bluuebunny/crossref_metadata_2025_split huggingface dataset:
Liver cirrhosis complicated with pulmonary Nocardia infection: A case report and literature review
Authors: Xiao Yan , Wang Hongwei , Tian Tingting , Zheng Juanjuan , Liu Mengyang , Wang Qing , Li Jing 
Published: December 2024

Rationale:

Nocardia is a conditionally pathogenic bacterium found in the natural environment and is an aerobic bacterium, which causes severe pneumonia relatively rarely. The diagnosis of primary Nocardia infection is always made late due to the nonspecific clinical presentation of patients with Nocardia infection and the time required for Nocardia culture. Due to its rarity and nonspecific clinical presentation, patient survival is often compromised due to misdiagnosis.

Patient concerns:
A 53-year-old woman with autoimmune cirrhosis was treated with glucocorticoids. Three and a half months later the patient developed fever, nausea, cough and sputum, treated with empirical antibiotics, and the patient occurred type I respiratory failure.

Diagnoses:

Nocardia was identified by sputum culture micromorphology and Meriere matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF).

Interventions:
The patient was treated with trimethoprim-sulfamethoxazole (TMZ), and the patient’s oxygen saturation recovered significantly.

Outcomes:
The patient’s overall recovery was slow due to decreased albumin (ALB) and increased procalcitonin (PCT) caused by the patient’s cirrhosis. Due to economic reasons, the family gave up, asked to be discharged.

Lessons:
In our report, patients with underlying disease are immunocompromised and at increased risk of infection with conditionally pathogenic bacteria. Nocardia has no specific presentation and is often overlooked clinically. Treatment of these patients should not be limited to common bacteria or viruses, but should consider rare opportunistic pathogens, and we need to be vigilant for Nocardia infections and timely use of sulfonamide antibiotics to reduce mortality.
Resolution of acute motor axonal neuropathy in a patient after treatment with efgartigimod: A case report
Authors: Zhou Jinli , Yu Weifei , Ding Siqi , Shi Chanhong , Liang Hui 
Published: December 2024

Background:
Guillain-Barré syndrome (GBS) is an acute autoimmune neuropathy characterized by progressive muscle weakness, often caused by immunoglobulin G (IgG) autoantibodies. There are several subtypes of GBS, of which acute motor axonal neuropathy (AMAN) is one of the most severe subtypes associated with axonal damage. It is well known that the current clinical standard of treatment is intravenous immunoglobulin (IVIg) and plasma exchange (PLEX), but some patients often show limited response or experience persistent disability. Efgartigimod, an Fc fragment of human IgG antibody, provides a way to target and reduce pathogenic IgG antibodies as a natural ligand Fc receptor (FcRn). The purpose of this study was to observe the therapeutic effect of efgartigimod on axonal GBS, which is expected to be a potential therapeutic method for GBS and AMAN.

Methods:
We present a case of a 58-year-old man diagnosed with AMAN, presenting with ascending symmetrical limb weakness, flaccid paralysis, and multiple cranial nerve palsies. Electromyography confirmed the axonal subtype of GBS. Despite receiving IVIg and PLEX, the patient showed suboptimal recovery. Subsequently, he was treated with efgartigimod at a dose of 10 mg/kg weekly for 4 weeks, demonstrating significant improvement in both clinical symptoms and electromyographic findings, with good tolerability.

Result:
This case highlights the potential efficacy and safety of a 4-dose efgart-igimod regimen in AMAN, particularly for patients with inadequate response to conventional therapies. By targeting FcRn and promoting IgG degradation, efgartigimod offers a novel mechanism to modulate the aberrant immune response underlying AMAN.

Conclusion:
Efgartigimod at a dose of 10 mg/kg weekly for 4 weeks demonstrated promising results in this case of AMAN. While further research is warranted, our findings suggest that efgartigimod may represent a valuable addition to the therapeutic armamentarium for AMAN and potentially other autoimmune neurological conditions. Well-designed clinical trials are crucial to confirm these findings and establish optimal treatment protocols for efgartigimod in AMAN.
Evaluating the efficacy of full-scale and tabletop exercises in enhancing paramedic preparedness for external disasters: A quasi-experimental study
Authors: Alakrawi Ghadeer A. , Al-Wathinani Ahmed M. , Gómez-Salgado Juan , Alobaid Abdullah M. , Abahussian Mohammed , Alhazmi Riyadh , Mobrad Abdulmajeed , Jebreel Albaraa , Althunayyan Saqar , Goniewicz Krzysztof 
Published: December 2024

Background:
The increasing frequency and severity of disasters worldwide needs effective disaster management training to enhance the preparedness and response capabilities of emergency responders. This study compares the outcomes of tabletop exercises (TTEs) and full-scale exercises (FSEs) in training senior paramedic students at Prince Sultan bin Abdul Aziz College for Emergency Medical Services, King Saud University, Riyadh, Saudi Arabia.

Methods:
A nonequivalent two-group quasi-experimental design was used. Forty-five senior paramedic students who had completed a disaster management course were randomly assigned to either TTE or FSE groups. Both groups participated in a simulated multi-vehicle accident scenario. Data were collected using an adapted CLUT Scale survey, which measured learning, trust, and usefulness immediately after the exercises. The Mann–Whitney U test was applied to compare outcomes between the 2 groups.

Results:
Both TTE and FSE resulted in high levels of perceived learning, trust, and usefulness among participants, indicating a positive impact on disaster preparedness. While the TTE group scored slightly higher on average across all variables, these differences were not statistically significant, suggesting that both exercise types are similarly effective. The findings underscore the cost-effectiveness of TTEs and their potential to foster collaborative learning environments

Conclusions:
Both TTE and FSE are effective in enhancing disaster preparedness among senior paramedic students. Given their adaptability and cost-effectiveness, TTEs may be particularly valuable for resource-constrained training settings. Further research with larger, more diverse samples and pre-/post-intervention assessments is recommended to validate these findings and improve disaster management training programs.
Lipoma arborescens in a 22-year-old male: A case report
Authors: Li Mingyang , Ding Ling , Nie Qilong , Jiang Zeping 
Published: December 2024

Rationale:
Lipoma arborescens (LA) is a rare, benign proliferative disorder of the synovial lining that typically affects middle-aged individuals, predominantly in the knee joint. However, its occurrence in younger patients is unusual and may pose unique diagnostic and therapeutic challenges. This case report aims to highlight the clinical, imaging, and therapeutic aspects of LA in a young adult, thereby expanding the understanding of its presentation in this age group.

Patient concerns:
A 22-year-old male presented with acute swelling and pain in the right knee following a minor sprain. Although the symptoms persisted and worsened over several days, the patient was particularly concerned about his prognosis and the possibility of recurrence. Given the rarity of LA, he expressed anxiety about being diagnosed with such an uncommon condition at his age and feared the potential impact on his future mobility. He was also apprehensive about the risks of surgery and questioned why he, at such a young age, was affected by this rare condition.

Diagnoses:
Initial clinical assessment was inconclusive, leading to further investigations. Magnetic resonance imaging findings suggested a meniscal tear and the presence of LA, characterized by fat globules and joint effusion, which was confirmed during arthroscopic surgery.

Interventions:
The patient underwent arthroscopic surgery involving trimming of the medial meniscus and debridement of the synovium. Surgical findings included extensive synovial proliferation and intra-articular fat globules, which were histologically confirmed as LA.

Outcomes:
Postoperative recovery was successful, with significant improvement in joint function and alleviation of symptoms. Follow-up magnetic resonance imaging confirmed the absence of abnormal synovial proliferation.

Lessons:
This case report underscores the diagnostic and therapeutic challenges associated with LA in a young patient, highlighting the importance of considering LA in differential diagnoses of acute knee swelling, especially when initial evaluations are inconclusive. Through a combination of imaging, arthroscopic intervention, and histopathological confirmation, this case illustrates an effective approach for accurately diagnosing and managing LA. By documenting this atypical presentation in a young adult, this report contributes to the limited literature on LA in younger populations, aiming to enhance clinical awareness and guide future cases involving similar joint lesions.
A rare case report of infective endocarditis caused by Enterococcus gallinarum following renal laser lithotripsy
Authors: Wang Ruoxin , Lv Meng , Fu Liang , Zhao Jinlong , Ni Yinkai , Li Tianyu , Chen Zonghui , Li Feng 
Published: December 2024

Rationale:

Enterococcus gallinarum is a part of the normal fecal microbiota in the general population and animals, and is rarely isolated in clinical specimens. Due to the increasing use of immunosuppressants, invasive treatments, and overuse of antibiotics, infections caused by enterococci are gradually increasing.

Patient concerns:
A 48-year-old man was admitted to our hospital due to a persistent fever for 1 month after renal laser lithotripsy.

Diagnoses:
The cardiac ultrasound showed a mass on the mitral valve leaflet of the left atrium. The metagenomic next-generation sequencing test results of blood and vegetation were positive, reporting a large number of characteristic reads of E. gallinarum. The patient was diagnosed with infective endocarditis.

Interventions:
Mechanical mitral valve replacement was performed, and daptomycin was administered during the perioperative period.

Outcomes:
After 4 weeks of antibiotic treatment, the patient’s inflammatory indexes were normal, and no abnormalities such as fever were found. Blood culture and metagenomic next-generation sequencing test results were negative. The patient was then discharged from the hospital.

Lessons:
This case emphasizes the possibility of E. gallinarum developing severe invasive infections after kidney surgery. Clinical doctors should strengthen their understanding of this type of bacteria, understand their sensitive characteristics and treatment principles.
Bibliometric analysis of autophagy in NAFLD from 2004 to 2023
Authors: Xu Sumei , Zhang Yating , Huang Qi , Xie Yiwen , Tong Xiaojuan , Liu Haoge 
Published: December 2024

Background:
Autophagy is a cellular process in which damaged organelles or unnecessary proteins are encapsulated into double-membrane structures and transported to lysosomes for degradation. Autophagy plays a crucial role in various liver diseases, including nonalcoholic fatty liver disease. This study aims to elucidate the role of autophagy in nonalcoholic fatty liver disease through bibliometric analysis.

Methods:
Literature was retrieved from Web of Science CoreCollection database, and the search time was from January 01, 2004 to December 31, 2023. Data retrieval was performed using the Bibliometrix package in R software. VOSviewer and CiteSpace were utilized to visualize the research hotspots and trends related to the effect of autophagy on nonalcoholic fatty liver disease.

Results:
A total of 966 papers were obtained, published in 343 journals from 1385 institutions across 57 countries. The journals with the most publications were the “International Journal of Molecular Sciences” and “Scientific Reports.” China had the highest number of published papers. The most productive authors were Yen Paul M and Jung Tae Woo, while Singh R was the most frequently co-cited author. Emerging research hotspots were associated with keywords such as insulin resistance, ferroptosis, endoplasmic reticulum stress, and mitochondrial function.

Conclusion:
Research on autophagy in nonalcoholic fatty liver disease is still in its early stages, with a growing body of literature. This study is the first to provide a comprehensive bibliometric analysis, synthesizing research trends and advancements. It identifies current development trends, global cooperation models, foundational knowledge, research hotspots, and emerging frontiers in the field.
Clinical and genetic characteristics of a case of Koolen-De Vries syndrome caused by KANSL1 gene mutation and literature review: A case report
Authors: Zhang Haozheng , Yuan Limei , Fan Meili , Liu Zhaotian , Yan Yuxi , Liu Qinghua , Zhang Kaihui , Li Chunmiao , Liu Deyao 
Published: December 2024

Rationale:
Koolen-De Vries syndrome (KdVS, OMIM: 612452), also known as 17q21.31 microdeletion syndrome, is an autosomal dominant genetic disease. In the study, we analyze of clinical phenotype and gene variation of a child with Koolen-De Vries syndrome, review the literature to improve the understanding of the disease.

Patient concerns:
The patient is a male, aged 1 month and 3 days. The patient has poor airway development, difficulty weaning from respiratory support, seizures, and recurrent low granulocyte counts.

Diagnoses:
High-throughput sequencing showed a heterozygous mutation NM_001193466.1: c.1574_1578del (P.525HFS *24) in the KANSL1 gene of the proband, which was considered a new mutation since neither of his parents carried this mutation based on Sanger sequencing results. Combining clinical features and genetic results, the proband was diagnosed as KdVS.

Interventions and outcomes:
The patient was in good condition after receiving bronchoscopy and laser interventional therapy, meeting the criteria for discharge. Follow-up for 1 year and 6 months indicated that the patient’s physical signs were normal and there was no recurrence.

Lessons:
According to literature review, KdVS is a multi-organ disease characterized by feeding difficulties, seizures, characteristic facial features, dysplasia of the respiratory system and cardiac abnormalities. In this study, laryngeal malacia accounted for 23.2% of the clinical manifestations of KdVS patients, limb convulsions/seizures accounted for 62.5%, and cardiac development defects accounted for 23.5%. The disease was rare in China and had a variety of clinical manifestations. The summary of reported cases can enable doctors to have more understanding of the disease. The new mutations enrich the KANSL1 gene mutation spectrum.
Ethnicity as a Risk Factor for Early Neurological Deterioration
Authors: Goldstein Eric D. , Liew Sabrina Q.R. , Shu Liqi , Rocha Alicia , Yaghi Shadi 
Published: December 2024

Objectives:
Nearly 25% of those with a small vessel stroke will develop early neurological deterioration (END). The objectives of this study were to identify clinical risk factors for small vessel stroke-related END and its associated impact on functional outcomes in an ethnically diverse data set.

Methods:
We performed a post hoc analysis of the “Secondary Prevention of Small Subcortical Strokes” trial. The primary outcome was END defined as progressive or stuttering stroke-related neurological symptoms. Standard descriptive and inferential statistical methods were used for analysis. Functional outcomes are reported by modified Rankin Scale score and analyzed by the Wilcoxon signed-rank test.

Results:
In all, 69 participants met the inclusion criteria; 21 (30%) had END. Of the cohort, Spanish, Hispanic, or Latino ethnicity (grouping per trial definition) most frequently developed END [11 (52.4%) vs 4 (8.3%), P < 0.001] with a higher adjusted likelihood of END (odds ratio: 14.1, 95% CI: 2.57-76.7, P = 0.002). Black or African-American race less commonly had END [3 (14.3%) vs 21 (43.8%), P = 0.03] but lost significance after adjustment (odds ratio: 1.46, 95% CI: 0.26-8.17, P = 0.67) due to powering. END was associated with a higher mean modified Rankin Scale (2.06 ± 0.94 vs 1.17 ± 0.79, P = 0.006) but did not differ in the shift analysis.

Conclusions:
We found that Spanish, Hispanic, or Latino ethnicity was the most consistent risk factor for END though it was without meaningful functional outcome differences.
Childhood Familial Mediterranean Fever in the United States
Authors: Fathalla Basil M. , Thomas Ronald 
Published: December 2024

Objectives
The aim of this study was to report the spectrum of Familial Mediterranean Fever (FMF) in children living in Southeast Michigan.

Methods
We reviewed prerecorded data in medical records of FMF patients. Statistical analysis of the data included Fisher exact test, Pearson χ2 procedure, parametric independent samples t test, and parametric analysis of variance using SPSS Version 29.0, IBM Inc.

Results
The study included 29 males and 21 females. The mean age at presentation was 4.63 ± 3.66 years, and the mean time to diagnosis was 2.1 ± 2.18 years. A slight majority presented in the first 3 years of age (54%). Family history of FMF was reported in only 58% of patients. Clinical manifestations included fever (84%), gastrointestinal (84%), musculoskeletal (64%; including chronic arthritis, sacroiliitis, and nonbacterial osteomyelitis), chest (28%), cutaneous (14%), and other manifestations (16%). Fever without other manifestations was reported only in patients presenting at ≤3 years of age (p = 0.016), whereas older patients reported more gastrointestinal manifestations (p = 0.04). Reported MEFV variants included p.M694V (n = 26), p.V726A (n = 23), p.M694I (n = 13), and others (n = 10). Homozygote and compound heterozygote patients had more gastrointestinal manifestations (p < 0.001), whereas fever was more common in the heterozygote patients (p = 0.04). The mean follow-up period was 5.34 ± 4.13 years with no renal disease.

Conclusions
We report the largest childhood FMF cohort in the United States. A negative family history should not preclude consideration of FMF as a cause of periodic fever. Recurrent fever can be the only manifestation, particularly in young patients with FMF. The absence of fever and chronic progressive musculoskeletal manifestations can uncommonly occur.
Protein Kinase C-β Inhibition and Survival Signaling after Simulated Cardioplegic Ischemia/Reperfusion in Non-Diabetic and Diabetic Human Coronary Arterial Endothelial Cells
Authors: Nho Ju-Woo , Banerjee Debolina , Harris Dwight D , Stone Christopher , Xing Hang , Kanuparthy Meghamsh , Li Janelle , Sellke Frank W , Feng Jun 
Published: December 2024

Background:
Cardioplegic ischemia/reperfusion (I/R) injury poses substantial challenges during postoperative recovery, with diabetic patients particularly susceptible to adverse events. Using a model entailing the subjection of human coronary artery endothelial cells (HCAECs) to simulated cardioplegic I/R, we investigated the potential of protein kinase c β (PKC-β) inhibition to augment cellular survival in this context.

Study Design:
HCAECs were isolated from harvested coronary arteries of diabetic (D) and nondiabetic (C) patients (N = 4 per group). HCAECs were either cultured in normoxic conditions without drug (D and C), subjected to hypoxia/reoxygenation alone (DH and CH), or subjected to hypoxia/reoxygenation and the PKC-β inhibitor LY333531 (DHT and CHT). Molecular signaling was assessed using immunoblotting.

Results:
Simulated I/R decreased anti-apoptotic phosphorylated protein kinase b (p-Akt, p = 0.04) and p-Akt:Akt ratio (p = 0.004) in CH versus C, with PKC-β inhibition restoring expression in CHT (p ≤ 0.04). Treatment also increased p-Akt:Akt ratio in DHT versus D (p = 0.03). Anti-apoptotic inducible nitric oxide synthase increased in CHT versus CH (p = 0.003), and pro-apoptotic phosphor-FOXO:FOXO ratio decreased in CHT versus CH (p = 0.001). I/R elevated Bcl-2-associated agonist of cell death (BAD) in CH versus C (p = 0.01), but PKC-β inhibition increased anti-apoptotic p-BAD (p = 0.001) and p-BAD:BAD ratio (p = 0.03) in CHT. I/R also increased cleaved PARP (p < 0.001) and cleaved caspase 3 (p < 0.001) in DH versus D, both of which were reversed by treatment (p < 0.001 for DHT versus DH).

Conclusion:
PKC-β inhibitor treatment increased pro-survival signaling and decreased pro-apoptotic signaling in nondiabetic and diabetic HCAECs subjected to simulated I/R, with mechanistic differences observed between these cohorts.
Dexmedetomidine and Nitrous Oxide as Anesthetic Agents for Electroconvulsive Therapy
Authors: Chung In Won , Kim Heung Sik , Lee Junhee , Kim Jee Hee , Kim Sang Ha , Kim Yong Sik 
Published: December 2024

Abstract
Electroconvulsive therapy (ECT) is a therapeutic intervention that induces generalized seizures under general anesthesia. This case report compares the efficacy of dexmedetomidine (DEX) and nitrous oxide (N2O) to that of propofol during ECT procedures. A 33-year-old woman with a 15-year history of schizophrenia and recurrent psychotic episodes underwent ECT. During a previous hospitalization, she underwent 14 ECT sessions using propofol and succinylcholine. The mean electrical stimulation dose was 598.9 ± 237.6 mC, with mean motor and electroencephalogram seizure durations of 23.8 ± 8.0 and 34.9 ± 8.3 seconds, respectively. During her current hospitalization, she underwent an additional 14 ECT sessions using DEX and N2O. The mean electrical stimulation dose was significantly lower, at 239.4 ± 54.7 mC, with comparable motor and electroencephalogram seizure durations of 34.6 ± 11.6 and 36.3 ± 12.5 seconds, respectively. Our case report emphasizes the effectiveness of using DEX and N2O for ECT anesthesia compared to propofol. Despite a significantly lower mean electrical stimulation dose requirement with DEX and N2O (t = 5.5155, P < 0.00001), no significant differences were observed in electroencephalogram seizure duration between the groups.
Translation and Critical Evaluation of the Columbia University Autobiographical Memory Interview—Short Form for use in the Dutch Language
Authors: Nwatarali Chioma , Verwijk Esmée , Blokland Dominique , Bergfeld Isidoor O. 
Published: December 2024

Introduction
Assessment of autobiographical memory following electroconvulsive therapy is lacking multilingual options. We therefore developed the Dutch translation of the Columbia University Autobiographical Memory Interview—Short Form (CUAMI-SF). We aimed to assess its practical use and identify segments for potential revision to improve the interview’s applicability.

Methods
The CUAMI-SF comprises 30 questions asking details about 6 segments (eg, last trip, last birthday) and 7 question categories (eg, dates, list of persons present). The interview was translated to Dutch using blind back-translation principles. We evaluated practicality and potential redundancy, through surveying a focus group and quantitative analyses of 60 participants, including depressed patients treated with electroconvulsive therapy (n = 14) or deep brain stimulation (n = 25) and healthy controls (n = 21). For the quantitative analyses, we compared the average percentage of nonanswered questions at baseline between groups. The average consistency scores after a follow-up of approximately 6 weeks were also calculated and compared for each segment between the groups.

Results
The focus group mostly found the CUAMI-SF easy to use and to score, but argued redundancy in specific questions asking for dates, numbers, and addresses. The largest differences in consistency scores between the groups were present for the segments “travel,” “birthdays,” “New Year’s Eve,” and “doctor’s visits,” indicating higher sensitivity compared with “family member” and “job.”

Discussion
The CUAMI-SF was found easy to use by administrators. The segments “family member” and “job” emerged as less sensitive. Therefore, removal might maintain sensitivity while reducing administration time, which could prove beneficial in clinical and research settings.