Risk of fracture prevention in spina bifida patients: correlation between bone mineral density, vitamin D, and electrolyte values. (2015) Martinelli V, Dell'Atti C, Ausili E, Federici E, Magarelli N, Leone A, Massimi L, Di Rocco C, Bonomo L, Rendeli C.
Childs Nerv Syst. 2015 May 1.Monday, May 11, 2015
Past and current use of walking measures for children with spina bifida: a systematic review.
Bisaro DL, Bidonde J, Kane KJ, Bergsma SA, Musselman KE. (2015) Past and current use of walking measures for children with spina bifida: a systematic review.
Arch Phys Med Rehabil. 2015 May 2. pii: S0003-9993(15)00384-6. doi: 10.1016/j.apmr.2015.04.014Abstract
OBJECTIVE:
To describe walking measurement in children with spina bifida, and to identify patterns in the use of walking measures in this population.DATA SOURCES:
Seven medical databases were searched from inception until March 2014. Search terms encompassed three themes: 1) children, 2) spina bifida, and 3) walking.STUDY SELECTION:
Articles were included if participants were children aged 1-17 years with spina bifida, and if walking was measured. Articles were excluded if the assessment was restricted to kinematic, kinetic or electromyographic analyses of walking. A total of 1,751 abstracts were screened by two authors independently, and 109 articles were included in this review.DATA EXTRACTION:
Data were extracted using standardized forms. Extracted data included study and participant characteristics, and details about the walking measures used, including psychometric properties. Two authors evaluated the methodological quality of articles using a previously published framework that considers sampling method, study design, and psychometric properties of the measures used.DATA SYNTHESIS:
Nineteen walking measures were identified. Ordinal-level rating scales (e.g., Hoffer Functional Ambulation Scale) were most commonly used (57% of articles), followed by ratio-level, spatiotemporal measures, such walking speed (18% of articles). Walking was measured for a variety of reasons relevant to multiple health care disciplines. A machine learning analysis was used to identify patterns in the use of walking measures. The learned classifier predicted whether or not a spatiotemporal measure was used with 77.1% accuracy. A trend to use spatiotemporal measures in older children and those with lumbar and sacral spinal lesions was identified. Most articles were prospective studies that used samples of convenience and unblinded assessors. Few articles evaluated or considered the psychometric properties of the walking measures.CONCLUSIONS:
Despite a demonstrated need to measure walking in children with spina bifida, few valid, reliable and responsive measures have been established for this population.Copyright © 2015 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.
The Severity of Bowel Dysfunction in Patients with Neurogenic Bladder.
Cameron AP, Rodriguez GM, Gursky A, He C, Clemens JQ, Stoffel JT. (2015) The Severity of Bowel Dysfunction in Patients with Neurogenic Bladder.
J Urol. 2015 May 5. pii: S0022-5347(15)03905-1. doi: 10.1016/j.juro.2015.04.100Abstract
PURPOSE:
Patients with neurological conditions often suffer from severe debilitating lower urinary and bowel dysfunction in addition to their physical disabilities. However, only the bladder has received the attention of medical providers with neurogenic bowel being poorly understood and characterized.MATERIALS AND METHODS:
This is a cross-sectional analysis of a prospective institutional Neurogenic Bladder Database from 2010-2013.RESULTS:
Among the 175 patients 60.6% had traumatic spinal cord injury (SCI) and 18.3% multiple sclerosis. Fecal Incontinence Severity Index (FISI) scores were a median of 18.0±1.39 (moderate). Neurogenic Bowel Dysfunction (NBD) score were a median of 11.0±0.63 (moderate). NBD Scores were worse in those patients with SCI and spina bifida compared to other diseases (P=0.020), in younger patients (p=0.020) and in the SCI group those with higher levels of injury (p=0.0046). Based on the Bristol stool scale 65% of patient had abnormal stool consistency, mostly constipation. None of the FISI, Bristol or NBD scores correlated significantly with SF-12 quality of life measures. However, both of the bladder symptom scores Michigan Incontinence Symptom Index (M-ISI)(p=0.05) and the AUA-SI (p=0.03) correlated with FISI severity and the NBD score correlated with the M-ISI (ρ=0.29, p=0.02). Those patients with abnormal stool consistency on the Bristol reported more urgency and stress incontinence on M-ISI.CONCLUSIONS:
Bowel dysfunction is very common among patients with neurogenic bladder. Those patients with worse bladder symptoms also suffered from worse bowel dysfunction. This highlights the importance of addressing both bowel and bladder dysfunction in this often poorly understood population.Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
Examination of the relationship between body mass index (BMI) and functional independence level in children with spina bifida.
Şimşek TT, Türkücüoğlu B, Tezcan S. (2015) Examination of the relationship between body mass index (BMI) and functional independence level in children with spina bifida.
Dev Neurorehabil. 2015 Jun;18(3):149-54. doi: 10.3109/17518423.2013.796419Abstract
OBJECTIVE:
The aim of this study is to examine the relationship between body mass index (BMI) and functional independence level in children with spina bifida (SB).METHODS:
The study included 116 children between 5 and 18 years. Subjects' socio-demographic characteristics, BMI values and functional independence levels were recorded.RESULTS:
There was negative correlation between BMI and communication and social cognition in girls (p < 0.05). No correlation was found between BMI and WeeFIM in boys (p > 0.05).CONCLUSION:
The results indicated that body weight in children with SB affected functional independency and that precautions to control weight important in these children.Tuesday, June 10, 2014
Systematic Review and Meta-Analysis: Parent and Family-Based Interventions for Children and Adolescents With Chronic Medical Conditions.
Law EF, Fisher E, Fales J, Noel M, Eccleston C. (2014) Systematic
Review and Meta-Analysis: Parent and Family-Based Interventions for
Children and Adolescents With Chronic Medical Conditions. Journal of Pediatric Psychology. 2014 May 30. pii: jsu032. [Epub ahead of print]
Abstract
OBJECTIVE:
To quantify the effects of parent- and family-based psychological therapies for youth with common chronic medical conditions on parent and family outcomes (primary aim) and child outcomes (secondary aim).METHODS:
MEDLINE, EMBASE, and PsycINFO were searched from inception to April 2013. 37 randomized controlled trials were included. Quality of the evidence was evaluated using GRADE criteria. Data were extracted on parent, family, and child outcomes.RESULTS:
Pooled psychological therapies had a positive effect on parent behavior at post-treatment and follow-up; no significant improvement was observed for other outcome domains. Problem-solving therapy (PST) improved parent mental health and parent behavior at post-treatment and follow-up. There was insufficient evidence to evaluate cognitive-behavioral and systems therapies for many outcome domains.CONCLUSIONS:
Parent- and family-based psychological therapies can improve parent outcomes, with PST emerging as particularly promising. Future research should incorporate consensus statements for outcomes assessment, multisite recruitment, and active comparator conditions.- PMID: 24881048
Spina bifida: a multidisciplinary perspective on a many-faceted condition.
Fieggen G, Fieggen K, Stewart C, Padayachy L, Lazarus J, Donald K, Dix-Peek S, Toefy Z, Figaji A. (2014) Spina bifida: a multidisciplinary perspective on a many-faceted condition. South African Medical Journal. 2014 Mar;104(3):213-7.
Open spina bifida
or myelomeningocele (SBM) is the most common birth defect involving the
central nervous system, second only in incidence to congenital cardiac
disease. Outcomes in this disorder were poor until the mid-20th century,
when modern neurosurgical techniques (closing the lesion and treating
hydrocephalus) and treatment for the neuropathic bladder addressed the
major causes of mortality, although SBM may still be poorly treated in
the developing world. Initial management - or mismanagement - has a
profound impact on survival and long-term quality of life.
- PMID: 24897828
Fetal surgery for myelomeningocele is effective: a critical look at the whys.
Meuli M, Moehrlen U. (2014) Fetal surgery for myelomeningocele is effective: a critical look at the whys Pediatric Surgery International. 2014 Jun 8. [Epub ahead of print]
Abstract
Formerly, the
disastrous cluster of neurologic deficits and associated neurogenic
problems in patients with myelomeningocele (MMC) was generally thought
to solely result from the primary malformation, i.e., failure of
neurulation. Today, however, there is no doubt that a dimensional
additional pathogenic mechanism exists. Most likely, it contributes much
more to loss of neurologic function than non-neurulation does. Today,
there is a large body of compelling experimental and clinical evidence
confirming that the exposed part of the non-neurulated spinal
cord is progressively destroyed during gestation, particularly so in
the third trimester. These considerations gave rise to the
two-hit-pathogenesis of MMC with non-neurulation being the first and
consecutive in utero acquired neural tissue destruction being the second
hit. This novel pathophysiologic understanding has obviously triggered
the question whether the serious and irreversible functional loss caused
by the second hit could not be prevented or, at least, significantly
alleviated by timely protecting the exposed spinal
cord segments, i.e., by early in utero repair of the MMC lesion. Based
on this intriguing hypothesis and the above-mentioned data, human fetal
surgery for MMC was born in the late nineties of the last century and
has made its way to become a novel standard of care, particularly after
the so-called "MOMS Trial". This trial, published in the New England
Journal of Medicine, has indisputably shown that overall, open prenatal
repair is distinctly better than postnatal care alone. Finally, a number
of important other topics deserve being mentioned, including the
necessity to work on the up till now immature endoscopic fetal repair
technique and the need for concentration of these extremely challenging
cases to a small number of really qualified fetal surgery centers
worldwide. In conclusion, despite the fact that in utero repair of MMC
is not a complete cure and not free of risk for both mother and fetus,
current data clearly demonstrate that open fetal-maternal surgery is to
be recommended as novel standard of care when pregnancy is to be
continued and when respective criteria for the intervention before birth
are met. Undoubtedly, it is imperative to inform expecting mothers
about the option of prenatal surgery once their fetus is diagnosed with
open spina bifida.
- PMID: 24908159
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