RESUMO
Congenital plasminogen (Plg) deficiency leads to the development of ligneous membranes on mucosal surfaces. Here, we report our experience with local and intravenous fresh frozen plasma (FFP). We retrospectively reviewed medical files of 17 patients and their eight first-degree relatives. Conjunctivitis was the main complaint. Thirteen patients were treated both with intravenous and conjunctival FFP. Venous thrombosis did not develop in any. Genetic evaluation revealed heterogeneous mutations as well as polymorphisms. Diagnosis and treatment of Plg deficiency is challenging; topical and intravenous FFP may be an alternative treatment.
Assuntos
Transfusão de Componentes Sanguíneos , Conjuntivite/terapia , Doenças Genéticas Inatas/terapia , Plasma , Plasminogênio/deficiência , Pré-Escolar , Conjuntivite/diagnóstico , Conjuntivite/genética , Feminino , Doenças Genéticas Inatas/diagnóstico , Doenças Genéticas Inatas/genética , Humanos , Lactente , Recém-Nascido , Masculino , Mutação , Polimorfismo GenéticoRESUMO
OBJECTIVE: The present study was designed to determine the effects of tirofiban (Tiro) infusion on angiographic measures, ST-segment resolution, and clinical outcomes in patients with STEMI undergoing PCI. Glycoprotein (GP) IIb/IIIa inhibitors are beneficial in ST-segment elevation myocardial infarction (STEMI) patients undergoing percutaneous coronary intervention (PCI), while the most effective timing of administration is still under investigation. METHODS: A total of 1242 patients (83.0% males, mean (standard deviation; SD) age: 54.7 (10.9) years) with STEMI who underwent primary PCI were included in this retrospective non-randomized study in four groups, composed of no tirofiban infusion [Tiro (-); n=248], tirofiban infusion before PCI (pre-Tiro; n=720), tirofiban infusion during PCI (peri-Tiro; n=50), and tirofiban infusion after PCI (post-Tiro; n=224). In all Tiro (+) patients, bolus administration of Tiro (10 µg/kg) was followed by infusion (0.15 µg/kg/min) for a mean (SD) duration of 22.4±6.8 hours. RESULTS: The pre-PCI Tiro group was associated with the highest percentage of patients with TIMI 3 flow (99.4%; p<0.001), the lowest corrected TIMI frame count [21(18-23.4); p<0.001], the highest percentage of patients with >75% ST-segment resolution (78.1%; p<0.001), and the lowest rate of in-hospital sudden cardiac death and in-hospital all-cause mortality (3.2%, p<0.05, 3.3%, p=0.01). Major bleeding was reported in 18 (1.8%) patients who received tirofiban. CONCLUSION: Use of standard-dose bolus tirofiban in addition to aspirin, high-dose clopidogrel, and unfractionated heparin prior to primary PCI significantly improves myocardial reperfusion, ST-segment resolution, in-hospital mortality rate, and in-hospital sudden cardiac death in patients with STEMI with no increased risk of major bleeding.