WBR0440
| Author | [[PageAuthor::Rim Halaby, M.D. [1]]] |
|---|---|
| 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 the patient has 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. Hence, idiopathic (essential) hypertension is the most common cause of hypertension, even in TS patients. |
| Answer B | AnswerB::Coarctation of the aorta |
| Answer B Explanation | AnswerBExp::Coarctation of the aorta is indeed associated with TS, but only 15-20% of patients of TS patients have coarctation of the aorta subsequent hypertension. |
| Answer C | AnswerC::Heart failure |
| Answer C Explanation | AnswerCExp::Heart failure is not directly associated with TS or hypertension in those patients. |
| Answer D | AnswerD::Pheochromocytoma |
| Answer D Explanation | AnswerDExp::Pheochromocytoma is not directly associated with TS or hypertension in those patients. |
| Answer E | AnswerE::Obstructive sleep apnea |
| Answer E Explanation | AnswerEExp::Obstructive sleep apnea is not directly associated with TS or hypertension in those patients. |
| 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 patients with TS at a young age compared to 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 in fact of undetermined etiology. They are thus diagnosed with idiopathic hypertension. Several hypotheses suggest that microvascular renal disease might be the actual cause of hypertension in patients with TS due to findings of elevated renin in TS patients. Educational Objective: Patients with TS have a short stature and have lymphatic defects that manifest as webbing of the neck and lymphedema of the hands and feet. There is increased incidence of horseshoe kidney in TS. TS patients are at increased risk of dysgerminoma. The risk of hypertension is approximately three times higher in patients with TS at a young age compared to the general population. The vast majority of TS patients who have hypertension are in fact of undetermined etiology. Reference:
Elsheikh M, Dunger DB, Conway GS, et al. Turner’s syndrome in adulthood. Endocrine reviews. 2002;23(1):120-140 |
| Approved | Approved::No |
| Keyword | WBRKeyword::Turner, WBRKeyword::syndrome, WBRKeyword::hypertension, WBRKeyword::essential, WBRKeyword::idiopathic |
| Linked Question | Linked:: |
| Order in Linked Questions | LinkedOrder:: |