A severe systemic presentation of pigmented villonodular synovitis in a child with underlying Chediak-Higashi syndrome

2.50
Hdl Handle:
http://hdl.handle.net/11287/594030
Title:
A severe systemic presentation of pigmented villonodular synovitis in a child with underlying Chediak-Higashi syndrome
Authors:
True, V. L.; Monsell, F. P.; Smith, T. A.; Parke, Simon C.; Grier, D. J.; Schneider, M. E.; Steward, C. G.
Abstract:
Pigmented villonodular synovitis (PVNS), a condition of synovial hyperproliferation that mostly affects large joints, is rare in children and conventionally lacks systemic symptoms. This report describes a complex paediatric patient who underwent bone marrow transplantation to control the accelerated phase of the Chediak-Higashi syndrome. Diffuse PVNS developed in one knee 2.75 years later. Progression of PVNS was accompanied by the development of severe systemic symptoms, which resolved rapidly following subtotal surgical debridement. The patient remains well with minimal elevation of inflammatory marker levels 10.5 years later. As PVNS and Chediak-Higashi syndrome are both very rare diseases we propose a potential unifying hypothesis for this combination.
Citation:
J Pediatr Orthop B. 2015 Nov;24(6):526-9.
Publisher:
Wolters Kluwer
Journal:
Journal of pediatric orthopedics. Part B
Issue Date:
1-Nov-2015
URI:
http://hdl.handle.net/11287/594030
DOI:
10.1097/BPB.0000000000000190
PubMed ID:
25967956
Additional Links:
http://www.ncbi.nlm.nih.gov/pubmed/25967956
Type:
Case Report
Language:
eng
ISSN:
1473-5865
Appears in Collections:
2015 RD&E publications; Paediatrics

Full metadata record

DC FieldValue Language
dc.contributor.authorTrue, V. L.en
dc.contributor.authorMonsell, F. P.en
dc.contributor.authorSmith, T. A.en
dc.contributor.authorParke, Simon C.en
dc.contributor.authorGrier, D. J.en
dc.contributor.authorSchneider, M. E.en
dc.contributor.authorSteward, C. G.en
dc.date.accessioned2016-01-19T12:38:49Zen
dc.date.available2016-01-19T12:38:49Zen
dc.date.issued2015-11-01en
dc.identifier.citationJ Pediatr Orthop B. 2015 Nov;24(6):526-9.en
dc.identifier.issn1473-5865en
dc.identifier.pmid25967956en
dc.identifier.doi10.1097/BPB.0000000000000190en
dc.identifier.urihttp://hdl.handle.net/11287/594030en
dc.description.abstractPigmented villonodular synovitis (PVNS), a condition of synovial hyperproliferation that mostly affects large joints, is rare in children and conventionally lacks systemic symptoms. This report describes a complex paediatric patient who underwent bone marrow transplantation to control the accelerated phase of the Chediak-Higashi syndrome. Diffuse PVNS developed in one knee 2.75 years later. Progression of PVNS was accompanied by the development of severe systemic symptoms, which resolved rapidly following subtotal surgical debridement. The patient remains well with minimal elevation of inflammatory marker levels 10.5 years later. As PVNS and Chediak-Higashi syndrome are both very rare diseases we propose a potential unifying hypothesis for this combination.en
dc.language.isoengen
dc.publisherWolters Kluweren
dc.relation.urlhttp://www.ncbi.nlm.nih.gov/pubmed/25967956en
dc.titleA severe systemic presentation of pigmented villonodular synovitis in a child with underlying Chediak-Higashi syndromeen
dc.typeCase Reporten
dc.identifier.journalJournal of pediatric orthopedics. Part Ben

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