CF + ARDS highlights Flashcards
1) Cystic fibrosis(CF), also calledmucoviscidosis, is genetic in what way?
2) What gene(s) is/ are affected?
1) Autosomal recessive genetic disease
2) CF transmembrane conductance regulator (CFTR) gene mutation
What are 4 pulmonary manifestations of CF?
1) Recurrent bacterial infection
2) Bronchiectasis
3) Pneumothorax
4) Pulmonary arterial hypertension
What other organs does CF affect?
Exocrine functs. of: lungs,liver,pancreas,small bowel,sweat glands,and the male genital system
What is pathognomonic for CF?
+ sputum culture for Burkholderia cepacia
CF Imaging? (2 things)
1) CXR
2) HRCT: Increased diameter of airways and pulmonary arteries
True or false: sweat chloride test is the only test for CF
False, more now (Molecular testing, NPD, etC)
1) What is the primary test for CF?
2) What is an abnormal value for this test?
3) What is another test?
1) Sweat Chloride Test
2) > 60 mmol/L = CF
3) Molecular
CF foundation (www.cff.org) clinical practice guidelines recommend use of _______________ to improve lung function and reduce exacerbations
Cystic fibrosis ________ referrals are encouraged and follow patients at least quarterly
1) chronic medications
2) clinic
What should you avoid unless clear asthma symptoms in CF?
ICS
Both transplanted and non-transplanted CF patients are at increased risk of some malignancies, like what?
1) Gastrointestinal malignancy: esophageal,gastric,small bowel, andcolorectal cancer gallbladderand extrahepaticbiliary tree lymphoma/leukemia
2) Testicular cancer
ARDS manifests what that presents as rapidly progressive dyspnea, tachypnea, & hypoxemia?
Acute, diffuse, alveolar injury
Give the OLDCARTS for ARDS
CC: acute &/or worsening dyspnea
Onset, duration, timing – acute new or worsening respiratory symptoms within 1 week of a known insult
Location: pulmonary
Characteristics – progressive dyspnea leading to respiratory failure
Aggravating – pulmonary or extrapulmonary insult
Alleviating – none (mechanical ventilation)
Radiation – hypoxia and respiratory failure
Associated symptoms – respiratory failure leading to hypoxia, MS changes, and cardiovascular collapse
What is a key thing you’ll see on an ARDS exam?
Progressive dyspnea (leading to hypoxia despite oxygen supplementation)
ARDS diagnosis: What values?
60/0.35=171
60/.60 = 100
(Ex: PAo2 = 60; FIO2 = 21% 60/0.21=285)
What is a distinguishing feature that makes you suspect ARDS complicating underlying conditions?
Severe hypoxia does not improve as expected with increasing FiO2%
ARDS Dx:
Ratio of PaO2:FiO2 of _______ on ventilator settings that include positive end-expiratory pressure (PEEP) or continuous positive airway pressure (CPAP) ≥5 cm H2O
< 300
Differentiate uncomplicated pneumonia from pneumonia with ARDS
Uncomplicated means hypoxia should respond to oxygen administration
