2.Incomplete P450 17 alpha enzyme deficiency:report of six cases
Qin-Jie TIAN ; Yi-Wen ZHANG ; Zhao-Lin LU ; Gui-Hua SHA ; Hui GAO ;
Chinese Journal of Obstetrics and Gynecology 2000;0(10):-
Objectives To summarize the characteristics,differential diagnosis and management of incomplete 17 alpha-hydroxylase/17,20-1yase deficiency(17 OHD)of Chinese patients.Methods Six cases of incomplete 17 OHD from Peking Union Medical College Hospital were studied retrospectively through analyzing their clinical data,and the molecular pathogenic mechanism was discussed after literature review.Results Four cases of 46,XX incomplete 17 OHD were reported.The clinical characteristics included female phenotype,various degrees of breast development and absent or sparse axillary/pubic hair, oligomenorrhea or secondary amenorrhea,recurrent luteinized ovarian cysts,hypogonadism with persistent hyperprogesteronemia or high serum 17 alpha-hydroxyprogesterone level,with or without hypokalemic hypertension.There were also 2 cases of 46,XY incomplete 17 OHD,in which ambiguous genitalia were present besides hypokalemic hypertension.Conclusions Incomplete 17 OHD is a very rare form of congenital enzymatic deficiencies of steroid synthesis,which should be included in the differential diagnosis when there are menstrual disorders,sexual infantilism,recurrent ovarian cysts or ambiguous genitalia.Under such circumstances,hyperprogesteronemia offers a valuable clue for further investigation.
3.Dynamic change of collagen typeⅠand fibronectin in posterior sclera of form-deprivation myopia after TIMP-2 gene transfect in guinea pig
Lu-qin, WAN ; Gui-xiang, LIU ; Ling, WANG ; Ai-hua, SUI ; Qiang-qiang, GUO ; Yong-zi, LI ; Rui-feng, LI
Chinese Journal of Experimental Ophthalmology 2011;29(7):646-650
Background The domestic and international researches discovered that many proteins and enzymes of the extracellular matrix (ECM) participate in the sclera remodeling by affecting the collagen typeⅠand fibronectin.Objective This study was to investigate the effect of matrixmetalloproteinase-2 (TIMP-2) on expression of collagen typeⅠand fibronectin of ECM in the posterior sclera by injecting liposomes containing tissue inhibitor of TIMP-2 gene into suprachoroidal space of the form-deprivation myopia in guinea pig.Methods Form-deprivation myopia was induced by translucent goggles in 36 clean guinea pig for 2 weeks.Then the animals were randomly assigned to TIMP-2 group,empty plasmid group,saline group and 12 for each group.Liposomes of 5μl containing TIMP-2 gene,empty plasmid and saline were suprachoroidally injected in the right eye respectively,and the left eyes without any treatment were used as self-control group.Other 12 matched guinea pigs only covered the right eyes through out the experimental duration as model control group.The guinea pigs were sacrificed and the posterior sclera tissue of the eyeballs were collected at 2,7 and 14 days after injection of drug.The expressions of collagen typeⅠmRNA and fibronectin mRNA were detected by semi-quantitative reverse transcription polymerase chain reaction (RT-PCR).This study followed the Regulation for the Administration of Affair Concerning Experimental Animals by State Science and Technology Commission.Results The expression level of collagen type Ⅰ mRNA in the posterior sclera of guinea pig was lower but that of fibronectin mRNA was higher in TIMP-2 group than self-control group,showing significant differences between them (P<0.05).The expression level of collagen type Ⅰ mRNA in the posterior scleral tissue began to increase from the 2nd day after drug injection and was obviously elevated at the 7th day and then gradually decreased at the 14th day.However,the expression level of fibronectin mRNA in the posterior scleral tissue showed the opposite pattern.The expression levels of collagen typeⅠmRNA and fibronectin mRNA at the 7th after drug injection were significantly lower than that at the 2nd day or 14th day (P<0.01).Conclusion Suprachoroidal injection of TIMP-2 in form-deprivation myopia could up-regulate the expression of collagen typeⅠmRNA and down-regulate the expression of fibronectin mRNA in the posterior scleral tissue.It may slow down the sclera remodeling of form-deprivation myopia in guinea pig in the early stage.
4.Postoperative intraportal vein antieoagulation in the prevention of portal vein thrombosis in portal hypertensive patients
Li-Hua WANG ; Wei LU ; Gui-Juan SHEN ; Yao-Sheng YU ; Yong-Hua ZHUGE ; Ya-Guo HU ; Xian-Qing WU ; Tian-sheng XU
Chinese Journal of General Surgery 2000;0(12):-
Objective To investigate the clinical significance of intraportal vein anticoagulation for the prevention of portal vein thrombosis after portaazygous devascularization and splenorenal shunt. Methods In this study 67 patients of portal hypertension undergoing surgery were randomly divided into two groups,receiving respectively intraportal vein heparin injection by 100 U?kg~(-1)?d~(-1)?7 d in group A (32 patients)and placebo in group B(35 patients).Portal vein thrombosis,the recurrent bleeding after operation and portal hypertensive gastropathy were compared between the two groups.Results The occurrence of portal vein thrombosis after operation in group A(0)was significantly lower than that in group B(20%,X~2=5.169,P
5.Carcinogenesis of hepatitis C virus core protein using recombinant adenoassociated virus technology.
Hua LI ; Gui-hua CHEN ; Xin-lu WANG ; Fu-xia HAN ; Chen-en PAN
Chinese Journal of Hepatology 2003;11(6):353-357
Animals
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Cell Transformation, Neoplastic
;
Cloning, Molecular
;
Hepacivirus
;
genetics
;
immunology
;
pathogenicity
;
Hepatitis C Antigens
;
toxicity
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Liver Neoplasms, Experimental
;
pathology
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virology
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Male
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Oncogenic Viruses
;
pathogenicity
;
RNA, Messenger
;
toxicity
;
Rats
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Rats, Sprague-Dawley
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Recombinant Fusion Proteins
;
toxicity
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Viral Core Proteins
;
toxicity
;
Virion
;
pathogenicity
6.Efficacy and Safety of Chemotherapy with or without Targeted Therapy in Biliary Tract Cancer: A Meta-analysis of 7 Randomized Controlled Trials
ZHUANG XIN ; XIAO YA-PING ; TAN LING-HUA ; WANG LU-TING ; CAO QIAN ; QU GUI-FANG ; XIAO SHUANG ; DUAN HUA-XIN
Journal of Huazhong University of Science and Technology (Medical Sciences) 2017;37(2):172-178
The systematic treatment based on gemcitabine plus cisplatin is recommended as the current standard chemotherapy for unresectable or metastatic biliary tract cancers.However,the exact benefits from the recognized regime are still dismal.We thus elicit this study in an attempt to analyze whether targeted therapy coupled with various chemotherapy could produce improvement of survival benefits.The clinical trials were searched electronically from databases till July 2016 published in English and Chinese.Nine hundred and sixty-four patients from 7 trials were identified in our analysis.The overall analysis achieved a significantly higher overall response rate (ORR) among the patients treated with targeted drugs plus chemotherapy than chemotherapy alone (OR=1.87;95% CI:1.37-2.57;P=0.000),but failed in the overall progression-free survival (PFS) [mean difference (MD)=0.63;95% CI:-0.45-1.72;P=0.26] and overall survival (OS) (MD=-0.67;95% CI:-2.54-1.20;P=0.49).In the sub analysis,better ORR was obtained with the addition of EGFR (OR=1.75;95% CI:1.20-2.56;P=0.004) and VEGFR (OR=2.5;95% CI:1.28-4.87;P=0.007) targeted therapy.Furthermore,the sub analysis of EGFR target showed an significant improvement on PFS (MD=l.36;95% CI:0.29-2.43;P=0.01).No significant differences were observed in the incidences ofneutropenia (OR=1.37;95% CI:0.89-2.12),thrombocytopenia (OR=l.40;95% CI:0.83-2.39),anemia (OR=l.21;95% CI:0.62-2.38),peripheral neuropathy (OR=1.52;95% CI:0.81-2.88),increased AST/ALT (OR=l.40;95% CI:0.82-2.39) as well as fatigue (OR=1.65;95% CI:0.96-2.84) in either of the treatment groups.In conclusion,better ORR associated with chemotherapy combined with targeted therapy (both targeting EGFR and VEGF) is found in the present mcta-analysis without the cost of increased unacceptable toxicities,but regretfully not for the OS.The sub-analysis of targeting EGFR instead of VEGF obtains a superior PFS.Otherwise,there is no statistically significant difference in the overall PFS between the combination regime and chemotherapy alone.Given the paucity of favorable data,we need further studies to characterize optimal targeted agents to confirm the potential value to biliary tract cancer.
7.Relationship between allergic symptoms and RORC2 and IL-17 in patients with allergic rhinitis
Han-Gui LU ; Hua PENG ; De-Hua CHEN ; Tian-Ying LI ; Jian-Bo SHI
Chinese Journal of Otorhinolaryngology Head and Neck Surgery 2011;46(2):144-148
Objective To evaluate the relationship between allergic symptoms and retinoic acid related orphan receptor variant 2 (RORC2) and interleukin(IL) 17 in patients with allergic rhinitis(AR).Methods Blood sample,nasal secretion and nasal mucosa were taken from 23 patients with AR and 16 health individuals.The expression of RORC2 and IL-17 were detected by immunohistochemistry and quantitative real-time fluorescence reverse polymerase chain reaction.The allergic symptoms in patients were graded.Results The rate of positive cells of RORC2 and IL-17 in AR group were 0.17 ±0.05 and 0.72 ±0.13,higher than the 0.05 ± 0.02 and 0.27 ± 0.11 of health controls,the difference was statistically significant(t were 9.51 and 11.92 respectively,all P <0.05=.The expression level of RORC2 mRNA in nasal mucosa and peripheral blood of AR group were 0.063 ± 0.011 and 0.452 ± 0.031,higher than the 0.029 ±0.009 and 0.239 ±0.027 of health controls,the difference was statistically significant (t were 6.51and 3.35 respectively,all P <0.05=.The concentrations of IL-17 in the nasal mucosa,nasal secretions and serum levels of AR group were (70.28 ± 10.69) ,(45.32 ± 8.55) and (6.76 ± 1.18) pg/ml,compared with (18.43 ± 8.34),(6.83 ± 1.31) and (0.74 ±0.05) pg/ml of controls,the difference was statistically significant(t were 7.92,17.66 and 15.43 respectively,all P < 0.05=.The allergy symptom scores of AR group were 9.43 ± 1.27.There were correlations between the allergic symptom and the expression of RORC2 mRNA and IL-17 in nasal mucosa and peripheral blood (r value were 0.820,0.746,0.629,0.841 respectively,all P < 0.05=.Conclusion RORC2 and IL-17 involved in the inflammatory response of AR and can be used as an indicator to judge the severity.
8.Effects of the HLA antibodies on allograft acute rejection after cadaveric liver transplantation.
Wei CHEN ; Gui-hua CHEN ; Min-qiang LU ; Yang YANG ; Chang-jie CAI ; Bao-hua HOU ; Ye LIN ; Wei-dong ZHANG ; Lu-lu XIAO
Journal of Southern Medical University 2006;26(6):774-776
OBJECTIVETo evaluate the effect of perioperative HLA antibody changes on acute allograft rejection in cadaveric liver transplantation.
METHODSTotally 134 patients received modified piggyback liver transplantation and enzyme-linked immunosorbent assay was performed for HLA antibody detection before and the 1, 7, 14 and 30 days after operation. B ultrasound-guided liver biopsy was employed for diagnosis of acute allograft rejection, and the perioperative changes of HLA antibodies were evaluated for their effect on allograft acute rejection.
RESULTSOf the 44 recipients with preoperative positivity for HLA antibodies, acute rejection occurred in 56.8% of the patients, as compared with 25.9% in those negative for HLA antibody (P=0.001). The patients who became positive for HLA antibody postoperatively had a rate of acute rejection of 60%, which was significantly higher than that in those persistently negative for HLA antibody (18.6%, P=0.003).
CONCLUSIONHLA antibody positivity before transplantation may contribute to acute rejection episode in liver transplantation, and persistent posttransplant HLA antibody positivity is closely associated with the occurrence of acute rejection.
Adult ; Aged ; Antibodies ; blood ; Female ; Graft Rejection ; immunology ; HLA Antigens ; immunology ; Humans ; Isoantibodies ; blood ; Liver Cirrhosis ; surgery ; Liver Neoplasms ; surgery ; Liver Transplantation ; immunology ; methods ; Male ; Middle Aged
9.Expression of ligand-gated cation channels P2X3 receptor in rat pulp during experimental tooth movement.
Yun LU ; Zhi YANG ; Xiao-chuam HUA ; Xin CHANG ; Lin GUI ; Sheng WANG ; Wen-li LAI
West China Journal of Stomatology 2011;29(2):183-186
OBJECTIVETo investigate ligand-gated cation channels P2X3 receptors changes in rat pulp during experimental tooth movement (ETM), and preliminarily find their possible effect during ETM.
METHODS54 male SD rats (200-250 g) were selected and randomly divided into blank group (5 rats), control group (14 rats) and experimental group (35 rats). The left maxillary first molar was selected as observation object, the pulp tissue biopsies was taken at different time points to carry out immunohistochemical study.
RESULTSPredominant up regulation of P2X3 receptors immunoactivity was found in pulp from 1/6 d to 7 d after experimental tooth movement. It started to significantly increase at 1/6 d, peaked at 3 d, and then decreased continuously until 7 d as compared with the beginning.
CONCLUSIONP2X3 receptors have a rhythm change in rat pulp as a result of ETM, speculated that P2X3 receptors is closely related to the tooth movement injury, but the mechanism of action need further researches.
Animals ; Cations ; Dental Pulp ; Male ; Molar ; Rats ; Rats, Sprague-Dawley ; Receptors, Purinergic P2X3 ; Tooth Movement Techniques
10.Prognostic impact of bone marrow involvement (BMI) and therapies in diffuse large B cell lymphoma.
Shu-hua YI ; Yan XU ; De-hui ZOU ; Gang AN ; Yao-zhong ZHAO ; Jun-yuan QI ; Lu-gui QIU
Chinese Journal of Hematology 2009;30(5):307-312
OBJECTIVETo explore the prognostic impact of bone marrow involvement (BMI) and therapy in diffuse large B cell lymphoma (DLBCL).
METHODSThe clinical characteristics and prognosis of 83 DLBCL patients with or without BMI were retrospectively analyzed. The treatment outcome of standard CHOP regimen (CHOP group), intensive-dose regimen (intensive-dose group) and rituximab combined therapy (rituximab group) were compared.
RESULTSThe adverse prognostic factors including LDH elevation, ECOG score > or =2, higher IPI and aaIPI score, B symptom, hepatomegaly, splenomegaly, hemoglobin <110 g/L, platelet <100 x 10(9)/L and serum albumin <35 g/L were more prevalent in DLBCL patients with BMI than in those without BMI. Multivariate analysis showed that BMI was an independent prognostic factor of DLBCL. The 3-year OS and PFS rates in rituximab group were 78.1% and 64.3%, respectively, being statistically higher than that in CHOP group (23.6% and 21.8% respectively, P = 0.000 for both) and in intensive-dose group (33.3% and 25.7% respectively, P = 0.002 and 0.001, respectively). But no difference between the latter two groups (P = 0.411 and 0.694, respectively). For the patients with BMI, the 3-years OS and PFS in rituximab group (57.1% and 57.1%) were statistically higher than that in CHOP group (13.9% and 14.1%) and intensive-dose group (29.5% and 16.8%) (P = 0.029 and 0.012 respectively), respectively and also no difference in the latter two groups (P = 0.226 and 0.376 respectively). In the rituximab group, the 3-years OS and PFS were 86.7% and 67.3% respectively in patients without BMI, being higher than that in patients with BMI (57.1% and 57.1%), but the difference was not statistically significant (P = 0.645 and 0.965 respectively).
CONCLUSIONBMI is a negative independent prognostic factors of DLBCL patients. The rituximab combined chemotherapy can significantly improve the therapeutic effect of the DLBCL, and relieve the negative impact of BMI.
Adolescent ; Adult ; Aged ; Antibodies, Monoclonal, Murine-Derived ; administration & dosage ; Antineoplastic Combined Chemotherapy Protocols ; administration & dosage ; therapeutic use ; Bone Marrow ; pathology ; Child ; Child, Preschool ; Cyclophosphamide ; administration & dosage ; therapeutic use ; Doxorubicin ; administration & dosage ; therapeutic use ; Female ; Humans ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; pathology ; Male ; Middle Aged ; Neoplasm Invasiveness ; Prednisone ; administration & dosage ; therapeutic use ; Prognosis ; Retrospective Studies ; Rituximab ; Treatment Outcome ; Vincristine ; administration & dosage ; therapeutic use ; Young Adult