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Restrictive Diseases

Definitions, etiology, Symptoms, Treatment

What is interstitial Pulmonary Fibrosis (IPF)? Excessive formation of connective (scar) tissue in the process of repairing chronic or acute tissue injury.
Etiology of IPF. An immune reaction to inhaled substances like, Chlorine, Ammonia, PVC, smoke inhalation, Radiation. May also result from complications of other pulmonary diseases such as TB, unresolved pneumonia,fungal infections,& diseases of unknown etiology.
Pathophysiology of IPF. Cellular infiltration, acute vasculitis, scar tissue, that can't be reversed.
What is Pnuemoconiosis? A group of occupational diseases causing chronic inflammation of the lungs and interstitial fibrosis due to inhalation of dust or chemical fumes.
Types and causes of Pneumoconioses. Silicosis(Silica dust),Farmer's lung(moldy hay), Silo-filler disease(Nitrogen dioxide), Coal miners pneumonia(coal dust), Asbestosis(Asbestos), Siderosis(Iron dust), Aluminosis(Aluminum)
Symptoms of IPF. Progressive dyspnea, on exertion in the beginning stages then at rest as the disease progresses (PRIMARY SYMPTOM), nonproductive cough, hemoptysis, hypoxemia,chest pain.
Physical examination findings for IPF. Increased WOB, tachypnea w/ shallow VT, accessory muscle use, breath sounds(diffused, dry, crackling rales) cyanosis, restricted chest wall movement, later stages show clubbing and cor pulmonale.
Chest x-ray findings for IPF. elevated diaphragm, small lung, large heart, honeycomb appearance from fibrotic changes.
Diagnostics used to confirm IPF. Bronchoscopy, biopsy, PFT, history.
Treatment of IPF. No known cure. Limit exposure to causative, corticosteroids, O2 therapy, antibiotics, treat symptoms and complications, pulmonary hygiene, bronchodilators.
Perimeters for mechanical ventilation in IPF. Vt = 8 to 12ml/kg b/c trying to compensate for diminished lung volume(5-8ml/kg is the norm) RR = 12-20 b/min I time = 1.0sec
What types of lung diseases are restrictive? Interstitial pulmonary fibrosis(IPF), Pleural effusion, Pneumothorax, Pulmonary edema(cardiogenic and non-cardiogenic), ARDS, Pneumonia, Pulmonary embolism, Postoperative atelectasis.
What is pleural effusion (hemothorax)? Presence of fluid in the pleural space causing compression of the lung on the affected side. If it is large enough the mediastinum will shift. Is is gravity dependent and may shift with changing of positions.
Etiology of pleural effusion. Inflammation, malignancy, CHF(most common), infection pulmonary infarction, and trauma.
Diagnostics used to confirm pleural effusion. Chest x-ray.
Types of pleural effusions. Hydrothorax, empyema, hemothorax, chylothorax, fibrothorax.
What is hydrothorax? Noninflammatory accumulation of serous fluid in one or both pleural cavities.
What is empyema? Effusion consisting entirely of pus caused by a bacterial infection.
What is hemothorax? Frank blood or fluid accumulation in the pleural cavity caused by, trauma, malignancy, or ruptured blood vessels.
What is chylothorax? Chyle from the thoracic duct into the pleural space, usually caused trauma or tumor.
Symptoms of pleural effusion. Dyspnea, tachypnea, chest pain, cough, hemoptysis, hypoxemia. Breath sounds are decreased or crackles over ther effected area.
X-ray findings. location of effusion w/radiopaque homogeneous mass,obliteration of costophrenic angle, mediastinal shift away from affected area, possible atelectasis.
Treatment for pleural effusion. Thoracentesis, chest tube drainage, O2, need to treat underlying cause, symptoms, and complications.
What is a pneumothorax? Presence of air in the intrapleural space or within other areas of the thorax, can be spontaneous or traumatic. Pressure increases and decompresses lung tissue, resulting in atelectasis and ventilation becomes decreased on the affected side.
Two types of pneumothorax. Open and tension.
Criteria for an open pneumothorax. Trauma, penetrating injury, lung puncture, rib fracture, rupture of chest wall.
Criteria for a tension pneumothorax (spontaneous). Acute medical emergency, air leaks into pleural space but can't escape, not from an injury, should see a shift in mediastinal. Increase in alveolar and intraplural pressure.
Characteristics of an open pneumothorax. Pressure does not build up, gas is allowed to move freely in and our of the pleural space
Characteristics of a tension pneumothorax. Gas allowed in but not out, significant increases in pressure.
Symptoms of a pneumothorax. *sudden chest pain *tachypnea *tachycardia *hypoxemia *diaphoresis *temp *anxiety *absent breath sounds on affected side *trachea & mediastinum shift toward unaffected side
X-ray findings for a pneumothorax. Hyperlucency and absent vascular markings at location, mediastinum shift.
Treatment for pneumothorax. Place in Fowler's position, O2 therapy w/ nasal cannula (unless contraindicated), chest tube w/ chest drain system, thoracentesis, IPPB to reinflate the lung, and if respiratory failure place on ventilation.
What is a chest tube? Drainage system used to remove air or fluid from the pleural space, allows negative intrapleural pressure to be re-established.
What are chest tubes used to resolve? *Pneumothorax(air) *hemothorax(blood) *pleural effusion *empyema(purulent fluid) *cardiac tamponade following open heart surgery.
What do chest tubes comprise of? *Sterile flexible catheter(sizes 7-40 French) *radiopaque stripe
Chest tube placement. Fowler's/semi-Fowler's position, 2in. into pleura cavity. Pneumothorax, placed @ apex of lung(2nd/3rd intercostal space) hemothorax, placed @ base of lung slightly lateral(6th/8th intercostal space)w/both place 2 tubes & connect w/ a "Y" connector.
What is pulmonary edema? Accumulation of excessive fluid in the alveloi and interstitium.
Causes of pulmonary edema. *CHF *renal failure *heart disease *myocardial infarction *sepsis *ARDS *pneumonia *near-drowning *embolism *O2 toxicity *smoke inhalation
Signs of pulmonary edema. *tachypnea *labored shallow breathing *cyanosis *pedal edema *neck vein distension *SOB *HR and BP are increased *respiratory acidosis *profuse diaphoresis *cough w/ pink frothy secretions *hyperventilation *breath sounds: rales.
X-ray findings for pulmonary edema. Prominent vascular markings, diffuse fluffy infiltrates w/ butterfly pattern and possible cardiomegaly.
Cardiogenic Pulmonary edema process. Alveolar edema; surfactant flushing which increases surface tension (alveolar collapse) a V/Q occurs (intrapulmonary shunting)and hypoxemia happens.
Treatment for cardiogenic pulmonary edema. Lasix -to decrease fluid, Morphine -vasodilation to decrease vascular resistance, Digitalis -increase contractility of heart and increase CO, O2 therapy, CPAP/BiPAP, ventilation w/PEEP
Non-cardiogenic pulmonary edema. Epithelial damage of alveolar allows fluid to enter the alveoli; CNS trauma, drug OD, high altitudes, re-expansion(rapid removal of pleural effusion fluid, >1000ml, may produce edema) Some consider to be early form of ARDS.
Diagnosis of Pulmonary edema. Wet bubbly rales in lung fields, pink frothy secretions.
What is ARDS? Severe pulmonary congestion. Reaction to the respiratory tract to high levels of physiologic stress.
Clinical signs of ARDS. REFRACTORY HYPOXEMIA(main sign), decreased lung compliance, decreased FRC, atelectasis, shunting, PE, PF, respiratory failure.
Chest x-ray findings for ARDS. Diffused alveolar infiltrated throughout lung "honeycomb", "ground glass" appearance.
Symptoms of ARDS. dyspnea, tachypnea, tachycardia, cyanosis, hypoxemia, resp. distress. Breath sounds: rales and/or wheezes, cough, SOB, increase WOB, decrease compliance and FRC.
Treatment of ARDS. Treat underlying cause, ventilation(IS,IPPB,Ventilator w/PEEP),low O2 therapy, steroids, antibiotics, diuretics.
What is pneumonia? Inflammation, infection, and consolidation of the lungs
Types of pneumonia. Viral, bacterial(lobar), fungal(Candida), immune-suppressed(pneumocystis Carinii), aspiration, broncho.
Signs of pneumonia. Cough w/sputum, dyspnea, chest pain, chills, fever, hypoxemia. Breath sounds:rhonchi, crackles, wheezes. At first it mimics a cold or flu.
X-ray findings for pneumonia. Reveals location and extent of infection, radiopacity, infiltrated, consolidation.
Why take a sputum culture for pneumonia? It can reveal type of pulmonary infection.
Etiology of lobar pneumonia. Usually caused by Streptococcus pneumoniae,localized to 1 or more lobes, rusty colored sputum, empyema, O2 problems like shunting, hypoxemia, and increased metabolic rate.
Etiology of viral pneumonia. Interstitial inflammation, primary atypical pneumonia. Caused by influenza A or B, RSV, and adenoviruses. Less severe than bacterial pneumonia. UNCOMMON to find consolidation.
Symptoms of bacterial(lobar) pneumonia. Onset is abrupt, high fever, chills, thick purulent sputum, frequent tachycardia, hypoxemia, consolidation, WBC >10,000/cu mm, occasional pleuritic pain
Symptoms of viral pneumonia. Onset is gradual, low fever, thin mucoid sputum, uncommon to have chills, tachycardia,hypoxemia, or pleuritic pain, x-rays look normal, <10,000/cu mm,
Other types of pneumonia. Legionnaire's disease(gram neg bacteria), pneumocystis carinii pneumonia(PCP)fatal infection in AIDS patients, vent acquired pneumonia(VAP)nosocomial exchange, bronchiolitis obliterans organizing pneumonia(BOOP)seen in COPD.
What is ventilator associate pneumonia(VAP)? Common with ICU, 70% of patients on vent >2 days will get & 50% will die. Can be caused by caregiver error, aspiration, weak immune system.
Treatment of VAP. remove ETT as soon as possible, check feeding tube placement, clear secretions orally and endotracheal before messing with ETT, elevate HOB 30-40%
General treatment for pneumonia. Antibiotic, O2, fluids, aerosol therapy if indicated, CPT in indicated.
Treatment of PCP. Pentamidine(NebuPent), 300mg powder in 6ml sterile water, given 300mg once a week with a nebulization via respigard II nebulizer(has a 1 way valve to keep meds from being exposed to air) Proper PPE.
What is pulmonary embolism? Sudden partial or complete blockage of pulmonary artery blood flow.
Types of pulmonary embolism (PE). Thrombus and Embolus.
What is a thrombus PE? A blood clot attached to its site of origin, usually deep in the leg veins.
What is embolus embolism? A thrombus that is detached from site of origin; can be fat, blood, air, amniotic fluid, or tissue fragment.
Most common sites of thrombus formation. Deep veins of lower extremities, deep veins of pelvis, right side of heart.
Most common causes for thrombus. Immobilized b/c of pain, debilitated, paralyzed, prolong bed rest, obesity, surgery, fracture in leg, hip, or pelvis; stroke, MI, cancer
Factors facilitating thrombus formation. Abnormal vessel walls, blood stagnation, coagulability increase.
X-ray findings for PE. Prominent vascular markings, diffuse fluffy infiltrated w/ butterfly pattern, possible cardiomegaly.
Pathophysiology of PE. Increase in dead space ventilation(Vd/Vt ratio)total ventilation is increased in an effort to maintain normal PaCO2(shallow breathing), large emboli
Clinical presentation of PE. Chest pain, dyspnea, cough, faintness, anxiety, increased Vd ventilation; if large enough-tachypena & tachycardia,cyanosis, decreased breath sounds, wheezing & rales, pleural friction rub.
Diagnosis of PE. V/Q lung scan and pulmonary angiography
Treatment for PE. Leg exercises, compression elastic stockings, increased activity, anticoagulant drugs, clot filter, O2, vent if needed.
What is postoperative atelectasis. Collapse of lung due to anesthetic drugs or the inability to breath deeply, cough effectively due to pain.
Effects and complications from post op atelectasis. Can lead to pneumonia, respiratory failure, and longer hospital stay.
Pathophysiology of post-op atelectasis. surfactant depletion b/c of inability of alveoli to maintain stability and prevent collapse.
Effects of post-op atelectasis. Decreased FRC, V/Q mismatch, intrapulmonary shunting, decrease lung compliance, increase WOB, possible respiratory failure.
Treatment for post-op atelectasis. IS, IPPB, CPAP, PEP, secretion removal, deep breathing and cough, CPT, IPV, mechanical ventilation if needed.
X-ray results for post-op atelectasis. Reduction in lung volume, elevation of hemidiaphragm, mediastinal shifts towards the affected side.
Clinical signs of post-op atelectasis. Decreased VC, dyspnea, tachypnea, increased WOB, late inspiratory crackles, bronchial breath sounds.
Created by: rdy2learn
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