Cystic fibrosis complicated with meconial ileus and intestinal inversion
https://doi.org/10.52485/19986173_2022_4_151
Abstract
Cystic fibrosis (CF) is a severe, progressive, multisystemic disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. Meconium intestinal obstruction is often the first manifestation of CF and occurs in about 20% of patients. This situation undoubtedly requires close attention from specialists who observe children with suspected CF, since early diagnosis is important for optimizing both short-term and long-term treatment. The article describes a clinical case of meconium ileus complicated by intestinal volvulus.
About the Authors
A. I. Markovskaya
Chita State Medical Academy
Russian Federation
Russian Federation
39а Gorky Street, Chita, 672000
N. L. Potapova
Chita State Medical Academy
Russian Federation
Russian Federation
39а Gorky Street, Chita, 672000
A. V. Ignatyeva
Chita State Medical Academy
Russian Federation
Russian Federation
39а Gorky Street, Chita, 672000
Yu. L. Mizernitsky
Research and Clinical Institute for Pediatrics and Child Surgery at the Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
1 Ostrovytyanova str, Moscow, 117997
References
1. Kondrat'eva E.I., Kashirskaja N.Ju., Kapranov N.I. National Consensus (2nd edition) "Cystic fibrosis: definition, diagnostic criteria, therapy". M. LLC "BORGES Company". 2018. 356. in Russian.
2. Ronin C., Mace P., Stenard F., Loundou A., Capelle M., Mortier M., Pellisier M.C., Sigaudy S., Levy A., Hoffmann P., Merrot Th., Lopater J., Lagausie P., Philip N., Bretelle F. Antenatal prognostic factor of fetal echogenic bowel. Maternal-Fetal Medicine. 2017. 212. 166-170. DOI: 10.1016/j.eogrb.2017.01/060.
3. Cochran W.J. Meconium Ileus. MSD Manual professional version. 2021. (Available on: Meconium Ileus - Pediatrics - MSD Manual Professional Edition (msdmanuals.com), access date 12.06.2022).
4. Dupuis A., Keenan K., Ooi C.Y., Dorfman R., Sontag M.K., Naehrlich L., Castellani C., Strug L.J., Rommens J.M., Gonska T. Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Genet Med. 2016. Apr 18(4). 333-40. DOI: 10.1038/gim.2015.79.
5. Sathe M., Houwen R. Meconium ileus in Cystic Fibrosis. J Cyst Fibros. 2017. Nov 16. Suppl 2. 32-39. DOI: 10.1016/j.jcf.2017.06.007.
6. Scotet V., Duguépéroux I., Audrézet M.P., Audebert-Bellanger S., Muller M., Blayau M. Focus on cystic fibrosis and other disorders evidenced in fetuses with sonographic finding of echogenic bowel: 16-year report from Brittany, France. Am J Obstet Gynecol. 2010 Dec;203(6):592.e1-6. DOI: 10.1016/j.ajog.2010.08.033.
7. Zybert K., Borawska-Kowalczyk U., Wozniacki L., Dawidziuk M., Ołtarzewski M., Sands D.Clinical complications in children with false-negative results in cystic fibrosis newborn screening. J Pediatr. 2021. Dec 22. S0021-7557(21)00169-8. DOI: 10.1016/j.jped.2021.11.007.
8. Lacrosniere S.M., Gerardin M., Clainche-Viala L.L., Houdoui V. Phenotypic Presentations of Cystic Fibrosis in Children of African Descent. Genes. 2021. Mar 23. 12(3). 458. DOI:10.3390/genes12030458.
9. Mentessidou A., Loukou I., Kampouroglou G., Livani A., Georgopoulos I., Mirilas P. Long-term intestinal obstruction sequelae and growth in children with cystic fibrosis operated for meconium ileus: expectancies and surprises. J Pediatr Surg. 2018. Aug. 53(8). 1504-1508. DOI: 10.1016/j.jpedsurg.2017.11.040.
10. Smith A., Ryan E., Keeffe D., Donovan D. Meconium Ileus in Two Irish Newborns: The Presenting Feature of Cystic Fibrosis Ir Med J. 2019 Mar 14.112(3):901.
11. Carlyle B.E, Borowitz D.S, Glick P.L. A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr Surg. 2012. 47(4):772-81.
12. Kucherov Ju.I., Simonova O.I., Zhirkova Ju.V., Rehviashvili M.G. The choice of surgical tactics for complicated meconium ileus in newborns. Pediatric Surgery. 2015. 19(1). 46-49. in Russian.
13. Long A.M., Jones I.H., Knight M., McNally J. Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study. J Pediatr Surg. 2021. Aug. 56(8). 1287-1292. DOI: 10.1016/j.jpedsurg.2021.02.047.
Review
For citations:
Markovskaya A.I., Potapova N.L., Ignatyeva A.V., Mizernitsky Yu.L. Cystic fibrosis complicated with meconial ileus and intestinal inversion. Transbaikalian Medical Bulletin. 2022;(4):151-156. (In Russ.) https://doi.org/10.52485/19986173_2022_4_151
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