Question 1

The alpha and beta thalassemias are inherited in an _____ fashion.

Accepted Answer

Alpha and beta thalassemias are inherited in an autosomal recessive manner, meaning that two copies of the mutated gene (one from each parent) are necessary for the condition to be expressed.

Question 2

If an infant has hydrops fetalis, which type of thalassemia does the nurse suspect?

Accepted Answer

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

Question 3

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

Accepted Answer

When the mother is Rh-negative and the fetus is Rh-positive, there is a risk of Rh incompatibility, which can lead to hemolytic disease of the newborn if not managed properly.

Question 4

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 described with this condition.

Question 5

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 clotting factor VIII.

Question 6

A 6-year-old male presents with 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 and reticulocyte count in patients with sickle cell disease.

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

The mother is a carrier of the sickle cell trait, meaning she has one normal hemoglobin allele (Hb A) and one sickle cell allele (Hb S). This allows her to pass the Hb S allele to her child, who can develop sickle cell anemia if they inherit another Hb S allele from the father.

Question 8

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

Accepted Answer

Pain is the major symptom of a vaso-occlusive crisis in sickle cell anemia, caused by the blockage of blood flow due to sickled red blood cells.

Question 9

A 2-year-old male presented with growth and maturation retardation and splenomegaly.He died shortly after arriving at the ER.Autopsy revealed thalassemia secondary to defective:

Accepted Answer

Thalassemia is a genetic disorder characterized by defective hemoglobin synthesis, leading to ineffective erythropoiesis and anemia.

Question 10

A 16-month-old female presents with tachycardia, pallor, anorexia, and systolic murmur.The nurse is checking the lab results, and the hemoglobin determination indicates a level below _____ grams per deciliter.

Accepted Answer

A hemoglobin level below 5 grams per deciliter is considered severe anemia in a 16-month-old female.

Question 11

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

Accepted Answer

Immune thrombocytic 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 12

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

Accepted Answer

Hb S has valine at the 6th position of the beta-globin chain, while normal Hb has glutamic acid at this position.

Question 13

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

Accepted Answer

Sequestration crisis occurs primarily in young children due to the sudden pooling of blood in the spleen, leading to splenic enlargement and potentially life-threatening anemia.

Question 14

A 12-month-old toddler weighing 18 pounds 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 symptoms and dietary history suggest iron deficiency anemia, which is common in toddlers who consume excessive cow's milk, as it is low in iron and can interfere with iron absorption.

Question 15

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 symptoms of bruising, petechial rash, frequent nosebleeds, and decreased platelet count are indicative of Immune Thrombocytopenic Purpura (ITP), a condition characterized by low platelet levels leading to increased bleeding and bruising.

Question 16

Autopsy on a stillborn fetus reveals cardiomegaly, hepatomegaly, edema, and ascites as well as fulminant intrauterine congestive 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 1-year-old female is diagnosed with anemia secondary to insufficient erythropoiesis.The most likely cause is:

Accepted Answer

Iron deficiency is the most common cause of anemia due to insufficient erythropoiesis in young children.

Question 18

In the United States, which group of people should be assessed first for sickle cell disease?

Accepted Answer

Sickle cell disease is most prevalent among individuals of African descent, so Blacks are the group that should be assessed first for this condition in the United States.

Question 19

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

Accepted Answer

Infection is the most common cause of death in individuals with sickle cell disease due to their increased susceptibility to infections, particularly from encapsulated bacteria, as a result of functional asplenia.

Question 20

Sickled cells will be removed from circulation mostly by the:

Accepted Answer

The spleen is responsible for filtering abnormal red blood cells, including sickled cells, from the bloodstream.

Quiz 24: Alterations of Hematologic Function in Children