Abstract

Hirschsprung disease (HSCR), represents a neurocristopathy, secondary to impairment in migration, proliferation, differentiation in addition to survival of the neural crest cells resulting in gut agangliosis. Its presentation is usually just following birth having an impact on 1/5000 live births all over the world. a significant enhancement in our insight with regards to genetics in addition to correlation with congenital anomalies, possessing a common etiopathogenetic mode of impairment of the generation of neural crest. Besides that various kinds of cell populations like Mast Cells as well as Interstitial cells of Cajal that do not take origin from the neural crest, yet aid In the generation of HSCR have got detailed. Further scientists have Concentrated on the variants of HSCR, that might simulate the Clinical signs but are separate conditions, that possess different treatment strategies in addition to separate prognosis. Thus we decided to conduct a systematic review, where we utilized the search engine pubmed, google scholar; web of science; embase; Cochrane review library utilizing the MeSH terms like embryonic; Early Diagnosis; enteric nervous system; neural crest cells; Vagal; agangliosis; hypogangliosis; dysgangliosis; receptor tyrosine kinase RET in ENS migration; Retinoic acid; congenital-hypothyroidism; thymus; Mast Cells; Interstitial cells of Cajal; associated congenital anomalies; pseudo- Hirschsprung disease animal studies; human studies; genetics; stem cells; induced pluripotent stem cells (iPSCs) variant HSCR; from 1985 to 2021 till date. We found a total of 10, 000 articles out of which we selected 67 articles for this updated review. No meta-analysis was done. thus early Diagnosis is essential, with surgical removal of the aganglioic area of the intestine. of Transanal endorectal pull-through (TEPT) is believed to be the better procedure yet several patients experience continuation of symptoms patients. Thus we have tried to discuss the future enhancement of results with the utilization of stem cells, induced pluripotent stem cells (iPSCs) in addition to tissue engineering for aid for the ones that experience recurrence In 30-50% post surgery.

Keywords: Hirschsprung Disease; Neural Crest Cells; Neurocristopathy; Mast Cells; Interstitial Cells of Cajal

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Citation

Citation: Kulvinder Kochar Kaur., et al. “An Update on the Insight of Embryonic, Etiopathogenesis, Current Surgical Advances Besides the Advocated ERNICA Guidelines for the Management of Rectosigmoid Hirschsprung Disease, and Clinical Referral Score Model for Early Diagnosis with Future Use of Stem Cells - A Systematic Review".Acta Scientific Ophthalmology 4.11 (2021): 64-72.

Copyright

Copyright: © 2021 Kulvinder Kochar Kaur., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.