Objective:To investigate the imaging characteristics of fibrous dysplasia(FD) in children with McCune-Albright syndrome(MAS) and the correlation between FD severity and bone metabolism markers, so as to provide a basis for clinical diagnosis and treatment.Methods:A total of 46 children(38 females and 8 males) with MAS with FD who were admitted to the Department of Pediatrics of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from January 2010 to December 2016 were included in the retrospective study, and all of them met the diagnostic criteria for either the MAS triad or dual manifestations. The extent and characteristics of FD lesions were evaluated by imaging analysis(X-ray and CT). The distribution of café-au-lait spots and endocrine abnormalities were recorded. The serum bone metabolism levels [total procollagen type 1 amino-terminal propeptide(TP1NP), osteocalcin, β-C-terminal telopeptide(β-CTX), alkaline phosphatase(ALP)], and other related indicators such as calcium, phosphorus, magnesium, and fibroblast growth factor(FGF23) levels were detected, and the association between FD severity and indicators was evaluated by Spearman correlation analysis.Results:Among the 46 children, there were 24 cases of triad(FD+ café-au-lait spots + precocious puberty) and 22 cases of dual manifestations(11 cases of FD+ café-au-lait spots or precocious puberty). The age of onset of FD patients(24 cases) with bilateral long bones and skull FD was significantly earlier than that in the unilateral FD group [(3.33±1.34)years vs(5.26±2.34)years, P<0.01], and all of them had extensive café-au-lait spots across the midline. Polyostotic FD accounted for 71.7%(33/46), mainly cystic expansive lesions involving the femur(30 cases) and tibia(24 cases), and skull FD(25 cases) mostly showed ground-glass changes; Monostotic FD(13 cases) was more common in the skull(5 cases) and phalanges(5 cases). FD severity was significantly positively correlated with ALP( ρ=0.554, P=0.002), and negatively correlated with serum phosphorus( ρ=-0.522, P=0.006). All 6 children with severe fractures had FGF23-mediated hypophosphatemia [(1.03±0.12) mmol/L vs control(1.52±0.15) mmol/L, P=0.003]. Conclusions:Extensive café-au-lait spots(across the midline) in children with MAS are strongly associated with early-onset polyostotic FD; FD severity was strongly associated with bone turnover markers(TP1NP, β-CTX, ALP) and FGF23-mediated hypophosphatemia. Early comprehensive skeletal assessment and regular FGF23 monitoring are recommended for children with MAS presenting with extensive cutaneous café-au-lait spots.

Aim To clarify the mechanism by which Qishen Yixin Granules improved microcirculation vas-cular endothelial dysfunction(VED)in mice,through activating the Nrf2/HO-1 signaling pathway to regulate oxidative stress.Methods C57 mice were randomly divided into six groups:blank group,model group,pos-itive drug group,and low-,medium-,and high-dose groups of Qishen Yixin Granules.The VED model was established by long-term infusion of Ang Ⅱ combined with a high-fat diet.Each treatment group received the corresponding drug intervention.After four weeks of drug intervention,cardiac function was assessed by echocardiography.Carstairs staining was used to ob-serve the formation of microthrombi in myocardial tis-sue.The micro vascular ischemia was evaluated by Hei-denhain staining.The ultrastructure of endothelial cells was observed by electron microscopy.The levels of EMPs,ROS,NO,ET-1,TF,TM,VWF,and TXA2 in serum were measured by ELISA.The expression levels of MDA,SOD,and GSH-Px in mouse heart tissue were determined by chemical methods.Cardiac microvascu-lar density and the expression of Nrf2,Keap1,and HO-1 proteins were detected by Immunohistochemical stai-ning.The protein expressions of Keap1,cytoplasmic Nrf2,nuclear Nrf2,and HO-1 in myocardial tissue were detected by Western blot.Results Qishen Yixin Granules could effectively improve the cardiac function of mice,alleviate the damage of endothelial cells and endothelial function.They could up-regulate serum NO levels and the activities of antioxidant enzymes SOD and GSH-Px,while down-regulating the expression of ROS and vascular inflammatory injury factors such as ET-1,VWF,TXA2,TF,TM,and EMPs.Qishen Yixin Granules also increased the positive counts of CD34,Nrf2,and HO-1,as well as microvessel density.Fur-thermore,they inhibited the expression of MDA,Keap1,and cytoplasmic Nrf2 protein in myocardial tis-sue,while increasing the expression of nuclear proteins HO-1 and Nrf2.Conclusions Qishen Yixin Granules may inhibit oxidative stress and inflammatory response by regulating the Nrf2/HO-1 signaling pathway,thereby improving vascular endothelial damage and cardiac function in VED mice.

Aim To clarify the mechanism by which Qishen Yixin Granules improved microcirculation vas-cular endothelial dysfunction(VED)in mice,through activating the Nrf2/HO-1 signaling pathway to regulate oxidative stress.Methods C57 mice were randomly divided into six groups:blank group,model group,pos-itive drug group,and low-,medium-,and high-dose groups of Qishen Yixin Granules.The VED model was established by long-term infusion of Ang Ⅱ combined with a high-fat diet.Each treatment group received the corresponding drug intervention.After four weeks of drug intervention,cardiac function was assessed by echocardiography.Carstairs staining was used to ob-serve the formation of microthrombi in myocardial tis-sue.The micro vascular ischemia was evaluated by Hei-denhain staining.The ultrastructure of endothelial cells was observed by electron microscopy.The levels of EMPs,ROS,NO,ET-1,TF,TM,VWF,and TXA2 in serum were measured by ELISA.The expression levels of MDA,SOD,and GSH-Px in mouse heart tissue were determined by chemical methods.Cardiac microvascu-lar density and the expression of Nrf2,Keap1,and HO-1 proteins were detected by Immunohistochemical stai-ning.The protein expressions of Keap1,cytoplasmic Nrf2,nuclear Nrf2,and HO-1 in myocardial tissue were detected by Western blot.Results Qishen Yixin Granules could effectively improve the cardiac function of mice,alleviate the damage of endothelial cells and endothelial function.They could up-regulate serum NO levels and the activities of antioxidant enzymes SOD and GSH-Px,while down-regulating the expression of ROS and vascular inflammatory injury factors such as ET-1,VWF,TXA2,TF,TM,and EMPs.Qishen Yixin Granules also increased the positive counts of CD34,Nrf2,and HO-1,as well as microvessel density.Fur-thermore,they inhibited the expression of MDA,Keap1,and cytoplasmic Nrf2 protein in myocardial tis-sue,while increasing the expression of nuclear proteins HO-1 and Nrf2.Conclusions Qishen Yixin Granules may inhibit oxidative stress and inflammatory response by regulating the Nrf2/HO-1 signaling pathway,thereby improving vascular endothelial damage and cardiac function in VED mice.

Objective:To investigate the imaging characteristics of fibrous dysplasia(FD) in children with McCune-Albright syndrome(MAS) and the correlation between FD severity and bone metabolism markers, so as to provide a basis for clinical diagnosis and treatment.Methods:A total of 46 children(38 females and 8 males) with MAS with FD who were admitted to the Department of Pediatrics of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from January 2010 to December 2016 were included in the retrospective study, and all of them met the diagnostic criteria for either the MAS triad or dual manifestations. The extent and characteristics of FD lesions were evaluated by imaging analysis(X-ray and CT). The distribution of café-au-lait spots and endocrine abnormalities were recorded. The serum bone metabolism levels [total procollagen type 1 amino-terminal propeptide(TP1NP), osteocalcin, β-C-terminal telopeptide(β-CTX), alkaline phosphatase(ALP)], and other related indicators such as calcium, phosphorus, magnesium, and fibroblast growth factor(FGF23) levels were detected, and the association between FD severity and indicators was evaluated by Spearman correlation analysis.Results:Among the 46 children, there were 24 cases of triad(FD+ café-au-lait spots + precocious puberty) and 22 cases of dual manifestations(11 cases of FD+ café-au-lait spots or precocious puberty). The age of onset of FD patients(24 cases) with bilateral long bones and skull FD was significantly earlier than that in the unilateral FD group [(3.33±1.34)years vs(5.26±2.34)years, P<0.01], and all of them had extensive café-au-lait spots across the midline. Polyostotic FD accounted for 71.7%(33/46), mainly cystic expansive lesions involving the femur(30 cases) and tibia(24 cases), and skull FD(25 cases) mostly showed ground-glass changes; Monostotic FD(13 cases) was more common in the skull(5 cases) and phalanges(5 cases). FD severity was significantly positively correlated with ALP( ρ=0.554, P=0.002), and negatively correlated with serum phosphorus( ρ=-0.522, P=0.006). All 6 children with severe fractures had FGF23-mediated hypophosphatemia [(1.03±0.12) mmol/L vs control(1.52±0.15) mmol/L, P=0.003]. Conclusions:Extensive café-au-lait spots(across the midline) in children with MAS are strongly associated with early-onset polyostotic FD; FD severity was strongly associated with bone turnover markers(TP1NP, β-CTX, ALP) and FGF23-mediated hypophosphatemia. Early comprehensive skeletal assessment and regular FGF23 monitoring are recommended for children with MAS presenting with extensive cutaneous café-au-lait spots.

Objective:To evaluate the efficacy and safety of triamcinolone acetonide-saline submucosal injection for prevention of esophageal stricture after endoscopic submucosal dissection (ESD) for extensive superficial esophageal neoplasms.Methods:A total of 75 patients who underwent ESD for superficial esophageal neoplasms involving larger than 2/3 of the esophageal circumference at the Department of Gastroenterology, Northern Jiangsu People's Hospital from January 2018 to December 2020 were enrolled. Patients were randomly assigned to triamcinolone acetonide-saline submucosal injection group (group A, n=25), triamcinolone acetonide injections immediately after ESD group (group B, n=25) and the control group undergoing only ESD (group C, n=25). Serial gastroscopy was performed to assess wound healing and esophageal stricture. Endoscopic balloon dilatation (EBD) was performed when patients experienced esophageal stricture. The completion of ESD, time of operation, the amount of triamcinolone acetonide, the incidences of esophageal stricture and the time of EBD treatment of the three groups were compared. Results:All ESD procedures were successfully performed without complications such as intraoperative perforation, massive bleeding or postoperative delayed perforation. The operation time of group A, B and C were 72.87±12.99 min, 94.15±14.22 min and 74.08±11.86 min, respectively, with significant difference ( F=20.925, P<0.001). In pairwise comparison the above indicator in group A and group C was significantly shorter than that of group B (LSD- t=5.759, P<0.001; LSD- t=5.432, P<0.001), but there was no difference between group A and group C (LSD- t=0.327, P=0.745). There was no significant difference in the amount of triamcinolone acetonide between group A and group B (125±15 mg VS 133±19 mg, t=1.673, P=0.101). The rates of wound healing under endoscopy after 1 month of group A, B and C were 76% (19/25), 84% (21/25), and 76% (19/25), respectively, with no significant difference ( χ2=0.636, P=0.728). The esophageal stricture rates were 52% (13/25) in both group A and B, and 84% (21/25) in group C, with significant difference among the three groups ( χ2=7.295, P=0.026), and group A and B showed a significantly lower stricture rate than that of group C ( P=0.015; P=0.015). The median time of EBD treatment of group A, B and C were 4 (range 0 to 9), 5 (range 0 to 13) and 9 (range 0 to 16), respectively, with significant difference ( H=17.58, P<0.001). In pairwise comparison the above indicator in group A and B was significantly less than that of group C ( H=23.96, P<0.001; H=19.00, P=0.002), but there was no significant difference between group A and group B ( H=4.96, P=0.407). Esophageal stricture was observed in all patients with circumferential mucosa resected in the three groups. But the times of EBD treatment were 6.90±1.10 in group A, 10.13±2.42 in group B and 15.29±0.76 in group C with significant difference ( F=57.754, P<0.001). In pairwise comparison the above indicator in group A was less than that in group B (LSD- t=4.294, P<0.001) and this indicator in group B was less than that in group C (LSD- t=6.294, P<0.001). Median EBD times in patients with non-circumferential mucosal defects in the three groups were 0 (range 0 to 9), 0 (range 0 to 6) and 8 (range 0 to10), respectively, with significant difference ( H=19.72, P<0.001). In pairwise comparison, the EBD time in group A and B was less than that in group C ( H=17.93, P<0.001; H=16.62, P<0.001), but there was no statistical difference between group A and B ( H=1.31, P=0.779). Conclusion:Triamcinolone acetonide-saline submucosal injection ESD can safely and effectively prevent esophageal stricture after ESD for large-area superficial esophageal neoplasms, reduce the operation time and time of EBD treatment in patients with circumferential mucosa defect compared with local injections of triamcinolone acetonide after ESD.

Objective:To evaluate the efficiency and safety of modified endoscopic anti-reflux mucosectomy (ARMS) for refractory gastroesophageal reflux disease (rGERD) with moderate hiatus hernia.Methods:A total of 30 patients with rGERD with moderate hiatus hernia (3-5 cm) diagnosed at the Department of Gastroenterology of Northern Jiangsu People's Hospital from June 2017 to June 2020 were randomly divided into 2/3 circumferential mucosal resection group ( n=15) and 3/4 circumferential mucosal resection group ( n=15) using random number table method, and received modified ARMS of the corresponding mucosal resection range. The GERD symptoms, esophagitis under endoscopy, 24 h pH results, and lower esophageal sphincter (LES) resting pressure were compared before and after the procedure. The therapeutic effect and complications of the two groups were analyzed. Results:In 2/3 resection group, the GERD questionnaire scores (9.53±0.36 VS 11.93±0.57, t=6.874, P<0.001), acid exposure time (19.81%±1.72% VS 31.45%±2.78%, t=8.020, P<0.001) and the DeMeester score based on 24 h esophageal pH monitoring (40.98±4.55 VS 55.33±5.65, t=6.408, P<0.001) at 6 months after the treatment showed a significant reduction compared with those before. In 3/4 resection group, the GERD questionnaire scores (9.0±0.57 VS 12.47±0.68, t=8.650, P<0.001), acid exposure time (20.07%±2.19% VS 29.96%±3.00%, t=7.444, P<0.001) and the DeMeester score (33.67±3.47 VS 51.17±6.03, t=4.973, P<0.001) at 6 months after the treatment were lower than those before. There was no significant difference in the GERD questionnaire scores ( t=0.790, P=0.436), acid exposure time ( t=0.093, P=0.926) or the DeMeester score ( t=1.278, P=0.212) between the two groups at 6 months after treatment. In the two groups, there was no significant difference in the ratio of esophagitis grade C and D (10/15 VS 5/15, χ2=3.894, P=0.063; 8/15 VS 4/15, χ2=2.778, P=0.125) or LES resting pressure [3.29 (2.66,8.29) mmHg VS 3.98 (3.67,9.43) mmHg, P=0.334;5.78 (1.9,8.46) mmHg VS 5.88 (3.28,8.99) mmHg, P=0.125] before and after the treatment. No postoperative delayed bleeding or perforation was observed. The incidence of postoperative esophageal stenosis of 2/3 resection group was lower than that of the other group (1/15 VS 6/15, χ2=4.658, P=0.021). Conclusion:Modified ARMS is effective for controlling reflux symptoms and esophageal acid exposure in rGRED patients with moderate hiatus hernia (3-5 cm), but cannot significantly increase the postoperative resting pressure of LES. Compared with 3/4 circumferential mucosal resection, 2/3 circumferential mucosal resection can reduce the incidence of postoperative esophageal stenosis.

Objective: To investigate the associations between the numbers of healthy lifestyles and overweight/obesity and abdominal obesity in adult twins in Shanghai. Methods: Based on the Shanghai Twin Registry System Phase Ⅱ survey data in 2017-2018, a case-control study was conducted to analyze the association between healthy lifestyles and obesity and further adjusted for confounders by a co-twin control study. Results: A total of 7 864 adult twins (3 932 pairs) were included. In the co-twin case-control analysis for monozygotic twins, compared with participants with 0 to 2 healthy lifestyles, those with 3 and 4 to 5 healthy lifestyles had a 49% (OR=0.51, 95%CI: 0.28-0.93) and 70% (OR=0.30, 95%CI: 0.13-0.69) lower risk of overweight/obesity, respectively, and a 17% (OR=0.83, 95%CI: 0.44-1.57) and 66% (OR=0.34, 95%CI: 0.14-0.80) lower risk of abdominal obesity, respectively. For each additional healthy lifestyle, the risk of developing overweight/obesity was reduced by 41% (OR=0.59, 95%CI: 0.42-0.85), and the risk of developing abdominal obesity was reduced by 37% (OR=0.63, 95%CI: 0.44-0.90). Conclusion: An increasing number of healthy lifestyles was associated with a marked decreased risk for both overweight/obesity and abdominal obesity.
Adult ; Humans ; Case-Control Studies ; China/epidemiology* ; Healthy Lifestyle ; Obesity/epidemiology* ; Obesity, Abdominal/epidemiology* ; Overweight/epidemiology* ; Twins, Monozygotic

PURPOSE: The long-term situation of children with spinal cord injury (SCI) was investigated, and suggestions for helping them better return to the society were provided. METHODS: SCI patients less than 18 years old hospitalized in Beijing Boai Hospital from January 2011 to December 2020 were retrospectively analyzed. Information including motor function, complications, characteristic changes, self-care abilities, school attendance and social participation were collected by telephone interview and electronic questionnaire. All the answers were statistically analyzed. RESULTS: A total of 86 cases were enrolled, 77 girls and 9 boys, with a median injury age of 6 years and 2 months. The follow-up time was 3-130 months. The main cause of trauma in these children was sport injury (66.3%), the thoracic spinal cord was involved the most (91.9%), and complete SCIs accounted for the majority (76.7%). In terms of complications, children with complete SCIs were more likely to have urinary incontinence, constipation and characteristic changes (p < 0.05); whereas the incomplete SCIs often have spasticity (p < 0.05). As to the daily living abilities, children with incomplete lumbar SCIs were more capable to accomplish personal hygiene, transfer, and bathing independently than those with complete injuries, or cervical/thoracic SCIs, respectively (p < 0.05). Moreover, children older than 9 years care more able to dress and transfer independently than the youngers (p < 0.05). Wheelchair users accounted for 84.9% and more than half of them were able to propel wheelchair independently, and those who move passively in wheelchairs were mostly introverted kids (p < 0.05). Almost all (93.8%) children with incomplete injuries were able to walk independently. Most (79.1%) children continued to attending school, and 41.9% participated in interest classes. Unfortunately, 67.4% of the children spent less time playing with their peers than before the injury. CONCLUSION SCIs impair physical structures and function of children, affect their independence in daily living, and restrict school attendance and social interaction. Comprehensive rehabilitation after injury is a systematic work. Medical staff and caregivers should not only pay attention to neurological function, but also help them improve self-care abilities. It is also important to balance rehabilitation training and school work and social participation.
Male ; Female ; Humans ; Child ; Adolescent ; Follow-Up Studies ; Retrospective Studies ; Spinal Cord Injuries/complications* ; Prognosis

AIM To optimize the TLC identification method for Cirsii Herba and to study the changes in quality properties"carbonizing retains characteristics"of Cirsii Herba Carbonisata.METHODS After the improvement of thin layer plate and solvent in the TLC identification based on the Chinese Pharmacopoeia 2020 Edition for Cirsii Herba,Cirsii Herba Carbonisata had its TLC identification method and UPLC fingerprint established for the determination of its content of buddleoside and acacetin as well.RESULTS We used silica gel G plate,and the solvent of toluene-acetone-formic acid-methanol(6 ∶ 3 ∶ 0.5 ∶ 2.5)for TLC identification of Cirsii Herba.The content variations of buddleoside and acacetin in Cirsii Herba and its differently charred products were consistent with the result of the TLC identification.CONCLUSION The improved TLC identification method for Cirsii Herba is of lower cost and less solvent toxicity compared to the method in the Chinese Pharmacopoeia 2020 Edition,and can identify the changes in quality properties"carbonizing retains characteristics"of differently charred Cirsii Herba,therefore it can be used to control the quality of Cirsii Herba Carbonisata in the market.

OBJECTIVE: To evaluate the risk of reentry in HBV reactive blood donors and feasibility of HBV reentry strategy. METHODS: HBsAg⁺ or HBV DNA⁺ donors who had been quarantined for more than 6 months in Jiangsu Province could propose for reentry application. Blood samples were routinely screened by dual-ELISA for HBsAg, anti-HCV, HIV Ab/Ag, and anti- Treponema pallidum and those non-reactive ones were tested by minipool nucleic acid testing (NAT) for three times. To identify occult HBV donors, samples of NAT non-reactive were further tested by electrochemiluminescence immunoassay (ECLIA) for HBV seromarkers (including HBsAg, HBsAb, HBeAg, HBeAb, and HBcAb). Donors of only 4 ECLIA patterns were accepted to reentry, including all 5 HBV seromarkers negative, anti-HBs only but having history of hepatitis B vaccine injection, HBcAb only, HBsAb⁺ / HBcAb⁺ with HBsAb more than 200 IU/L. Additionally, the detection rate of HBV infection was compared between routine screening mode and ECLIA, as well as the reentry qualified rate of HBsAg⁺ and HBV DNA⁺ blood donors. RESULTS: From Oct. 2016 to Aug. 2019, a total of 737 HBV reactive donors had applied for reentry, including 667 HBsAg⁺ reactive and 70 HBV DNA⁺ reactive donors. Among 3 screening methods, the highest HBV detection rate (43.15%, 318/737) was observed on ECLIA, while only 4.75% (35/737) on ELISA and 3.12% (23/737) on NAT, respectively. Among 4 qualified patterns of HBV serological markers, the highest proportion was found in the all negative group (22.90%, 155/677), followed by the group with HBsAb⁺ only and history of hepatitis B vaccine injection (19.35%, 131/677), and the median concentration of HBsAb was 237.7 IU/L. The unqualified rate of HBV DNA⁺ donors was 82.86%, which was significantly higher than 47.98% of HBsAg⁺ donors. CONCLUSION Routine screening tests merely based on ELISA and NAT could miss occult HBV donors and may not be sufficient for blood safety. HBsAb concentration and vaccine injection history should be included in the evaluation of HBV reactive donors who intend to apply for reentry. There is a relatively larger residual risk of occult HBV infection in blood donors quarantined for HBV DNA reactive.
Blood Donors ; DNA, Viral ; Hepatitis B ; Hepatitis B Surface Antigens ; Hepatitis B virus/genetics* ; Humans