Peds Chapter 19 - Respiratory Tract & Mediastinum 49 terms Je Terms in this set (147) Name the 5 overlapping stages of lung development and when each occurs 1) Embryonic (3-7 wks gestation) 2) Pseudoglandular (5-17 wks gest) 3)Canalicular (16-26

Peds Chapter 19 - Respiratory Tract & Mediastinum 49 terms Je Terms in this set (147) Name the 5 overlapping stages of lung development and when each occurs 1) Embryonic (3-7 wks gestation) 2) Pseudoglandular (5-17 wks gest) 3)Canalicular (16-26 wks gest) 4) Saccular (26-36 wks gest) 5) Alveolar (36 wk- 3-8yrs of age)* *controversy exists over the length of this developmental period. Abnormalities during this stage of lung development lead to lung hypoplasisa and can result in bronchopulmonary dyplasia (BPD). Alveolar stage (36wks gestation to 3-8yrs of age) In order to produce reliable lung function tests (FVC, FEV1, etc), what age should the child be? Most children can produce reliable results by age 4 yrs In peds, what is the most common mechanism of hypoxiema? Ventilation/Perfusion (V/Q) mismatch. This occurs in acute asthma, CF, pnuemonia, bronchiolitis, bronchopulmonary dysplasia (BPD). To culture material from the respiratory tract, spontaneous expectorated sputum is the least invasive way to collect a sample, however it is rarely attainable from kids less than years old. The best surrogate for this test is _. 6 years old; Oropharyngeal swabs T/F- In children it is safe to use aerosolized hypertonic saline as a means to induce a lower airway sputum sample. True; It is most often used to obtain cultures form pts with CF, TB, and PCP pneumonia. The foundational form of imaging of the lungs in peds is _. Radiographs; The radiograph is useful for evaluating chest wall abnormalities, heart size and shape, mediastinum, diaphragm, and lung parenchyma In peds, chest CT is useful for evaluation of ______. Congenital lung disease, among others uses seen in adults. Peds Chapter 19 - Respiratory Tract & Mediastinum Name some indications for bronchoscopy (direct visualization of the bonchi). Wheezing, suspected foreign body, pneumonia, atelectasis, chronic cough hemoptysis T/F- Environmental insults (smoking, pollution) aggravate lung disease in children, but it is unclear as to wether they CAUSE lung disease. True EXTRATHORACIC/upper airway obstruction disrupts (inspiratory or expiratory) breathing, producing (sound). INSPIRATORY; Stridor or "noisy breathing" INTRATHORACIC obstruction disrupts _______(inspiratory or expiratory) breathing, producing (sound). EXPIRATORY; Wheezing by prolonging the expiratory phase FIXED airway obstructions are associated with abnormalities. In what pattern do they affect breathing? Anatomical abnormalities; They affect each breath What are VARIABLE airway obstructions associated with? In what pattern do they affect breathing? Dynamic changes in lung CALIBER ie..tacheo-, laryngeo- or bronchomalacia. The abnormalities in breathing are interspersed with normal breathing. The general goal of oxygen therapy is to achieve an arterial oxygen tension of _______ or an oxygen saturation above _. 65-90 mm Hg; 92% In infants, the flow of O2 through the nasal cannula should generally not exceed ____ L/min to avoid excessive drying of the mucosa. 3 L/min Inhaled medications include "relievers" and "controllers". What category of drugs are included in each? Relievers= Bronchodialtors (short acting Beta Agonists and Anticholinergics). Controllers= Anti-inflammatory drugs (corticosteroids and cromones) What are currently available forms of "airway clearance therapy" (used most often for improving clearance of lower airway secretions in kids with CF, bronchiectasis, and neuromuscular disease)? chest physiotherapy, autogenic drainage, blowing therapies (bubbles, pinwheels), active coughing, positive expiratory pressure (PEP) with handheld devices, intrapulmonary percussive ventilation, or high-frequency chest compression What is an important adjunctive therapy for "airway clearance tx" and overall lung health? Daily exercise What inhaled meds are best given before and after "airway clearance therapy"? Before: Bronchodialtors and mucolytics (to enhance the effects of therapy) After: Corticosteroids and antibiotics (given once airway is clear to enhance penetration) What is the name of a benign congenital disorder in which the cartilaginous support for the supraglottic structures is underdeveloped. Laryngomalacia ______ is the MOST COMMON CAUSE of CONGENITAL VARIABLE extrathoracic airway obstruction Laryngomalacia Persistent stridor present within the first 6 weeks of life is suggestive of ______ Laryngomalacia What other condition may also be associated with laryngomalacia and requires treatment? GERD T/F- Laryngomalacia rarely improves on its own, often requiring life long ventilation. False - The condition usually improves with age and resolves by the time the child is 2-3 years old. In severe cases surgical epiglottoplasty may be beneficial. The diagnosis of laryngomalcia is established by _________, which shows _. direct laryngoscopy; inspiratory collapse of an omega-shaped epiglottis T/F -Laryngeal atresia presents immediately after birth with severe respiratory distress and is usually fatal. True T/F- FIXED causes of CONGENITAL EXTRATHORACIC obstruction usually require surgical intervention. True (they are due to anatomic abnormalities: laryngeal web, laryngocele, cysts of larynx, subglottic hemangioma, laryngeal cleft) T/F- Subglottic hemangiomas are seen in infancy with progressive signs of upper airway obstruction and can be associated with similar lesions of the skin (but not always) True. (Note: subglottic hemangiomas often regress spontaneously but may be treated with PROPANOLOL to speed regression) T/F- All types of laryngeal clefts (type 1 (mild),2,3(severe)) may result in recurrent or chronic pneumonia and failure to thrive. True. (due to silent aspiration that occurs with this condition) Typical features of CONGENITAL disorders of the extrathoracic airway are.... 1) Presentation from birth or within the first few months of life. 2) Inspiratory sounds usually of a high-pitched ("croup") nature but can be variable. Typical features of ACQUIRED disorders of the extrathoracic airway are.... 1) Presentation may be acute or subacute depending on diagnosis. 2) Inspiratory sounds consistent with stridor. Sudden onset of coughing or respiratory distress and Difficulty vocalizing are features of what condition? Foreign body aspiration What age is the highest risk for foreign body aspiraiton? 6m to 4 years T/F- In foreign body aspiration, If partial obstruction is present, then the choking child should be allowed to use his or her own cough reflex to remove the foreign body. True What is the age cut-off for using back thrust v Heimlich maneuver for a choking child? 1 years old; less than one use back blows, over one use Heimlich T/F- Blind finger sweeps should not be performed in infants or children because the finger may actually push the foreign body further into the airway causing further obstruction True What are the main differential diagnosis for patients presenting with acute stridor? acute inflammatory diseases of the larynx including viral croup (laryngotracheobronchitis), epiglottitis (supraglottitis), and bacterial tracheitis Viral croup generally affects younger children in the fall and early winter months and is most often caused by _. parainfluenza virus serotypes On examination of a child presenting with acute stridor, the presence of and the absence of favor the diagnosis of viral croup over epiglottitis. cough; drolling What is the determining factor, in a pt with croup, as to wether more than minimal supportive therapy (oral hydration) is needed? The presence of stridor at rest Pt with croup and stridor even at rest should be treated with what therapies Oxygen, nebulized epinephrine, dexamethasone (IM or oral), budesonide (inhaled steriod) T/F -Most children with viral croup have an uneventful course and improve within a few days True Epiglottitis is most likely due to what organism? Haemophilus influenzae. If children are vaccinated it may be due Neisseria meningitides or Streptococcus The classic presentation of this disease is a sudden onset of high fever, dysphagia, drooling, muffled voice, inspiratory retractions, cyanosis, and soft stridor. Patients often sit in the so-called sniffing dog position Epiglottitis Epiglottitis produces what classic XRAY finding Thumb-print sign T/F - Once the diagnosis of epiglottitis is made, endotracheal intubation must be performed immediately in children but not necessarily in adult populations. True Antibiotic treatment of epiglottitis Ceftriaxone The early clinical picture of this disease is similar to that of viral croup. However, instead of gradual improvement, patients develop higher fever, toxicity, and progressive or intermittent severe upper airway obstruction that is unresponsive to standard croup therapy. Bacterial Tracheitis which is most commonly caused by S. aureus T/F- Unilateral or bilateral vocal cord paralysis may be congenital, or more commonly may result from injury to the recurrent laryngeal nerves. True. ( Risk factors for acquired paralysis include difficult delivery (especially face presentation), neck and thoracic surgery, trauma, mediastinal masses, and CNS disease) T/F- Neonates and infants are particularly vulnerable to subglottic injury from intubation True. (The subglottis is the narrowest part of an infant's airway) Benign, warty growths that are difficult to treat and are the most common laryngeal neoplasm in children. Papillomas of the larynx What is the causative agents implicated in recurrent respiratory papillomatosis? Human papillomaviruses (HPV) 6, 11, and 16 The age of onset for papillomatosis Usually 2-4 years. A younger age of onset may be a worse prognostic indicator. What are signs of papillomatosis? Patients usually develop hoarseness, voice changes, croupy cough, or stridor that can lead to life-threatening airway obstruction. How are laryngeal papillomas diagnosed and treated? Direct laryngoscopy; Tx: laser surgical removal but recurrence is common. Fortunately, spontaneous remissions do occur, usually by puberty, so that the goal of therapy is to maintain an adequate airway until remission occurs. What are CONGENITAL causes of INTRAthoracic airway obstruction? Malacia of airways, vascular rings and slings, bronchogenic cysts Chronic wheeze with or without a barking cough and respiratory symptoms that do not respond to bronchodilators are typical features of what CONGENITAL INTRAthoracic obstruction? Malacia of airways (tracheomalacia or bronchomalacia) Conditions where cartilaginous framework of the airway is inadequate to maintain airway patency Malacia of airways (tracheomalacia or bronchomalacia) Treatment of airway malacia Conservative treatment is usually indicated for the isolated condition, which generally improves over time with growth. In severe cases of tracheomalacia, intubation or tracheostomy may be necessary. The most common vascular anomaly to compress the trachea or esophagus is _. A vascular ring A vascular ring or pulmonary sling (malformations of the great arteries) produce what type of symptoms? Symptoms of chronic airway obstruction (stridor, coarse wheezing, and croupy cough) are often worse in the supine position. Bronchogenic cyst are CONGENITAL INTRAthoracic airway obstructions that generally occur where? Bronchogenic cysts generally occur in the middle mediastinum near the carina and adjacent to the major bronchi (but can be found elsewhere in the lung). T/F- Bronchogenic cysts may be asymptomatic until adulthood, however, all asymptomatic cysts will eventually become symptomatic with chest pain being the most common presenting complaint True The two main genetic diseases of mucociliary clearance are... CF and Primary ciliary dyskinesia Greasy, bulky, malodorous stools; failure to thrive. Recurrent respiratory infections. Digital clubbing on examination. Bronchiectasis on chest imaging. Sweat chloride > 60 mmol/L. Are typical feature of what disease? CF Decribe the newborn screening for CF Blood draw measuring immunoreactive trypsin (IRT), a pancreatic enzyme with or without concurrent DNA testing for gene mutations in CFTR gene. T/F- Meconium ileus is virtually diagnostic of CF, so the infant should be treated presumptively as having CF until a sweat test or genotyping can be obtained. True What vitamins are children with CF at risk for deficiency? fat-soluble vitamins A, D, E, and K, because of ongoing steatorrhea (lack of pancreatic lipase). Chronic pneumonia is an issue in children with CF, what is the predominant pathogen as they age? Pseudomonas aeruginosa How is the diagnosis of CF made? The diagnosis of CF is made by a sweat chloride concentration greater than 60 mmol/L in the presence of one or more typical clinical features (chronic sinopulmonary disease, pancreatic insufficiency, salt loss syndromes) or an appropriate family history. A diagnosis can also be confirmed by genotyping that reveals two disease-causing mutations. T/F- Patients with mild CFTR mutations typically have no clinically significant issues. False. Patients with mild CFTR mutations typically have adequate pancreatic exocrine function, but are still at risk for severe lung disease. Describe an ideal diet composition for a child with CF Pancreatic enzyme supplementation combined with a high calorie, high protein, and high fat diet. Daily multivitamins that contain vitamins A, D, E, and K. Daily salt supplementation also is recommended to prevent hyponatremia, especially during hot weather. Describe the mainstay of pulmonary treatment in CF Airway clearance therapy and aggressive antibiotic use form the mainstays of treatment for CF lung disease. Antibiotic therapy appears to be one of the primary reasons for the increased life expectancy of persons with CF. What antibiotics are used? inhaled tobramycin (TOBI) or inhaled aztreonam, and chronic oral azithromycin. T/F- A few decades ago, CF was fatal in early childhood. Now the median life expectancy is around 35 years of age. True Also known as immotile cilia syndrome, it is a rare, inherited, usually autosomal recessive disorder with impaired ciliary function leading to progressive sinopulmonary disease. Of note half of these pts have their internal organs reversed. What condition is this? Primary ciliary dyskinesia (PCD) A rare chronic obstructive lung disease characterized by complete obliteration of the small airways following a severe insult. Bronchiolitis obliterans. The most common form in children is postinfectious, following a lower airway tract infection with adenovirus, although influenza, rubeola, Bordetella, and Mycoplasma are also implicated What are congenital pulmonary agenesis and hypoplasia? Ageneisis: unilateral pulmonary agenesis is complete absence of one lung and often associated other congenital abnormalities, such as absence of one or both kidneys or fusion of ribs Hypoplasia: incomplete development of one or both lungs, characterized by a reduction in alveolar number and a reduction in airway branches. Nonfunctional pulmonary tissue that does not communicate with the tracheobronchial tree and receives its blood supply from one or more anomalous systemic arteries. Pulmonary Sequestration This disorder accounts for 95% of cases of congenital cystic lung disease congenital pulmonary airway malformation (CPAM) T/F- Discrimination between viral and bacterial pneumonia is challenging as neither the white blood cell count nor differential nor the chest radiograph are strong predictors True but viral is more common The most common cause of bacterial pneumonia in children of all ages is _. S pneumoniae Hallmark signs of community acquired pneumonia in kids? Fevers (over 39°C), tachypnea, and cough In children less than 5 years old with cough, fever and/or abnormal chest x-ray, the most likely bacterial pathogen is and the antibiotic of choice is _. S pneumoniae; amoxicillin (2nd generation ceph or macrolide if PCN allergic). T/F- Children older than 5 years are more likely to have atypical pneumonia (eg, Mycoplasma) and a macrolide is the best empiric antibiotic choice True T/F- ALL children younger than 3 months of age are generally admitted to the hospital for treatment of bacterial pneumonia True The most common organism associated with empyema is _. S pneumoniae. T/F- Although in adults having thoracocentesis, the presence of low pH and glucose indicates the need for aggressive and thorough drainage procedures, the prognostic significance of these findings in children is unknown True Describe classic features of viral pneumonia Upper respiratory infection prodrome (fever, coryza, cough, hoarseness). Wheezing or rales. Myalgia, malaise, headache (older children). Patients at risk for life-threatening RSV infections (eg, those with BPD or other severe pulmonary conditions, congenital heart disease, or significant immunocompromise) should be hospitalized and should be considered ribavirin The most common serious acute respiratory illness in infants and young children. What is its cause? Bronchiolitis. RSV is by far the most common viral cause of acute bronchiolitis. The most common complication of bronchiolitis is _. Superinfection with a bacterial pathogen such as Streptococcus pneumoniae leading to pneumonia. Describe children at high risk for hospitalization due to bronchiolitis includes infants younger than 6 months of age, especially with any history of prematurity, and those with underlying chronic cardiopulmonary disorders. T/F- Conjunctivitis is a rare finding in pneumonia caused by C trachomatis False - About 50% of infants with C trachomatis pneumonia have active inclusion conjunctivitis or a history of it. Treatment of C trachomatis pneumonia macrolide or sulfisoxazole T/F- An increased incidence of obstructive airway disease and abnormal pulmonary function tests may occur for at least 7-8 years following pneumonia infection with C trachomatis. True T/F- Mycoplasmal pneumonia is more common in children under 1 years old False- it is more common in older childern (>5years) Sore throat, otitis media, otitis externa, and bullous myringitis are associated with what type of pneumonia? Mycoplasmal pneumonia