Immune thrombotic thrombocytopenic purpura in older patients: Results from the Spanish TTP Registry (REPTT).

Immune thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare disease that seldom occurs in the elderly. Few reports have studied the clinical course of iTTP in older patients. In this study, we have analysed the clinical characteristics at presentation and response to therapy in a series of 44 patients with iTTP ≥60 years at diagnosis from the Spanish TTP Registry and compared them with 209 patients with <60 years at diagnosis from the same Registry. Similar symptoms and laboratory results were described in both groups, except for a higher incidence of renal dysfunction among older patients (23% vs. 43.1%; p = 0.008). Front-line treatment in patients ≥60 years was like that administered in younger patients. Also, no evidence of a difference in clinical response and overall survival was seen in both groups. Of note, 14 and 25 patients ≥60 years received treatment with caplacizumab and rituximab, respectively, showing a favourable safety and efficacy profile, like that observed in patients <60 years.

Compression, Rupture, and Puncture of Model Membranes at the Molecular Scale.

Elastic properties of biological membranes are involved in a large number of membrane functionalities and activities. Conventionally characterized in terms of Young's modulus, bending stiffness and stretching modulus, membrane mechanics can be assessed at high lateral resolution by means of atomic force microscopy (AFM). Here we show that the mechanical response of biomimetic model systems such as supported lipid bilayers (SLBs) is highly affected by the size of the AFM tip employed as a membrane indenter. Our study is focused on phase-separated fluid-gel lipid membranes at room temperature. In a small tip radius regime (≈ 2 nm) and in the case of fluid phase membranes, we show that the tip can penetrate through the membrane minimizing molecular vertical compression and in absence of molecular membrane rupture. In this case, AFM indentation experiments cannot assess the vertical membrane Young's modulus. In agreement with the data reported in the literature, in the case of larger indenters (>2 nm) SLBs can be compressed leading to an evaluation of Young's modulus and membrane maximal withstanding force before rupture. We show that such force increases with the indenter in agreement with the existing theoretical frame. Finally, we demonstrate that the latter has no influence on the number of molecules involved in the rupture process that is observed to be constant and rather dependent on the indenter chemical composition.

Evaluation of Spatial Pattern of Altered Flow Regimes on a River Network Using a Distributed Hydrological Model.

Alteration of the spatial variability of natural flow regimes has been less studied than that of the temporal variability, despite its ecological importance for river ecosystems. Here, we aimed to quantify the spatial patterns of flow regime alterations along a river network in the Sagami River, Japan, by estimating river discharge under natural and altered flow conditions. We used a distributed hydrological model, which simulates hydrological processes spatiotemporally, to estimate 20-year daily river discharge along the river network. Then, 33 hydrologic indices (i.e., Indicators of Hydrologic Alteration) were calculated from the simulated discharge to estimate the spatial patterns of their alterations. Some hydrologic indices were relatively well estimated such as the magnitude and timing of maximum flows, monthly median flows, and the frequency of low and high flow pulses. The accuracy was evaluated with correlation analysis (r > 0.4) and the Kolmogorov-Smirnov test (α = 0.05) by comparing these indices calculated from both observed and simulated discharge. The spatial patterns of the flow regime alterations varied depending on the hydrologic indices. For example, both the median flow in August and the frequency of high flow pulses were reduced by the maximum of approximately 70%, but these strongest alterations were detected at different locations (i.e., on the mainstream and the tributary, respectively). These results are likely caused by different operational purposes of multiple water control facilities. The results imply that the evaluation only at discharge gauges is insufficient to capture the alteration of the flow regime. Our findings clearly emphasize the importance of evaluating the spatial pattern of flow regime alteration on a river network where its discharge is affected by multiple water control facilities.

Cell competition may function either as tumour-suppressing or as tumour-stimulating factor in Drosophila.

Drosophila endocytosis-defective cells develop tumour overgrowths in the imaginal discs. We have analysed the tumorigenic potential of cells mutant for Rab5, a gene involved in endocytosis. We found that while a compartment entirely made by Rab5 mutant cells can grow indefinitely, clones of Rab5 cells surrounded by normal cells are eliminated by cell competition. However, when a group of about 400 cells are simultaneously made mutant for Rab5, they form an overgrowing tumour: mutant cells in the periphery are eliminated, but those inside survive and continue proliferating because they are beyond the range of cell competition. These results identify group protection as a mechanism to evade the tumour-suppressing function of cell competition in Drosophila. Furthermore, we find that the growth of the tumour depends to a large extent on the presence of apoptosis inside the tumour: cells doubly mutant for Rab5 and the proapoptotic gene dronc do not form overgrowing tumours. These results suggest that the apoptosis caused by cell competition acts as a tumour-stimulating factor, bringing about high levels of Jun N-terminal kinase and subsequently Wg/Dpp signalling and high proliferation levels in the growing tumour. We conclude that under these circumstances cell competition facilitates the progression of the tumour, thus reversing its normal antitumour role.

Analysis of genetic diversity in Bolivian llama populations using microsatellites.

South American camelids (SACs) have a major role in the maintenance and potential future of rural Andean human populations. More than 60% of the 3.7 million llamas living worldwide are found in Bolivia. Due to the lack of studies focusing on genetic diversity in Bolivian llamas, this analysis investigates both the genetic diversity and structure of 12 regional groups of llamas that span the greater part of the range of distribution for this species in Bolivia. The analysis of 42 microsatellite markers in the considered regional groups showed that, in general, there were high levels of polymorphism (a total of 506 detected alleles; average PIC across per marker: 0.66), which are comparable with those reported for other populations of domestic SACs. The estimated diversity parameters indicated that there was high intrapopulational genetic variation (average number of alleles and average expected heterozygosity per marker: 12.04 and 0.68, respectively) and weak genetic differentiation among populations (FST range: 0.003-0.052). In agreement with these estimates, Bolivian llamas showed a weak genetic structure and an intense gene flow between all the studied regional groups, which is due to the exchange of reproductive males between the different flocks. Interestingly, the groups for which the largest pairwise FST estimates were observed, Sud Lípez and Nor Lípez, showed a certain level of genetic differentiation that is probably due to the pattern of geographic isolation and limited communication infrastructures of these southern localities. Overall, the population parameters reported here may serve as a reference when establishing conservation policies that address Bolivian llama populations.

Analysis of mitochondrial DNA in Bolivian llama, alpaca and vicuna populations: a contribution to the phylogeny of the South American camelids.

The objectives of this work were to assess the mtDNA diversity of Bolivian South American camelid (SAC) populations and to shed light on the evolutionary relationships between the Bolivian camelids and other populations of SACs. We have analysed two different mtDNA regions: the complete coding region of the MT-CYB gene and 513 bp of the D-loop region. The populations sampled included Bolivian llamas, alpacas and vicunas, and Chilean guanacos. High levels of genetic diversity were observed in the studied populations. In general, MT-CYB was more variable than D-loop. On a species level, the vicunas showed the lowest genetic variability, followed by the guanacos, alpacas and llamas. Phylogenetic analyses performed by including additional available mtDNA sequences from the studied species confirmed the existence of the two monophyletic clades previously described by other authors for guanacos (G) and vicunas (V). Significant levels of mtDNA hybridization were found in the domestic species. Our sequence analyses revealed significant sequence divergence within clade G, and some of the Bolivian llamas grouped with the majority of the southern guanacos. This finding supports the existence of more than the one llama domestication centre in South America previously suggested on the basis of archaeozoological evidence. Additionally, analysis of D-loop sequences revealed two new matrilineal lineages that are distinct from the previously reported G and V clades. The results presented here represent the first report on the population structure and genetic variability of Bolivian camelids and may help to elucidate the complex and dynamic domestication process of SAC populations.

Web-enabled registry of inherited bleeding disorders in Portugal: conditions and perception of the patients.

Local and national haemophilia registries are powerful instruments to support the healthcare and researchers and improve the communication between Comprehensive Haemophilia Diagnostic and Treatment Centres (HTCs) and patients. Hemo@care is an example of a Local Haemophilia Registry Systems (LHR_Sys) based on the Web, developed in collaboration with a HTC located in Portugal, to support the haemophilia treatments registry, collect and manage the clinical information and provide mechanisms to control the clotting factor concentrates (CFC) stock. To extend this solution (the hemo@care) to other Portuguese HTCs and consequently to meet the preconditions to create a National Haemophilia Registry Systems (NHR_Sys), a study based on a questionnaire was carried out at nationwide. This study aims to assess the conditions and motivations of people with haemophilia (PWH) geographically scattered throughout the country, to use a potential Web-enabled registry with the purpose of replacing the traditional paper-diaries, to understand their judgment about a potential NHR_Sys currently non-existent in Portugal, and at the same time, to characterize demographically and pathologically those people at the nationwide. The results based on the analysis of 168 responses (response rate of 31%) confirmed the high prevalence of the disease in haemophilia A (75%) compared with haemophilia B (11.3%) and a large incidence in the severe levels, or the existence of people with mild severity without diagnosis and treatment. Furthermore, the results also revealed the need, conditions and motivation for using a registry system by PWH; thus it is deemed to justify the extension of the hemo@care to other HTCs in Portugal and consequently to create the NHR_Sys.

Contaminación Enterobacteriana del Guarapo de una Fábrica de Cochabamba, en Fermentación Normal y Fermentación Flemosa / Guarapo Enterobacteriana Pollution in the Normal and Fermentation Fermentation Phlegmy of a Factory Cochabamba

El guarapo es una bebida alcohólica que sigue la vía de fermentación y un desvío del mismo resulta en guarapo de consistencia flemosa. La presencia de enterobacterias (entéricas fecales y la E. coli) son indicadores de contaminación y tienen relación con los grupos tifoide-paratifoide. El objetivo de la investigación fue determinar la contaminación enterobacteriana presente en el guarapo en fermentación normal y en fermentación de consistencia flemosa en muestras recolectadas en una fábrica de la provincia Cercado-Cochabamba. Se realizó un estudio de tipo transversal y descriptivo tomando en cuenta 9 muestras de guarapo de consistencia flemosa y una muestra de guarapo en fermentación normal tomada al azar, durante el tiempo de estudio establecido. Se encontró 100% de coliformes fecales en las muestras dos, tres y cinco, existiendo contaminación de gravedad entre 20.000 a 2.640.000 UFC/ml sobrepasando el parámetro normal < 10 UFC/ml, causado posiblemente por el escaso control de calidad y saneamiento.

The cane juice is an alcoholic beverage that follows the route of fermentation and diverted the same results in consistency guarapo phlegmy. The presence of Enterobacteriaceae (enteric coliforms and E. coli) are indicators of pollution and are linked to the typhoid groups - paratyphoid.The research objective was to determine the contamination present in the juice enterobacteriana in normal fermentation and fermentation phlegmy consistency in samples collected from a factory in the province Cercado - Cochabamba.A study of cross-sectional and descriptive sample taking into account 9 phlegmy consistency of juice and a sample of juice in normal fermentation chosen at random during the study period established. We found 100% of fecal coliform in the samples two, three and five, there contamination of gravity between 20,000 to 2,640,000 CFU / ml exceeding the normal parameter < 10 CFU / ml, possibly caused by the low quality control and sanitation.

Consenso elaborado por la Sociedad Chilena de Endocrinología y Diabetes sobre resistencia a la insulina(RI) y síndrome metabólico (SM): aspectos clínicos y terapéuticos / Chilean society of endocrinology and diabetes consensus on clinical and therapeutic aspects of insulin resistance and metabolic syndrome

Background: The concept insulin resistance as the basis for a series of metabolic alterations and diseases was introduced by Gerald Reaven in 1988, when he described a cluster of alterations that named syndrome X. Aim: To review and discuss the present information about insulin resistance (IR) and metabolic syndrome (MS). Material and methods: The IR concept is defined,the affected metabolic ways, its consequences and relationship with different diseases are presented. The importance of central obesity with its metabolic, inflammatory and prothrombotic consequences playing a key role in cardiovascular risk, is discussed. The cluster of factors focused on cardiovascular disease and eventually diabetes is named MS. Several definitions of MS are analyzed and compared. A proposition is made about the definition to be used in the Chilean population. Differences between IR syndrome and MS are discussed. Diagnostic methods of IR and MS are presented, recommendations are made about their usefulness and reliability. Non pharmacological and pharmacological treatments of IR and MS are analyzed. Other related diseases, such as polycystic ovary syndrome, non alcoholic steatohepatitis and sleep apnea are discussed. Conclusions. Until further studies are made to define a local waist circumference cut-off associated with high risk, the ATPIII MS definition is preferred. A clinical approach is recommended for diagnosis. A search for all components of the MS is important. There is no evidence about the benefits of MS treatment on the prevention of cardiovascular diseases or diabetes. Evidence supports the use of lifestyle changes and some drugs, such as metformin on the prevention of diabetes in prediabetic states.

Childhood injury prevention practices by parents in Mexico.

OBJECTIVE: Scientifically based injury prevention efforts have not been widely implemented in Latin America. This study was undertaken to evaluate the baseline knowledge and practices of childhood safety on the part of parents in Monterrey, Mexico and in so doing provide information on which to base subsequent injury prevention efforts. METHODS: Interviews were carried out with parents from three socioeconomic strata (upper, middle, lower). Questionnaires were based on Spanish language materials developed by The Injury Prevention Program (TIPP) of the American Academy of Pediatrics. RESULTS: Data were obtained from parents of 1123 children. Overall safety scores (percent safe responses) increased with increasing socioeconomic status. The differences among the socioeconomic groups were most pronounced for transportation and less pronounced for household and recreational safety. The differences were most notable for activities that required a safety related device such as a car seat, seat belt, helmet, or smoke detector. Appropriate use of such devices declined from 47% (upper socioeconomic group) to 25% (middle) to 15% (lower). CONCLUSIONS: Considerable differences in the knowledge and especially the practice of childhood safety exist among parents in different socioeconomic levels in Mexico. Future injury prevention efforts need to address these and especially the availability, cost, and utilization of specific highly effective safety devices.

Enfermedad luxante de cadera: tratamiento con osteotomía de Salter seguimiento a largo plazo / Congenital hip dislocation: treatment with Salterïs osteotomy a long-term review

Diecinueve pacientes con 22 caderas luxadas congénitas fueron tratadas con reducción abierta y osteotomía de Salter del hueso innominado. El seguimiento clínico promedio fue de 24,7 años (rango entre 20,5 a 30,4 años), y el seguimiento radiológico promedio fue de 14,8 años (rango entre 5,9 a 24 años). Resultados excelentes y buenos se obtuvieron en 18 caderas (82 por ciento), utilizando el score de Gulman. El resultado radiológico fue excelente y bueno en 13 caderas (59 por ciento), según el score de Severin. Complicaciones: 6 caderas (27 por ciento) desarrollaron necrosis de la cabeza femoral tipo II de Bucholz y Ogden. Hubo un caso de infección profunda (4,5 por ciento). Nuestros hallazgos demuestran que la osteotomía de Salter proporcionan una mejoría real y persistente en pacientes con enfermedad luxante de caderas con displasia acetabular severa

Metabolismo de lípidos: inactividad y ejercicio físico / Lipid metabolism: inactivity and physical activity

Sedentarism is a first rank risk factor for the development of obesity in genetically, susceptible individuals. The regular practice of physical activity is one of the most effective methods for the prevention of obesity and for the defense of a weight loss. Besides it has a beneficial influence on metabolic parameters, even independently of weight loss. Skeletal muscle of trained subjects has a greater capacity for the oxidation of lipids relative to carbohydrates as the result of a series of adaptive processes of the muscle; processes responsible of the beneficial effects of the regular practice of physical activity

Medicina de la actividad física en niños y adolescentes: evaluación médica previa a la práctica deportiva / Sport medicine in children and adolescents: sport preparticipation examination

Actualmente es habitual que en la supervisión de salud pediátrica se recomiende a niños y adolescentes la práctica del juego libre, ejercicios o deporte, dado que estos contribuyen a su óptimo desarrollo físico, socioemocional e intelectual; además pueden proporcionarles una mejor dimensión acerca de sus capacidades y habilidades, así como confianza en sí mismos y fomento de la sociabildad.. También los pacientes portadores de enfermedades crónicas pueden acceder a prácticas deportivas controladas para lograr estos objetivos. La Medicina del Deporte surgió como una necesidad ante las competencias deportivas que requieren altos rendimientos. Esta especialidad médica debiera estar dedicada a la promoción de la salud, prevención de lesiones y a la rehabilitación del deportista profesional o aficionado; aunque en Chile no esta reconocida como tal, existen médicos con formación en esta especialidad; pero dado que son escasos, es el pediatra el que recibe gran parte de las consultas para evaluación predeportiva en niños y adolescentes

La disciplina mucho más que un castigo: rol del pediatra / Discipline much more than a punishment: role of pediatrician

La crianza de los niños requiere de una continua dedicación por parte de los padres. Estos buscan enseñar a sus hijos a desarrollar sus máximas capacidades, autocontrol y una conducta moral, entre otras. La disciplina constituye una herramienta para lograr estos objetivos. Cuando los padres se ven desorientados respecto a estos temas, recurren al pediatra, al cual consideran muchas veces un experto en la crianza de niños. Sin embargo, puede ocurrir con frecuencia que los consejos dados por el médico puedan basarse en su experiencia personal más que en estudios sobre desarrollo, comportamiento y técnicas disciplinarias. Se revisará en este artículo un concepto actual de disciplina, sus componentes, factores que determinan su estructuración y diferentes técnicas disciplinarias que hoy se proponen. Se busca apoyar a pediatra, médicos, familiares y profesionales de la salud que acompañan al niño y su familia desde los primeros años de vida. Es útil e importante que conozcan diferentes estrategias de intervención frente al problema de la disciplina

Condrólisis idiopática de la cadera / Idiopathic chondrolysis of the hip

La condrólisis idiopática de la cadera es una entidad clínica caracterizada por destrucción extensa del cartílago de la epífisis y del acetábulo. Se analiza en forma retrospectiva 6 pacientes (4 mujeres y 2 hombres) tratados y controlados entre 1987 y 1999 en el Servicio de Ortopedia y Traumatología Infantil del Hospital Clínico San Borja Arriarán. Cuatro enfermos fueron diagnosticados como condrolisis idiopáticas de cadera en etapa activa y en período de secuela. La edad promedio de iniciación de los síntomas fue de 12 años 9 meses. La lesión estuvo localizada en el lado derecho en 4 pacientes y en el izquierdo en 2. Todos los enfermos presentaron dolor, claudicación con rigidez progresiva de la cadera con marcada limitación funcional e incluso anquilosis en 1 caso. Se observó disminución del espacio articular que en un caso mostró fusión articular parcial. Se comprobó ensanchamiento de epífisis y cuello femorales. Hubo un importante aumento del ángulo CE de Wiberg en comparación con el lado sano. El tratamiento efectuado fue artroplastía biológica en 3 enfermos. En dos de éstos se interpuso moltoprén entre las superficies articulares. En dos pacientes se realizó capsulectomía según técnica de Roy-Crawford y en uno osteomía de alineamiento. Hubo desaparición del dolor entre 5 meses a un año en todos los enfermos. Recuperación notable de la movilidad en los 3 enfermos con artroplastía biológica. En uno de estos pacientes reapareció el dolor después de 20 años de control. Dos enfermos con capsulectomía subtotal tuvieron una mínima recuperación de la movilidad articular. En la sexta enferma, diagnosticada en etapa secuelar, la osteotomía de alineamiento permitió corregir la flexo aducción de la cadera

Spontaneous murine lung adenocarcinoma (P07): A new experimental model to study paraneoplastic syndromes of lung cancer.

We report the histological and biological behavior characteristics of a lung tumor (P07) that arose spontaneously in a Balb/c mouse. P07 is a moderately to poorly differentiated adenocarcinoma that secretes granulocyte-macrophage colony stimulating factor (GM-CSF) in culture supernatants. This tumor presents some paraneoplastic syndromes, such as leukocytosis, hypercalcemia and cachexia. taken together with the peripheral blood leukocyte (PBL) counts and serum calcium levels during s.c. tumor growth and after surgery, this study suggests that P07 may be a useful experimental model to study the biology of lung cancer and paraneoplastic syndromes.

Trastornos de la conciencia del yo en la esquizofrenia / Disturbances of self-conciousness in schizophrenia

Se hace una revisión somera de los trastornos de la conciencia del yo, siguiendo el esquema introducido por Honorio Delgado, para la exposición de esta clase de temas; se describen sintéticamente las perturbaciones más importantes de esta función halladas en la esquizofrenia, varias de las cuales son capaces de fungir como patogenéticas, el A. postula la negación de la identidad como síntoma típicamente esquizofrénico.

[Reproducibility of nuclear grade in breast neoplasm. A multicenter experience]. / Reproducibilidad del grado nuclear en el cáncer de mama. Una experiencia multicéntrica.

Nuclear grade is considered a valuable prognostic factor in mammary carcinomas. Since the histological diagnosis of most of these tumors is made by "non expert" pathologists, it was considered interesting to find out the reproducibility of general pathologists to define the nuclear grade. In order to do this, a series of 15 mammary carcinomas, 10 of them randomly selected and 5 because they were considered difficult to classify for nuclear grade, were examined separately by 10 general pathologists. In a first round of observation, each one of them graded the cases according to their own criteria as used routinely, and for a second round they followed a written guide. An analysis of variance was applied to the data and no significant differences were found between observers, neither in the randomly selected cases nor in the total series. The written guide, surprisingly, instead of lowering the differences, increased them. Analysis of the individual performance of observers showed two of them having a great variation between both rounds of observation, and this was considered to influence the results of the whole group. Interobserver performance to discriminate high grade tumors (G3) from the rest, showed a good correlation in all the participants. These results allow us to conclude that in this series, examined by general pathologists, an acceptable reproducibility was observed, specially when high risk tumors were being identified.

CIE-10 y DSM-III-R: Semejanzas y diferencias / ICD-10 & DSM-III-R: Similarities and differences

Se presentan las categorías diagnósticas de la décima revisión de la Clasificación Internacional de Enfermedades (CIE-10) y la tercera edición revisada del Manual Diagnóstico y Estadístico de Trastornos Mentales de la Asociación Psiquiátrica Americana (DSM-III-R), para pacientes adultos y geriátricos, señalando sus semejanzas y diferencias. Asimismo, se comentan las bondades y deficiencias de cada una de ellas. Finalmente, se resumen las diferencias entre la CIE-10 y el DSM-III-R.

The diagnostic categories corresponding to adult and geriatric patients of the International Classification of Diseases, tenth Revision (ICD-10), and the American Psychiatric AssociationÆs Diagnostic and Statistical Manual of Mental Disorders, third edition revised (DSM-III-R) are presented. The similarities and differences are pointed out. Likewise, the goodness and deficiencies of each classification are commented. Finally, the differences between ICD-10 and DSM-III-R are summarized.