Vólvulo Intestinal en el Periodo Neonatal: 8 Años de Experiencia en un Hospital Pediátrico Terciario

Análise dos casos de volvo intestinal ocorridos no período neonatal nos últimos 8 anos (2002 a 2010). Material e métodos: Foram estudados os recém-nascidos admitidos na UCIN cujo diagnóstico de saída foi volvo intestinal. Foram estudados os seguintes parâmetros: idade gestacional e pós-natal, apresentação clínica e imagiológica, intervenção cirúrgica e resultados. Resultados: Foram identificados 15 doentes 7 dos quais no último ano do estudo. Sete RN eram pré-termo (PT) ou ex pré-termo. A mediana de peso ao nascer foi de 2665g (660-3900); 4 RN eram muito baixo peso. A mediana de idade de início dos sintomas foi 7 dias; em 5 RN a doença teve início nas primeiras 24 horas de vida; em 3 destes, o volvo ocorreu in utero. Sinais e sintomas: grande distensão abdominal-12; resíduo gástrico bilioso-11; alterações da parede abdominal-5; dejecções com sangue-4; instabilidade hemodinâmica-6. Imagiologia: grande distensão de ansas, sem ar ectópico- 10 doentes; ausência de ar no abdómen-4; trânsito intestinal contrastado sugestivo de malrotação e volvo-3; ecografia e Doppler abdominal com sinal de “whirlpool”-2. Todos foram submetidos a cirurgia de urgência, sendo o volvo confirmado intraoperatoriamente; foi necessária ressecção intestinal em 9 doentes; 3 ficaram com síndrome do intestino curto; registou-se um óbito por falência multi-orgânica no período pós-operatório. Conclusão: Foi encontrado um elevado número de casos de volvo intestinal em RN pré-termo ou ex pré-termo, de volvo in utero e de elevada ocorrência de casos no último ano do estudo. Resíduo gástrico bilioso e distensão abdominal foram os sintomas mais frequentes de volvo e devem ser tomados em consideração no diagnóstico diferencial com outras situações cirúrgicas abdominais. As sequelas são potencialmente graves.

How Were Conflicts of Interest Handled?

Conflicts of interest were potentially great but they were minimized by the great conviction from both Doctors and Health Ministry that something had to be done to improve data on perinatal health. To decrease the number of hospitals where deliveries took place, to concentrate doctors, nurses and equipment, to define staff and to acquire equipment and to train nurses and paediatricians was the way. One the point of view of cost-effectiveness, centralization of expensive technologies, and development of expertise concentrating cases in a same centre - Surgery, VLBW, etc- and lowering mortality rates and get better outcomes were clear health gains. In 1989 after the political decision of closing small maternities the committee return to villages and cities to explain to political local power and people, the decision, which kind of care they will have in the future, why and expected gains. Level I hospitals and Health Centers stop to have deliveries; Health Centers were given a great responsibility: the follow up of the most part of the normal pregnancies by GP. There was no economic pressure because the National Health Service is free, there are no economic incentives for obstetrical or neonatal care, hospitals are financed through ICD, hospital level is defined according to both delivery and newborn care. In 1989 the rule was “No results can be obtained without the interested and responsible participation of all – institutions and people”. At that time the emphasis was on training. There are geographic influences on regionalization for example for islands and inner and far geographic areas. Also we would like to emphasize the influence of demographics on regionalization. As birth rate continues to decrease the hospitals left open 20 years ago with more than 1500 deliveries have to be closed now because the number of deliveries decreased. It was much more difficult and unacceptable to close some few maternities now than 20 years ago. All the difference was that at that time reasons were explained and now it was a Minister order. Other fearful events are the opening of private hospitals, the lowering gross national income, the economic difficulties and financial problems.

Rastreio Neonatal Sistemático de Infecção por CMV

O virus citomegálico humano (HCMV) é a principal causa de infecção congénita. Estima-se que em Portugal se situe entre 0,7% e 1%. O registo nacional de casos de infecção congénita por CMV realizado pela UVP/SPP entre 2006 e 2011, encontrou uma incidência de 0.074/1000 nados vivos. Atendendo a que este é um registo de RN sintomáticos e que estes correspondem a 10% dos infectados, teremos cerca de 0,7/1000 RN infectados por ano em Portugal, um valor semelhante ao encontrado no Reino Unido e Irlanda. Uma revisão americana usando exclusivamente população de RN infectados diagnosticados em estudos de rastreio universal e englobando 117 986 RN, concluiu que a incidência da infecção foi de 0,7% e a percentagem de crianças sintomáticas foi de 12,7% das quais 40 a 58% vieram a ter sequelas permanentes; das crianças assintomáticas 13,5% vieram a desenvolver sequelas permanentes. A surdez neurosensorial é considerada a sequela mais frequente contudo há grande desconhecimento sobre as sequelas visuais. A correcção precoce da surdez melhora muito o prognóstico da criança pelo que um diagnóstico precoce é essencial. O rastreio auditivo neonatal detecta apenas cerca de 50% destas crianças uma vez que a surdez é evolutiva podendo manifestar-se mais tarde. O rastreio pós natal de infecção congénita assintomática seria de grande utilidade mas não está ainda determinado qual a melhor estratégia para atingir tal objectivo. A utilização dos cartões de Guthrie para este fim parece ser uma boa solução mas alguns estudos questionam a sensibilidade da técnica. O custo de um programa deste tipo em Portugal poderia rondar os 19 milhões de euros anuais contabilizando apenas o preço de uma PCR por RN. Obviamente que muitos resultados teriam que ser repetidos ou confirmados por cultura, o que agravaria mais o orçamento. Na ausência de metodologia de rastreio com sensibilidade adequada para detectar infecções assintomáticas, o meio mais correcto de diagnosticar surdez na criança terá que se basear na clínica e na sensibilização dos pais para a detecção precoce de défice auditivo. A intervenção terapêutica adequada melhorará em muito a função mas outras terapêuticas, nomeadamente antivírica, não estão aprovadas nos RN assintomáticos.

Why and How Was Regionalization of Perinatal Care Accomplished in Portugal?

Portuguese health care system was created in 1979. It is universal and for free. Expenses are supported by the State through taxes. The modern perinatal care system started by the end of 1970. The first neonatal intensive care units were created in 1980, the Portuguese Neonatal Society in 1985 and the National Neonatal Transport System in 1987. Until the seventies of twentieth century and even during eighties there were more than 200 hospitals with deliveries, a great part without obstetrician or paediatrician, a great percentage of pregnancies had no prenatal care, there were few neonatal intensive care units and perinatal mortality rate was one of the highest in the European countries. In 1987 an Experts Committee was nominated by the Health Ministry aiming to collect and analyse data on perinatal care and to suggest improvements. The Report resulting from this work is the main document on which is based the reform. The reform was a 9 years program in 3 years stages aiming to close hospitals with less than 1500 deliveries/year, to reclassify hospitals, to create Coordinating Units between health centres and hospitals, to equip neonatal intensive and intermediate care units, to define needs of obstetricians, paediatricians and nurses for each centre and to promote specialised training in neonatology for paediatricians and nurses. Levels of perinatal care were defined as well as localization of each level of hospital according to the number of deliveries in one geographic area, geographic difficulties and existing routes and connections. Steps for opening and closure of different levels of hospitals were very well programmed. The organization, capacities, number of obstetricians, neonatologists and nurses as well as equipment for each level of care was defined. Rules for pregnant women and newborns transfer from level II to level III hospitals were also well described. A specific training is neonatology was created starting in 1990. This organization resulted in an impressive decrease in mortality rates at all levels and still it is the policy we have today.

Mucoviscidose com Sintomatologia Respiratória no Período Neonatal

Descreve-se um caso de mucoviscidose com sintomatologia respiratória iniciada no período neonatal, associada a insuficiência pancreática invulgarmente precoce, o estudo da genética molecular revelou que, ao nível do gene CFTR, foi identificado na doente um composto genético das mutações FS08 e GS42X Realça-se a raridade desta forma de apresentação sendo no entanto lícito admitir-se esta entidade nosológica no diagnóstico diferencial da sindroma de dificuldade respiratória no recém-nascido. Discute-se a patogénese e alguns aspectos particulares da terapêutica instituida, os quais tem sido importantes para a melhoria da expectativa de vida de doentes com esta patologia.

Mortality after Discharge from Intensive Care: the Impact of Organ System Failure and Nursing Workload Use at Discharge

OBJECTIVES: Mortality after ICU discharge accounts for approx. 20-30% of deaths. We examined whether post-ICU discharge mortality is associated with the presence and severity of organ dysfunction/failure just before ICU discharge. PATIENTS AND METHODS: The study used the database of the EURICUS-II study, with a total of 4,621 patients, including 2,958 discharged alive to the general wards (post-ICU mortality 8.6%). Over a 4-month period we collected clinical and demographic characteristics, including the Simplified Acute Physiology Score (SAPS II), Nine Equivalents of Nursing Manpower Use Score, and Sequential Organ Failure Assessment (SOFA) score. RESULTS: Those who died in the hospital after ICU discharge had a higher SAPS II score, were more frequently nonoperative, admitted from the ward, and had stayed longer in the ICU. Their degree of organ dysfunction/failure was higher (admission, maximum, and delta SOFA scores). They required more nursing workload resources while in the ICU. Both the amount of organ dysfunction/failure (especially cardiovascular, neurological, renal, and respiratory) and the amount of nursing workload that they required on the day before discharge were higher. The presence of residual CNS and renal dysfunction/failure were especially prognostic factors at ICU discharge. Multivariate analysis showed only predischarge organ dysfunction/failure to be important; thus the increased use of nursing workload resources before discharge probably reflects only the underlying organ dysfunction/failure. CONCLUSIONS: It is better to delay the discharge of a patient with organ dysfunction/failure from the ICU, unless adequate monitoring and therapeutic resources are available in the ward.

Mortalidade numa Unidade de Cuidados Intensivos Pediátricos

Artigos descrevendo o movimento assistencial de Unidades de Cuidados Intensivos Pediátricos e analisando as respectivas mortalidades não são muito comuns. Este estudo retrospectivo tem como objectivo avaliar e analisar o movimento assistencial e a mortalidade da Unidade de Cuidados Intensivos Pediátricos (UCIP) do Hospital Dona Estefania (HDE) num período de 6 anos (0.4.04.91 e 03.04.97). Neste período houve 2987 internamentos na UCIP e foram registados 200 (6.70%) óbitos, os quais corresponderam a 40.48% dos 494 6bitos verificados no HDE. A análise estatística mostrou diferenças significativas, entre sobreviventes e falecidos, relativamente aos seguintes parâmetros: índice de gravidade (4.65 vs 21.42); probabilidade de morte (3,69% vs 39.97%); grau de intervenção terapêutica (14.70 vs 34.80); idade média (4.11 vs 3.33 anos), demora média (2.70 vs 8.86 dias); incidência de doença crónica (44.73 vs 62.79%); reinternamentos(2.20 vs 16.28%) e proveniencia - Serviço de Urgência / Outros Hospitais - (51.95 vs 25.58 / 18.87 vs 39.53%). Existia doença crónica em 108 (62.79%) dos falecidos (ligeira em 27.78% e significativa em 72.22%), salientando-se a cardíaca (27.78%), a do SNC (13.89%), e a relacionada com patologia do período neonatal (10.19%). A causa final de morte relacionou-se predominantemente com patologia infecciosa (40.70%), respiratória (19.77%), cardíaca (12.21 %) e do SNC (11.63%). Existia falência mono-orgão (OSF) em 24 (13.95%) e falência múltipla de orgão (MOSF) em 148 (86.05%) dos falecidos. A mortalidade esperada era de 5.807%, tendo a mortalidade observada sido de 5.840% (Standardized Mortality Ratio = 1.006).

Sepsis Mortality Prediction Based on Predisposition, Infection and Response

OBJECTIVE: To empirically test, based on a large multicenter, multinational database, whether a modified PIRO (predisposition, insult, response, and organ dysfunction) concept could be applied to predict mortality in patients with infection and sepsis. DESIGN: Substudy of a multicenter multinational cohort study (SAPS 3). PATIENTS: A total of 2,628 patients with signs of infection or sepsis who stayed in the ICU for >48 h. Three boxes of variables were defined, according to the PIRO concept. Box 1 (Predisposition) contained information about the patient's condition before ICU admission. Box 2 (Injury) contained information about the infection at ICU admission. Box 3 (Response) was defined as the response to the infection, expressed as a Sequential Organ Failure Assessment score after 48 h. INTERVENTIONS: None. MAIN MEASUREMENTS AND RESULTS: Most of the infections were community acquired (59.6%); 32.5% were hospital acquired. The median age of the patients was 65 (50-75) years, and 41.1% were female. About 22% (n=576) of the patients presented with infection only, 36.3% (n=953) with signs of sepsis, 23.6% (n=619) with severe sepsis, and 18.3% (n=480) with septic shock. Hospital mortality was 40.6% overall, greater in those with septic shock (52.5%) than in those with infection (34.7%). Several factors related to predisposition, infection and response were associated with hospital mortality. CONCLUSION: The proposed three-level system, by using objectively defined criteria for risk of mortality in sepsis, could be used by physicians to stratify patients at ICU admission or shortly thereafter, contributing to a better selection of management according to the risk of death.

Incontinentia Pigmenti in the Neonatal Period

Incontinentia pigmenti (IP) is a rare multisystem disease, X linked dominant disorder. As all X linked dominant diseases, it is usually male-lethal. Female newborn admitted to the neonatal intensive care unit on the fi rst day of life was diagnosed as having probable herpetic infection with vesicular skin lesions distributed on upper right limb and inferior limbs. Family history showed that her 22-year-old mother had hypopigmented lesions on the lower limbs and her 13-month-old sister had hyperpigmented lesions on the trunk and limbs. In newborns, herpes infection emerges as the principal diagnosis of vesicular rash, due to the importance of precocious diagnosis and treatment. Other hypothesis must be considered in a newborn with vesicobullous rash, such as IP.

Restrição do Crescimento Fetal - Casuística de 4 Anos

Overview and aims: Fetal growth restriction (FGR) affects 15% of pregnancies and is associated with both increased perinatal and neonatal morbidity and mortality and long-term effects in adult life. Our aim was to describe cases and outcomes of FGR from a tertiary perinatal care centre and identify the predictors of neonatal morbidity and mortality. Study design: retrospective cohort. Population: pregnancies with early or late FGR caused by placental factors followed from 2006 to 2009 in a tertiary perinatal care centre. Methods: we collected data from clinical records on demographics, clinical history and fetal ultrasound parameters. Perinatal and neonatal outcomes were stratiied according to gestational age (above or below 28 weeks) and we used bivariate analysis to identify any associations with clinical and imaging indings. Results: we included 246 pregnancies; hypertension was the most prevalent maternal risk factor (16%). There were 15 cases of early FGR, 11 of which had cesarean delivery due to deterioration of fetal Doppler parameters. Outcomes in this group included one fetal and three neonatal deaths. Of 231 cases of late FGR, 64% were delivered early given a non-reassuring fetal status i.e. due to changes in Doppler evaluation or altered Manning biophysical proile. There were four cases of perinatal death in this group, three of which delivered at 28 weeks. Neonatal morbidity was associated with lower gestational age, lower birthweight and progressive placental dysfunction (p<0.01). Conclusion: there was an association between neonatal morbidity and gestational age, birthweight and Doppler deterioration, particularly for deliveries below 28 weeks. The assessment of vascular changes through Doppler analysis allows anticipation of fetal deterioration and is a helpful tool in deciding the optimum timing of delivery.

Rastreio Universal da Audição Neonatal um Pequeno Teste para o Audiologista , um Grande Passo para a Humanidade?

O Rastreio Universal da Audição Neonatal tem sido um objectivo que várias gerações de audiologistas, otorrinolaringologistas e pediatras tem tentado ao longo dos anos. Os autores fazem uma revisão dos principais métodos utilizados para a avaliação da audição dos recém-nascidos, bem como das dificuldades encontradas e limitações da sua utilização. Desde o inicio da década de noventa vêm a ser implementados verdadeiros métodos de detecção precoce e universal da surdez infantil, utilizando métodos fisiológicos tais como os potenciais evocados auditivos e as otoemissões acústicas (c1ássicos e automáticos). São descritos os artigos mais importantes que fundamentam a necessidade de diagnóstico e intervenção precoces na surdez sensorioneural, com vista a melhorar a aquisição e desenvolvimento da fala e da competência linguística, o que permite uma melhor integração da criança, independentemente do seu grau de surdez. São também enunciadas as directivas do "European Consensus Development on Neonatal Hearing Screening" e do "Joint Comitee on Infant Hearing".

Gastropexia Laparoscópica em Volvo Gástrico Neonatal

Introdução: O Volvo gástrico é uma doença rara em idade pediátrica. Estão descritos casos crónicos recorrentes e agudos, mas a sua incidência real é desconhecida. Apresentamos a descrição cirúrgica de um caso de volvo gástrico crónico, recorrente, diagnosticado no período neonatal, tratado com sucesso por via laparoscópica. Caso clínico: Recém-nascido, do sexo masculino, portador de anomalia de Ebstein, com vómitos não biliosos recorrentes, desidratação e perda ponderal. O estudo contrastado, do tubo digestivo superior, mostrou volvo gástrico organoaxial, tendo sido submetido a gastropexia por via laparoscópica. Discussão: Segundo a maioria dos autores, a correcção cirúrgica do volvo gástrico é mandatória para prevenção de recorrências e complicações. A gastropexia por via minimamente invasiva é simples e segura.

MORBIDITY AND MORTALITY DUE TO AIDS: A STUDY OF BURDEN OF DISEASE AT A MUNICIPAL LEVEL

Introduction: The purpose of measuring the burden of disease involves aggregating morbidity and mortality components into a single indicator, the disability-adjusted life year (DALY), to measure how much and how people live and suffer the impact of a disease. Objective: To estimate the global burden of disease due to AIDS in a municipality of southern Brazil. Methods: An ecological study was conducted in 2009 to examine the incidence and AIDS-related deaths among the population residing in the city of Tubarao, Santa Catarina State, Brazil. Data from the Mortality Information System in the National Health System was used to calculate the years of life lost (YLL) due to premature mortality. The calculation was based on the difference between a standardized life expectancy and age at death, with a discount rate of 3% per year. Data from the Information System for Notifiable Diseases were used to calculate the years lived with disability (YLD). The DALY was estimated by the sum of YLL and YLD. Indicator rates were estimated per 100,000 inhabitants, distributed by age and gender. Results: A total of 131 records were examined, and a 572.5 DALYs were estimated, which generated a rate of 593.1 DALYs/100,000 inhabitants. The rate among men amounted to 780.7 DALYs/100,000, whereas among women the rate was 417.1 DALYs/100,000. The most affected age groups were 30-44 years for men and 60-69 years for women. Conclusion: The burden of disease due to AIDS in the city of Tubarao was relatively high when considering the global trend. The mortality component accounted for more than 90% of the burden of disease.

LATERAL FLOW ASSAY FOR CRYPTOCOCCAL ANTIGEN: AN IMPORTANT ADVANCE TO IMPROVE THE CONTINUUM OF HIV CARE AND REDUCE CRYPTOCOCCAL MENINGITIS-RELATED MORTALITY

SUMMARYAIDS-related cryptococcal meningitis continues to cause a substantial burden of death in low and middle income countries. The diagnostic use for detection of cryptococcal capsular polysaccharide antigen (CrAg) in serum and cerebrospinal fluid by latex agglutination test (CrAg-latex) or enzyme-linked immunoassay (EIA) has been available for over decades. Better diagnostics in asymptomatic and symptomatic phases of cryptococcosis are key components to reduce mortality. Recently, the cryptococcal antigen lateral flow assay (CrAg LFA) was included in the armamentarium for diagnosis. Unlike the other tests, the CrAg LFA is a dipstick immunochromatographic assay, in a format similar to the home pregnancy test, and requires little or no lab infrastructure. This test meets all of the World Health Organization ASSURED criteria (Affordable, Sensitive, Specific, User friendly, Rapid/robust, Equipment-free, and Delivered). CrAg LFA in serum, plasma, whole blood, or cerebrospinal fluid is useful for the diagnosis of disease caused by Cryptococcusspecies. The CrAg LFA has better analytical sensitivity for C. gattii than CrAg-latex or EIA. Prevention of cryptococcal disease is new application of CrAg LFA via screening of blood for subclinical infection in asymptomatic HIV-infected persons with CD4 counts < 100 cells/mL who are not receiving effective antiretroviral therapy. CrAg screening of leftover plasma specimens after CD4 testing can identify persons with asymptomatic infection who urgently require pre-emptive fluconazole, who will otherwise progress to symptomatic infection and/or die.