251 research outputs found
B-cell directed immunotherapy as a treatment of giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA).
Rituximab proved effective in inducing and maintaining remission in refractory GCH-AH
Applications of evolutionary computation, EvoApplications 2012: EvoCOMNET, EvoCOMPLEX, EvoFIN, EvoGAMES, EvoHOT, EvoIASP, EvoNUM, EvoPAR, EvoRISK, EvoSTIM, and EvoSTOC : Málaga, Spain, april 11-13, 2012 : proceedings
Bowel perforation from occult ileal involvement after diagnosis in a case of primary mediastinal large B-cell lymphoma
Primary mediastinal large B-cell lymphoma (PMBCL) is confined to the mediastinum or contiguous nodal areas in most cases. Extramediastinal and abdominal involvement, especially at diagnosis, is extremely rare. Our case describes the first case of histologically proven ileal involvement of PMBCL at diagnosis that led to ileal perforation. Positron emission tomography CT could increase the sensitivity of staging by detecting unusual sites of disease localisation, and could impact clinical management
iL CONTRASTO ALL'IMMIGRAZIONE IRREGOLARE
L'esame del contrasto all'immigrazione irregolare nell'ambito dell'Unione europea è svolto attraverso la ricostruzione sistematica e in chiave critica di due sistemi di norme: a) le disposizioni che rendono uniformi i controlli alle frontiere esterne degli Stati membri le quali integrano la c.d. "politica di gestione integrata delle frontiere " e svolgono una funzione preventiva dell'ingresso irregolare di stranieri; b) le disposizioni repressive che, sanzionando in modo uniforme i comportamenti che favoriscono e rendono possibile l'immigrazione irregolare, hanno una funzione deterrente e prevengono i movimenti secondari di persone che potrebbero crearsi qualora alcuni Stati membri avessero un atteggiamento più tollerante verso il fenomeno in esame
Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature
Dynamic Bayesian Networks and Transfer Learning Enable the Development of Deep Sequence-Based Models on Small-Sample Data
The development of prognostic models for rare diseases is often limited by the latter's extremely low prevalence, which, in turn, affects the breadth of modelling techniques that may be applied. Notably, a severe lack of training data may hinder or prevent the implementation of high-capacity models, such as deep neural networks, despite their known effectiveness in predicting adverse outcomes and efficiency in making use of longitudinal data. To address this issue, in the present work, we propose a novel methodological pipeline where we first train a dynamic Bayesian network (DBN) on the few available data to simulate an adequate number of virtual patients whose variables are linked by the same probabilistic relationship over time as in the original data; then, we train a deep learning model based on recurrent neural networks on the simulated data; and, finally, we apply finetuning, a transfer learning (TL) technique, to adapt the model to the real data. To demonstrate the potential usefulness of our approach, we apply it to the prediction of 3-year mortality in amyotrophic lateral sclerosis (ALS), a rare (<0.01% prevalence), fatal neurodegenerative disease, starting from a population of 985 patients from the Piemonte and Valle d'Aosta ALS (PARALS) register. We show that our pipeline of DBN and TL effectively combines the simulated data it is able to generate and the few available real data, leading to an 8.2% AUROC improvement over a reference deep learning model trained only on the real data
Treatment of immune thrombocytopenia (ITP) secondary to malignancy: a systematic review
Immune thrombocytopenia (ITP) can be associated with lymphoproliferative diseases (LPD) or solid tumors. A systematic review of published literature was conducted to evaluate response to treatment of ITP secondary to malignancy. Primary outcome was overall response (complete response+response) to first-line treatments [steroids alone or in combination with intravenous immunoglobulins (IVIg)]. Among secondary outcomes, overall response to second-line treatments [splenectomy, rituximab or thrombopoietin receptor agonists (TPO-RA)] and death were evaluated. Of the retrieved 238 text articles, 108 were analyzable, for a total of 154 patients: 142 in 105 case reports and 12 in 3 observational studies. Thirty-nine patients had solid tumors, 114 LPD, and 1 both. The median follow up was 19 months (IQR, 9–40). The overall response was 50% (62% in solid tumors, 46% in LPD) after steroids and 47% (67% in solid tumors, 36% in LPD) after steroids+IVIg, which are lower than historical responses observed in primary ITP (≈80%). The overall responses to rituximab (used in LPD only), splenectomy and TPO-RA (70%, 73% and 92%, respectively) were similar to those observed in primary ITP. Seven patients (6%) died due to bleeding events. ITP secondary to malignancy appears to be associated with unsatisfactory response to first-line treatments
B-cell depletion induces prolonged remission in patients with giant cell hepatitis and autoimmune hemolytic anemia
Background: Giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA) is a rare and severe immune-mediated disorder. Despite aggressive immunosuppressive treatments, the mortality is high. Prednisone has been effectively employed to achieve remission, but with a risk of relapse, if discontinued, and with severe side effects. A possible causative role of humoral immune response has paved the way to anti CD-20 monoclonal antibody (rituximab; RTX). Nevertheless, data about timing of remission and long-term side effects are sparse. Methods and matherials: We have retrospectively evaluated 3 refractory GCH-AHA patients in whom a prolonged remission has been achieved with RTX. In all patients, response to first and second line therapy was incomplete or transitory and severe steroid side effects occurred. Results: A stable and sustained remission was achieved after multiple doses of RTX allowing withdrawing all the other treatments. No life-threatening infections have been recorded, however two patients developed persistent, paucisymptomatic hypogammaglobulinaemia. The only patient who did not develop hypogammaglobulinemia received IgG replacement during RTX. Conclusion: RTX induced complete and long-lasting remission allowing discontinuing all the other immunosuppressive drugs. A persistent, paucisymptomatic hypogammaglobulinaemia has been the unique side effect. Although further studies need to replicate our data, RTX can be considered as an effective and safe therapy for sustained remission in patients with severe refractory GCH-AHA
Polymyositis in solid organ transplant recipients receiving tacrolimus.
Tacrolimus, also known as FK506, is an immunosuppressive agent widely used for the prevention of acute allograft rejection in organ transplantation and for the treatment of immunological diseases. This study reports two male patients who underwent solid organ transplantation (liver and kidney). After transplant, the patients received continuous immunosuppressive therapy with oral tacrolimus and later presented clinical manifestations and laboratory signs of myopathy. Muscle biopsies of both patients clearly documented an inflammatory myopathy with the histological features of polymyositis including CD8 + T cells which invaded healthy muscle fibers and expressed granzyme B and perforin, many CD68 + macrophages and MHC class I antigen upregulation on the surface of most fibers. Because of the temporal association while receiving tacrolimus and since other possible causes for myopathy were excluded, the most likely cause of polymyositis in our patients was tacrolimus toxicity. We suggest that patients on tacrolimus should be carefully monitored for serum CK levels and clinical signs of muscle diseas
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