Objective To explore the value of electroneurophysiological detection in diagnosis of children progressive muscular dystrophy (PMD).Methods The clinical features and laboratory data were analyzed in 32 children with PMD ,and electromyography(EMG) and nerve conduction velocity(NCV) were performed.Parameters studied included spontaneous activity , duration and amplitude of motor unit potential(MUP),pattern of recruitment as strong contracting,sensory conduction velocity(SCV),motor conduction velocity(MCV), distal latency and distal amplitude. Results The abnormality rates of spontaneous potentials was 49.2% and 80.9% in tibialis anterior.The decrease of duration of MUP was 29.7%-62.4%.Amplitude of strong contracting was significantly decreased.There were different from those in normal children(P

To study the related substances in nicergoline, electrospray positive ionization high resolution TOF/MS was used for the determination of the accurate mass and elemental composition of the related substances. Triple quadrupoles tandem MS/MS was employed for the determination of the fragmentations of the parent ions. 16 related substances were detected and identified to be eight synthetic by-products and eight degradation products, by using impurity references matching, product mass spectra fragmentations elucidation, and verified further according to synthetic processes and stress testing results. The results obtained are valuable for nicergoline manufacturing process control and quality assurance.
Chromatography, High Pressure Liquid ; Nicergoline ; chemical synthesis ; chemistry ; Quality Control ; Tandem Mass Spectrometry

Acupuncture Therapy ; Adult ; Humans ; Male ; Torticollis ; therapy

Adult ; Aged ; Aged, 80 and over ; Humans ; Middle Aged ; Pneumoconiosis ; diagnostic imaging ; Radiography, Thoracic ; methods ; Tomography, X-Ray Computed ; X-Ray Film

BACKGROUND:Adipose-derived stem cel s are totipotent stem cel s in the adipose tissue, and have the function of self-renewal and multi-directional differentiation. Human adipose-derived stem cel s are ideal seed cel s with stable genetic milieu and few rejections.
OBJECTIVE:To extract human adipose-derived stem cel s from human omental adipose tissue and to identify the cel s by adipogenic and osteogenic induction.
METHODS:Omental adipose tissues were col ected from surgical patients to isolate and culture adipose-derived stem cel s using type I col agenase digestion, filtration and centrifugation. Cel growth was observed and proliferative curve of human adipose-derived stem cel s were drawn by cel counting method to calculate the doubling time at logarithmic growth phase. After adipogenic and osteogenic induction, induced cel s were identified using oil red O and alizarin red staining, respectively.
RESULTS AND CONCLUSION:Human adipose-derived stem cel s were successful y isolated from the omentum tissues of surgical patients. Adherent cel s were fusiform-shaped and like fibroblasts. The growth curve of passage 3 cel s was in S shape, and the doubling time was 45.90 hours. After adipogenic and osteogenic induction for 2 and 3 hours, respectively, oil red O staining showed unequal-sized orange fat droplets, and alizarin red staining showed typical calcified nodules that were in orange. These findings indicate that adipose-derived stem cel s have the adipogenic and osteogenic capacity.

Objective To improve the recognition for infantile spinal muscular atrophy (SMA-Ⅰ) and the level of early diagnosis,intervention and treatment for SMA-Ⅰ.Methods The clinical data of 39 patients with SMA-Ⅰ were analyzed retrospectively, including the clinical manifestations, neural electrophysiological characteristics, geno-type, diagnosis,treatment and prognosis of SMA-Ⅰ.Results Of the 39 cases with SMA-Ⅰ , 37 cases (94.9％) had onset in 6 months after birth.The paralyses of the limbs were symmetrical and flaccid.The lower was more severe than the upper , and the proximal was more severe than the distal, hypotonia and tendinous reflex disappears.Thirty-two cases (82.1％) had normal serum creatine kinase, and 7 cases (17.9％) increased slightly.Nerve electrophysiological examination showed that 169 (96.0％) had spontaneous potentials in 176 muscles.Of 160 limb muscles,35 (21.8％) released few motor unit potential (MUP) ,117 (73.1％) extended the duration of MUP and 104 (65.0％) increased the amplitude of M UP.Of 167 peripheral motor nerves, 160 (95.8％)decreased the amplitude of the compound muscle action potential and 162 (97.0％) had normal motor conduction velocity.Of 93 peripheral sensory nerves, 93 (100.0％) had normal range of the conduction.The gene detection showed that 38 cases had homozygous deletion of exon 7,8, and 1 case had homozygous deletion of exon 7 and heterozygous deletion of exon 8.In the effective follow-up of 30 cases,6 cases died in the 2-3 months after birth,4 cases died in 10 months after birth, 12 cases died in 12-18 months after birth.Six cases survived to 2 years old,2 cases survived to 3 years old,and all of them died of pulmonary infection.Conclusions There are typical clinical and nerve electrophysiological characteristics for SMA-I.Nerve electrophysiological examination can be used as an important method for diagnosis and differential diagnosis for SMA-I.Genetic testing can be used to identify the disease and make prenatal diagnosis.Through comprehensive intervention the quality of life can be improved in SMA-Ⅰ children.

Aged, 80 and over ; China ; epidemiology ; Communicable Diseases ; epidemiology ; virology ; Female ; Humans ; Influenza A Virus, H7N9 Subtype ; Influenza, Human ; epidemiology ; virology

BACKGROUND: In vitro differentiation of embryonic stem cells into hepatocytes has been successfully reported to a certain degree; however, whether embryonic stem cells are able to effectively enter hepatic plate of host after intrahepatic transplantation, whether embryonic stem cells can further differentiate into hepatocytes and express hepatocyte function, and risk factors for neoplastic formation are still unclear at present. OBJECTIVE: To study the intrahepatic transplantation of embryonic stem cells-derived hepatic stem cells in therapeutic liver repopulation models, and to investigate the liver tissue replacement, growth and differentiation in vivo, and neoplastic formation.DESIGN: Randomized controlled animal study.SETTING: Department of Pediatric Surgery, the Second Hospital affiliated to Sun Yat-sen University. MATERIALS: Twenty-four BALB/c mice, 6-8 weeks old, weighing 20-35 g, irrespective of gender, were provided by Guangzhou Experimental Animal Center. Embryonic stem cells-derived hepatic stem cells were differentiated from embryonic stem cells. E14 was provided by Stem cell Center of our hospital. METHODS: This study was performed at the Stem Cell Center, the Second Hospital affiliated to Sun Yat-sen University from July 2006 to June 2007. Twenty-four mice were randomly divided into a liver repopulation model + stem cell transplantation group (group A) and a liver resection + stem cell transplantation group (group B), with 12 mice in each group. Mice in the group A were intraperitoneally injected with 50 mg/kg retrorsine once every two weeks for totally twice. Four weeks after the second injection, about 70% liver was resected. And then, the embryonic stem cells-derived hepatic stem cells, labeled by 1×105 carboxy fluoresce in diacetate succinimidyl ester (CFDA-SE), were transplanted into mouse liver through portal vein. On the other hand, 70% liver of mice in the group B was resected and embryonic stem cells-derived hepatic stem cells were transplanted into mouse liver. MAIN OUTCOME MEASURES: The distribution, incorporation, and proliferation of transplanted cells were observed under fluorescent microscopy. Two weeks later, hepatic function was stained with albumin fluorescence immunoassay (double fluorescence staining) and assayed by level of serum albumin. Embryonic stem cells-derived hepatic stem cells were poured into liver of remedial liver regeneration mice, and undifferentiated embryonic stem cells were transplanted into subcutaneous tissue in axillary region as the controls to observe neoplastic formation in embryonic stem cells-derived hepatic stem cells. RESULTS: ① Growth of hepatic stem cells in recipient mice: One week after transplantation of CFDA-SE-labeled embryonic stem cells-derived hepatic stem cells, some scattered region was green under fluorescent microscopy. The area of green region increased apparently in 2 weeks, and cord-like structure could be observed. ② Liver function: Immunofluorescent staining of albumin (double fluorescence staining) demonstrated that labeled cells expressed positive albumin (yellow fluorescence) in liver tissue of recipient mice, but there was not significant difference in serum albumin level between group A and group B (P > 0.05). ③ Reliability of hepatic stem cell transplantation: Teratoma did not form over 6 months; however, transplantation of undifferentiated embryonic stem cells in the axillary region could cause formation of teratoma after 6 weeks. CONCLUSION: The transplantation of embryonic stem cells-derived hepatic stem cells in therapeutic liver repopulation model mice can effectively and further grow and differentiate, or even partially express hepatocyte function; in particular, the transplantation is safe.

Adolescent ; Antimetabolites, Antineoplastic ; adverse effects ; therapeutic use ; Child ; Child, Preschool ; Drug Interactions ; Drug Therapy, Combination ; Female ; Humans ; Male ; Methotrexate ; adverse effects ; therapeutic use ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; Sex Factors ; Tetrahydrofolates ; administration & dosage ; therapeutic use ; Treatment Outcome

Adrenal Cortex Hormones ; therapeutic use ; Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; Female ; Humans ; Infant ; Treatment Outcome