São Paulo medical journal = Revista paulista de medicina
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Review Case Reports
Indolent systemic mastocytosis limited to the bone: a case report and review of the literature.
CONTEXT Systemic mastocytosis is defined as a clonal disorder of mast cells and their precursor cells and is currently classified as a myeloproliferative neoplasm. Its clinical course has a wide spectrum, ranging from indolent disease, with normal life expectancy, to highly aggressive disease, associated with multisystemic involvement and poor overall survival. The aim of this study was to report a case of indolent systemic mastocytosis, focusing on the diagnostic challenges, with a review of the literature. ⋯ Its symptoms are often unspecific and frequently ignored. Skeletal changes may be the first and only manifestation of the disease and in some cases, like this one, the diagnosis is made only after histological examination. The key point for the diagnosis is to contemplate the possibility of systemic mastocytosis.
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CONTEXT AND OBJECTIVE The term mastocytosis covers a group of rare disorders characterized by neoplastic proliferation and accumulation of clonal mast cells in one or more organs. The aim of this study was to assess the principal elements for diagnosing and treating these disorders. DESIGN AND SETTING Narrative review of the literature conducted at Grupo Fleury, São Paulo, Brazil. ⋯ Abnormal mast cells display atypical and frequently spindle-shaped morphology, and aberrant expression of the CD25 and CD2 antigens. Elevation of serum tryptase is a common finding in some subtypes, and more than 90% of the patients present the D816V KIT mutation in mast cells. CONCLUSION Here, we described the most common signs and symptoms among patients with mastocytosis and suggested a practical approach for the diagnosis, classification and initial clinical treatment of mastocytosis.
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Despite numerous spirituality and/or religiosity (S/R) measurement tools for use in research worldwide, there is little information on S/R instruments in the Portuguese language. The aim of the present study was to map out the S/R scales available for research in the Portuguese language. ⋯ Currently, 20 instruments for measuring S/R are available in the Portuguese language. Most have been translated (n = 15) or developed (n = 3) in Brazil and present good internal consistency. Nevertheless, few instruments have been assessed regarding all their psychometric qualities.
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Among the current treatments available for acne vulgaris, many widely practiced options lack support from studies at the best level of scientific evidence. The aim of this narrative review was to present the very latest information on topical and systemic treatments for acne vulgaris. ⋯ There are important issues regarding the clinical management of acne that still lack consistent grounding in scientific evidence. Among these are the optimum dose and duration of treatment with oral antibiotics that can be given without inducing bacterial resistance, and the safety of oral isotretinoin.
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The Beliefs about Medicines Questionnaire (BMQ-Specific) has proven useful for measuring patients' beliefs and associating them with non-adherence to treatment in several illness groups. The aim was to cross-culturally adapt the BMQ-Specific into Portuguese for the general population of medicine users. ⋯ A cross-culturally adapted Portuguese version of the BMQ-Specific questionnaire for use among the general population of medicine users was obtained, presenting good internal consistency and component structure identical to the original English version.