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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}}, {{AJL}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Pathology
|MainCategory=Pathology
Line 20: Line 20:
|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Renal
|SubCategory=Renal
|Prompt=A 52 year old male patient with an unknown past medical history dies after a motor vehicle collision.  On autopsy, his kidneys have the following appearance as seen in the image below.  Which of the following additional findings are most likely to be present in this patient?
|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?


[[Image:Polycystic kidneys, gross pathology 20G0027 lores.jpg|500px]]
[[Image:Polycystic kidneys, gross pathology 20G0027 lores.jpg|500px]]
|Explanation=[[Autosomal (adult) dominant polycystic kidney disease]] (ADPKD) is an inherited renal disease characterized by fluid-filled cystic enlargement in both kidneys during adulthood that progressively lead to [[renal failure]].  ADPKD is associated with [[berry aneurysms]] and multiple cysts of the liver.
|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 are caused by weakness of [[cerebral arteries]] that cause dilation of the affected artery. Aneurysm formation in the [[posterior communicating artery]] may compress [[CN III]].  
[[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.
 
|EducationalObjectives=
[[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


CN III contains an inner central motor and an outer peripheral parasympathetic component.  Hence, different diseases may affect CN III differently.  The mechanical compression affects the outer parasympathetic output leading to “blown pupils” with absent papillary light reflex; while vascular diseases, like [[diabetes mellitus]], cause ischemic injury to the inner motor component.  Patients present with a “down and out” gaze when 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 commonly affect the posterior communicating artery; compressing CN III.
|AnswerA=Large hepatic multi-acinar regenerative nodules
|AnswerA=Large hepatic multi-acinar regenerative nodules
|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) that presents early in childhood.
|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.
|AnswerB=Granulomatous inflammation and skip lesions in a bowel segment
|AnswerB=Granulomatous inflammation and skip lesions in a bowel segment
|AnswerBExp=Granulomatous inflammation and skip lesions are characteristic of Crohn’s disease.  ADPKD is not associated with Crohn’s disease.
|AnswerBExp=Granulomatous inflammation and skip lesions are characteristic of Crohn’s disease.  ADPKD is not associated with Crohn’s disease.
|AnswerC=Cranial nerve (CN) III compression by an adjacent structure
|AnswerC=Cranial nerve (CN) III compression by an adjacent structure
|AnswerCExp=Autosomal (adult) dominant polycystic kidney disease (ADPKD) is an inherited renal disorder characterized by the development fluid-filled cysts in both kidneys during adulthood.
|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.
|AnswerD=Granulomatous inflammation of a great vessel
|AnswerD=Granulomatous inflammation of a great vessel
|AnswerDExp=Granulomatous inflammation of the aorta is a characteristic feature of Takayasu vasculitis, or “Pulseless disease”.
|AnswerDExp=Granulomatous inflammation of the aorta is a characteristic feature of Takayasu vasculitis, or “Pulseless disease”.
|AnswerE=Atherosclerotic lesion of the coronary artery that supplies the anterior interventricular septum.
|AnswerE=Atherosclerotic lesion of the coronary artery that supplies the anterior interventricular septum.
|AnswerEExp=Atherosclerotic lesion of the left anterior descending artery (LAD) is not associated with ADPKD.
|AnswerEExp=Atherosclerotic lesions of the left anterior descending artery (LAD) are not associated with ADPKD.
|RightAnswer=C
|RightAnswer=C
|Approved=No
|WBRKeyword= kidney, excretory system, autosomal dominant, genetics, pattern of inheritance, berry aneurysms, CN III, artery
|Approved=Yes
}}
}}

Revision as of 14:59, 18 July 2014
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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