Kawasaki disease (KD) is an acute, self-limited, systemic vasculitis of unknown aetiology, extremely rare in infants younger than 6 months old. Younger infants are more likely to present with incomplete KD (IKD) and are at higher risk of developing coronary abnormalities. An early and specific clinical sign, not included in the classical diagnosis criteria, but that can be very useful in the diagnosis of KD, is the reaction at the Bacillus Calmette-Guérin (BCG) inoculation site. We describe a case of a 4-month-old boy, fully immunised, whose BCG scar reactivation led to the diagnosis of IKD. This case-report emphasises the importance of BCG site reactivation in establishing a diagnosis of IKD that clinicians should be aware of, especially in countries where BCG vaccination is still part of the immunisation schedule.
Introduction:Complex congenital heart disease is a group of severe conditions. Prenatal diagnosis has implications on morbidity and mortality for most severe conditions. The purpose of this work was to evaluate the influence of prenatal diagnosis and distance of residence and birth place to a reference center, on immediate morbidity and early mortality of complex congenital heart disease.
Material and Methods:Retrospective study of complex congenital heart disease patients of our Hospital, born between 2007 and 2012. Results: There were 126 patients born with complex congenital heart disease. In 95%, pregnancy was followed since the first trimester, with prenatal diagnosis in 42%. There was a statistically significant relation between birth place and prenatal diagnosis. Transposition of great arteries was the most frequent complex congenital heart disease (45.2%), followed by pulmonary atresia with ventricular septal defect (17.5%) and hypoplastic left ventricle (9.5%). Eighty-two patients (65.1%) had prostaglandin infusion and 38 (30.2%) were ventilated before an intervention. Surgery took place in the neonatal period in 73%. Actuarial survival rate at 30 days, 12 and 24 months was 85%, 80% and 75%, respectively. There was no statistically significant relation between prenatal diagnosis and mortality. Discussion: Most patients with complex congenital heart disease did not have prenatal diagnosis. All cases with prenatal diagnosis were born in a tertiary center. Prenatal diagnosis did not influence significantly neonatal mortality, as already described in other studies with heterogeneous complex heart disease. Conclusion: prenatal diagnosis of complex congenital heart disease allowed an adequate referral. Most patients with complex congenital heart disease weren't diagnosed prenatally. This data should be considered when planning prenatal diagnosis of congenital heart disease. Keywords: Heart Defects, Congenital; Prenatal Diagnosis.
INTRODUÇÃOAs cardiopatias congénitas são as malformações congénitas major mais comuns e constituem o grupo com maior relevância clínica, com uma prevalência de 0,5% a 1% no período neonatal.1-3 Cerca de metade destas cardiopatias são graves e necessitam de intervenção no período neonatal ou nos primeiros anos de vida.
2As cardiopatias congénitas complexas (CCC) constituem um grupo de doenças graves e incluem na sua grande maioria uma alteração do arranjo segmentar, sendo exemplos o síndrome de coração esquerdo hipoplásico (SCEH), transposição das grandes artérias (TGA) e o coração univentricular. De entre as cardiopatias congénitas,
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