Multisystem Inflammatory Syndrome in Children (MIS-C) is a new clinical entity occurring in children and young adults, which is associated with the SARS-CoV-2 infection. The first cases of MIS-C were diagnosed in Poland in May 2020. Since October 2020, a significant increase in the incidence of this new disease has been observed in Poland, reflecting the increased incidence of COVID-19 in the paediatric population. MIS-C develops as a result of dysregulation of the immune system occurring 4 weeks after the SARS-CoV-2 infection. Diagnosis is based on the following criteria: a set of clinical features (including fever and signs of multiple organ damage) and elevated inflammatory markers, with exclusion of other causes. The most common complications involve the cardiovascular system: acute myocardial damage with reduced left ventricular ejection fraction, shock, and coronary artery abnormalities and arrhythmias. Mortality in Western Europe and the United States is around 1-2%. Appropriate management, including vital function support and immunomodulatory treatment, allows for a quick recovery in the vast majority of patients. This document is an updated guideline for the diagnostic and therapeutic management of children with suspected MIS-C in Poland. The most important changes concern treatment, steroid therapy, and antiplatelet therapy in particular.
This study provides further evidence that Aboriginal and Torres Strait Island children have a suboptimal response to recombinant hepatitis B vaccine. It also indicates that a considerable number of Aboriginal and Torres Strait Island children in the study age cohort have been exposed to HBV. However, despite these concerns, this study and historical data provide strong evidence that there has been a marked reduction in the prevalence of HBV infection and carriage in previously 'high risk' Aboriginal and Torres Strait Island children since the introduction of hepatitis B vaccines. Aboriginal and Torres Strait Island children who have been fully vaccinated in infancy do not require a booster dose of hepatitis B vaccine at school entry.
Pediatric inflammatory multisystem syndrome (PIMS) is a new entity in children, likely associated with previous coronavirus disease 19 (COVID-19) infection. Most of the reports about PIMS come from countries particularly hit by the COVID-19 pandemic. Our aim was to investigate the nature of inflammatory syndromes in Poland (country with low COVID-19 prevalence) and to perceive the emergence of PIMS in our country. On 25 May 2020, we launched a nationwide survey of inflammatory syndromes in children for retrospective (since 4 March 2020) and prospective data collection. Up to 28 July, 39 reported children met the inclusion criteria. We stratified them according to age (<5 and ≥ 5 years old) and COVID-19 status. The majority of children had clinical and laboratory features of Kawasaki disease, probably non-associated with COVID-19. However, children ≥5 years of age had PIMS characteristics, and nine children had COVID-19 confirmation. This is, to our knowledge, the first report of the PIMS register from a country with a low COVID-19 prevalence, and it proves that PIMS may emerge in any area involved in the COVID-19 pandemic. In a context of limited COVID-19 testing availability, other risk factors of PIMS, e.g., older age, should be considered in the differential diagnosis of inflammatory syndromes in children.
Fabry disease is a multisystemic X-linked lysosomal storage disorder, caused by the partial or complete deficiency of alpha-galactosidase A activity. The storage of glycosphingolipids in the vascular endothelium and in various tissues can lead to a broad spectrum of clinical manifestations. Renal failure, cardiovascular disease, and strokes are the main causes of morbidity and mortality. Gastrointestinal symptoms, although common, are often under-reported in the literature. This review covers the gastroenterological aspects of Fabry disease.
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