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A 26-year-old male diagnosed with cutaneous leukocytoclastic vasculitis was admitted due to abdominal pain and rectal bleeding with slight clinical-analytical impact. On examination, he presented multiple palpable purpuric lesions on his legs.

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We aimed to identify the plasma miRNA profile of antiphospholipid syndrome (APS) patients and to investigate the potential role of specific circulating miRNAs as non-invasive disease biomarkers. Ninety APS patients and 42 healthy donors were recruited. Profiling of miRNAs by PCR-array in plasma of APS patients identified a set of miRNAs differentially expressed and collectively involved in clinical features. Logistic regression and ROC analysis identified a signature of 10 miRNA ratios as biomarkers of disease. In addition, miRNA signature was related to fetal loss, atherosclerosis, and type of thrombosis, and correlated with parameters linked to inflammation, thrombosis, and autoimmunity. Hard clustering analysis differentiated 3 clusters representing different thrombotic risk profile groups. Significant differences between groups for several miRNA ratios were found. Moreover, miRNA signature remained stable over time, demonstrated by their analysis three months after the first sample collection. Parallel analysis in two additional cohorts of patients, including thrombosis without autoimmune disease, and systemic lupus erythematosus without antiphospholipid antibodies, each displayed specific miRNA profiles that were distinct from those of APS patients. In vitro, antiphospholipid antibodies of IgG isotype promoted deregulation in selected miRNAs and their potential atherothrombotic protein targets in monocytes and endothelial cells. Taken together, differentially expressed circulating miRNAs in APS patients, modulated at least partially by antiphospholipid antibodies of IgG isotype, might have the potential to serve as novel biomarkers of disease features and to typify patients' atherothrombotic status, thus constituting a useful tool in the management of the disease.

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PURPOSE: The aim of this study was to assess the intraoperator and interoperator agreement for manual measurements of intima-media thickness (IMT) performed under a strict carotid ultrasound technical protocol. METHODS: Two blinded experienced operators independently performed an ultrasound examination at the distal common carotid of 242 subjects in the same patient's position, diastolic phase, probe type, zooming, and depth. Thirty-six subjects were reevaluated in another time point. Three different-angle manual measurements (IMTindiv) were obtained. Interoperator agreements for each IMTindiv, and their mean (IMTmean) and maximum (IMTmax) values, were assessed with the intraclass correlation coefficient and Bland-Altman analysis. Intraoperator agreement was tested taking advantage of the second ultrasound round in 36 subjects. RESULTS: IMTmean agreements (intraoperator, 0.665-0.913; interoperator, 0.856-0.897) were higher than IMTmax (intraoperator, 0.435-0.793; interoperator, 0.631-0.718) and any IMTindiv (intraoperator, 0.355-0.676; interoperator, 0.590-0.717). Despite the small systematic error for IMTmean (intraoperator, ≤0.03; interoperator, ≤0.02 mm), at best of times, the sampling error size reached at least 0.28 and 0.25 mm for intraoperator and interoperator agreements, respectively, and was never less than 0.13 mm. CONCLUSIONS: Although IMTmean agreement is excellent under a strict protocol, limits of agreement might be too wide to consider carotid ultrasound a robust cardiovascular risk biomarker.

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Wilson's Disease (WD) is an autosomal recessive disorder of copper metabolism resulting in a pathological accumulation of this metal, initially in the liver and later in other organs, mainly brain. Treatment with copper chelating agents and zinc salts results in a depletion of copper deposits and prevents or reverses the clinical manifestations. Copper deficiency may cause haematological and neurological changes, the latter principally being polyneuropathy and myelopathy. We report a patient with WD who developed a myelopathy associated with a deficiency of copper following prolonged treatment with D-penicillamine and zinc salts (AU)

La enfermedad de Wilson (EW) es una enfermedad autosómica recesiva del metabolismo del cobre que provoca la acumulación patológica de este metal, primero en el hígado y posteriormente en otros órganos, principalmente el cerebro. El tratamiento con agentes quelantes del cobre y sales de zinc conduce al agotamiento de los depósitos de cobre y previene o revierte las manifestaciones clínicas de esta enfermedad. El déficit de cobre puede causar alteraciones hematológicas y neurológicas, entre estas últimas principalmente polineuropatía y mielopatía. Se presenta un paciente con EW que ha desarrollado una mielopatía asociada con la deficiencia de cobre tras un tratamiento prolongado con D-penicilamina y sales de zinc (AU)

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Wilson's Disease (WD) is an autosomal recessive disorder of copper metabolism resulting in a pathological accumulation of this metal, initially in the liver and later in other organs, mainly brain. Treatment with copper chelating agents and zinc salts results in a depletion of copper deposits and prevents or reverses the clinical manifestations. Copper deficiency may cause haematological and neurological changes, the latter principally being polyneuropathy and myelopathy. We report a patient with WD who developed a myelopathy associated with a deficiency of copper following prolonged treatment with D-penicillamine and zinc salts.

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