Chapter 8: Pulmonology

Question 1

Most common cause of congenital stridor

Answer 1

Laryngomalacia

large floppy epiglottis or floppy arytenoid cartilages

Question 2

Tx of Laryngomalacia

Answer 2

usually resolves w/ growth over the first 1-3 years

-most only need to be followed

Question 3

Children who present in first year w/ persistent stridor and or hoarseness most likely have?

Answer 3

Vocal Cord paralysis or laryngeal papillomatosis

-most only need to be followed

Question 4

Presentation of OSA

Answer 4

• restless sleep with frequent position changes
• irregular snoring
• daytime somnolence
• poor growth
• behavioral problems
• enuresis
• poor academic performance

Question 5

Study of choice for OSA

Answer 5

polysomnography

Question 6

Tx of OSA

Answer 6

First: try to normalize airway anatomy by removing enlarged tonsils and/or adenoids (if indicated)
If that doesn’t work: CPAP is indicated

Question 7

Two major LOWER airway obstructive diseases in childhood

Answer 7

1) Asthma
2) Cystic Fibrosis
3) Primary ciliary dyskinesia (rare)
* *most patients with asthma wheeze but not all patients who wheeze have asthma**

Question 8

Risk Factors for Asthma

Answer 8

• genetic predisposition
• atopy
• cigarette smoke exposure
• living in urban areas in poverty
• African American race
• Puerto Rican ethnicity
• Upper respiratory tract infections w/ certain viruses (rhinovirus, RSV) at critical times in early life

Question 9

What is cough variant asthma?

Answer 9

Relatively uncommon

• produces a chronic cough that may be triggered by exercise or noted primarily at night during sleep
• wheezing may or may not be present

Question 10

Patients w/ persistent asthma should have PFTs how often?

Answer 10

at least once a year

Question 11

What do baseline CXR look like in asthma?

Answer 11

Mild hyperinflation and/or increased bronchial markings

Question 12

Obstructive PFTs =

Answer 12

find it

Question 13

Restrictive PFTs=

Answer 13

find it

Question 14

Mainstays of medical treatment of asthma

Answer 14

• inhaled corticosteroids
• leukotriene receptor antagonists
• short acting B2 agonists

Question 15

What is tx of exercise induced asthma?

Answer 15

-use SABA 5-20 minutes before vigorous activity

Question 16

Most effective treatment for chronic asthma?

Answer 16

Inhaled corticosteroid treatment

Question 17

Theophylline

Answer 17

has fallen out of favor as a first line treatment option

Question 18

Patients >12 w/ severe allergic asthma that remains poorly controlled with use of inhaled corticosteroids and leukotriene receptor antagonists and LABS may benefit from treatment w/?

Answer 18

-Omalizumab an injectable monoclonal antibody directed against IgE
(expensive; must be given q2-4 wks)

Question 19

Other tx for severe obstruction

Answer 19

• Ipratropium (anticholinergic)
• SubQ epinephrine
• Terbutaline

Question 20

Factors that increase risk of death from asthma

Answer 20

• noncompliance
• poor recognition of symptoms
• delay in treatment
• history of intubation
• AA race
• steroid dependence

Question 21

Pathogenesis of Cystic Fibrosis

Answer 21

• autosomal recessively inherited multi system disease characterized by disordered exocrine gland function
• *most common in caucasians 1/3500**
• average life expectancy is mid to late 30s

Question 22

Nasal polyps

Answer 22

should prompt further testing for CF

Question 23

What bugs usually colonize patients w/ CF?

Answer 23

Early childhood = s. aureus and h. influx
Later = p. aeruginosa (90% acquire and it is rarely eradicated)
Particularly ominous = burkholderia cepacia (accelerated pulmonary deterioration and early death)

Question 24

Most common manifestation of untreated CF in infants and children

Answer 24

failure to thrive

Question 25

Meconium ileus is pathognomonic for

Answer 25

``` Cystic fibrosis (neonatal intestinal obstruction in the absence of anatomic abnormalities) ```

Question 26

Classic diagnostic findings of CF

Answer 26

- elevated sweat chloride concnetration - pancreatic insufficiency - chronic pulmonary disease

Question 27

Initial diagnostic study of choice

Answer 27

-sweat chloride test | level >60 is is generally considered abnormal

Question 28

Tx of CF

Answer 28

1) most fundamental = maintain effective airway clearance -chest PT, vigorous exercise, frequent couging -Recominbinant human deoxyribonuclease admin via neb breaks down thick complexes -Alternate months of inhaled tobramycin maybe indicated for patient infected w/ Pseudomonas (frequently bacterial infections have to be treated w/ IV abx) 2) Maintain Near normal growth -Pancreatic enzyme replacement -fat soluble vitamin replacement -high calorie/high protein diets (maintenance above 25th%ile = best prognosis) 3) may develop insulin deficiency

Question 29

Complications of CF

Answer 29

1) Hemopthysis | 2) Spontaneous Pneumothorax

Question 30

Primary ciliary dyskinesia

Answer 30

-symptoms are similar to CF or asthma -Dx is made by demonstration of abnormal ciliary beat under light microscopy or characteristic US changes in samples of ciliated cells obtained from scrapings of the nasal or bronchial epithelium TX: similar to the pulmonary tx of CF but not same risk of p.aeuroginosa infection

Question 31

Tracheomalacia presentation

Answer 31

(common cause of expiratory airway obstruction) -si-widening of posterior membranous portion of the trachea w/ dynamic collapse during exhalation (severe) or forced expiration (less severe) HARSH, BRASSY, CROUPY COUGH (often misdiagnosed as having recurrent croup)

Question 32

What makes you think tracheomalacia vs asthma?

Answer 32

* *wheezing of tracheomalacia is made worse by bronchodilator** - often misdiagnosed as severe asthma

Question 33

Tx of tracheomalacia

Answer 33

most require no intervention | -there are surgical procedures to help some children

Question 34

List some examples of restrictive lung disease

Answer 34

- pectus excavatum - pectus carinatum - severe scoliosis - marked obesity - any lesion that occupies intrathoracic space - pulmonary hemosiderosis

Question 35

Symptoms of restrictive lung disease

Answer 35

- exercise intolerance - tachypnea - eventual dyspnea

Question 36

Define apnea

Answer 36

cessation of breathing for longer than 20 seconds or pauses of any duration tha are associated w/ color changes, hypotonia, decreased responsiveness or bradycardia

Question 37

Apnea is: diagnosis or sign?

Answer 37

Sign that is potentially dangerous that requires aggressive evaluation to define the underlying cause

Question 38

What is ALTE?

Answer 38

Apparent life threatening event - this is how apnea may come to medical attention - cause is not found in about 50% - when no treatable cause is found infant may be placed on a him monitor that senses chest movement, heart rate and sounds alarm when child may be apneic or bradycardic

Question 39

Apnea and SIDS

Answer 39

Apnea of infancy does not increase the risk of an infant dying from SIDS. **this may be why home monitors have never been proven to decrease the likelihood of SIDS**