Deck 28: Alterations of Hematologic Function in Children

Question 1

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

Answer 1

5

Question 2

Which blood cells are elevated at birth, but decrease to adult levels the first year of life?

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

Answer 2

Monocytes

Question 3

HDN can occur only if antigens on fetal erythrocytes differ from antigens on maternal erythrocytes.

Answer 3

True

Question 4

The most dramatic form of acquired congenital hemolytic anemia is hemolytic disease of the newborn, also called erythroblastosis fetalis.

Question 5

The most common cause of anemia from insufficient erythropoiesis in children is:

A)folic acid deficiency.
B)iron deficiency.
C)hemoglobin abnormality.
D)erythrocyte abnormality.

Question 6

Hyperdiploidy is associated with a poor prognosis in leukemia.

Question 7

In a developing embryo, erythrocyte production shifts from the vessel to the liver until the fifth month of gestation, when hematopoiesis begins to occur in the bone marrow.

Question 8

Hemoglobin in a fetus has less affinity for oxygen than hemoglobin in an adult.

Question 9

Between 4 years of age and the onset of puberty, dietary iron deficiency is common.

Question 10

In beta-thalassemia major, most erythroblasts are destroyed in the spleen.

Question 11

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 12

Sickle cell crisis can be prevented by avoiding fever, infection, acidosis, dehydration, constricting clothing, and exposure to cold.

Question 13

How does HDN cause acquired congenital hemolytic anemia?

A)HDN develops when hypoxia or dehydration cause 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 14

Rh incompatibility occurs in less than 10% of pregnancies and rarely causes hemolytic disease of the newborn in the first incompatible fetus.

Question 15

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

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

Question 16

Sickled erythrocytes (characteristic of sickle cell anemia) are stiff and cannot change shape as easily as normal erythrocytes and thus tend to plug the microcirculation.

Question 17

Fetal hemoglobin has a greater affinity for oxygen than adult hemoglobin because:

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 γ-chains on the hemoglobin, rather than two ß-chains as in the adult.
C)fetal hemoglobin interacts less readily with 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.

Question 18

Polycythemia occurs in a fetus because:

A)fetal hemoglobin has a greater affinity for oxygen due to DPG.
B)the fetus has a different hemoglobin structure of two α- and two γ-chains rather than two α-and two β-chains.
C)there is increased erythropoiesis in response to the hypoxic intrauterine environment.
D)the lungs of the fetus are undeveloped and unable to adequately diffuse oxygen to the pulmonary capillaries.

Question 19

Sickle cell trait may provide protection against the lethal form of malaria.

Question 20

Using the prepregnancy sickle test, fertilization produces several embryos that are tested for the sickle gene.An embryo without the gene is implanted in the mother and amniocentesis confirms whether the fetus has the sickle cell gene.

Question 21

The type of anemia that occurs as a result of thalassemia is:

A)microcytic, hypochromic.
B)microcytic, normochromic.
C)macrocytic, hyperchromic.
D)macrocytic, normochromic.

Question 22

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 23

G6PD and sickle cell disease are:

A)inherited X-linked recessive disorders.
B)inherited autosomal recessive disorders.
C)disorders initiated by hypoxemia and acidosis.
D)diagnosed equally in men and women.

Question 24

Hemophilia A is inherited in an _____ fashion.

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

Question 25

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune process involving antibodies against:

A)neutrophils.
B)eosinophils.
C)platelets.
D)basophils.

Question 26

The sickle cell trait differs from sickle cell disease in that the child with sickle cell trait:

A)inherited normal hemoglobin A from one parent and Hb S from the other parent, whereas the child with sickle cell disease has Hb S from both parents.
B)has a mild form of sickle cell disease that causes sickling during fever and infection, but not during acidosis or hypoxia, whereas the child with sickle cells disease develops sickling during each of these conditions.
C)has a milder form of the disease that is characterized by vaso-occlusive crises and is believed to result from higher hemoglobin values and viscosity.
D)has the mildest form of the disease with normal hemoglobin and hemoglobin F, which prevents sickling.

Question 27

The manifestations of chronic sickle cell disease in children include:

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 28

_____ is an autosomal dominant inherited hemorrhagic disease.

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

Question 29

Fetuses who do not survive anemia in utero are usually stillborn, with gross edema of the entire body, a condition called:

A)spherocytosis.
B)icterus gravis neonatorum.
C)erythroblastosis fetalis.
D)hydrops fetalis.

Question 30

Hemophilia B is caused by clotting factor _____ deficiency.

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

Question 31

Glucose 6-phosphate dehydrogenase (G6PD) is inherited as an _____ disorder.

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

Question 32

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

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

Question 33

There is a ___% chance with each pregnancy that a child born to two parents with sickle trait will have sickle cell disease.

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

Question 34

What prevents kernicterus?

A)Administration of intravenous fluids to dilute the blood and remove the bilirubin through the kidneys faster
B)Replacement transfusion of Rh-positive erythrocytes to prevent the deposit of bilirubin in the kidneys
C)Performance of a splenectomy to prevent the destruction of abnormal erythrocytes
D)Replacement transfusion of Rh-negative erythrocytes to prevent the deposit of bilirubin in the brain

Question 35

During HDN, the newborn develops hyperbilirubinemia after birth, but not in utero because:

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 36

Hemolytic disease of the newborn can occur if the mother is:

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

Question 37

Hemoglobin S (Hb S) is formed in sickle cell disease as a result of a(n):

A)deficiency in G6PD that changes Hb A to Hb S.
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 IgG.

Question 38

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

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

Question 39

Sickle cell disease (SCD) is inherited in an _____ fashion.

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

Question 40

Characteristics of beta-thalassemia major include:

A)a heterozygous form of thalassemia.
B)a defect in the uncoupling of α- and β-chain synthesis.
C)a fatal condition in which all four beta-forming genes are defective.
D)the development of Hb H when three genes are defective.

Question 41

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

A)Vaso-occlusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 42

Match the type of hemophilia with its characteristics. Terms may be used more than once.
Caused by clotting factor XI deficiency and an autosomal recessive trait

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

Question 43

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

A)Vaso-occlusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 44

Match the sickle cell crises with its description. Terms may be used more than once.
Occurs in association with certain drugs or infection

A)Vaso-occlusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 45

Match the type of hemophilia with its characteristics. Terms may be used more than once.
Caused by clotting factor IX deficiency and is an X-linked recessive trait

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

Question 46

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

A)Vaso-occlusive crisis
B)Aplastic crisis
C)Sequestration crisis
D)Hyperhemolytic crisis

Question 47

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

Question 48

Match the type of hemophilia with its characteristics. Terms may be used more than once.
Caused by clotting factor VIII deficiency and an X-linked recessive trait

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

Question 49

Match the type of hemophilia with its characteristics. Terms may be used more than once.
Caused by clotting factor VIII deficiency and an autosomal dominant trait

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