Cystic fibrosis disease, a genetically inherited disease of the exocrine glands in man, has no known counterpart in animals.
Cystic Fibrosis Symptoms
The symptoms commonly show up in mucous obstructing the lungs, pancreas and liver. The signs, symptoms of fibrosis and course of an analogous disease in animals have been predicted. If an analogous disease in animals can be discovered, it is anticipated that it will make possible significant advances in the study of the normal and pathological physiology of the abnormal exocrine glands and will lead to a means of controlling the human disease. In clasification detail cystic fibrosis symptoms describe above.
- A persistent cough that produces thick mucus (sputum)
- Wheezing is high-pitched whistling sound while take breathe. It’s heard most clearly on exhale, but in some cases can be heard when inhale. Wheezing caused by narrowed airways or inflammation in respiratory system.
- Breathlessness commonly in this case because the tissue organ become damege.
- Repeated lung infections. This repeat because this organ susceptible.
- Inflamed nasal passages or a stuffy nose
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The abnormal mucus can also plug up the channels that carry the enzymes produced by the pancreas to the small intestine. Without these digestive enzymes, the intestine can’t absorb the necessary nutrients from food. This can result in:
- greasy, foul-smelling stools
- a swollen abdomen
- loss of appetite
- poor weight gain in children
- delayed growth in children
Uncontrolled, progressive fibrosis disease is a driving factor in or hallmark of many debilitating disease states, afflicting millions of individuals in the United States and worldwide. Fibrosis-related diseases can affect organs including the kidney, liver, lungs, skin, and eye, leading to organ dysfunction and failure, and potentially death.
What Causes Cystic Fibrosis
Cystic fibrosis occurs as a result of a defect in what’s called the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene. This gene controls water and salt or mineral movement in and out of your body’s cells. A sudden mutation or change of CFTR gene causes your mucus to become thicker and stickier than normal. This abnormal mucus builds up in various organs throughout the body, including the:
It also increases the amount of salt in your sweat.
Many different defects can affect the CFTR gene. The type of defect is associate with the severity of cystic fibrosis. The damaged gene is passed on to the child from their parents. In order to have cystic fibrosis, a child must inherit one copy of the gene from each parent. If they only inherit one copy of the gene, they won’t develop the disease. However, they’ll be a carrier of the defective gene, which means that they may pass the gene on to their own children.
Cystic Fibrosis Carrier Testing
Immunoreactive Trypsinogen (IRT) Test
This is new method to check abnormal levels of the protein called IRT in the blood. This measurement using time-resolved fluoroimmunoassay in neonatal dried blood spots collected at 48th to 120th hour from birth. A second sample is usually obtained between the 20th and the 30th day from infants whose first result was higher than the cut-off value (99th centile). The predictive value of IRT dramatically decreases after the sixth week. All the neonates are systematically controlled for second sample IRT.
Sweat Chloride Test
This test measures the concentration of chloride that is excreted in sweat. Pilocarpine iontophoresis has role in inducing sweat. From that above this test need 2 electrode surrounding that. Electrode is place over gauze containing pilocarpine and electrolyte solution that will not interfere with the sodium and chloride measurement. A second ones that without pilocarpine place at another site and a mild electric current will draw the pilocarpine into the skin where it stimulates the sweat glands.
A sputum culture is a sample of the gooey substance that often comes up from your chest when you have an infection in your lungs or airways. Doctor will takes a sample of mucus.
X-ray is type of light that have power to penetrate skin.
Scan of internal organ with CT Scan devices.
Pulmonary Function Tests (PFTs)
PFT can be done with 2 methods. These 2 methods may be used together and perform different tests, depending on the information that your healthcare provider is looking for:
- Spirometry. A spirometer is a device with a mouthpiece hooked up to a small electronic machine.
- Plethysmography. You sit or stand inside an air-tight box that looks like a short, square telephone booth to do the tests.
This test will measure some standart :
- Tidal volume (VT) is the amount of air inhale or exhale during normal breathing.
- Minute volume (MV) is the total amount of air exhale per minute.
- Vital capacity (VC) is the total volume of air that can be exhale after inhaling as much as you can.
- Functional residual capacity (FRC) is the amount of air left in lungs after exhaling normally.
- Residual volume is the amount of air left in the lungs after exhaling as much as you can.
- Total lung capacity is the total volume of the lungs when filled with as much air as possible.
- Forced vital capacity (FVC) is the amount of air exhale forcefully and quickly after inhaling as much as you can.
- Forced expiratory volume (FEV) is the amount of air expire during the first, second, and third seconds of the FVC test.
- Forced expiratory flow (FEF) is the average rate of flow during the middle half of the FVC test.
- Peak expiratory flow rate (PEFR) is the fastest rate that you can force air out of your lungs.
While the underlying pathophysiology of fibrosis disease is complex, research continues to enhance our understanding of this disease process.However, there is a pressing need to identify and develop new therapies against additional novel targets and pathways.
The most common sources of added sugars include soft drinks, cakes, pies, chocolate, fruit drinks and desserts. Just a single can of cola can contain up to 7 tsps of added sugar, while an average-sized chocolate bar can contain up to 6 tsps.