Pediatric Pulmonary Disorders Flashcards
1
Q
What is the leading cause of infant hospitalization in US?
A
Bronchiolitis
2
Q
What is bronchiolitis?
A
- Common, acute lower respiratory tract infection that primarily affects small airways
- Clinical syndrome of respiratory distress in children under 2 years of age
- Frequent cause of hospitalization in infants/young children
3
Q
What are characteristics of bronchiolitis?
A
- Upper respiratory symptoms followed by
- Acute onset of wheezing
- Crackles
- Hyperinflation
- Tachypnea
- Results in acute inflammation of airways
4
Q
What is wheezing/lower airway infection under 2 until proven otherwise?
A
Bronchiolitis
5
Q
Pathophysiology of bronchiolitis
A
- Virus attacks and causes inflammation in small bronchioles
- Edema, excessive mucus, and sloughed epithelial cells
- Leads to obstruction of small airways and atelectasis making it difficult to breath
- RSV cause in majority of cases, followed by enterovirus, rhinovirus, parainfluenza
6
Q
Epidemiology of bronchiolitis
A
- Yearly outbreaks during winter, spring, and small extent fall
- Primarily in first 2 years of life
- Most during 1st year of life
- Peak ages 1-10 months
7
Q
Risk factors for bronchiolitis
A
- Prematurity
- Age <12 weeks
- Cardiopulmonary disorders
- Anatomic defects of airways
- Immunodeficiency
- Neurologic disease
- Lack of breastfeeding
- Environmental causes
8
Q
What is history in bronchiolitis
A
- Onset in spring or winter
- Age
- Prior history of wheezing
- Recent history of signs compatible with common cold
- Decreased appetite
- Decreased sleep
- Increased fussiness
9
Q
Physical exam in bronchiolitis
A
- Increased respiratory rate
- Irritable
- Lethargic
- Retractions
- Expiratory grunting
- Prolonged expiration
- Cough
- Expiratory wheeze
- Otitis media
10
Q
Diagnosis of bronchiolitis
A
- Clinical
- O2 sat
- NP swab (do not need unless suspecting flu or for quarantine reasons)
- Imaging usually not necessary
11
Q
Treatment of bronchiolitis
A
- Nonsevere managed outpatient
- Supportive care and anticipatory guidance mainstays
- Adequate hydration
- Relief of nasal congestion (nasal suction)
- Monitoring for disease progression
- Education on clinical course and when to seek medical treatment for worsening symptoms
No steroids or abx! Can get worse around day 2-3 but should get better by week, cough/wheezing can be present for several months
12
Q
When should bronchiolitis be hospitalized?
A
- Persistently increased respiratory effort
- Hypoxemia O2<92%
- Apnea
- Acute respiratory failure
- Toxic appearance
- Poor feeding
- Lethargy
- Dehydration
- Parents unable to care for child at home
13
Q
Inpatient management of bronchiolitis
A
- Supportive care and anticipatory guidance
- Adequate hydration
- Respiratory care in stepwise approach: nasal suctioning, supplemental oxygen to maintain between 90 and 92%, infants at risk of respiratory failure receive trial of CPAP, ET intubation
- Monitor for disease progression
14
Q
When would ribavirin be used in bronchiolitis?
A
- Significantly immunocompromised patients
15
Q
Discharge criteria for bronchiolitis
A
- Respiratory rate <60 breaths per minute for age <6 months
- Patient stable using ambient air
- Caretaker knows how to clear infant’s airway using bulb suctioning
- Patient has adequate oral intake
- Caretakers confident can provide care at home
- Resources at home adequate to support
16
Q
What should be avoided in bronchiolitis?
A
- Inhaled bronchodilators: albuterol –> may have modest short-term effect but doesn’t affect outcome and may have adverse events, can be tried if patient is severe
- Systemic glycocorticoids: little effect
- Inhaled saline: some studies show efficacy, some don’t
17
Q
Patient education in bronchiolitis
A
- Return to office or ED if symptoms worsen
- Explain course of illness
- 18% symptomatic after 3 weeks
- 9% after 4 weeks, especially in young ingants
- Link to recurrent wheezing within 2 years of initial episode
- Some can have lung abnormalities beyond 10 years, but rare
18
Q
What is the course of bronchiolitis?
A
- Most improve w/in several days
- Cough/congestion resolve within 1-2 weeks
- Hospitalized patients discharged within 3-7 days
19
Q
What is Palivizumab?
A
- Humanized monoclonal antibody against RSV F glycoprotein
- Immunoprophylaxis with this may prevent hospitalization in certain infants
- First dose given before RSV season, followed by dose every 28-30 days throughout RSV season
20
Q
Recommendations for palivizumab
A
- Infants born at < or = 28 weeks, 6 days gestational age and <12 months at start of RSV season
- Infants <12 months of age with chronic lung disease of prematurity
- Infants <12 months of age with hemodynamically significant CHD
- Infants and children <24 months of age with congenital lung disease of prematurity necessitating medical therapy (supplemental O2, bronchodilator, diuretic, or chronic steroid therapy) within 6 months prior to beginning of RSV season
21
Q
What is nirsevimab?
A
- Long-acting monoclonal antibody for use in newborns and infants to protect against RSV
22
Q
When is nirsevimab recommended?
A
- All infant younger than 8 months born during or entering their first RSV season, including those recommended by the American Academy of Pediatrics to receive palizumab
- Infants and children aged 8 through 19 months who are at increased risk of severe RSV disease and entering their second RSV season, including those recommended by AAP to receive palivizumab
23
Q
Considerations for 2023-2024 RSV season in regard to plaivizumab vs nirsevimab admin
A
- If Nirsevimab administered, palivizumab should not be administered later that season
- If Palivizumab initially administered for season and <5 doses administered, infant should receive 1 dose of nirsevimab. No further palivizumab should be administered
- If palivizumab administered in season 1 and child eligible for RSV prophylaxis in season 2, child should receive nirsevimab in season 2 if available. If nirsevimab is not available, palivizumab should be administered as previously recommended
23
Q
Timing of nirsevimab
A
- First week of life for infants born shortly before and during RSV season based on geography
- Nirsevimab should be administered shortly before start of RSV season for infants younger than 8
- Administer shortly before start of RSV season for infants and children 8-19 months of age who are at increased risk of severe RSV
- May be given to age-eligible infants and children who have no yet received a dose at any time during the season
23
Q
Which children are recommended to receive nirsevimab in their second RSV season?
A
- Children with chronic lung disease of prematurity who required medical support any time during the 6-month period before the start of 2nd RSV season
- Severely immunocompromised
- Cystic fibrosis with manifestations of severe lung disease or weight-for-length that is <10th percentile
- American Indian and Alaska Native children
24
Q
Is coadministration of nirsevimab with vaccines recommended?
A
Yes
25
Q
What is cystic fibrosis?
A
- Autosomal recessive disease involving multiple organs, especially pancreas and lung
- Most common lethal genetic disease in US
- Usually develop obstructive disease, that leads to progressive respiratory failure and death
- MC in caucasians
26
Q
Pathophysiology of cystic fibrosis
A
- Mucociliary clearance problem
- Defect in CF gene on chromosome 7 that encodes CFTR (epithelial chloride channel)
- Problems with salt and water movement across cell membranes –> abnormally thick secretions
- CFTR located in lungs, upper respiratory tract, sweat glands, pancreas, intestines, liver, reproductive tract
27
Q
Diagnosis of CF
A
- Newborn screening (heel prick in hospital)
- One of following symptoms:
- Meconium ileus: delay pass of meconium –> abd distension
- Respiratory symptoms: recurrent pna, wheezing
- Failure to thrive: fall off growth curve in 1st few years
in 19th century diagnosed with salty sweat
28
Q
What is meconium ileus in CF
A
- Obstruction of bowel by meconium in terminal ileum
- Diagnostic of CF and should be presumed as having CF until a sweat test or genotyping obtained
29
Q
Other GI tract issues in CF
A
- Volvulus, intestinal atresia, or meconoium peritonitis d/t meconium ileus
- Poor hydration of intestinal contents, decreased pancreatic secretions, and fecal statis can cause distal intestinal obstruction syndrome
- Rectal prolapse d/t excessive straining or difficulty passing stool with defecation, or coughing d/t increased intraabdominal pressure
30
Q
Symptoms in respiratory/sinus tract of CF
A
- Chronic sinusitis
- Nasal polyps
- Persistent cough
- Lower respiratory tract: infections with S. aureus, HIB, and pseudomonas –> persistent production cough, cycle of infection and inflammation, destruction of airways –> bronchiectasis
- Complications such as pneumothorax, death from cor pulmonale, respiratory failure caused by recurrent pulmonary infections
31
Q
Pancreas conditions in CF
A
- Abnormal electrolyte secretion –> dehydration of ductal secretions –> blockage of ducts
- Destruction of pancreatic acini –> decreased pancreatic enzymes –> pancreatic insufficiency
- Diabetes, secondary to destruction of pancreatic islet cells
- Recurrent pancreatitis
- Pancreatic insufficiency –> malabsorption of fats, proteins, carbohydrates, and fat-soluble vitamins such as A,D,E, and K
- Children have a hard time growing/gaining weight, which results in failure to thrive
32
Q
Liver conditions in CF
A
Biliary cirrhosis with portal hypertension
33
Q
Impact of CF on MSK
A
Osteopenia and osteoporosis
34
Q
Impact of CF on electrolyte imbalance
A
- Increased electrolytes in sweat –> loss of body electrolytes –> metabolic alkalosis
35
Q
Impact of CF on GU
A
- Male infertility
- Reduced testicular size and testosterone levels
- Bilateral absence of vas deferens
- Delayed pubertal development d/t poor nutritional status and decrease in glucose tolerance
- Abnormal menstrual cycles
- Female infertility due to thick, sticky vaginal mucosa
36
Q
Gold standard CF diagnostic
A
Sweat chloride testing
37
Q
Sweat chloride testing indications
A
- Two weeks old and weight >2 kg
- Positive screening of newborn
- Meconium ileus
- Older children and adults with suggestive symptoms
- Siblings of patients with confirmed CF
38
Q
How is sweat chloride testing performed?
A
Collection of sweat with pilocarpine iontophoresis
39
Q
What is normal sweat chloride? Borderline? POsitive?
A
- Normal <30 mmol/L
- Borderline: 40-60 mmol/L
- Positive >60 mmol/L
40
Q
When would genotyping of CF be performed?
A
- After positive sweat chloride test
- Carrier status patients or those with borderline sweat chloride testing
41
Q
What is the purpose of fecal elastase testing?
A
- Screens those with CF for pancreatic insufficiency
- Checks for pancreatic elastase-1, absent in over 80% with CF
42
Q
General Treatment of CF
A
- Followed by CF foundation accredited care center
- Evaluation by pediatrician, pediatric pulmonologist, respiratory therapist, nurse, dietician, social worker
- Should be seen quarterly to check adequacy of therapies, as well as growth, nutrition, and pulmonary function
43
Q
Respiratory treatment of CF
A
- Airway clearance and aggressive antibiotic use
- Pulmozyne: mucolytic decreases viscosity of purulent CF sputum
- Hypertonic saline
- Inhaled bronchodilators
- Chest physiotherapy
- Antibiotics for chronic pseudomonas infections, screen sputum every 3 months, IV and inhaled tobramycin
- CFTR modulator drugs: Kalydeco, Orkambi, Trikafta
- Vaccinations
44
Q
GI treatment of CF
A
- Pancreatic enzyme supplementation combined with high calorie, high protein, high fat diet
- Daily vitamins
- Caloric supplements
- G-tube placement and supplemental feedings in FTT
45
Q
`
Signs and symptoms of acute CF exacerbation
A
- New/increased cough
- New/increased sputum production or chest congestion
- Decreased exercise tolerance or DOE
- increased fatigue
- Decreased appetite
- Dyspnea at rest/increased RR
- Change in sputum appearance
- +/- fever
- Increased nasal congestion
46
Q
Treatment of acute CF exacerbation
A
- Systemic abx treatment always
- Identify from sputum cultures
- At least one antibiotic to cover each pathogenic bacteria that is cultured from respiratory secretions and two for P. aeruginosa infections
47
Q
Prognosis of CF
A
- Lung transplant: 5-year post survival 50-60%, risky, anti-rejection drugs
- Median survival 47 years, rate of lung disease progression determines survival rate
48
Q
What is Infant Respiratory Distress syndrome primarily a disease of?
A
- preterm infants
- especially of diabetic mothers
49
Q
Pathophysiology of Infant respiratory distress syndrome
A
- Surfactant expressed in lung in third trimester reduces alveolar surface tension allowing expansion of lungs
- Deficiency of pulmonary surfactant in immature lung = primary cause
- Noncompliant, stiff lungs that are structurally immature with insufficient surfactant –> amount of pressure needed to open alveoli increased leading to atelectasis at end expiration
- –> ventilation/perfusion mismatch, hypoxemia, hypercarbia, persistent HTN
50
Q
Clinical features of IRDS
A
- Almost always premature
- Presentation min to hours after birth
- Intercostal/subxiphoid retractions
- Tachypnea
- Nasal flaring
- Diminished breath sounds
- Cyanosis
- Expiratory grunting
51
Q
Diagnosis of IRDS
A
- Premature infant with onset of progressively worsening respiratory failure shortly after birth
- CXR shows low lung volume and a classic diffuse ground-glass appearance
- Pulse ox
- ABG
`
52
Q
Treatment of IRDS
A
- Basic principles of neonatal care: thermoregulation, cardio support, nutritional support, early infection care
- Nasal CPAP initial preferred intervention
- Surfactant replacement
53
Q
Prevention of IRDS
A
- Prevention of prematurity, asphyxia, avoidance of maternal fluid overload
- Prenatal administration of single course of steroids to women in preterm labor or at risk of delivery within next 7 days between 24-34 weeks gestation
- Dexamethasone
- If PROM give steroids and wait 1 week
54
Q
What is thyroglossal duct cyst?
A
- Cyst of epithelial remnants of thyroglossal tract
- Most common form of congenital neck mass
- Presents as midline neck mass at level of thyrohyoid membrane, closely associated with hyoid bone
- Arises as cystic expansion of remnant of thyroglossal duct tract
- Theory that lymphoid tissue associated with tract hypertrophies at time of regional infection, occluding tract with resultant cyst formation
55
Q
Clinical features of thyroglossal duct cyst
A
- Midline upper neck mass that is cystic
- No symptoms, may be slightly tender
- Often preceding upper respiratory infection: cyst from base of tongue to level of suprasternal notch, moves superiorly when swallowing
- Can become infected and have some degree of inflammation at presentation
56
Q
Diagnosis of thyroglossal duct cyst
A
- CT of neck with contrast: confirms diagnosis and gives info on size, extent, and location
- Fine-needle aspiration to diagnose or exclude other diagnoses
- MRI
- US –> would start with US to see texture but CT/ FNA definitive
57
Q
Treatment of TDS
A
- Surgery with sistrunk procedure
- Do not perform surgery during acute inflammation or infection
58
Q
What is sistrunk procedure?
A
Resection of cyst and mid-portion of hyoid bone in continuity and resection of a core of tissue from hyoid upwards towards foramen cecum
59
Q
What would you do if acute inflammation or infection of TDS?
A
- Treat with broad spectrum antibiotics
- Augmenting (amoxicillin-clavulanate)
- Clindamycin
- Cephalexin
- Surgical excision after inflammation/infection controlled
