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Purpose To report a case of symptomatic multiple retinal emboli during a carotid doppler ultrasonography (CDU) procedure. Material and methods Case report documented with clinical records, fundus photography (FP), optical coherence tomography angiography (OCT-A) of the retina and computed tomography angiography (CT-A) of the supra-aortic vessels. Results A 60-year-old male patient, presented to the emergency department with sudden vision loss in the left eye (LE) and pain in the left upper eyelid noticed during a CDU procedure. On the LE, the best corrected visual acuity (BCVA) was 20/200 and fundus observation revealed retinal arteriolar attenuation, retinal whitening with cherry point and multiple intra-arterial emboli in all arcades of the central retinal artery. CT-A of the supra-aortic vessels revealed an absence of filling throughout the cervical, petrous and cavernous segment of the left internal carotid artery, resuming only in the ophthalmic segment and an important stenosis of the left external carotid artery. He had no indication to be submitted to left carotid endarterectomy and began ocular massage and oral anticoagulant. 4 months later, ptosis was no longer present, BCVA improved to 20/20 with a residual scotoma and emboli disappeared on FP. Conclusion Embolic occlusion of retinal arteries may be an extremely rare but serious complication associated with CDU and patients and clinicians should be aware of it.

Nisa Silva,1,* Catarina Castro,1,* Filipa Caiado,1 Sofia Maia,1 Vasco Miranda,1,2 Ricardo Parreira,1 Pedro Menéres1,2 1Department of Ophthalmology, Centro Hospitalar Universitário do Porto, Porto, Portugal; 2Department of Ophthalmology, Instituto de Ciências Biomédicas Abel Salazar, University of Porto, Porto, Portugal*These authors contributed equally to this workCorrespondence: Nisa Silva, Department of Ophthalmology, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, 4099-001, Portugal, Tel +35 1918367661, Fax +35 1222077500, Email nisapinhosilva@gmail.comBackground: Understanding how strabismus impacts a child’s quality of life, as well as their families, should be an important key to guide treatment, not only from the ophthalmological point of view but also regarding psychological and social aspects, which are fundamental for a healthy and harmonious development.Methods: A cross-sectional study was performed to evaluate the functional vision and eye-related quality of life (ER-QOL) in a population of children with strabismus submitted or not to corrective surgery, using the recently developed Pediatric Eye Questionnaire (PedEyeQ) and to compare with age and gender-matched visually normal children. The PedEyeQ was applied to non-operated children with strabismus (n = 18), operated children with strabismus (n = 24), and visually normal children (n = 21). This instrument is composed of 3 components (Child, Proxy, and Parent) and has different versions according to the child’s age (0– 4, 5– 11, or 12– 17 years-old versions). Clinical data such as age, type of strabismus, angle of deviation, amblyopia, occlusion treatment, and surgical outcome were also recorded.Results: All PedEyeQ domain scores were significantly lower in children with strabismus compared with visually normal children, except the Child “functional vision” domain. Children with strabismus with successful corrective surgery had significantly lower scores in many domains of the Child, Proxy, and Parent components, compared with visually normal children.Conclusion: This study showed that strabismus has an important impact on affected children and their families, as assessed by PedEyeQ. Interestingly, children with prior successful corrective strabismus surgery had worse PedEyeQ scores compared to visually normal children. Educational programs and psychosocial rehabilitation interventions should be implemented in children with strabismus and their families.Keywords: strabismus, PedEyeQ, pediatric, parents, quality of life

Introduction: Accommodative esotropia (AET) is characterized by an esodeviation of the eyes due to uncorrected hyperopia, deficient fusional divergence, or high accommodative convergence. Decreasing hyperopia would reduce accommodative convergence and strabismus. We sought to review the existing evidence regarding the outcomes of refractive surgery in patients with AET. Methods: A four-database search (Pubmed, ISI Web of Science, Cochrane, and Scopus) was performed from inception to March 2021 using the following MeSH terms: (“Refractive Surgical Procedures” OR “Keratomileusis, Laser In Situ” OR “Photorefractive Keratectomy” OR “Lens Implantation, Intraocular”) AND (“Esotropia” OR “Accommodative Esotropia” OR “Refractive Esotropia” OR “Accommodative Strabismus”). No meta-analysis was performed due to studies’ heterogeneity. Results: Twenty-eight studies including 22 case series enrolling 378 patients and 6 case reports enrolling 8 patients were selected among 185 original abstracts. In the case series, a total of 378 patients (726 eyes) were recruited with an age range of 8–52 years. All studies reported mean follow-up periods of at least 12 months. Photorefractive keratectomy was performed in 7 studies, laser-assisted in situ keratomileusis in 9 studies, laser-assisted sub-epithelial keratectomy was reported in 1 study, and 3 studies implanted intraocular lenses, including iris-fixated and collamer. Considering the adult patients with a preoperative corrected esodeviation ≤10 prism diopters (PD) (n = 129), all but 5 (3.9%) presented orthophoria or ≤10PD after refractive surgery. All children but 4 (4.5%) ended up with an esodeviation ≤10PD after surgery with those exceptions being in the range of 11–15PD. Six case reports were included in this review, comprising a total of 8 patients (16 eyes) with an age range of 7–34 years and a follow-up range of 4–48 months. Six case reports were included in this review, comprising a total of 8 patients (16 eyes) with an age range of 7–34 years and a follow-up range of 4–48 months. Conclusion: Evidence produced so far points out that refractive surgery may be an alternative for spectacle correction for adults with AET ≤10PD. There is not enough evidence to recommend its use for patients under 18 years of age. The safety and predictability of these procedures for this purpose remains unclear as the selection criteria used for these patients are much different than the usual indications and there are no studies with long-term follow-up.

To develop the first Ophthalmology joint guidelines with Paediatric Rheumatology with recommendations on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis (JIA-U), endorsed by the Portuguese Society of Ophthalmology (SPO).A systematic literature review was conducted to include publications up to July 14th 2020, with no language restrictions, in order to include all the international position papers/guidelines concerning the medical management of JIA-U and randomised clinical trials assessing the efficacy and safety of medical treatment in this field. We searched through MEDLINE (PubMed), Scopus, Web of Science and Cochrane Library. The Delphi modified technique to generate consensus was used. Preliminary evidence statements were subject to an anonymous agreement assessment and discussion process using an online survey, followed by further discussion and update at a national meeting. A draft of the manuscript with all recommendations was then circulated among all participants and suggestions were incorporated. The final version was again circulated before publication.Twenty-six recommendations were developed focusing on the following topics: general management (3), screening and follow-up of uveitis (4), treatment (17) and health education in JIA-U among patients and families (2).These guidelines were designed to support the shared medical management of patients with JIA-U and emphasize the need for a multidisciplinary approach between Ophthalmology and Paediatric Rheumatology regarding the comprehensive care of JIA-U. We acknowledge that updating these recommendations will be warranted in the future, as more evidence becomes available.juvenile idiopathic arthritis, uveitis, biological treatment, conventional immunosuppressive treatment, multidisciplinary management, guidelines, consensus, review, Delphi Technique.

Purpose Amblyopia is a leading cause of preventable and treatable vision loss in the pediatric population. Instrument-based screening of amblyopia-risk factors is being widely adopted but the audit of its results is still lacking. We sought to review the existing evidence regarding the outcomes of photoscreening applied to children under the age of three years. Methods A three-database search (Pubmed, ISI Web of Science, and Scopus) was performed from inception to March 2021. A meta-analysis of proportions was conducted to summarize the referral rate, untestable rate and positive predictive value (PPV). Results Thirteen studies were selected among 705 original abstracts. The quantitative analysis included twelve studies enrolling 64,041 children. Of these, 13% (95%CI: 7–19%) were referred for further confirmation of the screening result. Astigmatism was the most common diagnosis both after screening and after ophthalmologic assessment of referred children. The pooled untestable rate and PPV were 8% (95%CI: 3–15%) and 56% (95%CI: 40–71%), respectively. Conclusion There is no global consensus on the optimal age, frequency or what magnitude of refractive error must be considered an amblyopia-risk factor. Optimization of referral criteria is therefore warranted.

Introduction Caring for a patient with end‐stage renal disease undergoing in‐centre haemodialysis can be a stressful experience, likely to involve significant burden. Within the context of the new coronavirus pandemic, these patients are highly vulnerable to infection by COVID‐19, which might increase the care demands and burden of family caregivers. Aim This study aimed to explore the subjective experiences of family caregivers of non‐COVID‐19 patients with end‐stage renal disease undergoing in‐centre haemodialysis during the COVID‐19 lockdown. Study design A qualitative study was performed with a purposive sample. Methods Semi‐structured telephone interviews were conducted with 19 family caregivers (50.7 ± 14 years old) of patients undergoing in‐centre haemodialysis in April 2020. Findings Four major themes were identified: (1) emotional distress; (2) changes in caregiving responsibilities; (3) educational and supportive needs; and (4) coping strategies to deal with the outbreak and with the lockdown. Discussion The findings suggest that family caregivers of patients undergoing in‐centre haemodialysis have to manage several additional care responsibilities due to COVID‐19 lockdown. The dialysis team should consider the development of educational and supportive interventions to meet family caregivers’ needs, mitigate emotional distress, fears and concerns, and prevent caregiver burden during the COVID‐19 pandemic.

PURPOSE To compare the retinal vasculature characteristics between eyes of patients with and without phacomatosis. METHODS Case-control observational study with retinal vasculature evaluation by optical coherence tomography and optical coherence tomography angiography of the macula and disk. RESULTS The study included 80 eyes. Neurofibromatosis Type 1 patients presented with a higher central macular thickness (P = 0.007), a lower optical disk nervous fiber layer (P = 0.006), a lower perimeter, area, and circularity of the foveal avascular zone (P < 0.05), a higher vascular density of macular avascular layer (AMVD) (P = 0.004), and a lower papillary vascular density of superficial capillary plexus (SPVD) (P = 0.048). Patients with tuberous sclerosis presented with an increase in central macular thickness (P = 0.024) and in vascular densities (P < 0.05) [except for macular vascular density of deep capillary plexus (PMVD), AMVD, and SPVD]. Patients with Sturge-Weber syndrome showed a decrease in optical disk nervous fiber layer (P < 0.001), subfoveal choroid thickness (P = 0.011), macular vascular density of superficial capillary plexus (SMVD) (P = 0.036), and SPVD (P < 0.001). CONCLUSION Phacomatosis patients showed statistically significant differences of retinal vasculature characteristics, compared to eyes without pathology. Further studies are needed to determine when and if these parameters change with the course of the disease and if they can be used as biomarkers for disease severity or progression.

This pilot study aimed to assess the feasibility, acceptability, and preliminary effects of a family-based psychoeducational intervention for patients undergoing hemodialysis (HD) and their family members. This was a single-group (six dyads), six-week, pre–post pilot study, delivered in a multifamily group format. Feasibility was based on screening, eligibility, content, retention, completion, and intervention adherence rates. Acceptability was assessed at post-intervention through a focus group interview. Self-reported anxiety and depression and patients’ inter-dialytic weight gain (IDWG) were also measured. The screening (93.5%), retention (85.7%), and completion (100%) rates were satisfactory, whereas eligibility (22.8%), consent (18.4%), and intervention adherence (range: 16.7–50%) rates were the most critical. Findings showed that participants appreciated the intervention and perceived several educational and emotional benefits. The results from the Wilcoxon Signed-Rank Test showed that a significant decrease in anxiety symptoms (p = 0.025, r = 0.646) was found, which was followed by medium to large within-group effect sizes for changes in depression symptoms (p = 0.261, r = 0.325) and patients’ IDWG (p = 0.248, r = 0.472), respectively. Overall, the results indicated that this family-based psychoeducational intervention is likely to be feasible, acceptable, and effective for patients undergoing HD and their family caregivers, nonetheless, further considerations are needed on how to make the intervention more practical and easily implemented in routine dialysis care before proceeding to large-scale trials.

Background and Aims Cardiovascular disease (CVD) is the major cause of mortality and morbidity in chronic kidney disease (CKD), especially in end-stage renal disease (ESRD) patients. Left ventricular hypertrophy (LVH) is a common cardiovascular complication in CKD. Growth differentiation factor (GDF)-15 increases in tissue injury and inflammatory states associated with cardiometabolic risk. GDF-15 and N-terminal pro B-type natriuretic peptide (NT-proBNP) are both synthesized by cardiomyocytes and may be associated with cardiorenal dysfunction. Our aim was to study the association of GDF-15 with LVH in ESRD patients on dialysis. Method This study included 196 ESRD patients on dialysis (hemodiafiltration and high-flux hemodialysis). Left ventricular mass (LVM) was evaluated through echocardiographic studies, corrected for body surface area and the values are presented as LVM index (LVMI). LVH was defined by a value of LVMI > 115 g/m2 in men and > 95 g/m2 in women. Patients were divided into two groups - LVH (n=131) and non-LVH (n=65). LVMI, clinical and analytical variables (age, body mass index, dialysis vintage, dialysis adequacy, GDF-15, NT-proBNP and pentraxin (PTX) 3 were evaluated. Results ESRD patients with LVH presented significantly higher levels of NT-proBNP and GDF-15, and a trend towards higher PTX3 values. In LVH patients, GDF-15 correlated positively and significantly with NT-proBNP and PTX3; LVMI correlated positively and significantly with pro-BNP and PTX3 levels; pro-BNP correlated significantly and positively with PTX3. Conclusion Our data show that in ESRD patients on dialysis with LVH, GDF-15 is raised and shows a strong association with NT-proBNP, PTX3 and LVMI. Further studies are needed to clarify if the rise in GDF-15 is a cause or a consequence of LVH development. Acknowledgments This work was supported by Applied Molecular Biosciences Unit-UCIBIO, financed by national funds from FCT/MCTES (UIDB/04378/2020), by North Portugal Regional Coordination and Development Commission (CCDR-N)/NORTE2020/Portugal 2020 (Norte-01-0145-FEDER-000024) and by REQUIMTE-Rede de Química e Tecnologia-Associação in the form of a researcher (S. Rocha) – project Dial4Life co-financed by FCT/MCTES (PTDC/MEC-CAR/31322/2017) and FEDER/COMPETE 2020 (POCI-01-0145-FEDER-031322).

Background and Aims Chronic inflammation plays an important role in the progression of chronic kidney disease (CKD) to end-stage renal disease (ESRD). The single nucleotide polymorphism (SNP) in the promoter region (-174G/C) of interleukin-6 (IL6) gene regulates the levels of this cytokine, which have been associated with a poor outcome in several pathologies. Our aims were to determine, according to the genotype distribution of this polymorphism, the association between the inflammatory mediators, high sensitivity C-reactive protein (hsCRP), IL6, and pentraxin 3 (PTX3), shown to be increased in ESRD, and to estimate the risk for all-cause mortality over a period of one year. Method 289 ESRD patients on hemodialysis (high-flux hemodialysis and hemodiafiltration) were included in this study. Real-Time PCR TaqMan SNP genotyping assays were used to assess allelic frequencies of IL6 (rs1800795). We evaluated the circulating levels of PTX3, hsCRP and IL6 using commercially available kits. Deaths occurring along 1-year follow-up period were recorded, and the all-cause mortality hazard ratio (HR), according to IL6 polymorphisms in this patient cohort were determined by Cox regression analysis. A p < 0.05 value was considered statistically significant. Results In all IL6 (-174G>C) genotypes, hsCRP was positively and significantly correlated with IL6. For hsCRP and PTX3, a positive correlation with significance was only found for the GG genotype. All genotypes showed positive correlations between IL6 and PTX3 circulating levels, although only the GG genotype achieved a significant value. The Cox regression survival analysis for all-cause mortality in ESRD patients, using as reference the heterozygous patients for IL6 polymorphism, showed that CC patients presented a significant higher mortality risk, with a HR of 3.275 [1.165 to 9.204]. Moreover, the median survival time of CC patients (100 [54 - 138] days) was lower than that presented by the GG genotype patients (211 [83 - 290] days, p < 0.05 vs. CC) and by the heterozygous patients (291 [72 - 332] days, p = 0.157 vs. CC). Conclusion We observed different correlation profiles between inflammatory biomarkers within each IL6 (-174G>C) genotype. The association of the CC genotype of the IL6 polymorphism with a poorer outcome and shorter survival time for ESRD patients was also observed. However, further studies are required and must consider the underlying individual genetic background, since the inflammatory state appears to be influenced by IL6 polymorphisms, which, in turn, might be determinant for disease progression and outcome. Acknowledgments This work was supported by Applied Molecular Biosciences Unit-UCIBIO, financed by national funds from FCT/MCTES (UIDB/04378/2020), by North Portugal Regional Coordination and Development Commission (CCDR-N)/NORTE2020/Portugal 2020 (Norte-01-0145-FEDER-000024) and by REQUIMTE-Rede de Química e Tecnologia-Associação in the form of a researcher (S. Rocha) – project Dial4Life co-financed by FCT/MCTES (PTDC/MEC-CAR/31322/2017) and FEDER/COMPETE 2020 (POCI-01-0145-FEDER-031322).

Cardiovascular disease (CVD) events are the main causes of death in end-stage renal disease (ESRD) patients on dialysis. The number and severity of CVD events remain inappropriate and difficult to explain by considering only the classic CVD risk factors. Our aim was to clarify the changes and the relationship of lipoprotein subfractions with other CVD risk factors, namely, body mass index (BMI) and adipokines, inflammation and low-density lipoprotein (LDL) oxidation, and the burden of the most prevalent comorbidities, diabetes mellitus (DM) and hypertension (HT). We studied 194 ESRD patients on dialysis and 22 controls; lipid profile, including lipoprotein subpopulations and oxidized LDL (oxLDL), C-reactive protein (CRP), adiponectin, leptin, and paraoxonase 1 activity were evaluated. Compared to controls, patients presented significantly lower levels of cholesterol, high-density lipoprotein cholesterol (HDLc), LDLc, oxLDL, and intermediate and small HDL and higher triglycerides, CRP, adiponectin, large HDL, very-low-density lipoprotein (VLDL), and intermediate-density lipoprotein- (IDL) B. Adiponectin levels correlated positively with large HDL and negatively with intermediate and small HDL, oxLDL/LDLc, and BMI; patients with DM (n=17) and with DM+HT (n=70), as compared to patients without DM or HT (n=69) or only with HT (n=38), presented significantly higher oxLDL, oxLDL/LDLc, and leptin and lower adiponectin. Obese patients (n=45), as compared to normoponderal patients (n=81), showed lower HDLc, adiponectin, and large HDL and significantly higher leptin, VLDL, and intermediate and small HDL. In ESRD, the higher adiponectin seems to favor atheroprotective HDL modifications and protect LDL particles from oxidative atherogenic changes. However, in diabetic and obese patients, adiponectin presents the lowest values, oxLDL/LDLc present the highest ones, and the HDL profile is the more atherogenic. Our data suggest that the coexistence of DM and adiposity in ESRD patients on dialysis contributes to a higher CVD risk, as showed by their lipid and adipokine profiles.

Background: Soluble transferrin receptor (sTfR) is a biomarker of erythropoiesis, which is often impaired in dialysis patients. The aim of our study was to evaluate sTfR levels in chronically dialyzed patients and assess potential determinants of its levels. Methods: We performed a cross-sectional study by evaluating 246 end-stage renal disease patients undergoing dialysis and 32 healthy controls. Circulating levels of interleukin (IL)-6, C-reactive protein (CRP), tumor necrosis factor (TNF)-α, hepcidin, sTfR, growth differentiation factor 15 (GDF15), and traditional iron metabolism markers were measured, as well as hemogram parameters. Clinical data was obtained from all patients. Results: Compared to controls, patients presented similar values of sTfR, reticulocytes and reticulocyte production index (RPI), and significantly higher levels of IL-6, CRP, ferritin, hepcidin, TNF-α, and GDF15. Iron, transferrin, hemoglobin levels, erythrocyte count, mean cell hemoglobin (MCH), and mean cell hemoglobin concentration (MCHC) values were significantly lower in dialysis group. Within patients, sTfR values were higher in diabetic patients and were positively and significantly correlated with reticulocytes and erythrocytes, RPI, and therapeutic doses of erythropoiesis stimulating agents (ESA) and intravenous iron; and inversely and significantly correlated with circulating iron, ferritin, transferrin saturation, hepcidin, MCH, and MCHC. In multiple linear regression analysis, ESA dose, RPI, serum iron, diabetes, and hepcidin levels were independently associated with sTfR levels in dialysis patients and, thus, with erythropoiesis. Conclusion: Our data suggest that, besides RPI and ESA dose, diabetes and hepcidin are closely related to erythropoiesis in dialysis patients. The influence of diabetes on sTfR levels deserves further investigation.

Chronic inflammation plays an important role in the progression and outcome of chronic kidney disease (CKD). The circulating levels of the inflammatory biomarkers interleukin 6 (IL6) and pentraxin 3 (PTX3) are enhanced in CKD patients, and are associated with the progression of the disease and with higher risk for cardiovascular events, the major cause of death in CKD patients. Our aim was to study how specific polymorphisms of IL6 and PTX3 encoding genes affect the inflammatory response and outcome of end-stage renal disease (ESRD) patients on dialysis. Methodology included the analysis of two single nucleotide polymorphisms (SNP), namely the IL6 (rs1800795) polymorphism in the promoter region (-174G > C), and the PTX3 (rs2305619) polymorphism in the intron 1 (+ 281A > G), which were analyzed in ESRD patients on dialysis and in a group of heathy individuals. The allelic frequencies, genotype distribution and their association with circulating levels of the inflammatory markers C-reactive protein (CRP), IL6, growth differentiation factor 15 (GDF15) and PTX3, were determined in ESRD patients. Events of death were recorded along one year, to assess the association of the studied SNPs with all-cause mortality and the inflammatory biomarkers, in ESRD patients. Results showed that the allelic frequencies and genotype distribution for IL6 and PTX3 SNPs in the control group and ESRD patients were similar and in agreement with other European reports. For the IL6 polymorphism, we found a trend towards higher levels of high-sensitivity (hs) CRP, IL6 and PTX3 in the homozygous genotypes; the CC genotype also showed the highest levels of GDF15. The mortality rate after the 1-year follow-up was 10.4%. The CC genotype (IL6 SNP) was associated to a higher risk of mortality and deceased patients carrying this genotype also showed the highest levels of hsCRP. Regarding the studied PTX3 SNP, the AA genotype was linked to an enhanced inflammatory response, showing the highest values of hsCRP and IL6. Nevertheless, this genotype had no significant impact on the mortality rate. In conclusion, both studied SNPs seem to modulate the inflammatory response in ESRD and may, therefore, be determinant on disease progression and patients’ outcome. Our data also highlights the importance of research on genetic variants that, although less frequent, may have significant biological value.

Background and Aims Chronic inflammation plays an important role in progression and outcome of chronic kidney disease (CKD) patients. The interleukin-6 (IL-6) polymorphism, rs1800795, is a single nucleotide polymorphism (SNP) in the promoter region (-174G/C) that regulates gene transcription and affects the levels of this cytokine. Genetic variants in the promotor region have been associated with a poor outcome in several pathologies, namely in coronary artery disease. Pentraxin 3 (PTX3) is an inflammatory protein, produced locally by different cell types (e.g. mononuclear phagocytes, dendritic cells, fibroblasts and endothelial cells). PTX3 polymorphism, rs2305619, is a SNP (+281A>G) in the intron 1 of PTX3 gene. PTX3 polymorphisms have been described as risk factor for development of coronary artery disease and for an impairment of kidney function. Our aim was to determine the allelic frequencies of IL6 and PTX3 in end-stage renal disease (ESRD) patients and controls, as well as their relationship with the inflammatory response. Method We studied 289 ESRD patients on hemodialysis (high-flux hemodialysis or hemodiafiltration) and 22 healthy individuals, matched for gender, body mass index, and, as far as possible, for age. Real-Time PCR TaqMan SNP genotyping assays were used to assess allelic frequencies of IL6 (rs1800795) and PTX3 (rs2305619). We evaluated the circulating levels of PTX3, growth differentiation factor (GDF)-15, high-sensitivity C-reactive protein (hsCRP), IL-6, tumor necrosis factor-alpha (TNF-α), soluble TNF receptor 2 (sTNFR2), hepcidin and transferrin, using commercially available kits. Deaths occurring along 1-year follow-up period were recorded and mortality rates were evaluated for homozygous and heterozygous alleles. Results Compared to controls, all inflammatory biomarkers were significantly higher in ESRD patients. Allelic frequencies in ESRD patients and controls were similar for both SNPs, IL-6 -174G/C and PTX3 +281A>G, considering the homozygous and the heterozygous alleles. For the IL-6 -174G/C, the CC patients, compared to GG and heterozygous patients, showed significantly higher GDF-15 and CRP concentrations and significantly lower values of transferrin; moreover, we found that CC patients presented a trend towards higher mortality rate (21%, 10% and 7% for CC, GG and GC, respectively; P=0.072). For PTX3 +281A>G polymorphism, the AA patients, compared to GG and heterozygous patients, presented higher hsCRP; no significant differences were found in mortality rate between different allele carrier groups (P=0.703). Conclusion Although no differences were found in the allelic frequencies between controls and ERSD patients, the CC patients for IL6 polymorphism (rs1800795) and AA patients for PTX3 polymorphism (rs2305619), showed an enhanced inflammatory response; the association of the IL6 polymorphism with a higher mortality needs further studies.

Background and Aims In patients with end-stage renal disease (ESRD) on hemodialysis, the preservation of residual kidney function may result in a diversity of benefits in terms of survival and quality of life. The control of fluid and electrolyte homeostasis may play an important role in this setting. Elevated predialysis serum potassium is a common electrolyte disturbance that may worsen patient’s outcomes. Our aim was to study the impact of furosemide therapy in predialysis serum potassium levels, indicators of estimated residual renal function, and inflammatory markers, in ESRD patients under hemodialysis; moreover, we aimed to study the impact of furosemide-associated changes on mortality rate. Method A cross-sectional study was carried out on 289 adult patients on chronic dialysis therapy (hemodiafiltration and high flux hemodialysis). Patients were divided in 2 groups: the diuretic group (DG, n=116; 120.0 (IQR: 80-160) mg/daily median furosemide dose) and the non-diuretic group (NDG, n = 173), in which patients did not use furosemide. A large set of data was analyzed, encompassing hematological data, serum electrolyte parameters, inflammatory markers, dialysis adequacy, and biomarkers of residual kidney function. A 2-year follow up study was also performed by registering events of death (all-cause mortality). Results The DG patients, compared with NDG patients, presented: significantly lower predialysis serum potassium; more favorable blood biomarkers of kidney function - lower β-trace protein, cystatin C, creatinine and urea; greater residual glomerular filtration rate derived from equations with cystatin C, creatinine and creatinine–cystatin C; lower inflammation (significantly lower levels of high-sensitivity C-reactive protein); intradialytic ultrafiltration volume (L) was similar for the two groups. Mortality was significantly lower for DG patients, compared with NDG (13.6% versus 24.7%; P=0.029). Conclusion In ESRD patients under chronic dialysis, we found a significant association between current diuretic therapy and lower predialysis serum potassium levels, more favorable biomarkers of kidney function and a decreased inflammatory response that seem to contribute to a higher survival rate. Acknowledgments: The work was supported by UIDB/04378/2020 with funding from FCT/MCTES through national funds, by North Portugal Regional Coordination and Development Commission (CCDR-N)/NORTE2020/Portugal 2020 (Norte-01-0145-FEDER-000024) and by REQUIMTE-Rede de Química e Tecnologia-Associação in the form of a researcher (S. Rocha) – project Dial4Life co-financed by FCT/MCTES (PTDC/MEC-CAR/31322/2017) and FEDER/COMPETE 2020 (POCI-01-0145-FEDER-031322).

Background and Aims Cardiovascular (CV) disease events are the major cause of death in end-stage renal disease (ESRD) patients on dialysis. Left ventricular hypertrophy, myocardial stress and disturbances in nutritional status are common findings in ESRD patients on dialysis. The adipokines, adiponectin and leptin, are known as important mediators of cardiometabolic risk in obesity, although their role in ESRD patients is poorly clarified. N-terminal pro B-type natriuretic peptide (NT-proBNP) is synthesized within cardiac myocytes, in response to cardiac wall stress. Our aim was to study the association of left ventricular hypertrophy with adipokines (adiponectin and leptin), myocardial stress (ProBNP) and nutritional status (BMI and albumin) in ESRD patients on dialysis. Method This study included 196 ESRD patients on dialysis (hemodiafiltration and high-flux hemodialysis). LVH was defined by a value of LVMI > 115 g/m2 in men and 95 g/m2 in women. Patients were divided into two groups - LVH (n=131) and non-LVH (n=65). Left ventricular mass (LVM) was evaluated through echocardiographic studies, corrected for body surface area and the values are presented as LVM index (LVMI). LVMI, clinical and analytical variables (age, body mass index, dialysis vintage, dialysis adequacy, albumin, adiponectin, leptin, NT-proBNP and hemoglobin concentration) were evaluated. Results The prevalence of LVH was 66.8%; LVH patients presented significantly higher levels for NT-proBNP, and significantly lower levels for hemoglobin, when compared with non-LVH patients; a trend towards (P=0.052) lower leptin values were observed. No differences were observed between groups, for age, BMI, dialysis vintage, URR, Kt/V, eKt/V, ultrafiltration volume, albumin and adiponectin. In LVH patients, we found that LVMI presented significant positive correlations with NT-proBNP and with adiponectin; and, significant negative correlations with markers of dialysis adequacy (URR and eKt/V) and leptin. Conclusion Our data suggest that a higher LVMI in ESRD patients is associated with higher myocardial stress, with lower leptin and higher adiponectin levels, showing no association of these adipokines with increased cardiovascular risk and with worsening of left ventricular hypertrophy. Further studies are needed to understand the roles of these adipokines, considering the controversial association with left ventricular hypertrophy, a cardiovascular risk condition. Acknowledgments: The work was supported by UIDB/04378/2020 with funding from FCT/MCTES through national funds, by North Portugal Regional Coordination and Development Commission (CCDR-N)/NORTE2020/Portugal 2020 (Norte-01-0145-FEDER-000024) and by REQUIMTE-Rede de Química e Tecnologia-Associação in the form of a researcher (S. Rocha) – project Dial4Life co-financed by FCT/MCTES (PTDC/MEC-CAR/31322/2017) and FEDER/COMPETE 2020 (POCI-01-0145-FEDER-031322).

Background and Aims Dyslipidemia is common in chronic kidney disease (CKD) and cardiovascular disease (CVD)-related events are the main causes of death in end-stage renal disease (ESRD) patients on dialysis. Concerning lipid management, clinical practice emphasized treatment escalation to achieve specific low-density lipoprotein cholesterol (LDLc) targets, which implies repeated LDLc evaluations, and enhancement of statin doses or combination of lipid-lowering therapies. However, the LDLc target is not consensual, with some entities suggesting 100 mg/dl and others a more conservative level. It has been hypothesized that lipoprotein’s quality (size, composition and functionality) may be more important than their total circulating levels, as CVD risk factor. Our aim was to evaluate and compare, in ESRD patients on dialysis and under statins treatment, the levels of lipoprotein fractions and subfractions and inflammatory markers, between patients who achieved LDLc levels < 100 mg/dl and those who did not achieve that target. Method We studied 110 ESRD patients on dialysis (high-flux hemodialysis or hemodiafiltration) and under statin therapy; 87 presented a LDLc < 100 mg/dl (group 1) and 23 a value > 100 mg/dl (group 2); levels of high-sensitivity C-reactive protein (hsCRP), interleukin(IL)-6, lipid profile including lipoprotein fractions/subfractions, and oxidized LDL (oxLDL) were evaluated. Results Group 1, as compared to group 2, presented lower values of total cholesterol (TC), triglycerides, oxLDL, TC/high-density lipoprotein cholesterol (HDLc) and LDLc/HDLc ratios. Concerning lipoprotein fractions/subfractions, group 1 presented significantly higher larger and intermediate LDL, and a trend towards lower small LDL (P=0.063), higher large HDL (P=0.069) and lower small HDL (P=0.080); no significant alterations were found for very-low-density lipoprotein and intermediate-density lipoprotein. Regarding inflammatory markers, no significant differences were observed between the 2 groups. Conclusion Patients who achieved the LDLc < 100 mg/dl target presented a better non-conventional lipid profile, including lower oxLDL levels and an increase in larger (less atherogenic) LDL subfractions. According to our data, a lower LDLc level associates with a better lipid profile; the benefits of this improvement on HDL fractions and CVD-related events in ESRD patients on dialysis needs to be better clarified.

Mapping accurately vegetation surfaces in space and time in the ice-free areas of Antarctica can provide important information to quantitatively describe the evolution of their ecosystems. Spaceborne remote sensing is the adequate way to map and evaluate multitemporal changes on the Antarctic vegetation at large but its nature of occurrence, in relatively small and sparse patches, makes the identification very challenging. The inclusion of an intermediate scale of observation between ground and satellite scales, provided by Unmanned Aerial Vehicles (UAV) imagery, is of great help not only for their effective classification, but also for discriminating their main communities (lichens and mosses). Thus, this paper quantifies accurately recent changes of the vegetated areas in Fildes Peninsula (King George Island, Antarctica) through a novel methodology based on the integration of multiplatform data (satellite and UAV). It consists of multiscale imagery (spatial resolution of 2 m and 2 cm) from the same period to create a robust classifier that, after intensive calibration, is adequately used in other dates, where field reference data is scarce or not available at all. The methodology is developed and tested with UAV and satellite data from 2017 showing overall accuracies of 96% and kappa equal to 0.94 with a SVM classifier. These high performances allow the extrapolation to a pair of previous dates, 2006 and 2013, when atmospherically clear very high-resolution satellite imagery are available. The classification allows verifying a loss of the total area of vegetation of 4.5% during the 11-year time period under analysis, which corresponds to a 10.3% reduction for Usnea sp. and 9.8% for moss formations. Nevertheless, the breakdown analysis by time period shows a distinct behaviour for each vegetation type which are evaluated and discussed, namely for Usnea sp. whose decline is likely to be related to changing snow conditions.

Vascular bypass is a surgical procedure widely used to treat peripheral vascular disease. The intraoperative anesthetic technique and the most appropriate postoperative analgesia for these high-risk patients remain controversial. We present the case of a patient undergoing femoropopliteal-distal bypass in our service, presenting with relevant comorbidities to the choice of anesthetic technique. This patient had several determining factors of difficult airway, especially thoracic kyphoscoliosis, which prevented him from being properly positioned for airway management, and chronic lung disease. This patient was also taken antiplatelet drugs, which is a contraindication for neuraxial block. So, we chose the anesthetic technique of peripheral nerve block, specifically the blockade of femoral and sciatic nerves. Resumo: A cirurgia para bypass vascular é um procedimento amplamente usado para o tratamento da insuficiência vascular periférica. A técnica anestésica para o intraoperatório e para analgesia pós-operatória mais apropriada para esses pacientes de alto risco ainda permanece controversa. Apresentaremos o caso de um paciente submetido a bypass femoropoplíteo distal no nosso serviço, que apresentava comorbidades relevantes para a escolha da técnica anestésica. Esse paciente apresentava fatores determinantes de via aérea difícil, principalmente cifoescoliose da coluna torácica, que o impedia de ser posicionado adequadamente para o manejo da via aérea, além de ser portador de patologia pulmonar crônica. Também fazia uso de antiplaquetários que contraindicavam o bloqueio de neuroeixo. Por isso, optou-se como técnica anestésica pelo bloqueio de nervos periféricos, especificamente o bloqueio dos nervos femoral e isquiático. Keywords: Femoral nerve block, Sciatic nerve block, Vascular surgery, Palavras-chave: Bloqueio de nervo femoral, Bloqueio de nervo isquiático, Cirurgia vascular

Background 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.

The loss of the red blood cells (RBCs) deformability is related with many human diseases, such as malaria, hereditary spherocytosis, sickle cell disease, or renal diseases. Hence, during the last years, a variety of technologies have been proposed to gain insights into the factors affecting the RBCs deformability and their possible direct association with several blood pathologies. In this work, we present a simple microfluidic tool that provides the assessment of motions and deformations of RBCs of end-stage kidney disease (ESKD) patients, under a well-controlled microenvironment. All of the flow studies were performed within a hyperbolic converging microchannels where single-cell deformability was assessed under a controlled homogeneous extensional flow field. By using a passive microfluidic device, RBCs passing through a hyperbolic-shaped contraction were measured by a high-speed video microscopy system, and the velocities and deformability ratios (DR) calculated. Blood samples from 27 individuals, including seven healthy controls and 20 having ESKD with or without diabetes, were analysed. The obtained data indicates that the proposed device is able to detect changes in DR of the RBCs, allowing for distinguishing the samples from the healthy controls and the patients. Overall, the deformability of ESKD patients with and without diabetes type II is lower in comparison with the RBCs from the healthy controls, with this difference being more evident for the group of ESKD patients with diabetes. RBCs from ESKD patients without diabetes elongate on average 8% less, within the hyperbolic contraction, as compared to healthy controls; whereas, RBCs from ESKD patients with diabetes elongate on average 14% less than the healthy controls. The proposed strategy can be easily transformed into a simple and inexpensive diagnostic microfluidic system to assess blood cells deformability due to the huge progress in image processing and high-speed microvisualization technology., Research supported by FCT with the reference projects POCI-01-0145-FEDER-016861 (PTDC/QEQFTT/4287/2014), NORTE-01-0145-FEDER-029394 (PTDC/EMD-EMD/29394/2017), NORTE-01-0145-FEDER030171 (PTDC/EME-SIS/30171/2017), UID/EMS/04077/2019, UID/EEA/04436/2019, UID/EMS/00532/2019, PTDC/SAU-ENB/116929/2010, by FEDER funds through the COMPETE 2020, NORTE2020, PORTUGAL2020— Programa Operacional Competitividade e Internacionalização (POCI) with the reference project POCI-01-0145-FEDER-006941 and by the NORTE-01-0145-FEDER-028178 (PTDC/EEI-EEE/28178/2017) project, funded 85% from Programa Operacional Regional do Norte and 15% from FCT. This study was also supported by FCT/MEC through national funds and cofinanced by FEDER, under the Partnership Agreement PT2020 from UCIBIO (UID/MULTI/04378/2013-POCI/01/0145/FEDER/007728), and North Portugal Regional Coordination and Development Commission (CCDR-N)/NORTE2020/Portugal 2020 (Norte-01-0145-FEDER-000024).

Successful dialysis in end-stage renal disease (ESRD) largely depends on the patients' ability to adhere to several clinical requirements and life-style changes. Social support has been consistently linked to better health outcomes in a number of chronic diseases. The current study presents a systematic review of the literature on the relationship between social support and treatment adherence in ESRD. The search was performed on Web of Science, PsycInfo, ScienceDirect, and Scopus from January 19 to May 15, 2019. Two hundred and twenty-four records were identified. After quality assessment, 17 studies were included for qualitative synthesis. This review comprised a total of 2362 patients. Most patients were on hemodialysis (97.5%) for an average of 58.2 months. Results suggested an overall pattern of mixed findings regarding the association between social support and treatment adherence. Adherence to fluid restrictions was the type of adherence with more significant associations with social support (63%). Mixed results were found for adherence to dialysis sessions (50%) and to medication (50%). Associations between adherence to diet restrictions and social support were found in 44% of the included studies. No significant associations were found between social support and adherence to follow-up consults. All of the included studies combining several types of adherence into an overall score found significant associations with social support. Findings suggested that it might be particularly beneficial to focus future research and clinical efforts toward social support from family, significant others, and health professionals, to improve patient's with ESRD treatment adherence.