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Neonatal - Ch. 31

Ch. 31 (Egan's) - Neonatal and Pediatric Respiratory Disorders

QuestionAnswer
how many infants does neonatal respiratory distress syndrome affect each year in the US? 60,000-70,000
what is another name for RDS and what is RDS? hyaline membrane disease; a disease of prematurity
what are the 4 major factors in the pathophysiology of RDS? qualitative surfactant deficiency, dec. alveolar surface area, incr. small airways compliance, presence of DA
what does the severe hypoxemia and acidosis increase in RDS? PVR
because of the low surfactant production, what happens to the alveolar? from this, what happens? instability and collapse, leads to increased WOB; incr. surface tension, fluid in alveoli
the increased PVR leads to what? which overall leads too? increases right-to-left shunting through the PDA; worsened acidosis, hypoxemia, surfactant production
what is the first sign in RDS? what occurs next? tachypnea (occurs soon after birth); retractions, paradoxical breathing, grunting, nasal flaring
what does chest auscultation reveal? fine inspiratory crackles
if central cyanosis is present, what is likely that the infant has? severe hypoxemia
what mimics the aspect of RDS? systemic hypotension, hypothermia, poor perfusion
what is definitive diagnosis of RDS made with? and what is typically found? chest radiography; diffuse, hazy, reticulogranular densities w/ air bronchograms w/ low lung vols
what are the traditional support modes used to manage RDS? what else has been added? CPAP and PEEP; surfactant replacement therapy and high-freq ventilation
if the condition is not severe, what is indicated? a trial of nasal CPAP, 4-6 cmH2O (nasal prongs preferred)
when should MV with PEEP be initiated? if oxygenation doesn't improve w/ CPAP or if the pt is apneic or acidotic
what is the goal of MV for RDS? prevent lung collapse and maintain alveolar inflation
what type of tubes are used for ETT? and what type of ventilation is used for infants? uncuffed; pressure
what does optimal PEEP provide for RDS? what is done if high PaCO2 persists? what should the PIP be to minimize potential for volutrauma? lowest PaCO2, highest PaO2; must increase f or PIP; less than 30 cmH2O, lower for more immature infants
what are the 3 surfactant preparations to manage RDS? 1. beractant (survanta) 2. calfactant (infasurf) 3. poractant alfa (curosurf)
how is the surfactant given to infant? liquid suspensions that are instilled directly into trachea; all given through ETT
what is the dosing, ml/kg, administration, and dosing interval for beractant (survanta)? 100; 4; 1/4 dose quickly in each of 4 positions; Q6 or more often
what is the dosing, ml/kg, administration, and dosing interval for calfactant (infasurf)? 100; 3; 1/2 dose slowly supine then rotated; Q12 or more often
what is the dosing, ml/kg, administration, and dosing interval for poractant alfa (curosurf)? 100-200; 1.25-2.5; whole or 1/2 dose supine; Q12 or more often
what is the most common respiratory disorder of the newborn? transient tachypnea of the newborn (type II RDS)
what is the likely cause of TTN? delayed clearance of fetal lung liquid
how much does the birth canal squeeze normally accounts for clearance? what accounts for the final third? 2/3; lyphatics' absorption (immature lymphatics impairs absorption)
what are the first clinical manifestations of TTN? tachypnea; normal VA, pH, and PaCO2
what does the chest radiograph reveal? looks like pneumonia; hyperinflation, pleural effusions, perihilar streaking (lymphatic engorgement)
what is the most common treatment that infants respond to in TTN? low FiO2 by infant oxygen hood or nasal cannula; need higher FiO2, use CPAP
what is given once a culture is obtained? antibiotics
how long should it take to show clearing of the lungs on chest radiograph and clinical improvement? 24-48 hours
what is meconium aspiration syndrome a disease of? what does it involve and associated with? term and near-term infants; aspiration of meconium in central airways of the lung; perinatal depression and asphyxia
what does meconium consist of? mucopolysaccharides, cholesterol, bile acids and salts, intestinal enzymes, others
amniotic fluid stained with meconium is found in approximately ___ of all births. it is rare if the infant is less than ___ weeks' gestation age. ___ of infants w/ inhaled meconium clear lungs spontaneously. 12%; 37; 95
what is the real causative agent in MAS? fetal asphyxia that precedes after aspiration
what are the 3 primary problems of MAS? 1. pulmonary obstruction 2. lung tissue damage 3. pulmonary HTN
the lung tissue injury caused by MAS is _________ __________. chemical pneumonia
what is evidence that the fetus is at high risk of MAS? thick meconium, fetal tachycardia, absent fetal cardiac accelerations during labor
what do infants normally have with MAS? gasping respirations, tachypnea, grunting, retractions
what does the chest radiograph show? irregular pulmonary densities (areas of atelectasis), hyperlucent areas (hyperinflation from air-trapping)
what does the ABG normally show? and what happens in severe cases? hypoxemia with mixed resp and metabolic acidosis; R-to-L shunting and persistent PHTN
what should be done first for treatment? immediate intubation and suctioning; after ETT is inserted, it is removed to see meconium, if there is new ETT is inserted
what is indicated if the primary problem is hypoxemia? what if resp acidosis is severe and excessive WOB? CPAP; MV
what is shown to decrease the risk of air leak in MAS? HFV and SIMV
what has become a major adjunt in the management of persistent pulmonary hypertension? nitric oxide (DO NOT use high mean airway pressures)
what infants may have bronchopulmonary dysplasia? infants with severe respiratory failure in the first few weeks of life
what have been implicated in the origin of BPD? immaturity, genetics, malnutrition, O2 toxicity, MV
what are the intiating factors of BPD? atelectrauma and volutrauma
what conditions lead to BPD? hyperoxia/hypoxia, mechanical forces, vascular maldevelopment, inflammation, nutrition, genetics
__________ is the term coined to describe loss of alveolar volume that is both a consequence and a cause of lung injury. ____________ is the term used to describe local overinflation (and thus stretch) of airways and alveoli. atelectrauma; volutrauma (both increase lung injury)
what does atelectrauma and volutrauma cause a need for? increased supplemental O2 concentrations
what is the "new" BPD? decreased alveolarization rather than prominent airway damage of the "old" BPD
what occurs approximately 2-3 weeks of life? progressive respiratory distress (needs O2 and MV)
what does the chest radiograph for severe disease show? atelectasis, emphysema, fibrosis diffusely intermixed
what does the ABG look like? hypoxemia and hypercapnia secondary to airway obstruction, air-trapping, pulm fibrosis, atelectasis
what is the best management of BPD? prevention
_________ should be delivered early in the course of treatment. surfactant
what is involved in the treatment steps of BPD? minimize additional lung damage and prevent pulmonary HTN and cor pulmonale
________ are given as needed to decrease pulmonary edema; __________ are given to manage existing pulmonary infection. ______ ___________ for retained secretions. ____________ therapy to decrease RAW. diuretics; antibiotics; chest physiotherapy; bronchodilator
what is given to produce substantial short-term improvement in lung function, often allowing rapid weaning from ventilatory support? steroid therapy with dexamethasone
what are the periods of apnea of prematurity in infants? 5-10 seconds followed by 10-15 seconds of rapid respiration
when are the apneic spells abnormal? 1. they last longer than 15 secs 2. they are associated with cyanosis, pallor, hypotonia, or bradycardia
when is it considered central apnea? if no effort to breathe occurs during a spell
when is it considered obstructive apnea? if breathing efforts occur but obstruction prevents air flow
when is it considered mixed apnea? combination, that starts as obstructive and develops into central
premature infants have an immature ____________ of ___________ ______. chemo-control; respiratory drive
what should be managed in infants with apnea? and what can terminate apnea periods in infants? underlying cause if identified; tactile stimulation
______ is used to reduce mixed and obstructive apnea by splinting the upper airway. CPAP
what does apnea secondary to prematurity respond well to? if they dont respond well to this, what is used? theophylline and caffiene; doxapram
_________ decreases hypoxic depression by increasing oxygen-carrying capacity. _________ __________ provides support when respiratory effort is inadequate. transfusion; mechanical ventilation
when does periods of apnea begin to disappear? 37-44 of postmenstrual age with no apparent long-term effects
what is a common denominator in persistent pulmonary hypertension of the newborn? return to fetal circulatory pathways, usually because of high PVR
what does this condition result in? further R-L shunting, severe hypoxemia, and metabolic and resp acidosis
what 2 anatomic shunts does the fetus have? foramen ovale and the ductus arteriosus
the intrauterine total pulmonary blood flow is ____. low
when is it definite that the infant has PPHN? if the PVR does not decrease to allow the PVR/SVR ratio to become less than 1
what are the 3 fundamental types of PPHN? 1. vascular spasm 2. increased muscle wall thickness 3. decreased cross-sectional area of pulmonary vessels
what is vascular spasm? acute event thats triggered by hypoxemia, hypoglycemia, hypotension, and pain
what is increased wall thickness? chronic condition develops in utero in response to several different factors (chronic fetal hypoxia, incr pulm blood flow, pulm venous obstruction)
what is decreased cross-sectional area of vasculature related to? hypoplasia of the lungs and occurs with congenital diaphragmatic hernia, absent kidneys, and decreased amniotic fluid
when should PPHN be suspected? when rapidly changing SpO2 hypoxemia is worse than indicated on chest radiograph
how is this detected? performing preductal and postductal SpO2 (pred should be >5% post)
what is the initial therapy for PPHN? removal of underlying cause, hypoxemia w/ O2 and surfactant for RDS
what if correction does not correct hypoxemia? needs intubation and MV; sedation, paralysis; HFV; inhaled nitric oxide; ECMO (if all fails)
what are the three fundamental mechanisms of airway abnormalities? internal obstruction, external obstruction, disruption
what does internal obstruction include? laryngomalacia, tracheomalacia, laryngeal webs, tracheal stenosis, hemangiomas
what do these conditions manifest as? inspiratory stridor, gas trapping, expiratory wheezing, accessory resp muscle activity
what is caused by external obstruction? hemangiomas, neck or thoracic masses, vascular rings
airway disruptions usually are related to ____________ ________ in a newborn. tracheoesophageal fistula (usually associated with esophageal atresia)
what are the 5 types of TEF? esophageal atresia w/ proximal fistula; distal fistula; w/ both; w/out either; w/ intact esophagus w/ H fistula
what is the most common of these? least common? esophageal atresia w/ a distal fistula (85%-90%); H fistula
what do all these malformations manifest as? difficulty swallowing, bubbling/frothing at mouth, choking
what is TEF managed with? surgical ligation of the fistula and reconnection of the interrupted esophagus
what is the most common severe lung malformation? congenital cystic adenomatoid malformation of the lung (C-CAM)
what is the usual treatment of C-CAM? surgical removal of the affected lobe
what are less common lung malformations? pulmonary sequestration and lobar emphysema
what is congenital diaphragmatic hernia? severe disease that usually manifests in newborns as severe respiratory distress
what is the pathophysiologic mechanism of CDH? lung hypoplasia and abnormal development
what are the 2 types of hernia? 1. Bochdalek hernia (lateral/posterior, left) 2. Morgagni hernia (medial/anterior, either side)
what might physical examination include in CDH? scaphoid abdomen, decreased breath sounds, displaced heart sounds, severe cyanosis
what is the initial treatment? intubation, paralysis, MV, continuous gastric suction
what is delayed in tx? surgical repair for PVR to fall
what can large defects in the abdominal wall cause? severe respiratory compromise, most commonly omphalocele
what is omphalocele? abdominal wall defect involving insertion of umbilical cord
what must omphalocele be distinguised from? gastroschesis (abdominal wall defect that is completely separate from the insertion of UC)
what diseases are included in poor neuromuscular control? spinal muscular atrophy, congenital myasthenia gravis, myotonic dystrophy, and others
what are the 2 large categories of congenital heart diseases? cyanotic and acyanotic
what are cyanotic heart diseases? blood shunts from R to L, bypassing the lungs, thus deoxygenated
what are acyanotic heart diseases? blood shunts from L to R, thus causes CHF
what are the 2 most common cyanotic heart diseases? 1. tetralogy of Fallot 2. transposition of the great arteries
what does tetralogy of Fallot include? 1. pulmonary stenosis 2. ventricular septal defect 3. dextroposition of aorta 4. RV hypertrophy
what does a mild case manifest as? heart murmur, intermittent severe cyanotic spells, infant squatting/entering knee chest position
what does a severe case manifest as? heart murmur and severe continuous cyanosis
what is transposition of the great arteries? the heart disease that most frequently causes severe cyanosis
what does it manifest as? mod-severe cyanosis immediately after birth
what treatment is frequently needed? emergency atrial septostomy (cutting a hole in the wall b/t two atria)
what is the goal? allow PVR to decrease and then perform arterial switch operation in the 2nd or 3rd wk of life
what is the most common congenital heart disease? ventricular septal defect
what is among the most severe of congenital heart diseases? hypoplastic left heart syndrome
what does VSD result in and when does it appear? left-to-right shunt and CHF; 6-8 wks as PVR falls
what is the most common type of atrial septal defect? small, slit-like opening that persists after closure of the foramen ovale
what is patent ductus arteriosus treated with? indomethacin (pharmacologic) or ligation (surgical)
what are the 3 accepted treatments of hypoplastic left heart syndrome? comfort care (allows infant to die), palliative surgical procedure, transplantation
what is the leading cause of death among infants younger than 1 year in the US? SIDS
what infant normally dies to SIDS? preterm african-american boy born to a poor mother <20 w/ inadequate prenatal care
what age is most suseptible and when does it normally occur? 1-3 months; night during winter months
the _______ sleeping position has been strongly associated with increased risk of SIDS. prone
_________ is the treatment of SIDS and what does this include? prevention; identification of risk, trained in apnea monitoring/CPR, supine/side-lying position, reduce soft objects during sleep
what is gastroesophageal reflux disease? regurgitation of stomach contents into the esophagus
what are the respiratory problems associated with GERD? reactive airway disease, aspiration pneumonia, laryngospasm, stridor, chronic cough, choke, apnea
___________ is an acute infection of the lower respiratory tract, usually caused by the RSV. bronchiolitis
what is the clinical manifestations of bronchiolitis? inflammation/obstruction of small bronchi/bronchioles
when does it occur? after a viral upper respiratory infection (fever, cough, dyspnea, tachypnea, wheezes)
what does the chest radiograph show? hyperinflation with areas of consolidation
what is the treatment of bronchiolitis? relief of airway obstruction and hypoxemia; systemic hydration, croup tent, O2 hood, NC
_________ is given to decrease the length of MV in severe cases. ribavirin
______ is a viral disorder of the upper airway that normally results in subglottic swelling and obstruction. croup (laryngotracheobronchitis)
what is viral infection resulting in subglottic swelling? the most common cause of obstruction in 6 month-6 year olds
when do the symptoms become evident? after 2-3 days of nasal congestion, fever, coughing; stridor, barking cough, dyspnea, cyanosis, exhaustion
what does the chest radiograph show? subglottic narrowing of trachea, "steeple sign"
what is the treatment of croup? cool mist; supplemental O2; aerosolized racemic epinephrine and dexamethasone; budesonide; MV
__________ is an acute and often life-threatening infection of the upper airway that causes severe obstruction secondary to supraglottic swelling. what is the most common cause? epiglottitis; H. influenzae type B infection
what are the clinical manifestations of epiglottitis? high fever, sore throat, stridor, labored breathing, muffled voice
what does the LATERAL NECK radiograph show? epiglottis thickened and flattened ("thumb sign"), aryepiglottic folds swollen, vallecula may not be visualized
what is the treatment of epiglottitis? elective intubation under general anesthesia, place on CPAP w/ low PSV (3 cmH2O)
what is the most common lethal genetic disease among caucasian americans? what does it involve? CF; gene mutation affecting chloride movement, particularly in exocrine glands
what are the most severely affected organs? sweat glands, pancreas, lungs; skin is salty
what does pancreatic insufficiency lead to? malnutrition, diarrhea, steatorrhea
what is the leading cause of death among patients with CF? complications of lung disease
what do the patients produce? copious amounts of thick sputum; retained secretions lead to recurrent infections, atelectasis, pneumonia or lung abscesses
what is the treatment of CF? pancreatic enzyme supplements; CPT, exercise; DNase, 7% saline; antibiotics (tobramycin); ibuprofen, bronchodilators
what is the most commonly used form of lung transplantation in the treatment of CF? double-lung transplantation
what is the median survival age of patients with CF? 38 years
Created by: christa_2008