Humans ; Ion Channels ; Myocytes, Smooth Muscle ; metabolism ; physiology ; Pulmonary Artery ; metabolism ; physiology

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.

Cerebral Veins ; Female ; Humans ; Middle Aged ; Prosthesis Implantation ; Stents ; Tinnitus ; etiology ; 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.

Objective To investigate the expression of vascular endothelial cell growth factor(VEGF) and its upregulation of matrix metalloproteinase-2(MMP-2)activation in synovial fluid of patients with rheumatoid arthritis(RA)as well as its role in the pathogeneses of RA.Mathods Expression of VEGF and receptor KDR in mononuclear cell(MNC)of synovial fluid of RA patients and controls were determined by Western blot;VEGF levels of supernantants from MNC was determined by ELISA;supernantants from KDR~+ MNC of synovial fluid of RA patients collected after incubation in serum-free medium with or without VEGF, their activity of MMP-2 was measured by gelatinolytic zymography;Boyden chamber-matrigel in vitro invasion assay was used to detect the invasive capacity in vitro in KDR~+ MNC of synovial fluid of RA patients incubat- ed with or without VEGF.Results The expressions of VEGF/KDR in MNC of synovial fluid of RA patients were significantly higher than those of controls;the MMP-2 activity and invasive ability of co-cuhured KDR~+ MNC with VEGF was higher than those of without VEGF.Conclusion VEGF upregulates MMP-2 activation and promotes invasion of MNC of synovial fluid of RA patients by interacting with receptor KDR,indicating that VEGF plays an important role in RA pathology.

Objective To explore the best way to diagnose and cure the nephrosis with ureteropelvic junction obstruction(UPJO) in children.Methods The diagnosis of 26 cases of nephrosis with UPJO were confirmed by ultrasonogram and IVU examinations.All patients underwent AndersonHynes procedures.These results were analyzed and summarized.Results All the diagnosis were proved to be correct by operation and pathology examinations,the operations were carried out successfully.Twenty-four cases were followed up for 6 months to 2 years.After the operation,the hematuria,urinary frequency,abdominal mass and distention disappeared,the urine analysis was normal,all cases cured clinically.Ultrasonogram examinations showed the thickness of parenchyma increased and the large kidneys lessened.Significantly improved renal fuoction on IVU examinations was observed in the 24 cases compared with that before the operations.The ureters of 20 cases displayed well.Conclusions The combination of ultrasonogram and IVU is very effective method to diagnose UPJO in children.Anderson-Hynes technique is the best procedure to cure the UPJO.

Aim The kinetics of nicotinic acetylcholine receptor (N-AChR) was stadied. MethodsThe saturation experiments of Torpedo electric organ N-AChR were taken with125I-?-BuTX. The results were calculated by Scatfit program. Experiments were thenperformed to study the kinetics of dissociation of ?-BuTX and nicotine from N-AChR,respectively. When their binding reached saturation, an excess of 1000 times ?-BuTX ornicotine was added. The saturation binding properties of the N-AChR extracted fromleg muscles of the 13th day chick embroys and optic lobes of the 20th day chick em-broys were studied. The results were calculated by Scatfit program. The othe experi-ments were taken to observe the competition between mcotine and 125I-?-BuTX forbinding with Torpedo, optic lobe and skeletal muscle of the chick N-AChRs. ResultsThe saturation experiments of Torpedo electric organ N-AChR resulted in a Scatchardplot of hyperbola which responded to the model of two kinds of receptor with on eli-gand.The difference of and B max between high affinity binding site and low affnitybineing site was significant. The dissaciation experiment showed that the fast dissocia-tion rate of tow ligands was 500 times more than that of the slow dissociation rate. Thisresult suggested that there may be two subtype N-AChRs.The saturation bindingproperties of N-AChR of leg muscles and optic lobe of the chick embroys revealed aScatchard plot of two kinds of N-AChR indicating a single type of site. The bindingaffinity of receptor of optic lobe was 100 times more than that of muscles. In competi-tion for Torpedo receptor by nicotine and 125I-?-BuTX, the values of IC50 were different:which suggested that two kinds of receptor sites were existent. In competition ofNicotine and 125I-?-BuTX for optic lobe and skeletal muscles of the chick N-AChR, thevalue IC50 of skeletal muscles N-AChR was 7. 7 times higher than that of optic lobe. Itindicated that two kinds of N-AChR subtype existed in the optic lobe and in theskeletal muscles of chick respectively. Conclusion N-AChR of Torpedo electric organcontains two kinds of subtype receptors. N-AChR of optic lobe of chick embroys is onesubtype receptor, and N-AChR of skeletal muscles of chick embroy is another subtypereceptor.

OBJECTIVETo investigate the effects of locking plate fixation through lateral deltoid approach for proximal humeral fracture combined with micro-invasive percutaneous plating (MIPPO) technique.

METHODSFrom April 2009 to March 2012,26 patients with proximal humeral fractures were treated with proximal humeral locking system plate fixation through lateral deltoid approach, including 17 males and 9 females with an average age of 58 years old ranging from 28 to 76 years old. The time from injury to operation was 3 to 10 days (averaged 5.6 days). According to Neer typing for the proximal humeral fractures, 7 cases had 2 parts of fracture,15 had 3 parts of fracture,and 4 had 4 parts of fracture. The Neer score for shoulder function was evaluated.

RESULTSAll patients were followed up,and the duration ranged from 10 to 21 months (averaged 13.6 months). All patients were achieved bony union,the average healing time was 12.5 weeks (ranged from 10 to 21 weeks). No humeral head necrosis and axillary nerve injury occurred. According to Neer scoring system,the total score was 88.36 +/- 7.82, pain 30.82 +/- 3.24, function 23.76 +/- 5.71, activity 17.59 +/- 5.36, anatomical position 7.03 +/- 2.39; the result was excellent in 18 cases, good in 5 cases, fair in 2 cases, poor in 1 case.

CONCLUSIONLateral deltoid approach combined with locking plate fixation for treatment of proximal humeral fracture has advantages of small invasion,less blood lossing, short operative time, stable fixation, high rate of fracture healing, and satisfactory functional recovery.

Adult ; Aged ; Bone Plates ; Female ; Fracture Fixation, Internal ; instrumentation ; methods ; Humans ; Humeral Fractures ; surgery ; Male ; Middle Aged ; Shoulder Fractures ; surgery ; Treatment Outcome

Background Our previous study demonstrated that kallikrein-kinin is a special protein in vitreous of the eye with proliferative vitreoretinopathy(PVR),and the expression intensity of kallikrein-kinin showed the positive correlation with the grade of PVR.Objective This study was to further explore whether kallikrein-kinin participate in the formation of PVR.Methods Rat retinal pigment epithelial cell line(RPE-J cells) was cultured in DMEM containing 4% fetal bovine serum and then prepared into suspension by PBS with the cells density of 2.5×108 cells/ml.Platelet-rich plasma was prepared by PBS with the platelet 2.5×108 /ml.RPE cell suspension(4μl) and platelet-rich plasma(6μl) was intravitreally injected in the left eyes of 30 clean Wistar rats to establish the PVR models,and 10μl sterile pyrogen-free normal saline solution was used in the same way in other matched rats as controls.The PVR was graded on Francine's criteria in 1 day,3,7,14,21,28 days after injection under the slit lamp.The serum,vitreous and retina were obtained in 28 days after injection to assess the expression of bradykinin using Western blot.The histopathology examination of rat retina was performed in the 28th day after injection.This experimental procedure followed the Regulations for the Administration of Affair Concerning Experimental Animals by State Science and Technology Commission.Results Typical PVR was seen in 25 models with the successful rate 89.3% at 28 days after injection.PVR 1,2,3 grades were respectively exhibited in 7,14,28 days under the slit lamp.Infiltration of inflammatory cells and migration of RPE cells were found in the 7th day.In the 14th day after injection,RPE cells transformed into fibroblasts and retinal detachment occurred after that.Western blot analysis revealed that bradykinin was detected in vitreous,serum and retinal samples of rats in experimental and control rats,but the expression intensity was higher in the rats of model groups.Conclusion Intravitreal co-injection of RPE cells and platelet-rich plasma can effectively induce a model of PVR in Wistar rat.The kallikrein-kinin system probably takes part in the onset of PVR.

Adenocarcinoma, Clear Cell ; pathology ; Ethmoid Sinus ; Humans ; Kidney Neoplasms ; pathology ; Male ; Middle Aged ; Neoplasm Metastasis ; Paranasal Sinus Neoplasms ; secondary