WBR0415

Author	[[PageAuthor::Rim Halaby, M.D. [1], Alison Leibowitz [2] (Reviewed by Alison Leibowitz)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Pathology
Sub Category	SubCategory::Renal
Prompt	[[Prompt::A 52-year-old male with an unknown medical history dies following a motor vehicle collision. On autopsy, his kidneys displayed image below. Which of the following additional findings are most likely to be present in this patient? ]]
Answer A	AnswerA::Large hepatic multi-acinar regenerative nodules
Answer A Explanation	AnswerAExp::Large hepatic multiacinar regenerative nodules are characteristic of hepatic fibrosis. Congenital hepatic fibrosis (CHF) is common among patients with autosomal recessive polycystic kidney disease (ARPKD), which manifest early in childhood.
Answer B	AnswerB::Granulomatous inflammation and skip lesions in a bowel segment
Answer B Explanation	AnswerBExp::Granulomatous inflammation and skip lesions are characteristic of Crohn’s disease. ADPKD is not associated with Crohn’s disease.
Answer C	AnswerC::Cranial nerve (CN) III compression by an adjacent structure
Answer C Explanation	AnswerCExp::Autosomal (adult) dominant polycystic kidney disease (ADPKD) is an inherited renal disorder characterized by the development of fluid-filled cysts in both kidneys during adulthood.
Answer D	AnswerD::Granulomatous inflammation of a great vessel
Answer D Explanation	AnswerDExp::Granulomatous inflammation of the aorta is a characteristic feature of Takayasu vasculitis, or “Pulseless disease”.
Answer E	AnswerE::Atherosclerotic lesion of the coronary artery that supplies the anterior interventricular septum.
Answer E Explanation	AnswerEExp::Atherosclerotic lesions of the left anterior descending artery (LAD) are not associated with ADPKD.
Right Answer	RightAnswer::C
Explanation	[[Explanation::Autosomal (adult) dominant polycystic kidney disease (ADPKD), an inherited renal disease, is characterized by fluid-filled cystic enlargement in both kidneys during adulthood that progressively leads to renal failure. ADPKD is associated with berry aneurysms and multiple cysts of the liver. Berry aneurysms result from weakness of cerebral arteries, which causes dilation of the affected artery. Aneurysm formation in the posterior communicating artery may compress CN III. CN III contains an inner central motor and an outer peripheral parasympathetic component, leading diseases to affect CN III differently. The mechanical compression affects the outer parasympathetic output leading to “blown pupils” with absent papillary light reflex, while vascular diseases, such as diabetes mellitus, cause ischemic injury to the inner motor component. Patients present with a “down and out” gaze when their motor component is affected. Educational Objective: Autosomal (adult) dominant polycystic kidney disease (ADPKD) is an inherited renal disorder characterized by the development fluid-filled cysts in both kidneys during adulthood. ADPKD is associated with berry aneurysms that frequently affect the posterior communicating artery, compressing CN III. References: First Aid 2014 page 87]]
Approved	Approved::Yes
Keyword	WBRKeyword::kidney, WBRKeyword::excretory system, WBRKeyword::autosomal dominant, WBRKeyword::genetics, WBRKeyword::pattern of inheritance, WBRKeyword::berry aneurysms, WBRKeyword::CN III, WBRKeyword::artery
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