Objective To explore the clinical manifestations, CT features, and inheritance pattern of Fahr disease. Methods Head CT was scanned and serum calcium and phosphorum were measured in 14 persons from 2 families, and the 2 families' history was investigated. Results The serum calcium and phosphorum were normal in the 14 persons. There were 8 cases of Fahr disease, and the head CT showed local or diffuse calcium deposition in bilateral basal ganglia, subcortex, and thalamus, respectively. The inheritance pattern of the 8 cases of Fahr disease in the two families showed holandric inheritance, The clinical symptom and sign included seizures, irritability, mental retardation or no abnormal findings. Conclusion Fahr disease is a hereditary disease characterized by idiopathy and calcium deposition in the central nervous system. The clinical feature is various and head CT is an important examination in the diagnosis of Fahr disease.

Objective To assess the effects of hydrocortisone (HC) on brain injury in septic shock rats induced by lipopolysaccharide (LPS).Methods Pathogen-free Wistar male rats were chosen to produce septic shock model by injection of LPS (Escherichia coil,O55:B5),25 mg/kg LPS was given by femoral vein.All rats in accordance with the random number table,were randomly divided into 3 groups:normal control group,LPS group,and low dose of HC group (HC 5 mg/kg).Every group had ten rats.Six hours after the septic shock model was established,the blood sample and brain tissue were collected to perform histopathological examination and measure the serum level of neuron specific enolase (NSE).Results The serum level of NSE,brain water content,inflammation score,neural damage score,vascular reaction score in LPS group were significantly higher than those in control group [(10.078 ± 0.526) μg/L vs.(3.719 ± 0.602) μg/L,(82.328 ±0.011) ％ vs.(78.896 ±0.005) ％,(2.750 ±0.463) scores vs.(0.125 ±0.345) scores,(2.875 ± 0.353) scores vs.(1.125 ± 0.354) scores,(2.875 ± 0.463) scores vs.(0.500 ± 0.534) scores,P ＜ 0.01,respectively].After HC treatment,the serum level of NSE,brain water content,and brain tissue pathological score [(6.297 ± 1.393) μg/L,(80.283 ± 0.005) ％,(0.625 ± 0.744) scores,(1.375 ± 0.744) scores,(1.250 ± 0.463) scores] were significantly lower than those in LPS group (P ＜ 0.05,respectively).Conclusion After giving low dose of HC,the serum level of NSE,brain edema,brain pathological changes all got improvement,which indicated that low dose HC could have protect effect on septic encephalopathy induced by LPS.

Objective To study the MR imaging features of intracranial epidermal cyst.Methods 18 cases(include 7 male,11 female) epidermal cyst pathology confirmed were analysed.Results The epidermal cyst usually exhibited heterogeneously low signal intensity on T 1WI(88.9%),and only 2 patients showed high signal intensity on T 1WI(11.1%).After contrast medium enhancement 16 cases had no obvious enhancement,only 2 patients had rim light enhancement .Conclusion Typical epidermal cyst commonly show heterogenously low signal intensity on T 1WI. A typcal epidermal cyst has high signal intensity on T 1WI.Differential diagnosis shoud be consider on these kind of epidermal cyst.

Objective To evaluate the images of MRCP acquired by adjusting 0.3 Tesla MR unit heavy T 2-weighted scanning parameters and altering scanning angle and diagnostic correctivity to biliary obstructive disease.Methods Routine MR T 1WI,T 2WI scanning were performed axial in 50 cases of patients with biliary obstruction.All of them were divided into two groups,20 cases of them were scanned coronal with FSE T 2-weighted fixed parameters,30 cases of them were scanned with altering scanning angle,increasing scanning slices,decreasing distance of two near slices,reducing signal collection times(NSA),shortening scanning time.Results The acquired images through adjusting FSE T 2-weighted scanning parameters were visualized clearly.The boundary of cholangiopancreatie ducts were showed clearly.The display rate of biliary and pancreatie ducts was elevaled from 20% to 83.3%.The accuracy of it for evaluating the causes of obstruction was increased from 88.9% to 93.3%.The accuracy of it in the detection of the location of bile duct obstruction was 100%.Conclusion Through adjusting scanning purameters low field MRCP is very helpful in improved images quality and reflecting veliable signs of biliary and pancreatie duct obstruction disease combined with MRI T 1-weighted T 2-weighted message.This method can increased the diagnostic accuracy of the causes of obseruction and supply the reliable ground for clinical treatment.

Objective To investigate the risk factors related to mortality of children with deep fungal infection in pediatric intensive care unit(PICU).Methods A retrospective case-control study was applied.Ninty-six patients admitted to PICU with clinical or definite diagnosis of deep fungal infection from Nov 2005 to Mar 2009 were included.The risk factors related to mortality wereanalyzed with the logistic regression analysis.The research factors included:sex,age,primary diseases,complications,invasive operations and therapeutic measures etc.Results Of all 96 children,28 died (28.2％).According to the analytical results of multivariate logistic regression,the variables significantly associated with mortality were immunosuppressive (OR =185.770,95 ％ CI 11.467 ～ 3 009.507),mechanical ventilation (OR =11.555,95 ％ CI 2.780 ～ 48.039),hypoproteinemia (OR =1.246,95％ CI 1.133 ～ 1.369) and low pediatric critical illness score (OR =1.086,95 ％ CI 1.008 ～ 1.169).Conclusion The risk factors related to mortality of children with deep fungal infection in PICU were immunosuppressive,mechanical ventilation,hypoproteinemia and low pediatric critical illness score.

AIM: To investigate the effects of ?-elemene,extracted from curcuma wenyujin,on proliferation, cell cycle and apoptosis of human multiple myeloma RPMI-8226 cells. METHODS: The effect of ?-elemene on the growth of human multiple myeloma RPMI-8226 cells was studied through MTT assay,cell cycle and apoptosis was studied by combined Annexin-V protein iodide staining,The morphological changes was studied by combining Hoechst33342-PI staining. RESULTS: ?-elemene inhibited the proliferation of RPMI-8226 cells in a time-and dose- dependent manner. Compared with the control cells,the proportions of the RPMI-8226 cells in the G0 /G1 phase rose,and the proportions of the RPMI-8226 cells in the S and G2 /M phases fell decreased. RPMI-8226 cells treated with ?-elemene for 48 h induced apoptosis in a dose-dependent manner. CONCLUSION: ?-elemene is able to inhibit the proliferation of RPMI-8226 cells by arresting the cell cycle and inducing the cell apoptosis.

Chronic obstructive pulmonary disease (COPD) is a serious disease constituting a major threat to public health. Current popularity of traditional Chinese medicine (TCM) in treating COPD implies its potential advantage in improving symptoms, reducing the frequency of acute exacerbation and improving the patients' quality of life. This study is to evaluate the efficacy and safety of a comprehensive intervention, Bufei Yishen Granule combined with acupoint-sticking therapy, in patients with stable COPD.

Objective To study the molecular characteristics of the Staphylococcus aureus isolated from the intensive care units (ICUs) of children's hospital.Methods From January 2016 to December 2016,a total of 39 S.aureus strains were collected and identified from various clinical specimens that were obtained from patients who were confined in the neonatal and pediatric ICUs of Beijing Childreng Hospital.Methicillin-resistant S.aureus (MRSA) and methicillin-susceptible S.aureus (MSSA) were identified using the cefoxitin disc method and the detection of the mecA gene.Multilocus sequence typing (MLST) and spa typing were analyzed using the PCR,and the staphylococcal chromosomal cassette mec (SCCmec) type was analyzed for the MRSA isolates.Twenty-one superantigen genes and the Panton-Valentine leukocidin (pvl) gene were also detected by PCR.Their susceptibility to 12 antibiotics was evaluated using the E-test method.The differences in prevalence of virulence genes and antimicrobial resistance were compared between the MRSA and MSSA isolates by Fisherg exact test.Results All the S.aureus strains were isolated from secretion inside the airway of pneumonia (including severe pneumonia),the blood of patients with bacteremia,and exudate of skin and soft tissue infections.ST59-SCCmecⅣa-t437 (55.6％) and ST398-t571 (28.6％) were the most predominant clones of MRSA and MSSA,respectively.Of the 39 isolates,26 strains (66.7％) had at least one superantigen gene,and seb (38.5％),sek (30.8％),and seq (20.5％) were the most common genes;seb-sek-seq (18.0％) was the main virulence genotype.The pvl geneg positive rate was 25.6％,and no significant difference between MRSA and MSSA was observed (P ＞ 0.05).Notably,79.9％ of the S.aureus isolates were multidrug resistant,and 94.9％,53.8％,and 51.3％ of the isolates were resistant to erythromycin,clindamycin,and chloramphenicol,respectively.The tested isolates were susceptible to trimethoprim/sulfamethoxazole,rifampicin,and vancomycin.Conclusions The S.aureus strains from the ICUs of childreng hospital were isolated from the secretion inside the airway of pneumonia (including severe pneumonia),the blood of patients with bacteremia,and exudate of skin and soft tissue infections.ST59-SCCmecⅣa-t437 (55.6％) and ST398-t571 (28.6％) were the common clones of MRSA and MSSA,respectively.The prevalence of superantigen genes and the multidrug resistant rate were relatively high.

Objective:To predict the risk of systolic anterior motion (SAM) after mitral valvuloplasty(MVP) by intraoperative transesophageal echocardiography (TEE) and its diagnostic value.Methods:From August 2016 to May 2020, 215 patients with mitral valve degeneration underwent MVP, including 182 patients without SAM (non-SAM group), and 33 patients with SAM (SAM group). TEE examination was performed immediately after operation to determine whether SAM phenomenon was relieved. According to the physiological basis of SAM, before cardiopulmonary bypass (CPB) and immediately after CPB, the parameters of SAM group and non-SAM group were measured and compared, including left atrial dimension(LAD), left ventricular end diastolic diameter(LVEDD), left ventricular end systolic diameter(LVESD), left ventricular ejection fraction(LVEF), basal septal diameter(basal-IVDd), left ventricular posterior wall thickness(LVPW), left ventricular outflow tract diameter(LVOTD), left ventricular outflow tract maximum velocity(LVOT-Vmax), left ventricular outflow tract pressure gradient(LVOTG), mitral valve maximum velocity(MV-Vmax), mitral valve mean pressure gradient(MVG-mean), mitral regurgitation area(MR-area), bulging subaortic septum, anterior leaflet length, posterior leaflet length, ratio between the lengths of the anterior and posterior leaflets, coaptation-septum distance(c-sept), nnular diameter of mitral valve, aorto-mitral angle (AMA) to screen the independent risk factors of SAM after MVP.Results:① Compared with the non-SAM group, LVEDd, LVESD, ratio between the length of the anterior and posterior leaflets, c-sep and AMA decreased in SAM group (all P<0.05), while basal-IVDd, LVEF, posterior leaflet length and bulging subaortic septum increased in SAM group (all P<0.05). ②Compared with that before the "edge to edge" technique, LVOT-Vmax decreased from (4.31±2.26)m/s to (2.55±1.39)m/s, LVOTG decreased from (43.58±10.89)mmHg to (23.36±12.76)mmHg, MVG-mean increased from (0.46±0.33)mmHg to (2.27±0.43)mmHg, and MR-area increased from (3.52±0.79)cm 2 to (0.96±0.57)cm 2 (all P<0.05). ③Multivariate logistic regression analysis showed that independent risk factors of SAM were LVEDd<45.430 mm ( OR=0.267, 95% CI=0.084-0.847), basal-IVDd>14.870 mm ( OR=12.049, 95% CI=1.619-89.661), length ratio of anterior and posterior leaflets of mitral valve>1.371 ( OR=0.159, 95% CI=0.045-0.562), angle of bulging angulated subaortic septum>62.330°( OR=18.246, 95% CI=2.824-117.896), c-sept<23.965 mm( OR=0.177, 95% CI=0.05-0.628), and AMA<123.730°( OR=0.197, 95% CI=0.098-0.396). Conclusions:Intraoperative TEE can evaluate the risk factors of SAM before MVP, and find the SAM phenomenon after MVP in time, which is helpful for surgeons to prevent and correct SAM after MVP and avoid secondary operation.

Objective To investigate the status of clinical nutrition management in patients in PICU,and to provide data for promoting the improvement and development of clinical nutrition of pediatric critically ill patients.Methods A questionnaire survey was conducted on PICU specialists.The nutrition assessment,nutrition intervention and nutrition management of critically ill children in PICU were investigated.The results were summarized and analyzed.Results A total of 39 PICU specialists were involved in this survey.The nutritional assessment methods and guidelines in domestic PICU were not unified.Twenty-five respondents (64.1%) believed that both clinical performance and the scales as the basis can decide whether the patients should be fed or not;all respondents believed that nutritional assessment and intervention time need to be determined by the needs of the patients;23 respondents(58.9%) used weight only as their nutritional monitoring indicators.Twenty-eight respondents(71.8%) considered that gastric tube was the first choice way to feeding for the critically ill children;20 respondents(51.3%) believed that critically ill children should be fed within 24 hours.Twelve respondents (33.3%) believed that critically ill children should be fed between 24 to 48 hours.Thirty-three respondents(84.6%) advocated early enteral nutrition;36 respondents (92.3%) considered that the main reasons of fasting in critically ill children were vomiting or abdominal distension or gastrointestinal bleeding.Twenty-eight respondents(71.8%) believed that according to the results of gastrointestinal function evaluation,they made decisions whether the patient to fast or not.Twenty-three respondents(59.0%) considered that specialists in PICU were the decision maker of the clinical nutrition in critically ill children.Twenty-four (61.5%) of the respondents believed that we needed to establish our own routines in management of nutrition in PICU.Conclusion At present in China,a lot of achievements have been made in the nutritional assessment,monitoring,early enteral nutrition intervention and management in critically ill children,but it is not enough.We need to make more effort to enhance the critically nutrition level in PICU,and we have a lot of research to do about nutrition assessment and nutrition intervention mode.It is recommended to establish Chinese guidelines or consensus to enhance the level of nutritional treatment of critically ill children.