Case: 66

Polycystic Kidney Disease

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History: 28 yo male with hematuria
Solution: Autosomal dominant polycystic kidney disease.

ADPKD is a hereditary disorder characterized by multiple renal cysts and various systemic manifestations, including: liver, pancreatic and splenic cysts, intracranial aneurysms, cardiac valve defects, and diverticulosis. About half go on to renal failure and require transplantation.

The findings on the IVP are typical with distortion of the renal collecting system on the excretory phase and an indistinct, enlarged renal contour on the nephrographic phase. The patient also has an incidental partial duplication on the right.

People with autosomal dominant PKD also can experience the following complications: * urinary tract infections * hematuria * high blood pressure * kidney stones

This patient subsequently underwent an US exam which showed very enlarged kidneys bilaterally with innumerable cysts.











Radiograph Images

Questions

The findings on the IVP include:
  • Enlarged nephrogram
  • Hydronephrosis
  • Renal calculi
  • Vascular renal mass
This patient is likely to have metastatic disease.
False
The most likely diagnosis is:
Autosomal dominant polycystic kidney disease.