Abstract
Background & aimsAbdominal 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.MethodsA 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.ResultsAS 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.ConclusionAs 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.
Highlights
Cystic fibrosis (CF) is the most common life threatening autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene
Increased Abdominal symptoms (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
First results obtained with the JenAbdomen-CF Score 1.0, a new CF-specific patient reported outcome measure
Summary
Cystic fibrosis (CF) is the most common life threatening autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane (conductance) regulator (CFTR) gene. CFTR dysfunction results in viscous luminal secretions obstructing the bile and pancreatic ducts as well as the intestine [3]. The resulting gastrointestinal (GI) manifestations include pancreatic insufficiency (PI), meconium ileus (MI), distal intestinal obstruction syndrome (DIOS), and biliary tract complications which can lead to cirrhosis and hepatic failure [4]. Even though GI symptoms are a hallmark of CF [5,6,7], often leading to diagnosis of the inherited disease, they are still insufficiently understood for why deeper investigations into the abdominal involvement in CF are needed [2]. 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
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