Lung: cystic fibrosis - Erler Zimmer 3D anatomy Series MP2063

This dissection model highlighting a Lung with cystic fibrosis is part of the exclusive Monash 3D anatomy series, a comprehensive series of human dissections reproduced with ultra-high resolution color 3D printing.

Clinical history

A 6-year-old girl was admitted with productive cough, dyspnea and fevers. She became increasingly hypotensive and died soon after admission. She had a previous history of recurrent pneumonia and meconium ileus. The clinical diagnosis was cystic fibrosis (mucoviscoidosis). Her sister died at the age of 3 from the same disease.

Pathology

Lung parenchyma shows extensive changes mainly with a bronchial distribution. Many bronchi are dilated (bronchiectasis) and contain thick, yellowish, purulent material. These changes are most pronounced in the upper lobe, at the apex of which a small focus of "honeycomb" change is also seen. Multiple abscesses are present, especially in the basal and central part of the lower lobe. The base of the lower lobe is severely affected with fibrosis and consolidation evident. There is very little remaining normal lung tissue. These pathologic changes are characteristic but not pathognomonic of cystic fibrosis.

Additional Information.

Cystic fibrosis (CF) is an inherited disease of chloride ion transport. Mutations in the cystic fibrosis conductance regulator (CFTR) gene on chromosome 7 cause defects in the chloride channel protein that lead to dysfunction of chloride channels. This causes increased water absorption in the exocrine glands and epithelium of the respiratory, gastrointestinal, and reproductive tracts. These viscous dehydrated secretions then obstruct the passage of these organs causing clinical features including: persistent lung infection, pancreatic insufficiency, liver cirrhosis, intestinal obstruction, male infertility, and elevated chloride levels in sweat. In the airway, CF patients have reduced chloride secretion and increased water reabsorption. This causes dehydration of the mucosal lining of the airways leading to defective mucociliary action, mucus obstructing the airways, dilation of the bronchioles (bronchiectasis) and secondary infection. Staphylococcus aureus, Haemophilus influenzae and Pseudomonas are the most common bacteria causing lower respiratory tract infections in CF patients. As a result, chronic bronchitis and bronchiectasis develop. Lung problems are the highest cause of mortality in CF patients. The average life expectancy is between 40 and 50 years in developed countries.

CF occurs in about 1 in 3000 live births. It is inherited in an autosomal recessive mode. It is most common in fair-skinned populations: 1 in 20 carries the gene. Symptoms can occur in utero or even into adolescence, depending on the severity of the disease. It is now most commonly diagnosed with the neonatal screening test for immunoreactive trypsinogen (a pancreatic enzyme precursor). If this screening test is positive, a formal diagnosis is made with a sweat test showing >60mmol/L chloride.

What advantages does the Monash University anatomical dissection collection offer over plastic models or plastinated human specimens?

• Each body replica has been carefully created from selected patient X-ray data or human cadaver specimens selected by a highly trained team of anatomists at the Monash University Center for Human Anatomy Education to illustrate a range of clinically important areas of anatomy with a quality and fidelity that cannot be achieved with conventional anatomical models-this is real anatomy, not stylized anatomy.
• Each body replica has been rigorously checked by a team of highly trained anatomists at the Center for Human Anatomy Education, Monash University, to ensure the anatomical accuracy of the final product.
• The body replicas are not real human tissue and therefore not subject to any barriers of transportation, import, or use in educational facilities that do not hold an anatomy license. The Monash 3D Anatomy dissection series avoids these and other ethical issues that are raised when dealing with plastinated human remains.