Objective: To investigate the characteristics of human epidermal growth factor receptor 2 (HER2) protein expression and gene status in uterine serous carcinoma (USC) patients. Methods: A total of 36 formalin-fixed and paraffin-embedded USC tissue specimens obtained between 2021 and 2022 in Peking Union Medical College Hospital were collected. The expression of HER2 protein and the gene status were detected by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) respectively, and the results were interpreted according to the 2020 International Society of Gynecological Pathologists recommendations. Results: Among the 36 cases, 11, 8, 12 and 5 cases showed HER2 IHC scores of 0, 1+, 2+, and 3+, respectively. All IHC 3+cases were pure USC. Out of 25 samples with different level of HER2 expression (IHC 1+, IHC 2+and 3+), 16 (64.0%) tumors with heterogeneous stain, which mainly affects the diseases with IHC 2+ (10/12) and IHC 3+ (3/5) lesions. Ten pure USC cases (27.8%, 10/36), involving HER2 IHC 0, IHC 1+, IHC 2+and IHC 3+tumors, harbored HER2 gene amplification by FISH (1, 1, 3 and 5 cases, respectively). All amplified cases exhibited a HER2/CEP17 ratio≥2.0. In addition, the incidences of chromosome 17 (CEP17) polysomy and monosomy were 25.0% (9/36) and 19.4% (7/36), respectively. Conclusions: Most of USC tumors exhibit intratumoral heterogeneity in HER2 IHC stain. Both HER2 IHC positive (3+) and FISH positive occur exclusively in pure USC tumors. HER2 gene amplification can be observed in any HER2 IHC levels.

Dynamics of the immune cell signature associated with the development of immune-related adverse event: Results from longitudinal immune profiling.

The aim of this study was to explore the application of enhanced recovery after surgery (ERAS) in patients undergoing gynaecological surgery. This investigation included an analysis of the postoperative recovery curve and the factors that influenced the number of postoperative recovery days. This study also aimed to further investigate the impact of various factors on health economics. A retrospective cohort study was conducted in the Fourth Ward of the General Gynaecology Centre of the Peking Union Medical College Hospital. A total of 1000 patients who had undergone elective benign gynaecological surgery between July 2021 and December 2022 were included. Demographic, perioperative and other relevant data were collected, and a visual analogue scale (VAS) survey was conducted using the European Five-Dimensional Health Scale (EQ-5D). The factors that influenced the number of postoperative recovery days were analysed using a multivariate linear regression analysis. Additionally, patients who had undergone laparoscopic myomectomy, laparoscopic ovarian cystectomy, laparoscopic total hysterectomy, abdominal myomectomy and abdominal total hysterectomy were grouped based on whether their ERAS implementation rate was ≥70%. Our goal was to evaluate the health economic value of the diagnosis-related group (DRG) payments from multiple perspectives and provide actionable recommendations for health insurance bureaus, hospitals and patients from a multi-dimensional perspective. ERAS completion rates for measures such as avoiding preoperative sedation and early postoperative ambulation exceeded 95%, whereas rates for chewing gum and intraoperative temperature monitoring were <5%. The follow-up EQ-5D questionnaire VAS scores were as follows: a preoperative score of 79.48±13.75, score on the day of surgery of 74.18±15.43, score on the first postoperative day of 76.43±14.25, and score on the second postoperative day of 79.84±13.12. Patients returned to a healthy state with a median recovery time of 3 (range, 0-8) days. The postoperative recovery curve was then drawn based on the VAS scores. Additionally, a multivariate linear regression analysis revealed that the prevention of postoperative nausea and vomiting, correction of preoperative anaemia and malnutrition, ERAS completion rate and VAS score on the second postoperative day significantly influenced the number of postoperative recovery days (p<0.05). Patients with ERAS compliance rates of <70% incurred an average hospital cost increase of 8% compared with those with a compliance rate of ≥70%. Specifically, patients who underwent laparoscopic myomectomy and laparoscopic total hysterectomy experienced more significant and more obvious increases in hospitalisation costs of approximately 15% (p<0.001) and 8% (p=0.031), respectively.An analysis of the relationship between hospital costs and different ERAS measures, insurance types and disease types showed that seven measures could reduce hospital costs without negatively affecting the patient recovery speed, and five measures could slightly increase hospital costs. In addition, hospitalisation cost differences based on various insurance types and disease categories were statistically significant (p<0.05). The postoperative recovery speed was accelerated, the recovery time was shortened and the patient's quality of life was enhanced during gynaecological surgery due to the implementation of ERAS practices. Increasing the ERAS completion rate can significantly reduce patient average hospitalisation costs. Additionally, variations in medical insurance, disease categories and specific ERAS measures influenced these costs. Therefore, hospitals that are unable to fully implement all ERAS measures must prioritise those that promote recovery. In addition, hospitals should adopt flexible strategies to minimise costs, thereby achieving mutual benefits for patients and hospitals. These findings establish a foundation for the implementation of a simplified ERAS version. It has been observed through the perspective of DRG implementation in China that payment standards exceed the average hospitalisation costs associated with specific surgical procedures. This result suggests that DRG implementation can benefit both patients and hospitals. These study results will serve as a valuable reference for decision-making by health insurance bureaus, hospitals and patients.

FANS: A framework for automatic assessment of nutritional status based on free-text clinical notes.

The optimal target of mean arterial pressure (MAP) remains controversial in sepsis management. Critical closing pressure (Pcc), the arterial pressure at which blood flow ceases, is the key determinant of vascular waterfall phenomenon. Tissue perfusion pressure (TPP), the difference between MAP and Pcc, represents the driving pressure for arterial blood flow. This study evaluated the prognostic value of Pcc and TPP for improving risk stratification in sepsis. This retrospective cohort study included adult patients with sepsis in 18 hospitals between August 2013 to October 2022 from two independent data sets (the Study on the Epidemiology, Diagnosis and Treatment of Sepsis [SEPSIS-EDT] registry and the critical care database of Peking Union Medical College Hospital, Beijing, China). Pcc was estimated via linear regression of hourly MAP against product of heart rate and pulse pressure, while TPP was calculated as MAP minus Pcc. Patients were categorized into four groups based on the optimal thresholds for mean Pcc and TPP within 24 h of sepsis diagnosis: low TPP-low Pcc, low TPP-high Pcc, high TPP-low Pcc, and high TPP-high Pcc. Clinical outcomes included mortality rates and development of acute kidney injury within 2 and 7 days of sepsis diagnosis. External validation was performed using the Medical Information Mart for Intensive Care IV (MIMIC-IV) cohort. A total of 6,769 patients (mean age, 61 yr; 61.0% men) were included. Intensive care unit mortality was highest in the low TPP-low Pcc group and lowest in the high TPP-high Pcc group (35.1% vs . 20.1%; risk difference, 15.0%; 95% CI, 10.2 to 19.8%). Similar patterns were observed for other outcomes. After adjustment for MAP, increased Pcc with concomitant reduced TPP showed a significant U-shaped association with both mortality and acute kidney injury development ( P < 0.001). The findings were consistent in the MIMIC-IV cohort. While MAP remains central to sepsis management, Pcc and TPP provide complementary prognostic information. Incorporating these parameters into clinical assessment may improve risk stratification and optimize blood pressure management.

To investigate the staining and serological characteristics of AC-30 pattern. A total of 184 participants were recruited from patients who underwent routine antinuclear antibody testing between 2022 and 2023 at Peking Union Medical College Hospital. Cohort 1 (n=47) showed the AC-30 pattern on HEp-2 indirect immunofluorescence assay, and cohort 2 (n=137) showed AC-2 pattern as control. Anti-DFS70 antibody detection and DFS70 antigen adsorption assays were conducted. Pattern simulation assays were performed by combining serum samples exhibiting classic AC-2 pattern with other common HEp-2 IIFA patterns. Anti-DFS70 antibodies were detected in 97 % of patients in cohort one and in all patients in cohort 2. The titers of the IIFA pattern showed a weak correlation with anti-DFS70 antibody levels in cohort 1 (r=0.35, p=0.0331). In DFS70 antigen adsorption assays, a higher proportion of homogeneous nuclear staining was observed in cohort 1 (79 %) than in cohort 2(62 %) (p=0.037). Simulated samples mixed classic AC-2 with homogeneous pattern resembled those of AC-30 pattern in both interphase and mitotic cells. Especially, the staining characteristics of AC-2 became increasingly indistinct when mixed with higher-titer homogeneous patterns. Among samples exhibiting homogeneous patterns post-DFS70 immunoadsorption, non-autoimmune conditions were more common in cohort one than cohorttwo. The presence of relatively lower anti-DFS70 antibodies levels or coexisting high-titer homogeneous patterns may contribute to the development of the AC-30 pattern rather than AC-2. This finding needs to be further confirmed in larger-scale studies.

Abstract: OBJECTIVE: To investigate the clinical characteristics, management and outcomes of cerebral venous thrombosis (CVT) patients with antiphospholipid syndrome (APS) in a Chinese cohort. METHODS: A retrospective cohort study was conducted on 121 consecutive CVT patients admitted to Peking Union Medical College Hospital from 2015 to 2023. Data on demographics, clinical manifestations, imaging findings, laboratory tests, treatment, and outcomes at discharge were analyzed. RESULTS: APS was identified in 16.5% (20/121) of CVT patients. The median age at onset was 34.5 years, with 60% (12/20) being female. 75% of patients had not experienced other thrombotic and obstetric events before CVT. In APS-CVT cases, chronic onset was observed in 50% (10/20), with a median diagnostic delay of 20 days. Headache was the most common symptom. Additional risk factors were present in 85% of APS-CVT patients. All patients received anticoagulation therapy, and 80% achieved favorable outcomes (modified Rankin Scale: &lt; 2) at discharge. While most clinical and imaging features showed no significant differences, APS-CVT patients exhibited less frequent transverse/sigmoid sinus involvement (55% [APS-CVT] vs. 72.3% [non-APS-CVT], P = 0.045) and multisinus thrombosis (15% [APS-CVT] vs. 40.6% [non-APS-CVT], P = 0.020). Laboratory findings revealed lower hemoglobin levels (118 g/L [APS-CVT] vs. 130 g/L [non-APS-CVT], P &lt; 0.05) and a higher prevalence of concomitant extracranial venous thrombosis (45% [APS-CVT] vs. 11.9% [non-APS-CVT], P &lt; 0.001) in the APS-CVT group. CONCLUSION: APS represents a significant risk factor for CVT. CVT associated with APS primarily affects young individuals and women, often presents insidiously, and is frequently accompanied extracranial venous thrombosis. Given the challenges in diagnosing APS-CVT, screening for APS should be strongly considered in CVT patients with these clinical contexts. The characteristic pattern of venous sinus involvement in APS-CVT may provide insights into its unique pathogenesis. Most patients were treated with warfarin, which may be associated with a favorable outcome.

Objective: This study processes and analyzes rectal MRI images of patients with mid-to-low rectal cancer using deep learning technology, and integrates these data with clinical baseline information to construct a fully automated end-to-end prediction model. The model is designed to assist colorectal surgeons in preoperatively assessing surgical difficulty and selecting the optimal surgical approach. Methods: We prospectively collected data from patients with mid-to-low rectal cancer who underwent laparoscopic total mesorectal excision (TME) and had been graded according to the surgical difficulty system recorded in Division of Colorectal Surgery, Department of General Surgery, Peking Union Medical College Hospital, between March, 2019 and May, 2025. Inclusion criteria: (1) age 18-75 years; (2) tumor lower edge within 10 cm of the anal verge as measured by rectal MRI; (3) pathologically confirmed rectal adenocarcinoma; (4) complete, accessible preoperative rectal MRI DICOM images; and (5) tumor invasion depth of T1-4aNanyM0. Exclusion criteria: (1) synchronous or metachronous multiple primary colorectal cancer with concurrent surgery; (2) Any surgery other than TME; (3) tumor involvement of surrounding organs requiring combined organ resection; (4) unfitness for laparoscopic surgery (e.g. extensive adhesions from previous abdominal surgery, contraindications to pneumoperitoneum for various reasons, etc.); and (5) Robot-assisted radical resection of rectal cancer. Included patients were divided into training and test datasets, and deep learning techniques (rectal MRI image annotation, image preprocessing, data augmentation, and feature extraction) were used for model construction. Results: A total of 366 patients were included, with 253 males. The median BMI was 24.1 (22.0, 26.6) kg/m², and the median distance from the tumor lower edge to the anal verge was 6.5 (4.7, 7.8) cm. A total of 288 patients received neoadjuvant chemoradiotherapy. Based on intraoperative difficulty grade, patients were divided into the difficult group (199 cases) and the nondifficult group (167 cases). Compared to the nondifficult group, the difficult group showed several statistically significant differences (all P<0.05): higher proportion of males [86.9%(173/199) vs. 47.9%(80/167), χ²=64.813, P<0.001]; higher BMI [25.4 (23.2, 27.6) kg/m² vs. 23.1 (21.2, 25.2) kg/m², Z=-6.082, P<0.001]; and higher proportion of neoadjuvant chemoradiotherapy [88.9% (177/199) vs. 66.5%(111/167), χ²=27.357, P<0.001]. However, there was no statistically significant differences in the distance from the tumor lower edge to the anal verge between the two groups [6.4 (4.7, 7.9) cm vs. 6.6 (4.7, 7.7) cm, Z=-0.001, P=0.999]. Importantly, our surgical difficulty prediction model achieved an accuracy of 0.729, a precision of 0.684, a specificity of 0.521, a recall of 0.915, an F1-score of 0.782, and an AUC of 0.83. Conclusions: We proposed a prediction model with reasonable accuracy using artificial intelligence that can assist surgeons in determining surgical difficulty and choosing the optimal surgery approach for mid-low rectal cancer.

Objective: To compare the differences in cardiac magnetic resonance (CMR) characteristics between dilated cardiomyopathy (DCM) patients accompanied by left ventricular hypertrabeculation and those with isolated DCM, and to investigate the impact of left ventricular hypertrabeculation on the prognosis of DCM patients. Methods: This single-center retrospective cohort study enrolled DCM patients who were admitted to Peking Union Medical College Hospital from November 2016 to March 2025. Basic clinical data and CMR parameters of the patients were collected. According to the results of echocardiography, the patients were divided into the isolated DCM group and the DCM with left ventricular hypertrabeculation group. Patients were followed up through outpatient visits or telephone calls. The composite endpoint consisting of cardiovascular death, heart failure hospitalization, and malignant arrhythmia events (ventricular fibrillation, sustained ventricular tachycardia) was used as the outcome event. Univariate and multivariate Cox proportional hazards regression models were used to analyze the risk factors affecting the prognosis of DCM patients. Survival analysis was performed using Kaplan-Meier curves, and the log-rank test was used to compare the differences in the incidence of outcome events between groups. Subgroup analysis was conducted according to the range of left ventricular late gadolinium enhancement (LGE) (≥7.5% and <7.5%). Results: A total of 114 DCM patients were enrolled, including 65 in the isolated DCM group and 49 in the DCM with left ventricular hypertrabeculation group. In the isolated DCM group, the patients had an age of (44±16) years, with 44 males (68%); in the DCM with left ventricular hypertrabeculation group, the patients had an age of (40±17) years, with 32 males (65%). Regarding echocardiographic parameters, there were no statistically significant differences in left ventricular ejection fraction ((34±8)% vs. (32±11)%) and left ventricular end-diastolic diameter ((65±8) mm vs. (66±8) mm) between the isolated DCM group and the DCM with left ventricular hypertrabeculation group (both P>0.05). In terms of CMR characteristics, the left ventricular end-diastolic volume (126 (105, 158) ml vs. 146 (114, 185) ml), left ventricular global longitudinal strain ((-8.15±3.17)% vs. (-8.56±3.76)%), and left ventricular LGE extent (11.4 (4.3, 26.6)% vs. 12.3 (5.4, 20.2)%) in the isolated DCM group were lower than those in the DCM with left ventricular hypertrabeculation group, while the left ventricular ejection fraction was higher than that in the DCM with left ventricular hypertrabeculation group (29 (23, 35)% vs. 27 (20, 39)%). However, there were no statistically significant differences in the above CMR parameters between the two groups (all P>0.05). For prognosis, during a follow-up of 368 (146, 652) days, 25 patients (22%) experienced outcome events, including 4 cases (4%) of cardiovascular death, 22 cases (19%) of heart failure hospitalization, and 1 case (1%) of malignant arrhythmia. Cox regression analysis showed that left ventricular hypertrabeculation was not associated with the occurrence of outcome events in DCM patients (HR=0.682, 95%CI 0.301-1.540, P=0.358), while left ventricular LGE extent was associated with the prognosis of DCM patients (HR=6.589, 95%CI 1.064-40.794, P=0.043). Kaplan-Meier curves showed that there was no statistically significant difference in the cumulative incidence of outcome events between the isolated DCM group and the DCM with left ventricular hypertrabeculation group (log-rank P=0.355); however, DCM patients with left ventricular LGE extent ≥7.5% had a higher risk of adverse prognosis than those with left ventricular LGE extent <7.5% (log-rank P=0.032). Conclusion: There are no significant differences in CMR characteristics between DCM patients with left ventricular hypertrabeculation and those with isolated DCM, and left ventricular hypertrabeculation had no impact on the prognosis of DCM patients. In contrast, left ventricular LGE extent was a risk factor affecting the prognosis of DCM patients.

Studies verifying the performance of antifibrotic drugs for the treatment of ANCA positive fibrotic ILD (ANCA-fILD) are lacking. This study assessed the clinical features of ANCA-fILD patients with or without add-on treatment with antifibrotic drugs. A retrospective study involving ANCA-positive patients treated from January 2012 to December 2023 at Peking Union Medical College Hospital was conducted, and a case‒control analysis was performed accordingly. A total of 105 ANCA-fILD patients were identified from 18,617 ANCA-positive patients treated from January 2012 to December 2023 at Peking Union Medical College Hospital; these patients were further divided into an add-on group (31 patients) and a non-add-on group (74 patients). On the basis of baseline FVC%pred matching (± 10%), 30 and 60 patients were ultimately included in the add-on and non-add-on groups, respectively. The decreases in pFVC (p = 0.03), FVC (p = 0.02), DLCO (p = 0.01), and pDLCO (p < 0.01) were less significant in the add-on group than in the non-add-on group. In the related sample analysis, the add-on group did not present significant differences in FVC (p = 0.47), pFVC (p = 0.53), DLCO (p = 0.90), and pDLCO (p = 0.43) between baseline and at the end of the 1-year follow-up, whereas the non-add-on group presented significant decreases in FVC (p < 0.01), pFVC (p < 0.01), DLCO (p < 0.0001) and pDLCO (p < 0.0001). The incidence rates of adverse events and acute exacerbations were similar between the two groups. Add-on antifibrotic medications seem to effectively slow the deterioration of lung function in patients with ANCA+ fILD without causing any obvious new-onset adverse reactions.

BackgroudUreteritis and cystitis is a rare immune-related adverse event (irAE) of immune checkpoint inhibitors (ICIs), challenging to distinguish from urinary tract infection (UTI), easily leading to missed diagnosis. We aim to describe clinical features, radiological characteristics and treatment of patients who suffer from ICI-related ureteritis and cystitis (ICI-UC).MethodsThis was a single centre case series of patients diagnosed with solid tumor who received ICIs treatment and subsequently suffered from ICI-UC. All clinical demographic data, laboratory parameters, imaging characteristics, and treatment information were collected.ResultsBetween Mar 1st, 2020 and Mar 31th 2025, 12 of 1239 patients treated at Peking Union Medical College Hospital with ICIs were confirmed to have ICI-related ureteritis and cystitis (0.96%), 10 males and 2 females. Only 1 patient received anti-programmed cell death protein 1 (PD-1) and cytotoxic T-lymphocyte associated protein-4 (CTLA-4) dual immunotherapy, the other 11 patients received PD-1/PD-ligand 1 plus chemotherapy or/and target therapy. The median time to onset was 83 days (range 28–442 days). All patients (100%) exhibited significant urinary tract irritation symptoms. 12 patients demonstrated characteristic imaging abnormalities, including hydroureteronephrosis, irregular ureteral wall thickening, bladder wall thickening with irregular margins, or/and conspicuous renal fascia. Spontaneous remission was observed in 2 patients, while 10 patients received steroids and all showed rapid improvement of symptom after treatment.The median time from symptom onset to the initiation of steroids was 24 days, and the median prednisone dose was 0.60 mg/kg/day. Six patients (6/10, 60%) experienced disease recurrence during the corticosteroid tapering phase, and two patients who failed steroid tapering were successfully treated with a combination of corticosteroid and JAK inhibitor therapy.ConclusionThis pioneering cohort study provides the first systematic investigation of ICI-UC, establishing its incidence and comprehensively characterizing clinical and imaging features. Through cohort analysis, we propose a novel severity grading system with corresponding treatment algorithms, while additionally exploring the therapeutic potential of JAK inhibitors for steroid-dependent cases.

Background/Objectives: Anti-mGluR1 encephalitis is a rare form of autoimmune encephalitis predominantly manifesting as acute/subacute cerebellar ataxia. We describe a newly diagnosed case series from our center and conduct a comprehensive review of reported cases worldwide to compare clinical manifestations, treatment options, and outcomes. Methods: We consecutively identified 11 patients at Peking Union Medical College Hospital, and additionally extracted clinical data from 42 previously published cases identified via PubMed and Google Scholar (search updated to 1 August 2025). Demographics, phenotypes, laboratory findings, imaging, treatment, and outcomes were systematically summarized. This pooled review was not prospectively registered, and extracted data from 21 published articles were analyzed alongside our 11 newly diagnosed cases. Results: The integrated cohort comprised 53 patients with anti-mGluR1 encephalitis, including 29 males and 24 females, with patients reported from Asia (n = 18), North America (n = 11), and Europe (n = 24). The median age at onset was 50 years (IQR 29.5–58.5; range 3–81), with North American patients presenting later than their Asian and European counterparts (median 60 vs. 48 and 45 years, respectively; all p < 0.05). Disease onset was subacute in most cases (58.7%). Comorbid tumors were present in nine patients, most commonly lymphomas. Clinical phenotypes were classified as pure cerebellar syndrome (n = 31), cerebellar ataxia with encephalitic features (n = 20), and non-cerebellar presentations (n = 2). Baseline severity differed across phenotypes (χ2 = 35.7, p < 0.001). Regional variability in severity was observed but did not reach significance. CSF analyses revealed pleocytosis in 59% (23/39), elevated protein in 31.3% (5/16), and oligoclonal bands in 52.2% (12/23). MRI abnormalities were detected in 34.7% (17/49) of patients, with 21.9% (7/32) developing cerebellar atrophy on follow-up. Therapeutic strategies varied significantly across regions (p = 0.041), with Asian cohorts more frequently receiving long-term immunosuppression, European cohorts favoring combined regimens, and North American cases relying predominantly on first-line therapies. Overall, 65.9% (29/44) of patients clinically improved, 13.6% (6/44) relapsed and 20.5% (9/44) remained unaffected. Conclusions: Anti-mGluR1 encephalitis presents with significant clinical heterogeneity, ranging from cerebellar-dominant ataxia to neuropsychiatric or non-cerebellar phenotypes, and demonstrates differences in reported age of onset, disease severity, and therapeutic approaches across publication regions. Our findings underscore the importance of early recognition, sustained immunotherapy, and international collaboration to establish standardized, evidence-based management for this rare but disabling disorder.

Effective preoperative patient counseling is essential to shared decision-making. In thyroid surgery, patient communication can be complicated by the complex anatomy and variable surgical approaches, which may not be fully conveyed through conventional verbal explanations or schematic drawings. Virtual three-dimensional (3D) thyroid models may provide an intuitive tool to enhance patient comprehension. We conducted a randomized controlled trial at Peking Union Medical College Hospital with 94 newly-diagnosed thyroid cancer patients scheduled for thyroidectomy. Participants were assigned to either the control group (n = 47), which received preoperative drawing-based counseling, or the intervention group (n = 47), which utilized a virtual 3D model for communication. The Thyroid Navigator app, developed by Kuma Hospital, was used to provide dynamic 3D representation of the thyroid gland, surrounding structures, and potential surgical procedures. After standardized preoperative consultations, patients were surveyed to assess their understanding in pertinent anatomy and postoperative complications. Patients in the 3D model group demonstrated similar correct response rates in lesion localization (p = 0.536) or parathyroid gland recognition (p = 0.071), but significantly higher accuracy in identifying the recurrent laryngeal nerve and the extent of lymph node dissection compared with the control group (p < 0.05). Moreover, comprehension of the causes of major postoperative complications-including hoarseness (recurrent laryngeal nerve injury, p = 0.004), hypocalcemia (parathyroid gland impairment, p = 0.015), and bleeding (inadequate hemostasis, p = 0.008)-was significantly improved in the 3D model group. Use of a virtual 3D thyroid model significantly improves patient comprehension of thyroid anatomy, surgical procedures, and potential complications, thereby enhancing clinician-patient communication. Virtual 3D models represent a practical and cost-effective supplement to conventional counseling in thyroid surgery, offering clear benefits in patient education and shared decision-making.

Objective: To determine whether sirolimus therapy at lower trough concentrations is non-inferior to the conventional target concentration (≥5 μg/L) with respect to pulmonary protective effects and lung function preservation in patients with lymphangioleiomyomatosis (LAM). Methods: A retrospective analytical dataset was constructed using patient-level data from the Peking Union Medical College Hospital LAM cohort registry between April 2017 and April 2022. Patients were stratified into a conventional-concentration group (≥5 μg/L) and a low-concentration group (<5 μg/L) according to the time-weighted average sirolimus trough concentration. The conventional concentration group comprised 103 LAM patients (mean age 40.1 years), and the low concentration group included 80 patients (mean age 38.8 years). All patients were female. Changes in FEV1 were analyzed with a linear mixed-effects model, and marginal mean FEV1 changes from baseline were estimated at prespecified time points after treatment initiation. The primary endpoint was the change in FEV1 after 1 year of sirolimus therapy. If no statistically significant difference was observed at one year, the change at three years was compared. The estimated marginal means of FEV1 change between groups at the same time point were compared using Welch's t-test or Welch's analysis of variance (ANOVA). Results: After one year, the estimated marginal mean FEV1 increase was 45.2 ml (95%CI: 13.9-76.5) in the low-concentration group versus 33.7 (95%CI: 6.6-60.8) ml in the conventional-concentration group (t=0.55, P=0.585). At three years, the between-group difference in the estimated marginal mean change in FEV1 from baseline remained statistically non-significant (low-concentration group:-24.9 ml, 95%CI:-115.7-65.9 ml; conventional-concentration group:-4.1 ml, 95%CI:-78.9-70.8 ml; t=0.35, P=0.728). Conclusions: Maintaining sirolimus at lower trough concentrations (median 4.1 μg/L, 3.5-4.5 μg/L) provides similar FEV₁ preservation to that achieved with the conventional target concentration. This suggested that the therapeutic target for sirolimus in LAM could be safely reduced.

Interferon-γ (IFNγ), a double-edge sword that affects prognoses in critically ill patients: A prospective study.

Objective: To summarize the clinical and genetic characteristics of Chinese patients with Birt-Hogg-Dubé syndrome (BHD). Methods: We retrospectively analyzed the clinical data of patients diagnosed with BHD between January 1, 2015, and April 30, 2025, at Peking Union Medical College Hospital, Chinese Academy of Medical Science (Beijing center); the First Affiliated Hospital of USTC, University of Science and Technology of China (Hefei center); and the First Affiliated Hospital of Guangzhou Medical University (Guangzhou center). Epidemiological characteristics, clinical manifestations, genetic variants were summarized, and the correlation between genetic mutations and phenotypic presentations were explored. Statistical analyses were performed using SAS 9.4 software. Results: A total of 168 BHD families comprising 256 patients (76 from Beijing, 141 from Hefei, and 39 from Guangzhou) were enrolled, including 201 (78.5%) probands and 55 (21.5%) family screening participants. The cohort included 188 females, with a male-to-female ratio of 1∶2.8. The mean age at diagnosis was 18-81 (44.1±12.6) years, with a median misdiagnosis duration of 12 (0, 72) months. Among 256 patients, family history was present in 195 patients (76.2%), including 71 (36.4%) pulmonary cysts with pneumothorax, 60 (30.8%) pulmonary cysts alone, 53 (27.2%) pneumothorax alone, and 11 (5.6%) renal tumors. Pulmonary cysts were present in 254 patients (99.2%), most commonly presenting as extensive cysts (≥10 cysts, 68.5%, 174/254) followed by moderate cysts (5-9 cysts, 24%, 61/254). Cysts were bilateral in 248 patients (97.6%), while only 6 (2.4%) had unilateral involvement. Lower lobe predominance was noted in 237 patients (93.3%), and paramediastinal distribution in 158 (62.2%). Pneumothorax occurred in 170 patients (66.4%), with 113 (66.5%) experiencing unilateral events and 57 (33.5%) bilateral events. The mean age at first pneumothorax was (37.4±11.6) years, with an average of 2.4 episodes per patient. Cutaneous manifestations were noted in 55.9% (143/256), but only 15.4% (22/143) underwent biopsy, confirming fibrofolliculomas (9 cases) and trichodiscomas (2 cases). Significant regional differences in skin lesion prevalence were observed (Beijing 48.7% (37/76) vs. Hefei 72.3% (102/141) vs. Guangzhou 10.3% (4/39), χ2=50.01, P<0.001). Renal imaging was performed in 232 patients, revealing renal tumors in 10 patients (4.3%), including 6 cases of renal cell carcinoma and 4 cases of angiomyolipoma. Ninety-two distinct genetic variants were identified, including 38 novel mutations. The most common variants in Chinese patients were exon 11 c.1285dupC (13.5%, 27/200), exon 11 c.1285delC (7.5%, 15/200), and exon 14 c.1579_1580insA (12/200, 6.0%). Variants associated with higher pneumothorax rates included exon 9 c.929_930insTT (3/3), large deletions in exons 1-3 (8/9) and exon 6 c.469_471delTTC (4/5). Cutaneous manifestations were more common with exon 11 flanking sequence c.1177-5_1177-3delCTC (5/6), exon 6 c.469_471del TTC (4/5), and exon 9 c.1015C>T (11/14). Conclusions: Chinese patients with BHD predominantly present with pulmonary cysts and pneumothorax, with a relatively low incidence of renal tumors. Although the detection rate of skin lesions is higher than previously reported, significant regional variations persist. Multidisciplinary collaboration is recommended in medical centers to improve diagnostic accuracy. Furthermore, Chinese patients exhibit a distinct genetic variant profile, with five variants potentially associated with pneumothorax and skin lesion phenotypes; however, further studies are required to validate these findings.

Objective This study aimed to investigate trends of metabolic syndrome (MetS) and its components across the menopausal transition and to identify key metabolic risk factors in Chinese midlife women. Method The longitudinal study included 841 women from the Peking Union Medical College Hospital Aging Longitudinal Cohort of Women in Midlife (PALM cohort, 2005–2024), classified by the Stages of Reproductive Aging Workshop +10 (STRAW +10) criteria. Repeated measures of metabolic indicators, sex hormones and sociodemographic factors were analyzed using generalized estimating equations. Results MetS prevalence rose from 15.0% in the reproductive stage to 43.9% in late postmenopause. All components except reduced high-density lipoprotein cholesterol (HDL-C) increased progressively. After adjustment, perimenopause and postmenopause stages showed higher MetS odds than the reproductive stage (p = 0.008), with the highest observed in the early postmenopause stage (odds ratio = 1.796, 95% confidence interval = 1.224–2.634), primarily driven by central obesity and elevated triglycerides. Age, follicle stimulating hormone (FSH) and estradiol (E2) were independently associated with MetS risk. Lower education and poor self-rated health correlated with higher MetS risk. Vasomotor symptoms were significantly associated with central obesity (p = 0.012) and elevated triglycerides (p < 0.001). Conclusion The menopausal transition is a critical period for metabolic deterioration. Central obesity and elevated triglycerides are key markers. Early identification and targeted intervention are essential during this transition.

To explore the risk of relapse after glucocorticoids (GCs) withdrawal in patients with stable Sjögren's disease-associated interstitial lung disease (SjD-ILD). This was a single-center, retrospective cohort study conducted at Peking Union Medical College Hospital between February 2016 and January 2024. The study compared two treatment strategies for patients with stable SjD-ILD: GC withdrawal versus GC maintenance. The primary endpoint was to compare the relapse rates between the two groups over a 3-year follow-up period. The secondary endpoints included changes in pulmonary function, serological markers, and adverse events. A total of 134 patients were included in this study, with 61 in the GC withdrawal group and 73 in the GC maintenance group. Over the 3-year follow-up, 16 patients (27.93%) in the withdrawal group and 19 patients (28.77%) in the maintenance group experienced a relapse. No significant difference was found in relapse rates between the two groups (HR = 1.011, 95% CI 0.517-1.977, p = 0.974). Changes in pulmonary function, ESR, hs-CRP, C3, and C4 levels showed no significant differences, while IgG changes differed at the 2- and 3-year follow-up (p < 0.05). Adverse event rates were similar between groups. GCs' withdrawal in patients with stable SjD-ILD did not increase the relapse risk compared to continuing low-dose GCs' therapy. These findings suggest that cautious GCs' withdrawal might be a viable option for patients with stable, clinically inactive SjD-ILD. Key Points • Glucocorticoid withdrawal in stable pSS-ILD did not increase relapse risk compared to continuing low-dose GC therapy.

To determine a direct method for diagnosing axillary lymph node (ALN) tumor burden preoperatively in cT1-2N0 breast cancer patients, we developed and validated a deep learning (DL) model based on ultrasound (US) images of sentinel lymph nodes (SLNs) detected by contrast-enhanced lymphatic ultrasound (CEUS). Women with cT1-T2N0 breast cancer who received CEUS were enrolled prospectively from Peking Union Medical College Hospital between April 2020 and July 2021 and from Sichuan Cancer Hospital between April 2022 and July 2022. Heavy ALN tumor burden was defined as > 2 metastatic lymph nodes according to the Z0011 criteria. We developed a DL model, the modality-adaptive network with clinicopathological information (MAN + C), using grayscale or color Doppler US images and radioclinicopathological information to predict heavy tumor burden. A total of 595 SLNs from 374 patients met the inclusion criteria. The areas under the receiver operating characteristic curve (AUCs) were calculated to evaluate the predictive performance of the model, yielding values of 0.91[95%CI(confidence interval):0.899–0.943], 0.98[95%CI:0.950-1], 0.89[95%CI:0.850–0.935], and 0.84[95%CI:0.811–0.869] in the training, validation, independent and external test datasets, respectively. Compared to current artificial intelligence (AI) models, this model extends usability by 30%, encompassing patients with multifocal lesions or those who have undergone primary breast cancer lesion therapy. Using this model, 88.9% of patients could safely decide whether they could waive unnecessary SLN biopsy. MAN + C provided a direct and efficient method for accurate preoperative assessment of ALN tumor burden in cT1-2N0 breast cancer patients.Supplementary InformationThe online version contains supplementary material available at 10.1038/s41598-025-34371-y.

P0274 The efficacy and tolerability of ultra-low dosage polyethylene glycol based modified bowel preparation in patients with Uulcerative Colitis: A multicenter randomized controlled trial