Current recommendations for the pharmacologic therapy in Kawasaki syndrome and management of its cardiovascular complications.

Kawasaki syndrome is a potentially life-threatening disease of early childhood that untreated holds a risk of severe coronary involvement. Its diagnosis is made via a list of clinical signs because etiology and pathophysiology are still unknown and no specific laboratory tool is available. Appropriate therapy with intravenous immunoglobulins and aspirin reduces the incidence of coronary abnormalities to less than 5%. Immunoglobulins have been shown to be highly effective in reducing disease symptoms or their severity and chiefly in reducing the rate of coronary artery aneurysm development. Aspirin is firstly used in high dose for its anti-inflammatory properties and then in low dose for its anti-thrombotic effects. Timely diagnosis and precociously administered treatment are two crucial points in the definition of prognosis for Kawasaki syndrome. In this review heart complications are discussed and therapeutic options stratified according to both severity of coronary involvement and grading of cardiovascular risk.

Outcome of newborns with asymptomatic monomorphic ventricular arrhythmia.

BACKGROUND: Frequent premature ventricular contractions (PVCs), couplets (CPLTs) and episodes of ventricular tachycardia are extremely rare in the neonatal population. Limited information is available with regard to clinical relevance and outcome. OBJECTIVES: To evaluate the clinical characteristics and outcomes of a group of newborns with ventricular arrhythmias without heart disease. PATIENTS AND DESIGN: Between January 2000 and January 2003, 16 newborns with ventricular arrhythmias in the absence of heart disease were studied. The newborns were divided into three groups: PVC group (n = 8), CPLT group (n = 4) and ventricular tachycardia group (n = 4). All patients underwent physical examination, electrocardiography, Holter monitoring and echocardiography at diagnosis and at follow-up (1, 3, 6 and 12 months, and yearly thereafter). RESULTS: Mean (standard deviation, SD) age of the patients was 3 (1.19) days in the PVC group, 3.25 (0.95) days in the CPLT group and 6.5 (9.1) days in the ventricular tachycardia group. Median follow-up was 36 months (range 24-48 months). PVCs disappeared during follow-up in all the neonates, in the PVC group, at a mean (SD) age of 2.1 (1.24) months; in the CPLT group, couplets disappeared at a mean (SD) age of 6.5 (1) months. All patients with ventricular tachycardia were treated; ventricular tachycardia disappeared at a mean (SD) age of 1.7 (0.9) months. Neither death nor complications occurred. CONCLUSIONS: Ventricular arrhythmias in newborns without heart disease have a good long-term prognosis. Frequent PVCs and CPLTs do not require treatment. Sustained ventricular tachycardia or high-rate ventricular tachycardia must be treated, but the prognosis is generally favourable.

Rheumatic heart disease in children: from clinical assessment to therapeutical management.

Rheumatic heart disease is still a relevant problem in children, adolescents and young adults. Molecular mimicry between streptococcal and human proteins has been proposed as the triggering factor leading to autoimmunity and tissue damage in rheumatic heart disease. Despite the widespread application of Jones' criteria, carditis is either underdiagnosed or overdiagnosed. Endocarditis leading to mitral and/or aortic regurgitation influences morbidity and mortality of rheumatic heart disease, whilst myocarditis and pericarditis are less significant in determining adverse outcomes in the long-term. Strategy available for disease control remains mainly secondary prophylaxis with the long-acting penicillin G-benzathine.

Herpes Virus Infections During Treatment With Etanercept in Juvenile Idiopathic Arthritis.

Incidence rates for varicella and herpes zoster were similar in patients with juvenile idiopathic arthritis receiving etanercept/methotrexate (n = 85, 184.9 patient-years [PY]) or methotrexate alone (n = 71, 199.4 PY); no complicated varicella or herpes zoster cases were reported; herpes labialis incidence was higher in patients receiving etanercept/methotrexate versus methotrexate alone (0.38 vs. 0.24 PY).

[Hepato-pulmonary syndrome and porto-pulmonary hypertension. Nosologic features and etiopathogenic considerations]. / Sindrome epato-polmonare e ipertensione porto-polmonare. Inquadramento nosologico e considerazioni eziopatogenetiche.

The liver controls pulmonary vascular tone by releasing vasoactive substances. In severe liver failure, the imbalance between vasodilator and vasoconstrictive mediators may lead to alteration of the respiratory function, called as hepatopulmonary syndrome (HPS, when a significant decrease of the vascular pulmonary resistance occurs, with development of intrapulmonary vascular dilatations) and portopulmonarv hypertension (PPH, when the vasoconstrictive prevalence, with an increase of the pulmonary vascular resistances). The clinical symptoms consist of various degree of dyspnea and hypoxemia. An overt "cor pulmonale" syndrome with right-side heart failure may be present in the most severe forms of PPH. The alteration of pulmonary vascularization may be diagnosed by means of pulmonary angiography, contrast-enhanced echocardiography and perfusion lung nuclear scanning of the lungs. Both clinical syndromes respond poorly to medical treatment, the unique therapeutical possibilities being represented by orthotopic liver transplantation (or combined heart-lung-liver or lung-liver transplantation in patients with severe PPH).

Is serum troponin T a useful marker of myocardial damage in newborn infants with perinatal asphyxia?

AIM: To assess the correlation of echocardiographic signs of myocardial damage to serum cardiac troponin T (cTnT) concentrations in newborn infants with perinatal asphyxia. METHODS: Electocardiograms (ECG) and echocardiograms (Echo) were obtained during the first 24 h of life from 29 asphyxiated and 30 control infants and correlated with cTnT concentrations. The echocardiographic parameters included systolic ventricular performance, preload, afterload, diastolic function, stroke volume (SV), left ventricular output (LVO), hyperechogenity of the papillary muscles and insufficiency of the atrioventricular valves. RESULTS: LVO and SV were lower but CTnT were significantly higher in asphyxiated than in control infants: 0.15 (010-0.23) vs. 0.05 (0.02-0.13), p < 0.001). Asphyxiated infants with signs of myocardial damage were associated with significantly higher cTnT than those without, 0.20 (0.11-0.28) and 0.11 (0.05-0.14 ug/L), p = 0.04. CONCLUSION: Cardiac troponin may prove to be valuable in evaluating myocardial damage in birth asphyxia. However, the degree of prematurity may complicate the assessment.