Humans ; Infant, Newborn ; Male ; Poland Syndrome

AlM: To investigate the effect of endocapsular phacoemulsification cataract extraction and intraocular lens (lOL) implantation with a 1. 8mm or 3. 0mm clear corneal incision on total root mean square ( RMS ) value of the cornea, corneal astigmatism, spherical aberration, coma, trefoil and tear film.
METHODS:ln a prospective study, 156 age- related patients ( 196 eyes ) were randomly distributed into two groups. 1. 8mm-group comprised 94 eyes that had a silicone lOL inserted through a 1. 8mm sutureless clear corneal incision, while, 3. 0mm- group comprised 102 eyes through a 3. 0mm clear corneal incision. Postoperatively, the changes in the total RMS value of the cornea, corneal astigmatism, spherical aberration, coma, trefoil and tear film at 1wk, 1 and 3mo were determined respectively.
RESULTS:ln both groups, postoperatively at 1wk,there were statistically significant differences ( P<0. 05 ) in the total RMS value of the cornea, corneal astigmatism, spherical aberration, coma, trefoil and tear film, while, there were statistically minimal differences ( P< 0. 05 ) between 1. 8mm-group and 3. 0mm-group at 1mo, but were not statistically significantly different ( P > 0. 05 ) between two groups at 3mo postoperative.
CONCLUSlON:This study confirms that incision size has strong impact on the corneal higher-order aberrations, especially, 3. 0mm incision caused significant differences in the total RMS value of cornea, corneal astigmatism, spherical aberration, coma, trefoil and tear film compared with 1. 8mm micro-incision, therefore, micro-incision is very beneficial for clinical use in phacoemulsification.

Objective The purpose of this paper is to screen inborn errors of meta bolism (IEM) by analyzing urinary components, so as to provide laboratory guide for their diagnosis and therapy.Methods Urine samples of patients suspected to have IEM were collec- ted.Urea was de compo sed with urease and n-heptadecanoic acid was added as internal standard.Protein was denatured with ethanol and precipitate was removed by centrifugation,dried b y evaporation, the residue was trimethylsilylly derivatized with BSTFA/TMCS,and then analyzed with GC-MS for quantification of organic acids, amino acids,suga rs, polyols, purines and pyrimidines, simultaneously. This procedure is denom inated as urease pretreatment-gas chromatography-mass spectrometry (UP-GC-MS) internationally.Results Urinary samples of 327 patients from 6 provinces, cities and autonomous regions were analyzed,and 16 kinds of 27 cases of IEM were screened out with a positiv e rate of 8.26%,among which there were 3 cases of hyperphenylalaninemia,3 cases of glyceroluria,3 cases of Leigh syndrome, 2 cases of propionic acidemia, 2 case s of methylmalonic aciduria, 2 cases of von Gierke′s disease, 2 cases of fructo se-1,6-diphosphatase deficiency, 2 cases of fructosuria, 1 cases of multiple car boxylase deficiency, 1 cases of glutaric acidemia typeⅠ, 1 cases of maple sy rup urine disease, 1 cases of hyperglycinemia, 1 cases of 3-aminoisobutyric acid uria,1 cases of adult-onset typeⅡcitrullinemia,1 cases of galactosemia and 1 ca ses of Fanconi′s syndrome.Several IEM patients above had died,but satisfactory therapeutic effects had been achieved in some diseases,in cluding multiple carboxylase deficiency,methylmalonic aciduria and galactosemia. Other patients′ condition remained to be followed up.Conclusion Analysis of urinary components by UP-GC-MS provides a valuable tool for screenin g of IEM and the results will help to provide effective diagnostic and therapeut ic guide for the patients. J Appl Clin Pediatr,2005,20(2):142-144

Objective Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD, OMIM 605814 ) is a novel autosomal recessive disease results from mutations in the gene SLC25A13 that encodes for citrin, a liver-type aspartate/glutamate cartier located in the mitochondrial inner membrane. Most of the Chinese NICCD patients diagnosed by genetic analysis had the sameSLC25A13 mutations as Japanese, however, in some cases, the known mutations were not detected. This research aimed to identify novel SLC25A13 mutations in Chinese NICCD patients and to explore the experimental conditions for their genetic diagnosis.Methods Genomic DNA was extracted from blood samples of 3 NICCD patients from Taiwan (P757), Guangdong (P1194) and Hebei (P1443) Province of China, respectively; and all the 18 exons and their flanking sequences of SLC25A13 gene were sequenced. Furthermore, the identified novel mutations were diagnosed by amplification with PCR, digestion with corresponding restriction endonuclease, and agarose gel electrophoresis.Results Three novel mutations identified in SLC25A13 gene of the 3 NICCD patients were an abnormal splicing IVS7-2A>G (P757), a missense A541D (c. 1622C > A, P1194) and a nonsense R319X (c. 955C > T, P1443). The PCR-RFLP procedures for their genetic diagnosis were also established, with specific fragments on electrophoresis after digestion of the PCR products with three different restriction endonucleases Msp Ⅰ, Hpy188Ⅰ and Taq Ⅰ, respectively.Conclusions The three novel mutations in SLC25A13 gene of Chinese NICCD patients were first identified, suggesting that SLC25A13 mutation distributed in Chinese population is somewhat different from that in Japanese. Moreover, the PCR-RFLP diagnostic procedures established in this research provide valuable tools not only for the genetic diagnosis of NICCD but also for further molecular epidemiologic investigations in Chinese population.Acknowledgement We are grateful to all research subjects and their family members for their cooperation, and to many members of medical staff who contributed much to this research. This study was financially supported partially by Guangdong Provincial Research Grant for Science and Technology (No. 2004B50301008) and the Major State Basic Research Development Program of China (No. 2007CB511901 ), and by Grants-in-Aid for Scientific Research (B: Nos. 16390100 & 19390096 ) and for Asia-Africa Scientific Platform Program (AASPP) from the Japan Society for the Promotion of Science.

Cryptococcosis ; etiology ; pathology ; Glycogen Storage Disease Type I ; complications ; pathology ; Humans ; Infant ; Male ; Meningitis, Fungal ; etiology ; pathology

The authors proposed remedial measures for traditional Chinese medicine (TCM) products export faced with technical barriers to trade (TBT) according to analyzing the impact of TBT on TCM product export with economic theories, and putting forward countermeasures based on evaluation researches. TBT can be effectively coped with by the joint efforts of government, enterprises and industry association. Not only TBT can be broken through but also TCM product export can be expanded by the coordination and cooperation of our country and the society.
Commerce ; Drug Industry ; Medicine, Chinese Traditional

To study the chemical constituents of Periplocae Cortex, the separation and purification of 70% alcohol extract were carried out by column chromatographies on AB-8 macroporous resin, silica gel and preparative HPLC. The structure of the compounds were identified by NMR and TOF-MS. A new compound was isolated and identified as 21-O-methyl-Δ5-pregnene-3β, 14β, 17β, 21-tetraol-20-one-3-O-β-D-oleandropyranosyl(1-->4)-β-D-cymaropyranosyl-(1-->4)-β-D-cymaropyranosyl (1), named as periplocoside P.
Glycosides ; chemistry ; isolation & purification ; Periploca ; chemistry ; Pregnenes ; chemistry ; isolation & purification ; Saponins ; chemistry ; isolation & purification

OBJECTIVETo investigate the effects of transforming growth factor-beta1 (TGF-beta1) gene therapy on bone defect and bone rarefaction around endosseous implant.

METHODSThe primary cultured bone marrow derived stroma cells (BMSCs) was transfected by plasmid pCDNA3.1(+) -TGF-beta1, and was adhered with polylactic-co-glycolic acid (PLGA) for constructing TGF-beta1 gene-modified artificial bone. The model of rats with placed titanium implants in the proximal metaphyses of the tibiae after ovariectomy was made. The TGF-beta1 gene-modified artificial bone (experimental group), BMSCs-PLGA compound artificial bone (control group) and nothing (blank control group) were placed in the bone defect around implant. The tibiae were examined by decalcified sections with immunohistochemical method and histological analysis methods at intervals of 4 and 8 weeks after implant surgery in order to detect the expression of TGF-beta1 in new bone adjacent to the implant and the healing of the bone defect around the implant.

RESULTSThe expression level of TGF-beta1 of experimental group was higher than that of control group and blank control group at the 4th week. The histological analysis indicated that the gene-modified artificial bone had stronger osetogenic potential than others.

CONCLUSIONTGF-beta1 gene-modified artificial bone promotes the repair of the bone defect around titanium implants in osteoporotic rats.

Animals ; Bone and Bones ; Cells, Cultured ; Dental Implantation, Endosseous ; Dental Implants ; Female ; Genetic Therapy ; Prostheses and Implants ; Rats ; Stromal Cells ; Titanium ; Transfection ; Transforming Growth Factor beta1

The first genetic linkage map of Salvia miltiorrhiza was constructed in 94 F1 individuals from an intraspecific cross by using simple sequence repeat (SSR), sequence-related amplified polymorphism (SRAP) and inter-simple sequence repeat (ISSR) markers. A total of 93 marker loci in the linkage map, consisting of 53 SSR, 38 SRAP and 2 ISSR locus were made up of eight linkage groups, covered a total length of 400.1 cm with an average distance of 4.3 cm per marker. The length of linkage groups varied from 3.3 -132 cm and each of them included 2-23 markers, separately. The result will provide important basis for QTL mapping, map-based cloning and association studies for commercially important traits in S. miltiorrhiza.
Chromosome Mapping ; Genetic Linkage ; Genetic Markers ; Microsatellite Repeats ; Polymorphism, Genetic ; Salvia miltiorrhiza ; genetics

OBJECTIVETo study the compatibility of composite herbal medicines of the Ling Gui Zhu Gan Decoction.

METHODEthanol extract test solutions of the different combinations were prepared according to the orthogonal layout L16(4(5)). Pharmacologic experiments, such as the time of surviving of mice in shortage of oxygen in regular air pressure, the antagonizing effect on arrhythmia induced by chloroform and diuresis were carried out with the solutions. Variance analysis, canonical correlation and stepwise regression analysis were applied to interrelate the amount of each drug and the pharmacologic data.

RESULTThe results confirmed that Fuling and Guizhi are the basis, while Baizhuand Gancao are the adjuvans, which is conformed to the theory of TCM.

CONCLUSIONThis study provides a significant try for studying the compatibility of composite herbal medicines.

Animals ; Anti-Arrhythmia Agents ; therapeutic use ; Diuretics ; pharmacology ; Drug Combinations ; Drugs, Chinese Herbal ; isolation & purification ; therapeutic use ; Female ; Hypoxia ; drug therapy ; Male ; Medicine, Chinese Traditional ; Mice ; Phytotherapy ; Plant Extracts ; isolation & purification ; therapeutic use ; Plants, Medicinal ; chemistry ; Ventricular Fibrillation ; drug therapy