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Background. Early-stage hepatocellular carcinoma could benefit from upfront liver resection (LR) or liver transplantation (LT), but the optimal strategy in terms of tumor-related outcomes is still debated. We compared the oncological outcomes of LR and LT for hepatocellular carcinoma, stratifying the study population into a low-, intermediate-, and high-risk class according to the risk of death at 5-y predicted by a previously developed prognostic model. The impact of tumor pathology on oncological outcomes of low- and intermediate-risk patients undergoing LR was investigated as a secondary outcome. Methods. We performed a retrospective multicentric cohort study involving 2640 patients consecutively treated by LR or LT from 4 tertiary hepatobiliary and transplant centers between 2005 and 2015, focusing on patients amenable to both treatments upfront. Tumor-related survival and overall survival were compared under an intention-to-treat perspective. Results. We identified 468 LR and 579 LT candidates: 512 LT candidates underwent LT, whereas 68 (11.7%) dropped-out for tumor progression. Ninety-nine high-risk patients were selected from each treatment cohort after propensity score matching. Three and 5-y cumulative incidence of tumor-related death were 29.7% and 39.5% versus 17.2% and 18.3% for LR and LT group (P = 0.039), respectively. Low-risk and intermediate-risk patients treated by LR and presenting satellite nodules and microvascular invasion had a significantly higher 5-y incidence of tumor-related death (29.2% versus 12.5%; P < 0.001). Conclusions. High-risk patients showed significantly better intention-to-treat tumor-related survival after upfront LT rather than LR. Cancer-specific survival of low- and intermediate-risk LR patients was significantly impaired by unfavorable pathology, suggesting the application of ab-initio salvage LT in such scenarios.

Primary immunodeficiencies (PIDs) are heterogeneous disorders, characterized by variable clinical and immunological features. National PID registries offer useful insights on the epidemiology, diagnosis, and natural history of these disorders. In 1999, the Italian network for primary immunodeficiencies (IPINet) was established. We report on data collected from the IPINet registry after 20 years of activity. A total of 3352 pediatric and adult patients affected with PIDs are registered in the database. In Italy, a regional distribution trend of PID diagnosis was observed. Based on the updated IUIS classification of 2019, PID distribution in Italy showed that predominantly antibody deficiencies account for the majority of cases (63%), followed by combined immunodeficiencies with associated or syndromic features (22.5%). The overall age at diagnosis was younger for male patients. The minimal prevalence of PIDs in Italy resulted in 5.1 per 100.000 habitants. Mortality was similar to other European registries (4.2%). Immunoglobulin replacement treatment was prescribed to less than one third of the patient cohort. Collectively, this is the first comprehensive description of the PID epidemiology in Italy.

The aim of this study was to evaluate the observers’ diagnostic performance in panoramic radiography using monitor, tablet, X-ray image view box, and against window daylight as a visualization method in different diagnostic tasks. Thirty panoramic radiography were assessed by three calibrated observers for each visualization method, in standardized light conditions, concerning dental caries, widened periodontal ligament space, and periapical bone defects from the four first molars; mucosal thickening and retention cysts in maxillary sinus; and stylo-hyoid ligament calcification and atheroma. A five-point confidence scale was used. The standard-reference was performed by two experienced observers. Diagnostic values using window light were significantly lower for caries and periapical bone defect and retention cyst, stylo-hyoid ligament calcification detection (p<0.05). For atheroma detection, X-ray image view box, tablet, and widow light had lower accuracy than the evaluation on the monitor (p<0.05). Observer’s diagnostic performances are worsened using window light as an evaluation method for panoramic radiography for dental, sinus, and calcification disorders, while the monitor was the most reliable method., El objetivo de este estudio fue evaluar el desempeño diagnóstico de los observadores en la radiografía panorámica utilizando monitor, tablet, caja de visualización de imágenes de rayos X y contra la luz del día de la ventana como método de visualización en diferentes tareas de diagnóstico. Treinta radiografías panorámicas fueron evaluadas por tres observadores calibrados para cada método de visualización, en condiciones de luz estandarizadas, con respecto a caries dental, espacio del ligamento periodontal ensanchado y defectos óseos periapicales de los cuatro primeros molares; engrosamiento de la mucosa y quistes de retención en el seno maxilar; y calcificación y ateroma del ligamento estilohioideo. Se utilizó una escala de confianza de cinco puntos. La referencia estándar fue realizada por dos observadores experimentados. Los valores diagnósticos con luz de ventana fueron significativamente menores para caries y defecto óseo periapical y quiste de retención, detección de calcificación del ligamento estilohioideo (p <0.05). Para la detección de ateroma, la caja de visualización de imágenes de rayos X, el tablet y la luz de viuda tuvieron una precisión menor que la evaluación en el monitor (p <0.05). El rendimiento diagnóstico del observador empeora al utilizar la luz de la ventana como método de evaluación de la radiografía panorámica para los trastornos dentales, de los senos nasales y de la calcificación, mientras que el monitor fue el método más fiable.

This study aimed to describe the imaging aspects of a concha bullosa discovered incidentally by means of cone-beam computed tomography (CBCT) imaging. A female patient underwent a CBCT exam, and a bilateral extensive pneumatization of the middle concha was verified, presenting a larger dimension of the right side and a deviated septum. The patient reported nasal obstruction and loss of smell. The CBCT for covering the maxillofacial region allowed the discovery of this incidental finding, allowing the patient to be referred for appropriate treatment., Este estudio tuvo como objetivo describir los aspectos imaginológicos de una concha bullosa hallada incidentalmente por medio de imágenes de tomografía computarizada de haz cónico (TCHC). Una paciente de sexo femenino fue sometida a examen de TCHC y se verificó una neumatización extensa bilateral de la concha media, presentando una mayor dimensión del lado derecho, asi como desvío del tabique. El paciente refirió obstrucción nasal y pérdida del olfato. La TCHC de la región maxilofacial permitió localizar este hallazgo incidental, permitiendo derivar al paciente para el tratamiento adecuado.

The comprehensive assessment of the transplantable tumor (TT) proposed and included in the last Italian consensus meeting still deserve validation. All consecutive patients with hepatocellular carcinoma (HCC) listed for liver transplant (LT) between January 2005 and December 2015 were post-hoc classified by the tumor/patient stage as assessed at the last re-staging-time (ReS-time) before LT as follow: High-risk-class (HRC) = stages TTDR, TTPR; intermediate-risk-class (IRC) = TT0NT, TTFR, TTUT; low-risk-class (LRC) = TT1, TT0L, TT0C. Of 376 candidates, 330 received LT and 46 dropped-out. Transplanted patients were: HRC for 159 (48.2%); IRC for 63 (19.0%); LRC for 108 (32.7%). Cumulative incidence function (CIF) of tumor recurrence after LT was 21%, 12%, and 8% at 5-years and 27%, 15%, and 12% at 10-years respectively for HRC, IRC, and LRC (P = 0.011). IRC patients had significantly lower CIF of recurrence after LT if transplanted >2-months from ReS-time (28% vs. 3% for <2 and >2 months, P = 0.031). HRC patients had significantly lower CIF of recurrence after-LT if transplanted <2 months from the ReS-time (10% vs. 33% for <2 and >2 months, P = 0.006). The proposed TT staging system can adequately describe the post-LT recurrence, especially in the LRC and HRC patients. The intermediate-risk-class needs to be better defined and further studies on its ability in defining intention-to-treat survival (ITT) and drop-out are required.

The comprehensive assessment of the transplantable tumor (TT) proposed and included in the last Italian consensus meeting still deserve validation. All consecutive patients with hepatocellular carcinoma (HCC) listed for liver transplant (LT) between January 2005 and December 2015 were post-hoc classified by the tumor/patient stage as assessed at the last re-staging-time (ReS-time) before LT as follow: High-risk-class (HRC) = stages TTDR, TTPR; intermediate-risk-class (IRC) = TT0NT, TTFR, TTUT; low-risk-class (LRC) = TT1, TT0L, TT0C. Of 376 candidates, 330 received LT and 46 dropped-out. Transplanted patients were: HRC for 159 (48.2%); IRC for 63 (19.0%); LRC for 108 (32.7%). Cumulative incidence function (CIF) of tumor recurrence after LT was 21%, 12%, and 8% at 5-years and 27%, 15%, and 12% at 10-years respectively for HRC, IRC, and LRC (P = 0.011). IRC patients had significantly lower CIF of recurrence after LT if transplanted >2-months from ReS-time (28% vs. 3% for <2 and >2 months, P = 0.031). HRC patients had significantly lower CIF of recurrence after-LT if transplanted <2 months from the ReS-time (10% vs. 33% for <2 and >2 months, P = 0.006). The proposed TT staging system can adequately describe the post-LT recurrence, especially in the LRC and HRC patients. The intermediate-risk-class needs to be better defined and further studies on its ability in defining intention-to-treat survival (ITT) and drop-out are required.

The Barcelona clinic liver cancer (BCLC) algorithm is the most widely accepted staging system form hepatocellular carcinoma (HCC). Liver resection is traditionally proposed to early stage HCC (BCLC-0/A), even if recent reports have shown that surgical resection could provide a safe and effective treatment also for intermediate-stage HCC (BCLC-B). In this study, we focused on surgical and oncological outcomes of hepatic resection in BLCB-B patients. Patients who received hepatic resection for early- (BCLC-0/A) or intermediate-stage (BCLC-B) HCC in two tertiary hepatobiliary centers between January 2003 and December 2016 were included in study. Four-hundred and twenty-nine patients were included in the analysis. At the time of resection, 298 patients were classified as BCLC-A/0 and 131 as BCLC-B. Despite a higher complication rate in BCLC-B group (49.6% vs 32.9%; p = 0.001), the incidence of clinically relevant complications did not differ significantly between the two groups (16.0% vs 10.1%; p = 0.079); moreover, postoperative mortality (4.6% vs 2.7%; p = 0.309) and relapse-free survival (RFS) were similar between BCLC-0/A and BCLC-B group (1-, 3-, and 5-year RFS: 74, 43, and 31% vs 59, 38, and 34%; p = 0.180). Overall survival was slightly worse in BCLC-B group (1-, 3-, and 5-year overall survival of 89, 70, and 52% vs. 77, 51, and 44%; p = 0.004). Focusing on BCLC-B group, a Child–Pugh score B (HR 2.47; p = 0.003), growing number of nodules (HR 3.04; p = 0.003), and R1 resection (HR 2.43; p = 0.005) beard a higher risk of tumor recurrence, while overall survival was negatively affected by the presence of more than two nodules (HR 3.66; p = 0.0001) and R1 resection (HR 3.06; p = 0.0001); patients presenting single-large HCC experienced a better overall survival (HR 0.53; p = 0.014) and lower recurrence-rate (HR 0.60; p = 0.046). Hepatic resection for intermediate-stage HCC shows acceptable results in terms of perioperative morbidity and mortality, with better oncolo

The use of laparoscopic liver resection (LLR) has progressively spread in the last 10 years. Several studies have shown the superiority of LLR to open liver resection (OLR) in term of perioperative outcomes. With this review, we aim to systematically assess short-term and long-term major outcomes in patients who underwent LLR for hepatocellular carcinoma (HCC) in order to illustrate the advantages of minimally invasive liver surgery. Through an advanced PubMed research, we selected all retrospective, prospective, and comparative clinical trials reporting short-term and long-term outcomes of any series of patients with diagnosis of HCC who underwent laparoscopic or robotic resection. Reviews, meta-analyses, or case reports were excluded. None of the patients included in this review has received a previous locoregional treatment for the same tumor nor has undergone a laparoscopic-assisted procedure. We considered morbidity and mortality for evaluation of major short-term outcomes, and overall survival (OS) and disease-free survival (DFS) for evaluation of long-term outcomes. A total of 1,501 patients from 17 retrospective studies were included, 15 studies compare LLR with OLR. Propensity-score matching (PSM) analysis was used in 11 studies (975 patients). The majority of the studies included patients with good liver function and a single HCC. Cirrhosis at pathology ranged from 33% to 100%. Overall mortality and morbidity ranges were 0–2.4% and 4.9–44% respectively, with most of the complications being Clavien-Dindo grade I or II (range: 3.9–23.3% vs. 0–9.52% for Clavien I–II and ≥ III respectively). The median blood loss ranged from 150 to 389 mL; the range of the median duration of surgery was 134–343 minutes. The maximum rate of conversion was 18.2%. The median duration of hospitalization ranged from 4 to 13 days. The ranges of overall survival rates at 1-, 3- and 5-year were 72.8–100%, 60.7–93.5% and 38–89.7% respectively. The ranges of disease free survival rates

Background: Laparoscopic liver resection (LLR) has gained significant popularity over the last 10 years. First experiences of LLR compared to open liver resection (OLR) reported a similar survival and a better safety profile for LLR. Materials and methods: This is a retrospective analysis of prospectively collected data of all consecutive patients treated by liver resection for HCC on liver cirrhosis between January 2005 and March 2017. The choice of procedure (LLR vs OLR) was generally based on tumor localization, history of previous upper abdominal surgery and patient's preference. The type of resection and indication for surgery were unrelated to the adopted technique. Based on pre-operative variables and confirmed cirrhosis, a 1:1 propensity score matching (PSM) model was developed to compare outcomes of LLR and OLR in patients with HCC. Outcomes of interest included morbidity, mortality and long-term cure potential. Results: After-PSM, the LLR group demonstrated better perioperative results including: lower complication rate (50.7% in OLR vs 29.3% in LLR, p = 0.0035), significantly lower intra-operative blood loss (200 ml in OLR vs 150 ml in LLR, p = 0.007) and shorter hospital length of stay (median 9 days in OLR vs 7 days in LLR, p = 0.0018). Moreover there was no significant difference between the two groups in 3-year survival (76%, CI: 60%–86% in LLR vs 68%, CI: 55%–79% in OLR, p = 0.32) or recurrence-free survival rates (44%, CI: 28%–58%, vs 44%, CI: 31%–57%, p = 0.94). Conclusions: Minor LLR appeared significantly safer compared to minor OLR for HCC. LLR was associated with fewer post-operative complication, lower operative blood loss and a shorter hospital stay along with similar survival and recurrence-free survival rates

Objective: To update the follow-up of the Euro-Lupus Nephritis Trial (ELNT), a randomised prospective trial comparing low-dose (LD) and high-dose (HD) intravenous (IV) cyclophosphamide (CY) followed by azathioprine (AZA) as treatment for proliferative lupus nephritis. Patients and methods: Data for survival and kidney function were prospectively collected during a 10-year period for the 90 patients randomised in the ELNT, except in 6 lost to follow-up. Results: Death, sustained doubling of serum creatinine and end-stage renal disease rates did not differ between the LD and HD group (5/44 (11%) vs 2/46 (4%), 6/44 (14%) vs 5/46 (11%) and 2/44 (5%) vs 4/46 (9%), respectively) nor did mean serum creatinine, 24 h proteinuria and damage score at last follow-up. Most patients in both groups were still treated with glucocorticoids, other immunosuppressant agents and blood pressure lowering drugs. After 10 years of follow-up, the positive predictive value for a good outcome of an early drop in proteinuria in response to initial immunosuppressive therapy was confirmed. Conclusion: The data confirm that a LD IVCY regimen followed by AZA - the "Euro-Lupus regimen" - achieves good clinical results in the very long term

The "Euro-Lupus Cohort" is composed by 1000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium-the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors. © 2005 Elsevier B.V. All rights reserved.

Objective. In the Euro-Lupus Nephritis Trial (ELNT), 90 patients with lupus nephritis were randomly assigned to a high-dose intravenous cyclophosphamide (IV CYC) regimen (6 monthly pulses and 2 quarterly pulses with escalating doses) or a low-dose IV CYC regimen (6 pulses of 500 mg given at intervals of 2 weeks), each of which was followed by azathioprine (AZA). After a median followup of 41 months, a difference in efficacy between the 2 regimens was not observed. The present analysis was undertaken to extend the followup and to identify prognostic factors. Methods. Renal function was prospectively assessed quarterly in all 90 patients except 5 who were lost to followup. Survival curves were derived using the Kaplan-Meier method. Results. After a median followup of 73 months, there was no significant difference in the cumulative probability of end-stage renal disease or doubling of the serum creatinine level in patients who received the low-dose IV CYC regimen versus those who received the high-dose regimen. At long-term followup, 18 patients (8 receiving low-dose and 10 receiving high-dose treatment) had developed permanent renal impairment and were classified as having poor long-term renal outcome. We demonstrated by multivariate analysis that early response to therapy at 6 months (defined as a decrease in serum creatinine level and proteinuria <1 gm/24 hours) was the best predictor of good long-term renal outcome. Conclusion. Long-term followup of patients from the ELNT confirms that, in lupus nephritis, a remission-inducing regimen of low-dose IV CYC followed by AZA achieves clinical results comparable with those obtained with a high-dose regimen. Early response to therapy is predictive of good long-term renal outcome

Objective. Glomerulonephritis is a severe manifestation of systemic lupus erythematosus (SLE) that is usually treated with an extended course of intravenous (IV) cyclophosphamide (CYC). Given the side effects of this regimen, we evaluated the efficacy and the toxicity of a course of low-dose IV CYC prescribed as a remission-inducing treatment, followed by azathioprine (AZA) as a remission-maintaining treatment. Methods. In this multicenter, prospective clinical trial (the Euro-Lupus Nephritis Trial [ELNT]), we randomly assigned 90 SLE patients with proliferative glomerulonephritis to a high-dose IV CYC regimen (6 monthly pulses and 2 quarterly pulses; doses increased according to the white blood cell count nadir) or a low-dose IV CYC regimen (6 fortnightly pulses at a fixed dose of 500 mg), each of which was followed by AZA. Intent-to-treat analyses were performed. Results. Followup continued for a median of 41.3 months in the low-dose group and 41 months in the high-dose group. Sixteen percent of those in the low-dose group and 20% of those in the high-dose group experienced treatment failure (not statistically significant by Kaplan-Meier analysis). Levels of serum creatinine, albumin, C3, 24-hour urinary protein, and the disease activity scores significantly improved in both groups during the first year of followup. Renal remission was achieved in 71% of the low-dose group and 54% of the high-dose group (not statistically significant). Renal flares were noted in 27% of the low-dose group and 29% of the high-dose group. Although episodes of severe infection were more than twice as frequent in the high-dose group, the difference was not statistically significant. Conclusion. The data from the ELNT indicate that in European SLE patients with proliferative lupus nephritis, a remission-inducing regimen of low-dose IV CYC (cumulative dose 3 gm) followed by AZA achieves clinical results comparable to those obtained with a high-dose regimen

The "Euro-Lupus Cohort" is composed by 1,000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium - the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors