Guillain-Barré Syndrome with Absent Brainstem Reflexes: a Case Report

A 41-year-old man was admitted to an intensive care unit following respiratory arrest. One day prior to admission, he had complaints of nausea and pain involving lower limbs. On the night of admission he developed diplopia, dysphagia, and rapidly progressive quadriparesis. He developed respiratory failure requiring mechanical lung ventilation 24 hours later. On the fifth day of hospital stay the patient became comatose with absent brainstem reflexes and appeared to be brain dead. The cerebrospinal fluid showed albuminocytological dissociation. The electroencephalogram revealed an alpha rhythmical activity. The electrophysiological evaluation revealed an inexcitability of all nerves. Guillain-Barré syndrome was suspected. With supportive treatment the patient had a remarkable recovery and now is able to independently conduct his daily activities.

Severe Case of Thrombotic Microangiopathy with a Delayed Diagnosis of Atypical Haemolytic Uraemic Syndrome Successfully Managed with Eculizumab

Objectives: To describe the diagnosis and treatment of a severely ill patient presenting with thrombotic microangiopathy (TMA) of unknown cause. Case presentation: An adult female presented to intensive care with abdominal pain and haemorrhagic shock, requiring reanimation. Results: Features of TMA were present, but initial plasma exchange was ineffective. Treatment with the anti-C5 antibody, eculizumab, improved laboratory parameters and organ function, albeit slowly. Eculizumab remains effective and well tolerated after 30 months of treatment. Conclusion: This case demonstrates the complexities and importance of early identification of atypical haemolytic uraemic syndrome in patients presenting with TMA.

Gestão e desenvolvimento regional na ride/DF: corsap-DF/GO como modalidade de associativismo territorial

Dissertação (mestrado)—Universidade de Brasília, Instituto de Ciências Humanas, Departamento de Geografia, Programa de Pós-Graduação em Geografia, 2015.

Spontaneous Cirrhosis Regression in an IFN-beta-induced AIH-like Syndrome Following Drug Withdrawal: Art of Facts or Artifacts?

Autoimmune hepatitis (AIH) is a disease of unknown aetiology with drug-induced AIH being the most complex and not fully understood type. We present the case of a 57-year-old female patient with acute icteric hepatitis after interferon-beta-1b (IFNβ-1b) administration for multiple sclerosis (MS). Based on liver autoimmune serology, histology and appropriate exclusion of other liver diseases, a diagnosis of AIH-related cirrhosis was established. Following discontinuation of IFNβ-1b, a complete resolution of biochemical activity indices was observed and the patient remained untreated on her own decision. However, 3 years later, after a course of intravenous methylprednisolone for MS, a new acute transaminase flare was recorded which subsided again spontaneously after 3 weeks. Liver biopsy and elastography showed significant fibrosis regression (F2 fibrosis). To our knowledge, this is the first report showing spontaneous cirrhosis regression in an IFNβ-1b-induced AIH-like syndrome following drug withdrawal, suggesting that cirrhosis might be reversible if the offending fibrogenic stimulus is withdrawn.

Diseño e implementación de un sistema de soporte de decisiones para el Centro de Documentación Regional “Juan Bautista Vázquez”

El volumen de datos en bibliotecas ha aumentado enormemente en los últimos años, así como también la complejidad de sus fuentes y formatos de información, dificultando su gestión y acceso, especialmente como apoyo en la toma de decisiones. Sabiendo que una buena gestión de bibliotecas involucra la integración de indicadores estratégicos, la implementación de un Data Warehouse (DW), que gestione adecuadamente tal cantidad de información, así como su compleja mezcla de fuentes de datos, se convierte en una alternativa interesante a considerar. El artículo describe el diseño e implementación de un sistema de soporte de decisiones (DSS) basado en técnicas de DW para la biblioteca de la Universidad de Cuenca. Para esto, el estudio utiliza una metodología holística, propuesto por Siguenza-Guzman et al. (2014) para la evaluación integral de bibliotecas. Dicha metodología evalúa la colección y los servicios, incorporando importantes elementos para la gestión de bibliotecas, tales como: el desempeño de los servicios, el control de calidad, el uso de la colección y la interacción con el usuario. A partir de este análisis, se propone una arquitectura de DW que integra, procesa y almacena los datos. Finalmente, estos datos almacenados son analizados y visualizados a través de herramientas de procesamiento analítico en línea (OLAP). Las pruebas iniciales de implementación confirman la viabilidad y eficacia del enfoque propuesto, al integrar con éxito múltiples y heterogéneas fuentes y formatos de datos, facilitando que los directores de bibliotecas generen informes personalizados, e incluso permitiendo madurar los procesos transaccionales que diariamente se llevan a cabo.

Prevalence of thoracic spine pain in a surveillance network

International audience

An epidemiological surveillance network of lumbar disc surgery to help prevention of and compensation for low back pain

International audience

Rett syndrome

Rett syndrome is one of the most common causes of complex disability in girls. It is characterized by early neurological regression that severely affects motor, cognitive and communication skills, by autonomic dysfunction and often a seizure disorder. It is a monogenic X-linked dominant neurodevelopmental disorder related to mutation in MECP2, which encodes the methyl-CpG-binding protein MeCP2. There are several mouse models either based on conditional knocking out of the Mecp2 gene or on a truncating mutation. We discuss the clinical aspects with special emphasis on the behavioral phenotype and we review current perspectives in clinical management alongside with perspectives in altering gene expression. Copyright © 2012 S. Karger AG, Basel.

Dor pélvica crónica de origem ginecológica

A dor pélvica crónica (DPC) é um problema comum, que se apresenta como um grande desafio para os clínicos que com ela têm que lidar, pela sua etiologia desconhecida, pela história natural complexa e pela má resposta terapêutica. São muitas e variadas as causas de DPC, devendo esta ser abordada numa perspectiva multidisciplinar. Afecta cerca de 1 em cada 7 mulheres nos EUA, com uma prevalência de cerca de 39% nas mulheres em idade fértil, sendo mais frequente nas mulheres entre os 26-30 anos de idade e nas mulheres de raça negra.As causas mais frequentes de DPC de origem ginecológica são: a endometriose, a adenomiose, os leiomiomas, o síndroma da congestão pélvica e a doença inflamatória pélvica crónica.

The treatment of oral lesions in Behçet's Syndrome: case report

Behçet's disease (BD) is a multi-systemic vascular disorder characterized by oral and genital ulcers, cutaneous, ocular, arthritic, vascular, central nervous system and gastrointestinal involvement. It usually affects young adults and the etiopathogenesis is unknown. A 21-year-old girl, Caucasian, with diagnostic BD, presented with rheumatoid arthritis, genital lesions and multiple recurrent ulcers inside the mouth, with an erythematous halo, covered by yellowish exudates exacerbated during menstrual periods, and in situations of stress and anxiety. The application of low power laser in ulcers was considered in order to decrease the inflammatory symptoms and pain, beyond getting the healing process accelerated. The proposed therapy was able to promote pain relief, increase local microcirculation and repair ulcerated lesions, eliminating the need for administration of systemic or topical medications, leading to improved quality of life.

Behçet's syndrome: literature review and clinical case report

Behçet's disease (BD) is a multi-systemic vascular disorder characterized by oral and genital ulcers, as well as cutaneous, ocular, arthritic, vascular, central nervous system and gastrointestinal involvement. It usually affects young adults, and its pathological origin is unknown. The case of a 47-year-old woman with recurrent ulcers in the oral cavity is presented. She linked the pain with sitting and during the sexual act, with vaginal and oral cavity pain, due to the lesions present at those sites, as well as swelling and pain in the knees, making walk painful. The patient was kept under observation and underwent multidisciplinary treatment with prescription of topical and systemic drugs to improve quality of life. Dentists should be aware of BD and the need of multidisciplinary treatment to increase the patient's quality of life.

Síndrome de Burnout : um estudo com guias de turismo regional em Natal-RN

Burnout is a psychological syndrome triggered in response to continuous exposure to interpersonal stressors. It is considered a multifactorial construct, which is commonly characterized by three dimensions: emotional exhaustion, dehumanization, and lack of personal accomplishment.This study aimed to verify if the three characteristics of burnout (exhaustion, lack of dehumanization and personal accomplishment) are present in people working as guides Tourism in Natal - RN. It is a descriptive and quantitative study. 109 subjects were surveyed. Data collection was done through the use of questionnaires, the instrument used was the characterization of the Burnout Scale (ECB) created and validated in Brazil by Trocoli and Tamayo (2000). In order to analyze data we used descriptive statistics, analysis of core measures, exploratory and confirmatory factor analysis, reliability analysis, cluster analysis, multiple discriminant and Spearman correlation. Factor analysis identified four factors that explain 58.3% of the total variance. Those factors were named exhaustion, deception, avoidance, and dehumanization. The reliability of the instrument, as measured by Cronbach's Alpha was 0.918, which is considered excellent reliability. The 109 subjects were grouped into three cluster, which had the deception, avoidance, and dehumanization as discriminant. It is possible to conclude that the characteristics of burnout syndrome are present in the studied population where 19 people are on the high level of burnout, moderate in 32 and 56 in the light. The correlations between socio-demographic variables studied and the dimensions of burnout, were few and weak. The variable leave for health reasons in the study appeared to be related to feelings of exhaustion and avoidance behavior appeared related to younger individuals and who work only in the activity of Receptive Tourism Guide. Verification of the incidence of burnout in individuals surveyed suggest the need to adopt intervention strategies are individual, organizational and / or combined

Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability

International audience

Clinical Practice Guideline: irritable bowel syndrome with constipation and functional constipation in the adult

In this Clinical Practice Guideline we discuss the diagnostic and therapeutic approach of adult patients with constipation and abdominal complaints at the confluence of the irritable bowel syndrome spectrum and functional constipation. Both conditions are included among the functional bowel disorders, and have a significant personal, healthcare, and social impact, affecting the quality of life of the patients who suffer from them. The first one is the irritable bowel syndrome subtype, where constipation represents the predominant complaint, in association with recurrent abdominal pain, bloating, and abdominal distension. Constipation is characterized by difficulties with or low frequency of bowel movements, often accompanied by straining during defecation or a feeling of incomplete evacuation. Most cases have no underlying medical cause, and are therefore considered as a functional bowel disorder. There are many clinical and pathophysiological similarities between both disorders, and both respond similarly to commonly used drugs, their primary difference being the presence or absence of pain, albeit not in an "all or nothing" manner. Severity depends not only upon bowel symptom intensity but also upon other biopsychosocial factors (association of gastrointestinal and extraintestinal symptoms, grade of involvement, and perception and behavior variants). Functional bowel disorders are diagnosed using the Rome criteria. This Clinical Practice Guideline has been made consistent with the Rome IV criteria, which were published late in May 2016, and discuss alarm criteria, diagnostic tests, and referral criteria between Primary Care and gastroenterology settings. Furthermore, all the available treatment options (exercise, fluid ingestion, diet with soluble fiber-rich foods, fiber supplementation, other dietary components, osmotic or stimulating laxatives, probiotics, antibiotics, spasmolytics, peppermint essence, prucalopride, linaclotide, lubiprostone, biofeedback, antidepressants, psychological therapy, acupuncture, enemas, sacral root neurostimulation, surgery) are discussed, and practical recommendations are made regarding each of them.

First case reported of Bouveret's syndrome associated to duodenal and biliary perforation to retroperitoneum

We present the case of a 69 year old woman with a history of cholecystitis, who consulted for severe abdominal pain, nausea and vomiting. Abdominal CT showed duodenal obstruction caused by a gallstone, cholecystoduodenal fistula and pneumobilia, what is known as Bouveret's syndrome, a rare form of gallstone ileus. Additionally, she presented free duodenal and vesicular perforation to retroperitoneum at the same level of the cholecystoduodenal transit point. The patient underwent a difficult cholecystectomy, enterolithotomy, repair of the duodenal defect, extensive washing and drainage of the retroperitoneum. The postoperative course was uneventful except for a laparotomy infection.