{"title":"[Massive ascites and gastrointestinal bleeding caused by idiopathic inferior mesenteric arteriovenous fistula: a case report].","authors":"H Xiao, Q Song, Y T Wang, D Dong","doi":"10.3760/cma.j.cn112138-20220718-00528","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20220718-00528","url":null,"abstract":"肠系膜下动静脉瘘(IMAVF)是指肠系膜下动脉与肠系膜下静脉之间的异常交通,临床上较为罕见。本文报道1例患者短期内出现无明显诱因的门脉高压、顽固性腹水、胃底食管静脉曲张及上消化道出血的症状,CT检查提示肠系膜下动脉远端与肠系膜下静脉之间存在动静脉瘘,经介入栓塞局部动静脉瘘后,患者病情好转出院。.","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"852-854"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10104054","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}
{"title":"[The 501st case: elevated blood glucose, chronic pancreatitis, and post- pancreatoduodenectomy malnutrition].","authors":"A Song, R Zhang, Y Chi, H B Zhang","doi":"10.3760/cma.j.cn112138-20221120-00870","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20221120-00870","url":null,"abstract":"<p><p>A 50-year-old man with a 15-year history of elevated blood glucose and an approximately 2-year history of diarrhea was admitted to the Peking Union Medical College Hospital. The initial diagnosis was type 2 diabetes. After repeated pancreatitis and pancreatoduodenectomy, severe pancreatic endocrine and exocrine dysfunction including alternating high and low blood glucose and fat diarrhea occurred. Tests for type 1 diabetes-related antibodies were all negative, C-peptide levels were substantially reduced, fat-soluble vitamin levels were reduced, and there was no obvious insulin resistance. Therefore, a diagnosis of pancreatic diabetes was clear. The patient was given small doses of insulin and supplementary pancreatin and micronutrients. Diarrhea was relieved and blood glucose was controlled. The purpose of this article is to raise clinicians' awareness of the possibility of pancreatic diabetes after pancreatitis or pancreatic surgery. Timely intervention and monitoring may reduce the occurrence of complications.</p>","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"891-895"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9800300","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}
{"title":"[Analysis of the clinical characteristics of 25 cases with parenchymal neuro-Behcet's disease].","authors":"Y Li, Q Shi, Y Lin, M Y Liu, J Q Liu","doi":"10.3760/cma.j.cn112138-20230126-00034","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20230126-00034","url":null,"abstract":"<p><p><b>Objective:</b> To retrospectively investigate the clinical data, radiological characteristics, treatment, and outcome of patients with parenchymal neuro-Behcet's disease (P-NBD) with particular emphasis on dizziness. <b>Methods:</b> This was a cross-sectional study of clinical data from 25 patients with a confirmed diagnosis of P-NBD who were admitted to the Department of Neurology of the First Medical Center of Chinese People's Liberation Army General Hospital between 2010 and 2022. The median age of the population was 37 years (range: 17-85 years). Clinical data were retrospectively analyzed, including gender, age of onset, disease duration, clinical manifestations, serum immune indicators, cerebrospinal fluid (CSF) routine biochemical and cytokine levels, cranial and spinal magnetic resonance imaging (MRI) findings, treatment, and outcome. <b>Results:</b> The majority of patients were male (16 cases; 64.0%), the mean age of onset was (28±14) (range: 4-58 years), and the disease course was acute or subacute. Fever was the most common clinical presentation, and the complaint of dizziness was not uncommon (8/25 patients). Analysis of serum immune indices, including complement (C3 and C4), erythrocyte sedimentation rate, interleukin-1 (IL-1), IL-6, IL-8 and tumor necrotic factor-alpha were abnormal in 80.0% of patients (20/25). Most of the 16/25 patients who underwent lumbar puncture tests had normal intracranial pressure and increased CSF white cell count and protein [median values were 44 (15-380) ×10<sup>6</sup>/L and 0.73 (0.49-2.81) g/L, respectively]. Of the five patients who underwent CSF cytokine tests, four patients had abnormal results; of these, an elevated level of IL-6 was most common, followed by IL-1 and IL-8. The most common site of involvement in cranial MRI was the brainstem and basal ganglia (60.0% respectively), followed by white matter (48.0%) and the cortex (44.0%). Nine cases (36.0%) showed lesions with enhancement and six cases (24.0%) showed mass-like lesions. Three patients (12.0%) patients had lesions in the spinal cord, most frequently in the thoracic cord. All patients received immunological intervention therapy; during follow up, the majority had a favorable outcome. <b>Conclusions:</b> P-NBD is an autoimmune disease with multiple system involvement and diverse clinical manifestations. The symptom of dizziness is not uncommon and can be easily ignored. Early treatment with immunotherapy is important and can improve the outcome of these patients.</p>","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"808-813"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10121238","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}
{"title":"[Brucella endocarditis: a case report].","authors":"H Liu, Y Ge, L B Xu, G T Ma, X J Ma","doi":"10.3760/cma.j.cn112138-20220709-00502","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20220709-00502","url":null,"abstract":"布鲁菌引起心内膜炎临床罕见,尽早明确诊断是治疗成功的关键,仅内科治疗通常效果欠佳,应尽早手术行瓣膜修复、置换。本文报道了1例布鲁菌心内膜炎患者内外科联合诊治的经过。.","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"850-852"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10104049","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}
Zhonghua nei ke za zhiPub Date : 2023-07-01DOI: DOI:10.3760/cma.j.cn112138-20230321-00164
T Chen, H T Lyu
{"title":"[Progress and challenges of clinical decision support system for rare diseases].","authors":"T Chen, H T Lyu","doi":"DOI:10.3760/cma.j.cn112138-20230321-00164","DOIUrl":"https://doi.org/DOI:10.3760/cma.j.cn112138-20230321-00164","url":null,"abstract":"","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"869-875"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9800297","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}
{"title":"[Missense mutation of SPRY4 gene in Kallmann syndrome: a case report].","authors":"Q Hui, Q Zhang, G F Qian","doi":"10.3760/cma.j.cn112138-20221009-00744","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20221009-00744","url":null,"abstract":"Kallmann综合征是一种罕见的先天性疾病,主要表现为嗅觉缺陷和低促性腺激素性性腺功能减退[1]。迄今为止,已发现30余种基因与该病相关。本文报道1例因自幼嗅觉缺失、发现阴茎短小4年就诊的青少年男性,经基因检测诊断为由少见基因SPRY4错义突变引起的Kallmann综合征,旨在丰富疾病基因突变谱,同时提高临床医生对该病的认识。.","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"860-863"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10104056","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}
{"title":"[Chinese expert consensus on intra-arterial drug and combined drug administration for primary hepatocellular carcinoma].","authors":"","doi":"10.3760/cma.j.cn112138-20230202-00049","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20230202-00049","url":null,"abstract":"<p><p>Transarterial interventional therapy is one of the most widely used treatment methods in patients with primary hepatocellular carcinoma. With the progress in interventional technology and the use of new drugs, transarterial interventional therapy has achieved favorable results in the treatment of primary hepatocellular carcinoma and has become the first choice non-surgical treatment for advanced liver cancer. However, at present, there are great differences in the drugs used in transarterial interventional treatment and the combined application of other drugs among centers, and there is no uniform consensus or guideline. Based on the latest research data and clinical practice experience, as well as the characteristics of Chinese patients, the Specialist Group of Interventional Drugs, Interventionalists Branch of the Chinese Medical Doctor Association was organized to formulate the Chinese expert consensus on intra-arterial drug and combined drug administration for primary hepatocellular carcinoma. The purpose of this consensus is to explore the efficacy and safety of drugs and drug combinations related to intra-arterial interventional therapy, the use of drugs in special populations, the management of adverse reactions, and adjuvant drugs to provide a reference for clinical practice.</p>","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"785-801"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9779037","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}
{"title":"[Progress in diagnosis and treatment of vertigo in children].","authors":"F Gao, S S Gong","doi":"10.3760/cma.j.cn112138-20230330-00177","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20230330-00177","url":null,"abstract":"儿童眩晕症涉及不同专业,其临床诊治需要多学科合作。受儿童年龄、语言表达能力、配合能力等诸多因素的影响,眩晕病的病因构成及临床表现与成人不同。客观采集病史是获得确定诊断的重要依据。平衡功能、眼球运动检查以及听力学评估具有重要的鉴别诊断意义。儿童眩晕疾病的治疗建议很少且治疗意见有明显不均衡性。.","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"876-880"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9800298","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}
{"title":"[Progress and challenges of clinical decision support system for rare diseases].","authors":"T. Chen, H. Lyu","doi":"10.3760/cma.j.cn112138-20230321-00164","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20230321-00164","url":null,"abstract":"\u0000 罕见病影响全球3亿多人口,提高疾病筛查与诊断能力对改善患者预后、减少医疗资源消耗至关重要。随着信息技术飞速进步,临床决策支持系统(clinical decision support system,CDSS)为疾病筛查与诊断提供了技术支撑,特别是在病历电子化时代,CDSS在数据集成、行为干预等方面有了更大进展,但同时亦面临诸多挑战。本文通过对罕见病 CDSS的进展进行综述,剖析存在的挑战与发展方向,为国内罕见病筛诊领域CDSS的建设提供参考。.\u0000","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"26 1","pages":"869-875"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80213687","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}
S Yan, S Jin, P F Wang, L Z Yan, J J Shang, X L Shi, X J Wu, Y Y Zhai, W Q Yao, J Wang, Y Yao, C C Fu
{"title":"[Efficacy and safety of VRD regimen of autologous hematopoietic stem cell transplantation in patients with newly diagnosed multiple myeloma].","authors":"S Yan, S Jin, P F Wang, L Z Yan, J J Shang, X L Shi, X J Wu, Y Y Zhai, W Q Yao, J Wang, Y Yao, C C Fu","doi":"10.3760/cma.j.cn112138-20220918-00694","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20220918-00694","url":null,"abstract":"<p><p><b>Objective:</b> To explore the stem cell collection rate and efficacy and safety of patients aged 70 and below with newly diagnosed multiple myeloma (MM) treated with the VRD (bortezomib, lenalidomide and dexamethasone) regimen followed by autologous stem cell transplantation (ASCT). <b>Methods:</b> Retrospective case series study. The clinical data of 123 patients with newly diagnosed MM from August 1, 2018, to June 30, 2020, at the First Affiliated Hospital of Soochow University and Suzhou Hopes Hematology Hospital, who were eligible for VRD regimen sequential ASCT, were collected. The clinical characteristics, efficacy after induction therapy, mobilization regimen of autologous stem cells, autologous stem cell collection rate, and side effects and efficacy of ASCT were retrospectively analyzed. <b>Results:</b> Of the 123 patients, 67 were males. The median patient age was 56 (range: 31-70) years. Patients with IgG, IgA, IgD, and light-chain types accounted for 47.2% (58/123), 23.6% (29/123), 3.2% (4/123), and 26.0% (32/123) of patients, respectively. In addition, 25.2% (31/123) of patients had renal insufficiency (creatinine clearance rate<40 ml/min). Patients with Revised-International Staging System (R-ISS) Ⅲ accounted for 18.2% (22/121) of patients. After induction therapy, the rates of partial response and above, very-good partial response (VGPR) and above, and complete response (CR)+stringent CR were 82.1% (101/123), 75.6% (93/123), and 45.5% (56/123), respectively. Overall, 90.3% (84/93) of patients were mobilized with cyclophosphamide+granulocyte colony-stimulating factor (G-CSF) and 8 patients with G-CSF or G-CSF+plerixafor due to creatinine clearance rate<30 ml/min and one of them was mobilized with DECP (cisplatin, etoposide, cyclophosphamide and dexamethasone)+G-CSF for progressive disease. The rate of autologous stem cell collection (CD34<sup>+</sup>cells≥2×10<sup>6</sup>/kg) after four courses of VRD regimen was 89.1% (82/92), and the rate of collection (CD34<sup>+</sup>cells≥5×10<sup>6</sup>/kg) was 56.5% (52/92). Seventy-seven patients treated with the VRD regimen sequential ASCT. All patients had grade 4 neutropenia and thrombocytopenia. Among the nonhematologic adverse events during ASCT, the highest incidence was observed for gastrointestinal reactions (76.6%, 59/77), followed by oral mucositis (46.8%, 36/77), elevated aminotransferases (44.2%, 34/77), fever (37.7%, 29/77), infection (16.9%, 13/77) and heart-related adverse events (11.7%, 9/77). Among the adverse events, grade 3 adverse events included nausea (6.5%, 5/77), oral mucositis (5.2%, 4/77), vomiting (3.9%, 3/77), infection (2.6%, 2/77), elevated blood pressure after infusion (2.6%, 2/77), elevated alanine transaminase (1.3%, 1/77), and perianal mucositis (1.3%, 1/77); there were no grade 4 or above nonhematologic adverse events. The proportion of patients who achieved VGPR and above after VRD sequential ASCT was 100% (75/75), and the proportion of patients ","PeriodicalId":24000,"journal":{"name":"Zhonghua nei ke za zhi","volume":"62 7","pages":"819-825"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9800293","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}