Question 1

A 25-year-old female has a child that is diagnosed with sickle cell anemia.She does not have the disease.Which of the following characterizes the genetic makeup of the parents with regard to this trait?

Accepted Answer

In this instance the child inherits Hb S from one parent and normal hemoglobin (Hb A),not Hb S,for both.
In this instance the child inherits Hb S from one parent and normal hemoglobin (Hb A).
In this instance the child inherits Hb S from one parent and normal hemoglobin (Hb A),not Hb C,and Hb S.
In this instance the child inherits Hb S from one parent and normal hemoglobin (Hb A).

Question 2

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 would be secondary to defective hemoglobin synthesis.
Thalassemia would be secondary to defective hemoglobin synthesis; it is not due to erythrocyte membrane abnormalities.
Thalassemia would be secondary to defective hemoglobin synthesis.It is not due to defective iron metabolism.
Thalassemia would be secondary to defective hemoglobin synthesis.It is not due to defective stem cell formation.

Question 3

A geneticist is discussing thalassemias.Which information should be included? The alpha and beta thalassemias are inherited in an _____ fashion.

Accepted Answer

The alpha and beta thalassemias are inherited autosomal recessive disorders.
The alpha and beta thalassemias are inherited autosomal recessive disorders,not dominant.
The alpha and beta thalassemias are inherited autosomal recessive disorders,not X-linked recessive.
The alpha and beta thalassemias are inherited autosomal recessive disorders,not X-linked dominant.

Question 4

A 22-year-old female gives birth at 20 weeks' gestation to a stillborn fetus.Physical exam of the fetus by the nurse reveals gross edema of the entire body and anemia.This condition is referred to as:

Accepted Answer

Fetuses that do not survive anemia in utero usually are stillborn,with gross edema in the entire body,a condition called hydrops fetalis.
Fetuses that do not survive anemia in utero usually are stillborn,with gross edema in the entire body,a condition called hydrops fetalis,not hyperbilirubinemia.
Fetuses that do not survive anemia in utero usually are stillborn,with gross edema in the entire body,a condition called hydrops fetalis,not erythroblastosis fetalis.
Fetuses that do not survive anemia in utero usually are stillborn,with gross edema in the entire body,a condition called hydrops fetalis,not ascites.

Question 5

A 5-year-old female dies from sickle cell disease.The pediatrician will document the most likely cause of death is:

Accepted Answer

Infection is the most common cause of death related to sickle cell disease.
Infection,not decreased hemoglobin,is the most common cause of death related to sickle cell disease.
Infection,not an obstructive crisis,is the most common cause of death related to sickle cell
disease.
Infection,not a hyperhemolytic crisis,is the most common cause of death related to sickle cell disease.

Question 6

When should the nurse start closely monitoring the patient with sickle cell anemia? If:

Accepted Answer

Sickling occurs when oxygen tension is low.
Sickling occurs when oxygen tension is low,not due to pH.
Sickling occurs when oxygen tension is low,not due to a decrease in plasma osmolality.
Sickling occurs when oxygen tension is low,now due to the development of fever.

Question 7

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.Considering this information,the nurse decides that the child probably has _____ anemia.

Accepted Answer

As many as one-third of infants with severe iron deficiency anemia have chronic intestinal blood loss induced by exposure to a heat-labile protein in cow's milk.Such exposure causes an inflammatory gastrointestinal reaction that damages the mucosa and results in diffuse hemorrhage.
The intake of cow's milk leads to iron deficiency anemia; it does not lead to pernicious anemia.
The intake of cow's milk leads to iron deficiency anemia; it does not lead to aplastic anemia.
The intake of cow's milk leads to iron deficiency anemia; it does not lead to hemolytic anemia.

Question 8

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

Accepted Answer

Vaso-occlusive crisis is extremely painful and may last for days or even weeks,with an average duration of 4 to 6 days.
Vaso-occlusive crisis is extremely painful; it does not promote peripheral edema.
Vaso-occlusive crisis is extremely painful; it does not promote petechiae.
Vaso-occlusive crisis is extremely painful; it does not lead to an enlarged spleen.

Question 9

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

Accepted Answer

In the United States,sickle cell disease is most common in African-Americans.
In the United States,sickle cell disease is most common in African-Americans,not Asians.
In the United States,sickle cell disease is most common in African-Americans,not Hispanics.
In the United States,sickle cell disease is most common in African-Americans,not Caucasians.

Question 10

Sickle cell disease is characterized by the presence of Hb S.A nurse is trying to differentiate between Hb S and normal Hb.Which of the following amino acids is present in Hb S and not present in normal Hb?

Accepted Answer

Hb S is formed by a genetic mutation in which one amino acid (valine)replaces another (glutamic acid).
Valine has replaced glutamic acid.
Hb S is formed by a genetic mutation in the amino acid (valine),not proline.
Hb S is formed by a genetic mutation in the amino acid (valine),not histidine.

Question 11

The mother of a 3-year-old child speaks to the pediatrician because the child took several hours to stop bleeding following a minor scrape.Testing reveals that the child has hemophilia A resulting from a deficiency in factor:

Accepted Answer

Hemophilia A is a deficiency in factor VIII.
Hemophilia A is a deficiency in factor VIII,not IX.
Hemophilia A is a deficiency in factor VIII,not factor XII.
Hemophilia A is a deficiency of factor VIII,not factor XIII.

Question 12

For which type of sickle cell crisis should the nurse assess for in young children?

Accepted Answer

Sequestration occurs when large amounts of blood become acutely pooled in the liver and spleen.This type of crisis is seen only in a young child.
Sequestration,not hyperhemolysis,occurs when large amounts of blood become acutely pooled in the liver and spleen.This type of crisis is seen only in a young child.
Sequestration,not vaso-occlusive crisis,occurs when large amounts of blood become acutely pooled in the liver and spleen.This type of crisis is seen only in a young child.
Sequestration,not aplastic crisis,occurs when large amounts of blood become acutely pooled in the liver and spleen.This type of crisis is seen only in a young child.

Question 13

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

Accepted Answer

Hydrops fetalis is due to alpha thalassemia major.
Hydrops fetalis is due to alpha thalassemia major,not beta minor.
Hydrops fetalis is due to alpha thalassemia major,not beta major .
Hydrops fetalis is due to alpha thalassemia major,not alpha minor which results in anemia.

Question 14

A 12-year-old male is diagnosed with sickle cell anemia.A blood smear reveals severely sickled cells.A nurse recalls the sickled cells will be removed from circulation mostly by the:

Accepted Answer

Sickled cells undergo hemolysis in the spleen or become sequestered there,causing blood pooling and infarction of splenic vessels.
Sickled cells undergo hemolysis in the spleen,not the liver.
Sickled cells undergo hemolysis in the spleen,not the pancreas.
Sickled cells undergo hemolysis in the spleen,not the kidney.

Question 15

A 1-year-old female is diagnosed with anemia secondary to insufficient erythropoiesis.When the parents asked what caused this,how should the nurse respond? The most likely cause(s)is (are):

Accepted Answer

The most common cause of insufficient erythropoiesis is iron deficiency.
The most common cause of insufficient erythropoiesis is iron deficiency,not genetic factors.
The most common cause of insufficient erythropoiesis is iron deficiency,not a hemoglobin abnormality.
The most common cause of insufficient erythropoiesis is iron deficiency,not an erythrocyte structural abnormality.

Question 16

A 10-year-old male is diagnosed with sickle cell anemia.When the parents ask who is responsible for this disease,what is the nurse's best response? He most likely inherited it from:

Accepted Answer

Sickle cell is a recessive disorder inherited from both parents.
Sickle cell is a recessive disorder inherited from both parents.
Sickle cell is a recessive disorder inherited from both parents.
Sickle cell is a recessive disorder inherited from both parents.

Question 17

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

Aplastic anemia is caused by diminished erythropoiesis despite an increased need for new erythrocytes.
Vaso-occlusive crisis is manifested by pain.
Sequestered crisis is manifested by enlarged spleen.
Hyperhemolytic crisis is due to infection.

Question 18

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

Accepted Answer

Maternal-fetal incompatibility exists if mother and fetus differ in ABO blood type or if the fetus is Rh-positive and the mother is Rh-negative.
Maternal-fetal incompatibility exists if mother and fetus differ in ABO blood type or if the fetus is Rh-positive and the mother is Rh-negative.
Symptoms do not occur in a type O fetus.
Symptoms do not occur in type AB mothers.

Question 19

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 _____ g/dL.

Accepted Answer

When hemoglobin levels fall below 5 g/dL,pallor,anorexia,tachycardia,and systolic murmurs may occur.
When hemoglobin levels fall below 5 g/dL,not 7,pallor,anorexia,tachycardia,and systolic murmurs may occur.
When hemoglobin levels fall below 5 g/dL,not 10,pallor,anorexia,tachycardia,and systolic murmurs may occur.
When hemoglobin levels fall below 5 g/dL,not 14,pallor,anorexia,tachycardia,and systolic murmurs may occur.

Question 20

A 38-year-old female gives birth to a stillborn fetus.Autopsy reveals cardiomegaly,hepatomegaly,edema,and ascites.Which of the following does the nurse suspect caused the death?

Accepted Answer

Alpha thalassemia major causes hydrops fetalis and fulminant intrauterine congestive heart failure.In addition to edema and massive ascites,the fetus has a grossly enlarged heart and liver.
The fetus has alpha thalassemia major,not minor.Major is manifested by heart failure and ascites.
The stillborn fetus has symptoms of alpha thalassemia major,not hemoglobin H disease.
The stillborn fetus has symptoms of alpha thalassemia major,not alpha trait,which is the mildest form of the disease.

Quiz 21: Alterations of Hematologic Function in Children