Pediatric Respiratory o Body plethysmography airway resistance, FRC, and RV cannot be measured with spirometery
and require other techniques
History o Obstructive airway disease low flow rates and increased RV or FRC o Includes onset, duration, and frequency of respiratory symptoms, and exposure to others with o Restrictive defect low FVC and TLC, with relative preservation of flow rates and FRC respiratory illness o Mean midexpiratory flow rate (FEF25-75%) o Obtain information concerning the severity and pattern o Peak expiratory flow rate (PEFR) can be obtained with a simple handheld device and may be o For infants, a feeding history should be obtained useful for home monitoring of older children with asthma o Family history should include questions about asthma and atopy, immune deficiencies, and CF o Inhalation challenge tests using methacholine, histamine, or cold, dry air are used to assess o Environmental history queries exposure to smoke, pets, and pollutants airway hyperreactivity Physical Exam Endoscopic Evaluation of the Airways o Any factor that impairs respiratory mechanics is likely to increase the respiratory rate o Endoscoic evaluation of the upper airways (nasopharyngoscopy) is performed with a flexible o It is important to observe the respiratory pattern and degree of effort (work of breathing) fiberoptic nasopharyngoscope to assess adenoid size, patency of the nasal passages, and o Hyperpnea (increased depth of respiration) may be observed with fever abnormalities of the glottis o Increased work of breathing can be described as inspiratory (intercostal, supraclavicular, or o Bronchoscopy is useful in identifying airway abnormalities and in obtaining airway samples for substernal retractions) or expiratory culture, especially in immunocompromixed patient s o Increased inspiratory effort is also manifested by nasal flaring o Transbronchial biopsies are rarely performed in children o Grunting suggests respiratory distress, but it may also be a manifestation Examination of Sputum o Stridor is a harsh sound caused by a partially obstructed extrathoracic airway, more commonly o Difficult to obtain in young children heard on inspiration Lung biopsy o Wheezing is produced by partial obstruction of the lower airways, more commonly heard during o Thoracoscopic procedure or a thoracotomy is preferred if thorough histologic evaluation is exhalation can be monophonic and low-pitched or high-pitched and musical desired o Crackles or rales fluid or secretions in small airways may produce sounds characteristic of Therapeutic measures crumpling cellophane o Oxygen administration any child in respiratory distress should be treated with supplemental o Digital clubbing seen in CF and in other, less common chronic pulmonary diseases O2 to maintain normal O2 sat levels Generally not seen in asthma, so its presence should heighten concern for other diagnoses Long term administration use a nasal cannula o Cough results from stimulation of irritant receptors in the airway mucosa Humidified high flow nasal cannula allows the provider to more comfortably deliver higher o Chronic cough is defined as a daily cough lasting longer than 3 weeks flow rates while controlling FIO2 and is widely used in neonates Diagnostic Measures o Aerosol therapy accomplished via inhalation of dry powder inhaler (DPI), metered dose o Imaging techniques inhaler (MDI), or nebulizer Chest radiographs determine lung abnormalities, they provide information about the bony o Chest physiotherapy and clearance techniques: chest percussion (moves secretions toward thorax, the heart and the great vessels. Should be obtained in both the PA and lateral central airways) projections and, if possible, following a full inspiration o Intubation: artificial airway; endotracheal tubes can damage larynx and airways if not properly Barium esophagram may be valuable in diagnosing disorders of swallowing (dysphagia) and sized esophageal motility, vascular rings (esophageal compression), tracheoesophageal fistulas, o Laryngeal mask airway (LMA) and, to a lesser extent, gastroesophageal fistula o Mechanical ventilation positive pressure ventilation requires an artificial airway o Computed tomography (CT) scan of the chest Noninvasive ventilation is useful in patients with obstructive sleep apnea and neuromuscular Image of choice for evaluating pleural masses, bronchiectasis, and mediastinal lesions as disease well as delineating pleural from parenchymal lesions Magnetic resonance imaging (MRI), useful in visualizing cardiac and great vessel anatomy, Ventilation controlled by central chemoreceptors less useful for evaluation of pulmonary parenchymal lesions o Modulated by peripheral receptors in the carotid and aortic bodies o Ultrasonography o Full lung inflation temporarily inhibits inspiratory effort in infants (Hering-Breuer reflex) Can be used to delineate some intrathoracic masses and is imaging procedure of choice for Acute Life-Threatening Events assessing parapneumonic effusion/empyema o Any unexpected and frightening change in condition characterized by apnea, color change (blue Useful for assessing diaphragmatic motion in small children or pale), sudden limpness, choking, or gagging Measures of Respiratory Gas Exchange o Gastroesophageal reflux and laryngospasm are the most common causes of ALTE and are a/w o Properly performed arterial blood gas analysis provides information about the effectiveness of emesis, choking, or gagging both oxygenation and ventilation o CNS causes: intracranial bleeding o Pulse oximetry measures the O2 saturation of hemoglobin by measuring the blood absorption of o Acute infection with respiratory viruses, pertussis, and serious bacterial infections can also cause two or more wavelengths of light; noninvasive, easy to use, and reliable apnea in infants o Transcutaneous electrodes can be used to monitor PCO2 and PO2 at the skin surface, but are o Diagnostic Studies less accurate History premature birth, prior apnea, level of consciousness at the time of event, presence Pulmonary Function Testing or absence of respiratory effort, limpness or stiffness, jerking movements, feeding history, o Measured with spirometry intercurrent illnesses, any trauma, social situation of family o During the forced expiratory maneuver, forced vital capacity (FVC), forced expired volume in PE bruising and injury, general and neurologic condition of infant, nutritional status, the first second (FEV1), and forced expiratory flow (FEF) rates are measured respiratory pattern, cardiac status Labs serum electrolytes, serum glucose, BUN, creatinine, hemoglobin, hematocrit, WBC, CT delineate the site of obstruction but may require sedation CXR, and ABG Flexible nasopharyngoscopy/laryngoscopy good choice Evidence of respirator infection test for respiratory viruses and pertussis o Adenoidal and Tonsillar Hypertrophy Gastroesophogeal reflux barium swallow or pH probe Most common cause of UAO Cardiorespiratory monitoring for 12-24 hours Signs: mouth breathing, snoring, and OSA Sudden Infant Death Syndrome Diagnostics: lateral xray of nasopharynx or by flexible nasopharyngoscopy o Unexpected death of an infant younger than 1 with and unexplained cause Tx: Most effective removal o Higher risk in premature and low birth weight infants, infants of young impoverished mothers o Choanal Stenosis (Atresia) who smoke, African American and Native American infants, and infants whose moms abused Congenital drugs Obstruction of nasal passages o Rare before 4 weeks or after 6 months Inability to easily pass a small catheter through the nostrils o Associated with prone position during sleep, esp. on soft bedding Dx confirmed by CT scan and inspecting the area with a flexible nasopharyngoscope o Some SIDS deaths may be due to prolongation of the Q-T interval, abnormal CNS control of Tx: surgery respiration, and CO2 rebreathing from sleeping face down o Croup (laryngotracheobronchitis) Apnea Most common infection of the middle respiratory tract o Cessation of airflow due to either lack of respiratory effort (central apnea) or upper airway Most common causes: parainfluenza viruses (1, 2, 3, 4) and RSV obstruction (obstructive apnea) Most common in children 6 months 3 years; peak in fall and early winter o Central apnea <10 seconds is common in healthy babies Typically follows a common cold o Pauses lasting longer than 15-20 sec is abnormal Adolescents laryngitis o Premature infants can have apnea of prematurity recurrent apneic episodes that are often of Symptoms: central origin; should resolve by 44 weeks postconceptional age Barking or brassy cough, hoarseness, inspiratory stridor, low-grade fever, respiratory o Congenital central hypoventilation rare genetic disorder in which there is a profound loss of distress respiratory control during sleep leading to central apnea, hypercarbia, and hypoxemia Labs and Imaging: Defect in the PHOX2B gene AP radiograph of neck shows subglottic narrowing of croup (steeple sign) A/w an increased risk of Hirschsprung disease and neural crest tumors Spasmodic croup sudden onset of symptoms, usually at night, but w/o a significant upper o Obstructive sleep apnea syndrome (OSA) complete or partial upper airway obstruction during respiratory tract prodrome sleep Tx: Adenotonsillar hypertrophy is the most common cause of OSA in young children Oral or intramuscular dexamethasone Diagnosis should be confirmed with a polysomnoram o Epiglottitis Continuous recording of EEG and eletro-oculogram for sleep staging, oronasal airflow Medical emergency and pressure, audible snore, chest and abdominal wall excursion, leg and chin Caused by group A strep or Staph aureus or Haemophilus influenza type b in unimmunized electromyogram, electrocardiogram, pulse ox, and capnography Measured score = apnea-hypopnea index or number of times in an hour of sleep that pts Pts will be sitting, often with head held forward, mouth open, jaw thrust forward (sniffing the child has cessation or reduction of airflow of a significant magnitude Treatment: CPAP or BiPAP position) Lateral xray = bulging epiglottis (thumb sign) and swelling of the aryepiglottic folds Dx confirmed by observation of the inflamed and swollen supraglottic structures and Upper Airway Obstruction (UAO) swollen, cherry-red epiglottitis o Blockage of any part of the airway located above the thoracic inlet Requires and antibiotic therapy and ET intubation to maintain airway o Supraglottic, glottis, and subglottic causes o Bacterial Tracheitis o More pronounced during inspiration Superinfecion of trachea that may follow viral croup o A/w stridor Most commonly caused by S. aureus Laryngomalacia (floppy larynx) is the most common cause of inspiratory stridor in infants Spasmodic croup and may be aggravated by swallowing o Laryngomalacia o Hoarsness suggests vocal cord involvement Exaggerated collapse of the glottis structures, especially the epiglottis and arytenoid o Children with UAO may have increased inspiratory work of breathing manifested by suprasternal retractions cartilages, during inspiration o Diagnostic May be due to decreased muscular tone of the larynx and surrounding structures or to Radiography immature cartilaginous structures Inspiratory stridor Lateral views of neck and nasopharynx Most common cause of stridor in infants o Adenoidal hypertrophy and airway swelling o Subglottic stenosis AP view of neck with head in extension Narrowing of the portion of the trachea immediately below the vocal cords o Subglottic space should be symmetrical and the lateral walls of the airway should May be congenital but more often is acquired fall away steeply ET intubation is required in some premature infants o Asymmetry = subglottic stenosis or a mass lesion Stridor that is frequently biphasic (inspiration and expiration) o Mass Lesions o Foreign Body Aspiration Most common laryngeal tumor in childhood = hemangioma (usually presents before 6 Relatively uncommon months) Most kids that do this are under 4 years of age Subglottic lesions produce asymmetrical narrowing of the subglottic space on AP Foreign bodies tend to lodge in the right-sided airways radiographs Most common foreign bodies aspirated are food and small toys Definitive diagnosis = endoscopy Coins more often lodge in the esophagus than in the airways Laryngeal webs = failed recanalization of the glottis airway in utero o Bronchiolitis Laryngeal cysts = consequence of airway trauma Disease of small bronchioles with increased mucus production and occasional Both can produce biphasic stridor bronchospasm, sometimes leading to airway obstruction Foreign body should be considered in any infant or child capable of ingesting foreign Most commonly caused by a viral lower respiratory tract infection (RSV) objects Presents as a progressive respiratory illness similar to the common cold Juvenile laryngeal papillomatosis Prevention injections of palivizumab (RSV specific monoclonal antibody) Rare condition of benign tumors caused by HPV-6 and HPV-11 acquired at birth from o Bronchopulmonary Dysplasia maternal genital warts Oxygen dependence at 36 weeks postconceptual age accompanied by characteristic clinical Manifestions start in infancy and include biphasic stridor and hoarse voice/cry and radiographic findings Lesions are most commonly located in the larynx, but they can spread to the trachea Radiographic lung opacification and subsequently by development of cysts accompanied o Vocal Cord Paralysis by areas of overdistension and atelectasis More often caused by damage to the recurrent laryngeal nerve (left more susceptible) Clinical O2 dependence, hypercapnia with a compensatory metabolic alkalosis, pulmonary Trauma and mediastinal lesions HTN, poor growth, and development of RHF Central causes: Arnold-Chiari malformation, hydrocephalus, and intracranial hemorrhage o Endobronchial Mass Lesions Biphasic stridor and alterations in voice and cry (weak cry, hoarseness, aphonia) Most common granulation tissue, results from local inflammation (TB) Children at risk for aspiration Most common airway malignancy (rare) is the nonsecreting carcinoid tumor o Congenital Lung Anomalies Lower Airway, Parenchymal, and Pulmonary Vascular Diseases Congenital lobar over distension over inflation of one lobe of a lung; may be mistaken for o Most common in children = asthma results in diffuse bronchial obstruction from airway a pneumothorax inflammation, constriction of bronchial smooth muscle, and excessive secretions Pulmonary hypoplasia may cause congenital diaphragmatic hernia o Wheezing in the first weeks or months of life is probably not asthma o Emphysema o Wheezing localized to one area of the chest suggests focal airway narrowing Disruption or destruction of alveolar septa o Obstruction below the thoracic inlet causes more wheezing on expiration than on inspiration Rare in children seen with alpha-1 antitrypsin deficiency o Radiographic eval is not needed with each episode of wheezing Kartagener syndrome situs inverus, pansinusitis, bronchiectasis o Generalized hyperinflation, indicated by flattening of the diaphragms and an increased AP o Pneumonia diameter suggests diffuse obstruction of the small airways Infection of the lower respiratory tract o Dysphagia leading to aspiration is best assessed with a videofluoroscopic swallowing study RSV infants o Asthma Other resp. viruses (flu, adeno, etc.) kids <5 Airway hyperresponsiveness Mycoplasma >5 Chronic inflammation can lead to airway remodeling Strep pneumo is the most common cause of lobar pneumonia in anyone other than infants Spirometry help establish the diagnosis Mycoplasma and Chlamydia cause atypical pneumonia (afebrile pneumonia) Allergy skin test should be included o Pulmonary Edema CXR should be preformed on the first episodes of asthma Dypspnea LOOK AT THE ASTHMA LECTURE Kerley B lines o Tracheomalacis o ARDS Floppy trachea due to lack of structural integrity of the tracheal wall Respiratory failure May be congenital or acquired Severe hypoxemia (200 mmHg of PaO2/FiO2) Tracheal collapse may only be apparent during force exhalation or with cough o Pulmonary Arterial HTN (PAH) Commonly aggravated by respiratory infections 5 groups: o Tracheoesophageal Fistula 1: PAH due to diseases within veins and small pulmonary muscular arteries and Most common forms occur with esophageal atresia idiopathic pulmonary arterial htn A/w vertebral anomalies, anal atresia, cardiac anomalies, TEF, renal anomalies, and limb 2: dysfunction of the left heart anomalies 3: secondary to lung disease or chronic hypoxia Barium should not be used 4: chronic pulmonary thromboembolism Tx: surgery 5: unclear multifactorial diseases o Extrinsic Tracheal Compression Results in cor pulmonale Compression by vascular structures or masses RHF leads to hepatic congestion, fluid retention, and tricuspid insufficiency Most common cause is anterior compression from an anomalous innominate artery o Pulmonary Hemorrhage Idiopathic pulmonary hemosiderosis rare disorder characterized by recurrent alveolar Antibiotic therapy to control chronic infection (Pseudomonas!!!) bleeding, iron deficiency anemia, and hemosiderin laden macrophages Pulmonary hemorrhage bleeding from an intrathoracic source Chest Wall and Pleura Present with hemoptysis o Thoracic scoliosis curve greater than 60 degrees; can lead to decreased lung volumes Symptomatic airway hemorrhage may result in significant hemoptysis with few o Pectus excvatum radiographic changes Sternal concavity Alveolar bleeding often causes profound respiratory symptoms, hypoxemia, diffuse May result in restrictive lung disease infiltrates on radiographs, and minimal hemoptysis o Pectus Carinatum o Pulmonary Emoblism Sternum bows out A/w indwelling central venous catheters, oral contraceptives, or hypercoaguable stats Not associated with abnormal pulmonary function Hypoxia common and nonspecific ST-segment and T-wave changes o Pneumothorax Diagnostic test of choice = CT angiogram of the chest Spontaneous primary pneumothorax teenagers and young adults (tall, thin males and Gold standard = pulmonary angiography smokers) Secondary pneumothorax barotrauma from mechanical ventilation, asthma, cystic fibrosis, Cystic Fibrosis trauma to the chest, and severe necrotizing pneumonia o Cystic fibrosis transmembrane regulator (CFTR) genre on chromosome 7 Clinical shoulder pain and dyspnea Chloride channel on apical surface of epithelial cells Tension pneumothorax severe respiratory distress and cyanosis; life threatening Most common mutation is a deletion of 3 base pairs resulting in the absence of o Pneumomediastinum phenylalanine at the deltaF508 position Dissection of air from the pulmonary parenchyma into the mediastinum o Clinical digital clubbing, nasal polyposis, protein and fat malabsorption Symptoms chest pain and dyspnea o Allergic bronchopulmonary aspergillosis is a hypersentivity rxn to Aspergillus o Pleural Effusion o 90% of pts born with exocrine pancreatic insufficiency Fluid accumulates in the pleural space when local hydrostatic forces that are pushing fluid o Fat malabsrotion leads to steatorrhea out of the vascular space exceed the oncotic forces that are drawing fluid back into the o 10% of pts are born with an intestinal obstruction caused by inspissated meconium (meconium vascular space ileum) Can be transudates or exudates o Older pts may have intestinal obstruction from thick inspissated mucus in the intestinal lumen Bacterial pneumonia can lead to an accumulation of pleural fluid (parapneumonic effusion) (distal intestinal obstruction syndrome) When the fluid is purulent or infected = empyema o May present with insulin deficiency (CF-related diabetes) Due to pneumonia caused by strep pneumo, group a strep, or staph aureus o Dx: sweat test; elevated sweat chloride tests Tx: o Tx: Drain fluid with a chest tube Airway secretion clearance techniques (chest physiotherapy) Surgery