Evidence of impaired sense of smell in hereditary angioedema.

Background: Hereditary angioedema (HAE) is an autosomal-dominant disorder resulting from C1-inhibitor (C1INH) deficiency. Smell impairments were found in patients affected with systemic lupus erythematosus, that, similarly to HAE, is characterized by the activation of the classical complement pathway with C4 consumption. In this study, we aimed at evaluating the sense of smell in patients with HAE.

Exploratory data analysis on the effects of non pharmacological treatment for knee osteoarthritis.

Objectives: Osteoarthritis (OA) is a chronic rheumatic disease characterized by progressive cartilage destruction mediated by cytokines and other molecules. Chondrocyte activity and metabolism have attracted interest as targets of drug intervention, and spa-therapy can influence the serum levels of several cytokines. We investigated the effects of spa-therapy on clinical and ultrasonographic (US) findings and serum levels of cartilage oligomeric matrix protein (COMP) and several cytokines, chemokines, and growth factors in a prospective cohort of patients with symptomatic knee OA.

The type I IFN system in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic inflammatory autoimmune disorder characterized by joint inflammation, immune cell infiltration of the synovia, and cartilage/bone destruction. Despite noteworthy progress in the treatment of RA in recent years, many patients remain refractory to current therapeutic strategies that target either the adaptive immune system or mediators of the innate system. Type I interferons (IFNs) play a significant role in regulation of the innate immune system, originally being discovered as part of intracellular immune defence against viral infection.

NK cells, autoantibodies, and immunologic infertility: a complex interplay.

Infertility and recurrent spontaneous abortion (RSA) are heterogeneous conditions that have been frequently explained with an immunological pathomechanism. A deeper insight into apparently unexplained infertility and RSA shows increasing evidences supporting both alloimmune and autoimmune mechanisms, in which natural killer (NK) cells and autoantibodies seem to play a relevant role. Successful pregnancy is considered as Th1-Th2 cooperation phenomenon, with a predominantly Th2-type lymphocytes response, together with the emerging role of interleukin (IL)-12, IL-15, and IL-18 and of other unidentified soluble factors dependent on NK cells.

Glutathione: a key player in autoimmunity.

Increasing attention in the physiopathology of inflammatory/immunomediated diseases has been focused on the role of reactive oxygen species (ROS), oxygen-based molecules possessing high chemical reactivity and produced by activated neutrophils during the inflammatory response. During chronic inflammation, when sustained production of ROS occurs, antioxidant defences can weaken, resulting in a situation termed oxidative stress. Moreover, antioxidant defence systems have been demonstrated to be constitutively lacking in patients affected with chronic degenerative diseases, especially inflammatory/immunomediated.

Radioimmunological detection of anti-transglutaminase autoantibodies in human saliva: a useful test to monitor coeliac disease follow-up.

Background: Serum radioimmunoassay (RIA) tissue transglutaminase autoantibodies (tTG-Abs) proved to be a sensitive test also during coeliac disease (CD) follow-up. We demonstrated that RIA tTG-Abs could be detected in human saliva.

Aim: To evaluate salivary RIA tTG-Abs in coeliac children on gluten-free diet (GFD).

NK cells in autoimmunity: a two-edg'd weapon of the immune system.

Natural killer (NK) cells are part of the innate-immune system and respond rapidly to a variety of insults via cytokine secretion and cytolytic activity. Their main function is first line of innate immunity across viral, bacterial and parasitic infections. NK-cells are not solely killers but can also act as regulators of adaptive immunity.

[Feasibility study of biological monitoring of chemical agents by means of evaluation of the effects on their in vitro effects on the complement system].

Immunological methods for the study of the plasma complement system have been standardized in order to be good and reproducible indicators of some biological effects of the substances under study in in vitro experiments. The substances tested were not capable of interfering within 10 times the possible hypothetical plasma concentration reached in vivo with the function of the different reagents used in the study of complement. Five substances (Skin-ACGIH) have been studied for their effects on the complement system in vitro; four of them could be fully studied (allylic alcohol, cyclohexanone, phenol, dimethylacetamide).

Intravenous immunoglobulin therapy in pregnant patients affected with systemic lupus erythematosus and recurrent spontaneous abortion.

Objectives: We aimed to test the maternal and fetal outcome of SLE patients who suffered from recurrent spontaneous abortion (RSA) treated with intravenous immunoglobulin (IVIg) alone during pregnancy and whether the clinical response to IVIg treatment is accompanied by modifications of SLE-associated antibodies and of complement levels.

Methods: Twelve SLE-RSA pregnant patients were treated with high-dose IVIg and compared with 12 SLE-RSA pregnant patients treated with prednisolone and NSAIDs. They were evaluated for the clinical response [lupus activity index-pregnancy (LAI-P) scale] and for ANA, anti-dsDNA, anti Ro/SS-A or La/SS-B, aCL, LAC, C4, C3 before and during pregnancy, and before and after each treatment course.

High levels of NK cells in the peripheral blood of patients affected with anti-phospholipid syndrome and recurrent spontaneous abortion: a potential new hypothesis.

Objectives: No data regarding phenotypic assets of circulating lymphocytes in anti-phospholipid syndrome (APS) are reported in the literature. Role of anti-phospholipid antibodies (aPL) in recurrent spontaneous abortion (RSA) remains uncertain, while natural killer (NK)-cells are involved in RSA pathogenesis. In this study, patients affected with APS without RSA, APS with RSA and RSA without aPL were studied for NK-cell subpopulation to evaluate its role in abortive events typical of APS.

High levels of peripheral blood NK cells in women suffering from recurrent spontaneous abortion are reverted from high-dose intravenous immunoglobulins.

Problem: To determine the levels of peripheral blood natural killer (NK) cells in healthy women and recurrent aborters, and the effect of intravenous immunoglobulins (IVIGs) on these levels.

Method Of Study: A total of 659 women were evaluated for NK cells by means of flow cytofluorimetry: 42 non-pregnant healthy women, 394 non-pregnant recurrent spontaneous abortion (RSA) women, 36 pregnant healthy women and 187 pregnant RSA women. Fifty-four of the pregnant RSA women were treated with IVIG; in 18 of them NK cells were measured immediately before and after the very first IVIG infusion (0.

Anti-thyroid antibodies and antiphospholipid syndrome: evidence of reduced fecundity and of poor pregnancy outcome in recurrent spontaneous aborters.

Problem: To determine the presence of anti-thyroid antibodies in patients with primary antiphospholipid syndrome (APS) [antiphospholipid antibodies (aPL) + recurrent spontaneous abortion (RSA)], compare APS alone with APS and thyroid autoimmunity for fecundity and for pregnancy outcome.

Method Of Study: A total of 203 non-pregnant women affected with primary APS were evaluated for anti-thyroid antibodies; 162 non-pregnant women affected with RSA and thyroid autoimmunity alone served as controls.

Results: Anti-thyroid antibodies were found in 27% of APS patients studied.

Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.

Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States.

A G-to-A mutation in IVS-3 of the human gamma fibrinogen gene causing afibrinogenemia due to abnormal RNA splicing.

Congenital afibrinogenemia is a rare autosomal recessive disorder characterized by a hemorrhagic diathesis of variable severity. Although more than 100 families with this disorder have been described, genetic defects have been characterized in few cases. An investigation of a young propositus, offspring of a consanguineous marriage, with undetectable levels of functional and quantitative fibrinogen, was conducted.

[Echographic diagnosis and treatment of urethral instability].

Unstable urethra as defined by the International Continence Society is the condition where loss of urine is caused solely by a fall in urethral pressure. But the condition is rare and therefore the term Urethral Pressure Variations (UPV) is better used. The UPV are caused by activity of smooth and striated muscles.

[Endoscopic treatment of sphincter insufficiency with autologous fat injection].

A total of 11 women with stress urinary incontinence related to intrinsic sphinteric incontinence (type 3) underwent periurethral injection of autogolous fat. The fat was harvested from the abdominal wall by special liposuction unit including: a special syringe for plastic surgery provided with 6 straight needles 12-20 Gauge connected to a vaum extractor for the liposuction; a 14 Gauge straight needle for lipofilling periurethrally and then a cystoscope with a 0 degree and 70 degree lenses. The fat is injected at 3 and 9 o'clock position and the mean quantity was 15,5 ml.

Caffeine stimulates in vivo platelet reactivity.

The effect of coffee drinking on platelet reactivity was studied in 12 healthy subjects. Plasma beta-thromboglobulin concentration was determined before and one hour after administration of 100 mg of caffeine, corresponding to one cup of coffee. Mean values were 47.

Prevention of platelet hyperaggregation during coronary angiography.

The authors previously reported an increase in platelet aggregration in the days after coronary angiography, accompanied at times by worrying cardiovascular disorders (ventricular fibrillation in one case, death in two others). In the present study, ten patients received a platelet antiaggregating drug (ticlopidin) 5 days before their coronary angiography. No significant changes were detected in the test for circulating platelet aggregates (CPA test) in these patients.