Achondroplasia (ACH) is a common inherited skeletal dysplasia (inherited dwarfism) that compromises quality of life across the lifespan. In 2021, vosoritide became the first approved precision therapy for ACH and is now available in more than 40 countries. Compared with prior symptomatic measures, vosoritide has demonstrated favorable efficacy and a reassuring safety profile. Nevertheless, existing international ACH guidelines largely emphasize complication management and symptomatic care, and there is no unified consensus on pharmacologic therapy. To address this gap, an international expert group developed the International Consensus Guidelines for the Implementation and Monitoring of Vosoritide Therapy in Patients with Achondroplasia providing systematic recommendations that span the continuum of care - from initial patient contact and pre-treatment assessment to medication counseling, injection training, and long-term outcome monitoring. These recommendations complement and refine current management and nursing protocols for individuals with ACH and offer practical guidance for clinicians across diverse regions. This article highlights key elements of the guideline to provide evidence-based support and clinical direction for healthcare professionals in China treating children with ACH using vosoritide.
Humans ; Achondroplasia/drug therapy* ; Consensus ; Practice Guidelines as Topic ; Child

OBJECTIVE: To summarize the clinical features and management of two brothers affected with X-linked inhibitor of apoptosis protein (XIAP) deficiency. METHODS: This study retrospectively analyzed the clinical presentations, treatment, and follow-up of two brothers with XIAP deficiency diagnosed at Shanghai Children's Hospital in 2020, and summarized similar cases recorded in databases such as PubMed, Wanfang, Chinese Medical Association Journals, and WIP from January 2006 to November 2024. This study was approved by the Medical Ethics Committee of our hospital (Ethics No.: 2025R128-E01). RESULTS: Patient 1 was the younger brother, who presented at 8 years of age with growth retardation, folliculitis, erythema nodosum, and perineal abscess. Sequencing revealed that he has carried a hemizygous c.566T>C (p.Leu189Pro) variant of the XIAP gene, which was inherited from his mother. He was allergic to infliximab treatment and underwent allogeneic stem cell transplantation (HSCT) in January 2021. During a follow-up of 3 years and 10 months post-transplantation, he showed no gastrointestinal symptoms and had a good outcome. Patient 2 was the elder brother, who presented at 10 years and 6 months of age with growth retardation, rash, and anal fistula. Genetic testing revealed the same variant. He was treated with oral azathioprine but did not have regular follow-ups. At 14-years-and-6-months of age, he had developed severe gastrointestinal infection and hemophagocytic lymphohistiocytosis, which was alleviated after treatment with antibiotics, glucocorticoids, immunoglobulin, and rituximab. He is currently being prepared for HSCT. A total of 13 publications were retrieved, which involved 64 patients from 23 families, with 23 different variants identified. The main clinical manifestations included splenomegaly (34 cases, 53.1%), hemophagocytic lymphohistiocytosis (27 cases, 42.2%), and inflammatory bowel disease or colitis (20 cases, 31.8%). There were significant phenotypic differences among patients from the same family. Thirteen patients (20.3%) underwent HSCT, with a survival rate of 61.5%. CONCLUSION For male children with early onset, poor treatment response, especially those with unexplained splenomegaly and IBD-like symptoms, early genetic testing is recommended. HSCT is a safe and effective treatment for XIAP deficiency. For patients with developmental delay, early onset, and severe IBD phenotype, early transplantation is recommended.
Humans ; Male ; X-Linked Inhibitor of Apoptosis Protein/deficiency* ; Child ; Genetic Diseases, X-Linked/therapy* ; Phenotype ; Siblings ; Retrospective Studies ; Hematopoietic Stem Cell Transplantation

Drug-induced sleep endoscopy (DISE) is used for directly visualizing sites of obstruction among patients with obstructive sleep apnea (OSA). Owing to the scarcity of data, there is still no consensus on the anesthetic regimen for conducting pediatric DISE.

This paper presents a 5-year-old patient who underwent DISE using an opioid-sparing regimen with dexmedetomidine and propofol infusion.

Simultaneous dexmedetomidine and propofol infusion is a promising opioid-sparing regimen for pediatric DISE.

Human ; Male ; Child Preschool: 2-5 Yrs Old ; Endoscopy ; Propofol ; Dexmedetomidine ; Sleep Apnea, Obstructive ; Anesthetics ; Apnea ; Consensus ; Paper ; Patients ; Pharmaceutical Preparations ; Research Report ; Sleep ; Sleep Apnea Syndromes ; World Health Organization

OBJECTIVES

This study explored the interplay of three Filipino cultural values—hiya (modesty/self-restraint), pakikisama (conflict-avoidance dimension of social harmony), and pakikipag-ugnayan (professional engagement, characterized by vigor, dedication, and absorption)—among Generation Z nurses in Quezon City. Specifically, it assessed their levels, examined interrelationships through correlation and path analysis, and developed a framework to illustrate their influence on nursing practice. In the Filipino context, hiya encompasses both positive dimensions, such as conscientiousness, moral accountability, and social responsibility, and constraining dimensions, including excessive self-restraint, fear of criticism, and inhibition. Pakikisama, while fostering harmony, may discourage assertiveness and limit open communication. Pakikipag-ugnayan, conceptualized here as engagement, reflects dedication, vigor, and absorption, which are vital for performance, patient safety, and workforce retention.

METHODS

A descriptive–correlational design was utilized to assess the relationships among hiya, pakikisama, and pakikipag-ugnayan. Data were gathered through a structured, self-administered questionnaire comprising validated scales for each construct, distributed in online formats to accommodate participants’ availability and work schedules. The purposive sample consisted of 115 Filipino Generation Z nurses engaged in direct patient care in various healthcare facilities in Quezon City. Data analysis involved descriptive statistics to summarize variable scores, Spearman’s rho to examine bivariate correlations, and path analysis to determine the direct and indirect effects among the variables. The results of the path analysis were used to develop a proposed framework illustrating the interplay of these cultural values in nursing practice.

RESULTS

Path analysis revealed distinct patterns in the interplay of hiya, pakikisama, and pakikipag-ugnayan among Generation Z nurses in Quezon City. Very high hiya scores (mean = 5.32) reflected both moral accountability and restraint-oriented tendencies. Despite these positive indicators, hiya showed a negative correlation with pakikipag-ugnayan, suggesting that its inhibiting side—marked by fear of criticism and avoidance of mistakes—may overshadow its constructive moral aspects. In contrast, the path analysis identified a moderate positive direct effect between hiya and pakikipag-ugnayan (β = 0.400, pCONCLUSION

Among Generation Z nurses, hiya demonstrates a dual role: while its ethical and conscientious aspects can strengthen pakikipag-ugnayan, its inhibiting side may suppress pakikipag-ugnayan. Pakikisama, operationalized as conflict avoidance and withholding dissent, emerged as a barrier to pakikipag-ugnayan by discouraging open communication. These findings informed the development of the Hiya–Pakikisama– Pakikipag-ugnayan Cultural Dynamics Framework, which illustrates how cultural values can simultaneously enable and constrain professional engagement. The framework has potential applications in nursing education and organizational practice, particularly in designing reflective learning, mentorship, and communication strategies that balance cultural sensitivity with assertiveness. Further validation across generations and healthcare settings is recommended to refine its applicability.

Human ; Weights And Measures ; Sensitivity And Specificity ; Retention (psychology) ; Education, Nursing ; Social Responsibility ; Appointments And Schedules ; Conflict (psychology)

BACKGROUND AND OBJECTIVES

One of the effects of the COVID-19 pandemic on medical education is an increased awareness and use of social media (SocMed) to facilitate learning. However, literature on the use of SocMed in medical education has focused primarily on educator-led teaching activities. Our study aimed to describe SocMed initiatives that were student-led, particularly for information dissemination and peer collaborative learning, and to elicit perceptions of medical students towards such activities.

METHODS

An online survey on SocMed usage in medical education was sent to all first- and second-year medical students at the University of the Philippines Manila College of Medicine from October to December 2021. The questionnaire collected data on demographics, SocMed habits and preferences, and perceived advantages and disadvantages of SocMed. Descriptive statistics were calculated while the free-text responses were grouped into prominent themes and summarized.

RESULTS

We received a total of 258 responses (71%) out of 361 eligible participants. Overall, 74% found SocMed platforms to be very and extremely helpful; 88% recommended its continued use. The most popular SocMed platforms for different tasks were as follows: Discord for independent study groups and for conducting peer tutoring sessions; Facebook Messenger for reading reminders; Telegram for reading announcements related to academics and administrative requirements, and for accessing material provided by classmates and professors.

CONCLUSION

The high uptake of SocMed among medical students may be attributed to its accessibility and costefficiency. The use of a particular SocMed platform was dependent on the students’ needs and the platform's features. Students tended to use multiple SocMed platforms that complemented one another. SocMed also had disadvantages, such as the potential to distract from academic work and to become a source of fatigue. Educators must engage with students to understand how SocMed platforms can be integrated into medical education, whether in the physical or virtual learning environment.

Human ; Education, Medical, Undergraduate ; Social Media ; Online Learning ; Education, Distance

OBJECTIVES: To study the therapeutic mechanism of Tuihuang Mixture against cholestasis. METHODS: Forty-eight Wistar rats were randomized equally into blank group, model group, ursodeoxycholic acid group and Tuihuang Mixture group. Except for those in the blank group, all the rats were given α‑naphthylisothiocyanate (ANIT) to establish rat models of cholestasis, followed by treatments with indicated drugs or distilled water. Serum levels of ALT, AST, ALP, γ-GT, TBA and TBIL of the rats were determined, and hepatic expressions IL-1β, IL-18, FXR, NLRP3, ASC, Caspase-1 and GSDMD were detected using q-PCR, ELISA or Western blotting. Histopathological changes of the liver tissues were observed using HE staining. RESULTS: The rat models of cholestasis had significantly increased serum levels of ALT, AST, ALP, γ-GT, TBA and TBIL with increased mRNA and protein expressions of IL-1β and IL-18, decreased protein and mRNA expressions of FXR, and increased protein expressions of NLRP3 and Caspase-1 and mRNA expressions of NLRP3, ASC, Caspase-1 and GSDMD in the liver tissue, showing also irregular arrangement of liver cells, proliferation of bile duct epithelial cells and inflammatory cells infiltration. Treatment of the rat models with Tuihuang Mixture significantly decreased serum levels of ALT, AST, ALP, γ-GT, TBA and TBIL, lowered IL-1β and IL-18 and increased FXR protein and mRNA expressions, and reduced NLRP3, ASC, Caspase-1 and GSDMD proteins and NLRP3, ASC and Caspase-1 mRNA expressions in the liver tissue. Tuihuang Mixture also significantly alleviated hepatocyte injury, bile duct epithelial cell proliferation and inflammatory cell infiltration in the liver of the rat models. CONCLUSIONS Tuihuang Mixture can effectively improve cholestasis in rats possibly by inhibiting NLRP3 inflammatosome-mediated pyroptosis via regulating FXR.
Animals ; NLR Family, Pyrin Domain-Containing 3 Protein ; Rats ; Receptors, Cytoplasmic and Nuclear/metabolism* ; Cholestasis/drug therapy* ; Rats, Wistar ; Inflammasomes/metabolism* ; 1-Naphthylisothiocyanate ; Drugs, Chinese Herbal/therapeutic use* ; Male ; Interleukin-18/metabolism* ; Caspase 1/metabolism* ; Interleukin-1beta/metabolism* ; Liver/metabolism*

OBJECTIVES: We propose a multimodal model integrating social media text and image data for automated assessment of psychological stress in college students to support the development of intelligent mental health services in higher education institutions. METHODS: Based on deep learning technology, we designed an evaluation framework comprising a text sentiment modeling module, an image sentiment modeling module, and a multimodal fusion prediction module. Text sentiment features were extracted using Bi-LSTM, and image semantic cues were extracted via U-Net. A feature concatenation strategy was used to enable cross-modal semantic collaboration to achieve automatic identification of 3 psychological stress levels: mild, moderate, and severe. We constructed a multimodal annotated dataset using social platform data from 1577 students across multiple universities in Guangdong Province. After data cleaning, 252 samples were randomly selected for model training and testing. RESULTS: In the 3-classification task, the model demonstrated outstanding performance on the test set, and achieved an accuracy of 92.86% and an F1 score of 0.9276, exhibiting excellent stability and consistency. Confusion matrix analysis further revealed the model's ability to effectively distinguish between different pressure levels. CONCLUSIONS The multimodal psychological stress assessment model developed in this study effectively integrates unstructured social behavior data to enhance the scientific rigor and practical applicability of psychological state recognition, and thus provides support for developing intelligent psychological service systems.
Humans ; Stress, Psychological/diagnosis* ; Students/psychology* ; Universities ; Social Media ; Deep Learning

The expert consensus on the clinical treatment of herpes zoster with fire needling was developed, and the commonly used fire needling treatment scheme verified by clinical research was selected to form a standardized diagnosis and treatment scheme for acute herpes zoster and postherpetic neuralgia (PHN), so as to answer the core problems in clinical application. The consensus focuses on patients with herpes zoster, and forms recommendations for 9 key clinical issues, covering simple fire needling and TCM comprehensive therapy based on fire needling, including fire needling combined with cupping, fire needling combined with Chinese herb, fire needling combined with cupping and Chinese herb, fire needling combined with filiform needling, fire needling combined with moxibustion, and provides specific recommendations and operational guidelines for various therapies.
Humans ; Herpes Zoster/therapy* ; Acupuncture Therapy/instrumentation* ; Consensus ; Clinical Protocols

INTRODUCTION: Obstructive sleep apnoea (OSA) is common in Singapore, with moderate to severe OSA affecting around 30% of residents. These consensus statements aim to provide scientifically grounded recommendations for the management of OSA, standar-dise the management of OSA in Singapore and promote multidisciplinary collaboration. METHOD: An expert panel, which was convened in 2024, identified several areas of OSA management that require guidance. The expert panel reviewed the current literature and developed consensus statements, which were later independently voted on using a 3-point Likert scale (agree, neutral or disagree). Consensus (total ratings of agree and neutral) was set a priori at ≥80% agreement. Any statement not reaching consensus was excluded. RESULTS: The final consensus included 49 statements that provide guidance on the screening, diagnosis and management of adults with OSA. Additionally, 23 statements on the screening, diagnosis and management of paediatric OSA achieved consensus. These 72 consensus statements considered not only the latest clinical evidence but also the benefits and harms, resource implications, feasibility, acceptability and equity impact of the recommendations. CONCLUSION The statements presented in this paper aim to guide clinicians based on the most updated evidence and collective expert opinion from sleep specialists in Singapore. These recommendations should augment clinical judgement rather than replace it. Management decisions should be individualised, taking into account the patient's clinical characteristics, as well as patient and caregiver concerns and preferences.
Humans ; Sleep Apnea, Obstructive/diagnosis* ; Singapore ; Consensus ; Adult

Humans ; Female ; Pregnancy ; Cesarean Section/standards* ; China ; Consensus ; Postoperative Complications/prevention & control* ; Obstetric Labor Complications/surgery*