Chronic Elevation of Liver Enzymes in Acute Intermittent Porphyria Initially Misdiagnosed as Autoimmune Hepatitis

Autoimmune hepatitis is a disease characterized by an elevation of liver enzymes, as well as specific autoantibodies. It is more common in women than men. We describe a 32-year-old woman with elevated transaminases, autoantibodies, and a liver biopsy result suggestive of autoimmune hepatitis. The indicated treatment was administered without showing a satisfactory response. The patient had a family history of acute intermittent porphyria (AIP) so we decided to begin treatment with hematin, achieving a complete remission of the symptoms. Acute intermittent porphyria is a rare condition characterized by neurovisceral symptoms, abdominal pain being the most common of them. The disease has a higher prevalence among young women and certain European countries such as Sweden, Great Britain, and Spain. A correct diagnosis and prompt treatment are essential because patients affected by AIP must have a strict followup due to the fatal outcome of the outbreaks

Role of the Renin-Angiotensin System and Aldosterone on Cardiometabolic Syndrome

Aldosterone facilitates cardiovascular damage by increasing blood pressure and through different mechanisms that are independent of its effects on blood pressure. In this respect, recent evidence involves aldosterone in the pathogenesis of metabolic syndrome. Although this relationship is complex, there is some evidence suggesting that different factors may play an important role, such as insulin resistance, renin-angiotensin-aldosterone system, oxidative stress, sodium retention, increased sympathetic activity, levels of free fatty acids, or inflammatory cytokines and adipokines. In addition to the classical pathway by which aldosterone acts through the mineralocorticoid receptors leading to sodium retention, aldosterone also has other mechanisms that influence cardiovascular tissue remodelling. Finally, overweight and obesity promote the adrenal secretion of aldosterone, increasing the predisposition to type 2 diabetes mellitus. Further studies are needed to better establish therapeutic strategies that act on the blockade of mineralocorticoid receptor in the treatment and prevention of cardiovascular diseases related to the excess of aldosterone and the metabolic syndrome

A systematic review of the adherence to home-practice meditation exercises in patients with chronic pain

Mindfulness-, compassion-, and acceptance-based (i.e., “third wave”) psychotherapies are effective for treating chronic pain conditions. Many of these programs require that patients engage in the systematic home practice of meditation experiences so they can develop meditation skills. This systematic review aimed at evaluating the frequency, duration, and effects of home practice in patients with chronic pain undergoing a “third wave” psychotherapy. A comprehensive database search for quantitative studies was conducted in PubMed, Embase, and Web of Sciences Core Collection; 31 studies fulfilled the inclusion criteria. The reviewed studies tended to indicate a pattern of moderately frequent practice (around four days/week), with very high variability in terms of time invested; most studies observed significant associations between the amount of practice and positive health outcomes. Mindfulness-Based Stress Reduction and Mindfulness-Based Cognitive Therapy were the most common interventions and presented low levels of adherence to home practice (39.6% of the recommended time). Some studies were conducted on samples of adolescents, who practiced very few minutes, and a few tested eHealth interventions with heterogeneous adherence levels. In conclusion, some adaptations may be required so that patients with chronic pain can engage more easily and, thus, effectively in home meditation practices

Stochastic resonance between dissipative structures in a bistable noise-sustained dynamics

We study an extended system that without noise shows a monostable dynamics, but when submitted to an adequate multiplicative noise, an effective bistable dynamics arise. The stochastic resonance between the attractors of the \textit{noise-sustained dynamics} is investigated theoretically in terms of a two-state approximation. The knowledge of the exact nonequilibrium potential allows us to obtain the output signal-to-noise ratio. Its maximum is predicted in the symmetric case for which both attractors have the same nonequilibrium potential value.Comment: RevTex, 13 pages, 6 figures, accepted in Physical Review

Clasificación de los diferentes modelos de Aula invertida y su aplicación en la Universidad Politécnica de Madrid

El método de enseñanza del Aula Invertida ha cobrado relevancia, en los últimos años, debido a los beneficios obtenidos en su aplicación en las enseñanzas universitarias. Con este método de aprendizaje activo se invierte, con respecto al modelo tradicional, los lugares donde se recibe la clase teórica y la clase práctica. Este método teórico simple, en la práctica, puede complicarse por las variaciones que pueden realizarse, por ejemplo, en las herramientas aplicadas, en la existencia o ausencia de refuerzos o en la evaluación de los conocimientos adquiridos. Se ha podido constatar la gran variedad existente en la aplicación del Aula Invertida al estudiar los proyectos de innovación educativa en la Universidad Politécnica de Madrid durante el curso 2017-18. Por ello, se ha realizado una clasificación de todos estos proyectos cuyo resultado indica que el modelo más aplicado ha sido el que sólo define el desarrollo teórico, sin llegar a ponerlo en práctica. Además, se han definido unos parámetros para definir las características que tiene cada proyecto realizado en dicha universidad. El estudio estadístico realizado indica que, aparentemente, no existen unas características similares en los proyectos clasificados dentro del mismo tipo de modelo de Aula Invertida

Clinical features and health-related quality of life in adult patients with mucopolysaccharidosis IVA: the Spanish experience

Background: Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is a progressive and disabling disease characterized by a deficiency of the enzyme N-acetylgalactosamine-6-sulphate sulphatase. Its clinical presentation is very heterogeneous and poorly understood in adults. The aim of this study was to describe the clinical manifestations of MPS IVA in adult patients in Spain and to assess their health-related quality of life (HRQoL). Results: Thirty-three patients from nine reference centres participated in the study. The median age was 32 (interquartile range [IQR]: 20.5–40.5) years. The phenotype was classical in 54.5% of patients, intermediate in 33.3% of patients, and non-classical in 12.1% of patients. The most common clinical manifestation was bone dysplasia, with a median height of 118 (IQR: 106–136) cm. Other frequent clinical manifestations were hearing loss (75.7%), ligamentous laxity (72.7%), odontoid dysplasia (69.7%), limb deformities that required orthopaedic aids (mainly hip dysplasia and genu valgus) (63.6%), and corneal clouding (60.6%). In addition, 36.0% of patients had obstructive sleep apnoea/hypopnoea syndrome and 33.3% needed non-invasive ventilation. Cervical surgery and varisation osteotomy were the most common surgical interventions (36.4% each). Almost 80% of patients had mobility problems and 36.4% used a wheelchair at all times. Furthermore, 87.9% needed help with self-care, 33.3% were fully dependent, and 78.8% had some degree of pain. HRQoL according to the health assessment questionnaire was 1.43 (IQR: 1.03–2.00) in patients with the non-classical phenotype, but 2.5 (IQR: 1.68–3.00) in those with the classical phenotype. Seven patients were initiated on enzyme replacement therapy (ERT), but two of them were lost to follow-up. Lung function improved in four patients and slightly worsened in one patient. The distance achieved in the six-minute walk test increased in the four patients who could perform it. HRQoL was better in patients treated with elosulfase alfa, with a median (IQR) of 1.75 (1.25–2.34) versus 2.25 (1.62–3.00) in patients not treated with ERT. Conclusions: The study provides real-world data on patients with MPS IVA. Limited mobility, difficulties with self-care, dependence, and pain were common, together with poor HRQoL. The severity and heterogeneity of clinical manifestations require the combined efforts of multidisciplinary teams

Performance evaluation of reverse osmosis (RO) pre-treatment technologies for in-land brackish water treatment

Integration of renewable energy with desalination technologies has emerged as an attractive solution to augment fresh water supply sustainably. Fouling and scaling are still considered as limiting factors in membrane desalination processes. For brackish water treatment, pre-treatment of reverse osmosis (RO) feed water is a key step in designing RO plants avoiding membrane fouling. This study aims to compare at pilot scale the rejection efficiency of RO membranes with multiple pre-treatment options at different water recoveries (30, 35, 40, 45 and 50%) and TDS concentrations (3500, 4000, and 4500mg/L). Synthetic brackish water was prepared and performance evaluation were carried out using brackish water reverse osmosis (BWRO) membranes (Filmtec LC-LE-4040 and Hydranautics CPA5-LD-4040) preceded by 5 and 1μm cartridge filters, 0.02μm ultra-filtration (UF) membrane, and forward osmosis (FO) membrane using 0.25M NaCl and MgCl2 as draw solutions (DS). It was revealed that FO membrane with 0.25M MgCl2 used as a draw solution (DS) and Ultra-filtration (UF) membrane followed by Filmtec membrane gave overall 98% rejection but UF facing high fouling potential due to high applied pressure. Use of 5 and 1μm cartridge filter prior to Filmtec membrane also showed effective results with 95% salt rejection

Coral Reef Degradation Differentially Alters Feeding Ecology of Co-occurring Congeneric Spiny Lobsters

Caribbean coral reefs are undergoing massive degradation, with local increases of macroalgae and reduction of architectural complexity associated with loss of reef-building corals. We explored whether reef degradation affects the feeding ecology of two co-occurring spiny lobsters: Panulirus guttatus, which is an obligate reef-dweller, and Panulirus argus, which uses various benthic habitats including coral reefs. We collected lobsters of both species from the back-reef zones of two large reefs similar in length (∼1.5 km) but differing widely in level of degradation, at the Puerto Morelos Reef National Park (Mexico). We measured the carapace length (CL) and weight (W) of lobsters, estimated three condition indices (hepatosomatic index, HI; blood refractive index, BRI; and W/CL ratio), and analyzed their stomach contents and stable isotope values (δ15N and δ13C). All lobsters tested negative for the presence of the virus PaV1, which can affect nutritional condition. Stomach contents yielded 72 animal taxa, mainly mollusks and crustaceans, with an average of 35 taxa per species per reef, but with much overlap. In P. guttatus, CL, HI, BRI, and W/CL did not vary with reef, but mean isotopic values did. The isotopic niche of P. guttatus showed little overlap between reefs, reflecting differences in local carbon sources and underlining the habitat specialization of P. guttatus, which exhibited a higher trophic position on the more degraded reef. Overall, the trophic position of P. guttatus was higher than that of P. argus. In P. argus, none of the variables differed between reefs and the isotopic niche was wide and with great overlap between reefs, reflecting the broader foraging ranges of P. argus compared to P. guttatus. Additional isotopic values from 16 P. argus caught at a depth of 25 m in the fore reef suggest that these larger lobsters forage over different habitats and have a higher trophic position than their smaller conspecifics and congeners from the back reef. The feeding ecology of P. argus appears to be less influenced by coral reef degradation than that of P. guttatus, but our results suggest a buffering effect of omnivory against habitat degradation for both lobster species