Italian Tertiary Center Research Articles

P540 Early response as a prognostic factor for vedolizumab: A real-life experience from a single Italian tertiary centre

P540 Early response as a prognostic factor for vedolizumab: A real-life experience from a single Italian tertiary centre

Correlation between Subpubic Arch Angle and Mode of Delivery in Large-for-Gestational-Age Fetuses

Objectives: A narrow subpubic arch angle (SPA) has been associated with a higher risk of operative delivery and prolonged labor. The aim of this study was to evaluate the correlation between SPA and labor outcome in a cohort of women delivering a large-for-gestational-age (LGA) fetus. Methods: An observational study involving two Italian tertiary centers (Parma and Rome) was carried out. Nulliparous women referred to the antepartum clinic between 35 and 39 weeks due to an increased risk of having an LGA fetus at birth were prospectively selected for the study purpose. Within the study cohort, SPA measurements were obtained by means of transperineal 3D ultrasound. Elective caesarean section and birth weight below 3,750 g represented exclusion criteria. In the final study group, SPA values were compared between the patients who underwent spontaneous vaginal delivery (SVD) and those who were submitted to unplanned obstetric intervention (UOI) due to prolonged or arrested labor (vacuum delivery or caesarean section). Results: Overall, 129 women were included, and the mean birthweight of the neonates was 4,066 ± 263.03 g. SVD occurred in 63 patients (48.8%), whereas UOI due to prolonged or arrested labor was performed in 66 (51.2%), including 21 cases of vacuum delivery and 45 caesarean sections. The SPA was significantly smaller among women who underwent UOI than in those who achieved SVD (107.9 ± 13.4 vs. 120.7 ± 9.4°, p < 0.001). Furthermore, SPA width was inversely correlated with labor length (p < 0.001). Multivariable logistic regression analysis showed that a smaller SPA (OR 1.091, 95% CI 1.051-1.134, p < 0.001) and an increased birthweight (OR 1.002, 95% CI 1.000-1.004, p = 0.037) were independent risk factors for operative delivery. Conclusion: SPA measurement before labor is helpful in predicting the risk of operative delivery due to prolonged or arrested labor among nulliparous women delivering LGA fetuses.

Safety and efficacy of abiraterone acetate in chemotherapy-naive patients with metastatic castration-resistant prostate cancer: an Italian multicenter \u201creal life\u201d study

BackgroundTo evaluate the safety and efficacy of abiraterone acetate (AA) in the “real life” clinical practice for men with chemotherapy-naïve metastatic castration-resistant prostate.MethodsA consecutive series of patients with mCRPC in 9 Italian tertiary centres treated with AA was collected. Demographics, clinical parameters, treatment outcomes and toxicity were recorded. The Brief Pain Inventory scale Q3 was tracked and patient treatment satisfaction was evaluated. Survival curves were estimated by the method of Kaplan-Meier and Cox regression and compared by the log-rank test statistic.ResultsWe included 145 patients (mean age 76.5y). All patients were on androgen deprivation therapy. Patients had prior radiotherapy, radical prostatectomy, both treatments or exclusive androgen deprivation therapy in 17%, 33%, 9% and 40%, respectively. 57% of the patients had a Gleason score higher more than 7 at diagnosis. 62% were asymptomatic patients. The median serum total PSA at AA start was 17 ng/mL (range 0,4–2100). The median exposure to AA was 10 months (range 1–35). The proportion of patients achieving a PSA decline ≥50% at 12 weeks was 49%. Distribution of patient satisfaction was 32% “greatly improved”, 38% “improved”, 24% “not changed”, 5.5% “worsened”. Grade 3 and 4 toxicity was recorded in 17/145 patients 11.7% (70% cardiovascular events, 30% critical elevation of AST/ALT levels). At the last follow-up, median progression free and overall survival were 17 and 26.5 months, respectively. Both outcomes significantly correlated with the presence of pain, patient satisfaction, PSA baseline and PSA decline.ConclusionsThe AA is effective and well tolerated in asymptomatic or slightly symptomatic mCRPC in a “real life” setting. The survival outcomes are influenced by the presence of pain, patient satisfaction, baseline PSA and PSA decline.Trial registrationThe study was retrospectively registered at ISRCTN as DOI:10.1186/ISRCTN 52513758 in date April the 30th 2016.

Mortality in Italian patients with rheumatoid arthritis: evidence for a low mortality rate from cancer and infections in patients followed up at a tertiary center.

ObjectivesMortality in patients with rheumatoid arthritis (RA) has never been investigated in Italy. This study is devoted to investigating all the distinct causes of mortality in Italian RA patients.MethodsClinical charts of patients consecutively admitted to an Italian tertiary center, from January 1, 2008 to December 31, 2014, were reviewed. Mortality rates (incidence mortality rate [IMR] and standardized mortality rate [SMR]) and causes of death as assessed at December 31, 2015, were registered. Mortality rates detected in our series were compared to those reported in other European cohorts and in the general Italian population.ResultsSix hundred and eight patients were observed for a median of 3.51 years. Overall IMR was 0.79 deaths/100 person-years. No significant difference between our IMR and that reported in Italian population by the National Institute of Statistics was observed. All-cause and neoplasm IMRs in our series were found to be significantly lower than that reported in the Norfolk Arthritis Registry, while no difference was detected in cardiovascular (CV) mortality. On the other hand, all causes and CV SMRs in our series were found to be higher than that reported in the general Italian population, while cancer and infectious SMRs were found to be lower.ConclusionIn our series, RA patients had an increased all-cause mortality, and in particular an increased death rate due to CV. However, a lower death rate due to cancer and infections was observed. This figure might be due to the careful follow-up of RA patients in tertiary centers, and the results underlines the need to improve the management of CV risk.

Iatrogenic Kaposi's Sarcoma: a Retrospective Cohort Study in an Italian Tertiary Care Centre

AimsKaposi's sarcoma (KS) is a lymphoangioproliferative multicentric disorder. Among its four distinct clinical variants, iatrogenic KS (iKS) typically affects patients who have received immunosuppressant regimens for organ transplants, proliferative disorders, or immune-mediated diseases. The aim of the current study was to examine the characteristics of a cohort of patients with iKS, evaluating the differences in terms of epidemiological and clinical features, management and outcomes between organ transplant recipients (OTR) and patients immunosuppressed for other medical conditions. Materials and methodsThis retrospective study included, out of 1389 KS patients, 143 patients suffering from iKS being followed in an Italian tertiary care centre from November 1995 to December 2016. Demographic data, clinical features, previous immunosuppressive therapies, management, and outcomes were recorded for each patient. ResultsWe detected iKS in 10.3% of the analysed KS population. The mean age was 71.9 years in non-OTR versus 51.4 years in OTR (P = 0.04). Staging at diagnosis showed a more severe disease in non-OTR than in OTR, with stage IA observed in 33.3% of OTR versus 11.8% of non-OTR (P < 0.001) and stage IVB in 29.1% of non-OTR versus 12.1% of OTR (P = 0.001). Corticosteroids represented the most frequent immunosuppressive drugs at diagnosis in both groups, in conjunction with cyclosporine A in OTR. Immunosuppressant reduction or withdrawal was carried out in 93.9% of OTR versus 63.6% of non-OTR (P < 0.001). ConclusionsAs corticosteroids and cyclosporine A are the most common iKS-inducing drugs, their reduction or withdrawal, wherever possible, is needed. Differences in disease severity at presentation between OTR and non-OTR may interfere with the choice of management strategy and the consequent outcome.

Eight-Year Retention Rate of First-Line Tumor Necrosis Factor Inhibitors in Spondyloarthritis: A Multicenter Retrospective Analysis.

To evaluate the 8-year survival of the first tumor necrosis factor inhibitor (TNFi) in patients with axial spondyloarthritis (SpA) or psoriatic arthritis (PsA), identify the predictive factors for withdrawal, and compare the discontinuation rates for infliximab, etanercept, and adalimumab. We evaluated PsA and axial SpA patients treated with a first-line TNFi between 2005 and 2015 at 4 Italian tertiary centers. Eight-year drug survival was calculated by the Kaplan-Meier method, and risk for discontinuation among treatment groups compared by stratified log-rank test. Univariate and multivariate Cox proportional hazard models were developed to examine predictors of withdrawal. Of 614 patients (316 axial with SpA, 298 with PsA), 203 received adalimumab, 131 etanercept, and 280 infliximab, with similar frequencies in axial SpA and PsA subgroups. The cumulative 8-year retention rate in the whole population was 55.1% (57.2% and 51.9% for axial SpA and PsA, respectively; P = not significant). No significant differences were observed in drug persistence among individual TNFi in either group. Male sex (hazard ratio [HR] 0.595 [95% confidence interval (95% CI) 0.405-0.875]; P = 0.008) and concomitant methotrexate use (HR 0.648 [95% CI 0.426-0.985]; P = 0.042) were associated with a lower risk of withdrawal in PsA. High baseline Bath Ankylosing Spondylitis Disease Activity Index (HR 0.9842 [95% CI 0.9708-0.9980]; P = 0.028) was associated with a lower risk of withdrawal in axial SpA. No difference was found in the comparative analysis of reasons for discontinuation between PsA and axial SpA. We reported that the real-life 8-year retention rate of the first TNFi in axial SpA and PsA is greater than 50%, with no significant differences between axial SpA and PsA, irrespective of the individual TNFi.

Serum C-Reactive Protein as a Prognostic Biomarker in Amyotrophic Lateral Sclerosis

ImportanceVarious factors have been proposed as possible candidates associated with the prognosis of amyotrophic lateral sclerosis (ALS); however, there is still no consensus on which biomarkers are reliable prognostic factors. C-reactive protein (CRP) is a biomarker of the inflammatory response that shows significant prognostic value for several diseases.ObjectiveTo examine the prognostic significance of CRP in ALS.Design, Setting, and ParticipantsPatients’ serum CRP levels were evaluated from January 1, 2009, to June 30, 2015, in a large cohort of patients with ALS observed by an Italian tertiary multidisciplinary center. Results were replicated in an independent cohort obtained from a population-based registry of patients with ALS. A post hoc analysis was performed of the phase 2 trial of NP001 to determine whether stratification by levels of CRP improves differentiation of responders and nonresponders to the drug.Main Outcomes and MeasuresSerum CRP levels from the first examination were recorded to assess their effect on disease progression and survival.ResultsA total of 394 patients with ALS (168 women and 226 men; mean [SD] age at diagnosis, 60.18 [13.60] years) were observed in a tertiary multidisciplinary center, and the analysis was replicated in an independent cohort of 116 patients with ALS (50 women and 66 men; mean [SD] age at diagnosis, 67.00 [10.74] years) identified through a regional population-based registry. Serum CRP levels in the 394 patients with ALS correlated with severity of functional impairment, as measured by total score on the ALS Functional Rating Scale–Revised, at first evaluation (r = –0.14818; P = .004), and with patient survival (hazard ratio, 1.129; 95% CI, 1.033-1.234; P = .007). Similar results were found in the independent cohort (hazard ratio, 1.044; 95% CI, 1.016-1.056; P ≤ .001). Moreover, a post hoc analysis of the phase 2 trial of NP001 using the same CRP threshold showed that patients with elevated baseline CRP levels receiving the higher dose of NP001 had significantly less functional impairment after the treatment period compared with patients with normal baseline CRP, regardless of whether patients with normal CRP levels received NP001 or placebo (3.00 [3.62] vs –7.31 [6.23]; P = .04).Conclusions and RelevanceThese findings suggest that patients with ALS and elevated serum CRP levels progress more rapidly than do those with lower CRP levels and that this elevation may reflect a neuroinflammatory state potentially responsive to the immune regulators such as NP001.

P.09.11: 10 Years Dili Experience in an Italian Tertiary Center

P.09.11: 10 Years Dili Experience in an Italian Tertiary Center

Differences in Clinical and Molecular Characteristics and Outcome in Prefibrotic and Overt Primary Myelofibrosis According to 2016 WHO Criteria. a Study on 639 Patients of the Agimm Group

▪The revised 2016 World Health Organization (WHO) classification of myeloid neoplasms dictated distinct criteria for prefibrotic (prePMF) and overt primary myelofibrosis (PMF), based on bone marrow (BM) morphology, including fibrosis grade (G)<1 in prePMF and G2-3 in PMF, and presence of leukoerythroblastosis in PMF.AIM: to describe the characteristics and outcome of patients (pts) with a diagnosis of prePMF versus PMF according to the 2016 WHO criteria.METHODS. We used a database of ca 800 pts collected in 5 Italian tertiary centers of the AGIMM project. Pts annotated with diagnosis of pre/early PMF and PMF were identified, and BM biopsies were re-classified according to current criteria. A total of 639 pts with full information were retrieved; all were annotated for both driver mutations (JAK2V617F, MPLW515x, CALR) and High Molecular Risk mutations (HMR; Vannucchi et al, Leukemia 2013;1861), including ASXL1, EZH2, SRSF2, IDH1/2.RESULTS. Of the 639 pts, 274 (42.8%) were re-classified as prePMF and 365 (57.2%) as PMF. After a median follow-up (FU) of 3.6y, 212 pts (33.2%) had died, 23% prePMF vs 40.8% PMF (P<.0001); 69 pts (10.8%) transformed to leukemia (AL), 8.0% vs 12.9% in prePMF vs PMF (P= .033). At diagnosis, compared to PMF, prePMF pts were enriched in females (57.2% vs 42.9%, P=.01), were younger (59.4 vs 64.7y; P<.001) and with less >65y old individuals (36.1% vs 46.8%, P= .004), had higher Hb (12.7 vs 10.7g/dL; P<.0001) and fewer anemic subjects (Hb<10g/dL:14.2% vs 38.4%; P<.0001), showed higher platelet (plt) count (453 vs 247x109/L; P<.0001) and fewer thrombocytopenic subjects (<100x109/L; 8.0% vs 18.1%; P<.0001); blasts >1% were found in 12.0% vs 26.3% (P<.0001), while leukocyte count and % of pts with >25x109/L were similar. Abnormal karyotype in 18.5% prePMF vs 38.6% PMF (n=317; P<.0001). Constitutional symptoms were reported in 20.8% prePMF vs 34.3% PMF (P<.0001), palpable splenomegaly in 64.2% vs 83.1% (P<.0001), spleen >10cm in 10.5% vs 24.1% (P<.0001). Major thrombosis occurred in 15.5% of prePMF vs 9.0% PMF (P=.02). According to IPSS, 74.2% and 25.8% of prePMF pts were in the lower and higher risk categories, respectively, vs 50.8% and 49.2% of PMF (P<.0001). At the latest FU, most prePMF pts maintained a lower risk category (65.8%) according to DIPSS unlike PMF with 69.3% being categorized as higher risk (P<.0001).The proportion of patients with JAK2V617F (and their median allele burden), MPLW515x and CALR (type I and type II) mutations was similar in prePMF and PMF; triple-negative (TN) pts were slightly more frequent in PMF (14%) than prePMF (9.9%; P=.041). Conversely, 24.8% of prePMF pts vs 41.1% PMF were HMR (P<.0001), the frequent mutated genes were ASXL1 and EZH2; >2 HMR mutations, that are prognostically negative (Guglielmelli et al, Leukemia 2014; 28), were found in 11.8% of PMF pts vs 4.7% prePMF (P<.0001).Median survival (OS) was 17.6y in prePMF vs 7.2y in PMF (P<.0001). OS was accurately predicted by IPSS in both prePMF and PMF. Using CALR type 1 mutation as the reference group, CALR type 2, JAK2/MPL mutations and triple negativity were negative predictors. HMR status was prognostically significant for OS in both prePMF (HR1.8, 95%CI 1.1-3.0, P=.03) and PMF (HR 2.5; 1.8-3.4, P<.0001) as it was >2 HMR mutations (HR 9.3, 4.5-19.0 and 3.4,2.1-5.3, respectively; P<.0001).CONCLUSIONS. This analysis of pts with contemporary diagnosis of prePMF and PMF disclosed important clinical, hematologic and molecular differences between the two, and indirectly suggested that they might represent a phenotypic continuum where increased grade of fibrosis associates with worsening of disease manifestations and outcome. DisclosuresVannucchi:Novartis: Consultancy, Research Funding, Speakers Bureau; Baxalta: Speakers Bureau; Shire: Speakers Bureau.

Impact of vitamin D deficiency on the clinical and biochemical phenotype in women with sporadic primary hyperparathyroidism.

The purpose of the study was to evaluate the relationship between serum 25(OH)D and the clinical phenotype in 215 consecutive Italian Caucasian women with sporadic primary hyperparathyroidism (PHPT) not taking vitamin D supplements. The study was performed at a single Italian tertiary center. PHPT-related manifestations, serum 25(OH)D, and other parameters of calcium metabolism and bone mineral density (BMD) by DXA were recorded. Serum 25(OH)D was negatively correlated with age (r=-0.18; P=0.006), BMI (r=-0.20; P=0.002), PTH (r=-0.21; P=0.001), bone-specific alkaline phosphatase (BSAP) (r=-0.27; P<0.001), and eGFR (r=-0.22; P=0.01), and positively with serum creatinine and 1/3 distal radius BMD (R-BMD; r=0.17; P=0.015). In a multivariate regression analysis, serum 25(OH)D remained significantly correlated with age (r=-0.18; P=0.005), BMI (r=-0.23; P=0.049), serum PTH (r=-0.01; P=0.023), BSAP (r=-0.01; P=0.023) and eGFR (r=-0.09; P=0.001), but not with R-BMD. Serum 25(OHD) was higher in patients with nephrolithiasis than in those without nephrolithiasis (18.5±8.8 vs. 15.6±8.0ng/ml; P=0.029), whereas no difference was found between fractured and unfractured patients (16.8±9.3 vs. 16.0±7.7; P=0.663). There was a statistically significant inverse correlation between vitamin D status [defined by quartiles of measured values as well as commonly accepted cutoffs of serum 25(OH)D] and severity of the disease, as reflected by higher PTH and BSAP, but not by meeting the latest guidelines for parathyroidectomy. In conclusion, a low vitamin D status is associated with some features reflecting a more severe biochemical and clinical phenotype of PHPT in Italian women not taking vitamin D supplements.

Pisa syndrome in Parkinson disease: An observational multicenter Italian study.

To estimate the prevalence of Pisa syndrome (PS) in patients with Parkinson disease (PD) and to assess the association between PS and demographic and clinical variables. In this multicenter cross-sectional study, consecutive outpatients with PD attending 21 movement disorders Italian tertiary centers were enrolled and underwent standardized clinical evaluation. PS was defined as trunk lateral deviation ≥10°. Patients with PD were compared according to the presence of PS for several demographic and clinical variables. Among 1,631 enrolled patients with PD, PS was detected in 143 patients (8.8%, 95% confidence interval 7.4%-10.3%). Patients with PS were older, had lower body mass index, longer disease duration, higher disease stages, and poorer quality of life. Falls were more frequent in the PS group as well as occurrence of "veering gait" (i.e., the progressive deviation toward one side when patient walked forward and backward with eyes closed). Patients with PS received higher daily levodopa equivalent daily dose and were more likely to be treated with combination of levodopa and dopamine agonists. Osteoporosis and arthrosis were significantly the most frequent associated medical conditions in patients with PS. Multiple explanatory variable logistic regression models confirmed the association of PS with the following variables: Hoehn and Yahr stage, ongoing combined treatment with levodopa and dopamine agonist, associated medical conditions, and presence of veering gait. Our results suggest that PS is a relatively frequent and often disabling complication in PD, especially in the advanced disease stages. The association is dependent on a number of potentially relevant demographic and clinical variables.

Centronuclear myopathies: genotype-phenotype correlation and frequency of defined genetic forms in an Italian cohort.

Centronuclear myopathies (CNMs) are a group of clinically and genetically heterogeneous muscle disorders. To date, mutation in 7 different genes has been reported to cause CNMs but 30% of cases still remain genetically undefined. Genetic investigations are often expensive and time consuming. Clinical and morphological clues are needed to facilitate genetic tests and to choose the best approach for genetic screening. We aimed to describe genotype-phenotype correlation in an Italian cohort of patients affected by CNMs, to define the relative frequencies of its defined genetic forms and to draw a diagnostic algorithm to address genetic investigations. We recruited patients with CNMs from all the Italian tertiary neuromuscular centers following clinical and histological criteria. All selected patients were screened for the four 'canonical' genes related to CNMs: MTM1, DNM2, RYR1 and BIN1. Pathogenetic mutations were found in 38 of the 54 screened patients (70%), mostly in patients with congenital onset (25 of 30 patients, 83%): 15 in MTM1, 6 in DNM2, 3 in RYR1 and one in TTN. Among the 13 patients with a childhood-adolescence onset, mutations were found in 6 patients (46%), all in DNM2. In the group of the 11 patients with adult onset, mutations were identified in 7 patients (63%), again in DNM2, confirming that variants in this gene are relatively more common in late-onset phenotypes. The present study provides the relative molecular frequency of centronuclear myopathy and of its genetically defined forms in Italy and also proposes a diagnostic algorithm to be used in clinical practice to address genetic investigations.

Clinical and serological features of juvenile systemic lupus erythematosus in an italian tertiary centre of pediatric rheumatology

38 (36.5%) patients, were an important cause of morbidity. Oral ulcers and serositis were found in 27 (25.9%) and 25 (24%) patients respectively. Neurologic involmentent occurred in 20 (19.2%) patients. Seizures were observed in 8 (7.6%) patients. Infections remain a major problem in morbidity: serious infective manifestations occurred in 21 (20.1%) patients. Avascular necrosis of bone occurred in 7 (6.7%) patients, 3 of whom suffered more than 1 episode. Six (5.7%) patients developed steroid induced cataract. Tendon ruptures were observed in 4 (3.8%) patients. Growth failure and established osteoporosis, resulting from prolonged corticosteroid treatment in chronically active disease, were unfrequent but severe complications in our jSLE series occurring respectively in 8 (7.6%) and 5 (4.8%) patients. Cushingoid features and striae rubrae were frequent problems observed in 17 (16.3%) patients, due to disease itself and/or its treatment, that caused increased psychological distress, particularly in adolescents. Finally there were 4 deaths. Two due to infection complications and two due to myocardial infarction.

Changes over time in delivery room management of extremely low birth weight infants in Italy

AimTo identify changes in practice between two historical periods (2002 vs. 2011) in early delivery room (DR) management of ELBWI in Italian tertiary centres. MethodsA questionnaire was sent to the directors of all Italian level III centres between April and August 2012. The same questionnaire was used in a national survey conducted in 2002. Among the participating centres, those that filled the questionnaire in both study periods were selected for inclusion in this study. ResultsThere was an 88% (n=76/86) and 92% (n=98/107) response rate in the 2 surveys, respectively. The two groups overlapped for 64 centres. During the study period, the use of polyethylene bags/wraps increased from 4.7% to 59.4% of the centres. The units using 100% oxygen concentrations to initiate resuscitation of ELBWI decreased from 56.2% to 6.2%. The approach to respiratory management was changed for the majority of the examined issues: positive pressure ventilation (PPV) administered through a T-piece resuscitator (from 14.0% to 85.9%); use of PEEP during PPV (from 35.9% to 95.3%); use of CPAP (from 43.1% to 86.2%). From 2002 to 2011, the percentages of ELBWI intubated in DR decreased in favor of those managed with N-CPAP; ELBWI receiving chest compressions and medications at birth were clinically comparable. ConclusionsDuring the two study periods, the approach to the ELBWI at birth significantly changed. More attention was devoted to temperature control, use of oxygen, and less-invasive respiratory support. Nevertheless, some relevant interventions were not uniformly followed by the surveyed centres.

The spread of drug-resistant tuberculosis in children: an Italian case series

Drug-resistant paediatric tuberculosis (TB) is an overlooked global problem. In Italy, the epidemiology of TB has recently changed and data regarding drug-resistant forms in the paediatric setting is scanty. The aim of this case series was to report the cases of drug-resistant TB, diagnosed between June 2006 and July 2010 in four Italian tertiary centres for paediatric infectious diseases, in children and adolescents living in Italy. Twenty-two children were enrolled, of these 17 were resistant to one or more drugs and five had multidrug-resistant TB. All but one child were either foreign born or had at least one foreign parent. Twenty-one patients completed their treatment without clinical or radiological signs of activity at the end of treatment, and one patient was lost to follow up. The outcomes were good, with few adverse effects using second-line anti-TB drugs. Although this series is limited, it might already reflect the worrisome increase of drug-resistant TB, even in childhood.

Hepatolithiasis-associated cholangiocarcinoma

Few papers focused on association between hepatolithiasis (HL) and cholangiocarcinoma (CCC) in Western countries. The aims of this paper are to describe the clinical presentation, treatment, and postoperative outcomes of CCC with HL in a cohort of Western patients and to compare the surgical outcomes of these patients with patients with CCC without HL. Among 161 patients with HL from five Italian tertiary hepato-biliary centers, 23 (14.3%) patients with concomitant CCC were analyzed. The results of surgery in these patients were compared with patients with CCC without HL. The 60.9% of patients with HL received the diagnosis of CCC intra- or postoperatively, with a resectability rate of 91.3%. The postoperative morbidity was 61.6%. The 1- and 3-year survival rates were 78.6% and 21.0%, respectively. The recurrence rate was 44.4% and the 3-year disease-free survival rates were 18.8%. The comparison with patients with CCC without HL showed a higher resectability rate (p = 0.02) and a higher frequency of earlier stage (p = 0.04) in CCC with HL. Biliary leakage was more frequent in CCC with HL group (p = 0.01) compared to CCC without HL group. We found no differences in overall and disease-free survival between the two groups. Patients with HL and CCC showed a high resectability rate but a higher morbidity. Nevertheless, overall and disease-free survival of patients with CCC and HL showed no differences compared to those of patients with CCC without HL. Also in Western countries, HL needs a careful management for the possible presence of CCC.

Outcome of liver transplantation in children under 10kg: Experience at a single Italian Tertiary Center (University of Padova, 1993–2012) and review of the literature

Background: Infants (<1 year) and small children (<10 kg) represent more than a third of pediatric liver transplant (LT) recipients. It is commonly assumed that young age and low body weight are associated with increased morbidity and mortality after LT.Objective: To evaluate the outcome of LT, in terms of patient and graft survival, in pediatric recipients < 10 kg.Methods: 1. All children with a body weight under 10 kg transplanted at our Institution between January 1993 and May 2012 were retrospectively evaluated. 2. A systematic literature search was conducted through MEDLINE/PUBMED to retrieve all studies, published from January 2000 to September 2012, investigating LT outcomes in children under 10 kg.Results: 1. 30 patients with a median weight of 8.4 kg (4.4-–10) and a median age of 1 year (0.1–3.6) were included in the analysis. All children received cadaveric grafts, of which 66.6% were split livers. Median follow up time per patient was 5.7 years. Overall patient and graft survival were 96.7% and 84.5% respectively. Median re-transplantation and re-operation rates were 13% and 40%. 2. 17 articles, were retrieved. Overall patient survival ranged from 60% to 97%, while graft survival from 56% to 93%. Median re-transplantation and re-operation rates were respectively 7% and 35%.Conclusions: Although technically demanding, LT can be performed in children under 10 kg with excellent patient and graft survival. Post-transplant complication rates are still high in this group of children; thus, further improvements are needed to reduce patient morbidity after transplant. Background: Infants (<1 year) and small children (<10 kg) represent more than a third of pediatric liver transplant (LT) recipients. It is commonly assumed that young age and low body weight are associated with increased morbidity and mortality after LT. Objective: To evaluate the outcome of LT, in terms of patient and graft survival, in pediatric recipients < 10 kg. Methods: 1. All children with a body weight under 10 kg transplanted at our Institution between January 1993 and May 2012 were retrospectively evaluated. 2. A systematic literature search was conducted through MEDLINE/PUBMED to retrieve all studies, published from January 2000 to September 2012, investigating LT outcomes in children under 10 kg. Results: 1. 30 patients with a median weight of 8.4 kg (4.4-–10) and a median age of 1 year (0.1–3.6) were included in the analysis. All children received cadaveric grafts, of which 66.6% were split livers. Median follow up time per patient was 5.7 years. Overall patient and graft survival were 96.7% and 84.5% respectively. Median re-transplantation and re-operation rates were 13% and 40%. 2. 17 articles, were retrieved. Overall patient survival ranged from 60% to 97%, while graft survival from 56% to 93%. Median re-transplantation and re-operation rates were respectively 7% and 35%. Conclusions: Although technically demanding, LT can be performed in children under 10 kg with excellent patient and graft survival. Post-transplant complication rates are still high in this group of children; thus, further improvements are needed to reduce patient morbidity after transplant.

Neurodevelopmental outcome of preterm very low birth weight infants born from 2005 to 2007

ObjectiveTo evaluate short-term neurodevelopmental outcome (at 24 months of corrected age) and correlations with obstetric and neonatal factors in a sample of preterm very low birth weight infants born and admitted to an Italian tertiary centre between 2005 and 2007. Methods156 infants with a birth weight ≤ 1500 g (gestational age, range: 27–31 weeks) were followed at regular intervals through neurodevelopmental (neurological and psychomotor) assessment up to 24 months of corrected age. A statistical analysis was conducted in order to look for correlations between pre- and perinatal variables and neuropsychomotor outcome at 24 months. Results131 children were classified as normal and the other 25 presented sequelae classified as “minor” in 17 cases and as “major” in eight. The most significant risk factors for a poor outcome were preterm premature rupture of the membranes, bronchodysplasia, late-onset sepsis, postnatal steroid therapy and male gender. The presence of severe abnormalities on brain ultrasound scan and of an abnormal neurological assessment at 40 weeks at term equivalent age were strong predictors of poor outcome. ConclusionsOur study is one of the few investigating the short-term outcome of preterm VLBW Italian children born in the second half of the 2000s. Neurodevelopmental assessment at 24 months revealed a marked reduction in major sequelae. Several risk factors for a poor neurodevelopmental outcome identified in children born in earlier periods were confirmed in these children born in recent years.

Favourable survival of in-hospital compared to out-of-hospital refractory cardiac arrest patients treated with extracorporeal membrane oxygenation: An Italian tertiary care centre experience

Extracorporeal membrane oxygenation (ECMO) support has been suggested to improve the survival rate in patients with refractory in- and out-of-hospital cardiac arrest (IHCA and OHCA). The aim of our study is to report our experience with ECMO in these patients. Retrospective, single-centre, observational study. From January 2006 to February 2011 we studied 42 patients (31 males) with refractory cardiac arrest. ECMO implantation was successful in 38 (90%) of the 42 patients. ECMO support was positioned: three times (8%) in the operating room, six (16%) in the cardiac surgery intensive care unit, 21 (55%) in the emergency room, five (13%) in the catheterisation laboratory and three (8%) in the general ward. A total of 14 IHCA (58%) and three OHCA (16%) patients were weaned from ECMO (p<0.05). Eleven IHCA (46%) and one OHCA (5%, p<0.05) patients were discharged from intensive care unit (ICU). Among IHCA patients, 10 were alive at 6 months, nine of whom (38%) with good neurological outcome. Among OHCA patients weaned from ECMO, one was alive at 6 months with good neurological outcome (5%, p<0.05 vs. IHCA). ECMO support should be considered as a resuscitation alternative in selected patients. More specifically, patients with witnessed IHCA benefit more from ECMO treatment compared to those who experience an out-of-hospital cardiac arrest.

Predictors of morbidity in patients with indwelling ureteric stents: results of a prospective study using the validated Ureteric Stent Symptoms Questionnaire

To assess the predictors of morbidity in patients with indwelling ureteric stents using a validated questionnaire. Eighty-six consecutive patients with indwelling double-J ureteric stent of different length and size enrolled at an Italian tertiary academic centre were prospectively evaluated with the Italian-validated Ureteric Stent Symptoms Questionnaire (USSQ), which explores the stent-related symptoms in six domains. Ureteric stents were placed for benign ureteric obstruction or after uncomplicated ureterorenoscopy, and were all removed after 28 days. The questionnaire was administered on days 7 and 28 after stent placement and on day 28 after removal. A plain abdominal X-ray was performed on days 7 and 28 after placement to determine stent location. Univariable and multivariable analyses tested the association of patient age, sex and body mass index (BMI), and stent side, length, calibre and distal loop location, with the index score of the various domains on days 7 and 28. All patients completed the study. At multivariable analysis, on day 7, sex, BMI and stent calibre were significantly associated with one domain (general health, body pain and work performance, respectively), while location of stent distal loop was significantly associated with five domains (urinary symptoms, body pain, general health, work performanc, and sexual matters). On day 28, body mass index was significantly associated with two domains (body pain and general health), while location of stent distal loop remained significantly associated with the same five domains (urinary symptoms, body pain, general health, work performance and sexual matters). Location of stent distal loop with respect to midline had the strongest association with most domains of the USSQ on both days 7 and 28 after stent placement. The visualization of stent distal loop crossing the midline may therefore identify patients at higher risk of post-procedural morbidity requiring early management.