Hirschsprung′s disease( HD) ,caused by the dysplasia of enteric neural crest cells during em-bryonic stage,is a multifactorial disorder with several genes or gene signaling pathways involved in its pathogen-esis. To date,at least 11 genes have been found to be associated with the pathogenesis of HD. The abnormal expression or interaction of these genes may lead to the displasia of neural crest cells,which may result in HD. Current knowledge of the molecular genetics underlying HD based on human and animal studies are reviewed.

Objective To explore the surgical intervention outcomes of necrotizing enterocolitis (NEC)pa-tients with different extent of the disease.Methods The data of 25 pediatric patients with NEC who were treated with surgical intervention in Shanghai Children′s Hospital from December 201 1 to December 201 5 were retrospectively ana-lyzed.According to the extent of the disease,the patients were divided into 3 groups:focal disease(F),multisegmental disease(M),and pan -involvement(P).The information including operation style,survival rate and time for close osto-my was analyzed.Results There were 1 1 cases with F,8 cases with M,and 6 cases with P.All patients received lapa-rotomy surgery,colostomy,or peritoneal drainage.There were 1 2 patients with very low birth weight,7 patients with low birth weight,6 patients with normal birth weight in this study.There were 1 7 cases with gastrointestinal perforation (9 cases with pneumoperitoneum,8 cases without pneumoperitoneum),8 cases without digestive tract perforation (4 cases without pneumoperitoneum,4 cases with enterostenosis after conservative treatment).In this study,close ostomy was commonly conducted 3 -6 months after the operation,except for 3 cases who received 2 or more times of operation.The survival rate in F group was 1 00.0%(1 1 /1 1 cases),higher than those in the Mgroup with 62.5%(5 /8 cases)and P group with 1 6.7%(1 /6 cases)(χ2 =4.898,1 0.31 2,all P <0.05).However,there was no difference between Mgroup and P group (χ2 =1 .367,P >0.05).Conclusions The extent of disease is correlated to the outcomes of surgical in-tervention,as F had a better outcome than Mand P.Low birth weight is a risk factor for NEC.Protecting the edge of the bowel is a key factor to ensure the survival and improve the quality of life of NEC patients.Close ostomy should be con-sidered when the patients are in a stable condition (liver function and intestinal function recovery,good nutrition condi-tion,etc),and under special circumstances to conduct early or delayed closure of fistula.

Objective:To investigate the effect of abnormal D-dimer level on the prognosis of patients with frontal lobe injury, and to provide a new idea for clinical diagnosis, treatment and prognosis evaluation of frontal lobe injury.Methods:A retrospective analysis was performed on 71 patients with traumatic brain injury (TBI) who were admitted to the Surgical Care Unit of the First Affiliated Hospital of Zhejiang University School of Medicine from November 1, 2020 to February 1, 2021. According to whether TBI involved in the frontal lobe, the patients were divided into the experimental group ( n = 44) and the control group ( n = 27). Clinical data including sex, age, body mass index (BMI), length of hospital stay, ISS trauma score, GCS coma score, qSOFA score, GOS prognostic score and coagulation index test results within 24 h of admission of the two groups were recorded, and the difference of each index between the two groups was compared. Results:① D-dimer levels were significantly higher in the experimental group than in the control group ( P<0.01); ② The incidence of complications in the experimental group was 40.9%, of which the incidence of lower limb vein thrombosis was 18.2%, and the incidence of complications in the control group was 18.5%, of which the incidence of lower limb vein thrombosis was 3.7%; there were statistical differences between the two groups ( P<0.05); ③ The average hospital stay of patients was 10.96 days in the control group, and 15.50 days in the experimental group with a statistically significant difference between the two groups ( P<0.05); ④ The 28-day mortality rate of patients in the experimental group was 18.2% and 7.4% in the control group. There was no significant difference in 28-day mortality between the two groups ( P>0.05); ⑤ The level of D-dimer was negatively correlated with GOS prognostic score in patients with frontal lobe injury ( r=-0.501, P<0.01). Conclusions:Patients with frontal lobe injury have increased D-dimer levels, increased incidence of complications, and longer hospital stay. There is a significant negative correlation between D-dimer level and prognosis in patients with frontal lobe injury. D-dimer can be used as a sensitive indicator to evaluate the prognosis of patients with frontal lobe injury. The higher the D-dimer level, the worse the prognosis of patients.