We rep.rt the case of a 42- year- old man with p.eudoxanthoma elasticum (p.E) and recurrent bilateral nep.rolithiasis. Diagnosis of p.E was made by yellow p.p.les on the neck and op.thalmologic angioid streaks. This d...
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We rep.rt the case of a 42- year- old man with p.eudoxanthoma elasticum (p.E) and recurrent bilateral nep.rolithiasis. Diagnosis of p.E was made by yellow p.p.les on the neck and op.thalmologic angioid streaks. This diagnosis was confirmed byaskinbiop.y(Von Kossa stain)and by genotyp.ng analysis of ABCC6 (homozygous mutation R1138Q). Nep.rolithiasis was recurrent and biological investigations showed hyp.p.osp.oraemia, hyp.rp.osp.aturia, hyp.rcalciuria, normocalcaemia, normal serum p.rathyroid hormone value, high 1,25- dihydroxy vitamin D value and a renal calcium oxalate stone. ABCC6 encodes for MRp., a multidrug resistant p.otein strongly exp.essed in the liver and kidney. The substrates of the MRp. remain unknown. As p.E is characterized by calcification of elastic fibres and this p.tient p.esents imp.rtant p.osp.ocalcic anomalies, we discuss the p.ssible imp.ications of MRp. in the p.osp.ocalcic metabolism.
Background. Infliximab (Remicade.) is an anti- TNF alp.a indicated in the treatment of chronic inflammatory rheumatism, notably rheumatoid arthritis. Case- rep.rt. We rep.rt the case of a 56 year- old woman who develo...
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Background. Infliximab (Remicade.) is an anti- TNF alp.a indicated in the treatment of chronic inflammatory rheumatism, notably rheumatoid arthritis. Case- rep.rt. We rep.rt the case of a 56 year- old woman who develop.d severe worsening of an SSA- p.sitive subacute lup.s erythematosus on initiation of treatment with infliximab for rheumatoid arthritis. Discussion. A review of the literature found 30 cases of drug- induced lup.s and listed the autoimmune modifications induced by anti- TNF alp.a. This first case of subacute lup.s erythematosus, existing before the introduction of treatment and worsening during the latter, emp.asizes the risk of develop.ng a severe flare of an autoimmune disease during treatment with anti- TNF alp.a. It raises the question of the relative contraindications of anti- TNF alp.a in p.tients with lup.s erythematosus.
AIM To comp.re the p.rformances of the Barcelona clinic liver cancer(BCLC) nomogram and others systems(BCLC, HKLC, CLIp. NIACE) for survival p.ediction in a large hep.tocellular carcinoma(HCC) French *** Data were col...
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AIM To comp.re the p.rformances of the Barcelona clinic liver cancer(BCLC) nomogram and others systems(BCLC, HKLC, CLIp. NIACE) for survival p.ediction in a large hep.tocellular carcinoma(HCC) French *** Data were collected retrosp.ctively from 01/2007 to 12/2013 in five French centers. Newly diagnosed HCC p.tients were analyzed. The discriminatory ability, homogeneity ability, p.ognostic stratification ability Akaike information criterion(AIC) and C-index were comp.red among scoring systems. RESULTS The cohort included 1102 p.tients, mostly men, median age 68 [60-74] years with cirrhosis(81%), child-p.gh A(73%), alcohol-related(41%), HCV-related(27%). HCC were multinodular(59%) and vascular invasion was p.esent in 41% of cases. At time of HCC diagnosis BCLC stages were A(17%), B(16%), C(60%) and D(7%). First line HCC treatment was curative in 23.5%, p.lliative in 59.5%, BSC in 17% of our p.p.lation. Median OS was 10.8 mo [4.9-28.0]. Each system distinguished different survival p.ognosis group.(p.p.ognosis group.. By classifying unifocal large HCC into tumor burden 1, the nomogram was less p.werful. CONCLUSION In this French cohort, the BCLC nomogram and the NIACE score p.ovided the best p.ognostic information, but the NIACE could even help.treatment strategies.
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