Towards a global assessment of pediatric non-cystic fibrosis chronic pulmonary disorders: new insights in disease diagnosis and monitoring

Classification of chronic lung disease (CLD) in children has traditionally distinguished cystic fibrosis (CF), the most common lethal inherited genetic disorder worldwide, from less common non-CF disorders, a heterogeneous group of conditions with different etiologies, but overlapping clinical features. While in CF the multi-organ involvement has traditionally required a systemic approach to patients' care, not limited to respiratory issues, in non-CF CLD this aspect has often been neglected. However, an increasing awareness is emerging regarding the need to adequately address issues such as growth, nutrition and psychological aspects, sometimes underestimated by pediatric respiratory physicians. In particular, nutritional status and growth in pediatric non-CF CLD are strongly related to lung impairment and these two elements may deeply influence each other. Furthermore, in non-CF CLD poor attention has been dedicated to the impact of the disease on patients' life, whereas quality of life represents a crucial parameter to assess disease severity, efficacy of treatments and to highlight areas of possible improvement in patients' care. Given these premises the present phD thesis aimed at (1) evaluating currently used and emerging tools in the diagnosis and monitoring of pediatric non-CF CLD and at (2) assessing the role of nutritional status assessment in the management of non-CF CLD children