Cystic fibrosis
ICD-10 E84 · ICD-11 CA25

Cystic Fibrosis with Downward Trajectory of Weight-for-Age Percentile or Z-Score

A declining weight-for-age percentile or z-score — crossing percentile lines over time — is a clinically significant finding in individuals with cystic fibrosis, including those with CRMS (CF screen positive, inconclusive diagnosis) or CFSPID (CF screen positive, inconclusive diagnosis, phenotype). This pattern warrants structured assessment and timely intervention.

The clinical scenario involves a person with cystic fibrosis (or CRMS/CFSPID) who is showing a downward shift in weight-for-age percentile or z-score — a pattern that signals nutritional risk and requires prompt evaluation by appropriately experienced specialists.

Approach (partial)

Management centres on nutritional screening and targeted intervention, delivered by a dietitian with specific experience in CRMS/CFSPID and CF. The full structured regimen — including the complete scope of assessment, intervention steps, and follow-up — is available via the protocol.

Complete regimen details are behind the link below.

References

DOI: 10.1542/peds.2023-064657

  • The CFF recommends, for people with CRMS/CFSPID with a downward trajectory of weight-for-age percentile or z-score (eg, crossing percentiles), that screening and evaluation be provided by a dietitian with experience in the management of CRMS/CFSPID and CF (Grade B).
  • Children who demonstrate this pattern should undergo nutritional screening and appropriate intervention by dietitians with experience in CRMS/CFSPID and CF.
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