Genetic Diseases, Inborn ; economics ; prevention & control ; therapy ; Humans ; Metabolic Diseases ; economics ; prevention & control ; therapy

Objective To explore the effect of the thyroid morphologic change,the initiating treatment time and the thyroid function(serum FT_4,TSH)on mental development in children with congenital hypothyroidism (CH).Methods After diagnosed,52 cases of CH were orally administrated with L-T_4,then their mental and physical development were monitored and thyroid morphologic changes were observed by thyroid imaging(~(99m)TcO_4) and type B ultrasonography.The Bayley test was performed at the age of 10 to 30 months and expressed as mental developmental index(MDI)and psychomotor developmental index(PDI).Analysis was conducted upon treatment timing and thyroid morphologic changes.Results The MDI in those cases with morphologic abnormalities treated within 90 days was significantly lower than that with normal morphology(91?20 vs 105?15,P

Adrenal Hyperplasia, Congenital ; complications ; metabolism ; Body Height ; Growth Disorders ; etiology ; Humans ; Risk Factors ; Steroid 21-Hydroxylase ; metabolism

Otolaryngology ; Periodicals as Topic

Objective To reveal and forecast the incidence trend of Brucellosis, in order to provide acientific basis for future intervention and policy-making. Methods Descriptive epidemiological method was used to analyze and statistically describe the distribution of the disease in different times, different locations and different (7.0783/10 million to 13.1257/10 million) and Qingxu ( 1.4811/10 million to 8.5241/10 million) were higher,followed by Yangqu county(0 to 5.8232/10 million), Xiaodian(0.8108/l0 million to 2.4229/10 million) and Jinyuan district ( 0.5329/ 10 million to 1.5896/10 million), and the remaining counties(districts) in the annual There were 223 cases of Brucellosis patients from 2006 to 2009 in Taiyuan. Vocational high risk population was farmers, with a total of 140 cases, accounting for 62.78% of the total number of incidence, followed by students and workers, respectively, 13, 14 cases, accounting for 5.83% and 6.28%, other occupational groups, 56 cases,77.58%;28 cases aged above 60 years, accounting for 12.56%;22 cases aged younger than 19 years, accounting identical in the four years, most cases occurred in spring and summer and showing a clear seasonal high.Conclusions The incidence trend of Brucellosis is on the rise from 2006 to 2009. High risk population is farmer,and the number of younger patients is on the rise, we propose strengthen protection for high risk groups.

OBJECTIVETo investigate the influence of down-regulating Smoothened (SMO) gene expression through short hairpin RNA (shRNA) on the proliferation of breast cancer stem cells.

METHODSHuman SMO shRNA was designed, synthesized chemically, and transfected into MCF-7 cells to down-regulate SMO gene. By using G418, stable cells with down-regulated SMO were selected. In vitro proliferation of these cells was measured by CCK8 assay. The proportion of CD44(+)/CD24(-) cells was detected by flow cytometry and the mammospheres formation was determined by suspension sphere culture. The expression of SMO, GLI1 and Oct4 was detected by Western blot. In vivo, the volume of tumor was measured every 3 days and the expression of SMO, GLI1 and Oct4 detected by Western blot.

RESULTSIn vitro, the cells were transfected with SMO-shRNA and selected by G418 after 21 days. SMO-shRNA effectively down-regulated the expression of SMO gene and protein, and inhibited the proliferation of MCF-7 and markedly reduced the proportion of CD44(+)/CD24(-) cells and mammospheres. In vivo, SMO-shRNA treatment of MCF-7 significantly inhibited the volume of tumor. The positive rate of SMO in negative control and SMO-shRNA group was 5/5 and 2/5, respectively. The expression of SMO, GLI1 and Oct4 in different groups were 0.72 ± 0.17 and 0.21 ± 0.09, 1.21 ± 0.21 and 0.47 ± 0.12, 0.83 ± 0.13 and 0.25 ± 0.07. SMO, GLI1 and Oct4 down-regulation significantly suppressed at protein levels (P < 0.05).

CONCLUSIONThe shRNA by chemical synthesis can effectively down-regulate SMO gene expression and inhibit the proliferation of breast cancer stem cells.

Animals ; Cell Proliferation ; Down-Regulation ; Female ; Gene Expression Regulation, Neoplastic ; Humans ; Hyaluronan Receptors ; metabolism ; MCF-7 Cells ; Mice ; Mice, Nude ; Neoplasm Transplantation ; Neoplastic Stem Cells ; pathology ; Octamer Transcription Factor-3 ; metabolism ; RNA, Small Interfering ; genetics ; Receptors, G-Protein-Coupled ; genetics ; metabolism ; Smoothened Receptor ; Transcription Factors ; metabolism ; Transfection ; Tumor Burden ; Zinc Finger Protein GLI1

Objective To investigate the immunosuppressive effect ofcurcumin on xenogenic skin transplantation in mice.Methods The skin transplantation model was established by an operation,tergal skin flaps from BALB/c donor mice transplanted to the back of C57BL/6 recipient mice.Then recipient mice were divided into five groups at random,namely sham,model,curcumin (50 mg/kg),Cyclosporin A (10 mg/kg) and curcumin + Cyclosporin A (50 mg/kg + 5 mg/kg).All mice were ip administered once daily for 10 d.The survival days of skin graft were recorded in all groups.The interleukin-2 (IL-2) levels in plasma of all mice were determined by ELISA 4 and 8 d after the operation,respectively.Results The mean survival time of skin graft in curcumin,Cyclosporin A and curcumin + Cyclosporin A groups were 14.77,16.81 and 19.96 d,respectively,which showed significant.differences comparing with 12.10 d of model group (P ＜ 0.01).Combination of curcumin and Cyclosporin A administration showed a longer mean survival days than curcumin or Cyclosporin A group (P ＜ 0.05 or 0.01).The IL-2 levels in plasma of mice in curcumin,Cyclosporin A and curcumin + Cyclosporin A groups on postoperative day 4 were 3.68,2.05 and 2.70 ng/mL,respectively,which were significantly reduced than 4.76 ng/mL of model group (P ＜ 0.05 or 0.01).The IL-2 levels in plasma of mice in curcumin,Cyclosporin A and combination of curcumin and Cyclosporin A groups on postoperative day 8 were 4.06,2.11 and 2.95 ng/mL,respectively,which were significantly reduced than 5.85 ng/mL of model group (P ＜ 0.01).Conclusion Curcumin may have a good immunosuppressive effect on mice with xenogenic skin transplantation.

Objective To observe toxic symptoms and signs , toxic damage extents and reversibility in rats after oral administration of Tangwang Mingmu granules .Methods Four dose groups with 40 rats in each group were designed in this study, including control group fed with distilled water and three groups at different dosages of the test drug .Tangwang Mingmu granules were orally administered to SD rats at the dosage of 8.4, 4.2 and 2.1 g/kg for 3 weeks and 14.0, 8.4 and 4.2 g/kg for 23 weeks, for 26 consecutive weeks .The general state of the rats was observed every day , while body mass and food consumption were calculated once a week .Halfway through and at the end of the administration (13 and 26 weeks) and after four weeks of recovery, parameters of body mass, hematology, hematological biochemistry, organ/body mass ratio and histopathology were measured .Results Compared with the control group at the same time-point, body mass of male rats in the other three groups was slightly reduced .Food consumption in high and medium dose groups was reduced (P<0.05), MCHC, ALT, TBIL and Na +in high dose group were decreased (P<0.05), TP, ALB and D-BIL were increased (P<0.05), the mean body mass and relative organ weight of thymus in medium dose male rats were decreased (P<0.05), relative organ weight of the liver and kidney in high dose male rats was increased (P<0.05), and focal chronic inflammation to different extent was observed in the liver , kidney and prostate gland .No dose-effect relationship was found in these perturbations that were all within the normal range of animals .No significant drug-related pathological changes were found.Conclusion The NOAEL of Tangwang Mingmu granules is considered to be 14.0 g/kg body mass/day (equal to 50 times the proposed clinical adult dosage ) for the 26-week repeated dose oral toxicity study in male andfemale rats.

OBJECTIVECarnitine deficiency has been associated with progressive cardiomyopathy due to compromised energy metabolism. The objective of this study was to investigate clinical features of carnitine deficiency-induced cardiomyopathy and the therapeutic efficacy of L-carnitine administration.

METHODBetween January 2010 and December 2011, filter-paper blood spots were collected from 75 children with cardiomyopathy. Free carnitine and acylcarnitine profiles were measured for each individual by tandem mass spectrometry (MS/MS). For those in whom carnitine deficiency was demonstrated, treatment was begun with L-carnitine at a dose of 150 - 250 mg/(kg·d). Clinical evaluation, including physical examination, electrocardiography, chest x-ray, echocardiography and tandem mass spectrometry, was performed before therapy and during follow-up.

RESULTOf 75 cardiomyopathy patients, the diagnosis of carnitine deficiency was confirmed in 6 patients, which included 1 boy and 5 girls. Their age ranged from 0.75 to 6 years. Free carnitine content was (1.55 ± 0.61) µmol/L (reference range 10 - 60 µmol/L). Left ventricular end-diastolic diameter (LVDd) was (5.04 ± 0.66) cm and left ventricular ejection fraction (LVEF) was (38.5 ± 10.5)%. After 10 - 30 d therapy of L-carnitine, free carnitine content rose to (30.59 ± 15.02) µmol/L (t = 4.79, P < 0.01). LVDd decreased to (4.42 ± 0.67) cm (t = 4.28, P < 0.01) and LVEF increased to (49.1 ± 7.6)% (t = 6.59, P < 0.01). All patients received follow-up evaluations beyond 6 months of treatment. Clinical improvement was dramatic. LVEF returned to normal completely in all the 6 patients. LVDd decreased further in all the 6 patients and returned to normal levels in 3 patients. No clinical signs or symptoms were present in any of the 6 patients. The only complications of therapy had been intermittent diarrhea in 1 patient.

CONCLUSIONTandem mass spectrometry is helpful to diagnose carnitine deficiency and should be performed in all children with cardiomyopathy. L-carnitine has a good therapeutic effect on carnitine deficiency-induced cardiomyopathy.

Adolescent ; Cardiomyopathies ; diagnosis ; drug therapy ; etiology ; Cardiotonic Agents ; administration & dosage ; therapeutic use ; Carnitine ; blood ; deficiency ; therapeutic use ; Child ; Child, Preschool ; Electrocardiography ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Retrospective Studies ; Tandem Mass Spectrometry ; Treatment Outcome ; Ventricular Function, Left ; drug effects

OBJECTIVETo determine the effects of nerve growth factor (NGF) on proliferation of hepatic stellate cells (HSCs) and investigate the related molecular mechanism.

METHODSAfter incubating cultured HSCs for 24 h with different concentrations of NGF (100, 200 or 400 ng/mL), the cell proliferation was observed by XTT colorimetric assay and cell cycle was detected by flow cytometry. Morphological changes in response to a 24 h exposure to 100 ng/mL NGF were observed by transmission electron microscopy.

RESULTSNGF significantly inhibited HSC proliferation (P less than 0.05) in a dose-independent manner. The optical densities of the XTT colorimetric assay were 0.66+/-0.03 for 100 ng/mL NGF, 0.69+/-0.03 for 200 ng/mL NGF, and 0.66+/-0.03 for 400 ng/mL NGF, all of which were significantly lower than that of the control group (0.73+/-0.01; P less than 0.05). All concentrations of NGF led to significantly higher numbers of HSCs in the G2 phase (100 ng/mL: 14.83+/-5.41%, 200 ng/mL: 14.73+/-2.50%, and 400 ng/mL: 14.87+/-2.06%), compared to that detected in the control group (7.47+/-4.39%; P less than 0.05). Twenty-four hours of exposure to 100 ng/mL NGF caused morphological changes indicative of apoptosis.

CONCLUSIONNGF inhibits the proliferation of HSCs, possibly by arresting the cells in the G2 phase of the cell cycle. NGF-inhibited cells may also undergo apoptosis.

Animals ; Apoptosis ; Cell Cycle ; Cell Proliferation ; drug effects ; Cells, Cultured ; Flow Cytometry ; Hepatic Stellate Cells ; cytology ; drug effects ; Nerve Growth Factor ; pharmacology ; Rats