Neonatal intrahepatic cholestasis caused by Citrin deficiency(NICCD) is one of phenotypes of Citrin deficiency.It's an autosomal recessive disorder which was mainly seen in East Asia,including China.Case of NICCD was reported firstly by Japanese in 2001.In south area of China,the morbidity of NICCD is higher than that in north area of China.Most of the patients with NICCD has benign prognosis.Symptoms resolve within the first year of life,thus making a diagnosis difficult after this time.But few of patients will develop liver failure,even be fatal to life.Early diagnosis,regular follow-up and proper management may improve the prognosis.

Objective To analyze homocysteine in patients with acute cerebral infarction and related indicators of coagulation and fibrinolysis,diagnosis and treatment of acute cerebral infarction in order to provide reference for the disease.Methods Paitents with acute cerebral infarction from April 2012 to June 2014 were selected,they were divided into acute cerebral infarction groups and non acute cerebral infarction,,The acute cerebral infarction patients and non-patients with acute cerebral infarction group 1 ∶1.homo-cysteine and related indicators of coagulation and fibrinolysis were compared between two groups.Results Non-acute cerebral in-farction group homocysteine(Hcy),D-dimer,tissue-type plasminogen activator(t-PA),plasminogen activator inhibitor-1 (PAI-1 ) levels were significantly lower than in acute cerebral infarction group;non-acute cerebral infarction group(INR),significantly higher than the acute cerebral infarction group,the difference was statistically significant(P <0.05);after treatment of acute cerebral in-farction group,progress group Hcy,D-dimer,t-PA,PAI-1 was significantly higher than non-progression group;progressive group of international normalized ratio (INR),activated partial thromboplastin time(APTT)was significantly lower than non-progressive group,the difference there was statistically significant(P < 0.05 );good prognosis group D-dimer,t-PA,PAI-1 were significantly lower than a poor prognosis group,the difference was statistically significant(P <0.05)Conclusion Detection Hcy and related indi-cators of coagulation and fibrinolysis can effectively determine the content of the disease in patients with acute cerebral infarction, prognosis and progress,prognosis D-dimer and patients.

Objective To investigate the value of detection of anti-cyclic peptide containing citrulline (anti-CCP) antibody, rheumatoid factor (RF) and the combined detection of anti-CCP antibody and RF in rheumatoid arthritis (RA) patients. Methods From 2012 to 2014,1 961 patients were divided into three groups:diagnosed RA group(509 patients), not RA group(1 028 patients) and firstly was not diagnosed RA but later was diagnosed RA group (424 patients). The levels of RF and anti-CCP antibody were separately measured by rate nephelometry method and the electrochemical luminescence method. Results The sensitivity of detecting by anti-CCP antibody alone or anti-CCP antibody in combination with RF between RA group and not RA group was not significant difference ( P>0.05), but the specificity between two groups (88.6%vs. 60.4%) was significant difference ( P<0.05). There were significant differences in the sensitivity (81.7% vs. 74.3%) and specificity (88.6% vs. 66.0%) between by using anti-CCP antibody alone and RF alone.In firstly was not diagnosed RA but later was diagnosed RA group, there were significantly difference in sensitivity (98.3%vs. 82.1%) and specificity (91.6%vs. 81.5%) by using anti-CCP antibody alone and RF alone. Conclusion There is important clinical value by using anti-CCP antibody alone for the early diagnosis of RA.

Objective To monitor the distribution of pathogenic bacteria causing wound infection in trauma patients and their susceptibility to antibiotics in an effort to offer evidence for the rational clinical use of antibiotics.Methods Pathogens were identified by expressive type,but a very few was determined by the molecular methods.Micro-dilution or Kirby-Bauer method for minimum inhibitory concentration (MIC) was adopted for drug susceptibility test.Results A total of 121 species of pathogens numbering 1,257 bacterial strains were isolated from 1,066 patients.Strain number of the top 10 species accounted for 70.09％.Gram-positive cocci accounted for 48.93％ and Gram-negative bacilli for 49.72％.Among Staphylococcus aureus and epidermidis,methicillin-resistant bacterial strains accounted for 34.76％ and 71.08％ respectively,but none were resistant to vancomycin or linezolid.Enterococcus faecalis contained 6.06％ vancomycin-resistant enterococcal (VRE) and remained ＞ 90％ sensitive to teicoplanin,ampicilin and vancomycin.Enterococcus faecium contained 10.53％ VRE and remained ＞90％ sensitive to teicoplanin and ＞ 80％ sensitive to vancomycin.Baumanii contained 12.77％ multidrug resistant strains and remained ＞ 70％ sensitive to imipenem and meropenem.Baumanii/calcoaceticus complex contained 29.72％ multi-drug resistant strains and remained ＞ 50％ sensitive to imipenem and meropenem.Pseudomonas aeruginosa contained 3.73％ multi-drug resistant strains and remained ＞90％ sensitive to amikacin,meropenem,imipenem and piperacillin/tazobactam.No E.coli,klebsiella pneumoniae and enterobacter cloacae resistant to imipenem or meropenem were found.E.coli contained 69.51％ extended-spectrum β-lactamases (ESBLs)-producing strains and remained ＞ 90％ sensitive to piperacillin/tazobactam and ＞ 80％ sensitive to amikacin.Klebsiella pneumoniae contained 53.13％ ESBLs-producing strains and remained ＞ 80％ sensitive to amikacin.Conclusions Common bacteria are the leading cause of posttraumatic infection.Enterococcus faecalis and enterococcus faecium demonstrate higher susceptibility to teicoplanin; two kinds of acinetobacter demonstrate higher susceptibility to imipenem.

0.05);at 30 minutes,60 minutes,and 120 minutes,but not 24 hours post-administration,Na,RBC,and HGB levels significantly changed compared to those before administration.24 hours post-administration,HCT,K,Cl,TBIL,and DBIL levels significantly changed compared to those before HSS40 administration(P

Objective To assess iodine nutritional status of lactating women and infants (0-2 years old) in six ecological regions of Gansu Province,and to provide a scientific basis for iodine supplementation of target people.Methods According to different topography and climate conditions,Gansu Province was divided into six ecological regions:the desert region,the Gobi region of Hexi Corridor,the hills and valleys region of Loess Plateau,the Zhongshan Valley of Qinling Mountains,the grassland meadow area of Gannan plateau and the Loess Plateaugully region.Totally 5 counties were selected from each ecological region,and each county was divided into five geographic locations (east,south,west,north and center).In each location,one township was identified and ten samples of lactating women and infants (0-2 years old) were selected randomly and 60 salt samples of residents were collected to determine iodine content.At the same time salt intake of 20 residents was surveyed in three townships as mentioned above.The water samples of all the centralized water supply projects were collected in each county,and one water sample of the decentralized water supply in ten townships with the largest population of each county from east,south,west,north and center locations was collected to determine iodine content.One soil sample was collected to determine iodine content in east,south,west,north and center townships of each county.The urinary iodine and water iodine levels were measured with the method for determination of iodine in urine by As3+-Ce4+ catalytic spectrophotometry (WS/T 107-2006).The direct titration method among thegeneric methods for iodide testing in salt production industry (GB/T 13025.7-2012) was used to determine the salt iodine level,and the arbitration method was adopted for quantitative determination in the case of Sichuan salt or other special salts and the salt intake was estimated based on three day weighed food record.The soil iodine was determined using the amplified colorimetric method.Results Totally 1 476 and 1 461 urine samples of lactating women and infants were collected,and the total urine iodine medians of lactating women and infants (0-2 years old) were 149.05 and 151.34 μg/L,respectively,the urine iodine median of lactating women and infants in each ecological region was at the 100-199 μg/L appropriate level.Not weaning infants urine iodine (155.32 μg/L) was higher than that of the weaning infants (146.30 μg/L),the difference was significant statistically (Z =-2.808,P ＜ 0.05).There was a correlation between urine iodine of not weaning infants aged 0-2 years old and breastfeeding women (r =0.133,P ＜ 0.01),and there was no correlation between weaning infants and breastfeeding women (r =0.045,P ＞ 0.05).About 9 008 salt samples were collected,and the rates of qualified iodized salt intake in the six ecological regions were all higher than 93％.The daily salt intake per person ranged from 4.5 g to 12.6 g,in which the Loess Plateaugully region was the highest with 12.6 g and the grassland meadow area of Gannan plateau was the lowest with 4.5 g.The water iodine median of 1 513 water samples was 3.3 μg/L.The water iodine medians of the rest 4 ecological regions were all less than 5 μg/L except for the desert region (42.4 μg/L) and the Loess Plateau-gully region (30.0 μg/L) of which the median of water iodine was higher than 20 μg/L.The soil iodine median of 155 soil samples was 127 μg/kg,in which the desert region was the lowest with 78 μg/kg and the grassland meadow area of Gannan plateau was the highest with 218 μg/kg.Conclusion Lactating women and infants are in good iodine nutritional status and the present iodized salt concentration can meet the demand of iodine nutrition of lactating women and infants well.

Objective To study the correlation between patellar stability and keen clinical manifestation in adults with Kaschin-Beck disease (KBD).Methods Through a cross-sectional study in September 2016,one hundred and forty-three adult patients with KBD were asked to accept a digital radiographic X-ray (DR) which included positive and lateral slices of knee joint and axis slice of patellar.Meanwhile,every patient must undergo a Lysholm function evaluation for knee joint and morphological measurement on the DR film which included Insall index,lateral patellofemoral angle,sulcus angle,congruence angle,lateral migrating ratio of patella,and patellofemoral index.Then,the regression equation was built and the correlation analysis was made with multiple linear regression test.Results One hundred and forty-three patients' average scores of Insall index,lateral patellofemoral angle,sulcus angle,congruence angle,lateral migrating ratio of patella,patellofemoral index and Lysholm score were 1.10 ± 0.17,(14.49 ± 1.47)°,(138.08 ± 3.86)°,(11.55 ± 2.17)°,(1.34 ± 0.13)％,1.18 ±0.10,and (62.96 ± 6.11) scores.By multiple linear regression test,Insall index (X1),congruence angle (X4),lateral migrating ratio of patella (X5),and patellofemoral index (X6) were selected to enter into the equation;while,lateral patellofemoral angle and sulcus angle were rejected.The multiple linear regression equation was as follows:Y =17.529 + 15.232X5 + 0.950X4 + 15.957X6-4.224X1.The adjusted determination coefficient (R2) of the equation was 0.559.Those indexes which were selected to enter into the equation were ranked from big to small based on the impact on Lysholm as follow:congruence angle,lateral migrating ratio of patella,patellofemoral index,and Insall index.Among them,Lysholm score had a negative correlation with Insall index.Through variance analysis,F valve was 46.642,and P ＜ 0.05,which meant the fitted equation had statistical significance.Conclusion There is significant correlation between the patellar stability and knee Lysholm scoring in adult with KBD,which is reflected with the following parameters from big to small in turn:patellofemoral index,lateral migrating ratio of patella,congruence angle and Insall index.

Objective To evaluate diagnostic value of CT coronal scan to foreign body in bronchus of children.Methods 30 cases appearances of CT coronal scan to foreign body in bronchus of children were analysed.The appearances between CT coronal scan and axial scan,X-ray examination of chest were contrasted in some cases.Results Foreign body in bronchus or granuloma around part of foreign body were shown clearly by CT coronal scan in 30 cases,28 cases obstructive emphysema,13 cases obstructive pneumonia and 3 cases obstructive atelectasis were also shown clearly.Another,CT coronal scan showed the double margin sign of 16 cases diaphragm and 11 cases mediastinum.Conclusion CT coronal scan could show direct and indirect signs of the most foreign body in bronchus of children and it may be acte as primary examintion.

Objective To monitor the clinical epidemiology and etiology of acute diarrhea in children in the outpatient setting in Shanghai .Methods An active surveillance study in Children′s Hospital of Fudan University between August 2013 and July 2014 was conducted .Outpatient children with acute diarrhea were enrolled in this study and stool samples were collected .Pathogens including norovirus ,diarrheagenic Escherichia coli (DEC) , nontyphoidal Salmonella spp .(NTS),Campylobacter,Shigella,pathogenic vibrio and Yersinia enterocolitica were identified and typed .The χ2 test was used for statistical analysis .Results Of the 881 stool samples from enrolled children , the pathogens included into the target detection were identified in 246 (27 .92% ) cases . Norovirus ,DEC ,NTS ,Campylobacter and Shigella were detected in 98 (11 .12% ) cases ,74 (8 .40% ) cases , 61 (6 .92% ) cases ,34 (3 .86% ) cases and 2 (0 .23% ) cases ,respectively .Neither pathogenic vibrio nor Yersinia enterocolitica was identified .Children younger than 36 months old (3 .27% ,26/794) had a lower risk (χ2=7 .41 ,P=0 .006) of Campylobacter infection compared with older children (9 .20% ,8/87) .Vomiting (37 .76% ) and watery diarrhea (21 .34% ) were more commonly seen in children with norovirus infection;fever and mucous stool were commonly seen in diarrheal children with NTS infection (40 .98% and 21 .31% ,respectively) and Campylobacter infection (29 .41% and 26 .47% ,respectively) .Conclusion Enteric pathogens play a major role in childhood acute diarrhea in Shanghai .Continuous monitoring of enteric pathogens will be helpful for reasonable treatment and prevention of acute diarrhea in children .

ObjectiveTo understand the clinical epidemiology of enterovirus 71 (EV71) in children with hand,foot,and mouth disease (HFMD) in Shanghai during 2010 to 2011.Methods The demographic,etiological and clinical data of children with HFMD were analyzed retrospectively.EV71 was tested in stool samples by one-step quantitative reverse transcription-polymerase chain reaction (RT-PCR).The date were analyzed by Chi-square test.ResultsEV71 was detected in 820 (54.45％) of 1506 inpatients in 2010 and in 924 (59.84％) of 1544 inpatients in 2011,respectively.The detection rates in severe cases and uncomplicated cases were 86.31％ and 46.67％ (x2 =247.146,P＜0.01) in 2010 and 88.78％ and 48.75％ (x2 =201.664,P＜0.01) in 2011,respectively.The detection rate of EV71 was the highest (60％- 67 ％) during May and June when HFMD peaked.Among 1744 EV71-infected HFMD inpatients,the male-to-female ratio was 1.78 ∶ 1 ; the proportion of cases was the lowest in infant ＜6 months of age (0.46％,8/1744),and the highest in children 1 years of age (34.92％,609/1744); children aged 1-3 years accounted for 76.78％ (1339/1744);and migrant children accounted for 72.76 ％ (1269/1744).The demographic characteristics in severe cases were similar to those in general EV71-infected children.Nine severe cases of pulmonary edema/hemorrhage were all infected with EV71.Conclusions EV71 was a major pathogen causing the outbreaks of HFMD and severe complications in Shanghai in 2010 and 2011.Most severe cases and all critically severe cases were associated with EV71 infection.