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(Created page with "{{WBRQuestion |QuestionAuthor={{Rim}} |ExamType=USMLE Step 1 |MainCategory=Genetics |SubCategory=Neurology |MainCategory=Genetics |SubCategory=Neurology |MainCategory=Genetics...")
 
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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}}, {{AJL}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Genetics
|MainCategory=Genetics
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|MainCategory=Genetics
|MainCategory=Genetics
|SubCategory=Neurology
|SubCategory=Neurology
|Prompt=A two year old male infant was brought by his mother to the pediatrician because she is feeling that her son is generally not doing fine.  The mother has been noticing that the development of her child for the last few months is not the same as it was initially.  The mother reports that the child is irritable most of the time, cries more than usual and is not growing well.  Upon physical exam, the pediatrician notices that the infant's weight dropped outside his normal growth curve. In addition , abdominal swelling is noted. The pediatrician suspects the presence of an extracranial neurological malignancy that commonly occurs in the adrenal glands.  Mutation in which of the following is associated with the patient's condition?
|Prompt=A mother brings her 2-year-old male child to the physician's office, after noticing that, over the past few months, he has been developing abnormally.  The mother reports that the child is frequently irritable, cries more than usual, and his growth has slowed.  Upon physical exam, you notice that the infant's weight falls lower than the 3rd percentile on the standard growth chart and is experiencing abdominal distention. Urine testing displays elevated levels of vanillymandelic acid (VMA) and homovanillic acid (HVA). You suspect the presence of an extracranial neurological malignancy, which frequently occurs in the adrenal glands.  Mutation in which of the following genes is most likely associated with the patient's condition?
 
|Explanation= The patient's symptoms of failure to thrive, irritability, and abdominal distention in conjunction with the elevated levels of urine VMA and HVA in the urine, points towards the diagnosis of [[neuroblastoma]], associated with a mutation in the [[N-myc]] oncogene.
 
|EducationalObjectives= The patient's symptoms of failure to thrive, irritability, and abdominal distention in conjunction with the elevated levels of urine VMA and HVA in the urine, points towards the diagnosis of [[neuroblastoma]], associated with a mutation in the [[N-myc]] oncogene.
|References= First Aid 2014 page 318
 
|AnswerA=N-myc
|AnswerA=N-myc
|AnswerAExp=[[N myc]] oncogene is associated with [[Neuroblastoma]].
|AnswerAExp= The [[N-myc]] oncogene is associated with [[Neuroblastoma]].
|AnswerB=Ret
|AnswerB=Ret
|AnswerBExp=[[Ret gene|Ret oncogene]] is associated with [[MEN|MEN syndrome]] type IIa and IIb.
|AnswerBExp= The [[Ret gene|Ret oncogene]] is associated with [[MEN|MEN syndrome]], type IIa and IIb.
|AnswerC=Abl
|AnswerC=Abl
|AnswerCExp=[[Abl gene|Abl oncogene]] is associated with CML.
|AnswerCExp= The [[Abl gene|Abl oncogene]] is associated with CML.
|AnswerD=L-myc
|AnswerD=L-myc
|AnswerDExp=[[L-myc]] oncogene is associated with [[lung cancer]].
|AnswerDExp= The [[L-myc]] oncogene is associated with [[lung cancer]].
|AnswerE=C-myc
|AnswerE=C-myc
|AnswerEExp=C-myc is associated with [[Burkitt's lymphoma]].
|AnswerEExp= The [[C-myc]] is associated with [[Burkitt's lymphoma]].
|RightAnswer=A
|RightAnswer=A
|Approved=No
|Approved=Yes
}}
}}

Revision as of 14:28, 11 July 2014
Author [[PageAuthor::Rim Halaby, M.D. [1], Alison Leibowitz [2] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Genetics
Sub Category SubCategory::Neurology
Prompt [[Prompt::A mother brings her 2-year-old male child to the physician's office, after noticing that, over the past few months, he has been developing abnormally. The mother reports that the child is frequently irritable, cries more than usual, and his growth has slowed. Upon physical exam, you notice that the infant's weight falls lower than the 3rd percentile on the standard growth chart and is experiencing abdominal distention. Urine testing displays elevated levels of vanillymandelic acid (VMA) and homovanillic acid (HVA). You suspect the presence of an extracranial neurological malignancy, which frequently occurs in the adrenal glands. Mutation in which of the following genes is most likely associated with the patient's condition?]]
Answer A AnswerA::N-myc
Answer A Explanation [[AnswerAExp::The N-myc oncogene is associated with Neuroblastoma.]]
Answer B AnswerB::Ret
Answer B Explanation [[AnswerBExp::The Ret oncogene is associated with MEN syndrome, type IIa and IIb.]]
Answer C AnswerC::Abl
Answer C Explanation [[AnswerCExp::The Abl oncogene is associated with CML.]]
Answer D AnswerD::L-myc
Answer D Explanation [[AnswerDExp::The L-myc oncogene is associated with lung cancer.]]
Answer E AnswerE::C-myc
Answer E Explanation [[AnswerEExp::The C-myc is associated with Burkitt's lymphoma.]]
Right Answer RightAnswer::A
Explanation [[Explanation::The patient's symptoms of failure to thrive, irritability, and abdominal distention in conjunction with the elevated levels of urine VMA and HVA in the urine, points towards the diagnosis of neuroblastoma, associated with a mutation in the N-myc oncogene.

Educational Objective: The patient's symptoms of failure to thrive, irritability, and abdominal distention in conjunction with the elevated levels of urine VMA and HVA in the urine, points towards the diagnosis of neuroblastoma, associated with a mutation in the N-myc oncogene.
References: First Aid 2014 page 318]]

Approved Approved::Yes
Keyword
Linked Question Linked::
Order in Linked Questions LinkedOrder::
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