Objective To detect the express of the pulmonary fibrosis factor CTGF,TGF-β1 and the senescence correlated β-Gal in the primary pulmonary bulla,and investigate the correlation of the pulmonary fibrosis factor,cell senescence and the development of the primary pulmonary bulla.Methods The expression of CTGF,TGF-β1 and β-Gal protein in the tissue of normal lung tissues and lung bullae were tested.The cell image extracted with the digital camera system was entered into the Image-pro Plus 6.0 morphology Image analysis system and analyzed with Semi-quantitative way.Results The expression level of TGF-β1 and CTGF in primary pulmonary bulla organization was obviously higher than that of normal lung tissue.There was a statistically significant difference(P ＜ 0.05).CTGF and TGF-β1 expression level had a significant correlation (r =0.965,P ＜ 0.01).β-Gal expression level of primary pulmonary bullae had no obvious difference with normal lung tissue.Conclusion CTGF and TGF-β1 may play an important role in the formation of primary pulmonary bulla.Both play a synergistic role in the formation of primary pulmonary bulla.Cell senescence is not relevanted with the formation of primary pulmonary bulla.

Objective To detect the mutations of transglutaminase 1 (TGM1) gene in a family with lamellar ichthyosis. Methods The genomic DNA was extracted from the proband and his family members. All the encoding exons and adjacent splice sites of TGM1 gene were amplified by PCR. Mutation scanning was carried out via direct bi-directional DNA sequencing. Also the homology of TGM1 was analyzed. Results In the proband, there was a C504T mutation located at codon 142 (R142C) in exon 3 of TGM1 gene, and a nonsense mutation of C1122T located in exon 7, which caused a premature termination of R348X and a defective polypeptide truncated by 470 amino acids in C-terminus. A heterozygote of C504T mutation was carried by the proband′s father and a heterozygote of C1122T mutation in the proband′s mother. The missense mutation of R142C was found at the conservation region of TGM1 gene. Conclusion The mutations of R142C and R348X in TGM1 gene are present in the patient with lamellar ichthyosis.

Objective To investigate the dynamic changes and coordination of macrocirculation and microcirculation in rats with severe hemorrhagic shock and septic shock. Methods A total of 20 male Sprague-Dawley rats (450～550 g) were randomly divided into two groups (n = 10) : hemorrhagic shock group (H-Shock) and septic shock group (S-Shock). 40％ of the systemic blood volume was withdrawn in 1 hour in H-Shock group.While in the S-Shock group, cereal ligation and puncture (CLP) was performed and then the abdomen was closed.Mean arterial pressure (MAP) and the end-tidal carbon dioxide (ETCO2) were recorded continuously. Cardiac ultrasonography and sublingual microcirculation were performed per hour till 12 hours or the rat die (End). A total of 12 hours were observed or the rat die. Arterial blood gas was measured at baseline (BL) , MAP ≤ 65 mmHg (Shock) and at the termination of observation respectively. Results The values reflect macrocirculation or microcirculation in the H-Shock group were all decreased than BL respectively at each time. In the S-Shock group, the values that reflect macrocirculation such as MAP, ETC02 and cardiac output (CO) showed no significant changes within 3 hours (P> 0.05) , while the values of microcirculation were decreased compared with BL respectively at each time (P < 0.05). With the progress of shock, the pH was decreased compared with BL in both groups, while the level of Lac were increased (P < 0.05). Conclusions The changes of macrocirculation and microcirculation in severe hemorrhagic shock are basically the same; while they are inconsistent in septic shock, the changes of microcirculation occurred earlier than that of macrocirculation.

To explore how to create and optimize a promotion index system of medical quality evaluation, this article focuses on the hospital visiting process from patients, using analyzing collected those index system from couples of Grade Ⅲ hospitals in Beijing, and combining the results of literal study, field study and specialist consult, according to the different situation of general hospitals and specially hospitals, with the spirit of "maintaining the patients benefits, safeguarding the patients safety,and enhancing the medical quality", introduces the framework of the promotion index system, the rules to select the indicator, and so on, and discusses several problerns related to creating the index system.

To analyze respectively a case,presented with recurrent petechial and epistaxis after a 2 years history of diabetes mellitus (DM),who was hospitalized in Beijing Children's Hospital Affiliated to Capital Medical University.The clinical manifestation,examination,diagnosis and treatment were recorded.The patient was diagnosed with immune thrombocytopenia (ITP) and DM at the first admission.The initial therapy with gamma globulin didn't show ideal effect.The pediatric specialists from the department of ENT,Hematology/Oncology,Endocrinology,Pharmacy and Immunodeficiency Clinic were invited to discuss the case.The final diagnosis of autoimmune polyglandular syndrome (APS) was made and supplementary steroid treatment was started.But the response of the steroid therapy was poor.Once again with the multidisciplinary consultation,the patient received several schemes of Rituximab under the informed consent.This treatment reached a stable condition for almost 7 years.APS should be considered when DM patient showed the manifestation of other immune organ damages.Rituximab immunosuppressive therapy should be tried when the response to first-line treatment was poor.