Manejo anestésico del déficit de glucosa 6-fosfato deshidrogenasa / Anesthetic management of glucose 6-phosphate dehydrogenase deficiency

El déficit de glucosa 6-fosfato deshidrogenasa es la enzimopatía más frecuente de los glóbulos rojos, contando con una prevalencia de 400 millones de personas en el mundo y una herencia ligada al cromosoma X, afectando sobre todo a varones. La glucosa 6-fosfato-deshidrogenasa participa en la vía de las pentosas-fosfato, encargada del metabolismo celular, y produciendo antioxidantes. Un déficit de esta enzima altera su capacidad de proteger a los hematíes del estrés oxidativo que producen ciertos medicamentos, condiciones metabólicas, infecciones y alimentos. Se requieren consideraciones anestésicas específicas para disminuir la morbimortalidad asociada a intervenciones médico-quirúrgicas en pacientes con esta enfermedad. En este artículo se expone el caso de un varón de 45 años con este déficit enzimático sometido a anestesia general combinada para resección anterior baja de recto programada.(AU)

Glucose 6-phosphate dehydrogenase deficiency is the most common enzyme disease of red blood cells, with around 400 million people suffering from it throughout the world and linked to the X chromosome inheritance, thus it predominantly affects men. Glucose 6-phosphate-dehydrogenase participates in the pentose-phosphate pathway, being responsible for cellular metabolism and the production of antioxidants. A deficiency of this enzyme alters its ability to protect red blood cells from oxidative stress caused by certain drugs, metabolic conditions, infections and food. Specific anesthetic considerations are required to reduce the morbidity and mortality associated with medical-surgical interventions in patients with this disease. This article presents the case of a 45-year-old man with Glucose 6-Phosphate Dehydrogenase deficiency who underwent combined general anesthesia for programmed low anterior resection of the rectum.(AU)

Anaesthetic implications for Pompe disease. A case description.

Pompe disease, or type II glycogenosis, is a rare metabolic myopathy inherited in an autosomal recessive pattern, characterized by progressive muscle weakness and multisystem involvement. The disease often results in premature death. Patients with Pompe disease are at high risk for anaesthesia-related complications, particularly cardiac and respiratory problems, although difficult airway management is the greatest complication. It is essential to perform a comprehensive preoperative study in order to reduce the risk of perioperative morbidity and mortality, and to obtain as much information as possible for the surgical procedure. In this article, we report the case of a patient with a history of adult Pompe disease who underwent combined anaesthesia for osteosynthesis of the proximal end of the left humerus.

Anesthetic management of glucose 6-phosphate dehydrogenase deficiency.

Glucose 6-phosphate dehydrogenase deficiency is the most common enzyme disease of red blood cells, with around 400 million people suffering from it throughout the world and linked to the X chromosome inheritance, thus it predominantly affects men. Glucose 6-phosphate-dehydrogenase participates in the pentose-phosphate pathway, being responsible for cellular metabolism and the production of antioxidants. A deficiency of this enzyme alters its ability to protect red blood cells from oxidative stress caused by certain drugs, metabolic conditions, infections and food. Specific anesthetic considerations are required to reduce the morbidity and mortality associated with medical-surgical interventions in patients with this disease. This article presents the case of a 45-year-old man with glucose 6-phosphate dehydrogenase deficiency who underwent combined general anaesthesia for programmed low anterior resection of the rectum.

Implicaciones anestésicas en la enfermedad de Pompe. Descripción de un caso / Anaesthetic implications for Pompe disease. A case description

La enfermedad de Pompe o glucogenosis tipo ii es una miopatía metabólica rara, de herencia autosómica recesiva, que se caracteriza por debilidad muscular progresiva y afectación multisistémica, acompañada, habitualmente, de muerte temprana. Los pacientes con esta enfermedad presentan alto riesgo anestésico en relación con problemas tanto de origen cardiaco como respiratorio, aunque las mayores complicaciones son las derivadas del manejo de la vía aérea, potencialmente dificultoso. Un buen estudio preoperatorio es fundamental para disminuir la morbimortalidad perioperatoria, optimizando y aportando la mayor información posible de cara a la intervención quirúrgica. En este artículo se expone el caso de un paciente con antecedentes de enfermedad de Pompe del adulto sometido a anestesia combinada para osteosíntesis de extremo proximal de húmero izquierdo.(AU)

Pompe disease, or type ii glycogenosis, is a rare metabolic myopathy inherited in an autosomal recessive pattern, characterized by progressive muscle weakness and multisystem involvement. The disease often results in premature death. Patients with Pompe disease are at high risk for anaesthesia-related complications, particularly cardiac and respiratory problems, although difficult airway management is the greatest complication. It is essential to perform a comprehensive preoperative study in order to reduce the risk of perioperative morbidity and mortality, and to obtain as much information as possible for the surgical procedure. In this article, we report the case of a patient with a history of adult Pompe disease who underwent combined anaesthesia for osteosynthesis of the proximal end of the left humerus.(AU)

Patient profiles as an aim to optimize selection in the second line setting: the role of aflibercept.

Colorectal cancer is the second leading cause of cancer-related death worldwide. For metastatic colorectal cancer (mCRC) patients, it is recommended, as first-line treatment, chemotherapy (CT) based on doublet cytotoxic combinations of fluorouracil, leucovorin, and irinotecan (FOLFIRI) and fluorouracil, leucovorin, and oxaliplatin (FOLFOX). In addition to CT, biological (targeted agents) are indicated in the first-line treatment, unless contraindicated. In this context, most of mCRC patients are likely to progress and to change from first line to second line treatment when they develop resistance to first-line treatment options. It is in this second line setting where Aflibercept offers an alternative and effective therapeutic option, thought its specific mechanism of action for different patient's profile: RAS mutant, RAS wild-type (wt), BRAF mutant, potentially resectable and elderly patients. In this paper, a panel of experienced oncologists specialized in the management of mCRC experts have reviewed and selected scientific evidence focused on Aflibercept as an alternative treatment.

Manejo clínico de la neoplasia trofoblástica gestacional: presentación de un caso / Clinical management of gestational trophoblastic neoplasia: presentation of a case

La enfermedad trofoblástica gestacional es una entidad poco frecuente que se produce por una proliferación anormal de la placenta. Engloba un diverso espectro de entidades histológicas, que conllevan a su vez diversas implicaciones clínicas. Unas son de carácter benigno (mola parcial y mola completa, placentomegalia, nódulo del sitio placentario) y otras de carácter maligno, estas últimas reciben en común la denominación de Neoplasia Tofoblástica Gestacional (NTG) y tienen un alto potencial de metastatización. Forman parte de las NTG: la mola invasiva, el tumor trofoblástico del sitio placentario, el tumor trofoblástico epitelioide y el coriocarcinoma gestacional. Lo más común es que la NTG debute tras la aparición de una gestación molar, pero también es posible que ocurra tras otro tipo de evento obstétrico como una gestación a término, o una gestación ectópica. Es pues de vital importancia realizar un correcto seguimiento tras evacuar una gestación molar, realizando una monitorización de los valores séricos de la BhcG y sospechando una enfermedad trofoblástica persistente ante los supuestos que posteriormente describiremos.La principal herramienta terapéutica para la NTG es el uso de la quimioterapia, aunque también se puede optar por la cirugía endeterminados casos. Habrá que valorar de modo individualizado en función de la histología, score pronóstico y deseos genésicos futuros de la paciente. Afortunadamente, la tasa de supervivencia y de curación de la NTG con un tratamiento y seguimiento adecuado es muy elevada. (AU)

Gestational trophoblastic disease is a rare entity that is caused by an abnormal proliferation of the placenta. It encompasses adiverse spectrum of histological entities, which carry various clinical implications. Some of them are benign (partial mole and complete mole, placentomegaly, placental site nodule) and others of a malignant nature, which are known as Gestational TrophoblasticNeoplasia (GTN) and have a high potential for metastasization. Are part of the GTN: invasive mole, trophoblastic tumor of theplacental site, trophoblastic tumor epithelioid and gestational choriocarcinoma. The most common is that NTG debuts after theappearance of a molar gestation, but it also may occur after another type of obstetric event such as a term gestation, or an ectopicgestation. It is therefore of vital importance to carry out a correct follow-up after evacuating a molar gestation, monitoring the serumvalues of BhcG and suspecting a persistent trophoblastic disease in the event that we will later describe. The main therapeutic toolfor NTG is the use of chemotherapy, although surgery can also be chosen in certain cases. It will be necessary to assess individuallyaccording to histology, prognostic score and future genetic desires of the patient. Fortunately, the survival and cure rate of NTG with proper treatment and follow-up is very high. (AU)

Phase separation enhanced magneto-electric coupling in La0.7Ca0.3MnO3/BaTiO3 ultra-thin films.

We study the origin of the magnetoelectric coupling in manganite films on ferroelectric substrates. We find large magnetoelectric coupling in La0.7Ca0.3MnO3/BaTiO3 ultra-thin films in experiments based on the converse magnetoelectric effect. The magnetization changes by around 30-40% upon applying electric fields on the order of 1 kV/cm to the BaTiO3 substrate, corresponding to magnetoelectric coupling constants on the order of α = (2-5) · 10(-7) s/m. Magnetic anisotropy is also affected by the electric field induced strain, resulting in a considerable reduction of coercive fields. We compare the magnetoelectric effect in pre-poled and unpoled BaTiO3 substrates. Polarized neutron reflectometry reveals a two-layer behavior with a depressed magnetic layer of around 30 Å at the interface. Magnetic force microscopy (MFM) shows a granular magnetic structure of the La0.7Ca0.3MnO3. The magnetic granularity of the La0.7Ca0.3MnO3 film and the robust magnetoelastic coupling at the La0.7Ca0.3MnO3/BaTiO3 interface are at the origin of the large magnetoelectric coupling, which is enhanced by phase separation in the manganite.

The effectiveness of a program of physical activity and diet to modify cardiovascular risk factors in patients with severe mental illness after 3-month follow-up: CAPiCOR randomized clinical trial.

BACKGROUND: The aim of this randomized clinical trial follow-up at three months was to evaluate the effectiveness of an educational intervention with a focus on diet and physical activity (PA) to change the amount of PA, body mass index (BMI) and the waist circumference (WC) in patients with severe mental illness. METHODS: We recruited 332 outpatients with severe mental disorders undergoing treatment with antipsychotic medication from Mental Healthcare Centers of Barcelona. They were randomly assigned to an intervention or a control group. The patients in the intervention group participated in a group PA and diet educational program. The blinded measurements at 0 and 3 months were: the level of PA (IPAQ questionnaire), BMI, WC, blood pressure, dietary habits (PREDIMED questionnaire), quality of life (SF-36 questionnaire) and laboratory parameters (cholesterol, triglycerides, glucose). RESULTS: The average age was 46.7 years and 55% were males. Schizophrenia had been diagnosed in 67.1% of them. At 3 months, the average weekly walking METs rose significantly in the IG 266.05 METs (95%CI: 16.86 to 515.25; P=0.036). The total MET average also rose although not significantly: 191.38 METs (95%CI: 1.38 to 381.38; P=0.086). However, the BMI decreased significantly more in the CG, by 0.26kg/m(2) (95%CI: 0.02 to 0.51; P=0.038), than in the IG. There were no significant differences in the WC. CONCLUSIONS: The short-term results suggest that the intervention increases the level of PA, but does not improve physical or laboratory parameters. TRIAL REGISTRATION: Clinicaltrials.gov NCT01729650 (effectiveness of a physical activity and diet program in patients with psychotic disorder [CAPiCOR]).

17ß Estradiol Modulates Perfusion Pressure and Expression of 5-LOX and CYP450 4A in the Isolated Kidney of Metabolic Syndrome Female Rats.

Prevalence of metabolic syndrome and progression of nephropathy depend on sex. We examined a protective effect of estradiol against nephropathy in metabolic syndrome through the modulation of the arachidonic acid metabolism by activating the 5-lipoxygenase and cytochrome p450 4A pathways. 28 female Wistar rats were divided into four groups of seven animals each: control, intact metabolic syndrome, ovariectomized metabolic syndrome, and metabolic syndrome ovariectomized plus estradiol. Blood pressure, body weight, body fat, triglycerides, insulin, HOMA-index, albuminuria, and TNF-α were increased in ovariectomized metabolic syndrome rats (p < 0.001). The perfusion pressure in isolated kidneys of ovariectomized metabolic syndrome rats in presence of 4 µg of arachidonic acid was increased. The inhibitors of the arachidonic acid metabolism Baicalein, Miconazole, and Indomethacin in these rats decreased the perfusion pressure by 57.62%, 99.83%, and 108.5%, respectively and they decreased creatinine clearance and the arachidonic acid percentage. Phospholipase A2 expression in the kidney of ovariectomized metabolic syndrome rats was not modified. 5-lipoxygenase was increased in metabolic syndrome ovariectomized rats while cytochrome p450 4A was decreased. In conclusion, the loss of estradiol increases renal damage while the treatment with estradiol benefits renal function by modulating arachidonic acid metabolism through the 5-lipoxygenase and cytochrome p450 4A pathways.

Polyphenolic compounds as chemical markers of wine ageing in contact with cherry, chestnut, false acacia, ash and oak wood.

The nonanthocyanic phenolic composition of four red wines, one white, and one rosé aged using barrels and chips of cherry, chestnut, false acacia, ash and oak wood was studied by LC-DAD-ESI/MS, to identify the phenolic compounds that woods other than oak contribute to wines, and if some of them can be used as chemical markers of ageing with them. A total of 68 nonanthocyanic phenolic compounds were identified, 15 found only in wines aged with acacia wood, 6 with cherry wood, and 1 with chestnut wood. Thus, the nonanthocyanic phenolic profile could be a useful tool to identify wines aged in contact with these woods. In addition, some differences in the nonanthocyanic phenolic composition of wines were detected related to both the levels of compounds provided by each wood species and the different evolution of flavonols and flavanols in wines during ageing in barrels or in contact with chips.

Volatile compounds and sensorial characterisation of red wine aged in cherry, chestnut, false acacia, ash and oak wood barrels.

The wood-related volatile profile of wines aged in cherry, acacia, ash, chestnut and oak wood barrels was studied by GC-MS, and could be a useful tool to identify the wood specie used. Thus, 2,4-dihydroxybenzaldehyde in wines aged in acacia barrels, and ethyl-2-benzoate in cherry barrels could be used as chemical markers of these wood species, for authenticity purposes. Also, the quantitative differences obtained in the volatile profiles allow a good classification of all wines regarding wood species of barrels, during all aging time, and they contributed with different intensities to aromatic and gustative characteristics of aged wines. Wines aged in oak were the best valuated during all aging time, but the differences were not always significant. The lowest scores were assigned to wines aged in cherry barrels from 6 months of aging, so this wood could be more suitable in short aging times.

[Quantitative and qualitative evaluation of the interim PET/CT in lymphoma treatment in the prediction of complete metabolic response]. / Evaluación cuantitativa y cualitativa de la PET/TC a mitad del tratamiento en linfomas en la predicción de respuesta metabólica completa.

OBJECTIVE: To compare two different methods for the interpretation of interim PET/CT (PET/CT-i) in lymphomas, and to establish which one best predicts a complete metabolic response (CMR) in the PET/CT study at the end of treatment (PET/CT-et). MATERIAL AND METHODS: Retrospective longitudinal analysis of the PET/CT studies for staging (PET/CT-s), PET/CT-i and PET/CT-et of 65 patients, 35 Hodgkin's lymphoma (HL) and 30 Non-HL. The PET/CT-i was performed between the second and fourth chemotherapy cycle. It was interpreted using two different criteria: qualitative criteria (5 point visual scale), semiquantitative criteria (percentage difference between the lesion with more SUVmax in the PET/CT-s and PET/CT-i). We analyzed the likelihood of obtaining a CMR in the PET/CT-et according to the results obtained on the PET/CT-i with these two criteria. RESULTS: We obtained sensitivity (S), specificity (Sp), positive predictive values (PPV), negative predictive values (NPV) and likelihood ratio (LR) for the qualitative/semiquantitative method of 91%/80%, 76.2%/67%, 88.9%/83.3%, 80%/60.9% and 32%/7.8%, respectively, to predict a CMR in the PET/CT-et. There were no statistically significant differences between the LR of both methods (p=0.1942). CONCLUSION: We found clear differences in S, Sp, PPV and NPV between both interpretation criteria for the PET/CT-i to predict a CMR in the PET/CT-et. Nevertheless, we cannot confirm the superiority of the qualitative method over the semiqualitative method for this purpose as no statistically significance differences were found in their LR in our study.

Derrame pericárdico postparto, toxicidad pleuropulmonar precoz asociada a Carbegolina / Pericardia effusion in a patient during the puerperium secundary to treatment with Cabergoline

La inhibición de la lactancia materna en el puerperio inmediato se realiza habitualmente con cabergolina, 1mgr en dosis única. La carbegolina es un agonista dopaminérgico selectivo de acción prolongada. Inhibe la síntesis de prolactina por estimulación directa de receptores D2 de los lactotrofos pituitarios de modo dosis dependiente, siendo la mayoría de sus reacciones adversas leves. Los efectos secundarios graves, y poco frecuentes, se asocian principalmente con trastornos pleuropulmonares, cardiacos y valvulopatías. Estas raras complicaciones se asocian más a pacientes con lesiones leves fibróticas de válvulas cardíacas y en tratamientos a largo plazo como en el caso de tratamiento enfermedad de Parkinson y de disfunciones hiperprolactinemicas (AU)

In order to inhibit lactation in women during the puerperal phase, a single, oral dose of cabergoline is administered. The cabergoline is a long-acting selective dopamine agonist. It inhibits the prolactin synthesis by direct stimulation of D2 receptor in pituitary lactotrophs in a dose dependent. Most of its adverse reactions are slight. Most commonly, this treatment causes only mild side effects, however, cabergoline can also cause severe side effects including valvulopathy and cardiopulmonary fibrotic reactions. These severe adverse reactions are predominantly observed in patients which are given high daily doses of the drug over a prolonged period of time, as is usual in the treatment of illnesses such as hyperprolactinaemia or Parkinson's disease. We present a case of pericardial effusion and bilateral pleural effusion in a patient during the puerperium, after being treated with two single low-dose cabergoline tablets to inhibit lactation (AU)

Fibromialgia y trastornos de personalidad / Fibromyalgia and personality disorders

Introducción. El objetivo es detectar, en atención primaria, posibles alteraciones de personalidad en pacientes con fibromialgia, que indiquen la valoración por el servicio de salud mental. Material y métodos. Estudio descriptivo transversal, en un ámbito urbano de atención primaria. Población de estudio: la totalidad de personas diagnosticadas de fibromialgia registradas en historia clínica informatizada, mayores de 18 años. Cumplieron criterios de selección 132 casos. Se recogieron variables sociodemográficas y clínicas y se administró el test Internacional Personality Disorder Examination screening (IPDE-s). Resultados. El 100% de los pacientes eran mujeres, con edad media de 54 años. Casadas/en pareja el 78,5%, no trabajaban el 89,2% y pertenecían a clase social baja el 58,7%. Refirieron enfermedad mental el 73,6% de casos, siendo este diagnóstico previo al de fibromialgia en un 55,1%. El diagnóstico psiquiátrico más frecuente fue el trastorno de ánimo (86,3%). La autopercepción de salud fue regular-mala en el 87,6% y la expectativa de curación favorable en solo un 18,2%. Resultados. El IPDE-s fue positivo en el 96,7% de casos; en el 94,2% además, a más de un rasgo de personalidad. Rasgos más prevalentes: límite 71,9%, obsesivo 71,1%, histriónico 66,1% y evitación 61,9%. Se encontró relación significativa entre padecer patología psiquiátrica y rasgo de personalidad límite o dependiente (p<0,006; p<0,03 respectivamente); así como entre una peor percepción de salud y rasgo dependiente (p<0,004). Conclusiones. Se detecta una alta sospecha de rasgos de personalidad anómalos en pacientes diagnosticadas de fibromialgia, que justificaría la derivación para un estudio/seguimiento más completo por salud mental (AU)

Introduction. The aim is to detect possible personality disorders in subjects with fibromyalgia in a Primary Care centre who have been assessed by the Mental Health Department. Material and methods. A cross-sectional descriptive study in an urban Primary Care centre. All patient diagnosed with fibromyalgia with a computerised clinical history, and over 18 years were included. A total 132 cases fulfilled the selection criteria. The main measurements were social, demographic and clinical variables, and the International Personality Disorder Screening Examination (IPDE-s). Results. All the cases (100%) were women and the average age was 54 years. Married or in couple (78.5%), did not work (89.2%) and from a low social class (58.7%). Mental illness was detected in 73.6% of cases, and in 55.1% of them its diagnosis was prior to fibromyalgia. Mood disorders (86.3%) were the most frequent diagnosis. Self perception of health was regular-bad in 87.6% of cases and the healing expectation 18.2%. Results. The IPDE-s was positive in 96.7% of the sample and more than one personality disorder detected in 94.2%. The more frequent personality disorders were: histrionic 71.9%, obsessive 71.1%, emotionally unstable 66.1%, and avoidance 61.9%. Significant relationships were found between suffering psychiatric disease and having personality traits of unstable emotions or dependency (P<0.006; p<0.03 respectively). As well between a self-perception of poorer health and dependent personality (P<0.004). Conclusions. The high rate of abnormal personality disorders in patients with fibromyalgia requires a more comprehensive study and holistic approach (AU)

Penile incarceration secondary to a ring.

Penile incarceration by foreign objects for sexual stimulation purposes is a situation described in universal literature, however it is a rare situation that the urologist must face in the emergency room. A 75-year-old male is presented to whom his sexual partner had placed a ring in the coronal sulcus.