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1.
Orphanet J Rare Dis ; 19(1): 271, 2024 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-39020342

RESUMO

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder characterized by PIG-A mutations, leading to glycophosphatidylinositol (GPI)-anchored proteins deficiency that triggers hemolysis - a hallmark of the disease. PNH diagnostics is based on high-sensitivity multicolor flow cytometry (MFC), enabling to detect even small populations of PNH cells. In this single-center, retrospective study, we aimed to characterize a cohort of PNH clone-positive patients first time screened from January 1st, 2013 until December 31st, 2022 with MFC according to International Clinical Cytometry Society PNH Consensus Guidelines. RESULTS: Out of 2790 first-time screened individuals, the presence of PNH clone in neutrophils was detected in 322 patients, including 49 children and 273 adults. Annual incidence was stable at a median of 31 patients (14 and 19 with clone sizes ≤ 1% and > 1%, respectively), with a decline in number of patients with clone sizes > 1% observed in 2020, potentially influenced by the COVID-19 pandemic. The most common screening indications were aplastic anemia and other cytopenias. CONCLUSIONS: A significant underrepresentation of hemolytic patients was observed as compared to the published cohorts suggesting that these patients are missed in diagnostic process and classic PNH remains underdiagnosed in Poland.


Assuntos
Citometria de Fluxo , Hemoglobinúria Paroxística , Hemoglobinúria Paroxística/diagnóstico , Hemoglobinúria Paroxística/epidemiologia , Humanos , Polônia/epidemiologia , Citometria de Fluxo/métodos , Masculino , Adulto , Feminino , Estudos Retrospectivos , Criança , Adolescente , Pessoa de Meia-Idade , Adulto Jovem , Pré-Escolar , Idoso , COVID-19/epidemiologia , COVID-19/diagnóstico , Lactente
2.
Pol Merkur Lekarski ; 32(187): 50-4, 2012 Jan.
Artigo em Polonês | MEDLINE | ID: mdl-22400181

RESUMO

Flavonoids are plant components of the diet. Their daily intake is about 1 g. In Poland the main source of flavonoids is tea, apples and onions. These compounds are characterized by a variety of biological activities and pharmacological effects. Especially important for human health are their antioxidant properties. This paper presents the current state of knowledge on the role and importance of flavonoids in the prevention of the initiation and development of cardiovascular diseases. Epidemiological observations, clinical research, in vitro and in vivo experimental studies clearly show a protective effect of flavonoids on the cardiovascular system. Their beneficial effects on the cardiovascular system involve the inhibition of low density lipoprotein (LDL) oxidation, the reduction of blood vessels permeability, antiplatelet and anti-inflammatory activity, and hypotensive and direct vasodilator action. Biological potential of flavonoids (especially the antioxidant activity) indicates the possibility of their use in primary and secondary prevention of atherosclerosis and its clinical consequences (heart attack, stroke, and others) as a safe potential therapeutic agents.


Assuntos
Antioxidantes/uso terapêutico , Doenças Cardiovasculares/tratamento farmacológico , Flavonoides/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Anti-Hipertensivos/uso terapêutico , Aterosclerose/tratamento farmacológico , Aterosclerose/etiologia , Aterosclerose/prevenção & controle , Vasos Sanguíneos/metabolismo , Doenças Cardiovasculares/complicações , Doenças Cardiovasculares/metabolismo , Humanos , Lipoproteínas LDL/metabolismo , Oxirredução , Vasodilatadores/uso terapêutico
3.
Pol Merkur Lekarski ; 33(198): 364-9, 2012 Dec.
Artigo em Polonês | MEDLINE | ID: mdl-23437710

RESUMO

On the basis of previous in vitro, in vivo and epidemiological studies was found that flavonoids among their many biological and pharmacological properties have a great anticancer potential. High intake of foods rich in these compounds, mainly vegetables and fruits is associated with a low rate of cancer in humans. Flavonoids may act at different stages of carcinogenesis: initiation, promotion and progression. The complex mechanism of antitumor action include: antioxidant activity, carcinogens inactivation, antiproliferation, block of the cell cycle, induction of apoptosis and differentiation, inhibition of angiogenesis and elimination of drug resistance. With multidirectional action in carcinogenesis and targeting its action only on cancer cells, it is suggested that these compounds may give a new perspective of their use in prevention and therapy of cancer.


Assuntos
Anticarcinógenos/farmacologia , Flavonoides/farmacologia , Neoplasias/tratamento farmacológico , Animais , Apoptose/efeitos dos fármacos , Ciclo Celular/efeitos dos fármacos , Diferenciação Celular/efeitos dos fármacos , Humanos , Neovascularização Patológica/tratamento farmacológico
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