Autologous peripheral blood stem cell transplantation (aPBSCT) provides optimal outcomes in POEMS syndrome but the definition of the best treatment before aPBSCT remains to be defined, because of the ...disease rarity and the heterogeneity of published case series.
We collected clinical and laboratory data of patients with POEMS syndrome undergoing aPBSCT from 1998 to 2020 in 10 Italian centres. The primary endpoint of the study was to evaluate the impact of prior therapies and mobilizing regimen on outcome.
We divided patients in three groups: patients who did not receive any treatment before transplant (15 patients, group A: front-line), pre-treated patients with other agents (14 patients, group B) and patients treated with cyclophosphamide as mobilizing regimen (16 patients, group C). The three groups did not show differences in terms of demographic and clinical characteristics.
All 45 patients underwent aPBSCT after high dose melphalan conditioning regimen, with a median follow-up of 77 months (37-169 months). The responses were not statistically different between the 3 groups (p 0.38). PFS and OS rates at 6 years were 65% (49-85) and 92% (84-100), respectively and did not differ in the 3 groups. The cumulative incidence of transplant related mortality and relapse was respectively 4% and 36%.
In conclusion, in a relatively large number of patients with POEMS syndrome, undergoing an autologous transplant, pre-treatment and disease status at transplant did not appear to have an impact on major transplant outcomes.
Ectodermal dysplasia (ED) is a genetic disorder affecting anatomical structures with an ectodermal origin. The consequent alveolar bone anomalies and lack of teeth require a multidisciplinary ...approach to rehabilitate patients function and esthetics. To avoid bone grafting procedures the application of zygomatic implants was suggested for the upper jaw treatment. The advancements in three-dimensional (3D) radiology and the introduction of digital implant planning software could enhance the approach to zygomatic implants in ED patients. The present case report describes the quad zygomatic implant treatment of the edentulous maxilla of an ED patient by means of computer guided implant surgery. The patient reported previous failure of bone grafting procedures in the upper jaw. The implants were immediately loaded with a screw-retained complete-arch resin prosthesis. The patient was followed up for 1 year with no biological nor mechanical complications reported, but a slight bone resorption in the anterior zone was observed. Zygomatic implants could be a potential alternative treatment to bone grafting in upper jaw and the digital implant planning could enhance the surgical procedure.
Background
Conduction disorders (CD) are the most common complications after Transcatheter Aortic Valve Implantation (TAVI). The last generation of Edwards balloon expandable valves, the SAPIEN 3 ...Ultra (S3U), is provided with an external sealing skirt that aims to further reduce paravalvular leakage (PVL) compared to SAPIEN 3 (S3) and could potentially lead to higher CD rate. We sought to investigate the rate of new-onset CD in patients undergoing TAVI with the S3 or S3U valve.
Methods
We included 582 consecutive patients undergoing TAVI in a single high-volume Center. Patients with previously implanted pacemaker and Valve in valve procedures were excluded. CD rate was evaluated early after implantation and at discharge.
Results
No significant difference in the overall CD rate was found between S3 and S3U patients both immediately after the procedure (S3 45.5% vs. S3U 41.8%,
p
= 0.575) and at discharge (S3 30.4% vs. S3U 35.6%,
p
= 0.348) with low rate of permanent pacemaker implantation (S3 6.3% vs. S3U 5.5%,
p
= 0.749). No significant differences were found also in patients with pre-existing atrial fibrillation (S3 8.2% vs. S3U 5%,
p
= 0.648). A significantly lower rate of PVL was found with S3U compared to S3 (S3 42% vs. S3U 26%,
p
= 0.007). According to the manufacturer’s guidelines we confirmed that S3U were implanted in a significantly higher position compared to S3 (S3 4.89 ± 1.57 mm vs. S3U 4.47 ± 1.36 mm,
p
= 0.001).
Conclusion
No significant difference in the rate of CD, including the need for PPM implantation, was found in patients undergoing TAVI with the S3 compared to S3U. Moreover, S3U significantly reduced the PVL rate.
► Pathological participants pointed out difficulties in maintaining posture due to proprioceptive system impairments. ► Analysis of entropy seems to provide more insight into mechanisms involved in ...the control of quite stance. ► The results of time, frequency and entropy analysis of posture signal should be taken into account in the planning of rehabilitation programme.
Ligament laxity in Ehlers–Danlos syndrome hypermobility type (EDS-HT) patients can influence the intrinsic information about posture and movement and can have a negative effect on the appropriateness of postural reactions. Several measures have been proposed in literature to describe the planar migration of CoP over the base of support, and the most used in clinical field are the CoP excursions in antero-posterior and medio-lateral direction. In recent years a growing number of studies have been designed to explore the complexity of the COP trajectories during quiet standing. We assessed 13 adults with EDS-HT (EDSG) and 20 healthy adults (CG) during static posture, evaluating the CoP using time and frequency domain analysis and entropy analysis (SampEn and ApEn parameters). Higher values of CoP displacements in medio-lateral and anterior–posterior directions for EDSG than CG were found; no differences were observed in CoP frequency. The entropy analysis showed lower value for EDSG than CG, pointing out the needing of EDSG to concentrate more attention on postural control, loosing complexity and reflecting a less automatized postural control.
The outbreak of coronavirus required adjustment regarding the delivery of interventions. Media literacy interventions are necessary to help people acquire relevant skills to navigate the complexities ...of media communications, and to encourage health-promoting behaviors. The present study aimed to promote a media literacy intervention regarding performance and appearance enhancement substances use in sports high school students. The COVID-19 contingency allowed us to evaluate whether online sessions can effectively promote greater awareness of media influence, a stronger sense of confidence in persuading others to deal with media messages, and healthier attitudes about PAES use among high school students. The study relied on an “intervention group” comprising 162 students (31.5% female) and a “control group” comprising 158 students (42% female). Data were analyzed through repeated measures of Group X Time MANOVA and ANOVA, demonstrating some degree of efficacy of the media literacy intervention. The “intervention group” reported higher awareness of potential newspapers’ influence and a significant increase in their sense of confidence in dealing with media influence compared to the “control group”. Findings support the efficacy of online media literacy programs to prevent doping consumption in adolescents.
Sexual assistance may have some aspects that resemble prostitution and others that might lead one to think of sexual assistants as similar to a group of subjects whose sexual object is disability ...(devotees). In this study, we investigate whether a rigorous selection and training process on the part of specialised organisations may reduce the risk of training subjects with an atypical sexual interest and behaviours resembling prostitution.
The study population consisted of 152 subjects defining themselves as sexual assistants. Subjects were initially contacted on websites specifically dedicated to sexual assistants and prostitutes. One hundred and twenty subjects were selected, by propensity score analysis, and studied by means of a modified version of a semi-structured questionnaire previously developed to investigate a population of subjects attracted by disability.
The study group was composed of 80 trained and 40 untrained sexual assistants, with mean ages of 41.5 (SD +/-12.58) and 44.5 (SD +/-11.62), respectively. A significant number of untrained subjects affirmed that their motivation in carrying out sexual assistance was of a remunerative nature, while this number was lower among the trained assistants (p = 0.001). Nearly all untrained subjects claimed to do one or more of the following activities during sexual assistance: sexual intercourse, oral sex, and masturbation. Among the trained subjects, however, only 47.5% claimed to do one or more of these activities, which means that there is a significant gap between trained and untrained assistants (p<0.0001). The existence of an atypical sexual interest was more evident between untrained rather than between trained subjects (p<0.0001).
Sexual assistance represents a way through which people affected by disabilities may attain the right to explore their sexuality in a safe setting. This can be guaranteed only if sexual assistants are trained and carefully selected by specialised organisations.
The new class of double-mesh carotid stents might reduce embolic complication through a better plaque containment and support.Methods One hundred four consecutive patients with carotid artery ...stenosis and lipid-rich plaque (<60 Hounsfield units on computed tomographic angiography) were randomized to CAS with CW versus RS and MO.MA versus FW in 4 groups: FW + RS (group 1, n = 27), FW + CW (group 2, n = 25), MO.MA + RS (group 3, n = 27), and MO.MA + CW (group 4, n = 25). The primary endpoint was the number of microembolic signals (MES) detected by transcranial Doppler in the following CAS steps: 1) target vessel access with diagnostic catheter; 2) target vessel in-cannulation with guide or MO.MA; 3) lesion wiring; 4) lesion pre-dilation; 5) lesion stent crossing; 6) stent deployment; 7) stent dilation; and 8) device retrieval and deflation. Secondary endpoints were in-hospital and 30-day major adverse cardiac and cerebrovascular events (MACCE) and acute and long-term stent and target vessel external carotid artery (ECA) patency by Doppler ultrasound at 1, 30, and 180 days after the procedureResults No significant differences were found in patient and lesion characteristics among the 4 groups.
Lymphoplasmacytic lymphoma (LPL) is usually associated with a serum IgM paraprotein, corresponding to Waldenström's Macroglobulinemia (WM). Cases presenting with IgG or IgA, or without a monoclonal ...protein are extremely rare. We analyzed clinical characteristics, frontline treatment, and the outcome of 45 patients with non‐IgM LPL, and compared them with a control group of WM patients. The median age was similar, with significantly higher prevalence of females in non‐IgM LPL, than in WM patients (60% vs 39%, P = .016). Patients with non‐IgM LPL more frequently presented with lymphadenopathies (53% vs 15%, P < .001), splenomegaly (22% vs 8%, P = .015) or extranodal involvement (20% vs 8%, P = .05). In non‐IgM LPL a serum monoclonal protein and bone marrow infiltration were less common than in WM patients (69% and 84% of cases respectively, P < .001 for both comparisons). The MYD88 (L265P) mutation was found in 8/19 patients using allele‐specific polymerase chain reaction. A CXCR4 mutation was found in 4/17 cases using Sanger. In 16 patients we performed targeted next‐generation sequencing of genes MYD88, CXCR4, ARID1‐A, KMT2D, NOTCH2, TP53, PRDM1, CD79B, TRAF3, MYBBP1A, TNFAIP3. Seven patients (44%) had a MYD88 mutation (S219C in one), four (25%) a CXCR4 mutation, three (19%) a KMT2D mutation, one (6%) a TP53 mutation and one (6%) a TRAF3 mutation. With a median follow‐up of 55.7 months, 36 non‐IgM LPL patients (80%) were treated. Non‐IgM LPL patients received more frequently anthracycline‐containing regimens, as compared with WM patients, who mainly received alkylating‐based therapies. Five‐year overall survival (OS) was 84%, similar to that of WM patients.
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