1. Ornithine carbamoyl transferases from liver of the dolphin Stenella and the shark Sphyrna zygaena were purified to homogenity and compared for some kinetic and structural properties. 2. The two enzymes showed a specific activity of 211 and 115 respectively. 3. With respect to molecular weight, trimeric subunit structure and Km values, they were alike and similar to enzymes from other species. 4. Both enzymes were thermolable, but they were protected from thermal inactivation in a different way by ornithine and phosphate. 5. The two enzymes focused, respectively, at pH 8.6 and between pH 6.4 and 8.0, the former value being appreciably higher than those of enzymes from other species.

Animals↗

PubMedDate not supplied

Classification of systemic sclerosis.

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Humans↗

PubMedDate not supplied

Low-dose intravenous cyclophosphamide in systemic sclerosis: an open prospective efficacy study in patients with early diffuse disease.

OBJECTIVE: To investigate the efficacy of a treatment with low-dose intravenous cyclophosphamide (CYC) and low-dose prednisone in early diffuse cutaneous systemic sclerosis (dcSSc). METHODS: Patients with dcSSc and a disease duration <24 months consecutively admitted to a tertiary centre underwent a prospective 1-year study. They were treated with i.v. CYC 500 mg/pulses, 10 mg prednisone equivalent, and supportive therapy. Modified Rodnan skin score (mRss), Health Assessment Questionnaire-Disability Index (HAQ-DI), forced vital capacity (FVC), and diffusing lung capacity for CO (DLCO) were assessed as outcome measures. In addition, the nine Medsger severity scale scores were evaluated. RESULTS: mRss and DLCO significantly improved at both 6 (p = 0.002 and 0.012, respectively) and 12 months (p = 0.002 and 0.003, respectively). HAQ-DI showed a nearly significant reduction at 12 months (p = 0.06). Medsger's severity scores also improved for general condition (p = 0.001), peripheral vascular (p = 0.05), skin (p = 0.02), joint/tendon (p = 0.001), muscle (p = 0.05), and lung (p = 0.02). No treatment interruption was needed. CONCLUSIONS: This preliminary study suggests a role for low-dose i.v. CYC in the treatment of early dcSSc. Controlled studies are warranted.

Adult↗

PubMedDate not supplied

[Therapy of systemic sclerosis].

The treatment of the patient with systemic sclerosis has greatly improved in the last ten years, because of two kinds of achievements. A number of drugs have been demonstrated to be active in some disease manifestations like alveolitis, pulmonary hypertension and complicated Raynaud's phenomenon. Some of these drugs namely cyclophosphamide and iloprost await to be confirmed as disease modifying agents. The methodological approach to be made in clinical trials has been defined allowing to correctly analyze the results of published trials and plan future ones.

Humans↗

PubMedDate not supplied

[Epidemiology and risk factors in osteoarthritis: literature review data from "OASIS" study].

Ostheoarthritis (OA) is a social disease characterized by pain, inflammation and stiffness due to an involvement of articular cartilage, soft tissues and bone. OA is the most common rheumatic disease, every age can be affected but prevalence increases dramatically with age with a greater incidence in subjects between 40 and 50 years of age. Hip OA has an important correlation with weight, genetic factors, sex, previous traumas, occupational factors and age. People older than 35 have a prevalence of hip OA of 10.8% that becomes 35.4% in people older than 85. Knee OA has a great correlation with weight ,life style and physical activity. An Italian study demonstrated that the prevalence of this kind of OA is highest in subjects older than 65 that becomes 44% in people older than 80. In this report we explain the results of a study conducted in the South of Italy called the OstheoArtrithis Southern Italy Study (OASIS) that involved 456 doctors and 1782 patients of three different regions. The mean age of these patients was 66.3 years and we evaluated prevalence of hip, knee, hand and spine OA and correlated it to sex, age, weight and BMI. We also evaluated what kind of drugs were used for these patients. Knee OA is the most common subset of OA, the one that requires the highest number of examinations and the one that causes the greatest disability. The most common used drugs are Fans and Coxibs. Condroprotectors were not used much, probably because they are not considered to be very effective.

Adult↗

PubMedDate not supplied

[Atherosclerosis and rheumatoid arthritis: relationships between intima-media thickness of the common carotid arteries and disease activity and disability].

OBJECTIVES: To investigate the intima-media thickness of the common carotid arteries (IMT-CCA) in patients with Rheumatoid Arthritis (RA), and its relationships with classical atherosclerosis risk factors and disease features i.e. duration, activity and disability. METHODS: 48 RA patients (35 F, 13 M; age ed 26-69 years median 55; disease duration 1-18 years, median 8), and 22 controls (16 F, 6 M; age 28-66, median 50) matched for classical atherosclerosis risk factors, i.e. age, sex, smoking, blood pressure, body mass index, diabetes, familiarity, and for postmenopausal status, were studied. IMT and plaques were measured in the left and right common carotid arteries. Serum total cholesterol, triglyceride, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, apoliporotein AI, apolipoprotein B, and rheumatoid factor were determined in patients and controls. The DAS28, the HAQ-DI and disease duration were considered as clinical parameters reflecting disease status. RESULTS: The IMT-CCA (mean +/- SD) was significantly greater in the 48 RA patients than in the 22 controls subjects (1.00+/-0.25 vs. 0.78+/-0.21; p=0.0007). In the 70 subjects investigated CCA-IMT resulted to be significantly correlated with diastolic blood pressure, body mass index, triglyceride and RA status. In the 48 RA patients no correlation was detected with either disease duration or activity or disability. CONCLUSION: our study confirms an increased IMT in RA patients without any clinically evident manifestation of cardiovascular disease. It supports the existence of subclinical atherosclerosis in RA.

Adult↗

PubMedDate not supplied

[HAQ-DI Italian version in systemic sclerosis].

OBJECTIVE: To investigate the Italian version of HAQ-DI (Health Assessment Questionnaire Disability Index) in systemic sclerosis (SSc). METHODS: 121 SSc patients, satisfying ACR criteria for the classification of this disease and consecutively admitted to a tertiary Unit, were invited to participate to the study. The Italian version of HAQ-DI, as validated in rheumatoid arthritis, was administered to each of them. The relationships between this parameter and the following disease aspects: disease subset, wide extent of skin sclerosis, joint contractures, myopathy, active digital ulcers, were investigated. RESULTS: HAQ-DI resulted to be 0.772+/-0.074 (mean+/-SE) Statistically significant differences in HAQ-DI scores were detected between patients with and respectively without wide extent of skin sclerosis (ie modified Rodnan skin score >14) (1.158+/-0.176 vs 0.652+/-0.076; P<0.001), joints contractures (0.839+/-0.076 vs 0.159+/-0.147; P<0.001), myopathy (1.875+/-0.184 vs 0.656+/-0.071; P<0.001), digital ulcers (1.047+/-0.135 vs 0.680+/-0.109; P=0.006). CONCLUSIONS: Our data support the validity of the Italian version of HAQ-DI in SSc.

Disability Evaluation↗

PubMedDate not supplied

[Metabolic syndrome in inflammatory rheumatic diseases].

Toward the end of the last century a better knowledge of cardiovascular (CV) risk factors and their associations led investigators to propose the existence of a unique pathophysiological condition called "metabolic" or "insulin resistance syndrome". Among all, insulin-resistance and compensatory hyperinsulinemia are considered its most important treatment targets. Different definitions have been provided by World Health Organization (WHO) and by The Third Report of The National Cholesterol Education Program's Adult Treatment Panel (NCEP-ATP III). In particular, abdominal obesity, hypertension, low HDL cholesterol and hyperglicemia are the most common items used for its definition. The presence of MetS is effective in predicting the future risk of diabetes and coronaropathies. The evidence of a higher CV risk rate among different rheumatic inflammatory diseases has recently been associated with high prevalence of MetS in some cases. Rheumatoid or psoriatic arthritis have the large series among arthritis, whereas systemic lupus erythematosus among connective tissue disorders. This review analyses all most important studies about the evidence of MetS in rheumatic patients and the main clinical and prognostic significance of this relation.

Antirheumatic Agents↗

PubMedDate not supplied

[Safety of methotrexate in rheumatoid arthritis: a retrospective cohort study in clinical practice].

OBJECTIVE: To evaluate the treatment duration with MTX monotherapy or in association with DMARDs or TNFalpha inhibitors and the incidence and typology of adverse events (AE) occurred in rheumatoid arthritis (RA) patients. METHODS: A retrospective large cohort study of RA outpatients, consecutively seen from January 2000 to June 2005 was performed. Study group were RA patients classified according to the 1984 ACR criteria for the classification of rheumatoid arthritis. The patients were divided in 3 groups according to the treatment regimen: MTX monotherapy, MTX in combination with DMARD or with anti TNFalpha agents. We analyzed 348 therapeutic cycles, 177 of whom using MTX monotherapy. RESULTS: The 224 RA patients accumulated 800 person-years of follow up. Follow up for each of the groups was: MTX monotherapy 479.4 person-years, MTX in combination with DMARDs 244.5, or with TNFalpha inhibitors, 75.7 person-years. From the Kaplan-Meier analysis, the probability of patients remaining on treatment 5 years was 58.5 after starting MTX. The incidence of any AE was 8.87 per 100 person-years. From all, 69 (97.2%) AE were no severe. Among those, more frequently were observed at gastrointestinal tract (31%), liver (19.7%), skin (15.5%). Incidence of severe AE (lung adenocarcinoma, 1 case; pancreatitis, 1 case) was 0.25 per 100 person-years, occurring in patients taking MTX monotherapy or MTX in combination with DMARDs, respectively. CONCLUSIONS: These data confirm that methotrexate is well tolerated in clinical practice in the medium-long term. Nevertheless, the occurrence of severe AE require an accurate vigilance for methotrexate toxicity.

Aged↗

PubMedDate not supplied

Serological and clinical markers of autoimmune disease in HCV-infected subjects with different disease conditions.

OBJECTIVE: To investigate whether the serological markers of autoimmunity and the clinical features of autoimmune disease which occur in hepatitis C virus (HCV)-infected subjects are correlated to each other and/or to the clinical pattern of the disease. METHODS: Seventeen symptom-free, anti-HCV antibody positive subjects, 17 patients with chronic hepatitis C, 21 patients with mixed cryoglobulinemia (MC), and as controls 17 anti-HCV negative patients with dyspepsia were enrolled in a prospective study. A patient history, clinical examination, self-administered questionnaire and laboratory investigations (hepatic enzyme levels, serum HCV-RNA and anti-HCV antibody testing, and serum autoantibody profile) were performed to detect liver and/or autoimmune disease. RESULTS: Serological markers of autoimmunity and clinical findings of autoimmune disease were found to be more frequent in the HCV-infected patients considered as a whole than in controls. However, rheumatoid factor and clinical findings of autoimmune disease were more frequent in MC patients, while anti-smooth muscle antibodies not linked to symptoms or signs of autoimmune disease were detected in all groups of HCV-infected individuals, including healthy carriers and subjects who had recovered from a previous HCV infection. CONCLUSION: Anti-smooth muscle antibodies, a serological marker of autoimmunity, are detectable in HCV-infected subjects whatever their clinical status. Clinical findings of autoimmune disease prevalently occur in patients with mixed cryoglobulinemia.

Adult↗

PubMedDate not supplied

Echographic and biochemical methods for evaluating hydrocephalus and shunting procedures.

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Cerebrospinal Fluid Shunts↗