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pediatric test 2

Terms in this set (134)

•Acute inflammatory disease of the lower respiratory tract, resulting in obstruction of the small airways, or bronchioles
•Common seasonal viral infection, with incidence peaking in the winter, that causes wheezing and congestion in infants up to 2 years of age
•presents with symptoms similar to a common cold; however, as the disease progresses, coughing, wheezing, and dyspnea often occur.
•Only a small percentage of children will require hospitalization, and of those, only a few will require treatment in the intensive care unit.
•Majority of children will respond well to supportive care and remain at home
-Viral pathogen (RSV, influenza and parainfluenza viruses, human metapneumo virus, or adenovirus)
-is highly contagious.
-Combination of two or more infectious organisms can result in a severe form of the disease
-Infection spread by direct contact with respiratory secretions.
-Virus spreads from upper respiratory tract to the medium and small bronchi and bronchioles.
-Within 24 hours, necrosis of the respiratory epithelium occurs and triggers an inflammatory response.
-Bronchial obstruction and airflow restriction most pronounced on expiration and can lead to alveolar air trapping and development of intrinsic positive end-expiratory pressure
-Adequate and thorough history, physical examination, consideration of the patient's age, season, and the patient's clinical presentation
-Tachypnea, tachycardia, fever, diffuse fine expiratory wheezing on chest auscultation, and possibly hypoxia
-Laboratory tests may be of value to guide care for patients with more severe forms of bronchiolitis or those who develop complications.
-Chest radiographs
-Lack of appetite
-Low-grade fever (<38.6°C)
-Hypothermia in children younger than 1 month
-Cough, dyspnea, wheezing, and feeding difficulties
-Respiratory distress in severe cases
-Paradoxical breathing
-Apnea
-Nonrespiratory manifestations (otitis media, myocarditis, and supraventricular and ventricular dysrhythmias)
-Antibacterial medications should be used only in children with bronchiolitis who have specific indications of the coexistence of a bacterial infection.
-Supplemental O2 is indicated if SpO2 falls persistently below 90% in previously healthy infants.
-Infants with a known history of hemodynamically significant heart or lung disease and premature infants require close monitoring as the oxygen is being weaned.
-Palivizumab prophylaxis to selected infants and children with chronic lung diseases, a history of prematurity (fewer than 35 weeks' gestation), or congenital heart disease
-Breastfeeding is recommended to decrease a child's risk of having lower respiratory tract disease.
•Leading cause of lower respiratory tract infections in young children
•Most common cause of bronchiolitis and pneumonia
•infections occur primarily during the fall, winter, and spring seasons.
-Exposure to the virus by another sibling
-Modes of transmission: cough and direct physical contact from infected individuals
-Risk factors: childcare attendance, older siblings in school, crowding and lower socioeconomic status, exposure to environmental pollutants, and minimal breastfeeding
-infection is limited to the respiratory tract.
-Starts in upper respiratory tract, then moves down the respiratory tract, causing bronchiolitis and later atelectasis and pneumonia
-Illness may begin with upper respiratory symptoms and progress rapidly over 1 to 2 days to the development of diffuse small airway disease
-infection leads to edema of the small airways and increased mucus production that causes partial or complete obstruction of the airway leading to air trapping or alveolar collapse and pneumonia, respectively.
-In very young infants, apnea may be present.
-Relatively nonspecific sepsis-like clinical presentation in infants younger than 6 weeks
-Reinfection can occur at any age, but limited to the upper respiratory tract.
-Upper respiratory tract infection due to this disease is usually more severe than the common cold.
-No lab tests are needed if patients are comfortable, well hydrated, and feeding well.
-CBC, serum electrolytes, urinanalysis, and ABGs when at risk for severe form of the disease
-Sputum culture, antigen testing, PCR assay, molecular probing for confirming this disease
-Chest X-ray in severe infection
-clinical presentation: Cold-like symptoms initially (cough, nasal congestion, sneezing, low-grade fever), Tachypnea, chest wall retractions, and wheezing later on, Cyanosis in severe cases, Otitis media, Dehydration
-Supportive care
-Bronchodilators in patients with RSV-related lower respiratory tract infection
-Alpha agonists (nebulized epinephrine) in acute RSV bronchiolitis episodes
-Ribavirin (Virazole) is primarily reserved for patients with significant risk factors and severe acute RSV disease (e.g., transplant recipients)
-Palivizumab prophylaxis for RSV should be limited to infants born before 29 weeks' gestation and to infants with chronic illness, such as congenital heart disease (cyanotic or acyanotic) or chronic lung disease
-Oxygen therapy to treat hypoxemia, decrease the work of breathing, and decrease myocardial work
-Bronchial hygiene to enhance the mobilization of bronchial secretions
•Extremely contagious respiratory illness caused by influenza A or B viruses
•Commonly known as "flu"
•Spreads through the upper and/or lower respiratory tract
•Appears most frequently in winter and early spring
-viruses are constantly changing, with new strains of influenza appearing every few years (Avian influenza [H5N1] and Swine flu [H1N1]).
-these viruses are spread by contact or by air.
-Enter the body when someone touches their nose, eyes, or mouth with contaminated hands or inhales contaminated droplets
-A, B, and C influenza viruses are distinguished based on the core nucleoproteins.
-Hemagglutinin (H) and neuraminidase (N) are the most significant surface proteins.
-New strains of may spread from animal species to humans, or an existing human strain may pick up new genes from a strain that usually infects birds or pigs.
-Antigenic drift: ability to evade annually acquired immunity in humans
-Antigenic shift: re-assortment of two strains of viruses, resulting in a virulent strain that causes a pandemic
-Infection occurs after an immunologically susceptible person directly inhales the aerosol or touches the deposited aerosols on surfaces.
-Once the virus is within host cells, cellular dysfunction and degeneration occur and release inflammatory mediators.
-The incubation period of influenza ranges from 1 to 4 days.
-Viral shedding occurs at the onset of symptoms or just before the onset of illness (0-24 hours) and continues for 7 to 10 days, but young children may shed influenza viruses for a longer duration.
-In highly immunocompromised persons, shedding may persist for weeks to months.
-Based on symptoms (headache, fever, chills, muscle aches, weakness and fatigue, nasal congestion, sore throat, and cough
-Clinical symptoms alone may not be very reliable.
-Testing for
-Rapid influenza diagnostic test, viral cultures, and real-time RT-PCR molecular tests
-Rapid influenza diagnostic test (RIDT): most common and completed in less than 15 minutes; detects 40% to 70% of influenza cases
-Viral cultures: the gold standard, identify both the presence of the virus and the strains of virus present. However, it can take 3 to 10 days to obtain the results.
-Real-time RT-PCR: most sensitive tests, can distinguish between A and B viruses as well as among different strains of influenza A virus
infection is optimally performed within 48 hours of the onset of symptoms.
-Testing is recommended in hospitalized patients, those who have weakened immune systems, or those who are otherwise at an increased risk of serious complications.
-Abrupt and include high-grade fever, headache, malaise, fatigue, body aches, cough, sore throat, runny or stuffy nose, and diarrhea and vomiting
-Greater intensity of symptoms
-Complications, such as viral or bacterial pneumonia, sepsis, pericarditis, myocarditis, and encephalitis, can be very serious.
-Worsening of long-term conditions, such as congestive heart failure or diabetes
-Prevention is the most effective treatment strategy.
-Routine annual vaccination for all persons aged 6 months or older, preferably before the onset of influenza activity in the community.
-The vaccination becomes effective 10 to 14 days after administration and usually has 50% to 60% efficacy against influenza A viruses and 60% to 70% efficacy against influenza B viruses.
-Antiviral agents: when indicated and to be effective, oseltamivir, zanamivir, peramivir, amantadine, and rimatadine must be administered within 48 hours of symptom onset.
-Cases of suspected severe infection should be treated early and aggressively, even before diagnostic tests can be confirmed.
•Affects the lower laryngeal (L) area, trachea (T), and occasionally the bronchi (B)
•AKA subglottic croup
•Common pediatric viral respiratory tract illness
•Most common etiology for hoarseness, cough, and onset of acute stridor in febrile children
•Inflammatory process that causes edema and swelling of the mucous membranes
-Viral infections (parainfluenza viruses 1, 2, and 3; nfluenza A and B viruses; RSV; rhinovirus; and adenoviruses)
-Viruses are spread through either direct inhalation from a cough and/or sneeze or by contamination of hands from contact with fomites and subsequent touching of the mucosa of the eyes, nose, and/or mouth..
-Mycoplasma pneumoniae has been identified in cases.
-occurs most often during the late fall and winter seasons.
-Viral upper respiratory tract infection causes nasopharyngeal inflammation that spreads to the larynx and trachea and occasionally to the mainstem bronchi.
-Subglottic inflammatory process produces edema, reduces the cross-sectional area of the airway
-Airway resistance markedly increases and leads to excessive work of breathing.
-Airway inflammation and edema impair vocal cord movement, leading to the characteristic cough.
-Clinically, based on the classical signs and symptoms (barking cough and stridor)
-AP and lateral X-ray of the neck (classic steeple sign)
-No laboratory tests are necessary.
Testing for the causative virus might be indicated
-Initially: nonspecific respiratory and cold-like symptoms (sore throat, cough, and low-grade fever)
-Hoarseness, a seal-like barking cough, and inspiratory stridor
-Varying degrees of respiratory distress
-Severe LTB: tachypnea; tachycardia; visible suprasternal, intercostal, and subcostal retractions with poor air entry; hypoxemia; hypercapnia
-Sudden respiratory arrest during an episode of severe coughing
Symptoms are usually worse at night
-Croup severity scores: Westley score
-Patients should be extubated as soon as the airway inflammation has decreased and when pulmonary secretions are minimal.
-Supportive care
-Keep the patient calm and nonagitated to prevent increased respiratory efforts.
-Medications for soothing the swollen airways
-Symptoms typically resolve within 3 to 7 days, butcan last as long as 2 weeks.
-Mild LTB: parental reassurance, education regarding the course of the disease, and supportive homecare guidelines
-Severe LTB: thorough clinical evaluation, airway patency, and effective oxygenation and ventilation, with close monitoring of vital signs and respiratory parameters
-Corticosteroids and nebulized racemic epinephrine
-Antibiotics are not required unless there is evidence of a secondary bacterial infection or in patients who appear toxic and do not adequately respond to corticosteroid administration and nebulized racemic epinephrine.
-Intravenous fluid hydration may be required to stabilize the fluid volume in patients with LTB who fail to maintain adequate oral intake and have increased insensible fluid loss.
-Heliox
•Serious airway emergency; can be life threatening
•Bacterial infection causing acute inflammation of the supraglottic region of the oropharynx
•Seen in children between 2 and 5 years of age
-Haemophilus influenzae type B is the most common organism (>80%)
-Other causative agents: group A Streptococcus pneumoniae and H. parainfluenzae
-Less common: Staphylococcus aureus, Escherichia coli, Klebsiella pneumoniae, and herpes simplex virus
-Candida and Aspergillus in immunocompromised patients
-Bacteremia and/or direct invasion of the pathogenic organism
-Transmitted by aerosol droplets
-Noninfectious causes: thermal injuries and throat burns affecting the epiglottis of bottle-fed infants, caustic insults with ingestion of dishwasher soaps, foreign body ingestion, and head and neck chemotherapy
-Swelling of the supraglottic structures
-Reduction in the caliber of the upper airway, causing turbulent flow during inspiration
-Dysmorphic shape of the epiglottis in the narrowed superglottic area acts as a ball-valve mechanism producing partial to complete airway obstruction.
-Airway resistance and WOB significantly increase.
-Inflamed and enlarged epiglottis may partially cover the esophageal opening and lead to dysphagia.
-Severe airway obstruction may cause cardiopulmonary arrest.
-Suspected when the child presents with signs of bacterial septicemia and an acute onset of upper airway obstruction
-Signs of airway obstruction and increased work of breathing, including tachypnea, tachycardia, stridor, intercostal and suprasternal retractions, and cyanosis
-Clinical features: tripod positioning, anxiety, drooling, dysphagia, and respiratory distress
-Chest radiographs
-Direct laryngoscopy or flexible fiberopticbronchoscopy
-Blood cultures or cultures from the surface of the epiglottis
-Initially mild clinical presentation that may progress rapidly over 2 to 4 hours.
-Sore throat in almost all children
-High-grade fever, difficulty swallowing and clearing secretions
-Pale and either lethargic or irritated child
-Noisy breathing with muffled cry or voice
-Air hunger and upright tripod sitting position
-Suprasternal retractions, tachycardia, tachypnea, and nasal flaring
-Cyanosis with impending complete airway obstruction
-Complete airway obstruction and cardiopulmonary arrest
-Complications: meningitis, epiglottic abscess, cervical adenitis, vocal granuloma, cartilaginous metaplasia of the epiglottis, pneumonia, pulmonary edema, empyema, cellulitis, and septic shock
-Keep child as calm as possible
-Examination of the child's mouth and upper airway when proper equipment to perform tracheal intubation or emergency cricothyroidectomy are available
-Tympanic rather than oral temperature
-Supplemental oxygen
-If needed, nasal rather than oral intubation
-Tracheostomy must be performed if intubation is not possible
-Percutaneous transtracheal jet ventilation
-MV and close monitoring in PICU
-Sedatives and antibiotics as per cultures
-Assessment for the presence of air leaks before extubation
•occur when air escapes from the lung into extra-alveolar spaces where it is not normally present.
•Common complications of high positive pressure applied during mechanical ventilation
•Can occur spontaneously
•Occur more frequently in the newborn period than at any other time of life
•Most common risk factors: lung immaturity, respiratory distress syndrome, aspiration syndromes, and congenital diaphragmatic hernia
Most common: pneumothorax andpneumomediastinum
-High pressures/volumes used during invasive mechanical ventilatory support
-Propensity increases in children with a pulmonary pattern of atelectatic alveoli adjacent to normal alveoli
-Prolonged inspiratory time and inverse ratio ventilation
-Spontaneously due to very high negative intrathoracic pressure created with the first breaths the infant takes after delivery
-With high transpulmonary pressures or tidal volumes, the distal airways and alveoli become overdistended or stretched and ruptured. Air will leak toward the visceral pleura and cause a pneumothorax with rupture of the visceral pleura.
-In some cases, air continues to accumulate, moves medially, and dissects out from the hila, causing a pneumomediastinum.
-Effect of pneumothorax depends on its size.
-Pneumothorax causes compression of the great veins and increases pulmonary vascular resistance.
-Venous return and cardiac output decrease.
-Shunting and hypoxemia
Large pneumothorax is a life-threatening conditions
-Suspicion with the sudden onset of respiratory distress
-Transillumination
-Chest X-ray
-Large pneumothorax is associated with tracheal deviation to the opposite side of the pneumothorax.
-Lateral X-ray view might be helpful in detecting anterior pneumothorax.
-No symptoms with small pneumothorax
-Rapid or gradual onset of symptoms
-Large pneumothorax: respiratory distress, tachypnea, grunting, pallor, retractions, cyanosis, acidosis, hypercarbia, hypotension, bradycardia
-Chest asymmetry with enlargement of the affected side
-Decreased breath sounds on the affected side, and a shift of the point of maximal cardiac impulse away from the affected side
-Decrease in lung compliance
-Hypoxemia-induced pulmonary arterial vasoconstriction, vasospasm, and pulmonary hypertension
-Decrease cardiac output and profound hypotension
-Pulmonary interstitial emphysema
-Close monitoring in spontaneously breathing infants with small pneumothoraces
-O2 supplementation
-Adjustment of ventilatory settings in mechanically ventilated patients (reduction of pressures and/or volumes)
-Thoracentesis: symptomatic spontaneously breathing infants who are not mechanically ventilated
-Chest tube placement: symptomatic and mechanically ventilated infants
•Any abnormal collection of fluid in the pleural space
•Most common manifestation of pleural disease
•Either a transudate or an exudate
•Transudate: imbalance in oncotic and hydrostatic pressures
•Exudates: inflammation of the pleura or decreased lymphatic drainage
•Combination of both transudate and exudate
•Indicator of an underlying disease that may be caused by pulmonary or nonpulmonary conditions
-Disruption of the balance between hydrostatic and oncotic forces in the visceral and parietal pleurae and lymphatic drainage
-Transudates: imbalance in hydrostatic and oncotic forces in the chest
-Major causes of transudative pleural effusion: congenital heart disease, congestive heart failure, hepatic cirrhosis, peritoneal dialysis, nephrotic syndrome, and pulmonary embolus
-Other causes of transudates: movement of fluid from peritoneal spaces or by iatrogenic infusion into the pleural space from misplaced or migrated central venous catheters or nasogastric feeding tubes
-Exudates: pleural or lung inflammation, impaired lymphatic drainage of the pleural space,transdiaphragmatic movement of inflammatory fluid from the peritoneal space, altered permeability of pleural membranes, and increased capillary wall permeability or vascular disruption
-Disruption of regulated fluid balance in the pleural space: local factors (leaky capillaries from inflammation due to infection, infarction, or tumor) vs. systemic factors (elevated pulmonary capillary pressure with heart failure, excess ascites with cirrhosis, or low oncotic pressure due to hypoalbuminemia)
-Exudate: accumulated fluid rich in protein and lactic dehydrogenase (LDH)
-Transudate: accumulated fluid low in protein and LDH
-Auscultation and percussion
-Chest X-ray
-Fluid aspiration by thoracentesis to differentiate between a transudate and an exudate
-Variable and often are related to the underlying disease process
-Dyspnea
-Mild nonproductive cough
-Mild to severe pleuritic chest pain
-Asymptomatic if small effusion
-Severe effusion: inaudible breath sounds, dullness to percussion, decreased tactile fremitus, asymmetrical chest expansion, diminished expansion on the side of the effusion, and mediastinal shift away from the effusion
-Increased peak airway and peak alveolar pressures and hypoxemia in intubated and mechanically ventilated patients
-Drainage of the pleural effusion
-Surgical intervention for undrainable effusions or for prevention of further accumulation
-Treating the underlying medical disorder
-Discontinuation of some causative medications
-Pleurodesis by insufflating talc directly onto the pleural surface
-Decortication
-Therapeutic thoracentesis with a catheter rather than a needle is used to remove larger amounts of pleural fluid.
Complicatedparapneumoniceffusions require drainage by tubethoracostomy
•AKA chylopleura
•Presence of chyle in the pleural space secondary to leakage from the thoracic duct
•Caused by trauma to the neck or thorax or by an obstruction of the thoracic duct
-Traumatic or nontraumatic
-Right side accumulation of chylous fluid in the pleural cavity leaking from the thoracic duct accumulates in the pleural cavity, usually on the right side, and causes acute or chronic alterations in the pulmonary mechanics.
-If left untreated, chylothorax can lead to significant nutritional and immunologic pathology: hypoalbunemia, nutritional compromise, and metabolic complications.
-The chyle appears to have a bacteriostatic property, which accounts for the rare occurrence of infection complicating chylothorax.
-Congenital and acquired
-Acquired chylothorax: secondary to traumatic injuries (direct traumatic injury to the thoracic duct, thrombosis of the superior vena cava)
-Thoracentesis and pleural fluid analysis and pH
-Imaging techniques: site and cause of chyleleakage
-Ingestion of lipophilic dye or radio-labeled triglyceride: presence of the dye color in the fluid within 1 hour or detection of high radioactivity in the pleural fluid after 48 hours
-Lymphangiography
-CT of the thorax and abdomen: identify the site of thoracic duct rupture
Lymphoscintigraphy
-Mostly asymptomatic
-Dyspnea and tachypnea
-Physical exam nonspecific: decreased breath sounds and shifting dullness
-Traumatic chylothorax usually develops within 2 to 10 days postinjury; in nontraumatic chylothorax, the onset of symptoms is more insidious
-Spontaneous chylothorax may rarely present as a sudden neck mass.
-Malnutrition, weakness, dehydration, metabolic acidosis, and compromised immunologic status
-Hypoalbuminemia and lymphopenia
-Directed at the underlying cause
-Surgical management is pursued after failure of conservative therapies.
-Chest tube and continuous drainage of fluid from the pleural space
-Replacements of fluids, electrolytes, protein, fat, and lymphocytes
-Radiotherapy to the mediastinum
-Somatostatin for postoperative and iatrogenic chylothorax
-Surgical intervention with thoracic duct ligation if: (1) average daily loss exceeds 1 L to 1.5 L per year of age in children for a 5-day period, (2) chyle flow has not diminished over 14 days, (3) nutritional complications appear imminent, or (4) accumulation of chyle is continuous despite chest tube drainage
-Video-assisted thoracic surgery and fluoroscopic percutaneous embolization
-A pleuroperitoneal shunt
-Talc pleurodesis for malignant chylothorax
•Presence of blood in the pleural space
•Source of blood: chest wall, lung parenchyma, heart, or great vessels
•Secondary mainly to a blunt trauma, iatrogenic causes, or a spontaneous occurrence
-Penetrating injuries of the lung, heart, great vessels, or chest wall are the most common causes
-Accidental, deliberate, or iatrogenic trauma
•Blunt chest trauma: laceration of internal vessels
•Iatrogenic: central venous catheter and thoracotomy tube placement
-Nontraumatic or spontaneous
-Hemodynamic and respiratory physiologic responses
-Dyspnea and increased WOB
-Derangements of ventilation and oxygenation in trauma cases
-Empyema and fibrothorax
-If undetected or improperly treated, empyema can lead to bacteremia and septic shock.
-Fibrothorax traps the lung in position and prevents it from full expansion, causing persistent atelectasis of portions of the lung and reduction in pulmonary function.
-Upright chest radiograph
-Ultrasonography
-CT
-Bedside echocardiography
-Hematocrit of pleural fluid in nontraumatic cause
-Widely variable symptoms and findings
-Chest pain, dyspnea, tachypnea, shallow breathing
-Hypoxemia, diminished ipsilateral breath sounds and dull percussion, hypotension, tachycardia in severe cases
-Mild to severe derangements in hemodynamic variables
-In nontraumatic hemothorax: depending on the underlying pathology
-Hemothorax due to acute hemorrhage from structures within the chest, such as arteriovenous malformation, can produce profound hemodynamic changes and symptoms of shock.
-Occult hemorrhage: dyspnea, anemia, dullness to percussion, and decreased breath sounds over the area of the hemothorax
-Cardiopulmonary stabilization and evacuation of the pleural blood collection
-Tube thoracostomy drainage (70% to 80%), but not with significant pleural adhesions
-Video-assisted thoracoscopic surgery
-Postoperative ventilator management should progress according to the individual status of the patient.
•One of the most common and serious chronic diseases in childhood
•Adds a big financial burden to our healthcare system
-Exact cause unknown
-No cure
-Many cells and cellular elements play a role (mast cells, eosinophils, neutrophils, T lymphocytes, macrophages, and epithelial cells)
-Relationship between host factors (genetic and environmental exposures) that occurs during the development of the immune system early in life
-Bronchi appear to be hyperreactive to stimuli, increasing the production of immunoglobulin E (IgE), mast cell degranulation, and release of inflammatory mediators.
-Due to this response in the airway, the lung tissue swells, causing the airways to be overly reactive.
Mucus production increases, adding to the constriction of the airways.
-Inflammation, if left untreated, can cause permanent structural changes, also known as airway remodeling.
-Can be prevented
-Detailed medical history and physical examination
-Family history of asthma or allergies
-Presence of factors or stimuli that trigger the acute asthma exacerbation
-Spirometry in children older than 5 years of age
•FEV1 or FEV1/FVC pre/post SABA
-Airway response to bronchodilator treatment
-Bronchoprovocation challenge (methacholine or mannitol challenges)
-Allergy testing
-Sputum, blood, or urine testing
-Exercise tolerance tests
-Asthma severity: intermittent, mild persistent, moderate persistent, and severe persistent
-Varying degrees of respiratory distress, depending on the severity of airway constriction, inflammation, and obstruction
-Physical exam is normal except during exacerbation.
-Four key components:
(1)Assess and monitor asthma severity and control
(2)Partner with patients and families to educate them on their asthma plan of care
(3)Control environmental factors and comorbid conditions that trigger asthma exacerbation
(4)Prescribe appropriate medications for treatment of asthma
-Assess and monitor asthma severity and control
•Step-wise approach to asthma treatment based on an assessment of the child's severity and control
•Treatment: collaboration between family and clinical team to identify and minimize triggers, formulate a plan to improve asthma control, and enhance patient and family satisfaction
•Asthma care: "step up" during acute exacerbation, "step down" if asthma is controlled
•Asthma care plan
-Trigger identification
-Trigger avoidance
-Use of quick-relief medication
-Use of long-term controller medications
-Escalate/Deescalate
-Educate children and their families/caregivers
•Provided and reinforced during each opportunity
•Peak flow monitoring
•Asthma action plan
•Basic asthma facts
•Tailored education
-Control environmental triggers and comorbid conditions:
Allergens and irritants; Secondhand smoke exposure as well as smoking cessation; Air quality; Strong fumes (e.g., cleaning products, gas stoves, wood-burning stoves, etc.); Extreme weather changes; Upper respiratory infections
-Prescribe pharmacologic asthma treatment:
•Quick relief (rescue): SABAs; 3-5 minutes onset; 4-6 hours effect; Normally not on a regular basis
•Controller (maintenance): Daily use; Corticosteroids (systemic and/or inhaled), leukotriene modifiers, LABAs, theophylline); Humanized anti-IgE agents (Omalizumab)
-Emergency department treatment: Important access point for children experiencing asthma exacerbations, Early identification and classification of the exacerbation severity, Prompt involvement of the interdisciplinary team, Relieve bronchospasm, reduce airway inflammation, and prevent respiratory failure, First-line therapy: quick-relief medication with/without corticosteroids, Second-line therapy: continuous nebulization of beta2-agonists and a dose of IV MgSO4, Heliox and noninvasive positive-pressure ventilation
•Asthma Management foundation
•Follow up with PCP
•Usually will be seen in the ED again within a year
•The following children are more likely to return to the ED within one week of discharge:
Less than 2 years old, Persistent asthma, On government assisted insurance, Poor QOL reported, Increased use of healthcare resources for asthma in the past year
-Management in the hospital
•Poor response to therapy within the first few hours of treatment (2-4) in the ED or a repeat visit within 24 hours of discharge home from the ED
•Previous hospital admission requiring treatment in the ICU or ventilatory assistance
•Refractory to first- and second-line therapy and high risk for respiratory failure
•Elective intubation and ventilatory support
•First- and second-line therapies
Sedation (helps with ventilation)
•Ventilator Management:
Air trapping, Auto-peep, Hypotension, Pulmonary leak, Sedation often needed, ARDS, Early intubation, Continuous SABA inline
•Complications:
Missed school and work time, Reduced ability to lead a healthy, active lifestyle, Respiratory failure, Death
•Life-limiting autosomal recessive disorder
-Both parents must carry the gene
-Single gene defect on chromosome 7
-Carriers have few or no symptoms.
-Each child conceived from two CF carriers has a 25% chance of being affected with CF, a 50% chance of being a CF carrier, and a 25% chance of not having a gene defect.
-More than 1,800 individual mutations
-F508del mutation more common in Caucasians
-CFTR regulates epithelial sodium, chloride, and potassium channels, as well as bicarbonate transport.
-Loss of a normally functioning CFTR can have a profound impact on water and electrolyte transport across the epithelium.
-Pulmonary impact: reduction in intact mucin (mucus), making the airway more vulnerable to chronic bacterial infection and airway inflammation
-The sequelae of mucus retention: chronic bacterial infection, airway obstruction contribute to lung damage, which can be quantified by the measurable reduction in lung function
-Combination of one or more typical phenotypic features and evidence of CFTR malfunction
-PFTs
-Chest imaging
-Laboratory evaluation
-Newborn screening
•In the United States, all newborns are screened for CF.
•ImmunoReactive Trypsinogen (IRT) screens (not used in all states)
•Sweat chloride test: Children older than 6 months, Minimum 75 mg of sweat over 30-minute interval, Chloride concentration >60 mmol/L on two or more occasions is suggestive of CF., Highly recommended to perform testing at a Cystic Fibrosis Foundation-accredited center (errors are common)
•Genetic testing: Compliment to IRT and sweat testing, Identification of two disease-causing CFTR mutations, Detect up to 95% of CF alleles, Family history of CF
-Broad range of clinical features
-Pulmonary and extrapulmonarymanifestations
-Depend on a combination of genetic (modifier genes) and environmental factors
-Extrapulmonary manifestations: GI, Reproductive, Pacreatic, Metabolic
Gastrointestinal: -Meconium ileus occurs in approximately 18% of all newborns with CF, Intestinal obstruction may also occur after the newborn period, Typically, the obstruction occurs in the distal or terminal ileum and is associated with the passage of large amounts of food that are not completely digested into the small bowel., Rectal Prolapse, Malnutrition
-Hepatic: Biliary Cirrhosis
-Pancreatic: Pancreatic insufficiency from birth in a majority of patients with CF, Stools are foul smelling, bulky, and have an oily appearance, If uncorrected, malabsorption results in failure to gain weight and stunting of linear growth, Children with CF may present with signs and symptoms of vitamin deficiency, including fatigue, muscle aches and weakness, pale or yellowish skin color, and tingling of extremities, Gradual loss of insulin production, Clinical manifestations of CF-related diabetes include failure to gain or maintain weight despite nutritional intervention, poor growth, and unexplained decline in lung function, High fat diet and enzymes
•Reproductive tract:
Nearly universal male infertility (>98% due to obstruction of the vas deferens), Can not palpate vas deferens, only 20% of females experience infertility issues: changes in the cervical mucus that impair implantation.
•Sweat glands:
Reduced sodium chloride absorption in the sweat duct (elevated sweat chloride), Salt depletion, Lethargy, anorexia, and hypochloremic acidosis
-Pulmonary manifestations, Upper respiratory tract: Chronic rhino-sinusitis and opacification of paranasal sinuses, headache, facial pain, nasal obstruction, chronic congestion, and nasal discharge, Mucosal edema, purulent discharge, and nasal polyps, Wide nasal bridge, Sinus hypoplasia or aplasia with generalized opacification on CT scan
-Lower respiratory tract:
Early evidence of increased airway resistance and gas trapping, Increased number of goblet cells and submucosal gland hypertrophy, Productive and persistent cough, During an acute exacerbation, increased cough, sputum, fatigue, anorexia, weight loss, decreased lung function, and possibly respiratory failure, Bronchiectasis development
•Sputum Cultures guide antibiotic therapy
-Currently there is a shift in focus to preventive care, including delaying the onset of the initial and minimizing subsequent pulmonary infections and preserving lung function.
-Four key pillars of CF care:
1.Prompt identification and treatment of pulmonary infections
2.Suppression of airway inflammation
3.Relief of airway obstruction
4.Attention to nutritional status and nutritional support
•Prevent deterioration of lung function and treat acute exacerbations
•Physical removal of airway secretions
•Reduction of airway infection and inflammation.
•CFTR modulator: ivacaftor (Kalydeco) therapy drug that targets the underlying cause of CF, There are now more gene therapies available for CF patients
-Identification of pulmonary exacerbation:
Sputum culture and sensitivity and antimicrobial pharmacological treatments, Radiography tests (chest X-ray, CT, MRI), PFTs (usually spirometry only), Lung clearance index (LCI)
Treatment of CF lung disease focuses on therapies aimed at preventing deterioration of lung function and those used to treat acute exacerbations. Inhaled medications, including a proteolytic, antibiotics, anti-inflammatory medications and airway clearance therapy (ACT), postural drainage and clapping are essential components of routine care.
•Inhaled Medications
Dornase Alfa, Beta-adrenergic agonist, Hypertonic saline or mannitol, Antiobiotics (TOBI or Cayston)
•Airway Clearance Techniques: CPT, ACB, Autogenic drainage, Oscillatory PEP, HFCWO&C, Exercise
•Use of pancreatic enzyme replacement therapy and follow a high-calorie, high-protein, unrestricted diet
•During an acute exacerbation, increasing calorie intake is necessary to combat the higher energy expenditure due to the increased work of breathing.
Routine assessment of the child's nutritional status includes anthropometric parameters and, as the child matures, an annual assessment of body composition, bone density, glucose tolerance, and various biochemical and micronutrient levels
-complications: hemoptysis (Can be caused by vitamin K deficiency, Minor is common and usually self-limited, Massive comes from bronchial arterial circulation can lead to airway obstruction and asphyxiation, Be careful about holding airway clearance due to minor hemoptysis as it can make patient worse) pneumothorax (Usually due to the rupture of subpleural air cysts), respiratory failure (NIV for impending respiratory failure and for palliative care)
-outcomes: Life-limiting disease, Longevity and quality of life, Good outcome with improved quality of life in CF lung transplant