Objective To describe the significance of pericardial effusion (PE), mitral regurgitation (MR) and impaired systolic function in predicting coronary artery lesions (CAL) at diagnosis and follow-up in Kawasaki disease (KD).
Design Echocardiographic records on admission, at 1–3 weeks of illness, and at 6–8 weeks of illness were retrospectively retrieved in children with acute KD treated by intravenous immunoglobulins.
Setting, patients The study included 194 consecutive children (113 male; median age 2.1 years) in a paediatric cardiology tertiary care centre, from 1988 to 2007.
Results Overall, children with CAL (64/194) were more likely to have PE (OR=3.00, CI 1.34 to 6.72) and MR (OR=2.51, CI 1.22 to 5.16) at diagnosis; PE was the sole echocardiographic abnormality associated with CAL in multivariable analysis. These abnormalities were predictive of the presence of CAL at the first echocardiography in the acute phase of the disease only. MR, systolic dysfunction and PE were not associated with persistence of CAL in the convalescent phase. Male gender, CAL size and resistance to immunoglobulin treatment were independent factors predictive of the persistence of CAL.
Conclusions Children with MR or PE should undergo careful assessment of coronary status at diagnosis. However, PE or MR at diagnosis is not predictive of persistent CAL at follow-up.
"Patients who already have evolving coronary and/or peripheral aneurysms with ongoing inflammation at presentation. It is increasingly recognised that echocardiographic studies performed in the first week of KD may already show vessel abnormality, including brightness (suggesting inflammation) or dilatation when compared with age-related normal ranges and/or extracoronary manifestations, including mitral regurgitation and pericardial effusion.62 Patients with these features may also be at greater risk of CAA and, therefore, may require corticosteroids.62 "
[Show abstract][Hide abstract] ABSTRACT: Kawasaki disease (KD) is an acute self-limiting inflammatory disorder, associated with vasculitis, affecting predominantly medium-sized arteries, particularly the coronary arteries. In developed countries KD is the commonest cause of acquired heart disease in childhood. The aetiology of KD remains unknown, and it is currently believed that one or more as yet unidentified infectious agents induce an intense inflammatory host response in genetically susceptible individuals. Genetic studies have identified several susceptibility genes for KD and its sequelae in different ethnic populations, including FCGR2A, CD40, ITPKC, FAM167A-BLK and CASP3, as well as genes influencing response to intravenous immunoglobulin (IVIG) and aneurysm formation such as FCGR3B, and transforming growth factor (TGF) β pathway genes. IVIG and aspirin are effective therapeutically, but recent clinical trials and meta-analyses have demonstrated that the addition of corticosteroids to IVIG is beneficial for the prevention of coronary artery aneurysms (CAA) in severe cases with highest risk of IVIG resistance. Outside of Japan, however, clinical scores to predict IVIG resistance perform suboptimally. Furthermore, the evidence base does not provide clear guidance on which corticosteroid regimen is most effective. Other therapies, including anti-TNFα, could also have a role for IVIG-resistant KD. Irrespective of these caveats, it is clear that therapy that reduces inflammation in acute KD, improves outcome. This paper summarises recent advances in the understanding of KD pathogenesis and therapeutics, and provides an approach for managing KD patients in the UK in the light of these advances.
Archives of Disease in Childhood 10/2013; 99(1). DOI:10.1136/archdischild-2012-302841 · 2.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: La enfermedad de Kawasaki es una vasculitis sistémica aguda que afecta con preferencia al lactante y al niño de corta edad; supone un riesgo secundario de aneurisma coronario. Concierne al lactante y al niño menor de 5 años en el 80% de los casos (promedio de 26 meses), con un ligero predominio masculino. Los criterios diagnósticos principales son clínicos, con fiebre elevada durante más de 5 días, anomalías de los miembros (eritema/edema precoz y descamación más tardía), exantema, hiperemia conjuntival bilateral, anomalías bucales (queilitis, faringitis y lengua aframbuesada) y linfadenitis, con frecuencia asimétrica. La alteración del estado general es casi constante y a menudo marcada. También pueden contribuir al diagnóstico la existencia de una inflamación cutánea en el sitio de vacunación con BCG (bacilo de Calmette-Guérin), dolor, incluso una masa en el hipocondrio derecho (hidrocolecisto), y signos biológicos como un aumento franco de la proteína C reactiva (CRP), leucocitosis con neutrofilia y trombocitosis progresiva, a veces después de una fase precoz de trombocitopenia relativa. Las formas atípicas son numerosas, paucisintomáticas e incompletas, sobre todo en el lactante. También puede tratarse de situaciones en las que algunas manifestaciones habitualmente secundarias (dolores abdominales, hidrocolecisto, meningitis linfocítica, alteraciones hemodinámicas) ocupan el primer plano. La conducta terapéutica de primera elección consiste en la asociación de inmunoglobulinas polivalentes, en perfusión única de 2 g/kg, y aspirina en dosis alta durante la fase inflamatoria; el relevo se hace con aspirina sola en dosis antiagregante durante al menos 6 meses. La fisiopatología de la enfermedad es poco conocida. La inflamación mayor, caracterizada por una gran producción de citocinas proinflamatorias, podría estar desencadenada por un agente infeccioso en pacientes genéticamente predispuestos.
[Show abstract][Hide abstract] ABSTRACT: Objectives: To establish the optimal inflammation control of Kawasaki disease (KD), we investigated the clinical and pathophysiological basis of pericardial effusion (PE) during the acute phase of KD.
Method: Clinical and laboratory features of Japanese KD children with PE (PE group: n = 9) and without PE (non-PE group: n = 89) were studied retrospectively by using the medical records. Serum levels of soluble tumour necrosis factor receptor 1 (sTNFR1), interleukin 6 (IL-6), and vascular endothelial growth factor (VEGF) were assessed by enzyme-linked immunosorbent assays (ELISAs).
Results: PE group patients had coronary artery lesions (CALs) more frequently than non-PE group patients during the acute phase of KD (33% vs. 5.6%, p = 0.024). PE patients also showed lower levels of haemoglobin (p < 0.01) and serum albumin (p < 0.01) and higher platelet counts (p = 0.013) than non-PE patients. The proportion of neurological symptoms, but not other manifestations, in the PE group was higher than in the non-PE group (p = 0.022). All patients survived free from coronary artery aneurisms. Serum levels of sTNFR1, but not the other cytokines, in the PE group were higher than those in the non-PE group (p < 0.001). The sTNFR1 levels correlated positively with C-reactive protein (CRP) (r = 0.30, p = 0.019) or total bilirubin (r = 0.40, p < 0.01) levels.
Conclusions: Acute PE in KD patients indicated the severity of TNF-mediated vascular inflammation and concurrent CALs. According to the progression, these patients might need more targeted therapy of anti-inflammation for a better coronary outcome.
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