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. |
| 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:: |