Medicine Marrow Pg 181-190 (Pulmonology)

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Questions and Answers

What is the most common congenital bleeding disorder?

Hemophilia A

Factor V Leiden

Sickle Cell Disease

Von Willebrand Disease (correct)

Von Willebrand Factor is primarily degraded by ADAMTS 12.

False

Which chromosome carries the gene for Von Willebrand Factor?

Chromosome 12

Von Willebrand Factor is released from __________ in the endothelium.

Weibel-Palade bodies Signup and view all the answers

Match the following types of Von Willebrand Disease with their characteristics:

Type 1 = Most common; partial deficiency Type 2 = Qualitative defect Type 3 = Rare; complete deficiency Signup and view all the answers

Which of the following is NOT a part of the clinical presentation of DIC?

Increased platelet counts Signup and view all the answers

In patients with DIC, a common laboratory finding is an increased level of fibrinogen.

False Signup and view all the answers

What is the most specific laboratory test indicator for DIC?

D-dimer Signup and view all the answers

The severe form of DIC, characterized by microvascular thrombosis and necrosis, is known as __________.

purpura fulminans Signup and view all the answers

Match the following investigations with their expected findings in DIC:

Platelet count = Decreased APTT = Increased PT = Increased Fibrin degradation products = Increased Signup and view all the answers

Which of the following statements regarding the right lung is correct?

It contains the RUL, RML, and RLL. Signup and view all the answers

The lower lobe of the lung can be visualized on a normal X-ray.

False Signup and view all the answers

Name the areas for auscultation of the lower lobe.

Infrascapular and Infraxillary Signup and view all the answers

The __________ fissure is found only in the right lung.

Minor Signup and view all the answers

Match the following lung fissures with their descriptions:

Major fissure = Bilateral lungs Minor fissure = Right lung only Azygos fissure = Tadpole appearance with azygos vein Signup and view all the answers

What is the primary function of Clara cells in the lungs?

Detoxification of xenobiotics Signup and view all the answers

Kulchitzky cells contain dense core vesicles with neurotransmitters such as serotonin.

True Signup and view all the answers

What type of epithelium is found in the upper part of the bronchus?

Pseudostratified ciliated columnar epithelium Signup and view all the answers

The most potent constrictor of bronchial smooth muscles is __________.

Leukotriene Signup and view all the answers

Match the following substances with their effects on the conducting airways:

Leukotriene = Bronchoconstriction Vasoactive Intestinal Peptide (VIP) = Bronchodilation Cholinergics = Bronchoconstriction Anticholinergics = Bronchodilation Signup and view all the answers

What is the correct order of airflow through the respiratory structures?

Terminal bronchioles → Respiratory bronchioles → Alveolar ducts → Alveoli Signup and view all the answers

The diameter of the right bronchus is narrower than that of the left bronchus.

False Signup and view all the answers

What is the anatomical unit of the lung supplied by a tertiary bronchus called?

Bronchopulmonary Segment Signup and view all the answers

Inhaled foreign body impaction is more common in the ______ bronchus.

right Signup and view all the answers

Match the following bronchi types with their descriptions:

Primary Bronchi = Main bronchi leading to each lung Secondary Bronchi = Bronchi supplying lobes of the lung Tertiary Bronchi = Bronchi supplying bronchopulmonary segments Terminal Bronchioles = Smallest bronchi leading to alveoli Signup and view all the answers

What is the main defect in Immotile Cilia Syndrome?

Defective dynein arm Signup and view all the answers

Cystic Fibrosis is caused by a dominant gene mutation.

False Signup and view all the answers

What is the typical age of presentation for Immotile Cilia Syndrome?

5 years Signup and view all the answers

In Cystic Fibrosis, the gold standard investigation is measurement of ___ levels.

nasal nitric oxide Signup and view all the answers

Match the following conditions with their clinical features:

Immotile Cilia Syndrome = Recurrent sinusitis and otitis media Cystic Fibrosis = Thick secretions and low nasal nitric oxide Kartagener's syndrome = Situs inversus and male infertility Nasal nitric oxide investigation = Gold standard for Cystic Fibrosis Signup and view all the answers

What is the incidence percentage of Type 1 Von Willebrand Disease?

85% Signup and view all the answers

Type 3 Von Willebrand Disease is inherited in an autosomal dominant manner.

False Signup and view all the answers

What type of bleeding is associated with Type 1 Von Willebrand Disease?

Platelet bleed Signup and view all the answers

Type 3 Von Willebrand Disease presents with __________ bleed.

severe platelet and coagulation Signup and view all the answers

Match the type of Von Willebrand Disease to its characteristic:

Type 1 = Partial semiquantitative defect Type 3 = Complete quantitative defect Signup and view all the answers

Which segment of the left lung is responsible for the superior region?

Superior Signup and view all the answers

The right lung contains 9 segments.

False Signup and view all the answers

List the parts of the acinus.

Respiratory bronchiole, Alveolar duct, Alveolus Signup and view all the answers

What is a common cause of severe bleeding according to the clinical disorder details?

Hemophilia B Signup and view all the answers

In a supine position, aspiration is most likely to involve the posterior segment of the right ________ lobe.

upper Signup and view all the answers

Match the type of emphysema with its associated condition:

Centriacinar emphysema = Common in smokers Panacinar emphysema = Associated with alpha-1 antitrypsin deficiency Signup and view all the answers

Warfarin inhibits vitamin K epoxide reductase.

True Signup and view all the answers

Which cells in the conducting airways are responsible for mucus secretion?

Goblet cells Signup and view all the answers

What clinical disorder is indicated by a prolonged APTT and a normal PT?

No bleeding Signup and view all the answers

The right lung has a superior lingular segment.

False Signup and view all the answers

A patient with a hematological malignancy may present with __________ due to disseminated intravascular coagulation.

severe bleeding Signup and view all the answers

Which of the following conditions does NOT cause bleeding disorders?

Hypertension Signup and view all the answers

What determines the segments most frequently involved in aspiration?

Patient’s position Signup and view all the answers

What type of bleeding is indicated by a negative Ristocetin Agglutination Test?

Platelet type bleed Signup and view all the answers

Match the following clotting factors to their potential clinical conditions:

Factor VIII = Hemophilia A Factor IX = Hemophilia B Lupus anticoagulant = Antiphospholipid syndrome High molecular weight kininogen = No bleeding Signup and view all the answers

____________ is the most common cause of disseminated intravascular coagulation.

Sepsis Signup and view all the answers

Which of the following structures is NOT found at the hila of the lungs?

Trachea Signup and view all the answers

Hilar lymphadenopathy is less common than pulmonary artery dilatation.

False Signup and view all the answers

What is the primary site for gas exchange in the lungs?

Alveolar sacs Signup and view all the answers

The site of gas exchange in the lungs is known as the __________.

acinus Signup and view all the answers

Match the airway generations with their corresponding structures:

0 = Trachea 5 = Terminal bronchioles 16-17 = Respiratory bronchioles 23 = Site of gas exchange Signup and view all the answers

Study Notes

Bronchi

Three types of bronchi: primary, secondary, and tertiary
- Primary are the main bronchi, secondary are lobar bronchi, and tertiary are segmental bronchi
Right bronchus is wider, shorter, and at a steeper angle to the carina than the left bronchus
- This makes the right bronchus more susceptible to inhaled foreign body impaction
Airway diameter decreases from trachea (25 mm) to alveoli (0.25-0.3 mm), resulting in a significant increase in cross-sectional area (from 5 cm² to 700,000 cm²)

Secondary Pulmonary Lobule

The functional unit of the lung
It consists of 3-5 terminal bronchioles enclosed in connective tissue septa
Airflow follows this pathway: terminal bronchiole → respiratory bronchioles → alveolar ducts → alveoli

Bronchopulmonary Segments (BPS)

Anatomical unit of the lung supplied by a tertiary bronchus
Contains a pulmonary artery within it (intrasegmental artery)

Disseminated Intravascular Coagulation (DIC)

Pathogenesis of DIC:
- Stimulus → Endothelial activation → Release of tissue factor (Thromboplastin) → Continuous and excessive thrombin formation and activation → Consumption of platelets and clotting factors → Bleeding + hyperfibrinolysis (Trapping of fibrinogen)
Etiology of DIC:
- Sepsis (most common)
- Hematological malignancy
- Trauma
- Pancreatitis
- Burns
- Massive blood transfusion
- Kasabach-Merritt syndrome

Clinical Approach to Bleeding

Prolonged APTT and normal PT suggests no bleeding, possibly due to Factor XII deficiency, High molecular weight kininogen deficiency, or Prekallikrein deficiency
Prolonged APTT and normal PT with reduced platelet count suggests mild bleeding, possible due to Hemophilia A (Factor VIII deficiency)
Prolonged APTT and prolonged PT with reduced platelet count suggests severe bleeding, possibly due to Hemophilia B (Factor IX deficiency), or von Willebrand disease (VWF)

Clinical Anatomy of Lungs

Right lung has three lobes: upper, middle, and lower
Left lung has two lobes: upper and lower
The lower/inferior lobe is challenging to visualize on a normal X-ray due to its posterior position
- Lateral X-ray or CT scan is needed for visualization
Fissures are depressions on the lung surface extending to the hilum
- Major fissure/Oblique fissure is present in both lungs
- Minor fissure/Horizontal fissure only in the right lung
- Azygos fissure is rare, has a tadpole appearance, and contains the azygos vein

Phantom tumor/Pseudotumor

Fluid collection in the horizontal fissures
Etiology: Heart failure with low ejection fraction
Resolves with diuretics

Hematology

The most common congenital bleeding disorder is Von Willebrand disease
Von Willebrand factor (VWF) is a protein essential for platelet adhesion and factor VIII carriage
Types of Von Willebrand disease:
- Type 1 (most common): Partial quantitative defect
- Type 2: Qualitative defect
- Type 3 (rare): Complete quantitative defect

Lung Anatomy and Physiology

Structures at the hila:
- Pulmonary artery (PA)
- Pulmonary vein (PV)
- Bronchus
- Lymphatics
Causes of hilar enlargement:
- Hilar lymphadenopathy (TB, sarcoidosis)
- PA dilatation
- Mediastinal mass
- Mass in the bronchus

Weibel's Generation of Airways

Generation 0: Trachea
Generation 1: Bronchi
Generation 2: Bronchioles
Generation 5: Terminal bronchioles
Generation 16-17: Respiratory bronchioles
Generation 18-21: Alveolar ducts
Generation 22: Alveolar sacs
Generation 23: Site of gas exchange (acinus)

Club/Clara Cells

Non-ciliated cuboidal epithelial cells in terminal bronchioles
Function: Detoxification of xenobiotics and progenitor cells for bronchiolar cells

Kulchitzky Cells

Dense core vesicles with serotonin, calcitonin, and CGRP
CGRP is a major neurotransmitter in migraine

Epithelium of Conducting Airways

Trachea: + cartilage, - smooth muscles, - mucus producing cells
Bronchi: + cartilage, + smooth muscles, + mucus producing cells
Bronchiole: - cartilage, + smooth muscles, + mucus producing cells (max in terminal bronchiole)

Bronchus Epithelium

Upper Part: Pseudostratified ciliated columnar epithelium
Middle Part: Ciliated simple cuboidal epithelium
Lower Part: Simple cuboidal epithelium

Bronchiole Epithelium

Lining: Simple cuboidal epithelium
No mucus glands
Occasional goblet cells
Few neuroendocrine cells
Clara cells

Substances Acting on Conducting Airways

Leukotriene: Potent constrictor of bronchial smooth muscles
Vasoactive Intestinal Peptide (VIP): Potent dilator; non-adrenergic non-cholinergic (NANC) peptide

Cholinergics

Broncho-constrictor
Methacholine challenge test: To assess airway hyperresponsiveness

Anticholinergics

Bronchodilators
Used in asthma
Examples: Ipratropium, tiotropium

Role of Cilia

Defective mucociliary escalator mechanism can lead to:
- Immotile Cilia Syndrome/Primary Ciliary Dyskinesia
- Cystic Fibrosis

Immotile Cilia Syndrome/Primary Ciliary Dyskinesia

Autosomal recessive inheritance
Defective dynein arm
Median age of presentation: 5 years
Clinical Features:
- Recurrent episodes of sinusitis and otitis media
- Neonatal respiratory distress
- Kartagener's syndrome (triad + male infertility) - cilia required for rotation of body organs → results in situs inversus

Cystic Fibrosis

Autosomal recessive inheritance
Defect: CFTR gene mutation (F508 mutation) on chromosome 7q → Cl ion defect
Clinical Features:
- Thick secretions
- Normal cilia
- Affected sites: Bronchi, biliary tract, intestine, reproductive system
Gold standard investigation: Nasal nitric oxide levels (Always low)
Rx: Ivacaftor (Cl channel opener)

Pulmonology

The text describes the structures of the lungs, causes of hilar enlargement, and the functional anatomy of airways in the context of medical imaging.

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Description

Explore the anatomy and function of bronchi in the respiratory system. Learn about the primary, secondary, and tertiary bronchi, as well as the secondary pulmonary lobule and bronchopulmonary segments. This quiz covers key concepts essential for understanding lung anatomy and physiology.