Pulmonology Flashcards
Defect in outer/inner dynein arms in more than 50% of cilia examined OR central microtubules of cilia
Dx?
How does this effect reproductive health?
Primary Ciliary Dyskinesia
Hypomotile sperm
Mode of inheritance PCD
AR
Situs inversus
Recurrent respiratory infections
Infertility
Primary ciliary dyskinesia
Testing for primary ciliary dyskinesia
- FeNO (would be low)
- Sweat chloride (to rule out CF)
- genetic (though lots of variants, 70% detectible)
- Now no longer the best test – Immunofluorescence using antibodies to dynein arm proteins can detect abnormal structural proteins in the cilia.
- high speed video fluorescence
Pleural effusion characteristics of congenital chylothorax
exudative
high lymphocytes
low pH
bilateral
decreased lymphatic drainage
milky
The most common cause of pleural effusion in children is __
community-acquired pneumonia (CAP)
Tx for croup if there is stridor or barky cough at rest
dexamethasone
Diagnostic criteria for persistent bacterial bronchitis
If no other signs/sx/hx of other diseases AND >6yo AND
1. Lasted >4 weeks
2. No abnormal findings on PFT
chronic brassy, barking cough that disappears with distraction and when the child is asleep
Habit cough
chronic wet cough associated with recurrent otitis and/or sinusitis suggests __
immunodeficiency or ciliary dyskinesia
dry cough that is exacerbated by exercise and associated with frequent night awakenings is often caused by __
asthma or airway reactivity
premature baby with h/o intubation, now discharged with stertor, poor weight gain, coarse wheezing that doesn’t respond to bronchodilators probably has ___
tracheomalacia
when should laryngomalacia improve in infants?
18-24mo
which can resolve on it’s own? laryngomalacia or tracheomalacia?
Laryngomalacia
When can you reassure parents vs. refer to ENT for stridor?
Child is feeding well
Good growth
There is intermittent resolution of stridor with position
Lack of respiratory compromise
when to consider bacterial tracheitis
had viral infection for some days with acute worsening, high fever, BARKING COUGH, wheeze, RETRACTIONS
most common cause of bacterial tracheistis
staph aureus
then pseudomonas, moraxella, strep pyo, GBS
Onset of hemangiomas
8-12 weeks (when growth of vascular malformation is at its peak)
Imaging of choice for evaluation of central vascular anomalies.
MRI
Congenital pulmonary airway malformation can present as __
cystic lesions
persistent cough not better with meds
70% diminish/resolve before birth if detected in fetal US
If child has hypoxia with infectious insults, think __
pulmonary HTN
most common cause of hemoptysis in children
infection and bronchiectasis
repeated episodes of subclinical pulmonary hemorrhage that may present with cough and pink frothy sputum, multifocal infiltrates, w/o evidence of autoimmune disease
Dx?
Pulmonary Hemosiderosis
First thing you look for in Pulmonary hemosiderosis?
CBC – usually has anemia
Antitrypsin deficiency in children most often manifests as ___
hepatic disease
What can pulm hemosiderosis be associated with and what antibodies are present?
milk allergy – kids (IgG4)
mold allergy – infants
sequelae of congenital diaphragmatic hernia after repair
pHTN, recurrent hiatal hernia, GERD, growth failure
if pHTN resolves, then cor pulmonale not likely
recurrent sinopulmonary infections and a history of unexplained neonatal respiratory distress, sinus invertus
Primary ciliary dyskinesia
Normal range of sweat chloride
Normal <30
Intermediate 30-59
Elevated >60
Bronchiectasis in CF occurs first in ___ lobes, vs. in PCD, they are in __ lobes
upper – CF
mid lung fields – PCD
Definition of apnea of prematurity
cessation of air flow for >20s OR <20s but with bradycardia +/- cyanosis
when does apnea of prematurity stop
36-40 wk GA
when to stop using apnea monitor if doing well
until 43 weeks GA
tx for aop
caffeine (methylxantine)
Definition of bronchopulmonary dysplasia
need for supplemental O2 for at least 28 days after birth and at 36wk corrected GA
Pathophys of neonatal respiratory distress syndrome
immaturity of lung = decreased lung compliant, need surfactant
Risk of RDS is increased in __
diabetic mothers
Complications of RDS
air leaks & pulmonary hemorrhage
When to give corticosteroids to mothers (GA)
Preterm delivery before 34 weeks
What is pulmonary interstitial emphysema (PIE)
Rupture of small airways resulting in gas in interstitium
Usually in neonates with RDS with mechanical ventilation
Which side to place neonate on if there is PIE
affected side down
Bacteria implicated in EARLY ONSET neonatal pna from transplacental organism (4 bacteria, 4 virus)
GBS (most common)
Listeria
MTB
Treponema pallidum
Rubella
CMV
HSV
Adenovirus
Late onset neonatal PNA causes
ConS, S. aureus, gram negs (if vented)
atypicals: chlamydia trachomatis
viral
Term neonate born with asymmetric patchy infiltrate, tachypnea retractions within the first 12h of life
MEC aspiration syndrome
Can cause air leaks
treatment for PPHN
iNO
phosphodiasterase inhibitor (sildenafil, milrinone)
Endothelin receptor antagonist (bosentan)
Type B surfactant protein deficiency
What is it and how is it inherited
Full term, presents within few hours of life with rapidly progressive respiratory failure and hypoxemia
AR
Type C Surfactant protein deficiency
What is it and how is it inherited
Variable
AD vs sporadic
How do you diagnose and treat type B surfactant protein deficiency
Biopsy
Lung Tx
What syndromes are Tracheoesophageal fistulas associated with
Trisomy 18
Trisomy 21
DiGeorge
VATER
VACTERL
CHARGE
Fetal presentation of TEF
polyhydramnios and small stomach
Dx of TEF
place NGT and take xray
H-type dx with barium study with contrast material infused through nasogastric tube
Transient tachypnea of newborn risk factors
C-section
Maternal DM
Maternal Asthma
Male sex
Low birth weight
Macrosomia
CPAM (congenital pulmonary airway malformation) pathophys/anatomical issue
multicystic mass of pulmonary tissue
vasculature is from pulmonary circulation (rather than bronchial)
Most CPAM presents by age __
6 mo if symptomatic
What is congenital lobar emphysema?
hyperexpanded lobe with
1. normal amount of alveoli
OR
2. increased number of alveoli
Air trapping occurs
Bronchopulmonary sequestration pathphys/issue
BPS: isolated segment of lung with systemic circulation (thoracic or abdominal aorta) with venous drainage to RA (R to L shunt)
can be within parenchyma (intrapulmonary) vs extrapulmonary
CPAM pulmonary circulation
Where is bronchopulmonary sequestration usually found
left sided, between lowerlobe and diaphragm
CPAM and BPS has increased risk of ___
infection
Issue caused by CPAM and BPS
mass effect,
BPS: high output cardiac failure (overcirculation)
Bronchogenic cyst - how does it form
piece of bronchial tissue separates from developing airway –> epithelium lined sac that contains cartilage in wall
sometimes there’s connection (most); sometimes there is –> increased infection risk
Problems from bronchogenic cysts
mass effect –> stridor or feeding difficulties
How to diagnose bronchogenic cyst
barium swallow and CT scan
Syndromes associated with Congenital diaphragmatic Hernia
trisomy 21, 18, 13
Beckwith-Wiedemann sd
Goldenhar
What does Potter syndrome cause
Etiology: renal agenesis
–> severe pulmonary hypoplasia due to oligohydramnios
What is pulmonary lymphangiectasia
dilation of ppulmonary lymphatic vessels and disordered drainage –> accumulation of lymph within the lungs
high mortality rate
How to diagnose pulm lymphangiectasia
chest xr: interstitial infiltrates/ hyperinflation
CT: thickening of peribronchovascular septa and septa surrounding lobules
Gold standard is BIOPSY: dilated lymphatic vessels in interlobular septa with thickening/widening of interlobular septa
Laryngomalacia starts and peaks at ___ months
2-4 mo of life
When does mild laryngomalacia resolve
12 - 18 mo
Nerve innervating vocal cords
recurrent laryngeal nerve
Bilateral vocal cord paralysis usually caused by ___
central abnormality
1. Arnold-Chiari malformation
2. hydrocephalus
and more…
Acquired VCP resolves by __ mo
6-12 mo
if no resolution by 2yo, won’t resolve by itself
Neonatal biphasic stridor
fixed obstruction (subglottic stenosis that is severe)
Tx for tracheomalacia
bethanechol (cholinergic agent that can increase smooth muscle tone in trachea
Ipatropium to increase tracheal smooth muscle
B2-agonist worsens
Manage GERD
Usual age for croup
6-36mo
When to add racemic epi for croup, and when to consider heliox
Racemic epi: audible stridor at rest, retractions at rest
Heliox: severe retractions and significant agitation, pronounced stridor, ill appearing, not taking PO
recurrent barking cough without viral prodrome
spasmodic croup
Management of spasmodic croup
look for allergic cause
Eval for GERD
AP/Lat neck radiographs
ENT if didn’t find anything
Most common cause of epiglotitis
h. influenzae, H. parainfluenzae, S. pneumonia, S. aureus
Diff between croup and epiglottitis
epiglotittis: toxic, hoarse voice
croup: can appear well, cough
Tx of epiglotitis
ceftriaxone
Epiglotittis age vs. Croup vs. bacterial tracheitis
Croup young 6-36mo
Epiglotitis kindgergarted 2-7yo
Bacterial tracheitis wide range 6mo to 8year (usually older)
Barking cough/stridor that doesn’t resolve with racepic epi or steroids
Bacterial tracheitis
In children with suspected OSA and T&A is performed, when do you get a repeat polysomnography?
6-8 weeks
Age of bronchiolitis
<2yo
When to give pavilizumab and to whom
Infants born <29 wk GA, every month during RSV season until 12mo.
Also infants born after 29wk GA with CHD or CLD
Age for high risk of foreign body aspiration
3yo
Children with developmental delay
Children with bronchiectasis are susceptible to recurrent infections from ___ (3 bugs)
h. influenza
strep
moraxella
infant 3wk - 3 mo old with staccato cough. Dx?
chlamydia pneumonia
Pneumonia in mississippi, misouri or Ohio River valley. Cause?
Histoplasma
Pneumonia in Southeast US and Great Lakes?
Blasto
someone owns birds and now has pneumonia, cause?
chlamydophila psittaci
someone has a farm and now has pneumonia, cause?
coxiella burnetti
pleural effusion with elevated amylase, ddx (3)
malignancy, pancreatitis, esophageal rupture
Definition of empyema
pH <7.3
Glucose <60
exudative
LDH elevated
+/- gram stain culture
ptx management (non-invasive)
max NRB for washout of normal air with high O2, to increase resorption
CFTR protein malfunction causes ___ to the electrolytes
- Decreased Chloride secretion
- Increased sodium absorption from airway lumen
most common CFTR mutation
deltaF508
Role of PMNs in CF
Neutrophil nets create oxidants, elastase, and other proteases, leading to destruction of the bronchial walls
Lumacaftor/ivacaftor (what is it and what does it treat?)
CFTR modulator
Improves misfolding and gating abnormality in CF
DeltaF508 mutation (severe phenotype)
Ivacaftor only (what does it do and what does it treat?)
CFTR modulator
Treats CF with G551D mutation
Improves gating abnormality
CF and pancreatic disease, which is defective first? exocrine or endocrine?
Exocrine
Reproductive dysfunction in CF is caused by what pathophysiology?
In men:
In women:
Men: congenital bilateral absence of vas deferens CBAVD (no spermatozoa present in semen – obstructive azoospermia) 95%
Women: Viscous cervical mucus, advanced lung disease, malnutrition
80-90% of infants with meconium ileus has ___
CF
Distal intestinal obstruction syndrome is associated with ___
CF (older kids)
“sausage-like mass in RUQ”
Equivalent to meconium ileus
What do you collect sweat with for sweat chloride test
pilocarpine inontophoresis
Next steps after intermediate sweat chloride result
repeat test and genetic analysis
Diagnosis of CF
- Clinical features or CF in sibling or +newborn screen
AND - elevated sweat chloride or
- Nasal potential difference (more negative charge due to hyperabsorption of Na) or
- identification of two disease-causing CF mutations
Different types of newborn screening for CF (3)
- Immunoreactive trypsinogen assay (IRT): pancreatic dysfunction if elevated (80% sensitive)
- DNA assay: primary or secondary screen (some only tests delta F508, others test 28-40). IRT + DNA has 96% sensitivity
- Sweat test: +IRT and/or positive genetic screen to confirm diagnosis
At what age is inhaled DNAse recommended and for what disease
> 6yo
CF
what’s fibrosing colonopathy
giving too much exogenous lipase can cause this side effect in CF patients
Definition of restrictive lung disease on PFT
<80% pred TLC
<80% FEV1 and FVC
>80% FEV1/FVC ratio
Definition of reestrictive lung disease on PFT
<80% pred TLC
<80% FEV1 and FVC
>80% FEV1/FVC ratio
In pts with neuromuscular diseases, how frequent should they follow up with pulmonologists?
Ambulatory: annually
Wheelchair-bound: biannual
FVC <60% quarterly visit, annual polysomnogram
Options for secretion and dysphagia management (4)
- anticholinergic medications that thicken secretions
- Botulinum toxin injection to salivary glands to decrease saliva production
- Enteral tube feeding
- OT
Bronchiolitis Obliterans
concentric bronchiolar narrowing due to inflammation and fibrosis
associated with CVID
Types of granulomatous lymphocytic ILD and what type of immunodeficiency is it associated with?
- lymphoid interstitial pneumonia (LIP)
- follicular bronchitis
- non-necrotizing granuloma formation
Common variable immune deficiency (CVID)
–also CTLA4 an LRBA deficiency
Type of pulmonary disease associated with ataxia-telangictasia
pulmonary fibrosis with lymphocytic/lymphohistiocytic inflammation
Which immunodeficiency type is most associated with ILD?
IgG subclass deficiency
Pulmonary Manifestation of systemic sclerosis
Percent of incidence in SSc
Autoantibody to look for
NSIP or UIP
50%
Scl-70
Pulmonary Manifestation of systemic sclerosis
Percent of incidence in SSc
Autoantibody to look for
NSIP or UIP
50%
Scl-70
Pulmonary manifestation of juvenile dermatomyositis & what autoantibody to look for
ILD (50% of adults)
neuromuscular weakness –> ineffective airway clearance and hypoventilation
Anti-Jo
Pulmonary manifestation of mixed connective tissue disease and what autoantibody to look for
Diffusion impairment, restrictive lung disease
Pleural effusions, NSIP, PF, PH
Anti-U1 RNP
Granulomatosis with polyangiitis pulm manifestation
subglottic stenosis
rhinitis
sinusitis
persistent otitis media with hearing loss
alveolar hemorrhage
pleuritis
pneumothorax
mediastinal lymph nodes
pleural effusion
nodular lung disease
Sarcoidosis pulm manifestation
noncaseating granulomas in lungs, lymph nodes, liver, skin, eyes
What is pulmonary hemosiderosis
recurrent intraalveolar bleeding leading to accumulation of iron in hemosiderin-laden macrophages
Iron-deficient anemia
hemoptysis
infiltrates on lung exam
pulmonary hemosiderosis
Common chemotherapeutic causes of pulmonary edema
cytarabine and bleomycin
Interstitial pneumonitis causing chemotherapeutic agent
methotrexate, bleomycin, procarbazine
Late complication of bleomycin and busulfan
pulm fibrosis
When does pulmonary fibrosis develop after radiation therapy
6-12 mo
early complication of radiation therapy (within 1-3 mo)
acute radiation pneumonitis (dyspnea, fever, cough, chest pain)
Pulmonary complication of hematopoietic stem cell transplant (HSCT) - PREENGRAFTMENT (0-30D)
primary pulm complication (infection (ANC<500), overload, hyperacute GVHD)
ENGRAFTMENT Syndrome (HSCT)
3-5% in autologous
7-10% in allogeneic
during neutrophil recovery, cytokine surge causing capillary leak
fever, hypoxemia, rash, noncardiogenic pulmonary edema, ARDS
Hyperacute and acute GVHD timing (HSCT)
2wk-3mo
Due to HLA mismatch
sx: skin rash, noncardiogenic pulmonary edema, pulmonary cytolytic thrombi may form and cause fever, cough and respiratory distress
Chest CT: subpleural nodules and opacities
Diffuse alveolar hemorrhage
POSTENGRATMENT period (ANC>500 early and late) HSCT
- Elevated infection risk (defective cellular and humoral immunity)
- Idiopathic pneumonia syndrome
- Endothelial dysfunction: transplant associated thrombotic microangiopathy (TMA) or pulmonary venoocclusive disease (PVOD)
How is TMA diagnosed? (thrombotic microangiopathy)
- thrombocytopenia
- microangiopathic hemolytic anemia (low hb, neg coombs, schistocytes on smear, increased LDH, decreased serum haptoglobin)
- increased creatinine
When does diffuse alveolar hemorrhage happen post HSCT
1-4 weeks
Late complications (30d or more)
autoimmune disease with pulm manifestation (autoantibody positive)
COP (1st 100 days)
CMV pneumonitis
Bronchiolitis obliterans
When does post transplant lymphoproliferative disorder occurs
When B-cells are infected with EBV in T-cell deficient seronegative transplant recipients
