WBR0315

Author	[[PageAuthor::Ogheneochuko Ajari, MB.BS, MS [1]]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Genetics, MainCategory::Pathophysiology
Sub Category	SubCategory::Vascular
Prompt	[[Prompt::A 15- year old female is brought to the physician’s office by the mother for complaints of abdominal pain. There is no history of trauma to any part of the body. Physical examination reveals delayed femoral pulses with respect to the brachial pulses and pulsatile vessels along the axilla and the ribs. The patient’s condition is likely to be seen with which one of the following?]]
Answer A	[[AnswerA::Marfan syndrome]]
Answer A Explanation	[[AnswerAExp::Incorrect. Marfan syndrome is associated with dissecting aortic aneurysms and aortic valve incompetence. There may also be an associated mitral valve prolapse]]
Answer B	[[AnswerB::Down syndrome]]
Answer B Explanation	[[AnswerBExp::Incorrect. Down syndrome is associated with endocardial cushion defects that results in atrial septal defect and aortic valve regurgitation]]
Answer C	[[AnswerC::Turner syndrome]]
Answer C Explanation	AnswerCExp::Correct. See Explanation
Answer D	[[AnswerD::Friedreich's ataxia]]
Answer D Explanation	[[AnswerDExp::Incorrect. Friedreich’s ataxia is associated with hypertrophic cardiomyopathy and the femoral pulses are not affected in this case]]
Answer E	[[AnswerE::Kartagener syndrome]]
Answer E Explanation	[[AnswerEExp::Incorrect. Kartagener syndrome is due to a microtubulular dynein arm defect and is associated with situs invertus and not aortic coarctation. Infertility, recurrent sinusitis and bronchiectasis result]]
Right Answer	RightAnswer::C
Explanation	[[Explanation::Diminished femoral pulses compared to the brachial pulses, pulsatile vessels along axilla and ribs in a young child/adult are characteristics of adult type congenital coarctation of the aorta. Turner syndrome (an XO sex chromosome disorder) is associated with coarctation of the aorta in females. Educational Objective: References: ]]
Approved	Approved::Yes
Keyword	WBRKeyword::Delayed femoral pulses, WBRKeyword::brachial pulse, WBRKeyword::Pulsatile vessels
Linked Question	Linked::
Order in Linked Questions	LinkedOrder::