Beijing United Family Hospital

This study analyzed the clinical features of central nervous system involvement (CNS3) in pediatric anaplastic large cell lymphoma (ALCL) and evaluated the CNCL-ALCL-2017 treatment protocol efficacy. The clinical data of 215 pediatric ALCL patients aged ≤18years, including 16 with CNS3, treated following the CNCL-ALCL-2017 protocol, were enrolled. Independent risk factors of CNS3 were determined using multi-factor logistic regression analysis, and survival curves were constructed. Brain parenchyma/spinal cord involvement, cranial nerve deficits, and cerebrospinal fluid (CSF) positivity were found in 11/16, 5/16, and 7/16 CNS3 patients, respectively, the latter including six positive on flow cytometry, one NPM1-ALK positive on PCR. Immunohistochemistry showed ALK and CD3 positivity in 15/16 and 13/16 patients, respectively. Peripheral blood ALK+ and bone marrow ALK+ were found in 10/16 and 9/16 patients, respectively. All CNS3 patients were treated with Regimen C. Multi-factor logistic regression analysis identified ear, nose, and throat involvement and CD3 expression as independent risk factors of CNS involvement. The median follow-up period was 35.4 months (range: 0.5-74.9 months). The 3-year overall and event-free survivals for the CNS3 group were 93.3%±6.4% and 86.2%±9.1%, respectively, with no differences from the whole cohort.

Although plenty of evidence supports the effectiveness of sacral neuromodulation (SNM) in improving urination and defecation, few studies concerned its effect on the patients with spinal dysraphism (SD). This study aimed to evaluate the effects of SNM on SD-induced neurogenic bladder and bowel dysfunction. We prospectively followed the patients with SD who underwent SNM in our department from May 2019 to June 2024. Fusion images of sacrococcygeal computed tomography and magnetic resonance sacral plexus nerve images were used as essential references for preoperative evaluation and intraoperative implantation. Patient's subjective improvement by ≥50% from baseline was defined as implantation procedure success. Among the included patients, urodynamics, urinary ultrasonography, daily urination frequency, daily urine leakage, and neurogenic bowel dysfunction score were compared before and after SNM and between unilateral and bilateral SNM. A total of 44 patients were included, of whom 97.73% reported improved symptoms and 47.73% achieved implantation procedure success. SNM significantly improved the average postvoid residual volume, maximum cystometric capacity, bladder compliance, daily urination frequency, daily urine leakage, and neurogenic bowel dysfunction score. We found a significantly higher success rate in the patients stimulated bilaterally compared with those stimulated unilaterally but no intergroup differences against age (between minors and adults), sex, lower urinary tract symptoms, and intestinal symptoms. Bilateral SNM performed significantly better in improving postvoid residual volume, daily urination frequency, and daily urine leakage than unilateral SNM. No SNM-related complications were reported during follow-up. SNM can improve urination and defecation in the patients with SD safely and effectively. We strongly recommend fusion imaging of sacrococcygeal computed tomography and magnetic resonance sacral plexus imaging as a valuable and promising technique for preoperative evaluation and intraoperative implantation. A flexible implantation strategy involving electrode location and number helps achieve ideal modulation effects in the patients with SD.

Chronic knee conditions and musculoskeletal injuries are prevalent and pose significant challenges to global healthcare systems. Current diagnostic methods, such as MRI and X-ray, provide valuable insights but are limited by high costs and the inability to continuously monitor knee health. In response to these challenges, the proposed work makes the following contributions. Firstly, a novel wearable knee health monitoring system is developed, incorporating a flexible composite piezoelectric film that captures subtle patellar displacements as early indicators of knee joint abnormalities. Secondly, a Bayesian machine learning framework incorporating both expert knowledge and diagnostic data is proposed to address the potential limitations of current machine learning-based diagnostic methods, such as low accuracy and poor interpretability in medical practice. This framework integrates causal discovery algorithms, clinical insights, and medical expertise to identify the key relationships between machine learning models and medical principles, ultimately improving diagnostic accuracy. Finally, the proposed system achieves an 83.3% success rate in distinguishing between healthy, sub-healthy, and unhealthy knee states, outperforming traditional methods with an accuracy improvement of 6.4% to 17%. Furthermore, the system integrates a wireless sensor network and user-friendly software, enabling convenient monitoring and diagnosis in both clinical and home settings. The main innovation of this study lies in the design of a wearable sensor network structure based on patellar activity and a medical knowledge-guided Bayesian framework to enable a three-state classification diagnosis, namely, healthy, sub-healthy, and unhealthy. This system is particularly suited for knee health assessments in outpatient clinics, community settings, and home environments..

Background:The potential risk of neuropsychiatric adverse events (NPAEs) associated with oseltamivir has been controversial since its market launch, with inconsistent conclusions across different studies, and the product label does not clearly describe the role of oseltamivir in these adverse events.Methods: This article integrates multisource evidence through evidence-based methods, critically evaluates and analyzes key evidence based on the hierarchy of evidence pyramid, and clarifies the specific association between oseltamivir and psychiatric adverse events, the strength of the association, and high-risk factors, providing a scientific basis for clinical decision-making and regulatory measures.Results: Based on current evidence, oseltamivir is unlikely to increase the risk of psychiatric adverse events under the standard dosage and administration guidelines and label instructions, irrespective of its use for the treatment or prevention of influenza.However, prolonged exposure (for 6 weeks, number needed to harm of 94), high-dose regimen (150 mg, twice daily, number needed to harm of 150), and adolescents aged 10-19 years (adjusted HR 2.69, 95% CI 1.05-6.93)may be high-risk factors for psychiatric adverse events. Conclusion:Clinical applications should extensively consider the indications, weigh the benefits and risks, and use the drug rationally; psychiatric-related adverse events should not be solely attributed to oseltamivir.

BACKGROUNDComplications arising from the polyps in Peutz-Jeghers syndrome (PJS) have historically been addressed through surgical treatment. Enteroscopic polypectomy is hypothesized to reduce the risk of surgery in PJS. However, the optimal timing for polyp screening and preventive intervention using enteroscopic polypectomy remains uncertain. This is primarily due to the extremely low incidence of the condition and the paucity of data regarding the natural risk of requiring surgery and its age distribution in PJS patients. In order to develop recommendations on the appropriate timing of polyp screening and preventive intervention in PJS, a comprehensive understanding of the natural surgical risks and their age distribution is essential.AIMTo investigate the natural surgical risks associated with polyps in PJS and to clarify their age distribution.METHODSA web-based open survey was launched to collect information from Chinese individuals suspected of having PJS. The questionnaire was distributed to the PJS instant messaging groups using a quick response code method. The data were analyzed using descriptive statistical methods, and the cumulative incidence of surgery was calculated using the Kaplan-Meier method.RESULTSOf the 442 patients enrolled, 301 (68.10%) had undergone 506 surgical procedures prior to enteroscopy or the survey deadline. Among the 506 surgical procedures, 388 (76.68%) were performed on patients aged between 6 and 25 years. The cumulative incidence rates of the first surgical procedure at 5, 10, 15, 20, 25, and 30 years of age were 5.0% (95% confidence interval [CI]: 2.9%-7.0%), 20.6% (95%CI: 16.6%-24.4%), 40.5% (95%CI: 35.5%-45.1%), 58.0% (95%CI: 52.7%-62.7%), 72.6% (95%CI: 67.3%-77.0%), and 82.4% (95%CI: 77.0%-86.5%), respectively. The primary indications for the first surgical procedures were intussusception (81.40%), obstruction (13.95%), and gastrointestinal bleeding (4.65%). The cumulative incidence rates of requiring a second surgical procedure within 1, 3, 5, 10 and 15 years following the first surgical procedure were 3.7% (95%CI: 1.5%-5.8%), 12.5% (95%CI: 8.6%-16.2%), 20.3% (95%CI: 15.6%-24.8%), 37.0% (95%CI: 33.1%-45.3%), 54.2% (95%CI: 46.8%-60.5%), respectively. Patients who underwent their first surgical procedure at the age of nine years or younger presented an elevated risk of requiring a second surgical procedure (P < 0.01).CONCLUSIONChinese patients with PJS have a high natural risk of undergoing surgery. Without preventive intervention, these procedures may become necessary at an early age and may be repeated. Early screening and regular surveillance, with preventive intervention if necessary, should commence at six years of age.

A 37-year-old man presented with recurrent, severe left-sided headaches occurring at the same time each evening, accompanied by ipsilateral lacrimation, conjunctival injection, and nasal congestion. The clinical features were suggestive of cluster headache (CH), but poor response to high-flow oxygen and triptans raised concern for a secondary cause. Neuroimaging revealed an ethmoid sinus mucocele with adjacent bone remodeling, mimicking trigeminal autonomic cephalalgia. Surgical drainage led to complete symptom resolution, suggesting a secondary headache etiology. This case highlights the need to consider structural causes in atypical or treatment-resistant headache presentations and the importance of neuroimaging in accurate diagnosis and management.

Objective: To evaluate the effectiveness and safety of robot-assisted neck dissection via retroauricular approach for head and neck squamous cell carcinoma patients. Methods: A retrospective analysis was conducted on 22 patients with head and neck squamous cell carcinoma who underwent robot-assisted neck dissection via the retroauricular approach at Beijing United Family Hospital from March 2018 to August 2023. Among them, 6 were female and 16 were male, with an age range of 39-75 years. Preoperative staging showed N0 for 9 patients (41%), N1 for 12 patients (55%), and N2 for 1 patient (4%). The relevant clinical pathological and follow-up data were collected, and the effectiveness and safety of this surgical approach were analyzed. Kaplan Meier method was used to analyze the patient survival rate. Results: All patients were completed the robot-assisted neck dissection without conversion to open neck surgery, including the dissections of Ⅱ-Ⅳ regions in 11 patients, Ⅱ-Ⅲ regions in 8 patients, Ⅰ-Ⅳ regions in 2 patients, and Ⅰ-Ⅲ regions in 1 patient. The total number of dissected lymph nodes ranged from 7 to 69 (average 25.5), and the total number of metastatic lymph nodes ranged from 0 to 4 (average 1.4). The average total drainage volume was 145.8 ml. During the operation, one patient had pharyngocervical communication with the neck, which was sutured without postoperative pharyngeal fistula. After the operation, one patient developed a wound hematoma and improved after bedside drainage and hemostasis. The median follow-up time was 30 months. At the end of follow-up, one patient developed parapharyngeal lymph node recurrence, but no recurrence in the conventional neck dissection area. The 3-year neck control rate was 94.7% and the 3-year overall survival rate was 83.3%. The median satisfaction score for appearance was 10 points. Conclusion: Robot-assisted neck dissection via the retroauricular approach for head and neck squamous cell carcinoma can achieve good oncological and functional outcomes, with fewer postoperative complications and higher patient appearance satisfaction.

To investigate the diagnostic value of magnetic resonance imaging (MRI) for persistent prostate cancer after irreversible electroporation (IRE) therapy. This is a post hoc analysis from a multicentre randomised trial, in which men with localised low- to intermediate-risk prostate cancer were randomised to receive either focal or extended IRE ablation. All patients underwent repeat MRI scans at 6 and 12 months and transperineal template mapping biopsy (TMB) at 6 months post-IRE. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of MRI were calculated for infield and outfield lesions using 2 × 2 contingency tables with 95% confidence intervals (CIs) for clinically significant prostate cancer and any-grade prostate cancer. A total of 106 patients were recruited to this study, including 39 patients (37%) with clinically insignificant prostate cancer and 67 patients (63%) with clinically significant prostate cancer (International Society of Urological Pathology grade ≥2). Of these, 101 patients underwent repeat MRI scan and prostate biopsy at 6 months after IRE. The rate of clinically significant prostate cancer detected by TMB infield and outfield was 9.9% (10/101) and 9.9% (10/101), respectively. In the treated area, the sensitivity, specificity, PPV and NPV for MRI to detect clinically significant prostate cancer were 30% (95% CI 6.7%-65%), 91% (95% CI 82%-96%), 27% (95% CI 6.0%-61%) and 92% (95% CI 84%-97%), respectively. In the untreated area, the sensitivity, specificity, PPV and NPV of MRI to detect clinically significant prostate cancer were 20% (95% CI 2.5%-56%), 91% (95% CI 82%-96%), 20% (95% CI 2.5%-56%) and 91% (95% CI 82%-96%), respectively. Favourable specificity but poor sensitivity was achieved with use of MRI to detect persistent clinically significant prostate cancer after IRE treatment. Repeat TMB should not be deferred, regardless of MRI results.

In cardiovascular disease, the discovery of the proprotein convertase subtilisin/kexin type 9 (PCSK9) has undoubtedly opened a new chapter in regulating blood lipids. Since its first identification as a key regulator of low-density lipoprotein receptor (LDLR) degradation in 2003, the role of PCSK9 in cholesterol metabolism has been extensively studied. However, with further research, the pleiotropy of PCSK9 has gradually emerged, and its impact extends far beyond cholesterol metabolism in the liver. The purpose of this review is to systematically explore the pleiotropy of PCSK9, extending from its important role in lipid regulation to its extensive effects in extrahepatic tissues, and to reveal its potential role in cardiovascular health, nervous system function, and tumor biology. By integrating the latest research findings, this paper summarizes the complex mechanisms of action of PCSK9 in different biological processes and explores its potential and challenges as a therapeutic target.