Objective To explore the features of clinical manifestations and electrophysiology of spinal muscular atrophy(SMA) in children.Methods The clinical features and laboratory data were analyzed in 32 children with SMA,electromyography tests were carried out in 28 patients and the muscle biopsy were performed in 24 cases.Results The 32 cases were subdivided into 3 clinical groups,15 cases were SMAⅠ,12 cases were SMAⅡ,5 cases were SMAⅢ.They were all characterized by progressive muscle weakness associated with hyptonia and atrophy.The clinical distinction between SMAⅠto SMAⅢ reflected different age of onset and disease severity.All cases of SMAⅠhad symptoms of respiratory disability,only 1 case of SMAⅡ had paradoxical breathing and none of SMAⅢ had similarly symptoms.Electromyographic studies showed a pattern of denervation with no sensory involvement.The rate of spontaneous potential was 87%,with gentle strain,the duration of motor unit was extended(30%-150%) and the amplitude of it was increased(90%-450%),the motor nerve conduction velocity was reduced slightly in 28% patients.The muscle biopsy provided evidence of skeletal muscle denervation with groups of atrophy.Conclusions The clinical features,the changes of electromyography and the muscle biopsy are valuable for diagnosis of SMA.Respiratory management will prolong survival and improve the quality of life for these patients.

0.05);but the levels of OPN in stageⅢ(84.0% and 88.0%)were significantly higher than those of stageⅡ(P

OBJECTIVETo investigate the factors influencing the pregnancy outcomes of artificial insemination with donor sperm (AID), improve the pregnancy rate, and evaluate the safety of the offspring.

METHODSWe retrospectively analyzed 7,761 cycles of AID for 5,109 infertile couples performed between July 1, 2005 and June 30, 2013 in the Center of Reproductive Medicine of Shenyang No 204 Hospital, the outcomes of pregnancy, and the incidence of birth defects.

RESULTSTotally, 2 252 clinical pregnancies were achieved by AID, in which the pregnancy rate per cycle was 29. 02% and the cumulative pregnancy rate was 44. 08%. The clinical pregnancy rate was remarkably higher in the females of ≤ 35 years than in those of > 35 years old (30.31% vs 20.18%, P < 0.01), in the women with < 5-year infertility than in those with > 5-year infertility (30.83% vs 28.16%, P < 0.01), and in the patients of the ovarian stimulation group than in those of the natural cycle group (33.22% vs 28.68%, P < 0.01) The clinical pregnancy rate was the highest in the first treatment cycle (29.87%), with statistically significant difference from the fourth cycle (23.61%) (P < 0.05), but not between the other cycles (P > 0.05). There were 28 cases of birth defects in the offspring (1.40%), including 6 cases (21.43%) involving the cardiovascular system, 4 (14.29%) involving the musculoskeletal system, 3 (10.71%) involving the urogenital system, 3 (10.71%) involving the central nervous system, 2 cases (7.14%) of cleft lip and palate, 2 (7.14%) involving the respiratory system, 2 (7.14%) involving the gastrointestinal digestive system, and other anomalies.

CONCLUSIONFemale age, infertility duration, and ovarian stimulation treatment are important factors influencing the clinical pregnancy rate of AID. Artificial insemination with cryopreserved donor sperm does not increase the incidence of birth defects, which is considered as a relatively safe technique of assisted reproduction.

Adult ; Cryopreservation ; Female ; Humans ; Infertility ; Insemination, Artificial, Heterologous ; methods ; Male ; Maternal Age ; Ovulation Induction ; Pregnancy ; Pregnancy Outcome ; Pregnancy Rate ; Retrospective Studies ; Semen Preservation ; methods ; Spermatozoa ; Time Factors

Animals ; Antibody-Producing Cells ; drug effects ; Female ; Immunity ; drug effects ; Male ; Mice ; Mice, Inbred BALB C ; Phenols ; toxicity ; T-Lymphocytes ; drug effects

Chromatography, Gas ; methods ; Chromatography, High Pressure Liquid ; methods ; Humans ; Hypnotics and Sedatives ; blood ; Pentobarbital ; blood ; Pharmaceutical Preparations ; blood ; Sensitivity and Specificity

Objective:To establish a detection method for ethanol in HuaiShi province Ye. Methods:Ethanol in HuaiShi Ye was determined by GC with a DB-ALC1 capillary column(30 m × 0. 53 mm,3. 0 μm), an FID detector and nitrogen as the carrier gas. The injector temperature was 200℃, the detector temperature was 250℃ and the column temperature was 80℃. The flow rate of N2 was 5 ml·min-1 . Tertbutyl alcohol was used as the internal standard, and 1. 0μl sample was injected by a headspace injector. Results:The solvents were completely separated, and the calibration curve of ethanol had good linear relationship within the range of 3. 177 0-15. 885 0 mg(r=0. 999 6), and the recovery was 98. 7%. The intra-day precision of different concentrations was less than 2. 5%. Conclusion:The method is sensitive, rapid, accurate and reliable, and can be used for the determination of ethanol in HuaiShi Ye.

Objective To review the experience of perioperative management and effect of valve replacement combined with renal dysfunction.Methods 536 cases of valve replacement.According to preoperative glomerular filtration rate(GFR),renal dysfunction was classified as normal in 322 cases,mild in 162,moderate in 40 and severe in 12.Logistic regression analysis was used to assess the effect of preoperative renal dysfunction on operative mortality and morbidity.Results Patients with a lower GFR were older and more likely to have hypertention.They also tended to have larger left ventricular end-diastolic diameter and worse left ventricular ejection factor.Operative mortality rose inversely with declining renal function,from 2％ for those with normal renal function to 17％ for patients with severe renal dysfunction.Renal dysfunction was significantly associated with a large blood transfusions,re-intubation,longer mechanical ventilation,intensive care unit stay and hospital stay.Conclusion Preoperative renal dysfunction is an important factor of postoperative mortality and morbidity for valve replacement.We must pay more attention to perioperative management.

Objective To compare the clinical effects of the traditional operation and the new patch technique for the elderly women with pelvic organ prolapse.Methods 60 elderly women with pelvic organ prolapse patients were randomly divided into the traditional group and the modern group with 30 cases.The traditional group was treated with traditional surgery.The madern group was treated by new patch repair.Compare of the operation time,operative blood loss,catheter duration,hospital stay,postoperative recovery and 3 years recurrence rate in two groups.Results The catheter duration and hospital stay of the mederm group were significantly shorter than those of the traditional group (t =8.9471,14.2584,P ＜ 0.05).The postoperative recovery of the new group was better than the traditional group (x2 =5.9341,P ＜ 0.05).The 3 years recurrence rate of the mederm group was significantly lower than those of the traditional group (x2 =4.7059,P ＜ 0.05).The operation time of the mederm group was longer than those of the traditional group (t =4.4631,P ＜ 0.05) and the operative blood loss was more than the traditional group (t =2.0318,P ＜ 0.05).Conclusion The effect of the new patchin the treatment of elderly women with pelvic organ prolapse is better than the traditional surgery.

Cardiovascular System ; GATA4 Transcription Factor ; metabolism ; Humans

Objective To investigate the effect and mechanism of hydrogen saline on oxidative stress damage in rats brain tissues after cardiopulmonary resuscitation (CPR). Methods Eighteen adult male pathogen-free Sprague-Dawley (SD) rats were randomly divided into control group (Con group), conventional resuscitation group (ROSC group) and hydrogen saline treatment group (ROSC+HRS group), with 6 rats in each group. All rats were asphyxiated by tracheal clip method to establish cardiac arrest (CA) model, and received first aid with CPR, electric defibrillation and adrenaline until return of spontaneous circulation (ROSC). The rats in ROSC+HRS group were intraperitoneally injected with 2% hydrogen saline (5 mL/kg for the first time and 3 mL/kg every 2 hours). The rats in Con group were only tracheal intubated and mechanical ventilated. The rats were sacrificed after ROSC for 12 hours, and the brain tissue was harvested to determine the contents of malonaldehyde (MDA), superoxide dismutase (SOD), and catalase (CAT). The protein expression of heme oxygenase-1 (HO-1) was determined with Western Blot, and the mRNA expression of HO-1 was determined with reverse transcription-polymerase chain reaction (RT-PCR). Results Compared with the Con group, the MDA was significantly elevated in ROSC group (nmol/mg: 8.15±0.11 vs. 3.68±0.16, P < 0.05), the SOD and CAT were significantly decreased [SOD (U/mg): 69.30±2.39 vs. 94.65±2.75, CAT (U/mg): 74.38±1.65 vs. 95.68±1.88, both P < 0.05], HO-1 mRNA expression was significantly elevated (gray value: 1.383±0.194 vs. 1.117±0.083, P < 0.05), and HO-1 protein expression showed no significant change (gray value: 0.350±0.049 vs. 0.175±0.026, P > 0.05). Compared with the ROSC group, the MDA was significantly decreased in ROSC+HRS group (nmol/mg: 4.72±0.28 vs. 8.15±0.11, P < 0.05), the SOD and CAT were significantly elevated [SOD (U/mg): 83.02±1.10 vs. 69.30±2.39, CAT (U/mg): 85.07±1.94 vs. 74.38±1.65, both P < 0.05], HO-1 mRNA expression was significantly elevated (gray value: 3.200±0.200 vs. 1.383±0.194, P < 0.05), and the HO-1 protein expression was significantly elevated (gray value: 0.788±0.059 vs. 0.350±0.049, P < 0.05). Conclusions Oxidative stress damage is an important mechanism of CPR brain damage. Hydrogen saline can increase the expression of HO-1 in brain tissue, and decrease oxidative stress damage of brain after CPR.