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

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

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.

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.

AIM: To test the effects of FTY720 on mouse intestinal allografts.METHODS: C_3H mice(H-2~k)were used as donor and C57BL/6 mice(H-2~b) as recipients.FTY720 group,allogeneic control group and isogeneic control group were set up.6 and 14 days after transplantation,murine intestinal grafts were harvested for histologic assessment.Lymphocytes were collected from mesenteric lymph nodes(MLN),Peyer's patch(PP),lamina propria lymphocytes(LPL) and intraepithelial lymphocytes(IEL) in the graft,then were analyzed by cytometry.RESULTS: Rejection was inhibited in FTY720 group at the 6th post-transplant day,although not at the 14th day.Recipient CD4~+ and CD8~+ T cells,CD19~+ B cells,as well as ?? TCR lymphocytes,were greatly reduced by FTY720 therapy.The similar action of FTY720 was also revealed in Gr1~+CD11b~+ monocytes.CONCLUSION: FTY720 is efficient on alleviating allo-immune response by reducing the infiltration of both lymphocytes and monocytes into the graft in a mouse intestinal transplantation model.

OBJECTIVE: To study the effects of Compound Rhizoma Coptidis capsule (CRCC) on hemorheology and blood lipid in rats with diabetes mellitus(DM). METHODS: DM model was induced by alloxan. The effects of CRCC on hemorheology and blood lipid in DM rats were observed. RESULTS: The levels of total cholesterol (TC), triglyceride (TG), low density lipoprotein (LDL), very low density lipoprotein (VLDL), lipoprotein, the whole blood viscosity, the whole blood reductive viscosity, aggregation index (AI), rigidity index (IR), distortion deformation transformation index (TK) and fibrinogen (Fib) were all increased by CRCC, while the level of high density lipoprotein (HDL) was increased. CONCLUSION: CRCC can significantly improve the abnormalities in hemorheology and lipid metabolism in DM rats.

Objective To report the result of analysis of 72 cases of facial cutaneous sporotrichosis. Methods Seventy-two patients were included in this study. They were diagnosed as facial cutaneous sporotrichosis according to clinical mainfestations and fungal culture. Histopathological examinations were done in some atypical cases. The patients were treated with combined antifungal agents. Results The lesion forms were varied, in which lymphangitic cutaneous form accounted for 9.72% and fixed cutaneous form accounted for 90.28% respectively. Nineteen cases (26.4%) had a history of skin trauma before the cutaneous lesions. All of the patients were successfully treated with potassium iodide and/or itraconazole, terbinafine for 6-8 weeks. Conclusions The skin lesions and mycological examination are the important evidences for the diagnosis of sporotrichosis. The main form of the facial cutaneous sporotrichosis is the fixed cutaneous form. The misdiagnosis rate is 16% in primary level clinics. Potassium iodide is an effective and inexpensive medicine in the treatment of cutaneous sporotrichosis.

Objective To investigate the changes of interleukin(IL)-2 and interferon(IFN)-γin serum and synovial fluid of patients with joint injure with Kashin-Beck disease(KBD), and study the roles of IL-2 and IFN-γ in KBD joint injure. Methods In accordance with the "Diagnostic Criteria of Kashin-Beck disease"(GB16003-1995),48 cases of KBD patients and 26 healthy people(control group) from KBD endemic area in Long county Shaanxi province were enrolled in the study. KBD patient were 24 males and 24 females, respectively, aged 40 to 65 years (mean age 51 years). Forty-eight serum specimens and 28 synovial fluid specimens of patients(14 males and 14 females,respectively) were collected. Healthy control group were 13 males and 13 females, respectively. Twenty-six serum specimens of healthy controls were collected. Serum and synovial fluid IL-2 and IFN-γ levels were determined by enzyme-linked immunosorbent assay(ELISA). Results In healthy controls and KBD patients, the midian of serum IL-2 were 46.8 ng/L and 55.7 ng/L, respectively, and IFN-γ were 52.3 ng/L and 48.8 ng/L, respectively. The difference was not statistically significant between healthy controls and KBD patients(t = 0.62, 0.70, all P ＞ 0.05).In synovial fluid of KBD patient, the midian of IL-2 and IFN-γwere 48.3 ng/L and 44.1 ng/L, respectively. The difference was not statistically significant between serum and synovial fluid in KBD patients(t = 0.69, 1.72, all P ＞0.05). Conclusion Serum and synovial fluid IL-2 and IFN-γare not significantly increased in KBD patients with articular damage, indicating that IL-2 and IFN-γare not involved in KBD joint injury.