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1.
Chinese Journal of Schistosomiasis Control ; (6): 590-603, 2023.
Article in Chinese | WPRIM | ID: wpr-1011417

ABSTRACT

Objective To investigate the effects of Echinococcus multilocularis on the phenotypic transformations of glucose metabolism, polarization types and inflammatory responses in macrophages, so as to provide insights into elucidation of echinococcosis pathogenesis. Methods Bone marrow cells were isolated from C57BL/6J mice at ages of 6 to 8 weeks, and induced into bone marrow-derived macrophages (BMDMs) with mouse macrophage colony-stimulating factor (M-CSF), which served as controls (BMDMs-M0). BMDMs-M0 induced M2 macrophages by interleukin-4 for 24 hours served as the IL-4 induction group, and BMDMs-M0 co-cultured with 2.4 ng/mL E. multilocularis cystic fluid (CF) served as the BMDM-CF co-culture group, while BMDMs-M0 co-cultured with E. multilocularis protoscolex (PSC) at a ratio of 500:1 served as the BMDM-PSC co-culture group. The types of polarization of BMDMs co-cultured with E. multilocularis CF and PSC were analyzed using flow cytometry, and the expression of macrophage markers, inflammatory factors, and glucose metabolism-related enzymes was quantified using fluorescent quantitative real-time PCR (qPCR) and Western blotting assays. Results There were significant differences among the four groups in terms of Arginase-1 (Arg1) (F = 1 457.00, P < 0.000 1), macrophages-derived C-C motif chemokine 22 (Ccl22) (F = 22 203.00, P < 0.000 1), resistin-like α (Retnla) (F = 151.90, P < 0.000 1), inducible nitric oxide synthase (iNOS) (F = 107.80, P < 0.001), hexokinase (HK) (F = 9 389.00, P < 0.000 1), pyruvate kinase (PK) (F = 641.40, P < 0.001), phosphofructokinase 1 (PFK1) (F = 43.97, P < 0.01), glucokinase (GK) (F = 432.50, P < 0.000 1), pyruvate dehydrogenase kinases1 (PDK1) (F = 737.30, P < 0.000 1), lactic dehydrogenase (LDH) (F = 3 632.00, P < 0.000 1), glucose transporter 1 (GLUT1) (F = 532.40, P < 0.000 1), glyceraldehyde-3-phosphate dehydrogenase (GAPDH) (F = 460.00, P < 0.000 1), citrate synthase (CS) (F = 5 642.00, P < 0.01), glycogen synthase1 (GYS1) (F = 273.30, P < 0.000 1), IL-6 (F = 1 823.00, P < 0.000 1), IL-10 (F = 291.70, P < 0.000 1), IL-1β (F = 986.60, P < 0.000 1), and tumor necrosis factor (TNF)-α (F = 334.80, P < 0.000 1) and transforming growth factor (TGF)-β mRNA expression (F = 163.30, P < 0.001). The proportion of M2 macrophages was significantly higher than that of M1 macrophages in the BMDM-PSC co-culture group [(22.87% ±1.48%) vs. (1.70% ±0.17%); t = 24.61, P < 0.001], and the proportion of M2 macrophages was significantly higher than that of M1 macrophages in the BMDM-CF co-culture group [(20.07% ±0.64%) vs. (1.93% ±0.25%); t = 45.73, P < 0.001]. The mRNA expression of M2 macrophages markers Arg1, Ccl22 and Retnla was significantly higher in the BMDM-CF and BMDM-PSC co-culture groups than in the control group (all P values < 0.01), and no significant difference was seen in the mRNA expression of the M1 macrophage marker iNOS among the three groups (P > 0.05), while qPCR assay quantified higher mRNA expression of key glycolytic enzymes HK, PK and PFK, as well as inflammatory factors IL-10, IL-1β, TNF-α and TGF-β in the BMDM-CF and BMDM-PSC co-culture groups than in the control group (all P values < 0.01). Western blotting assay determined higher HK, PK and PFK protein expression in the BMDM-PSC co-culture group than in the control group (all P values < 0.05), and qPCR quantified higher GLUT1, GAPDH and IL-6 mRNA expression in the BMDM-CF co-culture group than in the control group (all P values < 0.05), while higher HK, PK and PFK protein and mRNA expression (all P values < 0.01), as well as lower IL-6 and TNF-α and higher TGF-β mRNA expression (both P values < 0.05) was detected in the IL-4 induction group than in the control group. Glycolytic stress test showed no significant difference in the extracellular acidification rate (ECAR) of mouse BMDM among the control group, IL-4 induction group and BMDM-PSC co-culture group (F = 124.4, P < 0.05), and a higher ECAR was seen in the BMDM-PSC co-culture group and a lower ECAR was found in the IL-4 induction group than in the control group (both P values < 0.05). Conclusions Treatment of E. multilocularis CF or PSC mainly causes polarization of BMDM into M2 macrophages, and phenotypic transformation of glucose metabolism into high-energy and high-glycolytic metabolism, and affects inflammatory responses in BMDM.

2.
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery ; (12): 647-659, 2023.
Article in Chinese | WPRIM | ID: wpr-996476

ABSTRACT

@#According to the needs of CT image evaluation for transapical transcatheter aortic valve replacement (TAVR), 20 clinical questions were proposed by the Delphi method, 15 questions were initially determined, and 12 clinical questions were summarized and determined by domestic experts. PubMed, Web of Science, Wanfang, and CNKI databases were searched by computer to collect the relevant literature from inception to November 2022, and finally 53 studies were included. Based on evidence-based study and evaluation experience, 3 meetings were held to give recommendations for preoperative CT data acquisition method, preoperative imaging evaluation of aortic root, imaging evaluation of transapical approach, preoperative auxiliary guidance of TAVR by CT images combined with 3D printing, and postoperative imaging evaluation of transapical TAVR, hoping to promote the standardized and successful development of transapical TAVR in China.

3.
Chinese Journal of Radiology ; (12): 404-409, 2023.
Article in Chinese | WPRIM | ID: wpr-992974

ABSTRACT

Objective:To explore the value of cardiac MRI (CMRI) in evaluating left atrial function in patients with postoperative tetralogy of Fallot (rTOF) and postoperative pulmonary stenosis (rPS).Methods:Totally 67 pediatric patients (49 with rTOF, 18 with rPS) with preserved left ventricular ejection fraction (EF) were recruited between January 2019 and October 2021 in Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiao Tong University. Thirty-three healthy volunteers, matched in gender and age, were included as controls from July 2017 to August 2018. Left atrial EF, strain and strain rate of three phases (reservoir, conduit and pump), left atrial volume (maximum volume index, minimum volume index and pre-atrial contraction volume index) were measured with corresponding cardiac function analysis software. Then, the differences in these parameters were analyzed between the three groups by ANOVA or Kruskal-Wallis test with post hoc comparison and Bonferroni correction.Results:Compared with controls, patients with rTOF had lower reservoir function parameters (EF, strain and strain rate), conduit EF, conduit strain, and left atrial maximum volume index ( P<0.05), but higher pump EF ( P<0.05). In patients with rPS, only the reservoir strain rate decreased compared with controls ( P<0.05), and the remaining data showed no significant difference ( P>0.05). The reservoir and conduit EF and strain in patients with rPS were higher than those in patients with rTOF ( P<0.05). Conclusions:In patients with rTOF and rPS, left atrial function has changed despite the preservation of left ventricular EF, which may be an early marker of left ventricular diastolic dysfunction. In children with rTOF, left atrial reservoir and conduit functions decreased while the pump function increased. The reservoir and pump functions in rPS were better than those in rTOF. In addition, CMRI can detect left atrial dysfunction early before it enlarged.

4.
Chinese Journal of Applied Clinical Pediatrics ; (24): 816-820, 2022.
Article in Chinese | WPRIM | ID: wpr-930525

ABSTRACT

Objective:To analyze risk factors for duration of small or medium-sized coronary artery aneurysms (CAA) in children with Kawasaki disease (KD) so as to provide clinical guidance for early and full course treatment.Methods:The clinical data of 68 children diagnosed with KD in the Department of Pediatrics, the First Affiliated Hospital of Xinxiang Medical University from January 2018 to January 2021 were retrospectively analyzed.According to duration of CAA, all cases were divided into 2 groups, duration of CAA ≥ 8 weeks group and duration of CAA <8 weeks group.Risk factors associated with CAA duration were screened using univariate analysis, and then independent risk factors for CAA duration in children with KD were analysed using multiple Logistic regression analysis. Results:A total of 68 cases were enrolled in this study.Among these cases, 45 cases (66.18%) were male and 23 cases (33.82%) were female.The onset age was from 3 months to 10 years old, and the median onset age was 1.59 (1.02-3.19). There were 31 cases in the group with CAA duration ≥8 weeks and 37 cases in the group with CAA duration <8 weeks.Univariate analysis showed that patients with the total fever course >10 days[45.16%(14/31 cases) vs.21.62%(8/37 cases)], time of treatment with intravenous immunoglobulin (IVIG)>10 days[54.84%(17/31 cases) vs.16.22%(6/37 cases)], platelet (PLT)>600×10 9/L[32.26%(10/31 cases) vs.10.81%(4/37 cases)], hypersensitive C-reactive protein (HsCRP) >100 mg/L[38.71%(12/31 cases) vs.13.51%(5/37 cases)] (all P<0.05 ) in the group with CAA duration ≥8 weeks were significantly more than those in the group with CAA duration <8 weeks.However, there were no significant differences in gender, age, type of KD, etiology evidence, hormone application, duration of fever before IVIG application, IVIG sensitivity, IVIG application way, urine leukocytes, white blood cells, hemoglobin, percent of neutrophilic granulocyte, erythrocyte sedimentation rate, glutamic-pyruvic transaminase between the 2 groups (all P>0.05). Multivariate Logistic regression analysis showed that the course of IVIG before application >10 days ( OR=6.589, 95% CI: 1.678-25.867, P=0.007)and HsCRP >100 mg/L ( OR=7.949, 95% CI: 1.947-32.461, P=0.004)were independent risk factors for predicting the duration of KD complicated with small and medium-sized CAA ≥8 weeks. Conclusions:The course of IVIG before application >10 days and HsCRP>100 mg/L are independent risk factors for KD complicated with small and medium-sized CAA lasting ≥8 weeks.

5.
Chinese Journal of Anesthesiology ; (12): 1103-1107, 2022.
Article in Chinese | WPRIM | ID: wpr-957574

ABSTRACT

Objective:To evaluate the effects of exosomes derived from cardiac fibroblasts pretreated with sevoflurane on ventricular electrical conduction in isolated rat hearts subjected to hypothermic ischemia-reperfusion (I/R) using the multi-electrode array mapping technique.Methods:Primary cardiac fibroblasts were extracted by differential adhesion in SPF Sprague-Dawley rats of either sex.Cardiac fibroblasts of passage 2-4 were treated with 2.5% sevoflurane for 1 h, and then cultured for 24-48 h to extract exosome.SPF healthy male Sprague-Dawley rats, aged 2-3 months, weighing 280-320 g, were divided into 3 groups ( n=8 each) using a random number table method: control group (group C), I/R group and sevoflurane-pretreated cardiac fibroblast-derived exosome+ IR group (group S+ IR). Hearts were perfused for 110-min equilibration in group C. After 20 min of equilibration, the perfusion was suspended for 60 min (global ischemia) followed by 30 min of reperfusion in IR and S+ IR groups.Exosomes 1 ml (200 μg) derived from cardiac fibroblasts pretreated with sevoflurane were injected through the tail vein at 48 h before surgery in group S+ IR, and the equal volume of normal saline was injected instead in C and IR groups.The cardiac conduction velocity (CV), conduction absolute inhomogeneity (P 5-95) and inhomogeneity index (P 5-95/P 50) were obtained at 20 min of equilibration (T 0) and 15 and 30 min of reperfusion (T 1, 2) using the microelectrode array attaching to the left ventricular surface of the isolated heart. Results:Compared with group C, CV was significantly decreased and P 5-95 and P 5-95/P 50 were increased at T 1 ( P<0.05), and no significant change was found at T 2 in group S+ IR ( P>0.05), and CV was significantly decreased and P 5-95 and P 5-95/P 50 were increased at T 1, 2 in group IR ( P<0.05). Compared with group IR, CV was significantly increased and P 5-95 and P 5-95/P 50 were decreased at T 1, 2 in group S+ IR ( P<0.05). Conclusions:Exosomes derived from cardiac fibroblasts pretreated with sevoflurane can improve ventricular electrical conduction in isolated rat hearts subjected to hypothermic I/R.

6.
Chinese Journal of Ultrasonography ; (12): 885-888, 2022.
Article in Chinese | WPRIM | ID: wpr-956667

ABSTRACT

Objective:To study the clinical value of color Doppler ultrasonography in the diagnosis of persistent sciatic vein(PSV).Methods:A retrospective study was performed on 17 patients who were diagnosed with PSV by color Doppler ultrasound in the Second Hospital of Shandong University and the Shandong Provincial Hospital Affiliated to Shandong First Medical University from May 2010 to December 2021. Their sonographic features were analyzed, summarized and classified.Results:In all the 17 cases, the sciatic vein showed a vein adjacent to the sciatic nerve in the pelvis or back of the thigh. According to anatomy, persistent sciatic vein could be divided into three types: complete PSV, upper PSV and lower PSV. There were 7 cases of complete PSV, 2 cases of upper PSV and 8 cases of lower PSV. Femoral vein dysplasia was found in 11 of 17 patients with PSV. In addition to 1 case of bilateral PSV, the diameter of the femoral vein on the affected side was (0.36±0.19)cm in 16 cases, and the diameter of femoral vein at the corresponding position on the healthy side was (0.61±0.11)cm, there was significant difference between the two groups ( P<0.001). Conclusions:Color Doppler ultrasonography is the effective imaging method for diagnosis of the PSV.

7.
Chinese Journal of Primary Medicine and Pharmacy ; (12): 674-678, 2022.
Article in Chinese | WPRIM | ID: wpr-931675

ABSTRACT

Objectives:To analyze the clinical features of juvenile myelomonocytic leukemia (JMML) and investigate the characteristics of diagnosis and treatment of this disease.Methods:The clinical data of seven children patients with JMML who received treatment in The First Affiliated Hospital of Xinxiang Medical University between April 2015 and February 2020 were retrospectively analyzed. The clinical efficacy of different treatments was analyzed.Results:The median age at diagnosis of JMML was 8 months and 4 days for seven children patients. Fever was the principal cause of treatment, and it was mostly accompanied by hepatosplenomegaly. The median value of peripheral blood leukocyte count was 36.1 × 10 9/L, and it was 4.5 × 10 9/L for mononuclear cell count, 88 g/L for hemoglobin level, and 47 × 10 9/L for platelet count. Myeloid immature cells were found in peripheral blood smears of six patients. Chromosome examination results revealed 7-monomer in one patient, and normal karyotype in six patients. Hemoglobin level was increased in six patients. Gene detection results revealed PTPN11+NF1 mutation in one patient, N-RAS mutation in two patients, and K-RAS mutation in one patient. Three patients gave up treatment, three patients received low-intensity chemotherapy , and these six patients died of complicated infection. One patient received allogeneic hematopoietic stem cell transplantation and the patient survived without any event after 14 months of follow-up. Conclusion:The age of JMML onset is low. JMML has poor clinical specificity. Gene detection is helpful for early diagnosis of JMML. Low-intensity chemotherapy can prolong survival period, but it can not improve prognosis. Infection is the principal cause of death in patients with JMML. Hematopoietic stem cell transplantation is the only possible method to cure the disease.

8.
Chinese Journal of Thoracic and Cardiovascular Surgery ; (12): 399-403, 2021.
Article in Chinese | WPRIM | ID: wpr-912294

ABSTRACT

Objective:To explore the feasibility and effectiveness of 3D printing aortic model for preoperative evaluation and surgical simulation, and to assist interventional treatment of coarctation of the aorta(CoA).Methods:From December 2017 to January 2019, 8 patients with congenital coarctation of the aorta who underwent percutaneous balloon dilatation and covered stent placement in Xijing Hospital of Air Force Military Medical University were analyzed retrospectively. Among them, 7 cases were male and 1 case was female. The age was(32.00±14.93) years old. Before operation, CT data of patients' heart and aorta were collected, reconstructed with Mimics software, and 3D printing technology was used to make the model of patients' aortic lesions. Before operation, the operation simulation was carried out to determine the best operation scheme and estimate the possible situation, and the relevant clinical data of patients during hospitalization and follow-up were collected.Results:One stent graft was successfully implanted into CoA through femoral artery in all 8 patients. The mean diameter of CoA increased from(3.70±2.94) mm before operation to(18.01±1.51) mm immediately after operation( P<0.05), and the mean systolic pressure difference decreased from(83.75±25.44) mmHg before operation to(14.63±8.09) mmHg after operation( P<0.05). The mean systolic blood pressure of the right upper extremity decreased from(204.13±22.31) mmHg before operation to(145.63±32.08) mmHg after operation( P<0.05), and there was no significant difference between the two groups. During the period of hospitalization and follow-up, no corresponding cardiovascular complications were found. Conclusion:The short-term effect of percutaneous balloon dilatation covered stent implantation on CoA in adolescents and adults is obvious. 3D printing model can reproduce the anatomical model of CoA site of patients individually, which is feasible and effective for the preoperative evaluation of CoA and the preparation of operation plan.

9.
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery ; (12): 908-914, 2021.
Article in Chinese | WPRIM | ID: wpr-886533

ABSTRACT

@#Objective    To discuss the operation skill and clinical effects of using domestic balloon-expandable Prizvalve® transcatheter "valve-in-valve" to treat the degenerated bioprosthesis in the tricuspid position. Methods    All the admitted surgical tricuspid valve bioprosthetic valve replacement patients were evaluated by computerized tomography angiography (CTA), ultrasound, and 3D printing technology, and 2 patients with a degenerated bioprosthesis were selected for tricuspid valve "valve-in-valve" operation. Under general anesthesia, the retro-preset Prizvalve® system was implanted into degenerated tricuspid bioprosthesis via the femoral vein approach under the guidance of transesophageal echocardiographic and fluoroscopic guidance. Results    Transcatheter tricuspid valve implantation was successfully performed in both high-risk patients, and tricuspid regurgitation disappeared immediately. The operation time was 1.25 h and 2.43 h, respectively. There was no serious complication in both patients, and they were discharged from the hospital 7 days after the operation. Conclusion    The clinical effect of the degenerated tricuspid bioprosthetic valve implantation with domestic balloon-expandable valve via femoral vein approach "valve-in-valve" is good. Multimodality imaging and 3D printing technology can safely and effectively guide the implementation of this innovative technique.

10.
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery ; (12): 757-764, 2021.
Article in Chinese | WPRIM | ID: wpr-886496

ABSTRACT

@#Objective    To investigate the feasibility and safety of transcatheter aortic valve replacement (TAVR) through apical approach for aortic regurgitation of large annulus. Methods    From November 2019 to May 2020, 10 male patients aged 64.50±4.20 years with aortic valve insufficiency (AI) underwent TAVR in the Department of Cardiovascular Surgery, Xijing Hospital. The surgical instruments were 29# J-valveTM modified and the patients underwent TAVR under angiography. The preoperative and postoperative cardiac function, valve regurgitation, complications and left ventricular remodeling were summarized by ultrasound and CT before and after TAVR. Results    A total of 10 valves were implanted in 10 patients. Among them, 1 patient was transferred to the aortic arch during the operation and was transferred to surgical aortic valve replacement; the other 9 patients were successfully implanted with J-valve, with 6 patients of cardiac function (NYHA) class Ⅱ, 4 patients of grade Ⅲ. And there was a significant difference between preoperation and postoperation in left ventricular ejection fraction (44.70%±8.78% vs. 39.80%±8.48%, P<0.05) or aortic regurgitation (1.75±0.72 mL vs. 16.51±8.71 mL, P<0.05). After 3 months, the patients' cardiac function was good. Conclusion    TAVR is safe and effective in the treatment of severe valvular disease with AI using J-valve.

11.
Chinese Journal of Medical Education Research ; (12): 467-470, 2021.
Article in Chinese | WPRIM | ID: wpr-883646

ABSTRACT

Objective:To explore the application effect of Roy adaptation model combined situational teaching method in pediatric nursing teaching.Methods:A total of 108 nursing students interned in our hospital in 2019 were selected and randomly divided into two groups, among which, 54 nurses who accepted the traditional teaching method were taken as the control group, and 54 ones who adopted Roy adaptation model combined situational teaching method were taken as the research group. The scores of theory and operation skills of the two groups were evaluated before and after teaching, at the same time, the comprehensive ability was assessed and the satisfaction survey was conducted among the students after the teaching. SPSS 20.0 was used for data processing, the scores of theory and operation skills comprehensive ability and nursing students' satisfaction with teaching were described with ( ± s) and t test was performed. Results:After the teaching, the theoretical knowledge and operational skills scores of the two groups were significantly improved than those before the teaching ( P<0.05), and those of all students in the research group were significantly higher than those in the control group ( P<0.05). The scores of ability to integrate theory with practice, self-learning ability, communication ability, teamwork ability, independent thinking and problem solving ability, clinical thinking ability in the research group were higher than those in the control group, with statistical differences ( P<0.05). And students in research group had significantly higher overall satisfaction scores on the teaching than those in control group had ( P<0.05). Conclusion:Application of Roy adaptation model combined situational teaching method in pediatric practice teaching can improve the theoretical knowledge and operational level of nursing students, enhance their communication ability and other abilities, and help to improve their satisfaction with teaching.

12.
Chinese Journal of Medical Genetics ; (6): 147-149, 2020.
Article in Chinese | WPRIM | ID: wpr-781279

ABSTRACT

OBJECTIVE@#To explore the genetic etiology of a girl featuring epilepsy, speech delay and mild mental retardation.@*METHODS@#Peripheral blood samples of the child and her parents were collected. Genomic DNA was extracted and subjected to next generation sequencing. Suspected variant was confirmed by Sanger sequencing.@*RESULTS@#The child was found to carry a de novo heterozygous c.3592G>A (p.V1198M) variant of the SMARCA2 gene, which was predicted to be pathogenic by bioinformatic analysis.@*CONCLUSION@#The child was diagnosed with Nicolaides-Baraitser syndrome due to heterozygous variant of the SMARCA2 gene.

13.
Chinese Journal of Pediatrics ; (12): 414-420, 2018.
Article in Chinese | WPRIM | ID: wpr-809979

ABSTRACT

Objective@#To analyz the current situation of the diagnosis, treatment and prevention of methylmalonic acidemia, the phenotypes, biochemical features and genotypes of the patients in the mainland of China, were investigated.@*Methods@#Tottally 1 003 patients of methylmalonic acidemia from 26 provinces and municipalities of the mainland of China were enrolled. The clinical data, biochemical features and gene mutations were studied. Blood aminoacids and acylcarnitines, urine organic acids, and plasma total homocysteine were determined for the biochemical diagnosis. Gene analyses were performed for the genetic study of 661 patients. The patients were treated with individual intervention and long-term follow up. Prenatal diagnoses were carried out for 165 fetuses of the families.@*Results@#Among 1 003 patients (580 boys and 423 girls), 296 cases (29.5%) had isolated methylmalonic acidemia; 707 cases (70.5%) had combined homocysteinemia; 59 patients (5.9%) were detected by newborn screening; 944 patients (94.1%) had the onset at the ages from several minutes after birth to 25 years and diagnosed at 3 days to 25 years of age. The main clinical presentations were psychomotor retardation and metabolic crisis. Multi-organ damage, including hematological abnormalities, pulmonary hypertension, kidney damage, were found. MMACHC, MUT, MMAA, MMAB, HCFC1, SUCLG1, SUCLA2 mutations were found in 631 patients (96.6%) out of 661 patients who accepted gene analysis. MMACHC mutations were detected in 460 patients (94.7%) out of 486 cases of methylmalonic acidemia combined with homocysteinemia. MUT mutations were found in 158 (90.3%) out of 169 cases of isolated methylmalonic acidemia. The development of 59 patients detected by newborn screening were normal; 918 cases (97.2%) were diagnosed after onset accepted the treatment. Forty-five of them completely recovered with normal development. Twenty-six patients (2.7%) died; 873 (92.5%) patients had mild to severe psychomotor retardation. Methylmalonic acidemia were found in 35 out of 165 fetuses by metabolites assay of amniotic fluid and amniocytes gene analysis.@*Conclusion@#Combined methylmalonic acidemia and homocysteinemia is the common type of methylmalonic acidemia in the mainland of China. CblC defect due to MMACHC mutations is the most common type of methylmalonic acidemia combined with homocysteinemia. MUT gene mutations are frequent in the patients with isolated methylmalonic acidemia. Newborn screening is key for the early diagnosis and the better outcome. Combined diagnosis of biochemical assays and gene analysis are reliable for the prenatal diagnosis of methylmalonic acidemia.

14.
Journal of Clinical Pediatrics ; (12): 57-60, 2018.
Article in Chinese | WPRIM | ID: wpr-694640

ABSTRACT

Objective To investigate the clinical and molecular genetic characteristics of hypermethioninemia caused by methionine adenosyltransferase deficiency. Methods The clinical data and related gene analysis of hypermethioninemia caused by methionine adenosyltransferase deficiency in 3 children were retrospectively analyzed. The core pedigree analysis was carried out. Results Three children (2 boys and 1 girl) aged from 5 months to 3 years, were from 3 unrelated families. All of them had no family history. One case was found in neonatal screening. One case was onset with pathological jaundice at 1 month old. Another case was found due to tremor and growth retardation at 2 years old. Blood amino acid ester acyl carnitine spectrum analysis showed that all of them had significantly elevated levels of methionine at 134.50-790.67 μmol/L. All children had MAT1A mutation in methionine adenosyltransferase gene. One case was heterozygous mutations with third exon c.274T>C and seventh exon c.895C>T mutation; one case had sixth exon c.757G>A homozygous mutation; and another case had seventh exon c.791G>A homozygous mutation. The core pedigree analysis showed that the mutations were from theirs parents respectively. Conclusions For children with neurologic impairment, methionine metabolic disorders should be considered. Blood amino acids and gene analysis are important methods for confirmation of the diagnosis. Neonatal screening is an effective way to detect this disease.

15.
Cancer Research and Clinic ; (6): 38-42, 2018.
Article in Chinese | WPRIM | ID: wpr-712761

ABSTRACT

Objective To compare the clinical efficacy of single utility port and multiple utility ports thoracoscopic lobectomy in the treatment of peripheral lung cancer, and to study the operation skills, relative merit and feasibility of the single utility port thoracoscopic lobectomy. Methods The clinical data was analyzed retrospectively for 223 cases with stage Ⅰ orⅡ of peripheral lung cancer who underwent thoracoscopic lobectomy from July 2011 to November 2014 in Shanxi Provincial Cancer Hospital. Among 223 cases, 78 cases received single utility port thoracoscopic lobectomy (single utility port group), 145 cases received 2 or 3 utility ports thoracoscopic lobectomy (multiple utility ports group). The clinical outcomes involved time of operation, intraoperative blood loss,chest drainage, postoperative hospital stay, stations of lymph node dissection, numbers of lymph node dissection, rate of turn to open, postoperative complications, 2-year survival rate and disease free survival rate. Results No perioperative death occurred in both groups. There was no statistical difference between single utility port group and multiple utility ports group in operation time [(157.4 ±13.6) min vs. (151.3 ±23.2) min], intraoperative blood loss [(180.77 ±59.97) ml vs.(171.31 ±77.51) ml],chest drainage [(370.26 ±146.09) ml vs. (351.17 ±159.07) ml], lymph node dissection stations (4.29±0.65 vs. 4.21±0.73), lymph node dissection number (11.50±2.30 vs. 11.04±2.29), rate of turn to open [(5.13 % (4/78) vs. 4.83 % (7/145)], incidence of postoperative complications [17.95 % (14/78) vs. 15.86%(23/145)], postoperative hospital stay [(8.74±0.51) d vs. (9.48±0.63) d], 2-year survival rate [96.15 %(75/78) vs. 93.79%(136/145)] and 2-year disease free survival rate [80.77 % (63/78) vs. 82.07 % (119/145)] (all P>0.05). Conclusions The single utility port thoracoscopic lobectomy could achieve the same clinical results as the multiple utility ports. The single utility port thoracoscopic lobectomy is a safe, effective and feasible surgical procedure.

16.
Chinese Journal of Nursing ; (12): 334-337, 2018.
Article in Chinese | WPRIM | ID: wpr-708744

ABSTRACT

Objective To evaluate Chinese nurses' perception of the nursing work environment and to compare the differences among the eight economic regions in mainland China.Thus,policy-makers and nursing managers can optimize and coordinate resources based on the results of this study.Methods Chinese version of Nursing Working Environment Scale was used in the study.A total of 19,000 nurses from 31 provinces and municipalities in mainland China were investigated.Descriptive analysis,cluster analysis and nuclear density estimation were conducted.Results The average score of the nursing work environment was 4.1 points,which means nurses were somewhat satisfied with their work conditions.Specifically,nurses were most dissatisfied with the basic safeguard dimension (3.4 points),followed by sufficient manpower (3.9 points),career development (4.0) and leadership and management(4.0)dimensions.In addition,the eight economic regions could be divided into three categories(P<0.01)according to the differences of the nursing work environment.The first category included the northeast,northern coastal and middle reaches of the Yellow River economic zone;the second category included eastern coastal economic zone;and the third category included the southern coast,the middle reaches of the Yangtze River,southwest and northwest economic zone.Conclusion The development of the nursing work environment in the vast majority of mainland China is unbalanced and the regional differences are obvious.

17.
Basic & Clinical Medicine ; (12): 553-556, 2017.
Article in Chinese | WPRIM | ID: wpr-513832

ABSTRACT

Sirt3 is a kind of nicotinamide adenine dinucleotide (NAD+)-dependent deacetylases.Sirt3 is localized in mitochondria and has been involved in a wide range of mitochondrial biological functions, such as nutrient oxidation、ATP generation, reactive oxygen species (ROS) detoxification and mitochondrial homeostasis.Sirt3 plays an important role in the occurrence and development of cardiovascular diseases.Increased expression of Sirt3 gene has been associated with extended lifespan of humans.

18.
Chinese Journal of Endocrinology and Metabolism ; (12): 75-77, 2017.
Article in Chinese | WPRIM | ID: wpr-506941

ABSTRACT

[Summary] SIRT3 is a member of the silent information regulator 2 ( Sir2) family, located in the inner mitochondrial membrane, with a strong effect of deacetylation. SIRT3 not only modulates energy metabolism, cell apoptosis, tumor growth, anti-aging etc. , but also plays an important role in the field of cardiovascular diseases. In this review, we summarize recent findings related to SIRT3 with metabolic syndrome and cardiac hypertrophy, to provide a theoretical basis for the further study on the potential role of SIRT3 in cardiovascular diseases.

19.
Journal of Peking University(Health Sciences) ; (6): 691-698, 2017.
Article in Chinese | WPRIM | ID: wpr-617308

ABSTRACT

Objective: To evaluate the polysomnology results along with mandibular titrated advancement using oral appliance to treat obstructive sleep apnea and hyponea syndrome(OSAHS).Methods: Several electronic databases (PubMed, Embase, Cochrane Library, CNKI, VIP, and Wanfang) were systematically searched up to September 2015.There was no restriction of language or source of information.All randomized clinical trials (RCT) and before-after trials (BAT) comparing at least two different mandibular advancements were included.Two independent reviewers selected the studies, extracted data and evaluated risk of bias by quality assessment.Data were pooled using a fixed-effects model, and the summary effect measure was calculated by risk ratio (RR) and 95%CI.Meta-analysis was performed using RevMan 5.3 software.Results: Two RCTs and five BATs were included in the review.Among the five BATs, two of them were randomized, while the other three were not.Outcomes including apnea hypopnea index (AHI), oxygen desaturation index (ODI), success rate (reduction of AHI or ODI >50%), normalization rate (AHI or ODI<10/h) were assessed in this review.Based on the trial design and quality assessment, four studies were included for meta-analysis.No significant difference in the success rate was found between the group with 50% of the maximal mandibular advancement (MMA) and the group with 75% of MMA [I2=0%, RR=0.93, with 95%CI (0.80, 1.09)].No significant dif-ference in the normalization rate was found between the 50% of MMA and 75% of MMA groups [I2=45%, RR=0.85, with 95%CI (0.68, 1.06)].Subgroup analysis displayed that the severity of OSAHS before treatment was a potential factor affecting the normalization rate.Conclusion: Based on current available evidence, the success rate and normalization rate for treating OSAHS in the patients with 75% MMA were not found to be significantly higher than those with 50% MMA.Due to small simple size in this meta-analysis, the results of the present study should be interpreted with caution.Further prospective studies are needed to strengthen the evidence.

20.
Journal of Clinical Pediatrics ; (12): 894-897, 2017.
Article in Chinese | WPRIM | ID: wpr-664966

ABSTRACT

Objective To explore the clinical features and genetic etiology of children with cystinuria with onset of kidney stone. Methods The clinical data of 3 children with cystinuria with onset of kidney stone and the gene analysis results of SLC3A1 and SLC7A9 by PCR sequencing were retrospectively analyzed.Results Three male children were from three unrelated families, kidney stone were presented in 2 cases at 1 year old and 1 case at 14 years old. The blood amino acid spectrum was normal in all 3 cases, while the free carnitine were decreased. The urinary amino acid spectrum indicated that cystine, ornithine, arginine,and threonine increased.Gene analysis confirmed that 1 case had homozygous mutations of SLC7A9 gene c.325G>A, and his parents were carriers of c.325G>A heterozygous mutation;other 2 cases had heterozygous mutations of SLC3A1 gene, c.1365delG and c.1113C>A heterozygous mutation in one case, and c.1897_1898insTA and c.1093C>T heterozygous mutation in one case, and their parents were heterozygous mutation carriers. After treatment with potassium citrate and L-carnitine, the conditions were improved in all cases. Conclusions Inherited metabolic disease should be considered for children with kidney stone. Urine amino acid analysis and gene detection are important methods for the diagnosis of cystinuria.

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