The Respiratory System

This chapter addresses upper airway physiology for the pediatric intensivist, focusing on functions that affect ventilation, with an emphasis on laryngeal physiology and control in breathing. Effective control of breathing ensures that the airway is protected, maintains volume homeostasis, and provides ventilation. Upper airway structures are effectors for all of these functions that affect the entire airway. Nasal functions include air conditioning and protective reflexes that can be exaggerated and involve circulatory changes. Oral cavity and pharyngeal patency enable airflow and feeding, but during sleep pharyngeal closure can result in apnea. Coordination of breathing with sucking and nutritive swallowing alters during development, while nonnutritive swallowing at all ages limits aspiration. Laryngeal functions in breathing include protection of the subglottic airway, active maintenance of its absolute volume, and control of tidal flow patterns. These are vital functions for normal lung growth in fetal life and during rapid adaptations to breathing challenges from birth through adulthood. Active central control of breathing focuses on the coordination of laryngeal and diaphragmatic activities, which adapts according to the integration of central and peripheral inputs. For the intensivist, knowledge of upper airway physiology can be applied to improve respiratory support. In a second part the mechanical properties of the respiratory system as a critical component of the chain of events that result in translation of the output of the respiratory rhythm generator to ventilation are described. A comprehensive understanding of respiratory mechanics is essential to the delivery of optimized and individualized mechanical ventilation. The basic elements of respiratory mechanics will be described and developmental changes in the airways, lungs, and chest wall that impact on measurement of respiratory mechanics with advancing postnatal age are reviewed. This will be follwowed by two sections, the first on respiratory mechanics in various neonatal pathologies and the second in pediatric pathologies. The latter can be classified in three categories. First, restrictive diseases may be of pulmonary origin, such as chronic interstitial lung diseases or acute lung injury/acute respiratory distress syndrome, which are usually associated with reduced lung compliance. Restrictive diseases may also be due to chest wall abnormalities such as obesity or scoliosis (idiopathic or secondary to neuromuscular diseases), which are associated with a reduction in chest wall compliance. Second, obstructive diseases are represented by asthma and wheezing disorders, cystic fibrosis, long term sequelae of neonatal lung disease and bronchiolitis obliterans following hematopoietic stem cell transplantation. Obstructive diseases are defined by a reduced FEV1/VC ratio. Third, neuromuscular diseases, mainly represented by DMD and SMA, are associated with a decrease in vital capacity linked to respiratory muscle weakness that is better detected by PImax, PEmax and SNIP measurements.