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International Neurourology Journal Jun 2024In Korea, the field of transitional urology (TU) is in its nascent stages, with its introduction only beginning. This study aims to evaluate the existing state of TU...
PURPOSE
In Korea, the field of transitional urology (TU) is in its nascent stages, with its introduction only beginning. This study aims to evaluate the existing state of TU prior to implementing a transition protocol, and to identify key areas of focus for the development of an effective transition protocol.
METHODS
From June 1, 2021 to May 31, 2023, clinical data were retrospectively collected for patients who visited the adult urology or pediatric urology outpatient departments of this hospital and were aged 10 or older, with medical conditions falling under the category of TU. We analyzed the patient distribution across different disease groups. The transitional stages were categorized from T1, indicating initial care by pediatric urologists, to T4, denoting complete transition to adult care. 'T4x' was used for patients with unknown medical histories, and 'T4only' for those who had never been under pediatric urology care.
RESULTS
During a 2-year period, a total of 1,484 patients received outpatient care for diseases in TU field. The most prevalent diseases were hypospadias (40.4%), spinal bifida (37.3%), and congenital ureteral anomalies (17.7%), with other conditions accounting for 4.6%. Among 553 spinal bifida patients, only 5.3% completed transitional care (T4), while 80.1% were in the initial phase (T1). For patients introduced to adult urology (T2-T4), 37.7% reached T4, highlighting a marked increase in transition completion within this subset (P<0.001).
CONCLUSION
TU in Korea is in its nascent stage, with a significant gap in the initiation and completion of transitional care for patients with congenital urologic conditions. Early initiation and active engagement in transitional care are crucial for successful transition. This study highlights the need for structured transition protocols to address the complex needs of this patient population.
PubMed: 38956776
DOI: 10.5213/inj.2448186.093 -
Gait & Posture Jun 2024Individuals with myelomeningocele (MMC) present with neurological and orthopaedic deficiencies, requiring orthoses during walking. Orthoses for counteracting...
BACKGROUND
Individuals with myelomeningocele (MMC) present with neurological and orthopaedic deficiencies, requiring orthoses during walking. Orthoses for counteracting dorsiflexion may restrict activities such as rising from a chair.
RESEARCH QUESTION
How are sit-to-stand (STS) movements performed with ankle joint-restricted ankle-foot orthoses (AFO) and knee-ankle-foot orthoses with a free-articulated knee joint (KAFO-F)?
METHODS
Twenty-eight adults with MMC, mean age 25.5 years (standard deviation: 3.5 years), were divided into an AnkleFree group (no orthosis or a foot orthosis) and an AnkleRestrict group (AFOs or KAFO-Fs). Study participants performed the five times STS test (5STS) while their movements were simultaneously captured with a three-dimensional motion system. Centre of mass (CoM) trajectories and joint kinematics were analysed using statistical parametric mapping.
RESULTS
The AnkleRestrict group performed the STS slower than the AnkleFree group, median 8.8 s (min, max: 6.9, 14.61 s) vs 15.0 s (min, max: 7.5, 32.2 s) (p = 0.002), displayed reduced ankle dorsiflexion (mean difference: 6°, p = 0.044) (74-81 % of the STS cycle), reduced knee extension (mean difference: 14°, p = 0.002) (17-41 % of the STS cycle), larger anterior pelvic tilt angle (average difference: 11°, p = 0.024) (12-24 % of the STS cycle), and larger trunk flexion angle (on average 4°, p = 0.029) (6-15 % of the STS cycle).
SIGNIFICANCE
The differences between the AnkleFree and AnkleRestrict groups in performing the STS seem consistent with the participants functional ambulation: community ambulation in the AnkleFree group, and household and nonfunctional ambulation with less hip muscle strength in the majority of the AnkleRestrict group. No differences in the 5STS CoM trajectories or the kinematics were found with respect to the AFO and KAFO-Fs groups. Because orthoses are constructed to enable walking, the environment needs to be adjusted for activities in daily living such as the STS movement.
PubMed: 38954928
DOI: 10.1016/j.gaitpost.2024.06.025 -
JMIR Research Protocols Jul 2024Obesity prevalence in youth with spina bifida is higher than in their typically developing peers. Obesity is associated with lifelong medical, psychological, and...
BACKGROUND
Obesity prevalence in youth with spina bifida is higher than in their typically developing peers. Obesity is associated with lifelong medical, psychological, and economic burdens. Successful prevention or treatment of obesity in individuals with spina bifida is compromised by (1) the lack of valid and reliable methods to identify body fat in a clinical setting and (2) limited data on energy expenditure that are necessary to provide daily caloric recommendations.
OBJECTIVE
The objectives of this study will be to develop 2 algorithms for use in youth with spina bifida in a clinical setting, one to model body fat and one to predict total daily energy expenditure. In addition, physical activity and dietary intake will be described for the sample.
METHODS
This multisite, prospective, national clinical study will enroll 232 youth with myelomeningocele aged 5 to 18 years (stratified by age and mobility). Participants will be enrolled for 1 week. Data obtained include 4 measures of body composition, up to 5 height measures, a ramped activity protocol, and a nutrition and physical activity screener. Participants will wear an accelerometer for the week. On the final study day, 2 samples of urine or saliva, which complete the doubly labeled water protocol, will be obtained. The analysis will include descriptive statistics, Bland-Altman plots, concordance correlation, and regression analysis.
RESULTS
The study received extramural federal funding in July 2019. Data collection was initiated in March 2020. As of April 2024, a total of 143 (female participants: n=76, 53.1%; male participants: n=67, 46.9%) out of 232 participants have been enrolled. Data collection is expected to continue throughout 2024. A no-cost extension until November 2025 will be requested for data analysis and dissemination of findings.
CONCLUSIONS
This study furthers previous pilot work that confirmed the acceptability and feasibility of obtaining alternate height, body composition, and energy expenditure measures. The findings from this study will enhance screening, prevention, and treatment of abnormal weight status by facilitating the accurate identification of youths' weight status category and recommendations of daily caloric needs for this population that is at higher risk of obesity. Furthermore, the findings have the potential to impact outcomes for youth diagnosed with disabilities other than spina bifida who experience similar challenges related to alterations in body composition or fat distribution or measurement challenges secondary to mobility issues or musculoskeletal problems.
INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID)
DERR1-10.2196/52779.
Topics: Humans; Adolescent; Child; Spinal Dysraphism; Energy Metabolism; Cross-Sectional Studies; Body Composition; Female; Male; Child, Preschool; Prospective Studies; Exercise
PubMed: 38954458
DOI: 10.2196/52779 -
The Journal of Urology Jul 2024We aim to estimate the odds of urinary tract infection (UTI)-related hospital care in spina bifida (SB) patients age 18 to 25 years as compared with patients with SB in...
PURPOSE
We aim to estimate the odds of urinary tract infection (UTI)-related hospital care in spina bifida (SB) patients age 18 to 25 years as compared with patients with SB in adolescence (11-17 years) or adulthood (26-35 years). We hypothesize that patients with SB in the typical transitional age, 18 to 25 years, will have higher odds of UTI-related hospital care as compared to adolescent SB patients or adult SB patients.
MATERIALS AND METHODS
Using Cerner Real World Data, we performed a retrospective cohort analysis comparing SB patients to an age- and gender-matched controls. SB cases between 2015 and 2021 were identified and compared in 3 cohorts: 11 to 17 years (adolescents), 18 to 25 years (young adults [YA]), and 26 to 35 years (adults). Logistic regression analysis was used to characterize the odds of healthcare utilization.
RESULTS
Of the 5497 patients with SB and 77,466 controls identified, 1839 SB patients (34%) and 3275 of controls (4.2%) had at least 1 UTI encounter. UTI-related encounters as a proportion of all encounters significantly increased with age in SB patients (adolescents 8%, YA 12%, adult 15%, < .0001). Adjusting for race, sex, insurance and comorbidities, the odds of a UTI-related encounter in YA with SB was significantly higher than for adolescents with SB (adolescent OR = 0.65, 95% CI: 0.57-0.75, < .001). YA had lower odds of a UTI-related encounter as compared with adults with SB (adult OR = 1.31, 95% CI: 1.16-1.49, < .001).
CONCLUSIONS
Young adults with SB have higher odds of UTI-related hospital care than adolescents, but lower odds of UTI-related hospital care when compared with adults.
PubMed: 38950379
DOI: 10.1097/JU.0000000000004130 -
Magnetic Resonance Imaging Clinics of... Aug 2024This review covers the embryology, definition, and diagnosis of open spinal dysraphism with a focus on fetal ultrasound and MR imaging findings. Differentiating open... (Review)
Review
This review covers the embryology, definition, and diagnosis of open spinal dysraphism with a focus on fetal ultrasound and MR imaging findings. Differentiating open versus closed spinal dysraphic defects on fetal imaging will also be discussed. Current fetal surgery practices and imaging findings in the context of fetal surgery are also reviewed.
Topics: Humans; Magnetic Resonance Imaging; Female; Spine; Pregnancy; Prenatal Diagnosis; Spinal Dysraphism; Ultrasonography, Prenatal; Fetal Diseases
PubMed: 38944432
DOI: 10.1016/j.mric.2024.01.004 -
The Journal of Pediatrics Jun 2024To assess if ventricular size prior to shunting is correlated with neurodevelopmental outcomes in children with post-natal myelomeningocele closure.
OBJECTIVE
To assess if ventricular size prior to shunting is correlated with neurodevelopmental outcomes in children with post-natal myelomeningocele closure.
STUDY DESIGN
This was a retrospective review of children with post-natal surgical closure of myelomeningocele and neuropsychological testing between 2018 through 2023 at UCSF. Frontal-occipital horn ratio (FOHR) was measured immediately prior to shunt placement, or on the first study that reported ventricular stability for non-shunted patients. The primary outcome was full scale IQ (FSIQ) on the Weschler Intelligence Scale. Secondary outcomes included indices of the Weschler scale, the Global Executive Composite from the Behavior Rating Inventory of Executive Function, and the General Adaptive Composite from the Adaptive Behavior Assessment Scale. Uni- and multi-variable regression was used to determine if FOHR was correlated with neuropsychological scores.
RESULTS
Forty patients met inclusion criteria; 26 (65%) had shunted hydrocephalus. Age at neuropsychological testing was 10.9+/-0.6 years. FOHR was greater in the shunted group (0.64 vs 0.51, p<0.001). There were no differences in neuropsychological results between shunted and non-shunted groups. On univariable analysis, greater FOHR was associated with lower FSIQ (p=0.025) and lower Visual Spatial Index scores (p=0.013), which remained significant on multivariable analysis after adjusting for gestational age at birth, lesion level, shunt status, and shunt revision status (p=0.049 and p=0.006, respectively). Separate analyses by shunt status revealed that these effects were driven by the shunted group.
CONCLUSION
Greater FOHR prior to shunting was correlated with lower FSIQ and the VSI scores on the Weschler Intelligence Scales. Larger studies will be needed to explore further the relationship between ventricle size, hydrocephalus, and neurodevelopmental outcomes.
PubMed: 38944186
DOI: 10.1016/j.jpeds.2024.114167 -
Journal of Pediatric Orthopedics Jun 2024While the Ponseti method is the primary treatment for idiopathic clubfoot, its application in treating myelodysplastic clubfeet is less certain. Myelodysplastic clubfoot...
INTRODUCTION
While the Ponseti method is the primary treatment for idiopathic clubfoot, its application in treating myelodysplastic clubfeet is less certain. Myelodysplastic clubfoot tends to be more severe and difficult to treat. Although the Ponseti method can initially correct these cases, there is conflicting evidence about recurrence rates and the need for additional treatment. This study aims to assess the effectiveness of the Ponseti method in treating myelodysplastic clubfeet compared with idiopathic clubfeet over a 20-year period.
METHODS
The study conducted a retrospective review of medical records from patients treated for clubfoot at a single institution (2002 to 2021), comparing children with myelodysplastic and idiopathic clubfoot. Included patients were under 18, initially treated with Ponseti-casting, and had a minimum 2-year follow-up. Data on demographics, treatment details, recurrence, and Patient-reported Outcomes Measurement Information System (PROMIS) scores were analyzed.
RESULTS
Forty-nine myelodysplastic and 512 idiopathic clubfeet in 366 patients met the inclusion criteria. Myelodysplastic cases had a median age of 5 months at presentation versus 2 months for idiopathic cases (P=0.002). Initial correction was achieved in 95% of idiopathic and 87.8% of myelodysplastic feet (P=0.185). Recurrence rates were higher in the myelodysplastic cohort, 65.3% versus 44.1% (P=0.005). Surgery was necessary to treat recurrence in 59.2% of myelodysplastic and 37.7% of idiopathic cases, P=0.003. Follow-up was 3.9±1.8 years for myelodysplastic and 3.3±1.5 years for idiopathic feet, P=0.030. Myelodysplastic feet had lower PROMIS mobility scores; 31.94±7.56 versus 49.21±8.64, P<0.001.
CONCLUSIONS
To the best of our knowledge, we report the largest series of myelodysplastic clubfeet treated by Ponseti casting and the first to assess PROMIS data. Overall, the Ponseti method is as effective in obtaining initial correction in myelodysplastic clubfoot as it is in idiopathic clubfoot. However, myelodysplastic clubfeet has a higher risk of relapse and increased need for surgical interventions. Children with spina bifida may need closer follow-ups and more stringent adherence to bracing.
LEVEL OF EVIDENCE
Level III-therapeutic studies-investigating the results of treatment.
PubMed: 38934642
DOI: 10.1097/BPO.0000000000002745 -
Birth Defects Research Jun 2024On May 29, 2023, the 76th World Health Assembly (WHA) unanimously adopted the resolution entitled, "Accelerating efforts for preventing micronutrient deficiencies and...
World health assembly resolution for preventing micronutrient deficiencies and associated neural tube defects-A case study of global partnerships for a successful resolution adoption.
BACKGROUND
On May 29, 2023, the 76th World Health Assembly (WHA) unanimously adopted the resolution entitled, "Accelerating efforts for preventing micronutrient deficiencies and their consequences, including spina bifida and other neural tube defects, through safe and effective food fortification." The Society for Birth Defects Research and Prevention published their resolution in 2015 supporting mandatory fortification of staple foods with folic acid and recommendations aiming to achieve global total prevention of folate-sensitive spina bifida and anencephaly, setting a goal to achieve by the year 2024. The WHA resolution provides another global push for the cause, with recommendations to member nations for food fortification to be achieved by the year 2030.
METHODS
This short communication documents the steps, from inception up to the passage, of the 76th WHA resolution on food fortification, with a narrative on the nature of strategic advocacy efforts by multiple governmental and nongovernmental organizations.
RESULTS
WHA resolutions can take many years to be introduced and passed by the assembly; however, this is a case study of the swiftness of the process enabled by powerful global partnership.
CONCLUSION
The documentation of this process serves as an example for developing and processing future WHA resolutions aiming to improve global maternal and child health.
Topics: Humans; Neural Tube Defects; Micronutrients; Global Health; Food, Fortified; Folic Acid; World Health Organization; International Cooperation
PubMed: 38923368
DOI: 10.1002/bdr2.2375 -
Cureus May 2024Background Spinal dysraphism, characterized by incomplete closure of neural and bone spinal structures, manifests as congenital fusion abnormalities along the dorsal...
Background Spinal dysraphism, characterized by incomplete closure of neural and bone spinal structures, manifests as congenital fusion abnormalities along the dorsal midline, involving the skin, subcutaneous tissue, meninges, vertebrae, and neural tissue. Magnetic resonance imaging (MRI), the preferred imaging modality for assessing spinal dysraphism across all age groups, provides direct visualization of the spinal cord without the need for contrast or ionizing radiation while also eliminating bone artifacts and allowing multiplanar imaging. The objective of this study was to evaluate the range of spinal dysraphism lesions and assess the significance of MRI in their evaluation. Methodology Thirty patients with suspected spinal dysraphism underwent evaluation at the Medical College Hospital and Study Centre in Vijayapur, India. This cross-sectional observational study included patients diagnosed or provisionally diagnosed with spinal dysraphism based on clinical and imaging profiles. Cases were identified through preliminary findings on radiographs. Results The study encompassed individuals aged one month to 20 years, with the largest proportion of patients (36.67%) falling within the 1-5-year age group. Spina bifida was the most prevalent spinal abnormality, accounting for 70% of cases. In 12 patients (40%), the most prevalent location of involvement was the lumbosacral spine. Conclusion MRI provides excellent tissue differentiation, particularly of lipomatous tissue, with reproducible and comprehensive section planes and relative operator independence. Moreover, MRI is beneficial for children with suspected spinal dysraphism as it can be performed without ionizing radiation, biological risks, or the need for intrathecal contrast media.
PubMed: 38916024
DOI: 10.7759/cureus.60972 -
Urology Practice Jun 2024Our goal was to better understand the health care utilization of patients with spina bifida (SB) by evaluating length of hospital stay (LOS) as well as identifying what...
INTRODUCTION
Our goal was to better understand the health care utilization of patients with spina bifida (SB) by evaluating length of hospital stay (LOS) as well as identifying what characteristics within the SB population are contributing to shorter or longer LOS.
METHODS
By querying the Department of Health Care Access and Information database of all encounters at California-licensed hospitals from January 1995 through December 2017, this study analyzed LOS as a measure of health care utilization. Patients with SB were identified using the International Classification of Diseases-9 and -10 coding system, and the data collected for both SB and control cohorts were compared using linear and logistic regression models.
RESULTS
Patients with SB spent a mean LOS of 7.3 days compared to 4.7 days among the control cohort ( < .001). In multivariable analysis, SB was found to be an independent predictor of longer LOS. Within the SB encounters, increasing comorbidities and nonprivate insurance were associated with longer LOS, while being female and Hispanic were associated with a shorter LOS.
CONCLUSIONS
SB is an independent predictor of longer LOS when compared to the control cohort. These findings highlight the importance of understanding the preventive health care access and needs of the vulnerable SB population to decrease hospital utilization rates.
PubMed: 38913592
DOI: 10.1097/UPJ.0000000000000632