 繼續教育考題
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1.
(D) |
What is the cause of
anemia in this patient? |
| A | Iron deficiency anemia |
| B | Vit B12 deficiency |
| C | Folic acid deficiency |
| D | Hemolytic anemia |
2.
(D) |
Which evidence below is helpful in the
diagnosis of the cause of anemia in this patient?
(1) Hemoglobinuria (2) High LDH (3) low haptoglobin (4)
Coombs' test (5) reticulocytosis |
| A | (1), (2), (5) |
| B | (2), (3), (4), (5) |
| C | (1), (2), (3), (4) |
| D | (1), (2), (3), (4), (5) |
3.
(D) |
This patient had
hypokalemia, hypophosphatemia, hypouricidemia, glycosuria and
generalized aminoaciduria. What is the most likely cause? |
| A | Acute tubular necrosis |
| B | Hepatorenal syndrome |
| C | Distal renal tubular
dysfunction |
| D | Proximal renal tubular
dysfunction |
4.
(B) |
The initial symptoms and
signs of unexplained liver disorder in this patient led to the
suspicion of Wilson's disease. What is important diagnostic
criterion for Wilson's disease EXCEPT |
| A | Decreased incorporation of
isotopic copper into ceruloplasmin |
| B | Low 24-hour urine copper
excretion (less than 100 μg) |
| C | Low serum ceruloplasmin (less
than 20 mg/dl) |
| D | High copper concentration in
liver tissue (greater than 250 μg/g dry weight) |
5.
(D) |
Wilson's disease is
characterized by deposition of excessive copper in organs leading to
organ damage. Which organ is LESS frequently involved in this
disease? |
| A | Liver |
| B | Brain |
| C | Kidney |
| D | Pancreas |
6.
(A) |
In Wilson's disease,
what is the most frequent site of damage in the brain? |
| A | Lenticular nuclei |
| B | Thalamus |
| C | Cerebellum |
| D | Cerebral cortex |
7.
(C) |
Kayser-Fleischer ring
is formed by copper deposition in cornea. Which statement below is
WRONG? |
| A | This ring may also be present
in patients with chronic cholestatic liver disease such as primary
biliary cirrhosis |
| B | The presence of this ring in
Wilson's disease is best correlated with presence of
neuro-psychiatric symptoms |
| C | Whenever formed, it would
become permanent and would not fade or disappear even after
successful treatment |
| D | Slit-lamp examination is the
most sensitive method to demonstrate the lesion |
8.
(A) |
Wilson's disease is a
genetic disorder of autosomal recessive trait. The gene for this
disease was identified in 1993. Which statement about this gene is
CORRECT? |
| A | This gene encodes for a
membrane-ATPase located in Golgi-endosomal compartments of
hepatocytes. It transfers copper to the biliary secretory pathway.
Defects of this protein would impede excretion of copper into
bile. |
| B | This gene encodes for
ceruloplamin. Ceruloplamin is the most important copper-
transporting protein in the serum. Deficiency of serum ceruloplamin
results in inefficient movement of copper for excretion leading to
accumulation of copper in tissues. |
| C | This gene encodes for a cell
membrane-bound carrier protein responsible for transporting copper
outward cells. Defects of this protein would result in accumulation
of excessive copper in cells leading to cell injury and death. |
| D | Mutations of this gene in
Wilson's disease are clustered into a few forms. Thus, diagnosis of
this genetic disorder by DNA studies is easy. |
9.
(C) |
The following
statements about ceruloplasmin are correct EXCEPT |
| A | Serum ceruloplasmin levels do
not correlate with disease duration and severity |
| B | Ceruloplasmin is one of the
acute- phase proteins produced in hepatocytes |
| C | Copper is incorporated into
ceruloplasmin in the circulation |
| D | In Wilson's disease, copper
incorporation into ceruloplasmin is decreased |
10.
(C) |
About the treatment of
Wilson's disease, which statement below is WRONG? |
| A | Treatment must be instituted
once the diagnosis is established whether the patient is ill or
asymptomatic. |
| B | Penicillamine is administered
orally in an initial dose of 1g daily in a single or divided doses
at least 30 min before and 2h after meal. |
| C | Penicillamine should not be
discontinued even if rash, fever, leukopenia, thrombocytopenia,
lymphadenopathy, or proteinuria developed. |
| D | Liver transplantation is
indicated for fulminant hepatic failure and decompensated cirrhosis
with end-stage liver
disease |
Case Discussion