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Journal of Pediatric Orthopedics. Part B Mar 2022Quantitative data assessment on the basis of three-dimensional gait analysis has been routinely used in the evaluation of pathological gait of children with cerebral...
Quantitative data assessment on the basis of three-dimensional gait analysis has been routinely used in the evaluation of pathological gait of children with cerebral palsy. However, a similar quantitative methodology has not been applied for spina bifida patients in whom atypical gait patterns are thought to correlate with various levels of neurological paralysis. The purpose of this study is to investigate the differences among gait patterns in spina bifida between different levels of neurological lesions using quantitative methods: Gait profile score (GPS) and gait variable scores (GVS), scoring subject's gait deviation from a reference. In this cross-sectional study, 22 children with spina bifida (11 women, 11 men; mean age 9.4 years, SD 3.8 years, range 3-17 years), were examined using three-dimensional gait analysis from 2008 to 2018. Physical examination allowed for classification of each of the 44 limbs as either L4, L5 or S1 and comparison with the GPS and GVS using a linear mixed model. GPS and the GVS of the pelvis and hip range of motion in the coronal plane were significantly higher in the L4 group than in the L5 and S1 groups (GPS, P = 0.041, P = 0.003, respectively; GVS of pelvis, P = 0.001, P = 0.001; GVS of hip, P < 0.001, P < 0.001) GVS (foot progression angle) was significantly lower in the S1 group than in L4 and L5 groups (P < 0.001, P = 0.037). We found that GPS and GVS enable us to quantitatively assess the differences among gait patterns between different neurological levels. The scoring tool showed the potential for detecting individual neurological changes.
Topics: Adolescent; Child; Child, Preschool; Cross-Sectional Studies; Female; Gait; Gait Disorders, Neurologic; Humans; Male; Range of Motion, Articular; Spinal Dysraphism
PubMed: 34028379
DOI: 10.1097/BPB.0000000000000877 -
Child's Nervous System : ChNS :... Aug 2022Spina bifida is a type of a neural tube defect which affects 243.14 per 100,000 babies in Asia. Research articles on spina bifida have increased in the recent years....
BACKGROUND
Spina bifida is a type of a neural tube defect which affects 243.14 per 100,000 babies in Asia. Research articles on spina bifida have increased in the recent years. However, no study has focused on the research trends in this field in Asia.
METHODS
A systematic review of literature on spina bifida in Asia was performed using the Scopus database from inception to 2020. All published studies on spina bifida conducted in or published by authors from Asia were included in our analysis. Bibliometric information was obtained from Scopus and bibliometrics diagrams were created using VOSviewer software.
RESULTS
A total of 652 articles were obtained in this study. The number of publications showed an upward trend starting 2000s. The country with the greatest number of publications was Japan while All India Institute of Medical Sciences was the most productive institution in spina bifida research in Asia. The current focus of this field in Asia was prevalence of spina bifida, prenatal diagnosis, folic acid supplementation, and complications of spina bifida. Future areas of research in spina bifida include the genetic basis of neural tube defects and the use of stem cell technology as therapies for spina bifida.
CONCLUSION
This is the first bibliometric analysis on spina bifida in Asia. It showed the trend and future areas of research on spina bifida in Asia. Despite the increase in scientific literature on spina bifida research, more research outputs and collaborations are needed especially in developing countries in Asia.
Topics: Bibliometrics; Female; Humans; Neural Tube Defects; Pregnancy; Prenatal Diagnosis; Prevalence; Spinal Dysraphism
PubMed: 35474541
DOI: 10.1007/s00381-022-05527-2 -
Journal of Pediatric Rehabilitation... 2023Whether it is for collaboration on folic acid fortification or the standardization of care efforts concerning neurogenic bowel dysfunction, a global forum on neural tube...
Whether it is for collaboration on folic acid fortification or the standardization of care efforts concerning neurogenic bowel dysfunction, a global forum on neural tube defects related issues is needed. Propitiously, the 2023 Spina Bifida World Congress sponsored by the Spina Bifida Association (SBA) was a catalyst for transnational dialog in the field of spina bifida (SB) research. Concurrently, the Journal of Pediatric Rehabilitation Medicine (JPRM) provides a platform for both international research as well as numerous clinical and educational projects, such as The Lifespan Bowel Management Protocol, and social interventions taught through the American Academy of Pediatrics' Spina Bifida Transition ECHO. Through this open access issue, work by colleagues in Ethiopia, the Nordic countries, and Switzerland, as well as among other transnational populations is highlighted. The development of the Spina Bifida Global Learning Collaborative is also showcased, representing a training initiative across four continents. Correspondingly in this issue, JPRM published an update to the Transition Guidelines for the Care of People with Spina Bifida. The clinical guidelines are a product of the SBA Collaborative Care Network cooperative agreement with the National Center on Birth Defects and Developmental Disabilities in the Centers for Disease Control and Prevention. While colleagues across the globe remain committed to native, immigrant, and displaced populations of individuals affected by SB, JPRM will continue to distribute premier research in multidisciplinary care, education, and advocacy.
Topics: Pregnancy; Female; Humans; Child; United States; Prenatal Care; Longevity; Spinal Dysraphism; Neural Tube Defects; Emigrants and Immigrants
PubMed: 38160377
DOI: 10.3233/PRM-239020 -
Journal of Pediatric Rehabilitation... 2020The life expectancy for people with spina bifida has increased, thus resulting in greater need for guidelines in urologic care in order to protect normal renal function,... (Review)
Review
PURPOSE
The life expectancy for people with spina bifida has increased, thus resulting in greater need for guidelines in urologic care in order to protect normal renal function, to develop strategies for urinary continence, and to advance independence through adult years.
METHODS
The English literature was assessed from 2002-2015; greater than 300 publications identified. Case reports and opinion pieces were eliminated leaving 100 for in depth review. Clinical questions were then established for each age group that allowed for focused assessment.
RESULTS
There was no Level 1 evidence for any of the defined clinical questions. This resulted in group consensus for all questions throughout all age groups. Guidelines were provided for identifying a symptomatic urinary infection, the role of urodynamic bladder testing and identification of bladder hostility, determining methods of renal function assessment and surveillance, the initiation of continence control, and transitioning to self-care through the teen and adult years.
CONCLUSION
Urologic guidelines continue to be based on clinical consensus due to the lack of high level evidence-based research. Further research is required in all aspects of urologic management. While not the "Standard of Care," these guidelines should be considered "Best Practice".
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Practice Guidelines as Topic; Spinal Dysraphism; Urinary Bladder; Urinary Bladder, Neurogenic; Urodynamics; Young Adult
PubMed: 33252091
DOI: 10.3233/PRM-200712 -
Child Neuropsychology : a Journal on... Feb 2024The primary aim of this study was to characterize Cognitive Disengagement Syndrome (CDS) symptomatology in youth with spina bifida (SB). One hundred and sixty-nine...
The primary aim of this study was to characterize Cognitive Disengagement Syndrome (CDS) symptomatology in youth with spina bifida (SB). One hundred and sixty-nine patients aged 5-19 years old were drawn from clinical cases seen through a multidisciplinary outpatient SB clinic at a children's hospital between 2017 and 2019. Parent-reported CDS and inattention were measured using Penny's Sluggish Cognitive Tempo Scale and the Vanderbilt ADHD Rating Scale. Self-reported internalizing symptoms were measured with the 25-item Revised Children's Anxiety and Depression Scale (RCADS-25). We replicated Penny's proposed 3-factor structure of CDS with , , and components. The component of CDS overlapped heavily with inattention, while the and components were distinct from inattention and internalizing symptoms. Eighteen percent (22 of 122) of the full sample met criteria for elevated CDS, and 39% (9 of 22) of those patients did not meet criteria for elevated inattention. Diagnosis of myelomeningocele and presence of a shunt were associated with greater CDS symptoms. CDS can be measured reliably in youth with SB and can be discriminated from inattention and internalizing symptoms in this population. ADHD rating scale measures fail to identify a substantial portion of the SB population with attention-related challenges. Standard screening for CDS symptoms in SB clinics may be important to help identify clinically impairing symptoms and design targeted treatment plans.
Topics: Adolescent; Humans; Child; Child, Preschool; Young Adult; Adult; Attention Deficit Disorder with Hyperactivity; Anxiety; Self Report; Cognition; Spinal Dysraphism
PubMed: 36803439
DOI: 10.1080/09297049.2023.2181944 -
Journal of Pediatric Rehabilitation... 2023As with the wide range in spina bifida (SB) incidence rates across nations, there is also wide variance in topics encountered by clinicians today. Both the wide variance...
As with the wide range in spina bifida (SB) incidence rates across nations, there is also wide variance in topics encountered by clinicians today. Both the wide variance in SB incidence rates and the wide diversity of topics to be addressed provide the backdrop for any dialogue among professionals serving this population. On the international stage, the World Congress on Spina Bifida Research and Care has been the only conference dedicated solely to research, practical challenges, and real-life solutions for those living with SB, their families, and caregivers. As a conference with a clear sense of the growing global village, the 2023 congress showcased innovative research from junior to preeminent investigators. Topical areas included urology, neurosurgery, global health, prenatal surgery, and transition to adult care amid others. Ultimately, through the dissemination of a compendium of conference abstracts, we hope that professionals will be aided and inspired to continue to improve the education, advocacy, and care among the many communities of individuals affected by SB globally.
Topics: Adult; Pregnancy; Female; Humans; Spinal Dysraphism; Neurosurgery
PubMed: 37270821
DOI: 10.3233/PRM-239016 -
Journal of Pediatric Rehabilitation... 2020Bowel dysfunction, such as constipation and fecal incontinence, has a significant impact on health, activities of daily living, and quality of life among people with... (Review)
Review
PURPOSE
Bowel dysfunction, such as constipation and fecal incontinence, has a significant impact on health, activities of daily living, and quality of life among people with spina bifida. Secondary complications may result from bowel dysfunction and include urologic dysfunction, loss of skin integrity, shunt (hydrocephalus) function, as well as loss of social opportunities and employability.
METHODS
Using a consensus building methodology, the guidelines for management of bowel dysfunction in spina bifida were written by experts in the field of spina bifida and bowel function and care.
RESULTS
The evidence-based guidelines are presented in table format and provide age-specific recommendations to achieve fecal continence without constipation. Recommended treatments are presented from least to most invasive options. Literature supporting the recommendations and the interval research published to date is also presented.
CONCLUSION
These guidelines present a standardized approach to management of bowel dysfunction in spina bifida. Bowel management in children and young adults with spina bifida is limited by variability in clinical practice and paucity of robust research in neurogenic bowel. Collaborative multi-institutional efforts are needed to overcome research barriers and provide innovative solutions.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Intestinal Diseases; Male; Practice Guidelines as Topic; Spinal Dysraphism; Young Adult
PubMed: 33252093
DOI: 10.3233/PRM-200724 -
Journal of Pediatric Rehabilitation... 2020Sexual development is not only comprised of the changes to a person's body during puberty; it is a part of social development and should be considered in the context of... (Review)
Review
Sexual development is not only comprised of the changes to a person's body during puberty; it is a part of social development and should be considered in the context of basic and routine human desires for connectedness and intimacy, beliefs, values and aspirations. As is true for everyone, it is important that individuals with spina bifida have opportunities to acquire developmentally appropriate, relevant and accurate sexual health knowledge. Those with spina bifida need to be able to negotiate sexual desire, intimacy and sexual expression. They also need education about their sexual health and how to limit the negative outcomes of sexual activity related to sexually transmitted infections, unplanned pregnancy or sexual coercion, violence, abuse or exploitation. This article discusses the Spina Bifida Sexual Health and Education Healthcare Guidelines from the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida using the World Health Organization's framing of sexual health and reviews the literature on sexual health and education for individuals with spina bifida.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Practice Guidelines as Topic; Sex Education; Sexual Health; Spinal Dysraphism; Young Adult
PubMed: 33361622
DOI: 10.3233/PRM-200743 -
Journal of Pediatric Rehabilitation... 2023This project aimed to launch an international learning community to guide the development of a spina bifida (SB) curriculum for global health trainees.
PURPOSE
This project aimed to launch an international learning community to guide the development of a spina bifida (SB) curriculum for global health trainees.
METHODS
Using a descriptive study design, a convenience sample of SB curricula were identified in 2022-23 by members of the Spina Bifida World Congress Outreach Committee and evaluated during a series of monthly Zoom calls to discuss SB education in a global health context. Participants included (1) leadership from the ReachAnother Foundation, (2) invited panelists from the Spina Bifida World Congress Global Health Symposium, and (3) global health students and preceptors. Education initiatives in Ethiopia, Sweden, Argentina, Ecuador, and the United States were evaluated vis-à-vis format and content.
RESULTS
All of the education initiatives referenced the framework of the World Health Organization International Classification of Functioning, Disability and Health. Formats varied and included both virtual and interactive workshops, print materials, videos, and guides for small group discussion. Content addressed four domains: Folate Prevention, Neurosurgical Training, After Care, and Data Collection. A multidisciplinary approach, partnerships with families, and workforce pipeline training were identified as guiding themes for educating the next generation of SB researchers and clinicians in global health settings.
CONCLUSION
The Spina Bifida Global Learning Collaborative is a transnational group of advocates, clinicians, and investigators whose mission is the advancement of SB-related global health education. Lessons learned from the collaborative are being leveraged to develop a global health curriculum for learners, which may improve services for individuals with SB around the globe.
Topics: Humans; Spinal Dysraphism; Global Health; Curriculum; Argentina; Sweden
PubMed: 38143399
DOI: 10.3233/PRM-230037 -
Child's Nervous System : ChNS :... Jun 2021A total of 43 Italian children, aged between 6 and 16 years, diagnosed with spina bifida, myelomeningocele, and shunted hydrocephalus have been described clinically and...
PURPOSE
A total of 43 Italian children, aged between 6 and 16 years, diagnosed with spina bifida, myelomeningocele, and shunted hydrocephalus have been described clinically and completed a neuropsychological battery in order to evaluate their cognitive, personality, and behavior profile.
METHODS
Enrolled children underwent cognitive assessment by means of the Weschler WISC-IV cognitive test and assessment of the attention sustained through the LEITER test. In addition, parents were asked, in order to obtain a personality and behavior profile of the children, to fill in a "CBCL 6-18 years" questionnaire and to fill in a Barthel Index questionnaire.
RESULTS
Processing Speed Index of the WISC-IV QI scale was statistically significant (p = 0.027), with the highest value presented by autonomous patients (95.8 ± 12.8) and the lowest by patients using a wheelchair (75.5 ± 19). WISC-IV QI mean value is 98 (±15.7) for lipoma patients and 78.7 (±17.6) for LMMC and MMC patients (p = 0.001). In more detail, Perceptual Reasoning (p < 0.0005), Working Memory (p = 0.01), and Processing Speed Index (p = 0.001) highlighted a significant difference between the groups. The attention sustained subscale of the LEITER presented a mean of 6.9 (±3.1) for lipoma patients and a men value of 4.6 (±3.1) for LMMC and MMC patients (p = 0.024). Patients with hydrocephalus had statistically significant worse cognition and autonomy (Barthel Index) score (p < 0.001) compared with those without hydrocephalus, and normal scores regarding attention and depression scales.
CONCLUSION
These results can be useful in planning dedicated therapeutic protocols such as suitable rehabilitation treatments, speech therapy, psychomotor skills, and cognitive enhancement and to develop prevention protocols particularly tailored for children with hydrocephalus who appear to have the more deficient skills.
Topics: Adolescent; Child; Humans; Hydrocephalus; Male; Meningomyelocele; Neuropsychological Tests; Spinal Dysraphism; Wechsler Scales
PubMed: 33709156
DOI: 10.1007/s00381-021-05089-9