Evaluation of pediatric patients with јuvenile idiopathic arthritis treated with biological therapy Tocilizumab (Actemra)

Juvenile idiopathic arthritis (JIA) is the most common chronic disease in childhood. It manifests a heterogenic group of symptoms of arthritis, lasting at least 6 weeks and it appears before the age of 16. Patients who had no good therapeutic response to conventional therapy with Methotrexate were treated with biological therapy. The aim of this paper was to evaluate 9 patients who were receiving Tocilizumab at the Department of Rheumocardiology, University Clinic of Pediatric Diseases in Skopje. Materials and methods: Our study included 9 patients treated at our Department with biological therapy with Tocilizumab. Prior to initiation of the biological therapy, all patients underwent laboratory investigations, purified protein derivative (PPD) skin test for tuberculosis, X ray of the lungs and heart, and analysis of hepatitis markers. All patients were treated with amp. Actemra (tocilizumab) 8 mg/kg/tt i.v. Two of the patients had a severe form of the disease (one with severe systemic form and one with severe oligoarticular form of JIA). All presented patients had clinical remission of the disease. Conclusion: Therapy with tocilizumab in patients with juvenile idiopathic arthritis is a good therapeutic choice. The results obtained in our study have shown a significant therapeutic effect of tocilizumab even in severe forms of the disease.

Outbreak of a Systemic Form of Camelpox in a Dromedary Herd (Camelus dromedarius) in the United Arab Emirates

Camelpox virus (CMLV) is the causative agent of camelpox, which frequently occurs in the Old World camelids-rearing countries except for Australia. It has also been described in experimentally inoculated New World camelids. Camelpox outbreaks are often experienced shortly after the rainy season, which occurs twice a year on the Arabian Peninsula because of the increased density of the insect population, particularly mosquitos. A systemic form of camelpox outbreak in seven dromedary camels was diagnosed by histology, virus isolation, and PCR. A phylogenetic analysis using full length CMLV genomes of the isolated CMLV strains showed a single phylogenetic unit without any distinctive differences between them. The United Arab Emirates (UAE) isolate sequences showed phylogenetical relatedness with CMLV isolates from Israel with only minor sequence differences. Although the sequences of viruses from both countries were closely related, the disease manifestation was vastly different. Our study shows that the virulence is not only determined by genetic features of CMLV alone but may also depend on other factors such as unknown aspects of the host (e.g., age, overall fitness), management, and the environment.

Erasmus syndrome presented as CREST syndrome with Scl-70 positivity: a case report

Erasmus syndrome is a rare pathology defined as a systemic sclerosis secondary to contact with silica, associated or not with silicosis. More recent studies have related silica as an environmental factor stimulating different immune responses of the body. This report was made with the objective of presenting a rare case of systemic sclerosis, in a localized cutaneous form, also called CREST syndrome. A patient with a 20-year history of mining developed silicosis, cutaneous calcinosis, Raynauld's phenomenon, esophagopathy, sclerodactyly, telangiectasias and high positivity for the antitopoisomerase I antibody, the most common antibody in the systemic form.

IgA Vasculitis and IgA Nephropathy: Same Disease?

Many authors suggested that IgA Vasculitis (IgAV) and IgA Nephropathy (IgAN) would be two clinical manifestations of the same disease; in particular, that IgAV would be the systemic form of the IgAN. A limited number of studies have included sufficient children or adults with IgAN or IgAV (with or without nephropathy) and followed long enough to conclude on differences or similarities in terms of clinical, biological or histological presentation, physiopathology, genetics or prognosis. All therapeutic trials available on IgAN excluded patients with vasculitis. IgAV and IgAN could represent different extremities of a continuous spectrum of the same disease. Due to skin rash, patients with IgAV are diagnosed precociously. Conversely, because of the absence of any clinical signs, a renal biopsy is practiced for patients with an IgAN to confirm nephropathy at any time of the evolution of the disease, which could explain the frequent chronic lesions at diagnosis. Nevertheless, the question that remains unsolved is why do patients with IgAN not have skin lesions and some patients with IgAV not have nephropathy? Larger clinical studies are needed, including both diseases, with a common histological classification, and stratified on age and genetic background to assess renal prognosis and therapeutic strategies.

Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: a Chinese retrospective cohort study

Background Relapsing polychondritis (RPC) is a rare autoimmune disease and its early diagnosis remains challenging. Defining the clinical patterns and disease course may help early recognition of RPC. Results Sixty-six males and 60 females were included in this study. The average age at onset were 47.1 ± 13.8 years and the median follow-up period was 18 months. Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r = − 0.75, P < 0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%), and patients with Ear pattern and Airway pattern were further divided into limited and systemic form of RPC (27.8% with limited form of Ear pattern and 24.6% with limited form of Airway pattern initially). During follow-up, a minority of patients with Ear pattern and Airway pattern progressed into Overlap pattern, and some Airway-Ear negative pattern patients progressed into Ear pattern. While a large majority of limited RPC patients remained limited form during follow-up, a minority of limited RPC patients progressed into systemic form. Patients with Ear pattern had the highest survival rate and relatively lower inflammatory status. Conclusions RPC patients can be categorized as 4 different clinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement. The clinical patterns and presenting forms may evolve during follow-up. Our findings may facilitate early recognition of this rare disease.

Silicon content in blood and synovial fluid of patients with rheumatoid arthritis: clinical-pathogenetic significance of microelementosis

Background. The prevalence of rheumatoid arthritis (RA) among the world’s population is about 1 %, and among certain populations of people it reaches 5–7 %. The risk factors for the development and high rates of RA progression include high concentrations of quartz (silicon (Si) dioxide) in the inhaled air, the toxic effect of which is considered as one of the most powerful pathogenetic factors of this disease. The purpose of the work was to evaluate the clinical and pathogenetic significance of silicon microelementosis in RA. Materials and methods. Eighty-three patients with RA were examined (22 % were males and 78 % — females aged from 22 to 75 (mean of 45) years). The duration of the disease from the first signs of its manifestation was 9 years. Systemic form of the disease was diagnosed in 19 % of cases, RA seropositive for rheumatoid factor was detected in 68 %, for cyclic citrullinated peptide antibodies — in 80 % of individuals. The content of Si in the blood serum and in 19 patients — in cerebrospinal fluid was determined using an atomic absorption spectrometer SolAAr-Mk2-MOZe with an electrographite atomizer (Great Bri­tain). Using a computer tensiometer PAT2-Sinterface (Germany), the parameters of the surface tension of blood serum and synovial fluid were investigated with a surface life time of 0.01 sec and tending to infinity, and their ratio was calculated. Results. RA is accompanied by the absence of probable changes in the level of silicon. Si microelementosis is associated with the clinical, radiographic and sonographic signs of the disease course, the degree of activity and severity of arthritis, and silicon imbalance is involved in the pathogenesis of the articular syndrome, damage to the ske­letal muscle, the peripheral nervous system, wherein, Si indicators in blood may have prognostic significance.

Tobramycin: efficacy of intensive therapy

One of the leading cause of the decrease of treatment efficacy in patient with community-acquired infections in the intensive care department is a spread of antibiotic resistance in main causative agents. Among Gram-negative microorganisms Pseudomonas aeruginosa is one of the leading causative agents, that is related to health service. A review of foreign and domestic literature concerning tobramycin – systemic form use in the current medical practice in the conditions of increasing resistance of microorganisms to the most broad-spectrum antibiotics is presented in the article. Pharmacokinetic and pharmacodynamic characteristics of tobramycin are described, results of clinical trials, that demonstrate an efficacy of its use in the combination with other antibiotics in the life-threatening infections caused by Gram-negative microorganisms are presented in the article.

Anesthesia Management of Emergent Sternotomy for Acute Cardiac Tamponade in a Pediatric Hematopoietic Stem Cells Recipient

Hematopoietic stem cell transplantation (HSCT) is the infusion of hematopoietic stem and progenitor cells to reconstitute the bone marrow. Due to the long-term consequences of HSCT, anesthetic management of these patients could be complicated. Here the authors describe the clinical features and management of a hemopoietic stem cell recipient with a systemic form of GVHD, who presented with cardiac tamponade due to right ventricular injury, after pericardiocentesis for a severe pericardial effusion, which led to bedside pericardial window and sternotomy with multiple cardiac arrests in the operating room