The prevalence and incidence of systemic lupus erythematosus (SLE) in selected cities from three Commonwealth of Independent States countries (the Russian Federation, Ukraine and Kazakhstan).

We assessed the prevalence and incidence of Systemic Lupus Erythematosus (SLE) in 2010 in adults from four cities in Russia, Kazakhstan and Ukraine. Individuals with SLE were identified retrospectively from the medical records of specialized centers. Prevalent SLE patients were nondeceased city residents, diagnosed prior to December 31, 2010; incident patients were residents newly diagnosed between January 1 and December 31, 2010. Population size was obtained from official census data. The observed prevalence rates (per 100,000, 95% CI) were 9.0 (7.1-11.2) in Kursk and Yaroslavl, Russian Federation; 20.6 (15.4-27.0) in Semey, Kazakhstan; and 14.9 (10.9-19.9) in Vinnitsa, Ukraine. The cumulative incidence rates (per 100,000, 95% CI) were 1.4 (0.7-2.4); 1.6 (0.4-4.1) and 0.3 (0.0-1.8), correspondingly. All rates were higher among females compared to males, and incidence peaked in the population aged 25-44. These rates appear slightly lower than those reported from Western Europe and the USA. This could be because of study design (case-ascertainment), local health care practices or true differences in disease risk. Case age and sex distribution was similar to the known epidemiology of SLE. The rates were highest in Kazakhstan, likely because of a predominantly ethnic Asian population.

Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis.

OBJECTIVES: Myositis-specific autoantibodies (MSAs) may define homogeneous clinical subsets of adult patients with dermatomyositis (DM). Recently, there have been descriptions of novel autoantibodies in DM. This study was conducted to establish the clinical significance of anti-p155/140 autoantibodies in juvenile DM (JDM). METHODS: The first 116 children recruited to the JDM National Registry and Repository (UK and Ireland) were studied. Comprehensive clinical features were recorded and sera screened for anti-p155/140 autoantibodies using radio-immunoprecipitation. Sera from adults with DM (n = 20), PM (n = 25), SSc (n = 150), SLE (n = 40) and healthy subjects (n = 50) were used for comparison. Immunodepletion experiments were used to establish whether the p155/140 kDa targets recognized by JDM sera were the same as adult DM sera. RESULTS: Twenty-seven out of 116 (23%) JDM cases were positive for anti-p155/140 in comparison with 6/20 (30%) adults with DM. Immunodepletion confirmed that the 155/140 kDa proteins recognized by JDM and adult DM sera were the same targets. All other adult control sera were negative for anti-p155/140 autoantibodies. There was a higher frequency of males in the anti-p155/140-positive JDM group (P = 0.02). JDM patients with anti-p155/140 autoantibodies had significantly more cutaneous involvement including Gottron's papules (P = 0.003), ulceration (P = 0.005) and oedema (P = 0.013). The distribution of skin lesions was more extensive particularly periorbitally (P = 0.014) and over the small (P < 0.001) and large joints (P = 0.003). CONCLUSIONS: Anti-p155/140 autoantibodies are clinically significant in JDM and may define a clinical subset in terms of disease severity and outcome. The same autoantigen target is detected in adult DM patients.

Peripheral T-cell lymphoma in a five year old.

We report a case presenting with persistent pyrexia that led to the diagnosis of peripheral T-cell lymphoma, a rare malignancy in childhood. The case illustrates diagnostic conundrums in a patient who is not responding as expected to treatment.

Systemic lupus erythematosus and associated healthcare resource consumption in selected cities from the Russian Federation, Republic of Kazakhstan and Ukraine: the ESSENCE study.

OBJECTIVES: To evaluate healthcare resource (HR) consumption associated with Systemic Lupus Erythematosus (SLE) management in adult patients with active autoantibody positive disease in the Russian Federation, Republic of Kazakhstan, and Ukraine. METHODS: The ESSENCE was a retrospective, observational study, and included data on patients' clinical characteristics and SLE-related HR use (laboratory, biopsy, imaging tests, medications, visits to specialists, outpatient visits, hospitalizations) during 2010 from the 12 specialized rheumatologic centers. RESULTS: A total of 436 SLE patients were included in the analyses, with 232 patients being enrolled in Russia, 110 in Kazakhstan, and 94 in Ukraine. The mean age was 36-42 years and median SLE duration was 3-6.8 years across the countries. Extrapolation to total country population showed that, in 2010, visits to specialists (who assign treatment for organs involved/damaged by SLE) were the most frequently used HR (from 13,439 visits in Kazakhstan to 23,510 in Russia), followed by hospitalizations (from 2,950 in Kazakhstan to 6,267 in Russia) and outpatient visits (from 1,654 visits in Russia to 8,064 in Kazakhstan). Compared to chronic active patients (SLE persistent during last year), patients with relapsing-remitting SLE (at least one flare alternated by one remission per year) had a higher rate of visits to specialists (100% vs 60.8%, p < .001) and hospitalizations (98.9% vs 60.8%, p < .001). Compared to patients without flares, patients experiencing flares had a higher rate of unplanned visits to specialists (86.2% vs 6.3%, p < .001), were more often hospitalized (both ICU and non-ICU) (100.0% vs 50.0%, p < .001), and had a longer duration of ICU hospitalization (25.9 days vs 17.5 days, p < .001). CONCLUSIONS: Specialist visits are the most frequently consumed SLE-related healthcare recourse in the Commonwealth of Independent States (CIS) countries. A relapsing-remitting SLE profile and the occurrence of flares significantly raise healthcare resource consumption.

HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis-scleroderma overlap.

OBJECTIVES: To investigate a large cohort of children with juvenile dermatomyositis (JDM), and those with JDM-scleroderma (JDM-SSc) overlap, using detailed serological analysis, HLA class II genotyping and clinical characterization. METHODS: Children (114) with JDM were recruited, and clinical data collected, through the JDM National Registry and Repository (UK and Ireland). Sera were assayed for ANA using standard immunofluorescence techniques and specific antibodies characterized using ELISA, immunodiffusion and radioimmunoprecipitation. Patients and controls (n = 537) were genotyped at the HLA-DRB1 and DQB1 loci, and then the DQA1 locus data was derived. RESULTS: Over 70% of the patients were ANA-positive. Clear differences in serological and genetic data were demonstrated between JDM and JDM-SSc overlap groups. Strong associations were seen for HLA-DRB1*03 (all cases vs controls, P(corr) = 0.02; JDM-SSc vs controls, P(corr) = 0.001) and HLA-DQA1*05 (all cases vs controls, P(corr) = 0.01; JDM-SSc vs controls, P(corr) = 0.005). The frequency of the HLA-DRB1*03-DQA1*05-DQB1*02 haplotype was significantly increased in the JDM-SSc (P = 0.003) and anti-PM-Scl antibody (P = 0.002) positive groups. All anti-U1-RNP antibody-positive patients had at least one copy of HLA-DRB1*04-DQA1*03-DQB1*03 haplotype. Associations were observed between serology and specific clinical features. CONCLUSIONS: We present clinical data, HLA genotyping and serological profiling on a large cohort of JDM patients and a carefully characterized subset of patients with JDM-SSc overlap. The results confirm known HLA associations and extend the knowledge by stratification of data in serological and clinical subgroups. In the future, a combination of serological and genetic typing may allow for better prediction of clinical course and disease subtype in JDM.

Molecular dynamics simulations to aid the rational design of organic friction modifiers.

Molecular dynamics simulations were performed under conditions of constant volume and temperature and of constant pressure and temperature to elucidate the structure activity relationships of a series of non-ionic surfactant molecules derived from vegetable fat and employed as friction modifiers in commercial engine oils. The simulations show the extent to which intermolecular hydrogen bonding is important in determining the stability of the monolayer formed by the surfactant molecules and show that mono-alkanoyl glyceride molecules are able to pack more efficiently, forming significantly more intermolecular hydrogen bonds and occupying approximately half the volume needed by di-alkanoyl glyceride molecules. Density profiles are presented which show significant mixing of the hydrophobic tail groups and a non-polar solvent. The distribution of torsion angles in the tail groups shows that the conformation is consistent with a liquid at finite temperature rather than a crystal structure. The measured friction coefficients of equimolar solutions of the glycerides show that the efficacy as friction modifiers varies in the order mono-, di- and the tri-oleyl glyceride, which is consistent with the efficacy of film formation predicted by the molecular dynamics calculations.

Standard medical care of patients with systemic lupus erythematosus (SLE) in large specialised centres: data from the Russian Federation, Ukraine and Republic of Kazakhstan (ESSENCE).

OBJECTIVES: To describe disease characteristics and treatment regimens for adult patients with systemic lupus erythematosus (SLE) with autoantibody positive disease in three countries (the Russian Federation, Ukraine and Republic of Kazakhstan). METHODS: The Efficacy and Safety of Subcutaneous Enoxaparin in Non-Q wave Coronary Events (ESSENCE) study was a 1-year, retrospective, multicentre, observational study. Data included patients' characteristics, disease activity and severity, and healthcare resource use in 2010. RESULTS: Twelve centres enrolled 436 eligible patients: 232 in Russia, 110 in Kazakhstan and 94 in Ukraine. Mean age ranged from 36 to 42 years and median SLE duration from 3 to 6.8 years. According to study definitions, 69.2% of patients in Russia, 72.7% in Kazakhstan and 55.4% in Ukraine had severe disease at diagnosis. SLE activity (Nasonova classification, 1972) decreased from diagnosis to the last visit in 2010 in all countries. At the last visit, mean (SD) Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index score was 13.8 (10.5) in Russia, 19.4 (16.9) in Kazakhstan and 7.2 (6.8) in Ukraine, and Systemic Lupus International Collaborative Clinics/American College of Rheumatology damage index was 2.0 (2.2), 3.3 (3.2) and 2.2 (2.0), respectively. Treatment regimens included predominantly glucocorticoids (96.7-99.1%), immunosuppressants or cytotoxic drugs, for example, azathioprine and cyclophosphamide (20.7-53.2%), and antimalarial drugs (18.3-40.8%). CONCLUSIONS: The study provides reliable insight into the SLE clinical profiles in the referenced countries. Patients were 4-10 years younger in the study and had 3-7 years shorter SLE duration than in Western European countries and both SLE activity and severity were higher with higher rate of hospitalisations, but decreased during treatment. Local and international scales demonstrated correlation in SLE activity and organ damage evaluation. There were differences in clinical characteristics and healthcare features across the countries.

Making the most of your time: the benefits of converting patient education programs into continuing nursing education.

Educating hospital staff and patients is a time-consuming, yet essential component to a successful bariatric surgery program. The process of converting patient education and support group sessions into continuing education opportunities for hospital staff is described. This also provides hospital staff with direct exposure to patient testimony of concerns and obstacles to following treatment plans.

Care of the obesity surgery patient requiring immediate-level care or intensive care.

Most bariatric surgery patients are triaged directly to the medical surgical floor postoperatively. However, patients at high risk due to comorbid factors, who have failed postoperative extubation or have suffered intraoperative complication, may require intensive care unit (ICU) or intermediate-level care (IMC). The special needs of the morbidly obese IMC/ICU patient include: triage, mobility, visiting, fluid resuscitation, management of sleep apnea, airway management, transporting for out of ICU procedures, and preventing pressure ulcers. Traditional approaches to nursing care require new thought when dealing with the massively obese. Our experiences with the special needs of these critically ill morbidly obese bariatric surgery patients are described.

Acute, severe self-limiting dysautonomia and hypertensive encephalopathy.

We describe two unrelated children with almost identical clinical illnesses comprising of severe, burning dysesthesia, allodynia, hypertensive encephalopathy, and laboratory evidence of both sympathetic and parasympathetic autonomic disturbance after a nonspecific viral illness. No underlying etiology was identified. Both patients displayed complete resolution of their clinical and radiologic findings after a number of months, and there was no recurrence over a follow-up period of 17 months to 4 years. Treatment of the patients' dysesthesias proved difficult, requiring multiple analgesics and intensive physiotherapy. We speculate that their illnesses may represent a pure autonomic variant of Guillain-Barré syndrome.

Ulnar peripheral nerve stimulation by train-of-four technique in adult nonmedicated patients.

BACKGROUND: Peripheral nerve stimulation is necessary to quantify the level of neuromuscular blockade and prevent prolonged paralysis related to drug accumulation. Some nurses and physicians are hesitant to administer nerve stimulation because of concerns about inflicting pain on the patient. OBJECTIVE: To describe the feeling associated with train-of-four ulnar nerve stimulation, and to quantify discomfort, monitor heart rate response, and define the amount of current necessary to stimulate thumb adduction. METHODS: Healthy, nonmedicated volunteer subjects (N = 39) were asked to describe train-of-four ulnar nerve monitoring at 3 current strengths. Heart rate was monitored throughout the testing procedure. The milliamperes delivered at each current strength and the occurrence of thumb adduction were recorded. RESULTS: Subjects described nerve stimulation generally as an unusual prickly sensation. On a discomfort scale of 1 to 10, the mean discomfort score when stimulated with the current setting at 4 (15.5-23.6 mA) was 3.63. Level 4 stimulation produced thumb adduction in 54% of subjects. No heart rate change occurred in response to nerve stimulation. CONCLUSION: Nerve stimulation by train-of-four method was moderately uncomfortable but not painful. Heart rate response could not be relied on as a measurement of discomfort. Protocols for stimulation should include testing at level 4 and increasing as necessary to cause thumb adduction.

Thoracoscopy: nursing implications for optimal patient outcomes.

Many patients who once were considered for open chest procedures now benefit from examination and treatment of pleural disease via thoracoscopy. The thoracoscopist enters the chest cavity through small incisions through the chest wall. Procedures are performed through a video assisted thoracoscope (much like a bronchoscope or endoscope). Wound care, maintenance of the chest drainage system, pain management and vigilant assessment of the cardiorespiratory system following the procedure are integral nursing interventions when caring for these patients.

Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis.

OBJECTIVE: The identification of novel autoantibodies in juvenile dermatomyositis (DM) may have etiologic and clinical implications. The aim of this study was to describe autoantibodies to a 140-kd protein in children recruited to the Juvenile DM National Registry and Repository for UK and Ireland. METHODS: Clinical data and sera were collected from children with juvenile myositis. Sera that recognized a 140-kd protein by immunoprecipitation were identified. The identity of the p140 autoantigen was investigated by immunoprecipitation/immunodepletion, using commercial monoclonal antibodies to NXP-2, reference anti-p140, and anti-p155/140, the other autoantibody recently described in juvenile DM. DNA samples from 100 Caucasian children with myositis were genotyped for HLA class II haplotype associations and compared with those from 864 randomly selected UK Caucasian control subjects. RESULTS: Sera from 37 (23%) of 162 patients with juvenile myositis were positive for anti-p140 autoantibodies, which were detected exclusively in patients with juvenile DM and not in patients with juvenile DM-overlap syndrome or control subjects. No anti-p140 antibody-positive patients were positive for other recognized autoantibodies. Immunodepletion suggested that the identity of p140 was consistent with NXP-2 (the previously identified MJ autoantigen). In children with anti-p140 antibodies, the association with calcinosis was significant compared with the rest of the cohort (corrected P < 0.005, odds ratio 7.0, 95% confidence interval 3.0-16.1). The clinical features of patients with anti-p140 autoantibodies were different from those of children with anti-p155/140 autoantibodies. The presence of HLA-DRB1*08 was a possible risk factor for anti-p140 autoantibody positivity. CONCLUSION: This study has established that anti-p140 autoantibodies represent a major autoantibody subset in juvenile DM. This specificity may identify a further immunogenetic and clinical phenotype within the juvenile myositis spectrum that includes an association with calcinosis.

An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM).

OBJECTIVE: To develop revised criteria for the diagnosis of juvenile dermatomyositis (JDM) using an international consensus process. METHODS: An initial survey was circulated to members of the Network for JDM and the Paediatric Rheumatology International Trials Organisation (PRINTO). Each individual was asked to identify those criteria that were felt to be most helpful in the diagnosis of classical JDM. A second survey was derived from these results and used to rank these proposed criteria in order of their importance and usefulness in clinical practice. RESULTS: The first survey had a response rate of 49.8% (118 individuals) from 92 centres in 32 countries. All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. Muscle biopsy, magnetic resonance imaging (MRI) and changes on the electromyogram (EMG) were deemed important diagnostic criteria. Other criteria, including myositis-specific or -related antibodies, nailfold capillaroscopy, factor VIII-related antigen, muscle ultrasound, calcinosis and neopterin, were used by 35.3% of respondents. Seventy-eight respondents to the first survey (66%) responded to the second survey. Typical MRI and muscle biopsy changes were rated by all to be the most useful clinically relevant diagnostic criteria after proximal muscle weakness, characteristic skin rash and elevated muscle enzymes. These were followed by myopathic changes on EMG, calcinosis, dysphonia and nailfold capillaroscopy, which were ranked equally. CONCLUSION: This process identified nine criteria that clinicians felt to be helpful or important in the diagnosis of JDM. A further process of refinement and validation is necessary to agree an internationally acceptable, clinically usable set of diagnostic criteria.