Asthma Overview and Management

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Questions and Answers

What is the role of endothelin-1 in pulmonary hypertension?

  • It is a vasoconstrictor that is elevated in pulmonary hypertension. (correct)
  • It enhances nitric oxide production.
  • It is a vasodilator that decreases arterial pressure.
  • It has no significant impact on pulmonary vasculature.

Which class of medication is used to block endothelin-1 receptors?

  • Endothelin receptor antagonists like Bosentan (correct)
  • β-adrenergic agonists
  • Angiotensin-converting enzyme inhibitors
  • Calcium channel blockers

What is characteristic of limited scleroderma?

  • It includes widespread skin thickening and renal involvement.
  • It is also known as CREST syndrome. (correct)
  • It is marked by severe pulmonary fibrosis only.
  • It is synonymous with diffuse scleroderma.

Which symptom is NOT associated with sclerodactyly?

<p>Abnormal calcium deposition in tissues (C)</p> Signup and view all the answers

What condition is suggested by a significant increase in blood pressure due to renal involvement?

<p>Diffuse scleroderma (A)</p> Signup and view all the answers

What is a recommended strategy to reduce the recurrence of Raynaud's phenomenon?

<p>Dress warmly in cold weather. (B)</p> Signup and view all the answers

Which medication should be avoided in patients with Raynaud's phenomenon?

<p>α1 agonists (C)</p> Signup and view all the answers

Esophageal dysmotility related to scleroderma is often characterized by:

<p>Decreased esophageal peristalsis and reflux (C)</p> Signup and view all the answers

What is the primary defect in primary ciliary dyskinesia?

<p>Defective dynein arm function (B)</p> Signup and view all the answers

In a patient with situs inversus, which cardiac finding is most likely to be observed?

<p>Cardiac sounds loudest on the right (B)</p> Signup and view all the answers

What characterizes the granulomas found in sarcoidosis?

<p>Non-caseating granulomas (C)</p> Signup and view all the answers

What consequence might women with primary ciliary dyskinesia experience due to abnormal cilia in the fallopian tubes?

<p>Ectopic pregnancy (A)</p> Signup and view all the answers

What is the role of epithelioid macrophages in sarcoidosis?

<p>Secretion of 1a-hydroxylase (A)</p> Signup and view all the answers

In sarcoidosis, what happens to parathyroid hormone levels?

<p>They are suppressed (B)</p> Signup and view all the answers

Which gene mutation is primarily associated with primary pulmonary hypertension (PPH)?

<p>BMPR2 (C)</p> Signup and view all the answers

How does primary pulmonary hypertension differ from cor pulmonale?

<p>PPH originates intrinsically in the lungs (B)</p> Signup and view all the answers

Which demographic is typically associated with sarcoidosis?

<p>African-American women in their 20s-30s (C)</p> Signup and view all the answers

What imaging findings are typical for sarcoidosis?

<p>Bihilar lymphadenopathy (A)</p> Signup and view all the answers

Which of the following findings would indicate cor pulmonale rather than primary pulmonary hypertension?

<p>Right ventricular hypertrophy on ECG (A)</p> Signup and view all the answers

What is a likely finding in the semen of a patient with primary ciliary dyskinesia?

<p>Decreased sperm motility (A)</p> Signup and view all the answers

In the context of sarcoidosis, what does high levels of 1,25-(OH)2-D3 cause?

<p>Hypercalcemia (A)</p> Signup and view all the answers

Which symptom is NOT typically associated with sarcoidosis?

<p>Chest pain (D)</p> Signup and view all the answers

What symptom may indicate pulmonary hypertension in a patient in their 20s to 30s?

<p>Loud P2 or tricuspid regurgitation (C)</p> Signup and view all the answers

What should be chosen if a vignette suggests sarcoidosis but indicates a normal CXR?

<p>Asthma (B)</p> Signup and view all the answers

What is the primary clinical significance of clubbing in patients?

<p>It is commonly associated with bronchiectasis. (D)</p> Signup and view all the answers

What is the most common cause of fever within 24 hours post-surgery?

<p>Atelectasis (A)</p> Signup and view all the answers

What is the primary function of the sweat chloride test in cystic fibrosis diagnosis?

<p>To evaluate the chloride ion concentration in sweat. (B)</p> Signup and view all the answers

In cystic fibrosis, what genetic characteristic is common among siblings?

<p>There is a 2/3 chance a phenotypically normal sibling is a carrier. (A)</p> Signup and view all the answers

Which CFTR-related therapy aims at correcting channel localization and folding?

<p>Ivacaftor (B)</p> Signup and view all the answers

What does the transepithelial nasal voltage test assess?

<p>Nasal potential difference. (C)</p> Signup and view all the answers

Which of the following conditions can be associated with congenital bilateral absence of vas deferens (CBAVD)?

<p>Cystic fibrosis (C)</p> Signup and view all the answers

What is the role of Dornase-alfa in the treatment of cystic fibrosis?

<p>It acts as a nucleotidase to soften mucus. (A)</p> Signup and view all the answers

What indicates a possible diaphragmatic rupture in a trauma patient?

<p>Obscured left hemidiaphragm on CXR (C)</p> Signup and view all the answers

Which condition is characterized by acute-onset sharp chest pain in a tall, lanky teenager?

<p>Spontaneous pneumothorax (C)</p> Signup and view all the answers

What does a contralateral tracheal shift indicate in a patient?

<p>Tension pneumothorax (A)</p> Signup and view all the answers

What mechanism primarily leads to the symptoms of tension pneumothorax?

<p>Compression of the vena cavae (B)</p> Signup and view all the answers

What finding is expected in a physical examination of a patient with pneumothorax?

<p>Diminished breath sounds (D)</p> Signup and view all the answers

What is the standard initial treatment for pneumothorax?

<p>Needle decompression followed by chest tube (B)</p> Signup and view all the answers

Which of the following conditions could lead to water-soluble contrast being visualized in the mediastinum?

<p>Ruptured bronchus (A)</p> Signup and view all the answers

What correlates with low blood pressure in the context of pneumothorax?

<p>Tension pneumothorax (A)</p> Signup and view all the answers

What is a likely cause of low bicarbonate levels in a patient experiencing shock?

<p>Lactic acidosis due to poor perfusion (D)</p> Signup and view all the answers

Which clinical presentation is more indicative of an amniotic fluid embolism rather than a pulmonary embolism?

<p>Shortness of breath and tachycardia occurring 30 seconds to 2 minutes post-delivery (C)</p> Signup and view all the answers

What critical finding would suggest fat embolism in a patient with a long-bone fracture?

<p>Petechiae on the shoulders/chest (B)</p> Signup and view all the answers

What is the most common presentation of a patient experiencing an air embolism after central venous line insertion?

<p>Sudden death (C)</p> Signup and view all the answers

Which statement accurately describes lactic acidosis in the context of shock?

<p>It results from tissue hypoxia leading to anaerobic metabolism. (D)</p> Signup and view all the answers

What event is often the precursor to developing disseminated intravascular coagulation (DIC) after childbirth?

<p>Bleeding from catheter lines (B)</p> Signup and view all the answers

In the case of pulmonary embolism, which scenario would most likely lead to a diagnosis?

<p>Sudden chest pain and dyspnea two days after surgery (A)</p> Signup and view all the answers

What is a characteristic diagnostic method for identifying fat embolism in pulmonary biopsy?

<p>Oil Red O or Sudan black staining (D)</p> Signup and view all the answers

Flashcards

What is Atelectasis?

A condition where lung tissue collapses, usually due to blocked airways.

What is the significance of Atelectasis post-surgery?

Atelectasis is the most common cause of fever within 24 hours of a post-surgical procedure.

What is Cystic Fibrosis?

A genetic disorder that affects the CFTR gene, causing thick mucus buildup in the lungs, pancreas, and other organs.

What is the Sweat Chloride Test?

A test used to diagnose Cystic Fibrosis by measuring the amount of chloride in sweat.

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What is Congenital Bilateral Absence of the Vas Deferens (CBAVD)?

A condition where a male with Cystic Fibrosis has absent or blocked vas deferens, leading to infertility.

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What are -caftor medications?

A drug class used for Cystic Fibrosis treatment that helps CFTR proteins localize to the cell membrane and fold properly.

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What is Dornase-alfa?

An enzyme that helps break down thick mucus in patients with Cystic Fibrosis.

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What is Guaifenesin?

A medication used to thin mucus and make it easier to cough up.

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Pneumothorax

A condition where air leaks into the pleural space between the lung and the chest wall, causing the lung to collapse.

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Tension Pneumothorax

A type of pneumothorax where the pressure in the pleural space is greater than atmospheric pressure, compressing the lung and heart.

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Spontaneous Pneumothorax

A spontaneous pneumothorax that occurs without an obvious cause.

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Traumatic Pneumothorax

A type of pneumothorax that occurs as a result of injury, often from trauma.

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Diaphragmatic Rupture

A condition where the diaphragm ruptures, allowing abdominal contents to enter the chest.

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Needle Decompression

The process of inserting a needle into the chest to relieve pressure from a tension pneumothorax.

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Chest Tube Insertion

The process of inserting a chest tube into the chest cavity to drain air or fluid, often used after needle decompression for pneumothorax.

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Tube Thoracostomy

A surgical procedure where an incision is made in the chest wall to allow access to the chest cavity.

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Sarcoidosis

An autoimmune disorder characterized by non-caseating granulomas in the lungs, leading to hypercalcemia due to increased 1,25-(OH)2-D3 production.

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Epithelioid macrophages

Activated macrophages found in the granulomas of sarcoidosis.

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1,25-(OH)2-D3

The active form of vitamin D, produced by epithelioid macrophages in sarcoidosis.

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Parathyroid hormone (PTH)

The hormone responsible for regulating calcium levels in the blood.

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Erythema nodosum

A common presenting symptom of sarcoidosis, characterized by red and tender bumps on the shins.

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Bihilar lymphadenopathy

A diagnostic finding on chest X-ray or CT scan in sarcoidosis, indicating enlarged lymph nodes in the area near the lungs.

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Sarcoidosis vs. Asthma

If a patient presents with dry cough and a normal chest X-ray, suspect asthma instead of sarcoidosis.

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Sarcoidosis: Other Findings

Sarcoidosis can also present with elevated liver enzymes (AST, ALP) and weight loss, but hypercalcemia may be absent.

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What are P2 and Tricuspid Regurgitation in pulmonary hypertension?

A strong murmur in the pulmonic area (P2) and tricuspid regurgitation, however, are physical findings that suggest pulmonary hypertension.

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What is Endothelin-1?

A vasoconstrictor that is elevated in pulmonary hypertension, regardless of the cause, and particularly important in postpartum pulmonary hypertension.

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What is Bosentan?

A drug that blocks endothelin-1 receptors and is a high-yield treatment for pulmonary hypertension.

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What is Systemic Sclerosis (Scleroderma)?

An autoimmune disease marked by fibrosis (scarring) and hardening of multiple organs, including the skin.

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What is Limited Scleroderma (CREST syndrome)?

A subtype of systemic sclerosis involving skin tightening of fingers (sclerodactyly), Raynaud's phenomenon, esophageal dysmotility, and sometimes calcium deposits (calcinosis).

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What is Diffuse Scleroderma?

A subtype of systemic sclerosis characterized by CREST syndrome plus renal involvement, leading to high blood pressure.

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What is the Pulmonary Impact of Scleroderma?

Both limited and diffuse scleroderma can lead to pulmonary fibrosis, which in turn causes pulmonary hypertension.

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What is Pericardial Fibrosis in Scleroderma?

Scleroderma can cause inflammation and scarring of the pericardium, the sac surrounding the heart.

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Primary Ciliary Dyskinesia (Kartagener Syndrome)

A genetic disorder caused by abnormal cilia function due to defective dynein arms, leading to recurrent pneumonias and situs inversus or dextrocardia.

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Dynein

A molecule crucial for cilia function. Its defects are the underlying cause of Primary Ciliary Dyskinesia.

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Dextrocardia

A condition where the heart is on the right side of the body, and other organs may be reversed.

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Situs Inversus

Condition where the internal organs are arranged in a reversed position.

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Primary Pulmonary Hypertension (PPH)

Lung disease characterized by elevated pulmonary vascular markings and loud P2 heart sound (or tricuspid regurgitation) in a young, non-smoking female.

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BMPR2 gene

A gene mutation commonly found in patients with Primary Pulmonary Hypertension (PPH).

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Cor Pulmonale

A chronic condition where the right ventricle of the heart enlarges and weakens due to prolonged pressure from the lungs. It can be a consequence of Primary Pulmonary Hypertension.

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Cor Pulmonale due to Primary Pulmonary Hypertension

A condition characterized by right ventricular hypertrophy (RVH) and right heart failure, often seen in those with underlying Primary Pulmonary Hypertension.

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Amniotic Fluid Embolism

A life-threatening condition where amniotic fluid enters the maternal bloodstream, causing a range of complications, such as respiratory distress, cardiovascular collapse, and disseminated intravascular coagulation (DIC).

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Fat Embolism

A pulmonary embolism caused by fat emboli, typically originating from a long bone fracture. It presents with shortness of breath, petechiae (small red spots) on the shoulders/chest, and thrombocytopenia (low platelet count).

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Air Embolism

A life-threatening condition where air enters the bloodstream, usually during invasive procedures such as central venous line insertion, leading to blockage of pulmonary vessels and potentially fatal respiratory failure.

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Lactic Acidosis in Shock

A state of decreased blood flow and oxygen supply to the tissues, often caused by shock. This can lead to lactic acidosis, a condition where the body produces excessive lactic acid due to anaerobic metabolism.

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Lactic Acidosis

The buildup of lactic acid in the blood, often associated with shock or other conditions that restrict oxygen delivery to the tissues. It is characterized by low bicarbonate levels and decreased pH in blood.

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Dead Space

A condition where the lungs are unable to effectively exchange oxygen and carbon dioxide, often due to obstruction or perfusion issues. This can lead to a decrease in oxygen saturation and an increase in carbon dioxide levels in the blood.

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Pulmonary Edema

A condition where the lungs are filled with fluid, making it difficult to breathe. This can be caused by various factors such as heart failure, pneumonia, or embolism.

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Bicarbonate Level

A type of blood test that measures the amount of bicarbonate in the blood. Bicarbonate is an important component of the body's acid-base balance.

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Study Notes

Asthma

  • Asthma can be idiopathic or hereditary, triggered by allergens or cold air.
  • A third of asthma patients only present with a dry cough (cough-variant asthma). This cough is often worse in winter.
  • It can be part of a broader presentation of conditions like seasonal allergies, eye/nose irritation, hives, and skin rashes.
  • Aspirin can induce asthma in some, via a pathway impacting leukotrienes. Aspirin allergy, asthma, and nasal polyps are indicators of this triad.
  • Asthma often presents with increased expiratory effort, but this is not specific.
  • Acutely, asthma causes decreased CO2, increased pH (respiratory alkalosis). Increased breathing rate initially leads to decrease in CO2 buildup.
  • The combination of CO2 and O2 levels in an acute asthma attack can be indicative of type I respiratory failure (low CO2 and high pH).
  • Eventually, fatigue and slowing of breathing can lead to an increase in CO2 and pH reversal (transition to type II respiratory failure, marked by high CO2 and low O2).
  • Intubation may be a necessary intervention.
  • Spirometry and methacholine challenges can help diagnose asthma.
  • First-line outpatient treatment is a Beta-2 agonist (albuterol).
  • Patients with weekly episodes often require continuous use of inhaled corticosteroids (ICS) in addition to Beta-2 agonists.

Bronchiectasis

  • Most common causes worldwide and in Western countries are tuberculosis and cystic fibrosis respectively.
  • Patients often describe copious amounts of foul-smelling sputum.
  • Clubbing is frequently present.
  • This condition isn't related to asthma

Atelectasis

  • Lung collapse is the primary feature.
  • Post-surgery, this is a common cause of fever within 24 hours.
  • Bibasilar shadows seen on x-rays.
  • Related to slower, shallower breathing in patients on respiratory support post-surgery.
  • Resorptive atelectasis occurs when obstruction distally in a section of lung causes distal area collapse.
  • Obstruction can be due to a tumor.
  • Chronic respiratory acidosis (increased CO2, decreased pH) is a characteristic.

Obstructive sleep apnea (OSA)

  • OSA can contribute to pulmonary hypertension, leading to cor pulmonale.

Anaphylaxis

  • Characterized by acute dyspnea, bilateral wheezing, tachycardia, and low blood pressure.
  • Triggers include environmental or food allergens, insect stings and recently introduced medication.
  • Epinephrine is a key treatment.

Scombroid

  • An often misdiagnosed condition that may mimic seafood allergy;
  • Due to toxin produced in decaying fish;
  • Treatment involves antihistamine medications.

Shellfish allergy

  • Triggered by shellfish consumption.
  • Can display similar symptoms to asthma.
  • Not related to scombroid syndrome.

Cystic fibrosis

  • Autosomal recessive, caused by mutations in the CFTR gene.
  • Characterized by abnormal chloride channel function.
  • Causes thick secretions in the lungs, resulting in recurrent infections (pneumonia).
  • Pancreatic insufficiency is a common symptom, which leads to fat malabsorption and vitamin deficiency.
  • Males can present with infertility due to the absent vas deferens in congenital form.

Primary ciliary dyskinesia (Kartagener syndrome)

  • Autosomal recessive, a disorder of cilia function.
  • Characterized by situs inversus (organs reversed).
  • Presents with chronic respiratory infections.

Primary pulmonary hypertension (PPH)

  • Pulmonary hypertension originating in the lungs.
  • Elevated endothelin-1 levels often present.
  • Characterized by right-ventricle hypertrophy.

Systemic sclerosis

  • Autoimmune disorder causing fibrosis in various parts of the body (sclerosis).
  • Classified as limited or diffuse subtypes.
  • Limited scleroderma and CREST syndrome (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasias) are frequent presentations.
  • Associated with pulmonary fibrosis and hypertension.

Sarcoidosis

  • Idiopathic condition with non-caseating granulomas in various organs;
  • Often affects lungs.
  • Characteristic of altered vitamin D metabolism, and hypercalcemia.

Rheumatoid lung

  • Restrictive lung disease, is a complication of rheumatoid arthritis.
  • Fibrosis development can lead to problems breathing.

Pulmonary embolism

  • Obstruction by clots in the pulmonary arteries.
  • Risk factors include, recent surgery, in-situ issues, and inherited blood clotting disorders.
  • Characterized by acute shortness of breath, tachycardia, and hypoxia.
  • Possible for it to occur while on oral anticoagulation medicines.

Amniotic fluid embolism

  • Obstetric complication.
  • Related to placental separation and amniotic fluid entering the circulatory system after delivery.
  • Presents similar to pulmonary embolism and potentially leads to coagulopathy.

Fat embolism

  • Embolization of fat post-fracture;
  • Characterized by shortness of breath and petechiae over the body.

Air embolism

  • Air blockage in circulatory system via introduction via a medical procedure,
  • Often leads to sudden death.

Cholesterol embolism

  • Cholesterol plaques breaking off and lodging in the lungs (and vasculature).
  • Common presentation of a previously unremarked or uninvestigated abdominal aortic aneurysm.
  • Skin manifestations in the toes are a frequent presentation.

Pleurisy

  • Inflammation of lining of the lungs and the chest cavity.
  • Characterized with chest pain.
  • Can be indicative of an underlying condition like pneumonia, or malignant processes.

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