Effects of puerarin on maternal gestational diabetes mellitus rats and fetal growth / 中国药理学通报

Aim To observe the effects of oral puerarin (Pue) on the maternal gestational diabetes mellitus (GDM) rats and its fetal growth and development, so as to provide references for the application of Pue in the treatment of GDM. Methods The GDM rat model was established by injecting streptozotocin (STZ) into the tail vein of pregnant female rats, and the rats were treated with Pue orally for 12 days; the body weight and abortion of pregnant rats were recorded. The fasting blood glucose of pregnant rats was detected before and at the end of the treatment, and the glucose tolerance was tested on the 5th and 10th days after the administration of the drug. The cesarean sections were carried out on the 20th day of pregnancy. The blood glucose content of the fetal rats was detected, and the state of development was observed. The body weight and length were measured, as well as the placenta and the important organs weight, and the indexes of the organs were calculated. Results Compared with the model group, Pue could significantly reduce the fasting blood glucose of GDM pregnant rats and fetal rats, improve the glucose tolerance of pregnant rats, effectively alleviate the excessive weight gain of pregnant rats and overweight of fetal rats caused by GDM, and reduce the abortion rate; it could also reverse the decrease in the indexes of the organs of brain, heart, and liver, and the increase in the indexes of organs of kidney in fetal rats caused by GDM. Conclusions Pue can relieve the maternal and the fetal hyperglycemia in GDM, reduce the rate of miscarriage, reduce the incidence of macrosomia, and promote the development of vital fetal organs.

Kidney xenotransplantation: status quo and development trend of physiological research / 器官移植

Organ transplantation is the most effective treatment for all categories of end-stage organ diseases. To resolve the shortage of donors in organ transplantation, widespread attention has been diverted to xenotransplantation. At present, clinicians mainly highlight the problems related to xenotransplantation rejection and viral infection. The physiology of xenotransplantation has been rarely studied. Kidney performs endocrine function by producing erythropoietin (EPO), renin and activating vitamin D. Although these pathways are usually well preserved in allogeneic transplantation, species-specific differences, especially those between pigs and non-human primates, may still affect the physiological function of transplant organs. In this article, the changes of EPO, renin-angiotensin-aldosterone system (RAAS) and active vitamin D3 of pig and human after xenotransplantation were illustrated, aiming to provide reference for subclinical research of xenotransplantation.

Imaging characteristics and postoperative pathological analysis of bronchiolar adenoma / 中国胸心血管外科临床杂志

@#Objective    To analyze the pathological manifestations and imaging characteristics of bronchiolar adenoma (BA). Methods    The clinical data of 11 patients with BA who received surgeries in our hospital from January 2019 to September 2020 were retrospectively analyzed, including 5 males and 6 females aged 40-73 (62.40±10.50) years. The intraoperative rapid freezing pathological diagnosis, postoperative pathological classification, cell growth pattern, nuclear proliferation index Ki-67 and other immunohistochemical staining combined with preoperative chest CT imaging characteristics were analyzed. Results    The average preoperative observation time was 381.10±278.28 d. The maximum diameter of imaging lesions was 5-27 (10.27±6.34) mm. Eight (72.7%) patients presented with irregular morphology of heterogeneous ground-glass lesions, and 3 (27.3%) patients presented with pure ground-glass lesions. There were 10 (90.9%) patients with vascular signs, 8 (72.7%) patients with vacuolar signs, 1 (9.1%) patient with bronchus sign, 3 (27.3%) patients with pleural traction and 9 (81.8%) patients with burr/lobular sign. The surgical methods included sub-lobectomy in 10 patients and lobectomy in 1 patient. Five (45.5%) patients were reported BA by intraoperative frozen pathology. The postoperative pathological classification included 8 patients with distal-type and 3 patients with proximal-type, and the maximum diameter of the lesions was 4-20 (8.18±5.06) mm. Eight (72.7%) patients showed characteristic bilayer cell structure under microscope, and 10 (90.9%) patients showed thyroid transcription factor 1 expression in pathological tissues. The expression of NapsinA in intracavity cells was found in 9 (81.8%) patients. The Ki-67 index of the lesion tissue was 1%-5% (3.22%±1.72%). Conclusion    The pathological features and imaging findings of BA confirm the premise that BA is a neoplastic lesion. However, to identify BA as a benign or inert tumor needs more clinical data and evidence of molecular pathological studies.

Application of preoperative localization coupled with CT three-dimensional reconstruction in pulmonary nodule-centered uniportal thoracoscopic combined subsegmental/segmental resection / 中国胸心血管外科临床杂志

@#Objective     To assess the clinical value of preoperative localization coupled with computed tomography (CT) three-dimensional reconstruction in pulmonary nodule-centered uniportal thoracoscopic combined subsegmental/segmental resection. Methods     The clinical data of 30 patients of combined subsegmental/segmental resection in our hospital from December 2019 to October 2021 were retrospectively collected. There were 19 males and 11 females with the mean age of 56.4 (32.0-71.0) years. The pulmonary nodules were located by CT-guided injection of glue before operation. The three-dimensional reconstruction image and operation planning were carried out by Mimics 21.0 software. Results    The operations were all successfully performed, and there was no conversion to open thoracotomy or lobectomy. The mean tumor diameter was 11.6±3.5 mm, the mean distance between the nodule and the visceral pleura was 13.6±5.6 mm, the mean width of the actual cutting edge was 25.0±6.5 mm, the mean operation time was 110.2±23.8 min, the mean number of lymph node dissection stations was 6.5±2.4, the mean amount of intraoperative bleeding was 50.8±20.3 mL, the mean retention time of thoracic catheter was 3.2±1.1 d, and the mean postoperative hospital stay was 4.5± 1.7 d. There was 1 patient of subcutaneous emphysema, 1 patient of atrial fibrillation and 1 patient of blood in sputum. Conclusion     Preoperative CT-guided injection of medical glue combined with CT three-dimensional reconstruction of pulmonary bronchus and blood vessels is safe and feasible in pulmonary nodule-centered uniportal thoracoscopic  combined subsegmental/segmental resection, which ensures the surgical margin and reserves lung tissues.

X-ray evaluation of pulp calcification in adult permanent teeth after pulpotomy / 北京大学学报(医学版)

OBJECTIVE@#To compare the clinical effects of pulpotomy with two kinds of calcium silicate materials, and to evaluate the formation of dentin bridge and pulp calcification after pulpotomy of adult permanent teeth.@*METHODS@#Patients who visited the General Department of Peking University School and Hospital of Stomatology from November 2017 to September 2019 and planned for pulpotomy on permanent premolars and molars with carious exposed pulp were selected. They were randomly divided into two groups. Bioceramic putty material iRoot BP (iRoot group, n=22) and mineral trioxide aggregate MTA (MTA group, n=21) were used as pulp capping agents, respectively. The patients were recalled after one year and two years. The clinical efficacy, dentin bridge index (DBI) and pulp calcification index (PCI) were recorded. Blinding method was used for the patients and evaluators.@*RESULTS@#There was no significant difference in gender, mean age, dentition and tooth position between the two groups (P>0.05). Seven cases were lost during the first year (4 cases in iRoot group and 3 cases in MTA group). In the iRoot group, 1 case had transient sensitivity at the time of 1-year follow-up. The cure rate of the two groups was 100% at the time of 2-year follow-up. The proportion of dentin bridge formation was 38.9% one year after operation, 55.6% two years after operation. The proportion of partial or even complete disappearance of root canal image was 5.6% before operation, 38.9% and 55.6% one and two years after operation, respectively. The difference was statistically significant by rank sum test (P < 0.05). There was no significant difference in dentin bridge formation and pulp calcification between the two groups (P < 0.05). DBI and PCI after operation was as the same as those before operation (44.4% cases of DBI and 25% cases of PCI) or gradually increased (55.6% cases of DBI and 75% cases of PCI). Spearman's nonparametric correlation analysis showed that age was positively correlated with preoperative pulp calcification index (PCI0, P < 0.05), but not with the dentin bridge index (DBI1, DBI2), pulp calcification index (PCI1, PCI2) and the degree of change (DBI2 vs. DBI1, PCI1 vs. PCI0, PCI2 vs. PCI0) 1-year and 2-year after operation (P>0.05).@*CONCLUSION@#According to this study, good clinical effects were obtained within 2-year after pulpotomy of adult permanent teeth with MTA and iRoot. In some cases, the root canal system had a tendency of calcification aggravation, and there was no statistical difference in the development of this trend between the two groups.

Kinetics of MDSC in Patients Treated Steroids-Ruxolitinib as the First Line Therapy for aGVHD / 中国实验血液学杂志

OBJECTIVE@#To analyze the kinetic characteristics of lymphocyte subsets and myeloid-derived suppressor cell (MDSC) in patients who newly diagnosed intermediate- to high-risk aGVHD and treated with steroids-ruxolitinib as the first line therapy from a single-arm, open clinical trial (NCT04061876).@*METHODS@#We prospectively observed the efficacy of 23 patients having intermediate- to high-risk aGVHD and treated with steroids-ruxolitinib as the first line therapy. The kinetic characteristics of lymphocyte subsets and MDSC were monitored, and then we compared them in steroids-ruxolitinib group (n=23), free-aGVHD group (n=20) and steroids group (n=23).@*RESULTS@#Of the 23 patients, the CR rate was 78.26% (18/23) on day 28 after first-line treatment with steroids-ruxolitinib. On day 28 after treatment, patients had lower level of CD4+CD29+ T cells (P=0.08) than that of pre-treatment, whereas levels of other lymphocyte subsets in this study were higher than that of pre-treatment; CD4+CD29+ T cells in CR patients decreased, compared with refractory aGVHD patients. On day 28 of treatment, CD8+CD28- T cells (P=0.03) significantly increased in patients with aGVHD than that in patients without aGVHD, so did CD8+CD28- T / CD8+CD28+ T cell ratio (P=0.03). Compared with patients without aGVHD, patients with aGVHD had lower level of G-MDSC, especially on day 14 after allo-HSCT (P=0.04). Compared with pre-treatment, M-MDSC was higher in CR patients on day 3 and 7 post-treatment (P3=0.01, P7=0.03), e-MDSC was higher on day 28 post-treatment (P=0.01). Moreover, compared with CR patients, M-MDSC was lower in refractory aGVHD patients on day 3 post-treatment (P=0.01) and e-MDSC was lower on day 28 post-treatment (P=0.01). Compared with steroids group, MDSC in steroids-ruxolitinib group was higher, with the most significant difference in M-MDSC (P3=0.0351; P7=0.0142; P14=0.0369).@*CONCLUSION@#We found that patients newly diagnosed intermediate- to high-risk aGVHD receiving first-line therapy with steroids-ruxolitinib achieved high response rate. Moreover, the novel first-line therapy has a small impact on the immune reconstitution of patients after allo-HSCT. Elevated MDSC might predict a better response in aGVHD patients receiving this novel first-line therapy. M-MDSC responded earlier to steroids-ruxolitinib than e-MDSC, G-MDSC.

Analysis of ADAR gene variant in a Chinese pedigree affected with dyschromatosis symmetrica hereditaria / 中华医学遗传学杂志

OBJECTIVE@#To analyze the clinical features and genetic basis for a Chinese pedigree affected with hereditary dyschromatosis symmetrica hereditaria (DSH).@*METHODS@#Peripheral blood samples of the proband and his mother were collected and subjected to PCR and Sanger sequencing.@*RESULTS@#The patient has conformed to the typical pattern of DSH and manifested with hyperpigmentation, hypo- and hyperpigmentation spots on the back of hands, feet and face. Sanger sequencing confirmed that the proband and his mother have both harbored heterozygous splicing variant c.2762+1G>T in exon 9 of the ADAR gene, which was unreported previously. The same variant was not detected among 100 healthy controls. According to the guidelines of the American College of Medical Genetics and Genomics, the variant was predicted to be pathogenic (PVS1+PM2+PP4).@*CONCLUSION@#The c.2762+1G>T variant of the ADAR gene probably underlay the DSH in this pedigree. Above finding has enriched the spectrum of ADAR gene mutations.

Evaluation of bioceramic putty repairmen iRoot and mineral trioxide aggregate in mature permanent teeth pulpotomy / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the clinical characteristics and effectiveness of pulpotomy in mature permanent teeth with bioceramic putty repairmen iRoot and mineral trioxide aggregate (MTA).@*METHODS@#Pulpotomy was performed on mature permanent premolars and molars with carious exposures at the Department of General Dentistry of Peking University School and Hospital of Stomatology, from November 2017 to September 2019. The patients were randomly divided into 2 groups, Group iRoot (n=22) and Group MTA (n=21). In Group iRoot, bioceramic putty repairmen iRoot was used as pulp capping agent, while in Group MTA, mineral trioxide aggregate was used as pulp capping agent. All the patients had signed informed consent forms. The clinical efficacy was evaluated by clinical examinations (temperature and electrical activity test) and imaging examinations 3, 6, and 12 months after surgery. Blinding was used for the patients and evaluators, but due to the obvious differences in the properties of the two pulp capping agents, the blinding method was not used for the treatment provider (the attending physician).@*RESULTS@#There was no significant difference in gender, average age, dentition and tooth position distribution between the two groups (P>0.05). In the study, 7 cases were lost to follow-up 12 months after operation (4 cases in Group iRoot, and 3 cases in Group MTA). One case in each of the two groups had transient sensitivity at the end of the 3-month follow-up, and the pulp vitality was normal at the end of the 6-month follow-up. One case in Group iRoot showed sensitivity at the end of the 12-month follow-up. The success rates of the two groups at the end of 12-month follow-up were 100%, and the cure rates were 94.4% (Group iRoot) and 100% (Group MTA), respectively, and the difference was not statistically significant (P>0.05). No cases in Group iRoot had obvious crown discoloration, while 3 cases in Group MTA had.@*CONCLUSION@#The clinical characteristics and effectiveness of pulpotomy in mature permanent teeth with bioceramic putty repairmen iRoot were similar with MTA. Bioceramic putty repairmen iRoot is an acceptable material when used in pulpotomy of mature permanent teeth. Because it is not easy to cause tooth discoloration after treatment and is convenient to operate, bioceramic putty repairmen iRoot has a better clinical application prospect.

Diagnosing lung cancer through metabolic fingerprint based on machine learning / 中华检验医学杂志

Objective:To screen out the differentially regulated metabolites by the analysis of serum metabolic fingerprints, and to provide potential biomarkers for diagnosis of lung cancer.Methods:A total of 228 subjects were enrolled in Changhai Hospital from January 27, 2021 to June 4, 2021, including 97 newly diagnosed lung cancer patients and 131 healthy individuals. Serum samples were collected from the enrolled cohort according to a standard procedure, and the enrolled cohort was divided into a training set and a completely independent validation set by stratified random sampling. The metabolic fingerprints of serum samples were collected by previously developed nano-assisted laser desorption/ionization mass spectrometry (nano-LDI MS). After age and gender matching of the training set, a diagnostic model based on serum metabolic fingerprints was established by machine learning algorithm, and the classification performance of the model was evaluated by receiver operating characteristic (ROC) curve.Results:Serum metabolic fingerprint for each sample was obtained in 1 minute using a novel nano-LDI MS, with consumption of only 1 μl original serum sample. For the training set, the area under ROC curve (AUC) of the constructed classifier for diagnosis of lung cancer was 0.92 (95% CI 0.87-0.97), with a sensitivity of 89% and specificity of 89%. For the independent validation set, the AUC reached 0.96 (95% CI 0.90-1.00) with a sensitivity of 91% and specificity of 94%, which showed no significant decrease compared to training set. We also identified a biomarker panel of 5 metabolites, demonstrating a unique metabolic fingerprint of lung cancer patients. Conclusion:Serum metabolic fingerprints and machine learning were combined to establish a diagnostic model, which can be used to distinguish between lung cancer patients and healthy controls. This work sheds lights on the rapid metabolic analysis for clinical application towards in vitro diagnosis.

Gross motor functioning, manual ability and communication of children with cerebral palsy / 中华物理医学与康复杂志

Objective:To investigate the gross motor function, manual ability and language communication of children with cerebral palsy (CP) and their correlation.Methods:A total of 318 children with CP (132 with spastic diplegia, 27 with spastic quadriplegia, 32 with spastic hemiplegia, 54 with dyskinesia, 41 with ataxia and 32 children with multiple difficulties) aged 4 to 12 years were classified according to the Gross Motor Function Classification System (GMFCS), the Manual Ability Classification System (MACS), and the Communication Function Classification System (CFCS). Spearman correlation coefficients were used to analyze the correlation among the three ratings.Results:Only 125 of the 318 children (39%) had the same classification level according to all three scales, showing moderate correlation and different levels for patients with different types of disability. The GMFCS and MACS levels of the subjects with spastic quadriplegia and those with dyskinesia were highly correlated. The GMFCS and CFCS levels of the hemiplegic children and those with spastic quadriplegia were also highly correlated. The MACS and CFCS levels were strongly correlated for children with spastic quadriplegia and multiple disabilities.Conclusions:The functioning of children with CP differs with their CP subtype. Correlations among the three functional assessments also differ for children with different subtypes. Combining the three classification systems provides a more comprehensive picture of the children′s ability to function in daily life.

Expression and Clinical Significance of Galcetin-3 in the Serum of Non-small Cell Lung Cancer Patients / 中国肺癌杂志

BACKGROUND@#Lung cancer is one of the most dangerous diseases to human health, with high morbidity and mortality. It can be cured by surgery at early stage, therefore, the early detection and early treatment of lung cancer are especially important. Serum tumor markers play an important role in the detection and diagnosis of lung cancer. Galectin-3 is known to be expressed in a variety of malignant tumors. This study was to explore the serum levels of Galectin-3 and its clinical significance in non-small cell lung cancer (NSCLC) patients.@*METHODS@#The serum levels of Galectin-3 in peripheral blood were detected by enzyme linked immunosorbent assay (ELISA) in 69 NSCLC patients and 77 cases of healthy control subjects, and compared between the two groups. Then we analyze the correlations between the serum levels of Galectin-3 and the clinical features of lung cancer.@*RESULTS@#The serum levels of Galectin-3 in NSCLC patients were significantly higher than those of healthy control subjects (P<0.01). The serum levels of Galectin-3 with lymph node metastasis were significantly higher than those of patients without lymph node metastasis (P<0.01), and N2 lymph node metastasis had higher levels of serum Galectin-3 than those of N1 lymph node metastasis (P<0.01). Clinical stage III and stage IV patients had higher levels of serum Galectin-3 than those of clinical stage I and clinical stage II (P<0.05).@*CONCLUSIONS@#Our study showed the serum levels of Galectin-3 are highly expressed in NSCLC patients and are significantly related to lymph node metastasis. It may be a potential tumor marker for lung cancer.

Comparison of the treatment outcomes between video-assisted thoracoscopic lobectomy and stereotactic body radiotherapy for early-stage non-small cell lung cancer / 中华放射医学与防护杂志

Objective:To make comparisons of treatment outcomes between video-assisted thoracoscopic surgery (VATS) lobectomy and stereotactic body radiotherapy (SBRT) for early stage non-small cell lung cancer (NSCLC).Methods:Overall survival (OS), cancer specific survival (CSS), locoregional control (LRC), and disease-free survival (DFS) were retrospectively compared between early stage NSCLC patients who underwent VATS lobectomy and SBRT at our institution from January 2012 to December 2016. Propensity score matching (PSM) was carried out to reduce selection bias between two groups based on age, gender, Karnofsky performance score (KPS), Charlson comorbidity index (CCI), pulmonary function, and tumor diameter.Results:A total of 567 patients treated with VATS lobectomy ( n=458) or SBRT ( n=109) were included. 104 patients were matched for further analysis (52 in VATS lobectomy group and 52 in SBRT group). The median follow-up time was 44 months. the 3- and 5-year OS were 94.2% and 91.6% for VATS lobectomy and 88.6% and 79.9% for SBRT ( P=0.097), respectively. No statistically significant differences were noted in 5-year CSS (91.6% vs. 83.7%, P=0.270). The cumulative incidence of LRC was comparable between two group (94.0% and 85.9% vs. 93.5% and 93.5% at 3, 5 years, P=0.621). Differences in the DFS were not statistically significant (80.5% and 79.0% at 5 years, P=0.624). In the VATS lobectomy group, 10% patients ( n=5) experienced ≥ grade 3 CTCAE toxicity. One patient died of septicemia due to severe lung infection within 30 d after VATS lobectomy. In the SBRT group, one patient suffered from grade 3 radiation pneumonitis. There were no grade 4 or 5 toxicities in SBRT group. Conclusions:This propensity matched analysis suggests that SBRT can be an alternative option to VATS lobectomy for stage I-II NSCLC. Randomized trials are needed to evaluate the outcomes.

Research progress on molecular mechanism of traditional Chinese medicine against non-alcoholic fatty liver disease / 中草药

Non-alcoholic fatty liver disease (NAFLD) is a major form of chronic liver disease in recent years. Its main clinical manifestations are insulin resistance, lipid accumulation, oxidative stress injury, inflammation, and fibrosis of the liver. The pathogenesis of NAFLD is not fully understood, and there is a lack of effective drugs in clinical treatment. Currently, the clinically recommended therapeutic regimen for NAFLD is mainly to change living habits (including diet structure, work and rest schedule, and exercise mode, etc), supplemented by drug therapy (including insulin sensitizer, antioxidant, lipid-lowering drug, and liver-protecting anti-inflammatory drug, etc). However, due to its single composition and clear target, western drugs can only improve some indicators for the complex metabolic diseases such as NAFLD, and also produce some side effects (such as elevated serum transaminase, gastrointestinal discomfort and increased liver burden). Traditional Chinese medicine has the advantages of multi-target comprehensive treatment of NAFLD. Compound traditional Chinese medicine, single Chinese medicine and its active components against NAFLD were summarized and analyzed from the aspects of lowering blood lipid, anti-oxidation, anti-inflammation, anti-fibrosis, and improving insulin resistance of the liver, so as to provide reference for clinical diagnosis and treatment of NAFLD.

Establishment of transgenic mice-derived allografts in pancreatic cancer / 西安交通大学学报(医学版)

Objective: To establish the transgenic mice-derived allografts (MDAs) so as to provide an appropriate experiment model of pancreatic cancer. Methods: By using the tissue fragments derived from transgenic mice with pancreatic cancer named LSL-KrasG12D/+; Trp53fl/+; Pdx1-Cre (KPC) mice and subcutaneous transplant technique, we constructed the MDAs. We evaluated the histopathological characteristics of MDAs and infiltration of immune cells by H&E staining, Masson's staining and immunohistochemical method. Results: MDAs could mimic the pathological morphology, proliferation and fibrosis of pancreatic cancer precisely. Besides, the infiltration of immune cells in MDAs tumor was the same as that of pancreatic cancer. Conclusion: MDAs are an effective model to mimic the development of pancreatic cancer.

Activated mTOR signaling pathway in myofibers with inherited metabolic defect might be an evidence for mTOR inhibition therapies / 中华医学杂志(英文版)

BACKGROUND@#Abnormally activated mechanistic target of rapamycin (mTOR) pathway has been reported in several model animals with inherited metabolic myopathies (IMMs). However, the profiles of mTOR pathway in skeletal muscles from patients are still unknown. This study aimed to analyze the activity of mTOR pathway in IMMs muscles.@*METHODS@#We collected muscle samples from 25 patients with mitochondrial myopathy (MM), lipid storage disease (LSD) or Pompe disease (PD). To evaluate the activity of mTOR pathway in muscle specimens, phosphorylation of S6 ribosomal protein (p-S6) and p70S6 kinase (p-p70S6K) were analyzed by Western blotting and immunohistochemistry.@*RESULTS@#Western blotting results showed that p-p70S6K/p70S6K in muscles from LSD and MM was up-regulated when compared with normal controls (NC) (NC vs. LSD, U = 2.000, P = 0.024; NC vs. MM: U = 6.000, P = 0.043). Likewise, p-S6/S6 was also up-regulated in muscles from all three subgroups of IMMs (NC vs. LSD, U = 0.000, P = 0.006; NC vs. PD, U = 0.000, P = 0.006; NC vs. MM, U = 1.000, P = 0.007). Immunohistochemical study revealed that p-S6 was mainly expressed in fibers with metabolic defect. In MM muscles, most p-S6 positive fibers showed cytochrome C oxidase (COX) deficiency (U = 5.000, P = 0.001). In LSD and PD muscles, p-S6 was mainly overexpressed in fibers with intramuscular vacuoles containing lipid droplets (U = 0.000, P = 0.002) or basophilic materials (U = 0.000, P = 0.002).@*CONCLUSION@#The mTOR pathway might be activated in myofibers with various metabolic defects, which might provide evidence for mTOR inhibition therapy in human IMMs.

Clinical, Neuroimaging, and Pathological Analyses of 13 Chinese Leigh Syndrome Patients with Mitochondrial DNA Mutations / 中华医学杂志(英文版)

Background@#Leigh syndrome (LS) is a rare disease caused by mitochondrial defects and has high phenotypic and genotypic heterogeneity. We analyzed the clinical symptoms, neuroimaging, muscular histopathology, and genotypes of 13 Chinese LS patients with mitochondrial DNA (mtDNA) mutations.@*Methods@#Mutations in mtDNA were identified by targeted sequencing. The brain imaging features on magnetic resonance imaging (MRI) were analyzed. The levels of lactate in fasting blood and cerebrospinal fluid (CSF) were routinely tested. The levels of urinary organic acids, plasma amino acids, and acylcarnitines were examined with gas chromatography-mass spectrometry and tandem mass spectrometry. The histopathological traits of skeletal muscles were analyzed under microscope.@*Results@#Among 13 patients, mutations of MT-NDs (n = 8) and MT-ATP6 (n = 4) genes were most common. Strabismus (8/13), muscle weakness (8/13), and ataxia (5/13) were also common, especially for the patients with late-onset age after 2 years old. However, respiratory distress was common in patients with early-onset age before 2 years old. The most frequently affected brain area in these patients was the brain stem (12/13), particularly the dorsal part of midbrain, followed by basal ganglia (6/13), thalamus (6/13), cerebellum (5/13), and supratentorial white matter (2/13). Besides, the elevated lactate levels in CSF (6/6) were more common than those in serum (7/13). However, the analysis of abnormal plasma amino acid and urinary organic acid showed limited results (0/3 and 1/4, respectively). Muscular histopathology showed mitochondrial myopathy in the three late-onset patients but not in the early-onset ones.@*Conclusions@#Noninvasive genetic screening is recommended for mtDNA mutations in MT-NDs and MT-ATP6 genes in patients with ophthalmoplegia, muscle weakness, ataxia, and respiratory disorder. Furthermore, the lactate detection in CSF and the brain MRI scanning are suggested as the diagnosis methods for LS patients with mtDNA mutations.

Analysis of Percutaneous Biopsy of 41 Small Lung Lesions / 中国肺癌杂志

BACKGROUND@#When lung cancer screening work extensively developed in recent years, more and more small lung lesions were found in clinic. The aim of this study is to analysis computed tomography (CT) guided percutaneous biopsy for lung small lesions (diameter<2 cm) on results, complications and prognosis.@*METHODS@#Choose CT guided percutaneous lung biopsy were performed in 41 cases of pulmonary peripheral lesions, single lesion in 39 cases, multiple lesions in 2 cases, 5-20 (13.1±5.2) mm in maximum diameter, depth from lung surface 1-45 (16.5±13.7) mm, ground-glass opacity (GGO) components 0%-100% (66.8%±35.2%).@*RESULTS@#41 patients and 43 biopsies successfully obtained pathological tissue. Atypical adenomatous hyperplasia in 3 cases, squamous carcinoma in 1 case, adenocarcinoma in 37 cases( carcinoma in situ in 7 cases, micro-invasive carcinoma in 5 cases, invasive adenocarcinoma in 25 cases, double primary lung cancer in 2 cases), inflammatory lesions in 2 cases. Except 2 cases of inflammatory lesions are in follow-up, biopsy and surgical pathology alignment (specificity) was 100%. 41 patients occurred complications related to percutaneous biopsy. Pneumothorax were in 22 cases, drainage required in 2 cases. There were 17 cases with hemoptysis, accounting for 39.5% incidence are self-limited. Intracranial air embolism occurred in 2 cases by 4.6% incidence. They were fully recovered.@*CONCLUSIONS@#For small lung lesions, CT guided percutaneous biopsy is technically feasible. However, for small lung lesions especially pure GGO biopsy, it is still need to be cautious.

Experience of intracavitary surgical therapy for duplex kidney and ureter with upper urinary tract calculi / 中国内镜杂志

Objective To investigate the intracavitary surgical therapy and efficacy for the patients with duplex kidney and ureter with upper urinary tract calculi. Methods The clinical data of twenty-six cases with duplex kidney and ureter with upper urinary tract calculi were retrospectively analyzed. Results Of 26 patients who underwent intracavitary surgical treatment, 5 patients were treated by ureteroscopic lithotripsy (URL), 15 by retrograde intrarenal surgery (RIRS), 6 by mini-percutaneous nephrolithotomy (mPCNL). All 26 cases were performed successfully. No severe complications such as septic shock, heavy blood loss, ureter injury, and pneumothorax occurred. 23 patients with nephrohydrosis were followed up for 2 to 24 months and most of them improved at different extents. Conclusion The current technique of intracavitary surgery in the management of duplex kidney and ureter concomitant with upper urinary tract calculi, has the advantages of less trauma, rapid recovery, safety and so on.

A comparative study on children's basic medical insurance system and policies in four municipalities in China / 上海交通大学学报（医学版）

Objective·To analyze the basic medical insurance policies for children in Shanghai in 2011 and 2017, and compare with those of the other three municipalities to understand the basic medical security status of children in four municipalities in China and analyze the existing problems. Methods?·?The current effective children's basic medical insurance policies and child critical illness insurance policies and related insurance systems in four municipalities were collected systematically. The types of basic medical insurance for children in various municipalities and their funding standards, their own expenses and the proportion of government subsidies, the proportion of outpatient emergency payoff lines and reimbursement at all levels of medical institutions, and the coverage and coverage of major illness insurance reimbursement were collected and compared mainly. Results?·?The basic medical insurance for children in Shanghai is a "double insurance" system, which contained children's hospitalization fund and basic medical insurance for urban and rural residents. The basic medical insurance for children in other municipalities mainly consisted of basic medical insurance for urban (rural) residents. Compared with 2011, the level of financing and the reimbursement of the basic medical insurance for urban and rural residents and of the children's hospitalization fund in Shanghai in 2017 was increased. In 2017, the level of financing of basic medical insurance for urban and rural residents in Shanghai was 1?100 yuan, which was slightly lower than that of Beijing medical insurance for urban residents and new rural cooperative medical insurance and was higher than that of basic medical insurance for urban and rural residents in Tianjin and Chongqing, and the individual payment level of Shanghai was the lowest. The hospitalization payoff lines in Shanghai were lower than those of other municipalities. Shanghai's outpatient and inpatient reimbursement levels were higher those of than other municipalities. There wasn't a capping line in Shanghai. There were capping lines in other municipalities. There was a great difference in the insurance policy of the major diseases in four municipalities. Conclusion?·?According to their own actual conditions, models for children's medical care systems that suited each municipalities’ social and economic development have been established. Shanghai children's basic medical insurance basically meets the needs of children's growth but needs improvement in terms of the type and level of medical security for major illnesses.

Investigation of PRAM1 Expression Features and Their Clinical Significance in AML via Gene Expression Microarray Database / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To study the clinical phenotype and its prognostic value of PRAM1 in patients with acute myeloid leukemia(AML).</p><p><b>METHODS</b>Based on the gene expression microarray platform of 486 AML cases, the PRAM1 expression phenotypes were summarized in all of AML subtypes. The PRAM1 expression features were explored in every differentiation stage of hematocytes through normal human stem cell chips and bone marrow gene expression microarray. The clinical drugs which could up-regulate PRAM1 expression in AML cell lines should be found out.</p><p><b>RESULTS</b>The PRAM1 expression was the richest in the inv(16) AML and the lowest in the t(15;17)M3, almost the same in the other subtypes of AML. By the classification of molecular abnormalities, PRAM1 expression was more in the panel of CN-AML with CEBPAdm than the other two panels. Interestingly, high/low expression of PRAM1 could be re-classified in the CN-AML, and the EFS is statistically significant. It was proven again that PRAM1 is more expressed in the mature granulocytes. Finally, it was confirmed that decitabine and the chidamide could up-regulate PRAM1 expression in AML cell lines, and chidamide effect is better.</p><p><b>CONCLUSION</b>PRAM1 expression is the lowest in t(15;17) M3 and the highest in inv(16) AML. The high expression of PRAM1 is a sign for favorable prognosis in the CN-AML. PRAM1 is more expressed in mature granulocytes, chidamide can up-regulate PRAM1 expression in AML cell lines.</p>