Congenital musculoskeletal and limb deformities (CMLD) seriously affect the physical and mental health of patients, and pose great challenges to healthcare systems worldwide. We explored the specific situation and changes of incidence, prevalence, disability-adjusted life years rates, and mortality of CMLD in under-5 children from 1990 to 2021 in different groups, including different regions, periods, genders and socio-demographic indices (SDI), through corresponding analytical models. Overall, the global disease burden of CMLD in under-5 children has decreased from 1990 to 2021. The disease burden of CMLD in under-5 children varied significantly among different regions and countries, and there was a strong correlation between the corresponding burden of disease and the level of SDI. In addition, cross-country inequality analysis showed that while absolute inequalities in the disease burden of CMLD in under-5 children have improved, relative inequalities have worsened. It is essential to reduce the global health impact of CMLD by implementing targeted interventions to improve health care in underdeveloped areas.
Humans ; Global Burden of Disease/trends* ; Child, Preschool ; Global Health/statistics & numerical data* ; Infant ; Male ; Prevalence ; Limb Deformities, Congenital/mortality* ; Musculoskeletal Abnormalities/mortality* ; Female ; Disability-Adjusted Life Years ; Incidence ; Infant, Newborn ; Cost of Illness ; Socioeconomic Factors

Jasmonic acid (JA) is a common plant hormone with regulatory effects on plant growth and development. The jasmonate ZIM-domain (JAZ) proteins (JAZs), as key regulators in the JA signaling pathway, are involved in multiple biological processes such as anthocyanin accumulation, flowering time modulation, and secondary metabolite synthesis in plants. JAZs are essential components of many regulatory signaling networks. The JAZ genes, members of the plant-specific TIFY family, have been identified in the genomes of a variety of horticultural plants. Here, we summarized the research progress in the roles of JAZs in horticultural plants, aiming to give insights into the further study of the biological functions and regulatory networks of JAZ genes in plants.
Horticulture ; Repressor Proteins/metabolism* ; Plant Proteins/metabolism* ; Cyclopentanes/metabolism* ; Oxylipins/metabolism* ; Plants/metabolism* ; Plant Development

Objective:To study the effects of TCM ointment rubbing technique on pain, swelling, and knee joint function in patients with knee osteoarthritis (KOA) after total knee arthroplasty (TKA).Methods:A randomized controlled trial was conducted. In this study, 80 patients with KOA who underwent TKA treatment in the Department of Sports Medicine I, Wangjing Hospital, China Academy of Traditional Chinese Medicine from October 2022 to March 2024 were taken as the study subjects. They were divided into two groups with random number table method, with 40 cases in each group. Both groups were treated with conventional Western medicine + rehabilitation training after surgery, and the observation group was combined with TCM paste mo technique on this basis. VAS score was used to assess the degree of pain at different time points, and skin fold was used to measure the circumference of the upper knee circumference and the circumference of the lower knee circumference, and knee Injury and osteoarthritis outcome score (KOOS) was used to assess the degree of knee joint function recovery.Results:After treatment, the observation group after surgerythe at 7 d (2.57 ± 0.84 vs. 4.00 ± 0.85, t=7.54) and 14 d (0.80 ± 0.93 vs. 2.70 ± 1.04, t=8.56) VAS scores were lower than those in the control group ( P<0.001). After treatment, the observation group after surgerythe at 7 d the superior circumference [(48.32 ± 4.57) cm vs. (50.53 ± 3.97) cm, t=2.32], and inferior circumference [(36.71 ± 2.95) cm vs. (39.21 ± 6.86) cm, t=2.12], at 14 d the superior circumference [(45.68 ± 4.69) cm vs. (47.96 ± 3.89) cm, t=2.37], and inferior circumference [(34.96 ± 2.96) cm vs. (36.70 ± 4.35) cm, t=2.10] were lower than those in the control group ( P<0.05). The observation group after surgerythe at 14 d the the knee joint mobility [(115.32 ± 2.12) ° vs. (113.34 ± 2.16) °, t=4.14] and KOOS scores (85.52 ± 0.82 vs. 80.32 ± 1.13, t=23.56) were higher than those in the control group ( P<0.01). Conclusion:TCM ointment rubbing technique has significant advantages in improving pain, swelling and knee joint mobility after artificial knee arthroplasty, which can effectively restore knee joint function.

Objective:To evaluate the efficacy and safety of eculizumab in the treatment of paroxysmal nocturnal hemoglobinuria (PNH) in China.Methods:Data from PNH patients who received at least 3 months of full-dose eculizumab and were followed for at least 3 months between December 2022 and July 2023 were retrospectively collected. We evaluated changes in clinical and laboratory parameters after 1, 2, 3, and 6 months of eculizumab treatment. The rates of breakthrough hemolysis (BTH), extravascular hemolysis (EVH), and the occurrence of adverse reactions were also monitored.Results:The study included nine patients, six males and three females, with a median age of 54 (28–69) years. 5 of the patients had classic PNH, while 4 had PNH/AA. The number of episodes of hemoglobinuria was 5 (1–25) per month before eculizumab. 4 patients required blood transfusion, 5 had thrombosis and one had renal impairment before eculizumab. The median time to eculizumab was 6 (3–7) months and the followup period was 3 (3–6) months after treatment. The number of episodes of hemoglobinuria following eculizumab was 0 (0–1). During the followup period, no additional thrombotic events occurred. LDH at any time after eculizumab was lower than at baseline, and some patients' HGB increased. All transfused patients became transfusion-independent after receiving eculizumab. The FACIT-Fatigue score improved by an average of 17.3 points following treatment. 2 patients developed BTH and improved with symptomatic treatment. There were three adverse events that caused mild symptoms. There are no serious adverse events or deaths.Conclusion:Eculizumab can effectively control the hemolytic-related symptoms of PNH in China, reducing the need for blood transfusions to some extent, while also demonstrating a higher safety profile.

Objective:To evaluate the efficacy and safety of roxadustat in patients with refractory non-severe aplastic anemia (NSAA) .Methods:The clinical data of patients with refractory NSAA who had been treated with roxadustat continuously for at least 3 months and followed up for more than 6 months at Peking Union Medical College Hospital from October 2020 to August 2022 were retrospectively collected. The demographic information, clinical data, treatment efficacy, adverse reactions, and outcomes were evaluated, and the factors influencing efficacy were analyzed.Results:A total of 41 patients were included. The male-to-female ratio was 16∶25, and the median age was 52 (18-84) years. The median duration of roxadustat treatment was 5 (3-20) months, and the median follow-up was 15 (6-26) months. Hematologic improvement-erythroid (HI-E) was 12.2%, 29.3%, 46.3%, 43.9%, and 30.3% at 1, 2, 3, 6, and 12 months, respectively. The rate of transfusion independence was 28.5%, 38.1%, and 33.3% at 3, 6, and 12 months, respectively. Hemoglobin returned to normal in some patients after treatment with roxadustat. The incidence of adverse events was 22%, all of which were grade Ⅰ-Ⅱ and recoverable. No factors that could affect HI-E were identified. By the end of follow-up, 45% of the patients relapsed, with a median time to relapse of 7 (3-12) months. No clonal evolution was observed, and one patient died.Conclusion:Roxadustat effectively improved anemia with good tolerance in patients with refractory NSAA.

The chloroplast genome encodes many key proteins involved in photosynthesis and other metabolic processes, and metabolites synthesized in chloroplasts are essential for normal plant growth and development. Root-UVB (ultraviolet radiation B)-sensitive (RUS) family proteins composed of highly conserved DUF647 domain belong to chloroplast proteins. They play an important role in the regulation of various life activities such as plant morphogenesis, material transport and energy metabolism. This article summarizes the recent advances of the RUS family proteins in the growth and development of plants such as embryonic development, photomorphological construction, VB6 homeostasis, auxin transport and anther development, with the aim to facilitate further study of its molecular regulation mechanism in plant growth and development.
Female ; Pregnancy ; Humans ; Ultraviolet Rays ; Biological Transport ; Chloroplasts/genetics* ; Embryonic Development ; Plant Development/genetics*

BACKGROUND:Steroid-induced osteonecrosis of the femoral head is a refractory disease in the field of orthopedics.There is no definitive idea to fully explain its pathogenesis.With the increased research on the active ingredients of Panax notoginseng interfering with the signaling pathways related to various diseases,the active ingredients of Panax notoginseng that treat steroid-induced necrosis of the femoral head via the regulation of relevant signaling pathways have gradually become a hot research topic. OBJECTIVE:To systematically summarize the literature on the pathological mechanism of steroid-induced osteonecrosis of the femoral head and the regulation of signaling pathways by the active ingredients of Panax notoginseng in recent years,thereby providing a reference for the follow-up study on the active ingredients of Panax notoginseng in the treatment of this disease. METHODS:CNKI,WanFang,and PubMed were searched for relevant literature with the key words of"glucocorticoid,steroid-induced osteonecrosis of the femoral head,pathological mechanism,signaling pathway,Panax notoginseng,active ingredient"in Chinese and English.Documents related to the pathological mechanism of steroid-induced osteonecrosis of the femoral head as well as related to the intervention of active ingredients of Panax notoginseng on the signaling pathway of steroid-induced osteonecrosis of the femoral head were retrieved.A total of 63 documents were finally included according to the inclusion and exclusion criteria. RESULTS AND CONCLUSION:The main ingredients of Panax notoginseng include Panax notoginseng saponins,ginsenoside,Panax notoginseng saponins,quercetin,kaempferol,etc.Panax notoginseng saponins,ginsenoside Rb1 and quercetin can promote bone repair and angiogenesis by acting on the transforming growth factor-β/bone morphogenetic protein pathway.Panax notoginseng saponins,ginsenoside CK and kaempferol can promote osteogenic differentiation and lipid metabolism by acting on the Wnt/β-catenin pathway.Panax notoginseng saponins and Panax notoginseng saponins R1/R2 act on the MAPK pathway to inhibit osteoclastogenesis and promote bone repair.Panax notoginseng saponins,ginsenoside Rb2 and quercetin can inhibit osteoclast proliferation and promote osteoblastic differentiation by acting on the RANKL/RANK/OPG pathway.Panax notoginseng saponins,quercetin and kaempferol can repair vascular injury and promote osteogenesis by acting on the hypoxia-inducible factor-1α pathway.Panax notoginseng saponins R1,quercetin combined with hydroxyapatite nanoparticles,Panax notoginseng saponins combined with polyethylene-L-lactic acid and other biomaterials have good research prospects in the treatment of steroid-induced osteonecrosis of the femoral head.The active ingredients of Panax notoginseng can regulate the signaling pathways related to steroid-induced osteonecrosis of the femoral head through various mechanisms,and play an active intervention role in the disease.However,the depth and breadth of relevant research are insufficient at present,and the future research should be based on the existing mechanism to explore the specific mechanism of Panax notoginseng regulating different pathways and the interaction between pathways,which will be beneficial to the multi-development of the active ingredients of Panax notoginseng in the treatment of steroid-induced osteonecrosis of the femoral head.

Objective To investigate the overall and subgroup efficacy,subgroup differences,predictors of efficacy and safety of roxadustat in the treatment of refractory non-severe aplastic anemia(NSAA)and low-risk myelodysplastic syndromes(LR-MDS).Methods Patients with refractory NSAA and LR-MDS who were admitted to the Department of Hematology,Peking Union Medical College Hospital from August 2020 to December 2022 were enrolled.All patients received first-line standard treatment,including recombinant human erythropoietin(rhEPO)for at least 3 months before roxadustat treatment.All patients received roxadustat 2.5 mg/kg every other day for at least 3 months,and were followed up for at least 8 months.The clinical characteristics of patients,roxadustat efficacy,predictors of efficacy,adverse effects,relapse and disease clonal evolution were analyzed.Results A total of 40 patients including 24 refractory NSAA and 16 LR-MDS were included.median age was 56(18～81)years and 40%were males.65%of the patients were transfusion dependent.Median follow-up was 21(9～34)months.22.5%,25.0%,47.5%,55.0%,57.5%,60.0%and 50.0%of the patients achieved haemato-logical improvement-erythroid(HI-E)at months 1,2,3,4,5,6,and end of the follow-up period,respectively,and no factors affecting HI-E were identified.The hemoglobin change from baseline was statistically different between the two groups at the end of the follow-up period.50%of patients were relieved from transfusion dependence.Adverse reactions were reported in 22.5%of patients.28.5%of patients relapsed after achieving HI-E,with a median time to relapse of 7(4～12)months.No clonal evolution was observed at the end of the follow-up period.Conclusions Our preliminary findings suggested that Roxadustat may be effective for patients with NSAA or LR-MDS refractory to conventional therapies and rhEPO,with mild adverse effects and low relapse rate.The degree of hemoglobin improvement may be better in the refractory NSAA patients.

Background:Intestinal Beh?et's disease is a subtype of multisystem vasculitis that affects the intestine,leading to severe intestinal and extra-intestinal complications,increased hospitalization rate,and a decrease in the quality of life of patients.Aims:To analyze the clinical,endoscopic,and pathological characteristics of patients with intestinal Beh?et's disease.Methods:Twenty-five patients with intestinal Beh?et's disease admitted from April 2018 to April 2023 at Tongji Hospital,Tongji Medical College of Huazhong University of Science and Technology were enrolled.The demographic data,clinical symptoms and signs,endoscopic findings,laboratory results,and therapeutic strategies were retrospectively analyzed.Results:In 25 patients with intestinal Beh?et's disease enrolled in this study,12 were males and 13 females,with a median age of onset at 37 years old.Oral ulcer was reported in 72.0%of patients,with 60.0%presenting with oral ulcer as the initial manifestation.Before diagnosis,60.0%of patients suffered from abdominal pain,and 12.0%experienced retrosternal pain or discomfort.All patients presented with ileocecal ulcer under endoscopy,with 15 cases of typical ulcer and 10 cases of atypical ulcer.The difference in gender distribution was statistically significant between the two groups(P=0.041 4).The disease activity index for intestinal Beh?et's disease(DAIBD)in typical ulcer group was significantly higher than that in atypical ulcer group(P=0.019 4),while no significant differences in neutrophil count,hemoglobin,hypersensitive C-reactive protein,and γ-glutamyl transferase were found between the two groups(P>0.05).Five patients were accompanied with esophageal ulcer.During the course of the disease,5 patients escalated to biological therapy(adalimumab or infliximab).Four patients with typical ulcer underwent surgical treatment due to complications such as intestinal perforation,gastrointestinal bleeding,and anal fistula.Conclusions:There exists a significant association between gender,DAIBD score and typical ileocecal ulcer in patients with intestinal Beh?et's disease.

Protein corona is a protein layer that adsorbs on the surface after nonspecific interactions between nanoparticles and plasma proteins.In recent years, studies have shown that modification of specific plasma proteins on the surface of nanoparticles to construct protein corona can prolong the blood half-life of nanoparticles and promote the targeted delivery of nanoparticles, which has attracted widespread attention to the study of drug-carrying systems, among which, albumin corona, the most abundant protein in blood, is the most widely studied.Based on the above, this paper systematically summarized the method of constructing albumin corona and its application in the research on pharmaceutical preparations, in order to provide reference for the construction of albumin corona in the process of drug preparation.