Autoreactive CD4+ LKM‐specific and anticlonotypic T‐cell responses in LKM‐1 antibody‐positive autoimmune hepatitis

H F Lohr, J F Schlaak, A W Lohse, W O Bocher, M Arenz, G Gerken, K M Buschenfelde – 1 December 1996 – Peripheral blood mononuclear cells (PBMC) of patients with autoimmune hepatitis (AIH) and controls were studied for their proliferative response to six overlapping synthetic peptides covering the 33‐amino acid immunodominant region of cytochrome P450IID6, the main target antigen of LKM‐1 antibody‐positive type II AIH.

A novel two‐nucleotide deletion in the ornithine transcarbamylase gene causing fatal hyperammonia in early pregnancy

U Schimanski, D Krieger, M Horn, W Stremmel, B Wermuth, L Theilmann – 1 December 1996 – Ornithine transcarbamylase (OTC) deficiency shows X‐linked inheritance. Typically, symptomatic females (who constitute 15%‐20% of all carriers) have markedly reduced enzyme activity and show first symptoms in late infancy or early childhood. Here we present the case of a previously asymptomatic 24‐year‐old woman who died of severe hyperammonemia associated with orotic aciduria but normal OTC activity in the fourth month of pregnancy.

Effects of varying doses of spironolactone without and with nitrates on portal vein pressure and kidney function in partial portal vein ligated rats

M Van de Castelle, G Van Roey, F Nevens, J Fevery – 1 December 1996 – The optimal dose of spironolactone to reduce portal vein pressure, alone or in combination with isosorbide‐5‐mononitrate (IsMn), has not been identified. We studied the effects of 8 days oral treatment with spironolactone, IsMn or both on portal pressure, plasma volume and renal sodium handling in rats with partial portal vein ligation. At daily doses of 0.33; 0.50; 1, and 1.50 mg/kg, spironolactone reduced portal pressure (all P < .05) as compared with placebo.

Differential recognition of the 52‐kd Ro(SS‐A) antigen by sera from patients with primary biliary cirrhosis and primary Sjogren's syndrome

T Dorner, E Feist, C Held, K Conrad, G R Burmester, F Hiepe – 1 December 1996 – Antibodies against the 52‐kd Ro(SS‐A) protein are significantly associated with the primary Sjogren's syndrome (pSS). A small proportion of patients suffering from primary biliary cirrhosis (PBC) with secondary Sjogren's syndrome (PBC/SS) who are serologically characterized by antimitochondrial type 2 antibodies also express anti‐52‐kd Ro(SS‐A) antibodies.

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