Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe four unrelated children with neonatal maculopapular rash, fever, arthritis, hepatosplenomegaly, lymphadenopathy, eye involvement, and neurologic symptoms. Radiographs of the joints were surprisingly similar, showing an abnormal epiphyseal and metaphyseal appearance. These clinical and radiologic findings allowed us to include these children in a very peculiar syndrome described as infantile-onset multisystemic inflammatory disease. A chondrosarcoma developed in one of our patients.
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PMID:Infantile-onset multisystem inflammatory disease: a differential diagnosis of systemic juvenile rheumatoid arthritis. 910 44

Vasculopathic syndromes have been associated with hematological and solid organ malignancies. The pathogenesis of these syndromes remains largely unknown and there are no biologic markers identified. Whether it is or is not a paraneoplastic syndrome is under discussion, the close temporal relationship of cancer and vasculitis suggests that these vasculitides are a paraneoplastic condition. We report a case of a 45-year-old female patient with pelvic chondrosarcoma who underwent surgical treatment and started to present visual loss, systemic inflammatory response syndrome (SRIS), cardiac insufficiency, hepatosplenomegaly, cholestasis as well as pulmonary bleeding suggesting a sarcoma-associated vasculitis. All antibodies were negative as in secondary vasculitis. After corticoideal therapy the vasculitis resolved and at 3-year follow-up the patient had not showed any further medical complications or recurrences of the vasculitis. The parallel evolution of the vasculitis and the solid tumor combined with the resolution of the vasculitis after corticotherapy enhances the likelihood of a paraneoplastic vasculitis associated with a chondrosarcoma according to literature review.
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PMID:Paraneoplastic vasculitis associated to pelvic chondrosarcoma: a case report. 2716 97