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O artigo incide sobre a reforma legislativa desencadeada pela apresentação do Pacto Ecológico Europeu, no início do mandato da Comissão. Para o efeito, começa por analisar o roteiro normativo contido no Objetivo 55 e o primeiro passo para a sua concretização, com a adoção da chamada lei europeia do clima. De seguida, o artigo versa sobre elementos nucleares da ambiciosa reforma em vista da transição climática da União, a qual pretende alcançar um equilíbrio entre a redução de emissões de gases com efeito de estufa e o aumento da remoção natural de carbono através de sumidouros: por um lado, a revisão do sistema de comércio europeu de licenças de emissão e a reforma do regime de tributação da energia; por outro lado, a promoção da qualidade do uso do solo e o aumento da área florestal, bem como a restauração da natureza, por via da recuperação dos ecossistemas e da melhoria da biodiversidade.

Is the neuroanatomy of the language structural connectome modulated by the life-long experience of speaking a specific language? The current study compared the brain white matter connections of the language and speech production network in a large cohort of 94 native speakers of two very different languages: an Indo-European morphosyntactically complex language (German) and a Semitic root-based language (Arabic). Using high-resolution diffusion-weighted MRI and tractography-based network statistics of the language connectome, we demonstrated that German native speakers exhibited stronger connectivity in an intra-hemispheric frontal to parietal/temporal dorsal language network, known to be associated with complex syntax processing. In comparison, Arabic native speakers showed stronger connectivity in the connections between semantic language regions, including the left temporo-parietal network, and stronger inter-hemispheric connections via the posterior corpus callosum connecting bilateral superior temporal and inferior parietal regions. The current study suggests that the structural language connectome develops and is modulated by environmental factors such as the characteristic processing demands of the native language.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder of unknown etiology. Lupus nephritis (LN) is one of the most severe clinical manifestations observed in patients with SLE; it is more frequent and more severe in children than in adults. The aim of our study was to assess the predictive factors of poor outcomes in Tunisian children with LN. This was a multicenter retrospective observational study on 40 pediatric patients with biopsy-proven LN from five nephrology departments in Tunisia. The patients were 12.33 ± 3.3 years of age at the time of their kidney biopsy. Eleven patients developed end-stage renal disease (ESRD) (27.5%), and seven patients died. Overall, 18 (45%) patients reached our composite endpoint (ESRD or death). An age at diagnosis of more than 14 years, elevated serum creatinine at the time of the kidney biopsy, the existence of wire loops, thromboembolic complications as well as infectious complications are the most important clinical features associated with an increased risk of ESRD. Predictive factors of death were a baseline creatinine level of more than 2.26 mg/dL, a high proteinuria at baseline, fibrous crescents determined by renal biopsy, thromboembolic complications, infectious compli-cations, and ESRD. In summary, our results suggest that early and appropriate management is the best guarantee of a good renal outcome in children with LN.

Abstract Multiple myeloma (MM) results from malignant plasma cell disorder. It represents approximately 10% of hematological malignancies and it is typically diagnosed in the elderly with a median age of 70 years and has a steep increase in incidence with advancing age. N Engl J Med. 2004, 1860; Clin Interv Aging. 2020, 619. The incidence of MM has been increasing over time, mostly due to population aging. Mayo Clin Proc. 2010, 225 However, certain MMs are diagnosed at young age even under 40 years old (2%). Leuk Lymphoma. 1998, 493; Blood. 2010, 5501. We report a case of a MM in a thirty‐four‐year‐old woman whose circumstance of discovery was acute kidney failure.

The article examines the withdrawal agreement between the UK and the EU following the Brexit referendum including an analysis of the role of the main political actors. Beginning with the negotiation strategies of both the UK and the EU, it then concentrates on the political tensions between the British Government and Parliament with respect to the latter’s approval of the agreement – as well as EU behavior throughout that dispute. The article also focuses on how courts were asked to settle disputes between the main branches of power on the issue of Brexit and proposes a critical view on the role of the stakeholders of the agreement, as well as a reflection on the collateral effects of this process.

Abstract Background Karyomegalic interstitial nephritis (KIN) is a rare disease entity first described by Burry in 1974. The term KIN was introduced by Mihatsch et al. in 1979. KIN is characterized by chronic tubulointerstitial nephritis associated with enlarged tubular epithelial cell nuclei, which leads to a progressive decline of renal function. The prevalence of this disease is less than 1% of all biopsies, and its pathogenesis is unclear. KIN results from mutations in FAN1 (FANCD2/FANCI-Associated Nuclease 1), a gene involved in the DNA damage response pathway, particularly in the kidney. In this study, we report two Tunisian consanguineous families with KIN caused by mutations in the FAN1 gene. Methods Direct sequencing of the coding regions and flanking intronic sequences of the FAN1 gene was performed in three affected members. Three prediction programs (Polyphen-2 software, SIFT, and MutationTaster) were used to predict the functional effect of the detected variations. Results Two causative frameshift variants in the FAN1 gene were identified in each family: The previously described frameshift mutation c.2616delA (p.Asp873ThrfsTer17) and a novel mutation c.2603delT (p.Leu868ArgfsTer22) classified as "pathogenic" according to the American College of Medical Genetics and Genomics (ACMG) guidelines. Conclusion To our best knowledge, this is the first Tunisian study involving familial cases of KIN with mutations in the FAN1 gene. We hypothesize that these findings can expand the mutational spectrum of KIN and provide valuable information on the genetic cause of KIN.

Abstract Unexplained deep vein thrombosis may justify screening for antineutrophil cytoplasmic antibody‐associated vasculitis as it can be an unusual presentation of this disease.

The World Health Organization estimates that 3% of the general population is infected with this virus. Hepatitis C remains the main viral infection in dialysis patients, and the severity of this infection is the risk of developing cirrhosis or hepatocarcinoma. We aim to determine the prevalence of hepatitis C in dialysis patients, to calculate the rate of hepatitis C virus (HCV) seroconversion, and to identify the risk factors for seroconversion. This is a nationwide multicenter observational study including all dialysis patients regardless of age and gender. Those with acute renal failure and vacationers were excluded from the study. We included 185 centers including 176 hemodialysis (HD) centers and nine peritoneal dialysis (PD) centers with a total number of patients at 11,238, a number of HCV-positive patients at 402, and a number of functional machines at 3139. The mean age of a patient was 55.6 years (range: 18-65), and sex ratio was 0.9. The prevalence of HCV-positive patients is 3.6%; it is higher in private centers with an average of 2.7 compared to 1.18 in public centers with a significant difference (P = 0.009). The prevalence of HCV-positive patients was significantly higher in HD centers compared to PD centers, in centers where the number of generators was >15 and when the number of patients per center is >70. One hundred and fifty-six patients seroconverted after dialysis, i.e., a prevalence of seroconversion at 1.3% with a mean delay of 6.052 ± 5.3 years. Our study shows a lower prevalence of HCV seroconversion than that reported in the literature; this requires a national survey to be carried out by homogenizing virological diagnostic kits and treating infected patients to eradicate this infection in dialysis patients.

Abstract Proliferative glomerulonephritis with monoclonal immunoglobulin deposits is a new disorder with undefined treatment modalities. We propose cyclophosphamide‐bortezomib‐dexamethasone and autologous stem cell transplantation as a therapeutic protocol.

Abstract Renal involvement in Niemann‐Pick disease type B is very rare. Kidney check‐up and renal biopsy should be performed in any patient presented with hypertension and kidney disease. Histology identifies the lesion, the prognosis, and guide treatment.

This article is about the United Kingdom’s withdrawal procedure from the European Union, in the aftermath of the Brexit referendum. It analyses the action of the political institutions involved in the withdrawal agreement negotiations, in particular, the constitutional dispute about the exit deal between the UK Government and the Parliament, as well as the way the European Union acted in the course of the whole procedure. Special attention will be given to the decisions delivered by the UK Supreme Court and by the European Court of Justice regarding the withdrawal procedure. The article aims to offer a new perspective, different from the prevailing accounts on the United Kingdom withdrawal negotiations, one that is based on a critical analysis of the attitudes of the main actors in the exit agreement, trying to apprehend their interaction during the whole process.

Abstract Dermoscopy is a useful tool that helps distinguish lupus miliaris disseminatus faciei (LPDF) from sarcoidosis and tuberculosis. Follicular keratotic plugs (FKP) represent the hallmark of LPDF. Dermoscopic aspect of LPDF changes through the course of the disease.

Background. Renal amyloidosis is one of the main differential diagnoses of nephrotic proteinuria in adults and the elderly. The aim of this study with the most important series in our country is to contribute to the epidemiological, clinical, and etiological study of the renal amyloidosis. Methods. In a retrospective study carried out between 1975 and 2019, 310 cases of histologically proven and typed renal amyloidosis were selected for this study. Results. There were 209 men and 101 women with a mean age of 53.8 ± 15.4 years (range, 17–84 years). Of the 310 cases, 255 (82.3%) were diagnosed with AA renal amyloidosis and 55 (17.7%) with non-AA amyloidosis. Infections were the main cause of AA amyloidosis, and tuberculosis was the most frequent etiology. The period from the onset of the underlying disease to diagnosis of the renal amyloidosis was an average of 177 months. The most frequent manifestations at the time of diagnosis were nephrotic syndrome (84%), chronic renal failure (30.3%), and end-stage renal disease (37.8%). After a medium follow-up of 16 months (range, 0–68 months), mortality occurred in 60 cases. Conclusions. Given the high frequency of AA amyloidosis in our country, awareness of the proper management of infectious and chronic inflammatory diseases remains a priority in reducing the occurrence of this serious disease.

This article explores the Brexit referendum, focusing on the political events that led to the vote, namely, the Conservative Party’s return to power, David Cameron’s attempts to appease the European divide within his party and the role played by the British Parliament in the whole process. It then discusses whether the referendum was the most suitable way to decide on European Union membership, considering the sovereignty of the British Parliament, as well as the contrast between representative democracy and direct democracy.

Light-chain deposition disease (LCDD) reoccurs almost invariably after renal transplantation, leading to early graft loss. We report a case of LCDD with monoclonal gammopathy of renal significance diagnosed in the post-transplant period in a 28-year-old male and we discuss the diagnostic and therapeutic challenges in the clinical course.

Renal involvement is rare in systemic sarcoidosis. Among renal manifestations, tubulointerstitial nephritis (TIN) is the most commonly reported finding. We conducted the current study to investigate the clinical, laboratory, and histological features and to analyze the outcome of TIN due to sarcoidosis. We present a retrospective, single-center study of patients followed for sarcoidosis and presenting with TIN related to this systemic disease. Twenty-four patients were assessed (22 females/2 males). The mean age at diagnosis of TIN was 46.3 years. Extrarenal manifestations were dominated by thoracic involvement (95.8%), peripheral lymph nodes (54.2%), and skin lesions (33.3%). The mean proteinuria level was 0.68 g/24 h. Renal failure was diagnosed in 83.3% of cases with a median estimated glomerular filtration rate at 14.3 mL/min/1.73 m2. Nine patients presented with hypercalcemia and 12 patients with hypercalciuria. Renal biopsy was performed in 58.3% of cases. Six of the 14 patients presented with noncaseating granulomatous interstitial nephritis and eight with interstitial nephritis without granuloma. Granulomatous infiltration of renal parenchyma was complicated by vasculitis in two cases. Corticosteroid therapy was used in all patients. On follow-up analysis, four patients progressed to end-stage renal disease (ESRD) after a mean duration at 45.5 months. In the remaining patients, kidney function statistically significantly improved after one month of treatment compared to the time when the diagnosis was initially established (P = 0.031). We found that the predictive factors of progression to ESRD were multiorgan involvement (P = 0.032), advanced fibrosis F3 (P = 0.0006), and extensive interstitial granulomas (P = 0.007) and these were independently correlated with ESRD. Corticosteroid therapy seems to be effective in sarcoid TIN, but some degree of persistent renal failure is possible which can be predicted from both histologic findings and initial response to steroid therapy.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, which usually manifests in adulthood. It is characterized by the development of multiple cysts in the kidneys and many other extrarenal manifestations. We aimed to determine the factors that contribute to the progression of ADPKD to end-stage renal disease (ESRD). In a retrospective multicentric study, we reviewed the records of 569 patients with ADPKD, hospitalized at a nephrology department or followed up at the outpatient department of university and regional hospitals, covering the north and center of the country, during the period 1969–2016. The mean age of the study patients was 48.54 ± 13.68 years and 14% were young adults (40 years (P = 0.009), hematuria (P = 0.034), hemoglobin >14 g/dL (P = 0.0013), high uric acid level (P = 0.001), and leukocyturia (P = 0.02). Death occurred in 59 cases (10.3%), mostly caused by infections (44.1%). In our study, ADPKD was lately diagnosed in most cases. Family screening is important, which will enable early detection and management of the complications associated with ADPKD.

BackgroundPrimary membranous nephritis (PMN) is an autoimmune disease induced by the deposit of antibodies (Ab) to the phospholipase receptor A2 receptor (PLA2R) on podocytes. In this context, we aimed to assess the relationships between anti-PLA2R Ab, PLA2R rs4664308 SNP, PLA2R mRNA levels and PMN susceptibility and outcome.MethodsSixty-eight PMN patients, 30 systemic lupus erythematosus (SLE) patients with secondary MN and 30 healthy control subjects served for anti-PLA2R Ab measurement by ELISA and PLA2R rs4664308 SNP genotyping by a commercial real-time PCR. Twenty patients with tubulo-interstitial nephritis (TIN) were used as controls for renal PLA2R mRNA quantification in PMN patients from kidney biopsies. PLA2R mRNA quantification was carried-out by real-time PCR after RNA extraction.ResultsForty-three (63.2%) PMN patients received initial therapy consisting of alternating monthly cycles of corticosteroids and cyclophosphamide. Twelve (17.6%) patients had resistant PMN to initial therapy and were consecutively treated by cyclosporine or tacrolimus. Anti-PLA2R Ab were positive in 54 (79.4%) PMN patients, while all SLE patients and controls were negative, pConclusionAnti-PLA2R Ab and renal PLA2R mRNA could be useful markers for PMN outcome predicting. The PLA2R rs6446308 SNP is associated with PMN susceptibility in Tunisians.

Secondary renal amyloidosis (RA) is the most common type of renal involvement in ankylosing spondylitis (AS). We assessed the epidemiologic and clinico-biological profile of AS patients with RA, to analyze treatment modalities and prognostic aspects, and to determine predictive factors of RA during AS. This was a retrospective study including 13 cases of RA among 212 cases who presented with AS, during the period from 1978 to 2006. The median age of the patients at the time of diagnosing AS was 47 years (range: 19–67). There were 11 males and two females. RA onset was diagnosed after a mean follow-up of 144.6 months (range: 10–505) from the AS diagnosis. We noted erosive peripheral arthritis, lumbar stiffness with bamboo spine, and coxitis in 23.1%, 76.9%, and 30.8% of cases, respectively. Nephrotic syndrome was found in eight patients (61.5%). At the time of diagnosing RA, six patients had renal failure. Amyloid deposits were histologically proven by salivary gland biopsy in six cases (46.1%) and by renal biopsy in seven cases (53.8%). Four patients received a long-course treatment with colchicine but with a good outcome only in two cases. In our series, AS was more severe among patients with RA. Four predictive factors of RA were identified: smoking (P = 0.04), erosive peripheral arthritis (P = 0.002), bamboo spine (P = 0.001), and biologic indicators of inflammation (P = 0.0001). High erythrocyte sedimentation rate was identified as the only independent risk factor of RA during AS (P = 0.0001). Renal function as well as urinalysis should be monitored at regular intervals to detect early renal involvement during AS.

Adult second language (L2) learning is a challenging enterprise inducing neuroplastic changes in the human brain. However, it remains unclear how the structural language connectome and its subnetworks change during adult L2 learning. The current study investigated longitudinal changes in white matter (WM) language networks in each hemisphere, as well as their interconnection, in a large group of Arabic-speaking adults who learned German intensively for 6 mo. We found a significant increase in WM-connectivity within bilateral temporal-parietal semantic and phonological subnetworks and right temporal-frontal pathways mainly in the second half of the learning period. At the same time, WM-connectivity between the two hemispheres decreased significantly. Crucially, these changes in WM-connectivity are correlated with L2 performance. The observed changes in subnetworks of the two hemispheres suggest a network reconfiguration due to lexical learning. The reduced interhemispheric connectivity may indicate a key role of the corpus callosum in L2 learning by reducing the inhibition of the language-dominant left hemisphere. Our study highlights the dynamic changes within and across hemispheres in adult language-related networks driven by L2 learning. [ABSTRACT FROM AUTHOR]

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Diabetic nephropathy (DN) is one of the major complications of type 2 diabetes mellitus (T2DM). The diagnosis of DN is mostly clinical. Kidney biopsy is indicated only if nondiabetic renal disease (NDRD) is suspected. This study is aimed to assess the prevalence of NDRD and to determine predictor and prognostic factors of DN, NDRD. It was a retrospective analytic study including T2DM patients in whom renal biopsies were performed at our department from 1988 to 2014. Seventy-five patients were included. Mean age was 52.7 years with sex ratio at 1.56. Renal biopsy findings were isolated NDRD in 33 cases, NDRD superimposed on DN in 24 cases, and isolated DN in 18 cases. Most common NDRD found were focal segmental glomerulosclerosis (21%) and membranous nephropathy (19%). Multivariate analysis showed that the absence of ischemic heart disease [odds ratio (OR) = 0.178, 95% confidence interval (CI) = 0.041–0.762], absence of peripheral vascular disease (OR = 0.173, 95% CI = 0.045–0.669), and presence of hematuria (OR = 7.200, 95%CI = 0.886–58.531) were independent predictors of NDRD. 24 patients reached end-stage renal disease 55% in DN group, 16% in DN associated to NDRD group, and 30% in NDRD group. The prevalence of NDRD found in our study confirmed usefulness of renal biopsy in patients with T2DM, especially in those without degenerative complications, hypertension, and insulin therapy.

Mortality in systemic lupus erythematosus (SLE) has decreased with the advent of immunosuppressive therapy and the development of hemodialysis. This study aims to evaluate the survival rate, factors of poor prognosis, and causes of death in SLE in a Tunisian series. The records of all SLE patients followed up in a single center during 1974–2014 were reviewed. The causes of death were identified. Prognostic factors of survival were analyzed by multivariate analysis using the comparison of the survival rates by the log-rank test. Two hundred ninety-nine patients with SLE were included (274 women, 25 men) aged meanly of 27.52 years at diagnosis. The death occurred in 50 cases (16.7%). The mean age at death was 28.46 years (14–69 years). The patient survival rates at 5, 10, and 20 years were 83.8%, 78.6%, and 56.7%, respectively. The leading causes of death were active SLE (50%) and infectious complications (36%). Independent factors of poor prognosis identified by multivariate analysis were myocarditis (P = 0.029), splenomegaly (P = 0.0015), and worsening of renal function (P = 0.004). Remission was identified as a protective factor (P = 0.047). Our study shows that renal disease remains the primary cause and the main predictor of death in SLE, which is consistent with the literature data.

Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.