Difficulties in Histological diagnosis and treatment of Hirschprung’s disease

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 Traditionally Hirschsprung´s disease has been diagnosed and treated under the paradigm of the absence of submucosal and myenteric nerve plexus in a localized area of the colon. Surgical treatment consists of resection of the aganglionic area and anastomosis of the anal level of the ganglio...

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Detalles Bibliográficos
Autores: More-Flores, Mario Marcelino, Paico-Vílchez, Emiliano
Formato: artículo
Fecha de Publicación:2020
Institución:Sociedad Peruana de Medicina Interna
Repositorio:Revista de la Sociedad Peruana de Medicina Interna
Lenguaje:español
OAI Identifier:oai:medicinainterna.net.pe:article/561
Enlace del recurso:https://revistamedicinainterna.net/index.php/spmi/article/view/561
Nivel de acceso:acceso abierto
Materia:Enfermedad de Hirschsprung
colon
aganglionar
plexo mientérico
Hirschsprung’s disease
myenteric plexus
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spelling Difficulties in Histological diagnosis and treatment of Hirschprung’s diseaseDificultades en el diagnóstico histológico y en el tratamiento de la enfermedad de HirschprungMore-Flores, Mario MarcelinoPaico-Vílchez, EmilianoEnfermedad de Hirschsprungcolonaganglionarplexo mientéricoHirschsprung’s diseasecolonaganglionarmyenteric plexus Traditionally Hirschsprung´s disease has been diagnosed and treated under the paradigm of the absence of submucosal and myenteric nerve plexus in a localized area of the colon. Surgical treatment consists of resection of the aganglionic area and anastomosis of the anal level of the ganglionic region of  the colon. The review of the long-term results of the surgery, with the various surgical techniques existing up to now show complications related to incontinence and constipation in about 50 % of the cases. Many surgeons think that this disease is still unknown and even incurable. With the advancement in the field of research in molecular biology in the been discovered that addition to the absence of the nerve plexuses, and the Cajal cells in the aganglionic segment, failure in the secretion of mediators called purinergic, such as the ATP and its receptors, as well nitrate substance such as nitric oxide, which intervene in the relaxations of the intestinal muscle. On the other hand, research in the field of genetics has allowed us to know the role of pro-oncogenes mutations such as RET and others, in the genesis of Hirschsprung´s disease. In this paper. A review of the above-mentioned concepts is made and the difficulties in the histological diagnosis of the disease are discussed and a rational and intelligent approach is proposed, found in the reviewed literature.Tradicionalmente la enfermedad de Hirschsprung, se diagnostica y trata bajo el paradigma de la ausencia de plexos nerviosos submucoso y mientérico en una zona localizada del colon. El tratamiento quirúrgico consiste en la resección del colon agangliónico y la anastomosis del segmento gangliónico descendido con el ano. La revisión de los resultados a largo plazo de la cirugía, con las diversas técnicas quirúrgicas hasta hoy existentes, muestran complicaciones relacionadas a incontinencia y estreñimiento en cerca del 50 % de los casos. Muchos cirujanos piensan que esta enfermedad es aún desconocida e incluso incurable. En base a investigaciones de biología molecular, ahora se conoce que, además de la ausencia de los plexos nerviosos y de las células de Cajal en el segmento agangliónico, existe falla en la secreción de mediadores denominados purinérgicos, como el ATP y sus receptores, así como también de las sustancias nitraérgicas (óxido nítrico), que intervienen en la relajación del músculo intestinal. Por otro lado, la investigación en el campo de la genética nos ha permitido conocer el rol de las mutaciones de prooncoenes, como el RET y otros, en la génesis de la enfermedad de Hirschsprung. En el presente trabajo se hace una revisión de los conceptos arriba mencionados y se discuten las dificultades en el diagnóstico histológico de la enfermedad y se propone un enfoque racional e inteligente, encontrado en la literatura revisada.Sociedad Peruana de Medicina Interna2020-12-19info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://revistamedicinainterna.net/index.php/spmi/article/view/56110.36393/spmi.v33i4.561Revista de la Sociedad Peruana de Medicina Interna; Vol. 33 Núm. 4 (2020); 146-150Revista de la Sociedad Peruana de Medicina Interna; Vol. 33 No. 4 (2020); 146-1501609-71731681-972110.36393/spmi.v33i4reponame:Revista de la Sociedad Peruana de Medicina Internainstname:Sociedad Peruana de Medicina Internainstacron:SPMIspahttps://revistamedicinainterna.net/index.php/spmi/article/view/561/633Derechos de autor 2020 Mario Marcelino More-Flores, Emiliano Paico-Vílchezinfo:eu-repo/semantics/openAccessoai:medicinainterna.net.pe:article/5612021-11-13T22:06:34Z
dc.title.none.fl_str_mv Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
Dificultades en el diagnóstico histológico y en el tratamiento de la enfermedad de Hirschprung
title Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
spellingShingle Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
More-Flores, Mario Marcelino
Enfermedad de Hirschsprung
colon
aganglionar
plexo mientérico
Hirschsprung’s disease
colon
aganglionar
myenteric plexus
title_short Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
title_full Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
title_fullStr Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
title_full_unstemmed Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
title_sort Difficulties in Histological diagnosis and treatment of Hirschprung’s disease
dc.creator.none.fl_str_mv More-Flores, Mario Marcelino
Paico-Vílchez, Emiliano
author More-Flores, Mario Marcelino
author_facet More-Flores, Mario Marcelino
Paico-Vílchez, Emiliano
author_role author
author2 Paico-Vílchez, Emiliano
author2_role author
dc.subject.none.fl_str_mv Enfermedad de Hirschsprung
colon
aganglionar
plexo mientérico
Hirschsprung’s disease
colon
aganglionar
myenteric plexus
topic Enfermedad de Hirschsprung
colon
aganglionar
plexo mientérico
Hirschsprung’s disease
colon
aganglionar
myenteric plexus
description  Traditionally Hirschsprung´s disease has been diagnosed and treated under the paradigm of the absence of submucosal and myenteric nerve plexus in a localized area of the colon. Surgical treatment consists of resection of the aganglionic area and anastomosis of the anal level of the ganglionic region of  the colon. The review of the long-term results of the surgery, with the various surgical techniques existing up to now show complications related to incontinence and constipation in about 50 % of the cases. Many surgeons think that this disease is still unknown and even incurable. With the advancement in the field of research in molecular biology in the been discovered that addition to the absence of the nerve plexuses, and the Cajal cells in the aganglionic segment, failure in the secretion of mediators called purinergic, such as the ATP and its receptors, as well nitrate substance such as nitric oxide, which intervene in the relaxations of the intestinal muscle. On the other hand, research in the field of genetics has allowed us to know the role of pro-oncogenes mutations such as RET and others, in the genesis of Hirschsprung´s disease. In this paper. A review of the above-mentioned concepts is made and the difficulties in the histological diagnosis of the disease are discussed and a rational and intelligent approach is proposed, found in the reviewed literature.
publishDate 2020
dc.date.none.fl_str_mv 2020-12-19
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://revistamedicinainterna.net/index.php/spmi/article/view/561
10.36393/spmi.v33i4.561
url https://revistamedicinainterna.net/index.php/spmi/article/view/561
identifier_str_mv 10.36393/spmi.v33i4.561
dc.language.none.fl_str_mv spa
language spa
dc.relation.none.fl_str_mv https://revistamedicinainterna.net/index.php/spmi/article/view/561/633
dc.rights.none.fl_str_mv Derechos de autor 2020 Mario Marcelino More-Flores, Emiliano Paico-Vílchez
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Derechos de autor 2020 Mario Marcelino More-Flores, Emiliano Paico-Vílchez
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Sociedad Peruana de Medicina Interna
publisher.none.fl_str_mv Sociedad Peruana de Medicina Interna
dc.source.none.fl_str_mv Revista de la Sociedad Peruana de Medicina Interna; Vol. 33 Núm. 4 (2020); 146-150
Revista de la Sociedad Peruana de Medicina Interna; Vol. 33 No. 4 (2020); 146-150
1609-7173
1681-9721
10.36393/spmi.v33i4
reponame:Revista de la Sociedad Peruana de Medicina Interna
instname:Sociedad Peruana de Medicina Interna
instacron:SPMI
instname_str Sociedad Peruana de Medicina Interna
instacron_str SPMI
institution SPMI
reponame_str Revista de la Sociedad Peruana de Medicina Interna
collection Revista de la Sociedad Peruana de Medicina Interna
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repository.mail.fl_str_mv
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