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Abdominal manifestations of cystic fibrosis in children.
Pediatr Radiol. 2006 Mar; 36(3):233-40.PR

Abstract

Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities.

Authors+Show Affiliations

Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, 555 University Ave., Toronto, Ontario M5G 1X8, Canada. gulraiz.chaudry@sickkids.caNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

16391928

Citation

Chaudry, Gulraiz, et al. "Abdominal Manifestations of Cystic Fibrosis in Children." Pediatric Radiology, vol. 36, no. 3, 2006, pp. 233-40.
Chaudry G, Navarro OM, Levine DS, et al. Abdominal manifestations of cystic fibrosis in children. Pediatr Radiol. 2006;36(3):233-40.
Chaudry, G., Navarro, O. M., Levine, D. S., & Oudjhane, K. (2006). Abdominal manifestations of cystic fibrosis in children. Pediatric Radiology, 36(3), 233-40.
Chaudry G, et al. Abdominal Manifestations of Cystic Fibrosis in Children. Pediatr Radiol. 2006;36(3):233-40. PubMed PMID: 16391928.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Abdominal manifestations of cystic fibrosis in children. AU - Chaudry,Gulraiz, AU - Navarro,Oscar M, AU - Levine,Daniel S, AU - Oudjhane,Kamaldine, Y1 - 2006/01/04/ PY - 2005/07/14/received PY - 2005/10/10/accepted PY - 2005/09/29/revised PY - 2006/1/5/pubmed PY - 2006/9/13/medline PY - 2006/1/5/entrez SP - 233 EP - 40 JF - Pediatric radiology JO - Pediatr Radiol VL - 36 IS - 3 N2 - Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities. SN - 0301-0449 UR - https://www.unboundmedicine.com/medline/citation/16391928/Abdominal_manifestations_of_cystic_fibrosis_in_children_ L2 - https://dx.doi.org/10.1007/s00247-005-0049-2 DB - PRIME DP - Unbound Medicine ER -