Product information "Lung—Cystic Fibrosis"
Clinical History
A 6-year-old girl was admitted with productive cough, dyspnoea and fever. She rapidly deteriorated and died shortly after admission. She had a history of recurrent pneumonia and meconium ileus. A diagnosis of cystic fibrosis (CF) was made. Her sister had died from the same condition at age 3.
Pathology
The lungs show severe, predominantly bronchial changes. Many bronchi are dilated (bronchiectasis) and filled with thick, yellow pus. The upper lobe shows a honeycomb-like area, while the lower lobe contains multiple abscesses, fibrosis and consolidation. Only minimal normal lung tissue remains. These findings are characteristic of CF.
Further Information
Cystic fibrosis is an inherited disease caused by mutations in the CFTR gene on chromosome 7, leading to defective chloride channels. This results in dehydrated secretions in the lungs, pancreas and other organs, causing obstruction, infection and tissue damage.
In the lungs, mucus becomes thick and sticky, impairing clearance and causing chronic infection and bronchiectasis. Common pathogens include Staphylococcus aureus, Haemophilus influenzae and Pseudomonas. Lung complications are the main cause of death in CF. The average life expectancy is 40–50 years in developed countries.
CF affects about 1 in 3000 live births and follows an autosomal recessive inheritance. It is most common in light-skinned populations, with 1 in 20 people being carriers. Diagnosis is usually made via newborn screening and confirmed with a sweat chloride test (> 60 mmol/L).
A 6-year-old girl was admitted with productive cough, dyspnoea and fever. She rapidly deteriorated and died shortly after admission. She had a history of recurrent pneumonia and meconium ileus. A diagnosis of cystic fibrosis (CF) was made. Her sister had died from the same condition at age 3.
Pathology
The lungs show severe, predominantly bronchial changes. Many bronchi are dilated (bronchiectasis) and filled with thick, yellow pus. The upper lobe shows a honeycomb-like area, while the lower lobe contains multiple abscesses, fibrosis and consolidation. Only minimal normal lung tissue remains. These findings are characteristic of CF.
Further Information
Cystic fibrosis is an inherited disease caused by mutations in the CFTR gene on chromosome 7, leading to defective chloride channels. This results in dehydrated secretions in the lungs, pancreas and other organs, causing obstruction, infection and tissue damage.
In the lungs, mucus becomes thick and sticky, impairing clearance and causing chronic infection and bronchiectasis. Common pathogens include Staphylococcus aureus, Haemophilus influenzae and Pseudomonas. Lung complications are the main cause of death in CF. The average life expectancy is 40–50 years in developed countries.
CF affects about 1 in 3000 live births and follows an autosomal recessive inheritance. It is most common in light-skinned populations, with 1 in 20 people being carriers. Diagnosis is usually made via newborn screening and confirmed with a sweat chloride test (> 60 mmol/L).
Erler-Zimmer
Erler-Zimmer GmbH & Co.KG
Hauptstrasse 27
77886 Lauf
Germany
info@erler-zimmer.de
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