This is another good example of an SGS presentation that employs all of the standard teaching methol

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This is another good example of an SGS
presentation that employs all of the standard
teaching methologies, including identifying key
histology / radiological findings, summarizing
the case using few words, and recapping key
learning issues at the end of the case.
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Case 3 Important notes

• Hematuria after being hit in the flank area
  nothing too special
• Englarged kidneys Not normal
• Multiple, bilateral cysts
• Family history of renal problems
• Gradual decline of renal function more cysts
• Becoming increasingly uremic cinical
  manifestations of azotemia severely decreased
  renal function

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CT scan showing massively enlarged kidneys with
multiple cysts. Also, note multiple cysts in the
liver.
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Macro cystic kidney measuring 25 x 10 cm and
weighing 5650 grams (N 150). The appearance of
dark "chocolate" colored cyst is due to old
intraluminal hemorrhage.
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Micro wall of cyst (above) the epithelium is
sloughed off, compression of adjacent parenchyma
(below) with marked tubulointerstitial change and
variable glomerular sclerosis.
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Micro portions of three cysts with marked
tubulointerstitial change of intervening
parenchyma.
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Case 3 Sum up

• Adult Polycystic Kidney Disease
• Autosomal Dominant Inheritance
• Bilateral disease that develops around 20-25
  years of age (NOT AT BIRTH!). Cysts develop all
  over the kidney
• gt80 present with Hypertension
• Eventually develop CRF around age 40-60.
• Predisposition to two types of bleeds
• Intracranial berry aneurysms (subarachnoid bleed
  worst headache ever!)
• or intracerebral hemorrhage

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Clinical Vignette 1-

• Adult Polycystic Kidney Disease is associated
  with Mitral Valve Prolapse
• Scenario
• Patient comes in with hypertension, abnormal
  ultrasound in the renal area, and a click murmur
  (mitral valve prolapse)
• What is the most likely mode of inheritance of
  the disease causing these symptoms?

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Clinical Vignette 2

• Adult Polycystic Kidney Disease is also
  associated with Diverticulosis
• Scenario
• Patient comes in with hypertension, abnormal
  ultrasound in the renal area, and a 600 ml of
  bright red blood in the stool (hematochezia)
• What is the most likely etiology of the patients
  symptoms?