Rubins - respiratory system

Question 1

What is pulmonary hypoplasia?

What are 3 causes?

Answer 1

incomplete or defective lung development (i.e. in utero) that results in fewer acini or smaler sized than normal

Compression of lung (gongential diaphramatic hernia)

Oligohydramnios (inadequate volume of amniotic fluid due to genitourinary anomalies)

Decreased respiration

Question 2

Gestational congenital diaphragmatic hernia is associated with what?

Answer 2

pulmonary hypoplasia

specifically hypoplasia of the bronchi and pulmonary vessels

Question 3

congential cystic adenomatoid malformation?

Age affected?
Presenting symptoms?
Pathologic features?
Structure affected?

Answer 3

abnormal bronchiolar structions of varying sizes
presents with respriatory distress and cyanosis in first 2 years of life

affects one lobe (usually)

“multiple cyst like spaces lined by bronchiolar epithelium separated by loose fibrous tissue”

Question 4

Bronchiogenic cyst

Age affected?
Presenting symptoms?
Pathologic features?
Structure affected?

Answer 4

newborns: present with respiratory distress from compressed lungs

Adults: secondary infection of cyst can lead to hemorrhage or poerforation

discrete, extrapulminary, fluid filled mass lined by respiratory epithelium and limited by walls that contain muscle and cartilage

most common in middle mediastinum

Can be asymptomatic

Question 5

Extralobular sequestration

Age affected?
Presenting symptoms?
Pathologic features?
Structure affected?

Answer 5

Neonates: respiratory distress with cyanosis

Older children: recurrent bronchopulmonary infections

mass of lung tissue that is not connected to the bronchial tree and located OUTSIDE the visceral pleura