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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.

Lipoblastoma is a rare, benign tumor usually occurring in childhood. It is essentially localized in the extremities and trunk, with few cases reported in the neck. We report the case of a 2-year-old girl with a rapidly enlarging, painless neck mass. Magnetic resonance imaging (MRI) revealed a 3-cm mass in the right submandibular region. Review of literature, diagnostic methods, and genetics of lipomatous tumors are discussed. Complete surgical excision via a lateral cervical approach demonstrated a white soft tissue with an adherent ganglion. Histology and immunohistochemistry confirmed the diagnosis of lipoblastoma. Cervical lipoblastoma is rare, and typically asymptomatic, rarely causing nerve compression or airway obstruction. MRI can help identifying the lipomatous nature of the mass, but the findings can be inconsistent due to variable maturity of fat cells and the mesenchymal content of the tumor. Diagnosis is always based on pathological examination. Further chromosomal analysis is useful in differentiating lipoblastoma from liposarcoma. Complete surgical excision is the recommended treatment.

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.

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.

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.

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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Background: Mammary Paget’s disease is an uncommon form of primary breast cancer. The aim of this study is to assess our institution’s experience in its management. Methods:We retrospectively reviewed the medical records of 53 female patients with histologically confirmed Paget’s disease, treated at the Salah Azaïz Institute between 2001 and 2010. Results: There were palpable masses in 71.7% of cases, of which 90% revealed invasive carcinoma. Approximately 48% of underlying malignancies were multifocal/multicentric. Overall, invasive carcinoma accounted for 69.8% with a median tumor size of 40 mm, high grade in 62.2%, and negative hormone receptor in 47.6% of cases. There was only one case with direct dermis invasion among those with no underlying invasive carcinoma. After a median follow-up of 45 months, 49% of patients presented with relapse/progression and 47.1% died from their disease. Median overall survival was 67 months, whereas disease-free survival was 65 months. Tumor and node advanced clinical stages correlated with poor survival, as well as the presence of invasive carcinoma with additional negative impacts of large tumor size and lymph node involvement. Tumor stage was the only independent indicator on multivariate analysis. Conclusion: The general trend for decreased incidence of Paget’s disease is noted parallel to earlier breast cancer diagnosis. Paget’s disease is at high risk of multifocal/multicentric underlying tumors. The presence of a palpable mass is almost pathognomonic of invasive neoplasm. The major challenge concerns aggressiveness of surgical procedures with breast and axilla preservation perspectives. Prognosis is mainly determined by that of an eventual underlying breast tumor.

La néphropathie de la vascularite à IgA conditionne le pronostic de cette affection chez l’adulte. Le but de notre étude était d’étudier les caractéristiques cliniques de cette atteinte rénale chez l’adulte et d’identifier les facteurs de pronostic rénal. Il s’agit d’une étude monocentrique rétrospective portant sur les patients ayant une vascularite à IgA (purpura rhumatoïde) (critères de l’EULAR) avec une atteinte rénale prouvée histologiquement et classée selon la classification de Pillebout. Nous avons analysé la survie rénale et identifier les facteurs de pronostic rénal. Vingt cinq patients ont été inclus (genre ratio M/F = 2,57) d’âge moyen au diagnostic du purpura rhumatoïde de 35,76 ans. Un purpura était présent dans 100% des cas avec une atteinte articulaire dans 28%. Une insuffisance rénale était présente dans 44% des cas. La classification histologique la plus fréquente était la classe II. Une rémission clinique a été observée dans 44% des cas et une évolution vers le stade terminal de l’insuffisance rénale chronique dans 36% des cas. La survie rénale à 195 mois était de 57%. Les facteurs pronostiques identifiés étaient l’atteinte digestive (p = 0,022), l’insuffisance rénale initiale (p = 0,0004), la classification glomérulaire (p = 0001) et la sévérité des lésions histologiques, le traitement par bloqueurs du système rénine angiotensine (p = 0,01) et les échanges plasmatiques (p = 0,03). Notre étude montre que l’atteinte rénale au cours des vascularites à IgA peut être relativement sévère avec un mauvais pronostic rénal. L’identification des facteurs pronostiques cliniques et histologiques pourrait guider l’élaboration d’études thérapeutiques prospectives.

To determine the features of the different forms of kidney diseases associated with multiple myeloma (MM), we retrospectively studied 144 patients with kidney injury and MM at our institute from 1974 to 2014. The mean age of the patients was 60.1 years and the male:female ratio was 1.25. Renal disease was concomitant with the discovery of MM in 92% of cases. The mean follow-up of our patients was 2.1 years. Initial renal insufficiency was found in 131 (91%) patients. Cast nephropathy of the distal tubule was found in 110 (26%) patients, renal amyloidosis in 16 (11.1%), and light chain deposition disease in five (3.47%). Twelve (8.3%) patients had chronic glomerular nephropathy. Twenty-six patients reached end-stage renal failure within 13.4 months. Renal survival was 30.45 months. Predictive factors for improvement of renal function undergoing chemotherapy included serum creatinine

The article is intended to debate two questions regarding the involvement of the Commission in the Troika's action: firstly, considering the nature of financial assistance programs, it aims to discuss the effect of the Commission's participation in Troika negotiations on the balance of power of the EU institutions; and secondly, the article raises the issue of the Commission's liability for the results achieved by the financial assistance program, taking into account the extent of the conditions imposed, as well as the intensity of scrutiny by the Troika.

Light chain deposition disease (LCDD) is characterized by the tissue deposition of monotypic immunoglobulin light chains of either kappa or lambda isotype. It is the archetypal systemic disease that is most frequently diagnosed on a kidney biopsy, although the deposits may involve several other organs. This brief review focuses on the clinicopathological features of LCDD-associated nephropathy with an emphasis on the diagnostic and therapeutic difficulties related to this elusive condition.

Objectivos Descrever o perfil terapêutico da hipertensão na Rede de Médicos Sentinela e analisar o padrão de prescrição dos anti-hipertensores de acordo com a forma de hipertensão e co-morbilidades. Tipo de estudo Estudo transversal Local Rede de Médicos-Sentinela de Portugal População Doentes hipertensos que se deslocaram á consulta entre 15 de Setembro e 30 de Outubro de 1998, à excepção de doentes menores de 18 anos e de mulheres grávidas. Métodos Os Médicos Sentinela participantes recolheram informação referente a doentes hipertensos, informação essa que contemplou a caracterização demográfica dos doentes, a caracterização da hipertensão (forma e repercussão em orgãos alvo) e a caracterização da terapêutica instituída. Resultados Noventa e sete clínicos gerais recolheram informação de 1125 doentes hipertensos, dos quais 64,4% eram do sexo feminino (n=716). A média das idades foi de 62,6 e 60,7 anos, respectivamente para o sexo feminino e masculino. A forma de hipertensão moderada foi encontrada em 57,7% (n=646) dos casos, enquanto que 12,4% (n=139) tinham hipertensão grave. Da amostra de doentes 36,2% (n=398) tinham sido diagnosticados há 5 ou menos anos e 34,8% (n=383) há mais de 10 anos. As co-morbilidades mais frequentemente encontradas foram a dislipidemia (n=482), a hiperuricemia (n=199) e a diabetes (n=198). Um regime de monoterapia foi instituído a 48,0% (n=536) doentes, sendo os IECA o grupo terapêutico mais prescrito (50,9%). Conclusões Os IECA foram os fármacos mais frequentemente prescritos para o tratamento da hipertensão e a sua selecção não parece estar relacionada com a forma da hipertensão e com a existência de morbilidade associada. O número de fármacos prescrito verificou-se ser independente da existência de diabetes, dislipidémia e hiperuricémia nos doentes hipertensos em estudo. Publicado em: Rev Port Clín Geral. 2001; 17:359-72.

Author(s): Abir Boussetta [sup.1] [sup.2] , Manel Jellouli [sup.1] [sup.2] , Raja Aouaidia [sup.2] [sup.3] , Rim Goucha [sup.2] [sup.4] , Taher Gargah [sup.1] [sup.2] Author Affiliations: (1) grid.413827.b, 0000 [...]