The research aims to produce valid information and must use reliable instruments that guarantee accurate and make it quantifiable and possible reproducibility. Allowing the exclusion or at least control prejudice of personal insights and trends that may distort the results.
sábado, 25 de febrero de 2012
NASA Urges Vigilance For Weird Fireballs
The strange deep-diving, slow-moving fireballs started falling on Feb. 1.
They range in size from basketballs to buses and some are thought to have dropped meteorites.
Astronomers know the objects originate in the asteroid belt, but little else is known.
A fireball over north Georgia recorded on Feb. 13 by a NASA all-sky camera in Walker County, Georgia. Click to enlarge this image.
A strange breed of fireball is streaking through the skies this month, and NASA is urging folks on the ground to take notice.
February's fireballs -- a term that describes meteors that appear brighter in the sky than Venus -- aren't more numerous than normal, but their appearance and trajectory are odd, experts say.
"These fireballs are particularly slow and penetrating," meteor expert Peter Brown, a physics professor at the University of Western Ontario, said in a statement. "They hit the top of the atmosphere moving slower than 15 kilometers per second (33,500 mph), decelerate rapidly and make it to within 50 kilometers (31 miles) of Earth’s surface."
Beginning February With a Bang
The month's fireball action began on Feb. 1, when a meteor lit up the skies over central Texas, putting on a dazzling show for people in the Dallas-Fort Worth area.
"It was brighter and long-lasting than anything I've seen before," said eyewitness Daryn Morran. "The fireball took about eight seconds to cross the sky. I could see the fireball start to slow down; then it exploded like a firecracker artillery shell into several pieces, flickered a few more times and then slowly burned out."
The fireball was about as bright as the full moon, and was spotted by NASA cameras in New Mexico, more than 500 miles (805 km) away. It was likely caused by an object 3 to 6 feet (1 to 2 meters) wide, NASA researchers said.
And the meteors have kept coming, well into February.
"This month, some big space rocks have been hitting Earth's atmosphere," said Bill Cooke of the Meteoroid Environment Office at NASA's Marshall Space Flight Center in Huntsville, Ala. "There have been five or six notable fireballs that might have dropped meteorites around the United States."
So far in February, NASA's All-Sky Fireball Network -- which currently consists of six cameras set up in Georgia, Alabama, Tennessee and New Mexico -- has photographed about half a dozen of these strange slow-moving, deep-diving fireballs. They have ranged in size from basketballs to buses.
Cooke has analyzed their orbits and determined where the strange meteors are coming from.
"They all hail from the asteroid belt, but not from a single location in the asteroid belt," he said. "There is no common source for these fireballs, which is puzzling."
The "fireballs of February" have puzzled astronomers for decades. Skywatchers first noticed an increase in the number of deep-penetrating, bright meteors during February back in the 1960s and '70s, Brown said.
Research to date has been inconclusive, with some studies reporting a surge of these fireballs in February and others detecting no such trend, Brown added.
But NASA's All-Sky Fireball Network could end up solving the mystery. Cooke and his colleagues plan to keep adding cameras to the network, increasing its coverage across North America.
"The beauty of our smart multi-camera system," Cooke said, "is that it measures orbits almost instantly. We know right away when a fireball flurry is underway -- and we can tell where the meteoroids came from."
While Cooke and other researchers ponder the origins of February's fireballs, the rest of us still have a few days left to enjoy them this year.
"If the cows and dogs start raising a ruckus tonight," Cooke said, "go out and take a look.
Publicado por Nathaniel en 10:35 No hay comentarios:
Isolated Peru tribe makes uncomfortable contact
'Uncontacted' Indians involved in violent encounters; authorities try to keep outsiders away
Image: Mashco-Piro tribe
D. Cortijo / UncontactedTribes.org via AP
This November 2011 image, made available by Survival International on Tuesday, shows members of the Mashco-Piro tribe who were photographed at an undisclosed location near Manu National Park in southeastern Peru. According to Survival International, the image is one of the closest sightings of isolated Amazon Indians ever recorded with a camera.
By Frank Bajak
LIMA, Peru — Peruvian authorities say they are struggling to keep outsiders away from a clan of previously isolated Amazon Indians who began appearing on the banks of a jungle river popular with environmental tourists last year.
The behavior of the small group of Mashco-Piro Indians has puzzled scientists, who say the encounters may be related to the encroachment of loggers and by low-flying aircraft from nearby natural gas and oil exploration in the southeastern region of the country.
Clan members have been blamed for two bow-and-arrow attacks on people near the riverbank in Madre de Dios state, where officials say the Indians were first seen last May.
One badly wounded a forest ranger in October. The following month, another fatally pierced the heart of a local Matsiguenka Indian, Nicolas "Shaco" Flores, who had long maintained a relationship with the Mashco-Piro.
The advocacy group Survival International released photos Tuesday showing clan members on the riverbank, describing the pictures as the "most detailed sightings of uncontacted Indians ever recorded on camera."
The British-based group provided the photos exactly a year after releasing aerial photos from Brazil of another tribe classified as uncontacted, one of about 100 such groups it says exist around the world.
Pictures from birdwatcher and archaeologist
One of the Mashco-Piro photos was taken by a birdwatcher in August, Survival International said. The other two were shot by Spanish archaeologist Diego Cortijo on Nov. 16, six days before Flores was killed.
Cortijo, a member of the Spanish Geographical Society, was visiting Flores while on an expedition in search of petroglyphs and said clan members appeared across the river from Flores' house, calling for him by name.
Flores could communicate with the Mashco-Piro because he spoke two related dialects, said Cortijo, who added that Flores had previously provided clan members with machetes and cooking pots.
The Mashco-Piro tribe is believed to number in the hundreds and lives in the Manu National Park that borders Diamante, a community of more than 200 people where Flores lived.
Less isolated habitat
Although it's not known what provoked the Mashco-Piro clan to leave the relative safety of their tribe's jungle home, Beatriz Huerta, an anthropologist who works with Peru's agency for indigenous affairs, speculated their habitat is becoming increasingly less isolated.
The upper Madre de Dios region where the tribe lives has been affected by logging, she said. "They are removing wood very close."
Meanwhile, Huerta said, naturalists in the area and Manu National Park officials told her during a recent visit that a rise in air traffic related to natural gas and oil exploration in the region is adversely affecting native hunting grounds, forcing increasing migration by nomadic tribes.
The clan that showed up at the river is believed to number about 60, including some 25 adults, said Carlos Soria, a professor at Lima's Catholic University who ran Peru's park protection agency last year.
"It seemed like they wanted to draw a bit of attention, which is a bit strange because I know that on other occasions they had attacked people," Cortijo said by phone from Spain. "It seemed they didn't want us to go near them, but I also know that the only thing that they wanted was machetes and cooking pots."
Cortijo said the group lingered by the river a few minutes, apparently to see if a boat would pass by so they could ask for some tools, something authorities say they had done in the past.
"The place where they are seen is one of heavy transit" of river cargo and tourist passage, and so the potential for more violent encounters remains high, Soria said.
The situation is compounded by culture clash. The Mashco-Piro live by their own social code, which Soria said includes the practice of kidnapping other tribes' women and children.
He said the Mashco-Piro are one of about 15 "uncontacted" tribes in Peru that together are estimated to number between 12,000 and 15,000 people living in jungles east of the Andes.
"The situation is incredibly delicate," said Huerta, the government anthropologist.
"It's very clear that they don't want people there," she said of the area where the clan has been loitering, noting that it had ransacked a jungle ranger's post that authorities later removed.
One of the clan's likely fears is being decimated by disease borne by outsiders, as has occurred with other uncontacted peoples, Huerta said.
But it's also a mystery why they have appeared in an area so heavily trafficked, she added.
After the first sightings, and after tourists left clothing for the Mashco-Piro, state authorities issued a directive in August barring all boats from going ashore in the area. But enforcing it has been difficult as there are few trained and willing local officials.
Authorities say they aren't sure why Flores was killed. It could be that the Mashco-Piro were angry because he hadn't provided them with more machetes and cooking pots. Or perhaps it was because they considered the farming plot where he was killed too close to what they considered their territory.
Cortijo, the Spanish archaeologist, said the loss of Flores makes reaching any understanding with the Mashco-Piro very complicated.
"The problem is that 'Shaco' was the only person who could talk to them," he said. "Now that he's dead it's impossible to make contact."
Publicado por Nathaniel en 10:33 No hay comentarios:
Mount Etna's first eruption of the year
Mt. Etna erupted for the first time in the new year. As the cloud of smoke rose into the sky, the sun was also rising behind me, painting beautiful, vibrant colors on the smoke cloud, almost giving an illusion of fire in the sky. I created this HDR image by shooting three different exposures. It seemed to be the only way I could capture the colors I was seeing.
Publicado por Nathaniel en 10:32 No hay comentarios:
Etiquetas: photo story
Fold-up car of the future unveiled for Europe
A tiny revolutionary fold-up car designed in Spain's Basque country as the answer to urban stress and pollution was unveiled Tuesday before hitting European cities in 2013.
The "Hiriko," the Basque word for "urban," is an electric two-seater with no doors whose motor is located in the wheels and which folds up like a child's collapsible buggy, or stroller, for easy parking.
Dreamt up by Boston's MIT-Media lab, the concept was developed by a consortium of seven small Basque firms under the name Hiriko Driving Mobility, with a prototype unveiled by European Commission president Jose Manuel Barroso.
Demonstrating for journalists, Barroso clambered in through the fold-up front windscreen of the 1.5-metre-long car.
"European ideas usually are developed in the United States. This time an American idea is being made in Europe," consortium spokesman Gorka Espiau told AFP.
Its makers are in talks with a number of European cities to assemble the tiny cars that can run 120 kilometers (75 miles) without a recharge and whose speed is electronically set to respect city limits.
They envision it as a city-owned vehicle, up for hire like the fleets of bicycles available in many European cities, or put up for sale privately at around 12,500 euros.
Several cities have shows interest, including Berlin, Barcelona, San Francisco and Hong Kong. Talks are under way with Paris, London, Boston, Dubai and Brussels.
The vehicle's four wheels turn at right angles to facilitate sideways parking in tight spaces.
The backers describe the "Hiriko" project as a "European social innovation initiative offering a systematic solution to major societal challenges: urban transportation, pollution and job creation."
Publicado por Nathaniel en 10:31 No hay comentarios:
Steve Jobs brought back to life as an Android fan in Taiwanese ad
Taiwan's Action Pad uses Steve Jobs look-alike to drum up support, but has it backfired?
In the months following long-time Apple CEO Steve Jobs's untimely death, we've seen him resurrected in many forms. He's already been brought back in plastic and bronze, but we never thought we'd see him reborn as an Android supporter. Leave it to a Taiwanese tablet maker to dream up such a thing, and recreate an iconic Apple keynote presentation in the process.
The makers of the Taiwanese Action Pad tablet went to great lengths to honor (or mock?) the world-famous Apple co-founder by dressing a Taiwanese lookalike in Jobs's popular black turtleneck and Levi's blue jeans. For good measure, the Jobs-alike is even equipped with a heavenly halo and angel wings.
The fake CEO trots about a stage that looks very much like the ones Apple uses for its major product announcements, but it's not an iPad in his hands — it's the overseas knockoff. The ad boasts that the Action Pad is "the new generation of the pad," and shows off various app icons ranging from an RSS reader to the recognizable Android mascot.
In a move that you'd never see the real Steve Jobs make, the angelic speaker touts the benefits of the Android 2.3 operating system that comes standard on the Action Pad, and even goes so far as to say "Thank God I can finally play another pad."
While it might seem less than tasteful, the ad doesn't actually use Jobs's name or any copyrighted material that could land the Action Pad's manufacturers in legal trouble. However, that hasn't stopped many commenters from lambasting the parody on video hosting sites where it appears. Even diehard Android fans are coming out against the clip, calling it "very disturbing," and "extremely disrespectful."
The tablet itself is nothing particularly special: It appears to be wifi-only with no 3G or 4G mobile connection options, and its Android 2.3 operating system is somewhat outdated as nearly all new Android slates come with a much newer version. Whether the off-color commercial will help the Action Pad gain traction remains to be seen, but with many calling for a boycott of the company as a whole, it might prove more of a curse
Publicado por Nathaniel en 10:30 No hay comentarios:
See nano quadcopter robots swarm
CNET.com – 16 hrs ago
In the future, a swarm of flying robots may do the work now done by human search teams.
A robotics research team at the University of Pennsylvania has designed a system to coordinate a number of small quadcopters, a step toward coordinating multiple robots for tasks such as surveillance or searching areas after a disaster.
The General Robotics, Automation, Sensing, and Perception (GRASP) lab at UPenn yesterday posted a video on You Tube with nano quadcopters showing remarkable agility and the ability to perform as a team.
The quadcopters are able to flip over and maintain flight. More amazing (unnerving?) is their operation in formation. Based on commands, 16 quadcopters change direction, land, navigate past obstacles, and even fly in a figure-eight formation.
Coordinating the action of multiple robots is one of the big technical challenges in robotics research now. Small robots, such as these nano quadcopters, could be well-suited for certain missions, but people need methods for programming small, inexpensive robots in large groups rather than manually configuring each one.
Publicado por Nathaniel en 10:29 No hay comentarios:
"Monsignors' mutiny" revealed by Vatican leaks
VATICAN CITY (Reuters) - Call it Conspiracy City. Call it Scandal City. Call it Leak City. These days the holy city has been in the news for anything but holy reasons.
"It is a total mess," said one high-ranking Vatican official who spoke, like all others, on the condition of anonymity.
The Machiavellian maneuvering and machinations that have come to light in the Vatican recently are worthy of a novel about a sinister power struggle at a medieval court.
Senior church officials interviewed this month said almost daily embarrassments that have put the Vatican on the defensive could force Pope Benedict to act to clean up the image of its administration - at a time when the church faces a deeper crisis of authority and relevance in the wider world.
Some of those sources said the outcome of a power struggle inside the Holy See may even have a longer-term effect, on the choice of the man to succeed Benedict when he dies.
From leaked letters by an archbishop who was transferred after he blew the whistle on what he saw as a web of corruption and cronyism, to a leaked poison pen memo which puts a number of cardinals in a bad light, to new suspicions about its bank, Vatican spokesmen have had their work cut out responding.
The flurry of leaks has come at an embarrassing time - just before a usually joyful ceremony this week known as a consistory, when Benedict will admit more prelates into the College of Cardinals, the exclusive men's club that will one day pick the next Roman Catholic leader from among their own ranks.
"This consistory will be taking place in an atmosphere that is certainly not very glorious or exalting," said one bishop with direct knowledge of Vatican affairs.
The sources agreed that the leaks were part of an internal campaign - a sort of "mutiny of the monsignors" - against the pope's right-hand man, Secretary of State Cardinal Tarcisio Bertone.
Bertone, 77, has a reputation as a heavy-handed administrator and power-broker whose style has alienated many in the Curia, the bureaucracy that runs the central administration of the 1.3 billion-strong Roman Catholic Church.
He came to the job, traditionally occupied by a career diplomat, in 2006 with no experience of working in the church's diplomatic corps, which manages its international relations. Benedict chose him, rather, because he had worked under the future pontiff, then Cardinal Joseph Ratzinger, in the Vatican's powerful doctrinal office.
"It's all aimed at Bertone," said a monsignor in a key Vatican department who sympathizes with the secretary of state and who sees the leakers as determined to oust him. "It's very clear that they want to get rid of Bertone."
Vatican sources say the rebels have the tacit backing of a former secretary of state, Cardinal Angelo Sodano, an influential power-broker in his own right and a veteran diplomat who served under the late Pope John Paul II for 15 years.
"The diplomatic wing feels that they are the rightful owners of the Vatican," the monsignor who favors Bertone said.
Sodano and Bertone are not mutual admirers, to put it mildly. Neither has commented publicly on the reports.
The Vatican has been no stranger to controversy in recent years, when uproar over its handling of child sex abuse charges has hampered the church's efforts to stem the erosion of congregations and priestly recruitment in the developed world.
But the latest image crisis could not be closer to home.
It began last month when an Italian television investigative show broadcast private letters to Bertone and the pope from Archbishop Carlo Maria Vigano, the former deputy governor of the Vatican City and currently the Vatican ambassador in Washington.
The letters, which the Vatican has confirmed are authentic, showed that Vigano was transferred after he exposed what he argued was a web of corruption, nepotism and cronyism linked to the awarding of contracts to contractors at inflated prices.
As deputy governor of the Vatican City for two years from 2009 to 2011, Vigano was the number two official in a department responsible for maintaining the tiny city-state's gardens, buildings, streets, museums and other infrastructure, which are managed separately from the Italian capital which surrounds it.
In one letter, Vigano writes of a smear campaign against him by other Vatican officials who were upset that he had taken drastic steps to clean up the purchasing procedures and begged to stay in the job to finish what he had started.
Bertone responded by removing Vigano from his position three years before the end of his tenure and sending him to the United States, despite his strong resistance.
Other leaks center on the Vatican bank, just as it is trying to put behind it past scandals - including the collapse 30 years ago of Banco Ambrosiano, which entangled it in lurid allegations about money-laundering, freemasons, mafiosi and the mysterious death of Ambrosiano chairman Roberto Calvi - "God's banker."
Today, the Vatican bank, formally known at the Institute for Works of Religion (IOR), is aiming to comply fully with international norms and has applied for the Vatican's inclusion on the European Commission's approved "white list" of states that meet EU standards for total financial transparency.
Bertone was instrumental in putting the bank's current executives in place and any lingering suspicion about it reflects badly on him. The Commission will decide in June and failure to make the list would be an embarrassment for Bertone.
Last week, an Italian newspaper that has published some of the leaks ran a bizarre internal Vatican memo that involved one cardinal complaining about another cardinal who spoke about a possible assassination attempt against the pope within 12 months and openly speculated on who the next pope should be.
Bertone's detractors say he has packed the Curia with Italian friends. Some see an attempt to influence the election of the next pope and increase the chances that the papacy returns to Italy after two successive non-Italian popes who have broken what had been an Italian monopoly for over 450 years.
Seven of the 18 new "cardinal electors" -- those aged under 80 eligible to elect a pope -- at this Saturday's consistory are Italian. Six of those work for Bertone in the Curia.
Bertone, as chief administrator, had a key role in advising the pope on the appointments, which raised eyebrows because of the high number of Italian bureaucrats among them.
"There is widespread malaise and delusion about Bertone inside the Curia. It is full of complaints," said the bishop who has close knowledge of Vatican affairs.
"Bertone has had a very brash method of running the Vatican and putting his friends in high places. People could not take it any more and said 'enough' and that is why I think these leaks are coming out now to make him look bad," he said.
Leaked confidential cables sent to the State Department by the U.S. embassy to the Vatican depicted him as a "yes man" with no diplomatic experience or linguistic skills and the 2009 cable suggests that the pope is protected from bad news.
"There is also the question of who, if anyone, brings dissenting views to the pope's attention," read the cable, published by WikiLeaks.
The Vatican sources said some cardinals asked the pope to replace Bertone because of administrative lapses, including the failure to warn the pope that a renegade bishop re-admitted to the Church in 2009 was a well-known Holocaust denier.
But they said the pope, at 84 and increasingly showing the signs of his age, is not eager to break in a new right-hand man.
"It's so complicated and the pope is so helpless," said the monsignor.
The bishop said: "The pope is very isolated. He lives in his own world and some say the information he receives is filtered. He is interested in his books and his sermons but he is not very interested in government."
Publicado por Nathaniel en 10:28 No hay comentarios:
Drones: Who Is Watching You?
Military drones are being used by everyone from real estate agents to paparazzi. The military technology is now in the hands of police, the media — and maybe even your neighbors.
When it comes to privacy – we’re screwed. According to the Los Angeles Times – unmanned Predator drones aren’t just for hunting terrorists in the Middle East anymore – they’re being used to hunt American citizens too. Three men were arrested in North Dakota on suspicions they stole cattle from a nearby farm – and they were located thanks to the help of a Predator drone conducting surveillance above. Local police are now saying they’ve used Predator drones to run at least two dozen surveillance flights in North Dakota alone since June.
Although Congress authorized the drones to be used domestically in 2005 to patrol our Northern and Southern borders – their use for local law enforcement is increasing – with virtually no input by the American people. And privacy experts warn continued use of drones radically threatens the privacy of all of us. As Ryan Calo – the director of privacy and robotics at Stanford University – said, “Any time you have a tool like that in the hands of law enforcement that makes it easier to do surveillance, they will do more of it." The rise of the military industrial complex – and the militarization of our police are nearly complete. Drones aren't just for Afghanistan, Pakistan, Yemen, or Somalia anymore—they're coming to the good old US of A. The Senate sent a bill to President Obama yesterday that would require the Federal Aviation Administration to come up with rules to safely regulate domestic drones, USA Today reports. Civilian demand is already starting to heat up for various flying robots, which now come in a host of shapes and sizes, are surprisingly cost effective, and would occupy the same airspace as passenger planes.
Publicado por Nathaniel en 10:27 No hay comentarios:
Egypt begins restoring ancient boat near pyramids
CAIRO (AP) — Archaeologists on Monday began restoration on a 4,500-year-old wooden boat found next to the pyramids, one of Egypt's main tourist attractions.
The boat is one of two that were buried next to the Pharaoh Khufu, spokesmen for a joint Egyptian-Japanese team of archeologists said. The boats are believed to have been intended to carry pharaohs into the afterlife.
Khufu, also known as Cheops, is credited with building the Great Pyramid of Giza, the largest of the pyramids. Khufu, son of Snefru, was the second ruler of the 4th Dynasty around 2680 B.C. and ruled Egypt for 23 years.
Both boats, made from Lebanese cedar and Egyptian acacia trees, were originally discovered in 1954. One of the boats is on display at a museum near the pyramids.
The second boat, which is now undergoing the restoration, remained buried. It is thought to be smaller than its sister ship, which is about 140 feet (43 meters) long.
The head of Egypt's Supreme Council of Antiquities, Mustafa Amin, said Egyptologists began taking samples of the wood for restoration on Monday.
"The boat was found in a complete shape, intact and in place," he said, adding that the focus now is on taking samples of the wood.
He said Egyptologists are studying "the different components and fungus in the wood in order to find the most sufficient and advanced way to work on the wood."
Last year in June, a team of scientists lifted the first of 41 limestone slabs each weighing about 16 tons to uncover the pit in which the ancient ship was buried, said Sakuji Yoshimura, professor from Japan's Waseda University.
At the time, experts said restoration would likely take about four years and that at its completion, the boat would be placed on display at the Solar Boat Museum near the pyramids, which routinely attract millions of tourists and boost one of Egypt's most important industries.
The team had initially thought the vessel would be safer left underground than exposed to pollution, but evidence showed that pollution, water and insects had invaded the boat's chamber.
A $10 million grant from Waseda University has helped in preparing the ship's excavation process.
Publicado por Nathaniel en 10:25 No hay comentarios:
Blue People of Troublesome Creek
Benjamin "Benjy" Stacy so frightened maternity doctors with the color of his skin -- "as Blue as Lake Louise" -- that he was rushed just hours after his birth in 1975 to University of Kentucky Medical Center.
As a transfusion was being readied, the baby's grandmother suggested to doctors that he looked like the "blue Fugates of Troublesome Creek." Relatives described the boy's great-grandmother Luna Fugate as "blue all over," and "the bluest woman I ever saw."
In an unusual story that involves both genetics and geography, an entire family from isolated Appalachia was tinged blue. Their ancestral line began six generations earlier with a French orphan, Martin Fugate, who settled in Eastern Kentucky.
Doctors don't see much of the rare blood disorder today, because mountain people have dispersed and the family gene pool is much more diverse.
But the Fugates' story still offers a window into a medical mystery that was solved through modern genetics and the sleuth-like energy of Dr. Madison Cawein III, a hematologist at the University of Kentucky's Lexington Medical Clinic.
Cawein died in 1985, but his family charts and blood samples led to a sharper understanding of the recessive diseases that only surface if both parents carry a defective gene.
The most detailed account, "Blue People of Troublesome Creek," was published in 1982 by the University of Indiana's Cathy Trost, who described Benjy's skin as "almost purple."
The Fugate progeny had a genetic condition called methemoglobinemia, which was passed down through a recessive gene and blossomed through intermarriage.
"It's a fascinating story," said Dr. Ayalew Tefferi, a hematologist from Minnesota's Mayo Clinic. "It also exemplifies the intersection between disease and society, and the danger of misinformation and stigmatization."
Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin -- a form of hemoglobin -- is produced, according to the National Institutes for Health. Hemoglobin is responsible for distributing oxygen to the body and without oxygen, the heart, brain and muscles can die.
In methemoglobinemia, the hemoglobin is unable to carry oxygen and it also makes it difficult for unaffected hemoglobin to release oxygen effectively to body tissues. Patients' lips are purple, the skin looks blue and the blood is "chocolate colored" because it is not oxygenated, according to Tefferi.
"You almost never see a patient with it today," he said. "It's a disease that one learns about in medical school and it is infrequent enough to be on every exam in hematology."
The disorder can be inherited, as was the case with the Fugate family, or caused by exposure to certain drugs and chemicals such as anesthetic drugs like benzocaine and xylocaine. The carcinogen benzene and nitrites used as meat additives can also be culprits, as well as certain antibiotics, including dapsone and chloroquine.
The genetic form of methemoglobinemia is caused by one of several genetic defects, according to Tefferi. The Fugates probably had a deficiency in the enzyme called cytochrome-b5 methemoglobin reductase, which is responsible for recessive congenital methemoglobinemia.
Normally, people have less than about 1 percent of methemoglobin, a type of hemoglobin that is altered by being oxidized so is useless in carrying oxygen in the blood. When those levels rise to greater than 20 percent, heart abnormalities and seizures and even death can occur.
But at levels of between 10 and 20 percent a person can develop blue skin without any other symptoms. Most of blue Fugates never suffered any health effects and lived into their 80s and 90s.
"If you are between 1 percent and 10 percent, no one knows you have an abnormal level and this might be the case in a lot of unsuspecting patients," he said.
Many other recessive gene diseases, such as sickle cell anemia, Tay Sachs and cystic fibrosis can be lethal, he said.
"If I carry a bad recessive gene with a rare abnormality and married, the child probably wouldn't be sick, because it's very rare to meet another person with the [same] bad gene and the most frequent cause therefore is in-breeding," Tefferi said.
Such was the case with the Fugates.
Martin Fugate came to Troublesome Creek from France in 1820 and family folklore says he was blue. He married Elizabeth Smith, who also carried the recessive gene. Of their seven children, four were reported to be blue.
There were no railroads and few roads outside the region, so the community remained small and isolated. The Fugates married other Fugate cousins and families who lived nearby, with names like Combs, Smith, Ritchie and Stacy.
Benjy's father, Alva Stacy showed Trost his family tree and remarked, "If you'll notice -- I'm kin to myself," according to Trost.
One of Martin and Elizabeth Fugate's blue boys, Zachariah, married his mother's sister. One of their sons, Levy, married a Ritchie girl and had eight children, one of them Luna. Luna married John E. Stacy and they had 13 children.
Benjy descended from the Stacy line.
Modern Fugates Still in Kentucky
ABCNews.com was unable to determine if Benjamin Stacy is still alive -- he would be 37 today. Trost writes that he eventually lost the blue tint to his skin, but as a child his lips and fingernails still got blue when he was angry or cold.
His mother Hilda Stacy, who is 56, appears to still live in Hazard, Ky., but did not answer calls to her home. Other relatives are scattered throughout Virginia and Arkansas.
Most of what scientists know about the family was discovered by Cawein, the grandson of Kentucky's poet laureate, who had done pioneering research on L-dopa as a treatment for Parkinson's disease.
Later in 1965 he was famous for another reason. His wife was murdered by chemical poisoning, but no one was ever indicted.
Cawein heard rumors about the Fugates while working at his Lexington clinic and set off "tromping around the hills looking for blue people," according to Trost's account.
At an American Heart Association clinic in the town of Hazard, Cawein found a nurse, Ruth Pendergrass, and she was willing to assist. She remembered a dark blue woman who had come to the county health department on a frigid afternoon seeking a blood test.
"Her face and her fingernails were almost indigo blue," she told Trost. "It like scared me to death. She looked like she was having a heart attack. I just knew that patient was going to die right there in the health department, but she wasn't a'tall alarmed. She told me that her family was the blue Combses who lived up on Ball Creek. She was a sister to one of the Fugate women."
More families were found -- Luke Combs, and Patrick and Rachel Ritchie, who were "bluer'n hell" and embarrassed by their skin color.
Cawein and Pendergrass began to ask questions -- "Do you have any relatives who are blue?" -- and mapped a family tree and took blood samples.
The doctor suspected methemoglobinemia and uncovered a 1960 report in the Journal of Clinical Investigation. Dr. E. M. Scott, who worked in public health at the Arctic Research Center in Anchorage, had seen a recessive genetic trait among Alaskans that turned their skin blue.
That suggested an inbred line that had been passed from generation to generation. To get the disorder, a person would have to inherit two genes -- one from each parent. When both parents have the trait, their children have a 25 percent chance of getting the disorder.
Scott speculated these people lacked the enzyme diaphorase in their red blood cells. Normally diaphorase converts methemoglobin back to hemoglobin.
All of the blue Fugates he tested had the enzyme deficiency, just like the Alaskans Scott had observed.
Their blood had accumulated so much of the blue molecule that it over-powered the red hemoglobin that normally turns skin pink in most Caucasians.
The bluest of the bunch was Luna, and she lived a healthy life, bearing 13 children before she died at the age of 84.
As coal mining arrived in Kentucky in 1912 and the Fugates moved outside of Troublesome Creek, the blue people began to disappear.
Doctors say Benjy likely carried only one gene for methemoglobinemia, because he eventually had normal skin tones, and the likelihood of him marrying a woman with the same recessive gene would have been small.
By the time reports appeared in the media on the disorder, the Stacy family was upset with insinuations about in-breeding that fed into stereotypes of backwoods Appalachia.
"There was a pain not seen in lab tests," wrote Trost. "That was the pain of being blue in a world that is mostly shades of white to black."
Publicado por Nathaniel en 10:24 No hay comentarios:
Rare monster lobster caught on East Coast
BOOTHBAY HARBOR — A giant 27-pound lobster caught off the Maine coast will be released back into the wild, said Maine State Aquarium Director Aimee Hayden-Rodriques on Wednesday.
click image to enlarge
Maine State Aquarium Director Aimee Hayden-Rodriques holds a 27 pound, nearly 40 inch long, lobster caught by Robert Malone off the coast of Maine near Rockland on Feb. 17. The aquarium named the crustacean "Rocky."
AP Photo/Maine State Aquarium
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The 27-pound lobster is displayed at the Maine State Aquarium.
Courtesy photo of the Maine State Aquarium
Publicado por Nathaniel en 10:21 No hay comentarios:
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