Cantu syndrome, or hypertrichosis-osteodysplasia-cardiomegaly syndrome, is a rare disorder of unknown etiology, associated with hypertrichosis, characteristic facial features, skeletal abnormalities, cardiomegaly, and occasional pericardial effusions. Although autosomal recessive inheritance was originally proposed, a man with three affected children has been reported, making autosomal dominant inheritance likely. We report on a woman and her two daughters with Cantu syndrome, further confirming dominant inheritance. All three of our patients have cardiac involvement, and symptomatic pericardial effusions requiring surgical intervention occurred in the mother and one of her daughters. Chromosome microarray analysis was normal in one of the girls. The etiology of the cardiomegaly and pericardial effusions in Cantu syndrome is unknown. We review all previously reported cases of Cantu syndrome and the associated cardiac manifestations.
Childhood obesity is a major health care issue in the United States. This epidemic has important cardiovascular implications. Newer imaging modalities in obese adults have demonstrated abnormal systolic and diastolic cardiac function. The authors proposed to determine whether these abnormalities are present in obese children. A total of 168 children were identified from our echocardiographic database. Body mass index for age was calculated. Echocardiographic clips were analyzed using imaging technology to determine myocardial motion, strain, and strain rate. Patients at risk for obesity and those meeting criteria for obesity had increased late diastolic myocardial motion compared with normal-weight children. Obese patients had decreased systolic strain compared with normal-weight children. As with adults, obese children have significant differences in diastolic function and strain. These abnormalities may be subtle markers for the emergence of future cardiac disease.
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