Congenital, Developmental, and Vascular diseases Flashcards
Hyaline membranes are characteristic of which condition?
Acute Lung Injury/ARDS (ALI/ARDS)
Which type of epithelium is seen in bronchogenic cysts, often with squamous metaplasia?
Pseudostratified columnar ciliated epithelium
Hemosiderin-laden macrophages are found in which conditions?
Pulmonary edema (Hemodynamic) and Idiopathic pulmonary hemosiderosis
A mutation in the BMPR2 gene is associated with which condition?
Pulmonary hypertension
Red infarcts are commonly seen in which condition?
Pulmonary embolism
Congenital diaphragmatic hernia often results in which lung condition?
Pulmonary hypoplasia
Anti-glomerular basement membrane antibodies are seen in which condition?
Goodpasture syndrome
Obstruction of an airway can lead to which type of atelectasis?
Resorption atelectasis
Increased hydrostatic pressure in the lungs causes which condition?
Pulmonary edema
Neutrophil activation is a key mechanism in which condition?
Acute Lung Injury/ARDS
Waxy hyaline membranes are a hallmark histological feature of which condition?
Acute Lung Injury/ARDS
Squamous metaplasia can be found in which congenital lung abnormality?
Bronchogenic cyst
Type II pneumocytes proliferate during the repair phase of which lung injury?
Acute Lung Injury/ARDS and Atelectasis
Fibrosis is a key feature in which conditions?
Contraction Atelectasis, Pulmonary Edema, ARDS
Necrotizing granulomas are associated with which conditions?
Goodpasture syndrome and Polyangiitis with granulomatosis
Hemoptysis is a symptom of which three conditions?
Pulmonary Embolism, Goodpasture Syndrome, Idiopathic Pulmonary Hemosiderosis
Virchow’s Triad describes risk factors for which condition?
Pulmonary embolism
Chronic cor pulmonale results from which two lung-related conditions?
Pulmonary Embolism and Pulmonary Hypertension
Atheromas in the pulmonary arteries are found in which condition?
Pulmonary hypertension
Granulomatous disease may lead to which complication in the lungs?
Contraction Atelectasis
What distinguishes Intralobar from Extralobar Pulmonary Sequestrations?
Intralobar sequestrations are located within the lung parenchyma and usually present in older children. Extralobar sequestrations are outside the lung, often in the thorax or mediastinum, and commonly occur in infants.
How does Oligohydramnios contribute to Pulmonary Hypoplasia?
Oligohydramnios reduces amniotic fluid, decreasing pressure on the developing lungs, which disrupts normal growth and leads to underdeveloped lungs.
What are the key histological features of Hemodynamic Pulmonary Edema?
Engorged alveolar capillaries, pink edema fluid in alveoli, and hemosiderin-laden macrophages (“heart failure cells”) are characteristic.
What is the primary composition of hyaline membranes in Diffuse Alveolar Damage (DAD)?
Hyaline membranes consist of fibrin-rich fluid and necrotic epithelial cell debris lining the alveoli.
What role do neutrophils play in ARDS?
Neutrophils release proteases and oxidants, causing alveolar damage, inflammation, and hyaline membrane formation.
How does Pulmonary Infarction appear macroscopically, and why?
It appears as a hemorrhagic, wedge-shaped area, often in the lower lobes, due to dual blood supply from the bronchial arteries.
What clinical sign often indicates Pulmonary Infarction?
Hemoptysis (coughing blood) occurs as blood leaks into the alveolar spaces.
How does BMPR2 mutation contribute to Pulmonary Hypertension?
BMPR2 mutations increase smooth muscle cell survival and proliferation, leading to thickened arterial walls, higher resistance, and pulmonary hypertension.
What are the key clinical features of Goodpasture Syndrome?
Goodpasture Syndrome involves hemoptysis from lung hemorrhage and rapidly worsening kidney failure due to crescentic glomerulonephritis.
How do Idiopathic Pulmonary Hemosiderosis and Goodpasture Syndrome differ?
Both cause alveolar hemorrhage, but Goodpasture Syndrome has anti-basement membrane antibodies, while Idiopathic Pulmonary Hemosiderosis has no known cause or antibodies.
What best describes pulmonary hypoplasia?
Underdevelopment of the lungs
What is the most common type of foregut cyst found in the lungs?
Bronchogenic cyst
Which type of pulmonary sequestration is located within the lung parenchyma?
Intralobar
Resorption atelectasis is most commonly caused by:
Airway obstruction
Which of the following is a characteristic histological finding in hemodynamic pulmonary edema?
Hemosiderin-laden macrophages
Acute Respiratory Distress Syndrome (ARDS) is most often associated with which condition?
Sepsis
The hallmark histological feature of ARDS is:
Diffuse alveolar damage (DAD)
Which of the following describes the appearance of the lungs in the acute stage of ARDS?
Heavy
What type of cells proliferate during the organizing stage of ARDS?
Type II pneumocytes
Which transcription factor plays a key role in the inflammatory cascade associated with ARDS?
NF-κB
Which cell type plays a major role in the pathogenesis of ARDS by releasing inflammatory mediators and causing alveolar damage?
Neutrophils
The most common source of pulmonary emboli is:
Thrombi in the deep leg veins
What percentage of pulmonary emboli lead to pulmonary infarction?
0.1
Pulmonary infarcts are typically:
Hemorrhagic and wedge-shaped
Which of the following is a clinical sign associated with pulmonary infarction?
Hemoptysis
Multiple small pulmonary emboli can lead to which long-term complication?
Pulmonary hypertension
What is the defining characteristic of pulmonary hypertension?
Mean pulmonary arterial pressure ≥ 25 mmHg at rest
Mutations in which gene are commonly associated with familial pulmonary hypertension?
BMPR2
What is a key morphological feature of pulmonary hypertension?
Medial hypertrophy of pulmonary arteries
Goodpasture syndrome is an autoimmune disease characterized by antibodies targeting:
Type IV collagen
Which organ systems are primarily affected in Goodpasture syndrome?
Lungs and kidneys
A characteristic finding on immunofluorescence microscopy in Goodpasture syndrome is:
Linear deposits of immunoglobulins
What is the most common presenting symptom of Goodpasture syndrome?
Hemoptysis
Idiopathic pulmonary hemosiderosis is characterized by:
Intermittent
Which of the following is NOT a feature of polyangiitis with granulomatosis (formerly Wegener’s granulomatosis)?
Linear deposits of IgG along the glomerular basement membrane
Bronchogenic cysts are lined by what type of epithelium?
Pseudostratified columnar ciliated with squamous metaplasia
Which of the following is a potential consequence of a congenital diaphragmatic hernia?
Pulmonary hypoplasia
What is the difference between ALI and ARDS?
ALI is a milder form of lung injury
Pulmonary hypertension can result from which of the following?
All of the above
Which stain is commonly used to identify hemosiderin-laden macrophages in lung tissue?
Prussian blue
