarks of the city of Seville, a compact and relaxing city with a rich cultural heritage and the capital of Andalusia. Drive from the port of Cadiz to Seville along the highway through vineyards, cotton fields and olive groves. Enjoy the panoramic drive through some of its most iconic sites such as Plaza de España and Maria Luisa Park. Visit Casa Pilatos, the permanent residence of the Dukes of Medinaceli family and one of the city’s most glorious mansions. Originally dating to the late 15th century, it incorporates a wonderful mixture of Mudéjar, Gothic and Renaissance decor, with some beautiful tilework.
The guided visit will be focused on the ground floor premises, considered one of the finest examples of Andalusian architecthre of the 16th century in Seville. The Cathedral of Seville, built in the 15th century and considered the third largest in the world will impress you as well as La Giralda, an imposing 322-foot (98 meter) tower, formerly a Moorish-style minaret from a mosque that once stood on this site. Take a stroll through Barrio de Santa Cruz, the old Jewish Quarter and a labyrinth of narrow streets with wrought iron-grilled windows, white-washed buildings and tiled patios. Afterwards, you will enjoy lunch at a local establishment.
Need to Know:
Note: Tour involves extensive walking on mostly flat surfaces, cobblestones and a few steps.
CARACTERÍSTICAS DE LA ACTIVIDAD
Tipo de excursión:
Tours por la ciudad y panorámicos
Nivel de actividad:
Tours with this activity level involve a considerable amount of physical activity such as substantial walking over cobblestone streets, uneven or steep terrain, climbing stairs, or extended periods of standing. Not recommended for guests with physical limitations. Comfortable, sturdy shoes are recommended.
¿Tienes alguna pregunta sobre tu excursión en tierra?
Estamos aquí para responderte. Llámanos al número de abajo para hablar con un especialista de excursiones en tierra.


On the Pirates: Rival general managers play mind games
August 4, 2013 8:00 AM
Andrew McCutchen makes a diving catch earlier in Game 1 that turned into a double play.
The Pirates won four of five games from the Cardinals this past week in what was dubbed the biggest series in PNC Park history (so far). It produced its share of moments. A.J. Burnett, top, hits Alex Presley, the hero of Game 1 in a doubleheader sweep Tuesday, with a celebratory cream pie.
Russell Martin drives in the go-ahead run in a 5-4 comeback win Wednesday.
By Bill Brink Pittsburgh Post-Gazette
Gamesmanship pervades the trade deadline. Teams inquire about the availability of dozens of players. Potential returns become exaggerated to entice other teams to join the fray. Media reports fuel the fire: General managers themselves sometimes follow up on Internet rumors.
This year, opposing GMs applied a new tactic to pry the top members of the Pirates' strong minor league system away from the first-place team.
"There were some general managers that thought they could remind us that we'd lost 20 years in a row," said manager Clint Hurdle, who was involved in or kept abreast of all the Pirates' trade discussions. "That we might have overlooked that. 'You might really want to rethink how much you want to spend on a certain player, certain bats.' "
The Pirates, like many teams, held fast at the trade deadline after finding the expected returns too high for the offensive players they sought. They tried, according to Hurdle and general manager Neal Huntington, but even their aggressive efforts weren't enough.
"When we started digging into this thing and some of the names that were coming up that got my attention, I was like, Wow, attaboy," Hurdle said. "[Huntington] definitely opened up our farm system."
Other teams, however, tried to take advantage of the fact that the Pirates' recent performance guaranteed they would enter the weekend series against the Colorado Rockies in first place in the NL Central.
"There were some comments made about how important it was for us to do something properly because of the position we're in," Hurdle said.
The scarcity of talent available at this season's trade deadline encouraged teams to use every available means to receive a high return. Starting pitchers -- Matt Garza, Bud Norris, Ian Kennedy, Jake Peavy -- represented the major moves at the deadline, in addition to the Chicago Cubs finally trading Alfonso Soriano. Not many hitters of impact changed teams.
"We're not alone sitting here today with people asking why you didn't do anything," Huntington said after the deadline passed Wednesday. "We feel good about this group and we're ready to move forward."
A step in the right direction
In 2007, when Clint Barmes, pictured below, was traveling back and forth between Class AAA and the major leagues, he experimented with lifting his left leg at the beginning of his swing. He only received 39 major league plate appearances that year. In 477 plate appearances with Class AAA Colorado Springs, though, he hit .299 with 11 home runs, a .364 on-base percentage and a .451 slugging percentage.
In the ensuing years, Barmes said, he returned to a swing in which he took no stride to start it.
In 2012, Barmes posted the lowest on-base plus slugging percentage over a full season in his major league career. His OPS is lower this season. One day, frustration took over.
"One of those days, in [batting practice], I just kind of said, 'You know what, I'm going to take the slow-pitch softball hack at it and see what happens,' " Barmes said.
Barmes lifted his leg high, came down and hit a line drive with backspin.
"Wow," he said. "That's what that feels like."
Barmes carried it over into games and has liked it. In July, Barmes hit .296 with a .367 on-base percentage and a .407 slugging percentage.
"It keeps me on my back side," Barmes said. "Spread out. I wasn't getting back and staying back long enough to really transfer any power and any weight forward. A lot of times, I was really gone by the time I wanted to start my swing. The timing of trying to keep that, obviously, didn't work out very well. I was rolling over balls and I just couldn't stay back."
After giving way to Jordy Mercer at shortstop earlier in the summer, Barmes started 10 of the Pirates' first 14 games following the All-Star break. Mercer had a .434 OPS from June 23 to the All-Star break, but had a .382 in the first nine games after the break.
Cardinals not going away
For almost four months, the St. Louis Cardinals ruled the NL Central. At the end of July, the Pirates mounted an assault on the Cardinals' entrenched position atop the standings and started August in first place.
"There's no panic, but at the same time we are urgent to go out and win games," Cardinals starter Adam Wainwright said during the series this past week in which the Cardinals lost four of five games to the Pirates. "We are not taking this lightly."
The Cardinals lost seven games in a row, the first time they had done so during manager Mike Matheny's tenure. They lead the NL in runs per game, but scored just seven in the first four games in Pittsburgh before breaking out with 13 runs in the fifth.
"Still making some hard outs, but it's the big hit," Matheny said after the Cardinals' loss to the Pirates Wednesday. "That's what usually differentiates a game like this."
Wainwright understood that the length of the season lent itself to winning and losing streaks.
"We have a veteran clubhouse," he said. "We've been through those rough patches. We've been deep in the postseason.
"We know how to come out of stretches like we're in right now. We're just going to keep going out there and grinding. Eventually things will go your way if you have the right mind-set about things."
Losing catcher Yadier Molina to the disabled list because of a sprained right knee will not help the Cardinals' cause. But Wainwright fronts a strong rotation featuring Shelby Miller and Lance Lynn, and the Cardinals lineup, featuring Matt Holliday, Carlos Beltran, Matt Carpenter and Allen Craig, remains one of the strongest in the NL.
"I'm glad it's now and not in September, but you'd rather not have it ever obviously," Wainwright said. "It's a rarity for a team like we have to go through a seven-game stretch like we've gone through."
The week ahead
Schedule for the NL Central contenders (Monday-Sunday)
Tues-Thu: vs. Marlins: Pirates lost 2 of 3 in Miami July 26-28.
Fri-Sun: @ Rockies: Begins stretch of 13 of 16 on the road.
Mon-Thu: vs. Dodgers: L.A. is 35-17 since Yasiel Puig called up.
Fri-Sun: vs. Cubs: Chicago 14-13 in July -- first winning month of '13.
Tue-Wed: vs. Athletics: A's entered Friday 31/2 games up in AL West.
Fri-Sun: vs. Padres: Not good, but better than Giants in NL West.


text:
“He who asks is a fool for five minutes, but he who does not ask remains a fool forever.” ~ Chinese Proverb
“Knowing trees, I understand the meaning of patience. Knowing grass, I can appreciate persistence.” ~ Hal Borland
“He who is in love is wise and is becoming wiser, sees newly every time he looks at the object beloved, drawing from it with his eyes and his mind those virtues which it possesses.” ~ Ralph Waldo Emerson
“I’ve learned that no matter what happens, or how bad it seems today, life does go on, and it will be better tomorrow. I’ve learned that you can tell a lot about a person by the way he/she handles these three things: a rainy day, lost luggage, and tangled Christmas tree lights. I’ve learned that regardless of your relationship with your parents, you’ll miss them when they’re gone from your life. I’ve learned that making a “living” is not the same thing as making a “life.” I’ve learned that life sometimes gives you a second chance. I’ve learned that you shouldn’t go through life with a catcher’s mitt on both hands; you need to be able to throw something back. I’ve learned that whenever I decide something with an open heart, I usually make the right decision. I’ve learned that even when I have pains, I don’t have to be one. I’ve learned that every day you should reach out and touch someone. People love a warm hug, or just a friendly pat on the back. I’ve learned that I still have a lot to learn. I’ve learned that people will forget what you said, people will forget what you did, but people will never forget how you made them feel.” ~ Maya Angelou
1) Nothing ventured, nothing gained
2) Challenge, uncertainty, excitement
3) Fear makes you focus
4) Passion gives you confidence
5) Fun makes for a great team
6) Make sure you have more than one thing to live for
7) Resist the flock factor
8) You are all you have
9) Great challenges result in powerful experiences
10) A view from the summit – to new horizons
“Be true to yourself.
Make each day your masterpiece.
Drink deeply from good books.
Make friendship a fine art.
Build a shelter against a rainy day.
Pray for guidance and give thanks for your blessings every day.”
~ John Wooden
…And some simple, yet profound ways to keep the little things from taking over your life.
“Whenever you expect something to be a certain way and it isn’t, you’re upset and you suffer. On the other hand, when you let go of expectations, when you accept life as it is, you’re free.” ~ Richard Carlson, Don’t Sweat the Small Stuff

domain: psychology


text:
Water vapor is the most important contributor to the natural greenhouse effect. The amount of water vapor in the atmosphere is expected to increase under conditions of greenhouse-gas induced warming, leading to a significant feedback on anthropogenic climate change. The authors of a recent publication in the journal Nature, sponsored in part by BER, identify and explore the causes of changes in surface specific humidity over the late twentieth century using a new observational data set of surface humidity with output from a coupled climate model. They identify a significant global-scale increase in surface specific humidity attributable mainly to human influence. The changes in atmospheric humidity may have important implications in determining the geographical distribution and maximum intensity of precipitation, the potential maximum intensity of tropical cyclones, human heat stress, and surface hydrology.
Reference: Willett K.M., N.P. Gillett, P.D. Jones, and P.W. Thorne. 2007. "Attribution of observed surface humidity changes to human influence," Nature 449, 710.
Contact: Anjuli Bamzai, SC-23.3, (301) 903-0294
SC-33.1 Earth and Environmental Sciences Division, BER
(formerly SC-23.3 Climate Change Research Division, OBER)
BER supports basic research and scientific user facilities to advance DOE missions in energy and environment. More about BER
Mar 23, 2021
Molecular Connections from Plants to Fungi to Ants
Lipids transfer energy and serve as an inter-kingdom communication tool in leaf-cutter ants&rsqu [more...]
Mar 19, 2021
Microbes Use Ancient Metabolism to Cycle Phosphorus
Microbial cycling of phosphorus through reduction-oxidation reactions is older and more widespre [more...]
Feb 22, 2021
Warming Soil Means Stronger Microbe Networks
Soil warming leads to more complex, larger, and more connected networks of microbes in those soi [more...]
Jan 27, 2021
Labeling the Thale Cress Metabolites
New data pipeline identifies metabolites following heavy isotope labeling.
Aug 31, 2020
Novel Bacterial Clade Reveals Origin of Form I Rubisco
List all highlights (possible long download time)

domain: biology


text:
One can think of it as seeding for the future. Singapore’s first seed bank has opened at the Botanic Gardens and has the capacity to store the seeds of up to 25,000 plant species. The seeds can be used for habitat restoration and species conservation projects here and in the region. More than $1.17 million has been raised so far for research and development, and the Gardens plans to raise more than $5 million over the next 10 years to fund research, development and education efforts. The seed bank is both a nod to the history of the Gardens and a resource for the future. Singapore has a long history as a centre for botanical research.
Sir Stamford Raffles set up a 19ha garden at Fort Canning where cash crops such as nutmeg, cloves and cocoa were first cultivated. More famously, the Gardens’ first scientific director, Sir Henry Nicholas Ridley, perfected a rubber tapping technique that yielded maximum quantities of latex without destroying the trees, and spearheaded research which turned the Gardens into a major seed supplier for the industry. In today’s world, where climate change threatens to devastate both agriculture and nature, it is apt that the Gardens has reasserted its purpose as a core resource in conservation and cultivation. The Svalbard International Seed Vault, nicknamed the Doomsday Vault, is perhaps the world’s best-known seed bank, where a vast library of seeds are stored in conditions meant to survive bombings and earthquakes. Beyond mere preservation, seed banks help encourage crop diversity.
TO READ THE FULL ARTICLE: https://www.straitstimes.com/opinion/st-editorial/banking-to-seed-future-growth
# MANILA STANDARD
> Bells toll
# MANILA TIMES
>The West Philippine Sea
> Death-penalty enthusiasts
> Certainty of punishment
PILIPINO STAR NGAYON
> Solusyunan ang nararanasang kagutuman
All photographs, news, editorials, opinions, information, data, others have been taken from the Internet ..aseanews.net | [email protected]

domain: agronomy


From The Tribune staff reports
BIRMINGHAM — The Jefferson County Coroner’s Office has identified a 75-year-old man initially injured in a multi-vehicle wreck on Friday, February 4, 2022.
The accident happened at the intersection of Oporto Madrid and First Ave North, according to the coroner’s report.
“The decedent was an occupant of a motor vehicle involved in a multi-vehicle wreck,” Deputy Coroner Bill Yates stated. “The decedent was transported by paramedics to UAB Hospital, where he succumbed to his injuries three days later. The Birmingham Police Department is investigating the circumstances surrounding the wreck.”


text:
Photo by ISIPhotos.com
By DAN KARELL
Nearly two months after walking away from the game of soccer, Robbie Rogers has shared his story about leaving soccer and coming out as a gay man for the first time on television.
The former USMNT midfielder spoke on Monday to ABC’s Nightline program and CNN’s Anderson Cooper 360, detailing his fears about how he would be perceived by his peers in the locker room, and the public before his blog post in February when he announced his retirement.
“It’s hard to describe the feeling, but you go to work having the feeling that ‘I hope no one figures out I’m gay’,” Rogers said to Nightline.
You can watch clips of his interviews after the jump:

domain: celebrity


A non-flexible scope made of polished stainless steel and that utilizes an eye piece instead of a monitor.
Typical diameters range from 2.7mm up to 10mm and lengths of 175mm up to 305mm on average. These systems are ideal for the manufacturing industry, or companies that do the same inspection repetitively where they can mount the scope to a fixed position. Companies which specialize in metal casting and cylinder bore inspections are a good example of this.
The enclosed design of this system makes it water, dust, oil and diesel proof. Rigid Borescopes are known for their unmatched high image quality which is due to direct glass rod lenses rather than fiberoptic or camera sensor technology. All Rigid Borescopes are designed with light fiber bundles that transmit light to the end of the probe. These systems would still require an external light source. Taking pictures and/or videos is still possible by connecting a USB Camera and adaptor to the eye piece of the borescope along with a high intensity LED light source. These systems are designed for direct view or side viewing angles.
If an inspection requires elaborate angles or 90° bends then the user would find more success by using a flexible style borescope.


text:
Kinder Morgan began their TransMountain pipeline expansion last fall—without permits. The company quickly demonstrated their lack of care for the environment, when they illegally laid down plastic snow fencing in salmon streams to prevent spawning, in anticipation of work being done early this year. They received a letter from the National Energy Board which stated “The Board reminds Trans Mountain that construction of the pipeline shall not be undertaken until such time that Trans Mountain receives the applicable approvals…”
When I heard the call-out for Break Free 2016!—a global day of action against fossil fuels—I knew we had to go. The plan was to surround Kinder Morgan’s Westridge Marine Terminal in Burnaby, with marchers on land and ‘kayaktivists’ on the water.
Kayaking is the whole reason I am an activist—a 1990 circumnavigation of Vancouver Island shocked me into realizing how little old-growth forest was left, and that Clayoquot Sound is the Last Great Rainforest on Vancouver Island. This led to my involvement in organizing the Clayoquot Summer 1993 blockades—and the rest, as they say, is history.


Where possible, we will try to troubleshoot your issue and resolve it without the need for a returns process. Physical size and business note writer ultra slim Warranty and customer service: Gaming keyboards, the exception, will typically work as soon as you plug them in, but come with their own software that lets you adjust features such as onboard RGB lighting and the assignment of macro keys.
It uses fluctuating high-frequency sound waves to keep your home free of annoying and gross pests. Choose a mode and dpi level according to what kind of game, mood and occasion, get the Supreme Experience!
With a durable and tough twisted filament polyester exterior, water resistant, tear-proof. Both have their pros and cons. Should a return be deemed appropriate, we will provide a return authorisation RA.
Yes, smartphones are handy, but to complete real work, a laptop is often the best tool. The precise scissor key design built into each key requires minimal travel distance and provides a quiet, but solid click for tactile feel as you type.
They usually require a lot more vertical space than silicone dome switches, which means the keyboards tend to be thicker. Brushed texture shell brings you perfect touch and sight enjoyment. Sources Why you should trust us Wirecutter has researched and recommended hard drives since earlyand our PC team has over eight years of combined experience testing hard drives and solid-state drives.
Put Your Finger on the Right-Fit Keyboard Maybe your trusty old keyboard has typed its last letter or absorbed its last bagel crumb. With its lightweight design and long lasting rechargeable battery, the KeySlate is ideal for the business traveler operating on-the-go.
Rugged portable drives are bulkier and more expensive than the portable drives we recommend for most people. Stable and secure charging. Each "color" has a slightly different click feel, acoustic aspect, and pressure characteristic; see our roundup of the best mechanical keyboards for a lot more detail on that.
Bonus points if the keyboard maker implements the volume control as a dial or roller, like on some high-end Corsair and Das Keyboard models.
Small and quiet, each pest control ultrasonic repeller covers up to sq. Smaller distinctions include the placement of the directional-arrow, Page Up and Down, and Home and End keys.
But all hard drives die. Try different places and directions to find out the optimal location to pick up signals. Note that in some situations, these USB dongles can provide connectivity to more than one device, meaning you can use the single adapter for your wireless keyboard as well as a wireless mouse, assuming that both are the same brand and both support the same scheme.
These switches can also register each keystroke with a much shorter amount of travel, making them ideal for touch typists. Shopping for a great laptop requires that you understand some computing jargon. Cherry switches are still popular, but nowadays you'll also see homebrew mechanical switches from leading keyboard makers such as Logitech and Razer, as well as "imitation Cherry" switches, using the same color nomenclature, from makers such as Kaihua.
The Basics One aspect of keyboard design that you'll see mentioned in reviews—but about which most people don't give a second thought—is the type of switches used for individual keys.
We will take between 1 to 2 weeks after receipt of the faulty unit to process your warranty claim. Before you go shopping, though, it pays to know what makes a keyboard a good fit. Received a day unconditional return.
New innovations, including hand-proximity sensors, improve the battery life versus older Bluetooth devices, which maintained an always-on link, draining battery quickly. Our goods come with guarantees that cannot be excluded under the Australian Consumer Law. While the majority of portable hard drives we tested have two-year warranties, a couple have three-year warranties.
Perfect service and quality assurance: But these gray, plastic laptops are designed to be more affordable, while combining the best of new technologies with older gear, such as optical drives.
If any of the above item s is not included, we reserve the right to reject your DOA claim. Have high quality products. A portable hard drive is a great local backup for a computer you take from your house to the coffee shop, on business trips, or on vacations.
Buy a Google Pixelbook on Amazon Laptops with Optical Drives Years ago, every laptop came with an optical drive, which was crucial for installing software.Business (1) Card readers & adapters might be on your school's checklist, SuperSpeed memory card reader/writer transfers data between your tablet, laptop, Ultrabook, Chromebook or computer.
Blu-Ray Writer Business Laptops Gaming Laptops Please Note: cosmetic damage to the screen, few light scratches. packaging has been damaged. The Gemini NC14 (Pn:NC14V) at a glance: Gemini NC14 Ultra Slim Aluminium Laptop.
14" FHD Screen. 4GB RAM. GB SSD. Was £ Save £ ASUS VivoBook X Product Description. ASUS ZenDrive U7M is an external DVD Writer featuring an ultra-slim 13mm form factor and a Zen-inspired design with a concentric-circle hairline finish that adds sophisticated aesthetics to any computer agronumericus.coms: Samsung Slim DVD Writer - Glossy Black USB External CD DVD Drive Ultra Slim CD DVD Burner with Touch button Portable DVD Player Reader Writer for PC Laptop Desktop MacBook Windows XP/Win 7/Win 8/Win 10/Vista/Linux/Mac OS and More points to note: DVD write speed only 8x - (16x would be better but this seems 4/5.
A sales representative will email you back next business day. If you would like a call back from a sales representative, please include your telephone number and the best time to call. We will answer all questions pertaining to availabilities, special pricing, price beats, volume discounts, and rush shipping.
Dansrue Latest USB External CD DVD Optical Drive, Ultra Slim Portable CD DVD Player Rewriter Writer Burner For Laptop/Desktops with Mac 10 OS Windows/ Vista/7//10 Linux System External CD Drive, BOSLISA Touch Control USB CD/DVD+/-RW Burner Player, Optical Superdrive High Speed Data Transfer for .Download


text:
Description of an Amendment in the Nature of a Substitute to the Provisions of H.R. 4691, a Bill To Modify the Tax Rate for Excise Tax on Investment Income of Private Foundations [electronic resource].
- Corporate Author:
- United States. Congress. Joint Committee on Taxation
- Bethesda, Md. : ProQuest, 2014.
- Physical Description:
- 1 page, digital, PDF file
- Record is based on bibliographic data in ProQuest U.S. Congressional Research Digital Collection. Reuse except for individual research requires license from ProQuest, LLC.
- Reproduction Note:
- Electronic reproduction. [Bethesda, Md.] : ProQuest, 2014. digital, PDF file. ProQuest Congressional Research Digital Collection. Mode of access: World Wide Web via ProQuest website.
- Technical Details:
- System requirements: PDF reader software.
View MARC record | catkey: 19195746

domain: library


text:
Hi everyone. I'm back today with my card for the new Divas by Design Challenge. This week it's a nice easy one. The theme is 'Flowers'.
We're sponsored this week by Lili of the Valley.
Have you seen they've just released some more super cute stamps as well as sketch pads. I'm liking the look of those cute vintage boys. Just great for male cards!
I've used LOTV Cake Glorious Cake for my card. I'm ashamed to say that this is the first time she has seen ink. The sentiment is 'Sentiments Recipe' also LOTV.
The yummy papers are Pion Design 'Blue Wings' and the edges have been distressed with Walnut Stain.
She's been coloured with Copics.
Skin: E000, E00, E21, E11, R11
Hair: E21, E13, E15, E18
Top: Y00, Y11, Y13, Y15 YR12
Skirt: B91, B93, B95, B97
Wings: Y11, Y000, B91, B000 & Glamour dust
Cake: E50, E51, E53, E55, E57
The card shape was made using Go Kreate Large frames Frilly #2 and the image was cut using Nestabilities Floral Ovals. To embellish the card I've added mulberry flowers, DooHickey heart swirl and swirls dies along with some little EK Success butterflies.
I would like to enter my card into the following challenges:
Bearly Mine Designs - Anything Goes
Papertake Weekly - Bingo (glitter, dies & blooms)
Through the Craftroom Door - Anything Goes
Artistic Inspirations - Anything Goes
Lexi's Creations - Use Punches and/or Dies
There She Goes Clear Stamps - Yellow, Blue and Neutral
2 Sisters Challenge - Anything Goes
Thanks for dropping by.
I love this Janine. The design is gorgeous - love the Go Kreate dies. Love your new look blog by the way.
Hugs Sharon. x
Gorgeous card Janine. The image is so sweet and I love the design and colour scheme.
Totally super gorgeous card lurve the shape & the image. Love the blog. Thank you so much for following me sweetie.
Lotsa hugs Vicky xxx
Absolutely gorgeous Janine, adorable image.
This is stunning!! Love the others I've peeked at as well. The wings and glitter are soooo sweet! Hugs!
Love your gorgeous card Janine.
Good Morning sweetie this is just gorgeous,I love your creation, I got some big shaped dies as I loved the look of you sc suitcase one, I cannot work out how it would open, if you use them I tell you I am so thick hugs Pops xxx
Oh wow I love your blog design! it's fabulous.
Wonderful card, love this image and shape!
Love, Mel. xxx
What a cutie, and your colouring on the image is so fabulous! A really delicious card (and looks like a delicious cake too, as long as she doesn't drop it!).
so gorgeous janine.beautiful design and details and i love your colouring and sweet image :D
xx coops xx
Really stunning, I love the shape, the cream and blue look gorgeous together and the cute image is so beautifully coloured, really wonderful design
Beautiful! Love the blue and cream together, great coloring and layering... :)
Gorgeous! You've used my fave color combo, blue and yellow, plus the added bonus of the soft beige neutral inked. Lovely effect and an amazingly colored image. :-)
Oh what a beautiful creation! Jen xx
This is so pretty...beautifully coloured!
Wow this is just Gorgeous! Love your Stunning colouring and your Layering too! Have a great Weekend!
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domain: design


text:
The Faculty of Agriculture is a scientific knowledge institution that seeks through its various departments to influence the society and create a state of scientific and practical activity in line with what society needs to keep abreast of modern scientific developments and their application on the ground.
Preparation of scientific cadres specialized in applied agricultural sciences able to meet the requirements of the public and private sectors of qualified human cadres and conduct research and studies that contribute to improving the quality of society.
1- Preparing the cadres specialized in agricultural sciences and their field applications and providing them with the scientific and practical experiences required by the field of future work.
2- Raising the scientific and cultural awareness of graduate students and providing them with modern applied knowledge to serve the society and equip them with the necessary skills according to modern technological developments.
3 – Provide consultations and the establishment of seminars for extension of agricultural departments and research stations in the public and private sectors.


Welcome to my new video game club! We will help everyone who likes games to the most awesome lounge right here in the city.
Join the club of gamers in our gaming lounge! What? Yes, you read correctly.
Our gaming zone will be ready to serve you in September! Old blog: My new gaming lounge.
Welcome to our new gaming lounge. Yes you read right.
Welcome to our new gaming lounge It is an extremely useful tool for creating a new atmosphere.
We have been in game games and other video games our whole lives.
Welcome to our brand new gaming lounge. Come and join me, our new gamer friends.
www.womansclub.com Old blog: www.womansclub.
It is a great game room with a lot of possibilities.
This is a small club that we have created for you guys to get a taste of club life.
the new video game club will be up soon.Yes you read right.


text:
Kurdish Force Reports Rescuing Swedish Girl, 16, From ISIS In Mosul
Saying it had been asked to look for a teenager from Sweden who had been misled by a member of the Islamic militant group ISIS, a Kurdish security force says it recently rescued the girl, 16, from Mosul, Iraq.
Announcing the rescue Tuesday, the Kurdistan Region Security Council said that an ISIS operative in Sweden had persuaded the girl to travel to Syria and then to Mosul.
"She will be transferred to Swedish authorities to return home once necessary arrangements are put in place," the council says, according to Rudaw, an online news site based in Irbil.
Swedish news website The Local says the girl was pregnant when she initially traveled to the Middle East last June along with her boyfriend — and that by August, they were both being held by ISIS. In November, she gave birth to a son, according to multiple reports.
The Local adds that there has been confusion about which militant group in the Mosul area had been holding the girl, with some reports indicating that she had been taken away from ISIS' control during a raid on one of its camps last autumn, and other accounts saying she had returned to the group after an early attempt to rescue her.
The news site concludes:
"While much behind her journey to Syria and Iraq remains unconfirmed, news of her situation follows a rise in young people making the journey from Scandinavia to the Middle East to join radical groups."
Authorities in Sweden have not issued a statement or comment about the case.
According to the Aftonbladet news agency, the girl's boyfriend recorded a short video last spring in which he addressed the girl's family, telling them that they should forget about her, as she would not be coming back home.
Copyright 2021 NPR. To see more, visit https://www.npr.org.


text:
Kim Kardashian celebrated her late father's birthday by sharing the first photo of her three-month old son, Saint West.
Kim Kardashian celebrated her late father, Robert Kardashian’s, birthday, by sharing the first photo of her three-month old son, Saint West. The public has only seen a glimpse of the new addition to the West family when the reality star previously shared a photo of his hand wrapped around North West’s finger.
On what would have been her father’s 72nd birthday Kim wrote: “Today is my dad’s birthday. I know there’s nothing more in the world he would have wanted than to meet his grandchildren. So I wanted to share this pic of Saint with you all.”
What a cutie!
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Maestro and Caucasia stopped broadcasting to express protest2009-06-15 17:15
This afternoon Maestro and Caucasia stopped broadcasting for non-defined period - Novosti-Gruziagt-en-newsitem-inside
It is done because of the violence against journalists at today's action. According to Caucasia, cameraman Temur Pandashvili was severely beaten up, Georgi Kobakhidze suffered, police took away the camera and tapes with records of dispersal.
According to the Mamuka Glonti, the director of Maestro, two journalists of his company were taken away 2 cameras. He added that cessation of broadcasting is aimed to attract attention of the international organizations to the events in Georgia and violation of journalists' rights.
Nino Burjanadze informed police is trying to disperse a protest action near MIA in Digomi and opposition leaders are on the way to the place of incident. "The authorities attempted to create civil confrontation", she said.
Zurab Abashidze ws injured in the clash: he was taken to Gudushari hospital with concussion, broken nose, damaged jaw, with numerous hematomas, doctors explained.
In its turn, MIA reported of picket of the Ministry main building participants detained for disobedience to police.
According to the Ministry's official statement opposition representatives "blocked the entrance to the main building of the Ministry of Interior Affairs and the road". "Police tried to clear the entrance and the road, in the course of which protesters didn't obey policemen and resisted. The action participants are detained in regard to the fact of disobeying police, they will be transferred to pre-trial prison".
The number of detained is not mentioned.
Previously today about 20 people with clubs destroyed about 30 mock prison cells near the State Chancellery.


Petroleum giant BP has been publishing annual statistics on worldwide energy supply for 70 years. In the past, the Statistical Review of World Energy has already documented momentous events in the sector, from the Suez crisis of 1956 and the oil price shock at the beginning of the 1970s to the Fukushima disaster in 2011. However, the data analysts claim that none of these incidents have had ramifications as far-reaching as those of COVID-19, commenting on the figures for 2020 published in early July. The pandemic caused global primary energy consumption to drop by 4.5 percent – as much as at the end of World War II. Whereas demand for coal, oil and gas collapsed at record speed, solar and wind power posted substantial growth, based not only on the share of these renewable sources of energy in the electricity mix.
The report splits 2020 data for the energy sector into two categories. Clearly on the losing side are fossil fuels – particularly oil – which recorded consumption of 9.1 million barrels per day (BPD), representing a 9.3 percent decline to the lowest level since 2011. The steepest drops were registered by the USA (-2.3 million BPD) and the EU (-1.5 million BPD), with China reporting the only rise of a marginal 220,000 BPD. Owing to poor demand, oil saw its price plummet to the level of 2004. Production decreased by 6.6 million BPD, partially due to lockdown measures. By consequence, refinery capacity utilisation was a mere 74.1 percent – hitting its low point since 1985. Coal usage also dropped considerably in the wake of the pandemic, slipping by 4.2 percent, or 6.2 exajoules (EJ). This was mostly on account of the USA and India, which experienced declines of 2.1 and 1.1 EJ, respectively. Consumption in OECD countries recorded the lowest level since data collection started in 1965, with production shrinking by a whopping 5.2 percent.
The gas sector displayed more disparate trends. Consumption slipped by a total of 2.3 percent, and demand declined significantly above all in Russia and the USA. By contrast, it rose in other countries including China and Iran. Nevertheless, aggregate trading contracted by 5.3 percent, accompanied by a collapse in price, down to the 1995 level in the USA and the lowest figure ever recorded in Asia. Despite these developments, the share of the energy mix accounted for by gas increased to just under 25 percent. This record was achieved in part because oil usage dropped more considerably than total energy consumption. The resulting deficit was offset by gas and other sources of energy.
According to the report, the clear winners of the tumultuous year behind us are renewables: it has the portion of primary energy consumption commanded by renewable energy (including biofuel, but excluding hydropower) rising to 9.7 percent. This is slightly below the ten-year average (13.4 percent growth). Based on the 2.9 EJ in absolute energy volume, however, the figure registered was similar to those of the preceding years. In terms of generation, the analysts actually identified a gain of 12.5 percent. The main driver was the huge jump in solar power production of over 20 percent. Photovoltaics were among the frontrunners also with regard to the expansion of renewable energy. Despite the global crisis, solar systems with a total capacity of 127 gigawatts (GW were installed. This also represents more than 20 percent year-on-year growth. Wind energy capacity was up by 111 GW, or 17.5 percent. These developments culminated in 2.1 gigatonnes in CO2 savings. Carbon dioxide emissions were 6.3 percent down on the level achieved in 2019.
Trends observed in the electricity sector include the 0.9 percent year-on-year decline in generation in 2020. This is the steepest drop since when data collection started in 1985 and only the second time BP economists have measured a negative figure. Moreover, the energy mix turned greener here as well: oil, coal and gas provided much less energy, offset by gains in wind and solar power.
Besides presenting an overview of power production and consumption, the report also dedicates a chapter to select developments on the market for natural resources. After all, lithium, cobalt, graphite and rare earth metals are among the main commodities required to run renewable energy systems. And the effects of the COVID-19 pandemic were tangible here as well. Lithium mining, which had increased by an average of 16.3 percent in the preceding decade, recorded a drop of 4.6 percent, with graphite extraction declining by almost 20 percent. On the flip side, nearly three percent more cobalt and 23.3 percent more rare earth metals were mined. This was primarily due to the expansion of cobalt extraction in the Democratic Republic of Congo and the strong growth of the Australian and US markets for rare earth metals.


text:
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This vessel is being sold for parts and was built in 1975 and refurbished in 2009 including accommodations, main engines and generators.
Owners have recently received delivery of a new build and have retired this vessel and subsequently removed from class.
Tonnage: 990 Gross / 297 Net)
Lenght:170.06ft / 52.00m
Breadth : 40.05ft / 12.20m
Depth: 18.37ft / 5.60m
Max Speed: 14.5 knots
Bollard Pull: 83 tons
FUEL & WATER CAPACITIES:
Fuel: 236,168 gallons
Potable water:58,646 gallons
30 persons 12 crew,18 others
MACHINERY & EQUIPMENT:
Horsepower: 7374 BHP
Main Engines: ( 2 )Nohab Polar F2-16V
Auxiliary Engines; ( 2 ) Detroit 320 KVA ea.
Bow Thrusters: ( 1 ) 600HP
Tow Winch: 80 Ton x 2
250w SSB,VHF Radio,INMARSAT,GMDSS
||Retired Tug for parts
||Parting out vessel in US Gulf
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text:
Case Report | Open Access
Nivedita U. Jerath, Cameron D. Crockett, Steven A. Moore, Michael E. Shy, Conrad C. Weihl, Tsui-Fen Chou, Tiffany Grider, Michael A. Gonzalez, Stephan Zuchner, Andrea Swenson, "Rare Manifestation of a c.290 C>T, p.Gly97Glu VCP Mutation", Case Reports in Genetics, vol. 2015, Article ID 239167, 5 pages, 2015. https://doi.org/10.1155/2015/239167
Rare Manifestation of a c.290 C>T, p.Gly97Glu VCP Mutation
Introduction. The valosin-containing protein (VCP) regulates several distinct cellular processes. Consistent with this, VCP mutations manifest variable clinical phenotypes among and within families and are a diagnostic challenge. Methods. A 60-year-old man who played ice hockey into his 50’s was evaluated by electrodiagnostics, muscle biopsy, and molecular genetics. Results. With long-standing pes cavus and toe walking, our patient developed progressive weakness, cramps, memory loss, and paresthesias at age 52. An axonal sensorimotor neuropathy was found upon repeated testing at age 58. Neuropathic histopathology was present in the quadriceps, and exome sequencing revealed the VCP mutation c.290 C>T, p.Gly97Glu. Conclusions. Our patient reflects the clinical heterogeneity of VCP mutations, as his neurological localization is a spectrum between a lower motor neuron disorder and a hereditary axonal peripheral neuropathy such as CMT2. Our case demonstrates a rare manifestation of the c.290 C>T, pGly97Glu VCP mutation.
The valosin-containing protein (VCP) has been demonstrated to play a critical role in the maintenance of protein homeostasis through the regulation of protein degradation pathways . Mutations in the gene encoding VCP lead to disruption of autophagy and have been shown to manifest within families as a phenotypically heterogeneous group of presentations including hereditary inclusion body myopathy (IBM), Paget’s disease of the bone (PDB), and frontotemporal dementia (FTD), which are known collectively as IBMPFD . More recently, mutations in VCP have been implicated as having a role in familial amyotrophic lateral sclerosis (ALS) . Although there is variability in the phenotypic manifestations of VCP mutations within families, VCP-related disease is inherited in an autosomal dominant manner [2, 3]. Attempts at determining genotype-phenotype correlations for mutations in the VCP gene have been unsuccessful, though analysis of the two most common mutations (p.Arg155Cys and p.Arg155His) showed a significant variation in survival between the groups, suggesting that mutation variants may hold clinical relevance for patients .
We report a case of a patient presenting for evaluation of progressive lower extremity weakness and paresthesias in the context of family history of ALS, dementia, and PDB. Exome sequencing revealed a previously reported c.290 C>T, p.Gly97Glu mutation in VCP; our patient’s phenotype is different from the previously reported case of IBMPFD . The clinical presentation along a continuum from an axonal sensorimotor polyneuropathy to a lower motor neuronopathy in this patient expands the known clinical phenotypes of VCP-related disease.
2. Case Report
The proband is a 60-year-old man of Dutch and Italian descent who was the product of a normal pregnancy and delivery. Although he was a toe walker with high arches and hammertoes, he reached his developmental milestones (including walking) on time. In grade school, he kept up with his peers but was always the slowest runner. He rode a bicycle and played ice hockey until age 52 when he sprained his left ankle and began noticing progressive left greater than right leg weakness. At age 55, he began noticing paresthesias and numbness in his toes with the left side worse than the right. By age 57, holding his trumpet was difficult, although he could still play it well. Three years later, he was unable to climb stairs, had multiple falls, and began to use a walker. He later developed cramps in his hands and trouble with fine finger dexterity. Additionally, he noticed memory and word-finding difficulties.
Upon neurological examination in our clinic at age 60, he presented with bilateral scapular winging, lumbar lordosis, pes cavus, hammer toes, and tight heel cords (Figure 1). Strength exam (MRC scale) demonstrated proximal upper and proximal/distal lower extremity weakness. There was weakness in trapezius (R/L) 3/3 with scapular winging as well as weakness in the iliopsoas 4+/4+, quadriceps 4+/5, knee flexion 4/4, foot dorsiflexion 1/4, and foot plantar flexion 4+/5. Vibratory sense and pinprick were decreased distally in the lower extremities. Deep tendon reflexes were depressed throughout the upper and lower extremities with absent ankle jerks and downgoing toes. There was no tremor. Cerebellar testing was normal. Gait was wide-based with bilateral steppage; he was unable to walk 25 feet independently due to the weakness in his lower extremities. Speech and language were intact. Cranial nerves were normal including facial strength, facial sensation, and tongue strength. No fasciculations were noted on exam. Of note, he did not develop any upper motor neuron signs over the years including hyperreflexia, dysphagia, dysarthria, or dyspnea.
Laboratory testing included creatine kinase level of 930 u/L (normal 40–200 u/L) and a normal alkaline phosphatase level.
2.1. Electrodiagnostic Exam
Although the abnormalities on the EMG remained constant, nerve conduction studies (NCS) changed over time.
At age 56, needle EMG revealed fibrillations, positive sharp waves, fasciculations, and large amplitude motor unit potentials with reduced recruitment in bilateral tibialis anterior, bilateral vastus lateralis, left gastrocnemius, left deltoid, left first dorsal interosseous, and left thoracic paraspinal musculature. Nerve conduction studies of the left ulnar, sural, and tibial nerves were normal. At this time, the impression was a lower motor neuron syndrome.
At age 58, although the EMG remained the same, sensory and motor nerve conduction studies were now abnormal. The left median, ulnar, and peroneal motor nerve conduction velocities were normal. Compound muscle action potentials (CMAPs) of the left median, ulnar, and peroneal nerves were normal. Sensory nerve action potentials (SNAPs) of the left median, radial, and superficial peroneal nerves were normal; right ulnar SNAP had reduced amplitude (13 uV). The left sural and superficial peroneal sensory responses were absent; right sural sensory responses had reduced amplitude (4 uV). Left median and ulnar distal motor latencies were prolonged (5.5 ms and 4.2 ms, resp.); left median sensory distal latency was mildly prolonged at 4.0 ms. At this time, the impression was an axonal sensorimotor neuropathy.
The axonal sensorimotor neuropathy was worked up for acquired causes. Laboratory testing included normal thyroid stimulating hormone, serum protein electrophoresis, serum immunofixation, vitamin B-12, and fasting glucose.
2.2. Muscle Biopsy
Muscle biopsy of the quadriceps at age 60 revealed chronic, active neurogenic atrophy. Paraffin and frozen H&E sections, NADH, and trichrome staining revealed skeletal muscle with moderate variation in fiber size due to the presence of angulated, atrophic fibers in small groups (Figure 2(a)). Immunoperoxidase stains for slow and fast myosin heavy chains revealed small fiber type groups (Figures 2(b) and 2(c)). Rare targetoid fibers were seen (not shown here). Some muscle fascicles had end stage muscle pathology (Figure 2(d)), likely the result of longstanding neuropathic disease. No inflammation, myonecrosis, regeneration, endomysial fibrosis, inclusion bodies, rimmed vacuoles, or ragged-red fibers were identified. All fibers stained positively for cytochrome C oxidase.
2.3. Neuropsychological Testing
Neuropsychological testing revealed only mild impairments as he was noted to have high average to superior baseline functioning. He had only mild weakness in speed of information processing, problem solving, fine motor dexterity, speed in visual motor sequencing, rapid cognitive shifting, verbal memory, and short-term memory for simple geometric designs. He had an otherwise normal performance in all other areas (verbal, nonverbal intellectual skills, single word reading, and language). He was diagnosed with cognitive deficits, not otherwise specified.
2.4. Family History
Further investigation into the patient’s family history disclosed his father’s diagnoses of ALS, dementia, and PDB (Figure 3(a)). Muscle biopsy results from the father are not available. One sister had flat, narrow feet. The patient’s teenage daughter has high arches and was a toe walker as a child; she has not had a neurological evaluation and further evaluation has been declined at this time. His other teenage daughter is currently asymptomatic. There is no family history of IBM.
2.5. Genetic Testing
Exome sequencing was performed and revealed the VCP mutation c.290 C>T, p.Gly97Glu, G97E. This same mutation resulting in the same amino acid substitution has been reported in five family members with PDB. All five affected individuals had varying degrees of muscle weakness diagnosed as inclusion body myopathy, and none have FTD .
Phenotypic variability is a hallmark of VCP-related disease. VCP is a member of the type II AAA+ ATPase family mapped to 9p13.3 that is ubiquitously expressed at high levels and exerts an influence on a variety of cellular activities including cell cycle progression, DNA damage repair, the ubiquitin-proteasome system, and autophagic processes [7–9]. This variation in physiological activity allows for an equally impressive range of pathological manifestations to arise from mutations in the VCP gene, including IBM, PDB, FTD, and ALS . We believe the VCP mutation c.290 C>T, p.Gly97Glu, G97E mutation is pathogenic because the mutation in our patient shows increased ATPase activity, similar to other VCP mutations (Figure 3(b)).
Our patient’s presentation is not straightforward clinically. It is not simply a hereditary axonal sensorimotor polyneuropathy (given the asymmetry and bilateral scapular winging), a purely lower motor neuron syndrome (given the bilateral scapular winging and sensory nerve conduction abnormality) nor a myopathy (despite elevated CK and scapular winging, there were no myopathic features on quadriceps muscle biopsy). We propose that our patient presents along a spectrum of a lower motor neuron syndrome and axonal neuropathy (Figure 4). The difficulty in diagnosing a VCP-related condition is due to this extreme heterogeneity in clinical presentations. A recent study demonstrates that a VCP mutation can result in CMT 2 further validating the phenotypic variability seen in patients with a VCP mutation . Of note, exome sequencing was negative for other genetic variants that are known to cause CMT and a lower motor neuron disease phenotype; this included the HSPB, BSCL2, GARS, DCTN1, SLC5A7, FBX038, IGHMBP2, ATP7A, and SETX genes.
Of those individuals reported with VCP mutations, 87.7% presented with IBM, 45% with PDB, and 37.7% with FTD . VCP mutations are also estimated to be the cause of 1-2% of familial ALS cases . Further complicating this clinical scenario is the incomplete penetrance of phenotypes of IBM, PDB, and FTD as well as the variability in presentation for VCP-related ALS [12, 13]. The Gly97Glu mutation was previously reported in both affected and unaffected members of one family, suggesting that the mutation may not be fully penetrant . VCP mutations have also been demonstrated to manifest with variable upper motor neuron signs or bulbar findings. EMG results can be neuropathic or myopathic, with many displaying mixed morphology . Other features have been observed less frequently in VCP disease, including dilated cardiomyopathy, cataracts, and axonal sensorimotor polyneuropathy .
Because of the variability in clinical presentations, the family history is critical. Mutations in VCP are transmitted in an autosomal dominant fashion. Obtaining a pedigree that includes IBM, PDB, FTD, or ALS throughout multiple family members may lead to the consideration of VCP sequencing as a diagnostic step. Furthermore, repeated electrodiagnostic testing can be critical to evaluate the progression of the disease. Our case demonstrates how the electrodiagnostic testing changed over the years from the initial electrodiagnostic diagnosis of a lower motor neuron syndrome to the later diagnosis of motor neuron disease plus a sensorimotor axonal neuropathy. Although the neuropathy could be a manifestation of CMT 2 given his long-standing toe walking and pes cavus, the appearance of the neuropathy on electrodiagnostic testing was late. Basic testing for acquired neuropathies was unrevealing, and thus the electrodiagnostic changes could possibly reflect a late onset hereditary neuropathy like CMT 2.
Here we present a case with variable phenotypic manifestations ranging from an axonal sensorimotor polyneuropathy to a lower motor neuron syndrome or even a myopathy all arising from a mutation in the gene encoding VCP. This novel presentation further expands the clinical phenotype associated with VCP mutations and suggests that sequencing for this gene should be considered for any patients presenting with these symptoms who have family history positive for IBM, PDB, FTD, or ALS.
Informed consent was obtained from the patient.
Conflict of Interests
The authors declare that there is no conflict of interests regarding the publication of this paper.
Michael E. Shy would like to acknowledge support from the National Institute of Neurological Disorders and Stroke (NINDS) and Office of Rare Diseases (U54, NS065712) as well as grants from the Muscular Dystrophy Association (MDA) and Charcot Marie Tooth Association (CMTA). Nivedita U. Jerath would like to acknowledge support from an MDA Clinical Research Training grant and a University of Iowa Internal Funding Initiatives award. Cameron D. Crockett received funding by NIH through the Iowa Wellstone Muscular Dystrophy Cooperative Research Center (U54, NS053672). Steven A. Moore is supported in part by NIH through the Iowa Wellstone Muscular Dystrophy Cooperative Research Center (U54, NS053672). Tsui-Fen Chou is supported by the National Center for Advancing Translational Sciences through UCLA CTSI Grant UL1TR000124 and the LA BioMed Seed Grant program (20826-01) and is a member of UCLA Johnson Comprehensive Cancer Center.
- J.-S. Ju, R. A. Fuentealba, S. E. Miller et al., “Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease,” The Journal of Cell Biology, vol. 187, no. 6, pp. 875–888, 2009.
- G. D. J. Watts, J. Wymer, M. J. Kovach et al., “Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein,” Nature Genetics, vol. 36, no. 4, pp. 377–381, 2004.
- J. O. Johnson, J. Mandrioli, M. Benatar et al., “Exome sequencing reveals VCP mutations as a cause of familial ALS,” Neuron, vol. 68, no. 5, pp. 857–864, 2010.
- S. G. Mehta, M. Khare, R. Ramani et al., “Genotype-phenotype studies of VCP-associated inclusion body myopathy with Paget disease of bone and/or frontotemporal dementia,” Clinical Genetics, vol. 83, no. 5, pp. 422–431, 2013.
- J.-M. Gu, Y.-H. Ke, H. Yue et al., “A novel VCP mutation as the cause of atypical IBMPFD in a Chinese family,” Bone, vol. 52, no. 1, pp. 9–16, 2013.
- T.-F. Chou, S. L. Bulfer, C. C. Weihl et al., “Specific inhibition of p97/VCP ATPase and kinetic analysis demonstrate interaction between D1 and D2 ATPase domains,” Journal of Molecular Biology, vol. 426, no. 15, pp. 2886–2899, 2014.
- F. Bartolome, H.-C. Wu, V. S. Burchell et al., “Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels,” Neuron, vol. 78, no. 1, pp. 57–64, 2013.
- J.-S. Ju and C. C. Weihl, “Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy,” Human Molecular Genetics, vol. 19, no. 1, pp. R38–R45, 2010.
- Y. Iguchi, M. Katsuno, K. Ikenaka, S. Ishigaki, and G. Sobue, “Amyotrophic lateral sclerosis: an update on recent genetic insights,” Journal of Neurology, vol. 260, no. 11, pp. 2917–2927, 2013.
- A. Nalbandian, S. Donkervoort, E. Dec et al., “The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis,” Journal of Molecular Neuroscience, vol. 45, no. 3, pp. 522–531, 2011.
- M. A. Gonzalez, S. M. Feely, F. Speziani et al., “A novel mutation in VCP causes charcot-marie-tooth type 2 disease,” Brain, vol. 137, no. 11, pp. 2897–2902, 2014.
- V. E. Kimonis, E. Fulchiero, J. Vesa, and G. Watts, “VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder,” Biochimica et Biophysica Acta, vol. 1782, no. 12, pp. 744–748, 2008.
- M. Benatar, J. Wuu, C. Fernandez et al., “Motor neuron involvement in multisystem proteinopathy: implications for ALS,” Neurology, vol. 80, no. 20, pp. 1874–1880, 2013.
Copyright © 2015 Nivedita U. Jerath et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

domain: literature


text: A case-referent study, designed to test associations between asbestos, nickel, and the development of laryngeal cancer, was conducted in southern Ontario in 1977-1979. The cases were individually matched to neighborhood referents for gender and age. This constituted the primary study. Personal interviews had secured tobacco, alcohol, and detailed work histories. To 183 of the male pairs was added retrospective assessments of sulfuric acid exposure for each job, blind of disease status; this constituted the data base for an augmented secondary analysis. Logistic regression revealed statistically significant odds ratios when tobacco and alcohol were controlled. Exposure-response gradients were strongly positive with odds ratios in the range of 1.97 [95% confidence interval (95% CI) 0.63-6.13] for short duration-low level exposure through 6.91 [95% CI 2.20-21.74] for long duration-higher level exposure employing progressively more specific definitions of exposure. Asbestos as a confounder and the interaction terms examined were nonsignificant. These findings are corroborative of those of other studies.

domain: medical