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Background Asthma is a chronic inflammatory disease that is very common among youth worldwide. The burden of this illness is very high not only considering financial costs but also on emotional and social functioning. Guidelines and many researches recommend to develop a good communication between physicians and children/caregiver and their parents. Nevertheless, a previous Italian project showed some criticalities in paediatric severe asthma management. The consensus gathered together experts in paediatric asthma management, experts in narrative medicine and patient associations with the aim of identify simple recommendation to improve communication strategies. Methods Participants to the consensus received the results of the project and a selection of narratives two weeks before the meeting. The meeting was structured in plenary session and in three working groups discussing respectively about communication strategies with children, adolescents and parents. The task of each working group was to identify the most effective (DO) and least effective practices (DON’ T) for 5 phases of the visit: welcome, comprehension of the context, emotions management, duration and end of the visit and endurance of the relationship. Results Participants agreed that good relationships translate into positive outcomes and reached consensus on communication strategies to implement in the different phase of relationships. Conclusions The future challenges identified by the participants are the dissemination of this Consensus document and the implementation of effective communication strategies to improve the management of pediatric asthma.

Chronic spontaneous urticaria (CSU) is a clinical condition characterized by spontaneous or inducible recurrent wheals. This condition may significantly affect quality of life of patients and of their families. Etiology is not identified in 25-85% of cases that are indicated as 'idiopathic', because all diagnostic tests are negative. Autoimmune processes may be present in 30-50% of patients, although a definite etiological diagnosis is seldom possible. Some patients, in fact, have autoantibodies against the high-affinity IgE receptor FceR1 or IgE. These patients show an increased incidence of anti-thyroid autoantibodies and represent 30-50% of the patients designated as having CSU. Familial cold autoinflammatory syndrome (FCAS) must be distinguished from acquired cold urticaria, which is characterized by a rash occurring within a few minutes after cold exposure, and is often described as 'allergy to cold'. Cold urticaria (CU) is rare in childhood and is not linked to inflammatory markers. The treatment is based on antihistamines. However, in non-responders, a second-line treatment with omalizumab can show efficacy. We describe the clinical case of a 9-year-old-female with recurrent monthly episodes of fever, arthralgia, abdominal pain, and urticaria-angioedema who did not respond to steroids associated with antihistamines, however, showed the complete resolution of the disease with omalizumab.

Compared with the general population, incarcerated people have a higher prevalence of several communicable diseases, including viral hepatitis. Nevertheless, there is still a lack of literature in field. Our review aims to shed the actual knowledge on viral hepatitis among incarcerated people in Italy. We performed a comprehensive literature search, through key electronic databases (Scopus, Pubmed–MEDLINE) and search engines (Google Scholar), of peer-reviewed publications (articles and reviews), grey literature on viral hepatitis prevalence, and models proposed for active case finding and control strategies in prison settings. We found that viral hepatitis epidemiology drastically changed in the last five years, particularly on hepatitis C virus (HCV), reporting an HCV antibody (HCV-Ab) prevalence decrease from up to 38% to ˂20% in penitentiary institutes, as well as an even more important reduction in active infections. Probably, the availability of direct-acting antivirals is contributing to this scenario. However, there is a lack of data available regarding incarcerated women. For this reason, more tailored interventions are needed for this sub-population. Judiciary and regulatory bodies should be prompted to discuss and define specific regulations to optimize case active finding strategies, guarantee wide access to effective preventive and treatment options for viral hepatitis and enhance treatment management.

Aims Our observational study aimed to evaluate the impact of the lockdown period due to 2019 Coronavirus disease pandemic on glycaemic control in a cohort of paediatric patients with type 1 diabetes (T1D). Methods Eighty-five patients with T1D aged 5–18 years using continuous glucose monitoring (CGM) systems were enrolled. Demographic and clinical data, including glucose metrics generated by CGM-specific web-based cloud platforms, were collected in three different periods (pre-lockdown phase, lockdown phase, and post-lockdown phase) of 90 days each and were statistically analysed. Results During the lockdown period, a clear improvement in almost all CGM metrics (time in range, time above range, coefficient of variation, and glucose management indicator) was observed in our study population, regardless of age and insulin type treatment. In the months following lockdown, maintaining satisfactory diabetes outcomes was confirmed only in younger patients (aged 5–9 years) and in those individuals on hybrid closed loop therapy. Conclusions The increasing use of innovative technological devices together with data sharing systems and interaction with multidisciplinary diabetes team through telemedicine allowed paediatric patients with T1D to improve glucose metrics during the lockdown period. However, our findings showed that the achievement of better glycaemic control was transient for most patients.

This Phase II study assessed feasibility and efficacy of a biweekly R-COMP-14 regimen (rituximab, cyclophosphamide, non-pegylated liposome-encapsulated doxorubicin, vincristine and prednisone) in untreated elderly patients with poor-risk diffuse large B-cell lymphoma (DLBCL) and moderate to high ‘life threat’ impact NIA/NCI cardiac comorbidity. A total of 208 courses were delivered, with close cardiac monitoring, to 41 patients (median age: 73 years, range: 62–82; 37% >75 years) at a median interval of 15·6 (range, 13–29) days; 67% completed all six scheduled courses. Response rate was 73%, with 68% complete responses (CR); 4-year disease-free survival (DFS) and time to treatment failure (TTF) were 72% and 49%, respectively. Failures were due to early death (n = 3), therapy discontinuations (no-response n = 2; toxicity n = 6), relapse (n = 6) and death in CR (n = 3). Incidence of cardiac grade 3–5 adverse events was 7/41 (17%; 95% confidence interval: 8–31%). Time to progression and overall survival at 4-years were 77% and 67%, respectively. The Age-adjusted Charlson Comorbidity Index (aaCCI) correlated with failures (P = 0·007) with patients scoring ≤7 having a longer TTF (66% vs. 29%; P = 0·009). R-COMP-14 is feasible and ensures a substantial DFS to poor-risk DLBCL patients who would have been denied anthracycline-based treatment due to cardiac morbidity. The aaCCI predicted both treatment discontinuation rate and TTF.

Monoclonal gammopathies are characterized by serum monoclonal component (MC) plus an intact immunoglobulin and a free light chain (FLC), or a combination of both. The measurement of FLC with Freelite® is the standard practice recommended by International Myeloma Working Group guidelines. Recently, Hevylite® heavy/light chains (HLC) assays were introduced to specifically target junctional epitopes between the heavy and light chains of intact immunoglobulins, allowing the independent quantification of the involved (MC) and uninvolved (polyclonal immunoglobulin background) HLC isotype. Between January 2012 and March 2014, 90 patients were examined: 49 multiple myeloma (MM), 6 smoldering MM (SMM) and 35 monoclonal gammopathy of undetermined significance (MGUS). Of these 90 patients, 300 samples were collected at different times. The diagnostic and monitoring contribution of Hevylite A and G assays was assessed in all 90 patients examined. Additionally, 3 representative cases were selected. The Hevylite absolute values and ratio demonstrated high sensitivity and specificity with respect to serum protein electrophoresis and serum immunofixation. The combined use of Hevylite A and G with Freelite was particularly useful in dubious cases with more than one MC or with co-migrating components, as well as in the course of monitoring to assess the independent change of FLC and HLC, possibly reflecting the presence of clonal heterogeneity in the cohort. From this study, it can be concluded that FLC and HLC are independent, useful markers to monitor the MC and to assess with greater specificity and sensitivity the effect of therapy, thereby providing clinical support. Further studies are required to assess the prognostic potential of Hevylite in MGUS and SMM.

SUMMARYObjective — This study explored the effectiveness of a psychoeducational family intervention for schizophrenia on patients' clinical status and disability and relatives' burden and perceived support. Methods — The study has been carried out in 17 mental health centres. In each of them, 2 professionals were trained in a psychoeducational intervention and applied it for six months with families of users with schizophrenia. At baseline and six months later, patients' clinical status and disability, and relatives' burden, social network and professional support were assessed by validated tools. Results — Of the seventy-one recruited families, 48 (68%) completed the intervention. At six months, a significant improvement was found in patients' clinical status and social functioning, as well as in relatives' burden and social and professional support. In particular, the percentage of patients with poor or very poor global social functioning dropped from 50% to 27% at six months. Forty percent of patients and 45% of relatives reported a significant improvement in their social contacts over the intervention period. Conclusions — The results of this study confirm the hypothesis that psychoeducational family interventions may have a significant effect on social outcome and family burden in schizophrenia when provided in routine conditions.Declaration of Interest: none of the authors has had any interest or he/she has received any form of support, including that from drug companies and honoraria for lectures and consultancies, potentially in conflict with this scientific work. None of the authors has received any form of fee for his/her participation in this study. This study was supported by grants received from the “M. Lugli” Foundation (grant n. 2/18/8) and from the National Institute of Health, Italy (grant no. 1AL/F3).

The incidence of non-Hodgkin lymphomas increases with age. Non-pegylated liposomal formulations of doxorubicin (Myocet®) reduce systemic and cardiac toxicity especially in the elderly, who often have cardiac diseases. We treated 80 patients (mean age 70.9 years) with poor-risk diffuse large B-cell lymphoma with the R-COMP 21 regimen (Myocet® 50 mg/m(2), cyclophosphamide 750 mg/m(2), vincristine 1.4 mg/m(2), rituximab 375 mg/m(2), prednisone 100 mg/day). In all, 82.5% and 13.7% patients showed complete and partial responses, respectively. Sixty-two of the 80 patients are alive and disease-free (77.5%), while 3/80 are alive with active disease and 15 patients (18.7%) have died (median follow-up: 31 months). The estimated probability of overall survival at 12/24 months from admission was 93.5/87.3%, respectively. There were no therapy-related cardiac events and the ejection fraction improved (from 51.6 ± 6.9% to 54.2 ± 3.9%). Grade 3-4 neutropenia occurred in 22% of patients. We concluded that Myocet® shows both efficacy and tolerability, mainly at the cardiac level.

Functional impairment of dendritic cells (DC) appears to be one of the mechanisms responsible for tumor escape from the control of the immune system. DC isolated from tumor-bearing animals and cancer patients with solid or with hematological malignancies have phenotypic and functional abnormalities. In addition, supernatants from in vitro cultured tumor cells have been shown to interfere with DC differentiation from CD34+ and monocyte precursors. Primary effusion lymphoma (PEL) is a Human Herpesvirus-8 (HHV-8)-associated tumor, which releases several cytokines such as IL-6, IL-10 and VEGF and its growth seems to be dependent on them in vitro or in vivo. In the present study, we found that these cytokines released by PELs have also an important role in interfering with the in vitro differentiation of immature DC (iDC) from CD14+ monocytes. The iDC obtained in the presence of PEL supernatants showed reduction of FITC-dextran uptake, reduction of MLR allostimulatory activity and altered expression of surface molecules, suggesting that cytokines released by PEL adversely affect DC differentiation.

Silvia Scurati1, Franco Frati1, Gianni Passalacqua2, Paola Puccinelli1, Cecile Hilaire1, Cristoforo Incorvaia3, Italian Study Group on SLIT Compliance 1Scientific and Medical Department, Stallergenes, Milan, Italy; 2Allergy and Respiratory Diseases, Department of Internal Medicine, Genoa; 3Allergy/Pulmonary Rehabilitation, ICP Hospital, Milan, ItalyObjectives: Sublingual immunotherapy (SLIT) is a viable alternative to subcutaneous immunotherapy to treat allergic rhinitis and asthma, and is widely used in clinical practice in many European countries. The clinical efficacy of SLIT has been established in a number of clinical trials and meta-analyses. However, because SLIT is self-administered by patients without medical supervision, the degree of patient adherence with treatment is still a concern. The objective of this study was to evaluate the perception by allergists of issues related to SLIT adherence.Methods: We performed a questionnaire-based survey of 296 Italian allergists, based on the adherence issues known from previous studies. The perception of importance of each item was assessed by a VAS scale ranging from 0 to 10.Results: Patient perception of clinical efficacy was considered the most important factor (ranked 1 by 54% of allergists), followed by the possibility of reimbursement (ranked 1 by 34%), and by the absence of side effects (ranked 1 by 21%). Patient education, regular follow-up, and ease of use of SLIT were ranked first by less than 20% of allergists.Conclusion: These findings indicate that clinical efficacy, cost, and side effects are perceived as the major issues influencing patient adherence to SLIT, and that further improvement of adherence is likely to be achieved by improving the patient information provided by prescribers.Keywords: adherence, sublingual immunotherapy, efficacy, cost, side effects

The esophagus carcinoma has an incidence rate of 3–4 new cases per 100,000 inhabitants, per year, with a male/female ratio of 3:1 and with a low incidence between the VI–VII decade of life. In Italy, the incidence rate equals 2,000 new cases/year with a mortality rate of 3–5/100,000 inhabitants.

A multicenter randomized open-label long-term sequential deferiprone–deferoxamine (DFP-DFO) versus DFP alone trial (sequential DFP-DFO) performed in patients with thalassemia major (TM) was retrospectively reanalyzed to assess the variation in the left ventricular ejection fraction (LVEF) [1].

BACKGROUND CA 125 is a tumor marker used for the diagnosis and monitoring of ovarian carcinoma. This marker also has been found to be increased in patients with serosal effusion derived from nonneoplastic inflammatory disease and in a few instances of advanced non-Hodgkin lymphoma with serosal involvement. METHODS CA 125 levels were tested in the serum of 15 patients with acute myeloblastic leukemia (AML) at the time of diagnosis and in 3 patients with advanced leukemia with serosal involvement. In two patients with elevated serum CA 125 levels, a CA 125 assay was performed on leukemic cells and on the supernatant fluid of short term liquid culture. RESULTS Increased serum CA 125 was found in the three patients with acute leukemia with extramedullary localization and serosal effusion, whereas it was normal in 15 AML patients tested at the time of diagnosis. CA 125 was not detectable in leukemic cell extracts nor in the supernatant fluid of primary cultures. CONCLUSIONS These results indicate that leukemic cells were unable to produce CA 125 and suggest that its elevation in the serum is likely due to a serosal inflammatory reaction caused by the leukemic infiltration. Cancer 2000;88:75–8. © 2000 American Cancer Society.

To better understand the molecular mechanisms underlying the dendritic cell (DC) defects in cancer, we analyzed which signaling pathway is implicated in the abnormal monocyte differentiation into DC determined by the presence of Primary effusion lymphoma (PEL) released factors. Our results indicate that the DC, obtained in this condition, together with phenotypic abnormalities and reduced allostimulatory function, showed hyperphosphorylation of signal transducer and activator of transcription 3 (STAT3) and p38 mitogen-activated protein kinase (MAPK) molecules, in comparison to the DC differentiated in the absence of PEL-released factors. The inhibition of p38 MAPK but not of STAT3 phosphorylation, with specific inhibitors, was able to revert the effect of the PEL-released factors on the DC phenotype. This study suggests that p38 MAPK signaling pathway is an important contributor to the abnormal differentiation of DC in PEL.

Caveolin-3, a muscle specific caveolin-related protein, is the principal structural protein of caveolar membranes. We have recently identified an autosomal dominant form of limb girdle muscular dystrophy (LGMD-1C) that is due to caveolin-3 deficiency and caveolin-3 gene mutations. Here, we studied by electron microscopy, including freeze-fracture and lanthanum staining, the distribution of caveolae and the organization of the T-tubule system in caveolin-3 deficient human muscle fibers. We found a severe impairment of caveolae formation at the muscle cell surface, demonstrating that caveolin-3 is essential for the formation and organization of caveolae in muscle fibers. In addition, we also detected a striking disorganization of the T-system openings at the sub-sarcolemmal level in LGMD-1C muscle fibers. These observations provide new perspectives in our understanding of the role of caveolin-3 in muscle and of the pathogenesis of muscle weakness in caveolin-3 deficient muscle.

Caveolae are small pockets or invaginations localized at the plasma membrane. Caveolins are the principal protein components of caveolae and play an important structural role in the formation of caveolae membranes. Here, we studied by freeze fracture and immunological techniques the spatial organization of caveolae at the muscle cell plasma membrane and the expression of caveolin-3 in Duchenne muscular dystrophy (DMD) muscle fibers. In DMD muscle, we found an increased number of caveolae at the sarcolemma that corresponds to an overexpression of caveolin-3 by immunohistochemistry and by Western blot analysis. These findings suggest a possible role for caveolae and caveolin-3 in the pathogenesis of DMD.

Anterior cruciate ligament reconstruction with patellar tendon was performed in 50 rabbits (two groups of 25 animals) by the outside-in (Group I) and the inside-out (Group II) techniques. Five animals from each group were sacrificed at different times (2 weeks, 1, 3, 6, and 9 months). Histologic analysis showed that the intraarticular part of the graft was morphologically similar to a normal ligament in both groups at 9 months. In Group 1, a newly formed bone-graft junction along the tunnel walls was observed inside the femoral tunnel. At 6 months, this junction resembled a direct type junction. The old bone-tendon junction showed an early disappearance of the fibrocartilage and was differentiated as a direct junction only at 9 months. In Group 2, at the site of the old bone-tendon junction a fibrocartilaginous layer was present during the whole process of remodeling, and at 6 months this area resembled a direct junction. These observations would suggest that when the junction is placed inside the tunnel (outside-in technique) the process of remodeling is more dramatic and slower than when it is placed at the intraarticular exit of the tunnel (inside-out technique), probably because of the formation of a new bone-graft junction along the tunnel walls that partially unload the old junction.

The purpose of this study was to analyze age-related changes in the coracoacromial arch and correlate these degenerative changes with rotator cuff tears. We obtained 80 shoulders from 40 cadavers. The mean age at death was 58.4 years. We performed a gross examination of the rotator cuff and the acromion and histological examination of the coracoacromial ligament. The statistical significance of any difference for each group considered was determined by Student's t-test. The rotator cuff was normal in 66 specimens; there was an articular-side partial tear in 4 cases, a bursal-side partial tear in 6 cases, and a full-thickness tear in 4 cases. Age was correlated with increasing incidence and severity of cuff tears. We noted age-related degenerative changes in the coracoacromial ligament, degeneration of the acromial bone-ligament junction, and acromial spur formation. Anterior acromial spur was not related to the morphology of the acromion. We observed an increased incidence of bursal-side and complete cuff tears when the acromion was curved or beaked. Degenerative changes in the undersurface of the acromion were also present when the rotator cuff was normal. Bursal-side and complete cuff tears were associated with severe degenerative changes in the acromion in 100% of cases. Articular-side cuff tears were not related either to acromial morphology or degenerative changes in the coracoacromial arch. The association between cuff tears and acromial spur was more evident in the presence of a type III acromion. Our results would suggest that the incidence and severity of rotator cuff tears are correlated with aging and with the morphology of the acromion. Rotator cuff tears that involve the bursal side are often associated with changes in the coracoacromial ligament and the undersurface of the acromion. However, degenerative changes in the coracoacromial arch are always related to aging, also in the presence of a normal rotator cuff. Articular-side partial tears do not cause damage to the undersurface of the acromion.

Congenital thrombotic risk factors, oncological diseases and its therapies have been related to an increased occurrence of upper extremities deep venous thrombosis (UEDVT). We studied seven patients bearing lymphoma (one Hodgkin's and six non-Hodgkin's) who developed UEDVT, one at diagnosis and six during chemotherapy (two of these six cases had implantation of a central venous catheter and four received Growth Colony Stimulating Factors in addition to chemotherapy). Patients were screened for: factor V G1691A (Leiden), prothrombin G20210A, methylene tetrahydrofolate reductase (MTHFR) C677T mutations and antithrombin III, proteins C and S plasma activity. All patients were wild-type homozygotes for G20210A. One was heterozygote for factor V G1691A, the other 6 were wild-type homozygotes. Three of the 7 patients were homozygotes and 2 heterozygotes for the MTHFR mutation; the remaining 2 were wild-type homozygotes. Clotting inhibitor levels were normal in all patients. UEDVT in patients bearing haematological malignancies can occur irrespective of congenital thrombophilic alterations. However, in a subgroup of patients UEDVT could also depend on congenital thrombophilic alterations. A screening for inherited thrombophilia can identify high risk patients that could be specifically treated to prevent thrombotic complications.