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

321302 individuals

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Apply sex-specific rule None

321302

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Check conditions None

321302

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Filter registries

Hospital Discharge: ICD-10 E84.0
Hospital discharge: ICD-9 $!$
Hospital discharge: ICD-8 $!$
Cause of death: ICD-10 E84.0
Cause of death: ICD-9 $!$
Cause of death: ICD-8 $!$

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Check pre-conditions, main-only, mode, ICD version

Look only at ICD versions H.D: 10, 8, 9 ; C.O.D: 10, 8, 9

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Check minimum number of events None

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Include endpoints None

33

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E4_CYSFIBRO_PUL

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 32 14 18
Unadjusted prevalence (%) 0.01 0.01 0.01
Mean age at first event (years) 21.65 26.59 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

Plot

before Cystic fibrosis with pulmonary manifestations
after Cystic fibrosis with pulmonary manifestations

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