检索结果-南通市图书馆

CD117 is not always positive in infantile gastrointestinal stromal tumor

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2018年第1期14卷 100-103页

作者： Jin Peng He jie xiong feng Department of Pediatric Surgery Tongji HospitalTongji Medical CollegeHuazhong University of Science and Technology1095 Jiefang AveQiaokou DistrictWuhan 430030HubeiChina

Gastrointestinal stromal tumors (GISTs) are rare in infants. GIST was presumed to originate from the interstitial cells of Cajal (ICC), which has been proven by their immu-nophenotype (CD117 positive). About 95% of GISTs express CD117 and 80% of GISTs also express CD34. They locate mostly in the stomach or the small intestine and usu-ally cause obstruction (1)The UK National Registry of Childhood Tumors reported the incidence of GISTs at 0.02 per million per year in children below the age of 14 years (2)Here we will discuss a very rare case of infantile GIST with negative CD117 expression and make a review of lit-eratures about its clinical, histopathological, and prognos-tic findings.

关键词： CD117 infantile gastrointestinal stromal tumor Gastrointestinal stromal tumors (GISTs)

Clinical Features and Genetic Analysis of Pediatric Patients with Alagille Syndrome Presenting Initially with Liver Function Abnormalities

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Current Medical Science 2018年第2期38卷 304-309页

作者： Yan LIU Hong WANG Chen DONG jie-xiong feng Zhi-hua HUANG Department of Pediatrics Tongji Hospital Tongji Medical College Huazhong University of Science and Technology Wuhan 430030 China Genetic Diagnostic Centre Department of Internal Medicine Tongji Hospital Tongji Medical College Huazhong University of Science and Technology Wuhan 430030 China Department of Pediatric Surgery Tongji Hospital Tongji Medical College Huazhong University of Science and Technology Wuhan 430030 China

Alagille syndrome （AGS） is a multisystem disorder and caused by mutations in JAG1 or NOTCH2 gene. The diagnosis of AGS is hampered by its highly variable clinical manifestations. We performed a retrospective analysis on 16 children diagnosed as having AGS in recent five years in our hospital. Cholestasis was seen in 15 patients （93.8%）, heart disease in 12 （75%）, characteristic facies in 7 （43.8%）, and butterfly vertebrae in 7 （43.8%）. Ophthalmology examination was not performed on all the patients. Further, serum biochemical parameters were compared between AGS and 16 biliary atresia （BA） patients who were confirmed by surgery. Elevated liver enzymes were seen in all the patients. Serum total cholesterol （TC）（P=0.0007）, alanine aminotransferase （ALT）（P=0.0056）, aspartate aminotransferase （AST）（P=0.0114）, gamma-glutamyl transferase （GGT）（P=0.035） and total bile acid （TBA） levels （P=0.042） were significantly elevated in AGS patients compared to those in BA cases. However, there were no significant differences in serum total bilirubin （TB）, conjugated bilirubin （CB） and albumin （ALB） between the two groups. We identified 14 different JAG1 gene variations and 1 NOTCH2 gene mutation in 16 Chinese AGS patients. Our study suggested clinical features of AGS are highly variable and not all patients meet the classical diagnostic criteria. It was suggested that hypercholesterolaemia and significantly elevated GGT, TBA and ALT may be helpful to diagnose AGS. Genetic testing is integral in the diagnosis of AGS.

关键词： Alagille syndrome cholestasis biochemical characteristics gene infant

Ex utero intrapartum treatment for giant congenital omphalocele

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2018年第4期14卷 399-403页

作者： Xu-Yong Chen Ji-Xin Yang Hong-Yi Zhang Xiao-feng xiong Khalid Mohamoud Abdullahi Xiao-Juan Wu jie-xiong feng Department of Pediatric Surgery Tongji HospitalTongji Medical CollegeHuazhong University of Science and Technology1095 Jiefang AveWuhan 430030China

Background To determine whether ex utero intrapartum treatment (EXIT) is an appropriate approach for managing fetuses antenatally diagnosed with giant congenital omphaloceles. Methods We retrospectively reviewed patients with omphaloceles who underwent either an EXIT procedure or a traditional repair surgery. Basic and clinical parameters including gender, gestational age, birth weight, maternal blood loss, operative times and operative complications were analyzed. During the 6–12-month follow-ups, postoperative complications including bowel obstruction, abdominal infections, postoperative abdominal distension were monitored, and survival rate was analyzed. Results A total of seven patients underwent the EXIT procedure and 11 patients underwent the traditional postnatal surgery. We found no differences in maternal age, gestational age at diagnosis, gestational age at delivery and birth weight between the two groups. In the EXIT group, the average operation time for mother was 68.3 ± 17.5 minutes and the average maternal blood loss was 233.0 ± 57.7 mL. The operation time in the EXIT group (22.0 ± 4.5 minutes) was shorter than that in the traditional group (35 ± 8.7 minutes), but the length of hospital stay in the EXIT group (20.5 ± 3.1 days) was longer than that in the traditional group (15.7 ± 2.5 days,P < 0.05). During the follow-up, one patient in the EXIT group had an intestinal obstruction, one developed abdominal compartment syndrome and one died in the traditional group. Conclusions In our experience, EXIT is a safe and effective procedure for the treatment of giant congenital omphaloceles. However, more experience is needed before this procedure can be widely recommended.

关键词： Congenital omphalocele Ex utero intrapartum treatment Fetus antenatally diagnosed Safe and effective procedure

Construct hepatic analog by cell-matrix controlled assembly technology

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Chinese Science Bulletin 2006年第15期51卷 1830-1835页

作者： LIU Haixia YAN Yongnian WANG Xiaohong CHENG jie LIN feng xiong Zhuo Wu Rendong Institute of Life Science & Medicine/Center of Organism Manufacturing Department of Mechanical Engineering Tsinghua University Beijing 100084 China

A mixture of hepatic cells and chito- san/gelatin solution was deposited to construct a hepatic analog by way of layer-by-layer deposition technique using a home-made devise. The size and cell concentration of the analogs can be controlled freely. Approximately 90% of the hepatic cells re- mained viable under 0.2 Mpa extrusion pressure. Cultured in vitro 8 weeks before animal test, hepatic cells in structure maintained their phenotype and kept proliferating, and albumin and other secretion of the cells increased. Cords and hepaton-like structures were observed after culture for 20 d. These results indicate that hepatic cells could be assembled di- rectly into a 3D viable structure and expanded to form a hepatic organoid. This accomplishment is consid- ered to be an interesting means for the fabrication of liver replacements.

关键词：肝细胞动物模型壳聚糖凝胶肝类似物

Comparison of different noninvasive diagnostic methods for biliary atresia: a meta-analysis

在线全文

学校读者我要写书评

暂无评论

世界儿科杂志:英文版 2016年第1期12卷 35-43页

作者： Jin-Peng He Yun Hao Xiao-Lin Wang Xiao-Jin Yang Jing-Fan Shao jie-xiong feng Department of Pediatric Surgery Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanChina

Background:This study was undertaken to retrospectively analyze the accuracy of different methods in differentiating biliary atresia from neonatal ***:A search was made in MEDLINE,and the Web of Science for relevant original articles published in English;methodological quality of the included studies was also *** reviewers extracted data *** were pooled,summary receiver operating characteristics curve and diagnostic odds ratio(DOR)with corresponding confi dence intervals were ***:For diagnosis of biliary atresia,ultrasonography(US),hepatic scintigraphy(HBS),and magnetic resonance cholangiography(MRCP)had a pooled sensitivity of 74.9%(range:70.4%-79.1%),93.4%(range:90.3%-95.7%)and 89.7%(range:84.8%-93.4%),a specificity of 93.4%(range:91.4%-95.1%),69.2%(range:65.1%-73.1%)and 64.7%(range:58.0%-71.0%),a positive likelihood ratio of 12.16(range:6.41-23.08),3.01(range:2.15-4.20)and 3.10(range:1.59-6.06),a negative likelihood ratio of 0.23(range:0.13-0.38),0.13(range:0.06-0.25)and 0.16(range:0.06-0.44),DOR of 72.56(range:27.34-192.58),29.88(range:12.82-69.64)and 32.48(range:8.22-128.29),with an area under the curve of 0.96,0.91,and 0.92,and Q value of 0.90,0.85,and 0.85,***:US,HBS and MRCP can be very useful for the diagnostic work-up of neonatal *** improve the sensitivity and specificity,several additional measures can be used.

关键词： biliary atresia diagnostic methods meta-analysis noninvasive

Novel Insights into the Pathogenesis of I-lirschsprung＇s-associated Enterocolitis

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Chinese Medical Journal 2016年第12期129卷 1491-1497页

作者： Chun-Lei Jiao Xu-Yong Chen jie-xiong feng Department of Pediatric Surgery Tongji Hospital Huazhong University of Science and Technology Wuhan Hubei 430000 China

Objective： To systematically summary the updated results about the pathogenesis of Hirschsprung＇s-associated enterocolitis （HAEC）. Besides, we discussed the research key and direction based on these results. Data Sources： Our data cited in this review were obtained mainly from PubMed from 1975 to 2015, with keywords ＂Hirschsprung enterocolitis＂, ＂Hirschsprung＇s enterocolitis＂, ＂Hirschsprung＇s-associated enterocolitis＂, ＂Hirschsprung-associated enterocolitis＂, ＂HAEC＂, and ＂EC＂. Study Selection： Articles regarding the pathogenesis of HAEC were selected, and the articles mainly regarding the diagnosis, surgical approach, treatment, and follow-up were excluded. Results： Several factors, mainly including mucus barrier, intestinal microbiota, and immune function, as well as some other factors such as genetic variations and surgical reasons, have been found to be related to the pathogenesis of HAEC. Changed quantity and barrier property of mucus, different composition of microbiota, and an abnormal immune state work together or separately trigger HAEC. Conclusions： The maintenance of intestinal homeostasis is due to a well cooperation of microbiota, mucus barrier, and immune system. If any part presents abnormal, intestinal homeostasis will be broken. Meanwhile, for patients with Hirschsprung＇s disease or HAEC, dysfunction of these parts has been found. Thus, the happening of HAEC may be mainly attributed to the disorders of intestinal microbiota, mucus barrier, and immune system.

关键词： Enterocolitis Hirschsprung's-associated Enterocolitis Hirschsprung's Disease Pathogenesis

Comprehensive characterization of the genetic landscape of familial Hirschsprung's disease

在线全文

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2023年第7期19卷 644-651页

作者： Jun Xiao Lu-Wen Hao Jing Wang Xiao-Si Yu Jing-Yi You Ze-Jian Li Han-Dan Mao Xin-Yao Meng jie-xiong feng Department of Pediatric Surgery Tongji HospitalTongji Medical CollegeHuazhong University of Science and Technology1095 Jiefang AvenueWuhan 430030China Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders Wuhan 430030China Department of Radiology Tongji HospitalTongji Medical CollegeHuazhong University of Science and Technology1095 Jiefang AvenueWuhan 430030China

Background Hirschsprung's disease(HSCR)is one of the most common congenital digestive tract malformations and can cause stubborn constipation or gastrointestinal obstruction after birth,causing great physical and mental pain to patients and their *** have shown that more than 20 genes are involved in HSCR,and most cases of HSCR are ***,the overall rate of familial recurrence in 4331 cases of HSCR is about 7.6%.Furthermore,familial HSCR patients show incomplete *** still do not know the penetrance and genetic characteristics of these known risk genes due to the rarity of HSCR *** To find published references,we used the title/abstract terms"Hirschsprung"and"familial"in the PubMed data-base and the MeSH terms"Hirschsprung"and"familial"in Web of ***,we summarized 129 HSCR families over the last 40 *** The male-to-female ratio and the percentage of short segment-HSCR in familial HSCR are much lower than in sporadic *** primary gene factors in the syndromic families are ret proto-oncogene(RET)and endothelin B receptor gene(EDNRB).Most families show incomplete dominance and are relevant to RET,and the RET mutation has 56%pen-etrance in familial *** one of the parents is a RET mutation carrier in an HSCR family,the offspring's recurrence risk is 28%,and the incidence of the offspring does not depend on whether the parent suffers from *** Our findings will help HSCR patients obtain better genetic counseling,calculate the risk of recurrence,and provide new insights for future pedigree studies.

关键词： Genetic characteristics Hirschsprung's disease Penetrance Recurrence risk Ret proto-oncogene(RET)

Early complications after excision with hepaticoenterostomy for infants and children with choledochal cysts

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Hepatobiliary & Pancreatic Diseases International 2002年第2期1卷 281-284页

作者： Min-Ju Li jie-xiong feng Qi-Fei Jin From the Department of Pediatric Surgery, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China Department of Pediatric Surgery Children's Hospital Zhejiang University School of Medicine Hangzhou 310003 China. minjuli@***

Objective: To retrospectively study the early compli- cations of excision with hepaticoenterostomy for in- fants and children with choledochal cysts. Methods: We analyzed 16 patients with early posto- perative complications out of 173 patients with con- genital choledochal cysts aged 27 days to 14 years (mean 2.4 years) who had undergone excisional pro- cedures and biliary tract reconstruction. Results: The early complications included bile leak- age (10 patients), abdominal wall dehiscence (3), and hepatic failure, pancreatic juice leakage and postoperative intussusception (each in 1) respective- ly. Three patients died from bile leakage and 1 from postoperative hepatic failure. No statistical differen- ces were observed between the procedures of biliary tract reconstruction with jejunal segment interposi- tion hepaticoduodenostomy and Roux-en-Y hepatico- jejunostomy (P>0.75). The morbidity was signifi- cantly higher in infants below 1 year than in children (P<0.005). Prevention and treatment of the com- plications were discussed. Conclusion: Bile leakage and abdominal wall dehis- cence are major early postoperative complications. The morbidity of cholechal cysts is higher in infants than in children. Exploratory laparotomy should not be delayed when biliary leakage with diffuse peritoni- tis appears. The 'tension suture in the fascial space of the abdominal wall' is useful to prevent and treat wound dehiscence.

关键词： choledochal cyst postoperative complication infants children

基于加窗插值和Prony的电力系统间谐波算法

在线全文

学校读者我要写书评

暂无评论

电力系统保护与控制 2011年第7期39卷 8-13,19页

作者：熊杰锋南京信息工程大学电子信息工程学院江苏南京210044 河海大学电气工程学院江苏南京210098

邻近谐波或基波的间谐波,是导致电压闪变的直接原因,准确检出该成分,对于改善电能质量具有重要意义。考虑真实电网宽带多频的信号特性,分析了加窗插值算法和谱估计法局限性,提出基于加窗插值和Prony的间谐波检测法。先通过加窗插值求解... 详细信息

邻近谐波或基波的间谐波,是导致电压闪变的直接原因,准确检出该成分,对于改善电能质量具有重要意义。考虑真实电网宽带多频的信号特性,分析了加窗插值算法和谱估计法局限性,提出基于加窗插值和Prony的间谐波检测法。先通过加窗插值求解各个信号参数,进而采用频率分析定位临近谐波和间谐波所在频率区间,最后对时域滤波后的残差分量用Prony算法计算。Matlab仿真表明该方法频率分辨率和精度优于加窗插值,相对传统Prony法不需要估算信号个数,能有效消除伪谱的影响。

关键词：间谐波频率分辨率加窗插值 Prony算法