The circulatory system was studied in 43 children (ages 3-18 years, mean 10.6 years; 32 girls, 11 boys) suffering from localized cutaneous forms of scleroderma. The following studies were undertaken: general pediatric examination, cardiologic examination including routine electrocardiogram (ECG), 24-h Holter ECG monitoring, echocardiography plus Doppler study, and a treadmill exercise test. Three children found to have congenital heart malformations were excluded from the study. Children with localized scleroderma were often lighter and shorter than their appropriate controls. The most common ECG abnormality was incomplete right bundle branch block, but other ECG and Holter abnormalities were found as well. Abnormal echocardiographic results were obtained in 16 cases. The abnormalities concerned valvar function (in all heart valves but predominantly in the mitral valve). It was found that the children suffering from scleroderma had different indices than controls for left ventricular mass index, mitral valve function and left ventricular filling. During the exercise test (conducted according to Bruce's protocol) we found no difference between patients and controls.
Ultrasonographic measurements of the urinary bladder in 21 women with systemic sclerosis (SSc) revealed abnormal volume of the bladder in about 65% of patients; 6/21 had a smaller bladder and 7/21 had a larger bladder than the controls. The volume of the remaining urine after miction was higher in 7 patients. Fibrosis of the bladder wall leading to a small rigid bladder and ureteral obstruction resulting in a large bladder with low contractility are suggested as phenomena involved in disturbed function of the urinary bladder in SSc patients. Patients with symptoms lasting more than 14 yrs. were found to have larger premictional volume of the bladder than those with short-lasting disease. No correlation between age, number of deliveries and bladder volume was found.
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