Pulmonary alveolar microlithiasis and lymphocytic interstitial pneumonitis in a ten year old girl.

Pulmonary alveolar microlithiasis (PAM) is a rare lung disease, characterized by progressive formation of intra-alveolar calculi in response to an unknown stimulus. We report an unusual presentation of PAM in a 10 year old girl with clinically significant interstitial lung disease and histological evidence of both PAM and lymphocytic interstitial pneumonitis. A rapid improvement of pulmonary function and exercise tolerance was seen in response to glucosteroid therapy.

Variability of dynamic compliance measurements in spontaneously breathing and ventilated newborn infants.

We studied reproducibility and variability of dynamic pulmonary compliance (Cdyn) by making measurements with the esophageal balloon at multiple locations within the esophagus, in both spontaneously breathing and mechanically ventilated newborn infants. Reliable measurements could be obtained over a range similar to that reported for measurements with a liquid-filled catheter. In spontaneously breathing infants Cdyn was found to be highly variable. This variability was unrelated to catheter position but was associated with concomitant changes in pulmonary resistance. Probably because of the high variability, the correlation of Cdyn with a measurement of respiratory system compliance (Crs) was rather poor (r = 0.63). Cdyn measured in mechanically ventilated infants was significantly less variable and compared favorably to Crs (r = 0.86), but its accuracy could not be adequately assessed since the comparison of esophageal and airway occlusion pressure was not feasible in all infants. In addition, significant differences in Cdyn were found between spontaneous and ventilated breaths during mechanical ventilation. Further studies in both ventilated and spontaneously breathing infants are needed to assess the variability of Cdyn over extended time periods.

Transition from dynamically maintained to relaxed end-expiratory volume in human infants.

Newborn infants, in contrast to adults, dynamically maintain end-expiratory lung volume (EEV) above relaxation volume. The purpose of this study was to determine at what age children develop a breathing strategy that is relaxed, i.e., determined by the mechanical characteristics of the lung and chest wall. Forty studies were performed in 27 healthy infants and children aged 1 mo to 8 yr during natural sleep. Volume changes were recorded with the use of respiratory inductance plethysmography (RIP). The volume signal was differentiated to yield flow. Flow-volume representations were generated for a random sample of the recorded breaths to determine the predominant breathing strategy utilized, i.e., relaxed, interrupted, or indeterminate. The respiratory pattern was predominantly interrupted below 6 mo of age and predominantly relaxed over 1 yr of age. Mixed patterns were observed in children 6-12 mo of age. The number of breaths that could not be classified (indeterminate) decreased with age. Respiratory frequency measured from the sample of breaths decreased with age and was accompanied by an increase in expiratory time. We conclude that a relaxed EEV develops at the end of the first year of life and may be related to changes in the mechanical properties of the chest wall associated with growth as well as changes in respiratory timing.

Changes of time constants during infancy and early childhood.

We used respiratory inductance plethysmography to record tidal respiration in 27 healthy unsedated infants and children 1 mo to 8 yr of age during sleep. Rib cage and abdominal outputs were present at approximately equal gains and summed to obtain an estimate of volume. Flow-volume curves were generated from the uncalibrated volume signal and its flow derivative. Expiratory time constants (tau) were obtained by visually drawing a line through the linear portion of the expiratory flow-volume relationship. tau increased significantly during the first 10 mo of life. After 10 mo, the estimated rate of increase of tau for older children was less than 5% of the estimated initial rate and not significantly different from zero. Prolongation of tau was paralleled by an increase in expiratory time (Te), and no changes in Te/tau were observed in the first 2 yr of life. These changes in tau likely reflect the increase in lung compliance induced by rapid alveolar growth during infancy. After the first year, expiratory time constants appear to remain relatively constant and may be consistent with balanced changes in compliance and resistance beyond infancy.