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Background: A shift from a standard to a personalized prophylaxis has been increasingly adopted in patients with severe haemophilia A (SHA). This approach has raised the likelihood of a significant variability in the prophylactic approaches and the relative Factor VIII (FVIII) consumptions. The aim of our study was to assess the treatment variability of SHA patients without inhibitors and on prophylaxis regimen in Italy., Material and Methods: Data reported in the National Registry of Congenital Coagulopathies (NRCC) were analysed to assess treatment distribution within SHA patients without inhibitors, focussing on FVIII consumption in 2017, associated with prophylaxis regimen. The analysis was stratified based on age groups and Italian regions to describe the variability of FVIII consumption in Italy., Results: In 2017, the Registry reported the therapeutic plans of 1068 SHA patients without inhibitors on prophylaxis. The mean (95% CI) individual consumption ranges from 123 127 IU (99 736-146 518) in the age group 0-6 years to 345 000 IU (336 000-354 000) in the age group >20 years. A significant FVIII consumption variability was identified within the adult population. Regions with less than 50 patients reported the higher variability in mean FVIII consumption per patient-year within the different age groups. Similar difference in FVIII consumption variability was reported also in the age groups comparing "low," "middle" and "high" patient volume regions., Discussion: A reliable estimation of FVIII consumption for patients' treatment is necessary to manage and plan the appropriate budget and keep treatment's costs affordable. However, without the implementation of a methodology aiming to assess the overall value produced by these FVIII consumptions, the scenario will keep driven by FVIII consumptions, its costs and the budget available. An effort by haemophilic community, haemophilia treatment centres and institutions is required to develop and share this cultural shift in improving haemophilia management and assessment., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The experience of third-generation tyrosine kinase inhibitor ponatinib treatment in Philadelphia chromosome-positive acute lymphoblastic leukaemia (Ph'+ ALL) patients post-allogeneic transplantation is limited. We retrospectively collected data on 25 Ph'+ ALL patients who were started on ponatinib after allogeneic transplantation between July 2015 and July 2019 from nine transplantation centers in Italy. Ponatinib was given in prophylaxis in five (20%), as pre-emptive treatment in seven (28%), and as salvage therapy in thirteen (52%) patients. It was combined with donor leukocyte infusions in ten patients. Half of the patients (12/25) harbored T315I mutation of BCR/ABL1, while in the remaining mutational analysis was negative or not performed. Among the 20 patients who received ponatinib as pre-emptive/salvage treatment, complete molecular response was achieved in 15 (75%) patients. Estimated overall survival at 2-year post-initiation of treatment in the whole cohort was 65% (respectively 60%, 60%, and 78% for the prophylaxis, pre-emptive, and salvage therapy groups). In patients with T315I-positive mutational status, the estimated 2-year survival was 40%. Fourteen patients (56%) experienced toxicity, requiring temporary or definitive suspension of treatment. In conclusion, treatment of Ph'+ ALL patients with ponatinib after transplantation is effective, although the question of adequate drug dose and treatment duration remains unanswered.

Objectives: We examined the association between travellers' characteristics, compliance with pre-travel recommendations and health problems., Methods: Volunteer travellers were enrolled and data collected using a questionnaire between 30-60 days after returning home. We analyzed the associations through bivariate and multivariate models., Results: Of the 468 enrolled travelers, 68% consumed raw food and 81% food containing milk and/or eggs. 32% consumed street vendor food and 30% drinks containing ice. 24% used the recommended mechanical prophylaxis measures. 46% got sick during and/or after travel (gastrointestinal symptoms most frequently). Factors predisposing to health problems were female gender, youth/middle age, intermediate travel duration and profession. The American continent and staying in hostels and tents were significantly associated with febrile illness. Street vendor food was significantly associated with skin reactions., Conclusions: Adherence to behavioral recommendations remains low. Travellers must be informed of health risks during and after travel., (Copyright © 2021. Published by Elsevier Masson SAS.)

Background: Systemic photoprotection (i.e., administration of substances such as nicotinamide, carotenoids, and vitamin D) may be important to reduce photocarcinogenesis or to support long-term protection against UV irradiation. Clinical trials showed that oral nicotinamide is effective in reducing the onset of new nonmelanoma skin cancers (NMSCs), while other oral photoprotectors failed to achieve the reduction of new melanoma or NMSC formation in humans. The aim of this study was to summarize the current state of knowledge of systemic photoprotection and to evaluate the knowledge and attitude of dermatologists regarding these treatments., Methods: The survey was conducted on a sample of dermatologists recruited according to a snowball sampling procedure. The questionnaire consisted of a first part asking for characteristics of the participant and a second part with 12 specific questions on their knowledge about systemic photoprotection, particularly their knowledge of astaxanthin, β-carotene, nicotinamide, and vitamin D3., Results: One hundred eight dermatologists answered the survey. Most of them (85.2%) stated that oral photoprotectors have a role in the prevention of skin cancer, and responses mainly mentioned nicotinamide. More than half of them (54.6%) had prescribed all the considered oral photoprotectors, but the majority of them had prescribed nicotinamide, mainly for 2 to 3 months during summer, almost invariably ( n = 106) associated with topical photoprotectors. Most dermatologists (>80%) were aware of scientific publications demonstrating an effect of systemic photoprotectors on NMSC., Conclusions: Most Italian dermatologists have positive views on oral photoprotection in skin cancer and are aware of the demonstrated potential of nicotinamide in the prevention of NMSCs.

In patients with coronavirus disease 2019 (COVID-19), the prevalence of pre-existing cardiovascular diseases is elevated. Moreover, various features, also including pro-thrombotic status, further predispose these patients to increased risk of ischemic cardiovascular events. Thus, the identification of optimal antithrombotic strategies in terms of the risk-benefit ratio and outcome improvement in this setting is crucial. However, debated issues on antithrombotic therapies in patients with COVID-19 are multiple and relevant. In this article, we provide ten questions and answers on risk stratification and antiplatelet/anticoagulant treatments in patients at risk of/with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection based on the scientific evidence gathered during the pandemic.

In the attempt to establish definitions and provide shared approaches to breakthrough invasive fungal diseases (br-IFD) in acute myeloid leukemia (AML) patients submitted to intensive chemotherapy and receiving triazoles as mould active primary antifungal prophylaxis (MA-PAP), literature on br-IFD in AML patients receiving triazoles MA-PAP was reviewed and a Consensus Development Conference Project was convened. The following four candidate key-questions were generated and formed the set of questions of the present document: "definition of br-IFD," "diagnostic strategy during MA-PAP to detect br-IFD," "possible causes of MA-PAP failure," "management of br-IFD.", (© The Author(s) 2019. Published by Oxford University Press on behalf of The International Society for Human and Animal Mycology.)

Six cases of serogroup C invasive meningococcal disease were identified in Treviso district, Veneto region, Italy between December 13 and 15, 2007. The afflicted patients were found to have attended the same Latin-dance clubs on the same nights, and chemoprophylaxis was provided to potentially exposed individuals. Despite these efforts, 2 cases caused by the same meningococcal strain subsequently occurred in the same area, without any apparent epidemiological correlation to the initial cases. This may have resulted from a failure to neutralize the meningococcal carrier/s. The root cause analysis method applied to public health emergency preparedness was used to analyze the response to this critical incident. The root cause analysis revealed a need to develop regional guidelines for the classification and management of a meningococcal outbreak and for developing risk-communication strategies that include the identification of appropriate channels of communication for differing segments of the population. (Disaster Med Public Health Preparedness. 2019;13:368-371).

Background and Methods: As a consequence of the rapid evolution of malaria prophylaxis recommendations throughout the world, the Italian Society of Tropical Medicine (SIMET-Società Italiana di Medicina Tropicale) has set up a working group in charge of preparing a new national guideline. Other scientific societies interested in the topic were also involved in the project., Results and Conclusions: The group stated that awareness about malaria risk and characteristics, as well as protection from mosquito bites, are recommended for all travellers visiting malaria-endemic countries. The risk and benefit of malaria chemoprophylaxis must be carefully balanced before prescribing drugs: the disease-related risk must outweigh the possibility of drugs' side effects. As a general rule, malaria pills are the first choice for travellers to high-risk areas, such as sub-Saharan Africa, Eastern India, Myanmar, Eastern Indonesia, Papua New Guinea and, with some limitations, South-East Asia, and the Amazon part of Venezuela, Guyana and French Guyana. However, several other factors, such as itinerary, season, duration of trip, availability of insect bite protection, pre-existing conditions and compliance, must be taken into account. In low-risk areas, stand-by emergency treatment is the first option. In minimal-risk areas and in Plasmodium vivax areas, a prompt diagnosis only is advised (Central America, South America outside the Amazon basin, Middle East, China, Thailand, Nepal). Recommendations may be modified when particular groups of travellers are concerned, such as long-term residents, visiting friends and relatives, patients with pre-existing conditions, pregnant women and children.

Background: Malaria prevention policy is different among coalition troops in Afghanistan, ranging from the combined use of suppressive and terminal chemoprophylaxis to the absence of any prophylactic regimen. The objective of this study was to assess the compliance with malaria prevention measures and the risk of malaria among Italian troops in Afghanistan., Methods: Target population was the cohort of 32,500 army soldiers deployed in Afghanistan, 2002 to 2011; eligible subjects were the 21,900 soldiers stationed in endemic areas, who were prescribed mefloquine chemoprophylaxis. Adherence to chemoprophylaxis was assessed by a cross-sectional study in a volunteer sample of 5,773 (26.4%) of eligible subjects. The risk of malaria was assessed by detecting malaria cases in the target population., Results: Mefloquine chemoprophylaxis was administered to 4,123 (71.4%) of the 5,773 enrolled soldiers and 3,575 (86.7%) of these took it regularly; however, compliance dropped from 80.9% (2,592/3,202) in 2002 to 2006 to 59.5% (1,531/2,571) in 2007 to 2011 (p < 0.01). Adverse events were reported by 875 (21.2%) of the 4,123 soldiers taking mefloquine, but caused irregularity or interruption of chemoprophylaxis only in 48 (1.2%) and 113 (2.7%) subjects, respectively. No serious adverse events were reported. No malaria cases occurred in Afghanistan, and one Plasmodium vivax case was reported in Italy, yielding an incidence rate of 3.24 cases per 10,000 person-months of exposure (1/3,091) during the transmission season of 2003., Conclusions: In spite of the decreasing compliance with chemoprophylaxis, suggesting a low perception of the risk of malaria, this study confirmed the good tolerability of mefloquine in the military. The risk of malaria for Italian troops in Afghanistan was very low, and chemoprophylaxis was suspended in 2012. A similar policy may be adopted by the generality of International Security Assistance Force troops, and any chemoprophylaxis may be restricted to soldiers stationing in areas where the risk of malaria is substantial., (© 2013 International Society of Travel Medicine.)

Background: Group B Streptococcus (GBS) is a leading cause of neonatal bacterial infections. Early-onset infections have decreased in recent years but, despite considerable efforts poured into prevention, cases continue to occur., Objectives: To analyze trends and identify determining factors for the persistence of the GBS infections. To evaluate the impact of antenatal screening and intrapartum chemoprophylaxis on the clinical presentation of the infection., Methods: A prospective cohort, population-based study has been ongoing in Emilia-Romagna (Italy) since 2003. Invasive GBS infections, observed between 2003 and 2008 in infants aged < 7 days were analyzed., Results: Among 214,120 live births, 61 early-infections were observed. Fourteen infants (23.0%) were born preterm. Among 47 infants who were delivered at term, 28 were born to mothers who had no risk factors and 7 were born to mothers who had none other than GBS colonization. Forty-one women at term had been screened prenatally; among them, only 10 were documented as GBS culture-positive.Disease severity was highest in infants at lower gestational ages, but most meningitis cases were observed in term infants born to mothers who were GBS culture-negative at screening.Nine newborns had culture-proven infection despite having received intrapartum antibiotics. They were born to mothers with > or =1 obstetrical risk factors and 5 mothers had been treated during labor with macrolides., Conclusion: Most infections presented in infants whose mothers had been screened as GBS culture-negative. Missed opportunities for prevention contributed more than prophylaxis failures to the early-onset disease burden.

In recent years, prospective studies have been conducted to assess the role of prophylaxis and treatment of invasive fungal diseases (IFD) in allogeneic hematopoietic stem cell transplantation (allo-HSCT). Although results of these studies have been encouraging, they have been unable to generate a consensus for optimal prophylaxis and treatment of IFD in the complex scenario of allo-HSCT. A consensus process was undertaken to describe and evaluate current information and practice regarding key questions on IFD management in allo-HSCT recipients; these questions were selected according to the criterion of relevance by group discussion. The Panel produced recommendations for risk stratification, prophylaxis, monitoring, and therapy of IFD and identified top priority issues for further investigation. The definition of the level of risk for IFD associated with the various types and phases of transplantation and the implementation of surveillance and diagnostic strategies are the critical determinants of the antifungal prophylactic and therapeutic approach for allo-HSCT recipients.

Objectives: Candida spp. are the most important non-bacterial pathogens in critically ill patients. The aim of this study was to evaluate trends in the incidence of candidaemia and the distribution of Candida albicans and non-albicans over a 9 year period (1999-2007), and to assess their relationship with fluconazole use., Methods: This was an interventional cross-over study. Patients admitted to the intensive care unit (ICU) who developed a clinically and microbiologically documented candidaemia were analysed. Fluconazole was used as prophylaxis in critically ill patients until 2002; from January 2003 infectious disease consultants strongly discouraged its use. Fluconazole use, measured as defined daily dose per 1000 patient-days, was calculated. The main outcome of the study is the evaluation of the restriction policy in terms of change in fluconazole use and in incidence of candidaemia., Results: During the 108 month period (January 1999-December 2007), a total of 213 episodes of candidaemia (average incidence 1.42 episodes/10 000 patient-days/year, range 0.36-3.02 episodes) were recorded in a mixed medical and surgical ICU in Italy. C. albicans was the most prevalent isolated species (n = 98, 46%); non-albicans (n = 115, 54%) were mainly represented by Candida parapsilosis (n = 46, 22%) and by Candida glabrata (n = 28, 13%). Segmented regression analysis of the interrupted time series showed that a change in the fluconazole prophylactic strategy resulted in a significant reduction in fluconazole use from the second semester of 2002. A dramatic decrease in the incidence of fungaemia due to C. non-albicans was observed from the second semester of 2003 (intervention effect in the second semester of 2007: -2.31/10 000 patient-days); minor changes in the incidence of C. albicans fungaemia emerged (intervention effect in the second semester of 2007: -0.23/10 000 patient-days)., Conclusions: The study showed a clear correlation between fluconazole use control and decreasing incidence of non-albicans candidaemia. Even if fluconazole remains a first-line treatment option in several cases of invasive candidiasis, its prophylactic use should be carefully evaluated.

Patients with ulcerative colitis are at greater risk of developing colorectal cancer than the general population. Prophylactic colectomy is the only certain method of avoiding this risk. To avoid indiscriminate surgery, efforts have focused on colonoscopic surveillance in order to select for prophylactic surgery only patients at ultra-high risk. However this policy has not always been proven to reduce mortality for colorectal cancer in these patients. Recently, there has been growing interest in chemoprevention using folate. Folate maintains the normal DNA methylation process and steady-state levels of DNA precursors. These properties indicate that folate has potential as chemopreventive agent.