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

Narcotic plants are strictly regulated worldwide due to their ability to extract drug alkaloids and drug precursor components. Besides the three traditional core species, cannabis, opium poppy, and coca, the misuse of psychoactive plants with addictive properties has become increasingly prevalent globally in recent years, and the establishment of accurate identification methods for such plants has become an urgent need in the field of narcotics control. Within existing identification frameworks, the conventional morphological and chemical analysis methods, despite their long-term application, have demonstrated considerable limitations. In contrast, DNA-based molecular identification techniques have achieved significant advancement in recent years due to their high specificity and stability. This review comprehensively examines current DNA-based identification approaches for narcotic plants through three key dimensions: DNA molecular marker technology, DNA barcoding technology, and emerging molecular biological techniques, and elaborates on the principles, technical characteristics, application scenarios, and research progress of each technology, providing some reference for the scientific selection of DNA identification strategies for narcotic plants in different specific scenarios.

This study examined the regulatory effects of autonomic nervous system on aerobic endurance exercise performance in cold exposure, focusing on heart rate recovery (HRR) and heart rate variability (HRV) across genders. Thirty participants (17 males and 13 females) from a university track endurance program, classified as exercise grade II or above, underwent monitoring of HRV in time domain, frequency domain, nonlinear correlation indices and 1 min HRR. Measurements were taken before, during, and after aerobic endurance exercise in cold and normal environments, respectively. The results were as follows. (1) The duration of aerobic endurance exercise completed by all the subjects in cold environment was significantly increased compared with that in normal environment. The 1 min HRR after aerobic endurance exercise in cold environment was significantly lower than that in normal environment, and the decrease in the males was significantly higher than that in the females. (2) The time domain analysis results showed that, prior to the aerobic endurance exercise, there were no significant difference of standard deviation from the mean value of normal to normal intervals (SDNN), root mean square of successive differences (RMSSD), and percentage of adjacent normal-to-normal intervals differing by more than 50 ms (pNN50) between cold and normal environments. During aerobic endurance exercise in cold environment, SDNN, RMSSD and pNN50 were significantly higher than those in normal environment, with the females showing significantly greater increases compared with those of the males. The levels of SDNN, RMSSD and pNN50 in the males at different time points under different environments were significantly lower than those in the quiet state; The levels of SDNN and RMSSD of the females at different time points under different environments were significantly lower than those in the quiet state, while the pNN50 at different time points under cold environments was significantly lower than that in the quiet state. (3) Frequency domain analysis results showed that, prior to the aerobic endurance exercise, there was no significant difference of high frequency normalized units [HF (n.u.)], low frequency normalized units [LF (n.u.)] and LF/HF ratio between cold and normal environments. During aerobic endurance exercise in cold environment, the levels of HF (n.u.) significantly increased compared to normal environment in the females, while LF (n.u.) and LF/HF ratio levels significantly decreased compared to normal environments. The levels of HF (n.u.), LF (n.u.) and LF/HF ratio of different genders at different time points in the different environments showed no significant changes, compared to those in the quiet state. (4) Non-linear analysis results showed a significant increase in SD1 (standard deviation perpendicular to the line-of-identity)/SD2 (standard deviation along the line-of-identity) ratio during aerobic endurance exercise in cold environment in the females, while no significant changes were observed in the males. SD1/SD2 ratios in the males at different time points and in the females at 1 min under cold environments were significantly higher than those in the quiet state. These findings suggest that aerobic endurance performance increases during cold exposure, accompanied by gender-specific differences in the regulation of autonomic nervous system. Females exhibit higher vagal activity and faster autonomic nervous system recovery compared to males.
Humans ; Male ; Female ; Heart Rate/physiology* ; Cold Temperature ; Exercise/physiology* ; Physical Endurance/physiology* ; Autonomic Nervous System/physiology* ; Young Adult ; Adult ; Sex Factors

With Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum drug pair as the research object, supramolecular chemistry of traditional Chinese medicine(TCM) was used to study differences between the compatibility of herbal medicine Glycyrrhizae Radix et Rhizoma with mineral medicine Gypsum Fibrosum and its main component CaSO_4·2H_2O, so as to preliminarily discuss the scientific connotation of compatibility of Gypsum Fibrosum in clinical application. A Malvern particle sizer, a scanning electron microscope(SEM), and a conductivity meter were used to observe and determine the physical properties such as microscopic morphology, particle size, and conductivity of Gypsum Fibrosum, CaSO_4·2H_2O, and water decoctions of them with Glycyrrhizae Radix et Rhizoma. An inductively coupled plasma optical emission spectrometer(ICP-OES) was employed to detect the inorganic metal elements in Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum and Glycyrrhizae Radix et Rhizoma-CaSO_4·2H_2O. Isothermal titration calorimetry(ITC) was conducted to quantify the interactions of Gypsum Fibrosum and CaSO_4·2H_2O with Glycyrrhizae Radix et Rhizoma. A Fourier transform infrared spectrometer(FTIR) was used to analyze the characteristic absorption peak change of Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum and Glycyrrhizae Radix et Rhizoma-CaSO_4·2H_2O. X-ray diffraction(XRD) was performed to determine the crystal structure and phase composition of Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum and Glycyrrhizae Radix et Rhizoma-CaSO_4·2H_2O. Further, glycyrrhizic acid(GA) was substituted for Glycyrrhizae Radix et Rhizoma to co-decoct with Gypsum Fibrosum, CaSO_4·2H_2O, and freeze-dried powder of their respective water decoctions. The results of XRD were used for verification analysis. The results showed that although CaSO_4·2H_2O is the main component of Gypsum Fibrosum, there were significant differences between their decoctions and between the decoctions of them with Glycyrrhizae Radix et Rhizoma. Specifically,(1) Both CaSO_4·2H_2O and Gypsum Fibrosum were amorphous fibrous. However, the particle size and conductivity were significantly different between the decoctions of CaSO_4·2H_2O and Gypsum Fibrosum alone.(2) Under SEM, Glycyrrhizae Radix et Rhizoma-CaSO_4·2H_2O was a hybrid system with various morphologies, while Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum presented uniform nanoparticles.(3) The particle sizes and conductivities of Glycyrrhizae Radix et Rhizoma-CaSO_4·2H_2O and Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum were significantly different and did not follow the same tendency as those of the decoctions of CaSO_4·2H_2O and Gypsum Fibrosum alone.(4) Compared with Glycyrrhizae Radix et Rhizoma-CaSO_4·2H_2O, Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum had stronger molecular binding ability and functional group structure change.(5) The crystal form was largely different between the freeze-dried powder of CaSO_4·2H_2O decoction and Gypsum Fibrosum decoction, and their crystal forms were also significantly different from those of the freeze-dried powder of Glycyrrhizae Radix et Rhizoma-CaSO_4·2H_2O and Glycyrrhizae Radix et Rhizoma-Gypsum Fibrosum decoctions. The reason for the series of differences is that Gypsum Fibrosum is richer in trace elements than CaSO_4·2H_2O. The XRD results of GA-Gypsum Fibrosum and GA-CaSO_4·2H_2O decoctions further prove the importance of trace elements in Gypsum Fibrosum for supramolecule formation. This research preliminarily reveals the influence of compatibility of Gypsum Fibrosum or CaSO_4·2H_2O on decoction phase state, material form, and crystal form, providing a basis for the rational clinical application of Gypsum Fibrosum.
Drugs, Chinese Herbal/chemistry* ; Calcium Sulfate/chemistry* ; Glycyrrhiza/chemistry* ; Crystallization ; Particle Size ; Medicine, Chinese Traditional ; Rhizome/chemistry*

Objective:To compare the dosimetric characteristics of three fractionated stereotactic radiotherapy techniques,i.e.,tomo-therapy(TOMO),volumetric-modulated arc therapy(VMAT),and CyberKnife(CK),in the treatment of intracranial oligometastases,and to assess their dose distribution,treatment efficiency,and difference in dose delivered to organs at risk(OARs).Methods:A retro-spective analysis was performed for the clinical data of 54 patients with intracranial oligometastases who underwent fractionated stereo-tactic radiotherapy in The First Affiliated Hospital of Army Medical University in 2021-2023.Varian Eclipse 16.1 Physician Worksta-tion was used to perform tumor target volume delineation,and MANTEIA AccContour 3.2 software was used to perform the delineation of OARs,such as brainstem,spinal cord,and optical nerves.The delineated structures and images were transmitted to TOMO,CK,and Eclipse treatment planning systems to design three different radiotherapy treatment plans.Related key parameters were analyzed using the dose-volume histogram to evaluate the dosimetric characteristics of these three radiotherapy techniques,including conformity index(CI)of the target,dose homogeneity index(HI),beam-on time,the number of monitor units(MU),and the exposure dose of OARs.Results:All three treatment plans(TOMO,VMAT,and CK)met the requirements for prescribed dose.TOMO had a slightly better CI than VMAT and CK(1.05 vs.1.09 and 1.17,P<0.001).VMAT had a better HI than CK and TOMO(1.15 vs.1.28 and 1.46,P<0.001).In terms of execution efficiency,VMAT had a significantly shorter beam-on time than TOMO and CK(5 minutes,1 633 MU vs.10 minutes,8 932 MU and 39 minutes,5 191 MU,P<0.001).In terms of the exposure dose of OARs,CK provided the best protection for the lens,with a maximum dose of 15 cGy for the right lens and 17 cGy for the left lens,and TOMO had an advantage in dose control for the right cochlea,with a mean dose of 88 cGy,while VMAT had the best performance in limiting the dose for the spinal cord,with a maximum dose of 31 cGy(P<0.05).Conclusion:This study shows that TOMO,VMAT,and CK all meet the requirements for the prescribed dose and can effectively protect OARs in the treatment of in-tracranial oligometastases.In clinical practice,the most appropriate technique should be selected based on the features of lesions and treatment goals to achieve individualized treatment.

OBJECTIVES: To evaluate the efficacy and safety of avatrombopag in promoting platelet engraftment after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children, compared with recombinant human thrombopoietin (rhTPO). METHODS: A retrospective analysis was conducted on 53 pediatric patients who underwent allo-HSCT at the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences from April 2023 to August 2024. Based on medications used during the periengraftment period, patients were divided into two groups: the avatrombopag group (n=15) and the rhTPO group (n=38). RESULTS: At days 14, 30, and 60 post-transplant, platelet engraftment was achieved in 20% (3/15), 60% (9/15), and 93% (14/15) of patients in the avatrombopag group, and in 39% (15/38), 82% (31/38), and 97% (37/38) in the rhTPO group, respectively. There were no significant differences between the two groups in platelet engraftment rates at each time point, cumulative incidence of platelet engraftment, overall survival, and relapse-free survival (all P>0.05). Multivariable Cox proportional hazards analysis indicated that acute graft-versus-host disease was an independent risk factor for delayed platelet engraftment (P=0.043). CONCLUSIONS In children undergoing allo-HSCT, avatrombopag effectively promotes platelet engraftment, with efficacy and safety comparable to rhTPO, and represents a viable therapeutic option.
Humans ; Retrospective Studies ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Male ; Female ; Child ; Child, Preschool ; Infant ; Adolescent ; Transplantation, Homologous ; Blood Platelets/drug effects* ; Thiazoles/therapeutic use* ; Thrombopoietin/therapeutic use* ; Thiophenes

Accurate analysis of metal nanoparticles(MNPs)in sediments is a prerequisite for assessing the ecological risks of MNPs in aquatic environmental sediments.In this study,an analytical method for quantitative detection of concentration and particle size distribution of silver-containing nanoparticles(Ag-NPs),zinc-containing nanoparticles(Zn-NPs),cerium-containing nanoparticles(Ce-NPs),and titanium-containing nanoparticles(Ti-NPs)in sediments was established based on ultrasonic extraction-single particle inductively coupled plasma mass spectrometry(SP-ICP-MS).The effects of sample preparation conditions such as extraction solvent type,solid-liquid ratio,ultrasonic time,and settling time on the recovery of MNPs were investigated.The results showed that the extraction of MNPs from sediment by distilled water could effectively eliminate the high background signal interference introduced by the extractant under the conditions of solid-liquid ratio of 1∶400(g∶mL),ultrasonic extraction time of 1 h and settling time of 3 h.The detection limits for particle size of Ag-NPs,Zn-NPs,Ce-NPs and Ti-NPs in sediments were 31,35,26 and 85 nm,respectively,while the detection limits of particle concentrations were 1.21×104,1.90×104,5.26×107 and 1.48×107 particles/g,respectively.The spiking recoveries of Ag-NPs,Zn-NPs,Ce-NPs and Ti-NPs in sediments were 62.1％-108.7％,with relative standard deviations below 10％.This method could rapidly,accurately and simultaneously determine the concentration and particle size distribution of various MNPs in sediments,and was successfully applied to analysis of Ag-NPs,Zn-NPs,Ce-NPs,and Ti-NPs in authentic marine sediments.

Turning to critical illness is a common stage of various diseases and injuries before death. Patients usually have complex health conditions, while the treatment process involves a wide range of content, along with high requirements for doctor′s professionalism and multi-specialty teamwork, as well as a great demand for time-sensitive treatments. However, this is not matched with critical care professionals and the current state of medical care in China. Telemedicine, which shortens the distance of medical professionals and the gap of disease diagnosis and treatments in various regions through electronic information, can effectively solve the current problem. Therefore, there is an urgent need to develop a standardized, high-quality visualization telemedicine round system .Therefore, experts have been organized to search domestic and foreign literature on telemedicine round for critically ill patients and to form this consensus based on clinical experiences so as to further improve the level of critical care treatments in regions.

Thoracic anesthesia with spontaneous respiration represents a form of precision anesthesia meticulously customized to individual patients.Considering the more stringent requirements this anesthesia approach imposes on the regulation of respiratory function,the writing group of the"Consensus of Experts on the Management of Thoracic Anesthesia with Spontaneous Respiration"has formulated elaborate guidelines regarding indications and contraindications,preoperative evaluation,anesthesia implementation,common complications,and treatment strategies.This was accomplished by referencing relevant domestic and international literature and integrating it with actual clinical requirements.The objective is to standardize the rational application of this anesthesia method.