Objective To study the pathogenesis, clinical characteristics, laboratory tests, treatments and prognosis of con-genital factorⅦdeficiency. Methods The clinical data of two cases of congenital factorⅦdeficiency diagnosed at the Chil-dren’s Hospital of Fudan University and 9 cases reported in the past 10 years retrieved from Pubmed, Web of Knowledge and CNKI, Wangfang database by using the factorⅦdeficiency , congenital, newborn and case report as keyword were reviewed and analyzed. Results All cases were full term birth with normal birth weight (>2 500 g), including 4 females and 7 males. Pa-rental consanguinity was found in 3 cases, and a family history was found in 3 cases. The laboratory tests were characterized by significantly prolonged prothrombin time, normal partial thromboplastin time, and decreased coagulation factorⅦactivity. The coagulation factorⅦactivity of 10 cases were less than 5%. Five cases (45.5%) were treated with human recombinant activated factorⅦ. Four cases (36.4%) treated with plasma or human recombinant activated factorⅦare currently in normal growth and development. Four cases (36.4%) died during the hospitalization. Conclusions A diagnosis of congenital factorⅦdeficiency should be considered in the neonates with severe bleeding, prolonged prothrombin time, normal partial thromboplastin time, and being intractable to vitamin K treatment. Human recombinant activated factorⅦis the first choice of the treatment of congenital factorⅦdeficiency. The further study of gene mutation type will be of great significance for disease screening, diagnosis, treat-ment and prognosis prediction.

Objective To explore the best anticoagulant ratio in SD rats .To analyse the influence of insufficient blood specimens volume on coagulation tests .Methods 60 rats were divided into 2 groups.According to the method of vacuum blood, collect abdominal aortic blood after fasting 12 hours.The first group 20 rats were used only for routine blood test.Fully automatic hematology analyzer detected hematocrit and platelet .The second group 40 rats were used for coagulation test .Every rat was collected 2 blood specimens with different anticoagulant ratio [ the proportion of sodium citrate anticoagulation and the whole blood (vlume∶volume)]1∶9(the control group)and 1∶5( the experimental group),1∶8( the experimental group) and 1∶7( the experimental group).Get plasma without platelet through centrifugation.Fully automatic blood coagulation analyzer detected prothrombin time , activated partial thromboplatin time , thrombin time and Fibrinogen.Results HCT (%) and PLT (x 109/L) in SD rats were respectively 41.7 ±2.9 and 1114 ±173.As anticoagulant ratio was increased ,PT, APTT and TT were extended and FIB was decreased .Compared with the control group ,these PT,APTT,TT,FIB four results of 1∶8 group were not statistically different , of 1∶5 group were statistically different, these PT,APTT,FIB three results of 1∶7 group were not statistically different , TT of 1∶7 group was statistically different.Conclusions The detection of coagulation project coagulation test results were affected by the proportion of anticoagulant and blood .1∶9 was the best anticoagulant ratio in SD rats , 1∶8 can also.Rat had its unique physiological characteristics .The results could provide reference for the evaluation of rats .

Objective To study the clinical value of combined detection of serum interleukin(IL)-8 ,tumor necrosis factor(TNF)-α,alvedar cell surface antigen Ⅱ(KL-6) and surface protein D(SP-D) in the diagnosis of idiopathic pulmonary fibrosis(IPF) .Meth-ods Seventy three patients with IPF were selected as the research subjects ,other 73 patients with bacterial pneumonia were taken as the bacterial pneumonia group .The levels of serum IL-8 ,TNF-α,KL-6 and SP-D were detected by enzyme-linked immunosorbent assay (ELISA) .The serum levels of IL-8 ,TNF-α,KL-6 and SP-D were compared between the IPF group and bacterial pneumonia group .The sensitivity and specificity of IPF detection were compared between the 4-index combined detection and single item de-tection .Results The levels of IL-8 ,TNF-α,KL-6 and SP-D in the IPF group were significantly higher than those in the bacterial pneumonia group (P<0 .05) .The positive rate of single detection of four indexes in the IPF group was significantly higher than that in the bacterial pneumonia group (P<0 .05) .The sensitivity and specificity of the 4-index combined detection for diagnosing IPF were 90 .4% and 93 .2% respectively ,which were significantly higher than the those of single index detection (P<0 .05) .Con-clusion The combined detection of IL-8 ,TNF-α,KL-6 and SP-D has better sensitivity and specificity in IPF diagnosis compared with single detection of IL-8 ,TNF-α,KL-6 and SP-D .

Objective To report our experience of interventional procedure for arterial complications in post renal transplantation and to evaluate its clinical value.Methods In a retrospective analysis of renal transplantations in our center,52 cases of renal allograft artery abnormalities had taken angiography.Interventional procedure included transluminal angioplasty of arterial stenoses,treatment of arterial occlusion,and embolization of pseudoaneurysm.Results Renal allograft artery abnormalities included artery stenosis (n=21),artery thrombosis (n=13) and embolision (n=1),renal artery pseudoaneurysms (n=2),and decrease of renal artery flow (n=3).Of the 21 artery stenosis,2 grafts with artery stenosis were lost because the stenosis could not be corrected,and 3 with mild stenosis received no treatment.Another 16 accepted renal artery angioplasty (balloon dilation,n=12,and stent implantation,n=4).14 achieved long-term allograft function.1 graft was lost because renal function failed to recover.Restenosis occurred in one stent implantation,and lost the allograft function after secondary dilation.13 cases received thrombolytic therapy through artery catheter for thrombosis and 9 achieved long-term allograft function.Thrombolyses failed in 3 cases,and renal function failed to recover in 1 case.One pseudoaneurysm received stent implantation after embolization,and got a short-term allograft function.The other one received allograft excision.Conclusion Intravascular interventional therapy will be the first-line therapy for any indications of complication in post renal transplantation,and it can surely save the kidney in a majority of instances.

The gene encoding thermostable lactate dehydrogenase (Tm-LDH) was cloned into the plasmid pHsh from Thermotoga maritima, and expressed in Escherichia coli JM 109. The recombinant protein was purified to homogeneity by a simple step, heat treatment. The recombinant enzyme had a molecular mass of 33 kDa. The optimal temperature and pH of Tm-LDH were observed 95 degrees C and 7.0. The purified enzyme had a half-life of 2 h at 90 degrees C, and exhibited better stability over a pH range from 5.5 to 8.0. The K(m) and V(max) values were 1.7 mmol/L, 3.8 x 10(4) U/mg of protein for pyruvate, and 7.2 mmol/L and 1.1 x 10(5) U/mg for NADH, respectively. The expression of Tm-LDH in T7 system could not obtain high efficiency, but it has been soluble over-expression in pHsh system and reached 340 mg/L. The superior stability and productivity of Tm-LDH will lay the foundation of its industrial-scale fermentation and application in the NAD regeneration.
Cloning, Molecular ; Enzyme Stability ; Escherichia coli ; metabolism ; L-Lactate Dehydrogenase ; biosynthesis ; Molecular Weight ; Recombinant Proteins ; biosynthesis ; Temperature ; Thermotoga maritima ; enzymology

One hundred patients were diagnosed with coronary heart disease through dual-source CT coronary angiography (DSCTA) in our hospital,including 53 cases aged ≥60 y (elderly group) and 47 cases ＜60 y (middle-aged group).The curved-planar reconstruction (CPR),maximal intensity projection (MIP) and other post-processing reconstruction techniques were used to evaluate the indicators and differences of coronary stenosis ≥ 50％ ; the X-ray coronary angiography (CAG) findings were regarded as the gold standard.The evaluable rates of coronary arteries through DSCTA were 97.0％ and 98.6％ for two groups (P ＞0.05).The positive rate of coronary artery stenosis ≥ 50％ in elderly group was higher than that of middle-age group ( 12.6％ vs 9.0％ P ＜0.05 ).The rates of multi-vessel disease in elderly group (28.3％ ) was also significantly higher than that of middle-age group (6.4％,P ＜0.05 ).The sensitivity,specificity,positive predictive value and negative predictive value in evaluation of coronary artery stenosis ≥ 50％ for elderly patients were 92.3％,98.0％,88.0％ and 98.9％ respectively; those for middle-aged patients were 96.0％,99.0％,90.6％ and 99.6％ respectively ( P ＞0.05 ).DSCTA imaging provides an effective screening and evaluation method for coronary heart disease in elderly and middle-aged patients.

Objective To improve clinicians'understanding of nephrotic syndrome and myasthenia gravis as adverse events of penicillamine(D-PA).Methods Two patients were reported to develope adverse reaction after taking D-PA.The related literature were reviewed.Result The first case was a systemic sclero- sis(SSc)patient treated with D-PA who developed nephrotic syndrome and blepharoptosis.The second case was a rheumatoid arthritis patient treated with D-PA who developed dysphagia and drinking bucking.The clinical symptoms were improved after disconting D-PA.Their conditions were stable up to now.Conclusion D-PA is used widely for various of autoimmune diseases.Clinicians should pay attention to these special and rare adverse effects.

Objective To investigate and compare the effect of orally or intranasally administered borneol on ketamine‐mid‐azolam anesthesia anesthetized rats′regained consciousness and cognitive functions .Methods The rats were trained to escape laten‐cy and divided into 6 groups:control group ,model group ,low dose oral borneol group ,high dose group oral borneol group ,low dose intranasal borneol group and high dose intranasal borneol group ,16 rats in each group .All groups were given general anesthesia ,be‐side the control group .The reflex recovery time ,blood gas analysis ,escape latency and space exploration capability were recorded and compared within groups .Results Compared with the control group ,PaCO2 ,P(A‐a)DO2 and latent escape time increased (P<0 .05) ,pH ,PaO2 and times of platform across decreased in the model group (P<0 .05) .Compared with the model group ,the righ‐ting reflex test time ,times of platform across and latent escape time decreased (P<0 .05) ,blood gas analysis improved (P<0 .05) in intranasal borneol groups and oral borneol groups .The righting reflex test time ,blood gas analysis and times of platform across had no significantly differences within borneol oral groups(P> 0 .05) .Compared with low dose oral borneol group ,latent escape time decreased in high dose oral borneol group (P<0 .05) .Compared with borneol oral groups ,the righting reflex test time ,times of platform across and latent escape time decreased (P< 0 .05) ,blood gas analysis improved (P< 0 .05) in intranasal borneol groups .Compared with low dose intranasal borneol group ,the righting reflex test time and latent escape time decreased (P<0 .05) , blood gas analysis improved (P<0 .05) in high dose intranasal borneol group .Conclusion Intranasal borneol could reduce righting reflex test time and improve cognitive function by improving the blood gas analysis .

ObjectiveTo evaluate clinical significance of serum free light chains (sFLC) in diagnosis and response to the therapies of patients with multiple myeloma (MM).MethodssFLC (κ,λ and κ / λ ratio)were examed by immumoassay from 62 patients with MM at different stage. The results were analyzed associated with clinical data,and 35 cases of chronic renal failure(CRF)patients and 62 cases of healthy donors were taken as controls.ResultsMedium sFLC of normal κ value was (13.25±6.46) mg/L,λ value was (18.39±11.42) mg/L; and κ / λ ratio was (0.97±0.64) mg/L (range 0.33-1.61).sFLC κ and λ of CRF patients were (200.01±299.87) mg/L,(191.02±245.98) mg/L,significantly higher than that of the normal control group (t =-17.804,-16.894,both P ＜ 0.001),but the κ/λ ratio was at normal range (1.11±0.29).κ value range was at 16.20- 35 250 mg/L in newly diagnosed intact immunoglobulin MM patients with IgGκ,IgAκ and IgDκ type.The range of λ values was 15.70-4885 mg/L in IgGλ,IgAλ,IgDλ type,and κ/λ ratio was abnormal in 96.5 ％ (55/57) patients (＜0.5 or ＞1.5).The κ,λ value and κ/λ ratio were close to that of the normal after remmision.ConclusionsFLC ( κ,λ,and κ / λ ratio) are very good monitoring markers for MM.

Objective To investigate the correlation between relative cerebral blood volume (rCBV) and tumor character of meningiomas. Methods Thirty-six (GradeⅠ: n=30, GradeⅡ+Ⅲ: n=6) patients with meningioma underwent conventional MR and perfusion weighted imaging (PWI) . The pulse sequence of PWI was single shot GRE-EPI-T2~*WI. The CBV maps were calculated from the original data of perfusion images and the maximum rCBV of meningiomas was acquired from CBV maps through measurement on the region of interest (ROI). The differences of rCBV in tumor and edema between benign and malignant tumors were analyzed. Results The mean rCBV in tumor of benign meningioma (9.78±4.69) was higher than that of malignant ones (3.59±0.28) (t=7.168, P<0.001). The mean rCBV in edema of benign and malignant meningiomas was 3.06±1.76 and 3.58±1.93, respectively (P>0.05). Conclusion CBV map and quantitative study of rCBV are feasible for differential diagnosis of meningiomas and have instructive function for clinical treatment and assessment of prognosis.