Respiratory disorders


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Review of Respiratory Disorders from 2010 ABP Content Specs

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Respiratory disorders

  1. 1. Respiratory Disorders<br /><ul><li>General Signs and Symptoms
  2. 2. Stridor
  3. 3. Know the ddx of congenital stridor
  4. 4. Inspiratory stridor: Laryngomalacia, subglottic stenosis, vocal cord paralysis
  5. 5. Expiratory stridor: tracheomalacia or lesions in the airway structures or thoracic cavity
  6. 6. Biphasic: vascular rings and slings
  7. 7. Know the different etiologies of stridor in children of different ages
  8. 8. Birth: choanal atresia, laryngeal web or stenosis, vascular ring, vocal cord paralysis
  9. 9. 4-6 weeks: laryngomalacia, tracheomalacia
  10. 10. 1-4 years: croup, epiglottitis, foreign body aspiration
  11. 11. >5 years: vocal cord dysfunction, exercise, peritonsillar abscess, anaphylaxis
  12. 12. Know that endoscopy is the diagnostic tool of choice for laryngeal and vocal cord disorders
  13. 13. Laryngomalacia will reveal an omega shaped epiglottis that prolapses during inspiration
  14. 14. Paroxysmal vocal cord motion (PVCM)
  15. 15. Understand the appropriate approach to the evaluation of congenital stridor
  16. 16. Primary evaluation: thorough birth hx, current medical hx, , observation of breathing patterns in different positions, auscultation of the airway
  17. 17. Secondary evaluation: can include radiography, spirometry, direct airway visualization (laryngoscopy)
  18. 18. Know that vocal cord dysfxn may mimic asthma
  19. 19. Also called paroxysmal vocal cord motion (PVCM) or laryngeal dyskinesia
  20. 20. Differences between it and asthma: the dyspnea is primarily inspiratory, noise occurs on inspiration, does not respond to brochodilators, no hyperinflation on CXR, PFTs show a variable flattening of the inspiratory limb of the flow-volume curve (is the expiratory limb in asthma)
  21. 21. Respiratory failure
  22. 22. Know the clinical manifestations of acute hypercapnea: flushing, agitation, confusion, tachycardia, HA
  23. 23. Look for a case where a pediatric patient is being under-ventilated, i.e. giving breaths at too slow a rate while being BVMed
  24. 24. Know the potential risks and benefits of administering oxygen to children with chronic respiratory failure
  25. 25. The respiratory drive of some of these patients is dependent on hypoxemia, and correcting the hypoxemia could lead to respiratory arrest; goal should be to use the lowest amount of supplemental O2 possible to maintain an SaO2 of 90%
  26. 26. Know when to intubate and when to provide oxygen therapy in patients with respiratory failure of various etiologies
  27. 27. Failure to respond to O2 administration indicates the need for more aggressive management such as BVM or intubationif there is a process that is affecting their neurologic or neuromuscular processes and interfering with their ability to generate adequate tidal volumes, then earlier intubation would be likely
  28. 28. Cough
  29. 29. Know the ddx of chronic cough in children of different ages
  30. 30. Causes of chronic cough in kids with normal CXR:
  31. 31. Upper airway cough syndrome (previously post nasal drip syndrome)
  32. 32. Expect to find this in a kid over age 3 and with other sx of allergies
  33. 33. Asthma
  34. 34. GER
  35. 35. (usually creates sx during the first few postnatal months and then resolves by age 12 mos)
  36. 36. Tracheomalacia
  37. 37. Habit-cough syndrome
  38. 38. Vocal cord dysfxn
  39. 39. Distinguish between clinical manifestations of psychogenic cough and those caused by cough of organic etiology
  40. 40. Aka “habit cough syndrome”
  41. 41. Usually begin with an uncomplicated URI, but the cough lingers for months to years; will see a repetitive coughm cessation during sleep, and a hacky/barky nature to the cough
  42. 42. Recognize cough as a major and at times singular manifestation for asthma
  43. 43. Understand the limited indications for cough suppressants
  44. 44. Not better than placebo on several trials
  45. 45. Best rec is to use saline nasal drops, suctioning
  46. 46. Know the initial screening evaluation of chronic cough should include x-ray study if the chest, a sweat test, tuberculin skin test, and PFTs before resorting to other tests
  47. 47. Know which conditions that occur in childhood impair the effectiveness of cough: CO, muscle weakness, vocal cord dysfunction, CNS disease, thoracic deformities, pain
  48. 48. Exercise intolerance
  49. 49. Know that exercise intolerance may reflect etiologies other than pulmonary disease: anemia, muscle weakness, deconditioning, cardiac disease, psychogenic causes
  50. 50. Exercise intolerance = failure to tolerate physical exercise at the level that would be expected for the person’s age and condition
  51. 51. Measured by the maximal oxygen consumption test
  52. 52. Know that exercise intolerance may be a presenting sx of CLD (e.g. asthma, interstitial lung disease) or vocal cord dysfxn
  53. 53. Asthma = shortness of breath, chest tightness, cough about 10-15 mins after beginning exertion, nighttime cough
  54. 54. Vocal cord dysfxn= paradoxic adduction of vocal cords during inspiration, causing airway obstruction during exercise; also with inspiratory wheezing and throat tightness ; NO night time cough
  55. 55. Apnea
  56. 56. Distinguish between apnea and periodic breathing
  57. 57. Apnea is defined as a cessation of respiration for more than 20secs or if for less than 20 secs is associated with cyanosis or bradycardia
  58. 58. Know the difference between central and obstructive apnea
  59. 59. Central sleep apnea is a problem in which they lack the ability to sense hypercapnea
  60. 60. Obstructive sleep apnea = a disorder of breathing during sleep characterized by prolonged or partial airway obstruction or intermittent complete obstruction that disrupts normal ventilation during sleep and normal sleep patterns
  61. 61. Habitual (nightly) snoring, often with intermittent pauses, gasps or snorts
  62. 62. Sleep difficulty
  63. 63. Daytime neurobehavioral problems
  64. 64. Differentiate among obstructive, central, and mixed sleep-associated apnea
  65. 65. Obstructive – see above
  66. 66. Central – see above; myelomeningocele, hydrocephalus and Arnold- Chiari can be causes
  67. 67. Mixed – combines aspects of OSA and CSA
  68. 68. Know the testing procedures to evaluate the presence and degree of obstructive apnea on older children
  69. 69. Overnight polysonmnography is the gold standard but isn’t very practical
  70. 70. Overnight oximetry or nap time PSG (high specificities but low sensitivities)
  71. 71. Know the ddx of central sleep apnea in infancy
  72. 72. Have to differentiate it from apnea due to positions (neck overflexed), GER with reflex glottic closure and cessation of airflow, and mechanical obstruction of trachea or larynx
  73. 73. Central apnea can be due to:
  74. 74. Infection
  75. 75. Prolonged hypoxemia (lung dz, anemia, PDA)
  76. 76. Meds / CNS depressants
  77. 77. ICH
  78. 78. Hydrocephalus
  79. 79. Electrolyte abns
  80. 80. Hypoglycemia
  81. 81. Hypo/hyperthermia
  82. 82. Prematurity and immature resp drive
  83. 83. Know the tx of idiopathic recurrent apnea in premature infants
  84. 84. i.e. apnea of prematurity
  85. 85. MUST rule out other treatable causes
  86. 86. Treated with caffeine
  87. 87. Understand the association between apnea and anemia in premature infants
  88. 88. Anemia of prematurity can contribute to apnea of prematurity when profound (usually if hgb<7), but is a late phenomenon occurring 2-4 weeks after birth
  89. 89. Wheezing
  90. 90. Differentiate among the dxes responsible for persistent localized wheezes (e.g foreign body, malacia) versus diffuse or migratory wheezes
  91. 91. Know the ddx of recurrent wheezing
  92. 92. Asthma related causes and non-asthma related causes
  93. 93. Kids who wheeze during infancy continue to wheeze after age 6 about 15% of the time; also about 15% of asthmatics will develop wheezing after age 6
  94. 94. Non-asthma – foreign body, bronchiolitis obliterans, BOOP, bronchiectasis
  95. 95. Know when to consider foreign body in the ddx of recurrent wheezing
  96. 96. Age (older infants and toddlers), abrupt onset of symptoms especially if after eating “danger” foods (popcorn, nuts, hot dog), previously without sx or a long time since any respir probs
  97. 97. If the hx strongly suggests FB aspiration, they need prompt rigid bronochoscopy; can’t often see the effects of the FB for up to 24 h after aspirated (air trapping)
  98. 98. Contrary to popular belief, the aspiration of a small foreign body, including food, is as common in the left as the right mainstem bronchus in young children because the bifurcation of the trachea remains symmetric until the aortic knob grows larger in later childhood
  99. 99. Complication= secondary bacterial pneumonia
  100. 100. Tachypnea
  101. 101. Know that respir rates vary with age and that normal variations occur with sleep, eating and activity in normal children
  102. 102. Recognize that tachypnea is a sensitive indicator of respiratory disease
  103. 103. Especially lower airway disease and should be looked at in context of other symptoms
  104. 104. Hemoptysis
  105. 105. Know the ddx of hemoptysis in children
  106. 106. Most common etiologies are infection, foreign body and bronchiectasis
  107. 107. More rare causes include vasculitides (HSP, Wegener’s, Goodpasture’s and SLE), congenital heart or lung defects,vneoplasm, AV malformation, hemangioma, trauma, PE and idiopathic
  108. 108. Know the initial management of hemoptysis in children and adolescence
  109. 109. Assess other signs and sx
  110. 110. Get a CXR and if dx still not clear get a CT
  111. 111. After bleeding is controlled a bronchoscopy with BAL is done; look for hemosiderin laden macrophages (are diagnostic for pulm bleeding, don’t usually appear until 3 days after bleed)
  112. 112. Management is usually supportive, follow the ABCs of life support, might need embolotx of severe
  113. 113. Cyanosis
  114. 114. Know that cyanosis is not a sensitive indicator of oxyhemoglobin saturation (??)
  115. 115. Know the common extrapulmonary causes of cyanosis: R to L shunt, methemoglobinemia
  116. 116. The article cited only talks about methemoglobinemia; treat it with methylene blue
  117. 117. Know how to validate and quantitate a clinical observation of cyanosis: ABG, oxyhemoglobin sat
  118. 118. Clubbing
  119. 119. Recognize D/Os commonly associated with digital clubbing
  120. 120. Cyanotic heart disease (pulm atresia with unrepaired VSD and collateral pulmonary blood flow), chronic lung disease, biliary cirrhosis, infective endocarditis
  121. 121. Upper airway
  122. 122. General
  123. 123. Croup
  124. 124. Distinguish between viral and noninfectious croup
  125. 125. Viral/infectious croup
  126. 126. Usually parainfluenza
  127. 127. Low grade fever, rhinorrhea, mild cough followed 1-3 days later by a harsh, nonproductive “barking” cough and inspiratory stridor
  128. 128. Noninfectious/spasmodic croup
  129. 129. The cough is similar in that it is barking and nonproductive, but there is no fever, rhinorrhea or other sx of infection; possibly allergic
  130. 130. Know the appropriate management of croup
  131. 131. Mainstay = cool mist therapy
  132. 132. Some evidence that adding steroids to the mist can help decrease inflammation
  133. 133. Moderate to severe croup can benefit from heliox and also racemic epi
  134. 134. Know the clinical manifestations of laryngotracheobronchitis (croup)
  135. 135. See above for infectious croup
  136. 136. Epiglottitis
  137. 137. Know how to tx a child with it
  138. 138. Carefully
  139. 139. Don’t examine them invasively, don’t send them to radiology
  140. 140. Establish an airway (have anesthesia come ASAP) if looking crumpy
  141. 141. Give abx, maybe steroids
  142. 142. Differentiate the clinical and radiographic findings of viral croup from those of epiglottitis and bacterial tracheitis
  143. 143. Viral croup: cough, runny nose, possible prior low grade fever, don’t appear toxic
  144. 144. Epiglottitis: appear toxic, have inspiratory stridor, rarely a coughthumb sign; enlarged epiglottis protruding from the anterior wall of the hyopharynx
  145. 145. Bacterial tracheitis: toxic appearing, high fever, tachypnea, brassy cough; imaging = subglottic narrowing and a ragged tracheal air column
  146. 146. Know the risks of examination in a patient with suspected epiglottitis
  147. 147. Increased swelling and collapse of airway
  148. 148. Lower airway
  149. 149. Vascular anomalies
  150. 150. Recognize the variable presentation of vascular anomalies affecting the airway
  151. 151. Vascular rings and slings; often present with stridor while feeding; biphasic stridor
  152. 152. Congenital malformations
  153. 153. Recognize that congenital malformations of the lung (e.g. hypoplastic left lung, cystic adenomatoid malformation) may cause respiratory signs and symptoms
  154. 154. Pulmonary sequestration:
  155. 155. Intrapulmonary is most common (75-90% of cases); it presents later in life and is usually associated with recurrent unilateral pneumonias, cough, wheezing, fevers
  156. 156. Extrapulmonary are far less common and present in early infancy (prior to age 6 months) and in conjunction with other congenital abnormalities
  157. 157. Bronchogenic cysts are usually asx and found incidentally on cxr; sometimes present as airway compression or infection
  158. 158. Congenital cystic adenomatoid malformation (CCAM) is usually identified on prenatal ultrasound or present in the newborn period with respiratory distress; they may involve the entire lung, they may be associated with other abnormalities; if presenting later may do so with recurrent pneumonia, but would be earlier in life than a pulm sequestration
  159. 159. Bronchiolitis I think I’m all set with bronchiolitis, thanks.
  160. 160. Recognize the clinical manifestations of bronchiolitis
  161. 161. Know the indications for hospitalization for a child with bronchiolitis
  162. 162. Aspiration syndromes
  163. 163. Know how to eval for a suspected foreign body aspiration
  164. 164. See prior section
  165. 165. Know the long term complications for FB aspiration
  166. 166. Bacterial pneumonia os most common complication
  167. 167. Can also get atelectasis and bronchiectasis
  168. 168. Know the pulmonary complications of GER
  169. 169. Wheezing, scarring of vocal cords…
  170. 170. Know the possible radiographic manifestations of FB aspiration
  171. 171. Lung remains inflated on expiratory films, might see the foreign body, might see atelectasis, air trapping
  172. 172. Know that recurrent aspiration can recur with swallowing d/os independent of GER
  173. 173. Swallowing dysfunction can be a cause
  174. 174. Plan the management of a patient with FB aspiration
  175. 175. See above under the wheezing part
  176. 176. Understand that hydrocarbon pneumonitis may cause acute and chronic lung disease
  177. 177. Hydrocarbons are mineral spirits, gasoline, turpentine, pine oil
  178. 178. Acutely can cause aspiration, hypoxemia requiring O2 support, the resp sx usually resolve in 7 days
  179. 179. Chronically can cause penumatoceles, pneumothorax, empyema, bacterial pna, respiratory distress syndrome
  180. 180. May get proliferative alveolar thickening and that can cause permanent damage to pulm fxn
  181. 181. Know that aspiration can occur despite the presence of a tracheostomy
  182. 182. Bronchiectasis
  183. 183. Know the ddx of it
  184. 184. Bronchiectasis abnormal dilatation of the bronchi and bronchioles; may be localized or diffuse and the changes are irreversible
  185. 185. Most common cause is CF
  186. 186. Less common causes include allergic bronchopulmonary aspergillosis, primary ciliary dyskinesia, immunodeficiency and recurrent infections (TB, pertussis, measles); foreign body aspiration can be a cause as well
  187. 187. Know that high res CT of the chest is useful to dx it in a child
  188. 188. Tracheomalacia
  189. 189. Know that it can occur as a complication of chronic mechanical ventilation in children
  190. 190. High vent pressures can result in weakening or destruction of tracheal cartilage
  191. 191. Know that tracheoesophageal fistula may result in tracheomalacia
  192. 192. Can remain problematic despite repair of the fistula
  193. 193. Know the clinical manifestations of tracheomalacia and laryngomalacia
  194. 194. Laryngomalacia: most common cause of stridor in infants
  195. 195. Occurs during inspiration due to collapse of supraglottic structures unable to remain open during times of increased intrathoracic pressure
  196. 196. Exacerbated during times of crying or agitation; may be relieved when infant is in prone position
  197. 197. Usually resolve spontaneously in the first postnatal year; should also consider other problems like GER that can exacerbate it, and tx those comorbid conditions
  198. 198. Tracheomalacia
  199. 199. Occurs during expiration due to collapse trachea during expiratory maneuvers
  200. 200. Hallmark = expiratory wheeze
  201. 201. Can be isolated or in association with other clinical entities like TEF or trisomy 21; can be acquired due to vents
  202. 202. Differentiate between the two – see above
  203. 203. Tracheitis
  204. 204. Recognize the si/sx of bacterial tracheitis
  205. 205. toxic appearing, high fever, tachypnea, brassy cough; imaging = subglottic narrowing and a ragged tracheal air column
  206. 206. Know the clinical course of bacterial tracheitis, including biphasic illness, precipitous worsening, requirement for intubation, and relatively prolonged intubation
  207. 207. Often have sx of a croup-like illness for a few days and then a sudden deterioration with spiking fevers, increased hoarseness, toxic appearance, stridor, rtx and decreased O2 sats
  208. 208. Doesn’t respond to the usual txes for croup
  209. 209. Know the tx of it
  210. 210. Secure an airway; might need a prolonged intubation doe to need to get secrcxretions cleared and also due to degree of edema in the airway
  211. 211. Start abx including coverage for staph (nafcillin)
  212. 212. Know the microbiology of it
  213. 213. Most commonly due to s. aureus
  214. 214. Can also be due to parainfluenza, moraxella, nontypable h. flu and oral anaerobes
  215. 215. Hemosiderosis
  216. 216. Know that it is associated with hemoptysis
  217. 217. Parenchymal
  218. 218. Pneumonias
  219. 219. Know the etiologies of pna in children of different ages
  220. 220. AgePathogenComments3weeks to 3 monthsC. trachomatisRSVParainfluenzaS. pneumoBordatella pertussisVertical transmissionAfebrileInterstitial infiltrates on cxrBronchiolitis with wheezing most commonOnset usually late fallBronchiolitis or pneumoniaSeen fall through springMajor bacterial cause through childhoodTracheobronchitis with severe paroxysmal cough, no feverPneumonia occasionally seen; usually related to aspiration3 months to age 4 yrsRSV, parainfluenzae, human metapneumovirus, influenza, rhinovirusStrep pneumoMycoplasma pneumoniaMost toddler pneumonia is viralMajor treatable pathogen in this age groupPossible in all agesIncreased incidence in children approaching school age5yrs through adolescenceM pneumoniaC. pneumoniaS pneumoTBMajor treatable cause in school age children and adolescenceAlso an important cause, similar clinical presentation to mycoplasmaStill an important causeComplications, especially empyema, often ensuePrimarily in areas or populations of high TB prevalenceHigher risk at puberty and in pregnancy
  221. 221. Know the major acute and chronic complications of pneumonia, including empyema, sepsis, ptx, bronchopleural fistula, and pneumatoceles
  222. 222. Necrotizing pna: rare; liquefaction and necrosis of lung tissue due to toxins of highly virulent organisms
  223. 223. Lung abscess: usually develop after an aspiration event, frequently involve mouth organisms like streps, anaerobes, gram negative rods
  224. 224. Empyema: kids usually present wih persistent fever, decreased appetite, fatigue, chest pain and some degree of respiratory distress; usually requires surgical intervention of some kind (chest tube, VATS)
  225. 225. Bronchopleural fistula: development of a connection between the bronchial tree and the pleural space. Is associated with a pretty high morbidity and mortality (30-70%) and requires surgical repair. Usually seen in a very sick kids, if on a vent would likely have an air leak
  226. 226. Know the clinical manifestations of pnas with different etiologies
  227. 227. SyndromeTypcal causeAge groupTypical clinical featuresBacterial (suppurative)Pneumococcus, othersAll ages; younger children (<6mos) more commonAbrupt onset, high fever, ill/toxic appearance, more focal findings on exam, chest/abd pain, focal infiltrate if CXR is obtainedAtypical – infancyC. trachomatis<3mostachypnea, mild hypoxemia, lack of fever; wheezing, interstitial infiltrates on CXRAtypical – older childrenMycoplasma>5ygradual onset, low grade fever, diffuse exam findings, diffuse infiltrates if CXR obtainedViralMultiple All ages; 3mos – 5yrs more commonProminent URI sx, low grade or absent fever, diffuse findings/wheezes on exam, possible diffuse interstitial infintrates if CXR obtained
  228. 228. Know the lab tests for pneumonia, including x-ray study of the chest, blood cx, CBC, urine antigen detection studies, serology for mycoplasma, sputum cx
  229. 229. Rarely need lab if patient going to be managed as outpt
  230. 230. If being managed inpt the recs are for getting CBC, blood cx, chem panel; sputum cx of possible to test for bacterial pathogens; nasopharyngeal secretions can be tested for viral resp tract pathogens
  231. 231. Mycoplasma, chlamydophila and legionella can be determined serologically
  232. 232. Legionella and pneumococcal infections are detactable with urine antigen testing
  233. 233. Know the sequelae of pna and manage appropriately
  234. 234. ?
  235. 235. Know the methods of prevention and/or control of pna
  236. 236. Good hygiene
  237. 237. Breastfeeding
  238. 238. Immunization with Hib, pertussis and heptavalent pneumococcal vaccines
  239. 239. If kid is older than 2 and has risk facrots then needs the 23-valent pneumococcal polysaccharide vaccine
  240. 240. Flue shots
  241. 241. Know which organisms are likely to cause the pleural and parenchymal complications of pneumonia
  242. 242. Usually s. pneumo
  243. 243. GAS (strep pyogenes) can cause pneumatoceles
  244. 244. S. aureus can cause pneumatoceles, ptx, abscesses, empyema
  245. 245. Know that invasive studies (bronch, lung aspiration, open bx) may be indicated in pts with acute pna
  246. 246. Know the ddx of recurrent pna
  247. 247. Recurrent pna = more than one radiographically confirmed episode in a year, or more than three episodes in a lifetime, with clinical or radiographic resolution between episodes
  248. 248. Ddx includes anatomic lesions, i.e. vascular rings, cysts, pulmonary sequestration; resp tract disorders like CF, GER, aspiration, and immune disorders
  249. 249. Know that congenital lesions of the lung may mimic pna
  250. 250. Know the significance of pna in a child with NM dz
  251. 251. More likely to get aspiration pneumonia
  252. 252. Know the tx of pna in a child with NM dz
  253. 253. Cover for causes of aspiration pneumonia (ampicillin, ampicillin-sulbactam, clindamycin); probably needs to be admitted
  254. 254. Diaphragmatic hernia – covered in GI section
  255. 255. Recognize the clinical manifestations of it
  256. 256. Know the appropriate therapy
  257. 257. Know the initial stabilization maneuvers for a newborn infant with DH
  258. 258. Know that DH is associated with persistent pulmonary htn, and sybsequent abnormalities including poor growth, tracheomalacia and developmental delay
  259. 259. Know that pulm hypoplasia is associated with DH
  260. 260. Trauma
  261. 261. Know how to evaluate a child with sx following a chest wall trauma
  262. 262. Know how to stabilize a child with respiratory sx following a chest wall trauma
  263. 263. Drowning, near drowning, and acute respiratory distress syndrome
  264. 264. Know the clinical manifestations of ARDS
  265. 265. Tachypnea, decreased lung compliance, worsening hypoxemia leading to respiratory muscle fatigue
  266. 266. Disgnostic triad = noncardiogenic pulmonary edema, impaired oxygenation, bilateral pulmonary infiltrates
  267. 267. Know the natural hx of ARDS
  268. 268. Three stages:
  269. 269. Exudative stage – rapid development of respiratory failure; characterized by profound hypoxemia and atelectasis, sometimes resolves in 3-7 days, but can progress to
  270. 270. Inflammatory stage – fibrosing alveolitis and surfactant deficiency; characterized by persistent hypoxemia, decreased lung compliance, and development of pneumothoraces
  271. 271. Resolution – may eventually recover normal lung function; final stage consists of resolution of hypoxemia and improved lung compliance;c omplete resolution may take 6-12 months but some develop lifelong restrictive lung disease, lung cysts, or decreased exercise tolerance
  272. 272. Know the pulmonary sequelae of ARDS
  273. 273. Pulmonary fibrosis
  274. 274. Reactive airway disease
  275. 275. Severe chronic lung disease (rare)
  276. 276. Know that ARDS has multiple etiologies
  277. 277. Know that a pt with minimal sx following a near drowning may later develop sx that require hospitalization
  278. 278. Know that ARDS may result from a near drowning after a period of initial recovery
  279. 279. Know the major causes of death in children with ARDS: sepsis, extrapulmonary multiorgan failure, air leaks
  280. 280. Newborn infants
  281. 281. Bronchopulmonary dysplasia (chronic lung disease of infants)
  282. 282. Recognize that infants with BPD are prone to cor pulmonale and recurrent wheezing with infections
  283. 283. Recognize that failure to thrive and severe respiratory infections are common in infants with BPD
  284. 284. Recognize that aversive oral motor behavior is associated with BPD, thereby limiting ways to feed such infants
  285. 285. Other – not BPD
  286. 286. Know the specific radiographic findings in idiopathic neonatal RDS (see below)
  287. 287. Differentiate between normal results of a newborn cxr and the radiographic findings that reflect MAS (see below)
  288. 288. Differentiate between the normal results of a newborn cxr and the radiographic patterns that reflect pna
  289. 289. RDS: underinflation, “ground glass” appearance, air bronchograms
  290. 290. TTN: fluid in horizontal fissure, adequate lung expansion with perihilar and central vascular prominence
  291. 291. MAS: patchy areas of diffuse atelectasis, focal areas of air trapping, increased lung volumes
  292. 292. Pna:
  293. 293. Recognize that CLD may result from MAS
  294. 294. Asthma
  295. 295. CF
  296. 296. Know which vitamins are fat soluble and therefore are usually insufficient in children with CF
  297. 297. Understand the inheritance of CF
  298. 298. Autosomal recessive
  299. 299. Must have two mutations on both copies of the CFTR gene in order to be affected
  300. 300. For a healthy person whose parents are known carriers, the risk of being a carrier of a mutated CFTR gene is 2/3
  301. 301. Of the 4 possible outcomes from that person’s parents, one in four would have two mutated genes, and hence not be “healthy” so they are taken out of the equation on this tricky little scenario (question 2010: 151)
  302. 302. Know the association of rectal prolapse and CF
  303. 303. Accounts for about 10% of all reported cases of RP during infancy and early childhood
  304. 304. RP occurs in about 20% of patients with CF between age 6months and 3 years
  305. 305. This was in question 2010: 225, and the scenario was of a child whose weight/height were mismatched, had chronic constipations, and experienced the rectal prolapse after being txed for constipation and passing soft stools – these were supposed the be the CF “red flags”
  306. 306. Know the neonatal non-pulmonary manifestations of CF: meconium ileus, meconium peritonitis, prolonged jaundice
  307. 307. Mec ileus thick mec obstruction in the distal ileum, usually presents as a small bowel obstruction; consider in newborn with delayed passage of stool and bile stained emesis; abd film may show stacked loops of variably dilated bowel, soap suds bubbly like appearance of the mec (often in the RLQ); distal bowel with paucity of bowel gas
  308. 308. Mec peritonitis bowel obstruction leading to perf and spillage of mec into the abd cavity; it often calcifies and forms a “pseudocyst” that can be seen on abd films
  309. 309. Prolonged jaundice
  310. 310. Know the manifestations of CF in infancy: hypoproteinemia, anemia, steatorrhea, recurrent pulmonary sx, hypochloremic alkalosis
  311. 311. Know that hemoptysis and ptx can be potentially life threatening complications of CF
  312. 312. Primary ciliary dyskinesia (dysmotile cilia syndrome)
  313. 313. Know that otitis media, dextrocardia, and/or bronchiectasis may be due to PCD
  314. 314. Note that in question 2007:143 the vignette was a boy who had chronic infections, cludding, cough, nasal polyps, rhinorrhea and a “point of maximal impulse displaced t the right” this was supposed to be the clue to dextrocardia and therefore the PCD rather than CF
  315. 315. Extrapulmonary
  316. 316. Pleural fluid
  317. 317. Understand the etiologies of pleural fluid accumulations
  318. 318. Transudates = plasma ultrafiltrates that usually result from renal and liver dz or CHF
  319. 319. Exudates = inflammatory processes, like pna, malignancy, trauma, systemic inflammatory dz, impaired lymphatic drainage
  320. 320. Empyema is a type of exudate and is characterized by the presence of WBCs, a positive gram stain, or frank pus
  321. 321. Exudates have LOW pH (b/c of bacterial glucose consumption)LDH will be 2-3x the serum values, and high protein vaues (>3g/dL)
  322. 322. 70% of peduatric pleural effusion are parapneumonic effusions
  323. 323. Pleural effusions are seen in up to 40% af bacterial pnas, and about 50% of those will progress to empyema
  324. 324. Know the characteristics of pleural fluid due to chylothorax
  325. 325. Pneumothorax, pneumomediastinum
  326. 326. Know the si/sx of ptx
  327. 327. Know the appropriate tx for a child with ptx
  328. 328. Know that asthma may be associated with either of these
  329. 329. Know that ptx is a complication of rescusitation and mechanical ventilation
  330. 330. Thoracic deformities
  331. 331. Recognize the association between scoliosis and restrictive pulmonary disease
  332. 332. Scoliosis can restrict the thoracic cavity, thereby causing a restrictive lung dz
  333. 333. Recognize that severe progressive neuromuscular disease of any etiology can produce serious restrictive pulmonary disease
  334. 334. Recognize that pectus excavatum is not usually associated with any pulmonary disease or exercise limitation
  335. 335. Pulmonary htn and cor pulmonale
  336. 336. Know that oxygenation may decrease during normal abnormal sleep, which may cause pulmonary hypertension or exacerbate existing cor pulmonale
  337. 337. Know that PHTN is potentially reversible
  338. 338. Treatment is aimed at the underlying disease in cases of secondary PH
  339. 339. Treatment is sx management and treatment of pulm vasculopathy in idiopathic PH
  340. 340. Know the situations in which PHTN and or pulmonale may occur
  341. 341. PPHN
  342. 342. CHD
  343. 343. Lung disease that causes chronic hypoxia (leads to remodeling of the pulmonary vascular wall)
  344. 344. Thrombotic or embolic disease
  345. 345. Idiopathic
  346. 346. Sleep disorders (see prior sections on OSAS, also, its kind of common sense)
  347. 347. Know the respiratory and non-respiratory conditions that may cause sleep disorders
  348. 348. Understand the sx that reflect poor sleep quality in children
  349. 349. Know the appropriate eval of suspected OSA in children
  350. 350. Know that children with severe OSA due to upper airways obstruction are at significant risk for resp distress postoperatively (due to post op airway selling, post op obstructive pulmonary edema)
  351. 351. SIDS
  352. 352. Recognize a child with an ALTE
  353. 353. ALTE is an episode that is frightening to the observer and that is characterized by some combination of apnea (centrally or occasionally obstructive), color change (usually cyanotic or pallid but occasionally erythematous or plethoric), marked change in muscle tone (usually marked limpness), choking or gagging
  354. 354. Know the appropriate management of a child with ALTE
  355. 355. Get a good hx, full PE to assess the severity of the event
  356. 356. Article recommends screening for GER, UA and cx, brain neuroimaging, pneumogram, and WBC count
  357. 357. Know the risk factors for SIDS- got it.
  358. 358. Know the ddx of ALTE in infants includes infection, metabolic abnormality, GER, aspiration, cardiac dysrhythmia, seizures, nonaccidental trauma, apnea of infancy
  359. 359. Recognize the limitation of apnea monitors in following infants with ALTEs
  360. 360. Diagnostic testing – prior sections pretty much cover this
  361. 361. PFTs
  362. 362. Know what spirometry measures
  363. 363. Oximetry
  364. 364. Know the correlation between PaO2 and oxyhemoglobin saturation
  365. 365. Understand the value and limitations of pulse ox in caring for children with acute pulmonary dz
  366. 366. Blood gas analysis
  367. 367. Recognize the limitations of cap blood gas testing
  368. 368. Imaging
  369. 369. Recognize intrathoracic airway obstruction by x-ray study of the chest
  370. 370. Recognize atelectasis by x-ray study of the chest