Cirurgias de Derivação do Ventrículo Pulmonar

Cavopulmonary connections have been extensively used in the palliation of complex forms of congenital heart disease requiring some form of right heart bypass. We examine the mid term outcomes of pulmonary ventricle bypass operations in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and April 2006, 62 patients underwent pulmonary ventricle bypass operations: bidirectional cavopulmonary anastomosis (Glenn procedure), total cavopulmonary connections (Fontan procedure) and one and a half ventricle correction in two cases. Age at operation averaged three years (range: 0.42-25 years) for the Glenn procedure and seven years (range: 3-14 years) for the Fontan procedure. There were 36 male patients (58%) and 26 female patients (42%). The most common indication for surgery was the single ventricle defect, present in 66% of patients. Associated lesions included: transposition of the great arteries in 16 patients (35.6%), bilateral superior vena cava in four patients (8.9%), situs ambigus in five patients (11%), situs inversus in another patient (2.2%), Ebstein disease in one patient (2.2) and coronary fistula in another patient (2.2%). Sub-aortic stenosis was present in one patient (2.2%). Palliative surgery was performed in all, but three patients (5%), before the Fontan procedure. RESULTS: Thirty two patients underwent bidirectional cavopulmonary anastomosis and thirty patients underwent cavopulmonary connections, total or 2nd stage. Mean cardiopulmonary bypass times were 50.6+/-21.9 minutes for the Glenn procedure and 88.5+/-26.3 minutes for the Fontan procedure. There was no intra-operative mortality, but two patients (3.2% (died in the first month after surgery; one due to failure of the Glenn circuit and sepsis and the other due to a low cardiac output syndrome and multi-organ dysfunction. Mean ventilation time was 5.2+/-1.7 hours for the Glenn operation and 6.2+/-3.2 hours for the Fontan operation. The mean length of stay in ICU was 3.4+/-2.8 days for patients undergoing the Glenn operation and 4.6+/-3.1 days for patients undergoing the Fontan operation and the mean length of hospital stay was 10.6+/-5.8 days for the Glenn operation and 19.1+/-12.6 days for the Fontan operation respectively. The mean follow up time was 4+/-2.1 years (minimum 0 years and maximum seven years), most patients being in NYHA class I. Epicardiac pacemakers were implanted in three patients due to arrhythmias. Two re-operations (6.7%) were needed, both in the same patient, after the Fontan procedure, this patient eventually died a few years after surgery. CONCLUSIONS: The immediate and mid term outcomes of pulmonary ventricle bypass operations can have excellent results. From our point of view there has been an improvement, namely in the use of the extracardiac conduit technique in the 2nd stage of the Fontan operation.

Hearing impairment in patients with tuberculosis from Northeast Brazil

The aim of this paper is to describe the auditory profile of subjects who were given streptomycin treatment for tuberculosis in the years 2000 and 2001, in Recife, Northeast Brazil. The Injury Notification Database at the Municipal Department of Health was consulted and 78 individuals who had been on streptomycin during the period under study were selected. Forty-two individuals were excluded, of whom five were over 59 years of age and two were under 18 years. Nineteen turned out to be deceased, 13 could not be found, two were serving prison terms and one refused to participate. As a result, only 36 subjects participated in the study. These 36 individuals were interviewed and underwent meatoscopy and audiometry. The mean age of the group under study was 38.8 years old and males predominated (79.4%). Twenty-seven patients (75%) showed hearing impairment, the bilateral sensorineural type (63.9%) being the most frequent, mainly affecting the high frequencies over 4000 Hz. In the light of findings showing a high percentage of hearing impairment complaints among users of streptomycin, this issue seems to warrant an in-depth investigation, as does the implementation of an auditory follow-up routine for patients undergoing such chemotherapy for tuberculosis.

Pancreatite Aguda como Manifestação de Linfoma de Burkitt: em Idade Pediátrica

A 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.

Pés Talus. Caso Clínico

A propósito de um doente com pé talo bilateral sequela de mielomeningocelo L5-S1, procedeu-se a uma revisão bibliográfica desta rara situação clínica. Trata-se de um doente operado aos 28 anos de idade, por apoio instável sobre os calcâneos, calosidades exuberantes e impossibilidade de equilíbrio na posição de pé sem movimento, como resultado do predomínio dos dorsiflexores da tibio-társica sobre os flexores plantares. A solução cirúrgica adoptada visou a obtenção de pé plantigrado e estabilidade na marcha.

Abcesso Tubo-Ovárico. Análise de 20 Casos

Os autores procedem à análise retrospectiva dos casos de abcesso tubo-ovárico, internados no Serviço de Ginecologia da Maternidade Dr. Alfredo da Costa, num período de 3 anos (1991-1993). O número total de casos foi 20. A incidência em nulíparas foi 25%. Uma percentagem significativa das doentes (30%) tinha sido recentemente submetida a técnicas de instrumentação uterina. Em apenas 15% dos casos havia antecedentes de doença inflamatória pélvica. Nas doentes portadoras de DIU a incidência de abcessos unilaterias e bilaterais foi idêntica. Na maioria dos casos (85%) a apirexia surgiu até 48 horas após o início da antibioterapia. A maioria das doentes (90%) foi submetida a terapêutica cirúrgica. O tempo médio decorrido entre o início da antibioterapia e a intervenção cirúrgica foi 3 dias. Registou-se um caso de rotura de abcesso. Em 3 (15%) doentes verificou-se intraoperatoriamente a co-existência de um abcesso apendicular. Em 30% dos casos as doentes foram submetidas a histerectomia total com anexectomia unilateral ou bilateral. Uma das 2 (10%) doentes submetidas a terapêutica médica isolada apresentou recidiva 1 mês após a alta. A abordagem terapêutica do abcesso tubo-ovárico embora se tenha tornado mais conservadora, continua a incluir, na maioria dos casos, drenagem ou resecção cirúrgica após antibioterapia adequada.

Litíase Coraliforme: Caso Clínico Raro e Complicado

Introdução: Este artigo tem como objectivo apresentar um caso clínico de litíase coraliforme que exemplifica a gravidade resultante desta patologia. Caso Clínico: Caso clínico de uma doente do sexo feminino, 43 anos, sem antecedentes pessoais de relevo, que recorreu ao Serviço de Urgência por prostração, astenia e anorexia. Foi-lhe diagnosticada insuficiência renal grave, litíase coraliforme bilateral e hidronefrose com conteúdo não puro no excretor. A doente foi submetida a colocação bilateral de nefrostomias e uma semana depois, num contexto de pionefrose à esquerda e sépsis, foi submetida a nefrectomia total à esquerda. Ficou a realizar hemodiálise em ambulatório. Posteriormente concluiu-se perda irreversível da função renal, com consequente nefrectomia direita. Discussão: Este caso constitui um exemplo da gravidade resultante da litíase coraliforme. Mesmo sem manifestação clínica prévia, pode causar insuficiência renal avançada, com infecção urinária grave e sépsis que colocam a vida em risco.

Burkitt’s Lymphoma Related to Epstein–Barr Virus Infection During Pregnancy

Setting: Burkitt’s lymphoma is a rare form of cancer and is an extremely rare diagnosis during pregnancy. This form of lymphoma is a very fast growing B cell neoplasm and chemotherapy is the treatment of choice for the disease in all its stages. Case report: The authors describe the case of a Caucasian 40-year-old nulliparous woman, with previous known Epstein–Barr virus infection, that presents at 28 weeks gestation with supraclavicular adenopathy and multiple bilateral breast nodules, in which biopsy showed non-Hodgkin lymphoma, Burkitt’s type. Discussion: There are few described cases of Burkitt’s lymphoma during pregnancy and in general the outcomes have been poor. In most of the cases, the patients were not treated by current standards or instead had a late diagnosis. This neoplasia is the most rapidly progressive human tumor, and any delay in initiating therapy can adversely aVect patient’s prognosis. The authors discuss treatment options in pregnancy and its perinatal implications.

Decision Support for Energy Contracts Negotiation with Game Theory and Adaptive Learning

This paper presents a decision support methodology for electricity market players’ bilateral contract negotiations. The proposed model is based on the application of game theory, using artificial intelligence to enhance decision support method’s adaptive features. This model is integrated in AiD-EM (Adaptive Decision Support for Electricity Markets Negotiations), a multi-agent system that provides electricity market players with strategic behavior capabilities to improve their outcomes from energy contracts’ negotiations. Although a diversity of tools that enable the study and simulation of electricity markets has emerged during the past few years, these are mostly directed to the analysis of market models and power systems’ technical constraints, making them suitable tools to support decisions of market operators and regulators. However, the equally important support of market negotiating players’ decisions is being highly neglected. The proposed model contributes to overcome the existing gap concerning effective and realistic decision support for electricity market negotiating entities. The proposed method is validated by realistic electricity market simulations using real data from the Iberian market operator—MIBEL. Results show that the proposed adaptive decision support features enable electricity market players to improve their outcomes from bilateral contracts’ negotiations.

Ankle anticipatory postural adjustments during gait initiation in healthy and post-stroke subjects

Background: Anticipatory postural adjustments during gait initiation have an important role in postural stability but also in gait performance. However, these first phase mechanisms of gait initiation have received little attention, particularly in subcortical post-stroke subjects, where bilateral postural control pathways can be impaired. This study aims to evaluate ankle anticipatory postural adjustments during gait initiation in chronic post-stroke subjects with lesion in the territory of middle cerebral artery. Methods: Eleven subjects with post-stroke hemiparesis with the ability to walk independently and twelve healthy controls participated in this study. Bilateral electromyographic activity of tibialis anterior, soleus and medial gastrocnemius was collected during gait initiation to assess the muscle onset timing, period of activation/deactivation and magnitude of muscle activity during postural phase of gait initiation. This phase was identified through centre of pressure signal. Findings: Post-stroke group presented only half of the tibialis anterior relative magnitude observed in healthy subjects in contralesional limb (t=2.38, p=0.027) and decreased soleus deactivation period (contralesional limb, t=2.25, p=0.04; ipsilesional limb, t=3.67, p=0.003) as well its onset timing (contralesional limb, t=3.2. p=0.005; ipsilesional limb, t=2.88, p=0.033) in both limbs. A decreased centre of pressure displacement backward (t=3.45, p=0.002) and toward the first swing limb (t=3.29, p=0.004) was observed in post-stroke subjects. Interpretation: These findings indicate that chronic post-stroke subjects with lesion at middle cerebral artery territory present dysfunction in ankle anticipatory postural adjustments in both limbs during gait initiation.

Ankle antagonist coactivation in the double-support phase of walking: Stroke vs. healthy subjects

Introduction: Lesions at ipsilateral systems related to postural control at ipsilesional side, may justify the lower performance of stroke subjects during walking. Purpose: To analyse bilateral ankle antagonist coactivation during double-support in stroke subjects. Methods: Sixteen (8 females; 8 males) subjects with a first isquemic stroke, and twenty two controls (12 females; 10 males) participated in this study. The double support phase was assessed through ground reaction forces and electromyography of ankle muscles was assessed in both limbs. Results: Ipsilesional limb presented statistical significant differences from control when assuming specific roles during double support, being the tibialis anterior and soleus pair the one in which this atypical behavior was more pronounced. Conclusion: The ipsilesional limb presents a dysfunctional behavior when a higher postural control activity was demanded.

Epilepsy and Cerebral Palsy: Characteristics and Trends in Children Born in 1976 - 1998

Background: Although epilepsy is common in children with cerebral palsy (CP), no data exists on prevalence rates of CP and epilepsy. Aims: To describe epilepsy in children with CP, and to examine the association between epilepsy and neonatal characteristics, associated impairments and CP subtypes. Methods: Data on 9654 children with CP born between 1976 and 1998 and registered in 17 European registers belonging to the SCPE network (Surveillance of Cerebral Palsy in Europe)were analyzed. Results: A total of 3424 (35%) children had a history of epilepsy. Among them, seventy-two percent were on medication at time of registration. Epilepsy was more frequent in children with a dyskinetic or bilateral spastic type and with other associated impairments. The prevalence of CP with epilepsy was 0.69 (99% CI, 0.66e0.72) per 1000 live births and followed a quadratic trend with an increase from 1976 to 1983 and a decrease afterwards. Neonatal characteristics independently associated with epilepsy were the presence of a brain malformation or a syndrome, a term or moderately preterm birth compared with a very premature birth, and signs of perinatal distress including neonatal seizures, neonatal ventilation and admission to a neonatal care unit. Conclusions: The prevalence of CP with epilepsy followed a quadratic trend in 1976e1998 and mirrored that of the prevalence of CP during this period. The observed relationship between epilepsy and associated impairments was expected; however it requires longitudinal studies to be better understood.

Lúpus Eritematoso Sistémico

O Lúpus Eritematoso Sistémico (LES) é uma doença multissistémica, auto-imune, caracterizada por inflamação vascular e do tecido conjuntivo, com anticorpos antinucleares. As manifestações clínicas são variáveis, com uma história natural progressiva e imprevisível. Apresentamos o caso de uma adolescente, com antecedentes de artrite dos joelhos, febre e astenia de etiologia não esclarecida, com um ano de evolução. Internada na Unidade de Cuidados Intensivos Pediátricos por pneumonia bilateral a Streptococcus pneumoniae complicada com derrame pleural, anasarca e hematúria macroscópica. Iniciou antibioticoterapia, com evolução favorável, após o que se verifica agravamento clínico, com reaparecimento do derrame pleural, lesões vesiculares disseminadas sugestivas de etiologia herpética, hipertensão arterial sintomática e convulsão tónico-clónica generalizada. Da investigação, destaca-se estudo imunológico compatível com LES em actividade, poliserosite, proteinúria nefrótica, nefrite lúpica classe IV e anemia grave.

Síndrome de Hiperestimulação do Ovário em Gravidez Espontânea

Introdução: O Síndrome de Hiperestimulação do Ovário (SHO) geralmente é descrito como uma complicação iatrogénica dos tratamentos de indução da ovulação. Raramente, pode estar associado a ciclos ovulatórios espontâneos, sendo mais frequente em gravidez múltipla, patologia molar e hipotiroidismo. A apresentação clínica é variável, podendo nos casos mais graves ser fatal. Caso clínico: Grávida de 13 semanas, nulípara, sem história de tratamentos de infertilidade, referenciada por aumento bilateral do volume ovárico e ascite. Apresentava ligeiro desconforto abdominal, tendo a ecografia revelado ovários aumentados de volume, multiquísticos e ascite ligeira. Os níveis de estradiol estavam aumentados, com hCG e TSH normais. A terapêutica foi expectante, com uma evolução favorável. Discussão: O SHO espontâneo apesar de ser uma entidade rara, deve ser equacionada como hipótese diagnóstica nas situações de massa pélvica na gravidez. Estão descritos 3 possíveis mecanismos para o SHO espontâneo, baseados na permissividade do receptor ovárico da FSH para a hCG e/ou TSH, podendo ocorrer com níveis normais ou elevados de hCG e/ou TSH. C

Parotidite Como Primeira Manifestação de Infecção por VIH

Introdução: Actualmente, a maioria dos casos pediátricos de infecção por VIH é devida a transmissão materna do vírus. Na ausência de medidas de profilaxia, verificam se taxas de transmissão vertical do VIH-1 entre 15-25% na Europa Ocidental e Estados Unidos, 65% dos casos no peri-parto, 23% in útero e 12% no período pós-natal durante a amamentação. Caso clínico: Criança de 9 anos, sexo feminino, que recorre à urgência por febre, anorexia e tumefacção cervical com 2 dias de evolução. Dos antecedentes pessoais há a destacar: gravidez não vigiada, parto eutócico de termo, aleitamento materno até aos 3 anos e atraso do desenvolvimento estaturo-ponderal. Antecedentes patológicos de parotidite bilateral aos 5 anos e múltiplas cáries dentárias. À observação apresentava-se febril, emagrecida (peso < P5 e estatura no P5), com tumefacção cervical e retroauricular direitas, e aumento de volume das glândulas parótidas. Sem hepatoesplenomegalia e sem adenopatias palpáveis nas restantes cadeias ganglionares periféricas. Analíticamente, VS de 90 mm/h, sem outras alterações relevantes. Ecografia cervical mostrou adenofleimão e alterações compatíveis com parotidite. Internada com a hipótese diagnóstica de adenofleimão cervical e medicada com penicilina e clindamicina endovenosas (ev). Realizou serologias para VIH, com positividade para VIH tipo 1, confirmado por Western Blot. Contagem de linfócitos T CD4+ de 240 células/mm3. Carga viral de 3,82 x 103 cópias de RNA/mL. Genótipo HLA-B*5701 negativo. Confirmada infecção VIH 1 materna por Western Blot. Diagnóstico prévio de infecção VIH no período neonatal ocultado pela mãe. Restantes serologias negativas, assim como a pesquisa de BK no suco gástrico e o estudo do lavado bronco-alveolar. Ao 17º dia de internamento foi realizada punção do adenofleimão e alterada a antibioticoterapia para flucloxacilina ev (7 dias de terapêutica). Pesquisa de micobactérias e fungos no pús drenado negativa. Durante o internamento manteve-se clinicamente estável, iniciando profilaxia para Pneumocystis jirovecii com cotrimoxazol, e terapêutica anti-retroviral (Lamivudina, Abacavir, Lopinavir/Ritonavir), com melhoria clínica, virulógica e imunológica. Conclusões: Este caso ilustra um exemplo de transmissão vertical do VIH-1 caracterizado por uma evolução crónica, cuja apresentação cursou com parotidite, um dos sinais indicadores de infecção VIH.

On an acute case of Chagas disease in a region under vector control in the state of São Paulo, Brazil

No vector transmitted cases of Chagas disease had been notified in the state of São Paulo since the 1970s. However, in March, 2006, the death of a six-year-old boy from the municipality of Itaporanga was notified to the Center for Epidemiological Survey of the São Paulo State Health Secretariat: an autochthonous case of acute Chagas disease. The postmortem histopathological examination performed in the Hospital das Clínicas of the Botucatu School of Medicine confirmed the diagnosis. Reference to hospital records, consultation with the health professionals involved in the case and interviews with members of the patient's family supplied the basis for this study. We investigated parasite route of transmission, probable local reservoirs and vectors. No further human cases of acute Chagas disease were diagnosed. No locally captured vectors or reservoirs were found infected with Trypanosoma cruzi. Alternative transmission hypotheses - such as the possible ingestion of foods contaminated with vector excreta - are discussed, as well as the need to keep previously endemic regions and infested houses under close surveillance. Clinicians should give due attention to such signs as uni- or bilateral palpebral edema, cardiac failure, myocarditis, pericarditis, anasarca and atypical signs of nephrotic syndrome or nephritis and consider the diagnostic hypothesis of Chagas disease.