1.A Case Report of Multidisciplinary Diagnosis and Treatment of a Patient with Tuberous Sclerosis Complex and Multi-Organ Involvement
Hua ZHENG ; Yunfei ZHI ; Lujing YING ; Lan ZHU ; Mingliang JI ; Ze LIANG ; Jiangshan WANG ; Haifeng SHI ; Weihong ZHANG ; Mengsu XIAO ; Yushi ZHANG ; Kaifeng XU ; Zhaohui LU ; Yaping LIU ; Ruiyi XU ; Huijuan ZHU ; Li WEN ; Yan ZHANG ; Gang CHEN ; Limeng CHEN
JOURNAL OF RARE DISEASES 2024;3(1):79-86
Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.
2.Synthesis and characterization of matrix metalloproteinase-responsive BDNF controlled-release materials
Jun-Ru HEI ; Cui WANG ; Meng-Wen SONG ; Sheng-Qiang XIE ; Bing-Xian WANG ; Xiao-Juan LAN ; Han-Bo ZHANG ; Gang CHENG ; Zhi-Qiang LIU ; Xi-Qin YANG ; Jian-Ning ZHANG
Medical Journal of Chinese People's Liberation Army 2024;49(11):1319-1326
Objective To develop a matrix metalloproteinase(MMP)-responsive hyaluronic acid(HA)-based controlled-release material for brain-derived neurotrophic factor(BDNF)to provide a novel therapeutic strategy for intervention and repair of traumatic brain injury(TBI).Methods HA was modified with amination,followed by condensation with Suflo-SMCC carboxyl group to form amide,and then linked with glutathione(GSH)to synthesize HA-GSH.The recombinant glutathione S-transferase(GST)-tissue inhibitor of metalloproteinase(TIMP)-BDNF(GST-TIMP-BDNF)expression plasmid was constructed using molecular cloning technique with double enzyme digestion by Bam H Ⅰ and Eco R Ⅰ.The recombinant GST-TIMP-BDNF protein was expressed in the Escherichia coli prokaryotic expression system,and purified by ion exchange chromatography,confirmed by Western blotting.MMP diluents were supplemented with PBS,MMP inhibitor marimastat,and varing concentrations(0.4,0.6,0.8 mg/ml)of GST-TIMP-BDNF or GST-BDNF.MMP-2 activity was analyzed using an MMP activity detection kit to evaluate the inhibitory effect of the recombinant protein on MMP.Primary rat neurons were extracted and cultured to establish an iron death model induced by RSL3.The effect of recombinant protein GST-TIMP-BDNF on neuronal injury was detected by immunofluorescence staining.Results MRI hydrogen spectrum identification confirmed the successful synthesis of HA-GSH.Western blotting results showed the successful expression of the recombinant protein GST-TIMP-BDNF containing the GST tag using the E.coli prokaryotic expression system.MMP activity detection results indicated that the recombinant protein GST-TIMP-BDNF had a superior inhibitory effect on MMP-2 activity compared to GST-BDNF(P<0.05).Immunofluorescence staining results showed a significant increase in fluorescence intensity in rat neurons treated with GST-TIMP-BDNF after RSL3 induction(P<0.05).Conclusion A MMP-responsive HA-based BDNF controlled-release material has been successfully developed,exhibiting a protective effect on neuron damage.
3.In-silico annotation of the chemical composition of Tibetan tea and its mechanism on antioxidant and lipidlowering in mice
Ning WANG ; Linman LI ; Puyu ZHANG ; Muhammad Aamer MEHMOOD ; Chaohua LAN ; Tian GAN ; Zaixin LI ; Zhi ZHANG ; Kewei XU ; Shan MO ; Gang XIA ; Tao WU ; Hui ZHU
Nutrition Research and Practice 2023;17(4):682-697
BACKGROUND/OBJECTIVES:
Tibetan tea is a kind of dark tea, due to the inherent complexity of natural products, the chemical composition and beneficial effects of Tibetan tea are not fully understood. The objective of this study was to unravel the composition of Tibetan tea using knowledge-guided multilayer network (KGMN) techniques and explore its potential antioxidant and hypolipidemic mechanisms in mice.MATERIALS/METHODS: The C57BL/6J mice were continuously gavaged with Tibetan tea extract (T group), green tea extract (G group) and ddH 2 O (H group) for 15 days. The activity of total antioxidant capacity (T-AOC) and superoxide dismutase (SOD) in mice was detected.Transcriptome sequencing technology was used to investigate the molecular mechanisms underlying the antioxidant and lipid-lowering effects of Tibetan tea in mice. Furthermore, the expression levels of liver antioxidant and lipid metabolism related genes in various groups were detected by the real-time quantitative polymerase chain reaction (qPCR) method.
RESULTS:
The results showed that a total of 42 flavonoids are provisionally annotated in Tibetan tea using KGMN strategies. Tibetan tea significantly reduced body weight gain and increased T-AOC and SOD activities in mice compared with the H group. Based on the results of transcriptome and qPCR, it was confirmed that Tibetan tea could play a key role in antioxidant and lipid lowering by regulating oxidative stress and lipid metabolism related pathways such as insulin resistance, P53 signaling pathway, insulin signaling pathway, fatty acid elongation and fatty acid metabolism.
CONCLUSIONS
This study was the first to use computational tools to deeply explore the composition of Tibetan tea and revealed its potential antioxidant and hypolipidemic mechanisms, and it provides new insights into the composition and bioactivity of Tibetan tea.
4.A multicenter epidemiological study of acute bacterial meningitis in children.
Cai Yun WANG ; Hong Mei XU ; Jiao TIAN ; Si Qi HONG ; Gang LIU ; Si Xuan WANG ; Feng GAO ; Jing LIU ; Fu Rong LIU ; Hui YU ; Xia WU ; Bi Quan CHEN ; Fang Fang SHEN ; Guo ZHENG ; Jie YU ; Min SHU ; Lu LIU ; Li Jun DU ; Pei LI ; Zhi Wei XU ; Meng Quan ZHU ; Li Su HUANG ; He Yu HUANG ; Hai Bo LI ; Yuan Yuan HUANG ; Dong WANG ; Fang WU ; Song Ting BAI ; Jing Jing TANG ; Qing Wen SHAN ; Lian Cheng LAN ; Chun Hui ZHU ; Yan XIONG ; Jian Mei TIAN ; Jia Hui WU ; Jian Hua HAO ; Hui Ya ZHAO ; Ai Wei LIN ; Shuang Shuang SONG ; Dao Jiong LIN ; Qiong Hua ZHOU ; Yu Ping GUO ; Jin Zhun WU ; Xiao Qing YANG ; Xin Hua ZHANG ; Ying GUO ; Qing CAO ; Li Juan LUO ; Zhong Bin TAO ; Wen Kai YANG ; Yong Kang ZHOU ; Yuan CHEN ; Li Jie FENG ; Guo Long ZHU ; Yan Hong ZHANG ; Ping XUE ; Xiao Qin LI ; Zheng Zhen TANG ; De Hui ZHANG ; Xue Wen SU ; Zheng Hai QU ; Ying ZHANG ; Shi Yong ZHAO ; Zheng Hong QI ; Lin PANG ; Cai Ying WANG ; Hui Ling DENG ; Xing Lou LIU ; Ying Hu CHEN ; Sainan SHU
Chinese Journal of Pediatrics 2022;60(10):1045-1053
Objective: To analyze the clinical epidemiological characteristics including composition of pathogens , clinical characteristics, and disease prognosis acute bacterial meningitis (ABM) in Chinese children. Methods: A retrospective analysis was performed on the clinical and laboratory data of 1 610 children <15 years of age with ABM in 33 tertiary hospitals in China from January 2019 to December 2020. Patients were divided into different groups according to age,<28 days group, 28 days to <3 months group, 3 months to <1 year group, 1-<5 years of age group, 5-<15 years of age group; etiology confirmed group and clinically diagnosed group according to etiology diagnosis. Non-numeric variables were analyzed with the Chi-square test or Fisher's exact test, while non-normal distrituction numeric variables were compared with nonparametric test. Results: Among 1 610 children with ABM, 955 were male and 650 were female (5 cases were not provided with gender information), and the age of onset was 1.5 (0.5, 5.5) months. There were 588 cases age from <28 days, 462 cases age from 28 days to <3 months, 302 cases age from 3 months to <1 year of age group, 156 cases in the 1-<5 years of age and 101 cases in the 5-<15 years of age. The detection rates were 38.8% (95/245) and 31.5% (70/222) of Escherichia coli and 27.8% (68/245) and 35.1% (78/222) of Streptococcus agalactiae in infants younger than 28 days of age and 28 days to 3 months of age; the detection rates of Streptococcus pneumonia, Escherichia coli, and Streptococcus agalactiae were 34.3% (61/178), 14.0% (25/178) and 13.5% (24/178) in the 3 months of age to <1 year of age group; the dominant pathogens were Streptococcus pneumoniae and the detection rate were 67.9% (74/109) and 44.4% (16/36) in the 1-<5 years of age and 5-<15 years of age . There were 9.7% (19/195) strains of Escherichia coli producing ultra-broad-spectrum β-lactamases. The positive rates of cerebrospinal fluid (CSF) culture and blood culture were 32.2% (515/1 598) and 25.0% (400/1 598), while 38.2% (126/330)and 25.3% (21/83) in CSF metagenomics next generation sequencing and Streptococcus pneumoniae antigen detection. There were 4.3% (32/790) cases of which CSF white blood cell counts were normal in etiology confirmed group. Among 1 610 children with ABM, main intracranial imaging complications were subdural effusion and (or) empyema in 349 cases (21.7%), hydrocephalus in 233 cases (14.5%), brain abscess in 178 cases (11.1%), and other cerebrovascular diseases, including encephalomalacia, cerebral infarction, and encephalatrophy, in 174 cases (10.8%). Among the 166 cases (10.3%) with unfavorable outcome, 32 cases (2.0%) died among whom 24 cases died before 1 year of age, and 37 cases (2.3%) had recurrence among whom 25 cases had recurrence within 3 weeks. The incidences of subdural effusion and (or) empyema, brain abscess and ependymitis in the etiology confirmed group were significantly higher than those in the clinically diagnosed group (26.2% (207/790) vs. 17.3% (142/820), 13.0% (103/790) vs. 9.1% (75/820), 4.6% (36/790) vs. 2.7% (22/820), χ2=18.71, 6.20, 4.07, all P<0.05), but there was no significant difference in the unfavorable outcomes, mortility, and recurrence between these 2 groups (all P>0.05). Conclusions: The onset age of ABM in children is usually within 1 year of age, especially <3 months. The common pathogens in infants <3 months of age are Escherichia coli and Streptococcus agalactiae, and the dominant pathogen in infant ≥3 months is Streptococcus pneumoniae. Subdural effusion and (or) empyema and hydrocephalus are common complications. ABM should not be excluded even if CSF white blood cell counts is within normal range. Standardized bacteriological examination should be paid more attention to increase the pathogenic detection rate. Non-culture CSF detection methods may facilitate the pathogenic diagnosis.
Adolescent
;
Brain Abscess
;
Child
;
Child, Preschool
;
Escherichia coli
;
Female
;
Humans
;
Hydrocephalus
;
Infant
;
Infant, Newborn
;
Male
;
Meningitis, Bacterial/epidemiology*
;
Retrospective Studies
;
Streptococcus agalactiae
;
Streptococcus pneumoniae
;
Subdural Effusion
;
beta-Lactamases
5.Expert consensus on the use of human serum albumin in critically ill patients.
Yue-Tian YU ; Jiao LIU ; Bo HU ; Rui-Lan WANG ; Xiang-Hong YANG ; Xiu-Ling SHANG ; Gang WANG ; Chang-Song WANG ; Bai-Ling LI ; Ye GONG ; Sheng ZHANG ; Xin LI ; Lu WANG ; Min SHAO ; Mei MENG ; Feng ZHU ; You SHANG ; Qiang-Hong XU ; Zhi-Xiong WU ; De-Chang CHEN
Chinese Medical Journal 2021;134(14):1639-1654
6.Severe acute respiratory syndrome coronavirus 2 infection in renal transplant recipients: A case report.
Qiu Yu LI ; Qin CHENG ; Zhi Ling ZHAO ; Ni Ni DAI ; Lin ZENG ; Lan ZHU ; Wei GUO ; Chao LI ; Jun Hong WANG ; Shu LI ; Qing Gang GE ; Ning SHEN
Journal of Peking University(Health Sciences) 2020;52(4):780-784
The novel coronavirus is a newly discovered pathogen in late December 2019, and its source is currently unknown, which can lead to asymptomatic infection, new coronavirus pneumonia or serious complications, such as acute respiratory failure. Corona virus disease 2019 (COVID-19) is a new type of respiratory disease that is currently spreading all over the world and caused by this coronavirus. Its common symptoms are highly similar to those of other viruses, such as fever, cough and dyspnea. There is currently no vaccine or treatment for COVID-19. Everyone is susceptible to infection with this disease, and owing to the long-term use of immunosuppressants, the immunity of kidney transplant recipients is suppressed, and it is more likely to be infected with the disease. At present, its impact on kidney transplant recipients is unclear. This article reports the clinical features and therapeutic course of novel coronavirus infection in a patient after renal transplantation. A 37-year-old female patient who received a kidney transplant 6 months before was diagnosed with novel coronavirus pneumonia. The patient's symptoms (such as fever, chills, dry cough, muscle aches), laboratory tests (such as decreased white blood cell count, elevated liver enzymes and D-dimer, positive viral nucleic acid test), and chest CT (multiple left lower lung plaque ground glass shadow) were similar to those of non-transplanted novel coronavirus pneumonia patients. In terms of treatment, because the immunity of kidney transplant recipients has been suppressed for a long time, it is a very common strategy to suspend the use of immunosuppressive agents. Therefore, the patient immediately discontinued the immunosuppressive agent after admission, so that she could restore immunity against infection in a short time. At the same time, the use of glucocorticoids was also very important. Its immunosuppressive and anti-inflammatory effects played a large role in the treatment process.In addition, prophylactic antibiotics was needed, and nephrotoxic drugs should be used with caution. Finally, following discounting the use of immunosuppressant and a low-dose glucocorticoid-based treatment regimen, COVID-19 in this renal transplant recipient was successfully cured. The cure of this case was of great significance, and this adjuvant nonspecific antiviral therapy could provide a template for the treatment of other such patients.
Adult
;
Betacoronavirus
;
COVID-19
;
Coronavirus Infections
;
Female
;
Humans
;
Kidney Transplantation
;
Pandemics
;
Pneumonia, Viral
;
SARS-CoV-2
;
Transplant Recipients
7. Analysis and Comparison of Volatile Components in Trifolium pratense and Trifolium repens Seed by GC-MS
Chun-lan HE ; Gang-ping ZHANG ; Ping ZHOU ; Ru-yi WANG ; Zhi-hao WEN
Chinese Journal of Experimental Traditional Medical Formulae 2019;25(3):156-161
Objective: To analyze and compare the difference of volatile components between Trifolium pratense and T. repens seeds.Method: The volatile components were extracted by the headspace solid phase microextraction (HS-SPME) method and analyzed by gas chromatography-mass spectrometry (GC-MS),and the relative contents of the components were calculated by the area normalization method.Result: Fourty six compounds were identified in the seeds of T. pratense,with a total peak area of 3.48×109 for the volatile components. The compounds with high content in this species were 1-octen-3-ol (30.24%),1-hexanol (11.94%),heptane,2,2,4,6,6-pentamethyl (11.00%),nonanal (7.74%),octanal (6.79%) and hexanoic acid (6.04%). 44 compounds were identified from the seeds of T. repens,with a total peak area of 6.37×109 for the volatile components. The compounds with high content in this species were 1-octen-3-ol (42.98%),heptadecane (14.47%),3-octanol (9.42%),n-caproic acid vinyl ester (8.82%) and pyrimidine-4,6-diol,5-methyl (6.59%). 20 components were commonly shared in the seeds of T. pratense and T. repens,but the content of each component was quite different. 1-octen-3-ol had the highest content in the common components. T. pratense seeds had 26 unique components,accounting for 54.85%of volatile components. T. repens seeds had 24 unique components,accounting for 46.79%of volatile components.Conclusion: The seeds of both T. pratense and T. repens have rich volatile components,but there are great difference in the variety,peak area and relative content of the volatile components between these two varieties
8.Research actuality and quality-influencing factor of Epimedii Folium.
Ai-Ping DENG ; Wen-Tao FANG ; Qing-Gang ZHOU ; Hong-Jun YANG ; Ling WANG ; Tie-Gui NAN ; Li-Ping KANG ; Lan-Ping GUO ; Lu-Qi HUANG ; Zhi-Lai ZHAN
China Journal of Chinese Materia Medica 2018;43(5):1062-1070
Epimedii Folium has a long history in China as a common traditional Chinese medicine. Key factors of Epimedii Folium quality were summarized based on ancient literatures, Chinese Pharmacopoeias and modern research in different period of history. The main reason for unqualified Epimedii Folium is unstable icariin. Therefore, it's suggested that: the precondition of the quality control of epimedium is to find the proper quality marker. It's suggested that the medicinal parts should be reverted to "dry whole plant overground" to solve Epimedium resource shortage problem. In addition, it is necessary to strengthen the standardized cultivation, so as to ensure germplasm, production area, and producing method to guarantee the quality of Epimedium Folium. In the drying method, it is recommended to change "dry in the sun or shade" to "dry", namely dry in the sun, shade or drier, in order to provide a new method to improve the quality control and quality standard of Epimedii Folium.
9. A multicenter clinical study on 1 138 cases of invasive pneumococcal disease in children from 2012 to 2017
Liang ZHU ; Wenhui LI ; Xinhong WANG ; Kun TAN ; Qingfeng FANG ; Qingxiong ZHU ; Kangkang WU ; Qiaozhi YANG ; Aiwei LIN ; Huiling DENG ; Jing BI ; Jing LIU ; Shiyong ZHAO ; Yun LIU ; Shujun JING ; Yumin WANG ; Lianmei LI ; Qing ZHAO ; Kaihu YAO ; Xi WANG ; Li JIA ; Fang WANG ; Jikui DENG ; Jing SUN ; Chunhui ZHU ; Kai ZHOU ; Jun LIANG ; Xiuzhen NIE ; Sancheng CAO ; Dongmeng WANG ; Shuangjie LI ; Xuexia CHEN ; Juan LI ; Yi WANG ; Lan YE ; Yanhong ZHANG ; Fang DONG ; Zhi LI ; Yonghong YANG ; Gang LIU
Chinese Journal of Pediatrics 2018;56(12):915-922
Objective:
To explore the clinical features, the serotype distribution and drug resistance of the isolates in patient with invasive pneumococcal disease (IPD).
Methods:
By retrieving the laboratory information system in 18 children′s hospitals from 2012 to 2017, the children with IPD were enrolled.
10.Ventilator management for acute respiratory distress syndrome associated with avian influenza A (H7N9) virus infection: A case series
Hui XIE ; Zhi-Gang ZHOU ; Wei JIN ; Cheng-Bin YUAN ; Jiang DU ; Jian LU ; Rui-Lan WANG
World Journal of Emergency Medicine 2018;9(2):118-124
BACKGROUND: Data on the mechanical ventilation (MV) characteristics and radiologic features for the cases with H7N9-induced ARDS were stil lacking. METHODS: We describe the MV characteristics and radiologic features of adult patients with ARDS due to microbiologically confirmed H7N9 admitted to our ICU over a 3-month period. RESULTS: Eight patients (mean age 57.38±16.75; 5 male) were diagnosed with H7N9 in the first quarter of 2014. All developed respiratory failure complicated by acute respiratory distress syndrome (ARDS), which required MV in ICU. The baseline APACHE II and SOFA score was 11.77±6.32 and 7.71±3.12. The overall CT scores of the patients was 247.68±34.28 and the range of CT scores was 196.3–294.7. The average MV days was 14.63±6.14, and 4 patients required additional rescue therapies for refractory hypoxemia. Despite these measures, 3 patients died. CONCLUSION: In H7N9-infected patients with ARDS, low tidal volume strategy was the conventional mode. RM as one of rescue therapies to refractory hypoxemia in these patients with serious architectural distortion and high CT scores, which could cause further lung damage, may induce bad outcomes and requires serious consideration. Prone ventilation may improve mortality, and should be performed at the early stage of the disease, not as a rescue therapy.

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