Search

Objective To compare oral health status between renal transplant recipients ( RTRs) receiving tacrolimus ( Tac) or everolimus ( ERL) as immunosuppressive therapy. Design This study is a cross-sectional study. Methods Thirty-six RTRs receiving Tac and 22 RTRs receiving ERL were included in the study. Age, gender, time since transplant and pharmacological data were recorded for both groups. Oral health status was assessed through the evaluation of teeth, periodontal parameters as well as saliva flow rate and p H. Results RTRs receiving ERL were older than those receiving Tac. No differences were found between groups concerning oral hygiene habits, oral symptoms, smoking habits, unstimulated and stimulated saliva flow rate and p H, clinical attachment level or the number of decayed, missing and filled teeth. However, RTRs receiving ERL presented lower visible plaque index and lower values for bleeding on probing when compared to RTRs receiving Tac. In addition, RTRs receiving ERL presented a gingival index varying from normal to moderate inflammation whereas RTRs receiving Tac presented a gingival index varying from mild to severe inflammation. Conclusions RTRs receiving ERL have lower periodontal inflammation when compared to RTRs receiving Tac. [ABSTRACT FROM AUTHOR]

The environmental conditions in Madeira Island are favorable for the presence and dissemination of the pinewood nematode (PWN), Bursaphelenchus xylophilus. Five hundred Pinus pinaster wood samples were collected in several forest areas and PWN was detected in 22.8 % of the samples. Bursaphelenchus xylophilus isolates from Madeira Island displayed the species-specific diagnostic characters. A morphological variation in the female tail terminus was detected. In most females, the tail presented a broadly rounded terminus and, occasionally, a digitate terminus with a terminal nipple-like extension resembling a mucro. PCR ITS-RFLP analysis revealed that Madeira Island isolates exhibited patterns specific to the species B. xylophilus and similar to virulent isolates. Amplified ITS regions were further sequenced and no genetic diversity was found for this genomic region among 17 Portuguese isolates (Madeira Island and Continental Portugal). Phylogenetic analysis revealed that Portuguese isolates grouped with isolates from China, Korea and one isolate from Japan. [ABSTRACT FROM AUTHOR]

Pathogen genome sequencing during epidemics enhances our ability to identify and understand suspected clusters and investigate their relationships. Here, we combine genomic and epidemiological data of the 2022 mpox outbreak to better understand early viral spread, diversification and transmission dynamics. By sequencing 52% of the confirmed cases in Portugal, we identified the mpox virus sublineages with the highest impact on case numbers and fitted them into a global context, finding evidence that several international sublineages probably emerged or spread early in Portugal. We estimated a 62% infection reporting rate and that 1.3% of the population of men who have sex with men in Portugal were infected. We infer the critical role played by sexual networks and superspreader gatherings, such as sauna attendance, in the dissemination of mpox virus. Overall, our findings highlight genomic epidemiology as a tool for the real-time monitoring and control of mpox epidemics, and can guide future vaccine policy in a highly susceptible population., (© 2023. The Author(s).)

Introduction: Atopic dermatitis (AD) is a difficult-to-treat inflammatory skin disease with a high impact on patients' quality of life. Dupilumab, an IL-4 and IL-13 inhibitor, was the first monoclonal antibody approved for the treatment of moderate-to-severe AD and is currently approved in patients aged 6 or older., Methods: This is a nationwide, multicenter, retrospective, 48-week study designed by the Portuguese Group of AD to assess real-world efficacy and safety of dupilumab for the treatment of AD., Results: A total of 169 patients were enrolled, with a mean disease duration of 22.75 (±11.98) years. The percentage of patients achieving an improvement of at least 75% in Eczema Area and Severity Index (EASI) compared to baseline (EASI75 response) at weeks 12 and 48 was 67.6% and 74.1%, respectively. In the same timepoints, 25.0% and 44.1% achieved an EASI90 response. Patient-reported outcome measures also improved throughout the study period. Regarding safety, 32.0% of the patients developed adverse events, with conjunctivitis (26.6%), persistent facial erythema (4.7%), and arthritis/arthralgia (3.6%) as the more frequently reported., Conclusion: Data from real-world populations are crucial to guide clinicians in their daily decisions. This study provides data demonstrating that dupilumab is an effective and safe therapeutic option for AD.

Aim: To assess the feasibility of an integrated program of extracorporeal cardiopulmonary resuscitation (ECPR) and uncontrolled donation after circulatory determination of death (uDCDD) in refractory cardiac arrest (rCA)., Methods: Single center, prospective, observational study of selected patients with in-hospital (IHCA) and out-of-hospital (OHCA) rCA occurring in an urban area of ∼1.5 million inhabitants, between October-2016 and May-2018. 65 year old or younger patients without significant bleeding or comorbidities with witnessed nonasystolic cardiac arrests were triaged to ECPR if they had a reversible cause and high quality CPR lasting < 60 min. Otherwise they were considered for uDCDD after a ten minute no touch period using normothermic regional perfusion., Results: 58 patients were included, of which 41 (71%) were OHCA and 18 (31%) had ECPR initiated. Median age was 52 (IQR 45-56) years. Cannulation was successful in 49/58 (84%) cases. Compared to ECPR, patients referred for uDCDD were more frequently OHCA (90 vs. 28%), had bystander CPR (28 vs. 83%) and prolonged low-flow period (40 (35-50) vs. 60 (49-78) min). Survival to hospital discharge with full neurological recovery (cerebral performance category 1) occurred in 6/18 (33%) ECPR patients. uDCDD resulted in transplantation of 44 kidneys., Conclusions: An integrated program for rCA consisting of a formal pathway to uDCDD referral in ECPR ineligible patients is feasible. ECPR-referred patients had a reasonable survival with full neurologic recovery. Successful kidney transplantation was achieved with uDCDD., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Alport syndrome (AS) is caused by pathogenic mutations in the genes encoding α3, α4 or α5 chains of collagen IV (COL4A3/COL4A4/COL4A5), resulting in hematuria, chronic renal failure (CRF), sensorineural hearing loss (SNHL) and ocular abnormalities. Mutations in the X-linked COL4A5 gene have been identified in 85% of the families (XLAS). In this study, 22 of 60 probands (37%) of unrelated Portuguese families, with clinical diagnosis of AS and no evidence of autosomal inheritance, had pathogenic COL4A5 mutations detected by Sanger sequencing and/or multiplex-ligation probe amplification, of which 12 (57%) are novel. Males had more severe and earlier renal and extrarenal complications, but microscopic hematuria was a constant finding irrespective of gender. Nonsense and splice site mutations, as well as small and large deletions, were associated with younger age of onset of SNHL in males, and with higher risk of CRF and SNHL in females. Pathogenic COL4A3 or COL4A4 mutations were subsequently identified in more than half of the families without a pathogenic mutation in COL4A5. The lower than expected prevalence of XLAS in Portuguese families warrants the use of next-generation sequencing for simultaneous COL4A3/COL4A4/COL4A5 analysis, as first-tier approach to the genetic diagnosis of collagen type IV-related nephropathies., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Pathogenic mutations in genes COL4A3/COL4A4 are responsible for autosomal Alport syndrome (AS) and thin basement membrane nephropathy (TBMN). We used Sanger sequencing to analyze all exons and splice site regions of COL4A3/COL4A4, in 40 unrelated Portuguese probands with clinical suspicion of AS/TBMN. To assess genotype-phenotype correlations, we compared clinically relevant phenotypes/outcomes between homozygous/compound heterozygous and apparently heterozygous patients. Seventeen novel and four reportedly pathogenic COL4A3/COL4A4 mutations were identified in 62.5% (25/40) of the probands. Regardless of the mutated gene, all patients with ARAS manifested chronic renal failure (CRF) and hearing loss, whereas a minority of the apparently heterozygous patients had CRF or extrarenal symptoms. CRF was diagnosed at a significantly younger age in patients with ARAS. In our families, the occurrence of COL4A3/COL4A4 mutations was higher, while the prevalence of XLAS was lower than expected. Overall, a pathogenic COL4A3/COL4A4/COL4A5 mutation was identified in >50% of patients with fewer than three of the standard diagnostic criteria of AS. With such a population background, simultaneous next-generation sequencing of all three genes may be recommended as the most expedite approach to diagnose collagen IV-related glomerular basement membrane nephropathies., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Although several studies have demonstrated early survival advantages with peritoneal dialysis (PD) over hemodialysis (HD), the reason for the excess mortality observed among incident HD patients remains to be established, to our knowledge. This study explores the relationship between mortality and dialysis modality, focusing on the role of HD vascular access type at the time of dialysis initiation., Methods: A retrospective cohort study was performed among local adult chronic kidney disease patients who consecutively initiated PD and HD with a tunneled cuffed venous catheter (HD-TCC) or a functional arteriovenous fistula (HD-AVF) in our institution in the year 2008. A total of 152 patients were included in the final analysis (HD-AVF, n = 59; HD-TCC, n = 51; PD, n = 42). All cause and dialysis access-related morbidity/mortality were evaluated at one year. Univariate and multivariate analysis were used to compare the survival of PD patients with those who initiated HD with an AVF or with a TCC., Results: Compared with PD patients, both HD-AVF and HD-TCC patients were more likely to be older (p<0.001) and to have a higher frequency of diabetes mellitus (p = 0.017) and cardiovascular disease (p = 0.020). Overall, HD-TCC patients were more likely to have clinical visits (p = 0.069), emergency room visits (p<0.001) and hospital admissions (p<0.001). At the end of follow-up, HD-TCC patients had a higher rate of dialysis access-related complications (1.53 vs. 0.93 vs. 0.64, per patient-year; p<0.001) and hospitalizations (0.47 vs. 0.07 vs. 0.14, per patient-year; p = 0.034) than HD-AVF and PD patients, respectively. The survival rates at one year were 96.6%, 74.5% and 97.6% for HD-AVF, HD-TCC and PD groups, respectively (p<0.001). In multivariate analysis, HD-TCC use at the time of dialysis initiation was the important factor associated with death (HR 16.128, 95%CI [1.431-181.778], p = 0.024)., Conclusion: Our results suggest that HD vascular access type at the time of renal replacement therapy initiation is an important modifier of the relationship between dialysis modality and survival among incident dialysis patients.

Objectives: In the present study, we determined the cumulative costs and outcomes of endovascular treatment of thrombosed autogenous arteriovenous fistulae (AVF) at our medical center., Background: Previous studies examining the salvage procedures of thrombosed AVFs have focused exclusively on clinical outcomes, and, in the absence of costing data, current guidelines do not take into consideration economic issues., Methods: A retrospective cohort study was performed among local hemodialysis patients with completely thrombosed AVFs receiving endovascular treatment in our institution between January 1 and December 31, 2008. Forty-four patients were enrolled and followed-up for 1 year. Success and complications were recorded according to consensus definitions, and a comprehensive measurement of total vascular access care-related costs was obtained. Costs are reported in 2010 in U.S. dollars., Results: Clinical success was achieved in 95% of cases. The primary and secondary patency rates were 63 and 78% at 1 year, respectively. Primary patency rate at 12 months was significantly better for radiocephalic AVFs (70% vs. 43%; P = 0.047). The mean cumulative cost of all vascular access care during year 1 was $2,504 (median $1,484; range, $1,362-$18,279; Table V) per patient-year at risk. The mean cumulative cost for maintaining radiocephalic and brachiocephalic AVFs was $1,624 (median $1,381; range, $1,130-$3,116) and $3,578 (median $2,092; range, $1,470-$18,279) per patient-year at risk, respectively (P = 0.022)., Conclusion: The cost of maintenance of a thrombosed AVF by endovascular intervention is high, with patients with clotted radiocephalic fistulae incurring the lowest costs and achieving higher survival times., (Copyright © 2010 Wiley-Liss, Inc.)