1.Expert Consensus on the Diagnosis and Treatment of Acute Intermittent Porphyria in China(2024)
Songyun ZHANG ; Shuyang ZHANG ; Ming LIU ; Hongmei SONG
JOURNAL OF RARE DISEASES 2025;4(2):232-247
Acute intermittent porphyria(AIP)is a rare autosomal dominant disorder caused by reduced activity of hydroxymethylbilane synthase(HMBS),or porphobilinogen deaminase(PBGD),the third enzyme in the heme biosynthesis pathway,with very low penetrance.AIP mainly affects women and is triggered by vari-ous factors,leading to acute neurovisceral symptoms such as abdominal pain,nausea and vomiting,intestinal obstruction,constipation,and in severe cases,epilepsy,weakness of the limbs,and psychiatric disturbances.Recurrent attacks may result in chronic injuries.Due to nonspecific symptoms and limited clinical awareness,AIP is often misdiagnosed,contributing to high morbidity and mortality.To standardize diagnosis and treatment practices,the Chinese Society of Rare Diseases,in collaboration with the Study Group of Endocrinology Rare Diseases,the Chinese Society of Endocrinology,and the Chinese Pharmacists Association Rare Diseases Medi-cation Working Committee,developed the Expert Consensus on the Diagnosis and Treatment of Acute Intermittent Porphyria in China(2024).This consensus was based on a review of 116 relevant studies,with a professional panel of 27 experts using the Delphi method to reach agreement on 23 key recommendations,aiming to guide standardized clinical practice in the diagnosis,treatment,evaluation,and prevention of AIP.
2.2023 China minimally invasive cardiovascular surgery statistics and future development thinking
Yao WANG ; Ye YANG ; Shuyang LU ; Zhe ZHENG ; Nianguo DONG ; Huiming GUO ; Song XUE ; Liming LIU ; Yingqiang GUO ; Xuezeng XU ; Lai WEI ; Chunsheng WANG
Chinese Journal of Thoracic and Cardiovascular Surgery 2025;41(6):321-327
Objective:Committee of Minimally Invasive Cardiovascular Surgery(CMICS) conducts an annual summary of minimally invasive cardiovascular surgery procedures performed throughout the country, which includes a comprehensive survey of the total number of minimally invasive procedures by region and the distribution of minimally invasive procedures by hospital. Since CMICS first published the 2018-2019 China Minimally Invasive Cardiovascular Surgery Data White Paper in 2020, the report has received great attention from peers within and outside the industry. In this statistical report, CMICS will focus on publishing the data related to minimally invasive cardiovascular surgery in China from 2021 to 2023 for reference and use by industry peers.
3.Expert Consensus on the Diagnosis and Treatment of Acute Intermittent Porphyria in China(2024)
Songyun ZHANG ; Shuyang ZHANG ; Ming LIU ; Hongmei SONG
JOURNAL OF RARE DISEASES 2025;4(2):232-247
Acute intermittent porphyria(AIP)is a rare autosomal dominant disorder caused by reduced activity of hydroxymethylbilane synthase(HMBS),or porphobilinogen deaminase(PBGD),the third enzyme in the heme biosynthesis pathway,with very low penetrance.AIP mainly affects women and is triggered by vari-ous factors,leading to acute neurovisceral symptoms such as abdominal pain,nausea and vomiting,intestinal obstruction,constipation,and in severe cases,epilepsy,weakness of the limbs,and psychiatric disturbances.Recurrent attacks may result in chronic injuries.Due to nonspecific symptoms and limited clinical awareness,AIP is often misdiagnosed,contributing to high morbidity and mortality.To standardize diagnosis and treatment practices,the Chinese Society of Rare Diseases,in collaboration with the Study Group of Endocrinology Rare Diseases,the Chinese Society of Endocrinology,and the Chinese Pharmacists Association Rare Diseases Medi-cation Working Committee,developed the Expert Consensus on the Diagnosis and Treatment of Acute Intermittent Porphyria in China(2024).This consensus was based on a review of 116 relevant studies,with a professional panel of 27 experts using the Delphi method to reach agreement on 23 key recommendations,aiming to guide standardized clinical practice in the diagnosis,treatment,evaluation,and prevention of AIP.
4.2023 China minimally invasive cardiovascular surgery statistics and future development thinking
Yao WANG ; Ye YANG ; Shuyang LU ; Zhe ZHENG ; Nianguo DONG ; Huiming GUO ; Song XUE ; Liming LIU ; Yingqiang GUO ; Xuezeng XU ; Lai WEI ; Chunsheng WANG
Chinese Journal of Thoracic and Cardiovascular Surgery 2025;41(6):321-327
Objective:Committee of Minimally Invasive Cardiovascular Surgery(CMICS) conducts an annual summary of minimally invasive cardiovascular surgery procedures performed throughout the country, which includes a comprehensive survey of the total number of minimally invasive procedures by region and the distribution of minimally invasive procedures by hospital. Since CMICS first published the 2018-2019 China Minimally Invasive Cardiovascular Surgery Data White Paper in 2020, the report has received great attention from peers within and outside the industry. In this statistical report, CMICS will focus on publishing the data related to minimally invasive cardiovascular surgery in China from 2021 to 2023 for reference and use by industry peers.
5.Comparison of radionuclide myocardial perfusion/metabolic imaging with cardiac magnetic resonance for assessment of myocardial viability: current status and progress
Shuyang SONG ; Lei WANG ; Wei FANG
Chinese Journal of Nuclear Medicine and Molecular Imaging 2024;44(5):317-320
Radionuclide myocardial perfusion/metabolic imaging and cardiac MR play critical roles in assessing myocardial viability of patients with ischemic heart disease as noninvasive modalities. Given the technical difference between two imaging modalities, each of them has its unique strength to ascertain what causes the injured myocardium and differentiate the viable myocardium from the scarred myocardium. The purpose of this review is to compare the capability of two methods in assessment of myocardial viability, including summarize their consistency and discrepancy, as well as to explore the clinical significance to combine them serving as an essential tool to assist decision making for coronary revascularization comprehensively and accurately.
6.Application of artificial neural network algorithm in pathological diagnosis and prognosis prediction of digestive tract malignant tumors.
Ya XIAO ; Shuyang WANG ; Ren LING ; Yufei SONG
Journal of Zhejiang University. Medical sciences 2023;52(2):243-248
The application of artificial neural network algorithm in pathological diagnosis of gastrointestinal malignant tumors has become a research hotspot. In the previous studies, the algorithm research mainly focused on the model development based on convolutional neural networks, while only a few studies used the combination of convolutional neural networks and recurrent neural networks. The research contents included classical histopathological diagnosis and molecular typing of malignant tumors, and the prediction of patient prognosis by utilizing artificial neural networks. This article reviews the research progress on artificial neural network algorithm in the pathological diagnosis and prognosis prediction of digestive tract malignant tumors.
Humans
;
Neural Networks, Computer
;
Algorithms
;
Prognosis
;
Gastrointestinal Neoplasms/diagnosis*
7.Diagnostic and Therapeutic Strategy Updates of Rare Oncogenic Mutations in Chinese Society of Clinical Oncology Guidelines on Diagnosis and Treatment of Non-small Cell Lung Cancer (2023 Edition)
Yingge LI ; Yi DONG ; Shuyang YU ; Yingmei WEN ; Qibin SONG ; Yi YAO
Cancer Research on Prevention and Treatment 2023;50(12):1232-1236
The Chinese Society of Clinical Oncology (CSCO) issued the new version of the guidelines on diagnosis and treatment of NSCLC in April 2023.The new version updated the diagnostic and therapeutic strategy of rare oncogenic mutations, including
8.Application of body temperature rinse in percutaneous transforaminal endoscopic lumbar discectomy through intervertebral approach.
Li-Dong ZHANG ; Cheng-Liang ZHANG ; Da-Jiang SONG ; Gang CHEN ; Yan-Lei ZHUANG
China Journal of Orthopaedics and Traumatology 2023;36(9):854-858
OBJECTIVE:
To investigate the effects of two types of temperature rinses on body temperature, inflammatory cytokine levels, and bleeding volume in percutaneous endoscopic lumbar discectomy.
METHODS:
Eighty patients underwent percutaneous endoscopic lumbar discectomy from January 2018 to December 2020 were selected and divided into experimental group (40 cases) and control group(40 cases). In experimental group, there were 19 males and 21 females, aged (38.8±9.8) years old;7patients on L4,5 and 33 patients on L5S1;Body msss index(BMI) was (27.8±7.2) kg·m-2. In contral group, there were 18 males and 22 females, aged (41.5±10.9) years old, 5 patients on L4,5 and 35 patients on L5S1;BMI was (26.4±6.2) kg·m-2. The patients in the control group were received normal saline rinse at room temperature, and the patients in the experimental group were received normal saline rinse heated to 37 ℃. Body temperature, chills, nausea, vomiting, and other adverse reactions were recorded. The levels of tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6) and interleukin-10 (IL-10) in two groups were recorded before and 2 hours after operation. Visual analogue scale (VAS) was used to evaluate the degree of lumbar pain in two groups before and 2 hours after surgery. Fibrinolytic-coagulation indexes with preoperative and 2 hours after surgery, including the D-dimer (DD), fibrinogen degradation products (FDP), activated partial thrombin time (APTT) and prothrombin time (PT) were recorder. Operation time and blood loss in two groups were recorded.
RESULTS:
The body temperature of both groups showed a downward trend, while the body temperature of the control group was lower than that of the experimental group. The levels of TNF-α, IL-6 and IL-10 in two groups were increased 2 hours after surgery compared with those before surgery(P<0.05), while the levels in experimental group were lower than those in control group(P<0.05). Postoperative VAS in experimental group 2.19±1.13 was significantly lower than that in the control group 3.38±1.35(P<0.05). The levels of DD and FDP at 2 hours after surgery in both groups were higher than those before surgery (P<0.05), while the levels of DD and FDP in the experimental group were higher than those in the control group (P<0.05). There was no significant difference in APTT and PT levels between two groups after operation (P>0.05). The blood loss in the experimental group of (45.2±14.1) ml was lower than that in the control group of (59.52±15.6) ml. The operation time of experimental group (46.7±13.8) min was less than that of control group (58.3±15.2) min(P<0.05).
CONCLUSION
Body temperature rinse can reduce the incidence of adverse reactions, alleviate local inflammatory reactions, reduce intraoperative blood loss and shorten the operation time.
Female
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Male
;
Humans
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Adult
;
Middle Aged
;
Diskectomy, Percutaneous
;
Interleukin-10
;
Body Temperature
;
Interleukin-6
;
Saline Solution
;
Tumor Necrosis Factor-alpha
;
Intervertebral Disc Displacement/surgery*
;
Lumbar Vertebrae/surgery*
;
Diskectomy
9.Comparison of corneal refractive power and astigmatism measured by CASIA2, IOLMaster 700 and Pentacam in cataract patients
Tingting WANG ; Shuyang WANG ; Min ZHANG ; Luning QIN ; Song LIN
Chinese Journal of Experimental Ophthalmology 2022;40(11):1055-1061
Objective:To compare the difference and consistency of anterior corneal surface and total corneal refractive power and astigmatism measured by CASIA2, IOLMaster 700 and Pentacam in patients with age-related cataract.Methods:A diagnostic test was conducted.Two-hundred patients (200 eyes) with age-related cataract were enrolled in Tianjin Medical University Eye Hospital from March to April 2021.The steep keratometry (Ks), flat keratometry (Kf), mean keratometry (Km), degree and axis of astigmatism of the anterior and the total corneal surface of patients were measured by CASIA2, IOLMaster 700 and Pentacam, respectively.The astigmatism was transformed into J0 and J45 by Fourier transform formula.The differences and correlation of the measurements obtained with the three instruments were analyzed by one-way repeated measures analysis of variance and Pearson correlation analysis.The consistency was evaluated by Bland-Altman test.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Tianjin Medical University Eye Hospital (No.2021KY-07).Results:There were statistically significant differences in anterior corneal surface Kf and J0 measured by the three instruments ( F=18.563, 16.172; both at P=0.001). The Kf measured by CASIA2 was significantly higher than that measured by IOLMaster 700, and the J0 measured by IOLMaster 700 was significantly higher than that measured by Pentacam (both at P<0.05). There were statistically significant differences in total corneal Ks, Kf, Km and J0 measured by the three instruments, which from IOLMaster 700 were the largest, followed by CASIA2, then Pentacam ( F=1 300.447, 1 274.117, 1 609.713, 10.372; all at P=0.001). Pearson correlation analysis showed that the corneal refractive power measured by the three instruments was highly correlated (all at r>0.935, P<0.01), and the correlation of astigmatism values was weaker than the corneal refractive power ( r=0.623-0.908, all at P<0.01). Bland-Altman analysis showed that the three instruments had good consistency in measuring the anterior corneal surface refractive power, anterior corneal surface astigmatism and total corneal astigmatism, which were clinically acceptable, while the consistency of total corneal refractive power measurement was poor.The difference in measuring total corneal refractive power was large between IOLMaster 700 and Pentacam, and relatively small between CASIA2 and Pentacam. Conclusions:The consistency of CASIA2, IOLMaster 700 and Pentacam is good in measuring the anterior corneal surface refractive power of patients with age-related cataract, which can be substitutable, but poor in measuring the total corneal refractive power.The total corneal refractive power measurement from IOLMaster700 is the largest, the smallest from Pentacam, which is not recommended to be clinically exchangeable.
10.Correlation between curative effect and 5-HTTLPR polymorphism in treatment of diarrhea-predominant irritable bowel syndrome with acupuncture for regulating
Jing GUO ; Jian-Hua SUN ; Lu CHEN ; Hao GENG ; Xiao-Liang WU ; Ya-Fang SONG ; Guo-Hui YANG ; Rong-Rong SHEN ; Min DING ; Jin LU ; Lian LIU ; Xiang-Dong FANG ; Li-Xia PEI
Chinese Acupuncture & Moxibustion 2021;41(4):365-370
OBJECTIVE:
To compare the curative effect on diarrhea-predominant irritable bowel syndrome (IBS-D) between acupuncture for regulating
METHODS:
A total of 231 patients with IBS-D were randomized into an acupuncture group (154 cases) and a western medication group (77 cases) at the ratio of 2 to 1. In the acupuncture group, acupuncture was applied to acupoint regimen for regulating
RESULTS:
After treatment and in follow-up, the total scores of IBS-SSS in the patients of the two groups were all reduced as compared with those before treatment (
CONCLUSION
Acupuncture for regulating
Acupuncture Therapy
;
Diarrhea/therapy*
;
Humans
;
Irritable Bowel Syndrome/therapy*
;
Quality of Life
;
Serotonin Plasma Membrane Transport Proteins/genetics*
;
Spleen
;
Treatment Outcome

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