Deck 27: Alterations in Hematologic Function in Children

Question 1

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

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

Answer 1

60 to 80;120

Question 2

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.

Answer 2

iron deficiency.

Question 3

Hemolytic disease of the newborn can occur only if antigens on fetal erythrocytes differ from antigens on maternal erythrocytes.

Answer 3

True

Question 4

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

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

Question 5

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

Question 6

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

Question 7

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

Question 8

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

Question 9

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 10

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 11

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

Question 12

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

Question 13

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

Question 14

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

Question 15

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

A) Because the fetus does not have its own oxygen supply and must reply on oxygen from the maternal vascular system
B) Because the fetus has two-chains on the hemoglobin,rather than two ß-chains as in the adult
C) Because fetal hemoglobin interacts less readily with 2,3 diphosphoglycerate (2,3 DP),which inhibits hemoglobin-oxygen binding
D) Because fetal hemoglobin production occurs in the vessels and liver rather than in the bone marrow as in the adult

Question 16

Why does polycythemia occur in a fetus?

A) Because fetal hemoglobin has a greater affinity for oxygen due to 2,3 diphosphoglycerate (2,3 DP)
B) Because the fetus has a different hemoglobin structure of two- and two-chains rather than two- and two ß-chains
C) Because there is increased erythropoiesis in response to the hypoxic intrauterine environment
D) Because the lungs of the fetus are undeveloped and unable to adequately diffuse oxygen to the pulmonary capillaries

Question 17

How does hemolytic disease of the newborn (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' immune system produces antibodies against fetal erythrocytes,which are recognized as foreign and removed from circulation.

Question 18

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 19

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

Question 20

Hyperdiploidy is associated with a poor prognosis in leukemia.

Question 21

Hemophilia B is caused by clotting factor _____ deficiency.

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

Question 22

_____ is an autosomal dominant inherited hemorrhagic disease.

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

Question 23

What is the life span of an erythrocyte in sickle cell anemia?

A) 10 to 20 days
B) 40 to 60 days
C) 80 to 100 days
D) 100 to 120 days

Question 24

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 25

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 26

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 27

Which kind of anemia is occurs in thalassemia?

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

Question 28

Hemophilia A is inherited in an _____ fashion.

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

Question 29

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

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

Question 30

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 31

What are characteristics of ß-thalassemia major?

A) It is a heterozygous form of thalassemiA.
B) Its defect is the uncoupling of- and ß-chain synthesis.
C) It is a fatal condition in which all four ß-forming genes are defective.
D) It develops hemoglobin H when three genes are defective.

Question 32

What does glucose 6-phosphate dehydrogenase (G-6-PD)have in common with sickle cell disease?

A) Both are inherited X-linked recessive disorders.
B) Both are inherited autosomal recessive disorders.
C) Both disorders are initiated by hypoxemia and acidosis.
D) Both occur equally in males and females.

Question 33

How is glucose 6-phosphate dehydrogenase (G-6-PD)inherited?

A) As an X-linked dominant disorder
B) As an X-linked recessive disorder
C) As an autosomal dominant disorder
D) As an autosomal recessive disorder

Question 34

How is sickle cell trait different from sickle cell disease?

A) The child with sickle cell trait inherited normal hemoglobin A from one parent and hemoglobin S (Hb S)from the other parent,whereas the child with sickle cell disease has HbS from both parents.
B) The child with sickle cell trait 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) The child with sickle cell trait has a milder form of the disease that is characterized by vaso-occlusive crises and believed to result from higher hemoglobin values and viscosity.
D) The child with sickle cell trait has the mildest form of the disease with normal hemoglobin and hemoglobin F,which prevents sickling.

Question 35

Sickle cell disease is inherited in an _____ fashion.

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

Question 36

How is hemoglobin S (HbS)formed in sickle cell disease?

A) By a deficiency in glucose 6-phosphate dehydrogenase (G-6-PD)that changes hemoglobin A to hemoglobin S
B) By a genetic mutation in which two amino acids (histidine and leucine)are missing
C) By a genetic mutation in which one amino acid (valine)is replaced by another (glutamic acid)
D) By an autoimmune response in which one amino acid (proline)is detected as an antigen by abnormal immunoglobulin G (IgG)

Question 37

What are the manifestations of chronic sickle cell disease in children?

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 38

How is erythroblastosis fetalis defined?

A) An allergic disease in which maternal blood and fetal blood are antigenically incompatible
B) An alloimmune disease in which maternal blood and fetal blood are antigenically incompatible
C) An autoimmune disease in immature nucleated cells are released into the bloodstream
D) An autosomal dominant hereditary disease

Question 39

Hemolytic disease of the newborn can occur if:

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

Question 40

The
- and ß-thalassemias are inherited in an _____ fashion.

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

Question 41

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 42

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

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

Question 43

Match the type of hemophilia with its characteristics.(Terms can 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 44

Match the type of hemophilia with its characteristics.(Terms can 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

Question 45

Match the type of hemophilia with its characteristics.(Terms can 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 46

MATCHING
Match the sickle cell crises with its description.(Terms can 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 47

MATCHING
Match the sickle cell crises with its description.(Terms can 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 48

MATCHING
Match the sickle cell crises with its description.(Terms can 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 49

Match the type of hemophilia with its characteristics.(Terms can 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 50

MATCHING
Match the sickle cell crises with its description.(Terms can be used more than once. )
Vasospasm occurs,halting blood flow through vessels

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

Question 51

MATCHING
Match the sickle cell crises with its description.(Terms can 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