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A case of junctional epidermolysis bullosa (Herlitz) is reported. The patient has been treated with high doses of Dapson. The result is encouraging.

The cases of two patients with Bland-White-Garland-Syndrome are reported. A 13 year old girl had been operated during infancy (ligation of the left coronary artery at its origin from the pulmonary artery) and showed reopening of the ligation with marked collateral circulation between the right and left coronary artery. She was operated again, successfully. A second critically ill patient with a typical pattern of anterolateral infarction in his ECG recovered so well that only five years later he was readmitted; then he showed the adult-type ECG with good collateral circulation between the right and left coronary artery. He underwent an equally successful operation. In both cases there is good evidence for the development of a sufficient collateral circulation after ligation of the coronary artery to pulmonary artery connection. Spontaneous formation of anastomoses between the right and left coronary artery enables even infants with this anomaly to survive. These findings confirm the suggestion that the "infancy-type" and "adult-type" of the Bland-White-Garland-Syndrome represent only different stages of the same disease.

Cardiac function was measured in 10 children (mean age 12 years) undergoing chronic intermittent hemodialysis. Blood pressure, heart rate and body weight were checked three times before and after hemodialysis. Echocardiographic studies were performed according to the references of the American society of echocardiography. 6 patients were normotensive, 4 patients hypertensive. Cardiac function was normal before and after hemodialysis in all patients. Ultrafiltration reduced body weight on average by 1.2 kg. The mean systolic blood pressure remained unchanged, the mean diastolic blood pressure declined in normotensive patients from 66 mm Hg to 53 mm Hg and in hypertensive patients from 100 to 85 mm Hg on average; the systolic leftventricular diameter (LVES) was reduced by dialysis from 2.7 to 2.3 cm, the enddiastolic leftventricular diameter (LVED) from 4.42 to 4.02. Cardiac output declined from 62.04 ml to 56.68 ml/min, whereas heart rate, shortening fraction and mean circumferential fiber-shortening-time increased. In normotensive patients LVED shortened more (4.4 to 3.86 cm) than in hypertensive children (4.44 to cardiac output 4.18 cm), but there were no significant differences. Significantly decreased only in the normotensive children. In hypertensive children the shortening fraction increased significantly (35.54 to 42.48%) in contrast to normotensive children (40.84 to 38.44%). Cardiac preload (LVES) and (LVED) and afterload (diastolic pressure) was reduced significantly after volume elimination. These changes in cardiac function suggest, that cardiac alterations found before hemodialysis are only functional and not structural.

A 12 year old boy developed protein losing enteropathy 6 months after surgery for a severe cardiac malformation complex by a modified Fontan procedure. Gastrointestinal protein loss was due to sustained venous pressure elevation. Conservative therapy with diet, diuretics and albumininfusions remained ineffective. The patient died from right atrial and pulmonary thrombembolism 10 months after surgery.