Objective To explore the clinical features of Marfan syndrome (MFS) and its virulence gene mutation of FBN1.Methods Clinical data of 2 children with MFS were retrospectively analyzed, and pertinent literatures were reviewed. Results Case one was a 1 year and 10 months old boy with a special face, bilateral lower eyelid edema, high palatal arch, slender fingers and toes. A little of moist rales in lung could be heard, and systolic accentuated in apex could be heard too. Echocardiography showed that aortic coronary sinus dilated, aorta and pulmonary artery broadened, left ventricular diverticulum, a small amount of mitral regurgitation,and moderate tricuspid regurgitation. Electrocardiogram showed incomplete right bundle branch block. Gene detection found a c.3037G>A mutation (p.Gly1013Arg) inFBN1. Case two was a 12 years old slender boy with spider-like ifnger/toe, high myopia, 2/6 systolic and diastolic murmur in the ifrst and two auscultation area in aortic valves. Echocardiography showed the aortic sinus signiifcantly broadened, aortic incompetence, mild pulmonary regurgitation and left ventricular enlargement. Gene detection found heterozygous mutation of c.1876G>A (p.Gly626Arg) in FBN1, which has not been reported.Conclusion The diagnosis of MFS can be conifrmed byFBN1 gene detection. A new mutation of c.1876G>A (p.Gly626Arg) was detected.

Objective To investigate the association between MPO gene single nucleotide polymorphisms (SNP) loci (rs2333227,-643G/A) and clinical characteristics in Kawasaki disease (KD) in Han population in central China. Methods A case-control study was performed. Two hundred and thirty-seven children with KD and 249 normal children were recruited. The polymorphism distribution of SNP was detected using PCR-RFLP. The clinical data of children with KD were collected. Results The frequency of SNP loci (rs2333227) genotypes (GG, GA, AA) was signiifcantly different between children with KD and normal children (P=0.039), the allele frequency was also signiifcantly different between two groups (P=0.012), and the G allele was the risk factor. Compared with other genotypes, KD children with GG genotype had higher frequency in hand-foot edema (P=0.029). The SNP polymorphism was also associated with peritoneal effusion (P=0.028), however this SNP polymorphism was not associated with conjunctival hyperemia, oral mucosa lesions, and coronary artery lesion (P>0.05), also was not associated to imaging characteristics of hepatomegaly, splenomegaly, and lobular pneumonia (P>0.05). Conclusion The SNP loci (rs2333227) in MPO gene was associated with KD susceptibility, the G allele was a risk factor, and the SNP polymorphisms is associated with some clinical characteristic.

Objective To explore the influence of fixed in advance on the positive rate and integral of neutrophilic alkaline phosphatase (NAP) dyeing.Methods Totally 182 cases of fresh venous blood from inpatients in the top three hospital department of hematology were randomly selected and anticoagulated in the EDTA-K2 vacuum tube.Three blood smears from which were prepared as follows:the first blood smear(Named A) NAP dyeing completed within an hour;the second one (Named B) were fixed in advanceand NAP dyeing after one day;the third one (Named C) didn′t do any processing and NAP dyeing after one day.At the same time,the blood samples were taken from the fresh blood,and the blood smears were prepared and stained with NAP in an hour.NAP dyeing were performed by NAP dyeing for the blood smears,and 100 neutral rods,nucleus granulocyte were observed under microscope in the oil mirror vision,the test results record by positive rate and integral.Results No significant difference of NAP positive rate and integral was found in EDTA-K2 anticoagulation venous blood smear A when compared with fresh peripheral blood(P>0.05).The NAP positive rate and integral of EDTA-K2 anticoagulation venous blood smear B was slightly lower than that from fresh peripheral blood,but no significant difference was found(P>0.05).However,the NAP positive rate and integral in EDTA-K2 anticoagulant venous blood smear C has a significant difference from fresh peripheral blood(P<0.05).Conclusion The NAP dyeing results of EDTA-K2 anticoagulation venous blood smear fixed and placed one days are still reliable,while the NAP dyeing results was significantly reduced in the unfixed EDTA-K2 anticoagulation venous blood smear placed after one day.

Objective:To investigate the effect of PBL+LBL double-track in long-term-system clinical medicine in Gynecology and Obstetrics.Methods:We divided the students of 2001-seven-year-progrems in random into two groups: trial group practiced PBL+LBL double-track teaching,control group practiced LBL teaching.Results The results showed that although the two groups had no significant difference in examinational total score and in foundational subjects,but the trial group had a significant higher score in comprehend subjects.The students exhibited great enthusiasm to the PBL.And the students learned and absorbed more knowledge of gynecology and obstetrics and literature retrieval.The education method played a comprehensive functional role for the teacher-directed and student-oriented tutorial process,and the teaching effects improved.Meanwhile,the teachers need to be prepared with more knowledge to meet the students query.Conclusions:PBL+LBL double-track teaching method can improve the students'activeness and can culture their ability of self-study;and at the same time the new teaching method can remain the systematicness,profundity and extent of knowledge.It is a better way in clinical education,and is well accepted by both students and teachers as it can greatly improve teaching efficacy.

Objective To establish a transfer model for excess relative risk (ERR) for radiation-related leukemia from Japanese population to Chinese population.Methods Combined ERR of several subtypes of leukemia published in 1994, with the corresponding leukemia baseline incidence rates obtained from Cancer Incidence in Five Continents Vol.Ⅸ (CI5-Ⅸ) for Japanese population and Chinese population, a weighted risk transfer model was employed between an additive model and a multiplicative model, to execute ERR transfer.Results A range of weighing factors was proposed for risk transfer models:weighing factor was 0.4 for male and 0.3 for female, acute lymphoblastic leukemia, acute myeloid leukemia and chronic myeloid leukemia.The uncertainty for ERR transfer was characterized by lognormal distribution.Conclusions Based on the difference of baseline incidence rate for subtypes of leukemia between Japanese population and Chinese population, the transfer model and these weighing factors discussed in the present study could be applicable to transfer ERR for radiation-related leukemia from Japanese population to Chinese population.

Objective: To established a method for the quality standard of Yupingfeng Oral Liquid(Radix Astragali, Radix Saposhnikoviae and Rhizoma Atractylodis Macrocephalae). Methods:Radix Astragali, Radix Saposhnikoviae and Rhizoma Atractylodis Macrocephalae in oral liquid were identified by TLC. The content of astragaloside IV was determined by HPLC-ELSD. Results:The study showed that spots of samples on TLC can be well separated and the method had strong specificity. The average recovery was 97.75% and RSD was 1.31%. Conclusion:The method is simple, sensitive and accurate. It can be used for quality control of Yupingfeng Oral Liquid.

Background The ultrastructure change and growth of corneal nerve after laser in situ keratomileusis (LASIK) are some influent factors to the stability of tear film and the sensibility of cornea.Some relevant studies are lack up to now.Objective This study is to observe the changes of ultrastructure of corneal epithelium and regeneration of corneal nerve fiber.Methods LASIK was performed on the lateral eyes of 48 New Zealand white rabbits.Rabbit eyes were excavated at instant in postoperation,one day,seven days,one,three and six months after LASIK.Change of corneal ultrastructure and corneal nerve staining were examined under the scanning electron microscope (SEM) and transmmision electron microscope (TEM) at the time points mentioned above.The numbers of microvilli of corneal epithelial cells in different postoperative time were analyzed.10% AuCl was used to evaluate the growth status of corneal nerve in different time after LASIK.Results Irregularity of microvillus of corneal epithelial cells,degrease of cell density were seen under the TEM and some cavities could been observed in the instant of postoperation.The connection abnormality of intercells,dropsy and rupture of microvillus were presented under the SEM,However,the ultrastructure of corneal epithelial cells was almost normal in 7 days after LASIK.The numbers of microvillus in corneal epithelial cells were significantly declined in the postoperative instant group compared with preoperative group (P＜0.05),but no evidently difference was found in postoperative 1 day and 7 days groups compared with preoperative group(P＞0.05).Corneal nerve staining showed that in 1 day after surgery,nerve plexus was deprivation and boundary of nerve injury was clear and nerve fibers were cut off.After one month some reborn nerve fiber grew into the cornea flap.Reborn corneal nerve fiber can be seen at center laser ablation area after six months of LASIK.Conclusion The ultrastructure change of corneal epithelial cells in the early stage after operation may be a important factor causing dry eye symptom following LASIK.The growth fashion of corneal nerve fibers is from the cutting edge to the center of corneal flap.

Objective To study the pathogenicity of ureaplasma urealytieum serotype 3 (UU3) with different concentration in the genital tract of the mice. Methods A total of 156 Kunming mice were divided into 4 groups randomly, including group A, B, C (48 mice in every experimental group) and control group.(12 mice in control group). UU3 at concentration of 1×107eopy/g (group A), 1×106copy/g (group B),1×105copy/g (group C) were inoculated into 48 mice in every experimental group intravaginal]y, in the mean time, culture medium of UU was given into 12 mice in control group. They were neeropsied at 1, 3,7, 14, 21, 35 days of postinoeulatien randomly, which included 8 mice of every experimental group and 2 mice of control group every time, and to detect UU3 expression from cervical secretions by FQ-PCR andobserving the pathogenicity rate in tissues of cervix, endometrium, fallopian tube by light microscope and calculate the morbidity rate. Results (1) The total positive rates of UU3 were 63% (30/48) in group A,50% (24/48) in group B, 17% (8/48) in group C, which showed a significant difference(P<0.01).And at 1,3,7,14,21,35 days of postinoculation, the positive rates of group A were 8/8,7/8,6/8,5/8,4/8 and 0,group B were 7/8,5/8,5/8,4/8,3/8 and 0,group C were 3/8,2/8,2/8,1/8,0 and 0;all mice in control group were zero. At all time points, there were statistical difference in the positive rate among three experimental groups only at 1 day (P<0.05 ). (2) In the positive mice, their UU3 quantity concentration at 1,3,7,14,21 days were 1.70×107, 8.26×106, 4.04×106, 2.86×106,and 2.41 x105 copy/g in group A; 3.75×106, 2.56×106 , 1.37×106, 6.72×105, and 1.12 x 105 copy/g in group B, and 1.45×105,1.07×105, 5.43×104, 4.68×103, and 0 copy/g in group C. There were statistical difference among experimental groups at all time points except 21 days (P<0.05). Comparing the concentration among all time points of every group, both group A and B showed a significant difference(P<0.05) ,group C didn't reach it( P>0.05). (3) The total pathogenicity rates of three groups were significant different at 7-35 days, which were 56% (18/32) in group A, 44% (14/32) in group B, 6% (2/32) in group C (P<0.01 ). And at 7,14,21,35 days of postineculation, the pathogenicity rates in group A were 5/8,5/8,4/8 and 4/8, group B were 4/8,4/8,3/8 and 3/8, group C were 1/8,0,1/8 and 0; all mice in control group were zero, which demonstrated significant difference only at 14 days (P<0.05), no other statistical difference were observed (P>0.05) . Conclusions The pathogenicity of UU3 varies with different concentration in genital tract of mice. When UU3 concentration is more than 1×106 copy/g, the susceptibility to infection was intensified significantly.

Objective To study the kinetic expression level of chemokines (RANTESF) in the murine infection of vulvovaginal can-didiasis (VVC), and explore the function of chemokines in local immunity of VVC. Method Sixty-three female Kunming mice, at 8 ～ 10 weeks of age, were used in this study. All animals were divided into three groups. The content variation of RANTESF in blood and yaginal fluids and CFU of vaginal fluid in each separate group of mice were detected at days 2, 7, and 14 after infection. The first group was control group. The second group was infected only one time and the third group was infected twice. The results were analyzed with SPSS 13.0 statis-tical software. Results The content variation of RANTESF and CFU in vaginal fluid reached highest at days 7 in both the first and the sec-ond groups, as well as in the blood. There were no notable changes at days 2 and 14. The content variation in vaginal fluid or blood of the second group was higher than that in the first group after infection. Conclusion CMI, as a host defense mechanism, plays an important role in protecting against vulvovaginal candidiasis, especially in secondary infection. Local innate immunity is more important than systemic in-nate immunity for protection against vulvovaginal candidiasis. Cytokine about RANTES can promote innate immunity modulation; especially the local innate immunity modulation can promote the ehemotaxis of RANTES.

Objective The person's Mesangial Proliferative Glomemlonephritis (MsPGN) were divided into 3 types based on clini- cal manifestations: nephroticsyndrome, hematuria and proteinuria. To investigate the expression of CD11a, CD11b, CD62L and its signifi- cance in the kidneys patients with MsPGN. Methods CD11a, CD11b and CD62L expression in blood of MsPGN patients (n=35) were investigated by flow eytometry method, and the changes of these proteins in kidney were surveyed. Results In MsPGN, CD11a and CD11b expression in blood were significandy lower and CD62L expression in blood were markedly higher than that in normal humans. And the renal glomeruli of MsPGN also expressed CD11a, CD11b and CD62L. Conclusion The expression of CD11a, CD11b and CD62L are abnormal in MsPGN, and apoptosis may play certain role in the pathogenesis of MsPGN.