ICD-11 code : CA25;
Preferred Label : Cystic fibrosis;
ICD-11 definition : Cystic fibrosis (CF) is a genetic disorder characterized by the production of sweat
with a high salt content and mucus secretions with an abnormal viscosity. The disease
is chronic and generally progressive, with onset usually occurring during early childhood
or, occasionally, at birth (meconium ileus). Virtually any internal organ may be involved
but the principle manifestations concern the breathing apparatus (chronic bronchitis),
pancreas (pancreatic insufficiency, adolescent diabetes and occasionally pancreatitis)
and, more rarely, the intestine (stercoral obstruction) or liver (cirrhosis). The
usual presenting symptoms and signs include persistent pulmonary infection, pancreatic
insufficiency, and elevated sweat chloride levels. However, many patients demonstrate
mild or atypical symptoms, and clinicians should remain alert to the possibility of
CF even when only a few of the usual features are present. Both criteria; clinical
symptoms consistent with CF in at least one organ system and evidence of cystic fibrosis
transmembrane conductance regulator (CFTR) dysfunction must be met to diagnose cystic
fibrosis.;
ICD-11 synonym : nonproliferative fibrocystic disease; pancreatic cystic fibrosis; mucoviscidosis of pancreas; mucoviscidosis; fibrocystic disease; cystic fibrosis nos; CF - [cystic fibrosis]; fibrocystic disease of the pancreas;
ICD-11 acronym : CF;
ICD-11 inclusion : mucoviscidosis;
ICD-11 "unspecified" category code : CA25.Z;
Origin ID : 514403112;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- False automatic mappings
- ICD-10 Mapping
- See also inter- (CISMeF)
Cystic fibrosis (CF) is a genetic disorder characterized by the production of sweat
with a high salt content and mucus secretions with an abnormal viscosity. The disease
is chronic and generally progressive, with onset usually occurring during early childhood
or, occasionally, at birth (meconium ileus). Virtually any internal organ may be involved
but the principle manifestations concern the breathing apparatus (chronic bronchitis),
pancreas (pancreatic insufficiency, adolescent diabetes and occasionally pancreatitis)
and, more rarely, the intestine (stercoral obstruction) or liver (cirrhosis). The
usual presenting symptoms and signs include persistent pulmonary infection, pancreatic
insufficiency, and elevated sweat chloride levels. However, many patients demonstrate
mild or atypical symptoms, and clinicians should remain alert to the possibility of
CF even when only a few of the usual features are present. Both criteria; clinical
symptoms consistent with CF in at least one organ system and evidence of cystic fibrosis
transmembrane conductance regulator (CFTR) dysfunction must be met to diagnose cystic
fibrosis.
