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1.
Eur J Paediatr Dent ; 22(4): 291-297, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35034462

RESUMEN

AIM: To determine if there are differences between the dental arch dimensions and occlusal characteristics of children born prematurely and admitted into a Neonatal Intensive Care Unit (NICU) (study group) and children born at term (control group). The study group was analysed at the same time in order to find out if the parameters of the dental arches are related to specific perinatal variables. MATERIALS: Study design: Out of a total of 504 children born between 2011 and 2014 in high-risk conditions at the clinic of the University Hospital "San Cecilio" in Granada (Spain), 78 children were selected. Of these 42 had been born prematurely and admitted to the NICU (study group) and 36 had been born at term (control group). The age of the sample was 5 to 8 years. The parameters of the dental arches, together with the occlusal characteristics, were examined using dental casts. Both the upper and lower arches were measured for a total of 156 dental casts with a FINO digital caliper. CONCLUSION: The data confirm the smaller size of the dental arch dimensions of children born prematurely and admitted to a NICU compared with children born at term. This fact should be taken into consideration with regard to future orthodontic and paediatric dentistry treatments.


Asunto(s)
Arco Dental , Oclusión Dental , Estudios de Casos y Controles , Niño , Preescolar , Humanos , Recién Nacido , España
2.
Sci Adv ; 4(12): eaau9366, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30555920

RESUMEN

Carbon deposits are well-known inhibitors of transition metal catalysts. In contrast to this undesirable behavior, here we show that epitaxial graphene grown on Ru(0001) promotes the reversible formation of a C-C bond between -CH2CN and 7,7,8,8-tetracyano-p-quinodimethane (TCNQ). The catalytic role of graphene is multifaceted: First, it allows for an efficient charge transfer between the surface and the reactants, thus favoring changes in carbon hybridization; second, it holds the reactants in place and makes them reactive. The reaction is fully reversible by injecting electrons with an STM tip on the empty molecular orbitals of the product. The making and breaking of the C-C bond is accompanied by the switching off and on of a Kondo resonance, so that the system can be viewed as a reversible magnetic switch controlled by a chemical reaction.

3.
Transplant Proc ; 50(2): 650-652, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29579878

RESUMEN

BACKGROUND: Patients with dilated cardiomyopathy (DCM) and left ventricular dysfunction have a varied clinical course, not only dependent on left ventricular ejection fraction (LVEF) and symptoms. Finding prognostic markers for stratification in these 2 conditions is a critical area of research. Our aim was determine the prognostic value of blood panel basic parameters. METHODS: We analyzed all patients with idiopathic or familial DCM and LVEF <30% coming to our heart failure unit for evaluation for non-urgent heart transplant during the period of 2009 to 2011. With 5 years of follow-up data, we could study the prognostic value of blood panel parameters. Moreover, we determined the combination of platelet count and neutrophil to lymphocyte ratio score from the BIOSTAT-CHF study. RESULTS: Eighty-seven patients were included in the study. After 5 years follow-up, 49 patients (57%) remain alive (group A) and 38 (43%) either died or needed a heart transplant. There were no differences between groups with regard to age or sex. Patients with good progress showed a lower red cell distribution width (RDW), a higher lymphocyte count, and a lower neutrophil/lymphocyte ratio in the initial blood panel. An RDW ≥15% was associated with long-term mortality or heart transplant. CONCLUSION: A basic blood panel could be a useful tool in assessing patients with heart failure. Larger studies are necessary to confirm our findings. A multimarker strategy could also be useful for stratification of patients with advanced heart failure.


Asunto(s)
Biomarcadores/sangre , Cardiomiopatía Dilatada/sangre , Insuficiencia Cardíaca/sangre , Eritrocitos/patología , Femenino , Trasplante de Corazón , Humanos , Recuento de Linfocitos , Masculino , Persona de Mediana Edad , Neutrófilos , Recuento de Plaquetas , Pronóstico
4.
Transplant Proc ; 50(2): 653-654, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29579879

RESUMEN

The CentriMag (Levitronix LLC, Waltham, MA, USA) ventricular assist device is a centrifugal pump designed for short-term support in patients with cardiogenic shock. In patients with restrictive physiology, there is some concern about the use of these devices due a very small ventricular cavity. We review the evolution of a 32-year-old woman with restrictive cardiomyopathy, moderate pulmonary hypertension, and severe biventricular systo-diastolic dysfunction in whom a biventricular Centrimag device was implanted due to a progressive clinical worsening.


Asunto(s)
Cardiomiopatía Restrictiva/terapia , Corazón Auxiliar , Adulto , Femenino , Humanos
5.
Chem Commun (Camb) ; 53(45): 6164-6167, 2017 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-28534561

RESUMEN

Liquid-phase exfoliation is a technique capable of producing large quantities of two-dimensional materials in suspension. Despite many efforts in the optimization of the exfoliation process itself, not much has been done towards the integration of liquid-phase-exfoliated materials in working solid-state devices. In this article, we use dielectrophoresis to direct the assembly of liquid-phase-exfoliated TiS3 nanoribbons between two gold electrodes to produce photodetectors working in the visible region. Through electrical and optical measurements we characterize the responsivity of the device and we find values as large as 3.8 mA W-1, which are more than one order of magnitude higher compared to state-of-the-art devices based on liquid-phase-exfoliated two-dimensional materials assembled by drop-casting or ink-jet methods.

6.
Phys Chem Chem Phys ; 17(43): 29335-9, 2015 Nov 21.
Artículo en Inglés | MEDLINE | ID: mdl-26473177

RESUMEN

Langmuir-Blodgett films of polyvinylidene fluoride trifluoroethylene - P(VDF-TrFE)-copolymers possess substantially improved electrocaloric and pyroelectric properties, when compared with conventionally spin-cast films. In order to rationalize this, we prepared single-layered films of P(VDF-TrFE) (70 : 30) using both deposition techniques. Grazing incidence wide-angle X-ray scattering (GIWAXS), reveals that Langmuir-Blodgett deposited films have a higher concentration of the ferroelectric ß-phase crystals, and that these films are highly oriented with respect to the substrate. Based on these observations, we suggest alternative means of deposition, which may substantially enhance the electrocaloric effect in P(VDF-TrFE) films. This development has significant implications for the potential use of P(VDF-TrFE) in solid-state refrigeration.


Asunto(s)
Hidrocarburos Fluorados/química , Polivinilos/química , Difracción de Rayos X
7.
Infant Behav Dev ; 38: 135-46, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25645599

RESUMEN

The aim of this study was to assess the effect of massage therapy on the growth and development of infants of HIV-infected mothers in a low socio-economic community in Cape Town. It was a prospective, randomised, controlled intervention trial that included massage therapy and control groups of HIV-infected mothers and their normal birth weight infants who were enrolled in the prevention of mother-to-child transmission (PMTCT) programme. Participants were recruited at the 6-week clinic visit and followed up every 2 weeks until their infants were 9 months of age. Mother-infant pairs in the massage therapy and control groups included 73 and 88 at 6 weeks and 55 and 58 at 9 months, respectively. Mothers in the intervention group were trained to massage their infants for 15 min daily. The socioeconomic status, immunity, relationship with the partner and mental pain of mothers; the infants' dietary intake, anthropometry and development (Griffiths Mental Development Scales); and haematological and iron status of mothers and infants were assessed at baseline and follow-up. Nine infants (5.3%) were HIV-infected on the HIV DNA PCR test at 6 weeks. Despite significantly higher levels of maternal mental pain, infants in the massage therapy compared to control group scored higher in all five of the Griffiths Scales of Mental Development and significantly higher in the mean quotient (p=0.002) and mean percentile (p=0.004) for the hearing and speech scale at 9 months. Based on the mean difference in scores, the massage therapy group showed greater improvement for all five scales compared to the control group. The mean difference in scores was significantly greater for the hearing and speech quotient (21.9 vs. 11.2) (p<0.03) and the general quotient percentile (19.3 vs. 7.7) (p=0.03) in the massage therapy compared to the control group. These scales remained significant when adjusting for the relationship with the partner and maternal mental pain. Both groups had lower scores in the performance scale at 9 months although this was significantly worse in the control compared to the massage therapy group when adjusting for maternal CD4 count, anaemia, relationship with the partner and mental pain. There were no significant differences in the anthropometric measurements between the two groups. In conclusion, based on the Griffiths Scales, massage therapy improved the overall development and had a significant effect on the hearing and speech and general quotient of HIV-exposed infants in this study.


Asunto(s)
Países en Desarrollo , Discapacidades del Desarrollo/psicología , Discapacidades del Desarrollo/terapia , Seropositividad para VIH/psicología , Masaje/psicología , Áreas de Pobreza , Población Urbana , Adulto , Trastornos del Conocimiento/diagnóstico , Trastornos del Conocimiento/psicología , Trastornos del Conocimiento/terapia , Discapacidades del Desarrollo/diagnóstico , Discapacidades del Desarrollo/etnología , Femenino , Humanos , Lactante , Trastornos del Desarrollo del Lenguaje/diagnóstico , Trastornos del Desarrollo del Lenguaje/psicología , Trastornos del Desarrollo del Lenguaje/terapia , Masculino , Estudios Prospectivos , Sudáfrica , Adulto Joven
8.
J Comp Pathol ; 151(4): 380-3, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25443431

RESUMEN

Ganglioneuromatosis (GNM) is a rare condition characterized by the benign proliferation of ganglion cells, nerve fibres and supporting cells of the enteric nervous system. Necropsy examination of a female piglet weighing 4 kg revealed a well-demarcated 20 cm segment of terminal ileum with thickening of the wall. Microscopically, the lamina propria was infiltrated by enteric glial cells and large ganglion cells. Within the submucosal and muscular layers, aggregates of neurons were interlaced by Schwann cells and enteric glial cells arranged in concentric rings. Immunohistochemically, the neurons were weakly labelled for S-100 and neuron-specific enolase, Schwann cells expressed S-100 and vimentin and enteric glial cells expressed glial fibrillary acidic protein and S-100. Pathological and immunohistochemical findings supported the diagnosis of ileal GNM.


Asunto(s)
Ganglioneuroma/veterinaria , Neoplasias del Íleon/veterinaria , Enfermedades de los Porcinos/patología , Animales , Femenino , Ganglioneuroma/patología , Neoplasias del Íleon/patología , Inmunohistoquímica , Sus scrofa , Porcinos
9.
Transplant Proc ; 45(10): 3672-4, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24314993

RESUMEN

Noncompaction cardiomyopathy involves an infrequent pathology whose diagnosis has been rising in recent years owing to a better understanding of the disease and, therefore, better diagnosis. Today, there are no well established protocols for its treatment. For this reason, it is necessary to adapt the therapy of choice to each patient. We report the case of a 35-year-old man with no past medical history of interest who was admitted due to acute pulmonary edema. Diagnostic tests revealed noncompaction cardiomyopathy with biventricular severe systolic dysfunction. In this situation, heart transplantation was considered to be the best therapeutic option, which was carried out with an uneventful postoperative course. This is one of the few cases reported in the literature for this issue.


Asunto(s)
Cardiomiopatías/complicaciones , Cardiomiopatías/diagnóstico , Cardiopatías Congénitas/complicaciones , Insuficiencia Cardíaca/etiología , Adulto , Cardiomiopatías/cirugía , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/cirugía , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/cirugía , Trasplante de Corazón , Humanos , Imagen por Resonancia Magnética , Masculino , Edema Pulmonar/diagnóstico , Edema Pulmonar/etiología , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Disfunción Ventricular Izquierda/diagnóstico , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Derecha/diagnóstico , Disfunción Ventricular Derecha/etiología
10.
Transplant Proc ; 45(10): 3659-61, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24314988

RESUMEN

BACKGROUND: Despite an increase in patients with end-stage heart failure, the acceptance rate has been going down in recent years owing to a change in donor demographics. Furthermore, the rate of emergency heart transplantation has progressively increased. The result is an increase in the time awaiting heart transplantation in elective patients and therefore in the risk of sudden death in this population. Implantable cardioverter defibrillators (ICDs) could be a preventive option in these cases. However, indications for the implantation in this population are not well established. OBJECTIVE: We sought to evaluate the effectiveness of ICDs for primary prevention in patients with left ventricular ejection fraction (LVEF) ≤ 30% included on the heart transplantation list. METHODS: Records from patients accepted for heart transplantation in our institution from January 1, 2006, to July 30, 2012, and whose LVEF was <31% were reviewed. Patients who received ICDs for primary prevention (n = 28) were compared with patients without ICDs (n = 51). Descriptive and univariate (χ(2) and t tests) statistics and Kaplan-Meier survival curves were used for analyses. RESULTS: With a median follow-up of 77 days (range 1-1,231), the overall mortality in the ICD group was 7.1% (2/28) and in the non-ICD group was 17.6% (9/51; P = .062). The main cause of death in patients without ICDs was sudden death (5/9, 55.6%), followed by heart failure (4/9, 44.4%). In patients with ICDs, heart failure was the only reported cause of death. Appropriate ICD therapies were recorded in 42.9% (12/28) in this population. CONCLUSIONS: This study suggests that ICD could reduce the risk of sudden death in patients with LVEF ≤ 30% while awaiting heart transplantation. However, more studies are needed to confirm these results.


Asunto(s)
Muerte Súbita Cardíaca/prevención & control , Desfibriladores Implantables , Cardioversión Eléctrica/instrumentación , Insuficiencia Cardíaca/terapia , Trasplante de Corazón , Prevención Primaria/instrumentación , Disfunción Ventricular Izquierda/terapia , Función Ventricular Izquierda , Listas de Espera , Adulto , Anciano , Causas de Muerte , Distribución de Chi-Cuadrado , Muerte Súbita Cardíaca/etiología , Cardioversión Eléctrica/efectos adversos , Cardioversión Eléctrica/mortalidad , Femenino , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Volumen Sistólico , Factores de Tiempo , Resultado del Tratamiento , Disfunción Ventricular Izquierda/diagnóstico , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/mortalidad , Disfunción Ventricular Izquierda/fisiopatología , Listas de Espera/mortalidad
11.
Med Oral Patol Oral Cir Bucal ; 17(2): e223-7, 2012 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-22143682

RESUMEN

OBJECTIVE: To determine the oral treatment needs of a sample of patients diagnosed with multiple sclerosis in the Community of Madrid (Spain). PATIENTS AND METHODS: A cross-sectional epidemiological study was carried out with a sample of 64 patients who were aged 25 to 77 years. They were distributed into homogeneous age groups: < 46 years, 46-54 years and > 54 years. In order to evaluate the oral health status and treatment requirements, the parameters and guidelines of the WHO were used. RESULTS: The prevalence of caries was 100%, or very close in all three groups. As age increased, the morbidity rate decreased, but the mortality rate increased considerably. On analyzing gingival health, 65% of patients had calculus, 5% bleeding and 30% were healthy. CONCLUSIONS: The DMFT index found provided data that was, in general, very similar to that of the general population in Spain. However, the gingival health status found demonstrated that the population of multiple sclerosis patients requires specific assistance.


Asunto(s)
Estado de Salud , Enfermedades de la Boca/complicaciones , Enfermedades de la Boca/epidemiología , Esclerosis Múltiple/complicaciones , Salud Bucal , Enfermedades Dentales/complicaciones , Enfermedades Dentales/epidemiología , Adulto , Anciano , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Evaluación de Necesidades
13.
Vet Parasitol ; 170(3-4): 268-77, 2010 Jun 24.
Artículo en Inglés | MEDLINE | ID: mdl-20307938

RESUMEN

A cross sectional survey was performed to identify gastrointestinal helminths and protozoans in naturally infected horses from the biosphere reserve known as "La Sierra Madre de Chiapas", Mexico (El Triunfo and La Sepultura). During a three-year survey, fecal samples from 90 horses and parasites from 2 necropsied animals were collected. Five families from the Nematoda class: Ascaridae, Kathlanidae, Oxyuridae, Strongylidae and Trichostrongylidae were found, whereas, only one family from the class Cestoda, was observed: Anoplocephalidae. One family from the class Insecta, was observed: Gasterophiilidae. The number of species of parasites ranged from 13 to 18 with an average of 15 per animal. Adult parasites were recovered from the large intestine luminal contents at necropsy. Species recovered included: Strongylus vulgaris, S. equinus, S. edentatus, Oxyuris equi, Parascaris equorum, Coronocyclus coronatum, C. labiatus, C. labratus, Cyathostomum tetracanthum, Cylicocyclus insigne, C. leptostomus, Cylicodontophorus bicoronatus, Cylicostephanus asymetricus, C. bidentatus, C. minutus, C. longibursatus, Petrovinema poculatum, Poteriostomum imparidentatum, Cylicostephanus goldi, Tridentoinfundibulum gobi, Triodontophorus serratus and T. tenuicollis. One species of Diptera were recovered from stomach and identified: Gasterophilus intestinalis. Furthermore, different species of protozoa were recovered from fresh horse-dung and identified in four classes: Sporozoa, Litostomatea, Ciliasida and Suctoria. Nine families: Cryptosporidiidae, Eimeriidae, Balantidiidae, Buetschliidae, Blepharocorythidae, Cycloposthiidae, Spirodiniididae, Ditoxidae, Acinetidae; and 31 ciliates species were recorded: Allantosoma dicorniger, A. intestinalis, Alloiozona trizona, Blepharosphaera intestinalis, Blepharoprosthium pireum, Blepharoconus benbrooki, Bundleia postciliata, Didesmis ovalis, D. quadrata, Sulcoarcus pellucidulus, Blepharocorys angusta, B. cardionucleata, B. curvigula, B. juvata, B. uncinata, B. valvata, Cycloposthium bipalmatum, C. edentatum, C. scutigerum, Charonina equi, Ditoxum funinucleum, Spirodinium equi, Tetratoxum unifasciculatum, Triadinium caudatum, T. galea, T. minimum and Tripalmaria dogieli. Other ciliate observed and recorded was Ochoterenaia appendiculata. This study describes the biodiversity and distribution of common and new helminths and protozoas found in the gastrointestinal tract from horses in the biosphere reserve "Sierra Madre de Chiapas" in Mexico.


Asunto(s)
Apicomplexa/clasificación , Helmintiasis Animal/parasitología , Helmintos/clasificación , Enfermedades de los Caballos/parasitología , Infecciones Protozoarias en Animales/parasitología , Animales , Biodiversidad , Conservación de los Recursos Naturales , Ecosistema , Helmintiasis Animal/epidemiología , Enfermedades de los Caballos/epidemiología , Caballos , Masculino , México/epidemiología , Infecciones Protozoarias en Animales/epidemiología , Estaciones del Año
14.
Rev Neurol ; 48 Suppl 1: S61-9, 2009 Jan 23.
Artículo en Español | MEDLINE | ID: mdl-19222018

RESUMEN

INTRODUCTION: Dystonia is the second most common movement disorder after Parkinsonism. No exact figures are available on the incidence/prevalence of the different forms of dystonia, because the data vary considerably depending on the source, method and ethnic origin of the population under study. AIMS: To describe and summarise our current knowledge of the epidemiology, causation, diagnosis and treatment of dystonias. DEVELOPMENT: Dystonia is a movement disorder characterised by sustained muscular contractions that cause repeated twisting movements and abnormal postures. Dystonias can be classified according to their distribution, aetiology, clinical course and age at onset. A correct classification is very useful for evaluating the complementary tests that are needed, as well as the prognosis and treatment of the process. The diagnosis of dystonia is essentially clinical and is confirmed with electromyography. Both the different laboratory and neuroimaging studies are fundamentally used to help in the aetiological classification and to rule out secondary causes of dystonia. Among the different treatments that exist today (intrathecal, infiltrative, surgical, systemic pharmacotherapy), special attention should be given to the role of botulinum toxin as the preferred treatment in most cases of focal dystonias. CONCLUSIONS: Exhaustive epidemiological studies are needed to be able to get a more precise picture of the epidemiology of dystonias. Despite the great amount of progress made in recent years as far as genetics and neuroimaging are concerned, the diagnosis of dystonia remains essentially clinical. Botulinum toxin is the preferred treatment in most cases of focal dystonias.


Asunto(s)
Distonía/epidemiología , Distonía/etiología , Distonía/terapia , Edad de Inicio , Antidiscinéticos/uso terapéutico , Toxinas Botulínicas/uso terapéutico , Diagnóstico Diferencial , Distonía/clasificación , Humanos , Trastornos del Movimiento/fisiopatología
15.
Rev. neurol. (Ed. impr.) ; 48(supl.1): 61-69, 23 feb., 2009. tab
Artículo en Español | IBECS | ID: ibc-94964

RESUMEN

Resumen. Introducción. La distonía es el segundo trastorno del movimiento más frecuente después de los parkinsonismos. No se conocen con exactitud las cifras de incidencia/prevalencia de las distintas formas de distonía, ya que las cifras varían considerablemente en función de la fuente, método de estudio y origen étnico de la población estudiada. Objetivo. Describir y resumir los conocimientos actuales sobre la epidemiología, etiología, diagnóstico y tratamiento de las distonías. Desarrollo. La distonía es un trastorno del movimiento caracterizado por contracciones musculares sostenidas que causan movimientos de torsión repetidos y posturas anómalas. Las distonías pueden clasificarse según su distribución, etiología, curso clínico y edad de inicio. Una correcta clasificación resulta muy útil de cara a evaluar las pruebas complementarías necesarias, el pronóstico y el tratamiento del proceso. El diagnóstico de distonía es fundamentalmente clínico y se confirma con electromiografía. Tanto los diversos estudios de laboratorio como de neuroimagen sirven fundamentalmente para ayudar en la clasificación etiológica y para descartar causas secundarias de distonía. Dentro de los diversos tratamientos existentes hoy en día (farmacoterapia sistémica, intratecal, infiltrativa, quirúrgica), cabe destacar el papel de la toxina botulínica como tratamiento de elección en la mayor parte de las distonías focales. Conclusiones. Es necesaria la realización de estudios epidemiológicos exhaustivos que permitan conocer con mayor exactitud la epidemiología de las distonías. A pesar del gran avance en los últimos años en cuanto a genética y neuroimagen, el diagnóstico de la distonía sigue siendo fundamentalmente clínico. La toxina botulínica es el tratamiento de elección en la mayor parte de las distonías focales (AU)


Summary. Introduction. Dystonia is the second most common movement disorder after Parkinsonism. No exact figures are available on the incidence/prevalence of the different forms of dystonia, because the data vary considerably depending on the source, method and ethnic origin of the population under study. Aims. To describe and summarise our current knowledge of the epidemiology, causation, diagnosis and treatment of dystonias. Development. Dystonia is a movement disorder characterised by sustained muscular contractions that cause repeated twisting movements and abnormal postures. Dystonias can beclassified according to their distribution, aetiology, clinical course and age at onset. A correct classification is very useful for evaluating the complementary tests that are needed, as well as the prognosis and treatment of the process. The diagnosis of dystonia is essentially clinical and is confirmed with electromyography. Both the different laboratory and neuroimaging studies are fundamentally used to help in the aetiological classification and to rule out secondary causes of dystonia. Among he different treatments that exist today (intrathecal, infiltrative, surgical, systemic pharmacotherapy), special attention should be given to the role of botulinum toxin as the preferred treatment in most cases of focal dystonias. Conclusions. Exhaustive epidemiological studies are needed to be able to get a more precise picture of the epidemiology of dystonias. Despite the great amount of progress made in recent years as far as genetics and neuroimaging are concerned, the diagnosis of dystonia remains essentially clinical. Botulinum toxin is the preferred treatment in most cases of focal dystonias (AU)


Asunto(s)
Humanos , Distonía/clasificación , Toxinas Botulínicas/uso terapéutico , Distonía/tratamiento farmacológico , Distonía/epidemiología , Diagnóstico Diferencial , Tics/epidemiología , Discinesia Inducida por Medicamentos/diagnóstico
16.
Rev Neurol ; 39(4): 343-6, 2004.
Artículo en Español | MEDLINE | ID: mdl-15340893

RESUMEN

INTRODUCTION: L-2-hydroxyglutaric aciduria is a rare inborn error of metabolism, autosomal recessive, identified in about 50 patients. The primary defect is still unknown. The clinical phenotype is variable. Affected individuals show slowly progressive neurodegenerative disorder with cerebellar ataxia and mental retardation. Pyramidal, and extrapyramidal signs, seizures and macrocephaly have been reported. All patients previously described show a pattern of subcortical leukoencephalopathy with nearly empty gyral cores and cerebellar atrophy in neuroimaging studies. The diagnosis is established by detection of increased levels of L-2-hydroxyglutaric acid in urine, plasma and cerebrospinal fluid. CASE REPORTS: We here describe two patients 7 and 9 years old, who presented psychomotor retardation, seizures, progressive cognitive deterioration, and pyramidal, extrapyramidal and cerebellar signs. Magnetic resonance scanning of the brain demonstrated a bilateral subcortical leukoencephalopathy pattern and areas of increased T2-weighted signal in the basal ganglia and cerebellar dentate nuclei. The analysis of organic acids in urine by gas chromatography/mass spectrometry showed elevated 2-hydroxyglutaric acid, 100% of it in the form of L enantiomer. CONCLUSION: The diagnostic consideration is based on clinical findings and typical neuroimaging pattern and is established by detection of L-2-hydroxyglutaric acid in body fluids. Subcortical white matter loss is an important clue to diagnosis.


Asunto(s)
Encefalopatías/diagnóstico , Encefalopatías/orina , Demencia Vascular/diagnóstico , Demencia Vascular/orina , Glutaratos/orina , Errores Innatos del Metabolismo/diagnóstico , Errores Innatos del Metabolismo/orina , Niño , Humanos , Imagen por Resonancia Magnética , Masculino , Venezuela
17.
Rev Neurol ; 39(3): 213-7, 2004.
Artículo en Español | MEDLINE | ID: mdl-15284959

RESUMEN

INTRODUCTION: Interferon (IFN) diminishes the outbreaks of multiple sclerosis (MS) and slows down its progression. Follow-up of patients is performed using clinical and resonance imaging parameters, and no biological markers are available that allow us to determine its efficiency. AIMS: 1. To discover the effects of IFN on the serum levels of TNF-alpha, IL-4, IL-10, VCAM-1, neopterin and CD-30 in patients with MS; 2. To determine how these modifications evolve over time; 3. To find out the clinical value of its determination in isolation. PATIENTS AND METHODS: We studied 19 patients with MS who were clinically stable and undergoing IFN therapy. Samples were obtained every 3 months over a 2.5 year period and always immediately before injecting the drug. The ELISA method was used to determine interleukins. RESULTS: Serum levels of neopterin, CD-30 and VCAM-1 were not modified, TNF-alpha levels oscillated regardless of the clinical status of the patient and IL-4 and IL-10 had a significant serum peak at 9-12 months after beginning treatment. CONCLUSIONS: The existence of a significant IL-4 and IL-10 peak between 6 and 12 months of therapy indicates that IFN reaches its possible immunomodulatory effect after several months and, therefore, a poor initial clinical response must not be a reason for discontinuing medication. The specific determination of the serum levels of IL is not useful in following up patients treated with IFN.


Asunto(s)
Interferones/uso terapéutico , Interleucina-10/sangre , Interleucina-4/sangre , Antígeno Ki-1/sangre , Esclerosis Múltiple Recurrente-Remitente/sangre , Esclerosis Múltiple Recurrente-Remitente/tratamiento farmacológico , Neopterin/sangre , Factor de Necrosis Tumoral alfa/análisis , Molécula 1 de Adhesión Celular Vascular/sangre , Adulto , Femenino , Humanos , Masculino , Factores de Tiempo
18.
Rev Neurol ; 39(1): 37-40, 2004.
Artículo en Español | MEDLINE | ID: mdl-15257526

RESUMEN

INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is a rare disease characterized by deafness, ataxia and pyramidal dysfunction. It is due to hemosiderin deposition in the subpial membranes of the brain, spinal cord and cranial nerves. Most cases are secondary to chronic or recurrent bleeding into the subarachnoid space. Diagnosis is permitted by magnetic resonance imaging (MRI). CASE REPORTS: We report two patients with a chronic, slowly progressive cerebellar ataxia and hearing loss. MRI showed T2 hypointense signals in the brain, cerebellum and spinal cord diagnostic of superficial siderosis of the CNS. Xanthochromia was present in one patient. Evoked potentials showed retrochoclear hearing loss. Extensive vascular studies were negative for bleeding sources. One patient, treated with oral anticoagulants, benefited from reduction of the International Normalized Ratio. CONCLUSION: Due to its rarity, clinical suspicion is essential for diagnosis of superficial siderosis of the CNS.


Asunto(s)
Enfermedades del Sistema Nervioso Central/diagnóstico , Enfermedades del Sistema Nervioso Central/patología , Siderosis/diagnóstico , Siderosis/patología , Anciano , Ataxia , Cerebelo/patología , Cerebelo/fisiopatología , Femenino , Pérdida Auditiva/etiología , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad
19.
Rev. neurol. (Ed. impr.) ; 39(1): 37-40, 1 jul., 2004. ilus
Artículo en Es | IBECS | ID: ibc-33825

RESUMEN

Introducción. La siderosis superficial del sistema nervioso central (SNC) es una enfermedad rara, caracterizada por sordera, ataxia y disfunción piramidal. Se debe al depósito de hemosiderina en las membranas subpiales del cerebro, la médula espinal y los nervios craneales. La mayoría de los casos son secundarios a un sangrado crónico o recurrente en el espacio subaracnoideo. Se requiere la realización de una resonancia magnética (RM) para alcanzar el diagnóstico. Casos clínicos. Presentamos dos pacientes con un cuadro crónico de ataxia cerebelosa y pérdida auditiva progresivas. En ambos casos, la RM mostraba señales hipointensas en T2 en el cerebro, el cerebelo y la médula espinal, diagnósticas de siderosis superficial del SNC. En uno de los pacientes se demostró xantocromía en el análisis del líquido cefalorraquídeo. Los potenciales evocados auditivos revelaron, en ambos casos, una pérdida auditiva retrococlear. Se realizaron estudios vasculares exhaustivos sin que se pudiera demostrar la presencia de fuentes de sangrado. Uno de los pacientes, que recibía tratamiento con anticoagulantes orales, se benefició de la reducción del INR. Conclusión. Debido a su rareza, la sospecha clínica es esencial para el diagnóstico de esta enfermedad (AU)


Introduction. Superficial siderosis of the central nervous system (CNS) is a rare disease characterized by deafness, ataxia and pyramidal dysfunction. It is due to hemosiderin deposition in the subpial membranes of the brain, spinal cord and cranial nerves. Most cases are secondary to chronic or recurrent bleeding into the subarachnoid space. Diagnosis is permitted by magnetic resonance imaging (MRI). Case reports. We report two patients with a chronic, slowly progressive cerebellar ataxia and hearing loss. MRI showed T2 hypointense signals in the brain, cerebellum and spinal cord diagnostic of superficial siderosis of the CNS. Xanthochromia was present in one patient. Evoked potentials showed retrochoclear hearing loss. Extensive vascular studies were negative for bleeding sources. One patient, treated with oral anticoagulants, benefited from reduction of the International Normalized Ratio. Conclusion. Due to its rarity, clinical suspicion is essential for diagnosis of superficial siderosis of the CNS (AU)


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Asunto(s)
Persona de Mediana Edad , Anciano , Femenino , Humanos , Receptores de Superficie Celular , Ataxia , Enfermedades del Sistema Nervioso Central , Cerebelo , Pérdida Auditiva , Imagen por Resonancia Magnética , Siderosis , Demencia por Múltiples Infartos , Mutación , Linaje , Proteínas Proto-Oncogénicas
20.
J Immunoassay Immunochem ; 24(1): 1-10, 2003.
Artículo en Inglés | MEDLINE | ID: mdl-12680603

RESUMEN

A one-site ELISA for the quantification of recombinant human gamma interferon (rh-IFN-gamma) was developed and validated. A single monoclonal antibody (Mab) was used as a "catching" antibody and as a horseradish peroxidase (HRP)-labeled conjugate. Detection limit and quantification limit of this assay were estimated to be 1.26 and 15 ng/mL, respectively, and the coefficient of variation was below 15%. The ELISA was specific for rh-IFN-gamma, showing no cross reactivity to other related molecules in the range of the concentrations studied. The results correlated well with those obtained by a bioassay method. By using this assay, it was demonstrated that 0.01-1% (v/v) Tween 80 protected rh-IFN-gamma during freezing and thawing.


Asunto(s)
Ensayo de Inmunoadsorción Enzimática/métodos , Interferón gamma/análisis , Anticuerpos Monoclonales/inmunología , Sitios de Unión de Anticuerpos , Reacciones Cruzadas , Endopeptidasas , Calor , Humanos , Interferón gamma/inmunología , Proteínas Recombinantes , Reproducibilidad de los Resultados , Tensoactivos
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