Retinopathy of Prematurity in a Neonatal Intensive Care Unit: Experience of Eight Years

Introdução: A retinopatia da prematuridade ocorre por inadequada vascularização da retina dos recém-nascidos prematuros. É uma doença multifatorial, com risco inversamente proporcional à idade gestacional e ao peso de nascimento. Objetivos: Caracterizar uma amostra de prematuros com critérios de observação oftalmológica e identificar os principais fatores de risco de retinopatia da prematuridade. Métodos: Estudo descritivo de análise retrospetiva. Consulta dos processos dos prematuros internados de 2005 a 2012 com critérios de observação oftalmológica e colheita de dados demográficos, fatores de risco, observação oftalmológica, estadio da retinopatia e tratamento. Análise estatística: SPSS®v21, significância: p<0,05. Resultados: Obtiveram-se 343 prematuros, 54% do sexo masculino. A incidência de retinopatia foi 15,5%, grave em 2,6%. O grupo com retinopatia teve média de idade gestacional de 27,5±1,9 semanas e peso de nascimento de 937±264 gramas e o grupo sem retinopatia teve, respetivamente, 29,8±1,9 semanas e 1216 ± 277 gramas (p<0,001). Os fatores com diferença entre os dois grupos foram: oxigenoterapia (p<0,001), ventilação invasiva (p=0,003) e sua duração (p<0,001), surfactante (p<0,001), dificuldade respiratória (p<0,001), sépsis tardia (p=0,019), persistência do canal arterial (p<0,001) e seu tratamento médico (p<0,001). A regressão logística mostrou que a idade gestacional é o fator de risco com maior repercussão na ocorrência de retinopatia (OR:0,542). O grau máximo de retinopatia foi 3 com doença plus, tendo 8 prematuros sido submetidos a cirurgia. Discussão e conclusão: A incidência de retinopatia foi inferior a outros estudos. Confirma-se a importância de alguns fatores de risco, sendo a idade gestacional o fator mais determinante.info:eu-repo/semantics/publishedVersio

Insuficiencia hepática aguda asociada a enfermedades metabólicas hereditarias en ninos pequenos

INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology. RESULTS: From 34 ALF cases, 18 were related to IMD: galactosemia (4), mitochondrial DNA depletion syndrome (MDS) (3), ornithine transcarbamilase deficiency (3), congenital defects of glycosylation (2), tyrosinemia type 1 (2), long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (1), hereditary fructose intolerance (1), classic methylmalonic aciduria (1) and citrulinemia type 1 (1). The median age was 1.3 months. At least one previous suggestive sign/symptom of IMD (vomiting, failure to thrive, hypotonia or developmental delay) was observed in 67% of the cases. The most common physical signs at admission included: hepatomegaly (72%), jaundice (67%) and encephalopathy (44%). The peak laboratorial findings were: mean international normalizad ratio 4.5, median lactate 5mmol/L, mean bilirubin 201μmol/L, median alanine aminotransferase (ALT) 137 UI/L and median ammonia 177μmol/L. One patient was submitted to liver transplant in ALF context (MSD). The mortality rate was 44%. DISCUSSION: The identification of IMD as a frequent cause of ALF allowed specific therapeutic measures and adequate family counselling. Particular clinical features and moderated ALT and bilirubin levels can lead to its suspicion.info:eu-repo/semantics/publishedVersio

[The Importance of Early Referral in Pediatric Acute Liver Failure]

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.info:eu-repo/semantics/publishedVersio

Transient neonatal hyperthyroidism

Graves’ disease is the main cause of hyperthyroidism in women of childbearing age. It occurs by the presence of serum immunoglobulins which stimulate the thyrotropin receptor (TRAbs) and may cross the placenta. It has serious consequences when uncontrolled, leading to fetal and/or neonatal hyperthyroidism or hypothyroidism. The authors describe the case of a newborn from a mother with poorly controlled Graves’ disease during pregnancy. He had an uneventful early neonatal period but developed hyperthyroidism in the second week of life. He was treated for two days with propranolol to manage tachycardia and metimazol during 4 months, with favourable clinical and laboratory outcome. During pregnancy, it is essential to control thyroid function and TRAbs in women with Graves’ disease. Newborns should be screened for thyroid function at birth and must have a regular follow up as it allows the diagnosis of transient hyperthyroidism or hypothyroidism and its early treatment, avoiding short and long term complications. Based on this case and literature review, the authors present a proposal of protocol in infants born to mothers with Graves’ disease

Anesthesiology Consensus in the Management of the Airway

Os consensos na gestão clínica da via aérea em anestesiologia pretendem disponibilizar informação, baseada na evidência atual ou, na falta desta, na opinião de peritos, no que respeita à abordagem da via aérea difícil previsível ou não previsível. Reforçamos a importância da avaliação da via aérea e da identificação de potenciais problemas que possam condicionar dificuldade na sua abordagem e a adoção de uma estratégia segura que permita identificar e responder em crescendo de intervenção às dificuldades encontradas. Na impossibilidade de intubação traqueal (não intubo) otimizada e limitada a 4 tentativas, da impossibilidade de ventilar e oxigenar (não oxigeno) após 2 tentativas de usar um dispositivo supraglótico ou de uso de máscara facial inicialmente adequada é importante realizar, em tempo útil, uma cricotirotomia para assegurar oxigenação. As situações clínicas de exceção só com planos simples, conhecidos por todos e regularmente treinados e adaptados à nossa atividade clinica podem assegurar melhores “outcomes”. O registo destes eventos e a informação ao nosso doente da dificuldade encontrada e modo como foi resolvido o problema é essencial e constitui ainda um desafio a alargar a uma base nacional.info:eu-repo/semantics/publishedVersio