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Developmental Medicine and Child... Mar 2021
Topics: Brain; Humans; Nervous System Malformations; Spinal Dysraphism
PubMed: 33236354
DOI: 10.1111/dmcn.14745 -
Disability and Health Journal Jan 2021Self-management is critical to optimizing the health of individuals with a chronic condition or disability and is, therefore, a central concept in individual and... (Review)
Review
BACKGROUND
Self-management is critical to optimizing the health of individuals with a chronic condition or disability and is, therefore, a central concept in individual and family-centered healthcare delivery. The purpose of this review is to report the state of the science of self-management for individuals with spina bifida (SB) from a lifespan perspective.
OBJECTIVE
This review will summarize the (a) development and use of self-management skills and behaviors across the life span, (b) factors related to self-management behaviors, (c) development of generic or condition-specific measures of self-management used with a spina bifida population, and (d) development and/or outcomes of interventions to improve self-management in SB.
METHODS
The search strategy was limited to primary research articles published between 2003 and 2019 and followed PRISMA guidelines. The databases searched included: PubMed, CINAHL, PsycINFO, Web of Science, Cochrane, and Google Scholar. Studies that addressed self-management concepts in individuals throughout the life span and published in English were included.
RESULTS
The search yielded 108 citations and 56 articles met inclusion/exclusion criteria. A systematic narrative synthesis was reported. The level of evidence identified was primarily Level III articles of good quality. Multiple demographic, environmental, condition and process factors were related to self-management behaviors. SB self-management instruments and intervention development and testing studies were identified.
CONCLUSIONS
This review provides a synthesis of the state of the science of self-management including factors related to self-management behaviors, preliminary evidence of instruments for use in SB, factors important to consider in the development and testing of future interventions, and gaps in the literature.
Topics: Chronic Disease; Delivery of Health Care; Disabled Persons; Humans; Self-Management; Spinal Dysraphism
PubMed: 32980287
DOI: 10.1016/j.dhjo.2020.100940 -
Advances in Experimental Medicine and... 2020Transamniotic stem cell therapy (TRASCET) is a novel prenatal therapeutic alternative for the treatment of congenital anomalies. It is based upon the principle of... (Review)
Review
Transamniotic stem cell therapy (TRASCET) is a novel prenatal therapeutic alternative for the treatment of congenital anomalies. It is based upon the principle of augmenting the pre-existing biological role of select populations of fetal stem cells for targeted therapeutic benefit. For example, amniotic fluid-derived mesenchymal stem cells (afMSCs) play an integral role in fetal tissue repair, validating the use of afMSCs in regenerative strategies. The simple intra-amniotic delivery of these cells in expanded numbers via TRASCET has been shown to promote the repair of and/or significantly ameliorate the effects associated with major congenital anomalies such as neural tube and abdominal wall defects. For example, TRASCET can induce partial or complete coverage of experimental spina bifida through the formation of a host-derived rudimentary neoskin, thus protecting the spinal cord from further damage secondary to amniotic fluid exposure. Furthermore, TRASCET can significantly reduce the bowel inflammation associated with gastroschisis, a common major abdominal wall defect. After intra-amniotic injection, donor stem cells home to the placenta and the fetal bone marrow in the spina bifida model, suggesting a role for hematogenous cell routing rather than direct defect seeding. Therefore, the expansion of TRASCET to congenital diseases without amniotic fluid exposure, such as congenital diaphragmatic hernia, as well as to maternal diseases, is currently under investigation in this emerging and evolving field of fetal stem cell therapy.
Topics: Amnion; Amniotic Fluid; Female; Fetal Diseases; Humans; Mesenchymal Stem Cell Transplantation; Mesenchymal Stem Cells; Pregnancy; Spinal Dysraphism
PubMed: 31302870
DOI: 10.1007/5584_2019_416 -
World Neurosurgery Nov 2022Transition of care is the planned movement of adolescents and young adults from pediatric to adult health care. Many studies have demonstrated the importance of an... (Randomized Controlled Trial)
Randomized Controlled Trial
OBJECTIVES
Transition of care is the planned movement of adolescents and young adults from pediatric to adult health care. Many studies have demonstrated the importance of an organized transition process. The purpose of this study is to determine the efficacy of a telemedicine intervention for improving transition readiness among adolescents with spina bifida.
METHODS
The present study is a randomized, controlled trial, including children 14 years of age and older with myelomeningocele from a multidisciplinary spina bifida clinic. Subjects were randomized to standard care or to an intervention, consisting of video telemedicine contacts at 3, 6, and 9 months from the clinic visit. The primary outcome measure was a change in the Transition Readiness Assessment Questionnaire score from baseline to 1-year follow-up.
RESULTS
Twenty-four patients were enrolled in the study and underwent randomization. The mean age at enrollment was 15.8 years. Ten patients (40%) were female, and the majority were White, non-Hispanic (67%). Despite enrolling 24 patients, only 1 patient in the telemedicine group completed any of the planned telemedicine sessions. No other participant completed any telemedicine counseling sessions. The study was stopped early for lack of participation in the intervention. In a single-group, as-treated analysis, there was no significant change in the Transition Readiness Assessment Questionnaire score between enrollment and 1-year follow-up (Δ = 0.36, P = 0.46). However, there were significant improvements in subscores for Managing Medications, Appointment Keeping, and Managing Daily Activities.
CONCLUSIONS
The primary finding from this study was very low participation in a telemedicine video follow-up intervention among adolescents with myelomeningocele. Based on these results, this strategy alone is unlikely to significantly improve readiness for transition to adult care.
Topics: Adolescent; Young Adult; Humans; Male; Child; Female; Meningomyelocele; Pilot Projects; Transition to Adult Care; Spinal Dysraphism; Telemedicine
PubMed: 36156308
DOI: 10.1016/j.wneu.2022.09.076 -
Journal of Neurosurgery. Pediatrics Aug 2019The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early... (Review)
Review
The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early morphological changes in the fetus diagnosed with myelomeningocele. The Management of Myelomeningocele Study (MOMS) demonstrated reduced need for shunting and improved neurological function in patients treated in utero relative to postnatally, thereby offering level 1 evidence supporting fetal repair. Subsequent studies have offered additional information about urological, orthopedic, radiological, and maternal factors surrounding fetal repair. The quest for robust long-term neurocognitive and motor function data is underway and poised to shape the future of fetal repair. In addition, technical innovations such as fetoscopic surgery aim to minimize maternal morbidity while conferring the beneficial effects observed with open intrauterine intervention.
Topics: Adolescent; Adolescent Development; Animals; Cerebrospinal Fluid Shunts; Child; Child Development; Cysts; Disease Models, Animal; Fetoscopy; Humans; Hydrocephalus; Infant, Newborn; Infant, Premature; Medical Illustration; Meningomyelocele; Multicenter Studies as Topic; Neural Tube Defects; Patient Positioning; Photography; Postoperative Complications; Quality of Life; Randomized Controlled Trials as Topic; Spinal Dysraphism; Treatment Outcome; Wound Closure Techniques
PubMed: 31370010
DOI: 10.3171/2019.4.PEDS18383 -
The Urologic Clinics of North America Aug 2022We present a phenotype-based approach to neurogenic bladder (NGB) by describing prototypical patients with spinal cord injury (SCI), spina bifida (SB), cerebral palsy... (Review)
Review
We present a phenotype-based approach to neurogenic bladder (NGB) by describing prototypical patients with spinal cord injury (SCI), spina bifida (SB), cerebral palsy (CP), and multiple sclerosis (MS). Surgical management is categorized by failure to store and failure to empty, with a focus on catheterizable channels, bladder augmentation, and bladder outlet procedures. Mitigation and management of common complications are reviewed. Specific attention is paid to social support, body habitus, and extremity function, as we believe a holistic approach is necessary for appropriate surgical selection.
Topics: Humans; Spinal Cord Injuries; Spinal Dysraphism; Urinary Bladder; Urinary Bladder, Neurogenic; Urologic Surgical Procedures
PubMed: 35931441
DOI: 10.1016/j.ucl.2022.04.010 -
Journal of Medicine and Life 2021Spina bifida is a disorder characterized by failure of the neural tube to form during embryological development. The early signs in the head and spine may be detected on...
Spina bifida is a disorder characterized by failure of the neural tube to form during embryological development. The early signs in the head and spine may be detected on ultrasound from 11 weeks of gestation. Diabetes is a well-known teratogen factor that increases the chances of birth defects, such as neural tube defects. We report a 12 weeks case of spina bifida in type 1 diabetes.
Topics: Diabetes Mellitus; Female; Humans; Neural Tube Defects; Pregnancy; Spinal Dysraphism; Ultrasonography
PubMed: 35126760
DOI: 10.25122/jml-2021-0249 -
Child's Nervous System : ChNS :... Sep 2022Open spina bifida (open SB) is the most complex congenital abnormality of the central nervous system compatible with long-term survival. Multidisciplinary care is... (Review)
Review
Open spina bifida (open SB) is the most complex congenital abnormality of the central nervous system compatible with long-term survival. Multidisciplinary care is required to address the effect of this disease on the neurological, musculoskeletal, genitourinary, and gastrointestinal systems, as well as the complex psychosocial impact on the developing child. Individuals with SB benefit from the involvement of neurosurgeons, orthopedic surgeons, urologists, physical medicine and rehabilitation specialists, pediatricians, psychologists, physical/occupational/speech therapists, social workers, nurse coordinators, and other personnel. Multidisciplinary clinics are the gold standard for coordinated, optimal medical and surgical care. Ann and Robert H. Lurie Children's Hospital, formerly known as Children's Memorial Hospital, was one of the first hospitals in the USA to manage patients with this complex disease in a multidisciplinary manner. We describe the longitudinal experience of the multidisciplinary Spina Bifida Center at our institution and highlight the advances that have arisen from this care model over time. This clinic serves as an exemplar of organized, effective, and patient-centered approach to the comprehensive care of people living with open SB.
Topics: Chicago; Child; Humans; Neurosurgeons; Spina Bifida Cystica; Spinal Dysraphism
PubMed: 35870009
DOI: 10.1007/s00381-022-05594-5 -
Pediatrics in Review Sep 2021Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but... (Review)
Review
Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but so too with their adult equivalents. Some of the most common diseases are autoimmune (ie, multiple sclerosis, acute disseminated encephalomyelitis, and acute transverse myelitis), congenital (ie, dysraphism with spina bifida, split cord malformation, and tethered cord syndrome), tumor (ie, juvenile pilocytic astrocytoma, ependymoma, and hem-angioblastoma), and vascular (ie, cavernous malformations, arteriovenous malformations, and dural arteriovenous fistulas) in nature. These each require their own niche treatment paradigm and prognosis. Furthermore, presentation of different spinal cord diseases in children can be difficult to discern without epidemiologic and imaging data. Interpretation of these data is crucial to facilitating a timely and accurate diagnosis. Correspondingly, the aim of this review was to highlight the most pertinent features of the most common spinal cord diseases in the pediatric population.
Topics: Adult; Child; Encephalomyelitis, Acute Disseminated; Humans; Magnetic Resonance Imaging; Myelitis, Transverse; Neural Tube Defects; Spinal Cord; Spinal Cord Diseases; Spinal Dysraphism
PubMed: 34470868
DOI: 10.1542/pir.2020-000661 -
Anesthesiology Clinics Sep 2020The most common congenital anomalies are congenital heart defects, cleft lip and palate, Down syndrome, and neural tube defects. Anesthetic considerations for Down... (Review)
Review
The most common congenital anomalies are congenital heart defects, cleft lip and palate, Down syndrome, and neural tube defects. Anesthetic considerations for Down syndrome include cervical spine instability, history of congenital heart disease, risk of bradycardia, hematologic, endocrine, and behavioral considerations. Patients with cleft lip and palate can have associated syndromes, and the potential for underlying abnormalities should be investigated prior to their anesthetic. A major anesthetic consideration for neural tube defect surgery is positioning for intubation. Fetal surgery for myelomeningocele has been shown to reduce the need for ventriculoperitoneal shunting and improved motor outcomes.
Topics: Anesthesia; Child, Preschool; Heart Defects, Congenital; Humans; Infant; Meningomyelocele; Mouth Abnormalities; Neural Tube Defects; Spinal Dysraphism
PubMed: 32792188
DOI: 10.1016/j.anclin.2020.06.002