Browsing by Author "Ribeiro, A."
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- Caso electroencefalográfico: mioclonias palpebraisPublication . Velon, A.; Xavier, C.; Ribeiro, A.; Chorão, R.ABSTRACT Introduction. Eyelid myoclonia with or without absences may occur in several epileptic conditions, and they are frequently misinterpreted as movement disorders. Case report. A seven-year-old boy was admitted for evaluation of eye blinking that started at age of six. He had never had generalized tonic-clonic or absence seizures. Video-EEG monitoring revealed 3-5 Hz irregular occipital or generalized polyspike and polyspike-wave complexes, precipitated by eye closure. They were accompanied by eye-lid myoclonia. Photic stimulation induced photoparoxysmal response. Brain MRI was normal. He was initially treated with clobazam and then levetiracetam with no response. Valproate was added with control of the symptoms. Conclusion. Eyelid myoclonia without absences are often difficult to classify. Video-EEG may help to clarify these cases.
- Childhood Fructoholism and Fructoholic Liver DiseasePublication . Ribeiro, A.; Igual-Perez, M.; Santos Silva, Ermelinda; Sokal, E.Nonalcoholic fatty liver disease (NAFLD) is an emerging entity, becoming the most prevalent pediatric chronic liver disease. Its broad spectrum of histological findings, comorbidities, and complications, including cirrhosis and liver failure, can occur in childhood, emphasizing the severity of pediatric NAFLD. Current lifestyle and diet modifications have been linked to the increasing prevalence of NAFLD, including the rise of fructose consumption, a monosaccharide present in foods that contain added sugar, such as sugar-sweetened beverages. Excessive fructose consumption is believed to cause addiction like alcohol and other drugs. As such, the new term "fructoholism" refers to the consumption of a substance (fructose) that can cause psychological and physical damage and become a major public health concern, highlighting the seriousness of the excessive consumption of fructose in the pediatric age. Hepatic fructose metabolization leads to hepatic steatosis and progression to fibrosis through mechanisms comparable to alcoholic liver disease, hence the term "fructoholic liver disease." Conclusion: The importance of implementing reliable global strategies, such as education campaigns to promote healthy diet, increasing taxes on foods that contain added sugars, subsidies to promote accessibility to fruit and vegetables, and strict food industry regulation to reduce sugar intake in children and adolescents, cannot be overemphasized.
- Combined Pancreas-Kidney Transplantation: A New Program in Portugal, Results From the First 12 CasesPublication . Martins, L.; Henriques, A.; Dias, L.; Ventura, A.; Seca, R.; Almeida, R.; Dores, J.; Bacelar, C.; Oliveira, F.; Lhamas, A.; Amil, M.; Rua, F.; Coelho, T.; Esteves, S.; Ribeiro, A.; Pereira, R.; Sarmento, A.; Teixeira, M.; Pereira, M.Transplant Proc. 2003 May;35(3):1107-8. Combined pancreas-kidney transplantation: a new program in Portugal, results from the first 12 cases. Martins L, Henriques A, Dias L, Ventura A, Seca R, Almeida R, Dores J, Bacelar C, Oliveira F, Lhamas A, Amil M, Rua F, Coelho T, Esteves S, Ribeiro A, Pereira R, Sarmento A, Teixeira M, Pereira M. Transplantation Department, Hospital Santo António, 4050, Porto, Portugal. lasalete@clix.pt PMID: 12947877 [PubMed - indexed for MEDLINE]
- Fasceíte necrotizante e síndrome de choque tóxico estreptocócico numa criança com varicelaPublication . Cunha, O.; Mota, T.C.; Lopez, M.G.; Santos, E.; Lisboa, L.; Morgado, H.; Lima, F.F.; Castro, C.; Ribeiro, A.Introdução: A fasceíte necrotizante é uma infecção grave dos tecidos moles, geralmente causada por Streptococcus pyogenes. É uma complicação rara da varicela e o diagnóstico numa fase precoce é pouco frequente. Caso clínico: Apresenta-se o caso clínico de uma criança de oito anos de idade com varicela, complicada de fasceíte necrotizante e síndrome de choque tóxico estreptocócico, que necessitou de procedimentos invasivos e de um internamento prolongado em Unidade de Cuidados Intensivos Pediátrica. Conclusão: Os autores alertam para a importância do diagnóstico precoce das potenciais complicações da varicela, nomeadamente da fasceíte necrotizante, pois a precocidade da actuação é determinante para o seu prognóstico. A fasceíte necrotizante deve ser considerada sempre que uma criança com varicela se apresente com eritema, calor e induração da pele e tecidos moles, associado a uma recorrência da febre ou à sua persistência ao quarto dia após o início do exantema. ABSTRACT Introduction: The necrotizing fasciitis, a severe infection of soft tissues, usually caused by Streptococcus pyogenes, is a rare complication of chickenpox; early diagnosis is uncommon. Case report: We present a case of an eight-year-old child with chickenpox, complicated by a necrotizing fasciitis and streptococcal toxic shock, which required invasive procedures and prolonged hospitalization in an Intensive Care Unit Pediatric. Conclusions: The authors emphasize the importance of the potential complications of chickenpox, particularly the necrotizing fasciitis, as an early diagnosis and intervention are crucial for the outcome. The necrotizing fasciitis should be considered whenever a child with chickenpox presents with erythema, warmth, and induration of the skin and soft tissues, associated with a recurrence of fever or its persistence by the fourth day after the rash onset.
- Fatty Liver and Autoimmune Hepatitis: Two Forms of Liver Involvement in LipodystrophiesPublication . Ribeiro, A.; Brandão, J.; Cleto, Esmeralda; Santos, M.; Borges, T.; Santos Silva, E.Introduction: Lipodystrophies are a heterogeneous group of rare diseases (genetic or acquired) characterized by a partial or generalized deficit of adipose tissue, resulting in less energy storage capacity. They are associated with severe endocrine-metabolic complications with significant morbidity and mortality. In the pathogenesis of the acquired forms, immunological disorders may be involved. Case 1: A 13-year-old female was diagnosed with acquired generalized lipodystrophy and observed for suspicion of portal hypertension. She presented with generalized absence of adipose tissue, cervical and axillary acanthosis nigricans, and massive hepatosplenomegaly. Laboratory tests revealed AST 116 IU/L, ALT 238 IU/L, GGT 114 IU/L, HOMA-IR 28.2, triglycerides 491 mg/L, and leptin < 0.05 ng/mL. Upper gastrointestinal endoscopy saw no signs of portal hypertension. Hepatic histology showed macrovesicular fatty infiltration (60% of hepatocytes) and advanced fibrosis/cirrhosis. Her clinical condition worsened progressively to diabetes requiring treatment with subcutaneous insulin and hepatopulmonary syndrome. Case 2: A 15-year-old female, diagnosed with acquired partial lipodystrophy, Parkinson syndrome, autoimmune thyroiditis, and autoimmune thrombocytopenia was observed for hypertransaminasemia since the age of 8 years. She had absence of subcutaneous adipose tissue in the upper and lower limbs and ataxia. Laboratory tests showed AST 461 IU/L, ALT 921 IU/L, GGT 145 IU/L, HOMA-IR 32.6, triglycerides 298 mg/dL, normal leptin levels, platelets 84,000/μL, IgG 1,894 mg/dL, positive anti-LKM and anti-LC-1. Hepatic histology was suggestive of autoimmune hepatitis, without steatosis. She progressed favorably under metformin and immunosuppressive treatment. Conclusion: Early recognition and adequate characterization of liver disease in lipodystrophies is essential for a correct treatment approach. In acquired generalized lipodystrophy, the severe endocrine-metabolic disorder, which leads to steatohepatitis with cirrhotic progression, may benefit from recombinant leptin treatment.
- Imaging of pancreas transplantation and its complicationsPublication . França, M.; Certo, M.; Martins, L.; Varzim, P.; Teixeira, M.; Castro-Henriques, A.; Ribeiro, A.; Alves, F.Abstract Pancreas transplantation is an effective treatment for type 1 diabetes mellitus and is being increasingly performed worldwide. Early recognition of graft-related complications is fundamental for graft survival; thus, radiologists must be aware of the transplantation technique, pancreas-graft imaging and postoperative complications. We present normal pancreas-graft imaging appearances and the imaging features of postoperative complications.
- Qualidade de vida da pessoa com traqueostomia na área do Grande PortoPublication . Gonçalves, G.; Santos, C.; Ribeiro, A.
- Síndrome do bebé abanado – experiência de 10 anos de um Serviço de Cuidados Intensivos PediátricosPublication . Lourenço, L.; Silva, M.; Lisboa, L.; Mota, T.C.; Ribeiro, A.Introdução: O Síndrome do Bebé Abanado é uma das causas de lesão não acidental mais difíceis de diagnosticar. Objetivos: Caracterizar os doentes internados no Serviço de Cuidados Intensivos Pediátricos (SCIP) com Síndrome do Bebé Abanado. Material e Métodos: Estudo retrospetivo dos doentes internados no SCIP do Centro Hospitalar de São João com o diagnóstico de Síndrome de Bebé Abanado de 1 de Janeiro de 1999 a 31 de Dezembro de 2009. Resultados: Foram internadas oito crianças com diagnóstico de Síndrome do Bebé Abanado. A média de idades foi de 4,1 meses e cinco doentes eram do sexo masculino. Um caso apresentava história de traumatismo. O motivo de recurso aos cuidados de saúde mais frequente foram as crises convulsivas com paragem respiratória/cardiorrespiratória (50%). Verificou-se hemorragias retinianas bilaterais em 6 doentes (75%). Em todos a tomografia a axial computorizada cerebral mostrou hematomas subdurais. Sete doentes (87,5%) necessitaram de ventilação mecânica e cinco (62,5%) de suporte inotrópico. O tempo médio de internamento no SCIP foi de 5,25 dias (1-11 dias). Verificou-se um óbito. Verificou-se uma hemiparésia transitória num doente. Três casos apresentaram uma epilepsia de novo e défices na acuidade visual ou auditiva e num caso houve perda das aquisições prévias com um atraso moderado a severo do desenvolvimento psicomotor. Conclusão: O Síndrome do Bebé Abanado resultou, na nossa série, em elevada morbilidade com a presença de sequelas a longo prazo em cinco doentes (62,5%) compatíveis com os dados publicados em outras séries. A mortalidade encontrada foi inferior à apresentada em estudos anteriores (12,5%). A presença de história traumática só foi referida num caso e, apesar de todos terem apresentado hematomas subdurais, 25% dos casos não tinham hemorragias retinianas. A apresentação clínica foi muito grave na grande maioria dos casos, sendo fundamental um elevado grau de suspeição.