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Amyotrophic lateral sclerosis is a rapidly progressing neurodegenerative disease associated with protein misfolding and aggregation. Most cases are characterized by TDP-43 positive inclusions, while a minority of familial ALS cases are instead FUS and SOD1 positive respectively. Cells can generate inclusions of variable type including previously characterized aggresomes, IPOD or JUNQ structures depending on the misfolded protein. SOD1 invariably forms JUNQ inclusions but it remains unclear whether other ALS protein aggregates arise as one of these previously described inclusion types or form unique structures. Here we show that FUS variably partitioned to IPOD, JUNQ or alternate structures, contain a mobile fraction, were not microtubule dependent and initially did not contain ubiquitin. TDP-43 inclusions formed in a microtubule independent manner, did not contain a mobile fraction but variably colocalized to JUNQ inclusions and another alternate structure. We conclude that the RNA binding proteins TDP-43 and FUS do not consistently fit the currently characterised inclusion models suggesting that cells have a larger repertoire for generating inclusions than currently thought and imply that toxicity in ALS does not stem from a particular aggregation process or aggregate structure.

Abstract During this one year study, blood and fecal samples of doves (Zenaida asiatica), ducks (Anas platyrhynchos), pigeons (Columba livia), partridges (Alectoris chukar), turkeys (Meleagris gallopavo) and goose (Chen caerulescens) were collected to assess the parasitic prevalence in these birds. The birds were kept at Avian Conservation and Research Center, Department of Wildlife and Ecology, University of Veterinary and Animal Sciences, Lahore. All these avian species were kept in separate cages and their entire body was inspected on regularly basis to record external parasites. For internal parasites, 100 blood and 100 fecal samples for each species were analyzed. During present study, two species of ectoparasites i.e. fowl ticks (Args persicus) and mite (Dermanyssus gallinae) while 17 species of endoparasites; three from blood and 14 from fecal samples were identified. Prevalence of blood parasites was Plasmodium juxtanucleare 29.3%, Aegyptinella pullorum 15% and Leucoctoyzoon simond 13%. Parasitic species recorded from fecal samples included 6 species of nematodes viz. Syngamus trachea with parasitic prevalence of 50%, Capillaria anatis 40%, Capillaria annulata 37.5%, Heterakis gallinarum 28.3%, Ascardia galli 24% and Allodpa suctoria 2%. Similarly, two species of trematodes viz. Prosthogonimus ovatus having parasitic prevalence of 12.1% and Prosthogonimus macrorchis 9.1% were also recorded from fecal samples of the birds. Single cestode species Raillietina echinobothrida having parasitic prevalence of 27% and 3 protozoan species i.e. Eimeria maxima having prevalence 20.1%, Histomonas meleagridis 8% and Giardia lamblia 5.3% were recorded. In our recommendation, proper medication and sanitation of the bird’s houses and cages is recommended to avoid parasites. Resumo Durante este estudo de um ano, amostras de sangue e fezes de pombos (Zenaida asiatica), patos (Anas platyrhynchos), pombos (Columba livia), perdizes (Alectoris chukar), perus (Meleagris gallopavo) e ganso (Chen caerulescens) foram coletados para avaliar a prevalência de parasitas nessas aves. As aves foram mantidas no Centro de Conservação e Pesquisa de Aves, Departamento de Vida Selvagem e Ecologia, Universidade de Veterinária e Ciências Animais, Lahore. Todas essas espécies de aves foram mantidas em gaiolas separadas e todo o seu corpo foi inspecionado regularmente para registrar parasitas externos. Para parasitas internos, foram analisadas 100 amostras de sangue e 100 amostras fecais de cada espécie. Durante o presente estudo, duas espécies de ectoparasitas, ou seja, carrapatos de aves (Args persicus) e ácaros (Dermanyssus gallinae), enquanto 17 espécies de endoparasitas, três de sangue e 14 de amostras fecais, foram identificadas. Os parasitas sanguíneos prevalentes foram Plasmodium juxtanucleare, 29,3%, Aegyptinella pullorum, 15%, e Leucoctoyzoon simond, 13%. As espécies parasitas registradas em amostras fecais incluíram 6 espécies de nematoides viz. Syngamus traqueia com prevalência parasitária de 50%, Capillaria anatis, 40%, Capillaria annulata, 37,5%, Heterakis gallinarum, 28,3%, Ascardia galli, 24% e Allodpa suctoria, 2%. Da mesma forma, duas espécies de trematódeos viz. Prosthogonimus ovatus com prevalência parasitária de 12,1% e Prosthogonimus macrorchis, 9,1%, também foram registrados nas amostras fecais das aves. Espécies de cestoide único Raillietina echinobothrida com prevalência parasitária de 27% e 3 espécies de protozoários, ou seja, Eimeria maxima tendo prevalência de 20,1%, Histomonas meleagridis, 8%, e Giardia lamblia, 5,3%, foram registradas. Em nossa recomendação, são indicados medicação adequada e saneamento das casas e gaiolas dos pássaros para evitar parasitas.

Newer-generation CT scanners with ultrawide detectors or dual sources offer millisecond image acquisition times and significantly decreased radiation doses compared to historical cardiac CT and CT angiography. This technology is capable of nearly freezing cardiac and respiratory motion. As a result, CT is increasingly used for diagnosing and monitoring cardiac and vascular abnormalities in the pediatric population. CT is particularly useful in the setting of pulmonary vein evaluation because it offers evaluation of the entire pulmonary venous system and lung parenchyma. In this article we review a spectrum of congenital and acquired pulmonary venous abnormalities, including potential etiologies, CT imaging findings and important factors of preoperative planning. In addition, we discuss optimization of CT techniques for evaluating the pulmonary veins.

ObjectiveFUS gene mutations were recently identified in familial amyotrophic lateral sclerosis (ALS). The present studies sought to define the clinical, post-mortem and neurophysiological phenotypes in ALS families with FUS mutations and to determine the frequency of FUS mutations in familial and sporadic ALS.MethodsFUS was screened for mutations in familial and sporadic ALS cases. Clinical, post-mortem and neurophysiological features of large families with FUS mutations are described.Results and conclusionsFUS mutations were evident in 3.2% (4/124) of familial ALS, representing the second most common gene abnormality to be described in familial ALS after SOD1. No mutations were present in 247 sporadic ALS cases. The clinical presentation in 49 affected patients was consistent with a predominantly lower motor neuron disorder, supported by post-mortem findings. Upper motor neuron involvement varied, with Wallerian degeneration of corticospinal tracts present in one post-mortem case but absent in a second case from the same family. Features of cortical hyperexcitability demonstrated upper motor neuron involvement consistent with other forms of familial and sporadic ALS. One case presented with frontotemporal dementia (FTD) indicating that this may be a rare presenting feature in families with FUS mutation. Ubiquitin-positive cytoplasmic skein-like inclusions were present in lower motor neurons, but in contrast to sporadic ALS, no TDP-43 pathology was evident. Mutation-specific clinical features were identified. Patients with a R521C mutation were significantly more likely to develop disease at a younger age, and dropped-head syndrome was a frequent feature. Reduced disease penetrance was evident among most affected families.

Abstract Present study was planned to determine variations in external and internal quality egg parameters of different avian species including ostrich Struthio camelus, ducks Anas platyrhynchos, chicken Gallus gallus, turkeys Meleagris gallopavo and grey francolin Francolinus pondicerinus. All the birds were kept under similar rearing conditions. A total of 150 eggs were collected for each species to record external features of these eggs. Statistically significant (p<0.05) variations were recorded in egg weight, egg length and egg width between ostrich, ducks, chicken, turkey and quail eggs. Significantly (p<0.05) higher egg weight, egg length and egg width was observed for ostrich eggs while the same was lowest for grey francolin eggs. Similarly, significantly (p<0.05) greater shape index and egg volume values were observed for ostrich eggs while lowest shape index values were recorded for turkey eggs and egg volume was lowest for grey francolin. Significantly, higher (p<0.05) values of egg density were noted for eggs of the quail and the same were lowest for ostrich eggs. Non-significant variations in egg density values were observed between eggs of the ducks, chicken, turkey and grey francolin. It has been concluded that the positive correlations between the internal and external egg quality traits indicated that the traits can be improved through selection. Resumo O presente estudo foi planejado para determinar variações nos parâmetros externos e internos de qualidade dos ovos de diferentes espécies de aves, incluindo avestruz Struthio camelus, patos Anas platyrhynchos, frango Gallus gallus, perus Meleagris gallopavo e francolin cinza Francolinus pondicerinus. Todas as aves foram mantidas em condições de criação semelhantes. Um total de 150 ovos foi coletado para cada espécie para registrar as características externas desses ovos. Variações estatisticamente significativas (p < 0,05) foram registradas no peso do ovo, comprimento do ovo e largura do ovo entre os ovos de avestruz, patos, galinha, peru e codorna. Significativamente (p < 0,05) maior peso do ovo, comprimento e largura do ovo foram observados para ovos de avestruz, enquanto o mesmo foi menor para ovos de francolina cinza. Da mesma forma, significativamente (p < 0,05) maiores valores de índice de forma e volume de ovo foram observados para ovos de avestruz, enquanto os menores valores de índice de forma foram registrados para ovos de peru e o volume de ovo foi menor para francolina cinza. Significativamente, maiores (p < 0,05) valores de densidade de ovos foram observados para ovos de codorna e os mesmos foram menores para ovos de avestruz. Variações não significativas nos valores de densidade de ovos foram observadas entre os ovos de pato, frango, peru e francolina cinza. Concluiu-se que as correlações positivas entre as características internas e externas de qualidade do ovo indicaram que as características podem ser melhoradas por meio da seleção.

During this one year study, blood and fecal samples of doves (Zenaida asiatica), ducks (Anas platyrhynchos), pigeons (Columba livia), partridges (Alectoris chukar), turkeys (Meleagris gallopavo) and goose (Chen caerulescens) were collected to assess the parasitic prevalence in these birds. The birds were kept at Avian Conservation and Research Center, Department of Wildlife and Ecology, University of Veterinary and Animal Sciences, Lahore. All these avian species were kept in separate cages and their entire body was inspected on regularly basis to record external parasites. For internal parasites, 100 blood and 100 fecal samples for each species were analyzed. During present study, two species of ectoparasites i.e. fowl ticks (Args persicus) and mite (Dermanyssus gallinae) while 17 species of endoparasites; three from blood and 14 from fecal samples were identified. Prevalence of blood parasites was Plasmodium juxtanucleare 29.3%, Aegyptinella pullorum 15% and Leucoctoyzoon simond 13%. Parasitic species recorded from fecal samples included 6 species of nematodes viz. Syngamus trachea with parasitic prevalence of 50%, Capillaria anatis 40%, Capillaria annulata 37.5%, Heterakis gallinarum 28.3%, Ascardia galli 24% and Allodpa suctoria 2%. Similarly, two species of trematodes viz. Prosthogonimus ovatus having parasitic prevalence of 12.1% and Prosthogonimus macrorchis 9.1% were also recorded from fecal samples of the birds. Single cestode species Raillietina echinobothrida having parasitic prevalence of 27% and 3 protozoan species i.e. Eimeria maxima having prevalence 20.1%, Histomonas meleagridis 8% and Giardia lamblia 5.3% were recorded. In our recommendation, proper medication and sanitation of the bird's houses and cages is recommended to avoid parasites.

Extracardiac and lateral tunnel Fontan palliations result in caval venous return to the branch pulmonary arteries without prior blood mixing in the right atrium. Single contrast injection techniques result in suboptimal pulmonary computed tomography angiography since blood streaming results in asymmetric pulmonary flow of injected contrast. We used a dual injection technique––dorsal foot vein and upper extremity vein to achieve optimal simultaneous opacification of the inferior and superior venae cavae and branch pulmonary arteries. The resulting dataset allowed for excellent 3D volume renderings and multiplanar reformat images useful for stenosis, aneurysm, and metal stent evaluation.

Our purpose was to identify E- and A-wave flow patterns across the tricuspid valve in tetralogy of Fallot (TOF) patients following repair using magnetic resonance imaging phase contrast velocity-encoded flow quantification and to correlate them with measurements of right ventricle enlargement. The study included 33 children following TOF repair who had MRI examinations that included cine imaging to quantify ventricle size and function and flow analysis across the atria-ventricular valves to evaluate ventricle in-flow patterns. The E:A ratio was calculated for each patient and the population separated into α (E:A ratio ≥ 1.4) and β (E:A ratio < 1.4) groups. Significant association was present between the β group and right ventricle end diastolic volume index ≥140 ml/m 2 ( P  = 0.046), right ventricle end systolic volume index ≥70 ml/m 2 ( P  = 0.02), and end diastolic volume right ventricle to left ventricle ≥ 2.0 ( P  = 0.003). A reduction in the E:A wave ratio across the tricuspid valve is associated with right ventricle diastolic dysfunction and correlated well in our study with right ventricle enlargement. This may be a useful criterion for determining the timing of valved pulmonary conduit surgery in children following TOF repair.

Background Injuries related to all-terrain vehicle (ATV) use by children have increased in recent years, and the pattern of these injuries is not well known among radiologists. Objective Our purpose was to identify different radiologically diagnosed injuries in children suffering ATV-related trauma and determine associations among various injuries as well as between injuries and outcome. Materials and methods The study included 512 consecutive children suffering from ATV injuries treated at a tertiary care pediatric hospital. All imaging studies were reviewed and correlated with injury frequency and outcome using multivariate analysis. Results Head injuries occurred in 244 children (48%) and in five of six deaths. Calvarial skull fractures occurred in 104 children and were associated with brain, subdural and epidural injuries. Brain and orbit injuries were associated with long-term disability. A total of 227 extremity fractures were present in 172 children (34%). The femur was the most commonly fractured bone. Nine children had partial foot amputations. Multiorgan injuries occurred in nearly half of the 97 children with torso injuries. Determinants for long-term disability or death were head injuries (odds ratio 3.4) and extremity fractures (odds ratio 3.3). Conclusion Head and extremity injuries are the two most common injuries in children suffering ATV injuries and are associated with long-term disability. ATV use by children is dangerous and is a significant threat to child safety.

All-terrain vehicle (ATV) accidents are common in children. Our purpose was to identify imaging patterns associated with ATV injuries in children. The study group comprised 141 consecutive children admitted to a tertiary pediatric hospital following ATV accidents. Medical records were evaluated for demographics and patient outcome. All imaging studies were reviewed and abnormalities cataloged. Extremity fractures were the most common injuries in our study, occurring in 38% of patients. Lower extremity fractures were more common than upper extremity fractures. Partial foot amputation, an unusual injury, was present in three children. Torso injuries were present in 22% of the children. Pulmonary contusions, but not abdominal injuries, were significantly associated with long-term disability or death (p=0.01). Brain injuries occurred in 19% of the children and were significantly associated with death or long-term disability (p=<0.001). No association of brain injury and skull fracture was present. A wide variety of injuries were identified in children with ATV accidents. Partial foot amputation, an unusual injury, was identified in three children. Brain injuries and lung contusions detected by computed tomography were associated with long-term disability and death. Radiologists need to be aware of injuries associated with ATV accidents.