Objective The purpose of this study was to determine if recombinant ?-defensin-2 can prevent the lung from being injured by pseudomonas aeruginosa infection. Methods Ten male SD rats ( class SPF) weighing 250-300 g were randomly divided into 2 groups ( n = 5 each) : I defensin group and II control group. The animals were anesthetized with intraperitoneal thiopental 10 mg? kg-1 and intubated. 50 ? l of 5 ? 107 PFU? ml-1 adenovirus with or without ?-defensin-2 gene was instilled into the trachea via tracheal tube. 48 h later 200 ? l of 6? 108 CFU?ml-1 pseudomonas aeruginosa ATCC27853 was instilled into the trachea in both groups. All the rats were killed after 24 h and the lungs were removed for (1) histologic examination, (2) determination of ICAM-1 expression in the lung and (3) broncho-alveolar lavage. The number of WBC and pseudomonas aeruginosa in broncho-alveolar lavage fluid (BALF) was counted. Results The number of WBC and pseudomonas aeruginosa in BALF was significantly less in defensin group than in control group. The degree of instologic damage and the expression of ICAM-1 in lung tissue were significantly decreased in the defensin group as compared with control group. Conclusion Recombinant ?-defensin-2 can kill pseudomonas aeruginosa in vivo and modulate the expression of ICAM-1. It can protect the lung against injury induced by pseudomonas aeruginosa infection.

Objective To establish an accurate method for estimating the indoor gamma dose rate from decorative stones.Methods Combining a room model with decorating conditions,the gamma dose rates and dose rate conversion factors (DCF) at 1 m above the floor in the room center were calculated with the Monte Carlo simulations,and the calculation results were verified through experiments.Based on the results,the limit of radionuclide contents in stone materials was further discussed.Results The DCF increases with the increase of area or thickness of decorative stones in the same room.The increase of DCF with the thickness of decorative stone is approximately linear.The DCF also increases with the area of decorative stones,but the increasing trend slows down.For the same decorative stones,the smaller the room,the larger the increase of gamma dose rate.Experimental results were consistent with the simulation results within ± 20％.Conclusions The increase of indoor gamma dose rate depends not only on the radionuclide contents,but also on the area and thickness of the decorative stones as well as the room size.The method used in this study can be used to estimate,more accurately than ever,the additional external exposure to residents due to decorative stones,and it provides a theoretical basis for revising the limit standard on radionuclide contents in decorative materials.

A growing number of children and adolescents are being diagnosed as Chiari malformation type Ⅰ (CM-Ⅰ) for behavioral disorders,developmental delay,seizures,or abnormal orpharyngeal function.The aim of this study was to compare the clinical characteristics,imaging findings and surgical outcomes of CM-Ⅰ in pediatric and adult patients.Between January 2014 and June 2017,84 patients with CM-Ⅰ underwent surgical treatment in our department.We divided the patients into two groups:pediatric group (n=1 1,age ＜18 years)and adult group (n=73,age ≥18 years).Data on clinical characteristics,imaging findings,surgical outcomes,and prognosis were retrospectively reviewed and compared between these two groups.For clinical presentation,scoliosis (36.4％) and developmental delay (36.4％) were more common in pediatric patients,whereas,sensory disturbance (58.9％) and motor weakness (41.1％) were more common in adult patients.Imaging findings showed that the incidence of hydrocephalus and craniovertebral junctional abnormalities was significantly higher in pediatric group than in adult group (P＜0.05).Compared to adult group,pediatric group showed a better improvement or resolution of syrinx and tonsillar herniation after surgical treatments (P＜0.05).The total Chicago Chiari Outcome Scale (CCOS) score in pediatric patients at the last follow-up was significantly higher than that in adult patients (P=0.002).In conclusion,the clinical characteristics and imaging findings appeared to be different in pediatric and adult patients with CM-Ⅰ.The surgical outcomes of pediatric patients were shown to be significantly better than those of adult patients.

OBJECTIVETo assess the long-term clinical effectiveness of Chinese herbal medicines for benefiting qi and activating blood circulation (CHMBQABC) plus routine Western medical intervention in treating unstable angina (UA) patients of qi deficiency blood stasis syndrome (QDBSS) after percutaneous coronary intervention (PCI) based on Markov model.

METHODSA Markov model was established based on prognosis and sequelae of UA patients after PCI treated by CHMBQABC plus routine Western medical intervention or by routine Western medical intervention. According to the transition probabilities of 40 Markov cycles and quality-adjusted life years (QALYs) averagely gained, we assessed the therapeutic advantage of CHMBQABC plus routine Western medical intervention.

RESULTSBy the prediction of Markov model for 20 years, the transition probabilities of revascularization, non-fatal myocardial infarction, non-fatal stroke, and all-cause death in the CHMBQABC plus routine Western medical intervention group was 56.65%, 6.53%, 5.16%, and 31.66%, respectively, and the QALYs averagely gained was 12.95; while the transition probabilities of revascularization, non-fatal myocardial infarction, non-fatal stroke, and all-cause death in the Western medical intervention group was 55.31%, 6.87%, 5.25%, and 32.57%, respectively, and the QALYs averagely gained was 12.84. Compared with the Western medical intervention group, the QALYs averagely gained was 0.11 in the CHMBQABC plus routine Western medical intervention group.

CONCLUSIONBased on predicted results of the Markov model, CHMBQABC plus routine Western medical intervention got better efficacy in treating UA patients after PCI, indicating CHMBQABC plus routine Western medical intervention could improve the long-term clinical effectiveness for UA patients of QDBSS after PCI.

Aged ; Angina, Unstable ; drug therapy ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Male ; Markov Chains ; Middle Aged ; Models, Theoretical ; Phytotherapy ; Treatment Outcome

OBJECTIVETo discuss the mechanism of scar hypertrophy in adenosine receptor A(2A) (A(2A) R) knockout mice.

METHODSAnimal models of hypertrophic scar were established in 12 A(2A) R knockout mice and 12 wild-type mice as control. The thickness and the size of transverse section of the hypertrophic scar were observed by H-E staining. The hydroxyproline (HYP) in the scar was measured colorimetrically. The TGF-beta expression was tested by Western blotting method.

RESULTSThe hypertrophic scar in wild-type mice was more severe than that in knockout mice. Compared with self-control, the increase of the thickness and the size of transverse section of hypertrophic scar was markedly higher in wild-type group than in the knockout group (P < 0.01). There was significant difference in HYP content between the two groups (P < 0.01). Compared with self-control, the increase of TGF-beta expression in wild-type group was much more than that in knockout group (P < 0.01).

CONCLUSIONSThe TGF-beta expression decreases in the A(2A) R knockout mice. The scar hypertrophy is also much less in the A(2A) R knockout mice.

Animals ; Cicatrix ; metabolism ; pathology ; Disease Models, Animal ; Mice ; Mice, Knockout ; Receptor, Adenosine A2A ; genetics ; Transforming Growth Factor beta ; genetics ; metabolism

Atropa belladonna is a medicinal plant and main commercial source of tropane alkaloids (TAs) including scopolamine and hyoscyamine, which are anticholine drugs widely used clinically. Based on the high throughput transcriptome sequencing results, the digital expression patterns of UniGenes representing 9 structural genes (ODC, ADC, AIH, CPA, SPDS, PMT, CYP80F1, H6H, TRII) involved in TAs biosynthesis were constructed, and simultaneously expression analysis of 4 released genes in NCBI (PMT, CYP80F1, H6H, TRII) for verification was performed using qPCR, as well as the TAs contents detection in 8 different tissues. Digital expression patterns results suggested that the 4 genes including ODC, ADC, AIH and CPA involved in the upstream pathway of TAs, and the 2 branch pathway genes including SPDS and TRII were found to be expressed in all the detected tissues with high expression level in secondary root. While the 3 TAs-pathway-specific genes including PMT, CYP80F1, H6H were only expressed in secondary roots and primary roots, mainly in secondary roots. The qPCR detection results of PMT, CYP80F1 and H6H were consistent with the digital expression patterns, but their expression levels in primary root were too low to be detected. The highest content of hyoscyamine was found in tender stems (3.364 mg x g(-1)), followed by tender leaves (1.526 mg x g(-1)), roots (1.598 mg x g(-1)), young fruits (1.271 mg x g(-1)) and fruit sepals (1.413 mg x g(-1)). The highest content of scopolamine was detected in fruit sepals (1.003 mg x g(-1)), then followed by tender stems (0.600 mg x g(-1)) and tender leaves (0.601 mg x g(-1)). Both old stems and old leaves had the lowest content of hyoscyamine and scopolamine. The gene expression profile and TAs accumulation indicated that TAs in Atropa belladonna were mainly biosynthesized in secondary root, and then transported and deposited in tender aerial parts. Screening Atropa belladonna secondary root transcriptome database will facilitate unveiling the unknown enzymatic reactions and the mechanisms of transcriptional control.
Alkaloids ; biosynthesis ; genetics ; metabolism ; Atropa belladonna ; genetics ; metabolism ; Gene Expression Regulation, Plant ; genetics ; Hyoscyamine ; genetics ; metabolism ; Plants, Medicinal ; genetics ; metabolism ; Scopolamine Hydrobromide ; metabolism ; Tropanes ; metabolism

Objective To explore the value of monitoring in vertebral and spinal cord operation by the real-time monitoring of the spinal cord in the process of operation.Methods Nineteen cases were included by the real-time monitoring of spinal cord in the process of operation.Japanese Orthopaedic Association(JOA) score was used to evaluate the clinical efficacy during following-up before and discharge after operation,6 months and 12 months after operation.Results Intraoperative ultrasound monitoring in the 19 patients had no symptom of spinal cord injury and other complications within the plant without loosening fracture occurred were bony fusion; the average JOA scores in the patients before operation,discharge after operation,6 months and 12 months after operation were 8.80±1.60,14.00±1.57,14.60±1.61 and 14.80±1.58,respectively.The improvement rate of JOA score for discharge after operation,6 months and 12 months after operation were individually 51.6%,61.3%,and 64.5%.The JOA score after operation was obviously higher than that before operation.The significant difference between the two groups was observed in the experiment (P<0.05).The cervical spinal canal sagittal diameter significantly increased after operation (mean sagittal diameter of the expansion of 6.6 mm),compared with that before the trial (P<0.05).Conclusion The real-time monitoring with ultrasonography during operation can identify the change of spinal cord and provide the image evidence of operative efficiency to avoid the injury in vertebral and spinal cord operation.

Objective:To evaluate the relationship between Karyopherin β2 (Kapβ2)-mediated nuclear translocation of nuclear inhomogeneous ribonucleoprotein A2/B1 (hnRNPA2/B1) and sevoflurane-induced brain neurotoxicity in a cellular experiment.Methods:The mouse hippocampal neuronal cell line HT22 cells were inoculated in confocal culture dishes and 6-well culture plates at a density of 2×10 5 cells/well and 1×10 6 cells/well and divided into 4 groups( n=12 each) by a random number table method: control group (GFP-C group) carrying green fluorescent protein (GFP) with empty adenovirus transfection, sevoflurane group (GFP-Sev group) carrying GFP with empty adenovirus transfection, control group (GFP-Sev group) transfected with Kapβ2 gene-overexpressing adenovirus, and sevoflurane group (Kapβ2-Sev group) transfected with Kapβ2 gene-overexpressing adenovirus. After 48 h of conventional incubation, empty adenovirus-carrying GFP (GFP-C and GFP-Sev groups) and Kapβ2 gene-overexpressing adenovirus (Kapβ2-C and Kapβ2-Sev groups) were transfected. After 48 h of transfection, the cells were conventionally incubated continuously in GFP-C and Kapβ2-C groups, and the cells were incubated for 3 h with 3% sevoflurane and then were conventionally incubated for 48 h in GFP-Sev and Kapβ2-Sev groups. The expression of Kapβ2, synaptophysin (SYP), postsynaptic density protein 95 (PSD95) and hnRNPA2/B1 nucleoplasmic ratio were measured in cells by Western blot. Immunofluorescence assay was used for hnRNPA2/B1 subcellular localization. Results:Compared with GFP-C group, the expression of SYP and PSD95 was significantly down-regulated, hnRNPA2/B1 nucleoplasmic ratio was decreased, and cytoplasmic hnRNPA2/B1 expression was up-regulated in GFP-Sev group, and Kapβ2 expression was significantly up-regulated in Kapβ2-C group ( P<0.05). Compared with Kapβ2-C group, the expression of SYP and PSD95 was significantly down-regulated, hnRNPA2/B1 nucleoplasmic ratio was decreased, and cytoplasmic hnRNPA2/B1 expression was up-regulated in Kapβ2-Sev group ( P<0.05). Compared with GFP-Sev group, the expression of Kapβ2, SYP and PSD95 was significantly up-regulated, hnRNPA2/B1 nucleoplasmic ratio was increased, and cytoplasmic hnRNPA2/B1 expression was down-regulated in Kapβ2-Sev group ( P<0.05). Conclusions:Kapβ2-mediated hnRNPA2/B1 nuclear translocation may be the endogenous protective mechanism against sevoflurane-induced brain neurotoxicity.

OBJECTIVETo investigate the clinical features in children with tuberous sclerosis complex (TSC)-associated cardiac rhabdomyomas (CRM).

METHODSThe clinical data of 15 children with TSC complicated by CRM were collected. The clinical features of the patients were analyzed, and TSC gene mutations were detected.

RESULTSEleven cases (73%) developed multiple CRM. The majority of the tumors were located in the left and right ventricles. Most tumors presented as a round-like hyperechogenic mass with a clear margin on echocardiography. Arrhythmias occurred in 3 patients and 2 patients experienced heart failure. Gene mutation tests were performed in 2 patients, and pathogenic mutations were detected in both patients, which were TSC1 mutation and TSC2 mutation, respectively. Three patients were followed up for 6 to 38 months, and their CRM shrank or regressed spontaneously.

CONCLUSIONSTSC-associated CRM is generally multiple. Heart failure and arrhythmias may occur in some patients. Echocardiography is important for diagnosis of CRM. TSC-associated CRM has an inclination to spontaneous regression. TSC can be diagnosed at a molecular genetic level by TSC gene mutation detection.

Child, Preschool ; Female ; Heart Neoplasms ; complications ; genetics ; Hemodynamics ; Humans ; Infant ; Infant, Newborn ; Male ; Mutation ; Rhabdomyoma ; complications ; genetics ; Tuberous Sclerosis ; etiology ; Tumor Suppressor Proteins ; genetics

Objective:To explore the clinical characteristics of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.Methods:Eleven children with autoimmune GFAP astrocytopathy with positive GFAP antibody in serum and/or cerebrospinal fluid were collected in our hospital from January 2020 to February 2022. The clinical data of these children were analyzed retrospectively.Results:Among the 11 children, there were 6 males and 5 females, and the age of onset ranged from 3 years old and 10 months to 12 years old. The main clinical manifestations included fever ( n=8), headache ( n=5), vomiting ( n=6), ataxia ( n=2), limb weakness ( n=4), cranial nerve involvement ( n=6), disturbance of consciousness ( n=4), abnormal mental behavior ( n=3), seizures ( n=1), and autonomic nervous dysfunction ( n=3). Meningoencephalomyelitis was noted in one child, meningoencephalitis in one, encephalomyelitis in 7, and encephalitis was noted in two children. MRI showed brain involvement in all children, spinal cord involvement in 8 children, and optic nerve involvement in one child. Abnormal enhancement in different parts of cerebral lobe, meninges, sulcus, optic nerve and spinal cord were found in 3 children. Four children were positive for GFAP antibody in cerebrospinal fluid and serum, 3 patients were positive for GFAP antibody in cerebrospinal fluid, and 4 children were positive for GFAP antibody in serum. Four children were complicated with multiple antibodies, mainly myelin oligodendrocyte glycoprotein antibody. Tumor screening was all negative. All of the 11 children responded to immunotherapy, but two of them relapsed; one left visual and motor function impairment. Conclusions:The clinical manifestations of autoimmune GFAP astrocytopathy in children are diverse and non-specific, and the lesions mainly involve meninx, brain, spinal cord and optic nerve. Most children respond well to glucocorticoid treatment and have a good prognosis; but there is still a certain recurrence rate, and some children may leave neurological damage.