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Abnormal enteric ganglion morphology

MedGen UID:
927580
•Concept ID:
C4293671
•
Anatomical Abnormality
Synonyms: Abnormality of enteric ganglion morphology; Abnormality of the enteric ganglia
 
HPO: HP:0004362

Definition

An abnormality of the enteric nervous system, which comprises two types of ganglia, the myenteric (Auerbach's) and submucosal (Meissner's) plexuses. The enteric nervous system functions to control gut movement, fluid exchange between the gut and its lumen, and local blood flow. [from HPO]

Professional guidelines

PubMed

Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017.
Muto M, Matsufuji H, Taguchi T, Tomomasa T, Nio M, Tamai H, Tamura M, Sago H, Toki A, Nosaka S, Kuroda T, Yoshida M, Nakajima A, Kobayashi H, Sou H, Masumoto K, Watanabe Y, Kanamori Y, Hamada Y, Yamataka A, Shimojima N, Kubota A, Ushijima K, Haruma K, Fukudo S, Araki Y, Kudo T, Obata S, Sumita W, Watanabe T, Fukahori S, Fujii Y, Yamada Y, Jimbo K, Kawai F, Fukuoka T, Onuma S, Morizane T, Ieiri S, Esumi G, Jimbo T, Yamasaki T
Pediatr Int 2018 May;60(5):400-410. doi: 10.1111/ped.13559. PMID: 29878629
Hirschsprung's disease: current management and prospects for transplantation of enteric nervous system progenitor cells.
Theocharatos S, Kenny SE
Early Hum Dev 2008 Dec;84(12):801-4. Epub 2008 Oct 2 doi: 10.1016/j.earlhumdev.2008.09.007. PMID: 18835114
Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B).
Meier-Ruge WA, Brönnimann PB, Gambazzi F, Schmid PC, Schmidt CP, Stoss F
Virchows Arch 1995;426(6):549-56. doi: 10.1007/BF00192108. PMID: 7655734

Recent clinical studies

Etiology

Histopathology in gastrointestinal neuromuscular diseases: methodological and ontological issues.
Bernardini N, Ippolito C, Segnani C, Mattii L, Bassotti G, Villanacci V, Blandizzi C, Dolfi A
Adv Anat Pathol 2013 Jan;20(1):17-31. doi: 10.1097/PAP.0b013e31827b65c0. PMID: 23232568
Developmental determinants of the independence and complexity of the enteric nervous system.
Gershon MD
Trends Neurosci 2010 Oct;33(10):446-56. Epub 2010 Jul 13 doi: 10.1016/j.tins.2010.06.002. PMID: 20633936
The histopathology of gastrointestinal motility disorders in children.
Feichter S, Meier-Ruge WA, Bruder E
Semin Pediatr Surg 2009 Nov;18(4):206-11. doi: 10.1053/j.sempedsurg.2009.07.002. PMID: 19782302
Enteric ganglioneuritis and abnormal interstitial cells of Cajal: features of inflammatory bowel disease.
Ohlsson B, Veress B, Lindgren S, Sundkvist G
Inflamm Bowel Dis 2007 Jun;13(6):721-6. doi: 10.1002/ibd.20095. PMID: 17230538
Variant Hirschsprung's disease.
Puri P, Rolle U
Semin Pediatr Surg 2004 Nov;13(4):293-9. doi: 10.1053/j.sempedsurg.2004.10.017. PMID: 15660323

Diagnosis

Acetylcholinesterase staining for the pathological diagnosis of Hirschsprung's disease.
Yoshimaru K, Yanagi Y, Obata S, Takahashi Y, Irie K, Omori A, Matsuura T, Taguchi T
Surg Today 2021 Feb;51(2):181-186. Epub 2020 Jun 23 doi: 10.1007/s00595-020-02055-x. PMID: 32577882
Hirschsprung's disease: clinical dysmorphology, genes, micro-RNAs, and future perspectives.
Sergi CM, Caluseriu O, McColl H, Eisenstat DD
Pediatr Res 2017 Jan;81(1-2):177-191. Epub 2016 Sep 28 doi: 10.1038/pr.2016.202. PMID: 27682968
Nonruminant Nutrition Symposium: Involvement of gut neural and endocrine systems in pathological disorders of the digestive tract.
Furness JB, Poole DP
J Anim Sci 2012 Apr;90(4):1203-12. Epub 2011 Dec 16 doi: 10.2527/jas.2011-4825. PMID: 22178854
Variant Hirschsprung's disease.
Puri P, Rolle U
Semin Pediatr Surg 2004 Nov;13(4):293-9. doi: 10.1053/j.sempedsurg.2004.10.017. PMID: 15660323
Hirschprung's disease.
Sullivan PB
Arch Dis Child 1996 Jan;74(1):5-7. doi: 10.1136/adc.74.1.5. PMID: 8660047Free PMC Article

Therapy

Cholinergic Signaling Attenuates Pro-Inflammatory Interleukin-8 Response in Colonic Epithelial Cells.
Müller I, Kym U, Galati V, Tharakan S, Subotic U, Krebs T, Stathopoulos E, Schmittenbecher P, Cholewa D, Romero P, Reingruber B; NIGStudy Group, Holland-Cunz S, Keck S
Front Immunol 2021;12:781147. Epub 2022 Jan 6 doi: 10.3389/fimmu.2021.781147. PMID: 35069554Free PMC Article
Visualization of the human enteric nervous system by probe confocal laser endomicroscopy: a first real-time observation of Hirschsprung's disease and allied disorders.
Harada A, Shimojima N, Kobayashi M, Kamba S, Hirobe S, Sumiyama K
BMC Med Imaging 2021 Jul 31;21(1):118. doi: 10.1186/s12880-021-00648-7. PMID: 34332524Free PMC Article
Comparative proteomics of Hirschsprung's disease.
Fan Y, Wang L, Zhang Y, Huang T, Li H, Gu H, Wang W, Yuan Z
J Proteomics 2013 Jun 12;84:176-84. Epub 2013 Apr 3 doi: 10.1016/j.jprot.2013.03.024. PMID: 23563203
Mast cells and gut nerve development: implications for Hirschsprung's disease and intestinal neuronal dysplasia.
Kobayashi H, Yamataka A, Fujimoto T, Lane GJ, Miyano T
J Pediatr Surg 1999 Apr;34(4):543-8. doi: 10.1016/s0022-3468(99)90069-6. PMID: 10235318
Role of opioid ligands in the irritable bowel syndrome.
Corazziari E
Can J Gastroenterol 1999 Mar;13 Suppl A:71A-75A. doi: 10.1155/1999/598659. PMID: 10202212

Prognosis

Analysis of enteric nervous system and intestinal epithelial barrier to predict complications in Hirschsprung's disease.
Dariel A, Grynberg L, Auger M, Lefèvre C, Durand T, Aubert P, Le Berre-Scoul C, Venara A, Suply E, Leclair MD, de Vries P, Levard G, Parmentier B, Podevin G, Schmitt F, Couvrat V, Irtan S, Hervieux E, Villemagne T, Lardy H, Capito C, Muller C, Sarnacki S, Mosnier JF, Galmiche L, Derkinderen P, Boudin H, Brochard C, Neunlist M
Sci Rep 2020 Dec 10;10(1):21725. doi: 10.1038/s41598-020-78340-z. PMID: 33303794Free PMC Article
Simple rules for a "simple" nervous system? Molecular and biomathematical approaches to enteric nervous system formation and malformation.
Newgreen DF, Dufour S, Howard MJ, Landman KA
Dev Biol 2013 Oct 1;382(1):305-19. Epub 2013 Jul 6 doi: 10.1016/j.ydbio.2013.06.029. PMID: 23838398Free PMC Article
Enteric ganglioneuritis and abnormal interstitial cells of Cajal: features of inflammatory bowel disease.
Ohlsson B, Veress B, Lindgren S, Sundkvist G
Inflamm Bowel Dis 2007 Jun;13(6):721-6. doi: 10.1002/ibd.20095. PMID: 17230538
Variant Hirschsprung's disease.
Puri P, Rolle U
Semin Pediatr Surg 2004 Nov;13(4):293-9. doi: 10.1053/j.sempedsurg.2004.10.017. PMID: 15660323
Updated results on intestinal neuronal dysplasia (IND B).
Meier-Ruge WA, Ammann K, Bruder E, Holschneider AM, Schärli AF, Schmittenbecher PP, Stoss F
Eur J Pediatr Surg 2004 Dec;14(6):384-91. doi: 10.1055/s-2004-821120. PMID: 15630639

Clinical prediction guides

Glial Cell-Derived Neurotrophic Factor Induces Enteric Neurogenesis and Improves Colon Structure and Function in Mouse Models of Hirschsprung Disease.
Soret R, Schneider S, Bernas G, Christophers B, Souchkova O, Charrier B, Righini-Grunder F, Aspirot A, Landry M, Kembel SW, Faure C, Heuckeroth RO, Pilon N
Gastroenterology 2020 Nov;159(5):1824-1838.e17. Epub 2020 Jul 17 doi: 10.1053/j.gastro.2020.07.018. PMID: 32687927
Immature enteric ganglion cells were observed in a 13-year-old colon signet ring cell carcinoma patient: A case report and literature review.
Li H, Huang K, Wang H, Wang L, Yang M, Wang L, Lin R, Liu H, Gao J, Shuai X, Liu X, Tao K, Wang G, Wang Z
Medicine (Baltimore) 2017 Jun;96(25):e7036. doi: 10.1097/MD.0000000000007036. PMID: 28640080Free PMC Article
Simple rules for a "simple" nervous system? Molecular and biomathematical approaches to enteric nervous system formation and malformation.
Newgreen DF, Dufour S, Howard MJ, Landman KA
Dev Biol 2013 Oct 1;382(1):305-19. Epub 2013 Jul 6 doi: 10.1016/j.ydbio.2013.06.029. PMID: 23838398Free PMC Article
Advances in molecular genetics of Hirschsprung's disease.
Pan ZW, Li JC
Anat Rec (Hoboken) 2012 Oct;295(10):1628-38. Epub 2012 Jul 19 doi: 10.1002/ar.22538. PMID: 22815266
Enteric neuroplasticity evoked by inflammation.
Vasina V, Barbara G, Talamonti L, Stanghellini V, Corinaldesi R, Tonini M, De Ponti F, De Giorgio R
Auton Neurosci 2006 Jun 30;126-127:264-72. Epub 2006 Apr 19 doi: 10.1016/j.autneu.2006.02.025. PMID: 16624634

Recent systematic reviews

Skip segment Hirschsprung's disease: a systematic review.
O'Donnell AM, Puri P
Pediatr Surg Int 2010 Nov;26(11):1065-9. doi: 10.1007/s00383-010-2692-4. PMID: 20714729

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