Caso epidermólisis ampollosa en lactante

La epidermolisis ampollosa es un grupo heterogéneo de enfermedades hereditarias de la piel, en la que aparece de forma de flictenas de forma espontánea o por traumatismos mínimos. Esto se debe a una mutación genética que provoca una alteración de las proteínas estructurales que mantienen la unión dermoepidérmica, formando una ampolla subepidérmica, que facilita la aparición de cicatrices y retracciones de la piel. Las características principales de esta enfermedad son, erupción de flictenas de contenido serohemorrágico, pudiendo afectar a mucosas, piel y uñas, presencia de sindactilias, piel seca, manosen garra con formación de muñones, enfermedades dentales. Otras complicaciones más graves son la sineniquia conjuntival, ronquera, estenosisesofágica, neumonía.La epidermolisis ampollosa afecta a ambos sexos por igual y a todas las razas, la frecuencia oscila entre 1 de cada 50.000 nacidos y la edad de aparición es en el nacimiento o la lactancia.Enfermerí

Search for coherent charged pion production in neutrino-carbon interactions

We report the result from a search for charged-current coherent pion production induced by muon neutrinos with a mean energy of 1.3 GeV. The data are collected with a fully active scintillator detector in the K2K long-baseline neutrino oscillation experiment. No evidence for coherent pion production is observed and an upper limit of $0.60 \times 10^{-2}$ is set on the cross section ratio of coherent pion production to the total charged-current interaction at 90% confidence level. This is the first experimental limit for coherent charged pion production in the energy region of a few GeV.Comment: 5 pages, 4 figure

Evidence for muon neutrino oscillation in an accelerator-based experiment

We present results for muon neutrino oscillation in the KEK to Kamioka (K2K) long-baseline neutrino oscillation experiment. K2K uses an accelerator-produced muon neutrino beam with a mean energy of 1.3 GeV directed at the Super-Kamiokande detector. We observed the energy dependent disappearance of muon neutrino, which we presume have oscillated to tau neutrino. The probability that we would observe these results if there is no neutrino oscillation is 0.0050% (4.0 sigma).Comment: 5 pages, 4 figure

Usefulness of bone turnover markers as predictors of mortality risk, disease progression and skeletal-related events appearance in patients with prostate cancer with bone metastases following treatment with zoledronic acid: TUGAMO study

Owing to the limited validity of clinical data on the treatment of prostate cancer (PCa) and bone metastases, biochemical markers are a promising tool for predicting survival, disease progression and skeletal-related events (SREs) in these patients. The aim of this study was to evaluate the predictive capacity of biochemical markers of bone turnover for mortality risk, disease progression and SREs in patients with PCa and bone metastases undergoing treatment with zoledronic acid (ZA). Methods: This was an observational, prospective and multicenter study in which ninety-eight patients were included. Patients were treated with ZA (4mg every 4 weeks for 18 months). Data were collected at baseline and 3, 6, 9, 12, 15 and 18 months after the beginning of treatment. Serum levels of bone alkaline phosphtase (BALP), aminoterminal propeptide of procollagen type I (P1NP) and beta-isomer of carboxiterminal telopeptide of collagen I (b-CTX) were analysed at all points in the study. Data on disease progression, SREs development and survival were recorded. Results: Cox regression models with clinical data and bone markers showed that the levels of the three markers studied were predictive of survival time, with b-CTX being especially powerful, in which a lack of normalisation in visit 1 (3 months after the beginning of treatment) showed a 6.3-times more risk for death than in normalised patients. Levels of these markers were also predictive for SREs, although in this case BALP and P1NP proved to be better predictors. We did not find any relationship between bone markers and disease progression. Conclusion: In patients with PCa and bone metastases treated with ZA, b-CTX and P1NP can be considered suitable predictors for mortality risk, while BALP and P1NP are appropriate for SREs. The levels of these biomarkers 3 months after the beginning of treatment are especially importantThis study was supported by Novartis Oncology Spai

R-Allyl Nickel(II) Complexes with Chelating N-Heterocyclic Carbenes: Synthesis, Structural Characterization, and Catalytic Activity

The N-heterocyclic carbene (NHC) nickel complexes [(L)Ni(NHC)][BArF4] (ArF = 3,5-bis(trifluoromethyl)- phenyl; L = allyl (1), methylallyl (2); NHC = 1-(2-picolyl)-3-methylimidazol-2-ylidene (a), 1-(2-picolyl)-3-isopropylimidazol-2-ylidene (b), 1-(2-picolyl)-3-n-butylimidazol-2-ylidene (c), 1-(2-picolyl)-3-phenylimidazol-2-ylidene (d), 1-(2-picolyl)-3- methylbenzoimidazol-2-ylidene (e), 1-(2-picolyl)-4,5-dichloro-3-methylimidazol-2-ylidene (f)) have been obtained in high yields and characterized by NMR spectroscopy. Furthermore, 1d was unambiguously characterized by single-crystal X-ray crystallography. Complexes 1a−f/2a−f have shown catalytic activity toward dimerization and hydrosilylation of styrenes. In particular, 1a proved to be the most efficient catalyst in the dimerization of styrene derivatives in the absence of cocatalyst. Also, complexes 1a,d showed high selectivity and moderate to good yields in hydrosilylation reactions