[The QT syndrome. A family study of 4 generations].
Starting from two related children diagnosed with a syndrome of QT prolongation (Romano-Ward) a nearly complete family history was revealed in that 8 further patients with a QT-time over 115% of normal were found. Four additional family members had died of a sudden syncope at a young age, 2 of them in early childhood. No less than 6 sudden infant or early childhood deaths without clear cause were found in one generation of 12 members. This demonstrates the urgent necessity to examine the whole family with this syndrome in as much detail as possible. Quite often these syncopes are wrongly diagnosed as epileptic seizure. The treatment with beta-blocking medication very much improves the bad prognosis. This treatment should also include family members with QT-prolongation sofar without any symptoms.
Franek
A
A
Kinderklinik des Marienhospitals, Bottrop.
Böcker
H
H
Busam
A
A
D001145:Arrhythmias, Cardiac / Q000235:genetics*; D002648:Child; D003937:Diagnosis, Differential; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics*; D010375:Pedigree; D012307:Risk Factors; D013398:Sudden Infant Death
Das QT-Syndrom. Eine Familienstudie über vier Generationen.