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SARS-CoV-2 pandemics is characterized by a high level of infectivity and a high mortality among adults at risk (older than 65 years, obesity, diabetes, systemic hypertension). Following a common viral pneumonia, a multisystem inflammatory syndrome sometimes occurs, including an Acute Respiratory Distress Syndrome (ARDS) carrying a high mortality. Unlike most common respiratory viruses, children seem less susceptible to SARS-CoV-2 infection and generally develop a mild disease with low mortality. However, clusters of severe shock associated with high levels of cardiac biomarkers and unusual vasoplegia requiring inotropes, vasopressors and volume loading have been recently described. Both clinical symptoms (i.e., high and persistent fever, gastrointestinal disorders, skin rash, conjunctivitis and dry cracked lips) and biological signs (e.g., elevated CRP/PCT, hyperferritinemia) resembled Kawasaki disease. In most instances, intravenous immunoglobin therapy improved the cardiac function and led to full recovery within a few days. However, adjunctive steroid therapy and sometimes biotherapy (e.g., anti-IL-1Ra, anti-IL-6 monoclonal antibodies) were often necessary. Although almost all children fully recovered within a week, some of them developed coronary artery dilation or aneurysm. Thus, a new 'Multisystem Inflammatory Syndrome associated with SARS-CoV-2' has been recently described in children and helps to better understand Kawasaki disease pathophysiology.
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INTRODUCTION: In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants. METHODS: Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries. Pulmonary arteries were considered to be adequate when they measured > or = 4 mm. RESULTS: There were 8 deaths (17%). Complete repair was performed in 24 patients (18 in group III and 6 in group IV) with 79.3% had a right/left ventricular pressure <0.5, 16; 5%<1 and 4.2%>1. Eleven patients are waiting for complete repair and 4 will be operated on pulmonary arteries. One stage complete repair was performed in 3 patients, two and third stage repair (after unifocalisation or right ventricle to pulmonary arteries conduit) was attained in 21 patients. Among patients having had a complete repair and showing a right/left ventricular pressure <0.5, 88.9% had a pulmonary atresia with ventricular septal defect type III and 50% a pulmonary atresia with ventricular septal defect type IV. Only 56% of type III patients with a right/left ventricular pressure <0.5 had adequate central pulmonary arteries. CONCLUSION: In our study, the small size and the absence of central pulmonary arteries do not prevent a positive outcome.
Assuntos
Procedimentos Cirúrgicos Cardiovasculares/métodos , Comunicação Interventricular/etiologia , Comunicação Interventricular/cirurgia , Atresia Pulmonar/complicações , Atresia Pulmonar/cirurgia , Aorta , Circulação Colateral , Angiografia Coronária , Feminino , Comunicação Interventricular/patologia , Humanos , Recém-Nascido , Masculino , Prognóstico , Artéria Pulmonar , Resultado do Tratamento , Função Ventricular EsquerdaRESUMO
The late occurrence of complete atrio-ventricular block (CAVB) after cardiac surgery is rare but potentially responsible for cases of late sudden death. We searched for factors allowing prediction of this complication, retrospectively reviewing the case notes of 11 patients in hospital with complete AVB, 2 months to 10 years after correction of a cardiac malformation. All had a normal pre-operative ECG. The diagnosis had been made based on symptoms in 8 patients: syncope or collapse (4 cases) symptoms on effort (3 cases) or fatigue (1 case). In the others the diagnosis had been made on ECG. The block was infra-His in 5 patients who had electrophysiology. ECG analysis showed that all the patients had CAVB immediately post-operatively lasting 3-14 days. After restoration of conduction the ECGs showed the following anomalies compared to the pre-operative ECGs: long PR (1 case), long PR + right bundle branch block (2 cases), long PR + left axis deviation (1 case), RBBB + left deviation or rotation of the QRS axis (3 cases), long PR + RBBB + left axis deviation (4 cases). All of these patients had been fitted with a cardiac stimulator. In conclusion, the children who had CAVB immediately post-operatively lasting more than 48 hours and who then had an ECG showing different QRS compared to the pre-operative QRS and/or long PR had a risk of late complete AVB. These patients should have electrophysiology and a stimulator must be implanted in those who have an infra-His block.
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Bloqueio de Ramo/diagnóstico , Bloqueio de Ramo/etiologia , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias , Fascículo Atrioventricular , Bloqueio de Ramo/terapia , Criança , Pré-Escolar , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Fadiga/etiologia , Humanos , Lactente , Recém-Nascido , Marca-Passo Artificial , Estudos Retrospectivos , Fatores de Risco , Síncope/etiologiaRESUMO
Metabolic diseases are a rare cause of strokes. However, prevention and treatment are available for some of them. This work describes some metabolic diseases generating strokes by disturbing directly vascular function (homocysteine disorders, Fabry disease, congenital defects of glycosylation) and those for which clinical presentation is similar to a stroke (urea cycle disorders, branched-chain organic acidurias, mitochondrial diseases).
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Erros Inatos do Metabolismo/complicações , Acidente Vascular Cerebral/etiologia , Adulto , Erros Inatos do Metabolismo dos Aminoácidos/complicações , Doença de Fabry/complicações , Homocisteína/metabolismo , Humanos , Metilenotetra-Hidrofolato Redutase (NADPH2) , Oxirredutases atuantes sobre Doadores de Grupo CH-NH/deficiênciaAssuntos
Transtornos Mentais/etiologia , Erros Inatos do Metabolismo/complicações , Erros Inatos do Metabolismo/psicologia , Doenças do Sistema Nervoso/etiologia , Doença Aguda , Criança , Pré-Escolar , Diagnóstico Diferencial , Humanos , Lactente , Recém-Nascido , Erros Inatos do Metabolismo/diagnóstico , Fatores de TempoRESUMO
Tritiated cholesterol is rapidly converted to labelled ecdysone in vitro by prothoracic glands from last instar larvae of Locusta at the time of the maximum endogenous hormone increase of the insects. Glands from larvae with low hormone content or fat body fragments do not make similar conversions.
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Ecdisona/biossíntese , Gafanhotos/metabolismo , Animais , Colesterol/metabolismo , Gafanhotos/anatomia & histologia , LarvaRESUMO
A series of 117 cases of Pierre Robin Sequence are classified as isolated (48%), syndromic (35%), and with associated anomalies (17%); the latter group had a poor long-term prognosis. In isolated Pierre Robin Sequence, familial cases and a high incidence of twins were noted. Among syndromic Pierre Robin Sequence, 4 syndromes represent more than 50% of the diagnoses.