International guidelines for the management and treatment of Morquio A syndrome.

Morquio A syndrome (mucopolysaccharidosis IVA) is a lysosomal storage disorder associated with skeletal and joint abnormalities and significant non-skeletal manifestations including respiratory disease, spinal cord compression, cardiac disease, impaired vision, hearing loss, and dental problems. The clinical presentation, onset, severity and progression rate of clinical manifestations of Morquio A syndrome vary widely between patients. Because of the heterogeneous and progressive nature of the disease, the management of patients with Morquio A syndrome is challenging and requires a multidisciplinary approach, involving an array of specialists. The current paper presents international guidelines for the evaluation, treatment and symptom-based management of Morquio A syndrome. These guidelines were developed during two expert meetings by an international panel of specialists in pediatrics, genetics, orthopedics, pulmonology, cardiology, and anesthesia with extensive experience in managing Morquio A syndrome

Effect of temperature and flow rate on the cell-free area in the microfluidic channel

Blood cell manipulation in microdevices is an interesting task for the separation of particles, by their size, density, or to remove them from the buffer, in which they are suspended, for further analysis, and more. This study highlights the cell-free area (CFA) widening based on experimental results of red blood cell (RBC) flow, suspended in a microfluidic device, while temperature and flow rate incrementally modify RBC response within the microflow. Studies of human red blood cell flow, at a concentration of 20%, suspended in its autologous plasma and phosphate-buffered saline (PBS) buffer, were carried out at a wide flow rate, varying between 10 and 230 µL/min and a temperature range of 23 ◦C to 50 ◦C. The plotted measures show an increment in a CFA near the channel wall due to cell flow inertia after a constricted channel, which becomes more significant as temperature and flow rate increase. The temperature increment widened the CFA up to three times. In comparison, flow rate increment increased the CFA up to 20 times in PBS and 11 times in plasma

Reproducción sexual sincronizada del pasto marino Syringodium filiforme (Cymodoceaceae) en la laguna de un arrecife tropical en la costa Caribe de Costa Rica

There has been an increasing effort to understand the mechanisms of sexual reproduction in seagrasses, which is usually synchronized. Synchronization is caused by environmental cues, such as temperature and light availability, and most likely occurs to maximize pollination success. At higher latitudes where seagrass reproductive seasons are clearly marked, intraannual variability of environmental triggers is significant. Our aim was to identify the period and frequency of sexual reproduction for the manatee grass Syringodium filiforme in a tropical coral reef lagoon, where the above-mentioned environmental triggers are homogenous all year round. The reproductive state and frequency and shoot length of S. filiforme were measured non-destructively in a monospecific patch on the tropical Caribbean coast of Costa Rica. The meadow was surveyed at 1 to 4-month intervals between May 2010 and May 2012. Water turbidity, temperature, and salinity were measured in situ. During the surveyed period, sexual reproduction of S. filiforme was detected in February and May, while seagrass shoot length showed no variation. The moment of seagrass sexual reproduction was not fully explained by the environmental parameters studied. Regardless of the limited environmental variability compared to previous studies at higher latitudes, synchronization of seagrass sexual reproduction at this tropical location is interesting and requires further studies on mechanisms and its possible adaptive advantage.Ha habido un incremento en los esfuerzos por entender los mecanismos de reproducción sexual en pastos marinos, la cual usualmente ocurre de manera sincronizada. Se considera que esta sincronización se da para maximizar el éxito de la polinización. Esta sincronización en la reproducción sexual de pastos marinos es inducida por señales ambientales, como la temperatura y disponibilidad de luz. A mayores latitudes, donde los pastos marinos tienen períodos de reproducción estacional marcados, la variabilidad interanual de estas señales ambientales es significativa. Nuestro objetivo fue identificar el período de tiempo y frecuencia de reproducción sexual del pasto de manatí, Syringodium filiforme, en una laguna de arrecife tropical, donde los factores ambientales mencionados son homogéneos a lo largo del año. El estado y frecuencia reproductiva, así como la longitud de los haces, de S. filiforme se midieron de manera no destructiva en un parche monoespecífico de una pradera en la costa Caribe de Costa Rica. Esta pradera se muestreó en intervalos de 1 a 4 meses entre mayo del 2010 y mayo del 2012. La turbidez del agua, temperatura y salinidad se midieron in situ. Nuestro estudio identificó reproducción sexual durante el período de muestreo para S. filiforme en febrero y mayo en el sitio de estudio; mientras que la longitud de haces no evidenció variación. El momento de reproducción sexual de pasto marino no fue explicado de lleno por los parámetros ambientales estudiados. Su sincronización en esta ubicación tropical, a pesar de contar con variabilidad ambiental limitada en comparación con estudios previos a mayores latitudes, es interesante y amerita más investigación sobre los mecanismos y la posible ventaja adaptativa de la reproducción sexual sincronizada en pastos marinos.Centro de Investigación del Mar y Limnología/[808-96-298]/CIMAR/Costa RicaUCR::Vicerrectoría de Investigación::Unidades de Investigación::Ciencias Básicas::Centro de Investigación en Ciencias del Mar y Limnología (CIMAR)UCR::Vicerrectoría de Docencia::Ciencias Básicas::Facultad de Ciencias::Escuela de Biologí

Vibrational quenching of CO2(010) by collisions with O(3P) at thermal energies: A quantum-mechanical study

10 pages, 6 figures, 1 table, 1 appendix.-- PACS nrs.: 34.50.Ez; 33.20.Tp; 33.15.Mt; 31.15.Ar.The CO2(010)–O(3P) vibrational energy transfer (VET) efficiency is a key input to aeronomical models of the energy budget of the upper atmospheres of Earth, Venus, and Mars. This work addresses the physical mechanisms responsible for the high efficiency of the VET process at the thermal energies existing in the terrestrial upper atmosphere (150 K ≤ T ≤ 550 K). We present a quantum-mechanical study of the process within a reduced-dimensionality approach. In this model, all the particles remain along a plane and the O(3P) atom collides along the C(2v) symmetry axis of CO2, which can present bending oscillations around the linear arrangement, while the stretching C–O coordinates are kept fixed at their equilibrium values. Two kinds of scattering calculations are performed on high-quality ab initio potential energy surfaces (PESs). In the first approach, the calculations are carried out separately for each one of the three PESs correlating to O(3P). In the second approach, nonadiabatic effects induced by spin-orbit couplings (SOC) are also accounted for. The results presented here provide an explanation to some of the questions raised by the experiments and aeronomical observations. At thermal energies, nonadiabatic transitions induced by SOC play a key role in causing large VET efficiencies, the process being highly sensitive to the initial fine-structure level of oxygen. At higher energies, the two above-mentioned approaches tend to coincide towards an impulsive Landau-Teller mechanism of the vibrational to translational (V-T) energy transfer.This work has been partially supported by the European Project No. R113-2003-506079 and the DGICYT Spanish Grant Nos. FIS2004-02461 and CTQ-2004-02415/BQU. One of the authors (M.P.d.L.-C.) was supported by the "Ramón y Cajal" Programme and another author (M.L.-P.) by the Spanish projects Nos. REN2001-3249/CLI, ESP2004-01556, and by EC FEDER funds. The calculations presented here were performed at CINECA (the SuperComputer Center of the University of Bologna), the Instituto de Matemáticas y Física Fundamental (CSIC), and CESGA (the SuperComputer Center of Galicia).Peer reviewe

A hybrid classical/quantum approach to cluster fragmentation dynamics: Application to the vibrational predissociation of He2Cl2

A new hybrid classical/quantum method is proposed and applied to investigate the vibrational predissociation (VP) dynamics of the He2Cl2 complex. The full dimensionality of the system (assuming zero total angular momentum) is included in the method. The VP process He2Cl2 is dominated by a sequential mechanism of dissociation of the two van der Waals bonds. The hybrid approach describes the first weak bond fragmentation classically, and the second one quantum mechanically. The rotational distribution of the Cl2 fragment is calculated both with the hybrid method and with a fully classical trajectory simulation, and compared with the experimental distribution. The hybrid distribution is found to agree very well with the experimental one, and to involve a substantial improvement with respect to the classical result. © 1998 American Institute of Physics.This work has been supported by the D.G.I.C.Y.T. Grant No. PB95-0071 ~Spain! and the Spanish-French cooperation program PICASSO No. HF1996-0232.Peer Reviewe