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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}, {{AJL}} {{Alison}}
|QuestionAuthor={{Rim}} (Reviewed by {{YD}})
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Pathology
|MainCategory=Pathology
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|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Hematology
|SubCategory=Hematology
|MainCategory=Pathology
|MainCategory=Pathology
|MainCategory=Pathology
|MainCategory=Pathology
|MainCategory=Pathology
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|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Hematology
|SubCategory=Hematology
|Prompt=A 6-year-old Nigerian male, brought by his mother, presents to the physician's office for an unexplained fever of two weeks duration. The mother complains that her child has experienced recent weight and appetite loss, excessive fatigue, and malaise, associated with night sweats. The patient’s vital signs reveal a temperature of 39 °C, pulse measuring 98 beats per minute, and blood pressure measuring 110/85 mmHg. Upon physical examination, you note the severe disruption of orofacial aspect and teeth.  An image of the patient is displayed below. The child’s condition is most likely caused by a dysregulation of which of the following gene?
|Prompt=A 6-year-old Nigerian boy is brought to the physician's office for an unexplained fever of two weeks duration. The mother explains that her child has experienced recent weight loss and night sweats that are associated with excessive fatigue, malaise, and anorexia. The family has recently immigrated from Nigeria, and the patient has not received previous medical care. The patient’s temperature is 39 °C (102.2 °F), blood pressure is 110/85 mmHg, and heart rate is 98/min. Physical examination is remarkable for severe orofacial and teeth disruption (shown below). The child’s condition is caused by a dysregulation of which gene?
 
[[Image:Burkitt's_lymphoma_in_a_child.png|center|250px]]
|Explanation=The endemic variant of [[Burkitt's lymphoma]], which frequently occurs in equatorial Africa, is the most common malignancy the African children. Children with the disease often also experience chronic malaria, which is believed to result in a reduced resistance to Epstein-Barr virus ([[EBV]]).  The disease characteristically involves the jaw or other facial bones.  Mutation in the c-myc gene is frequently associated with Burkitt's lymphoma.


|EducationalObjectives= Mutation in the [[c-myc]] proto-oncogene is associated with [[Burkitt's lymphoma]].
[[Image:Burkitt's_lymphoma_in_a_child.png||250px]]
|References= First Aid 2014 page 232
|AnswerA=L-MYC
|AnswerA=C-kit
|AnswerAExp=The ''L-MYC'' oncogene is associated with lung cancers.
|AnswerAExp= The [[C-kit]] oncogene is associated with [[GIST]].
|AnswerB=''RET''
|AnswerB=Ret
|AnswerBExp=The ''[[RET]]'' oncogene is associated with [[multiple endocrine neoplasia]] (MEN) syndromes type 2A and 2B.
|AnswerBExp= The [[Ret gene|Ret oncogene]] is associated with [[MEN|MEN syndrome]] type IIa and IIb.
|AnswerC=''N-MYC''
|AnswerC=Abl
|AnswerCExp=''[[N-MYC]]'' oncogene is associated with neuroblastoma.
|AnswerCExp= The [[Abl gene|Abl oncogene]] is associated with CML.
|AnswerD=''HER2''
|AnswerD=Ras
|AnswerDExp=''[[HER2]]'' oncogene is associated with breast, ovarian, and gastric cancers.
|AnswerDExp= The [[Ras]] oncogene is associated with colon carcinoma.
|AnswerE=''C-MYC''
|AnswerE=C-myc
|AnswerEExp=''[[C-MYC]]'' oncogene is associated with [[Burkitt's lymphoma]].
|AnswerEExp= The [[C-myc]] is associated with [[Burkitt's lymphoma]].
|References=First Aid 2014 page 232
|RightAnswer=E
|RightAnswer=E
|WBRKeyword=Burkitt's lymphoma, C-myc, Oncogene, Hematologic malignancy, Fever, Constitutional symptoms
|Approved=Yes
|Approved=Yes
}}
}}

Revision as of 22:21, 7 November 2014

 
Author [[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Hematology
Prompt [[Prompt::A 6-year-old Nigerian boy is brought to the physician's office for an unexplained fever of two weeks duration. The mother explains that her child has experienced recent weight loss and night sweats that are associated with excessive fatigue, malaise, and anorexia. The family has recently immigrated from Nigeria, and the patient has not received previous medical care. The patient’s temperature is 39 °C (102.2 °F), blood pressure is 110/85 mmHg, and heart rate is 98/min. Physical examination is remarkable for severe orofacial and teeth disruption (shown below). The child’s condition is caused by a dysregulation of which gene?

]]

Answer A AnswerA::L-MYC
Answer A Explanation AnswerAExp::The ''L-MYC'' oncogene is associated with lung cancers.
Answer B AnswerB::''RET''
Answer B Explanation [[AnswerBExp::The RET oncogene is associated with multiple endocrine neoplasia (MEN) syndromes type 2A and 2B.]]
Answer C AnswerC::''N-MYC''
Answer C Explanation [[AnswerCExp::N-MYC oncogene is associated with neuroblastoma.]]
Answer D AnswerD::''HER2''
Answer D Explanation [[AnswerDExp::HER2 oncogene is associated with breast, ovarian, and gastric cancers.]]
Answer E AnswerE::''C-MYC''
Answer E Explanation [[AnswerEExp::C-MYC oncogene is associated with Burkitt's lymphoma.]]
Right Answer RightAnswer::E
Explanation [[Explanation::

Educational Objective:
References: First Aid 2014 page 232]]

Approved Approved::Yes
Keyword WBRKeyword::Burkitt's lymphoma, WBRKeyword::C-myc, WBRKeyword::Oncogene, WBRKeyword::Hematologic malignancy, WBRKeyword::Fever, WBRKeyword::Constitutional symptoms
Linked Question Linked::
Order in Linked Questions LinkedOrder::