Cystic fibrosis, other manifestations/unspecified

E4_CYSTFIBRO_NAS

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

429209 individuals

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Apply sex-specific rule None

429209

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Check conditions None

429209

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

Hospital Discharge: ICD-10 E84.8, E84.9
Hospital discharge: ICD-9 2770
Hospital discharge: ICD-8 2730
Cause of death: ICD-10 E84.8, E84.9
Cause of death: ICD-9 2770
Cause of death: ICD-8 2730

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

52

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Check minimum number of events None

52

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Include endpoints None

52

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Remove individuals based on genotype QC

51

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E4_CYSTFIBRO_NAS

Control definitions

Control exclude E4_METABOLIA

Extra metadata

Level in the ICD hierarchy 4
First used in FinnGen datafreeze DF2

Summary Statistics

Key figures

All Female Male
Number of individuals 51 26 25
Unadjusted prevalence (%) 0.01 0.01 0.01
Mean age at first event (years) 21.82 20.67 23.02

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_CYSTFIBRO_NAS – Cystic fibrosis, other manifestations/unspecified
GWS hits:

Survival analyses between endpoints

Plot

before Cystic fibrosis, other manifestations/unspecified
after Cystic fibrosis, other manifestations/unspecified

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Drugs most likely to be purchased after Cystic fibrosis, other manifestations/unspecified

Endpoint not on priority list, no data to show.