- published: 15 Aug 2022
- views: 158394
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Cystic fibrosis
Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in males among others. Different people may have different degrees of symptoms.
CF is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Those with a single working copy are carriers and otherwise mostly normal. CFTR is involved in production of sweat, digestive fluids, and mucus. When CFTR is not functional, secretions which are usually thin instead become thick. The condition is diagnosed by a sweat test and genetic testing. Screening of infants at birth takes place in some areas of the world.
This page contains text from Wikipedia, the Free Encyclopedia - https://wn.com/Cystic_fibrosis
Podcasts:
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Cystic Fibrosis: Pathophysiology, Genetics, Symptoms, Diagnosis and Treatments, Animation
Cystic fibrosis, CF, is the most common inherited disorder that leads to shortened life expectancy, especially among people of Northern European origin. CF is caused by mutations in the CF transmembrane conductance regulator - CFTR gene. Purchase a license to download a non-watermarked version of this video on AlilaMedicalMedia(dot)com Check out our new Alila Academy - AlilaAcademy(dot)com - complete video courses with quizzes, PDFs, and downloadable images. ©Alila Medical Media. All rights reserved. Voice by : Marty Henne All images/videos by Alila Medical Media are for information purposes ONLY and are NOT intended to replace professional medical advice, diagnosis or treatment. The CFTR protein encoded by CFTR gene functions as a chloride channel, allowing passage of chloride ion...
published: 15 Aug 2022 -
Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
What is cystic fibrosis? Can it be cured? How is it diagnosed? Manuela Cernadas, M.D., Pulmonologist at the Lung Center at Brigham and Women's Hospital and Assistant Professor of Medicine at Harvard Medical School, answers people's most commonly searched questions about cystic fibrosis (CF). Subscribe Link: https://www.youtube.com/channel/UCYrLjATd88gPwIKntCoR0WQ?sub_confirmation=1 0:00 - Intro 0:27 - What is cystic fibrosis? 1:03 - Can cystic fibrosis be cured? 1:47 - How is cystic fibrosis diagnosed? 2:46 - Can I carry cystic fibrosis without realizing it? 3:26 - What are the effects of cystic fibrosis? 4:07 - What is the treatment for cystic fibrosis? 5:29 - How do therapy vests work? 5:54 - Can I have children with cystic fibrosis? 6:24 - Is lung transplantation a possibility? Abou...
published: 28 Mar 2022 -
How Cystic Fibrosis Affects the Lungs
See how the lungs work normally and how cystic fibrosis can affect the lungs over time. https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/Basics-of-Lung-Care/
published: 19 May 2017 -
Cystic Fibrosis Nursing | Cystic Fibrosis Symptoms, Causes, Treatment, NCLEX Review
Cystic fibrosis NCLEX nursing review: Cystic fibrosis is a genetic disorder that causes the exocrine glands to work incorrectly. This can lead to major complications that affect the respiratory, digestive (pancreas, liver, intestines), integumentary, and reproductive systems along with the sinuses. What are exocrine glands? These are glands that produce and transfer it’s secretions (ex: mucous, tears, sweat, digestive enzymes) via DUCTS to it’s intended area of the body rather than directly into the bloodstream like the endocrine glands. In cystic fibrosis, there is a gene that is mutated that prevents the exocrine gland from working properly. It is called the CFTR gene! The CFTR gene, which stands for cystic fibrosis transmembrane regulator, is a protein that controls the channels...
published: 03 Jul 2018 -
What is cystic fibrosis, exactly?
Cystic fibrosis is a genetic condition mostly affecting the lungs and digestive system. You are born with cystic fibrosis and cannot catch it later in life, but one in 25 of us carries the faulty gene that causes it, usually without even knowing. Presented by Charles Michael Duke. Join the fight for a Life Unlimited by cystic fibrosis. Visit https://www.cysticfibrosis.org.uk
published: 19 Jun 2017 -
CYSTIC FIBROSIS, Causes, Signs and Symptoms, Diagnosis and Treatment.
. Chapters 0:00 Introduction 2:28 Causes of Cystic fibrosis 3:14 Symptoms of Cystic fibrosis 3:58 Diagnosis of Cystic fibrosis 5:27 Treatment of Cystic fibrosis Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine.Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males.Different people may have different degrees of symptoms. CF is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein.Th...
published: 11 Apr 2019 -
Living with Cystic Fibrosis
An adult with CF talks about how she has managed her disease growing up and has accomplished many milestones.
published: 20 Mar 2012 -
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America Subscribe to WVTM on YouTube now for more: https://bit.ly/2jvAaUD Get more Birmingham news: http://www.wvtm13.com Like us: https://www.facebook.com/WVTM13/ Follow us: https://twitter.com/WVTM13 Instagram: https://www.instagram.com/wvtm13/
published: 01 Jan 2025 -
Cystic Fibrosis - Definition, Pathogenesis, Symptoms and Complications - Genetics
Cystic fibrosis is an autosomal recessive genetic disease characterized by defective CFTR protein (defect in chloride channels in the sweat glands, pancreatic ducts, bronchi, bronchioles,…etc.)…Genetics lectures, Respiratory pathology. 😍🖼Animated Mnemonics (Picmonic): https://www.picmonic.com/viphookup/medicosis/ - With Picmonic, get your life back by studying less and remembering more. Medical and Nursing students say that Picmonic is the most comprehensive and effective way to bridge learning and test prep... Disclaimer: I use affiliate links.... ►👨🏫💊Antibiotics Lectures: https://www.medicosisperfectionalis.com/products/courses/antibiotics/ ... Check out my brand new "Electrolytes" course at https://www.medicosisperfectionalis.com/products/course/electrolytes/ and use the PROMO ...
published: 16 Sep 2019 -
Cystic Fibrosis Mechanism and Treatment | HHMI BioInteractive Video
Mutations in the CFTR gene, which encodes a transmembrane ion channel, cause mucus buildup in the airways of patients with cystic fibrosis. This animation can be used to illustrate the connection between genotype and phenotype using a human disease example. It also demonstrates how knowledge about the genetic mutations responsible for a particular disease can be used to develop a treatment. Cystic fibrosis is an autosomal recessive disease caused by any one of more than 2,000 mutations in a gene that codes for the cystic fibrosis transmembrane conductance regulator (CFTR). This protein functions as a channel that transports chloride ions across the membranes of cells that line airways, glands, and the digestive tract. This animation shows how some CFTR mutations prevent the channel from...
published: 19 Apr 2018
developed with YouTube
5:04
Cystic Fibrosis: Pathophysiology, Genetics, Symptoms, Diagnosis and Treatments, Animation
- Order: Reorder
- Duration: 5:04
- Uploaded Date: 15 Aug 2022
- views: 158394
Cystic fibrosis, CF, is the most common inherited disorder that leads to shortened life expectancy, especially among people of Northern European origin.
CF is ...
Cystic fibrosis, CF, is the most common inherited disorder that leads to shortened life expectancy, especially among people of Northern European origin.
CF is caused by mutations in the CF transmembrane conductance regulator - CFTR gene.
Purchase a license to download a non-watermarked version of this video on AlilaMedicalMedia(dot)com
Check out our new Alila Academy - AlilaAcademy(dot)com - complete video courses with quizzes, PDFs, and downloadable images.
©Alila Medical Media. All rights reserved.
Voice by : Marty Henne
All images/videos by Alila Medical Media are for information purposes ONLY and are NOT intended to replace professional medical advice, diagnosis or treatment.
The CFTR protein encoded by CFTR gene functions as a chloride channel, allowing passage of chloride ions across cell membranes. Because chloride drives water movement, CFTR plays an important role in regulation of water and salt balance in secreted fluids such as mucus and sweat. Mutations in CFTR lead to production of thicker mucus in various organs, causing obstruction and other problems. CF patients also have saltier sweat.
Cystic fibrosis is inherited in an autosomal recessive manner. It occurs when a child inherits one mutation of the CFTR gene from each parent. The parents typically do not have the disease but are carriers – they carry one mutated gene and one normal gene.
More than 2000 different CFTR mutations have been identified so far, with the most common – deletion of the amino acid phenylalanine at position 508 - accounting for about 70% of all cases. Different mutations affect gene function to different degrees. The severity of the disease depends on which mutations a patient has.
The most severe form may manifest in the first days of life, when the meconium – the first stool of the newborn - is so thick and sticky that it is unable to pass, resulting in bowel obstruction – an emergency that requires immediate attention. On the other hand, patients with a milder CF may not show symptoms until their late childhood or even adulthood. Usually, the later the symptoms manifest, the milder the disease.
CF is a multi-system disorder but it affects mainly the digestive, respiratory, and urogenital organs. While respiratory insufficiency is the leading cause of mortality, digestive symptoms are usually the first to appear.
In the digestive system, obstruction of bile ducts may lead to poor fat absorption and possible liver damage. Thick secretions may also block the pancreatic duct, preventing pancreatic enzymes from reaching the intestine. In addition to poor digestion, this leads to accumulation of digestive enzymes in the pancreas, which destroy pancreatic tissue, causing inflammation, or pancreatitis. Damage of the pancreas may also result in shortage of beta-cells that produce insulin, causing insulin-dependent diabetes – a very common complication.
Other digestive symptoms include abdominal distension, intestinal obstruction, and intussusception, in which a segment of the intestine slides inside its adjacent segment.
In the lung, thick mucus cannot be readily cleared and becomes an environment for bacteria to grow and cause infection. Chronic infections, together with the resulting overwhelming inflammatory response, eventually lead to airway destruction. Thick mucus may also block the airways. Common respiratory symptoms include chronic cough, wheezing, and recurrent pneumonia.
In the urogenital system, CF may cause infertility in men and decreased fertility in women.
CF is part of the newborn screening test in many countries. The screening typically involves an immunoreactive trypsinogen test and a DNA test for common CFTR mutations. If the result is positive, a sweat test for chloride content is performed to confirm the diagnosis.
Once diagnosed, chest radiographs, pulmonary function tests and arterial blood gas analysis, may be used to follow disease progression.
There is no definitive cure for CF but early diagnosis can help manage the condition and increase lifespan. A number of medications and therapies are available to help prevent or control lung infections, clear or loosen mucus from the lungs, prevent or treat intestinal blockage, and improve digestion. Lung transplantation maybe required for severe lung damage.
Recently, new modulator molecules have been developed to directly interact with the mutated CFTR protein to correct its folding and facilitate its function. Notably, a combination of 3 medications has been proven to effectively manage CF caused by the F-508 deletion.
https://wn.com/Cystic_Fibrosis_Pathophysiology,_Genetics,_Symptoms,_Diagnosis_And_Treatments,_Animation
Cystic fibrosis, CF, is the most common inherited disorder that leads to shortened life expectancy, especially among people of Northern European origin.
CF is caused by mutations in the CF transmembrane conductance regulator - CFTR gene.
Purchase a license to download a non-watermarked version of this video on AlilaMedicalMedia(dot)com
Check out our new Alila Academy - AlilaAcademy(dot)com - complete video courses with quizzes, PDFs, and downloadable images.
©Alila Medical Media. All rights reserved.
Voice by : Marty Henne
All images/videos by Alila Medical Media are for information purposes ONLY and are NOT intended to replace professional medical advice, diagnosis or treatment.
The CFTR protein encoded by CFTR gene functions as a chloride channel, allowing passage of chloride ions across cell membranes. Because chloride drives water movement, CFTR plays an important role in regulation of water and salt balance in secreted fluids such as mucus and sweat. Mutations in CFTR lead to production of thicker mucus in various organs, causing obstruction and other problems. CF patients also have saltier sweat.
Cystic fibrosis is inherited in an autosomal recessive manner. It occurs when a child inherits one mutation of the CFTR gene from each parent. The parents typically do not have the disease but are carriers – they carry one mutated gene and one normal gene.
More than 2000 different CFTR mutations have been identified so far, with the most common – deletion of the amino acid phenylalanine at position 508 - accounting for about 70% of all cases. Different mutations affect gene function to different degrees. The severity of the disease depends on which mutations a patient has.
The most severe form may manifest in the first days of life, when the meconium – the first stool of the newborn - is so thick and sticky that it is unable to pass, resulting in bowel obstruction – an emergency that requires immediate attention. On the other hand, patients with a milder CF may not show symptoms until their late childhood or even adulthood. Usually, the later the symptoms manifest, the milder the disease.
CF is a multi-system disorder but it affects mainly the digestive, respiratory, and urogenital organs. While respiratory insufficiency is the leading cause of mortality, digestive symptoms are usually the first to appear.
In the digestive system, obstruction of bile ducts may lead to poor fat absorption and possible liver damage. Thick secretions may also block the pancreatic duct, preventing pancreatic enzymes from reaching the intestine. In addition to poor digestion, this leads to accumulation of digestive enzymes in the pancreas, which destroy pancreatic tissue, causing inflammation, or pancreatitis. Damage of the pancreas may also result in shortage of beta-cells that produce insulin, causing insulin-dependent diabetes – a very common complication.
Other digestive symptoms include abdominal distension, intestinal obstruction, and intussusception, in which a segment of the intestine slides inside its adjacent segment.
In the lung, thick mucus cannot be readily cleared and becomes an environment for bacteria to grow and cause infection. Chronic infections, together with the resulting overwhelming inflammatory response, eventually lead to airway destruction. Thick mucus may also block the airways. Common respiratory symptoms include chronic cough, wheezing, and recurrent pneumonia.
In the urogenital system, CF may cause infertility in men and decreased fertility in women.
CF is part of the newborn screening test in many countries. The screening typically involves an immunoreactive trypsinogen test and a DNA test for common CFTR mutations. If the result is positive, a sweat test for chloride content is performed to confirm the diagnosis.
Once diagnosed, chest radiographs, pulmonary function tests and arterial blood gas analysis, may be used to follow disease progression.
There is no definitive cure for CF but early diagnosis can help manage the condition and increase lifespan. A number of medications and therapies are available to help prevent or control lung infections, clear or loosen mucus from the lungs, prevent or treat intestinal blockage, and improve digestion. Lung transplantation maybe required for severe lung damage.
Recently, new modulator molecules have been developed to directly interact with the mutated CFTR protein to correct its folding and facilitate its function. Notably, a combination of 3 medications has been proven to effectively manage CF caused by the F-508 deletion.
8:03
Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
- Order: Reorder
- Duration: 8:03
- Uploaded Date: 28 Mar 2022
- views: 20345
What is cystic fibrosis? Can it be cured? How is it diagnosed? Manuela Cernadas, M.D., Pulmonologist at the Lung Center at Brigham and Women's Hospital and Assi...
What is cystic fibrosis? Can it be cured? How is it diagnosed? Manuela Cernadas, M.D., Pulmonologist at the Lung Center at Brigham and Women's Hospital and Assistant Professor of Medicine at Harvard Medical School, answers people's most commonly searched questions about cystic fibrosis (CF).
Subscribe Link: https://www.youtube.com/channel/UCYrLjATd88gPwIKntCoR0WQ?sub_confirmation=1
0:00 - Intro
0:27 - What is cystic fibrosis?
1:03 - Can cystic fibrosis be cured?
1:47 - How is cystic fibrosis diagnosed?
2:46 - Can I carry cystic fibrosis without realizing it?
3:26 - What are the effects of cystic fibrosis?
4:07 - What is the treatment for cystic fibrosis?
5:29 - How do therapy vests work?
5:54 - Can I have children with cystic fibrosis?
6:24 - Is lung transplantation a possibility?
About Mass General Brigham:
Mass General Brigham combines the strength of two world-class academic medical centers, five nationally ranked specialty hospitals, 11 community hospitals, and dozens of health centers. Our doctors and researchers accelerate medical breakthroughs and drive innovations in patient care. They are leaders in medical education, serving as Harvard Medical School faculty and training the next generation of physicians. Mass General Brigham’s mission is to deliver the best, affordable health care to patients everywhere. Together, we transform the health of our communities and beyond.
#CysticFibrosis #CF #MassGeneralBrigham
Visit Mass General Brigham: https://www.massgeneralbrigham.org/
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Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
https://youtu.be/YpZdRRjbpi8
https://wn.com/Cystic_Fibrosis_(Cf)_Symptoms,_Inheritance,_Treatment,_And_More_|_Mass_General_Brigham
What is cystic fibrosis? Can it be cured? How is it diagnosed? Manuela Cernadas, M.D., Pulmonologist at the Lung Center at Brigham and Women's Hospital and Assistant Professor of Medicine at Harvard Medical School, answers people's most commonly searched questions about cystic fibrosis (CF).
Subscribe Link: https://www.youtube.com/channel/UCYrLjATd88gPwIKntCoR0WQ?sub_confirmation=1
0:00 - Intro
0:27 - What is cystic fibrosis?
1:03 - Can cystic fibrosis be cured?
1:47 - How is cystic fibrosis diagnosed?
2:46 - Can I carry cystic fibrosis without realizing it?
3:26 - What are the effects of cystic fibrosis?
4:07 - What is the treatment for cystic fibrosis?
5:29 - How do therapy vests work?
5:54 - Can I have children with cystic fibrosis?
6:24 - Is lung transplantation a possibility?
About Mass General Brigham:
Mass General Brigham combines the strength of two world-class academic medical centers, five nationally ranked specialty hospitals, 11 community hospitals, and dozens of health centers. Our doctors and researchers accelerate medical breakthroughs and drive innovations in patient care. They are leaders in medical education, serving as Harvard Medical School faculty and training the next generation of physicians. Mass General Brigham’s mission is to deliver the best, affordable health care to patients everywhere. Together, we transform the health of our communities and beyond.
#CysticFibrosis #CF #MassGeneralBrigham
Visit Mass General Brigham: https://www.massgeneralbrigham.org/
Find us on social:
Twitter: https://twitter.com/MassGenBrigham
Instagram: https://www.instagram.com/massgeneralbrigham/
Facebook: https://www.facebook.com/MassGeneralBrigham/
LinkedIn: https://www.linkedin.com/company/mass-general-brigham/
Mass General Brigham:
https://www.youtube.com/massgeneralbrigham
Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
https://youtu.be/YpZdRRjbpi8
- published: 28 Mar 2022
- views: 20345
2:03
How Cystic Fibrosis Affects the Lungs
- Order: Reorder
- Duration: 2:03
- Uploaded Date: 19 May 2017
- views: 260645
See how the lungs work normally and how cystic fibrosis can affect the lungs over time.
https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clear...
See how the lungs work normally and how cystic fibrosis can affect the lungs over time.
https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/Basics-of-Lung-Care/
https://wn.com/How_Cystic_Fibrosis_Affects_The_Lungs
See how the lungs work normally and how cystic fibrosis can affect the lungs over time.
https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/Basics-of-Lung-Care/
- published: 19 May 2017
- views: 260645
26:13
Cystic Fibrosis Nursing | Cystic Fibrosis Symptoms, Causes, Treatment, NCLEX Review
- Order: Reorder
- Duration: 26:13
- Uploaded Date: 03 Jul 2018
- views: 1055222
Cystic fibrosis NCLEX nursing review: Cystic fibrosis is a genetic disorder that causes the exocrine glands to work incorrectly.
This can lead to major compli...
Cystic fibrosis NCLEX nursing review: Cystic fibrosis is a genetic disorder that causes the exocrine glands to work incorrectly.
This can lead to major complications that affect the respiratory, digestive (pancreas, liver, intestines), integumentary, and reproductive systems along with the sinuses.
What are exocrine glands? These are glands that produce and transfer it’s secretions (ex: mucous, tears, sweat, digestive enzymes) via DUCTS to it’s intended area of the body rather than directly into the bloodstream like the endocrine glands.
In cystic fibrosis, there is a gene that is mutated that prevents the exocrine gland from working properly. It is called the CFTR gene!
The CFTR gene, which stands for cystic fibrosis transmembrane regulator, is a protein that controls the channels of sodium and chloride. Hence the sodium and water transport in and out of the cell, and these channels are within the membrane of the cells that makes our sweat, mucous, tears, and digestive enzymes.
However, in CF these channels don’t work properly. Therefore, normally these channels would properly regulated sodium and water transport in and out of the cells, which helps control the flow of water within these structures among other things. But this is not what happens in a person with cystic fibrosis.
The mucous is thick and sticky rather than thin and slippery. This mucous will block the airways in the lower respiratory system and block the pancreatic duct from delivering pancreatic enzymes. In addition, the sweat glands produce excessive amounts of salt.
CF tends to affect Caucasians and males more. The current median life expectancy of a person with cystic fibrosis is 37 years of age (source: NIH.gov). It is currently diagnosed with a sweat test where pilocaprine and electric current is delivered to the skin to make it sweat. The amount of salt in the sweat is measured. Results 60 mmol/L or more is considered positive for CF.
Nursing interventions to cystic fibrosis include: chest physiotherapy (chest PT), monitor for complications, educating about PEP devices, preventing infection, administering medications and more (see the video for a detailed review of the nursing interventions)
🟣ABG eBook: https://registerednursern.creator-spring.com/
🟣ABG physical book: https://amzn.to/3EsF0Mc (affiliate link)
Quiz: http://www.registerednursern.com/cystic-fibrosis-nclex-questions/
Notes: http://www.registerednursern.com/cystic-fibrosis-nclex-review/
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https://wn.com/Cystic_Fibrosis_Nursing_|_Cystic_Fibrosis_Symptoms,_Causes,_Treatment,_Nclex_Review
Cystic fibrosis NCLEX nursing review: Cystic fibrosis is a genetic disorder that causes the exocrine glands to work incorrectly.
This can lead to major complications that affect the respiratory, digestive (pancreas, liver, intestines), integumentary, and reproductive systems along with the sinuses.
What are exocrine glands? These are glands that produce and transfer it’s secretions (ex: mucous, tears, sweat, digestive enzymes) via DUCTS to it’s intended area of the body rather than directly into the bloodstream like the endocrine glands.
In cystic fibrosis, there is a gene that is mutated that prevents the exocrine gland from working properly. It is called the CFTR gene!
The CFTR gene, which stands for cystic fibrosis transmembrane regulator, is a protein that controls the channels of sodium and chloride. Hence the sodium and water transport in and out of the cell, and these channels are within the membrane of the cells that makes our sweat, mucous, tears, and digestive enzymes.
However, in CF these channels don’t work properly. Therefore, normally these channels would properly regulated sodium and water transport in and out of the cells, which helps control the flow of water within these structures among other things. But this is not what happens in a person with cystic fibrosis.
The mucous is thick and sticky rather than thin and slippery. This mucous will block the airways in the lower respiratory system and block the pancreatic duct from delivering pancreatic enzymes. In addition, the sweat glands produce excessive amounts of salt.
CF tends to affect Caucasians and males more. The current median life expectancy of a person with cystic fibrosis is 37 years of age (source: NIH.gov). It is currently diagnosed with a sweat test where pilocaprine and electric current is delivered to the skin to make it sweat. The amount of salt in the sweat is measured. Results 60 mmol/L or more is considered positive for CF.
Nursing interventions to cystic fibrosis include: chest physiotherapy (chest PT), monitor for complications, educating about PEP devices, preventing infection, administering medications and more (see the video for a detailed review of the nursing interventions)
🟣ABG eBook: https://registerednursern.creator-spring.com/
🟣ABG physical book: https://amzn.to/3EsF0Mc (affiliate link)
Quiz: http://www.registerednursern.com/cystic-fibrosis-nclex-questions/
Notes: http://www.registerednursern.com/cystic-fibrosis-nclex-review/
More Pediatric Reviews: https://www.youtube.com/watch?v=QCgcUtWkWqE&list=PLQrdx7rRsKfXmfA3CoozS5N767bLpnrbm
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- published: 03 Jul 2018
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3:46
What is cystic fibrosis, exactly?
- Order: Reorder
- Duration: 3:46
- Uploaded Date: 19 Jun 2017
- views: 326508
Cystic fibrosis is a genetic condition mostly affecting the lungs and digestive system.
You are born with cystic fibrosis and cannot catch it later in life, b...
Cystic fibrosis is a genetic condition mostly affecting the lungs and digestive system.
You are born with cystic fibrosis and cannot catch it later in life, but one in 25 of us carries the faulty gene that causes it, usually without even knowing.
Presented by Charles Michael Duke.
Join the fight for a Life Unlimited by cystic fibrosis.
Visit https://www.cysticfibrosis.org.uk
https://wn.com/What_Is_Cystic_Fibrosis,_Exactly
Cystic fibrosis is a genetic condition mostly affecting the lungs and digestive system.
You are born with cystic fibrosis and cannot catch it later in life, but one in 25 of us carries the faulty gene that causes it, usually without even knowing.
Presented by Charles Michael Duke.
Join the fight for a Life Unlimited by cystic fibrosis.
Visit https://www.cysticfibrosis.org.uk
- published: 19 Jun 2017
- views: 326508
6:26
CYSTIC FIBROSIS, Causes, Signs and Symptoms, Diagnosis and Treatment.
- Order: Reorder
- Duration: 6:26
- Uploaded Date: 11 Apr 2019
- views: 77341
.
Chapters
0:00 Introduction
2:28 Causes of Cystic fibrosis
3:14 Symptoms of Cystic fibrosis
3:58 Diagnosis of Cystic fibrosis
5:27 Treatment of Cystic fibros...
.
Chapters
0:00 Introduction
2:28 Causes of Cystic fibrosis
3:14 Symptoms of Cystic fibrosis
3:58 Diagnosis of Cystic fibrosis
5:27 Treatment of Cystic fibrosis
Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine.Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males.Different people may have different degrees of symptoms.
CF is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein.Those with a single working copy are carriers and otherwise mostly normal. CFTR is involved in production of sweat, digestive fluids, and mucus.When the CFTR is not functional, secretions which are usually thin instead become thick. The condition is diagnosed by a sweat test and genetic testing. Screening of infants at birth takes place in some areas of the world.
https://wn.com/Cystic_Fibrosis,_Causes,_Signs_And_Symptoms,_Diagnosis_And_Treatment.
.
Chapters
0:00 Introduction
2:28 Causes of Cystic fibrosis
3:14 Symptoms of Cystic fibrosis
3:58 Diagnosis of Cystic fibrosis
5:27 Treatment of Cystic fibrosis
Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine.Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males.Different people may have different degrees of symptoms.
CF is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein.Those with a single working copy are carriers and otherwise mostly normal. CFTR is involved in production of sweat, digestive fluids, and mucus.When the CFTR is not functional, secretions which are usually thin instead become thick. The condition is diagnosed by a sweat test and genetic testing. Screening of infants at birth takes place in some areas of the world.
- published: 11 Apr 2019
- views: 77341
2:48
Living with Cystic Fibrosis
- Order: Reorder
- Duration: 2:48
- Uploaded Date: 20 Mar 2012
- views: 36599
An adult with CF talks about how she has managed her disease growing up and has accomplished many milestones.
An adult with CF talks about how she has managed her disease growing up and has accomplished many milestones.
https://wn.com/Living_With_Cystic_Fibrosis
An adult with CF talks about how she has managed her disease growing up and has accomplished many milestones.
- published: 20 Mar 2012
- views: 36599
2:32
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
- Order: Reorder
- Duration: 2:32
- Uploaded Date: 01 Jan 2025
- views: 345
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
Subscribe to WVTM on YouTube now for more: https://bit.ly/2jvAaUD
...
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
Subscribe to WVTM on YouTube now for more: https://bit.ly/2jvAaUD
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https://wn.com/Meet_Miss_Alabama_And_Vestavia_Hills_Native,_Abbie_Stockard,_As_She_Competes_In_Miss_America
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
Subscribe to WVTM on YouTube now for more: https://bit.ly/2jvAaUD
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- published: 01 Jan 2025
- views: 345
17:19
Cystic Fibrosis - Definition, Pathogenesis, Symptoms and Complications - Genetics
- Order: Reorder
- Duration: 17:19
- Uploaded Date: 16 Sep 2019
- views: 144411
Cystic fibrosis is an autosomal recessive genetic disease characterized by defective CFTR protein (defect in chloride channels in the sweat glands, pancreatic d...
Cystic fibrosis is an autosomal recessive genetic disease characterized by defective CFTR protein (defect in chloride channels in the sweat glands, pancreatic ducts, bronchi, bronchioles,…etc.)…Genetics lectures, Respiratory pathology.
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https://wn.com/Cystic_Fibrosis_Definition,_Pathogenesis,_Symptoms_And_Complications_Genetics
Cystic fibrosis is an autosomal recessive genetic disease characterized by defective CFTR protein (defect in chloride channels in the sweat glands, pancreatic ducts, bronchi, bronchioles,…etc.)…Genetics lectures, Respiratory pathology.
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►👨🏫💊Antibiotics Lectures: https://www.medicosisperfectionalis.com/products/courses/antibiotics/ ... Check out my brand new "Electrolytes" course at https://www.medicosisperfectionalis.com/products/course/electrolytes/ and use the PROMO code: ELECTROLYTES50 to get a 50% discount. If you like my videos, please consider leaving a tip at https://www.paypal.me/perfectionalis/
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- published: 16 Sep 2019
- views: 144411
2:31
Cystic Fibrosis Mechanism and Treatment | HHMI BioInteractive Video
- Order: Reorder
- Duration: 2:31
- Uploaded Date: 19 Apr 2018
- views: 261428
Mutations in the CFTR gene, which encodes a transmembrane ion channel, cause mucus buildup in the airways of patients with cystic fibrosis.
This animation can ...
Mutations in the CFTR gene, which encodes a transmembrane ion channel, cause mucus buildup in the airways of patients with cystic fibrosis.
This animation can be used to illustrate the connection between genotype and phenotype using a human disease example. It also demonstrates how knowledge about the genetic mutations responsible for a particular disease can be used to develop a treatment.
Cystic fibrosis is an autosomal recessive disease caused by any one of more than 2,000 mutations in a gene that codes for the cystic fibrosis transmembrane conductance regulator (CFTR). This protein functions as a channel that transports chloride ions across the membranes of cells that line airways, glands, and the digestive tract. This animation shows how some CFTR mutations prevent the channel from functioning properly, leading to the production of a thick mucus that can obstruct airways and provide a breeding ground for infections. The animation also illustrates the process by which scientists identified small molecule drugs that can improve CFTR channel function and treat the disease in some patients.
https://wn.com/Cystic_Fibrosis_Mechanism_And_Treatment_|_Hhmi_Biointeractive_Video
Mutations in the CFTR gene, which encodes a transmembrane ion channel, cause mucus buildup in the airways of patients with cystic fibrosis.
This animation can be used to illustrate the connection between genotype and phenotype using a human disease example. It also demonstrates how knowledge about the genetic mutations responsible for a particular disease can be used to develop a treatment.
Cystic fibrosis is an autosomal recessive disease caused by any one of more than 2,000 mutations in a gene that codes for the cystic fibrosis transmembrane conductance regulator (CFTR). This protein functions as a channel that transports chloride ions across the membranes of cells that line airways, glands, and the digestive tract. This animation shows how some CFTR mutations prevent the channel from functioning properly, leading to the production of a thick mucus that can obstruct airways and provide a breeding ground for infections. The animation also illustrates the process by which scientists identified small molecule drugs that can improve CFTR channel function and treat the disease in some patients.
- published: 19 Apr 2018
- views: 261428
5:04
Cystic Fibrosis: Pathophysiology, Genetics, Symptoms, Diagnosis and Treatments, Animation
Cystic fibrosis, CF, is the most common inherited disorder that leads to shortened life ex...
published: 15 Aug 2022
Cystic Fibrosis: Pathophysiology, Genetics, Symptoms, Diagnosis and Treatments, Animation
Cystic Fibrosis: Pathophysiology, Genetics, Symptoms, Diagnosis and Treatments, Animation
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- published: 15 Aug 2022
- views: 158394
Cystic fibrosis, CF, is the most common inherited disorder that leads to shortened life expectancy, especially among people of Northern European origin.
CF is caused by mutations in the CF transmembrane conductance regulator - CFTR gene.
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The CFTR protein encoded by CFTR gene functions as a chloride channel, allowing passage of chloride ions across cell membranes. Because chloride drives water movement, CFTR plays an important role in regulation of water and salt balance in secreted fluids such as mucus and sweat. Mutations in CFTR lead to production of thicker mucus in various organs, causing obstruction and other problems. CF patients also have saltier sweat.
Cystic fibrosis is inherited in an autosomal recessive manner. It occurs when a child inherits one mutation of the CFTR gene from each parent. The parents typically do not have the disease but are carriers – they carry one mutated gene and one normal gene.
More than 2000 different CFTR mutations have been identified so far, with the most common – deletion of the amino acid phenylalanine at position 508 - accounting for about 70% of all cases. Different mutations affect gene function to different degrees. The severity of the disease depends on which mutations a patient has.
The most severe form may manifest in the first days of life, when the meconium – the first stool of the newborn - is so thick and sticky that it is unable to pass, resulting in bowel obstruction – an emergency that requires immediate attention. On the other hand, patients with a milder CF may not show symptoms until their late childhood or even adulthood. Usually, the later the symptoms manifest, the milder the disease.
CF is a multi-system disorder but it affects mainly the digestive, respiratory, and urogenital organs. While respiratory insufficiency is the leading cause of mortality, digestive symptoms are usually the first to appear.
In the digestive system, obstruction of bile ducts may lead to poor fat absorption and possible liver damage. Thick secretions may also block the pancreatic duct, preventing pancreatic enzymes from reaching the intestine. In addition to poor digestion, this leads to accumulation of digestive enzymes in the pancreas, which destroy pancreatic tissue, causing inflammation, or pancreatitis. Damage of the pancreas may also result in shortage of beta-cells that produce insulin, causing insulin-dependent diabetes – a very common complication.
Other digestive symptoms include abdominal distension, intestinal obstruction, and intussusception, in which a segment of the intestine slides inside its adjacent segment.
In the lung, thick mucus cannot be readily cleared and becomes an environment for bacteria to grow and cause infection. Chronic infections, together with the resulting overwhelming inflammatory response, eventually lead to airway destruction. Thick mucus may also block the airways. Common respiratory symptoms include chronic cough, wheezing, and recurrent pneumonia.
In the urogenital system, CF may cause infertility in men and decreased fertility in women.
CF is part of the newborn screening test in many countries. The screening typically involves an immunoreactive trypsinogen test and a DNA test for common CFTR mutations. If the result is positive, a sweat test for chloride content is performed to confirm the diagnosis.
Once diagnosed, chest radiographs, pulmonary function tests and arterial blood gas analysis, may be used to follow disease progression.
There is no definitive cure for CF but early diagnosis can help manage the condition and increase lifespan. A number of medications and therapies are available to help prevent or control lung infections, clear or loosen mucus from the lungs, prevent or treat intestinal blockage, and improve digestion. Lung transplantation maybe required for severe lung damage.
Recently, new modulator molecules have been developed to directly interact with the mutated CFTR protein to correct its folding and facilitate its function. Notably, a combination of 3 medications has been proven to effectively manage CF caused by the F-508 deletion.
8:03
Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
What is cystic fibrosis? Can it be cured? How is it diagnosed? Manuela Cernadas, M.D., Pul...
published: 28 Mar 2022
Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
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- published: 28 Mar 2022
- views: 20345
What is cystic fibrosis? Can it be cured? How is it diagnosed? Manuela Cernadas, M.D., Pulmonologist at the Lung Center at Brigham and Women's Hospital and Assistant Professor of Medicine at Harvard Medical School, answers people's most commonly searched questions about cystic fibrosis (CF).
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0:00 - Intro
0:27 - What is cystic fibrosis?
1:03 - Can cystic fibrosis be cured?
1:47 - How is cystic fibrosis diagnosed?
2:46 - Can I carry cystic fibrosis without realizing it?
3:26 - What are the effects of cystic fibrosis?
4:07 - What is the treatment for cystic fibrosis?
5:29 - How do therapy vests work?
5:54 - Can I have children with cystic fibrosis?
6:24 - Is lung transplantation a possibility?
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Mass General Brigham combines the strength of two world-class academic medical centers, five nationally ranked specialty hospitals, 11 community hospitals, and dozens of health centers. Our doctors and researchers accelerate medical breakthroughs and drive innovations in patient care. They are leaders in medical education, serving as Harvard Medical School faculty and training the next generation of physicians. Mass General Brigham’s mission is to deliver the best, affordable health care to patients everywhere. Together, we transform the health of our communities and beyond.
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Cystic Fibrosis (CF): Symptoms, Inheritance, Treatment, and More | Mass General Brigham
https://youtu.be/YpZdRRjbpi8
2:03
How Cystic Fibrosis Affects the Lungs
See how the lungs work normally and how cystic fibrosis can affect the lungs over time.
...
published: 19 May 2017
How Cystic Fibrosis Affects the Lungs
How Cystic Fibrosis Affects the Lungs
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- published: 19 May 2017
- views: 260645
See how the lungs work normally and how cystic fibrosis can affect the lungs over time.
https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/Basics-of-Lung-Care/
26:13
Cystic Fibrosis Nursing | Cystic Fibrosis Symptoms, Causes, Treatment, NCLEX Review
Cystic fibrosis NCLEX nursing review: Cystic fibrosis is a genetic disorder that causes th...
published: 03 Jul 2018
Cystic Fibrosis Nursing | Cystic Fibrosis Symptoms, Causes, Treatment, NCLEX Review
Cystic Fibrosis Nursing | Cystic Fibrosis Symptoms, Causes, Treatment, NCLEX Review
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- published: 03 Jul 2018
- views: 1055222
Cystic fibrosis NCLEX nursing review: Cystic fibrosis is a genetic disorder that causes the exocrine glands to work incorrectly.
This can lead to major complications that affect the respiratory, digestive (pancreas, liver, intestines), integumentary, and reproductive systems along with the sinuses.
What are exocrine glands? These are glands that produce and transfer it’s secretions (ex: mucous, tears, sweat, digestive enzymes) via DUCTS to it’s intended area of the body rather than directly into the bloodstream like the endocrine glands.
In cystic fibrosis, there is a gene that is mutated that prevents the exocrine gland from working properly. It is called the CFTR gene!
The CFTR gene, which stands for cystic fibrosis transmembrane regulator, is a protein that controls the channels of sodium and chloride. Hence the sodium and water transport in and out of the cell, and these channels are within the membrane of the cells that makes our sweat, mucous, tears, and digestive enzymes.
However, in CF these channels don’t work properly. Therefore, normally these channels would properly regulated sodium and water transport in and out of the cells, which helps control the flow of water within these structures among other things. But this is not what happens in a person with cystic fibrosis.
The mucous is thick and sticky rather than thin and slippery. This mucous will block the airways in the lower respiratory system and block the pancreatic duct from delivering pancreatic enzymes. In addition, the sweat glands produce excessive amounts of salt.
CF tends to affect Caucasians and males more. The current median life expectancy of a person with cystic fibrosis is 37 years of age (source: NIH.gov). It is currently diagnosed with a sweat test where pilocaprine and electric current is delivered to the skin to make it sweat. The amount of salt in the sweat is measured. Results 60 mmol/L or more is considered positive for CF.
Nursing interventions to cystic fibrosis include: chest physiotherapy (chest PT), monitor for complications, educating about PEP devices, preventing infection, administering medications and more (see the video for a detailed review of the nursing interventions)
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3:46
What is cystic fibrosis, exactly?
Cystic fibrosis is a genetic condition mostly affecting the lungs and digestive system.
...
published: 19 Jun 2017
What is cystic fibrosis, exactly?
What is cystic fibrosis, exactly?
- Report rights infringement
- published: 19 Jun 2017
- views: 326508
Cystic fibrosis is a genetic condition mostly affecting the lungs and digestive system.
You are born with cystic fibrosis and cannot catch it later in life, but one in 25 of us carries the faulty gene that causes it, usually without even knowing.
Presented by Charles Michael Duke.
Join the fight for a Life Unlimited by cystic fibrosis.
Visit https://www.cysticfibrosis.org.uk
6:26
CYSTIC FIBROSIS, Causes, Signs and Symptoms, Diagnosis and Treatment.
.
Chapters
0:00 Introduction
2:28 Causes of Cystic fibrosis
3:14 Symptoms of Cystic fibr...
published: 11 Apr 2019
CYSTIC FIBROSIS, Causes, Signs and Symptoms, Diagnosis and Treatment.
CYSTIC FIBROSIS, Causes, Signs and Symptoms, Diagnosis and Treatment.
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- published: 11 Apr 2019
- views: 77341
.
Chapters
0:00 Introduction
2:28 Causes of Cystic fibrosis
3:14 Symptoms of Cystic fibrosis
3:58 Diagnosis of Cystic fibrosis
5:27 Treatment of Cystic fibrosis
Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine.Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males.Different people may have different degrees of symptoms.
CF is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein.Those with a single working copy are carriers and otherwise mostly normal. CFTR is involved in production of sweat, digestive fluids, and mucus.When the CFTR is not functional, secretions which are usually thin instead become thick. The condition is diagnosed by a sweat test and genetic testing. Screening of infants at birth takes place in some areas of the world.
2:48
Living with Cystic Fibrosis
An adult with CF talks about how she has managed her disease growing up and has accomplish...
published: 20 Mar 2012
Living with Cystic Fibrosis
Living with Cystic Fibrosis
- Report rights infringement
- published: 20 Mar 2012
- views: 36599
An adult with CF talks about how she has managed her disease growing up and has accomplished many milestones.
2:32
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss Ameri...
published: 01 Jan 2025
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
- Report rights infringement
- published: 01 Jan 2025
- views: 345
Meet Miss Alabama and Vestavia Hills native, Abbie Stockard, as she competes in Miss America
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17:19
Cystic Fibrosis - Definition, Pathogenesis, Symptoms and Complications - Genetics
Cystic fibrosis is an autosomal recessive genetic disease characterized by defective CFTR ...
published: 16 Sep 2019
Cystic Fibrosis - Definition, Pathogenesis, Symptoms and Complications - Genetics
Cystic Fibrosis - Definition, Pathogenesis, Symptoms and Complications - Genetics
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- published: 16 Sep 2019
- views: 144411
Cystic fibrosis is an autosomal recessive genetic disease characterized by defective CFTR protein (defect in chloride channels in the sweat glands, pancreatic ducts, bronchi, bronchioles,…etc.)…Genetics lectures, Respiratory pathology.
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Cystic Fibrosis Mechanism and Treatment | HHMI BioInteractive Video
Mutations in the CFTR gene, which encodes a transmembrane ion channel, cause mucus buildup...
published: 19 Apr 2018
Cystic Fibrosis Mechanism and Treatment | HHMI BioInteractive Video
Cystic Fibrosis Mechanism and Treatment | HHMI BioInteractive Video
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- published: 19 Apr 2018
- views: 261428
Mutations in the CFTR gene, which encodes a transmembrane ion channel, cause mucus buildup in the airways of patients with cystic fibrosis.
This animation can be used to illustrate the connection between genotype and phenotype using a human disease example. It also demonstrates how knowledge about the genetic mutations responsible for a particular disease can be used to develop a treatment.
Cystic fibrosis is an autosomal recessive disease caused by any one of more than 2,000 mutations in a gene that codes for the cystic fibrosis transmembrane conductance regulator (CFTR). This protein functions as a channel that transports chloride ions across the membranes of cells that line airways, glands, and the digestive tract. This animation shows how some CFTR mutations prevent the channel from functioning properly, leading to the production of a thick mucus that can obstruct airways and provide a breeding ground for infections. The animation also illustrates the process by which scientists identified small molecule drugs that can improve CFTR channel function and treat the disease in some patients.
Cystic fibrosis
Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in males among others. Different people may have different degrees of symptoms.
CF is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Those with a single working copy are carriers and otherwise mostly normal. CFTR is involved in production of sweat, digestive fluids, and mucus. When CFTR is not functional, secretions which are usually thin instead become thick. The condition is diagnosed by a sweat test and genetic testing. Screening of infants at birth takes place in some areas of the world.
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by Malignancy
Cystic Fibrosis
by: MalignancyInherited disorder manifests with no cure
Defective genes carry this abnormality
Cell clusters fail to create necessary juices
Deficient pancreatic enzymes prohibit the digestive process
Weakling newborn not capable of fending for itself
Clogged passages instill upper lung infection
Obstruction of intestinal glands
Foul smelling stool blocks the bowel
Gradual liver atrophy impairs insulin production
Infant ravaged with diabetes
Medicine administered to stabilize blood sugar
Thick mucus blocks the exocrine glands
Respiratory contamination spreads within
Debilitating illness cuts chance of survival
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