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Robotic-assisted proctosigmoidectomy for hirschsprung’s disease:A multicenter prospective study

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World Journal of Gastroenterology 2023年第23期29卷 3715-3732页

作者： Meng-Xin Zhang Xi Zhang Xiao-Pan Chang Ji-Xiao Zeng Hong-Qiang Bian Guo-Qing Cao shuai Li shui-Qing Chi Ying Zhou Li-Ying Rong Li Wan shao-Tao Tang Department of Pediatric surgery Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430022Hubei ProvinceChina Department of Pediatric surgery Guangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangzhou 510623Guangdong ProvinceChina Department of General surgery Wuhan Children’s HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430019Hubei ProvinceChina

BACKGROUND Robotic surgery is a cutting-edge minimally invasive technique that overcomes many shortcomings of laparoscopic techniques,yet few studies have evaluated the use of robotic surgery to treat hirschsprung’s disease(HsCR).AIM To analyze the feasibility and medium-term outcomes of robotic-assisted proctosigmoidectomy(RAPs)with sphincter-and nerve-sparing surgery in HsCR *** From July 2015 to January 2022,156 rectosigmoid HsCR patients were enrolled in this multicenter prospective *** sphincters and nerves were spared by dissecting the rectum completely from the pelvic cavity outside the longitudinal muscle of the rectum and then performing transanal soave pull-through *** outcomes and continence function were *** No conversions or intraoperative complications *** median age at surgery was 9.50 months,and the length of the removed bowel was 15.50±5.23 *** total operation time,console time,and anal traction time were 155.22±16.77,58.01±7.71,and 45.28±8.15 *** were 25 complications within 30 d and 48 post-30-d *** children aged≥4 years,the bowel function score(BFs)was 17.32±2.63,and 90.91%of patients showed moderate-to-good bowel *** postoperative fecal continence(POFC)score was 10.95±1.04 at 4 years of age,11.48±0.72 at 5 years of age,and 11.94±0.81 at 6 years of age,showing a promising annual *** were no significant differences in postoperative complications,BFs,and POFC scores related to age at surgery being≤3 mo or>3 *** RAPs is a safe and effective alternative for treating HsCR in children of all ages;it offers the advantage of further minimizing damage to sphincters and perirectal nerves and thus providing better continence function.

关键词： Robotic-assisted hirschsprung’s disease Continence function sphincter Nerve

Genetic interactions and modifi er genes in hirschsprung's disease

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World Journal of Gastroenterology 2011年第45期17卷 4937-4944页

作者： Adam s Wallace Richard B Anderson Department of Anatomy and Cell Biology University of Melbourne

hirschsprung's disease is a congenital disorder that occurs in 1:5000 live births. It is characterised by an absence of enteric neurons along a variable region of the gastrointestinal tract. hirschsprung's disease is classified as a multigenic disorder, because the same phenotype is associated with mutations in multiple distinct genes. Furthermore, the genetics of hirschsprung's disease are highly complex and not strictly Mendelian. The phenotypic variability and incomplete penetrance observed in hirschsprung' s disease also suggests the involvement of modifier genes. Here, we summarise the current knowledge of the genetics underlying hirschsprung's disease based on human and animal studies, focusing on the principal causative genes, their interactions, and the role of modif ier genes.

关键词： Neural crest Enteric nervous system hirschsprung’s disease Aganglionosis Modif ier genes

Very early onset perinatal constipation:Can it be cow’s milk protein allergy?

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World Journal of Gastroenterology 2023年第33期29卷 4920-4926页

作者： Rajalakshmy Arakoni Hebat Kamal sam Xianjun Cheng Department of Pediatrics University of Florida Shands Children's HospitalGainesvilleFL 32608United States Department of Pediatric Gastroenterology Hepatologyand NutritionUniversity of Florida Shands Children's HospitalGainesvilleFL 32608United States

Delayed passage of meconium or constipation during the perinatal period is traditionally regarded as a signal to initiate further work up to evaluate for serious diagnoses such as hirschsprung’s disease(HD),meconium ileus due to Cystic Fibrosis,*** diagnosis of HD particularly warrants invasive testing to confirm the diagnosis,such as anorectal manometry or rectal suction *** if there was another etiology of perinatal constipation,that is far lesser known?Cow’s milk protein allergy(CMPA)is often diagnosed in infants within the first few weeks of life,however,there are studies that show that the CMPA allergen can be passed from mother to an infant in-utero,therefore allowing symptoms to show as early as day one of *** presentation is more atypical,with perinatal constipation rather than with bloody stools,diarrhea,and *** diagnosis and management would be avoidance of cow's milk protein within the diet,with results and symptom improvement in patients ***,we discuss whether an alternative pathway to address perinatal constipation should be further discussed and implemented to potentially avoid invasive techniques in *** entails first ruling out CMPA with safe,noninvasive techniques with diet modification,and if unsuccessful,then moving forward with further diagnostic modalities.

关键词： Delayed passage of meconium Perinatal constipation Cow's milk protein allergy In-utero Alternate pathway hirschsprung’s disease

Phenotypic and functional comparison of rat enteric neural crest-derived cells during fetal and earlypostnatal stages

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Neural Regeneration Research 2021年第11期16卷 2310-2315页

作者： Dong-Hao Tian Chuan-Hui Qin Wen-Yao Xu Wei-Kang Pan Yu-Ying Zhao Bai-Jun Zheng Xin-Lin Chen Yong Liu Ya Gao Hui Yu Department of Pediatric surgery the Second Affiliated HospitalXi’an Jiaotong UniversityXi’anShaanxi ProvinceChina Department of Anorectal Suizhou Central HospitalHubei University of MedicineSuizhouHubei ProvinceChina Institute of Neurobiology Environment and Genes Related to Diseases Key Laboratory of Chinese Ministry of EducationXi’an Jiaotong UniversityXi’anShaanxi ProvinceChina

In our previous study,we showed that with increasing time in culture,the growth characteristics of enteric neural crest-derived cells(ENCCs)change,and that the proliferation,migration and neural differentiation potential of these cells in vitro notably ***,there are no studies on the developmental differences in these characteristics between fetal and early-postnatal stages in vitro or in *** this study,we isolated fetal(embryonic day 14.5)and postnatal(postnatal day 2)ENCCs from the intestines of *** ENCCs had greater maximum cross-sectional area of the neurospheres,stronger migration ability,and reduced apoptosis,compared with postnatal ***,fetal and postnatal ENCCs had a similar differentiation *** and postnatal ENCCs both survived after transplant into a rat model of hirschsprung’s *** these rats with hirschsprung’s disease,the number of ganglionic cells in the myenteric plexus was higher and the distal intestinal pressure change was greater in animals treated with fetal ENCCs compared with those treated with postnatal *** findings suggest that,compared with postnatal ENCCs,fetal ENCCs exhibit higher survival and proliferation and migration abilities,and are therefore a more appropriate seed cell for the treatment of hirschsprung’s *** study was approved by the Animal Ethics Committee of the second Affiliated Hospital of Xi’an Jiaotong University(approval No.2016086)on March 3,2016.

关键词： cell transplantation enteric nervous system enteric neural crest-derived cells hirschsprung’s disease stem cells

Diagnostic Input of specialized Radiographic Examinations in Pediatric setting in Ouagadougou (Burkina Faso)

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Open Journal of Radiology 2022年第2期12卷 49-57页

作者： Nina-Astrid Ouedraogo Moussa Zanga Bénilde Marie Ange Tiemtoré Kambou Ky Davy sissiman Madina Aischa Napon Boubacar Ouattara Ousséini Diallo Léonie Claudine Lougue Radiology Department of Bogodogo Teaching Hospital Ouagadougou Burkina Faso Training and Research Unit in Health sciences University Joseph Ki-Zerbo Ouagadougou Burkina Faso Radiology Department of Charles de Gaulle Pediatric Teaching Hospital Ouagadougou Burkina Faso Radiology Department of Yalgado Ouédraogo Teaching Hospital Ouagadougou Burkina Faso

Context: In spite of the development of cross-sectional medical imaging and endoscopy means, the prescription of specialized radiographic examinations is still relevant in the pediatric environment in our country. Objective: show the contribution of specialized radiographic examinations performed in pediatric settings. Methodology: This was a retrospective descriptive study implemented over a five year-period, from June 10sup>thsup>, 2014 to June 10sup>thsup>, 2019 within the radiology department of Charles De Gaulle Pediatric Teaching Hospital in Ouagadougou. This study included the results of 192 specialized radiographic examinations collected within the concerned department. Results: The average age of the patients was 4.21 years with extremes of 8 days and 15 years. Infants (29 days to 24 months) were the predominant age group with a rate of 53.13% (n = 102 patients). Boys represented 76.51% of the sample with a sex ratio estimated at 3.25. specialized radiographic examinations represented 0.46% of the overall imaging examinations and 0.72% of radiographic examinations performed during the study period. 67.19% of pathological examinations were reported. Colonic enema and retrograde urethrocystography (RUC) were the most performed examinations respectively accounting for 33.34% and 27.6%. The radiographic findings were consistent with the diagnostic hypotheses in 41.6% of cases. Conclusion: specialized radiographic examinations keep their place in the exploration of several pathologies in pediatric settings.

关键词： Esophageal Transit Retrograde Urethrocystography hirschsprung’s disease