Single-mode Bragg gratings in tapered few-mode and multimode fibers.

We propose the implementation of fiber Bragg gratings in tapered few-mode and multimode fibers to accomplish single-mode operation by reducing the core diameter, while preserving the core-cladding structure. The gratings present a single reflection band, and the device shows low insertion losses after the taper fabrication and the fiber Bragg gratings inscription. The excitation of high-order odd modes in the core of the fiber has been identified as the main loss mechanism; it can be prevented by means of symmetric illumination of the fibers. We also demonstrate the excitation of high-order cladding modes (cladding-air modes) along the taper transitions; these modes can be removed without a significant increment of the insertion loss.

Successful use of cinacalcet to treat parathyroid-related hypercalcemia in two pediatric patients.

Two pediatric patients with different causes of hyperparathyroidism are reported. First patient is a 13-year-old male with severe hypercalcemia due to left upper parathyroid gland adenoma. After successful surgery, calcium and phosphate levels normalized, but parathormone levels remained elevated. Further studies revealed a second adenoma in the right gland. The second patient is a 13-year-old female with uncommon hypercalcemia symptoms. Presence of pathogenic calcium-sensing receptor gene (CASR) mutation was found, resulting in diagnosis of symptomatic familial hypocalciuric hypercalcemia. Cinacalcet, a calcium-sensing agent that increases the sensitivity of the CASR, was used in both patients with successful results. LEARNING POINTS: Hyperparathyroidism is a rare condition in pediatric patients. If not treated, it can cause serious morbidity.Genetic tests searching for CASR or MEN1 gene mutations in pediatric patients with primary hyperparathyroidism should be performed.Cinacalcet has been effective for treating different causes of hyperparathyroidism in our two pediatric patients.Treatment has been well tolerated and no side effects have been detected.

Instantaneous frequency measurement of dissipative soliton resonant light pulses.

We measured the instantaneous frequency profile of two different dissipative soliton resonant (DSR) light pulses, the usual flat-top and less-common trapezoid-shaped light pulses. The DSR light pulses were provided by an ytterbium-doped polarization-maintaining fiber ring passively mode-locked laser using the adequately selected amount of net-normal dispersion. We confirmed that the DSR light pulses have a (moderately) low linear chirp across the pulse, except at the edges, where the chirp changes exponentially. This unique instantaneous frequency behavior can be succinctly resumed by the following parameters: linear chirp slope and leading and trailing chirp lifetimes. As the pump power increases, the linear chirp slope decreases, whereas the leading and trailing chirp lifetimes do not show an appreciable change. The results are compared with previous theoretical works.

Dissipative soliton resonance in a full polarization-maintaining fiber ring laser at different values of dispersion.

We investigated the dissipative solitons resonance in an ytterbium-doped fiber ring laser in which all the elements are polarization maintaining (PM). A semiconductor saturable absorber mirror was used as a mode-locker. The cavity included a normal dispersion single-mode fiber (SMF) and an anomalous dispersion photonic crystal fiber. The change of the length of the PM SMF allows the variation of the net-normal dispersion of the cavity in the range from 0.022 ps2 to 0.262 ps2. As the absolute value of the net-normal dispersion increases from 0.022 ps2 to 0.21 ps2, a square-shaped single pulse transformed to a single right-angle trapezoid-shaped pulse, and, at the dispersion of 0.262 ps2, to multiple right-angle trapezoid-shaped pulses, per round-trip.

Experimental Transmission of African Swine Fever (ASF) Low Virulent Isolate NH/P68 by Surviving Pigs.

African swine fever (ASF) has persisted in Eastern Europe since 2007, and two endemic zones have been identified in the central and southern parts of the Russian Federation. Moderate- to low-virulent ASF virus isolates are known to circulate in endemic ASF-affected regions. To improve our knowledge of virus transmission in animals recovered from ASF virus infection, an experimental in vivo study was carried out. Four domestic pigs were inoculated with the NH/P68 ASF virus, previously characterized to develop a chronic form of ASF. Two additional in-contact pigs were introduced at 72 days post-inoculation (dpi) in the same box for virus exposure. The inoculated pigs developed a mild form of the disease, and the virus was isolated from tissues in the inoculated pigs up to 99 dpi (pigs were euthanized at 36, 65, 99 and 134 dpi). In-contact pigs showed mild or no clinical signs, but did become seropositive, and a transient viraemia was detected at 28 days post-exposure (dpe), thereby confirming late virus transmission from the inoculated pigs. Virus transmission to in-contact pigs occurred at four weeks post-exposure, over three months after the primary infection. These results highlight the potential role of survivor pigs in disease maintenance and dissemination in areas where moderate- to low-virulent viruses may be circulating undetected. This study will help design better and more effective control programmes to fight against this disease.

Phase recovery by using optical fiber dispersion.

We propose a simple and fast procedure to retrieve the phase profile of arbitrary light pulses. It combines a first experimental stage, followed by a one-step numerical stage. To this end, it is necessary to perform a Fresnel transform, which is obtained just by propagating the light pulses through an optical fiber. We experimentally test this proposal recovering the phase profile in the light pulses provided by a passively mode-locked laser. The proposal is then compared with a temporal variation of the Gerchberg-Saxton recursive algorithm, which is specially modified for this purpose.

Effect of malolactic fermentation by Pediococcus damnosus on the composition and sensory profile of Albariño and Caiño white wines.

AIM: This study was aimed to investigate the influence of malolactic fermentation (MLF) on sensory profile and organoleptic characteristics of Albariño and Caiño white wines. METHODS AND RESULTS: Autochthonous bacteria were isolated from wines after alcoholic fermentation (AF) and further identified as Pediococcus damnosus by 16S rRNA gene sequence analysis. When a commercial Oenococcus oeni starter was inoculated into Albariño and Caiño white wines to perform MLF, which was checked by HPLC quantification of malic and lactic acids, it was shown that autochthonous Ped. damnosus strains were able to predominate over the commercial O. oeni starter and perform MLF in Caiño wine. By contrast, neither commercial strain nor indigenous Pediococcus carried out MLF in Albariño wine. However, MLF was achieved when autochthonous strains that predominated in Caiño were inoculated into Albariño. Sensory analysis showed that after the MLF Albariño increased its body and softness, while Caiño result a more mature wine. CONCLUSIONS: MLF can positively affect Albariño and Caiño wines giving them new attributes. Pediococci isolated and characterized in this work can successfully perform MLF without negative effects on the wine, because no production of biogenic amines or exopolysaccharides by the selected pediococcus strains was detected. SIGNIFICANCE AND IMPACT OF THE STUDY: The effect of MLF in the sensory profile of Albariño and Caiño wines has never been studied before. Results obtained in this work showed that Ped. damnosus strains can be considered as a new topic of investigation on malolactic starter.

Chemical evaluation of white wines elaborated with a recombinant Saccharomyces cerevisiae strain overproducing mannoproteins.

In this study, a recombinant Saccharomyces cerevisiae strain EKD13 overproducing mannoproteins has been used to obtain Albariño white wines. The inoculated strain prevailed and produced complete fermentation of the must, as also occurred in the case of spontaneous (non-inoculated) fermentation and in the must inoculated with the S. cerevisiae EC1118 strain. The analytical study of the wines obtained showed that the most important chemical differences among the wines produced with EKD-13, corresponded to the high concentration of mannoproteins, 2-phenyl ethanol and tyrosol. These differences were attributed to the expression, during must fermentation, of genes modified in the recombinant EKD-13 strain. The results obtained imply that this strain could be potentially useful to produce wines rich in mannoproteins that have distinctive characteristics compared to other similar wines, modifying the sensorial and technological parameters of the wines obtained.

Centro de referencia: Hospital Universitario Vall d´Hebron / Reference centers: University Hospital Vall d´Hebron

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Deficiencia de hormona de crecimiento (GH) en la edad pediátrica / Growth hormone (GH) deficiency in the pediatric age

La deficiencia de hormona de crecimiento (DGH) provoca manifestaciones clínicas distintas, según su etiología y la etapa del desarrollo, pero siempre existe un denominador común: Las otras manifestaciones clínica dependerán d ela etiología (genética, adquirida o idiopática), de la intensidad de la deficienica y de si es la única hormona hipofisaria afectada o existe afectación de otras hormonas hipofisarias. Los avances de los últimos años han ampliado el conocimiento de sus bases moleculares y han caracterizado mejor las formas adquiridas. Sin embargo, la mayor parte de DGH no tienen una causa conocida y son catalogadas como idiopáticas. Mientras que los criterios clínicos y moleculares del diagnóstico de DGH están bien establecidos, los criterios hormonales continúan siendo un rompecabezas a esar de los esfuerzos realizados para armonizar las técnicas bioquímicas de análisis de GH y de IGF-1. Los diagnósticos basados en los estímulos secretores de GH han demostrado ser la escasa utilidad clínica para predecir la respueta terapéutica a la GH (AU)

Growth hormone (GH) deficiency manifests differently according to the individual´s developmental stage. During the paediatric period, one of the msot prominent clincial features is chronic skeletal growth retardation. Clinical signs also depend on the cause (genetic, acquired or idiopathic), deficiency intensity and whether GH is the only pituitary-affected hormone or is combined with that of other pituitary hormones. Growing knowledge of the genetic basis of GH deficiency continues to provide us with useful information to further characterise to provide us with useful information to further characterise mutation types and mechanisms for prevously-described and new candidate genes. Despite these advances, a high proportion of GH deficiencies with no recognisable acquired basis continue to be labelled as idiopathic. The hormonal diagnoses continue to be a conundrum despite efforts to harmonise biochemical assays for GH and IGF-1 analysis, and the diagnosis based on the so-called GH secretion stimulation tests with prove to be of limited usefulness for predicting response to GH therapy (AU)

Optiminzación del diagnóstico y tratamiento de la insuficiencia hepática aguda en Pediatría / Optimizing the diagnosis and treatment of acute liver failure in pediatrics

La insuficiencia hepática aguda en pediatría (IHAP) es una entidad poco, pero muy grave, que puede causar la muerte del paciente o requerir trasplante hepático en un 25-40% de los casos. Distinguir aquellos pacientes con IHAP que van a requerir un trasplante de aquellos que regenerarán con tratamiento médico sigue siendo unr eto para el hepatólogo e intensivista. Desafortunadamente, los sistemas de categorización utilizados en pacientes adultos no predicen correctamente la supervivencia libre de trasplante en pediatría. En nuestro centro, utilizamos el aclaramiento de verde de indocianina como herramienta para catalogar los pacientes afectos de una IHAP que requerirán un trasplante hepático. Según nuestra experiencia, el valor que marcaría la necesidad de trasplantar un pacient sería menor de 5,9%/min don una sensibilidad de 91,6% y una especificidad de 96,8%. Asimismo, en el contexto de una IHAP, los sitemas de asistencia hepática extracorpórea como el MARS (Molecular Adsorbent Recirculating System) pueden servir para comprar tiempo para la regeneración del hígado nativo como puente al trasplante disminuyendo el riesgo de complicaciones cerebrales hasta en pacientes con hígados completamente necróticos (AU)

Pediatric acute liver failure (p-ALF) is a rare disorder wich results in death or the need for liver transplantation (LT) in 25-40% of cases. Distinghishing the children with p-ALF who require LT from those patients who will likely survive with intensive medical care alone remains a unclear making a challenge the decision of when to transplant a patient. The scoring systems availables for prognosis evaluation in adult are unable to predict survival without LT of pediatric patient. Un our hostpial we use the indocyanine green plasma disapparance rate as a tool to precit the evolution of pediatric patients with acute liver failure. Following our experience, a cutoff value of ICG-PDR of 5,9% /min has 91,6% sensitiviry and 96,8% specificity for assessing the need for liver transplantation, improving the categorization of patients with pediatric acute liver failure. In the setting of actue live failure, extracorporeal liver assist devices like MARs (Molecular Adsorbent Recirculating System) may buy time for native liver recovery or serve as bridging therapy to liver transplantation, with reduced risk of cerebral complications, MARS treatment may alleviate hepatic encephalopathy even in patients with a completely necrotic liver (AU)

Enfermedad de Kawasaki. Actualización y revisión de la casuística en nuestro centro en los últimos 10 años / Kawasaki disease. Update and review of the casuistic in our center int he last 10 years

La enfermedad de Kawasaki (EK) es una vasculitis sistémica, aguda y autolimitada, con complicaciones potentcialmente peligrosas, que afecta principalmente a lactantes y niños pequeños (edad< 5 años), aunque también se observa en adolescentes y adultos. Es la principal causa de cardiopatía adquirida en niños de los países desarrollados; su incidencia es de 15,1/100.000 niños de edad < 5 años/año, siendo similar en EE.UU. y Europa. Sin tratmiento, hasta 25% de lso casos desarrolla ectasias o aneurismas coronarios, con riesgo de infarto agudo de miocardio y muerte súbita. Revisamos las manifestaciones clíncias, los criterios diagnósticos, el diagnóstico diferencial, el tratamiento y los marcadores predictivos de no respuesta al tratamiento con inmunoglobulinas. Finalmente, comentaremos los datos de un estudio descriptivo retrospectivo de lso casos de EK diagnosticados en nuestro centro desde enero de 2002 hasta diciembre de 2012 (AU)

Kawasakii disease (KD) is a systemic vasculitis, acute, self-limited, with potentially dangerous complications, which potentially dangerous complications, which manly affects infants and young children (age<5 years), but also seen in adolescents and adults. The EK is the leading cause o acquired heart disease in children in developed countries and their impact on our environment is 15.1/100,000 children < 5 years/year, similar to the U.S. and European countries. Without treatment, up to 25% fo cases of KD develop coronary ectasia or aneurysms with risk of acute myocardial infarction and sudden death. In this work we will review clincial manifestations, diagnostic criteria, differential diagnsois, therapy and predictive markers of non response immunoglobulins. We also present data of a retrospective study of children diagnosed in our center from January 2002 to December 2012 (AU)

Osteoporosis juvenil idiopática / Idiopathic juvenile osteoporosis

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Personalized approach to growth hormone treatment: clinical use of growth prediction models.

The goal of growth hormone (GH) treatment in a short child is to attain a fast catch-up growth toward the target height (TH) standard deviation score (SDS), followed by a maintenance phase, a proper pubertal height gain, and an adult height close to TH. The short-term response variable of GH treatment, first-year height velocity (HV) (cm/year or change in height SDS), can either be compared with GH response charts for diagnosis, age and gender, or with predicted HV based on prediction models. Three types of prediction models have been described: the Kabi International Growth Hormone Study models, the Gothenburg models and the Cologne model. With these models, 50-80% of the variance could be explained. When used prospectively, individualized dosing reduces the variation in growth response in comparison with a fixed dose per body weight. Insulin-like growth factor-I-based dose titration also led to a decrease in the variation. It is uncertain whether adding biochemical, genetic or proteomic markers may improve the accuracy of the prediction. Prediction models may lead to a more evidence-based approach to determine the GH dose regimen and may reduce the drug costs for GH treatment. There is a need for user-friendly software programs to make prediction models easily available in the clinic.

Technological and safety properties of lactic acid bacteria isolated from Spanish dry-cured sausages.

Technological and safety-related properties were analyzed in lactic acid bacteria isolated from Spanish dry-cured sausages in order to select them as starter cultures. In relation to technological properties, all the strains showed significative nitrate reductase activity; Lactobacillus plantarum, Lactobacillus paracasei and 52% of the Enterococcus faecium strains showed lipolytic activity and only Lactobacillus sakei strains (43%) were able to form biofilms. Related to safety aspects, E. faecium strains were the most resistant to antibiotics, whereas, L. sakei strains were the most sensitive. In relation to virulence factors, in the E. faecium strains analyzed, only the presence of efaA gene was detected. The analysis of biogenic amine production showed that most E. faecium strains and L. sakei Al-142 produced tyramine. In conclusion, L. paracasei Al-128 and L. sakei Al-143 strains possess the best properties to be selected as adequate and safe meat starter cultures.

Height gain at adult-height age in 184 short patients treated with growth hormone from prepubertal age to near adult-height age is not related to GH secretory status at GH therapy onset.

BACKGROUND: GH release after stimuli classifies short children as severe idiopathic isolated GH deficiency (IIGHD), mild IIGHD, dissociated GH release (DGHR) and normal GH release (NGHR) and anthropometric birth data as adequate for gestational age (AGA) or small for gestational age (SGA). GH release after stimuli classifies AGA patients as IIGHD or as idiopathic short stature (ISS). AIM: To compare height gain induced by GH therapy (31.8 ± 3.5 µg/kg/day, 7.7 ± 1.6 years) started at prepubertal age and stopped at near adult-height age. METHODS: A retrospective longitudinal multicenter study including 184 short patients classified as severe IIGHD n = 25, mild IIGHD n = 75, DGHR n = 55 and NGHR n = 29; or as IIGHD n = 78, ISS n = 57 and SGA n = 49. Height gain was evaluated throughout GH therapy and at adult-height age. RESULTS: Height-SDS gain at adult-height age was similar among severe IIGHD (1.8 ± 0.8 SDS), mild IIGHD (1.6 ± 0.6 SDS), DGHR (1.7 ± 0.7 SDS) and NGHR (1.6 ± 0.7 SDS), or among IIGHD (1.7 ± 0.7 SDS), ISS (1.7 ± 0.6 SDS) and SGA (1.6 ± 0.8 SD). CONCLUSION: GH-release stimuli are of little help for deciding on GH therapy in the clinical management of prepubertal children with IIGHD, ISS or SGA.

Photonic fractional Fourier transformer with a single dispersive device.

In this work we used the temporal analog of spatial Fresnel diffraction to design a temporal fractional Fourier transformer with a single dispersive device, in this way avoiding the use of quadratic phase modulators. We demonstrate that a single dispersive passive device inherently provides the fractional Fourier transform of an incident optical pulse. The relationships linking the fractional Fourier transform order and scaling factor with the dispersion parameters are derived. We first provide some numerical results in order to prove the validity of our proposal, using a fiber Bragg grating as the dispersive device. Next, we experimentally demonstrate the feasibility of this proposal by using a spool of a standard optical fiber as the dispersive device.

Growth hormone deficiency: an update.

Growth hormone (GH) deficiency (GHD) in humans manifests differently according to the individual developmental stage (early after birth, during childhood, at puberty or in adulthood), the cause or mechanism (genetic, acquired or idiopathic), deficiency intensity and whether it is the only pituitary-affected hormone or is combined with that of other pituitary hormones or forms part of a complex syndrome. Growing knowledge of the genetic basis of GH deficiency continues to provide us with useful information to further characterise mutation types and mechanisms for previously described and new candidate genes. Despite these advances, a high proportion of GH deficiencies with no recognisable acquired basis continue to be labelled as idiopathic, although less frequently when they are congenital and/or familial. The clinical and biochemical diagnoses continue to be a conundrum despite efforts to harmonise biochemical assays for GH and IGF-1 analysis, probably because the diagnosis based on the so-called GH secretion stimulation tests will prove to be of limited usefulness for predicting therapy indications.

Characterization of coagulase-negative staphylococci isolated from Spanish dry cured meat products.

Technological and safety-related properties were analyzed in a coagulase-negative staphylococci (CNS) collection isolated from Spanish dry-cured meat products in order to use them as starter cultures. The highest nitrate reductase and proteolytic activity was showed by Staphylococcus carnosus and Staphylococcus equorum. Only a few strains were able to form biofilms and the presence of the ica gene was analyzed on them. In relation to antibiotic resistance, all S. carnosus and most of the S. equorum strains were sensitive to the antibiotics tested and the presence of the blaZ gene in the ß-lactamic resistant strains was studied. Biogenic amines were produced by 25% of the strains analyzed being all the S. carnosus strains tyramine producers. Taking into account the studied properties, two S. equorum strains could be selected as adequate and safe potential starter cultures for the elaboration of meat products.