Transient neonatal cyanosis associated with a new Hb F variant: Hb F viseu.

Neonatal cyanosis in healthy newborns can be associated either with methemoglobin due to cytochrome b5 reductase deficiency or to M-hemoglobin, a group of hemoglobin variants resulting from mutations in the globin chain genes. We report the clinical case of a neonate with cyanosis and normal cardiac and respiratory function. At birth the hematological parameters were normal; however, the methemoglobinemia was 16%. Spontaneously, the cyanosis gradually decreased and by the fifth month of age the methemoglobin level was normal. A heterozygous Gγ-globin gene (HBG2) missense mutation 87 C-A (Leu28Met) was identified. His father, with a history of transfusion in the neonatal period, is heterozygous for the same mutation. This hemoglobin variant, not previously described, was called Hb F Viseu and is the sixth Gγ-chain variant reported in association with neonatal cyanosis.

[Multidimensional Strategy Regarding the Reduction of Central-Line Associated Infection in Pediatric Intensive Care]. / Estratégia Multidimensional na Redução de Infeção Associada a Cateter Venoso Central em Pediatria.

INTRODUCTION: To determine the central-line associated bloodstream infection rate after implementation of central venous catheter-care practice bundles and guidelines and to compare it with the previous central-line associated bloodstream infection rate. MATERIAL AND METHODS: A prospective, longitudinal, observational descriptive study with an exploratory component was performed in a Pediatric Intensive Care Unit during five months. The universe was composed of every child admitted to Pediatric Intensive Care Unit who inserted a central venous catheter. A comparative study with historical controls was performed to evaluate the result of the intervention (group 1 versus group 2). RESULTS: Seventy five children were included, with a median age of 23 months: 22 (29.3%) newborns; 28 (37.3%) with recent surgery and 32 (43.8%) with underlying illness. A total of 105 central venous catheter were inserted, the majority a single central venous catheter (69.3%), with a mean duration of 6.8 ± 6.7 days. The most common type of central venous catheter was the short-term, non-tunneled central venous catheter (45.7%), while the subclavian and brachial flexure veins were the most frequent insertion sites (both 25.7%). There were no cases of central-line associated bloodstream infection reported during this study. Comparing with historical controls (group 1), both groups were similar regarding age, gender, department of origin and place of central venous catheter insertion. In the current study (group 2), the median length of stay was higher, while the mean duration of central venous catheter (excluding peripherally inserted central line) was similar in both groups. There were no statistical differences regarding central venous catheter caliber and number of lumens. Fewer children admitted to Pediatric Intensive Care Unit had central venous catheter inserted in group 2, with no significant difference between single or multiple central venous catheter. DISCUSSION: After multidimensional strategy implementation there was no reported central-line associated bloodstream infection Conclusions: Efforts must be made to preserve the same degree of multidimensional prevention, in order to confirm the effective reduction of the central-line associated bloodstream infection rate and to allow its maintenance.

Introdução: Determinar a incidência de infeções da corrente sanguínea associadas ao uso de cateter venoso central, após reforço de medidas multidisciplinares de boa prática e a sua comparação com a taxa de incidência de infeções da corrente sanguínea associadas ao uso de cateter venoso central prévia. Material e Métodos: Estudo observacional descritivo, com colheita prospetiva de dados, durante cinco meses, após implementação de medidas multidisciplinares. Foram incluídas todas as crianças admitidas na unidade de Cuidados Intensivos Pediátricos, submetidas à colocação de cateter venoso central e foi efetuada comparação com controlos históricos.Resultados: Incluíram-se 75 doentes com idade mediana de 23 meses: 22 (29,3%) recém-nascidos, 28 (37,3%) submetidos a cirurgia e 32 (43,8%) com patologia subjacente. Foram colocados 105 cateteres venosos centrais, com tempo médio de permanência de 6,8 ± 6,7 dias. O tipo de cateter venoso central mais comum foi de curta duração (45,7%), sendo os locais de inserção mais frequentes a veia subclávia e da flexura braquial (ambos 25,7%). Não ocorreu nenhuma infeção da corrente sanguínea associada ao uso de cateter venoso central durante o período do estudo. Comparando com os controlos históricos, ambos os grupos eram semelhantes relativamente à idade, género, proveniência dos doentes e local de colocação de cateter venoso central. No estudo atual, a duração mediana de internamento foi superior, com tempo de permanência de cateter venoso central (excluindo epicutâneo-cava) semelhante. Não se verificou diferença em relação ao calibre e número de lumens do cateter venoso central utilizado. A percentagem de crianças que colocou cateter venoso central em relação ao total de crianças admitidas no serviço no mesmo período foi menor no estudo atual, não existindo diferença significativa entre colocação de cateter venoso central único ou múltiplo. Discussão: Após implementação da estratégia multidimensional não se registou nos Cuidados Intensivos Pediátricos ocorrência de infeções da corrente sanguínea associadas ao uso de cateter venoso central. Conclusões: Devem ser encetados esforços para preservar o mesmo grau de prevenção multidimensional, para que se confirme a redução efetiva da taxa de incidência de infeções da corrente sanguínea associadas ao uso de cateter venoso central.

[The Importance of Early Referral in Pediatric Acute Liver Failure]. / A Importância da Referenciação Precoce na Falência Hepática Aguda Pediátrica.

INTRODUCTION: Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus. OBJECTIVES: To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008. MATERIAL AND METHODS: Observational, retrospective study during a 20 year period (1994-2014). INCLUSION CRITERIA: age < 18 years old and acute liver failure (INR ≥ 2 without vitamin K response and hepatocellular necrosis). Children with previous liver disease were excluded. RESULTS: Fifty children were included, with median age of 24.5 months. The most common etiology under 2 years old was metabolic (34.6%) and above that age was infectious (29.2%). Forty six percent were submitted to liver transplantation and 78% of them survived. Overall mortality was 34%. Median referral time was 7 days in period A (n = 35) and 2 days in period B (n = 15; p = 0.006). Pediatric risk of mortality's median was 14.7 in period A and 6.5 in B (p = 0.019). Mortality was 37% vs 26% in periods A and B, respectively (p = 0.474). DISCUSSION AND CONCLUSIONS: Overall mortality was similar to the observed in other European centers. Liver transplantation is in fact the most effective therapeutic option. After 2008, there was a reduction in referral time and cases severity on admission; however, mortality has not reduced so far.

Introdução: A falência hepática aguda é uma doença rara associada a elevada morbilidade e mortalidade apesar do aumento da sobrevida devido ao transplante hepático. Em 2008, decorreu em Portugal uma reunião sobre esta patologia em pediatria, resultando num consenso de atuação que salientou a importância da abordagem multidisciplinar e referenciação precoce para um centro de transplantação hepática. Objetivos: Caracterizar as admissões por falência hepática aguda no Serviço de Cuidados Intensivos Pediátricos do centro português com transplante hepático pediátrico. Comparar resultados antes (A) e depois de 2008 (B). Material e Métodos: Estudo observacional retrospetivo de 20 anos (1994-2014). Critérios de inclusão: idade < 18 anos e falência hepática aguda (INR ≥ 2 sem resposta à vitamina K e necrose hepatocelular). Excluíram-se as crianças com doença hepática crónica. Resultados: Incluíram-se 50 crianças com idade mediana de 24,5 meses. A causa mais comum de falência hepática aguda abaixo dos 2 anos foi metabólica (34,6%) e acima infeciosa (29,2%). Foram submetidos a transplante hepático 46%, tendo sobrevivido 78%. A mortalidade global foi 34%. A mediana do tempo de referenciação foi 7 dias no período A (n = 35) e 2 no B (n = 15; p = 0,006). A mediana do risco de mortalidade prevista pelo PRISM foi 14,7% no período A e 6,5% no B (p = 0,019). A mortalidade foi 37% vs 26% no período A e B respetivamente (p = 0,474).Discussão e Conclusões: A mortalidade global foi sobreponível à de outros centros europeus, sendo o transplante hepático a opção terapêutica mais eficaz. Após 2008 o tempo de referenciação e a gravidade dos casos na admissão reduziram, ainda sem tradução significativa na mortalidade.

Overdose with antiepileptic drugs: the efficacy of extracorporeal removal techniques.

Drug overdose is a growing problem among adolescents. Clinical severity depends on the drug and ingested amount, which in some cases may be life-threatening. We present a clinical case of a previously healthy teenage girl who ingested 16.4 g of carbamazepine and 14.5 g of valproic acid. She presented with profound disturbance of consciousness and toxic levels of both drugs, raised in the first hours after the ingestion. She was successfully treated with charcoal haemoperfusion followed by continuous venovenous hemodiafiltration. Overdose with the two drugs separately is common, but there are no reports of intoxication by simultaneous ingestion. High levels of carbamazepine and valproic acid can lead to severe systemic effects and management is made difficult by the absence of specific antidotes. Extracorporeal removal techniques are a good therapeutic option in these cases as they enhance the clearance by reducing the half-life of both drugs thereby preventing serious complications.