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Case Reports
. 2001 Jul-Aug;21(4):315-7.
doi: 10.1159/000046267.

Two enlarged kidneys: a manifestation of Erdheim-Chester disease

M André  1 , I Delèvaux, B de Fraissinette, J Ponsonnaille, N Costes Chalret, B Wechsler, J C Piette, O Aumaître
Affiliations
Case Reports

Two enlarged kidneys: a manifestation of Erdheim-Chester disease

M André et al. Am J Nephrol. 2001 Jul-Aug.

Abstract

Erdheim-Chester disease is a rare form of non-Langerhans' cell histiocytosis. This disorder is characterized by a bone involvement and several extraskeletal manifestations. We describe the case of a patient with a pleural and pericardial effusion leading to tamponade. Pathological examination of pericardium and mediastinal adenopathy was normal. The abdominal computed tomography scan showed two enlarged kidneys suggestive of Erdheim-Chester disease. Bone scan scintigraphy demonstrated symmetrical increased labeling of the long bones. The biopsy of perirenal soft tissue confirmed the diagnosis of Erdheim-Chester disease.

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