WBR0440

Author	[[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Alison Leibowitz)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Pathology
Sub Category	SubCategory::Endocrine
Prompt	[[Prompt::A 25-year-old female presents to the physician’s office for hypertension management. Upon physical examination, the patient has a short stature and lymphedema in hands and feet. The patient’s medical history demonstrates that she had been treated for dysgerminoma and a recent abdominal computed tomography (CT) scan revealed horseshoe kidney. Which of the following is most likely the most common cause of hypertension in this patient?]]
Answer A	AnswerA::Idiopathic hypertension
Answer A Explanation	AnswerAExp::Most TS patients with hypertension have no clear etiology of their hypertension. Idiopathic (essential) hypertension is the most common cause of hypertension in TS patients.
Answer B	AnswerB::Coarctation of the aorta
Answer B Explanation	AnswerBExp::Coarctation of the aorta is associated with TS, but only 15-20% of TS patients have coarctation of the aorta and subsequent hypertension.
Answer C	AnswerC::Heart failure
Answer C Explanation	AnswerCExp::Heart failure is not directly associated with TS or hypertension.
Answer D	AnswerD::Pheochromocytoma
Answer D Explanation	AnswerDExp::Pheochromocytoma is not directly associated with TS or hypertension.
Answer E	AnswerE::Obstructive sleep apnea
Answer E Explanation	AnswerEExp::Obstructive sleep apnea is not directly associated with TS or hypertension.
Right Answer	RightAnswer::A
Explanation	[[Explanation::The patient in the vignette has findings consistent with Turner syndrome (TS). Patients with TS have a short stature, lymphatic defects that manifest as webbing of the neck, lymphedema of the hands and feet, an increased incidence of horseshoe kidney, and a high risk of dysgerminoma. The risk of hypertension is approximately three times higher in young patients with TS than the general population. Coarctation of the aorta and renal disease contribute to approximately 15-20% of the etiologies of hypertension in TS patients. The vast majority of TS patients who have hypertension are of undetermined etiology and instead are diagnosed idiopathic hypertension. Several hypotheses suggest that microvascular renal disease may be the cause of hypertension in patients with TS, due to elevated renin levels in TS patients. Educational Objective: Patients with TS have a short stature, lymphatic defects that manifest as webbing of the neck, lymphedema of the hands and feet, an increased incidence of horseshoe kidney, and a high risk of dysgerminoma. The risk of hypertension is approximately three times higher in young patients with TS than the general population. The vast majority of TS patients who have hypertension are of undetermined etiology. References: Elsheikh M, Dunger DB, Conway GS, et al. Turner’s syndrome in adulthood. Endocrine reviews. 2002;23(1):120-140]]
Approved	Approved::Yes
Keyword	WBRKeyword::Turner syndrome, WBRKeyword::hypertension, WBRKeyword::idiopathic, WBRKeyword::genetics, WBRKeyword::horseshoe kidney, WBRKeyword::TS
Linked Question	Linked::
Order in Linked Questions	LinkedOrder::