Clinical and genetic analysis of a pedigree with Wolfram syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To investigate the clinical features and genetic background of autosomal recessive Wolfram syndrome caused by WFS1 gene mutation.METHODS: A pedigree with autosomal recessive Wolfram syndrome was studied by clinical examination, gene analysis and bioinformatics.RESULT: It was found that the proband and his brother had diabetes, color weakness and optic neuropathy. In addition, his brother had diabetes insipidus. Whole-exome sequencing(WES)analysis showed that there were two heterozygous variations in the WFS1 gene exon 8 of the two brothers: c.941G>A(p.W314X)and c.2309T>G(p.F770C), and were co-separated from the clinical phenotype in this family.CONCLUSION: The compound heterozygous mutation of WFS1 gene is associated with Wolfram syndrome in this pedigree. Among them, c.941G>A(p.W314X)has not been reported yet.

Drugs and drug delivery strategies for treatment of pulmonary arterial hypertension / 药学学报

Pulmonary arterial hypertension (PAH), also named as a cancer of cardiovascular disease, is a rare disease and has complicated pathogenesis. Recently, there are more understandings of PAH pathogeneses. According to the pathogenesis and active pathways, the clinically used drugs are classified into several groups incluidng prostacyclin analogues and prostacyclin receptor agonists, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase inhibitors, etc. To elevate the efficacy of the drugs, numerous drug delivery systems are developed. This review mainly summarized the pathological mechanism of PAH, drugs and drug delivery approaches in the treatment of PAH.

A new phloroglucinol compound from Dryopteris fragrans / 中国中药杂志

Two phloroglucinol compounds(1-2) were isolated and purified from 95% ethanol extract of Dryopteris fragrans through various column chromatographies on silica gel, Sephadex LH-20, medium pressure column chromatography, and preparative HPLC. Their structures were elucidated as 2',4',6'-trihydroxy-5'-methyl acetate-3'-methyl-1'-butyrophenone(1) and aspidinol B(2) based on their chemical and physicochemical methods and spectroscopic data. Compound 1 is a new phloroglucinol compound named "dryofraginol".

Nonalcoholic Fatty Liver Disease Effect of Emodin Based on AMPK Signaling Pathway / 中国实验方剂学杂志

Emodin is a common Chinese medicine compound with anti-inflammatory, antibacterial, anti-oxidant and lipid-lowering effects. Modern studies have found that emodin activates adenylate-activated protein kinase (AMPK) signaling molecules and regulates transcriptional factors and biological functions of relevant pathways. Nonalcoholic fatty liver disease is a chronic liver disease with a high incidence in China. With the global prevalence of obesity and metabolic syndrome, the development of nonalcoholic fatty liver disease (NAFLD) is closely related to the expression of the metabolism-related signal molecule AMPK. AMPK is a key enzyme in glycolipid metabolism that can involve different stages of NAFLD development to non-alcoholic steatohepatitis (NASH) by regulating energy metabolism in the body. In recent years, many studies have suggested that the activation of AMPK signaling molecules is related to the function realization of emodin, and lipid synthesis, fatty acid oxidation, insulin sensitivity and mitochondrial function-related transcription factors affected by AMPK downstream signaling molecules and other biological effects can be interacted with each other. The detailed mechanism of action associated with AMPK activation provides new thought about the treatment of NAFLD by emodin. This paper mainly summarizes the research progress of emodin by participating in the various stages of NAFLD by AMPK-related signaling pathways through literature retrieval and comprehensive analysis. It lays a foundation for further research on the therapeutic effect and mechanism of emodin on NAFLD.

Analysis of Scores of SCL-90 of Patients with Traumatic Subdural Effusion / 法医学杂志

Objective To investigate the factors affecting changes of Symptom Check List-90 （SCL-90） of patients with traumatic subdural effusion. Methods One hundred and forty-two cases of patients with traumatic subdural effusion from the Center of Forensic Identification, Wannan Medical College collected from 2007-2018 were tested with SCL-90. The differences between SCL-90 results and the national norm and the influences of gender, age, education level, the number of effusion sites and location on SCL-90 results were analyzed. Results The differences between the scores of somatization, interpersonal sensitivity, depression, anxiety, hostility, terror, paranoia and psychosis factors and total mean scores in SCL-90 of traumatic subdural effusion and that of the national norm had statistical significance （P<0.05）. The differences in depression and hostility factor scores between males and females had statistical significance （P<0.05）. The differences in compulsion, anxiety and terror factor scores and total mean scores among different age groups had statistical significance （P<0.05）. The differences in hostility, paranoia and psychosis factors among patients with different degrees of education had statistical significance （P<0.05）. The differences in depression, anxiety, hostility and terror factor scores and total mean scores among single-site group, double-site group and multi-site group had statistical significance （P<0.05）. The differences in somatization, depression and anxiety factor scores and total mean scores between the right cerebral hemisphere group and the left cerebral hemisphere group and bilateral hemisphere group had statistical significance （P<0.05）. Conclusion Traumatic subdural effusion can cause certain psychological changes, which are related to the primary trauma of arachnoid tear as well as the number and location of effusion sites. Attention should be paid to the influence of the above factors during the assessment of psychological symptoms.

Biodegradable micelles for two-photon photodynamic therapy in a mouse model of breast cancer / 药学学报

Photodynamic therapy (PDT) is one of the new approaches for cancer treatment with high efficacy. However, applications of current photosensitizers are restricted to skin and superficial tumor due to poor in vivo targeting ability, poor water solubility and short wavelength excitement, which limits penetration therefore therapeutic depth. Here, a biodegradable polymeric micelle, methoxy poly(ethylene glycol)-polylactide copolymer (mPEG-PDLLA), is employed as drug delivery system to co-encapsulate strong two-photon absorption compound (LTPA) and photosensitizers. This delivery system is designed to target tumor passively, resulting in near infrared light with an approximately 808 nm wavelength becoming able to indirectly excite photosensitizers through fluorescence resonance energy transfer. Tumor cells and microvessels could be damaged by the generated singlet oxygen. The average size of drug loaded micelles was approximately 55 nm and showed a spherical shape. Both compounds could be released simultaneously from micelles under either weak acid and neutral pH conditions. Reactive oxygen species was produced intracellularly during two-photon PDT process and induced cell apoptosis/necrosis, which was quantified by Annexin-V/FITC assays. Time-dependent ex vivo organ distribution and in vivo anticancer efficacy results suggested that the drug carriers could accumulate in tumors and suppress tumor growth by two-photon PDT. All animals experiments were performed in line with national regulations and approved by the Animal Experiments Ethical Committee of College of Pharmaceutical Sciences, Southwest University. In summary, we have employed two-photon PDT for breast cancer treatment successfully in a mouse model and have demonstrated the significance of delivery system in such therapeutics.

Analysis of Effective Substance of Qingre Chushi Decoction by UPLC-Q-TOF-MS / 中国实验方剂学杂志

Objective:To characterize and compare the chemical information of four extracts of Qingre Chushi (QRCS) decoction by liquid chromatography-mass spectrometry (LC-MS), and combine the chemical information of the four extracts with their results of anti-inflammatory effect for a multivariate statistical analysis, in order to identify the compounds directly relating to the anti-inflammatory effects of QRCS decoction. Method:Four extracts of QRCS decoction were characterized by UPLC-Q-TOF-MS:①ethanol extract+water extract,② ethanol extract+supernatant after water extraction and alcohol precipitation, ③ ethanol extract+precipitation after water extraction and alcohol precipitation,and ④ standard decoction. On the basis of the results of inhibition of the four above extracts on xylene-induced ear swelling in mice,multivariate statistical analysis[principal component analysis (PCA) and orthogonal partial least squares-discriminant analysis (OPLS-DA)] were carried out to lock the chromatographic peaks with significant differences between group ① (the best pharmacological action group) and group ④ (standard decoction group). According to the accuracy of quasi-molecular ion and fragment ion data,and the reference materials and literature data,those chromatographic peaks were identified. Result:PCA could cluster the four extracts of QRCS decoction,and the differences between groups was reflected in the distance between groups. Group ④ (standard decoction) had the most significant differences with the other three groups, especially in the first principal component; group ① (ethanol extract+water extract),group ② (ethanol extract+supernatant after water extraction and ethanol precipitation) and group ③ (ethanol extract+precipitation after water extraction and ethanol precipitation) had certain differences in the second principal component. OPLS-DA was used to compare group ① (the best pharmacological action group) and group ④ (standard decoction group). Eleven chromatographic peaks with great contribution and high reliability to group differences,were identified as gentiopicrin,skimmin,baicalin,baicalin isomer,wogonoside,5,6,7-trihydroxy-8-methoxyflavone-7-O-glucurodonaldehyde,5,6-dihydroxy-6,8,2',3'-tetramethoxyflavone,salicin-6-C-arabinose-8-C-glucoside,plantamajoside and glycyrrhizic acid. Conclusion:In the mode of pectrum-effect combination, this study explores and identifies compounds relating to the anti-inflammatory effect of QRCS decoction,so as to provide the basis for screening the extraction and purification process and optimizing the formulation of preparation of Qingre Chushi decoction.

Chronic phosphoproteomic in temporal lobe epilepsy mouse models induced by kainic acid / 北京大学学报(医学版)

OBJECTIVE@#To investigate functions of proteins and signaling pathways involved in epileptogenesis during the chronic stage of temporal lobe epilepsy in mouse models.@*METHODS@#Kainic acid-induced temporal lobe epilepsy models were conducted, when reaching stage 4 using racine scale, the mice of experimental group were supposed to be successfully established. Pentobarbital sodium was injected to stop epileptic seizure in case of death. Twenty-eight days after the kainic acid injection, when the experimental group generally turned into chronic spontaneous seizures, mice hippocampal tissues were extracted from the control and the experimental groups respectively for phosphoproteomic. Enriched phosphorylated proteins were detected using mass spectrometry, only the proteins whose density was greater than 106 were analyzed by matching the Gene Ontology (GO) database, Kyoto Encyclopedia of Genes and Genomes (KEGG) database and STRING database to detect proteins involved in epileptogenesis in protein functions, signaling pathways and protein-protein interaction respectively. After that, literatures were reviewed about the key proteins.@*RESULTS@#(1) Total of 12 697 phosphorylation sites of enriched proteins were detected by mass spectrometry, and there were 159 sites whose phosphorylation levels were significantly different from the control (P<0.001). (2) GO database showed that 35.7% of the 159 sites were about "catalytic activity", 39.5% were about "binding" and 20.8% were about "cell communication", and the 159 proteins also participated in many biological processes, such as "primary metabolic process" "response to stimulus" "developmental process" "localization" and "phosphate-containing compound metabolic process". (3) KEGG database showed that the 159 protein sites mainly involved in 10 signaling pathways: glutamatergic synapse, Ras signaling pathway, African trypanosomiasis, Cocaine addiction, Circadian entrainment, Amyotrophic lateral sclerosis (ALS), Long-term potentiation, Endocytosis, Gap junction, Nicotine addiction. (4) STRING database showed that the protein-protein interaction network formed by the 159 proteins was focused on Grin1/Dlg3, Arhgef 2/Arhgap33/Tiam1 and Sptnb1/3/4/Add3/Ank2 protein group respectively. (5) Phosphorylation levels of Grin1, Arhgef 2, Arhgap33, Tiam1, Sptbn1/2/4 and Ank2 in experimental group were significantly higher than in the control (P<0.001).@*CONCLUSION@#Phosphoproteomic illustrated integral distribution of phosphorylated proteins at the chronic stage of temporal lobe epilepsy in the mouse model. Literatures showed that most key proteins were closely related to epileptogenesis, suggesting that some proteins or signaling pathways may play a role in epileptogenesis, such as dopamine and Kir3.1.

Effect of Twist on angiogenesis of nasopharyngeal cancer cells by regulating ERK signaling pathway / 中国病理生理杂志

AIM:To investigate the effect of Twist transcription factor on angiogenesis of nasopharyngeal cancer cells by regulating ERK signaling pathway in vitro.METHODS:Twist siRNA was transfected into the CNE2 cells with Lipofectamine 2000, and the CNE2 cell-conditioned medium (CM) was prepared, which was then used to treat human umbilical vein endothelial cells (HUVECs).The CCK-8 assay, Boyden chamber and capillary tube formation assay were used to evaluate the angiogenic activity of HUVECs.The mRNA expression of Twist in nasopharyngeal cancer cells was detected by real-time PCR.The protein levels of Twist, ERK, p-ERK, p38, p-p38, JNK, p-JNK and VEGF were detected by Western blot.The ERK, p38 and JNK signaling pathway inhibitors were employed to treat HUVECs, and their effect on the capillary tube formation of HUVECs was analyzed.RESULTS:The expression of Twist at mRNA and protein levels in the CNE2 cells was significantly inhibited after transfection with Twist siRNA (P<0.01).The abilities of proliferation, invasion and capillary tube formation of HUVECs were markedly suppressed after knockdown of Twist expression (P<0.01).The protein level of p-ERK was significantly elevated in the CNE2-siTwist cells, while VEGF expression was decreased.Knockdown of Twist did no change the levels of p-p38 and p-JNK.The ERK signaling pathway inhibitor partly abrogated the anti-angiogenic effect of Twist knockdown.CONCLUSION:Knockdown of Twist inhibits the angiogenesis of nasopharyngeal cancer cells in vitro partly through activation of ERK signaling pathway.

Clinical Features and Outcomes of Ulcerative Colitis Complicated with Acute Massive Lower Gastrointestinal Hemorrhage / 中国医学科学院学报

To analyze the clinical features and prognosis of ulcerative colitis(UC)complicated with acute massive lower gastrointestinal bleeding(LGIB). Methods Eleven patients hospitalized in Peking Union Medical College Hospital from January 2006 to December 2017 for treatment of UC,suffering from acute massive LGIB,were enrolled and descriptively analyzed. Results The proportion of UC patients with acute massive LGIB was 0.7% among all 1486 UC patients hospitalized during the study period.The disease was moderately or severely active in these 11 patients,among whom 9 patients(81.8%)had chronic relapsing pancolitis.Cytomegalovirus infection was present in 5 patients,among whom 4 patients received antiviral treatments.All the 11 patients received treatments including food and water fasting,rehydration,blood transfusion,and use of somatostatin.Four patients received emergency surgical treatment after the first episode of massive bleeding,and 3 of them suffered from re-bleeding after the surgery.Among the remaining seven patients,two underwent emergency total colectomy+subtotal rectectomy+ileostomy and three received elective total resection of colon and rectum or total colectomy+subtotal rectectomy+ileostomy.Thus,9 patients underwent emergency surgery,1 patient did not receive surgey during follow-up,and 1 patient was lost to follow-up. Conclusions Acute massive LGIB is a manifestation of active UC and can be associated with poor prognosis.Optimized perioperative management is important for improving the outcomes of such patients.

Effectiveness of Glaucoma Diagnostic Parameters from Spectral Domain-Optical Coherence Tomography of Myopic Patients / 中华医学杂志(英文版)

Background@#Currently, spectral-domain optical coherence tomography (SD-OCT) appears to be a new type of glaucoma diagnostic tool. Thus, this study aimed to evaluate the effectiveness of glaucoma diagnostic parameters from SD-OCT of patients with different severities of myopia.@*Methods@#This was a cross-sectional study. A total of 248 participants (248 eyes) were enrolled, including 51 cases in the early primary open-angle glaucoma group, 79 cases in the control group (0.50 D to -0.50 D, excluding -0.50 D), 47 cases in the low-myopic group (-0.50 to -3.00 D, excluding -3.00 D), 43 cases in the moderate-myopic group (-3.00 to -6.00 D, excluding -6.00 D), and 28 cases in the high-myopic group (≤-6.00 D). All participants were examined using the Humphrey visual field test and SD-OCT. The SD-OCT parameters of the retinal nerve fiber layer (RNFL) and ganglion cell complex were analyzed statistically using the receiver operating characteristic curve and area under the curve (AUC).@*Results@#The AUC showed that the best parameters for the control and low-myopic groups were the inferior and inferior temporal RNFL thicknesses (AUC >0.94), respectively; for the moderate- and high-myopic groups, the best parameter was the temporal low RNFL thickness (AUC, 0.926 and 0.896, respectively). The AUC of the inferior parameters of the moderate-myopic group (0.864) was lower, ranked 15 among all RNFL parameters. When the sensitivity was fixed at 85%, the specificity of the inferior, superior, inferior temporal, and superior temporal quadrants was higher (>80%) in the control and low-myopic groups, while they were lower (20-60%) for the moderate- and high-myopia groups. The green color based on the OCT database was also less for the high-myopic group compared with that of other groups (P < 0.05).@*Conclusions@#Glaucoma diagnostic parameters from SD-OCT were not clinically effective for the moderate- and high-myopic groups. The specificities were low. The moderate- and high-myopic groups require comprehensive analyses for the diagnoses of glaucoma. The SD-OCT database should be improved to better indicate the level of myopia based on the corresponding diopter readings.

Clinical Features of Pneumocystis Jiroveci Pneumonia in Patients with Inflammatory Bowel Disease / 中国医学科学院学报

Objective To investigate the clinical features of patients with inflammatory bowel disease (IBD) complicated with Pneumocystis Jiroveci Pneumonia (PJP). Methods We retrospectively analyzed the clinical data of 5 patients who were hospitalized in Peking Union Medical College Hospital from January 2012 to July 2017 for treatment of IBD complicated with PJP. Demographic characteristics,clinical manifestations,treatments,and outcomes were descriptively analyzed. Results Of these five patients,four had ulcerative colitis (UC) and one had Crohn's disease (CD). All patients were males,with an average age of (61.8±1.9) years. All patients were in active disease status and had symptoms including cough and suffocation. Three patients had hypoxemia,among whom two developed type 1 respiratory failure. Three patients were treated with immunosuppressive medications (corticosteroids and/or immunosuppressant drugs) before the diagnosis of PJP. Lymphocyte counts in three patients were less than 0.6×10/L. CD4+T cells in two patients were less than 200×10/L. Four patients had elevated serum cytomegalovirus DNA. The level of β-D-glucan was elevated in four patients. Chest CT showed bilateral diffuse ground glass opacification. PJP-DNA was positive in sputum or bronchoalveolar lavage fluid in all patients. Two patients with type 1 respiratory failure required invasive mechanical ventilation. All patients received trimethoprim-sulfamethoxazole and methylprednisolone treatment. Four patients recovered completely and one died. Conclusion Elderly (aged>55 years) IBD patients who are receiving immune-suppressive therapy or with decreased peripheral blood lymphocyte count are at higher risk of PJP.

Quality Control of Realgar in Compound Huanglian Ointment / 中国中医药信息杂志

Objective To control the quality of realgar in compound Huanglian Ointment.Methods As2O3which is the toxic component of realgar was carried limit test by Gutzeit's test; Potassium sulfate, ammonium sulfate and sulfuric acid were used to digest, and then titration method was used to determine the content of As2S2in compound Huanglian Ointment. Results The content of the soluble As in compound Huanglian Ointment was no higher than 15.6 μg/g. The content of As2S2in compound Huanglian Ointment was no less than 2.21 mg/g. Conclusion The method is simple and easy,which can be applied as the quantity control method of compound Huanglian Ointment.

Clinical efficacy of laparoscopic extralevator abdominoperineal excision for low rectal cancer without turning position / 中华消化外科杂志

Objective To explore the clinical efficacy of laparoscopic extralevator abdominoperineal excision (laparoscopic ELAPE) for low rectal cancer with modified Lloyd-Davies lithotomy position and without turning position.Methods The retrospective cross-sectional study was conducted.The clinicopathological data of 27 patients with low rectal cancer who underwent laparoscopic ELAPE without turning position in the West China Hospital of Sichuan University from September 2013 to January 2015 were collected.The modified Lloyd-Davies lithotomy position was used in perineal resection.Observation indicators:(1) surgical situation;(2) postoperative recovery situation;(3) postoperative pathological examination situation;(4) follow-up and survival situations.Follow-up using outpatient examination and telephone interview was performed to detect postoperative complications,survival of patients and tumor recurrence or metastasis up to March 2017.Measurement data with normal distribution were represented as (x)±s.Measurement data with skewed distribution were described as M (range).Results (1) Surgical situation:A total of 27 patients received laparoscopic ELAPE without turning position,and operation time and volume of intraoperative blood loss were (198±51)minutes and (85±66)mL.Among 5 of 27 patients with intraoperative complications,1 with intestinal perforation received successful intraoperative repair,1with presacral haemorrhage received successful hemostasis by intraoperative gauze pressing,1 with left and right pelvic plexus injury didn't receive special treatment,1 with left pelvic plexus injury + left internal iliac vein injury didn't receive special treatment and were repaired in vascular injury repair,1 with right neurovascular bundle injury didn't receive special treatment of nerve injury and received successful hemostasis by ultrasonic scalpel.There was no perforation in the site of the tumor.Number of lymph node dissected was 14 (range,9-22),and number of lymph node dissected ≥ 12 and ＜ 12 were detected in 15 and 12 patients,respectively.(2)Postoperative recovery situation:time to anal exsufflation and time for fluid diet intake in 27 patients were respectively (78±21)hours and (83±21)hours.Of 27 patients,8 with postoperative complications were improved by conservative treatment,including 1 in Clavien-Dindo Ⅰ (volume of perineal exudation ＞ 100 mL) and 7 in Clavien-Dindo Ⅱ (3 with pulmonary infection,2 with chylous fistula,1 with perineal incision infection and 1 with hematuria).There was no death within 30 days postoperatively.The median duration of hospital stay of 27 patients was 7 days (range,6-8 days).(3) Postoperative pathological examination situation:of 27 patients,1 and 26 had respectively positive and negative circumferential margins and median distance of circumferential margin was 0.7 cm (range,0.1-1.1 cm).T stage:14,12 and 1 patients were respectively detected in T2,T3 and T4.N stage:18,6 and 3 patients were respectively found in N0,N1 and N2.(4) Follow-up and survival situations:25 of 27 patients were followed up for 2-32 months,with a median time of 24 months.During the follow-up,5 had complications after discharge from hospital.Of 5 patients,2 with persistent anal pain didn't receive special treatment and were not relieved,and 3 with sexual dysfunction didn't receive special treatment and were followed up or observed.Of 25 patients,2 died of tumor-related diseases,1 died of non-tumor-related disease and other 22 had survival.No local tumor recurrence was detected.Eight patients had tumor distant metastases,including 4 with pulmonary metastases,3 with hepatic metastases and 1 with brain metastasis.Conclusion Laparoscopic ELAPE by modified Lloyd-Davies lithotomy position without turning position is safe and feasible,with closing pelvic floor peritoneum in stage Ⅰ.

Advances in the Avellino corneal dystrophy / 国际眼科杂志(Guoji Yanke Zazhi)

Avellino corneal dystrophy(ACD) is an autosomal dominant eye disorder caused by mutation of R124H in the transforming growth factor-beta induced gene (TGFBI) on chromosome 5,which was responsible for accumulating of abnormal TGFBI.Although the underlying mechanism by which mutations cause abnormal TGFBI deposition is not yet clear,but we have a better understanding of the etiology and possible pathogenesis of corneal dystrophy with the rapid development of human genetics and molecular biology,and summarizes the current achievement of this disease and understand the roles of TGFBI and its interaction with Periostin,which may contribute to further research in ACD.

The effect of positive pressure ventilation on dust removal in whole lung lavage / 中国职业医学

OBJECTIVE: To explore the effect of positive-pressure ventilation on dust removal in whole lung lavage( WLL).METHODS: By random sample method,21 patients with stage Ⅱ or Ⅲ pneumoconiosis were chosen for different WLL.Using the patients' own left and right lung was used for matched control study. The positive pressure ventilation was performed at the end of the 3rd,6th,9th and 12 th lavage in treatment lung( treatment groups). The positive-pressure ventilation was not implemented at the end of the 3rd and 6th lavage in the contralateral lung( control groups) but implemented at the end of the 9th,11 th and 12 th lavage. The recovery of lavage fluid,dust and dust concentration drained from 4th to 9th lavage were compared in the two groups. RESULTS: There was no statistical difference in the recovery of the lavage fluid in the 4th to 9th lavage in the two groups( P > 0. 05). The amount of dust and the dust concentration in the fourth lavage drainage in the treatment group was higher than that in the control group( P < 0. 01). The amount of dust and the dust concentration in the 6th,8th and 9th lavage drainage in the treatment group was lower than that in control group( P < 0. 01). The amount of dust and the dust concentration in the 3rd positive pressure ventilation were higher than that in the 6th positive pressure ventilation in the treatment group( P < 0. 01). The total amount of dust in the treatment group was higher than that in the the control group( P < 0. 01). CONCLUSION: In whole lung lavage,the positive pressure ventilation can accelerate the discharge of dust in the lung of patients with pneumoconiosis.

Clinical feature of 9 patients harboring mtDNA G11778 A mutation with Leber hereditary optic neuropathy / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To evaluate the clinical feature of 9 patients harboring mitochondrial DNA ( mtDNA ) G11778A mutation with Leber hereditary optic neuropathy ( LHON) . ●METHODS: Nine LHON patients were enrolled and followed- up between 2012 to 2015 in Shenzhen Eye Hospital, clinical data were collected and analyzed. ●RESULTS: Six cases had maternal inheritance history ( 67%) . Three were sporadic cases. The patients aged from 9 to 43 years old, with average age of (22. 00±9. 42) years. Simultaneous onset with both eyes was in 5 cases (56%). Successively onset was in other 4 cases (44%). The ratio between male and female was 2:1. In the last follow-up, the visual acuity was finger counted in 2 eyes (11%), 0. 01-0. 1 in 12 eyes (67%), 0. 12-0. 4 in 2 eyes (11%),≥0. 4 in 2 eyes (11%). All patients had pale disc and clear boundary. ln the Humphrey visual field examination, 10 eyes had typically cecocentral or paracentral scotoma, 8 eyes had diffuse visual field defect. ●CONCLUSION: ln the 9 LHON patients with mtDNA G11778A mutation, simultaneous onset cases were more than successively onset cases within 1y cases. In most cases, LHON patients kept stable visual acuity. Rare cases had a raise in visual acuity within 1y. Majority patients had typically cecocentral or paracentral visual field scotoma. In the last stage of LHON, visual field present diffused defect. The secondary affected eye was similar performed defect as the former one.

Chemical constituents from Dianthus superbus / 中草药

To investigate the chemical constituents of Dianthus superbus. The chemical constituents were isolated from 95% alcohol extract of D. superbus by silica gel column chromatography, Sephadex LH-20 column chromatography, and HPLC. Their chemical structures were elucidated on the basis of 1D NMR and ESI-MS spectroscopic analyses. Nine compounds including three flavanoids, two sterols, three triterpenes, and one γ-lactone were isolated. Their structures were identified as 5-hydroxy-7, 3', 4'-trimethoxyflavanone (1), 5, 3'-dihydroxy-7, 4'-dimethoxyflavanone (2), 5, 4'-dihydroxy-7, 3'-dimethoxyflavanone (3), β-spinasterol (4), sterculin A (5), (24R)-cycloart-25-ene-3β, 24-diol (6), (24S)-cycloart-25-ene-3β, 24-diol (7), stigmast-7-en-3β-ol (8), and hydoxydihydrobovolide (9). Conclusion All the compounds (1-9) are isolated from D. superbu for the first time.

Design, synthesis and biological evaluation of novel 1,3 dioxolo 4,5-fisoindolone derivatives / 药学学报

A series of [1,3]dioxolo[4,5-f]isoindolone derivatives were designed, synthesized and evaluated as inhibitors of acetylcholinesterases (AChE). Furthermore, their effects on memory impairment of mice induced by scopolamine were investigated with step-through test. The results suggested that most of the target compounds exhibited potential inhibition on AChE with IC50 values at micromolar range. Compounds I1 (IC50 value of 0.086 μmol · L(-1)) and I2 (IC50 value of 0.080 μmol · L(-1)) showed the strongest AChE inhibitory activity, which are equipotent to donepezil (IC50 value of 0.094 μmol · L(-1)). Moreover, compounds I1-I4 could improve the memory impairment induced by scopolamine in mice.

Design, synthesis and biological evaluation of novel 4-substituted-3-nitrobenzamide derivatives / 药学学报

A series of novel 4-substituted-3-nitrobenzamide derivatives were designed and synthesized. The structures of the target compounds were confirmed with 1H NMR, 13C NMR, MS and element analysis. Anti-tumor activities against HCT-116, MDA-MB435 and HL-60 cell lines in vitro were evaluated by SRB assay. The results indicated most of the target compounds exhibited potent anti-tumor activity. Compound 4a showed the most potent inhibitory activities against three cancer cell lines with the GI50 values of 1.904-2.111 micromol x L(-1). Compounds 4g, 41-4n exhibited more potent inhibitory activities against MDA-MB435 and HL-60 cell lines with the GI50 values of 1.008-3.586 micromol x L(-1) and 1.993-3.778 micromol x L(-1), respectively. The structure-activity relationship of these compounds is discussed preliminarily.