OBJECTIVE: To review the research progress and challenges of poly (L-lactic acid) (PLLA) membrane in preventing tendon adhesion. METHODS: The relevant literature at home and abroad in recent years was extensively searched, covering the mechanism of tendon adhesion formation, the adaptation challenge and balancing strategy of PLLA, the physicochemical modification of PLLA anti-adhesion membrane and its application in tendon anti-adhesion. In this paper, the research progress and modification strategies of PLLA membranes were systematically reviewed from the three dimensions of tissue adaptation, mechanical adaptation, and degradation adaptation. RESULTS: The three-dimensional adaptation of PLLA membrane is optimized by combining materials (such as hydroxyapatite, polycaprolactone), structural design (multilayer/gradient membrane), and drug loading (anti-inflammatory drug). The balance between anti-adhesion and pro-healing is achieved, the mechanical adaptation significantly improve, and degradation is achieved (targeting the degradation cycle to 2-4 weeks to cover the tendon repair period). CONCLUSION In the future, it is necessary to identify the optimal balance point of three-dimensional fitness, unify the evaluation criteria and solve the degradation side effects through the co-design of physicochemical modification and drug loading system to break through the bottleneck of clinical translation.
Tissue Adhesions/prevention & control* ; Polyesters/chemistry* ; Humans ; Biocompatible Materials/chemistry* ; Tendons/surgery* ; Membranes, Artificial ; Tendon Injuries/surgery* ; Wound Healing ; Animals ; Durapatite/chemistry*

Cornelia de Lange syndrome is a rare congenital malformation disease,and its typical features include growth restriction,mental retardation,craniofacial abnormality and hirsutism.This study reported 2 cases of CdLS patients,summarized their clinical manifestations and gene mutation characteristics,and the relevant literatures were reviewed.Patient 1,a 5-year-old girl,was admitted to the hospital due to growth retardation.Physical examination revealed hirsutism,monobrow,small and sparse teeth,hemangiomas(approximately 2 cm×2 cm)on the chest and back,delayed language development,and intellectual disability.The height was 98 cm[≤-2 standard deviation(SD)],the weight was 15 kg(-2SD--1SD),the head circumference was 46 cm(-3SD--2SD).Brain magnetic resonance imaging(MRI)plain scan showed slightly enlarged left lateral ventricle and bilateral lateral ventricle triangles,slightly thickened bilateral maxillary sinus and ethmoid sinus mucosa.Echocardiography revealed mild regurgitation of the mitral and tricuspid valves.Patient 2,a 1-month-old girl,was admitted to the hospital due to postpartum shortness of breath.The physical examination highlighted hirsutism,short nose,soft cleft palate,dysphagia,positive three-concave sign,audible throat sound,small hands,palm penetration in the left hand,short fifth finger of the right hand,limited right hip abduction,foot varus,and a white spot at the bottom of the right eye.Ultrasonography at 1 month showed mild regurgitation of the tricuspid valve and an open foramen ovale.Brain MRI at 2 d showed a few patchy low-signal shadows in the longitudinal fissure cistern and tentorium,a small amount of subarachnoid hemorrhage,and a small amount of fluid in the bilateral maxillary sinus,ethmoid sinus,and middle ear mastoid.No obvious structural or numerical abnormalities were observed in the chromosome karyotypes.Whole-exome sequencing detected a heterozygous variation of c.6653_6655del in the NIPBL gene in patient 1 and a heterozygous variation of c.337C＞T in the NIPBL gene in patient 2.These variations were not found in their parents.The study revealed that NIPBL gene variation could be the genetic cause of the two patients with CdLS.The identification of the variation c.337C＞T may expand the variation spectrum of the NIPBL gene,providing valuable insights into the pathogenic genetic basis of CdLS.

Alzheimer's disease(AD)is a progressive neurodegenerative disease with hidden onset.Recent researches have shown that apolipoprotein E4(APOE4)is a high-risk genetic factor of AD,and the clinical symptoms in APOE4 genotype AD patients are closely related to metabolic disorders.This paper reviewed the role of APOE4 in the pathogenesis of AD,and it is indicated that APOE4 contributes to the injury of the cerebral blood-brain barrier,is associated with the disorder of cerebral glucose and lipid metabolism,and leads to the damage of the scavenging ability of microglia.Moreover,this paper explored the approach of early intervention of AD with traditional Chinese medicine(TCM)based on the APOE4 pathogenesis.Based on the pathogenesis of AD being deficiency in origin and excess in superficiality,and under the guidance of the principle of prevention before illness in TCM,it is proposed that early TCM intervention for APOE4 genotype AD patients with metabolic disorders can be performed through improving the phlegm-dampness constitution,by focusing on insulin resistance-related indicators,and with traditional Chinese drug therapy based on syndrome differentiation alone or together with comprehensive management of exercises,diet control and drug interventions,thus to reduce or delay the onset of APOE4 genotype AD.

Perimenopausal generalized anxiety disorder belongs to the category of"depression syndrome"in traditional Chinese medicine.It is caused by disharmony of blood vessels,stagnation of qi,and disturbance of the mind and brain,leading to excessive or insufficient qi in the five zang organs.Based on different clinical manifestations,it can be divided into five types of depression.In clinical treatment,Professor Zhuang Lixing applies the mind-regulating acupuncture therapy,with the main acupoints being Sishenzhen,Shenting(GV24),Yintang(EX-HN3),Shenmen(HT7),and Sanyinjiao(SP6).Additional acupoints can be selected according to the patient's symptoms.During the manipulation,the"guiding qi and harmonizing essence"reinforcing and reducing technique is used to regulate emotions and guide qi to relieve depression.This method has shown excellent clinical efficacy and is suitable for clinical promotion.

Referring to ZHANG Xichun's experience in treating dysentery from cold-fire accumulation， the treatment of ulcerative colitis （UC） in this paper can be divided into three stages including cold-fire accumulation stage， excessive heat and putrid intestine stage， and healthy qi deficiency and pathogen lingering stage. For people with slippery and excess pulse in the cold-fire accumulation stage， Xiaochengqi Decoction （小承气汤） added with Baishao （Radix Paeoniae Alba） and Gancao （Radix et Rhizoma Glycyrrhizae） can be used for purgation， while those with deficient pulse， Huazhi Decoction （化滞汤） or Xieli Decoction （燮理汤） can be used. In the excessive heat and putrid intestine stage， Tongbian Baitouweng Decoction （通变白头翁汤） and Jiedu Shenghua Elixir （解毒生化丹） are suggested. In the healthy qi deficiency and pathogen lingering stage， it is advised to use Jiedu Shenghua Elixir added with Shanyao （Rhizoma Dioscoreae）， and Sanbao Porridge （三宝粥）. Additionally， the medication rules， dosage and administration characteristics of Huanglian （Rhizoma Coptidis）-Rougui （Cortex Cinnamomi）， Yadanzi （Fructus Bruceae）， Diyu （Radix Sanguisorbae）， Shanyao and Liuhuang （Sulphur） by ZHANG Xichun have been summarized with the help of modern pharmacological research， so as to provide new ideas for the treatment of UC by TCM.

Background and purpose:DDX is an adenosine triphosphate(ATP)-dependent RNA helicase closely related to mRNA regulation,tumor proliferation and invasion.This article aimed to explore the effect of DDX6,a member of the DDX family,on the stability of CKMT1A mRNA,as well as the effect of the DDX6 CKMT1A axis on the proliferation and migration ability of human nasopharyngeal carcinoma cell CNE2 and its molecular mechanism.Methods:We retrieved the data of expressions of DDX6 and CKMT1A in human head and neck squamous cell carcinoma from The Cancer Genome Atlas(TCGA)database and performed a correlation analysis.Western blot was performed to detect the expressions of CKMT1A and DDX6 in human nasopharyngeal carcinoma tissues and normal nasopharyngeal tissues preserved by Affiliated Hospital of Nantong University.This study was approved by the Ethics Committee of Affiliated Hospital of Nantong University(Number:2022-L114).We used transwell assay to detect cell migration ability,EdU assay to detect cell proliferation ability,and colony formation assay to detect clone formation ability.We transfect with lentivirus and plasmids to construct sh-DDX6,sh-CKMT1A,sh-CKMT1A+sh-DDX6 and oe-CKMT1A cell models derived from the human nasopharyngeal carcinoma cell line CNE2,preserved by Affiliated Hospital of Nantong University,to clarify the impact of DDX6 and CKMT1A expression levels on the malignant biological phenotypes of nasopharyngeal carcinoma cells.BALB/c nude mice subcutaneous xenograft tumor model was constructed to detect the effects of DDX6 and CKMT1A on nasopharyngeal carcinoma cells in mice.RNA stability assay was used to detect the effect of DDX6 knockout on CKMT1A mRNA and further clarify the molecular mechanism of DDX6.Results:DDX6 was highly expressed,CKMT1A level was low in human nasopharyngeal carcinoma tissue,and DDX6 was negatively correlated with CKMT1A expression.DDX6 inhibited protein translation of CKMT1A by disrupting its mRNA stability.Low expression of CKMT1A in CNE2 cells enhanced cell migration and proliferation ability,while high expression inhibited migration and proliferation ability.Knocking out DDX6 reversed the progression of malignant behavior caused by downregulation of CKMT1A.Low expression of CKMT1A promoted tumor cell growth in BALB/c nude mice subcutaneous xenograft tumor model,while low expression of DDX6 inhibited tumor cell growth.Knocking out DDX6 and CKMT1A simultaneously restored the inhibitory effect caused by knocking down DDX6 alone.Conclusion:DDX6 in nasopharyngeal carcinoma cells disrupts the stability of CKMT1A mRNA,negatively regulates CKMT1A protein translation,upregulates the proliferation and migration ability of nasopharyngeal carcinoma cells,and promotes malignant progression of nasopharyngeal carcinoma.

Objective:To summarize the genotype and clinical characteristics of chylomicron retention disease (CMRD) caused by secretion associated Ras related GTPase 1B (SAR1B) gene variations.Methods:Clinical data and genetic testing results of 2 children with CMRD treated at Children′s Hospital of Fudan University and Jiangxi Provincial Children′s Hospital from May 2022 to July 2023 were summarized. To provide an overview of the clinical and genetic characteristics of CMRD caused by SAR1B gene variations, all of the literature was searched and reviewed from China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, China VIP database, China Biology Medicine disc and PubMed database (up to January 2024) with "chylomicron retention disease" "Anderson disease" or "Anderson syndrome" as the search terms. All relevant literatures were reviewed to summarize the clinical and genetic features of CMRD caused by SAR1B gene variations.Results:One 11-year-old boy and one 4-month-old girl with CMRD. Both patients had lipid malabsorption, failure to thrive, decreased cholesterol, elevated transaminase and creatine kinase, and Vitamin E deficiency, with homozygous variations (c.224A>G) and compound heterozygous variations (c.224A>G and c.554G>T) in SAR1B gene, respectively. Case 1 was followed up for over a month, and he still occasionally experienced lower limb muscle pain. Case 2 was followed up for more than a year, and her had caught up to normal levels. Both patients had no other significant discomfort. Literature search retrieved 0 Chinese literature and 22 English literatures. In addition to the 2 cases reported in this study, a total of 51 patients were identified as CMRD caused by SAR1B gene variations. Twenty-one types of SAR1B variants 10 missense, 4 nonsense, 3 frameshift, 1 in-frame deletion, 1 splice, 1 gross deletion, and 1 gross insertion-deletion were found among the 51 CMRD cases. Among all the patients, 49 cases had lipid malabsorption (43 cases had diarrhea or fatty diarrhea, 17 cases had vomiting, and 12 cases had abdominal distension), 45 cases had lipid soluble Vitamin deficiency (43 cases had Vitamin E deficiency, 10 cases had Vitamin A deficiency, 9 case had Vitamin D deficiency, and 5 cases had Vitamin K deficiency), 35 cases had failure to thrive, 32 cases had liver involvement (32 cases had elevated transaminases, 5 cases had fatty liver, and 3 cases had hepatomegaly), 29 cases had white small intestinal mucosa under endoscopy, and 17 cases had elevated creatine kinase, 14 cases had neuropathy, 5 cases had ocular lesions, 2 cases had acanthocytosis, 1 case had decreased cardiac ejection fraction, and 1 case was symptom-free.Conclusions:Early infancy failure to thrive and lipid malabsorption are common issues for CMRD patients. The laboratory tests are characterized by hypocholesterolemia with or without fat-soluble Vitamin deficiency, elevated liver enzymes and (or) creatine kinase. Currently, missense variations are frequent among the primarily homozygous SAR1B genotypes that have been described.

Objective To analyze the relationship between the relevant imaging signs of the Bosniak classification system version 2019 and the benign or malignant of cystic renal masses(CRMs).Methods The CRMs were graded using the Bosniak classification system version 2019,and the overall inter-observer agreement,and the agreement between different subgroups were compared.In addition,the diagnostic value of benign and malignant lesions with weighted weakened imaging signs,redefined and quantified imaging signs in the Bosniak classification system after upgrading from version 2005 to version 2019 was analyzed.Results Fifty-six CRMs were enrolled,including 43 benign and 13 malignant lesions.Based on the Bosniak classification system version 2019,the overall inter-observer agreement was excellent(Kappa=0.881),while the agreement of ≤30 mm and MRI were moderate(Kappa=0.548,0.722).In the comparison of the differences between benign and malignant CRMs,there were significant differences between the groups in age,lesion maximum diameter,thick septa or cyst wall,number of septa ≥4,mural nodule,and enhancement(P＜0.05).In the logistic regression model,the mural nodule was the only imaging sign that was significantly correlated with the malignant CRMs[odds ratio(OR)=9.842,P=0.022].Conclusion The Bosniak classification system version 2019 has an excellent overall inter-observer agreement.When the lesions are small,MRI can provide more imaging details.The mural nodule is an independent risk factor for malignant CRMs.

Objective:The aim of noise quality management for ICU patients was to explore the clinical feasibility of noise quality management.Methods:A randomized controlled trial method and convenient sampling method were used to select 240 patients treated in the ICU of Shanghai Fengxian District Central Hospital from April 2021 to March 2023 as the study objects. According to the time of admission, 120 patients admitted from April 2021 to March 2022 were divided into the control group. A total of 120 patients admitted to hospital from April 2022 to March 2023 were included in the intervention group. The control group was given routine care, and noise quality management was implemented in the intervention group on the basis of routine care. The noise decibel value, sleep quality, incidence of delirium and patient satisfaction of the two groups were compared.Results:In the intervention group, there were 69 males and 51 females, aged (56.08 ± 5.74) years old. The control group included 68 males and 52 females, aged (56.11 ± 5.72) years old. The decibels of day and night in ICU of the intervention group were (42.62 ± 1.33) and (38.72 ± 1.28) dB, which were lower than those of the control group (67.49 ± 2.36) and (59.65 ± 2.37) dB, with statistically significant differences ( t=100.57, 85.12, both P<0.05). Total score of sleep quality of patients in the intervention group (78.40 ± 5.86) was higher than that of the control group (60.49 ± 6.25), with statistically significant differences ( t=24.32, P<0.05). The incidence of delirium in the intervention group 12.50%(15/120), was lower than that in the control group 26.67%(32/120) with a statistically significant difference ( χ2=7.65, P<0.05). Satisfaction of patients in the intervention group 97.50%(117/120) was higher than that in the control group 90.00%(108/120), with a statistically significant difference ( χ2=5.76, P<0.05). Conclusions:The application of noise quality management for ICU patients is conducive to improving the overall environment of the ward, improving the sleep quality of patients, reducing the occurrence of patients′ delirium and improving patient satisfaction.

Objective:To investigate the genetic, clinical, and post-treatment characteristics of patients with glycogen storage disease type Ⅲ (GSD Ⅲ).Methods:A retrospective cohort analysis was performed on the genetic and clinical data of 26 cases with GSD Ⅲ who visited the Children's Hospital affiliated with Fudan University from June 2017 to December 2023. The patients were divided into non-missense variation and missense variation groups according to the types of mutation in the AGL gene.The correlation between genotype and phenotype was analyzed. All patients were treated with uncooked cornstarch after diagnosis. The changes before and after treatment were compared in patients who underwent more than twelve months of follow-up. A P value of <0.05 was used to denote statistical significance. Results:Among the 26 cases enrolled, 13 were female and 13 were male, and the median age of diagnosis was 28 (6 to 134) months. A total of thirty-five different types of AGL gene variation were detected, with c.1735+1G＞T (9/52, 17.3%) as the hotspot variation. The common clinical manifestations were elevated aminotransferases (26/26, 100%), hepatomegaly (25/26, 96.2%), fasting hypoglycemia (25/26, 96.2%), hyperketonemia (16/18, 88.9%), hypertriglyceridemia (TG) (20/26, 76.9%), elevated CK (16/25, 64.0%), and an abnormal electrocardiogram (12/16, 75.0%). Four cases (15.4%) had symptoms of myopathy at diagnosis. Liver biopsy was performed in eighteen cases, among whom 83.3% (15/18) had liver fibrosis≥S2. The number of cases with elevated levels of CK ( P＝0.031) and ALT ( P＝0.038)was pronounced in the non-missense variation group compared to that in the missense variation group. There were no statistically significant differences in age, height, liver size, degree of fibrosis, fasting blood glucose (Glu) and TG ( P>0.05). The median follow-up time of 14 cases was 40.5 (20-73) months, with improvement in body stature, reduced liver size, decreased ALT and TG, and improved Glu. However, four (28.6%) cases had new myopathy symptoms with raised CK ( P<0.05) and with advancing age, increased ALT diminished while CK level elevated ( P<0.05). Conclusions:The common clinical manifestations at the early stage of the GSD Ⅲdiagnosis are elevated aminotransferases, hepatomegaly, fasting hypoglycemia, hyperketonemia, high triglycerides, elevated CK, and fibrotic liver in China. Myopathy symptoms may arise following uncooked cornstarch treatment; however, there is significant improvement in height, liver-related, and metabolic parameters.