Citations

We report the case of a term neonate who was somnolent at birth with ventilatory distress and experienced 2 seizures shortly after delivery. Laboratory tests revealed the neonate had a serum sodium of 113 mmol/L. The seizures stopped after treatment with midazolam, and the sodium was corrected slowly with 3% hypertonic saline without further sequelae. The severe neonatal hyponatremia and seizures were attributed to maternal consumption of excessive amounts of coconut water during labor. This case demonstrates the importance of careful consideration of both fluid volume and fluid electrolyte composition during labor to prevent adverse maternal and neonatal outcomes.

Objective We provide evidence‐based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs). Methods We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A panel of clinicians and patients reached consensus on the direction and strength of the recommendations. Results Thirty‐five recommendations were generated (including two strong recommendations) for first‐line SARD‐ILD treatment, treatment of SARD‐ILD progression despite first‐line ILD therapy, and treatment of rapidly progressive ILD. The strong recommendations were against using glucocorticoids in systemic sclerosis–ILD as a first‐line ILD therapy and after ILD progression. Otherwise, glucocorticoids are conditionally recommended for first‐line ILD treatment in all other SARDs. Conclusion This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the treatment of ILD in people with SARDs.

ObjectiveWe provide evidence‐based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease.MethodsWe developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation.ResultsFifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high‐resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6‐minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs.ConclusionThis clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.

Objective We provide evidence‐based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease. Methods We developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation. Results Fifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high‐resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6‐minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs. Conclusion This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.

Abstract Background Prevalence of metabolic dysfunction‐associated steatotic liver disease (MASLD), previously known as nonalcoholic fatty liver disease (NAFLD), and its sequelae of more severe forms such as metabolic dysfunction‐associated steatohepatitis (MASH) is rapidly increasing in children with the rise in obesity. Successful and sustainable treatments for MASLD are lacking in children. We determined the therapeutic effect of N ‐acetyl cysteine (NAC) on biomarkers of oxidative stress, inflammation and insulin resistance (IR), liver enzymes, liver fat fraction (LFF) and liver stiffness (LS) in children with obesity and biopsy‐confirmed MASLD. Methods Thirteen children ( n = 13; age: 13.6 ± 2.8 years; NAS score >2) underwent a double‐blind, placebo‐controlled trial of NAC (either 600 or 1200 mg NAC/day) or placebo for 16 weeks. Measurements included LFF (magnetic resonance imaging), LS (ultrasound elastography), and body composition. Erythrocyte glutathione (GSH), liver enzymes, insulin, glucose, adiponectin, high‐sensitivity c‐reactive protein (hs‐CRP), and interleukin‐6 (IL‐6) were also measured. homeostasis model assessment for insulin resistance (HOMA‐IR) was calculated. Results Sixteen‐week NAC treatment improved (baseline adjusted between‐group p < .05 for all) markers of inflammation (IL‐6 and hs‐CRP), oxidative stress (GSH), and IR (HOMA‐IR) and reduced liver enzymes, LFF and LS. Body weight and body composition did not show beneficial changes. Conclusions Sixteen‐week NAC treatment was well tolerated in children with obesity and MASLD and led to improvements in oxidative stress, inflammation and IR and liver outcomes. The results from this pilot study support further investigation of NAC as a therapeutic agent in children with MASLD.

AbstractThe role of long‐term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long‐term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro‐intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut–brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro‐gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify – and hopefully reduce the potential for harm associated with – the use of long‐term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life‐threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time‐limited period to achieve nutritional safety, while the wider multi‐disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition.

BACKGROUND Among the spectrum of adipose lesions associated with peripheral nerves, complete circumferential encasement of the nerve by the lesion is a very rare entity. The authors report the first case of a giant lipoma that completely and circumferentially encased the radial nerve. OBSERVATIONS A 57-year-old woman presented with a large left-arm mass that she had noticed over a year following significant weight loss. She had hypersensitivity and dysesthesias in the left posterior arm but no weakness or other symptoms. Magnetic resonance imaging of the left upper limb demonstrated a large lipoma completely encasing the radial nerve, originating at the level of the spiral grove proximally and extending distally to the lateral epicondyle. The lesion was completely resected at surgery with total preservation of radial nerve function. LESSONS Lipomas encasing peripheral nerves can be difficult to manage while preserving function. This case adds to the intriguing spectrum of adipose lesions associated with peripheral nerves. https://thejns.org/doi/10.3171/CASE24148

Background Mechanical thrombectomy has become the standard of care for treatment of acute ischemic stroke secondary to large‐vessel occlusion up to 24 hours from last known normal time. Multiple different techniques for mechanical thrombectomy have been described, including a direct aspiration first‐pass technique and stent retriever thrombectomy. With a direct aspiration first‐pass technique, classically, a large‐bore aspiration catheter is delivered over a microcatheter and microwire to the clot. Recently, a novel macrowire has been introduced as a potential alternative to the use of microwire–microcatheter to allow the delivery of the aspiration catheter. The aim of this study is to develop a multicenter registry comparing delivery of an aspiration catheter for intracranial thrombectomy for acute ischemic stroke secondary to emergent large‐vessel occlusion over a macrowire in comparison with traditional use of microcatheter and microwire. Methods MINT (Macrowire for Intracranial Thrombectomy) is a multicenter, observational study currently enrolling patients with large‐vessel occlusion who underwent mechanical thrombectomy using a macrowire to deliver the aspiration catheter to the intracranial occlusion. All the participating sites will screen and report cases on a monthly basis. The decision to use the macrowire and various aspiration catheters is at the discretion of the interventionalist. Results We will collect patient's clinical, demographic, and radiographic data. In addition, we plan to collect procedure variables and postprocedure clinical and imaging data. Outcomes include successful delivery of the reperfusion catheter to the clot interface, time taken from groin access to first pass, and a bailout strategy for thrombectomy in cases where this is not feasible. Conclusion The MINT registry will add to our understanding of safety and efficacy of this novel macrowire in intracranial thrombectomy. This registry will also highlight and allow for understanding in workflow improvements from simplifying setup and possibly cost effectiveness of this technique.