What is this pathology?
A 22-year-old female patient presents in the urgent care with chronic coughing, wheezing, and shortness of breath. Patient complains that her coughs expel sputum and notices that she gets lung infections frequently.
The urgent care doctor orders both a chest x-ray and a chest CT. You are the radiologic technologist, so you will be taking the patient's x-rays! Afterwards, take her to the CT department to get her chest CT done.
This is what your image looks like
DI: +4......What can you do to make this image look better? Maybe bring that technique down?
Info
That looks better!
DI: +0.3, Make sure you remember what technique you used to get this image! That will be important to know.
Info
Let's compare a normal chest x-ray to the one you took, Hover over the buttons to see the differences
When giving a presentation, there are
chest ct
You took the patient to the CT department where the patient got her chest CT done. Here's a CT axial slice of the patient's chest. What do you notice in the lungs? Hover over button to show more information.
Hover over button below before answering question
What is cystic fibrosis?
This pathology is a hereditary disease that causes thickening of mucus by the exocrine glands of the body. This can cause airway blockages, malabsorption of foods, and increased chances of getting infections due to the thick mucus trapping germs.
What is the etiology of cystic fibrosis? This pathology is caused by a gene mutation in the CFTR (Cystic fibrosis transmembrane conductance regulator) gene. The CFTR gene holds the instructions to making the CFTR protein. Every person has 2 copies of the CFTR gene, one from the biological mother and one from the biological father. If only one of the parents has a CFTR gene mutation (called a CF carrier) and the other parent doesn't, the child will not get cystic fibrosis. It is only when both parents have a gene mutation in their CFTR gene and the child recieves both of the mutated CFTR genes will the child get cystic fibrosis. Note: The parent can simultaneously have a mutated CFTR gene AND not have cystic fibrosis.
What is a hereditary disease?Hereditary diseases result from an abnormality in the DNA, passing from one generation down to the next generation genetic information contained in the nucleus of each cell.
What does the CFTR proteins do? It essentially controls the movement of water in between tissues which also helps create thin mucus.
Poor growth or weight gain in childhood
Fertility issues mainly in men
Nasal polyps in nose
What are the signs and symptoms of cystic fibrosis?
Thick and viscous mucus
Frequent lung and sinus infections
Click me
Coughs and wheezing
salty-tasting skin
Cystic fibrosis not only affects the lungs. It affects many other organs in the body such as the...- sinuses- skin - liver- pancreas - intestines - reproductive organs
Diagnosis and treatment
Treatment - Dietary changes - Physiotherapy and airway clearance techniques - CFTR modulators - Anti inflammatory therapy - antibiotics - lung transplantation- Oral and inhaled corticosteroids
DiagnosisCarrier screening Genetic testing done before or during pregnancy to see the chances of newborn having a genetic disorder. Newborn screening A blood sample is taken from the newborn and sent to a lab to test for any disorders. Medical Imaging such as x-rays and CTs can be used to diagnose cystic fibrosis.
With early and dedicated treatment, median predicted life expectancy has improved to over 48 years
McBennett K, Davis P, Konstan M.
Cystic fibrosis is a hereditary disease affecting the CFTR gene causing thicker and viscous mucus in the body. This can create complications such as chronic coughing, wheezing, lung and/or sinus infections, developmental issues, fertility issues, salty-tasting skin, and nasal polyps. This pathology can affect many organs in the body such as the pancreas, liver, and intestines. It primarily affects the lungs due to blockages in the airways. Medical imaging such as X-ray and CT can use that to diagnose cystic fibrosis because of the dilation in the bronchi. This dilation does mean that there is more air in the lungs and technique should be adjusted to a lower technique. One thing to know is that cystic fibrosis can look similar to COPD and should be diagnosed with further testing. Carrier and newborn screening are two of ways to diagnose cystic fibrosis early. Some treatment options to alleviate this pathology would be dietary changes, physiotherapy and airway clearance techniques, CFTR modulators, anti inflammatory therapy- antibiotics, lung transplantation, oral and inhaled corticosteroids. The prognosis on this pathology highly depends on whether treatment is begun in early-life and is maintained throughout. Life expectancy used to be in the 20s, however current studies show that median life span is over 40 years old as long as there is continuous treatment and proper managing of health.
Summary
Citations
- Adult cystic fibrosis > fact sheets > yale medicine. (n.d.-a). https://www.yalemedicine.org/conditions/adult-cystic-fibrosis - Carrier screening. ACOG. (n.d.). https://www.acog.org/womens-health/faqs/carrier-screening - Centers for Disease Control and Prevention. (n.d.). About newborn screening. Centers for Disease Control and Prevention. https://www.cdc.gov/newborn-screening/about/index.html - Cystic fibrosis | radiology reference article | radiopaedia.org. (n.d.-b). https://radiopaedia.org/articles/cystic-fibrosis?lang=us - Cystic fibrosis. Radiology at St. Vincent’s University Hospital. (n.d.). http://www.svuhradiology.ie/case-study/cystic-fibrosis/ - Increasing life expectancy in cystic fibrosis: Advances and challenges - mcbennett - 2022 - pediatric pulmonology - wiley online library. (n.d.-c). https://onlinelibrary.wiley.com/doi/10.1002/ppul.25733 - U.S. Department of Health and Human Services. (n.d.). Symptoms. National Heart Lung and Blood Institute. https://www.nhlbi.nih.gov/health/cystic-fibrosis/symptoms - U.S. National Library of Medicine. (n.d.). CFTR gene: Medlineplus Genetics. MedlinePlus. https://medlineplus.gov/genetics/gene/cftr/#conditions
end of presentation!
Generally, patients with this pathology who are in an advanced condition tend to get abnormally dilated bronchi in their lungs due to thick mucus build-up. Dilated bronchi means more air in the lungs. In effect, there is less part thickness to go through, requiring less technique.
Tip
Lower your technique for patients with this pathology, especially if they are in a more advanced condition
What is this pathology?
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Transcript
What is this pathology?
A 22-year-old female patient presents in the urgent care with chronic coughing, wheezing, and shortness of breath. Patient complains that her coughs expel sputum and notices that she gets lung infections frequently.
The urgent care doctor orders both a chest x-ray and a chest CT. You are the radiologic technologist, so you will be taking the patient's x-rays! Afterwards, take her to the CT department to get her chest CT done.
This is what your image looks like
DI: +4......What can you do to make this image look better? Maybe bring that technique down?
Info
That looks better!
DI: +0.3, Make sure you remember what technique you used to get this image! That will be important to know.
Info
Let's compare a normal chest x-ray to the one you took, Hover over the buttons to see the differences
When giving a presentation, there are
chest ct
You took the patient to the CT department where the patient got her chest CT done. Here's a CT axial slice of the patient's chest. What do you notice in the lungs? Hover over button to show more information.
Hover over button below before answering question
What is cystic fibrosis?
This pathology is a hereditary disease that causes thickening of mucus by the exocrine glands of the body. This can cause airway blockages, malabsorption of foods, and increased chances of getting infections due to the thick mucus trapping germs.
What is the etiology of cystic fibrosis? This pathology is caused by a gene mutation in the CFTR (Cystic fibrosis transmembrane conductance regulator) gene. The CFTR gene holds the instructions to making the CFTR protein. Every person has 2 copies of the CFTR gene, one from the biological mother and one from the biological father. If only one of the parents has a CFTR gene mutation (called a CF carrier) and the other parent doesn't, the child will not get cystic fibrosis. It is only when both parents have a gene mutation in their CFTR gene and the child recieves both of the mutated CFTR genes will the child get cystic fibrosis. Note: The parent can simultaneously have a mutated CFTR gene AND not have cystic fibrosis.
What is a hereditary disease?Hereditary diseases result from an abnormality in the DNA, passing from one generation down to the next generation genetic information contained in the nucleus of each cell.
What does the CFTR proteins do? It essentially controls the movement of water in between tissues which also helps create thin mucus.
Poor growth or weight gain in childhood
Fertility issues mainly in men
Nasal polyps in nose
What are the signs and symptoms of cystic fibrosis?
Thick and viscous mucus
Frequent lung and sinus infections
Click me
Coughs and wheezing
salty-tasting skin
Cystic fibrosis not only affects the lungs. It affects many other organs in the body such as the...- sinuses- skin - liver- pancreas - intestines - reproductive organs
Diagnosis and treatment
Treatment - Dietary changes - Physiotherapy and airway clearance techniques - CFTR modulators - Anti inflammatory therapy - antibiotics - lung transplantation- Oral and inhaled corticosteroids
DiagnosisCarrier screening Genetic testing done before or during pregnancy to see the chances of newborn having a genetic disorder. Newborn screening A blood sample is taken from the newborn and sent to a lab to test for any disorders. Medical Imaging such as x-rays and CTs can be used to diagnose cystic fibrosis.
With early and dedicated treatment, median predicted life expectancy has improved to over 48 years
McBennett K, Davis P, Konstan M.
Cystic fibrosis is a hereditary disease affecting the CFTR gene causing thicker and viscous mucus in the body. This can create complications such as chronic coughing, wheezing, lung and/or sinus infections, developmental issues, fertility issues, salty-tasting skin, and nasal polyps. This pathology can affect many organs in the body such as the pancreas, liver, and intestines. It primarily affects the lungs due to blockages in the airways. Medical imaging such as X-ray and CT can use that to diagnose cystic fibrosis because of the dilation in the bronchi. This dilation does mean that there is more air in the lungs and technique should be adjusted to a lower technique. One thing to know is that cystic fibrosis can look similar to COPD and should be diagnosed with further testing. Carrier and newborn screening are two of ways to diagnose cystic fibrosis early. Some treatment options to alleviate this pathology would be dietary changes, physiotherapy and airway clearance techniques, CFTR modulators, anti inflammatory therapy- antibiotics, lung transplantation, oral and inhaled corticosteroids. The prognosis on this pathology highly depends on whether treatment is begun in early-life and is maintained throughout. Life expectancy used to be in the 20s, however current studies show that median life span is over 40 years old as long as there is continuous treatment and proper managing of health.
Summary
Citations
- Adult cystic fibrosis > fact sheets > yale medicine. (n.d.-a). https://www.yalemedicine.org/conditions/adult-cystic-fibrosis - Carrier screening. ACOG. (n.d.). https://www.acog.org/womens-health/faqs/carrier-screening - Centers for Disease Control and Prevention. (n.d.). About newborn screening. Centers for Disease Control and Prevention. https://www.cdc.gov/newborn-screening/about/index.html - Cystic fibrosis | radiology reference article | radiopaedia.org. (n.d.-b). https://radiopaedia.org/articles/cystic-fibrosis?lang=us - Cystic fibrosis. Radiology at St. Vincent’s University Hospital. (n.d.). http://www.svuhradiology.ie/case-study/cystic-fibrosis/ - Increasing life expectancy in cystic fibrosis: Advances and challenges - mcbennett - 2022 - pediatric pulmonology - wiley online library. (n.d.-c). https://onlinelibrary.wiley.com/doi/10.1002/ppul.25733 - U.S. Department of Health and Human Services. (n.d.). Symptoms. National Heart Lung and Blood Institute. https://www.nhlbi.nih.gov/health/cystic-fibrosis/symptoms - U.S. National Library of Medicine. (n.d.). CFTR gene: Medlineplus Genetics. MedlinePlus. https://medlineplus.gov/genetics/gene/cftr/#conditions
end of presentation!
Generally, patients with this pathology who are in an advanced condition tend to get abnormally dilated bronchi in their lungs due to thick mucus build-up. Dilated bronchi means more air in the lungs. In effect, there is less part thickness to go through, requiring less technique.
Tip
Lower your technique for patients with this pathology, especially if they are in a more advanced condition