918 resultados para ACE Basin (S.C.)--Juvenile literature


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Infections are an important cause of morbidity and mortality in juvenile systemic lupus erythematosus (JSLE). Among them, invasive aspergillosis (IA), which is usually related to immunosuppressed patients, has been rarely reported in JSLE. From 1983 to 2011, 5604 patients were followed at our institution and 283 (5%) met the American College of Rheumatology (ACR) classification criteria for SLE. Six (2.1%) of our JSLE patients had IA. One of them was previously reported and five will be described herein. Four of them were female. The median age at JSLE diagnosis was 12 years (8-16) and the median interval between diagnosis of JSLE and IA was 6 months (1-38). All had pulmonary involvement and three of them had systemic involvement. The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) was 19 (7-22). Diagnosis of IA was performed by isolation of Aspergillus spp., two in bronchoalveolar lavage culture and by way of autopsy in the others. All of them were treated with corticosteroids and/or immunosuppressive drugs at IA diagnosis (azathioprine and/or intravenous cyclophosphamide). They all required treatment in the pediatric intensive care unit with mechanical ventilation and antifungal therapy (fluconazole, amphotericin B, itraconazole and/or voriconazole); nonetheless, none of them survived. In conclusion, this was the first report that evaluated the prevalence of IA in a large population of JSLE patients from a tertiary pediatric hospital, and clearly showed the severity of the outcome, especially in patients with active disease and treated with immunosuppressive agents. This study reinforces the importance of early diagnosis and treatment with certain antifungals, especially in critically ill patients. Lupus (2012) 21, 1011-1016.

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We compared outcomes of alveolar hemorrhage (AH) in juvenile (JSLE) and adult onset SLE (ASLE). From 263 JSLE and 1522 ASLE, the AH occurred in 13 (4.9%) and 15 (1.0%) patients, respectively (p < .001). Both groups had comparable disease duration (2.6 +/- 3.0 vs. 5.6 +/- 7.0 years, p = .151) and median SLEDAI scores [17.5 (2 to 32) vs. 17.5 (3 to 28), p = 1.000]. At AH onset, a higher frequency of JSLE were already on a high prednisone dose ( > 0.5 mg/kg/day) compared to ASLE (54% vs. 15%, p = .042). The mean drop of hemoglobin was significantly lower in JSLE (2.9 +/- 0.9 vs. 5.5 +/- 2.9 g/dL, p = .006). Although treatments with methylprednisolone, plasmapheresis, intravenous immunoglobulin and cyclophosphamide were similar in both groups (p > .050), regarding outcomes, there was a trend in high frequency of mechanical ventilation use (85% vs. 47%, p = .055) and also significant mortality (69% vs. 13%, p = .006) in JSLE compared to ASLE. The sepsis frequency was comparable in both groups (50% vs. 27%, p = .433). We have identified that AH in JSLE has a worse outcome most likely related to respiratory failure. The AH onset in JSLE already treated with high-dose steroids raises the concern of inadequate response to this treatment and reinforces the recommendation of early aggressive alternative therapies in this group of patients. Lupus (2012) 21, 872-877.

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We examined the factors controlling the variability in water-column respiration rates in Amazonian rivers. Our objectives were to determine the relationship between respiration rates and the in situ concentrations of the size classes of organic carbon (OC), and the biological source (C-3 and C-4 plants and phytoplankton) of organic matter (OM) supporting respiration. Respiration was measured along with OC size fractions and dissolved oxygen isotopes (delta O-18-O-2) in rivers of the central and southwestern Amazon Basin. Rates ranged from 0.034 mu mol O-2 L-1 h(-1) to 1.78 mu mol O-2 L-1 h(-1), and were four-fold higher in rivers with evidence of photosynthetic production (demonstrated by delta O-18-O-2<24.2 parts per thousand) as compared to rivers lacking such evidence (delta O-18-O-2>24.2 parts per thousand; 1.35 +/- 0.22 vs. 0.30 +/- 0.29 mu mol L-1 h(-1)). Rates were likely elevated in the former rivers, which were all sampled during low water, due to the stimulation of heterotrophic respiration via the supply of a labile, algal-derived substrate and/or the occurrence of autotrophic respiration. The organic composition of fine particulate OM (FPOM) of these rivers is consistent with a phytoplankton origin. Multiple linear regression analysis indicates that [FPOC], C:N-FPOC ratios, and [O-2] account for a high amount of the variability in respiration rates (r(2) = 0.80). Accordingly, FPOC derived from algal sources is associated with elevated respiration rates. The delta C-13 of respiration-derived CO2 indicates that the role of phytoplankton, C-3 plants, and C-4 grasses in supporting respiration is temporally and spatially variable. Future scaling work is needed to evaluate the significance of phytoplankton production to basin-wide carbon cycling.

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Malakoplakia is a rare chronic granulomatous disease of unknown cause. It is thought to be caused by an acquired bactericidal defect of macrophages. Malakoplakia is associated with chronic infections and immunosuppression. Although it occurs mainly in the urinary tract, it has already been reported in almost every organ system. The isolation of bacteria, especially Escherichia coli, is common in malakoplakia patients. Here, we present a case of primary cutaneous malakoplakia in a kidney transplant recipient who had been taking prednisone, tacrolimus, and mycophenolate. Culture of a lesion grew Burkholderia cepacia complex. Treatment with high doses of trimethoprim-sulfamethoxazole was successful. We also present a systematic review of the literature, identifying 4 previously reported cases of malakoplakia after renal transplantation under similar immunosuppressive therapy, most occurring in the urinary tract or perineum and following benign courses to cure. Data in the literature suggest that malakoplakia has become even rarer since changes were made in the immunosuppressive therapy employed after kidney transplantation.

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Trichomycterus anhanga is described from the Amazon basin, northern Brazil. The species is diagnosed by the latero-sensory system which is restricted to LL1 and LL2, the pectoral fin with two branched rays, the absence of pelvic fins and girdle, the reduced jaws and pharyngeal dentition, the presence of six to seven interopercular odontodes, the absence of a lateral series of spots, the presence of a small dark spot on the ventral surface of the mandibular symphysis, the narrow comma-shaped palatine, the absence of procurrent rays anterior to the dorsal and anal fins, the position of insertion of the first dorsal-fin pterygiophore and the presence of a single pair of pleural ribs. Trichomycterus anhanga shares with T. hasemani and T. johnsoni a wide cranial fontanel which occupies most of the skull roof. Miniaturization as well as synapomorphies for the T. hasemani group are discussed.

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A new armored catfish species of the genus Hypostomus is described from the upper rio Xingu basin, Mato Grosso State, Brazil. The new species is diagnosed from congeners by having high number of teeth (58 to 101, mean 77 on premaxilla, and 58 to 105, mean 80 on dentary), dark spots over body and fins, and abdomen mostly naked. The new species is known from the rapids of the rio Culuene, where it is sympatric with Hypostomus faveolus.

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Objectives The aim of the present paper is to assess the influence of demographic, muscle enzymes, JDM scores and treatment on non-adjuvanted influenza A H1N1/2009 vaccine immunogenicity in juvenile dermatomyositis (JDM) patients. Methods Thirty JDM patients and 81 healthy age-matched controls were vaccinated. All participants were evaluated pre- and 21 days post-vaccination and serology for anti-HI NI was performed by haemagglutination inhibition assay. Muscle enzymes, JDM scores and treatment were evaluated before and after vaccination. Adverse events were reported. Results After immunisation seroconversion rates were significantly lower in JDM patients compared to age-matched controls (86.7 vs. 97.5%, p=0.044), whereas seropmtection (p=0.121), geometric mean titres (GMT) (p=0.992) and factor increase (FI) in GMT (p=0.827) were similar in both groups. Clinical and labomtorial evaluations revealed that JDM scores and muscle enzymes remained stable throughout the study (p>0.05). A higher frequency of chronic course was observed in non-seroconverted compared to seroconverted (100% vs. 27%, p=0.012). Regarding treatment, a lower rate of seroconversion was observed in patients under prednisone>20mg/day (50% vs. 4%, p=0.039), and in those treated with a combination of prednisone, methotrexate and cyclosporine (50% vs. 4%, p=0.039). Local and systemic vaccine adverse events were mild and similar in patients and controls (p>0.05). Conclusion This study identified that chronic course and immunosuppressive therapy are the major factors hampering seroconversion was JDM, suggesting that a specific protocol may be required for this subgroup of patients. In spite of that, a single dose of non-adjuvanted influenza A/H1N1 2009 vaccine was generally seroprotective in this disease with no evident deleterious effect in disease itself (ClinicalTrials.gov, no. NCT01151644).

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Crenicichla chicha, new species, occurs in clear, fast-running waters with rocky substrates in the rio Papagaio and tributaries. It is distinguished from all other Crenicichla species by the combination of two character states: infraorbitals 3 and 4 co-ossified (vs. separated) and 66-75 scales in the row immediately above to that containing the lower lateral line (E1 row scales). Crenicichla chicha shares a smooth preopercular margin, co-ossification of infraorbitals 3 and 4, and some color features with C. hemera from the adjacent rio Aripuana drainage, rio Madeira basin. It differs from Crenicichla hemera in more E1 scales (6675 vs. 58-65) and presence of a conspicuous black narrow stripe running from infraorbital 3 obliquely caudoventrad toward the preopercular margin vs. a rounded and faint suborbital marking present on infraorbitals 3-4. Examination of the type series and additional material from the rio Aripuana confirms that Crenicichla guentheri Ploeg, 1991 is a junior subjective synonym of C. hemera Kullander, 1990.

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Kawasaki disease (KD) is a common vasculitis in childhood. To the authors' knowledge, only one case of juvenile systemic lupus erythematosus (JSLE)-like onset mimicking KD and another case of KD and JSLE association have previously been described. However, the prevalence of this association of the two diseases was not reported. Therefore, over 27 consecutive years, 5419 patients were followed at the Pediatric Rheumatology Unit and 271 (5%) of them met the ACR classification criteria for JSLE. Two (0.7%) of them were female. These also had KD according to European League against Rheumatism / Paediatric Rheumatology European Society (EULAR/PReS) consensus criteria and are described in this report. One case was a 13-year-old who presented all six KD criteria. Echocardiogram showed pericardial effusion, dilatation and tortuosity of right and left coronary, and her symptoms promptly improved after treatment with intravenous immunoglobulin (IVIG). Lupus diagnosis was established a few days later. Another case was a 4-year-old who had also met all six KD criteria, with improvement after IVIG, and lupus diagnosis was made 1 year later. In conclusion, the frequency of the association between these two autoimmune diseases was rare. The occurrence of a second autoimmune systemic disease in a patient with a history of KD should also be considered. Furthermore, the initial presentation of lupus may mimic KD. Lupus (2012) 21, 89-92.

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This study is aimed at evaluating the sublethal effects of endosulfan (EDS) in juvenile common carp (Cyprinus carpio). For this purpose, fish were exposed for 15 days to the technical EDS (95% pure) diluted in dimethyl sulfoxide (DMSO) 0.1% of the total volume in water solution in a semi-static system at sublethal concentration (1 mu g/L). Subsequently, the liver somatic index (LSI) and factor condition (K) were determined. The total cytocrome P450 (CYP), CYP1A isoform, and the ethoxyresorufin-O-deethylase (EROD) activity were determined from the hepatic microsomal fraction as well as the activity of the oxidative stress enzyme system such as superoxide dismutase (SOD), catalase (CAT), glutathione-S-transferase (GST), glutathione peroxidase (GP(X)), glutathione reductase (GR), and glucose-6-phosphate dehydrogenase (G6PDH). Among the parameters assessed, EDS at the sublethal concentration in subchronic exposure caused significant changes in liver somatic indices as well as induction of the phase I biotransformation system and oxidative stress in juvenile common carp (Cyprinus carpio). Thus, it is seen that the use of biochemical biomarkers of environmental contamination in this study proved to be an extremely important tool for detecting the adverse effects of xenobiotics in the aquatic environment, even at low concentration.

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Compartmentalization is a prerequisite to understand large wetlands that receive water from several sources. However, it faces the heterogeneity in space and time, resulting from physical, chemical and biological processes that are specific to wetlands. The Pantanal is a vast seasonally flooded continental wetland located in the centre of South America. The chemical composition of the waters that supply the Pantanal (70 rivers) has been studied in order to establish a compartmentalization of the wetland based on soil-water interactions. A PCA-based EMMA (End-Members Mixing Analysis) procedure shows that the chemistry of the rivers can be viewed as a mixture of 3 end-members, influenced by lithology and land use, and delimiting large regions. Although the chemical composition of the end-members changed between dry and wet seasons, their spatial distribution was maintained. The results were extended to the floodplain by simple tributary mixing calculation according to the hydrographical network and to the areas of influence for each river when in overflow conditions. The resulting map highlights areas of high geochemical contrast on either side of the river Cuiaba in the north, and of the rivers Aquidauana and Abobral in the south. The PCA-based treatment on a sampling conducted in the Nhecolandia, a large sub region of the Pantanal, allowed the identification and ordering of the processes that control the geochemical variability of the surface waters. Despite an enormous variability in electrical conductivity and pH, all data collected were in agreement with an evaporation process of the Taquari River water, which supplies the region. Evaporation and associated saline precipitations (Mg-calcite, Mg-silicates K-silicates) explained more than 77% of the total variability in the chemistry of the regional surface water sampling.

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Executive dysfunction is reported in juvenile myoclonic epilepsy (JME). However, batteries employed in previous studies included no more than three tests of executive function. In this study, we aimed to assess executive and attentional functions in JME using a comprehensive battery of eight tests (encompassing fifteen subtests). We also evaluated neuropsychological profiles using a clinical criterion of severity and correlated these findings with epilepsy clinical variables and the presence of psychiatric disorders. We prospectively evaluated 42 patients with JME and a matched control group with Digit Span tests (forward and backward), Stroop Color-Word Test, Trail Making Test, Wisconsin Card-Sorting Test, Matching Familiar Figures Test and Word Fluency Test. We estimated IQ with the Matrix Reasoning and Vocabulary subtests of the Wechsler Abbreviated Intelligence Scale. The patients with JME showed specific deficits in working memory, inhibitory control, concept formation, goal maintenance, mental flexibility, and verbal fluency. We observed attentional deficits in processes such as alertness and attention span and those requiring sustained and divided attention. We found that 83.33% of the patients had moderate or severe executive dysfunction. In addition, attentional and executive impairment was correlated with higher frequency of seizures and the presence of psychiatric disorders. Furthermore, executive dysfunction correlated with a longer duration of epilepsy. Our findings indicate the need for comprehensive neuropsychological batteries in patients with JME, in order to provide a more extensive evaluation of attentional and executive functions and to show that some relevant deficits have been overlooked. (C) 2012 Elsevier Inc. All rights reserved.

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Carbon supported Pt-Sn catalysts were prepared by reduction of Pt and Sn precursors with formic acid and characterized in terms of structure, morphology and surface properties. The electrocatalytic activity for ethanol oxidation was studied in a direct ethanol fuel cell (DEFC) at 70 degrees C and 90 degrees C. Electrochemical and physico-chemical data indicated that a proper balance of Pt and Sn species in the near surface region was necessary to maximize the reaction rate. The best atomic surface composition, in terms of electrochemical performance, was Pt:Sn 65:35 corresponding to a bulk composition 75:25 namely Pt3Sn1/C. The reaction products of ethanol electro-oxidation in single cell and their distribution as a function of the nature of catalyst were determined. Essentially, acetaldehyde and acetic acid were detected as the main reaction products; whereas, a lower content of CO2 was formed. The selectivity toward acetic acid vs. acetaldehyde increased with the increase of the Sn content and decreased by decreasing the concentration of the reducing agent used in the catalyst preparation. According to the recent literature, these results have been interpreted on the basis of ethanol adsorption characteristics and ligand effects occurring for Sn-rich electrocatalysts. (C) 2012 Elsevier Ltd. All rights reserved.

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Objective To evaluate the perinatal outcomes in hydropic fetuses with congenital microcystic pulmonary lesions that underwent percutaneous, invasive, laser therapy. Method This retrospective study reviews the literature and our experience between 2004 and 2010. Characteristics of the cystic lung lesions, liquor volume (presence of polyhydramnios or not), localization of ablation (vascular vs interstitial) and gestational age at which the procedure was performed were related to outcome (survival). Results In total, 16 fetuses with congenital lung lesions underwent invasive percutaneous laser ablation, seven performed in our center and nine published cases. Survival rate was higher in fetuses with a subsequent postnatal diagnosis of bronchopulmonary sequestration (87.5%) compared with congenital adenomatoid malformation (28.6%; p?=?0.04). The technique of vascular ablation was more successful (100%) than interstitial ablation (25.0%, p?<?0.01). Conclusion Percutaneous vascular laser ablation seems to be effective for bronchopulmonary sequestration in hydropic fetuses. Outcomes were worst following interstitial ablation for microcystic congenital adenomatoid with hydrops. (C) 2012 John Wiley & Sons, Ltd.

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This manuscript reports an uncommon case of inferior third molar facial abscess with purulent secretion drainage through the left external acoustic meatus. The patient's left external acoustic meatus was filled with a purulent secretion observed on a CT scan. He underwent surgery to drain the facial abscess. Despite facial abscesses being routine occurrences, the literature does not contain many case reports of odontogenic facial abscesses with drainage via the external acoustic meatus. These situations occur in two possible ways: multiple fissures in the anterior wall of the cartilaginous portion of the external acoustic meatus; and congenital defects that are occasionally present in the anterior-superior aspect of the external acoustic meatus, known as the foramen of Huschke, which allow communication between the external acoustic meatus and mandibular fossa. These defects may also predispose the patient to the spread of the infection or tumour from the external auditory canal to the infratemporal fossa and vice versa. No otological sequelae were observed in this case. The authors conclude that the hypothesis of bone malformation cannot be excluded, and affirm that any facial abscess requires appropriate and immediate treatment for adequate resolution, by removing the causal factor and providing systemic support.