Centro Hospitalar do Porto
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Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC)
Get PDFBackground: JIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. The MIWGUC was set up in order to propose specific JIAU activity and response items and to validate their applicability for clinical outcome studies.
Methods: The group consists of 8 paediatric rheumatologists and 7 ophthalmologists. A consensus meeting took place on November 2015 in Barcelona (Spain) with the objective of validating the previously proposed measures. The validation process was based on the results of a prospective open, international, multi-centre, cohort study designed to validate the outcome measures proposed by the initial MIWGUC group meeting in 2012. The meeting used the same Delphi and nominal group technique as previously described in the first paper from the MIWGUC group (Arthritis Care Res 64:1365-72, 2012). Patients were included with a diagnosis of JIA, aged less than 18 years, and with active uveitis or an uveitis flare which required treatment with a disease-modifying anti-rheumatic drug. The proposed outcome measures for uveitis were collected by an ophthalmologist and for arthritis by a paediatric rheumatologist. Patient reported outcome measures were also measured.
Results: A total of 82 patients were enrolled into the validation cohort. Fifty four percent (n = 44) had persistent oligoarthritis followed by rheumatoid factor negative polyarthritis (n = 15, 18%). The mean uveitis disease duration was 3.3 years (SD 3.0). Bilateral eye involvement was reported in 65 (79.3%) patients. The main findings are that the most significant changes, from baseline to 6 months, are found in the AC activity measures of cells and flare. These measures correlate with the presence of pre-existing structural complications and this has implications for the reporting of trials using a single measure as a primary outcome. We also found that visual analogue scales of disease activity showed significant change when reported by the ophthalmologist, rheumatologist and families. The measures formed three relatively distinct groups. The first group of measures comprised uveitis activity, ocular damage and the ophthalmologists' VAS. The second comprised patient reported outcomes including disruption to school attendance. The third group consisted of the rheumatologists' VAS and the joint score.
Conclusions: We propose distinctive and clinically significant measures of disease activity, severity and damage for JIAU. This effort is the initial step for developing a comprehensive outcome measures for JIAU, which incorporates the perspectives of rheumatologists, ophthalmologists, patients and families.info:eu-repo/semantics/publishedVersio
Effects of a nursing care program on functional outcomes in older acute medical in-patients: protocol for a randomized controlled trial
Get PDFBackground: Hospitalization often leads to long periods of bed rest and inactivity which is associated with an increase in length of hospital stay, loss of capacity for basic self-care and discharge into a nursing home.
Objective: This trial aims to verify if a nursing care program centered on basic self-care and predefined physical activity, improves functional outcomes in older hospitalized patients.
Methods: This is a 2-group randomized controlled trial with repeated measures: 182 older acute medical patients will be blindly randomly allocated to the control group (n = 91) or intervention group (n = 91). The intervention will consist of nursing care intervention centered on basic self-care that includes a twice daily walking training, plus privileging pre-established trips to the toilet by walking and all daytime meals seated, off the bed. The main outcome was changes in the number of independent activities of daily living from 2 weeks before admission (baseline) to discharge. Trial registration: ClinicalTrials.gov (Identifier NCT03106064).
Results: This intervention has the potential to change the outcomes of the older patient in the acute setting.
Conclusion: The loss of independence in self-care is determinant in future health care needs. If our hypothesis is correct and demonstrate that this nursing care program centered on basic self-care for older acute medical patients improves functional outcomes, a change in the paradigmatic organization of hospital care may be justifiable.info:eu-repo/semantics/publishedVersio
Hemodialysis Acess - A Creative Attitude is Necessary
Get PDFCreating and maintaining a functional vascular access (VA) is a critical factor in the survival of a dialysis patient. It implies a creative attitude either to maintain its functionality or to build a new one wherever possible, being it autologous or synthetic. We describe the VA history of a 59 years-old male patient, with extreme obesity, which started in 2012 with failed attempts of VA construction in both forearms until a functional brachiocephalic arteriovenous fistula (AVF) in the right upper limb was achieved. However, it required ligation due to severe venous hypertension secondary to central venous disease related to previous CVC use. As he had no good superficial conduit in the left arm we decided to harvest the arterialized right cephalic vein and implant it in the left arm, creating an autologous arteriovenous shunt between the brachial artery and axillary vein (AV). Despite initial patency, it failed irreversibly approximately one year after creation. As no more superficial veins were available in the upper limbs, a prosthetic access was the next step. We decided for a hybrid graft (HG) between the left brachial artery and the AV because of the patient's biotype and scarred axilla that impeded a safe re-intervention on the AV. This graft was used between 2015 and 2017 with multiple interventions to maintain patency. In 2017 a significant diffuse prosthesis deterioration and reduced AVF flow were noticed with no possible segmental reconstruction. We were then forced to proceed with subtotal graft substitution preserving the outflow stented segment of the HG, using an early cannulation graft to prevent CVC use. After this successful reconstruction, the patient started hemodialysis on the following day with no intercurrences registered.info:eu-repo/semantics/publishedVersio
Payment to gamete donors: equality, gender equity, or solidarity?
Get PDFPurpose: Regulation of payment to gamete donors varies substantially across countries. The development of an ethically sustainable governance system of payments in gamete donation demands that the preferences of different stakeholders be heard. This study intends to contribute to improving the understanding of payment to gamete donors by analysing the views of donors and recipients about the preferred form of payment and its associations with their sociodemographic characteristics.
Methods: This cross-sectional study included 70 donors and 172 recipients recruited at the Portuguese Public Bank of Gametes (July 2017-June 2018). Participants completed a self-reported questionnaire. Views about the preferred form of payment were collected through a multiple-choice question and an open-ended item. Associations were quantified through χ2 tests; content analysis was conducted with the open-ended answers.
Results: Both donors (48.6%) and recipients (40.7%) considered that reimbursement is the preferred form of payment to ensure solidarity-based motivations to donate. This option was followed by compensation for non-financial losses (41.4% of donors; 33.7% of recipients) based on gender equity. Preference for a fixed reward (22.7% of recipients; 8.6% of donors) was less frequent among younger donors and married/living with a partner or employed recipients, being based on the promotion of equality.
Conclusion: In the context of the search for cross-border reproductive care and gamete circulation across countries, the findings from this study claim for the need to create solutions for payment to gamete donors that take into account gender equity and are simultaneously sensitive to donor's actual expenses and further health complications.info:eu-repo/semantics/publishedVersio
Guselkumab for the treatment of psoriasis - evidence to date
Get PDFPsoriasis is a chronic, immune-mediated, inflammatory, and debilitating skin disease with significant impact on patients' quality of life. Its pathogenesis is complex and not yet fully understood. However, the IL-23/IL-17 axis is currently considered the main pathogenic pathway in psoriasis. Guselkumab is a fully human immunoglobulin G1 λ (IgG1λ) monoclonal antibody (mAb) that binds to the p19 subunit of IL-23. It is the first of its class, already approved by the US Food and Drug Administration (FDA), as well as the European Medicines Agency (EMA) for the treatment of adult patients with moderate-to-severe plaque psoriasis who are candidates for either systemic therapy or phototherapy. Several clinical trials have demonstrated potential benefits of guselkumab over other already approved immunomodulators in terms of safety and efficacy. The results of the head-to-head trial ECLIPSE were recently released and are addressed in this review. They contribute to the increasing confidence in guselkumab, demonstrating great potential for long-term treatment of psoriasis. However, further long-term data and additional comparative studies will be essential for positioning guselkumab in the therapeutic armamentarium for psoriasis.info:eu-repo/semantics/publishedVersio
Disseminated Well-Differentiated Gastro-Entero-Pancreatic Tumors Are Associated with Metabolic Syndrome
Get PDFThe association of well-differentiated gastro-entero-pancreatic neuroendocrine tumors (WD GEP-NETs) with metabolic syndrome (MetS), abdominal obesity, and fasting glucose abnormalities was recently described. The aim of this study was to evaluate whether the presence of MetS or any MetS individual component was also influenced by GEP-NET characteristics at diagnosis. A cohort of patients with WD GEP-NETs (n = 134), classified according to primary tumor location (gastrointestinal or pancreatic), pathological grading (G1 (Ki67 ≤ 2%) and G2 (>3 ≤ 20%) (WHO 2010), disease extension (localized, loco-regional, and metastatic), and presence of hormonal secretion syndrome (functioning/non-functioning), was evaluated for the presence of MetS criteria. After adjustment for age and gender, the odds of having MetS was significantly higher for patients with WD GEP-NET grade G1 (OR 4.35 95%CI 1.30-14.53) and disseminated disease (OR 4.52 95%CI 1.44-14.15). GEP-NET primary tumor location or secretory syndrome did not influence the risk for MetS. None of the tumor characteristics evaluated were associated with body mass index, fasting plasma glucose category, or any of the individual MetS components. Patients with GEP-NET and MetS depicted a higher risk of presenting a lower tumor grade and disseminated disease. The positive association between MetS and GEP-NET characteristics further highlights the potential link between the two conditions.info:eu-repo/semantics/publishedVersio
Soluble human Suppression of Tumorigenicity 2 is associated with endoscopic activity in patients with moderate-to-severe ulcerative colitis treated with golimumab
Get PDFBackground: Suppressor of Tumorigenicity 2 (ST2) is an IL33 receptor detected in the mucosa and serum of ulcerative colitis (UC) patients. We evaluated soluble ST2 (sST2) as a surrogate biomarker of disease outcome and therapeutic response, in moderate-to-severe UC patients treated with golimumab.
Methods: We conducted an open-label single-arm multicentre prospective study. At screening/baseline, week 6 (W6) and week 16 (W16), clinical and endoscopic activity (total Mayo score), histologic activity (Geboes index) and biomarkers were evaluated.
Results: From 38 patients, 34 (89.5%) completed W6 and 29 (76.3%) completed W16. Mean age (±SD) was 34.6 ± 12.6 years; 55.9% were female. At W16, 62.1% achieved clinical response. Patients with endoscopic activity at W6 (n = 20) had higher baseline sST2 (median, 24.5 versus 18.7 ng/ml, p = 0.026) and no decrease from baseline (median change, 0.8 versus -2.7, p = 0.029). At W6, sST2 levels correlated with endoscopic activity (rs = 0.45, p = 0.007) but not with histological activity (rs = 0.25, p = 0.151). The best cut-offs for endoscopic activity were sST2 = 16.9 ng/ml (sensitivity = 85%; specificity = 71%) and faecal calprotectin (FC) = 353 μg/g (sensitivity = 90%, specificity = 67%). Patients with histological activity at W6 (n = 27) had higher baseline ST2 levels (median, 23.0 versus 13.7 ng/ml, p = 0.035). sST2 did not correlate with FC or serum C-reactive protein. FC levels correlated with histological activity and baseline FC were higher when Geboes ⩾3.1 at W6.
Conclusions: sST2 may be a surrogate biomarker of UC activity and therapeutic response as it correlates with endoscopic and clinical activity at W6 of golimumab treatment, and subjects with endoscopic and histological activity at W6 had higher baseline ST2 levels.This work was supported by Merck Sharp and Dohme, Lda, Portugal, which provided drug and financial support for the interventional study (Protocol Nr MK8259-22)info:eu-repo/semantics/publishedVersio
Abdominal Actinomycosis misdiagnosed as liposarcoma
Get PDFActinomycosis is an uncommon, endogenous, and chronic infection with varied and nonspecific clinical features such as abdominal, pelvic or cervical masses, ulcerative lesions, abscesses, draining fistula, fibrosis, and constitutional symptoms. The disease ensues when the bacteria disrupt the mucosal barrier, invade, and spread throughout interfascial planes. Currently, the diagnosis of actinomycosis is challenging because of its very low frequency and depending on the clinical presentation it may masquerade malignancies. Therapy consists initially in intravenous penicillin, followed by an oral regimen that may be extended until a year of treatment. A timely diagnosis is crucial to avoid extensive therapeutic attempt as surgery. However, a biopsy or drainage of abscesses and fistula's tract may be required not only as a diagnostic procedure as part of the therapy. We report the case of a 72-year-old woman with an abdominal mass initially misdiagnosed as a liposarcoma. A second biopsy of a skin lesion of the abdominal wall made the diagnosis of actinomycosis, avoiding a major surgical procedure. The patient was treated with a long-term course of antibiotics with favorable outcome. Liposarcoma was ruled out after the patient's full recovery with antibiotics and the misdiagnosis was credit to the overconfidence on the immunohistochemical positivity to MDM2.info:eu-repo/semantics/publishedVersio
Leprosy presenting as remitting seronegative symmetrical synovitis with pitting oedema syndrome - a case report
Get PDFBackground: Leprosy typically manifests with skin and peripheral nerve involvement. Musculoskeletal complaints are the third most common, and can be the sole presenting manifestation. They range from arthralgia/arthritis in reactional states to full mimics of systemic rheumatic diseases. Remitting Seronegative Symmetrical Synovitis with Pitting Oedema syndrome has only been described once in a patient with already diagnosed Leprosy.
Case report: A 68-year-old male, from an endemic region of familial amyloid polyneuropathy, presented with an inaugural Remitting Seronegative Symmetrical Synovitis with Pitting Oedema like syndrome, more that 20 years after travelling to Leprosy endemic areas. Arthritis would resurface whenever oral prednisone was tapered, so methotrexate was started, controlling the complaints. Only one year later, after the appearance of peripheral neuropathy and skin lesions, it was possible to diagnose Leprosy, through the identification of Mycobacterium leprae bacilli in a peripheral nerve biopsy.
Conclusion: This report is an example of the heterogeneity of manifestations of Leprosy, namely rheumatic, and the challenge of diagnosing it when typical complaints are absent. It is also a reminder that this disease should be considered whenever a patient with a combination of skin/neurologic/rheumatic complaints has travelled to endemic countries in the past.info:eu-repo/semantics/publishedVersio
Advances in the treatment of hereditary transthyretin amyloidosis: A review
Get PDFIntroduction: Amyloid transthyretin amyloidosis (ATTR) is a progressive and often fatal disease caused by the buildup of mutated (hereditary ATTR [hATTR]; also known as ATTR variant [ATTRv]) or normal transthyretin (wild-type ATTR) throughout the body. Two new therapies-inotersen, an antisense oligonucleotide therapy, and patisiran, an RNA interference therapy-received marketing authorization and represent a significant advance in the treatment of amyloidosis. Herein, we describe the clinical presentation of ATTR, commonly used procedures in its diagnosis, and current treatment landscape for ATTR, with a focus on hATTR.
Methods: A PubMed search from 2008 to September 2018 was conducted to review the literature on ATTR.
Results: Until recently, there have been few treatment options for polyneuropathy of hATTR. Inotersen and patisiran substantially reduce the amyloidogenic precursor protein transthyretin and have demonstrated efficacy in patients with early- and late-stage disease and in slowing or improving neuropathy progression. In contrast, established therapies, such as liver transplantation, typically reserved for patients with early-stage disease, and tafamidis, indicated for the treatment of early-stage disease in Europe, or diflunisal, a nonsteroidal anti-inflammatory drug that is used off-label, are associated with side effects and/or unclear efficacy in certain patient populations. Thus, inotersen and patisiran are positioned to be the preferred therapeutic modalities.
Conclusions: Important differences between inotersen and patisiran, including formulation, dosing, requirements for premedications, and safety monitoring, require an understanding and knowledge of each treatment for informed decision making.info:eu-repo/semantics/publishedVersio