Redox balance in patients with Down's syndrome before and after dietary supplementation with alpha-lipoic acid and L-cysteine.

The aim of the present study was to investigate the possible normalizing effect of antioxidants on certain parameters indicative of oxidative stress in Down's syndrome (DS). The study was performed in pediatric patients with DS with proven redox imbalance, who were advised to take a dietary supplementation composed of alpha-lipoic acid and L-cysteine for several treatment cycles (one treatment cycle = 30 days dietary supplementation plus 30 days wash-out). Serum thiol groups, serum total and septic reactive oxygen species (ROS) and total antioxidant status of serum were determined before and after dietary supplementation, using commercially available kits. In all the evaluable patients (n = 20), after 3.8 +/- 1.1 treatment cycles, thiol group serum concentrations and total antioxidant status of serum significantly increased (p < 0.0001 for both parameters) in comparison with basal values, while serum total and septic ROS significantly decreased (p < 0.0001 for both parameters). On the basis of these results it is impossible to demonstrate the clinical effects of the biochemical normalization obtained in patients with DS after supplying alpha-lipoic acid and L-cysteine. These data suggest that delaying the clinical expression of redox imbalance in patients with DS might be feasible by normalizing their redox balance.

[Monoclonal gammopathy of uncertain significance in rheumatic disease]. / Le grammapatie monoclonali di incerto significato nelle malattie reumatiche.

Among 358 patients with rheumatic diseases, the incidence of monoclonal gammopathy of undetermined significance (MGUS) as detected by immunofixation was 4.4% (11 of 248 patients) in rheumatoid arthritis (RA), 3% (1 of 32 patients) in systemic lupus erythematosus (SLE), 6% (3 of 49 patients) in Sjögren's syndrome (SS) and 3% (1 of 29 patients) in progressive systemic sclerosis (PSS). Solid tumor was present in 4 (36%) of the 11 RA-MGUS patients. In these cases the monoclonal component could be related to a paraneoplastic syndrome rather than to rheumatic diseases. The association of rheumatic diseases, MGUS, solid tumor and immunological disorders are discussed.

[Cutaneous side effects induced by D-penicillamine in the treatment of rheumatoid arthritis]. / Gli effetti collaterali cutanei indotti dalla D-penicillamina nel trattamento della artrite reumatoide.

After considering the cutaneous side effects of D-penicillamine in rheumatoid arthritis management, a few cases of cutaneous adverse reaction observed in 5 (13%) of 38 patients, who received the drugs, are described. Two patients had an early rash; one showed a late rash; one had a late rash with symptoms of initial pemphigus and drug-induced LES (presence of antinuclear and anti-dsDNA antibodies). Finally, one patient showed a drug-induced pemphigus with high titre of reticular anti-skin antibodies. All these manifestations required definitive discontinuance of the drug.

Temporal arteritis in seropositive rheumatoid arthritis with rheumatoid nodule.

A 61-year-old woman, suffering from classic seropositive rheumatoid arthritis with rheumatoid nodule histologically documented, developed temporal arteritis. HLA-DR typing revealed the presence of DR3 and DR4 antigens. The findings from previous studies support the association of HLA-DR antigens, giant cell arteritis-polymyalgia rheumatica and rheumatoid arthritis, and suggest the participation of a common immunogenetic mechanism in their pathogenesis.