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Kaplan Qbank USMLE



Author7 Posts
  #1

A female infant with recurrent respiratory infections has no thymic shadow on chest X-ray. Serum immuneglubulin electrophoresis shows a low level of IgA but normal IgG level. Her ovaries are hypoplastic. Her serum calcium level is 10.2 mg/dL. Which of the following is the most likely answer?

A.Adenosin deaminase deficiency
B.Di George syndrom
C.Ataxia Teleangiectasia
D.Leukocyte adhesion deficiency
E.Acute lymphoblastic leukemia

  #2

Bwink

  #3

shaking head
try again

  #4

C.Ataxia Teleangiectasia

the reason its not DiGeorge Syndrome because her serum calcium levels are not in the hypocalcemic range.


___________________
FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."

  #5

yep, it's C.

we mustn't blindly fall for the absent thymic shadow trick anymore. they throw it in for a reason. [of course, don't ignore it if it's really DiGeorge! but use the other symptoms to arrive at the answer]


___________________
Prepare as if you're the worst, Perform as if you're the best! As you dream, so you manifest. So, DREAM BIG!! When you face hardship, remember, God never gives you more than you can handle. Keep your face to the sunshine and you cannot see the shadows.

  #6

Thymic shadow triggers a response which leads to DiGeorge normally but the hypoplastic ovaries and her serum calcium should have been the landmines or the alarms which should go off in your head while reading the question. And believe me when i say this that Serum Calcium was the biggest sign, signal or warning in this question and if your lucky will find questions in the real exam in which the warning is so subtle that you dont even know it. So Read and Think very carefully so that in the exam it doesnt slow you down.


___________________
FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."

  #7

nodnodnod
you guys are good...

The correct answer is C

This patient has thymic hypoplasia, depressed IgA, recurrent respiratory infections, hypoplastic ovaries and a normal serum calcium level. This fidings are consistent with ataxia teleangiectasia in an infant.
The normal calcium level differentieates this condition from Di George sy.
In AT: by age 2-3, cerebellar Purkinje cell degeneration causes ataxia. By age 4-6, the characteristic teleangiectasias appear on the face.









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