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CASE HISTORY

CASE HISTORY. 11 y.o. female presenting with hematuria following mild lumbar trauma US (not shown) cystic tumor in the LK CT-scan. WORK-UP. Normal lung CT No familial history Normal renal US of both parents. Preoperative diagnosis Multilocular cystic nephroma (multilocular cyst).

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CASE HISTORY

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  1. CASE HISTORY • 11 y.o. female presenting with hematuria following mild lumbar trauma • US (not shown) cystic tumor in the LK • CT-scan

  2. WORK-UP • Normal lung CT • No familial history • Normal renal US of both parents Preoperative diagnosis Multilocular cystic nephroma (multilocular cyst)

  3. PATHOLOGICAL DIAGNOSIS Unilateral autosomal dominant polycystic kidney disease(Left nephrectomy)

  4. DISCUSSION • PKD1: new mutation = 5 to 7% of pediatric cases • In contrast to adult, the disease appearsto be unilateral initially in 17% of children(Am J Kidney Dis 1999; 4:639-645) • Bilaterally detectable disease is the rule at FU, especially after the age of 13.

  5. CONCLUSION What should be avoided in unilateralinvolvement of ADPKD in children:to perform a nephrectomy.

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