Clinical presentation and management of atypical hemolytic uremic syndrome in Latin America: a narrative review of the literature.

INTRODUCTION: Comprehensive information about atypical hemolytic uremic syndrome (aHUS) is relatively scarce outside of Europe and North America. This narrative review assembles available published data about the clinical presentation and management of aHUS in Latin America. AREAS COVERED: A search conducted in February 2023 of the MEDLINE (from inception), Embase (from inception), and LILACS/IBECS (1950 to 2023) databases using search terms 'atypical hemolytic uremic syndrome' and 'Latin America' and their variations retrieved 51 records (full papers and conference abstracts) published in English, Spanish, or Portuguese. After de-duplication, manual screening of titles/abstracts and addition of author-known articles, 25 articles were included of which 17 (68%) are full papers. All articles were published during the years 2013-2022. Articles include cohort studies, a registry analysis, and case reports from Argentina, Brazil, Chile and Columbia. Overall, Latin American patients with aHUS present the classic epidemiological, clinical, and genetic characteristics associated with this condition as described in other world regions. Depending on the country and time of reporting, aHUS in Latin America was treated mainly with plasma therapy and/or eculizumab. Where reported, eculizumab substantially improved aHUS-related outcomes in almost all adult and pediatric patients. EXPERT OPINION: Eculizumab has dramatically altered the natural course of aHUS, improving prognosis and patient outcomes. Addressing economic challenges and investing in healthcare infrastructure will be essential to implement strategies for timely detection and early treatment of aHUS in Latin America.

Germination of small tropical seeds has distinct light quality and temperature requirements, depending on microhabitat.

The coexistence of plant species in tropical rainforests is related to specific abiotic resources, varying according to the occurrence microhabitat of each species. Light quality is the main abiotic factor influencing germination of small seeds; however, studies often do not discriminate its effect from that of light irradiance. This study compared specific requirements for seed germination of ten small-seeded species, with restricted occurrence in only one of three contrasting microhabitats: forest understorey, edge of clearings and open areas. Laboratory experiments were carried out to test temperature regime (constant or fluctuating), light quality (R:FR) and light irradiance (PAR), which reproduce high and low conditions commonly found in the microhabitats. Seed germination of all species occurred between 20 and 30 °C, only seeds of open area species were able to germinate at 35 °C and no species required alternating temperatures to germinate. Irrespective of species and microhabitat, a decrease in the R:FR reduced the germination percentage; however, there were differences in the capacity to germinate at low R:FR. The values of R:FR50% were higher for open area and edge species (0.441-0.345) than for understorey species (0.181-0.109), with few exceptions. For all species and most of the tests, germination was not influenced by PAR. Light quality is the most important light signal for germination of small seeds; irradiance has little effect. Our results suggest two distinct patterns of germination for small-seeded species: open area and edge species are light-demanding and require high R:FR to germinate, while understorey species are shade-tolerant and germinate at low R:FR. These differences are responsible for distinct microhabitat occurrence and help to explain the coexistence of species in tropical forests.

Germination, viability and dormancy of 47 species from threatened tropical montane grassland in southeast Brazil: Implications for ex situ conservation.

To mitigate anthropogenic impacts on plant diversity in tropical montane grasslands, one of the most threatened ecosystems in Brazil, it will be essential to develop ex situ conservation strategies to preserve wild species. The lack of basic research on the seed storage behaviour of grassland species may, however, limit their use for reintroduction and restoration projects. We investigated seed storage behaviour at the community level by comparing the effects of cold-low RH (10 °C; 10% RH) and freezing-low RH (20 °C; 10% RH) conditions on seed viability, germination and dormancy of 47 species. Fresh seeds of 43% of the species showed primary dormancy. More than half of the species showed high seed survival responses (viability >60%) under both storage temperatures. Despite a variety of dormancy responses among the different species, the low RH storage conditions tested released dormancy for most species during 12- and 30-month storage times. Multivariate analysis of the best (freezing-low RH, 30 months) storage condition evidenced the formation of five distinct groups, three with species having high conservation potential in seed banks. Although further studies are needed to test dormancy-breaking treatments and improve seed conservation practices, this first approach to assessing seed banking techniques could contribute to demand for locally adapted seeds for ecological restoration projects in tropical montane grasslands.

Tubulinopathies.

Mutations causing dysfunction of the tubulins and microtubule-associated proteins, otherwise known as tubulinopathies, are a group of recently described entities, that lead to complex brain malformations. An understanding of the fundamental principles of operation of the cytoskeleton and compounds in particular microtubules, actin, and microtubule-associated proteins, can assist in the interpretation of the imaging findings of tubulinopathies. Somewhat consistent morphological imaging patterns have been described in tubulinopathies such as dysmorphic basal ganglia-the hallmark (found in 75% of cases), callosal dysgenesis, cerebellar hypoplasia/dysplasia, and cortical malformations, most notably lissencephaly. Recognizing the common imaging phenotypes present in tubulinopathies can prove invaluable in directing the genetic workup for a patient with brain malformations.

An Energy-Efficient Approach to Enhance Virtual Sensors Provisioning in Sensor Clouds Environments.

Virtual sensors provisioning is a central issue for sensors cloud middleware since it is responsible for selecting physical nodes, usually from Wireless Sensor Networks (WSN) of different owners, to handle user's queries or applications. Recent works perform provisioning by clustering sensor nodes based on the correlation measurements and then selecting as few nodes as possible to preserve WSN energy. However, such works consider only homogeneous nodes (same set of sensors). Therefore, those works are not entirely appropriate for sensor clouds, which in most cases comprises heterogeneous sensor nodes. In this paper, we propose ACxSIMv2, an approach to enhance the provisioning task by considering heterogeneous environments. Two main algorithms form ACxSIMv2. The first one, ACASIMv1, creates multi-dimensional clusters of sensor nodes, taking into account the measurements correlations instead of the physical distance between nodes like most works on literature. Then, the second algorithm, ACOSIMv2, based on an Ant Colony Optimization system, selects an optimal set of sensors nodes from to respond user's queries while attending all parameters and preserving the overall energy consumption. Results from initial experiments show that the approach reduces significantly the sensor cloud energy consumption compared to traditional works, providing a solution to be considered in sensor cloud scenarios.

Good Outcomes in Kidney Transplantation With Deceased Donor With Acute Kidney Injury: Donor's Age and Not Acute Kidney Injury Predicts Graft Function.

BACKGROUND: With the increased demand for kidney transplants and the short supply of organs, it is necessary to have a better strategy to evaluate the available organs, especially from donors with acute kidney injury (AKI), because these organs are often rejected for transplantation. METHODS: We evaluated patients undergoing transplantation with kidneys from deceased donors with AKI. The cases were divided into AKI stages according to the Acute Kidney Injury Network (AKIN) criteria. The outcomes examined were delayed graft function (DGF), creatinine (Cr), and creatinine clearance (CrCl) at 6 months after transplantation. RESULTS: We evaluated 101 patients and included 53 in the final model. There was no statistical difference in the demographic characteristics, comorbidities, and immunosuppression according to each AKIN stage, showing a population of homogeneous transplant recipients. Recipients in AKIN stages I, II, and III, respectively had DGF in 72.7%, 61.9%, and 71.4% of cases; Cr of 1.6 ± 0.5, 1.7 ± 0.7, and 1.6 ± 0.2 mg/dL at 6 months; and CrCl of 60.6 ± 22.4, 52.4 ± 27.4, and 52.03 ± 12.1 mL/min at 6 months. Each additional year in donor age increased the relative risk of DGF by 1.08 (1.0-1.13) (P = .01), and organs from older donors were associated with worse renal function at 6 months. CONCLUSION: Kidney transplantation of organs from deceased donors with AKI showed greater DGF but good outcomes. Donor age was the only characteristic that correlated with outcome.

Methods of analysis for peritubular capillaritis and glomerulitis in acute renal rejection: capillaritis in management of routine diagnosis.

Glomerulitis and peritubular capillaritis have been recognized as important lesions in acute renal rejection (AR). We studied glomerulitis and peritubular capillaritis in AR by 2 methods and investigated associations with C4d, type/grade of AR, and allograft survival time. Glomerulitis was measured according to Banff scores (glomerulitis by Banff Method [gBM]) and by counting the number of intraglomerular inflammatory cells (glomerulitis by Quantitative Method [gQM]). Capillaritis was classified by the Banff scoring system (peritubular capillaritis by Banff Method [ptcBM]) and by counting the number of cells in peritubular capillaries in 10 high-power fields (hpf; peritubular capillaritis by Quantitative Method [ptcQM]). These quantitative analyses were performed in an attempt to improve our understanding of the role played by glomerulitis and capillaritis in AR. The g0 + g1 group (gBM) associated with negative C4d (P = .02). In peritubular capillaritis, a larger number of cells per 10 hpf in peritubular capillaries (ptcQM) were observed in positive C4d cases (P = .03). The group g2 + g3 (gBM) correlated with graft loss (P = .01). Peritubular capillaritis was not significantly related to graft survival time. Our study showed that the Banff scoring system is the best method to study glomerulitis and observed that the evaluation of capillaritis in routine biopsies is difficult and additional studies are required for a better understanding of its meaning in AR biopsy specimens of renal allografts.

Acute antibody-mediated rejection of renal transplant associated with cellular crescents: first case report.

Acute antibody-mediated rejection is characterized by histological abnormalities such as glomerulitis, capillaritis, or thrombosis associated with presence of C4d and specific anti-donor antibodies. Reports on the association of glomerular injuries with cellular crescents in antibody-mediated rejection are not found in the literature. We report a unique case of antibody-mediated rejection associated with cellular crescents and suggest that such histological abnormality should be considered in the differential diagnosis of acute antibody-mediated rejection.

Seguimento psicológico para agressores domésticos de crianças: protocolo de atendimento psicoterápico breve / Follow-up counseling for aggressors of domestic children: brief psychotherapy protocol / Tratamiento psicológico de agresores domésticos de niños: protocolo de psicoterapia breve

Pessoas que sofreram violência doméstica na primeira infância carregam sequelas emocionais, que se perpetuam em diferentes níveis. Isso representa um alto risco de repetição, quer no âmbito familiar, quer servindo de mola propulsora para a violência social, perpetuando-se um ciclo de violência que constitui uma epidemia no Brasil. Em especial porque na primeira infância ocorre a construção da autoestima e da visão de mundo, a partir das sensações experimentadas e das relações vivenciadas. Propor e reforçar a importância de um tratamento psicoterápico para o agressor doméstico de crianças trata-se de uma ação pioneira no Brasil, e constitui o objetivo do presente trabalho. Foram acompanhados 98 agressores em núcleo específico para esse fim. O recrutamento se deu por demanda espontânea e contou com uma rede social de apoio para divulgação na comunidade. O estudo revelou que tratar o agressor é uma alternativa viável e muito eficiente no que tange a proteger a própria vítima. Por meio dessa intervenção, objetiva-se contribuir para tentar interromper de forma eficaz o ciclo de repetição de violência que muitas famílias carregam.

People who have experienced domestic violence in their early infancy retain negative emotional experiences that continue to influence their lives in different levels. This may represent a high risk of violence repetition, either within the family environment or catalyzing social violence, perpetuating a cycle of violence that is epidemic in Brazil. This happens mainly because at that age, from zero to six years of age, the child is building up his or her self-esteem as well as their view of the outside world, based upon the sensations experienced and upon their personal relations. Proposing and enhancing a psychotherapeutic treatment for the offender as a more positive approach in fighting domestic violence against children is a pioneer effort in Brazil and was the goal of this study. Ninety-eight aggressors were followed-up in the core for this specific purpose. Recruitment took place spontaneously and had a community outreach social support network. The study showed that treating the offender is a viable alternative to benefit the victim more effectively than only treating the victim him/herself, as well as involving the victim in the aggressor's treatment. This intervention aims at contributing to effectively try to stop the repetitive cycle of violence that many families go through.

Las personas que sufren la violencia doméstica en la primera infancia llevan secuelas emocionales que se perpetúan en los diferentes niveles. Esto representa un alto riesgo de repetición, sea en la familia sea sirviendo como catalizador de la violencia social, perpetuando un ciclo de violencia, que constituye una epidemia en Brasil. En particular, porque la primera infancia es la construcción de la autoestima y de la visión del mundo, a partir de las sensaciones experimentadas y de las relaciones vividas. Proponer y reforzar la importancia de un tratamiento psicoterapéutico para el agresor de niños es un esfuerzo pionero en Brasil y constituyó el objetivo del estudio. Noventa y ocho agresores fueron seguidos en el centro para este propósito específico. El reclutamiento se llevó por la búsqueda espontánea y tenía una red de apoyo social para convencer la comunidad. Esto estudio reveló que el tratamiento del agresor es una alternativa viable y muy eficiente cuando se trata de proteger a la víctima. Esta intervención tiene como objetivo contribuir de forma efectiva para detener el ciclo de repetición de la violencia que muchas familias llevan.

Impact of oral problems on daily activities of HIV-infected children.

AIM: Oral manifestations are common in HIV+ children, but the impact of these diseases on their daily life is unknown. So the aim of this study was to assess the impact of oral problems on the daily activities of HIV+ children. METHODS: The Child-OIDP-B was used with 59 10-12 year-old HIV+ children, who were outpatients at two public hospitals for HIV treatment in Rio de Janeiro, Brazil. Caries, biofilm and gingival bleeding indexes were recorded. The Kruskall-Wallis and Mann-Whitney tests as well as the Spearman's correlation coefficient were used for analysis. Statistical evaluation: Replies were analysed using the Statgraphics ® Plus Version 5.0 statistics software system, in order to obtain comparative diagrams and graphs using the ANOVA multifactorial system. RESULTS: The Child-OIDP-B scores ranged from 0 to 30, (mean=6.09) and 71.2% of the children were affected by oral problems. Association was found between oral impact and number of caries (p=0.009). Children receiving HAART therapy had a Child-OIDP-B score (4.87), much lower than those who were not (8.87) (p=0.038). The most reported oral impact of the disease was eating (55.6%), but oral wounds were the most prevalent type of lesions (76.3%). As regards the level of intensity of the impact, moderate severity was prevalent in all 59 children and 66.1% reported that oral impacts affected 1-4 daily activities, 50.8% of all children were not satisfied with their appearance and oral health; 23.7% perceived the impact of HIV-infection on general health. CONCLUSION: Most children suffered the impact of oral problems on their daily activities, mainly functional impacts.

Role of peritubular capillaries and vascular endothelial growth factor in chronic allograft nephropathy.

OBJECTIVE: To investigate the role of peritubular capillary damage and vascular endothelial growth factor (VEGF) in chronic allograft injury and to evaluate their correlation with clinical factors. PATIENTS AND METHODS: The study included 56 patients who underwent transplantation between 1987 and 2004 and experienced chronic graft dysfunction. CD34 (peritubular capillaries) and VEGF were evaluated at histologic analysis. Patients were classified into 3 groups: 47 with chronic allograft injury, 9 with pure cyclosporine toxicity, and 26 who served as the control group (time 0 biopsy). RESULTS: Compared with the control group, CD34 total expression in chronic nephropathy was indirectly proportional to Banff stage (P < .05), and VEGF was increased in chronic allograft injury grade I or II or nephrotoxicity (P < .05). CD34 expression was correlated with age (P < .007) and number of acute rejection episodes (P = .005). A negative correlation was observed between expression of CD34 and of VEGF (P < .001). Low expression of CD34 was associated with risk of graft loss of 1.45 (95% confidence interval, 1.15-7.24; P = .04). CONCLUSION: Peritubular capillaries decreased progressively with development of chronic allograft injury. The VEGF demonstrated a bimodal behavior, increasing at the onset of nephropathy and decreasing in the final stages. Loss of peritubular capillaries was associated with worse graft survival and overexpression of VEGF.

Molecular imaging in hereditary forms of parkinsonism.

The development of in vivo molecular imaging to evaluate the dopamine (DA) system with positron-emission tomography and single photon emission computed tomography has been of key importance on monitoring in vivo nigrostriatal neuronal loss in Parkinson's disease (PD), mostly through assessments of pre- and post-synaptic DA receptors. The discoveries of genes related to hereditary forms of parkinsonism (PARK1, PARK2, PARK6, PARK7 and PARK8) have increased our understanding either of distinct subtypes of clinical expression in PD or its etiology. This article revises current data on molecular neuroimaging of genetic forms of parkinsonism comparing and contrasting its main features with the classical sporadic forms. Awareness of the spectrum variance in the genotype and its respective PD phenotype are useful to distinguish different pathophysiological mechanisms of PD.

On-line mercury and methylmercury pre-concentration by adsorption of their dithiophosphoric acid diacyl ester chelates on a C18 column and cold-vapor atomic-absorption detection.

The pre-concentration of mercury(II) and methylmercury by adsorption of their dithiophosphoric acid diacyl ester (DDTP) chelates on a C18 column, then detection with cold-vapor atomic-absorption spectrometry was investigated. Conditions such as sample pH, reductant and chelating agent flow and concentration, and eluent and carrier gas flow were optimized. Optimization was performed by use of evolutionary operation with a proper factorial design. At a sample flow of 5.3 mL min(-1) and a loading time of 4.5 min, column adsorption efficiency ranged from 88 to 93% for both species. Detection limits down to 10 ng L(-1) were obtained at a sample throughput of 12 h(-1). There was good agreement between found and certified values in the analysis of certified reference materials after their microwave-assisted mineralization with HNO3 and H2O2.

Alcohol-sensitive hereditary essential myoclonus with dystonia: a study of 6 Brazilian patients.

We present the clinical profile of a group of patients with myoclonus and dystonia sensitive to alcohol and address these cases in the context of essential myoclonus. Six patients from 4 families were selected: 4 men and 2 women with myoclonus affecting predominantly the arms. Active movements of these segments elicited the dystonic and myoclonic movements. A marked improvement with alcohol intake was seen. Laboratory findings including EEG, SSEP, and cranial CT and MRI were normal. Surface EMG recording showed bursts with duration of 30-112 ms in 3 patients. One patient showed a triphasic recording pattern (agonist-antagonist-agonist) of ballistic type. Our findings suggest that the myoclonus-dystonia disorder is present in Brazilian patients.

Complex partial seizures and aphasia as initial manifestations of non-ketotic hyperglycemia. Case report.

We describe a case of non-ketotic hyperglycemia (NKH), heralded by complex partial seizures and aphasia of epileptic origin, besides versive and partial motor seizures. This clinical picture was accompanied by left fronto-temporal spikes in the EEG. The seizures were controlled by carbamazepine only after the control of the diabetes. A month later, carbamazepine was discontinued. The patient remained without seizures, with normal language, using only glybenclamide. Complex partial seizures, opposed to simple partial seizures, are rarely described in association to NKH. Epileptic activity localized over language regions can manifest as aphasia.

[Stereotaxic thalamotomy and pallidotomy with computerized planning in Parkinson's disease: short-term evaluation of motor function in 50 patients]. / Talamotomia e palidotomia estereotáxica com planejamento computadorizado no tratamento da doença de Parkinson. Avaliação do desempenho motor a curto prazo de 50 pacientes.

We evaluated the motor function of 50 patients with Parkinson's disease, who underwent stereotaxic surgery with computerized planning, without ventriculography (ventrolateral thalamotomy- VLT- and/or posteroventral pallidotomy- PVP) before and one month after surgery. 27 unilateral TVL, 10 unilateral PVP, 6 bilateral PVP, and 7 TVL with PVP were performed. The motor evaluation was performed with the Unified Parkinson's Disease Rating Scale, motor score, during on and off periods. We observed a global motor improvement in all groups. The improvement of dyskinesias was obtained in the contralateral side of the body, in the PVP groups. From the 50 patients, 16 (32%) presented post-operative complications, 9 of these (56.25%) improved completely, 6 (37.25%) improved partially, and 1 (6.25%) did not improve during the first month. These results were considered satisfactory, and a long term analysis will show whether these benefits are long lasting or not.