The deadly VEXAS syndrome is more common than doctors thought

The deadly VEXAS syndrome is more common than doctors thought

Older men are more likely than women to get VEXAS syndrome, a genetic disease that arises later in life.

Thomas Barwick/Stone/Getty Images Plus

A mysterious new disease may be to blame for severe, unexplained inflammation in older men. Now, researchers have their first good look at who the disease strikes, and how often.

VEXAS syndrome, an illness discovered just two years ago, affects nearly 1 in 4,000 men over 50 years old , scientists estimate January 24 in JAMA . The disease also occurs in older women, though less frequently. Altogether, more than 15,000 people in the United States may be suffering from the syndrome, says study coauthor David Beck, a clinical geneticist at NYU Langone Health in New York City. Those numbers indicate that physicians should be on the lookout for VEXAS, Beck says. “It’s underrecognized and underdiagnosed. A lot of physicians aren’t yet aware of it.”

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Beck’s team reported discovering VEXAS syndrome in 2020, linking mutations in a gene called UBA1 to a suite of symptoms including fever, low blood cell count and inflammation. His team’s new study is the first to estimate how often VEXAS occurs in the general population — and the results are surprising. “It’s more prevalent than we suspected,” says Emma Groarke, a hematologist at the National Institutes of Health in Bethesda, Md., who was not involved with the study.

People with the disease may experience an assortment of symptoms across the body, including:

VEXAS tends to show up later in life ­­— after people somehow acquire UBA1 mutations in their blood cells. Patients may feel overwhelming fatigue, lethargy and have skin rashes, Beck says. “The disease is progressive, and it’s severe.” VEXAS can also be deadly. Once a person’s symptoms begin, the median survival time is about 10 years , his team has found.

Until late 2020, no one knew that there was a genetic thread connecting VEXAS syndrome’s otherwise unexplained symptoms. In fact, individuals may be diagnosed with other conditions, including polyarteritis nodosa, an inflammatory blood disease, and relapsing polychondritis, a connective tissue disorder, before being diagnosed with VEXAS.

To ballpark the number of VEXAS-affected individuals, Beck’s team combed through electronic health records of more than 160,000 people in Pennsylvania, in a collaboration with the NIH and Geisinger Health. In people over 50, the disease-causing UBA1 mutations showed up in roughly 1 in 4,000 men. Among women in that age bracket, about 1 in 26,000 had the mutations.  

A genetic test of the blood can help doctors diagnose VEXAS, and treatments like steroids and other immunosuppressive drugs, which tamp down inflammation, can ease symptoms. Groarke and her NIH colleagues have also started a small phase II clinical trial testing bone marrow transplants as a way to swap patients’ diseased blood cells for healthy ones.

Beck says he hopes to raise awareness about the disease, though he recognizes that there’s much more work to do. In his team’s study, for instance, the vast majority of participants were white Pennsylvanians, so scientists don’t know how the disease affects other populations. Researchers also don’t know what spurs the blood cell mutations, nor how they spark an inflammatory frenzy in the body.

“The more patients that are diagnosed, the more we’ll learn about the disease,” Beck says. “This is just one step in the process of finding more effective therapies.”

D.B. Beck et al . Estimated prevalence and clinical manifestations of UBA1 variants associated with VEXAS syndrome in a clinical population . JAMA . Vol. 329, January 24/31, 2023, p. 318. doi: 10.1001/jama.2022.24836.

M.A. Ferrada et al . Translation of cytoplasmic UBA1 contributes to VEXAS syndrome pathogenesis . Blood . Vol. 140, September 29, 2022, p. 1496. doi: 10.1182/blood.2022016985.

D.B. Beck et al . Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease . The New England Journal of Medicine . Vol. 383, December 31, 2020, p. 2628. doi: 10.1056/NEJMoa2026834.

Meghan Rosen is a staff writer who reports on the life sciences for Science News . She earned a Ph.D. in biochemistry and molecular biology with an emphasis in biotechnology from the University of California, Davis, and later graduated from the science communication program at UC Santa Cruz.

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The deadly VEXAS syndrome is more common than doctors thought

Here are 7 new science museums and exhibitions to visit in 2023

Here are 7 new science museums and exhibitions to visit in 2023

Visitors to the Grand Egyptian Museum will be greeted by a giant, 3,200-year-old statue of King Ramses II (shown here in 2019 during the museum’s construction).

KHALED DESOUKI/AFP VIA GETTY IMAGES

If you’re a museum aficionado itching for a new place to explore, 2023 has you covered. New science museums and exhibitions are opening, and some zoos are expanding. This sampling of destinations to check out in the new year or beyond has something for everyone, whether you’re a wildlife lover, space nerd or history buff.

Grand Egyptian Museum
Outside Cairo
Opens: To be announced

2022 marked the 100th anniversary of the discovery of King Tut’s tomb ( SN: 11/19/22, p. 14 ). Now, thousands of artifacts from the tomb — along with tens of thousands of other archaeological finds from ancient Egypt — will go on display when this museum, located within view of the Pyramids of Giza, opens. More than a decade in the making, it will be one of the largest archaeological museums in the world.

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Richard Gilder Center for Science, Education and Innovation
American Museum of Natural History
New York City
Opens: February 17

This multistory building will add tons of new exhibit space to the more than 150-year-old museum. Visitors can explore an insectarium that includes one of the world’s largest displays of live leaf-cutting ants and come face-to-face with dozens of butterfly species in a vivarium. Meanwhile, the interconnectedness of life will be on display in the immersive, 360-degree “Invisible Worlds” exhibition.

Galápagos Islands
Houston Zoo
Opens: April 2023

If you can’t travel to the Galápagos Islands, a trip to Texas might be the next best thing. Giant tortoises, iguanas, penguins, sea lions, sharks and other creatures will inhabit this new exhibition that will re-create the land and marine ecosystems of the archipelago made famous by Charles Darwin.

Kansas City Zoo Aquarium
Opens: September 2023

The 34 exhibits of this new aquarium will allow visitors to glimpse a wide variety of ocean locales without having to leave the Midwest. Underwater residents will include sea urchins and sea anemones in a warm intertidal zone, fish swimming in a coral reef, comb jellies floating in the open ocean and sea otters playing along a rocky shore.

SPACE
Franklin Institute
Philadelphia
Opens: Fall 2023

To design this new two-story gallery dedicated to the future of space exploration, exhibit planners met with local students and teachers to find out what they wanted to learn. The result is an experience that, among other things, will showcase the current and future technologies needed to live and work in space as well as the many career paths into the aerospace industry.

Bird House
Smithsonian’s National Zoo
Washington, D.C.
Opens: To be announced

With a focus on bird migration and conservation in the Americas, the zoo’s new bird house will feature three aviaries: The first will show how the Delaware Bay is a key refueling spot for migratory shorebirds, the second will demonstrate how seasonal wetlands in the Midwest serve waterfowl and the third will illustrate how a tropical coffee farm can provide respite for songbirds in winter.

Robot & AI Museum
Seoul, South Korea
Opens: To be announced

Though details are still scant, this museum dedicated to furthering public knowledge of robotics, artificial intelligence and machine learning is expected to open later this year.

This story was updated February 1, 2023, to correct that the Galápagos Islands exhibit opens in April 2023 (not September).

Erin Wayman is the magazine managing editor. She has a master’s degree in biological anthropology from the University of California, Davis and a master’s degree in science writing from Johns Hopkins University.

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Here are 7 new science museums and exhibitions to visit in 2023

76 percent of well-known insects fall outside protected areas

76 percent of well-known insects fall outside protected areas

Protected areas can provide safe havens for insect species, including the Australian painted lady ( Vanessa kershawi , shown). But many existing ones fall short, a new study finds.

S. Chowdhury

The existing boundaries of national parks and other habitat preserves aren’t enough to protect more than three-quarters of the world’s well-studied insects .

The finding, reported February 1 in One Earth , shows that people who design nature preserves “don’t really think about insects that much,” says coauthor Shawan Chowdhury, an ecologist at the German Centre for Integrative Biodiversity Research in Leipzig.

That’s a problem because insect populations around the globe are plummeting , a growing body of research suggests, probably due to climate change and human development ( SN: 4/26/22 ). For instance, insect abundance in Puerto Rico has dropped by up to 98 percent over the last 35 years. 

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Threats to insect survival could have ripple effects on plants and other animals. Insects help form the foundation for many ecosystems: They pollinate around 80 percent of all plant species and serve as a staple in the diets of hundreds of thousands of animals (and the occasional carnivorous plant).

One way to avert insect extinctions is to set aside the land they need to survive. But scientists know the ranges for only about 100,000 of the estimated 5.5 million insect species. To determine how well existing protected areas may be aiding insect conservation, Chowdhury and colleagues mapped the known habitats of about 89,000 of those species and compared the ranges with the boundaries of preserves from the World Database on Protected Areas .

Overall, these spaces don’t safeguard enough habitat for 67,384 insect species — about 76 percent of the species included in the study — the team found. Roughly 2 percent of species do not overlap with protected areas at all.

Conserving insects, Chowdhury says, will mean setting aside more insect-friendly spaces in the years ahead.

S. Chowdhury et al . Three quarters of insect species are insufficiently represented by protected areas . One Earth . Published online February 1, 2023. doi: 10.1016/j.oneear.2022.12.003.

Freda Kreier was a fall 2021 intern at Science News . She holds a bachelor’s degree in molecular biology from Colorado College and a master’s in science communication from the University of California, Santa Cruz.

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76 percent of well-known insects fall outside protected areas

Chemical residue reveals ancient Egyptians’ mummy-making mixtures

Chemical residue reveals ancient Egyptians’ mummy-making mixtures

Vessels from an ancient Egyptian embalming workshop, including these, provided scientists with clues to the ingredients used in various mixtures for preparing the dead for mummification.

Saqqara Saite Tombs Project/Univ. of Tübingen, M. Abdelghaffar

Scientists have unwrapped long-sought details of embalming practices that ancient Egyptians used to preserve dead bodies.

Clues came from analyses of chemical residue inside vessels from the only known Egyptian embalming workshop and nearby burial chambers. Mummification specialists who worked there concocted specific mixtures to embalm the head, wash the body, treat the liver and stomach, and prepare bandages that swathed the body , researchers report February 1 in Nature .

“Ancient Egyptian embalmers had extensive chemical knowledge and knew what substances to put on the skin to preserve it, even without knowing about bacteria and other microorganisms,” Philipp Stockhammer, an archaeologist at Ludwig Maximilians University of Munich, said at a January 31 news conference.

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The findings come courtesy of chemical residue inside 31 vessels found in an Egyptian embalming workshop and four vessels discovered in an adjacent pair of burial chambers. Writing on workshop vessels named embalming substances, provided embalming instructions (such as “to put on his head”) or both. All the artifacts — dating from Egypt’s 26th dynasty which rose to power between 664 B.C. and 525 B.C. — were excavated at a cemetery site called Saqqara in 2016. Archaeologist and study coauthor Ramadan Hussein, who died in 2022, led that project.

Five of the vessels had the label antiu . The substance was thought to have been a fragrant resin called myrrh. The antiu at Saqqara, however, consisted of oil or tar from cedar and juniper or cypress trees mixed with animal fats. Writing on these jars indicates that antiu could have been used alone or combined with another substance called sefet .

Three vessels from the embalming workshop bore the label sefet , which researchers have usually described as an unidentified oil. At Saqqara, sefet was a scented, fat-based ointment with added ingredients from plants. Two sefet pots contained animal fats mixed with oil or tar from juniper or cypress trees. A third container held animal fats and elemi, a fragrant resin from tropical trees.

Clarification of the ingredients in antiu and sefet at Saqqara “takes mummification studies further than before,” says Egyptologist Bob Brier of Long Island University in Brookville, N.Y., who was not part of the research.

Egyptians may have started mummifying their dead as early as 6,330 years ago ( SN: 8/18/14 ). Mummification procedures and rituals focused on keeping the body fresh so the deceased could enter what was believed to be an eternal afterlife.

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Embalming and mummification procedures likely changed over time, says team member Maxime Rageot, a biomolecular archaeologist also at Ludwig Maximilians University. Embalmers’ mixtures at Saqqara may not correspond, say, to those used around 700 years earlier for King Tutankhamun ( SN: 11/2/22 ).

Outside surfaces of other vessels from the Saqqara embalming workshop and burial chambers sported labels and, in some cases, instructions for treatment of the head, preparation of linen mummy bandages, washing the body and treating the liver and stomach. Inscriptions on one jar referred to an administrator who performed embalming procedures, mainly on the head.

Chemical residue inside these pots consisted of mixtures specific to each embalming procedure. Ingredients included oils or tars of cedar and juniper or cypress trees, pistachio resin, castor oil, animal fats, heated beeswax, bitumen (a dense, oily substance), elemi and a resin called dammar.

Most of those substances have been identified in earlier studies of chemical residues from Egyptian mummies and embalming vessels in individual tombs, says Egyptologist Margaret Serpico of University College London. But elemi and dammar resins have not previously been linked to ancient Egyptian embalming practices and are “highly unexpected,” notes Serpico, who did not participate in the new study.

Elemi was an ingredient in the workshop mixtures used to treat the head, the liver and bandages wrapped around the body. Chemical signs of dammar appeared in a vessel from one of the burial chambers that included remnants of a range of substances, indicating that the container had been used to blend several different mixtures, the researchers say.

Specific properties of elemi and dammar that aided in preserving dead bodies have yet to be investigated, Stockhammer said.

Elemi resin reached Egypt from tropical parts of Africa or Southeast Asia, while dammar originated in Southeast Asia or Indonesia, Rageot says. Other embalming substances detected at Saqqara came from Southwest Asia and parts of southern Europe and northern Africa bordering the Mediterranean Sea. These findings provide the first evidence that ancient Egyptian embalmers depended on substances transported across vast trade networks.

Egyptian embalmers at Saqqara took advantage of a trade network that already connected Egypt to sites in Southeast Asia, Stockhammer said. Other Mediterranean and Asian societies also engaged in long-distance trade during ancient Egypt’s heyday ( SN: 1/9/23 ).

It’s no surprise that ancient Egyptians imported embalming ingredients from distant lands, Brier says. “They were great traders, had limited [local] wood products and really wanted these substances to achieve immortality.”

M. Rageot et al . Biomolecular analyses enable new insights into ancient Egyptian embalming . Nature . Published February 1, 2023. doi: 10.1038/s41586-022-05663-4.

Bruce Bower has written about the behavioral sciences for Science News since 1984. He writes about psychology, anthropology, archaeology and mental health issues.

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Chemical residue reveals ancient Egyptians' mummy-making mixtures

Vikings brought animals to England as early as the year 873

Vikings brought animals to England as early as the year 873

By analyzing cremated animal and human bones (shown), researchers say they have found the first solid scientific evidence of animals traveling with Vikings across the North Sea to England.

Julian Richards/University of York

Vikings brought horses and dogs to the British Isles from Scandinavia, a new study suggests.

A chemical analysis of bone fragments from a cemetery in England provides the first solid scientific evidence of animals traveling with Vikings across the North Sea , scientists report February 1 in PLOS ONE.

In the 1990s, researchers unearthed the cremated remains of a human adult and child as well as of a dog, horse and probable pig from a burial mound in a Viking cemetery in Derbyshire, England. In previous work, radiocarbon dating of femur, skull and rib fragments revealed that the inhabitants all died sometime between the eighth and 10th centuries. That date was narrowed down to the year 873, thanks to the ninth-century Anglo-Saxon Chronicle, which records that a Viking army wintered near the site that year.

Where the animals came from has been a mystery. Norse raiders are known to have stolen horses from people in England around the time. And researchers have generally thought that Viking boats at the time were too small to allow for much transport of animals from Scandinavia to the British Isles. One entry in the Anglo-Saxon Chronicle describes Vikings moving from France to England along with their horses in the year 892, but no physical evidence of such activity had been found before.

In the new work, Tessi Löffelmann and colleagues turned to certain forms, or isotopes, of strontium to unravel the individuals’ provenance. The element accumulates in bones over time through diet, leaving a distinct signature of where an individual has lived ( SN: 4/2/19 ).

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Strontium ratios in the child’s remains matched those of shrubs growing at the burial site, suggesting the child spent most, if not all, of its life in England. The ratios of the adult and three animals, on the other hand, differed substantially from the local fauna, the team found. That suggests the individuals hadn’t spent much time in the country before they died. Instead, their ratios were similar to ones found in the Baltic Shield region in Norway, central and northern Sweden and Finland, suggesting a Scandinavian origin.

“One of the joys of isotope analysis is that you are able to really pinpoint things that previously we could discuss endlessly,” says Marianne Moen, an archeologist at the University of Oslo who was not involved in the study. Using strontium to analyze more cremated remains, which can elude common forms of isotope analysis including carbon and nitrogen, “is the next logical chapter for understanding prehistoric mobility.”

Isotope analysis helped reveal where these individuals lived and when they died, but it couldn’t answer why the dog, horse and pig made the journey to England in the first place. That’s where historical records can help, says Löffelmann, of Durham University in England and Vrije Universiteit Brussel in Belgium.

For Löffelmann, the small sizes of early Norse ships combined with the fact that the animals and people were buried together suggest Vikings may have initially brought animals with them for companionship, not just function.

“It could have only been selected animals that made that journey,” she says. “They were important to what the person was.… They went through life together, and now they’re going through death.”

T. Löffelmann et al. Sr analyses from only known Scandinavian cremation cemetery in Britain illuminate early Viking journey with horse and dog across the North Sea . PLOS ONE. Published online February 1, 2023. doi: 10.1371/journal.pone.0280589.

Anna Gibbs was the spring 2022 science writing intern at Science News . She holds a B.A. in English from Harvard College.

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Science News was founded in 1921 as an independent, nonprofit source of accurate information on the latest news of science, medicine and technology. Today, our mission remains the same: to empower people to evaluate the news and the world around them. It is published by the Society for Science, a nonprofit 501(c)(3) membership organization dedicated to public engagement in scientific research and education (EIN 53-0196483).

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Vikings brought animals to England as early as the year 873

Are your cats having fun or fighting? Here are some ways to tell

Are your cats having fun or fighting? Here are some ways to tell

These two cats are interacting in a way that falls somewhere between playing and fighting, according to a new study that could help owners better read their pets’ body language to determine if they are friends or foes.

Westend61/Getty Images Plus

Are your cats play fighting or fighting fur real?

It turns out that certain behaviors in domestic cats could be telltale signs that an interaction is friendly, aggressive or something in between, researchers report January 26 in Scientific Reports .

“It is a question we hear a lot from cat owners,” says cat behavior expert Mikel Delgado of Feline Minds, a cat behavior consulting company in Sacramento, Calif., who was not involved in the study. “So I was excited to see that researchers are taking on this topic.”

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Scientists have studied cats’ social relationships — both with other cats and humans — but it can be tricky to tell whether two cats are playing or fighting, says veterinarian and cat behavior researcher Noema Gajdoš-Kmecová of the University of Veterinary Medicine and Pharmacy in Košice, Slovakia ( SN: 9/23/19 ).

Sometimes cat owners miss the signs of a tense relationship because they think their pets are just playing, which can lead to stress and illness in the animals, she says. Other times, owners rehome their cats after incorrectly assuming their pets are fighting.

To assess and categorize interactions, Gajdoš-Kmecová and colleagues watched about 100 videos of different cats interacting in pairs. After viewing around one-third of the videos, Gajdoš-Kmecová identified six types of behaviors, including wrestling and staying still. She then watched all of the videos and noted how often each cat exhibited one of the specified behaviors, and for how long. By running statistical analyses on the behaviors, she pinpointed three types of interactions between the cat pairs: playful, aggressive and intermediate.

To confirm the outcome, other members of the team also watched the videos and classified each interaction between felines.

Some clear connections emerged. Quietly wrestling, for instance, suggested playtime, whereas chasing and vocalizations, like growling, hissing or gurgling, implied aggressive encounters.

Intermediate interactions had elements of both playful and aggressive encounters, but especially included prolonged activity of one cat toward the other, such as pouncing on or grooming its fellow feline. These in-between encounters could hint that one cat wants to keep playing while the other doesn’t, with the more playful cat gently nudging to see if its partner wants to continue, the authors say.

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This work provides initial insights into cat interactions, Gajdoš-Kmecová says, but it’s just the start. In the future, she plans to study more subtle behaviors, like ear twitches and tail swishes. Both Gajdoš-Kmecová and Delgado also stress that one contentious encounter doesn’t necessarily signal a cat-astrophic relationship.

“This is not just about one interaction,” Gajdoš-Kmecová says. Owners “really should look into the different, multiple interactions in multiple periods of life of the cats and then put it into context.”

N. Gajdoš-Kmecová et al. An ethological analysis of close-contact inter-cat interactions determining if cats are playing, fighting, or something in between . Scientific Reports . Published online January 26, 2023. doi: 10.1038/s41598-022-26121-1.

McKenzie Prillaman is the Spring 2023 science writing intern at  Science News . She holds a bachelor’s degree in neuroscience with a minor in bioethics from the University of Virginia and a master’s degree in science communication from the University of California, Santa Cruz.

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Are your cats having fun or fighting? Here are some ways to tell