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Registri

  • Emanuele Crocetti1

  1. ISPO Toscana, Firenze
Emanuele Crocetti -

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Ricerca effettuata il 19 agosto 2010

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ONCOLOGIA

1. D'alò D, Stracci F, Cassetti T, Scheibel M, Pascucci C, La Rosa F. Recent trends in incidence, mortality and survival after cancer of the female breast and reproductive organs. Umbria, Italy: 1978-2005. Eur J Gynaecol Oncol. 2010;31(2):174-80.
Abstract
This study analyzed the incidence, mortality and survival after cancer of the female breast and reproductive organs in the Umbria region of Italy with the aim of generating hypotheses to explain trends. Mortality data were supplied by ISTAT (1978-1993) and ReNCaM (1994-2005) and incidence (1994-2005) and survival (at 12/31/2007) data by RTUP. Joinpoint regression was applied to evaluate temporal trends of the age-adjusted incidence and mortality rates. Mortality, incidence and relative survival rates were compared with national and international data. The incidence of breast cancer increased up to 2001 and afterwards significantly decreased; mortality rates significantly decreased after 1994. Uterine corpus incidence was practically stable, and decreased over the study period; mortality from all uterine subsites significantly decreased from 1978 onwards. Trends in ovarian cancer incidence and mortality (after 1985) were constant. Trends in occurrence of breast and cervical cancer were linked to population screening of Umbrian women, noting a low compliance by younger females with cervical cancer screening and emphasizing the opportunity of starting breast cancer screening at a younger age. Trends in the incidence of cancer of the uterus and ovary, though unsteady, were probably related to modifications in risk factor exposure. Survival was better for breast and cervical cancers than in the 1978-1982 period and might be due to early diagnosis and progress in therapy.

2. Dal Maso L, Franceschi S, Lise M, Fusco M, Tumino R, Serraino D. Re: Papillary thyroid cancer incidence in the volcanic area of Sicily. J Natl Cancer Inst. 2010 Jun 16;102(12):914-5; author reply 915-6. Epub 2010 May 18. Comment on: J Natl Cancer Inst. 2009 Nov 18;101(22):1575-83.

Breve commento a cura di Emanuele Crocetti
Questo intervento analizza in maniera critica l’articolo di Gabriella Pellegrini et al. (JNCI 2009) che ipotizzava una relazione fra l’elevata incidenza di tumore tiroideo misurata nell’area di Catania e la presenza nell’acqua potabile dell’area di elevate concentrazioni di varie sostanze legate alla natura vulcanica del terreno etneo. Viene messa in dubbio questa ipotesi – suolo vulcanico – a favore di una diversa diffusione e aggressità diagnostica per spiegare i dati catanesi ed anche le differenze - elevate - di incidenza di tumore tiroideo che si osservano in Italia.

3. Mensi C, Sieno C, Bordini L, Dicredico N, Pesatori AC, Riboldi L. [Systematic surveillance of occupational cancer: the Lombardy Sinonasal Cancer Registry] Med Lav. 2010 Jan-Feb;101(1):19-25.
Abstract
BACKGROUND: Sinonasal cancer (SNC) includes uncommon tumours that are characterized by a high occupational etiologic component. For this reason they are an easy target for systematic surveillance by setting up a specific disease registry. The National Registry of these tumours is located at the Italian Institute for Occupational Safety and Prevention (ISPESL) and is based on data from Regional Operating Centres (ROCs). In the Lombardy Region a ROC was established in 2008 at the Clinica del Lavoro "Luigi Devoto" in Milan aimed at making surveillance systematic and thus provide adequate support for scientific research and prevention measures in the high risk work sectors.
OBJECTIVES: To estimate the incidence of SNC, to define different sources of occupational exposure both known (wood, leather, nickel, chromium) and unknown.
METHODS: The Registry collects all the new incident cases of epithelial SNC occurring among residents ofthe Lombardy Region. The Regional Registry is managed in accordance with the specific National Guidelines and uses the experience already acquired with the organizational and operational management of the Regional Mesothelioma Registry.
RESULTS: During the first period of activity (1.1.2008-30.9.2009) 556 cases of suspected SNC were reported to the Registry; only 62 (11.2%) of these actually complied with the inclusion criteria of the Registry and were mainly adenocarcinoma and squamous carcinoma. Occupational exposure to wood or leather dust was ascertained in 50% of cases.
CONCLUSION: Data collected up to now confirm that occupational exposure to wood and leather dusts are the most significant risk factors for SNC. The study of occupational sectors and job tasks in cases with as yet undefined exposure might suggest new etiologic hypotheses.
4. Scarselli A, Di Marzio D, Marinaccio A, Iavicoli S. [The register of exposed workers to carcinogens: legislative framework and data analysis] Med Lav. 2010 Jan-Feb;101(1):9-18.
Abstract
BACKGROUND: On the basis of the law which introduced the registration of occupational exposures to carcinogens (Legislative Decree 626/94), the National Institute for Occupational Safety and Prevention designed and implemented an information system for collecting and recording such information. The Ministry of Health Decree No 155/2007, which established the procedures for record keeping and transmission of registers of exposed workers, regulated the legislative fJamework in this field.
OBJECTIVES: The aim of the study was to illustrate some of the major legislative issues and toprovide summary statistics, after one year of entry into force of this Decree.
METHODS: The main information to record is: the carcinogenic agents used, the type of occupational exposure and data on the environmental measurements. Descriptive statistical analysis were carried out, by sector of economic activity, carcinogen agent and worker's occupation.
RESULTS: As at 31 December 2008 the information recorded, altogether, covered: 6000 firms, 79,000 workers, 164,000 exposures and 100,000 measurements. Most of the exposures occurred in the manufacturing and construction industries and in commercial activities.
CONCLUSIONS: Such surveillance system, established as a result of the institution of exposure registers, makes it possible to plan analytical studies, both for monitoring the effects of exposure, even at low doses, and for assessing the prevention and protection measures. It is hoped that the recent readjustment law (Legislative Decree 81/2008) will promote awareness of all subjects involved in the recording procedures (employers, physicians, local health units, research institutes, etc.), thus increasing the quality and coverage of data transmission.
5. Crocetti E, Ciatto S, Buzzoni C, Zappa M. Prostate cancer incidence rates have started to decrease in central Italy. J Med Screen. 2010;17(1):50-1.
Abstract
The widespread use of prostate-specific antigen (PSA) testing has dramatically changed the epidemiology of prostate cancer. Growing incidence rates have been documented in almost all western countries following the increased usage of PSA screening. In the United States after a period of huge increase in incidence, rates have decreased to values lower than those of the pre-PSA era. Similar changes have been documented also in the area of the Tuscany Cancer Registry, central Italy, where prostate cancer incidence rates doubled from the early 1990s to 2003 and afterwards decreased. This is the first evidence, to our knowledge, of a decline in prostate cancer incidence in Italy following the screening-related increase. PMID: 20356946 [PubMed - indexed for MEDLINE]

Breve commento a cura di Emanuele Crocetti
La disponibilità e la rapida diffusione del test PSA hanno cambiato completamente negli ultimi 15 anni l’epidemiologia del tumore prostatico. L’uso del test è divenuto abituale anche in Italia e questo prima della valutazione della sua efficacia da parte del trial ERSPC che ha sollevato forti perplessità sul costo in termini di sovra diagnosi e sovra trattamento del beneficio di riduzione della mortalità specifica osservato nel braccio attivo (PSA). La riduzione dell’incidenza per tumore della prostata ora segnalata anche in Italia, potrebbe rappresentare l’effetto di saturazione di una sorta di screening di prevalenza spontaneamente condotto nella popolazione.

6. Haupt R, Garaventa A, Gambini C, Parodi S, Cangemi G, Casale F, Viscardi E, Bianchi M, Prete A, Jenkner A, Luksch R, Di Cataldo A, Favre C, D'Angelo P, Zanazzo GA, Arcamone G, Izzi GC, Gigliotti AR, Pastore G, De Bernardi B. Improved survival of children with neuroblastoma between 1979 and 2005: a report of the Italian Neuroblastoma Registry. J Clin Oncol. 2010 May 10;28(14):2331-8. Epub 2010 Mar 29.
Abstract
PURPOSE: To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma.
PATIENTS AND METHODS: The study includes data from 2,216 children (age 0 to 14 years) diagnosed between 1979 and 2005. Overall survival (OS) was analyzed by clinical and biologic features at presentation and periods of diagnosis: 1979 to 1984, 1985 to 1991, 1992 to 1998, and 1999 to 2005. The relative risk of second malignant neoplasm (SMN) was assessed by the standardized incidence ratio (SIR), with the Italian population selected as referent.
RESULTS: Yearly patient accrual increased over time from 58 to 102. Patients age 0 to 17 months represented 45.6% of the total population, and their incidence increased over time from 36.5% to 48.5%. The incidence of stage 1 patients increased over time from 5.8% to 23.2%. A total of 898 patients (40.5%) developed disease progression or relapse, 19 patients developed SMN, and two patients developed myelodysplasia. The cumulative risk of SMN at 20 years was 7.1%, for an SIR of 8.4 (95% CI, 5.1 to 13.2). A total of 858 patients (39%) died (779 of disease, 71 of toxicity, six of SMN, and two of tumor-unrelated surgical complications). Ten-year OS was 55.3% (95% CI, 53.0% to 57.6%) and increased over time from 34.9% to 65.0%; it was significantly better for females and patients age 0 to 17 months at diagnosis, with extra-abdominal primary, and stage 1 and 2 disease. OS improved significantly over time in stage 1 and 3 patients. In patients with stage 4 disease, the improvement occurred between the first and second time cohorts (6.7% v 23.5%), but not afterward.
CONCLUSION: The outcome of children with neuroblastoma has progressively improved. Long-term survivors bear a significant risk of SMN.
7. Pagano L, Caira M, Nosari A, Rossi G, Locatelli F, Viale P, Aversa F; HemaE-Chart Group Italy. Hema e-Chart: Italian Registry for prospective analysis of epidemiology, management and outcome of febrile events in patients with haematological malignancies. J Chemother. 2010 Feb;22(1):20-4.
Abstract
The aim of the study was to create a prospective computerized registry to collect and analyze febrile events, particularly due to fungal infections, in patients with hematological malignancies. A systematic approach that starts from the registration of new diagnosis and complete follow-up can be of help for the study of treatment and evolution of these complications. The software allows several concurrent users to create and manage medical information in a website. Its aim is to improve the speed, quality and integration of information related to subjects with febrile event, ultimately resulting in improving patients' care.Patients included adults and children with acute and chronic myeloid or lymphoid leukemia, Hodgkin's and non-Hodgkin's lymphoma, myelodysplastic syndrome, or multiple myeloma. The registry also included data regarding event onset in hematopoietic stem cell transplants (HSCTs). In order to evaluate the incidence of febrile events, all new diagnoses of hematological malignancy and all HSCTs were reported.The Hema e-Chart can be a very useful network collecting information about febrile events in patients with hematological malignancy and HSCTs. Significant trends and treatment practices are expected to be observed. As enrollment continues, data will be analyzed and published, which will provide valuable information concerning the epidemiology, therapy, and outcome of infectious complications.
8. Scarselli A, Massari S, Binazzi A, Di Marzio D, Scano P, Marinaccio A, Iavicoli S. Italian National Register of Occupational Cancers: data system and findings. J Occup Environ Med. 2010 Mar;52(3):346-53.
Abstract
OBJECTIVE: To analyze data stored in the National Register of Occupational Cancers, set up by law at the Italian Institute for Occupational Safety and Prevention.
METHODS: Statistical analyses were performed on economic activity, cancer site, and carcinogenic agent. Mesothelioma cases were excluded because they were exhaustively recorded and managed in a proper register.
RESULTS: The number of cancers diagnosed between 1995 and 2008 was 936 (92% men). Lung cancer was the most frequently reported neoplasm (58% of cases). The metal industry had the highest reporting percentage (15%), and silica was the most common causative agent (20%).
CONCLUSIONS: Despite some limits and incompleteness pertaining to data flow, the results of this study are in accordance with epidemiological findings on occupational cancer risks. The collection of information on occupational cancers is of primary importance to plan targeted prevention programs.
9. Parodi S, Perfumo C, Garaventa A, Inga A, Mazzocco K, Defferrari R, Tonini GP, Fronza G, Haupt R. MDM2 SNP309 genotype is associated with ferritin and LDH serum levels in children with stage 4 neuroblastoma. Pediatr Blood Cancer. 2010 Aug;55(2):267-72.Comment in: Pediatr Blood Cancer. 2010 Aug;55(2):222-3.
Abstract
BACKGROUND: MDM2 SNP309, characterised by a T-to-G substitution in the MDM2 promoter, is associated with higher gene expression compared to wild type and was recently found to be a negative prognostic factor for patients with stage 4 neuroblastoma (NB), but not for children with localised disease. This polymorphism was not associated with any clinical or genetic tumour characteristics, including MYCN amplification and 1p chromosome deletion.
PROCEDURE: To better define the involvement of MDM2 SNP309 in NB, we explored its association with the main biochemical tumour markers, namely urinary concentrations of vanillyl mandelic acid (VMA) and homovanillic acid (HVA) and blood concentrations of ferritin and lactate dehydrogenase (LDH). A cohort of 497 NB children, enrolled in the Italian Neuroblastoma Registry between January 1985 and December 2005 and previously investigated for the prognostic role of MDM2 SNP309, was considered for this study.
RESULTS: VMA and HVA concentrations as well as HVA/VMA ratio were not affected by the MDM2 SNP309 genotype. Ferritin and LDH concentrations were significantly lower in TT than in TG/GG only in patients with stage 4 disease (P = 0.007 and 0.015, respectively). No association emerged in patients with localised disease. These findings were not affected by confounding from clinical or biological characteristics.
CONCLUSIONS: The association between MDM2 SNP309 and both ferritin and LDH in patients with stage 4 disease confirms the prognostic role of this polymorphism. The results suggest that the MDM2 SNP309 genotype can impact on tumour responses to hypoxia and might play an important role in the alteration of energetic metabolism in NB cells. PMID: 20232446 [PubMed - indexed for MEDLINE]
10. Bouvier AM, Sant M, Verdecchia A, Forman D, Damhuis R, Willem Coebergh J, Crocetti E, Crosignani P, Gafa L, Launoy G, Martinez-Garcia C, Plesko I, Pompe-Kirn V, Rachtan J, Velten M, Vercelli M, Zwierko M, Esteve J, Faivre J. What reasons lie behind long-term survival differences for gastric cancer within Europe? Eur J Cancer. 2010 Apr;46(6):1086-92. Epub 2010 Feb 15.
Abstract
BACKGROUND: Wide geographic variations in survival for gastric cancer in Europe have been reported. The aim of this study was to analyse the effect of stage at diagnosis, treatment and cancer characteristics on long-term survival for gastric cancer in populations covered by cancer registries.
METHODS: We analysed survival in 4620 cases of gastric cancer from 17 European population-based cancer registries from 8 countries. Univariate and multivariate regression of relative survival were performed.
RESULTS: Five-year relative survival varied between 10.6% and 24.0%, while 10-year survival ranged from 7.7% to 23.0%. After adjustment for age and sex, the regional excess hazard ratio (EHR) of death was significantly higher in Ragusa, Granada, Yorkshire, Slovakia, Slovenia and Poland than in France, Northern Italy, The Netherlands and the Basque Country. After further adjustment for surgical resection versus no resection (a proxy of stage), the EHR of death remained significantly higher only in Granada and Yorkshire than in the reference country (France). After adjustment for stage, the EHR was significantly higher only in Yorkshire (EHR: 1.51; 95% confidence interval (CI): 1.29-1.77). The EHR in this area was limited to the first year following diagnosis.
CONCLUSION: Differences across Europe in gastric cancer survival depend to a large extent on differences in stage at diagnosis. However they do not explain all variations. Quality of management and treatment can explain some differences.
11. Lepage C, Sant M, Verdecchia A, Forman D, Esteve J, Faivre J; and the EUROCARE working group. Operative mortality after gastric cancer resection and long-term survival differences across Europe. Br J Surg. 2010 Feb;97(2):235-9.
Abstract
BACKGROUND: Little is known at a population level about operative mortality after surgery for gastric cancer and whether differences between countries can explain differences in long-term survival. This study compared operative mortality recorded by ten cancer registries in seven European countries.
METHODS: Non-conditional logistic regression analysis was performed to estimate the independent effect of the studied factors on mortality within 30 days of surgery. A multivariable survival model was employed with and without operative mortality.
RESULTS: The overall operative mortality rate in 1611 patients studied was 8.9 (range 5.2-16) per cent. Country of residence was a significant prognostic factor in the multivariable analysis. The likelihood of operative mortality was lower in Italy, France and the UK than in the Netherlands, Spain, Slovenia and Poland. Age, type of gastrectomy and stage at diagnosis were also significant factors. Cancer site was not found to be significant in the multivariable analysis. The overall 5-year relative survival rate varied between 42.0 per cent (Italy) and 24 per cent (Poland); after excluding operative mortality, the 5-year survival rate was 44.3 and 28 per cent respectively.
CONCLUSION: Within Europe, the substantial differences in operative mortality after gastrectomy only partly explain marked differences in survival after gastric cancer resection.

12. Brennan B. Pediatric pancreatic tumors: the orphan looking for a home. Pediatr Blood Cancer. 2010 May;54(5):659-60. Comment on: Pediatr Blood Cancer. 2010 May;54(5):675-80.

13. Zanetti R, Tazi MA, Rosso S. New data tells us more about cancer incidence in North Africa. Eur J Cancer. 2010 Feb;46(3):462-6. Epub 2009 Dec 22.
Abstract
Over the last few years, Cancer Registries in North Africa (Morocco, Algeria, Tunisia, Libya and Egypt) increased in number from one to nine, and now covers 13% of the total regional population. Their data can be considered of good or acceptable quality, according to available indicators. The pattern of risk shown by these Registries is quite unique. The total cancer burden in the North African countries is between one third and one half of what is observed in Europe. The overall incidence rate in men (world age standardised, per 100,000) ranges from 86.3 in Sétif, Algeria, to 156.1 in Garbiah, Egypt. The range is similar in women: from 80.3 in Sétif to 164.0 in Algier, both in Algeria. The case mix and the level of rates are quite homogeneous in the countries considered. The most frequent cancers are the same as in Europe (Lung, Breast and Prostate). This pattern completely differs from that of Central and Southern African countries, where infection-related cancers are predominant. The well-known excess risk for nasopharyngeal carcinoma in this area is confirmed, with rates reaching the level of 5.4 in men and 1.9 in women, which is 10 times higher than that in Europe.
14. Luminari S, Cesaretti M, Marcheselli L, Rashid I, Madrigali S, Maiorana A, Federico M. Decreasing incidence of gastric MALT lymphomas in the era of anti-Helicobacter pylori interventions: results from a population-based study on extranodal marginal zone lymphomas. Ann Oncol. 2010 Apr;21(4):855-9. Epub 2009 Oct 22.
Abstract
BACKGROUND: Few studies have been carried out to date that have addressed the epidemiology of extranodal marginal zone lymphomas (EN-MZLs).
PATIENTS AND METHODS: We carried out a population-based study to investigate incidence rates (IRs) and time trends of EN-MZL diagnosed in the province of Modena (Italy) from 1997 to 2007.
RESULTS: One hundred and sixty-five cases were identified from the Modena Cancer Registry that corresponded to an age-standardized IR of 2.3 cases per 100 000. A bimodal distribution of age was shown with the group of young patients mostly represented by males with cutaneous lymphoma. No time trends were observed for the IR; the incidence of gastric mucosa-associated lymphoid tissue(g-MALT) lymphomas (N = 51) markedly declined during the study period, dropping from 1.4 in 1997 to 0.2 in 2002 and then remaining stable until 2007; the calculated annual percent change for g-MALT was -17.0% (95% confidence interval -26.6% to -6.2%). We also observed a significant decrease in the rate of g-MALT associated with Helicobacter pylori (HP) infection from 61% to 17% of patients diagnosed before and after 2002 (P = 0.007; P for trend = 0.016).
CONCLUSION: This population-based study provides new insights into recent changes in the epidemiology of EN-MZL, mainly represented by the sharp reduced incidence of HP-positive g-MALT lymphomas.

EMATOLOGIA

1. Cesaro S, Marsh J, Tridello G, Rovò A, Maury S, Montante B, Masszi T, Van Lint MT, Afanasyev B, Iriondo Atienza A, Bierings M, Carbone C, Doubek M, Lanino E, Sarhan M, Risitano A, Steinerova K, Wahlin A, Pegoraro A, Passweg J. Retrospective survey on the prevalence and outcome of prior autoimmune diseases in patients with aplastic anemia reported to the registry of the European group for blood and marrow transplantation. Acta Haematol. 2010;124(1):19-22. Epub 2010 Jul 6.
Abstract
BACKGROUND: Aplastic anemia (AA) is rarely described after a diagnosis of autoimmune disease (aID). AIMS: To assess the prevalence of prior aID in patients with AA recorded in the registry of the European Group for Blood and Marrow Transplantation (EBMT) and to evaluate treatment and outcome.
METHODS: 1,251 AA patients from 18 EBMT centers were assessed. RESULTS: Fifty patients (4%) were eligible: 22 males and 28 females with a median age of 46 years at the diagnosis of aID and of 51 years at the diagnosis of AA. Information on the treatment of AA was available in 49 patients: 38 received only immunosuppressive therapy (IST), 8 patients underwent hematopoietic stem cell transplantation (HSCT) - 6 as first-line therapy and 2 after failure of IST - whilst 3 patients had a spontaneous recovery. After a median follow-up of 3.19 years, 32 patients were alive, including 7 of the 8 patients who underwent HSCT. Only 6 of 32 patients who were alive at the last follow-up were receiving IST for AA.
CONCLUSIONS: Most cases of AA following aID benefitted from IST or HSCT if a matched donor was available. Further prospective investigation is needed to assess the effects of IST on the outcome of underlying aID.
2. Locasciulli A, Bacigalupo A, Bruno B, Montante B, Marsh J, Tichelli A, Socié G, Passweg J. Hepatitis-associated aplastic anaemia: epidemiology and treatment results obtained in Europe. A report of The EBMT aplastic anaemia working party. Br J Haematol. 2010 Jun;149(6):890-5. Epub 2010 Apr 29.
Abstract
In order to assess the epidemiology of Hepatitis-Associated Aplasia (HAA) and compare treatment outcome of HAA with non-HAA patients, we evaluated 3916 aplastic anaemia patients reported to the European Registry between 1990 and 2007. Year, month, season of diagnosis, type and outcome of first-line therapy were analysed. Prevalence of HAA (n = 214) in Europe was 5%. Compared to non-HAA patients, HAA patients were younger (15 vs. 20 years, P < 0.001), with a male prevalence (68% vs. 58% P = 0.002), and were treated earlier after diagnosis (46 vs. 62 d; P < 0.001). No significant differences were found regarding the year or month of diagnosis. No geographic clusters could be identified. Actuarial survival at 10 years after first-line immunosuppression was 69%, and did not differ according to aetiology. The 10-year actuarial survival after transplantation was 70%, and was comparable in HAA and non-HAA patients, when stratified for age and donor type. In a multivariate Cox analysis, increasing age and delayed treatment were significant negative indicators for survival. In conclusion, the incidence of HAA was 5% and was evenly distributed over time and geographic areas in Europe. Treatment outcome and predictive variables, were comparable in patients with or without HAA. PMID: 20456352 [PubMed - indexed for MEDLINE]
3. Alessandrino EP, Della Porta MG, Bacigalupo A, Malcovati L, Angelucci E, Van Lint MT, Falda M, Onida F, Bernardi M, Guidi S, Lucarelli B, Rambaldi A, Cerretti R, Marenco P, Pioltelli P, Pascutto C, Oneto R, Pirolini L, Fanin R, Bosi A. Prognostic impact of pre-transplantation transfusion history and secondary iron overload in patients with myelodysplastic syndrome undergoing allogeneic stem cell transplantation: a GITMO study. Haematologica. 2010 Mar;95(3):476-84. Epub 2009 Nov 10. Comment in: Haematologica. 2010 Mar;95(3):364-6.
Abstract
BACKGROUND: Transfusion-dependency affects the natural history of myelodysplastic syndromes. Secondary iron overload may concur to this effect. The relative impact of these factors on the outcome of patients with myelodysplastic syndrome receiving allogeneic stem-cell transplantation remains to be clarified.
DESIGN AND METHODS: We retrospectively evaluated the prognostic effect of transfusion history and iron overload on the post-transplantation outcome of 357 patients with myelodysplastic syndrome reported to the Gruppo Italiano Trapianto di Midollo Osseo (GITMO) registry between 1997 and 2007.
RESULTS: Transfusion-dependency was independently associated with reduced overall survival (hazard ratio=1.48, P=0.017) and increased non-relapse mortality (hazard ratio=1.68, P=0.024). The impact of transfusion-dependency was noted only in patients receiving myeloablative conditioning (overall survival: hazard ratio=1.76, P=0.003; non-relapse mortality: hazard ratio=1.70, P=0.02). There was an inverse relationship between transfusion burden and overall survival after transplantation (P=0.022); the outcome was significantly worse in subjects receiving more than 20 red cell units. In multivariate analysis, transfusion-dependency was found to be a risk factor for acute graft-versus-host disease (P=0.04). Among transfusion-dependent patients undergoing myeloablative allogeneic stem cell transplantation, pre-transplantation serum ferritin level had a significant effect on overall survival (P=0.01) and non-relapse mortality (P=0.03). This effect was maintained after adjusting for transfusion burden and duration, suggesting that the negative effect of transfusion history on outcome might be determined at least in part by iron overload.
CONCLUSIONS: Pre-transplantation transfusion history and serum ferritin have significant prognostic value in patients with myelodysplastic syndrome undergoing myeloablative allogeneic stem cell transplantation, inducing a significant increase of non-relapse mortality. These results indicate that transfusion history should be considered in transplantation decision-making in patients with myelodysplastic syndrome.

NEUROLOGIA

1. Beghi E, Logroscino G, Chiò A, Hardiman O, Millul A, Mitchell D, Swingler R, Traynor BJ. Amyotrophic lateral sclerosis, physical exercise, trauma and sports: results of a population-based pilot case-control study. Amyotroph Lateral Scler. 2010 May 3;11(3):289-92.
Abstract
Our objective was to investigate exposure to physical exercise and trauma in patients with amyotrophic lateral sclerosis (ALS) compared to the general population. Previous studies in this area have yielded conflicting results. Using population-based ALS registries from Italy, the UK and Ireland, newly diagnosed patients with definite, probable or possible ALS were enrolled in a case- control study with two age- and sex-matched controls for each patient. Source, intensity and duration of physical activity and history of trauma were recorded. We here present the results of a pilot investigation. We studied 61 patients and 112 controls. Forty-one per cent of cases and 17.0% of controls (p = 0.001) had blue-collar occupations; 13.1% versus 3.6% reported strenuous physical activity at work (p = 0.05). Compared with controls, ALS patients had a longer exposure to work-related (10.7 vs. 7.3 years; p = 0.02) and sport-related physical exercise (9.6 vs. 5.2 years; p = 0.005). Three patients (0 controls) reported professional sports (p = 0.04). Traumatic events were similar. Blue collar occupation (OR 4.27; 95% CI 1.68-10.88) and duration of sport-related physical exercise (OR 1.03; 95% CI 1.00-1.05) were independent variables in multivariate analysis. We concluded that ALS is associated with physical exercise but not with traumatic events. PMID: 20433412 [PubMed - indexed for MEDLINE]
2. Moruzzi S, Pesenti-Gritti P, Brescianini S, Salemi M, Battaglia M, Ogliari A. Clumsiness and psychopathology: causation or shared etiology? A twin study with the CBCL 6-18 questionnaire in a general school-age population sample. Hum Mov Sci. 2010 Apr;29(2):326-38. Epub 2010 Mar 24.
Abstract
In a sample of 398 twin pairs aged 8-17 belonging to the Italian Twin Registry we explored the extent to which physical clumsiness/motor problems covary with a broad spectrum of behavioral problems identified by the Child Behavior Checklist 6-18/DSM oriented scales, and the causes of such covariation. Only Anxiety and Attention Deficit Hyperactivity (ADH) Problems maintained significant correlation with Clumsiness after partialling out the effects of the other problem scales. By the co-twin control method we found no indication of clear, direct causal effect of Clumsiness upon Anxiety or ADH Problems, or vice versa. Twin bivariate analyses showed that the co-occurrence of motor problems and Anxiety/ADH Problems is best explained by genetic factors shared between Clumsiness and the behavioural problems phenotypes.
3. Cocito D, Paolasso I, Antonini G, Benedetti L, Briani C, Comi C, Fazio R, Jann S, Matà S, Mazzeo A, Sabatelli M, Nobile-Orazio E; Italian Network for CIDP Register. A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol. 2010 Feb;17(2):289-94. Epub 2009 Oct 23. Comment in: Eur J Neurol. 2010 Feb;17(2):171-2.
Abstract
BACKGROUND AND PURPOSE: The guidelines for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) therapy suggest to use immunoglobulins (IVIg) and steroid as first-line therapies. Patients who do not respond to one of the two drugs should be switched to the other drug. We collected therapeutic outcome data in patients followed at 11 centres in order to document the clinical practice in Italy.
METHODS: Clinical and electrophysiological data of patients with CIDP were entered into a central database. The clinical outcome (Rankin Scale) and drug side effects (SE) for first- and second-line therapies were recorded. RESULTS: A total of 267 patients were included. The percentage of responders (R) to first-line therapy [steroid or IVIg or plasma exchange (PE)] was 69%; this number increased to 81% when patients who switched to different therapies were included.Overall, the percentage of R to IVIg was similar to R to steroids (P = 0.07) and higher than R to PE (P < 0.001). Of the main therapies, PE frequently caused SE (19%), followed by steroids (12.5%) and IVIg (4%).
CONCLUSIONS: Switching between traditional therapies increases the number of responder patients. IVIg was confirmed to be a therapy with low SE.
4. Logroscino G, Traynor BJ, Hardiman O, Chiò A, Mitchell D, Swingler RJ, Millul A, Benn E, Beghi E; EURALS. Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry. 2010 Apr;81(4):385-90. Epub 2009 Aug 25.
Abstract
BACKGROUND: Geographical differences in the incidence of amyotrophic lateral sclerosis (ALS) have been reported in the literature but comparisons across previous studies are limited by different methods in case ascertainment and by the relatively small size of the studied populations. To address these issues, the authors undertook a pooled analysis of European population based ALS registries.
METHODS: All new incident ALS cases in subjects aged 18 years old and older were identified prospectively in six population based registries in three European countries (Ireland, UK and Italy) in the 2 year period 1998-1999, with a reference population of almost 24 million.
RESULTS: Based on 1028 identified incident cases, the crude annual incidence rate of ALS in the general European population was 2.16 per 100 000 person years; 95% CI 2.0 to 2.3), with similar incidence rates across all registries. The incidence was higher among men (3.0 per 100 000 person years; 95% CI 2.8 to 3.3) than among women (2.4 per 100 000 person years; 95% CI 2.2 to 2.6). Spinal onset ALS was more common among men compared with women, particularly in the 70-80 year age group. Disease occurrence decreased rapidly after 80 years of age.
CONCLUSIONS: ALS incidence is homogeneous across Europe. Sex differences in incidence may be explained by the higher incidence of spinal onset ALS among men, and the age related disease pattern suggests that ALS occurs within a susceptible group within the population rather than being a disease of ageing.

CARDIOLOGIA

1. Gronda E, Aronica A, Visconti M, Di Malta A, Pini D, Mangiavacchi M, Andreuzzi B, Municinò A, Genovese S, Morenghi E [Gender differences of at risk patients with overt heart failure in the real world of general practice. Data from the GIPSI (Gestione Integrata Progetto Scompenso in Italia) registry] G Ital Cardiol (Rome). 2010 Mar;11(3):233-8.
Abstract
BACKGROUND: Controlled clinical trials have defined the characteristics of specialized world populations, different from the real world population. On this basis, the GIPSI registry was created, aiming to collect data from heart failure populations managed by general practitioners, focusing on gender differences.
METHODS: The registry was based on family history, clinical and laboratory data collection from general practitioners. Patients were considered as being at risk for heart failure if data applied to stage A/B, or presenting overt heart failure if data applied to stage C/D of the American College of Cardiology/American Heart Association classification.
RESULTS: From June 2006 to October 2007, 757 consecutive patients (475 male, 62.7%) were enrolled from 260 general practitioner's practices; 227 patients (143 male, 63.0%) had overt heart failure. In the female population at risk, higher systolic and diastolic blood pressure values were observed, whereas males showed more frequently ischemic heart disease, hypercholesterolemia and hypertriglyceridemia, and were more often prescribed with statins and antiplatelet drugs. There were more heart failure females with diabetes and of advanced age. Moreover, females showed a higher pulse pressure and a significantly lower estimated glomerular filtration rate (by simplified MDRD equation) than males.
CONCLUSIONS: The data collected in a real world population show that heart failure has significantly different gender characteristics, especially for risk factors, age, blood pressure and renal function. This kind of investigation should be extended to larger patient populations for a better understanding of the disease.
2. Palmerini T, De Servi S, Politi A, Martinoni A, Musumeci G, Ettori F, Piccaluga E, Sangiorgi D, Lauria G, Repetto A, Castiglioni B, Fabbiocchi F, Onofri M, De Cesare N, D'Urbano M, Poletti F, Sangiorgi G, Zanini R, Lettieri C, Belli G, Pirelli S, Klugmann S; Lombardima Study Group. Prognostic implications of ST-segment elevation resolution in patients with ST-segment elevation acute myocardial infarction treated with primary or facilitated percutaneous coronary intervention. Am J Cardiol. 2010 Mar 1;105(5):605-10. Epub 2010 Jan 22.
Abstract
Scant data are available on the relation between ST-segment elevation (STE) resolution and 30-day mortality in patients with STE acute myocardial infarction treated with percutaneous coronary intervention in contemporary, real world, clinical practice. Furthermore, whether the prognostic value of STE resolution is influenced by the patient clinical risk profile or postprocedural Thrombolysis In Myocardial Infarction (TIMI) flow has never been investigated. Lombardima was an observational registry implemented in Lombardy, a Northern Italian region. The clinical characteristics, electorcardiographic parameters, and procedural data were prospectively entered into a Web-based database. In the present study, we enrolled 3,403 patients. STE resolution occurred in 2,452 patients (group 1) and did not in 951 patients (group 2). The mortality rate was 2.4% in group 1 and 11.3% in group 2 (p < 0.001). After stratifying patients according to their TIMI risk index, we observed that STE resolution was an independent predictor of 30-day mortality across all spectrum of clinical risk. Furthermore, in patients with TIMI 3 flow, STE resolution remained an independent predictor of 30-day mortality (p < 0.0001). In conclusion, STE resolution was a strong and independent predictor of 30-day mortality in patients with STE acute myocardial infarction undergoing percutaneous coronary intervention across all spectrum of clinical risk.
3. Liistro F, Fineschi M, Grotti S, Angioli P, Carrera A, Ducci K, Gori T, Falsini G, Pierli C, Bolognese L. Long-term effectiveness and safety of sirolimus stent implantation for coronary in-stent restenosis results of the TRUE (Tuscany Registry of sirolimus for unselected in-stent restenosis) registry at 4 years. J Am Coll Cardiol. 2010 Feb 16;55(7):613-6.
Abstract
OBJECTIVES: The aim of this study was to evaluate the long-term clinical outcome of the efficacy and safety of sirolimus-eluting stents (SES) for in-stent restenosis (ISR) in the TRUE (Tuscany Registry of Unselected In-Stent Restenosis) database.
BACKGROUND: The TRUE registry demonstrated that SES in the treatment of bare-metal stent ISR is efficacious (5% of target lesion revascularization [TLR]) and safe (stent thrombosis < 1%) at 9 months. Clinical outcome at 4 years is reported.
METHODS: A total of 244 patients with ISR who were treated with SES implantation represent the study population. The incidence of major adverse cardiac events was collected at 4 years.
RESULTS: At 4-year follow-up, overall mortality was 9.8% (24 patients). Cardiac death occurred in 11 (4.5%), nonfatal myocardial infarction in 8 (3.2%), and TLR in 27 (11.1%) patients for a cumulative event-free survival rate of 80.3%. Definite stent thrombosis occurred in 5 (2%) patients and possible stent thrombosis in 2 (0.8%). Diabetes remained an independent negative predictor of freedom from TLR (odds ratio [OR]: 0.38; 95% confidence interval [CI]: 0.20 to 0.71, p = 0.002) and major adverse cardiac events (OR: 0.38; 95% CI: 0.20 to 0.71, p = 0.002).
CONCLUSIONS: The clinical benefit of SES implantation for bare-metal stent ISR is maintained at 4 years with a low TLR rate and an overall incidence of stent thrombosis of 0.7% per year.
4. Probst V, Veltmann C, Eckardt L, Meregalli PG, Gaita F, Tan HL, Babuty D, Sacher F, Giustetto C, Schulze-Bahr E, Borggrefe M, Haissaguerre M, Mabo P, Le Marec H, Wolpert C, Wilde AA. Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry. Circulation. 2010 Feb 9;121(5):635-43. Epub 2010 Jan 25.
Abstract
BACKGROUND: Brugada syndrome is characterized by ST-segment elevation in the right precordial leads and an increased risk of sudden cardiac death (SCD). Fundamental questions remain on the best strategy for assessing the real disease-associated arrhythmic risk, especially in asymptomatic patients. The aim of the present study was to evaluate the prognosis and risk factors of SCD in Brugada syndrome patients in the FINGER (France, Italy, Netherlands, Germany) Brugada syndrome registry.
METHODS AND RESULTS: Patients were recruited in 11 tertiary centers in 4 European countries. Inclusion criteria consisted of a type 1 ECG present either at baseline or after drug challenge, after exclusion of diseases that mimic Brugada syndrome. The registry included 1029 consecutive individuals (745 men; 72%) with a median age of 45 (35 to 55) years. Diagnosis was based on (1) aborted SCD (6%); (2) syncope, otherwise unexplained (30%); and (3) asymptomatic patients (64%). During a median follow-up of 31.9 (14 to 54.4) months, 51 cardiac events (5%) occurred (44 patients experienced appropriate implantable cardioverter-defibrillator shocks, and 7 died suddenly). The cardiac event rate per year was 7.7% in patients with aborted SCD, 1.9% in patients with syncope, and 0.5% in asymptomatic patients. Symptoms and spontaneous type 1 ECG were predictors of arrhythmic events, whereas gender, familial history of SCD, inducibility of ventricular tachyarrhythmias during electrophysiological study, and the presence of an SCN5A mutation were not predictive of arrhythmic events.
CONCLUSIONS: In the largest series of Brugada syndrome patients thus far, event rates in asymptomatic patients were low. Inducibility of ventricular tachyarrhythmia and family history of SCD were not predictors of cardiac events.
5. Casella G, Cassin M, Chiarella F, Chinaglia A, Conte MR, Fradella G, Lucci D, Maggioni AP, Pirelli S, Scorcu G, Visconti LO; BLITZ-3 Investigators. Epidemiology and patterns of care of patients admitted to Italian Intensive Cardiac Care units: the BLITZ-3 registry. J Cardiovasc Med (Hagerstown). 2010 Jun;11(6):450-61. Comment in: J Cardiovasc Med (Hagerstown). 2010 Jun;11(6):406-7.
Abstract
BACKGROUND: Intensive cardiac care units (ICCUs) have shifted from the observation of patients with myocardial infarction to the care of different acute cardiac diseases. However, few data on such an evolution are available.
METHODS AND RESULTS: From 7 to 20 April 2008, 6986 consecutive patients admitted to 81% of Italian ICCUs were prospectively enrolled. Patients observed were mainly elderly men (median age 72 years) with several co-morbidities. Most of them were triaged to ICCU from the emergency room, but 15% of admissions were transfer-in from other hospitals. Several diagnostic and therapeutic procedures were applied (78% had echocardiography and 35% coronary angiography) during the ICCU stay [median length 4 days, interquartile range (IQR) 2-5]. The discharge diagnosis was ST-elevation acute coronary syndrome (ACS) in 21%, non-ST-elevation ACS in 31%, acute heart failure (AHF) in 14% and other acute non-ACS, non-AHF cardiac diseases in 34%. Of those with ST-elevation ACS, 60% received reperfusion (15% fibrinolysis and 45% primary percutaneous coronary intervention). The overall in-ICCU crude mortality was 3.3%.
CONCLUSION: The BLITZ-3 survey provides a unique snapshot of current epidemiology and patterns of care of patients admitted to ICCUs. Although ACS still remains the most frequent admission diagnosis, the number of non-ACS patients is substantial. However, the correct standard of care for these non-ACS patients has to be defined.
6. Balducelli M, Ortolani P, Marzaroli P, Piovaccari G, Menozzi A, Manari A, Sangiorgio P, Tarantino F, Rossi R, Maresta A, Tondi S, Passerini F, Guastaroba P, Grilli R, Marzocchi A. Comparison of 2-year clinical outcomes with sirolimus and paclitaxel-eluting stents for patients with diabetes: results of the Registro Regionale AngiopLastiche Emilia-Romagna Registry. Catheter Cardiovasc Interv. 2010 Feb 15;75(3):327-34.Comment in: Catheter Cardiovasc Interv. 2010 Feb 15;75(3):335-7.
Abstract
BACKGROUND: Long-term outcomes of percutaneous coronary interventions (PCI) with sirolimus-eluting stents (SES) compared to paclitaxel-eluting-stents (PES) in unselected diabetics in routine practice is still debated.
OBJECTIVE
: This study compared the 2-year incidence of MACE (all-cause mortality, nonfatal myocardial infarction and target vessel revascularization) of SES and PES in a real-world setting of patients with diabetes.
DESIGN
: Observational, multicenter, nonrandomized study.
SETTING
: Prospective web-based registry (REAL Registry; study period, 2002-2005) comprising all 13 hospitals performing PCI.
PATIENTS
: Among the 945 eligible patients treated with either SES alone (n = 606) or PES alone (n = 339), 29% were insulin-requiring, 72% had multivessel coronary disease, 26% had prior myocardial infarction and 10% had poor left ventricular function.
MEASUREMENTS
: Unadjusted and propensity score-adjusted 2-year clinical outcome.
RESULTS
: After propensity score adjustment, 2-year MACE incidence in the SES and PES groups was equivalent (23.3% vs. 23.7%, HR 1.01, 95%CI 0.72-1.42, P = 0.96). Adjusted 2-year angiographic stent thrombosis occurred in 1.1% of the SES patients versus 2.6% of the PES patients (P = 0.15). In this large, real-world, diabetic population treated with DES, there was no difference in outcome between SES and PES. Further studies are needed to demonstrate the long-term safety of different types of DES in patients with diabetes.
7. Proclemer A, Ghidina M, Gregori D, Facchin D, Rebellato L, Zakja E, Gulizia M, Esente P. Trend of the main clinical characteristics and pacing modality in patients treated by pacemaker: data from the Italian Pacemaker Registry for the quinquennium 2003-07. Europace. 2010 Feb;12(2):202-9. Epub 2009 Nov 9. Comment in: Europace. 2010 Feb;12(2):151.
Abstract
AIMS: To assess the impact on clinical practice of the major published studies, we report the information collected by the Italian Pacemaker Registry (IPR) in the quinquennium 2003-07.
METHODS AND RESULTS: The IPR collected prospectively main epidemiological, clinical, and electrocardiographic data of patients treated by pacemaker (PM) in Italy on the basis of European PM Card. The number of reported PMs in Italy was 30,820 in 2003, 32,047 in 2004, 31,870 in 2005, 31,813 in 2006, and 31,146 in 2007, respectively. The median age was 79 years in all 5 years. Among the atrio-ventricular (AV) conduction defects, third-degree AV block was the most common occurrence. Of the sick sinus syndrome (SSS), sinus node dysfunction involved the majority of cases followed by bradycardia-tachycardia syndrome. Year-over-year percentages among the different indications remained stable. Syncope and dizzy spells were by far the most common symptoms. Dual-chamber pacing showed an increasing utilization in all the examined years.
CONCLUSION: Italian PM Registry data for the study period reveal a stable pattern of PM utilization and indications. A higher use of dual-chamber pacing in comparison to single-chamber pacing was reported for all indications, despite inconclusive data of the major randomized trials.

REUMATOLOGIA

1. Lepore L, Paloni G, Caorsi R, Alessio M, Rigante D, Ruperto N, Cattalini M, Tommasini A, Zulian F, Ventura A, Martini A, Gattorno M. Follow-up and quality of life of patients with cryopyrin-associated periodic syndromes treated with Anakinra. J Pediatr. 2010 Aug;157(2):310-315.e1. Epub 2010 May 15.
Abstract
OBJECTIVE: To evaluate the quality of life and long-term follow-up of patients enrolled in the Italian registry of cryopyrin-associated periodic syndromes (CAPS).
STUDY DESIGN: Since 2004, 20 patients with CAPS were enrolled in a common registry from different Italian Centers of Pediatric Rheumatology; 14 patients were treated with Anakinra in an open fashion. Both treated and untreated patients were routinely followed according to standard of care. The Child Health Questionnaire (CHQ-PF 50) was used to assess the health-related quality of life.
RESULTS: The mean duration of follow-up was 37.5 months. In all treated patients, a complete and persistent control of the inflammatory manifestations was observed with no further progression of the disease. At enrollment in the registry, patients showed a poorer health-related quality of life than healthy children in both physical and the psychosocial summary scores. Treatment was associated with a dramatic and sustained amelioration of a variety of measures of poor quality of life, particularly in those concerning the global health perception, bodily pain-discomfort, and other physical domains.
CONCLUSIONS: Long-term IL-1 blockade produces a significant and persistent improvement in the clinical manifestations associated with the disease and on the overall quality of life.
2. Della Rossa A, Neri R, Talarico R, Doveri M, Consensi A, Salvadori S, Lorenzoni V, Turchetti G, Bellelli S, Cazzato M, Bazzichi L, Monicelli P, Moscardini S, Bombardieri S. Diagnosis and referral of rheumatoid arthritis by primary care physician: results of a pilot study on the city of Pisa, Italy. Clin Rheumatol. 2010 Jan;29(1):71-81. Epub 2009 Oct 6.
Abstract
The aims of the present study were to evaluate, in the city of Pisa: (1) the prevalence of rheumatoid arthritis; (2) the reliability of the prevalence estimated by primary care physicians, using the rheumatologist's diagnosis as the "gold standard" and (3) the economic impact of the disease. The Tuscany registry of primary care physicians constituted the framework from which a sample of subjects was selected. The rheumatoid arthritis (RA) subjects >18 years followed by each primary care physician constituted the population studied. Each general practitioner (GP) was asked to fill out a questionnaire regarding their patients affected by RA and to send it to the tertiary rheumatologic centre, where the diagnosis was confirmed/discarded, the clinical and epidemiological data were collected in a standardized form and a number of data for the estimation of costs were gathered. The estimated prevalence of RA was 5.1 per thousand (CI, 4.4-5.7). The reliability of general practitioners in the diagnosis of rheumatoid arthritis was on the whole 69%. However, when an analysis of every physician was carried out, a high degree of heterogeneity in the prevalence of RA per physician was found. Overall, the mean annual cost per patient with RA was estimated at about 5,878 euro (euro; median, 6,434 euro; inter quartile range, 669-7,052 euro), with a high variability mainly dependent on the degree of patient disability. More than 90% of the overall annual cost per patient was due to the medical and non-medical direct components of costs. The prevalence of RA in Tuscany seems highly comparable with similar prevalence studies in Italy. The annual cost per patient with RA was highly variable and strictly dependent on the level of disability. More than 90% of the overall cost was due to the direct burden of costs.

TRAPIANTI

1. Burra P, Senzolo M, Adam R, Delvart V, Karam V, Germani G, Neuberger J; ELITA; ELTR Liver Transplant Centers. Liver transplantation for alcoholic liver disease in Europe: a study from the ELTR (European Liver Transplant Registry). Am J Transplant. 2010 Jan;10(1):138-48. Epub 2009 Dec 1.
Abstract
Alcohol-related liver disease (ALD) is one of the most common indications for liver transplantation (LT). Long-term outcome after LT for ALD versus other etiologies is still under debate. The aim of this study was to compare outcome after LT of patients with ALD, viral (VIR), and cryptogenic cirrhosis. Donor, graft and recipient ELTR variables were analysed in transplants for alcoholic and nonalcoholic cirrhosis (1988-2005) and were correlated with patient survival. Causes of death and/or graft failure were compared between groups. Nine thousand eight hundred eighty ALD, 10,943 VIR, 1478 ALD+VIR and 2410 cryptogenic (CRYP) liver transplants were evaluated. One, 3, 5 and 10 years graft survival rates after LT in ALD patients were 84%, 78%, 73%, 58%, significantly higher than in VIR and CRYP (p=0.04, p=0.05). By multivariate analysis, ALD+VIR (RR 1.14) and viral alone (RR 1.06) were significant risk factors for mortality. De novo tumors, cardiovascular and social causes were causes of death/graft failure in higher percentage in ALD groups versus other etiologies. LT for ALD cirrhosis has a favorable outcome, however, hepatitis C virus co-infection seems to eliminate this advantage. Screening for de novo tumors and prevention of cardiovascular complications are essential to provide better long-term results.

MALATTIE INFETTIVE

1. Faustini A, Colais P, Fabrizi E, Bargagli AM, Davoli M, Di Lallo D, Di Napoli A, Pezzotti P, Sorge C, Grillo R, Maresca C, Recchia O, Perucci CA; HCV laboratori surveillance Lazio-Region Group. Hepatic and extra-hepatic sequelae, and prevalence of viral hepatitis C infection estimated from routine data in at-risk groups. BMC Infect Dis. 2010 Apr 19;10:97.
Abstract
BACKGROUND: Concerns about the hepatitis C virus (HCV) are due to the high risk of chronic liver disease and poor treatment efficacy. Synthesizing evidence from multiple data sources is becoming widely used to estimate HCV-infection prevalence. This paper aims to estimate the prevalence of HCV infection, and the hepatic and extrahepatic sequelae in at-risk groups, using routinely collected data in the Lazio region, Italy.
METHODS: HCV laboratory surveillance and dialysis, hospital discharge, and drug-user registers were used as information sources to identify at-risk groups and to estimate HCV prevalence and sequelae. Full name and birth date were used as linkage keys for the various health registries. Prevalence was estimated as the percentage of cases within the general population and the at-risk groups, with 95% confidence intervals (95% CI) from 1997 to 2001. The risk of sequelae was estimated through a follow-up of hospital discharges up to December 31, 2004 and calculated as the prevalence ratio in HCV-positive and HCV-negative people, within each at-risk group, with 95% CI.
RESULTS: There were 65,127 HCV-infected people in the study period; the prevalence was 1.24% (95%CI = 1.23%-1.25%) in the whole population, higher in males and older adults. Drug users (35.1%; 95%CI = 34.6-35.7) and dialysis patients (21.1%; 95%CI = 20.2%-22.0%) showed the highest values. Medical procedures with little exposure to blood resulted in higher estimates, ranging between 1.3% and 3.4%, which was not conclusively attributable to the surgical procedures. Cirrhosis, hepatocellular carcinoma and encephalopathy were the most frequent hepatic sequelae; cryoglobulinaemia and non-Hodgkin's lymphoma were the most frequent extrahepatic sequelae.
CONCLUSIONS: Synthesising data from multiple routine sources improved estimates of HCV prevalence and sequelae in dialysis patients and drug users, although prevalence validity should be assessed in survey and sequelae need a well-defined longitudinal approach.

TRAUMATOLOGIA

1. Di Bartolomeo S, Tillati S, Valent F, Zanier L, Barbone F.ISS mapped from ICD-9-CM by a novel freeware versus traditional coding: a comparative study. Scand J Trauma Resusc Emerg Med. 2010 Mar 31;18:17.
Abstract
BACKGROUND: Injury severity measures are based either on the Abbreviated Injury Scale (AIS) or the International Classification of diseases (ICD). The latter is more convenient because routinely collected by clinicians for administrative reasons. To exploit this advantage, a proprietary program that maps ICD-9-CM into AIS codes has been used for many years. Recently, a program called ICDPIC trauma and developed in the USA has become available free of charge for registered STATA users. We compared the ICDPIC calculated Injury Severity Score (ISS) with the one from direct, prospective AIS coding by expert trauma registrars (dAIS).
METHODS: The administrative records of the 289 major trauma cases admitted to the hospital of Udine-Italy from 1 July 2004 to 30 June 2005 and enrolled in the Italian Trauma Registry were retrieved and ICDPIC-ISS was calculated. The agreement between ICDPIC-ISS and dAIS-ISS was assessed by Cohen's Kappa and Bland-Altman charts. We then plotted the differences between the 2 scores against the ratio between the number of traumatic ICD-9-CM codes and the number of dAIS codes for each patient (DIARATIO). We also compared the absolute differences in ISS among 3 groups identified by DIARATIO. The discriminative power for survival of both scores was finally calculated by ROC curves.
RESULTS: The scores matched in 33/272 patients (12.1%, k 0.07) and, when categorized, in 80/272 (22.4%, k 0.09). The Bland-Altman average difference was 6.36 (limits: minus 22.0 to plus 34.7). ICDPIC-ISS of 75 was particularly unreliable. The differences increased (p < 0.01) as DIARATIO increased indicating incomplete administrative coding as a cause of the differences. The area under the curve of ICDPIC-ISS was lower (0.63 vs. 0.76, p = 0.02).
CONCLUSIONS: Despite its great potential convenience, ICPIC-ISS agreed poorly with its conventionally calculated counterpart. Its discriminative power for survival was also significantly lower. Incomplete ICD-9-CM coding was a main cause of these findings. Because this quality of coding is standard in Italy and probably in other European countries, its effects on the performances of other trauma scores based on ICD administrative data deserve further research. Mapping ICD-9-CM code 862.8 to AIS of 6 is an overestimation.

OSTETRICIA-GINECOLOGIA

1. Stivanello E, Knight M, Dallolio L, Frammartino B, Rizzo N, Fantini MP. Peripartum hysterectomy and cesarean delivery: a population-based study. Acta Obstet Gynecol Scand. 2010 Mar;89(3):321-7.
Abstract
OBJECTIVE: To estimate the incidence of peripartum hysterectomy in an Italian Region (Emilia-Romagna) and investigate its association with cesarean delivery.
DESIGN: Population-based retrospective study using hospital discharge records.
SETTING: All public and private hospitals in Emilia-Romagna region, Italy.
POPULATION: A total of 151,494 women delivering between 2003 and 2006, 131 of whom had a peripartum hysterectomy.
METHODS: Peripartum hysterectomy was defined as a hysterectomy performed at the time of delivery or afterwards during the same hospitalization. Incidence rates were calculated by type of delivery. Odds ratios (ORs) with 95% confidence intervals (CIs) were estimated with logistic regression to evaluate the association between peripartum hysterectomy and delivery type.
MAIN OUTCOME MEASURES: Incidence rates of peripartum hysterectomy by type of delivery; odds of peripartum hysterectomy after primary or repeat caesarean compared with vaginal delivery without previous cesarean.
RESULTS: A total of 131 peripartum hysterectomies were performed among 151,494 deliveries (0.86/1,000 deliveries; 95% CI 0.72-1.03) with 20.7% primary and 9.6% repeat caesarean deliveries. Women undergoing a primary caesarean delivery were more likely to have a peripartum hysterectomy than women having a vaginal delivery who had never had a cesarean delivery (OR 6.48; 95% CI 4.16-10.07). Women undergoing a repeat caesarean delivery were also at increased risk (OR 3.69; 95% CI 2.11-6.46).
CONCLUSIONS: In this population, primary and repeat cesarean deliveries are associated with an increased risk of peripartum hysterectomy. These results are of particular concern given the steady increase in the cesarean delivery rate in many countries. The pathological mechanism of this association requires further investigation.

CHIRURGIA

1. Casaccia M, Torelli P, Pasa A, Sormani MP, Rossi E; IRLSS Centers. Putative predictive parameters for the outcome of laparoscopic splenectomy: a multicenter analysis performed on the Italian Registry of Laparoscopic Surgery of the Spleen. Ann Surg. 2010 Feb;251(2):287-91.
Abstract
OBJECTIVE: To identify predictive risk factors for conversion to open splenectomy and postoperative complications in patients undergoing elective laparoscopic splenectomy.
BACKGROUND: The laparoscopic approach represents the "gold standard" for splenectomy, but its use in the treatment of splenomegaly and malignant disease is controversial. Factors that influence immediate outcome are clinical, anatomic, and pathologic.
METHODS: Univariate and multivariate analyses of data from the Italian Registry of Laparoscopic Surgery of the Spleen, a multicenter database supported by 25 referral centers. Analysis of data (1993-2007) was performed on a series of patients (n = 676) undergoing elective laparoscopic splenectomy. Demographic data, the operative indications, the surgical technique applied, and any intra- and/or postoperative complications with respect to the patients were assessed. Records were analyzed retrospectively using the Student t test, the chi test, and logistic regression.
RESULTS: Conversion to open splenectomy was necessary in 39 cases (5.8%). Perioperative deaths occurred in 3 cases (0.4%). There were no complications in 560 patients (82.8%), with a mean hospital stay of 5 days (range, 2-54). Overall, morbidity occurred in 116 patients (17.2%). Multivariate analysis found that the body mass index (P = 0.01) and the presence of hematologic malignancy (P < 0.001) were independent predictors for intraoperative complications and surgical conversion. Spleen longitudinal diameter (P = 0.001) and surgical conversion (P = 0.001) were independent predictors for the occurrence of postoperative complications.
CONCLUSIONS: This large multicenter study provides evidence for the significance of predictive risk factors for intra- and postoperative complications in laparoscopic splenic surgery. Besides splenic dimensions, other factors like the patient's habitus and the specific underlying hematologic pathology should be recognized by the surgeon to reduce complications and initiate adequate treatment.

PEDIATRIA

1. Salvatore D, Buzzetti R, Baldo E, Forneris MP, Lucidi V, Manunza D, Marinelli I, Messore B, Neri AS, Raia V, Furnari ML, Mastella G. An overview of international literature from cystic fibrosis registries 2. Neonatal screening and nutrition/growth. J Cyst Fibros. 2010 Mar;9(2):75-83. Epub 2009 Dec 2.
Abstract
BACKGROUND: This is the second article related to a review of the literature based on data from national cystic fibrosis (CF) registries up to June 2008 and covering a total of 115 studies. It focuses on two topics: neonatal screening (NS) and nutritional status, with particular reference to growth.
METHODS: Ten papers meeting the inclusion criteria were found on the topic of NS and its impact on the course of the disease, and were analyzed according to a dedicated grid. The issue of nutrition was addressed by 14 studies, analyzed according to similar criteria.
RESULTS: Most of the studies report benefits of early diagnosis by NS, albeit to variable degrees. The benefits were assessed in terms of better nutritional status and growth, but also in terms of lower overall morbidity rate as compared to subjects diagnosed by symptoms. The main biases of these studies, which partly undermine the validity of their results, are also analyzed. A part of our analysis on nutrition/growth is dedicated to the identification of the most suitable parameters to define malnutrition: in children older than two years the body mass index percentile (BMIp) appears to be the most sensitive and significantly associated with respiratory function. Better nutritional status and satisfactory growth appear to be associated with better lung function and lower risk of death. The relationship between nutritional status and socio-economic status is also of interest.
CONCLUSIONS: CF registry studies support the outcome of cohort observational studies i.e. that pre-symptomatic early diagnosis is beneficial, especially in terms of nutritional status and growth. Studies on nutrition indicate that good nutritional status is associated with better respiratory function and prognosis. Regarding methods, the need emerged to manage potential biases of this kind of non randomized studies, resorting to suitable statistical techniques, such as matching and stratification and, above all, to multivariate methods able to provide estimates adjusted for the main covariates tested.

PNEUMOLOGIA

1. Agabiti N, Belleudi V, Davoli M, Forastiere F, Faustini A, Pistelli R, Fusco D, Perucci CA. Profiling hospital performance to monitor the quality of care: the case of COPD. Eur Respir J. 2010 May;35(5):1031-8. Epub 2009 Oct 19.
Abstract
Comparative outcomes data are widely used to monitor quality of care in the cardiovascular area; little is available in the respiratory field. We applied validated methods to compare hospital outcomes for chronic obstructive pulmonary disease (COPD) exacerbation. From the hospital information system, we selected all hospital admissions for COPD exacerbation in Rome (for 2001-2005). Vital status within 30 days was obtained from the municipality mortality register. Each hospital was compared to a pool of hospitals with the lowest adjusted mortality rate (the benchmark). Age, sex and several potential clinical predictors were covariates in logistic regression analysis. 12,756 exacerbated COPD patients were analysed (mean age 74 yrs, 71% males). Diabetes, hypertension, ischaemic heart disease, heart failure and arrhythmia were the most common coexisting conditions. The average crude mortality in the benchmark group was 3.8%; in the remaining population it was 7.5% (range 5.2-17.2%). In comparison with the benchmark, the relative risk of 30-day mortality varied widely across the hospitals (range 1.5-5.9%). A large variability in 30-day mortality after COPD exacerbation exists even considering patients' characteristics. Although these results do not detect mechanisms related to worse outcomes, they may be useful to stimulate providers to revision and improvement of COPD care management.