Question 1

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, resulting in a(n):

Accepted Answer

The presenting symptoms and presence of sickled cells on blood smear suggest sickle cell anemia. The compromised erythropoiesis in this child is likely due to an aplastic crisis, which can be triggered by infection or other stressors and results in a reduction in red blood cell production. Vaso-occlusive crisis and sequestration crisis may also occur in sickle cell disease but would present with different symptoms and laboratory findings. Hyperhemolytic crisis is a rare complication that involves an increase in red blood cell breakdown and is unlikely to be the cause of the presenting symptoms.

Question 2

Iron deficiency anemia:

Accepted Answer

Iron deficiency anemia can be related to socioeconomic factors, as access to iron-rich foods and healthcare can vary based on socioeconomic status.

Question 3

The type of sickle cell crisis seen only in young children is:

Accepted Answer

Sequestration crisis is a type of sickle cell crisis that is seen in young children, especially those under the age of 5. It is characterized by the sudden and massive pooling of blood in the spleen and liver, which can lead to severe anemia, hypovolemia, and shock. It is considered a medical emergency and requires immediate treatment with blood transfusions and other supportive measures. The other types of sickle cell crises (hyperhemolytic, vaso-occlusive, and aplastic) can occur at any age.

Question 4

In the United States, sickle cell disease most commonly occurs in:

Accepted Answer

Sickle cell disease is most commonly found in African Americans, as it is a genetic condition that primarily affects those with African ancestry. It is estimated that 1 in 13 African Americans carry the sickle cell trait, which can lead to the development of sickle cell disease in their offspring.

Question 5

A 10-year-old male is diagnosed with sickle cell anemia. He most likely inherited it from:

Accepted Answer

Sickle cell anemia is an inherited disorder caused by a mutation in the HBB gene, which provides instructions to make a protein called beta-globin. This mutation is inherited in an autosomal recessive pattern, which means that the child must inherit two copies of the mutated gene (one from each parent) to develop sickle cell anemia. Therefore, both the mother and father must be carriers of the sickle cell gene for their child to inherit sickle cell anemia.

Question 6

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

Accepted Answer

Thalassemias are inherited in an autosomal recessive fashion, meaning that two copies of the mutated gene (one from each parent) are required for the disease to manifest.

Question 7

Sickle cell disease is characterized by the presence of Hb S. Which of the following amino acids is present in Hb S and not present in normal Hb?

Accepted Answer

Sickle cell disease is caused by a single point mutation in the β-globin gene, resulting in a substitution of valine for glutamic acid at position 6 of the β-globin chain. Normal hemoglobin (HbA) has a glutamic acid residue at this position.

Question 8

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

Accepted Answer

The spleen is responsible for removing old, damaged, or abnormal red blood cells from circulation. In sickle cell anemia, the sickled cells are more fragile and susceptible to damage, and the spleen will remove them from circulation at a faster rate than normal red blood cells. This increased rate of removal can lead to anemia in individuals with sickle cell anemia.

Question 9

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

Accepted Answer

Infection is a common cause of death in individuals with sickle cell disease, especially in children. Children with sickle cell disease have an increased risk of bacterial infections, especially with Streptococcus pneumoniae, which can cause sepsis or meningitis. Treatment with prophylactic antibiotics and immunizations can help prevent infections in individuals with sickle cell disease.

Question 10

A 16-month-old female presents with tachycardia, pallor, anorexia, and systolic murmur. Hemoglobin determination indicates a content below _____ g/dl.

Accepted Answer

The symptoms of tachycardia, pallor, anorexia, and systolic murmur suggest anemia. A hemoglobin content below 5 g/dl is considered severe anemia in children, which is likely the cause of the symptoms presented.

Question 11

Hydrops fetalis may result from which type of thalassemia?

Accepted Answer

Hydrops fetalis is associated with alpha thalassemia major, where there is a deletion of all four alpha-globin genes, leading to severe anemia and fetal complications.

Question 12

The erythrocytes of a person with sickle cell anemia may become sickled if:

Accepted Answer

The sickling of erythrocytes in sickle cell anemia is triggered by low oxygen tension, which causes hemoglobin to form long, rigid chains that distort the shape of the red blood cell into a sickle shape.

Question 13

A 38-year-old female gives birth to a stillborn fetus. Autopsy reveals cardiomegaly, hepatomegaly, edema, and ascites. The most likely cause is:

Accepted Answer

The findings of cardiomegaly, hepatomegaly, edema, and ascites suggest a diagnosis of hydrops fetalis, which is commonly seen in alpha-thalassemia major. Alpha-thalassemia minor and alpha-trait typically do not cause hydrops fetalis, and while hemoglobin H disease can present with hydrops fetalis, it is less common than alpha-thalassemia major.

Question 14

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

Sickle cell anemia is an autosomal recessive disorder. This means that for a child to have the disease, they must inherit two copies of the mutated HbS gene, one from each parent. The mother does not have the disease, meaning she must have at least one normal HbA gene. Therefore, the mother must be heterozygous for the sickle cell trait, with one mutated HbS gene and one normal HbA gene. The father must also be a carrier, which means he could either be heterozygous with one mutated HbS gene and one normal HbA gene, or he could be homozygous with two mutated HbS genes. However, we cannot determine the father's genotype based on the information given. Option B shows the correct combination of HbS and HbA genes for the parents.

Question 15

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

Accepted Answer

The symptoms of gross edema and anemia in a stillborn fetus at 20 weeks' gestation suggest the presence of hydrops fetalis, which is a severe form of fetal edema caused by various underlying conditions such as immune or non-immune fetal anemia, infections, heart or lung abnormalities, or genetic disorders. Hyperbilirubinemia and erythroblastosis fetalis are conditions that involve the destruction of red blood cells due to maternal-fetal blood group incompatibility or other factors. Ascites refers to the abnormal accumulation of fluid in the abdominal cavity.

Question 16

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 characterized by a deficiency in the synthesis of hemoglobin, which leads to anemia and splenomegaly. This deficiency can be caused by genetic mutations that affect the production of one or more globin chains. In this case, the presentation of growth and maturation retardation and splenomegaly at such a young age suggests a severe form of thalassemia caused by a defect in hemoglobin synthesis.

Question 17

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:

Accepted Answer

A 12-month-old toddler weighing 18 pounds should have transitioned from breastmilk or formula to solid food. Cow's milk does not provide adequate nutrients, including iron, which can lead to iron deficiency anemia. The symptoms of weakness, slow physical growth, and developmental delays are also characteristic of iron deficiency anemia. Pernicious anemia is caused by a deficiency in vitamin B12, which is not related to the information provided. Aplastic anemia is a rare disorder where the body doesn't produce enough new blood cells, and hemolytic anemia is caused by the destruction of red blood cells, neither of which are likely causes for the child's symptoms based on the information given.

Question 18

A major symptom of vaso-occlusive crisis in sickle cell anemia is:

Accepted Answer

The answer of A major symptom of vaso-occlusive crisis in...

Question 19

Maternal-fetal blood incompatibility may exist in which of the following conditions?

Accepted Answer

Maternal-fetal blood incompatibility occurs when the mother's blood type is Rh-negative and the fetus's blood type is Rh-positive. This can lead to hemolytic disease of the newborn, a potentially life-threatening condition. In the other scenarios, there is no Rh incompatibility, and therefore, no risk of hemolytic disease of the newborn.

Question 20

A 1-year-old female is diagnosed with anemia secondary to insufficient erythropoiesis. The most likely cause is:

Accepted Answer

An iron deficiency is the most common cause of anemia in young children, as they have a high demand for iron to support their rapid growth and development. Insufficient iron intake, poor iron absorption, or iron loss due to chronic blood loss may contribute to this deficiency. Genetic factors, hemoglobin abnormalities, and erythrocyte structural abnormalities are less common causes of anemia in young children.

Quiz 21: Alterations of Hematologic Function in Children

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, resulting in a(n) :