腓骨肌萎缩症外科治疗专家共识

腓骨肌萎缩症在我国已确定为罕见病。由于该病较为罕见,国内骨科界对其认识参差不齐,外科治疗缺乏循证医学的指导意见,欠缺规范化。为更好地认识与治疗该病,避免其并发症的发生,同时为获得更好的疗效,中华医学会骨科学分会足踝外科学组,国际矫形与创伤外科学会中国部足踝外科学会,中国医疗保健国际交流促进会骨科分会足踝外科学部,中国研究型医院学会足踝医学专业委员会,中国医师协会骨科医师分会足踝外科、基础研究与矫形学组联合邀请国内知名专家,参考国内外相关的最新研究成果,研究探讨了该病的病理特点、病程发展规律、检查流程与诊断标准,提出外科治疗的策略与方法,最终形成本共识,以促进我国在该领域的进一步研究和提高腓骨肌萎缩症的诊疗水平。

Association between Air Pollution and Type 2 Diabetes Mellitus in Developing Countries:A Systematic Review and Meta-Analysis

Objective In recent years,many studies have reported that air pollution is a risk factor for type 2 diabetes mellitus(T2DM).The aim of this systematic review and meta・analysis is to summarize the evidence about the association between exposure to air pollution andT2DM in developing countries.Methods The databases,including PubMed,EMBASE and Web of Science,were systematically searched for studies published up to 31 March 2022.Studies about the association between air pollution andT2DM prevalence or incidence in developing countries were included.The odds ratio(OR)was used as effect estimate.We synthesized the included studies in the meta-analysis.Results We included 8 cross-sectional studies and 8 cohort studies,all conducted in developing countries.Meta-analysis of 8 studies on PM_(2.5)(particulate matter ≤2.5 μm in diameter)showed that T2DM prevalence was significantly associated with PM_(2.5)exposure(OR=1.12;95%CI:1.07,1.17;P<0.001).The association between air pollutants andT2DM incidence was not estimated due to the limited relevant studies.Conclusions The exposure to PM_(2.5)would be positively associated with an increased prevalence of T2DM in developing countries.Some effective measures should be taken to reduce air pollutant exposure in people who are vulnerable to diabetes.

Reliability and Validity of the Chinese Version of the Lymphedema Quality of Life Questionnaire

Objective To study the reliability and validity of the Chinese version of the Lymphedema Quality of Life Questionnaire(LYMQOL)in lymphedema patients.Methods LYMQOL was translated into Chinese.The Chinese version of the LYMQOL was distributed with the official Wechat account"Lymphedema Channel"to lymphedema patients who were recruited from October 28^(th),2020 to February 23^(rd),2021.Patients with upper limb lymphedema and lower limb lymphedema completed the LYMQOL-ARM subscale and the LYMQOL-LEG subscale separately,at enrollment,1 week later,and 1 month later.Reliability,validity,feasibility,responsiveness and average time required for completing the questionnaire were assessed.Results A total of 195 patients participated in the study.The Chinese questionnaire showed high reliability with Cronbach’sαcoefficients of 0.849-0.902 for the LYMQOL-ARM and intraclass correlation coefficient(ICC)of 0.848-0.884 and Cronbach’sαcoefficients of 0.726-0.902 for the LYMQOL-LEG and ICC of 0.863-0.900.The LYMQOL showed moderate to good correlations with the EQ-5 D(0.4<r<0.6,P<0.01;for the LYMQOLARM,n=95,for the LYMQOL-LEG,n=102).Responsiveness analysis suggested that quality of life of upper limb lymphedema patients decreased after 1 month(P<0.05).The average time of patients to finish the questionnaire was approximately 12 minutes.Conclusions The Chinese version of the LYMQOL is easy to answer,comprehensive and appropriate in length,and has good reliability and validity.It may be utilized to assist treatment decision-making and track changes in clinical setting or research for lymphedema.

神经外科专业学位研究生的科研诚信建设

科研诚信是科研活动的基本准则。本文介绍了目前神经外科专业学位研究生的科研现状和神经外科学术环境滋生学术不端行为的隐患,并强调了建设及加强科研诚信的手段,旨在针对神经外科专业学位研究生人群,预防学术不端、强化科研诚信建设及促进学科发展。

External and Internal Validation of a Computer Assisted Diagnostic Model for Detecting Multi-Organ Mass Lesions in CT images

Objective We developed a universal lesion detector(ULDor)which showed good performance in in-lab experiments.The study aims to evaluate the performance and its ability to generalize in clinical setting via both external and internal validation.Methods The ULDor system consists of a convolutional neural network(CNN)trained on around 80 K lesion annotations from about 12 K CT studies in the DeepLesion dataset and 5 other public organ-specific datasets.During the validation process,the test sets include two parts:the external validation dataset which was comprised of 164 sets of non-contrasted chest and upper abdomen CT scans from a comprehensive hospital,and the internal validation dataset which was comprised of 187 sets of low-dose helical CT scans from the National Lung Screening Trial(NLST).We ran the model on the two test sets to output lesion detection.Three board-certified radiologists read the CT scans and verified the detection results of ULDor.We used positive predictive value(PPV)and sensitivity to evaluate the performance of the model in detecting space-occupying lesions at all extra-pulmonary organs visualized on CT images,including liver,kidney,pancreas,adrenal,spleen,esophagus,thyroid,lymph nodes,body wall,thoracic spine,etc.Results In the external validation,the lesion-level PPV and sensitivity of the model were 57.9%and 67.0%,respectively.On average,the model detected 2.1 findings per set,and among them,0.9 were false positives.ULDor worked well for detecting liver lesions,with a PPV of 78.9%and a sensitivity of 92.7%,followed by kidney,with a PPV of 70.0%and a sensitivity of 58.3%.In internal validation with NLST test set,ULDor obtained a PPV of 75.3%and a sensitivity of 52.0%despite the relatively high noise level of soft tissue on images.Conclusions The performance tests of ULDor with the external real-world data have shown its high effectiveness in multiple-purposed detection for lesions in certain organs.With further optimisation and iterative upgrades,ULDor may be well suited for extensive application to external data.

Effect of Focused Cardiac Ultrasound in Combination with Lung Ultrasound on Critically Ill Patients: A Multicenter Observational Study in China

Objective Focused cardiac ultrasound(FCU)and lung ultrasound(LU)are increasingly being used in critically ill patients.This study aimed to investigate the effect of FCU in combination with LU on these patients and to determine if the timing of ultrasound examination was associated with treatment change.Methods This is a multicenter cross-sectional observational study.Consecutive patients admitted to the intensive care unit(ICU)were screened for enrollment.FCU and LU were performed within the first 24 h,and treatment change was proposed by the performer based on the ultrasound results and other clinical conditions.Results Among the 992 patients included,502 were examined within 6 h of ICU admission(early phase group),and 490 were examined after 6 h of admission(later phase group).The early phase group and the later phase group had similar proportions of treatment change(48.8%vs.49.0%,χ^(2)=0.003,P=0.956).In the multivariable analysis,admission for respiratory failure was an independent variable associated with treatment change,with an odds ratio(OR)of 2.357[95%confidence interval(CI):1.284-4.326,P=0.006];the timing of examination was not associated with treatment change(OR=0.725,95%CI:0.407-1.291,P=0.275).Conclusions FCU in combination with LU,whether performed during the early phase or later phase,had a significant impact on the treatment of critically ill patients.Patients with respiratory failure were more likely to experience treatment change after the ultrasound examination.

BAG3-Related Myofibrillar Myopathy Presenting as Hypercapnia:A Case Report and Literature Review

Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia.Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209 Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation.Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation.The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209 Leu mutation in BAG3.Totally twentyone patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date,including this patient and literature review.The male to female ratio was 11:10 and most showed initial symptoms in the first decade of life.Most patients presented toe/clumsy walking or running as the onset symptom,followed by muscle weakness or atrophy.Creatine kinase levels were elevated in fourteen patients and were normal in three.Eighteen patients developed respiratory insufficiency during the disease course and thirteen(one could not tolerate non-invasive assisted ventilation)required non-invasive assisted ventilation for treatment.Except for one not reported,heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation.Z-disk streaming and aggregation could be observed in most of the patients’muscle histology.In the long-term follow-up,five patients died of cardiac or respiratory failure.Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy.It should be considered as a rare differential diagnosis of hypercapnia.

Government Provides Vigorous Supports to the Improvement of Health Care in Qinghai Province,China

Qinghai province is located in the northeastern part of the Tibetan Plateau,and is an underdeveloped province of inland China.Chinese government gives high priority to the improvement of the wellbeing of Qinghai people,and have provided great supports in aspects of policy,funding,and professional resource to the development of health care and medical system in Qinghai.Great progress has been made,and wellness of residents in Qinghai has been significantly improved.This article reviews the strategies and measures from central and provincial government in improving health care of Qinghai province under the leadership of the Communist Party of China.

The Great Imitator:Atypical Cutaneous Manifestations of Primary Syphilitic Chancre

Objective To analyze the reasons of misdiagnosis of primary syphilitic chancre and strengthen the understanding of atypical features of this disease.Methods A case series of twenty-seven challenging primary syphilis patients who were not immediately recognized as chancre was included in our study.The clinical data including the patients’age,sex,skin lesions,HIV status,syphilis serologic test results,treatment,and follow-up results were collected.Hematoxylin-eosin and immunohistochemistry staining of skin biopsy sections were reviewed.Results Four female cases with extragenital chancres presenting as erythema or erosive skin lesions on the nipple were misdiagnosed as Paget’s disease or eczema.The disorder of missed or misdiagnosed male cases manifested as syphilitic balanitis or multiple chancres on the penis root and adjacent pubis rather than coronal sulcus or frenum.Patients with nonreactive nontreponemal tests at initial presentation were also easily missed or misdiagnosed.Conclusion Primary syphilis presenting as multiple lesions rather than a single chancre,at atypical locations,or with a nonreactive nontreponemal test result,tends to be missed or misdiagnosed.

成人弥漫性低级别胶质瘤研究进展

在对于高级别胶质瘤研究逐步深入的同时，学者们也在关注成人低级别胶质瘤（LGG）。在分子肿瘤学、神经影像学和治疗学的推动下，对于成人LGG及其自然病史有了更深入的理解，形成了一些新的诊疗思路。

20例首诊于眼科的特发性颅内压增高症

特发性颅内压增高症（HH）是一类不明病因引起的颅内压力升高性疾病。

第352例发热-多发淋巴结肿大-M蛋白阳性

病历摘要患者男，41岁，因发热、淋巴结肿大19个月人院。患者于2013年3月无诱因出现干咳，伴午后低热，最高体温〈38℃，可自行退热，无咳嗽、咳痰、腹痛、腹泻、关节肿痛；伴有双侧颈根部多发淋巴结肿大，直径1～2cm，质地中等，无红肿、压痛，活动可。