Term
| What are the pathological traits of Cystic Fibrosis? |
|
Definition
| Acinar tissue of the pancreas is replaced by epithelium lined cysts containing concretions, lung shows bronchitis, broncheictasis, pulmonary abscesses, etc. |
|
|
Term
| What is the most common lethal genetic mutation? |
|
Definition
| Cystic Fibrosis, a mutation in the CFTR gene causing defective epithelial cell ion transport |
|
|
Term
| How is Cystic Fibrosis inherited? |
|
Definition
|
|
Term
| What is the median age of survival for people with Cystic Fibrosis? |
|
Definition
|
|
Term
| What other diseases and conditions are associated with cystic fibrosis? |
|
Definition
| Delayed puberty, impaired fertility, diabetes milletus, hyponatremia, anemia, digital clubbing, coagulation defects |
|
|
Term
| What is the pathophysiology of bronchiectasis? |
|
Definition
| Genetic defect -> altered bronchial dynamics -> poor mucous clearance -> chronic infection -> bronchial wall inflammation and destruction -> back to altered bronchial dynamics |
|
|
Term
| What are the clinical features of Bronchiectasis? |
|
Definition
| Dilated bronchioles, loss of normal tapering airway, thickened airway walls, varicose constriction of airway, ballooned cysts at end of bronchus, causes chronic cough, mucopurulent secretions, dyspnea, hemoptysis, fever, fatigue, pleuritic chest pain, weight loss |
|
|
Term
| What are the goals of cystic fibrosis therapy? |
|
Definition
| Decrease irreversible lung damage, infections, loss of time, respiratory symptoms, increase lung function, improve quality of life |
|
|
Term
| How is the forced expiration technique performed? |
|
Definition
| A few forced expirations followed by relaxed breathing |
|
|
Term
| How does a flutter device function? |
|
Definition
| Patient breathes out into device, causing airway walls to vibrate and loosen mucus buildup, huff maneuver then used to remove mucus |
|
|
Term
| How are lung transplants beneficial to CF patients? |
|
Definition
| Improves quality of life and extends life expectancy |
|
|
Term
| How is sleep associated with ventilation in children? |
|
Definition
| Sleep impairs ventilation in all children |
|
|
Term
| What are the phases of sleep? |
|
Definition
| 1, 2, 3, 4, and REM sleep. Takes about 90-110 min to cycle |
|
|
Term
|
Definition
| Disorder of breathing during sleep characterized by prolonged partial and / or intermittent complete upper airway obstruction that disrupts normal ventilation and patterns during sleep. |
|
|
Term
| What is the duration of episodes of sleep apnea? |
|
Definition
| Adults: >10 sec, Children: 2.5 missed breaths |
|
|
Term
| Obstructive sleep apnea is most prevalent in which stage of sleep? |
|
Definition
|
|
Term
| What are some etiological factors contributing to the prevalence of sleep apnea? |
|
Definition
| Obesity, craniofascial disorders, micrognathia. Others exist |
|
|
Term
| In what stage of sleep does Somnambulism (sleep walking occur)? |
|
Definition
|
|
