База тестів крок-1 з патофізіології (пмк-1)



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Test 197

A 45-year-old male presents with severe pain in both knee joints. At the time of surgery, his cartilage is found to have a dark blue-black color. Further evaluation reveals that the patient’s urine has darkened rapidly with time. The most likely diagnosis for this abnormality is



  1. Hyperbilirubinemia

  2. Tyrosinemia

  3. Albinism

  4. Alkaptonuria

  5. Gout


Test 198

An apathetic male infant in an underdeveloped country is found to have peripheral edema, a “moon” face, and an enlarged, fatty liver. Which one of the listed mechanisms is involved in the pathogenesis of this child’s abnormalities?



  1. Decreased protein intake leads to decreased lipoproteins

  2. Decreased caloric intake leads to hypoalbuminemia

  3. Decreased carbohydrate intake leads to hypoglycemia

  4. Decreased fluid intake leads to hypernatremia

  5. Decreased fat absorption leads to hypovitaminosis


Test 199

А 65 year-old man consults a doctor about strong pain in his great toes. He loves and often drinks beer. There is a suspicion on gout. Which of the followings substanses is necessary to identify in the blood for confirmation of a diagnosis?



  1. Urea

  2. Bilirubin

  3. Ketonic bodies

  4. Uric acid

  5. Cholesterol


Test 200

A 78 year-old man consults a doctor with complaints of pain in small joints of feet and hands. The joints are enlarged with thickened nodes. Urates in the serum of his blood are revealed. A disorder in metabolism of which of the following substances underlines this disorder?



  1. Pirimidins

  2. Amino acids

  3. Purines

  4. Lipids

  5. Carbonhydrates

Test 201

On examination of a 20-year old pregnant woman increased content of phenylalanine is revealed in her blood. Which of the following disorders is expected to be in her child?



  1. Mental retardation

  2. Galactosemia

  3. Turner’s syndrome

  4. Wilson’s disease

  5. Down syndrome

Test 202

Homogentisic acid is revealed in the urine of a 3-month old child. As a result of standing at air the urine becomes dark. Which of the following disorders is this finding characteristic for?



  1. Albinism

  2. Aminoaciduria

  3. Cystinuria

  4. Alkaptonuria

  5. Phenylketonuria


Test

It is known that phenylketonuria occurs as a result of a mutation in the gene which encodes for metabolism of amino acid phenylalanine. Indicate the disorder of phenylalanine metabolism that underlines phenylketonuria.



  1. phenylalanine  tyrosine  thyroxin

  2. phenylalanine  thyroxin  noradrenalin

  3. phenylalanine  thyroxin  alkapton

  4. phenylalanine  phenylpyruvat  ketoacids

  5. phenylalanine  tyrosine  melaline


Test 202

A 2-month old boy has dark spots on his sclera and ears. His urine becomes dark while standing on air. In his blood and the urine homogentisic acid is found out. Which of the following disorders does the patient have?



  1. Azotemia

  2. Bilirubinemia

  3. Albinism

  4. Alkaptonuria

  5. Phenilketonuria



Test 204

On examination alkaptonuria is identified in a newborn. Which of the following enzymes hereditary deficiency does this disorder result from?



  1. G-6-PDG

  2. Oxidase of homogentisic acid

  3. Phenilalaninehydroxilase

  4. Tyrosinase

  5. Monoaminoxidase


Test 205

In a month after a surgical removal of a large segment of the small intestine blood protein content in the patient is 35 g/L. Which of the following disorders in blood proteins does he have?



  1. Dysproteinemia

  2. Hyperproteinemia

  3. Paraproteinemia

  4. Hypoproteinemia

  5. Hypernitrogenemia


Test 206

A 6 –month old child develops mental and psychical retardation.His skin, hair and iris of eyes are pale.He has positive Felling’s test (with a 5% solution of trichloracetic iron). Which of the following inherited disorders does the patient have?



  1. Galactosemia

  2. Alkaptonuria

  3. Down desease

  4. Albinism

  5. Phenylketonuria





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