Airway disease Flashcards
What are the features of primary ciliary dyskinesia?
Congenital abnormality within the axoneme of cilia. Impaired muciliary clearance, considerable variation. Median age at Dx is 22y. Recurrent sinusitis, pulmonary infections with bronchiectasis, and otitis media with S aureus/P aeruginosa.
Triad of situs inversus, bronchiectasis, and infertility = Kartagener syndrome.
Diagnose with nasal NO with confirmatory testing with high speed video microscopy of nasal ciliary brushings. Genetic testing with DNAH5 or DNAI1 mutation
What are the features of bronchopulmonary dysplasia?
The need for oxygen for at least 28 days after birth (common in premature babies before 28w or under 1500g). Have increase respiratory symptoms, reduced FEV1 and FEV1/FVC ratio, often have fixed airway obstruction. All due to disrupted pulmonary development. Higher prevalence of exercised induced dyspnea. CT shows air trapping, bronchial wall thickening, multifocal emphysema
What are the feature of alpha-1 antitrypsin deficiency?
autosomal codominant inherited disorder leading to early onset COPD, commonly in their 40s. Bullous changes are often present symmetric, and basilar.
What are the features of tracheobronchopathia osteochondroplastica?
Rare, benign tracheobronchial disease. submucosal osteocartilaginous nodules that project into the lumen of the large airways. Usually anterior and lateral aspect of inner tracheal and proximal bronchial wall sparing posterior membrane. May have DOE, recurrent infections, wheezing, hemoptysis. severe cases with obstruction requires bronchoscopic dilation.
What are the features of relapsing polychondritis?
Progressive inflammation and destruction of cartilaginous structures. Airway involvement in 50% of cases. Dyspnea, cough, wheeze, stridor, hoarseness, aphonia. Recurrent pneumonia is frequent cause of death. Steroids and steroid-sparing agents can healp in earlier stages of disease. Advanced cases require balloon dilation, tracheobronchial stents, tracheostomy, or reconstructions.
What are the features of tracheobronchial amyloidosis?
5th-6th decade, multifocal submucosal plaques of amyloid deposits distributed in a diffuse way along the airway wall. Can result in airway calcifications and airway narrowing. Circumferential. Dyspnea, cough, hoarseness, wheezing, hemoptysis, stridor. Requires bronchoscopic techniques to open airways
What are the features of granulomatosis with polyangiitis (GPA)?
Dyspnea, hoarseness, stridor. Usually focal (2-4cm segments most commonly), subglottic portion of the trachea. Tracheal wall thickening usually circumferential and can be smooth or nodular, posterior membrane always involved. Immunosuppressive therapy for systemic GPA, surgery for subglottic stenosis.
What are the features of constrictive bronchiolitis
Dyspnea and airflow limitation not reversible with inhaled bronchodilators that is associated with small airways injury. Mosaic attenuation on CT
What are the consequences of de-escalation of triple therapy in COPD patients?
Reduction in lung function. No increased AECOPD in a non-frequently exacerbating population or decrease in QOL or mortality
The syndrome of MAC in elderly thin women with pectus excavatum and scoliosis has what associations?
Hormonal and immune abnormalities.
What does a pulmonary contusion look like on US
consolidation pattern or scattered B-line intermixed with A-lines
How can using supplemental oxygen during exercise benefit the patient?
Increased exercise tolerance while using oxygen. No proven benefit for hospitalization, QOL, dyspnea.
How are central airway obstructions from tracheal stenosis treated?
Balloon dilation, stenting, laser resection. Mitomycin C and/or steroids applied locally to prevent regrowth of scar tissue. Surgery for failed bronchoscopic interventions
What are the features of CVID?
primary immunodeficiancy with impaired Bcell differentiation Average age of diagnosis 20-40yrs. Often chronic lung disease (bronchiectasis), hematologic disease, poor response to vaccination, frequent infections with encapsulated organisms, inflammatory bowel disease, RA, Grave’s
How should patients admitted for AECOPD with hypoxemia and hypercapneia managed outpatient?
Supplemental oxygen and nocturnal NIV for at least 6 hrs to reduce nocturnal hypoventilation, increase QOL and reduce AECOPD frequency
