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Pediatrics > Pulmonology > Flashcards

Pulmonology Flashcards

1
Q

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?

A

Primary Ciliary Dyskinesia
Hypomotile sperm

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2
Q

Mode of inheritance PCD

A

AR

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3
Q

Situs inversus
Recurrent respiratory infections
Infertility

A

Primary ciliary dyskinesia

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4
Q

Testing for primary ciliary dyskinesia

A
  1. FeNO (would be low)
  2. Sweat chloride (to rule out CF)
  3. genetic (though lots of variants, 70% detectible)
  4. Now no longer the best test – Immunofluorescence using antibodies to dynein arm proteins can detect abnormal structural proteins in the cilia.
  5. high speed video fluorescence
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5
Q

Pleural effusion characteristics of congenital chylothorax

A

exudative
high lymphocytes
low pH
bilateral
decreased lymphatic drainage
milky

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6
Q

The most common cause of pleural effusion in children is __

A

community-acquired pneumonia (CAP)

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7
Q

Tx for croup if there is stridor or barky cough at rest

A

dexamethasone

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8
Q

Diagnostic criteria for persistent bacterial bronchitis

A

If no other signs/sx/hx of other diseases AND >6yo AND
1. Lasted >4 weeks
2. No abnormal findings on PFT

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9
Q

chronic brassy, barking cough that disappears with distraction and when the child is asleep

A

Habit cough

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10
Q

chronic wet cough associated with recurrent otitis and/or sinusitis suggests __

A

immunodeficiency or ciliary dyskinesia

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11
Q

dry cough that is exacerbated by exercise and associated with frequent night awakenings is often caused by __

A

asthma or airway reactivity

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12
Q

premature baby with h/o intubation, now discharged with stertor, poor weight gain, coarse wheezing that doesn’t respond to bronchodilators probably has ___

A

tracheomalacia

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13
Q

when should laryngomalacia improve in infants?

A

18-24mo

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14
Q

which can resolve on it’s own? laryngomalacia or tracheomalacia?

A

Laryngomalacia

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15
Q

When can you reassure parents vs. refer to ENT for stridor?

A

Child is feeding well
Good growth
There is intermittent resolution of stridor with position
Lack of respiratory compromise

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16
Q

when to consider bacterial tracheitis

A

had viral infection for some days with acute worsening, high fever, BARKING COUGH, wheeze, RETRACTIONS

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17
Q

most common cause of bacterial tracheistis

A

staph aureus
then pseudomonas, moraxella, strep pyo, GBS

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18
Q

Onset of hemangiomas

A

8-12 weeks (when growth of vascular malformation is at its peak)

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19
Q

Imaging of choice for evaluation of central vascular anomalies.

A

MRI

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20
Q

Congenital pulmonary airway malformation can present as __

A

cystic lesions
persistent cough not better with meds
70% diminish/resolve before birth if detected in fetal US

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21
Q

If child has hypoxia with infectious insults, think __

A

pulmonary HTN

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22
Q

most common cause of hemoptysis in children

A

infection and bronchiectasis

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23
Q

repeated episodes of subclinical pulmonary hemorrhage that may present with cough and pink frothy sputum, multifocal infiltrates, w/o evidence of autoimmune disease

Dx?

A

Pulmonary Hemosiderosis

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24
Q

First thing you look for in Pulmonary hemosiderosis?

A

CBC – usually has anemia

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25
Q

Antitrypsin deficiency in children most often manifests as ___

A

hepatic disease

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26
Q

What can pulm hemosiderosis be associated with and what antibodies are present?

A

milk allergy – kids (IgG4)

mold allergy – infants

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27
Q

sequelae of congenital diaphragmatic hernia after repair

A

pHTN, recurrent hiatal hernia, GERD, growth failure

if pHTN resolves, then cor pulmonale not likely

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28
Q

recurrent sinopulmonary infections and a history of unexplained neonatal respiratory distress, sinus invertus

A

Primary ciliary dyskinesia

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29
Q

Normal range of sweat chloride

A

Normal <30
Intermediate 30-59
Elevated >60

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30
Q

Bronchiectasis in CF occurs first in ___ lobes, vs. in PCD, they are in __ lobes

A

upper – CF

mid lung fields – PCD

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31
Q

Definition of apnea of prematurity

A

cessation of air flow for >20s OR <20s but with bradycardia +/- cyanosis

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32
Q

when does apnea of prematurity stop

A

36-40 wk GA

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33
Q

when to stop using apnea monitor if doing well

A

until 43 weeks GA

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34
Q

tx for aop

A

caffeine (methylxantine)

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35
Q

Definition of bronchopulmonary dysplasia

A

need for supplemental O2 for at least 28 days after birth and at 36wk corrected GA

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36
Q

Pathophys of neonatal respiratory distress syndrome

A

immaturity of lung = decreased lung compliant, need surfactant

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37
Q

Risk of RDS is increased in __

A

diabetic mothers

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38
Q

Complications of RDS

A

air leaks & pulmonary hemorrhage

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39
Q

When to give corticosteroids to mothers (GA)

A

Preterm delivery before 34 weeks

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40
Q

What is pulmonary interstitial emphysema (PIE)

A

Rupture of small airways resulting in gas in interstitium

Usually in neonates with RDS with mechanical ventilation

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41
Q

Which side to place neonate on if there is PIE

A

affected side down

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42
Q

Bacteria implicated in EARLY ONSET neonatal pna from transplacental organism (4 bacteria, 4 virus)

A

GBS (most common)
Listeria
MTB
Treponema pallidum
Rubella
CMV
HSV
Adenovirus

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43
Q

Late onset neonatal PNA causes

A

ConS, S. aureus, gram negs (if vented)

atypicals: chlamydia trachomatis

viral

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44
Q

Term neonate born with asymmetric patchy infiltrate, tachypnea retractions within the first 12h of life

A

MEC aspiration syndrome

Can cause air leaks

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45
Q

treatment for PPHN

A

iNO
phosphodiasterase inhibitor (sildenafil, milrinone)
Endothelin receptor antagonist (bosentan)

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46
Q

Type B surfactant protein deficiency

What is it and how is it inherited

A

Full term, presents within few hours of life with rapidly progressive respiratory failure and hypoxemia

AR

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47
Q

Type C Surfactant protein deficiency

What is it and how is it inherited

A

Variable

AD vs sporadic

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48
Q

How do you diagnose and treat type B surfactant protein deficiency

A

Biopsy

Lung Tx

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49
Q

What syndromes are Tracheoesophageal fistulas associated with

A

Trisomy 18
Trisomy 21
DiGeorge
VATER
VACTERL
CHARGE

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50
Q

Fetal presentation of TEF

A

polyhydramnios and small stomach

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51
Q

Dx of TEF

A

place NGT and take xray

H-type dx with barium study with contrast material infused through nasogastric tube

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52
Q

Transient tachypnea of newborn risk factors

A

C-section
Maternal DM
Maternal Asthma
Male sex
Low birth weight
Macrosomia

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53
Q

CPAM (congenital pulmonary airway malformation) pathophys/anatomical issue

A

multicystic mass of pulmonary tissue

vasculature is from pulmonary circulation (rather than bronchial)

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54
Q

Most CPAM presents by age __

A

6 mo if symptomatic

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55
Q

What is congenital lobar emphysema?

A

hyperexpanded lobe with
1. normal amount of alveoli
OR
2. increased number of alveoli

Air trapping occurs

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56
Q

Bronchopulmonary sequestration pathphys/issue

A

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

57
Q

Where is bronchopulmonary sequestration usually found

A

left sided, between lowerlobe and diaphragm

58
Q

CPAM and BPS has increased risk of ___

A

infection

59
Q

Issue caused by CPAM and BPS

A

mass effect,

BPS: high output cardiac failure (overcirculation)

60
Q

Bronchogenic cyst - how does it form

A

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

61
Q

Problems from bronchogenic cysts

A

mass effect –> stridor or feeding difficulties

62
Q

How to diagnose bronchogenic cyst

A

barium swallow and CT scan

63
Q

Syndromes associated with Congenital diaphragmatic Hernia

A

trisomy 21, 18, 13
Beckwith-Wiedemann sd
Goldenhar

64
Q

What does Potter syndrome cause

A

Etiology: renal agenesis
–> severe pulmonary hypoplasia due to oligohydramnios

65
Q

What is pulmonary lymphangiectasia

A

dilation of ppulmonary lymphatic vessels and disordered drainage –> accumulation of lymph within the lungs

high mortality rate

66
Q

How to diagnose pulm lymphangiectasia

A

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

67
Q

Laryngomalacia starts and peaks at ___ months

A

2-4 mo of life

68
Q

When does mild laryngomalacia resolve

A

12 - 18 mo

69
Q

Nerve innervating vocal cords

A

recurrent laryngeal nerve

70
Q

Bilateral vocal cord paralysis usually caused by ___

A

central abnormality
1. Arnold-Chiari malformation
2. hydrocephalus

and more…

71
Q

Acquired VCP resolves by __ mo

A

6-12 mo

if no resolution by 2yo, won’t resolve by itself

72
Q

Neonatal biphasic stridor

A

fixed obstruction (subglottic stenosis that is severe)

73
Q

Tx for tracheomalacia

A

bethanechol (cholinergic agent that can increase smooth muscle tone in trachea

Ipatropium to increase tracheal smooth muscle

B2-agonist worsens

Manage GERD

74
Q

Usual age for croup

A

6-36mo

75
Q

When to add racemic epi for croup, and when to consider heliox

A

Racemic epi: audible stridor at rest, retractions at rest

Heliox: severe retractions and significant agitation, pronounced stridor, ill appearing, not taking PO

76
Q

recurrent barking cough without viral prodrome

A

spasmodic croup

77
Q

Management of spasmodic croup

A

look for allergic cause
Eval for GERD
AP/Lat neck radiographs

ENT if didn’t find anything

78
Q

Most common cause of epiglotitis

A

h. influenzae, H. parainfluenzae, S. pneumonia, S. aureus

79
Q

Diff between croup and epiglottitis

A

epiglotittis: toxic, hoarse voice
croup: can appear well, cough

80
Q

Tx of epiglotitis

A

ceftriaxone

81
Q

Epiglotittis age vs. Croup vs. bacterial tracheitis

A

Croup young 6-36mo

Epiglotitis kindgergarted 2-7yo

Bacterial tracheitis wide range 6mo to 8year (usually older)

82
Q

Barking cough/stridor that doesn’t resolve with racepic epi or steroids

A

Bacterial tracheitis

83
Q

In children with suspected OSA and T&A is performed, when do you get a repeat polysomnography?

A

6-8 weeks

84
Q

Age of bronchiolitis

A

<2yo

85
Q

When to give pavilizumab and to whom

A

Infants born <29 wk GA, every month during RSV season until 12mo.

Also infants born after 29wk GA with CHD or CLD

86
Q

Age for high risk of foreign body aspiration

A

3yo
Children with developmental delay

87
Q

Children with bronchiectasis are susceptible to recurrent infections from ___ (3 bugs)

A

h. influenza
strep
moraxella

88
Q

infant 3wk - 3 mo old with staccato cough. Dx?

A

chlamydia pneumonia

89
Q

Pneumonia in mississippi, misouri or Ohio River valley. Cause?

A

Histoplasma

90
Q

Pneumonia in Southeast US and Great Lakes?

A

Blasto

91
Q

someone owns birds and now has pneumonia, cause?

A

chlamydophila psittaci

92
Q

someone has a farm and now has pneumonia, cause?

A

coxiella burnetti

93
Q

pleural effusion with elevated amylase, ddx (3)

A

malignancy, pancreatitis, esophageal rupture

94
Q

Definition of empyema

A

pH <7.3
Glucose <60
exudative
LDH elevated
+/- gram stain culture

95
Q

ptx management (non-invasive)

A

max NRB for washout of normal air with high O2, to increase resorption

96
Q

CFTR protein malfunction causes ___ to the electrolytes

A
  1. Decreased Chloride secretion
  2. Increased sodium absorption from airway lumen
97
Q

most common CFTR mutation

A

deltaF508

98
Q

Role of PMNs in CF

A

Neutrophil nets create oxidants, elastase, and other proteases, leading to destruction of the bronchial walls

99
Q

Lumacaftor/ivacaftor (what is it and what does it treat?)

A

CFTR modulator
Improves misfolding and gating abnormality in CF
DeltaF508 mutation (severe phenotype)

100
Q

Ivacaftor only (what does it do and what does it treat?)

A

CFTR modulator
Treats CF with G551D mutation
Improves gating abnormality

101
Q

CF and pancreatic disease, which is defective first? exocrine or endocrine?

A

Exocrine

102
Q

Reproductive dysfunction in CF is caused by what pathophysiology?

In men:
In women:

A

Men: congenital bilateral absence of vas deferens CBAVD (no spermatozoa present in semen – obstructive azoospermia) 95%

Women: Viscous cervical mucus, advanced lung disease, malnutrition

103
Q

80-90% of infants with meconium ileus has ___

A

CF

104
Q

Distal intestinal obstruction syndrome is associated with ___

A

CF (older kids)
“sausage-like mass in RUQ”
Equivalent to meconium ileus

105
Q

What do you collect sweat with for sweat chloride test

A

pilocarpine inontophoresis

106
Q

Next steps after intermediate sweat chloride result

A

repeat test and genetic analysis

107
Q

Diagnosis of CF

A
  1. Clinical features or CF in sibling or +newborn screen
    AND
  2. elevated sweat chloride or
  3. Nasal potential difference (more negative charge due to hyperabsorption of Na) or
  4. identification of two disease-causing CF mutations
108
Q

Different types of newborn screening for CF (3)

A
  1. Immunoreactive trypsinogen assay (IRT): pancreatic dysfunction if elevated (80% sensitive)
  2. DNA assay: primary or secondary screen (some only tests delta F508, others test 28-40). IRT + DNA has 96% sensitivity
  3. Sweat test: +IRT and/or positive genetic screen to confirm diagnosis
109
Q

At what age is inhaled DNAse recommended and for what disease

A

> 6yo
CF

110
Q

what’s fibrosing colonopathy

A

giving too much exogenous lipase can cause this side effect in CF patients

111
Q

Definition of restrictive lung disease on PFT

A

<80% pred TLC
<80% FEV1 and FVC
>80% FEV1/FVC ratio

112
Q

Definition of reestrictive lung disease on PFT

A

<80% pred TLC
<80% FEV1 and FVC
>80% FEV1/FVC ratio

113
Q

In pts with neuromuscular diseases, how frequent should they follow up with pulmonologists?

A

Ambulatory: annually
Wheelchair-bound: biannual
FVC <60% quarterly visit, annual polysomnogram

114
Q

Options for secretion and dysphagia management (4)

A
  1. anticholinergic medications that thicken secretions
  2. Botulinum toxin injection to salivary glands to decrease saliva production
  3. Enteral tube feeding
  4. OT
115
Q

Bronchiolitis Obliterans

A

concentric bronchiolar narrowing due to inflammation and fibrosis

associated with CVID

116
Q

Types of granulomatous lymphocytic ILD and what type of immunodeficiency is it associated with?

A
  1. lymphoid interstitial pneumonia (LIP)
  2. follicular bronchitis
  3. non-necrotizing granuloma formation

Common variable immune deficiency (CVID)
–also CTLA4 an LRBA deficiency

117
Q

Type of pulmonary disease associated with ataxia-telangictasia

A

pulmonary fibrosis with lymphocytic/lymphohistiocytic inflammation

118
Q

Which immunodeficiency type is most associated with ILD?

A

IgG subclass deficiency

119
Q

Pulmonary Manifestation of systemic sclerosis
Percent of incidence in SSc
Autoantibody to look for

A

NSIP or UIP
50%
Scl-70

120
Q

Pulmonary Manifestation of systemic sclerosis
Percent of incidence in SSc
Autoantibody to look for

A

NSIP or UIP
50%
Scl-70

121
Q

Pulmonary manifestation of juvenile dermatomyositis & what autoantibody to look for

A

ILD (50% of adults)
neuromuscular weakness –> ineffective airway clearance and hypoventilation

Anti-Jo

122
Q

Pulmonary manifestation of mixed connective tissue disease and what autoantibody to look for

A

Diffusion impairment, restrictive lung disease
Pleural effusions, NSIP, PF, PH

Anti-U1 RNP

123
Q

Granulomatosis with polyangiitis pulm manifestation

A

subglottic stenosis
rhinitis
sinusitis
persistent otitis media with hearing loss
alveolar hemorrhage
pleuritis
pneumothorax
mediastinal lymph nodes
pleural effusion
nodular lung disease

124
Q

Sarcoidosis pulm manifestation

A

noncaseating granulomas in lungs, lymph nodes, liver, skin, eyes

125
Q

What is pulmonary hemosiderosis

A

recurrent intraalveolar bleeding leading to accumulation of iron in hemosiderin-laden macrophages

126
Q

Iron-deficient anemia
hemoptysis
infiltrates on lung exam

A

pulmonary hemosiderosis

127
Q

Common chemotherapeutic causes of pulmonary edema

A

cytarabine and bleomycin

128
Q

Interstitial pneumonitis causing chemotherapeutic agent

A

methotrexate, bleomycin, procarbazine

129
Q

Late complication of bleomycin and busulfan

A

pulm fibrosis

130
Q

When does pulmonary fibrosis develop after radiation therapy

A

6-12 mo

131
Q

early complication of radiation therapy (within 1-3 mo)

A

acute radiation pneumonitis (dyspnea, fever, cough, chest pain)

132
Q

Pulmonary complication of hematopoietic stem cell transplant (HSCT) - PREENGRAFTMENT (0-30D)

A

primary pulm complication (infection (ANC<500), overload, hyperacute GVHD)

133
Q

ENGRAFTMENT Syndrome (HSCT)

A

3-5% in autologous
7-10% in allogeneic

during neutrophil recovery, cytokine surge causing capillary leak
fever, hypoxemia, rash, noncardiogenic pulmonary edema, ARDS

134
Q

Hyperacute and acute GVHD timing (HSCT)

A

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

135
Q

POSTENGRATMENT period (ANC>500 early and late) HSCT

A
  1. Elevated infection risk (defective cellular and humoral immunity)
  2. Idiopathic pneumonia syndrome
  3. Endothelial dysfunction: transplant associated thrombotic microangiopathy (TMA) or pulmonary venoocclusive disease (PVOD)
136
Q

How is TMA diagnosed? (thrombotic microangiopathy)

A
  1. thrombocytopenia
  2. microangiopathic hemolytic anemia (low hb, neg coombs, schistocytes on smear, increased LDH, decreased serum haptoglobin)
  3. increased creatinine
137
Q

When does diffuse alveolar hemorrhage happen post HSCT

A

1-4 weeks

138
Q

Late complications (30d or more)

A

autoimmune disease with pulm manifestation (autoantibody positive)

COP (1st 100 days)

CMV pneumonitis

Bronchiolitis obliterans

139
Q

When does post transplant lymphoproliferative disorder occurs

A

When B-cells are infected with EBV in T-cell deficient seronegative transplant recipients

Pediatrics (26 decks)

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