BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disorder characterized by fever,arthritis,skin rash,and systemic symptoms.The etiology of AOSD is unknown;however,it is thought to be relate...BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disorder characterized by fever,arthritis,skin rash,and systemic symptoms.The etiology of AOSD is unknown;however,it is thought to be related to immune dysregulation.Although a rare disease,AOSD can significantly impact reproductive health,particularly during pregnancy.This case study assesses the implications of pregnancy in a patient with AOSD,as well as the potential for heredity of the disease.Neonatal hemophagocytic lympho-histiocytosis(HLH)is a rare and lifethreatening disorder characterized by hyperinflammation and uncontrolled activation of immune cells,leading to multiple organ dysfunction.This case report aimed to introduce neonatal HLH from a mother with AOSD.CASE SUMMARY This case study presents a 29-year-old female with AOSD who became pregnant and gave birth to a premature infant who was diagnosed with neonatal HLH.AOSD can significantly impact pregnancy and childbirth,as it may become more severe during pregnancy,with an increased risk of fetal loss and preterm birth.The management of AOSD during pregnancy involves the use of nonsteroidal anti-inflammatory drugs and glucocorticoids,as well as immunosuppressive agents in severe cases.However,the use of immunosuppressive agents during pregnancy may be associated with potential risks to the fetus.The hereditary implications of AOSD are unclear;however,available evidence suggests that genetic factors may play a role in the disease development.CONCLUSION AOSD can have significant implications for pregnancy and childbirth,including an increased risk of fetal loss and preterm birth.Neonatal HLH,a complication of AOSD in pregnancy,requires prompt diagnosis and management.Women with AOSD who are considering pregnancy should discuss their options with their healthcare provider and develop a management plan that addresses the potential risks to both mother and fetus.展开更多
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 ...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 patients.METHODS From July 2015 to January 2022,156 rectosigmoid HSCR patients were enrolled in this multicenter prospective study.Their 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 procedures.Surgical outcomes and continence function were analyzed.RESULTS No conversions or intraoperative complications occurred.The median age at surgery was 9.50 months,and the length of the removed bowel was 15.50±5.23 cm.The total operation time,console time,and anal traction time were 155.22±16.77,58.01±7.71,and 45.28±8.15 min.There were 25 complications within 30 d and 48 post-30-d complications.For children aged≥4 years,the bowel function score(BFS)was 17.32±2.63,and 90.91%of patients showed moderate-to-good bowel function.The 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 trend.There were no significant differences in postoperative complications,BFS,and POFC scores related to age at surgery being≤3 mo or>3 mo.CONCLUSION 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.展开更多
AIM: To investigate whether the expression of plateletderived growth factor receptor-α-positive(PDGFRα^+)-cells is altered in Hirschsprung's disease(HD).METHODS: HD tissue specimens(n = 10) were collected at the...AIM: To investigate whether the expression of plateletderived growth factor receptor-α-positive(PDGFRα^+)-cells is altered in Hirschsprung's disease(HD).METHODS: HD tissue specimens(n = 10) were collected at the time of pull-through surgery, while colonic control samples were obtained at the time of colostomy closure in patients with imperforate anus(n = 10). Immunolabelling of PDGFRα^+-cells was visualized using confocal microscopy to assess the distribution of these cells, while Western blot analysis was undertaken to quantify PDGFRα protein expression.RESULTS: Confocal microscopy revealed PDGFRα+-cells within the mucosa, myenteric plexus and smooth muscle in normal controls, with a marked reduction in PDGFRα^+-cells in the HD specimens. Western blotting revealed high levels of PDGFRα protein expression in normal controls, while there was a striking decrease in PDGFRα protein expression in the HD colon.CONCLUSION: These findings suggest that the altered distribution of PDGFRα^+-cells in both the aganglionic and ganglionic HD bowel may contribute to the motility dysfunction in HD.展开更多
AIM:To define the topography of mast cells and their numbers in cases of Hirschsprung's disease(HD)and non-HD,assess neural hypertrophy using imaging software and to study the relationship between mast cells and n...AIM:To define the topography of mast cells and their numbers in cases of Hirschsprung's disease(HD)and non-HD,assess neural hypertrophy using imaging software and to study the relationship between mast cells and nerve fibers.METHODS:HE stained sections of 32 cases of chronic constipation in the age group of 0-14 years were reviewed for ganglion cells.AChE staining was performed on frozen sections of colonic and rectal biopsies.Based on their findings cases were divided into HD and non-HD and mast cells stained by toluidine blue were evaluated.Image analysis by computerized software was applied to S-100 stained sections for assessment of neural hypertrophy.RESULTS:Difference between number of mast cells in HD group(mean=36.44)and in non-HD group(mean =14.79)was statistically significant.Image analysis morphometry on S-100 stained sections served as a useful adjunct.The difference between number,size,and perimeter of the nerve fibers between HD and non-HD group was statistically significant.CONCLUSION:Mast cells are significantly increased in HD and their base line values are much higher in Indian children than that reported in Western literature.Their role in HD needs further research.Morphometry of S-100 stained nerve fibers is a useful adjunct to conventional methods for diagnosis of HD.展开更多
Hirschsprung’s disease(HD)is a congenital disorder,characterized by aganglionosis in the distal part of the gastrointestinal tract.Despite complete surgical resection of the aganglionic segment,both constipation and ...Hirschsprung’s disease(HD)is a congenital disorder,characterized by aganglionosis in the distal part of the gastrointestinal tract.Despite complete surgical resection of the aganglionic segment,both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options.There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD,which may play a role in persistent bowel dysfunction.These abnormalities include:(1)Histopathological abnormalities of enteric neural cells;(2)Imbalanced expression of neurotransmitters and neuroproteins;(3)Abnormal expression of enteric pacemaker cells;(4)Abnormalities of smooth muscle cells;and(5)Abnormalities within the extracellular matrix.Hence,a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction.In the long term,further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.展开更多
Background: Transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung’s disease (HD) has gained worldwide acceptance. However surgical success is often reported separately, while the...Background: Transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung’s disease (HD) has gained worldwide acceptance. However surgical success is often reported separately, while the necessity for true reoperation is difficult to establish. Aim: To evaluate the incidence of reoperations following TERPT procedure. The findings will be important in counseling and planning childcare for HD patients as well as providing a benchmark for single centers clinical results. Methods: A literature review of reported TERPT operations on children with HD between 1998 through 2011 was performed. Only planned TERPT operation reports were included. Information was collected with particular emphasis on reoperations and their reasons. Results: Out of 26 published articles 23 were included, reporting on 836 children, female/male ratio: 1/3.3, undergoing the TERPT procedure as the only operative intervention with described postoperative courses. The children comprised neonates, 3 years of age (12%). The average follow up was 18.5 (6 - 38) months. The resected bowel length mean was 20.5 cm. Forty-one reoperations were reported (4.9%), including 24 laparotomies, 8 laparoscopies, 6 colostomies and ileostomies in 3 children. Only 2 re-do TERPT were reported (0.2%). Seven patients were considered TERPT failures (0.8%) with 5 requiring diverting colostomies and additional transabdominal pull-through operations. Two myectomies were performed (0.2%). One child with aganglionosis underwent a Duhamel pull through. Two (0.2%) had serious damage to the urinary tract also one child with a vas deferens lesion was reoperated. Two bowel obstructions required adhesiolysis. Eight anastomotic dehiscences (0.9%) required surgery after reparation. One prolapse of the pulled through colon was reported. Six patients (0.7%) suffered anastomotic leaks. Anastomotic strictures rate was 2.8%, all repaired with anal dilatation. Conclusion: The review supports the low incidence of reported reoperations for the TERPT procedure.展开更多
Background: Transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung’s disease (HD) is frequently used worldwide. In order to give the families realistic expectations and to plan th...Background: Transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung’s disease (HD) is frequently used worldwide. In order to give the families realistic expectations and to plan the medical care for the years after TERPT, the long term outcome is of great importance. Aim: To collect information on the long term outcome reported after one stage TERPT procedure for HD in children 0 - 15 years. Method: A literature review on the outcome of planned TERPT from 2005 through 2012 was carried out. Information was collected on the number of daily stools a few months postoperatively, incontinence and constipation and the measures taken to deal with these. Results: The reports are few and prospective studies were missing. The results show an initial high frequency of daily stools, 12% had later abnormal stool patterns, 21% had fecal incontinence and 10% had problems with constipation. Conclusion: In order to compare the long term outcome, it would be desirable to have uniform regular reports on the daily frequency of passed stools, incontinence and constipation during the first years after TERPT. Such knowledge would be of importance for the information given to the guardians of children with HD preoperatively to TERPT and in the planning of the future care. The findings can, furthermore, provide a benchmark for the outcome from a single centre.展开更多
BACKGROUND In the past years,only a few studies with a limited number of adult patients analyzed clinical features of allied disorders of Hirschsprung’s disease(ADHD),most of which were individual case reports or lac...BACKGROUND In the past years,only a few studies with a limited number of adult patients analyzed clinical features of allied disorders of Hirschsprung’s disease(ADHD),most of which were individual case reports or lacked detailed clinical information.Although many studies have reported patients presenting to the emergency department(ED)with recurrent abdominal symptoms for a number of disorders,there are few data involving ADHD.However,owing to a lack of awareness of the disease,misdiagnoses and mistreatments are common.Severe complications such as perforation,bleeding,malabsorption,and even death in ADHD had been reported by many studies.AIM To assist ED clinicians in having a more comprehensive understanding of this disease and making an early suspected diagnosis of ADHD more effectively.METHODS We enrolled 53 patients who visited the ED and were eventually diagnosed with ADHD over the past 11 years in our hospital.Their basic information,clinical manifestations,and imaging findings were analyzed.Blood indices were compared between the ADHD and irritable bowel syndrome(IBS)groups.RESULTS Adult patients with ADHD had a mean age of 48.8±14.3 years,and 77.4%had been treated before admission.The transverse colon was the most common dilated part(73.6%),and constipation(67.9%)was the most common symptom.ADHD patients can present with uncommon symptoms and false-negative imaging findings.Logistic regression analysis indicated that body mass index(BMI)[odds ratio(OR)=0.786,P=0.013],cholinesterase(per 1000 units;OR=0.693,P=0.008),and blood chlorine(OR=0.816,P=0.022)were determined to be independent related factors between the ADHD and IBS groups.The area under the receiver operating characteristics curve of these three indices combined was 0.812(P<0.001).CONCLUSION Emergency physicians should be vigilant regarding patients with chronic constipation,abdominal pain,or abdominal distension,and consider the possibility of ADHD despite its rarity.Abdominal computed tomography examination is recommended as a useful tool in the suspected diagnosis of ADHD.BMI,cholinesterase,and blood chlorine have good discriminative abilities between ADHD and IBS.The nutritional status of adult patients with ADHD is worthy of further attention.Surgical treatment for adult patients with ADHD is important and inevitable.展开更多
Over the last two decades,advances in laparoscopicsurgery and minimally invasive techniques have transformed the operative management of neonatal colorectal surgery for conditions such as anorectal malformations(ARMs)...Over the last two decades,advances in laparoscopicsurgery and minimally invasive techniques have transformed the operative management of neonatal colorectal surgery for conditions such as anorectal malformations(ARMs) and Hirschsprung's disease.Evolution of surgical care has mainly occurred due to the use of laparoscopy,as opposed to a laparotomy,for intraabdominal procedures and the development of trans-anal techniques.This review describes these advances and outlines the main minimally invasive techniques currently used for management of ARMs and Hirschsprung's disease.There does still remain significant variation in the procedures used and this review aims to report the current literature comparing techniques with an emphasis on the short- and long-term clinical outcomes.展开更多
Some children with previously undiagnosed Hirschprung’s disease present severely ill at an advanced age with markedly dilated bowel and enterocolitis. In this manuscript, the authors propose the following treatment a...Some children with previously undiagnosed Hirschprung’s disease present severely ill at an advanced age with markedly dilated bowel and enterocolitis. In this manuscript, the authors propose the following treatment algorithm: 1) a diverting transverse loop colostomy, 2) a transanal Soave endorectal pull-through, and 3) colostomy closure. The authors report their experience with five patients.展开更多
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 ...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,etc.The diagnosis of HD particularly warrants invasive testing to confirm the diagnosis,such as anorectal manometry or rectal suction biopsy.What 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 life.The presentation is more atypical,with perinatal constipation rather than with bloody stools,diarrhea,and vomiting.The diagnosis and management would be avoidance of cow's milk protein within the diet,with results and symptom improvement in patients immediately.Therefore,we discuss whether an alternative pathway to address perinatal constipation should be further discussed and implemented to potentially avoid invasive techniques in patients.This entails first ruling out CMPA with safe,noninvasive techniques with diet modification,and if unsuccessful,then moving forward with further diagnostic modalities.展开更多
文摘BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disorder characterized by fever,arthritis,skin rash,and systemic symptoms.The etiology of AOSD is unknown;however,it is thought to be related to immune dysregulation.Although a rare disease,AOSD can significantly impact reproductive health,particularly during pregnancy.This case study assesses the implications of pregnancy in a patient with AOSD,as well as the potential for heredity of the disease.Neonatal hemophagocytic lympho-histiocytosis(HLH)is a rare and lifethreatening disorder characterized by hyperinflammation and uncontrolled activation of immune cells,leading to multiple organ dysfunction.This case report aimed to introduce neonatal HLH from a mother with AOSD.CASE SUMMARY This case study presents a 29-year-old female with AOSD who became pregnant and gave birth to a premature infant who was diagnosed with neonatal HLH.AOSD can significantly impact pregnancy and childbirth,as it may become more severe during pregnancy,with an increased risk of fetal loss and preterm birth.The management of AOSD during pregnancy involves the use of nonsteroidal anti-inflammatory drugs and glucocorticoids,as well as immunosuppressive agents in severe cases.However,the use of immunosuppressive agents during pregnancy may be associated with potential risks to the fetus.The hereditary implications of AOSD are unclear;however,available evidence suggests that genetic factors may play a role in the disease development.CONCLUSION AOSD can have significant implications for pregnancy and childbirth,including an increased risk of fetal loss and preterm birth.Neonatal HLH,a complication of AOSD in pregnancy,requires prompt diagnosis and management.Women with AOSD who are considering pregnancy should discuss their options with their healthcare provider and develop a management plan that addresses the potential risks to both mother and fetus.
基金Supported by the National Health and Family Planning of China,No.201402007the National Natural Science Foundation of China,No.81873848 and No.82170528.
文摘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 patients.METHODS From July 2015 to January 2022,156 rectosigmoid HSCR patients were enrolled in this multicenter prospective study.Their 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 procedures.Surgical outcomes and continence function were analyzed.RESULTS No conversions or intraoperative complications occurred.The median age at surgery was 9.50 months,and the length of the removed bowel was 15.50±5.23 cm.The total operation time,console time,and anal traction time were 155.22±16.77,58.01±7.71,and 45.28±8.15 min.There were 25 complications within 30 d and 48 post-30-d complications.For children aged≥4 years,the bowel function score(BFS)was 17.32±2.63,and 90.91%of patients showed moderate-to-good bowel function.The 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 trend.There were no significant differences in postoperative complications,BFS,and POFC scores related to age at surgery being≤3 mo or>3 mo.CONCLUSION 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.
基金Supported by National Children’s Research Centre/Children’s Medical Research Foundation,Ireland
文摘AIM: To investigate whether the expression of plateletderived growth factor receptor-α-positive(PDGFRα^+)-cells is altered in Hirschsprung's disease(HD).METHODS: HD tissue specimens(n = 10) were collected at the time of pull-through surgery, while colonic control samples were obtained at the time of colostomy closure in patients with imperforate anus(n = 10). Immunolabelling of PDGFRα^+-cells was visualized using confocal microscopy to assess the distribution of these cells, while Western blot analysis was undertaken to quantify PDGFRα protein expression.RESULTS: Confocal microscopy revealed PDGFRα+-cells within the mucosa, myenteric plexus and smooth muscle in normal controls, with a marked reduction in PDGFRα^+-cells in the HD specimens. Western blotting revealed high levels of PDGFRα protein expression in normal controls, while there was a striking decrease in PDGFRα protein expression in the HD colon.CONCLUSION: These findings suggest that the altered distribution of PDGFRα^+-cells in both the aganglionic and ganglionic HD bowel may contribute to the motility dysfunction in HD.
文摘AIM:To define the topography of mast cells and their numbers in cases of Hirschsprung's disease(HD)and non-HD,assess neural hypertrophy using imaging software and to study the relationship between mast cells and nerve fibers.METHODS:HE stained sections of 32 cases of chronic constipation in the age group of 0-14 years were reviewed for ganglion cells.AChE staining was performed on frozen sections of colonic and rectal biopsies.Based on their findings cases were divided into HD and non-HD and mast cells stained by toluidine blue were evaluated.Image analysis by computerized software was applied to S-100 stained sections for assessment of neural hypertrophy.RESULTS:Difference between number of mast cells in HD group(mean=36.44)and in non-HD group(mean =14.79)was statistically significant.Image analysis morphometry on S-100 stained sections served as a useful adjunct.The difference between number,size,and perimeter of the nerve fibers between HD and non-HD group was statistically significant.CONCLUSION:Mast cells are significantly increased in HD and their base line values are much higher in Indian children than that reported in Western literature.Their role in HD needs further research.Morphometry of S-100 stained nerve fibers is a useful adjunct to conventional methods for diagnosis of HD.
文摘Hirschsprung’s disease(HD)is a congenital disorder,characterized by aganglionosis in the distal part of the gastrointestinal tract.Despite complete surgical resection of the aganglionic segment,both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options.There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD,which may play a role in persistent bowel dysfunction.These abnormalities include:(1)Histopathological abnormalities of enteric neural cells;(2)Imbalanced expression of neurotransmitters and neuroproteins;(3)Abnormal expression of enteric pacemaker cells;(4)Abnormalities of smooth muscle cells;and(5)Abnormalities within the extracellular matrix.Hence,a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction.In the long term,further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.
文摘Background: Transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung’s disease (HD) has gained worldwide acceptance. However surgical success is often reported separately, while the necessity for true reoperation is difficult to establish. Aim: To evaluate the incidence of reoperations following TERPT procedure. The findings will be important in counseling and planning childcare for HD patients as well as providing a benchmark for single centers clinical results. Methods: A literature review of reported TERPT operations on children with HD between 1998 through 2011 was performed. Only planned TERPT operation reports were included. Information was collected with particular emphasis on reoperations and their reasons. Results: Out of 26 published articles 23 were included, reporting on 836 children, female/male ratio: 1/3.3, undergoing the TERPT procedure as the only operative intervention with described postoperative courses. The children comprised neonates, 3 years of age (12%). The average follow up was 18.5 (6 - 38) months. The resected bowel length mean was 20.5 cm. Forty-one reoperations were reported (4.9%), including 24 laparotomies, 8 laparoscopies, 6 colostomies and ileostomies in 3 children. Only 2 re-do TERPT were reported (0.2%). Seven patients were considered TERPT failures (0.8%) with 5 requiring diverting colostomies and additional transabdominal pull-through operations. Two myectomies were performed (0.2%). One child with aganglionosis underwent a Duhamel pull through. Two (0.2%) had serious damage to the urinary tract also one child with a vas deferens lesion was reoperated. Two bowel obstructions required adhesiolysis. Eight anastomotic dehiscences (0.9%) required surgery after reparation. One prolapse of the pulled through colon was reported. Six patients (0.7%) suffered anastomotic leaks. Anastomotic strictures rate was 2.8%, all repaired with anal dilatation. Conclusion: The review supports the low incidence of reported reoperations for the TERPT procedure.
文摘Background: Transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung’s disease (HD) is frequently used worldwide. In order to give the families realistic expectations and to plan the medical care for the years after TERPT, the long term outcome is of great importance. Aim: To collect information on the long term outcome reported after one stage TERPT procedure for HD in children 0 - 15 years. Method: A literature review on the outcome of planned TERPT from 2005 through 2012 was carried out. Information was collected on the number of daily stools a few months postoperatively, incontinence and constipation and the measures taken to deal with these. Results: The reports are few and prospective studies were missing. The results show an initial high frequency of daily stools, 12% had later abnormal stool patterns, 21% had fecal incontinence and 10% had problems with constipation. Conclusion: In order to compare the long term outcome, it would be desirable to have uniform regular reports on the daily frequency of passed stools, incontinence and constipation during the first years after TERPT. Such knowledge would be of importance for the information given to the guardians of children with HD preoperatively to TERPT and in the planning of the future care. The findings can, furthermore, provide a benchmark for the outcome from a single centre.
基金the Foundation of Key Discipline Construction of Zhejiang Province for Traditional Chinese Medicine,No. 2017-XK-A36
文摘BACKGROUND In the past years,only a few studies with a limited number of adult patients analyzed clinical features of allied disorders of Hirschsprung’s disease(ADHD),most of which were individual case reports or lacked detailed clinical information.Although many studies have reported patients presenting to the emergency department(ED)with recurrent abdominal symptoms for a number of disorders,there are few data involving ADHD.However,owing to a lack of awareness of the disease,misdiagnoses and mistreatments are common.Severe complications such as perforation,bleeding,malabsorption,and even death in ADHD had been reported by many studies.AIM To assist ED clinicians in having a more comprehensive understanding of this disease and making an early suspected diagnosis of ADHD more effectively.METHODS We enrolled 53 patients who visited the ED and were eventually diagnosed with ADHD over the past 11 years in our hospital.Their basic information,clinical manifestations,and imaging findings were analyzed.Blood indices were compared between the ADHD and irritable bowel syndrome(IBS)groups.RESULTS Adult patients with ADHD had a mean age of 48.8±14.3 years,and 77.4%had been treated before admission.The transverse colon was the most common dilated part(73.6%),and constipation(67.9%)was the most common symptom.ADHD patients can present with uncommon symptoms and false-negative imaging findings.Logistic regression analysis indicated that body mass index(BMI)[odds ratio(OR)=0.786,P=0.013],cholinesterase(per 1000 units;OR=0.693,P=0.008),and blood chlorine(OR=0.816,P=0.022)were determined to be independent related factors between the ADHD and IBS groups.The area under the receiver operating characteristics curve of these three indices combined was 0.812(P<0.001).CONCLUSION Emergency physicians should be vigilant regarding patients with chronic constipation,abdominal pain,or abdominal distension,and consider the possibility of ADHD despite its rarity.Abdominal computed tomography examination is recommended as a useful tool in the suspected diagnosis of ADHD.BMI,cholinesterase,and blood chlorine have good discriminative abilities between ADHD and IBS.The nutritional status of adult patients with ADHD is worthy of further attention.Surgical treatment for adult patients with ADHD is important and inevitable.
文摘Over the last two decades,advances in laparoscopicsurgery and minimally invasive techniques have transformed the operative management of neonatal colorectal surgery for conditions such as anorectal malformations(ARMs) and Hirschsprung's disease.Evolution of surgical care has mainly occurred due to the use of laparoscopy,as opposed to a laparotomy,for intraabdominal procedures and the development of trans-anal techniques.This review describes these advances and outlines the main minimally invasive techniques currently used for management of ARMs and Hirschsprung's disease.There does still remain significant variation in the procedures used and this review aims to report the current literature comparing techniques with an emphasis on the short- and long-term clinical outcomes.
文摘Some children with previously undiagnosed Hirschprung’s disease present severely ill at an advanced age with markedly dilated bowel and enterocolitis. In this manuscript, the authors propose the following treatment algorithm: 1) a diverting transverse loop colostomy, 2) a transanal Soave endorectal pull-through, and 3) colostomy closure. The authors report their experience with five patients.
文摘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,etc.The diagnosis of HD particularly warrants invasive testing to confirm the diagnosis,such as anorectal manometry or rectal suction biopsy.What 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 life.The presentation is more atypical,with perinatal constipation rather than with bloody stools,diarrhea,and vomiting.The diagnosis and management would be avoidance of cow's milk protein within the diet,with results and symptom improvement in patients immediately.Therefore,we discuss whether an alternative pathway to address perinatal constipation should be further discussed and implemented to potentially avoid invasive techniques in patients.This entails first ruling out CMPA with safe,noninvasive techniques with diet modification,and if unsuccessful,then moving forward with further diagnostic modalities.
