Perioperative blood transfusion therapy for coagulation factor deficiency in children is very important to save patient's life or conducive to the postoperative recovery.With the advances in transfusion medicine,the cooperation through pediatric hematologists and surgeons in the perioperative period,more and more operation of children accompany coagulation factor deficiency can be safety finished.Different types of coagulation factor deficiency in children are treated by high-purity clotting factor,in order to correct abnormal coagulation factors levels.Clinical data showed that coagulation factor deficiency in children after proper treatment can be safely performed surgical procedure.

Objective To observe the morphologic changes of of vascular buds in vertebral cartilage endplate in age-specific rabbits and also to investigate the correlation between the changes of vascular buds and interverbral disc degeneration. Methods There were 15 New Zealand white rabbits in our study,which include three groups, 2-week-old rabbits, 1-year-old rabbits and 3-year-old rabbits, and each groups had five rabbits. The X-ray radiograph, histology and scanning electron microscope were used to observe the changes of vertebral cartilage endplate. According to Miyamoto standard, the interverbral disc was graded 1-5, and scored 1-5 respectively. Results The changes of micro-vascular structure of vertebral cartilage endplate were observed during aging. Under the scanning electron microscope, the vascular structure degenerated gradually, and disappeared in the end. The blood vessels in the central region of the vertebral cartilage endplate reduced more obviously than those in periphery region. The severe degeneration was found in vertebral endplate, compared with intervertebral disc. The changes of vascular buds in rabbits vertebral cartilage endplate had positive correlation with the vertebral endplate calcification and the interbertebral disc degeneration. Conclusion Changes of vascular buds in vertebral endplate may accelerate intervertebral disc degeneration.

ObjectiveTo explore the feasibility of psychiatric assessment scale of cognitive and volition capacity(PASCVC) in violent crimes.MethodsThe PASCVC,criminal responsibility rating scale for violence(CRRSV) and criminal responsibility rating scale(CRRS)were used respectively in 244 forensic psychiatric cases.All cases were classified by the judicial surveyor into three grades including complete lost,partial lost and normal,the results were compared with expert's opinions.Results①PASCVC was composed of two subscales:cognitive capacity and volition capacity.There were significant differences in rating average score of three groups (complete lost,partial lost and normal) of total scale,cognitive capacity and volition capacity subscales were 9.96±7.53,69.15±10.290,101.16±6.034;20.29±4.971,37.13±5.388,2.55±2.612;18.32±2.595,30.07±4.595,48.34±4.147 respectively and there were significant differences between them.②27 items had good correlation with the scale(r=0.157~0.915).③According to the reference demarcation points of scale,12 criteria were carried out by using stepwise discriminant analysis.The accuracy of retrospective cumulative square was 97.1%.④Total scale and subscales consistency of three demarcation point grading assessment results were consistent with expert's opinions,the Kappa values were 0.925,0.848 and 0.930 respectively (P<0.01);PASCVC consistency with CRRSV and CRRS,the correlation coefficients were 0.952 and 0.961 respectively (P<0.01),the Kappa values were 0.830 and 0.881 respectively (P<0.01).Conclusion The PASCVC is feasible to be used in violent crimes.

Objective To investigate the influence of Chinese medicine ( CM) treatment on life span of middle-late primary hepatic carcinoma (PHC) patients. Results A multicenter retrospective cohort study was carried out in 489 PHC patients at the stages of Ⅱb, Ⅲa and Ⅲb collected from 15 domestic hospitals. With CM treatment as the exposure factor, the enrolled patients were divided into CM group, western medicine (WM) group and the CM-WM combination group. The main outcomes included median survival time (MST) and survival rate, and the effects of CM treatment and minimally invasive therapy on MST were observed. Results Half-a-year survival rate, one-year survival rate and two-year survival rate were 50%, 9%, 1% in CM group, 70%, 30%, 6% in CM-WM combination group, and 50%, 10%, 0% in WM group, respectively. The survival rates in CM-WM combination group differed from those in CM group and WM group (P<0.01), but the differences of survival rates between CM group and WM group were insignificant (P>0.05) . MST at the stages of Ⅱb, Ⅲa, Ⅲb in CM-WM group was obviously prolonged as compared with the other two groups (P<0.01), and MST at the stages of Ⅲa, Ⅲb in CM group was also obviously prolonged as compared with WM group (P<0.05) . The difference of MST at the stage of Ⅱb was insignificant between CM group and WM group ( P>0.05) . CM treatment and minimally invasive therapy were effective on prolonging the survival time of PHC patients ( P<0.01) . The results of COX regressionanalysis showed that Karnofsky scores, CM treatment and minimally invasive therapy were the preventive factors for the prognosis. Conclusion CM-WM combination group has the best long -term therapeutic effect. CM -WM combination treatment is effective on increasing MST and long-term survival rate, in particular for PHC patients at the stages ofⅡb, Ⅲa and Ⅲb. CM treatment and minimally invasive therapy are helpful for the prolongation of the survival time of PHC patients.

Disorders of sex development (DSD) is a complex disease that involves multiple traditional professional disciplines.Multidisciplinary team(MDT) mode is a particularly advantageous treatment mode that has been developed in recent years for the diagnosis and treatment of complex diseases.This article is based on the analysis of research progress at home and abroad about DSD and MDT,and the latest advances application of MDT treatment try for DSD,probe into how to establish a DSD-MDT treatment mode in theory preliminarily.

Adult ; Cytomegalovirus ; Cytomegalovirus Infections ; Humans ; Liver Failure

Objective To investigate the value of gradient echo T2'* -weighted imaging for detection of familial cerebral cavernous malformation (FCCM). Methods Twenty-six members in 2 families of FCCM were examined at 3.0 T by using CT, conventional MRI and GRE T2'*2'-WI sequences to detect numbers of FCCM. Results Twelve cases of FCCM were found by GRE T2'*-WI sequences. These patients all had multiple lesions(average of 23). The lesions were mainly located in ganglia area, followed by cortico-subcortical, thalamus, cerebellar and brain stem. These lesions appeared as special reticulated core of mixed signal intensity with a surrounding rim of decreased signal intensity representing bemosiderin from previous hemorrhages. The numbers of lesions (average of 5-17) and cases of FCCM (average of 3-9) examined by the conventional MRI were decreasing in the order of SE, DWI, T2FLAIR, T1WI and T2WI, each less than GRE T2'*-WI. CT only identified 3 cases with big lesions combined with hemorrhage and calcification.Conclusions GRE T2'*-WI could be a better choice of MRI sequence in diagnosing FCCM compared with CT and conventional MRI.

Objective To investigate the clinical,genetic and neuroimaging features by reporting a family with dyssynergia cerebellaris myoclonica. Methods The proband was examined clinically by neuroimaging,electromyography ( EEG),skin and muscles pathology and hematology.The patients with the illness in the family were followed up and the pedigree was drawn.Results There were 6 patients with dyssynergia cerebellaris myoclonica of the 27 family members in the family.All patients had disproportionate myoclonus,epilepsy,progressive cerebellar ataxia performance. Proband brain MRI showed cerebral atrophy.Cerebellar and cortical atrophy were more serious than other parts.There were long T,and long T2 signals in the white matter,high signal in T2FLAIR.EEG showed bursts of spike-low wave,polyspilke-low waves and polyspike waves distributing in the whole brain.Pathology of the skin and muscles was normal.Conclusions Dyssynergia cerebellaris myoclonica is an autosomal dominant disease,characterised by myoclonus,progressive cerebellar ataxia and epilepsy.Brain MRI shows cerebral cortical and cerebellar atrophy,abnormal signal in white matter.EEG showes spike and ware wave.The diagnosis is mainly based on family history,typical clinical manifestations,brain MRI and EEG changes.

Objective To examine the reliability and validity of compensative neurosis assessing scale (CNAS) and in order to develop the quantitative assessing method for distinguishing the compensation neurosis from malingering and typical neurosis. Methods The reliability and validity of the scale was tested based on the subjects including the cases involved forensic problems, the patients with typical neurosis in general psychiatric 0.944. The test-retest reliability was more than 0.820. The scorer reliability was more than 0. 812. The total accu-racy rate of CNAS for evaluating three groups of subjects based on the experts diagnosis, and with 91.2% of speci-ficity and 79.3% of sensitivity for evaluating compensation neurosis. Condusion The CNAS has acceptable psy-chometrics properties on reliability and validity. It will provide method to distinguish the compensation neurosis from malingering and typical neuroses.

Objective To evaluate the efficacy of double filtration plasmapheresis (DFPP) combined with immunosuppressive agents (leflunomide plus methotrexate) on synovitis in magnetic resonance imaging (MRI) in patients with high active rheumatoid arthritis (RA). Methods Fifty eight patients with RA (disease duration 6 months to 12 years) were randomly divided. Thirty-one were randomized to the treatment group and 27 were randomized to the control group. All patients received leflunomide 10 mg, two times daily; plus methotrexate 15 mg orally once weekly. DFPP was performed in the treatment group once 1-2 weeks for 3-4 sessions. Control patients did not receive DFPP. All patients underwent contrast-enhanced MRI of the right wrist at the baseline and 6 months, 1 month in the treatment group. The signs including synovitis pannus, bone marrow edema and effusion were observed on MRI. The scoring of synovial hypertrophy, pannus, bone marrow edema were measured according to the outcome measures in RA MRI scoring system. Comparisons between groups were performed with paired-samples t test and independent-sample t test. Results The MRI synovitis score, MRI pannus score and MRI bone marrow edema in the treatment group was (1.4±1.6), (0.13± 0.35) and (5±4) respectively,so was significantly lower than that of the control group [respectively for (7.9± 1.3), (2.76±0.43), (16±12),P<0.01]. 53% of the treatment group satisfied both the disease activity score 28-joint assessment and MRI synovitis assessment (no enhancement of synovium or pannus, no effusion), but none in the control group (P<0.01). Significant changes at 1 month was observed in DAS28 and HAQ scores (P<0.01), but not in the MRI synovitis score, MRI pannus score, MRI bone marrow edema score and effusion in the treatment group (P>0.05). Conclusion DFPP combined with immunosuppressive agents can significantly improve synovitis in MRI in patients with high active RA. Improvement of the signs of MRI is later than that in the clinic. So imaging assessment may be necessary for accurate evaluation of disease status and selection of therapy.