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Cystic Fibrosis Case Study Answer Key

WORKSHEET 2 Names:___Cristy Marshall__________________________________________Date:__09/01/2015______ For the following worksheet, Turn in through Blackboard by end of Week 2. This worksheet is related to cell pathology and diseases. It is worth 5 pts. Please answer the questions in RED A Case of Cystic Fibrosis Dr. Weyland examined a six month old infant that had been admitted to University Hospital earlier in the day. The baby's parents had brought young Zoey to the emergency room because she had been suffering from a chronic cough. In addition, they said that Zoey sometimes would "wheeze" a lot more than they thought was normal for a child with a cold. Upon arriving at the emergency room, the attending pediatrician noted that salt crystals were present on Zoey's skin and called Dr. Weyland, a pediatric pulmonologist. Dr. Weyland suspects that baby Zoey may be suffering from cystic fibrosis. CF affects more than 30,000 kids and young adults in the United States. It disrupts the normal function of epithelial cells — cells that make up the sweat glands in the skin and that also line passageways inside the lungs, pancreas, and digestive and reproductive systems. The inherited CF gene directs the body's epithelial cells to produce a defective form of a protein called CFTR (or cystic fibrosis transmembrane conductance regulator) found in cells that line the lungs, digestive tract, sweat glands, and genitourinary system. When the CFTR protein is defective, epithelial cells can't regulate the way that chloride ions pass across cell membranes. This disrupts the balance of salt and water n ee d e d t o maintain a normal thin coating of mucus inside the lungs and other passageways. The mucus becomes thick, sticky, and hard to move, and can result in infections from bacterial colonization. 1. "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon will die" This is an old saying from the eighteenth century and describes one of the symptoms of CF (salty skin). Why do you think babies in the modern age have a better chance of survival than babies in the 18th century? Drug development. Lung transplants. Research. 2. What symptoms lead Dr. Weyland to his initial diagnosis? Chronic cough, wheezing, salt crystals on skin. 3. Consider the graph of infections, which organism stays relatively constant in numbers over a lifetime. What organism is most likely affecting baby Zoey? Burkholderia capacia. Staphylococcus aureus. 4. What do you think is the most dangerous time period for a patient with CF? Justify your answer. 17-18. That is when two of the respiratory organisms are the highest at the same ±me. Dr. Elizabeth Granier, Bio 151, Worksheet 2, Week 2, Cell 1

Click on the following link, go through the gene therapy case study sections, and answer the questions below.http://learn.genetics.utah.edu/content/genetherapy/casestudy/Cystic FibrosisSection 1:1. What are the symptoms of cystic fibrosis?Coughing, poor digestion, and increased vulnerability to infection. 2. What is the (specific) cause of the symptoms?Abnormal mucus secretions due to a mutation of chromosome 73. What is the CFTR (Cystic Fibrosis Transmembrane Regulator)?membrane protein and chloride channel in vertebrates that is encoded by the CFTR gene.4. The three-base deletion in the CFTR gene sequence results in what?One deletion of the amino acid in the protein that is produced.5. What does the CFTR protein normally do?Functions as a channel to move chloride ions across cell membranes.6. When the CFTR channel protein is mutated, why does it result in the mucus layerdrying out?The mucus reverses direction flowing back into the cell and drying out the mucuslayer.

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