Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038220 (status epilepticus)
7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Prolonged spiking fever, an evanescent salmon-colored rash, arthralgia or arthritis, leukocytosis and organ dysfunction are characteristic of adult onset Still's disease (AOSD). A 25-year-old woman with fever lasting over 3 weeks presented to our clinic. The patient had a spiking fever, sore throat, tender lymph nodes, a fine pink-colored skin rash, arthralgia, myalgia with a high ESR, ferritin and elevated hepatic enzymes. NSAID and prednisolone were prescribed for AOSD with SIRS. After 4 days of therapy, with mild confusion, the patient went into status epilepticus lasting several hours and died after cardiovascular collapse. There has been only one case of status epilepticus associated with AOSD in the medical literature. Here we report a case of AOSD with SIRS complicated by fatal status epilepticus.
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PMID:A case of adult onset Still's disease with systemic inflammatory response syndrome complicated by fatal status epilepticus. 1830 88

An Afro-Caribbean woman presented with worsening breathlessness, weight loss, lethargy and fevers, developing a bilateral florid erythematous rash on her legs. She was recently diagnosed with rheumatoid arthritis and bilateral hilar lymphadenopathy was found on thoracic CT imaging. She was tachycardic and investigations revealed pancytopenia, eosinophilia, raised serum ACE, acute kidney injury and deranged liver function tests. Biopsy of the lymphadenopathy revealed mixed lymphoid cells and liver biopsy revealed extramedullary haematopoiesis, with hypercellular marrow found on bone marrow biopsy. Cardiac MRI was normal, excluding cardiac sarcoid. The patient developed status epilepticus and phenytoin was started. She subsequently developed skin desquamation, in keeping with toxic epidermal necrosis. Skin biopsies revealed atypical granulomas and multinucleated giant cells, which subsequently resolved on steroid treatment. This case highlights an overlap syndrome, with an unclear diagnosis between sarcoidosis, drug reaction or rash with eosinophilia and systemic symptoms and/or hypereosinophilic syndrome and Still's disease. Hence varied serological and clinical features can complicate the distinction between diagnoses.
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PMID:Is this still just sarcoidosis, or should we a-DRESS a different diagnosis? 2612 53