Amino Acid Metabolism, Inborn Errors ; pathology ; Child, Preschool ; Humans ; Male ; Twins

Child ; Epidermal Cyst ; Humans ; Keratoderma, Palmoplantar ; Nails, Malformed ; Skin Diseases ; Syndrome

As a clean, efficient, and renewable energy, hydrogen is regarded as a promising alternative. Because of using biomass as substrate, microbial fermentative hydrogen production can meet the need of sustainable development. The factors affecting the process of microbial fermentative hydrogen production, are analyzed in this paper on the basis of microorganisms, substrates, products and operative parameters. The parameters related to hydrogen production from organic wastes, are also mentioned.

Child ; Humans ; Hyperhomocysteinemia ; genetics ; Male

Objective To design phacoemulsification handpiece with small size and high amplitude amplification coefficient.Methods By introducing the electro-mechanical vibration characteristics of piezoelectric transducer and ultrasonic horn,and adopting the equivalent circuit method and analytical method,respectively,the size of the ultrasonic horn,quarter wavelength piezoelectric transducer and ultrasonic horn were designed.Then,modal and harmonic analysis in finite element analysis software ANSYS11.0 were used to simulate the handpiece,and to optimize the size of the handpiece after adding on needle.Results The results indicated that the longitudinal vibration frequency and amplification coefficient were very close to theoretical design.The errors were 1.4％ and 5.8％,respectively.When piezoelectric ceramics were loaded with 300 V sinusoidal voltage,the displacement amplitude at the end of the needle reached 125.5 μm,which could meet the phacoemulsification surgery needs.Conclusion This design greatly reduced the size of the handpiece,and obtained high amplitude amplification coefficient.The simulation results sufficiently validated the theoretical design.

Aldehyde Oxidoreductases ; deficiency ; Child, Preschool ; Female ; Humans ; Metabolism, Inborn Errors ; diagnosis ; enzymology ; metabolism ; Succinate-Semialdehyde Dehydrogenase ; gamma-Aminobutyric Acid ; metabolism

Objective To investigate the long-term prognosis an d recorery of children with juvenile rheumatoid arthritis(JRA).Methods The cases diagnosed JRA in our hospital over the period of 1988～1992 we re followed up for the conditions of disease,the deteriorated joints,the treatme nt and the living conditions of patients.Results Ninty-six ca ses were followed up(involving male 66 cases,female 29 cases,the mean age of on set 8.21?3.17 years )except for a case who died of lymphoma.There were 44 cases with systemic JRA ,2 cases of them died after 7-years onset and 23 cases developed severe destructive arthritis.There were 38 oligoarthritis cases,27 cases of them stiu had active disease during 10-year following-up and 9 cases were diagnosed sacroiliitis.There were 13 polyarthritis and 3 cases of them were RF positive, who had developed severe destructive arthritis.Conclusions The prognosis of juvenile rheumatoid arthritis is not desirable,especially in systemic JRA,whose prognostic factors are related to age of onset,the lasting of fever,the markers of phlegmasia activity and the condition of systemic involvement and treatment.Oligoarthritis about 30 % may develop into ankylosing spondylitis.The probability of destructive arthritis is hi gher in polyarthritis with more RF positive and poor prognosis.

Background Chemical crosslinking agent can be used to strengthen the intensity of sclera tissue,but the intensity of the sclera may be influenced by different crosslinking methods.Objective The aim of this study was to compare the effectiveness of collagen crosslinking on porcine sclera between whole-eye crosslinking method and scleral strip crosslinking method.Methods Whole-eye crosslinking or sclera strip crosslinking was performed on 70 fresh porcine eyeballs in five groups using 1％ genipin,1％ glutaraldehyde or PBS respectively for 40 minutes.After crosslinking,10 sclera strips with l0 mm×4 mm from the temporal lateral were prepared in every group for the stress-strain measurement using a Instron5848 microtester,and the other 4 scleral strips in each group were extracted for the thermal shrinkage temperature test.Results Biomechanical property test reveled that the elastic modulus value of sclera strips reduced by 70.0％-82.8％ in the whole-eye crosslinking method group compared with scleral stip crosslinking method group after treated with 1％ genipin ((8.98 ± 1.81) MPa vs.(10.85 ± 1.83) MPa,t =3.375,P =0.003)) and 1％ glutaraldehyde((12.78 ±2.91) MPa vs.(18.25 ±5.16) MPa,t =4.007,P =0.001)) ;The tensile stress of whole-eye crosslinking method group was 54.9％-90.1％ of scleral stips method group,showing significant decline after corsslinked of whole-eye in 5％,10％,15％ and 20％ strain conditions (all P ＜ 0.05).Thermomechanics test showed that the thermal shrinkage temperature was lower in the whole-eye crosslinking group compared with scleral stip crosslinking group after treated with both 1％ genipin ((68.8 ±0.9)℃ vs.(74.8± 1.3)℃,t=11.129,P=0.000)) and 1％ glutaraldehyde((73.3±0.9)℃ vs.(79.3±1.3)℃,t=11.112,P=0.000)).Conclusions Different crosslinking methods have an influence on the efficacy of collagen crosslinking on porcine sclera.Sclera strip crosslinking offers a better crosslinking intensity for selera.

In order to identify Aucklandiae Radix, Vladimiriae Radix, Inulae Radix, Aristolochiae Radix and Kadsurae Radix using ITS2 barcodes, genomic DNA from sixty samples was extracted and the ITS2 (internal transcribed spacer) regions were amplified and sequenced. The genetic distances were computed using MEGA 5.0 in accordance with the kimura 2-parameter (K2P) model and the neighbor-joining (NJ) phylogenetic tree was constructed. The results indicated that for Aucklandiae Radix (Aucklandia lappa), Vladimiriae Radix (Vladimiria souliei and V. souliei var. cinerea), Inulae Radix (Inula helenium), Aristolochiae Radix (Aristolochia debilis) and Kadsurae Radix (Kadsura longipedunculata), the intra-specific variation was smaller than inter-specific one. There are 162 variable sites among 272 bp after alignment of all ITS2 sequence haplotypes. For each species, the intra-specific genetic distances were also smaller than inter-specific one. Furthermore, the NJ tree strongly supported that Aucklandiae Radix, Vladimiriae Radix, Inulae Radix, Aristolochiae Radix and Kadsurae Radix can be differentiated. At the same time, V. souliei (Dolomiaea souliei) and V. souliei var. cinerea( D. souliei var. cinerea) belonging to Vladimiriae Radix were clearly identified. In conclusion, ITS2 barcode could be used to identify Aucklandiae Radix, Vladimiriae Radix, Inulae Radix, Aristolochiae Radix and Kadsurae Radix. Our study may provide a scientific foundation for clinical safe use of the traditional Chinese medicines.
Aristolochia ; classification ; genetics ; Base Sequence ; DNA Barcoding, Taxonomic ; methods ; DNA, Plant ; genetics ; DNA, Ribosomal Spacer ; genetics ; Drugs, Chinese Herbal ; chemistry ; classification ; Molecular Sequence Data ; Phylogeny ; Plants, Medicinal ; classification ; genetics ; Quality Control

Objective To appraise the bowel control and quality of life after transanal improvement of Soave one-stage pull-through operation for Hirschsprung′s disease(HD) in infants.Methods Twenty-five patients(aged from 2 months to 3 years old) with HD underwent improved endorectal pull-through procedure and postoperative follow-up for 5 months to 6 years(average 4.5 years) including the occurrence of operation related complications sush as bowel control,constipation or postoperative enterocolitis and quality of life.Results Twenty-one patients completed the study were with a mean follow-up.The assessment of anal continence was graded as normal(4 scores),good(3 scores),and fair(2 scores) according to the previous study of other investigators.Of the 21 patients,16 were graded as normal,whereas 4 were as good and 1 fair.One patient developed constipation and 2 patients had postoperative enterocolitis.Conclusion Modified Soave procedure for Hirschsprang′s disease can get a good bowel control outcome and quality of life.