MedPix® Case: 5990 - ADPKD -Autosomal Dominant Polycystic Kidney Disease

38.107.191.88

MedPix® Teaching File Case No: 5990

Case Summary: 5990 - \|\| - - \|\| - -
Contributed by: Aaron M Taylor ( National Capital Consortium ) - \|\| -
Approved by: Maria Elena Pace, M.D. ( Uniformed Services University ) - \|\| -
Diagnosis	ADPKD -Autosomal Dominant Polycystic Kidney Disease
Dx confirmed by	* Not Provided by Author *
Demographic	Age: 52 - Gender: woman - caucasian
History	52 year old female with chronic renal disease and increasing creatinine.
Exam and Lab	creatinine of 1.8
Images	- Click Here to Display Images
Findings	multiple bilateral renal cysts on ultrasound and CT multiple hepatic cysts on ultrasound and CT
Differential	renal cell carcinoma medullary cystic disease endstage renal failure hepatic abscess autosomal dominant polysystic kidney disease renal abscess
Treatment and Followup	dialysis renal transplant

Disease Topic	ADPKD -Autosomal Dominant Polycystic Kidney Disease
Disease Discussion	ADPKD is an autosomal dominant condition which is responsible for approximately 10% of ESRD. It may present at any age, but most commonly in the 3rd to 4th decade of life. Patients may present with hypertension (present in up to 75% of adult patients), microscopic hematuria, palpable abdominal mass, or flank pain secondary to mass effect. The flank pain is often related to cyst hemorrhage, cyst enlargement with stretching of the renal capsule, or ureteral obstruction. Other complications include stone formation, infection, cyst hemorrhage/rupture and end stage renal disease. Typically, the kidneys are markedly enlarged with a distorted contour caused by multiple cysts in both the cortex and medulla. A variable amount of functioning renal tissue is preserved. There is no increased risk of renal cell carcinoma unless the patient is on long-term dialysis. Extrarenal cysts are found in the liver of up to 50-70% of patients. Other extrarenal sites of cyst formation include spleen, pancreas, and ovaries. There is also an association with intracranial Berry aneurysms in the which are present in 5-10% of patients. Head imaging is not necessarily indicated unless there is a positive family history of subarachnoid hemorrhage. Ultrasound is generally the preferred method of detection in symptomatic patients, and it is also the best method of following progression of disease. CT is useful in locating smaller cysts and will reveal all of the pathologic cystic findings as described above.
Reference(s)	Fauci AS, et al. Harrison\'s Principles of internal Medicine. 1998. McGraw Hill. New York, NY. Juhl JH, Crummy AB, Kuhlman JE. Paul and Juhl\'s Essentials of Radiologic Imaging. 1998. Lippincott, Williams, and Wilkins. Philadelphia, PA. Slone RM, Fisher AJ, Pickhardt PJ, et al. Body CT: A Practical Approach. 2000. Mc-Graw Hill.

Use this for Comments and Suggestions

MedPix® is sponsored by the Department of Radiology and Radiological Sciences, USUHS, Bethesda, MD
We do not accept paid advertisements.

We comply with the HONcode standard
for trustworthy health information:
verify here.

Text and Images may be Copyrighted © 1999 - 2009 by the Original Content Contributors.
Copyrighted materials are reproduced here with their Permission.
MedPix® is a Registered Trademark of USUHS :: The MedPix® Database Engine is Patented - USPTO No. 7,080,098
Portions of MedPix® are Copyright © 1999 - 2009 by J.G. Smirniotopoulos, M.D. & H. Irvine, M.D.
The MedPix® Classification Schema Copyright © 1999 - 2009 by J.G.Smirniotopoulos,M.D.

MedPix® has displayed more than 324,659,311 pages since 3 September 2000.

Database Successfully Disconnected