Survival of the nematophagous fungus Duddingtonia flagrans to in vitro segments of sheep gastrointestinal tract.

The nematophagous fungus Duddingtonia flagrans is used in integrated management of gastrointestinal nematodes in ruminants. The chlamydospores of the fungus, orally administered, pass through the segments of the ruminant digestive tract and, in the feces, capture the nematodes preventing their migration to grasslands. The drastic conditions of the gastrointestinal segments can negatively affect the fungus' biocontrol activity. The aim of this study was to assess the effect of in vitro conditions of the sheep's main gastrointestinal segments on the concentration, viability and nematode predatory ability of D. flagrans chlamydospores. The segments evaluated separately in vitro were the oral cavity, rumen, abomasum, and small intestine. The results showed that chlamydospores concentration was not affected by exposure to the different segments. The viability of the chlamydospores after exposure to the oral cavity (2.53 × 106 CFU/mL) and small intestine (1.24 × 105 CFU/mL) was significantly lower than its control treatment, with values of 6.67 × 106 CFU/mL and 2.31 × 105 CFU/mL respectively. Nematode predatory ability after rumen exposure was reduced by 7% compared to the control treatment, by 25% after abomasum exposure and by 17% after small intestine. This study revealed the individual in vitro effect of each segment of ovine gastrointestinal tract on the integrity of this strain of the fungus D. flagrans affecting its viability and nematode predatory ability under the evaluated conditions. Delivery systems could be designed to protect chlamydospores considering the impact of each gastrointestinal segment.

[Does Alzheimer's disease exist in all primates? Alzheimer pathology in non-human primates and its pathophysiological implications (II)]. / ¿Existe la enfermedad de Alzheimer en todos los primates? Patología Alzheimer en primates no humanos y sus implicaciones fisiopatológicas (II).

INTRODUCTION: In the ageing process there are some species of non-human primates which can show some of the defining characteristics of the Alzheimer's disease (AD) of man, both in neuropathological changes and cognitive-behavioural symptoms. The study of these species is of prime importance to understand AD and develop therapies to combat this neurodegenerative disease. DEVELOPMENT: In this second part of the study, these AD features are discussed in the most important non-experimental AD models (Mouse Lemur -Microcebus murinus, Caribbean vervet -Chlorocebus aethiops, and the Rhesus and stump-tailed macaque -Macaca mulatta and M. arctoides) and experimental models (lesional, neurotoxic, pharmacological, immunological, etc.) non-human primates. In all these models cerebral amyloid neuropathology can occur in senility, although with different levels of incidence (100% in vervets;<30% in macaques). The differences between normal and pathological (Alzheimer's) senility in these species are difficult to establish due to the lack of cognitive-behavioural studies in the many groups analysed, as well as the controversy in the results of these studies when they were carried out. However, in some macaques, a correlation between a high degree of functional brain impairment and a large number of neuropathological changes ("possible AD") has been found. CONCLUSIONS: In some non-human primates, such as the macaque, the existence of a possible continuum between "normal" ageing process, "normal" ageing with no deep neuropathological and cognitive-behavioural changes, and "pathological ageing" (or "Alzheimer type ageing"), may be considered. In other cases, such as the Caribbean vervet, neuropathological changes are constant and quite marked, but its impact on cognition and behaviour does not seem to be very important. This does assume the possible existence in the human senile physiological regression of a stable phase without dementia even if neuropathological changes appeared.

¿La enfermedad de Alzheimer existe en todos los primates? Alzheimer patología en los primates no humanos y sus implicaciones fisiopatológicas (I) / Does Alzheimer's disease exist in all primates? Alzheimer pathology in non-human primates and its pathophysiological implications (I)

Introducción: Muchas publicaciones consideran que la enfermedad de Alzheimer (EA) es exclusivo de la especie humana, y que ningún otra especie animal sufre de la enfermedad. Sin embargo, diversos estudios han demostrado que algunas especies pueden presentar algunas de las características definitorias de la enfermedad humana, incluyendo tanto los cambios neuropatológicos y síntomas cognitivo-conductuales. Desarrollo: En este trabajo, los resultados publicados (PubMed) sobre cambios en el cerebro senil en los primates no humanos con diferentes grados de evolución, se revisan. Los cambios neuropatológicos asociados con la acumulación de amiloide o proteína tau fosforilada altamente son raras fuera del orden de los primates, pero en todos los sub-órdenes, familias, géneros y especies de primates no humanos que se han estudiado, algunos individuos seniles han demostrado amiloide acumulación en el cerebro. De hecho, en algunas especies la presencia de estos depósitos en la senilidad es constante. Cambios relacionados con la acumulación de la proteína tau son siempre de muy poca importancia, y se han detectado sólo en algunas especies de primates no humanos, tanto poco evolucionados y altamente evolucionada. En diferentes especies de primates no humanos, algunos tipos de cambios cognitivo-conductuales son más comunes en algunos individuos seniles en comparación con los individuos adultos normales y otras personas seniles de la especie. La importancia de determinar la longevidad de la especie en hábitats diferentes hábitats naturales, los hábitats nuevos, semi-cautividad, cautividad) se hace hincapié en estos estudios. Conclusiones: Las características morfológicas, histoquímicas y cognitivo-conductuales similares a los observados en los seres humanos de edad avanzada están presentes en seniles los primates no humanos. Además, otras características se observan en los primates no humanos podría ser indicativo de una patología «tipo Alzheimer» envejecimiento (AU)

Introduction: Many publications consider that Alzheimer's disease (AD) is exclusive to the human species, and that no other animal species suffers from the disease. However, various studies have shown that some species can present with some of the defining characteristics of the human disease, including both neuropathological changes and cognitive-behavioural symptoms. Development: In this work, the results published (PubMed) on senile brain changes in non-human primates of different degrees of evolution, are reviewed. The neuropathological changes associated with the accumulation of amyloid or highly phosphorylated tau protein are rare outside the primate order, but in all the sub-orders, families, genera and species of non-human primates that have been studied, some senile individuals have shown amyloid accumulation in the brain. In fact, in some species the presence of these deposits in senility is constant. Changes related to the accumulation of tau protein are always of very little significance, and have been detected only in some non-human primate species, both little evolved and highly evolved. In different species of non-human primates, some types of cognitive-behavioural changes are more common in some senile individuals when compared with both normal adult individuals and other senile individuals of the species. The importance of determining the longevity of the species in different habitats (natural habitats, new habitats, semi-captivity, captivity) is stressed in these studies. Conclusions: Morphological, histochemical and cognitive-behavioural features similar to those observed in elderly humans are present in senile non-human primates. Moreover, other characteristics seen in non-human primates could be indicative of a pathological «Alzheimer type» ageing (AU)

[Does Alzheimer's disease exist in all primates? Alzheimer pathology in non-human primates and its pathophysiological implications (I)]. / ¿Existe la enfermedad de Alzheimer en todos los primates? Afección de Alzheimer en primates no humanos y sus implicaciones fisiopatológicas (I).

INTRODUCTION: Many publications consider that Alzheimer's disease (AD) is exclusive to the human species, and that no other animal species suffers from the disease. However, various studies have shown that some species can present with some of the defining characteristics of the human disease, including both neuropathological changes and cognitive-behavioural symptoms. DEVELOPMENT: In this work, the results published (PubMed) on senile brain changes in non-human primates of different degrees of evolution, are reviewed. The neuropathological changes associated with the accumulation of amyloid or highly phosphorylated tau protein are rare outside the primate order, but in all the sub-orders, families, genera and species of non-human primates that have been studied, some senile individuals have shown amyloid accumulation in the brain. In fact, in some species the presence of these deposits in senility is constant. Changes related to the accumulation of tau protein are always of very little significance, and have been detected only in some non-human primate species, both little evolved and highly evolved. In different species of non-human primates, some types of cognitive-behavioural changes are more common in some senile individuals when compared with both normal adult individuals and other senile individuals of the species. The importance of determining the longevity of the species in different habitats (natural habitats, new habitats, semi-captivity, captivity) is stressed in these studies. CONCLUSIONS: Morphological, histochemical and cognitive-behavioural features similar to those observed in elderly humans are present in senile non-human primates. Moreover, other characteristics seen in non-human primates could be indicative of a pathological «Alzheimer type¼ ageing.

Fístula aortoentérica post-EVAR. Presentación de un caso y revisión de la literatura / Post-EVAR aortoenteric fistula. Presentation of a case and a review of the literature

El tratamiento endovascular del aneurisma de aorta abdominal (EVAR) no previene la aparición de fístula aortoentérica (FAE), rara y devastadora complicación. Su etiopatogenia es imprecisa, atribuyéndose a migración, angulación o dislocación del endoinjerto, a daño directo del intestino por erosión mecánica, o por presurización, inflamación, infección o rotura del aneurisma, pero también puede aparecer sin fallos en el dispositivo implantado y con pruebas de seguimiento de imagen normales. La clínica es larvada, por eso requiere un alto índice de sospecha. La angio-TC es el mejor método diagnóstico. El tratamiento precoz es esencial para obtener buenos resultados: consiste en la explantación del endoinjerto y la revascularización aórtica directa o extraanatómica. Presentamos el caso de un paciente varón de 73 años, portador de AAA, con alto riesgo quirúrgico, que fue tratado con endoprótesis aortomonoiliaca e injerto cruzado. Seis meses después de EVAR presentó una FAE, con evacuación del contenido aneurismático por vía digestiva.Con este se han publicado un total de 32 casos en la literatura. Es necesaria una constante vigilancia de la aparición de complicaciones en los pacientes sometidos a EVAR(AU)

Endovascular aneurysm repair (EVAR) is not immune to aorto-enteric fistulas (AEF), a rare and devastating high death rate complication. Pathogenesis is not clear, and may be attributed to graft migration, erosion, kinking, dislodging or adjacent organ injury due to mechanical forces, or aneurysm pressurisation, inflammation, infection and rupture. But AEF may appear despite accurate device placement or without stent or aortic failure in follow-up imaging studies. Symptoms are usually masked. A high level of suspiction, early recognition and treatment are essential for successful outcome. CT angiography is better than endoscopy for diagnosis. Surgical treatment consists of graft removal and direct aortic replacement or extra-anatomic bypass. A new case of post-EVAR AEF and a review of the literature reports a total o 32 cases. We emphasise the need for continued awareness in patients undergoing EVAR(AU)

Tratamiento endovascular del síndrome de robo vertebrosubclavio por obstrucción subclavia proximal. A propósito de dos casos / Endovascular treatment for vertebral-subclavian steal syndrome due t obstruction of the proximal subclavin artery. Two cases reports

Introducción. La obstrucción de la arteria subclavia prevertebral (ASP) es una causa importante de enfermedadcerebrovascular de origen extracraneal. Presentamos nuestra experiencia en el tratamiento endovascular de dos pacientescon obstrucción de la ASP que se acompañaba de síndrome de robo vertebrosubclavio sintomático. Casos clínicos.Caso 1: varón de 64 años, ingresado por ictus parietooccipital derecho. En el eco-Doppler de troncos supraaórticosse detectó la obstrucción completa de la carótida interna derecha e inversión del flujo en la arteria vertebral izquierda.En la angiorresonancia se objetivó una oclusión de la ASP izquierda. Caso 2: varón de 75 años, intervenido hace 10 añosde tromboendarterectomía de la carótida interna derecha, sin nuevos eventos neurológicos hasta la fecha. Presentó síncopesde repetición en el mes previo. En la arteriografía de troncos supraaórticos se comprobó la permeabilidad de lacarótida interna derecha, sin reestenosis. Se objetivó además la obstrucción de la ASP, que condicionaba un fenómeno derobo vertebrosubclavio. Por abordaje humeral ipsilateral, en cada caso se recanalizó la lesión y se implantó un stent balónexpandiblede 8 × 37 mm en la ASP, con buen resultado en la angiografía de control. En ambos casos, el postoperatoriotranscurrió sin complicaciones isquémicas neurológicas ni en el miembro superior. En controles con tomografía cranealy eco-Doppler de troncos supraaórticos al mes y a los tres y seis meses, se comprobó un flujo ortógrado en la arteria vertebral.Conclusiones. El tratamiento endovascular de las lesiones de la ASP que cursan con síndrome de robo vertebrosubclavioes una opción terapéutica eficaz, incluso en obstrucciones completas(AU)

Introduction. Obstruction of the prevertebral subclavian artery (PSA) is an important cause of cerebrovasculardisease with an extracranial origin. We report our experience in the endovascular treatment of two patientswith obstruction of the PSA accompanied by symptomatic vertebral-subclavian steal syndrome. Case reports. Case 1: a64-year-old male, admitted to hospital due to right-side parietal-occipital stroke. Doppler ultrasound recording of thesupra-aortic trunks revealed complete obstruction of the right internal carotid and reversed flow in the left vertebralartery. The MR angiography scan showed occlusion of the left PSA. Case 2: a 75-year-old male who had undergonesurgery 10 years before to perform a thromboendarterectomy of the right internal carotid artery, with no new neurologicalevents since then. The patient had suffered recurrent syncopes during the previous month. The arteriography of thesupra-aortic trunks confirmed the patency of the right internal carotid artery, without restenosis. Obstruction of the PSAwas also observed, which was conditioning a vertebral-subclavian steal syndrome. By means of an ipsilateral humeralapproach, in each case the lesion was recanalised and an 8 × 37 mm expandable-balloon stent was placed in the PSA,with good results in the follow-up angiography. In both cases there were no complications of a neurological ischaemicnature or in the upper limb in the post-operative period. In control tests using cranial tomography and Dopplerultrasound recording of the supra-aortic trunks carried out at one, three and six months, orthograde flow was found inthe vertebral artery. Conclusions. Endovascular treatment of lesions to the PSA that are accompanied by vertebralsubclaviansteal syndrome is an effective therapeutic option, even in cases of complete obstruction(AU)

Concordance between the Piko - 1 portable device and pneumotachography in measuring PEF and FEV(1) in asthmatic children.

OBJECTIVE: To assess concordance in the measurement of peak expiratory flow (PEF) and forced expiratory volume ino ne second (FEV(1)) between the portable device Piko-1 (Ferraris) and a pneumotachograph. PATIENTS AND METHODS: Forced spirometry (Master Screen Jaeger) was performed according to ATS/ERS norms, selecting the best value of three curves, and three measurements with the Piko-1 were recorded the recommendations of the manufacturer. RESULTS: Eighty patients between 5-18 years of age were studied. Based on the Bland-Altman method, the mean differences obtained were 9.82 (95%Cl: 2.43-17.21) for PEF and 0.17 (95%CL: 0.12-0.21 for FEV(1). The intraclass correlation coefficient was 0.96 (p <0,001; 95%Cl: 0.93-0.97) for PEV(1) and 0.93 (p<0,0001; 95%Cl: 0.89-0.95) for PEF. CONCLUSIONS: Piko-1 offers FEV(1) measurements close to those obtained with forced spirometry, thus allowing more exact patient assessment in home-based follow-up emergency services, or hospital wards.

Genes involved in mitochondrial biogenesis/function are induced in response to bilio-pancreatic diversion in morbidly obese individuals with normal glucose tolerance but not in type 2 diabetic patients.

AIMS/HYPOTHESIS: The mechanisms allowing normalisation of insulin sensitivity and reversal of type 2 diabetes after bilio-pancreatic diversion (BPD) have not been elucidated. We studied whether the expression of genes relevant to mitochondrial biogenesis/function is induced in response to BPD and whether the response differs between morbidly obese patients with normal glucose tolerance (NGT) and patients with type 2 diabetes. METHODS: The effect of stable weight reduction after BPD on metabolic variables and expression of nuclear genes encoding for mitochondrial proteins or regulators of mitochondrial function was investigated in skeletal muscle. Insulin sensitivity was assessed by euglycaemic-hyperinsulinaemic clamp and substrate oxidation by indirect calorimetry. RESULTS: Both NGT and type 2 diabetic patients showed a net improvement of insulin sensitivity, with the latter also showing blood glucose normalisation. NGT patients had a large increase in glucose oxidation and substantial reduction in lipid oxidation. In contrast, type 2 diabetic patients had a blunted response to BPD in terms of glucose oxidation. NGT patients showed increased expression of genes encoding mitofusin 2, porin or citrate synthase; no significant changes were detected in diabetic patients. The expression of genes regulating mitochondrial activity (PGC-1beta [also known as PPARGC1B], PGC-1alpha [also known as PPARGC1A], PPARdelta [also known as PPARD], SIRT1) was induced only in NGT patients. CONCLUSIONS/INTERPRETATION: These findings indicate that weight loss after BPD exerts a beneficial effect on insulin sensitivity via mechanisms that are independent of the expression of genes involved in mitochondrial biogenesis/activity. Furthermore, the observation that gene expression is not altered with weight loss in type 2 diabetic patients while it is induced in NGT patients suggests a heritable component.

[Bronchodilator test: is 9% of the predicted value the same as 12% of the initial value]. / Prueba de broncodilatación: ¿9% sobre el valor teórico o 12% sobre el valor basal es lo mismo?

INTRODUCTION: The bronchodilator test (BDT) is an important tool used in pulmonary function. Changes in forced expiratory volume in one second (FEV1) can be expressed as absolute change, or per cent of initial or predicted value. When the initial value is used, there may be a bias, as the smaller this value is, the greater the response will be. The main objective of this study is to establish whether there is any difference in using per cent of the initial spirometry value or per cent of the predicted value in order to consider a bronchodilator test positive, and if the initial obstruction of the patient influences such differences. MATERIAL AND METHODS: A retrospective analysis of the BDT made between October 1997 and February 2008. The results using an increase of 9% from the predicted FEV1 were compared with using 12% from the initial FEV1. The patients were divided into three groups depending on initial obstruction: no obstruction (FEV1>80% of predicted), mild (FEV1=60-80% of predicted) and moderate-severe (FEV1<60% of predicted). The kappa index of agreement between both methods was calculated. RESULTS: A total of 4352 BDT were analysed. The agreement between both methods was high (k=0.832). In the group without initial obstruction (N=3007) the kappa index was 0.781, in the mild obstruction group (N=1067) the kappa index was 0.966 and in the moderate-severe group (N=278) it was 0.788. CONCLUSION: This study demonstrates that, although there is a good agreement between both methods, in patients with initial moderate-severe obstruction and in patients without initial obstruction this agreement tends to be lower.

Tratamiento endovascular de aneurismas subclavios. A propósito de dos casos / Endovascular treatment of subclavian aneurysms. Two case reports

Introducción. Los aneurismas de la arteria subclavia intratorácica (AASI) son infrecuentes. La cirugía convencionalconlleva una alta morbimortalidad. Presentamos nuestra experiencia con dos casos de AASI complejos intervenidos con técnica endovascular. Casos clínicos. Caso 1: mujer de 74 años. Acude a urgencias por disnea aguda. En la radiografía torácica se observan un gran derrame pleural y una masa en el lóbulo pulmonar superior izquierdo. Con la sospecha de la presencia de una neoplasia pulmonar se realiza angiotomografía en la que se detecta un AASI izquierdo con signos de rotura, sacular, de 60 mm de diámetro, así como una atelectasia del pulmón izquierdo. Mediante el empleode toracocentesis, el derrame pleural se ha identificado como un hemotórax masivo. La paciente ha sido intervenida de urgencia, se le ha insertado una endoprótesis subclavia izquierda y se le ha realizado un drenaje pleural. Caso 2: varón de 76 años, con una arteria subclavia derecha aberrante (ASDA) detectada mediante angiografía realizada antes de una endarterectomía carotídea llevada a cabo cinco años antes. Presenta disfagia progresiva y deterioro del estado general, y se sospecha la existencia de una neoplasia esofágica. En el estudio con angiotomografía torácica se ha objetivado la presencia de un aneurisma del origen de la ASDA de 32 mm de diámetro que comprime el esófago. Se ha descartado la existencia de un proceso neoplásico. El paciente ha sido intervenido colocando una endoprótesis en la ASDA. No ha habido complicaciones intraoperatorias. El caso 1 presentaba, además, un aneurisma de la aorta abdominal que fue reparado de forma endovascular tres meses después. Continúa asintomática, y no se han observado complicaciones en las pruebas de imagen. El caso 2 ha presentado una mejoría subjetiva de la disfagia, y a los tres meses la prótesis ha continuado permeable y sin fugas, aunque el paciente falleció a los cuatro meses de la intervención a causa de un infarto de miocardio(AU)

Conclusión. El tratamiento endovascular de los AASI es posible, con buenos resultados y muy baja morbimortalidad(AU)

Introduction. Aneurysms of the intrathoracic subclavian artery (AISA) are rare. Conventional surgery entailsa high rate of morbidity and mortality. We report our experience with two complex cases of AISA that were treated using endovascular surgical techniques. Case reports. The first case was a 74-year-old female. This patient visited the emergency department due to acute dyspnea. An x-ray of the thorax showed an important pleural effusion and a mass in the left upper lobe of the lung. In the suspected presence of pulmonary neoplasia, an MR-angiography was performed in which a left AISA was detected with signs of rupture, a saccular shape, and a diameter of 60 mm, as well as atelectasis in the left lung. By means of a thoracentesis, the pleural effusion was identified as being a massive haemothorax. The patient was submitted to an emergency operation, a stent was placed in the left subclavian, and pleural drainage was performed. The second of our cases was a 76-year-old male, with an aberrant right subclavian artery (ARSA) that had been detected by means of an angiography carried out before a carotid endarterectomy performed five years earlier. The patient presented progressive dysphagia and a general deterioration of his status, and the existence of oesophagealneoplasia was suspected. A CT-angiography study of the thorax revealed the presence of an aneurysm in the origin of the ARSA with a diameter of 32 mm that was constricting the oesophagus. The presence of a neoplastic process was ruled out. The patient was submitted to surgery to place a stent in the ARSA(AU)

There were no intraoperative complications. The woman also presented an abdominal aortic aneurysm (AAA) that was operated on by an endovascular procedure three months later. She remains asymptomatic and no complications have been observed in the imaging tests. The man’s dysphagia improved subjectively, and at three months the stent was still patent and free of leaks, although he died fourmonths after the operation due to a myocar. Conclusions. Endovascular treatment of AISA is possible, withgood results and very low morbidity and mortality ratesdial infarct(AU)

Immunohistochemical increase in cyclooxygenase-2 without apoptosis in different brain areas of subchronic nicotine- and D-amphetamine-treated rats.

Cyclooxygenase-2 (COX-2) upregulation has been related to both neurodegeneration and physiological processes. To clarify whether nicotine-induced upregulation of COX-2 occurs, and to analyse its significance, a comparative immunohistochemical and Western blot study was performed on the frontoparietal cortex, hippocampus and cerebellar cortex of rats treated (14 days) with nicotine, D(+)amphetamine (0.35 and 1.16 mg free base/kg/day, respectively), or both drugs simultaneously. None of these treatments promoted neuronal apoptosis. Lipid peroxidation increased in the hippocampus of the nicotine-treated rats and in all the brain regions examined in the D(+)amphetamine rats, but not in the double-treated animals. Both molecules increased the COX-2 content (as determined by the number of immunopositive neurons and the intensity of their immunodeposits) in an area-, layer- and neuron type-dependent manner, in all brain regions in which a large number of COX-2 immunopositive neurons were observed in controls (the somatosensory cortical areas, CA-1, CA-3, the gyrus dentatus, the ectorhinal/perirhinal areas, and the gyrus cingularis). No increase was seen in the motor cortical areas, while a reduction was recorded in the cerebellar cortex; these regions had only a few immunopositive neurons in controls. Western blot analysis revealed a 50-80% increase in COX-2 in the brain cortex and hippocampus of nicotine-treated rats, and similar increases (150-200%) in the cortex of the D(+)amphetamine- and nicotine + D(+)amphetamine-treated rats. Nicotine-induced upregulation of COX-2 seems to be related to neuronal plasticity rather than neurodegeneration. Nicotine agonists might be useful in the treatment of cognitive disorders.

Exacerbación respiratoria en fibrosis quística / Respiratory exacerbation in Cystic Fibrosis

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[Lung function changes after haematopoietic stem cell transplantation]. / Alteraciones de la función pulmonar tras el trasplante de progenitores hematopoyéticos.

OBJECTIVE: To evaluate lung function abnormalities in children who underwent haematopoietic stem cell transplantation (HSCT) and to compare these abnormalities between autologous and allogenic transplantation. PATIENTS AND METHODS: Prospective observational study from 1996 to 2005. Ninety-three children receiving HSCT, 47 autologous and 46 allogenic, were included. Lung function tests were performed before transplantation and at 2, 6, 12 and 24 months afterwards. The following indices were determined: forced expiratory volume in 1 s (FEV1), FEV1/forced vital capacity (FVC), total lung capacity (TLC), and carbon monoxide diffusing capacity (DLCO). Paired Student's t-test was used for statistical analysis of data. RESULTS: Before HSCT, 6.8% of the children had FEV1<80%, 1% FEV1/FVC<80%, 7.8% TLC<80% and 13.5% DLCO<70%. At 2 months, FEV1/FVC, TLC and DLCO were significantly reduced, when compared to pre-transplantation values (p=0.05, 0.011 and p<0.001, respectively). Lung function gradually improved from 6 months post-transplantation, but did not reach pre-transplantation values at 24 months. No significant differences were found when comparing allogenic and autologous transplantation, apart from a lower FEV1/FVC value at 6 months (p=0.02) in the first group. CONCLUSIONS: An important proportion of children who undergo HSCT have early pulmonary abnormalities (at 2 and 6 months after transplantation) with partial recovery at 24 months.

Alteraciones de los fenómenos de neuroplasticidad en las enfermedades de Alzheimer y Creutzfeldt-Jakob. Interrelaciones con fenómenos involutivos / Neuroplasticity changes in Alzheimer´s and Creutztfeldt-Jakob´s Diseases. Relationships to involutive phenomena

Se ha postulado que en las enfermedades neurodegenerativas,las alteraciones en los fenómenos de neuroplasticidady adaptación tienen una función muy importante,como desencadenante o motor del curso patogénico delas enfermedades. Para aclarar este extremo se ha llevadoa cabo un estudio morfohistoquímico comparativo en lacorteza cerebral prefrontal y en la corteza cerebelosa(neocerebelo) de cerebros de enfermos de Alzheimer yCreutzfeldt-Jakob (EA y ECJ). Se han analizado las variacionesen marcadores supuestamente relacionados confenómenos de neuroplasticidad sináptica (Drebrina, SNAP-25), factor de activación nuclear (NF kappa Beta -NFkB) eisoforma neuronal de la óxido nítrico sintasa (nNOS) juntoa marcardores de neuropatología (acumulación de proteínasbeta –amiloide en EA y PrPsc en ECJ-, reacción microgliale inducción de enzimas pro-inflamatorias iNOS yciclo-oxigenasa 2 (COX-2). Los resultados han mostradograndes variaciones de los marcadores entre ambos procesospatológicos, entre las cortezas cerebral y cerebelosa,entre diferentes áreas de esas regiones y entre diferentesestirpes neuronales y gliales. El significado de algunosde los marcadores (NFkB, nNOS, proteínas sinápticas) puede ser variable (plástico o involutivo) según los casos(enfermedad, región, factores). La neuroplasticidad disminuyede manera diversa según las áreas cerebrales y susrelaciones con las manifestaciones neuropatológicas tambiénson variables, aunque son manifiestos fenómenos deneuroplasticidad/adaptación en muchas áreas y neuronas.Se concluye que la activación de los supuestos marcadoresde neuroplasticidad en fases avanzadas de la enfermedad,con una idea terapéutica, puede activar fenómenosinvolutivos en algunas regiones o neuronas del cerebro

Changes in neuroplasticity and neuronal adaptativemechanisms have been postulated as origin and/or mainpathophysiological factor in neurodegenerative diseases.To analyze these theories, a comparative morphohistochemicalstudy on the cerebral (prefrontal) and cerebellar(neocerebellar) cortex from Alzheimer´s (AD) and Creutzfeldt-Jakob (CJD) post-mortem brains has been carriedout. Variations in putative markers of neuroplasticity andneuronal adaptation (synaptic proteins such as drebrinand SNAP-25; nuclear factor NF kappa Beta –NFkB-; neuronalisoform of oxide nitric synthase -nNOS) have beenstudied in close association with neuropathological markers(beta-protein deposition – amyloid in AD and PrPsc inCJD-; microglial activation, induction of iNOS and cyclooxygenase2 –COX-2). Results have shown sharp variationsin these markers when compared AD and CJD; cerebraland cerebellar cortex; different areas of these anatomicalregions; and different sets of neurons and glial cells.The meaining of somme of these markers (NFkB; nNOS;synaptic proteins) could be variable (plastic/adaptative orinvolutive), depending on different factors (disease, anatomicalregion, general or local factors, etc.). Neuroplasticity is evident in several brain regions or neurons, but this neuronalfeature decreases in different form depending alsoon the disease and the anatomical region. Their relationshipsto the neuropathological findings were also variable.In conclusion, the activation of these putative markers ofneuroplasticity, considering as therapeutical targets, inadvanced steps of the diseases, could activate neuronalinvolutive phenomena in several regions or neurons

Mioclonías desencadenadas por diclofenaco / Myoclonias triggered by diclofenac

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Lesions and dysfunctions of the nucleus basalis as Alzheimer's disease models: general and critical overview and analysis of the long-term changes in several excitotoxic models.

The cholinergic hypothesis of Alzheimer's Disease (AD) has led to a number of animal models to study in vivo the pathogeny of cortical cholinergic involution. The lesion of the cholinergic neurons of the basal forebrain, especially of the nucleus basalis magnocellularis (nbm) of rodents, has been the most utilized method for obtaining these models. Toxic substances such as quinolic, kainic, NMDA, ibotenic and quisqualic acids, the specific cholinergic toxin AF64, amyloid, and antibodies to neurotrophic factors; etc, have been used to produce such lesions. These investigations have helped our understanding of the role of cerebral cholinergic innervation in cognitive disorders and their treatments. However, this research has provided conflicting results, and much controversy has developed surrounding the role of the cholinergic systems and the suitability of these models. It is very important to take into account the exact type of nbm/cortical lesion produced, and its evolution, if meaningful results are to be obtained. This review covers the theoretical and practical use of nbm lesion models, and examines the main positive and negative results obtained by different authors in the light of our own observations on the long-term (3 years) morphological and biochemical changes that occur in several kinds of nbm-lesion model rats. The changes seen were very different, but many of them were increased up to the end of life with no clear relationship with the development of the original lesion.

[Ring chromosome 20, hypersensitivity to valproate and hyperammonemic encephalopathy]. / Cromosoma 20 en anillo, hipersensibilidad al valproato y encefalopatía hiperamoniémica.

INTRODUCTION: Ring chromosome 20 syndrome (C20A) is characterised by mental retardation, behavioural disorders, dysmorphias and refractory epilepsy with polymorphic seizures. It should therefore be treated with broad-spectrum antiepileptic drugs (AEDs), such as valproate (VPA) and topiramate (TPM). The relatively frequent hypersensitivity reactions to aromatic AEDs, not to VPA, the hyperammonemic encephalopathy (HAE) caused by the combination of VPA and TPM and the chromosome disorder described, affecting the same patient, do not appear in the literature we reviewed. CASE REPORT: A patient aged 5 years who, at the age of 11 months, was seen to have psychomotor retardation, microcephaly, plagiocephaly, facial dysmorphia and hypotonia. At 26 months, the patient presented seizures with fever, sucking, perioral cyanosis, clonisms in the upper extremities and hypotonia. Karyotype: C20A, with no mosaicism. Treatment was started with VPA up to 600 mg/day, and moderate eosinophilia appeared from 450 mg/day onwards. At the age of 4 years, the patient suffered partial complex seizures, which stopped with the addition of TPM. At the same age there was also anorexia, loss of weight, adynamia, hypotonia, drowsiness, confusion, increased eosinophilia (20.3%) and IgE and a rash caused by the VPA. The clinical features yielded on adding dexchlorpheniramine. Nine months later, apathy, adynamia, eosinophilia and hyperammonemia reappeared; we therefore reduced and later stopped administration of VPA, although TPM was maintained. CONCLUSIONS: No relation between hypersensitivity to VPA, HAE and C20A has been described. The VPA TPM combination was effective, but in the end we had to stop administering VPA because of hypersensitivity and the side effects (HAE) of the combination.