Xerostomia in Sjogren`s syndrome and lupus erythematosus: a comparative histological and immunofluorescence study of minor salivary glands alterations

Background: Xerostomia is a symptom that can be triggered by chronic diseases such as Sjogren`s syndrome (SS) and lupus erythematosus (LE). Many authors regard most cases of salivary hypofunction in LE to secondary SS. Others believe that salivary changes in patients with LE might reflect a multisystem presentation of the disease. The present study compared histopathological and direct immunofluorescence (DIF) alterations in salivary glands of patients with xerostomia and diagnosis of LE or SS. Methods: Twenty-eight salivary gland biopsies from patients with xerostomia and diagnosed with LE or SS were submitted to histopathological and DIF exams. Results: From the 28 patients, 16 had SS and 12 had LE. In SS, a moderate to intense sialadenitis was detected, with infiltration and destruction of excretory salivary ducts. In LE, mild/moderate sialadenitis with thickening and hyalinization of the ductal basement membrane was observed. DIF revealed that 50% of SS patients presented intercellular ductal IgA deposits, whereas 58% of LE patients showed deposits of IgG in the ductal basement membrane. Conclusions: Alterations in salivary glands of LE patients may be a specific manifestation of the disease (lupus sialadenitis), reflecting its multisystemic presentation, instead of an association of secondary SS and LE.

Two-Year Outcome of Partial Lacrimal Punctal Occlusion in the Management of Dry Eye Related to Sjogren Syndrome

Purpose: To analyze the influence of thermal partial punctal occlusion on the ocular surface of dry eye related to Sjogren syndrome. Material and Methods: Thirty-seven eyes of 19 patients (3 male and 16 female; 49.11 +/- 14.33 years old) with keratoconjunctivitis sicca were enrolled in this study. Superior and inferior partial occlusion were performed in both eyes under topical anesthesia using thermal cautery with a sterile tip to obtain lacrimal punctum smaller than 0.5 mm. Schirmer I, break-up-time, diameter of lacrimal puncta, corneal fluorescein, and rose Bengal staining scores were analyzed before and after 24 weeks and after 24 months of the procedure. All measurements were performed under controlled climate. Results: The average lacrimal punctum diameter before the procedure was 0.65 +/- 0.134 mm. All lacrimal puncta were successfully reduced to less than 0.5 mm after 4 weeks of the procedure. The average Schirmer I test values improved statistically after 24 weeks and maintained stable after 24 months. Average break-up-time, rose Bengal, and fluorescein staining score values improved statistically after 24 weeks and improved even more after 24 months. Average Schirmer I test, break-up-time, rose Bengal, and fluorescein staining scores showed significant improvement (p < 0.0001) after 24 months of partial thermal punctal occlusion. Conclusion: Our study showed that reducing the punctum diameter to 0.5 mm can improve vital staining scores, break-up-time, and Schirmer I test in dry eye related to Sjogren syndrome.

Traditional risk factors for cardiovascular disease in primary antiphospholipid syndrome (APS) when compared with secondary APS: a study with 96 patients

Objective: To evaluate the prevalence of traditional risk factors in patients with primary antiphospholipid syndrome (APS) in comparison to those with systemic lupus erythematosus-secondary APS. Methods: Transversal study of 96 APS patients (Sapporo`s criteria). Demographic and clinical data, cardiovascular risk factors and drug use were investigated. Results: Thirty-nine Primary APS and 57 secondary APS were included. The groups did not differ regarding age (38.5 +/- 9.9 vs. 39.4 +/- 10.5 years, p=0.84) and female gender (84.6 vs. 96.5%, p=0.06), respectively. Arterial events were more observed in primary than secondary APS (59 vs. 36.8%, p=0.04) patients. No difference was seen concerning venous and obstetric events. In regard to traditional risk factors for cardiovascular disease, both groups were comparable related to current or previous smoking, sedentarism, family history for coronary disease, systemic hypertension, diabetes mellitus, overweight and obesity. The frequencies of altered lipid profiles were alike in the two groups, except for a higher prevalence of low HDL-c levels in primary APS group (84.6 vs. 45.5%, p=0.0001). Concerning drug use, no significant differences were observed related to chloroquine and statin use, however the secondary APS patients had a higher rate of prednisone use (10.2 vs. 57.9%, p<0.001) as well as mean dose of corticosteroid (1.5 +/- 5.7 vs. 9.2 +/- 12.5mg/ /day, p=0.0001). Conclusion: Traditional risk factors for cardiovascular disease are present and comparable between patients with primary and secondary APS, except for a high frequency of low HDL-c in primary APS patients.

Ultrasonography in pregnant women with antiphospholipid syndrome using salicylic acid and heparin

The objective of the present study was to evaluate fetal biometry, Doppler values, and perinatal outcomes in pregnant women with antiphospholipid syndrome treated with acetylsalicylic acid and heparin. Twenty-five pregnant women with antiphospholipid syndrome using 100 mg/day acetylsalicylic acid and 5,000 IU heparin every 12 h were evaluated in this prospective observational study. Ultrasonography was performed between 24 and 38 weeks of gestational age to assess estimated fetal weight, placental thickness, amniotic fluid index, fetal biophysical profile and Doppler evaluation of maternal uterine arteries, and fetal middle cerebral and umbilical arteries. Data regarding Apgar score, gender, delivery mode, and birth weight and length were recorded after birth. The observed values for ultrasonographic assessment and perinatal outcomes were not very different from the expected values for normal pregnancies. The birth weight was 2863.3 +/- A 737.7 g (mean +/- A SD) and length was 46.8 +/- A 4.2 cm. Only one newborn (4%) had the 1-min Apgar score < 7 and all had the 5-min Apgar score > 7. Gestational and perinatal evaluation of pregnant women with antiphospholipid syndrome using both acetylsalicylic acid and heparin was reassuring.

Pre-processing for noise detection in gene expression classification data

Due to the imprecise nature of biological experiments, biological data is often characterized by the presence of redundant and noisy data. This may be due to errors that occurred during data collection, such as contaminations in laboratorial samples. It is the case of gene expression data, where the equipments and tools currently used frequently produce noisy biological data. Machine Learning algorithms have been successfully used in gene expression data analysis. Although many Machine Learning algorithms can deal with noise, detecting and removing noisy instances from the training data set can help the induction of the target hypothesis. This paper evaluates the use of distance-based pre-processing techniques for noise detection in gene expression data classification problems. This evaluation analyzes the effectiveness of the techniques investigated in removing noisy data, measured by the accuracy obtained by different Machine Learning classifiers over the pre-processed data.

Adding fever to WHO criteria for diagnosing pneumonia enhances the ability to identify pneumonia cases among wheezing children

Objective To examine the ability of the criteria proposed by the WHO to identify pneumonia among cases presenting with wheezing and the extent to which adding fever to the criteria alters their performance. Design Prospective classification of 390 children aged 2-59 months with lower respiratory tract disease into five diagnostic categories, including pneumonia. WHO criteria for the identification of pneumonia and a set of such criteria modified by adding fever were compared with radio-graphically diagnosed pneumonia as the gold standard. Results The sensitivity of the WHO criteria was 94% for children aged <24 months and 62% for those aged >= 24 months. The corresponding specificities were 20% and 16%. Adding fever to the WHO criteria improved specificity substantially (to 44% and 50%, respectively). The specificity of the WHO criteria was poor for children with wheezing (12%). Adding fever improved this substantially (to 42%). The addition of fever to the criteria apparently reduced their sensitivity only marginally (to 92% and 57%, respectively, in the two age groups). Conclusion The authors' results reaffirm that the current WHO criteria can detect pneumonia with high sensitivity, particularly among younger children. They present evidence that the ability of these criteria to distinguish between children with pneumonia and those with wheezing diseases might be greatly enhanced by the addition of fever.

Kernel machines for epilepsy diagnosis via EEG signal classification: A comparative study

Objective: We carry out a systematic assessment on a suite of kernel-based learning machines while coping with the task of epilepsy diagnosis through automatic electroencephalogram (EEG) signal classification. Methods and materials: The kernel machines investigated include the standard support vector machine (SVM), the least squares SVM, the Lagrangian SVM, the smooth SVM, the proximal SVM, and the relevance vector machine. An extensive series of experiments was conducted on publicly available data, whose clinical EEG recordings were obtained from five normal subjects and five epileptic patients. The performance levels delivered by the different kernel machines are contrasted in terms of the criteria of predictive accuracy, sensitivity to the kernel function/parameter value, and sensitivity to the type of features extracted from the signal. For this purpose, 26 values for the kernel parameter (radius) of two well-known kernel functions (namely. Gaussian and exponential radial basis functions) were considered as well as 21 types of features extracted from the EEG signal, including statistical values derived from the discrete wavelet transform, Lyapunov exponents, and combinations thereof. Results: We first quantitatively assess the impact of the choice of the wavelet basis on the quality of the features extracted. Four wavelet basis functions were considered in this study. Then, we provide the average accuracy (i.e., cross-validation error) values delivered by 252 kernel machine configurations; in particular, 40%/35% of the best-calibrated models of the standard and least squares SVMs reached 100% accuracy rate for the two kernel functions considered. Moreover, we show the sensitivity profiles exhibited by a large sample of the configurations whereby one can visually inspect their levels of sensitiveness to the type of feature and to the kernel function/parameter value. Conclusions: Overall, the results evidence that all kernel machines are competitive in terms of accuracy, with the standard and least squares SVMs prevailing more consistently. Moreover, the choice of the kernel function and parameter value as well as the choice of the feature extractor are critical decisions to be taken, albeit the choice of the wavelet family seems not to be so relevant. Also, the statistical values calculated over the Lyapunov exponents were good sources of signal representation, but not as informative as their wavelet counterparts. Finally, a typical sensitivity profile has emerged among all types of machines, involving some regions of stability separated by zones of sharp variation, with some kernel parameter values clearly associated with better accuracy rates (zones of optimality). (C) 2011 Elsevier B.V. All rights reserved.

A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases

Objective To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry. Methods Inclusion criteria were onset age lower than 18 years and a diagnosis of any idiopathic inflammatory myopathy (IIM) by attending physician. Bohan & Peter (1975) criteria categorisation was established by a scoring algorithm to define JDM and JPM based oil clinical protocol data. Results Of the 189 cases included, 178 were classified as JDM, 9 as JPM (19.8: 1) and 2 did not fit the criteria; 6.9% had features of chronic arthritis and connective tissue disease overlap. Diagnosis classification agreement occurred in 66.1%. Medial? onset age was 7 years, median follow-up duration was 3.6 years. Malignancy was described in 2 (1.1%) cases. Muscle weakness occurred in 95.8%; heliotrope rash 83.5%; Gottron plaques 83.1%; 92% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 74.6% was abnormal in 91.5% and electromyogram performed in 39.2% resulted abnormal in 93.2%. Logistic regression analysis was done in 66 cases with all parameters assessed and only aldolase resulted significant, as independent variable for definite JDM (OR=5.4, 95%CI 1.2-24.4, p=0.03). Regarding treatment, 97.9% received steroids; 72% had in addition at least one: methotrexate (75.7%), hydroxychloroquine (64.7%), cyclosporine A (20.6%), IV immunoglobulin (20.6%), azathioprine (10.3%) or cyclophosphamide (9.6%). In this series 24.3% developed calcinosis and mortality rate was 4.2%. Conclusion Evaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with de, methotrexate combination, was the most indicated treatment.

Ethical preferences for influencing superiors: A 41-society study

With a 41-society sample of 9990 managers and professionals, we used hierarchical linear modeling to investigate the impact of both macro-level and micro-level predictors on subordinate influence ethics. While we found that both macro-level and micro-level predictors contributed to the model definition, we also found global agreement for a subordinate influence ethics hierarchy. Thus our findings provide evidence that developing a global model of subordinate ethics is possible, and should be based upon multiple criteria and multilevel variables. Journal of International Business Studies (2009) 40, 1022-1045. doi:10.1057/jibs.2008.109

Infection rate and risk factors associated with infections related to external ventricular drain

To describe incidence rates and risk factors associated with external ventricular drain (EVD)-related infections at a tertiary Brazilian teaching hospital. The patient cohort consisted of all patients at a major teaching hospital in Brazil with an EVD during the period 1 April 2007 to 30 June 2008 (15 months). Patients were followed up for 30 days after catheter removal. According to the Center for Diseases Control and Prevention criteria for meningitis/ventriculitis, all of the central nervous system (CNS) infections that occurred during this period could be considered to be meningitis or ventriculitis related to EVD placement. Infection rates were calculated using different denominators, such as (1) per patient (incidence), (2) per procedure, and (3) per 1,000 catheter-days (drain-associated infection rate). Patient demographic data, medical history of underlying diseases, antibiotic prophylaxis usage, American Society of Anesthesiologists Score classification, duration of surgery and hospitalization, length of time the EVD was in place, and overall mortality were evaluated during the study period. A logistic regression model was developed to identify factors associated with infection. A total of 119 patients, 130 EVD procedures, and 839 catheter-days were evaluated. The incidence of infection was 18.3%, the infection rate was 16.9% per procedure, and the drain-associated infection rate was 22.4 per 1,000 catheter-days; 77% of the infections were caused by Gram-negative micro-organisms. Only 75% of patients received antibiotic prophylaxis. The infection rate increased with length of the hospital stay. The length of time the catheter was in place was the only independent risk factor associated with infection (p = 0.0369). The incidence of EVD-related infections is high in our hospital, Gram-negative micro-organisms were the most frequent causal agents identified and length of time that the catheter was in place contributed to the infection rate.

DEVELOPING THE INTERNATIONAL SOCIETY OF PHYSICAL AND REHABILITATION MEDICINE (ISPRM): FOLLOWING THROUGH

In 2008 the International Society of Physical and Rehabilitation Medicine (ISPRM) started an initiative to systematically develop its capacity and its internal and external policy agenda. This paper sums up achievements that have been made with this ISPRM initiative as well as pending issues and strategies to address them. The paper treats the following: ISPRM`s policy agenda in collaboration with the World Health Organization (WHO), research capacity in functioning and rehabilitation, ISPRM world conferences, relationships with regional societies of Physical and Rehabilitation Medicine (PRM), and ISPRM`s membership and governance structure.

Juvenile-onset Takayasu arteritis: peculiar vascular involvement and more refractory disease

Objectives: To compare prognosis parameters and arterial site involvement in Takayasu arteritis (TA) patients with disease onset at age <= 18 and >= 21 years. Methods: Sixty-two TA patients [American College of Rheumatology (ACR) and European League Against Rheumatism/Paediatric Rheumatology European Society (EULAR/PreS) criteria] were enrolled consecutively and divided into two groups according to disease onset, and matched for disease duration: juvenile TA patients aged <= 18 years (n = 17) and adult TA patients aged >= 21 years (n = 45). The protocol evaluated the following prognostic factors: aortic insufficiency, ischaemic retinopathy, severe systemic hypertension, and arterial aneurysms. In addition, death and remission [defined as stable disease > 6 months (no complaints without immunosuppressive and prednisone use) and normal erythrocyte sedimentation rate (ESR)] were also analysed. Stenosis and aneurisms were investigated by magnetic angioresonance or arteriography and angiographic classification was defined according to Hata criteria. Results: Mean disease duration was similar in the juvenile and adult TA groups (13.50 +/- 10.73 vs. 13.80 +/- 7.17 years, p = 0.092) and a trend to a lower predominance of female gender in the juvenile TA group was observed (64.71% vs. 88.89%, p = 0.056). The prognosis was distinct in the two groups, with juvenile patients having a lower frequency of disease remission (23.53% vs. 55.56%, p = 0.04) and a significantly higher frequency of aneurism (41.0% vs. 11.1%, p = 0.013). Almost half of the juvenile TA patients had left renal stenosis, a frequency significantly higher than in the adult TA group (41.18% vs. 11.10%, p = 0.013), whereas the stenosis frequency was comparable in all other vascular sites evaluated. No differences were observed between the two groups regarding the frequency of aortic insufficiency, ischaemic retinopathy, severe systemic arterial hypertension, vascular procedures, and mortality. Angiographic classification revealed a similar distribution of arterial involvement in both groups (p > 0.05). Conclusions: Juvenile TA patients have distinct characteristics, with a peculiar renal vascular involvement, the presence of aneurism, and a more refractory disease compared with adult TA patients.

A Consensus on Criteria for Cure of Acromegaly

Objective: The Acromegaly Consensus Group met in April 2009 to revisit the guidelines on criteria for cure as defined in 2000. Participants: Participants included 74 neurosurgeons and endocrinologists with extensive experience of treating acromegaly. Evidence/Consensus Process: Relevant assays, biochemical measures, clinical outcomes, and definition of disease control were discussed, based on the available published evidence, and the strength of consensus statements was rated. Conclusions: Criteria to define active acromegaly and disease control were agreed, and several significant changes were made to the 2000 guidelines. Appropriate methods of measuring and achieving disease control were summarized. (J Clin Endocrinol Metab 95: 3141-3148, 2010)

Report from The International Society for Nomenclature of Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric cardiac disease (Part 1-Procedural nomenclature)

Interventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and on the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the first part of a two-part series. Part 1 will cover the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. This procedural nomenclature of The International Paediatric and Congenital Cardiac Code will be used in the IMPACT Registry (TM) (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry (R) of The American College of Cardiology. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care. While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient`s economic means, with emphasis on excellence in education, research and community service. We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].