Dengue importado y autóctono en España / Imported and autochthonous dengue in Spain

El dengue es, globalmente, la arbovirosis más importante. Está causado por el virus del dengue y transmitido generalmente por la picadura de mosquitos del género Aedes (Ae aegypti o Ae albopictus). En España fue inicialmente erradicado en el siglo xx, junto con el vector Aeaegypti, y en la actualidad la mayoría de los casos notificados en España son importados por viajeros procedentes de países con transmisión de dengue (dengue importado). Sin embargo, en los últimos años se han descrito casos de dengue de personas residentes en España que no habían viajado a zonas con transmisión conocida del virus (dengue autóctono), transmitidos por Aedes albopictus (el denominado mosquito tigre), presente especialmente en la cuenca mediterránea. Se requiere por lo tanto un buen conocimiento de esta enfermedad, ya que puede dar lugar a cuadros clínicos graves, de modo que pueda ser diagnosticada precozmente y manejada correctamente, disminuyendo con ello su mortalidad, así como su eventual transmisión autóctona (AU)

Dengue is globally the most important arboviral infection. It is caused by the dengue virus and it is generally transmitted by Aedes mosquitoes’ bites (Ae aegypti or Ae albopictus). In Spain it was initially eradicated in the 20th century, together with the Ae aegypti vector, and currently most of the cases reported in Spain are imported by travelers from countries with dengue transmission (imported dengue). However, in recent years, cases of dengue have been described in people residing in Spain who had not traveled to areas with known transmission (autochthonous dengue), transmitted by Aedes albopictus (the so-called tiger mosquito), present especially in the Mediterranean basin. Therefore, a good knowledge of this potentially severe disease is required, so that it can be diagnosed early, and managed correctly, thus reducing its mortality, as well as its eventual autochthonous transmission (AU)

Imported and autochthonous dengue in Spain.

Dengue is globally the most important arboviral infection. It is caused by the dengue virus and it is generally transmitted by Aedes mosquitoes' bites (Ae aegypti or Ae albopictus). In Spain it was initially eradicated in the 20th century, together with the Ae aegypti vector, and currently most of the cases reported in Spain are imported by travelers from countries with dengue transmission (imported dengue). However, in recent years, cases of dengue have been described in people residing in Spain who had not traveled to areas with known transmission (autochthonous dengue), transmitted by Aedes albopictus (the so-called tiger mosquito), present especially in the Mediterranean basin. Therefore, a good knowledge of this potentially severe disease is required, so that it can be diagnosed early, and managed correctly, thus reducing its mortality, as well as its eventual autochthonous transmission.

Real-life experience of hepatitis C treatment in a Spanish prison.

OBJECTIVE: Hepatitis C virus (HCV) infection is a major public health problem that causes multiple comorbidities. People in prisons who inject intravenous drugs are at increased risk of HCV infection, and HCV infection is 15-fold more prevalent among prisoners compared with the community. The objective of this study was to analyse the clinical and epidemiological characteristics of residents of a Spanish prison with HCV infection who received antiviral treatment. METHODS: An observational, descriptive and retrospective study was performed. All patients with HCV infection diagnosed or followed up in an Infectious Diseases attached to a penitentiary were included in this study. RESULTS: Of 81 patients analysed, sixty-nine (83.1%) patients were male. The mean age was 50.1 (SD8.8) years, and 70% of the inmates had a history of injection drug use. Coinfection with HIV was detected in 30%. In up to 25% of the sample, there were data on chronic liver disease in the degree of liver cirrhosis. The diagnosis of HCV infection had been made more than 15 years earlier in 28% of those studied. Decompensations from liver disease, hepatocellular carcinoma, or hospital admissions were exceptional. Most of the inmates with HCV accepted treatment, and approximately 94% of the patients who completed treatment achieved a sustained virological response without interactions or complications of interest. CONCLUSIONS: The availability of direct-acting antivirals and their exceptional side effects constitute an opportunity to reduce the burden of HCV infection in Spain, particularly in these high-risk populations.

Efficacy of dual carbapenem treatment in a murine sepsis model of infection due to carbapenemase-producing Acinetobacter baumannii.

OBJECTIVES: To evaluate the in vivo efficacy of a dual carbapenem combination containing imipenem plus meropenem against carbapenem-resistant Acinetobacter baumannii producing carbapenemases OXA-23 or OXA-58. METHODS: An experimental model of peritonitis using C57BL/6J female mice was developed and the minimum lethal doses were calculated for infections due to OXA-23 or OXA-58 producers of A. baumannii clinical isolates. The efficacies of the carbapenems in monotherapy and in combination were tested. RESULTS: Meropenem was better than imipenem in mice infected with either of the carbapenem-resistant A. baumannii (CRAb) strains. The combination of meropenem plus imipenem significantly improved the clearance of CRAbs from spleen compared with non-treated groups. The carbapenem-containing combination was better than imipenem for treating mice infected with both carbapenemase producers. In blood, the carbapenem combination significantly decreased the bacterial load of the OXA-23 producers compared with imipenem or meropenem used in monotherapy. CONCLUSIONS: These results suggest that dual carbapenem combination could be an option for the treatment of infections due to carbapenemase-producing A. baumannii such as OXA-23 and OXA-58 producers.

Sequential pretreatment to recover carbohydrates and phosphorus from Desmodesmus sp. cultivated in municipal wastewater.

This study focused on the simultaneous recovery of carbohydrates (CHO) and phosphorus (P) from Desmodesmus sp. biomass cultivated in municipal wastewater, through a sequential pretreatment. The pretreatment consisted first of ultrasound to trigger cell disruption followed by ozonation to recover CHO and P. For ozone pretreatment, three different parameters were considered: ozone concentration (9, 15, 21, 27, 36, and 45 mg O3/L), contact time (15, 25 and 35 min), and pH (8 and 11). The maximum simultaneous release of 84% of CHO and 58% of P was achieved at the experimental parameters of ozone concentration of 45 mg O3/L, contact time of 35 min, and pH of 11. Also, P was concentrated in solution by 8- to 14-fold with respect to municipal wastewater. The sequential pretreatment was conducted at alkaline pH of 11 and atmospheric conditions, which may considerably reduce energy demand and reagents, in comparison to a traditional hydrolysis pretreatment. The results found suggest that the sequential pretreatment could be feasible on a large scale.

Intensified recovery of lipids, proteins, and carbohydrates from wastewater-grown microalgae Desmodesmus sp. by using ultrasound or ozone.

This study evaluates the effect of ultrasound and ozone pretreatments for the subsequent recovery of Desmodesmus sp. biocomponents-lipids, proteins, and carbohydrates-using a response surface methodology. Both pretreatments impact on the recovered lipids quality, solvent waste production and extraction time is analysed for process intensification purposes. For ultrasound pretreatment, independent parameters were energy applied (50-200 kWh/kg dry biomass), biomass concentration (25-75 g/L), and ultrasonic intensity (0.32 and 0.53 W/mL). While for ozone pretreatment, independent parameters were ozone concentration (3-9 mg O3/L), biomass concentration (25-75 g/L), and contact time (5-15 min). In the case of ultrasound pretreatment, recovery yield reached 97 ±â€¯0.4%, 89 ±â€¯3%, and 73 ±â€¯0.6% for proteins, carbohydrates and lipids respectively. Given process required: energy applied of 50 kWh/kg dry biomass, 75 g/L of biomass concentration, 0.32 W/mL of ultrasonic intensity, and 56 min of time process. Ultrasound caused high cell disruption releasing all proteins, thereby obviating downstream processing for its recovery. Ozone pretreatment recovery yield was 85 ±â€¯2%, 48 ±â€¯1.4%, and 25 ±â€¯1.3%, for carbohydrates, lipids and proteins respectively, under the following conditions: 9 mg O3/L of ozone concentration, 25 g/L of biomass concentration, and 5 min of contact time that depicts an energy consumption of 30.64 kWh/kg dry biomass. It was found that ultrasound and ozone pretreatments intensified the lysis and biocomponents recovery process by reducing solvent consumption by at least 92% and extraction time between 80% and 90% compared with extraction of untreated biomass biocomponents. Both pretreatments improve the composition of the recovered lipids. It was noted that the yield of neutral lipids increased from 28% to 67% for ultrasound pretreatment while for ozone pretreatment from 49% to 63%. The method used for lipid extraction may also have an effect but here it was kept constant.

Sphingomyelin-induced inhibition of the plasma membrane calcium ATPase causes neurodegeneration in type A Niemann-Pick disease.

Niemann-Pick disease type A (NPA) is a rare lysosomal storage disorder characterized by severe neurological alterations that leads to death in childhood. Loss-of-function mutations in the acid sphingomyelinase (ASM) gene cause NPA, and result in the accumulation of sphingomyelin (SM) in lysosomes and plasma membrane of neurons. Using ASM knockout (ASMko) mice as a NPA disease model, we investigated how high SM levels contribute to neural pathology in NPA. We found high levels of oxidative stress both in neurons from these mice and a NPA patient. Impaired activity of the plasma membrane calcium ATPase (PMCA) increases intracellular calcium. SM induces PMCA decreased activity, which causes oxidative stress. Incubating ASMko-cultured neurons in the histone deacetylase inhibitor, SAHA, restores PMCA activity and calcium homeostasis and, consequently, reduces the increased levels of oxidative stress. No recovery occurs when PMCA activity is pharmacologically impaired or genetically inhibited in vitro. Oral administration of SAHA prevents oxidative stress and neurodegeneration, and improves behavioral performance in ASMko mice. These results demonstrate a critical role for plasma membrane SM in neuronal calcium regulation. Thus, we identify changes in PMCA-triggered calcium homeostasis as an upstream mediator for NPA pathology. These findings can stimulate new approaches for pharmacological remediation in a disease with no current clinical treatments.

Acquisition of Competencies by Medical Students in Neurological Emergency Simulation Environments Using High Fidelity Patient Simulators.

The training of medical students demands practice of skills in scenarios as close as possible to real ones that on one hand ensure acquisition of competencies, and on the other, avoid putting patients at risk. This study shows the practicality of using high definition mannequins (SimMan 3G) in scenarios of first attention in neurological emergencies so that medical students at the Faculty of Medicine of the University of Salamanca could acquire specific and transversal competencies. The repetition of activities in simulation environments significantly facilitates the acquisition of competencies by groups of students (p < 00.5). The greatest achievements refer to skills whereas the competencies that demand greater integration of knowledge seem to need more time or new sessions. This is what happens with the competencies related to the initial diagnosis, the requesting of tests and therapeutic approaches, which demand greater theoretical knowledge.

Electrochemical removal of carbamazepine in water with Ti/PbO2 cylindrical mesh anode.

Carbamazepine (CBZ) is one of the most frequently detected organic compounds in the aquatic environment. Due to its bio-persistence and toxicity for humans and the environment its removal has become an important issue. The performance of the electrochemical oxidation process and in situ production of reactive oxygen species (ROS), such as O3 and H2O2, for CBZ removal have been studied using Ti/PbO2 cylindrical mesh anode in the presence of Na2SO4 as supporting electrolyte in a batch electrochemical reactor. In this integrated process, direct oxidation at anode and indirect oxidation by in situ electrogenerated ROS can occur simultaneously. The effect of several factors such as electrolysis time, current intensity, initial pH and oxygen flux was investigated by means of an experimental design methodology, using a 2(4) factorial matrix. CBZ removal of 83.93% was obtained and the most influential parameters turned out to be electrolysis time, current intensity and oxygen flux. Later, the optimal experimental values for CBZ degradation were obtained by means of a central composite design. The best operating conditions, analyzed by Design Expert(®) software, are the following: 110 min of electrolysis at 3.0 A, pH = 7.05 and 2.8 L O2/min. Under these optimal conditions, the model prediction (82.44%) fits very well with the experimental response (83.90 ± 0.8%). Furthermore, chemical oxygen demand decrease was quantified. Our results illustrated significant removal efficiency for the CBZ in optimized condition with second order kinetic reaction.

Lower-normal TSH is associated with better metabolic risk factors: A cross-sectional study on Spanish men.

BACKGROUND AND AIMS: Subclinical thyroid conditions, defined by normal thyroxin (T4) but abnormal thyroid-stimulating hormone (TSH) levels, may be associated with cardiovascular and metabolic risk. More recently, TSH levels within the normal range have been suggested to be associated with metabolic syndrome and cardiovascular risk. This work studies the linearity of the relationship between metabolic syndrome and TSH across the euthyroid range. METHODS AND RESULTS: We studied 3533 male participants of the Aragon Workers' Health Study (AWHS) with normal TSH and free T4 levels, across quintiles of these variables, after adjusting for age, alcohol intake, and smoking. Compared with the lowest TSH quintile, the odds ratios for metabolic syndrome at the higher quintiles, which indicate lower thyroid function, were 1.34 (1.04, 1.73), 1.56 (1.21, 2.01), 1.57 (1.22, 2.03), and 1.71 (1.32, 2.21). The lowest free T4 quintile also showed an odds ratio of 1.49 (1.16, 1.90) with respect to the highest quintile. In addition, spline models showed departures from linearity: the risk of metabolic syndrome mostly increases at TSH values below the median (sample half-closest to subclinical hyperthyroidism). Interestingly, glucose also increases with TSH primarily below the median TSH, diastolic blood pressure shows similar changes across the entire TSH range, whereas body mass index, triglycerides, and high-density lipoprotein (HDL)-cholesterol change only at the highest normal TSH values, which are associated with lower free T4 concentration. CONCLUSIONS: TSH and free T4 within the normal range are associated with the metabolic syndrome. The sample half-below the TSH median (with probably higher functional thyroid status) exhibited better metabolic and cardiovascular profiles.

Theoretical and experimental studies of phenol oxidation by ruthenium complex with N,N,N-tris(benzimidazol-2yl-methyl)amine.

The ruthenium complex with (N,N,N-tris(benzimidazol-2yl-methyl)amine, L(1)) was prepared, and characterized. Fukui data were used to localize the reactive sites on the ligand. The structural and electronic properties of the complex were analyzed by DFT in different oxidation states in order to evaluate its oxidant properties for phenol oxidation. The results show that the hard Ru(IV) cation bonds preferentially with a hard base (Namine = amine nitrogen, or axial chloride ion), and soft Ru(II) with a soft base (Nbzim = benzimidazole nitrogen or axial triphenyl phosphine). Furthermore, the Jahn-Teller effect causes an elongation of the axial bond in the octahedral structure. The bonding nature and the orbital contribution to the electronic transitions of the complex were studied. The experimental UV-visible bands were interpreted by using TD-DFT studies. The complex oxidizes phenol to benzoquinone in the presence of H2O2 and the intermediate was detected by HPLC and (13)C NMR. A possible mechanism and rate law are proposed for the oxidation. The adduct formation of phenol with [Ru(O)L(1)](2+) or [Ru(OH)L(1)](+) is theoretically analyzed to show that [Ru(OH)L(1)-OPh](+) could produce the phenol radical.

Diagnosis of lesions of the acetabular labrum, of the labral-chondral transition zone, and of the cartilage in femoroacetabular impingement: Correlation between direct magnetic resonance arthrography and hip arthroscopy.

OBJECTIVE: To determine the sensitivity and accuracy of direct MR arthrography in the diagnosis of intra-articular lesions associated with femoroacetabular impingement. MATERIAL AND METHODS: We used direct MR arthrography to study 51 patients with femoroacetabular impingement who underwent arthroscopic hip surgery. Surgery demonstrated 37 labral tears, 44 lesions in the labral-chondral transitional zone, and 40 lesions of the articular cartilage. We correlated the findings at preoperative direct MR arthrography with those of hip arthroscopy and calculated the sensitivity, specificity, positive predictive value, negative predictive value, and validity index for direct MR arthrography. RESULTS: The sensitivity and specificity of MR arthrography were 94.5% and 100%, respectively, for diagnosing labral tears, 100% and 87.5%, respectively, for diagnosing lesions of the labral-chondral transition zone, and 92.5% and 54.5%, respectively, for diagnosing lesions of the articular cartilage. The negative predictive value of MR arthrography for lesions of the labral-chondral transitional zone was 100%. MR arthrography accurately defined extensive lesions of the cartilage and the secondary osseous changes (the main factor in poor prognosis), although its diagnostic performance was not so good in small chondral lesions. CONCLUSION: In patients with femoroacetabular impingement, direct MR arthrography can adequately detect and characterize lesions of the acetabular labrum and of the labral-chondral transitional zone as well as extensive lesions of the articular cartilage and secondary osseous changes.

Hepatitis B virus depicts a high degree of conservation during the immune-tolerant phase in familiarly transmitted chronic hepatitis B infection: deep-sequencing and phylogenetic analysis.

When intrafamilial transmission of hepatitis B virus (HBV) occurs, a virus with the same characteristics interacts with diverse hosts' immune systems and may thus result in different mutations to escape immune pressure. In this study, the HBV genomic characterization was assessed longitudinally after intrafamilial transmission using nucleotide sequence data of phylogenetic and mutational analyses, including those obtained by deep-sequencing for the first time. Furthermore, HBeAg-anti-HBe profile and variability of HBV core-derived epitopes were also evaluated. Strong evidence was obtained from intrafamilial transmission of HBV genotype D1 by phylogenetic inferences. HBV isolates exhibited high degree (~99%) of genomic conservation for almost 20 years, when patients were persistently HBeAg positive with normal amino transferase levels. This identity remained high among immune-tolerant siblings. In contrast, it diminished significantly (P = 0.02) when the mother cleared HBeAg (immune clearance phase). By deep-sequencing, the quantitative analysis of the dynamics of basal core promoter (BCP) (A1762T, G1764A; A1766C; T1773C; 8-bp deletion; and other) and precore (G1896A) variants among HBV isolates from family members exhibited differences during the follow-up. However, only those from the mother showed amino acid variations at core protein that would impair their MHC-II binding. Hence, when intrafamilial transmission occurs, HBV was highly conserved under the immune-tolerant phase, but it exhibited mutations more frequently during the immune clearance phase. The analysis of the HBV BCP and precore mutants after intrafamilial HBV transmission contributes to a better understanding of how they evolve over time.

Dolor neuropático en pacientes renales crónicos. Revisión de la literatura / Neuropathic pain in chronic kidney disease patients. Review

El dolor es un síntoma frecuente en pacientes con insuficiencia renal crónica (IRC), siendo referido por el 50 % de los mismos en forma habitual; la presentación más prevalente es el dolor osteoarticular, seguido por el neuropático, especialmente asociado a polineuropatía. Su abordaje, como así también sus complicaciones, son motivo de preocupación frecuente en los médicos tratantes. El dolor no es un síntoma aislado en dichos pacientes; habitualmente se encuentran expuestos a estresantes y presentan alteraciones emocionales como depresión o ansiedad. Actualizaciones recientes definen los síndromes funcionales dolorosos crónicos como aquellos cuadros clínicos caracterizados por dolor cotidiano y disconfort relacionado a diferentes partes del cuerpo; estas son manifestaciones habituales en estos pacientes. La teoría de analgesia multimodal plantea una opción para el manejo del dolor, en donde este es un síntoma dentro de un síndrome aún más complejo. El objetivo del presente trabajo consiste en realizar una revisión acerca de las opciones terapéuticas para tratar el dolor neuropático en pacientes con insuficiencia renal crónica (IRC) (AU)

Pain is a common symptom in patients with chronic renal failure (CRF), being reported by 50 % of such patients; the most common presentation is osteoarticular pain, followed by neuropathic pain, especially associated with polyneuropathy. Its approach, as well a sits complications are frequently a problem among physicians. Pain is not an isolated symptom in these patients, they are usually exposed to stressful and emotional alterations such as depression or anxiety. Recent updates define functional chronic pain syndromes as those diseases characterized by daily pain and discomfort related to different parts of the body, common manifestations in these patients. Multimodal analgesia theory is a choice for pain management, here pain is part of complex syndrome and not the only symptom. The aim of this study is to review therapeutic options for treating neuropathic pain in patients with chronic renal failure (CRF) (AU)

High sphingomyelin levels induce lysosomal damage and autophagy dysfunction in Niemann Pick disease type A.

Niemann Pick disease type A (NPA), which is caused by loss of function mutations in the acid sphingomyelinase (ASM) gene, is a lysosomal storage disorder leading to neurodegeneration. Yet, lysosomal dysfunction and its consequences in the disease are poorly characterized. Here we show that undegraded molecules build up in neurons of acid sphingomyelinase knockout mice and in fibroblasts from NPA patients in which autophagolysosomes accumulate. The latter is not due to alterations in autophagy initiation or autophagosome-lysosome fusion but because of inefficient autophago-lysosomal clearance. This, in turn, can be explained by lysosomal membrane permeabilization leading to cytosolic release of Cathepsin B. High sphingomyelin (SM) levels account for these effects as they can be induced in control cells on addition of the lipid and reverted on SM-lowering strategies in ASM-deficient cells. These results unveil a relevant role for SM in autophagy modulation and characterize autophagy anomalies in NPA, opening new perspectives for therapeutic interventions.

Síndrome de Allgrove (triple A). Hallazgo de una mutación no descrita en el gen AAAS / Allgrove syndrome (triple A). Finding of a mutation not described in the AAAS gene

El síndrome de Allgrove (triple A) es una enfermedad autosómica recesiva rara. La tríada clásica incluye insuficiencia adrenal congénita debida a resistencia a la ACTH, acalasia del cardias y alacrimia. Se asocia a anomalías neurológicas, como neuropatía autonómica, sensitiva y motora, sordera, retraso mental, parkinsonismo y demencia. El gen responsable es el AAAS o ADRACALIN, que codifica una proteína llamada ALADIN. Se presenta un caso de un varón de 19 años, valorado con 10 años en nuestro servicio ante la sospecha de enfermedad de depósito. Presenta retraso mental leve y del lenguaje, voz hipernasal, neuropatía sensitivo-motora con afectación autonómica y semiología de paraparesia espástica. Alacrimia. Reflujo gastroesofágico y acalasia. El estudio molecular demostró 2 mutaciones, la p. Tyr 19 Cys no descrita, y la IVS14+1G-A(AU)

Allgrove syndrome (triple A) is a rare autosomal recessive disease. The classic triad includes, congenital adrenal insufficiency due to ACTH resistance, achalasia of the cardia and alacrimia. Neurological abnormalities are associated with autonomic neuropathy, sensory and motor defects, deafness, mental retardation, Parkinsonism and dementia. The gene responsible is the ADRACALIN or AAAS encoding a protein called ALADIN. We report a case of a 19 year-old male, assessed when he was 10 years old in our department due to suspected storage disease. Mild mental and language retardation, hypernasal voice, sensory-motor neuropathy with autonomic involvement and signs of spastic paraparesis, alacrimia. gastroesophageal reflux, and achalasia. Molecular studies showed to mutations, the undescribed p.Tyr 19 Cys, and IVS14 +1 G(AU)

Characterization and description of a multiple sex chromosome system in Potamotrygon motoro (Chondrichthyes, Myliobatiformes) from the Paraná River, Argentina.

The Potamotrygonidae family represents the only freshwater group of specialized elasmobranchs and is composed of 4 genera: Plesiotrygon, Paratrygon, Potamotrygon, and Heliotrygon. Individuals of Potamotrygon motoro were collected from the Paraná River at Ituzaingó (Province of Corrientes) in Argentina. Mitotic preparations were obtained using direct techniques and lymphocyte cultures. Chromosomes were studied with conventional Giemsa, C-band and nucleolus organizer region stainings. The diploid number and karyotype formulae were 2n = 66 with 33 homomorphic pairs (14M + 16SM + 22ST + 14A) in females and 2n = 65 with 31 homomorphic pairs plus 3 heteromorphic chromosomes (13M + 15SM + 23ST + 14A) in males. Heterochromatin was distributed at the pericentromeric regions of almost all chromosomes and in the telomeric and interstitial positions of some chromosomes. nucleolus organizer regions were multiple and located at telomeric regions. Because of the clear karyotype differences between the 2 sexes and the presence of 3 unpaired chromosomes in males, it is possible to suggest the occurrence of a multiple sex chromosome system, with male heterogamety (X1X1X2X2/X1X2Y). Further cytogenetic studies are required to develop a hypothesis about the origin of the multiple sex chromosome system in P. motoro.

[Allgrove syndrome (triple A). Finding of a mutation not described in the AAAS gene]. / Síndrome de Allgrove (triple A). Hallazgo de una mutación no descrita en el gen AAAS.

Allgrove syndrome (triple A) is a rare autosomal recessive disease. The classic triad includes, congenital adrenal insufficiency due to ACTH resistance, achalasia of the cardia and alacrimia. Neurological abnormalities are associated with autonomic neuropathy, sensory and motor defects, deafness, mental retardation, Parkinsonism and dementia. The gene responsible is the ADRACALIN or AAAS encoding a protein called ALADIN. We report a case of a 19 year-old male, assessed when he was 10 years old in our department due to suspected storage disease. Mild mental and language retardation, hypernasal voice, sensory-motor neuropathy with autonomic involvement and signs of spastic paraparesis, alacrimia. gastroesophageal reflux, and achalasia. Molecular studies showed to mutations, the undescribed p.Tyr 19 Cys, and IVS14 +1 G.

[Questionnaire on the anaesthesiology treatment of patients subjected to posterior fossa neurosurgery]. / Encuesta sobre el tratamiento anestesiológico de los pacientes sometidos a neurocirugía de fosa posterior.

OBJECTIVES: To find out, by means of a questionnaire, the procedures used by Spanish anaesthetists in peri-operative management of patients subjected to neurosurgery of the posterior cranial fossa. MATERIAL AND METHODS: A closed-question type questionnaire was sent to Anaesthesiology Departments with a Neurosurgery Department on the participation of anaesthetists in the peri-operative treatment of patients subjected posterior fossa surgery. RESULTS: The questionnaire was completed by 42 (57.5%) of the 73 national public hospitals with a Neurosurgery Department. The posterior fossa surgery was performed in the sitting position in 36 hospitals, although it was less frequently used than the lateral decubitus or prone decubitus position. There was little specific neurological monitoring, as well as little use of precordial and/or transcranial Doppler for detecting vascular air embolism. Nitrous oxide was used in less than 10% of the centres, and 15% avoided neuromuscular block when neurophysiological monitoring was used during the surgery. Cardiovascular problems were mentioned as being the most frequent in 29% of the centres, while in the post-operative period the most common complications were, cranial nerve déficit, airway oedema (23%), and post-operative vomiting (47%). CONCLUSIONS: The results obtained from the questionnaire showed that the sitting position was less used than the prone position in posterior fossa surgery, and that neurophysiological monitoring is during surgery is hardly used.

Comorbilidad, discapacidad y mortalidad en pacientes pluripatológicos con enfermedad pulmonar obstructiva crónica / Comorbidity, discapacity and mortality in patients with multiple conditions and chronic obstructive pulmonary disease

Objetivo. Estudiar la asociación entre diferentes comorbilidades y condiciones clínicas con la mortalidad en pacientes pluripatológicos (PPP) con enfermedad pulmonar obstructiva crónica (EPOC). Pacientes y métodos Estudio observacional, prospectivo y multicéntrico. Se han incluido pacientes consecutivos con EPOC y criterios de PPP. Se han recogido las variables: edad, sexo, índice de Charlson, comorbilidades, índice de Barthel, índice de Lawton-Brody, test de Pfeiffer, escala de Gijón, nivel de educación, hospitalizaciones en los últimos 3 y 12 meses y supervivencia al cabo de un año. Mediante un análisis univariante y una regresión logística se ha establecido la relación de las variables con la mortalidad. Resultados. Se han incluido 688 PPP con EPOC con edad media 77,9 años. La puntuación media (DE) en el índice de Charlson fue 3,99 (2,07). Las comorbilidades más frecuentes son insuficiencia cardiaca (59%), diabetes (48%), infarto de miocardio (29,4%), insuficiencia renal moderada (22%), enfermedad cerebrovascular (19%), hipertensión arterial (71%), anemia (62%), fibrilación auricular (34%), dislipemia (28%) y obesidad (21%). El 26% tenía dependencia para las actividades básicas de la vida diaria, el 47% precisaba de un cuidador y el 54% tenía riesgo de problema social. Al cabo de un año fallecieron 258 pacientes (37%). El índice de Charlson, la dependencia para las actividades de la vida diaria y la anemia se asocian con una mayor mortalidad y la hipertensión arterial y saber leer y escribir con menor mortalidad. Conclusiones. La comorbilidad y la discapacidad son dos factores pronósticos en los pacientes con EPOC(AU)

Aims. To determine the association between different comorbidities and other clinical conditions with mortality in patients with multiple diseases (PMD) suffering from chronic obstructive pulmonary disease (COPD). Subjects and methods. Patients with COPD and PMD criteria were included in an observational, prospective and multicentrer study. Data on age, gender, Charlson index, Barthel index, Lawton-Brody index, Pfeiffer test, sociofamilial Gijon scale, education level, hospitalizations during the previous 3 and 12 months and survival at one year were collected. The relationship between the variables and mortality were established by means of a univariate analysis and logistic regression model. Results. A total of 688 PMD with COPD and mean age of 77.9 years were included. The mean score one the Charlson index was 3.99 (2.07). The most frequent comorbidities were heart failure (59%), diabetes (48%), myocardial infarction (29%), moderate kidney failure (22%), cerebrovascular disease (19%), hypertension (71%), anemia (62%), atrial fibrillation (34%), dyslipidemia (28%) and obesity (21%). A total of 26% of patients were dependent for activities of daily living, 47% needed a caregiver and 54% were at risk of having social problems. At one year, 258 patients (37%) had died. The Charlson index, dependency for activities of daily living and anemia were associated with mortality and hypertension and capacity for reading and writing were associated with survival(AU)