Objective To analyze the epidemiology and clinical characteristics of the children with measles. Methods Retro-spectively analyzed methods were applied to analyze the timing, and season of the break, age distribution, clinical manifestations and major complications of 3431 children with measles (<12 years) in the Second Hospital of Nanjing from 2008 to 2015. Results It found that there was a peak incidence of measles in children each in 2009, 2013, and 2015 , peaking in March to May. Onset age of measles mainly within the first year of life, and incidence increased with age within the first 8 months. Typical clinical manifestations are mainly fever, rash, cough, and catarrhal symptoms. The main complications of measles were pneumonia, laryngitis and heart failure. Conclusions Early diagnosis and effective treatment of the children with measles needs to be strengthened.

Objective To explore the effect of tetramethylpyrazine (TMP) on serum cytokines and cardiac function in patients with sepsis myocardial injury.Methods Fifty patients with sepsis myocardial injury were admitted in Hangzhou First People's Hospital from June 2015 to September 2016,and according to the computer generated random number,they were divided into conventional treatment group and TMP treatment group,25 cases in each group.The patients in the conventional treatment group were treated with antimicrobial agents,vasoactive drugs,fluid resuscitation and organ function support,etc.;TMP treatment group was treated with traditional Chinese medicine (TCM) TMP on the basis of routine treatment.TMP 120 mg was added to 250 mL normal saline (NS) for intravenous drip once a day for a therapeutic course of 10 days.Plasma N-terminal pro-B type natriuretic peptidec (NT-proBNP) and serum tmnor necrosis factor-oα (TNF-α) levels were measured before treatment and on 1,5 and 10 days after treatment.Left ventricular internal systolic dimension (LVIDs),fractional shortening (FS) and left ventricular ejection fraction (LVEF) were measured by cardiac ultrasound,3 cardiac cycles were measured and the average results were taken.The intensive care unit (ICU) hospital stay and mortality were calculated in the two groups.Results The levels of NT-proBNP and TNF-α in the two groups after treatment were lower than those before treatment,the differences in the levels were statistically significant between those before treatment and 5 days after treatment in the conventional treatment group [NT-proBNP (ng/L):334.25 ± 36.46 vs.577.72 ± 60.34,TNF-α (ng/L):388.48 ± 43.38 vs.507.74-± 31.63,both P ＜ 0.05],and in TMP treatment group,the levels from 1 day after treatment compared with those before treatment,there were statistical significant differences [NT-proBNP (ng/L):387.44 ± 36.39 vs.571.67-± 56.34,TNF-α(ng/L):403.79 ± 23.20 vs.505.82 ± 31.31,both P ＜ 0.05];the degrees of decrease in TMP treatment group were more obvious than those in conventional treatment group (all P ＜ 0.01).After treatment in the two groups,the LVIDs was decreased gradually,FS and LVEF were increased gradually,reaching the lowest or highest level on 10 days after treatment,and the changes of TMP treatment group were more significant than those in the conventional treatment group,LVIDs and LVEF of the two groups showed statistically significant differences on 5 days after treatment [LVIDs (mm):43.23 ± 5.57vs.48.21 ± 2.29,LVEF:0.47 ± 0.02 vs.0.41 ± 0.02,both P ＜ 0.05],FS on 1 day after treatment showed a statistically significant difference [FS:(23.92 ± 1.81)％ vs.(22.84 ± 1.79)％,P ＜ 0.05].The ICU stay in the TMP group was shorter than that in the conventional treatment group (days:13.16 ± 2.67 vs.16.48-± 3.08,P ＜ 0.05),and the mortality was lower than that of conventional treatment group [20％ (5/25) vs.36％ (9/25)],the difference being not statistically significant (P ＞ 0.05).Conclusions In septic myocardial injury,TNF-α plays an important role;after TMP treatment in patients with myocardial injury caused by viral myocarditis,the serum TNF-α level is decreased showing it has antagonizing TNF-α activity,thus it has protective effect on sepsis myocarditis,improves heart function and the disease prognosis.

Objective To analyze the mid- and long-term results after coronary artery bypass graft surgery (CABG), to evaluate the accuracy of multi-detector CT coronary angiography (MDCT) on the graft patency status and to compare the patency for different grafts. Methods One hundred and one cases underwent CABG from June 1992 to March 2008 were followed up by searching the database of MDCT (42 cases) and selective coronary angiography (SCA, 59 cases). The mean following up period was (66.79 ±44.27) months. Three hundreds and 10 grafts including 115 arterial and 195 venous were analyzed.A comparison of the patency rate between the arterial and venous grafts was also carried out. At the mean time, 2 groups were divided according to the paft lesions( patency of not). Uunivariate analysis and Logistic regression analysis were made for statistics. Results At the follow-up of (53.93 ± 36.80) months, the patent rate of LIMA, RA, SV and CV examined by MDCT was 94.7%, 92.0%, 85.9% and 60.0%, respectively. The patent rate for MDCT patients with angina was 83.5%, and 95.2% for angina-free group ( P = 0.046). At the follow-up of (75.95 ±47.09) months, the patent rate of LIMA, RA, SV and CV examined by SCA was 87.1%, 81.0%, 53.6% and 57. 1%, respectively. In SCA patients the patent rate was 62.0% for angina group and 100% for angina-free group ( P = 0.025 ). According to logistic regression analysis, only the postoperative period was statistically related to the graft lesion. Conclusion MDCT could find out the occlusions and obvious stenoses of the grafts after CABG. It might be served as a noninvasive method to evaluate the grafts patency postoperatively. The mid- and long-term patency of arterial grafts is better than venous grafts. Graft lesion is an important factor leading to postoperative recurrent angina pectoris. The severity of the graft lesion has a close relation to the lenth of postoperative period.

The index system of comparative advantages was constructed for the pharmaceutical manufacturing in-dustry in different provinces , municipalities directly under the Central Government , and autonomous regions ac-cording to the integrated comparative advantage theory .The integrated comparative advantages in different provinces , municipalities directly under the Central Government , and autonomous regions were scored by principal component analysis and qualitatively analyzed by clustering analysis to reveal their spatial distribution , thus providing the deci-sion-making support for the policy makers, manufacturers, investors and staff of pharmaceutical manufacturing in-dustry.

Objective:To analyze the clinical characteristics and SLC25A13 gene mutation in children with neonatal intrahepatic cholestasis caused by citrin deficiency(NICCD).Methods:The data of 18 children diagnosed with NICCD in Xi′an Children′s Hospital from January 2014 to December 2018 were collected.The clinical manifestations, biochemical characteristics, SLC25A13 gene mutation and prognosis were analyzed.Results:All the 18 cases of NICCD were from North China and the age of initial diagnosis averaged(63.4±19.5)days.The clinical manifestations included jaundice(100%), light yellow or white stool(38.9%), growth retardation(27.8%)and so on.All patients had cholestasis.Of 18 cases, the levels of glutamyltranspeptidase, total bile acid and alpha fetoprotein were all increased, and serum albumin was decreased.Elevated aspartate aminotransferase(94.4%), elevated glutamic pyruvic transaminase(72.2%), prolonged prothrombin time(88.9%), hyperlactemia(83.3%), hypoglycemia(77.8%), anemia(66.7%)and other biochemical abnormalities were observed.Citrulline and other serum amino acids of all cases were elevated in blood samples by tandem mass spectrometry.The increase of 4-hydroxyphenyllactate and 4-hydroxyphenylpyruvate was found in 70%(7/10)urine samples by gas chromatography.Age was negatively correlated with total bile acid( r=-0.469, P=0.049), and positively correlated with blood ammonia, threonine, methionine, ornithine and tyrosine( r=0.472, 0.690, 0.698, 0.678 and 0.769, respectively, P<0.05). A total of 16 SLC25A13 gene mutations were detected, of them c. 851_854del(33.3%)and c. 1638_1660dup(19.4%)were the most common.c.1841+ 3_1841+ 4del, c.980_981del(p.E327Vfs*45)and c. 602A>T(p.E201V)were novel mutations.Among the 17 children who were followed up, 1 case died and 16 cases had normal biochemical parameters within 1 year. Conclusion:The characteristic biochemical changes are helpful for early recognition of NICCD.The prognosis of NICCD is good if the treatment is appropriate and timely.c.851_854del and c. 1638_1660dup are high-frequency mutations of SLC25A13 gene in north China.

ObjectiveTo investigate the feasibility and efficacy of using the hepatoduodenal ligament tension-reduced operation (tension-reduced operation in short) for iatrogenic bile duct injury where the bile duct was severely defective. MethodsBetween March 2006 and May 2009, the authors treated 6 patients with iatrogenic bile duct injury (Bismuth type Ⅱ : 5 patients and type Ⅲ : 1 patient). A no. 7 black silk thread was used to hold the hilar plate tissues and the seromuscular layer of the bulbous part of the duodenum closer together and knots were tied. This method brought the porta hepatis and the duodenal bulb closer together and the hepatoduodenal ligament was shortened. An end to end anastomosis could then be made between the two broken ends of the defective bile duct without tension. ResultsSix patients suffered from bile duct injury and they recovered fully after the tensionreduced operation. There was no complication on follow-up. ConclusionsThe tension-reduced operation was efficacious in the treatment of iatrogenic bile duct injury. This technique should be popularized and more widely used.

Objective:To evaluate the clinical efficacy of staged surgery in treatment of calf Gustilo-Anderson type IIIC fracture.Methods:A retrospective case series was conducted to analyze clinical data of 16 patients with calf Gustilo-Anderson type IIIC fracture admitted to Shanghai Jiao Tong University Affiliated Sixth People's Hospital from January 2017 to December 2019. There were 12 males and 4 females, with the age of (38.6±8.2)years (range, 18-53 years). All patients had limb salvage treatment at one stage in the emergency department. The survival of the limb and the occurrence of vascular crisis were examined within one week after limb salvage. The second stage involved the repair of skin and soft tissue defects with the defect area from 12.0 cm×5.0 cm to 20.0 cm×8.0 cm using free flaps. The survival of the flap, vascular crisis, and donor site healing within two weeks after the flap procedure. The third stage used bone graft revision and bone lengthening technology to repair bone tissue. The lower extremity functional scale (LEFS) and Mazur ankle joint function score were used to evaluate the function of the affected limb before bone repair and at the last follow-up. The fracture healing and related complications were observed at the last follow-up.Results:All patients were followed up for (14.2±4.6)months (range, 8-20 months). At one stage, the limb-saving surgery was successful in all patients, among which one had vascular crisis. At second stage, free flaps survived in all patients, among which two had vascular crisis. All donor areas were healed by first intention. At third stage, the LEFS of the affected limb was increased from (32.0±7.4)points before bone repair to (48.0±10.2)points at the last follow-up ( P<0.01) and the Mazur score was increased from (50.9±15.3)points before bone repair to (73.8±11.9)points at the last follow-up ( P<0.01). All bone defects were repaired and healed without complications such as infection or osteomyelitis at the last follow-up. Conclusion:For calf Gustilo-Anderson type IIIC fracture, the staged strategy can effectively save limbs and restore limb function.

Surgery is the most effective way to cure non-small cell lung cancer currently. Although sleeve lobectomy, with less reduction of respiratory function and a lower mortality rate, would not compromise oncological results in well-selected patients with central lung cancer, pneumonectomy is still necessary when complete resection could not be achieved by other surgical procedures. Cardiopulmonary complications are the most common complication after pneumonectomy. Fully understanding the related complications after pneumonectomy can help surgeons make a diagnosis timely, and further take relevant measures to reduce the adverse effects of postoperative complications on patients. What's more, in order to avoid postoperative complications and improve the prognosis of patients as much as possible, it is necessary to fully understand the risk factors to minimize the risk and to make the patients benefit from pneumonectomy as much as possible.

OBJECTIVE: To explore the genetic basis of a pedigree affected with Alagille syndrome (ALGS). METHODS: Targeted capture and next generation sequencing was carried out for the proband. Candidate variants were verified by Sanger sequencing among his family members. Their pathogenicity of the variant was predicted with bioinformatic analysis. Clinical characteristics and genotype-phenotype correlation were analyzed. RESULTS: The proband, his elder sister and mother were found to carry a heterozygous c.1270dupG (p.Ala424Glyfs*5) variant of the JAG1 gene, which may lead to premature termination of translation and a truncated protein with loss of function. The variant was unreported previously. The phenotypes of the proband (cholestasis, pulmonary artery stenosis and peculiar faces) have differed from those of his elder sister (cholestasis with pruritus, posterior embryonic ring of cornea) and mother (with no clinical manifestation). Cholestasis and peculiar face of the proband became insignificant with age. CONCLUSION The c.1270dupG (p.Ala424Glyfs*5) variant of the JAG1 gene probably underlay the ALGS in this pedigree with incomplete penetrance.
Aged ; Alagille Syndrome/genetics* ; Heterozygote ; High-Throughput Nucleotide Sequencing ; Humans ; Pedigree ; Phenotype

Objective:To investigate the effect of smart air cell mattresses on sleep quality.Methods:Twenty healthy young people were enrolled as subjects, and each subject underwent a four-night polysomnographic monitoring experiment, including two nights each on a smart air cell mattress and a general mattress. The differences in sleep quality were compared by self-assessment of sleep quality, objective sleep indicators, and electroencephalogram (EEG) spectral analysis.Results:In the comparison between the smart air cell mattress and the general mattress, the differences in self-assessment of sleep quality and objective sleep indicators were not statistically significant (all P > 0.05), but the smart air cell mattress had a slight overall advantage. The relative power of EEG in the low-frequency band and the relative power of EEG in the high-frequency band were higher in the subjects with the smart air cell mattress. Conclusions:For the healthy young population, the smart air cell mattress can positively influence sleep quality to some extent, and the change in EEG relative power indicates an increase in sleep depth.