Deck 30: Alterations of Hematologic Function in Children

Question 1

Sickle cell disease is classified as a(an):

A) Inherited X-linked recessive disorder
B) Inherited autosomal recessive disorder
C) Disorder initiated by hypoxemia and acidosis
D) Disorder that is diagnosed equally in men and women

Answer 1

Disorder initiated by hypoxemia and acidosis

Question 2

In a full-term infant,the normal erythrocyte life span is _____ days,whereas the adult erythrocyte life span is _____ days.

A) 30 to 50; 80
B) 60 to 80; 120
C) 90 to 110; 140
D) 120 to 130; 150

Answer 2

60 to 80; 120

Question 3

Why does fetal hemoglobin have a greater affinity for oxygen than adult hemoglobin?

A) The fetus does not have its own oxygen supply and must rely on oxygen from the maternal vascular system.
B) The fetus has two g-chains on the hemoglobin, rather than two b-chains as in the adult.
C) Fetal hemoglobin interacts less readily with diphosphoglycerate (DPG), which inhibits hemoglobin-oxygen binding.
D) Fetal hemoglobin production occurs in the vessels and liver rather than in the bone marrow as in the adult.

Answer 3

Fetal hemoglobin interacts less readily with diphosphoglycerate (DPG), which inhibits hemoglobin-oxygen binding.

Question 4

Which manifestations of vasoocclusive crisis are associated with sickle cell disease (SCD)in infants?

A) Atelectasis and pneumonia
B) Edema of the hands and feet
C) Stasis ulcers of the hands, ankles, and feet
D) Splenomegaly and hepatomegaly

Question 5

Erythroblastosis fetalis is defined as an:

A) Allergic disease in which maternal blood and fetal blood are antigenically incompatible
B) Alloimmune disease in which maternal blood and fetal blood are antigenically incompatible
C) Autoimmune disease in immature nucleated cells that are released into the bloodstream
D) Autosomal dominant hereditary disease

Question 6

Glucose 6-phosphate dehydrogenase (G6PD)deficiency is what type of inherited disorder?

A) X-linked dominant
B) X-linked recessive
C) Autosomal dominant
D) Autosomal recessive

Question 7

What treatment prevents the development of kernicterus in an infant born with hemolytic disease of the newborn (HDN)?

A) Administration of intravenous fluids to dilute the blood
B) Replacement transfusion of new Rh-positive blood that is not contaminated with anti-Rh antibodies
C) Performance of a splenectomy to prevent the destruction of abnormal erythrocytes
D) Replacement transfusion of Rh-negative erythrocytes

Question 8

How does hemolytic disease of the newborn (HDN)cause acquired congenital hemolytic anemia?

A) HDN develops when hypoxia or dehydration causes the erythrocytes to change shapes, which are then recognized as foreign and removed from circulation.
B) HDN is an alloimmune disease in which the mother's immune system produces antibodies against fetal erythrocytes, which are recognized as foreign and removed from circulation.
C) HDN develops when the polycythemia present in fetal life continues after birth, causing the excessive number of erythrocytes to be removed from circulation.
D) HDN is an autoimmune disease in which the fetus's immune system produces antibodies against fetal erythrocytes, which are recognized as foreign and removed from circulation.

Question 9

What is the reason most children diagnosed with sickle cell anemia are not candidates for either bone marrow or stem cell transplants?

A) Well-matched stem cell donors are difficult to find.
B) The child is usually too weak to survive the procedure.
C) The child's immune system will not appropriately respond to the antirejection medications.
D) Although effective for adults, neither procedure has been proven effective for children.

Question 10

Sickle cell disease (SCD)is what type of inherited disorder?

A) Autosomal dominant
B) Autosomal recessive
C) X-linked dominant
D) X-linked recessive

Question 11

An infant's hemoglobin must fall below ___ g/dl before signs of pallor,tachycardia,and systolic murmurs occur.

A) 11
B) 9
C) 7
D) 5

Question 12

Which blood cell type is elevated at birth but decreases to adult levels during the first year of life?

A) Monocytes
B) Platelets
C) Neutrophils
D) Lymphocytes

Question 13

Hemolytic disease of the newborn (HDN)can occur if the mother:

A) Is Rh-positive and the fetus is Rh-negative
B) Is Rh-negative and the fetus is Rh-positive
C) Has type A blood and the fetus has type O
D) Has type AB blood and the fetus has type B

Question 14

Fetuses who do not survive anemia in utero are usually stillborn with gross edema of the entire body.Which term is used to identify this condition?

A) Spherocytosis
B) Icterus gravis neonatorum
C) Erythroblastosis fetalis
D) Hydrops fetalis

Question 15

What is the cause of polycythemia in the fetus?

A) Fetal hemoglobin has a greater affinity for oxygen as a result of diphosphoglycerate (DPG).
B) The fetus has a different hemoglobin structure of two a- and two g-chains rather than two a-and two b-chains.
C) Increased erythropoiesis occurs in response to the hypoxic intrauterine environment.
D) The lungs of the fetus are undeveloped and unable to diffuse oxygen adequately to the pulmonary capillaries.

Question 16

When diagnosed with hemolytic disease of the newborn (HDN),why does the newborn develop hyperbilirubinemia after birth but not in utero?

A) Excretion of unconjugated bilirubin through the placenta into the mother's circulation is no longer possible.
B) Hemoglobin does not break down into bilirubin in the intrauterine environment.
C) The liver of the fetus is too immature to conjugate bilirubin from a lipid-soluble to water-soluble form.
D) The destruction of erythrocytes producing bilirubin is greater after birth.

Question 17

Which vitamin improves the absorption of oral iron taken to treat iron deficiency anemia in children?

A) A
B) B
C) C
D) E

Question 18

Hemoglobin S (HbS)is formed in sickle cell disease as a result of which process?

A) Deficiency in glucose 6-phosphate dehydrogenase (G6PD) that changes hemoglobin A (HbA) to HbS.
B) Genetic mutation in which two amino acids (histidine and leucine) are missing.
C) Genetic mutation in which one amino acid (valine) is replaced by another (glutamic acid).
D) Autoimmune response in which one amino acid (proline) is detected as an antigen by abnormal immunoglobulin G (IgG).

Question 19

What is the name of the disorder in which levels of bilirubin remain excessively high in the newborn and are deposited in the brain?

A) Kernicterus
B) Icterus neonatorum
C) Jaundice
D) Icterus gravis neonatorum

Question 20

What is the most common cause of insufficient erythropoiesis in children?

A) Folic acid deficiency
B) Iron deficiency
C) Hemoglobin abnormality
D) Erythrocyte abnormality

Question 21

Which disease is caused by clotting factor VIII deficiency and is an autosomal dominant trait?

A) Hemophilia A
B) Hemophilia B
C) Hemophilia C
D) von Willebrand disease

Question 22

What is the chance with each pregnancy that a child born to two parents with the sickle trait will have sickle cell disease (SCD)?

A) 20%
B) 25%
C) 33%
D) 50%

Question 23

The alpha- and beta-thalassemias are considered what types of inherited disorder?

A) Autosomal recessive
B) Autosomal dominant
C) X-linked recessive
D) X-linked dominant

Question 24

Which type of hemophilia affects only men?

A) Hemophilia A
B) Hemophilia B
C) Hemophilia C
D) von Willebrand disease

Question 25

Match each sickle cell crisis with its description.Terms may be used more than once.
Crisis occurs in association with certain drugs or infection.

A)Vasoocclusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 26

Match each sickle cell crisis with its description.Terms may be used more than once.
Blood flow is impaired by tangled masses of rigid,sickled cells.

A)Vasoocclusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 27

What is the fundamental defect that results in beta-thalassemia major?

A) The spleen prematurely destroys the precipitate-carrying cells.
B) A severe uncoupling of a- and b-chain synthesis occurs.
C) All four beta-forming genes are defective.
D) Hemoglobin H (HbH) develops when three genes are defective.

Question 28

What is the significance of hyperdiploidy when diagnosing and treating leukemia?

A) Hyperdiploidy indicates a good prognosis.
B) Hyperdiploidy indicates a poor prognosis.
C) Hyperdiploidy indicates poor response to a specific treatment.
D) Hyperdiploidy indicates the achievement of remission.

Question 29

Hemophilia B is caused by a deficiency of which clotting factor?

A) V
B) VIII
C) IX
D) X

Question 30

Which hemophilia occurs equally in both men and women?

A) Hemophilia A
B) Hemophilia B
C) Hemophilia C
D) von Willebrand disease

Question 31

When does fetal erythrocyte production shift from the liver to the bone marrow?

A) Fourth month of gestation
B) Fifth month of gestation
C) Eighth month of gestation
D) At birth

Question 32

Which type of anemia occurs as a result of thalassemia?

A) Microcytic, hypochromic
B) Microcytic, normochromic
C) Macrocytic, hyperchromic
D) Macrocytic, normochromic

Question 33

Which disorder results in decreased erythrocytes and platelets with changes in leukocytes and has clinical manifestations of pallor,fatigue,petechiae,purpura,bleeding,and fever?

A) Idiopathic thrombocytopenic purpura (ITP)
B) Acute lymphocytic leukemia (ALL)
C) Non-Hodgkin lymphoma (NHL)
D) Iron deficiency anemia (IDA)

Question 34

During childhood,when is dietary iron deficiency commonly diagnosed?

A) Between 2 months and 1 year
B) Between 6 months and 2 years
C) Between 12 months and 3 years
D) Between 18 months and 4 years

Question 35

Idiopathic thrombocytopenic purpura (ITP)is an autoimmune process involving antibodies attacking which type of cells?

A) Neutrophils
B) Eosinophils
C) Platelets
D) Basophils

Question 36

Which disease is an autosomal dominant inherited hemorrhagic disease?

A) Hemophilia A
B) von Willebrand disease
C) Christmas disease
D) Hemophilia B

Question 37

Hemophilia A is considered to be what type of inherited disorder?

A) Autosomal dominant
B) Autosomal recessive
C) X-linked recessive
D) X-linked dominant

Question 38

Match each sickle cell crisis with its description.Terms may be used more than once.
Large amounts of blood become acutely pooled in the liver and spleen.

A)Vasoocclusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 39

Match each sickle cell crisis with its description.Terms may be used more than once.
Compensatory erythropoiesis is compromised,thus limiting the number of erythrocytes that are replaced.

A)Vasoocclusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 40

What are the common triggers for sickle cell crisis? (Select all that apply.)

A) Fever
B) Infection
C) Dehydration
D) Alkalosis
E) Exposure to the cold