Further studies on lymphocyte beta-glucuronidase abnormality in cystic fibrosis. Possible relations to carboxypeptidase B-like activity.

The presence in the serum of both cystic fibrosis (CF) homozygotes and heterozygotes of a factor inhibiting the response of lymphocyte lysosome beta-glucuronidase activity to in vitro phytohaemagglutinin (PHA) stimulation is confirmed. Studying lymphocyte beta-glucuronidase activity on PHA stimulation represents a way to confirm CF diagnosis and to screen CF heterozygotes. For technical complexity, however, the method cannot be used for mass screening, but it can be useful for confirming the diagnosis in suspected cases. Relationships between serum factor inhibiting the effect of PHA on beta-glucuronidase, ciliary dyskinesia factor and carboxypeptidase B-like activity are discussed.