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[Research progress on clinical characteristics of adult anti-nuclear matrix protein-2 antibody positive dermatomyositis]. [成人抗核基质蛋白-2 抗体阳性皮肌炎临床特征的研究进展]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240328-00201
C Y Li, S S Li
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引用次数: 0
[Percutaneous transhepatic papillary balloon dilation combined with dual-frequency double-pulse laser lithotripsy for large-diameter common bile duct stones]. [经皮经肝乳头球囊扩张术联合双频双脉冲激光碎石术治疗大直径胆总管结石]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20231201-00358
Y R Sun, T Jiang, H G Shang, G Chen, W Wang, Y Z Wang, Y L Li, W J Wang

Objective: To evaluate the safety and efficacy of percutaneous transhepatic papillary balloon dilation (PTPBD) combined with flexible ureteroscopy-guided dual-frequency double-pulse ND:YAG (FREDDY) laser lithotripsy (PTPBD-FREDDY) for the treatment of giant (>1.5 cm diameter) common bile duct stones. Methods: A retrospective analysis was conducted on 26 patients with large-diameter difficult choledocholithiasis admitted to two medical centers from December 2017 to October 2021. Among these patients, four could not tolerate surgery or endoscopic treatment, six experienced failure of endoscopic treatment, and 16 refused to undergo endoscopic or surgical treatment. All patients underwent the PTPBD-FREDDY procedure. The FREDDY laser lithotripsy was performed under ureteroscopic guidance, followed by a balloon to push the stones into the duodenum. The primary endpoint was the technical success rate, and the secondary endpoints included the rate of stone recurrence and related complications. Results: All 26 patients successfully completed the operation, achieving a technical success rate of 100%. The average lithotripsy frequency and operation time for bilirubin stones were significantly higher than those of mixed stones and cholesterol stones (P<0.01). The main postoperative complications included mild fever (n=3), abdominal pain (n=3), nausea (n=2) and vomiting (n=1). One patient experienced biliary tract bleeding, which improved after conservative treatment. No serious complications such as pancreatitis, sepsis, or biliary perforation were observed. After 2 years of follow-up, no cases of stone recurrence were observed. Conclusions: PTPBD-FREDDY is a safe and effective treatment for patients with giant common bile duct stones. It provides a new therapeutic option for patients with giant choledocholithiasis who can not tolerate surgery or have failed endoscopic treatment, demonstrating promising prospects.

目的评估经皮经肝乳头球囊扩张术(PTPBD)联合柔性输尿管镜引导的双频双脉冲 ND:YAG (FREDDY) 激光碎石术(PTPBD-FREDDY)治疗巨大(直径大于 1.5 厘米)胆总管结石的安全性和有效性。方法:对2017年12月至2021年10月两个医疗中心收治的26例大直径疑难胆总管结石患者进行回顾性分析。在这些患者中,4 人无法耐受手术或内镜治疗,6 人经历了内镜治疗失败,16 人拒绝接受内镜或手术治疗。所有患者都接受了 PTPBD-FREDDY 手术。FREDDY 激光碎石术是在输尿管镜引导下进行的,然后用气球将结石推入十二指肠。主要终点是技术成功率,次要终点包括结石复发率和相关并发症。结果:所有26名患者都成功完成了手术,技术成功率为100%。胆红素结石的平均碎石次数和手术时间明显高于混合结石和胆固醇结石(Pn=3)、腹痛(n=3)、恶心(n=2)和呕吐(n=1)。一名患者出现胆道出血,经保守治疗后好转。未发现胰腺炎、败血症或胆道穿孔等严重并发症。随访两年后,未发现结石复发病例。结论PTPBD-FREDDY 是治疗巨大胆总管结石患者的一种安全有效的方法。它为不能耐受手术或内镜治疗失败的巨大胆总管结石患者提供了一种新的治疗选择,前景广阔。
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引用次数: 0
[Gastrointestinal involvement in Ehlers-Danlos syndrome: A case series and systematic review]. [埃勒斯-丹洛斯综合征的胃肠道受累:病例系列和系统回顾]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240328-00203
Y P Shao, S Q Pan, Y Li, J Li, X Q Li

Objective: To explore the clinical and genetic characteristics of patients with Ehlers-Danlos syndrome (EDS) and gastrointestinal involvement. Methods: We retrospectively collected the clinical data of patients with EDS and gastrointestinal involvement from the electronic medical records at Peking Union Medical College Hospital (PUMCH) from January 2003 to September 2023. Additionally, we conducted a systematic review by searching cases with EDS and gastrointestinal involvement in PubMed, Embase, Web of Science, and the Cochrane Library databases from January 2000 to September 2023. Results: Ninety-four patients with EDS and gastrointestinal involvement were retrieved, including five patients from PUMCH and 89 patients from 80 published articles. The average age of patients was (29±14) years. The most common manifestation of gastrointestinal involvement was gastrointestinal perforation (n=46, 48.9%), followed by functional gastrointestinal symptoms (n=33, 35.1%), and digestive arterial disorders (n=10, 10.6%). The most common clinical subtype was vascular-EDS (vEDS) (n=50, 53.2%) followed by hypermobile-EDS (hEDS) (n=20, 21.3%). The most frequent genetic mutation occurred in the COL3A1 gene (n=30, 31.9%). Among patients with vEDS, gastrointestinal manifestations included gastrointestinal perforation (n=33, 66.0%), arterial lesions (n=9, 18.0%), and functional gastrointestinal symptoms (n=7, 14.0%). Among patients with hEDS, gastrointestinal manifestations included functional gastrointestinal symptoms (n=18, 90.0%), visceral prolapse (n=3, 15.0%) and intestinal volvulus (n=1, 5.0%). Conclusions: The most common subtypes of gastrointestinal involvement in EDS were vEDS and hEDS. Patients with hEDS mainly presented with functional gastrointestinal symptoms, whereas those with vEDS primarily showed gastrointestinal perforation and digestive arterial disorders.

研究目的探讨埃勒斯-丹洛斯综合征(EDS)和胃肠道受累患者的临床和遗传特征。方法回顾性收集北京协和医院(PUMCH)2003 年 1 月至 2023 年 9 月电子病历中 EDS 和胃肠受累患者的临床资料。此外,我们还通过在 PubMed、Embase、Web of Science 和 Cochrane Library 数据库中检索 2000 年 1 月至 2023 年 9 月期间 EDS 和胃肠道受累病例进行了系统性回顾。结果共检索到94例EDS合并胃肠道受累的患者,其中5例来自PUMCH,89例来自80篇已发表的文章。患者的平均年龄为(29±14)岁。胃肠道受累最常见的表现是胃肠道穿孔(46人,占48.9%),其次是功能性胃肠道症状(33人,占35.1%)和消化道动脉紊乱(10人,占10.6%)。最常见的临床亚型是血管性 EDS(vEDS)(50 人,占 53.2%),其次是高移动性 EDS(hEDS)(20 人,占 21.3%)。最常见的基因突变发生在COL3A1基因上(30人,占31.9%)。在vEDS患者中,胃肠道表现包括胃肠道穿孔(33人,66.0%)、动脉病变(9人,18.0%)和功能性胃肠道症状(7人,14.0%)。在 hEDS 患者中,胃肠道表现包括功能性胃肠道症状(18 人,占 90.0%)、内脏脱垂(3 人,占 15.0%)和肠套叠(1 人,占 5.0%)。结论EDS最常见的胃肠道受累亚型是vEDS和hEDS。hEDS患者主要表现为功能性胃肠道症状,而vEDS患者主要表现为胃肠道穿孔和消化道动脉紊乱。
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引用次数: 0
[Advances in diagnosis and subtyping of Gaucher disease & assessment and monitoring of neurological symptoms]. [戈谢病的诊断和亚型分类以及神经症状的评估和监测方面的进展]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240523-00339
X L Zhang, W H Yan, H Liu, R J Zhang
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引用次数: 0
[Expert consensus on the clinical application of bifidobacterium tetravaccine tablets for digestive diseases]. [消化系统疾病双歧杆菌四联活菌片临床应用专家共识]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240229-00135

Based on the fact that the human gut microbiota dysbiosis can cause various diseases, probiotics have a regulatory effect on human microecological imbalance and can be used to alleviate symptoms of some diseases and/or preventing and treating certain diseases. Probiotics have been widely applied in digestive diseases in both children and adults. Bifidobacterium tetravaccine tablets as a probiotic product contains Bifidobacterium infantis (CGMCC 0460.1), Lactobacillus acidophilus (CGMCC 0460.2), Enterococcus faecalis (CGMCC 0460.3), and Bacillus cereus (CGMCC 0460.4). By combining the unique characteristics of different strains, it exerts therapeutic effects through enhancing intestinal colonization resistance, improving intestinal mucosal barrier function, and modulating immune function, thereby maintaining gut and overall health. Bifidobacterium tetravaccine tablets have been widely used for digestive diseases, with a large number of fundamental and clinical research findings accumulated in the past years, a unified clinical guideline has yet to be achieved. To standardize and guide the rational clinical use of bifidobacterium tetravaccine tablets, this expert consensus have been formulated based on the summary and update of evidence-based medical data, providing a reference for its clinical application.

基于人体肠道微生物群失调可导致各种疾病的事实,益生菌对人体微生态失衡具有调节作用,可用于缓解某些疾病的症状和/或预防和治疗某些疾病。益生菌已被广泛应用于儿童和成人的消化系统疾病。双歧杆菌四联活菌片作为一种益生菌产品,含有婴儿双歧杆菌(CGMCC 0460.1)、嗜酸乳杆菌(CGMCC 0460.2)、粪肠球菌(CGMCC 0460.3)和蜡样芽孢杆菌(CGMCC 0460.4)。它结合了不同菌株的独特特性,通过增强肠道定植抵抗力、改善肠道粘膜屏障功能和调节免疫功能发挥治疗效果,从而维护肠道和整体健康。双歧杆菌四联活菌片已被广泛应用于消化系统疾病的治疗,多年来积累了大量的基础和临床研究成果,但尚未形成统一的临床指南。为规范和指导临床合理使用双歧杆菌四联活菌片,在总结和更新循证医学资料的基础上,制定了本专家共识,为其临床应用提供参考。
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引用次数: 0
[A case of subacute phosphate nephropathy caused by oral sodium phosphate]. [一例由口服磷酸钠引起的亚急性磷酸盐肾病]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240412-00237
B F Su, S S Lin, Y Liang, B Chen, C S Chen
{"title":"[A case of subacute phosphate nephropathy caused by oral sodium phosphate].","authors":"B F Su, S S Lin, Y Liang, B Chen, C S Chen","doi":"10.3760/cma.j.cn112138-20240412-00237","DOIUrl":"10.3760/cma.j.cn112138-20240412-00237","url":null,"abstract":"","PeriodicalId":68309,"journal":{"name":"中华内科杂志","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142395629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Research progress on the pathogenesis of acquired aplastic anemia]. [获得性再生障碍性贫血发病机制研究进展]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240411-00233
H Yu, Y Y Lei, S X Ding, R Fu
{"title":"[Research progress on the pathogenesis of acquired aplastic anemia].","authors":"H Yu, Y Y Lei, S X Ding, R Fu","doi":"10.3760/cma.j.cn112138-20240411-00233","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20240411-00233","url":null,"abstract":"","PeriodicalId":68309,"journal":{"name":"中华内科杂志","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142395643","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Report of six cases with mast cell leukemia and a literature review]. [六例肥大细胞白血病病例报告及文献综述]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240401-00209
F Zhu, Y Yu, C Y Chen, W B Duan, Q Jiang, R Yan, Y Sun, Y Q Han, J Zhang, H Wang, Q R Zhang, S N Chen, W H Yan, M J Cai, Z B Zhang, J Yin, Q Wang

From October 2021 to February 2023, we retrospectively analyzed the clinical and laboratory data of six patients (three male and three female, median age: 54 years, age range: 29-73 years) with mast cell leukemia (MCL) diagnosed in the First Affiliated Hospital of Soochow University (The Mastocytosis Collaborative Network of China). All patients had acute MCL, with at least one C-finding present. The main clinical presentations were hypoalbuminemia (n=4), fatigue (n=3), fever (n=2), abdominal discomfort (n=2), osteolytic lesions (n=2), dizziness (n=1), skin flushing (n=1), and weight loss (n=1). Splenomegaly and lymphadenopathy were noted in six and three patients, respectively. Six patients were strongly positive for CD117, five were positive for CD30 and CD25, and four were positive for CD2. Four patients had a normal karyotype and two patients had an abnormal karyotype. Gene mutations were detected in 4/6 cases. The median serum tryptase level was 24.9 (range: 20.1-171.9) μg/L. Two patients were treated with venetoclax and azacitidine for induction (one patient achieved partial remission by combination with afatinib, while there was no remission after combination with dasatinib in the other patient). Two patients did not achieve complete remission despite treatment with cladribine and imatinib, respectively. One patient treated with interferon combined with glucocorticoids was lost to follow-up, and one patient abandoned treatment. The follow-up time ranged from 1.1 to 21.7 months. Three patients died and two survived. Overall, MCL is a rare subtype of systemic mastocytosis with heterogeneous clinical course, and these patients have poor outcome. A better understanding of the clinical characteristics, treatment, and prognosis of MCL is urgently needed.

2021年10月至2023年2月,我们回顾性分析了苏州大学附属第一医院(中国肥大细胞白血病协作网)确诊的6例肥大细胞白血病(MCL)患者(3男3女,中位年龄:54岁,年龄范围:29-73岁)的临床和实验室数据。所有患者均为急性肥大细胞白血病,至少有一项C-发现。主要临床表现为低白蛋白血症(4 例)、乏力(3 例)、发热(2 例)、腹部不适(2 例)、溶骨性病变(2 例)、头晕(1 例)、皮肤潮红(1 例)和体重减轻(1 例)。分别有 6 名和 3 名患者出现脾肿大和淋巴结病。六名患者 CD117 强阳性,五名患者 CD30 和 CD25 阳性,四名患者 CD2 阳性。四名患者核型正常,两名患者核型异常。4/6的病例检测到基因突变。血清胰蛋白酶水平中位数为 24.9(范围:20.1-171.9)微克/升。两名患者接受了文尼考昔(venetoclax)和阿扎胞苷的诱导治疗(其中一名患者在与阿法替尼联合治疗后获得部分缓解,另一名患者在与达沙替尼联合治疗后无缓解)。两名患者分别接受了克拉利宾和伊马替尼治疗,但仍未获得完全缓解。一名接受干扰素联合糖皮质激素治疗的患者失去了随访,一名患者放弃了治疗。随访时间从1.1个月到21.7个月不等。三名患者死亡,两名患者存活。总之,MCL 是系统性肥大细胞增多症的一种罕见亚型,临床病程多变,这些患者的预后较差。我们迫切需要更好地了解 MCL 的临床特征、治疗和预后。
{"title":"[Report of six cases with mast cell leukemia and a literature review].","authors":"F Zhu, Y Yu, C Y Chen, W B Duan, Q Jiang, R Yan, Y Sun, Y Q Han, J Zhang, H Wang, Q R Zhang, S N Chen, W H Yan, M J Cai, Z B Zhang, J Yin, Q Wang","doi":"10.3760/cma.j.cn112138-20240401-00209","DOIUrl":"10.3760/cma.j.cn112138-20240401-00209","url":null,"abstract":"<p><p>From October 2021 to February 2023, we retrospectively analyzed the clinical and laboratory data of six patients (three male and three female, median age: 54 years, age range: 29-73 years) with mast cell leukemia (MCL) diagnosed in the First Affiliated Hospital of Soochow University (The Mastocytosis Collaborative Network of China). All patients had acute MCL, with at least one C-finding present. The main clinical presentations were hypoalbuminemia (<i>n</i>=4), fatigue (<i>n</i>=3), fever (<i>n</i>=2), abdominal discomfort (<i>n</i>=2), osteolytic lesions (<i>n</i>=2), dizziness (<i>n</i>=1), skin flushing (<i>n</i>=1), and weight loss (<i>n</i>=1). Splenomegaly and lymphadenopathy were noted in six and three patients, respectively. Six patients were strongly positive for CD117, five were positive for CD30 and CD25, and four were positive for CD2. Four patients had a normal karyotype and two patients had an abnormal karyotype. Gene mutations were detected in 4/6 cases. The median serum tryptase level was 24.9 (range: 20.1-171.9) μg/L. Two patients were treated with venetoclax and azacitidine for induction (one patient achieved partial remission by combination with afatinib, while there was no remission after combination with dasatinib in the other patient). Two patients did not achieve complete remission despite treatment with cladribine and imatinib, respectively. One patient treated with interferon combined with glucocorticoids was lost to follow-up, and one patient abandoned treatment. The follow-up time ranged from 1.1 to 21.7 months. Three patients died and two survived. Overall, MCL is a rare subtype of systemic mastocytosis with heterogeneous clinical course, and these patients have poor outcome. A better understanding of the clinical characteristics, treatment, and prognosis of MCL is urgently needed.</p>","PeriodicalId":68309,"journal":{"name":"中华内科杂志","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142395641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Study on the correlation between fatty liver index and the outcome of high normal blood pressure]. [脂肪肝指数与正常高血压结果的相关性研究]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240409-00229
J C Qu, A P Wang, J T Dou, W J Gu, Z H Lyu, Y M Mu

Objective: To analyze the correlation between fatty liver index (FLI) and the outcomes of individuals with high normal blood pressure. Methods: In this retrospective cohort study, data from the follow-up population of the Beijing branch of the Risk Evaluation of Cancers in Chinese Diabetic Individuals: A Longitudinal (REACTION) study conducted from December 2011 to August 2012 were selected. Obtain indicators such as height, weight, waist circumference, fasting blood glucose, 2-h postprandial blood glucose, triglycerides, high-density lipoprotein cholesterol, and glutamyl transpeptidase were measured, and the FLI was calculated. The population with high normal blood pressure was divided into the FLI<30 group (1 822 cases); 30≤FLI<60 group (1 026 cases); and FLI≥60 group (473 cases) based on FLI levels. The blood pressure outcome data from the follow-up survey of this population from April 2015 to September 2015 were collected. Single factor analysis of variance was used for intergroup comparison, and logistic regression was used to analyze the correlation between FLI and the outcome of high normal blood pressure in the population. Results: The FLI was an independent influencing factor for their conversion to normal blood pressure (all P<0.01). Among all observed populations, the likelihood of conversion to normal blood pressure in the 30≤FLI<60 group and FLI≥60 group was 0.63 (95%CI 0.51-0.78) and 0.61 (95%CI 0.45-0.82) of the FLI<30 group, respectively. In the population of 40≤age<60 years, this likelihood was 0.60 (95%CI 0.47-0.76) and 0.57 (95%CI 0.41-0.79), respectively. FLI is not an independent influencing factor for the conversion to normal blood pressure in individuals aged over 60 years (P=0.161). FLI is an independent risk factor for hypertension (all P<0.05). Among all observed populations and population of 40≤age<60 years and age>60 years, the risk of hypertension in the 30≤FLI<60 group and FLI≥60 group was 1.49 times (95%CI 1.23-1.80) and 1.54 times (95%CI 1.19-1.98); 1.41 times (95%CI 1.13-1.75) and 1.38 times (95%CI 1.04-1.83); and 1.75 times (95%CI 1.22-2.53) and 2.10 times (95%CI 1.24-3.58) of the FLI<30 group, respectively. Conclusions: There is a correlation between FLI levels and future outcomes of individuals with normal high blood pressure. Although people with higher FLI are more likely to develop hypertension, those with higher FLI are also less likely to develop normal blood pressure in the 40≤age<60-year group.

目的分析脂肪肝指数(FLI)与高正常血压患者预后的相关性。方法在这项回顾性队列研究中,数据来自 2011 年 12 月至 2012 年 8 月进行的中国糖尿病患者癌症风险评估北京分部的随访人群:纵向研究(REACTION)的数据。测量身高、体重、腰围、空腹血糖、餐后 2 小时血糖、甘油三酯、高密度脂蛋白胆固醇和谷氨酰转肽酶等指标,并计算 FLI。高正常血压人群被分为 FLIResults:FLI是他们转为正常血压的独立影响因素(均为P0.01)。在所有观察人群中,30≤FLICI 0.51-0.78) 和 0.61(95%CI 0.45-0.82)的人群转换为正常血压的可能性分别为 0.47-0.76) 和 0.57(95%CI 0.41-0.79)。FLI 不是 60 岁以上人群血压转为正常的独立影响因素(P=0.161)。FLI 是高血压的独立危险因素(所有 P60 岁的人,30≤FLICI 的高血压风险为 1.23-1.80 倍(95%CI 1.19-1.98);1.41 倍(95%CI 1.13-1.75)和 1.38 倍(95%CI 1.04-1.83);FLIC 结论为 1.75 倍(95%CI 1.22-2.53)和 2.10 倍(95%CI 1.24-3.58):FLI水平与正常高血压患者的未来结果之间存在相关性。虽然 FLI 较高的人更容易患高血压,但 FLI 较高的人在 40 岁以下年龄段患正常血压的可能性也较小。
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引用次数: 0
[Progresses and hot spots of cerebrovascular disease in the past decade]. [过去十年脑血管疾病的进展与热点]。
Pub Date : 2024-10-01 DOI: 10.3760/cma.j.cn112138-20240531-00354
J J Li, Y J Wang
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引用次数: 0
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