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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.

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Ultrasound (US) is an ideal diagnostic tool for paediatric patients owning to its high spatial and temporal resolution, real-time imaging, and lack of ionizing radiation and bedside availability. The lack of superficial adipose tissue and favorable acoustic windows in children makes US the first line of investigation for the evaluation of pleural and chest wall abnormali-ties. Lung parenchyma was previously thought to be inaccessible to ultrasound due to the presence of the air and bony thorax. The change in attitude and growing awareness of the diagnostic possibilities has led to lung ultrasound (LUS) being accepted as a valuable point of care method. In addition, the application of LUS has widened with improvements in technology such as higher resolution transducers, harmonic imaging and contrast-enhanced ultrasound. In the current World Federation of Societies for Ultrasound in Medicine and Biology (WFUMB) paper series the topic will be introduced, the technical requirements explained and the use of ultrasound in the lung and pleura in pediatric patients are discussed.