guangyu
Forum Elite
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17 year boy have moderate upper abdominal pain for 24 hours. jaundice. T 37.9, P 90, R 14, BP 110/60. Exam shows right upper quadrant tenderness. spleen is palpated 3 cm below left costal margin. Lab:
hematocrit 32%
leukocyte 9800
MCV 78
total bilirubin 4.1
direct bilirubin 306
blood smear show spherocytes. ultrosonography show gallstone and splenomegaly. which is underlying mechanism?
1. activation of factor 12
2. cytoskeletal protein deficiency
3. defective iron absorption
4. persence of IgM antibody
5. pyruvate kinase deficiency
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| guangyu
Forum Elite
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i have no idea about this question
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| hir
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2...spectrin deficiency????
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| guangyu
Forum Elite
Topics: 28
Posts: 281
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direct bilirubin 3.6
why increase direct bilirubin instead of undirect?
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| grechita12
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2 [font style="background-color: #00ffff"]blood smear show spherocytes. ultrosonography show gallstone and splenomegaly. which is underlying mechanism? [/font]
[font style="background-color: #ffffff"]THIS MEANS HEMOLYSIS
[/font]
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| Pioglitazone
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Thanx for correcting the value! Now, I'll side with B
Reason:
Spherocytes = Hereditary spherocytosis
Why Direct Bilirubin is raised?
This guy had repeated hemolytic episodes, he developed cholesterol stones, which led to obstructive jaundice. At the moment, he presents with cholestatic jaundice (Acute cholecystitis)
Splenomegaly is also due to the repeated hemolytic episodes.
Thats my two cents!!
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| nyimalay
Forum Elite
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2. cytoskeletal protein deficiency
Heriditary Spnerocytosis due to Spectrin deficiency.
Chronic haemolysis leads to formation of pigment stones and now the boy came with obstructive jaundice.
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| chemamr
Moderator and PGY1
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agree
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| guangyu
Forum Elite
Topics: 28
Posts: 281
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thanks
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| GOGETA
I PASS step 1
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I think I saw this question in NBME1 if that is the case please next time, make a little note in the heading stating that is from a NBME exam.
I go with 2 also.
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