Objective To analyze the clinical manifestations as well as correlation between clinical manifestations and the new neuroimage characteristics(pontine"cross sign"and"putaminal slit")for correct diagnosis of multiple system atrophy(MSA).Methods Eleven patients with MSA based on Gilman diagnostic criteria were retrospectively analyzed for clinical manifestations and brain MRI findings.Results Eleven patients were probably diagnosed as MSA,among which 8 patients were MSA-C(olivopontoeerebellar atrophy,OPCA),2 patients were MSA-P(striatonigral degeneration,SND),one patient was MSA-A(Shy- Drager syndrome,SDS).Five years after onset,MRI of MSA-A showed stage 0"cross sign"and "putaminal slit".In 8 patients with MSA-C,2 patients reached stage Ⅰ 3 years after onset,one patient reached stage Ⅱ 2 years after onset,3 patients respectively did stage Ⅲ 1 year,3 years,and 5 years after onset,2 patients reached Ⅳ stage 2 years,and 7 years after onset respectively.As for putaminal slit,all of these patients were in stage 0.One patient with MSA-P reached stage 0"cross sign"and stage Ⅰ "putaminal slit"6 years after onset;another patient showed stage Ⅳ"cross sign"and stage Ⅱ"putaminal slit"9 years after onset,which was high slit intensity on both sides,however much stronger in the fight side than in the left side.Conclusion Both"cross sign"and"putaminal slit"are neuroimage characteristics for early diagnosing MSA-C,while"putaminal slit"for MSA-P.

Heart Defects, Congenital ; diagnosis ; therapy ; Humans ; Infant, Newborn

Child, Preschool ; Coronary Vessel Anomalies ; physiopathology ; Coronary Vessels ; physiopathology ; Female ; Humans ; Pulmonary Artery ; abnormalities

Objective To analyze the characteristics,electroencephalographic(EEG)features,neuroimaging findings,therapeutic effects and prognoses of 114 cases of idiopathic nocturnal frontal lobe epilepsy (NFLE). Methods The clinical data of 114 patients with idiopathic NHLE admitted in the epilepsy outpatient department from June,1999 to January,2007 were collected and analyzed. Results Idiopathic NFLE was clinically characterized by nocturnal clustered,postural or dystonic seizures and complex motor activities.The interictal routine EEG in 22.9% and dynamic EEG in 28% of the cases in wakefuIness and the interictal dynamic EEG in 38% of tbe cases in sleep showed frontal lobe epileptiform discharge,which was found in the EEG during seizure attacks in66.7% of the cases.Drug therapy was effeclive in 79.8% of the cases.with 29.7% of the cases showing complete seizure control. Conclusions Idiopathic NFLE has distinct clinical features wim relatively low rate of EEG abnormality during seizure attack or the interictal period.Frontal lobe epilepsy is more likely to occur in sleep,and sleep EEG is therefore of much importance in the diagnosis of idiopathic NFLE.

OBJECTIVETo evaluate the efficacy and safety of transcatheter closure of perimembranous ventricular septal defects (VSD) in children following transthoracic echocardiography (TTE).

METHODSFrom September 2002 to December 2005, eighty-nine children (47 males and 42 females) with perimembranous (VSD) underwent an attempt of transcatheter interventional occlusion. Among the 89 children, one of them was diagnosed with patent ductus arterious (PDA) and six with VSD leakage after the surgical repair (three with leakage after the surgical repair of tetralogy of Fallot and three with leakage after the surgical repair of VSD). The mean age of patients was (6.4 +/- 3.9) years (ranged from 1 to 18 years). The mean body weight of patients was (22 +/- 11 )kg (ranged from 9 to 78 kg). The mean diameter of VSD measured by TTE was (4.3 +/- 1.5) mm(ranged from 2 to 8.5mm). The path of artery to vein was established following X-rays and TTE. Occluder was released through the right heart system. All patients were followed up in 1, 3, 6 and 12 months after procedure of TTE, X-ray and electrocardiography.

RESULTThe devices were deployed successfully in 85 patients, the rate of success was 95.5%. No death occurred during and after the procedure. There was trivial residual shunt in 12 patients immediately after the closure by TTE and angiography. Twenty-four hours later, only 3 patients had trivial residual and no shunt existed after 6 months follow-up. Convulsion occurred in 1 case due to serious cardiac arrhythmias. Hemolysis was found in 2 cases. Other complications included 2 cases of complete left bundle branch block, 1 cases of left anterior fascicular block and 3 cases of incomplete right bundle branch block. They recovered after 3 to 7 days of corticosteroid treatment. After 1 to 36 months (mean 9 months) follow-up, none of occluders displacement occurred and no valve was involved.

CONCLUSIONTranscatheter closure of membranous VSD using occluder would be safe and effective for children, and the results of short-term was satisfied. Transcatheter closure of VSD following TTE is a feasible method. TTE has the potential benefit of avoiding general anesthesia and esophageal intubation in children.

Adolescent ; Cardiac Catheterization ; methods ; Cardiovascular Surgical Procedures ; methods ; Child ; Child, Preschool ; Female ; Heart Septal Defects, Ventricular ; diagnostic imaging ; surgery ; Humans ; Infant ; Male ; Prostheses and Implants ; Prosthesis Implantation ; methods ; Treatment Outcome ; Ultrasonography, Interventional

OBJECTIVEThis study was to investigate an operation, in which removing breast cysts of foreign body resulted from augmentation with polyacrylamide hydrogel injection was performed simultaneously with silicone prosthesis implantation under SEPS endoscope in order to relieve tissue injury and increase the accuracy of clearance.

METHODSEight patients were included in this study. Preoperative type B ultrasound examination was performed to mark the mass. Through an axillary approach, the cysts of foreign body that were distributed in the subcutaneous tissue, breast or muscle were separated and ablated under SEPS endoscope. After removal of the foreign body, a silicone implant was located submuscularly for breast augmentation.

RESULTSThe operations were completed without hematoma and infection. Follow-up of the eight patients for 3 to 12 months showed that preoperative symptoms relating to the injected material, such as breast pain, lump and asymmetry, have no longer existed. The shapes of the breasts were satisfactory.

CONCLUSIONSWe believe that endoscope-assisted mammoplasty offers more satisfactory clinical results with less injury, less morbidity, less scars, more accuracy and greater patient acceptance.

Acrylic Resins ; Adult ; Breast Implantation ; methods ; Breast Implants ; Endoscopy ; Female ; Follow-Up Studies ; Foreign Bodies ; Humans ; Mammaplasty ; methods ; Silicone Gels ; Treatment Outcome ; Young Adult

OBJECTIVETo evaluate the effect and results of short and medium periods of follow-up of percutaneous balloon pulmonary valvuloplasty for critical pulmonary stenosis of neonates and infants under 6 months of age.

METHODSBetween January 2002 and December 2008, 34 consecutive patients aged from 13 to 175 days with critical pulmonary valvular stenosis underwent percutaneous balloon valvuloplasty. Patients records, catheterization data, angiograms and echocardiograms were reviewed. Patients were followed up for 6 months to 4 years (mean 25.5 months) by means of clinical examination and Doppler echocardiography.

RESULTSThe pulmonary valvuloplasty was accomplished in 32 (94%) of 34 attempts. Immediately after dilation, right ventricular systolic pressure (RVSP) decreased from (96 ± 28) mm Hg (1 mm Hg = 0.133 kPa) (49 ± 20) mm Hg (P < 0.01), the transvalvular peak to peak systolic gradient (ΔP) decreased from (89 ± 25) mm Hg to (25 ± 12) mm Hg (P < 0.01), and the right ventricular/aortic systolic pressure ratio decreased from 1.2 ± 0.5 to 0.7 ± 0.3 (P < 0.01). One patient died because of cardiac tamponade following rupture of the pulmonary valve annulus, 2 patients developed pericardial effusion, 3 patients had infundibular spasm, 3 patients had a pre-dilation by small balloon and 1 patient had weakened femoral artery pollex. After a follow up period of 6 months to 4 years 3 of 31 patients lost to follow-up. Repeat valvuloplasty was performed in 5 patients (3 neonates), no patient required surgery, and the other 23 patients did not undergo further intervention, a mean peak systolic Doppler gradient of (20 ± 13) mm Hg was found and no significant pulmonary regurgitation was seen.

CONCLUSIONSPercutaneous balloon pulmonary valvuloplasty was effective and safe for the treatment of critical pulmonary stenosis of neonates and infants under 6 months of age with good short and medium term results.

Catheterization ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Lost to Follow-Up ; Male ; Pulmonary Valve ; Pulmonary Valve Stenosis ; therapy ; Treatment Outcome

OBJECTIVETo observe mid- and long-term changes in the histopathology and electron microscopic characteristics of the acellular dermal matrix engrafted with thin split-thickness skin autograft.

METHODSTwenty-three biopsy samples were collected from 17 patients undergoing extremity scar resection, who received subsequent grafting using allogenic dermal matrix dressed with thin split-thickness skin autografts. Six months to 2 years after the grafting, the grafts were sampled for histopathological and electron microscopic observations of the layer of the epidermis, thickness of the basal membrane, structural components of the dermis, and infiltration of fibroblasts and revascularization. The data were compared with those of the normal skin samples from the patients.

RESULTSOnly the number of epidermal layers showed statistically significant difference between the skin grafts and the normal skin (16.33-/+5.89 vs 26.57-/+3.46, P=0.007). The thickness of the basal membrane of the skin grafts was similar to that of normal skin, and no significant difference was found in the number of fibroblasts and newly generated capillaries between them.

CONCLUSIONThe mid- and long-term histopathology and ultrastructures of the composite skin graft in the extremities are similar to those of normal skin, suggesting satisfactory effect of the skin grafts.

Adolescent ; Burns ; surgery ; Child ; Child, Preschool ; Cicatrix ; surgery ; Dermis ; transplantation ; ultrastructure ; Female ; Follow-Up Studies ; Graft Survival ; Humans ; Male ; Skin ; ultrastructure ; Skin Transplantation ; methods ; Skin, Artificial ; Transplantation, Autologous

OBJECTIVETo assess the effect of the balloon valvuloplasty for congenital valvular aortic stenosis (AS) in children.

METHODSA total of twenty one children with AS accepted the treatment of percutaneous balloon aortic valvuloplasty (PBAV). One of them complicated with PDA underwent PDA closure by using Amplatzer occluder in the mean time. PBAV could not be accomplished in 3 cases because the catheter could not be advanced into left ventricle and PBAV was not performed in 2 cases due to the gradient pressures across aortic valves less than 50 mm Hg (1 mm Hg = 0.133 kPa). The procedure was completed in 16 cases. The ratios of balloon/valve were 0.98 +/- 0.04 (0.92 - 1.10).

RESULTS13 cases had more than 50% gradient reduction (81.25%), 2 had 40% - 50% gradient reduction. The follow up period ranged from 3 months to 5 years. The gradient pressures rose to more than 50 mm Hg after follow up in 3 cases and they underwent repeat balloon valvuloplasty procedure or were operated successfully. There was no moderate to severe aortic insufficiency (AI).

CONCLUSIONThe result of balloon aortic valvuloplasty showed the significant hemodynamic improvement with relative safety in pediatric patients. PBAV provides another choice in comparison with surgery.

Adolescent ; Aortic Valve Stenosis ; congenital ; therapy ; Catheterization ; methods ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male

BACKGROUNDCell-based vascular therapies of endothelial progenitor cells (EPCs) mediated neovascularization is still a novel but promising approach for the treatment of ischemic disease. The present study was designed to investigate the therapeutic potentials of human umbilical cord blood-derived EPCs (hUCB-EPCs) in rat with acute myocardial infarction.

METHODSHuman umbilical cord blood (hUCB) mononuclear cells were isolated using density gradient centrifugation from the fresh human umbilical cord in healthy delivery woman, and cultured in M199 medium for 7 days. The EPCs were identified by double-positive staining with 1, 1'-dioctadecyl-3, 3, 3', 3'-tetramethylindocarbocyanine percholorate-labeled acetylated low-density lipoprotein (Dil-Ac-LDL) and fluorescein isothiocyanate-conjugated Ulex europaeus lectin (FITC-UEA-l). The rat acute myocardial infarction model was established by the ligation of the left anterior descending artery. The hUCB-EPCs were intramyocardially injected into the peri-infarct area. Four weeks later, left ventricular function was assessed by a pressure-volume catheter. The average capillary density (CAD) was evaluated by anti-VIII immunohistochemistry staining to reflect the development of neovascularization at the peri-infarct area. The graft cells were identified by double immunofluorescence staining with human nuclear antigen (HNA) and CD31 antibody, representing human origin of EPCs and vascular endothelium, respectively. Expressions of cytokines, proliferating cell nuclear angigen (PCNA), platelet endothelial cell adhesion molecule (PECAM) and vascular endothelial growth factor (VEGF) were detected to investigate the underlying mechanisms of cell differentiation and revascularization.

RESULTSThe donor EPCs were detectable and integrated into the host myocardium as confirmed by double-positive immunofluorescence staining with HNA and CD31. And the anti-VIII staining demonstrated a higher degree of microvessel formation in EPCs transplanted rats, associated with a significant improvement of global heart function in terms of the increase of left ventricular end-systolic pressure (LVESP), +dp/dtmax and -dp/dtmax as well as the decrease of LVEDP in rats with EPCs therapy comparing to the control rats (P < 0.05). Moreover, the expression of the rat PCNA mRNA and PECAM were both enhanced in the EPCs group compared with that of the control group.

CONCLUSIONSThe human umbilical cord blood-derived EPCs could incorporate into new-born capillaries in rat myocardium, induce revascularization and improve the proliferation activity in the peri-infarct area, resulting in the improvement of global heart function. This may indicate a promising stem cell resource in cell-based therapy for ischaemic diseases.

Animals ; Cells, Cultured ; Cytokines ; metabolism ; Endothelial Cells ; cytology ; physiology ; Endothelium, Vascular ; Fluorescent Antibody Technique ; Humans ; Male ; Myocardial Infarction ; metabolism ; therapy ; Neovascularization, Physiologic ; physiology ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Proliferating Cell Nuclear Antigen ; metabolism ; Rats ; Rats, Wistar ; Stem Cell Transplantation ; Stem Cells ; cytology ; Umbilical Cord ; cytology ; Vascular Endothelial Growth Factor A ; metabolism