Citations

AbstractNeuromuscular ultrasound has become an essential tool in the diagnostic evaluation of various neuromuscular disorders, and, as such, there is growing interest in neuromuscular ultrasound training. Effective training is critical in mastering this modality. Our aim was to develop consensus‐based guidelines for neuromuscular ultrasound training courses. A total of 18 experts participated. Expert opinion was sought through the Delphi method using 4 consecutive electronic surveys. A high degree of consensus was achieved with regard to the general structure of neuromuscular ultrasound training; the categorization of training into basic, intermediate, and advanced levels; the learning objectives; and the curriculum for each level. In this study, a group of neuromuscular ultrasound experts established consensus‐based guidelines for neuromuscular ultrasound training. These guidelines can be used in the development of the specialty and the standardization of neuromuscular ultrasound training courses and workshops.

Abstract Mesothelioma affects mostly older individuals who have been occupationally exposed to asbestos. The global mesothelioma incidence and mortality rates are unknown, because data are not available from developing countries that continue to use large amounts of asbestos. The incidence rate of mesothelioma has decreased in Australia, the United States, and Western Europe, where the use of asbestos was banned or strictly regulated in the 1970s and 1980s, demonstrating the value of these preventive measures. However, in these same countries, the overall number of deaths from mesothelioma has not decreased as the size of the population and the percentage of old people have increased. Moreover, hotspots of mesothelioma may occur when carcinogenic fibers that are present in the environment are disturbed as rural areas are being developed. Novel immunohistochemical and molecular markers have improved the accuracy of diagnosis; however, about 14% (high‐resource countries) to 50% (developing countries) of mesothelioma diagnoses are incorrect, resulting in inadequate treatment and complicating epidemiological studies. The discovery that germline BRCA1‐asssociated protein 1 ( BAP1 ) mutations cause mesothelioma and other cancers (BAP1 cancer syndrome) elucidated some of the key pathogenic mechanisms, and treatments targeting these molecular mechanisms and/or modulating the immune response are being tested. The role of surgery in pleural mesothelioma is controversial as it is difficult to predict who will benefit from aggressive management, even when local therapies are added to existing or novel systemic treatments. Treatment outcomes are improving, however, for peritoneal mesothelioma. Multidisciplinary international collaboration will be necessary to improve prevention, early detection, and treatment.

AbstractPurposeAnimal models can be helpful in evaluating new surgical strategies for brachial plexus reconstruction. While several groups have already used the rabbit brachial plexus to model injury, reports conflict in anatomic detail and do not identify a nerve‐muscle pair to measure motor function recovery after reconstruction. The purpose of the current study is to describe the innervations of the biceps and triceps muscles in rabbits, which are both amenable to study in brachial plexus injury models.Materials and MethodsThirteen rabbits weighing 2–2.5 kg were anesthetized. Six rabbits were sacrificed and dissected using loupe and microscope magnification to understand the overall morphology of the brachial plexus. Seven rabbits underwent electrophysiologic investigation. A bipolar nerve stimulator was used to systematically stimulate the roots, trunks and divisions, and nerve branches of the rabbit brachial plexus and compound muscle action potential was used to record muscle response. Nerve length and width measurements were not recorded.ResultsRoots contributing to the brachial plexus were C5, C6, C7, C8, and T1. In contrast to other anatomical studies, T2 did not contribute to the brachial plexus. The triceps was innervated by the radial nerve, which received contributions from C6 (1.6 mA), C7 (1.9 mA), C8, and T1 (12.2 mA).The biceps had dual innervation (proximally and distally). The proximal branch received contributions from C6 (3.5 mA) and C7 (5mA). The distal portion was innervated by a branch from the median nerve, which received innervation from C6, C7, C8, and T1.ConclusionsThe overall structure of rabbit brachial plexus is described and innervation of the biceps and triceps is described in detail. This anatomic investigation will form the basis of a future brachial plexus model of injury and repair.

Background Myoglobin-related renal injury (MRRI) is a significant cause of acute renal injury and may result in death. Common etiologies in forensic pathology include trauma and drug abuse. This study sought to determine the prevalence and causes of MRRI at autopsy and discuss diagnostic challenges. Methods The institutional autopsy archives were queried for cases in which a myoglobin (MG) immunohistochemical stain was performed or MRRI was diagnosed or considered. The clinicopathological characteristics of this population were described. Results Of 9996 cases over a period of 19.5 years, 17 cases (0.2%) fulfilled the criteria. Patient age ranged from 15 to 74 years; 11 were men. The MG stain was performed in 13 cases. In 11 (65%) cases, MRRI was diagnosed with or without immunohistochemistry. The clinical backgrounds were varied; sepsis and liver injury predominated. Time between original insult and death ranged from seven hours to four months. Patterns of MRRI included cast formation or fine brush border staining in the proximal tubules. Discussion MRRI is infrequently considered in the autopsy setting. Many causes of MRRI identified in this series involved circumstances placing the death into medical examiner jurisdiction; the MG stain contributed to understanding the mechanism of death. Acute tubular injury with cast formation was observed in the majority of cases. Conclusion MRRI is common in a select group of autopsies but may be underappreciated. The MG stain is a useful and inexpensive tool to aid in the diagnosis of MRRI in autopsy pathology.

Acute myeloid leukemia (AML) is the most common form of acute leukemia among adults and accounts for the largest number of annual deaths due to leukemias in the United States. Recent advances have resulted in an expansion of treatment options for AML, especially concerning targeted therapies and low-intensity regimens. This portion of the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for AML focuses on the management of AML and provides recommendations on the workup, diagnostic evaluation and treatment options for younger (age <60 years) and older (age ≥60 years) adult patients.

Background:In paroxysmal nocturnal hemoglobinuria (PNH), the granulocyte clone size is often used as an indicator of disease severity. However, the prognostic value of changing clone size on clinical outcomes in PNH is not well understood.Aims:To assess the prognostic value of change in PNH granulocyte clone size on the risk of developing major adverse vascular events (MAVE) or high disease activity (HDA: elevated hemolysis [lactate dehydrogenase {LDH} ≥1.5 x upper limit of normal] and one or more PNH‐related symptom[s]) in patients with PNH.Methods:The International PNH Registry is the largest worldwide observational study of patients with a diagnosis of PNH or a detected PNH clone as low as 0.01%. This study assessed patients with a baseline value for PNH clone size (defined as the closest value to disease onset) and at least one additional clone size value, who were untreated with complement inhibitor therapy at enrollment, and had ≥12 months of untreated follow‐up. Patients were stratified by clone size at baseline and change in clone size was estimated using regression models of clone size vs duration from disease start, adjusted for baseline clone size. Patients were categorized as having stable, increasing, or decreasing clone size depending on the slope, where a change in clone size >10% per year was considered increasing/decreasing. Prognostic value of change in clone size was assessed using Poisson regression to estimate rate ratios of MAVE and logistic regression to estimate odds ratios of HDA during follow‐up, with stable clone size as the reference. Adjusted models included baseline LDH ratio category, history of bone marrow disease at baseline, baseline clone size, and duration.Results:As of April 3, 2018, a total of 1894 patients were available for analysis: cohort (COH) 1 (clone size >0.01% to ≤1%, n = 381); COH 2 (clone size >1% to ≤5%, n = 318); COH 3 (clone size >5% to ≤10%, n = 159); COH 4 (clone size >10% to ≤50%, n = 480); COH 5 (clone size >50% to ≤100%, n = 556). Median duration was 3.5 years. Clone size was stable in only 16.2% of patients, as compared with 45.3% increasing and 38.5% decreasing; stable clone size was primarily seen in patients with a clone size ≤1% at baseline (58% of patients in COH 1 with a stable clone), with less than 10% of patients with a stable clone in the other cohorts. Clone size change over time was highly prognostic of HDA; patients with increasing and decreasing clone size had an increased risk of HDA, compared to patients with stable clone size, although this may be due to patients with a stable clone largely in COH 1 with a low risk of HDA; after adjusting, we see no increased risk of HDA in patients with decreasing clone size, but the increased risk of HDA in patients with increasing clone size is maintained (Table). Due to limited data on covariates at baseline, only 156 patients were available for this adjusted analysis. There was no difference in the MAVE rate in patients with increasing or decreasing clone size as compared with those with a stable clone size. The lack of effect was maintained even after adjusting for covariates (Table).Summary/Conclusion:This is the first analysis of the prognostic value of PNH clone size changes on clinical outcomes over time in patients with PNH. Increasing clone size over time was predictive of patients experiencing HDA during their disease course. Interestingly, no difference in the MAVE rate was seen in patients with increasing or decreasing clone size, suggesting that change in clone size does not change the increased risk of MAVE in patients with PNH.image