Outcomes of surgical intervention for necrotizing enterocolitis within different pathological range / 中华实用儿科临床杂志

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).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.

Advances in molecular genetics of Hirschsprung disease / 国际儿科学杂志

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.