Chapter 23 Flashcards
What is the etiology of the lipid storage diseases?
A) Caused by various enzyme defects (inborn errors) in lipid metabolism
B) Alteration to the multipotential stem cell
C) Red cell membrane defect
D) Hemoglobin defect
E) None of the above
A) Caused by various enzyme defects (inborn errors) in lipid metabolism
Which of the following lipid storage diseases has increased incidence in the Jewish ethnic group? A) Gaucher's B) Tay–Sachs C) Niemann–Pick D) All of the above E) None of the above
D) All of the above
The aim of control in the lipid storage diseases is directed at:
A) Controlling anemia
B) Prenatal detection
C) Correcting bone malformation
D) Dietary nutrition to lower cholesterol
E) Hyperlipidemia
B) Prenatal detection
Gaucher's disease is due to a deficiency of the enzyme \_\_\_\_\_\_\_\_\_\_. A) Sphingomyelinase B) Hexosaminidase A C) Beta-Glucocerebrosidase D) Sulfaminidase E) None of the above
C) Beta-Glucocerebrosidase
What substance is present in excess in Gaucher cells? A) Metabolized glucocerebroside B) Unmetabolized glucocerebroside C) Metabolized hexosaminidase D) Unmetabolized hexosaminidase E) None of the above
B) Unmetabolized glucocerebroside
Type I Gaucher's disease comprises the \_\_\_\_\_\_\_\_\_\_ form. A) Adult or non-neuronopathic B) Infantile acute neuronopathic C) Infantile malignant neuronopathic D) Juvenile neuronopathic E) None of the above
A) Adult or non-neuronopathic
Type II Gaucher's disease comprises the \_\_\_\_\_\_\_\_\_\_ form. A) Adult or non-neuronopathic B) Infantile acute neuronopathic C) Juvenile neuronopathic D) Subacute neuronopathic E) None of the above
B) Infantile acute neuronopathic
Type III Gaucher's disease comprises the \_\_\_\_\_\_\_\_\_\_ form. A) Adult B) Non-neuronopathic C) Acute neuronopathic D) Subacute neuronopathic E) None of the above
D) Subacute neuronopathic
All of the following are characteristic of the three types of Gaucher's disease except: A) Hepatosplenomegaly B) Gaucher cells present in bone marrow C) Increased serum acid phosphatase D) Hypercalcemia E) None of the above
D) Hypercalcemia
What is the most common type of Gaucher's disease? A) Adult B) Infantile C) Juvenile D) Subacute E) None of the above
A) Adult
Which of the following is a characteristic of type II Gaucher’s disease:
A) Occurs in infancy
B) Neurologic involvement
C) Hepatosplenomegaly
D) A much rarer form of Gaucher’s disease
E) All of the above
E) All of the above
Which sites are most frequently examined in patients with Gaucher's disease? A) Peripheral blood B) Bone marrow C) Spleen D) All of the above E) None of the above
D) All of the above
Which of the following are hematologic findings in Gaucher’s disease?
A) Normocytic normochromic anemia
B) Decreased iron in erythroid precursors
C) Leukopenia
D) Thrombocytopenia
E) All of the above
E) All of the above
What are the morphologic characteristics of a Gaucher cell?
A) 20–100 µm in diameter
B) A round to oval nuclei
C) Cytoplasm faintly blue
D) Cytoplasm has a “crumpled tissue paper” appearance
E) All of the above
E) All of the above
Gaucher cells are which type of cells? A) Megakaryocytes B) Erythrocytes C) Histiocytes D) Macrophages E) None of the above
C) Histiocytes