Question 1

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

Accepted Answer

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

Question 2

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

Accepted Answer

A) True 
 B)False

Question 3

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

Accepted Answer

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

Question 4

The manifestations of chronic sickle cell disease in children include:

Accepted Answer

A) atelectasis and pneumonia. 
B) edema of the hands and feet. 
C) stasis ulcers of the hands, ankles, and feet. 
D) splenomegaly and hepatomegaly. 
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

_____ is an autosomal dominant inherited hemorrhagic disease.

Accepted Answer

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

Question 6

G6PD and sickle cell disease are:

Accepted Answer

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. 
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 7

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

Accepted Answer

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

Question 8

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

Accepted Answer

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

Question 9

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

Accepted Answer

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. 
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.

Exam 28: Alterations of Hematologic Function in Children

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

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

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

The manifestations of chronic sickle cell disease in children include:

_____ is an autosomal dominant inherited hemorrhagic disease.

G6PD and sickle cell disease are:

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

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

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

Cellular Biology

Altered Cellular and Tissue Biology

The Cellular Environment: Fluids and Electrolytes, Acids and Bases

Genes and Genetic Diseases

Genes, Environment, and Common Diseases

Innate Immunity: Inflammation

Adaptive Immunity

Alterations in Immunity and Inflammation

Infection

Stress and Disease

Biology, Clinical Manifestations, and Treatment of Cancer

Cancer Epidemiology

Cancer in Children

Structure and Function of the Neurologic System

Pain, Temperature Regulation, Sleep, and Sensory Function

Alterations in Cognitive Systems, Cerebral Hemodynamics and Motor Function

Disorders of the Central and Peripheral Nervous Systems and the Neuromuscular Junction

Neurobiology of Schizophrenia, Mood Disorders, and Anxiety Disorders

Alterations of Neurologic Function in Children

Mechanisms of Hormonal Regulation

Alterations of Hormonal Regulation

Structure and Function of the Reproductive System

Alterations of the Reproductive Systems

Sexually Transmitted Infections

Structure and Function of the Hematologic System

Alterations of Erythrocyte Function

Alterations of Leukocyte, Lymphoid, and Hemostatic Function

Structure and Function of the Cardiovascular and Lymphatic Systems

Alterations of Cardiovascular Function

Alterations of Cardiovascular Function in Children

Structure and Function of the Pulmonary System

Alterations of Pulmonary Function

Alterations of Pulmonary Function in Children

Structure and Function of the Renal and Urologic Systems

Alterations of Renal and Urinary Tract Function

Alterations of Renal and Urinary Tract Function in Children

Structure and Function of the Digestive System

Alterations of Digestive Function

Alterations of Digestive Function in Children

Structure and Function of the Musculoskeletal System

Alterations of Musculoskeletal Function

Alterations of Musculoskeletal Function in Children

Structure, Function, and Disorders of the Integument

Alterations of the Integument in Children

Shock, Multiple Organ Dysfunction Syndrome, and Burns in Adult

Shock, Multiple Organ Dysfunction Syndrome, and Burns in Children

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