121,838 research outputs found
Cardiac amyloidosis: the pathologist’s point of view
Cardiac amyloidosis is a well-known entity recently recognized as a common etiology of heart failure. This infiltrative disease is caused by the deposition of misfolded proteins within the heart. The most common types of cardiac amyloidosis result from fibrils composed of monoclonal immunoglobulin light chains or transthyretin. Clinical presentation is usually elusive, and this can result in diagnostic delay. Diagnosis can be reached with non-invasive methods, but it often requires tissue sampling with pathological analysis. It is fundamental to determine the type of protein being deposited in order to indicate the specific treatment. In this article, we review the main features of cardiac amyloidosis with a focus on different pathological presentations of this rare disorder
Low mast cell density in the human duodenal mucosa from chronic inflammatory duodenal bowel disorders is associated with defective villous architecture
Correlating Noble Rot Infection of Garganega Withered Grapes with Key Molecules and Odorants of Botrytized Passito Wine
Experimental passito wines with dierent percentages of naturally noble-rotten grapes of the Garganega variety were analyzed to evaluate key molecules and odorants related to the typical aroma and sensory profile of botrytized passito wine. Remarkable changes in the concentration of 1-octen-3-ol, 4-terpineol, benzaldehyde, N-(3-methylbutyl)acetamide, and sherry lactone 1 and 2 were observed between sound and noble-rotten wines. Wines were perceived to be dierent for floral, honey, figs, apricot, and caramel scents. By partial least square regression these descriptors were well correlated to samples. An important positive contribution of sherry lactones, N-(3-methylbutyl)acetamide, vanillin, benzaldehyde, and -butyrolactone to honey, apricot, and caramel was observed. It is conceivable that oxidative eects of Botrytis cinerea infection play an important role in the genesis of these chemical and sensory aroma markers. This study provides a predictive tool for winemakers that use natural grape withering to produce wines whose aroma profile is not standardized due to the seasonal variation of noble rot incidence
Co-localization of tryptase and cathepsin-G in mast cells in cutaneous mastocytosis
Mastocytosis is a heterogeneous disease characterized by an abnormal growth and/or accumulation of clonal mast cells (MC) in one or more organs. The most frequent site of organ involvement is the skin. The aim of this study was to investigate the immunoreactivity to tryptase and to cathepsin-G of MC from human cutaneous mastocytosis and to compare their number in normal skin and cutaneous mastocytosis. Immunohistochemistry and dual immunofluorescence using anti-tryptase and anti-capthepsin-G antibodies was performed on biopsy specimens from 20 cases diagnosed as cutaneous mastocytosis. Tryptase-positive MC was more numerous as compared to cathepsin-G positive MC. Dual immunofluorescence for tryptase and cathepsin-G demonstrated a colocalization of tryptase and cathepsin-G in skin MC secretory granules. Morphometric evaluation of MC number demostrated that the number of both tryptase- and cathepsin-G-positive MC was significantly higher in cutaneous mastocytosis as compared to normal skin and that in both conditions the number of tryptase-positive MC was significantly higher as compared to the number of cathepsin-G-positive MC. In conclusion, in this study, for the first time we have demonstrated the presence of MC with immunoreactivity to cathepsin-G in human cutaneous mastocytosis, as well as the co-localization of tryptase and cathepsin-G in MC secretory granules
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