Síndrome de Rubinstein-Taybi. Um Caso Clínico de Apresentação no Período Neonatal

Os autores descrevem um caso de Síndrome de Rubinstein-Taybi em que as características faciais e os polegares alargados permitiram o diagnóstico na primeira semana de vida. Salientam na evolução as infecções respiratórias de repetição, o atraso psicomotor e estaturo ponderal. Discutem as prováveis formas de transmissão deste síndrome.

Notificação de incidentes e eventos adversos em neonatologia : adaptação de um sistema de notificação numa unidade de cuidados intermédios neonatal

RESUMO - A segurança do doente é um tema que tem sido amplamente estudado por todo o mundo. Com o desenvolvimento do conhecimento, das técnicas e o advento das learning organizations é possível detectar as áreas onde existe potencial risco, conhecer o número de incidentes de forma sistemática, promover a evolução das técnicas nas áreas mais urgentes, determinar o impacto de todos os incidentes e eventos adversos, aprender com eles e promover modificações nas organizações. A neonatologia não foi excepção, pelo que se pretende a criação e validação de um sistema de notificação de eventos adversos e de incidentes, anónimo e não punitivo, adaptado a uma Unidade de Cuidados Intermédios Neonatal. O delineamento do estudo passou pela revisão bibliográfica para a construção de um sistema e posterior análise do mesmo por um painel de especialistas, para a selecção e consenso de itens que integraram o modelo. Por fim este sistema foi sujeito a um pré-teste. Com a aplicação da Técnica de Grupo Nominal constatou-se que a confidencialidade dos dados é um tema muito sensível aos profissionais. Na aplicação do pré-teste as categorias de incidentes notificados relacionam-se com medicação, ventilação e identificação. Assim sendo, este sistema detém validade interna, no entanto com a aplicação do pré-teste verificou-se que este perde validade externa, pelo que os resultados apresentados neste projecto de investigação não podem ser generalizados. A notificação é uma área para a qual os profissionais estão sensibilizados, no entanto, ainda encontra muitos entraves à sua implementação e consequentemente à colheita de dados.

Effects of water deprivation on renal hydroelectrolytic excretion in chronically Trypanosoma cruzi-infected rats

The effect of an 8 hour-period of water deprivation on fluid and electrolyte renal excretion was investigated in male Wistar rats infected with the strain São Felipe (12SF) of Trypanosoma cruzi, in comparison with age and sex matched non-infected controls. The median percent reductions in the urinary flow (-40% v -63%) and excretion ofsodium (-57% v-79%) were smaller in chagasic than in control rats, respectively. So, chagasic rats excreted more than controls. On the other hand, the median percent decrement in the clearance of creatinine was higher in chagasic (-51%) than in controls (-39%). Thus, chagasic rats showed some disturbed renal hydroelectrolytic responses to water deprivation, expressed by smaller conservation, or higher excretion of water and sodium in association with smaller glomerularfiltration rate. This fact denoted an elevation in the fractional excretion of sodium and water.

Serial heart rate changes in rats inoculated by conjunctival instillation of Trypanosoma cruzi obtained from bug faeces

The cardiac effects of experimentally induced myocarditis, when the parasite is obtained from mouse blood, are well known. However, the consequences of the infection when the parasites are obtained from bug faeces are less well defined. In the present investigation, we have used the "Y" strain of Trypanosoma cruzi, which was maintained in Rhodnius prolixus by repeated passages in mice. The faeces of 30 infected bugs were collected, the number of parasites counted and 4,000 parasites inoculated by the conjunctival route in 60 rats. Twenty-nine other rats received faeces from noninfected bugs (sham-inoculated controls) and 40 were used as normal controls. The heart rate of the three groups of animals was recorded under general anesthesia with ether. The heart rate, at day 0 pre-inoculation, was similar in the three groups of animals (Controls: 379 ± 27 beats/min Mean ± SD; Sham-inoculated: 366 ± 31; Infected: 351 ± 29) (p> 0.05). In the infected animals, the mean heart rate began to increase significantly by day 12 following infection (375 ± 31), reaching the highest values between days 18 (390 ± 33) and 21 (403 ± 33) and returned to baseline by day 30 (359 ± 28) (p< 0.05). The heart rate changes were statistically different from those observed in the sham-inoculated controls and in the control animals. Therefore, these heart rate changes were provoked by the Trypanosoma cruzi-induced infection. Thus, it appears that irrespective of the source of the parasite and route of inoculation Trypanosoma cruziacute infection provokes a transient sinus tachycardia.

Pathogenesis of hepatic septal fibrosis associated with Capillaria hepatica infection of rats

Septal fibrosis is a common form of hepatic fibrosis, but its etiology and pathogenesis are poorly understood. Rats infected with the helminth Capillaria hepatica constitute a good experimental model of such fibrosis. To investigate the pathogenetic contribution of the several parasitic factors involved, the following procedures were performed in rats: a) regarding the role of eggs, these were isolated and injected either into the peritoneal cavity or directly into the liver parenchyma; b) for worms alone, 15-day-old infection was treated with mebendazole, killing the parasites before oviposition started; c) for both eggs and worms, rats at the 30th day of infection were treated with either mebendazole or ivermectin. Eggs only originated focal fibrosis from cicatricial granulomas, but no septal fibrosis. Worms alone induced a mild degree of perifocal septal fibrosis. Systematized septal fibrosis of the liver, similar to that observed in the infected controls, occurred only in the rats treated with mebendazole or ivermectin, with dead worms and immature eggs in their livers. Thus, future search for fibrogenic factors associated with C. hepatica infection in rats should consider lesions with both eggs and worms.

Production of septal fibrosis of the liver by means of foreign protein injections into rats

Similarities and differences in antigenic humoral responses and electrophoretic patterns between Capillaria hepatica and pig-serum were investigated as a contribution to the understanding of hepatic fibrosis induced by the parenteral administration of foreign proteins. Only two out of 10 rats receiving repeated intraperitoneal injections of an extract of Capillaria hepatica-infected mouse liver presented septal hepatic fibrosis (20%). Under the same experimental conditions, 4 out of 9 rats (44.4%) developed septal fibrosis following whole pig-serum administration. Injections of normal mouse liver extracts did not result in hepatic fibrosis. Since a 100% septal fibrosis rate is observed in experimentally Capillaria hepatica-infected rats, it appeared that Capillaria hepatica products continuously released from inside the liver creates a much more effective fibrosis inducing mechanism than the parenteral administration of such factors. Thus, repeated peritoneal administration of a foreign protein to rats would not reveal the full fibrogenic potential it may have under natural conditions.

Capillaria hepatica-induced hepatic fibrosis in rats: paradoxical effect of repeated infections

Multiple exposures to parasitic agents are considered an important factor in the genesis of the most severe forms of the diseases they cause. Capillaria hepatica-induced septal fibrosis of the liver in rats usually runs without signs of portal hypertension or hepatic failure. After determining the hepatic profile of 15 animals during the course of a single infection, we submitted 20 rats to multiple Capillaria hepatica infections to determine whether repeated exposures would augment fibrosis production, transforming septal hepatic fibrosis into a true cirrhosis. Ten single-infection rats served as controls. A total of 5 exposures, with 45-day intervals, were made. Histological changes were followed by means of surgical liver biopsies, collected prior to infection and to each re-infection. Functional changes were minimal and transient. Although a slight recrudescence of fibrosis was observed after the first two re-infections and when the single-infected control group was re-infected at the end of the experiment, subsequent re-infections failed to increase the amount of fibrosis. On the contrary, there occurred quantitative and qualitative evidence of collagen degradation and suppression of parasite development. These paradoxical results are in keeping with the hypothesis that a complex immunological modulation participates in the mechanism of hepatic fibrosis induced by Capillaria hepatica infection in rats.

Role of partial hepatectomy on Capillaria hepatica-induced hepatic fibrosis in rats

It is known that hepatic fibrosis may regress following partial hepatectomy, since the hepatic parenchyma regenerates very rapidly, but not the excess of fibrous tissue. The present study evaluated this hypothesis by observing the behavior of systematized septal fibrosis induced by either 30 or 90-day-old Capillaria hepatica infection, in rats subjected to partial hepatectomy. The results revealed that the morphology of the fibrosis was unaffected, but its relative quantity within the microscope field appeared significantly decreased, as a consequence of the increased liver tissue mass following regeneration.

Candidemia neonatal, em hospital público do Mato Grosso do Sul

O objetivo de nosso estudo foi realizar tipagem molecular de 25 amostras clínicas de Candida spp, isoladas de crianças com candidemia, internadas na unidade de terapia intensiva neonatal de um Hospital Universitário entre 1998 a 2006. Dados demográficos e clínicos foram obtidos de prontuários para conhecimento dos aspectos clínicos e epidemiológicos. Identificação das leveduras foi feita por método convencional e a susceptibilidade antifúngica por método de microdiluição. O perfil genético foi determinado pela técnica de RAPD-PCR. Candida albicans (11; 44%) e Candida parapsilosis (10; 40%) foram as mais isoladas. Dezessete (68%) dos recém-nascidos tinham peso inferior a 1.500g. Prematuridade (92%), uso de cateter venoso central (100%), foram as condições de risco mais associados. Dezenove (76%) pacientes foram a óbito. Apenas uma cepa de Candida parapsilosis, mostrou ser sensível dose dependente ao fluconazol. Na análise molecular, foram observados 11 padrões genéticos distintos. Somente em dois casos foi observada relação epidemiológica, sugerindo mesma fonte de infecção.

Triagem neonatal para infecção chagásica congênita: aplicação de análise de classe latente para avaliação dos testes diagnósticos

O presente estudo tem como objetivo avaliar os testes sorológicos convencionais usados na triagem neonatal para doença de Chagas, discutindo métodos estatísticos disponíveis. Estudou-se uma amostra aleatória dentre 23.308 recém-nascidos triados para doença de Chagas congênita por meio de três testes: imunoensaioenzimático, imunofluorescência indireta e hemoaglutinação indireta. Os dados obtidos foram analisados por diferentes metodologias estatísticas: a análise de classe latente, o teste Kappa e a análise de sensibilidade relativa. Utilizando a análise de classe latente, a maior sensibilidade foi do imunoensaioenzimático (48,6%), seguido pela imunofluorescência indireta (39,8%) e pela hemoaglutinação indireta (23,2%). O valor Kappa foi 0,496. A razão entre as sensibilidades dos testes imunoensaioenzimático e imunofluorescência indireta foi de 92% [0,74;1,13]. A análise de classe latente não se mostrou adequada para determinação de sensibilidade e especificidade, mas forneceu dados importantes sobre a equivalência dos testes, corroborados pela análise de sensibilidade relativa. Os resultados mostraram que o teste imunoensaioenzimático em sangue-seco pode ser utilizado com a mesma segurança do teste imunofluorescência indireta.

Fatores de risco associados à colonização por Candida spp em neonatos internados em uma Unidade de Terapia Intensiva Neonatal brasileira

Os objetivos desse estudo foram investigar a participação de Candida albicans e não-albicans como agente de colonização e sepse, bem como os fatores de risco associados aos neonatos internados na Unidade de Terapia Intensiva Neonatal do Hospital de Clínicas da Universidade Federal de Uberlândia. Foi realizada vigilância epidemiológica pelo sistema National Healthcare Safety Network no período entre agosto de 2007 e abril de 2008. A taxa de incidência de sepse com critério microbiológico foi de 6,7/1.000 paciente/dia, constatando-se apenas um caso de candidemia. Aproximadamente, 19% dos neonatos estavam colonizados por Candida, identificadas como Candida albicans (50%) e Candida não-albicans (50%). Os fatores de risco significantes para colonização por Candida spp foram a idade gestacional entre 26 e 30 semanas, o uso prévio de antibiótico e o cateter vascular central umbilical. A mortalidade total foi de 11,8% nos neonatos internados durante o período de estudo com sepse, porém o recém-nascido com candidemia não evoluiu para óbito.

Capillaria hepatica-induced septal fibrosis in rats: a contribution to the study of liver fibrogenesis

INTRODUCTION: Septal fibrosis of the liver regularly develops in rats infected with the nematode Capillaria hepatica. Curative treatment of the infection prevents the development of septal fibrosis when intervention occurs up to postinfection day (PID) 15, but not later. The present investigation aimed to demonstrate which parasitic factors are present when the process of septal fibrosis can no longer be prevented by curative treatment. METHODS: Wistar rats were infected with 600 embryonated eggs of C. hepatica administered by gavage and treated with ivermectin and mebendazole in separate groups at PIDs 10, 12, 15, 17 or 20. Rats from each group and their nontreated controls, were killed and examined 40 days after the end of treatment. RESULTS: Findings by PID 15 were compatible with the stage of complete maturation of infection, when worms and eggs were fully developed and a complex host-parasite multifocal necroinflammatory reaction showed greater intensity, but with no signs of septal fibrosis, which appeared from PID 17 onward. CONCLUSIONS: Since the worms spontaneously died by PID 15, not only septal fibrosis production, but also its maintenance and further development appeared dependent on the presence of eggs, which were the only parasitic factor remaining thereafter.