Bardet-Biedl Syndrome ; diagnosis ; therapy ; Child ; Female ; Humans

To obtain a number of extracellular penicillinase,the gene(penp)encoding penicillinse of Bacillus cereus ATCC10987 was cloned into expression vector in Bacillus subtilis,transformed into Bacillus subtilis DB104 deficient in two proteases.When recombinant bacteria was cultured in LB medium for 24 hours,the result of SDS-PAGE showed that the molecular weight of the protein was 28kDa and the penicillinase activity reached 339U/ml.By screening seven different fermentation media,the result showed that 4# medium is favorable to producing penicillinase by the recombinant cells than the others,with the yield of the enzyme being 1580U/ml.When the recombinant cells was cultured in 7 liter fermentor for 24h,the penicillinase activity reached 1255.8U/ml.

Inflammatory bowel disease (IBD) is a chronic, repeated intestinal inflammatory disease. Clinically commonly used therapeutic drugs have some disadvantages, such as poor efficacy and many adverse reactions after long-term application. Although new biological therapies such as anti-tumor necrosis factor agents, overcome common adverse reactions, also have problems such as high price, difficult storage, drug resistance and recurrence after application. In recent years, many new therapeutic methods for inflammatory bowel disease have emerged, for example, modulators that inhibit lymphocyte migration (integrin inhibitors and sphingosine 1-phosphate receptor agonists) have been introduced into the clinical treatment of inflammatory bowel disease, inflammatory cytokine inhibitors (interleukin-23 inhibitors, Janus kinase inhibitors, phosphodiesterase inhibitors, etc.) and inhibitors targeting fibrosis and intestinal tissue degradation and remodeling (matrix metalloproteinase inhibitors) are also being evaluated in clinical trials of IBD. Based on the mechanisms of action, this paper intends to outline the current mainstream IBD therapies and some emerging drugs, and briefly introduce their targets to provide reference for IBD drug design and development.

Abstract: Objective　To analyze the serotype distribution, drug resistance rate and drug resistance gene carrying of Streptococcus pneumoniae isolates in hospitalized patients, and evaluate the coverage of the vaccine to the serotype of Streptococcus pneumoniae in this area, so as to provide reference for the rational use of antibiotics in clinic. Methods　A total of 150 strains of non-repetitive Streptococcus pneumoniae isolated from inpatients from January 2015 to December 2019 were collected for serotyping and antimicrobial sensitivity test. The carrying rates of pbp2b, ermB and tetM were detected by PCR. Results　The PCR classification rate of 150 strains of Streptococcus pneumoniae was 93.1%, and the classification rate of capsular swelling test was 100%, and a total of 19 serotypes were divided, mainly 19F and 6B. Children's serotypes were predominantly 19F, 6B, and 15A; adult serotypes were predominantly 19F, 14, and 23F. The coverage rates of the PCV7, PCV10, PCV13 and PPV23 vaccines were 36.8%, 42.1%, 57.9% and 68.4%, respectively. Strains with serotypes of 19F, 6B, 3, and 23F had higher rates of resistance to antimicrobials. The sensitivity of Streptococcus pneumoniae to penicillin was greater than 96.0%. Antimicrobials with significant differences in resistance rates between invasive and non-invasive strains were penicillin, moxifloxacin, and levofloxacin. The percentage of strains carrying both ermB and tetM resistance genes was 96.0%, and the concordance rate between pbp2b, ermB and tetM resistance genes and the resistance phenotype was >98.0%. A total of 10 multi-resistance combinations were detected, with a multi-resistance rate of 62.6%, and the multi-drug resistance pattern of Streptococcus pneumoniae was mainly concentrated in the 19F and 6B serotypes. Conclusion　There are significant age differences in the serotypes of Streptococcus pneumoniae in this area. The vaccine currently used has low coverage in this region and therefore offer limited protection to the population. The drug resistance rates of Streptococcus pneumoniae varied significantly among serotypes. Erythromycin and tetracycline are not recommended for clinical treatment of Streptococcus pneumoniae. Penicillin can still be used as the first choice for clinical treatment of Streptococcus pneumoniae infection.

Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare autosomal recessive disorder. This paper reports three cases of SSADH deficiency in infants. The infants developed the symptoms including developmental delay, intellectual disability, hypotonia, hyporeflexia and seizures. The electroencephalogram (EEG) showed background slowing and focal spike discharges in all of 3 patients. Head magnetic resonance imaging (MRI) demonstrated abnormalities in 2 patients, including basal ganglia damage and increased T2-weighted signal in bilateral cerebral peduncles. Urinary organic acid analysis with gas chromatography-mass spectrometry (GC-MS) revealed increased levels of 4-hydroxybutyrate (GHB) in 3 patients. SSADH deficiency was definitely diagnosed based on the clinical manifestations and the results of urinary organic acid analysis in the 3 children. It was concluded that early urine organic acid analysis is essential for children presenting with mental retardation, neuropsychiatric disturbance or epilepsy of unknown etiology.
Amino Acid Metabolism, Inborn Errors ; diagnosis ; therapy ; Developmental Disabilities ; Diagnosis, Differential ; Female ; Humans ; Infant ; Succinate-Semialdehyde Dehydrogenase ; deficiency

Abnormalities, Multiple ; epidemiology ; Adolescent ; Child ; Child, Preschool ; Heart Defects, Congenital ; complications ; Humans ; Infant ; Infant, Newborn

0.05) and the two methods had good correlation(r=0.822).CONCLUSIONS The method of FCST established by as in this study is simple,repeatable,with high accuracy and easy to determine MIC and has good application prospects in clinical antifungal susceptibility testing.

OBJECTIVETo investigate the effects of sleep deprivation on intelligence development in primary school students.

METHODSBetween June 2009 and April 2010, 316 grade 5 students aged 10-11 years were selected from four primary schools in four administrative districts of Changsha, China by stratified random sampling. The intelligence characteristics of children with varying degrees of sleep deprivation were investigated using the Chinese Wechsler Intelligence Scale for Children.

RESULTSA total of 286 valid questionnaires were received, with a response rate of 90.5%. The survey was comprised of a sleep deprivation group (sleep time <8 hours per night; n=180) and a control group (sleep time ≥8 hours per night; n=106). The sleep deprivation group had significantly lower subtest scores, verbal intelligence quotient (IQ) (VIQ), performance IQ (PIQ) and full scale IQ (P<0.05) and significantly lower verbal comprehension factor score and memory/attention factor score compared with the control group (P<0.05). Compared with the control group, the moderate sleep deprivation subgroup had significantly decreased VIQ and full scale IQ as well as verbal comprehension factor score and memory/attention factor score (P<0.05), and the severe sleep deprivation subgroup showed decreases in all scores (P<0.05). The sleep deprivation group and moderate and severe sleep deprivation subgroups had significantly higher proportions of children with VIQ-PIQ imbalance than the control group.

CONCLUSIONSSleep deprivation adversely affects intelligence development, especially VIQ, in primary school students, and the adverse effects of sleep deprivation are mainly seen in students with moderate and severe sleep deprivation.

This study is to explore the effects of quercetin (QUE) on the 3 week-old mice ovarian development and relative hormone levels. The 3 week-old mice were exposed to QUE (45, 25, and 5 mg x kg(-1) x hd(-1)) by gavage for 50 days. The estrous cycle during 50 days and the changes of hormone level such as FSH, LH, etc were monitored. Moreover, the ovaries were removed after sacrifice. The organ index was measured, and the ratios of different stages of follicles were analyzed by HE staining. Furthermore, the proportion of PCNA positive cells during all stages was detected by immunohistochemistry. The results showed that QUE could increase body weight of mice and reduce the anogenital distance (AGD) to some extent, and was able to disrupt mice's estrous cycle, but it could not extend or reduce the cycle regularity. It increased ovarian organ index with a dose-dependent manner. The proportion of the primordial follicle and secondary follicles rose obviously, and that of mature follicles', atretic follicles' and corpus luteums' reduced, while primordial follicle had no change. Immunohistochemistry analysis showed that QUE could effectively increase the percentage of proliferating cells in all kinds of follicles. Serum hormone assay showed that there were significant changes of FSH and LH levels. In summary, QUE showed an estrogen-like effect on mice's ovarian development. The weight of ovary, the proportion of all kinds of follicles, the development of ovarian cells and the level of plasma hormone in mice were altered obviously by oral administration of QUE.
Animals ; Body Weight ; drug effects ; Dose-Response Relationship, Drug ; Estrous Cycle ; drug effects ; Female ; Follicle Stimulating Hormone ; blood ; Luteinizing Hormone ; blood ; Mice ; Ovarian Follicle ; drug effects ; metabolism ; Ovary ; drug effects ; growth & development ; Phytoestrogens ; administration & dosage ; pharmacology ; Proliferating Cell Nuclear Antigen ; metabolism ; Quercetin ; administration & dosage ; pharmacology ; Random Allocation

The purpose of this study was to investigate the efficacy of non-myeloablative allogeneic stem cell transplantation (allo-NST) and its related technologies in hematological malignancies. 26 patients with hematological malignancies (acute leukemia 10, chronic myeloid leukemia 14, multiple myeloma 2) received allo-NST following conditioning regimens with fludarabine/cyclophosphamide/ATG in 14 cases or busulfan or melphalan/cyclophosphamide/ATG in 12 cases prior to infusion of 2 or 3 collections of G-CSF (600 microg/d) or G-CSF (300 microg/d) plus GM-CSF (300 microg/d) mobilized blood stem cell on the fifth day. A combination of cyclosporine A (CsA) and methotrexate (MTX) was administered for GVHD prophylaxis. Patients were eligible for donor lymphocyte infusion (DLI) (or donor stem cell infusion (DSI)) given in graded increments according to the chimeric formation and clinical feature. Generally, the dose of the first infusion was 1 x 10(7)/kg in 4th week post-transplantation. The engraftment analyses included the detection of microsatellite short tandem repeats (STRs), bcr/abl fusion gene, Philadelphia chromosome, HLA-locus analysis, sex chromosome and ABO blood type or blood subtype. The results showed that out of 26 patients, 22 (84.62%) were engrafted, 18/22 were full donor chimerism (FDC) up to now. Acute GVHD occurred in 3/26 (11.54%), while chronic GVHD was diagnosed in 6 out of 26 (23.07%) patients. The incidence and degree of infection and hemorrhage were low and slight. It is concluded that NST is a safe and effective therapy for hematological malignancies, whereas related technologies such as adaptation selected, conditioning regimen and transplantation immunotherapy should be studied further.
Adult ; China ; epidemiology ; Female ; Graft vs Host Disease ; epidemiology ; Hematologic Neoplasms ; therapy ; Humans ; Male ; Middle Aged ; Peripheral Blood Stem Cell Transplantation ; adverse effects ; methods ; Transplantation Conditioning ; methods