Objective To investigate the clinical characteristics of Kennedy disease with secondary mitochondrial dysfunction and glutaric aciduria typeⅡ.Methods The clinical data of 1 case Kennedy disease with secondary mitochondrial dysfunction and glutaric aciduria typeⅡwas retrospectively analyzed.Results The patient presented muscle weakness in proximal limbs, fasciculation, bulbar palsy, amyotrophy, postural tremor, sensory disturbance, gynaecomastia, impotency.The level of serum creatine kinase and urinary glutaric acid were elevated. Electromyogram and nerve and muscle biopsy supported mixed damage in peripheral nerves, as well as neurogenic and secondary myogenic pathologic changes in muscles.Gene sequencing indicated trinucleotide CAG repeated amplification for 47 times in chromosome X.Diabetes was diagnosed at 7 years after onset.Conclusions The pathogenesis of Kennedy disease is very complex, which may involve myogenic factor.The treatment to the secondary mitochondrial and lipid metabolic disturbance in muscle can improve the muscle weakness to a certain extent.

Objective To investigate the effects of different doses of penehyclidine hydrochloride on postoperative cognitive function in the elderly patients.Methods Ninety-three ASA Ⅰ or Ⅱ elderly patients,aged ≥65 yr,weighing 55-71 kg,were randomly divided into 3 groups (n =31 each):penehyclidine hydrochloride 0.010 mg/kg group (group A),penehyclidine hydrochloride 0.015 mg/kg group (group B) and atropine 0.010 mg/kg group (group C).Their preoperative Mini-Mental State examination (MMSE) scores were ＞ 27.At 30 min before anesthesia,groups A,B and C received intramuscular penehyclidine hydrochloride 0.010 mg/kg,penehyclidine hydrochloride 0.015 mg/kg and atropine 0.010 mg/kg,respectively.The cognitive function of the patients was assessed within 72 h after operation using MMSE.Diagnostic criterion of postoperative cognitive dysfunction (POCD) was defined as MMSE score ≤27.POCD and the degree were recorded within 72 h after operation.Results Compared with group A,postoperative cognitive function was significantly decreased at each time point after operation and the incidence of POCD was significantly increased in group B (P ＜ 0.05),and no significant change was found in the parameters mentioned above in group C (P ＞ 0.05).Compared with group B,postoperative cognitive function was significantly enhanced at each time point after operation and the incidence of POCD was significantly decreased in group C (P ＜ 0.05).Conclusion Penehyclidine hydrochloride can depress postoperative cognitive function and the effect is related to the dose.

Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytosis, Sinus ; pathology ; surgery ; Humans ; Immunoglobulin G ; blood ; Paranasal Sinus Diseases ; pathology ; surgery ; Paranasal Sinuses ; pathology ; S100 Proteins ; metabolism ; Sclerosis ; pathology

Adolescent ; Adult ; Amnion ; transplantation ; Blood Transfusion, Autologous ; Eye Burns ; therapy ; Humans ; Male ; Middle Aged ; Occupational Injuries ; therapy ; Young Adult

Objective: To investigate the effect of fucoidan on multiple myeloma U266 cells and its possible molecular mechanism. Methods: The multiple myeloma U266 cells were treated with 10, 25 and 50 μg/mL fucoidan for 24, 48 and 72 h, respectively. The growth inhibitory rate of U266 cells was determined by cell counting kit-8 (CCK-8) assay. The apoptosis rate was measured by flow cytometry. The expression levels of RAS, p38, phospho-p38, sonic Hedgehog (Shh) and glioma-associated oncogene homolog 1 (GLI1) were detected by Western blotting. Results: The growth of multiple myeloma U266 cells treated with different concentrations of fucoidan (10, 25 and 50 μg/mL) was inhibited only in a dose-dependent manner. The apoptosis rates of U266 cells treated with 10, 25 and 50 μg/mL fucoidan for 24 h were (6.73±2.30)%, (9.12±1.90)% and (20.13±2.10)%, respectively, which were higher than that of the U266 cells without fucoidan intervention [control: (4.08±1.60)%; P < 0.05). The expression levels of RAS, phospho-p38, Shh and GLI1 proteins in U266 cells treated with fucoidan were significantly lower than that of the control (P < 0.05). Conclusion: Fucoidan inhibits proliferation and induces apoptosis of U266 cells by inhibiting Ras-p38MAPK and Shh-GLI1 signaling pathways. Copyright © 2013 by TUMOR.

Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Male ; Middle Aged ; Purpura, Thrombotic Thrombocytopenic ; etiology ; Transplantation, Autologous

OBJECTIVEThis study aimed to investigate the expression function of substance P and the formation of osteoclasts in the periodontal tissues after the inferior alveolar nerve sectioned in rats.

METHODSThirty Wistar male rats were used in the experiment and were divided into six groups (n = 5) randomly: 0 d (normal), 3 d, 7 d, 14 d, 21 d, and 28 d. The periodontal tissues were removed from the denervation of the inferior alveolar nerve in rats. The periodontal tissues were checked by paraffin sections through immunohistochemical staining to trace the expression of substance P and through tratrate resistant acid phosphatase (TRAP) staining to detect the osteoclasts. The average optical density and osteoclast were measured, and the obtained data was statistically analyzed.

RESULTSThe expression level of substance P in the first three days decreased significantly after the inferior alveolar nerve was cut. In addition, the lowest expression level was measured after 7 d. Normal levels in the periodontal tissue were measured after 21 d. In addition, we found that osteoclasts vary proportionally with the changes in substance P.

CONCLUSIONThe changes in substance P is positively correlated with the quantity of osteoclasts after the inferior alveolar nerve section. Therefore, we deduce that substance P may regulate the differentiation of osteoclasts formation, and thereby participate in the balancing of aveular bone metabolism.

Objective To investigate the levels of transformation growth factor-β1 (TGF-β1),vascular endothelial growth factor (VEGF) and insulin growth factor-Ⅰ (IGF-Ⅰ) role in the pathogenesis of serum of patients with combined pulmonary fibrosis and emphysema (CPFE).Methods Recruited 20 patients with CPFE,40 cases with idiopathic pulmonary interstitial fibrosis (IPF) and 40 cases with emphysema who were admitted to our hospital during July 2011 to February 2012.Serum levels of TGF-β1,VEGF and IGF-Ⅰ were detected by ABC-ELISA.Results Serum levels of TGF-β1 and IGF-Ⅰ were significantly higher in patients with CPFE and IPF than these in patients with emphysema (TGF-β1:(160.73 ± 40.62) ng/L vs.(167.35 ± 42.82) ng/L vs.(128.75 ±35.77) ng/L; IGF-Ⅰ:(179.65 ±60.73) ng/L vs.(192.32 ±58.75) ng/L vs.(148.73 ±49.67) ng/L,P ＜ 0.05 or P ＜ 0.01).The IPF group had significantly higher serum level of VEGF than the emphysema group ((506.12 ±82.37) ng/L vs.(437.31 ±62.58) ng/L,P ＜0.01).Serum levels of TGF-β1 and IGF-Ⅰ in CPFE and emphysema groups were positively correlated (r =0.885,0.918 respectively,P ＜ 0.01).Smokers in the CPFE group had significandy lower level of serum VEGF than those who did no smoke ((406.19 ± 66.94) ng/L vs.(482.88 ± 79.91) ng/L,t =-2.287,P =0.035).Conclusion Serum level of VEGF increased significantly in the IPF group,suggesting the participation of VEGF in pulmonary interstitial fibrosis.IGF-Ⅰ involved in the pathogenesis of pulmonary interstitial fibrosis.TGF-β1 and IGF-Ⅰ have a positive linear regressive relationship,which indicates that they may work synergistically in the process of the fibrosis.

Objective To observe the difference in serum level of oxidative stress between first diagnosed type 2 diabetes mellitus (T2DM) patients and healthy person with normal glucose,compare the difference in serum level of oxidative stress between T2DM patients with continuous subcutaneous insulin injection (CSII) and multiple subcutaneous insulin injection (MSII),and further explore the possible mechanism of CSII superior to MSII.Methods Sixty patients with first diagnosed T2DM were divided into CSII group and MSII group by random digits table,with 30 patients in each group and given 14 days treatment of CSII and MSII respectively.Thirty healthy person were selected with age and sex matched in same period as control group,then fasting plasma glucose (FPG),2-hour postprandial blood glucose (2 h PG),glycosylated serum protein (GA),fasting insulin (FINS),superoxide dismutase (SOD),reduced glutathione (rGSH) and malondialdehyde (MDA) before and after treatment was measured and analyzed.Results The FPG,2 h PG,GA,HOMA model of insulin resistance (HOMA-IR) and MDA before treatment in CSII group and MSII group was higher than that in control group [FPG:(13.81 ± 3.14),(13.58 ±2.10) mmol/L vs.(5.21±0.42) mmol/L; 2 h PG:(21.72 ±4.43),(22.07 ±3.86) mmol/L vs.(6.19 ± 0.47) mmol/L;GA:(28.74 ± 4.40),(28.83 ± 5.01) mmol/L vs.(13.24± 1.26) mmol/L;HOMA-IR:0.45 ±0.13,0.43 ±0.12 vs.0.20 ±0.15;MDA:(4.37 ± 1.13),(4.44 ± 1.07) mmol/L vs.(2.37 ±0.58) mmol/L] (P ＜ 0.01),and the HOMA model 3 cell function index (HOMA-β),SOD and rGSH was lower than that in control group [HOMA-β:0.98 ±0.17,0.96 ±0.12 vs.1.91 ±0.19;SOD:(29.63 ±9.29),(28.07 ±9.62) mU/L vs.(41.00 ± 12.89) mU/L;rGSH:(157.03 ±46.58),(165.23 ±45.66) ng/L vs.(212.00 ± 61.67) ng/L] (P ＜ 0.01).There was no significant difference between CSII group and MSII group (P ＞ 0.05).The FPG,HOMA-β,SOD and MDA after treatment in CSII group was no significant difference compared with that in control group (P ＞ 0.05).But the FPG and MDA after treatment in MSII group was higher than that in control group(P＜ 0.01 or ＜ 0.05),and the HOMA-β and SOD was lower than that in control group (P ＜ 0.01).The FPG,2 h PG,GA and MDA after treatment in CSII group was lower than that in MSII group (P ＜ 0.01 or ＜ 0.05).The HOMA-β was higher than that in MSII group (P ＜ 0.01).Multi-factor analysis results showed that the HOMA-β and FPG (r =-0.919,P ＜ 0.01),2 h PG (r =-0.904,P ＜ 0.01),GA (r =-0.830,P ＜ 0.01) and MDA (r =-0.653,P ＜ 0.01) was negatively correlated,and SOD (r =0.454,P ＜ 0.01) and rGSH (r =0.394,P ＜ 0.01) was positively correlated.Conclusions Oxidative stress level of first diagnosed T2DM is significantly higher than that of healthy people.Two intensive insulin treatment for first diagnosed T2DM can reduce blood glucose level,oxidative stress levels and improve insulin resistance and β cell function.CSII can effectively reduce blood glucose,reduce the level of lipid peroxidation and improve β-cell function compared with MSII.

Objective To evaluate the value of dual Doppler ultrasonography in the diagnosis of fetal atrial extrasystoles.Methods Seven thousands and thirteen fetuses were examined by fetal echocardiography.If fetal arrhythmias were found,information of pulsed wave Doppler signals in hepatic vein (HV) and descending aorta (DAo) using dual Doppler were colleted simultaneously.The detected times were compared between using general Doppler and dual Doppler in 20 cases.Results On dual Doppler examination,28 of the fetuses showed signals of atrial extrasystoles,14 fetuses atrial extrasystoles conducted to ventricle,8 fetuses atrial extrasystoles non-conducted to ventricle,6 fetuses with part of the extrasystoles conducted to ventricle and part of the extrasystoles non-conducted to ventricle.No case had congenital heart disease.Twenty-six fetuses with atrial extrasystole were converted to normal rhythm within the third trimester of pregnancy,and the others' were converted to normal rhythm during the first 3 days postnatally.The detected time of dual Doppler was shorter than that of general Doppler(P ＜0.05).Conclusions Dual Doppler ultrasonography of simultaneous recordings in HV and DAo is an easy,rapid method of assessing fetal atrial extrasystoles,which should allow precise diagnosis of atrial extrasystole.