Evidence for Three Distinct Classes of Phenotype Severity in Beta-Thalassaemia

Background: Classification of phenotype severity in patients with beta-thalassaemia has so far relied mainly on expert opinion using parameters of genotype, clinical features at diagnosis, and transfusion requirement. The aim of this study was to use a large dataset of patients with beta-thalassaemia and evaluate a classification system based on onset variables agreed on by an international expert group, including age at diagnosis, at first transfusion, and at first iron chelation. Methods: A retrospective dataset of 7910 patients with homozygous or compound heterozygous beta-thalassaemia was used and subjected to cluster and classification analysis starting with the onset variables. Result…

A retrospective follow up study on maternal age and infant mortality in two Sicilian districts

Abstract Background Infant mortality rate (IMR) is a key public health indicator. Maternal age is a well-known determinant of pregnancy and delivery complications and of infant morbidity and mortality. In Italy the Infant Mortality Rate was 3.7/1000 during 2005, lower than the average IMR for the European Union (4.94/1000). Sicily is the Italian region with the highest IMR, 5/1000, and neonatal mortality rate (NMR), 3.8/1000, with substantial variation among its nine districts. The present study compared a high IMR/NMR district (Messina) with a low IMR/NMR district (Palermo) during the period 2004-2006 to evaluate potential determinants of the IMRs' differences between the two districts and…

Diabetic foot ulcers: Retrospective comparative analysis from Sicily between two eras

Aim The aim of this study was to analyze changes in the incidence, management and mortality of DFU in Sicilian Type 2 diabetic patients hospitalized between two eras, i.e. 2008–2013 and 2014–2019. Methods We compared the two eras, era1: 2008–13, era2: 2014–19. In era 1, n = 149, and in era 2, n = 181 patients were retrospectively enrolled. Results In the population hospitalized for DFU in 2008–2013, 59.1% of males and 40.9% of females died, whilst in 2014–2019 65.9% of males and 34.1% of females died. Moderate chronic kidney disease (CKD) was significantly higher in patients that had died than in ones that were alive (33% vs. 43%, p < 0.001), just as CKD was severe (14.5% vs. 4%, p <…

Developing and validating a novel multisource comorbidity score from administrative data: a large population-based cohort study from Italy

ObjectiveTo develop and validate a novel comorbidity score (multisource comorbidity score (MCS)) predictive of mortality, hospital admissions and healthcare costs using multiple source information from the administrative Italian National Health System (NHS) databases.MethodsAn index of 34 variables (measured from inpatient diagnoses and outpatient drug prescriptions within 2 years before baseline) independently predicting 1-year mortality in a sample of 500 000 individuals aged 50 years or older randomly selected from the NHS beneficiaries of the Italian region of Lombardy (training set) was developed. The corresponding weights were assigned from the regression coefficients of a Weibull sur…

Allattamento al seno in Sicilia: analisi della prevalenza, delle disuguaglianze di contesto e di altri fattori di rischio associati

OBJECTIVES: to describe the prevalence of breastfeeding in Sicily Region (Southern Italy) and to analyze the socioeconomic status and other major risk factors on the attitudes towards exclusive breastfeeding.DESIGN: information on the practice of breastfeeding in Sicily have been collected from a Regional survey on Sudden Infant Death Syndrome (SIDS). An index of socioeconomic position was created by using data from the 2011 Census of the Italian National Institute of Statistics.SETTING AND PARTICIPANTS: in May 2015, a questionnaire was distributed to all mothers accessing to immunization services in Sicily for the first vaccination of their children. Two hundred seventy-three (273) vaccina…

A complication risk score to evaluate clinical severity of thalassaemia syndromes

The thalassaemia syndromes (TS) show different phenotype severity. Developing a reliable, practical and global tool to determine disease severity and tailor treatment would be of great value. Overall, 7910 patients were analysed with the aim of constructing a complication risk score (CoRS) to evaluate the probability of developing one or more complications. Nine independent variables were included in the investigation as predictors. Logistic regression models were used for Group A [transfusion-dependent thalassaemia (TDT)], Group B [transfused non-TDT (NTDT)] and Group C (non-transfused NTDT). Statistically significant predictors included age (years), haemoglobin levels, hepatic transaminas…

Development of a Severity Score System for Thalassemia Syndromes

Introduction Thalassemia Syndromes (TS) are a group of inherited haemoglobin disorders characterized by different phenotype severity falling among heterozygote state, no transfusion dependent thalassemia (NTDT) and transfusion dependent Thalassemia (TDT) (Graffeo et al, 2018; Taher & Saliba, 2017). Several factors, independently by genotype and globin chain unbalance, modulate the severity of ineffective erythropoiesis (Rivella et al, 2015). Considering the complexity of this pathophysiology, one tool to evaluate patients on an individual basis is needed. The aim of this study was to develop a severity scoring system with a view to initiate timely interventions that would prevent any fu…

Socio-economic deprivation and COVID-19 infection: a Bayesian spatial modelling approach

Il presente articolo ha l’obiettivo di analizzare l’effetto della deprivazione socio-economica sull’incidenza da COVID-19 a livello sub-comunale. Grazie alla disponibilit`a di informazioni sui tassi di incidenza mensili da COVID-19 a livello di sezione di censimento per i due comuni di Palermo e Catania (Italia), viene pro- posto l’utilizzo di un modello spaziale Bayesiano con distribuzione binomiale zero- inflated. I risultati mostrano un’associazione tra livelli di deprivazione e incidenza da COVID-19 nei due comuni, controllando per la struttura spaziale delle unit`a areali considerate. Alla luce dei risultati, si rendono necessarie azioni di politica sanitaria focalizzando gli intervent…

The impact of polypharmacy and drug interactions among the elderly population in Western Sicily, Italy.

Aim: Primary endpoint was to report polypharmacy distribution in the general population vs ≥65 years old people and to examine the frequency of drug–drug interactions (DDIs) in the Health Local Unit of Palermo, Italy, in relationship with patients’ age. Methods: Drug prescription data for the year 2014 were extracted from the database of the Local Health Unit of Palermo Province, Italy. Patients were divided into five age groups (0–13, 14–64, 65–69, 70–74, and ≥75 year old). The detection of potential DDIs in polypharmacy profiles was performed with NavFarma software (Infologic srl, Padova, Italia), with DDI classification provided by tool Micromedex Drug Reax (Truven Health Analitics, Mich…

Stroke incidence and case fatality: a 9-year prospective population-based study in an elderly population of Bagheria, Italy

Abstract Background The incidence of stroke in high-income countries has been on the decline; however, few epidemiological surveys have been conducted in recent years to specifically estimate the incidence along with outcome of stroke, in Italy. This study aimed to examine the incidence and case fatality rates of stroke in an elderly Italian population. Methods A cohort of 2200 people > 65 years was randomly stratified from the total elderly population of Bagheria, Italy. A 9-year prospective population-based study was performed (19,800 person/years). Results We identified 112 first-ever strokes, 53 females and 59 males: 82 (73.1%) ischemic, 13(11.6%) intracerebral haemorrhages, 6 (5.35%…

Primary HBB gene mutation severity and long-term outcomes in a global cohort of β-thalassaemia

In β-thalassaemia, the severity of inherited β-globin gene mutations determines the severity of the clinical phenotype at presentation and subsequent transfusion requirements. However, data on associated long-term outcomes remain limited. We analysed data from 2109 β-thalassaemia patients with available genotypes in a global database. Genotype severity was grouped as β0 /β0 , β0 /β+ , β+ /β+ , β0 /β++ , β+ /β++ , and β++ /β++ . Patients were followed from birth until death or loss to follow-up. The median follow-up time was 34·1 years. Mortality and multiple morbidity outcomes were analyzed through five different stratification models of genotype severity groups. Interestingly, β0 and β+ mu…

First detection of SARS-CoV-2 A.23.1 sub-lineage in migrants arriving to Italy via the Mediterranean Sea and public health implications

Socio-economic determinants of inter-regional mobility in patients with cancer

This paper presents an investigation of inter-regional mobility in patients with cancer. Thanks to the availability of geocoded information on patients’ place of residence, the effect of socio-economic status and of other individual characteristics on inter-regional mobility is analysed through multi-level logit models. The extent of patient’s mobility, and its dependence on socio-economic status raises equity issues, as well as regional policy considerations.

Survival and causes of death in 2,033 patients with non-transfusion-dependent β-thalassemia

Key-indicators for maternity hospitals and newborn readmission in Sicily

This paper proposes a composite indicator for the classification of maternity units, which takes into account for the different dimensions of service delivery, as potential predictors of health outcomes. As a measure of outcome, infant readmissions is considered, being a proxy of morbidity. The results highlight that after controlling for risk factors of the newborn, and for the presence of neonatal intensive unit, infants born in lower level hospitals show readmission rates higher than infants born in higher level hospitals.

[A composite indicator for maternity hospital classification].

OBJECTIVES: to propose a composite indicator for maternity hospital classification. DESIGN: descriptive analysis of maternity hospitals through a composite indicator and analysis of its association with infant readmissions within 30 days from the childbirth. SETTING AND PARTICIPANTS: 56 maternity units in Sicily, accounting for 44.436 newborns in 2014. MAIN OUTCOME MEASURES: infant readmission rates within 30 days from the childbirth. RESULTS: low-level hospitals show higher infant readmission rates (odds ratio: 1.3) which may be seen as a signal of inappropriateness of maternity care. CONCLUSIONS: the proposed indicator allows for a classification of maternity hospitals taking into account…

Are diabetes and its medications risk factors for the development of COVID-19? Data from a population-based study in Sicily

Abstract Background and aims Diabetes mellitus (DM) has been associated with higher incidence of severe cases of COVID-19 in hospitalized patients, but it is unknown whether DM is a risk factor for the overall COVID-19 incidence. The aim of present study was to investigate whether there is an association of DM with COVID-19 prevalence and case fatality, and between different DM medications and risk for COVID-19 infection and death. Methods and results retrospective observational study on all SARS-CoV-2 positive (SARS-CoV-2+) cases and deaths in Sicily up to 2020, May 14th. No difference in COVID-19 prevalence was found between people with and without DM (RR 0.92 [0.79–1.09]). Case fatality …

Malaria da importazione in Sicilia: scenario epidemiologico e indicazioni di prevenzione

This study analysed cases of imported malaria notified to the District Health Units of Palermo from 1998 to 2014. The aim was to assess epidemiological differences among cases, related to their reasons for travel. Eighty one cases were reported: 83% developed symptoms following a trip to Africa while 17% had travelled to Asia. Seventy-three percent of cases had travelled to visit family or friends in malaria-endemic countries (Visiting Friends and Relatives: VFRs), confirming the need for preventive measures targeted towards this at-risk population group.

Risk of mortality from anemia and iron overload in nontransfusion-dependent β-thalassemia

β-Thalassemia heterozygote state detrimentally affects health expectation.

Background: Thalassemia minor (Tm) individuals, are generally considered healthy. However, the prognosis of Tm individuals has not been extensively studied. The aim of this study was to evaluate the prognosis of Tm versus controls without β-thalassemia carrier state. Methods: A total of 26,006 individuals seeking thalassemia screening at the AOOR Villa Sofia-V. Cervello, Palermo (Italy) were retrospectively studied. Logistic penalised regression model was used to estimate risk of potential complications and survival techniques were used to study mortality. Results: We identified a total of 4943 Tm and 21,063 controls. Tm was associated with significantly higher risks of hospitalisation for …

The Management of Health Hazards Related to Municipal Solid Waste on Fire in Europe: An Environmental Justice Issue?

Landfilling should be the last option in an integrated Municipal Solid Waste (MSW) management approach. In the European Union (EU), the policy framework to protect the environment and the public health against the impact of health hazards related to urban solid waste management has been consistently implemented in recent decades. A growing interest in the negative impact of fires in waste landfills on the environment and people’s health was reported in some European countries. In Italy, an increasing occurrence of arsons in MSW and landfills has been reported in recent years. During the summer of 2012, a multi-site arson occurred in the Palermo Municipal solid waste landfill of Bellolampo (…

Three Distinct Groups of Phenotype Severity in Beta-Thalassemia

Background Thalassemia Syndromes (TS) are commonly classified as transfusion-dependent-thalassemia (TDT) or non-transfusion-dependent thalassemia (NTDT) at diagnosis on the basis of requirement for lifelong regular transfusion therapy for survival. However, data from observational studies and expert opinion suggest that these categories may reflect a wide spectrum rather than a dichotomy, and may actually be interchangeable at many parts of the disease journey. Thus, an evaluation of alternate clusters to classify TS patients remains of merit. Aims The aim of this study was to cluster TS patients on the basis of possible clinical indicators of phenotype severity (IPhS) using suitable algori…

Do emissions from landfill fires affect pregnancy outcomes? A retrospective study after arson at a solid waste facility in Sicily

ObjectivesIn response to public health concern about effects of arson at solid waste management plants in July 2012, we analysed vital statistics data to evaluate any potential effect on pregnancies at different gestational ages of pollutants emitted from the landfill on fire.SettingA community living near the largest landfill plant in Sicily.ParticipantsThe study group comprised 551 births, live births and stillbirths from pregnancies of mothers residing in the extra-urban exposed area, conceived during a 40 week period during which the highest fire’s peak might have influenced pregnancy.Primary and secondary outcome measuresBirth outcomes (gestational age <37 and <32 weeks, low birt…

Salvate Eva in Sicilia: indagine conoscitiva 2016 sull’adesione allo screening organizzato del cervicocarcinoma.

In Sicilia, a fronte di un incremento dell’estensione dello screening organizzato del cervicocarcinoma, non corrisponde un aumento dell’adesione. Nei mesi di gennaio-giugno 2016 è stata condotta un’indagine trasversale somministrando un questionario standardizzato a un campione di 365 donne, per descrivere le ragioni del fenomeno e promuovere interventi mirati. Per le variabili di atteggiamento e conoscenza è stato utilizzato l’Health Belief Model, che ha esplorato la percezione della donna della suscettibilità (rischio di sviluppare il cancro), della gravità della patologia e dei benefici derivanti dal pap-test. Dai risultati emerge che il 66,6% delle intervistate ha effettuato un pap- tes…