Lung Cystic Fibrosis 3D Model Erler-Zimmer Lung Cystic Fibrosis 3D Printed Model

„Erler-Zimmer GmbH & Co. KG 3D model of cystic fibrosis lungs:

• Model Number: MP2063
• Clinical history:
  A 6-year-old girl was admitted with a productive cough, dyspnea, and fever. She became increasingly hypotensive and died after admission. She had a history of recurrent pneumonia and meconium ileus. The clinical diagnosis was cystic fibrosis (mucoviscoidosis). Her sister died at the age of 3 years from the same disease.
• Pathology:
  The lung parenchyma shows extensive changes, mainly with a bronchial distribution. Many of the bronchi are dilated (bronchiectasis) and have thickened, yellow, purulent material. These changes are most prominent in the upper lobe of the lobe, where a small focus of "Medauskas" change is also seen. Numerous abscesses are visible, especially in the lower and central parts of the lower lobe. The base of the lower lung is heavily affected by fibrosis and consolidation is evident. Very little normal lung tissue remains. These pathological changes are characteristic but not pathognomonic of cystic fibrosis.
• Additional information:
  Cystic fibrosis (CF) is an inherited disorder of chloride ion transport. Mutations in the cystic fibrosis conductance regulator (CFTR) gene on chromosome 7 cause defects in the chloride channel protein, leading to ineffective chloride channels. This results in increased water absorption by exocrine glands and the epithelium of the respiratory, digestive, and reproductive tracts. These dehydrated viscous secretions then block the passage of these organs, causing clinical features including: persistent lung infections, pancreatic insufficiency, cirrhosis of the liver, intestinal obstruction, male infertility, and increased sweat chloride levels. In the respiratory tract, CF patients have decreased chloride secretion and increased water reabsorption. This results in a dehydrated, mucous-laden airway lining that impairs ciliary function, mucus obstructs the airways, bronchiole dilation (bronchiectasis), and secondary infection. Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas are the most common bacteria that cause lower respiratory tract infections in CF patients. Chronic bronchitis and bronchiectasis develop as a result. Lung problems are the leading cause of death in CF patients. The average life expectancy is between 40-50 years in developed countries.

3D models of cystic fibrosis lungs – Erler-Zimmer Anatomy Group

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