目的探讨深化术前营养预康复在小儿先天性巨结肠中的应用效果。方法回顾性分析2015—2020年右江民族医学院附属医院收治的先天性巨结肠患儿82例,男65例,女17例;年龄3~58个月,平均(5.2±1.8)个月;根据处理方法的不同分为对照组(36例,...目的探讨深化术前营养预康复在小儿先天性巨结肠中的应用效果。方法回顾性分析2015—2020年右江民族医学院附属医院收治的先天性巨结肠患儿82例,男65例,女17例;年龄3~58个月,平均(5.2±1.8)个月;根据处理方法的不同分为对照组(36例,2015—2017年)、预康复组(46例,2018—2020年)。对照组采用传统处理模式,预康复组术前1~2个月施行预康复,进行营养评估及营养干预,围手术期采用加速康复外科模式,比较预康复组预康复前后营养不良的发生率以及两组的各项围手术期指标、术后随访观察指标。结果预康复组中,预康复后患儿体重指数(BMI)和前白蛋白(PA)优于预康复前(P<0.05);预康复组在术后留置胃管时间、肠功能恢复时间、术后住院时间、小肠结肠炎的发生率方面少于对照组(P<0.05),对照组术后24 h C反应蛋白(CRP)高于预康复组(P<0.05)。结论深化术前营养预康复的加速康复外科流程,能够矫正部分患儿的营养不良,使患儿更安全地耐受手术,降低术后炎症水平,并能够减少术后小肠结肠炎的发生率。展开更多
Hirschsprung disease(HSCR)is a congenital intestinal motility disorder characterized by absent enteric ganglia in the distal intestine.1 While HSCR is primarily a genetic disorder,its inheritance pattern is complex an...Hirschsprung disease(HSCR)is a congenital intestinal motility disorder characterized by absent enteric ganglia in the distal intestine.1 While HSCR is primarily a genetic disorder,its inheritance pattern is complex and remains largely unexplored.2 Current genetic screening focuses on rare variants of several essential genes(e.g.,RET,EDNRB,GDNF,SOX10),which unfortunately could explain only a limited portion of genetic heritability.3 The interplay between common and rare variants may significantly impact disease risk.展开更多
Hirschsprung’s disease(HSCR)results from neural crest cell migration and differentiation issues.1 Previously,analysis was limited to bulk tissue samples,but single-cell RNA sequencing now offers microscopic insights....Hirschsprung’s disease(HSCR)results from neural crest cell migration and differentiation issues.1 Previously,analysis was limited to bulk tissue samples,but single-cell RNA sequencing now offers microscopic insights.Our pioneering single-cell RNA sequencing analysis of colon tissue is helping unravel HSCR’s origins and development.展开更多
Hirschsprung's disease(HSCR)is widely recognized in pediatric surgery.This condition has been elucidated,and therapeutic approaches have been developed.However,even when ganglion cells are present in the rectum,so...Hirschsprung's disease(HSCR)is widely recognized in pediatric surgery.This condition has been elucidated,and therapeutic approaches have been developed.However,even when ganglion cells are present in the rectum,some patients still experience symptoms such as bowel obstruction,intestinal dilatation,and chronic constipation,which are similar to those observed in HSCR.A consensus regarding the terminology for these diseases is yet to be established.This group of diseases was defined as‘allied disorders of Hirschsprung's disease'(ADHD).They are classified into two categories based on pathology:(1)Abnormal ganglia,including immaturity of ganglia,hypoganglionosis,and intestinal neuronal dysplasia;and(2)Normal ganglia,including megacystis microcolon intestinal hypoperistalsis syndrome(MMIHS),segmental dilatation,internal anal sphincter achalasia,and chronic idiopathic intestinal pseudo-obstruction(CIIP).Hypoganglionosis,MMIHS,and CIIP are particularly severe and not curable by surgery.Guidelines were compiled to facilitate an accurate clinical diagnosis and provide appropriate treatment strategies for each disease.A fullthickness rectal biopsy with H&E and acetylcholinesterase staining is often required for a differential diagnosis.Patients are now able to survive longer with enteral nutrition combined with long-term intravenous nutrition and decompression of the gastrointestinal tract.However,all treatment strategies are symptomatic.It is necessary to improve the results of small intestine transplantation and to develop new therapies using regenerative medicine.展开更多
Background This study aimed to investigate if mashed ripe bananas(bananagram)could be used to assess the integrity of the gut before transverse colostomy closure in patients with Hirschsprung disease(HSCR)in a low-and...Background This study aimed to investigate if mashed ripe bananas(bananagram)could be used to assess the integrity of the gut before transverse colostomy closure in patients with Hirschsprung disease(HSCR)in a low-and middle-income country tertiary-level hospital.Methods This is a prospective,single-center,hospitalbased cohort study.A feeding tube tip was inserted about 5–8 cm through the distal loop,and 15 mL of mashed banana was introduced.The amount introduced and the spontaneous expulsion of mashed banana were measured.We also measured colonic transit time(CTT),input and expulsion ratio,color of the expulsed banana stream,size of the banana stream passed through the anus and clinical signs of bowel obstruction or perforation after the procedure.Results From January 2018 to June 2023,266 HSCR patients with a transverse colostomy were included.The mean±standard deviation(SD)age was 26.45±6.41 months.The median CTT of mashed banana was 8 min(interquartile range(IQR):4–13 min).The mean amount of mashed banana spontaneously expelled through the anus was 13.25±0.95 mL.More than 90%of patients expelled unchanged mashed bananas,and 9.4%expelled stoolbanana mixtures.Banana stream was 0.3–1.5 cm in 257 patients and<0.3 cm in nine patients.These nine patients needed revision pull-through.Conclusion Bananagram can be performed at the bedside.It is easy to perform,cost-effective,available throughout the year and does not pose radiation hazards.展开更多
文摘目的探讨深化术前营养预康复在小儿先天性巨结肠中的应用效果。方法回顾性分析2015—2020年右江民族医学院附属医院收治的先天性巨结肠患儿82例,男65例,女17例;年龄3~58个月,平均(5.2±1.8)个月;根据处理方法的不同分为对照组(36例,2015—2017年)、预康复组(46例,2018—2020年)。对照组采用传统处理模式,预康复组术前1~2个月施行预康复,进行营养评估及营养干预,围手术期采用加速康复外科模式,比较预康复组预康复前后营养不良的发生率以及两组的各项围手术期指标、术后随访观察指标。结果预康复组中,预康复后患儿体重指数(BMI)和前白蛋白(PA)优于预康复前(P<0.05);预康复组在术后留置胃管时间、肠功能恢复时间、术后住院时间、小肠结肠炎的发生率方面少于对照组(P<0.05),对照组术后24 h C反应蛋白(CRP)高于预康复组(P<0.05)。结论深化术前营养预康复的加速康复外科流程,能够矫正部分患儿的营养不良,使患儿更安全地耐受手术,降低术后炎症水平,并能够减少术后小肠结肠炎的发生率。
基金founded by the National Natural Science Foundation of China(No.81970450 to Z.Y.,82201893 to Z.J.L.,82070528 to H.L.H.,82170528 to Z.J.X.)the Natural Science Foundation of Guangdong Province,China(No.2022A1515012254,2018A030313570 to Z.J.X.)+3 种基金the Guangdong Basic and Applied Basic Research Foundation(No.2021A1515220146 to Z.Y.)the Guangzhou Basic Research Plan City School(Institute)Enterprise Joint Funding Project(China)(No.SL2024A03J01 to Z.Y.)the Science and Technology Planning Project of Guangdong Province,China(No.2019B020227001 to X.H.M.)the Science and Technology Project of Guangzhou,China(No.202206080002 to X.H.M.).
文摘Hirschsprung disease(HSCR)is a congenital intestinal motility disorder characterized by absent enteric ganglia in the distal intestine.1 While HSCR is primarily a genetic disorder,its inheritance pattern is complex and remains largely unexplored.2 Current genetic screening focuses on rare variants of several essential genes(e.g.,RET,EDNRB,GDNF,SOX10),which unfortunately could explain only a limited portion of genetic heritability.3 The interplay between common and rare variants may significantly impact disease risk.
基金supported by the National Natural Science Foundation of China(No.81770514,81270439).
文摘Hirschsprung’s disease(HSCR)results from neural crest cell migration and differentiation issues.1 Previously,analysis was limited to bulk tissue samples,but single-cell RNA sequencing now offers microscopic insights.Our pioneering single-cell RNA sequencing analysis of colon tissue is helping unravel HSCR’s origins and development.
文摘Hirschsprung's disease(HSCR)is widely recognized in pediatric surgery.This condition has been elucidated,and therapeutic approaches have been developed.However,even when ganglion cells are present in the rectum,some patients still experience symptoms such as bowel obstruction,intestinal dilatation,and chronic constipation,which are similar to those observed in HSCR.A consensus regarding the terminology for these diseases is yet to be established.This group of diseases was defined as‘allied disorders of Hirschsprung's disease'(ADHD).They are classified into two categories based on pathology:(1)Abnormal ganglia,including immaturity of ganglia,hypoganglionosis,and intestinal neuronal dysplasia;and(2)Normal ganglia,including megacystis microcolon intestinal hypoperistalsis syndrome(MMIHS),segmental dilatation,internal anal sphincter achalasia,and chronic idiopathic intestinal pseudo-obstruction(CIIP).Hypoganglionosis,MMIHS,and CIIP are particularly severe and not curable by surgery.Guidelines were compiled to facilitate an accurate clinical diagnosis and provide appropriate treatment strategies for each disease.A fullthickness rectal biopsy with H&E and acetylcholinesterase staining is often required for a differential diagnosis.Patients are now able to survive longer with enteral nutrition combined with long-term intravenous nutrition and decompression of the gastrointestinal tract.However,all treatment strategies are symptomatic.It is necessary to improve the results of small intestine transplantation and to develop new therapies using regenerative medicine.
文摘Background This study aimed to investigate if mashed ripe bananas(bananagram)could be used to assess the integrity of the gut before transverse colostomy closure in patients with Hirschsprung disease(HSCR)in a low-and middle-income country tertiary-level hospital.Methods This is a prospective,single-center,hospitalbased cohort study.A feeding tube tip was inserted about 5–8 cm through the distal loop,and 15 mL of mashed banana was introduced.The amount introduced and the spontaneous expulsion of mashed banana were measured.We also measured colonic transit time(CTT),input and expulsion ratio,color of the expulsed banana stream,size of the banana stream passed through the anus and clinical signs of bowel obstruction or perforation after the procedure.Results From January 2018 to June 2023,266 HSCR patients with a transverse colostomy were included.The mean±standard deviation(SD)age was 26.45±6.41 months.The median CTT of mashed banana was 8 min(interquartile range(IQR):4–13 min).The mean amount of mashed banana spontaneously expelled through the anus was 13.25±0.95 mL.More than 90%of patients expelled unchanged mashed bananas,and 9.4%expelled stoolbanana mixtures.Banana stream was 0.3–1.5 cm in 257 patients and<0.3 cm in nine patients.These nine patients needed revision pull-through.Conclusion Bananagram can be performed at the bedside.It is easy to perform,cost-effective,available throughout the year and does not pose radiation hazards.
