No abstract

Background: Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. So far, the presence of endothelial dysfunction in patients with no coronary lesions has not been demonstrated. Peripheral arterial tonometry (Endo-PAT) measures the microvascular function in response to local ischaemia and has been validated in adult population, but its use in children is scarce. Aim: To evaluate endothelial dysfunction in children as a long-term complication after Kawasaki disease using Endo-PAT. Methods: We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed for .5 years, with no coronary lesions, or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically accessed. Endo-PAT was performed to determine reactive hyperaemia index and augmentation index. Results: A total of 35 individuals (21 males, age 21 6 6 years) were evaluated (group 1: 19; controls: 16). Kawasaki disease patients presented significant lower reactive hyperaemia index (1.68 6 0.49 versus 2.31 6 0.53; p 5 0.001). Augmentation index was similar in both groups (210 6 7 versus 211 6 5; p . 0.005). Most patients with Kawasaki disease disclosed endothelial dysfunction (68%) compared with only 12% in controls. Conclusions: Endo-PAT is feasible and reproducible in the child population. Endothelial dysfunction is a frequent long-term complication in patients after Kawasaki disease with normal appearing coronary arteries. However, these results need validation in a larger population.

Introduction: Infantile hemangioma (IH) is one of the most common childhood tumors. There are various medical or surgical therapeutic options, all with suboptimal results. Recently, the successful use of propranolol for involution of IH was described. We report the results of a single-center experience with this therapeutic option. Objective: To prospectively assess the efficacy and safety of propranolol in children with infantile hemangioma. Methods:We performed a prospective analysis of clinical data of all patients with IH referred to a pediatric cardiology center for baseline cardiovascular assessment prior to propranolol therapy. Propranolol was given at a starting dose of 1 mg/kg/day and titrated to a target dose of 2---3 mg/kg/day according to clinical response. Efficacy was assessed through a photographbased severity scoring scale. Safety was assessed by collecting data regarding significant side effects. Results: Starting in 2010, 30 patients (15 female) were referred for propranolol treatment of IH, at a median age of six months (1---63 months). The mean target propranolol dose was 2.8 mg/kg/day, with a mean duration of therapy of 12 months. All patients experienced significant reduction of IH size and volume. There were no side effects. Conclusions: In our experience propranolol appears to be a useful and safe treatment option for severe or complicated IH, achieving a rapid and significant reduction in their size. No adverse effects were observed, although until larger clinical trials are completed, potential adverse events should be borne in mind and consultation with local specialists is recommended prior to initiating treatment. PALAVRAS-CHAVE Hemangioma infantil; Propranolol O papel do propranolol no tratamento dos hemangiomas em idade pediátrica ResumoIntrodução: Os hemangiomas são a lesão tumoral cutânea mais frequente em idade pediátrica. Até ao momento todas as opções terapêuticas (tanto médicas como cirúrgicas) têm resultados sub-ótimos. Recentemente, foi descrita a utilização de propranolol para tratamento dos hemangiomas. Relatamos os resultados da nossa experiência com esta opção terapêutica. Objetivo: Avaliação prospetiva da eficácia e segurança de propranolol em crianças com hemangioma infantil. Métodos: Avaliação prospetiva de todos os doentes com hemangioma referenciados para avaliação cardiovascular prévia ao início de terapêutica com propranolol. O propranolol foi administrado numa dose inicial de 1 mg/kg/dia e titulada para uma dose alvo de 2---3 mg/kg/dia, de acordo com a resposta clínica. A eficácia foi avaliada através de uma escala fotográfica. A segurança foi avaliada através da recolha de dados sobre os efeitos secundários significativos. Resultados: Desde 2010, 30 crianças (15 do sexo feminino) com hemangiomas foram referenciados para avaliação cardiovascular prévia ao início de terapêutica beta-bloqueante, com uma idade média de 6 meses (1-63 meses). A dose alvo média atingida foi de 2,8 mg / kg / dia, com uma duração média de tratamento de 12 meses. Em todos os doe...

Introduction: Infantile hemangioma (IH) is one of the most common childhood tumors. There are various medical or surgical therapeutic options, all with suboptimal results. Recently, the successful use of propranolol for involution of IH was described. We report the results of a single-center experience with this therapeutic option. Objective: To prospectively assess the efficacy and safety of propranolol in children with infantile hemangioma. Methods:We performed a prospective analysis of clinical data of all patients with IH referred to a pediatric cardiology center for baseline cardiovascular assessment prior to propranolol therapy. Propranolol was given at a starting dose of 1 mg/kg/day and titrated to a target dose of 2---3 mg/kg/day according to clinical response. Efficacy was assessed through a photographbased severity scoring scale. Safety was assessed by collecting data regarding significant side effects. Results: Starting in 2010, 30 patients (15 female) were referred for propranolol treatment of IH, at a median age of six months (1---63 months). The mean target propranolol dose was 2.8 mg/kg/day, with a mean duration of therapy of 12 months. All patients experienced significant reduction of IH size and volume. There were no side effects. Conclusions: In our experience propranolol appears to be a useful and safe treatment option for severe or complicated IH, achieving a rapid and significant reduction in their size. No adverse effects were observed, although until larger clinical trials are completed, potential adverse events should be borne in mind and consultation with local specialists is recommended prior to initiating treatment. PALAVRAS-CHAVE Hemangioma infantil; Propranolol O papel do propranolol no tratamento dos hemangiomas em idade pediátrica ResumoIntrodução: Os hemangiomas são a lesão tumoral cutânea mais frequente em idade pediátrica. Até ao momento todas as opções terapêuticas (tanto médicas como cirúrgicas) têm resultados sub-ótimos. Recentemente, foi descrita a utilização de propranolol para tratamento dos hemangiomas. Relatamos os resultados da nossa experiência com esta opção terapêutica. Objetivo: Avaliação prospetiva da eficácia e segurança de propranolol em crianças com hemangioma infantil. Métodos: Avaliação prospetiva de todos os doentes com hemangioma referenciados para avaliação cardiovascular prévia ao início de terapêutica com propranolol. O propranolol foi administrado numa dose inicial de 1 mg/kg/dia e titulada para uma dose alvo de 2---3 mg/kg/dia, de acordo com a resposta clínica. A eficácia foi avaliada através de uma escala fotográfica. A segurança foi avaliada através da recolha de dados sobre os efeitos secundários significativos. Resultados: Desde 2010, 30 crianças (15 do sexo feminino) com hemangiomas foram referenciados para avaliação cardiovascular prévia ao início de terapêutica beta-bloqueante, com uma idade média de 6 meses (1-63 meses). A dose alvo média atingida foi de 2,8 mg / kg / dia, com uma duração média de tratamento de 12 meses. Em todos os doe...