Serum KL-6 reflects alveolar damage and regeneration of type II pneumocytes, indicating disease activity in various interstitial lung diseases. We conducted a descriptive and observational multiple case-control study to determine the distribution of serum KL-6 levels in pediatric patients with or without respiratory diseases. Subjects were recruited from the patients of a teaching hospital in the suburb of Tokyo. A consecutive series of 401 children (0-16 years old) underwent blood sampling for many clinical reasons. They comprised the following four groups: pneumonia (n = 96), bronchial asthma (n = 101), measles (n = 102), and nonrespiratory diseases (n = 102) as a control group. Standard upper limits of serum KL-6 in a group of children with nonrespiratory disease were 250 U/mL, or half the adult level. No gender or age differences were observed. Elevated serum KL-6 concentrations were observed in severe pneumonia, acute exacerbations of asthma, and measles pneumonia. In the measles group, KL-6 values reflected the presence and severity of complicating pneumonia. We conclude that serum KL-6 levels exceeding 250 U/mL were rarely observed in children without respiratory diseases. In contrast, a substantial proportion of children with common respiratory diseases showed mild to moderate increases in serum KL-6 levels. Elevated serum KL-6 in these children may reflect the presence of alveolar damage, followed by regeneration of type II pneumocytes. However, in order to use serum KL-6 as a marker of interstitial lung diseases in children, a cutoff level should be determined separately.

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