Atlas One · Host Biology · Scale 07 — System

Cystic Fibrosis

Cystic fibrosis is caused by biallelic CFTR mutations; defective chloride and bicarbonate transport → viscous mucus → progressive bronchiectasis, chronic Pseudomonas infection, and exocrine pancreatic insufficiency; median survival >40 years with CFTR modulator therapy.

Also known as: cystic fibrosis, CF, CFTR disease, CF lung disease, CF bronchiectasis, CF pancreatic insufficiency, CF-related diabetes, CFTR mutation disease, mucoviscidosis, cystic fibrosis Trikafta

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
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cystic-fibrosis

Cystic Fibrosis

Cystic fibrosis is caused by biallelic CFTR mutations; defective chloride and bicarbonate transport → viscous mucus → progressive bronchiectasis, chronic Pseudomonas infection, and exocrine pancreatic insufficiency; median survival >40 years with CFTR modulator therapy.

Entry Metadata

FieldValue
IDcystic-fibrosis
NameCystic Fibrosis
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toCftr connects-toPrss1 connects-toTgf Beta connects-toNlrp3 Inflammasome connects-toNeutrophil connects-toIl 1B connects-toHereditary Pancreatitis connects-toChloride connects-toLung connects-toStaphylococcus Aureus connects-toPancreas connects-toCopd connects-toType 2 Diabetes

Sources