Cystic fibrosis with pulmonary manifestations

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

Name E4_CYSFIBRO_PUL
Long name Cystic fibrosis with pulmonary manifestations
Hospital Discharge registry ICD-10: E840
Cause of Death registry ICD-10: E840
Level in the ICD-hierarchy 4
First defined in version DF2
Latin name Fibrosis cystica cum manifestationibus pulmonalibus

Summary Statistics

Key figures

All Female Male
Number of individuals 20 9 11
Unadjusted prevalence (%) 0.01 0.01 0.01
Mean age at first event (years) 21.85 21.42 22.20
Case fatality at 5-years (%) 5.00 11.11 0.00

Longitudinal metrics

All Female Male
Median nb. of events per indiv. 28.5 28.0 29.0
Recurrence at 6 months (%) 75.00 66.67 81.82

Survival analyses between endpoints

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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