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News Article | November 15, 2016
Site: globenewswire.com

MADISON, Wis., Nov. 15, 2016 (GLOBE NEWSWIRE) -- The prospect of creating artery "banks" available for cardiovascular surgery, bypassing the need to harvest vessels from the patient, could transform treatment of many common heart and vascular ailments. But it's a big leap from concept to reality. The Morgridge Institute for Research and the University of Wisconsin-Madison will address both the engineering and biomedical hurdles in this process through a five-year, $8 million project funded by the National Heart, Lung and Blood Institute (NHLBI). Patients needing bypass surgeries would benefit from a better source for arteries, says project leader James Thomson, a stem cell research pioneer and Director of Regenerative Biology at Morgridge. Replacement tissue currently comes from another part of the patient's body, and suitable tissue can't be found for many patients. Current synthetic alternatives also fail at a high rate. Diseases of blood vessels — including coronary artery disease — kill more people worldwide than any other single cause. "Tissue engineering for blood vessels is a pretty mature field," says Thomson. "But there are still two major problems: One is the time it takes to make the vessels, and the other is the source of the cells to grow them." For example, taking induced pluripotent (iPS) stem cells from an individual patient, growing the relevant cells and assembling them into an artery would overcome the problem of transplant rejection, Thomson says. However, it would be cost-prohibitive and take months to complete — too long to be clinically useful to a patient. The promising alternative is to create tissue with cells banked from a unique population of people who are genetically compatible donors, based on rare alleles that circumvent rejection. Alleles are gene pairings that control certain characteristics, such as blood type. It has been estimated that about 100 different cell lines from this rare population would be enough to cover a majority of the U.S. population. The Morgridge and UW-Madison effort covers four phases and addresses key questions about the feasibility of this approach. The model for the project is designed around treating critical limb ischemia, a debilitating condition that restricts blood flow to limbs and often leads to amputation or death. The Thomson group is working to create the optimal cellular building blocks of the artery — endothelial and smooth muscle cells — that will be most suitable for transplantation and continue to grow and remodel in the patient. In tandem, a team led by UW-Madison engineer Tom Turng will develop scaffolds from natural and synthetic materials to provide structure and shape for the artery. UW-Madison engineer Naomi Chesler will build a bioreactor that provides an environment in which the arterial cells can grow around the scaffolding. The transplant surgery and resulting immune response will then be tested using a monkey limb ischemia model at the Wisconsin Regional Primate Research Center. Having a primate model is important to produce results more relevant to human health than those from mice or other short-lived animals. Pathology and laboratory medicine Professor Igor Slukvin is leading the Primate Center effort. Finally, the UW-Madison Waisman Center Biomanufacturing facility will lead the production of arterial cells that meet FDA standards for human clinical trials, paving the way for potential treatments for limb ischemia in humans. If the entire process works, Thomson estimates that potential human therapies remain about 10 years away. "This is a collaboration that really highlights what the Morgridge Institute does well because we are able to bring together multiple investigators from different departments and centers around campus," Thomson says. "We have an unusual combination of resources in Madison to be able to pull this off." The heart of the partnership to date has been between Thomson's team and the BIONATES program in the Wisconsin Institute for Discovery, led by Turng. Both labs operate on the same floor of the Discovery building, and the three-year collaboration is a prime example of how the public and private institutes work together to foster innovation. As Turng works to determine the optimal choice of materials and methods for construction of the blood vessel scaffolds – biopolymers versus synthetic polymers or a combination of both – the partnership inside the Discovery Building is vital to progress. "We have direct feedback with Thomson and the other researchers, and the iterative process can be facilitated," says Turng. "We can speak the same language." Providing proof of concept on the blood vessel work should benefit other forms of stem cell therapy, Thomson says. "This has implications beyond making vessels for transplantation; it's sort of the stepping stone to more advanced tissue engineering," he says. "Any form of cellular transplant therapy is going to need a blood supply, and we need to learn how to engineer that blood supply to work with more complex tissues."


News Article | November 15, 2016
Site: globenewswire.com

MADISON, Wis., Nov. 15, 2016 (GLOBE NEWSWIRE) -- The prospect of creating artery "banks" available for cardiovascular surgery, bypassing the need to harvest vessels from the patient, could transform treatment of many common heart and vascular ailments. But it's a big leap from concept to reality. The Morgridge Institute for Research and the University of Wisconsin-Madison will address both the engineering and biomedical hurdles in this process through a five-year, $8 million project funded by the National Heart, Lung and Blood Institute (NHLBI). Patients needing bypass surgeries would benefit from a better source for arteries, says project leader James Thomson, a stem cell research pioneer and Director of Regenerative Biology at Morgridge. Replacement tissue currently comes from another part of the patient's body, and suitable tissue can't be found for many patients. Current synthetic alternatives also fail at a high rate. Diseases of blood vessels — including coronary artery disease — kill more people worldwide than any other single cause. "Tissue engineering for blood vessels is a pretty mature field," says Thomson. "But there are still two major problems: One is the time it takes to make the vessels, and the other is the source of the cells to grow them." For example, taking induced pluripotent (iPS) stem cells from an individual patient, growing the relevant cells and assembling them into an artery would overcome the problem of transplant rejection, Thomson says. However, it would be cost-prohibitive and take months to complete — too long to be clinically useful to a patient. The promising alternative is to create tissue with cells banked from a unique population of people who are genetically compatible donors, based on rare alleles that circumvent rejection. Alleles are gene pairings that control certain characteristics, such as blood type. It has been estimated that about 100 different cell lines from this rare population would be enough to cover a majority of the U.S. population. The Morgridge and UW-Madison effort covers four phases and addresses key questions about the feasibility of this approach. The model for the project is designed around treating critical limb ischemia, a debilitating condition that restricts blood flow to limbs and often leads to amputation or death. The Thomson group is working to create the optimal cellular building blocks of the artery — endothelial and smooth muscle cells — that will be most suitable for transplantation and continue to grow and remodel in the patient. In tandem, a team led by UW-Madison engineer Tom Turng will develop scaffolds from natural and synthetic materials to provide structure and shape for the artery. UW-Madison engineer Naomi Chesler will build a bioreactor that provides an environment in which the arterial cells can grow around the scaffolding. The transplant surgery and resulting immune response will then be tested using a monkey limb ischemia model at the Wisconsin Regional Primate Research Center. Having a primate model is important to produce results more relevant to human health than those from mice or other short-lived animals. Pathology and laboratory medicine Professor Igor Slukvin is leading the Primate Center effort. Finally, the UW-Madison Waisman Center Biomanufacturing facility will lead the production of arterial cells that meet FDA standards for human clinical trials, paving the way for potential treatments for limb ischemia in humans. If the entire process works, Thomson estimates that potential human therapies remain about 10 years away. "This is a collaboration that really highlights what the Morgridge Institute does well because we are able to bring together multiple investigators from different departments and centers around campus," Thomson says. "We have an unusual combination of resources in Madison to be able to pull this off." The heart of the partnership to date has been between Thomson's team and the BIONATES program in the Wisconsin Institute for Discovery, led by Turng. Both labs operate on the same floor of the Discovery building, and the three-year collaboration is a prime example of how the public and private institutes work together to foster innovation. As Turng works to determine the optimal choice of materials and methods for construction of the blood vessel scaffolds – biopolymers versus synthetic polymers or a combination of both – the partnership inside the Discovery Building is vital to progress. "We have direct feedback with Thomson and the other researchers, and the iterative process can be facilitated," says Turng. "We can speak the same language." Providing proof of concept on the blood vessel work should benefit other forms of stem cell therapy, Thomson says. "This has implications beyond making vessels for transplantation; it's sort of the stepping stone to more advanced tissue engineering," he says. "Any form of cellular transplant therapy is going to need a blood supply, and we need to learn how to engineer that blood supply to work with more complex tissues."


News Article | November 15, 2016
Site: globenewswire.com

MADISON, Wis., Nov. 15, 2016 (GLOBE NEWSWIRE) -- The prospect of creating artery "banks" available for cardiovascular surgery, bypassing the need to harvest vessels from the patient, could transform treatment of many common heart and vascular ailments. But it's a big leap from concept to reality. The Morgridge Institute for Research and the University of Wisconsin-Madison will address both the engineering and biomedical hurdles in this process through a five-year, $8 million project funded by the National Heart, Lung and Blood Institute (NHLBI). Patients needing bypass surgeries would benefit from a better source for arteries, says project leader James Thomson, a stem cell research pioneer and Director of Regenerative Biology at Morgridge. Replacement tissue currently comes from another part of the patient's body, and suitable tissue can't be found for many patients. Current synthetic alternatives also fail at a high rate. Diseases of blood vessels — including coronary artery disease — kill more people worldwide than any other single cause. "Tissue engineering for blood vessels is a pretty mature field," says Thomson. "But there are still two major problems: One is the time it takes to make the vessels, and the other is the source of the cells to grow them." For example, taking induced pluripotent (iPS) stem cells from an individual patient, growing the relevant cells and assembling them into an artery would overcome the problem of transplant rejection, Thomson says. However, it would be cost-prohibitive and take months to complete — too long to be clinically useful to a patient. The promising alternative is to create tissue with cells banked from a unique population of people who are genetically compatible donors, based on rare alleles that circumvent rejection. Alleles are gene pairings that control certain characteristics, such as blood type. It has been estimated that about 100 different cell lines from this rare population would be enough to cover a majority of the U.S. population. The Morgridge and UW-Madison effort covers four phases and addresses key questions about the feasibility of this approach. The model for the project is designed around treating critical limb ischemia, a debilitating condition that restricts blood flow to limbs and often leads to amputation or death. The Thomson group is working to create the optimal cellular building blocks of the artery — endothelial and smooth muscle cells — that will be most suitable for transplantation and continue to grow and remodel in the patient. In tandem, a team led by UW-Madison engineer Tom Turng will develop scaffolds from natural and synthetic materials to provide structure and shape for the artery. UW-Madison engineer Naomi Chesler will build a bioreactor that provides an environment in which the arterial cells can grow around the scaffolding. The transplant surgery and resulting immune response will then be tested using a monkey limb ischemia model at the Wisconsin Regional Primate Research Center. Having a primate model is important to produce results more relevant to human health than those from mice or other short-lived animals. Pathology and laboratory medicine Professor Igor Slukvin is leading the Primate Center effort. Finally, the UW-Madison Waisman Center Biomanufacturing facility will lead the production of arterial cells that meet FDA standards for human clinical trials, paving the way for potential treatments for limb ischemia in humans. If the entire process works, Thomson estimates that potential human therapies remain about 10 years away. "This is a collaboration that really highlights what the Morgridge Institute does well because we are able to bring together multiple investigators from different departments and centers around campus," Thomson says. "We have an unusual combination of resources in Madison to be able to pull this off." The heart of the partnership to date has been between Thomson's team and the BIONATES program in the Wisconsin Institute for Discovery, led by Turng. Both labs operate on the same floor of the Discovery building, and the three-year collaboration is a prime example of how the public and private institutes work together to foster innovation. As Turng works to determine the optimal choice of materials and methods for construction of the blood vessel scaffolds – biopolymers versus synthetic polymers or a combination of both – the partnership inside the Discovery Building is vital to progress. "We have direct feedback with Thomson and the other researchers, and the iterative process can be facilitated," says Turng. "We can speak the same language." Providing proof of concept on the blood vessel work should benefit other forms of stem cell therapy, Thomson says. "This has implications beyond making vessels for transplantation; it's sort of the stepping stone to more advanced tissue engineering," he says. "Any form of cellular transplant therapy is going to need a blood supply, and we need to learn how to engineer that blood supply to work with more complex tissues."


News Article | November 15, 2016
Site: www.eurekalert.org

MADISON -- The prospect of creating artery "banks" available for cardiovascular surgery, bypassing the need to harvest vessels from the patient, could transform treatment of many common heart and vascular ailments. But it's a big leap from concept to reality. The Morgridge Institute for Research and the University of Wisconsin-Madison will address both the engineering and biomedical hurdles in this process through a five-year, $8 million project funded by the National Heart, Lung and Blood Institute (NHLBI). Patients needing bypass surgeries would benefit from a better source for arteries, says project leader James Thomson, a stem cell research pioneer and Director of Regenerative Biology at Morgridge. Replacement tissue currently comes from another part of the patient's body, and suitable tissue can't be found for many patients. Current synthetic alternatives also fail at a high rate. Diseases of blood vessels -- including coronary artery disease -- kill more people worldwide than any other single cause. "Tissue engineering for blood vessels is a pretty mature field," says Thomson. "But there are still two major problems: One is the time it takes to make the vessels, and the other is the source of the cells to grow them." For example, taking induced pluripotent (iPS) stem cells from an individual patient, growing the relevant cells and assembling them into an artery would overcome the problem of transplant rejection, Thomson says. However, it would be cost-prohibitive and take months to complete -- too long to be clinically useful to a patient. The promising alternative is to create tissue with cells banked from a unique population of people who are genetically compatible donors, based on rare alleles that circumvent rejection. Alleles are gene pairings that control certain characteristics, such as blood type. It has been estimated that about 100 different cell lines from this rare population would be enough to cover a majority of the U.S. population. The Morgridge and UW-Madison effort covers four phases and addresses key questions about the feasibility of this approach. The model for the project is designed around treating critical limb ischemia, a debilitating condition that restricts blood flow to limbs and often leads to amputation or death. The Thomson group is working to create the optimal cellular building blocks of the artery -- endothelial and smooth muscle cells -- that will be most suitable for transplantation and continue to grow and remodel in the patient. In tandem, a team led by UW-Madison engineer Tom Turng will develop scaffolds from natural and synthetic materials to provide structure and shape for the artery. UW-Madison engineer Naomi Chesler will build a bioreactor that provides an environment in which the arterial cells can grow around the scaffolding. The transplant surgery and resulting immune response will then be tested using a monkey limb ischemia model at the Wisconsin Regional Primate Research Center. Having a primate model is important to produce results more relevant to human health than those from mice or other short-lived animals. Pathology and laboratory medicine Professor Igor Slukvin is leading the Primate Center effort. Finally, the UW-Madison Waisman Center Biomanufacturing facility will lead the production of arterial cells that meet FDA standards for human clinical trials, paving the way for potential treatments for limb ischemia in humans. If the entire process works, Thomson estimates that potential human therapies remain about 10 years away. "This is a collaboration that really highlights what the Morgridge Institute does well because we are able to bring together multiple investigators from different departments and centers around campus," Thomson says. "We have an unusual combination of resources in Madison to be able to pull this off." The heart of the partnership to date has been between Thomson's team and the BIONATES program in the Wisconsin Institute for Discovery, led by Turng. Both labs operate on the same floor of the Discovery building, and the three-year collaboration is a prime example of how the public and private institutes work together to foster innovation. As Turng works to determine the optimal choice of materials and methods for construction of the blood vessel scaffolds - biopolymers versus synthetic polymers or a combination of both - the partnership inside the Discovery Building is vital to progress. "We have direct feedback with Thomson and the other researchers, and the iterative process can be facilitated," says Turng. "We can speak the same language." Providing proof of concept on the blood vessel work should benefit other forms of stem cell therapy, Thomson says. "This has implications beyond making vessels for transplantation; it's sort of the stepping stone to more advanced tissue engineering," he says. "Any form of cellular transplant therapy is going to need a blood supply, and we need to learn how to engineer that blood supply to work with more complex tissues."


Cho W.,Waisman Center | Brenner M.,University of Alabama at Birmingham | Peters N.,University of Wisconsin - Madison | Messing A.,Waisman Center
Human Molecular Genetics | Year: 2010

Glial fibrillary acidic protein (GFAP) is the major intermediate filament protein of astrocytes in the vertebrate central nervous system. Increased levels of GFAP are the hallmark feature of gliosis, a non-specific response of astrocytes to a wide variety of injuries and disorders of the CNS, and also occur in Alexander disease where the initial insult is a mutation within the coding region of GFAP itself. In both settings, excess GFAP may cause or exacerbate astrocyte dysfunction. With the goal of finding drugs that reduce the expression of GFAP, we have devised screens to detect changes in GFAP promoter activity or protein levels in primary cultures of mouse astrocytes in a 96-well format. We have applied these screens to libraries enriched in compounds that are already approved for human use by the FDA. We report that several compounds are active at micromolar levels in suppressing the expression of GFAP. Treatment of mice for 3 weeks with one of these drugs, clomipramine, causes nearly 50% reduction in the levels of GFAP protein in brain. © The Author 2010. Published by Oxford University Press. All rights reserved.


News Article | September 14, 2016
Site: www.biosciencetechnology.com

Researchers at the University of Wisconsin–Madison have found a switch that redirects helper cells in the peripheral nervous system into "repair" mode, a form that restores damaged axons. Axons are long fibers on neurons that transmit nerve impulses. The peripheral nervous system, the signaling network outside the brain and spinal cord, has some ability to regenerate destroyed axons, but the repair is slow and often insufficient. The new study suggests tactics that might trigger or accelerate this natural regrowth and assist recovery after physical injury, says John Svaren, a professor of comparative biosciences at the UW–Madison School of Veterinary Medicine. The finding may also apply to genetic abnormalities such as Charcot-Marie-Tooth disease or nerve damage from diabetes. Svaren, senior author of a report published Aug. 30 in the Journal of Neuroscience, studied how Schwann cells, which hug axons in the peripheral nervous system, transform themselves to play a much more active and "intelligent" role after injury. Schwann cells create the insulating myelin sheath that speeds transmission of nerve impulses. In the repair mode, Schwann cells form a fix-up crew that adds house cleaning and stimulation of nerve regrowth to the usual insulating job. Svaren and his graduate student, Joseph Ma, compared the activation of genes in Schwann cells in mice with intact or cut axons. "We saw a set of latent genes becoming active, but only after injury," says Svaren, "and these started a program that places the Schwann cells in a repair mode where they perform several jobs that the axon needs to regrow." In the repair mode, but not in the normal one, Schwann cells start cleaning house, helping to dissolve myelin, which is essential for proper functioning but ironically deters regeneration after injury. "If you invite Schwann cells to a party," says Svaren, "they will clean up the bottles and wash your dishes before they leave the house." This cleanup must happen within days of the injury, says Svaren, who directs the cellular and molecular neuroscience core at the Waisman Center on the UW–Madison campus. The Schwann cells also secrete signals that summon blood cells to aid the cleanup, and they map out a pathway for the axon to regrow. Finally, they return to the insulator role to grow a replacement myelin sheath on the regenerated axon. Unexpectedly, the Schwann's transition into the repair form did not entail a reversion to a more primitive form, but rather was based on a change in the regulation of its genes. "Almost every other nervous-system injury response, especially in the brain, is thought to require stem cells to repopulate the cells, but there are no stem cells here," Svaren says. "The Schwann cells are reprogramming themselves to set up the injury-repair program. We are starting to see them as active players with dual roles in protecting and regenerating the axon, and we are exploring which factors determine the initiation and efficacy of the injury program." After the human genome was deciphered, epigenetics—the study of gene regulation—has moved to the forefront with the realization that genes don't matter much until they are switched on, and that genetic switches are the fundamental reason why a skin cell doesn't look like a nerve cell, and a nerve cells functions differently than a white blood cell. In epigenetics, as elsewhere in biology, processes are often regulated through a balance between "stop" and "go" signals. In the Schwann cell transition, Svaren and Ma identified a system called PRC2 that usually silences the repair program. "This pathway amounts to an on-off switch that is normally off," Svaren says, "and we want to know how to turn it on to initiate the repair process." The nature of the top-level gene-silencing system suggested drugs that might remove the silencing mark from the genes in question, and Svaren says he's identified an enzyme that may "remove the brakes" and deliberately activate the repair program when needed in response to injury. Even if the drug tests are promising, years of experiments will be necessary before the system can be tested in people. Furthermore, as Svaren acknowledges, "many factors determine how well an axon can regenerate. I am not saying this single pathway could lead to a cure-all, but we do hope it is an important factor." Svaren says it's not clear how the current finding on peripheral nerves relates to damage to the brain and spinal cord, where a different type of cell cares for neurons. There are some similarities, however. In multiple sclerosis, for example, cleanup must precede the replacement of damaged myelin. Ultimately, the study could open a new door on regeneration, even beyond one key sector of the nervous system. "We have thought of the Schwann cell as a static entity that was just there to make myelin, but they have this latent program, where they become the first responders and initiate many actions that are required for the axon to regenerate," Svaren says.


Grieco-Calub T.M.,Waisman Center | Litovsky R.Y.,Waisman Center | Litovsky R.Y.,University of Wisconsin - Madison
Ear and Hearing | Year: 2010

Objectives: To measure sound source localization in children who have sequential bilateral cochlear implants (BICIs); to determine whether localization accuracy correlates with performance on a right-left discrimination task (i.e., spatial acuity); to determine whether there is a measurable bilateral benefit on a sound source identification task (i.e., localization accuracy) by comparing performance under bilateral and unilateral listening conditions; and to determine whether sound source localization continues to improve with longer durations of bilateral experience. Design: Two groups of children participated in this study: a group of 21 children who received BICIs in sequential procedures (5 to 14 years) and a group of 7 typically developing children with normal acoustic hearing (5 years). Testing was conducted in a large sound-treated booth with loudspeakers positioned on a horizontal arc with a radius of 1.2 m. Children participated in two experiments that assessed spatial hearing skills. Spatial hearing acuity was assessed with a discrimination task in which listeners determined whether a sound source was presented on the right or left side of center; the smallest angle at which performance on this task was reliably above chance is the minimum audible angle. Sound localization accuracy was assessed with a sound source identification task in which children identified the perceived position of the sound source from a multiloudspeaker array (7 or 15); errors are quantified using the root mean square (RMS) error. Results: Sound localization accuracy was highly variable among the children with BICIs, with RMS errors ranging from 19 to 56°. Performance of the normal hearing group, with RMS errors ranging from 9 to 29° was significantly better. Within the BICI group, in 11 of 21 children, RMS errors were smaller in the bilateral versus unilateral listening condition, indicating bilateral benefit. There was a significant correlation between spatial acuity and sound localization accuracy (R = 0.68, p < 0.01), suggesting that children who achieve small RMS errors tend to have the smallest minimum audible angles. Although there was large intersubject variability, testing of 11 children in the BICI group at two sequential visits revealed a subset of children who show improvement in spatial hearing skills over time. Conclusions: A subset of children who use sequential BICIs can acquire sound localization abilities, even after long intervals between activation of hearing in the first- and second-implanted ears. This suggests that children with activation of the second implant later in life may be capable of developing spatial hearing abilities. The large variability in performance among the children with BICIs suggests that maturation of sound localization abilities in children with BICIs may be dependent on various individual subject factors such as age of implantation and chronological age. Copyright © 2010 by Lippincott Williams & Wilkins.


Van Hulle C.A.,Waisman Center | Schmidt N.L.,Waisman Center | Goldsmith H.H.,Waisman Center | Goldsmith H.H.,University of Wisconsin - Madison
Journal of Child Psychology and Psychiatry and Allied Disciplines | Year: 2012

Background: Although impaired sensory processing accompanies various clinical conditions, the question of its status as an independent disorder remains open. Our goal was to delineate the comorbidity (or lack thereof) between childhood psychopathology and sensory over-responsivity (SOR) in middle childhood using phenotypic and behavior-genetic analyses. Method: Participants (N = 970) were drawn from the Wisconsin Twin Project, a population-based sample of twins and their families. Mothers completed a sensory responsivity checklist when their offspring were on average 7 years old, followed by a diagnostic interview (Diagnostic Interview Schedule for Children; DISC) within 6-12 months. We examined the incidence of DISC diagnoses - attention deficit hyperactivity disorder, conduct disorder, oppositional defiant disorder, agoraphobia, general anxiety, obsessive-compulsive disorder, panic disorder, separation anxiety, social phobia, specific phobia, depression, enuresis, trichtollomaniatics, selective mutism, and pica - among children with SOR, and vice versa. Children with autism or pervasive developmental disorders were excluded from the present study. In addition, we examined parent-reported physical health diagnoses among nondiagnosed children and three groups of children with SOR and/or DISC diagnoses. Biometric models explored common underlying genetic and environmental influences on symptoms of SOR and psychopathology. Results: A majority of individuals who screened positive for SOR did not qualify for a DISC diagnosis (58.2%), and vice versa (68.3%). Children who screened positive for SOR only and typical children had similar rates of physical health problems. Turning to a dimensional approach, multivariate twin models demonstrated that modest covariation between SOR and DISC symptoms could be entirely accounted for by common underlying genetic effects. Conclusions: Our results suggest that SOR occurs independently of recognized childhood psychiatric diagnoses but is also a relatively frequent comorbid condition with recognized diagnoses. Genetic sources of this comorbidity are implicated. © 2011 Association for Child and Adolescent Mental Health.


Shriberg L.D.,Waisman Center | Potter N.L.,Washington State University | Strand E.A.,Mayo Medical School
Journal of Speech, Language, and Hearing Research | Year: 2011

Purpose: In this article, the authors address the hypothesis that the severe and persistent speech disorder reported in persons with galactosemia meets contemporary diagnostic criteria for Childhood Apraxia of Speech (CAS). A positive finding for CAS in this rare metabolic disorder has the potential to impact treatment of persons with galactosemia and inform explanatory perspectives on CAS in neurological, neurodevelopmental, and idiopathic contexts. Method: Thirty-three youth with galactosemia and significant prior or persistent speech sound disorder were assessed in their homes in 17 states. Participants completed a protocol yielding information on their cognitive, structural, sensorimotor, language, speech, prosody, and voice status and function. Results: Eight of the 33 participants (24%) met contemporary diagnostic criteria for CAS. Two participants, 1 of whom was among the 8 with CAS, met criteria for ataxic or hyperkinetic dysarthria. Groupwise findings for the remaining 24 participants are consistent with a classification category termed Motor Speech Disorder-Not Otherwise Specified (Shriberg, Fourakis et al., 2010a). Conclusion: The authors estimate the prevalence of CAS in galactosemia at 18 per hundred-180 times the estimated risk for idiopathic CAS. Findings support the need to study risk factors for the high occurrence of motor speech disorders in galactosemia despite early compliant dietary management. © American Speech-Language-Hearing Association.


News Article | November 29, 2016
Site: www.eurekalert.org

MADISON, Wis. -- It's not how many words a kid knows; it's how they choose them that tells Hilary Miller the most about their spatial skills. That grasp of the layout of their physical word -- understanding where they are relative to a friend, imagining how to rotate puzzle pieces to fit them together, conjuring a mental map of the park -- is important. "We know that better spatial abilities lead to better math skills in early childhood, and they are strong predictors of future interest in careers in science and technology and engineering," says Miller, a graduate student studying child development at the University of Wisconsin-Madison. "So we're targeting ways to enhance spatial skills at an early age." Working with UW-Madison psychology Professor Vanessa Simmering and educational psychology Professor Haley Vlach, Miller is studying the way 4-year-olds use words to describe spatial relationships. "At that age, they may have learned words like 'above' and 'below' that adults use often to describe a location," Miller says. "But simply knowing the words doesn't necessarily help. They have to know when that kind of information is useful." In a study published this month in the journal Child Development, Miller shows preschool age kids often skip location words and lean on other relevant information to describe important spatial details. Miller showed each child a series of pictures in which a mouse appeared in different spots -- atop objects that shifted from picture to picture in location, size and color -- and asked the kids to describe the mouse's location. She scored their answers based on how much relevant information they shared, weighing it against irrelevant cues (mentioning an attribute that all the objects share, for example). While previous research has often judged children's spatial skills based at least in part on the number of spatial words they know, Miller found her study subjects to have a range of descriptive abilities not limited by the size of their spatial vocabulary. "They are describing where the mouse is by saying, 'He's on the big table,' or 'on the brown box,'" Miller says. "Those size and color words aren't spatial terms in this context, but in the context of the picture they're seeing they are really useful." The better the kids were at adapting to each image and supplying relevant information, the higher their scores tended to be on the tests of other spatial skills that predict future success in, say, math. "We think it's attention to relevant information that is propelling them to perform better, and not their ability to say particular words," says Miller, whose work is supported by the National Institutes of Health and UW-Madison's Waisman Center. "We're hoping focusing on this early in childhood will help children get to the levels of success later on that seem to promote STEM interests." Because children learn about language by listening to the way it is used, adults can find everyday opportunities to help them develop the tools to build their spatial skills. "When you talk to your children, use different kinds of language," Miller says. "Be more conscious of how useful your spatial language is." The more diverse and relevant the words kids hear, the more they will start to make the same useful connections with language. "Pointing and saying, 'There are your shoes,' is the sort of thing parents are probably doing most often," Miller says. "But saying something like, 'Your shoes are on the red rug,' focuses your child on relevant spatial information. It's practice for them with a different way to approach a problem."

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