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Cyprus PIO: Turkish Press and Other Media, 07-01-03
Cyprus Press and Information Office: Turkish Cypriot Press Review Directory - Previous Article - Next Article
From: The Republic of Cyprus Press and Information Office Server at <http://www.pio.gov.cy/>
TURKISH PRESS AND OTHER MEDIA No.02/07 03.01.07
[A] NEWS ITEMS
[01] The Cyprus problem is reportedly not on top of the agenda of the new UN Secretary General
[02] Turkish Cypriots recourse to the ECHR demanding their property in Limassol
[03] Serdar Denktas wants new formulas to be created for the solution of the Cyprus problem
[04] Statements by Ferdi Sabit Soyer
Turkish Cypriot daily KIBRIS newspaper (03.01.07) reports that Mr Ban Ki-Moon has officially taken over the duty of the UN Secretary General after the completion of the term of office of Mr Kofi Annan on 31 December 2006.
Meanwhile, invoking UN sources, Turkish Cypriot daily AFRIKA newspaper (03.01.07) reports that Mr Ki-Moon is not yet focused on the Cyprus problem and this problem is not on top of his agenda. The paper argues that the new UN Secretary General keeps distance from the Cyprus problem and he does not consider it to be a problem which awaits urgent solution because there is no bloodshed on the island.
(I/Ts.)
Turkish Cypriot daily KIBRIS newspaper (03.01.07) reports that after the Greek Cypriots the Turkish Cypriots as well began their legal struggle on the property issue. For the first time four Turkish Cypriot brothers have filed a lawsuit at the European Court of Human Rights demanding the return of their property in the free areas of the Republic of Cyprus and compensation about it.
In their lawsuit at the ECHR the brothers Hasan Huseyin Cakartas, Nejla Cagis, Mumin Cakartas and Gokcen Bayer demand the return of a 15-donum vineyard and an office building of 15 shops in Limassol and compensations for the damage they suffered. In case the application is considered admissible, the brothers will demand from the Republic of Cyprus the sum of seven million euros as compensation and the return of their property.
Lawyer Asli Aksu, who had sent the application to the ECHR, stated to Anatolia news agency that now is the time for the Turkish Cypriots to seek for their rights.
In the application the case of the Greek Cypriot refugee Titina Loizidou is shown as precedent and it is reminded that Turkey had paid 1.2 million dollars as compensation to Mrs Loizidou.
Aksu said that many Turkish Cypriots applied to him for filing lawsuits regarding their properties in the government controlled areas of the Republic of Cyprus and added that within the coming days he will launch the necessary procedure for these persons.
Aksu said that they exhausted the local remedies in the Greek Cypriot sector and afterwards they applied to the ECHR. Aksu noted that if the case is considered admissible, Britain might also intervene into the case, because Hasan Huseyin Cakartas is a British citizen.
Turkish Cypriot daily KIBRIS newspaper (03.01.07) reports that Serdar Denktas, leader of the Democratic Party (DP), has argued that the time came for the Turkish Cypriots to discuss and create new solution formulas on the solution of the Cyprus problem.
In statements to KIBRIS, he said that they are planning to open a platform for debate in order to determine a new policy regarding the Cyprus problem. Mr Denktas noted that the solution formula of from state to state between two states and the policy of the recognition of the TRNC reached nowhere. He noted that he does not expect activity regarding the Cyprus problem in 2007.
Mr Denktas argued that the UN procedure cannot lead the Cyprus problem to a solution. A solution based on the partnership with the Greek Cypriots and the political equality is not possible. This has obviously been seen, he alleged and claimed that in case a solution is not reached until 2008, the Turkish Cypriots must take their own course.
Referring to the EUs Direct Trade Regulation, Mr Denktas said that this has not great economic benefit for the Turkish Cypriots but the free trade will be very important for the Turkish Cypriot economy. He said that the Greek Cypriots will do everything they can to prevent this.
Meanwhile, in a statement he issued yesterday, Mr Denktas argued that the bridge at Ledra Street had become a sign of claiming their rights and a political stance for Turkish Cypriots and claimed that after the latest decision the Turkish Cypriots became the side which made a step backwards from claiming their right and its political stance.
Mr Denktas said that they do not know on which basis the presidency took the above-mentioned decision and what condition has changed. Denktas asked whom they consulted with when taking this decision. He noted: Has the issue been discussed at the council of ministers? Has it been discussed at the republican assembly? Has any dialogue been established on the issue with the administration in the south? If the answer to these questions is negative, then is the result of this decision not a crushing defeat once more?
Turkish Cypriot daily AFRIKA newspaper (03.01.07) reports that while the citrus fruit producers, the farmers and the hotel owners who do not find customers in other times of the year except during the holidays, are deeply distressed.
In statements to the Anatolia news agency the self-styled Prime Minister Ferdi Sabit Soyer is boasting saying that the per capita national income in the occupied areas of Cyprus increased from five thousand to eleven thousand dollars in three years. He, however, does not reveal what the source of this wonder is, adds the paper, which covers Mr Soyers statements under the title From 5 to 11 thousand dollars and above the title it says that this happened thanks to the casinos, the bet offices, the night clubs and the looting of the Greek Cypriot properties.
Mr Soyer said that while in 2005 the salaries in the occupied areas were at 45 % of the salaries in the free areas, in 2006 this percentage increased to 78 %. He noted that the policy of let the state take from Turkey and give to us is not right and noted that the extravagance in the current expenses must be prevented.
Mr Soyer criticized those who ask for early elections now alleging that they are the main responsible for the unilateral EU accession of the South Cyprus, which took the Turkish Cypriots as its hostage and try to blackmail Turkey.
Mr Soyer claimed that the Greek Cypriot nationalism is escalating and that President Papadopoulos cannot put back into the bottle the demons he forced to come out of it.
Mr Soyer said that their primary target in 2007 is to take the necessary initiatives for the lifting of the isolations and bring onto the agenda in a powerful manner the UN solution procedure.
Mr Soyer described 2006 as an important year for politically gaining the ground they have lost in the Cyprus problem.
tcpr2html v1.00 run on Wednesday, 3 January 2007 - 12:43:50 UTC


The only way to fully appreciate the scale is from above. Well, that or actually splashing around in it.
Too dark for 9 more? Lights are on at the 30-acre driving range.
Stroll by the slips and make up fun names for your family favorites.
Private balconies offer an opportunity for peace and quiet.
Shop, eat and have a good time at Barefoot Landing.
Barefoot Resort & Yacht Club is located in Myrtle Beach, along the Intracoastal Waterway. After getting settled in your condo rental, change into swimsuits, grab goggles and pool toys, slather everyone head to toe in sunblock. Get ready to hit the large freeform pool – it's the largest in South Carolina. Set up camp in a poolside lounger with your latest beach read. I have a feeling that you're going to be here for a while. The Barefoot Resort has 4 golf courses on-site, plus a 30-acre lighted driving range. Round up the golfers in your crew and go for it. But that's not all! The resort also offers tennis, volleyball and basketball courts and the on-site marina has water sports rentals available.
Individually decorated two- and three-bedroom villas sleep six to eight guests. Upgrade to a two-bedroom condo for more space or a three-bedroom condo for an extra bathroom. Four-bedroom condos sleep ten guests. Stop by the Super BI-LO for groceries – your full kitchen won't stock itself. Recruit the little ones to help bake chocolate-chip cookies (not that they'll need convincing). You'll be thankful for the in-suite washer/dryer in case things get messy. Throw in a quick load of laundry while you watch a Big Bang Theory rerun on the Cable TV.
Barefoot Landing is just across the Intracoastal Waterway and features a variety of shopping and dining. Get a new pair of shades at Sunglass Hut. Barefoot General Store features specialty food products along with Myrtle Beach souvenirs. Looking for a unique gift? Best Gift Idea Ever has you covered. They are known for their Letter Art which is made up of photos that feature letters found in everyday objects. Grab a burger at House of Blues, feast on seafood at Joe's Crab Shack or build your own pizza at Ultimate California Pizza. Barefoot Resort & Yacht Club offers a seasonal shuttle to the Myrtle Beach Boardwalk. Go for a ride on the Myrtle Beach SkyWheel. At almost 200 feet tall, the SkyWheel is an exciting adventure that's fun for all ages. Ripley's Odditorium displays the personal collection of strange artifacts owned by Robert Ripley, the creator of the "Believe It or Not" cartoons. The Children's Museum of South Carolina has several interactive exhibits that allow children the freedom of using their imagination while expanding their mind at the same time. They will have so much fun that they won't even realize that they are learning.
- The front desk is open 7:00 AM - 11:00 PM daily. The check-in location differs from the property location. To check in, go to 4403 Highway 17 South, North Myrtle Beach, SC 29582-5254. In order to check in after hours, guests must pay a damage waiver fee or have USD 100.00 put on hold on their credit card. Afterward, they will receive a code to a lock box located outside the front desk office with keys to the unit. Guests planning to arrive after hours must contact the property at least 48 hours prior to arrival using information on the booking confirmation.
- The parking of motorcycles, campers, boats, trailers, and/or commercial vehicles is strictly prohibited on site.


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Евге́ний Влади́мирович Корнийчу́к (, род. 18 октября 1966, Винница) — украинский политик, чрезвычайный и полномочный посол Украины в Израиле, народный депутат Украины V и VI созывов, 1-й заместитель министра юстиции Украины (с ноября 2007 по март 2010 года). Экс-председатель Украинской социал-демократической партии.

Образование

В 1987 году окончил Минское высшее военно-политическое училище. В 1996 году окончил Киевский университет им. Т. Г. Шевченко, юрист, а в 1998 году — Школу Южного методистского университета (шт. Техас, США), магистр права.

Биография

1987—1992 — проходил службу в армии.

1992—2000 — старший консультант Министерства юстиции Украины, консул Украины в Нью-Йорке.

В 1997—1998 — секретарь судьи Верховного суда Джерри Бушмейера, Северный округ Техаса, США. Работал заведующим отдела международных договоров МИД Украины, с 2000 года — старшим преподавателем Дипломатической академии при МИД Украины, старшим партнёром юридической фирмы «Магистр & Партнёры».

Политическая деятельность
В апреле 2006 года избран депутатом Киевской городской рады. Председатель Киевской городской организации УСДП (с марта 2005 года).

Народный депутат Украины V созыва (апрель 2006 — июнь 2007) от Блока Юлии Тимошенко, № 113 в списке.

С июля 2006 года — 1-й заместитель председателя Комитета по вопросам правовой политики. Сложил депутатские полномочия 15 июня 2007 года.

В ноябре 2006 на партийном съезде избран председателем УСДП и занимал данный пост до декабря 2011.

Народный депутат Украины 6-го созыва с ноября 2007 года от Блока Юлии Тимошенко, № 10 в списке.

Член Киевской городской коллегии адвокатов, Украино-американской ассоциации адвокатов. Отмечен в рейтинге «100 рекомендуемых юристов» издательства «Юридическая практика».

В мае 2008 года МИД России запретил Евгению Корнийчуку въезд на территорию России, что явилось ответом на запрет въезда Юрия Лужкова на территорию Украины.

22 декабря 2010 года Евгений Корнийчук был взят под стражу Генеральной прокуратурой Украины по обвинению в коррупционных действиях во время работы 1-м заместителем министра юстиции Украины.

15 февраля 2011 года следователь ГПУ сменил меру пресечения с ареста на подписку о невыезде.

В марте 2011 года Европейский суд по правам человека принял в производство жалобу Корнийчука о незаконности его задержания и ареста.

11 мая 2016 года Высший специализированный суд Украины вынес оправдательный приговор в деле экс-первого заместителя министра юстиции Украины Евгения Корнийчука. Коллегия судей ВССУ отклонила кассационную жалобу на приговор Печерского райсуда.

В январе 2018 года Европейский суд по правам человека в деле «Korniychuk v. Ukraine (no. 10042/11)» принял сторону Евгения Корнийчука, признав незаконным его арест в декабре 2010 года, поскольку он был проведён без судебного ордера. ЕСПЧ также обязал Украину выплатить Корнийчуку EUR 6,5 тыс нематериального ущерба.

10 сентября 2020 года президент Украины Владимир Зеленский своим указом №385/2020 назначил Евгения Корнийчука чрезвычайным и полномочным послом в Государстве Израиль. Посол Израиля на Украине Йоэль Лион высоко оценил назначение Евгения Корнийчука послом Украины в Израиле и отметил, что уверен в том, что он будет хорошо представлять интересы Украины в Израиле. 16 ноября Евгений Корнийчук приступил к выполнению обязанностей посла, вручив копии Верительных грамот Главе государственного протокола МИД Израиля Гилу Хаскелю.

Семья

В разводе.

Примечания

Ссылки
 Биография

Политики по алфавиту
Выпускники юридического факультета Киевского университета
Народные депутаты Верховной рады Украины V созыва
Народные депутаты Верховной рады Украины VI созыва
Заместители министров Украины
Члены партии «Украина — Вперёд!»

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By Mackenzie Stewart
State explores privatizing Pioneer Homes
With the state anxiously seeking ways to cut costs, one idea being discussed is privatizing its Pioneer Home assisted living facilities. The state recently took another step in exploring this possibility, and issued a request for letters of interest for potential privatization of the six Pioneer Homes.
"We're in a horrible financial situation," said Vickie Wilson, director of the Alaska Pioneer Homes, Division of Health and Social Services. "The legislature is asking us to consider what's out there for privatization. All 24-hour facilities owned by the Department of Health and Social Services have been asked to do this."
Diane Boyer, president of the resident council at the Anchorage Pioneer Home, says privatization of the homes has been up for discussion many times now.
"About a year ago, someone here locally expressed an interest in purchasing the homes with some sort of trust. Myself and the vice president of the resident council met with them, and they indicated that they would be the landlord. That concerned me," Boyer said.
There was another instance many years ago where Providence in Anchorage offered to purchase the Anchorage Pioneer Home, hoping to combine it with their own assisted living home. However, as the homes can only be purchased as a system, the offers fell through, Boyer said.
"I don't think privatization is the best way to solve this, though," Boyer said. "I think they want a request for proposal to solve the budget problem."
On Feb. 15, Wilson and Jessica Bogard, administrative operations manager for the homes, met with the House Finance for the Department of Health and Social Services Budget Subcommittee in Juneau to discuss budget cuts for 2016 and 2017 and the potential for privatization.
According to a PowerPoint presentation provided by Wilson and Bogard, the homes currently take up 2.3 percent of the Dept. of Health and Social Services budget.
The state's six homes currently house 535 residents, with 325 on the active waiting list and another 4,093 on the inactive list, according to the presentation.
After the budget reductions for the 2016 fiscal year, two full time management positions, 17 positions within the homes and various services and commodities have been cut.
The recent rate increase, effective Feb. 1, will add an estimated revenue of $436,900 for the rest of the 2016 fiscal year. However, it isn't yet clear if the rate increase will cause certain residents to be forced to join the payment assistance program, Wilson said.
The projected budget cuts for the 2017 fiscal year will continue to reduce the homes' staffing levels and will cause longer wait times for admissions due to the reduced bed count.
According to the PowerPoint information, the homes' number one priority in terms of care is "health and wellness across the lifespan," which includes an emphasis on core services like memory care.
Most residents in the homes average 86 years of age and frequently request "level three" services like memory care and extra medical care. According to the presentation material, 58 percent of the residents in the homes have been diagnosed with dementia, compared with the rest of the United States' average of 39.6 percent for the number of assisted living community residents diagnosed with dementia.
"I've spent 18 years with the homes," said Wilson, "and this number is higher than it's been before. We're here to help people manage cueing and act as safety nets."
The Anchorage home has three units, and each one specializes in a certain type of care depending on the residents' care levels.
"Southside is for the level ones and twos," said Boyer. "A level one means that you are able to do 100 percent of the work on your own. A level two means that you are able to do 75 percent of the work on your own. You basically just need help with making your bed or taking your medications, little things like that.
"Sunset View is for level threes that require extra medical care. A level three means that you can do 25 percent of the work on your own, and you need help with the remaining 75 percent. Delaney Gardens is also for level threes, but these level threes have great mobility. They just need memory care."
According to Boyer, most other assisted living homes across the state and in the rest of the Lower 48 only do memory care or extra medical care, not both, and the level threes that would need the extra medical care would normally be considered for a nursing home.
"I've had the occasion to see other assisted living homes, and their employees don't give their residents the same level of care as ours do. Our staff has been doing a fabulous job, even with the budget cuts," Boyer said.
"All of our homes manage this type of care," Wilson said. "I think that people don't really realize the extent of the care we have and the dignity that we are able to continue to manage to provide for these individuals."
Future of the homes
After leaving a request for letters of interest open for 28 days, the homes have received a handful of responses.
NANA Management Services, who already provides food services for all the homes and housekeeping services for the Juneau home, offered to provide housekeeping, food service and maintenance services until they would be able to find a partner to provide direct care services. Southeast Alaska Regional Health Consortium has offered to purchase the entire Pioneer Home system. Marathon Development, Inc., from Washington has offered to manage operations and lease assets if it is determined profitable. Retirement Housing Foundation from California has offered to manage operations.
"The letters of interest were a no-cost attempt to test the waters of privatization," said Bogard. "At this point, we want to go forward with a feasibility study to see the options."
Although these initial letters are merely offers, there was nothing stating that the potential new owners would maintain the current client mix.
"There were no responses that guaranteed that those that were the most vulnerable or with a lack of funds would remain within the facilities and that they would be cared for in the future," Wilson said. "That was disappointing, but not surprising."
"I personally don't see how anyone could operate this any better than the state," said Boyer. "Someone that's not familiar with this type of business won't understand this type of care and the certain staffing ratios that we need to follow. There's a lot of unanswered questions. What would happen with the maintenance programs? What would happen with the activity programs or the resident advisory board? I don't think this is beneficial for the residents. Our level of care is excellent. I think privatization would take away from our level of care."
The subcommittee hearing discussed above is archived for viewing online at http://www.akleg.gov/basis/Meeting/Detail?Meeting=HDHS%202016-02-15%2008:30:00#tab4_4.
Click the "Documents" tab on that page to view or download the PowerPoint presentation.
Legislative conferences provide updates on senior bills, budgets
Learn what scams to watch for in "Age Smart" online forum, Dec. 8
Make a difference as an AARP Foundation Tax-Aide volunteer
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Exercise and fitness are more important than ever
Exercise and fitness a...Senior Voice Staff

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Chi Hi students open pop up shop
By Sarah Winkelmann |
Posted: Wed 5:05 AM, Dec 04, 2019 |
Updated: Wed 9:05 AM, Dec 04, 2019
CHIPPEWA FALLS, Wis. (WEAU) -- If you still have some holiday shopping to do, there is a pop up shop coming to the Chippewa Valley and its run entirely by students. Chi Hi students have been learning all year about retail merchandising and now they are putting their skills to the test.
Many people have worked in retail at some point during their time in high school, but instead of just working at the mall, a group of about 20 students from Chi Hi are taking their merchandise retail knowledge to the next level.
It's all hands on deck in Heidi Warren's classroom as all of the work the students do is hands-on. "I didn't realize how much work goes into it," said senior student, Emma Maes.
The students work on everything from the initial advertising to staffing the store. "I have pretty hands off experience with them and it's neat to see them roll with everything that we have going on," said marketing teacher, Heidi Warren.
Her class doesn't require any tests, just putting the material into action. "Everything we learned in the retail classes or principels of marketing classes that is all going to be put to the test, we get real world experience with it," said Jake Caron, a senior student. The class is transforming the typical classroom setting into a real life learning experience. "Students are working it, stocking it, figuring out what to purchase, how to price it and all of the market planning that goes into a retail space," Warren said.
But this week, they are packing up the school store to head downtown Chippewa Falls and open up a pop up shop. "We're selling sweatshirts, t-shirts, pants, pajama pants, bags, hats, water bottles, all kinds of apparel and accessories," Maes said.
They will be selling the Chi Hi apparel to the community and all of that money goes right back into the retail merchandising class. "I'm really interested in marketing and the whole business field and I plan on going into it in my future and getting the experience from this class is really going to be helpful and give me some future skills," Maes said.
The students are paving their way to the future, one purchase at a time. "It's not just something we learn and forget, it something we learn and really get to use," Caron said.
The pop up shop will be at the Legacy Community Center in downtown Chippewa Falls from Thursday through Sunday. The Shop will be open:
Thursday 8:30 a.m. to 7 p.m.
Friday 8:30 a.m. to 8 p.m.
Sunday 11 a.m. to 4 p.m.


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As Americans Observe Memorial Day, The U.S Inches Towards A Grim COVID-19 Milestone
Tomorrow on Memorial Day, the United States will probably reach 100,000 deaths in the current pandemic. It's a staggering, incomprehensible number, more than the nation lost in the Vietnam and Korean wars combined. Yet a pandemic isn't a war. The horrors of the battlefield are unique, and the fighters who fall there deserve the moment of honor and reflection we give them tomorrow. But there are echoes of wartime struggles in a pandemic, fear as we face harm and struggle, especially the real physical struggle of those who fall ill. We haven't been talking about things like ventilators recently, but there are people on them fighting to breathe, now in places like Montgomery, Ala., where the ICUs are full this weekend. There is also in this pandemic sacrifice. Forty million people are jobless, another staggering incomprehensible number. But there is also hope. New York state, the hardest hit by the pandemic, has seen its deaths plummet to below 100 a day from over a thousand. The lesson - a shared sense of duty to wear a mask, to keep our distance, to be mindful of the people and communities who are particularly vulnerable and to keep them from being needlessly in harm's way can save lives. Transcript provided by NPR, Copyright NPR.
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The Carolinas and Virginia brace for blast of ice and snow
Clemson fraternity suspended for 4 years after hazing probe
This Marine-turned-journalist interviewed the Taliban commander he had fought against


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Home>World >North Korea says it tested new long-range cruise missiles
North Korea says it tested new long-range cruise missiles
By PTI Updated: Sep 13, 2021 9:03 pm
People watch a TV screen showing a news program reporting about North Korea's long-range cruise missiles tests with images in Seoul, South Korea, Monday, Sept. 13, 2021. North Korea says it successfully test fired newly developed long-range cruise missiles over the weekend, its first known testing activity in months, underscoring how it continues to expand its military capabilities amid a stalemate in nuclear negotiations with the United States. The letters read, "The North newly test-fired long-range cruise missiles. " AP/PTI
Seoul, Sep. 13 (PTI/AP): North Korea said Monday that it successfully tested newly developed long-range cruise missiles over the weekend, the first known testing activity in months, underscoring how the country continues to expand its military capabilities amid a stalemate in nuclear negotiations with the United States.
The state-run Korean Central News Agency reported that the missiles showed they can hit targets 1,500 kilometers (930 miles) away on Saturday and Sunday. State media published photos of a projectile being fired from a launcher truck and what looked like a missile traveling in the air.
The North hailed its new missiles as a strategic weapon of great significance wording that implies they were developed with the intent to arm them with nuclear warheads.
North Korea says it needs nuclear weapons in order to deter what it claims is hostility from the U.S. and South Korea and has long attempted to use the threat of such an arsenal to extract much-needed economic aid or otherwise apply pressure. The North and ally China faced off against South Korea and U.S.-led U.N. forces in the 1950-53 Korean War, a conflict that ended in an armistice that has yet to be replaced with a peace treaty.
The international community is bent on getting the North to abandon its nuclear arsenal and has long used a combination of the threat of sanctions and the promise of economic help to try to influence the North. But U.S.-led negotiations on the nuclear issue have been stalled since the collapse of a summit between then-U.S. President Donald Trump and leader Kim Jong Un in 2019. At that time, the Americans rejected Kim's demand for major sanctions relief in exchange for dismantling an aging nuclear complex.
North Korea ended a yearlong pause in ballistic tests in March by firing two short-range missiles into the sea, continuing a tradition of testing new U.S. administrations to measure Washington's response. Kim's government has so far rejected the Biden administration's overtures for dialogue, demanding that Washington abandon its hostile policies first a reference to the U.S. maintaining sanctions and a military alliance with South Korea.
The United States keeps about 28,000 troops in South Korea to help deter potential aggression from North Korea, a legacy of the Korean War.
There hadn't been any known test launches for months since March, as Kim focused his efforts on fending off the coronavirus and salvaging an economy damaged by sanctions, bad flooding in recent summers, and border closures amid the coronavirus pandemic. Experts have warned that the economic situation is dire, although monitoring groups have yet to detect signs of mass starvation or major instability.
The report of the tests comes before U.S. President Joe Biden's special representative for North Korea, Sung Kim, was to meet his South Korean and Japanese counterparts in Tokyo on Tuesday to discuss the stalled nuclear diplomacy with North Korea.
South Korea's military is analyzing the North Korean launches based on U.S. and South Korean intelligence, the Joint Chiefs of Staff said in a statement. South Korean Foreign Minister Chung Eui-yong said after a meeting with Australian Foreign Minister Marise Payne that the resumption of testing activity illustrates an urgent need for reviving diplomacy with the North.
The U.S. Indo-Pacific Command said it was monitoring the situation with allies and that the North Korean activity reflects a continuing focus on developing its military program and the threats that poses to its neighbors and the international community. Japan said it was extremely concerned.
China, the North's staunchest ally, didn't comment on the missiles when asked. A spokesperson for its Foreign Ministry, Zhao Lijian, only urged all parties concerned to exercise restraint, move in the same direction, actively engage in dialogue and contact to reach a political settlement.
Kim doubled down on his pledge to bolster his nuclear deterrent in the face of U.S. sanctions and pressure at a congress of the ruling Workers' Party in January. He issued a long wish list of new sophisticated equipment, including longer-range intercontinental ballistic missiles, nuclear-powered submarines, spy satellites and tactical nuclear weapons.
KCNA said the missiles tested over the weekend travelled for 126 minutes above North Korean territory before hitting their targets.
"In all, the efficiency and practicality of the weapon system operation was confirmed to be excellent, it said.
It appeared that Kim wasn't in attendance to observe the tests. KCNA said Kim's top military official, Pak Jong Chon, observed the test-firings and called for the country's defense scientists to go all out to increase the North's military capabilities.
Japanese Chief Cabinet Secretary Katsunobu Kato said North Korean missiles of such range would pose a serious threat to the peace and safety of Japan and its surrounding areas.
He said Tokyo was working with Washington and Seoul to gather information on North Korea's latest tests but said there was no immediate indication that the weapons reached inside Japan's exclusive economic zone.
Kim's powerful sister last month hinted that North Korea was ready to resume weapons testing while issuing a statement berating the United States and South Korea for continuing their joint military exercises, which she said was the most vivid expression of U.S. hostile policy.
The allies say their drills are defensive in nature, but they have canceled or downsized them in recent years to create space for diplomacy or in response to Covid-19.
The latest tests came after Kim threw an unusual parade last week in the capital, Pyongyang, that was a marked departure from past militaristic displays, showcasing anti-virus workers in hazmat suits and civil defense organizations involved in industrial work and rebuilding communities destroyed by floods, instead of missiles and other provocative weaponry.
Experts said the parade was focused on domestic unity as Kim faces perhaps his toughest test, with his economy in tatters.
By PTI Updated: Sep 13, 2021 9:03:32 pm
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Digi-buzz


text:
Q: ElasticSearch use match_phrase and multi_match together I use ES 7.1, I want to query using multi match to grab result from several fields, but I want to grab results with match_phrase on a specific field.
I tried a lot of things, this is the version the closest on what I want :
'bool' : {
    'must' : {
        'multi_match' : {
            'fields' => ['titre', 'subtitre', 'description'],
            'query' => $query
        },
        'match_phrase' : {
            'titre' => $query
        }
    },
    'filters' : { // ... some filters }
}

I get an error :

parsing_exception","reason":"[multi_match] malformed query, expected
  [END_OBJECT] but found [FIELD_NAME]

Is there a way to combine both of criterias ? I don't want to create 2 queries.

A: When you want more than one must subquery, you just need to pass the subqueries in as an array. This modification should work (translated to JSON):
{
  "query": {
    "bool": {
      "must": [
        {
          "multi_match": {
            "query": "hello",
            "fields": ["name", "brand_name"]
          }
        },
        {
          "match_phrase": {
            "title": "hello"
          }
        }
      ]
    }
  }
}

redpajama_set_name: RedPajamaStackExchange


text:
Q: PSQL Hibernate Native insert values from a list I have a table user_services
    Column     |  Type   |
---------------+---------+
 user_id       | integer |
 subservice_id | integer |

I have a SortedSet of subservice_ids in Java that I want to insert into my table each with the same user_id. Can this be done in a single INSERT-statement using native Hibernate SQL?
What if I do something like:
String values = StringUtils.join(mySubServiceIds, ',');

Query query = createSQLQuery("INSERT INTO user_subservices " +
"(user_id, subservice_id) " +
"SELECT 1 id, x " +
"FROM unnest(ARRAY["+ values + "]) x");

Would that work and be good?


Locke the ingenious author of the late ' Moon Story' ' is putting on the stocks the frame of a new. The series takes place in a fictional universe, the same world as Toriyama' s previous series Dr. More more Kickstarter , Indiegogo project creators are trusting CrowdOx to send dynamic product surveys, raise additional funding, verify backer orders ship their projects on time. Pet rescue saga android level 224.
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8 Man ( 8マン) Eightman ( エイトマン, Eitoman) is a manga , anime superhero created in 1963 by science fiction writer Kazumasa Hirai manga artist Jiro Kuwata. It marked the beginning of the Mario the original Donkey Kong, including Mario himself, Donkey Kong franchises, introduced several of the earliest characters Lady. Case Title: 1: Mobile Value Added Services ( MVAS Mobile) : The Next Big Avenue for Mobile Operators?
The manga was published in Weekly. He is considered Japan' s earliest cyborg superhero predating even Kamen Rider ( the same year, Shotaro Ishinomori created Cyborg 009) was supposedly the inspiration for RoboCop. Qq音乐是腾讯公司推出的一款网络音乐服务产品, 海量音乐在线试听、 新歌热歌在线首发、 歌词翻译、 手机铃声下载、 高品质无损音乐试听、 海量无损曲库、 正版音乐下载、 空间背景音乐设置、 mv观看等, 是互联网音乐播放和下载的优选。.
Donkey Kong is an arcade game that was Nintendo' s first big hit in North America. Not wasting time: Bill Belichick has already changed name of his boat to reflect Super Bowl win. Pet rescue saga android level 224.
A version of the game was also created later for the Nintendo Entertainment System Nintendo' s first home console under Arcade jects Using CrowdOx. 2: Tech Mahindra Acquiring Majority Stakes in Satyam Computer Services Ltd.
Use the SPACE key to scroll down at this page, it is very handy at any long website, and you get used to it very fast! This is the permanent and primary page for Pet Rescue Saga Coins, you can also find coins in the other Pet Rescue Saga Coins Page here at the Blogging Witches, if the Admin is in need of extra' s ; - ) These coins are collected by a small community from Pet Rescue Saga.


SMSF members can now arrange binding Death Benefit Agreements that do not lapse after 3 years — the agreement binds the trustee(s) until the member revokes it.
Why are the new Death Benefit Agreements needed?
ClearLaw has previously warned on the risks associated with making binding death benefit nominations that are not consistent with all of the requirements specified in superannuation law, including the 3 year lapsing requirement (click here to read more).
Many SMSF members want to bind their trustees as to how their benefits are to be paid after they die. The trouble is, superannuation law requirements say that after 3 years, a binding death benefit nomination expires. After it expires, the trustees (or other directors of the trustee) are free to distribute the relevant money anyway they like that the law allows — often, even to themselves.
How can SMSF members eliminate uncertainty about how their death benefits will be paid?
incorporating specific death benefit payment provisions into the SMSF's deed through a Death Benefit Agreement and then implementing the permanent (until revoked) binding Death Benefit Agreement.
How can an SMSF deed allow Death Benefit Agreements?
allows the SMSF member to terminate the agreement by notice to the trustee or to replace it with another Death Benefit Agreement.
How does the Death Benefit Agreement work?
the member replaces it with another Death Benefit Agreement.
Do I need to update the SMSF deed?
The Death Benefit Agreement depends on the SMSF deed containing the clauses that support the Agreement. Without those clauses, the Agreement would be invalid. So, any SMSF member with an existing fund who wishes to make a Death Benefit Agreement, needs to arrange for the SMSF's deed to be updated first.
Other good reasons to update SMSF deeds now are set out in another ClearLaw article.
For questions or more information about SMSF deeds, the payment of death benefits and succession planning generally call Maddocks in Melbourne (03 9288 0555) and ask for a member of the Maddocks Superannuation Team.


text:
Precision Health Initiative Seminar
Time: Thu Mar 16, 2017, 12:00PM - 1:00PM
Location: IU School of Medicine - R3 203
"Development of new targeted therapies for leukemia: pathway to the clinic."
Dr. Jolanta Grembecka, Associate Professor, Proteomics and Small Molecule Experimental Pathology, University of Michigan
Thursday, March 16, 2017 | 12:00pm – 1:00pm | R3 C203
Dr. Jolanta Grembecka received her Ph.D. in Chemistry at Wroclaw University of Technology, Poland. She completed two postdoctoral trainings at the University of Virginia, in structural biology and drug discovery, before she got promoted to Research Assistant Professor. In 2009, Dr. Grembecka joined the Department of Pathology at the University of Michigan where she is now an Associate Professor. Dr. Grembecka's research has focused on the development of small molecule inhibitors of proteins involved in oncogenesis, with a particular focus on leukemia related proteins. Dr. Grembecka is a co-author on 60 scientific papers in top ranked journals, including Cancer Cell, Science, Nature Chemical Biology, Leukemia, and an inventor on 6 patents. She is regularly invited as a speaker at national and international symposia related to her field. She serves as a reviewer on different grant review committees, including the Career Development Program from the Leukemia and Lymphoma Society.
Recent work from Dr. Grembecka's laboratory has been dedicated to identify and develop small molecules targeting the protein-protein interaction between menin and MLL as a new targeted therapy for acute leukemia patients with translocations of the MLL gene. Her laboratory has developed the first small molecule inhibitors of the menin-MLL interaction, which they successfully optimized resulting in very potent compounds that are currently being translated to clinical studies. Her laboratory is also pursuing development of new targeted therapies for hematologic and solid cancers by blocking novel epigenetic targets, including histone methyltransferases and ubiquitin ligases.
For more information, contact Crissy Quenichet ctesta@iu.edu.
Originally published at ctsi.nd.edu.


text:
Log in via CCMG
Anderson-Fabry disease: clinical manifestations and impact of disease in a cohort of 60 obligate carrier females
K D MacDermota,
A Holmesa,
A H Minersb
aDepartment of Medicine, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK, bPrimary Care and Population Sciences, University College Medical School, Royal Free Campus, Rowland Hill Street, London NW3 2QG, UK
Dr MacDermot, kmacdermot{at}ucl.rfc.ac.uk
http://dx.doi.org/10.1136/jmg.38.11.769
Editor—Anderson-Fabry disease (AFD) is a sphingolipid storage disorder resulting from the deficiency of the lysosomal enzyme α-galactosidase. Unlike most other lysosomal diseases, the inheritance is X linked. Disease manifestations in female heterozygotes have been reported, but are considered to be rare and usually mild.1 Asymptomatic corneal dystrophy (cornea verticillata and posterior lenticular cataract) is present in about 70% and is useful for heterozygote detection. About 30% of women have minimal angiokeratomas and <10% have infrequent attacks of neuropathic pains.1 However, female heterozygotes with severe and early cerebrovascular disease, strokes, and renal failure have been documented, but these serious manifestations were estimated to occur in only 1%.1 Because this is an X linked disorder, these severe manifestations in females were explained by skewed X inactivation.2
Case reports are open to selection bias and there are no reported data on the frequency and severity of AFD manifestations in a large cohort of carriers. Women are not usually evaluated in the clinic unless they present with serious AFD complications, but they do attend for genetic counselling when their son or male relative is diagnosed with the disease.
During regular reviews of AFD families in the genetic clinic, we have observed multiple and more frequent AFD manifestations in female carriers than expected. Owing to the lack of clinical data and present health care policy, we were unable to perform clinical investigations. Therefore, a large cohort study using an AFD specific questionnaire was performed to establish the mortality and the frequency of all AFD manifestations in obligate carrier females.
All known (n=80) obligate carrier females, who were over the age of 18 and had demographic data recorded in the AFD register, were asked to participate in this study. Sixty (75%) agreed and provided data; non-responders were reminded on one occasion. Some participants did not complete all measures, so the exact number is reported for each result. Local ethical committee approval was obtained at the start of the study.
ASCERTAINMENT
We have studied women who are obligate carriers for AFD by family history. This approach eliminates selection bias, as complete clinical, biochemical, and genetic mutation data were available mainly for young women of childbearing age for the purpose of prenatal diagnosis. Obligate carrier females were ascertained from the UK AFD register, which contains data on 67 families and 80 currently living affected males. This genetic and clinical register has been maintained for 15 years and contains demographic, clinical, biochemical, and DNA data on families from the UK and Eire. Data were obtained from continuous assessment in the genetic clinic, which are shared with general practitioners and supplemented by results from individual patients' hospital specialists. α-galactosidase gene mutations were identified in all families (except one), were different in each family, and included nonsense, missense, stop, and exon skipping mutations.3
MORTALITY DATA
Obligate carriers included were those who have at least one first degree relative known to us, and who were able to give information on the diagnosis and cause of death or provide a death certificate.
AFD SPECIFIC QUESTIONNAIRE
Carriers for AFD were assessed in the genetic clinic, following the diagnosis of AFD in their family, or following incidental findings of cornea verticillata or angiokeratoma in a female without a previous family history of the disease. The AFD specific questionnaire was thus created, based on in depth interviews with patients and medical practitioners. (The text of this questionnaire is available electronically on request.) In the questionnaire, the obligate carrier status was first confirmed by a statement that the woman had/has an affected father or brother or has an affected son. The questionnaire contained questions on general health and the presence or absence of manifestations of the disease as is seen in affected males. The character and nature of neuropathic pains were clearly explained and direct responses requested as to the onset of pain, medication, and its effect on everyday life. The McGill Pain Questionnaire (short form)4 was further adapted for carriers by selecting the following: the intensity of the pain on average during an attack on a scale of 0 = no pain to 10 = pain as bad as you can imagine, the presence and intensity of background pain on average, its location, and effect on attendance at school, work, social activities, and participation at sports (scale 0-10). Further, women were asked direct questions: have you ever suffered a stroke and have you ever experienced a weakness (in your arm, leg, or face) or double vision lasting only minutes/hours? We have followed all reported TIA and CVA by a phone call to these women and to their general practitioners to obtain a detailed history. Information on renal function was obtained by asking whether any blood and urine test was done in the last year, whether it was normal, is the woman under assessment by a renal unit, is on renal dialysis, or had renal transplant. For heart function, a direct question was asked about chest pain/palpitations and results of tests performed in the last year. Next, women were asked to respond yes/no to specific questions on the presence of manifestations of AFD, which included "Fabry rash" (angiokeratoma), sweating, tinnitus, exhaustion, gastrointestinal symptoms, and others. X linked inheritance and the understanding of AFD transmission was assessed simply by asking whether an affected father can pass on the genetic mutation to his son or to his daughter and, similarly, what were the chances of transmission of the disease by a carrier mother.
Lastly, the ratings of medical care and this questionnaire were assessed, followed by a series of questions about the respondents' reproductive choices. The effect of the disease on emotional health was measured on a 10 item table from the McGill questionnaire (short form).4
The cumulative probability of death for AFD female carriers was calculated using the Kaplan-Meier survival method and current data on the UK female general population was provided by the UK Government Actuaries Department using 1997-1999 life tables.
The survival function in fig 1 shows the median cumulative survival to be 70 years, which represents an approximate reduction of 15 years from the general population. A gradual decline of the AFD female carrier curve is evident from the age of 35. Six of these 32 women had renal failure, of whom half have had a renal transplant and two have died of lymphoma following immunosuppression for renal transplantation. Results of renal biopsy were not available, as all these women presented with end stage renal failure (ESRF). A total of 15.6% (5/32) had mitral valve replacement and cardiomyopathy, 28% (9/32) died of cerebrovascular accidents (CVAs), often multiple, aged between 56 and 83 years, and four died of cancer.
Cumulative probability of death for AFD female carriers calculated using the Kaplan-Meier survival method. The survival function shows the median cumulative survival to be 70 years, which represents an approximate reduction of 15 years from the general population. A gradual decline of the AFD female carrier curve is evident from the age of 35.
The prevalence of AFD carriers is 1 in 339 000 females in the UK. The AFD register contains data on 67 families with AFD of whom 47 had a family history of the disease and 20 affected males did not. In half of these, carrier status in their mother was confirmed by clinical examination, leucocyte, and hair root α-galactosidase estimations, and subsequently by mutation analysis. In the remaining males, the origin of the new mutation is at present unknown.
The register contains data on 89 obligate and manifesting carriers and 40 female first degree relatives, whose carrier status is at present unknown. The cohort examined in this study consisted of 60 obligate carrier females, mean age 44.9 years (95% CI 41.01, 48.79). Past medical history included the following: bronchitis in 31.7%, hypertension in 31.7%, and heart attack, angina, or heart failure in 16.7%; a further 3.3% were treated for breast cancer and 5% were given a diagnosis of rheumatic fever in the past. None had diabetes.
SERIOUS AND DEBILITATING AFD MANIFESTATIONS
Multiple serious or debilitating complications of the disease were documented in affected males, but were not expected in carrier women. In 30% (20 women), multiple and serious manifestations were present (table 1). These are illustrated by the following case histories. A 37 year old carrier presented with ESRF and has attacks of diplopia and severe peripheral neuropathic pains. Unusually severe AFD symptoms, even for a hemizygous male, were observed in a young girl aged 17. She was confined to bed for three months during her last year at school with severe incapacitating peripheral neuropathic and muscle pains and extreme exhaustion. Following home tuition, she was able to complete a university course and obtain a job. At her present age of 26, her pain control has been obtained with high doses of Gabapentine, Amitryptiline, and Solpadeine. However, she is unable to travel to work or carry out her desk job because of extreme fatigue, myalgia, and acute attacks of pain. Another 36 year old carrier appears to suffer from a personality disorder and has severe attacks of pain requiring treatment with morphine. In childhood, the nature of her pains were not recognised and she was under psychiatric care for many years, until her father developed renal failure and the diagnosis was made.
Frequency of serious and debilitating manifestations in AFD carriers (n=60)
Seventy percent (n=60) of carriers have experienced some form of neuropathic pain (n=42) and 30% have not (n=18). Of the total number of AFD carriers who reported experiencing pain, 42 (80%) said that the pain had never stopped since onset. However, in 10 carriers (20%) the pain did stop, at a mean age of 28.8 (95% CI 17.3, 40.3). Of the 42 carriers with pain, 41 women had pain attacks during pyrexial illness or in hot weather, of whom 19 also had continuous background pain in between attacks. Only one woman experienced continuous pain alone. The mean pain score for pain intensity during an attack was 7.4 (scale 0-10) and for background pain was 6.8.
A total of 17.1% were taking anticonvulsants and/or opiates. Over half of the remainder took non-steroidal anti-inflammatory drugs regularly. The commonest pain location in women carriers for AFD were the hands and feet, followed by the large joints of the upper and lower limbs.
A total of 27.5% (n=29) stated that the pain affected their attendance at school (mean score of 8, McGill questionnaire), in 55.5% (n=28) it affected participation in sports (mean score 8.6), it affected family life in 27.5% (mean score 6.6), and social activities in 46.6% (mean score 6.5). These results suggest that neuropathic pain is a significant manifestation of AFD in a high proportion of carrier females.
Table 2 shows the onset and table 3 shows the frequency of AFD disease specific manifestations in carriers >18 years. The response to the questionnaire was complete in this cohort; however, data on renal and cardiac function are approximate for frequency in carriers as only a third of the women were investigated.
Onset of disease manifestations in AFD carriers >18 years
Frequency of disease manifestations in AFD carriers >18 years
TIA AND CVA
Twenty-three women stated that they had experienced at least one TIA. Table 4 shows the signs and symptoms in 17 carriers in whom TIA was considered likely; a further three women were excluded and three remained unknown. Four women with CVA have also recorded a previous history of TIAs.
Transient ischaemic attacks in AFD carriers
RENAL FUNCTION
Renal function was assessed in only 31.5% of carriers in the last year. Two women are receiving renal dialysis at a mean age 36, of whom one has presented with ESRF. None of the respondents has had renal transplant.
Cardiac function was assessed in only 35.6% in the last year, but 52.5% have reported chest pains and/or palpitations and ankle swelling; 16.9% of women had abnormal test results (table 3).
GASTROINTESTINAL (GI) SYMPTOMS
GI symptoms were commonly recorded; over half of the respondents complained of bloatedness, indigestion, and abdominal cramps. Diarrhoea and constipation were present in over 40% and vomiting in 14%. No additional diagnosis of bowel disease was reported by these women. The symptoms are suggestive of being the manifestation of AFD, but the results of GI investigations are required to confirm these findings.
The frequency of angiokeratoma is most likely an underestimate, as on clinical examination several carriers in this cohort had sparse lesions, in the form of diffuse tortuous capillaries on the upper chest or patches of angiokeratoma in dermatome distribution on the upper or lower limbs.
In this cohort, hypohidrosis was reported by 32.8% as an inability to sweat on exertion, during sport sessions, and when pyrexial. The triad of hypohidrosis, overheating, and mild peripheral pains was used by the women as diagnostic of their carrier status. Tinnitus was reported by 25%, in the form of unpleasant attacks, usually lasting seconds or minutes, often associated with dizziness.
Disabling joint and muscle pains, diagnosed as fibromyalgia were present in five carriers (7%) aged between 45 and 55 years. They have severely reduced mobility, experience constant pain, and are unable to work. One woman presented with polyarteritis nodosa-like symptoms and with proteinuria. One carrier presented with muscle pains and localised proximal muscle atrophy in the lower limb and neurological investigations failed to identify the cause.
MEDICAL CARE RATINGS
The majority of carriers (66%, n=59) rated the AFD information given at diagnosis as excellent or satisfactory and 44% rated it as poor. Genetic counselling was evenly divided between poor and satisfactory. Routine medical assessment is not being carried out in two-thirds of women.
REPRODUCTIVE DECISION MAKING
Responses are shown in fig 2.
Reproductive decisions taken by AFD carrier women who had children after 1980. TOP=termination of pregnancy.
About a third of the sample reported being happy, full of life, and energetic. Another third admitted to being depressed, anxious, tired, and frustrated some of the time; 15.8% were tired all the time, 5.7% were frustrated, and 5.4% were suicidal most/all of the time.
Personal needs summary listed the following: education of medical practitioners about clinical manifestations of AFD, improvement in genetic counselling, access to ERT trials information with implications for treatment in carriers and young males affected with AFD, regular monitoring for heart and kidney problems, and medical management with more understanding and sympathy.
This is the first report of a large cross sectional cohort survey in adult obligate carriers for AFD. Included were all obligate carriers known to the UK AFD register with confirmed pedigree structure and AFD diagnosis. Those under the age of 18 were excluded as published reports and clinical observation suggested mainly adult onset of AFD complications. The data are based on an AFD specific questionnaire survey for self reported signs and symptoms, and on clinical findings in symptomatic AFD carriers seeking medical attention. Asymptomatic women were not investigated in this study, as the present health care policy does not recognise them as patients affected with AFD. The results of abnormal renal and cardiac function reported here are therefore underestimates.
The median cumulative survival in 32 obligate carriers was 70 years, which represents an approximate reduction of 15 years from the general population. A gradual decline of the AFD female carrier curve is evident from the age of 35. These data suggest milder expression of the disease in AFD carriers than in affected males.
SERIOUS MANIFESTATIONS
The frequency of serious AFD manifestations in carriers is unknown. A number of carriers with cerebrovascular complications or with renal failure were reported.1
A review of clinical manifestations in heterozygotes for AFD estimated the frequency of neuropathic pain as <10%.1The accumulation of sphingolipid was determined at necropsy in the spinal and sympathetic ganglia in a carrier with severe neuropathic pains and cardiomyopathy. A significant increase of 34- and 48-fold respectively above normal was found,5 thus explaining the likely aetiology of severe neuropathic pains.
In this cohort, a substantially higher frequency of pain was evident and, as in AFD males, the mean onset of neuropathic pains in carriers was 15 years and in 20% the pain had stopped at the age of 28. The pain scores for both attacks and background pain in carriers were surprisingly high, >5, and suggest that these women require improved pain management. Only 17.1% of carriers were receiving anticonvulsants or opiates. High scores were obtained for the effects of pain on attendance at school, participation in sports, and the impact on family and social life. A third to half of respondents scored the impact of pain on their life as being high. Similarly to affected males, the duration of the neuropathic pain appears to be life long and stops in a small percentage of women.
The frequency of cerebrovascular complications in carriers was estimated as <1%, occurring in rare female variants with 0-5% α-galactosidase activity.1 Neurological manifestations such as TIAs, CVA, tinnitus, severe hypostatic hypotension, and sensorineural hearing loss have been documented as case reports.6 A cohort study of 10 female heterozygotes with cerebrovascular complications reported the mean age of onset as 40.3 years and the signs and symptoms, in descending order of frequency, as: memory loss, dizziness, ataxia, hemiparesis, loss of consciousness, and hemisensory symptoms.7 In another study, the incidence of thrombotic accidents was 10% (n=15), where two carriers had CVA aged 43 and 49 respectively and one had central retinal artery thrombosis at 31.8 The incidence of TIA in carriers is unknown.
In this cohort, a direct question was asked about past history of TIA and a high frequency was found (table 4). Several carriers in this group were in their 60s and 70s, when cerebrovascular events are common and not the result of AFD. However, the mean age for TIA at 52 years indicates that in the majority of these women the onset of TIA was early and most likely the result of AFD pathology. Results from this cohort suggest a higher frequency of cerebrovascular complications than previously reported.
Two unrelated carriers in this cohort had mild idiopathic MR, and both have developed ESRF, aged 35 and 37; further investigations are ongoing (one of these was not included in this study because of failure to return the questionnaire).
The incidence of renal failure in carriers was estimated as <1%, occurring in rare female variants with 0-5% α-galactosidase activity.1 Abnormal renal function, proteinuria, and ESRF were reported in several female carriers.1 Characteristic renal changes of AFD were also described in asymptomatic carriers with normal renal function, when investigated to become kidney donors for their male relatives.9 Two heterozygotes had a mosaic pattern of focal patchy AFD inclusions/degenerative changes among normal appearing glomeruli on electron microscopy.9 The Gb3 accumulation in the kidney was age related and Gb3 content in urine sediment of heterozygotes was increased up to 20-fold compared with normal.1 From these reports to date, we can conclude that probably all carriers accumulate Gb3 in the kidneys and usually at a much slower rate than AFD males. The rate of Gb3 deposition appears variable.
In this cohort, two women (3.3%) developed ESRF at ages 35 and 37 respectively, and so far neither has had a renal transplant. In these two women, the age at onset was comparable to AFD males, but the frequency of ESRF was much lower in carriers than the 26% found in hemizygous males.10 The frequency of abnormal renal function in this cohort could not be determined because of lack of data.
The incidence of cardiac involvement in carriers was estimated as <1%, occurring in rare female variants with 0-5% α-galactosidase activity.1 Cardiomyopathy and MV prolapse were documented in two studies of 13 and 12 carriers, respectively. Sakurabaet al 11 described MV prolapse, dilated aortic root, concentric LVH, and ECG abnormalities in 5/13 women aged 36-64. Only those over 50 years of age had symptoms of palpitations and dyspnoea. Goldman et al 12 found no LVH on echocardiogram in 12 carriers, who were however younger, aged between 25 and 37 years. In both studies, the frequency of MV prolapse was over 50%, with additional patients having redundant MV. Renovascular hypertension was present in 2/13. These cohort data suggest that the onset of cardiac lipid storage is evident around the age of 35 years in carriers and is present in around 50%. Cardiac histopathology in one carrier showed focal granular staining pattern with anti-CTH monoclonal antibody.13 This female developed proteinuria and LVH at 22 years, subsequently had CVA and renal and cardiac failure, and died of ventricular fibrillation aged 60. This report documented a mosaic pattern of myocardial involvement and subsequent multiorgan progression of AFD over 40 years. Further documentation of cardiomyopathy was shown by increased thallium-201 uptake by scintigraphy in three carriers14 and a 53 year old female carrier presented with end stage restrictive cardiomyopathy and underwent heart transplantation.15
In this cohort, chest pain and palpitations were reported by 52.5%, but only a third were investigated. Four carriers had mitral valve replacement aged between 55 and 65 years.
GASTROINTESTINAL MANIFESTATIONS
Gastrointestinal structure and function were investigated in 13 affected males and in 17 heterozygotes.16 Twenty-nine percent of heterozygotes were symptomatic and jejunal biopsy showed characteristic electron dense sphingolipid deposits situated in the neuronal and vascular tissue in the bowel submucosa. These lesions were not visible by light microscopy. The bowel involvement in carriers was less frequent than in males and less pronounced. The onset of symptoms was later than in males, at between 15-35 years, and several girls also had mild growth delay. In this cohort, 58.3% of carriers reported having GI symptoms, which included abdominal pain, diarrhoea, or constipation and vomiting. Most women had extensive GI and gynaecological investigations. None of the carriers in this cohort experienced GI complications in the form of bowel perforation or infarction and the severity of symptoms appeared to progress with age. In none of these women was the cause of GI symptoms identified.
OTHER MANIFESTATIONS OF AFD
Fatigue was the single most common manifestation reported by 66% of carrier women, which interfered with their family and social life. The second commonest manifestation was angiokeratoma and was reported by 35%. This observation is in agreement with the frequency reported previously,1 but discrete patches of angiokeratoma observed in this study became evident only on careful clinical examination. The findings of angiokeratoma in dermatome distribution supports the concept of mosaic X inactivation, reported in X linked disorders, such as incontinentia pigmenti.17 The incidence of hypohidrosis in carriers was estimated as <1%, occurring in rare female variants with 0-5% α-galactosidase activity.1 In this cohort, subjective hypohidrosis was reported by 32.8% of women documenting higher frequency than expected. Recurrent polyarthralgia in the hands and feet, often with pyrexia, occurs frequently at an early age and the diagnosis of rheumatic fever, chronic inflammatory syndrome, or polyarteritis nodosa has been made.18Perivascular inflammation and polyarteritis nodosa-like necrotising vasculitis pathological changes were found in several tissues including the bowel in an AFD carrier.19
In this cohort, severe disabling fibromyalgia was diagnosed in five women aged between 45 and 55 years and one had proximal muscle atrophy. A similar presentation was described in an AFD male, who had characteristic sphingolipid deposits seen on muscle biopsy.20 Large joint pains were a frequent complaint of AFD carriers over the age of 50, but these were not formally assessed.
REPRODUCTIVE DECISIONS
The majority of women understood X linked inheritance and their carrier status was known before pregnancy. Nearly half of women of childbearing age opted against having more children because of AFD. Prenatal diagnosis was used by only half of the women in this cohort, most likely reflecting the dilemma of termination of pregnancy for a disease with late onset of complications.
In this study, obligate female carriers reported multiple AFD manifestations, with high frequency, which was not recognised previously. However, similar findings were recently reported, in cohorts of 12 and 21 female carriers.21 22 The progression of the disease is slower in carrier females, as would be expected in subjects with partial enzyme deficiency. The patchy distribution of cells with lipid deposition in cardiac, renal, and skin tissues confirms random X inactivation even in carriers with severe manifestations of AFD.
We are grateful to the AFD patient support group, to the families for their cooperation and to Transkaryotic Therapies Inc, Boston, USA for financial support of AH. Professor B Winchester at the Enzyme and Molecular Genetics Laboratory, Institute of Child Health, London analysed most of the samples from this cohort.
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text:
Podcasting Resources
Podcasts / By Buzz Intersection Podcasts
Francesca O'Cathain
Title: Important Facets of Family Law Cases Involving Children with Disabilities
Recorded: 7/21/2021
https://buzzintersection.com/wp-content/uploads/2021/08/202107-Family-Law-Cases-Involvin_re-1.mp3
Speaker Bio:
Francesca O'Cathain is a divorce lawyer in Hackensack, NJ at Lesnevich, Marzano-Lesnevich, O'Cathain & O'Cathain, LLC. As a seasoned legal professional, Francesca has been providing quality legal services related to family law for many years. She exclusively helps clients who are dealing with issues like post-judgements, neglect or abuse accusations, support, prenuptial agreements, asset preservation and division, divorce, and child custody.
In addition to her commitment at the law firm, she also works as Of Counsel for Berkman, Bottger, Newman & Rodd, LLP, and serves as Co-Chair for the Legislative Committee of the Family Law Section. She holds prominent positions at the Irish American Bar Association of New York as Treasurer and Founding Member, and Brehon Law Society Member. In the past, Francesca has also been:
Board Member for Court Appointed Special Advocates (CASA) of New Jersey
Graduate of American Bar Assoication Section of Family Law National Institute for Trial Advocacy Institute
Graduate of the American Academy of Matrimonial Lawyers 'Summer Institute' for Training Associates (beginner and advanced programs)
Graduate of Bachelor of Arts at Claremont McKenna College (2001)
Earned Juris Doctorate at University of Connecticut School of Law (2004)
Francesca has not gone unnoticed within her community and beyond for her excellence. She was honored by Irish Voice as one of its Legal 100 (2010), named as a "Top Lawyers in Bergen County" by 201 Magazine (2013), listed as a "New Jersey Rising Stars Super Lawyer" (since 2012 and on), nominated as one of "New Leaders of the Bar" by New Jersey Law Journal (2015), named "Best of Bergen" by Bergen Magazine, and received Award of Merit from the National Center for Missing and Exploited Children (for her work on Hague Convention Cases).
As you can see, Francesca is an experienced family law lawyer, who continues to make a difference in the lives of her clients. As an adoption advocate, she enjoys spending time with her adopted children from China and husband. They reside in Hillsdale, New Jersey. For help with your family law case, call Francesca O'Cathain at Lesnevich, Marzano-Lesnevich, O'Cathain & O'Cathain, LLC today.
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Automation both saves and makes money, while ensuring faster innovation and time-to-market.
In our last several blogs we've looked at best practices in and around quality assurance and automated business process testing.
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A recent survey conducted by IDC of large customers quantified the significant benefits delivered by automation for global enterprises. The analyst group found that organizations saw an ROI of 537% and broke even in just less than 12 months. This return earned businesses an average of $13.59 million over five years, and accelerated innovation, as mentioned earlier.
How is this ROI achieved? The secret is automation – and the elimination of slow, incomplete, error-prone, and costly manual labor. With automation, fewer defects enter production and staff productivity is not lost on manual testing. In fact, studies found that Worksoft automation saves more than 50,000 hours per year for many firms.
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With the stresses put on businesses in today's market, the financial benefits of automated business process validation are extremely compelling, but the total benefits—faster innovation and high-quality execution—offer even more strategic value than its exceptional ROI.
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"When I was stationed at a field in Cassino, on the Arno River, a few of us would get a fisherman to carry us across the river on his boat to a small cafe, when we got tired of C rations. We would carry something to cook, like potatoes, french fries, and meat, and when it came time to go back across, we would whistle, and the fisherman would come back down the river and carry us across."
"When the war was over, we were living in a two story building within the town of Cassino. We didn't have a bathroom or toilet, so, we dug a hole close to the street, with enough lumber for the hole, but no walls. I was sitting on one, when a woman stopped and was trying to tell me something in Italian. When she kept grabbing my shirt, I finally understood that she wanted to do my laundry."