Hirschsprung's Disease

Rescorla, Frederick J.; Morrison, Albert M.; Engles, Drew; West, Karen W.; Grosfeld, Jay L.

doi:10.1001/archsurg.1992.01420080068011

Cited by 193 publications

(13 citation statements)

References 58 publications

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“…Data have shown that trisomy 21, HAEC, and TCA increase the risk for death, and in this study, the risk for death is still significantly increased although when adjusted for Down syndrome [ 3 , 6 , 7 ]. In addition, assessing the ICD classifications of causes of death indicates a difference between the exposed cohort and the unexposed.…”

Section: Discussionmentioning

confidence: 64%

“…The mortality in HSCR patients undergoing one-stage transanal pull-through varies between 0 and 2% [ 4 , 5 ]. Patients with Down syndrome (DS), total colonic aganglionosis (TCA), and Hirschsprung-associated enterocolitis (HAEC) seem to have an increased risk of mortality, as well as patients with anastomotic leakage after the pull-through [ 3 , 6 , 7 ]. HAEC is the most threatening complication of HSCR, since morbidity and mortality are possible outcomes [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Mortality in Swedish patients with Hirschsprung disease

Granström

Wester

2017

Pediatr Surg Int

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PurposeHirschsprung disease (HSCR) has previously been associated with increased mortality. The aim of this study was to assess mortality in patients with Hirschsprung disease in a population-based cohort.MethodsThis was a nationwide, population-based cohort study. The study exposure was HSCR and the study outcome was death. The cohort included all individuals with HSCR registered in the Swedish National Patient Register between 1964 and 2013 and ten age- and sex-matched controls per patient, randomly selected from the Population Register. Mortality and cause of death were assessed using the Swedish National Causes of Death Register.ResultsThe cohort comprised 739 individuals with HSCR (565 male) and 7390 controls (5650 male). Median age of the cohort was 19 years (range 2–49). Twenty-two (3.0%) individuals with HSCR had died at median age 2.5 years (range 0–35) compared to 49 (0.7%) controls at median age 20 years (0–44), p < 0.001. Hazard ratio for death in HSCR patients compared to healthy controls was 4.77 (confidence interval (CI) 95% 2.87–7.91), and when adjusted for Down syndrome, the hazard ratio was 3.6 (CI 95% 2.04–6.37).ConclusionsThe mortality rate in the HSCR cohort was 3%, which was higher than in controls also when data were adjusted for Down syndrome.

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Section: Discussionmentioning

confidence: 64%

Section: Introductionmentioning

confidence: 99%

Mortality in Swedish patients with Hirschsprung disease

Granström

Wester

2017

Pediatr Surg Int

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“…Postoperative manometric study showed some improvement in anal sensation as late as four years after the pull-through surgery. [18] In our study, we had five patients with intact rectoanal inhibitory reflex, after at least four years post-surgery. This is an intramural local axon reflex controlled by spinal and higher centers but it persists even after loss of extrinsic control.…”

Section: Discussionmentioning

confidence: 99%

Functional outcome after Swenson′s operation for Hirshsprung′s disease

El-Hak

Hemaly

Negm

et al. 2010

Saudi J Gastroenterol

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Background/Aim:Hirschsprung's disease (HD) is one of the most common causes resulting in lower intestinal obstruction in children with atypical clinical symptoms and inconspicuous morphological findings by barium enema X-ray. Recently, this situation has been largely ameliorated by improvement of instrument for measurement of anorectal pressure. By now, anorectal manometry has been regarded as a routine means for functional assessment and diagnosis of HD. It is accurate in nearly all cases of HD with characteristic absence of rectoanal inhibitory reflex. Different surgical modalities of treatment are available and Swenson's operation is one of the surgical procedures done for HD. Anorectal manometric findings may change after Swenson's operation with improvement of rectoanal inhibitory reflex in some cases. We aimed to evaluate functional results after Swenson's operation for HD using anorectal manometry.Patients and Methods:Between 1996 and 2005, 52 patients were diagnosed with HD and operated upon by Swenson's operation in Gastroenterology Center, Mansoura University. There were 33 males (63.46%) and 19 females (36.54%) with a mean age of 3.29 ± 1.6, (range 2-17 years). Anorectal manometry and rectal muscle biopsy were done preoperatively for diagnosis but after operation anorectal manometry was done after every six months and then yearly.Results:All of the 52 patients showed absent rectoanal inhibitory reflex on manometric study with relatively higher resting anal canal pressure and within normal squeeze pressure. Postoperatively, there were 35 continent patients (67.31%) with 11 patients (21.15%) showing minor incontinence and six (11.54%) with major incontinence. On the other side, there were five patients (9.62%) with persistent constipation after operation (three due to anal stricture and two due to residual aganglionosis). Postoperative manometric study showed some improvement in anal sensation with the rectoanal inhibitory reflex becoming intact in six patients (11.54%) four years after operation.Conclusion:Anorectal manometry is a more reliable method for diagnosis of HD than barium enema X-ray but for final diagnosis, it is reasonable to combine anorectal manometry with tissue biopsy. Functional outcome after Swenson's operation for HD may improve in some patients complaining of incontinence or constipation. Anorectal manometry may show improvement of the parameters after Swenson's operation.

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“…Despite surgical treatment available for Hirschsprung disease since 1948 (Swenson and Bill, 1948), approximately 5% of children with HSCR die at an early age (Rescorla et al, 1992; Suita et al, 2005) and > 40% have problems after surgery (El-Sawaf et al, 2013; Menezes et al, 2008). It would therefore be ideal if HSCR could be prevented from occurring in the first place.…”

Section: Discussionmentioning

confidence: 99%

Ibuprofen slows migration and inhibits bowel colonization by enteric nervous system precursors in zebrafish, chick and mouse

Schill

Lake

Tusheva

et al. 2016

Developmental Biology

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Hirschsprung Disease (HSCR) is a potentially deadly birth defect characterized by the absence of the enteric nervous system (ENS) in distal bowel. Although HSCR has clear genetic causes, no HSCR-associated mutation is 100% penetrant, suggesting gene-gene and gene-environment interactions determine HSCR occurrence. To test the hypothesis that certain medicines might alter HSCR risk we treated zebrafish with medications commonly used during early human pregnancy and discovered that ibuprofen caused HSCR-like absence of enteric neurons in distal bowel. Using fetal CF-1 mouse gut slice cultures, we found that ibuprofen treated enteric neural crest-derived cells (ENCDC) had reduced migration, fewer lamellipodia and lower levels of active RAC1/CDC42. Additionally, inhibiting ROCK, a RHOA effector and known RAC1 antagonist, reversed ibuprofen effects on migrating mouse ENCDC in culture. Ibuprofen also inhibited colonization of Ret+/− mouse bowel by ENCDC in vivo and dramatically reduced bowel colonization by chick ENCDC in culture. Interestingly, ibuprofen did not affect ENCDC migration until after at least three hours of exposure. Furthermore, mice deficient in Ptgs1 (COX 1) and Ptgs2 (COX 2) had normal bowel colonization by ENCDC and normal ENCDC migration in vitro suggesting COX-independent effects. Consistent with selective and strain specific effects on ENCDC, ibuprofen did not affect migration of gut mesenchymal cells, NIH3T3, or WT C57BL/6 ENCDC, and did not affect dorsal root ganglion cell precursor migration in zebrafish. Thus, ibuprofen inhibits ENCDC migration in vitro and bowel colonization by ENCDC in vivo in zebrafish, mouse and chick, but there are cell type and strain specific responses. These data raise concern that ibuprofen may increase Hirschsprung disease risk in some genetically susceptible children.

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Hirschsprung's Disease

Cited by 193 publications

References 58 publications

Mortality in Swedish patients with Hirschsprung disease

Mortality in Swedish patients with Hirschsprung disease

Functional outcome after Swenson′s operation for Hirshsprung′s disease

Ibuprofen slows migration and inhibits bowel colonization by enteric nervous system precursors in zebrafish, chick and mouse

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