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Chinese Medical Journal 2022年第2期135卷 216-218页

作者： Hui Wang Haoran Chen Yin Liu Xin Yan Yujuan Gao Hourong Cai Jinghong Dai Department of Pulmonary and Critical Care Medicine Nanjing University Medical School Affiliated Drum Tower HospitalNanjingJiangsu 210008China

The term of interstitial pneumonia with autoimmune features(IPAF),based on a combination of clinical,serological,and morphological domains(specific chest imaging features,histopathological features,or multi-compartment involvement,including non-specific interstitial pneumonia[NSIP],organizing pneumonia,NSIP with organizing pneumonia overlap and lymphoid interstitial pneumonia).

关键词： involvement Idiopathic clinical

A novel treatment workflow of Idiopathic hypereosinophilic syndrome:a single-center retrospective cohort study

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Chinese Medical Journal 2022年第21期135卷 2602-2604页

作者： Rui Tang Shubin Lei Shuyun Wang Yuanyuan Guan Hong Li Department of Allergy Peking Union Medical College Hospital(PUMCH)Chinese Academy of Medical Sciences and Peking Union Medical College(CAMS and PUMC)Beijing 100730China Eight-Year Program of Clinical Medicine PUMCHCAMS and PUMCBeijing 100730China Department of Allergy The First Affiliated Yantai Yuhuangding Hospital of Qingdao UniversityYantaiShandong 264099China Department of Allergy The First Affiliated Hospital of Harbin Medical UniversityHarbinHeilongjiang 150007China

Hypereosinophilic syndrome(HES),a rare systemic disease,was first described in 1968.As a subtype of HES,Idiopathic hypereosinophilic syndrome(IHES)is defined as hypereosinophilia of unknown cause,excluding tumor,infection,allergy,and immune system disease.In most cases,more than 1 organ is affected in patients with IHES.[1]The first-line drug for the treatment of IHES is glucocorticoid,which is effective for both hypereosinophilia and clinical manifestations.[2]However,when the outcome of hormone treatment is unsatisfactory,immunosuppressive or antineoplastic agents can also be administered.

关键词： eosinophilic treatment Idiopathic

SEMA4D acts as a novel oligogenic pathogenic gene of Idiopathic hypogonadotropic hypogonadism through thePlexinB1/MET/RND1/RHOA/RAF1/MAPK signaling axis

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Genes & Diseases 2023年第1期10卷 65-68页

作者： Daoqi Wang Yonghua Niu Jiahong Tan Jiaxin Wang Le Ling Yinwei Chen Jjianan Gong Hao Xu Qing Ling Jianhe Liu Jihong Liu Department of Urology The Second Affiliated Hospital of KunmingMedical UniversityKunmingYunnan 650033China Department of Urology Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubei 430030China Department of Pediatric Surgery Tongji HospitalTongji Medical CollegeWuhanHubei 430030China Department of Obstetrics and Gynecology The First People's Hospital of Yunnan ProvinceKunmingYunnan 650034China Department of Urology First Affiliated Hospital of Harbin Medical UniversityHarbinHeilongjiang 150007China

Idiopathic hypogonadotropic hypogonadism(IHH)is a rare genetic disease with clinical and genetic heterogeneity.This study aimed to investigate a novel causal gene of IHH and a homozygous mutation(p.Ala515Val)in SEMA4D... 详细信息

关键词： heterogeneity. clinical Idiopathic

Long-term management of Idiopathic cough with ultrasound-guided pulsed radiofrequency ablation of the phrenic nerve

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Chinese Medical Journal 2022年第12期135卷 1511-1512页

作者： Yoo Jung Park Yong Ho Lee Jinwoo Shim Jin Young Lee Hyunhwa Lee Hue Jung Park Department of Anesthesiology and Pain Medicine St.Vincent’s HospitalCollege of MedicineThe Catholic University of KoreaSuwonRepublic of Korea Department of Anesthesiology and Pain Medicine Seoul St.Mary’s HospitalCollege of MedicineThe Catholic University of KoreaSeoulRepublic of Korea Department of Anesthesiology and Pain Medicine Samsung Medical CenterSungkyunkwan UniversitySchool of MedicineSeoulRepublic of Korea

To the Editor:Idiopathic cough is associated with laryngeal hypersensitivity and chronic pain syndromes.Idiopathic cough can be diagnosed when patients have no cause of chronic cough after treatment of cough.Patients with chronic cough experience impaired quality of life(QOL),interruption of daily activity,and depression.Therefore,better approaches to refractory chronic cough areneeded.

关键词： impaired Idiopathic cough

Identification of autophagy-related genes in Idiopathic pulmonary fibrosis using bioinformatics methods

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Chinese Medical Journal 2022年第3期135卷 365-367页

作者： Shulei Sun Lixia Dong Shuo Li Jie Cao Department of Respiratory and Critical Care Medicine Tianjin Medical University General HospitalTianjin 300052China

To the Editor:Idiopathic pulmonary fibrosis(IPF)is a chronic,progressive,and fibrotic lung disease.The clinical processes of IPF include dyspnea,decreased lung function,and cough.The median survival time of patients with IPF is approximately 3 years.The 5-year survival rate is less than 30%.[1]Although some progress has been made in anti-fibrotic therapy,IPF is still an incurable disease.Therefore,it is necessary to explore the pathological process of IPF from the perspective of cell biology,and these explorations can provide a basis for the treatment of IPF.

关键词： lung clinical Idiopathic

Direct medical costs of hospitalized patients with Idiopathic pulmonary fibrosis in a tertiary hospital in China

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Chinese Medical Journal 2020年第20期133卷 2498-2500页

作者： Xiao-Fen Zheng Bing-Bing Xie Yan Liu Ming Zhu Shu Zhang Cheng-Jun Ban Jing Geng Ding-Yuan Jiang Yan-Hong Ren Hua-Ping Dai Chen Wang Department of Pulmonary and Critical Care Medicine Center of Respiratory MedicineChina-Japan Friendship HospitalNational Clinical Research Center for Respiratory DiseasesInstitute of Respiratory MedicineChinese Academy of Medical SciencesPeking Union Medical CollegeBeijing 100029China The Second Affiliated Hospital of Zhejiang Chinese Medical University HangzhouZhejiang 310000China Department of Pulmonary and Critical Care Medicine Beijing Chao-Yang HospitalCapital Medical UniversityBeijing 100012China Department of Respiratory Medicine Dongzhimen HospitalBeijing University of Chinese MedicineBeijing 100700China

Idiopathic pulmonary fibrosis(IPF)incidence shows wide variations in different countries,ranging from 3 to 9 per 100,000 inhabitants per year in North America and Europe,while in South America and Asia,incidence is lo... 详细信息

关键词： patients pulmonary Idiopathic

Assessing gray matter volume in patients with Idiopathic rapid eye movement sleep behavior disorder

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Neural Regeneration Research 2019年第5期14卷 868-875页

作者： Xian-Hua Han Xiu-Ming Li Wei-Jun Tang Huan Yu Ping Wu Jing-Jie Ge Jian Wang Chuan-Tao Zuo Kuang-Yu Shi PET Center Huashan Hospital Fudan University Department of Radiology Huashan Hospital Fudan University Department of Neurology Huashan Hospital Fudan University Department of Nuclear Medicine Technical University Munich

Idiopathic rapid eye movement sleep behavior disorder(iRBD) is often a precursor to neurodegenerative disease. However, voxel-based morphological studies evaluating structural abnormalities in the brains of iRBD patients are relatively rare. This study aimed to explore cerebral structural alterations using magnetic resonance imaging and to determine their association with clinical parameters in iRBD patients. Brain structural T1-weighted MRI scans were acquired from 19 polysomnogram-confirmed iRBD patients(male:female 16:3; mean age 66.6 ± 7.0 years) and 20 age-matched healthy controls(male:female 5:15; mean age 63.7 ± 5.9 years). Gray matter volume(GMV) data were analyzed based on Statistical Parametric Mapping 8, using a voxel-based morphometry method and two-sample t-test and multiple regression analysis. Compared with controls, iRBD patients had increased GMV in the middle temporal gyrus and cerebellar posterior lobe, but decreased GMV in the Rolandic operculum, postcentral gyrus, insular lobe, cingulate gyrus, precuneus, rectus gyrus, and superior frontal gyrus. iRBD duration was positively correlated with GMV in the precuneus, cuneus, superior parietal gyrus, postcentral gyrus, posterior cingulate gyrus, hippocampus, lingual gyrus, middle occipital gyrus, middle temporal gyrus, and cerebellum posterior lobe. Furthermore, phasic chin electromyographic activity was positively correlated with GMV in the hippocampus, precuneus, fusiform gyrus, precentral gyrus, superior frontal gyrus, cuneus, inferior parietal lobule, angular gyrus, superior parietal gyrus, paracentral lobule, and cerebellar posterior lobe. There were no significant negative correlations of brain GMV with disease duration or electromyographic activity in iRBD patients. These findings expand the spectrum of known gray matter modifications in iRBD patients and provide evidence of a correlation between brain dysfunction and clinical manifestations in such patients. The protocol was approved by the Ethics Committee of Huashan Hospital(approval No.

关键词： nerve regeneration Idiopathic rapid eye movement sleep behavior disorder synucleinopathies magnetic resonance imaging gray matter volume statistic parametric mapping voxel-based morphometry structure Parkinson’s disease neurodegenerative diseases neural regeneration

Acute exacerbation of Idiopathic pulmonary fibrosis: usual interstitial pneumonitis vs.possible usual interstitial pneumonitis pattern

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Chinese Medical Journal 2019年第18期132卷 2177-2184页

作者： Meng-Shu Cao Jian Sheng Tian-Zhen Wang Xiao-Hua Qiu Dong-Mei Wang Yang Wang Yong-Long Xiao Hou-Rong Cai Department of Respiratory and Critical Care Medicine Nanjing Drum Tower Hospital The Affiliated Hospital of Nanjing University Medical School Nanjing Jiangsu 210008 China Department of Respiratory and Critical Care Medicine Nanjing Drum Tower Hospital Nanjing Medical University Nanjing Jiangsu 210008 China Department of Radiology Nanjing Drum Tower Hospital The Affiliated Hospital of Nanjing University Medical School Nanjing Jiangsu 210008 China

Background:The prognosis of acute exacerbation of Idiopathic pulmonary fibrosis (AE-IPF) is very poor with a high mortality.The aim of this study was to describe the clinical features and survival of patients with AE-IPF with usual pulmonary fibrosis (UIP) and possible UIP (P-UIP) pattern on chest high resolution computed tomography (HRCT).Methods:This retrospective study included 107 patients with AE-IPF admitted to Nanjing Drum Tower Hospital from January 2010 to December 2016.The subjects were divided into UIP (n =86) and P-UIP group (n=21) based on chest HRCT.Continuous variables were analyzed using Student's t test or Mann-Whimey U test.Categorical variables were analyzed using x2 test.Log-rank test was used for the survival analysis.Cox proportional models evaluated the risk factors for AE occurrence and survival.Results:The male,older patients,previous N-acetylcysteine use,elevated white blood cell (WBC) counts,and microbiology infection were more common in the UIP group than the P-UIP group (X2 =13.567,P Idiopathic interstitial pneumonia (ⅡP) was significantly higher than P-UIP pattern (X2 =40.011,P < 0.001).Smoking was the risk factor for AE within 6 months after IPF diagnosis in the UIP group.The cumulative proportion survival of 30-days was significantly higher in the UIP group compared with the P-UIP group (x2 =5.489,P =0.019) despite of the similar overall survival in the two groups.Multivariate Cox regression analysis indicated WBC count,partial pressure of oxygen in artery (PaO2)/ffactional concentration of inspired oxygen (FiOz),and computed tomography (CT) score were the independent predictors for survival in the UIP group (hazard ratio [HR]:1.070,95% confidential interval [CI]:1.027-1.114,P=0.001;HR:0.992,95% CI:0.986-0.997,P=0.002;and HR:1.649,95% CI:1.253-2.171,P < 0.001,respectively).Conclusions:AE occurrence of UIP patients in IIP was significantly more t

关键词： Idiopathic pulmonary fibrosis Acute exacerbation Usual interstitial pneumonitis

Traditional Chinese medicine in the treatment of Idiopathic pulmonary fibrosis based on syndrome differentiation:Study protocol of an exploratory trial

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Journal of Integrative Medicine 2020年第2期18卷 163-168页

作者： Xue-qing Yu Shu-guang Yang Yang Xie Jian-sheng Li Department of Respiratory Diseases The First Affiliated Hospital of Henan University of Chinese MedicineZhengzhouHenan Province 450000China Co-construction Collaborative Innovation Center for Chinese Medicine and Respiratory Diseases by Henan&Education Ministry of P.R.China Henan University of Chinese MedicineZhengzhouHenan Province 450046China Henan Key Laboratory of Chinese Medicine for Respiratory Diseases Henan University of Chinese MedicineZhengzhouHenan Province 450046China

Background: Idiopathic pulmonary fibrosis(IPF) has a poor prognosis and is often a kind of heavy financial burden to patients. Currently, few treatments are available for IPF. Clinical practice of traditional Chinese medicine(TCM), using a syndrome differentiation approach, offers some treatment success in IPF. However, there is no sufficient evidence-based study of the role of TCM in IPF management to make strong conclusions. This study evaluates the efficacy and safety of TCM in the treatment of IPF.Methods and design: A multicenter, exploratory, randomized, double-blind and placebo-controlled trial is planned. A total of 80 patients will be enrolled in the study, which will include 26 weeks of treatment.Participants will be randomly assigned into TCM group or control group in a 1:1 ratio. The TCM group will be given TCM granules based on syndrome differentiation. Formulae include Bao-fei Hua-xian granule for lung qi deficiency, Jin-shui Huan-xian granule for lung-kidney qi deficiency and Yang-qing Kang-xian granule for yin deficiency and inner heat. The control group will be given a corresponding TCM granule placebo. The efficacy and safety of interventions will be evaluated by the outcome variables,including frequencies of acute exacerbations, pulmonary function, clinical symptoms, dyspnea, healthrelated quality of life(HRQo L), 6-minute walk distance and safety indicators.Discussion: It is hypothesized that TCM will decrease the frequency of adverse events, improve pulmonary function and HRQo L, based on our clinical experience. This trial is the first study of TCM treatment in IPF that is based on syndrome differentiation and will evaluate the efficacy and safety of TCM in IPF.

关键词： Idiopathic pulmonary fibrosis Traditional Chinese medicine Syndrome differentiation Protocol

Longitudinal observation of ten family members with Idiopathic basal ganglia calcification: A case report

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World Journal of Clinical Cases 2019年第12期7卷 1483-1491页

作者： Seiju Kobayashi Kumiko Utsumi Masaru Tateno Tomo Iwamoto Tomonori Murayama Hitoshi Sohma Wataru Ukai Eri Hashimoto Chiaki Kawanishi Shinyukai Nakae Hospital Sapporo 0010022 Japan Department of Neuropsychiatry Sapporo Medical University Graduate School of Medicine Sapporo 0608543 Japan Department of Psychiatry Sunagawa City Medical Center Sunagawa 0730196 Japan Tokiwa Child Development Center Tokiwa Hospital Sapporo Japan Department of Neuropsychiatry Sapporo Medical University Graduate School of Medicine Sapporo 0050853 Japan Department of Educational Development Sapporo Medical University Center for Medical Education Sapporo 0608543 Japan Department of Biomedical Engineering Sapporo Medical University School of Medicine Sapporo 0608543 Japan

BACKGROUND Familial Idiopathic basal ganglia calcification (FIBGC) is a rare autosomal dominant disorder that causes bilateral calcification of the basal ganglia and/or cerebellar dentate nucleus, among other locations. CASE SUMMARY The aim of this study is to report 10 cases of FIBGC observed in a single family. Seven patients showed calcification on their computed tomography scan, and all of these patients carried the SLC20A2 mutation. However, individuals without the mutation did not show calcification. Three patients among the 7 with calcification were symptomatic, while the remaining 4 patients were asymptomatic. Additionally, we longitudinally observed 10 subjects for ten years. In this paper, we mainly focus on the clinical course and neuroradiological findings in the proband and her son.CONCLUSION The accumulation of more case reports and further studies related to the manifestation of FIBGC are needed.

关键词： Idiopathic basal ganglia calcification Fahr’s disease SLC20A2 Diffuse neurofibrillary tangles with calcification Single-photon emission computed tomography Case report