SESARAM - Pedi - Artigos
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- Issacs Syndrome in a Pediatric Patient and Voltage-Gated Potassium Channels AntibodiesPublication . Sousa, Paulo Rego de; Vasconcellos, Rui; Forno, Andreia; Rodrigues, Alexandra
- Oral language of school-aged children born pretermaturely: a population-based analysis from Madeira Island, PortugalPublication . Santos, Maria Emilia; Marques, AnaPremature birth and low birth weight are very important factors in neurodevelopment. Current research in this population focuses on children born prematurely, with no underlying complications in the post-natal period, who are likely to develop specific disorders with their language development and consequently with their learning capabilities too. This study aims to analyse the oral language skills of prematurely born children in comparison to their school-aged peers. The children were assessed in the respective schools, 27 preterm children (16 under 32 weeks and 11 with 32 or more weeks of gestation) and 49 term paired by gender, age, and school year. Tests including simple and complex structures for assessing semantics, morphosyntax, and phonology were used, as well as a test of verbal memory. Preterm born children, regardless of their prematurity grade, showed significantly lower results than their peers, and more than a half of them, 52%, presented low scores in all language tests simultaneously, showing an important language deficit. In contrast, in the term born children group only 14% showed low scores simultaneously in all tests. Verbal memory ability proved to be lower than that of their term peers, regardless of the gestational age and birth weight of preterm children. As a result of this analysis we consider that the evaluation of the linguistic development of these children, even in cases of moderate to late prematurity, should be monitored in order to identify earlier the existence of deficits and prevent psychosocial and learning problems.
- Dengue em Portugal – Experiência da Região Autónoma da madeiraPublication . Castro, Leonor; Marçal, Filipa; Goncalves, Jenny F; Oliveira, Joana; Miranda, Victor; Freitas, Cristina; Barros, Andreia; Freitas, PedroIntroduction: Dengue is a self-limited viral disease, transmitted by infected Aedes mosquitoes. The introduction of the vector to the island of Madeira raised the menace of an epidemic outbreak. Despite the implementation of vector control measures, the first dengue outbreak was reported eight years after the first detection of the vector in the region. The objective of this study was to characterize cases in paediatric ages during the first outbreak of dengue in Madeira. Methods: We performed a retrospective, observational, descriptive study including children aged 0 to 14 years with laboratory confirmed dengue. Clinical, demographic and laboratory variables were characterized, followed by statistical analysis.Results: One hundred and eighty-two cases were confirmed by laboratory methods, predominantly male, mean age 9.6 years. The incidence was 413.5/100 000 population, the peak incidence being observed in November 2012. The most frequently described symptoms were fever (98.3%), headache (75.2%), myalgia (66.5%) and rash (51.6%). Bleeding was identified in 9.9% of cases. Of the paediatric cases,15.9% were admitted, with a mean duration of 3.8 days. No deaths were reported. Only the DEN-1 serotype was identified. Conclusions: The high density of the vector, together with susceptible hosts, may have triggered the outbreak following the introduction of the virus in the region. Given the possibility of a new outbreak, and since immunity is specific to the infecting serotype, vector management strategies and individual protection measures must be sustained.
- Wernicke encephalopathy in childrenPublication . Forno, Andreia; Cunha, Bruno; Luís, Catarina; Castro, Ana; Moniz, Marta; Escobar, Carlos; Lamy, Sérgio; Pereira, Andreia; Martins, Ana; Conceição, CarlaIntroduction : Wernicke Encephalopathy (WE) is an acute neurological disorder caused by thiamine deficiency. Usually associated with alcoholism in adults, WE can also occur in children with other etiologies.1 The classic triad of symptoms is present in a small number of patients. As a medical emergency, once suspected, high doses thiamine treatment should be started in order to decrease comorbidities and improve prognosis.
- Copper Toxicity Associated With an ATP7A-Related Complex PhenotypePublication . Natera-de Benito, Daniel; Sola, Abel; Sousa, Paulo Rego; Boronat, Susana; Expósito-Escudero, Jessica; Carrera-García, Laura; Ortez, Carlos; Jou, Cristina; Muchart, Jordi; Rebollo, Monica; Armstrong, Judith; Colomer, Jaume; Garcia-Cazorla, Àngels; Hoenicka, Janet; Palau, Francesc; Nascimento, AndresThe ATP7A gene encodes a copper transporter whose mutations cause Menkes disease, occipital horn syndrome (OHS), and, less frequently, ATP7A-related distal hereditary motor neuropathy (dHMN). Here we describe a family with OHS caused by a novel mutation in the ATP7A gene, including a patient with a comorbid dHMN that worsened markedly after being treated with copper histidinate.
- Spontaneous spinal epidural haematoma due to arteriovenous malformation in a childPublication . Cabral, A. J.; Barros, A.; Aveiro, C.; Vasconcelos, R.Spontaneous spinal epidural haematoma (SSEH) is a rare clinical entity, especially in infants, in whom only a few cases have been reported. In a paediatric emergency setting, SSEH should be considered as part of the differential diagnosis for acute extremity weakness and paraesthesia. Epidural vascular malformations are often suspected in these cases but have rarely been demonstrated. The authors report herein a case of SSEH in a 9-year-old boy arising from an epidural vascular malformation. He initially presented with sudden intense cervicodorsal pain followed by hypotonic lower extremities and progressive motor weakness, with no sensory change. The MRI showed an acute extradural haematoma extending from C7 to T4 with compression of the spinal cord. After submission to decompression surgery, he presented full recovery in 1 month. The histopathological analysis revealed a vascular malformation.
- Atenção primária à saúde no Rio de Janeiro – o que há de diferente?Publication . Forno, Andreia; Hakme, Pedro
- A evolução do refluxo vesico-ureteral com diagnóstico perinatalPublication . Leitão, Henrique; Correia, António Jorge; Gomes, Clara; Oliveira, Oliveira; Rolo, Vitor; Mimoso, Gabriela; Ramos, ConceiçãoResumo.Avaliar a evolução e tratamento do refluxo vesico--ureteral congénito (RVU) em recém-nascido ou lactente com diagnóstico pré-natal de anomalia nefro-urológica. Métodos. Estudo de coortes históricas de crianças com o diagnóstico perinatal de RVU, nascidas numa maternidade de apoio perinatal diferenciado da Região Centro do País, entre 1993 e 2002, e posteriormente acompanhadas no hospital pediátrico de referência da mesma Região. Dividimos as crianças em duas coortes, aquelas que efectuaram tratamento cirúrgico e aquelas em que foi decidido manter vigilância. Para cada coorte (se aplicável) avaliámos: sexo, o tipo e grau de RVU, tipo de cirurgia, complicações, frequência e tempo de evolução até à cura espontânea. Na coorte das crianças em vigilância comparámos a proporção de crianças que mantiveram RVU com aquelas em que se verificou cura espontânea, relativamente ao sexo e ao grau de RVU. Resultados. Oitenta e duas crianças cumpriam os critérios de inclusão. O RVU congénito foi mais frequente no sexo masculino (77%) e era maioritariamente unilateral (65%). O tratamento cirúrgico foi efectuado em 35% dos casos, sobretudo nas crianças com RVU bilateral, com unidades refluxivas (UR) de grau elevado – IV ou V - (80% vs. 12% na coorte de crianças em vigilância) e em crianças com lesão/malformação renal (ipsilateral ou contralateral) e/ou assimetria funcional renal (58% vs 24% na coorte de crianças em vigilância). Neste último grupo, verificámos uma frequência de resolução do RVU de 72% (76% no subgrupo que foi acompanhado pelos menos 48 meses). O período entre os 24 e os 36 meses foi aquele em que ocorreu o maior número de resolução de casos. Não encontrámos diferenças significativas entre sexo e grau de RVU no que respeita à cura espontânea do RVU. Conclusões. O refluxo vesico-ureteral tende a resolver-se nos primeiros anos de vida, parecendo a profilaxia e vigilância clínica medidas suficientes e seguras no casos de refluxo de baixo grau e, provavelmente, também nos casos mais graves.
- Paediatric acute basilar thrombosis successfully treated with intravenous alteplasePublication . Rego de Sousa, Paulo; Vasconcellos, RuiAcute ischaemic stroke has significant attendant morbidity and is one of the top ten causes of childhood death. It requires prompt investigation and management, however little is known about the safety and efficacy of acute thrombolytic therapies in childhood arterial ischaemic stroke. The authors report a case of a 13-year-old girl with an acute basilar thrombosis, successfully treated with intravenous recombinant tissue plasminogen activator and discuss the management of paediatric arterial ischaemic stroke.
- Idiopathic congenital chylothorax treated with octreotidePublication . Sousa, P. R.; Leitao, H.; Camacho, M. C.; Nunes, J. L.Idiopathic congenital chylothorax is defined as an abnormal accumulation of lymphatic fluid within the pleural space and is a relatively rare condition. It is a cause of progressive respiratory distress with nutritional and immunological consequences. Treatment of congenital chylothorax has been conservative management and cases unresponsive usually require surgery. We report a case of idiopathic congenital chylothorax treated with octreotide (a somatostatin analogue), avoiding surgery after failed conservative medical treatment. The patient promptly improved after initiation of endovenous octreotide treatment (10 µg/kg/h) with no observed side effects.
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