Clinical data of 2 children with cystic fibrosis (CF) and Pseudomonas aeruginosa infection in the Department of Pediatrics of Sichuan Provincial People′s Hospital from April 2018 to June 2019 were retrospectively analyzed.Patient 1 was an 11-year-old girl with no history of recurrent respiratory infections. Pseudomonas aeruginosa was found in the first sputum culture.A large number of yellow and white secretions were visible under repeated fiberoptic bronchoscopy.Chest CT showed multiple spots and tree-in-bud signs around the bronchi of both lungs. CFTR gene test results revealed 3 heterozygous mutations: c.2909G>A (chr7: 117246728), c.*133T>A (chr7: 117307295) and c. *125delT (chr7: 117307285). The other patient was a 7-year-old boy with a history of recurrent respiratory infections.His parents were close relatives.Multiple cultures of sputum and bronchoalveolar lavage fluid of the boy were Pseudomonas aeruginosa, and chest CT suggested dilation and inflammation in bronchi of both lungs.Gene detection showed that the c. 380T>G homozygous mutation at chromosome chr7-117171059 resulted in an amino acid change p. leu127stop (nonsense mutation). This article suggests that CF should be considered for Pseudomonas aeruginosa infected children having signs of bronchiectasis on chest CT and a large number of secretions under bronchoscopy.Besides, it is necessary for such kind of children to perform genetic testing in time to confirm the diagnosis as soon as possible.

Objective To evaluate the effects of penehyclidine hydrochloride pretreatment on the expression of nitric oxide synthase (NOS) and cell apoptosis in lung tissues in rats with endotoxin-induced acute lung injury (ALI).Methods Forty adult male Sprague-Dawley rats,weighing 220-270 g,were randomly divided into 5 groups (n =8 each) using a random number table:control group (group C),endotoxin-induced ALI group (ALI group),and penehyclidine hydrochloride 0.03,0.10,0.30 mg/kg groups (Pi-3 groups).ALI was induced with lipopolysaccharide (LPS) 5 mg/kg which was injected via the caudal vein.In P1-3 groups,penehyclidine hydmchloride 0.03,0.10 and 0.30 mg/kg were injected intraperitoneally,respectively,at 1 h before LPS injection,while the equal volume of normal saline was administered in C and ALI groups.The animals were sacrificed at 4 h after ALI models were successfully established and pulmonary specimens were obtained for determination of the cell apoptosis (by TUNEL),expression of NOS mRNA (using PT-PCR),and Bcl-2 and Bax protein (by Western blot),NOS activity (using chemical colorimetry) and NO content (by using nitrate reductase method) and for examination of pathological changes (by light and electron microscopes).Apoptotic index (AI) and the ratio of Bcl-2/Bax was calculated.Results Compared with group C,NOS mRNA and Bax protein expression was significantly up-regulated,NOS activity,NO content and AI were increased,Bcl-2 protein expression was down-regulated,the ratio of Bcl-2/Bax was decreased (P ＜ 0.01),and the degree of pathological changes of the lung was aggravated in ALI and P1-3 groups.Compared with ALI and P1 groups,NOS mRNA and Bax protein expression was significantly down-regulated,NOS activity,content of NO and AI were decreased,Bcl-2 protein expression was up-regulated,the ratio of Bcl-2/Bax was increased (P ＜ 0.01),and the degree of pathological changes of the lung was alleviated in P2-3 groups.There was no significant difference in the indexes mentioned above and results of pathological changes of the lung between group ALI and group P1,and between group P2 and group P3 (P ＞ 0.05).Conclusion Penehyclidine hydrochloride pretreatment ameliorates endotoxin-induced ALI by inhibiting NOS expression and cell apoptosis in rat lung tissues.

Objective To investigate the effectiveness of glucocorticoids in the treatment of granulomatous lobular mastitis (GLM), and to discuss the optimal stage to add glucocorticoids during the treatment. Methods Twenty-four patients having received the core needle biopsy were involved. Ten cases with the explicit pathological diagnosis received the glucocorticoids therapy following the subtotal excision after remission. Pathological diagnoses of the rest 14 patients were undefined. For these 14 patients, simple partial excisions were given and the postoperative pathological diagnoses were presented as the GLM. Of all the 14 patients who accepted the surgical treatment firstly, 8 patients received the postoperative glucocorticoids adjuvant therapy. For the rest 6 patients, steroid hormone therapy was not used after surgery, and they were followed up postoperatively. All patients' clinical and pathological information were collected and analyzed. Results All patients were followed up for 6-36 months (average 18) by the outpatient service. Of all the 10 patients who received the glucocorticoids therapy before surgery, only 1 patient of them got the GLM recurrence. For the 8 patients who received the postoperative glucocorticoids treatment, only 1 patient got the recurrence. For the 6 patients who received simple partial excision, the recurrence of the GLM may be up to 3. There was no statistical difference between the two groups who both received the 05). But compared with the pure surgery treatment, the difference was obviously (P<0.05). Conclusions The clinical presentation and imaging performance of GLM are unspecific, so the diagnosis of the GLM is difficult. There is no consensus regarding the optimal treatment for GLM. The glucocorticoids therapy may be necessary preoperatively or postoperatively. For the patient with clear preoperative biopsy diagnosis, preoperative glucocorticoids adjuvant chemotherapy followed by the wide excision may be an effective method.

Objective To study the effects of high matrix metalloproteinase 9 (MMP 9 ) on the biological behaviors of fibroblasts,in order to clarify the possible mechanisms in the wound healing of diabetic foot.Methods Establish the cell model of skin fibroblast with high expression of MMP 9 by high glucose (22.0 mmol/L) and high homocysteine (100 μmol/L) co-culture.Control group was incubated with normal glucose (5.5 mmol/L).Realtime PCR,ELISA and gelatin zymography were used to detect the MMP 9 mRNA,protein expression and activity of MMP 9.Flow cytometry,CCK-8,ELISA assay,scratch test and transwell were used to detect cell proliferation,viability,collagen (hydroxyproline) secretion,horizontal migration and vertical migration of cells.Results Data were expressed as (x- ± s).Differences were considered significant if their P value was ＜ 0.05.Results The expression of MMP 9 mRNA,protein levels and the activity of MMP 9 in high MMP 9 group were 6.05 folds,4.12 folds and 1.58 folds higher than those in control group (P ＜ 0.01,respectively).The proportion of S phase cells,proliferation index,cell viability,collagen (hydroxyproline) secretion,6 h horizontal migration rate and the number of vertical migration cells in high MMP9 group decreased by 29.8 ％,18.1％,23.3 ％,68.7 ％,45.0 ％ and 21.4 ％ than those in control group (P ＜ 0.01,respectively ). Conclusions Fibroblast with high expression of MMP 9 have decreased proliferation,activity,collagen secretion and reduced migration,which suggests that MMP 9 may inhibit the biological behaviors of fibroblasts.

Background and purpose: Uveal melanoma (UM) is the most common primary intraocular malignancy in adult. Due to a high tendency for early metastasis the treatment of UM is very difficult. This study aimed to explore an effective approach for the treatment of patients with UM, we designed a strategy that combined HtrA2 gene therapy and radiation therapy. Methods:pIRES-Egr1-Omi/HtrA2 (pEgr1-HtrA2) recombinant plasmids were constructed and transfected into human UM cells (OCM-1) in vitro. The transfected cells were exposed to irradiation. HtrA2 mRNA and protein levels were detected by qRT-PCR and Western blot respectively. Assays that evaluated the apoptosis inducibility caused by HtrA2 gene therapy combined with radiation was performed by lfow cytometry. Followingly, the effects of HtrA2 overexpression on the in vitro radiosensitivity of uveal melanoma cells were investigated by clonogenic formation assay. The in vivo effects of HtrA2 gene therapy combined with radiation therapy were evaluated in different groups. Results:The recombinant plasmids could be successfully transferred into OCM-1 cells and transfection of pEgr1-HtrA2 plasmids combined with radiotherapy caused dramatically elevation of HtrA2 compared with non-irradiation cells in mRNA and protein levels, which was associated with increased apoptosis.Furthermore, we observed that the transfection of pEgr1-HtrA2 could significantly enhance radiosensitivity of OCM-1 cell in vitro. In mice bearing xenograft tumors, pEgr1-HtrA2 combined with radiation therapy signiifcantly inhibited tumor growth compared with the other treatment groups (P<0.05). Conclusion:Our ifndings indicate that radiation-inducible gene therapy may have potential to be a more effective and speciifc therapy for uveal melanoma because the therapeutic gene can be spatially or temporally controlled by exogenous radiation.

Objective To investigate the dyslipidemia in patients with psoriasis,and the characteristics of the serum lipids in pa-tients with different types of psoriasis.Methods 332 psoriatic patients were devided into pustular psoriatic,psoriasis vulgaris and erythrodermic psoriasis based on clinical manifestations.Serum lipids of the three groups were analyzed including TC,TG,HDL-C, LDL-C,ApoA1,ApoB and Lp(a).Results The serum concentrations of TC,HDL-C,ApoA1 in patients with psoriasis were signifi-cantly lower than those in control group(P <0.05),while the concentration of Lp(a)was significantly higher in psoriasis patients compared with those in control group(P <0.05).The serum level of TC,HDL-C,LDL-C and ApoA1 in pustular psoriasis group were significantly lower than those in the other two groups(P <0.05).Conclusion The patients with psoriasis have dyslipidemia, and the serious disorder situation could be associated with psoriasis types.

Spinal muscular atrophy (SMA) is a group of neuromuscular disorders, caused by degeneration of the motor neurons in the anterior horn of the spinal cord, with prevalence of about 1 in 6000 to 1 in 10000 in newborn. The gene carrying frequency is about 1 in 40 to 1 in 50 all over the world. SMA is one of the most common autosomal recessive diseases causing infant death. SMA mainly refers to SMN1 dependent caused by SMN1 gene mutations. Noninvasiveness and specificity make genetic testing a recommended method for diagnosis of SMA. In addition to conventional methods such as neural nutrition, muscle exercise, etc., there is no specific treatment for SMA up to now. Nevertheless, HDAC inhibitors deserve attention as they are the only drugs completed Phase Ⅲ clinical trials to date. Furthermore, other ways as small-molecule SMN enhancers, induced pluripotent stem cell (iPSC), antisense oligonucleotides to correct SMN2 splicing, etc, were still on the way of in vitro stage at present.

Objective: To investigate the prevalence and influencing factors of adolescent depression symptoms in Zhejiang, so as to provide reference for improving their mental health. Methods: The middle school and university students in 11 cities of Zhejiang Province were selected by stratified cluster random sampling method. The depression symptoms of the adolescents were assessed by Center for Epidemiological Survey-Depression Scale ( CES-D ) and the influencing factors were analyzed by multivariate logistic regression model. Results: A total of 25 855 students were investigated, and 25 614 ( 99.07% ) valid questionnaires were collected. The detection rate of depressive symptoms was 26.86%(6 879 cases). The detection rate of depressive symptoms in girls was 29.75%, which was higher than 24.12% in boys ( P<0.05). The detection rate of depressive symptoms in high school students was 31.74%, the highest compared with other grades. The multivariate regression analysis showed that female students ( OR=1.690, 95%CI: 1.592-1.794 ), resident students ( OR=1.071, 95%CI: 1.010-1.137 ) , internet addiction ( OR=2.948, 95%CI: 2.527-3.439 ) , attempt smoking ( OR=1.516, 95%CI: 1.359-1.690 ), drinking ( OR=1.624, 95%CI: 1.525-1.729 ), bullied in the past 30 days ( OR=3.143, 95%CI: 2.938-3.363 and having serious injuries within a year ( OR=1.369, 95%CI: 1.263-1.543 ) were associated with adolescents who had depressive symptoms. Conclusions The detection rate of depressive symptoms is relative 26.86% among adolescents of Zhejiang Province. The students who are female, live on campus, have internet addiction, have been bullied or seriously injured, smoke and drink are more likely to have depressive symptoms.

Objective To explore the anti-infection mechanism of carboxyamidotriazole (CAI) through studying the effects of CAI on the proliferation, apoptosis and degranulation of RBL-2H3 mass cells.Methods Compound 48/80 (C48/80) was used to induce the model of activation and degranulation in RBL-2H3 cells.The morphological change of cell degranulation was observed by neutral red staining.The release levels of histamine and β-hexosaminidase were measured by ELISA method and chromogenic assay, respectively.The cell activity was determined by CCK-8 method.And cell apoptosis was detected by Hoechst 33342 fluorescent staining.Results Compared with the control group, 10, 20, 40 μmol/L CAI inhibited C48/80-induced degranulation of RBL-2H3 cells in different degrees.CAI (20, 40 μmol/L) reduced the histamine release (P<0.01), and CAI (40 μmol/L) decreased the β-hexosaminidase release (P<0.01).In addition, the viability and apoptosis of RBL-2H3 cells were not affected at the concentrations of CAI used.Conclusions CAI can effectively inhibit the activation and degranulation of RBL-2H3 mast cells, and this effect is not through cytotoxicity.The anti-infection effect of CAI may partially due to the down-regulation of mast cell activity.

Objective To discuss the ultrasonographic feature and prognosis of the fetal dacryocystocele and its correlation.Methods The ultrasonographic feature of 51 patients diagnosed as dacryocystocele prenatally by sonography were retrospectively analyzed.All fetus were followed up to induced labor or 6 months after birth.The correlation between the size and the gestational age were analyzed by correlation analysis method and compared the fetal prognosis adopt U test.Results The incidence of dacryocystocele diagnosed prenatally by ultrasound was about 0.47‰.The diagnosis was established by the detection of a cystic lesion in relation to the medial and inferior aspects of the fetal orbit.Unilateral dacryocystoceles were more often found than bilateral,and left more than right.The maximum diameter range from 2.0 mm to 12.0 mm,average (5.9 ± 2.2)mm.There is no correlation between cyst size and gestational age.Prognosis of isolated nasolacrimal duct cyst is good.Conclusions The fetal dacryocystocele have typical ultrasonographic feature,Ultrasound is a rapid and reliable method which can be used for diagnosis of dacryocystocele prenatally and follow-up after birth.