Pulm Disorders - Exam 1 Flashcards
What is bronchiolitis? Upper or lower airway? What is the age requirement?
A common, acute lower respiratory tract infection that primarily affects the small airways (bronchioles)
LOWER airway
under 2 years of age
What is the underlying cause of bronchiolitis? What will it present like on PE?
Due to acute inflammation of airways
rhinorrhea, wheezing, crackles, hyperinflation and tachypnea, decreased appetite and sleep, increased fussiness, increased resp rate, OM, prolonged expiration
bronchiolitis occurs secondary to _______ that attacked and causes inflammation in the small bronchioles. What other factors make it hard for the kiddo to breath?
VIRUS
Causes edema, excessive mucus and sloughed epithelial cells that lead to obstruction of small airways and atelectasis making it difficult for a child to breath
What is the #1 virus that causes bronchiolitis? What are 3 additional ones?
RSV
enterovirus, rhinovirus, parainfluenza
What time of the year does bronchiolitis occur the most? What age group is the most susceptible?
winter, spring
More than 80% of cases occur during 1st year of life. Peak age 1-10 months old
What are the risk factors for bronchiolitis?
premature babies
younger than 12 weeks old
cardiopulm disorders
anatomic defects of airways
IC
neurologic dz
lack of breastfeeding
environmental causes
How do you dx bronchiolitis?
clinicial dx!!
need to check O2 stat: (normal is 93% and above)
What is the tx for bronchiolitis in the outpt setting?
supportive care
adequate hydration
relief of nasal congestion
What are some s/s you want parents to be aware of to monitor for in their kiddo, if any are noticed need to go to ED?
increased RR
grunting when breathing
retractions
pulse ox 91 or below
no eating/dehydration
toxic appearance
What is the tx for bronchiolitis in the inpt setting?
hydration!!
nasal suctioning
supplemental oxygen to keep stat between 90-92
trial of CPAP
consider ET intubation if needed
**______ is an antiviral with good treatment response for bronchiolitis. **Only given to ____ pts
Ribavirin
significantly immunocompromised
What is the discharge criteria for bronchiolitis?
Respiratory rate < 60 breaths per minute for age < 6 months
Caretaker knows how clear infant’s airway using suctioning, confident in providing care, and enough resources at home to provide adequate support
Patient is stable using ambient air for at least 6-12 hours
Patient has adequate oral intake
f/u appt scheduled
What 3 things are NOT routinely used in the bronchiolitis?
inhaled bronchodilators
systemic steroids
inhaled saline
What is the important pt education for bronchiolits with regards to s/s improvement timeframe?
most super bad symptoms improve within several days
Cough/congestion resolve w/in 1 - 2 weeks
completely improvement could take up to 3 weeks
When is Palivizumab (Synagis) usually given?
The first dose of palivizumab injection is usually given before the beginning of RSV season, followed by a dose every 28 to 30 days throughout RSV season.
What 4 pt populations qualify for Palivizumab?
- 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
______ is the LONG acting monoclonal antibody product to protect against RSV.
Nirsevimab
What are the 2 inclusion criteria for Nirsevimab?
- All infants younger than 8 months born during or entering their first RSV season, including those recommended by the American Academy of Pediatrics (AAP) to receive palivizumab;
- 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 the AAP to receive palivizumab.
What are the 4 considerations with regards to the timing of Nirsevimab vaccine?
- give vaccine the first week of life for infants born shortly before and during the RSV season
- Nirsevimab should be administered shortly before the start of the RSV season for infants younger than 8 months.
- Nirsevimab should be administered shortly before the start of the RSV season for infants and children 8 through 19 months of age who are at increased risk of severe RSV disease
- Nirsevimab may be given to age-eligible infants and children who have not yet received a dose at any time during the season
What 4 populations should get the nirsevimab in their SECOND RSV season?
- chronic lung dz of prematurity that requires chronic steroids, diuretics or O2
- severely IC
- CF with severe lung disease
- American Indian and Alaska Native children
Is it safe to give nirsevimab at the same time as other vaccines?
YES!! do NOT need to alter vaccine schedule in any way
How is CF inherited? How common is it?
Autosomal-recessive disease involving multiple organs, especially pancreas and lungs
1:3,200 in Caucasians
_____ is the most common lethal genetic disease in the US
CF
**What is the genetic cause of CF? What does it result in?
**Defect in CF gene on chromosome 7 that encodes an epithelial chloride channel (CF transmembrane conductance regulator protein)—(CFTR)
mucociliary clearance problem
This in turn leads to problems in salt and water movement across cell membranes resulting in abnormally thick secretions
How is CF diagnosed? What are other effected organ systems?
newborn screening
meconium ileus, volvulus, intestinal atresia, decreased pancreatic secretion, rectal prolapse
In a meconium ileus, where does the obstruction occur?
terminal ileum
How does CF effect the upper respiratory tract?
Chronic sinusitis
Nasal polyps
Persistent cough
What pathogens are common the lower respiratory tract in CF?
S. aureus, H. influenzae B, and P. aeruginosa
What does destruction of the airway in CF lead to? What complications are likely?
bronchiectasis
Complications such as pneumothorax, respiratory failure caused by recurrent pulmonary infections, and death from cor pulmonale
How does CF effect the pancreas?
Abnormal electrolyte secretion leads to dehydration of ductal secretions and blockage of ducts
Destruction of pancreatic acini (cells that produce digestive enzymes), which leads to decreased pancreatic enzymes
recurrent pancreatitis which leads to insufficiency which leads to malabsorption of fats, proteins, carbs, and vit A, D, E, K which all lead to hard time gaining weight -> failure to thrive
What does destruction of the pancreatic acini lead to? pancreatic islet?
Leads to pancreatic insufficiency, which occurs in 85 - 90% of CF patients
diabetes results from the destruction of pancreatic islet cells
What electrolyte imbalance do kids with CF prone to?
Increased electrolytes in sweat, which can lead to loss of body electrolytes, resulting in metabolic alkalosis
Male pts with CF are ______ and both genders sometimes have trouble with ______
95% of males with CF are infertile
hormonal issues and delayed puberty (Reduced testicular size and testosterone levels and abnormal menstrual cycles)
______ is the gold standard to dx CF
sweat chloride test
What are the sweat chloride test requirements? _______ medication is used as part of the test
Two weeks old and weight > 2kg
pilocarpine
**What value is considered normal for the sweat chloride test? borderline? positive?
normal: < 30 mmol/L
borderline: 40 - 60 mmol/L
positive: > 60 mmol/L
What do you do next if the sweat chloride test comes back boderline?
need to repeat testing or confirm with genetic testing
When is genotyping for CF indicated? How many mutations have been identified?
Indicated after a positive sweat chloride test
Over 1800 CF mutations have been identified
______ screens those with CF for pancreatic insufficiency. What is it checking for?
fecal elastase
Checks for pancreatic elastase-1, absent in over 80% or more of those with CF
What is the management plan for a kid with CF? Who is part of the team?
followed be a CF foundation accredited care center
pediatrician, pediatric pulmonologist, respiratory therapist, nurse, dietitian, and mental health
To help maintain airway clearance _____ are mainstays of CF treatment
aggressive abx
________ is used in CF to decrease viscosity of CF sputum. What are 3 additional therapies used to keep lungs clear?
Pulmozyme (recombinant human DNAse)
hypertonic saline
inhaled bronchodilators
chest physiotherapy
CF pts are likely to have _____ chronic infections and need ________. What is the tx?
pseudomonas infections
Screening sputum cultures every 3 months
IV and inhaled tobramycin
Kalydeco, Orkambi, Trikafta are all used in the tx of CF. What is the drug class?
CFTR modulator drugs
What is the tx for the GI aspect of CF?
Pancreatic enzyme supplementation combined with high calorie, high protein, high fat diet
Daily vitamins
Caloric supplements
What is the tx for a CF exacerbation? How many do you need for pathogen?
systemic abx tx!!!!!
tx based off of sputum cultures
at least one antibiotic to cover each pathogenic bacteria that is cultured from respiratory secretions and TWO for P. aeruginosa infections
______ is also a tx for CF but is risky. What is the median survival age? What does it depend on?
Lung transplant
47 mean survival age
Rate of lung disease progression
Infant Respiratory Distress Syndrome primarily effects _____ infants and those born to _____ mother
preterm
DM
What is the primary cause of Infant Respiratory Distress Syndrome?
Deficiency of pulmonary surfactant in an immature lung
pts tend to have noncompliant, stiff lungs that are structurally immature and contain insufficient surfactant.
Due to lack of surfactant, the amount of pressure needed to open alveoli is increased leading to atelectasis at end expiration, which leads to ventilation / perfusion mismatch, resulting in hypoxemia, hypercarbia, and persistent HTN
What are the clinical features of IRDS?
prematurity
s/s start min/hours after birth
tachypnea with retractions
nasal flaring
diminished breath sounds
cyanosis
How do you make the dx of IRDS? What will the CXR show?
Made by clinical picture of premature infant with onset of progressively worsening respiratory failure shortly after birth
Chest x-ray will show low lung volume and a classic diffuse ground-glass appearance
_____ is the initial preferred intervention in IRDS. Then what? What is the prevention?
Nasal CPAP
surfactant replacement
single course of steroids in preterm deliveries between 24 and 34 weeks
What are thyroglossal duct cyst? Where do they present?
Cysts of epithelial remnants of thyroglossal tract
midline cystic neck mass closely associated with the hyoid bone but can be anywhere from base of tongue to the level of suprasternal notch
What do thyroglossal duct cyst arise from?
Arises as a cystic expansion of a remnant of the thyroglossal duct tract - stimulus for expansion not fully known
_____ is the MC form of congenital neck cyst. Does it move with swallowing?
Thyroglossal duct cyst
moves superiorly with swallowing
How do you dx TDC? ______ is also used to dx or to exclude other diagnoses
CT of the neck w/ contrast
fine needle aspiration
What is the tx for TDS? Explain. What abx?
Surgery is indicated with the Sistrunk procedure
A resection of cyst and mid-portion of hyoid bone in continuity and resection of a core of tissue from the hyoid upwards toward the foramen cecum
Amoxicillin-clavulanate
Clindamycin
Cephalexin
What 2 things should you NOT do in tx of TDS?
do NOT incise and drain!!!
do NOT remove if the duct is acutely inflamed or infections
Surgical excision after inflammation/infection controlled
