Browsing by Author "Cabral, J"
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- Autoimmune Pancreatitis with Associated Ulcerative Colitis in a TeenagerPublication . Gouveia, CI; Oliveira, L; Campos, AP; Cabral, JAutoimmune pancreatitis (AIP) is a rare entity that is extremely uncommon in children. Its diagnosis is also a clinical challenge. This form of chronic pancreatitis often presents itself with obstructive jaundice and/or a pancreatic mass and it is sometimes misdiagnosed as pancreatic cancer. We describe the case of a 13-year-old boy with obstructive jaundice and a 4 cm mass in the head of the pancreas that was diagnosed as AIP with associated ulcerative colitis.
- Comparison of Methods and Co-Registration Maps of EEG and fMRI in Occipital Lobe EpilepsyPublication . Forjaz Secca, M; Leal, A; Cabral, J; Fernandes, HClinically childhood occipital lobe epilepsy (OLE) manifests itself with distinct syndromes. The traditional EEG recordings have not been able to overcome the difficulty in correlating the ictal clinical symptoms to the onset in particular areas of the occipital lobes. To understand these syndromes it is important to map with more precision the epileptogenic cortical regions in OLE. Experimentally, we studied three idiopathic childhood OLE patients with EEG source analysis and with the simultaneous acquisition of EEG and fMRI, to map the BOLD effect associated with EEG spikes. The spatial overlap between the EEG and BOLD results was not very good, but the fMRI suggested localizations more consistent with the ictal clinical manifestations of each type of epileptic syndrome. Since our first results show that by associating the BOLD effect with interictal spikes the epileptogenic areas are mapped to localizations different from those calculated from EEG sources and that by using different EEG/fMRI processing methods our results differ to some extent, it is very important to compare the different methods of processing the localization of activation and develop a good methodology for obtaining co-registration maps of high resolution EEG with BOLD localizations.
- Doença de Crohn Complicada em Idade PediátricaPublication . Casella, P; Alves, F; Cabral, JApresentamos o caso clínico de uma criança de 15 anos portadora de doença de Crohn complicada(fistula ileo-sigmoideia, abcesso da parede e estenoses ileais múltiplas) em que a abordagem multidisciplinar com terapêutica combinada de imunosupressão, cirurgia de derivação, e programa de nutrição assistida (entérica e parentérica), permitiu a correcção cirúrgica electiva das manifestações ileo-cólicas da doença,utilizando estricturoplastias múltiplas e resecção limitada. Esta abordagem, que constitui o padrão nos doentes portadores de doença de Crohn na idade adulta e representa uma inovação em doentes pediátricos, permitiu o restabelecimento do trânsito intestinal com conservação do máximo possível de área de absorção, obtendo com um mínimo de morbilidade, uma excelente qualidade de vida.
- Doença Inflamatória Intestinal Pediátrica. Uma Patologia em Crescendo?Publication . Fernandes, A; Bacalhau, S; Cabral, JIntrodução: A Doença de Crohn (DC), Colite Ulcerosa (CU) e Colite Indeterminada (CI), habitualmente designadas por Doença Inflamatória Intestinal (DII), representam um grupo heterogéneo de patologias crónicas, de etiologia desconhecida e evolução variável, podendo manifestar-se, em idade pediátrica, em cerca de 25 a 30% dos casos. Estudos epidemiológicos internacionais comprovam o aumento exponencial da sua incidência nos países industrializados, em particular da DC, nos últimos 50 anos. Objectivos: Caracterização da população pediátrica com o diagnóstico de DII, seguida na consulta de Gastrenterologia Infantil do Hospital de Dona Estefânia (HDE). Material e Métodos: Estudo descritivo e retrospectivo, mediante consulta de processos clínicos, de doentes com o diagnóstico de DII, entre 1987 e 2009 (23 anos). Utilizaram-se critérios clínicos, radiológicos e histológicos para a definição de DII. Foram estudadas as seguintes variáveis: caracterização da DII, sexo, antecedentes familiares, idade à data do diagnóstico, intervalo de tempo entre o início da sintomatologia e respectivo diagnóstico e apresentação clínica. Foram comparados quatro intervalos de tempo: 1987-1992, 1993-1998, 1999-2004 e 2005-2009. Resultados: Foram incluídas 100 crianças, 51 pertencentes ao sexo feminino, das quais 59% correspondem a DC, 38% a CU e 3% a CI. Verificou-se a presença de antecedentes familiares de DII em sete casos, não se verificando diferença significativa de sexo entre a CU e a DC. No período compreendido entre 2005 e 2009 foi registado o maior número de novos casos (55 no total; média: 11 casos/ano) e entre 1987 e 1992 registou-se o menor número de novos casos (9; 1,5 casos/ano). O intervalo de tempo que decorreu entre o início dos sintomas e o diagnóstico de DII variou entre nove meses (1987-1992) e quatro meses (2005-2009). A idade no momento do diagnóstico variou entre os 14 meses e os 17 anos, com um valor médio de 10,5 anos. A sintomatologia inaugural mais frequente foi a presença de dor abdominal, a diarreia e a hematoquézia. Conclusão: A DII engloba um grupo heterogéneo de patologias, nem sempre fáceis de diagnosticar ou classificar, dada a ausência de critérios de diagnóstico uniformes. Os resultados apresentados mostram o aumento do número de novos casos, na consulta de Gastrenterologia do HDE, nas últimas duas décadas, não se verificando diferença no que diz respeito ao sexo. O tempo que decorreu entre o início dos sintomas e o diagnóstico diminuiu ao longo dos anos, tendo permanecido inalterada a idade no momento do diagnóstico e a apresentação clínica.
- Helicobacter Pylori Infection in Pediatric Patients Living in Europe: Results of the EuroPedHP Registry 2013 to 2016Publication . Kori, M; Le Thi, TG; Werkstetter, K; Sustmann, A; Bontems, P; Lopes, AI; Oleastro, M; Iwanczak, B; Kalach, N; Misak, Z; Cabral, J; Homan, M; Cilleruelo Pascual, ML; Pehlivanoglu, E; Casswall, T; Urruzuno, P; Martinez Gomez, MJ; Papadopoulou, A; Roma, E; Dolinsek, J; Rogalidou, M; Urbonas, V; Chong, S; Kindermann, A; Miele, E; Rea, F; Cseh, A; Koletzko, SObjectives: The aim of the study was to assess clinical presentation, endoscopic findings, antibiotic susceptibility and treatment success of Helicobacter pylori (H. pylori) infected pediatric patients. Methods: Between 2013 and 2016, 23 pediatric hospitals from 17 countries prospectively submitted data on consecutive H. pylori-infected (culture positive) patients to the EuroPedHP-Registry. Results: Of 1333 patients recruited (55.1% girls, median age 12.6 years), 1168 (87.6%) were therapy naïve (group A) and 165 (12.4%) had failed treatment (group B). Patients resided in North/Western (29.6%), Southern (34.1%) and Eastern Europe (23.0%), or Israel/Turkey (13.4%). Main indications for endoscopy were abdominal pain or dyspepsia (81.2%, 1078/1328). Antral nodularity was reported in 77.8% (1031/1326) of patients, gastric or duodenal ulcers and erosions in 5.1% and 12.8%, respectively. Primary resistance to clarithromycin (CLA) and metronidazole (MET) occurred in 25% and 21%, respectively, and increased after failed therapy. Bacterial strains were fully susceptible in 60.5% of group A, but in only 27.4% of group B. Primary CLA resistance was higher in Southern and Eastern Europe (adjusted odds ratio [ORadj] = 3.44, 95% confidence interval [CI] 2.22-5.32, P < 0.001 and 2.62, 95% CI: 1.63-4.22, P < 0.001, respectively) compared with Northern/Western Europe. Children born outside Europe showed higher primary MET resistance (ORadj = 3.81, 95% CI: 2.25-6.45, P < 0.001). Treatment success in group A reached only 79.8% (568/712) with 7 to 14 days triple therapy tailored to antibiotic susceptibility. Conclusions: Peptic ulcers are rare in dyspeptic H. pylori-infected children. Primary resistance to CLA and MET is markedly dependent on geographical regions of birth and residence. The ongoing survey will show whether implementation of the updated ESPGHAN/NASPGHAN guidelines will improve the eradication success.
- Impacto Alimentar Esofágico, um Dilema DiagnósticoPublication . Rúbio, C; Furtado, F; Cunha, F; Queirós, G; Oliveira, L; Cabral, JO impacto alimentar esofágico é uma situação que na maioria dos casos é secundária a patologia esofágica congénita ou adquirida, podendo constituir a primeira manifestação da doença. Descreve-se um rapaz, 11 anos, saudável, com sialorreia e dor retrosternal que surgiram subitamente durante o jantar. Foi submetido a endoscopia digestiva alta, que revelou um fragmento de carne impactado no terço distal do esófago (que foi extraído) e estrias longitudinais na mucosa. A análise histológica das biópsias esofágicas foi compatível com eosinofilia esofágica. Iniciou terapêutica com inibidor da bomba de protões com melhoria clínica e histológica. O diagnóstico diferencial das principais causas de impacto alimentar associadas a eosinofilia esofágica, nomeadamente esofagite eosinofílica, doença do refluxo gastroesofágico e eosinofilia esofágica respondedora a inibidor da bomba de protões, constitui um desafio clínico.
- Intracoronary Delivery of Human Mesenchymal/Stromal Stem Cells: Insights from Coronary Microcirculation Invasive Assessment in a Swine ModelPublication . Fiarresga, A; Mata, M; Cavaco-Gonçalves, S; Selas, M; Simões, I; Oliveira, E; Carrapiço, B; Cardim, N; Cabral, J; Cruz Ferreira, R; Silva, CBACKGROUND: Mesenchymal stem/stromal cells have unique properties favorable to their use in clinical practice and have been studied for cardiac repair. However, these cells are larger than coronary microvessels and there is controversy about the risk of embolization and microinfarctions, which could jeopardize the safety and efficacy of intracoronary route for their delivery. The index of microcirculatory resistance (IMR) is an invasive method for quantitatively assessing the coronary microcirculation status. OBJECTIVES: To examine heart microcirculation after intracoronary injection of mesenchymal stem/stromal cells with the index of microcirculatory resistance. METHODS: Healthy swine were randomized to receive by intracoronary route either 30x106 MSC or the same solution with no cells (1% human albumin/PBS) (placebo). Blinded operators took coronary pressure and flow measurements, prior to intracoronary infusion and at 5 and 30 minutes post-delivery. Coronary flow reserve (CFR) and the IMR were compared between groups. RESULTS: CFR and IMR were done with a variance within the 3 transit time measurements of 6% at rest and 11% at maximal hyperemia. After intracoronary infusion there were no significant differences in CFR. The IMR was significantly higher in MSC-injected animals (at 30 minutes, 14.2U vs. 8.8U, p = 0.02) and intragroup analysis showed a significant increase of 112% from baseline to 30 minutes after cell infusion, although no electrocardiographic changes or clinical deterioration were noted. CONCLUSION: Overall, this study provides definitive evidence of microcirculatory disruption upon intracoronary administration of mesenchymal stem/stromal cells, in a large animal model closely resembling human cardiac physiology, function and anatomy.
- Linfoma de Burkitt Gastrintestinal da CriançaPublication . Silva Couto, A; Cabral, JO Linfoma de Burkitt (LB) gastrintestinal é um subtipo de linfoma não Hodgkin (LNH) que surge com maior frequência no íleon e no cego, sobretudo na sua forma esporádica e na criança. O LB do estômago e do duodeno é particularmente raro e pode manifestar-se de diversas formas. Os autores apresentam dois casos clínicos de LB do estômago e do duodeno, em idade pediátrica.
- Optimization of fMRI Processing Parameters for Simutaneous Acquisition of EEG/fMRI in Focal EpilepsyPublication . Forjaz Secca, M; Fernandes, H; Cabral, J; Leal, AIn the context of focal epilepsy, the simultaneous combination of electroencephalography (EEG) and functional magnetic resonance imaging (fMRI) holds a great promise as a technique by which the hemodynamic correlates of interictal spikes detected on scalp EEG can be identified. The fact that traditional EEG recordings have not been able to overcome the difficulty in correlating the ictal clinical symptoms to the onset in particular areas of the lobes, brings the need of mapping with more precision the epileptogenic cortical regions. On the other hand, fMRI suggested localizations more consistent with the ictal clinical manifestations detected. This study was developed in order to improve the knowledge about the way parameters involved in the physical and mathematical data, produced by the EEG/fMRI technique processing, would influence the final results. The evaluation of the accuracy was made by comparing the BOLD results with: the high resolution EEG maps; the malformative lesions detected in the T1 weighted MR images; and the anatomical localizations of the diagnosed symptomatology of each studied patient. The optimization of the set of parameters used, will provide an important contribution to the diagnosis of epileptogenic focuses, in patients included on an epilepsy surgery evaluation program. The results obtained allowed us to conclude that: by associating the BOLD effect with interictal spikes, the epileptogenic areas are mapped to localizations different from those obtained by the EEG maps representing the electrical potential distribution across the scalp (EEG); there is an important and solid bond between the variation of particular parameters (manipulated during the fMRI data processing) and the optimization of the final results, from which smoothing, deleted volumes, HRF (used to convolve with the activation design), and the shape of the Gamma function can be certainly emphasized.
- Pancreatite Aguda como Manifestação de Linfoma de Burkitt: em Idade PediátricaPublication . Silva, F; Paiva, M; Tavares, A; Lacerda, A; Pereira, G; Marques, A; Barata, D; Cabral, JA pancreatite é uma patologia pouco frequente em Pediatria, com etiologia mais variada que no adulto, sendo as causas tumorais particularmente raras. Os autores apresentam o caso de um adolescente de 13 anos, previamente saudável, internado na Unidade de Cuidados Intensivos com os diagnósticos de pancreatite aguda, derrame pleural bilateral e ascite. Na avaliação imagiológica apareceram imagens sugestivas de processo infiltrativo da parede gástrica. A endoscopia digestiva alta mostrou tumor infiltrativo da mucosa gástrica e bulbo duodenal com úlcera gigante, com biópsias compatíveis com Linfoma de Burkitt, confirmado por citologia e citometria do líquido ascítico. Durante a evolução houve ainda necessidade de colocação de endoprótese biliar por colestase obstrutiva de agravamento progressivo. Após estadiamento (estádio III), iniciou o protocolo FAB LMB 96 obtendo remissão completa. A pancreatite aguda e a colestase são formas de apresentação raras de linfoma neste grupo etário.