Inherited metabolic disorder is a major class of clinical diseases with complexity and dififculty of being di-agnosed. Current clinical diagnosis has to rely on the metabolite analysis, gene analysis and enzyme activity (protein function) analysis of these three techniques. We analyse the features of the three diagnostic methods and clinical application of analysis, comparison and induction, to provide some experience and reference that help clinicians to better use of these three techniques. These three ways should be organically combined to form a diagnostic platform for inherited metabolic disorders.

The paper described development characteristics of international hospital accreditation, and challenges faced by China's hospital accreditation.The author proposed for such accreditation,as that accreditation entities are the organizational support for building a permanent mechanism of hospital accreditation,and two organizational modes:the first is societies and associations of capacity to build accreditation organizations and the government to build professional hospital accreditation bodies,and the second is to build the assessors system.

Evans syndrome is a rare autoimmune disease.Clinical presentation are the different degrees of bleeding and hemolysis,similar to immune thrombocytopenia and autoimmune hemolytic anemia.The treatments are divided into first-line therapy,second-line therapy and third-line therapy,including corticosteroids,intravenous immunoglobulin; immunosuppressive agents,splenectomy; haemopoietic stem cell transplantation,romiplostim and so on.The treatment of refractory Evans syndrome is difficult.Immunosuppressive agents and haemopoietic stem cell transplantation are effective.This review focuses on recent developments in the treatment of Evans syndrome.

Objective To study the relationship between the change of resting electrocardiogram ST-T in chronic stable coronary atherosclerotic heart disease(CAD) and left ventricle enjection fraction(LVEF).To know the significance that the change of ST-T may predict the function of left ventricle.Methods 80 patients with CAD in our c ardaic department were analyzed retrospectively,40 patients with the change of ST-T (positive group) and 40 patients without the change of ST-T(negative group) were analyzed the outcome of LVEF and the extent of coronary lesion.Results The LVEF of positive group was lower than negative group obviously,the comparision between both groups had statistical significance (t =6.125,P =0.000).In the electrocardiogram positive group,the number of the patients who had 2 and more than 2 branches lesion was more than negative group,the comparision between both groups had statistical significance(x2 =9.683,P =0.008).Conclusion The change of ST-T may predict the function of left ventricle of patients with chronic stable coronary atherosclerotic heart disease,the relationship may be connected with the degree of coronary lesion.

Objective To investigate the clinical significance of the serum levels of sICAM-1,ET,MCP-1 and APN in patients with acute cerebral infarction(ACI).Methods 120 patients with ACI were selected and divided into different groups according to the size of infarction and clinical neurologic impairment degree score.The serum levels of sICAM-1,ET,MCP-1 and APN were detected by enzyme-linked immunosorbent assay.A healthy control group which approximately matched the experimental group included 30 persons.Results The serum sICAM-1,ET and MCP-1 levels in the patients with ACI were significantly higher than those in the healthy groups (all P ＜ 0.05).The serum levels of sICAM-1,ET and MCP-1 were increased steply with the volume of infarction and the clinical neurologic impairment degree score,the differences between the groups was significant (all P ＜ 0.05).The serum APN levels.In the patients with ACI were significantly lower than that in the healthy groups (P ＜ 0.05).The serum levels of APN were decreased steply with the volume of infarction and the clinical neurologic impairment degree score,the differences between the groups were significant (all P ＜ 0.05).Conclusion The sICAM-1,ET,MCP-1 and APN may play an important role in the pathogenesis of ACI.The serum levels of sICAM-1,ET,MCP-1 and APN are closely correlated with the clinical neurologic impairment degree score and the volume of infarction,which may be of great value in predicting acute cerebral infarction and the severity of cerebral infarction.

Recombinant human growth hormone (rhGH) has been widely used in clinical treatment of growth hormone deficiency(GHD),and has achieved satisfactory effect.Growth hormone (GH) can stimulate liver cells to produce insulin-like growth actor(IGF-1) which mediated GH growth promoting effect.The majority of IGF-1 combine with insulin like growth factor binding protein 3.IGF-I promotes cell mitotic and inhibit cell apoptosis,in recent years,some scholars have reported that there is a certain relationship between IGF-1 and tumor occurrence and development,and thus leading to the attention to the efficacy and safety of rhGH treatment.

Objective To study the changes of the humoral immune parameters in patients after infusion of irradiated platelets and the correlation with platelet transfusion refractoriness(PTR).Methods 126 patients with malignant blood diseases of transfusion in our hospital by more than twice were randomly divided into observation group and control group by randomized single-blind allocation method.The observation group was treated with transfusion note γ irradiated platelets,and the control group was treated with infusion ordinary way of apheresis platelets.Before and after transfusion,the changes of vivo complement(C3,CA),immune globulin (IgG,IgM,IgA),C-reactive protein (CRP) and circulating immune complexes(CIC) were detected andplatelet transfusion efficiency and the correlation between changes of immune parameters and the occurrence of invalid infusion.Results The infusion of platelets in patients of C3,C4,IgG,IgM and CIC of the control group were significantly higher than the observation group(all P ＜0.05).There was no significant changes in IgA of the two groups,but the CRP had the high trends.The infusion efficiency of the observation group was 87.3％,which was significantly higher than that of the control group(61.9％)(χ2 =8.92,P ＜ 0.05),blood transfusion after the PTR immune parameters in patients with C3,C4,IgG,IgM,CRP,CIC significantly higher than before,the difference was statistically significant(all P ＜0.05).Conclusion The bodyimmune parameters with PTR is closely related to humoral immune parameters,and the infusion of γirradiated platelets alleviate to some extent,further improve clinical infusion effective rate.

Sepsis in premature is a major cause of morbidity and mortality.The definition of neonatal sepsis is complicated by the frequent presence of noninfectious conditions that resemble those of sepsis,especially in very low-birth-weight preterm infants.Although growth of an organism from a sterile site is the gold standard for definitive diagnosis,the positive rate is low.Therefore,assessment of history,risk factors,sepsis screening scores in combination with diagnostic tests are used to identify neonates who are more likely to be infected.Methods studied to prevent infection include improved hand hygiene,early feedings,immune globulin administration,prophylactic antimicrobial administration.It improves prognosis for early diagnosis and antibiotic administration.

Objective To observe the effect of gastroscopy injection of norepinephrine combined with routine triple therapy on peptic ulcer with gastrointestinal bleeding. Methods 84 cases of gastroscopy upper gastrointestinal bleeding caused by peptic ulcer patients were randomly divided into two groups, the control group 42 cases;the observation group 42 cases, the observation group was guven endoscopic injection of norepinephrine combined conventional triple therapy,the control group was given conventional triple therapy with hemostatic therapy. Efficiency and adverse reactions were observed in patients. Results In the observation group,36 csses was significantly effective,5 cases was effective,1 case was not effective,the total effective rate was 97.6%. In the control group,21 cases was significantly effective, 16 cases was effective ,5 cases was not effective,the total effective rate was 88. 1% ,the difference of the total effective rate between the two groups was significant( x2 =5. 137, P ＜ 0. 05 ). There was no serious adverse reactions in the two groups. Conclusion Endoscopic injection of norepinephrine and routine triple therapy in treatment of gastrointestinal hemorrhage of peptic ulcer was significantly better than conventional medical treatment alone.

Objective To comparatively analyze the results of the PT derivative method and the Clauss method for detecting fi-brinogen(Fib) by the automated coagulation analyzer and to explore their accuracy and clinical value .Methods Fib was detected in 156 blood samples selected from the patients in this hospital from March to May 2013 ,25 normal control samples and 13 positive samples of D-dimer (DD) by the TP derivative method and the Clauss method .Results The Clauss method and the PT derivative method by Sysmex CA-1500 coagulation analyzer had good repetitive rate in measuring Fib for 25 times ;when Fib<1 .0 g/L ,the results detected by these two methods had statistically significant difference (P<0 .05);when Fib in the range of 1 .0 -2 .0 g/L , 2 .1-3 .0 g/L ,3 .1-4 .0 g/L ,the detection results had no statistically significant difference between the two methods (P>0 .05);when Fib in the range of 4 .1 -5 .0 g/L ,the detection results had statistically significant difference (P<0 .05);when Fib>5 .0 g/L ,the detection results had statistically significant difference (P<0 .05);in 13 DD positive samples ,the detection results of the PT derivative method were significantly higher than those of the Clauss method with statistically significant difference (t=8 .530 , P<0 .05) .Conclusion The difference exists between the PT derivative method and the Clauss method for detecting Fib ,but the ac-curacy of the Clauss method is higher than its specificity ,the Clauss method should be the first choice for the clinical determination of plasma Fib .