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01-09-2006 | Clinical Image

Systemic hyalinosis with extensive lytic bone lesions

Authors: Steven Colley, Karl Johnson

Published in: Pediatric Radiology | Issue 9/2006

Excerpt

The systemic hyalinoses are rare autosomal recessive disorders, with multiorgan deposition of amorphous hyaline material. The conditions have a wide spectrum of expressivity, varying from infantile generalized forms with early death, to localized juvenile forms with prolonged survival [1]. Clinical features include skin lesions, gingival hyperplasia, joint contractures and bone involvement—typically diffuse osteopenia and small osteolytic lesions [2]. …

Urbina F, Sazunic I, Murray G (2004) Infantile systemic hyalinosis or juvenile hyaline fibromatosis? Pediatr Dermatol 21:154–159PubMedCrossRef

Suzuki S, Kashara Y, Seto Y, et al (1992) Juvenile hyaline fibromatosis. J Bone Joint Surg Am 74A:290–293

Title: Systemic hyalinosis with extensive lytic bone lesions
Authors: Steven Colley
Karl Johnson
Publication date: 01-09-2006
Publisher: Springer-Verlag
Published in: Pediatric Radiology / Issue 9/2006
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-006-0196-0

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Systemic hyalinosis with extensive lytic bone lesions

Excerpt

At a glance: The STEP trials

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Excerpt

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