hemophilia a or b

[arda]

A 2-year-old boy with normal size, weight, and development is brought to the physician because of a painful, swollen left knee for the past week. He has had two episodes of asymptomatic hematuria during the past 6 months. Both parents are healthy. He has achieved developmental milestones at appropriate ages. His height and weight are at the 70th percentile. Examination shows bruises and resolving hematomas on both elbows and signs of cutaneous hemorrhages at the site of a deep splinter. Laboratory studies show:

Hemoglobin 12 g/dL
Erythrocyte count normal
Leukocyte count normal
Bleeding time normal
Partial thromboplastin time increased

The most likely diagnosis can be proven by the therapeutic response to a single injection of which of the following materials?

A. Factor I (fibrinogen)
B. Factor VIII (antihemophilic factor)
C. Factor IX (plasma thromboplastin component)
D. Folic acid
E. Vitamin K

What is the dx ?

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[wonderbread12]

That's kind of weird that they give you both as choices but may want you to just know that Hemophilia A is more common

 

[EazyE1907]

the key thing to notice here is that "Painful swollen knee for the past week" = macrohemorrhage. then you can easily narrow it down to B or C and 9 out of 10 times it will be hemophilia A because as mentioned above it is more common. the injection of either factor would normalize PTT so yeah i think they are just trying to throw you off by giving hemp B as an option.

 

[J ROD]

That is another not so great question.

 

[EazyE1907]

That is another not so great question.

yeah i think its from nbme 4. figures!

 

[EazyE1907]

how about this one

43 yr old white male. complains of fatigue, dry mouth, and impotence. pe reveals hepatomegaly and atrophic testes. fasting blood glucose is 252. urine positive for glucose but negative for ketones and protein. he also says that he developed a strange tan despite spending alsmot all his time indoors. which of the following is most likely responsible for the skin changes.

high serum ACTH
dermal hemosiderin deposition
abnormal melanocyte migration
impaired tyrosine metabolism
reduced serum copper content

 

[arda]

how about this one

43 yr old white male. complains of fatigue, dry mouth, and impotence. pe reveals hepatomegaly and atrophic testes. fasting blood glucose is 252. urine positive for glucose but negative for ketones and protein. he also says that he developed a strange tan despite spending alsmot all his time indoors. which of the following is most likely responsible for the skin changes.

high serum ACTH
dermal hemosiderin deposition
abnormal melanocyte migration
impaired tyrosine metabolism
reduced serum copper content

Is it b ? hereditary hemochromatosis ?

Bronze diabetes,impotance

 

[J ROD]

Nice call. I agree that it be "B"

 

[EazyE1907]

yupp! i picked A like an idiot tho. surprisingly the majority of the ppl picked A like me lol.

 

[EazyE1907]

i talked myself out of B i have to stop doing that.

 

[J ROD]

A looks right but it did not fit all the criteria. It was my first thought but it just did not add up all the pieces.

 

[EazyE1907]

yeah i wanted to drop kick myself in the face when i read the explanation. super easy. oh well. it's all a learning experience.

 

[arda]

was it an UW question ? i do not remember it.