WBR0440

Author	[[PageAuthor::Serge Korjian M.D. (Reviewed by Alison Leibowitz) (Reviewed by Serge Korjian)]]
Exam Type	USMLE Step 1
Main Category	Pathology
Sub Category	Endocrine
Prompt	A 25-year-old woman presents to the physician’s office for the management of hypertension. During physical examination, the physician notes that the patient has a short stature and shows signs of lymphedema in her hands and feet bilaterally. The patient’s medical chart reveals that she has recently received 6 weeks of chemotherapy for dysgerminoma and has an anatomical kidney defect detected at a very young age. Which of the following is the most likely cause of hypertension in this patient?
Answer A	Idiopathic hypertension
Answer A Explanation	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	Coarctation of the aorta
Answer B Explanation	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	Heart failure
Answer C Explanation	Heart failure is not directly associated with TS or hypertension.
Answer D	Pheochromocytoma
Answer D Explanation	Pheochromocytoma is not directly associated with TS or hypertension.
Answer E	Obstructive sleep apnea
Answer E Explanation	Obstructive sleep apnea is not directly associated with TS or hypertension.
Right Answer	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	Yes
Keyword	Turner syndrome, hypertension, idiopathic, genetics, horseshoe kidney, TS
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