Pulm Disorders - Exam 1 Flashcards by Johannah Crumpton

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

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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

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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

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What is the #1 virus that causes bronchiolitis? What are 3 additional ones?

RSV

enterovirus, rhinovirus, parainfluenza

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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

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What are the risk factors for bronchiolitis?

premature babies

younger than 12 weeks old

cardiopulm disorders

anatomic defects of airways

neurologic dz

lack of breastfeeding

environmental causes

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How do you dx bronchiolitis?

clinicial dx!!

need to check O2 stat: (normal is 93% and above)

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What is the tx for bronchiolitis in the outpt setting?

supportive care

adequate hydration

relief of nasal congestion

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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

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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

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**______ is an antiviral with good treatment response for bronchiolitis. **Only given to ____ pts

Ribavirin

significantly immunocompromised

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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

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What 3 things are NOT routinely used in the bronchiolitis?

inhaled bronchodilators

systemic steroids

inhaled saline

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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

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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.

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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

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______ is the LONG acting monoclonal antibody product to protect against RSV.

Nirsevimab

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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.

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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

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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

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Is it safe to give nirsevimab at the same time as other vaccines?

YES!! do NOT need to alter vaccine schedule in any way

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How is CF inherited? How common is it?

Autosomal-recessive disease involving multiple organs, especially pancreas and lungs

1:3,200 in Caucasians

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_____ is the most common lethal genetic disease in the US

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**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