Question 1

What organ is responsible for removing sickled cells from circulation?

Accepted Answer

The spleen is responsible for filtering and removing abnormal or sickled red blood cells from circulation.

Question 2

A 1-year-old female is diagnosed with anemia secondary to insufficient erythropoiesis. What is the most likely cause?

Accepted Answer

Iron deficiency is the most common cause of anemia due to insufficient erythropoiesis in young children, as it is essential for hemoglobin production.

Question 3

By what other name is the clotting factor IX disorder, hemophilia B, known as?

Accepted Answer

Hemophilia B is also known as Christmas disease, named after Stephen Christmas, the first patient diagnosed with the disorder. The other options, classic hemophilia and von Willebrand disease, are separate bleeding disorders. Thalassemia is a genetic blood disorder that affects the production of hemoglobin.

Question 4

What type of genetic disorders are the alpha and beta thalassemias?

Accepted Answer

Alpha and beta thalassemias are autosomal recessive disorders, meaning that two copies of the mutated gene, one from each parent, are necessary for an individual to be affected.

Question 5

If an infant has hydrops fetalis, which type of thalassemia does the nurse know this to be?

Accepted Answer

Hydrops fetalis is associated with alpha thalassemia major, which occurs when there is a deletion of all four alpha-globin genes, leading to severe anemia and often resulting in fetal death.

Question 6

Which mother-fetus combination is at most risk for a maternal-fetal blood incompatibility?

Accepted Answer

When an Rh-negative mother carries an Rh-positive fetus, there is a risk that the mother's immune system will produce antibodies against the Rh factor in the fetus's blood. This can cause hemolytic disease of the newborn (HDN), a serious and potentially life-threatening condition. In the other combinations listed, there is still a risk of maternal-fetal blood incompatibility, but it is not as high as in the case of an Rh-negative mother carrying an Rh-positive fetus.

Question 7

A mother has a child that is diagnosed with sickle cell anemia. While the mother does not have the disease, which characteristic of her genetic makeup is responsible for the child's disease?

Accepted Answer

Sickle cell anemia is an autosomal recessive disorder caused by a mutation in the HBB gene, which instructs the body to make beta-globin subunits of hemoglobin. People with sickle cell anemia have two copies of the mutated HBB gene, resulting in the production of abnormal hemoglobin (Hb S). In the case of this question, the mother is a carrier, meaning she has one normal HBB gene (Hb A) and one mutated HBB gene (Hb S). If both parents are carriers, there is a one in four chance that their child will inherit two copies of the mutated HBB gene and develop sickle cell anemia. Therefore, the child inherited one Hb S gene from the mother and one Hb S gene from the father (who is not specified in the question).

Question 8

A 6-year-old male presents with marked fatigue, jaundice, and irritability. A blood smear shows the presence of sickled cells. Erythropoiesis is compromised in this child. Which crisis should the nurse monitor the patient for?

Accepted Answer

An aplastic crisis occurs when erythropoiesis is compromised, often due to parvovirus B19 infection, leading to a sudden drop in hemoglobin levels in patients with sickle cell disease.

Question 9

Testing reveals that a child has hemophilia A. This bleeding disorder results from a deficiency in factor:

Accepted Answer

Hemophilia A is caused by a deficiency in factor VIII, which is important for blood clotting. Deficiency in other factors such as IX, XII, and XIII can cause different types of bleeding disorders.

Question 10

A 2-year-old male presented with growth and maturation retardation and splenomegaly. He died shortly after arriving at the ER. Autopsy revealed thalassemia, a condition involving what feature?

Accepted Answer

Thalassemia is a genetic disorder characterized by defective hemoglobin synthesis, leading to anemia and other related symptoms.

Question 11

Which major symptom indicates to the nurse that patient diagnosed with sickle cell anemia is experiencing a vaso-occlusive crisis?

Accepted Answer

Pain is a major symptom of vaso-occlusive crisis in sickle cell anemia. Peripheral edema may occur in some cases, but it is not a major symptom of vaso-occlusive crisis. Petechiae and an enlarged spleen are not specific symptoms of vaso-occlusive crisis.

Question 12

A 5-year-old male is diagnosed with immune thrombocytopenic purpura (ITP). What is the most common cause of this condition?

Accepted Answer

Immune thrombocytopenic purpura (ITP) in children is most commonly caused by the production of antibodies following a viral infection, which leads to the destruction of platelets.

Question 13

In Canada, which group of people should be assessed first for sickle cell disease?

Accepted Answer

Black people of African descent are at a higher risk for sickle cell disease, so they should be assessed first.

Question 14

A 16-month-old female presents with tachycardia, pallor, anorexia, and systolic murmur. The nurse is checking the laboratory results, and the hemoglobin determination indicates a level below _____ mmol/L.

Accepted Answer

The answer of A 16-month-old female presents with tachycardia, pallor,...

Question 15

Which of the following amino acids is present in Hb S and not present in normal Hb?

Accepted Answer

Hb S has a substitution of valine for glutamic acid at position 6 in the beta chain, which is not present in normal Hb.

Question 16

Autopsy on a stillborn fetus reveals cardiomegaly, hepatomegaly, edema, and ascites as well as fulminant intrauterine heart failure. Considering these findings, what is the most likely cause of death?

Accepted Answer

Alpha thalassemia major, also known as hydrops fetalis, is characterized by the absence of alpha-globin chains, leading to severe anemia, heart failure, and fetal hydrops, which matches the findings of cardiomegaly, hepatomegaly, edema, and ascites.

Question 17

A 3-year-old child presents with bruising on the legs and trunk and a petechial rash. The mother also reports frequent nosebleeds. Lab tests reveal a decreased platelet count. This symptomology supports which medical diagnosis?

Accepted Answer

The presentation of bruising, petechial rash, frequent nosebleeds, and decreased platelet count are all consistent with a diagnosis of immune thrombocytopenic purpura (ITP). Leukemia may also present with similar symptoms, but the decreased platelet count is not always present in leukemia. Thalassemia and hemophilia do not typically present with these symptoms.

Question 18

What is the most likely cause of death associated with sickle cell disease?

Accepted Answer

Infection is the most common cause of death in sickle cell disease patients. They are at higher risk for bacterial infections due to functional asplenia and impaired immune function. Infections such as pneumonia, sepsis, meningitis, and osteomyelitis can lead to severe morbidity and mortality in sickle cell disease patients.

Question 19

Which type of sickle cell crisis occurs only in young children?

Accepted Answer

Sequestration crisis occurs only in young children, usually under 5 years old. It is characterized by a sudden and massive enlargement of the spleen and liver, leading to rapid pooling of blood in those organs and a significant decrease in circulating blood volume. This can be life-threatening and requires immediate medical attention.

Question 20

A 12-month-old toddler weighing 8 kilograms is brought to the clinic because of weakness, slow physical growth, and developmental delays. His mother reports that the only food he will consume is cow's milk. The symptoms support a diagnosis of which form of anemia?

Accepted Answer

The toddler's diet consisting only of cow's milk can lead to iron deficiency anemia, as cow's milk is low in iron and can interfere with iron absorption.

Quiz 22: Alterations of Hematological Function in Children