Surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval present at birth, and severe anaemia and syncopal attacks in childhood.
A case of the surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval in the electrocardiogram at birth, is described. The affected girl presented 3 1/2 years later with severe iron deficiency anaemia, despite apparently adequate nutrition. At the age of 4 1/2 years she had a sudden attack of unconsciousness. Treatment with the beta-adrenoceptor blocker practolol was started and 3 years after initiation of this treatment she has been free from syncopal attacks. The QT interval remains prolonged. Her brother also had severe iron deficiency anaemia and had several attacks of unconsciousness before he died suddenly at the age of 3 1/2 years.
D000433:1-Propanol / Q000627:therapeutic use; D000747:Anemia, Hypochromic / Q000209:etiology / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003638:Deafness / Q000150:complications / Q000151:congenital*; D004562:Electrocardiography; D005260:Female; D006331:Heart Diseases / Q000150:complications / Q000503:physiopathology; D006339:Heart Rate / Q000187:drug effects; D006801:Humans; D007231:Infant, Newborn; D007501:Iron / Q000008:administration & dosage; D011217:Practolol / Q000627:therapeutic use; D013575:Syncope / Q000150:complications / Q000188:drug therapy / Q000209:etiology; D013577:Syndrome
D000433:1-Propanol; D007501:Iron; D011217:Practolol