Wiskott-Aldrich syndrome (WAS) is due to the mutation of the WAS gene on the X chromosome,which has both features of primary immunodeficiency disease (PID) and congenital hereditary hematopoietic disease.Although the clinical manifestations of classic WAS were more obvious,the related gene (WAS) and its protein product (WASP) have the value of diagnosis,but due to the low incidence,and there were significant differences in the severity of clinical symptoms of the patients,so WAS is easily misdiagnosed.This article through collecting and analyzing the recent years of research progress of literature data and the clinical reports,and combined with the author＇s previous experience in the diagnosis and treatment,to induct the main points of diagnosis and treatment for WAS.

The incidence of children with iron deficiency (ID) and iron deficiency anemia (IDA) is still higher in ourcountry now, but the relevant prevention and treatment still lag behind. This paper provides a brief summary of the importanceand impact of childhood ID and IDA prevention and treatment practice based on the recent literatures andThe recommendationsof prevention and treatment for children with iron deficiency and iron-deficiency anemia recommended by the Pediatric HematologyGroup recently.

Function experiment is an important part of the basical medical science experiment.With the reform and construction of the function laboratory,a kind of new experiment teaching system is set up to improve laboratory utilization,promote the experiment effect and turn a student into an expert in medical science.

Objective To explore the effect of Wendan Decoction plus Saffron in the treatment of patients with endometriosis of uterus on serum matrix metalloproteninases-9(MMP-9).Methods 74 cases of endometriosis of uterus were selected and randomly divided into two groups, 37 cases in each group.The control group were treated with oral administration of Gestrinone Capsules, the research group were treated on the basis of the control group with Wendan Decoction plus Saffron treatment,two groups were treated for 24 weeks.The serum estradiol, MMP-9 and matrix metalloproteninases inhibitor-1(TIMP-1) were detected before and after treatment, the degree of pain and clinical efficacy were compared.Results Compared with before treatment, levels of estradiol(E2),progesterone(P) and luteotropic hormone(LH) decreased in two groups(P<0.01), the levels of MMP-9, MMP-9/TIMP-1 decreased(P<0.01), levels of TIMP-1 increased(P<0.01), levels of CA125 decreased(P <0.01), and the maximum diameter of pelvic mass line decreased(P<0.01), dysmenorrhea pelvic pain, dyspareunia and VAS score decreased(P<0.01).Compared with the control group, levels of E2, P and LH in the research group were lower(P<0.01), the levels of MMP-9, MMP-9/TIMP-1 were lower(P<0.01), levels of TIMP-1 were higher(P<0.01), levels of CA125 were lower(P<0.01), the maximum diameter of pelvic mass line were lower(P<0.01), dysmenorrhea and pelvic pain and VAS scores were lower(P<0.01), and the effective rate of research group was higher(P<0.05).Conclusion Wendan Decoction plus Saffron in the treatment of endometriosis of uterus was effective, and it can reduce endometriosis, dysmenorrhea and pelvic pain degree, reduce pelvic mass, and may reduce the level of serum MMP-9 related.

ObjectivesTo summarize the application ofGuidelines for the Management of Community-Acquired Pneu-monia (CAP) (2013 edition) in the diagnosis and treatment of children with CAP.MethodsThe clinical features of 209 children with non-severe CAP at different age stages were retrospectively analyzed. The curative effect of azythromycin was summarized which was used as ifrst-line antibiotics in the CAP treatment.ResultsIn 209 CAP children, the onset of 94 children was occurred in spring and summer and 115 occurred in autumn and winter. The white blood cell counts (WBC) in 51 CAP children (24.4%) were more than 10.0×109/L and absolute neutrophil counts (ANC) in 91 CAP children (43.5%) were more than 5.0×109/L. C-reactive protein levels in 199 CAP children (95.7%) were increased and MP-IgM showed positive in 94 CAP children (45.0%). 173 CAP children (82.8%) presented unilateral inlfammatory lesions in chest X-ray, and the right side was predominant. There was no difference in onset season among different age groups (P>0.05). However, the percentage of WBC≥10×109/L among different age groups were signiifcantly different (P=0.002) and the percentage of WBC≥10×109/L was highest in infants and young children group. The proportion of onset in spring and summer was signiifcantly higher in MP-IgM positive group than that in MP-IgM negative group (P<0.01). The total effective rate of azithromycin used as ifrst-line antibiotics was 98.1% (205/209), and there was no signiifcant difference in total effective rate of azithromycin between MP and non-MP pneumonia groups.Con-clusionsThe Guidelines for the Management of CAP (2013 edition) have a reference value in the diagnosis and treatment of CAP in our region where the CAP in children has some different features.

Objective To improve the early diagnosis of Wiskott-Aldrich syndrome (WAS),through summing up and analyzing the clinical characteristics of children with WAS.Methods The clinical and laboratory characteristics of 18 children with WAS,including the cases from Suzhou Jiulong Hospital,Shanghai Jiaotong University School of Medicine,and Tongji Hospital Affiliated to Tongji University,and the domestic reports in recent 6 years,were induced and analyzed.Results All patients were males;the average of incidence age was about 1.9 months and the average age at diagnosis was 10.8 months.All cases had the clinical characteristics of WAS including eczema,infection tendency and thrombocytopenia with small platelet size.Moreover,10/18 cases (55.56％) WAS patients progressed to severe cases (scores reached to 4 or 5 points)at diagnosis,and with the increase of age of the patients at diagnosis,the ratio of severe cases would be increased.The conventional immune function indexes showed no specific diagnostic value,and the typical clinical features and the WAS gene mutation detection were the key diagnostic basis.Seven cases used to be misdiagnosed as idiopathic thrombocytopenia,and received corticosteroids and intravenous immunoglobulin therapy.In a total of 18 cases,4 cases received allogeneic hematopoietic stem cell transplantation,of which,β cases were cured and 1 case died of transplantation-related interstitial pneumonia,while the remaining 14 cases are unknown for their with follow-up treatment and prognosis.Conclusions The typical clinical features and the WAS gene mutation detection were the key diagnostic basis.In order to improve the rate of early diagnosis and avoid misdiagnosis,it's a great need to improve the clinical understanding of WAS.

Objective To transform the equipment with traditional RS232 interface into that with CAN connection interface.Methods On the CAN main line,node is the station for information receiving and transmission in the network.The intelligent node can establish the work way ID,the address and the baud rate through the programming.It is mainly composed by the singlechip and the programmable CAN correspondence controller.Results The hardware and software were designed.SJA1000 initialization,transmission and receiving subroutines are including in the software design.Conclusion CAN is a kind of excellent field bus with high reliability,low cost and has been applied in many fields.It has already formed the international standard and been viewed as one of the most widely used field buses in future.Lots of traditional equipment with RS232 interface are facing the problem of CAN interface transformation.

AIM: To investigate the effect of activation of JAK/STAT signaling pathway on the transdifferentiation induced by high concentration of glucose in renal proximal tubular epithelial cells(HKCs).METHODS: Cultured HKCs cells were divided into four groups: low glucose group(LG),high glucose group(HG),high mannitol group(LG+M),and HG+AG490 group(AG).Immunoprecipitation and Western blotting analysis were used to determine the expression of tryosine phosphorylated Janus kinase 2(p-JAK2).The protein expressions of STAT1,STAT3,p-STAT1 and p-STAT3 and expressions of ?-SMA,E-cadherin were observed by Western blotting.The contents of TGF-?1 and type I collagen in the supernatants of the cultured HKCs were detected by enzyme-linked immunoadsorbent assay(ELISA).TGF-?1 mRNA were measured by reverse transcription and polymerase chain reaction(RT-PCR).RESULTS: Compared with low glucose control group,the expression of JAK2,p-STAT1,p-STAT3 and TGF-?1 mRNA was significantly increased in HG group at different stimulated time from 6 h to 72 h.Meanwhile,the contents of TGF-?1 and collagen I in the supernatants and the expression of ?-SMA were increased,the expression of E-cadherin were decreased.The expression of JAK2,p-STAT1,p-STAT3 and TGF-?1 mRNA and the levels of TGF-?1,collagen I in the supernatants in HG+AG490 group were obviously lower than those in HG group.The expressions of ?-SMA and E-cadherin were also decreased in HG+AG490 group.CONCLUSION: Activation of JAK/STAT signaling pathway may be involved in the high glucose-induced transdifferentiation and overproduction of TGF?1 and ECM proteins in HKCs.

Objective To avoid complicated wiring, a ward calling system based on wireless protocol is studied. Methods The system adopted ZigBee protocol, composed of calling console as network coordinator, bed-extensions as terminals and network routers in each ward. Results After initial configuration, the system could ring an alarm and gave the patient's bed number and ward number when patients called. Also it provided the automated network faults detection system. Conclusion The system is characterized with convenient installation, high intelligence and easy expansibility, and accords with the trend of technology.

Objective To investigate the effect of high glucose on the expression of transforming growth factor-?1(TGF-?1),?-SMA,E-Cadherin in renal proximal tubular epithelial cells(HKCs)and role of AG490,an antagonist of Janus kinase.Methods Cultured HKCs cells were divided into four groups:low glucose group(LG),high glucose group(HG),high mannitol group(LG+M),and HG+AG490 group(AG).Immunoprecipitation and Western blot analysis were used to measure the expressions of tryosine phosphorylated Janus kinase 2(p-JAK2),STAT1,STAT3,p-STAT1,p-STAT3 and ?-SMA,E-Cadherin.The contents of TGF-?1 and type I collagen in the supernatants of the cultured HKCs were detected by ELISA.TGF-?1 mRNA were measured by RT-PCR.ResultsCompared with low glucose control group,the expressions of JAK2,p-STAT1,p-STAT3 and TGF-?1 mRNA were significantly increased in HG group from 6 to 72 h.Meanwhile,the contents of TGF-?1 and collagen I in the supernatants and the expression of ?-SMA were increased,the expression of E-Cadherin was decreased.The expression of JAK2,p-STAT1,p-STAT3 and TGF-?1 mRNA and the level of TGF-?1,collagen I in the supernatant s in HG+AG490 group were obviously lower than those of the HG group.The expression of ?-SMA and E-Cadherinwas also decreased in HG+AG490 group.Conclusion Activation of JAK/STAT signaling pathway seems to be involved in the high glucos induced overproduction of TGF-?1 and ECM proteins in HKCs.