Centro Hospitalar do Porto
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Keratoconus and Visual Performance with Different Contact Lenses
Get PDFPurpose: To analyze the visual performance in contact lens wearers with keratoconus.
Methods: A retrospective study including contact lens (CL) wearers was performed. The current best-corrected visual acuity with contact lens (BCVA-CL) and with spectacles (BCVA-S) correction, contrast sensitivity (CS) (by Metrovision-MonPack3®), analysis of light scattering in the retina and vision break-up time (HD Analyzer®), and corneal tomography (Oculus Pentacam® HR) were evaluated.
Results: This study included 96 eyes of 59 patients with Keratoconus. Rigid gas permeable contact lenses (RGPCL), hybrid contact lenses (HCL), and silicone hydrogel/hydrogel contact lenses (HGCL) were fitted in 67, 17, and 12 eyes, respectively. Dynamic objective scatter index (OSI) (p = 0.024), minimum OSI (p = 0.037) and maximum OSI (p = 0.040) were significantly better with RGPCL and worse with HGCL. Mean CS in photopic conditions was significantly worse with HGCL and better with HCL (p = 0.006), without differences in mesopic conditions (p = 0.121). RGPCL wearers showed a higher mean K (p = 0.020), and a lower corneal thickness at the thinnest point (p=0.011).
Conclusion: Visual quality varied significantly with different types of CL. Although RGPCL was fitted in patients with worse Pentacam tomographic parameters, RGPCL was associated with a better dynamic visual quality.info:eu-repo/semantics/publishedVersio
Intravitreal Ranibizumab or Aflibercept After Bevacizumab in Diabetic Macular Edema: Exploratory Retrospective Analysis
Get PDFAim: To evaluate the efficacy of switching from bevacizumab to ranibizumab or aflibercept in eyes with diabetic macular edema (DME) unresponsive to bevacizumab.
Methods: Single-center retrospective comparative study of patients with DME unresponsive to intravitreal bevacizumab that was switched to ranibizumab or aflibercept. Best-corrected visual acuity (BCVA) and central foveal thickness (CFT) were analysed prior to and 4 months after the switch. Ocular coherence tomography (OCT) biomarkers were also analysed.
Results: Fifty-six eyes from 40 patients were included in the study, 33 eyes switched to ranibizumab and 23 to aflibercept. A significant median CFT decrease was observed in both groups (p<0.001), with no between-group differences. BCVA gain was only significant in the ranibizumab group (p<0.001). None of the pre-baseline or baseline parameters were associated with the response to ranibizumab or aflibercept.
Conclusion: In persistent DME unresponsive to bevacizumab, both anatomical and functional improvements were observed with ranibizumab whereas aflibercept only showed an anatomical improvement. Clinicaltrials.gov NCT04018833.info:eu-repo/semantics/publishedVersio
Total Knee Arthroplasty for Osteoarthritis in a Patient with Nail Patella Syndrome – A Case Report
Get PDFHereditary osteo-onychodysplasia (or nail patella syndrome) is a rare autosomal dominant disease defined by an association of severe characteristics, with a classic clinical tetrad of dysplasia of the fingernails, patellar hypoplasia or absence, presence of iliac horns, and deformities of the elbow, although renal, ocular and neurological involvement may also be present. The main orthopedic complaint of these patients is patellar instability associated with pain and functional limitation. Most of the literature regarding the treatment of NPS-related patellar problems, concerns pediatric population and there is no treatment algorithm defined for adult patients. NPS has a wide range of presentations in skeletally mature patients, and its severity is also very variable. The general practitioner should be familiar with NPS's clinical presentation spectrum, and the orthopedic surgeon with treatment options, in order to provide the most adequate treatment for each patient. Although there is no cure for NPS, several treatments have been useful in the symptomatic treatment of changes in the knees. We describe a rare clinical case of a 47-year-old female patient with NPS who underwent total knee arthroplasty for knee osteoarthritis.info:eu-repo/semantics/publishedVersio
REALMS study: real-world effectiveness and safety of fingolimod in patients with relapsing-remitting multiple sclerosis in Portugal
Get PDFackground: Fingolimod, an oral sphingosine 1-phosphate receptor modulator, is approved by EMA for relapsing-remitting multiple sclerosis (RRMS).
Objectives: To assess the effectiveness and safety of fingolimod in patients with RRMS in real-world clinical practice in Portugal.
Methods: Retrospective, multicentre, non-interventional study, reporting 3 years follow-up of data collected from October 2015 to July 2016. Sociodemographic data and previous treatments at baseline and data regarding disease evolution, including number of relapses, annualised relapse rates (ARR) and Expanded Disability Status Scale (EDSS), were collected.
Results: Two-hundred and seventy-five participants were enrolled in the REALMS study. Results showed that the main reason to switch to fingolimod was failure of previous treatment (56.7%) and only 3.6% were naïve patients. In the total population, there was a significant decrease in ARR of 64.6% in the first year of treatment, 79.7% in the second year and 82.3% in the third year, compared with baseline. More than 67.0% of patients had no relapses during the 3 years after switching to fingolimod. EDSS remained stable throughout the study.
Conclusions: Therapy with fingolimod showed a sustained effectiveness and safety over the 3 years, particularly on patients switched from first-line drugs (BRACE). No new safety issues were reported.This study was funded by Novartis Pharma, Portugal.info:eu-repo/semantics/publishedVersio
The Role of a Medical Intermediate Care Unit in the Management of Budd-Chiari Syndrome: Case Series
Get PDFBudd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). The therapeutic approach depends on disease severity and related etiology with patients with severe forms of presentation classically managed in intensive care units (ICUs). Here, we report a series of five BCS patients managed in a medical intermediate care unit (IntCU), with three of them presenting with acute liver injury. Progression to ALF was seen in three patients, two of whom died, with one being successfully submitted to liver transplantation. IntCUs allow a 24-h patient surveillance and a prompt management of BCS, with less economic impact when compared to ICUs. Mortality was related to the presence of associated comorbidities that limited therapeutic approach.info:eu-repo/semantics/publishedVersio
A Rare Cause of Abdominal Pain: Intestinal Angioedema
Get PDFAngioedema with evidence of mucosal swelling is a rare condition that can be caused by angiotensin-converting enzyme inhibitors. The authors describe a case of a 28-year-old woman medicated with lisinopril 2 months before, with abdominal pain associated with nausea, vomiting and a onetime watery stool. A small volume of ascites was found in the abdominal ultrasound and the parietal thickening of the middle ileum in the abdominal computed tomography (CT). The complementary study was negative. The symptoms stopped after drug withdrawal. The authors intend to warn to this diagnosis, which has non-specific symptoms and if not suspected, can lead to a great morbidity for patients. The authors also intend to identify various signs that can be clues for its correct diagnosis.info:eu-repo/semantics/publishedVersio
Immunoglobulin G4 Related-Disease: A Rare Presentation With Secondary Hypereosinophilic Syndrome and Eosinophilic Ascites
Get PDFImmunoglobulin G4 related-disease (IgG4-RD) is a multisystemic immune-mediated fibroinflammatory disease, with a strong predilection for salivary and lacrimal glands, pancreas, biliary tree, lungs, kidneys, aorta, and retroperitoneum. In the case of pancreatic involvement, it manifests as autoimmune pancreatitis. Patients with IgG4-RD usually have mild to moderate eosinophilia in the peripheral blood, however, they may present a secondary hypereosinophilic syndrome (HES). Although there are cases described with severe eosinophilia (> 5,000/µL), the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD considers peripheral eosinophilia above 3,000/µL as an exclusion criterion, although stating that assessment for the presence of exclusion criteria should be individualized depending on a patient's clinical scenario. Here, we describe the clinical case of a 30-year-old woman who presented with chronic diarrhea and abdominal pain. The diagnostic workup revealed eosinophilic ascites, severe peripheral blood eosinophilia (> 5,000/µL), IgG4 elevation (> 2 × upper normal limit), and also diffuse swelling in the body and pancreatic tail (computed tomography (CT) scan). There was a prompt response to corticosteroid therapy with clinical resolution and continued remission under therapy. The patient was diagnosed with IgG4-RD with secondary HES, explaining the gastrointestinal tract and peritoneum damage in the form of enterocolitis and ascites. Exclusion of alternative diagnosis was made.info:eu-repo/semantics/publishedVersio
PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemic
Get PDFBackground: During the COVID-19 pandemic, most of the health care systems suspended their non-urgent activities. This included the cancellation of consultations for patients with rare diseases, such as severe pulmonary hypertension (PH), resulting in potential medication shortage and loss of follow-up. Thus, the aim of the study was to evaluate PH patient health status evolution, access to health care and mental health experience during the early phase of the pandemic.
Methods: We conducted an online patient survey, available in 16 languages, between 22/05/2020 and 28/06/2020. The survey included questions corresponding to demographic, COVID-19 and PH related information.
Results: 1073 patients (or relatives, 27%) from 52 countries all over the world participated in the survey. Seventy-seven percent (77%) of responders reported a diagnosis of pulmonary arterial hypertension and 15% of chronic thromboembolic PH. The COVID-19 related events were few: only 1% of all responders reported a diagnosis of COVID-19. However, 8% of patients reported health deterioration possibly related to PH, and 4% hospitalization for PH. Besides, 11% of the patients reported difficulties to access their PH expert centre, and 3% interruption of treatment due to shortage of medication. Anxiety or depression was reported by 67% of the participants.
Conclusion: Although COVID-19 incidence in PH patients was low, PH related problems occurred frequently as the pandemic progressed, including difficulties to have access to specialized care. The importance of primary health care was emphasized. Further studies are needed to evaluate the long-term consequences of COVID-related PH care disruption.info:eu-repo/semantics/publishedVersio
Monitorizacão remota da insuficiência cardíaca com wearables - onde nos encontramos?
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Another Twist in the Tale: Intrafamilial Phenotypic Heterogeneity in ANO3‐Related Dystonia
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