HIRSCHSPRUNG’S DISEASE IN CHILDREN
##plugins.pubIds.doi.readerDisplayName##:
https://doi.org/10.61699/cjmps-v1-i4-p38-43关键词:
Hirschsprung’s disease, Megacolon, Congenital megacolon, Toxic megacolon摘要
Hirschsprung’s disease – megacolon - is a congenital anomaly, characterized by theabsence of nerve cells - ganglia in certain segments of the intestine, which is accompaniedby complete or partial functional obstruction of the intestines.Among the clinical complaints, it is worth noting: weight loss, it is possible to developanorexia, various types of physical development disorders, chronic constipation, flatulence,big belly and defecation disorders cause nervous system dysfunction.Early diagnosis is important, the longer the diagnosis is delayed, the greater the damagesymptoms and the risk of developing toxic processes - toxic megacolon, whichsometimes ends with fatal results.The aim of our work today is to diagnose 3-year-old and 15-year-old patients with similarclinical complaints.On the X-rays, the expansion of the rectum and sigmoid colon was observed, the mucousmembrane is rough, the sigmoid colon is long, the intestines remain dilated afterdefecation, a significant part of the contrast agent is in the intestines, the functionalcondition of the intestines is impaired - congenital megacolon - Hirschsprung’s disease.
参考
Mueller JL, Goldstein AM. The science of Hirschsprung disease: What we know and where we are headed. Semin Pediatr Surg. 2022;31(2):151157. doi:10.1016/j.sempedsurg.2022.151157
Urla C, Lieber J, Obermayr F, et al. Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome. BMC Surg. 2018;18(1):58. Published 2018 Aug 15. doi:10.1186/s12893-018-0383-6
Godbole K. Many faces of Hirschsprung’s disease. Indian Pediatr. 2004;41(11):1115-1123.
Mayo Clinic Family Health Book, 5th Ed: Completely Revised and Updated. Mayo Clinic Press, 2018
Amiel J, Lyonnet S. Hirschsprung disease, associated syndromes, and genetics: a review. J Med Genet. 2001;38:729-39.
Parisi MA, Kapur RP. Genetics of Hirschsprung disease. Curr Opin Pediatr. 2000;12:610-7.
Darejan G. Sturua, Nino J. Jojua, Tamar T. Dundua
Heuckeroth RO. Hirschsprung disease—integrating basic science and clinical medicine to improve outcomes. Nature Reviews Gastroenterology and Hepatology. 2018;15(3):152–167. doi: 10.1038/nrgastro.2017.149
Ambartsumyan L, Smith C, Kapur RP. Diagnosis of Hirschsprung disease. Pediatric Developmental Pathology. 2020;23(1):8–22. doi: 10.1177/1093526619892351
