[Acute pulmonary and non-pulmonary complications in adults with cystic fibrosis]. / Complications aiguës respiratoires et non respiratoires chez l'adulte mucoviscidosique.

INTRODUCTION: Cystic fibrosis (CF) is a genetic disease primarily affecting the lungs, which could lead to chronic respiratory failure and premature death. CF patients are usually followed in specialized centers, but may present outside of these centers when they seek care for acute pulmonary and/or non-pulmonary complications. The aim of this paper is to provide appropriate knowledge necessary for managing respiratory and non-respiratory emergencies in CF adults. METHODS: The review is based on international guidelines, extensive search of the available literature using Pubmed, and experience of the CF reference center at Cochin hospital (Paris, France). Complications occurring after solid organ transplantation (e.g., lung and/or liver) are excluded from this review. RESULTS: Main acute respiratory complications are pulmonary exacerbations, hemoptysis, pneumothorax and allergic bronchopulmonary aspergillosis. Acute non-respiratory complications include hyponatremic dehydration, acute pancreatitis, acute complications of gallstones, distal intestinal obstruction syndrome, symptomatic nephrolithiasis, acute kidney injury, drug intolerances and catheter-related acute complications. CONCLUSION: This review summarizes acute pulmonary and non-pulmonary complications occurring in adults with CF, focusing on diagnosis and principles of treatment, with the aim of providing a reference that can be used in clinical practice.

[Ageing with cystic fibrosis: Classical and emerging comorbidities in adults with cystic fibrosis]. / Vieillir avec la mucoviscidose : comorbidités classiques et émergentes chez l'adulte mucoviscidosique.

Cystic fibrosis (CF) is a genetic disease with pulmonary involvement being predominant and often leading to respiratory failure and premature death. Non-pulmonary complications related to CF transmembrane conductance regulator (CFTR) defect are numerous and account for significant morbidity (exocrine pancreatic insufficiency, diabetes, CF-related liver disease, chronic sinusitis, osteoporosis). Improvement in patients' care led to a continuous increase in life expectancy, with a subsequent increase in the number of adult CF patients worldwide. Increased life expectancy comes with increased prevalence of CF-related comorbidities, but also with new emerging complications directly related to ageing (chronic kidney disease, cardiovascular risk factors, cancers). CFTR modulators might also contribute to modify the face of CF epidemiology and prognosis. Ageing with CF has become a challenge for CF patients and caregivers. This review summarizes classic and emerging comorbidities in the context of current growth and ageing of the CF population. It also addresses potential roles of CFTR modulators.