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https://www.readbyqxmd.com/read/28716326/disease-specific-outcomes-of-botulinum-toxin-injections-for-neurogenic-detrusor-overactivity
#1
REVIEW
Aaron Kaviani, Rose Khavari
Intradetrusor injection of botulinum toxin A (BTX-A) is an effective option for managing patients with neurogenic detrusor overactivity (NDO) who do not respond to or tolerate oral pharmacologic agents. There is level I evidence that intradetrusor injection of onabotulinumtoxinA for refractory NDO in patients with multiple sclerosis and spinal cord injury is associated with a significantly greater achievement of goals and improved performance in urodynamic studies than placebo. Pilot studies or small case series support BTX-A for NDO in patients with Parkinson's disease and cerebrovascular accident...
August 2017: Urologic Clinics of North America
https://www.readbyqxmd.com/read/28716320/evaluation-and-lifetime-management-of-the-urinary-tract-in-patients-with-myelomeningocele
#2
REVIEW
Diana Cardona-Grau, George Chiang
In the United States, there are an estimated 25,000 children ages 0 to 19 years and about 166,000 of all ages currently affected by spina bifida. Management is multimodal and can be complex. Management techniques vary throughout a child's lifetime, but the goals remain the same: prevention of urinary tract infections and establishing acceptable continence. Continence is addressed as the child reaches school age. Additional considerations such as development of urolithiasis and the associated burden are highlighted in adolescence and into adulthood...
August 2017: Urologic Clinics of North America
https://www.readbyqxmd.com/read/28714784/the-management-of-paediatric-neurogenic-bladder-an-approach-in-a-resource-poor-setting
#3
Patrick Opoku Manu Maison, John Lazarus
BACKGROUND: If untreated, paediatric neurogenic bladder can cause renal failure and urinary incontinence. It is usually caused by neural tube defects such as myelomeningocele. Children with a neurogenic bladder should be monitored from birth and management should aim to preserve renal function and achieve social continence. This article outlines the management options appropriate for these children in resource-poor settings. ASSESSMENT: In most low- and middle-income countries, a general lack of awareness of the neurological effects on the urinary tract results in late presentation, usually with urological complications even when spina bifida is diagnosed early...
July 17, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28689244/changes-in-management-of-poorly-compliant-bladder-in-botulinum-toxin-a-era
#4
REVIEW
Nachiketh Soodana Prakash, Diana M Lopategui, Christopher Gomez
Bladder compliance is a measure of distensibility. Maladies such as myelodysplasia, myelomeningocele, spinal cord injury, multiple sclerosis and obstructive uropathy are known to decrease bladder compliance. Decrease in bladder compliance is a characteristic of neurogenic bladders. The pathophysiology of bladder compliance is complex but ultimately leads to high pressure during filling and storage phases. These high pressures lead to renal impairment, incontinence, and recurrent urinary tract infections. This review presents management of poorly compliant bladders with onabotulinumtoxinA...
August 2017: Current Urology Reports
https://www.readbyqxmd.com/read/28687411/systematic-review-of-bladder-cancer-outcomes-in-patients-with-spina-bifida
#5
K O Rove, D A Husmann, D T Wilcox, G J Vricella, T T Higuchi
BACKGROUND: In patients with congenital bladder anomalies, bladder augmentation is used as a last resort to reduce intravesical pressure, but concerns about malignant transformation in augmented patients were first raised in the 1980s. The best evidence to date indicates that augmentation does not appear to increase the risk of bladder cancer in spina bifida patients. To date, oncologic outcomes from patients with spina bifida with and without augmentation have only been available in small case reports...
June 1, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28686331/neural-tube-defects-in-waardenburg-syndrome-a-case-report-and-review-of-the-literature
#6
Joseph Hart, Kalpana Miriyala
Waardenburg syndrome type 1 (WS1) is an autosomal dominant genetic condition characterized by sensorineural deafness and pigment abnormalities, and is caused by variants in the PAX3 homeodomain. PAX3 variants have been associated with severe neural tube defects in mice and humans, but the frequency and clinical manifestations of this symptom remain largely unexplored in humans. Consequently, the role of PAX3 in human neural tube formation remains a study of interest, for clinical as well as research purposes...
July 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28676974/long-term-evaluation-of-intraoperative-neurophysiological-monitoring-assisted-tethered-cord-surgery
#7
S E Dulfer, G Drost, F Lange, H L Journee, F H Wapstra, E W Hoving
PURPOSE: Patients with tethered spinal cord have been investigated for short-term effects after tethered spinal cord surgery in the past. However, little is known about the long-term effects in this patient group. In this retrospective, longitudinal, observational study, a patient sample of a previous report of 65 patients was reassessed to observe the long-term effects of intraoperative neurophysiological monitoring-assisted tethered cord surgery. METHODS: With the use of patient charts and a survey, patients were scored on four domains: (1) neurological deficits, (2) urological deficits, (3) pain symptoms, and (4) orthopedic deficits...
July 4, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28673062/long-term-outcomes-of-augmentation-enterocystoplasty-in-patients-with-end-stage-bladder-diseases-a-single-institute-experience-involving-102-patients
#8
Shu-Yu Wu, Yuan-Hong Jiang, Hann-Chorng Kuo
PURPOSE: Augmentation enterocystoplasty (AE) has been shown to improve clinical symptoms in patients with end-stage bladder disease (ESBD). Herein, we report the long-term outcomes of a series of patients with different etiologies of ESBD who received AE. METHODS: We retrospectively reviewed 102 patients with ESBD who received AE at the Hualien Tzu Chi General Hospital from 1992 to 2014. ESBD in this study was defined as including neurogenic lower urinary tract dysfunction (NLUTD) due to spinal cord injury (SCI) or myelomeningocele, inflammatory bladder disease (IBD), ESBD occurring after pelvic cancer surgery, and other etiologies...
June 2017: International Neurourology Journal
https://www.readbyqxmd.com/read/28668954/lipoencephalocele-in-a-child-an-uncommon-swelling-in-the-occipital-region
#9
Ajay Kumar Verma, Anand Pandey, Digamber Chaubey, Kanoujia Sunil, Archika Gupta, Shiv Narain Kureel
Neural tube defects are common congenital malformations of the central nervous system. The 3 most common neural tube defects are anencephaly, myelomeningocele, and encephalocele. Lipoencephalocele is an extremely uncommon entity with sporadic reports in the literature. We treated a 4-year-old gild with occipital lipoencephalocele. This report presents the clinical presentation and management of the patient along with a review of the relevant literature.
July 1, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28656385/health-related-quality-of-life-in-non-paraplegic-ambulatory-children-with-myelomeningocele
#10
Huseyin Canaz, Gokhan Canaz, Irem Dogan, Ibrahim Alatas
PURPOSE: Evaluation of the effects of ventriculoperitoenal shunt and incontinence presence on health-related quality of life of ambulatory myelomeningocele patients. METHODS: The study group included 35 myelomeningocele patients, between 5 and 18 years old (mean age = 9.6), who were neonatally operated. All patients were ambulatory. The Child Edition of the Child Health and Illness Profile (CHIP-CE) used to evaluate the patient group. Seventeen patients were using clean intermittent catheterization and nine patients had ventriculoperitoneal shunt...
June 27, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28655526/agreement-between-electronic-medical-records-and-self-reported-urologic-domains-in-the-national-spina-bifida-patient-registry-nsbpr-implications-for-future-research
#11
Christopher G Halline, Theresa Meyer, Ilina Rosoklija, Elizabeth B Yerkes
BACKGROUND: Self-report (SR) is an efficient data collection method. However, SR data have been shown to be discrepant with medical record (MR) documentation, which raises questions about using SR to supplement retrospective chart review in research. In this study, pediatric spina bifida (SB) patients who completed SR interviews about continence status and personal bladder/bowel management were identified. We examined agreement between SR data and Urology provider notes in MRs. OBJECTIVE: This study aimed to (1) identify demographic, medical, or methodological factors that might contribute to SR/MR disagreement; (2) postulate how these findings might be significant clinically; and (3) recommend improvements to SR data collection and MR documentation...
June 15, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28617430/physician-views-regarding-the-benefits-and-burdens-of-prenatal-surgery-for-myelomeningocele
#12
R M Antiel, C A Collura, A W Flake, M P Johnson, N E Rintoul, J D Lantos, F A Curlin, J C Tilburt, S D Brown, C Feudtner
OBJECTIVE: Examine how pediatric and obstetrical subspecialists view benefits and burdens of prenatal myelomeningocele (MMC) closure. STUDY DESIGN: Mail survey of 1200 neonatologists, pediatric surgeons and maternal-fetal medicine specialists (MFMs). RESULTS: Of 1176 eligible physicians, 670 (57%) responded. Most respondents disagreed (68%, 11% strongly) that open fetal surgery places an unacceptable burden on women and their families. Most agreed (65%, 10% strongly) that denying the benefits of open maternal-fetal surgery is unfair to the future child...
June 15, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28600017/comparison-of-prenatal-and-postnatal-management-of-patients-with-myelomeningocele
#13
REVIEW
Sergio Cavalheiro, Marcos Devanir Silva da Costa, Antonio Fernandes Moron, Jeffrey Leonard
Myelomeningocele (MMC) is a costly lifetime disease with many comorbidities, including sensory and motor lower limb disability, bladder/bowel dysfunction, scoliosis, club foot, and hydrocephalus. MMC treatment options have changed over time because routine use of fetal ultrasonography and MRI has provided prenatal diagnosis and the potential for fetal surgery. There is still no consensus on how to treat the MMC diagnoses prenatally, mainly related to the infrastructure required to operate on pregnant patients...
July 2017: Neurosurgery Clinics of North America
https://www.readbyqxmd.com/read/28591652/fetal-therapy-model-of-myelomeningocele-with-three-dimensional-skin-using-amniotic-fluid-cell-derived-induced-pluripotent-stem-cells
#14
Kazuhiro Kajiwara, Tomohiro Tanemoto, Seiji Wada, Jurii Karibe, Norimasa Ihara, Yu Ikemoto, Tomoyuki Kawasaki, Yoshie Oishi, Osamu Samura, Kohji Okamura, Shuji Takada, Hidenori Akutsu, Haruhiko Sago, Aikou Okamoto, Akihiro Umezawa
Myelomeningocele (MMC) is a congenital disease without genetic abnormalities. Neurological symptoms are irreversibly impaired after birth, and no effective treatment has been reported to date. Only surgical repairs have been reported so far. In this study, we performed antenatal treatment of MMC with an artificial skin using induced pluripotent stem cells (iPSCs) generated from a patient with Down syndrome (AF-T21-iPSCs) and twin-twin transfusion syndrome (AF-TTTS-iPSCs) to a rat model. We manufactured three-dimensional skin with epidermis generated from keratinocytes derived from AF-T21-iPSCs and AF-TTTS-iPSCs and dermis of human fibroblasts and collagen type I...
June 6, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28586935/endoscopic-management-of-arnold-chiari-malformation-type-i-with-or-without-syringomyelia
#15
Shailendra Ratre, Nishtha Yadav, Yad Ram Yadav, Vijay Singh Parihar, Jitin Bajaj, Yatin Kher
Introduction Several different surgical techniques have been used in the treatment of patients with symptomatic Arnold-Chiari malformation type 1 (ACM-1) with or without syrinx. Endoscope-assisted decompression of the posterior fossa has been found to be safe and effective. We report our initial experience of endoscopic management of ACM-I. Material and Methods This was a prospective study of 15 symptomatic patients. Pre- and postoperative clinical status and computed tomography and magnetic resonance imaging findings were recorded...
June 6, 2017: Journal of Neurological Surgery. Part A, Central European Neurosurgery
https://www.readbyqxmd.com/read/28583853/lipomyelomeningocele-for-the-urologist-should-we-view-it-the-same-as-myelomeningocele
#16
E B Yerkes, C Halline, G Yoshiba, T A Meyer, I Rosoklija, R Bowman, D McLone, E Y Cheng
INTRODUCTION: The primary urologic objectives for lipomyelomeningocele (LMM) and myelomeningocele (MM) are preserving renal integrity and achieving continence. Due to this common ground, LMM and MM are urologically treated the same. However, unlike MM, LMM may present with no evident functional concerns. Indications for and timing of tethered cord release (TCR) in LMM are therefore controversial. Long-term urologic outcomes are not well defined. OBJECTIVE: Expectations for continence and potential for intermittent catheterization (CIC) following TCR in LMM are important for realistically counseling families regarding future needs...
May 22, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28578005/isolation-characterization-and-differentiation-of-multipotent-neural-progenitor-cells-from-human-cerebrospinal-fluid-in-fetal-cystic-myelomeningocele
#17
Mario Marotta, Alejandra Fernández-Martín, Marc Oria, Cesar G Fontecha, Carles Giné, Vicente Martínez-Ibáñez, Elena Carreras, Michael A Belfort, Gloria Pelizzo, Jose L Peiró
Despite benefits of prenatal in utero repair of myelomeningocele, a severe type of spina bifida aperta, many of these patients will still suffer mild to severe impairment. One potential source of stem cells for new regenerative medicine-based therapeutic approaches for spinal cord injury repair is neural progenitor cells (NPCs) in cerebrospinal fluid (CSF). To this aim, we extracted CSF from the cyst surrounding the exposed neural placode during the surgical repair of myelomeningocele in 6 fetuses (20 to 26weeks of gestation)...
May 15, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28569523/mastery-motivation-and-executive-functions-as-predictors-of-adaptive-behavior-in-adolescents-and-young-adults-with-cerebral-palsy-or-myelomeningocele
#18
Seth Warschausky, Jacqueline N Kaufman, Michael Evitts, William Schutt, Edward A Hurvitz
PURPOSE/OBJECTIVE: To examine mastery motivation and executive functions or behaviors as predictors of adaptive behavior in adolescents and young adults with congenital neurodevelopmental conditions. METHOD: Participants were 2 groups of adolescents and young adults, ages 13-29, including 43 with cerebral palsy and 36 with myelomeningocele living with a parent or caregiver. Participants completed measures of mastery motivation, executive functions or behaviors, and a measure of adaptive behavior...
June 1, 2017: Rehabilitation Psychology
https://www.readbyqxmd.com/read/28559064/endoscopic-third-ventriculostomy-for-the-treatment-of-hydrocephalus-in-a-paediatric-population-with-myelomeningocele
#19
Joana Rei, Josué Pereira, Carina Reis, Sérgio Salvador, Rui Vaz
BACKGROUND: Hydrocephalus develops in up to 90% of patients born with myelomeningocele. Although Endoscopic Third Ventriculostomy (ETV) is currently considered the preferred treatment for obstructive hydrocephalus, its results have been inconsistent in patients with myelomeningocele. This study focuses on clinical and radiological outcomes of ETV in children with hydrocephalus related to myelomeningocele. METHODS: Medical records of 18 paediatric patients with myelomeningocele submitted to ETV from 1998 to 2015, at the Centro Hospitalar S...
May 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28555310/antenatal-management-of-fetal-neurosurgical-diseases
#20
Sergio Cavalheiro, Marcos Devanir Silva da Costa, Jardel Nicacio Mendonça, Patricia Alesssandra Dastoli, Italo Capraro Suriano, Mauricio Mendes Barbosa, Antonio Fernandes Moron
The advance in the imaging tools during the pregnancy (ultrasound and magnetic resonance) allowed the early diagnose of many fetal diseases, including the neurological conditions. This progress brought the neurosurgeons the possibility to propose treatments even before birth. Myelomeningocele is the most recognized disease that can be treated during pregnancy with a high rate of success. Additionally, this field can be extended to other conditions such as hydrocephalus and encephaloceles. However, each one of these diseases has nuances in the diagnostic evaluation that should fit the requirements to perform the fetal procedure and overbalance the benefits to the patients...
July 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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