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[Massive ascites and gastrointestinal bleeding caused by idiopathic inferior mesenteric arteriovenous fistula: a case report]. 特发性肠系膜下动静脉瘘致大量腹水及消化道出血1例。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20220718-00528
H Xiao, Q Song, Y T Wang, D Dong
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。.
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引用次数: 0
[The 501st case: elevated blood glucose, chronic pancreatitis, and post- pancreatoduodenectomy malnutrition]. 第501例:高血糖,慢性胰腺炎,胰十二指肠切除术后营养不良。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20221120-00870
A Song, R Zhang, Y Chi, H B Zhang

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.

北京协和医院收治一名50岁男性,有15年高血糖史和约2年腹泻史。最初的诊断是2型糖尿病。反复胰腺炎和胰十二指肠切除术后,发生严重的胰腺内分泌和外分泌功能障碍,包括高低血糖交替和脂肪性腹泻。1型糖尿病相关抗体检测均为阴性,c肽水平大幅降低,脂溶性维生素水平降低,无明显胰岛素抵抗。因此,胰腺糖尿病的诊断是明确的。患者给予小剂量胰岛素和补充胰酶及微量营养素。腹泻缓解,血糖控制。本文的目的是提高临床医生对胰腺炎或胰腺手术后可能发生胰腺糖尿病的认识。及时干预和监测可减少并发症的发生。
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引用次数: 0
[Analysis of the clinical characteristics of 25 cases with parenchymal neuro-Behcet's disease]. [25例脑实质神经-白塞病临床特点分析]。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20230126-00034
Y Li, Q Shi, Y Lin, M Y Liu, J Q Liu

Objective: 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. Methods: 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. Results: 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) ×106/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. Conclusions: 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.

目的:回顾性分析以头晕为主的脑实质性神经白塞病(P-NBD)的临床资料、影像学特征、治疗及转归。方法:对2010年至2022年中国人民解放军总医院第一医疗中心神经内科收治的25例确诊为P-NBD患者的临床资料进行横断面研究。人口年龄中位数为37岁(范围:17-85岁)。回顾性分析临床资料,包括性别、发病年龄、病程、临床表现、血清免疫指标、脑脊液常规生化及细胞因子水平、颅脑及脊髓磁共振成像(MRI)表现、治疗及转归。结果:患者以男性居多(16例;(64.0%),平均发病年龄(28±14)岁(4 ~ 58岁),病程为急性或亚急性。发热是最常见的临床表现,主诉头晕也不少见(8/25例)。80.0%(20/25)患者血清免疫指标补体(C3、C4)、红细胞沉降率、白细胞介素-1 (IL-1)、IL-6、IL-8、肿瘤坏死因子- α异常。16/25行腰椎穿刺检查的患者中,大多数颅内压正常,脑脊液白细胞计数和蛋白升高[中位值分别为44 (15-380)×106/L和0.73 (0.49-2.81)g/L]。在接受脑脊液细胞因子检测的5例患者中,4例患者结果异常;其中,IL-6水平升高最为常见,其次是IL-1和IL-8。颅脑MRI最常见的受累部位是脑干和基底节区(分别为60.0%),其次是白质(48.0%)和皮层(44.0%)。病灶强化9例(36.0%),肿块样病变6例(24.0%)。3例(12.0%)患者有脊髓病变,最常见的是胸脊髓。所有患者均接受免疫干预治疗;在随访期间,大多数患者预后良好。结论:P-NBD是一种多系统受累、临床表现多样的自身免疫性疾病。头晕的症状并不罕见,很容易被忽视。早期免疫治疗很重要,可以改善这些患者的预后。
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引用次数: 0
[Brucella endocarditis: a case report]. 布鲁氏菌心内膜炎1例。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20220709-00502
H Liu, Y Ge, L B Xu, G T Ma, X J Ma
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。.
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引用次数: 0
[Progress and challenges of clinical decision support system for rare diseases]. [罕见病临床决策支持系统的进展与挑战]。
Pub Date : 2023-07-01 DOI: DOI:10.3760/cma.j.cn112138-20230321-00164
T Chen, H T Lyu
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引用次数: 0
[Missense mutation of SPRY4 gene in Kallmann syndrome: a case report]. 【Kallmann综合征SPRY4基因错义突变1例报道】。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20221009-00744
Q Hui, Q Zhang, G F Qian
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。.
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引用次数: 0
[Chinese expert consensus on intra-arterial drug and combined drug administration for primary hepatocellular carcinoma]. 【原发性肝细胞癌动脉给药及联合用药的中国专家共识】。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20230202-00049

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.

经动脉介入治疗是原发性肝癌最常用的治疗方法之一。随着介入技术的进步和新药物的应用,经动脉介入治疗原发性肝癌取得了良好的效果,成为晚期肝癌非手术治疗的首选方法。但目前各中心在经动脉介入治疗中使用的药物及其他药物联合应用方面存在较大差异,没有统一的共识或指南。根据最新的研究资料和临床实践经验,结合中国患者的特点,组织中华医师协会介入医师分会介入药物专家组,制定了原发性肝癌动脉内给药及联合给药的中国专家共识。本共识旨在探讨动脉内介入治疗相关药物及联合用药的有效性和安全性、特殊人群药物的使用、不良反应的处理、辅助用药等,为临床实践提供参考。
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引用次数: 0
[Progress in diagnosis and treatment of vertigo in children]. [儿童眩晕的诊断与治疗进展]。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20230330-00177
F Gao, S S Gong
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。.
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引用次数: 0
[Progress and challenges of clinical decision support system for rare diseases]. [罕见病临床决策支持系统的进展与挑战]。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20230321-00164
T. Chen, H. Lyu
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。.
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。.
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引用次数: 0
[Efficacy and safety of VRD regimen of autologous hematopoietic stem cell transplantation in patients with newly diagnosed multiple myeloma]. [VRD方案自体造血干细胞移植在新诊断多发性骨髓瘤患者中的疗效和安全性]。
Pub Date : 2023-07-01 DOI: 10.3760/cma.j.cn112138-20220918-00694
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

Objective: 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). Methods: 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. Results: 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+cells≥2×106/kg) after four courses of VRD regimen was 89.1% (82/92), and the rate of collection (CD34+cells≥5×106/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

目的:探讨70岁及以下新诊断的多发性骨髓瘤(MM)患者采用VRD(硼替佐米、来那度胺和地塞米松)治疗后自体干细胞移植(ASCT)的干细胞收集率、疗效和安全性。方法:回顾性病例系列研究。收集2018年8月1日至2020年6月30日苏州大学第一附属医院和苏州希望血液医院123例符合VRD方案序贯ASCT的新诊断MM患者的临床资料。回顾性分析ASCT的临床特点、诱导治疗后的疗效、自体干细胞动员方案、自体干细胞收集率、不良反应及疗效。结果:123例患者中,男性67例。患者年龄中位数为56岁(范围:31-70岁)。IgG型占47.2% (58/123),IgA型占23.6% (29/123),IgD型占3.2%(4/123),轻链型占26.0%(32/123)。此外,4个疗程VRD方案后,25.2%(31/123)的患者出现肾功能不全(肌酐清除率+细胞≥2×106/kg)为89.1%(82/92),收集率(CD34+细胞≥5×106/kg)为56.5%(52/92)。77例患者接受VRD方案序贯ASCT治疗。所有患者均有4级中性粒细胞减少症和血小板减少症。在ASCT期间的非血液学不良事件中,胃肠道反应发生率最高(76.6%,59/77),其次是口腔黏膜炎(46.8%,36/77)、转氨酶升高(44.2%,34/77)、发热(37.7%,29/77)、感染(16.9%,13/77)和心脏相关不良事件(11.7%,9/77)。不良事件中,3级不良事件包括恶心(6.5%,5/77)、口腔黏膜炎(5.2%,4/77)、呕吐(3.9%,3/77)、感染(2.6%,2/77)、输液后血压升高(2.6%,2/77)、丙氨酸转氨酶升高(1.3%,1/77)、肛周黏膜炎(1.3%,1/77);无4级及以上非血液学不良事件。VRD序贯ASCT术后达到VGPR及以上的患者比例为100%(75/75),最小残留病阴性(-4级)患者比例为82.7%(62/75)。结论:70岁及以下新发MM患者采用VRD诱导治疗,自体干细胞采集率好,随访ASCT后疗效和耐受性良好。
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引用次数: 0
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