Publications by authors named "Sirtori L"

Objective: The aim of the study was to evaluate the effectiveness of interventions designed to reduce problems with readability and comprehensibility in drug labels.

Methods: A systematic review of observational and experimental studies was conducted using MEDLINE (via PubMed), Embase, and Scopus databases. Two independent reviewers selected articles and collected data about study design, interventions, and outcomes.

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Objective: To evaluate the labeling preferences of medication users and characterize their perceptions of the comprehensibility and readability of medication labels.

Methods: We conducted a population-based cross-sectional study of medication users aged 18 years or older in 10 Brazilian capital cities. Perceptions of the comprehensibility and readability of medication labels in relation to sociodemographic characteristics were evaluated by Poisson regression models with robust variance.

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The mode of action of an antimicrobial peptide produced by Bacillus sp. P45 isolated from the intestine of the Amazonian basin fish Piaractus mesopotamicus was investigated. The antimicrobial peptide was purified from culture supernatants by precipitation with ammonium sulfate and gel filtration chromatography.

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X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder biochemically characterized by the accumulation of very long chain fatty acids (VLCFA), particularly hexacosanoic acid (C(26:0)) and tetracosanoic acid (C(24:0)), in tissues and biological fluids. Although patients affected by this disorder predominantly present central and peripheral demyelination as well as adrenal insufficiency, the mechanisms underlying the brain damage in X-ALD are poorly known. The current treatment of X-ALD with glyceroltrioleate (C(18:1))/glyceroltrierucate (C(22:1)) (Lorenzo's oil, LO) combined with a VLCFA-poor diet normalizes VLCFA concentrations, but the neurological symptoms persist or even progress in symptomatic patients.

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Phenylketonuria (PKU) is an autossomal recessive disease caused by phenylalanine-4-hydroxylase deficiency, which is a liver-specific enzyme that catalyzes the hydroxylation of l-phenylalanine (Phe) to l-tyrosine (Tyr). The deficiency of this enzyme leads to the accumulation of Phe in the tissues and plasma of patients. The clinical characterization of this disease is mental retardation and other neurological features.

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X-linked adrenoleukodystrophy (X-ALD) is a hereditary disorder of peroxisomal metabolism biochemically characterized by the accumulation of very long chain fatty acids (VLCFA), particularly hexacosanoic acid (C26:0) and tetracosanoic acid (C24:0) in different tissues and in biological fluids. The disease is clinically characterized by central and peripheral demyelination and adrenal insufficiency, which is closely related to the increased concentrations of these fatty acids. However, the mechanisms underlying the brain damage in X-ALD are poorly known.

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Background: Organic acidurias or organic acidemias are inherited metabolic disorders in which organic acids (carboxylic acids) accumulate in tissues and physiologic fluids of affected individuals. They are considered the most frequent metabolic disorders among severely ill children. Patients frequently present acute symptoms in early life.

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