Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis

Abstract

Cystic fibrosis (CF) is a multiorgan disease, and gastrointestinal (GI) manifestations can contribute to significant morbidity and mortality for individuals with CF. Up to 85% of patients with CF experience GI symptoms, thus addressing the GI aspects of this disease is paramount. With the advent of highly effective CF transmembrane conductance regulator modulators that are increasingly available, many individuals with CF now have significantly improved life expectancy. With these advances, GI manifestations that can be a detriment to quality of life such as gastroesophageal reflux disease, dysbiosis, and chronic abdominal pain have become a priority for patients and caregivers. In addition, as individuals have increased longevity, it has become essential for care providers to be aware of topics such as hepatobiliary disease and colorectal cancer screening. An understanding of the wide scope of GI manifestations in CF can enable providers to optimize the overall health and well-being of their patients. In this review, we aim to provide an up-to-date overview of key aspects of GI and hepatic disease in CF.

Preliminary results of abdominal simultaneous multi-slice accelerated diffusion-weighted imaging with motion-correction in patients with cystic fibrosis and impaired compliance

“…CF patients are able to live well into their sixth decade of life [ 24 , 25 ]. In that context, abdominal complications [ 26 ], including hepatic, pancreatic and gastrointestinal malignancy [ 27 – 30 ], are increasing. Monitoring of abdominal complications of CF [ 31 ] could be improved with native MRI diagnostics, including DWI [ 32 ].…”

Should atelectasis be considered a pulmonary complication and indicator of poor prognosis in cystic fibrosis?

“…Pancreatic insufficiency typically occurs in the "classic, typical, or severe" phenotype. 38 Although this condition was present in 69.1% of our patients, there was no correlation with atelectasis. 4,[21][22][23] A published article indicated that the formation of pulmonary atelectasis was related to acute pancreatitis, although not in patients with CF.…”

Introductory Chapter: Basics of Cystic Fibrosis

“…Clinical symptoms of pancreatic insufficiency include greasy Introductory Chapter: Basics of Cystic Fibrosis DOI: http://dx.doi.org/10.5772/intechopen.97537 stools, flatulence, abdominal bloating, and poor weight gain. The CF liver disease is characterized by the hyper-viscous bile causing obstructive cholangitis initially leading to focal biliary fibrosis and subsequently to biliary multinodular cirrhosis and portal hypertension [17]. Gastrointestinal manifestations of CF occur due to pancreatic insufficiency, thickened intestinal secretions, undigested food remnants, poor motility, and fecal stasis with resultant impaction of mucofeculent material in the distal ileum and right colon presented as meconium ileus in the newborn and distal intestinal obstruction syndrome in the post-neonatal life.…”