Spinal cord injury brings devastating consequences and huge economic burden. Different authoritative organizations have developed different guidelines for pharmacological treatments of spinal cord injury, but there is a lack of a critical appraisal of them.
To systematically review and appraise guidelines regarding their recommendations for pharmacological treatments for spinal cord injury.
Systematic review.
We searched Medline, Embase, Cochrane, and Web of Science from January 2000 to January 2022 as well as guideline-specific databases (eg, Congress of Neurological Surgeons) and Google Scholar. We included the most updated guideline containing evidence-based recommendations or consensus-based recommendations developed by specific authoritative organizations if multiple versions were available. We appraised guidelines through the Appraisal of Guidelines for Research and Evaluation, 2nd edition instrument consisting of six domains (eg, applicability). With supporting evidence, recommendations were classified as: for, against, neither for nor against. We utilized an evidence assessment system to categorize the quality of supporting evidence as poor, fair, or good.
Eight guidelines developed from 2008 to 2020 were included, but all of them scored lowest in the domain of applicability among all six domains. Twelve pharmacological agents (eg, methylprednisolone) were studied. For methylprednisolone, three guidelines (3/8=37.5%) recommended for (one evidence-based and two consensus-based), three (3/8=37.5%) recommended against (all evidence-based), and two (2/8=25%) recommended neither for nor against. For monosialotetrahexosylganglioside (GM-1), one guideline (1/4=25%) recommended for (consensus-based), one (1/4=25%) recommended against (evidence-based), and two (2/4=50%) recommended neither for nor against. For other agents (eg, minocycline), most guidelines (3/5=60%) recommended neither for nor against, one (1/5=20%) recommended against naloxone (evidence-based) and nimodipine (evidence-based), and one (1/5=20%) recommended for neural growth factor (consensus-based). The quality of most of the supporting evidence was poor, and the rest was fair.
There were inconsistencies among recommendations for methylprednisolone and GM-1. Evidence-based recommendations tended to recommend against, whereas consensus-based recommendations tended to recommend for.

A short course of corticosteroids is among the management strategies considered by specialists for the treatment of vestibular neuritis (VN). We conducted an umbrella review (systematic review of systematic reviews) to summarize the evidence of corticosteroids use for the treatment of VN.
We included systematic reviews of randomized controlled trials (RCTs) and observational studies that evaluated the effects of corticosteroids compared to placebo or usual care in adult patients with acute VN. Titles, abstracts, and full texts were screened in duplicate. The quality of reviews was assessed with the A MeaSurement Tool to Assess systematic Reviews (AMSTAR-2) tool. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) assessment was used to rate certainty of evidence. No meta-analysis was performed.
From 149 titles, five systematic reviews were selected for quality assessment, and two reviews were of higher methodological quality and were included. These two reviews included 12 individual studies and 660 patients with VN. In a meta-analysis of two RCTs including a total of 50 patients, the use of corticosteroids (compared to placebo) was associated with higher complete caloric recovery (risk ratio 2.81, 95% confidence interval [CI] 1.32 to 6.00, low certainty). It is very uncertain whether this translates into clinical improvement as shown by the imprecise effect estimates for outcomes such as patient-reported vertigo or patient-reported dizziness disability. There was a wide CI for the outcome of dizziness handicap score (one study, 30 patients, 20.9 points in corticosteroids group vs. 15.8 points in placebo, mean difference +5.1, 95% CI -8.09 to +18.29, very low certainty). Higher rates of minor adverse effects for those receiving corticosteroids were reported, but the certainty in this evidence was very low.
There is limited evidence to support the use of corticosteroids for the treatment of VN in the emergency department.

暂无摘要。

Objective:Intratympanic（IT） drug delivery receives attention due to its effectivity in treatment for Menière\'s disease（MD）. Due to the release of the consensuses and new evidence on IT drug delivery for MD have been published, the review with a view to supplementing the details of IT treatment of MD is indispensable. Methods:The literatures on IT injection for MD treatment over the last two decades are retrieved, International consensus（ICON） on treatment of Menière\'s disease（2018）, Clinical Practice Guideline（2020） and European Position statement on Diagnosis and Treatment of Menière\'s disease（2018） are taken into account for reference, and follow advice from experts from Europe, USA and China. Results:Experts agree on the following: ①The effectiveness of IT methylprednisolone（ITM） on vertigo control seems to be somewhat better than that of IT dexamethasone（ITD）, and ITM can restore hearing in some cases. ②Due to the ototoxicity of aminoglycosides, the application of intratympanic gentamicin（ITG） in MD patients with good hearing is conservative. However, some studies suggest that ITG with low doses has no significant effect on hearing, which needs to be further proved by clinical studies with high levels of evidence. ③Currently, generally accepted treatment endpoint of ITG is no vertigo attack in a 12-month period or a vestibular loss in objective tests in the affected ear. Conclusion:More studies with high level of evidence are needed to evaluate the drug type, efficacy, and therapeutic endpoint of IT therapy for MD.
目的：鼓室内（intratympanic，IT）注射药物治疗梅尼埃病（MD）因其疗效显著而备受关注。由于IT注射药物治疗MD的共识和新证据的发布，对IT注射药物治疗MD进行细节上的补充具有重要意义。 方法：检索近二十年来有关IT注射药物治疗MD的文献，参考《梅尼埃病治疗国际共识（ICON）》（2018年），《美国梅尼埃病临床实践指南》（2020年）和《梅尼埃病诊断治疗的欧洲立场声明》（2018），并遵循来自欧洲、美国和中国的专家意见。 结果：专家一致认为：①鼓室内注射甲泼尼龙（ITM）对眩晕控制的疗效优于鼓室内注射地塞米松（ITD），ITM有恢复MD患者听力的可能性。②由于氨基糖甙类药物的耳毒性，鼓室内注射庆大霉素（ITG）在听力良好MD患者中的应用持谨慎态度。但也有研究表明，小剂量ITG对听力没有显著影响，还需要高水平证据的临床研究进一步证明。③目前普遍接受的ITG治疗终点是在12个月内无眩晕发作或受累耳客观检查提示前庭功能丧失。 结论：对IT注射药物治疗MD的药物类型、疗效和治疗终点还需要更多高证据水平的研究进行评价。.

OBJECTIVE: Intratympanic (IT) drug delivery receives attention due to its effectivity in treatment for Menière\'s disease (MD). Due to the release of the consensuses and new evidence on IT drug delivery for MD have been published, the review with a view to supplementing the details of IT treatment of MD is indispensable.
METHODS: The literatures on IT injection for MD treatment over the last two decades are retrieved, International consensus (ICON) on treatment of Menière\'s disease (2018), Clinical Practice Guideline (2020) and European Position statement on Diagnosis and Treatment of Meniere\'s Disease (2018) are taken into account for reference, and follow advice from experts from Europe, USA and China.
RESULTS: Experts agree on the following: (1) The effectiveness of IT methylprednisolone (ITM) on vertigo control seems to be somewhat better than that of IT dexamethasone (ITD), and ITM can restore hearing in some cases. (2) Due to the ototoxicity of aminoglycosides, the application of intratympanic gentamicin (ITG) in MD patients with good hearing is conservative. However, some studies suggest that ITG with low doses has no significant effect on hearing, which needs to be further proved by clinical studies with high levels of evidence. (3) Currently, generally accepted treatment endpoint of ITG is no vertigo attack in a 12-month period or a vestibular loss in objective tests in the affected ear.
CONCLUSIONS: More studies with high level of evidence are needed to evaluate the drug type, efficacy, and therapeutic endpoint of IT therapy for MD.

Paediatric acute severe colitis (ASC) management during the novel SARS-CoV-2/COVID-19 pandemic is challenging due to reliance on immunosuppression and the potential for surgery. We aimed to provide COVID-19-specific guidance using the European Crohn\'s and Colitis Organisation/European Society for Paediatric Gastroenterology, Hepatology and Nutrition guidelines for comparison.
We convened a RAND appropriateness panel comprising 14 paediatric gastroenterologists and paediatric experts in surgery, rheumatology, respiratory and infectious diseases. Panellists rated the appropriateness of interventions for ASC in the context of the COVID-19 pandemic. Results were discussed at a moderated meeting prior to a second survey.
Panellists recommended patients with ASC have a SARS-CoV-2 swab and expedited biological screening on admission and should be isolated. A positive swab should trigger discussion with a COVID-19 specialist. Sigmoidoscopy was recommended prior to escalation to second-line therapy or colectomy. Methylprednisolone was considered appropriate first-line management in all, including those with symptomatic COVID-19. Thromboprophylaxis was also recommended in all. In patients requiring second-line therapy, infliximab was considered appropriate irrespective of SARS-CoV-2 status. Delaying colectomy due to SARS-CoV-2 infection was considered inappropriate. Corticosteroid tapering over 8-10 weeks was deemed appropriate for all. After successful corticosteroid rescue, thiopurine maintenance was rated appropriate in patients with negative SARS-CoV-2 swab and asymptomatic patients with positive swab but uncertain in symptomatic COVID-19.
Our COVID-19-specific adaptations to paediatric ASC guidelines using a RAND panel generally support existing recommendations, particularly the use of corticosteroids and escalation to infliximab, irrespective of SARS-CoV-2 status. Consideration of routine prophylactic anticoagulation was recommended.

BACKGROUND: Morphea is an inflammatory and fibrosing condition that affects the skin and subcutaneous structures. Morphea is managed by dermatologists, rheumatologists, or both. Prior studies have suggested there is significant variability in approach to treatment. In 2012, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) for pediatric morphea to develop more standardized treatment plans for patients requiring systemic therapy. We aimed to assess whether the publication of CTPs has impacted care of patients with morphea at our institution.
METHODS: Data were collected via a retrospective review of medical records of 61 pediatric patients diagnosed with morphea at Seattle Children\'s Hospital (SCH) from January 1, 2005, to December 12,2017.
RESULTS: Prior to the publication of CTPs, 2 out of 24 patients (8.3%) were treated with a regimen that matched a subsequent CTP. After publication of CTPs, 29 out of 37 patients (78.4%) were treated with a regimen that matched a CTP (P < 0.001). A subanalysis was performed to assess the number of patients who needed second- or third-line therapies. Of those who followed a CTP therapy plan (n = 26), 3 patients (11.5%) needed a second-line therapy compared with 11 patients (44%) in the no-CTP followed group (n = 25), (P = 0.012).
CONCLUSIONS: The publication of CTPs led to a significant change in treatment approach for patients with morphea requiring systemic therapy at SCH. Patients treated with one of the treatment plans recommended by the CTPs were less likely to need second-line systemic therapy.

Ulcerative colitis and Crohn\'s disease are the principal forms of inflammatory bowel disease. Both represent chronic inflammation of the gastrointestinal tract, which displays heterogeneity in inflammatory and symptomatic burden between patients and within individuals over time. Optimal management relies on understanding and tailoring evidence-based interventions by clinicians in partnership with patients. This guideline for management of inflammatory bowel disease in adults over 16 years of age was developed by Stakeholders representing UK physicians (British Society of Gastroenterology), surgeons (Association of Coloproctology of Great Britain and Ireland), specialist nurses (Royal College of Nursing), paediatricians (British Society of Paediatric Gastroenterology, Hepatology and Nutrition), dietitians (British Dietetic Association), radiologists (British Society of Gastrointestinal and Abdominal Radiology), general practitioners (Primary Care Society for Gastroenterology) and patients (Crohn\'s and Colitis UK). A systematic review of 88 247 publications and a Delphi consensus process involving 81 multidisciplinary clinicians and patients was undertaken to develop 168 evidence- and expert opinion-based recommendations for pharmacological, non-pharmacological and surgical interventions, as well as optimal service delivery in the management of both ulcerative colitis and Crohn\'s disease. Comprehensive up-to-date guidance is provided regarding indications for, initiation and monitoring of immunosuppressive therapies, nutrition interventions, pre-, peri- and postoperative management, as well as structure and function of the multidisciplinary team and integration between primary and secondary care. Twenty research priorities to inform future clinical management are presented, alongside objective measurement of priority importance, determined by 2379 electronic survey responses from individuals living with ulcerative colitis and Crohn\'s disease, including patients, their families and friends.

BACKGROUND: We designed and initiated a pilot comparative effectiveness study for juvenile localized scleroderma (jLS), for which there is limited evidence on best therapy. We evaluated the process we used, in relation to the specific protocol and to the general task of identifying strategies for implementing studies in rare pediatric diseases.
METHODS: This was a prospective, multi-center, observational cohort study of 50 jLS patients initiating treatment, designed and conducted by the jLS group of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) from 2012 to 2015. A series of virtual and physical meetings were held to design the study, standardize clinical assessments, generate and refine disease activity and damage measures, and monitor the study. Patients were initiated on one of three standardized methotrexate-based treatment regimens (consensus treatment plans, CTPs) and monitored for 1 year. An optional bio-banking sub-study was included.
RESULTS: The target enrollment of 50 patients was achieved over 26 months at 10 sites, with patients enrolled into all CTPs. Enrolled patients were typical for jLS. Study eligibility criteria were found to perform well, capturing patients thought appropriate for treatment studies. Minor modifications to the eligibility criteria, primarily to facilitate recruitment for future studies, were discussed with consensus agreement reached on them by the jLS group. There were marked differences in site preferences for specific CTPs, with half the sites treating all their patients with the same CTP. Most patients (88%) completed the study, and 68% participated in the bio-banking substudy.
CONCLUSIONS: We demonstrate the feasibility of our approach for conducting comparative effectiveness research in a rare pediatric disease. Multi-center collaboration by dedicated investigators who met regularly was a key factor in the success of this project. Other factors that facilitate these studies include having a sufficient number of investigators to enroll in each regimen, and streamlining study approval and management.

This article presents a hypothetical case of a patient with multiple sclerosis (MS), reviewing the use of clinical practice guidelines and incorporation of quality measures into practice. Appropriate diagnosis of MS is important to avoid the cost and consequences of a misdiagnosis. Ensuring that treatment discussion occurs when a patient with MS is receptive is good clinical practice and a guideline recommendation from the American Academy of Neurology. Continuing dialogue about disease-modifying therapy and ongoing monitoring are important for patient care and improved outcomes. Ultimately, cost-effective care in MS relates to using appropriate medicines in patients with active MS, ensuring adherence, and careful monitoring.