Cystic fibrosis with pulmonary manifestations

E4_CYSFIBRO_PUL

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.']

Endpoint definition

FinnGen phenotype data

392423 individuals

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Apply sex-specific rule None

392423

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Check conditions None

392423

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Check pre-conditions, main-only, mode, registry filters

Hospital Discharge: ICD-10 E84.0
Cause of death: ICD-10 E84.0

2 out of 7 registries used, show all original rules.

38

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Check minimum number of events None

38

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Include endpoints None

38

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Remove individuals based on genotype QC

37

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E4_CYSFIBRO_PUL

Control definitions

Control exclude E4_METABOLIA

Extra metadata

Level in the ICD hierarchy 4
First used in FinnGen datafreeze DF2
Parent code in ICD-10 E84
Name in latin Fibrosis cystica cum manifestationibus pulmonalibus

Summary Statistics

Key figures

All Female Male
Number of individuals 37 19 18
Unadjusted prevalence (%) 0.01 0.01 0.01
Mean age at first event (years) 19.78 21.64 17.80

Mortality

Follow-up Absolute risk HR [95% CI] p N
1998–2019 - - - -
15 years - - - -
5 years - - - -
1 year - - - -

Age distribution of first events

Year distribution of first events

Cumulative Incidence

Correlations

Index endpoint: E4_CYSFIBRO_PUL – Cystic fibrosis with pulmonary manifestations
GWS hits:

Survival analyses between endpoints

Not a core endpoint, no data to show.

Drugs most likely to be purchased after Cystic fibrosis with pulmonary manifestations

Endpoint not on priority list, no data to show.