E2- pulmonology

what is the cause of pyriform aperture stenosis?

bony overgrowth at the anterior bony opening of the nose

which condition has clinical features of respiratory distress that worsens with feeding and improves with crying, craniosynostosis, pituitary abnormalities and the 2 front teeth combine into one big one?

pyriform aperture stenosis

treatment for pyriform aperture stenosis?

nasal stenting

tracheostomy

what should you suspect ff a child has unilateral foul-smelling nasal discharge?

nasal foreign body

if a child has nasal polyps, what disease should you be thinking of?

CF

treatment for nasal polyps?

steroids

restriction of tongue movement caused by a prominent lingual frenulum that is more common in males, can exacerbate/cause mouth breathing and sleep issues

lingual ankyloglossia (tongue tie)

treatment for lingual ankyloglossia?

frenotomy

failure of thyroid tissue to descend into the neck (remains at the base of the tongue) causing airway obstruction

lingual thyroid

treatment for lingual thyroid?

surgery if severe

lifelong thyroid hormone replacement

what condition has clinical features of:

- inspiratory stridor

- hoarseness

- aphona

- feeding disorders

laryngeal lesions

if a child has expiratory stridor, what should you be thinking?

tracheal anomalies

what condition is caused by the collapse of the supraglottic structure during inspiration (omega shaped) causing clinical features of inspiratory stridor that is worse when crying/feeding/supine and progresses over week to months?

laryngomalacia

what is the MC congenital anomaly of the larynx?

laryngomalacia

treatment for laryngomalacia?

self limiting → resolves by 12-18 months

if severe → refer to ENT

narrowing of the cricoid lumen that is either congenital or acquired (from trauma) and has clinical features of recurrent croup and biphasic stridor

subglottic stenosis

diagnostic testing for subglottic stenosis?

direct visualization only for symptomatic pts

what condition causes complete upper airway obstruction, presents with asphyxia at birth and requires emergent tracheostomy?

laryngeal atresia

rare congenital anomaly resulting in incomplete separation of the vocal cords and presents in infancy with respiratory distress and an unusual cry

laryngeal webs → tx is surgery

how does tracheal atresia present?

respiratory distress and die soon after birth

complete or nearly complete rings of cartilage that presents with sternal retractions, dyspnea, stridor, monophonic wheeze that doesnt respond to bronchodilators

tracheal stenosis

what is the diagnostic test and treatment for tracheal stenosis?

dx → bronchoscopy

tx → surgery

what condition is caused by abnormal flaccidity of the trachea during breathing leading to abnormal collapse on expiration and has clinical features of expiratory stridor at birth?

tracheomalacia

treatment for tracheomalacia?

self resolves in 6-12 mos

CPAP

surgery

what condition is caused by weak cartilage in the walls of the bronchus on expiration and causes features of recurrent infections and in severe cases, respiratory distress?

bronchomalacia

what are the 2 types of bronchomalacia?

primary → congenital absence of cartilaginous rings

secondary → extrinsic compression from enlarged vessel or cyst

a developmental anomaly of the lower respiratory tract characterized by hyperinflation of one or more of the pulmonary lobes (MC L upper lobe) that causes progressive respiratory distress of tachypnea, cyanosis and wheezing

congenital lobar emphysema

what is the diagnostic test for congenital lobar emphysema?

CXR → distention of affected lobe + mediastinal shift, compression and atelectasis of non-affected lung

treatment for congenital lobar emphysema?

lobectomy

uncommon condition characterized by incomplete development of the lungs that leads to respiratory distress, hypoxia, hypercarbia and sometimes neonatal death

pulmonary hypoplasia

what is a risk factor for pulmonary hypoplasia?

premature rupture of membranes and premature delivery

treatment for pulmonary hypoplasia?

extracorporeal membrane oxygen or mechanical vent until adequate lung growth

abnormal fistula between pulmonary arteries and veins that creates a pathologic intrapulmonary R→L shunt and impairs gas exchange

pulmonary arteriovenous fistula

which condition has clinical features of:

- dyspnea

- hemoptysis

- exercise intolerance

- associated with hemorrhagic hereditary telangiectasia (aka Osler-Weber-Rendu syndrome)

pulmonary arteriovenous fistula

treatment for pulmonary arteriovenous fistula?

ablation by angiography or surgery

an uncommon anomaly characterized by non-functioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its arterial blood supply from the systemic circulation

bronchopulmonary sequestration

what are the 2 types of bronchopulmonary sequestration?

interlobar → contained w/in the normal lung parenchyma (MC)

- tx is lobectomy

extralobar → separate from normal lung parenchyma & visceral pleural, has its own pleural covering

- diagnosed in utero

- tx is resection

what condition is characterized by excessive curvature of the thoracic spine with several lateral curvature causing rib distortion?

kyphoscoliosis

what are clinical features of kyphoscoliosis > 50 degrees?

restrictive changes on PFTs → hypoventilation

what are clinical features of kyphoscoliosis > 90 degrees?

cardiopulmonary compromise

treatment for pectus excavatum/carinatum?

surgery (cosmetic)

what condition does the bowel herniate through the diaphragm into the chest, causing arrest of lung development (pulm hypoplasia)?

congenital diaphragmatic hernia → posterolateral defect MC

what condition is common in the fall/winter months and daycare/school and is caused by viruses rhinovirus, coronavirus, adenovirus or coxsackie virus?

upper respiratory infection → fever, rhinorrhea, cough, congestion peaking at 2-7 days

what are complications of upper respiratory infections?

AOM

sinusitis

asthma exacerbation

PNA

are antihistamines recommended for treating upper respiratory infections?

NO! they cause ↓ in ciliary motility and ↓ mucus clearance

what condition is caused by inflammation of the tracheobronchial tree and results in features of rhinorrhea, cough (dry to productive), malaise and rhonchi on auscultation?

acute bronchitis → self-limiting, associated w recent viral URI

what is seen on labs (CBC, PFT and CXR) of acute bronchitis?

CBC → WBC normal or viral shift

PFT → airway obstruction

CXR → increased pulm markings

treatment for acute bronchitis?

symptomatic → saline sprays, nasal suctioning

frequent position changes in young infants

persistent cough lasting > 4 wks caused by asthma, CF, bronchectasis, foreign body aspiration, or cigarette smoke exposure

chronic bronchitis

viral infection that affects the lower respiratory tract (bronchioles) most commonly caused by RSV and seen in < 18 mos of age

acute bronchiolitis

what condition has features of:

- fever

- respiratory distress → tachypnea, retractions, nasal flaring, cyanosis

- auscultation → wheezing, crackles

- breathing pattern shall w rapid respirations

acute bronchiolitis

what are risk factors for acute bronchiolitis?

- gestational age < 36 wks

- age of infant in distress < 12 wks

- chronic pulmonary disease

- congenital anatomical defect

- CHD

- immunodeficiency

what is seen on CXR of acute bronchiolitis?

hyperinflation of the lungs

↑ AP diameter on lateral view

areas of consolidation → atelectasis 2* to obstruction/inflam

if a pt is diagnosed with acute bronchiolitis, what other diagnoses should you be thinking of?

asthma

CF

CHF

pertussis

foreign body

treatment for acute bronchiolitis?

saline sprays & nasal suctioning

albuterol in office

admit to hospital if in distress

what is the discharge criteria for acute bronchiolitis?

pt stable on room air w adequate oral intake

< 6 mos → < 60 breaths per min

6-11 mos → <55 breaths per min

> 12 mos → < 45 breaths per min

what is the prevention for acute bronchiolitis?

Synagis vaccine

MCC of PNA in children < 5?

viral → RSV

bacterial → S. pneumo, S. aureus, S. pyogenes

MCC of PNA in children > 5?

S. pneumo (MC)

mycoplasma pneumo

C. pneumo

if a pt has PNA and has a WBC > 15-20K, what should you be thinking?

bacterial cause

for those < 2 y/o, what should you suspect if:

- fever + cough

- resp distress → tachypnea, subcostal retractions, cough, crackles, ↓ breath sounds

PNA

what type of PNA:

- rhinorrhea, nasal congestion

- low grade fever

- nonproductive cough

- tachypnea with wheezing or crackles

- CXR → perihilar & parenchymal infiltrates

viral PNA (caused by RSV)

treatment for viral PNA?

supportive

rehydration

what is the tx for this PNA:

- MCC of "typical" PNA

- abrupt infection w fever, cough, tachypnea, malaise and emesis

- ↓ breath sounds, localized crackles

S. pneumo PNA → tx w amoxicillin

what is the tx for this PNA:

- PNA usually after rash disease

- complications are abscess and empyema

S. pyogenes PNA → PCN

what is the tx for this PNA:

- pts have recent URI (influenza)

- fever, cough, tachypnea

- CXR → pneumotoceles

- complications of pneumotoceles, PTX, abscess and empyema

S. aureus PNA → naficillin or vanco

what is the tx for this type of PNA:

- common in children > 5 y/o

- fever, malaise, HA

- sore throat, nonproductive cough

- extrapulmonary manifestations → splenomegaly, bullous myringitis, pharyngitis, confusion

mycoplasma PNA → azithro (alt tetracycline)

what is the tx for this type of PNA:

- seen in children > 5 y/

- presents WITHOUT a fever

- sore throat 2-3 wks before onset of PNA

Chlamydophila PNA → azithro

true or false:

neonates are at higher risk for TB meningitis, pleural TB and pericardial TB

true

what type of TB are < 10 y/o at risk of?

clinically silent or latent TB → +CXR but - sxs

what condition has features of:

- chronic, unremitting cough > 3 wks

- fever > 100.4 for > 2 wks

- weight loss

TB

what is the screening criteria for TB?

recently immigrated → 8-12 wks after immigration & repeat in 6 mos

no risk factors = no screening

how many negative TB screenings does a recently immigrated individual need to be considered "negative" for TB?

2

what are the diagnostic tests for TB?

tuberculin skin test (TST) aka PPD

IGRA (quantiferon TB gold)

CXR → isolated hilar/mediastinal LAD

what are the measurements of induration during a PPD test to be considered positive for TB?

15 mm for individuals w/o risk

10 mm for members of high-incidence population

5 mm for HIV+ & the immunosuppressed individuals

treatment for TB? (we should know this by now)

6 mos regimen = RIPE x 2 months then RI x 4 months

or

4 mos regimen = RIPE x 2 months then RI x 2 months (for non-severe pulm TB in ages 3m-15y)

a highly contagious acute respiratory illness transmitted via respiratory droplets that occurs in 3 phases:

- catarrhal

- paroxysmal

- convalescent

pertussis (whooping cough)

what occurs during each phase of pertussis?

catarrhal → sneezing, coryza, night cough, injected conjunctiva

paroxysmal → paroxysmal cough followed by high-pitched whoop

convalescent → ↓ in frequency & severity of cough

how is pertussis diagnosed?

bacterial culture, PCR and serology

if a pt < 4 months old has a cough that is not improving, rhinorrhea, vomiting, cyanosis, poor weight gain and labs show leukocytosis with lymphocytosis, what should you be thinking?

pertussis

if a pt > 4 months old has a paroxysmal nonproductive cough > 7 days, rhinorrhea that remains watery, posttussive vomiting and sweating between paroxysms, what should you be thinking?

pertussis

what is the treatment and prevention of pertussis?

tx → macrolides (alt = bactrim only if > 2 mos old)

prevention → DTaP in infancy + booster Tdap at age 13

what are cautions of using macrolides and bactrim for treatment of pertussis?

macrolides ↑ risk of infantile hypertrophic pyloric stenosis

bactrim îs not indicated younger than 2 mos d/t SE of kernicterus

when do you hospitalize a pt with pertussis?

< 4 months old

CBC → WBC > 30K

abnormal dilation associated w chronic lung infections and inflammation MCC is obstruction of airway & poor drainage from CF

bronchiectasis

what condition has features:

- persistent, productive/wet cough that is worse w exercise or changes in position

- dyspnea on exertion

- moist crackles/rales and rhonchi

- digital clubbing

- hemoptysis

bronchiectasis

what diagnostic tests should be done for bronchiectasis?

CXR → ↑ bronchovascular markings, atelectasis

- CT is more sensitive

sweat chloride test

PFT → obstructive pattern

treatment for bronchiectasis?

chest physiotherapy → clear airway

inhaled mucolytics (hypertonic saline)

flu/PNA vaccine

ABX for acute exacerbations

what condition is this:

- inherited disease of exocrine gland caused by mutation of chromosome 7 (CFTR gene)

- delayed meconium (aka meconium ileus)

- abnormal thick, viscous secretions that can lead to PNA or pancreatic obstruction

- abnormally high Na in sweat → salty tasting skin

- excessive appetite but poor weight gain

- nasal polyps

- greasy/bulky stools

- clubbing of nails

cystic fibrosis (CF) → >60 on sweat chloride test is positive

treatment for CF?

chest physiotherapy → vigorous clapping on chest & back to dislodge phlegm

inhaled hypertonic saline

CFTR modulator therapy (> 6 y/o)

what condition is this:

- nocturnal cough, cough that recurs seasonally or in response to specific exposures

- wheezing

asthma

- if cough is > 3 wks you should suspect asthma!

what is the severity of asthma:

- sxs < 2x/wk

- nighttime sxs < 2x/month

- < 2 days/wk use of inhaled SABA

- no interference w normal activity

- FEV1 > 80%

intermittent

what is the severity of asthma:

- sxs > 2x/wk but not daily

- nighttime sxs 3-4x/month

- > 2 days/wk use of SABA

- minor limitation of normal activty

- FEV1 > 80%

- exacerbation > 2x/yr

mild persistent

what is the severity of asthma:

- sxs daily

- nighttime sxs > 1x/wk but not daily

- daily use of SABA

- some limitation of normal activity

- FEV1 60-80%

- exacerbation > 2x/yr

moderate persistent

what is the severity of asthma:

- daily sxs

- nighttime sxs every night of the wk

- several times a day use of SABA

- extremely limited normal activity

- FEV1 < 60%

severe persistent

review of short acting relief vs long acting control names

short acting relief:

- albuterol, Xopenex, ipratropium, systemic steroids

long acting control:

- inhaled steroids (Advair), salmeterol, theophylline, montelukast

who is long term asthma therapy recommended in?

- > 3 episodes of wheezing in 12 month period

- lasting more than 1 day

- affects sleep

- risk factors → parent hx of asthma, atopic triad

review asthma treatment step ladder for 0-4 y/o

review asthma treatment step ladder for 5-11 y/o

what condition is described:

- fever/chills, hematuria, telangiectasia, clubbing

- MCC is infection, foreign body and bronchiectasis

hemoptysis