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In two of these cases, two anastomoses were performed at the oral side to add graft—duodenal or graft—jejunal anastomosis to graft—gastric anastomosis. It was difficult, however, to establish oral intake in both cases. Only one of the three patients survived. Small bowel transplantation may be the only treatment for severe CIIP, but is difficult to establish oral intake after transplantation.

As for the current situation regarding small bowel transplantation for CIIP in Japan, multiple organ transplantation is not feasible. Isolated small bowel transplantation should be carefully provided with close consideration of the appropriate anastomosis approach to ensure gastric emptying function in each case. Of these cases, there were some in which disease control was obtained with oral intake alone, in which normal growth or only mild growth disorder was observed, and in which school attendance was achieved.

The actual prognosis of CIIP that develops in childhood is not poor, but adequate nutritional support and intestinal stoma care are required for a long period in many cases. Even for outpatients, the requirement of i. The individual author contributions to the establishment of the guidelines and the author affiliations are listed in Table S1. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors.

Any queries other than missing content should be directed to the corresponding author for the article. Volume 60 , Issue 5. The full text of this article hosted at iucr. If you do not receive an email within 10 minutes, your email address may not be registered, and you may need to create a new Wiley Online Library account. If the address matches an existing account you will receive an email with instructions to retrieve your username. Pediatrics International. Guidelines Free Access. Hiroshi Matsufuji Corresponding Author E-mail address: matufuhi luke.

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Tools Request permission Export citation Add to favorites Track citation. Share Give access Share full text access. Share full text access. Please review our Terms and Conditions of Use and check box below to share full-text version of article. Abstract Background Despite the presence of ganglion cells in the rectum, some patients have symptoms similar to those of Hirschsprung's disease.

Results We herein outline the newly established Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease. Conclusions Clinical practice recommendations for allied disorders of Hirschprung's disease are given for each CQ, along with an assessment of the current evidence. AchE, acetylcholinesterase; HE, hematoxylin and eosin. The size of intestinal nerve plexus is small and the number of ganglion cells is low in patients with isolated hypoganglionosis.

The individual ganglion cells are small during the neonatal period, but they grow over time as the patient matures, although their number will never increase. HE, hematoxylin and eosin. Functional ileus caused by systemic illness or the aforementioned drugs needs to be ruled out for the diagnosis of idiopathy. Supporting Information. Filename Description pedsupSupInfo. Pseudo Hirschsprung's disease. Google Scholar. PubMed Google Scholar. Crossref Google Scholar. Crossref PubMed Google Scholar. Citing Literature. Volume 60 , Issue 5 May Pages References Related Information.

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Old Password. Holschneider AM, Puri P, eds. In: Hirschsprung Disease and Allied Disorders. New York, NY: Springer; Clinical features' diagnostics and treatment of Hirschsprung's disease in adults. Colorectal Dis. Hirschsprung's disease: associated abnormalities and demography. A prospective observational study of associated anomalies in Hirschsprung's disease. Orphanet J Rare Dis. Yanchar NL, Soucy P. Long-term outcome after Hirschsprung's disease: patients' perspectives.

De la Torre L, Ortega A. Transanal versus open endorectal pull-through for Hirschsprung's disease.

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Management of Hirschsprung's disease in children with trisomy Am J Surg. The influence of Down's syndrome on the management and outcome of children with Hirschsprung's disease. Why do the patients with Hirschsprung disease get redo pull-through operation?. Eur J Pediatr Surg. Long-term results of bowel function after treatment for Hirschsprung's disease: a year review. The surgical treatment of toxic megacolon in Hirschsprung disease.

Pediatr Emerg Care. Dasgupta R, Langer JC. Hirschsprung disease. Curr Probl Surg. Friedmacher F, Puri P. Residual aganglionosis after pull-through operation for Hirschsprung's disease: a systematic review and meta-analysis. Langer JC. Repeat pull-through surgery for complicated Hirschsprung's disease: indications, techniques, and results. Reliability of intraoperative frozen sections in the management of Hirschsprung's disease.

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Hirschsprung's Disease and Allied Disorders by Prem Puri | | Booktopia

Acquired aganglionosis following surgery for Hirschsprung's disease: a report of five cases during a year experience with pull-through procedures. Reoperative surgery for Hirschsprung's disease. Semin Pediatr Surg. Hirschsprung's disease and intestinal neuronal dysplasia--a frequent association with implications for the postoperative course.

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Colonic motility after surgery for Hirschsprung's disease. Am J Gastroenterol. A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung's disease. Anal achalasia after pull-through operations for Hirschsprung's disease -- preliminary experience with topical nitric oxide. Percutaneous cecostomy: updates in technique and patient care. How accurate is prenatal sonography for the diagnosis of imperforate anus and Hirschsprung disease?.

Hirschsprung’s Disease: A Team Approach to Surgery and Care

The pathophysiology of Hirschsprung's-associated enterocolitis: importance of histologic correlates. A new diagnostic scoring system to differentiate Hirschsprung's disease from Hirschsprung's disease-allied disorders in patients with suspected intestinal dysganglionosis. Int J Colorectal Dis. Smith GH, Cass D. Infantile Hirschsprung disease - is barium enema useful?.


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Colonic manometry in children with defecatory disorders. Role in diagnosis and management. Acetylcholinesterase histochemistry AChE --a helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease. Diagn Pathol. Calretinin immunohistochemistry versus acetylcholinesterase histochemistry in the evaluation of suction rectal biopsies for Hirschsprung Disease. Pediatr Dev Pathol. Calretinin immunohistochemistry: a simple and efficient tool to diagnose Hirschsprung disease.

Mod Pathol. Diagnostic value of rectal suction biopsies using calretinin immunohistochemical staining in Hirschsprung's disease. Calretinin and microtubule-associated protein-2 MAP-2 immunohistochemistry in the diagnosis of Hirschsprung's disease. Redo pullthrough for Hirschsprung disease.