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 explore the influence of human resources management in emergency department on nurse's anxiety.Methods Self-Rating Anxiety Scale(SAS)was used to investigate 300 nurses in the emergency department in nine secondary level hospitals of Longgang District.Division of human resource management in these hospitals were examined and the correlation between them was statistically analyzed.Results In addition to the three hospitals,nurses anxiety status of the other six hospitals score showed different levels of anxiety,the average scores were above 50,but the hospitals,that the ratio of the three anxiety condition score was less than 50,moderate to severe anxiety were still more than 20.0％,and other six hospitals was up to 66.7％.In the human resource management to achieve excellent and qualified hospital,the anxiety rate was significantly lower than the substandard hospitals.At the same time there was close correlation between the situation of human resource management and nurses anxiety situation.Conclusions Emergency nurses generally have high anxiety,and in hospitals with poor human resource management,career anxiety is even more obvious.That is to say the anxiety of nurses is closely related to emergency department management of human resources.

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 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.

Objective To investigate the efficacy of contrast-enhanced ultrasound (CEUS) in diagnosing liver, gallbladder, spleen and renal diseases. Methods 27 patients with liver diseases, 30 patients with gallbladder diseases, 5 patients with renal diseases, 5 patients with renal trauma and 6 patients with spleen disease were examined by CEUS. Results There were 3 cases of primary hepatocellular carcinomas, 4 cases of metastatic hepatic carcinomas, 4 cases of liver abscess, 10 cases of hepatic hemangiomas and 3 cases of liver repture with active bleeding. The diagnostic coincidence rate of CEUS was 90%. 30 patients with gallbladder polyposis were diagnosed by CEUS, and the diagnostic coincidence rate was 100%. 5 cases with renal diaseses included 1 of renal abscess, 1 of renal column hypertrophy and 3 of renal hemangioma. In 5 cases with renal trauma, there were 3 cases with renal contusion and 2 cases with renal rupture and active bleeding. The coincidence rate was 61%. In 6 cases with spleen diseases, there were 1 case with splenic infraction, 1 case with splenic abscess and 4 cases with splenic rupture and hemorrhage. The coincidence rate was 100%. Conclusion CEUS has great value of clinical application in diagnosis of liver, gallbladder, spleen and renal diseases.

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 establish an HPLC method for the simultaneous determination of two ingredients in indometacin and furazolidone suppositories.Methods: The analysis was performed on an XTerra(R) RP18 column (250 mm×4.6 mm, 5 μm) with gradient elution of methanol and 0.01 mol·L-1 potassium dihydrogen phosphate-triethylamine (100∶0.02) at the flow rate of 1.0 ml·min-1.The column temperature was 30 ℃ and the detection wavelength was 263 nm.The injection volume was 10 μl.Results: The peaks of furazolidone and indometacin were successfully separated.The linear range of calibration curves was 5.12-81.87 μg·ml-1 (r =1.0000) and 3.78-60.45 μg·ml-1 (r =1.0000), respectively.The average recovey was 99.6% (RSD =1.5%, n =6) and 100.3% (RSD =1.6%, n =6), respectively.The limit of quantification (LOQ) was 0.02 μg·ml-1 and 0.05 μg·ml-1, respectively.Conclusion: The established method is validated to be suitable for the quality control of indometacin and furazolidone suppositories.