Manifestaciones renales en pacientes con síndrome de Alagille: Reporte de casos y revisión de la literatura.

El síndrome de Alagille es una condición autosómica dominante que se define clínicamente por alteraciones de cinco sistemas principales: escasez de ductos biliares con colestasis crónica, compromiso cardíaco (estenosis pulmonar), óseo (vétebras en mariposa), oftálmico (embriotoxon posterior) y de la cara (fascies dismórficas leves pero reconocibles). La afectación renal es común en estos pacientes; sin embargo, no hace parte de los criterios que definen el síndrome. Reportamos los casos de 3 pacientes con síndrome de Alagille y compromiso renal y realizamos una revisión de la literatura para establecer la importancia de incluir la evaluación de este sistema en el diagnóstico del síndrome. Concluimos que el compromiso renal es frecuente, y por lo tanto sugerimos que en todos los casos se evalúe la posibilidad de compromiso renal tanto a nivel estructural como funcional glomerular y tubular.

Some biological data on cetaceans populations present in the western coasts of Ireland

Ireland’s waters constitute one of the richest habitats for cetaceans in Europe. Marine mammals, particularly cetaceans, are known to be definitive hosts of digestive parasites from the Fm.Anisakidae. The main aim of this study is to collect and compile all the information available out there regarding parasites of the Fm. Anisakidae and their definitive hosts. Secondary objectives are to relate the presence of cetacean species with the presence of parasites of the Fm. Anisakidae and to determine whether this greater number of cetaceans relates to a greater level of parasitism. Prevalence and burdens of anisakids in definitive hosts vary widely with host species, geographic location, and season. Results from several post-mortem exams are given. However, they cannot be compared due to differences in collecting techniques. Anisakis simplex is the most commonly and widespread parasite found in the majority of the samples and in a major number of hosts, which include harbour porpoise, short-beaked common dolphin and bottlenose dolphin. Studies on harbour porpoise obtained prevalences of Anisakis spp. of 46% (n=26) and of 100% (n= 12). Another study in common dolphin reported a prevalence of 68% (n=25). Several reasons could influence the variations in the presence of Anisakis. Studies on commercially exploited fish have reported prevalences of Anisakis simplex ranging from 65-100% in wild Atlantic salmon and from 42-53.4% in Atlantic cod

Subglacial bedform evidence for a major palaeo-ice stream and its retreat phases in Amundsen Gulf, Canadian Arctic Archipelago

Ascertaining the location of palaeo-ice streams is crucial in order to produce accurate reconstructions of palaeo-ice sheets and examine interactions with the ocean-climate system. This paper reports evidence for a major ice stream in Amundsen Gulf, Canadian Arctic Archipelago. Mapping from satellite imagery (Landsat ETM+) and digital elevation models, including bathymetric data, is used to reconstruct flow-patterns on southwestern Victoria Island and the adjacent mainland (Nunavut and Northwest Territories). Several flow-sets indicative of ice streaming are found feeding into the marine trough and cross-cutting relationships between these flow-sets (and utilising previously published radiocarbon dates) reveal several phases of ice stream activity centred in Amundsen Gulf and Dolphin and Union Strait. A large erosional footprint on the continental shelf indicates that the ice stream (ca. 1000 km long and ca. 150 km wide) filled Amundsen Gulf, probably at the Last Glacial Maximum. Subsequent to this, the ice stream reorganised as the margin retreated back along the marine trough, eventually splitting into two separate low-gradient lobes in Prince Albert Sound and Dolphin and Union Strait. The location of this major ice stream holds important implications for ice sheet-ocean interactions and specifically, the development of Arctic Ocean ice shelves and the delivery of icebergs into the western Arctic Ocean during the late Pleistocene. Copyright (C) 2006 John Wiley & Sons, Ltd.

The 'Shatton and Windup' Affair: Beckett's dealings with the firm of Chatto & Windus, 'Wholesale, Retail and for Exportation'

This essay looks in detail at the brief history of Samuel Beckett's relations with Charles Prentice and the publishing firm of Chatto & Windus. It examines the fate of two of Beckett's early publications - his essay on Proust in the Dolphin Books and his volume of short stories More Pricks than Kicks - against the backdrop of the cultural, ideological and economic context of publishing in the 1930s.

Consensus Statement: Chromosomal Microarray Is a First-Tier Clinical Diagnostic Test for Individuals with Developmental Disabilities or Congenital Anomalies

Chromosomal microarray (CMA) is increasingly utilized for genetic testing of individuals with unexplained developmental delay/intellectual disability (DD/ID), autism spectrum disorders (ASD), or multiple congenital anomalies (MCA). Performing CMA and G-banded karyotyping on every patient substantially increases the total cost of genetic testing. The International Standard Cytogenomic Array (ISCA) Consortium held two international workshops and conducted a literature review of 33 studies, including 21,698 patients tested by CMA. We provide an evidence-based summary of clinical cytogenetic testing comparing CMA to G-banded karyotyping with respect to technical advantages and limitations, diagnostic yield for various types of chromosomal aberrations, and issues that affect test interpretation. CMA offers a much higher diagnostic yield (15%-20%) for genetic testing of individuals with unexplained DD/ID, ASD, or MCA than a G-banded karyotype (similar to 3%, excluding Down syndrome and other recognizable chromosomal syndromes), primarily because of its higher sensitivity for submicroscopic deletions and duplications. Truly balanced rearrangements and low-level mosaicism are generally not detectable by arrays, but these are relatively infrequent causes of abnormal phenotypes in this population (<1%). Available evidence strongly supports the use of CMA in place of G-banded karyotyping as the first-tier cytogenetic diagnostic test for patients with DD/ID, ASD, or MCA. G-banded karyotype analysis should be reserved for patients with obvious chromosomal syndromes (e.g., Down syndrome), a family history of chromosomal rearrangement, or a history of multiple miscarriages.

Ecology and conservation of bottlenose dolphins in Madeira Archipelago, Portugal

This study aimed to provide an insight on the ecology of the bottlenose dolphin population in Madeira archipelago. To achieve this, population structure; group dynamics, site-fidelity, residency and movement patterns within and out of the study area; survival and abundance estimates and spatial and temporal distribution and habitat preferences related to physiographic parameters using data collected between 2001-2011, were investigated. Photo-identification data analysis revealed strong evidences that bottlenose dolphins seen in the archipelago of Madeira belong to an open population with regular recruitment of new animals to the area. This population exhibited a typical fission-fusion society, in which short-term acquaintances prevail, with only a few long-lasting associations. Photo-identification methods demonstrated that there is a large variability in residency pattern, with resident, transient and migrant individuals. Only a small number of dolphins were found to be resident (4.3%). Social network diagram as well as SLAR analysis supported the existence of a mixed population of residents, migrants and transients. Mark-recapture methods estimated a high survival rate, within the range of other long-lived cetacean species. The resident community is composed of app. 180 individuals. In addition, around 400 dolphins of different residency patterns were found to use the south area of Madeira Island. Spatial distribution indicated that bottlenose dolphins were regularly found in shallow and closer to shore areas, suggesting the existence of biological processes influenced by bathymetry. Moreover, temporal patterns revealed no strong seasonal fluctuation in the presence of bottlenose dolphins in Madeira archipelago waters. Bottlenose dolphins are listed under the Annex II of the EU habitats Directive that requires the designation of Special Areas of Conservation (SACs) for this species; as such, the knowledge gained through this work can be used by governmental authorities to the establishment and management of areas for the conservation of bottlenose dolphin in Madeira archipelago.

Quantificação de Fases Cristalinas de Incrustações em Colunas de Produção de Petróleo pelo Método Rietveld

The scale is defined as chemical compounds from inorganic nature, initially soluble in salt solutions, which may precipitate accumulate in columns of production and surface equipment. This work aimd to quantify the crystalline phases of scale through the Rietveld method. The study was conducted in scale derived from columns production wells in development and recipients of pigs. After collecting samples of scale were performed the procedure for separations of inorganic and organic phase and preparation to be analyzed at the X-ray Laboratory. The XRD and XRF techniques were used to monitor whether identifying and quantifying crystalline phases present in the deposits. The SEM technique was used to visualize the morphology of the scales and assess their homogeneity after the milling process. XRD measurements were performed with and without milling and with or without the accessory spinner. For quantify crystalline phases the program DBWStools was used. The procedure for conducting the first refinement was instrumental in setting parameters, then the structural parameters of the phases in the sample and finally the parameters of the function profile used. In the diffraction patterns of samples of scale observed that the best measures were those that passed through the mill and used the accessory spinner. Through the results, it was noted that the quantitative analysis for samples of scale is feasible when need to monitor a particular crystalline phase in a well, pipeline or oil field. Routinely, the quantification of phases by the Rietveld method is hardwork because in many scale was very difficult to identify the crystalline phases present

Gross and microscopic observations on the lingual structure of the franciscana (Pontoporia blainvillei-gervais and d'orbigny, 1844)

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Etnobiologia do boto cinza (Sotalia guianensis, van Bénéden, 1864) por comunidades pesqueiras do Rio Grande do Norte, Basil

Ethnobiology studies Local Ecological Knowledge (LEK) as well as the use and management of natural resources by local communities in order to understand how the environment is perceived, known and classified by human groups. In fishing communities, LEK adds empirical information about the biology of aquatic species and complements scientific findings, especially when it is difficult to obtain factual information during studies on cetaceans, whose behavior is essentially underwater. Cetaceans (whales and dolphins) are constantly threatened by human activities, especially by accidental capture of small coastal species, as in the case of the estuarine dolphin (Sotalia guianensis), object of this study. Ethnobiological researches in fishing communities are of great importance and can clarify aspects of the biology and conservation of this species. Although extensively studied throughout its distribution range, there are still gaps in the knowledge about S. guianensis. Therefore, fishers local ecological knowledge becomes an additional tool to get and confirm information about S. guianensis. This study evaluated the LEK of artisanal fishers who are daily exposed to local population of S. guianensis, through the use of semistructured interviews (N=116). The interviewed fishers were asked about the biology and popular classification of S. guianensis and about possible interactions between this dolphin and them. The studied communities were located in Tibau do Sul (n=39), Pipa (n=36) and Baía Formosa (n=41), all on the south coast of Rio Grande do Norte, Brazil. The study was based on the assumption that differences in fishers LEK among those communities would be caused by both the variety of fishing environments (lagoon/estuary and ocean) and the intensity of tourism activities. Fishers knowledge is expressive and differed among the studied communities depending on the topic investigated. Fishers correctly reported the habitat, distribution, seasonality and behavioral particularities of S. guianensis. Tourism mainly affected the naming of the species. The study results also suggest that marine fishers have greater knowledge about the species than the estuarine/lagoon ones. Local populations accumulate empirical knowledge according to their environment. Hence, it is important to take into account both empirical knowledge and popular participation in management systems, in order to maintain information sharing among communities

Tensão superficial estática de soluções aquosas com óleos minerais e vegetais utilizados na agricultura

O trabalho teve como objetivo avaliar a tensão superficial estática de soluções aquosas com formulações de óleos minerais e vegetais emulsionáveis utilizados como adjuvantes na agricultura. Os óleos minerais e vegetais, quando adicionados aos produtos fitossanitários, podem imprimir características desejáveis à calda de pulverização, como reduzir a tensão superficial em soluções aquosas, possibilitar maior contato da calda com a superfície vegetal ou reduzir o potencial de deriva durante as pulverizações. Foram testados os seguintes produtos comerciais: óleos minerais (Assist, Attach, Dytrol, Iharol, Mineral Oil, Spinner, Sunspray-E e Triona) e óleos vegetais (Agrex'oil Vegetal, Crop Oil, Natur'l Óleo, Óleo Vegetal Nortox e Veget Oil), todos com registro de uso na agricultura. A tensão superficial das soluções aquosas foi avaliada em 11 concentrações para cada produto (0,025; 0,05; 0,1; 0,25; 0,5; 0,75; 1,0; 1,5; 2,0; 2,5 e 3,0% v/v). Essa propriedade dos óleos minerais e dos óleos vegetais foi estimada medindo-se a massa das gotas formadas na extremidade de uma bureta. Ao conjunto de dados obtidos para cada produto, na avaliação da tensão superficial, foram determinadas as análises de variância e de regressão, ajustando-se os dados ao Modelo de Mitscherlich. Entre os óleos minerais, destacaram-se os produtos: Assist, Dytrol, Iharol e Mineral Oil por apresentarem as menores tensões superficiais mínimas estimadas pelo Modelo, respectivamente, 29,255; 28,442; 26,097 e 28,584 mN m-1. Os óleos vegetais que apresentaram os menores valores de tensão superficial mínima estimados pelo Modelo, foram: Agrex' oil Vegetal (27,716 mN m-1), Natur'l óleo (28,216 mN m-1), Veget Oil (27,308 mN m-1) e Crop Oil (29,964 mN m-1).

Ecologia alimentar do boto cinza, sotalia guianensis (van banédén, 1864), no litoral do rio grande do norte (rn)

This study investigated factors that influence the group size during the behavior foraging of estuarine dolphin Sotalia guianensis in the coast south of the state Rio Grande do Norte (RN), it also characterized the diet of estuarine dolphins and elaborated an otoliths catalog for aid in the identification of the preys found in the stomach contents. In relation to the group size during behaviour foraging, inside of the Curral Bay, larger frequency of solitary hunt was observed. Factors as tide variation, group composition and seasons didn't present correlation with the number of animals observed during a feeding episode. Capture success for participant was shown significantly larger when the animals hunted alone, what possibly explains the largest frequency of solitary hunt inside of the Curral Bay. About diet, were identified 18 bone fishes species, with predominance of species the families Haemulidae and Sciaenidae and five cephalopods species, including two new species in the diet of Sotalia guianensis in Brazil. Our results indicated that estuarine dolphin in the coast oriental from Rio Grande do Norte (RN) feeds predominantly of fish that form shoal, of habitat estuarino and producing of sounds. The analysis of the images, otoliths of 43 species of coastal fish of the coast of the state of Rio Grande do Norte (RN), aided by the offered descriptions, demonstrated to be an effective methodology for the knowledge of those structures, as well as it represented a form of reducing the subjectivity in the identification of the bone fishes found in stomach content

Boto cinza (Sotalia guianensis, Van Benédén, 1864) (Cetacea, Delphinidae): Atividade aérea, forrageio e interações inter-específicas, na Praia de Pipa (Tibau do Sul RN) e estudo comparativo entre duas populações do Nordeste do Brasil

Sotalia guianensis is a small cetacean of the Delphinidae family, with coastal habits and whose area of distribution ranges from Florianópolis (27º35'S, 48º34'W), in Brazil, to Honduras (15º58'N, 85º42'W). At Pipa beach, on the south coast of RN state, in Brazil, the species occur throughout the year. The present study was carried out in two bays, which are bordered by cliffs. The animals were monitored from vantage points, using the "Ad libitum" and "all the occurrences" methods; during the years of 1999 and 2004. The study was divided in 4 chapters: Behavioral standards of two populations of gray dolphin, (Sotalia guianensis, Van Benédén, 1864) in the northeast of Brazil; Aerial activity of the gray dolphin: its possible function and the influence of environmental and behavioral factors; The influence of daily and monthly variation of the tides, of the period of the day and group size on the gray dolphin forage activity; kleptoparasitism interactions of frigatebird (Fregata magnificens, Mattheus, 1914) during the gray dolphin forage activity. The results have shown that the gray dolphin has a varied and complex behavioral repertoire. The leap is the most frequent behavior; the aerial activity is diffuse during daylight and is influenced by some factors, such as the level of the tide and social factors. The gray dolphin, when in the bay, most frequently feeds isolate or in small groups. The forage is diffuse during daylight; however, being more frequent in the morning and is influenced by the daily and monthly variation of the tide. At Pipa beach, kleptoparasitarian interactions were registered between the gray dolphin and the frigatebird (Fregata magnificens). The frigatebird forage strategy consists basically of two ways: to fly over great extensions searching for dead fish and to steal food (kleptoparasitism). These interactions were predominantly carried out between immature and female adult birds and adult and immature dolphins, and occurred during daylight. The present study can be considered an initial landmark to a better knowledge on the gray dolphin surface behavior, especially regarding the aerial behavioral repertoire and forage strategy of this species. However, it is necessary to continue these studies, so that we can understand better the complex social life of these animals and thus create effective measures for its conservation

Comportamento social de botos-cinza, sotalia guianensis, na praia de Pipa, RN, Brasil: dinâmica, sequência, sincronia e respostas ao turismo de observação

Social behavior of Guiana dolphins, Sotalia guianensis, at Pipa Beach, RN, Brazil: dynamics, sequence, breathing synchrony, and responses to dolphin watching. Social animals form groups that can range from temporary to permanent. Depending on the nature of the social relationships developed between individuals, groups present a particular social organization and the effect of these interactions shapes the activity patterns of these animals. This study investigates: (i) fission-fusion dynamics of Guiana dolphins, through the analysis of three dimensions of the social system (variation in spatial cohesion, variation in size and composition of groups), (ii) sequence, routine and behavioral stability, (iii) breathing intervals in synchronized groups and (iv) behavioral responses of the animals to dolphin watching. Systematic observations of Guiana dolphins were made from a platform located in cliffs about 25 m above sea level that surround Madeiro Bay, Pipa Beach. Sampling occurred from December 2007 to February 2009 between 0600 h and 1600 h, and the groups of Guiana dolphins were investigated according to their size (alone and group) and composition (adults, adults and juveniles, and adults and calves). According to the analysis of fission-fusion dynamics, Guiana dolphin groups frequently changed their composition, modifying their patterns of spatial grouping and cohesion every 20 minutes on average. More than 50% of the individuals maintained a distance of up to 2 m from other group members and new individuals were attracted to the group, especially during feeding, leaving it for foraging. Large groups were more unstable than small, while groups containing only adults were more stable than groups of adults and juveniles. According to the Z-score analysis to investigate the sequence and behavioral routine, lone individuals were more ! .7! ! involved in foraging and feeding, while resting was more common in groups. Foraging and feeding were more common in homogeneous groups (individuals of the same age class), while heterogeneous groups (different age classes) were often involved in socialization, displaying a broader behavioral repertoire. Foraging and resting behavior presented higher stability (continuous duration in minutes) than the other behaviors. The analysis of breathing intervals in synchronized groups showed significant differences depending on type of behavior, composition and area preference. During resting, breathing intervals were of longer duration, and groups with calves showed shorter breathing intervals than groups without calves. Lone individuals also preferred areas called corral , often used for the entrapment of fishes. The Markov chain analysis revealed behavioral changes in the presence of boats, according to the type of group composition. Groups composed of adults presented decreased resting and increased in traveling during the presence of boats. Groups of adults and juveniles showed a massive reduction of socialization, while the behavior transition probability traveling-traveling was higher in groups of adults and calves. In the presence of the boats, stability of resting was reduced by one third of its original duration and traveling more than doubled. The behavioral patterns analyzed are discussed in light of socio-ecological models concerning costs and benefits of proximity between individuals and behavioral optimization. Furthermore, significant changes in behavioral patterns indicate that Guiana dolphins, at Pipa Beach, have suffered the effects of tourism as a result of violation of rules of conduct established for the study area

Uso de habitat e comportamento do boto-cinza sotalia guianensis em uma zona estuarina do estado de Sergipe, nordeste do Brasil a biotecnologia vegetal como alternativa para a cotonicu

To clarify the functional mechanisms of habitat use is necessary to analyze it in conjunction with the conduct performed by animals. The occurrence, distribution and use of space are characteristic of a species resulting from habitat selection that is in search of conditions favorable to its survival. One can relate the physical and biological factors of the environment with the ecological characteristics of the species, since these factors act by regulating the ecological success of organisms, and from there you can get important information about the habitat use and behavior of individuals. This study aimed to characterize the use of habitat and diurnal activity expressed by the Guiana dolphin, Sotalia guianensis in an estuarine area of Sergipe state, Brazil, analyzing the influence of tide and time days on the occurrence of animals and behavior s state, and group s size and composition in this cetacean species. From March 2009 to February 2010, focal groups observations of dolphins were made from fixed - point and records snapshots of data taken every 5 min. in the interval from 6 a.m to 6 p.m, in alternating shifts. The results showed that the constant presence of animals in the area of the Sergipe River estuary indicates that this is an important area of occurrence of S. guianensis, which use the region mainly in the morning, at low tide and as a feeding. As in other regions of northeastern Brazil, small groups formed 2-12 individuals were most common, with adults and immatures. The high frequency of immature animals may indicate that this area of the estuary is used as brood area and parental care of pups and young animals, since the immature animals were very associated with adults and monitoring the activities of foraging / feeding may be related to a form of learning or training of such behavior