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Ventricular septal defect
2%
1/42
Fixed splitting of S2
12%
5/42
Hepatic vein thrombosis
14%
6/42
Mid-systolic click
24%
10/42
Preceding dermatologic infection
43%
18/42
Select Answer to see Preferred Response
This patient is presenting with hematuria, hypertension, and kidney failure (elevated creatinine), with cysts in the liver and bilateral kidneys on CT scan, suggestive of autosomal dominant polycystic kidney disease (ADPKD). ADPKD is associated with mitral valve prolapse (a mid-systolic click). Adult polycystic kidney disease (also known as autosomal-dominant polycystic kidney disease) is a genetic disease characterized by bilateral and massive enlargement of the kidneys secondary to multiple large cysts. The classic presentation is a patient greater than 30 years of age presenting with flank pain and hematuria who also is found to have hypertension and progressive renal failure. The disease is associated with polycystic liver disease, berry aneurysms, and mitral valve prolapse (a valvular abnormality known for causing a mid-systolic click). These patients are also at greater risk of diverticulosis. While the autosomal dominant form of the disease more often affects adults, the autosomal recessive form often presents in childhood or in utero. Figure/Illustration A is a CT scan with polycystic kidneys and cysts in the liver (red arrows). Incorrect Answers: Answer 1: Ventricular septal defect is commonly associated with Down syndrome, fetal alcohol syndrome, and infants born to diabetic mothers. Answer 2: Fixed splitting of S2 is associated with an atrial septal defect, which is a congenital heart defect not associated with ADPKD. Answer 3: Hepatic vein thrombosis describes Budd-Chiari syndrome which is when thrombosis of the hepatic veins leads to hepatic congestion, a nutmeg liver, and possibly signs of liver dysfunction with an elevation in AST/ALT. It is associated with polycythemia vera (an increase in red blood cells, white blood cells, and platelets). Answer 5: Preceding dermatologic infection describes poststreptococcal glomerulonephritis (PSGN). PSGN can present after impetigo or pharyngitis with periorbital edema, hypertension, and dark urine which is self-limited in nature. There are no flank masses or signs of severe kidney failure that are permanent in this condition. Bullet Summary: Autosomal dominant polycystic kidney disease (ADPKD) is associated with mitral valve prolapse.
4.5
(13)
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