1. A patient who suffers from congenital erythropoietic porphyria has skin photosensitivity. The accumulation of what compound in the skin cells can cause it?
A. *Uroporphyrinogen I
B. Uroporphyrinogen II
C. Protoporphyrin
D. Coproporphyrinogen III
E. Heme
2. A denaturation of proteins can be found in some substances. Specify the substance that is used for the incomplete denaturation of hemoglobin:
A. Sulfuric acid
B. Nitric acid
C. Toluene
D. Sodium hydroxide
E. *Urea
3. A patient is ill with diabetes mellitus that is accompanied with hyperglycemia of over 7,2 millimole/L on an empty stomach. The level of what blood plasma protein can estimate the hyperglycemia rate retrospectively {4-8 weeks before examination}?
A. C-reactive protein
B. Ceruloplasmin
C. Fibrinogen
D. Albumin
E. *Glycosylated haemoglobin
4. Examination of initial molecular structure revealed substitution of the glutamic acid by valine. What inherited pathology is it typical for?
A. Minkowsky-Shauffard disease
B. Hemoglobinosis
C. Thalassemia
D. Favism
E. *Sickle-cell anemia
5. A 48 y.o. patient was admitted to the hospital with complaints about weakness, irritability, sleep disturbance. Objectively: skin and scleras are yellow. In blood: conjugated bilirubin, cholamia. Feces are acholic. Urine is of dark colour (bilirubin). What jaundice is it?
A. Hemolytic
B. Gilbert’s syndrome
C. *Mechanic (obstractive)
D. Crigler-Najjar syndrome
E. Parenchymatous
6. A full-term newborn child has yellowish skin and mucous membranes. This might be probably caused by temporary deficiency of following enzyme:
A. *UDP glucoronyltransferase
B. Uridine transferase
C. Biliverdin reductase
D. Heme oxygenase
E. Heme synthetase
7. Blood analysis of a patient with jaundice reveals hyperbilirubinemia, increased concentration of bile acids in the blood plasma. There is no stercobolinogen in urine. What type of jaundice is it?
A. Hemolytic jaundice
B. Hepatocellular jaundice
C. Cythemolytic jaundice
D. Parenchymatous jaundice
E. Obstructive jaundice*
8. A patient presents with icteritiousness of skin, sclera and mucous membranes. Blood plasma total bilirubin content is increased, stercobilin is increased in feces, urobilin is increased in the urine of this patient. What type of jaundice is this one:
A. Obturational
B. Gilbert`s disease
C. Hemolytic*
D. Cholestatic
E. Parenchymatous
9. Jaundice treatment involves administration of barbiturates inducing the synthesis of UDP-glucuronyl transferase. Effects of barbiturates cause the production of :
A. Protoporphyrin
B. Indirect (unconjugated) bilirubin
C. Heme
D. Biliverdin
E. Direct (conjugated) bilirubin*
10. A 48-year-old patient was admitted to the hospital with complaints about weakness, irritability, sleep disturbance. Objectively: skin and scleras are of yellow colour. In blood: increased concentration of total bilirubin with prevailing direct bilirubin. The feces are acholic. The urine is dark (contains bile pigments). What type of jaundice is it?
A. Gilbert’s syndrome
B. Mechanic*
C. Crigler-Najjar syndrome
D. Haemolytic
E. Parenchmatous
11. A patient presents with icteritiousness of skin, scleras and mucous membranes. Blood plasma total bilirubin is increased, stercobilin is increased in feces, urobilin is increased in urine. What type of jaundice is it?
A. Gilbert’s disease
B. Cholestatic
C. Haemolytic*
D. Parenchymatous
E. Obturational
12. A mother consulted a doctor about her 5-year-old child who develops erythemas, vesicular rash and skin itch under the influence of sun. Laboratory studies revealed decreased iron concentration in the blood serum, increased uroporphyrinogen I excretion with the urine. What is the most likely inherited pathology in this child?
A. Intermittent porphyria
B. Coproporphyria
C. Hepatic porphyria
D. Methemoglobinemia
E. Erythropoietic porphyria*
13. Hemoglobin catabolism results in release of iron which is transported to the bone marrow by a certain transfer protein and used again for the synthesis of hemoglobin. Specify this transfer protein:
A. Albumin
B. Ceruloplasmin
C. Haptoglobin
D. Transferrin (siderophilin) *
E. Transcobalamin
14. Enzymatic jaundices are accompanied by abnormal activity of UDP-glucuronyltransferase. What compound is accumulated in blood serum in case of these pathologies?
Unconjugated bilirubin *
Dehydrobilirubin
Conjugated bilirubin
Choleglobin
Hydrobilirubin
15. A patient with jaundice has high bilirubin that is mainly indirect (unconjugated), high concentration of stercobilin in the stool and urine. The level of direct (conjugated) bilirubin in the blood plasma is normal. What kind of jaundice can you think about?
Neonatal jaundice
Gilbert’s disease
Parenchymal (hepatic)
Mechanical
Hemolytic *
16. Patients with erythropoietic porphyria (Gunther’s disease) have teeth that fluoresce with bright red color when subjected to ultraviolet radiation; their skin is light-sensitive, urine is red-colored. What enzyme can cause this disease, when it is deficient?
Ferrochelatase
Uroporphyrinogen decarbozylase
Uroporphyrinogen I synthase
Delta-aminolevulinate synthase
*Uroporphyrinogen III cosynthase
17. Along with normal hemoglobin types there can be pathological ones in the organism of an adult. Name one of them;
HbS*
HbA1
HbA2
HbO2
HbF
18. A 16-year-old adolescent is diagnosed with hereditary UDP--glucuronyltransferase deficiency. Laboratory tests revealed hyperbilirubinemia caused mostly by increase blood content of the following substance: