Hereditary Hemolytic Anemia

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50 Questions

Which component makes up almost half of the blood volume?

Red blood cells

What is the function of the lymphatic system?

Fighting infection

What is the usual cause of anemia as a result of bone marrow damage?

Decreased red blood cell production

What is the process of hematopoietic stem cell therapy?

Replacement of damaged bone marrow

Which of the following is a common manifestation of hemochromatosis?

Heart failure

What is the treatment for hemochromatosis?

Phlebotomy

Which of the following is a cause of anemia?

Vitamin B12 deficiency

What is the most common type of anemia?

Iron-deficiency anemia

Which part of hemoglobin forms different chains designated by Greek letters such as alpha, beta, gamma, delta, and epsilon?

Globin

What is the most common type of hemoglobin in adults?

HgbA

What happens to the level of gamma hemoglobin in sickle cell anemia?

It increases

What is the role of erythropoietin in red cell production?

Regulates oxygen content of arterial blood

Which of the following is a primary focus in the treatment of anemia?

Identifying the cause of anemia

What is the term used to describe anemia characterized by the presence of large red blood cells?

Macrocytic anemia

Which of the following can cause folic acid deficiency anemia?

All of the above

What is the main cause of pernicious anemia?

Gastric mucosal atrophy

Which component of blood is responsible for transporting substances to tissues via the circulatory system?

Plasma

What is the primary function of red blood cells (RBC)?

Transport of oxygen

Which type of leukocytes are the most numerous in adults and play a vital role in the first line of defense against infections?

Neutrophils

What is the function of platelets in the blood?

Blood coagulation

Which of the following is a characteristic of hereditary spherocytosis?

Abnormal shape of red blood cells

Which hemoglobin abnormality is predominantly found in persons of African descent?

Hemoglobin S (sickle hemoglobin)

Which condition is associated with defective synthesis of alpha or beta globulin?

Thalassemia

Which disease is characterized by the formation of rigid fibers in deoxygenated conditions, causing 'sickling' of red blood cells?

Sickle cell disease

Which of the following is a diagnostic evaluation for anemia?

History and physical examination

What is the most common cause of secondary polycythemia?

Chronic infection/inflammation

What is the treatment for primary polycythemia?

Drugs that suppress marrow function

What are the complications of polycythemia?

Clot formation due to increased blood viscosity and platelet count

What are the clotting disorders associated with clots forming in small blood vessels?

Thrombotic Thrombocytopenic Purpura (TTP)

What is the purpose of a bone marrow study in the evaluation of anemia?

To study characteristic abnormalities in marrow cells

What is the primary focus of chronic obstructive lung disease?

Inflammation and irreversible airflow obstruction

Which condition is characterized by the formation of rigid fibers in deoxygenated conditions, causing 'sickling' of red blood cells?

Sickle cell anemia

What are the major types of lung carcinoma?

Adenocarcinoma, squamous cell carcinoma, large cell carcinoma, and small cell carcinoma

Which condition is characterized by fibrous thickening of alveolar septa, making the lungs rigid and restricting normal respiratory excursions?

Pulmonary Fibrosis

Which lung injury is caused by inhalation of injurious dust or other particulate material, such as rock dust or asbestos fibers?

Pneumoconiosis

Which lung condition is associated with the inhalation of irritant or toxic gases, leading to alveolar damage?

ARDS

Which lung injury is characterized by the thickening of alveolar septa from irritant gases, organic and inorganic particles, inflammation, and repair post ARDS?

Pulmonary Fibrosis

Which lung condition is associated with lung injury from inhalation of injurious dust or other particulate material, such as rock dust and asbestos fibers?

Pneumoconiosis

Which structure in the respiratory system is responsible for the exchange of oxygen and carbon dioxide?

Alveoli

What is the functional unit of the lung for gas exchange?

Acinus

Which condition can result from the escape of air into the pleural space due to lung injury or disease?

Pneumothorax

Which factor contributes to the solidification of affected lung portions in pneumonia?

Inflammation leading to exudate filling alveoli

What is the primary etiology of pneumonia?

Bacterial infection

Which respiratory infection is characterized by a relatively low fatality rate but a high infection count?

COVID-19 (SARS Cov2)

Which respiratory disease is characterized by chronic inflammation of terminal bronchioles and purulent sputum?

Chronic bronchitis

What is the primary cause of chronic obstructive pulmonary disease (COPD)?

Chronic irritation of the respiratory mucosa by smoking or atmospheric pollution

Which form of tuberculosis is characterized by systemic spread and is more common in AIDS patients and immunocompromised individuals?

Miliary tuberculosis

What is the main cause of Pulmonary emphysema?

Destruction of the fine alveolar structure of the lungs

What is the primary focus in the treatment of Bronchial asthma?

Identifying and controlling triggers

What is the characteristic manifestation of Adult Respiratory Distress Syndrome?

Shock as a major manifestation

Study Notes

Respiratory Diseases Overview

  • Pulmonary emphysema is characterized by the destruction of the fine alveolar structure of the lungs, leading to the formation of large cystic spaces, initially affecting the upper lobes and eventually all lobes.
  • Chronic Obstructive Pulmonary Disease (COPD) is caused by chronic irritation, mainly from smoking and inhalation of injurious agents, leading to three main anatomic derangements in the lungs.
  • The pathogenesis of COPD involves chronic inflammatory swelling of the mucosa, which narrows bronchioles, increases resistance to expiration, and causes air trapping in the lungs.
  • Management of COPD includes promoting drainage of bronchial secretions, oxygen therapy, bronchodilators, inhaled steroids, and smoking cessation to halt disease progression.
  • Lung damage caused by emphysema cannot be restored to normal, and those with Alpha1-Antitrypsin Deficiency are prone to developing emphysema if their levels are low.
  • Antitrypsin, an alpha1 globulin, normally inactivates enzymes that may injure alveolar septa, and its deficiency permits enzymes to damage lung tissue.
  • Bronchial asthma is characterized by spasmodic contraction of smooth wall muscles of bronchi and bronchioles and increased bronchial mucous secretions in response to various stimuli, with treatment focused on identifying and controlling triggers.
  • Neonatal Respiratory Distress Syndrome presents as progressive respiratory distress soon after birth and is treated with adrenal corticosteroids to the mother before delivery, oxygen, and surfactant.
  • The pathogenesis of Neonatal Respiratory Distress Syndrome involves inadequate surfactant in the lungs, leading to compromised oxygen exchange and increased permeability of pulmonary capillaries.
  • Adult Respiratory Distress Syndrome is characterized by shock as a major manifestation, caused by damage to the alveolar membrane and fluid leaking in, and has a high mortality rate, especially in cases like COVID-19.
  • Treatment for Adult Respiratory Distress Syndrome involves treating the underlying condition that caused shock, oxygen ventilator with slightly increased pressure, and has a mortality rate of 30-40%.
  • The text is copyrighted by Jones & Bartlett Learning, LLC, an Ascend Learning Company, and it provides detailed information on the pathogenesis and management of various respiratory diseases.

Test your knowledge on Hereditary Hemolytic Anemia - round, a condition caused by either an intrinsic defect in red cell structure or an extrinsic condition that shortens the circulating life of red cells. Learn about the specific type of hereditary spherocytosis, characterized by an abnormal shape of red blood cells. Find out how damage to the cytoskeleton creates a disk shape that is not suitable for passing through thin capillaries, making them weaker and more susceptible to damage, resulting

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