RESUMO
OBJECTIVES: To determine cardiovascular risk profiles of patients with Kawasaki disease and to relate them to a noninvasive measure of endothelial function. STUDY DESIGN: Case-control study. Cardiovascular risk assessment including brachial artery reactivity was performed in 24 patients 11.3 +/- 1.8 (mean +/- SD) years after Kawasaki disease and in 11 subjects in a normal control group. RESULTS: The case versus control groups were similar regarding age, sex, race, body mass index, and percentage of ideal body weight, although cases had a higher mean z score of body mass index than normal (+1.00 +/- 1.18; P <.001). Cases had normal fasting total cholesterol levels but a higher mean z score of triglyceride levels (+1.35 +/- 2.04; P <.004). The case group had significantly higher mean systolic and diastolic resting blood pressure z scores (+0.76 +/- 1.06; P <.01 and +0.96 +/- 1.19; P <.01, respectively) than the control group and population norms. Endothelial function as indicated by brachial artery reactivity was not significantly different between the case versus control groups. In the case group higher blood pressure, increasing adiposity, and higher fasting triglyceride levels were significantly interrelated but did not relate to brachial artery reactivity or coronary artery abnormalities. CONCLUSIONS: Patients after Kawasaki disease tend to have a more adverse cardiovascular risk profile potentially indicative of an increased predisposition to premature atherosclerotic changes.
Assuntos
Doenças Cardiovasculares/etiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Adolescente , Artéria Braquial , Doenças Cardiovasculares/epidemiologia , Estudos de Casos e Controles , Endotélio Vascular , Feminino , Seguimentos , Humanos , Modelos Lineares , Masculino , Ontário/epidemiologia , Fatores de Risco , Estatísticas não Paramétricas , VasodilataçãoRESUMO
Alström syndrome is an autosomal recessive disorder characterized by cone-rod dystrophy, obesity, hearing impairment, and diabetes caused by insulin resistance. By reviewing the charts of eight patients followed for periods of 2 to 22 years, we established the natural history of this syndrome during childhood. Five patients, in four families, were seen between the ages of 3 weeks and 4 months with a dilated cardiomyopathy, a previously unrecognized feature of the syndrome. Photophobia and nystagmus were first documented in the eight patients between the ages of 5 months and 15 months. In all patients, electroretinography initially showed a severe cone impairment with mild (2/8) or no (6/8) rod involvement. Electroretinograms, obtained again at ages 9 to 22 years for four patients, revealed extinguished rod-and-cone responses. Obesity developed during childhood in seven patients, in at least three of them before age 2 years. Hearing impairment (5/8) and diabetes/glucose intolerance (4/8) were diagnosed at the end of the first decade or during the second decade. This constellation of features should facilitate early diagnosis of the syndrome.