Blau-Jabs Syndrome in a Tertiary Ophthalmologic Center

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dc.citation.issue1
dc.citation.volume49
dc.contributor.authorNascimento, Heloisa [UNIFESP]
dc.contributor.authorSousa, Jacqueline M. [UNIFESP]
dc.contributor.authorFernandez, Delia Gonzalez [UNIFESP]
dc.contributor.authorSalomao, Gustavo H. A. [UNIFESP]
dc.contributor.authorSato, Elcio H. [UNIFESP]
dc.contributor.authorMuccioli, Cristina [UNIFESP]
dc.contributor.authorBelfort, Rubens, Jr. [UNIFESP]
dc.coverageThorofare
dc.date.accessioned2020-07-02T18:52:11Z
dc.date.available2020-07-02T18:52:11Z
dc.date.issued2018
dc.description.abstractIn a prospective case series of patients with Blau-Jabs syndrome (BJS) conducted in the Ophthalmology Department/Federal University of Sao Paulo, seven patients math clinical and ophthalmologic manifestations of the disease and a positive genetic test result for the presence of a mutation in the CARD15/NOD2 gene were followed for a minimal period of 1 year. All patients had uveitis, five had nummular corneal subepithelial opacities, and four had multifocal choroiditis. Oral prednisolone was administered to all patientsen
dc.description.abstractinflammation was controlled in six patients with at least one immunosuppressive drug. Infliximab (Remicadeen
dc.description.abstractJanssen Pharmaceuticals, Beerse, Belgium) and etanercept (Enbrelen
dc.description.abstractAmgen, Thousand Oaks, CA) were used to treat two cases refractory to the antiinflammatory drugs. A subconjunctival dexamethasone implant (Ozurdexen
dc.description.abstractAllergan, Irvine, CA) and a periocular injection of triamcinolone were used in one case to achieve inflammation control. Six patients achieved a visual acuity of 20/25 or better. The authors conclude that periocular treatment with steroid injections might be effective adjuvant therapy to control ocular inflammation.en
dc.description.affiliationUniv Fed Sao Paulo, UNIFESP Escola Paulista Med Brazil, Dept Ophthalmol, Sao Paulo, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, UNIFESP Escola Paulista Med Brazil, Dept Ophthalmol, Sao Paulo, Brazil
dc.description.sourceWeb of Science
dc.format.extent70-75
dc.identifierhttp://dx.doi.org/10.3928/23258160-20171215-12
dc.identifier.citationOphthalmic Surgery Lasers & Imaging Retina. Thorofare, v. 49, n. 1, p. 70-75, 2018.
dc.identifier.doi10.3928/23258160-20171215-12
dc.identifier.fileWOS000419990200012.pdf
dc.identifier.issn2325-8160
dc.identifier.urihttps://repositorio.unifesp.br/handle/11600/53925
dc.identifier.wosWOS:000419990200012
dc.language.isoeng
dc.publisherSlack Inc
dc.relation.ispartofOphthalmic Surgery Lasers & Imaging Retina
dc.rightsinfo:eu-repo/semantics/openAccess
dc.titleBlau-Jabs Syndrome in a Tertiary Ophthalmologic Centeren
dc.typeinfo:eu-repo/semantics/article
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