M. 1: Respiratory Flashcards
Cystic Fibrosis Patho/Diagnostics
Sodium/chloride channel dysfunction related to autosomal recessive gene mutation (CFTR gene and most often on phenylalanine position 508). + newborn screen > IRT protocol x2. pilocarpine sweat test with >60 x2 being positive and 30-60 intermediate. Genetic tests can diagnose, nasal test can confirm. Patho: lack of chloride movement traps it on the sweat and also inside cells > increased intracellular Na > lack of water following > thick mucus and plugging of organs and cells. decreased mucociliary movement in the lungs causes chronic infections and inflammations.
CF signs/symptoms
Severe lung disease d/t chronic inflammation, thick mucus production with cough, airway obstruction, nasal polyps, pneumonias, clubbing, meconium ileus, pancreatic insufficiency, rectal prolapse, fatty stools, poor muscle mass, delayed maturation, (do poorly with soy based formula), can cause intussusception, hepatic steatosis, biliary fibrosis, GERD, fat soluble vitamins deficient, biliary cirrhosis. risk for DM so annual glucose screening. delayed sexual development with male sterility and female hypofertility. lack of vD > osteoporosis. hypochloremic acidosis.
CF management
CPT, bronchodilators (albuterol, anticholinergics), mucus breakers (mucomyst, 3%), O2 as needed, CFTR modulators (ivacaftor, lumacaftor), occasionally high dose ibuprofen and azithromycin, replacing exogenous pancreatic enzyme, fat-soluble vitamin soluble, hydration/laxatives if needed,
Asthma
remodeling pulmonary disease d/t inflammation, airway hyper responsiveness, and airway remodeling. s/s coughing, wheezing, chest tightness, respiratory distress, breathlessness. path: eosinophils, ig-E mediated, degranulated mast cells, lymphocytes, goblet cell alteration, cytokines, TLR/transcription factors > airway remodeling. obstruction is reversible but remodeling can lead to fibrosis that is chronic. can be d/t genetics too. Asthma risk is also related to allergen sensitization.
Asthma classification
intermittent: SABA <2 days/wek, brief exacerbations, nighttime s/s <2x/month, FEV1 >80%. Mild persistent: s/s or SABA >2x/week but not daily; nighttime s/s 3-4x/month, FEV1>80%. Moderate persistent: daily s/s and SABA, frequent exacerbations, nighttime s/s >1x/week not daily, fev1 60-80%. Severe persistent constant s/s, SABA multiple times a day, nightly s/s daily, FEV1 <60%
Asthma exacerbations; allergic
early asthmatic response EAR has bronchospasm/bronchoconstriction. various triggers, including at times aspirin. starts within 30min of mast cell activation, resolves within 1 hour of removal from offending allergen. use bronchodilator. Late phase response is 6-26 hours after exposure. needs steroid. Exercise-induced EIB is a thing.
Asthma diagnosis
delayed until 4years/age (reactive airway, wheezy bronchitis, recurrent bronchitis). Atopy is strongest risk factor for asthma. Allergens: mites, cockroaches, molds, dander, saliva, airborne pollens, food allergies. Screen with PFTs, FEV1, standard questionnaires. Peak flow, spirometry q1-2 years. can do bronchial provocation test with methacholine or exercise (20% reduction post med or 10% reduction post exercise is hyper responsive +). Biomarkers like iNOef.
Asthma s/s
family hx, associated conditions, chest tightness/dyspnea, cough wheezing particularly at night/early morning or SOB with exercise; allergen related; recurrent bronchitis. Wheezing, coughing, prolonged expiratory phase, diminished breath sounds, altered LOC, WOB, can have curschmann spirals (mucus casts/plugs). may have charcot-leyden crystals (needle shaped from breakdown of eosinophils). check sats, CBC, can do CXR but not routine. PFT gold standard for diagnosing with spirometry >5 years/age. look at FEV1; reversibility with bronchodilator is an increase of 12% or >100 from baseline. >75% normal, 60-75% mild, 50-59% moderate, <49% severe. PEF is by personal best, with >80 good, 50-70 yellow, <50 red.
Asthma treatment
bronchodilators, SABA, LABA (>4 years old), anticholinergics, cromolyn, anti IgE monoclonal antibody omalizumab. keep vaccines uptodate, do action plan. avoid triggers. coping mechs. inhaled glucocorticoids. PO steroids if major flare up. use MDI with spacer for children. rinse mouth after. for EIB: warm up for 5-10 min, SABA up to 20 min prior to exercise, cromolyn. if SABA daily, add daily scheduled meds. use scarf or mask in cold weather. high risk sports are high endurance sports, long distance running, track and field. stepwise approach for scheduled.
Asthma stepwise approach
Intermittent: SABA prn. step 2: low dose ICS or alternates; step 3: low dose ICS + LABA or medium dosed ICS or alternatives. step 4: medium dose ICS + LABA or alternates. step 5: high dose ICS + LABA. step 6: high dose ICS + LABA + PO steroids. step up if needed, step down if well controlled 3 months.
asthma exacerbation management
SABA 2-6 puffs q20 min 3x via MDI with spacer or a single neb. if good response, continue q3-4hours for 1-2 days with 3 day course PO steroids. incomplete response to initial (PEF or FEV1 40-69 or return of s/s in 4 hours) do continuing B2 agonists and adding PO steroids. if marked distress, repeat SABA immediately > ED or 911. if exacerbation more than once q4-6 weeks, eval compliance. ED tx: mag, terbutaline, theophylline, ketamine, atrovent, epinephrine, heliox.
Pectus excavatum
pigeon chest; treated post growth spurt so 14+, minimal surgery now. can have s/s chest pain, SOB, can be midline or not
