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Respiratory II - Exam 1 > 9-29 DSA - Pediatric Respiratory Pathology > Flashcards

9-29 DSA - Pediatric Respiratory Pathology Flashcards

1
Q

What are 2 intrauterine conditions that can lead to pulmonary hypoplasia?

A

low levels of amniotic fluid

restricted movement of the fetal chest wall

(basically anything that compress the lung or impede normal expansion in utero

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2
Q

What are the 3 types of tracheoesophageal fistula?

A
  1. blind ending pouch, esophagotracheal fistula for distal esophagus
  2. blind ending pouch, fibrous cord for distal esophagus
  3. communication of esophagus with trachea
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3
Q

What type of tracheoesophageal fistula is the most common?

A

blind-ending esophageal pouch with esophagotracheal fistula in distal part of esophagus

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4
Q

What type of tracheosophageal fistula causes the most respiratory complications?

A

communication of esophagus with trachea, aspiration pneumonia has a very high chance of developing

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5
Q

What is the embryological structure that congenital foregut cysts develop from?

A

the foregut

(if you get this wrong, go home. you’re probably drunk.)

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6
Q

How do congenital foregut cysts typically present?

A

mass or incidental finding, sometimes in adults

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7
Q

Where are congenital foregut cysts in the fully-developed person?

A

mediastinum or hilum

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8
Q

What is the general size and contents of congenital foregut cysts?

A

cyst spaces up to ~5 cm

contains bronchogenic tissue with respiratory epithelium

  • filled with mucin, and one or more of:

cartilage

smooth mm

esophageal tissue - squamous mucosa

enteric tissue - intestinal mucosa

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9
Q

When are congenital foregut cysts problematic?

A

Considering hilar/mediastinal location, problems happen when they compress on other structures

  • i.e. venal caval obstruction, jugular vein distension

Usually benign structures, rarely malignant

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10
Q

What is the histological description for a Congenital Cystic Adenomatoid Malformation?

A

hamartomatous lesion with abnormal bronchiolar tissue

(not a pulmonary hamartoma, which is made of proliferating lung tissue)

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11
Q

Which type of congenital cystic adenomatoid malformation has the worst outcomes? Why?

A
  1. Type I - large cysts, good prognosis
  2. Type II - medium cysts, poorer prognosis since associated with other congenital malformations
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12
Q

What is a bronchopulmonary sequestration?

A

area of lungs without normal connection to airways, with blood supply from systemic arteries

  • no gas exchange due to lack of pulmonary circulation
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13
Q

What are the 2 types of bronchopulmonary sequestrations? Where are they located?

A

Extralobar: external to lung

(thorax or mediastinum)

Intralobar: within lung

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14
Q

What is associated with each type of bronchopulmonary sequestration?

A

Extralobar - may have other congenital anomalies

Intralobar - associated with recurrent local infection and/or bronchiectasis,

also most likely an acquired lesion

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15
Q

Extralobar bronchopulmonary sequestration is a congenital anomaly, and intralobar is an an acquired lesion. Why are the 2 types grouped together?

A

common findings - no viable blood connection to pulmonary circulation, only from systemic circulation

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16
Q

What is the most common cause of respiratory distress in the newborn?

A
  • Hyaline membrane disease
  • most common (24,000/year)
  • 1,000 deaths/year in 2002
  • 25,000 deaths/year in 1960’s
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17
Q

In addition to hyaline membrane disease, what are some other common causes of respiratory distress in neonates?

A
  • Excessive maternal sedation
  • Fetal head injury
  • Blood or amniotic fluid aspiration
  • Intrauterine hypoxia from nuchal cord
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18
Q

What is a nuchal cord? Why is it dangerous?

A

nuchal cord is when umbilical cord wraps around the neck of the fetus in utero

  • true nuchal cord will leave indentations on the neck and trunk, and compress the neck enough to compress the large vessels of the neck, causing intrauterine hypoxia
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19
Q

What are the risk factors for neonatal respiratory distress syndrome/hyaline memb disease?

What are four other causes/associations?

A

preterm/age and normal weight

  • Can occur in term infants, but most are preterm and have adequate growth for gestational age
  • Rate inversely proportional to gestational age
  • 60% < 28 wks, 30% 28-34 wks, < 5% >34 wks

Other causes:

  1. • Associated with male sex,
  2. maternal diabetes mellitus,
  3. multiple gestation
  4. C- section before onset of labor
20
Q

Why is DM a risk factor for neonatal resp distress syndrome?

A

Mom has high levels of glucose, which crosses placenta and enters fetal circulation

fetus secretes lots of insulin

high levels of insulin inhibit surfactant secretion

21
Q

What induces surfactant secretion?

A

glucocorticoids/stress (why c-sections are risk factor, less stress in baby in birthing process)

thyroxine

22
Q

What physiological factors are associated with a higher risk for hyaline membrane disease?

A
  • Immaturity of lungs!!
  • Deficiency of pulmonary surfactant
  • The first breath of life requires a large inspiratory effort but once inflated the lungs remain ~ 40% inflated
  • If inadequate surfactant, lungs collapse back and every breath is as hard as the first
23
Q

What are the important stages of lung development? What are the weeks when they happen?

A

20 weeks - glandular

30 weeks - saccular

term - alveolar

24
Q

Describe the histological findings at each stage of lung development.

A

glandular - bronchioles and distal airspaces lined with cuboidal epithelium

frequent capillaries in loose CT in interstitium

Saccular - bronchioles still lined with cuboidal epithelium

alveolar ducts present

new alveoli lined with type I pneumocytes

rare type II pneumocytes

term - close to full developed, but thicker interstitium and increased diffusion barrier is present

25
Q

What secretes surfactant?

A

Type II pneumocytes

26
Q

When is there ‘mature’ levels of surfactant?

A

35 weeks

L/S ratio > 2 Lungs mature (except with some maternal diabetes)

< 1 Lungs are immature

27
Q

How is L/S ratio measured? 4 methods

What is L/S?

A

Methods:

  1. Thin layer chromatography
  2. Flourescence polarization
  3. Foam stability index
  4. Lamellar body count
  • Lecitithin/sphingomyelin ratio
28
Q

What does surfactant consist of?

A

Consists of

Phospholipids

  • Primarily dipalmitoyl phosphatidylcholine (lecithin)
  • Also phosphatidylglycerol

Glycoproteins

  • Hydrophylic SP-A & SP-D (immunity)
  • Hydrophobic SP-B & SP-C (surface tension)
29
Q

What happens with decreased pulmonary surfactant that leads to pulm edema in newborns?

A

increased alveolar surface tension

atelectasis

uneven perfusion& hypoventilation

hypoxemia & CO2 retention

acidosis

pulm vasoconstriction

pulm hypoperfusion

endothelial & epithelial damage

plasma leakage into alveoli

fibrin and necrotic cells (hyaline memb)

increased diffusion gradient

more hypoxemia and CO2 retention

30
Q

What is the clinical presentation for respiratory distress syndrome in neonates?

A
  • Preterm and AGA
  • Male sex, maternal DM, C-section
  • Low 1 minute Apgar score
  • May need resuscitation
  • Then may do well for short time (< 1 hour)
  • Become cyanotic
  • Fine pulmonary rales (crackles)
  • Reticulonodular/ground glass chest x-ray
  • Oxygen therapy needed
  • Death or recovery in 3 – 4 days
31
Q

Why does respiratory effort quickly cease in infants born without surfactant?

A

Lack of pulmonary surfactant leads to increased atelectasis, with increased work of breathing and inability to form residual capacity in lungs

MM of respiration eventually give out due to exhaustion

32
Q

How is neonatal respiratory distress syndrome treated?

A
  • Outlook much more favorable today
  • Administration of surfactant (<28 weeks)
  • Antenatal treatment with steroids (24-34 weeks)
  • Monitor amniotic fluid surfactant for lung maturity
  • Death now unusual
  • Recovery begins at about 4 days
33
Q

What are the risks associated with O2 therapy for neonatal respiratory distress syndrome?

A
  • Retinopathy of prematurity
  • Bronchopulmonary dysplasia
34
Q

When is the bronchopulmonary dysplasia complication more common?

A
  • > 28 days of O2 therapy in infant > 36 weeks post-menstrual age
  • Now infrequent in infants >1200g and 30 weeks
35
Q

Why have rates of bronchopulmonary dysplasia gone down?

A

•Gentler ventilation, steroids, and surfactant therapy reduced rates

Mild, moderate, or severe based on need for positive pressure O2 therapy

36
Q

Why is bronchopulmonary dysplasia throught to happen?

A
  • Alveolar hypoplasia & thickened walls
  • O2 thought to inhibit lung maturation
  • Dysmorphic capillaries and decreased VEGF
  • Cytokines (TNF, Il-8, etc) increased and may have a role
37
Q

What is cystic fibrosis?

A
  • Widespread disorder in epithelial transport affecting fluid secretion in exocrine glands and the epithelial lining of the respiratory, gastrointestinal, and reproductive tracts
  • Abnormally viscid mucus secretions
38
Q

What are the genetic factors involved in CF?

A
  • Autosomal recessive transmission
  • Primarily due to abnormal function of an epithelial chloride channel protein encoded by the cystic fibrosis transmembrane conductance regulator (CFTR) gene on chromosome band 7q31.2
  • Most common lethal genetic disease that affects Caucasians (1 in 2,500 live births in Caucasians)
  • Estimate 5% of Caucasians heterozygous carriers (can also be symptomatic)
39
Q

What is the diagnostic criteria for CF?

A

One or more characteristic phenotypic features

Or A history of cystic fibrosis in a sibling

Or A positive newborn screening test result (immunoreactive trypsinogen-pancreatic protein typically elevated in CF-affected infants)

And

An increased sweat chloride concentration on two or more occasions

Or Identification of two cystic fibrosis (CFTR) mutations

Or Demonstration of abnormal epithelial nasal ion transport (baseline nasal potential more negative than normal controls)

40
Q

What complications need to be avoided with CF?

A

pancreatic insufficiency

vitamin deficiency

pulmonary disease

41
Q

How is pancreatic insufficiency treated in CF patients?

A

•

  • Oral Pancrelipase (lipase, protease, and amylase)
  • May uncommonly need to treat diabetes mellitus if islets destroyed
42
Q

How is vitamin deficiency treated in CF patients?

A
  • Oral fat soluble vitamins (ADEK)
  • Parenteral nutrition
43
Q

How is pulmonary disease treated/avoided in CF patients?

looking for 9 here

A
  1. •Postural drainage and chest percussion
  2. •Bronchodilators (albuterol)
  3. •Mucolytic agents
  4. •Inhaled acetylcysteine to break down mucoproteins
  5. •Inhaled dornase alfa =Recombinant human DNase (rhDNase)
  6. •Antibiotics (need to cover Pseudomonus aeruginosa)
  7. •Hypertonic saline (inhaled)
  8. •High dose ibuprofen
    • slows lung disease progression
  9. •Lung or heart-lung transplants
44
Q

What is shown here?

How do you distinguish this in an adult vs. a kid?

A

Hyaline membrane disease

Distinguishable only by looking at the age of the patient apparently, per Gomez’s snark.

Wiggled Image: “Be able to recognize”

45
Q

At what stage is the lung tissue shown here?

A

Alveolar

(didn’t wiggle, but we needed to be able to distinguish last year)

46
Q

What stage of lung development?

A

Glandular

47
Q

What stage of lung development?

A

Saccular

Respiratory II - Exam 1 (11 decks)

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