Broodstock nutritional programming differentially affects the hepatic transcriptome and genome-wide DNA methylome of farmed gilthead sea bream (Sparus aurata) depending on genetic background.

BACKGROUND: Broodstock nutritional programming improves the offspring utilization of plant-based diets in gilthead sea bream through changes in hepatic metabolism. Attention was initially focused on fatty acid desaturases, but it can involve a wide range of processes that remain largely unexplored. How all this can be driven by a different genetic background is hardly underlined, and the present study aimed to assess how broodstock nutrition affects differentially the transcriptome and genome-wide DNA methylome of reference and genetically selected fish within the PROGENSA® selection program. RESULTS: After the stimulus phase with a low fish oil diet, two offspring subsets of each genetic background received a control or a FUTURE-based diet. This highlighted a different hepatic transcriptome (RNA-seq) and genome-wide DNA methylation (MBD-seq) pattern depending on the genetic background. The number of differentially expressed transcripts following the challenge phase varied from 323 in reference fish to 2,009 in genetically selected fish. The number of discriminant transcripts, and associated enriched functions, were also markedly higher in selected fish. Moreover, correlation analysis depicted a hyper-methylated and down-regulated gene expression state in selected fish with the FUTURE diet, whereas the opposite pattern appeared in reference fish. After filtering for highly represented functions in selected fish, 115 epigenetic markers were retrieved in this group. Among them, lipid metabolism genes (23) were the most reactive following ordering by fold-change in expression, rendering a final list of 10 top markers with a key role on hepatic lipogenesis and fatty acid metabolism (cd36, pitpna, cidea, fasn, g6pd, lipt1, scd1a, acsbg2, acsl14, acsbg2). CONCLUSIONS: Gene expression profiles and methylation signatures were dependent on genetic background in our experimental model. Such assumption affected the magnitude, but also the type and direction of change. Thus, the resulting epigenetic clock of reference fish might depict an older phenotype with a lower methylation for the epigenetically responsive genes with a negative methylation-expression pattern. Therefore, epigenetic markers will be specific of each genetic lineage, serving the broodstock programming in our selected fish to prevent and mitigate later in life the risk of hepatic steatosis through changes in hepatic lipogenesis and fatty acid metabolism.

Temperature variation in nests of Paleosuchus palpebrosus (Crocodylia: Alligatoridae) near the southern edge of the species´ range, Brazil.

We monitored the temperature of seven Paleosuchus palpebrosus nests found on the banks of streams surrounding the Brazilian Pantanal, near the southern limit of the species´ distribution, between 2008 and 2013. The mean temperature of the nests between 45 and 68 days incubation, the presumed period of sex determination, varied between 26.1 and 31.5o C. Nest temperatures were 2 to 5°C higher than air temperatures, presumably due to metabolic heat of decay of material within the nests, but air temperature explained 10-50% of the variance in egg-chamber temperatures. The estimated incubation periods for nests from which eggs hatched were 80, 84, 86, 90 and 104 days with a mean of 89 (SD =9.23) days, though these are probably slight overestimates because eggs may have hatched in the period between inspections. For these nests, there was no significant relationship between mean temperature and incubation period (r2 = 0.23, p = 0.411).

[Concomitant Ulcerative Colitis and Neurofibromatosis type 1: possible common origin?] / Colitis Ulcerosa y Neurofibromatosis tipo 1 concomitantes: ¿posible origen común?

In the last 15 years, 3 cases of concurrent Ulcerative Colitis with Neurofibromatosis Type 1 have been described in adults and adolescents, but not in children; although it may be a casual finding, a com mon pathogenic pathway between both diseases is postulated, based on mast cell dysregulation in the gastrointestinal tract. OBJECTIVE: To report the clinical case of a toddler with onset of concomitant Ulcerative Colitis with CMV infection, with history of Neurofibromatosis Type 1, and to discuss the common origin between both diseases. CLINICAL CASE: We describe the case of a 2-and-a-half-year-old toddler with history of Neurofibromatosis Type 1 who presented with bloody diarrhea. On endos copic examination, the mucosa from the anal margin to the cecum was erythematous, with loss of vascular transparency. Biopsies of colonic mucosa showed signs of chronic inflammation, consistent with the diagnosis of Ulcerative Colitis, and CMV infection was diagnosed by PCR. CONCLUSION: Previous studies have suggested that mast cells may have a pathogenic role in the development of UC, however, the clinical significance of these findings is unknown. Future research is needed to further investigate the role of mast cells in the development of UC and to confirm a genetic association bet ween the two diseases.

Impact of Covid-19 on farming systems in Europe through the lens of resilience thinking.

Context: Resilience is the ability to deal with shocks and stresses, including the unknown and previously unimaginable, such as the Covid-19 crisis. Objective: This paper assesses (i) how different farming systems were exposed to the crisis, (ii) which resilience capacities were revealed and (iii) how resilience was enabled or constrained by the farming systems' social and institutional environment. Methods: The 11 farming systems included have been analysed since 2017. This allows a comparison of pre-Covid-19 findings and the Covid-19 crisis. Pre-Covid findings are from the SURE-Farm systematic sustainability and resilience assessment. For Covid-19 a special data collection was carried out during the early stage of lockdowns. Results and conclusions: Our case studies found limited impact of Covid-19 on the production and delivery of food and other agricultural products. This was due to either little exposure or the agile activation of robustness capacities of the farming systems in combination with an enabling institutional environment. Revealed capacities were mainly based on already existing connectedness among farmers and more broadly in value chains. Across cases, the experience of the crisis triggered reflexivity about the operation of the farming systems. Recurring topics were the need for shorter chains, more fairness towards farmers, and less dependence on migrant workers. However, actors in the farming systems and the enabling environment generally focused on the immediate issues and gave little real consideration to long-term implications and challenges. Hence, adaptive or transformative capacities were much less on display than coping capacities. The comparison with pre-Covid findings mostly showed similarities. If challenges, such as shortage of labour, already loomed before, they persisted during the crisis. Furthermore, the eminent role of resilience attributes was confirmed. In cases with high connectedness and diversity we found that these system characteristics contributed significantly to dealing with the crisis. Also the focus on coping capacities was already visible before the crisis. We are not sure yet whether the focus on short-term robustness just reflects the higher visibility and urgency of shocks compared to slow processes that undermine or threaten important system functions, or whether they betray an imbalance in resilience capacities at the expense of adaptability and transformability. Significance: Our analysis indicates that if transformations are required, e.g. to respond to concerns about transnational value chains and future pandemics from zoonosis, the transformative capacity of many farming systems needs to be actively enhanced through an enabling environment.

Indicaciones inhabituales de trasplante hepático. resultados / Unusual indications for liver transplantation. results

Resumen Introducción: El trasplante hepático (TH), es una terapia establecida en el tratamiento de diversas enfermedades del hígado agudas y crónicas terminales y del carcinoma hepatocelular (CHC). Las principales indicaciones en nuestro medio son la cirrosis de diferentes etiologías, el CHC, la atresia de vías biliares en niños y la falla hepática fulminante (FHF). Menos del 10% corresponden a indicaciones inhabituales, que incluyen pacientes con una miscelánea de enfermedades entre las cuales están la enfermedad poliquística hepática (EPH), enfermedades metabólicas (Niemann-Pick, otras), el síndrome hepato/portopulmonar, metástasis de diferentes tumores, etc. Objetivo: Describir y evaluar los resultados obtenidos con el trasplante hepático en estas indicaciones. Materiales y Método: Estudio de cohorte no concurrente que incluyó los TH por indicaciones inhabituales realizados entre marzo de 1997 y diciembre de 2016. De 295 TH realizados, 34 (11,5%) fueron por estas indicaciones. Resultados: Las causas más frecuentes fueron el síndrome porto/hepatopulmonar en 11 (40,7%) pacientes y la EPH en 9 (26,5%). Las enfermedades metabólicas representaron la tercera indicación, con 5 (14,7%) casos. Siete (20,6%) pacientes eran menores de 18 años. Las complicaciones más frecuentes fueron biliares y la trombosis de arteria hepática en 6 (17,6%) y 4 (11,8%) casos respectivamente; estos últimos eran portadores de una EPH masiva. Cuatro (12,5%) pacientes requirieron retrasplante. La mortalidad a 90 días fue de 2 (5,9%) enfermos. Conclusión: El TH es una opción factible en este grupo de pacientes con resultados similares a los obtenidos en las indicaciones clásicas.

Introduction: Liver transplantation (LT) is an established therapy in the treatment of several acute and chronic end-stage liver diseases and hepatocellular carcinoma (HCC). The main indications worldwide are cirrhosis of different etiologies, HCC, biliary atresia in children, and fulminant hepatic failure (FHF). Less than 10% concerns unusual indications which include patients with miscellaneous diseases among which are hepatic polycystic disease (HPD), metabolic diseases (Niemann-Pick, others), portal/hepatopulmonary syndrome, metastasis of different tumors, among others. Aim: The objective of the study is to describe and asses the results obtained with liver transplantation in these indications. Materials and Method: We performed a non-concurrent cohort study that included all LT due to unusual indications between March 1997 and December 2016 in a university medical center. Of 295 TH performed, 34 (11.75%) were due to these indications. Results: The most frequent causes were the portal/hepatopulmonary syndrome in 11 (40.7%) patients and HPD in 9 (26.5%). Metabolic diseases accounted for the third indication in 5 (14.7%) cases. Seven (20.6%) patients were less than 18 years old. The most frequent complications were biliary and hepatic artery thrombosis (HAT) in 6 (17.6%) and 4 (11.8%) cases, respectively. Patients complicated by a HAT suffered a massive EPH. Four (12.5%), required retransplantation. Mortality at 90 days was 2 (5.9%). Conclusión: LT is a feasible option in this group of patients with results similar to those obtained in classic indications of LT.

Functional insights into the infective larval stage of Anisakis simplex s.s., Anisakis pegreffii and their hybrids based on gene expression patterns.

BACKGROUND: Anisakis simplex sensu stricto and Anisakis pegreffii are sibling species of nematodes parasitic on marine mammals. Zoonotic human infection with third stage infective larvae causes anisakiasis, a debilitating and potentially fatal disease. These 2 species show evidence of hybridisation in geographical areas where they are sympatric. How the species and their hybrids differ is still poorly understood. RESULTS: Third stage larvae of Anisakis simplex s.s., Anisakis pegreffii and hybrids were sampled from Merluccius merluccius (Teleosti) hosts captured in waters of the FAO 27 geographical area. Specimens of each species and hybrids were distinguished with a diagnostic genetic marker (ITS). RNA was extracted from pools of 10 individuals of each taxon. Transcriptomes were generated using Illumina RNA-Seq, and assembled de novo. A joint assembly (here called merged transcriptome) of all 3 samples was also generated. The inferred transcript sets were functionally annotated and compared globally and also on subsets of secreted proteins and putative allergen families. While intermediary metabolism appeared to be typical for nematodes in the 3 evaluated taxa, their transcriptomes present strong levels of differential expression and enrichment, mainly of transcripts related to metabolic pathways and gene ontologies associated to energy metabolism and other pathways, with significant presence of excreted/secreted proteins, most of them allergens. The allergome of the 2 species and their hybrids has also been thoroughly studied; at least 74 different allergen families were identified in the transcriptomes. CONCLUSIONS: A. simplex s.s., A. pegreffi and their hybrids differ in gene expression patterns in the L3 stage. Strong parent-of-origin effects were observed: A. pegreffi alleles dominate in the expression patterns of hybrids albeit the latter, and A. pegreffii also display significant differences indicating that hybrids are intermediate biological entities among their parental species, and thus of outstanding interest in the study of speciation in nematodes. Analyses of differential expression based on genes coding for secreted proteins suggests that co-infections presents different repertoires of released protein to the host environment. Both species and their hybrids, share more allergen genes than previously thought and are likely to induce overlapping disease responses.

Análisis de recursos asistenciales para el ictus en España en 2012: ¿beneficios de la Estrategia del Ictus del Sistema Nacional de Salud? / Analysis of stroke care resources in Spain in 2012: Have we benefitted from the Spanish Health System’s stroke care strategy?

Introducción: La Estrategia del Ictus del Sistema Nacional de Salud (EISNS) fue un documento de consenso entre las distintas administraciones y sociedades científicas que se desarrolló con el objetivo de mejorar la calidad del proceso asistencial y garantizar la equidad territorial. Nuestro objetivo fue analizar los recursos asistenciales existentes y si se había cumplido el objetivo de la EISNS. Material y métodos: La encuesta sobre los recursos disponibles se realizó por un comité de neurólogos de cada una de las comunidades autónomas (CC.AA), los cuales también realizaron la encuesta de 2008. Los ítems incluidos fueron el número de Unidades de Ictus (UI), su dotación (monitorización, neurólogo 24 h/7 días, ratio enfermería, protocolos), ratio cama UI/100.000 habitantes, recursos diagnósticos (ecografía cardíaca y arterial cerebral, neuroimagen avanzada), realización de trombolisis intravenosa, intervencionismo neurovascular (INV), cirugía del infarto maligno de la arteria cerebral media (ACM) y disponibilidad de la telemedicina. Resultados: Se incluyeron datos de 136 hospitales. Existen 45 UI distribuidas de un modo desigual. La relación cama de UI por habitantes y comunidad autónoma osciló entre 1/74.000 a 1/1.037.000 habitantes, cumpliendo el objetivo solo Cantabria y Navarra. Se realizaron por neurólogos 3.237 trombolisis intravenosas en 83 hospitales, con un porcentaje respecto del total de ictus isquémico entre el 0,3 y el 33,7%. Los hospitales sin UI tenían una disponibilidad variable de recursos. Se realiza INV en todas las CC.AA salvo La Rioja, la disponibilidad del INV 24 h/7 días solo existe en 17 ciudades. Hay 46 centros con cirugía del infarto maligno de la ACM y 5 con telemedicina. Conclusión: La asistencia al ictus ha mejorado en cuanto al incremento de hospitales participantes, la mayor aplicación de trombolisis intravenosa y procedimientos endovasculares, también en la cirugía del infarto maligno de la ACM, pero con insuficiente implantación de UI y de la telemedicina. La disponibilidad de recursos diagnósticos es buena en la mayoría de las UI, e irregular en el resto de hospitales. Las distintas CC.AA deben avanzar para garantizar el mejor tratamiento y equidad territorial, y así conseguir el objetivo de la EISNS

Introduction: The Spanish Health System’s stroke care strategy (EISNS) is a consensus statement that was drawn up by various government bodies and scientific societies with the aim of improving quality throughout the care process and ensuring equality among regions. Our objective is to analyse existing healthcare resources and establish whether they have met EISNS targets. Material and methods: The survey on available resources was conducted by a committee of neurologists representing each of Spain’s regions; the same committee also conducted the survey of 2008. The items included were the number of stroke units (SU), their resources (monitoring, neurologists on call 24 h/7d, nurse ratio, protocols), SU bed ratio/100 000 inhabitants, diagnostic resources (cardiac and cerebral arterial ultrasound, advanced neuroimaging), performing iv thrombolysis, neurovascular interventional radiology (neuro VIR), surgery for malignant middle cerebral artery (MCA) infarctions and telemedicine availability. Results: We included data from 136 hospitals and found 45 Stroke Units distributed unequally among regions. The ratio of SU beds to residents ranged from 1/74,000 to 1/1,037,000 inhabitants; only the regions of Cantabria and Navarre met the target. Neurologists performed 3,237 intravenous thrombolysis procedures in 83 hospitals; thrombolysis procedures compared to the total of ischaemic strokes yielded percentages ranging from 0.3 to 33.7%. Hospitals without SUs showed varying levels of available resources. Neuro VIR is performed in every region except La Rioja, and VIR is only available on a 24 h/7 d basis in 17 cities. Surgery for malignant MCA infarction is performed in 46 hospitals, and 5 have telemedicine. Conclusion: Stroke care has improved in terms of numbers of participating hospitals, the increased use of intravenous thrombolysis and endovascular procedures, and surgery for malignant MCA infarction. Implementation of SUs and telemedicine remain insufficient. The availability of diagnostic resources is good in most SUs and irregular in other hospitals. Regional governments should strive to ensure better care and territorial equality, which would achieve the EISNS objectives

Analysis of stroke care resources in Spain in 2012: have we benefitted from the Spanish Health System's stroke care strategy?

INTRODUCTION: The Spanish Health System's stroke care strategy (EISNS) is a consensus statement that was drawn up by various government bodies and scientific societies with the aim of improving quality throughout the care process and ensuring equality among regions. Our objective is to analyse existing healthcare resources and establish whether they have met EISNS targets. MATERIAL AND METHODS: The survey on available resources was conducted by a committee of neurologists representing each of Spain's regions; the same committee also conducted the survey of 2008. The items included were the number of stroke units (SU), their resources (monitoring, neurologists on call 24h/7d, nurse ratio, protocols), SU bed ratio/100,000 inhabitants, diagnostic resources (cardiac and cerebral arterial ultrasound, advanced neuroimaging), performing iv thrombolysis, neurovascular interventional radiology (neuro VIR), surgery for malignant middle cerebral artery (MCA) infarctions and telemedicine availability. RESULTS: We included data from 136 hospitals and found 45 Stroke Units distributed unequally among regions. The ratio of SU beds to residents ranged from 1/74,000 to 1/1,037,000 inhabitants; only the regions of Cantabria and Navarre met the target. Neurologists performed 3,237 intravenous thrombolysis procedures in 83 hospitals; thrombolysis procedures compared to the total of ischaemic strokes yielded percentages ranging from 0.3 to 33.7%. Hospitals without SUs showed varying levels of available resources. Neuro VIR is performed in every region except La Rioja, and VIR is only available on a 24h/7 d basis in 17 cities. Surgery for malignant MCA infarction is performed in 46 hospitals, and 5 have telemedicine. CONCLUSION: Stroke care has improved in terms of numbers of participating hospitals, the increased use of intravenous thrombolysis and endovascular procedures, and surgery for malignant MCA infarction. Implementation of SUs and telemedicine remain insufficient. The availability of diagnostic resources is good in most SUs and irregular in other hospitals. Regional governments should strive to ensure better care and territorial equality, which would achieve the EISNS objectives.

Recursos asistenciales en ictus en España 2010: análisis de una encuesta nacional del Grupo de Estudio de Enfermedades Cerebrovasculares / Health care resources for stroke patients in Spain, 2010: analysis of a national survey by the Cerebrovascular Diseases Study Group

Introduction: Stroke is currently a major social health problem. For this reason, the Spanish Ministry of Health approved the Stroke National Strategy (SNS) in 2008 to improve the prevention, treatment and rehabilitation of stroke patients. This plan intends to guarantee 24-hour, 365-days neurological assistance in the whole country by the end of 2010. Our aim was to analyse the situation of stroke assistance in Spain in 2009. Material and methods: A committee of neurologists practicing in the different autonomous communities (AC), and who had not participated in the preparation of the SNS, was created. A national survey was performed including the number of stroke units (SU) and their characteristics (monitoring, 24-h/7-day on-call neurology service, nursing staff ratio and the use of protocols), bed ratio of SU/100,000 people, availability of intravenous thrombolysis therapy, neurovascular intervention (NI) and telemedicine. Results: We included data from 145 hospitals. There are 39 SU in Spain, unevenly distributed. The ratio between SU bed/number of people/AC varied from 1/75,000 to 1/1,037,000 inhabitants; Navarra and Cantabria met the goal. Intravenous thrombolysis therapy is used in 80 hospitals; the number of treatments per AC was between 7 and 536 in 2008. NI was performed in the 63% of the AC, with a total of 28 qualified hospitals (although only 1 hospital performed it 24h, 7days a week in 2009). There were 3 hospitals offering clinical telemedicine services. Conclusions: Assistance for stroke patients has improved in Spain compared to previous years, but there are still some important differences between the AC that must be eliminated to achieve the objectives of the SNS (AU)

Introducción: El ictus constituye un importante problema sociosanitario. Por ese motivo, el Ministerio de Sanidad aprobó en 2008 la Estrategia Nacional en Ictus (ENI) con el objetivo de mejorar la prevención, tratamiento y rehabilitación del paciente con ictus. Se pretende garantizar una atención neurológica en todo el país y a cualquier hora del día para final del 2010. Nuestro objetivo fue analizar la situación de la atención al ictus en España en el año 2009. Material y métodos: Se constituyó un comité de neurólogos de las diferentes CC. AA. que no hubieran participado en la ENI. Se elaboró una encuesta nacional que recogió el número de unidades de ictus (UI) y la dotación (monitorización, guardia de neurología 24h/7 días, ratio de enfermería y existencia de protocolos), ratio cama UI/100.000 habitantes, presencia de trombólisis iv, intervencionismo neurovascular (INV) y telemedicina. Resultados: Se incluyeron datos de 145 hospitales. Existen 39 UI distribuidas de un modo desigual. La relación cama de UI/número de habitantes/comunidad autónoma osciló entre 1/75.000 a 1/1.037.000 habitantes, cumpliendo el objetivo Navarra y Cantabria. Se realiza trombólisis iv en 80 hospitales, el número osciló entre 7-536 tratamientos/CC. AA. durante el año 2008. Se realiza INV en el 63% de las CC. AA., teniendo 28 centros capacitados, aunque sólo 1 la realizaba en 2009 las 24h/7 día. Existen 3 centros con telemedicina. Conclusiones: La asistencia al ictus ha mejorado en España respecto a unos años atrás, pero todavía existen importantes desigualdades por CC. AA. que deberían superarse si se quiere cumplir el objetivo de la ENI (AU)

Health care resources for stroke patients in Spain, 2010: analysis of a national survey by the Cerebrovascular Diseases Study Group.

INTRODUCTION: Stroke is currently a major social health problem. For this reason, the Spanish Ministry of Health approved the Stroke National Strategy (SNS) in 2008 to improve the prevention, treatment and rehabilitation of stroke patients. This plan intends to guarantee 24-hour, 365-days neurological assistance in the whole country by the end of 2010. Our aim was to analyse the situation of stroke assistance in Spain in 2009. MATERIAL AND METHODS: A committee of neurologists practicing in the different autonomous communities (AC), and who had not participated in the preparation of the SNS, was created. A national survey was performed including the number of stroke units (SU) and their characteristics (monitoring, 24-h/7-day on-call neurology service, nursing staff ratio and the use of protocols), bed ratio of SU/100,000 people, availability of intravenous thrombolysis therapy, neurovascular intervention (NI) and telemedicine. RESULTS: We included data from 145 hospitals. There are 39 SU in Spain, unevenly distributed. The ratio between SU bed/number of people/AC varied from 1/75,000 to 1/1,037,000 inhabitants; Navarra and Cantabria met the goal. Intravenous thrombolysis therapy is used in 80 hospitals; the number of treatments per AC was between 7 and 536 in 2008. NI was performed in the 63% of the AC, with a total of 28 qualified hospitals (although only 1 hospital performed it 24h, 7 days a week in 2009). There were 3 hospitals offering clinical telemedicine services. CONCLUSIONS: Assistance for stroke patients has improved in Spain compared to previous years, but there are still some important differences between the AC that must be eliminated to achieve the objectives of the SNS.

Colestasias familiares: una causa común de hiperbilirrubinemia conjugada / Familial cholestasis: a comon cause of conjugated hyperbilurubinemia

Cholestasis is defined as a disorder affecting the production of bile resulting in the retention of its components in the liver and blood. In children, this disorder is almost always due to genetic alterations. Functionally, cholestasis may be the result of hepatic failure to secrete bile due to decrease in transport, synthesis or biliary obstruction. Extrahepatic cholestasis may be caused by biliary atresia and other obstructions of the bile ducts. Intrahepatic cholestasis may be the result of several disorders including progressive familial intrahepatic cholestasis (PFIC) types 1, 2 and 3, an autosomal recessive disease due to mutations in the genes ATP8B1, ABCBll and ABCB4 respectively. Pathophysiology and clinical presentation of this disease are now well understood. Clinically, these patients may present with jaundice, itching, anorexia, and generally unwell. Laboratory tests may disclose conjugated bilirubin over lmg/dl or larger than 20 percent of total bilirubin. Ursodeoxycholic acid, cholestiramine and biliary diversion may help in some of these conditions. Ongoing research into the mechanisms of genetic cholestasis could be key to therapy.

La Colestasia corresponde a un trastorno en la formación y excreción de la bilis que provoca retención de sus componentes y daño en hígado y sangre. La colestasia en el niño casi siempre se debe a una alteración hepática secundaria a causas ahora mayormente conocidas a nivel molecular. Desde el punto de vista funcional la colestasia resulta de una insuficiencia secretora del hígado debido a una disminución del flujo biliar por falla en los procesos de transporte o síntesis o a una obstrucción de la vía biliar. La colestasia extrahepática incluye la atresia de vías biliares y otras obstrucciones de la vía biliar. La colestasia intrahepática incluye las colestasias progresivas familiares PFIC 1, 2 y 3 causadas por fallas en los genes ATP8B1, ABCBll y ABCB4 respectivamente. Clínicamente pueden presentarse con ictericia, prurito, anorexia y compromiso del estado general. Desde el punto de vista del laboratorio las enfermedades colestásicas se caracterizan por hiperbilirrubinemia conjugada mayor a 1 mg/dl o mayor a 20 por ciento de bilirrubina total.

Microcyn: a novel super-oxidized water with neutral pH and disinfectant activity.

A new super-oxidized water (SOW) product, Microcyn, was tested for in vitro antimicrobial and antiviral activities. The effectiveness of this neutral-pH SOW at killing Staphylococcus aureus, Escherichia coli, Pseudomonas aeruginosa, Salmonella typhi and Candida albicans in pure culture was evaluated. One millilitre (approximately 10(8)colony-forming units/mL) of each micro-organism was subjected to 9 mL Microcyn or sterile water at room temperature for 30s. Under these conditions, a log(10) reduction factor of 8 in the level of all pathogens occurred in the treatment samples. In addition, results of tests with three batches of Microcyn exposed to Bacillus atrophaeus spores for 5 min demonstrated complete inactivation of the spores within 2-3 min (log(10) reduction factor >4). The effectiveness of Microcyn in reducing human immunodeficiency virus-1 (HIV-1) on hard surfaces (glass) was also evaluated in compliance with Environmental Protection Agency requirements for virucidal claims. After exposure of the tested surfaces to Microcyn for 5 min without agitation, there was a log(10) reduction factor >3 in the viral load as measured by both cytopathic effect and antigen p24 of HIV-1 production in MT-2 cultures. Microcyn activity against adenoviral vector type 5 was also analysed under simulated laboratory in-use conditions with viral suspensions. In order to increase the sensitivity of the test, the fluorescent light emitted by AdGFP-infected cells was measured with the use of a flow cytometer. A log(10) reduction factor >3 in the viral load was achieved after a 5-min exposure to Microcyn under these strict conditions. These results show that Microcyn exerts a wide antimicrobial spectrum with major advantages over acidic SOWs, including neutral pH, lower free active chlorine (51-85 ppm) and long shelf life (1 year).

Óxido nítrico exhalado en niños asmáticos tratados con corticoides inhalados / Exhaled nitric oxide in asthmatic children treated with inhaled corticosteroids

Objetivos: Estudiar los valores de óxido nítrico exhalado (ONE), la evolución clínica y la función pulmonar en una población de niños con asma episódica frecuente de nuevo diagnóstico, antes y después de recibir tratamiento con corticoides inhalados durante 8 semanas. Material y métodos: Estudio prospectivo de ámbito hospitalario en el que se analizaron distintos parámetros en un grupo de 21 niños asmáticos: 1. valores de ONE, mediante la técnica en T de exhalación lenta contra resistencia; 2. clínicos, evaluados mediante encuesta: episodios de sibilancias y síntomas nocturnos a la semana, y número de semanas que transcurrían entre las exacerbaciones asmáticas; 3. función pulmonar mediante espirometría. Resultados: Tras el tratamiento se constató: 1. las cifras de ONE disminuyeron de 22,47 antes del tratamiento a 7,95 ppb tras éste (p <0,0001); 2. mejoría clínica de los pacientes: disminución de los episodios de sibilancias de 2,68 días a la semana antes del tratamiento a 0,25 tras él (p <0,012); síntomas nocturnos de 2,45 días a la semana a 0,13 días (p <0,039); de una exacerbación asmática cada 3,64 semanas a una cada 7,94 (p <0,001); y 3. la función pulmonar mejoró en todos los parámetros evaluados FVC, FEV1, y FEF25-75%. Conclusiones: Los corticoides inhalados disminuyen los valores elevados de ONE en pacientes asmáticos. La medición de la concentración de ONE puede ser un medio complementario para monitorizar la inflamación en el tracto respiratorio inferior, y así poder valorar la eficacia de tratamientos antinflamatorios como los corticoides inhalados

Objectives. To study the exhaled nitric oxide (NO) levels, the clinical course and pulmonary function in a population of children with newly diagnosed asthma, before and after 8 weeks of treatment with inhaled corticosteroids. Material and methods. A prospective, hospital-based study was carried out to analyze different parameters in a group of 21 asthmatic children: the exhaled NO level, analyzed by the T-piece technique, which involves exhaling against expiratory resistance; clinical symptoms were evaluated by means of a survey to establish the number of episodes of wheezing and nocturnal symptoms a week and number of weeks between asthma exacerbations, and pulmonary function by spirometry. Results: Exhaled NO concentrations decreased from 22.47 ppb before treatment to 7.95 ppb afterwards (p <0.0001). Clinical symptoms improved after treatment. The frequency of episodes of wheezing decreased from 2.68 days a week before treatment to 0.25 after treatment (p <0.012), and nocturnal symptoms decreased from 2.45 days a week before treatment to 0.13 days (p <0.039). The children went from presenting an asthma exacerbation every 3.64 weeks before treatment to one every 7.94 weeks (p <0.001). Pulmonary function parameters (FVC, FEV1, MMEF25-75) improved after treatment. Conclusions: Inhaled corticosteroids decrease exhaled nitric oxide in asthmatic children. The measurement of expired NO concentrations can be used to monitor bronchial inflammation and, therefore, to evaluate the efficacy of antiinflammatory treatments such us inhaled corticosteroids

[Analysis of puberal development and influence of weight loss in obese adolescent girls]. / Análisis e influencia de la pérdida de peso en el desarrollo puberal de adolescentes obesas.

OBJECTIVES: To study several aspects of puberal development in obese adolescent girls, and the influence of weight loss on these aspects. METHODS: A longitudinal retrospective study was performed of a sample of 26 adolescent girls with normal weight and 46 obese adolescent girls at the onset of puberty. The obese teenagers were further divided into two groups (normal and obese) according to their body mass index (BMI) at the end of puberty. Height, chronological and bone age, and growth velocity were evaluated in both groups. RESULTS: Of the teenagers who were obese at the onset of puberty, 63 % remained obese at the end of puberty. The obese teenagers were significantly taller than non-obese teenagers at the onset of puberty (143.2 +/- 6.96 vs 138.9 +/- 5.95 cm, respectively; p < 0.01). However, there were no differences between the two groups in final height. No differences were found between obese teenagers who lost weight and those who did not. There were no differences in chronological or bone age throughout puberal development in any of the groups. The mean growth velocity during puberty was significantly lower in obese teenagers than in non-obese teenagers (6.18 +/- 1.94 and 6.90 +/- 127 cm/year, respectively; p < 0.02). However, there were no differences between obese teenagers who lost weight and those who did not. CONCLUSIONS: Height gain in obese girls is greater in childhood but lower in adolescence. Final height is similar in both groups. Chronological age at the onset and end of puberty and bone maturation are similar in both groups. Weight loss during puberty does not modify growth pattern during this period of development.

Análisis e influencia de la pérdida de peso en el desarrollo puberal de adolescentes obesas / Analysis of puberal development and influence of weight loss in obese adolescent girls

Objetivos: Estudiar aspectos del desarrollo puberal en adolescentes obesas, y la influencia de la pérdida de peso en las mismas. Métodos: Estudio retrospectivo y longitudinal de 26 adolescentes con peso normal y 46 obesas al inicio de la pubertad. Estas últimas se dividieron en dos grupos (normales y obesas) según su índice de masa corporal (IMC) al final de la pubertad. Se comparan: talla, edad cronológica, ósea y velocidad de crecimiento en los tres grupos. Resultados: De las adolescentes obesas al iniciar la pubertad, el 63 por ciento permanecieron obesas al finalizarla. Las adolescentes obesas estudiadas tenían una talla significativamente mayor que las no obesas al inicio de la pubertad (143,2 +/- 6,96 y 138,9 +/- 5,95 cm, respectivamente; p < 0,01). Sin embargo, no existieron diferencias significativas en las tallas finales de ambos grupos; tampoco entre las obesas que perdieron peso y las que no. No hubo diferencias en las edades cronológicas ni óseas a lo largo del desarrollo puberal en ninguno de los grupos. La velocidad de crecimiento global durante la pubertad en el grupo de obesas fue significativamente menor que en el grupo control (6,18 +/- 1,94 y 6,90 +/- 1,27 cm/año, respectivamente; p < 0,02). Sin embargo, no hubo diferencias entre las obesas que perdieron peso y las que no. Conclusiones: Las obesas tienen una ganancia de talla superior en la infancia pero inferior durante la adolescencia. La talla final es parecida en ambos grupos. El inicio y final de la pubertad son parejos y la maduración ósea similar. La pérdida de peso de las obesas durante la pubertad no altera el patrón global de crecimiento (AU)

Adenitis tuberculosa abdominal: a propósito de un caso / Abdominal tuberculous lympnadenitis: a case report

Se presenta el caso de una niña de 11 años con una masa pélvica izquierda calcificada, descubierta al practicar una radiografía abdominal tras consultar por dolor abdominal crónico y en cadera derecha reciente, y que corresponde a una adenitis tuberculosa granulomatosa paranexial izquierda caseificada y calcificada, en forma seudotumoral por confluencia de ganglios caseificados. Se expone un breve comentario acerca de la tuberculosis abdominal y un diagnóstico diferencial de las masas pélvicas y las linfadenitis granulomatosas mediante la exposición del diagnóstico diferencial del caso presentado y su diagnóstico final (AU)

Dynamic intracoronary thrombosis does not cause significant downstream platelet embolization.

OBJECTIVE: A mural intracoronary thrombus is a potential source of platelet emboli that may obstruct downstream microvessels, but this phenomenon has not been characterized. The present study aimed to assess the magnitude of myocardial platelet accumulation downstream of a mural intracoronary thrombus and its modification by a concomitant transient coronary occlusion (OC) or by treatment with aspirin. METHODS: The myocardial content of 99mTc-labelled platelets was analyzed in 26 pigs submitted to intimal injury of the left anterior descending coronary artery (LAD) followed by no intervention (n=6), 25-min OC (n=6), or 48-min OC preceded (n=8) or not (n=6) by intravenous administration of 250 mg aspirin. RESULTS: After 2 h, 24 animals had had 12+/-1 cyclic flow reductions (CFRs) reflecting dynamic LAD thrombosis. Myocardial platelet content in the inferior region was similar among groups. Platelet content in the LAD region was not significantly different to that in the inferior region (129+/-19%, P=NS) in the no intervention group, but was increased following OC (172+/-20 and 312+/-71% after 25- and 48-min OC, respectively, P<0.05). Pre-treatment with aspirin lessened the number of CFRs but did not reduce platelet accumulation in LAD myocardium (483+/-148%). Myocardial platelet accumulation was not associated with the magnitude of platelet deposition in the LAD nor with the number of CFRs, but was correlated with myeloperoxidase activity (r=0.91, P<0.001) and with infarct size (r=0.52, P=0.05). Histological analysis frequently showed sparse platelets or small platelet or leukoplatelet aggregates in small vessels, but arteriolar emboli were rare. In none of seven additional experiments coronary angiography showed obstructions of arterial branches during CFRs. CONCLUSION: The magnitude of platelet embolization from a mural intracoronary thrombus into downstream myocardium is small despite the presence of repetitive CFRs.