Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings

Background & aims Abdominal symptoms ( AS) are a hallmark of the multiorgan-disease cystic fibrosis CF). However, the abdominal involvement in CF is insufficiently understood and, compared to the pulmonary manifestation, still receives little scientific attention. Aims were to assess and quantify AS and to relate them to laboratory parameters, clinical findings, and medical history. Methods A total of 131 patients with CF of all ages were assessed with a new CF-specific questionnaire JenAbdomen- CF score 1.0) on abdominal pain and non-pain symptoms, disorders of appetite, eating, and bowel movements as well as symptom-related quality of life. Results were metrically dimensioned and related to abdominal manifestations, history of surgery, P. aeruginosa and S. aureus colonization, genotype, liver enzymes, antibiotic therapy, lung function, and nutritional status. Results AS during the preceding 3 months were reported by all of our patients. Most common were lack of appetite 130/131) and loss of taste 119/131) followed by abdominal pain 104/131), flatulence 102/131), and distention 83/131). Significantly increased AS were found in patients with history of rectal prolapse p = 0.013), distal intestinal obstruction syndrome p = 0.013), laparotomy p = 0.022), meconium ileus p = 0.037), pancreas insufficiency p = 0.042), or small bowel resection p = 0.048) as well as in patients who have been intermittently colonized with P. aeruginosa p = 0.006) compared to patients without history of these events. In contrast, no statistically significant associations were found to CF-associated liver disease, chronic pathogen colonization, lung function, CF-related diabetes, and nutritional status. Conclusion As the complex abdominal involvement in CF is still not fully understood, the assessment of the common AS is of major interest. In this regard, symptom questionnaires like the herein presented are meaningful and practical tools facilitating a wider understanding of the abdominal symptoms in CF. Furthermore, they render to evaluate possible abdominal effects of novel modulators of the underlying cystic fibrosis transmembrane (conductance) regulator (CFTR) defect.