erdheim-chester disease/amyloid

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Nephrotic syndrome and amyloid A amyloidosis in a patient with Erdheim-Chester disease.

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Plasma Cell Myeloma Presenting With Amyloid-Laden Crystal-Negative Histiocytosis

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Objectives: Crystal-storing histiocytosis (CSH) is rare in plasma cell dyscrasias, with only 3 cases reported in the setting of amyloid. No cases of crystal-negative histiocytosis coincident with multiple myeloma and amyloidosis have been

Ultra-rare cystic disease

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Diffuse cystic lung diseases include a group of heterogeneous disorders characterised by the presence of cysts within the lung parenchyma, sometimes showing a characteristic computed tomography scan pattern that allows diagnosis. The pathogenetic mechanisms underlying cyst formation in the lung are

Diagnostic approach to primary retroperitoneal pathologies: what the radiologist needs to know

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Retroperitoneal soft tissue lesions represent a wide range of disease processes with overlapping imaging findings. Familiarity with the CT and MR characteristics of these conditions is important to guide clinical evaluation. We review the tissue types, characteristic clinical, demographic, and

Ocular correlates of inborn metabolic defects.

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The eye provides unique opportunities for the detection, during life, of deposits of storage substances and other characteristic changes resulting from inborn metabolic defects. The cornea shows the macromolecular polysaccharides of Hurler's disease, the cystine crystals in cystinosis, and the

Unusual nonneoplastic peritoneal and subperitoneal conditions: CT findings.

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Peritoneal disease can manifest at computed tomography (CT) as fluid accumulation within the peritoneal cavity (ascites) or soft-tissue infiltration of the various peritoneal ligaments and mesenteries. Beyond the commonly encountered cases of typical ascites and peritonitis, there is a wide spectrum

Imaging Manifestations of Hematologic Diseases with Renal and Perinephric Involvement.

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The kidneys and perinephric tissues can be affected by a variety of hematologic disorders, which usually occur in the setting of multisystem involvement. In many of these disorders, imaging is used to evaluate the extent of disease, guide biopsy, and/or monitor disease activity and patient response

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