Respiratory System Diseases Flashcards
Bronchiectasis: description
Obstructive lung disease (no cure)
- Abnormal permanent dilation of large bronchi, resulting in weakness
- Weakened bronchial wall can outpouch- leading to more retained materials and weakening and destruction of the bronchial walls
- Increase in lung infections
Bronchiectasis: causes
1) Localized: from an airway obstruction ie tumors or foreign bodies
2) Generalized:
- congenital abnormalities
- lung infection (TB, fungal, abscesses)
- cystic fibrosis
- exposure to toxic gases
Bronchiectasis: development and progression of bronchial obstruction and infection
1) bronchial obstruction (mucus plug, tumor, etc)
2) Alveoli lose surface tension leading to atelectasis
- less O2 to body, can lead to full lung collapse
3) smooth muscle relaxation and dilation of the airways
- airway remains patent unless there’s an obstruction
4) Infection and Inflammation r/t debris accumulation and bacterial growth
5) impaired ciliary fxn d/t bacterial growth
6) chronic inflammation and development of new infections
Bronchiectasis: manifestations
- Recurrent infection
- Fever
- Coughing
- Producing copious amounts of foul-smelling, purulent sputum
- Hemoptysis (coughing up blood)
- Weight loss
- Anemia (can be a cause or manifestation)
- Marked dyspnea and cyanosis (again, d/t infections)
Cystic Fibrosis: Description
Genetic disorder which affects exocrine glands (systemic)
production of large amounts of thick mucus - secretions from exocrine glands inspissate (thicken) in ducts and act like a sponge => pull in fluid => pulling fluid and electrolytes out of other systems (ie lungs)
Cystic Fibrosis: genetics
autosomal recessive on chromosome #7
- 1/29 carry the gene
- > 200 types of defects (variability and severity of expression varies greatly)
- Not sex-linked
- most fatal genetic disease in Caucasian newborns, Dx usually b/t 1 month and 2 years old
- – median survival = age 38
Cystic Fibrosis: pulmonary effects
- secretions pull fluid out of alveoli, decreasing surface tension and leading to absorption atelectasis
- blocks alveolar ventilation
- chronic inflammation
- edema of capillary-alveolar interface (even when it’s not blocked) d/t fluid in interstitium
- bronchial scarring & fibrosis destroys airways
- reduced lung compliance d/t alveolar rigidity
- barrel chest
- increased risk of pneumonia and chronic bronchitis
- – s. aureus = yellow sputum/ pseudomonas = green
- linked to bronchiectasis - can impact e/o
- incr. incidence of nasal polyps
- digital clubbing
Cystic Fibrosis: GI effects (pancreas)
Pancreatic duct clogs with mucus
- digestive enzymes can’t reach SI
- enzymes begin to digest the pancrease - destroying parenchymal tissue
- Destroys both exocrine and endocrine fxn
===> decr. insulin / DM
Cystic Fibrosis: GI effects (liver and spleen)
Small bile duct obstruction means bile can’t get through
- backs up into liver and destroys it
- biliary cirrhosis
- liver failure
Portal vein moves blood from spleen/GI into liver
- incr. Portal vein pressure = Portal hypertension
- blockage leads to collateral vessel creation which bypass the liver; unfiltered blood in the body
Cystic Fibrosis: GI effects (intestine)
Blocks digestion and absorption
- malabsorption of fats, CHO, proteins
- In infants: failure to thrive d/t poor nutritional status
- poor nutritional status all ages
Cystic Fibrosis: Cardiac effects
Chronic lung disease leads to Right sided HF => Cor pulmonale
Cystic Fibrosis: Reproductive effects
Males: obstructed vas deferens leading to aspermia
Females: thick mucus secretion in cervix blocks entrance - sterility
Cystic Fibrosis: Sweat glands effects
(Normal sweat is isotonic [mostly H2O] because Cl- gets reclaimed and Na+ follows)
Impaired reabsorption of ions in sweat ducts
- 4-5x the concentration of salt in sweat compared to normal
- leads to heat prostration (exhaustion), cellular dehydration, hyponatremia, hypochloremia
Cystic Fibrosis: Dental effects
Salivary ducts close leading to caries and gingivitis
Cystic Fibrosis: Respiratory manifestations
- wheezing
- chronic cough
- dyspnea
- tachypnea
- barrel chest
- cyanosis
- may need daily chest physiotherapy to loosen mucus, lots of coughing, saline…
Cystic Fibrosis: Developmental manifestations
- delayed weight gane
- slowed bone growth
- delayed sexual development
Cystic Fibrosis: GI manifestations
- *Fat is needed for energy and myelination of nerves – especially for babies!!!**
- inspissated meconium in fetal gut
- meconium ileus (emergent, can’t pass meconium)
- GERD
- recurrent abdominal pain
- distention
- no bile = steatorrhea
- vit deficiencies (A,D,E,K)
- rectal prolapse from straining
- inspissated mucofecal masses
- chronic constipation w hemorrhoids
- voracious appetite d/t malabsorption
Cystic Fibrosis: diagnosis
Sweat test - 1month-20 years old - > 60 mEq/L Cl- in sweat Genetic Screening - recombinant DNA - Amniocentisis CXR - hyperinflation - widespread consolidation - fibrotic changes - bronchiectasis - Blebs - RUL (right upper lobe??) involvement (why?) Stool - trypsin and chymotrypsin absent - fecal fat 15-30g (norm = 4g)
Pulmonary Hypertension: description and etiology
(normal lung BP = 25/10)
Increased pressure in pulmonary circulation (>30syst & >12 diast)
Can be primary (idiopathic), genetic (autosomal DOMINANT), or secondary (d/t:
- alveolar hypoventilation
- COPD
- Chronic bronchitis, emphysema
- CF
- other causes)
*she also said something about low pH leads to vasoconstriction which leads to PulmHTN…
Pulmonary Hypertension: Signs and Symptoms
- murmurs & S3/S4
- dyspnea
- JVD
- chest pain
- palpitations
- dizziness
- syncope
- cough
- lower extremity dependent edema
Pulmonary Hypertension: Diagnosis
- EKG: R ventricular hypertrophy and R axis deviation
- PFT & ABG: arterial hypoxemia, reduced diff. capacity
- V/Q scan: to rule out pulmonary embolism
- CXR: enlarged central pulmonary arteries
- Echo doppler: R ventr enlargement and overload
- ***Cardiac cath: R cath to measure pressure
- angiography as indicated by V/Q
Cor Pulmonale: description
R ventricular dysfunction caused by pulmonary circulation chaos
- Acute: pulmonary embolism or other trigger causes pulmonary HTN => Acute dilation of RV
- **the right ventricle can EXPLODE so deal with this!!!!
- Chronic: vascular or pulmonary parenchymal disease leads to hypertrophy and dilation of the RV
Cor Pulmonale: Manifestations
Lung manifestations:
cough with or without sputum, dyspnea w/ exertion, rhonchi and wheezing
RHF manifestations:
S3 and tricuspid regurgitation, atypical chest pain,
JVD, hepatomegaly, ascites (backwards)
Cyanosis
Cor Pulmonale: Causes
Can be restrictive or obstructive Pulmonary Tree - COPD - Pulmonary fibrosis (restrictive) - Pulmonary resection - Granulomatous disease (sarcoidosis, RA, SLE), - Bronchiectasis - CF - Malignancy
Pulmonary Vessels
- Primary Pulm HTN
- Pulm Embolism
- Pulm vascular disease 2ndary to syst illness
- IV drug abuse
Thoracic Cage
- obesity
- kyphoscoliosis
- neuromusc disease (ie MS, MG)
- sleep apnea
Chronic hypoxia at high altitudes