Background: Kawasaki disease (KD) is a pediatric vasculitis, leading to coronary artery aneurysms (CAAs) in ~4-14%. Attention to the etiology and course of KD was generated by the close mimic of a SARS-CoV-2-induced phenotype, called multisystem inflammatory syndrome in children (MIS-C).
Methods: A total of 1179 cases were collected from 2012 with ~50% of cases retrospectively included.
Germline SMAD4 pathogenic variants (PVs) cause juvenile polyposis syndrome (JPS), which is known for an increased risk of gastrointestinal juvenile polyps and gastrointestinal cancer. Many patients with SMAD4 PV also show signs of hereditary hemorrhagic telangiectasia (HHT) and some patients have aneurysms and dissections of the thoracic aorta. Here we describe two patients with a germline SMAD4 PV and a remarkable clinical presentation including multiple medium-sized arterial aneurysms.
View Article and Find Full Text PDFArtificial intelligence (AI) broadly describes a branch of computer science focused on developing machines capable of performing tasks typically associated with human intelligence. Those who connect AI with the world of science fiction may meet its growing rise with hesitancy or outright skepticism. However, AI is becoming increasingly pervasive in our society, from algorithms helping to sift through airline fares to substituting words in emails and SMS text messages based on user choices.
View Article and Find Full Text PDFBackground: Children with SARS-CoV-2 related Multisystem Inflammatory Syndrome in Children (MIS-C) often present with clinical features that resemble Kawasaki disease (KD). Disease severity in adult COVID-19 is associated to the presence of anti-cytokine autoantibodies (ACAAs) against type I interferons. Similarly, ACAAs may be implicated in KD and MIS-C.
View Article and Find Full Text PDFKawasaki disease (KD) and SARS-CoV-2 related multisystem inflammatory syndrome in children (MIS-C) are clinically overlapping febrile syndromes based on clinical criteria. The diagnostic criterium of positive anti-SARS-CoV-2 serology will become inutile, as many children will have experienced COVID-19 infection or have been vaccinated. Given the risk of cardiovascular complications, alertness and timely treatment of both syndromes is very important in children with multiple days of unexplained fever.
View Article and Find Full Text PDFBackground: Approximately 25% of the patients with a history of Kawasaki disease (KD) develop coronary artery pathology if left untreated, with coronary artery aneurysms (CAA) as an early hallmark. Depending on the severity of CAAs, these patients are at risk of myocardial ischemia, infarction and sudden death. In order to reduce cardiac complications it is crucial to accurately identify patients with coronary artery pathology by an integrated cardiovascular program, tailored to the severity of the existing coronary artery pathology.
View Article and Find Full Text PDFBackground: Kawasaki disease (KD) is a pediatric vasculitis. Mainly the coronary arteries become affected due to acute inflammation and formation of coronary artery aneurysms (CAAs) can occur. The larger the CAA, the higher the risk for clinical complications and major adverse cardiac events, as the blood flow changes to vortex or turbulent flow facilitating thrombosis.
View Article and Find Full Text PDFKawasaki disease (KD) is a paediatric vasculitis associated with coronary artery aneurysms (CAA). Genetic variants influencing susceptibility to KD have been previously identified, but no risk alleles have been validated that influence CAA formation. We conducted a genome-wide association study (GWAS) for CAA in KD patients of European descent with 200 cases and 276 controls.
View Article and Find Full Text PDFKawasaki disease (KD) is an acute vasculitis that mainly affects the coronary arteries. This inflammation can cause coronary artery aneurysms (CAAs). Patients with KD need cardiac assessment for risk stratification for the development of myocardial ischemia, based on score (luminal diameter of the coronary artery corrected for body surface area).
View Article and Find Full Text PDFKawasaki Disease (KD) is a pediatric vasculitis of which the pathogenesis is unclear. The hypothesis is that genetically pre-disposed children develop KD when they encounter a pathogen which remains most often unidentified or pathogen derived factors. Since age is a dominant factor, prior immune status in children could influence their reactivity and hence the acquisition of KD.
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