Objective To investigate pathogenesis, clinical and pathological feature of a case of nemaline myopathy and review of relevant researches. Methods A ease of an 8-year-old girl with muscle weakness and her clinical presentation, family history, changes of serum enzymology and EMG, characteristic of light and electron microscopic studies were described. The earlier literature and new genetic findings concerning these muscle abnormalities are also briefly summarized. Results Nemaline myopathy is diagonosed by clinical manifestation and observed result of electron microscopy. Conclusions Muscle biopsy is the only way to diagnose nemaline myopathy and electron microscopy plays an important role in diagnosing it.

Diffuse alveolar hemorrhage (DAH) is a life threatening clinical syndrome caused by a variety of causes.Early identification and etiological diagnosis of DAH in children are challenging.Despite some advances have been made in the identification and management of DAH,the mortality rate is still high.This article aims to raise the cognition of clinicians on DAH by providing a general review of some recent researches.

OBJECTIVE:To provide reference for rational use of antibiotics in the clinic of our hospital. METHODS:Drug re-sistance of Gram-negative bacillus in the inpatients of our hospital were analyzed retrospectively during May 2013-Dec. 2015 as well as the situation of producing metallo-β-lactamase(MBLs). RESTUTS:A total of 2089 strains of Gram-negative bacillus were detected in our hospital during 2013-2015,among which there were 1456 strains of enterobacteria (69.70%) and 633 strains of non-fermentative bacteria,mainly involving Escherichia coli,Pseudomonas aeruginosa,Klebsiella pneumoniae,Acinetobacter bau-mannii and Enterobacter cloacae. A total of 406 strains of carbapenems-resistant bacteria were detected (19.44%),including 367 strains of non-fermentative bacteria and 39 strains of enterobacteria. The resistant rates of carbapenems-resistant strains to 16 antibi-otics were all higher than 50%,but those of non-carbapenems-resistant strains were in relative low level. Except for aztreonam,re-sistant rates of carbapenems-resistant strains to other 15 antbiotics were all higher than those of non-carbapenems-resistant strains, with statistical significance(P<0.05). A total of 36 strains of producing MBLs were detected(8.87%),including 13 strains of pro-ducing MBLs drug-resistant P. aeruginosa and 23 strains of producing MBLs drug-resistant A. baumannii;producing MBLs drug re-sistant enterobacteria had not been found. CONCLUSIONS:Gram-negative bacillus are mainly enterobacteria in our hospital;car-bapenems-resistant strains are mainly non-fermentative bacteria,resistant rate of them are commonly higher than that of non-drug-re-sistant strain. The situation of producing MBLs is serious,and enzyme producing strains are mainly non-fermentative bacteria. It is necessary to strengthen drug resistance of pathogen and enzyme producing strain monitoring,avoid the generation and spreading of drug-resistant strains due to irrational use of antibiotics.

Objective To investigate the effect of pyrrolidine dithiocarbamate(PDTC) on monocyte chemotactic protein1 (MCP-1) in rejection of cardiac allograft and its mechanisms.Methods Heterotopic cervical heart transplantation was performed by cuff-technique.The SD rat recipients were randomly divided into 3 groups:AR group (Acute rejection,n =12),both the recipients and donors were without any treatment.CsA group(n =12),the recipients were treated with 10 mg/kg cyclosporine A after transplantation.PDTC group(n =12),the recipients were treated with 100 mg/kg PDTC after transplantation.All the cardiac allografts were harvested at different time post transplantation according to requirements.We studied allograft myocardial fibrosis wih the help of Masson stain,immuno-histo-chemistry and western blot also were used to detect the expression of MCP-1.Results The survival time of the cardiac allografts was significantly longer in PDTC group than in acute rejection group and CsA group(P ＜ 0.01),and myocardial fibrosis of cardiac allografts in PDTC group was significantly decreased (P ＜ 0.01).The IOD in PDTC group was markedly lower than in CsA group (P ＜ 0.01).Conclusion As the inhibitor of NF-κB,PDTC can significantly relieve rejection of cardiac allograft by inhibiting the expression of MCp-1.

The association of non-HDL-cholesterol and non-HDL-C-to-HDL-cholesterol ratio (non-HDL-C-to-HDL-C ratio) with early diabetic nephropathy in patients with type 2 diabetes mellitus was investigated.Non-HDL-C and non-HDL-C-to-HDL-C ratio were positively related with microalbuminuria (P＜0.05 or P＜0.01).Non-HDL-C-to-HDL-C ratio is an independent risk factor of early diabetic nephropathy in patients with type 2 diabetes mellitus.

High cell density culture of Lactobacillus plantaru is a significant step of the preparation of lactic acid bacteria starter.Firstly,this experiment is designed to analyze the effects of different dissolved oxygen and pH on the batch fermentation of Lactobacillus plantarum.And then,on the base of the batch fermentation,fed-batch fermentation was studied,companied by chemic neutralization in the experiment to achieve the aim of high cell density cultivation.Finally,in the comparison of two kinds of feeding mode for fed-batch operation in 10L fermentor,it was demonstrated that two kinds of feeding mode made cell concentration increase differently,in which pH feedback feeding can control sucrose concentration within a certain lower range,therefore obtaining the maximum cell concentration.With the pH feedback feeding,the dry cell weight was up to 13.56g/L,which increased 90.05% over that of batch culture.

For investigating the epidemiology and genetic characteristics of enterovirus 71 (EV71) in Fujian from 2010 to 2015,we analyzed the surveillance data of EV71 and sequenced VP1 genes of 72 EV71 strains randomly picked from the past 6 years.The overall infection rate was gradually down and one incidence peak (from May to July) was observed each year.Major infectious population were focused on Xiamen,Fuzhou,Nanping and Quanzhou,the ages ranged from one to three years old.Scattered children were the most infected ones.The proportion of EV71 in the severe case was higher than in the HMFD(χ2 =732.064 5,P＜0.000 1).EV71 circulated from 2010 to 2015 in Fujian Province was belonged to subgenotype C4a in consistent with vaccine strain (H07).Compared with the VP1 of vaccine strains,the divergence of complete VP1 nucleotide sequence was gradually expanding as time distance increased,but the sequence of amino acid was not found obvious difference.Variations in 4 key immune epitopes of amino acid had not appeared a regular pattern in year and not consistent with the trend of proportion of EV71 in HMFD.As a result,we considered the epidemiology characteristics of EV71 in Fujian was obvious,72 strains still belonged to C4a subgenotype and had no outstanding antigenic drift or mutation.Extensive epidemiology surveillance and genetic characteristic are needed for the application of EV71 vaccine.

Congenital lung anomalies are rare lung diseases,which caused by the deformity of the anatomical structure of the respiratory system in the development during the embryonic development.At present,there is a lack of standardization of nomenclature and classification for children with congenital lung anomalies.Based on the most upto-date research and treatment,this paper classifies congenital lung anomalies into 5 broad categories:bronchial anomalies,vascular anomalies,pulmonary parenchyma anomalies,combined lung and vascular anomalies,pulmonary lymphatic vessels anomalies.Knowledge of these areas is essential for accurate,timely,diagnosis,which aids in optimizing patient outcomes.

Background and Objectives: To investigate the effect and the underlying mechanism of exosomes secreted by human umbilical cord mesenchymal stem cells (hUCMSCs) on diffuse alveolar hemorrhage (DAH) in murine lupus. Methods: and Results: Exosomes were extracted from cultured hUCMSCs by ultracentrifugation. The expressions of exosome markers (Alix, CD63 and TSG101) were measured for identification of hUCMSC-derived exosomes (hUCMSC-exosomes). The alveolar hemorrhage of DAH mice was revealed by H&E staining. The primary alveolar macrophages were isolated from bronchoalveolar lavage fluid (BALF) of DAH mice. The expressions of M1 macrophage markers (iNOS, IL-6, TNF-α and IL-1β ) and M2 macrophage markers (Arg1, IL-10, TGF-β and chi3l3) were detected. Flow cytometry measured the ratio of M1/M2 macrophages. ELISA measured the secretion of pro-inflammatory cytokines (IL-6 and TNF-α) and anti-inflammatory cytokines (IL-10 and TGF-β ). DAH mice had hemorrhage and small-vessel vasculitis in the lung, with neutrophil and monocyte infiltration observed around the capillary and small artery. Furthermore, increases of IL-6 and TNF-α, and decreases of IL-10 and TGF-β were detected in the BALF of DAH mice. M1 makers were overexpressed in alveolar macrophages of DAH mice while M2 makers were lowly expressed. DAH mice had a higher proportion of M1 macrophages than M2 macrophages. After hUCMSC-exosome or methylprednisolone treatment in DAH mice, the alveolar injuries and inflammatory responses were attenuated, and the proportion of M2 macrophages was increased. Conclusions hUCMSC-exosomes attenuate DAH-induced inflammatory responses and alveolar hemorrhage by regulating macrophage polarization.

Background and Objectives: To investigate the effect and the underlying mechanism of exosomes secreted by human umbilical cord mesenchymal stem cells (hUCMSCs) on diffuse alveolar hemorrhage (DAH) in murine lupus. Methods: and Results: Exosomes were extracted from cultured hUCMSCs by ultracentrifugation. The expressions of exosome markers (Alix, CD63 and TSG101) were measured for identification of hUCMSC-derived exosomes (hUCMSC-exosomes). The alveolar hemorrhage of DAH mice was revealed by H&E staining. The primary alveolar macrophages were isolated from bronchoalveolar lavage fluid (BALF) of DAH mice. The expressions of M1 macrophage markers (iNOS, IL-6, TNF-α and IL-1β ) and M2 macrophage markers (Arg1, IL-10, TGF-β and chi3l3) were detected. Flow cytometry measured the ratio of M1/M2 macrophages. ELISA measured the secretion of pro-inflammatory cytokines (IL-6 and TNF-α) and anti-inflammatory cytokines (IL-10 and TGF-β ). DAH mice had hemorrhage and small-vessel vasculitis in the lung, with neutrophil and monocyte infiltration observed around the capillary and small artery. Furthermore, increases of IL-6 and TNF-α, and decreases of IL-10 and TGF-β were detected in the BALF of DAH mice. M1 makers were overexpressed in alveolar macrophages of DAH mice while M2 makers were lowly expressed. DAH mice had a higher proportion of M1 macrophages than M2 macrophages. After hUCMSC-exosome or methylprednisolone treatment in DAH mice, the alveolar injuries and inflammatory responses were attenuated, and the proportion of M2 macrophages was increased. Conclusions hUCMSC-exosomes attenuate DAH-induced inflammatory responses and alveolar hemorrhage by regulating macrophage polarization.