High-output heart failure in a newborn.

High-output cardiac failure is rare in newborns. Emergent diagnosis and management of this pathology is crucial. We report the case of a child, currently 12-months old; obstetric background is non-contributory. Clinic observation on D1 was normal except for the presence of a systolic cardiac murmur; cardiological evaluation revealed mild ventricular dysfunction of the right ventricle. On the third day of life, she developed cardiac failure with gallop rhythm, hepatomegaly and a murmur in the anterior fontanel; an echocardiogram confirmed clinic aggravation with biventricular dysfunction and right cavities and superior vena cava dilatation. The cranial MRI confirmed the presence of a pial arteriovenous malformation (AVM) involving the anterior and middle cerebral arteries with an associated fronto-parietal ischaemic lesion. The infant underwent embolisations of AVM with successful flow reduction and cardiac failure improvement. The multidisciplinary follow-up showed no cardiac dysfunction or permanent lesions but confirmed a severe psycho-motor delay and left hemiparesiainfo:eu-repo/semantics/publishedVersio

Cavernous transformation of portal vein

Introdução: na transformação cavernosa da veia portal (TCVP) as opções terapêuticas são escassas mas em casos seleccionados a construção de shunt fisiológico meso-Rex (SMR) é uma opção. Caso Clínico 1: Rapariga de 13 anos de idade, com síndrome polimalformativo. Internamento aos 4 meses de idade na Unidade de Cuidados Intensivos por pneumonia com repercussão hemodinâmica. Aos sete anos por trombocitopénia e esplenomegália realiza eco-doppler abdominal que revela TCVP. Aos 13 anos realiza SMR. Caso Clínico 2 : Rapaz de nove anos; isoimunização Rh grave neonatal com necessidade de exsanguinotransfusões. Desde os seis meses com esplenomegália e trombocitopénia com diagnóstico de TCVP aos três anos. Dada progressão clínica, aos nove anos é proposto realização de SMR, que não realiza por trombose vascular. Discussão: Para além do SMP, existem poucas opções terapêuticas para TCVP em crianças. No entanto dada a especificidade do procedimento é essencial o diagnóstico precoce

Tuberous sclerosis: two cases of a rare disease

Introdução: A Esclerose tuberosa (ET) é uma doença neurocutânea rara, caracterizada pela presença de hamartomas em vários orgãos. A gravidade e prognóstico dependem dos órgãos afectados e da velocidade de crescimento dos hamartomas. Caso Clínico 1: Adolescente de 15 anos, previamente saudável, admitida por quadro de dor lombar súbita. Ao exame ob- jectivo apresentava: lesões faciais papulares, mácula hipopigmentada lombar e dor na palpação dos quadrantes abdominais direitos. A ecografia e TAC abdominal identificaram presença de angiomiolipomas renais e hepáticos e a RM-CE documen- tou lesões do SNC confirmando o diagnóstico de ET. Caso Clínico 2: Lactente de 2 meses, admitido por epilepsia focal. À observação com hipotonia axial ligeira e múltiplas máculas hipopigmentadas. O EEG identificou foco epiléptico parietal direito. RM-CE documentou nódulos subependimá- rios e túberos corticais e a ecografia abdominal revelou múltiplos quistos renais. Medicado com terapêutica anticonvulsivante tripla. Conclusão: A ET tem um espectro clínico alargado, desde assintomática até doença grave e de mau prognóstico. É neces- sário a sua identificação precoce e seguimento regular para antecipar e tratar as complicações

Rabdomiólise como manifestação de uma doença metabólica: relato de caso

Rhabdomyolysis is a process of muscle destruction that can present with varying clinical manifestations. In pediatric patients, its main etiology is infectious diseases. We present a previously healthy adolescent who was admitted to our emergency department with a four-day history of myalgia, muscle weakness and dark urine. At presentation, she was dehydrated. Blood analysis revealed acute renal failure and increased muscular enzymes. She was transferred to our pediatric intensive care unit. Medical therapies for correction of dehydration and the ionic and metabolic consequences of renal failure were performed. Due to oliguria, renal replacement therapy was initiated. An etiological investigation revealed a beta-oxidation defect. Metabolic diseases are a known cause of rhabdomyolysis. Muscular destruction should be diagnosed early in order to avoid its potential consequences. Generally, the treatment of rhabdomyolysis is conservative, although in some situations, a more invasive approach is needed.info:eu-repo/semantics/publishedVersio

Myocardial dysfunction due to vitamin d deficiency

O défice de vitamina D manifesta-se geralmente por alterações na mineralização óssea. No entanto, este défice pode associar-se a outras alterações, como a insuficiência cardíaca. É apresentado o caso clínico de uma lactente com 3 meses de vida admitida na unidade de cuidados intensivos pediátricos com sinais de instabilidade hemodinâmica e necessidade de suporte ventilatório e inotrópico. A avaliação laboratorial inicial revelou uma hipocalcemia grave refratária à terapêutica instituída. O ecocardiograma foi sugestivo de insuficiência cardíaca. A investigação etiológica revelou um défice grave de vitamina D. O défice de vitamina D é um problema cada vez mais frequente nos dias de hoje. Perante uma hipocalcemia grave deve-se suspeitar desta deficiência

Presentations of children to emergency departments across Europe and the COVID-19 pandemic: A multinational observational study

BACKGROUND: During the initial phase of the Coronavirus Disease 2019 (COVID-19) pandemic, reduced numbers of acutely ill or injured children presented to emergency departments (EDs). Concerns were raised about the potential for delayed and more severe presentations and an increase in diagnoses such as diabetic ketoacidosis and mental health issues. This multinational observational study aimed to study the number of children presenting to EDs across Europe during the early COVID-19 pandemic and factors influencing this and to investigate changes in severity of illness and diagnoses. METHODS AND FINDINGS: Routine health data were extracted retrospectively from electronic patient records of children aged 18 years and under, presenting to 38 EDs in 16 European countries for the period January 2018 to May 2020, using predefined and standardized data domains. Observed and predicted numbers of ED attendances were calculated for the period February 2020 to May 2020. Poisson models and incidence rate ratios (IRRs), using predicted counts for each site as offset to adjust for case-mix differences, were used to compare age groups, diagnoses, and outcomes. Reductions in pediatric ED attendances, hospital admissions, and high triage urgencies were seen in all participating sites. ED attendances were relatively higher in countries with lower SARS-CoV-2 prevalence (IRR 2.26, 95% CI 1.90 to 2.70, p < 0.001) and in children aged <12 months (12 to <24 months IRR 0.86, 95% CI 0.84 to 0.89; 2 to <5 years IRR 0.80, 95% CI 0.78 to 0.82; 5 to <12 years IRR 0.68, 95% CI 0.67 to 0.70; 12 to 18 years IRR 0.72, 95% CI 0.70 to 0.74; versus age <12 months as reference group, p < 0.001). The lowering of pediatric intensive care admissions was not as great as that of general admissions (IRR 1.30, 95% CI 1.16 to 1.45, p < 0.001). Lower triage urgencies were reduced more than higher triage urgencies (urgent triage IRR 1.10, 95% CI 1.08 to 1.12; emergent and very urgent triage IRR 1.53, 95% CI 1.49 to 1.57; versus nonurgent triage category, p < 0.001). Reductions were highest and sustained throughout the study period for children with communicable infectious diseases. The main limitation was the retrospective nature of the study, using routine clinical data from a wide range of European hospitals and health systems. CONCLUSIONS: Reductions in ED attendances were seen across Europe during the first COVID-19 lockdown period. More severely ill children continued to attend hospital more frequently compared to those with minor injuries and illnesses, although absolute numbers fell. TRIAL REGISTRATION: ISRCTN91495258 https://www.isrctn.com/ISRCTN91495258

Genotoxic potential generated by biomass burning in the Brazilian Legal Amazon by Tradescantia micronucleus bioassay: a toxicity assessment study

<p>Abstract</p> <p>Background</p> <p>The Brazilian Amazon has suffered impacts from non-sustainable economic development, especially owing to the expansion of agricultural commodities into forest areas. The Tangará da Serra region, located in the southern of the Legal Amazon, is characterized by non-mechanized sugar cane production. In addition, it lies on the dispersion path of the pollution plume generated by biomass burning. The aim of this study was to assess the genotoxic potential of the atmosphere in the Tangará da Serra region, using <it>Tradescantia pallida </it>as <it>in situ </it>bioindicator.</p> <p>Methods</p> <p>The study was conducted during the dry and rainy seasons, where the plants were exposed to two types of exposure, active and passive.</p> <p>Results</p> <p>The results showed that in all the sampling seasons, irrespective of exposure type, there was an increase in micronucleus frequency, compared to control and that it was statistically significant in the dry season. A strong and significant relationship was also observed between the increase in micronucleus incidence and the rise in fine particulate matter, and hospital morbidity from respiratory diseases in children.</p> <p>Conclusions</p> <p>Based on the results, we demonstrated that pollutants generated by biomass burning in the Brazilian Amazon can induce genetic damage in test plants that was more prominent during dry season, and correlated with the level of particulates and elevated respiratory morbidity.</p