1.Research Progress in VEXAS Syndrome
Xianghong JIN ; Jin XU ; Miao CHEN ; Junling ZHUANG ; Min SHEN
JOURNAL OF RARE DISEASES 2026;5(1):82-89
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset, X-linked clonal autoinflammatory disease caused by somatic mutations in the
2.A Case of Multidisciplinary Treatment for Deficiency of Adenosine Deaminase 2
Jingyuan ZHANG ; Xiaoqi WU ; Jiayuan DAI ; Xianghong JIN ; Yuze CAO ; Rui LUO ; Hanlin ZHANG ; Tiekuan DU ; Xiaotian CHU ; Peipei CHEN ; Hao QIAN ; Pengguang YAN ; Jin XU ; Min SHEN
JOURNAL OF RARE DISEASES 2025;4(3):316-324
This case report presents a 16-year-old male patient with deficiency of adenosine deaminase 2(DADA2). The patient had a history of Raynaud′s phenomenon with digital ulcers since childhood. As the disease progressed, the patient developed retinal vasculitis, intracranial hemorrhage, skin necrosis, severe malnutrition, refractory hypertension, and gastrointestinal bleeding. Genetic testing revealed compound heterozygous mutations in the
3.Effect of different intensity neuromuscular training on muscle strength and knee joint function of patients after anterior cruciate ligament reconstruction
Juan WANG ; Qing ZHANG ; Changlin ZHOU ; Changyun CHEN ; Feng DAI ; Xianghong SUN ; Ting ZOU ; Jian WANG ; Junkai GAO ; Weidong XU
Chinese Journal of Rehabilitation Theory and Practice 2025;31(9):1083-1091
Objective To compare the effect of different intensity of neuromuscular training(NMT)on muscle strength and knee joint function of patients after anterior cruciate ligament reconstruction(ACLR).Methods From January,2023 to January,2024,60 ACLR patients in Changhai Hospital were selected,and they received the same intensity of NMT from one to eight weeks after surgery.Eight weeks after surgery,they were randomly divided into low intensity group(n=30)and high intensity group(n=30),and then they received different inten-sities of NMT from nine to 16 weeks after surgery,each training session lasted one hour,with three sessions per week,totaly 48 sessions.The Lysholm score,knee flexor and extensor muscle strength and muscle endurance-were compared at eight weeks and 16 weeks after surgery.Results After group training,the Lysholm score significantly increased in both groups(|t|>13.739,P<0.001),and was higher in the high intensity group than in the low intensity group(t=-2.574,P<0.05);in the high intensity group,the relative peak torque and endurance of the extensor and flexor muscles improved at angular velocities of 60°/s,120°/s and 180 °/s(|t|>2.320,P<0.05);in the low intensity group,the flexor peak torque improved at all the three angular velocities(t>2.177,P<0.05),the extensor peak torque improved at angular velocities of 60°/s and 180°/s(|t|>1.715,P<0.05),and the extensor endurance improved at angular velocity of 60°/s(t=-2.293,P<0.05).However,there was no significant difference in the relative peak torque and endurance of the extensor and flexor muscles at all the three angular velocities(P>0.05).Conclusion Both high and low intensity NMT could improve the muscle strength,muscle endurance and knee joint func-tion.Maybe,high intensity is superior to low intensity.Further verification is still needed.
4.Research progress of burden in family caregivers of chronic critical illness survivors
Xingyun SHEN ; Xianghong SUN ; Xu WEN ; Chenjing WANG
Chinese Journal of Practical Nursing 2025;41(16):1275-1281
This article reviewed the concept, current status influencing factors and interventions of burden among family caregivers of chronic critical illness survivors, aiming to provide theoretical basis and practical guidance for the research on the burden of family caregivers of chronic critical illness survivors, effectively reduce the burden of family caregivers of chronic critical illness survivors, promote the recovery of chronic critical illness survivors, and improve the quality of life.
5.Effect of different intensity neuromuscular training on muscle strength and knee joint function of patients after anterior cruciate ligament reconstruction
Juan WANG ; Qing ZHANG ; Changlin ZHOU ; Changyun CHEN ; Feng DAI ; Xianghong SUN ; Ting ZOU ; Jian WANG ; Junkai GAO ; Weidong XU
Chinese Journal of Rehabilitation Theory and Practice 2025;31(9):1083-1091
Objective To compare the effect of different intensity of neuromuscular training(NMT)on muscle strength and knee joint function of patients after anterior cruciate ligament reconstruction(ACLR).Methods From January,2023 to January,2024,60 ACLR patients in Changhai Hospital were selected,and they received the same intensity of NMT from one to eight weeks after surgery.Eight weeks after surgery,they were randomly divided into low intensity group(n=30)and high intensity group(n=30),and then they received different inten-sities of NMT from nine to 16 weeks after surgery,each training session lasted one hour,with three sessions per week,totaly 48 sessions.The Lysholm score,knee flexor and extensor muscle strength and muscle endurance-were compared at eight weeks and 16 weeks after surgery.Results After group training,the Lysholm score significantly increased in both groups(|t|>13.739,P<0.001),and was higher in the high intensity group than in the low intensity group(t=-2.574,P<0.05);in the high intensity group,the relative peak torque and endurance of the extensor and flexor muscles improved at angular velocities of 60°/s,120°/s and 180 °/s(|t|>2.320,P<0.05);in the low intensity group,the flexor peak torque improved at all the three angular velocities(t>2.177,P<0.05),the extensor peak torque improved at angular velocities of 60°/s and 180°/s(|t|>1.715,P<0.05),and the extensor endurance improved at angular velocity of 60°/s(t=-2.293,P<0.05).However,there was no significant difference in the relative peak torque and endurance of the extensor and flexor muscles at all the three angular velocities(P>0.05).Conclusion Both high and low intensity NMT could improve the muscle strength,muscle endurance and knee joint func-tion.Maybe,high intensity is superior to low intensity.Further verification is still needed.
6.Research progress of burden in family caregivers of chronic critical illness survivors
Xingyun SHEN ; Xianghong SUN ; Xu WEN ; Chenjing WANG
Chinese Journal of Practical Nursing 2025;41(16):1275-1281
This article reviewed the concept, current status influencing factors and interventions of burden among family caregivers of chronic critical illness survivors, aiming to provide theoretical basis and practical guidance for the research on the burden of family caregivers of chronic critical illness survivors, effectively reduce the burden of family caregivers of chronic critical illness survivors, promote the recovery of chronic critical illness survivors, and improve the quality of life.
7.Advancements in the investigation of cardiovascular risk associated with idiopathic inflammatory myopa-thy
Xianghong CHEN ; Xiumin CHEN ; Yingyan ZHOU ; Li LI ; Zhenxiong XU
The Journal of Practical Medicine 2024;40(19):2801-2805
The idiopathic inflammatory myopathies encompass a diverse array of autoimmune diseases,characterized by muscular inflammation and various extramuscular manifestations.These conditions have the poten-tial to impact multiple organs,including the lungs,skin,joints,gastrointestinal tract,and heart.The defining features of these conditions are muscle weakness and myalgia.Although cardiac involvement is infrequent,its clinical manifestations are subtle and easily overlooked.Cardiac damage represents a significant contributor to mortality and morbidity in patients with idiopathic inflammatory myopathy.Early and accurate identification of cardiac involve-ment may facilitate improved patient outcomes.This article provides an overview of the potential etiology,clinical presentations,risk factors,biomarkers,and imaging studies for early diagnosis of cardiac involvement in idio-pathic inflammatory myopathy.This review aims to enhance clinicians'understanding and diagnostic capabilities re-garding cardiac involvement in idiopathic inflammatory myopathy while promoting early intervention strategies for lifelong management and improved prognosis.
8.A Preliminary Analysis of the Efficacy and Prescription of Electro-acupuncture for Sepsis Treatment
Mengyue FANG ; Man LI ; Chennan LI ; Xianghong JING ; Bin XU ; Yan MA ; Noiprasert SULUKKANA ; Lingling YU
World Science and Technology-Modernization of Traditional Chinese Medicine 2024;26(7):1742-1748
A clinically serious illness with a high death and morbidity rate is sepsis.Electro-acupuncture is a significant adjuvant treatment for sepsis,lowering the inflammatory response to sepsis,regulating the patient's immunological function,and preventing multi-system organ damage.However,no comprehensive study has been published.In order to review the clinical application of electro-acupuncture in the field of sepsis and its complications,analyze its clinical efficacy,and summarize effective acupuncture point prescriptions,we systematically compiled the clinical research literature on electro-acupuncture in the treatment of sepsis and its complications by searching PubMed,CNKI,and other databases.It was found that effective clinical evidence had been gathered for the administration of electro-acupuncture in uncomplicated sepsis as well as in sepsis aggravated by gastrointestinal,pulmonary,cardiac,and brain injury.The following successful acupoint prescriptions were indicated:①Simple sepsis was treated with"Zusanli+Guanyuan"or"Zusanli+Tianshu+Shangjuxu+Xiajuxu";②Combined gastrointestinal injury was treated with"Zusanli+Shangjuxu+Tianshu"or"Zusanli+Tianshu+Shangjuxu+Xiajuxu";③ Combined lung injury was treated with"Zusanli+Chize";④Combined myocardial injury was treated with"Neiguan+Zusanli+Shenmen+Taichong+Xuehai+Geshu+Sanyinjiao+Hegu";⑤Combined brain injury was treated with"Baihui+Shuigou".The study's findings can offer preliminary clinical direction for using electro-acupuncture to treat sepsis and associated complications in patients.
9.Application of serum SAA, CRP, PCT, WBC and N% in the diagnosis of neonatal septicemia
Qin YANG ; Jia FANG ; Ying XU ; Yan YANG ; Xianghong YAN
Chinese Journal of Preventive Medicine 2024;58(10):1587-1592
To explore the application value of SAA (serum amyloid A), CRP (C reactive protein), PCT (procalcitonin), WBC (white blood cell) and N% (neutrophil %) in the diagnosis of neonatal septicemia. This study was a retrospective study. 173 children with clinically diagnosed septicemia and 66 children with definitely diagnosed septicemia admitted to the Department of Neonatology, the Affiliated Changsha Central Hospital, Hengyang Medical School, University of South China,from January 2022 to January 2024 were selected as the case group, and 148 children with neonatal jaundice who were hospitalized during the same period were selected as the control group. Fasting venous blood was collected within 24 hours after the children′s admission to detect the levels of serum WBC, N%, SAA, CRP and PCT. One-way analysis of variance and Kruskal-Wallis H test were used to compare the general data and inflammatory index levels of the three groups of children. The correlation analysis between SAA and other inflammatory indicators was conducted using Spearman correlation analysis. The receiver operating characteristic (ROC) curve was used to determine the diagnostic efficacy of different inflammatory indicators for patients with definitely diagnosed septicemia and those with clinically diagnosed septicemia, and for those with clinically diagnosed septicemia and those without infection. The results showed that the levels of WBC [(16.88±5.64)×10 9/L], N% [70.00 (63.00, 75.00)], PCT [2.22 (1.20, 5.55) mg/L], CRP [3.00 (0.50, 10.30) mg/L], SAA [19.70 (10.82, 49.90) mg/L] in the clinically diagnosed septicemia group and WBC [(16.10±7.48)×10 9/L], N% [73.50 (61.50, 80.93)], PCT [5.35 (0.69, 20.07) mg/L], CRP [15.52 (4.98, 30.50) mg/L], SAA [43.95 (14.00, 175.98) mg/L] in the definitely diagnosed septicemia group were all higher than those in the control group (11.17±3.38)×10 9/L, 49.81 (36.93, 62.75), 0.20 (0.07, 0.99) mg/L, 0.54 (0.20, 1.40) mg/L, 5.15 (3.60, 8.68) mg/L, and the differences were all statistically significant (all P<0.05). Spearman correlation analysis showed that the level of SAA was positively correlated with WBC, N%, PCT and CRP ( rs=0.453, 0.540, 0.343, 0.550, all P<0.05). ROC curve analysis showed that the area under ROC curve(AUC) of SAA for the definitely diagnosed septicemia group and the clinically diagnosed septicemia group was higher than that of other inflammatory indicators, among them, the AUC of SAA for diagnosing the definitely diagnosed neonatal septicemia group was 0.933 (95% CI: 0.809-1.000, P<0.05), with a sensitivity of 92.90% and a specificity of 99.30%. The AUC of SAA for diagnosing the clinically diagnosed septicemia group was 0.861 (95% CI: 0.818-0.904, P<0.05), with a sensitivity of 83.20% and a specificity of 81.80%. In conclusion, compared with CRP, PCT, WBC and N%, SAA has higher sensitivity and specificity for distinguishing neonatal septicemia (including definitely diagnosed septicemia and clinically diagnosed septicemia), and has certain auxiliary diagnostic value for neonatal septicemia.
10.Application of serum SAA, CRP, PCT, WBC and N% in the diagnosis of neonatal septicemia
Qin YANG ; Jia FANG ; Ying XU ; Yan YANG ; Xianghong YAN
Chinese Journal of Preventive Medicine 2024;58(10):1587-1592
To explore the application value of SAA (serum amyloid A), CRP (C reactive protein), PCT (procalcitonin), WBC (white blood cell) and N% (neutrophil %) in the diagnosis of neonatal septicemia. This study was a retrospective study. 173 children with clinically diagnosed septicemia and 66 children with definitely diagnosed septicemia admitted to the Department of Neonatology, the Affiliated Changsha Central Hospital, Hengyang Medical School, University of South China,from January 2022 to January 2024 were selected as the case group, and 148 children with neonatal jaundice who were hospitalized during the same period were selected as the control group. Fasting venous blood was collected within 24 hours after the children′s admission to detect the levels of serum WBC, N%, SAA, CRP and PCT. One-way analysis of variance and Kruskal-Wallis H test were used to compare the general data and inflammatory index levels of the three groups of children. The correlation analysis between SAA and other inflammatory indicators was conducted using Spearman correlation analysis. The receiver operating characteristic (ROC) curve was used to determine the diagnostic efficacy of different inflammatory indicators for patients with definitely diagnosed septicemia and those with clinically diagnosed septicemia, and for those with clinically diagnosed septicemia and those without infection. The results showed that the levels of WBC [(16.88±5.64)×10 9/L], N% [70.00 (63.00, 75.00)], PCT [2.22 (1.20, 5.55) mg/L], CRP [3.00 (0.50, 10.30) mg/L], SAA [19.70 (10.82, 49.90) mg/L] in the clinically diagnosed septicemia group and WBC [(16.10±7.48)×10 9/L], N% [73.50 (61.50, 80.93)], PCT [5.35 (0.69, 20.07) mg/L], CRP [15.52 (4.98, 30.50) mg/L], SAA [43.95 (14.00, 175.98) mg/L] in the definitely diagnosed septicemia group were all higher than those in the control group (11.17±3.38)×10 9/L, 49.81 (36.93, 62.75), 0.20 (0.07, 0.99) mg/L, 0.54 (0.20, 1.40) mg/L, 5.15 (3.60, 8.68) mg/L, and the differences were all statistically significant (all P<0.05). Spearman correlation analysis showed that the level of SAA was positively correlated with WBC, N%, PCT and CRP ( rs=0.453, 0.540, 0.343, 0.550, all P<0.05). ROC curve analysis showed that the area under ROC curve(AUC) of SAA for the definitely diagnosed septicemia group and the clinically diagnosed septicemia group was higher than that of other inflammatory indicators, among them, the AUC of SAA for diagnosing the definitely diagnosed neonatal septicemia group was 0.933 (95% CI: 0.809-1.000, P<0.05), with a sensitivity of 92.90% and a specificity of 99.30%. The AUC of SAA for diagnosing the clinically diagnosed septicemia group was 0.861 (95% CI: 0.818-0.904, P<0.05), with a sensitivity of 83.20% and a specificity of 81.80%. In conclusion, compared with CRP, PCT, WBC and N%, SAA has higher sensitivity and specificity for distinguishing neonatal septicemia (including definitely diagnosed septicemia and clinically diagnosed septicemia), and has certain auxiliary diagnostic value for neonatal septicemia.

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