other pulmonary diseases

cystic fibrosis

-autosomal recessive disease

-chronic sinopulmonary infections, malabsorption, and nutritional abnormalities

-most common lethal disease

-lung disease= major cause of morbidity

cystic fibrosis patho

-single gene defect on chromosome 7 encodes for CTRF protein > absence of this protein leads to altering salt and water across cell membrane > thick secretions altering host defense lungs

cystic fibrosis etiology/RF

-genetic >2000 CF mutations

-deleted chromosome 7

-caucasians

-family hx

-jewish

cystic fibrosis sx

-neonatal: meconium ileus, failure to thrive

-HEENT/pulm: chronic productive cough, recurrent respiratory infection, crackles/wheeze, digital clubbing

-GI: steatorrhea (fat in stool), abdominal pain, constipation, rectal prolapse

-poor weight gain, delayed puberty

-salty tasting skin, nasal polyps, infertility, poor weight gain

-hepatomegaly, distension, palpable fecal mass

cystic fibrosis exacerbations

-acute worsening of respiratory sx

-leading cause of mortality

-sx: increased cough, change in sputum, increased dyspnea, new hemoptysis, fever, fatigue, malaise, dullness of percussion

cystic fibrosis dx

-prenatal screening

-sweat chloride test= confirmatory

-genetic testing: CTRF gene

fecal elastase testing: <200 indicates pancreatic insufficiency

-CXR: eventually shows progressive bronchiectasis hyperinflation

-high resolution chest CT: better detection of ling

-PFT: decline in FEV1

cystic fibrosis tx

-referral to a regional cystic fibrosis center

-airway clearance: chest physiotherapy, chest oscillation, posititive expiratory pressure device

-nutritional: high calorie, fat diet, fat soluble vitamins

-lung transplant= only definitive tx

-CFTR modulations, mucolytics

-must be up to date on vaccinations

-abx for exacerbations: MRSA and pseudomonas empiric tx and then narrow down based on sputum culture

cystic fibrosis complications

-pseudomonas aeruginosa infection becomes most predominant pathogen

bronchiectasis

-abnormal and PERMANENT, widening and thickening of airways due to infection and inflammation

-women and elderly

bronchiectasis patho

-initial injury > impaired clearance > infection > inflammation > structural damage and pathologic changes including bronchial wall thickening, loss of bronchial tapering, cystic changes leading to

-progressive airway dilation

-loss of ciliary function

-chronic bacterial colonization (pseudomonas aeruginosa)

-airway hyperresponsiveness and airflow obstruction

bronchiectasis etiology/RF

-post infectious

-genetic

-immunodeficiency

-inflammatory

-aspiration

-women

bronchiectasis sx

-daily productive cough, dyspnea, fatigue

-sputum: purulent often large volume

-recurrent respiratory infection

-persistant wet crackles= hallmark

-digital clubbing

-cyanosis, use of accessory muscles

bronchiectasis dx

-high resolution chest CT: dilated, tortus airways, tram tracks, signet ring sign, tree in bid pattern, cystic damage

-bronchoscopy: elvaulate hemoptysis, remove secretions, rule out

bronchiectasis tx

-manage underlying condition

-airwary clearance, pulmonary rehab, nutritional support, vaccination

-surgery: lobectomy for localized disease

-exacerbation: augmentin

-chronic suppresive therapy: macrolide

-mucolytics: hypertonic saline, dornase alfa

-bronchodilators, inhaled corticosteriods

obstructive sleep apnea (OSA)

-most common sleep related disorder

-50-70 yo

-male

OSA patho

repetitive complete apnea or partial upper airway collapse during sleep > hypoxemia, hypercapnia and arousal from sleep > sympathetic nervous system activation ``

OSA etiology/RF

-obesity BMI >30

-anatomical factors

- >40

-male

-postmenopausal

-family hx

-smoking, alcohol use, sedative mediaction

-large neck circumference >17in in men and 16 in women

OSA sx

-loud, habitual snoring with witnesses apneic episodes

-excessive daytime sleepiness

-unrefreshing sleep

-morning headache

-obesity, large neck circumference

-hypertension in 50-60% of pt

-mallampati score III-IV

OSA dx

-screening questionnaires: STOP-BANG

-snoring, tired, observed apnea, pressure, BMI >35, age >50, neck size, gender

-gold standard= overnight polysomnography (PSG) in sleep laboratory

-AHI (number of apneas and hypopnea per hour): mild, mod, severe

-if abnormal most pt undergo pap titration

OSA tx

-weight loss

-first line: CPAP for >4hrs (reduced risk of heart disease and stoke

-avoid sedating medications and agents like alcohol

-Surgical interventions: uvulopalatopharyngoplasty, genioglossus

advancement, maxillomandibular advancement

-treat underlying conditions

obesity hypoventilation syndrome (OHS)

-chronic hypercapnic respiratory failure in obese pt

-present in most pt with OSA

-aka pickwickian syndrome

OHS patho

-obesity induced restrictive lung disease with decreased lung volume + impaired central respiratory drive and blunt hypercapnic response > chronic hypoventilation leading to compensated acidosis

-pulmonary hypertension and cor pulmonale

OHS etiology/RF

-severe obesity (BMI >30)

-upper airway obstruction

-male

-advanced age

-hypothyroidism

-sedative use

OHS sx

-exessive daytime sleeping, morning headache, dysonea, orthopnea

-sx of OSA

-morbid obesity

-cyanosis

-signs of right heart faulure

-more respiratory sx than OSA

OHS dx

-BMI >30

-SpO2 <94%

-sx of pulmonary hypertension

-facial plethora

-diagnostic criteria: awake hypoventilation in obese pt in absence of other alveolar hypoventilation

-ABG: PaCO2 >40 while awake and stable

-CXR: cardiomegaly

-PFT: restrictive pattern

OHS tx

-weight loss= gold standard

=CPAP is OHS present, BiPAP is hypoventilation

-sleep positioning

-pulmonary rehab

-oxygen therapy

-avoid sedating meds

pulmonary hypertension

-hypertension in pulmonary arteries

-mPAP >20

-progressive disease

pulmonary hypertension patho

-increased pulmonary vascular resistance due to vasocontriction, remodeling, thrombosis > endothelial dysfunction with impaired vasodilation > vascular remodeling with smooth muscle proliferation

-results in por pulmonale

pulmonary hypertension etiology/RF

-group 3: lung disease/hypoxia

-female

-family hx

-connective tissue disease

-portal hypertension

-HIV infection

-chronic lung disease

-chronic hypertension

pulmonary hypertension sx

-progressice dyspnea on exertion (most common)

-fatigue, weakness, exercise intolerance

-chest pain

-dry cough

-loud foxed S2 with prominent S2 component

-right ventricular heave

-jugular venous distension, hepatojular reflux, peripheral edema

-cyanosis, clubbing

pulmonary hypertension > cor pulmonale

-when right sided heart failure is caused by lung condition

-long term high BP in the arteries of the lung and right ventricle of heart can lead to cor pulmonale

pulmonary hypertension dx

-echocardiogram: right ventricular systolic pressure

-gold standard: right heart catheterization, mean arterial pressure >20= diagnostic

-chest CT, VQ scan, seriology, genetic testing

pulmonary hypertension tx

-treat underlying lung disease

-diuretics and salt restriction

-pulm rehab

-lung transplant

-group 1: phosphodiesterase-5 inhibitor= sildenafil, tadalafil

foreign body (airway)

-leading cause of death <4yo

-less common in adults, usually with altered mental status

-nuts, seeds, small toys, food, dental appliances (adults)

foreign body sx

-even may or may not be witnesses

-acute: chocking, gagging, stridor, unilateral wheeze, cant speak or cry, cyanosis

-chronic: persistant cough, reccurent pneumonia, unilateral wheeze, decreased exercise tolerance, chest pain

foreign body dx

-witness choking episode, sudden onset sx

-unilateral wheeze

-CXR: object is visible, hyperinflation, left mediastinal shift, atelectasis, air trapping

-CT chest: more sensitive

-flexical bronchoscope= diagnostic

foreign body tx

-must be removed, urgency depends on location and presentation

-mainstay= flexible bronchoscopy

-complete obstruction: immediate intervention

-partial obstruction: avoid blind finger sweeps

-rigid bronchoscopy: larger objects

-surgical intervention: thoractomy, lobectomy

-post removal care: abc if secondary infection, bronchodilators