Protein-losing enteropathy caused by Yersinia enterocolitica colitis.

A 7-month-old boy was admitted with acute gastro-enteritis accompanied by fever and hyponatraemic dehydration. The clinical course was complicated by severe hypokalaemia and hypo-albuminaemia with anasarca. Protein-losing enteropathy (PLE) owing to Yersinia enterocolitica colitis was diagnosed and was complicated by fungal sepsis owing to Kodomaea ohmeri. Colonoscopy demonstrated multiple diffuse ulcers and sub-epithelial haemorrhages extending from the rectum to the hepatic angle. He required prolonged nutritional support comprising partial parenteral feeding for 10 days, followed by a hypo-allergenic diet until 13 months of age when cow milk was tolerated. He was discharged on a normal diet and in good health at 19 months of age.Abbreviations AVPU scale: A alert, V verbally responsive, P painfully responsive, U unresponsive; CMV: cytomegalovirus; EBV: Epstein-Barr virus; HIV: human immunodeficiency virus; Ig: immunoglobulin; IBD: inflammatory bowel disease; IPEX: immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome; PICU: paediatric intensive care unit; PLE: protein-losing enteropathy.

Health-related quality of life in hereditary transthyretin amyloidosis polyneuropathy: a prospective, observational study.

BACKGROUND: Hereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities. In this study, we aimed to assess the effect of the disease across health-related quality of life dimensions, in both carriers of the mutation and patients, to compare health-related quality of life with general population, as well as to explore health-related quality of life prognostic factors among patients, including disease progression and treatment. METHODS: This study was a multi-institutional, longitudinal, prospective, observational study of hereditary Transthyretin Amyloidosis Polyneuropathy Portuguese adult subjects (621 asymptomatic carriers and 733 symptomatic patients) enrolled in the Transthyretin Amyloidosis Outcomes Survey. Health-related quality of life was captured with the preference-based instrument EQ-5D-3 L. For general population the dataset included all subjects enrolled in a representative national study (n = 1500). Different econometric models were specified; multivariate probit, generalized linear model and generalized estimating equations model; including demographic and clinical covariates. RESULTS: Hereditary Transthyretin Amyloidosis Polyneuropathy patients have their health status severely impaired in all quality of life dimensions and more anxiety/depression problems were found among asymptomatic carriers. No differences on utility were found between carriers and general population (p = 0.209). Among patients, the utility value is estimated to be 0.51 (0.021), a decrement of 0.27 as compared with general population utility. Higher disease duration, advanced disease stage and not receiving treatment are associated with impaired health-related quality of life. No differences were found between genders (p = 0.910) or between late (≥50 years) and early-onset patients (p = 0.254). The utility estimate ranged from 0.63 (0.009) in stage I to 0.01 (0.005) in stage IV. CONCLUSIONS: Hereditary Transthyretin Amyloidosis Polyneuropathy symptoms and progressive associated disabilities substantially decrease patient's health-related quality of life. Clinical strategies focused on health-related quality of life preservation such as close follow-up of asymptomatic carriers, prompt diagnosis and adequate, early treatment would benefit patient's long-term outcomes, slowing the progressive decline in health-related quality of life.

Conduta terapêutica em pacientes com doença pulmonar obstrutiva crônica: monoterapia ou terapia cominada? / Therapy for chronic obstructive pulmonary disease patients: monotherapy or combination therapy?

Objetivo: Comparar a dupla terapia broncodilatadora com glicopirrônio mais indacaterol à monoterapia com glicopirrônio em pacientes portadores de doença pulmonar obstrutiva crônica. Métodos: Estudo clínico prospectivo, unicêntrico, controlado, cruzado, randomizado e duplo-cego realizado com 14 pacientes com diagnóstico de doença pulmonar obstrutiva crônica grau II. Os participantes receberam cada um dos tratamentos durante 30 dias. Antes de cada terapia, realizou-se período de wash-out por 7 dias, com broncodilador de curta ação. Antes e após cada intervenção, os pacientes passaram por exame de espirometria e responderam ao questionário COPD Assessment Test. Resultados: Observou-se melhora na função pulmonar medida por meio do volume expiratório forçado no primeiro segundo de 19mL (±36) para a monoterapia e 87mL (±33) para a terapia dupla. O ganho foi de 67mL (p=0,042) da associação dos medicamentos em relação ao glicopirrônio isolado. A melhora na qualidade de vida, medida a partir das pontuações do questionário, foi de 4,7 (±8,9) pontos para a monoterapia e 5,2 (±11) pontos para a dupla terapia (p=0,08). Conclusão: Ambos os tratamentos demonstram melhora na função pulmonar dos pacientes.

Objective: To compare dual bronchodilator therapy (Glycopyrronium with Indacaterol) versus Glycopyrronium monotherapy in patients with chronic obstructive pulmonary disease. Methods: This was a prospective, unicentric, controlled, crossover, randomized, and double-blind clinical trial with 14 patients diagnosed with grade II chronic obstructive pulmonary disease. The participants received each treatment during the period of 30 days. Before each therapy, a 7-day wash-out period with a short-acting bronchodilator was instituted. Before and after each intervention, the patients underwent spirometry and answered the COPD Assessment Test questionnaire. Results: An improvement in pulmonary function measured by forced expiratory volume during the first second of 19mL (±36) for monotherapy, and 87mL (±33) for dual therapy was observed. The gain was of 67mL (p=0.042) in the association of the drugs in relation to Glycopyrronium alone. The mean improvement in quality of life measured from the questionnaire scores was 4.7 (±8.9) points for monotherapy and 5.2 (± 11) points for dual therapy (p=0.08). Conclusion: Both treatments show improvement in the patients' pulmonary function.

[Erratum to "Does Aortic Valve Replacement Surgery Improve Patients' Quality of Life?", by Pedro COELHO, Lara N. FERREIRA, Clara VITAL, José FRAGATA. Acta Med Port 2018 Jul-Aug;31(7-8):399-408]. / Errata a "A Cirurgia de Substituição Valvular Aórtica Melhora a Qualidade de Vida dos Doentes?" Pedro COELHO, Lara N. FERREIRA, Clara VITAL, José FRAGATA. Acta Med Port 2018 Jul-Aug;31(7-8):399-408.

Article published with errors: https://actamedicaportuguesa.com/revista/index.php/amp/article/view/10241 On page 405, Table 3, where it reads: On bold, the value 34.4 in "limitação do Desempenho" concerning the column "Seguimento a 6 meses" It should read: On bold, the value of 37,9. On page 406, Figure 4, where it reads: The y-axis of index SF-6D values are designed between 0 and 50. It should read: The y-axis of index SF-6D values between are designed between 0 and 1.

Artigo publicado com erros: https://actamedicaportuguesa.com/revista/index.php/amp/article/view/10241 Na página 405, Tabela 3 onde se lê: A negrito o valor 34,4 na dimensão "limitação do Desempenho" referente à coluna "Seguimento a 6 meses"Deverá ler-se: A negrito o valor 37,9. Na página 406, Figura 4, onde se lê:O eixo do índice SF-6D com valores entre 0 a 50.Deverá ler-se:O eixo do índice SF-6D com valores entre 0 a 1.

[Does Aortic Valve Replacement Surgery Improve Patients' Quality of Life?] / A Cirurgia de Substituição Valvular Aórtica Melhora a Qualidade de Vida dos Doentes?

INTRODUCTION: Aortic valve replacement surgery is done to increase patients' life expectancy and improve their health-related quality of life. Several published studies have found improvement in health-related quality of life after this procedure, but none have involved patients in Portugal. This study sought to evaluate patients' health-related quality of life after the implantation of aortic prostheses and compare these values with preoperative health-related quality of life and the general Portuguese population's health-related quality of life. MATERIAL AND METHODS: A retrospective study was done with elective patients who underwent implantation of an aortic prosthesis between August 2011 and April 2016. Health-related quality of life was evaluated using the Short Form 36 Health Survey and Short Form 6 Health Survey questionnaires in the preoperative period and 3, 6 and 12 months post-surgery. Descriptive and inferential analyses were conducted to process the data and compare preoperative health-related quality of life with postoperative values and the Portuguese population's norms. RESULTS: The sample included 506 patients with an average age of 70.6 years. The majority are male (53.6%). The postoperative results show a statistically significant improvement compared to preoperative health-related quality of life in all eight dimensions of the Short Form 36 Health Survey. When contrasted with the general Portuguese population, patients' health-related quality of life is lower in the preoperative period, improving postoperatively and reaching higher than average health-related quality of life levels in some subgroups of patients and in various dimensions. An analysis of the Short Form 6 Health Survey results revealed that the patients undergoing this surgery have a higher level of preoperative problems compared with the general population, but these patients' values approach those of the general population by the end of one year post-surgery. DISCUSSION: This is the first study in Portugal that compares health-related quality of life using a validated scale, before and after the aortic prosthesis implantation surgery. The results demonstrate a significant improvement in all dimensions, which is not consistently observed in other published studies. CONCLUSION: The results confirm that the implantation of aortic prostheses improves patients' health-related quality of life, over time bringing it close to the general population's levels.

Introdução: O objetivo da cirurgia de substituição valvular aórtica é o aumento da esperança de vida e a melhoria da qualidade de vida relacionada com a saúde. Existem vários estudos que evidenciam melhoria da qualidade de vida relacionada com a saúde nestes doentes, mas não aplicados à população portuguesa. O objetivo deste estudo é avaliar a qualidade de vida relacionada com a saúde pós-implantação de prótese aórtica e comparar com a qualidade de vida relacionada com a saúde pré-operatória e da população em geral. Material e Métodos: Foi feito um estudo retrospetivo de doentes eletivos submetidos a implantação de prótese aórtica entre agosto de 2011 e abril de 2016. A qualidade de vida relacionada com a saúde foi avaliada com o Short Form 36 Health Survey Instrument e com o Short Form 6 Health Survey Instrument no pré-operatório e aos 3, 6 e 12 meses pós-cirurgia. Foram realizadas análises descritivas e inferenciais para analisar globalmente a amostra e para comparar a qualidade de vida relacionada com a saúde pré-operatória com a pós-operatória e com as normas da população portuguesa. Resultados: Foram incluídos 506 doentes, com idade média de 70,6 anos, sendo 53,6% do sexo masculino. Os resultados do pós-operatório evidenciam uma melhoria estatisticamente significativa quando comparados com o pré-operatório nas oito dimensões do Short Form 36 Health Survey Instrument. Comparando com a população Portuguesa em geral, a qualidade de vida relacionada com a saúde dos doentes é inferior no pré-operatório, melhorando no pós-operatório, atingindo níveis de qualidade de vida relacionada com a saúde superiores nalguns subgrupos de doentes e em algumas dimensões. Os resultados do Short Form 6 Health Survey Instrument revelam maior intensidade de problemas no pré-operatório quando comparados com a população em geral, aproximando-se dos valores população em geral ao final de um ano pós-cirurgia. Discussão: Este é o primeiro estudo realizado em Portugal que compara a qualidade de vida relacionada com a saúde, usando uma escala validada, antes e depois da cirurgia de implantação de prótese aórtica. Os resultados demonstram a existência de uma melhoria significativa em todas as dimensões, o que não acontece consistentemente noutros estudos publicados. Conclusão: A implantação de prótese aórtica melhora a qualidade de vida relacionada com a saúde dos doentes, permitindo que esta se aproxime dos resultados da população em geral.

Conventional and whitening toothpastes: cytotoxicity, genotoxicity and effect on the enamel surface.

PURPOSE: To evaluate the cytotoxicity and genotoxicity of whitening and common toothpastes, and the surface roughness of tooth enamel submitted to brushing with both toothpastes. METHODS: Samples of whitening toothpastes [Colgate Whitening (CW) and Oral-B Whitening (OBW)] and regular (non-whitening) toothpastes (Colgate and Oral-B) were extracted in culture medium. Gingival human fibroblasts (FMM-1) were placed in contact with different dilutions of culture media that had been previously exposed to such materials, and the cytotoxicity was evaluated using the MTT assay. The genotoxicity was assessed by the micronucleus formation assay in Chinese hamster fibroblasts (V79). The cell survival rate and micronuclei number were assessed before and after exposure to the toothpaste extracts. For the surface roughness evaluation, 20 bovine tooth specimens, divided into four groups according to toothpastes, were submitted to 10,000 brushing cycles. The results were analyzed using the Mann-Whitney U and two-way ANOVA tests (P < 0.05). RESULTS: MTT assay showed that Colgate was significantly less cytotoxic than CW, Oral-B and OBW at all dilutions (P < 0.01). CW was the most cytotoxic toothpaste (P < 0.01). The whitening toothpastes showed the highest numbers of micronuclei compared to the untreated control (UC) (P < 0.01). Colgate and Oral-B toothpastes were not genotoxic compared to UC (P = 0.326). The OBW toothpaste was statistically significantly abrasive to the enamel surface (P < 0.01). The whitening toothpastes and Oral-B were cytotoxic to the cells. The whitening toothpastes were more genotoxic to cells in vitro than the common toothpastes, and genotoxicity was more pronounced in the OBW toothpaste.

Sistema de arco reto para a Ortodontia Lingual: sugestão de técnica / Straight Wire System for Lingual Orthodontics: technique suggestion

Com a demanda por estética que existe atualmente, é cada vez maior o interesse de ortodontistas na técnica lingual, a qual ainda não é utilizada em larga escala, muitas vezes, devido às dificuldades iniciais em se trabalhar com a mesma. Em vista disso, o Prieto Straigth-Wire bracket (PSWb), primeiro bráquete específico para Ortodontia Lingual lançado no Brasil, tem por objetivo levar a Ortodontia Lingual a um maior número de ortodontistas, facilitando seu manejo clínico.

The demand for aesthetics in orthodontics has increased the interest of orthodontists for the lingual technique, which is still not widely used, mostly due to the initial difficulties in working on this technique. Therefore, Prieto’s Straight-Wire Lingual Bracket, the first Lingual Orthodontic specific bracket developed in Brazil, aims at taking Lingual Orthodontics to a larger number of orthodontists, simplifying its clinical use.

Efeito da sobreposição de um modelo de obesidade neuroendócrina experimental e hipertensão arterial sobre a pressão arterial, peso corporal e parâmetros metabólicos e renais de ratos / Effects of the overlapping between an experimental model of neuroendocrine obesity with arterial hypertension under blood pressure, body weight and metabolic and renal parameters in rats

INTRODUÇÃO: A elevação do índice de massa corporaleapresençadesíndromemetabólica se associam com diminuição da função renal e o aparecimento de doença renal terminal. OBJETIVO: Avaliar o efeito da sobreposição de um modelo de obesidade experimental e hipertensão arterial sobre a pressão arterial, peso corporal e parâmetros metabólicos e renais de ratos. MÉTODOS: Foram estudados ratos machos das cepas Wistar e espontaneamente hipertensos (SHR). Os grupos MSG receberam glutamato monossódico no período neonatal (WST + MSG e SHR + MSG). Os animais controles receberam salina no período neonatal (WST e SHR). Após completarem três meses de vida, por 12 semanas foram pesados e tiveram a pressão arterial de cauda aferida semanalmente. A determinação de microalbuminúria foi realizada nas semanas 0, 4, 8 e 12. Ao final do período de acompanhamento, coletou-se sangue para glicemia de jejum, creatinina e perfil lipídico. Os rins foram retirados, corados e o índice de esclerose glomerular foi calculado. RESULTADOS: A administração de MSG produziu maior ganho percentual de peso corporal, elevação da glicemia de jejum e maior grau de lesão glomerular nos ratos WST -MSG e SHR -MSG quando comparados aos seus controles. Houve maior excreção urinária de albumina nos ratos do Grupo SHR + MSG quando comparados aos SHR. Não houve diferença estatística na pressão arterial de cauda, creatinina e parâmetros do metabolismo lipídico. CONCLUSÕES: A associação de obesidade neuroendócrina e a hipertensão arterial promoveram alterações morfológicas e funcionais no glomérulo mais severas do que aquelas observadas nos ratos somente hipertensos.

INTRODUCTION: Increased body mass index and the metabolic syndrome are associated with decreased renal function and the development of end-stage kidney disease. OBJECTIVE: To evaluate the effect of the overlap between an experimental model of obesity and genetic hypertension on the blood pressure, body weight and metabolic and kidney parameters of rats. METHODS: We studied male rats of the Wistar (WST) and spontaneously hypertensive rats (SHR) strains. Monosodium glutamate (MSG) was administered in the neonatal period to both strains, to make up two groups: WST + MSG and SHR + MSG. Animals in the control groups (WST and SHR) received saline. After completing three months of life, a 12-week follow-up period ensued, during which bi-weekly measurements of body weight (BW) and tail-cuff blood pressure (TCBP) were obtained. Microalbuminuria was analyzed at weeks 0, 4, 8 and 12. At the end of the follow-up period, blood was obtained for fasting glucose, plasma creatinine, and lipid profile determinations. The kidneys were removed, stained, and the glomerular sclerosis index was calculated. RESULTS: The administration of MSG produced higher percentage body weight gain, higher fasting blood glucose and a higher degree of glomerular injury in WST-MSG and MSG-SHR rats, compared to their controls. Greater urinary albumin excretion was observed in SHR + MSG rats, when compared to SHR. There was no statistical difference in the TCBP, creatinine, and lipid profile. CONCLUSIONS: The association of neuroendocrine obesity and arterial hypertension promoted morphological and functional changes in the glomerulus. These changes were more severe than those observed in hypertensive-only rats.