A British girl who was told her afro hair was too delicate to donate for wig-making prompted a new wig-making approach to use it, BBC News reports.
When eleven year old Carly Gorton wanted to donate her afro hair to the Little Princess Trust charity, which makes natural hair wigs for children who have lost theirs from cancer treatment and other causes, she was initially frustrated as the charity said the hair was too delicate. Undeterred, Carly had urged the charity to rethink, which it did.
Following research and a trial to make them possible, the charity described the new wigs as a “historic breakthrough”.
“It’s really beautiful,” said Carly, of one of the new wigs.
A BMJ study showed that wigs positively impact psychological wellbeing for people with alopecia, attributed to increasing their confidence of going out in public and the perception of fewer comments about hair loss.
At a special school assembly, Carly’s mother Anna Mudeka then cut her daughter’s hair and it was donated for use in the first new wigs to be worn by other children.
Phil Brace, The Little Princess Trust’s chief executive, said Carly’s “determination” to donate her hair had pushed them to find a solution.
The charity worked with the 120-year-old London company Raoul to develop a wefting method to weave and tie the donated locks.
Carly’s mother, Anna Mudeka, said: “History has been made and we are so proud of Carly.
“Through her sheer determination and everyone pulling together to hear her voice, children of black and mixed heritage can now donate their hair to the Little Princess Trust.”
Ms Mudeka, of Southburgh, added that children needing wigs through illness could now receive a wig “true to their heritage”.
Carly and her mother’s campaign had created a “fundamental change in wig manufacturing”, said Mr Brace. “The commitment and work that has gone on has shown just what is possible when groups of people get together and bring different skills to find a solution.”
US regulators have approved the first new ADHD drug for children in over a decade.
The Food and Drug Administration last week approved viloxazine (Qelbree) for the treatment of attention deficit hyperactivity disorder in children ages 6 to 17. It was developed by Supernus Pharmaceuticals. The drug’s price was undisclosed but is likely to be higher than the generic ADHD pills.
In Europe, viloxazine was sold as an antidepressant for several decades, but never received FDA approval. It was discontinued nearly two decades ago, due to competition from popular pills like Zoloft and Prozac.
ADHD affects about 6 million American children and adolescents. For many, problems include trouble paying attention and completing tasks, fidgeting and impulsiveness.
Earlier ADHD treatments like Ritalin, nearly all of which contain the stimulants amphetamine or methylphenidate, which create the potential for abuse. Viloxazine however is not a stimulant or a controlled substance. It carries a warning of potential for suicidal thoughts and behaviour, which occurred in fewer than 1% of volunteers in studies of the drug.
Qelbree could be an option for children with substance use disorders, who do not cope well with stimulant side effects or who need more therapy, said Dr David W. Goodman, director of Suburban Psychiatric Associates near Baltimore and an assistant professor of psychiatry at Johns Hopkins School of Medicine.
Goodman said that long-acting stimulants prescribed to ADHD patients currently are harder to abuse to get a high than the older fast-acting versions.
In a late-stage study, 477 children ages 6 to 11 took viloxazine for six weeks. Compared to placebo, Inattention and hyperactivity symptoms were reduced by about 50%. Symptom reduction was seen within a week in some participants. Its common side effects include sleepiness, lethargy, decreased appetite and headache.
Supernus is in late-stage testing for adults with ADHD, who represent a small but growing market as adult treatment of the condition expands.
According to a new study by researchers at the Linda Crnic Institute for Down Syndrome, the reason that children with Down syndrome have a drastically elevated risk of leukaemiais due to a more prevalent condition increasing blood stem cell mutation.
Children with Down syndrome are 20-times more likely to develop acute lymphocytic leukaemia (ALL) and 150-times more likely to develop acute myeloid leukaemia (AML) compared to their typical peers. The researchers found that the reason for this is that they are more likely to present with clonal haematopoiesis (CH), a process in which a blood stem cell acquires a genetic mutation that promotes replication.
The findings add to a growing body of evidence linking immune dysregulation to a very different disease spectrum, whereby people with Down syndrome are highly predisposed to certain diseases such as leukaemia and autoimmune disorders, while being highly protected from others, such as solid tumours.
“We found a higher-than expected rate of CH in individuals with Down syndrome between the age of one to 20 years old,” said study author Dr Alexander Liggett, who as a doctoral candidate led the study in the lab of Dr James DeGregori, Professor of Biochemistry and Molecular Genetics. “It is a surprising finding, as the phenomenon is typically only observed in elderly people.”
The researchers used an advanced sequencing technique that they had developed, called FERMI, to blood samples from the Crnic Institute Human Trisome Project Biobank. Mutations were more likely to be detected in Down’s syndrome and also more likely to be oncogenic. In elderly people, oncogenic mutations are commonly found in the genes DNMT3A, TET2, ASXL1, TP53, and JAK2. In people with Down’s syndrome, oncogenic CH was found to be dominated by mutations of the TET2 gene.
“Given the increased risk of leukaemia that accompanies clonal expansion of blood cells carrying oncogenic mutations, these expansions may become an important biomarker of cancer risk in the future,” said Dr Liggett.
The study also found that CH in Down syndrome is associated with immune dysregulation biosignatures linked to diseases co-occurring with Down’s syndrome, including thyroiditis, Alzheimer’s disease, and leukaemia. This discovery opens new avenues in understanding the way CH impacts an array of health outcomes in Down syndrome and how to potentially counteract its effects.
“This is truly transformative. This team has identified a new trait of Down syndrome that has strong implications for understanding the appearance of comorbidities more common in this population, such as leukaemia and premature ageing,” said Dr. Joaquin Espinosa, Executive Director of the Crnic Institute. “The next step is to define the long-term impacts of this precocious clonal hematopoiesis and how to prevent its harmful effects.”
Journal information: Liggett, L.A., et al. (2021) Precocious clonal hematopoiesis in Down syndrome is accompanied by immune dysregulation. Gut. doi.org/10.1182/bloodadvances.2020003858.
Researchers have reported the case of a boy whose brain was able to rewire after a severe stroke that damaged much of his brain.
In the seventh grade, 13-year old Daniel Carr amazed his baseball coach with his ability to throw with his left hand, saying that it was the fastest he’d ever seen. However, he was unable to properly catch with his right hand.
Hearing this from the coach, Kellie Carr, Daniel’s mother, realised that his son had a number of quirks, such as favouring his left side when he was an infant, and his left-handedness emerged well before the normal age of two or three. However, she was unable to get any explanation for this until she met Nico Dosenbach, MD, PhD, who informed her that her son had had a stroke when he was a newborn.
MRI scans revealed large, bilateral voids in Daniel’s brain, but incredibly, he had no cognitive, behavioural or motor problems other than a lack of strength and dexterity in his right arm. “The extent of Daniel’s injuries may be on the edge of what’s compatible with life,” Dosenbach said.
Dainel’s remarkable recovery can be explained by his young age at the time the stroke.
“The brain can compensate more quickly and completely for strokes sustained in early childhood,” he said. “By contrast, large strokes in adults often cause death or severe functional impairment with little chance of recovery. However, the mechanics behind this are only beginning to be understood.”
More MRI scans were done on Daniel’s brain to determine its structure and pathology. Dosenbach and Laumann conducted high-resolution functional MRI scans to understand how Daniel’s brain had reorganised itself. With his mother’s consent, Daniel was further tested over a period of six years, including batteries of neurological tests, and more scans done. Timothy Laumann, MD, PhD, now a fourth-year psychiatry resident at Barnes-Jewish Hospital, had the expertise to analyse the data.
Looking at his medical records, the physician-scientists noted that he had an infection as a newborn, and was hospitalised with an IV drip. However, none of the physicians had suspected a stroke, which happens to one in every 4000 newborns. Daniel was sent home after a week, the doctors having suspected a viral infection.
“The risk of having a pediatric stroke greatly increases with a medical problem, especially an infection during the newborn period,” Dosenbach said. “However, usually there are more obvious signs that a stroke occurred. I can understand how no one suspected it.”
The researchers compared the images of Daniel’s brain to others of young adults, as well as Dosenbach’s own brain, which he had imaged and studied extensively.
“Part of Daniel’s brain structure is gone,” Laumann explained, referring to their analysis of the MRI data. “He’s missing almost a quarter of his cortex.”
The dead tissue was replaced by pockets of cerebrospinal fluid, which acts as a shock absorber, as well as delivering nutrients and removing waste. The surviving neurons formed interconnected islands that restored cognitive and motor functions, and neighbourhoods of healthy tissue were again reconnected.
“Our findings illustrate the brain’s tenacity at reorganizing and recovering functions damaged by a massive stroke affecting both sides of his brain,” Dosenbach said. “Future studies of functional remapping relative to tissue loss may provide additional insights. Our results raise the possibility that variability in outcomes may depend on specific features unique to an individual’s brain.”
Despite the extensive damage, Daniel completed tertiary education and now works as a diesel mechanic.
“His stroke still shocks me,” Kellie Carr said. “How could I have not known? But looking back, maybe it was better that way. I might have babied Daniel and been afraid to let him be a regular kid. Maybe the best thing for him was living normally.”
Daniel agreed: “I think about my right hand daily because I have to constantly think five steps ahead to figure out how to compensate for not being able to use it properly, like I did with the baseball glove. But the last thing I want is for people to act like something is wrong with me. I’m fine.”
Journal information: Timothy O Laumann et al. Brain network reorganisation in an adolescent after bilateral perinatal strokes, The Lancet Neurology (2021). DOI: 10.1016/S1474-4422(21)00062-4
In a new study, lab-made heart valves were shown to grow along with their recipient when implanted into lambs for a year, making a new alternative possible for thousands of paediatric patients who need replacement heart valves.
Researchers from the University of Minnesota Twin Cities’ College of Science and Engineering and the Medical School published the results in Science Translational Medicine. The production procedure for the valves has also been patented and licensed to the University of Minnesota startup company Vascudyne, Inc.
Compared to currently used animal-derived valves, these new valves also showed reduced calcification and improved blood flow when tested in the same growing lamb model. Current solutions for children involve prosthetic valves, but these calcify over time and cannot grow with the patient. This requires up to five open-heart surgeries to replace them as the children grow towards adulthood, involving considerable risk and expense, as well as demanding lifelong anticoagulation therapy.
“This is a huge step forward in paediatric heart research,” commented senior researcher Robert Tranquillo, a University of Minnesota professor in the Departments of Biomedical Engineering and the Department of Chemical Engineering and Materials Science. “This is the first demonstration that a valve implanted into a large animal model, in our case a lamb, can grow with the animal into adulthood. We have a way to go yet, but this puts us much farther down the path to future clinical trials in children. We are excited and optimistic about the possibility of this actually becoming a reality in years to come.”
Using a combination of tissue engineering and regenerative medicine, they were able to grow the heart valves. Implementing a tissue engineering technique they had previously developed, they grew tube-like structures out of skin cells. This involved combining the skin cells in fibrin, and providing nutrients in a bioreactor. After washing the skin cells out with detergent, the researchers were left with a collageneous matrix which would not provoke an immune response when implanted. They then sewed and trimmed three of these tubes together to make a 19mm diameter heart valve-like structure.
“After these initial steps, it looked like a heart valve, but the question then became if it could work like a heart valve and if it could grow,” Tranquillo said. “Our findings confirmed both.”
The valves grew from 19mm to 25mm over a year, and showed little of the calcification or clotting associated with prosthetic valves, while performing better than animal-derived valves.”We knew from previous studies that the engineered tubes have the capacity to regenerate and grow in a growing lamb model, but the biggest challenge was how to maintain leaflet function in a growing valved conduit that goes through 40 million cycles in a year,” said lead researcher Zeeshan Syedain, a University of Minnesota senior research associate in Tranquillo’s lab. “When we saw how well the valves functioned for an entire year from young lamb to adult sheep, it was very exciting.”
The next steps are to implant the valve into the right ventricle of the heart to see how it performs, and apply for FDA approval to proceed to human trials.
Journal information: Zeeshan H. Syedain et al, Pediatric tri-tube valved conduits made from fibroblast-produced extracellular matrix evaluated over 52 weeks in growing lambs, Science Translational Medicine (2021). DOI: 10.1126/scitranslmed.abb7225
Commencing chemotherapy several days before the first lumbar puncture for diagnosis and treatment of acute lymphoblastic leukaemia (ALL) may lower the risk of central nervous system (CNS) relapse in children, according to a study from St Jude Children’s Research Hospital and collaborators in China.
“This study identified factors to help us predict and better manage the risk of CNS relapse that will be useful for treating ALL patients worldwide, in both resource-rich and resource-limited countries,” said corresponding author Ching-Hon Pui, MD, chair of the St. Jude Department of Oncology. Dr Pui pioneered paediatric ALL treatment that has achieved 94% long-term survival for St. Jude patients that did not receive brain irradiation.
Using an adapted paediatric protocol from St Jude Hospital, 7640 children and adolescents across 20 Chinese hospitals were enrolled in the trial. However, there was a great disparity across the hospital settings. For example, just three of the hospitals offered total intravenous anaesthesia for children undergoing spinal taps, while only two had flow cytometry for the diagnosis of leukaemia cells in cerebrospinal fluid.
The five-year overall survival rate was 91% for study patients, and the cancer-free survival rate was 80%, which is a dramatic improvement over previous clinical trials in China. But 1.9% of patients relapsed in the CNS alone, and in another 2.7% of patients the relapse involved the CNS. In comparison, a Canadian study reported a 6.6% rate for CNS-involved relapse in paediatric ALL patients followed over 10 years.
According to Dr Piu, in order to increase the survival rate of paediatric ALL patients requires identifying those at risk for CNS relapse, along with increasing their quality of life. Three factors reduced the risk of CNS relapse. First, commencing dexamethasone a few days before the spinal tap, prevents leukaemia cells entering the cerebrospinal fluid (CSF). Second, intravenous anaesthesia reduced bleeding risk during lumbar punctures, and improved intrathecal therapy. Third, flow cytometry enables more accurate diagnosis of leukaemia cells in CSF, and reduced CNS relapse.
Journal information: Jingyan Tang et al. Prognostic Factors for CNS Control in Children with Acute Lymphoblastic Leukemia Treated Without Cranial Irradiation, Blood (2021). DOI: 10.1182/blood.2020010438
At the Cognitive Neuroscience Society’s (CNS) annual meeting, researchers from the University of Minnesota presented their work on early interventions to ameliorate negative effects on infant brain health.
Their two interventions consist of using engineered gut microbes for antibiotic-exposed infants and the other is a choline supplement to treat infants exposed to alcohol in the womb.
Dr Gale’s new research shows that infants with different compositions of gut bacteria process auditory and visual stimuli differently during memory tasks. “These results raise the possibility that gut bacteria are involved in the development of brain function,” she said.
The study compared the brain activity of infants who received antibiotics within their first month of life to those who did not. Using EEG, the researchers recorded a type of electrical activity called event related potentials (ERPs) in the infants’ brains in response to either their mother’s voice or a stranger’s voice – a “recognition memory” that can be assessed in preverbal infants before any behavioral changes are apparent. This has been shown to be an effective assessment of many aspects of cognitive development.
“Recognition memory is one of the earliest types of explicit memory to develop and is known to be dependent on medial temporal lobe structures, including the hippocampus, the brain region affected by microbiome perturbation in animal models,” explained Dr Cheryl Gale, of the University of Minnesota.
The ERP measurements of infants exposed to antibiotics showed an abnormal response to their mother’s voices compared to those unexposed. While antibiotics were associated with impact on brain function, a causal relationship could not be established. “We don’t yet know if there is a definitive cause and effect relationship between microbes and brain function in human infants, but future research will hopefully be able to shed light on this,” Gale says.
The work raises the prospect of creating engineered microbes as an early life intervention. “Infancy is a critical time window for brain development, when therapeutic interventions can have effects for the life-course,” Gale said.
The other study was on foetal alcohol exposure, which is still a widespread problem, involved in some 8 in 1000 births worldwide, resulting in serious cognitive consequences. Dr Jeff Wozniak became aware of a lack of neural imaging studies in this very high-need population.
“So I became interested in using some of the tools that we had available here at the University of Minnesota to do high-quality imaging of brain structure and function in this understudied population to learn something about how the brain is altered by prenatal alcohol exposure at the earliest stages of development,” he said.
Together with colleagues, they identified a number of pathways by which alcohol impacts the foetus, such as interfering with the myelination of nerves. The researchers came up with a treatment: choline, an essential nutrient. This has been used in a number of double-blind, placebo-controlled clinical trials in 2-5 year olds with foetal alcohol exposure. Children receiving choline early in life showed higher non-verbal intelligence, higher visual-spatial skill, higher working memory ability, better verbal memory, and fewer behavioral symptoms of attention deficit hyperactivity disorder (ADHD) than those in the placebo group.
“The further back you go and do your intervention, the more leverage you have to alter the developmental trajectory of that particular child,” Dr Wozniak said. “So that was the exciting thing about bringing those children back and looking at their development and seeing much larger choline versus placebo effects in cognitive functions like working memory and even behavioural differences in terms of ADHD.”
A small-scale study led by Durham University in the UK, has shown that play with ultra-thin dolls may negatively affect body image in girls as young as five years old.
The researchers warn that the dolls, combined with exposure to ‘thin ideals’ in the media, could lead to body dissatisfaction in young girls, which has been shown to be a factor in the development of eating disorders. A Dutch study showed that girls randomised to receive an ultra-thin doll to play with ate less than those who received a realistic adult doll.
The study had 30 girls aged between 5-9 years old play with an ultra-thin doll, a realistic childlike doll or a car. Before and after each play session, the girls were asked about their perceived own body size and ideal body size via an interactive computer test using pictures.
Playing with the ultra-thin dolls reduced girls’ ideal body size immediately after play. There was no improvement even when they subsequently played with the childlike dolls or cars afterwards, demonstrating that playing with other toys cannot quickly counteract the effects. The realistic children’s dolls had a neutral effect on body ideals.
Lead author Professor Lynda Boothroyd, from Durham University’s Department of Psychology, said: “Body dissatisfaction is a huge problem, particularly amongst young girls. It can have serious consequences for girls’ wellbeing and lead to eating disorders and depression.
“The results from our study indicate that playing with ultra-thin dolls, which are sold in the millions each year, could have a real negative impact on girls’ body image. This is on top of all the images of unrealistic body sizes they see on TV, in films and on social media. This is something that needs to be addressed in order to reduce the pressure on girls and women to aspire to a ‘thin ideal body’.”
The psychologists had found in previous research that the more TV we watch, the more we prefer thinner female bodies. Of the girls who took part in the study, 80% said they had ultra-thin dolls at home or with their friends, and nearly all watched films which tend to portray very thin female bodies. Dolls available in shops tend to have a projected BMI of 10 to 16 (underweight). The study used realistically proportioned dolls resembling healthy children of 7 and 9.
Dr Elizabeth Evans, from Newcastle University’s School of Psychology, said: “This study isn’t intended to make parents feel guilty about what’s in their child’s toy box, and it certainly isn’t trying to suggest that ultra-thin dolls are ‘bad’.
“What our study provides is useful information that parents can take into account when making decisions about toys. Ultra-thin dolls are part of a bigger picture of body pressures that young children experience, and awareness of these pressures is really important to help support and encourage positive body image in our children.”
The study, though small, tested the children before and after doll play, an unusual approach which nevertheless adds to growing evidence that doll play affects young girls’ beauty ideals.
Professor Martin Tovee, from Northumbria University’s Department of Psychology, said: “Our study shows how perception of ideal body size and shape is moulded from our earliest years to expect unrealistic ideals. This creates an inevitable body image dissatisfaction which is already known to lead towards disordered eating.”
Around 20% of healthy children may possess benign tumours, according to a review of radiographs taken nearly a century ago.
Although it sounds alarming, non-ossifying fibromas and other common benign bone tumours in symptom-free children are not dangerous. Such bone tumours are often discovered on x-rays taken for other causes, such as a fracture.
“Understandably, these tumours cause a lot of anxiety for patients and families as they await confirmation that the tumour is benign,” said Christopher Collier, MD, Indiana University School of Medicine, Indiana University. “They need reassurance and often ask how common these tumours are, when did they first appear, and whether they will resolve over time? We don’t have much evidence to date to address these questions.”
To address these questions, the researchers analysed annual x-rays taken of children’s bones as they grew, however such studies today are not feasible today due to ethical concerns over sensitivity of children to ionising radiation. Therefore, they drew on a unique collection of radiographs from the Brush Inquiry, a study in which a series of healthy, ‘normal’ children in Ohio, underwent annual radiographs from 1926 to 1942.
Dr Collier’s team analysed a total of 25 555 digitised radiographs of 262 children, followed from infancy to adolescence, finding a high prevalence of bone tumours. A total of 35 benign bone tumors were found in 33 children – an overall rate of 18.9 percent when considering that only the left side of the children was radiographed.
Over half of the tumours were non-ossifying fibromas, which are connective tissue masses that have not hardened into bone. Generally, these fibromas appeared around age five, and again around the time of skeletal maturation, possibly linked to growth spurts. Of 19 non-ossifying fibromas detected, seven disappeared over time. Others may have resolved some time after the annual radiographs stopped.
Rarer benign bone tumoors included enostoses, sometimes called ‘bone islands’; and osteochondromas or enchondromas (tumours in cartilage). In patients with these tumours, they persisted to the last available radiograph.
The findings are similar to the rates of benign bone tumours in healthy adults. Dr Collier noted: “Despite the inherent limitations of our historical study, it may provide the best available evidence regarding the natural history of asymptomatic benign childhood bone tumors.”
Research in Australia on new pneumonia vaccines show that while pneumonia in children is being suppressed, empyaema is increased.
The research, which was led by the University of New South Wales (UNSW), examined the impact of the new 13-valent pneumococcal conjugate vaccine (13vPCV) on childhood pneumonia and empyaema. Empyaema, which is the collection of pus in the lungs, occurs in about 1% of children with pneumonia. In children, empyaema is far less fatal than it is in adults, but it does extend hospitalisation, requiring antibiotics and surgery or installation of a drain. The findings of the study showed that while 13vPCV resulted in a 21% drop in childhood pneumonia hospitalisations, there was a contemporaneous 25% rise in empyaema hospitalisations.
According to senior author Professor Adam Jaffe, Head of the School of Women’s and Children’s Health at UNSW Medicine & Health, said the findings suggested an emergence of non-vaccine serotypes—those which 13vPCV does not cover.
13vPCV was introduced to cover the 13 most common serotypes that cause invasive pneumococcal infection, adding six more serotypes over the seven serotypes covered by its predecessor, 7cPCV.
Prof Jaffe said: “Although we found a substantial reduction in serotype 1, serotype 3 is now the predominant organism which causes childhood empyema—in 76% of cases—so, efforts must be made to create a vaccine which is more effective against serotype 3.
“In fact, Australia recently changed the vaccination dosage schedule to try and improve the effectiveness of 13vPCV against serotype 3, but we need to continue monitoring patients using molecular techniques to see if this change has had an impact.
“Childhood bacterial pneumonia and empyema are potentially preventable diseases through vaccination. So, if Australia can develop an effective vaccine, we could prevent children from being hospitalized with pneumonia and empyema.”
The researchers conducted a similar study over four years during the 7vPCV era.
“Our new study had two parts,” Prof Jaffe said. “We analysed national hospitalisations for childhood empyaema and childhood pneumonia, then we conducted an enhanced surveillance study on children with empyaema.”
The first part of the research used publicly available hospitalisations data to find out if the introduction of 13vPCV changed how many children were admitted to hospital with pneumonia and empyaema.
The enhanced surveillance study involved the collection of blood and lung fluid samples from 401 children with empyaema, followed by molecular testing on these samples and comparing the results to their previous study undertaken during the period of 7vPCV.
Prof Jaffe said research with a larger sample was ongoing, and 13vPCV monitoring was needed.
Journal information: Roxanne Strachan et al. Assessing the impact of the 13 valent pneumococcal vaccine on childhood empyema in Australia, Thorax (2021). DOI: 10.1136/thoraxjnl-2020-216032
