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[The 507th case: hemolytic anemia, parvovirus B19, and multiple organ dysfunction]. [第 507 个病例:溶血性贫血、细小病毒 B19 和多器官功能障碍]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20231210-00376
Y Liu, X H Zhang, L Q Cao, X J Huang, X D Mo

A 19-year-old male patient with high-risk acute B-cell lymphoblastic leukemia received haploidentical stem cell transplantation. He developed anemia repeatedly and parvovirus B19 nucleic acid was positive in blood plasma. The patient was diagnosed with cold agglutinin syndrome and multiple organ dysfunction including respiratory failure and hepatitis. In the conflict between viral infection and the treatment of cold agglutinin syndrome, we provided supportive treatment, complement inhibitors to control hemolysis, and antiviral therapy. After timely glucocorticoid and immunosuppressant therapy, the patient had achieved a good response.

一名患有高危急性 B 细胞淋巴细胞白血病的 19 岁男性患者接受了单倍体干细胞移植。他反复出现贫血,血浆中 parvovirus B19 核酸呈阳性。患者被诊断为冷凝集素综合征和多器官功能障碍,包括呼吸衰竭和肝炎。在病毒感染与冷凝集素综合征治疗的矛盾中,我们给予了支持治疗、补体抑制剂控制溶血和抗病毒治疗。经过及时的糖皮质激素和免疫抑制剂治疗,患者取得了良好的反应。
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引用次数: 0
[Advances in the specific treatments of Gaucher disease]. [戈谢病特殊疗法的进展]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20240328-00199
Y Meng
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引用次数: 0
[A study of the correlation between gray matter atrophy in multiple sclerosis and impairment of cognitive function domains]. [多发性硬化症灰质萎缩与认知功能障碍之间的相关性研究]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20231129-00350
J Han, Q J Wang, C H Wang, Z H Li, R H Bai, X Zhang, J G Liu

Objective: To quantify cerebral cortical and deep gray matter atrophy in patients with multiple sclerosis (MS) and explore its correlation with impairment in domains of cognitive function. Methods: Twenty patients with MS and 16 healthy controls (HC) matched for age, sex, and education level were included. Using FreeSurfer software, based on 3D-MRI technology, the differences in cortical thickness and deep gray matter volume between the two groups were comparatively analyzed. A neuropsychological scale that included six domains of cognitive function was scored on both study groups to analyze the correlation between cortical thickness and volume of deep gray matter in MS patients with impairment in cognitive function domains. Results: Impairment in domains of cognitive function: cognitive impairment was present in 60% MS patients in this study, mainly manifesting as impairment of verbal memory, verbal fluency, visuospatial memory, and information processing speed function (all P<0.05). Of these, the majority had impaired visuospatial memory function (55.0%), and the least number of patients had impaired information processing speed (15.0%). Changes in cortical thickness: compared with the HC group, the MS group showed that cortical atrophy was mainly concentrated in the frontoparietal region, including significant thinning of cortical thickness in the left inferior parietal gyrus, right superior frontal gyrus, and the right superior parietal gyrus (all P<0.05). Among them, atrophy of the left inferior parietal gyrus was significantly positively correlated with the impairment of verbal memory, verbal fluency, and information processing speed (all P<0.05). There was a significant positive correlation between the right superior frontal gyrus atrophy and verbal memory, verbal fluency, and visuospatial memory impairment (all P<0.05). Changes in deep gray matter volume: compared with the HC group, deep gray matter volume in the MS group decreased significantly in the bilateral thalamus, bilateral putamen, bilateral pallidum (all P<0.01), and right nucleus accumbens (P<0.05). Among them, left thalamus atrophy was significantly positively correlated with visuospatial memory impairment (r=0.45, P=0.046), and left putamen atrophy was both significantly positively correlated with visuospatial memory (r=0.45, P=0.047) and information processing speed impairment (r=0.50, P=0.026). Conclusions: Early structural brain changes in MS are dominated by gray matter atrophy. Deep gray matter is more prominent than cortical atrophy.

目的量化多发性硬化症(MS)患者的大脑皮质和深部灰质萎缩情况,并探讨其与认知功能障碍的相关性。方法:20 名多发性硬化症患者和 16 名健康对照者:纳入 20 名多发性硬化症患者和 16 名在年龄、性别和教育程度方面匹配的健康对照组(HC)。使用基于 3D-MRI 技术的 FreeSurfer 软件,比较分析两组患者皮质厚度和深部灰质体积的差异。对两组研究人员进行包括六个认知功能领域的神经心理学量表评分,以分析认知功能领域受损的多发性硬化症患者的皮质厚度和深部灰质体积之间的相关性。结果显示认知功能领域损害:本研究中60%的多发性硬化症患者存在认知功能损害,主要表现为言语记忆、言语流畅性、视觉空间记忆和信息处理速度功能损害(均PPPPr=0.45,P=0.046),左侧普特蒙萎缩与视觉空间记忆(r=0.45,P=0.047)和信息处理速度损害(r=0.50,P=0.026)均呈显著正相关。结论多发性硬化症的早期脑结构变化以灰质萎缩为主。深部灰质比皮质萎缩更为突出。
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引用次数: 0
[Vertigo and dizziness in child: a clinical analysis in 120 cases]. [儿童眩晕症:120 例临床分析]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20240318-00179
F Gao, S S Gong

Objective: To investigate the disease composition, clinical features, diagnosis, and treatment characteristics of vertigo in children. Methods: A total of 120 children with vertigo diagnosed and treated in the Department of Otorhinolaryngology, Children's Hospital, Capital Institute of Pediatrics in Beijing from February 2018 to February 2022 were retrospectively analyzed to explore the clinical characteristics of common peripheral vertigo in children and to summarize the experience of diagnosis and treatment. Results: The etiological composition of 120 cases of vertigo in children are as follows: 63 (52.5%) cases of vestibular migraine of childhood (VMC), 19 (15.8%) of recurrent vertigo of childhood (RVC), 11 (9.2%) of probable vestibular migraine of childhood (PVMC), 10 (8.3%) of secretory otitis media (SOM), 6 (5.0%) of persistent postural-perceptual dizziness (PPPD), 4 (3.3%) of benign paroxysmal positional vertigo (BPPV), 2 (1.7%) of vestibular neuritis (VN), 2 (1.7%) of Meniere's disease (MD), 2 (1.7%) of inner ear malformation (IEM), and 1 (0.8%) of vestibular paroxysmal syndrome (VP).The major cause of vertigo in children of different ages was different. SOM was the most important cause in preschool children, followed by RVC and VMC; VMC was the most important cause in school-age children, followed by RVC; and MD and BPPV were exclusive found in adolescents. The incidence rate of PPPD was higher in adolescents than in preschool and school-age children. Children with vertigo had good prognosis in general. Conclusions: VMC, RVC and SOM are the most common causes in vertigo in children, and their proportion was different in different aged children. Transforming abstract feelings into specific information is the skill required for collecting medical history of children with vertigo. Considering the age and cooperation of children, appropriate hearing and vestibular examination techniques are recommended. We should pay more attention to the mental health of children with vertigo and their parents.

目的研究儿童眩晕症的疾病构成、临床特征、诊断和治疗特点。方法回顾性分析2018年2月至2022年2月在北京首都儿科研究所附属儿童医院耳鼻咽喉科诊治的120例眩晕患儿,探讨儿童常见周围性眩晕的临床特点,总结诊治经验。结果显示120例儿童眩晕的病因构成如下:63例(52.5%)儿童前庭性偏头痛(VMC),19例(15.8%)儿童复发性眩晕(RVC),11例(9.2%)儿童可能前庭性偏头痛(PVMC),10例(8.3%)分泌性中耳炎(SOM),6例(5.0%)持续性姿势感知性眩晕(PPPD),4例(3.不同年龄段儿童眩晕的主要原因各不相同。学龄前儿童最主要的眩晕病因是 SOM,其次是 RVC 和 VMC;学龄儿童最主要的眩晕病因是 VMC,其次是 RVC;而 MD 和 BPPV 则仅见于青少年。青少年 PPPD 的发病率高于学龄前儿童和学龄儿童。患有眩晕症的儿童一般预后良好。结论VMC、RVC 和 SOM 是导致儿童眩晕的最常见原因,它们在不同年龄儿童中所占的比例不同。将抽象感受转化为具体信息是收集眩晕患儿病史所需的技能。考虑到儿童的年龄和合作能力,建议采用适当的听力和前庭检查技术。我们应更加关注眩晕患儿及其家长的心理健康。
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引用次数: 0
[Analysis of the characteristics of patients with amyotrophic lateral sclerosis with neuromuscular junction dysfunction prior to motor neuron degeneration]. [运动神经元变性前神经肌肉接头功能障碍的肌萎缩性脊髓侧索硬化症患者特征分析]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20230811-00049
J H Zhang, Z H Chen, L Ling, H M Cheng, Y Zhang, J R Zhao, X S Huang

Objective: To investigate the clinical and electrophysiological characteristics of patients with amyotrophic lateral sclerosis (ALS) with positive repetitive nerve stimulation (RNS) test results on the accessory nerve and negative needle electromyography (EMG) test results on the sternocleidomastoid with the goal to enrich the knowledge of disease progression in patients with ALS. Methods: The clinical data of 612 patients diagnosed with ALS at the Neurology Department of the First Medical Center, Chinese PLA General Hospital from June 2016 to August 2022 were collected. In total, 267 cases had undergone EMG tests on the sternocleidomastoid following a positive 3 Hz RNS test result on the accessory nerve, who were selected as the study subjects. The differences in clinical indicators were compared between RNS (+)/EMG (-) group and RNS (+)/EMG (+) group. A binomial distribution model with multiple variables was built to quantitatively analyze the major factors and their effects. Results: At the initial visit, 15.8% of patients with ALS were 3 Hz RNS (+) on the accessory nerve and EMG (-) on the ipsilateral sternocleidomastoid, accounting for 36.3% of RNS (+) patients. The decremental range of the 3 Hz RNS test delivered to the accessory nerve in these patients [-14% (-19%, -12%)] was lower than that in patients with RNS (+)/EMG (+) [-17% (-23%, -13%)] (P<0.05), while the ratio of upper limb onset (64.9%) and non-definite diagnosis (28.9%) were higher [54.7% and 13.5% for patients with RNS (+)/EMG (+), P<0.05]. Furthermore, the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) score [40 (37, 42)], body mass index (BMI) [23.8 (22.0, 25.4) kg/m2] and forced vital capacity (FVC) [92.8% (76.6%, 103.8%)] were higher in patients with RNS(+)/EMG(+) (P<0.05). The multivariate model suggested that, in patients with RNS (+)/EMG (-), the ratio of upper limb onset to lower limb onset was 1.04, while that of upper limb onset to bulbar onset was 2.02, and that of lower limb onset to bulbar onset was 1.94. The ratio of non-definite ALS to definite ALS was 1.13. The ALSFRS-R score, BMI, and FVC had a protective contribution to the electrophysiological function of the motor neurons. The ratio of the effect size of the ALSFRS-R or BMI to that of FVC was 3.37 and 1.14, respectively. Conclusions: Patients with ALS that were 3 Hz RNS (+) on the accessory nerve and EMG (-) on the ipsilateral sternocleidomastoid had a smaller decremental range of the compound muscle action potential amplitude, and a higher proportion of upper limb onset and non-definite ALS. A higher ALSFRS-R score, BMI, and FVC have a protective effect to the electrophysiological function of motor neurons. The effect size of the ALSFRS-R score is the largest, followed by BMI and FVC.

目的研究肌萎缩性脊髓侧索硬化症(ALS)患者辅助神经重复刺激(RNS)测试结果阳性和胸锁乳突肌针刺肌电图(EMG)测试结果阴性的临床和电生理特点,以丰富对ALS患者疾病进展的认识。研究方法收集中国人民解放军总医院第一医学中心神经内科2016年6月至2022年8月确诊的612例ALS患者的临床资料。其中,267 例患者在附属神经 3 Hz RNS 检测结果阳性后接受了胸锁乳突肌肌电图检测,这些患者被选作研究对象。比较了 RNS(+)/EMG(-)组与 RNS(+)/EMG(+)组在临床指标上的差异。建立多变量二项分布模型,定量分析主要因素及其影响。结果初诊时,15.8% 的 ALS 患者附属神经 3 Hz RNS(+),同侧胸锁乳突肌 EMG(-),占 RNS(+)患者的 36.3%。与 RNS(+)/EMG(+)患者[-17% (-23%, -13%)](PP2)相比,这些患者的副神经 3 Hz RNS 测试递减范围[-14% (-19%, -12%)]更小,而 RNS(+)/EMG(+)患者的强迫生命容量(FVC)[92.8% (76.6%, 103.8%)]更高(PC结论:附属神经3赫兹RNS(+)和同侧胸锁乳突肌EMG(-)的ALS患者的复合肌动作电位振幅递减范围较小,上肢发病和非终末期ALS的比例较高。ALSFRS-R 评分、体重指数和肺活量越高,对运动神经元的电生理功能越有保护作用。ALSFRS-R 评分的效应大小最大,其次是体重指数和肺活量。
{"title":"[Analysis of the characteristics of patients with amyotrophic lateral sclerosis with neuromuscular junction dysfunction prior to motor neuron degeneration].","authors":"J H Zhang, Z H Chen, L Ling, H M Cheng, Y Zhang, J R Zhao, X S Huang","doi":"10.3760/cma.j.cn112138-20230811-00049","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20230811-00049","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the clinical and electrophysiological characteristics of patients with amyotrophic lateral sclerosis (ALS) with positive repetitive nerve stimulation (RNS) test results on the accessory nerve and negative needle electromyography (EMG) test results on the sternocleidomastoid with the goal to enrich the knowledge of disease progression in patients with ALS. <b>Methods:</b> The clinical data of 612 patients diagnosed with ALS at the Neurology Department of the First Medical Center, Chinese PLA General Hospital from June 2016 to August 2022 were collected. In total, 267 cases had undergone EMG tests on the sternocleidomastoid following a positive 3 Hz RNS test result on the accessory nerve, who were selected as the study subjects. The differences in clinical indicators were compared between RNS (+)/EMG (-) group and RNS (+)/EMG (+) group. A binomial distribution model with multiple variables was built to quantitatively analyze the major factors and their effects. <b>Results:</b> At the initial visit, 15.8% of patients with ALS were 3 Hz RNS (+) on the accessory nerve and EMG (-) on the ipsilateral sternocleidomastoid, accounting for 36.3% of RNS (+) patients. The decremental range of the 3 Hz RNS test delivered to the accessory nerve in these patients [-14% (-19%, -12%)] was lower than that in patients with RNS (+)/EMG (+) [-17% (-23%, -13%)] (<i>P</i><0.05), while the ratio of upper limb onset (64.9%) and non-definite diagnosis (28.9%) were higher [54.7% and 13.5% for patients with RNS (+)/EMG (+), <i>P</i><0.05]. Furthermore, the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) score [40 (37, 42)], body mass index (BMI) [23.8 (22.0, 25.4) kg/m<sup>2</sup>] and forced vital capacity (FVC) [92.8% (76.6%, 103.8%)] were higher in patients with RNS(+)/EMG(+) (<i>P</i><0.05). The multivariate model suggested that, in patients with RNS (+)/EMG (-), the ratio of upper limb onset to lower limb onset was 1.04, while that of upper limb onset to bulbar onset was 2.02, and that of lower limb onset to bulbar onset was 1.94. The ratio of non-definite ALS to definite ALS was 1.13. The ALSFRS-R score, BMI, and FVC had a protective contribution to the electrophysiological function of the motor neurons. The ratio of the effect size of the ALSFRS-R or BMI to that of FVC was 3.37 and 1.14, respectively. <b>Conclusions:</b> Patients with ALS that were 3 Hz RNS (+) on the accessory nerve and EMG (-) on the ipsilateral sternocleidomastoid had a smaller decremental range of the compound muscle action potential amplitude, and a higher proportion of upper limb onset and non-definite ALS. A higher ALSFRS-R score, BMI, and FVC have a protective effect to the electrophysiological function of motor neurons. The effect size of the ALSFRS-R score is the largest, followed by BMI and FVC.</p>","PeriodicalId":68309,"journal":{"name":"中华内科杂志","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141478076","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
[A case report of a MODY 10 family presenting as hypokalemic periodic paralysis]. [出现低钾性周期性麻痹的 MODY 10 家族病例报告]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20231019-00230
B Li, T Wu, W J Gu
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引用次数: 0
[A case report of ACTH-independent Cushing's syndrome associated with bilateral adrenal adenomas]. [与双侧肾上腺腺瘤相关的 ACTH 依赖性库欣综合征病例报告]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20231008-00188
Y Song, H Shen, Z P Feng, Z P Du, S M Yang, Q F Li, B Tian
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引用次数: 0
[Expert consensus on stroke prevention and blood glucose management in patients with type 2 diabetes]. [关于 2 型糖尿病患者预防中风和血糖管理的专家共识]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20231201-00356

Abnormal glucose metabolism is closely related to stroke and has adverse effects on the occurrence, development, and prognosis of stroke. Ideal glycemic control is of great significance in improving the prognosis of stroke. Some hypoglycemic drugs can reduce the risk of stroke occurrence and recurrence in patients with type 2 diabetes. Furthermore, such patients with stroke should strengthen their blood pressure and blood lipid control and use antiplatelet drugs reasonably. The expert consensus group finally established this consensus after discussions pertaining to evidence-based medicine and clinical practice, with the aim to provide a reference for clinical practice.

糖代谢异常与脑卒中密切相关,对脑卒中的发生、发展和预后都有不利影响。理想的血糖控制对改善脑卒中预后具有重要意义。一些降糖药物可以降低 2 型糖尿病患者脑卒中发生和复发的风险。此外,此类脑卒中患者应加强血压、血脂控制,合理使用抗血小板药物。专家共识组经过循证医学和临床实践的讨论,最终确立了本共识,旨在为临床实践提供参考。
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引用次数: 0
[Advances in early diagnosis of non-thromboembolic tumor related pulmonary vascular disease]. [非血栓栓塞性肿瘤相关肺血管疾病的早期诊断进展]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20231031-00266
Y J Ding, L Zhang, Q Y Li
{"title":"[Advances in early diagnosis of non-thromboembolic tumor related pulmonary vascular disease].","authors":"Y J Ding, L Zhang, Q Y Li","doi":"10.3760/cma.j.cn112138-20231031-00266","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20231031-00266","url":null,"abstract":"","PeriodicalId":68309,"journal":{"name":"中华内科杂志","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141478074","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
[Progresses and hot spots of gastrointestinal microecology in the past decade]. [过去十年胃肠道微生态学的进展和热点]。
Pub Date : 2024-07-01 DOI: 10.3760/cma.j.cn112138-20240218-00112
L H Peng, Y S Yang
{"title":"[Progresses and hot spots of gastrointestinal microecology in the past decade].","authors":"L H Peng, Y S Yang","doi":"10.3760/cma.j.cn112138-20240218-00112","DOIUrl":"https://doi.org/10.3760/cma.j.cn112138-20240218-00112","url":null,"abstract":"","PeriodicalId":68309,"journal":{"name":"中华内科杂志","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141478082","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
期刊
中华内科杂志
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