Pneumatosis Cystoides Intestinalis Induced by Acarbose Treatment

A "Pneumatosis Cystoides Intestinalis" é uma situação clínica rara que se caracteriza pela presença de múltiplos quistos de conteúdo gasoso ao nível da submucosa ou subserosa na parede do tracto gastrointestinal. A pneumatose intestinal classifica-se em idiopática e secundária. Na última forma admite-se uma relação causal com doença pulmonar obstrutiva crónica, conectivites, amiloidose, colites infecciosas, oclusão intestinal, isquémia, doença de Crohn, fármacos e iatrogenia cirúrgica e endoscópica. O espectro de manifestações clínicas compreende dor abdominal, oclusão intestinal, diarreia e hemorragia digestiva. Todavia, é frequentemente assintomática ou constitui um achado incidental no decurso de uma investigação não relacionada. Os autores apresentam o caso clínico de uma doente com pneumatose quística intestinal associada à utilização terapêutica de um antidiabético oral - acarbose (inibidor da alfa-glucosidase)

Primary HIV Infection in a Crohn's Disease Patient Receiving Infliximab Maintenance Therapy

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Lymphoproliferative Disorders in Inflammatory Bowel Disease Patients: Is It the Drugs or the Disease

Introduction: Systemic inflammatory diseases are related to an increased risk of lymphoproliferative disorders. Although inflammatory bowel disease (IBD) was also associated with these conditions, population-based studies failed to demonstrate this relationship, and most studies only identified a very small number of cases. In the last few years, concerns arose regarding the role of thiopurines and tumour necrosis factor-alpha (TNF-α)-blocking agents in the development of lymphoma, influencing therapeutic decisions in IBD patients. The aim of this study was to describe a case series of IBD patients who developed a lymphoproliferative disorder in our tertiary referral centre. Material and methods: The clinical records of all IBD patients who were observed in our unit between January 2007 and December 2016 were retrospectively reviewed, and IBD subjects who were diagnosed with a lymphoproliferative disorder were selected. Clinical and demographic data regarding both conditions were collected. Results: Six IBD patients were diagnosed with a lymphoma - 4 Hodgkin lymphomas and 2 B-cell non-Hodgkin lymphomas - of which 3 corresponded to primary colonic lymphomas. Immunohistochemical analysis detected the presence of Epstein-Barr virus in the tumour cells of 2 patients, both of them with Hodgkin lymphomas. Only 2 patients were previously treated with thiopurines or anti-TNF-α drugs; none of the remaining had any history of immunosuppressive treatment. Discussion and conclusions: Despite major attention being currently focused on the effect of treatment, which may play the main role in the increased susceptibility to lymphoma in IBD patients, and although it may be difficult to demonstrate, IBD itself may contribute to the development of lymphoproliferative disorders, particularly primary intestinal lymphomas.info:eu-repo/semantics/publishedVersio

Linfoma de Hodgkin em Doença de Crohn Medicada com Infliximab

INTRODUCTION: Lymphoproliferative disorders, particularly non-Hodgkin's and Hodgkin's lymphomas, are rare in patients with inflammatory bowel diseases. The use of thiopurines and infection by Epstein-Barr virus are well-known cofactors that can raise its prevalence. Other risk factors such as disease activity and biological treatment are the subject of discussion, without enough data in the literature to confirm a potential association. METHODS: We report a case of Hodgkin's lymphoma in a patient who had been treated with azathioprine and was on long-term monotherapy with infliximab. CONCLUSIONS: We stress the importance of recognizing the possible occurrence of a lymphoproliferative disorder in association with anti-tumor necrosis factor-α therapy.info:eu-repo/semantics/publishedVersio

A composite measure to explore visual disability in primary progressive multiple sclerosis

Background: Optical coherence tomography (OCT) and magnetic resonance imaging (MRI) can provide complementary information on visual system damage in multiple sclerosis (MS). Objectives: The objective of this paper is to determine whether a composite OCT/MRI score, reflecting cumulative damage along the entire visual pathway, can predict visual deficits in primary progressive multiple sclerosis (PPMS). Methods: Twenty-five PPMS patients and 20 age-matched controls underwent neuro-ophthalmologic evaluation, spectral-domain OCT, and 3T brain MRI. Differences between groups were assessed by univariate general linear model and principal component analysis (PCA) grouped instrumental variables into main components. Linear regression analysis was used to assess the relationship between low-contrast visual acuity (LCVA), OCT/MRI-derived metrics and PCA-derived composite scores. Results: PCA identified four main components explaining 80.69% of data variance. Considering each variable independently, LCVA 1.25% was significantly predicted by ganglion cell-inner plexiform layer (GCIPL) thickness, thalamic volume and optic radiation (OR) lesion volume (adjusted R2 0.328, p = 0.00004; adjusted R2 0.187, p = 0.002 and adjusted R2 0.180, p = 0.002). The PCA composite score of global visual pathway damage independently predicted both LCVA 1.25% (adjusted R2 value 0.361, p = 0.00001) and LCVA 2.50% (adjusted R2 value 0.323, p = 0.00003). Conclusion: A multiparametric score represents a more comprehensive and effective tool to explain visual disability than a single instrumental metric in PPMS

Safe Use of Infliximab for the Treatment of Severe Perianal Crohn's Disease After Diagnosis and Treatment of Lymphoma

Inflammatory bowel disease is associated with an increased likelihood of developing lymphoma. However, it is still controversial if this risk may be attributed to the disease itself or rather represents an effect of immunosuppressive treatment. Although tumor necrosis factor alpha (TNFα) is a key cytokine for cancer immunosurveillance, the potential relationship between anti-TNFα agents and the pathogenesis of lymphoproliferative disorders remains unclear. Here, we describe the case of a patient with severe perianal Crohn's disease, treated with infliximab monotherapy, whose unusual presentation with acute groin pain required surgical intervention and led to the diagnosis of diffuse large B-cell lymphoma. However, 10 months after this episode, treatment with infliximab was restarted because the patient continued with refractory and disabling perianal disease. Currently, with a follow-up of 36 months, under infliximab 10 mg/kg every 4 weeks, he maintains mild perianal Crohn's disease and persists in sustained clinical and imaging remission of the lymphoproliferative disorder.info:eu-repo/semantics/publishedVersio

Synchronization and variability imbalance underlie cognitive impairment in primary-progressive multiple sclerosis.

We aimed to investigate functional connectivity and variability across multiple frequency bands in brain networks underlying cognitive deficits in primary-progressive multiple sclerosis (PP-MS) and to explore how they are affected by the presence of cortical lesions (CLs). We analyzed functional connectivity and variability (measured as the standard deviation of BOLD signal amplitude) in resting state networks (RSNs) associated with cognitive deficits in different frequency bands in 25 PP-MS patients (12 M, mean age 50.9 ± 10.5 years) and 20 healthy subjects (9 M, mean age 51.0 ± 9.8 years). We confirmed the presence of a widespread cognitive deterioration in PP-MS patients, with main involvement of visuo-spatial and executive domains. Cognitively impaired patients showed increased variability, reduced synchronicity between networks involved in the control of cognitive macro-domains and hyper-synchronicity limited to the connections between networks functionally more segregated. CL volume was higher in patients with cognitive impairment and was correlated with functional connectivity and variability. We demonstrate, for the first time, that a functional reorganization characterized by hypo-synchronicity of functionally-related/hyper-synchronicity of functionally-segregated large scale networks and an abnormal pattern of neural activity underlie cognitive dysfunction in PP-MS, and that CLs possibly play a role in variability and functional connectivity abnormalities

Synchronization and variability imbalance underlie cognitive impairment in primary-progressive multiple sclerosis

open11siWe aimed to investigate functional connectivity and variability across multiple frequency bands in brain networks underlying cognitive deficits in primary-progressive multiple sclerosis (PP-MS) and to explore how they are affected by the presence of cortical lesions (CLs). We analyzed functional connectivity and variability (measured as the standard deviation of BOLD signal amplitude) in resting state networks (RSNs) associated with cognitive deficits in different frequency bands in 25 PP-MS patients (12 M, mean age 50.9 ± 10.5 years) and 20 healthy subjects (9 M, mean age 51.0 ± 9.8 years). We confirmed the presence of a widespread cognitive deterioration in PP-MS patients, with main involvement of visuo-spatial and executive domains. Cognitively impaired patients showed increased variability, reduced synchronicity between networks involved in the control of cognitive macro-domains and hyper-synchronicity limited to the connections between networks functionally more segregated. CL volume was higher in patients with cognitive impairment and was correlated with functional connectivity and variability. We demonstrate, from the first time, that a functional reorganization characterized by hypo-synchronicity of functionally-related/hyper-synchronicity of functionally-segregated large scale networks and an abnormal pattern of neural activity underlie cognitive dysfunction in PP-MS, and that CLs possibly play a role in variability and functional connectivity abnormalities.openPetracca M.; Saiote C.; Bender H.A.; Arias F.; Farrell C.; Magioncalda P.; Martino M.; Miller A.; Northoff G.; Lublin F.; Inglese M.Petracca, M.; Saiote, C.; Bender, H. A.; Arias, F.; Farrell, C.; Magioncalda, P.; Martino, M.; Miller, A.; Northoff, G.; Lublin, F.; Inglese, M

Rectal Bleeding as a Form of Presentation of Amebiasis

A amebíase é uma doença infecciosa com diferentes formas de apresentação. Trata-se de um doente de 32 anos, sexo masculino, raça caucasiana, natural e residente em Lisboa, admitido na consulta de Proctologia por rectorragias. O exame objectivo e a avaliação laboratorial não mostraram alterações. Na sigmoidoscopia observaram-se úlceras entre 3 e 5 mm na mucosa rectal e da sigmoideia com friabilidade. A histologia revelou: manutenção da arquitectura, sem depleção das células caliciformes, infiltrado inflamatório intenso, misto com muitos polimorfonucleares neutrófilos e agregados linfóides da lâmina própria, criptite e úlcera com exsudado necroinflamatório, identificando-se Entamoeba histolytica. Medicado com metronidazol com resolução clínica, endoscópica e histológica