Abstract
To understand the defect that leads to accumulation of proteins in CF meconium we measured immunoreative trypsinogen (IRT) by radioimmunoassay and trypsin catalytic activity (CA) by fluorimetric method. IRT concentration was normal in CF meconium based on total protein content, but was elevated based on wet weight, as was total protein and albumin. Trypsin CA was reduced in CF meconium compared to normal meconium:
Trypsin inhibitory activity, measured by incubating porcine trypsin with meconium was: CF, 33 ± 27; normal, 1 ± 3 μg porcine trypsin inactivated/g meconium. Trypsinogen, an inactive precursor of trypsin, is synthesized by pancreatic acinar cells and secreted into intestinal lumen. The results show this process is normal in infants with CF. Presence of trypsin inhibitor(s) rather than absence of IRT accounts for accumulation of proteins in CF meconium.
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Hsieh, M., Berry, H. MECONIUM FROM INFANTS WITH CYSTIC FIBROSIS (CF) CONTAINS IMMUNOREATIVE TRYPSINOGEN. Pediatr Res 18 (Suppl 4), 200 (1984). https://doi.org/10.1203/00006450-198404001-00640
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DOI: https://doi.org/10.1203/00006450-198404001-00640
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