Profiles of functioning of a group of children with cerebral palsy in Argentina: Preliminary data from the first national study. / Perfiles de funcionamiento en un grupo de niños con parálisis cerebral en Argentina: datos preliminares del primer estudio nacional.

Cerebral palsy (CP) affects body posture and movement coordination and is the most common cause of severe disability in the pediatric population. The diagnosis of CP is not a description of a person's functioning or interaction with their environment. Therefore, the diagnosis should be complemented with a description of functioning, using tools based on the biopsychosocial model proposed by the World Health Organization's International Classification of Functioning, Disability and Health (ICF). This report describes the preliminary data from a multicenter study conducted in Argentina with the aim of standardizing the description of the profiles of functioning of children and adolescents with CP. These data showed that the participants had some skills in sleep functions, mental functions of language, seeing functions, and in some environmental factors. They showed significant difficulties in categories such as maintaining body position, walking, and toileting.

La parálisis cerebral (PC) afecta la postura y la coordinación del movimiento, y es la causa más común de discapacidad grave en la población pediátrica. El diagnóstico de PC no describe el funcionamiento ni la interacción de la persona con el contexto en el cual se desarrolla. Por lo tanto, el diagnóstico se debe complementar con una descripción del funcionamiento, utilizando herramientas basadas en el modelo biopsicosocial de la Clasificación del Funcionamiento (CIF) de la Organización Mundial de la Salud (OMS). En esta comunicación, describimos los datos preliminares de un estudio multicéntrico en Argentina destinado a estandarizar la descripción del perfil de funcionamiento en niños y adolescentes con PC. Estos datos mostraron que los participantes tienen algunas habilidades en las funciones del sueño, las funciones mentales del lenguaje y las visuales, así como en algunas relacionadas con el contexto. Presentan dificultades importantes en categorías tales como mantener la posición del cuerpo, el andar y la higiene.

Cytokines and growth factors in a biologic product obtained from patients' urine as immune-modulators to treat autoimmune and allergic diseases.

At "Instituto de Alergias y Autoinmunidad Dr. Maximiliano Ruiz Castañeda, A.C." in Mexico City, a non-traditional health care center focused on the treatment of autoimmune and allergic diseases using personalized medicine, an alternative treatment referred to as an "immune-modulator" has been developed. In this study, we will refer to this treatment substance as the "immune-modulator." In brief, a urine sample is collected from the patient and processed to obtain the peptide fraction, which is conditioned and then administered sublingually to the patient. Sample processing involves multiple steps aimed at the removal of toxic compounds and enrichment for cytokines, growth factors, and other immune peptides that may contribute to the function of the immune-modulator. This treatment has been administered for many years, and patients testify that it is useful and reliable. Despite the benefits of this treatment, the molecular mechanisms underlying its effects have not been thoroughly investigated. Therefore, this study aims to identify immunoregulatory peptides, such as cytokines and growth factors, in the immune-modulator. Urine and immune-modulator concentrations of cytokines and growth factors were assessed using a Luminex assay. Twenty-one cytokines and growth factors were identified in immune-modulator samples. MCP-1 was identified in 100% of the samples; MIP-1ß, IL-8, RANTES, INF-γ, and IP-10 were identified in approximately 65-70% of samples; IL5, IL-1B, and IL-17 in 50-60%; eotaxin, VEGF, IL-6, and FGF in about 40%; MIP-1α, IL-9, GM-CSF, G-CSF, IL-12, and IL-15 in about 20-30%; and IL-13 and PDGF-bb were identified in <6% of samples. Additionally, patients exhibited significant changes in IL-1ß, IFN-γ, and MCP-1 concentrations after treatment with the immune-modulator, whereas healthy individuals showed no significant change in response to the treatment. The immune-modulator is an alternative treatment based on the administration of cytokines and growth factors obtained from the urine of patients. In this study, its composition was characterized. The isolated products could be responsible for the effects of the immune-modulator. Further trials are required to evaluate the effective delivery of these molecules by the administration route described.

Evidence-based clinical practice guidelines for the management of sedoanalgesia and delirium in critically ill adult patients. / Guías de práctica clínica basadas en la evidencia para el manejo de la sedoanalgesia y delirium en el paciente adulto críticamente enfermo.

Given the importance of the management of sedation, analgesia and delirium in Intensive Care Units, and in order to update the previously published guidelines, a new clinical practice guide is presented, addressing the most relevant management and intervention aspects based on the recent literature. A group of 24 intensivists from 9 countries of the Pan-American and Iberian Federation of Societies of Critical Medicine and Intensive Therapy met to develop the guidelines. Assessment of evidence quality and recommendations was made according to the Grading of Recommendations Assessment, Development and Evaluation Working Group. A systematic search of the literature was carried out using MEDLINE, Cochrane Library databases such as the Cochrane Database of Systematic Reviews and the Cochrane Central Register of Controlled Trials (CENTRAL), the Database of Abstracts of Reviews of Effects, the National Health Service Economic Evaluation Database and the database of Latin American and Caribbean Literature in Health Sciences (LILACS). A total of 438 references were selected. After consensus, 47 strong recommendations with high and moderate quality evidence, 14 conditional recommendations with moderate quality evidence, and 65 conditional recommendations with low quality evidence were established. Finally, the importance of initial and multimodal pain management was underscored. Emphasis was placed on decreasing sedation levels and the use of deep sedation only in specific cases. The evidence and recommendations for the use of drugs such as dexmedetomidine, remifentanil, ketamine and others were incremented.

A new species of Piaractus (Characiformes: Serrasalmidae) from the Orinoco Basin with a redescription of Piaractus brachypomus.

Piaractus orinoquensis, a new species of serrasalmid fish, is described from the Orinoco River basin. The new species differs from congeners by having a slenderer body, relatively smaller head and snout, more compressed mid-body, fewer scales above and below the lateral line and diagnostic molecular characters in the coI mitochondrial gene region. We also provide a re-description of Piaractus brachypomus, restricting its geographic distribution to the Amazon River basin. Both species are economically important in their respective basins and need to be independently managed as distinct species.

Conformational selectivity and high-affinity binding in the complexation of N-phenyl amides in water by a phenyl extended calix[4]pyrrole.

We describe the synthesis of a tetrapyridinium phenyl extended calix[4]pyrrole that is soluble in neutral water solution at mM concentrations. We show that, in pure water, the synthesized calix[4]pyrrole receptor selectively binds the cis-(E) conformers of secondary N-phenyl-amides and tertiary N-methyl-N-phenyl-formamide with binding affinities larger than 103 M-1. The conformational selectivity is remarkable owing to the energetic preference of amides to adopt the trans-(Z) conformation in solution. In this respect, we used two binding models for the mathematical analyses of the titration data and calculated apparent and intrinsic binding constants. The combined action of hydrogen bonding and the hydrophobic effect that operates in the binding of the amides in water is responsible for the large affinities displayed by the receptor.

Locus coeruleus at asymptomatic early and middle Braak stages of neurofibrillary tangle pathology.

AIMS: The present study analyses molecular characteristics of the locus coeruleus (LC) and projections to the amygdala and hippocampus at asymptomatic early and middle Braak stages of neurofibrillary tangle (NFT) pathology. METHODS: Immunohistochemistry, whole-transcriptome arrays and RT-qPCR in LC and western blotting in hippocampus and amygdala in a cohort of asymptomatic individuals at stages I-IV of NFT pathology were used. RESULTS: NFTs in the LC increased in parallel with colocalized expression of tau kinases, increased neuroketal adducts and decreased superoxide dismutase 1 in neurons with hyperphosphorylated tau and decreased voltage-dependent anion channel in neurons containing truncated tau were found. These were accompanied by increased microglia and AIF1, CD68, PTGS2, IL1ß, IL6 and TNF-α gene expression. Whole-transcriptome arrays revealed upregulation of genes coding for proteins associated with heat shock protein binding and genes associated with ATP metabolism and downregulation of genes coding for DNA-binding proteins and members of the small nucleolar RNAs family, at stage IV when compared with stage I. Tyrosine hydroxylase (TH) immunoreactivity was preserved in neurons of the LC, but decreased TH and increased α2A adrenergic receptor protein levels were found in the hippocampus and the amygdala. CONCLUSIONS: Complex alteration of several metabolic pathways occurs in the LC accompanying NFT formation at early and middle asymptomatic stages of NFT pathology. Dopaminergic/noradrenergic denervation and increased expression of α2A adrenergic receptor in the hippocampus and amygdala occur at first stage of NFT pathology, suggesting compensatory activation in the face of decreased adrenergic input occurring before clinical evidence of cognitive impairment and depression.

Potential distribution of the viral haemorrhagic septicaemia virus in the Great Lakes region.

Viral haemorrhagic septicaemia virus (VHSV) genotype IVb has been responsible for large-scale fish mortality events in the Great Lakes of North America. Anticipating the areas of potential VHSV occurrence is key to designing epidemiological surveillance and disease prevention strategies in the Great Lakes basin. We explored the environmental features that could shape the distribution of VHSV, based on remote sensing and climate data via ecological niche modelling. Variables included temperature measured during the day and night, precipitation, vegetation, bathymetry, solar radiation and topographic wetness. VHSV occurrences were obtained from available reports of virus confirmation in laboratory facilities. We fit a Maxent model using VHSV-IVb reports and environmental variables under different parameterizations to identify the best model to determine potential VHSV occurrence based on environmental suitability. VHSV reports were generated from both passive and active surveillance. VHSV occurrences were most abundant near shore sites. We were, however, able to capture the environmental signature of VHSV based on the environmental variables employed in our model, allowing us to identify patterns of VHSV potential occurrence. Our findings suggest that VHSV is not at an ecological equilibrium and more areas could be affected, including areas not in close geographic proximity to past VHSV reports.

Endometrioma complicado por absceso ovárico espontáneo / Endometrioma complicated by spontaneous ovarian abscess

RESUMEN OBJETIVO: reportar un caso de endometrioma complicado con absceso ovárico, exponer y discutir los factores de riesgo asociados, con la finalidad de difundir esta rara alteración y establecer el diagnóstico oportuno. CASO CLÍNICO: paciente de 34 años de edad, que acudió a consulta al Hospital Santa Rosa de Lima (Ensenada, Baja California Norte), con antecedente de dolor pélvico crónico de 5 meses de evolución y fiebre intermitente. El tratamiento con antibiótico de amplio espectro no mostró reacción satisfactoria. El ultrasonido transvaginal del ovario derecho mostró una imagen compatible con endometrioma. Mediante laparotomía se disecó la cápsula y se drenaron múltiples adherencias tubo-ováricas. El informe histopatológico confirmó el diagnóstico de endometrioma complicado por absceso. CONCLUSIÓN: es importante conocer los factores de riesgo para definir la causa de un absceso en un endometrioma, a través de la historia clínica detallada. La sospecha y diagnóstico oportuno es trascendental para reducir la morbilidad y mortalidad de esta complicación. Cualquier mujer con endometriosis en etapa avanzada es susceptible de complicaciones, como la formación de un absceso ovárico espontáneo.

ABSTRACT OBJETIVE: To report a case of Endometrioma complicated with ovarian abscess, expose and discuss the factors of risk associated with the purpose of disseminating this rare alteration and establishing the appropriate diagnosis. CASE REPORT: A 34 year old, seen at the Santa Rosa de Lima Hospital in Ensenada, Baja California, for a history of 5 months long chronic pelvic pain with intermittent fever, under treatment with broad spectrum antibiotic with no improvement. Transvaginal ultrasound revealed a compatible image with an endometrioma on the right ovary, followed by a laparotomy, capsule dissection and release of multiple tube-ovarian adhesions. The histo-pathological report confirmed that it was an ovarian endometrioma complicated by abscess. CONCLUSION: We emphasized in the study the risk factors and define a cause of an endometrioma and the clinical history details. Prevention and a good diagnoses as a priority to reduce morbi-mortality. It is very important to know that any woman with advanced stage history of endometriosis is susceptible to complications.

ASPP2 deficiency causes features of 1q41q42 microdeletion syndrome.

Chromosomal abnormalities are implicated in a substantial number of human developmental syndromes, but for many such disorders little is known about the causative genes. The recently described 1q41q42 microdeletion syndrome is characterized by characteristic dysmorphic features, intellectual disability and brain morphological abnormalities, but the precise genetic basis for these abnormalities remains unknown. Here, our detailed analysis of the genetic abnormalities of 1q41q42 microdeletion cases identified TP53BP2, which encodes apoptosis-stimulating protein of p53 2 (ASPP2), as a candidate gene for brain abnormalities. Consistent with this, Trp53bp2-deficient mice show dilation of lateral ventricles resembling the phenotype of 1q41q42 microdeletion patients. Trp53bp2 deficiency causes 100% neonatal lethality in the C57BL/6 background associated with a high incidence of neural tube defects and a range of developmental abnormalities such as congenital heart defects, coloboma, microphthalmia, urogenital and craniofacial abnormalities. Interestingly, abnormalities show a high degree of overlap with 1q41q42 microdeletion-associated abnormalities. These findings identify TP53BP2 as a strong candidate causative gene for central nervous system (CNS) defects in 1q41q42 microdeletion syndrome, and open new avenues for investigation of the mechanisms underlying CNS abnormalities.

Coexistencia de enfermedad de Crohn y trombocitopenia inmune primaria y sus implicaciones en el tratamiento / Co-existence of Crohn's disease and primary immune thrombocytopenia and its implications in treatment

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First isolation, identification, phenotypic and genotypic characterization of Brucella abortus biovar 3 from dairy cattle in Tanzania.

BACKGROUND: Brucellosis is a disease of worldwide public health and economic importance. Successful control is based on knowledge of epidemiology and strains present in an area. In developing countries, most investigations are based on serological assays. This study aimed at investigating a dairy herd experiencing abortions in order to establish within-herd seroprevalence to Brucella spp., identify, characterize Brucella strains by Multiple Loci Variable Number of Tandem Repeats Analysis (MLVA-VNTR) and investigate possible spillover to other species. RESULTS: The within-herd seroprevalence in cattle (n = 200) was 48 % (95 % CI 41-55), using an indirect ELISA, while the Rose Bengal Test (RBT) yielded lower prevalence (21.5 %; 95 % CI 16-27). Two sheep (n = 35) and one goat (n = 50) were seropositive using ELISA while none of the dogs (n = 6) was positive with the RBT. Three Brucella were isolated from an aborted fetus and associated membranes. Real time PCR (IS711), Bruce-ladder and classical biotyping classified the isolates as B. abortus biovar 3. MLVA-VNTR revealed two different but closely related genotypes. The isolates showed unique profiles, providing the first genotypic data from Tanzania. These genotypes were not related to B. abortus biovar 3 reference strain Tulya originally isolated from a human patient in Uganda in 1958, unlike the genotypes isolated and characterized recently in Kenya. High within-herd prevalence, isolation of the pathogen and abortion confirm that B. abortus is circulating in this herd with cattle as reservoir hosts. A low seroprevalence in sheep and goats suggests a spillover of B. abortus from cattle to small ruminants in the herd. CONCLUSIONS: This is the first isolation and characterization of B. abortus biovar 3 from a dairy cow with abortion in Tanzania. The origin of the Tanzanian genotypes remain elusive, although they seem to be related to genotypes found in Europe, Turkey and China but not related to B. abortus biovar 3 reference strain or genotypes from Kenya. Importantly, replacement heifers are commonly sourced from large farms like this to smallholder farmers, which poses risk of spread of bacteria to other herds. B. abortus is a significant zoonotic risk and animal health problem in this production system, therefore further studies on humans is recommended.

Diagnostic utility of microarray testing in pregnancy loss.

OBJECTIVES: To determine the frequency of clinically significant chromosomal abnormalities identified by chromosomal microarray in pregnancy losses at any gestational age and to compare microarray performance with that of traditional cytogenetic analysis when testing pregnancy losses. METHODS: Among 535 fetal demise specimens of any gestational age, clinical microarray-based comparative genomic hybridization (aCGH) was performed successfully on 515, and a subset of 107 specimens underwent additional single nucleotide polymorphism (SNP) analysis. RESULTS: Overall, clinically significant abnormalities were identified in 12.8% (64/499) of specimens referred with normal or unknown karyotypes. Detection rates were significantly higher with earlier gestational age. In the subset with normal karyotype, clinically significant abnormalities were identified in 6.9% (20/288). This detection rate did not vary significantly with gestational age, suggesting that, unlike aneuploidy, the contribution of submicroscopic chromosomal abnormalities to fetal demise does not vary with gestational age. In the 107 specimens that underwent aCGH and SNP analysis, seven cases (6.5%) had abnormalities of potential clinical significance detected by the SNP component, including female triploidy. aCGH failed to yield fetal results in 8.3%, which is an improvement over traditional cytogenetic analysis of fetal demise specimens. CONCLUSIONS: Both the provision of results in cases in which karyotype fails and the detection of abnormalities in the presence of a normal karyotype demonstrate the increased diagnostic utility of microarray in pregnancy loss. Thus, chromosomal microarray testing is a preferable, robust method of analyzing cases of pregnancy loss to better delineate possible genetic etiologies, regardless of gestational age.

Diagnóstico de sospecha de cáncer en los servicios de urgencias / Diagnosis of cancer in hospital emergency services

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Dog ownership, abundance and potential for bat-borne rabies spillover in Chile.

Rabies is a viral infectious disease that affects all mammals, including humans. Factors associated with the incidence of rabies include the presence and density of susceptible hosts and potential reservoirs. Currently, Chile is declared free of canine-related rabies, but there is an overpopulation of dogs within the country and an emergence of rabies in bats. Our objectives are to determine potential areas for bat-borne rabies spillover into dog populations expressed as a risk map, and to explore some key features of dog ownership, abundance, and management in Chile. For the risk map, our variables included a dog density surface (dog/km(2)) and a distribution model of bat-borne rabies presence. From literature review, we obtained dog data from 112 municipalities, which represent 33% of the total municipalities (339). At country level, based on previous studies the median human per dog ratio was 4.8, with 64% of houses containing at least one dog, and a median of 0.9 dog per house. We estimate a national median of 5.3 dog/km(2), and a median of 3680 dogs by municipality, from which we estimate a total population of 3.5×10(6) owned dogs. The antirabies vaccination presented a median of 21% of dogs by municipality, and 29% are unrestricted to some degree. Human per dog ratio have a significant (but weak) negative association with human density. Unrestricted dogs have a negative association with human density and income, and a positive association with the number of dogs per house. Considering dog density by municipality, and areas of potential bat-borne rabies occurrence, we found that 163 (∼48%) of Chilean municipalities are at risk of rabies spillover from bats to dogs. Risk areas are concentrated in urban settlements, including Santiago, Chile's capital. To validate the risk map, we included cases of rabies in dogs from the last 27 years; all fell within high-risk areas of our map, confirming the assertive risk prediction. Our results suggest that the use of dog population parameters may be informative to determine risk areas for bat-rabies spillover events. In addition, we confirm that dog abundance is a neglected and emerging public health concern in Chile, particularly within urban areas, which deserves prompt intervention.

Spatial and temporal trends of bat-borne rabies in Chile.

In Chile, while dog rabies has decreased markedly over the last 30 years, bat rabies is still reported frequently. In order to shed new light on the spatiotemporal trends of these reports, we analysed active and passive data from years 1985 and 2012, which included 61 076 samples from 289 counties of Chile. We found that from 1994 to 2012, more than 15 000 bat samples were submitted for diagnostics through passive surveillance, 9·5% of which tested positive for rabies. By contrast, the prevalence of infection was only ~0·4% among the nearly 12 000 bat samples submitted through active surveillance. We found that the prevalence of dog rabies dropped steadily over the same period, with just a single confirmed case since 1998. None of the 928 samples from wild animals, other than bats, were positive for rabies. Although there has been only one confirmed case of human rabies in Chile since 1985, and a single confirmed case in a dog since 1998, bats remain a reservoir for rabies viruses. While active surveillance indicates that rabies prevalence is low in bat colonies, the high proportion of positive bats submitted through passive surveillance is a concern. To prevent human rabies, local public health agencies should increase research on the basic ecology of bats and the role of stray dogs and cats as potential rabies amplifiers.

[Ulcerative colitis associated with autoimmune hepatitis: a differential form of inflammatory bowel disease?]. / Colitis ulcerosa asociada a hepatitis autoinmune: ¿una forma diferencial de enfermedad inflamatoria intestinal?

Inflammatory bowel Disease (IBD) is a group of chronic inflammatory diseases that can be associated with different autoimmune diseases, including autoimmune hepatitis (AIH). Some specific and differential characteristics in children with IBD associated to AIH have been described. Our aim is to describe the clinical pattern of this association observed in our patients, confirming its differential characteristics as compared to classical IBD in children.

Strategies for improving the welfare of working equids in the Americas: a Chilean example.

In the past, the use of animals for transport and traction has been of economic importance all over the world, and this is still the case in certain areas of the world today, especially in rural and peri-urban areas of developing and transition countries. In Chile, for example, thousands of families rely on draught animal power as a fundamental source of income. This provides an opportunity to generate scientific information to increase understanding of the relationship between human well-being and that of domestic animals. Minimising the risks associated with poor draught animal welfare should be beneficial for this relationship. Given the important role of working equids as a source of wealth, in Chile and similar countries, different strategies have been implemented to address the health and welfare of these animals. For these strategies to be successful it is essential to establish appropriate government policies as well as to consider the cultural, political and geographical situation of the regions in which these equids work.