Alagille syndrome [ALGS] is an autosomal dominant, complex multisystem disorder that includes a wide range of clinical aspects, most commonly manifest in infancy or early childhood. It is mostly caused by mutations of genes involved in the Notch signaling pathway. The major of clinic manifestations occur in liver, but also other organs, like heart, eye and skeleton can be impaired. Herein we report the story of a family in which four members were diagnosed with ALGS, leading the same gene mutation. The peculiarity of our case lies in the fact that they manifested the disease in different time of their life and with many different symptoms, highlighting the impressive clinical variability of the ALGS and the importance of considering it in the differential diagnosis of liver impairment disease.

Until a few years ago, pediatric pulmonology was one of the most static branches of pediatrics. In recent years, the description of new pathological entities affecting the lung and airways, the identification of new disease biomarkers, the use of new respiratory function tests suitable not only for collaborating but also for non-collaborating children, the enhancement of respiratory endoscopy, the advent of new therapies such as biological drugs and genetic modulators have made pediatric pulmonology one of the most dynamic branches of pediatric medicine.The objective of this article will be to explore the new fields of pediatric pulmonology by making a parallelism with the evolutions that our center had to follow in order to adapt to the new healthcare and research standards. These same standards of care are needed by patients, their families, and need to be known by primary care pediatricians. In addition, health policy must take into account these upgrades in pediatric pulmonology to provide the best quality of care to all patients and uniformly throughout the country. CF is the most common life-shortening autosomal recessive hereditary disease in Caucasians with a prevalence of 1 case per 2500 live births. CF is a multisystem disease caused by mutations in the cystic fibrosis trans-membrane conductance regulator (CFTR) gene (11, 12). The lung is the primary site of CF, to which most of the CF-associated patient morbidity and mortality is linked. CF occurs very early in infancy and is defined by the presence of infections and chronic inflammation leading to a deterioration in lung function, respiratory exacerbations, and bronchiectasis (13,14). Thus, identifying a biomarker for disease progression might be crucial for improving these patients' outcomes. Moreover, with therapeutic introduction of potentiators and correctors of the CFTR protein, the study of biomarkers could be a valuable tool for monitoring their effectiveness over time (15). Globally, therefore, the study of biomarkers could have a strategic role in CF management and also in asthma. FRACTIONAL-EXHALED NITRIC OXIDE (FeNO)asthma treatment. Finally, CaNO is the least used in clinical practice because lack of standardization of measurement techniques. VOLATILE ORGANIC COMPOUNDS (VOCs) AND EXHALED BREATH CONDENSATE (EBC)