Citations

ABSTRACTIntroduction: The objective of this study was to answer the typical questions from patients regarding the likely neurologic outcome and likelihood of recurrence when discussing peroneal intraneural ganglion cysts preoperatively. Methods: Retrospective analysis of all patients who underwent surgery for a peroneal intraneural ganglion cyst between January 1, 2000, and April 1, 2017, was performed. Postoperative neurologic outcomes and radiologic recurrences are reported. Results: There were 65 patients. Average clinical follow‐up was 14 months. Median dorsiflexion and eversion preoperatively were 2/5 and 4/5, respectively. Median dorsiflexion and eversion at last follow‐up postoperatively were 5/5. Radiologic recurrence occurred in 6 (9%) patients, all extraneural. Discussion: The data support excellent postoperative motor outcomes, despite frequent dense weakness of peroneal‐innervated musculature preoperatively. The surgical approach appears to eliminate risk of intraneural recurrence and minimizes risk of extraneural recurrence. Muscle Nerve 57: 989–993, 2018

Objective The objective of this study was to evaluate length of induction and postdelivery complications before and after implementation of a standardized approach to second-trimester medical termination of pregnancy. Study Design This was a retrospective cohort study of all women undergoing medical termination of pregnancy between 130/7 and 266/7 weeks of gestation at a single, academic institution from July 1, 2012, through June 30, 2015. The primary outcome was the time from the start of induction of labor to delivery of the fetus. Postdelivery complications including the need for dilation and curettage (D&C), blood transfusion, and readmission to the hospital were secondary outcomes of interest. Results A total of 62 women met inclusion criteria; 38 before and 24 after the intervention. There were no differences in measured baseline characteristics (p > 0.05). There was a significant decrease in induction time after the intervention (12.3 compared with 8.6 hours, p = 0.031). There was no significant difference in rates of D&C or other measured complications (p > 0.05). Conclusion Implementation of a standardized clinical guideline for second-trimester medical termination was associated with a decrease in length of induction. There was no significant difference in need for D&C or postdelivery complications; however, we were underpowered for these secondary outcomes.

ABSTRACTIntroduction: Multifocal motor neuropathy (MMN) is a motor only, asymmetric onset neuropathy that is relatively treatment‐refractory compared with classic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy. Methods: We reviewed 35 patients seropositive for GM1 (monosialo‐asialo [immunoglobulin M, IgM; immunoglobulin G, IgG]) and/or GD1b (disialo [IgG, IgM]) autoantibodies having MMN, classic CIDP, or MADSAM. Immune‐treatment responsiveness and clinical course was compared with antibody negative disease controls. Results: Seventy‐nine percent of seropositives with an initial diagnosis of MMN were immunotherapy responsive compared with 46% of seronegatives (P = 0.045). Eight ganglioside antibody positive MMN patients of 19 (42%) developed sensory findings consistent with MADSAM compared with 3 of 41 (7%) seronegative MMN patients (P = 0.003). MMN and MADSAM patients with ganglioside antibody positivity had more sustained treatment responses (P = 0.03). Discussion: Patients initially diagnosed with MMN seropositive for diverse GM1 autoantibodies appear more likely to have sustained treatment response and evolution to MADSAM. Muscle Nerve 57: 1000–1005, 2018

Objective: Predictors of long-term functional impairment in acute respiratory failure of all causes are poorly understood. Our objective was to assess the frequency and predictors of long-term functional impairment or death after invasive mechanical ventilation for acute respiratory failure of all causes. Design: Population-based, observational cohort study. Setting: Eight adult ICUs of a single center. Patients: All adult patients from Olmsted County, Minnesota, without baseline functional impairment who received mechanical ventilation in ICUs for acute respiratory failure of all causes from 2005 through 2009. Interventions: None. Measurements and Main Results: In total, 743 patients without baseline functional impairment received mechanical ventilation in the ICU. At 1- and 5-year follow-up, the rates of survival with return to baseline functional ability were 61% (366/597) and 53% (356/669). Among 71 patients with new functional impairment at 1 year, 55% (39/71) had recovered and were alive without functional impairment at 5 years. Factors predictive of new functional impairment or death at 1 year were age, comorbidities, discharge to other than home, mechanical ventilation of 7 days or longer, and stroke. Of factors known at the time of intubation, the following are predictive of new functional impairment or death: age, comorbidities, nonsurgical condition, Acute Physiology and Chronic Health Evaluation III score, stroke, and sepsis. Post hoc sensitivity analyses revealed no significant change in predictor variables in patient populations when stroke was excluded or who received more than 48 hours of mechanical ventilation. Conclusions: At 1- and 5-year follow-up, many patients who received mechanical ventilation for acute respiratory failure from all causes are no longer alive or have new moderate-to-severe functional impairment. Functional recovery between year 1 and year 5 is possible and common. Sepsis, stroke, illness severity, age, and comorbidities predict long-term functional outcome at intubation.

Background— Postmortem genetic testing for heritable cardiovascular (CV) disorders is often lacking because ideal specimens (ie, whole blood) are not retained routinely at autopsy. Formalin-fixed paraffin-embedded tissue (FFPET) is ubiquitously collected at autopsy, but DNA quality hampers its use with traditional sequencing methods. Targeted next-generation sequencing may offer the ability to circumvent such limitations, but a method has not been previously described. The primary aim of this study was to develop and evaluate the use of FFPET for heritable CV disorders via next-generation sequencing. Methods and Results— Nineteen FFPET (heart) and blood (whole blood or dried blood spot) specimens underwent targeted next-generation sequencing using a custom panel of 101 CV-associated genes. Nucleic acid yield and quality metrics were evaluated in relation to FFPET specimen age (6 months to 15 years; n=14) and specimen type (FFPET versus whole blood and dried blood spot; n=12). Four FFPET cases with a clinical phenotype of heritable CV disorder were analyzed. Accuracy and precision were 100% concordant between all sample types, with read depths >100× for most regions tested. Lower read depth, as low as 40×, was occasionally observed with FFPET and dried blood spot. High-quality DNA was obtained from FFPET samples as old as 15 years. Genomic analysis of FFPET from the 4 phenotype-positive/genotype unknown cases all revealed putative disease-causing variants. Conclusions— Similar performance characteristics were observed for next-generation sequencing of FFPET, whole blood, and dried blood spot in the evaluation of inherited CV disorders. Although blood is preferable for genetic analyses, this study offers an alternative when only FFPET is available.

ABSTRACT Background Physician well-being is garnering increasing attention. In 2016, the Journal of Graduate Medical Education (JGME) published a review by Kristin Raj, MD, entitled “Well-Being in Residency: A Systematic Review.” There is benefit in contextualizing the literature on resident well-being through an academic journal club. Objective We summarized an asynchronous, online journal club discussion about this systematic review and highlighted themes that were identified in the review. Methods In January 2017, JGME and the Academic Life in Emergency Medicine (ALiEM) blog facilitated an open-access, online, weeklong journal club on the featured JGME article. Online discussions and interactions were facilitated via blog posts and comments, a video discussion on Google Hangouts on Air, and Twitter. We performed a thematic analysis of the discussion and captured web analytics. Results Over the first 14 days, the blog post was viewed 1070 unique times across 52 different countries. A total of 130 unique participants on Twitter posted 480 tweets using the hashtag #JGMEscholar. Thematic analysis revealed 5 major domains: the multidimensional nature of well-being, measurement of well-being, description of wellness programs and interventions, creation of a culture of wellness, and critique of the methodology of the review. Conclusions Our online journal club highlighted several gaps in the current understanding of resident well-being, including the need for consensus on the operational definition, the need for effective instruments to evaluate wellness programs and identify residents in distress, and a national research collaboration to assess wellness programs and their impact on resident well-being.

ABSTRACTDegenerative disk disease of the spine is a major cause of back pain and disability. Optimization of regenerative medical therapies for degenerative disk disease requires a deep mechanistic understanding of the factors controlling the structural integrity of spinal tissues. In this investigation, we sought to identify candidate regulatory genes controlling extracellular matrix synthesis in spinal tissues. To achieve this goal we performed high throughput next generation RNA sequencing on 39 annulus fibrosus and 21 nucleus pulposus human tissue samples. Specimens were collected from patients undergoing surgical discectomy for the treatment of degenerative disk disease. Our studies identified associations between extracellular matrix genes, growth factors, and other important regulatory molecules. The fibrous matrix characteristic of annulus fibrosus was associated with expression of the growth factors platelet derived growth factor beta (PDGFB), vascular endothelial growth factor C (VEGFC), and fibroblast growth factor 9 (FGF9). Additionally we observed high expression of multiple signaling proteins involved in the NOTCH and WNT signaling cascades. Nucleus pulposus extracellular matrix related genes were associated with the expression of numerous diffusible growth factors largely associated with the transforming growth signaling cascade, including transforming factor alpha (TGFA), inhibin alpha (INHA), inhibin beta A (INHBA), bone morphogenetic proteins (BMP2, BMP6), and others. Clinical significance: this investigation provides important data on extracellular matrix gene regulatory networks in disk tissues. This information can be used to optimize pharmacologic, stem cell, and tissue engineering strategies for regeneration of the intervertebral disk and the treatment of back pain. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 36:1356–1369, 2018.