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

With the deepening of population aging and the rising prevalence of chronic diseases among the elderly in China， the traditional elderly care models are facing challenges. This paper proposed a community-integrated medical-nursing-rehabilitation-care service model， characterized by “integration， flexibility， community orientation， and demand orientation.” This model optimizes and combines various service resources such as healthcare， elderly care， rehabilitation， and nursing at the community level， providing more comprehensive and targeted services for the elderly with chronic diseases in the community. However， a series of challenges existed in dimensions such as the service integration， benefit distribution， resources and funds， as well as policies and systems， hindering the sustainable development of the community-integrated medical-nursing-rehabilitation-care service model. Future efforts should focus on constructing a more standardized collaborative system， clarifying standards for resource investment and benefit distribution， as well as strengthening policy guidance， financial support， and talent team development.

Background:Ideal bowel preparation is the prerequisite for the successful diagnostic and therapeutic colonoscopy.The retention of intestinal bubbles can seriously affect the clarity of the intestinal mucosa and subsequently decrease the detection rate of colonoscopy.Aims:To investigate the application value of dyclonine hydrochloride mucilage in bowel preparation for colonoscopy.Methods:This study was a randomized double-blinded placebo-controlled trial.Patients who underwent colonoscopy from October 2020 to October 2023 at Hainan West Central Hospital were enrolled and randomly allocated into the dyclonine hydrochloride mucilage group and the control group.3 L polyethylene glycol(PEG)+dyclonine hydrochloride mucilage and 3 L PEG+placebo were given for bowel preparation,respectively.The quality of bowel preparation was evaluated by Boston bowel preparation scale(BBPS)score and bubble score.Furthermore,a questionnaire was conducted.The cecal intubation time,withdrawal time,adenoma detection rate and adverse reaction were compared between the two groups.Results:A total of 482 patients who underwent colonoscopy were included.No significant differences in clinical characteristics such as gender,age,body mass index(BMI)and main reasons for colonoscopy were found between the dyclonine hydrochloride mucilage group and the control group(all P>0.05).Compared with the control group,no significant differences existed in total BBPS score and segment scores for right,transverse,and left colon in the dyclonine hydrochloride mucilage group(all P>0.05),but the total bubble score and segment scores for right,transverse,and left colon were significantly decreased(all P<0.001).The withdrawal time in the dyclonine hydrochloride mucilage group was significantly decreased compared to the control group(P<0.001),and the adenoma detection rate was significantly increased(P=0.001).However,no significant differences in cecal intubation time and incidence of adverse reaction were found between the two groups(all P>0.05).Conclusions:Administration of dyclonine hydrochloride mucilage during bowel preparation for colonoscopy can reduce the formation of intestinal bubbles,shorten the withdrawal time and increase the adenoma detection rate.

Objective To study the intervention effect of nursing staff's participation in antimicrobial stewardship(AMS)on the rational use of antimicrobial agents,and explore its role in constructing a scientific healthcare-associa-ted infection(HAI)control management.Methods The data on perioperative prophylactic use of antimicrobial agents,surgical-related HAI control,and pathogen detection before therapeutic use of antimicrobial agents among hospitalized patients in a hospital from January 2016 to December 2024 were collected.Relevant evaluation indica-tors before and after nursing staff participating in AMS were compared.2016-2018,2019-2021,and 2022-2024 were stages before intervention,during intervention,and after intervention,respectively.Results After nursing staff participated in AMS,the use rate of prophylactic antimicrobial agents 0.5-1 hour before surgery and discon-tinuation rate of antimicrobial agents within 24 hours after class Ⅰ incision surgery increased from 64.54％and 81.41％before intervention to 75.31％and 84.56％after intervention,respectively.Incidences of surgical-related HAI and surgical site infection in patients decreased from 3.11％and 0.96％before intervention to 1.37％and 0.17％after intervention,respectively.Pathogen detection rates before restricted-and special-grade antimicrobial agents treatment increased from 50.80％and 68.70％before intervention to 55.19％and 80.53％after interven-tion,respectively.Proportion of blood specimen from which coagulase-negative Staphylococcus was detected de-creased from 29.30％before intervention to 21.26％after intervention.Proportion of respiratory specimen from which Haemophilus influenzae was detected increased from 2.00％to 3.98％.Differences were all statistically sig-nificant(all P＜0.05).Conclusion As important members of the AMS team,nursing staff can effectively reduce irrational antimicrobial use,optimize medication timing and duration,and have a positive effect on ensuring patient safety through participating in the use and management of antimicrobial agents in hospitalized patients.

Objective To explore the characteristics and prognosis of follicular lymphoma(FL)with bulky disease under rituximab-based first-line treatment.Methods A retrospective analysis was conducted on 525 FL patients diagnosed between September 2009 and September 2021 who received rituximab as a first-line treat-ment[342 patients received rituximab combined with chemotherapy(R-chemo),183 patients received rituximab plus lenalidomide(R2)].The clinicopathologic characteristics,gene mutations,and prognosis of bulky FL patients were analyzed.Results Compared to non-bulky FL patients,bulky FL patients had a significantly higher proportion of lymph node≥5 sites,≥2 extranodal involvement,bone marrow involvement,elevated LDH,and a higher proportion in the high-risk group of FLIPI1 and FLIPI2.Gene sequencing revealed a significantly higher mutation rate of ZNF608 in bulky FL patients compared to non-bulky FL patients.In patients receiving R-chemo as the first-line treatment,there was no significant difference in progression-free survival(PFS)and overall survival(OS)between bulky and non-bulky FL patients.However,in patients treated with R2,the PFS and OS of bulky FL patients was significantly shorter.Conclusions Bulky FL patients compared to non-bulky FL patients have a significantly higher proportion of high-risk baseline characteristics.For bulky FL at diagnosis,chemo-free regimens require further exploration on the basis of R2.

With the extended survival period of breast cancer patients and the increasing health demands, the concomitant diseases of breast cancer have gradually attracted the attention of both doctors and patients, and it is imperative to conduct comprehensive management of these diseases, in which the general practitioners, as the more comprehensive and complex medical talents, have not yet played their due roles. In this article, we report two cases of comprehensive management of concomitant diseases of breast cancer through collaboration of general practitioners and specialists (integrated general and specialist care). The role and function of general practitioners in this process were deeply analyzed, and the establishment of a consultation-liaison general practice model to further promote the role of integrated general and specialist care in integrated oncology care was advocated.

Objective To evaluate the efficacy and safety of enteric-coated metformin hydrochloride capsules(Junlida?)in patients with T2DM and poor glycemic control under lifestyle interventions.Methods In this study,419 patients with T2DM were recruited from 15 research centers from July 2020 to March 2022,and randomly divided into observation(Obs)group(n=209)and control group(Con,n=210)using a multicenter,randomized,double-blind,non-inferiority trial design.Patients in the Obs group were treated with enteric-coated Metformin hydrochloride capsules(Junlida?),and patients in the Con group were treated with Metformin hydrochloride tablets(Glucophage?).The optimal effective dose of 2 g/d was achieved within 4 weeks,and the reasonable dose was maintained until the end of treatment.The treatment period was 24 weeks.HbA1c and its compliance rate,FPG,and body weight were compared between the two groups in full analysis set(FAS)and protocol set(PPS).Safety and adverse events(AE)were evaluated in safety set(SS).Results A total of 414 participants were randomized(207 cases in Obs group and 207 cases in Con group).414 cases in FAS population(207 cases in Obs group and 207 cases in Con group),and 328 cases in PPS population(164 cases in Obs group and 164 cases in Con group),and 414 cases in SS population(207 cases in Obs group and 207 cases in Con group).After treatment,HbA1c,FPG and body weight were lower in both groups(P<0.05)in FAS and PPS.HbA1c compliance rate was not significantly different between the two groups in FAS and PPS(P>0.05).The results of non-inferiority test showed that the lower limit was>-0.4%in both FAS(-0.154,95%CI-0.384～0.069)and PPS(-0.139,95%CI-0.390～0.112),and the Obs group reached non-inferiority end point.The achievement rate,compliance rate,safety index and incidence of AE were not significantly different between the two groups(P>0.05).Conclusions Junlida? demonstrated non-inferiority to Glucophage? in glycemic control and can be safely and effectively used in patients with diabetes.

Objective:To analyze the equity of elderly care institution resource allocation in Northeast China and its influencing factors,and provide certain reference for the further development of elderly care care in Northeast China.Methods:The total amount of nursing institution resource allocation and the per capita old and above per 1 000 km2 were summarized by using general descriptive analysis.The Lorenz curve,Gini coefficient,and Theil index were calculated to evaluate the equity of nursing institution resource allocation.Results:Jilin Province did not have an advantage in the total allocation of elderly care insitution resources in Northeast China.In terms of resource allocation per 1 000 km2,Heilongjiang Province still has considerable room for improvement.The overall arc of the Lorenz curve for allocating resources by geographical area has become smaller year by year,while the overall arc of the Lorenz curve for allocating resources by population has become larger year by year.Conclusion:The equity of allocating institution home resources by population has generally weakened over the years,while the equity of allocating elderly care institution resources by geographical area has generally strengthened over the years.The development pressure of nursing care in Liaoning Province is relatively large,and efforts should be made to enhance the fairness of nursing home resource allocation.

AIM To study the chemical constituents from the water fraction of rhizoma of Smilax trinervula Miq.and their biological activities.METHODS Polyamide,silica gel,Sephadex LH-20,ODS and semi-preparative HPLC were used for isolation and purification,then the structures of obtained compounds were identified by physicochemical properties and spectral data.The antitumor activities were determined by MTT mothod,and the inhibitory activities on α-glucosidase were determined by PNPG method.RESULTS Eleven compounds were isolated and identified as tyrosine(1),uridine(2),2-(2',3',4'-trihydroxybutyl)-6-(2",3",4"-trihydroxybutyl)-pyrazine(3),2-(1',2',3',4'-tetrahydroxybutyl)-6-(2",3",4"-trihydroxybutyl)-pyrazine(4),2-(1',2',3',4'-tetrahydroxybutyl)-5-(2",3",4"-trihydroxybutyl)-pyrazine(5),uracil(6),2-(1',2',3',4'-tetrahydroxybutyl)-5-(1",2",3",4"-tetrahydroxybutyl)-pyrazine(7),dioscin(8),shikimic acid(9),pyrazine(10),3,4-dihydroxyphenyethyl alcohol 8-O-β-D-glycopyranoside(11).The IC50 values of compounds 8 to human breast cancer cell MCF-7 was(2.36±0.26)μg/mL,and the IC50 values of compounds 3-5 and 7 to α-glucosidase were(1.54±0.15)-(10.53±0.38)μg/mL.CONCLUSION Compounds 1-7,10 are isolated from Smilax genus for the first time,and compound 9,11 are first isolated from this plant.Compound 8 has anti-tumor activity,and compounds 3-5,7 have α-glucosidase inhibitory activities.

BACKGROUND:The pelvis has abundant trabecular bone content,but the ability of conventional sacroiliac percutaneous fixation to control trabecular bone is limited,leading to fixation failure.Therefore,the development of devices that can more effectively control trabecular bone tension is of significant importance.OBJECTIVE:The mechanical properties of a novel sacroiliac tension screw were investigated using biomechanical testing and numerical modeling analysis,along with an assessment of the reliability of the pull-out force numerical model.METHODS:A mechanical model was established based on the working principle of the novel sacroiliac tension screw.Numerical methods were employed to analyze its pull-out performance,validated through mechanical testing with polyurethane material to assess the reliability of the pull-out force numerical model.Using pelvic specimens,the mechanical effectiveness of the novel sacroiliac tension screw in repairing sacroiliac joint injuries was analyzed under normal standing posture,along with an evaluation of the load stiffness of different pelvic models in the standing position.RESULTS AND CONCLUSION:(1)The average error between the computed values of the numerical model and the measured values was 13.19％,indicating a certain level of validity for the numerical model.(2)The damage to the polyurethane material after the extraction of the screw was less pronounced in the novel screw group.(3)The average effective holding displacement for the novel screw was approximately(9.24±0.27)mm,significantly greater than the average displacement of(1.71±0.57)mm observed with the lag screws.However,the maximum resistance to pullout for the lag screws was significantly higher than that for the novel screws.(4)The novel screw effectively repaired sacroiliac joint injuries.(5)The stiffness after repair of sacroiliac joint injuries was equivalent when using a single novel screw compared to using two lag screws.(6)These results prove that the theoretical model for the maximum resistance to pullout of the screws established in this study has a certain level of validity and can guide the design of them with improved mechanical performance.The novel sacroiliac spiral blade screw can effectively hold trabecular bone and has practical clinical utility.