Intussusception in Adults with Cystic Fibrosis: A Case Series with Review of the Literature
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Edward F. Nash Anne Stephenson Emma J. Helm Terence Ho Chandra M. Thippanna Asad Ali Joanna L. Whitehouse David Honeybourne Elizabeth
- چاپ و سال / کشور: 2011
Description
Intussusception occurs when a proximal segment of bowel (intussusceptum) telescopes into the lumen of the adjacent distal segment (intussuscepiens). It is a relatively common cause of an acute abdomen in the first two years of life, but is uncommon in older children [1]. The diagnosis is rare in adult life, with presentations in adulthood comprising 5% of all intussusceptions and 1% of bowel obstructions [2]. In children it is most commonly idiopathic, in contrast with adults, for most of whom an underlying etiology is found. Cystic fibrosis (CF) is the most common fatal inherited condition in Caucasians [3]. It is caused by abnormal function of the cystic fibrosis transmembrane conductance regulator (CFTR), an epithelial chloride channel. This primary defect causes epithelial secretions to be abnormally dehydrated and viscous, affecting several organs including the lungs, pancreas, intestines, and liver. CF is a well-recognized risk factor for childhood intussusception, with an incidence of 1% reported in the largest case series [4]. The peak age of presentation in this case series was between 9 and 12 years of age, much older than in the non- CF population. Although the clinical presentation and management of intussusception in children with CF is well described, that of adults is less well characterized. We therefore present a case series of CF adults diagnosed with symptomatic intussusception at our centers and perform a systematic review of the literature in order to identify previously described cases. Our aim is to examine the clinical and imaging findings of intussusception in CF adults in order to identify features of the presentation that should suggest this diagnosis
Dig Dis Sci DOI 10.1007/s10620-011-1790-z Received: 22 November 2010 / Accepted: 6 June 2011
