Saturday, May 31, 2014

Duchenne drug 'nears approval' in EU

24 May 2014 Last updated at 01:55 Pills A new drug could be used to treat children in the UK with Duchenne in 2015 A drug to treat a particular form of Duchenne muscular dystrophy has been given the green light by the European Medicines Agency and could be available in the UK in six months.

Translarna is only relevant to patients with a 'nonsense mutation', who make up 10-15% of those affected by Duchenne.

The EMA decided not to pass the drug in January, but they have since re-examined the evidence.

A campaign group said the drug must reach the right children without delay.

There are currently no approved therapies available for this life-threatening condition.

The patients who will benefit the most are those aged five years and over who are still able to walk, the EMA said.

Duchenne muscular dystrophy is a genetic disease that gradually causes weakness and loss of muscle function.

Patients with the condition lack normal dystrophin, a protein found in muscles, which helps to protect muscles from injury.

In patients with the disease, the muscles become damaged and eventually stop working.

There are 2,400 children in the UK living with muscular dystrophy, but only those whose condition is caused by a particular 'nonsense mutation' - namely 200 children - are suitable to use Translarna.

Urgency in UK

The drug, ataluren, will be known by the brand name of Translarna in the EU. It was developed by PTC Therapeutics.

The next step will see the European Commission rubberstamp the EMA's scientific 'green light' within the next three months and authorise the drug to be marketed in the European Union.

At that point, individual member states, including the UK, must decide how it will be funded.

The Muscular Dystrophy Campaign is calling for urgent meetings with National Institute of Health of Clinical Excellence (NICE) and NHS England to discuss how Translarna can be cleared for approval and use in the UK.

It said families in the UK could have access to the drug by spring 2015.

Robert Meadowcroft, chief executive of the campaign, said: "This decision by the EMA is fantastic news.

"Most of those diagnosed with Duchenne muscular dystrophy, usually before the age of five, will use a powered wheelchair before they are 12, will not have the muscle strength to pick up a glass of water by the age of 20 and will not live to see their 30th birthday.

"This step forward for Translarna offers much needed hope."

He added that extending the numbers of patients taking Translarna would help to accelerate the development of the drug.

Clinical trials are still ongoing on the drug and the results will have to be sent to the EMA.


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Deadly attack on Djibouti restaurant

24 May 2014 Last updated at 22:16 Map locator At least two people have been killed in an attack on a restaurant popular with Westerners in Djibouti, police say.

Grenades were thrown at La Chaumiere restaurant in the capital of the Horn of Africa country, officials said. At least another 11 people were wounded.

Unconfirmed reports said that two employees had been killed and several foreigners wounded.

No group has said it carried out the attack and the motive was not immediately clear.

Colonel Omar Hassan, head of police in Djibouti City, told Reuters: "It's a criminal act. We have two people dead and 11 wounded. It was grenades."

Djibouti's ADI news agency reported several explosions around 20:00 local time (17:00 GMT) and said that the area was quickly sealed off by police.

Djibouti, a former French colony, is home to US and French military bases and also contributes troops to the African Union force fighting al-Shabab militants in Somalia.

Its port is also used by foreign navies protecting the Gulf of Aden's shipping lanes from Somali pirates.


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VIDEO: Film Review - the week's new films

Film critic Mark Kermode reviews the week's film releases, including X-Men: Days Of Future Past, Heli and Fading Gigolo.


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Pope praises Jordan as tour begins

24 May 2014 Last updated at 16:13 The Pope greets reporters on his plane to the Middle East, including the BBC's Jeremy Bowen

Pope Francis has praised Jordan for its "generous welcome" to Syrian refugees at the start of a three-day visit to the Middle East.

The Pope was welcomed by King Abdullah II and in a speech at the royal palace, he stressed the need for an "urgent" solution to the Syrian conflict.

He went on to celebrate Mass at a stadium in the capital Amman and will later meet Syrian refugees.

The tour's official purpose is to improve ties with the Orthodox Church.

However, correspondents say many will expect Pope Francis to use his influence to try to ease tensions in the Middle East.

His highlighting of Jordan's role in accepting Syrian refugees came weeks after the kingdom opened a new camp which could accommodate up to 130,000 refugees. Jordan is already host to almost 600,000 people fleeing the conflict, according to the UN.

In his speech, he also called for more respect for religious freedom, calling it "a fundamental human right".

"I cannot fail to express my hope that it will be upheld throughout the Middle East and the entire world," he said. Christian minorities have been subject to increasing attacks in some parts of the Middle East in recent years.

He thanked Jordan for its "efforts to seek lasting peace for the entire region" and said this would also require a "just solution to the Israeli-Palestinian conflict".

On Sunday he is to begin two days in the West Bank and Israel.

He told journalists that the trip would be "challenging" but rewarding, AP reports.

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By BBC Middle East Editor Jeremy Bowen, travelling with Pope Francis

Pope Francis comes across as an approachable, amiable man. Not long after takeoff he came to greet the 50 or so journalists travelling with him. They're mostly reporters who follow the Pope full-time. One woman said that Pope Benedict, Francis's predecessor, never came back to talk to journalists. She asked him to pose for a selfie, which he did with a smile.

He told me he had been in Jerusalem only once before, in 1973 at the time of the Yom Kippur war. The Pope insists his trip is about religion, officially marking the fiftieth anniversary of a visit by Pope Paul VI 50 years ago. But he's heading for Jerusalem, where everything is political. It will take diplomatic skill to avoid controversy.

Palestinians hope he will have something to say about their desire for independence. In Bethlehem, which is in the Israeli-occupied West Bank, Palestinians say they are threatened by the encroachment of Jewish settlements. The Israeli government will hope he stays as neutral as possible - a position which would disappoint Palestinian Christians.

line Restraining orders

The Pope will be accompanied by a rabbi and an imam - friends from his native Argentina - and hopes to improve relations between Christians, Muslims and Jews in the Holy Land.

His journey comes only a few weeks after the latest round of Israeli-Palestinian peace talks collapsed.

Israel has issued restraining orders against several Jewish right-wing activists this week over concerns that they could try to disrupt the visit.

Police said offensive "anti-Christian graffiti" was discovered on the wall of a church in the southern city of Beersheba on Friday.

A general view of Manger Square as workers prepare for the visit of Pope Francis to the West Bank town of Bethlehem on 23 May 2014 Preparations were well under way at Manger Square in Bethlehem ahead of the Pope's visit

The Pope's journey marks the 50th anniversary of the historic meeting in Jerusalem between Pope Paul VI and the head of the Orthodox Church, Patriarch Athenagoras.

The meeting ended 900 years of separation and enduring antagonism between the Eastern and Western branches of Christianity.

On Sunday, Pope Francis will travel to Bethlehem in the West Bank and preside over Mass in Manger Square, near the site where Jesus is believed to have been born.

He will also meet the current Ecumenical Orthodox Patriarch, Bartholomew, and they will sign a declaration of friendship.

His schedule on Monday is set to include a visit to the al-Aqsa mosque complex in Jerusalem's Old City followed by the Dome of the Rock and the Western Wall.

Pope Francis will be the fourth leader of the Roman Catholic Church to visit Jerusalem, after Popes Paul VI, John Paul II and Benedict XVI, who went there in 2009.


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NI21's McKenzie quitting politics

24 May 2014 Last updated at 09:17 Ms McKenzie resigned from the party executive on Thursday night Tina McKenzie is quitting active politics NI21's European election candidate Tina McKenzie is quitting politics.

The party is in disarray, amid calls on its leader Basil McCrea to step aside while allegations of sexual impropriety are investigated.

Mr McCrea has denied the claims that emerged on polling day.

Ms McKenzie told the Irish News she was "disillusioned and no longer interested in politics".

She confirmed to the BBC that while she would remain an NI21 member and still supported its concept, she wanted to step back from active politics to concentrate on her family and business.

While the European election result will not be known until Monday, Ms McKenzie also stood in the council election but was eliminated in the Balmoral district electoral area, having only received 256 first-preference votes.

NI21 was set up by MLAs Basil McCrea and John McCallister, after they quit the Ulster Unionist Party in 2013.

Just before polling day on Thursday, the two men had a very public falling-out.

The party announced on Tuesday that it was dropping its unionist designation at Stormont to describe itself as "other" - a decision described by Mr McCallister on the front page of the following day's News Letter as "crazy" and "dysfunctional".


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Friday, May 30, 2014

VIDEO: Should more UK pupils learn Mandarin?

Thousands of pupils across England, Wales and Northern Ireland are due to sit their Mandarin GCSE exams on Friday.

The number of students make up only fraction of those studying European languages and many argue that is a wasted opportunity which could be costing our economy billions.

The BBC's China editor Carrie Gracie reports.


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Miliband: Labour 'can win in 2015'

24 May 2014 Last updated at 23:07 Ed Miliband Ed Miliband has said his party's local election performance shows it can win at Westminster in 2015.

The Labour leader insisted the party was ahead in its target seats.

The claim came despite complaints from some Labour figures about him personally, and its local election campaign.

While it won more than 330 extra councillors in Thursday's poll, projections suggested Labour only secured a 2% lead over its Tory rivals.

Mr Miliband is due to visit Thurrock where his party lost control of the council after a surge in support for UKIP.

He had hailed the Essex town as evidence Labour was "winning back trust" when it took the town hall last year.

But it was left with no one party in control after UKIP took two Labour and three Tory seats on Thursday.

'Desire for change'

The Thurrock constituency is one of Labour's top target seats for 2015.

Polling data compiled by Conservative peer Lord Ashcroft on the voting intentions of 26,000 people in 26 Conservative-Labour battlegrounds suggested there could be an average 6.5% swing from the Conservatives to Labour in these marginal seats.

This would be enough to oust 83 Conservative MPs and secure Labour a healthy Commons majority.

Mr Miliband said he was determined to attract voters "from every walk of life".

"The local elections show Labour can win because it is our party which is winning where it matters in dozens of our target seats for the next election," he said.

"From Cambridge to Redbridge, from Crawley to Amber Valley, people are electing Labour councils to meet their desire for change."


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