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|Prompt=A 6 year old Nigerian boy presented by his mother to the physician for unexplained fever of two weeks duration.  Mother complains that her child has recent weight loss with no appetite.  She also notes he has excessive fatigue and malaise, associated with night sweats.  Patient’s vital signs reveal a temperature measuring 39o C (102 F), pulse measuring 98 beats per minute, blood pressure measuring 110/85 mmHg.  On physical examination, severe disruption of orofacial aspect and teeth was noted.  An image of a patient with the same presentation is shown below.  You explain to the mother that her child’s condition is best caused by a dysregulation of which of the following gene?
|Prompt=A 6 year old Nigerian boy presented by his mother to the physician for unexplained fever of two weeks duration.  Mother complains that her child has recent weight loss with no appetite.  She also notes he has excessive fatigue and malaise, associated with night sweats.  Patient’s vital signs reveal a temperature measuring 39o C (102 F), pulse measuring 98 beats per minute, blood pressure measuring 110/85 mmHg.  On physical examination, severe disruption of orofacial aspect and teeth was noted.  An image of a patient with the same presentation is shown below.  You explain to the mother that her child’s condition is best caused by a dysregulation of which of the following gene?


[[Image:Burkitt's_lymphoma_in_a_child.png|300px]]
[[Image:Burkitt's_lymphoma_in_a_child.png|center|250px]]
|Explanation=The endemic variant of [[Burkitt's lymphoma]] occurs in equatorial Africa.  It is the most common malignancy of children in this area.  Children affected with the disease often also had chronic malaria, which is believed to have reduced resistance to Epstein-Barr virus ([[EBV]]), allowing it to take hold.  The disease characteristically involves the jaw or other facial bone.  Mutation in c-myc gene is associated with Burkitt's lymphoma.
|Explanation=The endemic variant of [[Burkitt's lymphoma]] occurs in equatorial Africa.  It is the most common malignancy of children in this area.  Children affected with the disease often also had chronic malaria, which is believed to have reduced resistance to Epstein-Barr virus ([[EBV]]), allowing it to take hold.  The disease characteristically involves the jaw or other facial bone.  Mutation in c-myc gene is associated with Burkitt's lymphoma.



Revision as of 21:46, 2 September 2013

 
Author [[PageAuthor::Rim Halaby, M.D. [1]]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Hematology
Prompt [[Prompt::A 6 year old Nigerian boy presented by his mother to the physician for unexplained fever of two weeks duration. Mother complains that her child has recent weight loss with no appetite. She also notes he has excessive fatigue and malaise, associated with night sweats. Patient’s vital signs reveal a temperature measuring 39o C (102 F), pulse measuring 98 beats per minute, blood pressure measuring 110/85 mmHg. On physical examination, severe disruption of orofacial aspect and teeth was noted. An image of a patient with the same presentation is shown below. You explain to the mother that her child’s condition is best caused by a dysregulation of which of the following gene?
]]
Answer A AnswerA::C-kit
Answer A Explanation [[AnswerAExp::C-kit oncogene is associated with GIST.]]
Answer B AnswerB::Ret
Answer B Explanation [[AnswerBExp::Ret oncogene is associated with MEN syndrome type IIa and IIb.]]
Answer C AnswerC::Abl
Answer C Explanation [[AnswerCExp::Abl oncogene is associated with CML.]]
Answer D AnswerD::Ras
Answer D Explanation [[AnswerDExp::Ras oncogene is associated with colon carcinoma.]]
Answer E AnswerE::C-myc
Answer E Explanation [[AnswerEExp::C-myc is associated with Burkitt's lymphoma.]]
Right Answer RightAnswer::E
Explanation [[Explanation::The endemic variant of Burkitt's lymphoma occurs in equatorial Africa. It is the most common malignancy of children in this area. Children affected with the disease often also had chronic malaria, which is believed to have reduced resistance to Epstein-Barr virus (EBV), allowing it to take hold. The disease characteristically involves the jaw or other facial bone. Mutation in c-myc gene is associated with Burkitt's lymphoma.

Educational objective: Mutation in c-myc proto-oncogene is associated with Burkitt's lymphoma.
Educational Objective:
References: ]]

Approved Approved::No
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