Objective To summarize the diagnosis and surgical treatment of mesenteric cyst in children.Methods The clinical records of mesenteric cyst cases from January 2011 to December 2015 were reviewed retrospectively.The diagnosis and treatment options were analyzed, and the prognosis of laparoscopic surgery and laparotomy was compared.Results The main clinical symptoms included abdominal mass, abdominal pain, and abdominal distension.Abdominal ultrasound and/or CT scan were the diagnostic tools in all cases.Traditional laparotomy was performed in 14 cases, while laparoscopy in 7 cases (1 case switched to laparotomy).2 cases had emergency surgery due to acute abdomen, laparotomy and laparoscopy in each case.Simple cyst resections were completed in 14 cases, of which 2 cases with a small amount of residual in the mesenteric root.Intestinal resection and anastomosis were required in other 7 cases.The average time of hospital stay for laparotomy group was 12 days, and 10.14 days for laparoscopy group.There was no significant difference.All patients were discharged without postoperative complications.With 1-4 years follow-up, there was no recurrence.Conclusion The operation for mesenteric cysts depends on the relationship between the cyst and the adjacent bowel or organs, and the overall outcome is favorable.The selective use of laparoscopy will benefit more children.

[Objective] To explore the clinical features,genetic characters in family amyotrophic lateral sclerosis (ALS10)patients.[Methods] TARDBP gene mutations in Chinese Han family patients with ALS10 diagnosed by the First Affiliated Hospital of Sun Yat-sen University in 2013 was screened by high-throughput sequencing.[Results] There were 5 patients in three generations in this family.The initial symptoms in all affected members were distal limb muscle weakness and dystrophy at their 50 age.With a rapid progression of symptoms about 8 to 18 months.A homozygous missense mutation (c.892G＞A) were detected in TARDBP gene exon 6 of the propositus,as well as the other three family members without any clinical symptoms.[Conclusion] ALS10 is a faster progressive and shorter survival time FALS.Since there was no effective treatment in ALS10,hereditary consultation and prenatal diagnosis play an important role in disease prevention and hereditary.

Objective: To analyze the association between each regional fat mass and total body bone mineral density (BMD) in children and adolescents aged 7-17 years in Beijing, so as to provide theoretical basis and practical guidance for implementing interventions. Methods: From September to December 2020, a stratified cluster random sampling method was used to select 1 423 children and adolescents aged 7-17 years in Tongzhou District, Beijing. Dual energy X-ray absorptiometry (DXA) was employed to measure regional body composition and total body BMD. Multiple linear regression was used to analyze the association between regional fat mass and total body BMD. Results: The median (interquartile range) fat mass values for total body, upper limbs, abdomen, hips, and thighs were 13.51(8.84, 19.21), 1.59(1.08, 2.23), 0.73(0.39, 1.29), 2.32(1.46, 3.26), 5.29(3.59, 7.21)kg, respectively. After adjusting for covariates, the results of multiple linear regression analysis showed that total body fat mass (β=0.010), abdominal fat mass (β=-0.100), and hip fat mass (β=0.104) were significant associations with total body BMD (all P<0.01). Sexstratified analysis revealed that in boys, total body fat mass (β=0.008) and hip fat mass (β=0.058) were positively associated with BMD, while thigh fat mass (β=-0.038) showed a negative association with total body BMD (all P<0.05). In girls, total body fat mass (β=0.013), hip fat mass (β=0.163), and thigh fat mass (β=0.023) were positively associated with total body BMD, whereas abdominal fat mass (β=-0.196) showed a negative association with total body BMD (all P<0.05). Among children and adolescents with body fat percentage below the standard range, within the standard range and above the standard range, total body fat masses were positively associated with total body BMD (β=0.021, 0.016, 0.015); among children and adolescents with body fat percentage within the standard range while upper limb (β=-0.042), abdominal (β=-0.067), and thigh fat mass (β=-0.018) showed negative associations with total body BMD, and hip fat mass demonstrated a positive association with total body BMD (β=0.082) (all P<0.05). Conclusion Regional fat distribution is associated with total body BMD in children and adolescents, with the nature of these associations varying by sex and body fat percentage.

OBJECTIVETo explore the significance of SMN1 gene mutations among patients with spinal muscular atrophy (SMA) and the value of multiplex ligation dependent probe amplification (MLPA) for its diagnosis.

METHODSPotential mutations of the SMN1 gene were detected among 78 SMA patients with a MLPA assay.

RESULTSHomozygous deletion of SMN1 exons 7 and 8 was detected in 70 (89.7%) of all patients. Homozygous deletion of exons 7 and heterozygous deletion of exon 8 was detected in 3 patients (3.8%). Homozygous deletion of SMN1 exons 7 alone was detected in 3 patients (3.8%). Heterozygous deletion of SMN1 exons 7 and 8 was detected in 2 patients (2.6%). For 77 of the patients, both parents were found to carry heterozygous deletion of the SMN1 gene, which was consistent with the recessive inheritance of SMA. One patient with SMA type I was found to be rather rare. The patient was found to carry homozygous deletion of SMN1 exons 7 and 8, for which her mother was heterozygous, while no mutation was found in her father.

CONCLUSIONHomozygous deletion of the SMN1 gene have been detected in more than 95% of SMA patients. No homozygous deletion of exon 8 has been found. Homozygous deletion of exon 7 is more significant in the pathogenesis of SMA.

Exons ; Female ; Gene Deletion ; Humans ; Male ; Multiplex Polymerase Chain Reaction ; Muscular Atrophy, Spinal ; genetics ; Mutation ; Survival of Motor Neuron 1 Protein ; genetics

Objective To investigate the mechanism of MDM2-p53 signaling pathway in the development of colorectal cancer and correlation between p53 with clinicopathological parameters, so as to further analyze the effect of p53 on prognosis. Methods The colorectal cancer tissues and the adjacent normal tissues from 86 cases of patients with colorectal cancer were collected . The expression of p53 and murine double minute 2 (MDM2) in colorectal cancer and adjacent normal tissues were detected by immunohistochemistry, Western Blot and real-time fluorescence quantitative polymerase chain reaction (RT-PCR). The prognosis of the patients was analyzed by the Kaplan-Meier survival curves. Results The protein expression and the mRNA expression of p53 and MDM2 in colorectal cancer tissues were significantly higher than that in the adjacent non-cancerous tissues (all P<0.01). A positive correlation was observed between the expression of p53 and MDM2 (r=0.785). The expression of p53 in colorectal cancer tissues were correlated well with the degree of tumor differentiation, TNM stage, lymph node metastasis and infiltration depth (all P<0.05). Survival analysis demonstrated that the mean overall survival time in p53 high expression group was (53.92±1.56) months which was significantly lower than that in p53 low expression group of (69.16±3.72) months, and the difference was statistically significant (χ2=14.78, P<0.01). Conclusions The risk and prognosis of colorectal cancer are closely related to the MDM2-p53 signaling pathway. p53 can be used as a potential target for the prognosis and treatment of colorectal cancer.

Objective To summarize the etiology,diagnosis and treatment of three cases of duodenal perforation. Methods The data of 3 cases of children with duodenal perforation in our hospital from September 14,2016 to June 20,2017 were retrospectively analyzed. The causes,clinical features and treatment of children's duodenal perforation were summarized. Results A total of 2 males and 1 females aged 2 years,3 years and 5 years were included in the 3 cases. All 3 cases had fever history of upper respiratory tract infection before onset. Acute abdominal pain occurred after oral administration of ibuprofen several times,and all the abdominal plain films prompted pneumoperitoneum. Three cases of perforation sites were duodenal anterior wall,diameter were 0. 5-1 cm. Two cases of small amount of pneumoperitoneum were explored and repaired the duodenal perforation by 3D laparoscopic,1 case underwent laparotomy to repair the duodenal perforation due to a large number of liquid pneumoperitoneum and severe shock. Repair of 3 cases were covered with omentum. Three cases were all cured without anastomotic fistula, ulcer, adhesive intestinal obstruction or other complications. Followed-up in the department of gastroenterology,3 cases had no Helico-bacter pylori infection. Conclusion Repeatedly oral administration of ibuprofen can cause duodenal perfora-tion in children in the short term. Once the digestive tract perforation confirmed,emergency surgical explora-tion is needed. Laparoscopic repair of duodenum perforation is safe and effective and may have a faster recov-ery. We can choose exploratory laparotomy if conditions are not allowed.

Objective: To evaluate the methodological quality of papers that performed meta-analyzed and systematically reviewed acupoint selections for the treatment of functional dyspepsia (FD) and to identify the ideal acupoint combinations for FD. Methods: Chinese databases including China National Knowledge Infrastructure (CNKI), China Science and Technology Journal Database (VIP), China Biology Medicine (CBM), and Wanfang Database, as well as English databases including PubMed, Embase, and Cochrane Library were searched to retrieve papers about meta-analysis and systematic literature reviews on acupuncture for FD. The time span for the paper retrieval was set from the foundation of the databases to April 30, 2022. The Veritas scores of the papers based on their publication year, study type, Assessment of Multiple Systematic Reviews 2 (AMSTAR2), Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), heterogeneity, and publication bias were rated to assess the methodological quality of the included studies. Then, randomized controlled trials (RCTs) were extracted from those meta-analysis papers or systematic literature reviews for analyzing acupoints frequency, meridian frequency, and association rules with the use of R software (V 4.3.1). Results: Eight meta-analysis papers were included in the study after screening. The mean Veritas scores of the papers based on publication year, type of study, AMSTAR2, PRISMA, heterogeneity, and publication bias were 4.50, 8.00, 4.63, 4.63, 4.50, and 6.13, respectively. The analysis of the scores revealed insufficiencies in the reviews pertaining to the methodology, comprehension of the research strategy, detailed list of excluded studies, sources of funding, assessment of potential bias risks impact on meta-analysis results in each study, explanation of heterogeneity, and identification of potential conflicts of interest. Furthermore, a total of 85 RCTs were obtained from the eight meta-analysis papers involving 85 acupuncture prescriptions and 67 acupoints for subsequent data mining. The most commonly used meridian was Stomach meridian of Foot-Yangming (ST). Zusanli (ST36), Neiguan (PC6), Zhongwan (CV12), Taichong (LR3), Tianshu (ST25), Gongsun (SP4), Weishu (BL21), Pishu (BL20), Neiting (ST44), and Yinlingquan (SP9) topped the list of frequently selected acupoints. Additionally, a total of 28 association rules were identified, including 10 second-order, 15 third-order, and 3 fourth-order association rules. The top-ranking association rules in each order were “Neiguan (PC6) → Zusanli (ST36)” “Zhongwan (CV12) + Neiguan (PC6) → Zusanli (ST36)” and “Zhongwan (CV12) + Taichong (LR3) + Neiguan (PC6) → Zusanli (ST36)”, respectively. Conclusion Acupuncture could alleviate the clinical symptoms of FD. However, the quality of methodology applied in the meta-analysis papers on the subject needs to be improved. Through data mining, a combination of Neiguan (PC6), Zusanli (ST36), Zhongwan (CV12), and Taichong (LR3) was identified as an essential acupoint combination for the treatment of FD.

OBJECTIVETo discuss the distribution of the respiratory complications in severely burned patients and the prevention and treatment experience against them.

METHODSMedical records of 922 adult patients with severe or extremely severe burn hospitalized in our burn ICU from January 2005 to December 2012 were screened and retrospectively analyzed, including patients transferred from other hospitals, patients with total burn area above 50% TBSA, the distribution and treatment of respiratory complications, and the mortality. Data were processed with chi-square test.

RESULTSThe constituent ratio of patients transferred to our hospital was 71.1% in 2007 and 40.2% in 2010, while it remained about 50.0% in the other years. The ratios of patients with total burn area larger than 50% TBSA and that of patients with respiratory complications (χ(2) = 2.637, P > 0.05) showed no significant changes each year. Among these 922 burn patients, 523 patients suffered respiratory complications, among which laryngeal edema (50.9%, 266 cases), pulmonary infection (21.6%, 113 cases), and ARDS (11.9%, 62 cases) were the main components, with no significant change each year (with χ(2) values respectively 6.132, 6.319, 0.016, P values above 0.05). Among the patients with respiratory complications, except for 36 were not treated actively, 487 were treated by ventilator among which 228 had undergone tracheostomy, and the constituent ratios in the 8 years were close. Fifteen patients died, with 2 died of laryngeal edema, 3 of ARDS, and 10 of sepsis or MODS as a result of sepsis.

CONCLUSIONSPatients with severe burns were at high risk of respiratory complications, among which laryngeal edema was common, followed by pulmonary infection and ARDS. Prophylactic tracheostomy, mechanical ventilation, wound therapy, and anti-infection were all effective measures of prevention and treatment against these complications.

Adult ; Aged ; Burns ; complications ; therapy ; Humans ; Laryngeal Edema ; etiology ; physiopathology ; therapy ; Lung ; physiopathology ; Respiration, Artificial ; Respiratory Distress Syndrome, Adult ; etiology ; physiopathology ; therapy ; Retrospective Studies ; Sepsis ; etiology ; physiopathology ; therapy ; Treatment Outcome