Síndrome del ocho y medio como manifestación de enfermedad desmielinizante aguda por adenovirus / Eight-and-a-half syndrome as manifestation of acute disseminated adenovirus encephalomyelitis

La encefalomielitis diseminada aguda es una enfermedad inflamatoria-desmielinizante inmunomediada que suele manifestarse tras una infección o vacunación en niños en edad escolar. Típicamente presenta una fase prodrómica con un cuadro pseudogripal seguida de una fase con clínica muy variada, pudiendo aparecer alteraciones neurooftalmológicas como oftalmoplejía o neuritis óptica.La etiología es variada, incluyendo enfermedades tumorales, vasculares, infecciosas, inflamatorias y desmielinizantes. El diagnóstico se basa en la historia clínica y en las características de la resonancia magnética cerebral, prueba de imagen de elección. El estudio del líquido cefalorraquídeo puede servir de ayuda en la orientación del cuadro clínico.El pronóstico es favorable, con excelente respuesta a los corticoides e inmunoglobulinas y con mínimas secuelas a largo plazo en la mayoría de los casos.Presentamos el caso de un varón de 8años con enfermedad desmielinizante aguda por adenovirus cuya manifestación fue un síndrome del ocho y medio. (AU)

Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur.The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture.The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases.We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome. (AU)

Eight-and-a-half syndrome as manifestation of acute disseminated adenovirus encephalomyelitis.

Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur. The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture. The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases. We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome.

Obstetric anal sphicnter injury in a Spanish hospital.

OBJECTIVE: Estimation of the prevalence of obstetric anal sphincter injury (OASIS) in our environment and study of the associated risk factors. STUDY DESING: A retrospective observational study of cases and controls of assisted deliveries at the Severo Ochoa University Hospital of Leganés during the period from January 1, 2012, to December 31, 2017. A total of 88 OASIS diagnosed in the study period is compared with a randomly selected group of 181 controls of similar characteristics, vaginal births of cephalic of 36 weeks gestation or more, occurring during the same period. RESULTS: During the study period, a total of 8160 deliveries were attended in our hospital, of which 6187 were vaginal and we diagnosed a total of 88 OASIS at the time of delivery. The prevalence of OASIS is 1.07 % for total births and 1.42 % for total vaginal deliveries. In the case-control study, the univariate analysis shows statistical significance for nulliparity (OR 3.84; 95 % CI 2.155-6.834; p < 0.001), instrumental delivery (OR 8.73; 95 % CI 4.706-16.2016; p < 0.001), occipital posterior position (OR 7.23; 95 % CI 2.535-20.633; p < 0.001), long duration of the second stage of labor (OR 1.99; IC95 % 1,159-3,438; p 0.01), episiotomy (OR 3.51; 95 % CI 1,956-6,309; p < 0.001) and OBGYN labor assistant (<0.001). When performing the multivariate analysis, forceps delivery (OR19.68), Thierry spatulas delivery (OR 8.15), vacuum delivery (OR 2.74), nulliparity (OR 2.56) and fetal weight in grams (OR 1.12) remain significant in the final model. CONCLUSION: The main risk factors for the onset of OASIS are instrumental delivery, nulliparity and fetal birth weight.

Trichoderma asperellum Induces Maize Seedling Growth by Activating the Plasma Membrane H+-ATPase.

Although Trichoderma spp. have beneficial effects on numerous plants, there is not enough knowledge about the mechanism by which they improves plant growth. In this study, we evaluated the participation of plasma membrane (PM) H+-ATPase, a key enzyme involved in promoting cell growth, in the elongation induced by T. asperellum and compared it with the effect of 10 µM indol acetic acid (IAA) because IAA promotes elongation and PM H+-ATPase activation. Two seed treatments were tested: biopriming and noncontact. In neither were the tissues colonized by T. asperellum; however, the seedlings were longer than the control seedlings, which also accumulated IAA and increased root acidification. An auxin transport inhibitor (2,3,5 triiodobenzoic acid) reduced the plant elongation induced by Trichoderma spp. T. asperellum seed treatment increased the PM H+-ATPase activity in plant roots and shoots. Additionally, the T. asperellum extracellular extract (TE) activated the PM H+-ATPase activity of microsomal fractions of control plants, although it contained 0.3 µM IAA. Furthermore, the mechanism of activation of PM H+-ATPase was different for IAA and TE; in the latter, the activation depends on the phosphorylation state of the enzyme, suggesting that, in addition to IAA, T. asperellum excretes other molecules that stimulate PM H+-ATPase to induce plant growth.

Efectividad a largo plazo de una política continuada de acceso vascular autógeno para hemodiálisis desde una perspectiva centrada en el paciente / Long-term efficacy of a continuous policy of autologous vascular access for haemodyalisis from a patient-centred perspective

OBJETIVO: Las guías recomiendan la realización de accesos vasculares autógenos frente a protésicos o catéteres, si bien su efectividad desde una perspectiva centrada en el paciente es poco conocida. Analizamos la efectividad de una política continuada de acceso vascular autógeno (PCAVA) en pacientes incidentes en hemodiálisis. MATERIAL Y MÉTODOS: Estudio retrospectivo, observacional, con inclusión de 130 pacientes (edad media 62,3 años; 67% hombres; 52,3% con catéter como acceso vascular inicial) incidentes en hemodiálisis entre 2006 y 2009 en nuestro centro y en quienes se aplicó una PCAVA. Análisis estadístico por intención de tratamiento (regresión de Cox). RESULTADOS: En 118 pacientes (90,8%), el primer acceso útil (PAU) fue autógeno y en 12 (9,3%), protésico. En 41 pacientes (31,5%), fue preciso más de un procedimiento para alcanzar un PAU. La probabilidad de dializarse a través de un PAU autógeno fue del 63,1 y 43,2% a 1 y 5 años, respectivamente. Durante el seguimiento (tiempo medio = 28,8 meses), 75 pacientes (57,7%) precisaron reparaciones o nuevos accesos, extendiendo la efectividad de la PCAVA al 86,5 y al 68,8%, a 1 y 5 años. La efectividad de la PCAVA disminuyó si el paciente requirió catéter inicial (HR: 3,2; p = 0,014), si hubo mayor filtrado glomerular inicial (HR: 1,1; p = 0,023), con antecedentes de accesos fallidos previos al PAU (HR: 3.9; p = 0,001) y en mujeres (HR: 2; p = 0,077). CONCLUSIONES: La efectividad a largo plazo de una PCAVA es elevada. Sin embargo, el porcentaje de pacientes que requieren diversos procedimientos para alcanzar un PAU y la necesidad de reintervenciones manifiestan la necesidad de optimizar la evaluación preoperatoria y el seguimiento posterior

OBJECTIVE: The guidelines recommend performing autologous vascular access rather than catheters or prosthetic grafts. An analysis is performed on the long-term effectiveness of a continued policy of priority autologous vascular access (CPAVA) in incident haemodialysis patients. MATERIAL AND METHODS: A retrospective and observational study was conducted on 130 patients (mean age 62.3 years, 67% male), of whom 52.3% had a catheter as initial vascular access, and who started chronic haemodialysis between 2006-2009 in our centre and in whom a CPAVA was applied. Statistical analysis by intention to treat using Cox regression. RESULTS: The first useful access (FUA) was autogenous in 118 patients (90.8%), and prosthetic in 12 (9.3%). More than one procedure was necessary to achieve a FUA in 41 (31.5%) patients. The probability of maintaining the dialysis throughout the FUA was 63.1 and 43.2%, at 1 and 5 years, respectively. During follow-up (mean = 28.8 months), 75 patients (57.7%) required repairs or new accesses, extending the effectiveness of CPAVA to 86.5 and 68.8%, at 1 and 5 years, respectively. The effectiveness decreased if the patient required an initial catheter (HR: 3.2, P = .014), had higher initial glomerular filtration rates (HR: 1.1; P=.023), history of failed access before the FUA (HR: 3.9, P=.001), and in women (HR: 2, P =.077). CONCLUSIONS: The effectiveness of a CPAVA is high. However, a third of patients require more than one procedure to achieve FUA. Several factors adversely affect the outcome of autogenous vascular access, showing the need for optimising the preoperative evaluation and follow-up

In vivo postprandial bioavailability of interesterified-lipids in sodium-caseinate or chitosan based O/W emulsions.

Recent studies have shown that it should be possible to control lipid bioavailability through food structural approaches. Nevertheless, the gastrointestinal-tract physiological conditions must also be considered. To get a better understanding of this phenomenon, we evaluated the effect of emulsification, as well as the use of sodium caseinate or chitosan, on the postprandial bioavailability of interesterified-lipids in O/W emulsions after oral gastric feeding Sprague-Dawley rats. We verified that emulsification may increase lipid absorption, as determined after feeding sodium-caseinate emulsions. However, this result could not be generalised. Interesterified-lipids that were emulsified with chitosan were equally absorbed as those contained in non-emulsified interesterified-lipids/distilled-water blends.

Expresión inmunohistoquímica de p53 en individuos con liquen / Immunohistochemical expression of p53 in individuals with lichen planus

Objetivo: Estimar el riesgo del potencial de malignización del liquen plano bucal analizando la expresión de la proteína p53. Materiales y metodos: Se realizó un diseño de cohorte de sujetos con diagnostico histopatológico de liquen. El desenlace fue el desarrollo de cáncer si/no. El total de individuos que cumplió con los criterios de inclusión/exclusión fue de 58. A los 58 sujetos se les realizó la determinación de p53. Resultados: Cuarenta y nueve individuos mostraron una expresión de p53 menor al 5% con una P>0,05% intrasujeto no transformado. En los 9 individuos transformados se observaron diferencias significativas entre la determinación pre y post de la proteína p53. El Riesgo Relativo fue de 188 con una significación estadística de P< 0,01. Conclusion: La expresión de la proteína p53 en los individuos con liquen apoya la hipótesis que niveles superiores al 5%constituye un factor de incremento del riesgo en la transformación maligna de esta patología. Nuestros hallazgos deberían ser corroborados en el futuro con mayor número de individuos. La determinación de la p53 mediante IHQ en individuos con liquen plano bucal podría modificar el seguimiento clínico de estos individuos. Esto permitiría un diagnóstico precoz de cualquier alteración que pueda indicar un posible cambio hacia la malignización.

Objective: To determine the risk for potential malignant transformation of oral lichen planus by analyzing protein p53 expression. Materials and methods: Cohort study of subjects with a histopathologic diagnosis of lichen. The study analyzed whether or not subjects developed cancer. A total of 58 subjects fulfilled the inclusion/exclusion criteria, and were analyzed for p53. Results: Fortynine(49) subjects showed a p53 expression <5%, with a p>0.05 among subjects undergoing no transformation. In the 9 subjects undergoing malignant transformation, significant differences were observed between pre and post p53 expression. The relative risk was 188, with a statistical significance of p< 0.01. Conclusions: Protein p53 expression in subjects with lichen supports the hypothesis that levels >5% are associated with an increased risk of malignant transformation of this condition. Should our findings be proved in a larger series in the future, the clinical follow-up of these subjects could be modified. This would allow an early diagnosis of any disorder indicative of a potential malignant transformation.

The effect of interesterification on the bioavailability of fatty acids in structured lipids.

Fatty acid (FA) profile is a critical factor in the nutritional properties of fats, but, stereochemistry may also play a fundamental role in the rate and extent to which FAs are absorbed and become available. To better understand this phenomenon, we evaluated the bioavailability of FAs in linseed-oil and palm-stearin blends compared to their interesterified mix, using a sn-1,3 stereospecific lipase, to determine if there was any difference in terms of FA availability when using this technology. Test meals were fed through an intragastric feeding tube on Sprague-Dawley male rats after 18 h fasting. Postprandial blood samples were collected after meal or physiological serum (control) administration and the FA profile of plasma lipids was determined. Results showed that modification of the melting profile through interesterification, without altering the bioavailability determined by sn-2 stereochemistry, could delay lipid absorption at the beginning, but had no effect on total lipid absorption.

Inhibition of Ephrin B3-mediated survival signaling contributes to increased cell death response of non-small cell lung carcinoma cells after combined treatment with ionizing radiation and PKC 412.

Radiation therapy is frequently used to treat non-small cell lung cancers (NSCLCs). We have previously shown that a combination of ionizing radiation (IR) and the staurosporine analog PKC 412, but not Ro 31-8220, increases cell death in NSCLC cells. To identify genes involved in the enhancement of cell death, a total gene profiling in response to co-administration of (i) PKC 412 with IR, or (ii) Ro 31-8220 with IR was implemented. These combined treatments caused upregulation of 140 and 179 genes and downregulation of 253 and 425 genes, respectively. Certain genes were selected and verified by real-time quantitative PCR and, of these genes, robust suppression of Ephrin B3 expression was suggested as a possible cell death-inducing mechanism of combined treatment with IR and PKC 412. Indeed, silencing of Ephrin B3 using siRNA in NSCLC cells resulted in a major alteration of their morphology with an elongated phenotype, decreased proliferation and increased cell death signaling. Moreover, silencing of Ephrin B3 in combination with IR caused a decrease in IR-mediated G(2)-arrest, induced cellular senescence, inhibited MAPK ERK and p38 phosphorylation, and caused an upregulation of p27(kip1) expression. Finally, silencing of Ephrin B3 in combination with IR sensitized U-1810 cells to IR-induced apoptosis. In conclusion, we identify and describe Ephrin B3 as a putative signaling molecule involved in the response of NSCLC cells to combined treatment with PKC 412 and ionizing radiation.

Prevalencia de alteraciones de la conducta alimentaria, trastornos gastrointestinales e infecciones recurrentes en niños afectos de Trastornos del Espectro Autista (TEA) en comparación con sus hermanos sanos / Prevalence of feeding disorders, gastrointestinal disorders and recurrent infections in children with Autism Spectrum Disorders (ASD) compared with their healthy siblings

Introducción: Los Trastornos del Espectro Autista (TEA) están constituidos por un grupo heterogéneo de procesos neurobiológicamente diversos, que se caracterizan por la existencia de déficit en múltiples áreas funcionales. Una de las áreas de interés creciente es la relacionada con la alimentación. La causa de las alteraciones en el desarrollo del área alimentaria del niño autista no está clara y de momento no existe acuerdo para definir el carácter primario o secundario de estas alteraciones. Objetivo: Analizar los hábitos alimentarios, antecedentes de trastornos intestinales, alergias e infecciones recurrentes en una población de niños y adolescentes con TEA. Sujetos y métodos: Estudio de diseño transversal y retrospectivo de casos y controles. La muestra está compuesta por 138 sujetos: 84 niños y adolescentes afectos de TGD según criterios del DSM-IV y 54 controles escogidos entre los hermanos de los anteriores. Se diseñó un cuestionario específico para este estudio que fue cumplimentado por los padres. Resultados: Al comparar los hábitos alimentarios de los niños autistas con los controles, vemos que los niños autistas presentan más dificultades en la incorporación de alimentos sólidos, de absorber con pajita, retraso evolutivo para beber en vaso, incorporación de alimentos nuevos, dificultades en la masticación, mayores rechazos alimentarios y conductas de pica. Las diferencias en infecciones recurrentes o trastornos gastrointestinales no fueron estadísticamente significativas. Conclusión: Los niños y adolescentes afectos de trastorno autista presentan más alteraciones en el desarrollo del área alimentaria que sus hermanos sanos. Las alteraciones encontradas no se corresponden con una mayor frecuencia de trastornos gastrointestinales ni alergias

Introduction: Autism Spectrum Disorders (ASD) are a heterogeneous group of different neurobiological processes, which are characterized by the existence of deficits in multiple functional areas. One area of growing concern is that related to the diet. The cause of the alterations in the development of the feeding area in autistic children is unclear, and there is currently no agreement to define the primary or secondary nature of these alterations. Aim: To analyze feeding habits, history of intestinal disorders, allergies and recurrent infections in a population of children and adolescents with ASD. Subjects and methods: A cross-sectional design and retrospective case-control study was made. The sample comprised 138 subjects: 84 children and adolescents suffering from ASD (DSM-IV criteria) and 54 controls (brothers of the sample subjects). A questionnaire was designed specifically for this study that was completed by parents. Results: When comparing the feeding habits of children with autism and controls, we see that autistic children have more difficulties in: incorporating solid foods, absorb with straw, developmental delay to drink from a cup, incorporating new foods, difficulties chewing food, more rejections and pica behavior. Differences in recurrent infections or gastrointestinal disorders were not statistically significant. Conclusion: Children and adolescents suffering from autistic disorders have more alterations in the development of the feeding area that their siblings. These alterations do not correspond to a higher frequency of gastrointestinal disorders and allergies

Degradation of phenol by mechanical activation of a rutile catalyst.

In the present paper a novel mechanochemical process for the elimination of organic pollutants dissolved in water is proposed. In this regard, phenol aqueous solutions (100mgL(-1)) were ball-milled for 0, 12, 18, 24, 36, 48, and 72h with and without a well-characterized (XRD, SEM, and N(2) Adsorption), rutile powder catalyst and the reaction products analyzed with UV and GC/MS. It was found that when the catalyst was not included in the process, phenol was not affected, but when it was included, phenol was decomposed. The catalyst itself did not change and the reaction follows a pseudo-first-order kinetics. Besides, intermediates which are characteristic of the ()OH radical mechanism were found in the reaction products. Then, a mechanism similar to those accepted for other advanced oxidation processes was proposed. The value measured for the pseudo-first-order reaction constant was very low, indicating that the reported process is inefficient. Nevertheless, this problem could be solved by applying catalysts consisting of particles with smaller diameters.

Hole distribution in phononic crystals: design and optimization.

An exhaustive study has been made into the potential improvement in attenuation and focusing of phononic crystal arrays resulting from the deliberate creation of vacancies. Use is made of a stochastic search algorithm based on evolutionary algorithms called the epsilon variable multi-objective genetic algorithm which, in conjunction with the application of multiple scattering theory, enables the design of devices for effectively controlling sound waves. Several parameters are analyzed, including the symmetries used in the distribution of holes and the optimum number of holes. The validity and utility of the general rules obtained have been confirmed experimentally.

Intracerebral Hodgkin's lymphoma in a patient with human immunodeficiency virus.

Central nervous system (CNS) involvement by Hodgkin Lymphoma (HL) is rarely reported. Retrospective and prospective cohort studies suggest an incidence of 0.2-0.5%, mostly in relapsed disease. In spite of a 3 to 18-fold increased risk of HL in patients with human immunodeficiency virus (HIV), only two cases have been reported so far. In this paper, we now report a third case of HIV patient with HL who progressed with isolated CNS infiltration after a standard chemotherapy induced clinical remission. In 1991, when the first case of intracerebral involvement in HIV+ HL was reported an increase of this type of cases would have been expected, but only one more case has been reported since then.

Primary malignant schwannoma of the cervical plexus with melanocytic differentiation.

Primary malignant schwannomas are rare neoplasms of nerve sheath origin, especially in the location of the head and neck where few cases are described in the literature. These tumours may pose a diagnostic dilemma in the work-up of a neck mass. The case presented here is of a malignant schwannoma that originated in the cervical plexus with the rare histological feature of melanocytic differentiation. Histopathological examination with immunostaining techniques is essential for the diagnosis of these tumours. The management of these neoplasms is still controversial, although the treatment of choice is radical surgical excision of the lesion. The role of postoperative radiotherapy or chemotherapy is not clear, although some authors recommend its use to prevent local recurrence, for unresectable recurrent tumours or in cases of distant metastases.

Rapidly-progressive liver failure secondary to melanoma infiltration.

We describe the case of a 51-year-old man with a history of intraocular melanoma treated with radiotherapy 2 years previously. The patient was diagnosed with mild hypertransaminasemia that progressed to acute liver failure and death in a period of one month. Radiological investigations such as spiral computed tomography and abdominal ultrasonography failed to give an etiologic diagnosis. Autopsy revealed melanoma with diffuse infiltration of the hepatic parenchyma. Because diagnosis is usually delayed, the prognosis of intraocular melanoma is poor. In 40% of cases metastases are present at diagnosis, and the most frequently affected organ is the liver (93-95%). Presentation as acute liver failure can appear after a long disease-free period. For this reason, periodic laboratory tests and hepatic ultrasound examination are recommended in patients diagnosed with this malignancy.

Insuficiencia hepática rápidamente progresiva secundaria a infiltración por melanoma / Rapidly-progressive liver failure secondary to melanoma infiltration

Se describe el caso clínico de un paciente varón de 51 años con antecedentes personales de melanoma coroideo trata do con radioterapia 2 años antes. Se le diagnosticó hipertransaminasemia leve, que progresó a fallo hepático fulminante, con el fallecimiento del paciente en el período de un mes. Las pruebas de imagen (tomografía axial computarizada y ecografía abdominal) no fueron diagnósticas para el estudio etiológico. La autopsia reveló la existencia de melanoma que infiltraba difusamente el parénquima hepático. El melanoma coroideo presenta un mal pronóstico debido a que la mayoría de los casos se diagnostican de forma tardía. En el 40% de los casos se objetivan metástasis en el momento del diagnóstico, y el hígado es el órgano más frecuentemente afectado (93-95%). La presentación como insuficiencia hepática puede aparecer después de un largo período libre de enfermedad. Por este motivo se recomienda realizar periódicamente análisis y ecografías hepáticos en pacientes diagnosticados de melanoma ocular

We describe the case of a 51-year-old man with a history of intraocular melanoma treated with radiotherapy 2 years previously. The patient was diagnosed with mild hypertransaminasemia that progressed to acute liver failure and death in a period of one month. Radiological investigations such as spiral computed tomography and abdominal ultrasonography failed to give an etiologic diagnosis. Autopsy revealed melanoma with diffuse infiltration of the hepatic parenchyma. Because diagnosis is usually delayed, the prognosis of intraocular melanoma is poor. In 40% of cases metastases are present at diagnosis, and the most frequently affected organ is the liver (93-95%). Presentation as acute liver failure can appear after a long disease-free period. For this reason, periodic laboratory tests and hepatic ultrasound examination are recommended in patients diagnosed with this malignancy

[Functional disorders of FOF1-ATPase in submitochondrial particles obtained from platelets of patients with a diagnosis of probable Alzheimer's disease]. / Alteración funcional de la F0F1-ATPasa en partículas submitocondriales obtenidas de plaquetas de pacientes con diagnóstico de enfermedad de Alzheimer probable.

INTRODUCTION: Recent studies indicate that decreased energy generation by mitochondria is a feature that is common across neurodegenerative diseases. PATIENTS AND METHODS: In order to obtain direct evidence that mitochondrial functioning is altered, we measured the hydrolytic activity of F0F1-ATPase and its capacity to generate a stable proton gradient in submitochondrial particles in 29 patients diagnosed with probable Alzheimer's disease (AD). Submitochondrial particles were obtained from platelets of patients with a diagnosis of probable AD and from clinically healthy controls. RESULTS: Data revealed that the hydrolytic activity of F0F1-ATPase increases significantly in patients with probable AD (41.7+/-4.3 nmol PO4 min-1[mg protein]-1, n=29) as compared to the control subjects (29.1+/-1.9 nmol PO4 min-1 [mg protein]-1, n=29). It is important to note that, in the male population with probable AD, we found that hydrolytic activity of F0F1-ATPase increased as cerebral deterioration progressed. We also detected a lower pH gradient in the submitochondrial particles of patients with probable AD (0.28+/-0.08 pH units, n=25) as compared to the controls (0.5+/-0.1 pH units, n=20). CONCLUSIONS: Overall, these data point to an alteration in the functioning of the enzyme.

Alteración funcional de la F0F1-ATPasa en partículas submitocondriales obtenidas de plaquetas de pacientes con diagnóstico de enfermedad de Alzheimer probable / Functional disorders of fof1-atpase in submitochondrial particles obtained from platelets of patients with a diagnosis of probable alzheimer’s disease

Introducción. Los estudios recientes indican que una disminución en la generación de energía de la mitocondria es una característica que unifica a las enfermedades neurodegenerativas. Pacientes y métodos. Para obtener evidencia directa que la función mitocondrial se altera, cuantificamos en 29 pacientes diagnosticados con probable enfermedad de Alzheimer (EA) la actividad hidrolítica de la F0F1-ATPasa y su capacidad para generar un gradiente estable de protones en partículas submitocondriales. Las partículas submitocondriales se obtuvieron de plaquetas de pacientes con diagnóstico probable de EA y de controles clínicamente sanos. Resultados. Los datos revelaron que la actividad hidrolítica de la F0F1-ATPasa se incrementa de manera significativa en los pacientes con la probable EA -41,7 ± 4,3 nmol PO4 min-1(mg proteína)-1, con n = 29- en comparación con los controles -29,1 ± 1,9 nmol PO4 min-1 (mg proteína)- 1, con n = 29-. De manera importante, encontramos que en la población masculina con probable EA, la actividad hidrolítica de la F0F1-ATPasa aumenta conforme progresa el deterioro cerebral. También detectamos un gradiente de pH menor en las partículas submitocondriales de los pacientes con probable EA (0,28 ± 0,08 unidades de pH, n = 25) comparados con los controles (0,5 ± 0,1 unidades de pH, n = 20). Conclusión. Estos datos, en conjunto, indican una alteración funcional en la enzima

Introduction. Recent studies indicate that decreased energy generation by mitochondria is a feature that is common across neurodegenerative diseases. Patients and methods. In order to obtain direct evidence that mitochondrial functioning is altered, we measured the hydrolytic activity of F0F1-ATPase and its capacity to generate a stable proton gradient in submitochondrial particles in 29 patients diagnosed with probable Alzheimer’s disease (AD). Submitochondrial particles were obtained from platelets of patients with a diagnosis of probable AD and from clinically healthy controls. Results. Data revealed that the hydrolytic activity of F0F1-ATPase increases significantly in patients with probable AD (41.7 ± 4.3 nmol PO4 min-1[mg protein]-1, n = 29) as compared to the control subjects (29.1 ± 1.9 nmol PO4 min-1 [mg protein]-1, n = 29). It is important to note that, in the male population with probable AD, we found that hydrolytic activity of F0F1-ATPase increased as cerebral deterioration progressed. We also detected a lower pH gradient in the submitochondrial particles of patients with probable AD (0.28 ± 0.08 pH units, n = 25) as compared to the controls (0.5 ± 0.1 pH units, n = 20). Conclusions. Overall, these data point to an alteration in the functioning of the enzyme