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Abstract: Pain experiences of youth with brain-based developmental disabilities are often overlooked and/or misinterpreted, increasing the risk for poor or inadequate pain assessment and management. Ample measures exist to assess acute and chronic pain, yet their utility and frequency of use in youth with brain-based developmental disabilities is unclear and available measures do not have strong measurement properties for this diverse group. This systematic review identified the scope of self-reported and observer-reported pain assessment in studies of youth (aged 3-24 years) with brain-based developmental disabilities (phase 1) and summarized other measures of pain-related functioning for acute and chronic pain (ie, physical, emotional, social, sleep, and quality of life, within the subset of quantitative studies focused primarily on pain, phase 2). A comprehensive search for English-language studies was conducted in August 2022 in Web of Science, CINAHL, MEDLINE, Cochrane CENTRAL, EMBASE, and APA PsychINFO (PROSPERO registration: CRD42021237444). A total of 17,029 unique records were screened. Of the 707 articles included in phase 1, most assessed chronic pain (n = 314; 62.0%) and primarily used observer-report (n = 155; 31%) over self-report (n = 67; 13%). Of the 137 articles included in phase 2, other outcomes assessed alongside pain intensity included motor ability (16.8%), adaptive functioning (11%), quality of life (8%), pain interference (6.6%), mental health (5.8%), and communication ability (2.9%). Cerebral palsy was the most common population in both phase 1 (n = 343; 48.5%) and phase 2 (n = 83; 59.7%). This review provides a foundational understanding of pain assessment in brain-based developmental disabilities and highlights continued inequities in holistic pain assessment for this population., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the International Association for the Study of Pain.)

Background: Policy decisions in health care, such as hospital performance evaluation and performance-based budgeting, require an accurate prediction of hospital length of stay (LOS). This paper provides a systematic review of risk adjustment models for hospital LOS, and focuses primarily on studies that use administrative data., Methods: MEDLINE, EMBASE, Cochrane, PubMed, and EconLit were searched for studies that tested the performance of risk adjustment models in predicting hospital LOS. We included studies that tested models developed for the general inpatient population, and excluded those that analyzed risk factors only correlated with LOS, impact analyses, or those that used disease-specific scales and indexes to predict LOS., Results: Our search yielded 3973 abstracts, of which 37 were included. These studies used various disease groupers and severity/morbidity indexes to predict LOS. Few models were developed specifically for explaining hospital LOS; most focused primarily on explaining resource spending and the costs associated with hospital LOS, and applied these models to hospital LOS. We found a large variation in predictive power across different LOS predictive models. The best model performance for most studies fell in the range of 0.30-0.60, approximately., Conclusions: The current risk adjustment methodologies for predicting LOS are still limited in terms of models, predictors, and predictive power. One possible approach to improving the performance of LOS risk adjustment models is to include more disease-specific variables, such as disease-specific or condition-specific measures, and functional measures. For this approach, however, more comprehensive and standardized data are urgently needed. In addition, statistical methods and evaluation tools more appropriate to LOS should be tested and adopted.

Objective: To compare the diagnostic accuracy of computed tomographic angiography (CTA) with digital subtraction angiography (DSA) for blunt cerebrovascular injury (BCVI) detection in trauma patients., Background: Controversy exists as to whether the diagnostic performance of CTA compares favorably with the reference-standard, DSA., Methods: We searched electronic databases (1950 to May 22, 2012), article bibliographies, conference proceedings (2008-2011), and clinical trial registries for studies comparing the accuracy of CTA with DSA for BCVI detection in trauma patients. Pooled estimates of sensitivity, specificity, and positive and negative likelihood ratios were calculated using bivariate random effects models., Results: Eight studies that examined 5704 carotid or vertebral arteries in 1426 trauma patients met inclusion criteria. The pooled sensitivity and specificity for BCVI detection with CTA versus DSA was 66% (95% CI, 49%-79%; I = 80.4%) and 97% (95% CI, 91%-99%; I = 94.6%), respectively. Corresponding pooled positive and negative likelihood ratios were 20.0 (95% CI, 6.9-58.4; I = 87.7%) and 0.35 (95% CI, 0.22-0.56; I = 74.9%), respectively. Although pooled sensitivity varied with the number of available CT slices, the training of interpreting radiologists, and in a pattern suggestive of differences in diagnostic threshold for judging CTA positivity, it remained 80% or less among studies that used scanners with 16 or more slices per rotation and where the CTA was read by neuroradiologists., Conclusions: Existing evidence suggests that the diagnostic performance of CTA varies considerably across studies, likely due to an implicit variation in diagnostic threshold across trauma centers. Moreover, although CTA appears to lack sensitivity to adequately rule out BCVI, it may be useful to rule in BCVI among trauma patients with a high pretest probability of injury.

Study Design: Systematic Review., Objective: (1) To qualitatively analyze the literature on the efficacy and effectiveness of artificial cervical disc arthroplasty (ACDA). (2) To highlight methodological and reporting issues of randomized controlled trials (RCT) reports on effectiveness of ACDA compared to cervical fusion., Summary of Background Data: ACDA is an alternate surgical procedure that may replace cervical fusion in selected patients suffering from cervical degenerative disc disease., Methods: We searched seven electronic databases, including MEDLINE, Cochrane Library, and EMBASE, unpublished sources, and reference lists for studies on the efficacy and effectiveness of ACDA compared to cervical fusion--the surgical standard of care for patients with cervical degenerative disc disease., Results: A total of 622 studies were retrieved, of which 18 (13 case series, four RCT reports, one nonrandomized comparative study) met the inclusion criteria for this review. The four RCTs and the nonrandomized comparative study concluded that the effectiveness of ACDA is not inferior to that of cervical fusion in the short term (up to 2-yr follow-up). The safety profile of both procedures appears similar. The case series reviewed noted improved clinical outcomes at 1 or 2 years after one or multiple-level ACDA., Conclusion: ACDA is a surgical procedure that may replace cervical fusion in selected patients suffering from cervical degenerative disc disease. Within 2 years of follow-up, the effectiveness of ACDA appears similar to that of cervical fusion. Weak evidence exists that ACDA may be superior to fusion for treating neck and arm pain. Future studies should report change scores and change score variance in accordance with RCT guidelines, in order to strengthen credibility of conclusions and to facilitate meta-analyses of studies.

The autosomal dominant spinocerebellar ataxias (ADSCAs) are a heterogeneous group of late-onset neurodegenerative disorders with overlapping clinical features. Genetic linkage studies have identified at least seven distinct loci for the ADSCAs, allowing the genetic classification of these disorders. The spinocerebellar ataxia type 2 (SCA2) locus was mapped to chromosome 12, and a gene responsible for this disorder was recently isolated. The mutation causing SCA2 is an expansion of a trinucleotide CAG repeat contained within the coding region of a novel gene. We describe the results of genotypic analysis for the SCA2 repeat in individuals with ADSCA who were previously found negative for CAG repeat expansions in the SCA1, SCA3, or SCA6 genes. The expanded CAG repeat has been identified in 15 independent families. Repeat instability and anticipation were observed in two large kindreds. The SCA2 mutation was found in 18% of our ADSCA kindreds, confirming the high proportion of SCA2 among this group of disorders.

Hereditary neuropathy with liability to pressure palsies (HNPP) is commonly associated with a 1.5-megabase deletion on chromosome 17p11.2-12. We analyzed the phenotypic expression of the deletion in 39 HNPP patients from 16 families carrying the deletion. Two-thirds of the individuals had episodes of acute mononeuropathy, often involving nerve territories of the upper limbs or brachial plexus; however, 41% of affected subjects were unaware of their disease, and 25% were almost or totally free of symptoms; one-third complained of chronic symptoms and four older patients had a picture of polyneuropathy. Electrophysiologic abnormalities differed among affected subjects, ranging from conduction abnormalities localized at common entrapment sites to diffuse conduction slowing, usually more evident at entrapment sites; patients from one family had preeminent proximal involvement. The spectrum of phenotypic expression of deletion-associated HNPP appears to be broader than previously thought. The prevalence of the disease is probably underestimated, and the availability of molecular diagnosis should increase disease detection.