HIV used to cure 'bubble boy' disease – BBC News

HIV used to cure ‘bubble boy’ disease – BBC News

HIV used to cure ‘bubble boy’ disease 17 April 2019 These are external links and will open in a new window Close share panel Image copyright Courtesy of St Jude Children’s Research Hospital Image caption Gael, a patient at St Jude Children’s Research Hospital who received treatment, with his mother US scientists say they used HIV to make a gene therapy that cured eight infants of severe combined immunodeficiency, or “bubble boy” disease.
Results of the research, developed at a Tennessee hospital, were published in the New England Journal of Medicine.
The babies, born with little to no immune protection, now have fully functional immune systems.
Untreated babies with this disorder have to live in completely sterile conditions and tend to die as infants.
The gene therapy involved collecting the babies’ bone marrow and correcting the genetic defect in their DNA soon after their birth. Image copyright SPL Image caption David Vetter, who came to be known in the 1970s as the bubble boy
The “correct” gene – used to fix the defect – was inserted into an altered version of one of HIV, the virus that causes AIDS.
Researchers said most of the babies were discharged from the hospital within one month.
Dr Ewelina Mamcarz of St Jude, an author of the study, said in a statement : “These patients are toddlers now, who are responding to vaccinations and have immune systems to make all immune cells they need for protection from infections as they explore the world and live normal lives.”
“This is a first for patients with SCID-X1,” she added, referring to the most common type of SCID.
The patients were treated at St Jude Children’s Research Hospital in Memphis and at UCSF Benioff Children’s Hospital in San Francisco.

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‘Giant lion’ fossil found in Kenya museum drawer – BBC News

Image copyright AFP A new species of giant mammal has been identified after researchers investigated bones that had been kept for decades in a Kenyan museum drawer.
The species, dubbed “Simbakubwa kutokaafrika” meaning “big African lion” in Swahili, roamed east Africa about 20 millions years ago.
But the huge creature was part of a now extinct group of mammals called hyaenodonts.
The discovery could help explain what happened to the group.
Hyaenodonts – so called because their teeth resemble those of a modern hyena – were dominant carnivores more than 20 million years ago, National Geographic reports.
But they are not related to hyenas.
Africa Live: More on this and other stories How a shepherd found a dinosaur graveyard Lost history of African dinosaurs revealed “Based on its massive teeth, Simbakubwa was a specialised hyper-carnivore that was significantly larger than the modern lion and possibly larger than a polar bear,” researcher Matthew Borths is Quote: d by AFP news agency as saying.
In 2013 he was doing research at the Nairobi National Museum when he asked to look at the contents of a collection labelled “hyenas”, National Geographic says.
Image copyright Matthew Borths/ Nancy Stevens The creature’s jaw and other bones and teeth had been put there after being found at a dig in western Kenya in the late 1970s.
Mr Borths teamed up with another researcher, Nancy Stevens, and in 2017 they began analysing the unusual fossil specimens.
Their findings were reported in the Journal of Vertebrate Paleontology this week.

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St Patrick’s Cathedral: Man arrested carrying petrol – BBC News

Image copyright AFP/Getty Image caption St Patrick’s is a Catholic cathedral in Manhattan built in the 19th Century A man has been arrested after walking into New York’s St Patrick’s Cathedral carrying two full petrol cans, lighter fluid and lighters, police say.
They say guards confronted the 37-year-old as he entered the Manhattan church on Wednesday evening.
He spilt gasoline on the ground and officers took him into custody.
Deputy police commissioner John Miller noted that the “suspicious” incident occurred just two days after a fire gutted Notre-Dame cathedral in Paris.
St Patrick’s is the seat of New York’s Roman Catholic archdiocese.
“An individual walking into an iconic location like St Patrick’s cathedral carrying over four gallons of gasoline, two bottles of lighter fluid and lighters, is something we would have grave concern over,” Mr Miller told reporters.
The NYPD deputy commissioner of intelligence and counterterrorism however said it was “too early to say” whether terrorism was a motive.
How Notre-Dame became the heart of France Notre-Dame: The story of the fire in graphics When confronted outside the cathedral, the man told officers his vehicle had run out of fuel and he was cutting through the cathedral to get to it. He was arrested when police checked the van and saw it was not out of petrol.
“We don’t know what his mindset was,” Mr Miller said.
Investigators in Paris say renovation works at Notre-Dame could have accidentally sparked Monday’s fire.
The disaster has led to a surge in fundraising for black churches destroyed by an arsonist in the US earlier this year.
A 21-year-old accused of burning down three African-American churches in Louisiana was on Tuesday charged with hate crimes.

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Notre-Dame before the fire in 360° video – BBC News

Just months before Notre-Dame was severely damaged by fire , a French camera team filmed the iconic cathedral from both inside and out.
Revisit the famous landmark in its former glory with our 360° video above.
Courtesy Forum des images – TV5Monde. Original footage directed by Raphael Beaugrand .
BBC VR Producer: Stephen Beckett. Watch more

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Climate change: Sir David Attenborough warns of ‘catastrophe’ – BBC News

Warning from ‘Antarctica’s last forests’
Sir David’s new programme laid out the science behind climate change, the impact it is having right now and the steps that can be taken to fight it.
“In the 20 years since I first started talking about the impact of climate change on our world, conditions have changed far faster than I ever imagined,” Sir David stated in the film.
“It may sound frightening, but the scientific evidence is that if we have not taken dramatic action within the next decade, we could face irreversible damage to the natural world and the collapse of our societies.”
Speaking to a range of scientists, the programme highlighted that temperatures are rising quickly, with the world now around 1C warmer than before the industrial revolution.
“There are dips and troughs and there are some years that are not as warm as other years,” said Dr Peter Stott from the Met Office.
“But what we have seen is the steady and unremitting temperature trend. Twenty of the warmest years on record have all occurred in the last 22 years.”
The programme showed dramatic scenes of people escaping from wildfires in the US, as a father and son narrowly escape with their lives when they drive into an inferno.
Scientists say that the dry conditions that make wildfires so deadly are increasing as the planet heats up. Image copyright Getty Images Image caption Greenland is losing ice five times as fast as it was 25 years ago
Some of the other impacts highlighted by scientists are irreversible.
“In the last year we’ve had a global assessment of ice losses from Antarctica and Greenland and they tell us that things are worse than we’d expected,” said Prof Andrew Shepherd from the University of Leeds.
“The Greenland ice sheet is melting, it’s lost four trillion tonnes of ice and it’s losing five times as much ice today as it was 25 years ago.”
These losses are driving up sea levels around the world. The programme highlights the threat posed by rising waters to people living on the Isle de Jean Charles in Louisiana, forcing them from their homes.
“In the US, Louisiana is on the front line of this climate crisis. It’s losing land at one of the fastest rates on the planet – at the rate of of a football field every 45 minutes,” said Colette Pichon Battle, a director of the Gulf Coast Center for Law & Policy. Image copyright Julie Dermansky Image caption People are moving from parts of Louisiana in the US as a result of rising waters
“The impact on families is going to be something I don’t think we could ever prepare for.”
Sir David’s concern over the impacts of climate change has become a major focus for the naturalist in recent years.
This has also been a theme of his Our Planet series on Netflix. His new BBC programme has a strong emphasis on hope.
Sir David argues that if dramatic action is taken over the next decade then the world can keep temperatures from rising more than 1.5C this century. This would limit the scale of the damage.
“We are running out of time, but there is still hope,” said Sir David.
“I believe that if we better understand the threat we face the more likely it is we can avoid such a catastrophic future.”
The programme said that rapid progress is being made in renewable energy, with wind now as cheap as fossil fuels in many cases. It shows how technologies to remove and bury carbon dioxide under the ground are now becoming more viable.
But politicians will need to act decisively and rapidly.
“This is the brave political decision that needs to be taken,” said Chris Stark from the UK’s Committee on Climate Change. Image copyright Getty Images Image caption Teenage campaigner Greta Thunberg has helped spark school strikes all over the world
“Do we incur a small but not insignificant cost now, or do we wait and see the need to adapt. The economics are really clear on this, the costs of action are dwarfed by the costs of inaction.”
The programme also highlights the rising generation of young people who are deeply concerned about what’s happening to the planet.
Swedish teenager Greta Thunberg explained that things can change quickly, despite the scale of the challenge on climate change.
“The first day I sat all alone,” she said, speaking of her decision to go on strike from school and sit outside the Swedish parliament to highlight the climate crisis.
“But on the second day, people started joining me… I wouldn’t have imagined in my wildest dreams that this would have happened so fast.”
“Change is coming whether you like it or not.”
Follow Matt on Twitter @mattmcgrathbbc
Climate Change – The Facts was on BBC One on Thursday 18 April at 9pm and is available on iPlayer.

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