[Objective]To explore the occurrence of gasdermin D(GSDMD)-mediated pyroptosis and its effect on cell proliferation,migration and tubular formation abilities of synovial vascular endothelial cells(VEC)in rheumatoid arthritis(RA).[Methods]Synovium tissues from knee joints of 22 RA patients and 18 orthopaedic arthropathies(Orth.A)patients were collected.The level of activated GSDMD-NT segment in synovium was detected by Western blot.The clinical characteristics of RA patients were compared between high and low synovial GSDMD-NT groups.The cell localization of GSDMD in RA synovium was detected by immunofluorescence staining.RA synovial fluid was added to the culture of human umbilical vein endothelial cells(HUVEC)in vitro,and the level of apoptosis and expression of pyroptosis pathway proteins were detected.The effects of GSDMD on apoptosis,proliferation,migration and tubule formation of HUVEC cells were analyzed.[Results]GSDMD expression in RA synovium was significantly higher than that in Orth.A,and more severe joint destruction and higher microvascular count score were found in RA patients with high GSDMD-NT expression.Synovial VEC had positive expression of GSDMD.Stimulation with RA synovial fluid could induce GSDMD-mediated pyroptosis in HUVEC,increased the secretion of vascular endothelial growth factor(VEGF)and their abilities of proliferation,migration and tubule formation.Knockdown of GSDMD could reverse the above effects.[Conclusion]GSDMD-mediated pyroptosis of partial synovial VEC aggravates RA joint destruction through VEGF secretion that promotes proliferation,migration and angiogenesis of the remaining VEC,which may be a new target to block neovascularization and inhibit joint destruction in RA.

Objective: To evaluate the effect of Triangle hierarchical management among community patients with hypertension, so as to provide insights into the improvements of standardized hypertension management. Methods: Patients with newly diagnosed hypertension from 2 community health service centers in Qiantang District of Hangzhou City by the end of 2020 were randomly assigned to the conventional group and the Triangle group. Patients in the conventional group were given health management services according to the requirements of basic public health service standard for one year, while patients in the Triangle group were given Triangle hierarchical management. The blood pressure, self-management behaviors, treatment compliance, smoking, alcohol consumption and exercise were collected using the self-management behavior scale, treatment compliance scale and self-designed questionnaires, and were compared before and after intervention with analysis of covariance and generalized estimation equations. Results: Totally 200 patients with hypertension were recruited, including 100 patients in the conventional group and 100 patients in the Triangle group. There were no significant differences between the two groups before implementation of interventions in terms of gender, age, educational level or occupation (P>0.05). The reduction in blood pressure, increase in the score of treatment, diet, exercise and living habitat management and the total score of self-management behaviors, increase in the score of adherence to medication regimens, daily living management behaviors, smoking and alcohol consumption preference and the total score of treatment compliance, and increase in the number of patients with normal standard of quitting smoking, quitting alcohol consumption, exercise and blood pressure were significantly higher in the Triangle group than in the conventional group after intervention (P<0.05). Conclusions Triangle hierarchical management may increase the treatment compliance, improve the self-management behavior and facilitate hypertension control among hypertensive patients, which may be popularized for health management among community patients with hypertension.

A 58-year-old male patient with angioimmunoblastic T-cell lymphoma developed a rash and skin tightness on the face, limbs, and trunk together with joint stiffness and dysfunction after 6 months of treatment with the programmed cell death protein-1 inhibitor camrelizumab. Laboratory tests revealed progressive eosinophilia over 6 months, with the eosinophil count increasing from 0.07×10 9/L to 3.3×10 9/L. Magnetic resonance imaging showed thickened skin of both forearms, while T 2-weighted imaging showed markedly increased signal intensity within the myofascia. Skin biopsy of the right forearm showed thickened and fibrosed fascia and infiltration of inflammatory cells, including lymphocytes, plasma cells, and eosinophils. The patient was diagnosed with immune checkpoint inhibitor (ICI)-induced eosinophilic fasciitis (EF). After beginning treatment with methylprednisolone (40 mg daily), methotrexate (10 mg/week), and baricitinib (4 mg daily), his symptoms of skin tightness and joint dysfunction significantly improved within 1 month, and his peripheral blood eosinophil count decreased to 0.17×10 9/L. ICI-induced EF is a rare immune-related adverse reaction. To date, only 20 cases have been reported in published foreign literature, and their clinical characteristics are summarized here. The time from ICI treatment to EF was 12 (8,15) months, and the main clinical manifestations included skin involvement ( n=19), joint dysfunction ( n=11), myalgia/muscle weakness ( n=9), and peripheral eosinophilia ( n=16). After treatment, the clinical symptoms of EF improved in 17 patients, and eosinophil counts returned to normal after 3 (1,8) months. EF is a dysfunctional adverse response to ICI therapy. Tumor patients undergoing immunotherapy should be monitored for symptoms of EF. Early treatment is essential for preventing complications.

OBJECTIVE: To explore the clinical and genetic characteristics of a Chinese pedigree affected by glycogen storage disease (GSD) type Ia with gout as the first manifestation. METHODS: Clinical and biochemical data of the pedigree were collected. Available members of the pedigree were subjected to gene sequencing, and the result was analyzed by bioinformatics software. The pedigree was followed up for five years. RESULTS: The proband was a young female manifesting recurrent gout flare, hypoglycemia, and hypertriglyceridemia. One of her younger brothers also presented with dysplasia and hepatic adenoma. Gene sequencing revealed that the proband and her younger brother both harbored c.1022T>A (p.I1e341Asn) and c.230+5G>A compound heterozygous variants of the G6PC gene , which were inherited from their father and mother, respectively. Among these, the c.230+5G>A is an intron region variant which was unreported previously, and bioinformatics analysis showed that it may impact mRNA splicing of the gene. The proband was treated with raw corn starch, allopurinol, and fenofibrate. Gout was well controlled, and she had given birth to a baby girl without GSD. CONCLUSION GSD Ia should be considered among young gout patients with hypoglycemia and hepatomegaly, for which gene sequencing is warranted. GSD Ia has a good prognosis after comprehensive treatment with diet and medicine.
China ; Female ; Glycogen Storage Disease Type I ; Gout/genetics* ; Humans ; Hypoglycemia ; Male ; Pedigree ; Symptom Flare Up

[摘 要] 目的：检测lncRNA LOC440173在NSCLC组织和细胞中的表达及探讨其对癌细胞恶性生物学行为的影响。方法：选取河北医科大学第四医院生物标本库中2014至2017年手术切除的72例NSCLC患者的癌及癌旁组织标本，应用qPCR法检测NSCLC组织和癌旁组织中，以及6种NSCLC细胞株（H520、H358、A549、HCC827、H1703和H1299）中LOC440173的表达水平；构建LOC440173的敲低及过表达载体，分别转染H520和H1703细胞，应用MTS、克隆形成及Transwell小室迁移和侵袭实验分别检测敲低及过表达LOC440173对NSCLC细胞增殖、迁移及侵袭能力的影响，qPCR法检测LOC440173对于EMT过程相关标志物（E-cadherin、N-cadherin及vimentin）mRNA表达水平的影响，WB法检测其对E-cadherin、N-cadherin蛋白表达的影响。结果：LOC440173在NSCLC组织中的表达明显高于癌旁组织（P<0.01），并与淋巴结转移、组织学分化程度、TNM分期和肿瘤大小有关联（P<0.05或P<0.01）。敲低LOC440173可以抑制H520细胞的体外增殖、迁移和侵袭（P<0.05或P<0.01），过表达LOC440173可显著促进H1703细胞的增殖、迁移和侵袭（P<0.05或P<0.01）。在转录水平上，敲低LOC440173后，E-cadherin的表达水平升高，间充质相关标志物N-cadherin、vimentin的表达水平降低（P<0.05或P<0.01）；而过表达LOC440173后，E-cadherin的表达水平降低，间充质相关标志物N-cadherin、vimentin的表达水平升高（P<0.05或P<0.01）。在转录后水平上，LOC440173负向调节E-cadherin蛋白的表达、正向调节N-cadherin的蛋白表达（均P<0.05）。结论：LOC440173在NSCLC组织中的异常高表达可能与NSCLC的发生发展有关，LOC440173可显著提高NCSCL细胞的体外增殖、迁移、侵袭能力，且其作用机制可能与调控EMT相关基因表达有关。

Objective:To improve the awareness of cholesterol crystal embolism syndrome (CCE) inrheumatologists.Methods:The clinical characteristics of 40 Chinese CCE patients admitted to our department (one case) were summarize and in the literature (thirty-nine cases) were reviewed.Results:Among these 40 patients, 87.5%(35/40) were male and the mean age was (68±6) years. All patients suffered from athero-sclerosis and 87.5%(35/40) of them had precipitating factors such as endovascular intervention, vascular surgery, anticoagulant, or thrombolytic therapy. The clinical manifestations included renal insufficiency (90.0%, 36/40), blue toe syndrome (82.5%, 33/40), ulceration or gangrene (25.0%, 10/40), and livedo reticularis (15%, 6/40). Acute phase reactant was tested in 25 cases, of whom 84.0%(21/25) showed elevated C-reactive protein (CRP) and 56.0%(14/25) showed elevated erythrocyte sedimentation rate (ESR).Conclusion:Rheumatologists should be alert that CCE is one of the differential diagnosis of systemic vasculitis, especially for patients with severe atherosclerosis.

Objective To evaluate the clinical outcomes of K-line(-) ossification of posterior longitudinal ligament (OPLL) between single open-door posterior decompression with instrumented in situ fusion(PDF) and laminoplasty (LMP).Methods From February 2008 to February 2015,38 cases including 30 males and 8 females underwent posterior decompression due to K-line(-) OPLL in our institution.The age ranged from 48 to 76 years,mean 56.3±9.7 years.The OPLL canal occupation ratio ranged from 38.6％ to 72.5％,mean 58.1％± 13.6％.According to surgical procedures,26 cases were in PDF group and 12 cases were in LMP group.The data collected from both groups included complications,C2-C7 Cobb angle and neurologic symptoms evaluated based on the Japanese Orthopedic Association (JOA) score and JOA score recovery rate,and were analyzed with statistics in and between groups.Results All the patients were completed follow up with a mean of 3.6±2.1 years (range,2.5 to 7.0 months).At the final follow-up,the postoperative JOA score was 11.8± 1.9 points on average,improved from preoperative 7.7± 1.6 points (t=3.757,P＜0.05),the mean JOA score recovery rate was 44.2±6.7％in PDF group;and the postoperative JOA score was 9.1 ±2.1 points,improved from preoperative 7.9± 1.5 points (t=1.327,P＜0.05),the mean JOA score recovery rate was 29.5±5.0％ in LMP group.No significant difference was found in preoperative JOA score between the two groups (t=0.365,P＞0.05),however,there were significant differences in the postoperative JOA score (t=3.941,P＜0.05) and JOA score recovery rate (t=6.741,P＜0.05) at the final follow-up.In PDF group,the C2-C7 Cobb angle was 4.1±2.0°,similar with preoperative 3.8±1.6° (t=0.587,P＞0.05).On the contrary,the C2-C7 Cobb angle was-2.1°±1.8°,lower than preoperative 3.9°±1.2° (t=6.824,P＜0.05) in LMP group.Ten cases occurred C5 palsy (PDF:7;LMP:3),and 3 cases occurred wound infection (PDF:2;LMP:1).The complication rate was 34.6％ and 33.3％ (x2=0.003,P＞0.05),respectively.Conclusion Compared with LMP,PDF without correcting cervical alignment for patients with K-line(-) OPLL showed better neurological recovery and clinical efficacy.

Objective: To analyze the type of malocclusion which was more likely to cause undesirable orthodontic results and the reasons for orthodontic re-treatment. Methods: In this study, 202 patients who received orthodontic retreatment in School of Stomatology, China Medical University from 2004 to 2016 were collected. Forty-seven patients were teenagers under 18 years old, and 155 patients were adults over 18 years old. The chief complaint of all patients was analyzed. Seven orthodontic specialists were invited to analyze the reasons caused the orthodontic re-treatment according to the treatment records, plaster models, photographs, panoramic radiographs and lateral cephalograms. They were offered an evaluation form to fill in by their judgement. The reasons for orthodontic re-treatment and the problems related to previous orthodontic treatment were analyzed. Results: In 202 patients, the chief complaints were protrusion of the teeth [30.2% (61/202)], protrusion of the mandible [20.8% (42/202)], and irregularity of the teeth [17.8% (36/202)]. The proportion of patients chosen undesirable treatment design was 68.3% (138/202). The proportion of patient having unusual growth tendency was 24.3% (49/202). The proportion of patients with poor compliance during treatment and retention was 24.3% (49/202). Conclusions In this study, the protrusion of the teeth, the protrusion of the mandible and the dental crowding were the main chief complaints for orthodontic re-treatment. Undesirable treatment design and unfavorable growth tendency were the main reasons for orthodontic re-treatment. Poor compliance of patients during the period of treatment or retention was an important reason for the patient to seek orthodontic re-treatment.

Objective: To analyze factors affecting stability after fixed orthodontic treatment. Methods: Five hundred and forty-four patients who had finished fixed orthodontic treatment more than two years in First Department of Orthodontics, China Medical University from January, 2000 to December, 2017 were investigated and the rate of regular revisit was counted. The data of 288 patients were successfully collected by calling or sending text messages and the rate of standard use of retainers as prescribed (patients wore retainers for 24 months or longer) was counted. According to the variation of peer assessment rating (PAR) index at the start of retention and the time the data collected, the patients were divided into relapse group (variation of PAR index >5) and non-relapse group (variation of PAR index ≤5). Difference significance analysis and multiple-factor logistic regression analysis were used. Sixty patients wearing retainers well were collected and the curative effects of Hawley retainer and vacuum formed retainer (VFR) were compared, which included overbite, overjet, maxillary irregularity index, mandibular irregularity index, width betwenn canine and width between first molar. Results: The rate of regular revisit was 41.0% (223/544). Two hundred and one of 288 patients (69.8%) who were visited successfully had regular revisit, and 60.4%(174/288) of the patients wore retainers well; 30.2% (87/288) of the patients who were visited successfully didn't have regular revisit, and 10.4% (30/288) of the patients wore retainers well. Difference significance analysis showed that there was highly significant difference between relapse group and non-relapse group in the type of retainer and duration of retention (P<0.01). One hundred and forty of 224 patients (62.5%) in non-relapse group and 37.5% (24/64) of the patients in relapse group used VFR, and the duration of retention in non-relapse group was significantly longer than that in relapse group (P<0.01). Multiple-factor Logistic regression analysis showed that wearing Hawley retainers (OR=3.067, P<0.05) was the risk factor influencing relapse. The duration of retention (OR=0.832, P<0.01) was the protective factor influencing relapse. Independent-sample t test indicated that the variations of maxillary [(0.82±0.36) mm] and mandibular [(1.05±0.22) mm] irregularity index in Hawley retainer group were larger than maxillary [(0.64±0.29) mm] and mandibular [(0.72±0.35) mm)] irregularity index in VFR group, respectively. The differences between the two groups were significant (P<0.05). Conclusions Duration of retention was implicated in stability after orthodontic treatment. VFR had better effect in the aspects of irregularity index than Hawley retainer.

Objective To evaluate the clinical outcome of anterolateral lumbar interbody fusion (ALLIF) for the treat-ment of degenerative lumbar spondylolisthesis(DLS). Methods All of 53 patients with degenerative spondylolisthesis were en-rolled in this study during January 2014 to January 2016. All patients underwent stand-alone ALLIF with self-locked cage, includ-ing 15 males and 38 females with an average of 57.4±11.2 years old. The surgical level included L3,4(4 cases), L4,5(33 cases) and L5S1(16 cases). The operative time, bleed loss and complications were recorded. Clinical outcome was measured by visual analogue scale (VAS), Oswestry disability index (ODI) and the health questionnaire (SF-36). Radiological measures were slippage, disc height, lumbar lordosis, sagittal diameter and area of dural canal, foraminal height and area, and fusion rate. All the data were sta-tistically analyzed. Results All patients were followed up for at least 24 months. The mean operation time was 64.2 ± 12.5 min-utes, and the mean bleed loss was 72.8 ± 14.3 ml. No serious complications such as vascular or nerve injury occurred during peri operation. VAS for low back pain,VAS for leg pain and ODI were significantly decreased from 6.8 ± 1.5,5.6 ± 2.4 and 58.5％± 18.6％preoperatively to 1.6±1.2, 1.2±1.1 and 18.5％±8.8％at 24 months postoperatively.The SF-36 scores were improved signif-icantly from 56.7 ± 12.8 preoperatively to 79.4 ± 14.3 at 24 months postoperatively. Slippage of patients was reduced significantly from 22.5％± 8.3％preoperatively to 5.7％± 2.3％. Disc height and lumbar lordosis were significantly increased from 6.2 ± 1.6 mm and 46.4°±9.2° preoperatively to 10.5±1.4 mm and 53.2°±11.2° at 24 months postoperatively. Sagittal diameter and cross-section-al area of thecal sac were significantly increased from 11.7±2.2 mm and 78.4±28.6 mm2 preoperatively to 13.8±1.6 mm and 112.5± 23.9 mm2 at 6 months postoperatively. Intervertebral foramina height and area were significantly increased from 15.8 ± 4.3 mm and 136.5±25.6 mm2 preoperatively to 19.2±2.5 mm and 157.6±23.1 mm2 at 6 months postoperatively. At 24 months follow-up, the fusion rate was 100％and the subsidence rate was 10.5％. Conclusion Stand-alone ALLIF could achieve satisfactory clinical re-sults and reduction for DLS. Disc height and lumbar lordosis were restored. Efficient decompression was obtained with significant-ly increased sagittal diameter and cross-sectional area of thecal sac and intervertebral foramina height and area. Stand-alone AL-LIF provides a new surgical strategy for the treatment of DLS.