Cystic fibrosis with intestinal manifestations

Description

obsolete_cystic fibrosis: ['A congenital metabolic disorder affecting the exocrine glands, inherited as an autosomal trait. The secretions of exocrine glands are abnormal, resulting in excessively viscid mucus production which causes obstruction of passageways (including pancreatic and bile ducts, intestines, and bronchi). The sweat sodium and chloride content are increased. Symptoms usually appear in childhood and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather. -- 2003', 'A congenital metabolic disorder affecting the exocrine glands, inherited as an autosomal trait. The secretions of exocrine glands are abnormal, resulting in excessively viscid mucus production which causes obstruction of passageways (including pancreatic and bile ducts, intestines, and bronchi). The sweat sodium and chloride content are increased. Symptoms usually appear in childhood and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather.']

Data source
FinnGen phenocode E4_CYSTFIBRO_INT
Hospital Discharge registry ICD-10: E841
Cause of Death registry ICD-10: E841
KELA reimboursements codes ANY
KELA reimbursements ICD-10: E84
Level in the ICD-hierarchy 4
First defined in version DF2
Ontology
DOID 1485
GWAS catalog 0000390
MESH D003550

Key figures

Sex All Female Male
Number of individuals 13 7 6
Unadjusted prevalence (%) 0.01 0.01 0.01
Mean age at first event (years) 26.66 30.46 22.23
Case fatality at 5-years (%) 0.0 0.0 0.0

Longitudinal metrics

Sex All Female Male
Median nb. of events per indiv. 2 1 4
Recurrence at 6 months (%) 23.08 - 50.0

Associations

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