Human biomonitoring in the area around the petrochemical site in Gela, Sicily-Italy

Close to the town of Gela (Sicily, Italy) a petrochemical site is operating since 1962. A power station, chemical plants and an oil refiney plant incleded. In 1990 a large area around the site was declared " at high risk of environmental cirsis" in 2000 a subarea was designated " Reclamation Site of National Inerest" Extremely high concentrations of hazardous chemicals have been measured in soil, surface and groundwater, in marine water and sediments. Mortality, hospital discharges and birth defects were reported higher than neighbouring areas and other references

The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders

OBJECTIVES: To test the usefulness of an extended panel of lymphocyte subsets in combination with Oliveira's diagnostic criteria for the identification of autoimmune lymphoproliferative syndrome (ALPS) in children referred to a paediatric rheumatology centre. METHODS: Patients referred from 2015 to 2018 to our rheumatology unit for an autoimmune or autoinflammatory condition were retrospectively analysed. Oliveira's required criteria [chronic lymphoproliferation and elevated double-negative T (DNT)] were applied as first screening. Flow cytometry study included double-negative CD4-CD8-TCR\u3b1\u3b2+ T lymphocytes (DNT), CD25+CD3+, HLA-DR+CD3+ T cells, B220+ T cells and CD27+ B cells. Data were analysed with a univariate logistic regression analysis, followed by a multivariate analysis. Sensitivity and specificity of the Oliveira's required criteria were calculated. RESULTS: A total of 264 patients were included in the study and classified as: (i) autoimmune diseases (n\u2009=\u200926); (ii) juvenile idiopathic arthritis (JIA) (35); (iii) monogenic systemic autoinflammatory disease (27); (iv) periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (100); (v) systemic undefined recurrent fever (45); (vi) undetermined-systemic autoinflammatory disease (14); or (vii) ALPS (17). Oliveira's required criteria displayed a sensitivity of 100% and specificity of 79%. When compared with other diseases the TCR\u3b1\u3b2+B220+ lymphocytes were significantly increased in ALPS patients. The multivariate analysis revealed five clinical/laboratory parameters positively associated to ALPS: splenomegaly, female gender, arthralgia, elevated DNT and TCR\u3b1\u3b2+B220+ lymphocytes. CONCLUSIONS: Oliveira's required criteria are useful for the early suspicion of ALPS. TCR\u3b1\u3b2+B220+ lymphocytes should be added in the diagnostic work-up of patients referred to the paediatric rheumatology unit for a suspected autoimmune or autoinflammatory condition, providing a relevant support in the early diagnosis of ALPS

Patologie respiratorie tra i bambini in eta’ prescolare : analisi geografica del primo ricovero, Pisa, 1998-2004

Obiettivo. Evidenziare la eventuale presenza di cluster spaziale di casi per patologie respiratorie tra i bambini in eta prescolare, residenti a Pisa negli anni 1998-2004. L’analisi presentata costituisce il primo stadio di uno studio piu ampio che vuole esaminare l’eventuale associazione tra l’insorgenza di patologie respiratorie nei bambini e la residenza in strade o zone con elevati livelli di inquinamento dell’aria. Metodi. I dati provengono dal Sistema Epidemiologico Integrato (SEI), realizzato a Pisa, che include dati anagrafici, sanitari, ambientali georeferenziati. I casi sono costituiti dai bambini di eta compresa tra i 7 giorni (per escludere i ricoveri per patologie perinatali) e i 1825 giorni (5 anni). Successivamente e stato selezionato il primo ricovero che presentava come diagnosi principale una patologia respiratoria. Per ogni caso sono stati selezionati 5 controlli appaiati per sesso, classe di eta e stesso anno di ricovero. I controlli sono stati selezionati tra i bambini residenti mai ricoverati. Cause esaminate (ICD.9): “Malattie dell’apparato respiratorio” (460-519), “Sintomi relativi all’apparato respiratorio ed altri sintomi toracici”, (786) escludendo le “Altre malattie dell’apparato respiratorio” (470-478) e la “Influenza”. (487). Per la individuazione dei cluster e stato utilizzato il metodo SaTScan, di Kulldorff (1997) su tutto il territorio del Comune di Pisa, le analisi sono state condotte anche per sesso e per due classi di eta’(0-2 e 3-5 anni) Risultati. Il dataset dell’analisi e costituito da 535 casi e 2675 controlli. 396 casi sono sotto i due anni di eta e 139 sono maschi. Il tasso di incidenza del primo ricovero per patologia respiratoria decresce negli anni in tutte le Circoscrizioni, ma nella Circoscrizione 1 (litorale pisano) risulta sempre piu alto rispetto alle altre, nonostante l’andamento della popolazione sia leggermente decrescente. Sono stati identificati i seguenti cluster significativi: Ospedaletto, zona industriale a sud-ovest di Pisa (n. casi 6; RR=6,09; P=0,075; maschi 0-3 anni) e Gagno, nord di Pisa (n. casi 11; RR=4,538; P=0,037; maschi 0-3 anni); Coltano zona agricola a sud di Pisa (n. casi 8; RR=6,314; P=0,007; femmine 0-3 anni); Tirrenia, litorale pisano (n. casi 8; RR=6,076; P=0,009; maschi + femmine di 3-5 anni).Sono stati esplorati gli aspetti socio-economici dei cluster identificati, utilizzando i dati ISTAT, con le sezioni di censimento al 2001. Rispetto ai valori medi di Pisa, le aree dei cluster presentano una popolazione con alta % di persone con bassa istruzione, di disoccupati, di famiglie unigenitoriali con figli dipendenti. Coltano e Tirrenia presentano un alto numero di persone per stanza e maggiori mq/ persona (abitazioni datate, con grandi stanze occupate da molte persone). Infine i modelli di diffusione degli inquinanti provenienti dal polo industriale di Livorno, prodotti dall’ARPAT mostrano una diffusione alquanto suggestiva degli inquinanti proprio sulla zona a sud del comune che include anche il litorale. Conclusioni. E’ la prima volta che viene condotto uno studio di questo genere basato sui sistemi elettronici integrati di flussi sanitari e demografici di cui e’ stata completata anche la validazione di qualita. I cluster significativi sono stati individuati nelle zone periferiche mentre nel centro urbano la distribuzione dei ricoveri risulta omogenea, verosimilmente a causa della elevata densita’ di popolazione e la omogenea esposizione ad inquinanti; occorrera quindi effettuare una analisi piu’ mirata. Dovra essere approfondito sia il ruolo di confondimento giocato dai fattori socio-economici sia il ruolo degli andamenti giornalieri degli inquinanti provenienti dal polo di Livorno. I risultati sono interessanti e promettenti, in quanto offrono validi spunti per ulteriori approfondimenti in particolar modo di tipo ambientale

State of health of populations residing in geothermal areas of Tuscany

OBJECTIVE The limited scientific knowledge on relationship between exposure and health effects in relation to geothermal activity motivated an epidemiologic investigation in Tuscan geothermal area. The study aims to describe the health status of populations living in Tuscany municipalities where concessions for exploitation of geothermal resources were granted. DESIGN This is an ecological study, so it is not useful to produce evidence to sustain a judgment on the cause-effect link. The major limits of this type of study are the use of the residence at municipal level as a proxy of exposure to both environmental and socioeconomic factors and the use of aggregated data of health outcomes that can lead to the well-known ecological fallacy. SETTING AND PARTICIPANTS Sixteen municipalities were included in the study area: eight are part of the so-called “traditional” geothermal area, defined as Northern Geothermal Area (NGA) and eight located in the Amiata Mountain defined as Southern Geothermal Area (SGA). In 2000-2006, the average resident population in the overall area was approximately 43,000 inhabitants. Thirty one geothermal power plants were active, with a production capacity of 811 MW, 5 of them with 88 MW located in the SGA. Statistical analyses on the entire geothermal area, NGA and SGA subareas, and the sixteen municipalities were performed. MAIN OUTCOME MEASURES MORTALITY DATA were obtained from Tuscany Regional Mortality Registry for the 1971-2006 period, analysing 60 causes of death, of interest for population health status or consistent with “Project SENTIERI” criteria. HOSPITAL DISCHARGE RECORDS of residents in Tuscany Region in 2004-2006, anywhere admitted to hospital, were analyzed considering only the main diagnosis, excluding repeated admissions for the same cause. The causes taken into account are the same analysed for mortality were considered. Age-standardized mortality rates (TSDM) and the temporal trends of TSDM for four periods (1971-1979, 1980- 1989, 1990-1999, 2000-2006) were computed. Age-standardized mortality/hospitalization ratios (SMR/SHR), with and without adjustment for the deprivation index based on 2001 census data, were calculated: mortality in the years 2000-2006 and hospitalization in 2004-2006. The expected number of events were computed using rates of residents in neighbouring municipalities (municipalities included in 50 km radius circle centred on the study area). Bayesian estimates of mortality/hospitalization ratios (BMR/BHR) at municipal level only and relating maps of the Bayesian risk estimators were elaborated. CONGENITAL MALFORMATIONS (MC) were analysed using data from Tuscan Registry of Birth Defect in 1992-2006 period, relative to outcomes of pregnancies in women resident in the municipalities of study area, wherever the birth or termination of pregnancy occurred. The ratio between observed and expected cases (O/A), with expected defined according to regional rate, were calculated and O/A Bayesian estimates (BMR) are showed only at municipal level . The low weight and the males/females ratio at birth were analysed using data from Tuscany Birth Certificates, covering period 2001-2007, excluding biths occurred in facilities outside Tuscany Region. For Low birth weight ( 64 years). Among females resident in SGA a mortality excess from digestive system diseases was observed (72 observed, 55 expected). The hospitalization in the overall Geothermal Area did not show any excess for all causes and all tumours in both genders. Statistically significant excesses for hospital admission from stomach cancer among males (49 observed, 38 expected) and females (42 observed, 28 expected), and from lymphohematopoietic tumours among females, particularly from lymphatic leukaemia (15 observed, 5 expected), were observed. As mortality analysis highlighted, also hospital admissions by geothermal areas and gender showed a worst picture in SGA than in NGA. In the latter, a significant excess of hospital admissions from all causes among females (1,357 observed, 1,284 expected) but not among males (1,193 observed, 1,141 expected) and an excess - close to statistical significance - from all tumours only among females (297 observed; 272 expected) were observed. Furthermore, statistically significant excesses of hospital admissions from digestive system diseases in both genders (M: 392 observed, 350 expected; F: 300 observed, 268 expected), from dementias (16 observed, 8 expected) and from lymphhematopoietic cancers among females, particularly from lymphatic leukaemia (9 observed, 2 expected), were observed. In the SGA, statistically significant excesses of hospital admissions for stomach cancer (M: 32 observed, 21 expected, not significant after adjusting by DI; F: 29 observed, 18 expected), for respiratory diseases (M: 408 observed, 351 expected; F: 339 observed, 277 expected) and for renal failure (M: 61 observed, 41 expected; F: 52 observed, 34 expected) were observed in both genders. Among males, statistically significant excesses of hospital admissions for liver cancer (23 observed, 14 expected), Parkinson’s disease (10 observed, 4 expected) and genitourinary diseases (312 observed, 282 expected) emerged; among females statistically significant excesses of hospital admissions for oesophagus cancer (4 observed, 1 expected), for circulatory disorders of the brain (235 observed, 209 expected), for chronic-obstructive pulmonary disease (70 observed, 42 expected), and an excess close to the statistical significance for cancer of the central nervous system (22 observed, 14 expected) was observed. Congenital malformations results, which take into account births and pregnancy interruption after prenatal anomaly diagnosis show a statistically significant excess of urogenital anomalies in the SGA. Analysis of low birth weight, preterm birth and ratio O/A in males, showed no deviations from the regional references. CONCLUSIONS Overall this descriptive epidemiological study showed that the health status picture of Geothermal Area is not dissimilar from the one observed in neighbouring communities, with the exception of a worst general mortality among males. However, some mortality and hospitalization excesses are worth of note, most evident in the geothermal area of Amiata (SGA) compared to that of the province of Pisa (NGA). The overall results, showing more critical traits for males and a substantial non alignment of mortality and hospitalization, are suggestive of an etiological role of occupational exposures or individual lifestyles, even if effects of environmental factors cannot be excluded. Among the causes of death and hospitalization which showed excesses, acute respiratory diseases, with mortality excess among females in the SGA, circulatory disorders of the brain, with mortality excess among females in the NGA, respiratory system diseases and renal failure, with hospitalization excess in the SGA in both genders, are proposed for further insights. In fact, these excesses observed in areas characterized by documented presence of pollutants of different sources and great concern of the local communities, reinforced the indications for improving environmental quality and environment and health monitoring. Recommendations to the regional and local authorities for ad hoc epidemiological investigations and public health interventions were released

Aggiornamento del profilo di mortalità nel sin di Massa e Carrara

Introduzione. Il SIN di Massa e Carrara si caratterizza per pressioni ambientali derivanti da impianti industriali di diverso tipo, la maggior parte chiusi negli anni Ottanta. Dagli studi sono emersi eccessi di mortalità per numerose cause, ponendo quest’area tra i SIN a maggiore criticità (SENTIERI 2011). Obiettivi. Fornire un quadro aggiornato dellamortalità della popolazione residente nell’area diMassa Carrara. Metodi. E’ stata confrontata lamortalità dei periodi 1995-2000 e 2001-2006. Sono state analizzate le cause principali di morte e quelle suggerite da SENTIERI come associabili alle esposizioni ambientali specifiche dell’area. Sono stati calcolati i tassi di mortalità standardizzati conmetodo diretto della popolazione residente nell’area, nella regione, nei comuni appartenenti alla stessa classe di deprivazione socioeconomica (più deprivata). Per i confronti tra periodi e tra aree sono stati calcolati i rapporti tra i tassi standardizzati (SRR) e gli intervalli di confidenza al 95%. Risultati. Lamortalità aMassa Carrara nel 2001-2006 risulta in sostanziale decremento rispetto al periodo precedente. Emergono SRR in difetto significativo, in entrambi i sessi, per la mortalità generale, le malattie del sistema circolatorio e del digerente; tra gli uomini, per tutti i tumori emalattie polmonari croniche.Non emergono incrementi dimortalità significativi,ma si evidenzia un incremento per tumore della pleura tra le donne. Tra gli uomini, nel 2001-2006 l’SRR rispetto alla regione risulta in eccesso significativo per mortalità generale, tutti i tumori, sistema circolatorio, apparato respiratorio (acute e croniche), tumore del polmone, della pleura e in particolare del fegato (SRR 2,46; IC95% 2,03-2,98); tali eccessi sono confermati dal confronto con l’area simil-deprivata a eccezione del sistema circolatorio e del tumore pleurico; il differenziale di mortalità rispetto al periodo precedente diminuisce soprattutto per lemalattie dell’apparato digerente, genito-urinario e per il tumore della pleura, aumenta per il tumore del fegato e il linfoma non-Hodgkin. Nelle donne, eccessi di mortalità rispetto a quella regionale si riscontrano permortalità generale, apparato respiratorio, apparato genito-urinario, malattie respiratorie acute, tumore del fegato e del sistema linfoematopoietico; rispetto all’area con pari deprivazione si confermano solo gli eccessi per le cause respiratorie. Conclusioni.Mentre lamortalità generale nell’area diMassaCarraramostra un sostanziale decremento, permangono eccessi per alcune cause già evidenziati nei periodi precedenti: si osserva un aumento del rischio dimortalità per tumore epatico, soprattutto negli uomini; le malattie dell’apparato digerente hanno segnato un importante decremento.Uno studio sulla coorte dei residenti dal 1990 è in corso

Failure of anti Interleukin-1 \u3b2 monoclonal antibody in the treatment of recurrent pericarditis in two children

Background: Recurrent pericarditis (RP) is a complication (15-30%) of acute pericarditis with an unknown etiology. Treatment regimen consists of a combination of non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine, with the addition of corticosteroids in resistant or intolerant cases. In the last decade anakinra was shown as an effective treatment in patients with colchicine resistant and steroid-dependent RP, initially in anecdotal reports in children and more recently in a randomized trial. Canakinumab is a monoclonal antibody selectively blocking IL-1\u3b2 and its use is only anecdotally reported to treat pericarditis. We report two pediatric patients with refractory recurrent pericarditis, who presented an optimal response to anakinra treatment but prompt relapse after switch to canakinumab. Case presentation: The first patient is a girl with Recurrent Pericarditis started in April 2015, after heart surgery. NSAIDs and oral steroids were started, with prompt relapse after steroid suspension. The child showed a steroid-dependent RP; anakinra was therefore started with excellent response, but discontinued after 2 weeks for local reactions. In July 2016 therapy with canakinumab was started. She experienced four relapses during canakinumab therapy despite dosage increase and steroid treatment. In January 2018 a procedure of desensitization from anakinra was performed, successfully. Anakinra as monotherapy is currently ongoing, without any sign of flare. The second patient is a girl with an idiopathic RP, who showed an initial benefit from NSAIDs and colchicine. However, 10 days after the first episode a relapse occurred and therapy with anakinra was established. Two months later, while being in complete remission, anakinra was replaced with canakinumab due to patient's poor compliance to daily injections. She experienced a relapse requiring steroids 10 days after the first canakinumab injection. Anakinra was subsequently re-started with complete remission, persisting after 24 months follow-up. Conclusions: We describe two cases of failure of the treatment with anti-IL-1\u3b2 monoclonal antibodies in steroid- dependent idiopathic RP. This anecdotal and preliminary observation suggests a different efficacy of the two IL-1 blockers in the management of RP and support a possible pivotal role of IL-1\u3b1 in the pathogenesis of this condition

The Effect of temperature, rainfall, and light conditions on hair cortisol concentrations in newborn foals

The aim of this study was to investigate the possible effects of environmental factors such as temperature, rainfall, and light conditions on hair cortisol concentrations in foals during the perinatal period. The study, performed during three consecutive foaling seasons from January to July, enrolled 219 foals from one farm. Hair samples were collected from each foal immediately after birth and at 30days of age, and the samples were analyzed by radioimmunoassay to measure the cortisol concentrations. The mean cortisol concentration of hair collected at 30days of age was significantly (P < .01) lower than that found at birth, but none of the evaluated environmental factors (temperature, rainfall, or day length) influenced the hair cortisol concentrations. \ua9 2014 Elsevier Inc