Machynlleth school asks 'Has the Old Society Disappeared?' as eight win national Welsh heritage award
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Cambrian News
16-07-2025
- Cambrian News
Machynlleth school asks 'Has the Old Society Disappeared?' as eight win national Welsh heritage award
Individual awards were also given out, including one to student Grace Jarman from Llanidloes High School for her project on the Physicians of Myddfai - herbalists from the old village of Myddfai in Carmarthenshire who are the subject of legends dating back to around the 12th Century.
Daily Mail
10-07-2025
- Daily Mail
Compound in common over-the-counter supplement gives little boy, 8, the ability to walk again
A compound in a common over-the-counter supplement has given an eight-year-old the ability to walk again, in what has been called a 'dream come true'. The eight-year-old, who was not named, used to be the fastest runner in his class and an avid soccer player, regularly enjoying games with friends. But in August 2023, he started to struggle to walk — and within three months was limping, suffering frequent falls and needing a wheelchair. His parents rushed him to doctors, where physicians at NYU Langone diagnosed him with a genetic condition called HPDL deficiency. The disease is rare — with only 90 recorded cases since it was discovered in 2022 — and inhibits the body's ability to make enough of an enzyme called coenzyme Q10 (CoQ10), a compound that plays a vital role in giving cells energy. Taking CoQ10 supplements, available over-the-counter for around 20 cents per pill, can help, but they aren't enough to fuel cells in the brain because the enzyme is too large to cross the blood-brain barrier. Without enough of the enzyme in the brain, sufferers struggle to walk or control their muscles and are at risk of suffering from seizures. So, rather than simply give the little boy a supplement, his doctors said: 'We figured, why not give the cell the building blocks so that the cell can make it itself?' The 'building block' of CoQ10 is called 4-HB and is thin enough to cross the blood-brain barrier. Doctors were able to put this in a powder, which the boy took dissolved in a solution of water. His parents, speaking to STAT News on the condition of anonymity, said: 'Our son's condition dramatically changed in a short period. 'He went from being the fastest runner in his class and an avid soccer player to struggling just to walk, often limping and experiencing frequent falls'. 'It was one of the hardest decisions we've ever made [to try the experimental treatment], but doing nothing felt riskier. We saw how quickly our son was declining and knew we had to act. 'After speaking with doctors and doing our research, we got hope and confidence to step into the unknown.' Within a month of receiving the treatment in November 2023, he was smiling and walking again — completing nearly a mile-long hike across New York City's Central Park. He now regularly enjoys long hikes and sports and has celebrated two birthdays since. The case was revealed in the journal Nature, with researchers now saying they hope it can be used to help others. HPDL deficiency occurs when someone inherits two mutated versions of the human 4-hydroxyphenylpyruvate dioxygenase-like (HPDL) gene, which is used to make the CoQ10 enzyme. In some cases, the condition is very severe and strikes at an early age, causing significant cognitive delays and seizures. In these cases, researchers say patients often do not live beyond 18 months. But it can also strike at later stages in childhood and adolescence, leading otherwise healthy children to suffer muscle weakness and stiffness in the legs. Doctors have linked the condition to abnormal activity in the cerebral cortex — the outermost layer of the brain — which is involved in thought and muscle movement. In this case, the child reportedly suffered no symptoms until he turned eight years old — although doctors said it was likely that there were warning signs. The little boy was initially started on CoQ10 supplements before doctors suggested to the family that he try 4-HB - which is not approved by the FDA. His use of the supplements was then discontinued. Over the first 30 days, he drank the supplement in a 600ml solution, equivalent to nearly three glasses of water, which often led him to throw up afterward. This was then reduced to a 300ml solution, or just over one glass of water. The FDA has not approved CoQ10 or 4-HB as a treatment for any specific disease or condition, although CoQ10 is available as an over-the-counter supplement. The supplement is often recommended to boost energy and fight feelings of fatigue. Doctors say it can also provide support to people with conditions like congestive heart failure and migraines. 4-HB was initially tested on mice in a 2021 study that had HPDL deficiency, with researchers finding it restored the rodents' ability to walk. Its use in the eight-year-old was the first time it's been used for this condition in humans. '[CoQ10] is safe. It's reasonably effective at treating symptoms outside of the brain, but almost completely ineffective at treating symptoms within the brain, because it doesn't get through the blood-brain barrier,' Michael Pacold, an associate professor of radiation oncology at NYU Langone and one of the study authors, told STAT. The suggestion to use the 4-HB building block in the little boy was consented by the parents, the boy and the FDA — which approved its use as a single patient investigational drug, meaning the doctors could use an unapproved drug or substance for a specific patient to treat a specific condition. Based on its success, scientists are calling it a breakthrough in medicine. 'We all dream of this as scientists. And every morning I pinch myself … is this really a dream?' said Pacold. Dr Navdeep Chandel, a medicine expert at Northwestern University who was not involved in the research, added: 'That is basic science is translating into clinical medicine, a dream come true. '[But] here's somebody who took a very basic biochemical approach, and they found the enzyme, and they found what the enzyme does, and they gave that enzyme to people who had genetic mutations. And it made a difference.' Now, Pacold and his team are working on a larger study to test the approach on more children.
The Independent
01-07-2025
- The Independent
Older adults are waiting even longer in emergency departments, study finds
Older Americans are spending more time in emergency rooms than they used to, according to researchers. Boston physicians announced this week that the average length of stay and boarding times for people over the age of 65 had significantly increased between 2017 and 2024. The trends, the doctors note, indicate systemic challenges in hospitals around the country. They also signal an increasingly frustrating and potentially dangerous experience for patients. "Worsening emergency department lengths of stay and boarding contribute to emergency department crowding, reflect systemic health care dysfunction, and, most importantly, harm individual patients," they explained. To reach these conclusions, the doctors used data from 1,633 hospitals and 295 million patients. They analyzed emergency department encounters that were recorded between January 2017 and December 2024. They focused on the proportion of older adults with a length of stay over eight hours and the proportion of admitted patients waiting more than three hours between when they requested a bed and their admission. In 2017, 12 percent of more than 4 million emergency department encounters involved a length of stay over eight hours. By last year, that percentage had surged to 20 percent of more than 12 million encounters. During the same period, encounters involving boarding times over three hours increased from 22 percent of 1.8 million encounters in 2017 to 36 percent of 4.3 million encounters in 2024. The largest increase was reported in academic hospitals, with boarding times over three hours rising from 31 percent in 2017 to 45 percent in 2024. Before Covid, there were small spikes observed in both metrics that were followed by sharper rises during the pandemic's peak. From 2022 to 2024, as more Americans were vaccinated against Covid, both trends slightly declined. The authors suggested that these increases may be driven by several factors, such as increased patient complexity, growing demand, and ongoing staffing and resource shortages. Health care workforce shortages are projected to rise across the U.S. in the coming years, with demand far outpacing supply in some specialties, according to a report from the non-profit National Institute for Health Care Management Foundation. There could be a shortage of as many as 100,000 critical health care workers by 2028, the consulting firm Mercer warned. In recent years, many nurses and doctors have reported feeling burnt out — although burnout rates reportedly aren't as high as they were in the first years of Covid, a study out of Stanford Medicine found. But, emergency room overcrowding is nothing new. A 2022 survey by the American College of Emergency Physicians found that 97 percent of emergency room doctors said they experienced patient boarding times of more than 24 hours, and more than a quarter said patients were forced to stay in the emergency room for more than two weeks before getting a hospital bed. Solutions to this issue aren't yet ironclad. To attempt to fix it, doctors have suggested only treating patients with emergencies, expanding hospital capacity, changing admitting patterns, and making sure patients don't end up in the ER in the first place through enhanced disease prevention. One program started in 2020 set up hospital beds at patients' homes. Without established solutions, the outcomes can be negative for older adults, who make up more than 20 percent of emergency department visits, according to Centers for Disease Control and Prevention data from 2022. "You really don't want an 85-year-old or older patient lingering and sort of stranded in the emergency department for hours and days on end," Dr. Ula Hwang, medical director of geriatric emergency medicine at NYU Langone Health in New York City, told the American Heart Association in May. "It's been shown that if you're an older adult and you board in the emergency department overnight, you are at greater risk for developing delirium, even potentially having an inpatient mortality." The researchers noted that a new measure effective this year limits stays to under eight hours and admissions to within three hours. "Addressing these trends is critical to safeguarding both the health of older adults and the health systems caring for them,' the authors concluded. The findings were announced in a research letter published on Monday in the journal JAMA Internal Medicine.
