key: cord-0037145-wc3mw30l authors: Hardy, Karen; Naga, Osama title: Respiratory Disorders date: 2015-03-28 journal: Pediatric Board Study Guide DOI: 10.1007/978-3-319-10115-6_14 sha: c20d8b30c563f2fb4985421eca71d8a0389155bd doc_id: 37145 cord_uid: wc3mw30l This chapter provides the reader with a concise outline of the topics required for general pediatric board examination, respiratory component. Basic diagnostic testing is reviewed. Common upper airway problems, lower airway issues, and parenchymal diseases are covered. Congenital malformations and common diseases of the lung are reviewed. The physiology of extrapulmonary problems is reviewed. Sleep disordered breathing and the evaluation of apneas and ALTE/SIDS are also discussed. Hints regarding physiology, clinical features, diagnostic testing, and management are present with references to national guidelines and resources. − Oxygen saturation shifts to the left with alkalosis, hypocarbia and hypothermia, shifting to the right with increased temperature, acidosis, hypercarbia and increased 2,3 DPG. A shift to the left means oxygen binds more avidly to the hemoglobin, to the right less so. • Oximetry readings are affected by dyes, nail polish, highintensity light, impaired perfusion, artificial nails, methemoglobin, and carboxyhemoglobin. It is less accurate at low saturations or with motion. • SpO2 and PaO2 are not equal. It is important to obtain a blood gas to understand acid-base balance and carbon dioxide as well as saturation in sick patients, especially those supported on supplemental oxygen which can improve saturation and falsely reassure a caregiver. • Capillary blood gas can give representation of arterial sample if heel is adequately warmed and perfusion is excellent. • Blood gases will be inaccurate if permitted to remain warm outside of the body and delays in reading occur since WBC metabolism will continue consuming oxygen and generating waste products leading to increased acidosis. − mSv is a milliSievert unit which reflects the ionizing radiation from plain films, fluoroscopy, and CT imaging − A single chest X-ray (CXR) provides 0.1 mSv = background radiation on the earth in 10 days time − A standard helical CT of newborn chest 1.7 mSv to 5.4 mSv in an adult-sized teenager • Low-dose protocols can halve these numbers • Ultra-low dose protocols are as low as 0.14 mSv and useful for children likely to require multiple CT during a lifetime • Suggested modalities for various issues -Plain CXR: suspected vascular ring, pneumonia -Plain expiratory or decubitus views: suspected foreign body -Plain decubitus: pleural fluid, pneumothorax -Virtual bronchoscopy: persistent anomalies of tracheobronchial tree -Ultrasound: pleural effusion, complicated pneumonia -CT scan: chest wall structure, lung abscess, airspace disease, bronchiectasis, anterior or middle mediastinal masses, complicated pneumonia, asthma complications -PET scan: anterior, middle mediastinal masses -MRI: posterior mediastinal mass, pulmonary vascular bed, rings, new programs to better visualize the lung and spare radiation being evaluated Upper airway obstruction • Early increase in PCO2 and proportionate decrease in PO2 and responds well to supplemental oxygen initially Intrapulmonary airway obstruction • Mild: decrease PCO2, normal to decreased PO2 • Moderate: Normal PCO2, decrease PO2 moving toward failure • Severe: increase PCO2 and decrease PO2 • Supplemental oxygen will support patient, but imperative to monitor carbon dioxide as well Alveolar interstitial pathology • Diffusion defects occur and cause poor transit of oxygen with desaturation first at exercise and then also at rest R-L shunt • Early decrease in PO2 • Normal or low PCO2, high PCO2 if fatigue develop • Testing with 100 % oxygen helps to define this issue -Response to supplemental oxygen is fair to poor depending on shunt volume • Stridor is an abnormal, high-pitched sound. • Produced by turbulent airflow through a partially obstructed airway. • The timing of the sound can help to localize the narrowing. Because the extrathoracic airway collapses with inspiration any swelling/obstruction in this location will cause inspiratory stridor. Glottic obstruction/right at the cords will produce a to-and-fro noise. Subglottic will produce noise first on exhalation. • Could be supraglottic, glottic subglottic, and/or trachea. • Foreign body aspiration (see amplified discussion) • Bacterial tracheitis (see amplified discussion) • Retropharyngeal abscess -Children younger than 6 years -Abrupt onset of high fevers, difficulty swallowing, refusal to feed, sore throat, hyperextension of the neck, and respiratory distress • Peritonsillar abscess -Adolescents and preadolescents -Severe throat pain, trismus, and trouble swallowing or speaking • Spasmodic croup, also termed acute spasmodic laryngitis -Occurs most commonly in children aged 1-3 years -May be associated with GI reflux • Allergic reaction or anaphylaxis -History of allergy -Other organ involvement, e.g., itchiness or hives • Epiglottitis (see amplified discussion) -Background • The most common cause of inspiratory stridor in the neonatal period and early infancy • Accounts for up to 75 % of all cases of stridor -Clinical presentation • Exacerbated by crying or feeding • Placing the patient in a prone position with the head elevated improves the stridor • Supine position worsens the stridor -Diagnosis • Flexible laryngoscopy can confirm the diagnosis but may miss tracheal abnormalities • If moderate to severe obstruction, difficulty in feeding and breathing, unable to gain weight then use flexible bronchoscopy to r/o other associated airway anomalies -Management • Laryngomalacia is usually benign and self-limiting and improves as the child reaches age 1-2 years • Careful observation and growth monitoring for most patients • Surgical correction or supraglottoplasty may be considered in severe cases • Vocal cord abnormalities -The second most common cause of stridor in infants -Unilateral vocal cord paralysis can be congenital or secondary to birth or surgical trauma, such as cardiothoracic surgery. -Patients with a unilateral vocal cord paralysis present with a weak cry and biphasic stridor that is louder when awake and improves when lying with the affected side down. • Brassy and barking cough, similar to croup but the patient has high fever and looks very toxic, with respiratory distress and stridor. • Patient may lie flat and does not have drooling or dysphagia associated with epiglottitis. • Rapid progression and purulent secretion to obstruct airway may mandate early endotracheal intubation. • Failure to respond to racemic epinephrine or corticosteroids. • Intubation especially younger patients; 50-60 % do not need intubation. • High fever, purulent airway secretions, absence of finding in epiglottitis. • X-ray is not needed, but may show the classic finding of pseudomembrane detachment in the trachea. • Humidification and careful suctioning of the ET Tube are important. • Antistaphylococcal treatment, e.g., nafcillin or vancomycin. • Prognosis is excellent. • Complications can include toxic shock, septic shock, pulmonary edema, ARDS, and subglottic stenosis. General considerations • Change in color or consistency in nasal secretions is common during the course of illness does not indicate sinusitis. • Presence of polymorphonuclear leukocytes in nasal secretions does not indicate bacterial superinfection. • Bacterial culture is indicated only if Group A streptococcus, Bordetella pertussis, or nasal diphtheria is suspected. • Codeine, dextromethorphan hydrobromide, has no effect on cough from cold. • Guaifenesin is not an antitussive agent. • First-generation antihistamine reduces rhinorrhea by 25-30 %, via its anticholinergic effect; therefore, using second-generation antihistamine is not helpful. • Allergic rhinitis-prominent itching and sneezing, and nasal eosinophils (Nasal smear may be useful if allergic rhinitis is suspected) • Nasal foreign body-unilateral foul-smelling secretions, and bloody nasal secretions • Sinusitis-presence of fever, headache, facial pain, periorbital edema, persistence of rhinorrhea > 14 days • Streptococcosis-nasal discharge that excoriates the nares • Pertussis-viral prodrome with prolonged persistent staccato cough • Congenital syphilis-persistent rhinorrhea with onset in the first 3 months of life Background • Viral bronchiolitis is the most common lower respiratory tract infection in infants and children who are 2 years of age and younger. • Respiratory syncytial virus (RSV) responsible for more than 50 % of acute bronchiolitis. • Other causes: human metapneumovirus, parainfluenza virus, adenovirus, influenza, rhinovirus, and mycoplasma. • Maternal asthma • Maternal smoking • Persistent rhinitis • Eczema at < 1 year of age • Nasal congestion, rhinorrhea, and cough. • Tachypnea or elevated respiratory rate is the earliest and most sensitive vital sign change. • Nasal flaring; grunting; and suprasternal, intercostal, and subcostal retractions demonstrate increased respiratory effort. • Nasal suctioning and repositioning may allow a more accurate assessment of lower respiratory tract involvement. • Crackles, wheezes, and referred upper airway noise are commonly auscultated sounds. • Apnea may be prominent than wheezing early in very young infants < 2 months or former premature infants. • Bronchiolitis can range from mild tachypnea to impending respiratory failure. • Patients can be expected to have worsening clinical symptoms, with peak symptomatology around day 3-4 of illness "Day of illness". • "Day of illness" is an important variable in providing anticipatory guidance for outpatient management and in making decisions regarding admission and discharge of patients. • Clinical features lead to diagnosis; subsequent evaluation important to determine treatment. • Initial step is an evaluation of respiratory rate and oxygen saturation. • CXR is warranted for any infants with respiratory distress. • Common radiological findings include hyperinflation, areas of atelectasis, and infiltrates. • Because of the risk of serious bacterial infection (SBI) among infants 30 days of age or younger, they should receive conservative management for fever, including full evaluation for SBI and administration of empiric antibiotics. • Recognition that infants older than 30 days who have clinical bronchiolitis are at a lower risk for SBIs may allow for decreased invasive testing and observation without administering antibiotics to patients who have classic presentations. • Hyperinflation and atelectasis are common in acute bronchiolitis. • Respiratory rate, work of breathing, and hypoxia are the most clinically significant parameters in determining illness severity and should be assessed routinely in all patients who have bronchiolitis. • Mainstay of treatment is supportive, oxygen if hypoxia, hydration, frequent nasal suctioning, position to elevate chest 30°. • Oxygen should be discontinued once pulse oximetry saturations rise to between 90 and 92 % for most of the time and the patient is demonstrating overall clinical improvement, as evidenced by adequate feeding and improved work of breathing. • Infants with respiratory distress and desaturation or dehydration should be hospitalized. • The American Academy of Pediatrics (AAP) does not recommend the use of bronchodilators or systemic steroids in the routine treatment of bronchiolitis. • Those with recurrent wheezing may respond to bronchodilator therapy. • Corticosteroid medications, inhaled or administered systemically, should not be used in the treatment of bronchiolitis. • If bronchodilator makes the wheezing worse discontinue and consider pulmonary consultation for tracheo or bronchomalacia. • Sweat chloride test for patient with recurrent wheezing and resistant to treatment. • Ribavirin should not be used routinely in the treatment of bronchiolitis. • Synagis 15 mg/kg IM for prematures and high-risk infants as monthly IM monoclonal antibody injection. • Hand washing is the best measure to prevent nosocomial infection. • Once asthma has been diagnosed, the physician should determine the degree of severity in the individual patient. • Severity is determined best at the time of diagnosis, before initiation of therapy. • Respiratory infections (most common trigger). • Allergens, airway irritants (e.g., environmental tobacco smoke and air pollution), exercise. • Medications (e.g., nonsteroidal anti-inflammatory medications and beta blockers). • Exposure to environmental tobacco smoke. • Common indoor allergens include house dust mite, cockroach allergen, animal dander, and molds. • Prick skin testing or blood testing (allergen-specific immunoglobulin E [IgE] concentrations) to detect sensitization to common indoor allergens should be considered for any child experiencing persistent asthma. • Wheezing -A musical, high-pitched whistling sound produced by airflow turbulence. -It is one of the most common symptoms of asthma. -Adolescents may not have these symptoms until they are in frank respiratory failure. • Pulmonary function tests: -Spirometry: obstructive pattern with response to bronchodilators. Plethysmography: may have air trapping with increased RV/TLC ratio • Exercise challenge -Involves baseline spirometry followed by exercise on a treadmill -Bicycle to a heart rate greater than 60 % of the predicted maximum, with monitoring of the electrocardiogram and oxyhemoglobin saturation -Repeat spirometry documenting drop in airflow rates • Radiography -May reveal hyperinflation and increased bronchial markings; radiography may also show evidence of parenchymal disease, atelectasis, pneumonia, congenital anomaly, or a foreign body • Allergy testing: -Can identify allergic factors that may significantly contribute to asthma Exercise-induced asthma • Shortness of breath along with coughing or wheezing during physical exertion can be a symptom of poorly controlled asthma. • Some patients experience symptoms associated with bronchoconstriction only with exercise and otherwise have no history consistent with asthma. • Symptoms typically start within few minutes of initiation of vigorous exercise and subside within 20-30 min, although they can last up to 90 min when left untreated. • Usually self-limited but rare cases of severe attacks and even death have been reported. • Depending on the age and cognitive ability of the child it may be difficult to obtain spirometry data and the diagnosis largely based on clinical presentation and response to therapy. • Differential diagnosis of asthma (Table 1 ) • Assessment and monitoring: in order to assess asthma control and adjust therapy, impairment and risk must be assessed; because asthma varies over time, follow-up every 2-6 weeks is initially necessary (when gaining control of the disease), and then every 1-6 months thereafter. • Education: self-management education should focus on teaching patients the importance of recognizing their own level of control and signs of progressively worsening asthma symptoms. • Educational strategies should also focus on environmental control and avoidance strategies, as well as on medication use and adherence (e.g., correct inhaler techniques and use of other devices) (Fig. 2 ). • Control of environmental factors and comorbid conditions. • Long-term control medications depend on severity of asthma. Step 3: Medium-dose ICS and consider short-course OCS OCS oral corticosteroids, LABA long-acting beta2 agonist, SABA short-acting beta2 agonist, FEV1 forced expiratory volume in 1 s, FVC forced vital capacity, ICS inhaled corticosteroid • Inhaled corticosteroids are the most commonly prescribed maintenance therapy for asthma. • They effectively decrease airway inflammation, decrease bronchial hypersensitiveness, relieve asthma symptoms, and improve lung function. • Slowing growth and adrenal suppression is a risk in the patients who require high-dose ICSs. • To minimize the risk of adverse effects, eliminate triggers that contribute to airway inflammation. • They block inflammatory pathways that are active in the disease. • Most commonly used in children younger than 12 years is montelukast. • It is usually well tolerated. • Montelukast frequently used as add-on therapy in addition to ICSs. • Can be beneficial in patients with comorbid allergic rhinitis, recurrent viral-induced asthma exacerbation, and children with exercise-induced asthma. • Children at significant risk of having asthma symptoms later in life − Children with early onset asthma < 3 years of age: • Who had three or more episode of wheezing per year and at least one major criterion (Eczema or parental eczema). • Or at least two minor criteria (allergic rhinitis, wheezing unrelated to colds, or blood eosinophil count > 4 %). • Infection of lung parenchyma • Absence of fever, tachypnea, increased work of breathing, and auscultatory abnormalities, bacterial pneumonia is unlikely. • Typically clinical due to above. • Rapid influenza test may help to identify the cause of fever and to reduce the subsequent use of antibacterial agents. • CBC, chemistries, or serology will not help to identify the cause or aid in management. • Blood culture rarely helpful (10 % of the time organism are recovered). • Erythrocyte sedimentation rate and C-reactive protein determinations may be elevated. • Chest X-ray (Fig. 3) -A chest radiograph will not change clinical management for most children who are being treated as outpatients. • Normal fluid balance -0.1-0.2 ml/kg of sterile colorless fluid -Ninety percent filters from arterial capillaries, reabsorbed at venous capillaries -About 10 % returned via lymphatic channels • Effusions -> 10 ml of fluid in thoracic cavity -Due to excessive filtration or defective absorption -Transudates: low protein, lactate dehydrogenase (LDH), -Exudates: • Pleural fluid-to-serum protein ratio is 0.5 or greater, • Pleural fluid-to-serum LDH ratio is more than 0.6, • Pleural fluid LDH concentration is more than 66 % of the upper limit of normal for serum • Should be suspected in any child with worsening pneumonia • Respiratory distress, tachypnea, pain with pleural inflammation, cough • Decreased to absent breath sounds, pleural rub if smaller collection of fluid • Egophony • Dullness to percussion • Midline shift • CXR: opacification of the thorax, blunted costophrenic angle. • Decubitus views helpful if fluid is free flowing. • Ultrasound is helpful to determine the presence or absence of loculations. • CT scan is helpful to define pulmonary and fluid characteristics for complicated effusions/empyemas • Thoracentesis is helpful to relieve dyspnea for large effusions and determine characteristics of the fluid for treatment of underlying cause. • Oxygen for hypoxemia. • Consultation with experts as needed. • Possible causes: infection, chyle, blood, malignancy, and drug exposures. • In the neonatal period, chylothorax is the most frequent type of pleural effusion. • Primary spontaneous pneumothorax -Occurs without trauma or underlying cause -More frequently in tall, thin male, thought to have subpleural bleb -Family history is positive in many patients • Secondary pneumothorax -Underlying lung disease -Trauma -Loud music (air pressure) -Catamenial pneumothorax (unusual condition associated with menses due to passage of intra abdominal air through a diaphragmatic defect) • The onset is abrupt, and the severity depends on lung collapse. • In simple pneumothorax, the lung collapses up to 30 %. • In tension pneumothorax, the patient will be hypoxemic dyspneic, and cyanotic. • PMI shifts due to displacement of intrathoracic organs to opposite side. • CXR. • Expiratory film accentuates the contrast between lung marking and the clear area of pneumothorax. • A small pneumothorax < 5 % may resolve spontaneously. • If > 5 % of pneumothorax or collapse, or if pneumothorax is recurrent or under tension, chest tube drainage is necessary. • Pneumothoraces complicating CF frequently recur and definitive treatment may be justified with the first episode. • Sclerosing with doxycycline (chemical pleurodesis). • Video assisted thoracic surgery is preferred therapy for blebectomy, pleural stripping, pleural brushing, and instillation of sclerosing agents over open thoracotomy. • Extensive pleural adhesion and aggressive pleural stripping may interfere with lung transplant in the future; these options must be discussed with the family. General issues • Small volume > 0.8 ml/kg and/or PH < 2.5 can cause hemorrhagic pneumonitis and atelectasis; large volume can cause pulmonary edema. • Most clinical changes appear within minutes to 1-2 h after aspiration event, radiographic change/infiltrate within 1-2 days unless large volume. • Immediate suctioning of airway (do not attempt to neutralize acid). • Image chest. • Intubation and mechanical ventilation in severe cases. • Antibiotics may be used to cover anaerobes if definitive aspiration and usually only if admitted. • If the CXR is clear and patient is asymptomatic can be observed in the hospital or the office for few hours then home observation. • Most dangerous is hydrocarbon: patient may deteriorate impressively though may be minimally or asymptomatic initially. Prolonged observation (minimum 8 h) in setting able to manage respiratory failure is optimal. • Gastric emptying is contraindicated in hydrocarbon aspiration. • Nuts especially peanuts are one 1/3 of cases. • Round globular FB, e.g., hotdog, grape, nuts, and candies are the most frequent offender to cause complete obstruction. Hotdogs are rarely seen as airway FB, because most of victims asphyxiate on the scene unless treated immediately. • Age < 3 years of age. • Initial event: violent paroxysms of coughing, choking, gagging, possible airway obstruction if the FB aspirated. • Asymptomatic interval: FB become lodged, reflexes fatigue, the immediate irritation subsides; this stage is the most dangerous, and account for most of delayed diagnosis, during the second stage (asymptomatic interval) that the physician may minimize the possibility of an FB accident, being reassured by absence of symptoms that no FB is present. • Positive history must never be ignored. • Negative history may be misleading. • Choking or coughing episode accompanied by wheezing are highly suggestive FB in airway • Physician should question parents about nuts, small toys, or anything similar. • 58% lodge in the right bronchus. • CXR is negative in 10-30 % of cases. • Patients suspected of having airway foreign bodies should undergo chest radiography. • The lack of radiological findings can never be used to exclude an airway foreign body; most objects are organic and likely to be radiolucent. • Positive findings on radiography can include hyperinflation, atelectasis, or infiltrate. • Inspiratory/expiratory or decubitus films may be helpful, although reports of sensitivity and specificity vary. • Soft-tissue films of the neck can be beneficial for detecting objects in the upper airway. • Patients with tracheostomy are at a higher risk. • Treatment of choice prompt removal with rigid bronchoscopy. • Bronchoscopy can be deferred until proper hydration, emptying the stomach. • Retained foreign body is associated with bronchiectasis, hemoptysis and lung abscess. Background • Cystic area due to necrotic lung tissue at least 2 cm in diameter. • Primarily due to aspiration or infection. • Secondarily related to predisposing condition cavitary lesion, dysphagia, developmental delays and poor airway protective reflexes and/or poor airway clearance from neuromuscular weakness. • Both aerobic and anaerobic are common causes. • Anaerobic, e.g., bacteroides, fusobacterium, and aerobic Staphylococcus aureus. • PCD is an autosomal recessive disease with extensive genetic heterogeneity. • Sixty percent of patients have identifiable mutations documented. • Abnormal ciliary motion and impaired mucociliary clearance. • Ultrastructural and functional defects of cilia result in the lack of effective ciliary motility. • In 50 % of the patients, PCD is associated with partial or complete Situs inversus. • Male infertility. • Some patients have asplenia or polysplenia with immune dysfunction. • Hundred percent of children have productive cough, sinusitis, and otitis media. • Chronic or recurring upper and lower respiratory infection. • Recurrent otitis media, otorrhea, may begin in neonates. • Lower lobe bronchiectasis, and frequent wheezing and diagnosed as asthma. • The gold standard test is documentation of abnormal cilia ultrastructure (absent, abnormal dynein arms, radial spokes, doublet arrangements) on nasal and bronchial biopsies or scraping viewed on electron microscope. • CF is the most common cause of bronchiectasis in the children of the USA. • Impaired mucociliary clearance (CF and ciliary dyskinesia). • Infections (especially M Tb, Pseudomonas, adenovirus). • Immunodeficiency syndromes (humoral and cellular). • Immune mediated (connective tissues diseases, ABPA, IBD). • Airway injury (aspiration, inhalation of toxic fumes, hot gases). • Congenital or connective tissues abnormalities (yellow nail, Marfan, alpha 1 antitrypsin deficiency, airway cartilage deficiency, tracheobronchomegaly, young syndrome). • Obstructed airways (retained foreign body, intraluminal masses, and extraluminal compression). • Productive cough is the most common symptom of bronchiectasis. • Dyspnea, rhinosinusitis, and hemoptysis are less common. • Crackles, wheezing, and rhonchi; digital clubbing may also be present. • Pulmonary function testing may show obstruction, restriction, and combinations depending on etiology. • Chest radiograph may reveal airway dilation, increased pulmonary markings with tram tracking (thickening of the bronchial walls), and areas of atelectasis. • (High resolution) HRCT scan is the gold standard for diagnosis and reveals detailed anatomy of the bronchial tree. • Lack of airway tapering with luminal dilation, bronchial wall thickening, honeycombing, and mucus plugging. • Establishing the primary cause is of critical importance and is best undertaken with direction from a pediatric pulmonologist. • Mucus clearance may be enhanced with hypertonic saline nebulization, inhaled mucolytics, and chest physiotherapy. • Inhaled corticosteroids can reduce airway obstruction. • Chronic macrolide therapy has also been found to be beneficial as anti-inflammatory. • Aggressive treatment of pseudomonal and Staphylococcal infections is indicated, but antimicrobial therapy should be targeted to specific pathogens. • Lobectomy is a last resort in refractory cases without systemic etiology. • Recognized since 1960 following invention of positive pressure ventilation for premature infants and their survival. • "Old BPD" in late preterm infants with aggressive ventilation causing significant cystic disease. • "New BPD" is a chronic lung disease of extreme prematurity; lung immaturity typically associated with prolonged ventilation. • Volutrauma and barotrauma from positive pressure ventilation • Premature or immature lung • Inflammatory response to lung injury -Chorioamnionitis and ureaplasma associated with increased incidence of BPD • Repeated episodes of intra-alveolar bleeding that lead to abnormal accumulation of iron as hemosiderin in alveolar macrophages. • Subsequent development of pulmonary fibrosis and severe anemia. • Idiopathic pulmonary alveolar hemosiderosis (IPH) • Secondary pulmonary hemosiderosis -Cardiovascular: -Congestive heart failure -Pulmonary hypertension -Mitral valve stenosis -Inflammatory/autoimmune -Goodpasture syndrome -Rheumatoid arthritis -Wegener granulomatosis -HSP -Allergic -Heiner syndrome (cow's milk hypersensitivity) • Iron deficiency. • Hemoptysis (helpful if occurs). • Alveolar infiltrate. • Presence of hemosiderin, it takes 48-72 h for macrophages to convert erythrocyte to hemosiderin • Widely variable from asymptomatic to shock and sudden death. • After episode of hemorrhage, the patient will present with wheezing, cough, dyspnea, bronchospasm, and alteration of blood gases. • Best guided by consulting pulmonologist • Recurrent "pneumonia" fever, cough, abnormal chest radiograph • Hypochromic microcytic anemia • Elevation of plasma bilirubin • Infiltrate typically bilateral, and may spare the apices, often with hyperaeration • Ig E, cow's milk antibody levels, stool specimen for heme • Urinalysis for nephritis • ANCA, ANA, Anti-GBM • Lung biopsy if diffuse alveolar hemorrhage (DAH) • Corticosteroid is the treatment of choice for IPH. • Highly dependent on the underlying cause. • Sarcoidosis is a noncaseating granuloma multisystem disease. • More common in African Americans. • Approximately 5 % of cases are asymptomatic and incidentally detected by chest radiography. • Systemic complaints (fever, anorexia): 45 % of cases. • Dyspnea on exertion, cough, chest pain, and hemoptysis (rare)-occur in 50 % of cases. • Crackles may be audible. • Anterior or posterior granulomatous uveitis (most frequent). • Erythema nodosum. • Chest radiography is bilateral hilar or mediastinal adenopathy -Stage 0: normal chest radiographic findings -Stage I: bilateral hilar lymphadenopathy -Stage II: bilateral hilar lymphadenopathy and infiltrates -Stage III: infiltrates alone -Stage IV: fibrosis • PFTs may either be normal or show restrictive +/− obstructive mechanics. • Hypercalcemia (about 10-13 % of patients) • Hypercalciuria (about one third of patients) • Elevated alkaline phosphatase level • Elevated angiotensin-converting enzyme (ACE) levels • Asymptomatic patients may not require treatment. • In patients with minimal symptoms, serial reevaluation is prudent. • Treatment is indicated for patients with significant respiratory symptoms. • Corticosteroids can produce small improvements in the functional vital capacity and in the radiographic appearance in patients with more severe stage II and III disease. • The most common life shortening autosomal recessive disease due to mutation on the long arm of chromosome 7. • Highest incidence in Caucasians, highly prevalent in Latinos, African Americans and seen rarely in African, Asian, and Native Americans races. • > 1500 CF transmembrane regulator (CFTR protein) polymorphisms are associated with CF. • The most prevalent mutation is F508 deletion (85 % of US population have at least one copy) associated with both pulmonary disease and pancreatic insufficiency. • Different classes of gene mutation are identified each with different level of CFTR production and function. • CFTR dysfunction/absence is associated with excessive reabsorption of sodium and deficient chloride secretion. The passive movement of water is decreased and airway secretions are dehydrated with very low surface liquid layer. Cilia become compressed inhibiting ciliary clearance and cough clearance, bacteria thrive; immune function is also abnormal at the airway surface. Repeated and chronic infection leads to airway damage and bronchiectasis in the lung and dysfunction of other organs. • Four organ systems prominently involved, respiratory, GI, GU, and integumentary (sweat glands). • Pulmonary: -Cough is the most constant symptom dry at times, frequently productive. -Increased anteroposterior diameter of the chest. -Hyperresonance, scattered, and localized crackles. -Clubbing, cyanosis, acute sinusitis, and nasal obstruction. -Rhinorrhea and nasal polyps. -As the lung disease progresses; exercise intolerance, shortness of breath, growth failure, cor-pulmonale (rarely), respiratory failure, and death -Common pathogens include Staphylococcus aureus, and Pseudomonas aeruginosa though multidrug resistant organisms are increasingly common (MRSA, MDR, Stenotrophomonas maltophilia, and Burkholderia cepacia complex). • Antacids (to improve pancreatic enzyme function) • Agents to treat associated conditions or complications (e.g., insulin) • Agents devised to reverse abnormalities in chloride transport (e.g., ivacaftor) • Surgical therapy may be required for the treatment of the following respiratory complications: − Respiratory-pneumothorax, massive recurrent or persistent hemoptysis, nasal polyps, persistent and chronic sinusitis. − Lung transplantation is indicated for the treatment of end-stage lung disease. − GI-meconium ileus, intussusception, gastrostomy tube placement for supplemental feeding, rectal prolapse. • Transition to adult caregivers required at all US nationally accredited centers -Programs often formal to educate families and patients for transition. -Typical transition over 18 years old, often at 21 years old. • Median cumulative survival is exceeding 35 years, male survival is somewhat better than females without apparent reason • Infants born now with CF center care likely to survive beyond 50 years • Known survivors in their eighties. • OSA must be distinguished from primary snoring -Primary snoring = no associated obstructive events or gas exchange abnormalities, incidence 12-20 %. -OSA = obstructive apnea and hypopneas often with arousal and gas exchange abnormalities. • The prevalence of OSA is 2-4 % in healthy children. • The disorder can occur at any age but is most common in the preschool age group (2-6 years) and adolescents. • A higher prevalence has been reported in African-American children. • Adenotonsillar hypertrophy • Obesity • Craniofacial abnormalities, specifically midface hypoplasia and micrognathia • Hypotonia, e.g., Down syndrome • Neuromuscular disease • Cerebral palsy. • Loud nightly snoring with observed apnea spells • Parents may note that the child is a restless sleeper • Sweats while sleeping • Sleeps in an abnormal position with the neck extended • Chronic mouth breathing with chronic nasal congestion • Morning headaches • Excessive daytime sleepiness is more common among older children. • Mood changes • ADHD-like symptoms involving inattention and easy distractibility, or academic problems due to difficulty concentrating • Adenoidal facies as well as signs of atopy or nasal congestion such as "allergic shiners," • Nasal septal deviation • Enlarged turbinates • Redundant soft palate with a long uvula • Cor pulmonale or systemic hypertension (rare in children) • Nocturnal enuresis • All children should be screened for snoring. • Complex, high-risk patients should be referred to a specialist, e.g., craniofacial disorders, genetic syndromes, and neuromuscular disorders. • History and physical examination cannot distinguish between primary snoring and OSA. • Polysomnography is the diagnostic test of choice. • Adenotonsillectomy is the first line of therapy and curative for about 80 % of children with OSA. • Noninvasive positive airway pressure is an option for those who are not surgical candidates or who respond poorly to surgery. • High-risk patients (those with complicated diseases and severe OSA) should be monitored as inpatients postoperatively. • Patients should be reevaluated postoperatively to determine if additional treatment is required. • Patients with neuromuscular disease may desaturate in sleep but appear well when awake. Overnight saturation monitoring can be very helpful to recognize issues that require additional support. • ALTE a subjective report of a death like event. -GERD most common association for awake ALTE -Neurologic from seizure second most common association -Respiratory from pertussis and RSV third most common association -High index of suspicion of child abuse important -Observation, testing, and treatment as supported by history and exam • SIDS age 2-4 months with most deaths having occurred by 6 months • National recommendation on SIDS prevention -"Back to Sleep" supine position except few conditions -Marked decline in SIDS rate following this public policy education -Tummy time while awake -No smoking pre-or postnatally • Recognized risk factors: -Next born siblings of first born infants dying of any noninfectious natural causes are at significant increased risk of infant death from the same cause -ALTE very rarely associated -Infant factors: prematurity, low birth weight, co-sleeping, prone sleeping, and overheating -Maternal factors: young maternal age, smoking during pregnancy, and late or absent prenatal care • More than 95 % of SIDS cases are associated with one or more risk factors. • National recommendation on pacifiers: -Use pacifier once breast feeding has been established -Offer pacifier at bedtime or nap time -No correlation between pacifier use and length of breast feeding Pectus Excavatum (Funnel Chest; Fig. 4 • Based on severity of deformity and physiologic compromise • If neuromuscular disease worsens due to excessive efforts and retractions, responds to ventilation such as noninvasive positive pressure ventilation (NIPPV) • Mild: observation and physical therapy to maintain posture • Corrective surgery if significant physiologic compromise (Nuss procedure) • Background -Anterior displacement of midsternum and adjacent costal cartilage -It is rare 1/1500 of chest wall deformities -Associated with mild to moderate scoliosis, mitral valve prolapse, and coarctation of aorta • Clinical presentation -Rarely causes limitations -Physical appearance most common complaint -Haller index less than two is significant • Treatment -Surgery for cosmetic and psychological stress Suggested Readings Committee on Fetus and Newborn. Apnea, sudden infant death syndrome, and home monitoring Usefulness of chest radiographs in children with acute lower respiratory tract disease Clinical practice: croup Stridor and airway obstruction Cystic Fibrosis mortality and survival in the UK Stanson BF, editors. Nelson textbook of pediatrics Use of CT scans in selection of patients for pectusexcavatum surgery: a preliminary report Airway Clearance Techniques. AAP section on pediatric pulmonology executive committee Pediatric laryngology & bronchoesophogology Long-term complication of congenital esophageal atresia and/or tracheaoesophageal fistula Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines Chest wall and spinal deformities pediatric pulmonology: AAP section on pediatric pulmonology executive committee Pectus Excavatum 12-year-old healthy boy with funnel shaped chest "pectus excavatum Effective doses in radiology and diagnostic nuclear medicine: a catalog A clinical guide to pediatric sleep: diagnosis and management of sleep problems EPR-3). Guidelines for the Diagnosis and Management of Asthma. Bethesda: National Institutes of Health Pulmonary hemosiderosis Pulmonary disease following respirator therapy of hyaline-membrane disease. Bronchopulmonary dysplasia Primary ciliary dyskinesia and other genetic lung diseases pediatric pulmonology: AAP section on pediatric pulmonology executive committee Pediatric pulmonology: AAP section on pediatric pulmonology executive committee epiglottitis and bacterial tracheitis pediatric pulmonology: AAP section on pediatric pulmonology executive committee Image gently, step lightly: promoting radiation safety in pediatric interventional radiology Bronchiectasis pediatric Pulmonology: AAP section on pediatric pulmonology executive committee Pediatric pulmonology: AAP section on pediatric pulmonology executive committee