Objective     To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods     The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results     There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P<0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P>0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion     There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.

Objective    To summarize the results of surgical treatment for 79 patients with coarctation of the aorta (CoA) combined with complex anomalies (CA) in recent years. Methods    The data from 79 patients with CoA combined with CA admitted to Guangzhou Women and Children's Medical Center between January 19, 2010 and September 7, 2017 were collected and analyzed. There were 52 males and 27 females. The median age was 71 days, and the median weight was 4.3 kg. There were 26 patients combined with tracheostenosis and 7 patients with preoperative tracheal intubation. Extended end-to-side anastomosis was used to correct the CoA, and the associated cardiac abnormalities were treated simultaneously by an incision through median sternotomy. All operations were performed by the same group of surgeons. Results    The median deep hypothermic circulatory arrest was 18 (13-28) minutes, the median aorta cross-clamp time was 62 (15-199) minutes, the median cardiopulmonary bypass time was 145 (71-674) minutes, the median ventilation time was 72 (9-960) hours, the median length of ICU stay was 144 (12-1 944) hours, and the median length of hospital stay was 24 (2-93) days. Early death occurred in 9 patients and late death occurred in 5 patients. Reoperation occurred in 28 patients and recoarctation developed in 10 patients. After operation, transcoarctation gradient was reduced, and the transcutaneous oxygen saturation, C-reactive protein (CRP), lactate and creatinine were increased. Compared to the survival group, both preoperative and postoperative transcutaneous oxygen saturation were worse and the postoperative levels of plasma brain natriuretic peptide (BNP) and CRP were higher in the death group. Conclusion    Correcting the CoA and the associated CA simultaneously with extended end-to-side anastomosis by an incision through median sternotomy is effective and safe, and the outcomes of immediate and medial-term are satisfactory. Improving oxygenation and limiting elevation of BNP and CRP levels may reduce the death rate.

Pregnancy ; Female ; Humans ; Mucolipidoses ; Placenta

Objective: To clarify the values of autotaxin (ATX) in patients with primary biliary cholangitis (PBC) and PBC-related hepatocellular carcinoma (HCC). Methods: 179 patients with PBC were selected from prospective cohorts of autoimmune liver diseases at the time of first diagnosis of PBC in Department of Hepatology, the Fifth Medical Center of PLA General Hospital, from January 2016 to January 2018, all patients with PBC received UDCA therapy, primary endpoint was event of HCC, the follow-up period was censored at the date of HCC. The relationship between level of ATX and clinical features in patients with PBC and its potential value in predicting disease progression and PBC-related HCC were analyzed. Results: The ATX level in the peripheral blood of patients with PBC was significantly higher than that of alcoholic liver cirrhosis(ALC) (t = 3.278, P = 0.001) and healthy controls(HC) (t = 6.594, P < 0.001), however, when comparing PBC to non-PBC related HCC, no significant difference was found between the groups(t=-0.240, P = 0.811). Consistent with peripheral blood levels, histochemical staining indicated that ATX in the liver of patients with PBC was significantly higher than that of HC (Z=-3.633, P < 0.001) and ALC (Z=-3.283, P < 0.001), and the expression of ATX in PBC with advanced histological stage was significantly higher than PBC with early stage (Z=-2.018, P = 0.034). The baseline ATX level in PBC patients without developing to HCC during follow-up had significant difference to patients with developing to HCC (228.451 ± 124.093 ng/ml vs 301.583 ± 100.512 ng/ml, t = 2.339, P = 0.021). The result in multivariate logistic regression analysis showed that ATX were independent predictors of PBC related HCC(OR 1.245, 95%CI 1.097-1.413). The optimal critical value of peripheral blood ATX level at baseline for predicting HCC was 235.254 ng/ml, with the cut-off value of 0.714 in AUC of the ROC (95% CI was 0.597~ 0.857), sensitivity and specificity were 84.6% and 59.0%, respectively. Conclusion: ATX level was significantly higher in PBC patients over controls, and it's concentration was correlated with UDCA efficacy and fibrosis stage. ATX has potential values in predicting disease progression and PBC-related HCC.

Objective: To understand the dairy product intake in residents of Shanghai, its seasonal changes and the influencing factors. Methods: A group of representative residents was surveyed and followed up in four seasons in Shanghai during 2012-2014. Information of the dairy product intake was collected by food frequency questionnaire. Results: Liquid milk and yogurt were the main dairy products consumed by the residents of Shanghai. The annual dairy product intake rate was 41.8%, and the median of dairy intake of milk was 145.3 g/d, accounting for 48.4% of reference dietary intake value, and only 4.5% of people's diary product intake reached intake reference value. The area specific differences in intake rate of liquid milk, yogurt and total dairy product intake were significant in four seasons and in whole year (P<0.05). The area specific differences in total dairy product intake were significant (P<0.05) in spring, summer and winter. In general, it was shown that dairy product intake in urban area was higher than that in suburban area and in rural area. The data of Shanghai and urban area showed that the total dairy product intake was significant different among four seasons (P<0.05), but no significant difference was observed among four seasons in suburban and rural areas. The logistic regression analysis results showed that people with average household income higher than local average level or higher education levels were more likely to consume dairy products daily. People who reported to have good health status or sleep quality were more likely to drink milk compared to those who have worse health status or sleep quality (P<0.05). Conclusions: Even though dairy product intake rate and volume have been improved in residents in Shanghai, it is still stay at lower level, especially in rural area. To promote consumption of dairy products, it is necessary to control diary product price from macro perspective, and strengthen healthy education about the importance of dairy product intake and guide residents to select suitable dairy products.
Animals ; China ; Dairy Products ; Diet ; Humans ; Milk ; Seasons ; Surveys and Questionnaires

Objective: To analyze dietary pattern and its seasonal characteristic in residents of Shanghai. Methods: A representative sample was surveyed and followed up in four different seasons during 2012-2014. Information of food consumption was collected by 3-day 24-hour dietary recall and condiments weighing method. The intake of energy and macronutrients were calculated according to the Chinese Food Composition Table. Results: Annual average daily intake was 191.09 g for grain, 250.69 g for vegetable, 107.23 g for fruit, 223.53 g for animal food, 96.39 g for dairy products, 11.19 g for soy bean and its product, 36.54 g for cooking oil, and 7.57 g for salt. Significant differences were observed in food consumption among different seasons (P<0.05). Annual average daily intake of energy was 2 048.70 kcal, carbohydrates was 241.04 g, protein was 82.35 g, fat was 85.99 g and proportion of energy contributed by fat was 37.99%. Different seasons, age, residential areas, and income were influence factors for food consumption (P<0.05). Conclusions: Energy intake was adequate in residents of Shanghai. Unhealthy dietary pattern, including high proportion of fat and consumption of cooking oil and salt (lower than average intake of large cities, but higher than recommendation), was observed. It is necessary to take appropriate nutrition intervention, and future researches on dietary intake should consider seasonal influences.
China ; Cities ; Diet ; Energy Intake ; Feeding Behavior ; Humans ; Seasons

Shanghai Diet and Health Survey (SDHS) was designed to prospectively access local residents' food consumption, energy and nutrient intake, related chemical contaminant exposure, and the seasonal change trend to explore the relationship of diet with health. Data from SDHS can be used as fundamental information and scientific evidences for the development of local nutrition and food safety policies.
China ; Diet ; Energy Intake ; Health Surveys ; Nutrition Policy ; Nutrition Surveys