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1.
Mediterr J Hematol Infect Dis ; 15(1): e2023005, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36660351

RESUMO

Background: The health-related quality of life (HRQoL) of people with hemophilia (PWH) is an important issue, especially considering people suffering from chronic diseases beyond hemophilia. The principal aim of this study was to investigate the presence and relevance of psychological symptoms, both internalizing and externalizing, lifestyle, and HRQoL in a group of Italian PWH with chronic bloodborne co-infections and comorbidities. Furthermore, the research describes the association between psychological aspects and the impact of disease-related characteristics (type of hemophilia, presence of co-infections, and comorbidities) on them. Methods: Seventy patients (mean age 46.77±11.3), 64 with severe hemophilia A (Factor VIII: C < 1 IU/dL) and 6 with severe hemophilia B (Factor IX <1 IU/dL), were consecutively recruited from seven Hemophilia Centers in Italy of Italian Association of Hemophilia Centers (AICE). In order to assess psychological symptoms, HRQoL, and lifestyle, three psychological questionnaires were administered (the SCL-90-R, SF-36, and PSQ, respectively). Results: A general decline in the quality of life and an increase in the tendency to adopt a lifestyle characterized by hyperactivity emerged. Inverse correlations were found between HRQoL and psychological distress. Although the SCL-90-R did not reveal symptoms above the clinical cut-off, co-infections significantly increased anxiety, depression, somatizations, paranoia, and social withdrawal. Lastly, HRQoL is impaired by co-infections as well as comorbidities. Conclusion: Our preliminary results must be confirmed to deepen the findings between mental health and hemophilia.

2.
Dig Liver Dis ; 52(9): 1002-1010, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32576522

RESUMO

BACKGROUND: Gut microbiota exerts a crucial role in gastrointestinal (GI) and extra-intestinal (EI) disorders. In this context, Akkermansia muciniphila is pivotal for the maintenance of host health and has been correlated with several disorders. AIM: To explore the potential role of A. muciniphila as common dysbiotic marker linked to the disease status. METHODS: A cohort of patients affected by GI and EI disorders was enrolled and compared to healthy controls (CTRLs). A targeted-metagenomics approach combined to unsupervised cluster and machine learning (ML) analyses provided microbiota signatures. RESULTS: Microbiota composition was associated to disease phenotype, therapies, diet and anthropometric features, identifying phenotype and therapies as the most impacting variables on microbiota ecology. Unsupervised cluster analyses identified one cluster composed by the majority of patients. DESeq2 algorithm identified ten microbial discriminatory features of patients and CTRLs clusters. Among these microbes, Akkermansia muciniphila resulted the discriminating ML node between patients and CTRLs, independently of specific GI/EI disease or confounding effects. A. muciniphila decrease represented a transversal signature of gut microbiota alteration, showing also an inverse correlation with α-diversity. CONCLUSION: Overall, A. muciniphila decline may have a crucial role in affecting microbial ecology and in discriminating patients from healthy subjects. Its grading may be considered as a gut dysbiosis feature associated to disease-related microbiota profile.


Assuntos
Disbiose/fisiopatologia , Fezes/microbiologia , Microbioma Gastrointestinal/fisiologia , Mucosa Intestinal/metabolismo , Akkermansia/fisiologia , Estudos de Casos e Controles , Disbiose/metabolismo , Gastroenteropatias/metabolismo , Gastroenteropatias/microbiologia , Humanos , Mucosa Intestinal/microbiologia , Permeabilidade , Fenótipo
3.
Blood Transfus ; 18(2): 143-151, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31657709

RESUMO

Emicizumab has been approved in several countries for regular prophylaxis in patients with congenital haemophilia A and FVIII inhibitors because it substantially reduces their bleeding risk and improves quality of life. However, although significantly less frequent, some breakthrough bleeds may still occur while on emicizumab, requiring treatment with bypassing or other haemostatic agents. Thrombotic complications have been reported with the associated use of activated prothrombin complex concentrates. In addition, when surgery/invasive procedures are needed while on emicizumab, their management requires multidisciplinary competences and direct supervision by experts in the use of this agent. Given this, and in order to expand the current knowledge on the use of emicizumab and concomitant haemostatic agents, and reduce the risk of complications in this setting, the Italian Association of Haemophilia Centres (AICE) here provides guidance on the management of breakthrough bleeds and surgery in emergency situations in patients with haemophilia A and inhibitors on emicizumab prophylaxis. This paper has been shared with other National Scientific Societies involved in the field.


Assuntos
Anticorpos Biespecíficos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Hemofilia A/prevenção & controle , Hemostáticos/uso terapêutico , Anticorpos Biespecíficos/efeitos adversos , Anticorpos Monoclonais Humanizados/efeitos adversos , Fator VIII/antagonistas & inibidores , Hemorragia/prevenção & controle , Hemostáticos/efeitos adversos , Humanos , Itália , Qualidade de Vida
4.
Front Med (Lausanne) ; 6: 261, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31850352

RESUMO

The current interest in recombinant factor VIII (rFVIII) products stems from the fact that they offer a technological solution to prolonging the half-life of and reducing the risk of formation of alloantibodies (inhibitors) against FVIII in treated patients with hemophilia A (HA). The Italian health care system has authorized the use of a wide range of rFVIII concentrates of the first, second, and third generation, as well as new innovative rFVIII preparates with an extended half-life (EHL) (Kogenate FS®-Bayer, belonging to the second generation and replaced since 2017 by a product consisting of the same modified molecule; because it is only available until the end of the current year, it will not be considered in this review). Some of these products have unique pharmacodynamic and pharmacokinetic (PK) profiles, including an EHL. The first-generation full-length rFVIII (FL-rFVIII), octocog alfa (Recombinate® Baxter/BIOVIIIx), although the oldest rFVIII product, has several desirable features. Third-generation products include two modified octocog alfa molecules (Advate®, Shire; Kovaltry®, Bayer) as well as the B domain-deleted rFVIII (BDD-rFVIII) moroctocog alfa (ReFacto®-Pfizer). The B domain-truncated (BDT-rFVIII) turoctocog alfa (NovoEight®, Novo Nordisk), the BDD-rFVIII simoctocog alfa (Nuwiq®, Kedrion), the single-chain BDT-rVIII lonoctocog alfa (Afstyla®, CSL Behring), and the BDD-rFVIIIFc efmoroctocog alfa (Elocta®, Sobi-Biogen) are new, innovative products. Simoctocog alfa, because its peculiarities, is considered a fourth-generation rFVIII concentrate. Turoctocog alfa, simoctocog alfa, and lonoctocog alfa have a high affinity for von Willebrand factor (vWF) that reduces renal clearance and prolongs the half-life of rFVIII. Efmoroctocog alfa, a first-in-class rFVIII-Fc fusion protein (rFVIIIFc), has a half-life 1.5-1.8 times longer than that of conventional plasma-derived FVIII (pd-rFVIII) and other rFVIII products. Clinical studies have evaluated the efficacy, safety, and inhibitor development of all these innovative concentrates in both previously treated (PTPs) and untreated patients (PUPs). This review considers the rFVIII products that are indicated for the treatment of patients with severe HA, focusing on those that are commercially available in Italy. Their PK characteristics, immunogenicity, and clinical benefits are discussed and compared.

5.
Proc Natl Acad Sci U S A ; 116(38): 18777-18782, 2019 09 17.
Artigo em Inglês | MEDLINE | ID: mdl-31451632

RESUMO

As meticulously observed and recorded by Darwin, the leaves of the carnivorous plant Drosera capensis L. slowly fold around insects trapped on their sticky surface in order to ensure their digestion. While the biochemical signaling driving leaf closure has been associated with plant growth hormones, how mechanical forces actuate the process is still unknown. Here, we combine experimental tests of leaf mechanics with quantitative measurements of the leaf microstructure and biochemistry to demonstrate that the closure mechanism is programmed into the cellular architecture of D. capensis leaves, which converts a homogeneous biochemical signal into an asymmetric response. Inspired by the leaf closure mechanism, we devise and test a mechanical metamaterial, which curls under homogeneous mechanical stimuli. This kind of metamaterial could find possible applications as a component in soft robotics and provides an example of bio-inspired design.


Assuntos
Materiais Biomiméticos/química , Drosera/fisiologia , Fenômenos Fisiológicos Vegetais , Fenômenos Biomecânicos , Parede Celular/fisiologia , Módulo de Elasticidade , Ácidos Indolacéticos/metabolismo , Movimento , Folhas de Planta/anatomia & histologia , Folhas de Planta/crescimento & desenvolvimento , Folhas de Planta/metabolismo , Folhas de Planta/fisiologia
6.
Blood Transfus ; 16(2): 209-214, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-28287377

RESUMO

BACKGROUND: Direct oral anticoagulants (DOAC) have been shown to be non-inferior to traditional vitamin K antagonists in preventing stroke and arterial thromboembolism in patients with non-valvular atrial fibrillation. Nevertheless, it is mandatory to record side effects and individual adherence to DOAC treatment. MATERIALS AND METHODS: In this single-centre experience, patients with non-valvular atrial fibrillation were prospectively observed after switching from a vitamin K antagonist to dabigatran or rivaroxaban. The efficacy, safety, and tolerability of the novel treatment, and adherence to it, were evaluated over a period of 1 year. Clinical data were integrated with records of haemorrhagic and non-haemorrhagic complications. All the subjects were given an anonymous self-report questionnaire on the degree of their adherence/satisfaction with the treatment. RESULTS: Of 196 patients with non-valvular atrial fibrillation (median age, 78.5 years) who switched from a vitamin K antagonist to DOAC, 178 completed the 1-year follow up, of whom 87 were given dabigatran and 91 rivaroxaban. The efficacy of the two DOAC was similar. Patients given dabigatran had a higher frequency (n=32) of non-haemorrhagic complications (OR: 3.3; 95% CI: 1.7-7.8), which occurred earlier (HR: 6.1; 95% CI: 3.0-12.6) than those (n=7) recorded in subjects on rivaroxaban. The degree of satisfaction with therapy was higher among patients on rivaroxaban (mean score 9.1, SD 1.0) than among those on dabigatran (mean score 8.7; SD 0.9; p=0.01). DISCUSSION: Overall, in this experience, DOAC were shown to be effective, safe alternatives to vitamin K antagonists. Nevertheless, compared with rivaroxaban, dabigatran resulted in a higher rate and earlier occurrence of non-haemorrhagic events, and a lower satisfaction score.


Assuntos
Fibrilação Atrial/tratamento farmacológico , Dabigatrana , Adesão à Medicação , Rivaroxabana , Inquéritos e Questionários , Administração Oral , Idoso , Idoso de 80 Anos ou mais , Dabigatrana/administração & dosagem , Dabigatrana/efeitos adversos , Feminino , Seguimentos , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Rivaroxabana/administração & dosagem , Rivaroxabana/efeitos adversos
7.
J Med Case Rep ; 10(1): 276, 2016 Oct 05.
Artigo em Inglês | MEDLINE | ID: mdl-27716428

RESUMO

BACKGROUND: Evidence from clinical trials shows rivaroxaban to be effective for the treatment of deep vein thrombosis. Switching to rivaroxaban following failure of indirect anticoagulants in deep vein thrombosis has not been demonstrated in a real-life setting. CASE PRESENTATION: A 43-year-old white woman was switched from warfarin to rivaroxaban for the treatment of thrombosis of her right common femoral vein after saphenectomy. The reason for the switch was due to the instability of anti-coagulation therapy with vitamin K antagonists over a period of 3 months during which she did not reach the "therapeutic range" of prothrombin time-international normalized ratio. The ineffectiveness of the conventional oral anticoagulant was confirmed by persistence of moderate-high values of fibrin D dimers (780 ng/ml) and by residual vein thrombosis at an ultrasound examination. Objectively, her right leg appeared to be still edematous and warm and pain was elicited by deep palpation. Rivaroxaban was administered after warfarin discontinuation (prothrombin time-international normalized ratio = 1.43) at a dosage of 15 mg every 12 hours for 3 weeks, followed by 20 mg once daily for 3 months. After this period, her objective symptoms significantly improved, with reduction of edema of her lower limb and pain relief. Her fibrin D dimer values returned to normal (210 ng/ml). An ultrasound showed recanalization of the obstructed venous segment. CONCLUSIONS: In this case report, a switch to rivaroxaban from warfarin was shown to be effective and safe for the treatment of postoperative deep vein thrombosis, whereas standard oral anticoagulation therapy, which required dose adjustments over a period of 3 months, was not able to stabilize the therapeutic range of prothrombin time-international normalized ratio nor improve our patient's outcome.


Assuntos
Anticoagulantes/administração & dosagem , Extremidade Inferior/irrigação sanguínea , Complicações Pós-Operatórias/tratamento farmacológico , Rivaroxabana/uso terapêutico , Trombose Venosa/tratamento farmacológico , Varfarina/administração & dosagem , Adulto , Anticoagulantes/efeitos adversos , Esquema de Medicação , Feminino , Humanos , Resultado do Tratamento , Varfarina/efeitos adversos
8.
Blood ; 123(26): 4037-44, 2014 Jun 26.
Artigo em Inglês | MEDLINE | ID: mdl-24786773

RESUMO

Analyses of the bleeding tendency by means of the bleeding score (BS) have been proposed until now to confirm diagnosis but not to predict clinical outcomes in patients with inherited von Willebrand disease (VWD). We prospectively followed up, for 1 year, 796 Italian patients with different types of VWD to determine whether the previous BS of European VWD1 is useful to predict the occurrence of spontaneous bleeds severe enough to require replacement therapy with desmopressin (DDAVP) and/or von Willebrand factor (VWF)/factor VIII concentrates. Among the 796 patients included, 75 (9.4%) needed treatment of 232 spontaneous bleeding events. BS >10 and VWF:ristocetin cofactor activity <10 U/dL were associated with the risk of bleeding, but only a BS >10 remained highly associated in a multivariable Cox proportional hazard model (adjusted hazard ratio: 7.27 [95% confidence interval, 3.83-13.83]). Although the bleeding event-free survival was different in VWD types, only a BS >10 could predict for each type which patient had bleeding events severe enough to require treatment with DDAVP and/or concentrates. Therefore, BS can be considered a simple predictor of clinical outcomes of VWD and may identify patients needing intensive therapeutic regimens.


Assuntos
Desamino Arginina Vasopressina/administração & dosagem , Hemorragia , Hemostáticos/administração & dosagem , Índice de Gravidade de Doença , Doenças de von Willebrand , Fator de von Willebrand/administração & dosagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Intervalo Livre de Doença , Fator VIII , Feminino , Seguimentos , Hemorragia/tratamento farmacológico , Hemorragia/epidemiologia , Hemorragia/mortalidade , Hemorragia/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Risco , Taxa de Sobrevida , Doenças de von Willebrand/sangue , Doenças de von Willebrand/tratamento farmacológico , Doenças de von Willebrand/mortalidade , Doenças de von Willebrand/patologia
9.
Haemophilia ; 20(2): e128-35, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24533954

RESUMO

Despite great advances in haemophilia care in the last 20 years, a number of questions on haemophilia therapy remain unanswered. These debated issues primarily involve the choice of the product type (plasma-derived vs. recombinant) for patients with different characteristics: specifically, if they were infected by blood-borne virus infections, and if they bear high or low risk of inhibitor development. In addition, the most appropriate treatment regimen in non-inhibitor and inhibitor patients compel physicians operating at the haemophilia treatment centres (HTCs) to take important therapeutic decisions, which are often based on their personal clinical experience rather than on evidence-based recommendations from published literature data. To know the opinion on the most controversial aspects in haemophilia care of Italian expert physicians, who are responsible for common clinical practice and therapeutic decisions, we have conducted a survey among the Directors of HTCs affiliated to the Italian Association of Haemophilia Centres (AICE). A questionnaire, consisting of 19 questions covering the most important topics related to haemophilia treatment, was sent to the Directors of all 52 Italian HTCs. Forty Directors out of 52 (76.9%) responded, accounting for the large majority of HTCs affiliated to the AICE throughout Italy. The results of this survey provide for the first time a picture of the attitudes towards clotting factor concentrate use and product selection of clinicians working at Italian HTCs.


Assuntos
Hemofilia A/epidemiologia , Hospitais Especializados , Corpo Clínico Hospitalar , Padrões de Prática Médica/estatística & dados numéricos , Fatores de Coagulação Sanguínea/uso terapêutico , Pesquisas sobre Atenção à Saúde , Hemofilia A/tratamento farmacológico , Humanos , Itália , Inquéritos e Questionários
10.
Thromb Res ; 129(5): e177-84, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-21930293

RESUMO

BACKGROUND: The diagnosis and treatment of disseminated intravascular coagulation (DIC) remain extremely controversial. PURPOSE: The Italian Society for Thrombosis and Haemostasis commissioned a project to develop clinical practice guidelines for the diagnosis and treatment of DIC. METHODS: Key questions about the diagnosis and treatment of DIC were formulated by a multidisciplinary working group consisting of experts in clinical medicine and research. After a systematic review and discussion of the literature, recommendations were formulated and graded according to the supporting evidence. In the absence of evidence, evidence of low quality, or contradictory evidence, a formal consensus method was used to issue clinical recommendations. RESULTS AND CONCLUSIONS: In suspected DIC, we suggest the use of the diagnostic scores ISTH (grade C), JMHW (grade C) or JAAM (grade D) over stand alone tests. The cornerstone of the management of DIC remains the treatment of the underlying triggering disease. We do not suggest the use of antithrombin (grade D), dermatan sulphate (grade D), gabexate (grade D), recombinant factor VIIa (grade D), activated protein C (grade D), thrombomodulin (grade B). The use of unfractionated heparin or low-molecular-weight heparin is not suggested except for thromboembombolic prophylaxis in patients a high risk who do not have active bleeding (grade D). In patients with severe sepsis/septic shock and DIC we suggest the use of human recombinant activated protein C (grade D). In patients with DIC and active bleeding we suggest the use of transfusion therapy (platelets, plasma, cryoprecipitate) (grade D).


Assuntos
Neoplasias/sangue , Neoplasias/terapia , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/terapia , Humanos , Itália , Neoplasias/patologia , Resultado do Tratamento , Tromboembolia Venosa/patologia
11.
Semin Thromb Hemost ; 37(5): 511-21, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22102194

RESUMO

Von Willebrand disease (VWD) is the most common inherited bleeding disorder and is due to quantitative and/or qualitative defects of von Willebrand factor (VWF). Despite the improved knowledge of the disease, detailed data on VWD types requiring specific treatments have not been reported thus far. To determine the number and types of VWD requiring therapy with desmopressin (DDAVP) and/or VWF/FVIII concentrates in Italy, a national registry on VWD (RENAWI) was organized. Only 16 of 48 centers included VWD in the RENAWI with diagnoses performed locally. Patients with uncertain results were retested by two expert laboratories using multimeric analysis and mutations of the VWF gene. A total of 1234 of 1529 (81%) cases satisfied the inclusion criteria and could be classified as VWD1 (63%), VWD2A (7%), VWD2B (6%), VWD2M (18%), VWD2N (1%), and VWD3 (5%). VWD types were also confirmed by DNA analyses and occur in young adults (83%), mainly in women (58%). Mucosal bleedings (32 to 57%) are more frequent than hematomas (13%) or hemarthrosis (6%). Most patients were exposed to an infusion trial with desmopressin (DDAVP) and found responsive with the following rates: VWD1 (69%), VWD2A (26%), VWD2M (29%), and VWD2N (71%). However, DDAVP was not always used to manage bleeding in all responsive patients and VWF/FVIII concentrates were given instead of or together with DDAVP in VWD1 (30%), VWD2A (84%), VWD2B (62%), VWD2M (63%), VWD2N (30%), and VWD3 (91%). Data of the RENAWI showed that correct VWD identification and classification might be difficult in many Italian centers. Therefore, evidence-based studies should be organized only in well-characterized patients tested by laboratories that are expert in the clinical, laboratory, and molecular markers of VWD.


Assuntos
Mutação , Doenças de von Willebrand/tratamento farmacológico , Doenças de von Willebrand/genética , Fator de von Willebrand/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Desamino Arginina Vasopressina/uso terapêutico , Fator VIII/uso terapêutico , Feminino , Frequência do Gene , Hemostáticos/uso terapêutico , Humanos , Lactente , Itália , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem , Doenças de von Willebrand/diagnóstico , Fator de von Willebrand/uso terapêutico
12.
Transfus Med ; 21(4): 280-4, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21733006
13.
Haematologica ; 91(5): 634-9, 2006 May.
Artigo em Inglês | MEDLINE | ID: mdl-16670069

RESUMO

BACKGROUND AND OBJECTIVES: This open-label multicenter trial of solvent/detergent (SD) plasma involving 17 patients with recessively inherited coagulation disorders (one afibrinogenemia, four FV, six combined FV and FVIII, one FX and five FXI deficiencies) evaluated the pharmacokinetics of the deficient factors and hemostatic efficacy. DESIGN AND METHODS: In vivo recovery (IVR) of the deficient coagulation factor was determined in a non-bleeding state in all patients and the mean values for FV, FVIII, FX, FXI and fibrinogen were 1.3, 1.2, 1.5, 1.3 and 1.5 dL/Kg, respectively. The mean plasma half-life of FV, FVIII and FX was 18, 43 and 33 hours, respectively. All patients underwent replacement therapy for elective procedures at risk of bleeding (surgery in 14 cases and vaginal delivery in two patients), except one treated for a central nervous system surgical emergency. RESULTS: Treatment courses with SD plasma were judged fully effective in 13/16 cases (81%). In the remaining three cases, mild bleeding occurred after major surgery in a FV deficient patient with a factor level of 43% and in a FXI deficient patient when factor levels were between 20% and 41%; and after minor surgery in a patient with FV and FVIII deficiency when factor levels were 41% and 18%, respectively. Bleeding was controlled by continuing or increasing treatment with SD plasma. INTERPRETATION AND CONCLUSIONS: These results suggest that, even though the current absolute risk of blood-borne infections associated with fresh-frozen plasma is relatively small, SD plasma should be preferred in patients with recessively inherited coagulation disorders who need replacement therapy when virus-inactivated single-factor concentrates are not available.


Assuntos
Transtornos Hemorrágicos/tratamento farmacológico , Octoxinol/uso terapêutico , Sistema ABO de Grupos Sanguíneos/análise , Adolescente , Adulto , Idoso , Testes de Coagulação Sanguínea , Perda Sanguínea Cirúrgica/prevenção & controle , Parto Obstétrico , Procedimentos Cirúrgicos Eletivos , Feminino , Genes Recessivos , Humanos , Controle de Infecções , Masculino , Pessoa de Meia-Idade , Complicações do Trabalho de Parto/prevenção & controle , Octoxinol/administração & dosagem , Octoxinol/farmacocinética , Plasma , Gravidez , Resultado do Tratamento , Hemorragia Uterina/prevenção & controle
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