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Clinical characterization and diagnosis of cystic fibrosis through exome sequencing in Chinese infants

Liru Qiu, Fengjie Yang, Yonghua He, Huiqing Yuan, Jianhua Zhou

《医学前沿（英文）》 2018年第12卷第5期页码 550-558 doi: 10.1007/s11684-017-0567-y

摘要：

Cystic fibrosis (CF) is a fatal autosomal-recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. CF is characterized by recurrent pulmonary infection with obstructive pulmonary disease. CF is common in the Caucasian population but is rare in the Chinese population. The symptoms of early-stage CF are often untypical and may sometimes manifest as Bartter syndrome (BS)-like hypokalemic alkalosis. Therefore, the ability of doctors to differentiate CF from BS-like hypokalemic alkalosis in Chinese infants is a great challenge in the timely and accurate diagnosis of CF. In China, sporadic CF has not been diagnosed in children younger than three years of age to date. Three infants, who were initially admitted to our hospital over the period of June 2013 to September 2014 with BS-like hypokalemic alkalosis, were diagnosed with CF through exome sequencing and sweat chloride measurement. The compound heterozygous mutations of the CFTR gene were detected in two infants, and a homozygous missense mutation was found in one infant. Among the six identified mutations, two are novel point mutations (c.1526G>C and c.3062C>T) that are possibly pathogenic. The three infants are the youngest Chinese patients to have been diagnosed with sporadic CF at a very early stage. Follow-up examination showed that all of the cases remained symptom-free after early intervention, indicating the potential benefit of very early diagnosis and timely intervention in children with CF. Our results demonstrate the necessity of distinguishing CF from BS in Chinese infants with hypokalemic alkalosis and the significant diagnostic value of powerful exome sequencing for rare genetic diseases. Furthermore, our findings expand the CFTR mutation spectrum associated with CF.

关键词： cystic fibrosis pseudo-Bartter syndrome hypokalemic alkalosis CFTR gene mutations infants diagnosis

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mutation c.1210-3C>G in with a poly-T tract of 5T affects mRNA splicing in a Chinese patient with cysticfibrosis

《医学前沿（英文）》 2022年第16卷第1期页码 150-155 doi: 10.1007/s11684-021-0846-5

摘要： Cystic fibrosis (CF) is a rare autosomal recessive disease with only one pathogenic gene cystic fibrosis transmembrane conductance regulator (CFTR). To identify the potential pathogenic mutations in a Chinese patient with CF, we conducted Sanger sequencing on the genomic DNA of the patient and his parents and detected all 27 coding exons of CFTR and their flanking intronic regions. The patient is a compound heterozygote of c.2909G>A, p.Gly970Asp in exon 18 and c.1210-3C>G in cis with a poly-T of 5T (T5) sequence, 3 bp upstream in intron 9. The splicing effect of c.1210-3C>G was verified via minigene assay in vitro, indicating that wild-type plasmid containing c.1210-3C together with T7 sequence produced a normal transcript and partial exon 10-skipping-transcript, whereas mutant plasmid containing c.1210-3G in cis with T5 sequence caused almost all mRNA to skip exon 10. Overall, c.1210-3C>G, the newly identified pathogenic mutation in our patient, in combination with T5 sequence in cis, affects the CFTR gene splicing and produces nearly no normal transcript in vitro. Moreover, this patient carries a p.Gly970Asp mutation, thus confirming the high-frequency of this mutation in Chinese patients with CF.

关键词： cystic fibrosis CFTR splicing mutation minigene

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Diagnosis and management against the complications of human cystic echinococcosis

Hao WEN, Tuerganaili AJI, Ying-Mei SHAO,

《医学前沿（英文）》 2010年第4卷第4期页码 394-398 doi: 10.1007/s11684-010-0180-9

摘要： Cystic echinococcosis (CE) (hydatidosis, hydatid disease) is a zoonosis caused by the larval stage of , typically affecting the liver. Hepatic cystic echinococcosis (HCE) is often asymptomatic, and symptoms occur largely when complications develop. Up to one-third of HCE can be shown their complications such as rupture of the cyst, secondary infection, and anaphylactic reaction. Clinically, patients present with pain, obstructive jaundice, cholangitis, anaphylactic reaction, and shock. Early diagnosis and treatment of complications of CE must be very important, since mortality is high when obstruction of the biliary ducts occurs, leading to ascending cholangitis and septicemia, anaphylactic shock, or even life-threatening conditions.

关键词： cystic echinococcosis complication diagnosis treatment

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Strategies for preventing peritoneal fibrosis in peritoneal dialysis patients: new insights based on

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《医学前沿（英文）》 2017年第11卷第3期页码 349-358 doi: 10.1007/s11684-017-0571-2

摘要：

Peritoneal dialysis (PD) is an established form of renal replacement therapy. Long-term PD leads to morphologic and functional changes to the peritoneal membrane (PM), which is defined as peritoneal fibrosis, a known cause of loss of peritoneal ultrafiltration capacity. Inflammation and angiogenesis are key events during the pathogenesis of peritoneal fibrosis. This review discusses the pathophysiology of peritoneal fibrosis and recent research progress on key fibrogenic molecular mechanisms in peritoneal inflammation and angiogenesis, including Toll-like receptor ligand-mediated, NOD-like receptor protein 3/interleukin-1β, vascular endothelial growth factor, and angiopoietin-2/Tie2 signaling pathways. Furthermore, novel strategies targeting peritoneal inflammation and angiogenesis to preserve the PM are discussed in depth.

关键词： peritoneal dialysis peritoneal fibrosis inflammation angiogenesis

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Expression of integrin in hepatic fibrosis and intervention of resveratrol

Jianye WU, Chuanyong GUO, Jun LIU, Xuanfu XUAN

《医学前沿（英文）》 2009年第3卷第1期页码 100-107 doi: 10.1007/s11684-009-0013-x

摘要： The aim of this study was to explore the expression of integrin-β1 in different stages of hepatic fibrosis and intervention of resveratrol as well as the way by which integrin-β1 promoted hepatic fibrosis. Hepatic fibrosis models of male Sprague Dawley (SD) rats were created and intragastric administration of resveratrol was given in low (40 mg/kg), middle (120 mg/kg) and high (200 mg/kg) dose groups. The expression of integrin-β1, tumor growth factor-β (TGF-β) and tissue inhibitor of metalloproteinase-1 (TIMP-1) in different stages of hepatic fibrosis was detected by using RT-PCR. The expression of hexadecenoic acid (HA) and precollagen III (pc III) was assayed by radioimmunoassay. The expression of integrin-β1, TGF-β and TIMP-1 was determined in each group. Liver function and pathological sections of each group in different stages of hepatic fibrosis was tested to judge the therapeutic efficacy of resveratrol at different doses. The expression of integrin-β1 in normal control group was low and steady and was not increased as the development of hepatic fibrosis, but it is increased in other groups. The expression levels of integrin-β1 in the model control group (0.878±0.03, <0.01) and low dose group (0.855±0.04, <0.01) were higher than other groups, but there was no difference between model control group and low dose group ( >0.05). The expression levels of integrin-β1 and TGF-β in middle dose group and high dose group were higher than other groups ( <0.01). The expression levels of integrin-β1 and TGF-β in model control group and low dose group were lower than the normal control group ( <0.01). The expression levels of TIMP-1 in the model control and low dose groups were higher than the other groups ( <0.01). The expression levels of TIMP-1 in the middle dose group and the high dose group were lower than the normal control group ( <0.01). The expression of integrin-β1 existed in all stages of hepatic fibrosis of SD rats, and it was increased as the development of hepatic fibrosis. The expression of TGF-β and TIMP-1 was consistent with that of integrin-β1 in different stages of hepatic fibrosis. Resveratrol could improve the degree of hepatic fibrosis of SD rats and decrease the expression of integrin-β1 markedly at a dose of 120 mg/kg.

关键词： liver fibrosis integrin-β1 resveratrol tumor growth factor-β tissue inhibitor of metalloproteinase-1

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Chronic inhibition of cyclic guanosine monophosphate-specific phosphodiesterase 5 prevented cardiac fibrosis

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《医学前沿（英文）》 2014年第8卷第4期页码 445-455 doi: 10.1007/s11684-014-0378-3

摘要：

Recent evidences suggested that cyclic guanosine monophosphate-specific phosphodiesterase 5 (PDE5) inhibitor represents an important therapeutic target for cardiovascular diseases. Whether and how it ameliorates cardiac fibrosis, a major cause of diastolic dysfunction and heart failure, is unknown. The purpose of this study was to investigate the effects of PDE5 inhibitor on cardiac fibrosis. We assessed cardiac fibrosis and pathology in mice subjected to transverse aortic constriction (TAC). Oral sildenafil, a PDE5 inhibitor, was administered in the therapy group. In control mice, 4 weeks of TAC induced significant cardiac dysfunction, cardiac fibrosis, and cardiac fibroblast activation (proliferation and transformation to myofibroblasts). Sildenafil treatment markedly prevented TAC-induced cardiac dysfunction, cardiac fibrosis and cardiac fibroblast activation but did not block TAC-induced transforming growth factor-β1 (TGF-β1) production and phosphorylation of Smad2/3. In isolated cardiac fibroblasts, sildenafil blocked TGF-β1-induced cardiac fibroblast transformation, proliferation and collagen synthesis. Furthermore, we found that sildenafil induced phosphorylated cAMP response element binding protein (CREB) and reduced CREB-binding protein 1 (CBP1) recruitment to Smad transcriptional complexes. PDE5 inhibition prevents cardiac fibrosis by reducing CBP1 recruitment to Smad transcriptional complexes through CREB activation in cardiac fibroblasts.

关键词： PDE5 cardiac fibrosis TGF-β CREB

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Diffuse cystic lung diseases

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《医学前沿（英文）》 2013年第7卷第3期页码 316-327 doi: 10.1007/s11684-013-0269-z

摘要：

Diffuse cystic lung diseases are uncommon but can present a diagnostic challenge because increasing number of diseases have been associated with this presentation. Cyst in the lung is defined as a round parenchymal lucency with a well-defined thin wall (<2 mm thickness). Focal or multifocal cystic lesions include blebs, bullae, pneumatoceles, congenital cystic lesions, traumatic lesions, and several infectious processes such as coccidioidomycosis, Pneumocystis jiroveci pneumonia, and hydatid disease. “Diffuse” distribution in the lung implies involvement of all lobes. Diffuse lung involvement with cystic lesions can be seen in pulmonary lymphangioleiomyomatosis, pulmonary Langerhans’ cell histiocytosis, lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, amyloidosis, light chain deposition disease, honeycomb lung associated with advanced fibrosis, and several other rare causes including metastatic disease. High-resolution computed tomography of the chest helps define morphologic features of the lung lesions as well as their distribution and associated features such as intrathoracic lymphadenopathy. Correlating the tempo of the disease process and clinical context with chest imaging findings serve as important clues to defining the underlying nature of the cystic lung disease and guide diagnostic evaluation as well as management.

关键词： cyst lung disease interstitial lung disease lymphangioleiomyomatosis Langerhans’ cell histiocytosis Birt-Hogg-Dubé syndrome

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Mature cystic teratoma of the parotid gland: a case report and review of the literature

Lenan SHAO DMD, MD, Hanxiong GUAN MD, Jie WAN MS,

《医学前沿（英文）》 2009年第3卷第4期页码 503-506 doi: 10.1007/s11684-009-0076-8

摘要： We report one case of mature cystic teratoma that presented as a tender, well-defined round mass in the right parotid gland region. Teratomas are common neoplasms but are very rarely found in the parotid gland region, so diagnosis and treatment when such occurs is a challenge. Magnetic resonance imaging scanning is useful in determining the nature of teratomas, and only surgery and pathology can provide a final diagnosis. The treatment strategy is to preserve both the facial nerve and the surrounding parotid gland tissue, while completely extirpating the teratoma.

关键词： teratoma parotid gland

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Effect of Feixian Recipe on laminin, collagen I and III in rats with pulmonary fibrosis induced by bleomycin

ZHANG Xiaomei, JIANG Liangduo, ZHANG Wei, WU Jianjun, LU Xiangfeng

《医学前沿（英文）》 2008年第2卷第3期页码 314-316 doi: 10.1007/s11684-008-0060-8

摘要： The aim of this paper is to observe the effect of the Feixian Recipe on pulmonary fibrosis in rats. A rat model with pulmonary fibrosis was established by intratracheal injection of bleomycin. On days 14, 28 and 45, the contents of laminin, collagen I and collagen III in lung tissue homogenate in the model group, the sham operated group, the Feixian group and the prednisone group were measured. The contents of laminin and collagen I and III were decreased significantly by the Feixian Recipe. Feixian Recipe has a significant therapeutic effect on bleomycin-induced pulmonary fibrosis in rats.

关键词： bleomycin intratracheal injection prednisone collagen bleomycin-induced pulmonary

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Paeoniflorin prevents hepatic fibrosis of by inhibiting TGF-β1 production from macrophages in mice

CHU Deyong, LI Conglei, SHEN Jilong, WU Qiang

《医学前沿（英文）》 2008年第2卷第2期页码 154-165 doi: 10.1007/s11684-008-0029-7

摘要： In order to investigate the effect of paeoniflorin (PAE) on hepatic fibrosis of mice with and , a model of hepatic fibrosis caused by schistosomiasis was established in mice infected with cercariae of . Then, PAE was orally administered before and after praziquantel treatment and both therapeutics were given simultaneously at different time points after the infection. The concentration of serum hyaluronic acid (HA) was determined by radioimmunoassay (RIA). Hepatic granuloma and fibrosis were evaluated via HE and Masson staining. The expression of ?-smooth muscle actin (?-SMA), transforming growth factor ?1 (TGF-?1) and collagen I (Col I) protein was detected by immunohistochemistry. The effect of soluble egg antigen (SEA) and PAE on the production of TGF-?1 from mouse peritoneal macrophages (PM?s) was investigated by RT-PCR, Western blotting and ELISA. The effect of TGF-?1 in optimum macrophage-conditioned medium (OPMCM) on the proliferation of hepatic stellate cells (HSCs) and collagen secretion from HSCs with anti-TGF-?1 antibody was explored by MTT assay and ELISA. The results show that PAE could significantly reduce the concentration of serum HA, the size of egg granuloma, the severity of hepatic fibrosis and the expression of ?-SMA, TGF-?1 and Col I protein in the pre-treatment group. However, in sim- or post-treatment group, PAE did not have any significant therapeutic effect. TGF-?1 could be secreted from PM?s stimulated by SEA. Meanwhile, the production of TGF-?1 from PM?s could be depressed significantly by PAE in a concentration-dependent manner. TGF-?1 could promote the proliferation of HSCs and the secretion of collagens. In a word, PAE can prevent hepatic granuloma and fibrosis caused by schistosomiasis japonica through the inhibition of the secretion of TGF-?1 from PM?s, the proliferation and activation of HSCs and the secretion of collagens from HSCs.

关键词： cercariae collagen hyaluronic OPMCM soluble

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mycelium inhibits epithelial-mesenchymal transition by inactivating the midkine pathway in pulmonary fibrosis

Li Lu, Haiyan Zhu, Hailin Wang, Huaping Liang, Yayi Hou, Huan Dou

《医学前沿（英文）》 2021年第15卷第2期页码 313-329 doi: 10.1007/s11684-020-0737-1

摘要： The medical fungus has been used as a Chinese folk health supplement because of its immunomodulatory properties. Our previous studies established the antifibrotic action of mycelium (HSM) in the lung. The epithelial–mesenchymal transition (EMT) is involved in the pathogenesis of idiopathic pulmonary fibrosis. The present study investigates the role of HSM in mediating EMT during the development of pulmonary fibrosis. HSM significantly inhibits bleomycin (BLM)-induced pulmonary fibrosis by blocking the EMT. In addition, the expression levels of midkine are increased in the lungs of the BLM-induced group. Further analysis of the results indicates that the mRNA level of midkine correlated positively with EMT. HSM markedly abrogates the transforming growth factor β-induced EMT-like phenotype and behavior . The activation of midkine related signaling pathway is ameliorated following HSM treatment, whereas this extract also caused an effective attenuation of the induction of EMT (caused by midkine overexpression) . Results further confirm that oral medication of HSM disrupted the midkine pathway . Overall, findings suggest that the midkine pathway and the regulation of the EMT may be considered novel candidate therapeutic targets for the antifibrotic effects caused by HSM.

关键词： epithelial−mesenchymal transition H. sinensis mycelium midkine pulmonary fibrosis

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Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis

null

《医学前沿（英文）》 2012年第6卷第4期页码 395-405 doi: 10.1007/s11684-012-0231-5

摘要：

Pulmonary lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that targets women during their reproductive years. A confident diagnosis can often be based on clinical grounds, but diagnostic certainty requires pathological analysis. Although surgical lung biopsy is considered the gold standard for obtaining tissue in patients with diffuse lung disease, it is also associated with higher morbidity and mortality than alternative, less invasive techniques. The objective of our study was to examine the utility of transbronchial biopsy in the diagnosis of LAM. We conducted two online surveys of over 1 000 LAM patients registered with the LAM Foundation who were accessible by email. Transbronchial biopsy specimens were subsequently collected and reviewed by an expert pathologist to validate the diagnosis. We found that transbronchial biopsy has a yield of approximately 60% in patients with LAM. We conclude that transbronchial biopsy may be a safe and effective method for establishing the diagnosis of LAM, obviating the need for surgical lung biopsy in more than half of LAM patients.

关键词： lymphangioleiomyomatosis lymphangiomyomatosis multicystic lung disease diffuse cystic lung disease transbronchial biopsy perivascular epithelioid cell tumor (PEComa) HMB-45

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自体骨髓干细胞移植与归元方联用治疗急慢性肝损伤实验研究

吴理茂,李连达,刘红,宁可永,李贻奎

《中国工程科学》 2004年第6卷第7期页码 34-42

摘要：

研究中药归元方与自体骨髓干细胞移植对急慢性肝损伤的治疗作用。研究方法：用肝脏局部注射乙醇的方法复制急性局限性肝损伤模型，复合因素（CCl₄、乙醇、高脂、低蛋白）刺激复制大鼠肝纤维化模型，通过定量组织学、肝功能检查、免疫组化、肝组织羟脯氨酸含量、损伤或纤维区骨髓干细胞观察等综合评价中药、自体骨髓干细胞移植及两者合用的疗效。结果：归元方与自体骨髓干细胞移植可减小肝损伤区域，改善肝功能，使纤维肝组织表达μPA增强，降低血清ALT，AST，PCⅢ，HA和肝组织羟脯氨酸的含量，改善肝组织肝纤维化评分，骨髓干细胞能在肝损伤、肝纤维化形成环境中存活、增殖，并向肝细胞分化，表达肝脏特异的角蛋白CK18。结论：归元方与自体骨髓干细胞移植对急慢性肝损伤有明确的治疗作用，两者合用可优势互补，协同增效。临床上有良好的应用前景。

关键词：归元方骨髓干细胞自体移植肝损伤肝纤维化

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γδ T cells in liver diseases

null

《医学前沿（英文）》 2018年第12卷第3期页码 262-268 doi: 10.1007/s11684-017-0584-x

摘要：

γδ T cells display unique developmental, distributional, and functional patterns and can rapidly respond to various insults and contribute to diverse diseases. Different subtypes of γδ T cells are produced in the thymus prior to their migration to peripheral tissues. γδ T cells are enriched in the liver and exhibit liver-specific features. Accumulating evidence reveals that γδ T cells play important roles in liver infection, non-alcoholic fatty liver disease, autoimmune hepatitis, liver fibrosis and cirrhosis, and liver cancer and regeneration. In this study, we review the properties of hepatic γδ T cells and summarize the roles of γδ T cells in liver diseases. We believe that determining the properties and functions of γδ T cells in liver diseases enhances our understanding of the pathogenesis of liver diseases and is useful for the design of novel γδ T cell-based therapeutic regimens for liver diseases.

关键词： γδT cells liver infection non-alcoholic fatty liver disease autoimmune hepatitis liver fibrosis and cirrhosis liver cancer liver regeneration

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Effect of pirfenidone on renal tubulointerstitial fibrosis

Dixin LI MM , Hongbing ZENG MD , Chunyang JI MM ,

《医学前沿（英文）》 2009年第3卷第3期页码 316-322 doi: 10.1007/s11684-009-0045-2

摘要： Renal tubulointerstitial fibrosis (TIF) is the common end stage of various chronic renal diseases, and pirfenidone (PFD) is a novel, broad-spectrum anti-fibrotic compound but little is known about its effect and mechanism of action on renal TIF. In this work, we employed a unilateral ureteral obstruction (UUO) rat model to investigate the apoptosis of renal tubular epithelial cells (RTC) after PFD treatment. Thirty-five Sprague Dawley (SD) rats were randomized into three groups: sham-operated group (=7), UUO group (=14) and PFD group (=14). All rats were sacrificed at day 7 or 14 after operation. Renal histology was studied by using periodic acid schiff reagent (PAS) and Masson trichromic stain (MASSON); apoptosis was detected by terminal deoxynucleotide transferase-mediated dUTP-biotin nick end-labeling (TUNEL); tubular caspase-3 expression was assessed by immunohistochemistry. The content of malondialdehyde (MDA) and total activity of superoxide dismutase (T-SOD) in the renal cortex was determined by chemical colorimetry method. TIF, apoptosis of RTC, tubular expression of caspase-3 and the content of MDA were increased in the UUO group compared with those in the sham-operated group, and were ameliorated significantly by PFD treatment (<0.05). The activity of SOD was decreased in the UUO group, but was improved by PFD treatment (<0.05). Our results showed that PFD could ameliorate TIF in the UUO group, and the possible mechanism was by reducing the apoptosis of RTC, which involved oxidative stress and caspase-3.

关键词： pirfenidone apoptosis caspase 3 oxidative stress