1.Clinical Characteristics and Prognostic Analysis of Extracranial Malignant Rhabdoid Tumor in Children
Shihan ZHANG ; Wen ZHAO ; Mei JIN ; Hongjun FAN ; Xisi WANG ; Libing FU ; Tong YU ; Yan SU
JOURNAL OF RARE DISEASES 2026;5(1):34-42
To investigate the clinical characteristics and prognosis of extracranial malignant rhabdoid tumor (eMRT) in children, and to provide a reference for the clinical treatment of this disease. A retrospective analysis was performed on the clinical data of children with newly diagnosed eMRT who were admitted and treated in the Department of Pediatric Oncology, Beijing Children's Hospital Affiliated to Capital Medical University, from March 2009 to December 2024. The clinical characteristics were summarized, and survival analysis and prognostic risk factor analysis were conducted. A total of 43 children with eMRT were included in this study, the median age at diagnosis of all patients was 20 months (range: 2-138 months). Among them, 24 cases were malignant renal rhabdoid tumors and 19 cases were extracranial, extrarenal rhabdoid tumors. Of the 43 children, 23 cases (53.5%) were complicated with distant metastasis. Twenty-nine (67.4%) underwent primary tumor resection. Among the children, 24 (55.8%) underwent gross total resection (GTR), 5 (11.6%) partial resection, and 14 (32.6%) biopsy only. Their 3-year overall survival (OS) rates were 40.8%, 35.3%, and 33.3%, respectively ( Children with eMRT have an overall poor prognosis. A diagnostic age < 12 months is an independent risk factor for higher mortality in these children. Further large-scale, long-term follow-up studies are needed to explore the prognostic factors of this disease.
2.Effect of Wulao Qisun Prescription on Proliferation and Osteogenic Differentiation of AS Fibroblasts by Regulating Wnt/β-catenin Signaling Pathway
Juanjuan YANG ; Ping CHEN ; Haidong WANG ; Zhendong WANG ; Haolin LI ; Zhimin ZHANG ; Yuping YANG ; Weigang CHENG ; Jin SU ; Jingjing SONG ; Dongsheng LU
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(2):67-73
ObjectiveTo investigate the effect and underlying mechanism of the Wulao Qisun prescription on pathological new bone formation in ankylosing spondylitis (AS). MethodsSynovial fibroblasts were isolated from the hip joints of AS patients and observed under a microscope to assess cell morphology. The cells were identified using immunofluorescence staining. The isolated AS fibroblasts were divided into blank group, low drug-containing serum group, medium drug-containing serum group, high drug-containing serum group, and positive drug group. After drug intervention, cell proliferation was measured using the cell counting kit-8 (CCK-8) assay to observe fibroblast growth and determine the optimal intervention time. Alkaline phosphatase (ALP) activity was measured using the alkaline phosphatase assay. Protein expression of osteocalcin (OCN), osteopontin (OPN), and runt-related transcription factor 2 (Runx2) was detected by Western blot. The mRNA expression levels of Wnt5a, β-catenin, and Dickkopf-1 (DKK-1) were measured by real-time quantitative polymerase chain reaction (Real-time PCR). ResultsCompared with the blank group, each drug-containing serum group of Wulao Qisun prescription and the positive drug group inhibited the proliferation of AS fibroblasts and reduced ALP expression (P<0.01). Compared with the blank group, the low drug-containing serum group of Wulao Qisun prescription downregulated β-catenin mRNA expression (P<0.05). The medium and high drug-containing serum groups and the positive drug group significantly downregulated Wnt5a and β-catenin mRNA expression (P<0.05, P<0.01), with the positive drug group showing the most pronounced effect (P<0.01). The high drug-containing serum group and the positive drug group significantly upregulated DKK-1 mRNA expression (P<0.01). Compared with the blank group, the low drug-containing serum group of Wulao Qisun prescription inhibited the expression of OPN and Runx2 proteins (P<0.05, P<0.01), while the medium and high drug-containing serum groups and the positive drug group inhibited the expression of OCN, OPN, and Runx2 proteins (P<0.05, P<0.01). ConclusionThe Wulao Qisun prescription can inhibit the proliferation and osteogenic differentiation of AS fibroblasts, thereby delaying the formation of pathological new bone in AS. The possible mechanism involves the regulation of Wnt/β-catenin-related gene expression, further inhibiting the transcription of downstream target genes.
3.Research progress on the mechanism of action of rosmarinic acid in the prevention of cardiovascular diseases
Ke CAI ; Sheng-ru HUANG ; Fang-fang GAO ; Xiu-juan PENG ; Sheng GUO ; Feng LIU ; Jin-ao DUAN ; Shu-lan SU
Acta Pharmaceutica Sinica 2025;60(1):12-21
With the rapid development of social economy and the continuous improvement of human living standard, the incidence, fatality and recurrence rates of cardiovascular disease (CVD) are increasing year by year, which seriously affects people's life and health. Conventional therapeutic drugs have limited improvement on the disability rate, so the search for new therapeutic drugs and action targets has become one of the hotspots of current research. In recent years, the therapeutic role of the natural compound rosmarinic acid (RA) in CVD has attracted much attention, which is capable of preventing CVD by modulating multiple signalling pathways and exerting physiological activities such as antioxidant, anti-apoptotic, anti-inflammatory, anti-platelet aggregation, as well as anti-coagulation and endothelial function protection. In this paper, the role of RA in the prevention of CVD is systematically sorted out, and its mechanism of action is summarised and analysed, with a view to providing a scientific basis and important support for the in-depth exploration of the prevention value of RA in CVD and its further development as a prevention drug.
4.Mid-long term follow-up reports on head and neck rhabdomyosarcoma in children
Chao DUAN ; Sidou HE ; Shengcai WANG ; Mei JIN ; Wen ZHAO ; Xisi WANG ; Zhikai LIU ; Tong YU ; Lejian HE ; Xiaoman WANG ; Chunying CUI ; Xin NI ; Yan SU
Chinese Journal of Pediatrics 2025;63(1):62-69
Objective:To analyze the clinical characteristics of children with head and neck rhabdomyosarcoma (RMS) and to summarize the mid-long term efficacy of Beijing Children′s Hospital Rhabdomyosarcoma 2006 (BCH-RMS-2006) regimen and China Children′s Cancer Group Rhabdomyosarcoma 2016 (CCCG-RMS-2016) regimen.Methods:A retrospective cohort study. Clinical data of 137 children with newly diagnosed head and neck RMS at Beijing Children′s Hospital, Capital Medical University from March 2013 to December 2021 were collected. Clinical characteristic of patients at disease onset and the therapeutic effects of patients treated with the BCH-RMS-2006 and CCCG-RMS-2016 regimens were compared. The treatments and outcomes of patients with recurrence were also summarized. Survival analysis was performed by Kaplan-Meier method, and Log-Rank test was used for comparison of survival rates between groups.Results:Among 137 patients, there were 80 males (58.4%) and 57 females (41.6%), the age of disease onset was 59 (34, 97) months. The primary site in the orbital, non-orbital non-parameningeal, and parameningeal area were 10 (7.3%), 47 (34.3%), and 80 (58.4%), respectively. Of all patients, 32 cases (23.4%) were treated with the BCH-RMS-2006 regimen and 105 (76.6%) cases were treated with the CCCG-RMS-2016 regimen. The follow-up time for the whole patients was 46 (20, 72) months, and the 5-year progression free survival (PFS) and overall survival (OS) rates for the whole children were (60.4±4.4)% and (69.3±4.0)%, respectively. The 5-year OS rate was higher in the CCCG-RMS-2016 group than in BCH-RMS-2006 group ((73.0±4.5)% vs. (56.6±4.4)%, χ2=4.57, P=0.029). For the parameningeal group, the 5-year OS rate was higher in the CCCG-RMS-2016 group (61 cases) than in BCH-RMS-2006 group (19 cases) ((57.3±7.6)% vs. (32.7±11.8)%, χ2=4.64, P=0.031). For the group with meningeal invasion risk factors, the 5-year OS rate was higher in the CCCG-RMS-2016 group (54 cases) than in BCH-RMS-2006 group (15 cases) ((57.7±7.7)% vs. (30.0±12.3)%, χ2=4.76, P=0.029). Among the 10 cases of orbital RMS, there was no recurrence. In the non-orbital non-parameningeal RMS group (47 cases), there were 13 (27.6%) recurrences, after re-treatment, 7 cases survived. In the parameningeal RMS group (80 cases), there were 40 (50.0%) recurrences, with only 7 cases surviving after re-treatment. Conclusions:The overall prognosis for patients with orbital and non-orbital non-parameningeal RMS is good. However, children with parameningeal RMS have a high recurrence rate, and the effectiveness of re-treatment after recurrence is poor. Compared with the BCH-RMS-2006 regimen, the CCCG-RMS-2016 regimen can improve the treatment efficacy of RMS in the meningeal region.
5.Ectopic Mediastinal Thyroid Successfully Removed by Surgical Excision via Transcervical Approach
Seung Yup SON ; Se Hyeon JIN ; Jong Hwan LEE ; Su Il KIM
Korean Journal of Otolaryngology - Head and Neck Surgery 2025;68(4):168-171
Ectopic thyroid is thyroid tissue found in places other than the anterolateral aspect of the second to fourth tracheal ring. Mediastinal ectopic thyroid is rare and only few cases have been reported. The authors experienced a case of 41-year-old female patient with an anterior neck mass. The patient had mild chest discomfort when breathing with no other symptoms. Imaging studies suggested tumor of thymic tissue origin and surgical excision was done. The mass was successfully removed and histopathologically determined to be thyroid tissue. We hereby report with a review of literature a case of ectopic thyroid found in the mediastinum, which was successfully removed by transcervical incision.
6.A Case of Multiple Cranial Neuropathies Caused by Anaplastic Lymphoma Kinase-Negative Anaplastic Large Cell Lymphoma
Hyeop OH ; Su Mi SEONG ; Eo Jin KIM ; Bo Hae KIM
Korean Journal of Otolaryngology - Head and Neck Surgery 2025;68(3):121-126
Multiple cranial neuropathies (MCN) can be caused by various etiologies, such as autoimmune diseases, neurovascular diseases, tumors, or infections. Among the various etiologies of MCN, malignant lymphoma is a major cause. Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma (ALK-ALCL) is an extremely rare subtype of T-cell lymphoma that exhibits aggressive behavior, particularly when affecting the central nervous system (CNS). The rarity of ALK-ALCL often leads to a relative delay in diagnosis compared to other types of lymphoma. We experienced a patient with MCN, for whom malignant lymphoma was suspected and diagnosed with ALK-ALCL, which simultaneously involved multiple cranial nerves, bilateral submandibular glands (SMGs), and the stomach within a relatively short time. Herein, we report our diagnostic experience of ALK-ALCL, along with a literature review.
7.Early effects of PCSK9 inhibitors: evolocumab versus alirocumab
Su-Hyun BAE ; Bong-Joon KIM ; Soo-Jin KIM ; Sung-Il IM ; Hyun-Su KIM ; Jung-Ho HEO
Kosin Medical Journal 2025;40(1):49-54
Background:
The significance of risk modification in patients with acute coronary syndrome (ACS) is well recognized; however, the optimal timing for adminstering PCSK9 inhibitors remains unclear. Additionally, the lipid-lowering efficacy of evolocumab and alirocumab has not been fully established. This study evaluated the lipid-lowering effects of these two PCSK9 inhibitors.
Methods:
Patients diagnosed with ACS, including unstable angina, ST-segment elevation myocardial infarction, and non-ST-segment elevation myocardial infarction, who were treated with a PCSK9 inhibitor (evolocumab or alirocumab) during hospitalization for ACS between 2021 and 2023 were retrospectively analyzed. Baseline low-density lipoprotein cholesterol (LDL-C) levels were assessed, and changes in LDL-C levels during the acute and subacute phases after PCSK9 inhibitor administration were compared between the evolocumab and alirocumab groups.
Results:
Among 80 patients diagnosed with ACS, 36 received evolocumab, while 44 were treated with alirocumab. The mean baseline LDL-C level was 123 mg/dL in the evolocumab group and 128 mg/dL in the alirocumab group (p=0.456). In the subacute phase, the mean follow-up LDL-C levels were 47.05 mg/dL in the evolocumab group and 49.5 mg/dL in the alirocumab group (p=0.585). The mean percentage reduction in LDL-C levels during the subacute phase was 60.41% in the evolocumab group and 58.51% in the alirocumab group (p=0.431). These differences were not statistically significant.
Conclusions
No significant differences were observed between evolocumab and alirocumab. LDL-C levels exhibited a similar trend, characterized by a rapid decline in the acute phase, followed by a slight rebound in the subacute phase.
8.Observer-Blind Randomized Control Trial for the Effectiveness of Intensive Case Management in Seoul: Clinical and Quality-of-Life Outcomes for Severe Mental Illness
Hye-Young MIN ; Seung-Hee AHN ; Jeung Suk LIM ; Hwa Yeon SEO ; Sung Joon CHO ; Seung Yeon LEE ; Dohhee KIM ; Kihoon YOU ; Hyun Seo CHOI ; Su-Jin YANG ; Jee Eun PARK ; Bong Jin HAHM ; Hae Woo LEE ; Jee Hoon SOHN
Psychiatry Investigation 2025;22(5):513-521
Objective:
In South Korea, there is a significant gap in systematic, evidence-based research on intensive case management (ICM) for individuals with severe mental illness (SMI). This study aims to evaluate the effectiveness of ICM through a randomized controlled trial (RCT) comparing ICM with standard case management (non-ICM).
Methods:
An RCT was conducted to assess the effectiveness of Seoul-intensive case management (S-ICM) vs. non-ICM in individuals with SMI in Seoul. A total of 78 participants were randomly assigned to either the S-ICM group (n=41) or the control group (n=37). Various clinical assessments, including the Brief Psychiatric Rating Scale (BPRS), Montgomery–Åsberg Depression Rating Scale, Health of the Nation Outcome Scale, and Clinical Global Impression-Improvement (CGI-I), along with quality-of-life measures such as the WHO Disability Assessment Schedule, WHO Quality of Life scale, and Multidimensional Scale of Perceived Social Support (MSPSS) were evaluated over a 3-month period. Statistical analyses, including analysis of covariance and logistic regression, were used to determine the effectiveness of S-ICM.
Results:
The S-ICM group had significantly lower odds of self-harm or suicidal attempts compared to the control group (adjusted odds ratio [aOR]=0.30, 95% confidence interval [CI]: 0.21–1.38). Psychiatric symptoms measured by the BPRS and perceived social support measured by the MSPSS significantly improved in the S-ICM group. The S-ICM group also had significantly higher odds of CGI-I compared to the control group (aOR=8.20, 95% CI: 2.66–25.32).
Conclusion
This study provides inaugural evidence on the effectiveness of S-ICM services, supporting their standardization and potential nationwide expansion.
10.Acute gouty arthritis of the atlantoaxial joint
Su Jin CHOI ; Min Wook SO ; Sunggun LEE ; Seung Won CHOI ; Doo-Ho LIM
The Korean Journal of Internal Medicine 2025;40(2):341-342

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