Why the study?
Is Romano-Ward syndrome linked to specific HLA haplotypes in affected families?
Is Romano-Ward syndrome linked to specific HLA haplotypes in affected families?
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The genes for Romano-Ward syndrome appear to be closely linked to the major histocompatibility complex on the short arm of chromosome 6, specifically associated with the HLA haplotype A9-Bw54.
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 histocompatibility complex on the short arm of chromosome number 6.
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Itoh et al. (1982) studied this question.
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