The Role of Vulnerability, Anticipation, and Narrative Structure in California’s Drought Management and Water Allocation Strategies

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Longitudinal and transverse noise in a moving Vortex Lattice

We have studied the longitudinal and the transverse velocity fluctuations of a moving vortex lattice (VL) driven by a transport current. They exhibit both the same broad spectrum and the same order of magnitude. These two components are insensitive to the velocity and to a small bulk perturbation. This means that no bulk averaging over the disorder and no VL crystallization are observed. This is consistently explained referring to a previously proposed noisy flow of surface current whose elementary fluctuator is measured isotropic.Comment: accepted for publication in Phys Rev

Experimental study of the correlation length of critical-current fluctuations in the presence of surface disorder: Probing vortex long-range interactions

We report on critical currents and voltage noise measurements in Niobium strips in the superconducting state, in the presence of a bulk vortex lattice ($B < B_{C2}$) and in the surface superconducting state ($B_{c2}< B < B_{C3}$). For homogeneous surfaces, the correlation length of the current fluctuations can be associated with the electromagnetic skin depth of vortex superficial instabilities. The modification of the surface state by means of low energy irradiation induces a strong modification of the critical current and of the noise. The appearance of a corner frequency in the spectral domain can be linked with the low wave-vectors of the artificial corrugation. Since this latter occurs only for $B < B_{C2}$, we propose that the long-range interactions allow the correlation length to extend up to values imposed by the surface topography.Comment: accepted for publication in PR

Metastable states of a flux line lattice studied by transport and Small Angle Neutron Scattering

Flux Lines Lattice (FLL) states have been studied using transport measurements and Small Angle Neutron Scattering in low T$_c$ materials. In Pb-In, the bulk dislocations in the FLL do not influence the transport properties. In Fe doped NbSe$_{2}$, transport properties can differ after a Field Cooling (FC) or a Zero Field Cooling (ZFC) procedure, as previously reported. The ZFC FLL is found ordered with narrow Bragg Peaks and is linked to a linear V(I) curve and to a superficial critical current. The FC FLL pattern exhibits two Bragg peaks and the corresponding V(I) curve shows a S-shape. This can be explained by the coexistence of two ordered FLL slightly tilted from the applied field direction by different superficial currents. These currents are wiped out when the transport current is increased.Comment: accepted for publication in Phys. Rev.

Molecular characterisation of congenital myasthenic syndromes in Southern Brazil

Objective To perform genetic testing of patients with congenital myasthenic syndromes (CMS) from the Southern Brazilian state of Parana. Patients and methods Twenty-five CMS patients from 18 independent families were included in the study. Known CMS genes were sequenced and restriction digest for the mutation RAPSN p.N88K was performed in all patients. Results We identified recessive mutations of CHRNE in ten families, mutations in DOK7 in three families and mutations in COLQ, CHRNA1 and CHRNB1 in one family each. The mutation CHRNE c. 70insG was found in six families. We have repeatedly identified this mutation in patients from Spain and Portugal and haplotype studies indicate that CHRNE c. 70insG derives from a common ancestor. Conclusions Recessive mutations in CHRNE are the major cause of CMS in Southern Brazil with a common mutation introduced by Hispanic settlers. The second most common cause is mutations in DOK7. The minimum prevalence of CMS in Parana is 0.18/100 000

Molecular characterisation of congenital myasthenic syndromes in Southern Brazil

Objective To perform genetic testing of patients with congenital myasthenic syndromes (CMS) from the Southern Brazilian state of Parana. Patients and methods Twenty-five CMS patients from 18 independent families were included in the study. Known CMS genes were sequenced and restriction digest for the mutation RAPSN p.N88K was performed in all patients. Results We identified recessive mutations of CHRNE in ten families, mutations in DOK7 in three families and mutations in COLQ, CHRNA1 and CHRNB1 in one family each. The mutation CHRNE c. 70insG was found in six families. We have repeatedly identified this mutation in patients from Spain and Portugal and haplotype studies indicate that CHRNE c. 70insG derives from a common ancestor. Conclusions Recessive mutations in CHRNE are the major cause of CMS in Southern Brazil with a common mutation introduced by Hispanic settlers. The second most common cause is mutations in DOK7. The minimum prevalence of CMS in Parana is 0.18/100 000

A Rare Cause of Chronic Hypokalemia with Metabolic Alkalosis: Case Report and Differential Diagnosis

Hypokalemia and metabolic alkalosis can be present in different rare diseases, and the differential diagnosis of these forms is challenging. Apparent mineralcorticoid (AME) excess syndrome is one of these conditions. Characterized by increased blood pressure due to excessive sodium retention and plasma volume, it is caused by a mutation in the HSD11B2 gene encoding the oxydoreductase enzyme 11β-hydroxysteroide dehydrogenase type 2. We report the case of a child presenting with failure to thrive associated with early detection of hypokalemia, metabolic alkalosis, nephrocalcinosis and hypertension in which AME syndrome was detected. A novel mutation in the HSD11B2 gene was identified in this patient. In clinical pictures characterized by metabolic alkalosis and hypokalemia, the evaluation of renin, aldosterone and blood pressure is crucial for accurate diagnosis. AME syndrome is a rare disorder that can be an insidious but lethal disease, if untreated. With clinical signs appearing during the first days of life. Early diagnosis is imperative in order to enable prompt and adequate treatment to improve the outcome of these patients

Redox Modulations, Antioxidants, and Neuropsychiatric Disorders

Although antioxidants, redox modulations, and neuropsychiatric disorders have been widely studied for many years, the field would benefit from an integrative and corroborative review. Our primary objective is to delineate the biological significance of compounds that modulate our redox status (i.e., reactive species and antioxidants) as well as outline their current role in brain health and the impact of redox modulations on the severity of illnesses. Therefore, this review will not enter into the debate regarding the perceived medical legitimacy of antioxidants but rather seek to clarify their abilities and limitations. With this in mind, antioxidants may be interpreted as natural products with significant pharmacological actions in the body. A renewed understanding of these often overlooked compounds will allow us to critically appraise the current literature and provide an informed, novel perspective on an important healthcare issue. In this review, we will introduce the complex topics of redox modulations and their role in the development of select neuropsychiatric disorders