Part III—Your Explanation of Sam’s Condition
Objectives and Issues
1. What type of disease is Sam suffering from?
2. What tissues and organs does this disease affect? Once you identify all the tissues and organs, briefly explain their respective dysfunction.
Exocrine Glands – glands may become blocked, produce excessive secretions
Sweat Glands – extreme decrease in salt reabsorbtion, resulting in high salt content in sweat.
Lungs – Mucus plugging, chronic bacterial infections, pronounced inflammatory response, damaged airways leading to respiratory insufficiency, progressive decline in pulmonary function.
Male reproductive tract – males with CF are sterile due to degeneration and/or obstruction of the vas deferens.
Pancreas – reduced secretions, damage to acini, blacked ducts due to mucus accumulation, eventual destruction of the pancreas.
Sinuses – thickened mucosa, underdevelopment of frontal sinuses
Intestines – intestinal blockages, abnormally viscous mucus secretions, impaction, lack of secretions
Bile Ducts – thickened mucosa and secretions, blockages
3. Does this disease affect exocrine or endocrine glands; please explain your answer. In addition, what role do goblet cells play in this disease state?
Cystic Fibrosis affects nearly all exocrine glands although the distribution and degree of severity may vary. Glands may become blocked by exceptionally viscid or solid material in the lumens of the pancreas, intestinal glands, intrahepatic bile ducts, gallbladder, and submaxillary glands. The tracheobronchial glands may appear abnormal and produce excessive secretions. The sweat and small salivary glands may appear normal but secrete excessive amounts of Na and Cl.
The Merck Manual pg 2881
Mucus is a substance that coats our epithelial surfaces. It keeps surfaces lubricated and helps prevent disease by trapping dirt and bacteria. Mucus is a mixture of predominantly water
Bibliography: Brand PL, van der Ent CK. The Practical Application and Interpretation of Simple Lung Function Tests in Cystic Fibrosis. J R Soc Med. 1999;92 (Suppl 37):2–12. [PMC free article] [PubMed] Martini, Frederic, Judi L. Nath, and Edwin F. Bartholomew.Fundamentals of Anatomy & Physiology. 9th ed. San Francisco, CA: Benjamin Cummings, 2012. Print. Porter, Robert S, and Justin L. Kaplan. The Merck Manual of Diagnosis and Therapy. 19th Edition. Whitehouse Station, N.J: Merck Sharp & Dohme Corp, 2011. Print. http://www.colorado.edu/outreach/BSI/pdfs/osmosis.pdf http://www.stanford.edu/class/psych121/humangenome-CF.htm#organs http://www.webmd.com/lung/nasal-potential-difference-test-for-cystic-fibrosis