Humans ; Stem Cell Transplantation ; Tissue Engineering ; trends ; Wound Healing

OBJECTIVETo investigate the effect of compound Danshen injection combined with prostaglandin E1 low molecular weight heparin calcium to dextran-40 preventing hepatic veno-occlusive disease (HVOD) after hematopoietic stem cell transplantation (HSCT).

METHODSA total of 520 patients who received HSCT in the authors' hospital from May 1998 and December 2009 were subjected, among whom 231 patients received autologous peripheral blood stem cell transplantation, 125 received HLA-identical sibling HSCT, 49 received HLA-identical/mismatched unrelated HSCT and 115 received HLA-haplotype HSCT. All patients were treated by intravenous dripping of CSI 40-60 mL, dextran-40 250-500 mL, prostaglandin-E1 40-60 microg, and subcutaneous injection of low molecular weight heparin calcium 3 000-5 000 IU every day, the preventive effect on HVOD after HSCT was observed.

RESULTSHVOD occurred and caused death only in 1 case of the 520 patients observed, the incidence was 0.19%. Neither obvious adverse reaction nor coagulation disorder was found.

CONCLUSIONCompound Danshen Injection combined with prostaglandin E1, low molecular weight heparin calcium and dextran-40 is a safe and effective protocol for the prevention of HVOD after HSCT.

Adolescent ; Adult ; Alprostadil ; therapeutic use ; Child ; Child, Preschool ; Combined Modality Therapy ; Dextrans ; therapeutic use ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Heparin, Low-Molecular-Weight ; therapeutic use ; Hepatic Veno-Occlusive Disease ; etiology ; prevention & control ; Humans ; Male ; Middle Aged ; Phenanthrolines ; therapeutic use

Objective: To compare the efficacy of different treatment protocols in treating transient synovitis of the hip (TSH) in children and to optimize the clinical treatment strategy for this condition. Methods: Ninety kids with TSH were divided into a control group, a chiropractic group and a chiropractic plus foot bath group using the random number table method, with 30 cases in each group. The control group was treated with conventional traction; the chiropractic group was given chiropractic treatment based on the control group; the chiropractic plus foot bath group was given Chinese medicine foot bath based on the chiropractic group. Traction and foot bath were conducted once daily while chiropractic was done once every other day, all with 14 d as a treatment course for a total of two courses. Changes in the visual analog scale (VAS) score and range of motion (ROM) of the hip joint in the three groups were observed, and the efficacy was compared. Results: The total effective rate was 93.3％ in the chiropractic plus foot bath group, versus 76.7％ in the chiropractic group and 66.7％ in the control group, and the total effective rate was notably higher in the chiropractic plus foot bath group than in the other two groups (both P<0.05). Respectively after the first and second treatment course, the VAS score decreased significantly in each of the three groups compared with that before treatment (all P<0.01), and the ROM of the hip joint in flexion increased significantly (all P<0.01). After two treatment courses, the VAS score was lower in the chiropractic plus foot bath group than in the other two groups (both P<0.05), and its ROM of the hip joint in flexion was larger than that in the other two groups (both P<0.05). Conclusion: Based on traction, chiropractic plus Chinese medicine foot bath can effectively reduce pain and improve motor function of the hip joint in treating TSH.

A strain was isolated from internal organ of died porcine about 8 weeks with purulent pneumonia,arthritis,pyogenic arthritis and endocarditis in April 2007.Objectives of the study are to confirm the genus of the strain,pathopoiesis,and drug sensitivity.The mainly study methods:the first,the strain was identified by the phenotype and the characteristics of the biochemistry,sequence 16S rDNA genes of the strain was analyzed by molecular biology technology,finally animal experiment and drug sensitivity testing were done.The results of the phenotype and the characteristics of the biochemistry showed that it is greatly similar to Actinomyces hyovaginalis,16S rRNA sequence analysis exhibited the homology achieved to 99.2% com-pared with group III strains of Actinomyces hyovaginalis,and the phyletic evolution analysis also indicated that it has mostly relationship with group III strains of Actinomyces hyovaginalis.Animal experiment dis-covered it has highly pathogenicity to Mus musculus albus;Drug sensitivity testing showed that it is hyper-sensitive to Erycin,Gentamicin and Amikacin.So,the result of the study confirmed that the strain is Actin-omyces hyovaginalis III with the pathogenicity.

Background Statistic data revealed that different retinopathy of pre-term infants have different susceptibility to retinopathy of prematurity (ROP),which may be associated with polymorphism of vascular endothelial growth factor(VEGF) gene.Objective This study was to determine the association of polymorphisms of VEGF gene with the risk for ROP.Methods This research was approved by Ethics Committee of Hunan Children's Hospital,and written informed consent was obtained from the parents of patients.A prospective case-controlled study was designed.Ninety-nine ROP patients in Hunan Children' s Hospital and 88 pre-termed children without ROP were included from January,2006 to December,2009.Thirty-nine patients who received retinal photocoagulation or cryotherapy were included as the treatment group,and 60 untreated but spontaneously regressed ROP patients as the non-treatment group.No significant differences were seen in demography between with the ROP group and the without ROP group,or between the treatment group and the non-treatment group (all P＞0.05).2 mL of peripheral blood was collected for the extraction of DNA.Gene polymorphisms of VEGF-A+405 and VEGF-A936 were detected using the pyrosequencing method.Results No significant difference was found in the frequencies of the VEGF-A+405 gene polymorphisms between the ROP group and without ROP group (P =0.071,OR =0.675,95 ％ CI =0.444-1.026).Also no significant difference was found in the frequencies of the VEGF-A936 gene polymorphisms between with the ROP group and without the ROP group (P =0.118,OR =0.768,95 ％ CI=2.823-4.614).However,the frequencies of the VEGF-A+405 gene polymorphisms were significantly higher in the ROP treatment group than the non-treatment group (P＜0.01,OR--0.857,95 ％ CI =5.239-14.024),and VEGF-A936 gene polymorphisms was also significantly higher in the ROP treatment group than the non-treatment group (P =0.000,OR =3.609,95 ％ CI =0.711-0.829).Conclusions There is no association between the VEGF-A+405/VEGF-A936 single nucleotide polymorphism with the risk of ROP,but polymorphisms of VEGF gene may be correlated with the prognosis of ROP.The carrier of VEGF-A +405 /VEGF-A936 allele is more susceptible to ROP progression.

Objective To investigate the effect of 1 800 MHz electromagnetic radiation on activity of SOD and GSH－Px in the skin tissues of SD rats.Methods A total of 98 healthy SD rats with SPF level,aged 4 weeks, were randomly divided into radiation group and control group.The radiation group was totally exposed under 1 800 MHz electromagnetic wave with seven different power density of radiation of 0.1 mW/cm2,0.3 mW/cm2,0.5 mW/cm2 , 0.7 mW/cm2, 0.9 mW/cm2, 1.0 mW/cm2and 1.2 mW/cm2respectively.It lasted 21 days and for a period of 12 hours a day. After radiation,the activity of SOD and GSH－Px in the skin tissues were detected by enzyme marker. Results In radiation group,the activity of SOD and GSH－Px in the skin tissues of SD rats were decreased under 0.3 mW/cm2and 0.5 mW/cm21 800 MHz electromagnetic wave. Compared with the control group, there was a significantly difference in radiation group (P＜0.05) .While under other four 1 800 MHz electromagnetic waves, the activity of GSH－Px and SOD in the skin tissues showed no statistical difference between the two groups (P＞0.05) . Under 1 mW/cm21 800 MHz electromagnetic wave, the activity of GSH－Px showed no statistical difference between two groups (P＞0.05) . Conclusion The power density of 0.3 mW/cm2and 0.5 mW/cm21 800 MHz electromagnetic wave can reduce the activity of GSH－Px and SOD in the skin tissues of rats.

Adult ; China ; epidemiology ; Circoviridae Infections ; epidemiology ; virology ; DNA, Viral ; analysis ; Female ; Genotype ; Hepatitis, Viral, Human ; virology ; Humans ; Male ; Substance Abuse, Intravenous ; virology ; Torque teno virus ; genetics ; isolation & purification

OBJECTIVETo delineate the characteristics of the clinical manifestation, pathology of skeletal muscle and gene mutations of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episode (MELAS) in children.

METHODThe clinical manifestation, laboratorial data, brain images, muscle pathology and mitochondrial gene mutations were analyzed in 24 patients with MELAS who were diagnosed in Department of Pediatrics, Peking University First Hospital. Their prognosis was evaluated by following up.

RESULTSymptoms of central nervous system such as stroke-like episodes, vomiting, convulsion and headache were present in all the 24 cases. Nine cases had the symptoms of myopathy. Twenty cases had developmental delay. Short stature, being thin and hairy was very common in these cases. Serum lactate level increased in all the cases, pyruvate increased in 17 cases. Elevated CSF lactate was found in 2 cases. Magnetic resonance imaging (MRI) was performed on 24 cases, out of them 23 were abnormal. The lesions mainly involved cerebral lobes. Occipital lobe was the most common site of lesions. Computed tomography (CT) was performed on 13 cases, low density lesions were present in 10 cases, basal ganglia calcifications in 5 cases. Muscle biopsy was performed on 8 cases, ragged-red fibers (RRF) were found in 4/8 cases, and abnormal accumulation of mitochondria were found in 3/8 cases. The mtDNA gene mutational analysis showed A3243G mutation in these patients. The mutation rates varied from 11.6% to 75.0%. The same mutation were found in 4/5 mothers who had the genetic tests, and the mutation rates of the mothers varied from 15.0% to 23.6%. The clinical information of 11 cases was available through recent following up. Three cases died, the others had some degrees of mental retardation.

CONCLUSIONChildren with MELAS had various clinical manifestations. Central nervous system and skeletal muscle were usually involved. Short stature and hypertrichosis were common signs. The prognosis of this disease was disappointing. mtDNA A3243G was the most common mutation in MELAS. Fully understanding the characteristics of its clinical manifestation, laboratory tests, brain image, muscle pathology and molecular features can be helpful to the early diagnosis and treatment.

Acidosis, Lactic ; blood ; Adolescent ; Brain ; diagnostic imaging ; pathology ; Child ; Child, Preschool ; DNA Mutational Analysis ; DNA, Mitochondrial ; genetics ; Electroencephalography ; Female ; Follow-Up Studies ; Humans ; Infant ; MELAS Syndrome ; diagnosis ; genetics ; pathology ; Magnetic Resonance Imaging ; Male ; Muscle, Skeletal ; diagnostic imaging ; pathology ; Point Mutation ; Pyruvic Acid ; blood ; Stroke ; diagnostic imaging ; genetics ; pathology ; Syndrome ; Tomography, X-Ray Computed

OBJECTIVERett syndrome (RTT) is a neurodevelopmental disorder occurring almost exclusively in females as sporadic cases due to de novo mutations in the methyl-CpG-binding protein 2 gene (MECP2). Familial cases of RTT are rare and are due to X-chromosomal inheritance from a carrier mother. Recently, DNA mutations in the MECP2 have been detected in approximately 84.7% of patients with RTT in China. To explain the sex-limited expression of RTT, it has been suggested that de novo X-linked mutations occur exclusively in male germ cells resulting therefore only in affected daughters. To test this hypothesis, we have analyzed the parental origin of mutations and the XCI status in 15 sporadic cases with RTT due to MECP2 molecular defects.

METHODSAllele-specific PCR was performed to amplify a fragment including the position of the mutation. The allele-specific PCR products were sequenced to determine which haplotype contained the mutation. It was then possible to determine the parent of origin by genotyping the single nucleotide polymorphism (SNP) in the parents. The degree of XCI and its direction relative to the X chromosome parent of origin were measured in DNA prepared from peripheral blood leucocytes by analyzing CAG repeat polymorphism in the androgen receptor gene (AR).

RESULTSExcept for 2 cases who had a frameshift mutation; all the remaining 13 cases had a C-->T transition mutation. Paternal origin has been determined in all cases with the C-->T transition mutation. For the two frameshift mutations, paternal origin has been determined in one case and maternal origin in the other. The frequency of male germ-line transmission in mutations is 93.3%. Except for 2 cases who were homozygotic at the AR locus, of the remaining 13 cases, 8 cases had a random XCI pattern; the other five cases had a skewed XCI pattern and they favor expression of the maternal origin allele.

CONCLUSIONDe novo mutations in sporadic RTT occur almost exclusively on the paternally derived X chromosome and that this is most probably the cause for the high female: male ratio observed in sporadic cases with RTT. Random XCI was the main XCI pattern in sporadic RTT patients. The priority inactive X chromosome was mainly of paternal origin.

Chromosome Aberrations ; Chromosomes, Human, X ; Female ; Humans ; Male ; Methyl-CpG-Binding Protein 2 ; genetics ; Mutation ; Polymorphism, Single Nucleotide ; Rett Syndrome ; genetics ; X Chromosome Inactivation

OBJECTIVESpinal muscular atrophy (SMA), characterized by degeneration of the anterior horn cells in the spinal cord and symmetric proximal muscle weakness, is the most common autosomal recessive neuromuscular disease in infants and children. In Caucasian population, about 95% of clinically typical patients lack both copies of the telomeric survival motor neuron gene (SMN 1). However, the detection rate of the homozygous absence in Chinese patients is still controversial, which may lead to reduced confidence in the SMA genetic testing in clinical practice. The purpose of the current study was to determine the frequency of homozygous deletions of SMN 1 in Chinese patients, to evaluate the significance of the SMN 1 homozygous deletion assay in clinical applications, and the impact of the clinical re-visit followed by the genetic testing.

METHODSTotally 85 patients initially suspected of SMA were referred for SMA genetic testing. A polymerase chain reaction and restriction fragment length polymorphism (PCR-RFLP) assay was used to detect the homozygous absence of SMN 1. Clinical re-visit was performed by the pediatric neurology specialists according to the international SMA diagnostic criteria, and histological examinations were carried out when they were necessary.

RESULTSAbsence of both copies of SMN 1 exon 7 were found in 57 (67%) of the 85 patients, and 28 patients (33%) had at least one copy. For the 28 patients with negative results, 19 were followed up by the pediatric neurologists. The clinical diagnosis of SMA could be excluded in 15 patients, but retained in the other 4 patients after the clinical re-evaluation and histological examinations. Thus, approximately 95% of the patients with clinically typical SMA in our cohort lacked both copies of SMN 1. Homozygous deletions of SMN 1 were detected in 96% (22/23), 93% (28/30) and 100% (7/7) of the patients with SMA type I, type II and type III, respectively. There was no significant difference in the deletion frequency among the subtypes.

CONCLUSIONSThe frequency of homozygous deletions of SMN 1 in this series of Chinese SMA patients was about 95%, which is similar to that reported in Caucasian population. The genetic test of homozygous deletions of SMN 1 should be considered as the first line test for the Chinese patients suspected of SMA. The clinical re-visit and re-evaluation which is essential in clinical diagnosis, genetic counseling and medical management, should be routinely performed after the genetic testing.

Asian Continental Ancestry Group ; genetics ; Child ; Child, Preschool ; Exons ; Female ; Gene Deletion ; Genetic Testing ; Homozygote ; Humans ; Infant ; Infant, Newborn ; Male ; Muscular Atrophy, Spinal ; diagnosis ; genetics ; Survival of Motor Neuron 1 Protein ; genetics