A Dutch guideline for the treatment of scoliosis in neuromuscular disorders

<p>Abstract</p> <p>Background</p> <p>Children with neuromuscular disorders with a progressive muscle weakness such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy frequently develop a progressive scoliosis. A severe scoliosis compromises respiratory function and makes sitting more difficult. Spinal surgery is considered the primary treatment option for correcting severe scoliosis in neuromuscular disorders. Surgery in this population requires a multidisciplinary approach, careful planning, dedicated surgical procedures, and specialized after care.</p> <p>Methods</p> <p>The guideline is based on scientific evidence and expert opinions. A multidisciplinary working group representing experts from all relevant specialties performed the research. A literature search was conducted to collect scientific evidence in answer to specific questions posed by the working group. Literature was classified according to the level of evidence.</p> <p>Results</p> <p>For most aspects of the treatment scientific evidence is scarce and only low level cohort studies were found. Nevertheless, a high degree of consensus was reached about the management of patients with scoliosis in neuromuscular disorders. This was translated into a set of recommendations, which are now officially accepted as a general guideline in the Netherlands.</p> <p>Conclusion</p> <p>In order to optimize the treatment for scoliosis in neuromuscular disorders a Dutch guideline has been composed. This evidence-based, multidisciplinary guideline addresses conservative treatment, the preoperative, perioperative, and postoperative care of scoliosis in neuromuscular disorders.</p

A comparative biochemical and pathological evaluation of brain samples from knock-in murine models of Gaucher disease

Gaucher disease (GD) is a lysosomal storage disorder stemming from biallelic mutations i

Clinical features of children and adults with a muscular dystrophy using powered indoor/outdoor wheelchairs (EPIOCs): disease features, comorbidities and complications of disability.

Purpose: To describe the clinical features of electric powered indoor/outdoor wheelchair users with a muscular dystrophy, likely to influence optimal prescription; reflecting features of muscular dystrophies, conditions secondary to disability and comorbidities impacting on equipment provision. Methods: cross-sectional retrospective case note review of recipients of electric powered indoor/outdoor wheelchairs provided by a specialist regional wheelchair service. Data on demography, diagnostic/clinical and wheelchair prescription were systematically extracted. Results: Fifty-one men and 14 women, mean age 23.7 (range 10-67, sd 12.95) years, were studied. Forty had Duchenne muscular dystrophy, 22 had other forms of muscular dystrophy and three were unclassified. Twenty-seven were aged under 19. Notable clinical features included problematic pain (10), cardiomyopathy (5) and ventilatory failure (4). Features related to disability were (kypho)scoliosis (20) and oedema/cellulitis (3) whilst comorbidities included back pain (5). Comparison of younger with older users revealed younger users had more features of muscular dystrophy affecting electric powered chair provision (56%) whilst older users had more comorbidity (37%). Tilt-in-space was prescribed for 81% of users, specialised seating for 55% and complex controls for 16%. Conclusions: Muscular dystrophy users were prescribed electric powered indoor/outdoor chairs with many additional features reflecting the consequences of profound muscle weakness. In addition to facilitating independence and participation, electric powered indoor/outdoor chairs have major therapeutic benefits

Glucosamine and chondroitin sulfate supplementation to treat symptomatic disc degeneration: Biochemical rationale and case report

BACKGROUND: Glucosamine and chondroitin sulfate preparations are widely used as food supplements against osteoarthritis, but critics are skeptical about their efficacy, because of the lack of convincing clinical trials and a reasonable scientific rationale for the use of these nutraceuticals. Most trials were on osteoarthritis of the knee, while virtually no documentation exists on spinal disc degeneration. The purpose of this article is to highlight the potential of these food additives against cartilage degeneration in general, and against symptomatic spinal disc degeneration in particular, as is illustrated by a case report. The water content of the intervertebral disc is a reliable measure of its degeneration/ regeneration status, and can be objectively determined by Magnetic Resonance Imaging (MRI) signals. CASE PRESENTATION: Oral intake of glucosamine and chondroitin sulfate for two years associated with disk recovery (brightening of MRI signal) in a case of symptomatic spinal disc degeneration. We provide a biochemical explanation for the possible efficacy of these nutraceuticals. They are bioavailable to cartilage chondrocytes, may stimulate the biosynthesis and inhibit the breakdown of their extracellular matrix proteoglycans. CONCLUSION: The case suggests that long-term glucosamine and chondroitin sulfate intake may counteract symptomatic spinal disc degeneration, particularly at an early stage. However, definite proof requires well-conducted clinical trials with these food supplements, in which disc de-/regeneration can be objectively determined by MRI. A number of biochemical reasons (that mechanistically need to be further resolved) explain why these agents may have cartilage structure- and symptom-modifying effects, suggesting their therapeutic efficacy against osteoarthritis in general

Other title: CIIC report : Ohio Penal Industries; Other title: Correctional Institution Inspection Committee report : Ohio Penal Industries

Title from PDF p. [1] (viewed on Dec. 9, 2011); Includes bibliographical references (p. 35-36).Provides findings and recommendations from CIIC's research and analysis of the Ohio Penal Industries (OPI) program. The report also includes national information regarding correctional industries to provide a comparison for Ohio

International students and reference work : A phenomenographic study of academic librarians and their experiences of reference work aimed at international students

The purpose of this Master’s thesis is to investigate how academic librarians experience reference work aimed at international students. International students are a growing usergroup in Swedish academic libraries. The study’s main research question is:- How do academic librarians experience reference work aimed at international students?The study was performed with a phenomenographic methodological approach where seven qualitative interviews with academic librarians were conducted. These interviews were transcripted in their entirety and analysed using three key concepts based on the study’s research questions that formed the different experiences that created the study’s outcome space. The results show three major viewpoints on reference work aimed at international students. These viewpoints are based foremost on variations on how the barriers that international students meet in their library use are experienced. Within experience category A barriers are perceived as a hindrance for effective reference service. Barriers are acknowledged within experience category B but are seen as possible to overcome. Within experience category C barriers are overlooked, international students are here perceived as user group in no more need than other user groups. Two of the experience categories also represent different views on the relationship between the library system and the user. Experience category A is characterised by a user perspective whilst perception category B has a system oriented view of the interaction.Uppsatsnivå:

Emergency surgery for intestinal obstruction revealing ileal Crohn's disease (CD) during pregnancy

Few data are available regarding emergency surgery for Crohn's disease (CD) during pregnancy. A 22-year -old woman, at 26 weeks of pregnancy, was admitted for premature labour, intestinal obstruction and systemic inflammation. Biology and ultrasonography were not helpful; imaging was completed with a CT scan, which confirmed the mechanical ileal obstruction. The intestinal obstruction worsened and led to emergency surgery. An inflammatory stenosis of the terminal ileum with two fistulas was found. An ileal resection and ileostomy were performed. The pathological analysis of the resected specimen confirmed CD. The postoperative course was uneventful. The delivery of a baby with normal weight and status occurred at term (40 weeks of amenorrhoea). There have been only 10 case reports of intestinal complications revealing CD during pregnancy. The delay in diagnosis and adequate treatment led to significant maternal and fetal morbidity and mortality. This case suggests that intestinal surgery for CD is feasible during pregnancy and may be associated with a favourable outcome for the baby as well as for the mother