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Abstract
Lung disease is the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Although CF lung disease is primarily an infectious disorder, the associated inflammation is both intense and ineffective at clearing pathogens. Persistent high-intensity inflammation leads to permanent structural damage of the CF airways and impaired lung function that eventually results in respiratory failure and death. Several defective inflammatory responses have been linked to cystic fibrosis transmembrane conductance regulator (CFTR) deficiency including innate and acquired immunity dysregulation, cell membrane lipid abnormalities, various transcription factor signaling defects, as well as altered kinase and toll-like receptor responses. The inflammation of the CF lung is dominated by neutrophils that release oxidants and proteases, particularly elastase. Neutrophil elastase in the CF airway secretions precedes the appearance of bronchiectasis, and correlates with lung function deterioration and respiratory exacerbations. Anti-inflammatory therapies are therefore of particular interest for CF lung disease but must be carefully studied to avoid suppressing critical elements of the inflammatory response and thus worsening infection. This review examines the role of inflammation in the pathogenesis of CF lung disease, summarizes the results of past clinical trials and explores promising new anti-inflammatory options.
Keywords
Lung inflammation
Cystic fibrosis
Neutrophils
Anti-inflammatory therapies
Bronchiectasis
Mucosal immunity
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Copyright © 2015 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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Respiratory Therapy
| What are the major pathologic or structural changes associated with CF? | (1)Excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree (2)Partial bronchial obstruction (mucus plugging) (3)Hyperinflation of the alveoli (4)Total bronchial obstruction (mucus plugging)(5) Atelectasis |
| How many people does CF affect in the U.S. alone? | 30,000 |
| What is the most common inherited disorder in childhood? | CF |
| How does a person develop CF? | Both parents must be carriers of the CF gene for any child to be born with CF |
| If both parents carry the CF gene, what are the odds their offspring will develop CF? | 1:4 |
| Which organs are most severely affected? | Sweat glands, pancreas, and lungs |
| How do you diagnose CF? | Sweat chloride test |
| What percent of patients are diagnosed with CF by performing the sweat chloride test? | 98% |
| What does the sweat chloride test measure? | The amount of sodium and chloride in the patient's sweat |
| In children, a sweat chloride concentration greater than ____ is required to confirm CF diagnosis. | 60 mEq/L |
| In adults, a sweat chloride concentration greater than ____ is required to confirm CF diagnosis. | 80 mEq/L |
| When would a sweat test be considered for a child? | If the child has recurrent pulmonary infections or failure of infant to grow as rapidly as expected |
| At times, mucus can obstruct the airway and cause what? | Atelectasis, pneumonia, or lung abscesses |
| Patients with CF have bronchiolits and bronchiectasis which produce what? | Copius amounts of thick mucinous secretions |
| How can we minimize the decline in pulmonary function and improve their quality of life? | Help CF patients achieve optimal nutritional helath |
| What are the goals of nutrition management in CF? | (1)Maximize a nutritional intake through calorically dense foods (2)Balance intake with pancreatic enzymes to maximize absorption (3)Provide a nutrition plan that meets the changing clinical and psychosocial needs of the patient |
| Partial bronchial obstruction leads to what? | Overdistention of the alveoli |
| Total bronchial obstruction leads to what? | Patchy areas of atelectasis |
| The abdundance of stagnant mucus in the tracheobronchial tree serves as an excellent culture medium for bacteria, particularly: | Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa |
| Common symptoms of CF include: | Vitamin A,D,E and K defifiencis, diarrhea, malnutrition, and weight loss. Some infants develop a blockage of the intestines shortly after birth (meconium ileus). Most men are infertile due to missing or underdeveloped vas deferens. |
| Immunoreactive trypsinogen test (IRT) may be ordered as an initial test for: | (1)Babies who are not creating enough sweat to perform a sweat test, or (2)infants with meconium ileus (no stools in the first 24 to 48 hours of life) |
| Patients with CF have ____ blood levels of IRT. | Elevated |
| What do two positive IRT tests indicate? | CF, abnormal pancreatic enzyme production, pancreatitis, or pancreatic cancer |
| What does the stool fecal fat test measure? | Measures the amount of fat in the infant's stool and the percentage of dietary fat that is not absorbed by the body |
| The stool fecal fat test is used to evaluate what? | Evaluate how the liver, gallbladder, pancreas, and intestines are functioning |
| Under normal conditions the fat absorption is less than ____ per ____ hours. | Less than 7g of fat per 24 hours |
| An elevated stool fecal fat test (decreased fat absorption) is associated with what? | A variety of disorders, including CF |