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A Study of the Inheritance Pattern of Romano-Ward Syndrome

Prolonged Q-T Interval, Syncope, and Sudden Death

Department of Pediatrics. Niigata University School of Medicine, Niigata City (Dr. Munemura), Yokohama Kowan Hospital

S. Munemura

Department of Pediatrics. Niigata University School of Medicine, Niigata City (Dr. Munemura), Yokohama Kowan Hospital

H. Satoh

Department of Pediatrics. Niigata University School of Medicine, Niigata City (Dr. Munemura), Yokohama Kowan Hospital

Romano-Ward syndrome consists of attacks of syncope, possible sudden death, accompanied by prolonged Q-T interval on ECG. We carried out HLA antigen typing of a family of 16 members having ten cases of Romano-Ward syndrome. All the ten cases had the common HLA haplotype of A9-Bw54, while the six not showing the symptoms did not have A9-Bw54 haplotype. The genes of this syndrome, considered to be inherited through the autosomal dominant patterns, are assumed to have a close linking to the major histo compatibility complex on the short arm of chromosome number 6.

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