Abstract
Cystic fibrosis (CF) is associated with different gastrointestinal motility disturbances and syndromes. We aim to assess gastric emptying in patients with CF compared to healthy controls by a systematic review of existing literature. Medical databases and abstracts from major gastroenterology and CF meetings were reviewed. Emptying times in CF patients were compared with healthy controls using random effects models. Subgroup analysis stratified results by age and diagnostic modality. Nineteen studies from 7 countries included 574 subjects (359 CF patients and 215 controls). Using pooled analysis frequency of gastroparesis was high (38%, 95% CI 30–45%) but results were highly dependent on the diagnostic modality. Delayed gastric emptying is more common in CF compared to general population. Scintigraphy identified rapid gastric emptying in a subgroup of CF patients, but this finding disappeared with adequate pancreatic enzyme replacement and after other diagnostic modalities were included.
Highlights
Cystic fibrosis (CF) is associated with gastrointestinal dysmotility such as gastroparesis (GP), gastroesophageal reflux, meconium ileus, distal intestinal obstruction syndrome, and chronic constipation [1]
This systematic review shows that patients with CF have a high frequency of GP, up to 38% according to our estimates
Gastroparesis is a diverse syndrome that varies by gender, body mass, symptoms, and severity of gastric emptying delay [38]
Summary
Cystic fibrosis (CF) is associated with gastrointestinal dysmotility such as gastroparesis (GP), gastroesophageal reflux, meconium ileus, distal intestinal obstruction syndrome, and chronic constipation [1]. About 90% of patients with CF have exocrine pancreatic insufficiency and require regular PERT to improve the digestion of dietary fat, protein, and other nutrients [6]. Differences in response to PERT may be related to gastric emptying rates, as digestion of fat in patients with CF and pancreatic insufficiency is strongly affected by how rapidly fat enters the duodenum [9]. Macrolides (e.g., azithromycin) are used as a chronic anti-inflammatory therapy in the patients who suffer from Pseudomonas infection in their lungs. This therapy has prokinetic effects that may improve GP or cause GI distress due to rapid gastric emptying [10, 11]
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